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.
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.