Diffuse lymphangiomatosis-A fatal case with atypical skeletal features

Eyal Meltzer*, Elinor Goshen, Eduard Fridman, Yechezkel Sidi

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

Diffuse lymphangiomatosis is a rare idiopathic condition that occurs mostly in children, is characterized by a non-neoplastic proliferation of lymphatic vessels, leading to organ dysfunction, chylous effusions, and death. A closely related condition-the Gorham-Stout syndrome-is also characterized by lymphangiomatosis and chylous effusions, but also with massive osteolytic changes ("vanishing bone disease"). A 33-year-old woman presented with a 5-year history of worsening chylous effusions and organomegaly. An extensive evaluation has ruled out most diagnoses. A complete radiographic skeletal study did not disclose any osteolytic changes. However, a Tc99 bone scan has demonstrated an absence of osteoblastic activity in some bones. An autopsy confirmed the diagnosis of diffuse lymphangiomatosis, but with histologically normal bone. If this unusual imaging pattern will be reproduced in future cases, a much needed diagnostic aid may help decrease the frequent diagnostic delays in diffuse lymphangiomatosis.

Original languageEnglish
Pages (from-to)445-448
Number of pages4
JournalAmerican Journal of the Medical Sciences
Volume336
Issue number5
DOIs
StatePublished - Nov 2008
Externally publishedYes

Keywords

  • Adynamic bone
  • Chylothorax
  • Diffuse lymphangiomatosis
  • Gorham-Stout syndrome
  • Lymphangiogenesis

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