Dermoid tumours in children usually occur in two locations: at the anterior fontanelle and on the occipital squama. An exceptional site of origin for a posterior fossa dermoid cyst is the extradural space. There are only six previous cases of this situation reported in the literature. A series of 103 subscalp and calvarial masses in children were reviewed and three children are reported with extradural dermoids of the posterior fossa, which communicated with the skin through midline occipital dermal sinuses. All three children were seen after the rapid growth or the formation of an abscess in a previously noted occipital subcutaneous mass present since birth. Although computed tomography or magnetic resonance imaging showed the dermal sinus and the intracranial tumour, these studies were unable to ascertain the intradural or extradural nature of the tumours, their exact origin only being established at operation.Histopathological study showed preclinical signs of infection in the two patients that had not yet formed an abscess. It is suggested that early neurosurgical treatment of these neoplasms should be done to prevent the development of severe intracranial infection. The previously reported simplicity of surgical removal of occipital extradural dermoids was not confirmed in this series.
The case of a neonate with a dermoid cyst originating within an occipital encephalocele is reported. The finding of the tumor was made during the routine repair of the meningoencephalocele. To our knowledge this is the first time that this association has been described.
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