The Pediatric Airway and related syndromes
Descriptions of these patients are a dominant theme in the pediatric difficult airway management literature . A common feature among children in this population is the finding of a micrognathic mandible.
Goldenhar syndrome (oculo-auriculo-vertebral syndrome) occurs one in every 3,000 to 5,000 live births . It is characterized by facial asymmetry and hypoplasia, micrognathia, hypoplastic zygomatic arch, external ear malformations, hearing loss, and ocular dermoids or lipodermoids. Other oral cavity anomalies may include a high-arch or cleft palate and abnormalities of the tongue . Difficult or impossible laryngoscopy has been described in Goldenhar syndrome patients and is attributed to the finding of micrognathia .
Our patient in my post titled " The Pediatric Airway:More questions than Answers" was a case of Goldenhar's Syndrome. As you can see in the visuals the external ear malformations ( Pre auricular pits / tags) are quite prominent. There is also a distinct element of mandibular hypoplasia alongwith micrognathia and facial asymmetry.
This patient was scheduled for surgery for an ocular dermoid. Fortunately she had been picked up by the pediatrics team and there was sufficient documentation of this anomaly in her records.We did a thorrough clinical and radiological pre-operative evaluation and discussed her case with the pediatrics and the radiodiagnosis teams at our center. Though examination did not reveal any obvious anticipated airway difficulties which would give us sleepless nights'...we did not take any chances and had a difficult airway cart ready ( which included an ENT surgeon standby for emergency tracheostomy) in the OR on the day of surgery.
We followed a standard inhalational induction with incremental Sevoflurane in oxygen. The view at laryngoscopy with a Miller blade was CL II-III, however an oral ETT ID 4.5mm (uncuffed) could be passed without trouble and satisfactory ventilation was achieved.
Respecting the age old norm that all difficult intubations are difficult extubations....we allowed the child to be fully awake before extubating. She was kept for a prolonged period of time in the PACU and shifted to the floor only when she was taking oral sips and was fully alert.
The Goldenhar syndrome is notorious for presenting with increasing airway difficulty as the child grows. That means with every passing year in the child's life any further administration of a general anesthetic will be fraught with further potential danger.
Our patient was 2years old and maybe that was one reason we did not need to resort to extreme measures of securing an airway with her.
The carry home message with such syndromal babies is always to have a very high index of suspicion of a potential unanticipated difficult airway. Remember Goldenhar's syndrome gets more difficult with every passing year!
And yes....talk to the whole team before you decide an anesthetic for your patiet. It always pays to have a team perspective!
Suggested reading:
F.A. Berry, Anesthesia for the child with a difficult airway. In: F.A. Berry, Editor, Anesthetic management of difficult and routine pediatric patients, Churchill Livingstone, New York (1986), pp. 137–167.
Ashish K Khanna MD
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