Novel approach to locating a hypoplastic kidney in a unique variant of uterine didelphys syndrome presenting with continuous incontinence

Mullerian abnormalities such as uterine didelphys have an association with renal abnormalities. Ureteric ectopia must be considered as a differential diagnosis of incontinence. We describe the case of a 21-year-old woman who presented with low volume continuous incontinence with a history of right renal agenesis and uterus didelphys. A right kidney was not identified on CT intravenous pyelogram but excretory phase images suggested the presence of a right ureter. Diethylenetriaminepentaacetic acid renogram confirmed an area of perfusion consistent with a poorly functioning right kidney remnant. Cystoscopic investigation demonstrated an orthotopic left ureteric orifice, and an ectopic right ureteric orifice was identified in the posterior fornix of the right vagina. A laparoscopic right nephrectomy was performed, with a new application of indocyanine green aiding in identification of the right hypoplastic kidney with fluorescence imaging. The patient recovered well postoperatively and experienced complete resolution of incontinence, with preserved normal renal function.

Unilateral Hypoplastic Kidney in a Case of Holt-Oram Syndrome

Holt-Oram syndrome (HOS) is characterised by skeletal abnormalities of the upper limb & congenital cardiac defects. Here we report a case of one day old male baby with skeletal abnormalities of forearms and hands, atrial septal defect of ostium secundum type and unilateral hypoplastic kidney. Till date, other associated anomalies like extra-hepatic portal hypertension, renal artery malformations etc have been reported with Holt-Oram syndrome, but it is the first case to be reported in association with hypoplastic kidney. DOI: http://dx.doi.org/10.3126/jnps.v33i1.7127 J Nepal Paediatr Soc. 2013;33(1):77-79