Anterior meningocele is a rare disease which is commonly observed in the sacral region. Anterio cervicothoracic meningocele is generally diagnosed in adult age and there are few reports in infancy. Different treatment procedures are performed such as ligation, resection and wrapping. The case of a 9-month-old male infant with acute respiratory distress and cyanosis is described. He had also recurrent lung infections for 3 months. The magnetic resonance imaging revealed an anterior cervicothoracic meningocele which was compressing the lung, aorta, esophagus and trachea associated with skeletal abnormalities and C2 - C7 syringohydromyelia. There was no evidence of neurofibromatosis. He was submitted to thoracotomy and treated with cystopleural shunt without valve system. Postoperatively his respiratory distress improved dramatically. Radiologically, the size of meningocele decreased and the syringomyelic cavity disappeared. There was no evidence of recurrence during his followup. Cystopleural shunt procedure is a reliable, simple and effective treatment modality in anterior cervicothoracic meningocele cases. This treatment regimen prevents possible neural damage and also treatments to the associated anomalies such as syringohydromyelia.

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