Oral Lymphangioma in a Patient with Koolen de Vries Syndrome: Case Presentation and Literature Review

2024 • Volume 18 • Issue 1

María Carolina Stierling D.; Diego Ramírez V. & Sebastián Lazo R.



Lymphangiomas are benign hamartomatous tumors of the lymphatic vessels, originating from a sequestration of the lymphatic sac and enlarged by inadequate drainage, lack of communication with the central lymphatic channels or excessive secretion of lining cells. The incidence of these tumors in the lymphatic system is low, with a frequency of 1.2 to 2.8/1000 in children, with no predilection for sex. Three types of lymphangioma can be distinguished in the maxillofacial territory: simple lymphangioma, cavernous lymphangioma, and cystic hygroma or cystic lymphangioma. Clinically, these lesions present as painless, slow-growing soft tissue masses. Their clinical appearance depends on the extent of the lesion. Various treatment methods for lymphangioma have been reported in the literature, with surgical excision being indicated mainly when vital structures are not involved in the lesion. This article presents a case of a 13-year-old female patient with an increase in volume in the vermilion of the upper lip, with a history of Koolen De Vries syndrome, in which the excision of the lesion was performed.

KEY WORDS: lymphangioma, lymphatic vessel tumors, Koolen De Vries.


How to cite this article

STIERLING, C. D.; RAMÍREZ, V. D. & LAZO, R. S. Oral lymphangioma in a patient with Koolen de Vries syndrome: case presentation and literature review. Int. J. Odontostomat., 18(1):8-11, 2024.


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