Nesidioblastosis de páncreas: presentación de un caso / Pancreatic nesidioblastosis: Case report

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Functional imaging in hyperinsulinemic hypoglycemia after gastric bypass surgery for morbid obesity.

CONTEXT: Hyperinsulinemic hypoglycemia after Roux-en-Y gastric bypass (RYGB) has been increasingly reported. It is induced by ß-cell hyperplasia often referred to as nesidioblastosis. Positron emission tomography (PET) with [11C]-5-hydroxytryptophan ((11)C-HTP) and 6-[18F]fluoro-3,4-dihydroxy-l-phenylalanine ((18)F-DOPA) has been successfully applied to image neuroendocrine tumors. No data are available of the usefulness of these functional imaging techniques in post-RYGB in this new endocrine disorder, neither for diagnostic purposes nor for follow-up. OBJECTIVE: We present a patient with post-RYGB hypoglycemia who underwent (11)C-HTP and (18)F-DOPA PET scintigraphy for diagnostic purposes and to evaluate the effect of additional laparoscopic adjustable banding of the pouch as a surgical therapy for this disorder. PATIENT: We describe a woman with biochemically confirmed post-RYGB hypoglycemia who showed diffuse uptake of the (11)C-HTP and (18)F-DOPA tracers in the entire pancreas. After failure of dietary and medical treatment options, she underwent a laparoscopic adjustable banding for pouch dilatation. Subjective improvement was noted, which coincided with decreased uptake of (18)F-DOPA and (11)C-HTP in the head of the pancreas. CONCLUSIONS: Functional imaging by (18)F-DOPA- and (11)C-HTP-PET can accurately visualize diffuse endocrine pancreatic activity in post-gastric bypass hyperinsulinemic hypoglycemia. Both (11)C-HTP- and (18)F-DOPA-PET imaging appear to have a similar diagnostic performance in the presented case, and uptake of both tracers potentially relates to disease activity after surgical intervention.

Intraoperative sonography: a technique for localizing focal forms of congenital hyperinsulinism in the pancreas.

Congenital hyperinsulinism (CHI), syn. nesidioblastosis, is the most frequent cause of persistent, recurrent hypoglycemia in infancy. One third of patients show a single circumscribed focus. Enucleation of the focus and the removal of all affected ß-cells with preservation of healthy tissue is the treatment of choice. The intrapancreatic choledochus as well as the ductus pancreaticus major must remain intact. The diagnostic gold standard is 18F-DOPA-PET/CT. Intraoperative sonography is carried out to correctly visualize the focus preoperatively localized by PET/CT in situ during the operation. The enucleation of the focus was carried out 3 - 20 days after PET/CT in 5 patients at an age of 3.5 - 14 months. Intraoperative ultrasound was carried out with high-capacity devices of different manufacturers under use of broadband probes (9 - 14 MHz). The localization by intraoperative ultrasound was accurate in all 5 patients with focal CHI, with regard to the intraoperative localization as previously described by PET/CT and histology. D. choledochus and D. pancreaticus major were separated intraoperatively by ultrasound. 3 of 5 patients were cured by complete enucleation of the focus. Nevertheless, the entire intraoperative identification of the segmented focus is still problematic. Characteristic sonographic features of a CHI focus are: hypoechogenicity, variable homogeneous and inhomogenous texture, blurred, irregular limitation without capsule, filiform, lobular processes, and insular dispersal into the surrounding tissue. Intraoperative high-resolution sonography helps the pediatric surgeon to determine size, configuration and topography of a CHI focus.

Pancreatic regeneration after near-total pancreatectomy in children with nesidioblastosis.

BACKGROUND: Nesidioblastosis is often resistant to medical therapy and requires near-total pancreatectomy. There is little information on the postoperative imaging findings of these patients. OBJECTIVE: To demonstrate by US the late imaging findings in these patients. MATERIALS AND METHODS: Children diagnosed with nesidioblastosis and who had undergone 90-95% pancreatectomy received preoperative, immediate-postoperative (within 10 days of surgery) and long-term annual US examinations. In the preoperative study, three anterior-posterior (AP) measurements were obtained of the head, body and tail of the pancreas. In the postoperative and long-term follow-up US, AP and transverse measurements of the pancreatic remnant were obtained. Pancreatic echogenicity was also assessed. The results were compared with normal pancreatic dimensions as a function of age. Glucose metabolism and pancreatic enzymes were also analysed. RESULTS: The study group comprised 22 patients (aged 9 days to 2 years). The pancreas was normal in all preoperative US examinations. The first postoperative examination showed, in all patients, a remnant of the pancreatic head measuring 8-13 mm. The last follow-up US was similar to the first postoperative study in 6 patients, and 12 showed complete pancreatic regeneration (normal size, echogenicity and function), and 4 had incomplete regeneration with head and body normal in size, but lack of a pancreatic tail. All patients were asymptomatic and showed normal laboratory tests. CONCLUSIONS: US measurements indicated normal age-dependent growth after near-total resection in 54% of patients. The function and echogenicity of the regenerated pancreas indicate that the increase in organ size was due to normal pancreatic tissue.