2017年 · 第55卷第12期

Abernethy's malformation is a rare malformation of the portal system, first described in detail by Abernethy in 1793. It is mainly characterized by abnormal portosystemic shunt, also known as congenital extrahepatic portosystemic shunt (CEPSS). According to the presence or absence of a portal vein, Abernethy's malformation can be divided into two types: type I with congenital portal vein absence and type II with portal vein dysplasia^[1]。 According to different types, the surgical treatment methods are also different. Type Ⅰ is often treated with liver transplantation due to the absence of portal vein, which causes continuous insufficient blood supply to the liver, which easily leads to cirrhosis and liver cancer^[2,3]； Type Ⅱ can use the method of ligating the abnormal portosystem shunt blood vessel to increase the blood supply of the liver^[4,5]。 We treated 5 children with type Ⅱ Abernethy malformation with Rex surgery combined with portosystemic shunt ligation and portosystemic shunt ligation alone. The results are reported below.

A 37-year-old male was admitted to the hospital on 31 August 2013 due to "upper abdominal pain for 2 d". The patient had sudden distension and pain in the upper abdomen 2 days before seeing the doctor, with paroxysmal aggravation, accompanied by nausea and vomiting, and the vomit was stomach content. The results of abdominal CT examination in the local hospital showed acute pancreatitis (AP). The pain did not improve significantly after symptomatic supportive treatment such as anti-inflammatory and fluid rehydration, and it was accompanied by systemic muscle soreness and fatigue, so it was transferred to the pancreatic center of our hospital for treatment. During the course of the disease, the patient did not eat or eat, his urine and stools resolved themselves, and his urine was brown, accompanied by low fever. Physical examination at admission: body temperature 38.1 ℃, pulse 120 beats/min, respiratory rate 24 beats/min, blood pressure 152/90 mmHg (1 mmHg =0.133 kPa), flat and soft abdomen, obvious tenderness in the whole abdomen, rebound pain, insignificant muscle tension, no mass, no subcostal reach of liver and spleen, negative Murphy sign, negative mobile voiced sound, and normal intestinal sound. Auxiliary examination after admission: arterial blood gas pH 7.371, PaO₂162 mmHg (nasal tube oxygen inhalation 5 L/min), PaCO₂162 mmHg with 6 mmol/L of base remaining; Serum amylase 121 U/L (normal value: 0-110 U/L), serum lipase 241.1 U/L (normal value: 0-60 U/L); Biochemical examination: urea nitrogen 7.03 mmol/L, creatinine 99.3 μ mol/L, ALT 42.7 U/L, AST 63.6 U/L, creatine kinase (CK) 7 337 U/L (normal: 25-190 U/L), lactate dehydrogenase 2 150 U/L (normal: 313-618 U/L), triglyceride 9.65 mmol/L, blood glucose 18.0 mmol/L, blood calcium 2.07 mmol/L; myoglobin>1 000 μ g/L (normal value: 0-46 μ g/L); Blood routine: white blood cell count 7.78×10⁹/L, neutrophil percentage 86%, hemoglobin concentration 156 g/L; Urine routine: urine glucose (+ + +), urine ketone body (-), urine protein (-); Glycosylated hemoglobin 9.3%. Abdominal enhanced CT examination showed pancreatic swelling and large peripancreatic exudation, which was in line with AP manifestations (Figure 1，Figure 2)。

A 36-year-old male was admitted to the Department of Hepatobiliary and Pancreatic Medicine of the First Hospital of Jilin University on June 1, 2011 mainly due to "abdominal distension and abdominal pain for 1 week". Physical examination: No mass was palpable in the abdomen, tenderness in the whole abdomen, mainly under the umbilicus, no rebound pain, and slight tension in the abdominal muscles. Abdominal CT examination showed: cystic space-occupying lesions in the middle and lower abdomen, with high possibility of co-infection, accompanied by peripheral mesangial exudation and slight enlargement of multiple lymph nodes in the abdominal cavity (Figure 1)。 Blood routine results: white blood cell count 10.8×10⁹/L, neutrophil percentage 80%. Admission diagnosis: abdominal cystic mass complicated with infection. Antibacterial drugs were given to fight the infection. After the abdominal pain was significantly relieved, the patient underwent partial small intestine resection and intestinal anastomosis on June 9, 2011. During the operation, a cystic mass was seen in the terminal mesileal, running parallel to the intestinal lumen, with a size of 30 cm ×5 cm. The distal end communicated with the terminal ileal wall, and the proximal end was blind. Intraoperative diagnosis: small intestinal duplication malformation (extraintestinal tubular type), partial small intestinal resection and enterostomy were performed after sufficient free duplication of small intestine, and the patient recovered well after operation. Pathological examination results: Duplicate malformation of small intestine (the intima of the duplicate intestine is mainly covered with squamous epithelium, mucous gland tissue is seen under the epithelium, and a few parts are covered with small intestinal epithelium).

The patient was a 35-year-old male, right-handed. He was admitted to the First Hospital of Jilin University on December 16, 2015 because he was found to occupy space in the spinal canal for 2 years with swelling and pain in the left lower limb for 3 months. Two years ago, the patient underwent lumbar internal fixation in the local hospital due to lumbar disc herniation. During the operation, it was found that the spinal canal occupied space, but the tumor was not removed due to the restrictions of the local hospital conditions. Physical examination: There was no melanin nevus and pigmentation in the skin and mucosa of the whole body, clear and sharp speech, muscle strength of both lower limbs grade V, normal muscle tone, swelling and pain in the left lower limb and left buttock, hypoesthesia on the outside of the left calf, physiological reflex, and pathological reflex were not drawn out. Lumbar spine MRI plain scan + enhanced display (Figure 1): L_4～5Changes after vertebral internal fixation, L_4～5Planar spinal canal and left intervertebral foramen occupy space, consider schwannoma, L_3～4and L₅~ S₁Mild disc herniation. Preoperative diagnosis: L₅Schwannomas. On December 20, 2015, intraspinal tumor resection was performed under general anesthesia. During the operation, part of the tumor invaded the dura mater and vertebrae, and the dura mater was incised longitudinally. The tumor originated from the nerve root, was gray, tough in texture, with abundant blood supply, and the size was about 3.3 cm ×3.0 cm ×2.6 cm. The tumor was removed in blocks under the microscope. Histopathology: Tumor cells have obvious atypia, loose intercellular connections, large and deformed nuclei, obvious nucleoli, and mitotic images. Immunohistochemical results (Figure 2): broad-spectrum keratin (+), cytokeratin 20 (-), cytokeratin 7 (-), Ki-67 (+20%), villin (-), vimentin (+), acid calcium binding protein (-), CD10 (-), cytokeratin 5/6 (-), calretinin (partial +), desmin (-), epithelial membrane antigen (+), phosphatidylinositol proteoglycan 3 (-), inhibin (-), Wilm's tumor gene 1 (-), chromaffin (-), hepatocyte monoclonal antibody (-), progesterone receptor (-), synaptophysin (-), pairing box gene 2 (-). Pathological diagnosis: malignant mesothelioma. The distension and pain of the patient's left lower limb and left buttock disappeared after operation, and there were no related complications such as urinary and urinary incontinence. The numbness of the left thumb existed and disappeared 9 days after operation. There were no obvious abnormalities in the postoperative lung CT and whole abdominal CT examinations. The patient was hospitalized for 15 days and discharged without chemoradiotherapy. The patient underwent PET-CT at 3 months postoperative reexamination (Figure 3) indicates: L_4～5Hypermetabolic foci in the spinal canal, considering postoperative changes or residual tumor activity in them; L_4～5The spinous process was absent and the metabolism of the adjacent muscles was increased. Taking into account the postoperative changes, the standardized maximum uptake was 8.5. Eight months after the operation, the patient experienced posterior lumbar pain and difficulty in defecation, and the lumbar spine MRI was reviewed to consider tumor recurrence. On September 2, 2016, the patient underwent the second operation. During the operation, it was found that the tumor invaded the surrounding bone seriously, the vertebral body and spinal cord were invaded, and it was impossible to completely resect or perform artificial bone replacement. Subtotal resection was performed, and the patient's pain was relieved after the operation.

The 4th National Academic Forum for Young Physicians in General Surgery and the competition of "Chinese Young Scholars in Surgery" jointly sponsored by the Surgery Branch of Chinese Medical Association and the Editorial Committee of Chinese Journal of Surgery have been successfully concluded recently. This forum aims to promote academic exchanges among young general surgeons, encourage more outstanding young talents to stand out, discover and cultivate academic talents of young general surgeons, and select reviewers and reserve talents of the editorial board of Chinese Journal of Surgery. At the same time, the forum selected the "2017 Chinese Surgical Young Scholar Award".