Follow up of precocious pseudopuberty associated with isolated ovarian follicular cysts
K A Rodriguez-Maciasa, E Thibauda, M Houanga, C Duflosa, C Beldjordb, R Rappaporta
a Pediatric
Endocrinology Unit, Hôpital des Enfants Malades, 149 rue de Sèvres,
75015 Paris, France, b Biochemistry and Molecular Genetic Laboratory,
Hôpital Cochin, Paris, France
Correspondence to: Dr Thibaud.
Accepted 8 December 1998
The clinical outcomes of seven girls presenting with
pseudosexual precocity caused by isolated autonomous ovarian follicular cysts are presented. Six of the seven girls, aged 11 months to 6.9 years, had a unilateral ovarian cyst detected by ultrasound at the
first acute episode. Plasma oestradiol was raised in only five of the
cases, but all had a low response to luteinising hormone releasing
hormone stimulation. Follow up lasted for up to eight years with
recurrent episodes of variable frequency and severity in all seven
patients. Evidence of McCune-Albright syndrome appeared later in only
three patients. It could not be predicted from the initial symptoms or
the clinical course. Mutations of the Gs
protein leading
to activation were investigated in the lymphocytes and ovarian and bone
tissues of four patients. Only one patient showed a mutation in bone
tissue. Close follow up with repeated searches for skeletal lesions
remains necessary since the distribution of somatic mutations cannot be
assessed by molecular studies. Most patients with recurrent ovarian
cysts require a conservative approach.
Keywords: pseudopuberty; ovarian follicular cyst; G protein mutation
© 1999 by Archives of Disease in Childhood
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