Imaging features of kaposiform lymphangiomatosis

Goyal, Pradeep; Alomari, Ahmad I.; Kozakewich, Harry P.; Trenor, Cameron C.; Perez‐Atayde, Antonio R.; Fishman, Steven J.; Greene, Arin K.; Shaikh, Raja

doi:10.1007/s00247-016-3611-1

Cited by 43 publications

(51 citation statements)

References 15 publications

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“…Whole‐body MRI is the most sensitive imaging technique for fluid and will identify any effusions (pleural, pericardial, or ascites), soft‐tissue mass, and bone lesions associated with LM. The imaging features for KLA are similar to those in GLA but also specifically include contrast enhancing and infiltrative soft‐tissue masses along the lymphatic vasculature of the mediastinum and retroperitoneum …”

Section: Recommendationsmentioning

confidence: 99%

“…Evaluation should start with a simple chest radiograph, and if any abnormalities are identified, the next step would be an ultrashort TE MRI chest or noncontrast computed tomography (CT) chest. Typically, cross‐sectional imaging will show a soft‐tissue mass in the mediastinum extending to the hilum and infiltrative soft‐tissue thickening along the bronchovascular bundle with or without septal thickening …”

Section: Recommendationsmentioning

confidence: 99%

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Multidisciplinary guidelines for initial evaluation of complicated lymphatic anomalies—expert opinion consensus

Iacobas

Adams

Pimpalwar

et al. 2019

Pediatric Blood & Cancer

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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.

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Section: Recommendationsmentioning

confidence: 99%

Section: Recommendationsmentioning

confidence: 99%

Multidisciplinary guidelines for initial evaluation of complicated lymphatic anomalies—expert opinion consensus

Iacobas

Adams

Pimpalwar

et al. 2019

Pediatric Blood & Cancer

Self Cite

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“…3,18 Complex and syndromic malformations can be associated with elevated D-dimer and thrombocytopenia (Kasabach-Merritt phenomenon), especially KLA. 16,19 KLA and kaposiform hemangioendothelioma (KHE) patients may have elevated angiopoietin-2 serum levels at presentation, which decreases after sirolimus treatment. 20…”

Section: Clinical Presentation and Workupmentioning

confidence: 99%

“…16 KLA presents similarly with GLA but with a predilection for the mediastinum and cystic splenic lesions. 16,19 KLA is histologically distinct from other complex lymphatic anomaly subtypes with characteristic sheets of spindled lymphatic endothelial cells, and it is associated with respiratory distress from pericardial and pleural effusions and worse clinical outcomes. 19,72,73 Primary lymphedema is another congenital LM caused by dysgenesis of the lymphatic system and is associated with several genetic mutations.…”

Section: Complex Lymphatic Anomaliesmentioning

confidence: 99%

“…16,19 KLA is histologically distinct from other complex lymphatic anomaly subtypes with characteristic sheets of spindled lymphatic endothelial cells, and it is associated with respiratory distress from pericardial and pleural effusions and worse clinical outcomes. 19,72,73 Primary lymphedema is another congenital LM caused by dysgenesis of the lymphatic system and is associated with several genetic mutations. 2 Although congenital, primary lymphedema typically presents clinically around puberty with lower extremity edema, may be hormonally sensitive, and includes entities such as Milroy's disease.…”

Section: Complex Lymphatic Anomaliesmentioning

confidence: 99%

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The Role of Interventional Radiologists in the Treatment of Congenital Lymphatic Malformations

Cronan

Gill

Shah

et al. 2020

Semin intervent Radiol

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Lymphatic malformations are low-flow vascular malformations that are typically apparent in the pediatric population and can cause significant functional limitations and effects on quality of life. While surgical resection has historically been the mainstay of therapy, percutaneous sclerotherapy has garnered increasing popularity due to its efficacy and low complication rates. The role of interventional radiology in the multidisciplinary management of these often complex malformations requires thorough understanding of the disease process. This article will review the pathophysiology, clinical presentation, imaging workup, and management options of lymphatic malformations. Special attention will be devoted to available sclerosants, the mammalian target of rapamycin inhibitor sirolimus, and complex lymphatic anomalies.

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How we approach the diagnosis and management of complex lymphatic anomalies

Ricci

Iacobas

2021

Pediatric Blood & Cancer

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Complex lymphatic anomalies (CLA) are congenital diseases of the lymphatic circulation system that are associated with significant morbidity and early mortality.While guidelines for the comprehensive evaluation of the CLA were recently published, the diagnostic approach and medical management are not standardized. This article presents the clinical features of four CLA: Gorham-Stout disease, generalized lymphatic anomaly, kaposiform lymphangiomatosis, and central collecting lymphatic anomaly. We also offer three cases from the authors' practice and our views on diagnostic testing and disease management including supportive care, medical therapies, and other interventions.

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Imaging features of kaposiform lymphangiomatosis

Cited by 43 publications

References 15 publications

Multidisciplinary guidelines for initial evaluation of complicated lymphatic anomalies—expert opinion consensus

Multidisciplinary guidelines for initial evaluation of complicated lymphatic anomalies—expert opinion consensus

The Role of Interventional Radiologists in the Treatment of Congenital Lymphatic Malformations

How we approach the diagnosis and management of complex lymphatic anomalies

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