A term baby was born by Caesarean section for failure to progress in labour following an uncomplicated pregnancy to a primigravida mother. Antenatal scans were normal. A 3.4 kg (50th centile) baby girl was born requiring no resuscitation. However, the baby had frontal bossing, a large anterior fontanelle and widely separated sutures with a head circumference of 39.7 cm (>99.6th centile). A neurological examination showed mild generalised hypotonia but was otherwise normal and she was able to begin establishing oral feeds.
A cranial ultrasound scan () showed hydrocephalus and an urgent CT brain showed marked ventriculomegaly with associated white matter change and calcification scattered throughout the cerebellum and occipital lobe. MRI angiography of the brain () confirmed a very large heterogeneous posterior fossa tumour causing massive obstructive hydrocephalus supplied by multiple blood vessels. There was no evidence of metastases elsewhere.
Following lengthy discussions with her parents, it was agreed to proceed with resection given the baby’s good current neurological condition. Embolisation of several vessels was carried out prior to tumour resection but despite this there was significant bleeding leading to cardiac arrest and death. Tumour histology confirmed an immature teratoma not likely to be due to an inherited predisposition.