Mr. Ren, 60, found a liver placeholder during a physical examination, which was significantly enlarged during the follow-up review, and the possibility of malignancy was considered. Mr. Ren previously suffered from severe hemophilia with severe cirrhosis, which was once an absolute contraindication to surgery, and once a major bleeding occurred during the operation, it was difficult to stop. Such a case, the first time encountered by the liver surgery department of Peking Union Medical College Hospital, is also very rare worldwide. Consultation on difficult diseases of hepatobiliary malignancy was quickly initiated, and under the escort of hematology, anesthesia operating room and intensive care medicine, liver surgery successfully performed left hepatoma resection for patients. The patient recovered well after surgery and was successfully discharged from the hospital after 12 days.
As early as the age of 1, Mr. Ren was diagnosed with hemophilia A, and every other day he had to inject intravenous coagulation factor VIII. to improve coagulation function. Unfortunately, he contracted viral hepatitis A at the age of 26 and again at the age of 56. At the age of 58, his hemophilia-related arthritis symptoms worsened, and he was subject to repeated bleeding from joints and deep tissues (muscles, internal organs), resulting in limited mobility of the left upper extremities, limited bilateral knee flexion, and a maximum distance of about 100 meters. At the age of 59, he found a slightly less dense shadow of the left lateral lobes of the liver, about 3.8cm×3 .6cm in size, accompanied by bloating. The review found that the occupancy increased significantly, reaching 6.7cm×5.8cm.
Mr. Ren traveled to many places and finally came to Peking Union Medical College Hospital. Based on imaging results, doctors consider that hepatic mass hyperplasm may be large. Mr. Ren is eager to receive treatment as soon as possible to relieve his pain. The Department of Liver Surgery attaches great importance to it, and after Mr. Ren was admitted to the hospital, he quickly organized a consultation for difficult diseases of hepatobiliary malignancy.
▲ Multidisciplinary experts discuss the condition
Multidisciplinary experts from hematology, oncology, radiology interventional therapy, radiotherapy, liver surgery and other disciplines gathered to examine the patient's medical history and signs in detail. Considering that the patient's liver tumor occupies a large place, with hemophilia, and the cirrhosis and portal hypertension are serious and complex, after repeated discussions, the experts finally agreed on the treatment measures: open abdominal left hepatoma resection, preoperative coagulation factor replacement therapy pre-trial, for the safe escort of surgery.
Hemophilia is a hereditary coagulation disorder with a lifelong propensity to bleed and was once an absolute contraindication to surgery. In particular, surgery on organs with abundant blood supply such as the liver, once the intraoperative bleeding occurs, it is difficult to stop, and the consequences are unimaginable. At the same time, the risk of perioperative bleeding is further increased due to the patient's combination of cirrhosis, hypersplenism, and thrombocytopenia.
With the advancement of medical technology, although there are more academic reports of surgical procedures on patients with hemophilia, cases of surgery on patients with hepatocellular carcinoma, cirrhosis and hemophilia at the same time are still very rare, with no mature experience and no guidelines to follow.
The forbidden area of life is foggy, but someone has to cut through the thorns. With the support of the Department of Hematology, the Liver Surgery Department monitored the patient's coagulation factor VIII. and its inhibitors and activated partial thromboplastin time respectively, and adjusted the coagulation factor supplementation regimen according to the pre-test results. After adequate preparation, the liver surgery team, with the cooperation of multidisciplinary teams such as the Department of Critical Care Medicine and the Department of Anesthesiology and the nursing staff of the operating room, completely removed the tumor in less than 4 hours, and the intraoperative bleeding was about 200ml. After surgery, patients will continue to be treated with platelet supplementation and alternative treatment of coagulation factors to ensure wound healing, while strengthening nutritional support, liver protection and other treatments. The patient recovered well and was successfully discharged from the hospital within 12 days, and the pathological result was reported as moderately differentiated hepatocellular carcinoma.
▲Zheng Yongchang and Xu Haifeng, deputy chief physicians of liver surgery, led the team to carry out the operation
Sang Xinting, director of liver surgery, said: "The liver surgery department of Peking Union Medical College Hospital should do surgery that others can't do, and bring patients hope for life. "The success of this operation not only saved Mr. Ren's life, but also a frontier exploration in the medical community, proving that under the framework of multidisciplinary diagnosis and treatment model, it is safe and feasible to perform liver cancer surgery for patients with cirrhosis with hemophilia through adequate preoperative assessment preparation, intraoperative fine operation and postoperative meticulous management."
Text/Dried Bamboo Yang Fan
Audit/Zheng Yongchang Du Shunda Zhu Tienan
Photo/Liver Surgery courtesy
Editor/Dried Bamboo Chen
Editor-in-Chief/Chen Mingyan
Producer/Wu Peixin