International Journal of Clinical Pediatrics, ISSN 1927-1255 print, 1927-1263 online, Open Access |
Article copyright, the authors; Journal compilation copyright, Int J Clin Pediatr and Elmer Press Inc |
Journal website https://www.theijcp.org |
Case Report
Volume 13, Number 2, June 2024, pages 48-53
Perioperative Care of a Child With Allan-Herndon-Dudley Syndrome
Table
AHDS: Allan-Herndon-Dudley syndrome; ICU: intensive care unit. |
1. Airway concerns |
a. No reports of difficulties with endotracheal intubation or bag-valve-mask ventilation |
b. Macroglossia |
i. Potential impact on airway management |
ii. May exacerbate obstructive sleep apnea (OSA) |
2. Hypotonia |
a. No information regarding safety of succinylcholine |
b. Potential for increased sensitivity to non-depolarizing neuromuscular blocking agent |
c. Consider use of sugammadex to reverse neuromuscular blockade |
3. Cardiovascular involvement related to high peripheral thyroid hormones |
a. Tachycardia |
b. Arrhythmias |
c. Potential for myocardial dysfunction |
4. Potential for postoperative respiratory insufficiency |
a. Associated hypotonia |
b. Potential for increased sensitivity to opioids and anesthetic agents |
c. Impaired upper airway control with OSA |
d. Poor cough effort leading to mucus plugging and atelectasis |
e. Increased oropharyngeal secretions, consider use of anti-sialagogue |
f. Impact on perioperative care |
i. Consider postoperative monitoring of respiratory function (pulse oximetry) |
ii. May require ICU care |
iii. Use short acting anesthesia agents |
iv. Maximize non-opioid analgesia adjuncts, consider regional anesthesia for postoperative analgesia |
v. Limit use of systemic opioids |
5. Gastrointestinal involvement |
a. Failure to thrive |
b. Poor oral intake |
c. Gastroesophageal reflux |
6. Central nervous system involvement |
a. Developmental delay, intellectual impairment |
b. Failure to thrive |
c. Hypotonia |
d. Extra-pyramidal involvement, movement disorder, choreoathetosis |
e. Seizures |
7. Untreated central hypothyroidism |