Outcome of boys with posterior urethral valves from a single tertiary hospital in Singapore.

INTRODUCTION: Posterior urethral valve (PUV) is the most common congenital cause of bladder outflow obstruction in male infants. Despite timely treatment, renal damage can still occur in the long-term leading to chronic kidney disease (CKD). METHODS: A retrospective review of all PUV patients in a single tertiary institution between April 1998 and July 2019 was conducted to analyze their presentations, management and outcomes. Long-term renal function, radiologic scans and somatic growth were evaluated. RESULTS: A total of 16 patients were included in this study. Two patients who defaulted all follow-ups were excluded. Seven patients (43.7%) presented in the antenatal period; four patients (25%) presented in the neonatal period and five patients (31.3%) presented in the post-neonatal period. Primary transurethral fulguration of valves was done in 13 patients, while three had vesicostomies as the primary procedure. Three patients had associated anterior urethral valves (AUV), which were treated endoscopically. Nine boys had additional procedures for diversion and undiversion, VUR, non-functioning kidney and clean intermittent catheterization. Ten patients had urodynamic studies performed, of which eight patients received anticholinergic therapy. Eleven patients had DMSA scans, of which three patients had a normal study and eight patients showed unilateral reduced function. Four patients were diagnosed with CKD on long-term follow-up duration over 5 years. All patients were shown to have good somatic growth. CONCLUSION: Patients with PUV can suffer from complications despite primary treatment. In our small cohort, a quarter of our patients developed CKD on follow-up. Thus, patients need long-term follow-up to optimize bladder and renal function.

Müllerian agenesis in the presence of anorectal malformations in female newborns: a diagnostic challenge.

Rectovestibular fistula is the most common type of anomaly found in a female newborn with anorectal malformation. However, when the baby is found to have two orifices in the introitus, rectovaginal fistula is much less common and suspected. The rare differential diagnosis of Müllerian agenesis, a condition in which the rectum shifts anteriorly and the vagina is absent, is seldom considered. In many cases, the diagnosis of Müllerian agenesis is made only during definitive anorectoplasty. In view of its impact on management, a proper examination under anaesthesia, imaging studies and a diagnostic laparoscopy may be required to confirm the presence or absence of Müllerian structures in such patients. We herein describe a patient with the rare coexistence of VACTERL association and Müllerian agenesis, and discuss the management of anorectal malformations in female patients with Müllerian agenesis.

Hepaticoduodenostomy versus hepaticojejunostomy after resection of choledochal cyst: a systematic review and meta-analysis.

BACKGROUND: Excision has been established as a standard management practice for choledochal cysts in the last few decades. The two most commonly performed methods of reconstruction after excision are hepaticoduodenostomy (HD) and Roux-en-Y hepaticojejunostomy (HJ), of which the HJ is favored by most surgeons. Evidence concerning the optimal method of reconstruction is, however, sparse. MATERIALS AND METHODS: Studies comparing outcomes from HD and HJ after choledochal cyst excision were identified by searching Medline, Ovid, Search Medica, Elsevier Clinicalkey, Google Scholar and Cochrane library. Suitable studies were chosen and data extracted for meta-analysis. Outcomes evaluated included operative time, hospital stay and incidence of postoperative bile leak, cholangitis, reflux/gastritis, anastomotic stricture, bleeding, intestinal obstruction and re-operative rate. Pooled odds ratios (OR) were calculated for dichotomous variables; pooled mean differences (MD) were measured for continuous variables. RESULTS: Six retrospective studies were included in this meta-analysis, comprising a total of 679 patients, 412 of whom (60.7%) underwent HD, and the remainder, 267 (39.3%) underwent HJ. Although, HD group had slightly shorter hospital stay (MD: 0.30; 95% CI: -0.22-0.39; P < 0.00001) it showed a higher incidence of postoperative reflux/gastritis (OR: 0.08; 95% CI: -0.02-0.39; P = 0.002). However, the other outcomes such as bile leak, cholangitis, anastomotic stricture, bleeding, operative time, reoperation rate and adhesive intestinal obstruction did not differ between HD and HJ groups. CONCLUSIONS: HD shows higher postoperative reflux/gastritis than HJ but a shorter hospital stay. There are few good-quality studies that compare the outcomes from HD and HJ, meaning that caution should be exercised in the generalization of the results of this meta-analysis, which suggests HD to be comparable with HJ in terms of other complications, operative benefits and outcomes.

Embryogenesis of triple neural tube defects: sonic hedgehog--a key?

Neural tube closure is a complex spatio-temporal process. Multiple neural tube defects (NTDs) in a single patient are extremely rare. Only a few cases of multiple NTDs have been reported in the world literature, including less than 20 cases of double NTDs and 3 cases of triple NTDs. We report a fourth case of triple NTDs and review the literature regarding the embryogenesis. An effort is made to understand the theories of neural tube closure in light of recent advances in molecular biology and identification of the signaling pathways involved.

Association of angiotensin converting enzyme and angiotensin type 2 receptor gene polymorphisms with renal damage in posterior urethral valves.

OBJECTIVE: To examine the association with renal damage in patients with posterior urethral valves (PUV) of two renin-angiotensin system gene polymorphisms: angiotensin converting enzyme insertion/deletion (ACE I/D) and angiotensin type 2 receptor (AT2R A1332G), PATIENTS AND METHODS: In 120 patients with PUV, after stabilization, transurethral fulguration or a Blocksom vesicostomy was performed. Records were reviewed for age at diagnosis, biochemical renal function at diagnosis, results of urine cultures, voiding cystourethrograms, radiologic, sonographic and nuclear medicine scan findings, and follow-up data. ACE I/D genotypes were determined by the polymerase chain reaction using allele specific primers. RESULTS: The frequency of the ACE DD genotype was significantly higher in patients with chronic kidney disease (P=0.02) and renal scarring (P=0.05). These genotypes were also associated with a statistically higher incidence of vesicoureteral reflux, diurnal incontinence, proteinuria and hypertension. A significantly higher frequency of the AT2R GG genotype was found in PUV patients as compared to healthy unrelated control subjects (P=0.001), and in PUV patients with scarring (P=0.02). CONCLUSION: The ACE DD and AT2R GG genotypes are associated with chronic kidney disease and scarring in PUV patients. The GG genotype incidence is higher among PUV patients compared to the control population, and further studies in this area may help understanding of the genetic basis of PUV.

Comparative efficacy and safety of extended-release and instant-release tolterodine in children with neural tube defects having cystometric abnormalities.

AIM: To evaluate the comparative efficacy and safety of extended-release (ER) and instant-release (IR) tolterodine preparations in a pediatric population with neural tube defects having cystometric abnormalities. MATERIALS AND METHODS: Twenty-five patients with neural tube defects and a similar demographic profile underwent a routine hemogram, liver function tests, renal function tests, urine culture, X-ray lumbo-sacral spine, and renal and bladder ultrasound. Vesicoureteric reflux was diagnosed by micturating cystourethrogram under fluoroscopy. Dimercaptosuccinic acid renal scintigraphy was performed to study the presence or absence of renal scars. Patients were treated with tolterodine ER (Group I: 2mg once daily for 21 days) and tolterodine IR (Group II: 2mg twice daily for 21 day) in a cross-over study with a 10-day washout period between administrations. Evaluation was by subjective assessment, visual analog scale, urodynamic assessment and adverse drug reaction monitoring. RESULTS: There was ultrasound evidence of hydroureteronephrosis in 20% of the patients. One patient out of 25 had impaired renal function and eight patients had renal scarring on dimercaptosuccinic acid scans. Both forms of the drug increased the maximum cystometric bladder capacity, decreased detrusor leak pressures and increased compliance compared to pre-therapy levels (P=0.0001). Visual analog scale showed a significant clinical improvement with both ER and IR tolterodine. A significant increase in maximum bladder capacity in the group receiving IR tolterodine as compared to the ER preparation was noted (P=0.0001). The decrease in detrusor leak pressures and improvement in compliance were not significantly different between the groups. No adverse effects of hyperpyrexia, flushing or intolerance to outdoor temperatures, or dryness of mouth were observed in either group. No patient suffered from constipation. CONCLUSION: ER tolterodine 2mg once daily is as effective and well tolerated in children with neurogenic bladder as IR tolterodine 2mg twice a day. The latter was found to be more effective in terms of urodynamic parameters. ER formulation of tolterodine is less expensive and has the advantage of single dosage.

Endoscopic management of traumatic hepatobiliary injuries.

BACKGROUND: Non-surgical treatment has become the therapeutic method of choice in hemodynamically stable patients with liver trauma. There are a few reports of endoscopic management of traumatic hepatobiliary injuries in such patients; however, the optimal intervention is not known. METHODS: Twenty patients with traumatic hepatobiliary injuries from May 1997 to November 2005 were retrospectively evaluated. RESULTS: There were 18 male and two female patients with a mean age of 21.45 +/- 10.17 years (range 7-42 years). Seven patients were children. Patients presented 19.4 +/- 17.04 days following trauma. Computed tomography (CT) revealed hepatic laceration in right lobe in 14 (70%) and in left lobe in six (30%) patients. Endoscopic retrograde cholangiopancreatography (ERCP) revealed biliary leak in right duct in 14 (70%) and in left duct in six (30%) patients. Five patients also had bilhemia and one had hemobilia. Thirteen patients (65%) were treated by endoscopic sphincterotomy with nasobiliary drainage and seven (35%) were treated by nasobiliary drainage alone, which enabled fistula closure in 15.76 +/- 4.22 days and 12.14 +/- 3.93 days, respectively (P > 0.05). One patient in sphincterotomy group died due to multiple bony injuries and fat embolism. Two patients developed fever following ERCP, which responded to antibiotic treatment. CONCLUSIONS: Endoscopic treatment with nasobiliary drainage without sphincterotomy is the optimal method of management of traumatic hepatobiliary injuries in hemodynamically stable patients.

Angiotensin-converting enzyme gene polymorphism in North-west Indian children with posterior urethral valves.

PURPOSE: To investigate the association of angiotensin-converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism and other risk factors with renal scarring in patients with posterior urethral valves (PUV). MATERIALS AND METHODS: Forty consecutive patients from North-west India were treated for PUV in 1997-2004. The patients were divided into group 1 (no renal scarring, n=12) and group 2 (renal scars present, n=28) based on dimercato-succinic acid scans. ACE I/D polymorphism was determined by polymerase chain reaction in PUV patients and unrelated healthy controls (n=100). RESULTS: Mean age at presentation was 23.7+/-37.2 months and mean follow up was 4.8+/-1.5 years. Preoperative mean serum creatinine levels for group 1 (non-scarred) and group 2 (scarred) were 1.1+/-1.6 mg/dl and 1.7+/-1.6 mg/dl, respectively. One year after treatment, the serum creatinine levels had decreased to 0.6+/-0.1 mg/dl and 0.8+/-0.3 mg/dl in group 1 and group 2, respectively. ACE genotype distribution in children with PUV was no different from that of controls. The occurrence of D allele was significantly (p=0.04) higher in patients of group 2. Multivariate logistic regression analysis showed that D allele had a significant impact on renal scar formation, introducing a 4.6-fold risk (odds ratio 4.6, 95% confidence interval 1.03-20.38, p=0.04). A highly significant correlation between the occurrence of renal scarring and presence of breakthrough urinary tract infection (odds ratio=7.5, 95% confidence interval 1.60-35.07, p=0.006) and serum creatinine at follow up (odds ratio=0.6, 95% confidence interval 0.47-0.81, p=0.03) was observed. The mean values for glomerular filtration rate (GFR) after 1 year of treatment (p=0.006) and at follow up (p=0.027) were significantly different between the patients with II genotype and ID/DD genotype. Hypertension was observed in 13 patients and proteinuria in nine patients with no significant difference between the patients having II/I D/DD genotypes. CONCLUSION: The presence of D allele is associated with progression of renal scarring and reduced GFR in PUV patients.

Does mode of treatment affect the outcome of neonatal posterior urethral valves?

PURPOSE: A prospective study on the outcome of posterior urethral valves (PUV) was performed. The data analyzed were whether the modality of treatment (fulguration vs vesicostomy) affected renal function and somatic growth, and whether the presence of vesicoureteral reflux (VUR) and abnormal serum creatinine levels affected somatic growth. MATERIALS AND METHODS: A total of 45 consecutive neonates were diagnosed and treated for PUV with fulguration (24) or vesicostomy (21) between 1997 and 2003. Postoperative stable creatinine values, renal function and somatic growth were recorded. Well tempered renal scans using diethylenetetraminepentaacetic acid were performed during the first and second years of life at followup. Standard anthropometric techniques and statistical methods were used to compute distance statistics for body weight and crown-heel length at age intervals of 3 months for year 1 and 6 months for year 2. RESULTS: Of the patients 9 were lost to followup and 6 died in the first year of life due to renal failure. Preoperative and postoperative mean serum creatinine was 1.6 +/- 1.5 and 0.7 +/- 0.2 mg/dl, for the fulguration group and 1.7 +/- 1.5 and 0.9 +/- 0.7 mg/dl, respectively, for the vesicostomy group. In 10 patients renal function deteriorated or there was no improvement after treatment. With the advancement of age neonates with PUV showed normal increase in body weight and crown-heel length. Those with VUR were significantly shorter compared to neonates with no VUR at the end of year 2 of life (p <0.05). Patients with creatinine less than 1 mg/dl had significantly greater increase in body weight (at 3 and 6 months) as well as crown-heel length (at 3 and 9 months), respectively (p <0.05). Physical growth of the fulguration and vesicostomy groups remained substantially lower than that of their normal healthy counterparts. Newborns treated with vesicostomy did not have any breakthrough urinary tract infections. Although patients treated with vesicostomy were lighter and smaller initially (p <0.05), they were comparable to those treated with fulguration at the end of 2 years, thus demonstrating catch-up growth. CONCLUSIONS: Our prospective study demonstrated that transurethral fulguration and vesicostomy are equally effective for neonatal valves and achieve similar renal function. Both groups showed retarded growth compared to healthy counterparts. Somatic growth was delayed by serum creatinine greater than 1.0 mg/dl and the presence of VUR. Vesicostomy seemed to help neonates catch-up the growth deficit in the first 2 years of life.