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A 29-year-old lady
was admitted to the hospital because of right upper quadrant
pain, fever and jaundice for three days.
Brief History
This
patient, a foreign labor from Indonesia, came to Taiwan in
last June. She had been well until last early July when she
experienced right upper quadrant pain, fever and jaundice for
3 days. She visited local Hospital and then was transferred to
our emergent department on July 8 with the impression of acute
cholangitis with Klebsiella pneumoniae bacteremia. After
administration of two doses of parental cefoxitin(MefoxinTM), she was
transferred to another hospital due to unavailable bed at our
hospital.
She visited our emergent
department again on July 15th with a 2- week history of pain
in the right upper quadrant along with fever and chills. An
abdominal ultrasonography (figure 1) showed gall bladder
polyp, dilated right intrahepatic duct, marked
hepatosplenomegaly and enlarged, lobulated-shaped kidneys with
heterogeneous echogenecity. An abdominal CT scan (figure 2)
revealed marked hepatosplenomegaly, suspicious liver abscess
over segment 6 & 7, streaky enhancement of bilateral renal
parenchyma and minimal ascites. Her cholangitits improved with
cefoxitin. Mild disseminated intravascular coagulation process
was detected by laboratory study but recovered soon after
control of infection. Serial examinations for evaluation of
the hepatosplenomegaly including hematologic, rheumatologic
and virologic studies did not yield definite diagnosis. She
was discharged on July 24thafter cholangitis under control. No
further study was performed due to fear of losing her job in
Taiwan.
She presented to our emergent
department again on Aug 5th with fever, skin rash and jaundice
for 3-4 days. Antibiotics with cefoxitin was given again based
on previous result of blood culture. Abdominal ultrasonography
on Aug 8th demonstrated similar result as previous abdominal echo. Fever and leukocytosis
subsided after administration of cefoxitin for 3
days at ER. She was then admitted for further evaluation of the
hepatosplenomegaly.
On admission, her body temperature was 36°C, the pulse rate
was 72/min and the respiration rate 24/min. The
blood pressure was 130/70 mmHg. On physical examination,
the patient was relatively well nourished but appeared acutely ill.
Pale conjunctiva and icteric sclera were noted. The breath
sound was clear without crackles or wheezing. The heart
sounds were regular without murmur. The liver was
1.5 finger- breadth's palpable below xyphoid process and the splenic
tip was palpated at 2 finger- breadth's
below left subcostal margin. The abdomen was soft without shifting dullness or
tenderness.
Course and treatment:
Initially,
infiltrative disease such as disseminated tuberculosis with
liver, spleen and kidney involvement was suspected yet the PPD
test was negative. However, another spiking fever accompanied
with vomiting with greenish content developed on Aug 15th (Day
10 of cefoxitin). No abdominal pain was complained. The blood
test showed leukocytosis with a left shift. Since her general
condition was fine, all antimicrobial agents were held.
Klebsiella pneumoniae sepsis with disseminated intravascular
coagulation and elevated bilirubin, ALP and gamma-GT were
noted on Aug 19th. Antibiotics with ceftriaxone (RocephinTM)
and metronidazole were used under the impression of suspected
Klebsiella pneumoniae liver abscess or partial treatment of
Klebsiella pneumoniae cholangitis or ameobiasis. Fever
subsided on the following day and then metronidazole was
discontinued. An endoscopic retrograde
cholangio-pancreatographic study (ERCP) (figure 3) was
performed on Aug 22nd with the suspicion of possible
structural anomaly of biliary system because of repeated
biliary tree infection and it disclosed dilated right
intrahepatic ducts. Liver biopsy was performed on Aug 25th and
congenital hepatic fibrosis (figure 4) was diagnosed. No fever
occurred and antibiotic was then shifted to oral ciprofloxacin
since Aug 26th. Upper gastrointestinal panendoscopy was also
performed on Aug 31st to rule out portal hypertension with
resultant esophageal varices but only superficial gastritis
was found. She was then discharged on Sep 1, 2000 under stable
condition.
 案例分析
本病例產生右上腹痛、發燒及黃疸, 即典型膽管炎的Charcot's triads 症狀。
因病人反覆發生膽管炎, 而進一步的腹部超音波發現局部膽道擴張但並無明顯膽結石或腫瘤,
因此懷疑是否為膽道先天性異常。 一系列影像學檢查包括Abdominal CT, ERCP 和
MRCP診斷為Caroli’s disease/syndrome。(Caroli's disease/syndrome: a
characteristic biliary disease resulting in cholangitis, lithiasis and
liver abscess...., and often associated with congenital
hepatic fibrosis, polycystic liver disease or renal cystic disease.)
。 因 Caroli's disease 可能合併 congenital hepatic
fibrosis 加上臨床上無法解釋的hepatosplenomegaly
此病人接受了肝切片。病理報告證實病人合併有congenital hepatic fibrosis. 此外從一系列影像學檢查推測腎臟變化為medullary sponge
kidney. 只可惜未能有病理佐證。 Caroli's
disease以膽管炎及相關的併發症為主要表現, 根據擴張膽管的型式及有無合併congenital hepatic fibrosis可分為：(1) Bilobar
(Diffuse type) 及Unilobar type (2)Simple type (Classic
Caroli's disease) 及 Periportal fibrosis type (Caroli's syndrome)。
本病例診斷為Caroli's syndrome, unilobar type, associated with
congenital hepatic fibrosis and medullary sponge kidney
。診斷可藉由1. Abdominal sonography 2. Abdominal CT 3. ERCP 4. MRCP
等顯示極具特徵性的膽管變化。 因常併發膽管炎, 而且因合併congenital hepatic fibrosis
導致肝硬化及厲害門脈高壓, 故長期預後欠佳。
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Case Discussion