ESPE Yearbook of Paediatric Endocrinology

J Clin Endocrinol Metab. 2021 Mar 25:dgab190. doi: 10.1210/clinem/dgab190. PMID: 33765130. https://academic.oup.com/jcem/article-abstract/106/7/e2557/6188450?redirectedFrom=fulltext

In brief: This multicentre cohort study explores the frequency, long-term outcomes and potential predictors of central nervous system (CNS) lesions in 770 Turkish girls with central precocious puberty (CPP). The authors conclude that cranial MRI remains justified in all girls with CPP.

Comment: The proportion of girls with CPP with a CNS abnormality varies between 0-27% (1-4). Such abnormalities can be hamartomas, hydrocephaly, cysts, neoplasia, trauma, inflammatory diseases or developmental abnormalities. The 2007 ESPE-LWPES consensus discussed that the MRI requirement for girls who present CPP between ages 6-8 years is controversial (5). Girls with CPP < 6 years, with rapid pubertal progression or neurological symptoms are more likely to have a CNS abnormality. However, a small proportion of girls without neurological symptoms or very early presentation might still have CNS abnormalities on MRI.

The current study retrospectively analysed a cohort of 770 girls with CPP in 9 Turkish reference centres. 13.5% had an abnormal brain MRI, of whom 2.8% had a previously known lesion, and 3.8% had a causally related lesion. Two neoplastic lesions were identified, one low grade glioma and one meningioma. Five patients (0.6%) presented with pathologies that required neurosurgery. The most common brain abnormality was hamartoma, consistent with previous studies (4, 6). Age at breast development < 6 years and LH/FSH ratio < 0.6 were positively associated with a cerebral lesion, although those indicators were not robust enough to be used in clinical decision making. The prevalence of brain abnormalities in this cohort was comparable to previous studies (1,4, 6). Long term follow-up identified a low proportion of neoplasia (0.25%).

In summary, this retrospective study identified a new and causal MRI abnormality in 1:14 girls with CPP < 6 years, and 1:31 girls with CPP > 6 years. The authors conclude that, in the absence of strong clinical or biological predictors, cranial MRI remains justified in all girls with CPP. This raises the question of MRI accessibility in low income countries.

Reference: 1. Cisternino M, Arrigo T, Pasquino AM, Tinelli C, Antoniazzi F, Beduschi L, Bindi G, Borrelli P, De Sanctis V, Farello G, Galluzzi F, Gargantini L, Lo Presti D, Sposito M, Tatò L. (2000) Etiology and age incidence of precocious puberty in girls: a multicentric study. J Pediatr Endocrinol Metab. 13 Suppl 1:695–701.2. Chalumeau M, Hadjiathanasiou CG, Ng SM, Cassio A, Mul D, Cisternino M, Partsch CJ, Theodoridis C, Didi M, Cacciari E, Oostdijk W, Borghesi A, Sippell WG, Bréart G, Brauner R. (2003) Selecting girls with precocious puberty for brain imaging: validation of European evidence-based diagnosis rule. J Pediatr 143(4):445–50.3. Mogensen SS, Aksglaede L, Mouritsen A, Sørensen K, Main KM, Gideon P, Juul A. (2012) Pathological and incidental findings on brain MRI in a single-center study of 229 consecutive girls with early or precocious puberty. PLoS One 7(1):e298294. Pedicelli S, Alessio P, Scire G, Cappa M, Cianfarani S. (2014) Routine screening by brain magnetic resonance imaging is not indicated in every girl with onset of puberty between the ages of 6 and 8 years. J Clin Endocrinol Metab. 99 (12):4455–4461.5. Carel JC, Eugster EA, Rogol A, Ghizzoni L, Palmert MR; ESPE-LWPES GnRH Analogs Consensus Conference Group, Antoniazzi F, Berenbaum S, Bourguignon JP, Chrousos GP, Coste J, Deal S, de Vries L, Foster C, Heger S, Holland J, Jahnukainen K, Juul A, Kaplowitz P, Lahlou N, Lee MM, Lee P, Merke DP, Neely EK, Oostdijk W, Phillip M, Rosenfield RL, Shulman D, Styne D, Tauber M, Wit JM. (2009) Consensus statement on the use of gonadotropin-releasing hormone analogs in children. Pediatrics. 123(4):e752–e762.6. Chalumeau M, Chemaitilly W, Trivin C, Adan L, Bréart G, Brauner R. (2002) Central precocious puberty in girls: an evidence based diagnosis tree to predict central nervous system abnormalities. Pediatrics 109 (1):61–67.