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March 2010 Case of the Month
Compiled by: Amit Verma, M.D.
History: A 54-year-old female with a chronic sinus infection
Exam: Multiple axial, coronal and sagittal CT images of the chest were obtained with IV contrast
Findings: Large left chest mass is identified with massive splenomegaly. There is likely portal vein cavernous transformation given small enhancing vessels in the porta hepatic with no identification of a normal portal vein. Splenorenal shunt is seen. Incidental right aortic arch.
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Diagnosis: Splenopneumopexy
Discussion: Upon further interrogation of the patient’s medical and surgical history, it was learned that the patient had a history of portal vein thrombosis, portal hypertension and ruptured varices. The patient had undergone an anastomosis of the left lower lung lobe to the spleen.
The procedure of splenopneumopexy was developed in Japan in the 1960s. The surgery is performed to induce collateral circulation between the portal venous system and the pulmonary venous system. Originally, portopulmonary shunting was utilized in patients with Budd-Chiari syndrome. There are various methods to treat Budd-Chiari syndrome, and in Japan partial splenectomy or splenopneumopexy may be used as the initial choice.
Collaterals develop not only between the splenic and pulmonary venous circulations but with the phrenic, intercostal, pericardiophrenic and mediastinal veins. An anastomosis is made between an amputated superior pole of the spleen and exposed left lower lobe pulmonary veins. The patients undergo splenic artery embolization prior to the splenopneumopexy to decrease bleeding during the surgery, possibly decrease splenomegaly and possibly benefiting the regional varices if the splenic vein is thrombosed. Splenopneumopexy has been used in patients who fail sclerotherapy of bleeding varices and patients who have had portal vein thrombosis and cirrhosis.
Multiple treatments for portal vein occlusive disease include portosystemic shunts, variceal ligation, esophageal replacement, and sclerotherapy. The main cause of death in patients with portal vein occlusive disease is mesenteric venous intestinal infarct and gastrointestinal bleeding.
References:
1. Masayuki, Tominanga et al. “Prominent hepatic encephalopathy 19 years after splenopneumopexy in Budd-Chiari syndrome.” J Hepatobiliary Pancreas Surg, 2007; 14: 200-203.
2. Reese, Jeffery C. et al. “Splenopneumopexy to Treat Portal Hypertension Produced by Venous Occlusive Disease.” Arch Surg, Sep 1992; 127: 1129-1132.
3. Xu, PQ et al. “Surgical treatment of 1360 cases of Budd-Chiari syndrome: 20-year experience.” Hepatobiliary Pancreat Dis Int. Aug 2004; 3(3): 391-4.
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