A 66-year-old Japanese male was admitted to the authors’ hospital for fever and abdominal pain. He had a past history of multiple renal cysts, chronic renal failure on chronic hemodialysis for 2 years, and multiple liver cysts due to autosomal dominant polycystic kidney disease (ADPKD). He showed the relapse of infection of liver cysts and received longtime antibiotics therapies.
On admission, his vital signs were as follows: body temperature, 39.4 °C; heart rate, 101 beats per minutes (bpm); and respiratory rate, 20 bpm. Laboratory examination revealed a white blood cell (WBC) count of 7480 cells/mm3 with a high neutrophil count of 90.7%, and a C reactive protein of 18.08 mg/dl. He was diagnosed as systemic inflammatory response syndrome (SIRS). Diffusion-weighted magnetic resonance imaging (DW-MRI) showed an abnormal high intensity at the multiple liver cysts, indicating that polycystic infection was strongly suspected. Although antibiotic therapy with intravenous meropenem at a dose of 0.5 g/day was started, fever and inflammatory reactions were unable to improve. Therefore, percutaneous echo-guided drainage was introduced for the most accumulated liver cyst. Because of the improvements of clinical symptoms and laboratory findings, the drainage tube was removed at 19 days after drainage. However, the low-grade fever and inflammatory reactions recurred, and the discontinuation of antibiotic therapy was difficult. Because the most of the liver showed multilocular cystic changes, the control of infection was considered to be difficult by conservative treatments such as a local drainage or a partial liver resection, and the severe infection was expected to be repeated in the future.
As a fundamental treatment, a surgical approach was considered to be necessary, and a liver transplantation surgery was considered to be an indication for the disease. Therefore, he was planned to undergo living-donor liver transplantation with a right lobe graft from his wife. The graft was selected with considering a graft-to-recipient weight ratio of 0.88%, which is larger than the minimal recommended ratio of 0.8%.
Before the liver transplantation, he was 168.0 cm tall and weighed 68 kg. Physical examination revealed hepatomegaly extending below the level of the umbilicus. Laboratory data showed anemia (Hb 10.4 g/dL), and the elevation of C-reactive protein (2.61 mg/dL). Liver and renal profiles were as follows: total bilirubin, 0.8 mg/dL; albumin, 3.3 g/dL; aspartate aminotransferase (AST), 17 U/L; alanine aminotransferase (ALT), 10 U/L; international normalized ratio (INR), 1.3; creatinine, 5.09 mg/dL. His Child-Pugh score was 7 (grade B), and Model for End-Stage Liver Disease (MELD) score was 22. Arterial blood gas analysis showed that blood pH was 7.44, PaCO2 32.9 mmHg, PaO2, 82.1 mmHg in room air, and alveolar-arterial oxygen tension gradient (A-aDO2) was increased to 26.8 mmHg. Chest X-ray revealed an elevation of right diaphragm. Computed tomography (CT) showed hepatomegaly with multiple liver cysts, multiple renal cysts, mild ascites, and no abnormal findings in the lung (Fig. 1). Respiratory function test showed the forced vital capacity (FVC) was mildly reduced down to 72% of predicted value, and the adjusted diffusing capacity of the lungs for carbon monoxide (DLco) by Cotes’ method was moderately reduced down to 57.8% of predicted value. Venous ultrasonography revealed lower extremity venous thrombosis, and the day before the operation, an inferior vena cava filter was inserted by a right internal-jugular-vein approach without any complications. At 114 days after admission, a living related liver transplantation was performed. After endotracheal intubation, two central venous catheters were inserted from left and right internal jugular veins before surgery without any complications.
General anesthesia was induced with propofol and remifentanil, and maintained with sevoflurane and remifentanil. The artificial ventilation was uneventful, and the airway pressure was kept below 30 cmH2O during the intraoperative period.
The abdominal cavity was reached through the upper abdominal reverse-T incision. The liver was found to swell markedly and to push the right diaphragm upward. Although the liver was firmly adhered to the right diaphragm, the adhesion was sharply dissected by electrocautery under appropriate traction. After the extraction of the liver, the right diaphragm was found to swell markedly and occupy the abdominal cavity. Although there was no significant change in the vital signs, the right pneumothorax was suspected, a thoracic drain was immediately inserted through the right tenth intercostal space, and continuous drainage was initiated. However, the right diaphragm distended by a massive air leak and prevented the operators from anastomosing the liver vessels and the bile duct. Therefore, a small relaxing incision was made in the right diaphragm for depressurizing the right thoracic cavity and making an adequate operative field to anastomose. Thoracoscopic observation was performed through the diaphragm incision. There was no adhesion in the right thoracic cavity, and the visceral pleura of the bottom of the right lung was widely expanded like a giant cyst due to dissection from the lung parenchyma, and a marked air leak was recognized from a pin hole in the dissected pleura. Since controlling the air leakage in the patient was considered difficult only by drainage, a leakage closure operation was decided to be performed after the completion of the liver transplantation. Following the transplantation procedure, the thoracoscopic leakage closure operation was performed for the patient in the supine position.
During operation, a thoracoscope was inserted through the right tenth intercostal space, and endoscopic surgical instruments were inserted through the small diaphragm incision (Fig. 2). The expanded visceral pleura was incised by electrocautery to drain air and pooled blood in the cyst, and bleeding sites in the lung parenchyma were identified and controlled by electric coagulation. Because the lung parenchyma and the visceral pleura of the bottom of the lung was widely dissected and the base of the cyst was large, cystectomy was found to be difficult to be performed by a stapler or sutures. For controlling the air leakage, absorbable fibrin sealant patches (TachoSil®, Baxtor Healthcare, Deerfield, Illinois, USA) were patched on the lung parenchyma under dissected visceral pleura, and fibrin glue (Beriplast P®, CSL Behring, Tokyo, Japan) was injected into the dissected space. After no air pooling was confirmed under the visceral pleura with positive pressure ventilation, polyglycolic acid (PGA) sheets (Neoveil®, Gunze, Kyoto) were applied, and fibrin glue was sprayed on the visceral pleura surface (Fig. 3). Finally, a 21Fr silicone thoracic tube was inserted through the right 10th intercostal space and placed on the apex of right thoracic cavity, and the diaphragm and abdominal incision was closed. Although a postoperative minor air leakage was observed, chest X-ray showed the complete expansion of the right lung. The patient was treated with water seal drainage. After the air leakage disappeared, the drainage tube was removed at 27 days after surgery. The course after liver transplantation was also smooth, and he was discharged at 67 days after surgery. The cyst on the bottom of the right lung was confirmed to disappear on chest CT after discharge (Fig. 4).
