The Obv-Eas Method: An Easy Way to Facilitate Fiberoptic Intubation in Pediatric Patients: Case of an Infant with Freeman-Sheldon Syndrome

Dear Editor, We describe a case of Freeman-Sheldon syndrome (FSS) in a 22-month-old infant weighing 6.5 kg scheduled to undergo knee web-plasty in the prone position, in which fiber-optic intubation was successfully performed in an easy way. We briefly review the features of FSS, discuss anesthetic challenges associated with intubation of pediatric patients, and describe the ease and success of fiber-optic intubation using a new technique. FSS, also known as cranio-carpo-tarsal dysplasia and whistling face syndrome, is a rare congenital myopathy disorder first reported in 1938 and characterized by malformations of the face, oral cavity, and musculoskeletal system (1). Common facial features include microstomia, microglossia, a high arched palate, hypoplastic mandible, and a short neck with relatively limited mobility. The combination of abnormal facial characteristics makes direct laryngoscopy and orotracheal intubation difficult or impossible (2). According to the literature, the most frequent challenge during anesthesia is difficult intubation. Studies also reported that FSS can be related with muscle rigidity, pyrexia, and malignant hyperthermia, however, these findings have not been confirmed (3, 4). Our patient was a 22-month-old with Freeman-Sheldon syndrome (FSS) scheduled for web-plasty in the prone position. The male patient weighed only 6.5 kg and had dysmorphic features (Figure 1), with retrognathia, microstomia, microglossia, a high arched palate, a hypoplastic mandible, ulnar deviation of fingers, bilateral congenital talipes equinovarus, and knee dislocation. Intubation had been unsuccessful on two previous occasions due to the presence of the aforementioned anomalies associated with FSS. Given the patient’s history of unsuccessful intubation and the need to perform the surgery in the prone position, we chose fiberoptic-assisted nasotracheal intubation, with the patient sedated but maintaining spontaneous respiration. In addition, spinal anesthesia was administered to prevent postoperative pain. The procedure was explained to the parents and they provided signed informed consent for a combination of regional and general anesthesia. Pre-operative chest radiography, electrocardiography (ECG), hematological and biochemical investigations, baseline vitals recorded using ECG, pulse oximetry, and noninvasive blood pressure were normal. A skin temperature probe was placed after intubation. Anesthesia was induced via a mask with sevoflurane, oxygen, and nitrous oxide, with gradual increases in concentration every few breaths, up to a concentration of 6%. Upon loss of consciousness, the sevoflurane-inspired concentration was reduced to 2% - 4%. Anesthesia was achieved via incremental doses of 1 mg/kg propofol (Lipuro, Braun, Germany) administered intravenously. Spontaneous respiration was maintained during the induction of intravenous (i.v.) anesthesia