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Year : 2013  |  Volume : 3  |  Issue : 3  |  Page : 222-225

Syndromic exomphalos in Ile-Ife Nigeria: Management challenges

Department of Surgery Paediatric Surgery Unit, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Nigeria

Correspondence Address:
Lofty-John C Anyanwu
P. O. Box 2536, Kano, Kano State
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2278-9596.129569

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Background: Between 50% and 70% of children with exomphalos have other associated anomalies, which may involve the heart, abdominal viscera and urogenital organs resulting in high morbidity and mortality. The aim of this study is to review the management of patients with syndromic exomphalos in our center and to highlight the management challenges. Materials and Methods: We retrospectively reviewed the records of patients with pentalogy of cantrell, cloacal exstrophy and Beckwith-Wiedemann syndrome who presented to our unit between January 1997 and December 2007. Results: There were a total of 10 patients. Pentalogy of cantrell was the diagnosis in three patients while four of them had cloacal exstrophy and another three patients had Beckwith-Wiedemann syndrome. Their ages at presentation ranged between 2 h and 6 weeks, with a median of 27 h. Their birth weights ranged between 1.6 kg and 4.95 kg, median 2.45 kg. 5 patients (50%) had a low birth weight (<2.5 kg). Of the 10 patients, 4 (40%) were discharged against medical advice (1 was readmitted) and 2 (20%) were lost to follow-up. There were 5 (50%) mortalities during the course of treatment. None of them had karyotyping done. Conclusion: The management of congenital ventral body wall defects is challenging in resource poor settings like ours, due to lack of appropriate facilities and relevant manpower.

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