Patient, male, 40 years old. He was seen at a local hospital for spontaneous abdominal pain, with progressive decrease in blood pressure and diagnostic abdominal puncture to draw non-coagulated blood, and an emergency dissection was performed. About 2000 ml of abdominal bleeding was seen, and active bleeding was found at the branch of the superior mesenteric artery at the inferior margin of the pancreas. Due to surgical technique and conditions, complete hemostasis could not be achieved, and only the large omentum was used to wrap the hematoma in order to confine the bleeding relatively to the small omental sac. Postoperative hemostasis, blood transfusion, fluid replacement and symptomatic treatment were performed. He was referred to our hospital on January 18, 2007 for further consultation and treatment. Detailed follow-up was performed with a family history of hypertension and bleeding tendencies such as soft tissue hematoma and epistaxis, and his brother died early due to cerebral hemorrhage. Physical examination: T 36.7℃, P 130 times/min, R 25 times/min, BP 100/70 mmHg. severe anemic appearance, epigastric subxiphoid mass 13 cm in diameter, peritoneal irritation sign (+), mobile turbid sounds (+), bowel sounds 4-6 times/min. on admission, white blood cells 10.1×109/L, neutrophils 0.82, hemoglobin 110 g/L, platelets ( PLT) 126×109/L, prothrombin time (PT) 21.4s (normal control value 11-13s) and partial thromboplastin (APTT) 63.6s (normal control value 30-45s), urinary occult blood (++). fⅧ:C 5.7% (normal value 50%-150%). Admission diagnosis: hypertension, abdominal hemorrhage, hemorrhagic shock. Blood transfusion, rehydration, anti-shock nutritional support, symptomatic treatment. further improvement of auxiliary examinations and organization of hospital-wide consultation on January 19, considering the complex and critical condition, and by hematology consultation, the diagnosis of hemophilia A was considered to be established. in addition, due to the first surgery hematoma mechanization, normal anatomy could not be revealed, the preferred treatment plan was angiography and interventional therapy. Ren Hui, Department of Colorectal and Anorectal Surgery, Second Hospital of Jilin University
On January 20, the first interventional angiography was performed, and hemorrhage from the branch of superior mesenteric artery was considered; on January 22, another interventional angiography was performed, and the diagnosis was: ruptured hemangioma at the root of the branch of superior mesenteric artery (whether it was a true aneurysm or a pseudoaneurysm could not be defined at the moment, and pseudoaneurysm was considered to be more likely), active bleeding, and the epigastric mass was a hematoma (Figure 1a and b). Since the ruptured bleeding was at the root of the superior mesenteric artery, embolization would inevitably affect the blood flow of the whole small intestine and even the right hemicolectomy, and even necrosis, which could not be embolized. The abdominal pain worsened, the epigastric mass gradually increased in size, and the whole abdominal pressure pain appeared, especially obvious in the upper abdomen. Non-clotting blood is drawn by laparotomy. Hemoglobin was progressively decreasing. He explained his condition to his family and performed another dissection on January 23: the large omentum was wrapped and the omental sac was full of fresh blood and clots, the volume was about 2500 ml, some of the hematoma was removed, the pancreatic parenchyma was dark, the surrounding inflammation was heavy and the normal anatomy could not be revealed (Figure 2), two active ruptures and bleeding of the superior mesenteric artery were seen, they were ligated and closed with local protein gel, two abdominal drains were left in place. The abdomen was closed and the operation was completed.
On January 23, the patient’s P was 110 beats/min, R 20 beats/min, BP 160/90 mmHg. 150 ml of dark red blood fluid was drained from the abdominal drainage tube, PT 18.6s, APTT 48.4s, fibrinogen (FIB) 1.58 g/L, PLT 66×109/L. On January 27, flaky bruises around the incision and 400 ml of fresh blood was drained from the abdominal drainage. bedside B ultrasound showed bilateral restricted pleural effusion, and thoracentesis drew 500 ml of bloody pleural fluid from the left side of the chest and 100 ml from the right side. the patient was comatose in the early morning of 28th and died due to multi-system organ failure.
2 Discussion
Hemophilia A is a hemorrhagic disease with X-chromosome chain recessive inheritance. Based on clinical manifestations, laboratory tests and the fact that the patient was male, hemophilia A was diagnosed. It is an X-linked recessive disorder with gene locus Xq28. It is the first among hereditary coagulopathies, accounting for 79.4%, and has a wide regional and ethnic distribution. It is caused by a defect in the factor VIII (F VIII) gene, which leads to a decrease in the procoagulant activity of F VIII in the circulation and causes bleeding. This case is only a patient with hemophilia A. The medical history and family history are typical. Patients with hemophilia should avoid surgery and trauma. In this case, the patient with hemophilia had a malformation of the superior mesenteric artery vasculature and a family history of hypertension, and died of spontaneous abdominal hemorrhage. The patient had vascular hemophilia syndrome, a subtle deletion of large molecular weight (HAW) multimers in vascular hemophilia factor (vWF) in such patients, resulting in congenital abnormal vascular development. The rate of combined vascular malformations at various sites in patients with hemophilia still needs to be reported in large case reviews.
Surgeons need to be more aware of hemophilia. In this case, the local first-time surgeon only paid attention to abdominal bleeding and hemorrhagic shock and neglected to take a detailed preoperative history, but it was not contrary to the principles of general surgery. When the patient was transferred to our hospital, although the platelets and other tests of the emergency test were in the normal range, it was because the hemorrhagic shock was not treated with better transfusion and rehydration, and the test results were “normal” due to blood concentration, and the real data of the blood test indexes were revealed only after a large amount of rehydration at the right time. Therefore, for patients with hemorrhagic shock, surgical rehydration is extremely important and quite critical. If the diagnosis of hemophilia is made early or the possibility of hemophilia is considered, fresh blood, plasma or factor F VIII can be transfused, and the consequences can be expected to be better if the causes of internal bleeding are prevented and treated early. In the case of hemophilia, attention should be paid to: (1) preoperative supplementation of F VIII; (2) intraoperative use of fibrin glue containing F VIII to close bleeding points; and (3) postoperative control of bleeding and infection. In this case, it was not possible to stop the transfusion of fresh plasma and cold precipitation, so there was not enough time to perform the relevant tests to further clarify the diagnosis of hemophilia.
Angiography and interventional techniques were preferred in this case, but because the location of the bleeding lesion was located in the vascular branch of the root of the superior mesenteric artery, neither embolization nor stenting was applied to ensure that the blood supply to the small intestine and right hemicolectomy of the superior mesenteric artery branch was not affected, or even completely blocked, resulting in ischemic necrosis of the intestine. Therefore, if the patient still has progressive bleeding, re-operation is not an option.
The only way to fight for survival is to find the bleeding point clearly and stop the bleeding exactly in the first surgery. However, due to (1) the limitations of local surgical level and technology, it was not possible to fully reveal the branches of the superior mesenteric artery at the lower margin of the pancreas, and the surgical anatomy of the pancreas and superior mesenteric artery system is indeed a difficulty in general surgery; (2) or due to the critical condition of the patient at that time, it was not possible to have sufficient time to find and reveal a clear bleeding point, so only palliative treatment was performed, and thus, due to the mechanization of the hematoma and severe local inflammatory reaction, the opportunity for the second surgery was lost. the opportunity for a second surgery was lost.