ABSTRACT
OBJECTIVES: The objectives of the study were to provide a review of patients with giant omphalocele managed in a single institution (2001-2006), focusing on medical management in the neonatal period, and to evaluate short-term outcomes. METHODS: Data from 14 neonates with giant ompholocele (abdominal wall defect >5 cm and/or containing liver) and the absence of malformation and chromosomal anomalies during fetal screening were retrospectively reviewed. All were intubated and sedated before surgical treatment. Initial management consisted of progressive reduction of the herniated organs by gentle compression. After sequential reduction, abdominal wall closure was attempted at the skin and fascia level and, when necessary, with a Gore-Tex patch. RESULTS: Median gestational age was 39 weeks (38-40), and median birth weight was 3100 g (2470-3700). Median age at closure was 6 days (0-20). A central Gore-Tex patch was inserted in 10 cases. Median ventilation length was 26 days (2-78). Full enteral diet was achieved after an average of 33 days (8-82), and median time until discharge from the intensive care unit was 24.5 days (11-85). Nine patients developed sepsis in the postoperative course. In 10 patients, at least 1 associated malformation was diagnosed in the postnatal course, among which cardiac and diaphragmatic defects were the most common. Survival rate was 85.7%. CONCLUSION: Mortality rate of giant omphalocele without chromosomal anomaly or major malformations is low when treated by gradual reduction of the contents. Parents should be informed of the long hospitalization in the intensive care unit at birth, the potential nonthreatening associated malformations to be diagnosed after birth, and the high risk of sepsis.
Subject(s)
Abnormalities, Multiple/therapy , Hernia, Umbilical/surgery , Hernia, Umbilical/therapy , Abnormalities, Multiple/surgery , Adolescent , Adult , Female , Gestational Age , Hernia, Umbilical/mortality , Humans , Infant , Infant, Newborn , Intensive Care, Neonatal/methods , Length of Stay , Male , Maternal Age , Postoperative Complications/etiology , Pregnancy , Plastic Surgery Procedures/methods , Retrospective Studies , Sepsis/etiology , Treatment Outcome , Ultrasonography, PrenatalABSTRACT
The aim of this study is to analyze neonatal outcome of isolated congenital diaphragmatic hernia and to identify prenatal and postnatal prognosis-related factors. A retrospective single institution series from January 2000 to November 2005 of isolated congenital diaphragmatic hernia neonates was reviewed. Respiratory-care strategy was early high-frequency oscillatory ventilation, nitric oxide in pulmonary hypertension, and delayed surgery after respiratory and hemodynamic stabilization. Survival rate at 1 month was 65.9%. None of the prenatal factors were predictive of neonatal outcome, except an intra-abdominal stomach in left diaphragmatic hernia. Preoperative pulmonary hypertension was more severe in the nonsurvivor group and was predictive of length of ventilation in the survivors. During the first 48 hours of life, the best oxygenation index above 13 and the best PaCO2 above 45 were predictive of poor outcome. When treating isolated congenital diaphragmatic hernia with early high-frequency ventilation and delayed surgery but excluding extracorporeal membrane oxygenation, survival rates compare favorably with other reported series, and the respiratory morbidity is low.
