Koyanagi-Nonomura One-Stage Repair for Severe Perineal Hypospadias 19 Tomohike Koyanagi Katsuya Nonomura, Hidehiro Kakizaki Masashi Murakumo, Takashi Shibata Introduction Considerable difficulties of various kinds accompany ‘the surgical management of severe perineal hypospa- dias, Whether the case is hypospadias ofthe severest form o1 phenotypic expression of amore complicated underlying intersexuality must be verifiec diagnos- tically. To name a few characteristic features, hypo- spadias is exemplified by intrinsically deficient skin, along necurethra mustbe constructed from the per ‘neum to the glans, and its associated with propenile scrotal transposition, Subsequently very few have dared to perform repair in one stage, although spo- radic reports of the use of a free flap (Devine) or ‘vascularized pedicled island skin flap (Duckett) tech- nique are available (Ehrlich and Scardino 1982). Early in the 1980s some experts considered staged repair preferable in this group of children (Hodgson 19 King 1984). Later Woodard and Parrott (1991) devel ‘oped a one-stage repair combining the vagaries of ‘urethroplasty. From their experience they concluded that although these patients are amenable to a one- stage procedure at an early age with a high degree of success, over one third will require a secondary pro- cedure for complete correction of the anomaly, ren- dering one-stage repair @ challenging issue in this particular group. Over the past several years we have addressed the issue first by reporting the technique (Koyanagi etal. 1984), then by refining and expand- ing our experience (Koyanagi et al. 1988, 1993), as ‘well as by confirming the viability of a parameatal- based foreskin flap (Nonomuraet al. 1992), which has been consistently used in our one-stage repairregard- less ofits severity (Koyanagi etal. 1994). The follow- ing is a detailed description of our technique, which has been bestowed with the eponym Koyanagi-Nono- ‘mura one stage repair (Glassberg et al. 1998) Operative Technique Step 1: Outlining the Skin Incision and Dartos Mobilization A traction suture is placed on the glans and on each side of the dorsal foreskin. The meatus is dilated with the tip of a fine curved mosquitc clamp, or ventral ‘meatotomy is done in the usual fashion, wide enough toaccommodatea 6-Furethral silicone catheter (Cliny Create Medic, Japan). Using gentian violet the skin is ‘marked before incision. The incision encircles proxi- mal to the meatus, runs laterally on both sides along. the medial edge of the scrotal raphe, which by virtue ofthe bifid state isadherentto thelateral aspect ofthe penile shaft, and dorsally along the sulcus coronalis, over the foreskin until it meets with its counterpart at 12 o'clock on the dorsum of the penis. Lateral parameatal skin marking should be wide, as this is to form a base for the parameatal foreskin flap, but must stay medial to the scrotal raphe so that the scrotal skin is not included or incorporated in the flap. Similarly, citcumcoronal outlining of the dorsal foreskin should be away from the sulcus so that ‘enough preputial foreskin is included, as this is where the parameatal-based pedicle foreskin flap isharvest- cd in an extended form for eventual urethroplasty (© Figs 19.18, 1922). With fine curved iris scissors a sharp incision is made along the full circumference of the previously defined skin marking. While the glans and shaft are held under a fine dressing gauze, countertraction is applied to the dorsal foreskin by holding the traction stitches, and the skin proximal to the incision is mobilizedby cutting sharply in the plane between the dartos and Buck's fascia. This dartos mobilization of the skin should be extensive enough in both the dorsal and ventral aspects ofthe penis sothat when it is completed the penile shaft is completely degloved, being treed from the penile foreskin and lateroven- trally adherent bivalved scrotum. During the mobil- ization ofthe bivalveé scrotum one can often appre-192 Tomohike Koyanagi, Katsuye Nonomura, Hidehiro Kakizaki, Masashi Murakumo, Takashi Shibata Fig, 19.1a-k Schematic drawings of the operative technique. The solid lines outline the incisions (a, de) For explanation > please refer to the text. see p. 193Koyanagi-Nonomura One-Stage Repair for Severe Perineal Hypospadias 193194 Tomohiko Koyanagi, Katsuya Nonomura, Hidehiro Kakizaki, Masashi Murakumo, Takashi Shibata Fig. 19.2. a Outline ofthe skin incision b Penile shaft with parameatal foreskin is degloved of phallic foreskin and bivalved scrotum after dartos mobilization Fig, 19.5d) secu zes its watertightness, which should be confirmed by saline flushing of the neourethra. While closing the proximal portion of the neourethra, the laterally splayed out bulbocavernous muscle is alse brought to midline, restores normal perineal muscular anatomy as well as reinforcing the two-layered ure- thral closure, Inadvertent “bite” of axially running blood vessels in the flaps should be avoided. Several reinforcing stitches may be added as needed, Step 5:Glanulomeatoplasty ‘The distal end of the neourethra is anchoredto the tip of the bisected glans (> Figs. 19.1h, 19.6a), and then the glanular wings are approximated in the midline over the neourethra while itis held somewhat taut at 6 o'clock witha traction stitch. Temporary placement cof a small Penrose drainage tube over the neourethra facilitates glans approximation without undue bite of the urethra ( Fig, 19.66). As « consequence of ade- ld * stance is approximated at the renulum to scare vo ae Almureod loporton eAppeaancestthepansattheendot slanulomeatoplasty. Note the semicuffed sppearanveotthe eomeatus quate lateralization of the glanular wings this glanu- loplasty is done without any tension, which is con- firmed by free movement of the Penrose tube. Usual- ly two rows of vertical mattress sutures of 6-0 PDS are all that is necessary tor glanuloplasty. After enrollment of the neourethra with glanulo- plasty and removal ofthe Penrose tube, the somewhat protruding ventral edge of the neourethre is tlipped down sc that it can be stitched to the glanular edge in a semicufied fashion, thus completing the meato- plasty (. Fig 19.6c, d). Again, free movement of the inlying urethral catheters to be confirmed. Step 6: Byarsization of the Dorsal Foreskin and Its Subcutaneous Tissue for Skin Closure ‘The moistened gauze dressing protecting the dorsal penile foreskin is removed. Before byarsization of this flap for wound closure, another mark is added with gentian violet. On the dorsal cutaneous side, an ‘ordinary distal half-length of the fap is satisfactory (Figs. 19.1, 19.7a). The subcutaneous portion of the flap, however, should extend proximally deep 197198 Tomohiko Koyanagi, Katsuys Nonomura, Hic hiro Kakizaki, Masashi Murakumo Takashi Shibata Fig. 197. aDorsumof the foreskin before byarsization along. the marked line. The dorsally displaced appearance of the scrotum is beter appreciated. b Marking ofthe dorsal sub- cutaneous layer for byarsizaton,c The subcutaneous tissue i enough to include the subcutaneous layer of the dorsally displaced scrotal tissue (Fig, 19.7b). By cutting along the proposed outline, the foreskin flap is bivalved and the proximal subcutaneous layer is lateralized judiciously so that the subcutaneous trough thus created is wide enough to enroll the penile shaft with no tension at later skin closure (Fig 19.76). Needless to say, care should be taken not to injure the axially running branches of the superficial dorsal arterial system during this byars- ization, Step 7:Skin Closure A Penrose tube is left in the subcutaneous space as a drain ané brought out through a separate incision in the perineum. The wound is closed in multiple layers. With the penis held in dorsiflexion, the subcuta- neous tissue isapproximated ventrally over the penile shaft proximally to distally. The most proximal part of the subcutaneous layer is not the penile foreskin but rather the dorsally displaced scrotal skin (© Figs. 19.1, 19.8a), Thanks to adequate dartos mo- lization of the scrotal skin (step 1) ané judicious incised deep and lateralized wide to ensure snug rolling ofthe shaft atthe ventral midline, d Note the relevance for byas- ization ofnot only the subcutaneoustissue ofthe foreskin but also the dorsally displaced scrotal septum byarsization, this subcutaneous closure is done with- cout any tension. In so doing, the dorsally displaced scrotumis now replaced to a more natural ventriposi tion. Closure should be symmetrical and avoid in- advertent bite ofthe shaft so that it will not cause any torsion of the shaft of restrict its free movement on. erection (Eig. 19.8). ‘The coverage of the distal shaft is done similarly, proximally to distally in multiple layers (usually three to four) with byarsized foreskin flaps ( © Fig. 19.8). ‘The ventrally repositioned subcutaneous layer of the distal foreskin is then stitched to the subcutaneous tissue of the glans underneath the frenulum, thus securing the coverage of this portion of the urethra, which is potentially the most likely site of fistula formation (~» Fig 19.84). Once multilayered subcuta- neous closure is completed, the skin edges are ap- proximated loosely to avoid edema of the foreskin, thus concluding skin closure (> Figs. 19.1k, 19.8). ‘The traction suture on the tip of the glans is tied around the inlying urethral catheter, thus securing its position during the postoperative period. A flufly compression dressing is applied to the wound, and the penis is held in dorsiflexion under the compres- sion coverage. An antibacterial ointment is appliedKoyanagi-Nonomura On-Stage Repair or Severe Perineal Hypospadias Fig. 19.8. a The subcutaneous tissue ofthe dorsally displaced. 4ctotal septum is repositioned ventrally while the proximal Ihalfofthe wound is being close. b Several layers of einfore- ing subcutaneous sutures closing the scrotum are added. «Similar multilayer closure ofthe subcutaneous tissue of the foreskin lapis carried out. d The distal edge of the foreskin flap is stitched to the subcutaneous tissue of the glanular substance. e One-stage urethrophsty with simultaneous cor- rection of bifid scrotum is completed over the glans and around the meatus to prevent surface drying before the child is woken, Postoperative Care ‘The Penrose drain is removed on the Ist or 2nd post: ‘operative day. The compression coverage is released ‘on the 4th or Sth postoperative day, and the child takes a warm sitz bath two or three times a day during hospitalization, Similarly, meatal flushing with a jet stream of normal saline should be continued to pre- vent crust formation around the meatus (Redman 1983: Parsons and Abercrombie 1982). The inlying urethral catheter, which should be checked daily for patency, is to be removed on the 10th to 14th post: ‘operative day. No attempt should be made to mani- plate the urethra with acatheter or a sounc once the inlying catheter has been taken out. Results In 1995 we reported 10 years’ experience based on 70 patients subjected to this technique (Koyanagi et al. 1995). Their ages ranged from 2 to 12 years (mean. of 3.7 years). Primary success was obtained in 53% (37/70). Complications requiring secondary repair ‘occurredin 33 cases: these comprised meatal stricture requiring meatal recession or glanular dehiscence with meatal recession in 12, urethrocutaneous fistula, in 15 and urethral stricture in 6 cases. The patients concerned were subjected to secondary procedures such as extension urethroplasty or fistula closure, With a satisfactory result in over 90% of cases. Thus the overall success rate with this technique was 87% (61/70). This general trend has been the same over the subsequent years. The final outcome was satisfactory both cosmetically and functionally ( Fig, 19.9, 199200 Tomohike Koyanagi, Katsuya Nonomura, Hidehiro Kakizaki, Masashi Murakumo, Takashi Shibata Fig. 199. An example of the postoperative result. Inst: pre: ‘operative appearance showing the distinct propenile scrotal transposition often associated with this severe perineal ‘hypospadias Comments One-stage repair, which is currently considered stan- dard in the majority of cases of distal hypospadias ‘with ot without chordee, is still a challenging issue in more severe perineal hypospadias with various degrees of scrotal transposition, We have been per- forming one-stage repair ever since we first reported 1 prototype method for this severe hypospadias with bifid scrotum (Koyanagi et al. 1984). Our method of ‘one-stage repair, which has similarities with Russells ‘method (Russell 1900), has evolved since then. The ‘refined form reported here is distinct in several aspects First, dartos mobilization of the penile fore- skin and scrotum is extensive for eventual simulta- ‘neous correction of scrotal transposition with skin closure. Second, by virtue of the natural continuity with spongy urethra, the parameatal foreskin flap maintains satisfactory microcirculatory parameters (Nonomura et al. 1992). When blood flow measured ‘on laser Doppler was compated atthe tip ofthe para- ‘meatal flap before and after harvesting, there was only an 18% reduction with a parameatal-based and fully extended circumferential foreskin flap. Viability of this flap is further acknowledged with its response to papaverineload. This, along with the lack of necessity of performing end-to-end anastomosis to the old urethra, is beneficial in our method. Furthermore, excision of the urethral plate while harvesting the arameatal flap militates against the recurrence of chordee (Demitbilek et al. 2001). Third, approxima- tion of splayed out bulbocavernous musculature with formation of neourethra is expected to lessen post- micturition dribbling, Fourth, to bring the neomeatus to the most distal tip ofthe glans, it is bisected (Barcat 1973) rather than channeled by removing the core of glanular substance. We believe this is more an- atomical in dissection (Altemus and Hatchins 1991) and corrects the rather flattened globular deformity of the glans to a more natural cone-shaped one when it enrolls the neourethra (Turner-Warwick 1979). Lastly, meatoplastyin a semicuffed fashion adds afew extra millimeters to the urethral length and prevents ‘meatal stricture, Our complication rate of 40% is certainly higher than that of distal hypospadias, where less than 5% required a secondary procedure (Redman 1983), but not as high as our own complica- tion rate with multistage repair for severe hypospa- dias (Adachi et al 1987), and others are more candid by reporting an even higher chance (50%) of requir- inga secondary or moreoperations in their experience with pedicle tube urethroplasty (Dewan et al. 1991). By its sheer severity, one-stage repairfor this group of children is demanding, Some of our patients (n=14) required topical application of testosterone ointment for several weeks to months preceding the operation (Sakakibara et al. 1991) (Fig. 19.10a,b). Operation time is longer than in the distal type ofhypospadias. ‘Theurethral catheter has to remain in place longer for the wound to heal. All these perioperative obstacles and ditficulties notwithstanding, we still believe ‘one-stage repair is worthy of recommendation in all children with severe proximal hypospadias, as final ‘outcomes are satisfactory, both cosmetically and func- tionally, and in the long term as well (°Fig. 19.10c), It is pleasing to witness satisfactory results from ‘others who advocated our principles (Glassberg etal 1998; Emir et al. 2000; Sugita et al. 2001). Editorial Comment Emir et al. (2000) described a modification of the procedure of Koyanagi et al. designed to reduce the complication rate and the need for reoperation. The meatal-based yoke is outlined and the incision is 3 to allow mobilization of the urethral plate sufficiently to excise all chordee. The artificial erec- tion test is then performed to check for residual chordee, which, if present, should be corrected at this stage. Only then is the other incision made. To preserve the vascular supply to skin flaps, the flaps are joined together to form the neourethra after being tubularized and the tip is placed on the glans after dissection or by tunneling, The repair is completed by multilayer closure and skin coverage. The focus of this modification is to preserve the lateral blood supply to the necurethral laps.Koyanagi-Nonomura One-Stage Repair for Severe Perineal Hypospadias Fig. 1910a-c. A boy with severe perineal hypospadias. ‘a Genital appearance at the age of II days. b Appearance ‘at age 3 years affer nine cycles of topical application of References ‘Adachi Y, Nonomura X, Togashi M, et a Comparison of one stage and two stage urethroplasty for hypospadias. Jpn} ‘Urol, 1987; 7:667-673. Altemus AR, Hatchins GM: Development of human anterior ‘urethra. J Urol, 1991; 146:1085-1093, Barca J: Current concepts of treatment. In: Horton CE (ed): Plastic and reconstructive surgery of the genital area Litle Brown, Boston, pp 249-263; 1973. Demirbilek S, Kanmaz 7, Aydin G, eta: Outcomes of one: ‘stage technigues for proximal hypospadias repair. Urology. 2001; 58:267-270, Dewan PA, Dinneen MD, Winkle D, et al: Hypospadias Duckett pedicle tube urethroplasty. Eur Urol. 1991; 20: 39-42 Ehrlich RM, Scardino PT: Surgical correction ot scrotal trans- position and perineal hypospadias. J Pediatr Surg. 1982; 1775-177. ‘Emir H, Jayanthi VR, Nitahara K, etal: Modification of the ‘Koyanagi technique the singe stage repair of proximal hypospadias. | Urol 2000; 164:973-976 Glassberg KI, Hansbrough F, Horowitz M: The Koyanagi- ‘Nonomura I-stage bucket repair of severe hypospadias with and without penoscrotal transposition. J Urol. 1998; 160:1104-1107; discussion 1137 Hodgson NB: Commentary: review of hypospacias repair. Tin: Whitehead ED, Leiter E (eds): The current operative urology, 2nd edn Harper and Row, New York, pp 1233- 1242; 1984, King LR: Overview: Hypospadias repair. 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