Pierre Robin Sequence With Full Stomach for Emergency Ventriculoperitoneal Shunt: Anesthetic Challenges

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To JNA Readers:
A 14-month-old male child weighing 6.5 kg was rushed to the operating room (OR) at 20:00 hours for an emergency ventriculoperitoneal (VP) shunt revision. On examination, his GCS was 6/15 (E1V1M4) and his heart rate was 65 beats per minute. Airway assessment revealed severe micrognathia and glossoptosis (backward and downward displacement of tongue) causing respiratory obstruction. His mother gave a history of noisy breathing since 2 months of age. The child was given about 50 mL of cow’s milk 2 hours prior.
The child’s past history revealed that he had a VP shunt inserted electively at 3 months of age for aqueductal stenosis-induced hydrocephalus in our institution. As we anticipated a difficult airway, his old anesthetic record was reviewed. The previous anesthesia team had documented a very difficult airway with 3 failed attempts even after attempting with the video laryngoscope and the VP shunt was performed using a Proseal LMA.
The child was taken to the OR keeping the challenges of anesthetizing a difficult airway with full stomach and raised intracranial pressure in mind. The child was placed on the operating table with the head end elevated to 45 degrees to prevent passive aspiration. Standard monitors were established (NIBP, ECG, SpO2, ETCO2). His baseline heart rate was 64 beats/minute and blood pressure was 110/55 mm Hg. A 24-G intravenous cannula was inserted and 0.2 mg of atropine was administered, which increased the heart rate to 120 beats/minute and the child started moving. The child was induced with propofol (15 mg) and fentanyl (10 μg). On assisting the breathing gently, we confirmed the ability to ventilate and the child was paralyzed with atracurium (10 mg). After about 30 seconds, the stomach was decompressed twice using a wide-bore suction tube (K60 catheter) and 25 to 30 mL of milk was aspirated. As the airway abnormalities improve with age in Pierre Robin Sequence, we made a single attempt of laryngoscopy using the videolaryngoscope (Glidescope) which revealed grade 4 scopy. As the child needed urgent ventricular decompression, we deferred further attempts of laryngoscopy using other airway gadgets (C-Mac or fiberoptic) and secured the airway using a 1.5-size Proseal LMA. A suction catheter (K61) was lubricated and inserted through the gastric suction port of the Proseal LMA, which continued to drain milk. In view of possible aspiration during insertion of the abdominal end of the VP shunt (due to peritoneal handling), we requested the surgeons to insert an external ventricular drainage (EVD) and revise the VP shunt on an elective basis. Soon after tapping the ventricle, clear CSF drained under very high pressure. There was a transient drop in blood pressure (systolic of 110 to 70 mm Hg) indicating a drop in systemic vascular resistance while decreasing the high ICP.1,2 At the end of the procedure the child was reversed and extubated awake in the lateral position and shifted to the ICU. The next day the child underwent a VP shunt on an elective basis using a Proseal LMA without any problems and was discharged on the third postoperative day.
Our child had features of Pierre Robin Sequence consisting of micrognathia, glossoptosis, and airway obstruction.3 Our challenges in this case were: (1) well-documented difficult airway, (2) full stomach, and (3) raised intracranial pressure compromising cerebral perfusion.
Sudden neurological worsening warrants immediate EVD insertion to prevent neurological morbidity and mortality. Although this can be done in the ward setting, the insertion of an EVD in this scenario (difficult airway with full stomach in a small child without an intravenous line) can cause morbidity and mortality.

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