Abstract
Purpose
This paper reviews the altered cerebrospinal fluid dynamics that can be associated with paediatric Chiari I malformation and we present our own institutional experience.
Methods
We conducted a thorough review of the literature and retrospectively analyzed all cases of operatively managed paediatric Chiari 1 malformation at our institution between February 2006 and February 2019.
Results
Acquired Chiari malformation (ACM) can radiologically mimic Chiari I and has been associated with both intracranial hypotension (either secondary to lumboperitoneal shunting or spontaneous CSF hypotension) and idiopathic intracranial hypertension (IIH). At our institution, 61 paediatric cases (range, 2–15 years) underwent foramen magnum decompression (FMD) for Chiari I malformation. Whilst 80% (50/61) of cases underwent FMD with no preceding or post-operative problems of CSF dynamics, 8% (5/61) of cases had hydrocephalus at initial presentation requiring CSF diversion followed by FMD for persistent Chiari, and 10% (6/61) developed hydrocephalus following FMD and required long-term CSF diversion.
Conclusions
In paediatric ACM, the management of intracranial hypotension involves thorough radiological assessment and inclusion/adjustment of a valve in the case of lumboperitoneal shunting or epidural blood patch or interventional techniques in the case of spontaneous CSF leak. Thereby, unwarranted posterior fossa decompression surgery is avoided. In the case of IIH and Chiari I malformation, children who have recurrent symptoms despite adequate posterior fossa decompression surgery (failed Chiari), there is a strong role for intracranial pressure monitoring as raised intracranial pressure may indicate long-term CSF diversion.
Similar content being viewed by others
References
Elster AD, Chen MY (1992) Chiari I malformations: clinical and radiologic reappraisal. Radiology 183:347–353
Atkinson JL, Weinshenker BG, Miller GM, Piepgras DG, Mokri B (1998) Acquired Chiari I malformation secondary to spontaneous spinal cerebrospinal fluid leakage and chronic intracranial hypotension syndrome in seven cases. J Neurosurg 88:237–242
Banik R, Lin D, Miller NR (2006) Prevalence of Chiari I malformation and cerebellar ectopia in patients with pseudotumor cerebri. J Neurol Sci 247:71–75
Ferguson AH (1898) Intraperitoneal diversion of the cerebrospinal fluid in cases of hydrocephalus. N Y Med 67:902
Jackson IJ, Snodgrass SR (1955) Peritoneal shunts in the treatment of hydrocephalus and increased intracranial pressure; a 4-year survey of 62 patients. J Neurosurg 12:216–222
Spetzler RF, Wilson CB, Grollmus JM (1975) Percutaneous lumboperitoneal shunt. Technical note. J Neurosurg 43:770–773
Kestle J, Drake J, Milner R, Sainte-Rose C, Cinalli G, Boop F, Piatt J, Haines S, Schiff S, Cochrane D, Steinbok P, MacNeil N (2000) Long-term follow-up data from the Shunt Design Trial. Pediatr Neurosurg 33:230–236
Jea A, Al-Otibi M, Rutka JT, Drake JM, Dirks PB, Kulkarni AV, Taylor MD, Humphreys RP (2007) The history of neurosurgery at the Hospital for Sick Children in Toronto. Neurosurgery 61:612–624 discussion 624–625
Mukherjee S., Chumas P.D. (2017) In-depth view: how to perform a lumboperitoneal CSF shunt. In: Di Rocco C., Pang D., Rutka J. (eds) Textbook of pediatric neurosurgery. Springer, Cham
Aoki N (1990) Lumboperitoneal shunt: clinical applications, complications, and comparison with ventriculoperitoneal shunt. Neurosurgery 26:998–1003 discussion 1003–1004
Chumas PD, Kulkarni AV, Drake JM, Hoffman HJ, Humphreys RP, Rutka JT (1993a) Lumboperitoneal shunting: a retrospective study in the pediatric population. Neurosurgery 32:376–383
Chumas PD, Armstrong DC, Drake JM (1993b) Tonsillar herniation: the rule rather than the exception after lumboperitoneal shunting in pediatric population. J Neurosurg 78:568–573
Kulkarni AV, Chumas PD, Drake JM, Armstrong DC (1999) The reliability of the “absent cistern sign” in assessing LP shunt function. Can J Neurol Sci 26:40–43
Yadav YR, Pande S, Raina VK, Singh M (2004) Lumboperitoneal shunts: review of 409 cases. Neurol India 52:188–190
Payner TD, Prenger E, Berger TS, Crone KR (1994) Acquired Chiari malformations: incidence, diagnosis and management. Neurosurgery 34:429–434
Rekate HL, Wallace D (2003) Lumboperitoneal shunts in children. Pediatr Neurosurg 38:41–46
Duthel R, Nuti C, Motuo-Fotso MJ, Beauchesne P, Brunon J (1996) Complications of lumboperitoneal shunts: a retrospective study of a series of 195 patients (214 procedures). Neurochirurgie 42:83–89 discussion 89–90
Wang VY, Barbaro NM, Lawton MT, Pitts L, Kunwar S, Parsa AT, Gupta N, McDermott MW (2007) Complications of lumboperitoneal shunts. Neurosurgery 60:1045–1048 discussion
Riffaud L, Moughty C, Henaux PL, Haegelen C, Morandi X (2008) Acquired Chiari I malformation and syringomyelia after valveless lumboperitoneal shunt in infancy. Pediatr Neurosurg 44:229–233
Nadkarni TD, Rekate HL, Wallace D (2008) Concurrent use of a lumboperitoneal shunt with programmable valve and ventricular access device in the treatment of pseudotumor cerebri: review of 40 cases. J Neurosurg Pediatr 2:19–24
Johnson MP, Sutton LN, Rintoul N, Crombleholme TM, Flake AW, Howell LJ, Hedrick HL, Wilson RD, Adzick NS (2003) Fetal myelomeningocoele repair: short-term clinical outcomes. Am J Obstet Gynecol 189:482–487
Johnston MP, Gerdes M, Rintoul N et al (2006) Maternal-fetal surgery for myelomeningocoele: neurodevelopmental outcomes at 2 years of age. Am J Obstet Gynecol 194:1145–1152
Danzer E, Finkel R, Gerdes M, Schwartz EMS, Rintoul N, Adzick N, Johnson M (2010) The relationship of seizure activity and chronic epilepsy in early infancy and short-term neurodevelopmental outcome following fetal myelomeningocoele closure. Neuropaediatrics 41:140–143
Danzer E, Gerdes M, Bebbington MW, Sutton LN, Melchionni J, Adzick NS, Wilson RD, Johnson MP (2009) Lower extremity neuromotor function and short-term ambulatory potential following in utero myelomeningocoele surgery. Fetal Diagn Ther 25:47–53
Adzick SN, Thom EA, Spong CY, Brock JW, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL, MOMS Investiagtors (2011) A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 364:993–1004
Schievink WI (2000) Spontaneous spinal cerebrospinal fluid leaks: a review. Neurosurg Focus 9:e8
Chung SJ, Kim JS, Lee MC (2000) Syndrome of cerebral spinal fluid hypovolemia: clinical and imaging features and outcome. Neurology 55:1321–1327
Schievink WI, Maya MM, Louy C, Moser FG, Sloninsky L (2013) Spontaneous intracranial hypotension in childhood and adolescence. J Pediatr 163:504–510
Mokri B (2001) Spontaneous intracranial hypotension. Curr Pain Headache Rep 5:284–291
Ferrante E, Citterio A, Savino A, Santalucia P (2003) Postural headache in patient with Marfan’s syndrome. Cephalalgia 23:552–555
Pannullo SC, Reich JB, Krol G, Deck MD, Posner JB (1993) MRI changes in intracranial hypotension. Neurology 43:919–926
Schönberger J, Möhlenbruch M, Seitz A, Bußmann C, Bächli H, Kölker S (2017) Chiari-like displacement due to spontaneous intracranial hypotension in an adolescent: successful treatment by epidural blood patch. Eur J Paediatr Neurol 21:678–681
Schrijver I, Schievink WI, Godfrey M, Meyer FB, Francke U (2002) Spontaneous spinal cerebrospinal fluid leaks and minor skeletal features of Marfan syndrome: a microfibrillopathy. J Neurosurg 96:483e9
Mokri B, Maher CO, Sencakova D (2002) Spontaneous CSF leaks: underlying disorder of connective tissue. Neurology 58:814–816
Mokri B, Hunter SF, Atkinson JL, Piepgras DG (1998) Orthostatic headaches caused by CSF leak but with normal CSF pressures. Neurology 51:786–790
Schievink WI, Dodick DW, Mokri B, Silberstein S, Bousser MG, Goadsby PJ (2011) Diagnostic criteria for headache due to spontaneous intracranial hypotension: a perspective. Headache 51:1442–1444
Schievink WI, Maya MM, Louy C, Moser FG, Tourje J (2008) Diagnostic criteria for spontaneous spinal CSF leaks and intracranial hypotension. AJNR Am J Neuroradiol 29:853–856
Puget S, Kondageski C, Wray A, Boddaert N, Roujeau T, Di Rocco F, Zerah M, Sainte-Rose C (2007) Chiari-like tonsillar herniation associated with intracranial hypotension in Marfan syndrome. Case report. J Neurosurg 106:48–52
Bladen J, Moosajee M, Renowden S, Carter M, Sainsbury C, Jardine P (2007) Neurological pictures. Spontaneous intracranial hypotension in adolescence. J Neurol Neurosurg Psychiatry 78:167
Önal H, Ersen A, Gemici H, Adal E, Güler S, Sander S, Albayram S (2018) Acquired Chiari I malformation secondary to spontaneous intracranial hypotension syndrome and persistent hypoglycemia: a case report. J Clin Res Pediatr Endocrinol 10:391–394
Mokri B (2015) Spontaneous intracranial hypotension. Continuum (Minneap Minn) 21:1086–1108
Wilmott RW (2013) Pediatric spontaneous intracranial hypotension. J Pediatr 163:311
Cho KI, Moon HS, Jeon HJ, Park K, Kong DS (2011) Spontaneous intracranial hypotension: efficacy of radiologic targeting vs blind blood patch. Neurology 76:1139–1144
Khurana RK (1991) Headache spectrum in Arnold-Chiari malformation. Headache 31:151–155
Pillay PK, Awad IA, Little JR, Hahn JF (1991) Symptomatic Chiari malformation in adults: a new classification based on magnetic resonance imaging with clinical and prognostic significance. Neurosurgery 28:639–645
Caldarelli M, Di Rocco C (2004) Diagnosis of Chiari I malformation and related syringomyelia: radiological and neurophysiological studies. Childs Nerv Syst 20:332–335
Fishman RA, Dillon WP (1993) Dural enhancement and cerebral displacement secondary to intracranial hypotension. Neurology 43:609–611
Marcelis J, Silberstein SD (1990) Spontaneous low cerebrospinal fluid pressure headache. Headache 30:192–196
Rando TA, Fishman RA (1992) Spontaneous intracranial hypotension: report of two cases and review of the literature. Neurology 42:481–487
Fernandez E (1990) Headaches associated with low spinal fluid pressure. Headache 30:122–128
Johnston I, Hawke S, Halmagyi M, Teo C (1991) The pseudotumor syndrome. Disorders of cerebrospinal fluid circulation causing intracranial hypertension without ventriculomegaly. Arch Neurol 48:740–747
Aiken AH, Hoots JA, Saindaine AM, Hudgins PA (2012) Incidence of cerebellar tonsillar ectopia in idiopathic intracranial hypertension; a mimic of the Chiari I malformation. AJNR 33:1901–1906
Bejjani GK (2003) Association of the adult Chiari malformation and Idiopathic Intracranial hypertension: more than a coincidence. Med Hypotheses 60:859–863
Bejjani GK, Cockerham KP, Rothfus WE, Marroon JC, Maddock M (2003) Treatment of failed adult Chiari malformation decompression with CSF drainage: observations in 10 patients. Acta Neurochir 145:107–116
Fagan LH, Ferguson S, Yassari R, Frim DM (2006) The Chiari pseudotumor syndrome: symptom recurrence after decompressive surgery for Chiari malformation type I. Pediatr Neurosurg 42:14–19
Author information
Authors and Affiliations
Ethics declarations
Conflict of interest
On behalf of all authors, the corresponding author states that there is no conflict of interest.
Additional information
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Mukherjee, S., Kalra, N., Warren, D. et al. Chiari I malformation and altered cerebrospinal fluid dynamics—the highs and the lows. Childs Nerv Syst 35, 1711–1717 (2019). https://doi.org/10.1007/s00381-019-04233-w
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-019-04233-w