Abstract
A case of a 61 year old woman with Ehlers-Danlos syndrome who developed recurrent gastrointestinal complications is reported herein. She has been followed by Kyoundo Hospital since 1965, when she originally underwent treatment for eventration of the diaphragm and a volvulus of the stomach at the age of 41. During the past 22 years she has experienced perforation of the colon three times and jejunal perforation once. This case was considered to be type II Ehlers-Danlos syndrome. We have found 12 other cases of Ehlers-Danlos syndrome with gastrointestinal complications in the Japanese literature. More thorough examination of Ehlers-Danlos syndrome cases may reveal more subclinical gastrointestinal abnormalities. We concluded from our experience and from the literature that when colonic perforation occurs in this syndrome, total colectomy and ileo-rectal anastomosis is reasonably indicated.
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Isobe A, Sato Y, Kawada A. Ehlers-Danlos syndrome. Seikei Geka (Orthopedics) 1984; 35: 638–639. (in Japanese)
Uitto J, Richtenstein JR. Defects in the biochemistry of collagen in diseases of connective tissue. J Invest Dermatol 1976; 66: 59–79.
Beighton P. Lethal complications of the Ehlers-Danlos syndrome. Br Med J 1968; 3: 656–659.
Sykes EM. Colon perforation in Ehlers-Danlos syndrome. Report of two cases and review of the literature. Am J Surg 1984; 147: 410–413.
Sygurdson E, Stern HS, Houpt J, El-Sharkway TY, Huizinga JD. The Ehlers-Danlos syndrome and colonic perforation. Dis Colon Rectum 1985; 28: 962–966.
Hayakawa A, Fujimoto K, Ibayashi H. Two cases of Ehlers-Danlos syndrome with gastrointestinal complications. Gastroenterol Jpn 1982; 17: 61–67.
Bain NH. Ehlers-Danlos syndrome. Am J Gastroenterol 1977; 67: 167–170.
Aldridge RT. Ehlers-Danlos syndrome causing intestinal perforation. Brit J Surg 1967; 54: 22–25.
Harland D. Ehlers-Danlos syndrome. Proc Roy Soc Med 1970; 63: 286–287.
Zalis EG, Roberts DC. Ehlers-Danlos syndrome with a hypoplastic kidney, bladder diverticulum and diaphragmatic hernia. Arch Dermatol 1967; 96: 540–544.
Miyakawa K, Fukuda Y. A case of Ehlers-Danlos syndrome. Nihon Byori Gakkai Zasshi (J Jpn Soc Pathol) 1965; 54: 40–41. (in Japanese)
Aochi I, Ishii T, Awazu Y, Tanetani S, Kitada S, Sato M, Awai K, Doi H, Moriwaki S. A case of Ehlers-Danlos syndrome. Naika (Internal Medicine) 1967; 20: 1183–1189. (in Japanese)
Morishima A, Hasuno K, Ueno T, Morio H. A case of Ehlers-Danlos syndrome. Shonika Shinryo (Pediatric Clinic) 1972; 35: 160–166. (in Japanese)
Fujimoto S, Misawa T, Hayakawa H, Kimura S, Ibayashi H. A case of Ehlers-Danlos syndrome with large and multiple duodenal and jejunal diverticulosis. Nihon Naika Gakkai Zasshi (J Jpn Soc Inter Med) 1981; 70: 488 (in Japanese)
Nagahara A, Rikusha M, Isoda N, Kinoyama S, Takahashi K. A case of Ehlers-Danlos syndrome with malabsorption. Nihon Shokaki Naishikyo Gakkai Zasshi (Gastroent Endoscopy) 1984; 26: 302–303. (in Japanese)
Kurokawa H, Kamita N, Sasaki K, Yamaguchi Y, Kojimahara M, Arita E. A case of two brothers with Ehlers-Danlos syndrome with intestinal malrotation. Geka Shinryou (Surgical Diagnosis and Treatment) 1984; 27: 381–385. (in Japanese)
Shitano T, Tateno M, Mori Y, Nakasone K, Minami M, Nakano T, Maruyama C. A lost case of Ehlers-Danlos syndrome by massive bleeding at delivery. Rinsho Iden Kenkyu (Clin Research of Genetics) 1985; 5: 3–5. (in Japanese)
Kojima T, Hujita Y, Kitagawa G, Shinkai H. A case of Ehlers-Danlos syndrome with defect of collagen type 1. Nishi Nihon Hifuka Gakkai Zasshi (J Soc West Jpn Dermatol) 1986; 48: 781. (in Japanese)
Nunoda S, Ueno T, Miyamori I, Takeda R, Nakamura Y. A case of Gigantism with hyper-motidity of joints and dextrocardia. Shindan to Chiryo (Diagnosis and Treatment) 1987; 75: 376–378. (in Japanese)
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Iwama, T., Sato, H., Matsuzaki, T. et al. Ehlers-danlos syndrome complicated by eventration of the diaphragm, colonic perforation and jejunal perforation —A case report—. The Japanese Journal of Surgery 19, 376–380 (1989). https://doi.org/10.1007/BF02471417
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DOI: https://doi.org/10.1007/BF02471417