Cranial growth restriction, a fundamental measure for success of the endoscopy in children under 1 month of age. Is it possible to improve the outcome?

BACKGROUND: Endoscopic third ventriculostomy has been shown to be efficient for the treatment of non-communicating hydrocephalus. However, it is not recommended as the first option in the treatment of obstructive hydrocephalus in children under 3 months of age, because the success rate is less than 35%. METHODS: We reviewed all the cases of triventricular hydrocephalus treated between 2007 and 2011 in patients under 1 month of age in the case of normal term births or under 1 month of corrected age, in the case of pre-term births. The first treatment option was endoscopic fenestration and a restriction of cranial volume during the two months after surgery. RESULTS: Ten patients under 1 month of age underwent 13 ventriculostomies for non-communicating hydrocephalus of varying etiology (suprasellar arachnoid cyst (3), stenosis of the Sylvian aqueduct (2), post-infectious meningitis (3), and intrauterine bleeding (2)). Three required surgical endoscopic revision at 3, 4, and 5 months, respectively, after the initial surgery due to progressive ventricular enlargement. One of these three patients presented with Klebsiella pneumoniae ventriculitis as a complication after the second endoscopy. After a mean follow-up of 32 months, none has required a shunt. CONCLUSIONS: In our limited experience in triventricular hydrocephalus in patients under 1 month, the third ventriculostomy technique may be a better option than the shunt in selected cases.

Intracranial hypertension in two cases of craniometaphyseal dysplasia: differing surgical options.

Craniometaphyseal dysplasia (CMD) is a very rare bone disorder characterized by abnormally developed metaphyses in long bones and sclerosis of the craniofacial bones. In this paper, the authors report 2 cases of children diagnosed with CMD and chronic intracranial hypertension with deletion in exon 9 of the human ANK gene (ANKH). After intracranial monitoring, a different treatment was chosen for each patient. One of the patients was treated using CSF shunting because ventriculomegaly in the absence of a Chiari malformation was also observed on cerebral MR imaging. The other patient underwent cranial expansion and decompressive craniotomy of the posterior fossa, because ventriculomegaly was excluded after cerebral MR imaging and cervical MR imaging showed a Chiari malformation Type I. The origin of intracranial hypertension in CMD is multifactorial. Previous intracranial pressure monitoring and a thorough understanding of neuroimaging studies are essential to achieve an accurate diagnosis and effective treatment.