Supraclavicular Fat Pads

Arquivos Brasileiros de Endocrinologia & Metabologia - Pseudotumor ...

Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS, Brazil

Correspondence to

SUMMARY

Benign intracranial hypertension (Pseudotumor cerebri) has been described as related to the reduction in steroid levels in Cushing's disease (CD), especially after surgical remission. Ketoconazole is a common and effective adjuvant therapy for hypercortisolism, but the major concern is liver enzyme dysfunction. We describe here the case of a 12-year old girl with CD who developed benign intracranial hypertension during treatment with ketoconazole. She presented headache, vomiting, a black spot on her right temporal visual field, and signs of elevated intracranial pressure. Pituitary image was normal on magnetic resonance image (MRI), and all symptoms improved after treatment with acetazolamide. We call attention to the diagnosis of this disorder in CD patients, especially children on ketoconazole treatment, because it could be confounded with adrenal insufficiency and lead to definitive severe visual impairment.

SUMÁRIO

Hipertensão intracraniana benigna (Pseudotumor cerebral) tem sido descrita relacionada à redução dos níveis de esteroides séricos na doença de Cushing (DC), especialmente após a remissão cirúrgica. O cetoconazol é uma opção efetiva e de uso rotineiro como adjuvante na terapêutica do hipercortisolismo, tendo como paraefeito mais temido a toxicidade hepática. Relatamos o caso de uma menina com 12 anos de idade portadora de DC que desenvolveu hipertensão intracraniana benigna durante tratamento com cetoconazol. Apresentou-se com cefaleia, vômitos, comprometimento do campo visual temporal direito e sinais de hipertensão intracraniana. A ressonância magnética (RM) de hipófise era normal e todos os sinais e sintomas resolveram-se com uso de acetazolamida. Chamamos a atenção para esse diagnóstico nos pacientes com DC, especialmente crianças, em tratamento com cetoconazol, porque ele pode ser confundido com insuficiência adrenal e causar comprometimento visual severo e definitivo.

INTRODUCTION

Cushing's disease (CD) patients may be a group at risk for benign intracranial hypertension as a consequence of the long suppression of normal pituitary corticotrophs, exposure to high circulating levels of se-rum cortisol, and abrupt and intense drop of cortisol levels with intervention. Patients effectively treated for hypercortisolism present reduction in the levels of these hormones, what may affect control of brain volume and lead to increased intracranial pressure (1). It is thought that altered cerebrospinal fluid (CSF) absorption gradient across the arachnoid villi may be the primary factor in resetting intracranial fluid compartments (2). There are some reports of benign intracranial hypertension, also called pseudotumor cerebri, associated with CD as a result of the reduction in corticosteroid levels after treatment with metyrapone, aminoglutethimide (1,3), and mitotane (4), or after a reduction in steroid levels in patients treated with pituitary surgery (3,5-7). Adrenal deficiency symptoms, such as headache and nausea, can be manifestations of intracranial hypertension in patients who have recently had the levels of exogenous or endogenous corticosteroids reduced.

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