Clinical Features and Prognosis of Generalized Lymphatic Anomaly, Kaposiform Lymphangiomatosis, and Gorham-Stout Disease

2016 ◽  
Vol 63 (5) ◽  
pp. 832-838 ◽  
Author(s):  
Michio Ozeki ◽  
Akihiro Fujino ◽  
Kentaro Matsuoka ◽  
Shunsuke Nosaka ◽  
Tatsuo Kuroda ◽  
...  
Keyword(s):  
Clinical Features ◽  
Generalized Lymphatic Anomaly ◽  
Kaposiform Lymphangiomatosis

Chest imaging in generalized lymphatic anomaly and kaposiform lymphangiomatosis

2018 ◽  
Vol 60 (7) ◽  
pp. 667-668 ◽  
Author(s):  
Hiroki Kato ◽  
Michio Ozeki ◽  
Toshiyuki Fukao ◽  
Masayuki Matsuo
Keyword(s):  
Chest Imaging ◽  
Generalized Lymphatic Anomaly ◽  
Kaposiform Lymphangiomatosis

scholarly journals CT and MRI Findings of Focal Splenic Lesions and Ascites in Generalized Lymphatic Anomaly, Kaposiform Lymphangiomatosis, and Gorham-Stout Disease

2021 ◽  
Vol 11 ◽  
pp. 44
Author(s):  
Fumihiko Nakamura ◽  
Hiroki Kato ◽  
Michio Ozeki ◽  
Masayuki Matsuo
Keyword(s):  
Ct Images ◽  
Maximum Diameter ◽  
Imaging Findings ◽  
Mri Findings ◽  
Abdominal Ct ◽  
Generalized Lymphatic Anomaly ◽  
Kaposiform Lymphangiomatosis ◽  
Focal Splenic Lesions ◽  
Ct And Mri

Objectives: This study aimed to evaluate the CT and MRI findings of focal splenic lesions and ascites in generalized lymphatic anomaly (GLA), kaposiform lymphangiomatosis (KLA), and Gorham-Stout disease (GSD). Material and Methods: Twenty-three patients (10 with GLA, 5 with KLA, and 8 with GSD) who underwent abdominal CT and/or MRI before treatment were included in this study, and their imaging findings were retrospectively evaluated. Results: Focal splenic lesions were observed in nine patients; these lesions were observed frequently in GLA (n = 5; 50%) or KLA (n = 3; 60%) compared with GSD (n = 1; 13%); however, no significant differences were found between the three groups (P = 0.190). On CT images among eight patients (4 with GLA, 3 with KLA, and 1 with GSD) with focal splenic lesions who underwent CT, the number of focal splenic lesions per patient ranged from 2 to 189 (mean, 42) and the maximum diameter of focal splenic lesions ranged from 2 to 39 mm (mean, 8 mm), while more than 30 focal splenic lesions per patient were observed in 2 (50%) GLA and focal splenic lesions with maximum diameters of ≥10 mm were observed in 4 (100%) GLA but not in KLA or GSD. Ascites was observed in five patients; significant differences were observed among KLA (n = 4; 80%), GLA (n = 1; 10%), and GSD (n = 0; 0%) (P < 0.01). Ascites was significantly more frequent in KLA than in GSD (P < 0.05). Conclusion: More than 30 focal splenic lesions per patient and/or focal splenic lesions with maximum diameters of ≥10 mm were observed only in GLA. Focal splenic lesions tended to be less frequent in GSD, whereas ascites tended to be frequent in KLA.

scholarly journals BONE DEVELOPMENT AND FRACTURE HEALING IS NORMAL IN MICE THAT HAVE A DEFECT IN THE DEVELOPMENT OF THE LYMPHATIC SYSTEM

Lymphology ◽  
2021 ◽  
Vol 53 (4) ◽  
Author(s):  
AL McCarter ◽  
A Khalid ◽  
Y Yi ◽  
M Monroy ◽  
H Zhao ◽  
...  
Keyword(s):  
Fracture Healing ◽  
Lymphatic System ◽  
Bone Development ◽  
Bone Destruction ◽  
Wild Type ◽  
Normal Bone ◽  
Adult Mice ◽  
Generalized Lymphatic Anomaly ◽  
Kaposiform Lymphangiomatosis ◽  
Lymphatic Vasculature

Ectopic lymphatics form in bone and promote bone destruction in diseases such as Gorham-Stout disease, generalized lymphatic anomaly, and kaposiform lymphangiomatosis. However, the role lymphatics serve in normal bone development and repair is poorly understood. The objective of this study was to characterize bone development and fracture healing in mice that have a defect in the development of the lymphatic vasculature. We found that bones in wild-type adult mice and mouse embryos did not have lymphatics. We also found that bone development was normal in Vegfr3Chy/Chy embryos. These mice do not have lymphatics and die shortly after birth. To determine whether lymphatics serve a role in postnatal bone development and fracture healing, we analyzed bones from Vegfr3wt/Chy mice. These mice are viable and have fewer lymphatics than wild-type mice. We found that postnatal bone development and fracture healing was normal in Vegfr3wt/Chy mice. Taken together, our results suggest that lymphatics do not play a major role in normal bone development or repair.

scholarly journals Sirolimus in the treatment of kaposiform lymphangiomatosis

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Jiangyuan Zhou ◽  
Kaiying Yang ◽  
Siyuan Chen ◽  
Yi Ji
Keyword(s):  
Clinical Practice ◽  
Poor Prognosis ◽  
Standard Treatment ◽  
Mammalian Target Of Rapamycin ◽  
Refractory Disease ◽  
Generalized Lymphatic Anomaly ◽  
The Poor ◽  
Poor Understanding ◽  
Kaposiform Lymphangiomatosis ◽  
New Treatments

Abstract Background Kaposiform lymphangiomatosis (KLA), which is a new subtype of generalized lymphatic anomaly, is a rare disease with a poor prognosis. Currently, there is no standard treatment due to the poor understanding of KLA. Sirolimus, which is an inhibitor of mammalian target of rapamycin, has been shown to have promising potential in the treatment of complicated vascular anomalies. The aim of this study was to introduce the use of sirolimus for the treatment of KLA and to highlight the challenges of managing this refractory disease. Results We reported seven patients with KLA who received sirolimus therapy in our center. Combined with previously reported cases, 58.3% achieved a partial response, 25.0% had stable disease, and 16.7% experienced disease progression. No severe sirolimus-related adverse events occurred during treatment. Conclusions This study suggests that sirolimus is currently an option for the treatment of KLA, and it is hoped that more specific therapies will be developed in the future. Rapid advances in basic science and clinical practice may facilitate the development of important new treatments for KLA.

scholarly journals How we approach the diagnosis and management of complex lymphatic anomalies

2020 ◽  
Author(s):  
Kiersten Ricci ◽  
Ionela Iacobas
Keyword(s):  
Disease Management ◽  
Comprehensive Evaluation ◽  
Diagnostic Testing ◽  
Early Mortality ◽  
Circulation System ◽  
Significant Morbidity ◽  
Congenital Diseases ◽  
Lymphatic Circulation ◽  
Generalized Lymphatic Anomaly ◽  
Kaposiform Lymphangiomatosis

Complex lymphatic anomalies 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 complex lymphatic anomalies were recently published, the diagnostic approach and medical management are not standardized. The current manuscript presents the clinical features of 4 complex lymphatic anomalies: 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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