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

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.

Persistent cloaca and caudal duplication in a monovular twin, a rare case report

“…Caudal duplication can encompass a variety of phenotypes. In cases of caudal duplication, leg or leg bones may be duplicated or triplicated [6,7]. Duplication of the cloaca may also be seen [6].…”

“…Dominguez et al explained that the embryologic basis for this was a developmental defect occurring to the caudal cell mass or eminence. The caudal cell mass is made of undifferentiated mesenchyme formed when the notochord and neural tube unite at 23–25 days gestation [8,9] This mass gives rise to the entire terminal spinal cord, hindgut, sacrum and pelvic soft tissues [6]. It was postulated that Kovalevsky’s canal, a communication that connects the amniotic and yolk sac fails to regress which can form fibrous bands that can divide the notochord and adjacent mesoderm or form dorsal enteric fistulous connections [7].…”

“…When there are 2 organizing centers the productivity of 1 or 2 centers may be decreased. Since the caudal eminence arises late in gastrulation these may be at particular risk [6,10].…”

“…In general embryos conceived with in vitro fertilization have a higher rate of twinning. One such explanation is the iatrogenic damage to the zona pellucida allowing a blastomere to separate and potentially form a separate embryo with placental tissue [6]. It is possible that our patient represented an incomplete form of twinning after twinning had already occurred through iatrogenic damage to the zona pellucida or that this was secondary to failed triplet formation from a single embryo.…”

Surgical management of caudal duplication syndrome: A rare entity with a centered approach on quality of life

“…[1] In this similar case, the surgical approach must be tailored to the individual using a multidisciplinary team. [7]…”