Food Protein Induced Enterocolitis Syndrome Presenting With Life-Threatening Methemoglobinemia: A Case Report and Review of the Literature

Abstract

Food protein induced enterocolitis syndrome (FPIES) can present with diarrhea, hypovolemia and electrolyte imbalance in infancy. We present a case of life-threatening methemoglobinemia in a 1-month-old infant, a rare complication of FPIES triggered by cow’s milk protein intake. A previously healthy 1-month-old boy presented with lethargy, increased work of breathing and 2-day history of vomiting. Review of systems revealed a 3-week history of diarrhea. He was lethargic, shocky and dusky, and was intubated for persistent hypoxia. His blood work revealed severe acidemia with pH of 6.95 and methemoglobin level of 66% (normal range < 3%). His methemoglobin level and clinical status normalized following volume resuscitation, packed red blood cell transfusion and prompt intravenous methylene blue administration. Further investigations revealed a diagnosis of FPIES which was managed with a hypoallergenic formula. Methemoglobinemia should be considered in young infants presenting with severe vomiting and diarrhea, secondary to dietary protein intolerance syndromes. Prompt management with methylene blue and fluid resuscitation can result in excellent prognosis, along with specific ongoing management for FPIES. Int J Clin Pediatr. 2020;9(2):35-40 doi: https://doi.org/10.14740/ijcp366