Hostname: page-component-848d4c4894-8kt4b Total loading time: 0 Render date: 2024-06-27T00:08:02.798Z Has data issue: false hasContentIssue false

Newborn Apnea Caused by a Neurofibroma at the Craniocervical Junction

Published online by Cambridge University Press:  18 September 2015

David B. Clarke
Affiliation:
Division of Neurosurgery, The Montreal Children's Hospital, McGill University, Montreal
Jean-Pierre Farmer*
Affiliation:
Division of Neurosurgery, The Montreal Children's Hospital, McGill University, Montreal
José L. Montes
Affiliation:
Division of Neurosurgery, The Montreal Children's Hospital, McGill University, Montreal
Gordon V. Watters
Affiliation:
Department of Neurology, The Montreal Children's Hospital, McGill University, Montreal
Guy Rouleau
Affiliation:
Department of Neurology, The Montreal Children's Hospital, McGill University, Montreal
*
Division of Neurosurgery, The Montreal Children's Hospital, McGill University, 2300 Tupper Street, Montreal, Quebec, Canada H3H 1P3
Rights & Permissions [Opens in a new window]

Abstract:

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

The authors report, for the first time, the finding by magnetic resonance imaging of a neurofibroma at the craniocervical junction with upper cervical cord and lower brainstem compression causing complete apnea from birth. Subsequent subtotal resection of the neurofibroma resulted in the successful extubation of a previously ventilator-dependent patient. After a two month period of breathing spontaneously, the newborn developed an upper respiratory tract infection and was reintubated. The patient, unable to be weaned off of the respirator, was extubated and expired shortly thereafter, at the age of five months. The authors suggest that in newborns with unexplained apnea, MRI of the craniocervical junction is indicated. Certain patients may be discovered who have less compromised cervicomedullary function and are afflicted by less aggressive forms of neurofibromatosis type 1. These patients may benefit permanently from a surgical decompression.

Type
Articles
Copyright
Copyright © Canadian Neurological Sciences Federation 1994

References

REFERENCES

1.Brady, JP and Brooks, JG.Abnormalities of control of respiration. In: Rudolph, MR, Hoffman, JIE, eds. Pediatrics. Norwalk, Connecticut: Appleton and Lange, 1987: 13761380.Google Scholar
2.Epstein, FJ and Farmer, JP.Trends in surgery: laser surgery, use of cavitron and debulking surgery. Neurol Clin North Am 1991; 9.2: 307316.CrossRefGoogle Scholar
3.Camfield, P, Camfield, C, Bagnell, P, and Rees, E.Infant apnea syndrome: a prospective evaluation of etiologies. Clin Pediatr 1982; 21: 684.CrossRefGoogle ScholarPubMed
4.Johnston, K, Newth, CJ, Sheu, KF, et al. Central hypoventilation syndrome in pyruvate dehydrogenase complex deficiency. Pediatrics 1984; 74: 1034.CrossRefGoogle ScholarPubMed
5.Pauli, RM, Scott, CI, Wassman, ER Jr, et al. Apnea and sudden unexpected death in infants with achondroplasia. J Pediatr 1984; 104: 342.CrossRefGoogle ScholarPubMed
6.Sidman, J, Wood, RE, Poole, M, and Postma, DS.Management of plexiform neurofibroma of the larynx. Ann Otol Rhinol Laryngol 1987; 1: 5355.Google Scholar
7.Winter, RB, Lonstein, JE, and Anderson, M.Neurofibromatosis hyperkyphosis: a review of 33 patients with kyphosis of 80 degrees or greater. J Spinal Disord 1988; 1: 3949.Google ScholarPubMed