Abstract
Object
Chiari malformation type II is almost exclusively found in patients with open spinal dysraphism. Etiology and pathophysiology are not yet completely understood, and management guidelines regarding the best follow-up and treatment of this pathological entity do not exist. In order to assess essential management aspects, literature and a series of secondary neurosurgical interventions in Chiari II patients have been reviewed.
Methods
A literature review regarding etiology, diagnostics, pathophysiology, and management of Chiari malformation type II (CMII) and a retrospective evaluation of a series (2009–2012) of secondary interventions in Chiari II patients have been performed. Inclusion criteria were ICD for myelomeningocele with or without hydrocephalus and ICD for Chiari malformation and neurosurgical OR procedure. Evaluated parameters were: patient demographics, primary management, secondary neurosurgical operations (cranio-cervical decompression, shunt revision, myelolysis) as well as specific findings pre- and postoperatively. Essential results from literature review and patients' series are compiled in order to define management recommendations.
Results
Fifty patients (28 f, 22 m; mean age, 7.1 years (range, 0.5–26 years)) with myelomeningocele-associated Chiari malformation type II were operated on between 2009 and 2012. Twenty-four patients had syringomyelia and scoliosis each, and 12 suffered from both. Orthopedic surgery for scoliosis or kyphosis had been performed in 13 cases. Shunt revision was performed in 38 cases, myelolysis in 17, and decompression of the foramen magnum in 14 (28 %). After a mean follow-up of 1.9 years, syringomyelia decreased from 24 to 16 cases. There was a postoperative reduction of neck pain (one third), sensorimotor (two fifths), and cranial nerve deficits (one half). CSF flow at the foramen magnum did not change visibly after surgery. Ventricular size improved in about half of the patients. Slit-like ventricles were found in nine (6 pre-surgical) and enlarged ventricles in nine (23 pre-surgical). Complication rate was 6 % (3/50) per cases, and no patient died or deteriorated neurologically after surgery.
Conclusion
CMII-related management guidelines are not well defined, since clinical constellations and presentations are varying. Often associated findings are syringomyelia, hydrocephalus, and scoliosis, and symptomatic CMII may be triggered by more than one underlying condition. According to literature and clinical experience, management recommendations can be defined. The most important finding is that hydrocephalus is often involved in symptomatic CMII and must always be considered first in any symptomatic patient. Intrinsic brain stem dysfunctions cannot be treated surgically, and monitoring of vital functions is sometimes the only clinical means that can be offered to the patient. Knowledge of the complex background has led to improved follow-up programs for the affected children and thus also improved longtime survival.
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References
Adzick NS, Thom EA, Spong CY, Brock JW III, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL (2011) A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 364:993–1004
Alsaadi MM, Iqbal SM, Elgamal EA, Gozal D (2012) Sleep-disordered breathing in children with Chiari malformation type II and myelomeningocele. Pediatr Int 54:623–626
Aronson DD, Kahn RH, Canady A, Bollinger RO, Towbin R (1991) Instability of the cervical spine after decompression in patients who have Arnold-Chiari malformation. J Bone Joint Surg Am 73:898–906
Beuls E, Vanormelingen L, Van AJ, Vandersteen M, Adriaensen P, Cornips E, Vles H, Temel Y, Gelan J (2003) The Arnold-Chiari type II malformation at midgestation. Pediatr Neurosurg 39:149–158
Caldarelli M, Di Rocco C, Colosimo C Jr, Fariello G, Di GM (1995) Surgical treatment of late neurological deterioration in children with myelodysplasia. Acta Neurochir (Wien) 137:199–206
Caldarelli M, Di Rocco C, La Marca F (1998) Treatment of hydromyelia in spina bifida. Surg Neurol 50:411–420
Cardoso M, Keating RF (2009) Neurosurgical management of spinal dysraphism and neurogenic scoliosis. Spine (Phila Pa 1976) 34:1775–1782
Christensen B, Rand-Hendriksen S (1998) The significance of associated malformations of the central nervous system in myelomeningocele. Tidsskr Nor Laegeforen 118:4232–4234
Cochrane DD, Adderley R, White CP, Norman M, Steinbok P (1990) Apnea in patients with myelomeningocele. Pediatr Neurosurg 16:232–239
Dias MS (2005) Neurosurgical causes of scoliosis in patients with myelomeningocele: an evidence-based literature review. J Neurosurg 103:24–35
Gorayeb RP, Cavalheiro S, Zymberg ST (2004) Endoscopic third ventriculostomy in children younger than 1 year of age. J Neurosurg 100:427–429
Gozal D, Arens R, Omlin KJ, Jacobs RA, Keens TG (1995) Peripheral chemoreceptor function in children with myelomeningocele and Arnold-Chiari malformation type 2. Chest 108:425–431
Henriques Filho PS, Pratesi R (2009) Sleep disorder: a possible cause of attention deficit in children and adolescents with Chiari malformation type II. Arq Neuropsiquiatr 67:29–34
Marca L, Herman M, Grant JA, McLone DG (1997) Presentation and management of hydromyelia in children with Chiari type-II malformation. Pediatr Neurosurg 26:57–67
Luigetti M, Losurdo A, Dittoni S, Testani E, Colicchio S, Gnoni V, Farina B, Scarano E, Zampino G, Mariotti P, Rendeli C, Di RC, Massimi L, Della MG (2010) Improvement of obstructive sleep apneas caused by hydrocephalus associated with Chiari malformation type II following surgery. J Neurosurg Pediatr 6:336–339
Milhorat TH, Nishikawa M, Kula RW, Dlugacz YD (2010) Mechanisms of cerebellar tonsil herniation in patients with Chiari malformations as guide to clinical management. Acta Neurochir (Wien) 152:1117–1127
Miller E, Widjaja E, Blaser S, Dennis M, Raybaud C (2008) The old and the new: supratentorial MR findings in Chiari II malformation. Childs Nerv Syst 24:563–575
Morota N, Ihara S (2008) Postnatal ascent of the cerebellar tonsils in Chiari malformation type II following surgical repair of myelomeningocele. J Neurosurg Pediatr 2:188–193
Nishimura T, Mori K (1996) Blink reflex in meningomyelocele, with special reference to its usefulness in the evaluation of brainstem dysfunction. Childs Nerv Syst 12:2–12
Nishimura T, Mori K (1996) Somatosensory evoked potentials to median nerve stimulation in meningomyelocele: what is occurring in the hindbrain and its connections during growth? Childs Nerv Syst 12:13–26
Piatt JH Jr (2010) Treatment of myelomeningocele: a review of outcomes and continuing neurosurgical considerations among adults. J Neurosurg Pediatr 6:515–525
Salman MS, Dennis M, Sharpe JA (2009) The cerebellar dysplasia of Chiari II malformation as revealed by eye movements. Can J Neurol Sci 36:713–724
Salman MS, Sharpe JA, Lillakas L, Dennis M, Steinbach MJ (2008) The vestibulo-ocular reflex during active head motion in Chiari II malformation. Can J Neurol Sci 35:495–500
Salman MS, Sharpe JA, Lillakas L, Dennis M, Steinbach MJ (2009) Visual fixation in Chiari type II malformation. J Child Neurol 24:161–165
Stritzke AI, Dunham CP, Smyth JA, Steinbok P (2011) Congenital stridor in the context of Chiari malformation type II: the etiological role of vernix caseosa granulomatous meningitis. J Neurosurg Pediatr 8:372–376
Sweeney KJ, Caird J, Sattar MT, Allcutt D, Crimmins D (2013) Spinal level of myelomeningocele lesion as a contributing factor in posterior fossa volume, intracranial cerebellar volume, and cerebellar ectopia. J Neurosurg Pediatr 11:154–159
Tulipan N, Hernanz-Schulman M, Bruner JP (1998) Reduced hindbrain herniation after intrauterine myelomeningocele repair: a report of four cases. Pediatr Neurosurg 29:274–278
Tulipan N, Hernanz-Schulman M, Lowe LH, Bruner JP (1999) Intrauterine myelomeningocele repair reverses preexisting hindbrain herniation. Pediatr Neurosurg 31:137–142
Waters KA, Forbes P, Morielli A, Hum C, O’Gorman AM, Vernet O, Davis GM, Tewfik TL, Ducharme FM, Brouillette RT (1998) Sleep-disordered breathing in children with myelomeningocele. J Pediatr 132:672–681
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Messing-Jünger, M., Röhrig, A. Primary and secondary management of the Chiari II malformation in children with myelomeningocele. Childs Nerv Syst 29, 1553–1562 (2013). https://doi.org/10.1007/s00381-013-2134-4
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DOI: https://doi.org/10.1007/s00381-013-2134-4