Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa...

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Djamila KafoufiEl Galaa Military Hospital

Cairo, Egypt

Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle encloses a single ,large multiloculated cavity.

Compartments within the ventricular system.

Many designations : Multiseptate HDC, Polycystic HDC, Polycystic Brain disease, Ventricular Compartmentalization, Intraventricular Septum..

In the last 30 y, the mortatlity 50 and 70%.

Nowdays mortality decreased /very poor quality of life(severe psychomotor retardation, semivegetativestatus).

The progressive dilatation of the loculated cavities is IIary to CSF Production inside the isolated compartment,

Entrapment CSF by a valve mecanism,

Futur formation of inflammatory exudates(meningitis),

Osmotic gradient caused by lysis of clotted blood(posthemorrhage) .

1ST time 1985 by Eller and Pasternak ,a case of unilateral ventricular dilatation caused by clot and obstruction of the foramen of Monro,

And a case of delayed isolated lateral Vc caused by chronic inflammation of ependyma, formation of ependymal flaps, then membranes.

Jamjoon et al. found in most of their patients who developed MH after SI had other predisposing factors(IV hemorrhage or neonatal meningitis as the initial cause).

Nida &Haines suggested that the pathological process of MH become quicker after SI.

MH should be regarded as a condition that predisposes to persistent and recurrent shunt infection.

Courtesy Sgouros

Direct ependymal trauma during catheter insertion may contribute to loculation.

Overdrainage of CSF might result in unilateral ventricular collapse or in obstruction of the acqueduc(Chiari II and Meningocele)

Child Nerv.Syst (2011) 27:1609-1619

Courtesy Zuccaro

High morbidity.

septa is developped by intraVc antibiotic therapy administrated before the compartmentalization, and the role of elevated fluid pr©.

Severe Factors as Premature birth, Perinatal cpl, Congenital CNS malformation are associated with MH because the high risk of bacterial meningitis.

MH has been reported as a consequence of Tumor removal in children and adults.

Supratentoriel malignant glioma was associated with ventricular entry during surgery>>intraventricular precipitation>>glial proliferation and formation of septa>>compartment.

IntraV. Septations rarely occur at birth.

Congenital septa may be caused by developmental error,oftenassociated with other CNS or systemic anomalies,intrauterineinflammatory process(congenital Toxoplasmosis or Cytomegalovirus infection).

Bauman et al. have described obstruction of the a foramen Monro in a case of intrauterine Mumps ependimitis.

New born with Ependymitis

In the case of cystic periV leukomalecia and cystic encephalopathies, the lesion may enlarge>>become confluent>>multicompartmentalcavities

The choice of temporary diversion is determinatedby the severity of the infection, the hege and the resources available.

EVD = continuous drainage but not for long periods du to infection ,and child in bed for a long time and big cost.

VP shunt conversion is 56 days(R.Kaviattil 2008).

Avoid ventricular taps as causes parenchymal injury and gliotic changes.

Simple to perform,

Physiological drainage of the csf,

Prevent the brain by dampening the P0 wave,

Some amount of csf absorbed in the subgaleal space so reducing Pr and electrolytis loss.

VP shunt is required after 2 months of a functioning VSG.

MH challenging pathology that creates a feeling of failure in the neurosurgeon, and generate frustration and tedium in the Doctor-Patient relationship.