A. D. Menchaca1, 2, C. C. Style1, C. A. Mirhaidari1, 3, J. C. Xia1, T. A. Kyhl1, O. O. Olutoye1, 4 1Nationwide Children’s Hospital, Center For Regenerative Medicine, Abigail Wexner Research Institute, Columbus, OH, USA 2Indiana University School Of Medicine, Department Of General Surgery, Indianapolis, IN, USA 3The Ohio State University College of Medicine, Columbus, OH, USA 4The Ohio State University College of Medicine, Department Of Surgery, Columbus, OH, USA
Background: An omphalocele is a rare congenital anomaly characterized by failure of closure of the anterior abdominal wall. This leads to an abdominal wall defect (AWD)with amnion covered viscera outside of the abdomen. The clinical significance of this anomaly and its management vary considerably depending on the size of the defect. Small fascial defects result in umbilical cord hernias, whereas large defects result in giant omphaloceles with multiple organs outside the abdomen and loss of domain. Given the wide range of presentation, we sought to characterize our experience in management of this rare anomaly over an 11-year period.
Methods: We conducted a retrospective review of all patients evaluated for an abdominal wall defect at a single tertiary children’s hospital between January 2010 and January 2021. Infants with gastroschisis were excluded. Covered AWDs were classified as a cord hernia, small, or giant omphalocele. A giant omphalocele was defined as having a fascial defect >5cm or large amount of liver in the sac. Cord hernias were surgeon described or smaller than 2 cm. Data analyzed included the time to repair and whether the repair occurred during the index hospitalization. We also evaluated the presence of major associated structural, cardiac, or genetic abnormalities and syndromes.
Results: We identified 108 patients with covered AWDs. Of these, 37% (n=40) were cord hernias, 12% (n=13) small omphaloceles, and 51% giant omphaloceles (n=55). Major associated anomalies were present in 63% (n = 25) of cord hernias, 46% (n = 6) of small omphaloceles, and 24% (n = 13) of giant omphaloceles. In the case of cord hernias, major anomalies included intestinal atresia (5%, n = 2), Beckwith-Wiedemann syndrome (15%, n = 6), cardiac structural anomalies (48%, n = 19), and genetic anomalies (7.5%, n = 3). Percent repaired during index hospitalization and median days to repair from birth were as follows: 90% of cord hernias at a median of 1 day [1-353], 100% of small omphaloceles at a median of 1 day [0-6], 75% of giant omphaloceles at a median of 26.5 days [0-1929].
Conclusions: The range of clinical presentation of covered AWDs is broad, with a surprisingly high number of cord hernias seen at our institution. While cord hernias are typically associated with the least morbidity, a high percentage are associated with other intestinal anomalies that may warrant surgical exploration, not least of which are intestinal atresias. Furthermore, while the defect may be small, the association to other major congenital anomalies is quite high and must remain a critical part of workup and management.