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CT and MR imaging of Generalized Cystic Lymphangiomatosis in Pediatric Patients

¹ Department of Radiology, University of Vienna, Waehringer Guertel 18-20, A-1090 Vienna, Austria.
² Department of Pediatric Surgery, University of Vienna, A-1090 Vienna, Austria.

View larger version (154K): [in a new window]   Fig. 1A. —10-year-old boy with generalized cystic lymphangiomatosis. Radiograph of skull shows sharply delineated osteolytic lesions (arrowheads) of skull.

View larger version (148K): [in a new window]   Fig. 1B —10-year-old boy with generalized cystic lymphangiomatosis. CT scan also reveals lesions (arrowheads) seen in A.

View larger version (170K): [in a new window]   Fig. 2A —1-year-old girl with generalized cystic lymphangiomatosis. Unenhanced axial T1-weighted turbo spin-echo MR image shows partly cystic, partly solid mass in right laryngealcervical region (arrowheads).

View larger version (196K): [in a new window]   Fig. 2B —1-year-old girl with generalized cystic lymphangiomatosis. Enhanced coronal T1-weighted turbo spin-echo MR image better depicts septated nature of mass (arrowheads) and shows deep extension of this mass into cervical spaces (arrow).

View larger version (127K): [in a new window]   Fig. 2C —1-year-old girl with generalized cystic lymphangiomatosis. Unenhanced coronal T2-weighted turbo spin-echo MR image of same mass reveals its cystic nature (arrows).

View larger version (151K): [in a new window]   Fig. 2D —1-year-old girl with generalized cystic lymphangiomatosis. Photograph of autopsy specimen of laryngeal—cervical region reveals diffuse lymphatic infiltration and swelling of entire larynx including vocal chords (arrowheads).

View larger version (138K): [in a new window]   Fig. 3A —4-year-old-boy with generalized cystic lymphangiomatosis. Unenhanced CT scan shows bilateral pleural effusions (large arrowheads), left-sided interstitial thickening with interstitial nodules (arrows), and lytic vertebral bone lesion (small arrowhead).

View larger version (150K): [in a new window]   Fig. 3B —4-year-old-boy with generalized cystic lymphangiomatosis. After tapping bilateral pleural effusions, unenhanced axial T2-weighted turbo spin-echo MR image also reveals interstitial nodules (arrow) seen in A, which proved to be chyle-filled cystic tumors at surgery.

View larger version (111K): [in a new window]   Fig. 4A —4-year-old-boy with generalized cystic lymphangiomatosis. Enhanced CT scan at level of pelvis shows extensive cystic masses (arrows) in mesentery confirmed as chyle-filled dilated, cystic lymphatics at surgery.

View larger version (141K): [in a new window]   Fig. 4B —4-year-old-boy with generalized cystic lymphangiomatosis. Unenhanced coronal T2-weighted turbo spin-echo MR image performed after splenectomy reveals complete craniocaudal extension of masses (arrows) seen in A.

View larger version (137K): [in a new window]   Fig. 5. —5-year-old boy with generalized cystic lymphangiomatosis. Enhanced CT scan at level of liver shows cystic lesions in spleen (solid curved arrow) and reveals presence of pleural effusion (straight arrow) and lytic vertebral bone lesion (open curved arrow).

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