Summary
A 26-year-old patient presented with epigastric pain of sudden onset and severe puffy swelling of both legs and forearms. An irregularly shaped nodular filling defect on selective jejunal films, severe hypoproteinemia, low IgG concentration, and lymphopenia were suggestive of primary intestinal lymphangiectasia with protein-losing enteropathy, and the patient was placed on a low-fat diet with medium-chain triglycerides. This initially improved his condition, but some weeks later he developed obstructive ileus of the small intestine. On laparotomy yellowish to whitish deposits were found to be present in some segments of the small intestine and a fist-sized mass 100 cm distal to the duodenojejunal flexure was resected without complications. Histologically, the submucosal lymphatics were dilated, and the jejunal wall showed extensive pseudocystic, intramural submucosal lymph edema with secondary bleeding and tight stenosis of the jejunal lumen. During the 14-month follow-up time after discharge the patient has been asymptomatic and working, on no treatment other than a low-fat diet with medium-chain triglycerides.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Asakura H, Morishita T, Morita A, Hibi T, Ono A, Tsuchiya M (1974) Klinische und immunologische Aspekte der exsudativen Gastroenteropathie. Leber Magen Darm 4:109–116
Bender SW, Posselt HG, Strobel S, Röttger R (1976) Neuere Aspekte der pädiatrischen Gastroenterologie. Internist 17:169–180
Blaschke-Zimmermann E, Lagenstein I, Winkler K (1978) Intestinale Lymphagiektasie bei exsudativer Enteropathie. Monatsschr Kinderheilk 126:572–574
Bookstein JJ, French AB, Pollard HM (1965) Protein-losing gastroenteropathy: concepts derived from lymphangiography. Am J Dig Dis 10: 573–581
Esterling JR (1965) Congenital heriditary lymphedema. J Med Genet 2:93–96
Furstenau M, Kratzsch KH, Zimmermann S, Büttner W (1977) Fiberendoskopischer Nachweis, Häufigkeit und klinische Bedeutung der intestinalen Lymphangiektasie. Z Inn Med 32:638–640
Gold RH, Youker JE (1973) Idiopathic intestinal lymphangiectasis (Primary protein-losing enteropathy). Radiology 109:315–316
Hargrove MD, Mathews WR, McIntyre PA (1967) Intestinal lymphangiectasia with response to corticoisteroids. Arch Intern Med 119:206–210
Heresbach D, Raoul JL, Bretagne JF, Gosselin M (1991) Intestinal lymphangiectasia: lack of efficacy of antiplasmin therapy? Gastroenterology 100:1152–1153
Heuck F, Fuchs HF, Donner MW (1990) Klinische Radiologic. Gastrointestinaltrakt. Diagnostik mit bildgebendem Verfahren. Springer, Berlin Heidelberg New York
Holt PR (1964) Dietary treatment of protein loss in intestinal lymphangiectasia. Pediatrics 34:629–635
Kinmonth JB, Cox SJ (1974) Protein-losing enteropathy in primary lymphoedema: mesenteric lymphography and gut resection. Br J Surg 61:589–593
Martini GA, Dölle W, Petersen F, Treske U, Strohmeyer G (1963) Die exsudative Gastroenteropathie, ein polyatiologisches Syndrom. Internist 4:197–209
Mine K, Matsubayashi S, Nakai Y, Nakagawa T (1989) Intestinal lymphangiectasia markedly improved with antiplasmin therapy. Gastroenterology 96:1596–1599
Mistilis SP, Skyring AP, Stephen DD (1965) Intestinal lymphangiectasia. Lancet I:77–80
Molnar Z, Karoliny G, Mozsik G, Nemeth A (1974) Lymphogenic enteropathy from thoracic duct obstruction. Lymphology 3:130–136
Mührer A (1971) Intestinale Lymphangiektasie bei exsudativer Enteropathie. Zentralbl Allg Pathol 114:23–29
Muzaffar K, Block MA, Fox TA (1972) Primary intestinal lymphangiectasia: surgical implications. Am Surg 38:288–291
Pomerantz M, Waldmann TA (1963) Systemic lymphatic abnormalities associated with gastrointestinal protein loss secondary to intestinal lymphangiectasia. Gastroenterology 45:703–711
Rao SS, Dundas S, Holdsworth CD (1987) Intestinal lymphangiectasia secondary to radiotherapy and chemotherapy. Dig Dis Sci 32:939–942
Röttger P, Bender SW, Strobel S, Klempaer J (1976) Intestinale Lymphangiektasie, morphologische Befunde und ihre diagnostischen und therapeutischen Konsequenzen. Z Gastroenterol 14:261–269
Schmidt H, Riemann JF (1980) Enterales Eiwei\verlust-Syndrom durch primary intestinale Lymphangiektasie. Med Klinik 75:813–817
Shimkin PM, Waldmann TA, Krugman RL (1970) Intestinal lymphangiectasia. AJR 110:827–841
Tift WL, Lloyd JK (1975) Intestinal lymphangiectasia long-term results with MCT diet. Arch Dis Child 50:269–276
Vardy PA, Lebenthal E, Schwachman H (1975) Intestinal lymphangiectasia: a reappraisal. Pediatrics 55:842–851
Waldmann TA (1966) Protein-losing enteropathy. Gastroenterology 50:422–443
Waldmann TA, Steinfeld JL, Dutcher TF, Davidson JD, Gordon RS Jr (1961) The role of the gastrointestinal system in “idiopathic hypoproteinemia.“ Gastroenterology 41:197–207
Ward M, Le Roux A, Small WP, Sircus W (1977) Malignant lymphoma and extensive viral wart formation in a patient with intestinal lymphangiectasia and lymphocyte depletion. Postgrad Med J 53:753–757
Westergaard H, Jarnum S, Jensen H, Soltoft J, Yssing M (1968) Topographic diagnosis of gastrointestinal protein loss. Digestion 1:341–352
Wood JE, Esterly JR, McKusick VA (1960) Genetic, clinical and physiologic observations in Milroy's disease. Circulation 22:834
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Lenzhofer, R., Lindner, M., Moser, A. et al. Acute jejunal ileus in intestinal lymphangiectasia. Clin Investig 71, 568–571 (1993). https://doi.org/10.1007/BF00208483
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00208483