Posterior urethral valve in thai boys.

BACKGROUND: Posterior urethral valve (PUV) is the most common congenital bladder outlet obstruction in boys, causing renal damage beginning in utero. There are scarce data from Thailand regarding the long-term outcomes of PUV in boys, thus the aim of this study was to examine the presentation, clinical course, complications, outcomes and renal survival in PUV boys. METHODS: We reviewed the medical records of PUV boys treated at the Pediatric Nephrology Clinic, Prince of Songkla University, Thailand, over a 30-year-period. RESULTS: Seventy-seven PUV boys were identified, with a median age at diagnosis of 4.8 months. The most common presentations were urinary tract infection (UTI), poor urine stream and urinary dribbling in 26 (33.8%), 19 (24.7%) and 11 (14.3%) boys, respectively. Renal ultrasound results in 70 boys showed 8 (11.4%) unilateral and 56 (80%) bilateral hydronephroses. Of 72 voiding cystourethrograms, 18 (25.0%) showed unilateral and 22 (30.6%) bilateral vesicoureteral refluxes. 99mTc dimercaptosuccinic acid renal scans in 30 boys showed 12 (40%) unilateral and 8 (26.7%) bilateral renal damage. Fifty-nine (76.6%) boys had 149 UTIs; 42 (54.4%) had recurrent UTI. Forty-eight boys had valve ablation at the median age of 30.3 months. 22 boys (28.6%) developed chronic kidney disease (CKD) at a median age of 15.0 years. CONCLUSION: Of 77 PUV Thai boys, UTI was the most common presentation. Recurrence of UTI and CKD was the most common consequence. Lifelong follow-up for renal and bladder functions is essential for all PUV patients.

Congenital Anterior Urethral Diverticulum in Identical Twins.

Congenital anterior urethral diverticulum (CAUD) is an uncommon abnormality of the male urethra. In the literature, cases of CAUD affecting both children within a set of identical twins or presenting concomitantly with another urethral condition are exceedingly rare. We describe 2 cases of CAUD in identical twins: a pair of newborns in which Twin 1A and Twin 1B both present with CAUD, and a second pair of newborns in which only Twin 2A presents with CAUD along with a partial collateral urethral duplication. In doing so, we aim to add to the incomplete literature on the embryological development of CAUD.

Robotic-assisted laparoscopic repair of a congenital rectourethral fistula with duplicated urethra.

OBJECTIVE: To describe our experience with robot-assisted laparoscopic transperitoneal repair of a congenital rectourethral fistula in a pediatric patient with a urethral duplication. METHODS: The patient is a 2-year-old male with a past medical history of Tetralogy of Fallot presenting with a febrile urinary tract infection (UTI). He was diagnosed with urethral duplication and a rectourethral fistula by voiding cystourethrogram (VCUG). The parents were counseled on various options and agreed to proceed with a robotic repair. RESULTS: Robotic-assisted transperitoneal rectourethral fistula repair was performed. The procedure time was 229 min with an estimated blood loss (EBL) of 15 mL. His postoperative course was unremarkable. At his 2-week follow-up, the urethral catheter was removed and the patient was voiding normally and having normal bowel movements. CONCLUSION: Congenital rectourethral fistula with urethral duplication is a rare anomaly with only a few reports in the literature. Pediatric robotic-assisted transperitoneal rectourethral fistula repair is a technically feasible approach in infants with minimal morbidity that allows for excellent visualization and avoids open repair.

Misdiagnosis of Congenital Posterior Urethroperineal Fistula and Comparison With Urethral Duplications and Rectourethral Fistula.

Congenital posterior urethroperineal fistula (CUPF) is a urothelium-lined tract between the posterior urethra and perineum. This condition is rare and has been proposed to be a urethral duplication variant. A case of CUPF that was misdiagnosed and surgically treated as a rectourethral fistula is presented. The clinical presentation, diagnosis, and treatment of CUPF are discussed and compared with those of Y-type urethral duplications and H-type rectourethral fistulas.

Paraurethral Cyst in a Newborn: Case Report and Discussion.

BACKGROUND: Paraurethral cyst is a rare cause of interlabial mass in neonates with an incidence of 1 in every 2000-7000 live births and represents less than 0.5% of congenital malformations of the urinary tract. CASE: We report the case of a paraurethral cyst in a neonate, which regressed spontaneously during follow-up without complications. SUMMARY AND CONCLUSION: Paraurethral cyst should be considered in the differential diagnosis of interlabial masses in newborns. Because of the high probability of spontaneous regression, expectant management appears to represent the management of choice.

Single-Incision Laparoscopic Versus Conventional Laparoscopic Surgery for Rectobladderneck and Rectoprostatic Anorectal Malformations.

Background: We compared the safety and feasibility of single-incision laparoscopic surgery (SILS) and conventional laparoscopic-assisted anorectoplasty (CLAARP). Materials and Methods: We compared sacral ratio, age, operation time, blood loss, and length of hospital stay after the operation, and complications in 105 patients with rectobladderneck and rectourethral fistula who underwent operations from January 2010 to May 2014 in our institution (SILS, n = 51; CLAARP, n = 54). The Krickenbeck classification was used to evaluate the anorectal functions. Results: The mean operative time in the SILS group (113.5 ± 12.7 minutes) was significantly shorter than that in the CLAARP group (126.2 ± 10.2 minutes) (P = .003). The intraoperative blood loss did not differ between groups (P = .75). There were no significant differences in the time needed to resume diet and length of hospital stay after the operation. The overall incidence rate of postoperative complications was similar (7.8% versus 7.4%, P = 1.00). There were no significant differences in voluntary bowel movement, soiling, and constipation between the two groups. No injuries to vessels, urethral or vas deferens occurred in either group. No mortality or morbidity of wound infection, rectal retraction, recurrent fistula, urethral diverticulum, or anal stenosis were encountered in our study. Conclusions: SILS is a feasible and safe technique compared with CLAARP in terms of surgical outcomes.

Long-term renal outcome in infants with congenital lower urinary tract obstruction.

INTRODUCTION: Congenital Lower Urinary Tract Obstructions (LUTO) is a heterogeneous group of diseases in which urine elimination is obstructed at the level of bladder neck or urethra. The aim of the study is to evaluate the long-term renal outcome of patients suffering of congenital LUTO. PATIENTS AND METHOD: We retrospectively reviewed patients with congenital LUTO. All patients had at least 1 year follow-up. Data on surgery, renal imaging and Schwartz estimate creatinine clearance were collected. Incidence of Chronic Renal Disease (CRD) is presented with Kaplan-Meier method. RESULTS: 40 patients were included, 23 patients with Posterior Urethral Valve (PUVs) and 17 patients with other aetiologies: anterior urethral valve (2), urethral atresia (2), urethral stenosis (2), cloacal malformations (2), obstructive ureterocele (1), bladder trigone malformation (1) and neonatal bladder-sphincter dysfunction without neurological abnormalities (7). Incidence of CRD at age 10 years was 37% in congenital LUTO, 42% in PUVs and 30% in other aetiologies, and was significantly higher in PUVs (P=0.032). Renal prognosis was significantly worsened by discover of retentional bladder wall changes in initial cystoscopy, and by loss of parenchymal differentiation or cortical microcysts in first ultrasonography. The use of urinary diversion was significantly higher in LUTO of other aetiologies. CONCLUSION: A high incidence of CRD is observed in patients with congenital LUTO, significantly higher in patients with PUV. LUTO of other aetiologies require step by step surgical management and higher use of urinary diversion. Precise initial evaluation in cystoscopy and ultrasonography is required and participate to evaluate future renal outcome. LEVEL OF INCIDENCE: 4.

Suspected congenital urethral diverticulum in a dog.

A 2-year-old neutered male dog with a history of urinary incontinence, recurrent urinary tract infections, and unilateral cryptorchidism was presented with an acute onset of perineal swelling. Urinary contrast studies revealed a urothelial lined structure in the perineum. Surgical resection of 80% to 90% of this structure, suspected to be a congenital urethral diverticulum, was successful.

Diverticule urétral congénital soupçonné chez un chien. Un chien mâle stérilisé âgé de 2 ans ayant une anamnèse d'incontinence urinaire, d'infections des voies urinaires récurrentes et de cryptorchidie unilatérale a été présenté suite à l'apparition aiguë d'enflure périnéale. Des épreuves de contraste urinaire ont révélé une structure urothéliale recouverte dans le périnée. Une résection chirurgicale de 80 % à 90 % de cette structure, soupçonnée d'être un diverticule urétral congénital, a été réalisée avec succès.(Traduit par Isabelle Vallières).

Congenital anterior urethrocutaneous fistula: A systematic review.

Congenital anterior urethrocutaneous fistula (CAUF) is a rare anomaly characterized by fistulization of penile urethra to skin. It's usually seen as an isolated deformity or may accompany genitourinary or anorectal malformations. We aim to define the common properties of patients mentioned in literatures by systematic review. A comprehensive search of PubMed, Embase, Web of Science, and Cochrane Library was performed including cross-referencing independently by two assessors. Selections were restricted to human studies in English. Based on the systematic review, 63 patients in 34 articles were included in the study. Most common fistula site was subcoronal in 29 (46.0%) patients. Chordee was in 8 (14.5%) and associated genitourinary anomaly was detected in 19 (30.2%) of patients. Fistula recurrence ratio was 6/59 (11.3%) using different surgical techniques and 3/6 was closed spontaneously. CAUF is frequently located in subcoronal level and usually an intact urethra distal to it. Success rates are high with the principles of hypospadias surgery.

[IIA2-Y-type urethral duplication]. / Duplicité de l'urètre de type IIA2 en "Y".

Urethral duplications are extremely rare congenital malformations. The most used classification is that of Effmann and Lebowitz, describing 6 types of urethral duplications. The under type IIA2-Y is one of the most rare duplications corresponding to duplicated urethra originating from the bladder neck and extending toward the ectopic perineal or anal insertion. We here report the case of a 32 year patient, with no previous personal history, suffering from urinary leakage during and after urination since childhood. Clinical examination showed a bottleneck at the level to the perineum initially suggesting urethral fistula. However, the absence of fistula-associated urinary disorders as well as urinary leakage since childhood refuted this assumption. The patient underwent Micturating Retrograde Urethrocistography after perineal orifice catheterization confirming well systematized tract. Surgical exploration was based on excision of the duplicate urethra after hydrophilic catheterization by inserting hydrophilic guidewire up to its anastomosis at the level of the prostate. The examination of the surgical specimen confirmed the diagnosis of supernumerary urethra.

Laparoscopic dissection and division of distal fistula in boys with rectourethral fistula.

BACKGROUND: Congenital rectourethral fistula (RUF) is the most common form of anorectal malformations found in boys. The aim of this study is to review our experience with dissection and division of distal fistula using laparoscopic surgery in the management of RUF, especially rectourethral bulbar fistula. METHODS: One hundred and two consecutive boys with congenital RUF who underwent conventional or single-incision laparoscopic surgery between July 2008 and June 2015 were enrolled in the study. The dissection of the distal fistula was performed along submucosal layer to a level 0.5 cm proximal to the urethra. Rectal mucosa of the fistula was dissected to the distal most point and completely transected flush with the posterior urethra. The residual muscular cuff was ligated with Hem-o-Lock clip or 5-0 PDS suture. Voiding cystourethrography and pelvic magnetic resonance imaging were performed at 3 mo, 6 mo, and 1 y postoperatively. RESULTS: All patients successfully underwent laparoscopic surgery without conversion. The mean age at the time of operation was 4.3 ± 2.9 mo. The operative times for the rectoprostatic fistula and rectobulbar fistula were similar (118.2 versus 119.4 min, P = 0.082). There was no significant difference in average operative time between conventional laparoscopic surgery group and single-incision laparoscopic surgery group (118.8 versus 119.1 min, P = 0.281). There was no injury to the urethra or vas deferens. The urethral catheter was removed on postoperative day 10. All patients were followed up. The median follow-up period was 3.3 ± 1.8 y. No recurrent fistula or urethral diverticulum was detected on voiding cystourethrography and pelvic MRI at 1 y. CONCLUSIONS: Submucosal dissection and division of distal fistula using a laparoscopic approach is safe, feasible, and effective for congenital RUF, especially bulbar fistula, in boys.

Rare association of congenital penile urethrocutaneous fistula with Y-type urethral duplication.

A 24-year-old man presented with symptoms of difficulty in passage of urine, mixed with gas and stool from penoscrotal junction since birth. He had a history of surgery (posterior sagittal anorectoplasty) for imperforate anus in first week of his life. On physical examination, external meatus was adequate with small 5×5 mm fistulous opening noted at penoscrotal junction with normal anal tone and opening. On evaluation with retrograde urethrography, voiding cystourethrogram, cystoscopy and barium enema, he was found to have penile urethrocutaneous fistula of 0.5×0.5 cm with Y-type incomplete urethral duplication. Urethroanal fistula (Y-type urethral duplication) was of small size (<5 mm), so closed primarily through perineal route with interposition of ischiorectal fat. Penile urethrocutaneous fistula (4 mm) too was closed primarily and covered with tunica vaginalis flap in single sitting. It has been 6 months since surgery and the patient is fully continent and voiding well with no urinary or faecal leak.

Long-term Follow-up of Median Raphe Cysts and Parameatal Urethral Cysts in Male Children.

OBJECTIVE: To clarify the long-term outcome of median raphe cysts (MRCs) and parameatal urethral cysts (PUCs) in males, the clinical, anatomical, and histological findings of these cysts are described, along with treatment and follow-up. MATERIALS AND METHODS: Forty-six children diagnosed with PUCs and 23 children with MRCs at our institute during the past 14 years were retrospectively reviewed. The indications for surgery were symptoms or family preference. Telephone interviews of the parents or patients were conducted to ascertain the current condition of the cyst at a median age of 12.2 years. Fifty-nine children (85.5%) agreed to the telephone interview. RESULTS: Median age at first presentation was 41 months; 81% of children were asymptomatic. The main symptoms were voiding symptoms with PUCs and pain of the ruptured cyst in MRCs. A total of 41 (59%) children underwent complete excision at a median age of 58 months. No recurrences were encountered with surgery in all children. Of 28 children managed nonoperatively, 22 agreed to an interview. All children (95.5%), except for 1, remained free of symptoms. Six cysts showed spontaneous resolution at a median age of 24 months, and 15 improved or stabilized. No one was concerned about the genital appearance and opted for surgical intervention. CONCLUSION: Complete excision of the cyst is a durable procedure without recurrences in childhood. In the nonoperative management, several cysts may continue to be free of symptoms during childhood, and some cysts may resolve spontaneously during the first 2 years.

Fate of males with urethral "Y-duplication": 40-year long follow-up in 8 patients.

PURPOSE: The spectrum of male urethral duplication is heterogeneous and it includes the Y-duplication. The malformation is rare and there is only a few case series reported in the literature. The management of Y-forms remains challenging for the surgeon and the long-term follow-up is still scarcely investigated. We report our 40-year experience in the management of patients with Y-duplication. MATERIALS AND METHODS: We conducted a restrospective analysis collecting information of patients with urethral Y-duplication treated at our department from April 1975 to April 2015. We investigated long-term effects of surgery by using a questionnaire. RESULTS: Ten male patients with Y-duplication came to our attention. One was treated conservatively, seven underwent surgery and two were lost. Surgery consisted of removal of the ectopic branch (via perineal or ASTRA/anterior sagittal trans-rectal approach approach) and reconstruction of the orthotopic urethra. Post-operative complications included stenosis and infections. Long-term results are influenced by associated anomalies and significant problems (incontinence, urinary tract infections and orchiepididimitis) have been reported. CONCLUSIONS: Y-duplication (or λ-duplication, as we prefer calling it) is a particular form of urethral duplication. The management of patients should be based upon the identification of the functional channel. The removal of the ectopic channel with ASTRA approach is safe and feasible. On the other hand, the reconstruction of the anterior urethra (when steno-atresic) is more challenging and justifies the need for many procedures. The P.A.D.U.A. (progressive augmentation by dilating the anterior urethra) technique was not effective. Skin tube grafts were responsible for infections ("hairy urethra"). BMFG (bladder mucosa free graft) urethroplasty is a good alternative, although associated with well-known complications. Associated anomalies influence long-term outcomes. Clinical study with type IV level of evidence.

Novel Plicating "Wrap" Technique for Treating Anterior Urethral Ballooning.

OBJECTIVE: To describe an original approach, inspired by the technique proposed by Kalicinsky, which has the advantages of not requiring opening of the urethra, modeling the urethra to a suitable caliber without urethral anastomosis and providing the urethra with a solid and stretchable ventral support. MATERIALS AND METHODS: We operated on 3 cases of anterior urethral ballooning between November 2013 and June 2014. Retrograde and micturition urethrography ruled out any urethral anastomosis and disclosed the ballooning. A 10-F silicone catheter was placed in all 3 cases with the purpose of monitoring the tailoring. Complete dissection of the urethral "sac" was carried out regarding circumference and length, and the result was a normal urethra with respect to both the penoscrotal angle and the glans. Urethral tapering was then undertaken with a continuous 5-0 polyglactin suture, which produced a straight urethra just a little wider than the previously placed monitoring catheter. The remnants of the dilated urethra were wrapped around the urethra with the aid of 2 traction stitches. Urethral tailoring was completed with a longitudinal suture that fixed the wrap. The urethral catheter was left 1 week after. RESULTS: The children urinated without problems and with a good micturition jet that remained unaltered over the ensuing months. CONCLUSION: A novel technique for treating a rather infrequent pathology has been described. The reported technique is simple and reproducible, and our 3 cases had excellent cosmetic and functional results.

Practical and functional classification of the double urethra: A variable, complex and fascinating malformation observed in 20 patients.

INTRODUCTION: Urethral duplication is a rare and variable malformation of the urinary tract, with non-univocal and complex management. In addition, different classification schemes have been proposed, but none have considered all the possible variants. OBJECTIVE: To report experience with the management of 20 urethral duplication patients and propose a classification of this anomaly. MATERIALS AND METHODS: A retrospective analysis collecting information regarding 20 patients (Table) with urethral duplication treated at a single institution over the past 40 years. Three females and 17 males were treated: two had vesico-urethral duplication, eight had urethral duplication with a single bladder, and eight had 'λ' duplication. RESULTS: Immediate postoperative complications included urethral dehiescence (n = 1) and urethral stenosis (n = 2). The progressive augmentation by dilating the urethra (PADUA) technique was ineffective. During follow-up, the following were recorded: urinary incontinence (n = 2), urinary tract infection (n = 3), hypertension (n = 3) and erectile dysfunction (n = 1). All patients were satisfied with the aesthetic result. DISCUSSION: Urethral duplication is a rare anomaly with male preponderance. Four types of duplication were described, on the basis of anatomy and management: vesical and urethral duplication (type 1), urethral duplication with single bladder (type 2), 'λ-type' duplication (type 3) and 'miscellanea' (multiple urethral channels, spindle urethra, other female forms, type 4). A full description of the malformation and surgical approach was given for each type. The advantages of the classification were compared with the literature. CONCLUSIONS: The proposed classification should be a useful tool, based on the required surgical approach, even if surgery should be tailored to the patient. It is important to restore the anatomy and achieve urinary continence. Surgery can be challenging and a multi-step process, especially in cases of 'λ' duplications.

Posterior urethral valves and vesicoureteral reflux: can prenatal ultrasonography distinguish between these two conditions in male fetuses?

OBJECTIVE: The objective of the study was to evaluate prenatal sonographic signs that distinguish male fetuses with posterior urethral valves (PUV) from those with vesicoureteral reflux (VUR). METHODS: Prenatal data were retrospectively retrieved from all consecutive women delivering between 2003 and 2012 of a male newborn with a postnatal diagnosis of PUV or VUR. Prenatal parameters included fetal bladder characteristics, identification of a dilated posterior urethra, and change in shape and size in the fetal renal pelvis or ureter. RESULTS: One hundred thirty-six women gave birth to a male newborn with a postnatal diagnosis of PUV (n = 49) or VUR (n = 87). The presence of posterior urethral dilatation [21 (42.9%) fetuses versus 0 (0%), p = 0.000], a thickened fetal bladder wall [37 (75.5%) vs 4 (4.6%), p = 0.000], and anhydramnios [14 (28.6%) vs 0, p = 0.000] were strongly associated with the postnatal diagnosis of PUV. Change in shape and size in the fetal renal pelvis or ureter was observed in 15 (17.2%) of 87 children with VUR versus 1 (2.0%) out of 49 with PUV (p = 0.010). CONCLUSIONS: Prenatal ultrasound may differentiate with reasonable accuracy male fetuses with a postnatal diagnosis of PUV from those with VUR. © 2016 John Wiley & Sons, Ltd.