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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.
Insights
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.
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.
Abstract:
Two children with lymphangioma circumscriptum (LC), a rare cutaneous lymphangioma, are described. One was diagnosed antenatally as having a right axillary cystic hygroma; in addition, postnatally LC was noted on the right forearm. A few years later a mediastinal lymphangioma was diagnosed. This combination of LC associated with additional malformations of the lymphatic system has not been reported previously, and may point to a generalized developmental disorder of the lymphatic system. The pathophysiology of the lesion is discussed with a review of the pertinent literature. It appears that LC primarily affects the subcutaneous tissue in the form of cystic dilatation of lymphatic channels without systemic lymphatic communication. The skin lesions are probably secondary to the increased intraluminal pressure. The typical clinical appearance is manifested by multiple skin vesicles and lymphorrhea. The main treatment modality is surgical excision. Recurrences are not unusual and require re-excision.