Overview.
Cyanosis-pestle finger-hepatopathy syndrome is also known as Fluckiger syndrome and cyanosis-pestle finger-hepatopathy syndrome. A case of cirrhosis with cyanosis and pestle finger was first reported by Fluckiger in 1984. A study was also reported in 1990 in China.
Causes
Cyanosis may be caused by an abnormal substance produced by the liver that leads to arteriovenous shunting in the lungs, resulting in an increase in reduced hemoglobin in the blood or the presence of abnormal hemoglobin derivatives, such as methemoglobin and hemosulfated hemoglobin, which cause the skin and membranes to appear purplish-blue in color. Cyanosis can appear in the skin and mucous membranes throughout the body, but in the thinner skin, less pigmented and capillary-rich blood circulation endings, such as the lips. It is more easily observed on the tongue, mouth, tip of the nose, cheeks, earlobes, and nail beds. The dye-dilution test may indirectly indicate an arteriovenous shunt in the lungs and suggest the severity of cirrhosis. It may also be a manifestation of hepatopulmonary syndrome.
Symptoms
The disease can affect both men and women at any age, but is more common in children. Signs and symptoms of cirrhosis are present. Cyanosis may be accompanied by dyspnea, followed by the development of mortar and pestle fingers.
Examination
1. Medical history
The history is important in identifying the cause of cyanosis, with particular attention to the mode of onset and age of the patient. Acute generalized cyanosis with impaired consciousness but not obvious dyspnea should be noted as chemical cyanosis; the sudden onset of generalized cyanosis in children or the infirm after eating sauerkraut should be noted as cyanosis of enteric origin, and cyanosis in infants and young children after enemas should be thought of in the absence of misuse of nitrites; cyanosis occurring with the menstrual cycle is characterized by idiopathic, paroxysmal methemoglobinemia.
2.Physical examination
The main purpose is to know whether the patient has signs of cardiac, pulmonary or thoracic diseases, thoracic deformity, heart murmur, etc., as well as the condition of blood circulation at the site of cyanosis.
Diagnosis
Cirrhotic patients with cyanosis and pestle finger can be diagnosed if cardiopulmonary diseases are excluded.
Treatment
The mainstay of treatment is hepatic cirrhosis, with cyanosis decreasing as portal pressure falls. Periportal vascular dissection and splenic vein-deep vein anastomosis can be performed to reduce portal pressure and minimize complications. However, this type of surgery carries an increased risk of hepatic encephalopathy, so the best procedure is pericardial vascular dissection. Symptomatic supportive therapy such as oxygenation, maintenance of airway patency, and hepatoprotection can be given at the same time.