[Dynamic functional assessment of internal carotid artery tortuosity in patients with multifocal atherosclerosis]. / Dinamicheskaya funktsional'naya otsenka patologicheskikh izvitostei vnutrennikh sonnykh arterii u bol'nykh s mul'tifokal'nym aterosklerozom. Klinicheskii sluchai.

A personalized approach with attention to anamnesis and specific symptoms is necessary in patients with internal carotid artery tortuosity. Neuroimaging (especially before elective surgery) or functional stress tests following ultrasound of supra-aortic vessels may be necessary depending on medical history and complaints. In addition to standard Doppler ultrasound, these patients should undergo rotational and orthostatic transformation tests. We analyze changes in shape and hemodynamic parameters within the tortuosity area in various body positions. This is especially valuable for patients with concomitant carotid artery stenosis. The article presents a clinical case illustrating the importance of such approach.

[Functional assessment of internal carotid artery tortuosity in patients with multifocal atherosclerosis]. / Vazhnost' dinamicheskoi funktsional'noi otsenki patologicheskoi izvitosti vnutrennikh sonnykh arterii u bol'nykh s mul'tifokal'nym aterosklerozom.

The review is devoted to diagnosis and treatment of internal carotid artery tortuosity. The authors consider modern classification, epidemiology and diagnostic options using neuroimaging or ultrasound-assisted functional stress tests depending on medical history and complaints. In addition to standard Doppler ultrasound, rotational and orthostatic tests are advisable due to possible changes of local shape and hemodynamic parameters following body position changes, especially in patients with concomitant atherosclerotic stenosis. Thus, a personalized approach is especially important for treatment and diagnostics of internal carotid artery tortuosity.

Clinical characteristics associated with pain in patients with peripheral vascular malformations.

OBJECTIVE: Vascular malformations (VM) can negatively impact the patient's quality of life (QoL). Pain is a common problem in these patients. The aim of this study was to investigate risk factors associated with pain and to assess how pain affects QoL. METHODS: This prospective cross-sectional study was conducted in a tertiary vascular anomaly expertise center. Between June and December 2020, all patients from our local database (334 adults and 189 children) with peripheral VMs were invited to complete the Outcome Measures for VAscular MAlformations questionnaire to evaluate the presence, frequency, and intensity of pain. Additionally, patients were asked to complete several Patient-Reported Outcome Measurement Information System scales to evaluate their QoL. Risk factors associated with pain were identified in bivariate analysis and multivariable logistic regression. QoL domains were compared between patients who experienced pain and patients who did not. RESULTS: A total of 164 patients completed the questionnaire about pain and 133 patients completed all QoL questionnaires. Approximately one-half of the patients (52%) reported pain in the past four weeks and 57% of these patients reported pain daily or several times a week. Female sex (P = .009), lesions located in the upper extremity (P < .001) or lower extremity (P < .001), and intramuscular/intraosseous lesions (P = .004) were independently associated with the presence of pain. The following QoL domains were diminished in patients who experienced pain in comparison with patients who did not: pain interference (P < .001), physical functioning (P < .001), and social participation (P < .001) in adults, and pain interference (P = .001), mobility (P = .001), and anxiety (P = .024) in children. CONCLUSIONS: Pain is a frequently reported complaint in patients with VMs and is present in approximately one-half of the patients. Patients with lesions located in the upper or lower extremity, intramuscular/intraosseous lesions, and female patients are more likely to experience pain. The presence of pain negatively impacted patients' QoL. Although VM are a benign condition and expectative management is frequently applied, our study shows that pain is a serious concern and needs to be actively assessed. Pain is a sign of various etiologies and should be examined to properly treat the pain.

Efficacy and safety of oral sirolimus for high-flow vascular malformations in real clinical practice.

BACKGROUND: Vascular malformations are a complex pathology with few treatment options. In previously published studies, oral sirolimus (rapamycin) has shown promising results in the treatment of low-flow vascular malformations, but its usefulness in high-flow vascular malformations is controversial. AIM: To evaluate the efficacy and safety of sirolimus for the treatment of high-flow vascular malformations in real-life practice. METHODS: In a unit specializing in vascular anomalies, patients treated with oral sirolimus for high-flow vascular malformations were located by consulting the drug dispensations. Reviewing the electronic medical records, data on patient demographics, vascular malformation characteristics, treatments, toxicity and clinical course were collected and statistically analysed. RESULTS: Nine patients with vascular malformations were included: eight had arteriovenous malformation and one had arteriovenous fistula. Six of these malformations were isolated while three were part of a syndrome. Sirolimus was initiated at a dosage of 1-4 mg/day to be taken as a single dose. Partial response was observed in eight of the nine patients (88.9%) with high-flow vascular malformation, while worsening was observed in the remaining patient. The treatment was well tolerated and at the most recent follow-up, five patients remained on treatment with oral sirolimus. CONCLUSION: Our results show that oral sirolimus is a well-tolerated therapeutic option, with an excellent safety profile, which can be useful in the long-term stabilization of patients with high-flow vascular malformations. Single-daily dosage may improve long-term adherence to treatment without worsening its effectiveness.

Development and Preliminary Validation of the Patient-Reported Outcome Measure for Vascular Malformation Questionnaire: A Prospective Cohort Study.

PURPOSE: To develop and validate the Patient-Reported Outcome Measure for Vascular Malformation (PROVAM) questionnaire to assess the health-related quality of life in patients with vascular malformations. MATERIALS AND METHODS: We developed and validated PROVAM using a mixed methods design during a prospective clinical trial at a vascular anomalies clinic. From July 2019 to February 2020, 108 consecutive patients completed 130 questionnaires. The 30-item instrument assessed the domains of pain, emotional/social well-being, functional impact, and treatment satisfaction. Two additional items assessed ease of understanding and relevance. The primary outcomes of instrument reliability and validity were evaluated across several indices. The secondary outcome of responsiveness evaluated total score changes for patients who completed questionnaires both before and after treatment. RESULTS: Instrument reliability, as measured by Cronbach alpha, was ≥0.79 for pain, emotional/social well-being, and functional impact domains. Primary domain structure was confirmed by factor analysis (P <. 001) and convergent construct validity for all but 1 Likert scale item. In the subgroup analysis of 13 participants who completed PROVAM before and after treatment, instrument responsiveness, as measured by the total score, showed a significant decrease (median, -10 points; interquartile range [IQR], -3 to -16; P = .04). Participants found the questions easy to understand (median, 5 points; IQR, 4-5 on a 5-point scale) and relevant (median score, 4; IQR, 3-5). CONCLUSIONS: Preliminary data support the reliability and validity of PROVAM in measuring the health-related quality of life in patients with vascular malformations.

Ultrasound Evaluation of Pediatric Slow-Flow Vascular Malformations: Practical Diagnostic Reporting to Guide Interventional Management.

OBJECTIVE. This article reviews the ultrasound characteristics of pediatric slow-flow vascular malformations and underscores findings that significantly impact diagnosis and treatment. Key imaging features are discussed including lesion size, malformation location, morphology, and mimics. CONCLUSION. Ultrasound findings affect the management of slow-flow vascular malformations and should be emphasized in lesion diagnosis. Superficial, focal lesions with well-defined margins are ideal for ultrasound evaluation.

Endovascular Treatment of Persistent Sciatic Artery Occlusion: Case Report and Literature Review.

Persistent sciatic artery (PSA) is a rare congenital anatomic variant of the lower limb vascular system with highly variable presentations. The management of lower limb ischemia due to PSA disease is not specifically recommended in guidelines, and surgical by-pass is usually the most described treatment. We reported a case of a 46-year-old patient with bilateral PSA and right chronic limb-threatening ischemia due to PSA occlusion at the PSA-popliteal junction which was successfully treated with percutaneous transluminal balloon angioplasty. In addition to this case report, a systematic review of the literature regarding the endovascular management of PSA stenosis and occlusion was conducted.

Internal Carotid Artery Agenesis in a Trauma Patient with Ipsilateral Neck GSWs.

A 38-year-old man presented to the emergency room in the trauma bay for multiple ballistic injuries to the right neck. He was hemodynamically stable, protecting his airway, and neurologically intact. Computed tomography angiography (CTA) revealed absent filling the right internal carotid artery from its origin to the circle of Willis, which was intact, as well as absent petrous carotid canal on the right. The patient was diagnosed with right internal carotid artery (ICA) agenesis and discharged in several days. This report demonstrates the importance of an in-depth knowledge of vascular embryology and anatomy. The patient has agreed to have images and case details published.

Renal Artery Reconstruction for Refractory Hypertension Caused by Congenital Renal Artery Deficiency.

Renovascular hypertension is a common cause of secondary hypertension. According to the epidemiological survey, the prevalence of renovascular hypertension accounts for 1-5% of the population with hypertension. Most of the cases are associated with atherosclerosis and Fibromuscular Dysplasia (FMD). Owing to the lack of standard treatment, they will eventually develop into chronic kidney disease, which significantly affects the patient's quality of life. Hypertension is considered a prerequisite for renal artery surgery; renal function research is used to guide the treatment of unilateral lesions because endovascular intervention can only slightly improve hypertension and renal function. We advocate open surgery for patients with congenital dysplasia of renal vascular hypertension, in which the most common surgical operations are aortorenal artery bypass, renal artery endarterectomy, and renal artery replantation. This paper reports a rare case of renovascular hypertension. The patient was a 13-year-old female, and the operation was risky and complicated. He was diagnosed with a congenital absence of the right renal artery. The right renal function was recovered, and the blood pressure was well controlled after the Aorta-Right Renal Artery Bypass.

Surgical Management of Intramuscular Venous Malformations.

BACKGROUND: Intramuscular venous malformations, often erroneously called "intramuscular hemangiomas," present to pediatric orthopaedic surgeons either as a differential diagnosis of tumor or as a cause of muscle pain. Treatment options include injection sclerotherapy or surgery. There is some literature to indicate that sclerotherapy can reduce pain, but little evidence on the effectiveness of surgery. The primary aim of this study was to evaluate the efficacy of surgical resection for intramuscular venous malformations, with a secondary aim to evaluate the natural history and presentation of intramuscular venous malformations to improve clinician understanding of this condition. METHODS: A retrospective chart analysis was performed of cases identified from a vascular anomalies database from January 2004 and December 2018. Primary outcome was change in preoperative and postoperative pain. Natural history of the lesion was assessed, including age when the lesion was first noticed, when it became painful, and when it required treatment. RESULTS: Fifty-four cases were identified in the study period of which 40 underwent surgery. Pain improved in 36 of 39 patients (92.3%) who had pain before surgery and 29 (74.4%) were pain free after surgery. All 13 patients who required whole muscle excision to resect the lesion experienced an improvement in pain and 10 (76.9%) were pain free. A history of previous intervention, with surgery or sclerotherapy showed a trend towards less successful surgical outcomes. Patients presented across a wide age range from infancy to adulthood, but the most common presentation was pain with exercise between 9 and 16 years of age, with presence of a mass in about half of cases. CONCLUSIONS: Surgery, when performed by a surgeon with appropriate experience, is an effective first-line treatment for painful intramuscular venous malformations, offering pain relief in the majority of cases. Magnetic resonance imaging and ultrasound are diagnostic in most cases. The majority of lesions are resectable, meaning they can be removed with a margin leaving a functional limb. Sometimes resection of a whole muscle is required. LEVEL OF EVIDENCE: Level IV-case series.

Involvement of Microglia in the Pathophysiology of Intracranial Aneurysms and Vascular Malformations-A Short Overview.

Aneurysms and vascular malformations of the brain represent an important source of intracranial hemorrhage and subsequent mortality and morbidity. We are only beginning to discern the involvement of microglia, the resident immune cell of the central nervous system, in these pathologies and their outcomes. Recent evidence suggests that activated proinflammatory microglia are implicated in the expansion of brain injury following subarachnoid hemorrhage (SAH) in both the acute and chronic phases, being also a main actor in vasospasm, considerably the most severe complication of SAH. On the other hand, anti-inflammatory microglia may be involved in the resolution of cerebral injury and hemorrhage. These immune cells have also been observed in high numbers in brain arteriovenous malformations (bAVM) and cerebral cavernomas (CCM), although their roles in these lesions are currently incompletely ascertained. The following review aims to shed a light on the most significant findings related to microglia and their roles in intracranial aneurysms and vascular malformations, as well as possibly establish the course for future research.

Non-Dolichoectatic Vertebral Artery Compression of the Medulla: A Comprehensive Literature Review.

OBJECTIVE: Vertebral artery compression of the medulla is a rare vascular finding that causes a variety of clinical presentations, from asymptomatic to neurological disability. This article presents the largest literature review to date on medullary compression of the vertebral arteries. METHODS: An English literature search was performed using the PubMed database and the keywords vertebral artery tortuosity, vertebral artery compression, and medullary compression. RESULTS: A comprehensive literature search yielded 68 patients (57% male) with medullary compression by an intracranial vertebral artery (ICVA). The left side of the medulla was compressed in 44, the right side in 19, and bilateral in 7. The most common clinical symptom was weakness - 26 patients (36%) - 6 had quadriparesis and 6 had hemiparesis. 21 patients reported imbalance; 12 various sensory symptoms; 4 patients were asymptomatic. CONCLUSIONS: Understanding the anatomy of the vasculature can help mitigate future debilitating stroke symptoms. Concrete guidelines for revascularization surgery in symptomatic patients may also be effective. Future studies are needed to further clarify the prevalence, natural history, vascular etiology, and treatment of this condition, including asymptomatic patients and the likelihood that they will develop further neurological signs and disability.

CT Angiography of Agenesis of the Left Internal Carotid Artery.

Online supplemental material is available for this article.

Efficacy and safety of sirolimus in the treatment of vascular anomalies: A systematic review.

OBJECTIVE: The management of vascular anomalies is complex and requires a multidisciplinary team with a combination of medical, surgical, and intervention treatments. Medical treatment is limited and has conflicting results. Off-label use of mammalian target of rapamycin inhibitors shows promising results. The objective of this study was to systematically evaluate the literature published about the efficacy and safety of sirolimus in the treatment of vascular anomalies. METHODS: A systematic review of the published literature was conducted using the PubMed database and Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. RESULTS: There were 73 articles included: 2 randomized controlled studies, 2 nonrandomized prospective studies, and 69 retrospective case reports and case series. In total, 373 patients were included. Sirolimus was administered topically to 56 patients and orally to 317 patients. Sirolimus was highly effective in the treatment of vascular tumors associated with Kasabach-Merritt phenomenon (95.5% of the patients clinically improved and 93% had normalization of coagulopathy), venous malformations (size reduction was observed in 88.9% of patients), and lymphatic malformations (clinical improvement in 94.9% of patients). Topical sirolimus results were conflicting. Arteriovenous malformations were not improved by sirolimus. CONCLUSIONS: Low-level evidence suggests that sirolimus can improve the prognosis of vascular anomalies, most notably vascular tumors associated with life-threatening coagulopathy and venous and lymphatic malformations. Further research is needed to establish the benefits of sirolimus in the management of vascular anomalies.

Abernethy Malformations: Evaluation and Management of Congenital Portosystemic Shunts.

PURPOSE: To assess the utility of preoperative venography in evaluating and managing patients with congenital portosystemic shunts (CPSSs). MATERIALS AND METHODS: A retrospective study was performed of 42 patients (62% female; median age, 4.1 years) diagnosed with a CPSS from 2005 to 2018. Preoperative venography (n = 39) and balloon occlusive pressure measurements (n = 33) within the mesenteric venous system guided treatment. Primary outcome was serum ammonia levels at 1 month after shunt closure. Management strategies included single (n = 12) or staged (n = 18) operative ligation, endovascular occlusion (n = 8), combined surgical and endovascular closure (n = 2), and observation (n = 2). RESULTS: At 1 month, serum ammonia levels decreased from 82.5 ± 10.3 µmol/L to 38.4 ± 4.6 µmol/L (P < .001). No difference was observed in the decrease between patients treated surgically or endovascularly (P = .91). Mean occluded to non-occluded pressure gradients were significantly lower for endovascular closure (5.3 ± 1.8 mmHg) than for surgical closure (12.3 ± 3.3 mmHg, P = .02). Shunts were classified as extrahepatic in 29 patients and as intrahepatic in 13 patients; all shunts demonstrated filling of the portal system with occlusive venography. Broad and short shunts were closed surgically; narrow and long shunts were closed endovascularly. Shunts were closed in a single session (n = 20) if the pressure gradient was less than 10 mmHg and the occluded mesenteric pressure was less than 25 mmHg. CONCLUSIONS: Preoperative venography delineates shunt morphology, and balloon occlusion simulates closure hemodynamics. This information is necessary to determine whether definitive closure should be performed through endovascular or surgical methods and whether closure should be performed in a single or staged setting.

CT Angiographic Evaluation of Congenital Anastomoses between Femoropopliteal Vein and Deep Femoral Vein: Types and Incidence.

PURPOSE: To evaluate congenital anastomotic channels between femoropopliteal veins and deep femoral veins with the use of computed tomographic (CT) venography. MATERIALS AND METHODS: CT venography of 488 limbs in 244 patients (105 men; mean age, 61 y; age range, 18-96 y) performed between January 2016 and December 2016 was retrospectively evaluated. The presence or absence of anastomotic channels (> 3 mm in diameter) connecting a femoropopliteal vein to a deep femoral vein was determined, and the observed channels were classified based on their location and course. RESULTS: Two types of anastomotic channels were observed in 32 patients (13%): a persistent sciatic vein (PSV) in 24 limbs of 15 patients (6%) and a retrofemoral channel (RFC) in 22 limbs of 19 patients (8%). A PSV anastomosed with the popliteal vein in the popliteal fossa, coursed posterior to the adductor magnus muscle, and drained into the deep femoral vein. An RFC anastomosed with the femoral vein at the level of the adductor hiatus, coursed posterior to the femoral shaft, and drained into the deep femoral vein. PSVs and RFCs were of similar size or larger than adjacent femoropopliteal veins in 50% (12 of 24) and 22% of limbs (4 of 22), respectively. CONCLUSIONS: Anastomotic channels connecting a femoropopliteal vein to a deep femoral vein are not a rare variation and may have an important role in collateral venous drainage of the lower extremities.

Hepatopulmonary Syndrome in an Adolescent With Insidious Hypoxia and Small Intrahepatic Portal Venous Shunts: Posttransplant Benefit From Sildenafil.

We report a patient without known preexisting liver disease who presented with hepatopulmonary syndrome (HPS) due to aberrant intrahepatic portal venous development leading to portosystemic shunting. Liver transplantation resulted in resolution of portal hypertension and HPS and sildenafil was safely tolerated in the treatment of persistent fatigue and hypoxemia. Twelve months later, patient has normal allograft function and has returned to normal activity.

Major Limb Amputations in Patients with Congenital Vascular Malformations.

BACKGROUND: The need for major amputations in patients with vascular malformations is rare. This study reviews our contemporary experience with major amputations in patients with vascular malformations. METHODS: A retrospective review from April 2014 to November 2018 identified 993 patients undergoing management of a vascular malformation involving the upper or lower extremity at a tertiary center. This population was analyzed to identify those requiring either a transfemoral or transhumeral amputation. This cohort was investigated for clinical course, surgical procedures, and outcomes. RESULTS: Five patients (0.5%) underwent major amputation, including 3 transhumeral and 2 above-knee amputations. The median age was 37.8 years (interquartile range (IQR): 25.4-40.2), and 2 (40%) were male. Four (80%) patients had high-flow arteriovenous malformations, including 1 (20%) with Parkes-Weber syndrome. One (20%) patient had a low-flow venous malformation associated with Klippel-Trénaunay syndrome. All patients had malformation extending into the chest or pelvis, with the amputation being at the level of residual malformation. As such, amputation had been initially felt to be high risk because of the proximal extent of the lesions. Before amputation, a median of 11 procedures (IQR: 4-39) were performed per patient. This included 29 transarterial embolizations, 4 transvenous embolizations, 20 direct stick embolizations, 3 debulking procedures, 38 debridements, 6 skin grafts or muscle flaps, and 4 minor amputations. The median time course of treatment before amputation was 117 months (IQR: 44-171). Indications for major amputation included chronic pain and recurrent bleeding in all 5 (100%) patients, loss of function in 2 (40%), nonhealing wounds in 2 (40%), and sepsis in 1 (20%) patient. There were no perioperative deaths. The median blood loss was 1,000 mL (IQR: 650-2,750). All patients required transfusion of packed red blood cells with a mean of 1.6 units (standard deviation: 0.54). Transhumeral amputation was facilitated by transcatheter embolization in 1 (33%) and an occlusion balloon within the subclavian artery in 2 (66%) patients. The median length of stay was 6 days (IQR: 5-13). The median length of follow-up was 132 months (IQR: 68-186) from initial intervention and 12 months (IQR: 8-31) from amputation. Two patients (40%) who had undergone transhumeral amputation required revision of the amputation site for recurrent ulceration at 2 and 38 months. Of these, 1 patient underwent 3 transcatheter embolization procedures before revision and 1 underwent 1 embolization after revision. CONCLUSIONS: Although rare, successful amputation at the level of residual malformation can be performed in select patients with refractory complications of vascular malformations including intractable pain, bleeding, or nonhealing wounds. Specific preoperative and intraoperative measures may be critical to achieve satisfactory outcomes, and endovascular techniques continue to play a role after amputation.

Surgical Ligation for the Treatment of an Unusual Presentation of Type II Abernethy Malformation.

Abernethy malformation is a very rare congenital vascular malformation consisting of diversion of portal blood away from liver, and it is commonly associated with multiple congenital anomalies. Here, we present a case of a male from China with nonspecific abdominal pain associated with an unusual pattern of type II Abernethy malformation, whose was diagnosed with a portosystemic shunt via a giant portal-inferior vena cava fistula (17.22 mm in diameter). The patient underwent a surgical ligation of the portocaval shunt and recovered well. We believe that this is the first case of a type II Abernethy malformation presenting as a portosystemic shunt via the giant portal-inferior vena cava fistula.

INSIGHTS INTO PERIFOVEAL EXUDATIVE VASCULAR ANOMALOUS COMPLEX.

PURPOSE: To report a series of eight patients with perifoveal exudative vascular anomalous complex imaged with optical coherence tomography angiography and the results of anti-vascular endothelial growth factor therapy or laser photocoagulation. METHODS: Retrospective analysis of demographic data, imaging including color pictures, spectral domain optical coherence tomography, and optical coherence tomography angiography, and fluorescein angiography, course, and outcome. RESULTS: Age at onset ranged from 45 to 84 years (mean ± SD: 68.6 ± 13.7). Five cases were initially misdiagnosed. The perifoveal exudative vascular anomalous complex lesion was unique in seven eyes and located predominantly in the superficial capillary plexus in two eyes, strictly in the deep capillary plexus in two eyes, but observed at the level of both plexi (3 eyes). One patient presented two lesions, one in the superficial capillary plexus and one in the deep capillary plexus. Capillary rarefaction was observed around the lesion in six eyes. Sustainable resolution of exudation could be achieved in 2 patients, one after 2 sessions of focal thermal laser photocoagulation and one after 13 intravitreal injections of anti-vascular endothelial growth factor. CONCLUSION: The present series confirms that perifoveal exudative vascular anomalous complex corresponds to a new entity that differs from other conditions associated with capillary aneurysmal lesions. Visual improvement could be obtained after treatment with focal laser or intravitreal anti-vascular endothelial growth factor agents.