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
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Case Report

Volume 13, Number 2, June 2024, pages 48-53


Perioperative Care of a Child With Allan-Herndon-Dudley Syndrome

Table

Table 1. End-Organ Involvement and Perioperative Concerns of Patients With AHDS
 
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