A Nutritional Conundrum: Feeding Options in Neonatal Congenital Lipodystrophy

Background Neonatal presentation of congenital lipodystrophy is rare. The nutritional management of high triglyceride (TG) levels in neonates is mostly derived from familial hypertriglyceridemia conditions. We describe a neonatal presentation of familial congenital lipodystrophy and the challenges we faced in optimizing nutritional management. Case Description A neonate presented with phenotypical features of lipodystrophy, including sub-optimal weight gain, prominent musculature, and lack of subcutaneous fat. TG levels were high. The unit and the family made a shared decision to continue breastfeeds. The risks of acute pancreatitis, stroke, and lipemia retinalis were concerns associated with continuing the “high-fat” diet of breastmilk. Fortunately, with close follow-up and monitoring, the infant’s TG values serially reduced, and growth is adequate with normal development. Conclusion Nutritional management of neonatal hypertriglyceridemia requires a case to case approach, especially if the condition is not due to familial hyperlipidemia states. These should focus on informed decisions based on the benefits of breastfeeding over fat-free formula.