Seminars in Fetal & Neonatal Medicine (2008) 13, 164e170

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Abnormalities of the fetal bladder

Jenny Yiee a, Duncan Wilcox b,*
a b

David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA Department of Urology, UT Southwestern, Medical Center at Dallas, Dallas, TX 75257, USA

KEYWORDS
Bladder exstrophy; Bladder outlet obstruction; Megacystis; Posterior urethral valves

Summary This review aims to outline the management of a fetus in whom a distended bladder or an absent bladder has been identied during prenatal fetal screening. The causes, predelivery and immediate post-delivery treatment options are discussed, as well as prognosis. 2007 Elsevier Ltd. All rights reserved.

Introduction
The fetal bladder can be identied in the pelvis from 11e 12 weeks post-menstrual age1,2 and persistent absence of the bladder should be considered as abnormal from 15 weeks gestation. Fetal urine production begins between 8 and 10 weeks gestation. Prior to this, amniotic uid is thought to be primarily a dialysate of fetal blood across the skin, which is permeable at this stage. Thus, oligohydramnios cannot be found prior to 10 weeks of age.3 The review will address causes of abnormalities in the fetal bladder from absence to enlargement.

Megacystis
Denition
Megacystis in the rst trimester of gestation occurs in 1 in 1831 pregnancies.3 Half of these resolve spontaneously. In
* Corresponding author. Tel.: 1 214 456 3299; fax: 1 214 648 4789. E-mail address: duncan.wilcox@childrens.com (D. Wilcox).

a screening study by Sebire et al., normal controls all had longitudinal bladder diameters less than 6 mm in weeks 10e14.4 Although bladder diameter increased with gestational age, bladder diameter was always less than 10% of the crownerump length. Patients with bladder diameters >17 mm in the rst trimester experienced progressive obstruction, while most patients with diameters of 8e 12 mm had resolved by 20 weeks. In a follow-up study, fetuses with bladder diameters >15 mm were found to have a poor prognosis.5 Abdominal ultrasound is able to assess the bladder during gestation weeks 11e14 in 90% of cases. Combined use of abdominal and vaginal ultrasound improves this ability to 99%.6 In the second trimester, megacystis has been dened as an abnormally large appearing bladder, with or without the failure of the bladder to empty over 45 min.7 Other criteria used to evaluate megacystis include gender, chromosome analysis, the presence of oligohydramnios, posterior urethral dilation (keyhole sign), thickness of bladder wall, presence of a patent urachus, other organ or limb anomalies, presence of ureteral dilation, renal pelvic diameter, renal echogenicity, renal cysts, and renal cortical thinning.3,7,8 The abnormal range for anterioreposterior diameter of the renal pelvis in the transverse view is widely

1744-165X/$ - see front matter 2007 Elsevier Ltd. All rights reserved. doi:10.1016/j.siny.2007.10.002

Abnormalities of the fetal bladder debated. However, less than 10 mm is often designated as abnormal in the second trimester.7e9 Aneuploidy can be found in 25% and associated malformations in 33% of fetuses with megacystis.10 The most commonly found abnormalities are trisomy 13 and 18.5 These factors are used in combination in an attempt to predict aetiology and prognosis of the megacystis. Diagnosis is important as some conditions, such as posterior urethral valves (PUV), may benet from in-utero treatments, e.g. vesicoamniotic shunt, which would not benet other conditions such as vesicoureteral reux (VUR). The correct diagnosis can help to predict fetal viability and prognosis when counselling the family.7,8 Some studies have found that megacystis and hydronephrosis in combination with renal echogenicity predict an obstructive, and thus poor prognostic, cause.7,11 Harrison et al. was the rst to show that bladder outlet obstruction during fetal development led to renal dysplasia and subsequent pulmonary hypoplasia.12 In evaluating amniotic uid, one study has suggested that an amniotic uid index <25th percentile when plotted against age is suggestive of future abnormal renal function.13 Traditional denitions of oligohydramnios are below the second to fth percentiles. It is not clear whether or not prenatal ultrasound can effectively diagnose the causes of megacystis. Robyr et al. reported a series of 24 male megacystic fetuses with prenatal ultrasounds prior to 25 weeks gestation.8 All pregnancies were terminated and all but one underwent autopsy. They found ultrasound diagnosis was incorrect in two-thirds of cases when conrmed by postmortem examination. This study suggested that the absence of hydronephrosis might point to urethral atresia, whereas the presence of hydronephrosis may indicate PUV or urethral stenosis. Urethral stenosis or atresia was more prevalent prior to 28 weeks gestation than PUV.

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Figure 1 Prenatal ultrasound of a male patient with posterior urethral valves. The thick-walled bladder and dilated posterior urethra (keyhole sign) are clearly visible.

Posterior urethral valves

PUVs occur in 1 in 8000e25,000 live births. The cause of valves is unknown, as they do not arise from a normal embryological developmental stage. One theory suggests they are the result of an abnormal insertion of the mesonephric ducts into the fetal cloaca.14 The classic ultrasound ndings PUV in a male patient include bilateral hydroureteronephrosis, megacystis, dilated posterior urethra and a thickened bladder wall (Fig. 1). Bilateral hydronephrosis is associated with a wide differential diagnosis, including PUV, prune-belly syndrome, urethral atresia and bilateral ureteropelvic junction obstruction, and VUR. Affected females, although rare, can present with bilateral hydronephrosis, and in this situation cloacal and urogenital sinus anomalies must be considered. When the primary ultrasound nding is megacystis, 25% have PUV.15 Montemarano et al. found thick bladder walls and a dilated posterior urethra suggestive of PUV,7 though bladder walls may not thicken until later in pregnancy. However, these authors also noted the same ndings in some cases of prune-belly syndrome, as well as an absence of ndings in patients with PUV. Some series suggest that early ultrasound (prior to 24 weeks) may miss up to 92% of PUV,16,17 thus advocating a second, later, ultrasound.

Postnatal management of PUV starts with immediate catheter placement for bladder drainage. Denitive treatment of choice is cystoscopic valve ablation.18 Outcome parameters of concern in PUV are renal function and continence. Smith et al. reviewed the long-term outcomes of 100 patients with PUV. Most (74%) were treated with valve ablation with an average follow-up of 11 years. Renal function at age 20 showed end stage renal disease in 38% and chronic renal failure in 51%. Continence was delayed, but 46% were continent at age 10, and all except one patient were continent at age 20. There was no difference in rates of renal failure or continence as related to primary treatment by valve ablation, vesicostomy, or high diversion.19

Prune-belly syndrome
The denition for this syndrome includes abnormal abdominal muscles, bilateral cryptorchidism, and dilated ureters (Fig. 2). The exact mechanism of this constellation of ndings is still unknown. Prenatal ultrasound ndings can include renal dysplasia (50% of cases), hydroureteronephrosis, enlarged abdomen, and a patent urachus (up to 30%).20,21 Postnatal evaluation should rst involve a cardiopulmonary examination, as this is the most life-threatening problem, followed by an ultrasound scan to evaluate the kidneys, ureters, and bladder. Voiding cystourethrogram (VCUG) was not routinely performed, as the introduction of bacteria from a catheter was felt to have serious infectious consequences;21 however, more recently, it has been performed to exclude any obstructive pathology. Reconstruction can include urethral reconstruction, ureteric re-implantation, orchiopexy, and abdominoplasty. In a series of 32 patients, Arap et al.22 63% had normal bladder emptying by urodynamics, 23% had daytime incontinence, and 9% were using CIC or a vesicostomy. Renal function in these series deteriorated in 6% of patients,

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J. Yiee, D. Wilcox no bladder obstruction, and no renal obstruction can be managed non-surgically.26

Vesicoureteral reux (VUR)

Severe reux can lead to progressive dilation of the ureters and bladder leading to a megacystisemegaureter complex.27 Montemarano et al. suggest that megacystis alone, in the absence of a thickened bladder wall, posterior urethral dilation, patent urachus, renal echogenicity or associated limb anomalies, is suggestive of VUR or non-reuxing, non-obstructive megacystisemegaureter.7 The goal is to determine the roles of reux or obstruction in the megaureter. Fetal intervention is usually not required due to lack of oligohydramnios. Based on the postnatal investigations, the child is placed on a treatment regimen for reux, monitored with serial examinations, or recommended for surgery.27 Currently, there is considerable controversy in the management of patients with VUR ranging from no treatment to surgical reconstruction.

Prenatal management
The majority of fetuses with a normal karyotype and bladder length in the range of 7e15 mm will resolve spontaneously whereas those with a bladder length >15 mm have a poor prognosis. In a large rst trimester screening study, carried out over a 10-year period, Liao et al. reported on 145 cases of megacystis.5 The karyotype was found to be abnormal in 30 cases (21%). Of these 30 cases of abnormal karyotype, 26 underwent elective terminations of pregnancy. The remaining four cases resulted in spontaneous abortions. Of the 84 patients with a normal karyotype and a bladder length between 7 and 15 mm, there was 1 spontaneous abortion, 12 elective terminations, and 71 complete resolutions. Of the 31 patients with a normal karyotype and a bladder length >15 mm, 1 was terminated early in pregnancy. The other 30 underwent serial scans to reveal echogenic kidneys and worsening megacystis. These 30 cases underwent eight spontaneous abortions and 22 elective terminations. The remaining two patients underwent vesicoamniotic shunting at 14 weeks gestation, both of which resulted in miscarriage.

Figure 2 Postnatal picture of a patients abdomen with the classic features of prune-belly syndrome.

though the traditional rate of chronic renal failure is higher at 30%.23

Urethral atresia
When severe megacystis is found in the rst trimester, the most common diagnosis is urethral atresia.4 In a series of 55 patients with oligohydramnios by Freedman et al.,24 20% were found to have urethral atresia. This nding can be associated with prune-belly syndrome. Urethral atresia portends a poorer prognosis than other diagnoses in megacystic patients.7,24

Prenatal interventions
The goals of prenatal intervention are to preserve renal function and to provide the necessary aqueous environment for lung maturation. Prenatal assessment should consider unilaterality versus bilaterality of hydronephrosis, presence of oligohydramnios, presumed postnatal diagnosis, evidence of dysplasia on renal ultrasound, renal function as measured by amniotic electrolytes in severe cases, and concurrent congenital diagnoses. Management options include follow-up ultrasounds in utero, follow-up imaging postnatally, termination of pregnancy, induced early delivery, fetal surgery and vesicoamniotic shunting.28 Unilateral hydronephrosis rarely requires intervention as a normal contralateral kidney will prevent oligohydramnios and intervention has not been proven to improve outcomes.29 One scenario in which intervention can be

Ureterocele
Many ureteroceles and ectopic ureters are associated with the upper pole of a duplicated kidney, especially in females. Ultrasound can demonstrate the presence of a duplicated system, a thin ureterocele wall in the bladder and dilated ureters. A postnatal VCUG can reveal a characteristic cobra head, representing a ureterocele as well as investigate for reux.25 Although prolapsed ureteroceles can cause outlet obstruction, and thus require early postnatal intervention, ureteroceles with mild (<grade 3) reux,

Abnormalities of the fetal bladder considered is the case of severe hydronephrosis causing compression of neighbouring organs, specically the lungs. Intervention is generally limited to males with bilateral hydronephrosis and outlet obstruction who develop oligohydramnios.29 The most common causes of obstruction are PUV and urethral atresia (often with prune-belly syndrome), though severe hydronephrosis due to reux and obstructing ureterocele have been reported.30,31 Extensive counselling with parents is needed in order to determine a course of action. Specic issues to address are the high mortality rate, with or without intervention, long-term outcomes with intervention and a high complication rate.30e32 The timing and utility of intervention is still a topic of controversy. One variable that may affect prognostic counseling is fetal renal status as assessed by percutaneous bladder aspiration. The ability of fetal urine sampling to predict renal outcome is not rmly accepted, but certainly it provides another variable to consider when evaluating the need for intervention. Fetal urine is hypotonic with normal parameters being a sodium < 100 mmol/L, chloride < 90 mmol/L, calcium < 2 mmol/L, osmolarity < 200 mmol/kg, beta-2 microglobulin < 508 mmol/L, and total protein < 0.2 g/L, although these values do change during gestation.33 Some have suggested that the sensitivity and specicity of sampling can be improved with sequential sampling of three specimens taken 48e72 h apart.34 Sequential specimens attempt to eliminate the possibility of electrolyte shifts and protein degradation in stagnant urine. Decreasing values with serial sampling suggest favourable renal function. Other parameters that should be considered include the ultrasound appearance of the kidneys to exclude dysplastic (and thus poor prognostic) kidneys, chromosome analysis to exclude anomalies and conrm male sex and gestational age, as oligohydramnios earlier than the third trimester carries a worse prognosis from pulmonary and mortality standpoints.27,35 Survival of patients without intervention ranges from 0% in those with poor prognosis by urinalysis to 42% in those with a good prognosis. Intervention may raise these rates to 38% in those with a poor prognosis and 69% in those with a good prognosis. An analysis of the ve largest published series by Coplen et al. revealed an overall survival rate of 47% in those with successful shunt placements.28 Success of shunt placement and retention in proper position is not guaranteed. In a 10-year series with 10 patients, Coplen et al. reported a 50% rate of successful shunt placement. Only 50% of these successful shunts remained in place for the duration of gestation.31 In addition, the rate of shunt-related complications in this series was 50% and included retraction requiring laparotomy, evisceration, premature labour and death. The successful placement of a shunt still requires longterm multispecialty follow-up. In two large studies with 4e5 year follow-up, postnatal death rate in those with successful shunts ranged from 9% to 34%.30,36 The most common cause of death in both series was pulmonary hypoplasia. Respiratory problems are common. Between 43% and 67% of patients will have asthma or pulmonary infections later in life; however, only 0e11% will have restrictions in daily activities secondary to pulmonary issues. In assessing renal function, 33e36% had renal failure, 21e 22% had renal insufciency and 45e46% had normal renal

167 function. Nadir creatinine has been suggested as a predictor of future renal function. Freedman et al. reported a series of 14 patients with mean 54-month follow-up. The results showed that, while all patients with a nadir >1 mg/dL progressed to end-stage renal disease, 75% of those with a creatinine <0.8 mg/dL had normal renal function.30 The need for intermittent catheterisation varied from 33% to 43% for bladder dysfunction, though most patients were continent. Interestingly, in a quality-of-life survey, patients with a history of shunts reported the same satisfaction with life as healthy subjects.36 Other prenatal interventions for bilateral hydronephrosis are fetal surgery and induced delivery. No studies exist as to the utility of early delivery. Open fetal vesicostomies and ureterostomies have been performed in the past. However, these are not an active area of investigation due to concerns of morbidity combined with a success rate not higher than less invasive procedures.37,38 Endoscopic cystoscopy, placement of vesicoamniotic shunts and ablation of valves have been described both from antegrade and retrograde approaches.39,40 Possible advantages to treating valves as opposed to placing shunts include allowing the bladder to develop with physiological urine cycling, elimination of the possibility of shunt migration and similar calibre instrumentation, as used as for vesicoamniotic shuts. Currently, cystoscopic ablation is not widely available.

Absent bladder
When amniotic uid is present, but no bladder can be seen sonographically, this should be further investigated. A sonographically absent bladder results from the inability of urine to pass into the bladder or an inability of the bladder to store passed urine.

Epispadias, bladder exstrophy, cloacal exstrophy

The incidence of these malformations varies according to disease. Epispadias occurs in 1:117,000 births, bladder exstrophy occurs in 1:10,000 to 1:50,000, and cloacal exstrophy occurs in 1:250,000 births. Epispadias and bladder exstrophy occur more often in males with a maleto-female ratio of 2.3e5:1.41

Epispadias
Epispadias in boys is associated with a short, broad penis, a gapped pubic symphysis, dorsal chordee and usually descended testicles. Ejaculation can be abnormal due to a malformed posterior urethra, though testicular function is normal. In girls, the clitoris is split with an anteriorly placed vagina, with normal fertility, though uterine prolapse can be present due to the lack of puborectalis muscle pelvic support. Both males and females may be incontinent due to an inadequate bladder neck mechanism. Therefore, prenatal diagnosis is based on an absent bladder (though in less severe cases of epispadias, the bladder may be small but present), a low-set umbilicus, and, in males, abnormal genitalia.

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J. Yiee, D. Wilcox

Bladder exstrophy
Bladder exstrophy starts in the fourth week of gestation due to the failure of mesenchymal cells to migrate between the ectoderm of the abdomen and the cloaca. As a result, no muscle or connective tissue forms over the anterior abdominal wall. In a retrospective analysis of prenatal ultrasounds of babies born with exstrophy, ve sonographic ndings were often seen: (1) absent bladder; (2) lower abdominal bulge of the exstrophied bladder; (3) small penis with anteriorly displaced scrotum; (4) low umbilical insertion; (5) widening of the iliac crests. Despite the classic ndings listed, the diagnosis often cannot made on prenatal ultrasound.42 The differential diagnosis of classic ultrasound exstrophy ndings includes omphalocele, gastroschisis and cloacal exstrophy. In the rst two, there should be a normally lled bladder within the pelvis. Cloacal exstrophy is difcult to diagnose, but should be suspected when concurrent bowel and genital abnormalities are seen. Postnatal management includes closure of the bladder and abdominal wall, usually within the rst 48 h of life (Fig. 3). Female genital reconstruction is often done concurrently; however, male genital reconstruction is often delayed to allow for penile growth. There is signicant morbidity in this disease including incontinence and poor fertility. Extensive reconstruction is often required to achieve continence and spontaneous voiding. Fertility is decreased, probably due to the reconstruction needed for genital cosmesis. Mortality is low, but some have suggested that termination of pregnancy should be discussed.43

Cloacal exstrophy
Cloacal exstrophy is also known as the OEIS complex (omphalocele, exstrophy of the bladder, imperforate anus, and spinal anomalies).44 Criteria for prenatal sonographic diagnosis are not as well delineated for this disease as for bladder exstrophy. The main criterion is an absent bladder combined with another anomaly, such as omphalocele, persistent cloacal membrane, lumbosacral anomalies, limb defects, renal anomalies, ascites, widened pubis or one umbilical artery.45 Renal abnormalities, such as agenesis, hydronephrosis, multicystic dysplastic kidney or hydroureter, have been described in 60% of patients. Spinal defects, such as spina bida or kyphoscoliosis, have been described in 70% of patients. Lower limb anomalies have been seen in 18e65% of patients. Other ndings can include a single umbilical artery and elevated alpha-fetoprotein levels. As with bladder exstrophy, there is signicant morbidity associated with this disease. Extensive surgery is required with the future likelihood of fecal and urinary incontinence. Initial surgery is undertaken in the rst week of life and includes closing the bladder, abdominal wall and extrophied bowel. The majority of patients require a permanent intestinal end-stoma. Historically, males have been raised as females with gonadectomies performed early in life, though improvements in the understanding of the disease and in reconstruction of the penis are changing this approach. Termination of pregnancy should be discussed if this complex disease is discovered early in gestation.

Figure 3 A male patient with bladder exstrophy. The penis can be seen as short and epispadiac.

Bilateral single ectopic ureters

Single-system ectopic ureters are more common in males, as opposed to duplex systems, which are more common in female.25 Ectopic ureters are the result of abnormal ureteric bud development. The ureteric bud enters the metanephric blastema medially, resulting in an abnormal ureteric insertion point. In males, this insertion can be at the bladder neck, prostate, posterior urethra or vas deferens. In females, this insertion can be at the bladder neck, any portion of the urethra, the vagina or the introitus.25 If the urine does not enter the bladder due to an ectopic insertion bilaterally, the bladder will never be seen on ultrasound while having normal amniotic uid. There are both bladder and renal issues postnatally in these patients. There can be urinary incontinence due to insertion distal to the urinary sphincter mechanism. Also, if the bladder never cycles, it can be underdeveloped. Ureters can be re-implanted, but this does not assure future incontinence. Subsequent reconstruction is invariably required. Renal dysplasia can also occur due to the abnormal interaction with the metanephric blastema in utero.46

Abnormalities of the fetal bladder

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urinary tract obstruction in the rst half of pregnancy. Ultrasound Obstet Gynecol 2005;25:478e82. Arger P, Coleman B, Mintz M, et al. Routine fetal genitourinary tract screening. Radiology 1985;156:485e9. Favre R, Kohler M, Gasser B, Muller F, Nisand I. Early fetal megacystis between 11 and 15 weeks of gestation. Ultrasound Obstet Gynecol 1999;14:402e6. Kaefer M, Peters C, Retik A, Benacerraf B. Increased renal echogenicity: a sonographic sign for differentiating between obstructive and nonobstructive etiologies of in utero bladder distension. J Urol 1997;158:1026e9. Harrison M, Ross N, Noall R, de Lorimier A. Correction of congenital hydronephrosis in utero. I. The model: fetal urethral obstruction produces hydronephrosis and pulmonary hypoplasia in fetal lambs. J Pediatr Surg 1983;18:247e56. Zaccara A, Giorlandino C, Mobili L, et al. Amniotic uid index and fetal bladder outlet obstruction. Do we really need more? J Urol 2005;174:1657e60. Casale A. Posterior urethral valves and other urethral anomalies. In: Wein A, Kavoussi L, Novick A, Partin A, Peters C, editors. Campbell-Walsh urology. 9th ed., vol. 3. Philadelphia: Saunders; 2007. p. 3583e603. Freedman A, Johnson M, Gonzalez R. Fetal therapy for obstructive uropathy: past, present. future? Pediatr Nephrol 2000;14: 167e76. Hutton K, Thomas D, Arthur R, Irving H, Smith S. Prenatally detected posterior urethral valves: is gestational age at detection a predictor of outcome? J Urol 1994;152:698e701. Dinneen M, Dhillon H, Ward H, Duffy P, Ransley P. Antenatal diagnosis of posterior urethral valves. Br J Urol 1993;72: 364e9. Gonzales E. Posterior urethral valves and other urethral anomalies. In: Walsh PC, Vaughan ED, Wein AJ, editors. Campbells urology. 8th ed., vol 3. Philadelphia: WB Saunders; 2002. p. 2207e28. Smith G, Canning D, Schulman S, Snyder H, Duckett J. The long-term outcome of posterior urethral valves treated with primary valve ablation and observation. J Urol 1996;155: 1730e4. Stephens F, Gupta D. Pathogenesis of the prune belly syndrome. J Urol 1994;152:2328e31. Smith E, Woodard J. Prune-belly syndrome. In: Walsh PC, Vaughan ED, Wein AJ, editors. Campbells urology. 8th ed., vol 3. Philadelphia: WB Saunders; 2002. p. 2117e33. Arap M, Giron A, Silva F, Arap S. Comprehensive surgical treatment of prune belly syndrome: 17 years experience with 32 patients. Urology 2004;64:789e93. Geary D, MacLusky I, Churchill B, McLorie G. A broader spectrum of abnormalities in the prune belly syndrome. J Urol 1986;135:324e6. Freedman A, Bukowski T, Smith C, et al. Fetal therapy for obstructive uropathy: diagnosis specic outcomes. J Urol 1996; 156:720e4. Schlussel R, Retik A. Ectopic ureter, ureterocele, and other anomalies of the ureter. In: Walsh PC, Vaughan ED, Wein AJ, editors. Campbells urology. 8th ed., vol 3. Philadelphia: WB Saunders; 2002. p. 2007e43. Direnna T, Leonard M. Watchful waiting for prenatally detected ureteroceles. J Urol 2005;175:1493e5. Atala A, Keating M. Vesicoureteral reux and megaureter. In: Walsh PC, Vaughan ED, Wein AJ, editors. Campbells urology. 8th ed., vol 3. Philadelphia: WB Saunders; 2002. p. 2053e94. Coplen D. Prenatal intervention for hydronephrosis. J Urol 1997;157:2270e7. Herndon C, Ferrer F, Freedman A, McKenna P. Consensus on the prenatal management of antenatally detected urological abnormalities. J Urol 2000;164:1052e6.

Conclusion
The fetal bladder can be assessed by ultrasound in the rst trimester. Bladders larger than 15 mm in longitudinal diameter are associated with a poor prognosis. Associated ndings to assess on ultrasound include presence of oligohydramnios, posterior urethral dilation, thickness of bladder wall, ureteral or renal pelvic dilation, and kidney cortical appearance. Common aetiologies of megacystis include PUV, ureteral atresia and VUR. The utility of in-utero interventions, such as vesicoamniotic shunts or cystoscopy with valve ablation, is still under investigation. An absent fetal bladder can be due to epispadias, bladder exstrophy, cloacal exstrophy, or bilateral ectopic ureters. Classic bladder exstrophy sonographic ndings include absent bladder, lower abdominal bulge, small penis with anteriorly displaced scrotum, low umbilical insertion, and widening of the iliac crests. Bladder and cloacal exstrophy require early postnatal operative intervention. All these conditions may be associated with incontinence and infertility later in life, despite surgical intervention.
9. 10.

11.

12.

13.

14.

15.

16.

Practice points
 Fetal megacystis is associated with a poor outcome.  Megacystis is not always associated with obstruction.  In a newborn with megacystis, bladder outlet obstruction has to be excluded.  Absent bladder can be due to inadequate lling of the bladder or because there is not an intact bladder.
17.

18.

19.

20. 21.

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