The unusual case of a 10-month-old infant with an ectopic refluxing ureter entering into a seminal vesical cyst associated with ipsilateral renal dysplasia is described. The embryology, differential diagnosis, and therapy are discussed.
Four infants with obstructive hydrocephalus caused by space occupying third ventricle and mesencephalic cysts are reported. Despite immediate shunt insertion in all patients, there was either lack of clinical improvement or late onset of clinical deterioration. Neuroimaging (CT, MRI, and ventriculography) diagnosed the presence of non-communicating midline outpouchings of the CSF pathways causing obstruction of aqueductal CSF flow and brainstem signs. The cysts were of different origin. In one patient it was caused by a previous thalamic haemorrhage, in another patient by neonatal Escherichia coli meningoventriculitis. In two cases with obstructive hydrocephalus at birth, the aetiology is unclear. Direct puncture and drainage of the cysts led to clinical improvement. The present report highlights the clinical and neuroradiological findings as well as the neurosurgical management in four cases with congenital and postnatally acquired obstructive hydrocephalus due to blockage of CSF flow by midline cysts or outpouchings of suprasellar subarachnoid spaces.
Case reports CASE 1After a normal pregnancy, the first daughter of healthy parents had to be delivered by caesarian section because of heart beat deceleration and craniopelvic disproportion. The neonatal period was uneventful. At the age of nine weeks she was referred to us because of accelerated head growth, poor suckling, and generalised hypotonia. Her head circumference was enlarged (42 cm; >P97) and the anterior fontanel was full and tense with separated cranial sutures. On clinical examination she had a high pitched cry, poor suckling and swallowing, generalised hypotonia with normal reflexes, and failure of visual fixation with disconjugate and roving eye movements. Obstructive hydrocephalus was confirmed by cranial ultrasonography and CT, which showed pronounced dilatation of the third and lateral ventricles as well as an infratentorial midline cyst ( fig 1A). After insertion of a ventriculoperitoneal shunt into the right frontal horn, the size of the ventricles diminished but clinical signs of hypotonia, poor feeding, and intermittent disconjugate upward eye deviation persisted. After one week follow up CT and MRI ( fig 1C and D) showed persistance of a CSF isodense midline cyst with pontomesencephalic location. The expanding cyst caused distortion of brainstem structures and compression of the aqueduct. Ventriculography (fig 1B) showed the absence of free communication between the ventricular system and the pontomesencephalic cyst. The life threatening brainstem compression by this expanding cystic lesion urged further neurosurgical intervention. A communication between the cyst and right lateral ventricle was established by
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