腦積水Hydrocephalus

1、hydrocephalusfrom greek hydrokephalos, from hydr- + kephale headdefinition: an abnormal increase in the amount of cerebrospinal fluid within the cranial cavity that is accompanied by expansion of the cerebral ventricles, enlargement of the skull and especially the forehead, and atrophy of the brain

2、overview of csf production the csf volume of an average adult ranges from 80 to 160 ml the ventricular system holds approximately 20 to 50 ml of csf csf is produced in the choroid plexuses at a daily rate of 14-36 ml/hr overview of csf production the choroid plexuses are the source of approximately

3、80% of the csf the blood vessels in the subependymal regions, and pia also contribute to the formation of csfoverview of csf circulation the csf flows from the lateral ventricles downward to the foramina of magendie and luschka, to the perimedullary and perispinal subarachnoid spaces, and then upwar

4、d to the basal cistern and finally to the superior and lateral surfaces of the cerebral hemispherescsf circulation the pressure gradient is highest in the lateral ventricles and diminishes successively along the subarachnoid space arterial pulsations in the choroid plexuses help drive the fluid from

5、 the ventricular system normally, the periventricular tissues offer little resistance to the flow of csf+-csf pressure the csf volume and pressure are maintained on a minute to minute basis by the systemic circulation csf pressure is in equilibrium with capillary pressure (determined by the arteriol

6、ar tone) an increase in blood pco2 (hypoventilation) decreases ph and arteriolar resistance, this in turn gives rise to increased csf pressure by increasing cerebral blood flow hyperventilation has the opposite effectcsf pressure normal intracranial pressure (icp) in an adult is between 2-8 mmhg. le

7、vels up to 16 mmhg are considered normal icp higher than 40 mmhg or lower bp may combine to cause ischemic damagecsf pressure increased venous pressure has a direct effect on csf pressure downstream block of venous flow increases the volume of cerebral veins, dural sinuses, and the subarachnoid spac

8、ethe function of the csf the csf acts as a “water jacket” for the brain and spinal cord the 1300 g adult brain weighs approximately 45 g when suspended in csfthe function of the csf the csf acts like a “sink”, effectively flushing waste products as new fluid is secreted reabsorbed a constant csf ele

9、ctrolyte composition helps maintain a stable medium for excitable cells (neurons)mechanisms of increase intracranial pressure brain, blood and csf are held in an inelastic container (cranium, vertebral canal and dura) changes in volume of either element (brain,csf, blood) is at the expense of the ot

10、her two hydrocephaluscommunicating vs. non-communicating (dandy) this is an old classification of hydrocephalus the terms refer to the presence or absence of a communication of the lateral ventricles with the spinal subarachnoid spacecommunicating vs. non-communicating this classification was based

11、on the imaging findings after injection of dye into the ventricular system and simultaneous injection of air into the subarachnoid space diffusion of dye into the subarachnoid space and passage of air into the ventricular space were the criteria for communicating hydrocephalus non-communicating hydr

12、ocephalus there is no communication between the ventricular system and the subarachnoid space. the commonest cause of this category is aqueduct blockage or stenosis. aqueductal stenosis the normal aqueduct measures about 1 mm in diameter, and is a b o u t 1 1 m m i n length.aqueductal stenosis is th

13、e most common cause of congenital hydrocephalus(43%) aqueduct develops about the 6th week of gestation m:f = 2:1 other congenital anomalies (16%): thumb deformities prognosis: 11-30% mortality etiology of aqueductal stenosis intrinsic pathology of the aqueductseptum or membrane formation: a thin mem

14、brane of neuroglia may occlude the aqueduct. it commonly occurs caudally. there may be a primary developmental defect or it may follow granular ependymitis from intrauterine infections. this is the rarest of the types of narrowing.forking of the aqueduct:typically, there are two channels seen in mid

15、sagittal plane unable to handle csf volume. most often seen with spina bifida.gliosis of the aqueduct: usually of infectious origin showing a marked gliofibrillary response. the lumen is devoid of ependyma.stenosis of the aqueduct: narrowed aqueduct without evidence of gliosis. this may have heredit

16、ary basis.etiology of aqueductal stenosis extrinsic pathology of the aqueduct:infectious. abscesses.neoplastic. pineal tumors, brainstem gliomas, medulloblastoma, ependymoma.vascular. avm, aneurysm, galen aneurysm.developmental. arachnoid cysts.clinical features of aqueductal stenosis o b s t r u c

17、t i v e hydrocephalus: presents with macrocephaly and/or intracranial hypertension. parinauds syndrome. inability to elevate eyes colliers sign. retraction of the eyelidsimaging of aqueductal stenosis ultrasonography can detect aqueductal stenosis in utero.sonogramimaging of aqueductal stenosis ct a

18、nd mri. mri is essential if third ventriculostomy is to be considered.treatment of aqueductal stenosis treatment and resultsremove underlying cause of obstruction if possible.third ventriculostomy as initial treatment of choice.vp shunt if technical reasons do not allow third ventriculostomy or i f

19、t h e c h i l d f a i l s a f t e r ventriculostomy.aqueductal stent can be placed if technically feasible. usually rarely done due to risk of upper brain stem injury.communicating hydrocephalus in communicating or non-obstructive hydrocephalus there is communication between the ventricular system a

20、nd the subarachnoid space. the commonest cause of this group is post-infectious and post-hemorrhagic hydrocephalus. causes of communicating hydrocephalus overproduction of csf blockage of csf circulation blockage of csf resorption hydrocephalus ex-vacuo normal pressure hydrocephalusoverproduction of

21、 csf excessive secretion of csf by the choroid plexus as in cases of choroid plexus papilloma or carcinoma. this is a rare cause. blockage of csf circulationthis could be at any level of the csf circulation. it could be at the level of the foramen of monro, with either unilateral or bilateral occlus

22、ion of the foramen of monro giving dilatation of one or both lateral ventricles. this is commonly seen in the colloid cyst and tumors of the third ventricle. dandy walker syndrome a common cause of obstructive hydrocephalus is dandy walker syndrome where there is blockage of foramina of the 4th vent

23、ricle. this is a congenital condition associated with agenesis of the cerebellar vermisblockage of csf resorption poor resorption of csf into the venous sinuses caused by scarring of the arachnoid villi and is commonly seen after meningitis or hemorrhagehydrocephalus ex vacuohydrocephalus ex-vacuo i

24、nvolves the presence of too much csf, although the csf pressure itself is normal. this condition occurs when there is damage to the brain caused by stroke or other form of injury or chronic neurodegeneration, and there may be an actual shrinkage of brain substance. normal pressure hydrocephalusnorma

25、l pressure hydrocephalus (nph) is usually due to a gradual blockage of the csf drainage pathways in the brain. nph is an unusual cause of dementia, which can occur as a complication of brain infection or bleeding (hemorrhage).normal pressure hydrocephalusin some patients, no predisposing cause can b

26、e identified. in patients with nph, although the ventricles enlarge, the pressure of the csf remains within normal range. nph is characterized by gradual memory loss (dementia), balance disorder (ataxia), urine incontinence, and a general slowing of activity.symptoms progressively worsen over weeks.

27、 in some patients, an improvement of symptoms is noted immediately after the removal of spinal fluid with a lumbar procedure.treatment of hydrocephalusthe two most commonly used shunt systems are the ventriculoatrial (va) and ventriculoperitoneal (vp) shunts. the vp shunt is most commonly used as it

28、 is simpler to place, extra tubing may be placed in the peritoneum and the consequences of infection are less. treatment of hydrocephalusthe va shunt must be accurately located in the atrium and requires frequent revisions as the child grows to maintain the proper position of the distal end. in addi

29、tion, infection is a more serious complication with a va shunt as its location in the blood stream may lead to sepsis. treatment of hydrocephalusrecently, in situations where both the abdomen and vascular system can no longer function to absorb csf, pediatric neurosurgeons have begun to place the di

30、stal catheter in the pleural space (v-pl shunt). the distal catheter is placed through a small incision in the anterior chest wall. as with the peritoneal shunt, extra tubing can be placed, reducing the need for further shunt revisions. treatment of hydrocephalusshunt systems include three component

31、s: (1) a ventricular catheter, (2) a one way valve and (3) a distal catheter. the ventricular catheter is a straight piece of tubing, closed on the proximal end and usually with multiple holes for the entry of csf along the proximal two centimeters of the tube. treatment of hydrocephalusshunts are c

32、omposed of a material called silastic. silastic is made from a family of polymerized organic compounds termed silicone. silicone is the substance that has caused controversy in breast implants because of the association with auto immune disorders. so far no cases of auto immune disease have been lin

33、ked to the silastic used in shunts. treatment of hydrocephalusthe most common sites for entry of the ventricular catheter are a frontal position in line with the pupil at the coronal suture, a parietal position just above and behind the ear, or an occipital position three centimeters off the posterior midline. the position used varies with the configuration of the ventricles, the shape and size of the