Diagnostic criteria for Buerger's disease: International Consensus of VAS - European Independent Foundation in Angiology/Vascular Medicine.

Buerger's disease (BD) remains a debilitating condition and early diagnosis is paramount for its effective management. Despite many published diagnostic criteria for BD, selective criteria have been utilized in different vascular centers to manage patients with BD worldwide. A recent international Delphi Consensus Study on the diagnostic criteria of BD showed that none of these published diagnostic criteria have been universally accepted as a gold standard. Apart from the presence of smoking, these published diagnostic criteria have distinct differences between them, rendering the direct comparison of patient outcomes difficult. Hence, the expert committees from the Working Group of the VAS-European Independent Foundation in Angiology/Vascular Medicine critically reviewed the findings from the Delphi study and provided practical recommendations on the diagnostic criteria for BD, facilitating its universal use. We recommend that the 'definitive' diagnosis of BD must require the presence of three features (history of smoking, typical angiographic features and typical histopathological features) and the use of a combination of major and minor criteria for the 'suspected' diagnosis of BD. The major criterion is the history of active tobacco smoking. The five minor criteria are disease onset at age less than 45 years, ischemic involvement of the lower limbs, ischemic involvement of one or both of the upper limbs, thrombophlebitis migrans and red-blue shade of purple discoloration on edematous toes or fingers. We recommend that a 'suspected' diagnosis of BD is confirmed in the presence of a major criterion plus four or more minor criteria. In the absence of the major criterion or in cases of fewer than four minor criteria, imaging and laboratory data could facilitate the diagnosis. Validation studies on the use of these major and minor criteria are underway.

Review of Renal Artery Stenosis and Hypertension: Diagnosis, Management, and Recent Randomized Control Trials.

Renal artery stenosis is one of the most common causes of secondary hypertension (HTN). Renal artery stenosis-induced HTN can occur in the presence of unilateral or bilateral narrowing and a solitary kidney with stenotic artery, which may subsequently lead to renal insufficiency (e.g., ischemic kidney disease) or pulmonary edema. Renal artery stenosis can be diagnosed using multiple modalities, including Doppler ultrasound, computed tomography angiography, magnetic resonance angiography, or selective angiogram. Although atherosclerotic renal artery stenosis management in patients with HTN has been greatly controversial, it is inevitable in the treatment of some selected cases. These cases can be treated by either percutaneous angioplasty (with or without stenting) or less common, open surgical approach revascularization, both of which have excellent primary patency rates. Generally, several trials on renal artery angioplasty or stenting in patients with atherosclerotic disease have shown that the long-term benefits in terms of blood pressure control and renal function over pharmacological management is not substantial. Furthermore, studies could not demonstrate a prolongation of event-free survival after renal vascularization. Moreover, endovascular procedures have substantial risks. Careful patient selection is required when considering revascularization, for including those with refractory HTN or progressive renal failure, to maximize the potential benefits. This paper discusses the epidemiology of atherosclerotic renal artery stenosis and its clinical presentation, diagnosis, treatment, prognosis, and future perspectives.

Milestones in thromboangiitis obliterans: a position paper of the VAS-European independent foundation in angiology/vascular medicine.

Even today thromboangiitis obliterans has disease features that remain misunderstood or underappreciated. The epidemiology, etiology and pathophysiology of the disease are still unclear. Biomarkers and disease activity markers are lacking, thus clinical assessment is difficult. We are still struggling to establish unique diagnostic, staging and treatment criteria. This is an academic-collaborative effort to describe the pathophysiology, the clinical manifestations, the diagnostic approach, and the challenges of management of patients with TAO. A systematic search for relevant studies dating from 1900 to the end of 2020 was performed on the PubMed, SCOPUS, and Science Direct databases. Given the intriguing nature of presentation of TAO, its management, to some extent is not only different in different regions of the world but also varies within the same region. Following this project, we discovered ambiguity, overlap and lack of clear-cut criteria for management of TAO. An international group of experts however came to one conclusion. They all agree that management of TAO needs a call for action for a renewed global look with multi-center studies, to update the geographical distribution of the disease and to establish a unique set of diagnostic criteria and a consensus-based guideline for best treatment based on current evidence.

Stroke from cervicocephalic arterial dissection in Saudi children.

Cervicocephalic arterial dissection CCAD is an important, but rarely recognized, cause of stroke in children. We describe 3 cases of CCAD who were diagnosed during a study on childhood stroke which included 104 patients. A high index of suspicion and targeted investigations are needed for the diagnosis and management of CCAD in childhood.

Moyamoya syndrome as a risk factor for stroke in Saudi children. Novel and usual associations.

OBJECTIVE: To report on moyamoya syndrome (MMS) as a risk factor for stroke in a prospective and retrospective cohort of Saudi children. The usual and novel associations of MMS in this cohort will also be described. METHODS: Children with stroke were evaluated at the Division of Pediatric Neurology at King Khalid University Hospital, College of Medicine, King Saud University, Riyadh, Kingdom of Saudi Arabia during the periods July 1992 to February 2001 (retrospective study) and February 2001 to March 2003 (prospective study). Investigations for suspected cases included hemostatic assays, biochemical, and serological tests. Neuroimaging included CT, MRI, magnetic resonance angiography (MRA), single photon computerized tomography (SPECT) brain scan and conventional cerebral angiography. RESULTS: Moyamoya syndrome was the underlying risk factor for stroke in 6 (5.8%) of the 104 children (aged one month to 12 years). They were 4 females and 2 males. Their first cerebral ischemic event occurred at a mean age of 45 months (median = 44 months, range 17-66 months). In all 6 cases, MMS was associated with an underlying hematologic abnormality or other diseases. Protein C deficiency was identified in one girl and protein S deficiency in another. Two patients had respectively, sickle cell disease (SCD) and sickle cell-beta-thalassemia (S beta-thalassemia), which had been associated in the latter with membranous ventricular septal defect. Adams-Oliver syndrome (AOS, OMIM 100300) was associated with MMS in an 18-month-old girl. A 4-year-old boy had wrinkly skin syndrome (WSS, OMIM 278250) phenotype. The association of MMS and protein C deficiency was first reported in this cohort of patients, whereas the association of the syndrome with WWS and AOS has not, hitherto, been described. The 3 patients who had MMS associated with protein C deficiency, SCD, and AOS underwent successful revascularization surgery in the form of encephaloduroarteriosynangiosis. CONCLUSIONS: Moyamoya syndrome constitutes an important risk factor of stroke in Saudi children. Comprehensive clinical evaluation and investigations, including screening for thrombophilia and neuroimaging studies, are required for the primary diagnosis of the disease and for unraveling other diseases associated with MMS. This will help in managing these patients and in guiding genetic counseling for their families.

Vascular anomalies - diagnosis and therapy.

OBJECTIVE: Vascular anomalies were once thought to be impossible to properly diagnose and treat. Hence, we aimed to evaluate the different diagnostic and therapeutic modalities in the management of vascular anomalies. METHODS: We carried out a retrospective review of our experience to evaluate different diagnostic and therapeutic modalities in the management of 25 patients with vascular anomalies over a 2-year-period at King Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia and follow-up period ranging from 2 months to 2 years. RESULTS: Vascular anomalies were more common in male patients (N=19). Age range was 7 to 46 years. Vascular anomalies were categorized as hemangioma (N=2) or malformation (N=23). The vascular malformation were further subdivided into slow flow (N=5) and fast flow (N=18). Duplex (N=12) and radiographic studies; angiography (N=21), venography (N=7), computerized tomography (N=10) and magnetic resonnance angiography (N=8) were used to confirm diagnosis. The treatment of hemangiomas were surgical resection (N=1) and conservative treatment (N=1). Embolization was the main modality of treatment in vascular malformation (N=16), with surgical resection in 4 patients, sclerotherapy in one and conservative in the other 2. All cases had successful outcome with no complications. CONCLUSION: Control of large vascular malformations with acceptable results can be achieved nowadays. Intra-arterial embolization is the mainstay of treatment and long term follow-up with serial physical examination, duplex and arteriography is required.