The dynamic MR lymphangiographic technique with intranodal injection of gadolinium-based contrast material is feasible and can provide useful information in a variety of lymphatic flow abnormalities involving the CCL.
Objective
Complicated lymphatic anomalies (CLAs) are chronic, progressive, and debilitating conditions that share clinical features, yet key elements for optimal evaluation and management have not been established. We aimed to formulate expert opinion consensus‐based guidelines for comprehensive evaluation of CLAs.
Study Design
Patient support groups dedicated to CLAs organized an international conference for vascular anomaly experts from 16 specialties to address the objective. Participants received a set of questions before the meeting and reviewed the literature. Data extracted from international lymphatic anomaly registries were presented and the group separated for panel discussions during the conference. The recommendations achieving consensus within the panel were presented to the entire audience. Open debate occurred until majority approval was achieved.
Results
The expert group was composed of 52 physicians who defined the clinical elements required to evaluate and diagnose a CLA. The radiology panel established the preferred anatomical and functional imaging methods for diagnosis and the elements required to be described during interpretation. Two medical panels compiled the metabolic and hematologic tests at diagnosis and also recommended functional studies. The surgical group recommended precautions for biopsy and the pathology panel provided biopsy specimen processing guidelines.
Conclusions
Patients with CLAs require a comprehensive and targeted diagnostic plan for appropriate management, prevention of complications, and conservation of resources. As this population is managed by diverse medical and surgical specialties, we offer an expert multidisciplinary consensus‐based opinion on the current literature and on data extracted from international lymphatic anomaly registries.
Management of patients with symptomatic venous malformations (VMs) starts with a conservative approach. Regular daytime use of a custom-made compression stocking and muscle strengthening exercises together play a very important role in reducing the severity and frequency of pain. Extremity elevation and application of ice are useful adjunctive measures during acute painful episodes. Correction of leg-length discrepancy improves pain related to gait imbalance. Prophylactic anticoagulation is helpful in alleviating pain in patients with extensive VMs with evidence of localized intravascular coagulopathy.Indications for invasive treatment include (1) unsatisfactory pain relief using conservative measures, (2) bleeding episodes from superficial VMs, and (3) functional impairment and deformity. In patients with extensive VMs, the treatment is focused to the symptomatic region.
TechniqueTreatment is performed under general anesthesia. Intravenous antibiotic prophylaxis is administered using cefazolin 25 mg/kg. Clindamycin (10 mg/kg) is used in patients with penicillin allergy and for intraoral procedures. A Foley catheter is placed for procedures more than 2 hours duration.The region of interest is prepped and draped. For extremity VMs (►Fig. 1A), the entire extremity is prepped and draped and a 22G peripheral intravenous (IV) line is placed on the dorsum of the hand/foot followed by digital subtraction step
AbstractChildren with vascular malformations are best managed with a multidisciplinary team of specialists. Interventional radiology may deliver primary treatment such as staged sclerotherapy and embolization for malformations that are poor candidates for primary surgical resection or play a supportive role such as preoperative or intraoperative embolization. A thorough understanding of vascular morphology and flow dynamics is imperative to choosing the best treatment tool and technique. In this review, the author discusses the selection of techniques and tools used to treat vascular malformations based on their angiographic morphology.
Trans-splenic access is a useful technique for successful pediatric portal interventions. Although it entails a substantial risk of intraperitoneal bleeding, this can be managed conservatively.
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