Cloacal exstrophy with extensive Chiari II malformation: case report and review of the literature

Introduction

Cloacal exstrophy is a rare condition characterized by a defect in the abdominal wall with extrusion of malformed abdominal contents. The normal separation of the genitourinary and gastrointestinal systems does not occur. While patients with cloacal exstrophy have a high incidence of spinal defects, cranial defects are rare. Chiari malformation has been rarely reported in children with cloacal exstrophy, although the embryogenesis is unknown.

Objective

In this report, the authors describe a child with cloacal exstrophy and a large myelocystocele. The child also had an extensive Chiari II malformation.

Results

We review the relevant embryology and suggest a possible mechanism for Chiari formation in this patient.