Unpredictable responses to anesthesia drugs and difficulty to intubate should be taken into consideration before Angelman syndrome patients undergo surgery, according to a case study.
In the case report, “Angelman syndrome and anaesthetic considerations,” published in the Indian Journal of Anaesthesia, researchers presented the case of a 36‑year‑old woman with Angelman syndrome who was referred to surgery to have her gallbladder removed through open cholecystectomy (a large incision in the abdomen).
A physical examination revealed the patient was only 3.2 feet high and was malnourished, weighing only 22 pounds.
She presented muscle contraction (spasticity) affecting all four limbs and had prominent upper incisors, a high palatal arch, and increased distance between her chin and sternum, all features that could make airway access more difficult. No additional physical alterations were reported.
Due to her severe malnourishment, the patient was orally fed with high-nutritional and protein- and calorie-rich formula at four-hour intervals under close monitoring. Six weeks later, after a weight gain of 15 pounds, she was submitted to surgery.
The patient showed signs of severe parental separation anxiety. To manage this situation, sedation with Versed (midazolam) was administered, but no signs of response to the drug were observed, raising concerns about a possible reduced response to anesthesia.
Anesthesia was given as per standard protocol, despite some difficulties for initial intubation. A small tube was placed in her back (via epidural catheter) to administrate additional anesthetic agents before initiating the surgery, and post-operative analgesia.
“Epidural analgesia is usually not the procedure of choice in patients with tardy neurodevelopmental syndromes,” researchers wrote.
However, the parents “expressed fears of repeated pulling out of any intravenous line,” so the team decided this approach would be the best option.
Sanfilippo patients also present bone alterations such as abnormal curvature of the spine (scoliosis) which can make epidural drug administration more difficult. Vertebral and rib abnormalities can also increase the risk of post‑operative respiratory and heart failures.
The patient’s surgery was uneventful, with no respiratory or cardiac alterations, and proceeded as planned. She was discharged from the hospital on the 10th day.
“[Anti-anxiety agents] and analgesia are important, as proper assessment in these smiling patients can be challenging,” the authors said.
“Unpredictable response to anesthetic agents with a difficult airway, difficult regional block [local anesthesia], and sudden asystole [cardiac arrest] should be anticipated and warrant adequate pre‑operative preparations,” they added.