Stroke in hemoglobin (SD) sickle cell disease with moyamoya: successful hydroxyurea treatment after cerebrovascular bypass surgery. (9/349)

An 11-year-old boy with hemoglobin sickle disease (HbSD), bilateral stenosis of the intracranial carotid arteries, and moyamoya syndrome had recurrent ischemic strokes with aphasia and right hemiparesis. His parents (Jehovah's Witnesses) refused blood transfusions. After bilateral extracranial-intracranial (EC-IC) bypass surgery, hydroxyurea treatment increased hemoglobin F (HbF) levels to more than 30%. During a follow-up of 28 months, flow velocities in the basal cerebral arteries remained stable, neurologic sequelae regressed, and ischemic events did not recur. This is the first report of successful hydroxyurea treatment after bypass surgery for intracranial cerebral artery obstruction with moyamoya syndrome in sickle cell disease. The patient's religious background contributed to an ethically challenging therapeutic task. (Blood. 2001;97:2165-2167)  (+info)

Prothrombotic disorders in children with moyamoya syndrome. (10/349)

BACKGROUND AND PURPOSE: Moyamoya syndrome is an uncommon chronic occlusive cerebrovascular disease in children. The origin of moyamoya syndrome remains undetermined. The role of the prothrombotic disorders contributing to its pathogenesis has not been completely elucidated. The purpose of this study was to determine the frequency of prothrombotic disorders in a pediatric population with moyamoya syndrome. METHODS: From May 1992 to April 2000, a prospective study of 10 consecutive children with moyamoya syndrome was carried out at a single center. Evaluation included the following assays: protein C, protein S, antithrombin, plasminogen, activated protein C resistance, factor V Leiden, and prothrombin gene mutations. Lupus anticoagulant, anticardiolipin antibodies, and anti-beta(2)-glycoprotein I antibodies assays were also performed. The clinical characteristics, underlying diseases, family history of thrombosis, radiological findings, treatment, and outcome were also recorded. RESULTS: In our series, prothrombotic disorders were detected in 4 patients (40%). Inherited protein S deficiency was found in 1 patient; lupus anticoagulant and anticardiolipin antibodies were detected in the remaining 3 patients. One presented persistent lupus anticoagulant for 2.7 years until his death. In the case of the other 2 patients, 1 has maintained lupus anticoagulant for 9 months, whereas the other has kept anticardiolipin/anti-beta(2)-glycoprotein I antibodies for 10 months. CONCLUSIONS: We report the hemostatic data of the largest prospective pediatric study carried out at a single center in the western hemisphere. In 4 patients (40%), a prothrombotic disorder was detected. It is tempting to speculate that these hemostatic abnormalities may contribute to the pathogenesis of moyamoya syndrome in some of our patients.  (+info)

Reversal of severe SPECT asymmetry after venous extra-intracranial high flow bypass in a patient submitted to therapeutic internal carotid occlusion: case report. (11/349)

Therapeutic occlusion of the internal carotid artery is the main option for the treatment of the symptomatic intracavernous internal carotid artery aneurysms, but the issue of the best way of doing the balloon test occlusion (BTO) regarding prediction of future ischemic events remains debatable. Single photon emission computerized tomography (SPECT) has been offered as one of the best option of monitoring regional cerebral blood flow (rCBF) during the BTO, where severe asymmetry is predictive of delayed ischemia. We describe a case of important SPECT asymmetry during BTO clinically negative and its complete reversal with carotid occlusion after extra-intracranial bypass with high flow safenous vein bypass between the cervical carotid artery and the middle cerebral artery.  (+info)

Endarterectomy for narrowing of the external carotid artery in a patient who underwent extracranial-intracranial bypass for occlusion of the internal carotid artery--case report. (12/349)

A 56-year-old man had undergone extracranial-intracranial (EC-IC) bypass surgery for occlusion of the right internal carotid artery. Six years later, he complained of transient episodes of numbness in the left arm, occurring periodically over the previous 3 months. Neuroimaging showed the right external carotid artery was severely narrowed and cerebral blood flow (CBF) was diffusely low. Endarterectomy of the right external carotid artery was performed. Postoperative CBF was markedly improved, suggesting that EC-IC bypass became effective again by endarterectomy of a parent artery.  (+info)

Cerebral ischemic complications following intracranial bleeding in patients with moyamoya disease--three case reports. (13/349)

Three patients with bleeding-type moyamoya disease suffered ischemic complications following their first episode of intracranial bleeding. The patients presented with intraventricular hemorrhage and suffered cerebral infarction on the 14th, 16th, and 11th days after the ictus, respectively. The clinical records revealed no obvious hypotension before progression to infarction and suggested that dehydration accelerated by the administration of hyperosmotic drugs was a contributing factor to the infarction. Two patients underwent superficial temporal artery-middle cerebral artery anastomoses in the chronic stages and have never experienced further ischemic complications. The other patient died despite receiving intensive care. Hemodynamic reserve may be severely impaired in patients with bleeding-type moyamoya disease. Adequate control of hydration is important to prevent cerebral infarction after intracranial bleeding in patients with moyamoya disease.  (+info)

Cerebral hemodynamics on MR perfusion images before and after bypass surgery in patients with giant intracranial aneurysms. (14/349)

BACKGROUND AND PURPOSE: Preoperative assessment of the anatomy and dynamics of cerebral circulation for patients with giant intracranial aneurysm can improve both outcome prediction and therapeutic approach. The aim of our study was to use perfusion MR imaging to evaluate cerebral hemodynamics in such patients before and after extraintracranial high-flow bypass surgery. METHODS: Five patients with a giant aneurysm of the intracranial internal carotid artery underwent MR studies before, 1 week after, and 1 month after high-flow bypass surgery. We performed MR and digital subtraction angiography, and conventional and functional MR sequences (diffusion and perfusion). Surgery consisted of middle cerebral artery (MCA)-internal carotid artery bypass with saphenous vein grafts (n = 4) or MCA-external carotid artery bypass (n = 1). RESULTS: In four patients, MR perfusion study showed impaired hemodynamics in the vascular territory supplied by the MCA of the aneurysm side, characterized by significantly reduced mean cerebral blood flow (CBF), whereas mean transit time (MTT) and regional cerebral blood volume (rCBV) were either preserved, reduced, or increased. After surgery, angiography showed good canalization of the bypass graft. MR perfusion data obtained after surgery showed improved cerebral hemodynamics in all cases, with a return of CBF index (CBFi), MTT, and rCBV to nearly normal values. CONCLUSION: Increased MTT with increased or preserved rCBV can be interpreted as a compensatory vasodilatory response to reduced perfusion pressure, presumably from compression and disturbed flow in the giant aneurysmal sac. When maximal vasodilation has occurred, however, the brain can no longer compensate for diminished perfusion by vasodilation, and rCBV and CBFi diminish. Bypass surgery improves hemodynamics, increasing perfusion pressure and, thus, CBFi. Perfusion MR imaging can be used to evaluate cerebral hemodynamics in patients with intracranial giant aneurysm.  (+info)

Venous stasis retinopathy in symptomatic carotid artery occlusion: prevalence, cause, and outcome. (15/349)

BACKGROUND AND PURPOSE: Chronic ocular ischemia is a rare form of ischemia of the eye in patients with carotid artery occlusion (CAO). The early and often asymptomatic stage of chronic ocular ischemia is referred to as venous stasis retinopathy (VSR). The aim of this study was to gain insight into the prevalence, cause, and outcome of VSR in patients with symptomatic CAO. METHODS: In 110 patients with symptomatic CAO, we prospectively investigated the frequency of VSR, the association between the presence of VSR and impaired cerebral blood flow, and the proportion of patients who developed clinically manifest chronic ocular ischemia with ischemia of the anterior eye segment or blindness. RESULTS: At study entry, VSR was found in 32 patients (29%; 95% CI, 21 to 38), particularly in those with symptoms classically associated with a hemodynamic cause, such as limb shaking (relative risk, 2.4; 95% CI, 1.0 to 5.9). Patients with VSR had lower pulsatility indexes in the ophthalmic artery in case of reversed flow, lower cerebral CO2 reactivity, and lower cerebropetal blood flow than patients without VSR. On follow-up (mean, 29 months), clinically manifest chronic ocular ischemia developed in 4 patients (annual rate, 1.5%; 95% CI, 0.4 to 3.8); it tended to occur more often in patients in whom VSR was present at study entry (relative risk, 7.3; 95% CI, 0.8 to 68). CONCLUSIONS: One third of patients with symptomatic CAO has VSR on ophthalmoscopy. VSR is associated with an impaired flow state of the brain. Development of clinically manifest chronic ocular ischemia is rare.  (+info)

Timing of surgery for supratentorial aneurysmal subarachnoid haemorrhage: report of a prospective study. (16/349)

OBJECTIVES: The debate on the timing of aneurysm surgery after subarachnoid haemorrhage (SAH) pivots on the balance of the temporal risk for fatal rebleeding versus the risk of surgical morbidity when operating early on an acutely injured brain. By following a strict management protocol for SAH, the hypothesis has been tested that in the modern arena of treatment for aneurysmal SAH the timing of surgery to secure supratentorial aneurysms does not affect surgical outcome. METHODS: Over a 6 year period, patients admitted with a diagnosis of SAH to a regional neurosurgical unit have been prospectively studied. All have been on a management protocol in which early transfer and resuscitation has been followed regardless of age and clinical condition. Angiographic investigation and surgery have been pursued in those who have been able to at least flex to pain. A total of 1168 patients (60.7% female, mean age 54.3) with proved SAH were received on median day 1 (86.4% arrived within 3 days) of the ictus. Of these, 784 (67.1%) showed aneurysms on angiography and were prepared for surgery. Those who received surgery for a supratentorial aneurysm within 21 days of the ictus were included in the final analysis (n=550). Patients with an initial negative angiogram, with posterior circulation aneurysms, or aneurysms treated by endovascular means, with aneurysms requiring emergency surgery for space occupying haematomas, with aneurysms which re-bled before surgery, and those who received very late surgery (after 21 days from ictus) were excluded. Surgical outcomes at hospital discharge and after 6 months were assessed using the Glasgow outcome score (GOS). Discharge destination and duration of stay in a neurosurgical ward were also documented. The influence of the timing of surgery (early group day 1-3 postictus, intermediate group day 4-10, or late group day 11-21) was analysed prospectively. RESULTS: 60.2% of cases fell into the early surgery group, 32.4% into the intermediate group, and 7.5% into the late operated group. Late surgery was due to delays in diagnosis, transfer, and logistic factors, but not clinical decision. The demographic characteristics, site of aneurysm, and clinical condition of the patients at the time of initial medical assessment were balanced in the three surgical timing groups. There was no significant difference in GOS between the surgical timing groups at 6 months (favourable GOS score 4 and 5: 83.2%, 80.5%, and 83.8% respectively; p=0.47, Kruskal-Wallis test). Outcome was favourable in 84% of patients under 65 years, and 70% in those over 65. The discharge destinations (home, referring hospital, nursing home, rehabilitation centre) showed no significant difference between surgical timing groups. There was no significant difference in mean time to discharge after admission to this hospital from the referring hospital (16.2, 16.2, and 14.6 days for early, intermediate, and late groups respectively; p=0.789, Analysis of variance (ANOVA)). As a result, there was reduction in the mean duration of total hospital inpatient stay in favour of the earliest operated patients (mean time 18.1, 22.0, and 28.3 days respectively; p=0.001. ANOVA showed that besides age, the only determinant of surgical outcome and duration of stay was presenting clinical grade (p<0.0005). CONCLUSION: The current management of patients presenting with SAH from anterior circulation aneurysms allows early surgery to be followed safely regardless of age. The only independent variables affecting outcome are age and clinical grade at presentation. The timing of surgery did not significantly affect surgical outcome, promoting a policy for early surgery that avoids the known risks of rebleeding and reduces inpatient stay.  (+info)