Summary: A transient dilatation of the lymphatic vessels.

Top Publications

  1. Esther C, Barker P. Pulmonary lymphangiectasia: diagnosis and clinical course. Pediatr Pulmonol. 2004;38:308-13 pubmed
    ..In this report, we describe the etiology, clinical presentation, diagnosis, management, and outcome of PL. A revised classification of PL is proposed, based on the current state of knowledge of its etiology and presentation. ..
  2. Meisler D, Eiferman R, Ratliff N, Burns C. Surgical management of conjunctival lymphangiectasis by conjunctival resection. Am J Ophthalmol. 2003;136:735-6 pubmed
    To report the treatment of symptomatic conjunctival lymphangiectasis with conjunctival resection. Interventional case series...
  3. Barker P, Esther C, Fordham L, Maygarden S, Funkhouser W. Primary pulmonary lymphangiectasia in infancy and childhood. Eur Respir J. 2004;24:413-9 pubmed
    ..In conclusion, these results suggest that primary pulmonary lymphangiectasia does not have as dismal a prognosis as previously described and symptoms and clinical findings improve after the first year of life. ..
  4. Hagmann C, Berger T. Images in clinical medicine. Congenital pulmonary lymphangiectasia. N Engl J Med. 2003;349:e21 pubmed
  5. Bellini C, Mazzella M, Campisi C, Taddei G, Mosca F, Toma P, et al. Multimodal imaging in the congenital pulmonary lymphangiectasia-congenital chylothorax-hydrops fetalis continuum. Lymphology. 2004;37:22-30 pubmed
    ..Non-Immune Hydrops Fetalis (NIHF) may be the final consequence of impaired systemic venous return and may help to explain pleural-pulmonary involvement in this generalized lymph-vessel malformation syndrome. ..
  6. Welch J, Srinivasan S, Lyall D, Roberts F. Conjunctival lymphangiectasia: a report of 11 cases and review of literature. Surv Ophthalmol. 2012;57:136-48 pubmed publisher
    ..All underwent surgical excision of the involved conjunctiva, either with no graft (n = 6), combined with amniotic membrane transplant (n = 3), or combined with conjunctival autograft (n = 2). ..
  7. Lee S, Yang S, Lee K. Congenital pulmonary lymphangiectasia with chylothorax. Asian Cardiovasc Thorac Ann. 2002;10:76-7 pubmed
    ..After conservative management failed, open thoracotomy was performed. Lung biopsy confirmed the diagnosis of congenital pulmonary lymphangiectasia. Chylothorax was successfully controlled by fibrin glue pleurodesis in this patient. ..
  8. Bellini C, Boccardo F, Campisi C, Toma P, Taddei G, Villa G, et al. Pulmonary lymphangiectasia. Lymphology. 2005;38:111-21 pubmed
    ..Recent advances in intensive neonatal care have changed the previously nearly fatal outcome of PL at birth. Patients affected by PL who survive infancy present medical problems which are characteristic of chronic lung disease. ..
  9. Jacquemont S, Barbarot S, Boceno M, Stalder J, David A. Familial congenital pulmonary lymphangectasia, non-immune hydrops fetalis, facial and lower limb lymphedema: confirmation of Njolstad's report. Am J Med Genet. 2000;93:264-8 pubmed
    ..Similarity with our cases and Hennekam syndrome will be discussed...

More Information


  1. Wilson R, Pawel B, Bebbington M, Johnson M, Lim F, Stamilio D, et al. Congenital pulmonary lymphangiectasis sequence: a rare, heterogeneous, and lethal etiology for prenatal pleural effusion. Prenat Diagn. 2006;26:1058-61 pubmed
    Case report and literature review for congenital pulmonary lymphangiectasis (CPL) CASE REPORT: Male fetus with bilateral pleural effusion, thoracoamniotic shunt, preterm delivery, and prolonged neonatal course with neonatal death at 3 ..
  2. MacLean J, Cohen E, Weinstein M. Primary intestinal and thoracic lymphangiectasia: a response to antiplasmin therapy. Pediatrics. 2002;109:1177-80 pubmed
    ..She continues to have exacerbations of her condition, as well as persistent lymphopenia and chronic pleural effusions. ..
  3. Van Balkom I, Alders M, Allanson J, Bellini C, Frank U, De Jong G, et al. Lymphedema-lymphangiectasia-mental retardation (Hennekam) syndrome: a review. Am J Med Genet. 2002;112:412-21 pubmed
  4. Stevenson D, Pysher T, Ward R, Carey J. Familial congenital non-immune hydrops, chylothorax, and pulmonary lymphangiectasia. Am J Med Genet A. 2006;140:368-72 pubmed
  5. Khalil B, Jesudason E, Featherstone N, Sarginson R, Kerr S, Ashworth M, et al. Hidden pathologies associated with (and concealed by) early gestational isolated fetal hydrothorax. J Pediatr Surg. 2005;40:e1-3 pubmed
    ..This rare combination caused significant problems in diagnosis and subsequent treatment...
  6. Soferman R, Mussaffi H, Schreiber L, Nagar H, Sivan Y. Congenital pulmonary lymphangiectasis. Clin Pediatr (Phila). 2003;42:831-4 pubmed
  7. Finder J, Steinfeld J. Congenital pulmonary lymphangiectasia. N Engl J Med. 2004;350:948; author reply 948 pubmed
  8. Hirano H, Nishigami T, Okimura A, Nakasho K, Uematsu K. Autopsy case of congenital pulmonary lymphangiectasis. Pathol Int. 2004;54:532-6 pubmed
    Congenital pulmonary lymphangiectasis (CPL) is a rare anomaly. We report a female infant born at 39 weeks of gestation who was found to have CPL. Cyanosis and tachypnea were noted immediately after birth, and, at room air, PaO2 was 30...
  9. Yamada S, Hisaoka M, Hamada T, Araki S, Shiraishi M. Congenital pulmonary lymphangiectasis: report of an autopsy case masquerading as pulmonary interstitial emphysema. Pathol Res Pract. 2010;206:522-6 pubmed publisher
    We describe the clinicopathologic features of a case of congenital pulmonary lymphangiectasis (CPL)...
  10. Nobre L, Muller N, de Souza Júnior A, Marchiori E, Souza I. Congenital pulmonary lymphangiectasia: CT and pathologic findings. J Thorac Imaging. 2004;19:56-9 pubmed
    ..The histologic findings consisted of ectatic and tortuous lymphatic channels in the interlobular septa, bronchovascular sheaths, and pleura. ..
  11. Njølstad P, Reigstad H, Westby J, Espeland A. Familial non-immune hydrops fetalis and congenital pulmonary lymphangiectasia. Eur J Pediatr. 1998;157:498-501 pubmed
    ..To our knowledge, this is the first report of non-immune hydrops fetalis and congenital pulmonary lymphangiectasia occurring in siblings. ..
  12. Okumura Y, Takeda S, Sawabata N, Maeda H, Hirano H. Pulmonary lymphangiectasis in an asymptomatic adult. Respiration. 2006;73:114-6 pubmed
    Pulmonary lymphangiectasis is a rare anomaly in infancy that is characterized by dilatation of pulmonary lymphatic vessels resulting in fetal respiratory distress...
  13. Bachiri A, Djebara A, Klosowski S, Haouari N, Thelliez P, Voisin O, et al. [Congenital pulmonary lymphangiectasia revealed by cardiac arrest]. Arch Pediatr. 2003;10:615-8 pubmed
    ..The diagnosis is established at lung microscopy. ..
  14. Scott C, Wallis C, Dinwiddie R, Owens C, Coren M. Primary pulmonary lymphangiectasis in a premature infant: resolution following intensive care. Pediatr Pulmonol. 2003;35:405-6 pubmed
    Primary pulmonary lymphangiectasis is a rare disease, often described as fatal within the neonatal period...
  15. Dempsey E, Sant Anna G, Williams R, Brouillette R. Congenital pulmonary lymphangiectasia presenting as nonimmune fetal hydrops and severe respiratory distress at birth: not uniformly fatal. Pediatr Pulmonol. 2005;40:270-4 pubmed
    ..One patient survived and is thriving at 9 months of age. With continuing advances in antenatal and neonatal care, an improved outcome may be possible in what was previously described as a uniformly fatal condition. ..
  16. Sass U, Andre J, Heid E, Grosshans E. [Acquired lymphangiectasias or mammary dermolytic blisters with spontaneous resolution]. Ann Dermatol Venereol. 2004;131:61-4 pubmed
    ..The unusual clinical presentation of the lesions observed in these two women constitutes a differential diagnosis of acquired dermolytic blisters. ..
  17. Shekhel T, Glick R, Cranmer L. In-transit metastasis from melanoma presenting as lymphangiectasis: a case report. Cutis. 2009;84:151-8 pubmed
    ..We report an unusual case of a patient who developed left leg lesions resembling lymphangiectasis. Punch biopsy results revealed atypical cells consistent with melanoma...
  18. Kaur T, Kumar B, Chawwla Y. Epidermal naevi associated with extrahepatic portal venous obstruction, hypoplastic kidney and lymphangiectasia: a new syndromic variant?. Br J Dermatol. 2004;150:618-9 pubmed
  19. Martin J, Monteagudo C, Montesinos E, Guijarro J, Llombart B, Jorda E. Lipedematous scalp and lipedematous alopecia: a clinical and histologic analysis of 3 cases. J Am Acad Dermatol. 2005;52:152-6 pubmed
    ..Our findings suggest a lessened role of racial factors, but confirms the sex implications in these related conditions, and stress the potential significance of lymphangiectatic vessels in the development of alopecia in these patients. ..
  20. Bellini C, Mazzella M, Arioni C, Campisi C, Taddei G, Toma P, et al. Hennekam syndrome presenting as nonimmune hydrops fetalis, congenital chylothorax, and congenital pulmonary lymphangiectasia. Am J Med Genet A. 2003;120A:92-6 pubmed
    ..Hydrops fetalis may be present in newborns with the Hennekam syndrome. Lymphoscintigraphy can be useful in explaining pleural-pulmonary involvement of this generalized lymph vessel malformation syndrome...
  21. Satria M, Pacheco Rodriguez G, Moss J. Pulmonary lymphangiomatosis. Lymphat Res Biol. 2011;9:191-3 pubmed publisher
    ..Treatments have been tested in unblinded manner with promising results; however, further understanding of the pathogenesis of disease, as well as its natural history, is needed to facilitate drug development. ..
  22. Paul J, Carlson J. Lymphangiectases are common underlying warts and in normal peritumoral skin: histologic evidence of decreased immune surveillance. Am J Dermatopathol. 2011;33:152-60 pubmed publisher
    ..Lymphangiectases are a histologic sign of lymphostasis, which is associated with decreased immune cell trafficking and cell-mediated immunity...
  23. Kalbermatten N, Vock P, Rufenacht D, Anderson S. Progressive melorheostosis in the peripheral and axial skeleton with associated vascular malformations: imaging findings over three decades. Skeletal Radiol. 2001;30:48-52 pubmed
    ..Lymphangiectasia of the mesentery and thorax led to chylothorax resistant to therapy for which the patient underwent a pleuropericardiectomy. Death ensued due to respiratory failure...
  24. Somos S, Farkas B. Immunomodulatory treatment with low-dose interferon-alpha and oral retinoic acid in lymphangioma-like Kaposi's sarcoma. Anticancer Res. 2000;20:541-5 pubmed
    The presence of lymphangiectasis without the characteristic spindle cell proliferation may lead to diagnostic difficulties in Kaposi's sarcoma...
  25. Boccardo F, Bellini C, Eretta C, Pertile D, da Rin E, Benatti E, et al. The lymphatics in the pathophysiology of thoracic and abdominal surgical pathology: immunological consequences and the unexpected role of microsurgery. Microsurgery. 2007;27:339-45 pubmed
    ..All patients had an improvement of their immunocompetence. Median follow-up was 5 years. We demonstrated that the use of microsurgery is remarkably advantageous for performing a causal treatment of the dysfunction. ..
  26. Limbach H, Hasenfus A, Bohle R, Baghai A, Loffler G, Gortner L, et al. Severe respiratory distress syndrome unresponsive to intensive care treatment--diagnostic and therapeutic considerations. Klin Padiatr. 2011;223:283-6 pubmed publisher
    ..The diagnostic and therapeutic approach in this patient is presented. ..
  27. Porzionato A, Alaggio R, Aprile A. Perianal and vulvar Crohn's disease presenting as suspected abuse. Forensic Sci Int. 2005;155:24-7 pubmed
    ..If the patient is hospitalised and the question of protection does not arise, physicians should exclude dermatological diseases before reporting to the judicial authorities. ..
  28. Safatle Ribeiro A, Iriya K, Couto D, Kawaguti F, Retes F, Ribeiro U, et al. Secondary lymphangiectasia of the small bowel: utility of double balloon enteroscopy for diagnosis and management. Dig Dis. 2008;26:383-6 pubmed publisher
    ..In this report we present three different cases of small bowel lymphangiectasia detected by double balloon enteroscopy. The patients were diagnosed with South American blastomycosis, tuberculosis and primary small bowel lymphangioma. ..
  29. Rastogi R, Rastogi V. Computed tomographic scan in the diagnosis of bilateral renal lymphangiectasia. Saudi J Kidney Dis Transpl. 2008;19:976-9 pubmed
    ..Awareness about this condition will result in early diagnosis, early treatment and reduced morbidity. ..
  30. Kyprianou I, Nessim M, Kumar V, Chew C. A case of lymphangiectasia haemorrhagica conjunctivae following phacoemulsification. Acta Ophthalmol Scand. 2004;82:627-8 pubmed
  31. Lu Y, Zhu W. Linear and whorled nevoid hypermelanosis complicated with inflammatory linear verrucous epidermal nevus and ichthyosis vulgaris. J Dermatol. 2007;34:765-8 pubmed
    ..Notable lymphangiectasis could also be seen in the mid part of dermis, which had never previously been described...
  32. Sura R, Colombel J, Van Kruiningen H. Lymphatics, tertiary lymphoid organs and the granulomas of Crohn's disease: an immunohistochemical study. Aliment Pharmacol Ther. 2011;33:930-9 pubmed publisher
    ..These findings have aetiological, therapeutic and prognostic implications. ..
  33. al Gazali L, Hertecant J, Ahmed R, Khan N, Padmanabhan R. Further delineation of Hennekam syndrome. Clin Dysmorphol. 2003;12:227-32 pubmed
  34. Verma S. Lymphangiectasias of the skin: victims of confusing nomenclature. Clin Exp Dermatol. 2009;34:566-9 pubmed publisher
    Lymphangiectasias are known by a variety of names, in the dermatology literature, including lymphangiectasis, acquired lymphangiomas, secondary lymphangiomas and acquired lymphangioma circumscriptum, which has led to confusion...
  35. Steines J, Larson J, Wilkinson N, Kirby P, Goodheart M. Intestinal lymphangiectasia mimicking primary peritoneal carcinoma. Am J Obstet Gynecol. 2010;203:e9-e11 pubmed publisher
    ..Further evaluation and bowel resection identified intestinal lymphangiectasia. This disease should be considered in patients with mesenteric adenopathy and a small bowel mass. ..
  36. Chan D, Francis I, Filipic M, Coroneo M, Yong J. Clinicopathologic study of conjunctivochalasis. Cornea. 2005;24:634; author reply 634-5 pubmed
  37. Fraunfelder F. Liquid nitrogen cryotherapy of conjunctival lymphangiectasia: a case series. Arch Ophthalmol. 2009;127:1686-7 pubmed publisher
  38. Scalzetti E, Heitzman E, Groskin S, Randall P, Katzenstein A. Developmental lymphatic disorders of the thorax. Radiographics. 1991;11:1069-85 pubmed
    ..There are four major types of developmental lymphatic disorders that affect the thorax: lymphangiectasis, characterized by congenital anomalous dilatation of pulmonary lymph vessels; localized lymphangioma, a rare ..
  39. Häner N, Zinkernagel M. Conjunctival Lymphangiectasia Imaged with Anterior Segment OCT. Klin Monbl Augenheilkd. 2015;232:388-9 pubmed publisher
  40. Walker S, Ruhl U. [4199 biopsies from the endoscopic normal lower duodenum]. Z Gastroenterol. 2003;41:69-74 pubmed
    ..LDB can be dispensed within EGD when there is neither diarrhoea nor loss of weight, and no anemia, iron deficiency, vitamin deficiency, macrocytosis, hypoproteinaemia, meteorism, joint symptoms or fever. ..
  41. Fraunfelder F. Liquid nitrogen cryotherapy for conjunctival lymphangiectasia: a case series. Trans Am Ophthalmol Soc. 2009;107:229-32 pubmed
    ..Average length of follow-up was 24.5 months for all subjects. Liquid nitrogen cryotherapy may be an effective surgical alternative in the treatment of conjunctival lymphangiectasia. Cryotherapy may need to be repeated in some instances. ..
  42. Fraunfelder F. Liquid nitrogen cryotherapy of lymphangiectasia. Cornea. 2009;28:594-6 pubmed publisher
    ..One year after cryotherapy, the patient has had no more eye symptoms or signs of lymphangiectasia. Liquid nitrogen cryotherapy may be an effective alternative to surgery in the treatment of conjunctival lymphangiectasia. ..
  43. Elston D, Vinson L. What is your diagnosis? Lymphangioma circumscriptum (benign lymphangiectasia). Cutis. 2005;76:301, 310-2 pubmed
  44. Matarieh A, Harinstein M, Salanitri J, Maganti K. Adult cardiac cystic lymphangiectasia of the right atrium. Echocardiography. 2008;25:772-7 pubmed publisher
    ..There are reports of cardiac cystic lymphangiectasias in children. A case of right atrial lymphangiectasia, initially identified by echocardiography, is presented here. This is the first report of such a mass. ..
  45. Rastogi R, Rastogi U, Sarikwal A, Rastogi V. Renal lymphangiectasia associated with chronic myeloid leukemia. Saudi J Kidney Dis Transpl. 2010;21:724-7 pubmed
    ..We des-cribe the rare association of renal lymphangiectasia with chronic myeloid leukemia, which is probably not yet reported in the medical literature. ..
  46. Akcakus M, Koklu E, Bilgin M, Kurtoglu S, Altunay L, Canpolat M, et al. Congenital pulmonary lymphangiectasia in a newborn: a response to autologous blood therapy. Neonatology. 2007;91:256-9 pubmed
    ..We present a newborn with primary pulmonary lymphangiectasis who developed progressively profuse chylous pleural effusions after enteral full feeding from the 8th day of ..
  47. Mordant P, le Pimpec Barthes F, Riquet M. [Primitive thoracic lymphatic disease in adults]. Rev Pneumol Clin. 2012;68:152-60 pubmed publisher
    ..Classifications' efforts and radiologic progress may lead to an improvement in the management of these patients. ..
  48. Franco G, Toma L, Nosotti L, Muscardin L, Morrone A. Vulvar lymphangiectases mimicking genital warts in female genital mutilation. Eur J Dermatol. 2006;16:587-8 pubmed
  49. Mouta Bellum C, Kirov A, Miceli Libby L, Mancini M, Petrova T, Liaw L, et al. Organ-specific lymphangiectasia, arrested lymphatic sprouting, and maturation defects resulting from gene-targeting of the PI3K regulatory isoforms p85alpha, p55alpha, and p50alpha. Dev Dyn. 2009;238:2670-9 pubmed publisher
    ..Our data suggest that Pik3r1 isoforms are required for distinct steps of embryonic lymphangiogenesis in different organ microenvironments, whereas they are largely dispensable for hemangiogenesis...
  50. Mettauer N, Agrawal S, Pierce C, Ashworth M, Petros A. Outcome of children with pulmonary lymphangiectasis. Pediatr Pulmonol. 2009;44:351-7 pubmed publisher
    Pulmonary lymphangiectasis (PL) is a very rare developmental defect of the lungs, which has previously been reported to have a very poor prognosis...
  51. De Hertogh G, Aerssens J, de Hoogt R, Peeters P, Verhasselt P, Van Eyken P, et al. Validation of 16S rDNA sequencing in microdissected bowel biopsies from Crohn's disease patients to assess bacterial flora diversity. J Pathol. 2006;209:532-9 pubmed
    ..Further study is needed to confirm these preliminary findings...
  52. Laje P, Wilson R, Guttenberg M, Liechty K. Survival in primary congenital pulmonary lymphangiectasia with hydrops fetalis. Fetal Diagn Ther. 2008;24:225-9 pubmed publisher
    ..The different aspects of the management of congenital pulmonary lymphangiectasia are discussed in this report, together with a review of the literature. ..
  53. Amadori G, Micciolo R, Poletti A. A case of intra-abdominal multiple lymphangiomas in an adult in whom the immunological evaluation supported the diagnosis. Eur J Gastroenterol Hepatol. 1999;11:347-51 pubmed
    ..The importance of T-cell lymphopenia and hypogammaglobulinaemia in the diagnosis of intra-abdominal lymphangiomatosis with lymphangiectasia is stressed. ..