A 2-year-old girl presented with a congenital occipital lump centered by a tuft of coarse hair with a small orifice (Figure, A); this had grown and developed a purulent flow over 1 year. She had no neurologic symptoms and no history of fever. Her general practitioner prescribed topical antiseptic and antibiotic, but the lump recurred. An ultrasound scan revealed a subcutaneous fistulous tract communicating with a deeper mass lysing the occipital bone. Computed tomography and magnetic resonance imaging scans documented a dermal sinus connected to an intradiploic occipital dermoid cyst (Figure, B-E).The full lesion was resected through a suboccipital approach. The cyst wall was opened, revealing a yellow, smooth and nonhemorrhagic content, composed of sebum and hair follicles ( Figure, F). Postoperative course was uneventful, and histopathologic examination confirmed the diagnosis of dermoid cyst.A congenital dermal sinus consists of a tract lined by epidermis that can connect the skin and the deeper tissues of the central nervous system. 1 It is often clinically occult, but in infants the presence of a midline cutaneous pit or mass should raise awareness, particularly in the occipital or lumbosacral regions. 2,3 Dermoid cyst is a congenital benign neoplasm resulting from inclusion of ectodermal elements within the neural tube during its closure between the third and the fifth weeks of embryonic development and, thus, occurs in the midline. 4 It accounts for 0.1%-0.7 % of all intracranial tumors. 4 The association of the 2 lesions, which is well-known, should be suspected and investigated in depth, especially when a cutaneous lesion, such as a lump or pit, lies on the midline. 5 This association can predispose to recurrent meningitis and intracranial suppuration by allowing cutaneous micro-organisms to penetrate into the cyst by means of the sinus tract, explaining their early detection during childhood. 5 Computed tomography and magnetic resonance imaging findings of dermoid cysts can vary according to the relative proportions of the elements of a dermoid cyst. A hyperintense signal on T1 and hypointense signal on T2-weighted images indicates the presence of lipid inside the cyst, as in our case. 6 Dermoid cysts are well-circumscribed lesions lined by stratified squamous epithelium. They contain thick, viscous, and yellowish material composed of secretion of sebaceous glands, calcifications, desquamated epithelium, and whorls of hair. Treatment consists of complete surgical removal of the tract and the cyst. 4 ■
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