Abstract Introduction Omphaloceles are midline, abdominal wall defects where an organ-containing membranous sac extrudes from the abdominal cavity. Increasing intra-abdominal pressures resulting from reduction of giant omphaloceles (GO) may result in significant respiratory compromise. Pulmonary complications can include left mainstem bronchus narrowing and consensus is lacking regarding whether this bronchial narrowing requires surgical intervention. We present a patient with GO, left mainstem bronchus compression and resultant tracheostomy and continuous ventilator dependence who was successfully decannulated without surgical intervention on the tracheobronchial tree. Case Presentation A late preterm female was born with GO containing intestines, liver, spleen and partial stomach. She underwent operative reduction of the omphalocele with resultant chronic respiratory failure requiring tracheostomy and ventilator support. Echocardiogram revealed hemodynamically significant secundum atrial septal defect (ASD) with left to right shunt, dilation of the main, left and right pulmonary arteries and right heart strain. Bronchoscopy demonstrated significant malacia and stenosis of the left mainstem bronchus with extrinsic pulsatile compression, likely by the left pulmonary artery (LPA) and aorta. The left mainstem required positive end expiratory pressure (PEEP) of 10 cmh2o to maintain patency. Staged abdominal surgical course was complicated by wound dehiscence delaying ASD closure. She tolerated slow weans in mechanical ventilatory support. Following ASD device closure at 4 years and 1 month of age, her left mainstem bronchus compression improved as evidenced by direct visualization on bronchoscopy and respiratory stability with decreasing PEEP. She tolerated day time tracheostomy capping and nocturnal continuous positive airway pressure. At 4 years and 8 months old, airway evaluation demonstrated improvement in malacia. She was successfully decannulated. Two months later, her tracheostoma had healed and she did not require any respiratory support. Discussion Existing case reports of left sided bronchial abnormalities in GO theorize that a narrow and elongated thoracic cavity and omphalocele sac manipulation lead to distortion of the bronchus. Staged abdominal closure has been proposed to mitigate risk. This case demonstrates persistent ventilatory dependence complicated by left mainstem stenosis persisting beyond sac reduction. There is no consensus regarding treatment of left mainstem narrowing in these patients. Closure of the hemodynamically significant ASD and subsequent relative constriction of the LPA decreased external bronchial compression resulting in improvement of left mainstem bronchus narrowing allowing for reduction in PEEP and successful deccanulation without surgical intervention on the bronchus. Cardiac defects in the setting of GO must be considered when patients continue to require invasive respiratory support. This abstract is funded by: None
Hoffman et al. (Fri,) studied this question.