The current profile of persistent cloaca and cloacal exstrophy in Japan: the results of a nationwide survey in 2014 and a review of the literature

PURPOSE: The current profile of persistent cloaca (PC) and cloacal exstrophy (CE) in Japan was first examined in 2014. MATERIALS AND METHODS: Information was obtained by sending a questionnaire to 244 university hospitals and children’s hospitals. RESULTS: Responses from 113 institutions reported 466 PC cases and 229 CE cases. The incidences of PC and CE from 1980 to 2012 were 0.97 and 0.49 per 100,000 live births, respectively. In the previous 5 years, antenatal abnormalities were found in 57.6% of PC and 72.7% of CE patients. Myelomeningocele was observed in 45.6% of CE patients. As a result of various surgical treatments used in the neonatal and infantile periods, the respective rates of bladder dysfunction, clean intermittent catheterization, and permanent enterostomy were 32.6, 22.5, and 7.3% in PC patients and 60.7, 28.4, and 73.8% in CE patients. Menstrual outflow obstruction was found in 22.5% of PC and 48.9% of CE patients with menstruation. CONCLUSION: The clinical outcomes of PC and CE remain unsatisfactory. Therefore, the establishment of treatment guidelines might be a useful objective for improving the current status of PC and CE.