Fallberichte - Case reports 156
Unique complications of cerebrospinal fluid shunts in children - AReport of Two Cases
Sum mary
Th e authors rep ort on two cas es with un usual CSF shunt complications. Th e firs t cas e had a peri toneal ca theter whi ch migra ted dow n a patent proces sus vaginalis into a hydrocoele. Th c second ease ha d a n a tr ial ca the ter wh ich perforated th e a trial wall a nd came to lie in the per icardium ca using an effusion. Clinicians sho uld be a wa re of th e fre quent and aceasiona lly biza rre complications of CSF shunting. Un gewöhnliche Komplikation en bei liquorshunt bei Kind ern Zwe i Fallberichle
Es wird üb er zwei Fälle un gewöhn licher Kom plikationen bei Liquorshunt berichtet. Im ersten Fall wanderte ein Peritonealkat heter in eine Hydrozele durch einen durchgä ngigen Processus vagina lis. Im zweiten Fall perfori erte ein Vorholkatheter die Vor hofwan d und gela ngte in das Perika rdium. wo sich ein Er guß bildete. Klinike r so llten a uf die Möglichkeit häufiger und gelegentlich bizarrer Komplikationen bei lJq uorshu nts achte n. Key wo rds Ch ildhood hydro cephalus - Venlriculoperitoneal sh unt - Ventriculoat rial shunt - CompUcation s Abdomin al pseud ocyst - Hydr ocoele - Vascula r perforation
Introducti on Ch ildhood hydrocephalus is a common disord er which is usua lly tr eat ed by cere brospina l fluid (CSF) diversion using a ven trieuloperiton eal (VP) or a ve ntric uloatrial (VA) sh unts. Th e com plications of these devices a re varied an d largely weil docum ented (9. 17. 20. 21, 32). While neurosurgeon s are a ware of the eommon complica tions, they a re still surprised to see the oecas iona l rare a nd biza rre com plieations. The re is a n increasi ng nu mber of hydrocephalic children with CSF shunts in the communily.
Neurochirurgia 35 (1992 ) 156 - 159 © Georg Thieme Verlag. Stuttgart . New York
a nd these patients may develop symp toms and signs relat ed to the a bdomen or ehes t and may present to other clinicia ns with a pro blem that may initially seem unrelat ed to the shunt (1 5). We describ e two case s with unu sual shu nt cornplications. Th e first case had a per itoneal catheter wh ich pr olaps ed into a hydrocoele a nd the seco nd cas e had an atrial catheter which per forated the right a triu m a nd came to He in the pericardium causing an effusion . Case llist ori es Case 1: A 14-month old ma le child with congenital hydr oceph aJus had a right-sided VP shun t 3 wee ks afte r birth . A year later he pr esented with a 3-day history of head ache. vomit ing and drowsiness. In additic n. the patien t ha d a swelling at th e site of the reservoir exte nd ing down to the n eck. Pla in ab dominal radi ograp h showed the per itonea l cathe ter had disconne cted and migr ated dow n to th e abdomen. A n ew peri ton ea l cath eter was con necte d to the existing functioni ng ven tricu lar catheter a nd reservolr, At ope ra tion there wa s some difficu lty remo ving the old periton eal ea the te r via the sma ll right s ubccstal incision so the tube was left behind . Th e pat ien t made a good post-operative re covery. He was noted to have s ma ll bila teral hydroeoeles which were ma naged conse rva tively in the first instance . 1Wo months lat er; the patient pr es ent ed with an enla rging right-s ided scrotal swelllng. The s hunt tubing wa s elinically palpable in the hydroeoele . Plain abdominal radi ograph (Figure 11showed th at the old and the n ew perit oneal cathe ters had prolapsed into the right hydroeoele. Via a righ t gro in incision the old catheter was removed. the new funetio ning catheter was rep laeed in the peritoneum an d the proeessus vagina lis was cIosed . The left side was also repaired . The patient mad e a good recovery. Case 2: A fs-yea r-old girl with conge nita l hydro ceph alus ha d a right -sid ed VA shunt soo n a fter birth . A year later the shunt beeam e obstr ucted and a new left VP s hunt was inserted . Over the next four years the pati en t had live revision s of the per iton ea l cathe ter elsewhere . A yea r ago the patien t was admitted to King Khalid Un iversity Hospita l with a swelling uver the abdom ina l sea r. The patien t was oth erwise cIinica lly weil a nd the CT sean s howed norma l s ized ventricles . At explorano n. the peri to nea l cathet er was foun d to be d rai ning CSF satisfactori ly. The cathe te r was retn serted into a fresh a rea of the a bdome n.
Four months lat er the patient wa s readmltted with a large abdo mina l swe lling an d vomiti ng. Abdomi nal ultra sound (Fig. 2 ) sh owed a larg e int ra peritoneal eyst conta ining fluid proved to be CSF on per cuta neous aspiration . At exploration ther e were multiple a dhes ions a ll over the perito neal cavi ty. Th e left-sid ed s hu nt system was therefore cha nged to a VA sh un t by con necting the old functionin g rese rvoir to a n atria l cathe te r w hich was inserted via the left intern al jugular vein into the right atrium . The patient recovered we il.
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A. Jamjoom ' , N. Ur-Rahman J. Z. A . Jamj oom J. A. Jauiad ", F. Fadl ey 3 1 Divi sion of Neu rosurgery. Z Paediatric Surgery and 3 Paed iatr ic King Khalid Unive rslty Hospi tal. Hiya dh. Sa udi Ara bia
Neurochiru rgia 35 (199 2)
Fig.3 Shuntogram shows a long atrial catheter (arrowedl lying in the pericardium.
Fig. 1 Plain abdominal radiograph shows peritonea l catheters (arrowed) in a right hydrocoele.
er
scan of the neart shows dye in the pericardial effusion Fig.4 (artowed).
Fig.2 Abdominal ultrasound shows a large intraperitoneal pseudocyst (arrowed).
Flve months later the pati ent pre se nted with symptoms and signs uf congestive hca rt failure . Echoca rd iogram showed evidence of pericardial effuslon. CT scan of the hea d showe d norma l sized ventricles. Shuntogram with CT sca n of the heart (Fig. 3. 4) showed a long atria l ca theter tying in the per icard ium a nd dra ining CSF there . The cat heter was rem oved and a new sho rter cathe ter was inse rt ed into the right atr ium using the left externa l jugula r vein (Fig. fi). The patient recovered a nd remained weil at 2 months follow-up.
Fig.5 ehest radiograph shows the new posltion of the atrial catheters.
157
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Unique complications 0/ cerebrospinalfluid shunts in children
158 Neurochirurgia 35 (19 92)
A. Jamjoom, N. Ur-Rahmen. Z. Jamjoom, A. Jauiad, F. Fad/ey
Discussion
CSF loculation within the abdom en is a well-recognised compllcation of VP shunts. This will lead to the form ation of intraperitoneal pseud ocysts (4. 5.7. 29). Occas ionally the CSF loculation may be in the subphren ic spa ce (1) . lesser sac (8). subhepatic spac e (10). omentum (25). mesentery (18). extraperitoneal space (24) or leads to the forrnation of as cites (11). The path ophysiological rnech-
drocephalus is likely to be matched by an in crease in shunt
com plications. the most cornrnon complications being mechani cal malfunction of the shunt assembly either by dis connection or blockage of one or more of its campone nt
parts and shunt infection (17.20.21). The cases described here illustrate a nu mber of complications. Case 1 had disconnection of the peri-
tonea l cat heter that migrated to the abdomen, developed bilateral hydro coeles. a nd finally the peritoneal cathe ter migrat ed into the right-sided hydrocoele. Case 2 had obstr uction of the VA shunt, recurren t intraperitoneal pseudocysts, and finally the atria J catheter perforated the atrium causing a pericardial effusion.
Disconnection of the shunt asse mbly is a common problern. the preeise ca use of whi ch is not clear. Techn ical errors such as loose ligatur es, over-tight ligat ures cutting through the tubing (32) and th e use of the absorbable ligature material or too many connectors in the shunt assembly may be respons ible. Occasionall y. the disconnection may be caus ed by head injur y when a blow in the vicinity of the va lve or the reservoir may rupturc thc silas tic
tubing (17). The incr eased risk of disconnection with the wire-reinforced periton ea l eathe ters is a clinic al obse rvation mad e by the authors.
anis m for CSF loeulation in the abdom en is not clear. It has been sugges ted that it is related to chronie low- grade in fla mrnatory responses with fibrous eneapsulation of the ir-
ritated area . The irritable focus may be the catheter itself, infccted CSF or xanthochromic CSF (5. 26). It has also been suggested thatthere may be a relation between cyst formation and abdo mina l proeed ures after the initial insertion of
the shunt (4). Dur Case 2 had a pseudocyst ma naged in our hosp ital on two oceasion s . Prior to that sh e had 5 revis ions ofthe peritonea l catheter. It is not clear whe ther thes e revi sions were due to pseud ocysts or obstruction and
wheth er she had a shunt infection in the past which may have bee n resp onsible for the abdominal adhes ions . The
availability of ultrasound (I. 4) made the diagnosis of ab dominal CSF cysts simple. The cysts may be drain ed percutan eously (1); however, lapa rotomy is usually required to reposition the catheter (4. 5). Some authors report that the peritoneal site is unsuitable for shunting after the oee ur-
rence of pseudocysts (26). The association betw een VP shunts and
herni as and hydro coeles in childhood is recognised (2.6. 13.22.23.31). Grosfeld et al. O:J} found 17 % incidence of inguinal hernias in ehildr en with VP shunts. The hernias were bilateral in 70 % of eases and appeared at an average of 6 .8 month s after s hunt insertion . Moa zam et al. (22) con-
cluded that the placement of a VP shunt significantly inObstruction of the VA shunt in Cas e 2 early in her history is a common complication. It is genera lly agreed that the atrial cathe ter is llkely to get obstruc ted if
erease the risk of a child developin g a hernia . Faetors that
its distal end reeedes as high as the s uperi or vena eava or
creased intr a-ab dominal pr essure (6. 13). The pathop hysiology of increased intra-abd omin al pressur e is unclear. Uncertainty exists about wh ether the production of CSF exceeds the ability of the periton eal lining to absorb it or
T4 (17). A review by Keucher and Mealey (19) concluded that periton ea l sh unts rcquired les s revisions than atrial
shunts and that pat ients with VP sh unts suffered from
have been implicated in the developm ent of this comp lication are the patency of the processu s vaginalis and in-
fewer and less serious eomplieations making VP shunts
wh ether CS F malabsorption oceurs se condary to chronie
mor e pop ular than VA shunts (19.21).
irritation of the peritoneum or subclinical peritonitis (2). The processus vaginalis is norm ally patent in 60 -70 % of
Perforation of the bowel by a peritonea l catheter is weil reported (13.16. 33. 35). Less commonly is the perforation of the vagina (27). bladd er (13) or gallbladder (2 8) . Vaseular perforation by an atrial eath etcr is not so
well-described. Other comp llcations of VA shunts such as nephritis (34). endocarditis (3). thrombo embo lism (23) and pulmon ary hypertensi on (30) are, however, documented. In our Cas e 2, there was a tee hnical erro r in placin g the atrial cathe ter too long. It is likely that the tip of the cath eter wh ieh must have been in eo ntaet with the atrial wall, adhere d to it an d then erode d through the wall. The functioning tub e th en proceeded to lie in the pericardium causing a CSF effusion which led to the clinica l man ifestati on of hea rt faiJur e. To our knowledge, this is the first report of a perieardial effusion as a cornplica tion of a VA sh unt. It is unIikely that the catheter was placed in the pericardium at the time of the revision as this wo uld have been technically im-
possible and in addition. the symptoms relat ed to the peri eardial effus ion developed 5 months after the revision .
infants at 3 months of life, in 50- 60 % at age 1 year, and in
40 % of age 2 (13). From 2 to 16 yea rs of age the ineidence of pateney remains about 40 %. The peritoneal eatheter may migrate down the patent proeessus to reach the scrotum (2. 6. 13. 31). Once a hydro coele is diagnosed, repair is required. The functioning catheter should be replaced in the abdo men and the proces sus vagi nalis ligated. In eo nclusion , c1 inician s involved in the long-term managem ent of any patien t with a CSF shunt should be awa re of the multiplicity and the varied presen tation of shunt eomplications .
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Shun ting of CSF remains the tr eatm ent of choiee in ac tive childhood hydroceph alus. The results of othe r therap eutic methods su ch as choroid plexus coagulation are not su fficiently encouraging (l2). The increasing longevity following su ccessful tre atm ent of childhood hy-
Unique complications 01 cerebrospinal fl uid shu nts in children
Agha F P. M. A. Amendola . K. K. S hira zi et al.: Abdom inal eornplications of ventrl culopen tone a l s hunts with em phas is on the role of imaging methods. Sur g. Gyneeol. Obste t. 156 (1983) 473 - 478 2 Albeta. D. M .• J. U-: Danaher. W Th omas Huntsman Ventri cu loperitoneal shunt migrati on into the scrotum. Am. Surg . 55 (1989) 685 - 688 3 Bellamy. C. M .• D. 11. Rooens. D. R. Rams dale: Ventricu loatr ial shunt causing tr icusp id en docarditis: Its percuta neous removal . Int. J . Cardio!. 28 (1990 ) 260-262. -I Briggs. J. R.• G. M . A. /lendry. R. A. Minns: Abdomin al u ltra sound in th e d iagnosis of cereb rospinal f luid pseu docysts com plicating ventri culoper itonea l s hunts. Arch. Dis. Child . 59 (1984) 66 1-664 5 Bryant. M . 5. . A. M . Bre mer. J. 1. Tepas 11/ et al. : Abdominal complication of ventriculo peritoneal shunts . Case re port and revtew of literature. Am . Surg . 54 (198 8) 50 -55 6 Crofford. .\1. 1.. D. Balsam: Sero ta l migration of ventriculoperitonea l sh unts. AJR 14 1 (1983) 36 9- 371 7 Egelhoff. 1.. D. 5. Bab cock. R. M cl.aurin: Cere bros pina l fluid pseudoeysts: Son ographie appeara nee and c1iniea l man agement. Ped iatr. Neurosei. 12 (19 85) 80 -86 S Esch eiman. D. J.. V. W Lee: Lcsser sae eerebrosp inal Iluld eolleetions. An un usual cc mplication of a ventr ieu!oper itoncal s hunt. Clin . Nucl. Med . 15 (1990) 41 5 - 417 . 9 Dav ids on. R. I.: Peritonea l bypa ss in the treatm ent of hydroce phalus: histo riea l revtew a nd a bdo mina l eom plicat ions. J . Neurol. Neu ros urg. Psyeh iat. 39 (19 76) 640 -64 6 10 Gebars k i. K. S. S. Gebar sk i. J. E. JlcGillieuddy: Ccrebrospin al fluid abdo mina l cysts . Computed tomogr a phie resolution of sonograp hlc dilemma . Surg . Neurol. 21 (984 ) 4 14 - 416 11 Goodman. G. M.• G. R, Gour leu. Ascitcs eomp lieati ng ventriculo peritoncal shunts. J . Pediatr. Gastroente rol. Nutr. 7 (19 88) 780 782 12 Griffi th. 11. IJ.. A. B. Jam joom: Th e treat ment of ehildhood hydroceph alus by cho roid plexus eoagulation an d ar tilicial cerebrospinal flui d per fusion . Br, J . Neurosurg . 4 (199 0), 95 - 100 13 Grosfeld. 1. 1..• D. R. Cooney. J. Smith et al. : Intraabdo minal complica tions follow ing ventrieuloperi toneai sh unt pr oc..-dures . Ped iatr ics 54 (1974) 79 1-796 14 /lager. J.. G. Mena rdi. W S chullian: Thro mboe m bolism complication s in ventrlculo at rial dr ninage of cere brospina l Ouid in ch ildho od . Neurochiru rgia (Stuttg) 28 (1985) 8- 11 15 Jamj oom. A. /1.• P. J. E Wil son : Misleading c1iniea l syn dromes of CSF sh unt ma lfunction. Hr. J . Neurosurg. 2(988) 391-394 16 Ja mjoo m. A. B.. J. N. Rawlinson. 1. N. Kirkpalr iek: Pa ssage of tu be per reetu m: as unusual eomplication of a ventric uloperitoneal shunt. BJCI' 44 ( 990 ) 525- 526 17 Jerffrey s. R. Y.: Hydrocephalus. In Mill er; J. D. Ied.]: Northliel ds surgery of the centr al ner vous system. 2nd ed ition. Oxford : Blackwe ll 1987; 567 18 Keen . P E.. S. Weit zn er: Inflammatory pscu dotu mour of mesen tery. a com plication of VP shunt. J . Neurosurg . 38 (1973 ) 37 1373 1
s..
Keu ch er. T. R.. J. Mea ley : Long term resulu.. after ventr iculoatria l and ventriculope ritoneal shunting for infantile hydroe ep hal us . J. Neurosurg . 50 (197 9) 179 - 186 20 McCullough. D. C: Hydroceph alus: Treatment. In wilk ins. H. H.. Hen gachary, S. S. Iedsl , Neurosurgery. New York : Mrfi ra w IliII 1985; 2147 -2 149 21 Mcl.aurln. R. L.: Shunt complication s. In Paedl at ric Neuressurgery. Surge ry of the deve loping nc rvous svste rn. New Yor k: • Gru ne and Stratton 1982; 24-3 -2 52 22 Moazam. F.. J. D. Glenn. B. 1. Kaplan et a l.: Ingu inal hernia after ventriculoperit oneal shunt procedures in pedlatric pat ients. Surg . Gyneeol. Obstet . 159 (198 41 570-572 23 Murtagh. P. R. Lehman : Peritoneal shunts in the ma nage ment of hyd rocephalus . JA\IA 202 (1967) 98- 102 24 N Ilgen t. P.. 5. lIoshek: Large extra -abdomi na l cyst as post-partu rn complicat lons of peritoneal shunt. J . Neurosurg. 64- (1986) 15 1 - 152 25 Parrish . R. A.. 1. ,\I. Polis: Torsion of omental cyst. a rar e com pli cation of ventri culoperi toneal shunt. J . l'ed iatr. Surg . 8 (1973 ) 969- 970 26 Par ry. S. U:-. J. F Sc hu hmake r. R. C. l.l ell'ellyn: Abdominal pseudocysts a nd ascites formation a fter ventriculoperitc nea l shunt proc edure s. J . Neurosurg . 43 (197S) 476 - 480 2 7 Pat ei. C. D.. fl. Mc tlcud: Vaginal perforan on as a com plication of VP shunts. J . Neuros urg. 38 (1973176 1- 762 28 Portn oy. 11. D.. P. D. Croissant: Two unusual com pllcations of a VP shunt dr ainage. J . Neur osu rg. 40 (1974) 772 -77 3 2'J Priee. 11. I.. S. 1. Roeen tha l. S. Betll itzky et a l.: Abdonima l pseudocysts as a com plication of ventrie uloperito nea l shunt . Areport of two cases . Neuroradiol. 2 1 ( 9 81) 273 - 276 30 Pryds. 0. A.. S . A. Pet ersen. 1. H. Ja coösen e t ul.. I'ulmonary hypertension in eh ildren trca tcd with ventriculoatria l shunt in hydr ocephal us. Ugcskr, Lacger. 15 1 (1989) 570 -57 2 31 Rtnnani. P. 5.: Extr usion of a bdominal cntheter of vc ntriculoper itoncal shunt tnto seroturn. J . Neuros urg. 40 (19741772 - 773 32 Sa yers. M . P. : Shunt eornplieations. Clin. Neurosorg. 23 (9 76) 393 -400 :JJ S ch ul hof. L. A.. R. M. IVorlh. J. 1:". Kcls beck: Hew el perforanon due to perit oneal shunt. Surg. Neuro l. 3 (1975) 265 -269 3-4 loth. T.. J. Redl. E Heregi : Shunt ne phr itis with cres cent for mauon. Int. J . Pcd iatr. Nephro l. 8 (987) 231- 234 35 wttson. C. H.• V. Bertan: Perforation of the bowe l com plica.ling perito nea l shunt for hydroeephalus. Hep ort of t\'I.'O cases. Am. SUIll. 32 (1966) 601 - 603
159
I 'J
Dr. A. Jamjoom Assistant Professor or Neurosurgcry King Kha lid Uni\"ersity Ilosp ita l 1'.0 . Box 2925. Riyadh 11472 Sa udi Arabi a
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Refcr en ces
Nell ro chiru rgia 35 (1 992)
Unique complications of cerebrospinal fluid shunts in children - AReport of Two Cases
Sum mary
Th e authors rep ort on two cas es with un usual CSF shunt complications. Th e firs t cas e had a peri toneal ca theter whi ch migra ted dow n a patent proces sus vaginalis into a hydrocoele. Th c second ease ha d a n a tr ial ca the ter wh ich perforated th e a trial wall a nd came to lie in the per icardium ca using an effusion. Clinicians sho uld be a wa re of th e fre quent and aceasiona lly biza rre complications of CSF shunting. Un gewöhnliche Komplikation en bei liquorshunt bei Kind ern Zwe i Fallberichle
Es wird üb er zwei Fälle un gewöhn licher Kom plikationen bei Liquorshunt berichtet. Im ersten Fall wanderte ein Peritonealkat heter in eine Hydrozele durch einen durchgä ngigen Processus vagina lis. Im zweiten Fall perfori erte ein Vorholkatheter die Vor hofwan d und gela ngte in das Perika rdium. wo sich ein Er guß bildete. Klinike r so llten a uf die Möglichkeit häufiger und gelegentlich bizarrer Komplikationen bei lJq uorshu nts achte n. Key wo rds Ch ildhood hydro cephalus - Venlriculoperitoneal sh unt - Ventriculoat rial shunt - CompUcation s Abdomin al pseud ocyst - Hydr ocoele - Vascula r perforation
Introducti on Ch ildhood hydrocephalus is a common disord er which is usua lly tr eat ed by cere brospina l fluid (CSF) diversion using a ven trieuloperiton eal (VP) or a ve ntric uloatrial (VA) sh unts. Th e com plications of these devices a re varied an d largely weil docum ented (9. 17. 20. 21, 32). While neurosurgeon s are a ware of the eommon complica tions, they a re still surprised to see the oecas iona l rare a nd biza rre com plieations. The re is a n increasi ng nu mber of hydrocephalic children with CSF shunts in the communily.
Neurochirurgia 35 (1992 ) 156 - 159 © Georg Thieme Verlag. Stuttgart . New York
a nd these patients may develop symp toms and signs relat ed to the a bdomen or ehes t and may present to other clinicia ns with a pro blem that may initially seem unrelat ed to the shunt (1 5). We describ e two case s with unu sual shu nt cornplications. Th e first case had a per itoneal catheter wh ich pr olaps ed into a hydrocoele a nd the seco nd cas e had an atrial catheter which per forated the right a triu m a nd came to He in the pericardium causing an effusion . Case llist ori es Case 1: A 14-month old ma le child with congenital hydr oceph aJus had a right-sided VP shun t 3 wee ks afte r birth . A year later he pr esented with a 3-day history of head ache. vomit ing and drowsiness. In additic n. the patien t ha d a swelling at th e site of the reservoir exte nd ing down to the n eck. Pla in ab dominal radi ograp h showed the per itonea l cathe ter had disconne cted and migr ated dow n to th e abdomen. A n ew peri ton ea l cath eter was con necte d to the existing functioni ng ven tricu lar catheter a nd reservolr, At ope ra tion there wa s some difficu lty remo ving the old periton eal ea the te r via the sma ll right s ubccstal incision so the tube was left behind . Th e pat ien t made a good post-operative re covery. He was noted to have s ma ll bila teral hydroeoeles which were ma naged conse rva tively in the first instance . 1Wo months lat er; the patient pr es ent ed with an enla rging right-s ided scrotal swelllng. The s hunt tubing wa s elinically palpable in the hydroeoele . Plain abdominal radi ograph (Figure 11showed th at the old and the n ew perit oneal cathe ters had prolapsed into the right hydroeoele. Via a righ t gro in incision the old catheter was removed. the new funetio ning catheter was rep laeed in the peritoneum an d the proeessus vagina lis was cIosed . The left side was also repaired . The patient mad e a good recovery. Case 2: A fs-yea r-old girl with conge nita l hydro ceph alus ha d a right -sid ed VA shunt soo n a fter birth . A year later the shunt beeam e obstr ucted and a new left VP s hunt was inserted . Over the next four years the pati en t had live revision s of the per iton ea l cathe ter elsewhere . A yea r ago the patien t was admitted to King Khalid Un iversity Hospita l with a swelling uver the abdom ina l sea r. The patien t was oth erwise cIinica lly weil a nd the CT sean s howed norma l s ized ventricles . At explorano n. the peri to nea l cathet er was foun d to be d rai ning CSF satisfactori ly. The cathe te r was retn serted into a fresh a rea of the a bdome n.
Four months lat er the patient wa s readmltted with a large abdo mina l swe lling an d vomiti ng. Abdomi nal ultra sound (Fig. 2 ) sh owed a larg e int ra peritoneal eyst conta ining fluid proved to be CSF on per cuta neous aspiration . At exploration ther e were multiple a dhes ions a ll over the perito neal cavi ty. Th e left-sid ed s hu nt system was therefore cha nged to a VA sh un t by con necting the old functionin g rese rvoir to a n atria l cathe te r w hich was inserted via the left intern al jugular vein into the right atrium . The patient recovered we il.
Downloaded by: University of Pennsylvania Libraries. Copyrighted material.
A. Jamjoom ' , N. Ur-Rahman J. Z. A . Jamj oom J. A. Jauiad ", F. Fadl ey 3 1 Divi sion of Neu rosurgery. Z Paediatric Surgery and 3 Paed iatr ic King Khalid Unive rslty Hospi tal. Hiya dh. Sa udi Ara bia
Neurochiru rgia 35 (199 2)
Fig.3 Shuntogram shows a long atrial catheter (arrowedl lying in the pericardium.
Fig. 1 Plain abdominal radiograph shows peritonea l catheters (arrowed) in a right hydrocoele.
er
scan of the neart shows dye in the pericardial effusion Fig.4 (artowed).
Fig.2 Abdominal ultrasound shows a large intraperitoneal pseudocyst (arrowed).
Flve months later the pati ent pre se nted with symptoms and signs uf congestive hca rt failure . Echoca rd iogram showed evidence of pericardial effuslon. CT scan of the hea d showe d norma l sized ventricles. Shuntogram with CT sca n of the heart (Fig. 3. 4) showed a long atria l ca theter tying in the per icard ium a nd dra ining CSF there . The cat heter was rem oved and a new sho rter cathe ter was inse rt ed into the right atr ium using the left externa l jugula r vein (Fig. fi). The patient recovered a nd remained weil at 2 months follow-up.
Fig.5 ehest radiograph shows the new posltion of the atrial catheters.
157
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Unique complications 0/ cerebrospinalfluid shunts in children
158 Neurochirurgia 35 (19 92)
A. Jamjoom, N. Ur-Rahmen. Z. Jamjoom, A. Jauiad, F. Fad/ey
Discussion
CSF loculation within the abdom en is a well-recognised compllcation of VP shunts. This will lead to the form ation of intraperitoneal pseud ocysts (4. 5.7. 29). Occas ionally the CSF loculation may be in the subphren ic spa ce (1) . lesser sac (8). subhepatic spac e (10). omentum (25). mesentery (18). extraperitoneal space (24) or leads to the forrnation of as cites (11). The path ophysiological rnech-
drocephalus is likely to be matched by an in crease in shunt
com plications. the most cornrnon complications being mechani cal malfunction of the shunt assembly either by dis connection or blockage of one or more of its campone nt
parts and shunt infection (17.20.21). The cases described here illustrate a nu mber of complications. Case 1 had disconnection of the peri-
tonea l cat heter that migrated to the abdomen, developed bilateral hydro coeles. a nd finally the peritoneal cathe ter migrat ed into the right-sided hydrocoele. Case 2 had obstr uction of the VA shunt, recurren t intraperitoneal pseudocysts, and finally the atria J catheter perforated the atrium causing a pericardial effusion.
Disconnection of the shunt asse mbly is a common problern. the preeise ca use of whi ch is not clear. Techn ical errors such as loose ligatur es, over-tight ligat ures cutting through the tubing (32) and th e use of the absorbable ligature material or too many connectors in the shunt assembly may be respons ible. Occasionall y. the disconnection may be caus ed by head injur y when a blow in the vicinity of the va lve or the reservoir may rupturc thc silas tic
tubing (17). The incr eased risk of disconnection with the wire-reinforced periton ea l eathe ters is a clinic al obse rvation mad e by the authors.
anis m for CSF loeulation in the abdom en is not clear. It has been sugges ted that it is related to chronie low- grade in fla mrnatory responses with fibrous eneapsulation of the ir-
ritated area . The irritable focus may be the catheter itself, infccted CSF or xanthochromic CSF (5. 26). It has also been suggested thatthere may be a relation between cyst formation and abdo mina l proeed ures after the initial insertion of
the shunt (4). Dur Case 2 had a pseudocyst ma naged in our hosp ital on two oceasion s . Prior to that sh e had 5 revis ions ofthe peritonea l catheter. It is not clear whe ther thes e revi sions were due to pseud ocysts or obstruction and
wheth er she had a shunt infection in the past which may have bee n resp onsible for the abdominal adhes ions . The
availability of ultrasound (I. 4) made the diagnosis of ab dominal CSF cysts simple. The cysts may be drain ed percutan eously (1); however, lapa rotomy is usually required to reposition the catheter (4. 5). Some authors report that the peritoneal site is unsuitable for shunting after the oee ur-
rence of pseudocysts (26). The association betw een VP shunts and
herni as and hydro coeles in childhood is recognised (2.6. 13.22.23.31). Grosfeld et al. O:J} found 17 % incidence of inguinal hernias in ehildr en with VP shunts. The hernias were bilateral in 70 % of eases and appeared at an average of 6 .8 month s after s hunt insertion . Moa zam et al. (22) con-
cluded that the placement of a VP shunt significantly inObstruction of the VA shunt in Cas e 2 early in her history is a common complication. It is genera lly agreed that the atrial cathe ter is llkely to get obstruc ted if
erease the risk of a child developin g a hernia . Faetors that
its distal end reeedes as high as the s uperi or vena eava or
creased intr a-ab dominal pr essure (6. 13). The pathop hysiology of increased intra-abd omin al pressur e is unclear. Uncertainty exists about wh ether the production of CSF exceeds the ability of the periton eal lining to absorb it or
T4 (17). A review by Keucher and Mealey (19) concluded that periton ea l sh unts rcquired les s revisions than atrial
shunts and that pat ients with VP sh unts suffered from
have been implicated in the developm ent of this comp lication are the patency of the processu s vaginalis and in-
fewer and less serious eomplieations making VP shunts
wh ether CS F malabsorption oceurs se condary to chronie
mor e pop ular than VA shunts (19.21).
irritation of the peritoneum or subclinical peritonitis (2). The processus vaginalis is norm ally patent in 60 -70 % of
Perforation of the bowel by a peritonea l catheter is weil reported (13.16. 33. 35). Less commonly is the perforation of the vagina (27). bladd er (13) or gallbladder (2 8) . Vaseular perforation by an atrial eath etcr is not so
well-described. Other comp llcations of VA shunts such as nephritis (34). endocarditis (3). thrombo embo lism (23) and pulmon ary hypertensi on (30) are, however, documented. In our Cas e 2, there was a tee hnical erro r in placin g the atrial cathe ter too long. It is likely that the tip of the cath eter wh ieh must have been in eo ntaet with the atrial wall, adhere d to it an d then erode d through the wall. The functioning tub e th en proceeded to lie in the pericardium causing a CSF effusion which led to the clinica l man ifestati on of hea rt faiJur e. To our knowledge, this is the first report of a perieardial effusion as a cornplica tion of a VA sh unt. It is unIikely that the catheter was placed in the pericardium at the time of the revision as this wo uld have been technically im-
possible and in addition. the symptoms relat ed to the peri eardial effus ion developed 5 months after the revision .
infants at 3 months of life, in 50- 60 % at age 1 year, and in
40 % of age 2 (13). From 2 to 16 yea rs of age the ineidence of pateney remains about 40 %. The peritoneal eatheter may migrate down the patent proeessus to reach the scrotum (2. 6. 13. 31). Once a hydro coele is diagnosed, repair is required. The functioning catheter should be replaced in the abdo men and the proces sus vagi nalis ligated. In eo nclusion , c1 inician s involved in the long-term managem ent of any patien t with a CSF shunt should be awa re of the multiplicity and the varied presen tation of shunt eomplications .
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Shun ting of CSF remains the tr eatm ent of choiee in ac tive childhood hydroceph alus. The results of othe r therap eutic methods su ch as choroid plexus coagulation are not su fficiently encouraging (l2). The increasing longevity following su ccessful tre atm ent of childhood hy-
Unique complications 01 cerebrospinal fl uid shu nts in children
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Dr. A. Jamjoom Assistant Professor or Neurosurgcry King Kha lid Uni\"ersity Ilosp ita l 1'.0 . Box 2925. Riyadh 11472 Sa udi Arabi a
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Refcr en ces
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