We report on two girls with atypical, self-remitting central precocious puberty and their subsequent development until (near) final height. Both showed thelarche prior to chronological age 4 years and an accelerated progressive bone maturation (delta bone age/delta chronological age 1.86 and 3.21, respectively). In patient 1, the hormonal findings were not typical for central precocious puberty (Gonadotropin-releasing-hormone-stimulated LH/FSH ratio 0.54, estradiol 14 pg/ml). In patient 2 the hormonal results were borderline (stimulated LH/FSH ratio 1.0, estradiol <5 pg/ml). During the next 5 years a spontaneous arrest of pubertal development was seen in patient 1 until normal puberty started at age 10 years (menarche 12.7 years). Patient 2 showed a complete regression of pubertal signs until now (age 8 years). Both patients were not treated. Patient 1 reached a normal adult height (168 cm). The remaining growth potential of patient 2 is favourable (height SDS for bone age). Discussion: A review of the literature on atypical central precocious puberty showed that there is a continuum between the extremes of isolated premature thelarche on one end and of progressive central precocious puberty at the other end of the spectrum. This variability refers to the clinical appearance and also to bone maturation and hormonal findings. Even the progression of premature thelarche to overt central precocious puberty is possible. At initial evaluation a clear distinction between simple, non-progressive forms and aggressive, progressive forms is difficult. Thus,an observation period of several months before initiation of treatment is necessary in order to establish a correct diagnosis,the necessity to treat,and a correct evaluation of the efficacy of treatment.
