Omphalocele, Exstrophy of Cloaca, Imperforate Anus, and Spinal Defects Complex: A Case Report

Abstract

Omphalocele, exstrophy of cloaca, imperforate anus, and spinal defects complex is a rare malformationcomplex that includes omphalocele, cloacal exstrophy, imperforate anus and spinal defects with theincidence of 1 in 200,000 to 400,000 pregnancies and is even rarer in twin gestation. The etiology ofthis complex is still unclear. Most cases are sporadic. Prenatal screening must be done for diagnosisand appropriate multidisciplinary management of cases. In severe cases, termination of pregnancy isconsidered. We present a 4-day first twin child with underdeveloped ambiguous genitalia deliveredvia emergency lower section cesarean section at 32+3 weeks of gestation with giant liver containingomphalocele, cloacal exstrophy, imperforate anus and meningocele with severe pulmonary arteryhypertension and non-visualization of right kidney and ureter, absence of uterus, fallopian tubes and right ovary. Separation and repair of the cecum and bladder were done. The ladd procedure was performed. Ileostomy was created and single-stage repair of the abdominal wall was done.

Detection and management of a case of single system ectopic ureteral insertion to vagina with atrophic ectopic kidney

“…Pathognomonic feature of an infrasphincteric ureteral opening is normal urination together with persistent incontinence [2] . Due to insufficient assessment, the diagnosis of ectopic ureteral insertion is delayed.…”