Congenital hepatic cysts in children

　　1. Etiology

　　Liver cysts are mostly congenital and often concurrently associated with cysts in other organs, such as renal cysts. Some foreign scholars have conducted a survey of 184 patients with renal cysts and their families, finding that 64 cases of liver cysts were found in 173 autopsies, and 70% of patients with renal cysts have a family history. He proposed that these two diseases are caused by a single gene.

　　2. Pathogenesis

　　It is generally believed that liver cysts are caused by abnormal development of bile ducts in the porta hepatis during embryogenesis. Moschowitz conducted research and found that the wall of fetal cystic liver is lined with bile duct epithelium and rectangular epithelial cells. He believes that this is due to the merging of wandering bile duct epithelium with inflammatory hyperplasia and obstruction, leading to the retention of intraluminal contents and the formation of cysts. Some scholars also believe that during embryonic development, the redundant intrahepatic bile ducts did not regress spontaneously and did not connect with the distal bile ducts, forming cysts. The physiological condition of the endothelium of congenital liver cysts has been understood less in the past. Everson et al. (1990) conducted a percutaneous cystocentesis and injection of secretin (considering that the cyst endothelium is bile duct epithelial cells, and secretin has a regulatory effect on epithelial secretion) in clinical patients. The results showed that the electrolyte composition of the cyst fluid is the same as that of serum, and the γ-GT level in the cyst fluid is higher than that in serum, with a low glucose content.

　　Complications are relatively rare, and the most common symptom is intracystic hemorrhage, which is manifested by sudden severe abdominal pain and cyst enlargement. However, in a small number of patients, the abdominal pain is relatively mild, even without it, and the ultrasound shows that the contents of the cyst are fluid-like during hemorrhage. In addition, when the cyst ruptures or is complicated by infection, chills, high fever, and other symptoms may occur; compression of the duodenum can also form an internal fistula; portal hypertension, and so on. In 1977, Kasai et al. reported 3 cases of cancerous changes in the wall of liver cysts, pointing out that if the contents of the cyst are turbid and there are irregular nodules on the cyst wall, one should be vigilant about the problem of malignant transformation. In addition, multiple cysts can also be complicated with bile duct stenosis, cholangitis, obstructive jaundice, portal hypertension, and compression of the inferior vena cava.

　　Patients with congenital liver cysts often show no symptoms in their youth, but symptoms gradually appear after the age of 35 to 40, manifested as an abdominal mass in the upper abdomen, pain in the liver area and upper abdomen, usually mild pain, but if there is bleeding within the cyst, it can also be accompanied by severe abdominal pain. Compression of adjacent organs by the cyst can also cause decreased appetite, distension, pain, vomiting, jaundice, and so on; a few severe liver cysts can also cause ascites, portal hypertension, and patients with concurrent cysts in other organs can present with corresponding symptoms. Clinical examination in the early stage often shows no positive findings. For larger liver cysts, a mass in the upper right abdomen can be palpated, which is smooth, tough, and may be accompanied by tenderness if infected.

　　2, Pregnant women should try to avoid harmful factors, including staying away from smoke, alcohol, drugs, radiation, pesticides, noise, volatile harmful gases, and toxic and harmful heavy metals, in order to avoid gene mutation.

　　1, According to survey data, those with a family tendency may also have multiple organ cysts. Currently, there are no effective preventive measures. During pregnancy, regular checks should be conducted. If it is found that the child has a clear tendency for multiple cysts or abnormalities, especially giant multiple cysts, an induced abortion should be performed promptly after confirmation to avoid the birth of a child with the disease.

　　For children with congenital liver cysts without complications, even if the cysts are large, they usually do not affect liver function. Therefore, laboratory tests rarely have positive results. When complications occur, patients may show elevated levels of liver bilirubin, transaminases, alkaline phosphatase, and transpeptidase; patients with concurrent infection may show elevated levels of blood leukocytes and classifications.

　　1, Ultrasound is the main examination method for diagnosing congenital liver cysts, which is non-invasive, accurate, and the vast majority of congenital liver cysts are diagnosed by ultrasound. Ultrasound can determine the size, quantity, distribution of the lesion, and can differentiate between extraperitoneal cysts, bile duct cysts, parasitic cysts, liver abscesses, and tumor liquefaction, and so on. At the same time, ultrasound can also be used for treatment.

　　1, Computed tomography (CT) is as effective a diagnostic method as ultrasound. CT scan can show the size, shape, location, distribution, and whether there are associated cysts in other abdominal organs. At the same time, since CT can provide a more comprehensive liver scan, it is more objective and comprehensive. In recent years, the application of CT-guided liver puncture technology has made it participate in the treatment of congenital liver cysts. Other methods such as magnetic resonance imaging, radionuclide scanning, abdominal X-ray, selective celiac arteriography, and gastrointestinal contrast imaging can also be used for the diagnosis and differential diagnosis of congenital liver cysts. However, compared with ultrasound and CT scanning, they have no significant advantages. Laparoscopic examination is an invasive examination. It can diagnose and perform window treatment for liver surface cysts, but it is difficult to observe intraparenchymal cysts, so it is rarely used in clinical practice.

　　1, Avoid:Spicy, cold, sour, and spicy foods. For example, hot pot, beer, yogurt, and so on. Cold drinks can cause a great temperature difference, strongly stimulating the gastrointestinal tract, leading to sudden spasms. When gastric spasm occurs, the patient can immediately drink a cup of hot water or hot soup.

　　2, Avoid:Eat bananas and pineapples on an empty stomach. Bananas contain a large amount of magnesium, which is one of the sensitive elements affecting heart function. If eaten on an empty stomach, it will cause a sudden increase in the magnesium content in the blood. Pineapple contains strong enzymes that can hurt the stomach when eaten on an empty stomach, and its nutritional components can only be better absorbed after eating a meal.

　　3, Note:Eat more fruits and vegetables, such as bok choy, chive, ginger, lotus root, tomato, potato, chestnut, walnut kernel, peanut, and so on.

　　1. Surgical Methods for Congenital Liver Cysts

　　1. Liver Cyst Resection:

　　(1) The indications for surgery are: A. Liver cysts with obvious clinical symptoms. B. Liver cysts located in the superficial lower segment of the liver. C. Liver lobe atrophy and fibrosis caused by cyst compression (commonly seen in the left liver lobe), the atrophied liver lobe along with the cyst can be resected, liver lobectomy is not suitable for multiple liver cysts. D. Localized liver cysts with complications, such as intracystic hemorrhage, bile fistula, chronic infection, suspected malignant transformation, should undergo cyst resection. E. Patients who can withstand major surgery.

　　(2) Contraindications for surgery include: A. Elderly patients with incomplete function of important organs. B. Multiple liver cysts or polycystic liver. C. Cysts located deeply, close to important structures near the liver hilum, extensive stripping area, abundant bleeding during cyst wall separation, technically difficult.

　　2. Liver Cyst Windowing Surgery (Opening of Liver Cyst):This surgical method is simple, minimally invasive, suitable for decompression and drainage of multiple liver cysts (polycystic liver) and solitary simple liver cysts without complications, generally with good effects, but sometimes recurrence of cysts may occur due to adhesion and obstruction of abdominal viscera at the opening. The surgical method is to remove a piece of cyst wall and liver capsule protruding to the liver surface. There are two methods: laparotomy and laparoscopy.

　　(1) Indications for surgery: A. Large cysts protruding to the liver surface with obvious clinical symptoms. B. Clear diagnosis, cyst without complications. C. Treatment of cysts during other upper abdominal surgeries (most commonly cholecystectomy). D. Patients who are suitable for surgery.

　　(2) Contraindications for surgery: A. Liver cystic lesions caused by other reasons. B. Communicating multiple liver cysts. C. Liver cystic adenoma. D. Liver cysts with complications. E. Small asymptomatic cysts. F. Cysts located deeply without protruding from the liver surface.

　　2. Liver Cyst Sclerosis Treatment

　　Simple liver cysts can be treated by injecting vascular sclerosing agent (commonly anhydrous ethanol 95% to 99.8%) into the cyst cavity to destroy the endothelium of the cyst. After 1 to several times of aspiration and medication, the cyst cavity can gradually shrink, achieving good short-term effects. For smaller liver cysts.