Portovenous occlusice disease is a disorder of ucertian etiology and unpredictable cource. It can occur with or with out liver disease in children and adults. This disorder has been long recognized as an important clinical entity. and recent studies have outlined the clinical characteristics of the disease.
A variety of acceptable therapeutic alternatives have been used to treat portovenous occlusive disease. Treatment options include portosystemic shunts, variceal ligation, esophageal replacement, and more recently, sclerotheraphy. Patients with multivessel portovenous occlusion can potentially exhaust their therapeutic alternatives and dies of recurrent hemorrhage.
Splenopneumopexy is intended to induce collateral circulation between the portal and pulmonary venous systems. This operation has been recently used to control refractory cariceal hemorrhage in four patients with extended portal-splenic-mesenteric venous thrombosis, who no longer responded to sclerotherapy.
A splenopneumopexy was performed 24 hours following transfemoral splenic artery embolization. Forty hours after surgery, the patient had obvious intra-abdominal sepsis, promptiong a celiotomy and resection of 60cm of infarcted iejumum apparently caused by messenteric venous thrombosis, The patient recovered uneventfully and has had no futher gastrointestinal hemorrhage for 4 years, even though she developed spontaneous unilateral lower extremity deep venous thrombosis requiring long-term oral anticoagulant therapy.
A postoperative splenoportogram portopulmonary collateral circulation with contrast filling the left atrium and no esophageal varices. A chest computed tomogram with intravenous contrast demonstrated large vascular structures in the area of the left lower lobe and hilum of the left lung.
Mesenteric angiography and splenoportography revealed no patent anatomic portal vessels to use for a portosystemic shunt . Transfemoral splenic artery embolization was performed, followed 24 hours later by a splenopneumopexy. The patient has no gastrointestinal bleeding since the operation. A splenoportogram performed 1 year following the operationdemostrated an absence of esophageal varices and large, portopulmonary shunt.
The patient underwent splenic artery embolization and splenopneumopexy, and has had no subsequent gastrointestinal bleeding. A splenoportogram 1-year after surgery demonstrated that pulmonary and mediastinal collaterals has developed.
Subsequent to hepatic artery embolization, a splenopneumopexy was performed without complications. The patient was discharged on the eighth postoperative day and has had no futher gastrointestinal bleeding.
Splenopneumopexy was performed with the patient inthe standard right lateral decubitus position and the thoracic cavity and was entered through the left seventh intercostal space. In cases of splenomegaly, the dome of the spleen was found to displace the medial part of the diphragm superiorly, delineating the site where the incision in the diaphram should be made. When the left lower love retracted, a 10- to 12- cm opening was made in the diaphragm.
A biopsy of the left lower lobe of the liver was performed though the diaphragmatic opening. Three of the four patients has discernible ascites when the diaphragm was opened. The spleen had fibrous attachments to the diaphragm, which required cutting to mobilize the superior pole of the spleen to pass through the diaphram.
The upper third of the spleen was placed in the intrathoracic position by a circumferential running 3-0 polypropylene suture, fixing the edge of the diaphragm to the spleen. A scalpel was used to amputate the tip of the spleen to expose a 5-cm bare area. Surface bleeding was controlled by pressure and coagulation, and then holes several centimeters deep in the spleen were make with an ultrasonic sugical aspirator.
An area of the inferior surface of the left lower lobe of the lung was outlined to correspond to the bare area of the spleen. The pleura was incised with a scalpel and stripped away to produce another bare area. Using the ultrasonic dissector, 1- to 2- cm deep holes placed in the lung and venous structures were opened and cauterized. The bare area of the lung was sewn to the bare area of the spleen with a running 3-0 polypropylene suture placed circumferentially. A chest tube was placed and the thoracic cavity was closed.
the operative procedures on the four patients too a mean (+/-SD) of 158 +/-20 minutes, and no patients required operative or postoperative blood transfusions. The mean length of postoperative hospitalization was 9 +/- 2 days. No specific problems related to the operation developed in hte postoperative period, except for one patient who had intermittent hemoptysis for approximately 30 days following operation. He had several episodes of coughing up blood-stained sputum. This stopped spontaneously and has not recurred.
Experimental information regarding splenopneumopexy documents the development of large venous collaterals between the portal and pulmonary circulations. The clinical information in the English-language literature concerning the procedure is summarized in the Table. Numerous foreign-language reports were published between 1960 and 1970 and are summarized in the study by Hastbacka. Similar to the present report, most studies document radiographically the development fo collaterals and clinical follow-up. While long term information is limited, the clinical results are favorable with many patients experiencing cessation of variceal hemorrhage.
The majority of patients undergoing splenopneumopexy have had portal vein thrombosis or cirrhosis. One series of patients underwent the procedure for Budd-Chairi syndrom and our patients had extended extrahepatic portovenous occlusive disease. It is unlikely that splenopneumopexy would play a significant role in hte treatment of patients with portal hypertension and cirrhosis, with those patients having the potential of being treated with sclerotherapy, standard portosystemic shunts, and hepatic transplantation. Contrariwise, splenopneumopexy may be more advantageous in those patients with extrahepatic portovenous occlusive disease who no longer responds to sclerotheraphy.
The primary cause of death in patients with portovenous occlusive disease are gastrointestinal bleeding and mesenteric venous intestinal infarction. It is difficult to ascertain exact survival patterns. Children presention with portovenous occlusivedisease have a 20- to 30 year survival rate of approximately 70% to 75%. Occurring in adults, the disorder has a much worse prognosis, with only 15% of the patients surviving 20 years following diagnosis. One author stresses the importance of intestinal infarction, while another does not mention it. It is likely the disease is more severe and has a higher mortality rate when multiple anatomic veins are occluded. Patients with extended portal-splenic-mesenteric occlusion are likely to have more bleeding episodes, more intestinal infarction, and a higher mortality rate than patients with isolated, single vessel occlusion.
In patients reported by Belli et al, 46% of the 55 patients with portovenous occlusive disease had widespread occlusion with extended portal-splenic-mesenteric thrombosis. Prognosis and care patterns would, perhaps, be more definable if the reports described which anatomic vessels were occluded.
It is unclear what causes this disease and many factors have been implicated. Child suggested patients have "progressive portal phlebitis", and we are folloing up a patient being treated with sclerotherapy who progressively, during 3 years, obliterated the portal vein, and 2 years later obliterated the splenic veing. Other patients obviously develop large, natural collaterals around stabe anatomic occlusions and do very well for many years. The growing use of sclerotherapy may increase the incidents of venous thrombosis.
All of the patients in this report were screened and have normal antithrombin III and protein C and S levels.
Splenopneumopexy has evolved over the years from splenic transposition to a parenchymatous anastomosis with splenic artery embolization, which appears to be the currently recommended technique. The parenchymatous anastomosis appears to have been developed by Walker and Coned in 1959. Bourgeon and coworkers, in 1964, also reported using the technique of resecting a portion of the spleen and implanting it into the left lobe of the lung to produce collateral development between the portal an pulmonary circluation. Auvert et al reported, in 1966, splenopneumopexy as used currently and in 1966 Seringe et al reported combining splenopneumopexy with splenic artery ligation.
The parenchymatous anastomosis will increase the development of collateral channels. In addition to collateral vessels developing between the splenic and pulmonary venous circulations, collaterals also develop with the phrenic veins, intercostal veins, pericardiophrenic veins, and mediastinal veins. Splenic artery embolization decreases arterial bleeding when splenopneumopexy is performed, decreseses the hypersplenism, and theoretically benifits the regional varices present if the splenic vein is thrombosed. We do no know the relative importance of the splenic artery embolization compared with the splenopneumopexy.
Splenopneumopexy and splenic artery occlusion are obviously effective in lowering splenic pulp pressure and wedged hepatic vein pressure in patients with cirrhosis. Three patients in this series has splenic pulp pressures lowered by 19, 23, and 12 cm of water when postoperative values were compared with preoperative values. Following splenopneumopexy, portopulmonary shunt blood flow has been estimated to represent 47% of total portal flow.
Negative reports concerning the effectiveness of splenopneumopexy could not be found. Our experience, and that of others, suggest that this operation may play a role in treating selected patients with portal hypertension, particularly those with limited therapeutic alternatives.