1228861004: Kaposiform lymphangiomatosis (disorder)

• SNOMED CT Concept\Clinical finding (finding)\...
• \Mass of body structure\Mass of soft tissue\Lymphangiomatosis\A rare vascular anomaly or angioma characterised by multifocal malformed lymphatic channels lined by clusters or sheets of spindled lymphatic endothelial cells with a predilection for the thoracic cavity, but also involving extra-thoracic locations, especially bones and spleen. Typical clinical signs and symptoms are pericardial and pleural effusions, cough, dyspnoea, bleeding, and fractures secondary to bone involvement. Prognosis is generally poor due to the progressive nature of the condition.
• \General finding of soft tissue\Mass of soft tissue\Lymphangiomatosis\A rare vascular anomaly or angioma characterised by multifocal malformed lymphatic channels lined by clusters or sheets of spindled lymphatic endothelial cells with a predilection for the thoracic cavity, but also involving extra-thoracic locations, especially bones and spleen. Typical clinical signs and symptoms are pericardial and pleural effusions, cough, dyspnoea, bleeding, and fractures secondary to bone involvement. Prognosis is generally poor due to the progressive nature of the condition.
• \General finding of soft tissue\Disorder of soft tissue\Lesion of soft tissue (disorder)\Lymphangiomatosis\A rare vascular anomaly or angioma characterised by multifocal malformed lymphatic channels lined by clusters or sheets of spindled lymphatic endothelial cells with a predilection for the thoracic cavity, but also involving extra-thoracic locations, especially bones and spleen. Typical clinical signs and symptoms are pericardial and pleural effusions, cough, dyspnoea, bleeding, and fractures secondary to bone involvement. Prognosis is generally poor due to the progressive nature of the condition.
• \General finding of soft tissue\Disorder of soft tissue\Disorder of lymphatic vessel (disorder)\Lymphatic malformation\Lymphangiomatosis\A rare vascular anomaly or angioma characterised by multifocal malformed lymphatic channels lined by clusters or sheets of spindled lymphatic endothelial cells with a predilection for the thoracic cavity, but also involving extra-thoracic locations, especially bones and spleen. Typical clinical signs and symptoms are pericardial and pleural effusions, cough, dyspnoea, bleeding, and fractures secondary to bone involvement. Prognosis is generally poor due to the progressive nature of the condition.
• \Disease\Disorder of foetus and/or newborn\Congenital disease\Congenital malformation\Lymphatic malformation\Lymphangiomatosis\A rare vascular anomaly or angioma characterised by multifocal malformed lymphatic channels lined by clusters or sheets of spindled lymphatic endothelial cells with a predilection for the thoracic cavity, but also involving extra-thoracic locations, especially bones and spleen. Typical clinical signs and symptoms are pericardial and pleural effusions, cough, dyspnoea, bleeding, and fractures secondary to bone involvement. Prognosis is generally poor due to the progressive nature of the condition.
• \Disease\Neoplasm and/or hamartoma (disorder)\Angiomatosis\Lymphangiomatosis\A rare vascular anomaly or angioma characterised by multifocal malformed lymphatic channels lined by clusters or sheets of spindled lymphatic endothelial cells with a predilection for the thoracic cavity, but also involving extra-thoracic locations, especially bones and spleen. Typical clinical signs and symptoms are pericardial and pleural effusions, cough, dyspnoea, bleeding, and fractures secondary to bone involvement. Prognosis is generally poor due to the progressive nature of the condition.
• \Disease\Disorder of body system\Disorder of lymphatic system\Disorder of lymphatic vessel (disorder)\Lymphatic malformation\Lymphangiomatosis\A rare vascular anomaly or angioma characterised by multifocal malformed lymphatic channels lined by clusters or sheets of spindled lymphatic endothelial cells with a predilection for the thoracic cavity, but also involving extra-thoracic locations, especially bones and spleen. Typical clinical signs and symptoms are pericardial and pleural effusions, cough, dyspnoea, bleeding, and fractures secondary to bone involvement. Prognosis is generally poor due to the progressive nature of the condition.
• \Disease\Disorder of soft tissue\Lesion of soft tissue (disorder)\Lymphangiomatosis\A rare vascular anomaly or angioma characterised by multifocal malformed lymphatic channels lined by clusters or sheets of spindled lymphatic endothelial cells with a predilection for the thoracic cavity, but also involving extra-thoracic locations, especially bones and spleen. Typical clinical signs and symptoms are pericardial and pleural effusions, cough, dyspnoea, bleeding, and fractures secondary to bone involvement. Prognosis is generally poor due to the progressive nature of the condition.
• \Disease\Disorder of soft tissue\Disorder of lymphatic vessel (disorder)\Lymphatic malformation\Lymphangiomatosis\A rare vascular anomaly or angioma characterised by multifocal malformed lymphatic channels lined by clusters or sheets of spindled lymphatic endothelial cells with a predilection for the thoracic cavity, but also involving extra-thoracic locations, especially bones and spleen. Typical clinical signs and symptoms are pericardial and pleural effusions, cough, dyspnoea, bleeding, and fractures secondary to bone involvement. Prognosis is generally poor due to the progressive nature of the condition.
• \Disease\Developmental disorder (disorder)\Congenital malformation\Lymphatic malformation\Lymphangiomatosis\A rare vascular anomaly or angioma characterised by multifocal malformed lymphatic channels lined by clusters or sheets of spindled lymphatic endothelial cells with a predilection for the thoracic cavity, but also involving extra-thoracic locations, especially bones and spleen. Typical clinical signs and symptoms are pericardial and pleural effusions, cough, dyspnoea, bleeding, and fractures secondary to bone involvement. Prognosis is generally poor due to the progressive nature of the condition.

Status: current, Not sufficiently defined by necessary conditions definition status (core metadata concept). Date: 31-May 2022. Module: SNOMED CT core

Descriptions:

Id	Description	Lang	Type	Status	Case?	Module
5063491018	Kaposiform lymphangiomatosis (disorder)	en	Fully specified name	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
5063492013	Kaposiform lymphangiomatosis	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
5400048011	A rare vascular anomaly or angioma characterized by multifocal malformed lymphatic channels lined by clusters or sheets of spindled lymphatic endothelial cells with a predilection for the thoracic cavity, but also involving extra-thoracic locations, especially bones and spleen. Typical clinical signs and symptoms are pericardial and pleural effusions, cough, dyspnea, bleeding, and fractures secondary to bone involvement. Prognosis is generally poor due to the progressive nature of the condition.	en	Definition	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
5400049015	A rare vascular anomaly or angioma characterised by multifocal malformed lymphatic channels lined by clusters or sheets of spindled lymphatic endothelial cells with a predilection for the thoracic cavity, but also involving extra-thoracic locations, especially bones and spleen. Typical clinical signs and symptoms are pericardial and pleural effusions, cough, dyspnoea, bleeding, and fractures secondary to bone involvement. Prognosis is generally poor due to the progressive nature of the condition.	en	Definition	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
5063491018	Kaposiform lymphangiomatosis (disorder)	en	Fully specified name	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
5063492013	Kaposiform lymphangiomatosis	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
5063493015	A rare vascular anomaly or angioma with characteristics of multifocal malformed lymphatic channels lined by clusters or sheets of spindled lymphatic endothelial cells with a predilection for the thoracic cavity, but also involving extra-thoracic locations, especially bones and spleen. Typical clinical signs and symptoms are pericardial and pleural effusions, cough, dyspnea, bleeding and fractures secondary to bone involvement. Prognosis is generally poor due to the progressive nature of the condition.	en	Definition	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
5063494014	A rare vascular anomaly or angioma with characteristics of multifocal malformed lymphatic channels lined by clusters or sheets of spindled lymphatic endothelial cells with a predilection for the thoracic cavity, but also involving extra-thoracic locations, especially bones and spleen. Typical clinical signs and symptoms are pericardial and pleural effusions, cough, dyspnoea, bleeding and fractures secondary to bone involvement. Prognosis is generally poor due to the progressive nature of the condition.	en	Definition	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
5400048011	A rare vascular anomaly or angioma characterized by multifocal malformed lymphatic channels lined by clusters or sheets of spindled lymphatic endothelial cells with a predilection for the thoracic cavity, but also involving extra-thoracic locations, especially bones and spleen. Typical clinical signs and symptoms are pericardial and pleural effusions, cough, dyspnea, bleeding, and fractures secondary to bone involvement. Prognosis is generally poor due to the progressive nature of the condition.	en	Definition	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
5400049015	A rare vascular anomaly or angioma characterised by multifocal malformed lymphatic channels lined by clusters or sheets of spindled lymphatic endothelial cells with a predilection for the thoracic cavity, but also involving extra-thoracic locations, especially bones and spleen. Typical clinical signs and symptoms are pericardial and pleural effusions, cough, dyspnoea, bleeding, and fractures secondary to bone involvement. Prognosis is generally poor due to the progressive nature of the condition.	en	Definition	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
3408171001000116	Kaposiforme Lymphangiomatose	de	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module
3408171001000116	Kaposiforme Lymphangiomatose	de	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module

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