Journal of the Formosan Medical Association
Volume 106, Issue 3, Supplement , Pages S10-S14, 2007

Generalized Lymphangiomatosis Presenting as Cardiomegaly

  • Yi-Ling Chen

      Affiliations

    • Department of Pediatrics, Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan
    • ,
  • Chin-Cheng Lee

      Affiliations

    • Department of Pathology and Laboratory Medicine Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan
    • ,
  • Ming-Lun Yeh

      Affiliations

    • Department of Pediatric Surgery, Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan
    • ,
  • Jing-Sheng Lee

      Affiliations

    • Department of Pediatrics, Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan
    • ,
  • Tseng-Chen Sung

      Affiliations

    • Department of Pediatrics, Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan
    • Corresponding Author InformationCorrespondence to: Dr Tseng-Chen Sung, Department of Pediatrics, Shin Kong Wu Ho-Su Memorial Hospital, 95 Wenchang Road, Shilin District, Taipei 111, Taiwan

Received 18 October 2005; received in revised form 23 November 2005; accepted 10 January 2006.

Article Outline

Lymphangioma refers to the local proliferation of well-differentiated lymphatic tissue. Generalized lymphangiomatosis is rare. We report a previously healthy 8-month-old infant who suffered from tachypnea with mild fever for 2 weeks. Imaging studies revealed a well-defined, large mass occupying the mediastinum, which presented as cardiomegaly. The disseminated mass extended to the thymus, lung, and spleen. Lymphangiomatosis was diagnosed by biopsy. Drainage of the pericardial fluid and total parenteral nutrition did not result in improvement of chylopericardium. Secondary hypogammaglobulinemia and septic shock developed sequentially. Surgical removal of the mediastinal mass and spleen were performed. Daily subcutaneous injection of interferon (IFN) alpha-2b was then given for 3 months. No recurrence was noted during 2 years of follow-up. IFN alpha-2b may be considered as an alternative for the treatment of generalized lymphangiomatosis. [J Formos Med Assoc 2007;106(3 Suppl):S10-S14]

Key Words:  chylopericardium , generalized lymphangiomatosis , interferon alpha-2b

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PII: S0929-6646(09)60359-4

doi:10.1016/S0929-6646(09)60359-4

Journal of the Formosan Medical Association
Volume 106, Issue 3, Supplement , Pages S10-S14, 2007

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