Precocious Pseudopuberty Treatment & Management

Updated: Oct 18, 2017
  • Author: Sunil Kumar Sinha, MD; Chief Editor: Robert P Hoffman, MD  more...
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Treatment

Approach Considerations

Medical and surgical therapies are directed at treatment of the underlying cause of precocious puberty. The therapies are designed to minimize both the short-term and the long-term morbidity and mortality of precocious puberty. The initial evaluation can usually be performed on an outpatient basis. However, inpatient studies and surgical treatment may be required. 

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Medical Care

Therapy is primarily carried out by a pediatric endocrinologist. Treatment options based on etiology include: the following:

  • MAS may be mild and slowly progressive; thus, no outpatient medical management is required. If the puberty is rapidly progressive or adult height is severely compromised, treatment may be indicated. Drugs used to treat PPP have included cyproterone acetate (CPA) and medroxyprogesterone acetate (MPA), which, despite  short-term reports of efficacy in controlling breast development and vaginal bleeding, do not appear to influence growth rate or final adult height. [16]  Letrozole was effective in a small cohort of 9 girls. Growth rate and vaginal bleeding decreases were reported, but long-term safety and efficacy have not been established. [16]  A gonadotropin agonist may have an additional benefit but only if the bone age is so advanced that central precocious puberty has begun.
  • The management of testotoxicosis is difficult and controversial. Treatment options include CPA, MPA, ketoconazole, spironolactone, testolactone, anastrozole, letrozole, and bicalutamide. [16] Ketoconazole is associated with decreased glucocorticoid production and hepatotoxicity. An alternative regimen of spironolactone and testolactone has been shown to be an effective treatment, although both drugs require multiple daily dosing. Bicalutamide along with anastrozole have been effective in slowing growth velocity and bone age advancement with once-daily dosing. Progression to CPP is common, and the addition of GnRHa is often required. [6]  

  • Treatment of CAH with near-physiologic replacement doses of hydrocortisone is used to suppress adrenal androgen production.

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Surgical Care

If the patient has a tumor that is causing the precocious puberty, surgical consultation is indicated.

In girls with MAS, surgical options such as cystectomy or oophorectomy are considered suboptimal, as there is a high likelihood of cyst recurrence. These surgeries may also impact fertility in girls with the potential for otherwise normal reproductive function and thus are not recommended. [16]

 

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Consultations

The following specialists may be consulted in the diagnosis and treatment of PPP:

  • Pediatric endocrinologist: Pediatric endocrinologists may be invaluable in the diagnosis of precocious puberty, as well as in the treatment and follow-up care for the patient.

  • Pediatric hematologist and oncologist (if supported by the diagnosis): In the case of malignancy leading to the sexual precocity, the oncologist needs to be involved for possible chemotherapy.

  • Surgeon: In the case of malignancy, surgical subspecialists need to be consulted. Recommendation for clitoral reduction in virilized females is controversial.

  • Pediatric urologist: A urologist may be needed for patients with testicular tumors.

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Long-Term Monitoring

After the initial diagnosis of peripheral precocious puberty and the determination of its etiology, most patients require continued monitoring.

  • McCune-Albright syndrome (MAS): Closely follow the cases of patients with MAS for the occurrence of other endocrinopathies or associated pathology.

  • Testotoxicosis: Treatment decisions of these patients are complex. The most extensive report followed 10 boys during 6 years of treatment, but none had reached final height. [17]  Thus, no firm recommendations for one particular form of therapy are currently available. Offer genetic counseling for families affected with this condition.

  • Congenital adrenal hyperplasia (CAH): Classic simple virilizing CAH requires lifelong therapy with glucocorticoids in both males and females. Most males with late-onset nonclassic CAH do not require glucocorticoid treatment. Treat symptomatic girls with nonclassic CAH with low-dose glucocorticoids throughout their reproductive years.

  • Testicular, ovarian, and adrenal tumors: These tumors are typically unilateral and should be surgically removed. Once removed, the remaining contralateral testis, ovary, or adrenal gland should recover from suppression and be adequate for normal function.

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