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

Nakamura, Fumihiko; Kato, Hiroki; Ozeki, Michio

doi:10.25259/jcis_101_2021

Cited by 4 publications

(4 citation statements)

References 14 publications

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“…28 MRI is particularly useful to identify KLA lesions in the thorax, abdomen, pelvis, and skeleton. 29 present, splenic lesions can appear cystic. On MRI, lesions in the bone marrow may be well defined and cystic-appearing or poorly defined and demonstrate increased fat deposition.…”

Section: Clinical Presentation and Imagingmentioning

confidence: 95%

“… 16,25–27 CT may also reveal randomly distributed osteolytic lesions 28 . MRI is particularly useful to identify KLA lesions in the thorax, abdomen, pelvis, and skeleton 29 . MRI chest findings largely are parallel to those of CT, with interstitial thickening, mediastinal infiltration, and effusions generally appearing bright on T2‐weighted images (Figure 3A–D).…”

Section: Clinical Presentation and Imagingmentioning

confidence: 99%

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Kaposiform lymphangiomatosis: Diagnosis, pathogenesis, and treatment

McDaniel

Adams

Steele³

et al. 2023

Pediatric Blood & Cancer

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Kaposiform lymphangiomatosis (KLA) is a life‐threatening rare disease that can cause substantial morbidity, mortality, and social burdens for patients and their families. Diagnosis often occurs long after initial symptoms, and there are few centers in the world with the expertise to diagnose and care for patients with the disease. KLA is a lymphatic anomaly and significant advancements have been made in understanding its pathogenesis and etiology since its first description in 2014. This review provides multidisciplinary, comprehensive, and state‐of‐the‐art information on KLA patient presentation, diagnostic imaging, pathology, organ involvement, genetics, and pathogenesis. Finally, we describe current therapeutic approaches, important areas for research, and challenges faced by patients and their families. Further insights into the pathogenesis of KLA may advance our understanding of other vascular anomalies given that similar signaling pathways may be involved.

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Section: Clinical Presentation and Imagingmentioning

confidence: 95%

Section: Clinical Presentation and Imagingmentioning

confidence: 99%

Kaposiform lymphangiomatosis: Diagnosis, pathogenesis, and treatment

McDaniel

Adams

Steele³

et al. 2023

Pediatric Blood & Cancer

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“…Indeed, the diagnosis of KLA is often delayed because KLA usually mimics GLA or kaposiform hemangioendothelioma (KHE) ( 1 , 6 , 13 ). Thus, imaging examinations are very important ( 4 , 14 – 16 ). Imaging studies combined with medical history can help us make a clinical diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Refractory kaposiform lymphangiomatosis relieved by splenectomy

Lan,

Zhou,

Qiu

et al. 2023

Front. Pediatr.

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IntroductionKaposiform lymphangiomatosis (KLA) is a rare and complex lymphatic anomaly with a poor prognosis. There is no standard treatment, and drug therapies are the most common therapeutic method. However, some patients' symptoms become gradually aggravated despite medical treatment. Splenectomy may be an alternative option when pharmacological therapies are ineffective.Materials and MethodsWe reviewed and evaluated the cases of 3 patients with KLA who ultimately underwent splenectomy. Results: The lesions were diffusely distributed and involved the lungs and spleens of the 3 patients. Laboratory examinations revealed that all three patients had thrombocytopenia and reduced fibrinogen levels. All patients underwent symptomatic splenectomy after the medication failed. Surprisingly, their symptoms greatly improved. Histopathological investigation of the splenic lesions of the three patients confirmed the diagnosis of KLA. Immunohistochemical staining showed positivity for CD31, CD34, podoplanin, Prox-1 and angiopoietin 2 (Ang-2).DiscussionThis study aimed to review the features of KLA patients treated by splenectomy and explore the underlying link between splenectomy and prognosis. The reason for the improvement after splenectomy may be related to increased Ang-2 levels and platelet activation in patients with KLA. Future research should seek to develop more targeted drugs based on molecular findings, which may give new hope for the treatment of KLA.

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“…The imaging criteria for GSD were defined as progressive osteolysis with cortical bone resorption. 11 All radiologic studies were reviewed by a pediatric radiologist with special attention to the extent of bone, soft tissue, effusion density, and visceral involvement. The imaging techniques used to evaluate the patients with GSD in this study included computed tomography (CT) (n = 7) and bone survey (n = 7).…”

Section: Methodsmentioning

confidence: 99%

Evaluation of Clinical Findings in Children with Chylothorax: A Descriptive Study

Büyükşahin

Emiralioğlu

Özcan

et al. 2023

Turk Arch Pediatrics

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Objective: Chylothorax refers to the presence of chyle in the pleural space. There are multiple etiologies of chylothorax. Our aim in this study was to evaluate the clinical manifestations, causes, and treatment of chylothorax in childhood and also to show the differences between the 2 age groups admitted to a tertiary care children’s hospital. The second aim was to evaluate the clinical and radiologic features of patients diagnosed as having Gorham-Stout disease via chylothorax. Materials and Methods: The archives were reviewed for chylothorax documented in the last 31 years. Twenty-two patients (11 girls and 11 boys) were included. Patients were divided into 2 groups: the younger group aged under 24 months and the older group aged over 24 months. Results: A total of 22 patients had chylothorax, and 10 were aged younger than 24 months. In the younger group, etiologies were in order congenital heart surgery, congenital chylothorax, and Gorham-Stout disease. In the older group, etiologies were Gorham-Stout disease, congenital heart surgery, heart failure, heart transplantation, thrombus, intestinal lymphangiectasia, and idiopathic. The most common treatment in the younger group was the medium-chain triglyceride diet (70%), and in the older group, it was sirolimus (50%). Conclusion: There is a wide variety of underlying etiologies in childhood, so a multidisciplinary approach is important to identify the underlying diagnosis. The common etiologies were postoperative and Gorham-Stout disease in our study. All patients with Gorham-Stout disease had a good prognosis. Gorham-Stout disease should be considered in patients of any age with a diagnosis of chylothorax who have bone lesions.

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

Cited by 4 publications

References 14 publications

Kaposiform lymphangiomatosis: Diagnosis, pathogenesis, and treatment

Kaposiform lymphangiomatosis: Diagnosis, pathogenesis, and treatment

Refractory kaposiform lymphangiomatosis relieved by splenectomy

Evaluation of Clinical Findings in Children with Chylothorax: A Descriptive Study

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