Polycystic liver

　　1. Etiology

　　Most polycystic liver is congenital, caused by developmental disorders of intralobular bile ducts. During the period of embryonic development, the excess bile ducts regress spontaneously without connecting to the distal bile ducts; if the excess bile ducts in the liver do not regress and absorb, and gradually become segmented and cystic in expansion, polycystic liver can form. Polycystic liver is often accompanied by polycystic kidney, pancreatic cysts, lung or spleen cysts, and other malformations, such as cerebral aneurysms, diverticula, double ureters, horseshoe kidney, or ventricular septal defect, which can also serve as evidence of congenital developmental abnormalities. Polycystic liver can occur in different family members of the same family and is non-dominant hereditary.

　　2. Pathology

　　Most cysts in polycystic liver are multiple, a few are solitary, and some are solitary due to the fusion of multicystic cysts. The vast majority of polycystic liver involves the entire liver, containing clear, bile-free fluid inside. The liver increases in size and shape, with grayish-white cysts of varying sizes visible on the liver surface, which can be as small as a needle tip and as large as a child's head, and the liver section appears honeycomb-like. Cysts can also be dense in one lobe of the liver, with the right lobe being more affected. The size of the cysts can range from needle tip size to 8-10 cm, but rarely exceed 10 cm. The cyst fluid can be as much as 2000 ml. The cyst wall is thin and can contain clear fluid, serous fluid, or gelatinous fluid, and can be hemorrhagic or purulent in cases of hemorrhage or infection. It does not contain bile, and the specific gravity is usually 1.010.

　　Cysts generally increase slowly with age, but the liver volume can remain unchanged for several years. When the cystic septum ruptures, it can fuse into larger cysts. Histologically, there is no change in the liver lobular structure, liver cells are normal, and cysts are surrounded by a fibrous capsule. The cyst wall is composed of collagenous connective tissue, lined with columnar epithelium or flat bile duct epithelium, and the outer layer is collagenous tissue. If there is a lot of fluid inside the cyst, the epithelium can become flat or even absent. In addition to the liver and kidneys, other organs such as the pancreas, spleen, lungs, ovaries, and seminal vesicles can also have cysts. This disease can also be accompanied by other congenital malformations, including spina bifida and polydactyly. The space between cysts is generally normal liver tissue, and in patients with long-standing disease, fibrosis and bile duct hyperplasia can occur, which can lead to liver dysfunction, liver cirrhosis, and portal hypertension in the late stage.

　　Cysts generally increase slowly with age, but the liver volume may remain unchanged for several years. When the cyst septa rupture, they can fuse into larger cysts. When the liver cysts reach a certain size and affect health, such as the appearance of poor digestion and absorption, or complications such as cyst rupture and infection, surgical treatment should be considered. Acute severe abdominal pain, hematemesis, or black stools should be treated immediately. Long-standing cases may also develop fibrosis and bile duct hyperplasia, leading to liver dysfunction, liver cirrhosis, and portal hypertension in the later stage.

　　After suffering from multiple liver cysts, the first symptoms to appear are gastrointestinal symptoms. Such as indigestion, decreased appetite, nausea, vomiting, and right upper quadrant pain, but the degree is not severe. Liver enlargement and a mass in the right upper quadrant are palpable with a cystic sensation, without significant tenderness. Secondary infection may cause chills and fever.

　　The surface of the liver in patients with multiple liver cysts can be felt to have scattered cystic nodules. If there is bleeding within the cyst, combined infection, or pedunculated cyst torsion, acute abdominal symptoms may occur. Rarely, there may be jaundice due to compression of the common bile duct or hepatic duct by a large cyst, which may not have positive signs, and some patients may feel an abdominal mass.

　　After suffering from multiple liver cysts, the first symptoms to appear are gastrointestinal symptoms. Polycystic liver can severely affect the patient's daily life, so it should be actively prevented. In daily life, one should pay attention to avoiding collisions with the right intercostal and right upper abdomen to prevent cyst rupture.

　　1. Laboratory tests

　　Cyst hemorrhage may occasionally cause anemia, with white blood cell count generally normal, but it can also be as high as 76×10/L. Changes in liver function tests are not proportional to liver enlargement, and only occasionally there is a slight increase in indirect bilirubin and BSP. Alkaline phosphatase elevation is only seen in some cases. Blood urea nitrogen and creatinine may increase in patients with reduced renal units in polycystic kidney disease.

　　2. Imaging examinations

　　Ultrasound shows areas of varying sizes and cavities, and liver scanning reveals multiple defects. Barium meal examination of the gastrointestinal tract shows displacement of the stomach and duodenum, and the colon. Renal pelvis venography may show signs of polycystic kidney disease. Laparoscopic examination shows liver enlargement with many cysts on its surface. Liver biopsy may sometimes yield cyst wall tissue. CT shows smooth, well-defined circular low-density lesions with linear edges, uniform density of the cyst contents, no septation, and can be diagnosed by unenhanced scan. After contrast enhancement, the contrast is clearer. CT values >20Hu may be seen in cases of hemorrhage or secondary infection. Liver angiography shows avascular areas, forcing vessel displacement.

　　Clinically, for those over 30 years old, with healthy appearance, liver enlargement, normal liver function, and accompanied by polycystic kidney disease. Those with family history should be suspected of having polycystic liver disease. If there are compression symptoms and the above special examinations are performed, and the diagnosis is still suspicious, laparotomy exploration can be performed.

　　1. Diet is very important for the postnatal care of patients with polycystic kidney disease and polycystic liver disease. In general, it is not advisable to eat salty and fishy foods, pay attention not to be too hungry or too full, too cold or too hot, so as not to hurt the spleen. For those with high blood uric acid and high blood pressure, a low purine, low-fat diet should be adopted, and animal internal organs and high-fat foods should be avoided. For those with chronic renal failure in the later stage, attention should be paid to controlling the intake of protein in food.

　　2. Patients with polycystic kidney disease and polycystic liver disease should avoid strenuous exercise, wearing halter tops, and not using abdominal safety belts around the abdomen. This can prevent the kidneys from being injured due to collision or compression.

　　3. Patients with polycystic kidney disease and polycystic liver disease should avoid kidney infection, avoid taking a bath and holding urine, urinate immediately after sexual intercourse, try to avoid urinary catheterization, and if there is intracystic infection, seek treatment from a kidney specialist physician and extend the use of antibiotics (4-6 weeks).

　　4. For patients with polycystic kidney disease and polycystic liver disease complicated with kidney stones, they should drink an adequate amount of water every day to reach a urine output of 2 liters per day. If it is still not possible to excrete, consider surgery.

　　Treatment mainly includes low copper diet, reducing copper absorption, increasing copper excretion, and liver transplantation. The first choice of medicine is D-penicillamine. Liver transplantation is the best treatment for the disease.

　　Polycystic liver disease, if accompanied by polycystic kidney disease, etc., belongs to polycystic organs, often related to genetic factors. For larger liver cysts, if the patient has symptoms, consider ultrasound-guided puncture and fluid aspiration treatment, but the effect is not very satisfactory. If it seriously affects liver function, further treatment should be considered.