1.4 Hydrocephalus in Childhood - A Study of 440 Cases. w.j. Peacock and t.h. Currer

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  • 8/13/2019 1.4 Hydrocephalus in Childhood - A Study of 440 Cases. w.j. Peacock and t.h. Currer

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    SA MEDICAL JOURNAL VOLUME 66 1 SEPTEMBER 984 323

    Hydrocephalus in hil hooA stu y of casesw J. PEACOCK T CURRERumm ryReview of a series of 440 children treated for hydrocephalus shows that the commonest cause of thiscondition in CapeTown is meningitis both bacterialand tuberculous. followed by congenital anomaliesand brain tumours. Ventriculoperitoneal shunts wereused to treat the majority of cases while lumboperitoneal shunts were reserved for patients with communicating hydrocephalus. Ventriculo-atrial shuntswere only used twice. Blockages affected 20 ofcasesbutusuallyoccurred within 3monthsofsurgery.

    Th e term hydrocephalus was for centuries associated withchildren who had large heads an d were mentally retarded. Sincethe advent of modern shunting devices in the 1950s th e outlookhas changed completely an d now, because of recent refinementsin technique and eq uip men t, th e majority of treated hydrocephalic children should have normal-sized heads an d average ornear-average intelligence.1-3 In this study 440 cases of hydrocephalus are reviewed.

    atients and methodsTh e Paediatric Section of t he Depar tment of Neurosurgery atth e University of Cape Town treated 440 children with hydrocephalus during th e period 1979-1982. There were 387 88 )new patients and 53 12 ) patients who had previously beentreated either at this department or elsewhere and required shuntrevision because o f complications. A shunt procedure wasperformed on 432 children. Sixof th e 8 patients who di d not havea shuI1t ha d temporaryventricular drains inserted. Tw o patientsdid not undergosurgicaltreatment; in onecase th e hydrocephalusd id n ot progress while in th e other resolution followed percutaneous ventriculography. Ventricular drains inserted beforeperformanceof definitive surgery were used early in th e series in4 pat ient s with brain t um ou rs w ho di d not ult imately needcerebrospinal fluid CSF) diversion. Tw o patients with myelomeningoceles required ventricular drainage for the treatment ofventriculitis, bu t no shunts.

    In cases 25 ) the primary lesion couldno t be determinedfrom th e history. Among th e remaining 329 75 ) there were 158patients 48 ) with hydrocephalus following meningitis; in 93cases th e cause was bacterial and in 65 tuberculous. Congenitalhydrocephalus was diagnosed in 105 cases 32 ) because it waspresent at or shortly after birth; this included 65 children with

    D e p ar tm e n t o f Neurosurgery, Red Cross W ar MemorialChi l dr en s Hospit al an d University o f C a pe T o w nW. PEACOCK, F.R.C.S.T H. CURRER, M.B. CHB.

    myelomeningocele. A tumour in t he f ou rt h ventricle, pinealregion or third ventricle was th e cause of CS F pathwayobstruction in 36 cases 11 ). Intraventricular haemorrhage inbabies with a low birth weight resulting in post-haemorrhagichydrocephalus accounted for 12 cases; sagittal sinus thrombosisinterfered with CS F absorption in 4 cases, the thrombosis beingcaused bymiddle earinfection in 2, severe dehydration associatedwith gastro-enteri tis in I , an d blunt trauma in 1; 8 cases ofhydrocephalus followed trau ma, p resumably as a result ofsubarachnoid haemorrhage, an d 6 were d ue t o intracranial cystsobstructing CS F flow. Of the p.ew patients treated 70 wereunder 1 year of age, an d only 10 more than 5 years old . Th einvestigations used to establish th e diagnosis of hydrocephaluswere computed tomography CT), percutaneous ventriculography an d real-time ultrasonography.Ventriculoperitoneal VP) shunts were inserted into 308 newpatients, an d of these 63 20,45 ) required revision. Of th erevisions 50 were carried ou t within th e 1st month an d 76within 3 months of th e initial shunt. Th e commonest shuntcomplications were blockage, infection an d skin ulceration, th eusual site of blockage being at the ti p of the ventricular catheter

    that had been incorrectly positioned in th e su rto un d in g b rain o rchoroid plexus. Other complications 9) were subdural effusion3), extradural haemorrhage I) , epilepsy 2) a nd m in or CS Fleaks 2) with ventricular collapse th e slit-ventricle syndrome)in I patient. Th e operative mortality was nil. Lumboperitoneal LP) sh un tswere inserted into 69 patients, 13 18,8 ) of themblocking and req uirin g conversion to a VP shunt. Ventriculoatrial VA) shunts used in 2 new pat ients; 8 patients whohad had VA shunts inserted elsewhere presented because ofcomplications such as blockage or pulmonary hypertension an dthe shunts were converted to VP shunts.

    iscussionCS F is produced mainly b y t he c ho ro id plexus of th e lateralventricles at a rate of about 10-20ml/h an d secretion is relativelyunaffected by age or changes in intracrania l pressure. Afterflowing through th e ventricular system an d subarachnoid spaceCS F is absorbed via th e arachnoidvilli in to th e sup erior sagittalsinus. Hydrocephalus is a condition in which there is animbalance betweenCS F production an d absorptionwith resultantraised intracranial pressure. In children under t he age of about 2years th e raised intracranial pressure splays the unfused sutures,enlarging th e v au lt an d bulging the anterior fontanelle. In olderchildrenwithclosed sutures headache, vomiting an d papilloedemaare th e usual presenting clinical features.

    Th e cause could not be established in about 25 of th e cases inthis series because of a n i na de qu at e history, bu t neonatalmeningitis, congenital obstructions an d intraventricular haemor-rhage probably accounted for most of these. Meningitis wasresponsible in nearly half of those patients in whom a cause wasdiscovered, neonatal meningitis an d tuberculous meningitisbeing th e tw o main types. Since t he advent of CT an dultrasonography the clinical deterioration so frequently seen inpatients with tuberculous meningitis ClID often b e s ho wn to bedu e to the development of hydrocephalus; insertion of a shuntdramatically improves th e outcome. 4 Congenital hydrocephalus

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    4 SA MEDIESE TYDSKRIF DEEL66 1 SEPTEMBER 1984

    accounted for 105 cases and was diagnosed at or shortly afterbirth because of th e presence or development of a large head; onoccasions itwasdetected antenatally by ultrasonographythroughth e maternal abdominal wall. In this group there were 65children with myelomeningocele, of whom 90 developedprogressive hydrocephalus requiring th e insertion of a VP shunt.Th e children found to have bra in tumours almost invariablypresented with th e features of raised intracranial pressurebecause in this age group these tumours tend to expand into theventricular system and obstruct CS F flow. Except for patientsseen early in the series, all chi ldren with tumours received ashunt as an emergency procedure a nd t he t um o ur was removedabout 10 days later . Th e insertion of a shunt to normalizeintracranial pressure has been an important factor responsiblefor the improved results in th e tre a tme nt of paediatric braintumours. In Hoffman er al. s series I of chi ldren treated forhydrocephalus 29, I had myelodysplasia while other congenitalanomalies accounted for 14,1 of cases. A further 18,8 had atumour as a cause for their hydrocephalus, 10,6 had a posttraumatic hydrocephalus an d only 5,7 had post-infectioushydrocephalus. Ou r incidence. of 48 for postmeningitic hydrocephalus is approximately tenfold that reported by Hoffman eral.; this is du e to th e great frequency of neonatal an d tuberculousmeningitis in our population.Intraventricular haemorrhage is a common problem in lowbirth-weight babies under 1500 g) an d many that survive willdevelop hydrocephalus. It has been ou r policy to introduce ashunt in these babies despite heavy bloodstaining of theventricular CSF; shunt blockage has no t been a major problem.Long-term follow-up has shown good resul ts in about 60 ofcases of post-haemorrhagic hydrocephalus.;Saginal sinus thrombosis reducesCSF absorption and is a rarecomplication of conditions such as chronicear infections, gastroenteritis with severe dehydration an d trauma involving thesaginal or transverse sinuses. In most cases spontaneous resolution occurs but in some troublesome headache or failing visionrequires treatment with a LP s hu n t. We found that in all 4 casessymptoms disappeared following shunting, and where long-termsteroid therapy had been used it could be withdrawn.

    This group of patients was investigated in order to confIrm thepresence of large ventricles an d to exclude other lesions such assubdural effusion or tumour. LP shunts were used if ventriculography h a d d emo ns tr ated a communicating form of hydrocephalus. LP shunts have certain advantages over VP s hu n ts asthey can be inserted percutaneously, are inexpensive, do nottransgress th e cerebra l cortex and allow more normal CS Fdynamics to be maintained. Th e complication rate for LP shunts

    was about the sameas that for VP shunts in this series. In cases ofnon-communicating hydrocephalus VP shunts were used andthe alternative method, the VA shunt, was only chosen forpat ients in whom th e peritoneal cavity ha d been distorted byprevious peritonitis or because major abdominal surgery wasplanned.Ou r shunt blockage rate compares favourably with that

    reported in other series. 1.6 Approximately 20 of th e VP an d LPshunts became blocked bu t three-quarters of these blockagesoccurred during thefIrst 3 months after surgery. Thus, if a childhad been trouble-free for 3 postoperative months, there was onlya 5 chance of a shunt complication. ,Hydrocephalic children who have received a shunt have beenshown to have m uc h h ig he r intelligence than was geperallybelieved.1.3 Hoffman er l I assessed the intelligence qU otients(IQs) of 889 hydrocephalic children with shunts an d found that60 ha d normal or above-normal IQs a nd t ha t 10 had mildlysubnormal IQs. Mild-to-severe retardation was present in 30 .In ou r series we have no t been able to c ar ry o u t psychometricanalyses on all the children bu t have been pleased with the largenumber shown to have normal intelligence. Poor results wereassociated with a delay in shunt insertion and with shuntinfection.

    ConclusionsIn Cape Town, the commonest causesofchildhood hydrocephalusare meningitis, congenital anomalies an d bra in tumours. VPshunts were the most frequently used form of treatment,followed by LP shunts which were reserved for cases ofcommunicating hydrocephalus. Blockages with both types ofshunt affected only 20 of patients, usually occurring within 3months of surgery, a nd s hu nt infection was uncommon.REFERENCES1. Hoffman HJ, Hendrick EB, Humphreys RP. Managemenr of hydrocephalus.Monogr Neural Sci 1982; 8: 21.2. Pudenz RH. Th e surgical treatment of hydrocephalus - an historical review.

    Surg Neuro11981; 15: IS.3. Raimondi AJ, Soare P. Intellectual development in shunted hydrocephalicchildren. Am] Dis Child 1974; 127: 664.4. Bullock MRR, Van Dellen JR. Th e role of cerebrospinal fluid shunring intuberculous meningitis. Surg Neurol 1982; 18: 274.5. Bada HS , Salmon JH , Pearson DH . Early surgical intervention in posthemorrhagic hydrocephalus. Child Brain 1979; 5: 109.6. De Vi lli ers JC, Cluver PFD, Handler L Complications following shumoperationsforpost meningitichydrocephalus. In: WullenweberR, WenkerH,Brock 1\1 Klinger eds. Adl. Qllces in l\leurosllrgery \ 01. 6. Heidelberg:Springer Verlag, 197 : 23-27.