Cloacal exstrophy: A comprehensive review of an uncommon problem

Abstract

To provide a comprehensive overview of the clinical features, diagnosis, current management strategies, and outcomes of cloacal exstrophy. A PUBMED/Medline search of the literature was performed on cloacal exstrophy focusing on associated anomalies, treatment, and quality of life issues. The incidence of cloacal exstrophy is between 1 in 200,000 and 400,000 live births. Survival rates now approach 100% secondary to improved understanding of underlying abnormalities and advances in neonatal care and surgical technique. Important principles of initial management include proper nutritional support, early closure of exstrophy, and preservation of intestinal length. The achievement of urinary and fecal continence remains a challenge. Data for long-term outcomes are now emerging which provide new insight into issues of gender identity, function, and psychosocial development of these patients. Cloacal exstrophy remains a rare and complex congenital anomaly, characterized by an array of anatomical defects affecting multiple organ systems. A multidisciplinary approach to management is advocated with a focus on optimization of patient function and quality of life.