Publication: Omphalocele, Exstrophy of Cloaca, Imperforate Anus, and Spinal Defects Complex: A Case Report
Date
2023
Journal Title
Journal ISSN
Volume Title
Publisher
Nepal Medical Association
Abstract
Abstract
Omphalocele, exstrophy of cloaca, imperforate anus, and spinal defects complex is a rare malformation
complex that includes omphalocele, cloacal exstrophy, imperforate anus and spinal defects with the
incidence of 1 in 200,000 to 400,000 pregnancies and is even rarer in twin gestation. The etiology of
this complex is still unclear. Most cases are sporadic. Prenatal screening must be done for diagnosis
and appropriate multidisciplinary management of cases. In severe cases, termination of pregnancy is
considered. We present a 4-day first twin child with underdeveloped ambiguous genitalia delivered
via emergency lower section cesarean section at 32+3 weeks of gestation with giant liver containing
omphalocele, cloacal exstrophy, imperforate anus and meningocele with severe pulmonary artery
hypertension 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.
Description
Suraj Singh
Department of Pediatrics Surgery, Kanti Children’s Hospital, Maharajgunj, Kathmandu, Nepal
Anuj Kayastha
Department of Pediatrics Surgery, Kanti Children’s Hospital, Maharajgunj, Kathmandu, Nepal
Anupama Thapa
Department of Pediatrics Surgery, Kanti Children’s Hospital, Maharajgunj, Kathmandu, Nepal
Bijay Thapa
Department of Pediatrics Surgery, Kanti Children’s Hospital, Maharajgunj, Kathmandu, Nepal
Sulochana Dahal
Department of Pediatrics Surgery, Kanti Children’s Hospital, Maharajgunj, Kathmandu, Nepal
https://orcid.org/0000-0002-4389-0168
Keywords
Anorectal malformations, Bladder exstrophy, Case reports, Neural tube defects, Umbilicus, 375