Background: The association of umbilical cord ulcer (UCU) and congenital upper intestinal atresia (CUIA) has been reported since 1991. CUIA is a treatable disease itself with fair prognosis. It is not well acknowledged that some infants who have CUIA and UCU develop hemorrhagic shock followed by intrauterine demise/neonatal death. Most of the reported cases were from Asia, especially from Japan.
Objective: We reviewed reported cases with CUIA accompanied by UCU. We intend to find out clinic-pathological characteristics of this serious condition.
Method: We searched the above-mentioned association using Pub Med, Japanese journals, and congress abstracts. We reviewed clinical summaries the specimens of placentas and the umbilical cords of patients with CUIA who or or who's mothers were admitted to our hospital.
Result: Sixty-four cases with CUIA accompanied by UCU have been identified. Only 7 cases of this association have been published in countries other than Japan. In contrast, in Japan, the reported number of cases has increased since the first report from our hospital in 1996. As of December 2011 57 cases have been reported in Japan, including 15 cases from our hospital. Outcome was described in 63 of the 64 cases: 28 infants (44%) resulted in intrauterine or neonatal death, while 35 lived, of whom 8 had severe sequel.
Pathological examination of 108 placentas of infants with CUIA in our hospital revealed 15 of complete/lacerated and 1 of abortive UCU were found.
Conclusion: Mortality rate of the association is as high as 44%. Close observation using fetal ultrasonography might predict massive hemorrhagic event.
intestinal atresia, umbilical cord ulcer