Caudal Duplication Syndrome: the Vital Role of a Multidisciplinary Approach and Staged Correction.

David Ben-Meir,Dragan Kravarusic,Elena Dlugy,Enrique Freud,Gustavo Rajz,Inbal Samuk,Marc Levitt,Osnat Konen

doi:10.1055/s-0035-1570370

Abstract

Caudal duplication syndrome is a rare entity that describes the association between congenital anomalies involving caudal structures and may have a wide spectrum of clinical manifestations. A full-term male presented with combination of anomalies including anorectal malformation, duplication of the colon and lower urinary tract, split of the lower spine, and lipomyelomeningocele with tethering of the cord. We report this exceptional case of caudal duplication syndrome with special emphasis on surgical strategy and approach combining all disciplines involved. The purpose of this report is to present the pathology, assessment, and management strategy of this complex case.

Highlights

Caudal duplication syndrome (CDS) represents a rare entity of congenital anomalies that involve the gastrointestinal and urogenital tracts and neural tube.[1,2] The term caudal duplication described a spectrum in which structures derived from the embryonic cloaca and notochord are duplicated to various degrees.[3]
A full-term male presented with combination of anomalies including anorectal malformation, duplication of the colon and lower urinary tract, split of the lower spine, and lipomyelomeningocele with tethering of the cord. We report this exceptional case of caudal duplication syndrome with special emphasis on surgical strategy and approach combining all disciplines involved
We report an exceptional case of a male baby with CDS and delineate the surgical approach and objectives

Summary

Introduction

CDS represents a rare entity of congenital anomalies that involve the gastrointestinal and urogenital tracts and neural tube.[1,2] The term caudal duplication described a spectrum in which structures derived from the embryonic cloaca and notochord are duplicated to various degrees.[3]. Contrast study through both perineal fistulae demonstrated duplication of the rectum and colon to the level of the hepatic flexure, it was difficult to define the exact extension level of duplication (►Fig. 2). Two hemibladders were clearly noted with grade II left vesicoureteral reflux (►Fig. 3) The baby was formula fed since birth and had daily soft bowel movements He gained weight and his developmental milestones were normal. At age 1.5 years, a posterior anorectoplasty of the duplicated perineal fistulae was performed. During the procedure, both fistulae were mobilized to the level of the previously constructed rectal reservoir (►Fig. 7). The postoperative period was uneventful and the baby was discharged for further ambulatory follow-up

Discussion

Summary