Missing something?

A middle aged patient presents with obstructive jaundice.   There is a history of upper abdominal pain for 2 months and jaundice for about 4 weeks.  She has also lost some weight loss and had night sweats.  On examination she is apyrexial, obviously jaundiced and slightly tender in the epigastrium 

Hb 98 
MCV 71                  
WCC 4.17 
Plat 338 

CRP <5 (<10)                                
Amylase 102 (<110)

Bili 75 µmol/L
ALT 910 iu/L
ALP 302 iu/L
Albumin 37 g/L
INR 1.0

An abdominal ultrasound is carried out (images below) 

If you work outside of the UK, and actually do abdominal ultrasound examinations you will be able to see that the gallbladder is extremely thick walled and hyperaemic.  There is also intrahepatic biliary duct dilatation but the CBD is of normal calibre (6mm) and no stones can be seen.  I haven't included the images showing that the pancreas,  pancreatic duct, liver, abdominal aorta, spleen and kidneys were all normal.

WHAT IS YOUR CLINICAL DIAGNOSIS?

■ Biliary colic

There is no pain!

■ Acute cholecystitis

How then do you explain the jaundice?

■ Choledocholithiasis

Would explain the jaundice and a CBD stone can be missed on US

■ Ascending Cholangitis

But there is no fever, raised WCC or CRP !!!

■ Acalculous cholecystitis

Sure no stones seen in the gallbladder but why the jaundice?

explanation of the case  (so far - there is more to follow!!! )

Choledocholithiasis should be your clinical diagnosis at this point.  This is because the ‘strong likelihood criteria’ are fulfilled as follows; 

1. Although no stone has been seen on imaging, the common bile duct is dilated and in addition,
2. The bilirubin level is elevated above > 4mg/dl (70 µmol/L). 

However, in this case there is no clinical evidence of an ascending cholangitis (fever, jaundice, ↑bilirubin, ↑WCC and a dilated CBD on US).  For this reason the likelihood of choledocholithiasis is strong rather than very strong (see table below)

To search further for gallstones, a CT scan was then requested (below).  

You can probably tell from the CT that The gallbladder is confirmed as thickened with some extrinsic compression of the bile duct at the porta hepatis to explain the intrahepatic ductal dilatation already seen on ultrasound. The distal bile duct is confirmed as collapsed with normal appearance of the pancreas, kidneys, adrenal glands and spleen. 

WHAT IS NOW THE DIAGNOSIS? 

■ Mirizzi’s syndrome

Absolutely

■ Choledocholithiasis

Nope, no stones seen within the CBD

second explanatation (and yes there are further developments around the corner)

The CT report essentially describes a Mirizzi syndrome type 1.  Pablo Mirizzi was an Argentinian Surgeon who first described the obstruction of the common hepatic duct (CHD) by an impacted stone in the cystic duct or Hartmann's pouch of the gallbladder. In Mirizzi syndrome type I there is no fistula between the gallbladder and CHD whilst type II-IV have a fistulous communication. Of course its difficult to tell on imaging if there  is a fistula. For this reason the subtype of the Mirizzi syndrome is usually something discovered at surgery.

The patient does undergo a laparoscopic cholecystectomy but to the surgeons surprise there are no stones found within the thickened gallbladder or within the bile ducts.  The gallbladder is analysed and the pathologists report that the:  “Gallbladder measures 52 x 30 x 20 mm.  The serosa is congested and wall thickness is 3 mm.  No stone and no focal lesions. There is subacute cholecystitis with myofibroblastic proliferation of the wall and mild acute on chronic inflammation”

Strangely enough, the jaundice does not resolve after surgery and for this reason an MRI scan is carried out which confirms a stricture at the common hepatic duct with mild intrahepatic ductal dilatation.   At this point we decide to carry out an ERCP to sample the stricture and place a stent.

Now you have all the pieces in this jigsaw and should know what's going on! 
WHAT DID WE MISS?

■ Primary cancer of the gallbladder

No cancer found in resection specimen!

■ Cholangiocarcinoma

You missed something on the video!

■ Gastric cancer

Well done, you spotted this on the video?

■ Duodenal cancer

But the duodenum look fine on video?!

FINAL EXPLANATION

Thanks for sticking with this case until the end!   Actually,  you did have an opportunity of getting the diagnosis when you heard that the gallbladder histology showed; "markedly thickened wall but with only mild inflammation of the gallbladder mucosa" ...

Of course, this doesn't make any sense!  In acute cholecystitis there should be an INTENSE inflammation of the mucosa.  So why is the gallbladder wall grossly thickened?  A second look into the deeper aspects of that thickened gallbladder wall led to a revelation !

The 'second look histology'  reported a 'poorly differentiated diffuse type adenocarcinoma deep within the gallbladder wall with single and files of small neoplastic epithelial cells (histology slides below). The pathologists reported that the tumour did appear to be coming from outside the gallbladder.  Of course in the video you should have noticed that the gastric antrum was abnormal, a little indurated and thickened.  The samples taken from the gastric antrum confirmed the same diffusely infiltrating adenocarcinoma.  Those with VERY sharp eyes, would have seen the antral thickening on the initial CT which wasn't commented upon by the radiologist!  

So what was the final diagnosis?  A diffuse type gastric cancer invading into the gallbladder, cystic duct and hepatic duct ! 

This may a good time to remind you of the management of gallstone related disease.  The 'infographic' below from J Int Care Med 2016;31(1):3-13  summarises everything !