Basal Encephalocele Associated with Teratoma; Pathogenesis and Management, Case Report

Shima Shahjouei, MD; Farideh Nejat*, MD, MPH; El Mostafa Khashab, MD, PhD 1. Department of Neurosurgery, Tehran University of Medical Sciences, Tehran, Iran 2. Children’s Medical Center, Pediatric Center of Excellence, Tehran, Iran 3. Department of Neurosurgery, Hackensack University Medical Center, New Jersey, USA Received: Jun 21, 2012; Accepted: Oct 31, 2012; Online Available: Dec 05, 2012 Encephaloceles are rare entities presenting as protrusion of intracranial structures through a defect in the skull[1,2]. Teratomas, tumors with the potential of giving rise to all three germ cell layers, are other rare entities which can involve the intracranial components. Here we describe a patient with basal encephalocele associated with teratoma. A 9-month-old girl was referred to the neurosurgical department due to small frontal mass since birth and progressive hypertelorism. On examination she had a bifid nose, prominent hypertelorism and small midline mass covered by abnormal skin and small thick hairs (Fig 1). She was normal in developmental status. Brain magnetic resonance imaging showed normal brain but nonhomogenous anterior fossa mass. Computed tomography scan confirmed a small hole in proximal portion of nasal bone, associated with a nonhomogenous mass between the two right and left ethmoidal sinuses that seemed to be in continuity with extracranial mass descending through the nasal bone defect (Fig. 1). Surgery was performed through bifrontal craniotomy. The nasal small defect contained a lipomatous tissue that traversed the bone defect to intracranial space and was going to anterior fossa intradurally to reach the basal mass. There was a 2×2 cm bone and dural defect in the anterior fossa, around foramen caecum, which was filled with basal mass (Fig. 2). The mass contained soft tissue and cartilaginous material that invaginated into nasal cavity and displaced both ethmoidal sinuses Fig. 1: A: Photograph of the patient shows hypertelorism, small midline mass associated with nasal pole widening. B: Brain CT scan reveals a small hole in the proximal portion of nasal bone. C: The mass is located between two ethmoidal sinuses displacing two sinuses laterally. laterally which was en bloc resected. The dural defect was repaired with pericranium patch graft and the basal bone defect was covered with bone harvested from posterior part of craniotomy. Pathological examination of the surgical specimen revealed a variety of tissues including neural tissue, muscle, cartilage, adipose tissue, vascular structures, and respiratory epithelium diagnosed as mature teratoma. The postoperative period was uneventful. She had regular follow up and now one year after surgery, she has normal development and growth without recurrence of the tumor or progression of her hypertelorism. Teratoma is the most frequent congenital tumor with early presentation at birth[3]. Head and neck teratomas account for 2-9% of all teratomas[2].