Case report Surgical management of chylopericardium and chylothorax in a patient with Behçet's disease

S-68, Emad Al Jaaly, Kamran Baig, Ravi Patni, Jon Anderson, Dorian Haskard, E Al Jaaly, K Baig, R Patni, J Anderson, D Haskard
2011 Clin Exp Rheumatol   unpublished
Behçet's syndrome is a chronic mul-tisystem inflammatory disease char-acterised classically by recurrent oral and genital ulcers with ocular lesions. It can affect blood vessels of all sizes, but involves veins more commonly than arterie. The presence of chylothorax in Behçet's syndrome is rare, with only a few cases cited in the literature. The most likely pathogenesis is SVC thrombosis with obstruction of the orifice of the thoracic duct resulting in leakage of chyle from the pleural
more » ... s into the pleural space. The majority of the previously reported cases were managed medically without surgical intervention. We believe that this report describes the first use of surgery to ligate the thoracic duct and create a pericardial window in a Behçet's syndrome with chylothorax and chyloperi-cardium. Case presentation A 20-year-old man was admitted to hospital with a 3 month history of feeling generally unwell with neck swelling , facial redness, difficulty of swallowing , fevers, mouth ulcers as well as widespread ulcers over the genitouri-nary regions. A diagnosis of Behçet's syndrome had been made three months prior to admission by a consultant dermatologist , and he was started on cy-closporine 75 mg BD. He also had been taking oral prednisolone 30 mg OD, co-chicine 0.5 mg TDS and topical agents to treat the oral and genital ulcers. His past medical history and family history were unremarkable, other than admission 2 months prior with epididymo-orchitis. Blood tests on admission revealed raised inflammatory markers, with a white cell count of 22.9 x 10 9 /L and C-reactive protein of 211mg/L. The patient developed hypoxia and the subsequent chest radiograph demonstrated left lower lobe consolidation along with some pleural effusion. A CT and MRI scan of the thorax, abdomen and pelvis confirmed the presence of large left pleural and pericardial ef-fusions (Figs. 1-3). A Venogram was performed as though Doppler is able to demonstrate flow, it is limited in delin-eating the anatomy and location of obstruction. Venogram imaging revealed thrombosis of the left brachiocephalic vein and superior vena cava, with extensive mediastinal and pericardial collaterals (Fig. 4). In an attempt to drain the left pleural effusion a 12 F Seldinger Portex chest drain was inserted and over 6 litres of chylous fluid was drained over the next 4 days. The patient subsequently developed sinus tachycardia (240 beats per minute). He was then transferred to a tertiary centre with expertise in Behçet's syndrome. Soon after his arrival , the patient developed clinical signs of tamponade and was confirmed by transthoracic echocardiogram. An emergency pericardiocentesis was performed and 1.1L of chyle was drained with immediate relief of tamponade and symptoms. In view of the life threatening chylopericardium and continued high chyle output from the left pleural drain, the patient was referred for definitive surgical management. The patient underwent a right tho-racotomy and the thoracic duct was identified in the posterior mediastinum between the descending thoracic aor-ta and the azygos vein. The duct was ligated and divided. A pericardial window was constructed to allow pericar-dial fluid to drain into the right pleural cavity. On the fourth postoperative day the pericardial and pleural drains were removed and transthoracic echocardio-gram and chest radiograph confirmed