Related Experiment Videos
[Giant multifocal venous malformation with monomelic predominance]
C Langella1, E Delaporte, J P Beregi
1Service de Dermatologie A, Hôpital Claude-Huriez, Hôpital Cardiologique, CHRU, 59037 Lille Cedex.
Annales De Dermatologie Et De Venereologie
|December 29, 1999
Summary
This case study details a rare venous malformation with extensive, disfiguring nodules, initially suspected to be Maffucci's syndrome. Surgical excision provided functional improvement, highlighting a unique presentation of vascular anomalies.
Area of Science:
- Vascular Surgery
- Dermatology
- Medical Genetics
Background:
- Venous malformations (VMs) are common vascular anomalies.
- This report details a rare case where clinical presentation mimicked Maffucci's syndrome.
Observation:
- A 44-year-old male presented with numerous, compressible angiomatous nodules since infancy.
- Progressive lesions caused significant deformity and disability, affecting limbs, torso, and oral cavity.
- Diagnosis of VM was confirmed by clinical, radiological (phleboliths), and histological findings.
Findings:
- Histological examination confirmed spindle cell hemangioendothelioma, 'blue rubber bleb nevus,' and glomangiomatosis.
- Maffucci's syndrome was excluded due to the absence of chondromas.
- Klippel-Trenaunay syndrome was ruled out by the lack of port-wine stains or bone hypertrophy.
Implications:
- This case highlights a severe, multifocal VM with oral involvement, presenting diagnostic challenges.
- Surgical management, despite initial embolization failure, yielded positive functional outcomes.
- The unusual presentation underscores the importance of comprehensive evaluation for complex vascular malformations.