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Lymphangioma circumscriptum

J Mordehai1, E Kurzbart, D Shinhar

  • 1Department of Pediatric Surgery, Soroka Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel.

Pediatric Surgery International
|May 1, 1998
PubMed
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This study describes two children with lymphangioma circumscriptum (LC), a rare skin condition. The cases suggest LC may be linked to broader lymphatic system developmental disorders, highlighting unique presentations and treatment challenges.

Area of Science:

  • Dermatology
  • Pediatric Medicine
  • Vascular Malformations

Background:

  • Lymphangioma circumscriptum (LC) is a rare cutaneous lymphatic malformation.
  • Understanding its pathophysiology and association with other lymphatic anomalies is crucial.

Observation:

  • Two pediatric cases of lymphangioma circumscriptum are presented.
  • One case involved antenatal cystic hygroma, postnatal forearm LC, and later mediastinal lymphangioma.
  • This combination represents a previously unreported association.

Findings:

  • LC appears to primarily affect subcutaneous tissue with cystic lymphatic dilatation.
  • Skin lesions and lymphorrhea are likely secondary to increased intraluminal pressure.
  • The findings suggest a potential generalized lymphatic system developmental disorder.

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Implications:

  • This unique combination of lymphatic malformations may indicate a systemic disorder.
  • Further research into the pathophysiology of LC and associated anomalies is warranted.
  • Management typically involves surgical excision, with a notable risk of recurrence.