Description of Congenital anomalies Associated with omphalocele in children attended to in Mulago Hospital

Abstract

Introduction: Omphalocele is a common congenital midline abdominal wall defect and its impact is great on new born infants and is more severe in low and middle income countries (LMICs) where health care resources are limited. However, there is inadequate description and documentation of the associated congenital anomalies or birth defects in LMICs, Uganda inclusive. Objective: The aim of the study was to describe the frequency and nature of birth defects associated with omphalocele amongst neonates. Methods: A cross - sectional descriptive study with consecutive enrolment of all neonatal omphalocele seen in Mulago National Referral Hospital (MNRH) between June 2015 and March 2016 after consent of a parent. Participants underwent physical examination, ultrasonography of the abdomen, babygram, echocardiography and random blood sugar estimation. Data was collected on a designed questionnaire and univariate analysis performed using SSPS version 20 for frequencies and proportions. Results: A total of 38 omphalocele cases were enrolled out of 30,003 neonates attended to with prevalence of 0.12%, F:M ratio of 1:1.1 and median age of 4 days (Range: 1 – 28 days). Full term delivery was 76 % (29/38) and 78.9% (30/38) of the neonates weighed 2.5 kg and above. Antenatal care attendance and folic acid usage was 100% (38/38). The commonest syndrome was Beckwith – Wiedemann (26%; 10/38), pentalogy of Cantrell (3%; 1/38). Cardiac birth defects was (10.5%; 4/38) and musculoskeletal system (13.2%; 5/38). xv Conclusion: Omphalocele was still a rare congenital anomaly and the commonest syndrome associated with it was Beckwith – Wiedemann. Sonographic and radiologic investigations are needed to assess the associated defects.