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Case Reports
. 2011 Sep;21(3):399-403.

A Case of Atypical McCune-Albright Syndrome with Vaginal Bleeding

Noushin Rostampour  1 , Mahin Hashemipour, Roya Kelishadi, Silva Hovsepian, Ali Hekmatnia
Affiliations
Case Reports

A Case of Atypical McCune-Albright Syndrome with Vaginal Bleeding

Noushin Rostampour et al. Iran J Pediatr. 2011 Sep.

Abstract

Background: McCune-Albright syndrome (MAS) is a rare non-inherited disorder characterized by the clinical triad of precocious puberty, cafe-au-lait skin lesions, and fibrous dysplasia of bone.

Case presentation: We report a girl with MAS, presenting initially with vaginal bleeding at the age of 17 months. Ultrasonography revealed unilateral ovarian cysts and ureteral and ovarian enlargement. Considering the clinical and paraclinical findings, the patient diagnosed as a case of gonadotropin-independent precocious puberty was treated with medroxy-progestrone acetate (MPA) for three months. During the follow up, recurrent episodes of bleeding, ovarian activation and cyst formation, as well as breast size development were reported. At the age of 5.5 years, fibrous dysplasia was detected, which in coexistence with precocious puberty confirmed the diagnosis of MAS. The patient had no cafe-au-lait skin macles during follow up.

Conclusion: Considering that clinical manifestations of MAS appear later in the course of recurrent periods of ovarian activation and cyst formation, a careful clinical observation and follow up of patients is necessary and the diagnosis of MAS must be kept in mind in cases with gonadotropin-independent precocious puberty.

Keywords: Bleeding; Fibrous Dysplasia of Bone; McCune-Albright Syndrome; Precocious Puberty.

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Figures

Fig. 1
Fig. 1
Brain CT scan of the patient with gonadotropin-independent precocious puberty and maxillary fibrous dysplasia
See this image and copyright information in PMC

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