eMedicine Specialties > Endocrinology > Multiple Endocrine Disease and Miscellaneous Endocrine Disease

McCune-Albright Syndrome: Follow-up

Author: Gabriel I Uwaifo, MBBS, Clinical and Research Attending, Assistant Professor of Medicine and Endocrinology, MedStar Clinical Research Center, MedStar Research Institute and Washington Hospital Center
Coauthor(s): Nicholas J Sarlis, MBBS, MD, PhD, FACP, Medical Director, Department of Medical Affairs, Oncology Business Unit, US Pharmaceutical Operations, Sanofi-Aventis US
Contributor Information and Disclosures

Updated: Dec 18, 2008

Follow-up

Deterrence/Prevention

  • No measures are available to prevent McCune-Albright syndrome (MAS); however, appropriate care must be taken for fracture prevention in patients with severe polyostotic fibrous dysplasia.

Complications

  • Depending on the specific bone involved and the specific location, potential complications of fractures include secondary osteomyelitis, compressive neuropathy, Volkmann contractures, sympathetic algodystrophy, myositis, ligamentous ossifications, and pseudoarthrosis.
  • The most dreaded complication of polyostotic fibrous dysplasia (PFD) is osteosarcoma, most often in the setting of irradiation of PFD-affected bones. It is very uncommon; the overall incidence rate of sarcomatous degeneration in the setting of PFD is less than 1%. Most frequently, it involves the bones of the face and femur.
  • Skull involvement can be associated with blindness due to optic nerve compression. Deafness also can occur and is associated with vestibulocochlear nerve compression. Other potential complications can result from compressive neuropathies of the cranial nerves located at the base of the skull.
  • A small subset of patients has significant risk for perioperative sudden death. This is presumed to be secondary to either cardiomyopathy or arrhythmia from excess cAMP expression in cardiac tissue. This is a direct consequence of the constitutive activation of adenylate cyclase by the Gs alpha mutation within the cardiac myocytes.

Prognosis

  • Apart from the small subgroup of patients with increased perioperative mortality and those patients who develop malignancies, McCune-Albright syndrome is not associated with a significantly increased mortality risk.
  • Deformities associated with polyostotic fibrous dysplasia result in variable degrees of morbidity, ranging from mild to very severe.

Miscellaneous

Medicolegal Pitfalls

  • Early recognition of McCune-Albright syndrome (MAS) is vital. Consider the possibility of the diagnosis in the appropriate setting. The physician should have a high index of suspicion for other endocrinopathies associated with the condition.
  • Emphasize the need to avoid irradiation of the bone unless the treatment is absolutely necessary, because irradiation may increase the risk for sarcomatous degeneration.
  • For most physicians who are not endocrinologists, the crucial point in the management of MAS is the recognition of the disease and the referral of the patient to an endocrinologist who is experienced in its management. The endocrinologist will, in turn, offer other referrals as indicated (eg, to an experienced orthopedic surgeon).
  • Consider the diagnostic possibility of MAS in all patients with recurrent fractures, particularly if the fractures occur in the setting of minimal trauma or without trauma. The common pitfall is to simply refer the patient for orthopedic procedures whenever a fracture occurs, without considering the underlying primary diagnosis.
 


More on McCune-Albright Syndrome

Overview: McCune-Albright Syndrome
Differential Diagnoses & Workup: McCune-Albright Syndrome
Treatment & Medication: McCune-Albright Syndrome
Follow-up: McCune-Albright Syndrome
Multimedia: McCune-Albright Syndrome
References
Further Reading
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References

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Further Reading

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Keywords

McCune-Albright syndrome, hyperthyroidism, gynecomastia, thyroid gland, hyperparathyroidism, acromegaly, dysplasia, endocrine glands, Cushing syndrome, Cushing's syndrome, precocious puberty, fibrous dysplasia, endocrine gland, thyroid glands, cafe au lait spots, café au lait spots, endocrinopathy, McCune Albright syndrome, hyperprolactinemia, polyostotic fibrous dysplasia, Mazabraud syndrome

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Contributor Information and Disclosures

Author

Gabriel I Uwaifo, MBBS, Clinical and Research Attending, Assistant Professor of Medicine and Endocrinology, MedStar Clinical Research Center, MedStar Research Institute and Washington Hospital Center
Gabriel I Uwaifo, MBBS is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Physicians-American Society of Internal Medicine, American Diabetes Association, American Medical Association, American Society of Hypertension, and Endocrine Society
Disclosure: Nothing to disclose.

Coauthor(s)

Nicholas J Sarlis, MBBS, MD, PhD, FACP, Medical Director, Department of Medical Affairs, Oncology Business Unit, US Pharmaceutical Operations, Sanofi-Aventis US
Nicholas J Sarlis, MBBS, MD, PhD, FACP is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Association of Clinical Endocrinologists, American College of Endocrinology, American College of Physicians, American Federation for Medical Research, American Head and Neck Society, American Medical Association, American Society for Therapeutic Radiology and Oncology, American Society of Clinical Oncology, American Thyroid Association, Association for Psychological Science, Endocrine Society, European Society for Medical Oncology, New York Academy of Sciences, and Royal Society of Medicine
Disclosure: Sanofi-Aventis Salary Employment

Medical Editor

Ghassem Pourmotabbed, MD†, Former Associate Professor, Department of Internal Medicine, Division of Endocrinology and Metabolism, University of Tennessee School of Medicine and Health Science Center
Ghassem Pourmotabbed, MD† is a member of the following medical societies: American Diabetes Association, American Federation for Medical Research, and Endocrine Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Arthur B Chausmer, MD, PhD, FACP, FACE, FACN, CNS, Professor of Medicine (Endocrinology, Adj), Johns Hopkins School of Medicine; Affiliate Research Professor, Bioinformatics and Computational Biology Program, School of Computational Sciences, George Mason University; Principal, C/A Informatics, LLC
Arthur B Chausmer, MD, PhD, FACP, FACE, FACN, CNS is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Endocrinology, American College of Nutrition, American College of Physician Executives, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Medical Informatics Association, American Society for Bone and Mineral Research, American Society of Law Medicine and Ethics, Endocrine Society, and International Society for Clinical Densitometry
Disclosure: Nothing to disclose.

CME Editor

Mark Cooper, MBBS, PhD, FRACP, Head, Diabetes & Metabolism Division, Baker Heart Research Institute, Professor of Medicine, Monash University
Disclosure: Nothing to disclose.

Chief Editor

George T Griffing, MD, Professor of Medicine, St Louis University School of Medicine
George T Griffing, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Medical Practice Executives, American College of Physician Executives, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Heart Association, Central Society for Clinical Research, Endocrine Society, International Society for Clinical Densitometry, and Southern Society for Clinical Investigation
Disclosure: Nothing to disclose.

 
 
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