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Anesthesia for Freeman-Sheldon syndrome using a laryngeal mask airway

Abstract

Purpose

To present a case of Freeman-Sheldon syndrome (FSS) with a previously unreported technique of anesthetic management, consisting of a malignant hyperthermia free anesthetic and laryngeal mask airway.

Clinical features

Freeman-Sheldon syndrome (also known as whistling face syndrome, Windmill-Vane-Hand syndrome, cranio-carpo-tarsal dysplasia and distal arthrogryposis type 2) is a rare congenital disorder defined by facial and skeletal abnormalities. The three basic abnormalities are microstomia with pouting lips, camptodactyly with ulnar deviation of the fingers and talipes equinovarus. Patients with FSS frequently present for surgical correction of musculoskeletal or facial abnormalities. There are several anesthetic challenges including difficult airway, intravenous cannulation and regional technique. They may be at increased risk for malignant hyperthermia and postoperative pulmonary complications. We present a case of a two-year-old child with FSS undergoing elective unilateral inguinal hernia repair. A non-triggering anesthetic technique was used, consisting of 2 mg·kg−1 propofol followed by a continuous infusion, nitrous oxide 50%/oxygen, and 3 μg·kg−1 fentanyl. Intraoperative and postoperative analgesia was provided by an ilioinguinal nerve block with 10 ml bupivacaine 0.25% with epinephrine 1:200,000. The airway was maintained with a #2 laryngeal mask airway. The anesthetic was uneventful and there were no signs or symptoms of malignant hyperthermia. The patient was discharged home later the same day in good health.

Conclusion

The use of a laryngeal mask airway and non-triggering anesthetic technique should be considered as options for anesthetic management in patients with FSS for short procedures that do not require neuromuscular blockade.

Résumé

Objectif

Présenter un cas de syndrome de Freeman-Sheldon (SFS) et une technique d’anesthésie inédite avec l’utilisation d’un anesthésique libre d’effet déclencheur d’hyperthermie maligne, et d’un masque laryngé.

Éléments cliniques

Le syndrome de Freeman-Sheldon (ou syndrome du siffleur, dystrophie ou dysplasie cranio-carpotarsienne) représente un ensemble rare de malformations congénitales intéressant la face et le squelette. Les trois principales anomalies sont la microstomie avec lèvres éversées, la camptodactylie avec déviation des doigts du côté cubital et les pieds bots varus équins. Ces anomalies musculo-squelettiques ou faciales exigent souvent une correction chirurgicale. Lanesthésie peut alors présenter certains obstacles: intubation, canulation intraveineuse et technique régionale difficiles. Les patients peuvent être à risque élevé d’hyperthermie maligne et de complications pulmonaires postopératoires. Nous présentons ici le cas d’un enfant de deux ans admis pour la réparation élective d’une hernie inguinale unilatérale. Lanesthésie sans effet déclencheur d’hyperthermie comprenait 2 mg·kg−1 de propofol suivis d’une perfusion continue, d’un mélange de protoxyde d’azote et d’oxygène à 50% et de 3 μg·kg−1 de fentanyl. Lanalgésie peropératoire et postopératoire a été assurée par un blocage dunerf ilio-inguinal avec 10 ml de bupivacaïne 0,25% et de l’épinéphrine 1:200 000. Le dégagement des voies aériennes a été maintenu avec un masque laryngé n∘. 2. Lanesthésie a été sans incident et sans signe ou symptôme d’hyperthermie maligne. Lenfant est retourné chez lui le même jour, en bonne santé.

Conclusion

L’utilisation du masque laryngé et d’une technique anesthésique sans effet déclencheur d’hyperthermie est un choix dont il faut tenir compte pour l’anesthésie de patients atteints du SFS lors d’interventions courtes qui ne nécessitent pas de blocage neuromusculaire.

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Correspondence to Stephen Brown.

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Cruickshanks, G.F., Brown, S. & Chitayat, D. Anesthesia for Freeman-Sheldon syndrome using a laryngeal mask airway. Can J Anesth 46, 783 (1999). https://doi.org/10.1007/BF03013916

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Keywords

  • Laryngeal Mask Airway
  • Difficult Airway
  • Malignant Hyperthermia
  • Ulnar Deviation
  • Treacher Collins Syndrome