Hypothalamic-Pituitary Lesions in Pediatric Patients: Endocrine Symptoms Often Precede Neuro-Ophthalmic Presenting Symptoms - 23/10/12
, Ana-Claudia Couto-Silva, MD 1, Luis Adan, MD, PhD 1, Christine Trivin, PhD 2, Christian Sainte-Rose, MD 3, Michel Zerah, MD 3, Dominique Valteau-Couanet, MD 4, François Doz, MD 5, Martin Chalumeau, MD, PhD 6, Raja Brauner, MD, PhD 1
Reprint requests: Melissa Taylor, Université Paris Descartes and AP-HP, Hôpital Bicêtre, Unité d’Endocrinologie Pédiatrique, Le Kremlin Bicêtre, 94275, France.Abstract |
Objective |
To evaluate whether analyses of clinical and endocrine presenting symptoms could help to shorten the time to diagnosis of hypothalamic-pituitary lesions in children.
Study design |
A retrospective, single-center, cohort study of 176 patients (93 boys), aged 6 years (range, 0.2-18 years), with hypothalamic-pituitary lesions was performed.
Results |
The lesions were craniopharyngioma (n = 56), optic pathway glioma (n = 54), suprasellar arachnoid cyst (n = 25), hamartoma (n = 22), germ cell tumor (n = 12), and hypothalamic-pituitary astrocytoma (n = 7). The most common presenting symptoms were neurologic (50%) and/or visual complaints (38%), followed by solitary endocrine symptoms (28%). Precocious puberty led to diagnosis in 19% of prepubertal patients (n = 131), occurring earlier in patients with hamartoma than in patients with optic-pathway glioma (P < .02). Isolated diabetes insipidus led to diagnosis for all germ-cell tumors. For 122 patients with neuro-ophthalmic presenting symptoms, the mean symptom interval was 0.5 year (95% CI, 0.4-0.6 year), although 66% of patients had abnormal body mass index or growth velocity, which preceded the presenting symptom interval onset by 1.9 years (95% CI, 1.5-2.4 years) (P < .0001) and 1.4 years (95% CI, 1-1.8 years) (P < .0001), respectively. Among them, 41 patients were obese before diagnosis (median 2.2 years [IQR, 1-3 years] prior to diagnosis) and 35 of them had normal growth velocity at the onset of obesity. The sensitivity of current guidelines for management of childhood obesity failed to identify 61%-85% of obese children with an underlying hypothalamic-pituitary lesion in our series.
Conclusions |
Endocrine disorders occurred in two-thirds of patients prior to the onset of the neuro-ophthalmic presenting symptom but were missed. Identifying them may help to diagnose hypothalamic-pituitary lesions earlier.
Le texte complet de cet article est disponible en PDF.Keyword : BMI, DI, NF1, PP
Plan
| The authors declare no conflicts of interest. |
Vol 161 - N° 5
P. 855 - novembre 2012 Retour au numéro
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