Cerebellar Dermoid Cyst with Occipital Dermal Sinus

Abstract

Intracranial dermoid cyst is a rare entity accounting for 0.1–0.7% of all intracranial tumors. The most common location is in the posterior fossa, at or near the midline. We present 2 pediatric cases with dermal sinus. The first case presented with clinical signs of increased intracranial pressure and cerebellar symptoms. CT scan showed a large and regular midline posterior fossa cyst without contrast enhancement. The second case was revealed by recurrent meningitis. CT scan showed a midline vermis low-density mass with capsular contrast enhancement. Dermal sinus was found in 2 cases. Complete surgical removal was performed followed, in a second operation, by ventriculoperitoneal shunt in 2 cases. There was no postoperative complication in our patients. The aim of this study is to discuss the clinical aspects of dermoid cyst, especially in cases with dermal sinus, and to review the therapeutic strategies in case of associated hydrocephalus.

Unusual Clinical Presentation and Association of Cranial Dermoid Tumor and Tethered Cord Syndrome

“…CDT, derived from the entopic inclusion of epithelial cells and containing hair follicles, sweat, and sebaceous gland, is a congenital benign tumor that grows slowly [6]. The defective cleavage between the ectoderm and the neuroderm during the 3rd to 5th gestational week is responsible for the pathogenesis.…”

“…However, due to the fact that it is an uncommon disease, the pathophysiology of CDT is not well defined [7]. The tumor generally sits on the midline of the posterior fossa, vermis, and spine, which are on the closing line of the neural tube [6]. Logue and Till [15] grouped posterior fossa dermoid tumors according to their location (intradural, extradural) and the degree of development of the dermal sinus.…”

“…The reddish skin lesion and the small dermal tract were only examined just after preoperative shaving of the head. For exposing the extent of the tumor and connection with the other anatomical structures, CT and MRI are valuable [6, 20, 21]. …”

“…Total microsurgical resection of the CDT is the main intention but could not be achieved due to the adhesion with the surrounding structures [6]. Schijman et al [22] recommend a percutaneous aspiration of the dermoid cyst before total resection.…”

“…Intracranial dermoid tumor (CDT) is a congenital benign tumor which accounts for 0.1–0.7% of all intracranial tumors [6]. It is a rare dystrophic lesion that grows slowly and is characterized by progressive epithelial desquamation and gland secretion within the cyst [7].…”