Inspissated bile syndrome: Definition, Description, Causes and Risk Factors:Inspissated bile syndrome is defined as partial or complete obstruction of the extrahepatic biliary system by impaction of thick bile or sludge in the distal common bile duct during the neonatal period.Bile is a very alkaline liquid made in liver, it is stored in the gallbladder and secreted by common bile duct into small intestine. There, it mixes with food that comes from the stomach. A very important role played by bile is neutralizing the acidity of stomach contents as they enter the small intestine.Bile duct obstruction by inspissated bile is a rare but correctable cause of conjugated hyperbilirubinemia in infancy. The condition is associated with cystic fibrosis, erythroblastosis fetalis and total parenteral nutrition. The composition of inspissated bile syndrome varies. The most common particulate components of inspissated bile syndrome are cholesterol crystals and calcium salts.Inspissated bile syndrome is a common term that is applied to an abnormality of bile that is seen with ultrasonography of the gallbladder. Specifically, the bile within the gallbladder is seen to be of two different densities with the denser bile on the bottom. The bile is denser because it contains microscopic particles, usually cholesterol or pigment, embedded in mucus. (The mucus is secreted by the gallbladder). Over time, sludge may remain in the gallbladder, it may disappear and not return, or it may come and go. As discussed previously, these particles may be precursors of gallstones, and they occur often in some situations in which gallstones frequently appear, for example, rapid weight loss, pregnancy, and prolonged fasting.Nevertheless, it appears that sludge goes on to become gallstones in only a minority of individuals. Just to make matters more difficult, it is not clear how often - if at all - sludge alone causes problems. Sludge has been blamed for many of the same symptoms as gallstones-biliary colic, cholecystitis, and pancreatitis, but often these symptoms and complications are caused by very small gallstones that are missed by ultrasonography. Moreover, it is possible that these gallstone-like symptoms and complications are actually caused by small gallstones that have passed through the bile ducts and into the intestine rather than the sludge itself. Thus, there is uncertainty about the significance of sludge.It is clear, however, that sludge is not the equivalent of gallstones. The practical implication of this uncertainty is that unless an individual's symptoms are typical of gallstones, sludge should not be considered as the cause of the symptoms.The other risk factors may include:Low birthweight: premature and small for dates.
Infants of mothers who have diabetes.
Populations living at high altitudes.
Prognosis is poor if the biliary system is not decompressed, with 100% mortality. Mortality even 50% with treatment.Symptoms:Inspissated bile syndrome usually causes no symptoms and may appear and disappear over time. Inspissated bile syndrome, however, may cause intermittent symptoms and, on occasion the particles may grow in size and become larger gallstones.The most common symptom of inspissated bile syndrome - when it causes symptoms - is pain in the abdomen often associated with nausea and vomiting. This occurs when the particles obstruct the ducts leading from the gallbladder to the intestine.Inspissated bile syndrome also may cause more serious complications, including inflammation of the pancreas (pancreatitis), and inflammation of the gallbladder (cholecystitis).
Inspissated bile syndrome diagnosis:
Diagnosis may include the following tests:Serum bilirubin: total, conjugated, unconjugated.
Liver function tests: hepatitis, cholestatic disease.
Reducing substance in urine: screening test for galactosaemia (provided the infant has received sufficient quantities of milk).
Thyroid function tests.
Infection screen (must be excluded in any baby that is unwell or presents in the first 24 hours or after day 3): toxoplasmosis, rubella, cytomegalovirus and herpes simplex (TORCH) congenital infection screen, surface swabs including umbilicus, throat swabs, urine culture, blood culture, lumbar puncture, chest X-ray.
Blood type and Rh (Rhesus) determination in mother and infant.
Direct Coombs' testing in the infant.
Hemoglobin and hematocrit values.
Peripheral blood film for erythrocyte morphology.
Red cell enzyme assays: G6PD deficiency, pyruvate kinase deficiency.
Ultrasound, hepatobiliary iminodiacetic acid (HIDA) radionuclide scan, liver biopsy and laparotomy may be required for cholestatic jaundice in the differentiation between hepatitis and biliary atresia.
Treatment:The treatment strategy for inspissated bile syndrome with laparoscopic cholecystostomy and biliary duct lavage is feasible and effective.Laparoscopic surgery procedure has a rapid postoperative recovery and fewer wound complications. The liver and gallbladder can be inspected by direct vision. The use of laparoscopic techniques and cholangiography is conducive to an early diagnosis in patients with obstructive jaundice. It is an alternative treatment that can be recommended for children with inspissated bile syndrome when conservative therapy is unsuccessful.Laparoscopic cholecystostomy and bile duct lavage can avoid surgical exploration. After cholecystostomy, repeated bile duct lavage can be taken according to bilirubin levels and liver function of the patients so as to relieve the jaundice more quickly.Levels of bilirubin can be controlled by placing the baby under a lamp emitting light in the blue spectrum, which is known as phototherapy. Light energy of the appropriate wavelength converts the bilirubin in the skin to a harmless form that can be excreted in the urine. Phototherapy has proved to be a safe and effective treatment for jaundice in newborn babies, reducing the need to perform an exchange transfusion of blood (the only other means of removing bilirubin from the body).NOTE: The above information is for processing purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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