Introduction: Post-prandial hyperinsulinaemic hypoglycaemia (PPHH) is a recognised complication of various gastric surgeries in children, but rarely reported after oesophageal atresia repair. We report two children diagnosed with PPHH post-oesophageal surgery and the challenges of their management.
Case 1: A 2-year-old boy diagnosed with oesophageal atresia at birth, was surgically repaired requiring six oesophageal dilatations the first year of life. At 11-months of age he manifested hypoglycaemic seizures and investigations confirmed PPHH. Acarbose and diazoxide trials failed. He was managed with 17-hours continuous gastrostomy feeds. Currently, he is 28-months-old with euglycaemia on daytime bolus gastrostomy feeds and overnight 12-hours continuous gastrostomy feeds.
Case 2: A 6-month-old girl diagnosed with Wolf-Hirschhorn syndrome and tracheo-oesophageal fistula, was surgically repaired, requiring monthly oesophageal dilatations. At 5-months of age she was reported to have hypoglycaemia and PPHH was confirmed. She responded to diazoxide and continuous nasogastric tube feeds, but developed pulmonary hypertension possibly diazoxie-induced. Subsequently diazoxide was stopped and normoglycaemia was secured via 20-hours continuous gastrostomy feeds.
Conclusion: PPHH may be an under-diagnosed complication in children undergoing surgery for oesophageal atresia. These children must be monitored closely for symptoms of hypoglycaemia and if there are concerns must be screened for possible PPHH. Our cases demonstrate that continuous feeding regimens might be the only therapeutic option, until PPHH gradually lessens in intensity over time.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology