Peripheral Vascular Anomalies Research Articles

A Case of Arteriovenous Malformation in the External Auditory Canal

Benign vascular tumors are relatively common in the head and neck region, but are rarely found in the ear. Among vascular tumors, infantile hemangioma stands as the prevailing entity, characterized by a rapid initial proliferation of endothelial cells, succeeded by a gradual regression over the course of several years. In contrast, arteriovenous malformation (AVM) is a less common phenomenon, originating from peripheral vascular anomalies during embryogenesis, and it does not undergo spontaneous regression. Specifically, the occurrence of AVM within the external auditory canal are exceedingly uncommon. Here, we describe a case of a 49-year-old male patient who had a mass incidentally discovered in the external auditory canal, but refused initial treatment. He later returned as the mass enlarged. This case could have been clinically misdiagnosed as a hemangioma, but was ultimately diagnosed pathologically as an AVM.

Safety and Efficacy of Prestige Coils for Embolization of Vascular Abnormalities: The Embo-Prestige Study.

A wide variety of coils are available for vascular embolization. This study aimed to evaluate the safety and efficacy of a new Prestige coil. We carried out retrospective analysis of a multicenter's registry data collected between February 2022 and November 2022. The choice of embolization agent used to treat peripheral vascular anomalies was left to the investigator's discretion. Patients for whom at least one Prestige coil was used were included in Series 1. All other patients were included in Series 2. Efficacy and safety were evaluated. Patients were followed up for one month. In total, 220 patients were included, 110 in each series. Patients included 149 men (67.7%) and 71 women (32.3%), with a median age of 62.5 years (IQR: 35.8-73). Patient ages were similar in the two series. Complete occlusion of the targeted vessel was reported in 96.4% (n = 106/110) of patients in Series 1 and in 99.7% (n = 109/110) in Series 2. Four patients experienced non-serious adverse events (1.8%, n = 4/220): one experienced back pain and one vomiting in Series 1; one patient had off-target embolization and one a puncture site hematoma in Series 2. Sixteen patients (7.2%, n = 16/220) were lost to follow up. Improvement in the patient's general state at one month was reported in 79.0% (n = 83/105) of patients in Series 1 and in 74.7% (n = 74/99) in Series 2. Ten deaths occurred, five in Series 1 (4.8%, n = 5/105) and five in Series 2 (5.1%, n = 5/99). These deaths all concerned critically ill patients embolized for emergent arterial bleeding. In conclusion, the 1-month follow-up showed that Prestige coils, alone or in combination, are efficient and safe.

Percutaneous MR Imaging-Guided Laser Ablation and Cryoablation for the Treatment of Pediatric and Adult Symptomatic Peripheral Soft Tissue Vascular Anomalies

PurposeTo evaluate the safety and effectiveness of percutaneous magnetic resonance (MR) imaging-guided laser ablation and cryoablation for the treatment of symptomatic soft tissue vascular anomalies (VAs) of the trunk and extremities. Materials and MethodsAn institutional review board-approved retrospective review was undertaken of all pediatric and adult patients who underwent MR imaging-guided and monitored laser ablation and/or cryoablation for the treatment of symptomatic peripheral soft tissue VA. Preablation and postablation MR imaging was independently reviewed. Pain scores on the visual analog scale (0 to 10) and self-reported subjective symptomatic improvement were assessed. Change in VA size and pain scores were compared using a paired t test. ResultsThirty patients (24 females; age, 10–75 years) with 34 VAs were treated for moderate to severe pain (n = 27) or swelling/mass effect (n = 3) with 60 total ablation sessions. The baseline maximum VA diameter was 9.5 cm ± 8.6. At baseline, all VAs (100%) demonstrated variable T2-weighted signal hyperintensity and enhancement. The baseline pain score was 6.4 ± 1.6. Clinical follow-up was available for 23 patients. At a mean follow-up time of 12.2 months ± 10.1, 19 of 20 (95%) patients treated for pain and 2 of 3 (67%) patients treated for swelling/mass effect reported partial or complete symptomatic relief. There was a significant decrease in the postablation pain scores (−5.7 ± 1.0, P < .001) and maximum VA size (−2.3 cm ± 2.7, P = .004), with >50% reduction in VA T2 signal (59%) and enhancement (73%). Nine of 30 (30%) patients experienced minor complications. ConclusionsMR imaging-guided and monitored percutaneous laser ablation and cryoablation appear to be safe and effective for the treatment of symptomatic peripheral soft tissue VAs.

Retinal Features of Family Members With Familial Exudative Vitreoretinopathy Caused By Mutations in KIF11 Gene.

PurposeTo determine the clinical characteristics of patients and family members with familial exudative vitreoretinopathy (FEVR) caused by mutations in the KIF11 gene.MethodsTwenty-one patients from 10 FEVR families with mutations in the KIF11 gene were studied. The retinal and systemic features were examined. The genetic analyses performed included Sanger sequencing of the KIF11 gene, whole exome sequencing, as well as array comparative genomic hybridization (CGH) analysis and multiple ligation probe assay (MLPA).ResultsSequence analysis revealed seven different KIF11 mutations. Array CGH with MLPA revealed two different exon deletions. All probands had advanced FEVR with retinal detachments (RDs) and microcephaly with or without developmental disabilities. Patients with bilateral RDs were more frequently associated with developmental disabilities (P = 0.023). Multimodal imaging of the family members revealed that six of nine patients without RDs (66%) had varying degrees of chorioretinopathy. The retinal folds in FEVR patients were associated with severe retinal avascularization. However, funduscopic changes in the peripheral retina were unremarkable in family members without RDs. A score representing the peripheral vascular anomalies determined from the fluorescein angiograms was lower than that of control eyes of patients with mutations of the Wnt signaling genes (P = 0.0029).ConclusionsThe probands with KIF11 mutations were associated with severe ocular and systemic pathologies, whereas affected family members showed highly variable clinical manifestations. Peripheral vascular anomalies can often be unremarkable in eyes without RDs.Translational RelevanceThese findings highlight more diverse mechanisms that underlie the pathological changes in patients with FEVR.

Limited surface examination to evaluate potential teratogens in a resource-limited setting.

To determine the frequency of malformations that would be identified in the limited surface examination of a newborn by the delivering nurse midwife in a resource-limited setting. The limited surface examination will identify visible external anomalies, but not abnormalities inside the mouth, most heart defects, undescended testes, inguinal hernias, hip dysplasia, peripheral vascular anomalies, and some internal anomalies. The findings in a malformations surveillance program, involving 289,365 births in Boston, have been used to establish the prevalence rate of malformations that would be identified and not identified. In African countries, the number of anomalies to be identified should also be reduced by excluding polydactyly, postaxial, type B, a common minor finding, from the list of potential malformations. Of note, 2.05% (n = 5,941) of the 289,365 births surveyed had one or more malformations. The abnormalities that would have been missed, using surface exam alone, accounted for 0.5% of all of malformations identified and reduced the overall prevalence rate of malformations to 1.5%. In addition, excluding all infants with isolated postaxial polydactyly, type B reduced the expected prevalence rate of malformations to 1.3% in unexposed newborn infants. A limited surface examination can detect the majority of malformations among newborn infants.

The spectrum of genetic mutations in patients with asymptomatic mild familial exudative vitreoretinopathy

Familial exudative vitreoretinopathy (FEVR) is a disease exhibits a wide range of clinical signs, ranging mild peripheral retinal vascular anomalies to severe retinal detachments. Individuals with mild FEVR are frequently asymptomatic with good visual function and are often undiagnosed. However, little is known about the genetic characters of the cohort. The purpose of this study was to investigate the clinical characteristics and genetic spectrum of in patients with asymptomatic mild FEVR. Herein, sixty-two patients (124 eyes) with asymptomatic mild FEVR were studied in a case series. Comprehensive ophthalmic examinations and genetic testing were performed in all patients. Clinical examinations showed that the avascular zone was seen in all 124 eyes and was the most common abnormality observed. Increased vessel branching and straightened peripheral vessel branches were found in 122 (98.4%) eyes. Late-phase angiographic posterior and peripheral leakage (LAPPEL) was observed in 80 (64.5%) eyes and V-shape degeneration was noted in 36 (29.0%) eyes. Other manifestations including extensive anastomoses, retinal ridges, and extraretinal neovascularization, which were detected in 30 (24.2%), 10 (8.1%) and 2 (1.6%) eyes respectively. Overall, pathogenic mutations were identified in 48.4% (30/62) of individuals with asymptomatic mild FEVR. Mutations in FZD4, LRP5, TSPAN12, and KIF11 were detected in 21.0% (13/62), 12.9% (8/62), 12.9% (8/62), and 1.6% (1/62) of our patients respectively. Ten novel mutations were found. In conclusion: Pathogenic mutations in the known FEVR-associated genes were detected in nearly half (48.4%) of the asymptomatic mild FEVR cohort. Among these mutations, FZD4 was predominant, appearing in 21.0% of all individuals. Patients with asymptomatic mild FEVR should receive timely examinations, lifelong monitoring, and some of them need preventive therapy and treatment. Additionally, we discovered 10 novel variants, which may enable a deeper understanding of this disease.

Screening and Follow-Up of Acute ROP: Reproducibility of Fluorescein Angiography

Binocular indirect ophthalmoscopy (BIO) is fundamental for screening of retinopathy of prematurity (ROP). Digital retinal imaging devices with fluorescein angiography (FA) proved to be useful in screening and management of ROP. FA provides valuable additional information that is not detectable through ophthalmoscopy. FA images are relatively easy to interpret even by personnel without specific experience in ROP. The aim of this study is to evaluate reproducibility of FA for the screening and follow-up of ROP. A total of 106 pairs of FA images of 30 eyes of 15 premature infants with stage II ROP were evaluated by 5 ophthalmologists: 2 experts, 2 non-experts, and 1 expert in reading FA in adult patients. Each operator gave a score to each of following parameters: leakage, ischemic areas, peripheral plus disease and vascular anomalies. The images were reviewed twice. Intra- and inter-concordance between the readers of the FA findings was evaluated by the means of Cohen's kappa coefficient (κ). The intra-operator concordance was very good (κ > 0.81) for all FA findings. Inter-operator concordance was good (κ> 0.41) for all operators and all FA findings. Global concordance was: substantial (intra-inter readers: κ > 0.61) for leakage, ischemic areas, and plus disease; almost perfect (κ> 0.81) for vascular anomalies; and moderate (κ = 0.41-0.60) for continuity/discontinuity of the ischemic areas. Total FA score was directly correlated to the percentage of treatment: a score ≥ 7 was correlated with 100% treatment and a score ≤ 3 with no treatment. Treatment timing was inversely correlated to FA score: a score ≥ 8 was correlated with a timely treatment (≤ 6days), and a score ≤ 7 was correlated with a delayed treatment (< 10days). This study showed that FA represents a reproducible imaging technique. It is useful for detecting ROP progression, and to define the treatment timing and type.

Fluorescein Angiographic Findings of Peripheral Retinal Vasculature after Intravitreal Conbercept versus Ranibizumab for Retinopathy of Prematurity.

Purpose To evaluate angiographic findings of peripheral retina vasculature in retinopathy of prematurity (ROP) neonates who received intravitreal conbercept (IVC) or ranibizumab (IVR). Methods Fluorescein angiography (FA) findings were retrospectively evaluated for ROP neonates who received IVC or IVR. Outcome measures included peripheral avascular zone, vascular leakage, vascular blunting, vascular loops, vascular dilatation, arteriovenous shunt, and capillary dropout. Results Fifty-four eyes (28 patients) with ROP were included. Twenty-nine eyes (15 patients) received IVC, and 25 eyes (13 patients) received IVR. For infants of the IVC group, the mean gestational age, birth weight, and postmenstrual age (PMA) at the initial treatment were 28.96 ± 2.36 weeks, 1168.8 ± 344.5 g, and 41.22 ± 4.39 weeks, respectively. For the IVR group, they were 28.83 ± 2.34 weeks, 1255.0 ± 356.9 g, and 39.42 ± 2.77 weeks, respectively (P=0.817, 0.522, and 0.075). For the IVC group, FA performed at 71.29–115.43 weeks PMA showed 96.55% of eyes had avascular zone; vascular leakage was found in 24.14% eyes; vascular blunting, vascular dilation, vascular loops, arteriovenous shunt, and capillary dropout were found in 96.55%, 72.41%, 79.31%, 48.28%, and 68.97% eyes, respectively. For the IVR group, FA performed at 65.57–133.71 weeks PMA showed 92.0% of eyes had avascular zone; vascular leakage was found in 40.0% eyes; vascular blunting, dilatation, loops, arteriovenous shunt, and capillary dropout were found in 100%, 60.0%, 64.0%, 36.0%, and 68.0% eyes, respectively. Conclusion No significant difference can be observed between the IVC group and IVR group for peripheral vascular structure anomalies with FA evidence. FA studies should be considered to assess the status of the peripheral retinal vasculature to determine therapeutic outcomes and potential functional outcomes.

Peripheral Vascular Anomalies - Essentials in Periinterventional Imaging.

Peripheral vascular anomalies represent a rare disease with an underlying congenital mesenchymal and angiogenetic disorder. Vascular anomalies are subdivided into vascular tumors and vascular malformations. Both entities include characteristic features and flow dynamics. Symptoms can occur in infancy and adulthood. Vascular anomalies may be accompanied by characteristic clinical findings which facilitate disease classification. The role of periinterventional imaging is to confirm the clinically suspected diagnosis, taking into account the extent and location of the vascular anomaly for the purpose of treatment planning. In accordance with the International Society for the Study of Vascular Anomalies (ISSVA), vascular anomalies are mainly categorized as slow-flow and fast-flow lesions. Based on the diagnosis and flow dynamics of the vascular anomaly, the recommended periinterventional imaging is described, ranging from ultrasonography and plain radiography to dedicated ultrafast CT and MRI protocols, percutaneous phlebography and transcatheter angiography. Each vascular anomaly requires dedicated imaging. Differentiation between slow-flow and fast-flow vascular anomalies facilitates selection of the appropriate imaging modality or a combination of diagnostic tools. Slow-flow congenital vascular anomalies mainly include venous and lymphatic or combined malformations. Ultrasound and MRI and especially MR-venography are essential for periinterventional imaging. Arteriovenous malformations are fast-flow vascular anomalies. They should be imaged with dedicated MR protocols, especially when extensive. CT with 4D perfusion imaging as well as time-resolved 3D MR-A allow multiplanar perfusion-based assessment of the multiple arterial inflow and venous drainage vessels of arterio-venous malformations. These imaging tools should be subject to intervention planning, as they can reduce procedure time significantly. Fast-flow vascular tumors like hemangiomas should be worked up with ultrasound, including color-coded duplex sonography, MRI and transcatheter angiography in case of a therapeutic approach. In combined malformation syndromes, radiological imaging has to be adapted according to the dominant underlying vessels and their flow dynamics. Guide to evaluation of flow dynamics in peripheral vascular anomalies, involving vascular malformations and vascular tumors with the intention to facilitate selection of periinterventional imaging modalities and diagnostic and therapeutic approach to vascular anomalies. · Peripheral vascular anomalies include vascular malformations and vascular tumors. Both entities represent a rare disease with an underlying congenital mesenchymal or angiogenetic disorder. · The role of periinterventional imaging is confirmation ofthe diagnosis by assessing the flow dynamics of the vascular anomaly. · Slow-flow congenital vascular anomalies include venous, lymphatic and venolymphatic malformations. Arteriovenous malformations are fast-flow vascular anomalies, whereas hemangiomas are fast-flow vascular tumors that are frequently associated with fast-flow arteriovenous shunts. The periinterventional imaging modalities of choice include dedicated MR protocols and CT with 4D perfusion imaging as well as invasive transcatheter angiography.. · Sadick M, Overhoff D, Baessler B et al. Peripheral Vascular Anomalies - Essentials in Periinterventional Imaging. Fortschr Röntgenstr 2020; 192: 150 - 162.

Value of ultrasonography in diagnosing peripheral intraneural vascular anomalies

Objective To evaluate the value of ultrasonography in diagnosing peripheral intraneural vascular anomalies. Methods The ultrasonographic images of 10 patients with peripheral intraneural vascular anomalies confirmed by surgery or pathology were retrospectively analyzed. The ultrasonographic features were summarized. Results The ultrasonographic images could not only clearly show the location, involvement and blood supply of peripheral intraneural vascular anomalies, but also determined the classification of them. Ultrasonography showed enlarging nerves, within which tubular structures could be observed, and the boundary between the vascular malformations and the perineurium was not clear. Abundant arteriovenous blood flow (arteriovenous malformation) or low velocity venous blood flow (venous malformation) within the vascular anomalies could be detected by color Doppler examination. Conclusions Ultrasonography is the preferred imaging method for diagnosis of peripheral intraneural vascular anomalies. Key words: Ultrasonography, Dopper, color; Peripheral nerve; Vascular malformations

MRI evaluation of peripheral vascular anomalies using time-resolved imaging of contrast kinetics (TRICKS) sequence.

We describe the use of time-resolved imaging of contrast kinetics (TRICKS) sequence in the diagnosis of Peripheral Vascular Anomalies. In case of suspected vascular malformations time-resolved MR angiography might add important information for therapeutic decisions and follow-up. The objective of our study was to assess the usefulness and diagnostic performance of time-resolved imaging of contrast kinetics sequence in the evaluation of peripheral vascular anomalies. Sixty-six patients (23 pediatric, 43 adult; mean age 26) affected by upper or lower limb vascular anomalies and studied using time-resolved imaging of contrast kinetics sequence were prospectively evaluated. All studies were performed on a 1.5-T whole-body MR system. Two independent readers tried to categorized the suspected vascular anomalies in pre-contrast and post-contrast MR sequences and assessed the overall TRICKS image quality. In 11 patients, the diagnostic performance comparability between TRICKS sequence and digital subtraction angiography was evaluated. On the basis of time-resolved imaging of contrast kinetics, 31 of the vascular anomalies were classified as high-flow vascular malformations, 29 as low-flow vascular lesions and 6 as hemangiomas. There was no significant difference in image quality evaluation and vascular anomaly classification between the two observers. The vascular anomalies characteristics provided by moderate, good or excellent quality TRICKS images were confirmed by digital subtraction angiography. Time-resolved imaging of contrast kinetics sequence let the radiologist to acquire useful temporal information to correctly evaluate vascular anomalies components, adding more data to those provided by conventional MR sequences, especially in case of arteriovenous malformation. Therefore, both in pediatric and adult population, TRICKS could be used as an additional initial diagnostic tool to rightly classify these lesions and evaluate if a treatment is needed and which.

Abstract No. 116 - MRI-guided laser ablation for symptomatic, peripheral soft-tissue vascular anomalies: feasibility, safety, and effectiveness in the first 19 patients

Abstract No. 116 - MRI-guided laser ablation for symptomatic, peripheral soft-tissue vascular anomalies: feasibility, safety, and effectiveness in the first 19 patients

ROP laser treatment based on fluorescein angiography classification

PurposeTo report laser treatment of babies with retinopathy of prematurity (ROP) based on diagnostic signs in the fluorescein angiography images (FA). FA reliability and accuracy were previously demonstrated [EJ opht 2013; 23(6):881–886].MethodsThis is a cross‐sectional study with retrospective diagnostic data from 72 eyes of 36 premature infants (average gestational age: 26 weeks; average birth weight: 742 g) with Type 1 ROP stage 2 evaluated by RetCam3 and FA. RetCam funduscopic images were compared with FA images. A grid with ocular fundus divided into 3 concentric zones (ICROP 1984–87) and into 4 quadrants centered on optic nerve was superimposed on 360° retina photomontages (Photoshop) obtained from the RetCam and FA images. The FA diagnostic signs included leakage, ischemic areas, peripheral plus disease and vascular anomalies. These signs were not detectable in the RetCam images of the same patients. A scoring system was derived considering the presence (1) or absence (0) of the diagnostic signs. The scores of each single sign were summed to obtain a total score for each quadrant. These scores were then summed to get the global score of each eye. Three different fluorangiographic categories were defined in ROP stage 2 (fundoscopic image): mild (score &lt; 4), medium (4 ≤ score ≤ 10) and severe (score &gt; 10).The decision of either laser‐treatment or observation was made based on this FA classification.Results58 eyes were classified as severe stage 2, 22 as medium and 14 as mild. Complete (360°) and partial (90° or 180°) laser‐treatments were performed in severe and medium ROP, respectively; for mild ROP closely observation was preferred.ConclusionsFA classification is a useful tool to detect early signs of ROP progression to threshold disease. It improves treatment timing and extension in order to obtain the best anatomical and functional outcomes.

Classification, Imaging, and Interventional Procedures in Peripheral Congenital Vascular Anomalies

: Classification of vascular lesions is often confusing and various systems have been described. Pathologically, they can be classified as capillary, cavernous, venous, arteriovenous, lymphatic, and mixed types. Another pathologic classification describes them in two groups including hemangiomas and vascular malformations. Vascular lesions can also be classified for low and high flow lesions depending of blood flow velocity in the lesion. Although vascular lesions are congenital but they may not be evident until adulthood. Imaging modalities that can be used for evaluation of vascular anomalies include: conventional radiography, ultrasonography, CT Scan, MRI, catheter angiography, CT and MR angiography. MRI is the most valuable modality in the classification and diagnosis of vascular lesions. Other modalities also have important roles in diagnosis of problematic lesions and also in interventional procedures such as percutaneous treatment. Most of vascular lesions can be treated conservatively, but some of them especially high flow lesions need interventional treatment and in such lesions, usually a multidisciplinary approach is necessary. Embolization and sclerotherapy are the most used interventional techniques. The aim of this review is describing not only the multiple classification systems of peripheral vascular anomalies, but also more importantly the diagnostic role of different imaging modalities and also embolization or sclerotherapy planning for control of them.

High Prevalence of Peripheral Retinal Vascular Anomalies in Family Members of Patients with Familial Exudative Vitreoretinopathy

To describe the prevalence and severity of familial exudative vitreoretinopathy (FEVR) in asymptomatic relatives of known symptomatic FEVR patients. Uncontrolled and retrospective case series at a single tertiary referral vitreoretinal practice. A total of 148 eyes of 74 subjects were studied. A retrospective chart review was conducted of patients with a diagnosis of FEVR between January 2011 and January 2013 at a single tertiary care retina practice. Data were collected from patient charts, including sex, gestational age at birth, age at presentation, referring diagnosis, family history, prior ocular surgery, clinical presentation, and diagnostic imaging in each eye. Inclusion criteria included confirmed clinical diagnosis of FEVR in patients referred to our clinic for evaluation of decreased vision. Patients were excluded if a definitive diagnosis of FEVR could not be made. Clinical and angiographic findings. A total of 74 subjects from 17 separate families met the inclusion criteria for this study. There were an average of 4.4 subjects per family included in this study. The cohort was 55% male and included 17 patients and 57 family members who agreed to undergo genotyping, examination, and diagnostic imaging. Forty-three percent of FEVR patients had detectable mutations in FZD4, NDP, or TSPAN12. Only 8% of the cohort reported a positive family history of FEVR in a first-degree relative. Among the index patients, 76% had clinical stage 3, 4, or 5 FEVR and 24% had stage 1 or 2 FEVR. Among the asymptomatic family members screened, 58% demonstrated clinical or angiographic findings consistent with stage 1 or 2 FEVR and 21% demonstrated clinical or angiographic findings consistent with stage 3, 4, or 5 FEVR. Asymptomatic family members of FEVR patients frequently have early manifestations of FEVR (stage 1 or 2). Early-stage FEVR may progress to more advanced stages, which can result in vision loss. These data support the use of angiographic screening and clinical examination in immediate relatives of patients with symptomatic FEVR.

Asymptomatic aneurysm of the proximal right subclavian artery: a rare ultrasound diagnosis

True aneurysms of otherwise normal subclavian arteries are uncommon peripheral vascular anomalies. Most patients with subclavian artery aneurysms are symptomatic by presenting neurologic signs. We report a young woman who had an asymptomatic true aneurysm of the right subclavian artery assumed to be of congenital origin. This case is unique in that the aneurysm was in the extremely rare anatomic location of the right supraclavicular fossa between the origins of the right subclavian artery and the vertebral artery. Aneurysms of the right subclavian artery may represent a potential pitfall in conventional gray-scale ultrasound of the neck particularly the supraclavicular fossa. Differential diagnosis includes cervical cyst, pharyngo-esophageal diverticulum, vascular anomalies, struma, enlarged lymph node, as well benign or malignant neoplasms. Color duplex ultrasound should be performed as the method of choice for further analysis of suspected aneurysms. In this report the role of B-mode ultrasound and color duplex ultrasound is discussed in relation to digital subtraction- and MR angiography in confirmation of the diagnosis.

Intermittent Claudication.

Pain from intermittent claudication can impair physical activity. A directed history, physical exam, and noninvasive tests can help physicians detect peripheral vascular anomalies among patients. Management includes risk factor modification, exercise, foot care, and lipid-lowering therapies.

Intracranial arterial aneurysms in children. Clinical, neuroradiological and histological findings

Of our five cases of intracranial arterial aneurysms in children, there were two typical saccular aneurysms and three with fusiform or large peripheral vascular anomalies. Angiographic and histological examinations pointed to an additional arteriovenous malformation in the area of the aneurysm in two cases, combined in a third case with a cutaneous hemangioma of the brow. Other characteristics were typical of aneurysms in children such as low frequency, male preponderance, and location at the internal carotid artery bifurcation. Large peripheral aneurysms are not unusual in childhood. However, neuroradiological and neuropathological criteria reveal that such 'aneurysms' are often part of a complex arteriovenous malformation as has been presumed by some authors.

Systemic Hypertension Secondary to Peripheral Vascular Anomalies in Patients With Williams Syndrome

ng/ml , n = 20). Although whole blood serotonin concentrations may decrease with increasing age, 1~ values in patient 1 were also elevated compared with those in an age-matched normal pediatric population we are studying (values to be reported elsewhere). Elevated serotonin levels in children with Williams syndrome adds to the growing list of disorders that have evidence of central nervous system serotonergic dysfunction? In summary, medical, developmental, and psychologic data have been described in two children with Williams syndrome and autism. The documentation and comparison of specific abnormalities in autistic Children with different associated organic conditions may help to delineate the neurobiologic cause of both the behavioral phenotype and the underlying genetic conditions.

Systemic hypertension secondary to peripheral vascular anomalies in patients with Williams syndrome

ng/ml , n = 20). Although whole blood serotonin concentrations may decrease with increasing age, 1~ values in patient 1 were also elevated compared with those in an age-matched normal pediatric population we are studying (values to be reported elsewhere). Elevated serotonin levels in children with Williams syndrome adds to the growing list of disorders that have evidence of central nervous system serotonergic dysfunction? In summary, medical, developmental, and psychologic data have been described in two children with Williams syndrome and autism. The documentation and comparison of specific abnormalities in autistic Children with different associated organic conditions may help to delineate the neurobiologic cause of both the behavioral phenotype and the underlying genetic conditions.