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Congenital spinal tumor in a patient with encephalocele and hydrocephalus: a case report

DOI: 10.1186/1752-1947-5-9

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Abstract:

We report the case of an Iranian girl with a history of encephalocele surgery, who, at the age of four years, developed an intramedullary spinal teratoma, and discuss the pathogenesis of this association.To the best of our knowledge, this is the first report of an association between encephalocele and spinal teratoma.Encephalocele refers to a group of rare congenital anomalies of the central nervous system (CNS), where brain tissue protrudes from a defect in the skull [1]. Its prevalence has been estimated to be 0.8 to four in every 10,000 live births [2].Teratomas are tumors derived from all three germ layers [3]. In children, teratomas are more commonly found in the sacrococcygeal region than in the spinal cord [4], which occurs in one of 38,500 viable births. Intramedullary spinal teratomas are rare tumors [5]. In 41.7% of teratomas, a concomitant anomaly of the vertebral canal is found, most commonly a diastematomyelia, [4]. However to the best of our knowledge, there is nor repot of an association with encephalocele in the English literature.We report a case of encephalocele and lumbar intramedullary teratoma and discuss the possible etiology.A four-year old Iranian girl was referred to the neurosurgical department with severe back pain and motor regression. She was the second child of nonconsanguineous parents, and was delivered by elective Cesarean section due to being repeat. She had a history of occipital encephalocele, which was treated surgically during the neonatal period and she later received a shunt to treat progressive hydrocephalus. She could sit at nine month of age and stand at two years, but was unable to walk. Six months before her referral, she had developed back pain, which was particularly severe at night, and after three months, she was unable to stand.On physical examination, our patient was found to be generally normal, with good mental performance, and normal results from a neurological examination of the arms. She had a head circumferenc

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