European Journal of Pediatrics

, Volume 163, Issue 12, pp 731–737 | Cite as

Pulmonary capillary haemangiomatosis in children and adolescents: report of a new case and a review of the literature

  • Katalin Bartyik
  • Olga Bede
  • Laszlo Tiszlavicz
  • Beata Onozo
  • Istvan Virag
  • Sandor Turi
Original Paper


Pulmonary capillary haemangiomatosis (PCH) in childhood is a rarity, characterised by the uncontrolled proliferation of pulmonary microvessels which may invade pulmonary, bronchial and vascular structures, resulting in diffuse alveolar haemorrhage, manifesting clinically in haemoptysis, dyspnoea and symptoms of pulmonary hypertension (PH). A 14-year-old boy with some particular features (pericardial effusion and thrombocytopenia) is presented and 14 paediatric/adolescent cases from the literature are surveyed. The diagnostic problems and difficulties are discussed, including the importance of imaging (high-resolution CT) and histopathological studies, with the aim of providing a clear-cut distinction of PCH from other conditions such as primary PH (PPH). The literature data can be regarded as ambiguous: both similarities and relatively sharp distinctions between PCH and PPH are to be found. New developments in the field of genetics are also discussed. The early coexistence of PCH and other (vascular) disorders and associations, involving focal or diffuse, disseminated forms is summarised briefly. Conclusion:The diagnosis of this progressive disorder may lead to effective therapy. Treatment possibilities include the rapidly evolving field of anti-angiogenic therapy, but at present lung transplantation is universally accepted as the final definitive treatment for pulmonary capillary haemangiomatosis.


Adolescents Children Pulmonary capillary haemangiomatosis Pulmonary hypertension Primary pulmonary hypertension 



familial primary pulmonary hypertension


high resolution computed tomography


pulmonary capillary haemangiomatosis


pulmonary hypertension


primary pulmonary hypertension


  1. 1.
    Abramson LP, Gerber S, Chen YH, Crawford SE (2002) Fetal constrictive pericardial defect with pulmonary capillary hemangiomatosis. J Pediatr Surg 37: 1512–1514CrossRefPubMedGoogle Scholar
  2. 2.
    Ahmed Q, Chung-Park M, Thomashefski JF Jr (1997) Cardiopulmonary pathology in patients with sleep apnea/obesity hypoventilation syndrome. Hum Pathol 28: 264–269CrossRefPubMedGoogle Scholar
  3. 3.
    Al-Fawaz IM, Al-Mobaireck KF, Al-Suhaibani, Ashour M (1995) Pulmonary capillary hemangiomatosis. A case report and review of the literature. Pediatr Pulmonol 19: 243–248PubMedGoogle Scholar
  4. 4.
    Almagro P, Julia J, Sanjaume M, Gonzalez G, Casalots J, Hereida JL, Martinez J, Garan J (2002) Pulmonary capillary hemangiomatosis associated with primary pulmonary hypertension. Report of 2 new cases and review of 35 cases from the literature. Medicine (Baltimore) 81: 417–424Google Scholar
  5. 5.
    Bandres-Gimeno R, Gonzalez-Carrero J, Iglesias B, Vicente C, Torres Duran ML (2002) Pulmonary capillary hemangiomatosis: a rare cause of pulmonary hypertension. Arch Bronconeumol 38: 291–294PubMedGoogle Scholar
  6. 6.
    Bhargava P (2000) Antiangiogenesis. In: Abeloff MD, Armitage JO, Lichter AS, Niederhuber JE (eds) Clinical oncology, 2nd edn. Churchill Livingstone, New York, pp 243–250Google Scholar
  7. 7.
    Cioffi U, De Simone M, Pavoni G, Poggi L, Pisani D, Ferrero S, Santambrogio L (1999) Pulmonary capillary hemangiomatosis in an asymptomatic elderly patient. Int Surg 84: 168–170PubMedGoogle Scholar
  8. 8.
    Darmstadt GL (2004) Disseminated hemangiomatosis. In: Behrman RE, Kliegman R, Jenson HB (eds.) Nelson textbook of pediatrics, 17th edn. Saunders, Philadelphia, p 2169Google Scholar
  9. 9.
    Dufour B, Maitre S, Humbert M, Capron F, Simonneau G, Musset D (1998) High-resolution CT of the chest in four patients with pulmonary capillary hemangiomatosis or pulmonary venooclusive disease. AJR 171: 1321–1324PubMedGoogle Scholar
  10. 10.
    Erbersdobler A, Niendorf A (2002) Multifocal distribution of pulmonary capillary haemangiomatosis. Histopathology 40: 88–91CrossRefPubMedGoogle Scholar
  11. 11.
    Faber CN, Yousem SA, Dauber JH, Griffith BP, Hardesty RL, Paradis IL (1989) Pulmonary capillary hemangiomatosis. A report of three cases and review of the literature. Am Rev Respir Dis 140: 808–813PubMedGoogle Scholar
  12. 12.
    Fernandez-Alonso J, Zulueta T, Reyes-Ramirez JR, Castillo-Palma MJ, Sanchez-Roman J (1999) Pulmonary capillary hemangiomatosis as a cause of pulmonary hypertension in a young woman with systemic lupus erythematosus. J Rheumatol 26: 231–233Google Scholar
  13. 13.
    Fishman AP (2001) Clinical classification of pulmonary hypertension. Clin Chest Med 22: 385–391PubMedGoogle Scholar
  14. 14.
    Folkman J (1984) Toward a new understanding of vascular proliferative disease in children. Pediatrics 74: 850–856PubMedGoogle Scholar
  15. 15.
    Friesel R, Komoriya A, Maciag T (1987) Inhibition of endothelial cell proliferation by gamma-interferon. J Cell Biol 104: 689–696CrossRefPubMedGoogle Scholar
  16. 16.
    Ginns LC, Roberts DH, Mark EJ, Brusch JL, Marler JJ (2003) Pulmonary capillary hemangiomatosis with atypical endotheliomatosis. Successful antiangiogenic therapy with doxycycline. Chest 124: 2017–2022CrossRefPubMedGoogle Scholar
  17. 17.
    Gugnani MK, Pierson C, Vanderheide R, Girgis R (2000) Pulmonary edema complicating prostacyclin therapy in pulmonary hypertension associated with scleroderma. A case of pulmonary capillary hemangiomatosis. Arthritis Rheum 43: 699–703CrossRefPubMedGoogle Scholar
  18. 18.
    Havlik DM, Williams WL, Hanzlick R and the Autopsy Committee of the College of American Pathologists (1999) Case of the month. Incidental and not-so-incidental findings. Arch Intern Med 159: 1655–1656CrossRefPubMedGoogle Scholar
  19. 19.
    Havlik DM, Massle LW, Williams WL, Crooks LA (2000) Pulmonary capillary hemangiomatosis-like foci. An antopsy study of 8 cases. Am J Clin Pathol 113: 655–662CrossRefPubMedGoogle Scholar
  20. 20.
    Humbert M, Maitre S, Capron F, Rain B, Musset D, Simonneau G (1998) Pulmonary edema complicating continuous intravenous prostacyclin in pulmonary capillary hemangiomatosis. Am J Respir Crit Care Med 157: 1681–1685PubMedGoogle Scholar
  21. 21.
    Ishii H, Iwabuchi K, Kameya T, Koshino H (1996) Pulmonary capillary haemangiomatosis. Histopathology 29: 275–278PubMedGoogle Scholar
  22. 22.
    Janssen B, Rindermann M, Barth U, Mittelberger-Miltenyi G, Mereles D, Abushi A, Seeger W, Kübler W, Bartram CR, Grünig E (2002) Linkage analysis in a large family with primary pulmonary hypertension. Genetic heterogeneity and a second primary pulmonary hypertension locus on 2q31–32. Chest 121: 54–56CrossRefGoogle Scholar
  23. 23.
    Jing X, Yokoi T, Nakamura Y, Nakamura M, Shan L, Tomimoto S, Hano T, Kakudo K (1998) Pulmonary capillary hemangiomatosis: a unique feature of congestive vasculopathy associated with hypertrophic cardiomyopathy. Arch Pathol Lab Med 122: 94–96PubMedGoogle Scholar
  24. 24.
    Kakkar N, Vasishta RK, Banerjee AK, Singh S, Kumar L (1997) Pulmonary capillary haemangiomatosis as a cause of pulmonary hypertension in Takayasu’s aortoarteritis. Respiration 64: 381–383PubMedGoogle Scholar
  25. 25.
    Kieran MW, Billett A (2001) Antiangiogenesis therapy. Current and future agents. Hematol Oncol Clin North Am 15: 835–851PubMedGoogle Scholar
  26. 26.
    Kuhn JP (2002) High-resolution CT of pediatric lung disease. Radiol Clin North Am 40: 89–110PubMedGoogle Scholar
  27. 27.
    Langleben D, Heneghan JM, Batten AP, Wang NS, Fitch N, Schlesinger RD, Guerraty A, Rouleau JL (1988) Familial pulmonary capillary hemangiomatosis resulting in primary pulmonary hypertension. Ann Intern Med 109: 106–109PubMedGoogle Scholar
  28. 28.
    Langner C, Thonhofer R, Hegenbarth K, Trauner M (2001) Diffuse hemangiomatosis of the liver and spleen in an adult. Pathologie 22: 424–428CrossRefGoogle Scholar
  29. 29.
    Lippert JL, White CS, Cameron EW, Sun CC, Liang X, Rubin LJ (1998) Pulmonary capillary hemangiomatosis: radiographic appearance. J Thorac Imaging 13: 49–51PubMedGoogle Scholar
  30. 30.
    Loyd JE (2002) Genetics and pulmonary hypertension. Chest 122: 284–286CrossRefGoogle Scholar
  31. 31.
    Loyd JE, Philips JA Primary pulmonary hypertension. Gene Reviews available online at
  32. 32.
    Maloney JP (2003) Advances in the treatment of secondary pulmonary hypertension. Curr Opin Pulm Med 9: 139–143CrossRefPubMedGoogle Scholar
  33. 33.
    Masur Y, Remberger K, Hoefer M (1996) Pulmonary capillary hemangiomatosis as a rare cause of pulmonary hypertension. Pathol Res Pract 192: 290–295PubMedGoogle Scholar
  34. 34.
    Mendeloff EN, Meyers BF, Sundt TM, Guthrie TJ, Sweet SC, Shapiro S, Balzer DT, Trulock EP, Lynch JP, Pasque MK, Cooper JD, Huddleston CB, Patterson GA (2002) Lung transplantation for pulmonary vascular disease. Ann Thorac Surg 73: 209–217CrossRefPubMedGoogle Scholar
  35. 35.
    Nakano H, Aizawa H, Inoue H, Takata S, Minami T, Hara N, Watanabe T, Sueishi K (2001) A case of pulmonary capillary hemangiomatosis (in Japanese). Nihon Kokyuki Gakkai Zasshi 39: 471–475PubMedGoogle Scholar
  36. 36.
    Oviedo A, Abramson LP, Worthington R, Dainauskas JK, Crawford SE (2003) Congenital pulmonary capillary hemangiomatosis: report of two cases and review of the literature. Pediatr Pulmonol 36: 253–256CrossRefPubMedGoogle Scholar
  37. 37.
    Results of the EU project group on Rare Pulmonary Diseases. 2. Capillary haemangiomatosis. Available online at
  38. 38.
    Results of the EU project group on Rare Pulmonary Diseases. 3. Primary pulmonary hypertension. Available online at
  39. 39.
    Rich S (1998) WHO executive summary from the World Symposium on Primary Pulmonary Hypertension 1998 Evian, France. Available online at or: Adv Pulm Hypertension (2002) Vol.1, No. 1. Available online at
  40. 40.
    Rich S, McLaughlin VV (2001) Primary pulmonary hypertension, pulmonary capillary hemangiomatosis. In: Braunwald E (ed) Heart disease: a textbook of cardiovascular medicine, 6th edn. Saunders, Philadelphia, pp 1912–1928, 1951Google Scholar
  41. 41.
    Rowen M, Thompson JR, Williamson RA, Wood BJ (1978) Diffuse pulmonary hemangiomatosis. Radiology 127: 445–451PubMedGoogle Scholar
  42. 42.
    Rush C, Langleben D, Schlesinger RD, Stern J, Wang NS, Lamoureux E (1991) Lung scintigraphy in pulmonary capillary hemangiomatosis. A rare disorder causing primary pulmonary hypertension. Clin Nucl Med 16: 913–917PubMedGoogle Scholar
  43. 43.
    Schoen FJ, Cotran RS (1999) Vascular tumors. In: Cotran RS, Kumar V, Collins T, Robbins SL, Schmitt B (eds) Robbins pathologic basis of disease, 6th edn. Saunders, Philadelphia, p 531Google Scholar
  44. 44.
    Schwarz MI, Cherniack RM, King TE Jr (2000) Diffuse alveolar hemorrhage. In: Murray JF, Nadel JA, Murray R, Mason RJ (eds) Textbook of respiratory medicine, 3rd edn. Saunders, Philadelphia, p 1746Google Scholar
  45. 45.
    Shiran A, Naschitz JE, Yeshurun D, Misselevitch I, Boss JH (1990) Diffuse hemangiomatosis of the spleen: splenic hemangiomatosis presenting with giant splenomegaly, anaemia and thrombocytopenia. Am J Gastroenterol 85: 1515–1517PubMedGoogle Scholar
  46. 46.
    Sitbon O, Humbert M, Simonneau G (2002) Primary pulmonary hypertension: current therapy. Progr Cardiovasc Dis 45: 115–128CrossRefGoogle Scholar
  47. 47.
    Slodkowska J, Slupek A, Burakowski J, Bestry I, Filipecki S, Radomsky P (1996) Kartagener syndrome and hemangiomatous proliferation of lung capillaries: case report and literature review (in Polish). Pneumonol Alergol Pol 64: 217–224Google Scholar
  48. 48.
    Slovis BS, Chazova I, Loyd JE, Meyrick BO (1998) Pulmonary capillary haemangiomatosis coexistence with sinus venosus ASD: morphometric analysis and literature review. Eur Respir J 12: 240–244CrossRefPubMedGoogle Scholar
  49. 49.
    Takiguchi Y, Uruma T, Hiroshima K, Motoori K, Watanobe R, Hamaoka T, Okada O, Kimura H, Kuriyama T (2001) Stable pulmonary capillary haemangiomatosis without symptomatic pulmonary hypertension. Thorax 56: 815–817CrossRefPubMedGoogle Scholar
  50. 50.
    Thomas AQ, Gaddipati R, Newman JH, Loyd JE (2001) Genetics of primary pulmonary hypertension. Clin Chest Med 22: 477–491PubMedGoogle Scholar
  51. 51.
    Tron W, Magee F, Wright JL, Colby T, Churg A (1986) Pulmonary capillary hemangiomatosis. Hum Pathol 17: 1144–1150Google Scholar
  52. 52.
    Umezu H, Naito M, Yagisawa K, Hattori A, Aizawa Y (2001) An autopsy case of pulmonary capillary hemangiomatosis without evidence of pulmonary hypertension. Virchows Arch 439: 586–592CrossRefPubMedGoogle Scholar
  53. 53.
    Wagenaar SS, Mulder JJ, Wagenwoort CA, van den Bosch JM (1989) Pulmonary capillary haemangiomatosis diagnosed during life. Histopathology 14: 212–214PubMedGoogle Scholar
  54. 54.
    White CW, Sondheimer HM, Crouch EC, Wilson H, Fan LL (1989) Treatment of pulmonary hemangiomatosis with recombinant interferon alfa-2a. N Engl J Med 320: 1197–1200PubMedGoogle Scholar
  55. 55.
    White CW, Wolf SJ, Korones DN, Sondheimer HM, Tosi MF, Yu A (1991) Treatment of childhood angiomatous diseases with recombinant interferon alfa-2a. J Pediatr 118: 59–66PubMedGoogle Scholar
  56. 56.
    Whittaker JS, Pickering CA, Heath D, Smith P (1983) Pulmonary capillary haemangiomatosis. Diagn Histopathol 6: 77–84PubMedGoogle Scholar

Copyright information

© Springer-Verlag 2004

Authors and Affiliations

  • Katalin Bartyik
    • 1
  • Olga Bede
    • 1
  • Laszlo Tiszlavicz
    • 2
  • Beata Onozo
    • 1
  • Istvan Virag
    • 1
  • Sandor Turi
    • 1
  1. 1.Department of Paediatrics, Albert Szent-Györgyi Medical and Pharmaceutical CentreFaculty of Medicine, University of SzegedSzeged Hungary
  2. 2.Department of Pathology, Albert Szent-Györgyi Medical and Pharmaceutical CentreFaculty of Medicine, University of SzegedSzeged Hungary

Personalised recommendations