Abstract
Background
Stridor, associated with vocal cord paralysis, in neonates with myelomeningocele (MMC) is a recognized symptom related to Chiari II malformation (CM). In most children, stridor appears after birth. Control of hydrocephalus, if present, and urgent decompression of the CM are recommended for treatment of these patients. Such management typically improves symptoms. Occasionally, stridor is present at birth and may be secondary, in part, to maldevelopment or prenatal ischemia of the brain stem, rather than treatable compression. There is minimal literature describing the outcome after Chiari decompression in this population. The purpose of this study was to review the outcomes of neonates with MMC and stridor at birth and compare it to MMC patients who develop stridor later. We hypothesized that unlike stridor which develops after birth, stridor at birth predicts a dismal outcome, despite aggressive surgical treatment.
Methods
Retrospective review of newborns with MMC and CM was performed in our institution from 1975 to 2010. Patients with stridor at birth and those who developed stridor later in infancy were identified. Outcomes were analyzed. Autopsy findings were reviewed when available.
Results
Six patients with MMC who presented with stridor at birth were identified. Five of these patients had decompression of CM and treatment of hydrocephalus, if present, within the first 2 weeks of life. All patients died: three within 1 month and the oldest at 62 months. In the three patients with autopsies, vernix caseosa meningitis was present. Eight patients presented with stridor later in infancy. CM decompression was performed in seven of them. One patient out of the seven with late onset of stridor died at 13 months after CM surgery. The mortality rate after CM decompression was worse in patients with stridor at birth than those presenting later with stridor (chi-square p = 0.015).
Conclusions
In newborns with MMC, stridor at birth may predict dismal outcome despite CM decompression. Unlike the situation in neonates who develop stridor after birth, the outcome in those presenting with stridor at birth does not seem to be impacted by decompression of the CM. Nonoperative management may be an option to offer in this population. Additionally, vernix caseosa meningitis may contribute to the severe irreversible brain stem dysfunction in these newborns.
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References
Dias MS (2005) Neurosurgical management of myelomeningocele (spina bifida). Pediatr Rev 26(2):50–59
Park TS, Hoffman HJ, Hendrick B, Humpherys RP (1983) Experience with surgical decompression of the Arnold–Chiari malformation in young infants with myelomeningocele. Neurosurgery 13:147–152
Pollack IF, Kinnunen D, Albright AL (1996) The effect of early craniocervical decompression on functional outcome in neonates and young infants with myelodysplasia and symptomatic Chiari II malformations: results from a prospective series. Neurosurgery 38(4):703–710
Vandertop WP, Asai A, Hoffman HJ, Drake JM, Humphreys RP, Rutka JT, Becker LE (1992) Surgical decompression for symptomatic Chiari II malformation in neonates with myelomeningocele. J Neurosurg 77:541–544
Dyste GN, Menezes AH, Vangilder JC (1989) Symptomatic Chiari II malformations: an analysis of presentation, management and long term outcome. J Neurosurg 71:159–168
Hoffman HJ, Hendrick EB, Humphreys RP (1975) Manifestations and management of Arnold–Chiari malformation in patients with myelomeningocele. Childs Brain 1:255–259
Holinger PC, Holinger LD, Reichert TJ, Holinger PC (1978) Respiratory obstruction and apnea in infants with bilateral abductor vocal cord paralysis, meningomyelocele, hydrocephalus and Arnold–Chiari malformation. J Pediatr 92(3):368–373
Cochrane DD, Adderley R, White CP, Norman M, Steinbok P (1990) Apnea in patients with myelomeningocele. Pediatr Neurosurg 16:232–239
Gilbert JN, Kl J, Rorke LB, Chernoff GF, James HE (1986) Central nervous system anomalies associated with meningocele, hydrocephalus, and the Arnold–Chiari malformation: reappraisal of theories regarding pathogenesis of posterior neural tube closure defects. Neurosurgery 18:559–564
Volpe JJ (2008) Neural tube formation and prosencephalic development. In: Volpe JJ (ed) Neurology of newborn, 5th edn. Saunders-Elsevier, Philadelphia, pp 3–50
Sieben RL, Hamida MB, Shulman K (1971) Multiple cranial nerve deficits associated with the Arnold–Chiari malformation. Neurology 21:673–681
Pollack IF, Pang I, Albright AL, Krieger D (1992) Outcome following hindbrain decompression of symptomatic Chiari malformations in children previously treated with myelomeningocele closure and shunts. J Neurosurg 77:881–888
Charney EB, Rorke LB, Sutton LN, Schut L (1987) Management of Chiari II complications in infants with myelomeningocele. J Pediatr 111:364–371
Correa-Restrepo A, Robertson C, Rozdilsky B (1975) Vernix caseosa meningitis and laryngeal stridor in an infant with myelomeningocele: case report. J Neurosurg 42:718–722
Agamanolis DP, Hite SH, Platt MS, Boeckman CR (1986) Arnold–Chiari malformation: report of four cases with contamination of the central nervous system by amniotic contents. Surg Neurol 25:261–266
Visscher MO, Narendran V, Pickens WL, LaRuffa AA, Meinzen-Derr J, Allen K, Hoath SB (2005) Vernix caseosa in neonatal adaptation. J Perinatol 25(7):440–446
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, MOMS Investigators (2011) A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 364:993–1004
Acknowledgments
The authors would like to thank Dr. Christopher Dunham from British Columbia Children's Hospital, Division of Anatomic Pathology for his valuable contributions to this paper by supplying specimens of vernix caseosa meningitis.
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No financial relationships exist that are relevant to this study.
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The authors would like to declare that no conflict of interest exists.
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Ocal, E., Irwin, B., Cochrane, D. et al. Stridor at birth predicts poor outcome in neonates with myelomeningocele. Childs Nerv Syst 28, 265–271 (2012). https://doi.org/10.1007/s00381-011-1585-8
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DOI: https://doi.org/10.1007/s00381-011-1585-8