UNASSIGNED: Vascular anomalies are a spectrum of disorders, including vascular tumors and malformations, that often require multispecialty care. The rarity and variety of these lesions make diagnosis, treatment, and management challenging. Despite the recognition of the medical complexity and morbidity associated with vascular anomalies, there is a general lack of education on the subject for pediatric primary care and subspecialty providers. A needs assessment and the lack of an available standardized teaching tool presented an opportunity to create an educational workshop for pediatric trainees using the POGIL (process-oriented guided inquiry learning) framework.
UNASSIGNED: We developed a 2-hour workshop consisting of an introductory didactic followed by small- and large-group collaboration and case-based discussion. The resource included customizable content for learning assessment and evaluation. Residents completed pre- and posttest assessments of content and provided written evaluations of the teaching session.
UNASSIGNED: Thirty-four learners in pediatrics participated in the workshop. Session evaluations were positive, with Likert responses of 4.6-4.8 out of 5 on all items. Pre- and posttest comparisons of four content questions showed no overall statistically significant changes in correct response rates. Learners indicated plans to use the clinical content in their practice and particularly appreciated the interactive teaching forum and the comprehensive overview of vascular anomalies.
UNASSIGNED: Vascular anomalies are complex, potentially morbid, and often lifelong conditions; multispecialty collaboration is key to providing comprehensive care for affected patients. This customizable resource offers a framework for trainees in pediatrics to appropriately recognize, evaluate, and refer patients with vascular anomalies.

OBJECTIVE: Currently, the focus on limb lymphedema (LE) is on classification and staging. However, few scholars have conducted staging for Klippel-Trenaunay syndrome complicated LE (KTS-LE). This study aimed to investigate the value of the short time inversion recovery sequence of magnetic resonance imaging (MRI) in the staging of KTS-LE.
METHODS: Forty-six patients who were diagnosed with KTS-LE were recruited for this retrospective study from July 2011 to November 2022. Referring to the clinical staging standard of lower extremity LE of the International Society of Lymphology in 2020, all patients were divided into three groups: stages I, II, and III. The MRI indicators of the three groups were recorded and statistically compared: LE range (unilateral bilateral, lower limbs, only thighs, only calves and ankles), abnormal parts (skin thickening, abnormal subcutaneous fat signal, abnormal muscle signal, muscle hypertrophy or contraction, abnormal bone signal, hyperostosis), and subcutaneous soft tissue signs (parallel line sign, grid sign, band sign, honeycomb sign, lymph lake sign, crescent sign, and nebula sign).
RESULTS: There was a significant difference in the honeycomb sign among the three periods (P = .028). There was a significant difference between stage II and stage I disease (P < .05). There was a significant difference between stage II and stage III disease (P < .05). The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of the honeycomb sign in diagnosing KTS-LE of stage II were 87.5%, 63.2%, 33.3%, 96.0%, and 67.4%, respectively. In contrast, the other signs were not statistically significant among the three periods.
CONCLUSIONS: The short time inversion recovery sequence of MRI is of great value in KTS-LE. The honeycomb sign is an important imaging indicator for the diagnosis of stage II disease. It is necessary to evaluate the severity of edema with MRI for KTS-LE, which is very important for therapeutic options.

A human aorta from a female donor affected by Klippel-Trenaunay syndrome was retrieved during a surgery for organ donation for transplant. The aorta was preserved in refrigerated Belzer UW organ preservation solution and tested within a few hours for mechanical characterization with and without vascular smooth muscle activation. KCl and Noradrenaline were used as vasoactive agents in bubbled Krebs-Henseleit buffer solution at 37 °C. A quasi-static and a dynamic mechanical characterization of the full wall and the three individual layers were carried out for strips taken in longitudinal and circumferential directions. The full wall in the descending portion of the aorta underwent mechanical tests with and without smooth muscle activation. Results were compared to data obtained from healthy aortas and show a reduced stiffness of the full wall in circumferential direction. Also, a significant reduction of the response to vasoactive agents in circumferential direction was observed, while the longitudinal response was similar to healthy cases.

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Klippel-Trenaunay syndrome (KTS) is a congenital mixed mesenchymal malformation syndrome that includes varicose veins, capillary and venous malformations, lymphatic abnormalities, and hypertrophy of various connective tissue elements. The purpose of the present study was to describe the clinical characteristics and outcomes in a subset of patients with KTS in whom venous interventions, including iliofemoral venous stenting, were performed after failure of conservative therapy.
A single-center retrospective data review of 34 patients with KTS who had undergone interventions for venous disease between January 2000 and December 2020 was performed.
Their mean age was 38.4 ± 17.5 years (range, 12-80 years). No gender predilection was found. Of the 34 patients, 61% had had all three features of the classic triad for KTS. Varicose veins were present in all 34 patients (100%), and 30% had had a history of bleeding varicosities. Most patients (79%) had CEAP (Clinical, Etiology, Anatomy, and Pathophysiology) class ≥C4. Of the 34 patients, 30% had a history of deep vein thrombosis and/or pulmonary embolism. Factor VIII elevation was the most common thrombophilia condition (12%). The venous filling index was elevated at baseline (5.9 ± 5.1 mL/s) and did not normalize despite intervention (3.5 ± 2.3 mL/s; P = .04). The superficial venous interventions (n = 35) included endovenous laser therapy; stripping of the great saphenous vein, small saphenous vein, anterior thigh vein, or marginal vein; ultrasound-guided sclerotherapy; and stab avulsion of varicose veins. One coil embolization of a perforator vein was performed. Deep interventions (n = 19) included endovenous stenting (n = 15), popliteal vein release (n = 3), and valvuloplasty (n = 1). The venous clinical severity score had improved from 9.4 ± 4.5 to 6.2 ± 5.6 (P = .04). The visual analog scale for pain score had improved from 5.5 ± 2.7 to 2.5 ± 3.3 (P = .008). Healing of ulceration was noted in 75% of the patients. Significant improvements in the total pain (P = .04) and total psychological (P = .03) domains were noted in the 20-item chronic venous disease quality of life questionnaire.
Superficial and deep venous interventions are safe and effective in patients with KTS when conservative therapy has failed. Iliofemoral venous stenting is a newer option that should be considered in the treatment of chronic deep venous obstructive disease in patients with KTS in the appropriate clinical context. An aggressive perioperative deep vein thrombosis prophylaxis protocol should be in place to reduce thromboembolic complications in these patients.

UNASSIGNED: Klippel Trenaunay Syndrome (KTS) is a rare congenital malformation with capillary and venous malformations and soft tissue/bony overgrowth with or without lymphatic malformation. Cutaneous vascular stain, varicosities and tissue hypertrophy represent its main clinical features. Besides, the patient can develop thromboembolic pathologies, recurrent bouts of infection, stasis eczema, limb length discrepancy and intolerable pain typical of intraosseous involvement.
UNASSIGNED: Here, we report a case series of seven patients aged 10-45 who presented to our centre with clinical features suggestive of KTS. Out of them, six patients had involvement of unilateral lower limb, while only one had involvement of bilateral lower limb. They all had typical cutaneous vascular stains and underlying venous malformation, while one patient had developed complications with multiple ulcer formation.
UNASSIGNED: An interdisciplinary team of vascular surgeons, dermatologists, interventional radiologists, orthopaedics, and physiotherapists managed the cases. We performed an individualized treatment as per the patient\'s presentation, which included a combination of supportive, medical, interventional radiologic, and surgical interventions. The follow-up outcomes of all the patients revealed significant resolution of symptoms.
UNASSIGNED: Patients with KTS can have diverse presentations. Therefore, clinicians should ensure an individualized treatment with the involvement of a multidisciplinary team for proper management and prevention of complications.

UNASSIGNED: Klippel-Trenaunay Syndrome (KTS) is a rare congenital vascular disorder characterized by capillary malformation, varicosities, and tissue overgrowth. It usually affects the unilateral lower extremities manifesting commonly as pain, localized rise of temperature, and venous tortuosity. However, in severe cases, ulceration, cellulitis, and chronic lymphatic malformation may be present. Management is mostly supportive and involves the use of compression stockings.
METHODS: Here, we report a case of KTS with a persistent lateral marginal vein of Servelle managed with radiofrequency ablation along with sclerotherapy of selected perforators. On a two-year follow-up, the symptoms had resolved and Doppler ultrasonography revealed resolution of the defective vein along with the absence of incompetent perforators.
UNASSIGNED: In cases with venous malformation with the persistence of embryonic avalvular venous structures, like the lateral marginal vein of Servelle, surgical intervention is warranted especially at a younger age to reduce the risk of future thromboembolic events and recurrence.
CONCLUSIONS: Varicosities of the lateral marginal vein of Servelle can be managed successfully by radiofrequency ablation and adjunct sclerotherapy in selected cases.

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Catastrophic antiphospholipid syndrome (CAPS) is an uncommon and the most severe form of antiphospholipid syndrome (APS). A 33-week pregnant patient with Klippel-Trenaunay syndrome, past SARS-CoV-2 infection and type I fetal growth restriction with shortening of the fetal long bone was diagnosed in our center with a probable CAPS. Cesarean section was performed four days after the diagnosis due to the torpid evolution of the patient. Clinical improvement was noted a few days later and the mother and baby were discharged within a week. We review the current literature on CAPS during pregnancy and provide an updated view.