Please enable JavaScript.
Coggle requires JavaScript to display documents.
Biliary atresia (Complications (Chronic liver failure, Death, Liver…
Biliary atresia
-
Pathophysiology
Unclear cause, results in fibrosis and obliteration of the biliary tree
-
Failure to treat <60d of life leads to irreversible CLF and cirrhosis, hepatomegaly and splenomegaly
due to portal HTN, will need liver transplant
-
-
Diagnosis
Examination
Abdo: yellow skin/sclera, hepatomegaly, splenomegaly
Investigations
Bedside: obs (nil)
Bloods: FBC, CRP/ESR, U+E, LFT (high bilirubin, ALP/GGT)
Imaging: abdo USS (abnormal gallbladder); cholangiogram/ECRP + biopsy (atretic abnormal tree)
History
Jaundice in first few days of life,
pale stools, dark urine, weight loss
Management
Conservative
Information, advice, support
Surgical
Kasai procedure
Indication: all babies ASAP
MOA: hepatoportoenterostomy, where an intestinal limb of jejunum (Roux-en-Y) attached to drain bile
SEs: bleeding, infection; unsuccessful if
late presentation (>100d)
-
-
Prognosis
-
Even if prompt treatment, progression
to chronic liver disease in most children
-
-