Nesidioblastosis and Subsequent Diabetes Mellitus in a Dog with Hyperinsulinemic Hypoglycemia Treated with Partial Pancreatectomy.

A 6.5 yr old castrated male mixed-breed dog was presented for clinical signs associated with hypoglycemia. Hyperinsulinemic hypoglycemia was diagnosed as the cause of the persistent hypoglycemia. No obvious pancreatic mass was seen on abdominal computed tomography and exploratory laparotomy. A partial pancreatectomy was performed with the suspicion of an insulinoma-causing hyperinsulinemic hypoglycemia. Nesidioblastosis was diagnosed based clinical, biochemical, and histopathologic findings. There was beta cell hyperplasia and no evidence of neoplasia. The dog was euglycemic postoperatively after a partial pancreatectomy. Long-term follow-up after 2 yr revealed that the dog was diagnosed with diabetes mellitus.

Masa pancreática: una presentación inhabitual de nesidioblastosis en el adulto / Pancreatic mass: an unusual presentation of nesidioblastosis in adults

A 41-year-old woman, multiparous of 4, with personal or familiar history of diabetes, with a history of Nissen fundoplication due to pathological gastro-oesophageal reflux, is refer to an endocrinologjst during her post-operatiye follow up, 4 months after her surgery for a 14 kg weight loss in 10 months and symtomatic hypoglycemia to repetition. A positive prolonged fasting test for hypoglycemia was performed. In addition, abdominal computed axial tomography was performed, which resulted normal and endosonography, showing a lesion on the head of the pancreas. Octreoscan positive for pancreatic head focal lesion with positive somatostatin receptors compatible with insulinoma. Whipple surgery was performed where surgeon palpated pancreatic tumor, biopsy showed tissue compatible with diffuse nesidioblastosis. In the postoperative period, the patient decreased frequency and intensity of hypoglycemic episodes compared to their previous stage. Control prolonged fasting test and Octreoscan were within normal ranges. However, 4 months after surgery, the patient presented hypoglycemia of lower intensity and frequency than before surgery. Currently he remains in control with nutritionist and endocrinologist, mainly adjusting diet and with good control glycemias.

Recidiva de fibroadenoma juvenil en paciente afecta de síndrome de Beckwith-Wiedemann / Recurrence of juvenile fibroadenoma in a patient with Beckwith-Wiedemann syndrome

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Síndrome de hipoglucemia por hiperinsulinismo endógeno: tratamiento quirúrgico / Endogenous hyperinsulinemic hypoglycemia syndrome: Surgical treatment

INTRODUCCIÓN: El síndrome hipoglucémico por hiperinsulinismo endógeno (SHHE) puede estar originado por un insulinoma o, menos frecuentemente, por la nesidioblastosis en niños, conocida en la población adulta con el nombre de síndrome hipoglucémico pancreático no insulinoma (SHPNI). El objetivo de este trabajo es mostrar la estrategia para el tratamiento quirúrgico del SHHE. MATERIAL Y MÉTODO: Se incluyó a un total de 19 pacientes con diagnóstico final de insulinoma o SHPNI que fueron tratados quirúrgicamente desde enero del 2007 hasta junio del 2012. Se describió la forma de presentación clínica y estudios preoperatorios. Se hizo hincapié en la técnica quirúrgica, las complicaciones y el seguimiento a largo plazo de los pacientes. RESULTADOS: Todos los pacientes en estudio tuvieron un test de ayuno positivo. Las lesiones que originaron el SHHE pudieron ser localizadas preoperatoriamente en el 89,4% de los casos. La cirugía más frecuente fue la pancreatectomía distal con preservación de bazo (9 casos). Tres pacientes con diagnóstico de insulinoma se presentaron con metástasis sincrónicas, que fueron tratadas con cirugía simultánea. No tuvimos mortalidad perioperatoria y la morbilidad fue del 52,6%. El análisis histológico reveló que 13 pacientes (68,4%) presentaban insulinoma benigno, 3 insulinoma maligno con metástasis hepáticas y 3 con diagnóstico final de SHPNI. La mediana de seguimiento fue de 20 meses. Todos los pacientes con diagnóstico de insulinoma benigno o SHPNI resolvieron el síndrome de SHHE. CONCLUSIÓN: El tratamiento quirúrgico del SHHE logra excelentes resultados a largo plazo en el control de los síntomas de hipoglucemia

BACKGROUND: The endogenous hyperinsulinemic hypoglicemia syndrome (EHHS) can be caused by an insulinoma, or less frequently, by nesidioblastosis in the pediatric population, also known as non insulinoma pancreatic hypoglycemic syndrome (NIPHS) in adults. The aim of this paper is to show the strategy for the surgical treatment of EHHS. MATERIAL AND METHODS: A total of 19 patients with a final diagnosis of insulinoma or NIPHS who were treated surgically from January 2007 until June 2012 were included. We describe the clinical presentation and preoperative work-up. Emphasis is placed on the surgical technique, complications and long-term follow-up. RESULTS: All patients had a positive fasting plasma glucose test. Preoperative localization of the lesions was possible in 89.4% of cases. The most frequent surgery was distal pancreatectomy with spleen preservation (9 cases). Three patients with insulinoma presented with synchronous metastases, which were treated with simultaneous surgery. There was no perioperative mortality and morbidity was 52.6%. Histological analysis revealed that 13 patients (68.4%) had benign insulinoma, 3 malignant insulinoma with liver metastases and 3 with a final diagnosis of SHPNI. Median follow-up was 20 months. All patients diagnosed with benign insulinoma or NIPHS had symptom resolution. CONCLUSION: The surgical treatment of EHHS achieves excellent long-term results in the control of hypoglucemic symptoms

Pancreatectomía proximal en nesidioblastosis / Proximal pancreatectomy in case of nesidioblastosis

En 1938 Laidiaw acuñó el término de nesidioblastosis refiriéndose a una neodiferenciación de los islotes de Langerhans, originada del epitelio de los conductos pancreáticos. Se presenta un caso clínico de un niño de 19 meses de edad, con convulsiones secundarias a hipoglucemia intratable. Se diagnosticó hiperinsulinismo con base en criterios clínicos y bioquímicos. Los hallazgos histopatológicos del páncreas mostraron la presencia de racimos de islotes de células P por todo el tejido acinar localizados en cabeza y un tercio proximal del cuerpo. Se realizó el diagnóstico de nesidioblastosis y para su tratamiento se practicó pancreatectomía proximal (60%), con una pancreático yeyunostomía distal (Y en Roux). Después del tratamiento, el paciente tuvo normalización de los niveles séricos de glucosa y de insulina. Se concluye que este procedimiento resultó efectivo al revertir la hipoglucemia, por lo que constituye el primer tratamiento alternativo al método convencional.

A 19 month old child who presented seizures secondary to intractable hypoglycemia, fulfilling the clinical and biochemical criteria for hyperinsulinism was studied. Histopathological findings of the pancreas showed the presence of small clusters of b cell islets throughout acinar tissue near ducts, in both the head and the proximal third of the body. Proximal pancreatectomy (60%) and distal pancreatic jejunostomy (Roux in Y) were performed. This procedure was effective in reverting hypoglycemia and constitutes the first successful alternative treatment.