Clinical analysis of 31 cases of Pcutz-Jeghers syndrome

Abstract

Objective To investigate the clinical features, complications, treatment and prognosis of patients with Pcutz-Jeghers syndrome. Methods The clinical data of thirty-one cases of Pcutz-Jeghers syndrome treated in the First Hospital of Shanxi Medical University from January 1998 to December 2016 was analyzed retrospectively. Results There were11 cases with definite family history (35%, 11/31). There were 21 cases of mucocutaneous pigmentation (67.7%, 21/31), 31 cases of gastrointestinal polyps (100%, 31/31), including 8 cases of canceration (25.0%, 8/31), 24 cases of digestive tract bleeding (77.4%, 24/31), 20 cases of intussusception (64%, 20/31), and 1 case of intestinal perforation (3.2%, 1/31).6 cases were treated with endoscopic high frequency electrocoagulation, 12 cases received polypectomy, 17 cases underwent resection of intestinal tract.3 patients with intractable intussusception underwent plication of small intestine, and 4 patients with colon cancer underwent total colectomy combined with lymph node dissection. Conclusion Peutz-Jeghers syndrome often induce intractable intestinal obstruction and gastrointestinal tumor, resection of intestinal lesions should be favorable.For small intractable intussusception, the plication of small intestine has certain value; for its potential malignancy, it should be strictly monitored and actively treated.Gene therapy and drug therapy will guide for the future development. Key words: Pcutz-Jeghers syndrome; Tumor; Plication of intestine