A previously healthy 47-year-old man presented to our institution
with a 6-month history of recurrent episodes of jaundice and pruritus. Over
this period a number of endoscopic retrograde cholangiographies (ERCs) had been
undertaken, demonstrating mucin at the ampullary orifice and a dilated biliary
system packed with mucin plugs ([Fig. 1]).
Fig. 1 Cholangiographic image
demonstrating a dilated bile duct containing amorphous filling defects
consistent with mucus plugs of biliary papillomatosis.
Repeated biliary stenting had been performed for symptomatic relief.
On referral to our institution, the patient was jaundiced with a bilirubin of
426 µmol/L (normal range
5.1 – 17.0 µmol/L).
A mother-daughter video cholangioscopy with narrow-band imaging
(NBI) capability (CHF-Y0002, Olympus, Melville, New York, USA) was carried out
on the suspicion of presence of a mucin-secreting tumor within the biliary
tree. A nasobiliary drain was placed for 48 hours to facilitate irrigation of
the biliary tree. At cholangioscopy, multiple lesions, characterized by a fish
egg-like appearance, were identified at the bifurcation of the intrahepatic
ducts and in the distal bile duct just proximal to the ampulla, consistent with
multicentric papillomatosis ([Fig. 2],
[Video 1]).
Fig. 2 Cholangioscopic image
demonstrating the characteristic fish egg-like appearance of biliary
papillomatosis using: a white light and
b narrow-band imaging (NBI).
Video
1 Biliary papillomatosis
diagnosed with mother-daughter narrow-band imaging (NBI) cholangioscopy. The
cholangioscope is positioned in the common bile duct. Normal biliary epithelium
is seen first with white light and subsequently with NBI. Mucin and the fish
egg appearance of the papillary biliary tumor is demonstrated with both white
light and NBI.
The patient was subsequently referred for consideration for liver
transplantation and Whipple resection. However, in the course of this workup,
he was deemed unsuitable for surgical resection because of the spread of tumor
to surrounding lymph nodes. The patient has since been managed endoscopically
with bilateral hilar stenting without complication.
Biliary papillomatosis, first reported by Chappet in 1894, is a rare
disease characterized by multiple papillary adenomas in the biliary tree
[1]. It should be considered a premalignant condition
with a high malignant potential [2]. The disease is
characterized by relapsing episodes of acute cholangitis and obstructive
jaundice resulting from intermittent biliary obstruction from secreted mucin
and the tumor itself, and secondary cirrhosis is often present by the time of
presentation [3]. ERC has a key role in the diagnosis of
biliary papillomatosis. Diffuse bile duct dilatation associated with amorphous
filling defects are characteristic findings at cholangiography. However, a
large amount of mucin secretion and obstruction by the tumor prevent complete
opacification of the entire biliary tract. As a result, precise evaluation of
ductal extent by ERC is often suboptimal. Diagnosis is usually confirmed by
tissue biopsy either through peroral or percutaneous transhepatic
cholangioscopy [2].
Peroral cholangioscopy facilitates noninvasive visualization of the
biliary system. While the conventional method involved the use of fiberoptic
mother-daughter cholangioscopy, its use is becoming less common with the advent
of the Spyglass system. However, our case, in addition to the report by Itoi et
al., demonstrates that NBI video cholangioscopy provides superior images in
terms of quality and size in contrast with both Spyglass and fiberoptic
cholangioscopy [4]
[5]. Video
cholangioscopy in our patient detailed clearly the multicentric extent of the
disease, thereby having the potential to guide the appropriate surgical
procedure. In addition, mucosal imaging techniques such as NBI may facilitate
improved characterization and targeted biopsies of abnormal lesions in the
biliary tract similar to their emerging role in the gastrointestinal tract
[6].
Endoscopy_UCTN_Code_CCL_1AZ_2AN
