Large frontal osseous hemangioma with dural sinus involvement in a patient with Klippel-Trenaunay syndrome: A rare case report

Abstract

Background:We present one of the first documented cases in the literature of an adult with Klippel-Trenaunay syndrome (KTS) with a large frontal osseous hemangioma.Case Description:A 30-year-old male presented with a rapidly enlarging frontal skull lesion that had developed in only 3 months. Radiological investigation revealed a highly vascular lesion attached to the frontal bone. The lesion was surgically resected with the patient making complete recovery. Histopathology was consistent with an osseous hemangioma.Conclusion:We report the clinical presentation and surgical management of a rare presentation of osseous hemangioma in a patient with KTS.

Diversity and frequency of symptoms in Klippel–Trénaunay syndrome

“…All 3 KTS symptoms are not usually observable in all patients; for example, the triad can be present in 63% of the studied pool, while the remaining 37% presented only 2 of 3 symptoms [9]. Diagnosed Klippel-Trénaunay syndrome does not exclude the patients from the risk of developing other rare ailments, described in singular instances, such as: -newly-developed periosteal bone formation, potentially linked to KTS [15]; -broad, numerous lymphatic haemangiomas of underscribed relation to KTS, based on the uniqueness of the case [11]; -osseous haemangioma that developed in patient's frontal bone, affecting the superior agittal sinus, which is an uncommon and probably the only published instance of a patient with a cranial location of haemangioma in a KTS patient [20]; -a case of a giant uterus weighing 6.3 kgs, with leiomyomatosis and diffuse vascular malformations [21].…”

“…Also, while instances of rare ailments present in KTS patients are described, they remain isolated cases and are not part of a typical picture of patients affected by this syndrome. [11,15,20,21].…”

Surgical Management of a Massive Frontal Bone Hemangioma: Case Report

“…The treatment of choice is en bloc resection with 5 to 10 mm wide bone margin for the primary goals of limiting painful progression, improving cosmesis, and avoiding potential complications such as hemorrhage. 1 2 4 5 7 8 9 11 12 13 14 17 19 20 25 34 35 Utilization of preoperative embolization can be considered to aid surgical resection by decreasing intraoperative blood loss. 7 10 20 25…”

“…The treatment of choice is en bloc resection with 5 to 10 mm wide bone margin for the primary goals of limiting painful progression, improving cosmesis, and avoiding potential complications such as hemorrhage. 1,2,4,5,[7][8][9][11][12][13][14]17,19,20,25,34,35 Utilization of preoperative embolization can be considered to aid surgical resection by decreasing intraoperative blood loss. 7,10,20,25 Following surgical resection, the remaining cranial defect must be reconstructed which is particularly challenging with large lesions and those involving the forehead.…”