Atypical Femoral Fracture in McCune-Albright Syndrome

McCune-Albright Syndrome (MAS) is characterized by endocrinopathies, café-au-lait spots, and fibrous dysplasia. Bisphosphonates are the most prescribed treatment for reducing the pain but their long-term use has been associated with atypical fractures of cortical bones like femur in patients. We present a 23-year-old girl diagnosed with MAS. She had an atypical mid-shaft left femoral fracture that happened during simple walking. She also had a history of long-term use of alendronate. Because of the narrow medullary canal, we used 14 holes hybrid locking plate for the lateral aspect of the thigh to fix the fracture and 5 holes dynamic compression plate (instead of the intramedullary nail) in the anterior surface to double fix it, reducing the probability of device failure. With double plate fixation and discontinuation of alendronate, the complete union was achieved five months after surgery

Download Full-text

McCune Albright Syndrome: A Case Report

Journal of Nepal Paediatric Society ◽

10.3126/jnps.v40i2.29099 ◽

2020 ◽

Vol 40 (2) ◽

pp. 134-137

Author(s):

Subhana Thapa Karki ◽

Vandana Jain

Keyword(s):

Case Report ◽

Fibrous Dysplasia ◽

Precocious Puberty ◽

Genetic Disease ◽

Clinical Presentation ◽

Endocrine Disorders ◽

Mccune Albright Syndrome ◽

Café Au Lait Spots ◽

Albright Syndrome ◽

Mccune Albright

McCune Albright syndrome (MAS) is a very rare genetic disease characterised by any two of the following three findings: café au lait spots, polyosteotic fibrous dysplasia and endocrine disorders. The clinical presentation of MAS may vary depending on which of the various components of the syndrome predominate. Here, we report one case of MAS presenting with precocious puberty.

Download Full-text

Transsphenoidal surgery in a patient with acromegaly and McCune–Albright syndrome: application of neuronavigation

Journal of Neurosurgery ◽

10.3171/jns/2008/108/01/0164 ◽

2008 ◽

Vol 108 (1) ◽

pp. 164-169 ◽

Cited By ~ 8

Author(s):

Wanchen Dou ◽

Ning Lin ◽

Wenbin Ma ◽

Yi Yang ◽

Huijuan Zhu ◽

...

Keyword(s):

Fibrous Dysplasia ◽

Transsphenoidal Surgery ◽

Tolerance Test ◽

Oral Glucose ◽

Mccune Albright Syndrome ◽

History Of ◽

Albright Syndrome ◽

Clinical Triad ◽

The Right ◽

Mccune Albright

✓The McCune–Albright syndrome (MAS) is characterized by a clinical triad of polyostotic fibrous dysplasia, café-au-lait hyperpigmented macules, and hypersecretory endocrinopathies. Acromegaly is an uncommon manifestation of the endocrine disturbance associated with MAS, and the role of surgery in managing these cases has been a topic of debate. The authors present the case of a 35-year-old man with MAS who was also diagnosed with acromegaly, hyperprolactinemia, and pituitary macroadenoma. The patient had an 18-year history of fibrous dysplasia involving the right frontal bone and ribs as well as multiple endocrinopathies, but no cutaneous hyperpigmented macules. An oral glucose tolerance test demonstrated partial suppression of plasma levels of growth hormone (GH). The patient underwent transsphenoidal resection of the pituitary tumor, performed with assistance of neuronavigation, and tolerated the procedure well. After the surgery, both prolactin and GH levels returned to normal. These results suggest that neuronavigation-assisted transsphenoidal surgery can safely remove pituitary adenomas associated with MAS and successfully treat the underlying endocrine abnormalities.

Download Full-text