Anesthetic Management of a Patient with West Syndrome

Vol 4 | Issue 3 | Sep-Dec 2018 | page: 11-13| Prerna Phulkar, Pallavi Waghalkar


Authors: Prerna Phulkar [1], Pallavi Waghalkar [1].

[1] Department of Anaesthesiology, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, Maharashtra, India

Address of Correspondence

Dr.Prerna Phulkar,

B-501, Downtown Langston, Opposite Victorious Kids School, Fountain Road, Kharadi, Pune-411014, Maharashtra, India.

E-mail: prerna.phulkar19@gmail.com


Abstract

West syndrome is a rare and severe form of epilepsy that occurs in early infancy. It is characterized by a triad consisting of infantile spasms, mental retardation, and interictal electroencephalogram pattern termed hypsarrhythmia. A thorough preoperative assessment forms a very important part in the anesthetic management of such patients considering the possibility of difficult intubation because of the coexisting anatomical malformations, establishment of peripheral intravascular access, and careful positioning due to contractures, seizures, and the adverse effects of drugs taken for it. We present anesthesia management of a case of West syndrome with subtrochanteric fracture femur posted for intramedullary nailing.

Keywords: Difficult intubation, infantile spasms, mental retardation, West syndrome.


References

  1. Pies NJ, Beardsmore CW. West & west syndrome-a historical sketch about the eponymous doctor, his work and his family. Brain Dev 2003;25:84-101.
  2. Proposal for Revised Classification of Epilepsies and Epileptic Syndromes. Commission on classification and terminology of the international league against epilepsy. Epilepsia 1989;30:389-99.
  3. Taghdiri MM, Nemati H. Infantile spasm: A review article. Iran J Child Neurol 2014;8:1-5. 4. Zupanc ML. Infantile spasms. Expert Opin Pharmacother 2003;4:2039-48.
  4. Zupanc ML. Infantile spasms. Expert Opin Pharmacother 2003;4:2039-48.
  5. Kaushik JS, Patra B, Sharma S, Yadav D, Aneja S. Clinical spectrum and treatment outcome of west syndrome in children from Northern India. Seizure 2013;22:617-21.
  6. Eling P, Renier WO, Pomper J, Baram TZ. The mystery of the doctor’s son, or the riddle of West syndrome. Neurology 2002;58:953-5.
  7. Millichap JG. History of West syndrome. Pediatr Neurol Briefs 2002;16:29.
  8. Şahin SH, Çopuroğlu E, Uğur H, Sağıroğlu G, Çolak A. Anaesthesia management of a child with west syndrome. Turk J Anaesthesiol Reanim 2014;42:362-4.
  9. West Sendromlu Çocukta Anestezi Yönetimi Anaesthesia Management of a Child with West Syndrome. Turk J Anaesth Reanim 2014;42:362-4.
  10. Khatri A, Kalra N, Tyagi R, Baweja M, Khandelwal D. Dental findings in patients with west syndrome: A report of two cases. J Indian Soc Pedod Prev Dent 2014;32:168-71.
  11. Yi Y, Lee JH, Suh ES. Toxic epidermal necrolysis induced by lamotrigine treatment in a child. Korean J Pediatr 2014;57:153-6.
  12. Kälviäinen R, Nousiainen I. Visual field defects with vigabatrin: Epidemiology and therapeutic implications. CNS Drugs 2001;15:217-30.
  13. Frost JD Jr. Hrachovy RA. Pathogenesis of infantile spasms: A model based on developmental desynchronization. J Clin Neurophysiol 2005;22:25-36.
  14. Indian Society of Anaesthetists, National Institutes of Health (U.S.). PubMed Central. S, SPARC (Organization) S, Iqbal A, Pal S, Wankhede R. Indian Journal of Anaesthesia. Vol. 52. New Delhi: Indian Society of Anaesthetists; 2008. p. 397.


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