University of Groningen
Severe bacterial meningitis due to an enterothecal fistula in a 6-year-old child with Currarino
syndrome
Jeltema, Hanne-Rinck; Broens, Paul M. A.; Brouwer, Oebele F.; Groen, Rob J. M.
Published in:CHILDS NERVOUS SYSTEM DOI:
10.1007/s00381-019-04138-8
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Jeltema, H-R., Broens, P. M. A., Brouwer, O. F., & Groen, R. J. M. (2019). Severe bacterial meningitis due to an enterothecal fistula in a 6-year-old child with Currarino syndrome: evaluation of surgical strategy with review of the literature. CHILDS NERVOUS SYSTEM, 35(7), 1129-1136. https://doi.org/10.1007/s00381-019-04138-8
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REVIEW ARTICLE
Severe bacterial meningitis due to an enterothecal fistula
in a 6-year-old child with Currarino syndrome: evaluation of surgical
strategy with review of the literature
Hanne-Rinck Jeltema1 &Paul M. A Broens2&Oebele F. Brouwer3&Rob J. M. Groen1 Received: 12 February 2019 / Accepted: 21 March 2019 / Published online: 9 April 2019
# The Author(s) 2019 Abstract
Meningitis is a rare but serious complication in patients with Currarino syndrome. We present a 6-year-old girl with a fulminant meningitis due to an enterothecal fistula involving the anterior sacral meningocele. Initial treatment consisted of broad-spectrum intravenous antibiotic therapy and laparoscopic construction of a deviating double-loop ileostomy. This was followed by an elective posterior neurosurgical approach with a sacral laminectomy, evacuation of the empyema, and securing the disconnection of the anterior meningocele from the thecal sac, 10 days after initial hospital admission. The girl made a good postoperative recovery. The treatment strategy in the setting of meningitis due to an inflamed anterior meningocele is discussed and the available literature on the topic is reviewed.
Keywords Bacterial meningitis . Currarino syndrome . Enterothecal fistula
Introduction
In 1981, Guido Currarino et al. were the first to describe a triad consisting of (1) partial sacral agenesis; (2) presacral mass (an-terior meningocele, enteric cyst, teratoma); and (3) anorectal malformation/stenosis [10]. There are no generally accepted guidelines about the indication and timing of surgical correction of an anterior sacral meningocele. Neither are there known risk factors predicting which patients with Currarino syndrome are prone to develop meningitis due to an enterothecal fistula. It is also unknown whether there is a relationship between (increase of) the size of the anterior meningocele and the chance of de-veloping an enterothecal fistula. Different surgical approaches (anterior/posterior/sagittal) to close and resect the meningocele have been reported in the literature [25]. Here, we present our experience with a young patient with Currarino syndrome
suffering from meningitis due to an enterothecal fistula and give an overview of the available literature on the topic.
Case report of an illustrative patient
A 6-year-old girl presented with headache, drowsiness, and opisthotonus. She had been diagnosed with familial Currarino triad with associated constipation and micturition problems. Repeated lumbosacral MRI scans over the years had revealed slight increase of the anterior meningocele (see Fig.1). Four days before admission, the patient was treated with antibiotics in another hospital because of a suspected urinary tract infec-tion. At presentation in the emergency room, she was drowsy (GCS 3-5-2) with severe opisthotonic posturing (see Fig.2a), but without focal neurological signs. Her body temperature was 36.6 °C. Blood leucocyte count and C-reactive peptide were 23.2 × 109/L and 214 mg/L, respectively. Analysis of the cerebrospinal fluid (CSF) revealed a pleocytosis with 6659 × 106/L cells and glucose < 0.1 mmol/L. Antibiotic treatment was immediately started and consisted of intrave-nous ceftazidime and metronidazole for 6 weeks. Culture of the CSF rendered Streptococcus anginosus (milleri) and Bacteroides fragilis. Because of the multimicrobial culture and her medical history, an enterothecal fistula was suspected. Gadolinium-enhanced MRI of the lumbosacral region * Hanne-Rinck Jeltema
j.r.jeltema@umcg.nl 1
Department of Neurosurgery, University Medical Center Groningen, Hanzeplein 1, 9713 GZ Groningen, Netherlands
2
Department of Pediatric Surgery, University Medical Center Groningen, Hanzeplein 1, 9713 GZ Groningen, Netherlands 3 Department of Pediatric Neurology, University Medical Center
Groningen, Hanzeplein 1, 9713 GZ Groningen, Netherlands
revealed inflammation of the anterior meningocele with ab-scess formation (see Fig.2b).
Multidisciplinary rounds with pediatric neurologists, pediatric surgeons, pediatricians, microbiologists, and neurosurgeons re-sulted in the decision to first perform a laparoscopic deviating double-loop ileostomy in the acute stage, in order to stop the inflow of enteral commensals in the CSF space and inflamed retroperitoneal and epidural area. A few days after the formation of an ileostomy, a contrast study of the rectal stump confirmed leaking of contrast through a rectal fistula to the area of the anterior meningocele. Ten days after hospital admission, a pos-terior sacral laminectomy was performed with evacuation of a large amount of empyema and debris from the anterior meningocele and the region around the sacral roots. There was severe fibrosis in the operating field. The connection between the anterior meningocele and thecal sac had closed spontaneously due to inflammation. No patent fistula was found.
After uncomplicated surgery, the clinical course was dom-inated by the aftermath of the fulminant meningitis. Postoperative MRI confirmed obliteration of the anterior sa-cral meningocele/enterothecal fistula and a decline of
inflammation (see Fig. 3). Initially, the ventricular system was dilated, which was managed by intermittent lumbar CSF-taps. No internal CSF shunt was needed as the hydro-cephalus resolved after recovery from the meningitis. Successful restoration of the ileostomy was performed several months later. The girl made a good physical and neurological recovery.
Review of the literature
In an attempt to collect data supportive for any specific strategy or approach, we performed a literature search on the topic of Currarino syndrome and meningitis in the databases of PubMed, Web of Science, and Embase, using the following search terms: [currarino], [meningitis], [anterior meningocele], and [inflammation]. The search rendered 37 publications describ-ing 38 patients (Table1). All were single case studies, except for one article describing two young infants suffering from the con-dition. The series comprises 17 pediatric patients and 20 adult Fig. 2 a Sagittal T2–weighted
MRI shows severe opisthotonic posturing at presentation in the emergency department.b Sagittal T1–weighted MRI with gadolineum shows enhancement of the anterior sacral meningocele with the formation of several in-flamed empyema pockets
Fig. 3 Postoperative sagittal T2–weighted MRI shows obliteration of the anterior sacral meningocele after neurosurgical exploration and evacua-tion of the empyema
Fig. 1 Sagittal T2–weighted MRI shows the anterior sacral meningocele during follow-up, approximately 1 year before the patient developed meningitis
Table 1 Ov erv iew of the articles d escrib ing patients w ith C urrarino syndr ome/an terior sacral menin go cele w ho develope d m eningitis Author Age of the patient(s ) and sex (M/F ) B rief summary Microbial pathogen(s ) Inform ation o n sur gical approach/strategy and timing of sur gery Br ac zy nsk i et al . 20 17 [ 7 ] 71 years (F) Meningitis becaus e o f an infected anterior me ning oc ele in a pa tie nt su ff er ing fr o m colo re cta l ca rc ino ma . Fuso bac te riu m nuc le atu m Po ste rio r appr oa ch . L am ine ct omy S1 –3w it hc lo su re of th e the ca l sa c an d amp uta tio n o f the me ning oc ele .Sur g er y ap p roxim ate ly 2 w ee k s afte r the p resent ati o n wit h m eningiti s. Al Qahtani et al. 20 16 [ 1 ] 14 mont hs (F ) P assi ng aw ay of an in fa nt wi th Cur ra ri n o sy ndr om e because o f a sacral fi st ul a causing meningitis . Ext en ded -s pec tr um b eta -la cta m ase s Esch er ich ia co li , Ente ro co cc us fa ec iu m No surger y p er for me d. Pa ul et al. 2 01 5 [ 32 ] 30 yea rs (F) Py oge nic m en in giti s in a pa tie nt wi th an an te rio r me ning oc ele En ter o co cc i N/A M o rg en stern et al. 20 15 [ 28 ] 9 d ays (1s t episo de of me ni ngit is) and 7 m ont hs (2nd epis ode of meningitis ) (M ) The article describes 4 pa ti ent s with re cu rr en t meningitis . O ne of these p at ients h ad a fi stul a and Cu rr ar ino syn dro m e. 1s t episode of meningitis Str eptococcus b ovis 2nd episode of meningitis Enter o coccus faeci u m, Klebsiella pneumonia, Escher ichia coli , Citr obacter fr eu ndi i, E. faecium The C SF fist ula was su rg ic ally repaired NOS. T iming o f su rg ery n ot sp ec if ied . Ganeshalingham et al. 2 0 1 4 [ 17 ] 8 w ee k s (M ) P assi ng aw ay of an infa nt d ue to p olym icr ob ial (enteral) m eningitis . S tr on g suspi cion of Currarino sy nd ro m e, b ut n o po st -m or tem inv es tiga tio n p er -fo rme d. Escherichia col i, B. fr agi lis No surger y p er for me d. Patnai k et al. 2013 [ 31 ] 8 m onths (M) Meningitis in a p atient wit h an anterior sacral me ning oc ele and ep id ura l ab sc es s Sta phy lo coc cus a ur eu s Po ste rio r appr oa ch . L am ine ct omy L5 –S3. D ra inage of the ep idur al ab sce ss an d int ra dur al clo sur e o f the me ning oc ele w ith a fas cia l pa tc h. T imi ng o f sur g er y n o t specifi ed. M o n sea ux et al. 20 13 [ 27 ] 29 yea rs (F) Pr es en tat ion wi th pa ra pl eg ia in the co n te xt o f a me ning ea l in fe cti on in a p ati en t with an an te ri or me ning oc ele N/A N/A Kan sal et al. 2 0 1 2 [ 21 ] 45 years (F) Recurrent aseptic meningitis in a p atient with an ep ide rmo id tumo r in h er an te rio r me nin goc el e. Aseptic meningitis Po ste rio r ap p ro ach. Sa cr al lami ne cto m y. Th e d u ra w as ope ne d in the m idl ine . An an te ro la te ra l d ur a d ef ec t was clo sed b y d irect sut u ri ng. T im ing o f su rg ery not sp ec if ie d. C al leja A gu ay o et al. 2 0 1 2 [ 8 ] 8 m on ths (M) R ecu rr en t m en ing iti s in a patient wi th Currarino syn dro me an d an an ter ior sa cr al men ing oc ele an d a re ct al fi stu la . S tr ep toc oc cu s b o vis , E nte roc oc cu s fa ecium, Escherichia coli, Klebsiella pneumoniae M ass ex cision , co m plica ted by a rec to-cu tan eo u s fi s-tula NOS. A colost oma and VP shunt inserti on was ne ce ssa ry . T imin g of surger y n ot sp ec if ied . Ant una -Ra m os et al. 2 0 11 [ 2 ] 10 years (M) Meningitis due to a para rectal abs cess with connect ion to the anteri o r sacral m eni ngocele Bacterial m eni ngi tis NOS Po ste rio r appr oa ch . T imin g of sur g er y no t spe ci fi ed. Kok sa l et al . 20 1 1 [ 23 ] 44 years (F) Meningitis in a p revi ous ly healthy woman wit h an an ter ior sa cr al men ing oc ele Esch erich ia co li Po ste rio r appr oa ch . S ac ra l route. E xcis ion o f the fi stu la tra ct. R ep ai r o f th e sac o ri fice with sutu re s an df ib ri ng lu e. S u rg er yp er fo rm edo nt h e 4 thd ay of hospital admis sion. Ra cz ynski et al. 20 10 [ 35 ] 5m o n th s (F ) 2m o n th s (F ) Meningitis caused by a fist u la in two young pati ents with Currarino syndrome. Esc h eric hia col i and Pr ote u s m ir abi lis (5 -mo nth -ol d pa tie nt) and Ps eu domo nas ae ru gino sa (2 -mont h-o ld p atie nt) Sur g ical repair NOS. T imi n g o f sur gery not speci fied. B ah tia et al . 201 0 [ 5 ] 9 y ea rs (F ) M enin gitis in a ch ild wi th mult iple occult spi n al dys ra ph ism stigmata, among whic h an anter ior meningoce le , a de rmal sinus tract, caudal regression sy ndrome, and tethered spinal cord. After sur gery , a second episode of meningitis occurr ed. Kleb sie lla pne umon iae , St re pt oc occ u s spe ci es Po ste rio r appr oa ch . L am ino tomy L 5 and in tra du ra l ex plor ation .Disc on ne cti o n o f the pyo gen ic sa c an d the thecal sac and secti oning of the fatty fil u m te rm ina le . Su rg er y p erf o rm ed af ter 3 week s o f intravenous antib io tic treatment.
Ta bl e 1 (continued) Author Age of the patient(s ) and sex (M/F ) B rief summary Microbial pathogen(s ) Inform ation o n sur gical approach/strategy and timing of sur gery Simon et al. 20 10 [ 38 ] 3 0 y ea rs (F ) Men ingo-en ce p halitis and mening o-myelitis in a patient with Currarino syndrom e an d an anterior sacral m en ingo cele, a d ermoid cyst, and te thered spinal cord . N/A A su rgic al in ter ve nti o n w ith aspir at ion an d liga tio n o f the an ter ior me nin goc el e and te th er ed spin al cor d re lea se NOS. T im ing o f sur ge ry not spe ci fie d. B er g er on et al. 20 10 [ 4 ] 40 years (F) Ascending m eningitis and cauda equi na syndrome ca u sed by a rec tal-th ec al fistula in a pati ent w ith Cu rr ar ino syn dro m e. Esc h eric hia col i, gr o u p F st re pt oc occ i, Bac te ro ide s fr agil is, Pe pto str ep to co cc us an aer o biu s, C and ida g la brat a An ter ior ap p roa ch . R em ov al of th e m en ing o cele w ith a tr an s-abd omi na l ap pro ac h. C lo sur e o f th e sac ra l def ic it b y sut uri n g a str ip o f w ell-va scu lar iz ed ome n -tum and fib rin glu e. S ur g er y on the d ay of hos pita l adm iss ion. Kief er et al . 2 0 0 9 [ 22 ] 20 day s (F) T re at me nt -r esi sta nt me ni ngit is (CSF p le oc ytos is) in a patient with Currar ino syndrome and a rectal-thecal fis tula p rove n b y my elo gr aph y. No pa thog en isol ate d fr o m CS F a nd b loo d cultur es Po ste rio r appr oa ch . S ac ra l la min ec tom y an d re pa ir of the anterior d ural defect with fibrin glue and m uscl e g raft. T im ing o f su rg er y n ot sp ecif ie d . San che z et al. 2 008 [ 36 ] 64 yea rs (M ) Ba ct er ia l me n in git is in a pa tie nt wi th an an te ri or sa cr al me ning oc ele . En ter o co cc us fa eca lis, E sch erich ia co li Ante rio r appr oa ch . L ap ar ot omy . Res ec tion o f a por tio n o f th e re ct um an d the m en ingo ce le .A ter min al col ost omy w as p er fo rme d. T im ing o f su rge ry not speci fied. Mi let ic et al. 2008 [ 26 ] 39 years (M) Meningitis and a la rg e anteri o r sacral m eningocele. Esch erich ia co li Po ste rio r ap pro ac h. Lam ine ct omy w it h d ura l ope ni ng. Oblit er ati o n o f th e co mm uni cation o f the intrat hecal com pa rtm en t and the me nin goc el e. T iming of su rge ry not sp ec if ie d. Fitouri et al. 2007 [ 12 ] 3. 5 y ears (F) R epetiti ve mening itis in a p at ient with Currarino syndrome including a mature te rat o ma Esc h eric hia col i, St re pt oc occ u s B , H ae moph ilus inf lue nza e Su rg ic al cu ra ti on o f th e p re sa cr al cy stic mas s NOS . T im ing o f su rg er y not sp ec if ie d. F leu ry et al . 200 7 [ 15 ] 29 day s (F) M ul tipl e fa mi ly me mb er s w ith Cu rr ar ino syn dro me . A p atient with meningitis and C urrarino syndrome with a m atu re te ra tom a is d escr ib ed . Esch erich ia co li, Ba cter o id es Posterior approa ch. R epair b y w ay of a sagittal app ro ac h . S ur g ic al tr eatm en t 21 da ys af ter antib ioti c treat m ent/meni ngi tis. Hata no et al. 2 0 0 6 [ 20 ] 46 yea rs (F) Ma rf an synd ro me an d inc omp le te C ur ra ri no tr ia d, p re sen tin g with re cu rr en t m en in g itis an d an an ter io r sa cr al m en ingo ce le . N/A S u rg ical app roa ch was limited to plasty of the m ening oc ele NOS. T im in g o f su rgery no t spe cified . Phill ips et al. 2006 [ 33 ] 48 years (M) Meningitis due to a rectal -thecal fist u la in a p atient with an an ter io r sa cr al m en in g o ce le An ae ro bic g ra m-n eg a tiv e b ac illu s Ante rio r appr oa ch . L ap ar ot omy w as p er fo rme d. Th e nec k o f th e m eni ngo ce le was o ve rse w n. Co ver age o f the d ef ec t w it h o m en tum. T im ing o f sur ge ry not speci fied. Sc hijm an et al. 20 05 [ 37 ] 1 mo n th (F ) T he p ati ent d ev elo p ed mult ibact erial meningi tis at th e ag e o f 1 mo nth. At the age o f 3 m onth s, she de vel ope d p ar ap leg ia d ue to an in tra me dul lar y ab sce ss . Pse udo mona , P ro te us , E sc her ic h ia co li , Aer o bac te r P o sterior app roa ch. Sacra l lamino to my . A cystic te ra to m a wa s rem ov ed . W at er ti g h t cl o su re of th e spi na l canal w ith an ap one uro sis p atc h g raft. S u rgical trea tm ent of th e anterior men in goc ele w as p erfo rme d 3 w eek s af ter th e p resen ta tion w ith p ar apleg ia. Ema n s et al. 200 5 [ 11 ] N/A E xpr es sion pa tte rns o f C u rr ar ino synd ro me ar e described. In this arti cle, one p ati ent with meni ngi tis is me ntio ne d. N/A Operative treatment NOS . T iming o f surgery not speci fied. Bal et al. 200 4 [ 3 ] 3 5 y ears (F) (Poss ibly iatrogenic ) infected anterior m eningocele af te r tr an sre ct al pun ctu re . N/A Po ste rio r appr oa ch . S ac ra l la min ec tom y. T h e n ec k of the anterior sacral m eningo cele was tied o ff . T iming of su rg ery not spe ci fie d. Ha ga et al. 200 3 [ 19 ] 5 8 y ear s (F) P atien t with re cu rr en t m en in g itis an d Cu rr ar in o triad (wit h int ra dur al ep idermoid cyst ). Co ryn efo rm ba cteria Po ste rio r appr oa ch . S ac ra l la min ec tom y. O p en ing of the dur a. Nec k li gat ion wa s p er fo rme d . T im ing o f su rg er y n ot sp ecif ied .
Ta bl e 1 (continued) Author Age of the patient(s ) and sex (M/F ) B rief summary Microbial pathogen(s ) Inform ation o n sur gical approach/strategy and timing of sur gery C hou et al . 2 0 0 2 [ 9 ] 3 m o n th s (M ) In fa nt wit h me nin g iti s an d Curr ar in o sy ndr ome . Bacterial m eni ngi tis NOS N/A Gu er in et al. 2 0 0 0 [ 18 ] 23 yea rs (F) Po lymi cr obi al m en ingi tis (me n in giti s af te r a gyn ec olo gic pun ct ure ) le ads to dis co ve ry o f an an ter ior sa cr al men ing oc ele . Enter o coccus faecalis , Pr evotella bivia, Str epto co cc u s con ste llat u s Co mmu nic ati on be twee n the en do dur al lum en an d the men ing oc ele o n the S2 le ve l w as cl ose d wit h ad ipo se tiss u e and biol ogi ca l g lu e NOS. S ur g er y on the 22n d day o f hos pita l admis sio n . Fitzpatrick et al. 19 99 [ 13 ] 31 years (F) The p at ient develo ped m eningitis aft er a diagnos tic laparotomy as part of the invest igation o f h er infertili ty . A presacral m as s was found, but n ot further ex plor ed at th at tim e. S ur g er y w as p er fo rme d 18 y ea rs la te r b ec au se o f m u co pu ru len t re cta l d isch ar g e. Esch erich ia co li, Ba cter o id es Po ste rio r appr oa ch . A sa cr al app ro ac h from the mids ac ru m to the ana l ma rg in wa s u se d. Th e n ec k o f th e m en in go ce le was liga ted . T he sa cr al d ef ect wa s re p air ed u sin g adja ce n t fascia. Th e su rge ry was planned in el ective setting. T ama yo et al . 199 9 [ 40 ] 24 years (M) Patient wi th multibacte rial (ent eral ) m eningit is and Cu rr ar ino syn dro m e. an aer o bic E nte roc oc ci, B act er o id es fra g ili s, Esch erich ia co li N/A Funa ya ma et al . 19 95 [ 16 ] 4 m o n th s (1 st epis ode of me ni ngit is) an d 1 ye ar (2 n d ep isod e of m en ing itis ) (M ) The p at ien t die d 1 m ont h af te r hi s la st ho spi tal admiss ion, due to se vere meni ngeal infection and se psis . A uto p sy co nfi rme d an ante ri o r sac ra l me ning oc ele and in tra sp ina l ab sc es s fo rma tio n. Pr oteus mi rabilis, K le bsiella pneumoniae No surger y p er for me d. O ’R ior da in et al . 19 91 [ 29 ] 15 yea rs (F) T en fa m il y m em be rs with Cur ra ri n o synd rom e. Meningitis described. Bacterial m eni ngi tis NOS N/A Blon d et al. 19 91 [ 6 ] 7 y ear s (F) T h e pa tie n t d ev elo p ed po lym icr ob ial m en ing itis after falling o n h er os coccygi s. A small ant erior sacral me ning oc ele w as dis co ver ed together with spina bif ida oc cu lta at L5 lev el and a sc imi tar sa cr u m . Str epto co cc us spe cie s, B a ct er oi des spe ci es Po sterior appro ach. T h e fis tula w as ligated. Su rg ery p erform ed d urin g the th ird week of an tib iotic therap y. Pa ge et al. 1 9 9 0 [ 30 ] 27 years (M) Meningitis due to rect al fis tulation o f the meni ngocele S ter co ra l flo ra NOS Cu ra tion af ter sev en sur gi ca lp rocedures NOS. T iming of su rg ery not spe ci fie d. Fiuma ra et al . 1 989 [ 14 ] 36 yea rs (F) Pu rul en t m eni ngi tis in a p atient with an ante ri or sa cr al me ning oc ele Ba cteriu m co li N/A S yno witz et al . 19 88 [ 39 ] 19 yea rs (M ) The p at ien t pr es ent ed w ith an in fe ct ed ant er ior me ning oc ele Esch erich ia co li Po ste rio r appr oa ch . S ur gi ca l cl osu re of the men ing oc ele w as obt ain ed usin g a dor sa l tra ns dur al app ro ac h with su tur es and fi bri nou s adh es ive . T im ing of su rg ery not spe ci fie d. Qui g ley et al. 1984 [ 34 ] 21 years (F) Recurrent aseptic meningitis in a p atient with an an ter ior sa cr al men ing oc ele an d a de rmo id tu mor Aseptic meningitis Po ste rio r appr oa ch . T ra nssa cra l ap pr oa ch wi th a lam ine ct omy L 4 to S 1. Ex tir pa tion o f the d er m o id tumor , detethering and obl iteration o f communicat ion bet w ee n th e th ec al sac and th e ante rior sac ra l men ing oc ele . Sur g er y w as pla n n ed in el ec tiv e se tti ng af ter re cove ry fr om th e last epi sod e of as ep tic meningitis . N/A not avai labl e, NO S not otherwise specified
patients (information on the age of one patient missing). The known female predominance of Currarino syndrome was con-firmed with a F:M ratio of 24:13 (information on the gender of one patient missing). These case reports show that meningitis due to formation of an enterothecal fistula can be fatal [1,16,17]. In the series here described, a posterior surgical approach was cho-sen in 16 patients and an anterior surgical approach was chocho-sen in 3 patients. For 16 patients, the surgical technique was not described in detail. In 3 cases, no surgery was performed (these 3 cases related to the three deceased patients in the series). Concerning the timing of surgery in the setting of meningitis and an infected meningocele, only 8 publications give adequate information. The timing of surgery ranged between day 1 and day 22 after hospital admission (and starting of antibiotic thera-py), with a mean of 16 days and a median of 21 days before surgical correction of the fistula and the meningocele. For two patients, it was decided to plan the surgical repair in elective setting, after initial discharge home from the hospital when the meningitis was treated sufficiently (this included one patient with aseptic meningitis due to a dermoid tumor).
Discussion
In the case presented, it was decided to first perform an ileostomy, to be completely sure of a stop of leaking of intes-tinal microbial flora through the enteral fistula(s). This was followed by an elective posterior neurosurgical exploration. This seems to be an effective strategy. In the literature, we noticed the preference for a posterior approach in the situation of severe inflammation. This is in line with our experience in the case here described. In a posterior approach, important neurological structures are directly visualized and can be spared. A detethering procedure, for example cutting of the filum terminale, or removal of an intradural tumor (e.g., dermoid or teratoma), is conveniently possible during the same approach. A possible disadvantage is the suboptimal view on the rectum and the retroperitoneal/enteral anatomy. This is especially true in a situation of severe inflammation. In our experience, successful surgical closing of an enteral defect in the setting of active inflammation is not possible. Hence, a temporary ileostomy is an indispensable and elegant solution to overcome this problem. It is known from a significant body of literature, mainly from the GE-surgical field, that rectal fistula will close spontaneously if there is no passage of fecal material for some time because of an ileostomy [24].
The current available literature on the topic of meningitis, due to an inflamed anterior meningocele caused by an enterothecal fistula, is limited to case reports only. Therefore, evidence-based guidelines/protocols for Currarino patients developing meningitis due to an enterothecal fistula cannot be formulated. There is no high-quality literature on the natural history of Currarino patients with an anterior sacral
meningocele to justify the prophylactic surgical correction of an anterior meningocele in all Currarino patients, solely aiming to prevent meningitis. Numbers needed to treat (NNT) to prevent one case of meningitis are unavailable. If the patient experiences other clinical symptoms that could be alleviated by surgical correction of the anterior sacral meningocele, this would of course justify a more aggressive surgical strategy towards closure and resection of the meningocele.
Conclusion
The present case and review of the literature illustrates that in patients with Currarino syndrome potentially lethal meningitis can occur due to the development of an enterothecal fistula. In our own limited experience and supported by the literature, the construction of an ileostomy in the acute stage seems a safe and rational start of the (surgical) treatment, together with administration of high-dose, broad-spectrum intravenous an-tibiotics. Subsequent surgical treatment of the enterothecal fistula and infected anterior sacral meningocele can be per-formed in an elective procedure, as soon as the patient has recovered from the most severe symptoms of the meningitis. A posterior approach is most often described in the literature, and seems to offer the best anatomical overview in the setting of (recent) inflammation. There is currently no supportive ev-idence for early prophylactic surgery in Currarino patients with an anterior sacral meningocele to prevent meningitis.
Compliance with ethical standards
Conflict of interest On behalf of all authors, the corresponding author states that there is no conflict of interest.
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References
1. Al Qahtani HM, Aljoqiman KS, Arabi H, Al Shaalan H, Singh S (2016) Fatal meningitis in a 14-month-old with Currarino triad. Case reports in radiology 2016:1–5.https://doi.org/10.1155/2016/ 1346895
2. Antuna-ramos A, Garcia-Fructoso G, Alamar-Abril M, Guillén-Quesada A, Costa-Clara y JM (2011) Meningocele sacro anterior oculto. Neurocirugia 22:342–346
3. Bal S, Kurtulmus¸ K, Koçyig H, Gürgan (2004) A case with cauda Equina syndrome due to bacterial meningitis of anterior sacral Meningocele. Spine 29(14):E298–E299
4. Bergeron E, Roux A, Demers J, Vanier LE, Moore L (2010) A 40-year-old woman with cauda equina syndrome caused by rectothecal fistula arising from an anterior sacral meningocele: case report. Neurosurgery 67:E1464–E1468
5. Bhatia S, Tullu MS, Date NB, Muzumdar D, Muranjan M, Lahiri KR (2010) Anterior sacral pyocele with meningitis: a rare pre-sentation of occult spinal dysraphism with congenital dermal sinus. J Child Neurol 25(11):1393–1397. https://doi.org/10. 1177/0883073810365010
6. Blond MH, Borderon JC, Despert F, Laugier J, Maheut J, Robert M, Sirinlli D (1991) Anterior sacral meningocele associated with men-ingitis. Paediatr Infect Dis J 10(10):783–784
7. Braczynski AK, Brockmann MA, Scholz T, Bach JP, Schulz JB, Tauber SC (2017) Anterior sacral meningocele infected with fusobacterium in a patient with recently diagnosed colorectal carci-noma - a case report. BMC Neurol 17(1):212.https://doi.org/10. 1186/s12883-017-0992-1
8. Calleja Aguayo E, Estors Sastre B, Bragagnini Rodriguez P, Fustero de Miguel D, Gonzalez Martinez-Pardo N, Elias Pollina J (2012) Triada de Currarino: sus diferentes formas de presentacion. Cir Pediatr 25:155–158
9. Chou IC, Mak SC, Lin TP, Chi CS, Pen HC (2002) Bacterial men-ingitis of an infant with Currarino triad. Acta Paediatr Taiwan 43(5): 288–290
10. Currarino G, Coln D, Votteler T (1981) Triad of anorectal, sacral and presacral anomalies. AJR 137:395–398
11. Emans PJ, Kootstra G, Marcelis CLM, Beuls EAM, van Heurn LWE (2005) The Currarino triad: the variable expression. J Pediatr Surg 40:1238–1242.https://doi.org/10.1016/j.jpedsurg. 2005.05.004
12. Fitouri Z, Ben Slima S, Matoussi N, Aloui N, Bellagha I, Kechrid A, Ben Becher S (2007) Syndrome de Currarino cause rare de méningites purulentes récidivantes. Med Mal Infect 37:S264– S267.https://doi.org/10.1016/j.medmal.2006.10.008
13. Fitzpatrick MO, Taylor WAS (1999) Anterior sacral meningocele associated with a rectal fistula. J Neurosurg (Spine1) 91:124–127 14. Fiumara F, Foti N, Gambardella A, Mazzeo A, Oliveri RL, Pagano
VD, Quattrone A (1989) Purulent meningitis due to spontaneous anterior sacral meningocele perforation. Case report. Ital J Neurol Sci 10:211–213.https://doi.org/10.1007/BF02333622
15. Fleury J, Picherot G, Cretolle C, Podevin G, David A, Caillon J, Roze JC, Gras-le Guen C (2007) Currarino syndrome as an etiology of a neonatal Escherichia coli meningitis. J Perinatol 27:589–591. https://doi.org/10.1038/sj.jp.7211783
16. Funayama CAR, de Turcato MF, Moura-Ribeiro R, Rocha GM, Pina Neto JM, Moura-Ribeiro MVL (1995) Recurrent meningitis in a case of congenital anterior sacral meningocele and agenesis of sacral and coccygeal vertebrae. Arq Neuropsiquiatr 53(4):799–801. https://doi.org/10.1590/S0004-282X1995000500016
17. Ganeshalingham A, Buckley D, Shaw I, Freeman JT, Wilson F, Best E (2014) Bacteroides fragilis concealed in an infant with Escherichia coli meningitis. J Paediatr Child Health 50:78–80. https://doi.org/10.1111/jpc.12394
18. Guerin JM, Leibingerl F, Raskine L, Ekheriad JM (2000) Polymicrobial meningitis revealing an anterior sacral meningocele in a 23-year-old woman. J Inf Secur 40(2):195–197.https://doi.org/ 10.1053/jinf.1999.0617
19. Haga Y, Cho H, Shinoda S, Masuzawa T (2003) Recurrent menin-gitis associated with complete Currarino triad in an adult-case re-port. Neurol Med Chir (Tokyo) 43:505–508
20. Hatano A, Akiyama K, Nagayama M, Takagi S (2006) Case of Marfan’s syndrome with anterior sacral meningocele along with recurring bacterial meningitis. Rinsho Shinkeigaku 46(9):658–660 21. Kansal R, Mahore A, Dange N, Kukreja S (2012) Epidermoid cyst inside anterior sacral meningocele in an adult patient of Currarino
syndrome manifesting with meningitis. Turk Neurosurg 22(5):659– 661.https://doi.org/10.5137/1019-5149.JTN.3985-10.1
22. Kiefer AS, Gupta P, Kirmani S, Schwartz K, Henry N, Fischer PR (2009) Treatment-resistant meningitis leading to the diagnosis of currarino syndrome: a case report. Pediatr Infect Dis J 28:547– 549.https://doi.org/10.1097/INF.0b013e3181945875
23. Koksal A, Canyigit M, Kara T, Ulus A, Gokbayir H, Sarisahin M (2011) Unusual presentation of an anterior sacral meningocele: magnetic resonance imaging, multidetector computed tomography, and fistulography findings of bacterial meningitis secondary to a rectothecal fistula. Jpn J Radiol 29:528–531.https://doi.org/10. 1007/s11604-011-0582-x
24. Martin ST, Vogel JD (2012) Intestinal stomas: indications, manage-ment, and complications. Adv Surg 46:19–49.https://doi.org/10. 1016/j.yasu.2012.04.005
25. Massimi L, Calisti A, Koutzoglou M, Di Rocco C (2003) Giant ante-rior sacral meningocele and posteante-rior sagittal approach. Childs Nerv Syst 19:722–728.https://doi.org/10.1007/s00381-003-0814-1 26. Miletic D, Poljak I, Eskinja N, Valkovic P, Sestan B,
Troselj-Vukic B (2008) Giant anterior sacral meningocele presenting as bacterial meningitis in a previously healthy adult. Orthopedics 31(2):182–184
27. Monseaux C, Coulon J-M, Etosse A, Nicomette-Bardel J, Regrain E, Peruzzi P, Boyer F-C (2013) Paraplegia after meningoencepha-litis complicated by an arachnoisis in a patient with a Currarino syndrome. About a case. Ann Phys Rehabil Med 56S:e82–e83. https://doi.org/10.1016/j.rehab.2013.07.451
28. Morgenstern IA, Bach FA, Martinez S, Martin-Nalda A, Vazquez Mendez E, Pumarola Segura F, Soler-Palacin P (2015) Recurrent meningitis due to anatomical defects: the bacteria indicates its ori-gin. An Pediatr 82(6):388–396.https://doi.org/10.1016/j.anpedi. 2014.09.008
29. O’Riordain DS, O’Connell PR, Kirwan WO (1991) Hereditary sa-cral agenesis with presasa-cral mass and anorectal stenosis: the Currarino triad. Br J Surg 78:536–538
30. Page Y, Thevenet D, Bertrand M, Chatard N, Gery P, Barral FG, Tardy B, Bertrand JC (1990) Polymicrobial meningitis revealing a meningorectal fistula of an anterior sacral meningocele. Clin Intensive Care 1(1):29–31
31. Patnaik S, Mohapatra M, Satpathy DK, Das S, Mohanty AK (2013) Anterior sacral meningocele with spinal epidural abscess: a case report with review of literature. J Pediatr Neurol 11:141–145 32. Paul P, Tiwari M, Kumar H (2015) Anterior sacral meningocele
presenting with purulent rectal discharge and altered mental status. Neurol Clin Pract 5:89–90. https://doi.org/10.1212/CPJ. 0000000000000102
33. Phillips JT, Brown SR, Mitchell P, Shorthouse AJ (2006) Anaerobic meningitis secondary to a rectothecal fistula aris-ing from an anterior sacral menaris-ingocele: report of a case and review of the literature. Dis Colon Rectum 49:1633– 1635. https://doi.org/10.1007/s10350-006-0646-7
34. Quigley MR, Schinco F, Brown JT (1984) Anterior sacral meningocele with an unusual presentation. J Neurosurg 61:790–792 35. Raczynski RA, Fisher RG, Sass LA (2010) Unusual cases of
men-ingitis as a clue to the diagnosis of Currarino syndrome. Pediatr Infect Dis J 29(1):90
36. Sánchez AA, Iglesias CD, López CD, Cecilia DM, Gomez JA, Barbadillo JG, Pena SR (2008) Rectothecal fistula secondary to an anterior sacral meningocele. J Neurosurg Spine 8:487–489. https://doi.org/10.3171/SPI/2008/8/5/487
37. Schijman E, Rugilo C, Seviever G, Menendez R (2005) Anterior sacral meningocele. Child Nerv Syst 21:91–93.https://doi.org/10. 1007/s00381-004-1086-0
38. Simon LG, Constantinescu A, Maftei AG, Popescu CD (2010) A particular evolution of Currarino syndrome. Eur J Neurol 17(Suppl. 3):319
39. Synowitz HJ, Lehmann R (1988) Meningitis as a complica-tion of anterior-sacral meningocele. Zentralbl Neurochir 49(1):15–21
40. Tamayo JA, Arraez MA, Villegas I, Ruiz J, Rodriguez E, Fernandez O (1999) Partial Currarino syndrome in a
non-pediatric patient. A rare cause of bacterial meningitis. Neurologia 14(9):460–462
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