Description and early clinical outcome of patients with omphalocele admitted to a tertiary hospital in Kigali, Rwanda over a three-year period – a cross-sectional study

BACKGROUND: Studies suggest that patients with omphalocele have poor outcomes which are associated with the high rate of associated congenital abnormalities. A variety of surgical management techniques have been described, with some requiring equipment not available in resource-limited countries.
OBJECTIVES: This study aimed to describe associated abnormalities, management and early hospital outcomes of patients with omphalocele.
METHODS: A retrospective study of newborns diagnosed with omphalocele hospitalized from January 2014 to December 2016 at a tertiary level facility, the University Hospital of Kigali (CHUK) in Kigali, Rwanda. Case-file review of included newborns and analysis of the data collected was performed with SPSS version 16.0.
RESULTS: Thirty-one patients were hospitalized with omphalocele during the three-year study period. A limited number of investigations were done to diagnose possible associated congenital abnormalities. Three patients (9.7%) were found to have cardiac abnormalities, nine (27.0%) had suspected genetic abnormalities including Trisomy 13, 18 and Beckwith Wiedemann Syndrome. In terms of management, ten patients (32.3%) underwent primary closure. Sixteen patients (51.6%) developed sepsis during hospitalization. Of the thirty-one patients hospitalized, nine (27.0%) died in the hospital. The only factor found to be associated with increased mortality was the size of the omphalocele, with small omphaloceles associated with lower mortality than large omphaloceles (OR 0.909, P-value 0.028).
CONCLUSION: Mortality of these patients is significant and slightly higher in patients with larger omphalocele. Newborns with omphalocele should receive appropriate investigations to diagnose associated congenital abnormalities and have early recognition and prompt management of complications, especially sepsis. The results are limited by the small sample size and limited investigations performed to detect associated abnormalities.

Keywords
Omphalocele; abdominal wall defect; congenital abnormalities; early mortality.