Anesthetic management of a patient with Mulvihill-Smith syndrome

Junya Kuribayashi, Tatsuya Yamada, Hiroshi Morisaki, Junzo Takeda

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Mulvihill-Smith syndrome is a rare disease that belongs to progeroid syndromes. This syndrome is characterized by a senile face with an underdeveloped lower half, short stature, microcephaly, multiple pigmented nevi, immunodeficiency, hearing loss, and high-pitched voice. We report anesthetic management of a 27-year-old woman, 138 cm and 27 kg, with this syndrome, who underwent removal of mandibular cyst, partial resection of tongue and keratoplasty. Anesthesia was induced with fentanyl, propofol and vecuronium. There was difficulty in maintaining adequate ventilation with a face mask for children, and we used a mask for infants. Her Cormack grade was rated 3 but her trachea could be intubated assisted by BURP procedure. Anesthesia was maintained with sevoflurane, nitrous oxide and oxygen supplemented with fentanyl. The changes of blood pressure during anesthesia were extraordinary, suggesting the presence of advanced arteriosclerosis. The postoperative course was uneventful, with stable hemodynamics, and the patient was discharged from the hospital on 9th postoperative day. Anesthesia for Mulvihill-Smith syndrome should be performed with caution for the potential risk of difficult airway and unstable hemodynamics.

Original languageEnglish
Pages (from-to)835-837
Number of pages3
JournalJapanese Journal of Anesthesiology
Volume56
Issue number7
Publication statusPublished - 2007 Jul

Fingerprint

Anesthetics
Anesthesia
Fentanyl
Masks
Hemodynamics
Pigmented Nevus
Vecuronium Bromide
Microcephaly
Corneal Transplantation
Arteriosclerosis
Nitrous Oxide
Propofol
Rare Diseases
Trachea
Hearing Loss
Tongue
Ventilation
Cysts
Oxygen
Blood Pressure

Keywords

  • General anesthesia
  • Mulvihill-Smith syndrome
  • Progeroid syndromes

ASJC Scopus subject areas

  • Anesthesiology and Pain Medicine

Cite this

Anesthetic management of a patient with Mulvihill-Smith syndrome. / Kuribayashi, Junya; Yamada, Tatsuya; Morisaki, Hiroshi; Takeda, Junzo.

In: Japanese Journal of Anesthesiology, Vol. 56, No. 7, 07.2007, p. 835-837.

Research output: Contribution to journalArticle

Kuribayashi, J, Yamada, T, Morisaki, H & Takeda, J 2007, 'Anesthetic management of a patient with Mulvihill-Smith syndrome', Japanese Journal of Anesthesiology, vol. 56, no. 7, pp. 835-837.
Kuribayashi, Junya ; Yamada, Tatsuya ; Morisaki, Hiroshi ; Takeda, Junzo. / Anesthetic management of a patient with Mulvihill-Smith syndrome. In: Japanese Journal of Anesthesiology. 2007 ; Vol. 56, No. 7. pp. 835-837.
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