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| Case of the Week Tuesday 20/12/2005 |
Presented by Dr. Mahmoud Magdy.
Staff Round Persentation
Click here to download a power point presentation for the case. CLINICAL CASES
CHOLESTASIS
CASE I
• 24 yrs old male patient presented with jaundice, pruritus ,dark colored urine, epigastric pain ,vomiting and weight loss of one month duration.
• There was a history of occasional alcohol intake few months earlier .
• On examination the patient was jaundiced with scratch marks.
• No hepatomegaly ,splenomegaly or ascites.
• Urine analysis :+++bile pigments
• CBC: normal
• Liver functions:
• - Total Bilirubin: 9.5mg/dL
• - Conjugated: 4.9mg/dL
• - ALT:90 U/L
• - AST:109 U/L
• - ALP:301 U/L
• - GGT: 72 U/L
• - PC :100%
• FBS:98mg/dL
ABDOMINAL U.S.
• Liver is average in size, homogenous echo pattern with considerable dilatation of IHBRs and dilatation of the proximal part of the CBD=8mm yet no definite stones seen inside. No HFL. PV is not dilated.
• No other abnormal findings were noticed.
ABDOMINAL C.T.
• Moderate dilatation of the IHBRs as well as CBD down to the porta hepatis with normal caliber down to the duodenum ,no definite masses or stones.
• Pancreas and para-aortic area are free with no focal lesions or lymphadenopathy.
Differential Diagnosis
• Luminal lesions: e.g. stones ,worms.
• Wall lesions:
• - Cholangicarcinoma
• - Benign bile duct stricture (e.g. PSC, benign tumors of the biliary tree).
• Extra-luminal lesions:
• - carcinoma of the pancreas
• - chronic pancreatitis
• - cystic lesions of the pancreas
• - enlarged lymph node.
ERCP
• Revealed a stricture in the CHD ,marked IHBR dilatation and a 19Fr - 12cm stent was inserted with good drainage.
• On the following day, the pt. developed fever, rigors, Rt. hypochondrial pain , rising bilirubin (up to 21.6) and leucocytosis (most probably cholangitis).
• He received antibiotics and the condition started to improve gradually within few days.
Serum bilirubin was falling gradually but very slowly.
• A follow up abdominal U.S. showed:
• . No evidence of IHBRs dilatation.
• . CBD is not dilated( 4mm at the level of hepatic hilum).
• . A biliary stent is seen inside it reaching proximally into its intra-hepatic portion.
Hepatitis Viral Markers
• HBsAg : Negative
• HB core total and IgM were Negative.
• HAV IgM : Negative.
• HCV Ab. : Negative.
• CA 19-9: 354.2ng/ml (37.0ng/ml)
• CEA : 6.5ng/ml. (5.0ng/ml)
• Anti fascoila antibodies 1/80.
• MRCP revealed
• - Pancreatic cystic lesion related to the pancreatic tail at the region of lesser sac.
• - No biliary tree obstruction : Normal caliber of the CBD and IHBRs.
• Surgical intervention was performed revealing
-A cystic lesion(3x2x2cm) at the area of the pancreatic body.
-The lesion was excised with part of the body and neck of the pancreas with resection of the lesser sac of the stomach related to the cyst.
-Roux en Y pancreatic jujonostomy was done for drainage.
Histo-pathological examination
• Aspirated fluid from the cyst showed inflammatory cells.
• Sections examined from the cyst wall revealed a pyloric wall showing a pseudo-cystic lesion lined by septic granulation with evidence of marked fibrosis and hemorrhage. The cyst is adherent to the pancreatic tissue with evidence of pancreatitis.
• - No evidence of specific inflammation or malignancy.
• Conclusion: pancreatic pseudocyst adherent to the pyloric wall and pancreas.
PANCREATIC PSEUDO-CYST
• Definition: a localized fluid collection that is rich in amylase and other pancreatic enzymes, that has a nonepithelialized wall consisting of fibrous and granulation tissue, and that usually appears several weeks after the onset of pancreatitis.
• Number : Pancreatic pseudocysts can be single or multiple.
• Size: varies from 2-30 cm.
• Site: About 1/3rd of pseudocysts manifest in the head of the gland, and 2/3rd appear in the tail.
• Etiology: 75-85% of cases are caused by alcohol or gallstone disease - related pancreatitis.
• Sex: The male predominance mirrors the male predominance in the incidence of pancreatitis.
• Age: Pseudocysts may occur after pancreatitis in any age group.
Clinical picture
• History:
• - consider the possibility of a pseudocyst in a patient who has persistent abdominal pain, anorexia, or abdominal mass after a case of pancreatitis.
• - Rarely, patients present with jaundice or sepsis from an infected pseudocyst.
• Physical:
• - Patients very frequently have a tender abdomen.
• - Patients occasionally have a palpable mass in the abdomen.
• - Peritoneal signs suggest rupture of the cyst or infection.
• - Other possible findings include: Fever ,jaundice & pleural effusion.
Lab studies
• Serum tests have limited utility.
• - Amylase and lipase levels are often elevated but may be within reference ranges.
• - Bilirubin and LFT findings may be elevated if the biliary tree is involved.
• Analysis of the cyst fluid may help differentiate pseudocysts from tumors.
• - CEA and CA-125 tumour marker levels are low in pseudocysts.
• - Amylase levels are usually high in pseudocysts and low in tumors.
• - Cytology is occasionally helpful in diagnosing tumors, but a negative result does not exclude tumors.
Imaging studies
• Abdominal ultrasound: ultrasound is not the study of choice for diagnosis.
• Abdominal CT scan
• - CT scan is the imaging criterion standard for pancreatic pseudocysts. It has a sensitivity of 90-100% and is not operator dependent.
• ERCP
• - It is not necessary in diagnosing pseudocysts; however, it is useful in planning drainage strategy.
Imaging studies
• MRI
• - MRI is not necessary for the diagnosis of pseudocysts; however, it is useful in detecting a solid component to the cyst and in differentiating between organized necrosis and a pseudocyst.
• Endoscopic ultrasound
• - Endoscopic ultrasound (EUS) is not necessary for diagnosis but is very important in planning therapy, particularly if endoscopic drainage is contemplated.
Medical Care
• The goal of therapy is avoidance of complications.
• - About 10% of pseudocysts become infected.
• - Pseudocysts can also rupture.
• u A controlled rupture into an enteric organ can sometimes cause GI bleeding.
• u A free rupture into the peritoneal cavity produces abdominal pain and, rarely, peritonitis or even death.
• Most pseudocysts resolve without interference and only require supportive care.
• Indications for drainage include the following:
• - Complications
• - Symptoms
Concern about possible malignancy
CASE II
• A 46years old male patient presented to us with jaundice ,pruritus and pale stools of one month duration following an attack of abdominal pain , vomiting .He lost 12kg within the period of illness .
• Examination revealed deep jaundice ,scratch marks but no hepato-splenomegaly ,ascites or other significant clinical finding.
• Urine analysis :+++bile pigments
• CBC was normal
• ESR was 50 in the 1st hour.
• Bilirubin: Total : 4.6 mg/dL
• u Direct: 3.4 mg/dL
• ALT:118 U/L
• AST:30 U/L
• ALP:342 U/L (up to 104)
• GGT: 22U/L
• PC :100%
• FBS:94mg/dL
• Serum bil. has raised up to 63 during hospital stay.
• Abdominal U.S. :
• . Liver: Average sized with bright echo pattern. No IHBR dilatation or HFL. Main portal vein is not dilated. CBD is not dilated.
• . No other abnormal findings in the U.S.
DIFFERENTIAL DIAGNOSIS
• Causes of intra-hepatic cholestasis:
• - Cholestatic viral hepatitis
• - Drug induced cholestasis
• - PBC
• - PSC
• - Septicemia
• - Rare causes: Benign recurrent cholestasis, Hodgkin ' s disease and amyloidosis.
Hepatitis Viral Markers
• HBsAg : Negative
• HB core total and IgM were Negative.
• HAV IgM : Negative.
• HCV Ab. : Negative.
• EBV IgG : Positive.
• EBV IgM : Negative.
• CMV IgG :75.8 (up to 15)
AUTOANTIBODIES
• ANA
• SMA
• AMA
• LKM
• ANCA
• Protein electrophoresis showed mild hypo-proteinemia and hypo-albuminemia.
• Serum amylase was 54 (90)
• Abdominal C.T was normal.
• Ultra-sound guided Liver biopsy was done and histopathological examination of the specimen revealed:
• - canalicular / hepatocellular cholestasis ,predominant acinar zone 3 involvement with minimal reactive inflammation &unremarkable portal pathology.
• On reviewing drug history there was occasional intake of NSAIDS for headache.
DRUG INDUCED HEPATO-TOXICITY
• Common causes of drug induced hepatic reactions are antibiotics, NSAIDs, cardiovascular drugs and CNS modifiers.
DRUG INDUCED HEPATO-TOXICITY
Drug toxicity mechanisms:
• Intrinsic or predictable drug reactions: The injury can be due to the drug itself or to a metabolite. Acetaminophen and carbon tetrachloride are classic examples.
• Idiosyncratic drug reactions:
• Hypersensitivity : Phenytoin is a classic. The response is characterized by fever, rash, and eosinophilia and is an immune-related response
• Metabolic-idiosyncratic: This type of reaction occurs through an indirect metabolite of the offending drug. The response rate is variable and can occur within a week or up to one year later. It occurs in a minority of patients taking the drug, and no clinical manifestations of hypersensitivity are noted. INH toxicity is considered to fall into this class .
CASE III
• 57 years old male presented to us with jaundice of 4 months duration, dark colored urine,pruritus, pale colored stools, bleeding tendency in the form of bleeding gums, but no fever or abdominal pain.
• The patient reported bilateral lower limb swelling 1 month prior to the onset of jaundice and recurrent attacks of DCL for 2 years which resolve with enemas, lactulose, hepamerz.
• Examination revealed :-
• General examination:
• - Pulse:88/min regular BP:110/70
• - Disturbed conscious level
• - Jaundice
• - Flapping tremors.
• - Bilateral soft pitting lower limb edema till knees.
• - Generalized scratch marks.
Abdominal examination
• - Shrunken liver
• - Splenomegaly: 5cm from the costal margin, firm, smooth surface, rounded border, not tender.
• - NO ascites
• Urine analysis : bile pigments+++
• HB : 11 TLC: 5,700 PLAT :94000
• Liver biochemical profile :
• - Bil T:25.9 Bil D:14.12
• - AST:85 ALT:29
• - ALP:125 GGT:67(11-49)
• - T.proteins:6.3 Albumin:2.1
• - PC: 55%
• Normal kidney functions .
• Abdominal Ultrasound:detailsssss
• - Liver cirrhosis with IHBR dilatation centrally more in right lobe.
• - Chronic calcular cholecystitis.
• - Splenomegaly
• - Mild pelvic ascites.
• Upper endoscopy:
- portal hypertensive gastropathy.
-superficial duodenal ulcer on the posterior wall.
ERCP
• Selective cannulation was done with some difficulty due to resistance in the lower most part of the CBD.Contrast injection revealed :
• - markedly dilated CBD with a short tight stricture segment at its lower most part.
• - Dilatation of the stricture was done using a 10 Fr dilator.
• - A 10 cm 10 Fr stent was placed with good bile flow seen coming from the stent.
• - Internal biliary drainage is established.
• Tumor markers
• - CEA:5.6( )
• - CA19-9 :172( )
• - (40xCEA +CA19-9) =396
• - Alfa feto protein:3.1( )
Follow up
• Bilirubin(T) :8.00
• Bilirubin(D):5.00
• Abdominal ultrasound:
-No dilated IHBR
-Good function of the stent.
Gall Stones in Liver Cirrhosis
• An increased prevalence of gallstones was demonstrated in patients with liver cirhosis, higher in the advanced stages of the disease. Some studies have found impaired emptying of the gallbladder in cirrhotic patient
• Cirrhotic patients showed a higher prevalence of gallstones than healthy subjects (41% vs 15%, P = 0.003), and the prevalence increased with the progression of liver cirrhosis (Child-Pugh class A: 26%, B: 44%, and C: 65%, P = 0.02).
• Gallbladder contractility is impaired in patients with liver cirrhosis and gallstones. Hypomotility is proportional to the severity of liver disease. Gallbladder hypomotility might contribute to the increased gallstone formation in patients with advanced cirrhosis.
THANK YOU
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