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1 47 Hypospadias Warren T. Snodgrass, MD, and Nicol Corbin Bush, MD, MSCS Preoperative Assessment and Management Complications Intraoperative Assessment and Management| Hypospalas Reoperatons Postoperative Management Adult Outoomes after Prepubertal Repair Outcomes Assessment | ypospadias refers toa urethral opening proximal to the normal flanlar location. The defect is commonly considered arrested evelopment, even though embryo penises do not exhibit a similarappeating phase, Correction is surgical and includes not only urethroplasty but also straightening ventral penile curvature, ‘rcumcision or prepucioplasty, and scrotoplasty with the goal to restore as normal function and appearance as possible. ‘We discuss hypospadias from a surgical perspective in this chapter, with sections discussing preoperative assessment, intraop- ‘erative decision making and management, postoperative care, and ‘complications and their reoperations. Given that complications from initial surgery increase the risk for subsequent complications, ‘we have described operative techniques in detail, emphasizing key steps to reduce the likelinood that additional surgery will be needed ‘Within each section we briefly recount the best studies available and summarize this evidence in bold type. PREOPERATIVE ASSESSMENT AND MANAGEMENT Diagnosis Hypospadias is diagnosed by physical examination, Typically pre pputial development is asymmetrical, with a dorsal "hood" and ‘ventral deficiency that exposes the glans and proximal meatus (Fig. 147-1). Other abnormal ventzal findings potentially include dowa- ‘ward glans tlt, deviation of the median penile raphe, ventsal cue vvatuze (VC), scrotal encroachment onto the penile shall, midline scrotal cleft, and penoscrotal transposition, The main differential diagnosis is chordee without hypespadias, ‘which refers to asymmetrical preputial development with a normal glanular meatus. The term implies ventral penile curvature, although in the majority of cases apparent downward bending is corrected simply by degloving the ventral skin, This categorization. has included patients with a glanular meatus but deficient compus spongiosum and a thin distal urethra that others consider a hypo: spadias variant. To end confusion, boys with a hooded prepuce and bending should be diagnosed with congenital VCif the urethra is grossly nommal, or otherwise with hypospadias (Snodgrass, 2008). Some hypospadias variants present with a normal foreskin ‘concealing a glanular to distal shaft meatus, These generally have a deeply grooved urethral plate, which sometimes extends later ally under the skin edge creating a phenotype known as the megameatus with intact prepuce (Pig. 117-2). The diagnosis of ‘these vasiants is made alter citcumcision or when the foreskin becomes retractable Improving Outcomes Prevalence and Inheritance Hypospadias occurs in 1 in 300 males (0.39), Recurrence tisk is approximately 13 times greater in first-degree relatives (brothers, fathers, offspring), Several bist segiaties suggested an increasing prevalence in the 1990s, possibly linked to environmental toxins, but changes in and accuracy ofthe diagnosis potentially account ‘Three case-control studies of births in Denmark, France, and ltaly reported prevalence of hypospadias in 0.3% t0 0.45% of male births. The relative risk for recurrence in first-degree relatives was 13, times greater, found in 9% to 17% of brothers and 1% to 3% of fathers. Rskin same-sex twins was 50%. Recurrence risk in offspring ‘was the same as in first-degree relatives (Calzolari etal, 1986; Stoll etal, 1990; Schnack et al, 2008). Isolated versus Syndromic Hypospadias Approximately 90% of hypospadias cases are isolated penile defects, Case-contol studies indicate that in most patients hypospadias is an isolated anomaly (Calzolari etal, 1986; Stoll etal, 1990), Syndromic hypospadias is suspected with development delay, dys- morphic facies, andjor anorectal malformation. Examples include: + Smith-Lemli-Opite syndrome—results from an autosomal reces five mulation of the DIICR7 gene on chromosome Iql3 coding for 7-dehydrocholesterol reductase. Affected individuals hhave mental retardation, facial dysmorphism, microcephaly, and, syndactyly, 1+ WAGR syndrome (Wilms tumor, anividia, genital anomalies, mental retardation) —results fom a deletion in chromosome 11p13, + G syuirome (Opite G/8BB syndrome)—occurs from X-linked ‘mutations in the midline-1 gene of autosomal dominant dele- sions in chromosome 22411. The resultant phenotype includes hhypertelorism, tracheoesophageal defects, cleft lip/palate, and, mild mental retardation + WoifHtinchhom syndrome—derives fom deletions in chromo. some 4p resulting in mental retardation, seizures, abnormal facies, and midline detects + 13q deletion syndrome—characterized by mental retardation, facial dysmorphia, imperforate anus, and hypospadias with penoscrotal transposition, + Hand foot-uterus syndrome—an autosomal dominant condition caused by mutations in the HOXAI3 gene on chromosome ‘7p14-15, resulting in bilateral thumb and great toe hypoplasia. 3399 3400 PART XV Pediatric Urology Figure 147-1. Spectrum of hypospadias. A, Coronal hypospadias. B, Penoscrotal hypospadias. , Perineal hypospadias with scrotal transposition. i. 4 Figure 147-2. Hypospadias variants with a normal prepuce. A, Apparently normal penis with ‘complete foreskin. B, Foreskin retracted, revealing a coronal hypospadias. C, Glanular mot ‘meatus intact prepuce discovered after circumcision. Disorders of Sex Development Disorders of sex development (DSDs) are possible in pheno typic boys with both hypospadias and undescended testes, which is considered an indieation for karyotyping. The most common diagnosis is mixed gonadal dysgenesis, followed by ovotesticular DSD. Isolated hypospadias is not considered a DSD. The coexistence ‘of hypospadias with undescended testes may indicate a DSD, expe- cially when there is proximal hypospadias and a nonpalpable tests, although the prevalence is dilheult to determine ffom published reports, which are all retrospective and subject to selection bias for evaluation. Reasons for evaluating some but not other patients with hypospadias and undescended testes were not described in the articles summarized here. For example, a review by Kacler and colleagues (1999) identified ‘79 male-appearing patients with both hypospadias and undescended testes of whom only 54 (689%) were evaluated by karyotyping. Of the 79 patients, 23 (29%) were diagnosed with a DSD, comprising mixed gonadal dysgenesis ( = 11), ovotesticular DSP (n = 5), Socreductase deficiency (n = 2), Klinefelter syndrome (n= 2), and Partial androgen resistance (ni ‘Two other studies conceming hypospadias with undescended testes similarly reported karyotyping in 4296 and 57% of patients, finding either autosomal or sex chromosome anomalies in 1796 and 2496, respectively. OF the total of 157 patients represented in these ‘wo reports, mixed gonadal dysgenesis occurred in 5 and ovotestica lar DSD in’1 (McAleer and Kaplan, 2001; Cox etal, 2008) ‘We harvest tunica vaginalis to cover the neourethra in all primary proximal and staged reoperative repaits, and have occasionally encountered an ovotestis fully descended in the scrotum in phenotypic boys with 46XY karyotype. In such cases we remove the ovarian tissue and explore the contralateral testis then of subsequently. Imaging Isolated hypospadias, regardless of severity, is not considered an indication for urinary tract imaging, ‘One prospective study in Saudi Arabia obtained intravenous pyelography and voiding cystourethrography in patients less than 2 ‘years of age, reporting results in 153 boys with glanular to perineal hypospadias over an Il-year period ending in 1983. Of these, 35 (2492) had abnormal findings, incuding vesicoureteral reflux (a= 18) and a variety of upper urinary tract conditions, including horse shoe kidney, solitay kidney, ureterovesical junction obstruction, and ureteral duplication. Surgery was thought to be indicated in 18 ff the 36 patients (12%) (Moore, 1990) ‘wo retrospective series obtained intravenous pyelography or renal sonography in 41% and 72% of patients, both reporting 18% to be abnormal. Possibly significant findings of hydronephrosis ‘occurred in 49% and 19% (Lutaker et al, 1977; Faedman etal, 2008), Voiding cystourethrography in 163 cases, of which 47% were peno- scrotal, diagnosed vesicourcteral reflux in 6 patients (1%) and bladder diverticulum in 2. There was no mention of prostatic utile (Friedman et al, 2008), ‘We do not obtain either renal sonography or voiding gystoure thrography in boys with nonsyndromiec hypospadias, regardless of ts severity, ‘Age for Surgery Hypospadias repair can be performed as an outpatient procedure in otherwise healthy full-term babies 3 months of age or older. Considerations in determining the timing of operation include anesthetic risks, psychosexual factors, and the potentially varying risk for urethroplasty complications at different ages. Anesthetic Risks Bush and colleagues (2012) reported no unplanned hospital admis sions for anesthetic complications in 230 babies 3 to 5 months of age, in whom bronchospasm was documented in the anesthetic record in 5 cases (2%). Preterm babies can undergo outpatient surgery after 36 gestational weeks Psychosexual Risks ‘The American Academy of Pediatrics recommended suy completed by age 18 months to limit paychosemual stress ("himing of elective surgery,” 1996). However, a study that used question naires and a standardized interview by a psyehologist to compare patients ages 6 to 17 years operated before versus after 18 months found no differences in health-related quality of life, psychological adjustment, gender role behavior, or penile self perception (Weber ‘et al, 2009) Urethroplasty Complications Various reports suggest urethroplasty complications increase with increasing patient age, although the time at which this increased risk occurs is not clear In contrast, our data question if age is an independent risk factor for complications. This subject is discussed later under Risk Factors in the section on Complications. Preoperative Androgen Stimulation ‘Androgens increase penile length and glans circumference, with varying duration of effect alter stimulation ends. Only two studies concern the impact of preoperative androgen therapy on ‘urethroplasty complications, one finding a significant reduction in those treated and the other reporting complications remained increased in those stimulated to increase glans size versus those hhaving the same glans size without treatment, ‘Androgens are documented to increase penile length and glans circumference. However, only one published tial reports the impact Chapter 147 Hypospadas 3401 of therapy on urethroplasty complications, Otherwise, most series used subjective criteria in selecting patients for stimulation, used ‘empirical treatment regimens, and had no objective end point, ‘We (Bush et al, 2013; Snodgrass et al, 2014b) found that ure throplasty complications increased in patients with glans width less than 14 mm, and based on that observation instituted a pro: tocol using testosterone gypionate intramuscular injections to increase width to 15 mm or greater Of 62 consecutive boys with ‘midshaft and proximal hypospadias, 5 of 15 (33%) and 29 of 47 (60%), respectively, were treated. Initially testosterone 2 mg/kg was given for two or three injections, with all midshaft cases but only’ 43% of proximal cases achieving the desired glans width, indicating relative androgen resistance The protocol was changed to administer an escalating dove of testosterone from 2 to 32 mal kg per injection based on remeasurement 1 month after each injection. Next Bush and colleagues (2013) analyzed urethroplasty com: plications in patients who received adjuvant testosterone injections versus those with glans 1d mm or greater who did not. Mean glans width before stimulation was 12 mm, increasing to a mean Of 16.5 mm with testosterone injections. Untreated patients had a mean glans width of 15.4 mm, Urethroplasty complications Sccurted in 34% with versus 1196 without adjuvant androgens (P< 0001), Because the goal of therapy was to reduce complica: tions, we stopped preoperative testosterone stimulation, In contrast, a trial by Kaya and coworkers (2008) randomized, 75 consecutive boys of mean age 33 months (range 10 to 159) to preoperative topical dibydrotestosterone (2.5% to glans and shalt daily for 3 months) versus no therapy. Treated versus control patients had coronal (70% vs. 84%), penile (2496 vs. 16%), and penoscrotal (5% vs_0%) hypospadias, and all underwent tubular: ized incised plate (TIP) urethroplasty. Chi-square analysis reported fewer urethroplasty complications in those patients receiving adju vant stimulation (1 of 37 vs 9 of 38, P=.01), Hypospadias Encountered During Newborn Circumcision Caregivers desiring newborn circumcision should be assured that the newborn with a normal prepuce can undergo the procedure without concern for a concealed hypospadias, and that circumcision should not be stopped if hypospadias is encountered, ‘Original descriptions of the megameatus intact prepuce variant warned that circumcision should be avoided when this vatiant is iscovered, although a review of the literatare found no case men tioned in which the prepuce was needed for repair. We have been referred infante whose circumcision was stopped when the practi tioner erroneously thought there was a urethral defect, requiring general anesthesia to complete the procedure. An evaluation of Concealed hypospadias repair in those with versus without prior cixcumdsion found no difference in urethroplasty complications, “Therefore there is no reason to stop circumcision in a newborn with ‘a normal prepuce even if a concealed hypospadias is suspected (Snodgrass and Khavari, 2006) INTRAOPERATIVE ASSESSMENT AND MANAGEMENT General Aspects of Surgical Repair Sutures ‘There is no evidence that suture materials impact uretheoplasty complications ‘Guarino and associates (2009) compared primary distal TI Sistula rates for two types of sutures, randomizing 100 boys to either polyglyione (rapid absorption) of polydioxanone (slow absorp tion). All operations were done by one surgeon, performing ure throplasty in two layers using running subepithelial stitches Follow-up assessment was blinded to suture type. At 2 years after repair there was no difference inthe fistula rates: 4 of 50 (8%) with polyglytone versus 6 of 50 (1296) with polydioxanone, 3402 PART XV. Pediatic Urology We prefer 7.0 polyglactin for urethroplasty because the TG-140 needle is significantly smaller than the needle available on 7-0 polydioxanone. Perioperative Antibiotics ‘There are no trials concerning preoperative antibiotics before hypospadias surgery. A single trial reported that febrile uri- nary tract infection (UTI) was reduced by postoperative oral antibiotics, ‘Meir and Livne (2004) randomized 101 patients undergoing TIP ‘urethroplasty to intraoperative intravenous cefonicid versus intra- ‘operative cefonicid plus postoperative oral cephalexin 3 times daily for 8 days during urinary diversion. Urethroplasty complications were the same, but febrile UTT occurred less often in those treated with oral antibiotics (3 of 52 vs. 12 of 49, P= 05). We do not use preoperative antibiotic exeept when harvesting ‘oral mucosa, in which case we administer intravenous cefazolin, Patients with postoperative urinary diversion are given trimethopri sulfamethoxazole during catheterization Nerve Blocks ‘One randomized controlled trial (RCT) reported penile block to be superior to caudal nerve block for distal hypospadias repair, Penile engorgement was more likely after caudal blocks. ‘A double blind RCT by Koncra and colleagues (2012) allocated 154 boys ages 4 to 12 years with distal hypospadias to either penile ‘oF caudal nerve block using 0.254% bupivacaine afer induction of general anesthesia, Duration of surgery was similar in both groups at 68 4 15 minutes. A significant mean arterial pressure increase ‘occurred after surgical incision in the caudal versus penile block groups, although only 1 patient war considered to have a failed block: Duration of block was significantly (82 minutes) fonger in the penile versus caudal group (302 + 25 minutes vs. 220+ 23 ‘minutes, P = 00) and there was 43% less postoperative morphine use afler penile black Penile engorgement was determined by measuring. stretched penile length and midshaft circumference before and 10 minutes after block, Mean penile volume increased 27% with caudal versus 2.5% with penile block (P-<.001) (Kanda etal, 2012}, For distal hypospadias we use a dorsal penile nerve block supple mented by a second midline scrotal injection, because infrapubic Dlodks do not reach sensory branches innervating the ventral ‘midline penis and scrotal and perineal area (Kundra etal, 2012} Caudal nerve blocks are used in proximal cases. When a caudal block cannot be performed, penile and scrotal blocks are used, with a wider area of infiltation at the scrotal base and an additional region of injection superolateral to the scrotum on the side where tunica vaginalis will be harvested. Urethral Plate Assessment ‘One study found poor agreement between surgeons judging ure- thral plate suitability for TIP urethroplasty from photographs. ‘Three studies reported that designation of the urethral plate {groove as fat, intermediate, or deep did not predict urethroplasty ‘complications, Perhaps inevitably, some surgeons wish to rate urethral plate “quality” as a factor in theit selection of surgical technique. When done, such subjective assessment makes comparisons between seties difficult because various surgeons likely would characterize the plates differently. Supporting this concern was a study by f= Hout and colleagues (2009) invalving 21 pediatric urologists who were asked to review photographs and rate the suitability of the ‘urethral plate for IP urethroplasty using a Likert scale. The authors found poor to slight agreement between the responses regardless of ‘meatal location (distal, midshaft, proximal) or years of surgical experience (kappa = 0.06) “Three studies characterized the urethral plate groove as fa, inter- ‘mediate, ot deep, finding no differences in distal TIP urethroplasty Figure 147-3. Urethral plato unsuitable fr tubularization. The arrow Indicates unilateral absence of the corpus spongiosum. Tubularized incised plate urethroplasty was considered a poor option without the usual subepithelial tissues comprising the urethral plate. ‘complications (Holland and Smith, 2000 Sathan etal, 2009; Snod ‘gas etal, 2010). Two of these studies reported that urethral plate preincision width les than 8 mm predicted increased urethroplasty ‘complications. In one the final neourethra calibrated to less than 6 French in 1986 of cases (Hollane and Smith, 2000), which we ‘suspect indicates inadequate midline TIP incision. In the other both distal and midshaft patients were included (Sashan esl, 2009), but ‘the results were not adjusted for meatal location, which also impacts ‘outcomes, We ate currently measuring urethral plate width at its ‘widest point under stretch and find that only 109 are greater than 8mm. It TIP urethroplasty is contraindicated by width less than 8 mm, reported complications should be much greater Snodgrass and colleagues (2010) reported outcomes in 551 con- secutive boys presenting with distal hypospadias, Only TIP urethro- plasty was done, with no contraindication encountered, Similarly ‘we have used TIP urethroplasty for nearly all proximal cases with VC less than 30 degrees regardless ofthe appearance ofthe urethral plate In 7% of proximal cases there was intraoperative recognition Df rigidity or deficient subepithelial tissues for tubularization (ig. 147-3) (Snodgrass and Bush, 2011), Admittedly this is subjective, so we emphasize our intention to use TIP urethroplasty in all primary proximal hypospadias cases in which the urethral plate is hot transected to achieve penile straightening, Ventral Curvature Prevalence Only 10% of distal hypospadias cases have VC that is less than. 30 degrees after degloving. Approximately 50% of proximal hypospadias eases have either no VC or VC less than 30 degrees ater degloving, whereas the other 30% have greater than 30 degrees afer degloving Preoperative assessment cannot accurately predict either the extent of curvature or the means required for straightening. Appar- ent bending may improve ot resolve asthe skin is degloved,s0 the common maneuver of compressing penopubic and penoscrotal tissues atthe base ofthe penis to beter visualize the penile shaft tay falsely suggest or exaggerate VCby action on deficient vena skin, The finding that kin adjacent tothe meatus may retract as far as the penoscrotal junction during degloving provides additional, proof that relatively short ventral skin Contributes to curvature. Snodgrass and colleagues (2010) performed artificial erection in. 4440 boy with distal hypospadias after degloving, Gnding VC that ‘was Jest than 30 egret in 119% and lateral bending in another 2%. In no case was bending greater than 30 degrees by visual estimation Snodgrass and Prieto (2009) studied 70 consecutive boys with prox smal sha to perineal hypospadias, Nineteen percent had no VC and 31% had less than 30 degrees bending after the penis was egloved and scrotal attachments were dissected. The other 50% hhad curvature greater than 30 degrees. These reported degrees of curvature were derived by visual est ‘mation without objective measurement, which we have found cum: bersome to perform. Few publications regarding VC report using protractors to accurately determine degrees of bending. Significance Several studies of men with Peyronie disease or congenital curvature report that those desiring surgical straightening had 25 degrees o areater bending (Savoca etal, 2000; Gholami and Lue, 2002; Green Feld etal, 2006) Attiticial Erection Heparinized saline injected into the corpora was first described by Gittes and McLaughlin (1974) to create an erection intraoperatively in men with Peyronie disease, and subsequently was adapted for use in boys with hypospadias, ‘We perform artifical etection during hypospadias repair after egloving, dissection of ventral dartos, and release of the corpus spongiosim wings fiom the underlying corpora cavernosa and glans wings in proximal hypospadias because shortened ventral Shaft skin, dartos, and spongiosum can contribute to apparent VC. Nozmal saline is injected into a single cozpora using a 25-gauge buttery needle until erection is achieved. We do not use a tournt quet because occasionally t can mask curvature if positioned at the point of bending. If compresion is needed to slow fluid outflow and obtain erection, manial pressure is applied below the base of the penis, pressing the coxpora against the crura Criticisms of saline injection include supraphysiologic or sub» physiologic itracorporea! filling that would over- or underestimate ‘curvature: Vasoactive druge have also been injected to induce erec tion during hypospadias repair (Perovic et al, 1997; Kogan, 2000), ‘with the proposed advantage of being a more physiologic assese ‘ment in contrast to saline injection, We have no experience with this method. ‘Means for Correction VC less than 30 degrees is straightened by dorsal plication. VC greater than 30 degrees after degloving, dissection of ventral artos and scrotal attachments, and division of the corpus spon: giosum wings near their fision with the glans wings next leads {o urethral plate transection and dissection to the meatus. Per sistent VC greater than 30 degrees can be corrected by ventral corporotomies with or without grafting. ‘We correct VC less than 30 degrees with a single midline dorsal plication using either 5-0 or 6-0 polypropylene. Should plication fail, this extent of VC is unlikely to binder sexual activity. We do not use multiple plications to straighten VC greater than 30 degrees out of concern that results may not be durable into adulthood Rather, when VC greater than 30 degrees persists after the penis is egloved, ventral dartos dissected, and corpus spongiosum released from the compora and its fusion to glans wings, the urethral plate is, transected at the corona and freed proximally to the meatus. Then artificial erection is done and persisting VC less than 30 degrees is Corrected by dorsal plication, whereas VC greater than 30 degrees leads to three transverse corporotomies through the area of greatest, bending, ‘Although single comporotomy with grafting can also be done for ventral lengthening when VC exceeds 30 degrees, this limits ure throplasty options to flaps because a urethroplasty graft placed onto a corporal graft may not take. Because we choose not to perform fap urethroplasties, we lengthen the shortened ventral tunica albu ginea of the corpora using three corporotomies without grating Chapter 147 Hypospadas 3403 Figure 147-4. Midline dorsal plication. The surface of the corpora cavernosa is exposed in the midline opposite the region of curvature, avoiding the dorsal veins, and then polypropylene suture is placed, burying the knot Surgical Technique Dorsal Plication. Midline plication is illustrated in Figure 147-4, Attifical erection identifies the point of greatest bending, and the [Buck fascia is incised there longitudinally to expose the underlying tunica albuginea. single 60 or 5-0 polypropylene stitch plicates the midline septum of the corpora, burying the knot. Repeat rection confirms straightening. We donot perform multiple plications Results, Recurrent VC was reported in two series in 7% of patients. One stated that all recurrences occurred in plications ‘done for VC greater than 30 degrees. Penile shortening less than (0.5 em occurred in adults having a mean of three plications, “Two setroxpectve studies using 5-0 (one also used 4-0) polypro- pylene both reported recurrent VC in 7% of patients during median follow-up of 16 months (Chertin et al, 2004) and 6 years (Bar Yosef ct al, 2001). Chertin and colleagues (2004) did not state the extent ‘of VC, whereas Bar Youe! and associates (2004) used one of two ‘midline plications for VC estimated as less than 30 degrees in 47%, from 30 to 45 degrees in 44%, and greater than 45 degrees in 9% of patients. All patients with recurzent VC had greater than 30 degrees initially, including two of four with VC greater than 45 degrees (Bar Yosef et al, 2004). ‘One retrospective study involved 154 men with either Peyronie disease or congenital curvature who had objective measurement of both curvature and penile length before and after straightening (Greenfield ec al, 2008), Mean curvature determined intraopera tively using papaverine and saline injection and measured using a protractor was 45 degrees (range 25 t0 105) for Peyronie disease and 57 degrees (range 25 to 90) for congenital curvature. An average of thrce plications (range one to six) was done. Mean penile length loss was 0.36 cm (range 0 to 2.5) Although itis frequently stated that dorsal plication shortens the penis, this study suggests that any loss in length is likely subclinical 3404 PART xv Pediatric Urology Figure 147-5. A, Ventral corporotomies to straighten curvature greater than 30 degrees 8B, Three transverse corporotomies are made just through the tunica albuginea from 4 to 8 o'clock (arrows). Then the urethroplasty graft is placed over these incisions. Ventral Corporal Lengthening. There are two methods for ventral corporotomy. Results appear equivalent, but options for subsequent urethroplasty are impacted by which technique is used, ‘A single ventral comporotomy can be made from 3 t0 9 ofdock ‘though the area of greatest bending The opening in the tunica albuginea is patched using a graft, Reported mateuals for grafting include dermis from the groin (hernia-ike incision), small intestine submucosa, and tunica vaginalis (as either a graft or flap). Incision with grafing requires flap urethroplasty because a graft uretheo plasty would involve placing the urethroplasty graft onto the cor- poral gral, which likely would not adequately revascularize Altematively, the ventral corpora can be incised from 4 to 8. ‘o'clock beginning Uhrough the point of greatest curvatute and then ‘making similar incisions approximately 4 mm distally and proxi- ‘mally fora total of three (Fig. 147-5). These incisions through the tunica albuginea are not grafted, and so a urethroplasty graft can be placed direcly over them. Results, Retrospective studies all find recurrent curvature in less ‘than 109% of patients following ventral lengthening by single corporotomy with graiting. One study reported no difference in ‘outcomes from single corporotomy with grafting versus three corporotomies without grafting. One adult with corporotomy plus grafting before puberty reported erectile dysfunction requir- ing vasoactive drugs. ‘Snodgrass and Prieto (2009) reported outcomes in 18 consecu- tive boys with proximal hypospadias, the fist 7 having corporot ‘omy with dermal grafting and the next 11 having three transverse corporotomies without grafting There was no recurrent curvature with either method during follow-up of 27 and 19 months, respec- tively. This observation demonstrates that corporotomies for ventral lengthening do not require grafting ‘Results of single cosporotomy with grafting suggest there is lite difference in recurrent curvature regardless of the material used to graft the defect For example, three reviews concerning dermal grafts reported no recurrent curvature requiring additional straightening during follow-up of 2 to 10 years (Pope et al, 1996, Caesar and Caldamone, 2000; Badawy and Motsi, 2008). Badawy and Morsi (2008) studied 16 postpubertal men after prepubertal corporotomy with dermal grafting and stated that one of the three who reported sexual activity needed vasoactive drug comporal injections to main lain a sullident erection. To our knowledge there reports of erectile dysfunction after incision with gr authors stated that this individual recovered natural etections after their publication (Badawy, personal communication). Both singleply and 4-ply small intestine submucosa have been ‘used for corporal grafting. Of three reports, two observed no secur- rent VC during follow up of 1.5 to 3 years (Weiser et al, 2003; Elmore e¢ al, 2007). A dhied review afer 4-ply grafting stated that 17% of patients had either recurrent VC ora palpable fibrotic mass deemed necessary to excise (Soeigel et al, 2003). ‘Three studies found recurrent VC in 10% or less of patients following grafting using tunica vaginalis (Perlmutter etal, 1985; Ritchey and Ribbeck 2003; Kajbafzadeh etal, 2007). One other ssudy used tunica vaginalis as a flap with similar results (Braga ‘ec a, 2007) The “Thin” Urethra ‘The urethra proximal to the meatus may appear “thin” for a varying distance owing to absent or deficient dartos and corpus spongiosum covering (Fig. 17-6), Management depends in part fon whether a surgical plane can be established to separate the overlying shaft skin from the urethra, and also on the extent of ‘VC when present. Most often the “thin” segment is only a few millimeters long and the shaft skin can be separated from it. In ‘that circumstance urethroplasty is not influenced except that spongioplasty can be done (o cover the thin-appearing region, ‘When shaft skin cannot be separated from the urethra, the “thin” ‘urethra is split down the midline proximally until normal spon- ‘giosum is encountered, and then the sti-atlached skin is incor- porated into the neourethra. In the presence of VC greater than 30 degrees after degloving, the urcthral plate and the “thin” ‘urethra are excised as part of the straightening process, Algorithm for Hypospadias Urethroplasty Figure 117-7 shows the algorithm for bypospadias repair based on. tubularization of the urethral plate or a neoplate substitute. All hypospadias can be repaired using either of two operative tech- niques: TIP and two-stage graft urethroplasty Inlay graft procedures ate a vatiation on TIP urethroplasty. Two-stage gralis ute prepuce fr oral mucosa depending on clinical circumstances (discussed late), Flap options are shown in Figure 147-7 with dashed lines. A ‘comparison of laps versus grafts is summarized later. We choose to not tse flaps because cosmetic eaults appeat to be inferior to these other alternatives Distal Hypospadias Repair Tubularized Incised Plate Urethroplasty Indications. Snodgrass and colleagues (2010) used TIP urethro- plasty as the only repair for 551 consecutive patients with distal U-shaped skin incision should Chapter 147 Hypospadas 3405 2 mm proximal to the meatus. fates and spongiosum between shaft skin and urethra (arrow). Initial lateral and proximal to the visible urethra, ~ UP maintained i VG «30° no gross scar Disa Proxal Roaperaton| vo>s0° [ Grogs searing 8x0 ae Veo 00: seen Haire wrth UP prevousy excised] [Stage orga ‘son sip ih no sor Toy ora grat Figure 147-7. Algorithm for hypospadias urethroplasty. BXO, balanitis xerotica obliterans; TIP, ‘ubularized incised plate; UP, urethral plate; VC, ventral curvature. hypospadias, finding no contraindication to the technique. As dis cussed in the earlier Urethral Plate Assessment section, three studies reported no difference in TIP outcomes whether the groove was deep, intermediate, or fat (Holland and Smith, 2000; Sarhan etal, 2008; Snodgrass et a, 2010), Two ofthese stated that a narrow plate (<8 mm) increased ‘complications (Holland and Smith, 2000; Sashan etal, 2009), but we believe this indicated failure to ade quately incse the plate Surgical Technique (Fig. 147-8). Skin management varies accor ing to family preference for circumcision versus prepucioplasty. Because most request circumcision in the United States, we illus- trate that method in this section and describe prepucioplasty sepa rately later, “The glans width is determined at its widest point using calipers (Pig. 147-9) and then a 5-0 polypropylene stay stitch is placed. The comets of the dorsal prepuce are held and the line for incision is ‘marked. Ventrlly the incision is approximately 2 mm below the ‘meatus, ot more proximal if the distal urethra is found by sound ing to appear “thin” from deficient underlying dartos and corpus spongiosum (Tig. 147-10) For glanular cases without fusion of the glans wings, the incision is made a few millimeters below the corona, The oblique dorsal incision is made to preserve sufficient inner prepuce to transfer ventrally and create a uniform "collar" resembling a normal circumeision (Fil, 1987), Degloving is done in different planes: dorsally along the Buck fascia and ventrally just under the shaft skin, preserving available dartos. Dissection continues to the penopubic and penoscrotal junctions. Artificial erection is done, and if curvature less than 30 degrees is demonstrated it is comrected by dorsal plication as described earlier. fext a coumiquet is placed at the base of the penis and the visible junctions of the glans wings to dhe urethral plate are marked, ‘These lines are injected with 1:100,000 epinephrine and incised using a Beaver 69 blade (Beaver Visitec International, Waltham, MA): Dissection continues down to the surface of the corpora and then laterally on each side to approximately 3 and 9 o'clock. If the glans width i less than 14 mm, orf there is tension on glans wings approximation after this “standard” mobilization, then additional 3406 PART XV. Podiatric Urology E F Figure 147-8, Distal tubularzed incised plate repair. A, Gircumscribing skin incision. ‘sions along the visible junction ofthe glans wings tothe urethral plate. C, Incision ofthe urethral plate extending to near the underlying corpora. D, Tubularizing the urethral plate from distally to proximally. Note thatthe first stitch isa ‘an oval apeni rneomeatus ant ut 3 mm proximal tothe end of the plate, ereating E, The neourethra is covered with a dartos flap. F, Glansplasty creating the tinuing down to the corona. G, Repair and circumcision completed. (From ‘Snodgrass WT. Snodgrass technique for hypospadias repair. BJU Int 2005;95:685-03}) Figure 147-8, Measuring glans width. Glans width less than 14 mm predicts increased urethroplasty complications. “extended” dissection is next done at 3 and 9 o'clock, farther releasing the sings for a distance of approximately 4 mm distally (ig 147-11), ‘The urethral plate is held on either side and gently stretched laterally using 0.5 Castroviejo forceps. Tenotomy scissors incise the ‘midline from within the meatus to the tip ofthe plate dawn to the surface ofthe underiying corpora. Depth of incision varies acord- ing to the preexstent plac groove; a flat plate requires @ desper dissection than an already deeply grooved plate. Distal plate inci ‘sion may leave a small shelf at the glans junction but should not ‘extend into the glans, which is distinguished by its more dll and ‘granular appearance (Fg. 47-12) ‘A Gebrstent is passed into the bladder and ted to the glans trac tion suture: Urethral plat tubularization is done in wo subepithe lial layers using 7-0 polyglactin on a TG-140 needle. The st stch is placed distally approximately 3 mm below the end of the plate to create an oval, not a rounded, opening, Suturing farther distally ineeases tik for iatrogenic meatal stenosis. Continuous sitching proceeds proximally to the meatus where iti Ged, and then the Same suite returns distally forthe second layer. Next a ventral dartos flap is raised, split into two longitudinal segments when possible, and crossed over the neaurethra to provide two-layer cov" rage. We tack this ito place using 90 polyglactin Glansplastyapproxitiates the wings with 6-0 polyglactin subepi- thelial interupied stitches, beginning distally and continuing othe corona proximally. Most often three stiches are placed. Iti not necessary to additionally suture the epithelium, which could leave visible marks, If there is tension on ths closure afer usual glans ‘wings mobilization, the sbches ate removed and an extended mobilization is done as described previously. The glans wings are nocsututed tothe undetying neouethra even though thee is gaP between the tabularized end of the urethral plate and the Bret distal stitch of the glans that creates the meomestus (Fig 147-13). This fap heals spontancousy. Residual venta shat skin attached tothe inner prepuce is then cased and the “collar” approximated Using 7-0 polyglactin inter rupted subepithelial stitches (see Fig. 147-10C and D)-and usally 2 single epithelial 9-0 polyglacun sich atthe corona. The dossal prepuce is split in the midline to the edge of the inner prepatial Colac and then fixed inthe midline using 7-0 polyglactin subep- thelial stitch. The vental midline skin is closed to re-create a median raphe, and remaining excess skin laterally on either side is excised Chapter 147 Hypospadas 3407 c Figure 147-10. Skin incision for circumcision. , Ventral ine. C, Perimeatal shaft skin to be excised before the inner preputial “collar” is made ventrally. D, Completed repair “with circumeision demonstrating preputial collar. Figure 147-11. Glans wings dissection. , “Standard” mobilization along the corpora trom the Urethral plate medially toward 3 and 9 o'clock laterally. B, “Extended” mobilization with addi- ‘onal dissection at 3 and 9 o'clock for about 4 mm distally along the corpora. The arrows indicate the surface of the corpora exposed by this further moblizatio to complete circumcision. All skin edges are closed with subepithe. are availabe elsewhere. The most common include the meatal advance lial stitches. We use urinary diversion into diapers for approximately ment and glanuloplasty incorporation (MAGDI) and Mathicu or 1 week, flip-fap techniques. Alternative Methods. Multiple methods have been described for The MAGPI proceduce is an operative technique for glanular and istal hypospadias that remain in use in various centers Descriptions coronal hypospadias in which the urethral plate is cut in the dorsal 3408 PART XV Pediatric Urology Figure 147-12. Urethral plate incision extends deeply to near the ‘corpors, from within the meatus to the end of the plate alstally (arrow). Distal glans ‘losure Distal urtial ‘bulaizaton Figure 147-18. Tubularized incised plate (TIP) glansplasty. The most distal stitch approximating glans Wings, creating the neomeatus, usually is beyond the most distal stitch of the tubularized urethral plate. Its not necessary to sulure the glans wings to the urethral plate in TIP repair. ‘midline and then the dorsal meatus is advanced distally and sutured. Next the ventral lip of the meatus is pulled distally and the slang closed beneath it (Duckett, 1981) “The Mathiew procedure, of Mip-flap, is an_ operative technique {or distal hypospadias in which a rectangular fap is outlined proxi rally from the meatus on the ventral penile shaft, and then ele- vated and sutured distally to the urethral plate (Mathieu, 1932) ‘This repair is typically stented for a shore period, Results. Most articles report complications in less than 10% of cates after distal TIP urethroplasty. Snodgrass and colleagues (2010) reported outcomes in 426 boys. a mean of § months after distal TIP repair had been performed by Snodgrass. Astessment included calibration, uroflowmetry, and/or urethroscopy done in 279 patients (65%). Urethroplasty complica tions occurred in 19 patients (436), including nine fistulas, nine glans dehiscences, and one meatal stenosis that developed later from balanitis xerotica obliterans (BXO). There were no strictures cor diverticula, Aaystematic literature review by Wilkinson and associates (2012), included 15 articles on distal TP urethroplasty from 1994 through 2008, comprising 1872 boys. The authors reported 4% fistulas and 534% meatal stenoses with no urethral stricutes, Glan dehiscence ‘was not reported ‘Snodgrass (2011) also reviewed 36 articles on distal TIP urethro- plasty published in English between 1994 and 2009. Reported com- plications ranged from none to 24%, with 25 of the articles reporting 10% or less, mostly fistulas and meatal stenoses Proximal Hypospadias Repair Decision Making Choice of technique for proximal hypospadias repair is largely determined by the extent of VC after degloving and excision of any scrotal extensions onto the penile shaft. Options for urethroplasty ‘when there is curvature less than 30 degrees include TIP repair and ‘onlay preputial lap, Curvature greater than 30 degrees resulting in ‘wansection of the urethral plate for straightening limits urethro~ plasty options to single-stage tubularized preputial aps, wwo-stage Dreputial laps, or two-stage preputial graft repairs. Tubutarized incised Plate Urethroplasty Indications. Proximal TIP urethroplasty can be done when there is VC less than 30 degrees. Greater curvature prompts urethral plate transection and so precludes TIP repair. As discussed in the eatlier Urethral Plate Assessment section, in approximately 7% of cases the plate lacks sufficient subepithelial tissues to tubulatize, ors subjec- tively rigid and therefore unsuitable to fashion the neourethra. Surgical Technique (Fig. 147-14). Proximal TIP urethroplasty can be done with either eizcumeision or prepucioplasty. In this section we describe the operation as performed when circumeision is requested by the family; prepucioplasty ie discussed later, ‘The glans width is first measured and then a 5-0 polypropylene stay sutch is placed. The dorsal line for incision excends adjacent to the corona approximately 3 mm proximally, preserving most of the inner prepuce for use as a graft should there be either VC greater than 30 degrees or a urethral plate not suitable for TIP urethro- plasty. Ventrally the incision runs in a U shape alongside the plate, avoiding visible hair follicles, and then continues dovin the midline ff the scrotum, The ventral incision lines adjacent to the urethral plate are injected with 1: 100,000 epinephrine to minimize bleed- Ing from underlying corpus spongiosum. The penis is degloved 10 the penopubic and penoscrotal junctions. All ventral dartos and scrotal attachments are dissected off to the bate of the penis. ‘Next the glans wings are marked along their junction with the ‘urethral plate and also injected with 1: 100,000 epinephrine before incision, The glans wings are dissected laterally along the surface of the corpora cavernosa to 3 and 9 o'clock In patients with a glans ‘width less than 14 mm of with tension of glans wings approxima- UUon, dissection is extended along the corporal bodies distally for about 4 mm (see Fig. 147-11). The attachments of the corpus spon- _gosum wings tothe ipsilateral glans wings on eitherside are divided ‘The spongiosum on either side of the urethral plate is farther die- sected off the corpora cavernosa for subsequent spongioplasty ‘Atlifcial erection is done and VC is addvessed a8 discussed earlier When the penis is straight and the urethral plate conserved, the plate is incised dorsally from the meatus to its distal end extend ing to near the underlying corporal bodies. A G-Fr stent is passed into the bladder and tubularization is done in two subepithelial layers, the first using an interrupted 7-0 polyglactin stitch and the second a continuous 7-0 polydioxanone stitch. ‘Spongioplasty approximates the corpus spongiosum wings over the neourethra, Then a hemiscrotum is entered and the testis ‘exposed. The tunica vaginalis is opened wansversely and stay stitches are placed into its distal comers. A stay is also placed in the adventitia near the inferior pole of the testis for countertraction. A tunica vaginalis ap is created by dissecting along the spermatic cord to near the exennal ring, Faty scrotal tissues are excluded Chapter 147, Hypospadas Figure 147-14. Proximal tubularized incise lurethral plate in patient desiring circumcision. B, After degloving, {rom the urethral plate. Corpus spongiosum is dissected from incision. D, Two-layer urethral plate tubularization using interrupted subepithelial 7-0 polyglac- ‘tin followed by running 7-0 pelydioxanone. E, Spongioplasty approximates divergent corpus spongiosum over the neourethra, bef grass WT. Snodgrass technique for hypospadias rey (Pig. 147-15). The testis is retumed to its normal position and suture pexed into place, and then its compartment is closed. The flap is brought over the neourethra, shiny surface down, and tacked swith 7-0 polydioxanone. Glansplasty is done in one layer using 6-0 interrupted subepi. thelial polyglactin, usually with three stitches from distal to the corona. As described for distal TIP urethroplasty, the glans wings fre not sutured to the neourethra, ‘Shaft skin attached to the inner prepuce ventrally is excised (see Fig. 147-10C) and the preputil collar is completed using inter rupted subepithelial 7-0 polyglactin and one epithelial 9-0 polygla un suture a the corona, Then the dorsal prepuce is divided in the midline to the level of the preputial collar and sutured there using 70 subepithelial polyglactin, Ventrally the penoscrotal junction ‘ypically is incised to approximately 3 and 9 o'clock and then the scrotum near these points is sutured to the corpora on either side of the true penoscrotal junction with 5-0 polydioxanone, moving the tunica vaginalis flap aside to do so. In nearly all cases this maneuver corrects penosctotal Uansposition without need for scrotal flaps and the visible scars those produce (Pig. 147-16). Excess prepuial skin is excised to complete the circumcision and the ventral skin is closed, creating a median raphe. All skin stitches are subepithelial, We use urinary diversion into diapers for 2 weeks. ‘An example of the final commetic appearance is shown in Figure 7-17. Results, Urethroplasty complications have been reported in from 15% to aver 50% of cases. One report described technical ‘modifications in the urethroplasty that reduced complications. ‘Snodgrass and Bush (2011) reported outcomes in 59 consecutive patients, with urethroplasty complications occurring in 53% of the initial 15 cases, 259% of the next 20, and 13% of the last 24, Most of these were fistulas or glans dehiscences, and various technical changes were made specifically o reduce fistula occurrence, includ ing a change ftom single layer to two layer urethroplasty, epithelial to subepithelial suring, and 7-0 chromic eatgut to polyglactin and polydioxanone, All ofthe first 35 patients had a dattos Nap placed fover the neourethra, The final 24 had cunica vaginalis rather than ‘artos flaps, There were no fistulas in the final cohort Glans dehis- ‘ence is now our most common complication, and is discussed in ‘etail later in this chaptes ‘A report by Ghanem and Nijman (2010) concerned 49 patients with proximal TIP repair in which urethroplasty was done in one layer using continuous subepithelial 6-0 polyglactin covered by dartos. During mean follow-up of 3 years there were urethroplasty complications in 12% of patients that included four Bitulas, one ‘meatal stenosis, and one glans dehiscence, 3410 PART xv Pediatric Urology Figure 147-15. Tunlea vaginalis barier flap. A, Testicle delivered and tunica vaginalis opened transversely. B, Tunica vagin ‘ected to near the external ing to avoid tension on either the testicle for tho penis. C, Flap covers the entire neourethra. Testicle is pexed Inte its serotal compartment. A retrospective review by Braga and associates (2008) compared, 35 proximal TIP epaits o 40 onlay preputial fap repairs with mean follow-up of 3 years, There was no significant difference in urethto- plasty complications: 6096 for TIP and 45% for onlays. Two-Stage Gratt Indications. The main indication for a two-stage graft repair is VC greater than 30 degrees after degloving and excision of ventral ddartor and scrotal attachments to the penis. The urethral plate is transected as part of the straightening maneuvers, and either prepu> tial of oral labial graft is used to bridge from the native urethra 10 the glans tip. The choice of graft is devermined by the family’s preference for ether circumcision or prepucioplasty. When circum- cision is done the discarded prepuce is used for urethroplasty, ‘whereas oral mucosa from the lower lip is taken when prepucio- plasty is desired. Surgical Technique First Stage. The initial skin incision isthe same as described earlier for proximal TIP urethroplasty, maintaining most of the inner prepuce for a graft if needed. The operation proceeds as described for proximal TIP uretaroplasty to the point that artificial erection demonstrates VC greater than 30 degrees. Urethral plate transection is done distally at the coronal level, and then the plate is excised from the compota, moving proximally to the meatus and beyond toward the membranous portion, The native urethra is then gently stretched distally and anchored at intervals to the corpora using 6-0 polydioxanone This maneuver moves the urethrostomy distally, reducing the length of graft needed, The native urethral mucosa is sutured to the corpora using 70 polyglactin at 10, 12, and 2 o'clock, Proximal urethtostomy is completed by suturing the native urethra to penile skin or scrotum at 4, 6, and 8 o'clock with 7-0 polyglactin, Stay stiches ate placed into the corners of the dorsal prepuce ang the underlying dartos is excised. Typically the graft is mostly inner prepuce with less outer preputial skin, with the width deter- ‘mined by the lower edge of the subcoronal collar (Fig. 117-18). The dorsal shaft skin is sutured to the preputial collar using interrupted subepithelial 7-0 polyglactin, Then the graft is placed into the ventral defect and fist stitched tothe glans, which has been. ‘opened widely, at the level of the corona with 7-0 polyglactin, Additional stitches secure the graft to the distal end of the glans, placed subepithelially © avoid marks where the neomeatus will be ‘created during the second procedure. The graft is gently stretched proximally and sewn to the shaft skin on either side using inter rupted 7-0 polyglactin. The proximal end is split in the micline to extend graft to either side of the urethrostomy, which is sutured at the 2, 10, and 12 o'dock positions medially and to the shaft skin fr scrotum laterally. A preputial graft harvested as described in Figure 147-25 (see later) will il the defect from the glans tip to ddeep within the scrotum, [Next che graft is quilted onto the cospora at I-em intervals using 6-0 polyglactin on an RB-1 needle, which easily penetrates the graft and adheres itto the underlying tunica albuginea (Fig. 147-19). 8 ‘catheter is placed in the bladder Then a rolled Vaseline gauze (Conopco, Englewood Cliffs, NJ) is laid onto the graft and helé fizmly, but not tightly, by 5-0 polypropylene stay stitches tied over ‘the gauze. This ti-over bandage further immobilizes the graft and helps prevent seroma or hematoma accumulation beneath it. The catheter and tie-over bandage are maintained for 7 days, Physical activity isnot limited in infants and young children. No special care is needed for the graft after the bandage is removed during the interval before the second stage. We always wait 6 months before second-stage repair Second Stage. An incision is marked along the glans wings and shaft skin adjacent to the now revascularized graft, moving into the ‘urethrostomy ventrally to remove the penile or scrotal skin that was ‘sutured there from 4 to § o'clock The glans wings are injected with 1:100,000 epinephrine and incised and dissected laterally, as i the remainder of the marked incision, If the glans width is less than 14 mm, extended dissection is done as described earlier to reduce tension on the subsequent approximation of its wings. A 6-fr stent is passed into the bladder and secured to the glans traction stitch, Preputial grafts are very thin and can be tubu- [ized in two layers similarly to the urethral plate in proximal ‘TP repait using 7-0 polyglactin and polydioxanone. Then a tunica vaginalis flap is created and placed over the entire neourethra Glansplasty is completed as described earlier for proximal TIP ‘urethroplasty ‘A subepithelial 5.0 polydioxanone suture secures the scrotum, to the corpora on either side of the neourethra to establish the penoscrotal junction, and then penile and scrotal skin are closed in the midline using subepithelial sutures. Urine is diverted for 2 weeks, Chapter 147 Hypospadas 3411 Figure 147-18. Serotoplasty without scrotal laps. A, Penoscrotal transposition. B, Ventral inci sions at the ponoscrotal junction extending to approximately 3 and 9 o'clock to allow the scrotum to be pulled downward and secured to the corpora on either side of the urethra, C, Correction of transposition without rotational scrotal flaps. D, Sears ater rotational flaps. ‘These are not always later concealed by pubic hair. Figure 147-17. A and 8, Appearance after proximal tubularized incised plate repair. 3412 PART XV. Pediatic Urology Fgure 147-18. Preputial graft harvest. A, Initial degloving incision runs a few millimeters proxi- ‘mal to the corona, preserving as much inner prepuce as possible forthe graft. B, Lower line of incision for graft harvest. Figure 147-19. Preputial graft quilting and final result. A, Defect to be grated after the urethral plate has been excised, glans wings develo ‘and proximal urethrostomy created. B, Graft ‘secured to glans, shaft skin, and scrotum at the perimeter of the defect and then quilted to the ‘corpora first slong the midline and then to either side at approximately -cm intervals. C, Detail showing the graft extending to either side of the urethrestomy before quilting. D, ently compresses the graft to reduce dessin ulate eath it. E, Healed graft, ‘Results. There are few published results for two-stage primary araft repairs, with urethroplasty complications reported in from 25% to 50% of patients, ‘A retrospective review of 34 patients with proximal shaft to peri= neal hypospadias operated using two-stage preputial grafts reported "urethroplasty complications in 26%, comprising four glans dehis- sibility for seroma or hematoma te sccu- cences, two fistulas, one diverticulum, and one neourethral stricture (Ferro etal, 2002}, ‘We currently have unpublished follow-up results in 24 patients with proximal shaft (n = 3), penoscrotal (n = 8), scrotal (n= 7), and perineal (n = 8) hypospadias, Ventral lengthening using trans- verse corporotomies without corporal grafting was needed after ™ Figure 147-20. Onlay preputial flap. A, Lines of incision to create the ‘preputial lap and preserve the urethral plate. 8, Preputial lap mebi- lized on its vascular pedicle. C, Flap sewn to the urethral plate. urethral plate transection in 20 (83%). Three patients (12.5%) had graft contracture requiring regraiting as a separate procedure, two involving lip grafts and one after preputial grating. Urethroplasty complications occurred in 12 patients (52%) 11 glans dehiscences and 2 fistulas, Mean glans diameter for the entire group, measured in 22 patients, war 12 mm, with 15 (689%) less than 14 mm, Extended glans wings mobilization was not used in any of these patients because we had not yet started using this technique Preputial Flaps Indications. Onlay and tubularized preputial flaps are single-stage alternatives to TIP and two-stage graft repairs, respectively. Surgical Technique Onlay Plap. The technique for onlay preputial Hap is illustrated in Figure 147-20, The inital lines of incision, degloving and release of dartos and scrotal atachments, and development of glans wings are the same as described earlier for proximal TIP urethroplasty Artificial erection demonstrates 30 degrees or greater VC, sttaight ‘ened by dorsal plication when present ‘The comers ofthe dorsal prepuce are held with stay stitches and a 10-mm-wide strip of its inner surface is harvested, preserving the ‘underlying dartos vascular supply. Dissection ofthe pedicle extends to the penopubic junction to prevent tension when the lap is moved ventrally either around the side of the penis or via a but toohole incision over the glans. ‘A G-Er stent is pasted into the bladder. The flap is then sewn to the urethral plate using subepithelial 70 polyglactn, gently etch, ing it distally and trimming it as needed to maintain uniform dimensions. The dartos pedicle is used to cover the suture lines. Next, glansplasty Sst secures the glans wings to the flap edges using interrupted subepithelial 7-0 polyglactin. Proximally the wings are approximated together with interrupted subepithelial 6-0 polygla ‘in, Circumcision and skin closures are done as described for prox ‘mal TIP urethroplasty, Tubularized Flap. The technique for tubularized preputial flaps is iMlustrated in Figuce 147-21, When attificial erection finds VC greater Chapter 147, Hypospadas 341g A Figure 147-21. Tubularized preputial flap. A, After degloving and release of ventral dartos, persisting ventral curvature greater than 30 degrees led to excision of the urethral plate. An inner preputial flap approximately 10 mm wide is dissected on its dartos vascular and transposed ventral imal end anastomozed fend tothe glans wings. B, Alternatively, one edge ofthe flap can be ‘fixed with interrupted sutures to the corpora cavemosa from the proximal meatus distally inte the glans. Then the flapis trimmed and the opposite edge sutured along the first to create a tube with uniform caliber. Glansplasty and skin closure are similar to that described for onlay preputial flaps. than 30 degrees, the urethral plate is transected and additional Mraightening maneuvers as described earlier are performed as needed, “The corners of the dorsal prepuce ate held with stay stitches and a fap 12-10 15 mm wide is outlined horizontally on its inner postion, The flap is released and its pedicle dissected to the peno: pubic junction. The flap can then be tubularized over a 6-Fr stent {in cwo layers, the ist using a running subepithelial 7-0 polyglactin. followed by several more interrupted stitches, This tube is moved ‘ventrally, sewn to the spatulated native urethral meatus, and then stretched distally with the suture line down against the coxpora. The fap is sewn to the glans wings using interrupted subepithelial 7.0 polyglactin. Remaining glans wings ae approximated using subepi- thelial intersupted 6-0 polyglactin ‘Alternatively, the flap can be brought ventally before its tubut larization and gewn to the native urethra dorsally. Then itis stretched dlistally and one edge is sewn to the underlying corpora to create a pscudoplate, Excess flap skin is excised and the remaining free end fs sewn to the lateral edge to complete a tube Results, Urethroplasty complications after proximal preputial flap repairs have been reported in 27% to 45% of onlay flaps and in 14% to 33% of tubularized flaps. Two articles suggested that complications are fewer with tubularized flaps when they are first secured to the corpora along one edge to create a pseudoplate and then fashioned into a tube, Onlay fap repair for proximal hypospadias was reported in 126 patients with mean follow-up to 22 months. Usethroplasty compli cations developed in 27%, with 18 fistulas, 13 glans dehiscences, 2 strictures, 1 diverticulum, and 4 flap prolapses through the meatus {de Matios e Silva et al, 2009). Another retrospective review described outcomes for penoscotal onlay flaps in 75 cases with postoperative follow-up to a mean of 39 months. Complications were encountered in 45%: eight fistulas, two dehiscences, (wo strc tures, one meatal stenosis, and five patients with recurrent VC after dorsal plication (Braga etal, 2007) Tubularized preputial lap outcomes for penoscrotal or more proximal hypospadias were described for 27 cases with median 9 ‘months of follow-up. Of these, 33% developed urethroplasty com- plications, including seven fistulas, one stricture, and one meatal stenosis (Powell et al, 2000) 3414 PART XV. Pediattic Urology ‘Two reports described outcomes from tubed preputial laps in which tubularization was accomplished after first suturing the flap to the corpora. In one, 12 patients with penoscrotal or scrotal hypo- spadias had postoperative follow-up to a mean of 24 months, luring which time 2 (17%) had complications: one fistula and one meatal stenosis with diverticulum (Shukla etal, 2004). In the othet, 22 boys with proximal hypospadias had a similar repair and sub- sequent follow-up also to an average of 24 months. There were complications in thiee (1496) patients: one fistula and two meatal stenoses (Aoki ct al, 2008), Byars Flaps Byars lap refers to a two-stage operation in which the urethral plate is excised during penile straightening in the initial operation. The dorsal prepuce is split down its midline and the two parts ate ans- ferred ventrally with their dartos vascular pedicles and sutured into the defect from the meatus to the glans. At the second operation the previously ansfered prepuce is tubularized (Syars, 955) “This technique has been used following comporotomy with sgraling by those who prefer a two-stage urethroplasty rather than 4 single-sage tubularized preputil flap. However, there are few fepored outcomes. The largest, with 58 patients, only mentioned postoperative fistulas among all the possible urethroplasty compli- {ations (Ret etal, 1994). Another three studies had fewer patents, Shukla and colleagues (2004) reported results in only 10 patients with an average 43 months of follow-up, noting urethroplasty com plications in 70%, including seven Sstulas, Uaree meatal stenoses, and one diverticulum, Getshbaum and colleagues (2002) had IL patients with follow-up of "5 to 15° yeats, with complications in 18% (one fistula and one diverticulum), although the authors stated that 2 more patients had a “subterminal meatus or skin irregularities” that potentially increased the rate to 36%. In addi tion, they stated that 37% had abnormal voiding and spraying, 1 (WS. used the operation in 9 patients with a 100% complica- tion rae, with two fistulas, five diverticula, one stricture, and two glans dchiseences. Although fistulas and glans dehiscence are common in proximal hypospadias repair, it was diverticula, and the stieture that resulted when a less wide skin strip was tubularized to try to prevent a diverticulum, that prompted me to abandon this technique, We no longer perform Byars flaps or recommend their use Flaps versus Grafts ‘There are no tials randomizing patients with proximal hypospadias and VC greater than 30 degrees to subularize flap versus two-stage sgraft repair, Proponents of flaps state that their vascularity is assured trom the pedicle, whereas that of grafts is less reliable because they rust revascularize. However, Duckett once commented that fio- rescein showed devascularized edges to his flaps that had 10 be ‘xeised, although he never published these observations in a clinical series (Duckett unpublished comment to Hodgson, 1981). Graft take was successful in all 43 cases eeported by Feo and colleagues (2002) using. prepuce. We encountered. contracture ‘esulling in an additional procedae to pastally ot tually reratin 4 of 65 (6%) patients, without a difference between prepuce and oral mucosa (Siodgrass and Bush, 2015). Our series fers ftom that of Feo and colleagues in that we straightened VC in 26 (909%) of these cases using transverse comporotomies, which was done in 5 ofthe 4 patents with contracture. No patient has required more than one regrating Urethroplasty complications that potentially indicate impaited vascularity inciide meatal stenosis and strictures. Tubularized Sap outcomes described elie eported meatal stenosis and/or suictte in approximately 8% (Powell et l, 2000; Shula eal, 2004; Aokt ‘al 2008), whereas 3% of two stage grafts developed a stricture (Gero et a) 2002), None of our patients hat had either meatal stenosis oF stitute ‘There are also few data regarding cosmetic results, Patents we Ihave evaluated who were operated elsewhere with flaps, an admit tedly potentially biased group, most often have had glans dehis- cence anda less cjlindrical shape to the penis (Fig, 147-22). This slans dehiscence may be protective against diverticulum, but at the potential cost of urinary spraying Currently there are insufficient functional or cosmetic data to cstablish the best practice and determine if benefits of a two-stage repair outweigh the need for two operations Prepucioplasty Prepucioplasty can be done in neazly all patients, with both distal ang proximal hypospadias, whose caregivers request it (Fig. 17 25); In 1% of eases a patient has a large glans and small dotsal hood ‘that prevent prepucioplasty. When prepucioplastyis done for proxi- ‘mal hypospadias in which urethral plate transection is needed for VC straightening, two-stage repair uses an oral labial mucosa graft Indications. Foreskin seconstruction is indicated in any primary hypospadias repair when caregivers prefer it to circumcision, We simply ask if newbom circumcision was anticipated and, if not, oller prepucioplasty. Surgical Technique. Stay sutures ae placed into the comers of the dorsal prepuce (see Fig. 147-23). Initial incision extends from these points, lateral to the glans, and then to a point approximately + oi Figure 147-22. Appearance after flap and graft repair for proximal hypospadias. A, Tubularized {lap repair with a pyramidal shape of the penis. ‘ll meatus, is noted instead to have glans dohi penile shape and slit meatus with well-healed glar ‘The same patient, who appears to have @ jscence with @ coronal meatus. C, Cylindrical nsplasty after two-stage preputial graft repair. Chapter 147 Hypospadas 3415 Figure 147-28. Prepuctoplasty. A, V-shaped incision trom extending ventrally to below the meatus. B, plate urethroplasty with prepucioplasty. Note the excellent ventral exposure gained without ‘dogloving the penis. E and F, Two-stage proximal repair using oral mucosa graft 3416 PART XV. Pediattic Urology 2 mm below the meatus, The penis is not degloved, and ventrally dissection is done immediately under the skin to preserve dartos for a barsier flap until normal tissues are encountered, generally neat the penosctotal junction Urethroplasty and glansplasty are done as already described for distal or proximal TIP or two-stage graft repairs ‘Afr glansplasty the foreskin stays are pulled down below the glans and the inner prepuce is approximated using subepithelial 7-0 polyglactin. Then the stays are pulled distal to the glans. These comers ate approximated together also using subepithelial 7-0 polyglactin. Previously we adjusted the position of this initial stitch to allow the foreskin to readily retract back and forth over the glans, However, this sometimes leaves the prepuce visibly deficient ven tral, and s0 today we suture it to achieve the best appearance and are not concerned about it retracabilty, given that normal boys the same age as those undergoing hypospadias repair often have similarly nonretractable foreskin, The remainder of the incision is closed using interrupted 7-0 polyglactin. Caregivers are instructed not to retract the foreskin Results. Urethroplasty and skin complications are the same after distal or proximal TIP or two-stage graft repairs, whether circumcision ot prepucioplasty is done. ‘Suoub and coworkers (2008) compared 25 distal TIP repairs with prepucioplasty to an age- and time-matched cohort of 49 distal ‘TIP repairs with circumcision, reporting no difference in either ‘urethroplasty or skin complications. The only urethroplasty com- plications were fistulas, occurting in 12% and 89%, respectively: One patient with “recalcitrant” phimosis had secondary circumcision after prepucioplasty versus two with “redundant skin’ after circum sion who had eizcumesion revision (Suow etal, 2008) Snodgrass and colleagues (2013) also reported a case-cohort study of 428 consecutive distal TIP urethroplasties of which 85 had prepucioplasy. There were no intraoperative conversions to eixcum- Gision. Urethroplasty complications developed in 8% after prep coplasty and 9% afer circumeision, Two percent of each group had subsequent skin revision, which included one circumeision for BKO 5 years later and one excision of an unsightly dorsal whorl without circumcision following prepucioplasty. Snodgrass and Bush (2011) did prepuctoplasty during proximal, TIP urethroplasty m 21% of cases (all who requested it), with none having postoperative urethroplasty or skin complications. Prepucio plasty was also done in 25% of those undergoing two-stage graft repait (all who requested it), with none having recurrent curvatre cor urethroplasty or skin complications (unpublished data). Recatise prepucioplasty does not increase either urethroplasty or skin complications, the choice between it and cireumeision should bbe mentioned to all caregivers, allowing them to determine the final cosmetic appearance. Scrotoplasty In the last edition of this textbook “major” scrotoplasty using rota- tional skin flaps to correct penoscrotal transposition was illustrated, Today we no longer perform this maneuver, having found that we ‘an correct transposition with ventral penoscrotal incisions leaving no visible scar. Instead, shaft skin adjoining the scrotum is incised venitally to 3 and 9 o'clock, and then the scrotum is rotated down to create a new penoscrotal junction and sutured to the corpora on. cither side of the neourethra with 3-0 polydioxanone as shown in Figure 147-16, POSTOPERATIVE MANAGEMENT Urinary Diversion Several studies reported that distal TIP urethroplasty in pre- toilet rained boys can be done without diversion, expecting less than 5% to need catheterization early postoperatively and no increase in urethroplasty complications. ‘One trial of toilet trained boys found greater dysuria, reten- tion, and extravasation in those not catheterized, resulting in catheter placement in 40% of those not randomized to diversion, Urethroplasty complications were not impacted by whether o not diversion was used, There are no data indicating benefit of suprapubic diversion, im addition to or ax a substitute for urethral catheters. Three studies reported results for midshatt to distal TIP urethro: plasty without urinary diversion in non-toilet-trained patients: + Almodhen and associates (2008) reported on 32 consecutive non-toilet-trained boys (mean age 18 months) who had TP ‘urethroplasty for distal to midshaft and proximal shaft (n = 6) hypospadias without a catheter. One (distal vs. proximal not stated) developed urinary extravasation on the second postop: ‘erative day that was treated with catheterization, One patient (39%) had a urethroplasty complication (meatal stenosis) during, followup of 9 + 6 months ‘+ Samuel and colleagues (2002) reported on 170 consecutive patients (mean age 19 months) who had distal TIP repair without diversion. None had urinary retention or needed eath- ‘terization. Urethroplasty complications occurred in 7% during follow-up to a mean of 3 years. + Leclair and associates (2004) reported on 162 consecutive patients (mean age 16 months) with distal or midshatt (n = 6) ‘THP repair without diversion. Catheterization was needed for ‘urinary retention in 4 patients (2.59%), 2 within hours of surgery snd 2 at 1 week postoperatively, without subsequent complica- tions, Urethroplasty complications occurred in 8%, both fistulas and meatal stenoses, ‘An RCI by H-Sherbiny (2003) compared outcomes in 64 toilet- trained boys (median age 6 years) to distal TIP urethroplasty with versus without cathetetization, decided at the end of the operation, Urethroplasty complications were similar in both groups (3 of 33, stented vs. 6 of 29 not catheterized, P= 3). Howeves, dysuria (14% ‘vs 4596), retention (0 vs. 2496), and extravasation (0 vs. 179%) ‘ccurted significantly mote often in those not diverted. Of the 29 patients not catheterized, 12 (41%) were catheterized within 3 days bf operation ‘We have used urinary diversion to avoid need for postoperative catheterization in the minority of pre-toilet-trained patients who otherwise will develop retention or extravasation. A 6-Pr bladder sent is used forall repairs in prepubertal boys, versus a 12-to 14-Fr catheter after puberty. For patients who are operated before toilet training, the catheter drains into a single diaper. We never use suprapubic tubes in either primary or reoperative hypospadias tepait. Given that most infants undergoing distal repairs in these studies did not require diversion, we recently began performing distal TIP ‘urethroplasty without a stent, which obviates need for postopera: tive antibiotics and facilitates eatly, normal bathing resumed a 48. hours after surgery, Two tials reported no differences in urethroplasty outcomes whether or not bandages were used, ‘To our knowledge only two studies consider the possible impact, of postoperative bandages on urethroplasty outcomes: + Van Savage and colleagues (2000) randomized 100 patients to a Uanaparent waterproo! adhesive bandage around the penis removed by parents 2 days after surgery versus no bandage. Two were excluded for bleeding at the end of the operation. There ‘were no differences in urethroplasty complications at mean follow-up of 1 year, but telephone calls were significantly more frequent from parenis of those without a bandage than from parents of those with a bandage (0.8 vs. 0.3 ealls/patient), The authors did not state if these reported calls refered to wound ‘questions versus other concerns. + MeLorie and associates (2001) allocated 120 patients at the end ff repair to a transparent biomembrane adhesive film versus compressive wrap versus no bandage with polymyxin B and bacitracin zine in white petrolatum applied at cach diaper change for 7 days, Three patients were withdrawn for bleeding Figure 147-24. Postoperative bandagt srequising a compressive dressing, Bandages were removed at 3 for moze days and white petrolatum was then applied for another 7 days, There were no differences among groups with regard to Urethroplasty complications bandages do not impact urethroplasty outcomes, then various wraps that may be painful to remove can be avoided, We use a ‘Tegaderm (3M, St. Paul, MN) adhesive around the penis and a second holding a gauze over the wound, both of which fall off spontaneously at home (Fig. 147-24), Medications Antibiotics One tril reported that postoperative oral cephalexin reduced the incidence of febrile UTI “The only ial regarding postoperative antibiotics after hypospa dias repair included 101 patients undergoing TIP urethroplasty who all recetved intraoperative intravenous cefonicid and then were ran domized to postoperative oral cephalexin for & days during urinary tiversion versus no antibiotic Usethroplasty outcomes were the same but febrile LTT occurred in 3 of 52 antibiotic-treated versus 12 of 49 untreated patients (P < 05) (Mir and Livne, 2004), We do not use intraoperative antibiotics during hypospadias repair except for those patienls having oral mucosa harvest, to ‘whom intravenous cefazolin is given, Postoperatively trimethoprim sulfamethoxazole is given during urinary diversion. Analgesics and Antispasmodics ‘We recommend oral ibuprofen 4 times daily alternating with acct aminophen for infants to children approximately 2 years of age Older children are provided hydrocodone with acetaminophen to use between ibuprofen doses as needed (Oxybutynin 0.2 g/kg per dose up to 5 mgs given twice daily, or asa single extended release tablet, (o patients 3 yea of age or olde OUTCOMES ASSESSMENT Hypospadias repaic is much mote than simply urethroplasty, and futtcome assesement inchudes genital appearance as well as penile functions of urination, erection, and ejaculation, In children most emphasis has been on urethroplasty complications, with less on commetic results, Available data regarding sexual functions in adults Chapter 147 Hypospadas 3417 are reviewed in the later section on Adult Outcomes after Prepuber: tal Repait, Duration of Follow-Up Eighty percent of urethroplasty complications are diagnosed within 1 year after surgery, with indefinite follow-up needed in 14 patients for each complication subsequently encountered Snodgrass and colleagues (20142) reported the time at which any urethroplasty complication was diagnosed after 887 primary and reoperative TIP tepaits. There were a total of 125 ‘complications 34 fistulas, 59 glans dehiscences, 9 meatal stenoses for neourethral strictures, and 3 diverticula—of which 64¥% were diagnosed at the first postoperative visit and 80% within the frst postoperative year. Median time to encounter fistulas, meatal ste hoses and striciures, and diverticula was 6 months, whereas glans dehiscence was diagnosed at a median of 2 months. Aer | year ve calculated that 14 patients would require indefinite follow up for cach additional complication eventually diagnosed (Snodgrass etal, 2014a) ‘Continuous longitudinal follow-up to puberty has never been reported for patients undergoing prepubertal hypospadias repair Several retrospective reviews evaluating the time to diagnosis of urethroplasty complications reported late complications (after 1 year) in those patients who returned because of their complication, Wood and associates (2008) studied fistulas and found that 7096 were diagnosed by 1 yea but the tally did not reach 90% and 99% until follow-up at 8 and 20 years. Spinoit and colleagues (2013) reported 24% of reoperation’ for urethroplasty complications or ‘unsatisfactory appearance were done at more than 2 Yeats postop: tratively, but that after 3 years 15 boys would need assessment for each additional complication found. Clearly more complications ate potentially found as duration of follow-up increases. However, many boys who will never have a complication have to be reviewed indefinitely for each additional ‘one diagnosed after 1 year We recommend office assessment at 5 weeks and then 6 months later (8 months postoperatively) after Gistal TIP urethroplasty and advise caregivers at the last visit that a complication may become apparent ata future date. After proximal repaire we request annual follow-up with the academic goal of determining functional outcomes pre- and postpuberty, given the greater degree of VC and the longer urethroplasty with proximal hypospadias. Calibration ‘The minimum caliber of the normal urethra in boys varies in published reports. One study found that 14% of boys less than, 5 years of age were less than & French, ‘Calibration of the neourethra isan objective means co establish that there is no anatomic obstruction after urethtoplatly. We 10u- tinely calibrate pre-toilet- trained patients with a 10-Fr sound atthe ‘S-month postoperative visit, but the very low prevalence of obstruc tion supports limiting calibration to infants with questionable obstructive voiding and/or a small-appeaing meatus. Normal meatal size was determined by Allen and colleagues (1972) in 100 consecutive full-term newborns using bougies 4 boule of olive-ipped catheters on day 2 of life. The mean and median wae § French, with half the patients less than 8 French to as small a8 4 French in 10%, Another study also used bougies & boule in 200 referred patients, eporting that 14% were less than 8 French to age 3 years (Litvak et al, 1976). Uroflowmetry [No study provides flow rates in patients compared to age-matched contols, and outcomes based on nomograms may vary depend: ing on the nomogram ured, ‘Approximately 25% of patients after TIP repair or onlay or tubularized preputial lap repair have Qmax less than 2 standard deviations below normal based on varying nomograms, yet have 3418 PART XV. Pediatic Urology 1no symptoms. The significance of this finding in asymptomatic patients is unknown, Following Tubularized incised Plate Urethroplasty ‘Andersson and colleagues (2011) reported flow rates in 37 asymp. tomatic boys from a total of 126 distal and proximal TIP repairs. AU year the mean Qmax was 13.6 ml/see (range § to 28), with half below the Sth percentile on the Miskole nomogram, AL an average of 5 years later the mean Qmax was 19 ml/sec and 3296 were below the 5th percentile, a significant improvement. The authors stated that {ewer patients would have been categorized as below the sth percen- tile had the Togusi nomogram been tised (Andersson et al, 2011), Snodgrass (1999) reported wroflowmeury in 17 of the Brst 50 toilet rained boys following TIP urethroplasty, determined at a ‘mean of 45 months (range 6 months to 7 yeats) postoperatively. Al pea loves were above the 3th percentile based on the nomo- gram used by Jayanthi and colleagues (1995) (which was not reported in the article) ‘Considering Qmax less than 2 standard deviations from normal 4 possible indication of obstruction, a review by Gonzalez and Tudiwikowsk: (2011) of reported TIP uroflows found that 36 of 140 (26%) asymplomatic patients in three articles using different nomograms met that criterion. ‘We hypothesize that the Qmax changes little after initial healing bbut that at puberty the increased urethral diameter should increase the flow rate. Currently we have limited data in patients before and after pubertal development. In two the Qmax improved from 7 to ‘msec and 13 10 20 msec at Tanner stage 4, Three others have no change in Qmax at Tanner stage 2 Following Preputial Flaps Jayanti and colleagues (1995), in a review of uroflows in SI coilet trained boys following either onlay or tubularized preputial flaps, reported that 27% had Qmax below the Sth percentile of an inst tutional nomogram, Patel and coworkers (2004) obtained uroflowmetry a mean of 14 years ater proximal repaie in infancy (mean age 17 months) and. reported a mean Qmax of 17 mlysce without differences between clay and tubularized flaps Cosmetic Results ‘Two studies used standardized photographs to compare TIP versus flap cosmetic outcomes, both reporting higher scores for TIP repairs. A questionnaire study compared IP patients to con- twols after circumcision and found similar ratings by caregivers 6 weeks after surgery ‘Objective assessment of genital appearance afier hypospadias surgery is not commonly reported. Two studies used photography scored by blinded reviewers t compare TIP to Mathiet (appendix) ‘or onlay flaps. Both reported TIP scores significantly higher (Verver= di etal, 2005; Scarpa et al, 2009). Snodgrass and colleagues (2008) used a nonvalidated question. naire answered by caregivers before physician examination 6 weeks after distal or proximal TIP urethroplasty versus controls following CGrcumecision, There were no differences in Likert seale scores regatd~ {ing overall appearance or the specific appearance of the meats of penile skin, Hayashi and associates (2007) compared photographs after stan- dard onlay to photographs after a modified V shaped incision ven wally to create a more vertical meatus shape. Overall improvement ‘was reported, with 8 of 25 standard versus 12 of 18 modified repairs achieving a slit meatus (P = 03). A V-shaped incision was effective in all 4 patients with a deeply grooved and in 6 of 9 with a mod= rately grooved plate, but in only 2 of $ with a flat configuration There are no other studies concerning aesthetic appearance of| the penis after flap repairs. Although the V-shaped incision pro posed by !layashi and associates (2007) did result in more patients ‘with a slit meatus, the patients most likely to have a rounded appearance with flaps ate those with a flat plate, and V-shaped inci son was effective in fewer than 50% of those. COMPLICATIONS Risk Factors Risk factors for urethroplasty complications include proximal meatus, reoperation, and glans width less than 14 mm. Studies in which patients did not routinely have barrier flaps over the rneourethra have found that thi is also a risk factor for complica tions (fistulas). Bush ane colleagues (2012) used multivariate analysis to eval ate potential risk factors for hypospadias urethroplasty complica- tions among 669 consecutive prepubertal TIP repairs, using prospectively recorded data. These inchided patient age, meatal location, reoperation, glaneplasty suture type (chromic vs, polygla- clin), and surgeon learning curve (detined as the fist 50 cases). OF these, the only independent factors were reoperation (odds ratio [OR] 3.07, 95% confidence interval [CT] 1.54 t0 6.13) and proximal meatus (OR 1.79, 959 Cl 1.33 to 2.40) Bush and colleagues (2013) subsequently analyzed 391 patients with glans measurements for patient age, meatal location, reopera- ton, and glans width (in millimeters). Meatal location and reopera- tion remained independent factors, but so was glans width less than 14 mm (OR 3.7, 95% CI 1.6 to 85), with each I-mm increase in slans size decreasing complications Two other reports used multivasiate analysis of retrospectively collected data after TIP urethroplasty, Eassa and associates (2011) evaluated 391 patients operated by five surgeons, analyzing for age, ‘meatal location, reoperation, surgeon, urethroplasty sures (polygla- clin vs polydioxanone) and methods (interrupted vs. continuous), a flap over the neourethra, and urinary diversion. Only proximal mat location (relative risk [RR] 2.81, 95% CI 1.42 (0 3.52), age greater than 4 years (RR 3.25, 959% Cl 1.44 to 7.35), and no barter lap (RR 6.23, 95% CI 1.87 to 20.77) were risk factors Sarban and coworkers (2009) evaluated 500 patients operated by five surgeons, analyzing for age, meatal location, reoperation, urethroplasty suturing method (nterrupted vs. continuous), neourethral coverage urinary diversion, and learning carve (defined as the fret 100 cares), Independent rile faccors were proximal meatus, no barter layer, and learning curve ‘We cannot model for barrier layers or urinary diversion because both are used systematically, As discussed earlier, we did not find age al repair to be an independent risk factor for urethroplasty complications Modifying Risk Factors Meatal Location Oly 10% of primary cases present with a meatus on the proximal shaft to the perineum, Case logs reported to the American Board of Urology by US. pediatric urologists requesting a centficate of added qualification indicated the average number of proximal repairs done annually was two (Kogan anc Feustel, 2011). Given that proxi ‘mal meatus location is a consistent risk factor for urethroplasty complications, we recommend that centers designate a single surgeon to perform these cases ta increase his or het expertise Reoperation Initial failure increases risk for additional failure, We recommend that surgeons review their personal outcomes and consider changes in procedure and/or technique to reduce complications, as we dis cussed earlier in the chapter. The Results section under Proximal Hypospadias Repair details technical modifications we have made that significantly reduced urethroplasty complications after proxi mal TIP urethroplasey ‘Academic surgeons must ensure good outcomes for the patient ‘when allowing trainees to actively participate in key steps of the surgery, especially urethroplasty and glansplasy. A survey by Delair and coworkers (2008) of mostly senior trology residents having completed more than 75% of theie waining found that few had performed glans wings dissection or urethro: plasty. Fellows in our program also observe these key steps until faculty conclude that their skills are satisfactory, and they rarely perform more than 50% of any given repair. Bush compared distal ‘TIP outcomes of our former fellows in consecutive cazes done over 4 2-year period beginning 3 or fewer years after taining to those of Snodgrass dusing the same time frame. There wete no significant differences in urethroplasty complications among the former fellows or between them and Snodgrass (Bush et al, unpublished) ‘We also recommend that reoperations in major centers be done by a single surgeon Glans Size ‘As discussed in the ealier section on Preoperative Androgen Stimnula \Uon, preoperative androgens are known to increase glans width. We analyzed urethroplasty complications in patients who received adj vant testosterone injections versus those with glans 14 mm oF greater ‘who did not Mean glans width before sumulation was 12 mm, increas ing to a mean of 16.5 mm with testosterone injections. Untreated palients had a mean glans width of 15.4 mm, Usethroplasty complica ‘dons occurred in 34% with versus 119% without adjuvant androgens (P <.0001), Testosterone was found to be an independent tsk factor for complications inthis analysis (OR 3.1, 9596 C1 12t0 8.1). Accor ingly, we have stopped preoperative testosterone stimulation (Bus!) ‘etal, 2013), Now weuse the extended glans wings disection described eatler for patients with glans width less than 14 mm, Although we ddo not yet have outcomes data, this technique has a reported glans Sehiscence rate of | in 150 cases despite an average glans width of 12 mm (Tanakazi and Yoshino, personal communication) Fistulas Prevention. Subepithelial suturing and dartos flap coverage over the neourethra are thought to reduce fistulas. Two-layersubepi- ‘heli urethroplasty with tunica vaginalis lap coverage was reported to significantly reduce fistulas when compared to single- layer epithelial closure and dartos flap coverage in proximal 1 urethroplasty. Chapter 147 Hypospadas 3419 Subepithelial urethroplasty was superior in a retrospective analysis of Mathieu operations by Ulman and associates (1997). ‘They compared single-layer continuous urethroplasty done in 36 initial patients with 6-0 polyglactin sutured through the epithe- lium to that done in 61 later boys using 7-0 polydioxanone subepithelial stitching. With follow-up of 6 to 12 months, fistu- las occurred in 6 of the 36 patients with epithelial stitching (17%) versus 3 of the 60 patients with subepithelial stitching (6%) (P< 0). {Use of a barter flap or not was the subject of a tial by Savanelli and coworkers (2007) that randomized 130 patients undergoing distal TP repair Three surgeons performed the operations using the same urethroplasty technique and suture. During median follow-up (of 24 months, Sst occurred more often in those without a dartos fap: 15 of 65 (23%) versus § of 65 (8%) (P=.03), Meatal stenosis and glans dehiscence were similar in both groups (4% and 5%, respectively) Bakan and Yildiz (2007) compared a single layer dartos lap to 1 two-layer flap. The single-layer dorsal dartos flap was used in 29 consecutive patients, followed by a two-layer flap in the next 45, ‘Mote boys in the second group had midshal to proximal hypospa- dias or reoperations, but fistulas only occurred in the single layer patients (4 [1496] vs. 0, P= 02) Asmentioned, 1 (WS. made technical modifications to proximal ‘TIP urethroplasty that reduced fistulas from an initial 5 of 15 (339%) t0 2 of 20 (10%) to 0 of 24 consecutive patients (see Revuls section under Proximal Typospadias Repair). The first 15 patients had single-layer epithelial suturing of the neourethra using 7-0 chromic catgut The next 20 had two-layer subepithelial urethroplasty using interrupted 7-0 polyglactin and continuous 7-0 polydioxanone Spongioplasty after urethral tubulanzation was added to the second group. Inthe last 24 cases a tunica vaginalis flap, rather than dartos, covered the repair (Snodgrass and Bush, 2011). Surgical Repair. Repair includes assessment for distal obstruc- tion, excision of the fistula tract with closure of the urethral ‘opening, and flap coverage over the defect, ‘The neourethra i calibrated distally o determine itis 8 French ‘or greater, Then fluid is injected into the neourethra to confirm the fistula site(s). We make a median raphe incision that encircles the fistula and continues proximally (Fig. 47-25). The tract ie excised and the urethral hole is closed in one layer using subepithelial age with a ventral dartos flap without need for reoperative glansplasty. C, Coronal fistula with ‘band of skin holding the glans wings together. This requi eration rather than simple fistula closure, tubularized incised plate reop- 3420 PART XV. Pediatic Urology interrupted 7-0 polyglactin. Repeat fluid injecion confirms water tight closure. A ventral dartos flap is raised and used to cover the repair. We do not use diversion. Repair of coronal fistulas depends on the extent of glans fasion. When the glans is well formed the fistula can be closed by clevating the glans without reoperative hypospadias repait When the glans wings are separated and held by only a band of skin (see ig. 147-25C), reoperation is done as described in the later section fon Glans Dehiscence, Results. Three series reported failure in from 6% to 29% of cases, with no differences whether of not urinary diversion was used. A retrospective review by Shankar and colleagues (2002) had 113 cases of fistulas, of which 7% also had distal obstruction. Subepi- thelial closure and dap coverage were done, with urinary diversion for 1 week. total of 29% of patients developed recurrent fistulas, ‘more likely in those with inital fistulas greater than 2 mm versus those smaller, Wave:man and colleagues (2002) used diversion in 54 of 100 fistula closures with “larger” defects but found no differ- ence in recurrences, wich also developed in 29% of patients, based fon stenting. A third review by Santangelo and asvociates (2003) considered’ 69 “simple” and 25 “complex” fistulas (larger, andor ‘with distal obstruction or a diverticulum) that were corrected by closure an flap coverage generally without stent or by reoperation/ ‘meatotomy plus fistula repair in which stents were used, tespec- tively. Both groups had similar recurrences, which overall developed in 6%, Our recurrence rate for fistula closure as described earlier, exelud~ ing those patients with hypospadias reoperation, is 8% (Snodgrass, ‘unpublished data). Glans Dehiscence Glans dehiscence occurs more often following reoperative surgeries, and in patients with glans width less than Ad mm, We define glans dehiscence as complete separation of the glans ‘wings, with of without a band of skin bridging the gap berween the wings (Fig. 187-26). In addition to abnormal appearance, glans dehiscence creates a functional impairment with a deviated and/or spraying stream, Partial dehiscence results in a larger meatus, but ‘with glans wings fusion between the meatus and corona, We do not repair these unless there is a spraying stream, which is the same decision-making citerion we use in patients presenting with glanu- lar hypospadias to determine who will have repait. Snodgrass and colleagues (2011) used multivariate analysis in, 641 consecutive patients following distal, proximal, and reoperative “TIP urethroplasty (most of which was for prior dehiscence), and found glans dehiscence in 5%. Risk was neatly four times greater in proximal repairs and almost five times greater in reoperations This is our most frequent hypospadias complication, yet itis not often reported in other series. Our glanaplasty technique may be inferior andjor this complication is underreported. Based on patients referred to us after failed surgery elsewhere, se believe the complication is more common than realized. However, after observ- ing glansplasty in glans less than 14 mm in japan, we also realized a potentially better glansplasty involving extended glans wings dis- section could be done co teduce this occurrence, as described in this chapter Prevention ‘We first changed sutures fiom chromic to polyglactin after recogni- ing glans dehiscence, but the subsequent analysis mentioned earlier showed no difference based on these sutures. Next we used preop- ‘erative testosterone to increase glans size to 15 mm or greates, Dut similarly found no decrease in this complication in treated patients, Currently we dissect the glans wings from the corpora completely to 3 and 9 o'lock in all patients, which is a more systematic dis- section than previously. Then in patients with glans width less than 14 mm, prior dehiscence, and/or subjective tight approximation afer standard dissection, we further release the wings superiorly for about 4 mm as shown in Figure 147-11 Surgical Repair Reoperative TIP or inlay grafting is used to repair glans dehiscence, as described in the later section on Hlypospatias Reoperations, Results Villanueva and colleagues (2012) reported outcomes for reopera- tions to correct glans dehiscence before adopting the extended glans wings dissection currently used. Instead, the glansplasty was ‘basically repeated using dissection of the glans wings to approxi- mately 3 and 9 o'clock and then approximation with three inter- rupted subepithelial 6-0 polyglactin sutures, Recurrent dehiscence developed in 18 of 111 patients (16%). Of these 18, 10 had a third similar glansplasty, but 5 of 8 (63%) with follow-up dehisced again. Today, if reoperative glansplasty with extended glans wings Figure 147-28. Glans dehiscence. A, Complete separation ofthe glans wings with ‘meatus. B, The glans wings are separated, but a skin bri sbeoronal ‘between them gives the avision ‘of a glanular meatus. This patient had a spraying urinary stream corrected by reoperative slansplasty. dissection fails, we advise no further surgery until the patient reaches late puberty Meatal Stenosis ‘There is no agreed-upon definition for meatal stenosis, We diag, nose stenosis when the neomeatus is less than & French in a boy ‘with voiding symptoms. Our results suggest that most meatal stenosis is iatrogenic. There is no report of outcomes for mea totomy after hypospadias repair. ‘We have evaluated for second opinions patients with a small appearing meatus who are asymptomatic following hypospadias repair and have been recommended for meatotomy, yet a 10-Fr sound passes easily. These boys do not need intervention. There is rho accepted definition of meatal stenosis, which we define as meatal size less than 8 French after repair in a symptomatic patient (ie, fone with dysuria, sranguria, retention, andor febrile UTI) A stan dardized literature review by Wilkinson and colleagues (2012) included 15 ease series describing distal TIP outcomes in. 1872 patients, The authors noted that the diagnosis of meatal stenosis ‘was not standardized, and so likely varied among these publica tions, but was Feported in 3% of patients, Sodgrase ancl coworkers (2010) reported outcomes in 426 con. secutive patients with distal hypospadias, all of whom had TIP urethroplasty. OF these, 263 (62%) had calibration with none having meatal size ess than § French, One patient developed sec ondary meatal stenosis from BXO 6 years following surgery. Prevention Given our outcomes, meatal stenosis after IP urethroplasty appears to be avoidable. We have emphasized technical factors to reduce risk, including incision limited to the urethral plate, not extending into the glans distally, and continuing to near the underlying corpora; tubularization of the plate that begins at least 3 mm {rom its distal enc, creating an oval opening, and independent glans Wings approximation without suturing to the neourethra, Surgical Repair We incise the neomeatus dorsally to enlarge it without recreating the bypospadias defect, Meatal stenosis that nearly obliterates the opening, or is the result of BXO, requires reoperative hypo: spadias repair as described in the later section on Ilypospacias Reoperations. Results We found no articles defining meatal stenosis or reporting out comes from meatotomy after hypospadias repair, Neourethral Stricture Stricures of the neourethra are unusual follovting hypospadias repair using any of the techniques described in this chapter. A review of the outcomes tables that accompanied the hypospadias chapter in the prior edition of this textbook shows few mentions of stricwse, the highest prevalence being 996 in both a report of tub: larized preputial flaps (Ghali, 1999) and a report of Koyanagi flaps (Koyanagi et al, 1994). ‘We encountered no strictures in our series of 426 distal TIP repairs (Snodgrass etal, 2010), However, stictures did occur in 5 of 2 (179%) proximal TIP repairs in which the urethral plate and native urethra were dissected from the corpora to preserve the plate ‘while suaightening VC. All these patients presented 6 weeks to 1.5 years postoperatively with symptoms of retention and/or febrile UTL Another 47 proximal TIP repaits without this maneuver had no strictures, Accordingly, we have discontinued this maneuver and ‘not described it in this chapter, However, a retrospective review by Bhat (2007) of 32 patients ‘with urethral plate and native urethra elevation from the corpora Chapter 147 Hypospadas 3421 followed by tubularization done with (n = 20) or without TIP inci: sion reported no strictures during an average 24 months of follow-up. We cannot explain the difference in these observations Treatment Options include direct vision internal urethzotomy (DVIU), which is effective for strictures less than 1 cm following urethtal plate tubulatizations and onlay preputial laps, but not tbularized flaps fr grafts, Repeat DVIUs for recurrent strictures less than 1 cm alll failed Mobilization of the urethra with stricture excision has not been reported for strictures after bypospadias repai, to our knowledge Inlay or two-stage oral mucosa grafting, described in the later section on Hypospadias Reoperations, are both options depending fon the etiology of the stricture (focal ischemia versus BXO, respec tively), the extent to which the neourethra is obliterated, and the presence of secondary VC when there is neourethral contracture Results A single DVIUI was successful in approximately 66% of patients with strictures less than 1 em after urethral plate tubularizations for onlay flaps. Repeat DVIUs all failed. Dorsal inlay grafting wae tused in one series with success in 94% at 2-year follow-up. DVIU for strictures less than 1 cm achieved relief of voiding symptoms and Qmax greater than 12 mLsec in 0 of 32 tubularized grafls and 2 of 18 (11%) tubularized flaps, versus 8 of 11 (728) ‘nky flaps and 7 of 11 (6396) urethral plate tubularizations (each P <.05). Patients were randomized to post DVIU dilations or not with no difference im outcomes. Repeat DVIU for 12 of 32 recurrent strictures still Jess chan 1-cm were all failutes. Follow-up was a ‘minimum of 2 years (Ilusmann and Rathbun, 2006), Dorsal inlay graft was used in 37 strictures after a mean of two hypospadias surgeries in a series of patients with mean age 12 years, uring follow-up for a mean of 2 years, recurrent strictures were diagnosed in only 3 patients (6%) (Ye etal, 2008), Diverticulum Five of nine patients undergoing Byass fap repair for proximal hypospadias by me (W.S.) developed diverticula. None had distal obstruction and the strip that was tubularized was approximately the width of the open glans. 1 concluded that this ballooning resulted from the Telatively fixed resistance of the glans andjot turbulent flow from poor fixation of the flap to the corpora, causing the preputial skin to stretch (Fig. 117-27) Figure 147-27. Byars flap. 3422 PART XV. Pediatic Urology artos under the flap prevents adherence of the epithelium 0 the underlying corpora, potentially increasing risk for turbulent flow and diverticulum formation, A retrospective review of onlay versus tubularized preputial flap, repairs by Wiener and colleagues (1997) found that 12% of those tubulatized developed diverticulum during follow-up to a mean of 20 months, which might suggest that tubed laps have greater risk than onlays for this complication. However, Vallasciani and associ~ lcs (2013) reported 7% diverticula after both onlay and tbular- ‘zed preputial flap repairs during mean follow-up of 7 years; none hhad distal obstruction, Therefore, diverticulum can develop after either single- or two-stage tubularized laps or onlay preputial aps despite absence of distal obstruction. Diverticula are much less often encountered after urethral plate ot graft cubularizations Surgical Repair Calibration is done to detect associated distal stenotis, Then the diverticulum ie exposed by a ventral median raphe incision and ‘opened. A dorsal strip of sufficient width is outlined and the exces- sive tissue to either side is de-epitheliaized, The neourethra is sutured in two layers over a catheter to restore a normal caliber and the redundant and de-epitheliaized flaps are dosed with vestover- pants suture to cover the repair. Results AL 5 patients reported by Vallasciani and associates (2013) were successfully repaired without recurrent diverticulum during follow-up for an average of 9 years Balanitis Xerotica Obliterans BXO can present both preoperatively and following hypospadias fepat Iie cinealy diagnosed by the characteristic white discolor ation of the involved tissues (Fig. 117-28). BXO at the urethral Ineatus can both cause stenosis and extend into and along the lurethr, inducing steceure ‘Medical teatment using a topical steroid or tacrolimus has been reported. A double-blind RCI compared. 0.03% mometasone furoate to placebo applied daily for 5 weeks for BXO phimosis. ‘Although steroid therapy significantly improved foreskin retraction, histologic findings of BXO persisted (Kis el, 2001), Therefore best weatment is complete excision of al involved lssues with ral ruucora graft urethral replacement 'We have anecdotal experience with 2 patents with recurrent [XO after ptior excision and oral mitcosa grafting. In both the condition recurred at the new junction of the glans to the neoure- thra, in one 9 years later. Both have been managed with periodic topical therapy to control symptoms, given concerns that BXO ‘could recur again at the skin margin if further excision is done. Surgical Repair XO is surgically excised, When it occurs at the meatus or within the urethra, all grosely involved issues are removed and replaced ‘with oral mucosa in a two-stage graft urethroplasty described in the later section on Two Stage Oral Mucosa Graft Use of ether genital ‘or nongenital skin rather than oral mucosa is astociated with BXO Results Bracka (2011) changed ftom skin graft to oral mucosa to treat BXO- after observing initially good results with subsequent failure when BXO recurred. However, he did not present data indicating the percentage of recurrences or the time frame in which they occurred, ‘To our knowledge there are no credible reports of BXO recur. rence within oral mucosa HYPOSPADIAS REOPERATIONS Although fistulas are the most common complication following bbypospadias repair, glans dehiscence is the most common indica: tuon for reoperative urethroplasty, Regardless of the problem requir ing reoperation, decision making is systematic (see Fig, 147-7). 1f the urethral plate remains and is not grossly scarred, TIP urethro- plasty isthe first option. When the plate has been excised buta skin Sip without gross scarring remains, then inlay grafting isthe pre- ferred repair. When the urethral plate or skin substitute is grossly scarred of there is VC greater than 30 degrees, neourethral hait, BXO, or stricture that nearly obliterates the lumen, two-stage oral mucosa grafting is done, ‘Although skin flap reoperations are an option when there is suf- ficient skin for urethroplasty without compromising penile shaft covering, we do not often encounter such patients and do not use flaps mn addition, when a Mathieu or onlay flap has been done in a patient requiring reoperation, we remove the fap and perform TIP Urethroplasty, both to securely approximate the glans wings and (o avoid a potential diverticulum once the glans is well closed. ‘When there ate indications for a two-stage graft repair, we com- pletely excie the unhealthy neourethra to native urethra so that the Figure 147-28. Balanitis xerotica obliterans (BXO). A, BXO causing secondary phimosis after prepuciopla 8B, Perimeatal BXO causing meatal stenosis. C, Wound dehiscence subs ‘quently developed extensive BXO around the meatus and surrounding shaft skin. final neourethra has uniform composition to reduce concem for ‘contracture at tissue junctions or a diverticulum from tubularizing tissues of varying elasticity Tubularized incised Plate Urethroplasty Indications Reoperative TIP urethroplasty is an option when the urethral plate remains after prior surgery and lacks gross scarring. In our series of 133 consecutive reoperations (Snodgrass et al, 2009), 69 patients (5296) with a mean of 11 failed repairs (range 1 to 3) met those criteria Surgical Technique [A ventral Y incision is made, re-creating glans wings along their visible junction to the urethral plate and continuing in the median raphe to near the penoscrotal janction (Fig. 117-29). The glans ‘wings are first dissected laterally along the corpora to 3. and 9 o'dock and then from superiorly another approximately 4 mm off ‘the corpora as described in the earlier section on Distal Hypospa- dias Repait (see Fig. 147-11B), Dissection along the ventral penile shaft is done immediately, under the skin o preserve ventral dartos fora barrie lap. Degl is not necessary unless there is excessive dorsal shaft skin of VC to Hf Mathieu or onlay laps were previously done, this skin is now removed. Then the urethral plate is incised in the dorsal midline as with primary TIP repair and tubularized over a 6-Fr stent passed into the bladder For distal reoperations, continuous subepithelial, 7-0 polyglactin is used in two layers. More proximal reoperations have two layer subepithelial urethroplasty using interrupted poly. glactin followed by continuous polydioxanone, The neourethra is Figure 147-29. Ventral incision for hypospadias reaperations. Ventral Incision for eoperative urethroplasty and glansplasty provide sure to ventral dartos for barrier flap and facilitates cosmetic skin revision without need to deglove the penis. Chapter 147 Hypospadas 3423 next covered with either a ventral dartos flap of, if dartos is insuf- ficient, a tunica vaginalis Nap. Glansplasty is done using subepithelial interrupted 6-0 poly- glactin as described earlier for primary distal TIP urethroplasty. [Excess ventral shaft shan is excised as needed and the skin edges are closed using subepithelial 7-0 polyglactin. The stent is maintained for 7 to 10 days, Results Urethroplasty complications were reported in 12% to 30% of patients after reoperative TIP repair, Prior urethral plate incision from either MAGPI or TIP repair does not impact outcomes. A dartos flap over the neourethra significantly reduced fistulas in fone report, Ninety percent ofthe patients in out series had distal hypospa: dias and failed an average of one prior repair (maximum of three) (Snodgrass e< al, 2009). The indieation for reoperation was glans chiscence in 91%, with coronal fistulas, meatal stenoses, and diverticulum in the others. Initially (WS) used a smaller incision and did not routinely cover the neourethra with a dartos flap, but after $ of the first 10 cases developed fistulas a barrier layer was ‘used systematically in all others, resulting in a significant reduction in fistulas (2 of 53 cases). Urethroplasty complications occurred in 12 of 63 patients (19%) with follow-up to a mean of 6 months (cange 1 to 53), including seven fistulas and six recurrent glans dehiscences, before we implemented the extended glans wings Uisseetion. Prior urethral plate incision from TIP urethroplasty or MAGPI (appendix) did not impact outcomes (Snodgrass al, 2005). AA retrospective review by Ziada and colleagues (2006) had 30 ‘TIP reoperations after a mean of 1.6 prior repaits (range 1 to 3) in which 63% were distal. Urethroplasty used subepithelial polygla {in covered by a dartos flap. During follow-up of more than 4 years there were nine complications (30%): eight meatal stenoses (Give ‘with fistulas) and one isolated fistula ‘Another review included 40 patients for whom the meatal loca tion and number of prior operations were not stated. During follow-up to a mean of 42 months, urethroplasty complications were diagnosed in 5 patients (12.5%) (Riceabona et al, 2003). Dorsal Inlay Graft Indications ‘This technique is used when the urethral plate has been removed but a strip of grossly healthy skin remains init place [tis also used for neourethral strictures unless the lumen is nearly obliterated seh that a dorsal incision and grafting are not practical. ‘Surgical Technique ‘The operation is done as described for TIP reoperations. The skin strip substituting for the urethral plate is incised dorsally as for TIP ‘urethroplasty, but then the defect created is grafted using oral smiicosa harvested from the upper lip for small grate or the lower lip for longer grafts (Fig, 147-30). The visible margin of the lip is smatked and two 5-0 polypropylene stays are placed to retract it ‘open. The desired graft is outtined, injected with 1: 100,000 epi nephrine, and harvested. The graft is defatted. A small graft is easiest to handle by wetting a mall area on the paper drapes, which holds it sll. Then itis sewn (o the perimeter of the incision using 7.0 polyglactin and quilted in the midline with 6-0 polyglactin om an RB-1 needle, which readily punctures the graft and secures it to the underlying tunica albuginea of the corpora, Urethtoplasty, glans plasty, and skin closure then proceed as in TIP reoperations, Results ‘Three reports all found urethroplasty complications in 15% of patients after inlay graft reoperation. 3424 PART XV. Pediatic Urology jure 147-20. Inlay graft. A, Skin strip has boon incised dorsally as for tubularzed incl plate repair B, Oral mucosa grafts sewn into the defect. Tubularization for single-stage repair fs done next. ‘Our report with 153 reoperations included only 16 patients (1296) meeting criteria for inlay grafts (Shoxlgass etal, 2009), The reals eas distal in 62% and the mean number of filed repairs ‘was 1.9 (fange 1 to 3). Indications were glans dehiscence in 13 patients and meatal sicnosi with a diverticulum in 1 patient. Ure- {hroplasty complications developed in 2 of 13 patients (15%) with follow-up: one recurrent glans dehiscence and one Betula (Saod- sass etal, 2003), ‘A retospective report by Ye and colleagues (2008) included 53 patients with an average of 2 failed repairs (range 1 to 6). Seventy percent had strictures; the remainder had glans dehiscence. Mean salt length oeas 5 cm taken from the lower ip. Urethroplastycom- Plcatons occured in 8 patients (15%) during follow-up fora mean (of 25 months: five fstlas and lee recurrent sects all at the proximal junction to the urethe ‘Another review had 52 patients with an average of 4 failed repairs (fange 1 to 18). Skin gras harvested from prepuce, pele shaft or groin were used with a mean length of 4 cm (range 1 15). Complications developed in 1696 during follow-up to 8 mean ‘30 months one fistula and four strctres athe proxitoal urethral junetion (Sdswennner et al, 2000) Teis not clear from the reports by Ye and colleagues and Schwen- ter and coworkers why suietures occured atthe proximal junction to the urethra. We redhice likeshood for this complication by estending the midline dorsal incision approximately mm into the rormal uecthra Two-Stage Oral Mucosa Graft Indications ‘This reoperation is used when the urethral plate or skin substiture is grossly scarred, or there is VC greater than 30 degrees, BXO, stic- tures that nearly obliterate the lumen, of hair in the neouethra Surgical Technique (Fig. 147-31) ‘The same initial ventral Y incision is made as described earlier for ‘other teoperations,re«reating glans wings and opening the median raphe, Then the entire neouretha is excised until normal urethra is encountered. All scarred ventral tissues are excised from the coxpo- ral surface, which also reestablishes @ groove between the glans ‘wings. Polypropylene stays are placed into the glans wings at the corona on either side ‘Most often VC is corrected by removal of the neourethra and scarred tissues, but when it persists ventral transverse corporotomies andjor dorsal plication are used for straightening as described in the section on Ventral Curvature earlier in this chapter. The native urethra is spatulated ventally and proximal urethrostomy is done, ‘suturing penile shaft skin or scrotum to the urethra at 4, 6, and 8 o'dock. Intravenous cefazolin is given before graft harvest. We ido not prep the mouth or change gloves after harvest. The visible ‘margin of the lower lip is marked and two 5-0 polypropylene stay itches are placed o pull the lip down. A damp gauze is packed lover the tongue to prevent blood from entering the throat. All available tissue should be harvested from neat the gum line to near the vermillion border and laterally on either side where the lip joins the cheek (Fig. 147-32). This extent of graft will cover from the tip of the glans to deep within the scrotum, The graft is injected with 1: 100,000 epinephrine and harvested. A gauze soaked in 1:1000 epinephrine is laid onto the harvest site to aid in hemostasis The graft is defatted and then laid into the defect. The distal end. is secured first, suturing the graft to the glans near the stays using interrupted, epithelial 7-0 polyglactin. Then the graft is stitched along the distal edge of the glans with subepithelial sutures to prevent marks atthe site of the future meatus, The graft is gently suciched proximally and secured (o the shaft skin along its perim- ster with 7-0 polyglactin. The proximal end is incised in the midline and each arm is extended to either side of the urethrostomy (Fig, 147-53). The graft is then quilted to the underlying tunica albuginea of the compora using 6-0 polyglactin on an RB-1 needle Sutures are placed at I-cm intervals beginning in the midline and then on either side (see Fig, 147 330). ‘Next, 5-0 polypropylene stitches are placed on either side near the urethrostomy and at intervals distally. A rolled Vaseline gauze ‘gently compresses the graft, held into place by these stays tied lover it The tie-over bandage and a catheter in the urethrostomy are maintained for 1 week, The donor site is left untreated to e-epitheliaize spontaneously, ‘st stage 2nd stage | E F 6 H Chapter 147 Hypospadas 3425 Figure 147-81. Two-stage buccal graftreoperation. A, Neourethra opened with ventral incision. B, Allunhealthy tissues, usualy entire neourethra, are excised. C, Deep glans groove reestab- lished and proximal urethrostomy completed. D, Buccal graft quted into place from meatus ‘to glans tip. Lip is used within the glans and subcoronal region, and cheek along the penile shaft. The junction of lip to cheek is made diagonally to minimize contraction. E, U-shaped incision along perimeter of neaurethra 6 months later. F, Two-layer tubulavization of the neo- lurethra using interrupted subepithelial polyglactin followed by continuous polydioxanone. , Barrier fap, usually tunica vaginalis, covers the entire neourethra, H, Glansplasty and skin closures completed with subepithelial sutures. (From Snodgrass W, Eimore J. Initial experience ‘with staged buccal graft [Bracks] hypospadias reoperations. J Urol 2004;172(4 Pt 2:1720-4) ‘There is no need for any care ofthe revascularizing raft between, stages. We always wait for 6 months before performing the second stage of the urethroplasty. In less than 10% of cases the graft will scar of contract such that partial or complete regrafting is required (Fig. 147-34). Focal narrowing can be corrected during the second stage either by inlay grafting ot by incorporating shaft skin into the neourethra, At the second stage the oral mucosa is outlined with a arker and the glans wings ate injected with 1: 100,000 epineph rine. The wings and shaft skin are dissected from the neo-urethral plate. Thete is litle need to dissect under the neoplate because ‘usually its edges are sufliciently mobile for approximation. Its then ‘ubularized using 6-0 polyglactin interrupted subepithelial stitches followed by continuous 6-0 polydioxanone for two-layer closure ‘A eslide is exposed and tunica vaginalis harvested for a baztier flap as described earlier in the chapter. Then glansplasty is done with 6-0 polyglactin, and skin edges are closed with subepithelial stitches, The eatheter remains for 2 weeks. Typical cosmetic results are shown in Figure 147-35, Results Graft Take. Graft contractures requiring regrafting occur in less than 10% of two-stage oral mucosa graft reoperations. ‘Snodgrass and colleagues (2009) reported the need for addi tional grafting in 5 of 48 cases (10%), requiring a patch in 4 and complete replacement in 1. Since that report we changed to the 3426 PART XV. Pediatic Urology lower lip as the donor site and harvested larger grafts, In the next 63 patients, 2 (396) had regrafting for contractures. Because some fextent of contractare can occur with grafts, we now harvest the largest piece available within the donor site, and then excise any ‘excess during the second stage ‘Two retrospective series also reported regrafting before tubular: ization. One noted partial graft loss needing regrafting in 2 of 34 (64) buccal mucosa grafts (Gill and Hameed, 2011) and the other ound 4 of 30 (13%) cheek and/or lip grafts vequiting regrafting (leslie eval, 201), Urethroplasty Complications, Urethroplasty complications ‘were reported in up 10 38% of patients after the second stage Shouigrass andl colleagues (2009) reported urethroplasty compli ‘ations after the second stage in 17 of 45 (38%) patients with follow-up, These included glans dehiscence (n = 8), fistulas (n-— 7), and meatal stenosis (n= 2). ll glans dehiscences in this series ‘occurred when cheek, rather than lip, was sed, possibly because Figure 147-92, Labial extending aft harvest, Oulline for graft harvest from ‘the gum to within approximately 3 mm of lip. This graft will surface from the glans to ximally,n boys. The donor site fs not sutured, a Beil Figure 147-83. Oral mucos the p {grafts from the cheek are visibly thicker, making glans closure more difficult and tenuous (Fig. 147-36). Dehiscence likely also related to glans width, because most of these reoperations were in patients born with proximal hypospadias, but we did not measure the glans that time, We have subsequently observed glans dehiscence fol- lowing tworstage graft repairs despite using lip mucosa, Therefore ‘we continue to harvest lip but routinely additionally perform the more extended glansplasty we have described throughout this chapter atthe second stage to reduce risk for this complication “The series published by Gill and Hameed (2011) included a total of 100 patients, with most having two-stage grating using other graft sources such as prepuice, postauricular skin, and medial upper arm skin, They did not report the total number of patients with complications but stated there were nine fistulas, six stictures, six patients with persistent hypospadias (most likely dehiscence), and fous patients with persistent VC. Leslie and colleagues (201!) found complications in 1 of 30 (3798) cases: meatal stenosis in 5, fistulas in, and glans dehiscences in 3 ADULT OUTCOMES AFTER PREPUBERTAL REPAIR Despite the obvious need for information regarding urinary and sexual finction in adults following hypospadias repair in child- hhood, few data are available. Rynja and colleagues (2011) conducted a systematic literature review through 2010 to determine outcomes in men of mean age 27 years operated for hypospadias a lese than G years of age. Twenty stidies with 1069 patients were included, The ‘mean number of surgeries was 2,7, and proximal repair outcomes Were available from 180 men. Proceduzes used included Ombre- danne, Denis Browne, van det Meulen, and Cecil-Culp (no longer widely used), as well as MAGPI and Mathieu procedutes, onlay and tubularized preputial flaps, and Byars laps. There were 742 controls ‘with mean age of 20 years. At this time there areno similar data for TP outcomes in adults operated as children, Urinary Function ‘Symptoms Patients reported significantly more obstructive symptoms (77 of 217 [35.5%] vs. 30 of 196 [1594)), spraying (245 of 818 [30%] ve. BS ee TY dd ‘raft. A, A single pit dofect from the glans to within the scrotum. B, Note the extensions of graft to either si imal urethrostomy. C, The graft is quited and next atie-over bandage will be placed. ‘of lower lip graft has been used to fll the ot Chapter 147 Hypospadas 3427 Figure 147-84. Graft scar and contracture. A, Stllat sear in midportion of graft. B, istal graft contracture. C, Desired healthy appearance of well-vascularized graft. From Snodgrass W, Elmore J. inital experience with staged buceal grat [Bracka hypospadias reoperations. J Urol 2004;17246 Pt. 1720-4) Figure 147-38. Cosmetic outcomes after two-stage buceal graft re W, Elmore J. intial experionce with staged buccal graft [Bracka] hypospadias reoperations. J Urol 2008;17244 Pt. 2}:1720-4), 17 of 231 (796), and deviated stream (69 of 267 [269] vs. 9 of 81 (19%)) than did control Those with proximal bypospadias bad more spraying (46 of 106 [43%]) than did pacents with distal repais (285 of 818 [30%] Uroflowmetry (Qmax was significandy less in patients than controls (mean 24 ml sec vs. 30 mljsec), as was Qmax less than two standard deviations (36 of 265 [13.5%] vs. 4 of 138 [3%]}. Patients with proximal hypospadias bad significantly lower Qmax (mean 21 mlsee) than did those with distal hypospadias, Sexual Function Ejaculation Fjaculation problems, including milking semen and poor force, were significantly more common in patients than controls (99 of 385 [26%] vs. 0 of 48, P <.01) 3428 PART XV. Pediatic Urology Figure 147-36. Cheek versus lip grafts. A, Cheek mucosa grafts are thicker and may com- plicate glansplasty. 8, Labial grafts are thinner with significant less likelihood for glans dehiscence. Sexual Satisfaction Patients were les satisfied with sexual function than controls (153 of 188 [81%] vs. 235 of 252 [93%], P < 01). Mean frequency of intercourse per month did not vary (5.8 v8. 6.4). Cosmesis Patients were more likely to be dissatisfied with penile appearance (143 of 495 [29%]) than were controls (24 of 581 [4%]), Those with proximal hypospadias were more dissatisfied with penile appearance than those with distal hypospadias (25 0f 46 [5496] vs, 143 0f 493 (29%). IMPROVING OUTCOMES Upon relocating to Dallas in 1999, 1 (WS.) began recording data prospectively into Excel spreadsheets. The articles referenced in this chapter since that date ae all based on analysis of these databases, hich today contain information on more than 1600 consecutive patients, Reviews of these data have improved our surgical tech- ‘niques, outcomes, and understanding of the underlying factors that impact results in hypospadias repair. Determining Results [At this time there is no computer software that connects preopera- tive, intraoperative, and postoperative data to create a surgeon scorecard, but with the growing reliance on electronic medical records iC is only a matter of time before this occurs. Meanwhile, surgeons can enter pertinent data into an Excel spreadsheet to rapidly determine their personal outcomes. Recognizing the factors that best predict urethroplasty complications, a surgeon need only. enter the patient name, date of operation, meatal location, glans ‘width, primary versus reoperation, operative procedure, date of follow-up, and any complication noted. Depending on individual volume of repairs, that surgeon will leam bis or her complication rate using reliable data within as litte as 1 year ata cost of only a few minutes a week (o enter the information after surgery or clinic, Technical Changes When surgeons decide to perform a quality assesement of their surgical outcomes, whether of hypospadias or other conditions, they most often learn there are opportnitie for improvement. For ‘example, I (W'S.) was surprised that my fistula rate afer proximal ‘TIP urethroplasty was 259% despite using a dartos flap over the neourethra, However, there cates are relatively infrequent for most pediatiic urologists, who according to the American Board of Urology pesform an average of two per year, and so even this high a complication rate may go undetected because patients with com. plications present sporadically and recall bias limits our ability 10 tabulate them without spreadsheets or chart review. Having recog- nized this, (WS.) madea series of echnical modifications described in this chapter that significantly reduced my fitula rate ‘We similarly noted glans dehiscence atrates higher than reported, and initially thought it might be duc to glans wings approximation using chromic catgut. I (WS. therefore changed to polygiactin, but subsequent multivariable analysis, made possible by ongoing data collection, showed that suture type did ot impact this complica- tion, which was seen to be more common in proximal than distal repairs despite the same aurgeon using the same technique for glansplasty, That led to measurements of glans size, which con- firmed suspicions that dehiscence and other urethroplasty compli- cations were more prevalent when glans width was less than 14 mm. Knowing androgen will increase glans circumference, we embarked fn a program of preoperative testosterone injections for the small glans. We leamed that this objective patient selection resulted in double the number of boys receiving stimulation compared to our previous subjective use for a “small appearing” glans. We also ‘encountered unexpected androgen resistance in two thirds of those treated, requiring injections greater than 2 mg/kg to achieve tar {geled growth to 15 mm of greater. Most importantly, further out- comes review ultimately found that despite growing the glans to a size previously determined (@ have low complication rates, those ppatients who needed stimulation continued to have significantly more dehiscence than other boys whose glans was the desired size ‘without stimulation, Because reduction in complications, not ‘growth of the glans, was the aim of therapy, we then stopped andro- ‘gen tealment ‘This focus on glans size and dehiscence made me (W'S.) more aware of variations in glans wings dissection, and more receptive to change in my technique. When I observed two senior Japanese ‘surgeons perform a more extended glans wings mobilization, T recognized ite potential and incoxporated it into out practice. Improving Results Prospective data collection, periodie outcomes review, and practice changes such as these make us better hypospadiologists and improve resus for the young patients entrusted to our hands Of these, data collection is most important, because once a surgeon learns his or her actual results from data he or she knows are reliable, changes in technique and improved outcomes inevita- bly follow: Conversely, if low complication rates are found, a surgeon benefits from knowing there is no need to change current practice, which is especially useful should a cluster of complications ‘occur that otherwise might raise doubts, This isthe core of evidence- based surgical practice Please visit the accompanying website at ww expertconsult com to view videos associated with this chapter REFERENCES. @ Mie coma ce ona ont ate eprom ‘SUGGESTED READINGS Braga LH, Lorenzo Al, Bag Dl, etal Venta penile lengdhening versus ‘loral pheaton for severe vente curvatine sn chidsen wily proximal hypospadias.) Urol 2008;180:1743-7, Bush Villanueva C, Snodgrass W Glans size and urethroplasty compl ‘dons after hypospadias cepaic. Paper presented at. Society of Pediatc {Urology Ansttal Meeting: 2013; San Diego, CA Chapter 147 Hypospadas 3429 Bush NC, Holzer M, Zhang S, eal. Age does not impact isk for wrethro- plasty complications afer ebslaried incised plate repair of ypospacas {in prepubertal boy. | Pediat Urol 2012:9:252-6 Ferra ZaceatuA, Spano eal Skin graft for 2stage restment of severe Thypospadias: back tothe futute! J Urol 2002,1681730-3, ayashi ¥, Kojima Y, Nakane A etal. Can a shiclike meator be achieved ‘with the Vincision sutured meatoplasty fr onlay island Nap hypospadias epae? BML Int 200799147982, Snodgrass W, Bush N. Tubularzed incised plate proximal hypospadias ‘epale continued evolution and exiended applications. | Pediatr Urol 2ou472-9, Snodgrass W, Cost N, Nakonezny PA, etal. Analysis of rik factors for glans Gehiscence afer tubulanzed incised plate hypospadias repair. J Urol doltstes.183-9, Snodgrass WT, Bush N, Cost N. Algorithm for comprehensive approach to ‘hypospadias eoperation wsing 3 techniques.) Utol 2009,182-2885-91, Snodgrass WT, Bush N, CostN. Tubulasized incised plate hypospadias pa {for distal hypospadias} Pediatr Urol 2010;6:408~13. Wilkinson Dy, Farell Kenny SE. Outcomes in distal hypospadias: a ‘systematic review of the Mathieu and tubulariaed incised plate rept, Pediat Urol 2012:8:307-12 REFERENCES Allen JS, Summers JL, Wilkerson JE, Meatal alibration of newer boys Pte! 1972,107.438 Almodhen F,Alzahrani A, Jednak R, etal. Nonstented (ubulatized incised plate utthroplasty with Yo. spongioplasty in non tilt trained chil fren, Can Urel Assoc} 200852: 110-4 Andesson M, Dorosekiewicz M, Asfwidsson C, etal. Hypospadias repair ‘with tubularzed incised plate: does the obstructive ow pattern resolve spontaneously | Pediatr Urol 20117:441-3, ‘Aoki KFsjimoto K, Yoshida Kec al. 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