Pediatric Anesthesia 2005 15: 790–792 doi:10.1111/j.1460-9592.2004.01533.x Case report Awake nasotracheal intubation using fiberoptic bronchoscope in a pediatric patient with Freeman–Sheldon syndrome J.S. KIM MD,S.Y.PARKMD,S.K.MINMD,J.H.KIMMD, S.Y. LEE MD AND B.K. MOON MD Department of Anesthesia and Pain Medicine, Ajou University School of Medicine, Suwon, Korea Summary The Freeman–Sheldon syndrome is a congenital disease primarily affecting the facial, limb and respiratory muscles that give rise to classical clinical features including typical whistling face and short webbed neck associated with difficult intubation. We present successful awake nasotracheal intubation in a 6-year-old patient with typical clinical features of Freeman–Sheldon syndrome by using fiberoptic bronchoscope on two separate occasions. Keywords: intubation: nasotracheal, difficult; equipment The Freeman–Sheldon syndrome (also known as by using a fiberoptic bronchoscope on two separate craniocarpotarsal dysplasia and whistling face syn- occasions. drome) is a rare congenital dysplasia and myopathy that was first reported in 1938 by Freeman and Case report Sheldon (1). The syndrome is thought to be due to a slowly progressive myopathy primarily affecting the A 6-year-old male with Freeman–Sheldon syn- limb and respiratory muscles (2). The three basic drome, weighing 12 kg and 90 cm tall, was sched- abnormalities are whistling face, camptodactyly uled for correction of clubfeet under general with ulnar deviation of the fingers and talipes anesthesia. Feeding difficulties because of micro- equinovarus. In addition to these abnormalities, stomia led to delayed development. Although over- patients with Freeman–Sheldon syndrome suffer all growth was retarded, intelligence and ability in from multiple musculoskeletal abnormalities and verbal communication were normal. frequently present for surgical correction of musculo- Difficulty in tracheal intubation was anticipated skeletal or facial abnormalities (3). Because of micro- because of microstomia with limited mouth open- stomia, kyphoscoliosis and other changes, there are ing and short webbed neck with poor mobility several anesthetic concerns including difficulty in (Figures 1 and 2). We visited the patient preopera- airway management, intravenous cannulation and tively and explained the awake nasotracheal intuba- administering regional techniques (4). tion technique using a fiberoptic bronchoscope. We We report the case of a 6-year-old boy with typical planned blind nasotracheal intubation or tracheo- clinical features of Freeman–Sheldon syndrome who stomy in case the fiberoptic bronchoscopy technique received successful awake nasotracheal intubation failed and asked the otolaryngologists to stand by during induction of anesthesia so an emergency Correspondence to: J.S. Kim, Department of Anesthesia and Pain tracheostomy could be performed if necessary. Medicine, Ajou University School of Medicine, San-5, Wonchon- Dong, Yongtong-Gu, Suwon, Kyungki-Do 442 721, South Korea Preoperative investigations were normal except (email: [email protected]). mild elevation of SGOT. Following an intramuscular 790 Ó 2004 Blackwell Publishing Ltd AWAKE INTUBATION IN FREEMAN–SHELDON SYNDROME 791 the tube were maximal. The fiberoptic bronchoscope (OD 2.7 mm; Karl Storz, Tu¨ ttlingen, Germany) was inserted through the tracheal tube. Because we had not anesthetized the larynx, it was not easy to find the vocal cords; because of the gag reflex it took several attempts over 10 min to insert the fiberoptic bronchoscope into the trachea. After tracheal intubation was achieved, anesthe- sia was induced with intravenous thiopental 50 mg and maintained with isoflurane with 50% nitrous oxide in oxygen and vecuronium bromide. After induction of anesthesia, the body temperature measured in the distal esophagus was 38°C and after 1 h, had increased to 40°C. With the use of Figure 1 alcohol sponges and gauze in iced water to the Frontal photograph of the patient, showing the typical features of upper trunk and forehead, body temperature Freeman–Sheldon syndrome – whistling face and short webbed neck. decreased to 37.4°C by the end of surgery. Apart from the elevation of temperature, there were no intraoperative problems. The patient was transferred to the postanesthetic care unit (PACU) after surgery which lasted 2.5 h. When fully responsive, he was extubated without problem and the postoperative recovery was uneventful. Two weeks later, the same patient was scheduled for surgical correction of microstomia. The day before surgery, we visited the patient again and explained the anesthesia procedure. After premedication with 0.04 mg IM glycopyrro- late, the patient arrived in the operating room when routine monitors were applied. After applying the mixture of 4% lidocaine and vasoconstrictor (1 : 200 000 epinephrine) to the nasal mucosa, an Figure 2 internal diameter 4.5 mm tracheal tube was intro- Lateral photograph of the patient showing micrognathia. duced into the nose to the point where breath sounds through the tube were maximal. The fiberoptic injection of glycopyrrolate 0.04 mg, the patient was bronchoscope (OD 2.7 mm; Karl Storz) was then taken to the operating room and routine monitors inserted through the tube lumen. Because advance- (ECG, blood pressure, and pulse oximeter) applied. ment of the fiberoptic bronchoscope was stopped by Inspection of the mouth revealed poorly visualized coughing, 1.5 ml 1.5% lidocaine was sprayed on the uvula (Mallampati class IV). In addition, it seemed larynx via the suction port of the fiberoptic broncho- to be impossible to insert the proper size of laryngeal scope. Afterward, loss of reflexes allowed easy mask airway (LMA) because of severe microstomia. advancement and in a few minutes, the tracheal tube About 10 min after administering topical anesthesia was introduced into the larynx. Anesthesia was then with mixture of 4% lidocaine and 1 : 200 000 epi- induced with intravenous thiopental 50 mg and nephrine to the nasal mucosa by the otolaryngolo- maintained with isoflurane with 50% nitrous oxide gist, the tip of the tracheal tube (ID 4.5 mm) was in oxygen and vecuronium bromide. gently introduced into the nostril with the patient’s Unlike the previous anesthesia, body temperature cooperation to the point that breath sounds through did not increase. The operation ended without Ó 2004 Blackwell Publishing Ltd, Pediatric Anesthesia, 15, 790–792 792 J.S. KIM ET AL. problem and the patient was extubated fully awake nasotracheal intubation without sedatives or muscle in the operating room. relaxants. The second problem in anesthesia for patients Discussion with Freeman–Sheldon syndrome is hyperpyrexia. There have been reports of malignant hyperthermia Most patients with Freeman–Sheldon syndrome in these patients exposed to halothane and succinyl- have typical dysmorphic features, although there is choline (6) and others reported intraoperative hyper- a spectrum of deformity and disability. Diagnosis pyrexia during a halothane anesthetic that was not can be made prenatally by ultrasound or by clinical thought to be malignant hyperthermia (6). We features present at birth (2). Increased tone and avoided halothane and succinylcholine and were fibrosis of facial muscles result in microstomia and able to correct temperature with alcohol sponge and pursed lips (whistling face), which may be accom- gauze in iced water massage and thought it not panied by micrognathia. They may also have likely that the rise in temperature after induction camptodactyly with ulnar deviation, talipes equino- was triggering of malignant hyperthermia However varus, ocular abnormalities including strabismus, it was probably not prudent to continue isoflurane blepharophimosis, hypertelorism and exotropia. The when we first noticed the increase in temperature microstomia and pursed lips are due to diffuse after induction and reuse of isoflurane during the fibrosis within the orbicularis oris muscle and a second procedure could have been dangerous. fibrous band along the vermilion border of the lower Although respiratory complications did not devel- lip (2). Facial contractures tend to push the lower op in this case, some authors have reported postop- incisors lingually and a mound of soft tissue results erative respiratory complications, particularly in vertical furrows on both sides of the chin. pneumonia and respiratory insufficiency (1). To A generalized myopathy has been implicated in prevent respiratory complications, we recommend the development of multiple musculoskeletal abnor- adequate postoperative analgesia. malities. As many of the features of Freeman– In conclusion, based on this case of successful Sheldon syndrome persist into adulthood and nasotracheal intubation on a 6-year old with typical because of the number and complexity of deform- features of Freeman–Sheldon syndrome, awake ities, these patients may be frequently scheduled for nasotracheal intubation technique using fiberoptic anesthesia and surgery. bronchoscope should be considered as an option for Because of the musculoskeletal abnormalities anesthesia management in a pediatric patient with a including microstomia with limited opening and difficult airway. short webbed neck, patients with this syndrome may present anesthetic problems during induction and References maintenance of anesthesia. Intubating conditions do not improve with general anesthesia or neuro- 1 Munro HM, Butler PJ, Washington EJ. Freeman–Sheldon (whistling face) syndrome. Anaesthetic airway management. muscular