Prenatally diagnosed giant omphaloceles: short‐ and long‐term outcomes

Abstract

Respiratory and feeding problems were the most common neonatal and long-term medical management issues. Parents need to be counseled prenatally about the probability of multiple surgeries and long hospitalization following birth.

Fetal MRI-Calculated Total Lung Volumes in the Prediction of Short-Term Outcome in Giant Omphalocele: Preliminary Findings

“…It is generally classified into three groups (small, giant, or ruptured) depending on the size of the abdominal protrusion and/or its contents [2,3]. Giant omphalocele (GO) is defined as a large central membrane-covered defect containing most of the liver (>75%) [4].…”

“…Over the last 2 decades, advances in surgical technique and neonatal intensive care have reduced the overall mortality for GO. Despite these improvements in survival, GO is associated with variable degrees of pulmonary hypoplasia which may lead to respiratory insufficiency, prolonged intensive care support, assisted ventilation, possible tracheostomy in order to maintain ventilation and oxygenation, and possible delayed abdominal wall closure [2,4,5]. In addition, children with GO have been reported to experience ongoing medical and surgical morbidities, including impaired musculoskeletal development, gastroesophageal reflux disease, nutritional problems, failure to thrive, and significant neurodevelopmental delays [2,4,5,6,7,8].…”

Congenital defects of abdominal wall

“…У пациентов с «гигантским» омфалоцеле существует большое количество проблем в от-даленном после операции периоде: гастроэзофагеальный рефлюкс (ГЭР), легочная недостаточность, повторяющие-ся инфекции легких, астма и дефицит питания [101]. Чем больше дефект, тем больше вероятность возникновения ГЭР.…”

Four wishes for Aubrey

“…Perhaps the wishes were engendered by past discussions that had addressed the possibility that Aubrey might not survive even this long, given the high morbidity and mortality associated with giant omphaloceles. 1 The wishes may have been hoped for during the past 5 months as efforts to provide family-centered care for Aubrey, and his family played out with variable success during any given week. There were always challenges, decisions and compromises as caregivers learned together with each passing day.…”