Transverse fusion of the renal pelves and single ureter

Abstract

We present a case of a rare renal anomaly in which the 2 kidneys (separate or fused across the midline) are drained by a common renal pelvis and ureter. Previously reported cases have been reviewed and are classified in 3 groups according to their anatomical features. The lesion is associated with some frequency with imperforate anus, sacral and other vertebral defects, neurogenic bladder, vesicoureteral reflux, upper tract dilatation, and urinary tract infections.

Urorectal septum malformation sequence: Insights into pathogenesis

“…The homeobox gene HLXB9 (Genbank #142994) is mutated in the Currarino triad (OMIM #176450), which is composed of dominantly inherited anorectal and GU malformations, a presacral mass, and partial sacral agenesis (Currarino and Weisbruch, 1989;Currarino, 1996;Belloni et al, 2000;McKusick, 2002). The observed pattern of anomalies can be explained by a malformation in the caudal portion of the notochord, resulting in aberrant secondary neurulation.…”

Renal pyelic fusion with crossed solitary ureter: Case report and review of literature

“…A single ureter with both the renal units in as normal position, as in the present case, is exceedingly rare with very few cases reported in literature (Table ). Anomalies of kidney fusion are usually associated with neurogenic bladder, VUR, recurrent UTI, pelvi‐ureteric junction obstruction, and upper tract dilatations and extra‐urinary anomalies, such as imperforate anus, cardiac valvular defects and sacral or other vertebral anomalies . In the present case, the child had recurrent UTI, VUR, upper tract dilatation, neurogenic bladder and lumbar vertebrae fusion defects.…”

“…Anomalies of kidney fusion are usually associated with neurogenic bladder, VUR, recurrent UTI, pelvi-ureteric junction obstruction, and upper tract dilatations and extra-urinary anomalies, such as imperforate anus, cardiac valvular defects and sacral or other vertebral anomalies. 6 In the present case, the child had recurrent UTI, VUR, upper tract dilatation, neurogenic bladder and lumbar vertebrae fusion defects. This anomaly occurs at an early phase in embryological development.…”

“…Cass and Vitko proposed that a single ureter crosses to the opposite side and then divides into two halves, with half crossing the midline before each half influenced the development of its renal mass. Another possible explanation is that of failure of one ureter to develop, associated with branching of the other ureteral bud in early fetal life to meet both renal blastemas . According to Schwartz, the explanation of a single ureter was to ensure coexisting absent vas deferens.…”

“…Another possible explanation is that of failure of one ureter to develop, associated with branching of the other ureteral bud in early fetal life to meet both renal blastemas. 6 According to Schwartz, the explanation of a single ureter was to ensure coexisting absent vas deferens. Absent vas deferens imply Wolfian duct abnormalities, and therefore potential anomalies of the ureteral buds.…”