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Gastrointestinal
Diagnosis
Small Intestine
Metabolic or Inherited
Intestinal Lymphangiectasia
Intestinal Lymphangiectasia
Atif Zaheer, MD; Michael P. Federle, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Synonyms

              • Primary intestinal lymphangiectasia (Waldmann disease)

            • Definitions

              • 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

                                  1. Borzutzky A et al: Primary intestinal lymphangiectasia (Waldmann's disease). Am J Gastroenterol. 114(2):197, 2019
                                  2. 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
                                  3. Vignes S et al: [Primary intestinal lymphangiectasia (Waldmann's disease).] Rev Med Interne. 39(7):580-5, 2018
                                  4. 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
                                  5. Ersoy O et al: Evaluation of primary intestinal lymphangiectasia by capsule endoscopy. Endoscopy. 45 Suppl 2 UCTN:E61-2, 2013
                                  6. 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
                                  7. Hauser B et al: Intestinal lymphangiectasia. J Pediatr Gastroenterol Nutr. 48(2):125, 2009
                                  8. Lobato Salinas Z et al: [Primary intestinal lymphangiectasia. A rare diagnosis of protein-losing enteropathy.] An Pediatr (Barc). 70(6):606-8, 2009
                                  9. Fry LC et al: Utility of double-balloon enteroscopy for the evaluation of malabsorption. Dig Dis. 26(2):134-9, 2008
                                  10. Katoch P et al: Lymphangiectasia of small intestine presenting as intussusception. Indian J Pathol Microbiol. 51(3):411-2, 2008
                                  11. 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
                                  12. Vignes S et al: Primary intestinal lymphangiectasia (Waldmann's disease). Orphanet J Rare Dis. 3:5, 2008
                                  13. 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
                                  14. Pratz KW et al: Intestinal lymphangiectasia with protein-losing enteropathy in Waldenstrom macroglobulinemia. Medicine (Baltimore). 86(4):210-4, 2007
                                  15. Mazzie JP et al: Congenital intestinal lymphangiectasia: CT demonstration in a young child. Clin Imaging. 27(5):330-2, 2003
                                  16. Yang DM et al: Localized intestinal lymphangiectasia: CT findings. AJR Am J Roentgenol. 180(1):213-4, 2003
                                  17. Stevens RL et al: The CT halo sign: a new finding in intestinal lymphangiectasia. J Comput Assist Tomogr. 21(6):1005-7, 1997
                                  18. Puri AS et al: Intestinal lymphangiectasia: evaluation by CT and scintigraphy. Gastrointest Radiol. 17(2):119-21, 1992
                                  Related Anatomy
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                                  Related Differential Diagnoses
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                                  References
                                  Tables

                                  Tables

                                  KEY FACTS

                                  • Terminology

                                    • Imaging

                                      • Top Differential Diagnoses

                                        • Pathology

                                          • Clinical Issues

                                            TERMINOLOGY

                                            • Synonyms

                                              • Primary intestinal lymphangiectasia (Waldmann disease)

                                            • Definitions

                                              • 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

                                                                  1. Borzutzky A et al: Primary intestinal lymphangiectasia (Waldmann's disease). Am J Gastroenterol. 114(2):197, 2019
                                                                  2. 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
                                                                  3. Vignes S et al: [Primary intestinal lymphangiectasia (Waldmann's disease).] Rev Med Interne. 39(7):580-5, 2018
                                                                  4. 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
                                                                  5. Ersoy O et al: Evaluation of primary intestinal lymphangiectasia by capsule endoscopy. Endoscopy. 45 Suppl 2 UCTN:E61-2, 2013
                                                                  6. 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
                                                                  7. Hauser B et al: Intestinal lymphangiectasia. J Pediatr Gastroenterol Nutr. 48(2):125, 2009
                                                                  8. Lobato Salinas Z et al: [Primary intestinal lymphangiectasia. A rare diagnosis of protein-losing enteropathy.] An Pediatr (Barc). 70(6):606-8, 2009
                                                                  9. Fry LC et al: Utility of double-balloon enteroscopy for the evaluation of malabsorption. Dig Dis. 26(2):134-9, 2008
                                                                  10. Katoch P et al: Lymphangiectasia of small intestine presenting as intussusception. Indian J Pathol Microbiol. 51(3):411-2, 2008
                                                                  11. 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
                                                                  12. Vignes S et al: Primary intestinal lymphangiectasia (Waldmann's disease). Orphanet J Rare Dis. 3:5, 2008
                                                                  13. 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
                                                                  14. Pratz KW et al: Intestinal lymphangiectasia with protein-losing enteropathy in Waldenstrom macroglobulinemia. Medicine (Baltimore). 86(4):210-4, 2007
                                                                  15. Mazzie JP et al: Congenital intestinal lymphangiectasia: CT demonstration in a young child. Clin Imaging. 27(5):330-2, 2003
                                                                  16. Yang DM et al: Localized intestinal lymphangiectasia: CT findings. AJR Am J Roentgenol. 180(1):213-4, 2003
                                                                  17. Stevens RL et al: The CT halo sign: a new finding in intestinal lymphangiectasia. J Comput Assist Tomogr. 21(6):1005-7, 1997
                                                                  18. Puri AS et al: Intestinal lymphangiectasia: evaluation by CT and scintigraphy. Gastrointest Radiol. 17(2):119-21, 1992