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Hydrocephalus.

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Presentation on theme: "Hydrocephalus."— Presentation transcript:

1 Hydrocephalus

2 Hydrocephalus Not a specific disease
Represents a diverse group of conditions that result from impaired circulation and absorption of CSF Hydrocephalus is not a specific disease; rather, it represents a diverse group of conditions that result from impaired circulation and absorption of CSF or, in the rare circumstance, from increased production by a choroid plexus papilloma ( Table ).

3 CSF Pathway Normally, CSF flows from the lateral ventricles through the foramina of Monro into the 3rd ventricle. It then traverses the narrow aqueduct of Sylvius, which is ≈3 mm long and 2 mm in diameter in a child, to enter the 4th ventricle. The CSF exits the 4th ventricle through the paired lateral foramina of Luschka and the midline foramen of Magendie into the cisterns at the base of the brain Hydrocephalus resulting from obstruction within the ventricular system is called obstructive or noncommunicating hydrocephalus. The CSF circulates from the basal cisterns posteriorly through the cistern system and over the convexities of the cerebral hemispheres. CSF is absorbed primarily by the arachnoid villi through tight junctions of their endothelium by the pressure forces that were noted earlier. CSF is absorbed to a much lesser extent by the lymphatic channels directed to the paranasal sinuses, along nerve root sleeves, and by the choroid plexus itself. Hydrocephalus resulting from obliteration of the subarachnoid cisterns or malfunction of the arachnoid villi is called nonobstructive or communicating hydrocephalus.

4 Hydrocephalus Noncommunicating Communicating

5 Noncommunicating Hydrocephalus
Obstructive Hydrocephalus resulting from obstruction within the ventricular system Aqueductal stenosis  Infectious X-linked Chiari malformation Dandy-Walker malformation Klippel-Feil syndrome Mass lesions  Abscess  Hematoma Tumors and neurocutaneous disorders  Vein of Galen malformation  Walker-Warburg syndrome Hydrocephalus resulting from obstruction within the ventricular system is called obstructive or noncommunicating hydrocephalus. The CSF circulates from the basal cisterns posteriorly through the cistern system and over the convexities of the cerebral hemispheres. CSF is absorbed primarily by the arachnoid villi through tight junctions of their endothelium by the pressure forces that were noted earlier. CSF is absorbed to a much lesser extent by the lymphatic channels directed to the paranasal sinuses, along nerve root sleeves, and by the choroid plexus itself. Hydrocephalus resulting from obliteration of the subarachnoid cisterns or malfunction of the arachnoid villi is called nonobstructive or communicating hydrocephalus.

6 Communicating Hydrocephalus
Nonobstructive Hydrocephalus resulting from obliteration of the subarachnoid cisterns of malfunctoin of the arachnoid villi Achondroplasia  Basilar impression Benign enlargement of subarachnoid space Choroid plexus papilloma Meningeal malignancy Meningitis Posthemorrhagic Hydrocephalus resulting from obliteration of the subarachnoid cisterns or malfunction of the arachnoid villi is called nonobstructive or communicating hydrocephalus.

7 Clinical Features of HCP
Variable, depends on many factors Age at onset, nature of lesion causing obstruction, rate of increase of ICP Infant Rapidly enlarging head most prominent sign Anterior fontanelle is wide open and bulging Scalp veins dilated Broad forehead Setting-sun eye sign Eyes deviated downward Due to impingement of dilated suprapineal recess on the tectum the age at onset, the nature of the lesion causing obstruction, and the duration and rate of increase of the intracranial pressure (ICP). eyes may deviate downward because of impingement of the dilated suprapineal recess on the tectum

8 Clinical Features of HCP
Long-tract signs Brisk tendon reflex Spasticity Clonus Babinski Long-tract signs including brisk tendon reflexes, spasticity, clonus (particularly in the lower extremities), and Babinski sign are common owing to stretching and disruption of the corticospinal fibers originating from the leg region of the motor cortex

9 Clinical Features of HCP
Older child Cranial sutures partially closed Signs of hcp subtler Irritability Lethargy Poor appetite Vomiting Headache Papilledema Abducens nerve palsy Pyramidal tract signs Macewen sign Percussion of skull  cracked pot sound Indcateds separation of sutures In an older child, the cranial sutures are partially closed so that the signs of hydrocephalus may be subtler. Irritability, lethargy, poor appetite, and vomiting are common to both age groups, and headache is a prominent symptom in older patients. A gradual change in personality and a deterioration in academic productivity suggest a slowly progressive form of hydrocephalus. Serial measurements of the head circumference indicate an increased velocity of growth. Percussion of the skull may produce a cracked pot sound or Macewen sign, indicating separation of the sutures. A foreshortened occiput suggests Chiari malformation, and a prominent occiput suggests the Dandy-Walker malformation. Papilledema, abducens nerve palsy, and pyramidal tract signs, which are most evident in the lower extremities, are apparent in most cases. Papilledema, abducens nerve palsy, and pyramidal tract signs, which are most evident in the lower extremities, are apparent in most cases.

10 Clinical Features of HCP
Chiari malformation Foreshortened occiput Dandy-Walker malformation Prominent occiput A foreshortened occiput suggests Chiari malformation, and a prominent occiput suggests the Dandy-Walker malformation. Papilledema, abducens nerve palsy, and pyramidal tract signs, which are most evident in the lower extremities, are apparent in most cases.

11 Chiari Malformation Type I
Produces sx during adolescence or adult life Usually not associated with hcp Recurrent headache, neck pain, urinary frequency, progressive lower extremity spasticity Consists of displacement of cerebellar tonsils into the cervical canal Unknown pathogenesis Type I typically produces symptoms during adolescence or adult life and is usually not associated with hydrocephalus. Patients complain of recurrent headache, neck pain, urinary frequency, and progressive lower extremity spasticity. The deformity consists of displacement of the cerebellar tonsils into the cervical canal ( Fig ). Although the pathogenesis is unknown, a prevailing theory suggests that obstruction of the caudal portion of the 4th ventricle during fetal development is responsible. Other theories include tethering of the cord or additional anomalies (syrinx).

12 Chiari Malformation Type II Progressive hcp with a myelomeningocele
Represesents an anomaly of the hindbrain due to failure of the pontine flexure during embryogenesis Results in elongation of the 4th ventricle and kinking of brainstem Displacement of inferior vermis, pons, medulla into the cervical canal The type II Chiari malformation is characterized by progressive hydrocephalus with a myelomeningocele. This lesion represents an anomaly of the hindbrain, probably due to a failure of pontine flexure during embryogenesis, and results in elongation of the 4th ventricle and kinking of the brainstem, with displacement of the inferior vermis, pons, and medulla into the cervical canal ( Fig ). Approximately 10% of type II malformations produce symptoms during infancy, consisting of stridor, weak cry, and apnea, which may be relieved by shunting or by posterior fossa decompression. A more indolent form consists of abnormalities of gait, spasticity, and increasing incoordination during childhood. Plain skull radiographs show a small posterior fossa and a widened cervical canal. CT scanning with contrast and MRI display the cerebellar tonsils protruding downward into the cervical canal and the hindbrain abnormalities. The anomaly is treated by surgical decompression.

13 Chiari Malformation 10% produce sx during infancy Stridor Weak cry
apnea Approximately 10% of type II malformations produce symptoms during infancy, consisting of stridor, weak cry, and apnea, which may be relieved by shunting or by posterior fossa decompression. A more indolent form consists of abnormalities of gait, spasticity, and increasing incoordination during childhood. Plain skull radiographs show a small posterior fossa and a widened cervical canal. CT scanning with contrast and MRI display the cerebellar tonsils protruding downward into the cervical canal and the hindbrain abnormalities. The anomaly is treated by surgical decompression.

14 Dandy-Walker Malformation
Consists of a cystic expansion of the 4th ventricle in the posterior fossa and midline cerebellar hypoplasia Results from a developmental failure of the roof of the 4th ventricle during embryogenesis 90% have hcp Associated anomalies Agenesis of the posterior cerebellar vermis and corpus callosum Infants present with a rapid increase in head size + prominent occiput The Dandy-Walker malformation consists of a cystic expansion of the 4th ventricle in the posterior fossa and midline cerebellar hypoplasia, which results from a developmental failure of the roof of the 4th ventricle during embryogenesis ( Fig ). Approximately 90% of patients have hydrocephalus, and a significant number of children have associated anomalies, including agenesis of the posterior cerebellar vermis and corpus callosum. Infants present with a rapid increase in head size and a prominent occiput. Transillumination of the skull may be positive. Most children have evidence of long-tract signs, cerebellar ataxia, and delayed motor and cognitive milestones, probably due to the associated structural anomalies. The Dandy-Walker malformation is managed by shunting the cystic cavity (and on occasion the ventricles as well) in the presence of hydrocephalus.

15 Dandy-Walker Malformation
(+) skull transillumination Long-tract signs Cerebellar ataxia Delayed motor and cognitive milestones Most children have evidence of long-tract signs, cerebellar ataxia, and delayed motor and cognitive milestones, probably due to the associated structural anomalies. The Dandy-Walker malformation is managed by shunting the cystic cavity (and on occasion the ventricles as well) in the presence of hydrocephalus.

16 Baby Girl Mendoza CT scan results

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