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Use of the laryngeal mask airway during thoracotomy in a pediatric patient with cri-du-chat syndrome.

Cri-du-chat syndrome, first described in 1963, is a genetic disorder caused by an abnormality of the Number 5 Group B chromosome (1). It is characterized by the presence of numerous congenital anomalies affecting multiple organ systems. A defect in the larynx is responsible for the characteristic cry that gives the name to the syndrome. We report a case in which the laryngeal mask airway (LMA) was utilized in a patient with cri-du-chat syndrome associated with an abnormal upper airway after traditional techniques of endotracheal intubation had failed. A 15-mo-old, 7.2-kg black male with cri-du-chat syndrome required general anesthesia for ligation of a patent ductus arteriosus (PDA). His past medical history included gastroesophageal reflux diagnosed shortly after birth that required a Nissen fundoplication repair at 27 days of age. On the morning of surgery for the PDA, routine noninvasive monitors were applied for blood pressure, electrocardiogram, 0, saturation, and temperature. Preparations were made in the event of a difficult intubation, i.e., fiberoptic bronchoscope and LMA. Anesthesia was then induced with oxygen and halothane by mask. Ventilation was easily accomplished by gentle manual bag compression, and a 22-g intravenous line was then inserted. Oral intubation with a 3.5and a 4-mm endotracheal tube (Portex, Keene, NH) was attempted several times by a pediatric anesthesiologist and a pediatric surgeon without success. As attempts progressed, bag-mask ventilation became progressively more difficult although adequate ventilation and oxygenation could be maintained. A decision was made to perform a fiberoptic intubation. However, this did not succeed because bleeding and edema impaired visualization of the anatomic structures. A Number 1 LMA (Intavent@; Gensia Pharmaceutical Inc., San Diego, CA) was then inserted without difficulty. Proper positioning was confirmed clinically by bilateral breath sounds, chest movement, presence of expired CO,, and a Sao, of 100%. Ventilation and oxygenation could then be easily maintained. Consequently, it was decided to proceed with the surgery. The patient was placed in the right lateral position, and ventilation and oxygenation were monitored for 5 min. Oxygen saturation remained 100% with a FIO~ of 50%. Anesthesia was maintained with isoflurane and fentanyl (4 pg/kg). After the thoracotomy incision was made, the patient was paralyzed with 0.06 mg/kg of vecuronium and mechanically ventilated with pressure control mode. Oxygen saturation and end-tidal Pco, remained 100% and 3 8 4 2 mm Hg, respectively, for the duration of the procedure. The patient was transferred to the postanesthesia care unit with the LMA in place. When the patient was fully awake, the LMA was removed without complications. The postoperative recovery was uneventful, and he was discharged from the hospital on the fifth postoperative day. Cri-du-chat syndrome is a rare genetic anomaly associated frequently with airway abnormalities, e.g., micrognathia, dysmorphism, and laryngeal structural defects (2,3). In our patient many of the classical findings of the syndrome were present. However, the fact that this patient underwent surgery at 27 days of age without apparent anesthetic complications, and the lack of documentation on the patient records of difficult intubation at that time, led us to initially attempt an inhalation induction followed by orotracheal intubation. After that failed, fiberoptic intubation was the most rational choice since the airway and oxygenation could be maintained, but visualization was difficult because of the trauma inflicted by the repetitive laryngoscopies. The next logical step would have been to awaken the patient and postpone the surgery, but, because bag-mask ventilation was becoming progressively more difficult, we chose to place a Number 1 LMA to improve ventilation. The facts that ventilation was improved and that fiberoptic intubation via the LMAcould be attempted if necessary prompted us to proceed with surgery. Because of the nature of this procedure, we chose to paralyze the patient with a short-acting muscle relaxant and to use controlled ventilation with peak inspiratory pressure below the LMA seal pressure of 20 cm H,O. This was sufficient to maintain adequate ventilation and oxygenation. The LMA has been used successfully for emergency airway management in adult and pediatric patients undergoing different surgical procedures (4-6). This is the first report of the use of the LMA in a patient with cri-du-chat syndrome undergoing PDA repair in which endotracheal intubation by conventional techniques failed. We believe that the clinical circumstances surrounding this case justified our decision to proceed with surgery with the LMA in place. However, it is important to emphasize that the lack of prospective studies evaluating the safety of the LMAduring thoracotomy and the potential risk of aspiration make its routine use during this type of procedure inadvisable at the present time. Manuel R. Castresana, MD Sturla Stefansson, MD Angel R. Cancel, MD Kelly J. Hague, CRNA Deprtiiieizt of Anestliesiokiggy Medical Center C J ~ Ceritrnl Georgia Mercer Uniuersit!y Sclzool of Medicine Mncon, G A 31201