Ethmoidal meningoencephalocele and CSF leak after posthaemorrhagic ventricular dilatation in a newborn child

Background Rhinoliquorrhoea suggests a communication between the subarachnoid space and the sinonasal tract. Clinical presentation includes clear nasal discharge, headache, pneumocephalus, meningitis or brain abscess. Cerebrospinal fluid (CSF) leaks are mostly of traumatic origin (skull base fractures), iatrogenic (secondary to endoscopic endonasal surgery) or associated with tumour aetiology. Occasionally, hydrocephalus has been the cause of rhinoliquorrhoea in adults, presumably secondary to the chronically raised intracranial pressure with skull base erosion and meningocele. To our knowledge, the association of hydrocephalus and ethmoid meningoencephalocele/CSF leak has not been previously reported in a newborn child. Case presentation We present the case of a 9-month-old girl who was referred for rhinorrhoea. She had a history of posthaemorrhagic ventricular dilatation. Brain computed tomography (CT) and magnetic resonance imaging (MRI) showed a left ethmoidal meningoencephalocele and small ventricular size. The meningoencephalocele was surgically repaired using an intradural subfrontal approach. During the postoperative period, after the transient lumbar drain was withdrawn, she developed symptomatic hydrocephalus. Ventriculoperitoneal shunting was required. Conclusion Progressive ventricular dilatation may arise from a meningoencephalocele/CSF leak in paediatric patients. Early identification and repair of the meningoencephalocele are critical to avoid development of complications.