Devoted to diagnostic and interventional spine imaging and therapeutics

Library

Generalized Lymphangiomatosis in an Adult: A Case Report 2012

General Spine

Edward, C, Fourgas, MD
Suresh, Patel, M.D., Non ASSR Member
Eric, Spickler, M.D., Non ASSR Member

Poster

Purpose

Generalized lymphangiomatosis is a rare congenital malformation of the lymphatics characterized by cystic lesions in the parenchymal organs and lytic bone lesions. In this report we review the CT imaging features of generalized lymphangiomatosis in a 68 year-old male patient.

Methods & Materials

CT studies performed in a 68 year-old male patient with surgically excised, pathologically-proven cystic lymphangiomas of the thorax were reviewed. They included images of the head, chest, abdomen, pelvis and right lower extremity obtained at our institution over a six year period.

Results

A 68 year-old male has multiple cystic lesions in the thorax including the lungs and mediastinum. Multiple cystic lesions in the spleen were asymptomatic. A small right chylous pleural effusion was stable over the course of six years. Multiple CT studies demonstrated stable numerous lytic osseous lesions throughout the skull base, axial skeleton including scapula, entire spine and pelvis as well as bilateral femuri and right tibia. All of the osseous lesions had a sharp zone of transition and did not demostrate sclerosis or pathologic fractures.

Conclusion

Generalized lymphangiomatosis may affect any organ that contains lymphatic tissue but the most common imaging features include cystic lesions in the visceral organs, lungs and mediastinum. Pleural and pericardial effusions as well as lytic bone lesions are also seen. This condition is typically encountered in early childhood when the rate of morbidity and mortality is high. Our patient, however, is an adult and relatively asymptomatic. Recognizing the features of generalized lymphangiomatosis in an older patient may eliminate the need for more invasive diagnostic or surgical procedures.

References/Financial Disclosures

1. Aviv RI, McHugh K, Hunt J. Angiomatosis of Bone and Soft Tissue: a Spectrum of Disease from Diffuse Lymphangiomatosis to Vanishing Bone Disease in Young Patients. Clinical Radiology 2001; 56: 184:190. 2. Liu H, Shatzke DR, Holliday RA. Bilateral Orbital Lymphangiomas in Two Patients with Generalized Lymphangiomatosis. AJNR 2007; 28:491-492. 3. Wunderbaldinger P, Paya K, Partik B, et al. CT and MR imaging of Generalized Cystic Lymphangiomatois in Pediatric Patients. AJR 2000; 174: 827-832.

[gallery]