Rare disease or syndrome characterized by hypoproteinemia, peripheral edema, and lymphocytopenia resulting from loss of lymphatic fluid into intestine
Important cause of protein-losing enteropathy
IMAGING
Radiographic Findings
CT Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
Borzutzky A et al: Primary intestinal lymphangiectasia (Waldmann's disease). Am J Gastroenterol. 114(2):197, 2019
Dong J et al: CT lymphangiography (CTL) in primary intestinal lymphangiectasia (PIL): a comparative study with intraoperative enteroscopy (IOE). Acad Radiol. 26(2):275-81, 2019
Vignes S et al: [Primary intestinal lymphangiectasia (Waldmann's disease).] Rev Med Interne. 39(7):580-5, 2018
Wen Z et al: The lymphoscintigraphic manifestation of (99m)Tc-dextran lymphatic imaging in primary intestinal lymphangiectasia. Nucl Med Commun. 35(5):493-500, 2014
Ersoy O et al: Evaluation of primary intestinal lymphangiectasia by capsule endoscopy. Endoscopy. 45 Suppl 2 UCTN:E61-2, 2013
Urganci N et al: Evaluation of paediatric patients with protein losing enteropathy a single centre experience. West Indian Med J. 62(3):186-9, 2013
Hauser B et al: Intestinal lymphangiectasia. J Pediatr Gastroenterol Nutr. 48(2):125, 2009
Lobato Salinas Z et al: [Primary intestinal lymphangiectasia. A rare diagnosis of protein-losing enteropathy.] An Pediatr (Barc). 70(6):606-8, 2009
Fry LC et al: Utility of double-balloon enteroscopy for the evaluation of malabsorption. Dig Dis. 26(2):134-9, 2008
Katoch P et al: Lymphangiectasia of small intestine presenting as intussusception. Indian J Pathol Microbiol. 51(3):411-2, 2008
Safatle-Ribeiro AV et al: Secondary lymphangiectasia of the small bowel: utility of double balloon enteroscopy for diagnosis and management. Dig Dis. 26(4):383-6, 2008
Vignes S et al: Primary intestinal lymphangiectasia (Waldmann's disease). Orphanet J Rare Dis. 3:5, 2008
Yakami Y et al: [Efficacy of double balloon enteroscopy for patients with intestinal lymphangiectasia, case report of primary intestinal lymphangiectasia.] Nippon Shokakibyo Gakkai Zasshi. 105(11):1612-8, 2008
Pratz KW et al: Intestinal lymphangiectasia with protein-losing enteropathy in Waldenstrom macroglobulinemia. Medicine (Baltimore). 86(4):210-4, 2007
Mazzie JP et al: Congenital intestinal lymphangiectasia: CT demonstration in a young child. Clin Imaging. 27(5):330-2, 2003
Yang DM et al: Localized intestinal lymphangiectasia: CT findings. AJR Am J Roentgenol. 180(1):213-4, 2003
Stevens RL et al: The CT halo sign: a new finding in intestinal lymphangiectasia. J Comput Assist Tomogr. 21(6):1005-7, 1997
Puri AS et al: Intestinal lymphangiectasia: evaluation by CT and scintigraphy. Gastrointest Radiol. 17(2):119-21, 1992
Rare disease or syndrome characterized by hypoproteinemia, peripheral edema, and lymphocytopenia resulting from loss of lymphatic fluid into intestine
Important cause of protein-losing enteropathy
IMAGING
Radiographic Findings
CT Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
Borzutzky A et al: Primary intestinal lymphangiectasia (Waldmann's disease). Am J Gastroenterol. 114(2):197, 2019
Dong J et al: CT lymphangiography (CTL) in primary intestinal lymphangiectasia (PIL): a comparative study with intraoperative enteroscopy (IOE). Acad Radiol. 26(2):275-81, 2019
Vignes S et al: [Primary intestinal lymphangiectasia (Waldmann's disease).] Rev Med Interne. 39(7):580-5, 2018
Wen Z et al: The lymphoscintigraphic manifestation of (99m)Tc-dextran lymphatic imaging in primary intestinal lymphangiectasia. Nucl Med Commun. 35(5):493-500, 2014
Ersoy O et al: Evaluation of primary intestinal lymphangiectasia by capsule endoscopy. Endoscopy. 45 Suppl 2 UCTN:E61-2, 2013
Urganci N et al: Evaluation of paediatric patients with protein losing enteropathy a single centre experience. West Indian Med J. 62(3):186-9, 2013
Hauser B et al: Intestinal lymphangiectasia. J Pediatr Gastroenterol Nutr. 48(2):125, 2009
Lobato Salinas Z et al: [Primary intestinal lymphangiectasia. A rare diagnosis of protein-losing enteropathy.] An Pediatr (Barc). 70(6):606-8, 2009
Fry LC et al: Utility of double-balloon enteroscopy for the evaluation of malabsorption. Dig Dis. 26(2):134-9, 2008
Katoch P et al: Lymphangiectasia of small intestine presenting as intussusception. Indian J Pathol Microbiol. 51(3):411-2, 2008
Safatle-Ribeiro AV et al: Secondary lymphangiectasia of the small bowel: utility of double balloon enteroscopy for diagnosis and management. Dig Dis. 26(4):383-6, 2008
Vignes S et al: Primary intestinal lymphangiectasia (Waldmann's disease). Orphanet J Rare Dis. 3:5, 2008
Yakami Y et al: [Efficacy of double balloon enteroscopy for patients with intestinal lymphangiectasia, case report of primary intestinal lymphangiectasia.] Nippon Shokakibyo Gakkai Zasshi. 105(11):1612-8, 2008
Pratz KW et al: Intestinal lymphangiectasia with protein-losing enteropathy in Waldenstrom macroglobulinemia. Medicine (Baltimore). 86(4):210-4, 2007
Mazzie JP et al: Congenital intestinal lymphangiectasia: CT demonstration in a young child. Clin Imaging. 27(5):330-2, 2003
Yang DM et al: Localized intestinal lymphangiectasia: CT findings. AJR Am J Roentgenol. 180(1):213-4, 2003
Stevens RL et al: The CT halo sign: a new finding in intestinal lymphangiectasia. J Comput Assist Tomogr. 21(6):1005-7, 1997
Puri AS et al: Intestinal lymphangiectasia: evaluation by CT and scintigraphy. Gastrointest Radiol. 17(2):119-21, 1992
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