Herlyn–Werner–Wunderlich Syndrome: Case report of a newborn presenting with interlabial cyst

A newborn with Herlyn–Werner–Wunderlich Syndrome presented with interlabial cyst. The cyst was punctured and instilled with contrast medium for cystography to demonstrate the anatomy. Simple resection of the hemivagina septum at the same sitting resolved the obstruction. Early interventions done to clinch diagnosis and institute treatment would help to prevent future complications associated with menses retention and preserve fertility

Should micturating cystourethrogram be routinely performed for paediatric pyeloplasty patients?

Objective: Micturating cystourethrogram (MCUG) has traditionally been recommended as part of the preoperative evaluation of all cases of hydronephrosis. Several studies have shown that the majority of cases with pelviureteric junction obstruction (PUJO) and concomitant vesicoureteral reflux (VUR) were of low-grade and generally resolved spontaneously. We therefore retrospectively evaluated the need for routine MCUG in all cases of PUJO. Methods: We conducted a retrospective review of clinical records of all patients who underwent pyeloplasty in our institution between 2003 and 2015. Data collected included patient demographics, clinical presentation, radiological procedures performed, operative details as well as postoperative outcomes. Results: A total of 119 patients underwent pyeloplasty for PUJO during this study period. MCUG was performed in 88 patients (74%), of whom eight patients had VUR. All eight patients had unilateral PUJO and two patients had bilateral VUR. Only two patients had high-grade VUR (grade 4). These two patients also had hydroureter detected on ultrasound scan (US). All patients with VUR had spontaneous resolution of reflux. While comparing outcomes for patients with and without VUR, there was no statistically significant difference in terms of the need for redo surgery and improvement in differential renal function post-pyeloplasty. Conclusion: The outcome of pyeloplasty appears to be independent of the presence of concomitant VUR. Therefore, we conclude that MCUG does not need to be performed routinely for all PUJO patients. However, patients with history of recurrent urinary tract infection or findings of hydroureter on US should still be investigated with MCUG

Neuronal defects an etiological factor in congenital pelviureteric junction obstruction?

Introduction: Congenital pelviureteric junction obstruction (PUJO) is one of the most frequent causes of neonatal hydronephrosis. Obstruction at the PUJ has potential severe adverse outcomes, such as renal damage. While pyeloplasty has been established as the definitive treatment, the exact pathophysiology of congenital PUJO remains unknown. Recent research has proposed neuronal innervation defects as an etiological factor in congenital PUJO. We aim to study the expression of various neuronal markers in PUJO specimens compared with controls, and evaluate whether severity of renal disease or dysfunction pre-operatively is related to expression of neuronal markers in resected PUJO specimens. Materials and methods: All consecutive patients who underwent dismembered pyeloplasty at KK Women's and Children's Hospital, Singapore, for intrinsic PUJO from 2008 to 2012 were included. Patients with other co-occurring renal pathologies were excluded. Controls were obtained from nephrectomy patients with Wilm's tumor or other benign renal conditions during the same period. Specimens were stained immunohistochemically with neuronal markers protein gene product 9.5 (PGP9.5), synaptophysin, and S-100, and with CD-117, a marker for interstitial cells of Cajal (Table). Levels of expression of the markers were assessed semiquantitatively (decreased, increased or no change) in comparison with controls by two independent observers. Pre-operative data of patients’ renal anatomical (ultrasonography measurements of renal pelvis size) and functional parameters (differential renal function measured using MAG-3 renal scans) were obtained. Discussion: Thirty-eight PUJO specimens (38 renal units) and 20 controls were studied. Mean patient age at pyeloplasty was 25.3 months (2.9–167.6 months). Median pre-operative pelvic size was 25.0 mm (17.0–50.0 mm). Both PUJO specimens and controls showed great heterogeneity in distribution of innervation. All four immunohistochemical markers were not predictive of significant pre-operative renal pelvis dilation or pre-operative diminished renal function of the operated kidney. Conclusions: There exists marked variability in expression of neuronal markers synaptophysin, PGP9.5, and S-100, and CD-117 in PUJO specimens compared with controls. Our results show no clinical significance of the expression of neuronal markers in predicting degree of pre-operative renal pelvis dilation or differential renal function. The heterogeneity of expression of neuronal markers in PUJO specimens and controls in our population is at variance with prior studies. The etiology of PUJO is likely to be complex and multifactorial. [Table presented