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Exomphalos – a major or minor problem?
Author(s) -
O'Neill T.,
Kirby F.,
O'Brien K.,
Warde D.
Publication year - 2002
Publication title -
pediatric anesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.704
H-Index - 82
eISSN - 1460-9592
pISSN - 1155-5645
DOI - 10.1046/j.1460-9592.2002.10271_8.x
Subject(s) - medicine , omphalocele , congenital diaphragmatic hernia , pulmonary hypoplasia , pediatrics , maldevelopment , intensive care medicine , gestation , pregnancy , fetus , genetics , anatomy , biology
The mortality and morbidity statistics associated with exomphalos major remain discouraging despite advances in management techniques (1). Congenital lung pathology, in particular pulmonary hypoplasia, and thoracic maldevelopment, have been strongly allied to this condition, accounting for the high incidence of pulmonary insufficiency necessitating prolonged ventilatory support in these infants (2). We discuss the respiratory issues in an infant with a particularly severe form of exomphalos major, and the impact of a comprehensive parental website devoted to the infants' management and progress. Case Report A female infant, born at 38 weeks' gestation, was referred for management of exomphalos major. Due to the extensive nature of the abdominal wall defect, primary surgical closure was impossible and initial management consisted of staged reduction by external compression of the exomphalos. This resulted in escalating cardiovascular and respiratory embarrassment, and was abandoned in favour of conservative treatment, whereby the sac was dressed and allowed to epithelialise. Thereafter the clinical course was characterised by chronic pulmonary insufficiency requiring prolonged ventilatory support. Ventilator dependence did not significantly decrease until lung growth occurred and the sitting position was adopted, enabling weaning from conventional ventilation to genuine BIPAP at 6 months. Currently, after 11 months, we are preparing her for entry into a home ventilation programme. Throughout this period, progress and realistic goals were discussed at multidisciplinary case conferences involving the parents. The interpreted medical management has subsequently been displayed by the parents on an elaborate, up‐to‐date website, which is part of a larger ‘Mother Of Omphalocele’ network. Although innovative, this highlights the fact that we the medical profession, should be vigilant with regard to potential public exposure of patient management. Whilst the Internet has become an integral part of our own continuing education, this case highlights a new aspect of how it may be used by our patients and their relatives to compare and contrast management policies in various institutions.