Rarities With Anesthetic Implications: Allgrove Syndrome
Allgrove syndrome, also known as triple A syndrome, is an inherited condition that encompasses achalasia, addisonianism, which is primary adrenal insufficiency and alacrima, which is insufficient tear secretion. The syndrome was first identified by Jeremy Allgrove ,it is a rare autosomal recessive congenital progressive disorder, usually without any family history. Gazarian et al suggested that autonomic and other neurologic abnormalities also might be associated with the syndrome classified as 4A.2

Replacement of glucocorticoids in patients with known adrenal insufficiency is critical to avoid an adrenal crisis and to allow for normal growth in children. Growth must be closely monitored because overtreatment with glucocorticoids impairs linear development. Otherwise, treatment is symptomatic. Achalasia usually is managed surgically (i.e., Heller myotomy). Physical therapy also may prove beneficial. Lubricants help manage alacrima.

Anesthetic consideration:
Patients usually present for a laparoscopic or, more recently, peroral endoscopic myotomy. They may also undergo nerve biopsies for diagnostic purposes. Consent may be hampered by deafness, dysarthria, blindness or mental retardation. Short stature and microcephaly may compromise airway management. Autonomic sensory and motor abnormalities should be documented. Spastic tetraparesis may make IV cannulation difficult. Postural hypotension and intraoperative lability are common, requiring early treatment with vasopressors. Preoperative hydrocortisone is essential. Hypoglycemia should be anticipated and managed. Nerve conduction is prolonged, and muscle relaxants should be used with caution, if at all. Preoperative antacids are indicated. Inhalation or IV techniques may be preferable to neuraxial blocks because of inconsistent and developing neurologic changes.

Source: https://www.anesthesiologynews.com/Clinical-Anesthesiology/Article/02-20/Rarities-With-Anesthetic-Implications-Allgrove-Syndrome-Progeria-and-Pancoast-Tobias-Syndrome/57175
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