Predicting postnatal renal function of prenatally detected posterior urethral valves using fetal diffusion-weighted magnetic resonance imaging with apparent diffusion coefficient determination.

OBJECTIVE: The objective of this study was to evaluate the accuracy of fetal diffusion-weighted magnetic resonance imaging with apparent diffusion coefficient (ADC) determination to predict postnatal renal function (nadir creatinine at 1 year and eGFR) of men with posterior urethral valves (PUV). METHODS: Between 2003 and 2014, 11 MRI were performed on fetuses (between 28 and 32 weeks) in whom second trimester sonography suggested severe bilateral urinary tract anomalies, suspected of PUV. RESULTS: The ADC of the 11 fetuses ranged from 1.3 to 2.86 mm2  s-1 (median = 1.79 mm2  s-1 , normal range for fetal kidney: 1.1-1.8). Two pregnancies with ADC > 2.6 mm2  s-1 were interrupted; the autopsy confirmed PUV and Potter syndrome. For the remaining nine babies, the follow-up was 5.4 years (0.8-10). Four children with abnormal ADC (1.8-2.3) had chronic kidney disease. The remaining five cases with normal nadir creatinine and eGFR had normal ADC. One case with unilateral elevated ADC had a poor ipsilateral renal function on dimercaptosuccinic acid scan. CONCLUSION: Here, it seems that diffusion-weighted magnetic resonance imaging with ADC determination could be useful in accurately evaluating fetal kidneys in PUV and predicting renal function. It may be an additional, non-invasive method when biologic and sonographic findings are inconclusive, especially in the case of oligohydramnios. Further studies are needed to confirm our data. © 2017 John Wiley & Sons, Ltd.

Is Hypospadias Associated with Prenatal Exposure to Endocrine Disruptors? A French Collaborative Controlled Study of a Cohort of 300 Consecutive Children Without Genetic Defect.

BACKGROUND: Numerous studies have focused on the association between endocrine-disrupting chemicals (EDCs) and hypospadias. Phenotype variability, the absence of representative comparison groups and concomitant genetic testing prevent any definitive conclusions. OBJECTIVE: To identify the role of occupational and environmental exposures to EDCs in nongenetic isolated hypospadias. DESIGN, SETTING, AND PARTICIPANTS: A total of 408 consecutive children with isolated hypospadias and 302 normal boys were prospectively included (2009-2014) in a multi-institutional study in the south of France, the area of the country with the highest prevalence of hypospadias surgery. OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS: In patients without AR, SRD5A2, and MAMLD1 mutations, parental occupational and professional exposures to EDCs were evaluated based on European questionnaire QLK4-1999-01422 and a validated job-exposure matrix for EDCs. Environmental exposure was estimated using the zip code, the type of surrounding hazards, and distance from these hazards. Multivariate analysis was performed. RESULTS: Fetal exposure to EDCs around the window of genital differentiation was more frequent in the case of hypospadias (40.00% vs 17.55%, odds ratio 3.13, 95% confidence interval 2.11-4.65). The substances were paints/solvents/adhesives (16.0%), detergents (11.0%), pesticides (9.0%), cosmetics (5.6%), and industrial chemicals (4.0%). Jobs with exposure were more frequent in mothers of hypospadiac boys (19.73% vs 10.26%, p=0.0019), especially cleaners, hairdressers, beauticians, and laboratory workers. Paternal job exposure was more frequent in the cases of hypospadias (40.13% vs 27.48%, p=0.02). Industrial areas, incinerators, and waste areas were more frequent within a 3-km radius for mothers of hypospadiac boys (13.29% vs. 6.64%, p<0.00005). Association of occupational and environmental exposures increases this risk. CONCLUSIONS: This multicenter prospective controlled study with a homogeneous cohort of hypospadiac boys without genetic defects strongly suggests that EDCs are a risk factor for hypospadias through occupational and environmental exposure during fetal life. The association of various types of exposures may increase this risk. PATIENT SUMMARY: Our multi-institutional study showed that parental professional, occupational, and environmental exposures to chemical products increase the risk of hypospadias in children.

Fetal megacystis: etiologies, management, and outcome according to the trimester.

OBJECTIVE: To describe the diagnostic criteria and outcome of fetal megacystis according to the gestational age at diagnosis. METHODS: A 7-year retrospective study was carried out from 2004 to 2011, including cases of megacystis referred to 2 prenatal fetal medicine units. The following data were collected and analyzed: maternal age, term at diagnosis (gestational weeks), ultrasonographic and magnetic resonance imaging data, karyotype, decision of the multidisciplinary prenatal team, fetopathology in cases of termination of pregnancy or fetal death, final diagnosis at birth after ultrasonography and voiding cystourethrography, and medical and surgical follow-up. RESULTS: Of the 69 fetuses included in this study, 82.6% were males; 26 were diagnosed during the first trimester, 21 during the second, and 22 during the third. During the first trimester, the main etiologies were urethral occlusions and prune-belly syndrome with poor fetal prognosis. Nineteen pregnancies (69%) were terminated for medical reasons including the association with other malformations, poor evolution, or miscarriage. Only 4 children were born alive. The main etiologies of megacystis discovered during the second and third trimesters were vesicoureteral reflux and urethral occlusion. Twenty of 22 fetuses (91%) were born alive when the fetal megacystis was discovered after 27 weeks of gestation. CONCLUSION: Antenatal discovery of megacystis is a complex and challenging prognostic situation. The prognosis depends on the gestational age at discovery. Megacystis is not always associated with obstruction. In a newborn with megacystis, bladder outlet obstruction has to be excluded. Optimal counseling of the involved parents requires a multidisciplinary approach.

Value of diagnosis imaging in the evaluation of the severity of histological lesions in duplex systems.

OBJECTIVE: In order to determine the effectiveness of imagery in the assessment of the severity of histological lesions in duplex systems in children we compared histology results from heminephrectomies with diagnosis imaging findings [renal ultrasound (US), scintigraphy, unenhanced and contrast-enhanced magnetic resonance imagery (MRI)]. MATERIALS AND METHODS: Between 2007 and 2013, 34 children with duplex system underwent surgery. The results from US (n = 34), dimer captosuccinic acid scintigraphy (n = 23) and MRI (n = 16) were compared with histological data. Five histological lesions were found (chronic interstitial inflammation, interstitial fibrosis, tubular atrophy, glomerulosclerosis and dysplasia) and categorized as severe (>25%) or moderate (≤ 25%). RESULTS: Severe histological lesions were found in 76.5% and moderate lesions in 23.5%. Radiological features were compared with histological results. In US, severe parenchymal thinning was associated with chronic interstitial inflammatory. The absence of parenchymal enhancement and/or severe cortical thinning in MR urography (MRU) was significantly associated with interstitial fibrosis. All poorly functioning poles were associated with severe histological lesions (p = 0.091), but not to a specific category of lesions. CONCLUSIONS: MRI sensibility was excellent (90%) in the diagnosis of poorly functioning pole. Severe thinning on US and minimal pole function on MRU can be used to predict the severity of histological lesions.

Minor hypospadias: the "tip of the iceberg" of the partial androgen insensitivity syndrome.

BACKGROUND: Androgens are critical in male external genital development. Alterations in the androgen sensitivity pathway have been identified in severely undermasculinized boys, and mutations of the androgen receptor gene (AR) are usually found in partial or complete androgen insensitivity syndrome (AIS). OBJECTIVE: The aim of this study was to determine whether even the most minor forms of isolated hypospadias are associated with AR mutations and thus whether all types of hypospadias warrant molecular analysis of the AR. MATERIALS AND METHODS: Two hundred and ninety-two Caucasian children presenting with isolated hypospadias without micropenis or cryptorchidism and 345 controls were included prospectively. Mutational analysis of the AR through direct sequencing (exons 1-8) was performed. In silico and luciferase functional assays were performed for unreported variants. RESULTS: Five missense mutations of the AR were identified in 9 patients with glandular or penile anterior (n = 5), penile midshaft (n = 2) and penile posterior (n = 2) hypospadias, i.e., 3%: p.Q58L (c.173A>T), 4 cases of p.P392S (c.1174C>T), 2 cases of p.A475V (c.1424C>T), p.D551H (c.1651G>C) and p.Q799E (c.2395C>G). None of these mutations was present in the control group. One mutation has never been reported to date (p.D551H). It was predicted to be damaging based on 6 in silico models, and in vitro functional studies confirmed the lowered transactivation function of the mutated protein. Three mutations have never been reported in patients with genital malformation but only in isolated infertility: p.Q58L, p.P392S, and p.A475V. It is notable that micropenis, a cardinal sign of AIS, was not present in any patient. CONCLUSION: AR mutations may play a role in the cause of isolated hypospadias, even in the most minor forms. Identification of this underlying genetic alteration may be important for proper diagnosis and longer follow-up is necessary to find out if the mutations cause differences in sexual function and fertility later in life.

Congenital Megaprepuce: surgical approach.

OBJECTIVE: To describe surgical procedure and results of Congenital Megaprepuce (CMP) that consists in phimosis and megaprepuce; glans and corpora trapped into pre-pubic fat; incomplete micturition with typical "ballooning" aspect. METHODS: Genitoplasty with penoscrotal separation (GPS) was performed at Pediatric Surgery Department of Hospital Nord - Marseille, between July and December 2010, in 5 uncircumcised children with CMP, by the same surgeon. Transversal incision at the ventral penoscrotal junction was used, with shaft bipartition and Buck's ventral fascia exposition. The inner layer of prepuce was resected while the outer layer was preserved for coverture of the penile shaft. The dorsal foreskin was medially and longitudinally incised to obtain adequate length of the dorsal shaft. The first middle dorsal suture was made using PDS 6.0 and completed in ventral direction with interrupted stitches. The urinary catheter was placed and hydrocolloid duoderm dressing used. Perioperative prophylactic antibiotic was given and pain control achieved with paracetamol and codeine. RESULTS: All patients presented urinary troubles before surgery. Skin penile coverage was complete in all patients with cosmetically and functionally satisfying results; no complication was observed at 12 months mean follow-up. CONCLUSION: CMP is a specific entity of "inconsistent penis." Surgical treatment should be discussed at diagnosis, resolving micturition troubles. GPS is a simple and reproducible procedure by experienced surgeons. Excellent cosmetic and functional results are obtained at mean follow-up, even if long-term results are required. GPS can be useful in other malformations of the penile shaft.

Antegrade percutaneous balloon dilation of ureteral strictures after failed pelviureteric or ureterovesical reimplantation in children.

OBJECTIVES: To assess the morbidity and success rate of percutaneous treatment of the postoperative ureteric strictures in children. MATERIALS AND METHODS: Between January 1994 and December 2003, 12 children were treated by antegrade percutaneous balloon dilation for postoperative ureteric strictures. Stenosis occurred at the pelviureteric junction in 5 cases and ureterovesical junction in 7 cases. The 10 boys and 2 girls were between 3 months and 14 years old (mean, 5 years ± 4.7 years). General anesthesia was used in 10 cases for nephrostomy catheter placement. Five ureteral stents were used additionally for nephrostomy drainage with a 6-F catheter. Both nephrostomy and ureteric stents were in place for 28.5 ± 12 days, then removed after control antegrade pyelography. RESULTS: Dilation was technically successful in 9 of our patients. Two peroperative complications occurred. Postoperative results were evaluated by ultrasonography, intravenous urography, antegrade pyelography, and diethylene triamine pentaacetic acid renography that confirmed no obstacle in all 5 cases of pelviureteric stricture with a follow-up of 4 ± 2.9 years and in 4 cases of ureterovesical junction with a follow-up of 4.7 ± 2.8 years. Three unsuccessful results were reported: in 2 cases, the guide wire could not be advanced over the stenotic ureterovesical junction and in 1 case an early restenosis occurred that eventually required surgery. CONCLUSIONS: Although the main treatment of the postoperative ureteral strictures is surgical, the percutaneous antegrade balloon dilation seems to be an alternative to surgery with a low morbidity rate and short hospitalization period.

Prenatal urinoma related to ureteropelvic junction obstruction: poor prognosis of the affected kidney.

OBJECTIVES: We evaluated functional outcome of kidneys with fetal urinoma associated to ureteropelvic junction obstruction. METHODS: We retrospectively reviewed 5 cases of antenatally diagnosed urinoma associated to hydronephrosis. Prenatal imaging work-up consisted of ultrasound (US) and magnetic resonance (MR) examination. Differential renal function was assessed postnatally with renal scintigraphy. RESULTS: Four male fetuses and 1 female fetuse presented with urinoma with hydronephrosis diagnosed by US at 24-25 weeks gestational age and confirmed by MRI examination at 28-29 weeks gestational age. On postnatal US, urinoma had disappeared in 4 cases. Parenchyma of the affected kidney was poorly differentiated in all cases. Ureteropelvic junction obstruction was confirmed in the 5 cases. Renal scintigraphy revealed poor functioning kidney (1%) in 2 cases, no function in 1 case, and impaired function in 2 cases (17%). CONCLUSIONS: Association of upper urinary tract dilatation caused by ureteropelvic junction obstruction and prenatally diagnosed urinoma may suggest a poor ipsilateral functioning kidney.

Balloon sphincteroplasty in the management of choledocholithiasis in a 10-week-old infant.

The management of common bile duct stones in neonates has not been clearly fully standardized, although percutaneous image-guided washing of the bile duct is generally adopted as the first-line treatment. We report the case of a 10-week-old infant with a choledocholithiasis in whom anterograde sphincter balloon dilation was achieved by combining image-guided access and flexible gastroduodenoscopy. This mini-invasive strategy may be an alternative to surgery in cases of impacted choledocholithiasis resistant to bile duct washing.

[Value of low uretero-ureterostomy in 5 cases of functional ectopic ureter on duplicated collecting system in children. Conservative management of ectopic ureter]. / Uretère ectopique fonctionnel sur système urinaire double chez l'enfant: intérêt de l'urétéro-urétérostomie basse a propos de 5 cas. Traitement conservateur des uretères ectopiques.

INTRODUCTION: The objective of this study was to analyse the postoperative results of conservative management of functional ectopic ureter on duplicated collecting system by low uretero-ureterostomy in children. MATERIALS AND METHOD: The medical records of children with duplicated collecting system and a functional ectopic ureter admitted to our department between 2001 and 2005 were reviewed. A postnatal radiological assessment comprising urinary tract ultrasound, retrograde cystography, intravenous urography and DMSA renal scintigraphy was performed. In each patient, the operative technique consisted of low uretero-ureterostomy with end-to-side uretero-ureteric anastomosis. RESULTS: This series comprised 3 girls and 2 boys. The ectopic ureter was located on the left side in all patients. Postnatal ultrasound confirmed the presence of dilatation of the upper collecting system and the retrovesical ureter estimated at 10 +/- 2 mm in 3 cases; in one case, dilatation of the upper collecting system was estimated at 25 mm and the retrovesical ureter was estimated at 16 +/- 2 mm; in the last patient, dilatation of the upper collecting system above the retrovesical ureter was estimated at 6.5 mm. Mean renal function of the upper pole (DMSA) was 23 +/- 7%. The mean age at the time of the operation was 8 months. The immediate postoperative course was uneventful in all patients. Mean follow-up was 38 months (range: 18 months to 4.5 years). All children were asymptomatic and a reduction of collecting system and ureteric dilatation was confirmed on follow-up ultrasound at 10 months in all patients. CONCLUSION: Low uretero-ureteric anastomosis is an alternative to high collecting system anastomosis or ureteric reimplantation in the presence of a functional ectopic ureter on duplicated collecting system in children.

Atypical aphallia.

Aphallia is a rare genital anomaly poorly reported in literature. The authors describe an atypical case of aphallia characterized by ultrasound and magnetic resonance imaging findings of atrophic cavernous corpus associated with anterior urethral opening. Aphallia is a complex malformation, featuring various anomalies which should be assessed before electing a specific treatment.

Multicystic dysplastic kidney with ipsilateral abnormalities of genitourinary tract: experience in children.

OBJECTIVES: To investigate the incidence, nature, and management of associated ipsilateral genitourinary malformations in children with multicystic dysplastic kidney (MCDK). METHODS: In this retrospective study, we analyzed the medical records and imaging studies of 93 patients with MCDK. Patients underwent ultrasonography, voiding cystourethrography, intravenous urography, and radionuclide renal imaging studies during their first month of age. RESULTS: A diagnosis of MCDK associated with malformation of the ipsilateral internal genitalia was confirmed in 11 patients after birth investigations of prenatal MCDK. Three were diagnosed at 1, 12, and 14 years of age because of epididymitis, pelvic pain associated with amenorrhea, and accidentally during lumbar pain assessment, respectively. The male/female sex ratio was 10:4. The left side was involved in 9 patients. We had 3 cases of Gartner duct persistence, 6 of cystic retrovesical and laterovesical masses with vanishing MCDK, 4 of cystic retrovesical or laterovesical masses with compressive MCDK, and 1 of a blind-ending hemivagina. Nine patients were periodically observed, and four underwent nephroureterectomy. All patients underwent 6-month follow-up examinations with ultrasonography (mean follow-up 6.54 years, range 36 months to 14 years). CONCLUSIONS: Of the 93 patients with MCDK, 14 (15%) had malformations of the ipsilateral internal genitalia. Persistence of seminal cysts in boys and Gartner ducts were encountered even if the MCDK had involuted. These results suggest that follow-up of patients with MCDK should be performed until the end of puberty to detect genitourinary malformations.

[Neonatal uro-haematoma secondary to posterior urethral valve]. / Uro-hématome néonatal secondaire à une valve de l'urètre postérieur.

The authors report the case of a male neonate presenting with a heterogeneous cystic mass in the right renal area with no identifiable renal unit. The diagnosis of right perirenal extracapsular uro-haematoma secondary to posterior urethral valve was proposed on the basis of the antenatal clinical history. The late discovery at 39 weeks of amenorrhoea (pregnancy not followed) of a foetus with trabeculated bladder associated with left ureteropelvic dilatation, and a cystic mass occupying the right lumbar fossa was highly suggestive of right urinoma secondary an infravesical obstacle. In this case, vaginal delivery was probably responsible for intracystic bleeding of the urinoma. The postnatal morphological assessment including ultrasound, retrograde cystourethrography and MRI confirmed the presence of a posterior urethral valve complicated by right uro-haematoma displacing the right kidney and left ureteropelvic dilatation. This lesions corresponds to a collection of urine and blood in the right perirenal space due to caliceal or parenchymal perforation secondary to raised urinary tract pressure above an obstacle. Subcapsular or extracapsular perirenal urinoma, sometimes detected in neonates with posterior urethral valve, reflects protective decompression of the urinary tract (safety valve effect), as in our case. The neonatal diagnosis of a heterogeneous renal cystic mass is sometimes difficult (polycystic renal dysplasia, cystic lymphangioma, cystic neuroblastoma, adrenal haematoma). In some cases, the antenatal history may suggest the diagnosis of perirenal urohaematoma secondary to infravesical obstruction.

[Preliminary results of endoscopic treatment of vesicoureteric reflux in children. Prospective comparative study of Deflux vs. Coaptite]. / Résultats préliminaires: traitement endoscopique du reflux vésico-urétéral chez l'enfant: etude prospective comparative Deflux/Coaptite.

INTRODUCTION: The objective of this study is to compare the short-term and medium-term efficacy of Coaptite versus Deflux in the endoscopic treatment of vesicoureteric reflux in children. MATERIALS AND METHODS: Over a period of 24 months, a cohort of 31 children with 44 refluxing vesicoureteric units were treated alternately by either Deflux implants (Group I, 24 refluxing units) or Coaptite implants (Group II, 20 refluxing units). This series comprised 40 solitary ureteric systems and 2 complete duplications. The two groups were comparable in terms of age at the time of the operation and distribution of severity of reflux. The quantity of product injected was identical in the 2 groups: 0.5 cc per ureter. All patients in group II received only one injection, while 2 Deflux injections were necessary in 2 patients and 3 injections were necessary in 1 patient in group I. All children were reviewed by ultrasound and retrograde cystography 3 months after the operation. RESULTS: Reflux, regardless of grade, resolved in 70% of cases in the 2 groups (no significant difference). The cure rate was 75% for grade II and III in group I and 62.5% (grade II) and 70% (grade III) in group II, with no significant difference between the 2 groups. Two cases of grade IV reflux were cured after a single implantation of Coaptite in 1 case and Deflux in the other case. Complications, such as ureteric stasis or haemorrhage at the injection site, were not observed. CONCLUSION: Although the efficacy of endoscopic treatment has now been clearly established, the choice of material to be injected is still controversial. This preliminary study did not reveal any significant difference in terms of cure rate, regardless of grade, between patients treated with Coaptite or Deflux. The medium-term and especially the long-term morbidity of these products still needs to be evaluated.

Small bowel obstruction caused by congenital mesocolic hernia: case report.

Transmesocolic hernias are extremely rare. Their exact incidence is still unknown. A strangulated hernia through a mesocolic opening is a rare operative finding. Preoperative diagnosis still is difficult in spite of imaging techniques currently available. This is the case of a 4-month-old boy with transmesocolic internal hernia and coincident intestinal malrotation and volvulus of small bowel.

[An example of diphallia in children]. / Un exemple de diphallia chez l'enfant.

Diphallus is a rare malformation with an estimated frequency of one case per five million births. The authors report a case of incomplete diphallus associated with glanular hypospadias in a 2-month-old. There are various more or less severe anatomical and clinical forms of this urogenital abnormality. The therapeutic management is very variable, ranging from simple resection of the supernumerary penis, as in our case, to complex reconstruction when the anomaly is associated with severe malformations (oesophageal atresia, anorectal malformation, spinal malformation).

Fetal bladder rupture due to anteriorurethral valves.

A rupture of the fetal bladder that resulted in urinary ascites has rarely been reported in published studies. We present the first case of a spontaneous rupture of the fetal bladder, due to an anterior urethral valve, in which the diagnosis was suspected prenatally by means of Doppler ultrasonography and was confirmed postnatally.

[Urinary tuberculosis in children. Report of a severe form with a followup of 19 years]. / La tuberculose urinaire chez l'enfant. A propos d'une forme sévère avec 19 ans de recul.

The authors report the case of a 13-year-old migrant girl with urinary tuberculosis, presenting with urinary tract infection, severe frequency and unexplained fever. Intravenous urography demonstrated a non-functioning left kidney and a small fibrotic bladder. Retrograde cystography revealed stage 4 right vesicoureteric reflux. The presence of the Koch bacillus was identified on urine culture. Medical treatment by Rifampicin and Isoniazid was instituted for 18 months. Surgical management, consisting of left nephroureterectomy, subtotal cystectomy with augmentation enterocystoplasty and resection of the pelvic part of the right ureter with ureterointestinal reimplantation, was performed. With a follow-up of 19 years, this patient has normal renal function and no urinary disorders. This case recalls the reality of urinary tuberculosis in children and confirms its insidious nature. The destructive course of urinary tuberculosis may require major urinary tract reconstruction, as in our case.

Major renal injuries in children: the real incidence of kidney loss.

BACKGROUND: The prognosis of major renal injuries appears to have improved since a conservative approach has been promoted. The overall prognosis might be less optimistic if all cases are pooled, whatever the type of initial injury (parenchymal or vascular) or the type of treatment (operative or conservative). METHODS: The records of 32 children were reviewed. Six patients had a vascular injury, and 26 had a parenchymal injury. Ten were operated on within one month after trauma, and 3 (including one previously operated) underwent delayed surgery for sequelae. Parenchymal loss from atrophy was estimated on echography, computerized tomography, or radionuclide scintigraphy. RESULTS: Fourteen patients (44%) recovered a functioning kidney. Nine (28%: all 6 patients with vascular injury and 3 with parenchymal trauma) lost their kidney from nephrectomy (n = 3) or atrophy (n = 6). Seven patients had severe or moderate sequelae from either partial nephrectomy or partial atrophy. Two had a minimal loss from polar hypotrophy. The incidence of kidney loss or sequelae was 56% (18 of 32 patients). CONCLUSION: In keeping with the literature data provided all cases are analyzed, at least one out of 4 kidneys will be lost or severely damaged after major kidney trauma in children.

[Urethral sagittal duplication in children. Report of 7 cases]. / Duplications sagittales de l'urètre chez l'enfant. A propos de 7 observations.

UNLABELLED: Urethral duplication is a rare disease with various clinical expressions and treatments. The authors report 7 cases. MATERIAL AND METHODS: The authors retrospectively review 7 children with sagittal duplication of the urethra seen between 1995 and 2000. All children were investigated by ultrasonography of the urinary tract, retrograde cystography and voiding cystourethrography. RESULT: Except for one case of neonatal discovery, the mean age at diagnosis in the other six children was 4 years. In one case, the malformation consisted of blind incomplete urethral duplication (type I B) and urethroscopy confirmed the presence of an orifice distal to the urethral cap; symptomatic treatment was successful in this patient. Two children with complete urethral duplication with epispadias and two bladder orifices (type II A1) were successfully treated by resection of the supernumerary urethra. One child presented an U-shaped duplication (type II A2 "Y"). Unsuccessful opacification of the accessory urethra prior to its resection induced sclerosis and no flow with a follow-up of 24 months. Penile surgery was performed in two children with incomplete urethral duplication with only one vesical orifice (type II A2) transforming duplex urethra into distal bifid urethra after resection of the accessory urethra and its epispadic meatus. One child had a suspended duplication of the urethra with only one glandular meatus (type II B2) and a single vesical orifice. He was treated by endoscopic section of the urethral membrane separating the two urethras before their fusion in the bulbar urethra. With a mean follow-up of 36 months (range: 6 months to 4 years) no infectious or voiding complications were observed in these 7 children. CONCLUSION: Sagittal duplication of the urethra is a rare disease requiring precise anatomical and functional assessment. The ideal treatment for symptomatic forms is resection of the duplication without impairing sphincter function.