先天性高胰岛素血症手术治疗效果分析

The surgical treatment of congenital hyperinsulinism

目的分析手术治疗先天性高胰岛素血症(congenital hyperinsulinism,CHI)的有效性及安全性。方法2012年12月至2017年12月在广州市妇女儿童医疗中心行胰腺部分或次全切除术治疗的9例CHI患者,其中男6例,女3例,术前均行B超、CT和MR检查,予二氮嗪治疗7~10 d无效后行手术治疗,手术时日龄为32~163 d,术后进行随访观察。结果1例术前行18 F-DOPA PET-CT检查,考虑为局灶型CHI,但术中冰冻病理证实为非典型型CHI;术后石蜡病理证实局灶型CHI 1例,弥漫型CHI 7例,非典型型CHI 1例。1例非典型型CHI行胰头次全切除术和胰体尾-空肠吻合术,术后只需三餐外定期口服果汁即可维持血糖于正常水平。1例局灶型CHI患者行95%胰腺次全切除后,出院时血糖波动于4.2~7.2 mmol/L之间,不需药物治疗,该患者随访至今,正常饮食情况下均可维持正常血糖。7例弥漫型CHI患者均行胰腺次全切除术,其中1例行90%胰腺切除的患者出院时仍有间断性低血糖,需口服二氮嗪才能控制,术后第2年发展为Ⅰ型糖尿病,目前需要口服药物降低血糖;1例行95%胰腺切除的患者出院后,在饮奶前或夜间偶有低血糖,添加糖水喂养方可控制,术后第3年发展为Ⅰ型糖尿病,目前需要用胰岛素泵降低血糖;其余5例均行95%胰腺切除,出院时无低血糖发作,血糖波动于3.9~10 mmol/L之间,无需药物治疗。目前5例均正常饮食,血糖正常,无低血糖或糖尿病发生。结论对二氮嗪等药物治疗无效的CHI患者需要采用手术治疗。18 F-DOPA PET-CT对非典型型CHI的诊断价值需要进一步探讨。弥漫型CHI患者行胰腺次全切术可有效控制血糖,部分患者可获得正常血糖,但部分患者会发展为Ⅰ型糖尿病,尤其是术后短期仍出现低血糖发作的患者,发生糖尿病的可能性更大。对大多数弥漫型CHI患者行95%胰腺次全切是合适的。术前PET-CT对鉴别局灶型CHI有重要意义,术前PET-CT和术中冰冻病理检查有助于明确CHI的分型,指导选择正确的手术方式。

Objective The purpose of the study is to discuss the strategy,effectiveness and safety of surgical treatment for CHI patients by using the clinical data in our hospital retrospectively.Methods From December 2012 to December 2017,a total of 9 patients with CHI underwent partial or subtotal pancreatectomy in Guangzhou Women and Chilren's Medical Center,including 6 males and 3 females.Preoperative ultrasonography,CT and MR examinations were performed on all of them,and the age of operation ranged from 32 days to 163 days.Results 1 patient was considered as focal CHI by preoperative 18 F-DOPA PET-CT examination,but was confirmed as atypical CHI by intraoperative frozen pathology.1 case of focal CHI,7 cases of diffuse CHI and 1 case of atypical CHI were confirmed by postoperative paraffin pathology.After partial pancreatectomy for atypical type,the patient only needs regular intake of juice and starch to maintain normal blood glucose except for three meals now.The patient with focal CHI underwent 95%subtotal pancreatectomy,and he needed no drugs at discharge with his blood glucose fluctuating between 4.2 and 7.2mmol/L.At present,the patient has a normal diet and maintains normal blood glucose.All 7 diffuse CHI patients underwent subtotal pancreatectomy.1 patient who underwent 90%pancreas resection still had intermittent hypoglycemia at discharge and needed oral diazoxide to control her blood glucose.She developed type 1 diabetes 2 years after surgery and needs oral drugs to lower her blood glucose now.At discharge,1 patient who underwent 95%pancreas resection had occasional hypoglycemia before milk drinking or at night,requiring additional sugar water feedings to control her blood glucose.She developed type 1 diabetes in the third postoperative year and needs an insulin pump to lower blood glucose until now.The remaining 5 patients underwent 95%pancreatectomy and were fed normally at discharge without episodes of hypoglycemia.Their blood glucose fluctuated between 3.9 and 10mmol/L without drug treatment.At present,all the 5 patients have normal diet and no hypoglycemic episode or diabetes.Conclusion In our study,patients with CHI who have failed to respond to medication such as diazoxide may be treated surgically.The diagnostic value of 18 F-DOPA PET-CT in atypical CHI needs to be further explored.Patients with diffuse CHI can effectively control blood glucose by subtotal pancreatectomy,and some of them can obtain normal blood glucose,but some of them will develop into type 1 diabetes,especially those who still have hypoglycemic episodes in the short term after the surgery have a higher possibility of developing diabetes.A 95%subtotal pancreatectomy is appropriate for most patients with diffuse CHI.Preoperative PET-CT is of great significance for identifying focal CHI.Preoperative PET-CT and intraoperative frozen pathology examination can confirm the classification of CHI and guide the surgical procedure.