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puncture or drainage and to perform this testing at periodic intervals for several days after the procedure in order to be certain that improvement is genuine Even more persuasive is a de nite improvement followed days later by worsening of gait As a group, patients who have a sustained response to drainage of CSF by shunting, as described below, have had the rst two elements of the clinical triad (only half of our successfully treated patients have been incontinent), and their lateral ventricular span at the level of the anterior horns was in excess of 50 mm (a true dimension calculated from CT scans), equivalent to approximately 18 mm on the usual CT lm, and at the same time, relatively little cerebral atrophy Treatment of Hydrocephalus in Adults The development of ventricular shunt tubing with one-way valves opened the way to successful treatment of hydrocephalus The valve can be set at a desired opening pressure, allowing the CSF to escape directly into the bloodstream (ventriculoatrial shunt), peritoneal cavity (ventriculoperitoneal shunt), or pleural space whenever the pressure level is exceeded Gratifying success can be obtained, often a complete or nearly complete restoration of mental function and gait, by the placement of such shunts Regarding NPH, the most consistent improvement has been attained in that minority of patients in whom a cause could be established (subarachnoid hemorrhage, chronic meningitis, or tumor of the third ventricle) As already noted, other predictors of success are considerable enlargement of the ventricles in comparison to the degree of cortical atrophy, CSF pressures above 155 mmH2O, and improvement after spinal puncture, but none of these is entirely dependable Deviations from the characteristic syndrome such as the occurrence of dementia without gait disorder or the presence of apraxias, aphasias, and other focal cerebral signs should lead one to a diagnosis other than NPH Fisher, on analyzing successfully shunted cases, noted that almost without exception gait disturbance was an early and prominent symptom Uncertainties of diagnosis increase with advancing age owing to the frequent association of degenerative dementia and vascular lesions However, in Fisher s experience, age alone does not exclude the existence of NPH as a cause of gait disorder, and long duration of symptoms does not preclude a salutary outcome from shunting In patients who are averse to the shunting procedure or who have medical conditions that make the surgery inadvisable, it is sometimes possible to produce a reasonable improvement in gait for several months by repeating the spinal puncture and drainage of large amounts of uid every few weeks or months The potential failure of shunting must be anticipated in patients who do not conform to the typical syndrome or whose disease has advanced to the stage of long-standing incontinence or dementia In some instances, a lack of improvement is explained by inadequate decompression, which justi es a revision of the shunt with a valve that drains at lower pressures Overdrainage, in contrast, causes headaches that may be chronic or orthostatic and may be associated with small subdural collections of uid These uid collections, or hygromas, consisting of CSF and proteinacious uid derived from blood products, are generally innocuous and do not require drainage unless they enlarge or cause focal neurologic symptoms or, rarely, seizures Some surgeons prefer to insert an externally programmable valve that allows adjustments if the ventricles do not reduce in size or if the opposite, overdrainage, occurs Although shunting is relatively simple as a surgical procedure, it is associated with complications, the main ones being a postop-.

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erative subdural hygroma or hematoma (even at times, if the ventricular pressure is reduced gradually, the bridging dural veins may stretch and rupture); infection of the valve and catheter, sometimes with ventriculitis and occasionally bacteremia; occlusion of the tip of the catheter in the ventricle; and, particularly in infants and children, the slit ventricle syndrome (see below) Orthostatic headaches can be overcome by raising the opening pressure of the shunt valve and, if the headaches persist, by utilizing an externally programmable valve Misplacement of the catheter may rarely transect tracts in the deep hemispheral white matter and cause serious neurologic de cits It is our impression that this occurs more often when the catheter is inserted from the back rather than through the frontal or parietal regions The incidence of blockage may be reduced by placing the catheter in the anterior horn of the right ventricle, where there is no choroid plexus Meticulous aseptic technique and the preoperative and postoperative administration of antibiotics have apparently reduced the incidence of shunt infections Most shunts in adults are brought to termination in the peritoneum (ventriculoperitoneal shunt) Rare complications of ventriculoatrial shunting are pulmonary hypertension and pulmonary embolism and nephritis, which is due to low-level infection of the shunt tube with Staphylococcus Puncture of the oor of the fourth ventricle by endoscopic techniques ( third ventriculostomy ) has been explored as an alternative to shunting, but, for a number reasons, it has not attained popularity among surgeons Once the CSF is shunted, the ventricles diminish in size within 3 or 4 days even when the hydrocephalus has been present for a year or more This indicates that hydrocephalic compression of the cerebrum is largely reversible Indeed, in Black s series, the ventricles failed to return to normal in only 1 of his 11 shunted patients, and in that patient there was no clinical improvement Clinical improvement occurs within a few weeks, the gait disturbance being slower to reverse than the mental disorder Symptoms of cerebral atrophy due to Alzheimer disease and related conditions are not altered by shunting, but this approach has been periodically resurrected, as discussed by Silverberg Treatment of Infantile and Childhood Hydrocephalus Here one encounters more dif culties than in the treatment of the adult disorder The ventricular catheter may wander or become obstructed and require revision Peritoneal pseudocysts may form (most shunts in children are ventriculoperitoneal) Another unexpected complication of shunting has been collapse of the ventricles, the so-called slit ventricle syndrome (the appearance of the ventricles on imaging studies is slit-like) This occurs more frequently in young children, though we have observed it in adults These patients develop a low-pressure syndrome with severe generalized headaches, often with nausea and vomiting, whenever they sit up or stand Some children become ataxic, irritable, or obtunded or may vomit repeatedly The CSF pressure is extremely low and the volume of CSF much reduced In babies, the cranium may fail to grow even though the brain is of normal size In most shunted patients, the ICP in the upright position is diminished to 30 mmH2O; but in patients with the low-pressure shunt syndrome, it may reach 100 to 150 mmH2O To correct the condition, one would imagine that replacing the shunt valve with another that opens under a higher pressure would suf ce Indeed, this may be successful But once the condition is established, the most effective measure has been the placement of an antisiphon device, which prevents valve ow when the patient stands (See further on for a discussion of intracranial hypotension in adults).

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