Primary Congenital Lymphedema. 2018

Lubomir Mrhac, and Kamil Bukovansky, and Jana Kafkova, and Otakar Kraft

We present the case of a 26-year-old man with congenital primary lymphedema. A lymphedema of the right upper limb appeared at the age of 2 years, followed by edemas of the right lower limb and of the right part of the head and neck. A lymphedema of the left lower limb began 5 years ago, and finally an edema of the external genitalia began 2 years ago. Lymphoscintigraphy of the lower limbs and pelvic/abdominal region (including SPECT/ldCT) revealed interesting abnormalities.

UI MeSH Term Description Entries
D008209 Lymphedema Edema due to obstruction of lymph vessels or disorders of the lymph nodes. Milroy's Disease,Congenital Familial Lymphedema,Congenital Hereditary Lymphedema,Early Onset Lymphedema,Hereditary Lymphedema,Hereditary Lymphedema 1,Hereditary Lymphedema Type I,Lymphedema, Early-Onset,Lymphedema, Hereditary, Ia,Milroy Disease,Nonne-Milroy Disease,Nonne-Milroy Lymphedema,Nonne-Milroy-Meige Disease,Primary Congenital Lymphedema,Congenital Hereditary Lymphedemas,Congenital Lymphedema, Primary,Congenital Lymphedemas, Primary,Early Onset Lymphedemas,Early-Onset Lymphedema,Early-Onset Lymphedemas,Hereditary Lymphedema 1s,Hereditary Lymphedema, Congenital,Hereditary Lymphedemas,Hereditary Lymphedemas, Congenital,Lymphedema, Congenital Hereditary,Lymphedema, Early Onset,Lymphedema, Hereditary,Lymphedema, Nonne-Milroy,Lymphedema, Primary Congenital,Lymphedemas,Lymphedemas, Congenital Hereditary,Lymphedemas, Early Onset,Lymphedemas, Early-Onset,Lymphedemas, Hereditary,Lymphedemas, Primary Congenital,Milroys Disease,Nonne Milroy Disease,Nonne Milroy Lymphedema,Nonne Milroy Meige Disease,Primary Congenital Lymphedemas
D008297 Male Males
D010388 Pelvis The space or compartment surrounded by the pelvic girdle (bony pelvis). It is subdivided into the greater pelvis and LESSER PELVIS. The pelvic girdle is formed by the PELVIC BONES and SACRUM. Pelvic Region,Region, Pelvic
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000072098 Single Photon Emission Computed Tomography Computed Tomography An imaging technique using a device which combines TOMOGRAPHY, EMISSION-COMPUTED, SINGLE-PHOTON and TOMOGRAPHY, X-RAY COMPUTED in the same session. CT SPECT,CT SPECT Scan,SPECT CT,SPECT CT Scan,CT SPECT Scans,CT SPECTs,CT Scan, SPECT,CT Scans, SPECT,SPECT CT Scans,SPECT Scan, CT,SPECT Scans, CT,SPECT, CT,SPECTs, CT,Scan, CT SPECT,Scan, SPECT CT,Scans, CT SPECT,Scans, SPECT CT
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D061305 Lymphoscintigraphy Radionuclide imaging of the LYMPHATIC SYSTEM. Lymphoscintigraphies
D035002 Lower Extremity The region of the lower limb in animals, extending from the gluteal region to the FOOT, and including the BUTTOCKS; HIP; and LEG. Extremity, Lower,Lower Limb,Membrum inferius,Extremities, Lower,Limb, Lower,Limbs, Lower,Lower Extremities,Lower Limbs

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