Surgical experience in 130 pediatric patients with Chiari I malformations

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Object. The aim of this study was to present the long-term findings of a surgical series of pediatric patients with Chiari I malformations.

Methods. One hundred thirty symptomatic pediatric patients with Chiari I malformations underwent posterior fossa decompression. The age in this group of patients ranged from 2 months to 20 years (mean 11 years). The length of the hospital stay extended from 2 to 7 days (mean 2.7 days), and follow up was from 3 months to 15 years (mean 4.2 years). Patients most often presented with headache/neck pain (38%) and scoliosis (18%). Examples of associated diagnoses included neurofibromatosis Type 1 (5.5%), hydrocephalus (11%), idiopathic growth hormone deficiency (5.5%), and Klippel—Feil anomaly (5%). Syringes were present in 58% of patients. Seventeen percent of patients had caudal displacement of the brainstem and fourth ventricle. Postoperative relief of preoperative pathologies was experienced in 83% of patients. Of the most common presenting symptoms—headache/neck pain and scoliosis—12 and 17%, respectively, were not alleviated postoperatively. Complications occurred in 2.3% of this group and included the development of acute hydrocephalus postoperatively and severe life-threatening signs of brainstem compression that necessitated a transoral odontoidectomy. Nine patients have had to undergo repeated operations for continued symptoms or persistent large syringes. During surgery 10 patients (7.7%) were found to have arachnoid veils occluding the fourth ventricular outlet, and nine of these had syringomyelia. In our experience almost all syringes will stabilize or improve with posterior fossa decompression and duraplasty.

Conclusions. The authors believe this to be the largest reported series of pediatric patients who have undergone posterior fossa decompression for Chiari I malformations.

Article Information

Address reprint requests to: R. Shane Tubbs, Ph.D., Pediatric Neurosurgery, 1600 Seventh Avenue South Acc 400, Birmingham, Alabama 35233. email: richard.tubbs@ccc.uab.edu.

© AANS, except where prohibited by US copyright law.

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References

1.

Adelman SDinner DSGoren Het al: Obstructive sleep apnea in association with posterior fossa neurologic disease. Arch Neurol 41:5095101984Adelman S Dinner DS Goren H et al: Obstructive sleep apnea in association with posterior fossa neurologic disease. Arch Neurol 41:509–510 1984

2.

Anderson NEWilloughby EWWrightson P: The natural history and the influence of surgical treatment in syringomyelia. Acta Neurol Scand 71:4724791985Anderson NE Willoughby EW Wrightson P: The natural history and the influence of surgical treatment in syringomyelia. Acta Neurol Scand 71:472–479 1985

3.

Aronson DDKahn RHCanady Aet al: Instability of the cervical spine after decompression in patients who have Arnold-Chiari malformation. J Bone Joint Surg (Am) 73:8989061991Aronson DD Kahn RH Canady A et al: Instability of the cervical spine after decompression in patients who have Arnold-Chiari malformation. J Bone Joint Surg (Am) 73:898–906 1991

4.

Ball MJDayan AD: Pathogenesis of syringomyelia. Lancet 2:7998011972Ball MJ Dayan AD: Pathogenesis of syringomyelia. Lancet 2:799–801 1972

5.

Ball WS JrCrone KR: Chiari I malformation: from Dr Chiari to MR imaging. Radiology 195:6026041995Ball WS Jr Crone KR: Chiari I malformation: from Dr Chiari to MR imaging. Radiology 195:602–604 1995

6.

Barkovich AJWippold FJSherman JLet al: Significance of cerebellar tonsillar position on MR. AJNR 7:7957991986Barkovich AJ Wippold FJ Sherman JL et al: Significance of cerebellar tonsillar position on MR. AJNR 7:795–799 1986

7.

Bertrand SLDrvaric DMRoberts JM: Scoliosis in syringomyelia. Orthopedics 12:3353371989Bertrand SL Drvaric DM Roberts JM: Scoliosis in syringomyelia. Orthopedics 12:335–337 1989

8.

Bondurant CPOro JJ: Spinal cord injury without radiographic abnormality and Chiari malformation. J Neurosurg 79:8338381993Bondurant CP Oro JJ: Spinal cord injury without radiographic abnormality and Chiari malformation. J Neurosurg 79:833–838 1993

9.

Coria FQuintana FRebollo Met al: Occipital dysplasia and Chiari type I deformity in a family. Clinical and radiological study of three generations. J Neurol Sci 62:1471581983Coria F Quintana F Rebollo M et al: Occipital dysplasia and Chiari type I deformity in a family. Clinical and radiological study of three generations. J Neurol Sci 62:147–158 1983

10.

Dauser RCDiPietro MAVenes JL: Symptomatic Chiari I malformation in childhood: a report of 7 cases. Pediatr Neurosci 14:1841901988Dauser RC DiPietro MA Venes JL: Symptomatic Chiari I malformation in childhood: a report of 7 cases. Pediatr Neurosci 14:184–190 1988

11.

Dure LSPercy AKCheek WRet al: Chiari type I malformation in children. J Pediatr 115:5735761989Dure LS Percy AK Cheek WR et al: Chiari type I malformation in children. J Pediatr 115:573–576 1989

12.

Dyste GNMenezes AH: Presentation and management of pediatric Chiari malformations without myelodysplasia. Neurosurgery 23:5895971988Dyste GN Menezes AH: Presentation and management of pediatric Chiari malformations without myelodysplasia. Neurosurgery 23:589–597 1988

13.

Dyste GNMenezes AHVanGilder JC: Symptomatic Chiari malformations. An analysis of presentation, management, and long-term outcome. J Neurosurg 71:1591681989Dyste GN Menezes AH VanGilder JC: Symptomatic Chiari malformations. An analysis of presentation management and long-term outcome. J Neurosurg 71:159–168 1989

14.

Ellis RS: Norms for some structural changes in the human cerebellum from birth to old age. J Comp Neurol 32:1331920Ellis RS: Norms for some structural changes in the human cerebellum from birth to old age. J Comp Neurol 32:1–33 1920

15.

Elster ADChen MYM: Chiari I malformation: clinical and radiologic reappraisal. Radiology 183:3473531992Elster AD Chen MYM: Chiari I malformation: clinical and radiologic reappraisal. Radiology 183:347–353 1992

16.

Elton STubbs RSWellons JC III: Acute hydrocephalus following a Chiari I decompression. Pediatr Neurosurg 36:1011042002Elton S Tubbs RS Wellons JC III: Acute hydrocephalus following a Chiari I decompression. Pediatr Neurosurg 36:101–104 2002

17.

Erlich VSnow RHeier L: Confirmation by magnetic resonance imaging of Bell's cruciate paralysis in a young child with Chiari type I malformation and minor head trauma. Neurosurgery 25:1021051989Erlich V Snow R Heier L: Confirmation by magnetic resonance imaging of Bell's cruciate paralysis in a young child with Chiari type I malformation and minor head trauma. Neurosurgery 25:102–105 1989

18.

French BM: Midline fusion defects and defects of formation in Youmans JR (ed): Neurological Surgeryed 2. Philadelphia: WB Saunders1982 pp 12361380French BM: Midline fusion defects and defects of formation in Youmans JR (ed): Neurological Surgery ed 2. Philadelphia: WB Saunders 1982 pp 1236–1380

19.

Gozal DArens ROmlin KJet al: Peripheral chemoreceptor function in children with myelomeningocele and Arnold-Chiari malformation type 2. Chest 108:4254311995Gozal D Arens R Omlin KJ et al: Peripheral chemoreceptor function in children with myelomeningocele and Arnold-Chiari malformation type 2. Chest 108:425–431 1995

20.

Grabb PAMapstone TBOakes WJ: Ventral brain stem compression in pediatric and young adult patients with Chiari I malformations. Neurosurgery 44:5205281999Grabb PA Mapstone TB Oakes WJ: Ventral brain stem compression in pediatric and young adult patients with Chiari I malformations. Neurosurgery 44:520–528 1999

21.

Haines SJBerger M: Current treatment of Chiari malformations type I and II: A survey of the Pediatric Section of the American Association of Neurological Surgeons. Neurosurgery 28:3533571991Haines SJ Berger M: Current treatment of Chiari malformations type I and II: A survey of the Pediatric Section of the American Association of Neurological Surgeons. Neurosurgery 28:353–357 1991

22.

Hoffman HJNeill JCrone KRet al: Hydrosyringomyelia and its management in childhood. Neurosurgery 21:3473511987Hoffman HJ Neill J Crone KR et al: Hydrosyringomyelia and its management in childhood. Neurosurgery 21:347–351 1987

23.

Hunter JVYoul BDMoseley IF: MRI demonstration of midbrain deformity in association with Chiari malformation. Neuroradiology 34:3994011992Hunter JV Youl BD Moseley IF: MRI demonstration of midbrain deformity in association with Chiari malformation. Neuroradiology 34:399–401 1992

24.

Isu TChono YIwasaki Yet al: Scoliosis associated with syringomyelia presenting in children. Childs Nerv Syst 8:971001992Isu T Chono Y Iwasaki Y et al: Scoliosis associated with syringomyelia presenting in children. Childs Nerv Syst 8:97–100 1992

25.

Isu TSasaki HTakamura Het al: Foramen magnum decompression with removal of the outer layer of the dura as treatment for syringomyelia occurring with Chiari I malformation. Neurosurgery 33:8458501993Isu T Sasaki H Takamura H et al: Foramen magnum decompression with removal of the outer layer of the dura as treatment for syringomyelia occurring with Chiari I malformation. Neurosurgery 33:845–850 1993

26.

Koehler PJ: Chiari's description of cerebellar ectopy (1891). With a summary of Cleland's and Arnold's contributions and some early observations on neural-tube defects. J Neurosurg 75:8238261991Koehler PJ: Chiari's description of cerebellar ectopy (1891). With a summary of Cleland's and Arnold's contributions and some early observations on neural-tube defects. J Neurosurg 75:823–826 1991

27.

Kruyff E: Occipital dysplasia in infancy. The early recognition of craniovertebral abnormalities. Radiology 85:5015071965Kruyff E: Occipital dysplasia in infancy. The early recognition of craniovertebral abnormalities. Radiology 85:501–507 1965

28.

Listernick RTomita T: Persistent crying in infancy as a presentation of Chiari type I malformation. J Pediatr 118:5675691991Listernick R Tomita T: Persistent crying in infancy as a presentation of Chiari type I malformation. J Pediatr 118:567–569 1991

29.

Logue JEdwards MR: Syringomyelia and its surgical treatment—an analysis of 75 patients. J Neurol Neurosurg Psychiatry 44:2732841981Logue J Edwards MR: Syringomyelia and its surgical treatment—an analysis of 75 patients. J Neurol Neurosurg Psychiatry 44:273–284 1981

30.

Marin-Padilla MMarin-Padilla TM: Morphogenesis of experimentally induced Arnold-Chiari malformation. J Neurol Sci 50:29551981Marin-Padilla M Marin-Padilla TM: Morphogenesis of experimentally induced Arnold-Chiari malformation. J Neurol Sci 50:29–55 1981

31.

Mikulis DJDiaz OEgglin TKet al: Variance of the position of the cerebellar tonsils with age: preliminary report. Radiology 183:7257281992Mikulis DJ Diaz O Egglin TK et al: Variance of the position of the cerebellar tonsils with age: preliminary report. Radiology 183:725–728 1992

32.

Milhorat THJohnson WDMiller JIet al: Surgical treatment of syringomyelia based on magnetic resonance imaging criteria. Neurosurgery 31:2312451992Milhorat TH Johnson WD Miller JI et al: Surgical treatment of syringomyelia based on magnetic resonance imaging criteria. Neurosurgery 31:231–245 1992

33.

Nagib MG: An approach to symptomatic children (ages 4–14 years) with Chiari type I malformation. Pediatr Neurosurg 21:31351994Nagib MG: An approach to symptomatic children (ages 4–14 years) with Chiari type I malformation. Pediatr Neurosurg 21:31–35 1994

34.

Nohria VOakes WJ: Chiari I malformation: a review of 43 patients. Pediatr Neurosurg 16:2222271990/91Nohria V Oakes WJ: Chiari I malformation: a review of 43 patients. Pediatr Neurosurg 16:222–227 1990/91

35.

Oakes WJ: Chiari malformations, hydromyelia, and syringomyelia in Wilkins RHRengachary SS (eds): Neurosurgery. New York: McGraw-Hill1985 pp 21022124Oakes WJ: Chiari malformations hydromyelia and syringomyelia in Wilkins RH Rengachary SS (eds): Neurosurgery. New York: McGraw-Hill 1985 pp 2102–2124

36.

Oakes WJGaskill SJ: Symptomatic Chiari malformations in childhood in Park TS (ed): Spinal Dysraphism. Boston: Blackwell Scientific1992 pp 104125Oakes WJ Gaskill SJ: Symptomatic Chiari malformations in childhood in Park TS (ed): Spinal Dysraphism. Boston: Blackwell Scientific 1992 pp 104–125

37.

Oakes WJTubbs RS: Chiari malformations in Winn HR (ed): Youman's Neurological Surgery: A Comprehensive Guide to the Diagnosis and Management of Neurological Problemsed 5. Philadelphia: WB Saunders (In press)Oakes WJ Tubbs RS: Chiari malformations in Winn HR (ed): Youman's Neurological Surgery: A Comprehensive Guide to the Diagnosis and Management of Neurological Problems ed 5. Philadelphia: WB Saunders (In press)

38.

Oldfield EHMuraszko KShawker THet al: Pathophysiology of syringomyelia associated with chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg 80:3151994Oldfield EH Muraszko K Shawker TH et al: Pathophysiology of syringomyelia associated with chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg 80:3–15 1994

39.

Papasozomenos SRoessmann U: Respiratory distress and Arnold-Chiari malformation. Neurology 31:971001981Papasozomenos S Roessmann U: Respiratory distress and Arnold-Chiari malformation. Neurology 31:97–100 1981

40.

Paul KSLye RHStrang FAet al: Arnold-Chiari malformation. Review of 71 cases. J Neurosurg 58:1831871983Paul KS Lye RH Strang FA et al: Arnold-Chiari malformation. Review of 71 cases. J Neurosurg 58:183–187 1983

41.

Pillay PKAwad IALittle JRet al: Symptomatic Chiari malformation in adults: a new classification based on magnetic resonance imaging with clinical and prognostic significance. Neurosurgery 28:6396451991Pillay PK Awad IA Little JR et al: Symptomatic Chiari malformation in adults: a new classification based on magnetic resonance imaging with clinical and prognostic significance. Neurosurgery 28:639–645 1991

42.

Pollack IFPang DKocoshis Set al: Neurogenic dysphagia resulting from Chiari malformations. Neurosurgery 30:7097191992Pollack IF Pang D Kocoshis S et al: Neurogenic dysphagia resulting from Chiari malformations. Neurosurgery 30:709–719 1992

43.

Putnam PEOrenstein SRPang Det al: Cricopharyngeal dysfunction associated with Chiari malformations. Pediatrics 89:8718761992Putnam PE Orenstein SR Pang D et al: Cricopharyngeal dysfunction associated with Chiari malformations. Pediatrics 89:871–876 1992

44.

Schady WMetcalfe RAButler P: The incidence of craniocervical bony anomalies in the adult Chiari malformation. J Neurol Sci 82:1932031987Schady W Metcalfe RA Butler P: The incidence of craniocervical bony anomalies in the adult Chiari malformation. J Neurol Sci 82:193–203 1987

45.

Stovner LJ: Headache and Chiari type I malformation: occurrence in female monozygotic twins and first-degree relatives. Cephalalgia 12:3043071992Stovner LJ: Headache and Chiari type I malformation: occurrence in female monozygotic twins and first-degree relatives. Cephalalgia 12:304–307 1992

46.

Stovner LJ: Headache associated with the Chiari Type I malformation. Headache 33:1751811993Stovner LJ: Headache associated with the Chiari Type I malformation. Headache 33:175–181 1993

47.

Tomaszek DETyson GWBouldin Tet al: Sudden death in a child with an occult hindbrain malformation. Ann Emerg Med 13:1361381984Tomaszek DE Tyson GW Bouldin T et al: Sudden death in a child with an occult hindbrain malformation. Ann Emerg Med 13:136–138 1984

48.

Tubbs RSElton SGrabb Pet al: Analylsis of the posterior fossa in children with the Chiari 0 malformation. Neurosurgery 48:105010552001Tubbs RS Elton S Grabb P et al: Analylsis of the posterior fossa in children with the Chiari 0 malformation. Neurosurgery 48:1050–1055 2001

49.

Tubbs RSWellons JC IIIBlount JPet al: Inclination of the odontoid process in the pediatric Chiari I malformation. J Neurosurg (Spine 1) 98:43492003Tubbs RS Wellons JC III Blount JP et al: Inclination of the odontoid process in the pediatric Chiari I malformation. J Neurosurg (Spine 1) 98:43–49 2003

50.

Tubbs RSWellons JC IIIOakes WJ: Asymmetry of tonsillar ectopia in Chiari I malformation. Pediatr Neurosurg 37:1992022002Tubbs RS Wellons JC III Oakes WJ: Asymmetry of tonsillar ectopia in Chiari I malformation. Pediatr Neurosurg 37:199–202 2002

51.

Tubbs RSWellons JC IIISmyth MDet al: Children with growth hormone deficiency and Chiari I malformation: a morphometric analysis of the posterior cranial fossa. Pediatr Neurosurg 38:3243282003Tubbs RS Wellons JC III Smyth MD et al: Children with growth hormone deficiency and Chiari I malformation: a morphometric analysis of the posterior cranial fossa. Pediatr Neurosurg 38:324–328 2003

52.

Venes JL: Multiple cranial nerve palsies in an infant with Arnold-Chiari malformation. Dev Med Child Neurol 16:8178201974Venes JL: Multiple cranial nerve palsies in an infant with Arnold-Chiari malformation. Dev Med Child Neurol 16:817–820 1974

53.

Venes JLBlack KLLatack JT: Preoperative evaluation and surgical management of the Arnold-Chiari II malformation. J Neurosurg 64:3633701986Venes JL Black KL Latack JT: Preoperative evaluation and surgical management of the Arnold-Chiari II malformation. J Neurosurg 64:363–370 1986

54.

Vega AQuintana FBerciano J: Basichondrocranium anomalies in adult Chiari type I malformation: a morphometric study. J Neurol Sci 99:1371451990Vega A Quintana F Berciano J: Basichondrocranium anomalies in adult Chiari type I malformation: a morphometric study. J Neurol Sci 99:137–145 1990

55.

Willhite CC: Dose-response relationships of retinol in production of the Arnold-Chiari malformation. Toxicol Lett 20:2572621984Willhite CC: Dose-response relationships of retinol in production of the Arnold-Chiari malformation. Toxicol Lett 20:257–262 1984

56.

Williams B: Difficult labor as a cause of communicating syringomyelia. Lancet 2:51521977Williams B: Difficult labor as a cause of communicating syringomyelia. Lancet 2:51–52 1977

57.

Williams B: Pathogenesis of syringomyelia in Batzdorf U (ed): Syringomyelia: Current Concepts in Diagnosis and Treatment. Baltimore: Williams & Wilkins1991 pp 5990Williams B: Pathogenesis of syringomyelia in Batzdorf U (ed): Syringomyelia: Current Concepts in Diagnosis and Treatment. Baltimore: Williams & Wilkins 1991 pp 59–90

58.

Williams B: Simultaneous cerebral and spinal fluid pressure recordings. 1. Technique, physiology and normal results. Acta Neurochir 58:1671851981Williams B: Simultaneous cerebral and spinal fluid pressure recordings. 1. Technique physiology and normal results. Acta Neurochir 58:167–185 1981

59.

Williams B: Simultaneous cerebral and spinal fluid pressure recordings. 2. Cerebrospinal dissociation with lesions at the foramen magnum. Acta Neurochir 59:1231421981Williams B: Simultaneous cerebral and spinal fluid pressure recordings. 2. Cerebrospinal dissociation with lesions at the foramen magnum. Acta Neurochir 59:123–142 1981

60.

Williams B: Syringomyelia. Neurosurg Clin N Am 1:6536851990Williams B: Syringomyelia. Neurosurg Clin N Am 1:653–685 1990

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