Omphalocoeles: A decade in review
Background. Omphalocoeles are associated with significant morbidity and mortality. The presentation varies greatly and management options differ accordingly. Limited literature exists regarding the varied presentation, associated congenital abnormalities and survival from a South African, or even an...
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doaj-04d7064c6c134da688efda43daa22e692020-11-24T23:31:24ZengHealth and Medical Publishing GroupSouth African Journal of Child Health1994-30321999-76712016-12-01104211214Omphalocoeles: A decade in reviewSimmi Singh0Anil Madaree1Department of Plastic Surgery, Inkosi Albert Luthuli Central Hospital and School of Clinical Medicine, College of Health Sciences, Nelson R Mandela School of Medicine, University of KwaZulu-Natal, Durban, South AfricaDepartment of Plastic Surgery, Inkosi Albert Luthuli Central Hospital and School of Clinical Medicine, College of Health Sciences, Nelson R Mandela School of Medicine, University of KwaZulu-Natal, Durban, South AfricaBackground. Omphalocoeles are associated with significant morbidity and mortality. The presentation varies greatly and management options differ accordingly. Limited literature exists regarding the varied presentation, associated congenital abnormalities and survival from a South African, or even an African perspective. Objective. To describe the presentation of omphalocoeles, associated abnormalities and survival rates. Methods. A retrospective epidemiological chart review of patients referred to the paediatric surgical service with newly diagnosed omphalocoeles, between January 2002 and December 2012. Data retrieved included patient demographics, perinatal history, HIV status, associated abnormalities, size of the omphalocoele, management and outcome. Results. One hundred and fifty-four patients were diagnosed with an omphalocoele during the study period. There were 117 (75.9%) associated congenital abnormalities, 64 (41.5%) minor omphalocoeles (defined as <5 cm) and 66 (42.8%) major omphalocoeles (defined as >5 cm). Eleven patients (7.1%) had ruptured omphalocoeles. Beckwith-Wiedemann syndrome was the most commonly associated abnormality (37.6%), followed by cardiac defects (34.4%). Conclusion. Omphalocoeles are associated with high numbers of congenital abnormalities. This further complicates management in a resource-poor environment. There is an increased association with Beckwith-Wiedemann syndrome compared with previous studies. This highlights the need to be vigilant with glucose monitoring and to prevent secondary, avoidable complications.http://www.sajch.org.za/index.php/SAJCH/article/view/1149/743congenital abnormalitiesexomphalosOmphalocoele |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Simmi Singh Anil Madaree |
spellingShingle |
Simmi Singh Anil Madaree Omphalocoeles: A decade in review South African Journal of Child Health congenital abnormalities exomphalos Omphalocoele |
author_facet |
Simmi Singh Anil Madaree |
author_sort |
Simmi Singh |
title |
Omphalocoeles: A decade in review |
title_short |
Omphalocoeles: A decade in review |
title_full |
Omphalocoeles: A decade in review |
title_fullStr |
Omphalocoeles: A decade in review |
title_full_unstemmed |
Omphalocoeles: A decade in review |
title_sort |
omphalocoeles: a decade in review |
publisher |
Health and Medical Publishing Group |
series |
South African Journal of Child Health |
issn |
1994-3032 1999-7671 |
publishDate |
2016-12-01 |
description |
Background. Omphalocoeles are associated with significant morbidity and mortality. The presentation varies greatly and management options differ accordingly. Limited literature exists regarding the varied presentation, associated congenital abnormalities and survival from a South African, or even an African perspective.
Objective. To describe the presentation of omphalocoeles, associated abnormalities and survival rates.
Methods. A retrospective epidemiological chart review of patients referred to the paediatric surgical service with newly diagnosed omphalocoeles, between January 2002 and December 2012. Data retrieved included patient demographics, perinatal history, HIV status, associated abnormalities, size of the omphalocoele, management and outcome.
Results. One hundred and fifty-four patients were diagnosed with an omphalocoele during the study period. There were 117 (75.9%) associated congenital abnormalities, 64 (41.5%) minor omphalocoeles (defined as <5 cm) and 66 (42.8%) major omphalocoeles (defined as >5 cm). Eleven patients (7.1%) had ruptured omphalocoeles. Beckwith-Wiedemann syndrome was the most commonly associated abnormality (37.6%), followed by cardiac defects (34.4%).
Conclusion. Omphalocoeles are associated with high numbers of congenital abnormalities. This further complicates management in a resource-poor environment. There is an increased association with Beckwith-Wiedemann syndrome compared with previous studies. This highlights the need to be vigilant with glucose monitoring and to prevent secondary, avoidable complications. |
topic |
congenital abnormalities exomphalos Omphalocoele |
url |
http://www.sajch.org.za/index.php/SAJCH/article/view/1149/743 |
work_keys_str_mv |
AT simmisingh omphalocoelesadecadeinreview AT anilmadaree omphalocoelesadecadeinreview |
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