Spagnoletti Gionata, Larghi Laureiro Zoe, Maria Fratti Alberto, Maiorana Arianna, Garganese Maria Carmen, Pizzoferro Milena, Dionisi-Vici Carlo, Spada Marco
Division of Hepatobiliopancreatic Surgery, Liver and Kidney Transplantation, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Italy.
Division of Metabolic Diseases, Department of Pediatric Specialties, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Italy.
Front Surg. 2023 Jan 30;9:1085238. doi: 10.3389/fsurg.2022.1085238. eCollection 2022.
In focal congenital hyperinsulinism (CHI), surgery is the gold standard of treatment, even for lesions localized in the head of the pancreas. We report the video of the pylorus-preserving pancreatoduodenectomy performed in a five-month-old child with focal CHI.
Baby was placed in the supine position with both arms outstretched to the up. After transverse supraumbilical incision and mobilization of ascending and transverse colon, exploration and multiple biopsies of the tail and the body of the pancreas ruled out multifocality. Pylorus-preserving pancreatoduodenectomy was performed according to the following steps: extended Kocher maneuver, followed by retrograde cholecystectomy and common bile duct isolation; division of the gastroduodenal artery and of the gastrocolic ligament; division of the duodenum, Treitz ligament and jejunum; transection of the pancreatic body. The reconstructive time was with: pancreato-jejunostomy; hepaticojejunostomy; pilorus-preserving antecolic duodeno-jejunostomy. The anastomoses were accomplished with synthetic absorbable monofilament sutures; two drains were placed close to the biliary and pancreatic anastomoses and to the intestinal anastomosis, respectively. Total operative time was 6 h, with no blood loss and/or intra-operative complications, immediate normalization of blood glucose levels and discharge from surgical ward 19 days after surgery.
Surgical treatment of medical unresponsive focal forms of CHI is feasible in very small children: it is mandatory to refer the baby to a high-volume centre for a multidisciplinary management involving hepato-bilio-pancreatic surgeons and experts in metabolic disease.
在局灶性先天性高胰岛素血症（CHI）中，手术是治疗的金标准，即使对于位于胰头的病变也是如此。我们报告了一名5个月大患有局灶性CHI的儿童接受保留幽门胰十二指肠切除术的手术视频。
患儿仰卧位，双臂向上伸展。经脐上横切口并游离升结肠和横结肠后，对胰腺尾部和体部进行探查及多处活检以排除多灶性病变。保留幽门胰十二指肠切除术按以下步骤进行：扩大Kocher手法，随后逆行胆囊切除术及分离胆总管；切断胃十二指肠动脉和胃结肠韧带；切断十二指肠、Treitz韧带和空肠；横断胰体。重建步骤为：胰空肠吻合术；肝空肠吻合术；保留幽门结肠前十二指肠空肠吻合术。吻合口用合成可吸收单丝缝线完成；分别在胆管和胰管吻合口及肠吻合口附近放置两根引流管。总手术时间为6小时，无失血和/或术中并发症，血糖水平立即恢复正常，术后19天从外科病房出院。
对于药物治疗无效的局灶性CHI患儿，手术治疗是可行的：必须将患儿转诊至大型中心，由肝胆胰外科医生和代谢疾病专家进行多学科管理。
