Review JK - PRACT rIONER MANAGEMENT OF HYDROCEPHALUS IN TUBERCULAR MENINGITIS Gautam Das,M.B..B.S., Basharat Hameed, M.B..B.S., M. Asim Siddiqui M.D., Jamal Ahmad.M.D., D.M.. Ph.D ‘The first clinical description of exudate that infiltrates the tubercular meningitis in the late 18th cortiomeningeal blood vessels and century is credited to the Scottish causes inflammation and obstruction of Physiologist Sir Robert Whytt!, even vessels around the interpeduncular and before Robert Koch isolated pontine cisterns in acute stages and mycobacterium tuberculosis in 1882°. adhesive leptomeningitis in the chronic Tuberculosis of the central nervous stages’. This is reported to be more system is the most dreaded complication common in the paediatric age group as of tuberculosis and the incidence is ever several case series have reported a 100% increasing in developing countries and occurence in children4 and is usually in developed ones. related to the duration of sy Neurotuberculosis is HYDROCEPHALUSINTUBERCULAR, encountered in upto 10% of the immuno MENINGITIS competent patients’ and emergence of Hydrocephalus can be defined as an HIV (Human immuno deficiency virus) abnormal accumulation of cerebrospinal epidemic and multidrug resistant fluid (CSF) within the ventricles and/or (MDR)TB has further compounded the subarachnoid spaces. Exact incidence in problem as around 5-9% of AIDS patients adults patients with TBM is not known have pulmonary or extrapulmonary but itis nearly always present in all cases, tuberculosis'. It is fatal without effective who had the illness for more than 4-6 treatment and is associated with a weeks!" Authors’ affiliations: significant morbidity rate even after HYDROCEPHALUS IN TBM IS. Gautam Das,Basharat Hameed, treatment. M. Asim Siddiqui, Prof. Jamal CNS tuberculosis may take Ahmad several forms including tubercular meningitis (TBM), meningoencephalitis, Department of Endocrinology JN tuberculomas, tubercular abscess, Medical College. AMU Aligarh, @tachnoiditis and rarely myelo- ndia radioculopathy. Of these TBM is the commonest accounting for upto 70-80% of the cases of neurotuberculosis and 7- 12% of all patients in developing countries*. Damaging effects to the brain in TBM occurs due to formation of granulomatous basal exudates and periarteritis of the blood vessels supplying the brain, Blindness, deafness and cranial nerve palsies (3rd, 4th, 6th and Professor of Endocrinology & 7th) may be encountered in 25% cases* Choirman, Department of whereas motor deficits is noted in 40% Medicine, J.N.Medical College, andseizure in 10-15% casest ccepted for publication: March, 2004 Conespondence to: Jamal Ahmad A.MU., Aligarh Tubercular meningitis usually Tel. & Fax: +91-571-2721173 results from the haematogenous spread ‘mail jamalahmad 1@reciifmati com of bacteria to the CNS.from a primary focus elsewhere in the body usually the lungs. A caseous lesion is formed in the cerebral cortex or meninges which JK-Practi ruptures to discharge the tubercle bacilli ner 2004:11(3):161- USUALLY a Obstructive or non-obstructive a Communicating or non- communicating a Acute or chronic a Active or arrested Excessive accumulation of gelatinous exudate at the bases of the brain interferes with the normal flow of cerebro-spinal fluid (CSP) in TBM leads to a obstructive hydrocephalus (more common in TBM) of either a communicating or non- communicating type COMMUNICATING In this type the blockage is outside the ventricular system ie. within the subarachnoid space at the base of the brain or over the cerebral convexity and is not apparent on routine imaging" Gelatinous and nodular exudates comprising of fibrin and inflammatory cells ‘occur in subarachaoid space and cisterns at the base of brain" and in the region of optic chaisma and the interpeduncular and prepontine cisterns" Encovering of the ependyma and choroid 165 into the subarachnoid space leading to plexus with exudate and scierosis of the formation of a thick gelatinous arachnoid villi and granulations over Key words: Meningins, Tuberculosis, Hydrocephalus 161 Vol. 11, No. 3, July-September 2004cerebral convexity with overlying adhesion of leptomeninges impedes the CSF outflow and leads to hydrocephalus! 5 NON-COMMUNICATING In this type the blockage is within the ventricular system itself Le. at the level of the aqueduct of Sylvius or the fourth .dymitis"", Exudate also block the foramen of Magendie and Lushka at the fourth ventricular outlet SIGNS ANDSYMPTOMS, The clinical presentation of TBM is quite variable as it generally progresses insidiously but may be rapid particularly in infants and young children. The progress of symptoms occurs over several weeks but not in a smooth continuum. Alteration in consciousness (79%), fever (66%), focal neurologic signs (66%) and seizures (53%) are a common presentation’. TBM complicated with hydrocephalus is very low in grade I, low in grade fl and maximum in grade IL and IVI, Common symptoms of hydrocephalus include headache, vomiting, drowsiness, lethargy, irritability, behavioural abnormalities. visual blurring and diplopia whereas signs include tense fomtanelle's, distended scalp veins, frontal bossing, macrocephaly and sunsetting sign in infants and papilloe 1 o bilateral lateral recuts palsies, hyperreflexia, clonus and gait ataxia in older children and adults". Bradycardia. slow respiration, hypertension, decetebrate posture, coma and transient and permanent blindness may occur due to severrise in ICP" DIAGNOSIS The diagnosis of tuberculous meningitis may be difficult because of its non-specific and variable presentation. The tuberculin skin test is positive in 30%-50% patients and a pulmonary focus on chest radiograph is detected in 50-87% cases'”"*, Examination of the CSF and identification or isolation of mycobacteria on smear or culture is the gold standaed for diagnosis of TBM, some series have reported isolation rates as high as 80% after multiple sample processing". in general the yield is much lower'®™. Radiometric culture (BACTEC), CSF PCR, ADA levels, ELISA and other antigen and antibody detection tests have been reported to be usefial with varying degrees of sensitivity and specificity Diagnosis of hydrocephalus in TBM is often difficult as the signs and symptoms are non-specific, hence a high index of suspicion is important for its diagnosis. Before the advent of CT scan in 1976, skull radiograph. cranial ultrasonography, ventriculography and angiography were used for the diagnosis'* of limited application today Neuroimag invaluable in the diagnosis of hydrocephalus complicating TBM. Completed tomognaply (CT) or magnetic resonance imaging (MRI) of the brain may be normal during early stages but shows thick basilar enhancement, parenchymal enhancement, hydrocephalus, cerebral edema, focal ischaemia and infarcts ata later stage CT scan of head (plain and contrast) will demonstrate*'* ventricle and associated eper lema. unilat which are a Hydrocephalus induced ventricular enlargement (communicating or non-communicating). A Obliteration of the sulci and subarachnoid spaces UK - PRACTITIONER over the cerebral convexity due to raised ICP. fa Periventricular lucencies (PVL) due to transependymal absorption of CSF into the white matter'® a Intense of basal cisterns (interpeduncular, ambient, prepontine and the chiasmatic region) due to the presence of exudates. Enhancement may also extend over the cerebral or cerebellar hemisphe! enhancement Meningeal enhancement is more in HIV infected patients (23% in HIV vs 6% in non-HIV cases)* Q ——Tuberculomas/Tubercular abscess Q _ Infarction secondary to vasculitis (most common in the middle cerebral artery territory). MRI Gadolinium (Gd-DTPA) is sensitive for demonstrating meningeal enhancement and associated infarcts, granuloma and hydrocephalus’ and MRI is more valuable as a single test because it also shows ventricular volume and CSF flow through the foramen of Monro or aqueduct of Sylvius besides the source of hydrocephalus" Management Acute hydrocephalus which evolves over a course of days or weeks is a neurological emergency which has to be intervened upon as early as possible whereas chronic hydrocephalus is usually presented over months or years and it gets invariably arrested to a non-progressive hydrocephalus in which compensatory mechanisms comes into force and the ICP (intracranial pressure) is normal or mildly elevated withouta shunt 15. The goal of therapy remains to reduce the ICP and to decrease the size of the ventricle so as to improve the periventricular perfusion, MEDICAL THERAPY Anti-tubercular drugs are the mainstay in managing such patients supplemented with steroids, frusemide, acetazolamide and repeated lumbar puncture. All regimens include isoniazid (H) and rifampicin (R) and a third drug used is pyrizinamide (Z) and at times Exhambutol (E) or Steptomycin (S). Recommendations by expert committees suggested that in managing CNS tuberculosis, initially four drugs are preferred and a longer duration of continuation phase is advocated. The World Health Organization suggest a total of 9 month treatment (2+7 months) whereas the American thoracic Society® recommends, 410 month continuation phase with two drugs. The Indian Academy of Paediatrics** endorses the WHO recommendation regarding the initial therapy but includes 3 drugs for 10 months, inthe continuation phase. However till date there are no studies to support the superiority ofa three drug regimen over a two- drug regimen during the continuation phase. Drug resistance has been reported worldwide and managed with second line drugs" and the duration of treatment extends to atleast 18-24 months Steroids have been shown to reduce the neurologic sequelae especially when administered early in the course of the disease”. Steroids decrease brain edema and the production of'inflammatory cytokines and exudates, and minimize damage to the blood brain barrier. Girsis NI et al” showed that the use of dexamethasone routinely as an adjunctive treatment for Vol. L1,No. 3, July-September 2004 162TBM reduces fatality rate (43% vs 59%). Moreover they also showed that the neurologic complications after initiation of therapy (4 vs 10) and permanent sequelae were also lower in the dexamethasone treatment group. Similarly Schoeman DF et al’ showed that corticosteroid significantly improves survival rate and intellectual outcome in TBM as they cause an enhanced resolution of basal exudates and tuberculomas. Butthey failed to showa dramatic effect of steroids on the ICP due to hydrocephalus". Dexamethasone in a dose of 0.2 mg/Kg/day in 3-4 divided doses via parenteral route or prednisolone in a dose of Img/ Ky/day is given for 4-6 weeks and then gradually tapered over a equal period of time" Diuretics such as frusemide may be given in a dose upto Iing/Kg/ at 6-8 hour interval by the oral, IM or IV route with an average adult dose of 40 mg at 8 hour interval. Injection mannitol may be used in life threatening situations or acritical rise in ICP. Acetazolamide in a dose of 100 mg/Kg/day in 3 divided doses in often required for therapy with an average adult dose of 250 mg, twice or thricea day. Continued therapy with frusemide and acetazolamide may decrease production for few days but do not cause ventriculomegaly to resolve instead causes a rebound increase in ICP". SURGICAL THERAPY Hydrocephalus being a common complication of TBM it is suggested that once ventriculomegaly sets in, surgical drainage is the only effective way of reducing raised intracranial pressure!’ SURGICAL OPTIONS Q Ventricular tap Q Ventricular shunt Q _Endoscopie third ventriculostomy VENTRICULAR TAP This procedure is indicated when a shunt cannot be placed immediately due to poor general condition of patient, lack of operative facilities and concurrent pyogenic infection. However, if offers advantages like a Helps in assessing CSF pressure and defining the role oF indication of surgery. a Provides CSF sample for diagnostic study. a Fastest in reducing intracranial pressure, but it improves the clinical condition only for 6-8 hours hence, repeated tapping is therefore needed till the patient is favourable for shunt surgery. Complications like infection, intraventricular bleeding, bleeding along the track, pneumocephalus. poren cephalic cysts along line of ventricular puneture and localized brain damage may occur. Endoscopic third Ventriculostomy (ETV) A percutaneous or stereotactic ETV creates an opening in the floor of the third ventricle to alleviate hydrocephalus. It can be used in cases where the CSF is obstructed at the level of the aqueduct or distal to it'’. A success rate of 40-60% has been reported in the setting of post tubercular hydrocephalus. SHUNTSURGERY Shunt surgery is often needed in patients with TBM who develop hydrocephalus. While some workers have managed JK ~ PRACTITIONER early hydrocephalus with anti-tubercular therapy (ATT) without surgery, others advocate shunt placements in all patients with hydrocephalus” ‘TIMING FORSHUNTSURGERY Kemaloglu S et al” showed that early surgical procedure for mild/moderate hydrocephalus has a positive effect on the morbidity and mortality of hydrocephalus in childhood TBM. Moreover, in severe hydrocephalus, early shunting have a positive effect on morbidity. However, in several patients diversion of ventricular CSF doesn’t improve their clinical condition and may die despite the intervention’. Some researchers have proposed selection of patients on the basis of staging of tubercular meningitis. As hydrocephalus rarely develops in stage I and II TBM indication for shunt surgery is low here'® whereas a shunt is mandatory for all grade I or IV patients with attendant hydrocephalus”. From a prognostic point of view altered sensorium (grade HII) is a better stage to put the shunt rather than waiting for decerebration to occur (grade 1V)!" CRITERIA FORSHUNT SURGERY 1 Clinical" Symptomatic hydrocephalus with one ormore ofthe following, signs and symptoms: Persistent headache and vomiting Papilloedemaloptic atrophy Decreased level of consciousness Gait ataxia or progressive neurological deficit Decerebration / Decortication ‘Neuro-imaging"” CT/MRUevidence of active hydrocephalus i.e. ventriculomegaly with periventricular lucency (PVL) or edema. 3 CSF Criteria! oxooogg, Q —_Celleount- CSF polymorph count should be less than Siem3 irrespective of the number of lymphocytes. GA protein content of fess than 100 mg/dl is no longer required or considered mandatory for shunt surgery 4 Ventricular tap eriteria Q Evidence of raised ICP and a significant fallin the ICP along with clinical improvement following the ventricular tap, is a definitive indication for performing a surgery. METHODS AND RESULTS CSF shunts divert CSF from the ventricles into the body cavities. Of the various shunt options available a ventriculo peritoneal shunt (VP) is the most accepted procedure for hydrocephalus. Venticulo-atrial shunts (VA) have a higher ‘complication rate and therefore not preferred'™"*, Choice of shunt is based on cost, availability and intraventricular pressure. Putting up a low pressure device in high ventricular pressure hydrocephalus would result in over drainage syndrome. Similarly a high pressure device will not drain hydrocephalus with low or moderate intraventricular pressure'®, Connection is maintained between a burr hole created behind the ear where the tip is placed in the frontal hor of the right lateral ventricle and the peritoneal connection is often placed in the epigastrium or in the tight upper or Iower quadrant after being tunneled subcutaneously across the chestwall” 163 Vol. 11, No. 3, July-September 2004Alleviation of increased ICP after shunt may result in improvement in sensorium within 48-72 hours. A postoperative CT isadvised in all cases ideally by 1 week after shunt surgery or earlier ifthere is clinical deterioration or no improvement. The CSF parameters may take months to normalize”™™. The CSF glucose returns to normal within 2 months in $0% of the patients and within 6 months in the majority. The CSF cellular response tales almost 6 months to normalize in approximately 25% cases. whereas CSF protein remains elevated for more than 6 months in 40% cases. COMPLICATIONS The overall complication rate of shunt surgery is about 20%". Common complication includes shunt obstruetion and infection, Others include hematoma, hemiparesis, seizures, shunt migration/disconnection’ fracture, Peritonitis, Peritubal leakage/shunt excoriation, overdrainage, endocarditis, nephritis". Shunt malfunction may develop within 7 days due to shunt misplacement or shunt blockage by blood clots and fibrin debris, Patient have symptoms like headache, vomiting, drowsiness which may appear, disappear and recur again with increasing frequency and severity"’. Patency of the shunt is confirmed by clinical method, transcranial Doppler (TCD) study and CT and MRI imaging. Delayed onset malfunctioning occurs afier 7 days'* due to infection, shunt migration or plugging of the catheter by choroid plexus or the peritoneal catheter by omental adhesions. 5 to 10% of shunts are complicated by infection’ caused by S. epidermidis (60%) and S. aureus (30%) cases. The remainder is caused by coliform bacteria, streptococci or Hemophilus influenza" OUTCOME Although morbidity rates reported may be as high as 88%- 100% despite shunt surgery" but still with effective procedure ‘neurologic improvement following ventricular decompression may be dramatic particularly in children with improvement in the level of consciousness and other signs and symptoms". Daljit Singh et all 1 showed that only 60.3% of patients with grade II and 34.5% of patients with grade IV showed improvement following ventriculo-peritoneal shunt surgery whereas the improvement rate was 100% for group 1 and IT ‘TBM patients! |. Palur R et ab found that the mortality rates in grade Ill ancl IV were $1.9%and 100% respectively, despite policy of early shunt surgery ofall patients. John M Mathew etal" suggested a tial for an external ventricular drainage as, a preliminary procedure for grade IV patients who has otherwise a poor prognosis with shunt surgery. They suggested that all patients of grade 111 should be given the benefits of shunt surgery without atrial of external ventricular drainage but poor outcome for grade IV patients (88% ‘mortality) underlines a need for them to undergo a trial with external ventricular drainage and response to the drainage of CSF to determine the necessity or otherwise of shunt surgery. BIBLIOGRAPHY 1. Whytt R. Observation on the nature causes and cure of those disorders which are commonly called nervous hypochondriae or hysterie. 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