Top

Child's Nervous System

Published in:

01-08-2007 | Case Report

Chiari I malformation and intra-cranial hypertension:a case-based review

Authors: Senta Kurschel, Richard Maier, Verena Gellner, Hans Georg Eder

Published in: Child's Nervous System | Issue 8/2007

Abstract

Objective

To present clinical and morphological findings before and after surgery in a child with Chairi I malformation (CMI) and intra-cranial hypertension (IH). The literature is reviewed and pathophysiologic factors are discussed.

Clinical presentation

A 13-year-old obese boy with a 3-week history of headaches, neck pain, torticollis and progressive visual deterioration was admitted. Bi-lateral chronic papilloedema and decrease in visual acuity were found in the presence of a previously diagnosed CMI.

Intervention and follow-up

Intra-cranial pressure monitoring demonstrating increased pressure levels was followed by a sub-occipital decompression, C1 laminectomy and duroplasty. Post-operatively, the boy improved markedly, the 6 months follow-up opthalmological examination demonstrated resolution of papilloedema, but consecutive bi-lateral optic nerve atrophy.

Conclusion

IH with progressive visual deterioration represents one of the varying clinical presentations of CMI and may be classified as a secondary form of idiopathic IH. Neuro-ophthalmological examination in all patients with CMI is recommended to identify the real incidence of this presentation. Altered CSF dynamics, venous hypertension and obesity as co-factors may be causative pathophysiologic factors.

Alperin N, Kulkarni K, Roitberg B, Loth F, Pandian NK, Mafee MF, Foroohar M, Lichtor T (2001) Analysis of magnetic resonance imaging-based blood and cerebrospinal fluid flow measurement in patients with Chiari I malformation: a system approach. Neurosurg Focus 11:1–10, Article 6

Badie B, Mendoza D, Batzdorf U (1995) Posterior fossa volume and response to suboccipital decompression in patients with Chiari I malformation. Neurosurgery 37:214–218PubMedCrossRef

Banik R, Lin D, Miller NR (2006) Prevalence of Chiari I malformation and cerebellar ectopia in patients with pseudotumor cerebri. J Neurol Sci 247:71–75PubMedCrossRef

Bateman GA (2006) Association between arterial inflow and venous outflow in idiopathic and secondary intracranial hypertension. J Clin Neurosci 13:550–557PubMedCrossRef

Bejjani GK, Cockerham KP, Rothfus WE, Maroon JC, Maddock M (2003) Treatment of failed adult Chiari malformation decompression with CSF drainage: observations in six patients. Acta Neurochir (Wien) 145:107–116CrossRef

Bejjani GK (2003) Association of the adult Chiari malformation and idiopathic intracranial hypertension: more than a coincidence. Med Hypotheses 60:859–863PubMedCrossRef

Brazis PW, Lee AG (1998) Elevated intracranial pressure and pseudotumor cerebri. Curr Opin Ophthalmol 9:27–32PubMed

Choudhari KA, Cooke C, Hong Tan M, Gray WJ (2002) Papilloedema as the sole presenting feature of Chiari I malformation. Br J Neurosurg 16:398–400PubMedCrossRef

Chumas PD, Armstrong DC, Drake JM, Kulkarni AV, Hoffman HJ, Humphreys RP, Rutka JT, Hendrick EB (1993) Tonsillar herniation: the rule rather than the exception after lumboperitoneal shunting in the pediatric population. J Neurosurg 78:568–573PubMedCrossRef

10.

Di Rocco C, Velardi F (2003) Acquired Chiari type I malformation managed by supratentorial cranial enlargement. Childs Nerv Syst 19:800–807PubMedCrossRef

11.

Dolar MT, Haughton VM, Iskandar BJ, Quigley M (2004) Effect of craniocervical decompression on peak CSF velocities in symptomatic patients with Chiari I malformation. Am J Neuroradiol 25:142–145PubMed

12.

Dure LS, Percy AK, Cheek WR, Laurent JP (1989) Chiari type I malformation in children. J Pediatr 115:573–576PubMedCrossRef

13.

Fagan LH, Ferguson S, Yassari R, Frim DM (2006) The Chiari pseudotumor cerebri syndrome: symptom recurrence after decompressive surgery for Chiari malformation type I. Pediatr Neurosurg 42:14–19PubMedCrossRef

14.

Greitz D (1993) Cerebrospinal fluid circulation and associated intracranial dynamics. A radiologic investigation using MR imaging and radionuclide cisternography. Acta Radiol Suppl 386:1–23PubMed

15.

Iskandar BJ, Quigley M, Haughton VM (2004) Foramen magnum cerebrospinal fluid flow characteristics in children with Chiari I malformation before and after craniocervical decompression. J Neurosurg 101(2 Suppl):169–178PubMed

16.

Johnston I, Jacobson E, Besser M (1998) The acquired Chiari malformation and syringomyelia following spinal CSF drainage: a study of incidence and management. Acta Neurochir (Wien) 140:417–428CrossRef

17.

Lancione RR Jr, Kosmorsky GS (2001) When does low mean high? Isolated cerebral ventricular increased intracranial pressure in a patient with a Chiari I malformation. J Neuroophthalmol 21:118–120PubMed

18.

Menick BJ (2001) Phase-contrast magnetic resonance imaging of cerebrospinal fluid flow in the evaluation of patients with Chiari I malformation. Neurosurg Focus 11:1–4, Article 5

19.

Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C, Speer MC (1999) Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery 44:1005–1017PubMedCrossRef

20.

Nagib MG (1994) An approach to symptomatic children (ages 4–14 years) with Chiari type I malformation. Pediatr Neurosurg 21:31–35PubMedCrossRef

21.

Padmanabhan R, Crompton D, Burn D, Birchall D (2005) Acquired Chiari I malformation and syringomyelia following lumboperitoneal shunting for pseudotumor cerebri. J Neurol Neurosurg Psychiatry 76:298PubMedCrossRef

22.

Pardo Munoz A, Reche Sainz JA, Sanz Lopez A, Diaz Orro B, Lorenzo G, Sanmillan J (2002) A case of papilledema and Arnold–Chiari type I malformation. Arch Soc Esp Oftalmol 77:449–453PubMed

23.

Payner TD, Prenger E, Berger TS, Crone KR (1994) Acquired Chiari malformations: incidence, diagnosis, and management. Neurosurgery 34:429–434PubMedCrossRef

24.

Poca MA, Sahuquillo J, Topczewski T, Lastra R, Font ML, Corral E (2006) posture-induced changes in intracranial pressure: a comparative study in patients with and without a cerebrospinal fluid block at the craniovertebral junction. Neurosurgery 58:899–906PubMedCrossRef

25.

Sgouros S, Kountouri M, Natarajan K (2006) Posterior fossa volume in children with Chiari malformation type I. J Neurosurg 105(2 Suppl):101–106PubMed

26.

Sgouros S, Willshaw H (2006) Intracranial hypertension associated with Chiari I malformation. Childs Nerv Syst 22:1053CrossRef

27.

Sinclair N, Assaad N, Johnston I (2002) Pseudotumor cerebri occurring in association with the Chiari malformation. J Clin Neurosci 9:99–101PubMedCrossRef

28.

Skau M, Brennum J, Gjerris F, Jensen R (2005) What is new about idiopathic intracranial hypertension? An updated review of mechanism and treatment. Cephalalgia 26:384–399CrossRef

29.

Speer MC, George TM, Enterline DS, Franklin A, Wolpert CM, Milhorat TH (2000) A genetic hypothesis for Chiari I malformation with or without syringomyelia. Neurosurg Focus 15:E12

30.

Sullivan LP, Stears JC, Ringel SP (1988) Resolution of syringomyelia and Chiari I malformation by ventriculoatrial shunting in a patient with pseudotumor cerebri and a lumboperitoneal shunt. Neurosurgery 22:744–747PubMedCrossRef

31.

Surgerman HJ, DeMaria EJ, Felton WL 3rd, Nakatsuka M, Sismanis A (1997) Increased intra-abdominal pressure and cardiac filling pressures in obesity-associated pseudotumor cerebri. Neurology 49:507–511

32.

Surgerman HJ (2001) Effects of increased intra-abdominal pressure in severe obesity. Surg Clin North Am 81:1063–1075CrossRef

33.

Tubbs RS, Elton S, Grabb P, Dockery SE, Bartolucci AA, Oakes WJ (2001) Analysis of the posterior fossa in children with the Chiari 0 malformation. Neurosurgery 48:1050–1055PubMedCrossRef

34.

Vaphiades MS, Eggenberger ER, Miller NR, Frohman L, Krisht A (2002) Resolution of papilledema after neurosurgical decompression for primary Chiari I malformation. Am J Ophthalmol 133:673–678PubMedCrossRef

35.

Ventureyra ECG, Aziz HA, Vassilyadi M (2003) The role of cine flow MRI in children with Chiari malformation. Childs Nerv Syst 19:109–113PubMed

36.

Vrabec TR, Sergott RC, Savino PJ, Bosley TM (1989) Intermittent obstructive hydrocephalus in the Arnold–Chiari malformation. Ann Neurol 26:401–404PubMedCrossRef

37.

Zatorska B, Herba E, Pojda-Wilczek D, Jedrzejewski W, Pojda SM (2004) The intracranial hypertension in the course of Arnold-Chiari malformation. Klin Oczna 106:657–660PubMed

Title: Chiari I malformation and intra-cranial hypertension:a case-based review
Authors: Senta Kurschel
Richard Maier
Verena Gellner
Hans Georg Eder
Publication date: 01-08-2007
Publisher: Springer-Verlag
Published in: Child's Nervous System / Issue 8/2007
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-007-0355-0

2024 ASCO Annual Meeting

Springer Medicine

Chiari I malformation and intra-cranial hypertension:a case-based review

Abstract

Objective

Clinical presentation

Intervention and follow-up

Conclusion

2024 ASCO Annual Meeting

Springer Medicine

Abstract

Objective

Clinical presentation

Intervention and follow-up

Conclusion

Please log in to get access to this content

Other articles of this Issue 8/2007

Corpus callosotomy using conformal stereotactic radiosurgery

Endoscopic observation of pathophysiology of ventricular diverticulum

Posterior fossa tumors in children: how long does it take to establish the diagnosis?

Progressive myelopathy due to meningeal thickening in shunted patients: description of a novel entity and the role of surgery

Severe head injury in early infancy: analysis of causes and possible predictive factors for outcome

Management of vestibular schwannomas in young patients—comparison of clinical features and outcome with adult patients