The Relevance of Follow-up after Primary Management of Omphalocele in Beckwith-Wiedemann Syndrome: A Series of Five Cases

Omphalocoele, also called exomphalos, though a large disease by itself, can just be one part of association of much broader growth disorder of Beckwith-Wiedemann Syndrome (BWS), which one needs to be familiar with primarily. The omphalocoele is amenable to primary surgical correction in neonatal period. With the successful treatment of omphalocoele in the spectrum of BWS does not conclude the management, as the literature suggests the follow-up is imperative as there is increased incidence of visceral malignancy and blood cancers in these patients over the years of life. Present series is of five cases of BWS treated at birth for omphalocoele by surgery successfully, kept on regular follow-up, with one child developing leukaemia and loss of life due to the disease. The emphasis thus is on the need identification of the syndrome of BWS in a case of omphalocoele and also elaborating on their follow-up, long enough after surgery because of the risk of development of various malignancies.