What Caused This Man’s Pruritis?

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64 year-old-male with a history of MI, CAD, HTN, DM, alcohol and tobacco use disorder presented to the ED for jaundice, itching and abnormal LFTs. The patient stated that he had been experiencing itching for the last three to four weeks along with jaundice and icterus. It was worse when he showered and was associated with light stools and very dark urine. He had tried Benadryl for the itching, but this only helped temporarily. He had one episode of abdominal pain with this that was sharp, severe, located in the upper abdomen and only lasted about an hour before completely abating. He denied any fevers or chills, altered mental status, weight changes, previous episodes of itching or jaundice and  any new drug use, prescribed or non-prescribed.

The Exam
On physical examination, the patient was in no distress. He was afebrile, mildly hypertensive and grossly jaundiced with obvious icterus. His mucous membranes were moist, and he had sublingual jaundice. He had a normal respiratory rate, his breath sounds were clear bilaterally and he had no increased work of breathing. He had a regular heart rate without any murmurs. His abdomen was obese and without scars. There was no abdominal tenderness nor ascites. On neurological examination, he was alert and oriented, his cranial nerves were intact and his strength and sensation were equal bilaterally. He did not have asterixis. His cervical and axillary lymph nodes were non-enlarged nor tender.

The Labs
Laboratory evaluation demonstrated a mild leukocytosis of 13.0, an alkaline phosphatase of 1000,  ALT  300 and AST 150. His direct bilirubin was 17, total bilirubin was 30 and ammonia was 55. Lipase and amylase were normal. His INR was normal.

Differential Diagnosis:

  1. Klatskin Tumor
  2. Pancreatic Cancer
  3. Cholecystitis
  4. Hepatitis
  5. Biliary Stricture

The Images
A right upper quadrant ultrasound revealed hepatomegaly (16 cm) with echogenicity, intrahepatic and biliary dilatation (image 1 below). The common bile duct was 5 mm (image 2 below). There were no masses, and the gallbladder was contracted with sludge. No Murphy’s sign was elicited.

An abdominal and pelvic CT showed marked diffuse intrahepatic biliary dilation. An oval hyperdense structure was seen approximately 1.2 by 1.1 cm within the cystic duct (image 3 below) and there was mild wall thickening of the distal common bile duct. 

What is your diagnosis?

 

Discussion
This patient had Mirizzi’s syndrome, an obstruction of the common bile duct from a gallstone within the cystic duct [1]. This is a rare syndrome found in less than 0.1% of patients with gallstones [2]. One of the ongoing theories regarding pathogenesis of this illness is that one or multiple gallstones in the cystic duct or gallbladder infundibulum causes inflammation in the common bile duct and subsequent obstruction [3]. It is also theorized that cystic ducts running parallel and in close proximity to the common bile duct increase the risk of Mirizzi’s syndrome. While treatment may differ from case to case, typical treatment involves performance of ERCP with decompression and with stent placement to relieve the obstruction [2]. This is typically followed by removal of the gallbladder for definitive treatment.

This patient was initially believed to have cholangiocarcinoma of the perihilar region causing obstructive jaundice. Cholangiocarcinoma is a rare cancer with about 5000 to 6000 cases worldwide each year [4]. The incidence is increased in male gender, Asian ethnicity, patient’s with known hepatitis and chronic biliary inflammation.

Pancreatic carcinoma was the top diagnosis prior to the imaging results for this patient. Pancreatic cancer is the tenth most common cause of cancer in the United States with around 50,000 cases per year, with the mortality rate approaching 100% [5]. The risk factors for pancreatic cancer remain ill-defined and it is estimated that only 10% can be attributed primarily to genetic susceptibility [6]. Chronic pancreatitis, tobacco use, diabetes and dietary factors are thought to contribute to likelihood of developing pancreatic cancer in combination with genetic factors.

Case Conclusion
In the emergency department, this man received IVF and was admitted to the hospital for his obstructive jaundice. Gastroenterology performed an ERCP and placed a stent in the CBD to restore flow and relieve the obstruction. His elevated bilirubin and liver function tests slowly trended downward. A percutaneous cholecystectomy was attempted, but dense adhesions were present and it had to be converted into a subtotal cholecystectomy. The gallstone was ultimately left inside as there was too great of a risk to injure vital nearby structures. Repeat RUQ ultrasound showed improved intrahepatic congestion, but CBD dilation was still present. ERCP was repeated and stent placed again as previous stent had migrated to the intestine. The patient was discharged from the hospital with follow up scheduled.  He followed up with his primary care physician since the repeat stent placement but did not follow up with his gastroenterologist or surgeon.


REFERENCES

  1. Johnson LW, Sehon JK, Lee WC, Zibari GB, McDonald JC. Mirizzi’s syndrome: experience from a multi-institutional review. Am Surg. 2001 Jan;67(1):11-4. PubMed PMID: 11206888.
  2. Hazzan D, Golijanin D, Reissman P, Adler SN, Shiloni E. Combined endoscopic and surgical management of Mirizzi syndrome.Surg Endosc. 1999 Jun;13(6):618-20. Review. PubMed PMID: 10347304.
  3. Pemberton M, Wells AD. The Mirizzi syndrome. Postgrad Med J. 1997 Aug;73(862):487-90. Review. PubMed PMID: 9307740; PubMed Central PMCID: PMC2431374.
  4. Brown KM, Geller DA. Proximal biliary tumors. Surg Clin North Am. 2014 Apr;94(2):311-23. doi: 10.1016/j.suc.2013.12.003. Epub 2014 Feb 4. Review. PubMed PMID: 24679423.
  5. Fernandez-del Castillo C, Jimenez R. Epidemiology and nonfamilial risk factors for exocrine pancreatic cancer. [revised 2017 March]. [Internet]. 2017 March. Available from: https://www.uptodate.com/contents/epidemiology-and-nonfamilial-risk-factors-for-exocrine-pancreatic-cancer?source=search_result&search=pancreatic%20cancer&selectedTitle=6~150.
  6. Camara SN, Yin T, Yang M, Li X, Gong Q, Zhou J, Zhao G, Yang ZY, Aroun T, Kuete M, Ramdany S, Camara AK, Diallo AT, Feng Z, Ning X, Xiong JX, Tao J, Qin Q, Zhou W, Cui J, Huang M, Guo Y, Gou SM, Wang B, Liu T, Olivier OE, Conde T, Cisse M, Magassouba AS, Ballah S, Keita NL, Souare IS, Toure A, Traore S, Balde AK, Keita N, Camara ND, Emmanuel D, Wu HS, Wang CY. High risk factors of pancreatic carcinoma. J Huazhong Univ Sci Technolog Med Sci. 2016 Jun;36(3):295-304. doi: 10.1007/s11596-016-1583-x. Epub 2016 Jul 5. Review. PubMed PMID: 27376795.

ABOUT THE AUTHORS

Dr. Effron is the assistant professor of emergency medicine at Case Western Reserve University and attending physician in the department of emergency medicine at the MetroHealth Medical Center, in Cleveland, Ohio.

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