Stuve Wiedemann syndrome and related syndromes: Case report and possible anesthetic complications

Dominique Bonthuis, Eva Morava-Kozicz, Leo H D J Booij, Jacques J. Driessen

Research output: Contribution to journalReview article

9 Citations (Scopus)

Abstract

Stuve Wiedemann syndrome (SWS) is an autosomal recessively inherited syndrome which is characterized by bowing of the long bones, camptodactyly, facial dysmorphism, hypotonia, feeding and swallowing difficulties, and respiratory distress. In most cases episodes of unexplained hyperthermia are present. Patients with SWS can develop hyperthermia in conjunction with anesthesia and surgery, and a relationship has been suggested between the syndrome and malignant hyperthermia. We describe a 3-year-old child diagnosed with SWS to whom we administered general anesthesia during the removal of a corneal ulcer and dilatation of the lacrimal duct. Our patient had received, uncomplicated, inhalational anesthesia five times previously for different operations. There were no anesthesia-related complications in the present or previous perioperative periods. On one occasion the patient developed mild postoperative hyperthermia. We believe that this hyperthermia is different from the specific disorder of malignant hyperthermia and that sevoflurane can be safely used in patients with SWS. We also describe symptomatically related syndromes and their theoretical risks for anesthesia.

Original languageEnglish (US)
Pages (from-to)212-217
Number of pages6
JournalPaediatric Anaesthesia
Volume19
Issue number3
DOIs
StatePublished - Mar 1 2009
Externally publishedYes

Fingerprint

Anesthetics
Fever
Anesthesia
Malignant Hyperthermia
Facial Bones
Corneal Ulcer
Lacrimal Apparatus
Perioperative Period
Muscle Hypotonia
Deglutition
General Anesthesia
Dilatation
Stuve-Wiedemann syndrome

Keywords

  • Anesthesia
  • CNTF receptor pathway
  • Crisponi syndrome
  • Pediatric
  • Schwartz-Jampel syndrome
  • Stuve Wiedemann syndrome

ASJC Scopus subject areas

  • Anesthesiology and Pain Medicine
  • Pediatrics, Perinatology, and Child Health

Cite this

Stuve Wiedemann syndrome and related syndromes : Case report and possible anesthetic complications. / Bonthuis, Dominique; Morava-Kozicz, Eva; Booij, Leo H D J; Driessen, Jacques J.

In: Paediatric Anaesthesia, Vol. 19, No. 3, 01.03.2009, p. 212-217.

Research output: Contribution to journalReview article

Bonthuis, Dominique ; Morava-Kozicz, Eva ; Booij, Leo H D J ; Driessen, Jacques J. / Stuve Wiedemann syndrome and related syndromes : Case report and possible anesthetic complications. In: Paediatric Anaesthesia. 2009 ; Vol. 19, No. 3. pp. 212-217.
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