[Localizing of Langerhans islets adenoma by transhepatic portal catheterization].

Archives francaises de pediatrie Pub Date : 1993-10-01
J Zeller, A Roche, J Rahier, C Adamsbaum, J C Carel, P Hélardot, P F Bougnères
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Abstract

Background: The procedures used to locate pancreatic endocrine tumors have only limited success in infants and children in whom the nodules may be small. Portal catheterization may therefore be useful.

Case report: A child aged 6 yrs 4 months was admitted because of several recent episodes of pallor and sweating associated with hypoglycemia. Further investigation showed moderate hyperinsulinemia and low blood levels of ketone bodies and branched amino-acids after a 15 hr fast. Celiac angiography was normal. The hypoglycemic episodes recurred despite treatment with diazoxide for 6 months. A transparietal portal catheterization was therefore performed. Selective pancreatic venous sampling showed high concentrations of insulin in two small veins draining one part of the head of the pancreas (117 and 89 microU/ml). The head of pancreas was removed 16 months later. Extemporaneous examination revealed an adenoma 0.8 cm in diameter. This patient has completely recovered, 8 months after surgery.

Conclusion: Transparietal portal catheterization can detect pancreatic areas with high insulin secretion. It may also help the interpretation of celiac angiographs in children, as diagnosis may be obscured by the normal rich vascularity of the pancreas in these patients.

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经肝门静脉导管定位朗格汉斯胰岛腺瘤
背景:用于定位胰腺内分泌肿瘤的手术在婴儿和儿童中成功率有限,因为他们的结节可能很小。因此,门静脉导管置入可能是有用的。病例报告:一名6岁4个月的儿童因最近几次发作的苍白和出汗与低血糖而入院。进一步的调查显示,禁食15小时后,中度高胰岛素血症和低血酮体和支链氨基酸水平。腹腔造影正常。治疗6个月后,再次出现低血糖发作。因此进行了经门静脉导管置入。选择性胰静脉取样显示胰腺头部部分的两条小静脉中胰岛素浓度较高(117和89微u /ml)。16个月后,胰头被切除。临时检查显示一个直径0.8 cm的腺瘤。术后8个月,患者已完全康复。结论:经门静脉门静脉置管可发现胰岛素分泌高的胰腺区。这也有助于解释儿童的乳糜泻血管造影,因为这些患者的胰腺正常丰富的血管可能使诊断模糊不清。
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