Giant omphalocele: current perspectives

Abstract

Giant omphalocele (GO) is a congenital ventral abdominal wall defect characterized by a large opening with herniated abdominal organs, including liver, loss of abdominal cavity volume, and other associated congenital anomalies. Treatment of patients with GO represents a major challenge for involved caregivers. Despite significant improvements in neonatal intensive and surgical care over the last decades, the condition is still associated with high mortality rates and a high risk of severe morbidity in survivors. The principles of the earliest attempts to treat GO surgically and conservatively are still easily recognized in the main approaches used today. In this review, we discuss the more recent developments in the treatment of GO, including perioperative management and associated morbidities of the condition.

Giant Isolated Omphalocele: Role of Prenatal Diagnosis in Prognostic Asessment and Perinatal Management

“…Regarding surgery, early surgical closure using patient's own tissues is proposed as the most appropriate nowadays [8,12,13], as it has lower infection rate and better chances of early enteral feeding than staged and delayed surgical repair, though there is a higher risk of abdominal compartment syndrome due to the increase of abdominal pressure after closure. In order to predict and plan the best surgical procedure, different prenatal indices have been proposed, such as the relationship between fetal biometric measurements like abdominal circumference and the diameter of the omphalocele [10,21].…”

“…e size of the omphalocele is also important. Although there is no consensus on the prenatal classification, a giant omphalocele is generally considered when the sac is larger than 5 cm [8,9]. e relevance of this assessment lies in the further associated complications: the larger the hernia, the greater the likelihood of pulmonary hypoplasia, abdominal compartment syndrome, and surgical repair challenge.…”

“…Omphalocele surgical treatment can be primary, staged, or delayed repair, depending on its size and content, the size of the baby, and the intra-abdominal pressure, which must be monitored during surgery to avoid abdominal compartment syndrome [8]. Primary treatment is that performed in the first hours or days of life and can be done using the patient's own tissues (muscles, skin, etc.).…”

“…Staged repair is used when the defect is too large to be closed by a primary closure; usually this surgery is performed in more than one step and Silo bags, tissue expanders, or meshes are used to enhance the volume of the abdominal cavity to host omphalocele content. Finally, delayed repair is that carried out months or even years after birth; it is based on the transformation of the omphalocele membrane into a neoskin containing the sac [8,11]. When possible, early surgical closure is the most appropriate treatment [8,12].…”

“…Finally, delayed repair is that carried out months or even years after birth; it is based on the transformation of the omphalocele membrane into a neoskin containing the sac [8,11]. When possible, early surgical closure is the most appropriate treatment [8,12]. A coordinated multidisciplinary team including Prenatal Diagnosis, Obstetrics, Neonatology, Pediatric Surgery, and Anesthesiology is essential to achieve success in the management of these patients [13].…”