Anaesthesia challenges in Freeman-Sheldon syndrome

Freeman–Sheldon Syndrome

“…Some studies have used laryngeal mask airway and regional anesthesia for management. 3,4 Our patient had limited mouth opening that took Millers 1 blade and we were able to intubate. But everything anticipating difficult airway was kept ready.…”

“…Freeman-Sheldon syndrome, a slowly progressive congenital myopathy, usually presents with multiple body contractures, skeletal malformations, and craniofacial deformities such as microstomia, micrognathia, microglossia, a high-arched palate, and midfacial hypoplasia characteristically known as whistling mouth syndrome. 3 These patients undergo multiple orthopedic, soft tissue, and cosmetic surgeries for improving quality of life and hence multiple anesthesia exposures. Keeping in mind the difficult airway and the risk of malignant hyperthermia, the anesthesia management becomes challenging.…”

Anesthetic Considerations for an Adult Patient with Freeman-Sheldon Syndrome Undergoing Open Heart Surgery

“…Establishing venous and arterial access can also be difficult in patients with contractures. The widespread utilization of ultrasound to visualize vessels has significantly improved success rates of vascular cannulation [ 6 ] (Figures 1 and 2 ).…”

Dental management of patients with microstomia. A review of the literature and update on the treatment.

“…Similarly, severe cases of microstomia can occur in rare syndromes such as Freeman-Sheldon with craniofacial involvement, giving the patient the appearance of a "whistling face" [22][23][24][25] . Other syndromes with microstomia are Hallerman-Streiff, Schwartz-Jampel, Burton skeletal dysplasia, Leopard syndrome and Moebius syndrome 22,28 .…”

“…Other syndromes with microstomia are Hallerman-Streiff, Schwartz-Jampel, Burton skeletal dysplasia, Leopard syndrome and Moebius syndrome 22,28 . Table 1 summarizes the causes of chronic microstomia.…”