Multistage Reconstruction of a Severe Cloacal Exstrophy Variant With Associated Myelomeningocele and Multisystem...

To our knowledge, this case represents one of the rarest and most severe forms of cloacal exstrophy (CE), distinguished by extensive multisystem involvement, including a large omphalocele, imperforate anus, bladder exstrophy, bilateral clubfeet, and the unusual concomitant presence of a lumbosacral myelomeningocele. This constellation of findings is exceptionally uncommon and poses significant surgical and multidisciplinary challenges from birth. Prenatal MRI demonstrated a large omphalocele containing liver, bowel, and gallbladder; a lumbar myelomeningocele (L5-S5); nonvisualization of the bladder and external genitalia; unilateral renal agenesis; and bilateral clubfeet. The infant was delivered at 37 weeks via cesarean section and required intubation at 10 minutes of life. Postnatal evaluation confirmed omphalocele, imperforate anus, meningocele, bladder exstrophy, and bilateral clubfeet. Echocardiography revealed a small mid-muscular ventricular septal defect and a moderate patent ductus arteriosus with bidirectional shunt, requiring no intervention. SNP microarray testing was negative for copy number abnormalities. At five days of life, the patient underwent excision of the omphalocele, colostomy creation, colon conduit cystoplasty, and first-stage closure of the bladder plate. At four months, neurosurgical repair of the lipomeningocele with resection of an extradural lipoma, bilateral muscle flaps, and complex soft-tissue reconstruction was performed. Orthopedic management included treatment of a right leg fracture, followed by bilateral pelvic osteotomies and application of a hip spica cast at eleven months. Definitive bladder exstrophy closure was achieved after orthopedic stabilization. The patient demonstrated stable cardiopulmonary status, preserved renal function, and appropriate recovery following each stage of repair. This case illustrates the extreme end of the CE spectrum and underscores the necessity of multidisciplinary coordination among pediatric surgery, urology, neurosurgery, orthopedics, and plastic surgery. Through careful sequencing and staged reconstruction, preservation of gastrointestinal, neurologic, orthopedic, and urologic function was achieved. Our experience highlights that, even in the most severe and rare presentations, particularly those with concurrent myelomeningocele, functional survival and meaningful quality of life are possible with meticulous planning and comprehensive care.