McCune–Albright Syndrome: Anesthetic Considerations in Neurointerventional Suite

McCune–Albright syndrome is a rare chromosomal endocrine disorder characterized by hypersecretion of hormones, polyostotic fibrous dysplasia, café au lait spots, and sexual precocity, affecting around 1 in 100,000 to 1,000,000.[1] There is limited information about the neuro-anesthetic management of this disorder. We describe anesthetic concerns of such a patient scheduled for embolization of jugular arterio-venous fistula (AVF). Informed consent was obtained from the patient for publication of this report.

An 18-year-old male weighing 30 kg diagnosed with McCune–Albright syndrome having right jugular AVF was scheduled for embolization in the neurointerventional radiology suite. The patient had associated hyperthyroidism, hypophosphatemia, café au lait spots, fibrous dysplasia, and left facial lower motor neuron palsy. He gave history of multiple times fracture of long bones. In the past, he had undergone optic nerve decompression for sudden loss of vision. The patient had history of gradually progressive dysphagia and nasal regurgitation (cranial nerve IX and X involvement) that improved after a course of oral steroids. On examination, the patient was conscious, oriented with no cardiorespiratory compromise. He had short, deformed, and swollen upper and lower limbs and coarse facial features including frontal bossing, wide nose, flared nostrils, thickened jaw and chin, and large tongue with café au lait spots on the forehead. The patient's angle of mouth was deviated to the right and he was unable to close the left eye (CN VII involvement). On airway assessment he had Modified Mallampati grade 3, mouth opening 2.5 finger breadths, thyromental distance of 6 cm, upper lip bite test class 1, and normal neck extension. He also had thyroid swelling in the neck, suggestive of multinodular goiter on ultrasound. Chest X-ray was normal and there was no tracheal compression. Hormonal profile showed normal thyroid-stimulating hormone, growth hormone, cortisol, and insulin-like growth factor 1 levels and slightly elevated parathyroid hormone level. The patient was on tab Neomercazole 2.5 mg once daily (for hyperthyroidism) and phosphate solution.

In neurointerventional suite, a difficult airway cart was prepared. Standard ASA (American Society of Anesthesiologists) monitors revealed normal baseline vitals: heart rate: 98/min, BP (blood pressure): 138/86 mm Hg, and oxygen saturation of 98% on room air. Anesthesia was induced with fentanyl 2 µg/kg and sleep dose of propofol. Adequate mask ventilation could be achieved with the help of oropharyngeal airway. After rocuronium administration, videolaryngoscopy revealed a percentage of glottic opening to be 50% and larynx anteriorly placed. Tracheal intubation was successful with the help of a bougie, in first attempt. Anesthesia was maintained with sevoflurane and nitrous oxide in air, along with fentanyl and rocuronium infusion, with a target partial pressure of carbon dioxide of 40 to 43 mm Hg. A radial arterial line was placed for invasive BP monitoring. Supine positioning was done with utmost care to avoid any fractures and pressure points were padded properly. The patient was handed over to the neuroradiologist. Through left femoral arterial access, diagnostic cerebral angiography was done, which showed an osteodural fistula at the left clival region fed by bilateral ascending pharyngeal arteries, vertebral artery draining into aneurysmally dilated left sigmoid sinus ([Fig. 1A]). Using a microcatheter, glue was injected to obliterate the fistula and postprocedure angiogram showed complete obliteration of the osteo-dural fistula ([Fig. 1B]). After reversal of neuromuscular blockade, trachea was extubated and patient was shifted to neuro-intensive care unit for observation.

Fig. 1 Digital subtraction angiography showing (A) preprocedure right jugular AV fistula with multiple feeders; (B) postembolization view of obliterated fistula with dye uptake in normal vessels. AV, arterio-venous.

The cause of McCune–Albright syndrome is a spontaneous mutation of the GNAS gene during embryogenesis that codes for G-signaling complex found in multiple tissue types, including bone, skin, and endocrine tissues. An increase in intracellular cAMP signaling is responsible for the clinical manifestations of McCune–Albright syndrome.[1] These patients might present in operation room for biopsies, transsphenoidal hypophysectomy, fixation of fractures, thyroidectomy, adrenalectomy, and excision of fibrous dysplastic bone causing compression symptoms. Vascular fragility may make venous access difficult in these patients and might require central venous access depending on the type of procedure. For embolization of cerebral fistula, general anesthesia is the preferred technique. The important preoperative considerations include complete evaluation of endocrine abnormalities and assessment of airway.[2] Hyperthyroidism, growth hormone excess, hyperparathyroidism, hyperprolactinemia, and/or hypercortisolism may coexist in the same patient. Recognition and control of hyperthyroidism are of particular importance as cardiac complications and thyroid storm hugely increase morbidity. Presence of Cushing's syndrome requires preoperative treatment of hypertension, hyperglycemia, and/or hypokalemia. Patients who have undergone adrenalectomy for Cushing's disease, require subsequent replacement with steroids. Etomidate may be omitted in these patients when cortisol dysfunction is suspected. The associated craniofacial fibrous dysplasia, coexisting Cushing's syndrome, and acromegaly can result in a potential difficult airway that requires adequate planning and preparation.[3] History of obstructive sleep apnea remains an important factor in deciding the perioperative management. Upper lip bite test has been suggested as a more reliable indicator of easy intubation in these patients.[4] At times, it might even require awake fiberoptic intubation in small children.[5] Altered airway anatomy due to craniofacial fibrous dysplasia may make airway blocks less reliable as well as difficult to perform. These patients can have coexisting cardiac disease secondary to hyperthyroidism or acromegaly and can develop arrhythmias and hemodynamic instability. Increased bone fragility and nerve entrapment (secondary to fibrous dysplasia) require adequate padding and vigilance during positioning. Fractures are not uncommon in these patients, so careful positioning of the patient remains a prime concern. Restrictive lung disease may be present secondary to spinal column deformity and requires careful perioperative respiratory support. Postoperatively, these patients should be closely monitored for vitals and particularly for any obstructive sleep apnea, electrolyte abnormalities, thyroid storm, and peripheral nerve injuries.

To conclude, perioperative management of patients with McCune–Albright syndrome involves optimization of endocrine abnormalities, care of difficult airway, proper positioning, and attention to neuropathies. Out-of-operation room anesthesia in neurointerventional suite has its own challenges and requires proper preoperative assessment and adequate preparation, beforehand.

 

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Conflict of Interest

None declared.

• References

• 1 Dumitrescu CE, Collins MT. McCune-Albright syndrome. Orphanet J Rare Dis 2008; 3: 12
• 2 Langer RA, Yook I, Capan LM. Anesthetic considerations in McCune-Albright syndrome: case report with literature review. Anesth Analg 1995; 80 (06) 1236-1239
• 3 Koc D, Bayri Y, Seker A. Anesthetic management of an acromegalic patient with McCune Albright syndrome for endoscopic transsphenoidal adenoma removal. J Neurosurg Anesthesiol 2014; 26 (03) 267-269
• 4 Chakravarty C, Yadav N, Ali Z, Prabhakar H. Upper lip bite test in a patient with McCune Albright syndrome with acromegaly. J Clin Neurosci 2010; 17 (02) 258-259
• 5 Bohman JK, Segura L. Awake tracheal intubation in an 8-year-old girl with McCune-Albright syndrome. A A Case Rep 2013; 1 (01) 23-25

Publication History

Article published online:
16 July 2025