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

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Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt

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

Page 1: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle

Djamila KafoufiEl Galaa Military Hospital

Cairo, Egypt

Page 2: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle

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..

Page 3: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle

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

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

Page 4: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle

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) .

Page 5: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle
Page 6: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle

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.

Page 7: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle

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.

Page 8: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle

Courtesy Sgouros

Page 9: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle

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)

Page 10: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle

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

Courtesy Zuccaro

Page 11: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle

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.

Page 12: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle
Page 13: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle

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.

Page 14: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle

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.

Page 15: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle

New born with Ependymitis

Page 16: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle

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

Page 17: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle

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

Page 18: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle

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.

Page 19: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle

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.

Page 20: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle

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

Page 21: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle
Page 22: Djamila Kafoufi El Galaa Military Hospital Cairo, Egypt · Progressive disease with new septa appearing until the ventricular patern becomes unrecognizable and the cerebral mantle