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Peutz-Jeghers 症候群의 3 治驗例

Clinical Experiences with Three Cases of Peutz-Jeghers Syndrome

대한외과학회지 1964년 6권 7호 p.405 ~ 411
김상직, 金世顯, 吳熙秀,
소속 상세정보
김상직 (  ) - 慶北大學校 醫科大學 外科學敎室
金世顯 (  ) - 慶北大學校 醫科大學 外科學敎室
吳熙秀 (  ) - 慶北大學校 醫科大學 外科學敎室

Abstract


In 1921, Peutz reported the first case of familiar polyposis of the gastro-intestinal tract with mucocutaneous pigmentation. The syndrome has become a definite clinical entity since its latest rediscovery in 1944 by Jeghers, McKusick and Katz, although many problems are not solved yet.
Since its extreme rarity, less than two hundreds cases of the syndrome have been reported on articles throughout the world, either as isolated cases or as small family groups, so far.
Further interest centers around the malignant potential of the polyps.
This report is concerned with our clinical experiences with three cases of Peutz-Jeghers syndrome, one of which had malignant change in the polyp.
Summarization of the cases is as follows:
Case 1. A 14 year-old girl was admitted to the hospital, because of a 7 year history suggestive of recurrent intussusceptien. Diagnostic studies revealed many polyps over the upper part of small intestine and were suggestive of intussusception. Spots of deep brown and black pigmentation were noticed on the lips, buccal mucosa and fingers. At the time of operation, a huge intussusception of small bowel was found. The jejunum was thickened, dilated, dilated and contained many polyps. This large segment, approximately 250 ㎝ in length, was excised. Intestinal continuity was re-established by end-to-end anastomosis. All these polyps were pathologically benign. The patient has remained free of symptoms until the present time.
Case 2. A 12 year-old boy was admitted to the hospital, because of a 10 month history of dull abdominal pain and postdefecational bleeding, prolapse of the rectal polyps had been occaaionally noticed. Roentgenography showed many polyps in the large bowel. No evidence of intussusception was found. At the exploration, many polyps in the ascending, descending and rectum, were palpable, and were removed by polypectomy. All these polyps were pathologically benign.
Postoperative course was uneventful, except for occasional minimal anal bleeding which persisted to date of discharge.
Case 3. A 24 year-old man was admitted to the hospital with chief complaints of longstanding intractable bloody diarrhea and lower abdominal discomfort of 18 months´ duration. The patient appeared markedly anemic and poorly nourished. Diagnostic X-ray revealed multiple polyps in the small bowel and several in the large bowel. The characteristic dark brown pigmentation were found on the lips and face near the nostril. At operation, about 60 ㎝ of ileum which contained multiple small polyps was excised. The intestinal continuity was re-established by end-to-side anastomosis. The biopsy specimen revealed adenocarcinoma.
The patient did well for over 10 days postoperatively and then became progressively worse. On the 39 th postoperative day, the patient expired abruptly due to massive upper G-I bleeding.
Addendum
After completion of this manuscript, one more patient, 8 year-old boy, visited our clinic complaining of occasional bloody stool for 2 months.
We could find out several brownish or black spots on the mouth-lips and digits on this patient, without any other abnormal findings through physical examination.
Roentgenography revealed many suggestive polypoid tumors in ileum and cecum.
Patient refused further investigation against medical advice.

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