| 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 |