The Anesthetic Management of Patients with Multiple Pterygium Syndrome

Multiple pterygium syndrome is a rare, autosomal recessive disorder that involves webbing of the skin across joints, cleft palate, syngnathia (congenital bands of tissue between the maxilla and mandible), ankyloglossia (extensive adhesions of the tongue to the palate), micrognathia, and webbing of the neck [1-3]. These anomalies limit mouth opening, displace the tongue posteriorly, obscure visualization of the pharynx, and prevent neck extension. Patients have contracture bands, known as pterygia, across many of their joints that significantly limit mobility, in addition to other associated anomalies. They often require multiple operations for repair of cleft palate, syndactyly, scoliosis, talipes equinovarus, genital anomalies, umbilical hernias, inguinal hernias, and congenital hip dislocation. Only 60 cases of this syndrome have been reported in the world literature [4-8]. An association with malignant hyperthermia (MH) has been postulated [9,10]. We present our experience with the anesthetic management of two patients with multiple pterygium syndrome. Case Reports Case 1 A 12-wk-old, 3-kg male, with multiple pterygium syndrome, presented for inguinal herniorrhaphy. Examination revealed manifestations of the syndrome including micrognathia, ankyloglossia, cleft palate, and pterygium colli (bands across the neck causing flexion and rotation). Neck extension was significantly limited. The posterior pharynx was unable to be visualized as a result of the airway deformities. He had a small muscular ventricular septal defect, camptodactyly, clubfoot deformities, and pterygia across the elbows, axilla, and popliteal fossae. In the operating room standard noninvasive monitors were placed. Multiple laryngoscope blades, a fiberoptic bronchoscope, a laryngeal mask airway, and a jet ventilator were available. A pediatric otolaryngologist was present to perform a tracheotomy if necessary. Awake fiberoptic intubation using an Olympus LF-P fiberoptic bronchoscope was unsuccessful because the child used his tongue to move the scope when it entered the posterior pharynx. Anesthesia was then induced with halothane and nitrous oxide and the child continued to breathe 100% oxygen and halothane spontaneously. A peripheral intravenous line was placed and 20 micro gram/kg of atropine was administered. Nasotracheal fiberoptic intubation was unsuccessful because of the extreme anterior flexion of the scope required to visualize the glottic opening. A No. 1 laryngeal mask airway (LMA) was then placed, the fiberoptic bronchoscope was placed through the lumen of the LMA and a 3.0 endotracheal tube (ETT) was advanced over the bronchoscope into the trachea. The LMA and the ETT were secured as a unit, and the case proceeded uneventfully. The trachea was extubated after the child awakened from the anesthesia and he did well postoperatively. The child returned 2 mo later for repair of his clubfoot malformation. In the interval the contracture of his neck had worsened, with flexion and rotation of his head to the right. An inhaled induction of anesthesia was performed, and spontaneous ventilation was maintained. Fiberoptic intubation through a No. 1 LMA was performed, although with greater difficulty because of the increased rotation and flexion of his neck. Case 2 A 26-mo-old, 10-kg female, with multiple pterygium syndrome, presented for spinal instrumentation. Examination revealed micrognathia, pterygium colli, and cleft palate. Neck extension was limited. The posterior pharynx was able to be visualized and appeared normal for her age. She had pterygia of her extremities, bilateral clubfoot deformities, and scoliosis. The manifestations of the syndrome were not as severe in this child, and the airway examination was less remarkable than for the first child. After an inhaled induction of anesthesia, the airway was secured by direct laryngoscopy without difficulty. At 34 mo of age she returned for repair of clubfoot deformities. Since difficulty with ventilation or intubation was not anticipated, muscle relaxants were administered. Anesthesia was induced with halothane and nitrous oxide, and paralysis was achieved with vecuronium. Ventilation was easily maintained. Upon direct laryngoscopy, difficulty was encountered in visualization of the glottic opening. Mask ventilation was maintained and the child was repositioned. On a repeat attempt at direct laryngoscopy, the posterior vocal cords were visualized, and the ETT was advanced into the anteriorly positioned trachea. The case proceeded without event and the awake child's trachea was extubated at the end of the procedure. At 38 mo of age she returned for lengthening of her spinal hardware. Her examination results had not significantly changed. Anesthesia and paralysis were induced. While ventilation was controlled, she was placed in an exaggerated "sniffing" position. Direct laryngoscopy was performed but increased difficulty was encountered with visualization of the glottic opening. Positioning was further optimized by a second anesthesiologist who was present. On the third attempt, a Grade 3 laryngoscopic view was obtained when cricoid pressure was held, and a 4.5 ETT was placed. The case proceeded uneventfully, and the awake child's trachea was safely extubated. Discussion This is the first description of the anesthetic management of patients with multiple pterygium syndrome. The management of the airway in these children became more difficult as the children got older as a result of increased deformity of the airway by the pterygia. This led to difficulty in positioning for laryngoscopy and in visualizing the glottic opening. This differs from many other congenital syndromes in which airway management becomes easier as the children grow older and larger. An airway management plan should be established after the conduct of an airway history and physical examination. This is based upon recognized associations between the difficult airway and a variety of congenital, acquired, and traumatic disease states. The intent is to detect medical, surgical, and anesthetic factors that may indicate the presence of a difficult airway [11]. Preparations for airway management include having several anesthesiologists and a pediatric otolaryngologist present with multiple methods of establishing an airway. The first child described presented difficulty with tracheal intubation, ventilation, and cooperation. We attempted an awake nasal fiberoptic intubation but the child repeatedly used his tongue to move the scope away from the larynx, making an awake fiberoptic intubation impossible. General anesthesia was then induced with preservation of spontaneous ventilation. After further unsuccessful attempts at fiberoptic intubation, controlled ventilation by mask was demonstrated before proceeding. As an alternative method of intubation, a No. 1 LMA was placed, a fiberoptic scope was passed through the LMA into the trachea, and an ETT was advanced over the scope. This technique was similar to that described by Theroux et al. [12], although we secured the ETT and LMA together as a unit. In our experience, this provides a secure airway and the LMA remains in place as a backup in the event that the ETT becomes dislodged. The second child's airway did not appear as difficult. An inhaled induction of anesthesia was chosen for the first procedure to allow the child to ventilate spontaneously in the event that the airway proved difficult to manage. With each subsequent procedure, the airway became more difficult to manage, as deformity of the airway increased. An association between multiple pterygium syndrome and MH has been implied by Gericke [9], but no substantive data support this position. It is based on a single case report that did not present sufficient information to confirm or refute the diagnosis of MH [10]. Critical analysis of clinical evidence is required before an association between diseases can be established. There is no substantial evidence associating multiple pterygium syndrome and MH. We used volatile anesthetics in the management of these patients because the advantage of preserving spontaneous ventilation in a child with a difficult airway outweighed the theoretical risk of triggering MH. Multiple pterygium syndrome is a rare, congenital syndrome that has many features associated with difficult airway management. The degree of difficulty in securing the airway increased as these patients increased in age and size. This results from increased rotation, flexion, and decreased mobility of the head and neck caused by the pterygia as the children grew larger. Preparations for alternative techniques of intubation should be made before anesthetic induction; fiberoptic intubation through a LMA was useful. Prior consultation with a pediatric otolaryngologist is strongly advised. Finally, with the current available information, multiple pterygium syndrome patients should not automatically be considered susceptible to MH.