Continuous Positive Airway Pressure Belly Syndrome: Challenges of a Changing Paradigm

Abstract

In extreme preterm infants, early use of continuous positive airway pressure (CPAP) for respiratory support reduces the incidence of chronic lung disease. However, as a sequel, inadvertent passage of air into the gastrointestinal tract leads to abdominal distension often with visibly dilated loops. The first description of “CPAP belly syndrome” in 1992, originates from the study in premature infants weighing less than 1,000 g and managed on nasal CPAP. The description of this phenomenon included benign episodic abdominal distension, with no associations to feed intolerance, and no radiological evidence of bowel wall thickening, pneumatosis or free air. With improving perinatal care, lesser gestational age infants are increasingly managed on early CPAP with resultant more frequent occurrence of CPAP belly syndrome. When extreme preterm infants on CPAP develop tense, marked abdominal distension, clinical decisions to cease feeds, administer empiric antibiotics and perform plain abdominal radiographs are all justified to screen for potentially serious causes. With rampant use of non-invasive respiratory support in extreme preterm infants, the occurrence of severe CPAP belly syndrome now extends to include clinical scenario mimicking a “necrotizing enterocolitis (NEC) scare”. We present the case of an extreme preterm infant with severe CPAP belly syndrome that required rescue intubation due to a massively distended abdomen. The emergency management included change to invasive ventilation and exclusion of serious intestinal conditions such as NEC. In retrospect, the life-threatening marked abdominal distension was due to severe CPAP belly syndrome, contrary to its well-recognized benign description, three decades ago. The clinical paradigm of CPAP belly syndrome is evolving, and in its severe form in extreme preterm infants, warrants vigilant monitoring to differentiate it from severe progressive intestinal conditions, such as NEC. Further research is required to describe its causes, associated morbidities and the need to evaluate the utility of other diagnostic modalities to reassure clinicians. Int J Clin Pediatr. 2020;9(1):9-15 doi: https://doi.org/10.14740/ijcp352