ABSTRACT
El hiperinsulinismo congénito (HC) es la causa más frecuente de hipoglicemia persistente del recién nacido (RN), y se caracteriza por secreción no regulada de insulina frente a hipoglicemia. El manejo inicial es médico, pero en caso de fracaso, la pancreatectomía parcial es el tratamiento quirúrgico de elección. Objetivo: Comunicar el caso de un RN con HC con énfasis en el estudio genético, de imágenes y manejo quirúrgico, incluyendo la cirugía laparoscópica. Caso clínico: RN de término, con hipoglicemia prolongada por HC que no respondió al tratamiento médico. La ultrasonografía y resonancia nuclear magnética abdominal fueron normales. PET/CT Galio 68 evidenció aumento de metabolismo glucídico focalizado en el cuerpo pancreático. Se realizó pancreatectomía córporo-caudal laparoscópica sin incidentes, con mejoría de la hipoglicemia. El estudio genético fue compatible con HC focal, confirmado luego por biopsia. Conclusión: Se expone acerca de las características clínicas y diagnóstico del HC, en particular el rol de la cirugía y ventajas del abordaje laparoscópico.
Congenital hyperinsulinism (CH) is the most frequent cause of persistent hypoglycemia in the newborn and it is characterized by an inappropriately elevated insulin level in presence of hypoglycemia. Initial management is medical treatment, but if it fails, partial pancreatectomy is the surgical procedure of choice. Objective: To report a case of a newborn with CH to aware on this condition and update its multidisciplinary management. Case report: The case of a full term newborn with severe symptomatic hypoglycemia unresponsive to medical treatment is reported . Ultrasonography and abdominal MRI were normal, Gallium 68 PET/CT showed uptake in the body of the pancreas. Laparoscopic corporocaudal pancreatectomy was performed. The patient resolved hypoglycemia and was discharged without incidents. Genetic study was consistent with focal CH, confirmed later by biopsy. Discussion: This case report exposes the clinical presentation of CH and its diagnosis. It emphasizes on the surgical treatment and the advantages of minimally invasive technique.
Subject(s)
Humans , Male , Infant, Newborn , Congenital Hyperinsulinism/diagnosis , Pancreatectomy/methods , Congenital Hyperinsulinism/surgeryABSTRACT
Hyperinsulinemia, diagnosed by laboratory tests, should be diagnosed and treated as soon as possible to prevent fatal complications such as neurological damage. Patients who are resistant to medical therapy should be treated surgically. Minimally invasive surgery, a newly developed approach, is a good choice among surgical procedures to avoid unnecessary extensive pancreatectomy. Here, a 12-year-old boy is presented with diagnosis of hyperinsulinemic hypoglycemia who had recurrent attacks of hypoglycemia and seizures from infancy. Because of his unresponsiveness to medical therapy and his family’s preference, he underwent laparoscopic pancreatectomy to reduce morbidity and hospital stay. Two years postsurgical follow-up revealed a normo-glycemic state.
Subject(s)
Child , Humans , Male , Congenital Hyperinsulinism/surgery , Pancreas/pathology , Congenital Hyperinsulinism/pathology , Hyperplasia/pathology , Laparoscopy/methods , Overweight , Pancreatectomy/methods , Treatment OutcomeABSTRACT
Persistent neonatal hyperinsulinism is the most common cause of refractory hypoglycemia during the first year of life. Inadequate insulin secretion is associated to mutations of four different genes, that can be diagnosed to orient patient management. We report two patients: a female newborn that presented a hypoglycemia of 16 mg/dl two hours after birth, was subjected to a subtotal pancreatectomy that did not correct hypoglycemia, requiring a total pancreatectomy. Pathological study of the pancreas showed a focal adenomatous hyperplasia. At the present time, she is three years of age and maintains euglycemia with fractionated feeding. A male newborn that had seizures at 28 hours of life and a hypoglycemia of 15 mg/dl was detected. He was also subjected to a subtotal pancreatectomy, that did not correct hypoglycemia and had to be extended to a total pancreatectomy. At the present time, he is 3 years and 11 months of age and has a normal psychomotor development (Rev Méd Chile 2004; 132: 995-1000).