Peutz-Jeghers综合征24例临床分析

Clinical analysis of 24 patients with Peutz-Jeghers syndrome

目的分析Peutz—Jeghers综合征的临床特征，采用合理治疗手段，改善患者的生存质量。方法分析空军总医院2004年1月～2008年4月收治的24例Peutz—Jeghers综合征患者的临床资料，胃、结肠息肉可在胃镜、肠镜下给予切除；小肠息肉可在双气囊小肠镜检查时给予切除；较大无蒂息肉手术切除，并存术中配合内镜检查。结果24例患者在口唇、口腔颊黏膜、手指、足趾末端均表现有散在分布黑／褐色斑，阵发性腹痛为突出表现（24例），最早腹痛发病年龄5岁，伴腹胀，血便或无排便、排气，息肉主要分布于胃、空回肠、结肠、直肠共20例，主要分布于结肠2例，胃2例。息肉为无蒂、亚蒂、长蒂息肉，呈蘑菇状、分叶状或菜花状，部分息肉顶端黏膜糜烂、溃疡。术后第3天发生便血4例，小肠穿孔行手术修补治疗1例。病理证实错构瘤22例，十二指肠黏液腺癌1例，空肠黏液性腺癌1例。结论Peutz—Jeghers综合征患者应定期复查胃镜、小肠镜、结肠镜，3cm以下息肉行镜下切除，〉3cm无蒂或亚蒂息肉或可疑癌变倾向患者行手术治疗，术中配合内镜检查进一步切除息肉是治疗Peutz—Jeghers综合征的有效方法。

Objective To analyze the clinical characteristics of Peutz-Jeghers syndrome in order to apply rational therapy and to improve patients＇ living quality. Methods The clinical characteristics of 24 patients with Peutz-Jeghers syndrome （PJS）who admitted in air-force general hospital from January 2004 to April 2008 were analyzed retrospectively. The gastric polyp, colonic polyp and proctopolypus were resected under the gastroscope or colonoscope. The polyps of small intestine were resected under the double-balloon enteroscopy. Comparatively large sessile polyps should be operated meanwhile combined with colonoscope. Results Twenty-four patients had distinct perioral or extremities of limbs blue/black freckling, and paroxysmal abdominal pain was outstanding clinical sign. The age of onset of abdominal pain was about 5 years old. Most of them were accompanied with abdominal distention, bloody stools or without defaecation and venting. Most polyps were found in stomach, jejunoileum, colon, and rectum in 20 patients, only 2 patients the most polyps were found in colon and 2 patients in stomach. Its shapes were macro stem, inferior or sessile polyps, some were mushroom or cauliflower, and some polyps were erosion or ulceration. Among these patients, only 4 patients had hemafecia and 1 patients had perforation of intestine after resection 3 days. Twenty-two patients were hamartomatous polyps, one patient was jejunum adenocarcinoma and 1 patient was duodenum adenocarcinoma. Conclusion The patients suffered from Peutz-Jeghers syndrome should be regularly examined by gastroscope, colonoscope and double-balloon enterscopy. The size of polyps under 3 cm can be resected under the gastroscope or colonoscope. On the contrary, upper 3 cm inferior or sessile polyps especially some doubtful cancerization polyps must be operated. The more effective method to cure Peutz-Jeghers syndrome is combined with endoscope when patients were operated in order to resect the polyps of small intestine.