Fax +41 61 306 12 34 E-Mail karger@karger.ch www.karger.com Case Report Pediatr Neurosurg 2010;46:39–42 DOI: 10.1159/000315000 Infratorcular Meningoencephalocele with Dermoid Cyst Amit Mahore Alejandro Fonseca Nitin Dange Rakesh Redhu Dattatreya Muzumdar Atul Goel Department of Neurosurgery, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, India cyst has been reported [3–6]. We report a case of infrator- cular occipital meningoencephalocele with dermoid cyst. The finding of the dermoid was made during the routine repair of the meningoencephalocele. Case Report A 12-month-old female child, born by full-term normal deliv- ery, was brought with the complaint of a progressively enlarging congenital swelling on the back of the head. There was history of recurrent episodes of fever. On admission, the patient was con- scious, playful and actively moving all four limbs. Developmental milestones were normal. Local examination showed soft, fluctu- ant, translucent swelling in the occipital area with a bony defect palpable at the margins. It was nontender without signs of inflam- mation and no leakage of CSF. Sinus or ulceration of overlying skin was conspicuously absent. An MRI revealed a Chiari type 3 malformation without hydrocephalus. There was a defect in the occipital bone in the midline below the inion and torcula with herniation of the vermis and paravermian cerebellar tissue into a CSF-containing sac ( fig. 1a, b). The patient underwent repair of her meningoencephalocele. A transverse elliptical skin incision was taken around the base of the encephalocele sac and deepened up to the dura. To our surprise, there was an epidural dermoid cyst with fat and hair content near the neck of the sac (fig. 2). The dermoid cyst was adherent to the surrounding tissues and dura. It was excised in toto. The dural sac was excised, keeping some redundant dura to accommodate the Key Words Occipital encephalocele  Dermoid cyst  Meningoencephalocele  Neural tube defect Abstract Encephalocele represents one end of the spectrum of open neural tube defects. We report an infant with Chiari type 3 malformation manifesting as infratorcular occipital menin- goencephalocele with dermoid cyst. To our knowledge, this is the first time that this association is described. The patho- genesis, surgical treatment, prognosis and management dif- ficulties are also being discussed. Copyright © 2010 S. Karger AG, Basel Introduction Encephalocele represents one end of the spectrum of open neural tube defects. Encephalocele is defined as ce- rebral tissue herniation through a congenital or acquired defect in the skull. The majority of congenital encepha- loceles occur in the occipital or frontal regions [1, 2]. Der- moid cyst with frontal encephalocele is not uncommon, but only one case of occipital encephalocele with dermoid Received: June 3, 2009 Accepted after revision: December 4, 2009 Published online: May 27, 2010 Dr. Amit Mahore Department of Neurosurgery King Edward VII Memorial Hospital Parel, Mumbai 400012 (India) Tel. +91 993 040 0107, Fax +91 22 2414 3435, E-Mail amit2mahore @ yahoo.co.in © 2010 S. Karger AG, Basel 1016–2291/10/0461–0039$26.00/0 Accessible online at: www.karger.com/pne