Generalized lymphangiomatosis in patients treated for chylothorax following thoracoscopic sympathectomy: Case report

Abstract

Generalized lymphangiomatosis (GLA) is a very rare condition in adults, characterized by diffused proliferation of lymphatic vessels that requires differential diagnosis from other vascular disorders such as cavernous or capillary hemangioma. This is because of overlapping characteristics on histopathological examination. Therefore, imaging features such as CT and MRI are useful to evaluate morphological characteristics, location, and the extent of the spread as well as differential diagnosis with other pathologies. We report a case of a 22-year-old female patient with left hemothorax after thoracoscopic sympathectomy for the treatment of hand sweating. The patient underwent drainage and cleaning of the left pleura. Chest computed tomography and lumbar spine magnetic resonance imaging showed multiple fat infiltration foci of the lumbar spine and pelvis. A wing bone biopsy of the pelvis was initially performed for the diagnosis of chronic osteomyelitis. Afterwards, the patient continued to have pleural drainage and developed hemothorax and chylothorax, amounting to 3000 mL. The chest tube was blocked with a mixture of biological glue and lipiodol (2 mL of glue, ratio of glue to lipiodol: 1:4) and a 3 i-ED coil complex. After the intervention, the pleural fluid decreased; the left pleural fluid was still 15 mm thick, and the amount of fluid drained after 1 week was 100 mL. Aspiration of the chest wall lesion showed fluid rich in fat droplets. Combined with the results of lumbar spine magnetic resonance imaging and the old biopsy, this was consistent with generalized lymphangiomatosis.

Keywords: Embolization therapeutic; Generalized lymphangiomatosis; Interventional radiology; Magnetic resonance imaging; Thoracic duct.

Fig. 1

Purple spots on the chest.

Fig. 2

(A-C) CT scan before intervention, showing multiple foci of bone loss in the posterior ribs and vertebrae (arrows). (D) CT postintervention shows lipiodol deposits in bone lytic lesions (head arrow).

Fig. 3

CT images and lymphatic DSA. (A) Thin red pleural fluid with cloudy yellow deposits. (B, C) CT lymphatic images show tortuous dilation of the thoracic duct (straight white arrow, B), with contrast leaking into the left hilum (curved white arrow, C). (D, E) Prenodal lymphatic DSA image with contrast leakage into the left lung (straight black arrow, D), postnodal chest tube with coils and Histoacryl and Lipiodol mixture on DSA (curved black arrow, E). Chest tube image after vascular intervention on CT scan (white arrow, F).