Hydrocephalus - demonstrační kasuistika

Noncommunicating obstructive hydrocephalus caused by obstruction of the foramina of Luschka and Magendie. This MRI sagittal image demonstrates dilatation of lateral ventricles with stretching of corpus callosum and dilatation of the fourth ventricle.

Noncommunicating obstructive hydrocephalus caused by obstruction of foramina of Luschka and Magendie. This MRI axial image demonstrates dilatation of the lateral ventricles.

Noncommunicating obstructive hydrocephalus caused by obstruction of foramina of Luschka and Magendie. This MRI axial image demonstrates fourth ventricle dilatation.

Communicating hydrocephalus with surrounding "atrophy" and increased periventricular and deep white matter signal on fluid-attenuated inversion recovery (FLAIR) sequences. Note that apical cuts (lower row) do not show enlargement of the sulci.

Anamnéza:

Normal pressure hydrocephalus (NPH) describes a condition that rarely occurs in patients younger than 60 years.2 Enlarged ventricles and normal CSF pressure at lumbar puncture (LP) in the absence of papilledema led to the term NPH. However, intermittent intracranial hypertension has been noted during monitoring of patients in whom NPH is suspected, usually at night. The classic Hakim triad of symptoms includes gait apraxia, incontinence, and dementia. Headache is not a typical symptom in NPH.

Zobrazovací metody:

CT/MRI criteria for acute hydrocephalus include the following:

• Size of both temporal horns is greater than 2 mm, clearly visible. In the absence of hydrocephalus, the temporal horns should be barely visible.
• Ratio of the largest width of the frontal horns to maximal biparietal diameter (ie, Evans ratio) is greater than 30% in hydrocephalus.
• Transependymal exudate is translated on images as periventricular hypoattenuation (CT) or hyperintensity (MRI T2-weighted and fluid-attenuated inversion recovery [FLAIR] sequences).
• Ballooning of frontal horns of lateral ventricles and third ventricle (ie, "Mickey mouse" ventricles) may indicate aqueductal obstruction.
• Upward bowing of the corpus callosum on sagittal MRI suggests acute hydrocephalus.

Diagnóza:

Diferenciální diagnóza:

Brainstem syndromes
Macrocephaly
Hydranencephaly
Chronic subdural hemorrhages
Cerebral atrophy
Cerebral tumors
Periaqueductal glioma
Agenesis of corpus callosum
Septo-optic dysplasia
Neuroimaging of vascular malformations and hematomas of the brain

Histologie:

• Thinning and stretching of the cortical mantle may be seen as a result of ventricular dilation.
• In the acute phase, edema of the periventricular white matter is observed. Relatively few neuronal lesions are present. Ventricular ependyma shows cellular flattening and loss of cilia.
• At a later stage, the edema disappears and is replaced by fibrosis, axonal degeneration, demyelination, focal loss of cerebral cortical neurons, cellular flattening, and further loss of cilia.

Terapie:

Acetazolamide (ACZ) and furosemide (FUR) treat posthemorrhagic hydrocephalus in neonates. Both are diuretics that also appear to decrease secretion of CSF at the level of the choroid plexus. ACZ can be used alone or in conjunction with FUR. The combination enhances efficacy of ACZ in decreasing CSF secretion by the choroid plexus. If ACZ is used alone, it appears to lower risk of nephrocalcinosis significantly.

Diskuze/komentář:

Medication as treatment for hydrocephalus is controversial. It should be used only as a temporary measure for posthemorrhagic hydrocephalus in neonates.

Klasifikace ICD-10:

G91.1 Obstrukční hydrocefalus