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Peutz-Jeghers Syndrome - JMI ArticlesArticles in our collection about Peutz-Jeghers Syndrome and treatmentTreatment Title: Long-term results of polyp clearance by intraoperative enteroscopy in the Peutz-Jeghers syndrome Authors: Edwards DP;Khosraviani K;Stafferton R;Phillips RK; Journal: Dis Colon Rectum Date: 2003 Jan Abstract: PURPOSE: Enteroscopy during laparotomy for Peutz-Jeghers syndrome was introduced in our unit in 1987. Its aim is to achieve more complete polyp clearance and thereby reduce the number of subsequent laparotomies for small intestinal polyps. METHODS: All patients with Peutz-Jeghers syndrome who had undergone intraoperative enteroscopy since its introduction into our unit in 1987 were identified. The numbers of polyps identified by palpation and transillumination and by enteroscopy were recorded. The timing of, indications for, and findings of all subsequent laparotomies were analyzed and compared with data from our unit before the introduction of intraoperative enteroscopy. RESULTS: Twenty-five patients (14 females) were studied. Enteroscopy identified 350 (median 12, range 0-35) polyps not detected by palpation and transillumination. All impalpable polyps were removed endoscopically by snare or biopsy. The median follow-up was 53 (interquartile range, 13-133) months. Six patients have had an additional laparotomy (1 urgent relaparotomy for small-bowel perforation after endoscopic polypectomy, 4 polypectomies, and 1 adhesion obstruction). No patient has required operative polypectomy within 4 years of polyp clearance by intraoperative enteroscopy, compared with registry data of 4 (17 percent) of 23 patients who had more than 1 laparotomy within 1 year. CONCLUSION: Intraoperative enteroscopy for Peutz-Jeghers syndrome improves polyp clearance without the need for additional enterotomies and may help to reduce the frequency of laparotomies Link: Click to see Pubmed record Title: Intussusceptions in a man with Peutz-Jeghers syndrome Authors: Marschall J;Hayes P; Journal: CMAJ Date: 2003 Feb 4 Abstract: Link: Click to see Pubmed record Title: Peutz-Jeghers syndrome managed with a complete intraoperative endoscopy and extensive polypectomy Authors: Amaro R;Diaz G;Schneider J;Hellinger MD;Stollman NH; Journal: Gastrointest Endosc Date: 2000 Oct Abstract: Link: Click to see Pubmed record Title: Combined endoscopic and surgical treatment for the polyposis of Peutz-Jeghers syndrome Authors: Lin BC;Lien JM;Chen RJ;Fang JF;Wong YC; Journal: Surg Endosc Date: 2000 Dec Abstract: Repeated laparotomy with extensive small bowel resectioning and eventual short-bowel syndrome is a major problem in Peutz-Jeghers syndrome (PJS) patients. This problem is caused by gastrointestinal polyposis with intussusception. A combined surgical and endoscopic approach can assess the extent of the polyposis, and small polyps can be removed by snare polypectomy. This can avert multiple enterotomies and decrease bowel resection segments. We applied an intraoperative colonscope via the enterotomy route in an 20-year-old PJS woman, and successfully removed the other 10 polyps distributed in the whole small bowel. As part of an aggressive approach to the management of polyposis in PJS, complete polypectomy can provide a longer symptom-free interval and remove potentially premaligment polyps Link: Click to see Pubmed record Title: A new simple technique for performing intraoperative endoscopic resection of small-bowel polyps in patients with Peutz-Jeghers syndrome Authors: Ishida H;Murata N;Tada M;Takada S;Fujioka M;Idezuki Y; Journal: Surg Today Date: 1999 Abstract: We describe herein a simple method for performing intraoperative endoscopic resection of small-bowel polyps associated with Peutz-Jeghers syndrome, using a corrugated anesthetic tube. A 34-year-old man with Peutz-Jeghers syndrome underwent emergency surgery for an ileo-ileo-colic intussusception. A sterile corrugated anesthetic tube was inserted into the small-bowel, proximal to the affected lesion. The small bowel was then telescoped sequentially over the tube using a pleating technique. Consequently, a colonoscope inserted through the tube was easily able to reach the duodeno-jejunal junction, and ten small-bowel polyps were removed using a wire snare and electrocauterization. All resected specimens were washed out by the instillation of saline through a nasogastric tube, then collected on gauze placed near the outlet of the tube. Our technique has the following merits: it is feasible even in emergency surgery; it prevents contamination of the surgical field; and it facilitates the easy collection of polypectomized specimens Link: Click to see Pubmed record Title: Ruby laser therapy for labial lentigines in Peutz-Jeghers syndrome Authors: Kato S;Takeyama J;Tanita Y;Ebina K; Journal: Eur J Pediatr Date: 1998 Aug Abstract: Some patients with Peutz-Jeghers syndrome may be disturbed by the appearance of lentigines. Such patients require management of their lentigines as well as their gastro-intestinal polyps. We describe ruby laser therapy of labial lentigines in two children with Peutz-Jeghers syndrome. The response to treatment was excellent and no sequelae or recurrence of the lesions was noted. CONCLUSION: Our experience suggests that ruby laser therapy is safe and a suitable approach for the treatment of labial lentigines in children with Peutz-Jeghers syndrome Link: Click to see Pubmed record Title: Gastrointestinal bleeding and gastric outlet obstruction from Peutz-Jeghers polyposis. Diagnosis and treatment Authors: Corley DA;Uyeki TM;Cello JP; Journal: West J Med Date: 1997 May Abstract: Link: Click to see Pubmed record Title: Overview of screening and management of familial adenomatous polyposis Authors: Rhodes M;Bradburn DM; Journal: Gut Date: 1992 Jan Abstract: The rarity of familial adenomatous polyposis (FAP) means that many clinicians may be unaware of the major advances that have taken place in screening for the condition over the past five years. This review is not only to document the current scene but also to give details of those involved in establishing registries throughout the country. FAP is a hereditary disorder which carries with it almost a 100% risk of colorectal cancer. The aim of screening is to detect gene carriers before they present with symptoms attributable to colonic polyps. In this way the incidence of colorectal cancer can be greatly reduced. The use of gene probes to identify patients with FAP is in its infancy but in selected pedigrees gene carriers can be identified using a venous blood sample. The recognition that congenital hypertrophy of the retinal pigment epithelium is an extracolonic manifestation of FAP in most pedigrees allows non-invasive ophthalmological screening of relatives at risk. The combination of these new screening methods with an effective regional registry for FAP can increase the number of patients detected by screening rather than by symptoms. This facilitates appropriate prophylactic surgery and reduces mortality related to colorectal cancer Link: Click to see Pubmed record Title: Towards decreasing the relaparotomy rate in the Peutz-Jeghers syndrome: the role of peroperative small bowel endoscopy Authors: Spigelman AD;Thomson JP;Phillips RK; Journal: Br J Surg Date: 1990 Mar Abstract: Relaparotomy occurs commonly in patients with the Peutz-Jeghers syndrome, and at quite short intervals. Between 1943 and 1987 laparotomies were performed on 54 occasions in 23 patients with the Peutz-Jeghers syndrome who at some time came under the care of St. Mark's Hospital. In four patients repeat laparotomy was performed within a single year. Between 1987 and 1989 a further five patients have undergone laparotomy with on-table small bowel endoscopy. External palpation and small bowel transillumination failed to demonstrate 17 out of a total of 45 hamartomas (38 per cent). Of these 17 polyps identified endoscopically, 11 (65 per cent) were sufficiently large that a snare was used to remove them. We recommend peroperative enteroscopy as an adjunct to surgery in the Peutz-Jeghers syndrome Link: Click to see Pubmed record Title: Towards decreasing the relaparotomy rate in the Peutz-Jeghers syndrome: the role of peroperative small bowel endoscopy Authors: Spigelman AD;Thomson JP;Phillips RK; Journal: Br J Surg Date: 1990 Mar Abstract: Relaparotomy occurs commonly in patients with the Peutz-Jeghers syndrome, and at quite short intervals. Between 1943 and 1987 laparotomies were performed on 54 occasions in 23 patients with the Peutz-Jeghers syndrome who at some time came under the care of St. Mark's Hospital. In four patients repeat laparotomy was performed within a single year. Between 1987 and 1989 a further five patients have undergone laparotomy with on-table small bowel endoscopy. External palpation and small bowel transillumination failed to demonstrate 17 out of a total of 45 hamartomas (38 per cent). Of these 17 polyps identified endoscopically, 11 (65 per cent) were sufficiently large that a snare was used to remove them. We recommend peroperative enteroscopy as an adjunct to surgery in the Peutz-Jeghers syndrome Link: Click to see Pubmed record Title: Preoperative endoscopy in Peutz-Jeghers syndrome Authors: Fujita S;Kusunoki M;Sakanoue Y;Yamamura T;Utsunomiya J; Journal: Lancet Date: 1990 Feb 17 Abstract: Link: Click to see Pubmed record Title: Peroperative endoscopy in Peutz-Jeghers syndrome Authors: Petroianu A;Garcia dC; Journal: Lancet Date: 1989 Dec 9 Abstract: Link: Click to see Pubmed record Title: Peutz-Jeghers syndrome. Surgical challenge Authors: Paglia MA;Ghosh B;Sherlock P;Kurtz R; Journal: N Y State J Med Date: 1975 Feb Abstract: Link: Click to see Pubmed record Title: Endoscopy in the Peutz-Jeghers syndrome Authors: Kurtz RC;Winawer SJ;Sherlock P; Journal: Am J Gastroenterol Date: 1974 Feb Abstract: Link: Click to see Pubmed record Title: Surgical implications of the Peutz-Jeghers syndrome Authors: Beck AR;Jewett TC; Journal: Ann Surg Date: 1967 Feb Abstract: Link: Click here to read article Title: MANAGEMENT OF PEUTZ-JEGHERS SYNDROME Authors: LLOYD G. BARTHOLOMEW, DAVID G. DAHLIN, JOHN M. WAUGH Journal: MODERN MEDICINE Date: 1962 Abstract: THE PEUTZ-JEGHERS SYNDROMEINTESTINAL POLYPOSIS ASSOCIATED WITH MUCOCUTANEOUS PIGMENTATION-APPARENTLY IS BENIGN. OPERATION IS WARRANTED TO RELIEVE SYMPTOMS, BUT A MINIMAL AMOUNT OF INTESTINE SHOULD BE REMOVED, SINCE MORE POLYPS INVARIABLY DEVELOP IN LATER YEARS. THE SYNDROME HAS NO PREDILECTION FOR AGE OR SEX AND IS OFTEN FAMILIAL, BEING TRANSMITTED BY A SIMPLE MENDELIAN DOMINANT TRAIT. Link: Click here to read article Title: SYSTEMATIC CLEARANCE OF INTESTINAL POLYPS FOR HEMORRHAGE IN PEUTZ-JEGHER'S SYNDROME Authors: MAX J. ALLEN, JOHN E. HERSHEY Journal: ARCHIVES OF SURGERY Date: 1960 Abstract: THE SYNDROME OF SMALL INTESTINAL POLYPOSIS ASSOCIATED WITH MELANIN SPOTS OF THE ORAL MUCOSA, LIPS, AND DIGITS WAS FIRST DESCRIBED IN 1921 BY PEUTZ IN HOLLAND. A COMPREHENSIVE REPORT OF 10 CASES BY JEGHER AND ASSOCIATES IN 1949 HAS BEEN FOLLOWED BY A NUMBER OF SEPARATE REPORTS. BECAUSE OF THE RARITY OF THIS SYNDROME AND THE SUCCESSFUL TREATMENT OF THE PRESENT CASE, IT IS CONSIDERED WORTH WHILE TO PRESENT THIS REPORT WITH SOME PERTINENT FACTS RELATIVE TO THE ORIGIN, DIAGNOSIS, AND TREATMENT UTILIZED. Link: Click here to read article
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Jeghers Medical Index, St. Elizabeth Health Center, 1044 Belmont Ave., Youngstown, OH 44501
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