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Transverse venous sinus stenting for pseudotumor cerebri syndrome in a child using recombinant human growth hormone
To report a rare case of a child treated with recombinant human growth hormone (rhGH) for growth failure who presented visual field loss due to refractory pseudotumor cerebri (PTC), successfully managed with a single transverse sinus stent.
Relato do Caso
A 13-year-old boy started treatment with rhGH on a dose of 2,5 mg/daily SC. Eight weeks later, he was complaining of mild headache, blurry of vision and pulsatile tinnitus. Visual acuity was 20/40 in both eyes and fundoscopy revealed extensive fluid around the optic nerve and blurring of the disc margins bilaterally consistent with papilledema. Octopus visual fields revealed inferior defect in right eye and inferonasal defect in left eye. The initial lumbar puncture demonstrated an elevated opening cerebrospinal fluid (CSF) pressure of 47 cmH2O. The rhGH was discontinued and acetazolamide 2g daily was initiated. One week later, his best corrected visual acuity was 20/70 in both eyes and tinittus persisted. Lumbar puncture was repeated and the opening CSF pressure was found to be 51cmH20. Cerebral angiography showed a severe focal stenosis of the right dominant transverse sinus with a congenitally hypoplastic left transverse and sigmoid sinus. A stent was placed across the stenosis and his lumbar CSF pressure dropped to 09 cmH2O. The symptoms and visual fields improved gradually.
Ophthalmologic and neuroradiologic evaluation is recommended for children before and during the use of rhGH therapy. Although long- term studies are needed in this field, this case support the safety and efficay of venous sinus stenting in patients with PTC who are refractory or intolerant to medical therapy or with a fulminante presentation.
PAULO DE TARSO PONTE PIERRE-FILHO, FREDERICO CASTELO MOURA, PAULO PUGLIA JR