Miller A R, Nagorney D M, Sarr M G
Department of Surgery, Mayo Clinic, Rochester, Minnesota 55906.
Ann Surg. 1992 Jan;215(1):39-43. doi: 10.1097/00000658-199201000-00006.
The role of operative intervention for hereditary pancreatitis, a rare form of chronic parenchymal destruction, is unclear. To determine whether surgical therapy is safe and provides prolonged symptomatic relief, the authors reviewed the management of 22 adults (11 men, 11 women) with hereditary pancreatitis treated surgically between 1950 and 1989. Hereditary pancreatitis was defined as a family history of two or more relatives with pancreatitis and clinical, biochemical, or radiologic evidence of pancreatitis. The mean ages at onset of symptoms and at operation were 15 years (range, 3 to 52 years) and 31 years (range, 18 to 54 years), respectively. Pain was the primary indication for operation in all patients. Additional symptoms included nausea, vomiting (73%), weight loss (55%), and diarrhea (41%). Ductal dilatation was present in 68%, pancreatic parenchymal calcifications in 73%, pseudocysts in 36%, and splenic vein thrombosis in 18%. Primary operations included ductal drainage in 10 patients, pancreatic resection alone in three, resection with drainage in three, cholecystectomy plus sphincteroplasty in two, cholecystectomy with or without common bile duct exploration in two, pancreatic abscess drainage in one, and pseudocyst drainage in one. There were no perioperative deaths, and the morbidity rate was 14% (intra-abdominal abscess, wound infection, and urinary tract infection). Symptoms recurred in nine patients. Severity prompted reoperation in five. Secondary operations included pancreatic resection in three, pseudocyst excision in one, and pancreaticolithotomy in one. Follow-up to date is complete and extends for a median of 85 months. Eighteen patients (82%) are clinically improved or asymptomatic. Symptoms have persisted in four patients, and two patients have died of pancreatic carcinoma. Two patients died of unrelated causes. Surgical therapy for patients with hereditary pancreatitis selected on the basis of the traditional indications for surgical treatment of chronic pancreatitis is safe and efficacious.
手术干预对于遗传性胰腺炎（一种罕见的慢性实质破坏形式）的作用尚不清楚。为了确定手术治疗是否安全并能提供长期的症状缓解，作者回顾了1950年至1989年间接受手术治疗的22例成年遗传性胰腺炎患者（11名男性，11名女性）的治疗情况。遗传性胰腺炎定义为有两名或更多亲属患胰腺炎的家族史以及胰腺炎的临床、生化或放射学证据。症状出现时的平均年龄和手术时的平均年龄分别为15岁（范围3至52岁）和31岁（范围18至54岁）。疼痛是所有患者手术的主要指征。其他症状包括恶心、呕吐（73%）、体重减轻（55%）和腹泻（41%）。68%的患者存在导管扩张，73%有胰腺实质钙化，36%有假性囊肿，18%有脾静脉血栓形成。初次手术包括10例患者进行导管引流，3例单纯胰腺切除术，3例切除加引流术，2例胆囊切除术加括约肌成形术，2例胆囊切除术伴或不伴胆总管探查术，1例胰腺脓肿引流术，1例假性囊肿引流术。无围手术期死亡病例，发病率为14%（腹腔内脓肿、伤口感染和尿路感染）。9例患者症状复发。其中5例因症状严重而再次手术。二次手术包括3例胰腺切除术，1例假性囊肿切除术和1例胰管结石取出术。目前随访完整，中位随访时间为85个月。18例患者（82%）临床症状改善或无症状。4例患者症状持续存在，2例患者死于胰腺癌。2例患者死于无关原因。基于慢性胰腺炎传统手术指征选择的遗传性胰腺炎患者的手术治疗是安全有效的。
