What is the role of surgery in the treatment of precocious puberty in McCune-Albright syndrome (MAS)?

Updated: Jan 17, 2019
  • Author: Gabriel I Uwaifo, MD; Chief Editor: George T Griffing, MD  more...
  • Print
Answer

The need for excision of hyperfunctioning endocrine tissue is directed by the severity of the patient’s endocrine imbalance and the efficacy of medical treatment. When medical therapy fails, oophorectomy or ovarian cystectomy has been used as a last resort for the control of precocious puberty. Despite this approach, most female patients with MAS who have had this surgery have retained normal fertility.

Historically, wedge resection of the ovary was performed if a single large follicular cyst was found. Unfortunately, this approach was often only temporarily successful in treating the estrogen hypersecretion, and other large follicular cysts subsequently formed. Accordingly, many advise against surgical treatment of precocious puberty in MAS.

Laparoscopy minimizes surgical aggression and allows the acquisition of tissue biopsy specimens for molecular analysis. [62] Additionally, hyperestrogenism can be arrested with the excision of hyperactive ovarian tissue. In girls younger than 3 years, laparoscopy can be performed by using the transumbilical laparoscopic ovarian cystectomy approach. In older females, traditional techniques are used.


Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!