Intracranial hypertension presenting with severe visual failure, without concurrent headache, in a child with nephrotic syndrome

BMC Pediatrics

Case Report

Madeleine Barnett¹  Ming Lim¹  Manish D Sinha²  Danny Morrison³ 

[1] Children’s Neurosciences, Evelina Children’s Hospital at Guy’s and St Thomas’ NHS Foundation Trust, King’s Health Partners Academic Health Science Centre, Westminster Bridge Road, SE1 7EH, London, England;Paediatric Nephrology, Evelina Children’s Hospital at Guy’s and St Thomas’ NHS Foundation Trust, King’s Health Partners Academic Health Science Centre, Westminster Bridge Road, SE1 7EH, London, England;Paediatric Ophthalmology, Evelina Children’s Hospital at Guy’s and St Thomas’ NHS Foundation Trust, King’s Health Partners Academic Health Science Centre, Westminster Bridge Road, SE1 7EH, London, England;

关键词: Pseudotumour cerebri;    Benign intracranial hypertension;    Idiopathic intracranial hypertension;    Optic atrophy;    Papilloedema;    Cyclosporine;   

DOI  :  10.1186/1471-2431-13-167

 received in 2013-06-21, accepted in 2013-10-10,  发布年份 2013

来源: Springer

【 摘 要 】

BackgroundIdiopathic intracranial hypertension is a condition typically characterised by headache, normal level of consciousness, papilloedema and raised cerebrospinal fluid pressure. Children often present with visual loss and atypical features of raised pressure, posing a diagnostic and management challenge. A range of renal disorders can predispose to developing this raised intracranial pressure syndrome. We present a case of severe visual failure in a child with nephrotic syndrome, with no headache when elevated pressure was proven. In nephrotic syndrome, visual failure related to elevated intracranial pressures without concurrent headache symptoms has not been reported previously.Case presentationWe discuss a 5-year-old Caucasian girl with steroid sensitive nephrotic syndrome who went on to become a late non-responder and presented with intracranial hypertension. Following initial response to steroids, she had a relapse of her nephrotic syndrome; her proteinuria did not resolve on steroid treatment, requiring addition of cyclosporine therapy to manage her nephrotic syndrome. Three months following this, she presented with visual failure in the right eye with bilateral central scotoma and papilloedema. At the time of presentation of visual impairment, she was otherwise well, with no symptoms of a raised intracranial pressure syndrome or associated systemic illness. Medical management was initiated following confirmation of a raised intracranial pressure. Her intracranial pressure remained elevated requiring serial therapeutic lumbar punctures before some improvement in visual acuity was observed. Later in the clinical course, she presented with worsening of her visual impairment with further deterioration of the vision in the left eye, again associated with elevated intracranial pressure. An urgent surgical cerebrospinal fluid diversion procedure was performed. At review, three years after presentation our patient has severe visual impairment with no perception of light in her right eye and 6/36 Snellen acuity in the left secondary to optic atrophy.ConclusionOur case demonstrates the occurrence of intracranial hypertension in nephrotic syndrome, highlighting the atypical presentation of severe visual failure without concurrent headache at presentation. This demonstrates the management complexities and the need for clear guidelines for ophthalmological surveillance to aim to reduce permanent visual impairment.

【 授权许可】

Unknown   
© Barnett et al.; licensee BioMed Central Ltd. 2013. This article is published under license to BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.