Zeller J, Roche A, Rahier J, Adamsbaum C, Carel J C, Hélardot P, Bougnères P F
Service d'Endocrinologie, Hôpital St-Vincent-de-Paul, Paris.
Arch Fr Pediatr. 1993 Oct;50(8):675-80.
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.
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.
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.
用于定位胰腺内分泌肿瘤的方法在结节可能较小的婴幼儿中成功率有限。因此,门静脉插管可能有用。
一名6岁4个月的儿童因近期多次出现与低血糖相关的面色苍白和出汗而入院。进一步检查显示,禁食15小时后,存在中度高胰岛素血症,酮体和支链氨基酸血水平较低。腹腔血管造影正常。尽管用二氮嗪治疗6个月,低血糖发作仍复发。因此进行了经壁门静脉插管。选择性胰腺静脉采样显示,引流胰腺头部一部分的两条小静脉中胰岛素浓度较高(117和89微单位/毫升)。16个月后切除了胰腺头部。术中检查发现一个直径0.8厘米的腺瘤。该患者术后8个月已完全康复。
经壁门静脉插管可检测出胰岛素分泌高的胰腺区域。它还可能有助于解读儿童的腹腔血管造影,因为这些患者胰腺丰富的正常血管可能会掩盖诊断。