Abstract
Purpose
The Marshall-Smith Syndrome (MSS) is a rare disease characterized by orofacial dysmorphism, failure to thrive, accelerated osseous maturation and mental retardation. It has anaesthetic implications due to upper airway problems and possible atlanto-axial instability. We present the perioperative problems (difficult intubation, airway obstruction) encountered in a child with MSS who underwent several anaesthetics during his first two years of life.
Clinical features
At birth, the child presented with asphyxia due to obstructive apnoea. His trachea was, therefore, intubated immediately. The morphological diagnosis of MSS was confirmed by the pathognomonic radiological appearance of the bones (bone age was eight months at the age of four days). Upper airway difficulty was caused by functional problems at the level of the hypopharynx (inspiratory collapse at the level of the velum palatinum), and was solved by the use of a nasopharyngeal airway (NPA) during the induction of anaesthesia and early postoperative period.
Conclusion
The use of an NPA during both induction and recovery of anaesthesia may be particularly useful to prevent upper airway problems in children with MSS.
Résumé
Objectif
Le syndrome de Marshall-Smith (SMS) est une maladie rare d’étiologie inconnue, caractérisée par une dysmorphie faciale, un retard de croissance, une maturation osseuse accélérée et un retard mental. Il a des implications anesthésiques liées à une possible instabilité cervico-atlantoïdienne et à une obstruction chronique des voies aériennes supérieures. Nous présentons les problèmes périopéraoires (intubation difficile, obstruction des voies aériennes) rencontrés chez un enfant atteint de ce syndrome, et qui a bénéficié de plusieurs anesthésies durant ses deux premières années de vie.
Éléments cliniques
Le patient a présenté des problèmes respiratoires qui ont nécessité une intubation dès la naissance. Le diagnostic morphologique de SMS a été confirmé par l’aspect très caractéristique, voire pathognomonique, de la radiologie des mains et par l’âge osseux qui était de huit mois à l’âge de quatre jours. Les problèmes respiratoires étaient dus à un dysfonctionnement vélo-palatin; l’induction et les premières heures postopératoires ont été facilitées par la mise en place prophylactique d’un tube nasopharyngé (TNP), lors de chaque anesthésie.
Conclusion
Dans notre expérience, l’utilisation du TNP à l’induction et en postopératoire s’est révélée particulièrement utile pour résoudre les problèmes d’obstruction des voies aériennes supérieures rencontrés dans le SMS.
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References
Marshall RE, Graham CB, Scott CR, Smith DW. Syndrome of accelerated skeletal maturation and relative failure to thrive: a newly recognized clinical growth disorder. J Pediatr 1971; 78: 95–101.
Charon A, Gillerot Y, Van Maldergem L, Van Schaftingen MH, de Bont B, Koulischer L. The Marshall-Smith syndrome. Eur J Pediatr 1990; 150: 54–5.
Johnson JP, Carey JC, Glassy FJ, Paglieroni T, Lipson MH. Marshall-Smith syndrome: two case reports and a review of pulmonary manifestations. Pediatrics 1983; 71: 219–21.
LaPenna R, Folger GM Jr. Extreme upper airway obstruction with the Marshall syndrome. Clin Pediatr 1982; 21: 507–10.
Cullen A, Clarke TA, O’Dwyer TP. The Marshall-Smith syndrome: a review of the laryngeal complications. Eur J Pediatr 1997; 156: 463–4.
Pappas CTE, Rekate HL. Cervicomedullary junction decompression in a case of Marshall-Smith syndrome. J Neurosurg 1991; 75: 317–9.
Sperli D, Concolino D, Barbata C, Strisciuglio P, Andria G. Long survival of a patient with Marshall-Smith syndrome without respiratory complications. J Med Genet 1993; 30: 877–9.
Cole TRP, Dennis NR, Hughes HE. Weaver syndrome: seven new cases and a review of the literature.In: Donnai D, Winter RM (Eds.). Congenital Malformation Syndromes. London: Chapman & Hall Medical, 1995; 38: 267–80.
Donnelly J, Thirlwell J. Nasopharyngeal airways in infants after palatal surgery (Letter). Br J Anaesth 1992; 68: 227.
Benjamin B, Walker P. Management of airway obstruction in the Pierre Robin sequence. Int J Pediatr Otorhinolaryngol 1991; 22: 29–37.
Ochiai R, Guthrie RD, Motoyama EK. Effects of varying concentrations of halothane on the activity of the genioglossus, intercostals, and diaphragm in cats: an electromyographic study. Anesthesiology 1989; 70: 812–6.
Steven JM, Cohen DE, Sclabassi RJ. Anesthesia equipement and monitoring.In: Motoyama EK, Davis PJ (Eds.). Smith’s Anesthesia for Infants and Children, 6th ed. St. Louis: Mosby, 1996: 229–79.
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Dernedde, G., Pendeville, P., Veyckemans, F. et al. Anaesthetic management of a child with Marshall-Smith syndrome. Can J Anaesth 45, 660–663 (1998). https://doi.org/10.1007/BF03012097
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DOI: https://doi.org/10.1007/BF03012097