Anesth Analg 2005;101:614-615
© 2005 International Anesthesia Research Society
doi: 10.1213/01.ANE.0000159025.99280.93
LETTER TO THE EDITOR
Anesthesia for Freeman-Sheldon Syndrome Using a Folded Laryngeal Mask Airway
Albert Chen, MD,
Hsien-Yong Lai, MD, PhD,
Yi Lee, MD,
Yao-Lin Yang, MD,
Jyu-Shiou Ho, MD, and
Ming-Hwang Shyr, MD, PhD
Department of Anesthesiology; Buddhist Tzu-Chi Medical Center; Tzu-Chi University School of Medicine; Hualien, Taiwan; mmhr1{at}seed.net.tw
To the Editor:
Freeman-Sheldon syndrome is characterized by abnormal face with severe microstomia, camptodactyly with ulnar deviation, and talipes equinovarus. Anesthetic challenges (1–5) include poor venous access, difficult airway, possible malignant hyperthermia association, postoperative pulmonary complications, and analgesia. Microstomia is a result of diffuse fibrosis within the orbicularis oris muscle. In addition, associated high-arched palate, hypoplastic mandible, short, relatively immobile neck, and high position of the larynx make direct laryngoscopy and orotracheal intubation difficult or impossible.
A 21-yr-old female with the diagnosis of Freeman-Sheldon syndrome and mental retardation presented for surgical correction of a right clubfoot. Because of the need for patient cooperation, awake intubation with fiberoptic bronchoscope or regional anesthesia would generally not be a viable option. We chose a laryngeal mask airway after inhalational induction to preserve spontaneous ventilation and retain the option of waking the patient easily. Given the minimal mouth opening of our patient, complete deflation and folding of the laryngeal mask airway (Fig. 1), along with midline insertion, allowed for its successful placement. We noted that despite microstomia and micrognathia, the oral cavity seemed to be not so small. This may have been because of the microglossia and high arched palate. Once the large elliptical cuff passed through the teeth, insertion of the mask into the hypopharynx and inflation to seal around the entrance into the larynx was not a problem.
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