Introduction: Organic lesion underlying central precocious puberty (CPP) is common in boys, and rare in girls. We aimed to compare the etiological distribution of organic causes according to gender, and define the clinical-laboratory characteristics that predict an organic cause to CPP.
Subject and methods: Medical records of 260 girls and 120 boys with CPP were reviewed retrospectively to analyze the clinical, laboratory characteristics, radiological findings, and gender related differences in organic etiology.
Results: Organic pathologies were more common in boys (26/120; 21.7%) than girls (16/260; 6.2%) (P<0.001). Among girls 3.5% (9/260), and 5% of boys (6/120) had epilepsy and mental motor retardation with developmental anomalies of CNS on admission. None of the remaining 251 girls and 114 boys had any sign or symptom suggesting a CNS lesion, however pituitary MRI revealed a space-occupying lesion in 7.4% (27/365) of this population. A newly identified space occupying lesion was more common in boys (20/114, 17.5%) than in girls (7/251, 2.8%). Suprasellar arachnoid cysts (8/20) and hypothalamic hamartomas (6/20) were more common in boys, whereas less common lesions were hemorrhagic macroadenoma (1/20), optic gliomas (2/20), craniopharyngioma (1/20), pineal germinoma (1/20) and pinealoblastoma (1/20). In girls, suprasellar arachnoid cysts (2/7), hypothalamic hamartomas (2/7) and hemorrhagic macroadenomas (2/7) were equally common; one girl had chiasmatic optic glioma (1/7). Pituitary MRI also revealed incidental findings (microadenoma and pars intermedia cyst) in 22 girls and 11 boys. Overall, developmental anomalies of CNS (56.2%) is more frequent in girls, whereas space-occupying lesions (76.9%) are more frequent in boys (P<0.05). Arachnoid cysts were nine times (8/114 vs 2/251), and hamartomas were seven times (6/114 vs 2/251) more common in boys in comparison to girls, whereas incidental findings were similar (8.8% vs 9.6%). Age of onset was younger, bone age more advanced, height SDS corrected for bone age lower, and sex steroid levels were higher in organic vs idiopathic PP. Onset of pubertal findings was before 6 years in all girls, and 7 years in all boys with newly identified CNS pathology.
Conclusion: Organic cause underlying CPP is quite rare in girls older than 6 years, and boys older than 7 years. The frequency and distribution of organic etiology differ between girls and boys. It is more likely to identify a new asymptomatic space-occupying CNS lesion, mainly arachnoid cysts and hamartoma underlying CPP in boys, whereas previously known symptomatic developmental anomalies are more common underlying organic cause in girls.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology