a condition in which there is aplasia or obliteration of some or all of the extrahepatic biliary tree: the gallbladder may or may not be involved. It presents in the neonate or young infant with clinical findings of obstructive jaundice and conjugated hyperbilirubinaemia. If undiagnosed or unrelieved progressive biliary cirrhosis will develop. The prognosis is inversely related to the age at which surgery is undertaken and the degree of liver damage. The aetiology is unknown but some consider it to be due to an inflammatory process which leads to progressive obliteration of the bile ducts in the perinatal period.
At ultrasound examination, which should be done following a 4-hour fast, before the onset of cirrhosis, the liver is normal. There is no intrahepatic bile duct dilatation. The gallbladder is not usually seen but if identified it may be spherical. The extrahepatic biliary tree is not seen. A normal ultrasound examination does not exclude the diagnosis. Hepatobiliary scintigraphy using a Tc99m-labelled iminodiacetic acid product (e.g. HIDA) typically demonstrates good hepatic uptake of tracer, with progressive accumulation within the liver but no evidence of excretion into the extrahepatic bile ducts or into bowel. Premedication with phenobarbitone for 5 days prior to the study is recommended to ensure maximal hepatic enzyme function should there be some impairment of liver function. The absence of bowel activity at 24 hours is generally taken to be an indication for liver biopsy and/or operative cholangiography. Findings in neonatal hepatitis include poor hepatocellular uptake of tracer, delayed excretion into bowel and occasionally non-excretion as in biliary atresia.
Treatment is surgical and involves anastomosing an intrahepatic bile duct (usually in segment III) to a loop of jejunum to bypass the extrahepatic obstruction (Kasai procedure). Complications include sepsis and anastamotic stricture. The procedure should be performed as early as possible after diagnosis to avoid complications related to cirrhosis and portal hypertension.
- ► 2012 (229)
- ► 2011 (282)
- ► 2010 (171)
- ► 2009 (191)
- ► 2008 (194)
- ► 2007 (131)
- ► 2006 (221)
- ► 2005 (158)
- Sequestrum- A large rod-like sequestrum is observe...
- Chest radiograph demonstrates decreased pulmonary ...
- STRING OF BEADS
- Fibromuscular dysplasia condition of unknown ae...
- Pulmonary edema
- Lateral radiograph of a lumbar vertebral body demo...
- Paget's disease Lateral radiograph of the skull d...
- Barium swallow in a child showing typical appearan...
- sheperd's crook deformity femur-typical of fibrous...
- air bronchogram-- a classical sign described by fl...
- small densely calcified nonfunctioning right kid...
- IMAGING IN BILIARY ATRESIA
- the classic waterlily sign of hydatid!!!!!COPYRIGH...
- History of X-rays
- indian conference calender
- hydatid cyst lung.. a classical CXR appearance alt...
- MITRAL STENOSIS-RADIOLOGICAL FINDINGS
- on line book purchasing
- cranial USG showing bilateral subdural effusion.. ...
- choledocolithiasis-the classical meniscus sign at ...
- MR imaging bladder
- normal barium meal follow through jejunum has valv...
- "pneumatocele" a characterstic of staphylococcal p...
- visit my message board
- "widened intercondylar notch knee" classical featu...
- Nonvisualization of Appendix on CT Linked With Low...
- OSTEOSARCOMA-"SUNRAY APPEARANCE"
- HEMOLYTIC ANEMIA
- posterior urethral valve
- Short Service Commission Notification with the Arm...
- important facts radiotherapy
- MR SAFETY AND CEREBROSPINAL FLUID SHUNT VALVES
- COPD X-RAY changes
- Alobar Holoprosencephaly Holoprosencephaly is a c...
- Renal Transit Time with MR Urography in Children
- Müllerian Duct Anomalies: Imaging and Clinical Iss...
- interesting syndrome
- RSNA 2004
- WHITAKER TEST
- official links for indian medicos
- Radiology quiz
- Radiology of pulmonary infections
- Reviews to My Book "Review of Radiology"
- quote of the day
- RADIOTHERAPY TUTORIAL
- SGPGI FORMS OUT!!!
- Immediate Computed Tomography Scanning of Acute St...
- radiologists, say good bye to cardiac CT!!!!
- PRIMARY PULMONARY TUBERCULOSIS
- PGI forms out
- DOUBLE BUBBLE SIGN
- quote of the day
- orbital cysticercosis
- PUTTY KIDNEY
- ultrasound simulators
- ▼ October (57)