This chapter includes an accompanying lecture presentation that hhas been prepared by the authors: Video 41.1, KEY CONCEPTS * Idiopathic normal pressure hydrocephalus (NPE) is often undiagnosed or misdiagnosed because it presents with symptoms of gait dysfunction, urinary incontinence, and dementia that are common in other disorders. * The diagnosis of iNPH depends on several factors, including enlarged ventricles, advanced age, and clinical improvement after CSF drainage. © The cause of NPH has not been clearly established, although several reports of familial iNPH have been published in the literature. The incidence of familial iNPH has been reported to be between 7% and 11%. Additionally, there have been several reports of autosomal dominant inheritance. * To date, surveys of iNPH patients have identified a possible affected family member in less than 10% of cases. Intron Joss in the SFMBT1 gene, which has been associated with iNPH, occurs in 10% to 21% of Finnish and Norwegian iNPH patients. Based on these data, one might predict that at least 10% to 20% of INPH patients are genetically susceptible to this disorder. This represents a minimum estimate because it focuses only on SEMBTI and does not consider CFAP43, NMES, or other yet to be discovered ANPH-associated genes. Additional genetic studies of patients with sporadic iNPH are needed. * Defects in cilia-related genes have been associated with congenital hydrocephalus in humans and animals, The finding that genetic variations involving cilia-related genes such as CFAP43, NMES, and SFMBTI are associated with iNPH raises the possibility that NPE may be caused by defects in ventricular ciliated epithelia. Idiopathic normal pressure hydrocephalus (iNPH) is a fReMROIOBIE Fal disorder of aging that is characterized by Nemetiewlomegaly (.._), altapraxia, urinary incontinence, and dementia, Gait dysfunction is the most commonly observed symptom of NPE, occurring in about 00% of cascs, The gait dysfunction of IPH is characterized by decreased step iieight, decreased stride length, decreased cadence, and impaired balance ( Because the symptoms of iNPHI are commonly seen in other neurological disorders such as Alzheimer disease and Parkinson disease," it often remains undiagnosed or is misdiagnosed.’ Unlike these neurodegencrative disorders, however,. the symptoms of shunt-responsive iNPH can be improved by (GSP drainage Indeed, ventricular enlargement, the presence of the characteristic iNPH symptoms, and a response to shunt placement are necessary for establishing a certain diagnosis of shunt-responsive iNPH?* (Figs. 41.3 and 41.4). The lack of knowledge about the etiology of iNPH and the empirical method of diagnosis has led some practitioners to question whether iNPH exists as a distinct disease entity. This skepticism has contributed to the low rates of diagnosis of the disorder. iNPH is a neurological disorder of aging. In a study that examined clinical variables associated with shunt-responsive iNPH, it was observed that only 1 of 418 patients with proven shunt-responsive iNPH was younger than 60 years of age.’ Tt is estimated that approximately 700,000 Americans arc affected by iNPH, with the average ageMe diagnosis being: 75.years The prevalence of iNPH has been estimated to be 1.4% to 2.9% in individuals older than 65 years of age and 5.6% in those older than 75 years of age.” The prevalence of iNPH in nursing hhomes has been estimated to be between 9% and 14%." Although first described in 1965, the etiology and pathophysiology of sporadic iNPH remain a mystery, Radiographically, patients withiNPH arenotedtohaveenlanged Serebial wenticles (sco Tis. 11.10), periventricular white matter abnormalities, alterations in size of the subarachnoid Spaces (i-c., disproportionatcly enlarged subarachnoid spaces IDESHD, and gbnormal CSE) flow through ithe eérebral figueduet on MRI-™” A common measure of ventriculomegaly Figure 41.1. Noncontrast head CT revealing normal ventricular size (A). Noncontrast head CT demonstrating (ERSUSHIBSH in 2 patient with Idiopathic normal pressure hydrocephalus (B). The EBMISINIGAR 's represented by the largest axial distance from the right veririce to the left ventricle vided by the largest right-to-left extent of the cranium (C). The (lOSANBAIB's the angle between the lateral ventricles displayed on a coronal plane through the posterior commissure, perpendicular to the anterior-posterior commissure plane (0). 294 efMagnetic" “Bradlykinetct “Shutting “Short stepped” - Decreased Stop height - Decreased stride length Impaired balance Figure 41.2. Characteristics of gait dysfunction inifopathic normal Pressure hydrovephals. Figure 41.3. Risk factors, sgns, and symptoms of ciopathic normal pressure hydrocephalus. CSF; Cerebrospinal thi. is the Evans index, although this i not specific to &NDHL on MRL Important "maces Although several theories have been propose to explain these phenomena, experimental evidence to support 5 lim sn INPH jons are not known.*+!Dk includes Parkinson disease, Do MRI findings cervical myelopatty vitamin deficiency, ‘suggest ypical Typathyraiiem, ete Neu? ve No ‘High-volume LP Ex vacuo Consider Does clinical orlumer dain hycrecephalis |neuropsychologial | "suspicion al ‘may be more testing for remains Fgh? ‘key nouredegenaratve 1 tisoooee a OP > 250m H0. i igh-voume LP pleocytosis? ‘rlumbar drain Yes, No. i Investigate Ciearcnical Gear cncal causes ot high reeponse 10 response to ‘nvacraniah, CSF removal? SF removal? pressure ves No ve No PH tel INPH tess key, PH Ike, NPH less ikely consider VP iteinica consider VP iteinial shunt suspicions shunt suspicions high, may high, may: consider VP consider VP ‘shunt shunt Figure 41.4, Approach to the dlagnosis and treatront of icfopathic normal pressure hydrocephalus. CSF, Cerebrospinal fuid; LP lumbar puncture; 'NPH, normal pressure hydrocephalus; VP ventricuoperitoneal,hallmarks include chorea, behavioral disturbance, and executive impairment. Symptoms typically begin in the fourth or fifth decade, but there is a wide range, from childhood to >70 years. Memory is frequently not impaired until late in the disease, but attention, judg- ment, awareness, and executive functions are often defi- cient at an early stage. Depression, apathy, social with- drawal, irritability, and intermittent disinhibition are common. Delusions and obsessive-compulsive behavior may occur. Disease duration is typically around 15 years but is quite variable. Norma presmiemhiydiocephalis(NIPED) is a relatively uncommon but treatable syndrome. The clinical, physi- ologic, and neuroimaging characteristics of NPH must be carefully distinguished from those of other demen- tias associated with gait impairment. Historically, many patients treated for NPH have suffered from other dementias, particularly AD, vascular dementia, DLB, and PSP. For NPH, the @imiealeaaainelidestanlabnonmnal gait (ataxic or apractic), dementia (usually mild to mod- erate, with an emphasis on executive impairment), and (innaryarseneysoriincontinence. Neuroimaging reveals enlarged lateral ventricles (hydrocephalus) with little or no cortical atrophy, although the Bylwianlifissiresiimay appear propped) open) (soscalledl"boxcaraing ), which can be mistaken for perisylvian atrophy. This syndrome is a communicating hydrocephalus with a patent aqueduct OASYS (Fig. 29-10), in contrast to aqueductal ste- nosis, in which the aqueduct is small. [GEiDaEpOReHaRS opening pressure falls in the high normal range, and the CSF protein, glucose, and cell counts are normal. NPH may be caused by obstruction to normal CSF flow over the cerebral convexities and delayed resorption into the Wenousisystem. The iA@6IEGE nature of the process results in enlarged lateral ventricles with relatively little increase in CSF pressure. Presumed stretching and distortion of subfrontal white matter tracts may lead to clinical symp- toms, but the precise underlying pathophysiology remains unclear. Some patients provide a [RiS®Sig—ORICORCHEORS that produce meningeal scarring (blocking CSF resorp- tion) such as previous meningitis, subarachnoid hemor- Hiageerneadsiaama Others with long-standing but asymptomatic congenital hydrocephalus may have adult- onset deterioration in gait or memory that is confused with NPH. In contrast to AD, the patient with NPH complains of an S@Hy§andlipromineneleaierdiscurbance without cortical atrophy on CTor MRI. Numerous attempts to improve NPH diagnosis with various special studies and predict the success of ventricu- lar shunting have been undertaken. These tests include radionuclide cisternography (showing a delay in CSF absorption over the convexity) and various efforts to monitor and alter CSF flow dynamics, including a con- stant-pressure infusion test. None has proven to be spe- cific or consistently useful. A GAGSiGHEGmprovementln gait or cognition may follow lumbar puncture (or serial punctures) with removal of 30-50 mL of CSF, but this finding has also not proved to be consistently predictive of postshunt improvement. Perhaps the HHOSEQEGLaBIE strategy is a period of close inpatient evaluation before.Gunna eerlumbayesrydminage. Occasionally, when a patient with AD presents with gait impairment (at times due to comorbid subfrontal vascular injury) and absent or only mild cortical atrophy on CT or MRI, dis- tinguishing NPH from AD can be challenging. Hippo- campal atrophy on MRI favors AD, whereas a character- istic “magnetic” gait with external hip rotation, low foot clearance and short strides, along with prominent truncal SMAPOTANSAbIEEAVORUNPEL The diagnosis of NPH should be avoided when hydrocephalus is not detected on imaging studies, even if the symptoms otherwise fit. Thirty to fifty percent of patients identified by careful diagnosis as having NPH will improve with ventricular shunting. Gait may improve more than cognition, but many reported failures to improve cognitively may have resulted from comorbid AD. Short-lasting improve- ment is common. Patients should be carefully selected for shunting, because subdural hematoma, infection, and shunt failure are known complications and can be a cause for early nursing home placement in an elderly patient with previously mild dementia.NORMAL-PRESSURE HYDROCEPHALUS. In_ meningeal and ependymal diseases, YaROeepHalus fmayidevelopland reachialstablelstate. It is then said to be “compensated” in the sense that formation of CSF equili- brates with absorption. Once equilibrium is attained, the ICP gradually falls, though it still maintains a gradient from ventricle to basal cistern to cerebral subarachnoid space. A stage is reached where the @SEIpressure Reaches & high|normalllevel/ofis0\t0200 mim HO, but the patient nonetheless eventually manifests the cerebral effects of the hydrocephalic state. The name given to this condition by Adams and colleagues and Hakim and Adams was normal- pressure hydrocephalus (NPH). There continues to be controversy regarding the fre- quency of this condition in older adults, and there have even been reasonable arguments questioning the exis- tence of the entity as a coherent disorder or syndrome and, in response to guidelines for treatment issued by the American Academy of Neurology (see Halperin et al) there has certainly been divergence of opinion (Saper). As to the frequency of the disorder, estimates vary widely and depend on the age group studied but Jaraj and colleagues using four cohorts, estimated the prevalence as 0.2 percent of those aged 70 to 79 and 5.9 percent of those over 80. A triad of clinical findings is characteristic of NPH: slowly progressive gait disorder is usually the earliest fea- ture, followed by impairment of mental function and later, sphincter incontinence. The reader should be alerted to the fact that the fully developed triad is not usually pres- ent in the early stages of the process. (SEaSpIReHexeS in the feet and falling attacks may also occut, but there are no Babinski signs. aGaeHES are infrequently a complaint, and there is no papilledema. ‘The gait disturbance that accompanies NPH may be of several different types, as discussed in Chap. 6. These are difficult to classify and only vaguely simulate the patterns observed in Parkinson disease or cerebellar ataxia, but cer- tain features predominate. Most often, there is [InStadia ness and impairment of balance and shortening of thestep length, with the greatest difficulty being encountered on stairs and curbs (Fisher, 1982). Weakness and tiredness of the legs are frequent complaints, although examination, discloses no paresis or ataxia. An impression of Parkinson disease may be conveyed, with short steps and slightly stooped, forward-leaning posture, but the resemblance is superficial because there is Sypieally HO SHURINe) HOES there festination, rigidity, slowness of alternating move- iment, Or tremot Some patients present with unexplained falls, often helplessly backward, but on casual inspection the gait may betray little abnormality except a minimal reduction in step length and overall slowness. When the condition remains untreated, the steps become shorter, with more frequent shuffling and falls; eventually standing and sitting and even turning over in bed become impos- sible. Fisher referred to this advanced state as “hydroce- phalic astasia-abasi ‘The mental changes in the cases we have encountered have been, broadly speaking, ‘fighital’jinljeharaeter and embodied mainly apathy, dullness in thinking and actions, andslightinattention. Memory trouble is eventually a com- ponent of the overall problem and has been predominant in some cases, for which reason the diagnosis of Alzheimer ase is sometimes considered before the discovery of hydrocephalus, but as a rule, the Galt\derangementyot stantially impaired. There is usually a degree of affective indifference, but the patient reports little in the way of emo- tionality. Patients who display gait difficulty with prominent and progressive verbal, graphical, and calculation difficul- ties are more likely to have a degenerative or cerebrovascu- lar disease. In those cases, the difficulty with walking and stability is ostensibly a result of frontal lobe disease, either degenerative or infarctive, as discussed in Chap. 6. Unfor- tunately, beyond the above-noted defects that are elicitable by routine testing, we have not found neuropsychologic tests of great value in the diagnosis of NPH. Urinary symptoms appear relatively later in the illness. Initially, they consist of urinary urgency and frequency. Later, the urgency is associated with incontinence, and ultimately there is fRORtaIIODENREOMEINERES” in which the patientis indifferent to his lapses of continence, and bowel control becomes similarly disordered. ‘The cause of the syndrome of NPH in most cases can- not be established and on weak grounds, BSjEpEOHaHe fibiosing meningitis is often presumed to have been pres- ent. An uncertain proportion of cases can be traced to congenital aqueductal stenosis that has allowed normal brain function into adulthood and, for unknown rea- sons, decompensates; a few of our patients have become symptomatic after mild head trauma. This is probably the most common imputed cause of the syndrome but again, on uncertain grounds. An {@entieallsynidrome may ols low subarachnoid hemorrhage from ruptured aneurysm, resolved acute meningitis or a chronic meningitis (tuber- cular, fungal, syphilitic, or other), Paget disease of the base of the skull, mucopolysaccharidosis of the meninges, and achondroplasta. ‘That the mechanical effect of ventricular enlargement GHUEE|adjaeent brain is responsible for the syndrome is supported by Fisher's observations (2002) that a reduction. in ventricular size caused by extrinsic compression from subdural collections has been associated with clinical improvement. One presumes that the main clinical fea- tures are due to dysfunction of the frontal lobes and their connections with the striatum, from mechanical pressure or distortion, but this is conjecture. Diagnosis of NPH Verification of the diagnosis of NPH, and the selection of patients for ventriculoatrial or ventriculoperitoneal shunt has presented difficulties. The CT scan, as shown in Fig. 29-4, displays enlarged ventricles) without) convolutional ROPY. This disproportionate enlargement of the ventricu- lar system in comparison to the degree of cortical atrophy is judged by the CT and MRI appearance, but there is no broadly agreed upon method for its determination. Vari- ous unwieldy formulas have been designed to assess this ratio. As a group, patients who have a sustained response to drainage of CSF by shunting, as described below, have had the ifStEWO SISRentS One lifleal tid (fewer than half of our successfully treated patients have reached the point of incontinence) fi Heit lateral Ventricular spain atte level ofthe anterior horns has been in excess of approximately 39 fim (a true dimension calculated from CT or MRI scans). MRI may show some degree of transependymal egress of ‘water surrounding the ventricles, but this is not usually the ‘case, and this sign is sometimes difficult to differentiate from the periventricular white matter change that is ubiquitous in the elderly. There may be an indication of inadequate pulsatile flow of CSF through the aqueduct as appreciated on T2-weighted MRI. A [MiinbAE BUREAUS is often performed for diagnostic purposes and the pressure measured carefully but here too, there is no uniformly agreed upon approach. In most cases of NPH, the GSRipreSsutelisiabove 150 mimiIHyO with the patient fully telaxed, but the disorder has occurred, at least as judged by improvement with shunting, with lower pressures, in a few instances as low as 120 mm H,0. Drainage of large amounts of CSF (20 to 30 mL or more) by lumbar puncture often results in plinieal improvement filStaniee|anialgalt for a few days, usually with a delay of hours or a day after the puncture so the patient and fam- ily must be depended upon to report these changes and these reports are prone to excessive optimism. Objective improvement in gait after spinal drainage, measured by reduced time to walk a predetermined distance and fewer steps than prior to drainage, is one way to select patients for shunt operations when the clinical picture is not entirely clear, but the even this test is not infallible. Several small series suggest that a negative test does not preclude benefit from shunting (see Walchenbach et al). However, in these same series, improvement after removal of CSF has had a high predictive value for success of shunting, ‘When there has been doubt as to the effects of lum- bar puncture, one appropriate course is to admit the patient to the hospital, and insert a lumbar drain for up to 3 days, removing approximately 50 mL of CSF daily in order to observe the response in gait and mentation. It is worthwhile to quantify the speed and facility of gait two or three times before the lumbar puncture or drainageand to perform this testing at periodic intervals for sev- eral days after the procedure in order to be certain that improvement is genuine. Even more persuasive is a defi- nite improvement followed days later by worsening of gait. Monitoring of CSF pressure over a day or more may show intermittent rises of pressure, possibly correspond- ing to the A-waves of Lundberg, but this undertaking is not generally practical and is now done by only a few centers. According to Katzman and Hussey, the infusion of normal saline into the lumbar subarachnoid space at a rate of 0.76 mL/min in NPH provokes a rise in pressure to greater than 300 mm H,0 that is not observed in normal individuals. Theoretically, this test or any ofits derivatives, such as the one proposed by Borgesen and Gjertis, should reflect the adequacy of CSF absorption, but they too, have yielded unpredictable results. Radionuclide cisternogta- phy had been used in the past to demonstrate persistence of CSF labeling in the ventricles and, although we still use it occasionally in uncertain cases of NPH, it is no longer considered a compelling test. Treatment of NPH in Adults ‘The development of ventricular shunt tubing with one- way valves opened the way to successful treatment of hydrocephalus. or less often, a vent uloatrial or ventriculopleural shunt is used. The valve can be selected for a desired fixed opening pressure, or a vari- able valve can be inserted and adjustments can be made by an external magnetic device. Gratifying success can be obtained, often a complete or nearly complete restoration of mental function and gait after several weeks or months, by the placement of a shunt. of patients in whom a. ‘As already noted, other predictors of success are considerable enlargement of the ventricles in comparison to the degree of cortical atrophy, CSF pressures above 150 mm H,O, 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 are associated with poorer outcomes after shunting, 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 associa- tion of degenerative dementia and vascular lesions. How- ever, in Fisher's experience, age alone did not exclude NPH as a cause of gait disorder, and long duration of gait symp- toms did 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 inad- visable, it is sometimes possible to produce a reasonable improvement in gait by repeating the drainage of large amounts of fluid every few weeks. It is usually feasible to discontinue anticoagulants for a brief period to accom- modate shunting or lumbar puncture but the small risk of stroke in patients with atrial fibrillation or cardiac valvular diseases must be considered. Between the many reported series of NPH treated with shunting, outcomes vary greatly—almost certainly art dependent on the accuracy of the initial diagnosis and the duration of symptoms. For example, 45 patients reported by Shaw and associates, 69 percent improvedin speed of gait, 63 percent improved at least 2 points in MMSE (mini-mental status examination), and 69 percent improved in UPDRS, a standard overall score of assess- ing Parkinson disease (Chap. 38). While only 38 percent improved on multiple measures, only 15 percent improved on none. These are representative or better than other comparable series. Gauging what happens to incontinence after shunting is difficult and there is little information but we have had patients with advanced disease who reported some improvement, contrary to the usual dictum. Not all neurologists agree to such an optimistic assessment of the benefits of shunting and they also point to the limited durability of effect in case series, such as the one from Kahlon and colleagues with long-term follow-up, and high rate of complications, approximately 10 percent in some series (see editorial by Saper in response to AAN In some {in stances, a lack of improvement, or marked improve- ment followed by subacute deterioration is explained by inadequate decompression, which justifies a revision of the shunt or downward adjustment of a variable pressure These fluid collections, or hygro- ‘mas, consisting of CSF and proteinaceous fluid derived from blood products, are generally innocuous and do not require drain age unless they enlarge or cause focal neuro- logic symptoms or, rarely, seizures. Although shunting is relatively simple as a surgical wocedure, it has complications, the main ones being a FostperavelSuBaURANERIOR (the bridging dural veins stretch and rupture but the procedure has been performed safely in patients who must take anticoagu- ants after shunting); infection of the valve and catheter, sometimes with ventriculitis and occasionally bacteremi occlusion of the tip of the catheter in the ventricle; and, particularly in infants and children, the “slit ventricle syn- drome” (see below). Orthostatic headaches can be over- come by raising the opening pressure of the shunt valve. Misplacement of the catheter may rarely transect tracts in the deep hemispheral white matter and cause serious neurologic deficits, mainly hemiplegia. Itis our impression that this occurs more often when the catheter is inserted from the posterior rather than through the frontal or pari- etal regions. The incidence of catheter blockage is reduced by placing it in the anterior horn of the ventricle (usually the right side is used), where there is no choroid plexus. Meticulous aseptic technique and the preoperative and postoperative administration of antibiotics have appar- ently reduced the incidence of shunt infections but the latter has been difficult to establish, Most shunts in adults are brought to termination in the peritoneum (ventriculo- peritoneal shunt). Perforation of the stomach or bowel is possible. Rare complications of ventriculoatrial shunting are pulmonary hypertension and pulmonary embolism and nephritis, which are caused by low-level infection of the shunt tube with Staphylococcus. Puncture of the floor of the third ventricle by endo- scopic techniques (“third ventriculostomy”) has been explored as an alternative to shunting, especially in chil- dren with congenital aqueductal stenosis. Cinalli and colleagues have suggested, and we concur based on expe- rience with a limited number of our own adult patients, that third ventriculostomy is sometimes an effective treat- ‘ment of shunt failure but Sankey et al had disappointing Jong-term results when the procedure was used as a pri- mary approach. (Once the CSF is shunted, the ventricles may diminish in size within a week or two, even when the hydrocephalus has been present for a year or more. This indicates that hydrocephalic compression of the cerebrum is at least partly 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 improve- ment. Clinical improvement occurs within a few weeks, the gait disturbance being slower to reverse than the mental disorder, Symptoms of cerebral atrophy because of Alzheimer disease and related conditions are not altered by shunting, but this approach to the treatment of degen- erative dementia has been periodically, and unadvisedly, resurrected, as discussed by Silverberg and associates. The use of acetazolamide to reduce CSF volume and pressure has gone through periods of popularity for treat- ing NPH in adults (comments about its use in children are found below) and Alperin et al have shown imaging changes of reduced periventricular white matter changes, presumed to be a reflection of transependymal transgres- sion of water, but clinical effects in our patients have been minimal. A few case series. For example, by Aimard and colleagues have reported benefit Treatment of Infantile and Childhood Hydrocephalus Here one encounters more difficulties than in the treat- ment of the adult disorder. The ventricular catheter may wander or become obstructed and require revision. Peri- toneal pseudocysts may form (most shunts in children are ventriculoperitoneal). Another unexpected complica- tion 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, although we have observed it in adults. ‘These patients develop an intracranial low-pressure syn- drome 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 is much reduced. In babies, the cranium ‘may fail to grow even though the brain is of normal size. In most shunted patients with slit ventricle syndrome, the ICP in the upright position is diminished to 30 mm H,0. ‘To correct the condition, one would imagine that replac- ing the shunt valve with another that opens under a higher pressure or raising the opening pressure of an adjust- able valve would suffice. Indeed, this may be successful. But once the condition is established, the most effective measure has been the placement of an antisiphon device,Ventriculoperitoneal Shunts Indications AVP shunt is the current standard treatment modality for hydro: éephilus The primary indication for VP shunt placement ip Children today is Spee lalng tat R) ad@ amseeable Rope In recent years there has been renewed interest in ET'V (with or without choroid plexus cauterization [CPC)) as a therapy for hydrocephalus." The primary advantage of ETV is that it subverts the needfona perinanentshunt, with its associated long- term complications. The ETV Success Score is a validated tool that may be used to predict who would benefit from ET'V com- pared with CSF shunt insertion based on age, etiology of hydro- cephalus, and presence of a previous shunt."*'-!8* ETV may be a preferable option in low-resource settings, where surgeons may not be immediately available to revise a failed shunt, A recent ran- domized trial of 100 Ugandan infants with postinfectious hydro- cephalus demonstrated no significant difference between ETV with CPC and VP shunting with regard to cognitive outcomes at 12 months.'”! However, data from North America have sug- gested that ETV with CPC may have a higher failure rate than shunt." Specific Complications With regard to perioperative complications, placement of the distal catheter of a VP shunt is more difficult in the context of previous abdominal surgery (including multiple shunt revisions), obesity, or prior intra-abdominal infection. These factors may increase the likelihood of inadvertently placing the catheter in the preperitoneal fat rather than within the peritoneal space. Vascular or visceral injury, fortunately, is a far rarer complication in the intraoperative setting."°° However, over time the distal catheter may erode through virtually any of the abdominal viscera into one of many different locations. Reports have been published of the peritoneal catheter migrating into the gallbladder,"”! oral cavity, P21) anus, !495 serotam,!+0 umbilicus,!94097.08 heart, 199-20) thorax,29*206 pulmonary artery,!"2220” and bladder 205209 among other sites. bclomindl CSISPSEIMOS)SEOMEAEOH is known complication of VP shunts, with a reported incidence of 1% to 4.5%.?!"?!! The etiology of CSF pseudocyst is unclear; however, the prevailing hypothesis is that an inflammatory process, either infectious or sterile(c.g., tissue reaction tosomecomponentoftheshuntsystem), results in formation of the pseudocyst wall, consisting of thickened peritoneal serous membrane.”!? Many factors predisposing to abdominal pseudocyst formation have been identified, the most important one being infection.’!??!* Other risk factors include previous abdominal surgery (including multiple shunt revisions)and allergic reaction to silicone.’!*!> Patients most often present with headache, vomiting, and decreased level of consciousness although pseudocyst may also manifest as an acute abdomina condition.’!!!21° ‘The diagnosis is confirmed by abdomina ultrasound examination and/or CT.2!7=!® A common treatment approach involves antibiotic therapy and shunt externalizatior for underlying infection, followed by exploratory laparotomy 01 laparoscopy with pseudocyst excision/aspiration and VP shun replacement.?!?2)) Rarer abdominal complications include volvulus, CSF ascites, inguinal hernia, and hydrocele.2!9-221