A 17 Year Old Boy with Biliary Obstruction

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A 17- Year- Old Boy with Biliary Obstruction

• CC

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HPI- 17 months prior to admission to MGH

• Symptoms Bloody diarrhea ? admission to a
  hospital
• ? what exams to do?

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LAB

• Serum aspartate aminotransferase level 75 U/l
• Test for Clostridium difficile positive
• ? had not taken antibiotics before!
• ? management?

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MANAGEMENT

• One month course of metronidazole
• ? patient feels well

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Several weeks prior to admission to MGH

• Symptoms
• constant crampy, nonradiatingpain in the
  epigastrium, right upper quadrant, periumbilical
  area,
• pain exacerbates by eating, accompanied by nausea
• intermittent loose stools without frequent or
  voluminous diarrhea
• temp. rises intermittently to 38.3 C

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Five days prior to admission

• Symptoms
• leftsided pleuritic chest pain
• dry cough
• ?admission to a hospital
• ?exams?

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LAB

• Leucocytosis with a leftward shift

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CXR

• Suggesting presence of pneumonia
• of the right lower lobe

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Abdominal US

• No abnormalities

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Progress of the patient

• Various pain medications are
• ineffective.
• ? transfer to MGH
• ? what to do?

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PMH

• No informations

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Immunizations

• His immunizations are up to date
• and include viral hepatitis B
• vaccination.

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FH

• No family history of inflammatory
• bowel disease or rheumatic disorders

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SH

• 17- year- old student
• No history of alcohol or illicit drugs

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ROS

• Constitutional
• lost 3kg in weight during the preceding two
  weeks
• GIstools of normal color
• GUurine darker than usual

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Physical Examination

• VS
• Temp. 37.8 C
• Pulse 85
• BP 120/55 mm Hg
• Resp. 20

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Physical Examination

• Eyes mild scleral icterus
• Chest supsternal pain
• Lungs clear
• Abdomen
• soft with slight tenderness in the right upper
  quadrant
• no hepatomegaly
• ?admission testings

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LAB (1)
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LAB (2)
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LAB (3)
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Assessment

• The patient is a 17-year-old boy,who
• suffers from epigastrical pain and
• intermittently from diarrhea (even
• bloody in the past).
• Moreover there is evidence of biliary
• obstruction.

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CXR

• Bilateral prominence of the interstitial
• markings

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Adominal US

• Liver of normal texture
• Inrahepatic ducts and the common bile duct of
  normal diameter
• Partially collapsed gallbladder
• Normal pancreas

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Stool

• Stool specimen positive () for occult blood
• Microscopical examination
• excessive number of undigested muscle fibers and
  abundant yeasts
• no protozoa or helminthic ova
• No C. difficile toxin
• No enteric pathogens

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Urine

• Positive () for bile
• Minimally positive for urobilinogen
• Normal sediment

? management?
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Management

• Ranitidine, clarithromycin and
• acetaminophen are given

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Progress of the patient

• Temp. rises to 39.7 C

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2nd hospital day

• Temp. does not exceed 39C
• Abdominal pain ceases
• ?exams?

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Physical Examination

• Unchanged

? additional testings
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LAB
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Abdominal US

• No abnormalities

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CT

• CT of the abdomen and pelvis after
• oral and iv. administration of
• contrast material
• ? no abnormalities

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Intestinal disease-differential diagnosis

• Infectious disease
• Celiac sprue
• Inflammatory bowel disease

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Infectious disease

• The patients clinical course and the result
• of the limited testing that was performed
• make it very improbable that the illness
• has an infectious cause.

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Celiac sprue

• Unlikely diagnosis in this case because the
  illness generally developes in adults or in
  children younger than this patient.
• An acute onset of marked upper gastrointestinal
  symptoms is atypical of celiac disease.

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Inflammatory bowel disease

• The initial signs, symptoms and laboratory
  findings that suggest inflammatory bowel disease
  include diarrhea, fever, weight loss,
  leukocytosis, thrombocytosis and occult blood in
  the stool.
• Upper gastrointestinal involvement is more common
  in children with this disease than in adults.

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Liver disease-differential diagnosis

• Primary sclerosing cholangitis
• Autoimmune hepatitis

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Liver disease-differential diagnosis

• Primary sclerosing cholangitis can involve the
  extrahepatic ducts, the intrahepatic or both
• Autoimmune hepatitis characteristically involves
  the hepatic parenchyma
• gtboth are common in inflammatory
  bowel disease

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Exams

• Evaluation of autoimmune markers
• Liver biopsy
• Endoscopic retrograde cholangiopancreatography

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Autoimmune markers
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Liver biopsy
The expanded portal tract (arrows) contains a
duct surrounded by edema (arrowheads)
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Liver biopsy

• The pericuctal edema (arrow) results in an
  onionskin appearance.
• There is no inflammation at the interfaces of the
  portal tracts and
• hepatic lobules.

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Pathological discussion

• Preservation of the hepatic architecture
• Expansion of the portal tracts, which are rounded
  and edematous
• Within the portal tracts almost all the
  interlobular bile ducts are acutely inflamed
• No inflammation at the interfaces of the portal
  tracts and hepatic lobules
• A singel so-called bile infarct

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ERCP

• Specimen of the Gastric Fundus. There is a
  granulomatous
• reaction around a damaged gastric gland (arrows).

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ERCP

• Specimen of the Duodenum. The central duct is
  acutely inflamed
• and ruptured and is surrounded by acute and
  chronic
• inflammation.

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Pathological discussion

• No evidence of extrahepatic bile-duct obstruction
• Severe inflammation and an epithelioid granuloma
  in the gastric wall
• Patchy, superficial inflammation and deep acute
  and chronic inflammation

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Diagnosis

• Primary sclerosing cholangitis
• associated with Crohns disease.

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Treatment

• Treatment with prednisone and ursodiol.Later on
  p. is replaced with mesalamine.
• Patient gets introduced to the idea that he
  might be a candidate for liver transplantation
  (p.s.c.risk for bile-duct-cancer).

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Addendum

• 36 months later the aminotransferase levels are
  still slightly and the y-glutamyltransferase
  level is moderately elevated.
• A ERCP showes no change in the degree of
  narrowing of the intrahepatic ducts.

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