ABSTRACT
Idiopathic intracranial hypertension (IIH) is a disease characterized by an increase in intracranial pressure, without presence of parenchymal lesions or hydrocephalus that justify it. Over 90% of cases there is association with stenosis of the dural venous sinuses. It is characterized by headache, tinidus, nausea, vomiting and visual disturbances. Initial treatment is clinical and when it fails there is indication of invasive procedures, among them shunts and fenestration of the optic nerve sheath. Angioplasty of dural venous sinuses, when indicated, has shown an alternative with better results and less complications. We report a case of a female patient, with 27 years old, diagnosed with IIH and bilateral transverse sinus stenosis, which was treated by bilateral stenting and total resolution of symptoms. Besides describing the case we review the literature about the subject.
Subject(s)
Humans , Female , Adult , Pseudotumor Cerebri/diagnosis , Pseudotumor Cerebri/therapy , Constriction, Pathologic/complications , Transverse Sinuses/abnormalities , Treatment Outcome , Angioplasty/methods , Endovascular ProceduresABSTRACT
Progressive diaphyseal dysplasia , also called Camurati-Engelmann disease (CED), is a kind of autosomal dominant disease mainly involved in long diaphysis , characterized as progressive and symmetric cortical thickening as well as low prevalence.The pathogenic gene of the disease is TGFB 1 located in 19q13, resulting in abnormal bone metabolism.We report a young woman suffering from CED , mainly presented as decreased visual acuity , chronic high intracranial pressure and skull damages by cerebral angiography.The digital subtraction angiography revealed the right transverse sinus stenosis .We first revealed a CED patient with chronic high intracranial pressure caused by sinus stenosis , and selected intravascular therapy for the sinus stenosis.
ABSTRACT
Objective To explore the efficacy and safety of venous sinus stenting in the treatment of refractory idiopathic intracranial hypertension (IIH) complicated with venous sinus stenosis. Methods We reviewed our clinical database for 18 patients with resistant or fulminant IIH and cerebral venous sinus stenosis,who underwent placement of venous sinus stents between January 2013 and December 2016. Patients were considered eligible for stenting based on the following criteria: (1) medically refractory IIH;(2)papilledema confirmed by an ophthalmologist;and(3)dural venous sinus stenosis of the dominant venous outflow system with a gradient of ≥10 mmHg(1 mmHg=0.133 kPa). Prior to intervention, diagnostic angiography and venous pressure measurements were performed. Clinical follow-up was available in all patients at 6 to 48 months after stenting.Angiography to evaluate stent patency and restenosis was performed at 6 months after intervention;Relevant clinical, demographic and radiographic data were extracted after review of these records. The paired t test was used to compare the changes before and after the operation. Results The mean lumbar CSF pressure was reduced from (385±72) mmH2O(1 mmH2O=0.009 8 kPa) to (201±24)mmH2O(t=13.02,P<0.05).The mean pressure gradient across the venous stenosis was reduced from(31.5±11.4)mmHg before the procedure to(2.5±2.1)mmHg(t=10.96,P<0.05).Headache in 13 cases,vision in 14 were resolved. Papilledema and pulsatile tinnitus resolved in all patients. There was no instances of restenosis among the 18 patients with follow-up imaging.Conclusion In patients with IIH and documented evidence of venous sinus stenosis with a high pressure gradient,venous sinus senting represents an effective treatment strategy.