lymphangiomatosis


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lymphangiomatosis

A rare lesion that usually affects women of child-bearing years, which is characterised by well-demarcated osteolytic lesions variably accompanied by sclerosis.

Clinical findings
Slowly progressive dyspnoea, chylous effusion, recurring pneumothorax, haemoptysis, massive haemorrhage.

DiffDx
Fibrous dysplasia.
References in periodicals archive ?
Miscellaneous Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary vessels (adenopathy, tumor, fibrosing mediastinitis) (a) Modifications to the previous classification are represented in bold.
In children, these tumors may be part of a systemic lymphangiomatosis syndrome, which involves the mediastinum, lungs, liver, kidney, GI and GU tracts.
It is known by different synonyms such as renal lymphangiomatosis, peripelvic lymphangiectasia, hygromarenale, polycystic disease of renal sinuses8.
Diagnostic Criteria for Tuberous Sclerosis (2012) Major Criteria Minor Criteria Cortical tubers Non-renal hamartoma Subependymal nodules Confetti-like skin lesions Hypomelanocytic macule Multiple renal cysts (at least 3 or more and larger than 5 mm) Shagreen patch Hypopigmented patch in the retina Angiofibroma in the face (more than three) Ungal and periungal fibromas Retinal hamartomas Rhabdomyoma in the heart Lymphangiomatosis in the lung Angiomyolipoma in the kidney (more than two) Definite diagnosis: 2 major or 1 major + >2 minor criteria Possible diagnosis: 1 major or >2 minor criteria Table 2.
CT and MR imaging in cystic abdominal lymphangiomatosis.
Exacerbation of familial renal lymphangiomatosis during pregnancy.
Little Alfie is thought to be one of only three people in the UK to suffer from the rare condition lymphangiomatosis, which has left him with a growth so big surgeons are unable to remove it.
The diagnosis could be suspected on plain radiograph but MRI is the better diagnostic imaging modality as it can rule out other differential diagnoses such as neurofibromatosis, haemangiomatosis, lymphangiomatosis, Proteus syndrome and fibrolipomatous hamartoma.
At post-mortem, a diagnosis of lymphangiomatosis was made.
Miscellaneous Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary vessels (adenopathy, tumor, fibrosing mediastinitis) * Guidelines for Classification of Congenital Systemic-to-Pulmonary Shunts 1.
Miscellaneous Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary vessels (adenopathy, tumor, fibrosing mediastinitis) * Classification does not include pulmonary hypertension due to end-stage renal disease.