2021年 · 第36卷第09期

The patient is a 52-year-old male, a civil servant, who was admitted to our hospital on December 13, 2018 due to "anorexia and abdominal distension for more than half a month". In the past 5 years, there have been repeated nasal and gum bleeding, memory loss, vague speech, and involuntary trembling of limbs. The younger brother died of cirrhosis after hepatitis B. Admission physical examination: liver disease face, hobbling gait. The skin and mucosa of the whole body are slightly yellowed, and liver palms and spider nevus can be seen. The abdomen is slightly swollen, soft, mild tenderness, no rebound pain, liver and spleen are not reached under the costs, and mobile voicing (-). Involuntary tremor of the limbs, increased muscle tone of the limbs, and positive bilateral pathological signs. Admission completed relevant tests, liver function: albumin 32.48 g/L, total bilirubin 47.87 µmol/L, direct bilirubin 14.19 µmol/L, indirect bilirubin 33.68 µmol/L, aspartate aminotransferase 87.88 U/L, alanine aminotransferase 77.70 U/L, cholinesterase 2 068.73 U/L. Blood routine: platelet count 61×10⁹/L. Serum ceruloplasmin 0.034 g/L, prothrombin activity 44.2%; Alpha-fetoprotein 8.6 ng/ml. Six items of hepatitis B, IgM antibody determination of hepatitis A, four items of blood transfusion, autoantibody combination and autoimmune liver disease antibody combination were not obviously abnormal. Magnetic resonance examination showed that rich blood supply occupied the space in the liver, growing across the S4 and S8 segments, and the size was about 5.2 cm ×5.0 cm, considering liver cancer; Liver cirrhosis, splenomegaly with portal hypertension and collateral vein opening, and a little ascites; Cholecystitis (Figure 1）。 Electronic gastroscopy revealed esophagogastric varices. Fundus examination under slit lamp revealed K-F rings visible in both eyes. At 24 hours, urine copper was 52.6 µg and blood copper was 0.681 mg/L. Cranial magnetic resonance examination showed abnormal symmetric signals in bilateral basal ganglia and cerebral feet. Considering metabolic encephalopathy, combined with the medical history, the possibility of hepatolenticular degeneration is high. Clinical diagnosis: hepatolenticular degeneration, metabolic encephalopathy, liver cancer, cirrhosis with esophagogastric varices, ascites, splenomegaly. It is recommended to improve the detection of ATP7B gene mutation, and the clinical diagnosis is clear. Due to financial difficulties, the gene mutation has not been detected. Multidisciplinary comprehensive diagnosis and treatment opinions: the patient has space-occupying lesions in the liver and elevated alpha-fetoprotein, so the possibility of liver cancer needs to be considered; Liver function score: Child grade 8, grade B; MELD scores 14. Single tumor diameter ≤6.5 cm; No macrovascular invasion, in accordance with Hangzhou Standard Class A^［1］； If economic conditions permit, liver transplantation is feasible. Liver transplantation was performed in our hospital on January 10, 2019. Postoperative pathology: (liver) moderately differentiated bile duct adenocarcinoma, hepatocytes were positive for copper staining (Figure 2）。 Upper abdominal CT revealed normal liver shape and size. After 1 year follow-up, the patient's liver function recovered well, and the extrapyramidal signs such as involuntary limbs fibrillation, vague speech and unstable walking were significantly improved.

A 56-year-old female was seen for "repeated epigastric discomfort for 1 month". Physical examination: There was no yellowing stain on the sclera, and mild tenderness in the right upper Laboratory tests after admission showed no abnormalities in blood routine, liver function, tumor indexes and coagulation function. MRI of the biliary tract: choledochal cyst type I. Upper abdominal enhanced CT: cystic dilatation of the common bile duct with low bifurcation of the left hepatic artery (Figure 1）。 Diagnosis: Congenital choledochal cyst. "Laparoscopic choledochal cyst resection plus bile duct-jejunal Roux-Y anastomosis" was performed on the 4th day of admission after perfect preoperative preparation. After general anesthesia, the patient underwent 5-hole laparoscopic surgery. During the operation, the small omental sac was opened first, and the ligament of the small omental sac of the left liver was clamped with a vascular clip (Figure 2) is used to suspend the left liver, which is convenient for exposure of the first hepatic hilum and hepatoduodenal ligament during operation. Choledochal cyst was completely removed by surgery, and precolonic bile duct-jejunostomy was performed. The operation went smoothly, taking a total of 230 min, with intraoperative bleeding of 100 ml. The patient was successfully extubated and returned to the ward after surgery. His blood pressure was slightly lower, 98/66 mmHg (1 mmHg =0.133 kpa), and his body temperature reached 39 degrees. He was given physical cooling and anti-inflammatory therapy. Six hours after operation, the patient's heart rate increased from 84 beats/min to 110 beats/min, and his breathing increased from 20 beats/min to 26 beats/min. He showed chills and shaking, and his body temperature rose to 40 degrees. After administration of drugs and physical cooling, his body temperature did not decrease significantly. The oxygen saturation decreased to 90% at 12 hours after surgery, and increased to 96% after the mask was given oxygen inhalation. Only 30 ml of light blood fluid flowed out of the abdominal drainage tube. Emergency blood routine: white blood cells 2.1×10⁹/L, neutrophils 90%; PT was extended to 20 s (reference value 11~14.5 s); Liver function : ALT 3 006 U/L, AST 4 069 U/L; D-dimer>20 mg/L; Chest CT: a small amount of pleural effusion on both sides, local pulmonary consolidation, and no pulmonary artery embolism; Enhanced CT of the abdomen: left hemihepatic ischemia with vacuolar necrosis. Discipline MDT discussion, diagnosis considered left hemihepatic ischemic necrosis with severe infection. Reoperation was performed 18 h after the operation, and complete ischemia, necrosis and swelling of the left liver were found during the operation (Figure 3), peripheral exudation, the left hepatic artery was clamped by a suspension vascular clip, and the left hemihepatic was completely removed during the operation. Postoperative pathology: coagulative necrosis of liver tissue. The tube was taken to the intensive care unit after operation. The postoperative body temperature and heart rate became normal, and the liver function ALT decreased to 1 829 U/L and AST decreased to 1 141 U/L on the first day after operation; D-dimer was reduced to 11 mg/L; Liver function ALT decreased to 968 U/L and AST to 300 U/L on the second postoperative day; The extubation was successful and returned to the general ward on the 3rd day after surgery. ALT decreased to 182 U/L and AST decreased to 55 U/L on the 6th postoperative day; D-dimer was reduced to 3 mg/L. There was no bile leakage after the operation, and the function of the digestive tract returned to normal. He was successfully discharged from the hospital on the 15th day after the second operation. After 1 month follow-up, his liver function had completely returned to normal.

A 13-year-old child was seen in an outside hospital for "low back pain with fever for 5 days and dyspnea for 3 days". Ultrasound: No abnormalities in kidney, inferior vena cava thrombosis (Figure 1）。 CT of the lungs: flaky exudation of both lungs (pneumonia). Thrombolysis and anti-inflammatory therapy were performed through jugular vein puncture, but the patient's symptoms did not improve, so he was admitted to our hospital for further diagnosis and treatment. Emergency ultrasound of inferior vena cava: ① inferior vena cava thrombolytic catheter; ② Thrombosis below the level of inferior vena cava and renal vein with severe luminal stenosis was significantly extended compared with the range before thrombolysis (Figure 2）。 WBC 2.90×10⁹/L, neutrophil ratio 91.69%; CRP 191.8 mg/L, FDP 91.81 ng/L, D-dimer 7.6 μ g/ml. Blood culture: Staphylococcus aureus positive. Diagnosis: severe pneumonia, sepsis, inferior vena cava thrombosis with abscess. Removal of jugular vein catheter, anticoagulation, anti-infection, intravenous injection of human immunoglobulin support therapy. On the 6th day of hospitalization, the body temperature was as high as 38.8℃, and intermittently complained of chest tightness, dyspnea, irritability and low back pain; WBC 15.08×10⁹/L, neutrophil ratio 93.42%; CRP 286.00 mg/L, FDP 15.07 ng/L, D-dimer 1.35 μ g/ml. Lung CT: Extensive multiple clusters and flaky nodules in both lungs, with small cavities in some lesions, multiple infections are considered. Follow-up to the medical history, the child suffered from facial boils 1 month ago, with a large range of involvement, a deep location, a history of crushing, and a prolonged self-healing. On the 16th day, the temperature returned to normal, and I had a paroxysmal cough with right chest pain, occasionally complaining of low back pain. CT: Most of the multiple spherical or mass lesions in both lungs form cavities or cavities, and the inner walls of most lesions are smooth, thick and uneven; Left lung pneumothorax (Figure 3）。 Continue fighting the infection until the lung infection disappears. After 3 months of anticoagulant therapy, color ultrasound showed disappearance of inferior vena cava thrombosis.

isolated distal deep venous thrombosis (IDDVT) is a subtype of lower extremity deep vein thrombosis (DVT), which includes anterior tibial vein, posterior tibial vein, peroneal vein, and intermuscular venous plexus thrombosis that occurs below the plane of the knee joint^［1］。 According to the International Association for Thrombosis and Hemostasis (ISTH), the incidence of DVT worldwide is about 1.15‰ to 2.69‰^［2］While the incidence of IDDVT accounts for about 20% ~50% of DVT^［3］。 Because the symptoms of IDDVT are changeable and the onset is hidden, the diagnosis and treatment of IDDVT often occur with overtreatment or failure to treat it in time and reasonably. However, the guidelines at home and abroad have not specifically elaborated on the diagnosis and treatment of IDDVT. Therefore, the author hopes to provide some consensus opinions in the vascular surgery community on the reasonable diagnosis and treatment of IDDVT.