RUQ Pain and Impaired LFTs: Diagnoses to Consider

Q: A 56-Year-Old Female Presents with Recurrent RUQ Pain, Impaired LFTs, Elevated ALKP and Transaminases. She had a Prior ERCP and Possible Biliary Papillotomy for Similar Complaints but she does not Recall the Diagnosis. What Should I do?

Issue: December 2016

ByJoseph Leung, MD, FRCP, FACP, FASGE, MACG

A: The common complaint of right upper quadrant (RUQ) pain and impaired liver function tests (LFTs) could suggest biliary obstruction secondary to ductal stones and possible cholangitis if there is fever or chills and other constitutional symptoms. Less commonly, it can be related to attacks of pain associated with sphincter of Oddi dysfunction (SOD). However, late complications of postpapillotomy stenosis remain a possibility in the differential diagnosis.

The clinical diagnosis of SOD is based on the presence of biliary-type pain and evidence of bile duct obstruction, ie, dilated biliary system and/or impaired LFTs with elevated transaminases more than 3 times the normal level. Based on the Milwaukee (Geenen) classification, SOD can be classified as Type I, where patients present with typical biliary pain with imaging evidence of a dilated common bile duct (CBD) and abnormal LFTs; Type II, where pain is associated with either a dilated CBD or abnormal LFTs during attacks of pain; or Type III where there is pain only, without evidence of ductal dilation on imaging and no LFT abnormalities during attacks of pain. The clinical diagnosis of the classic Type I case is easy, whereas Type III patients may pose a challenge to the clinician. Sphincter of Oddi manometry (SOM) can be performed to document basal pressure in an otherwise normal or intact papilla. A basal pressure of > 40 mm Hg is considered abnormal and supports the diagnosis of sphincter dysfunction. This is especially important in patients with suspected Type III SOD for proper documentation before considering a papillotomy. The conventional treatment for established SOD is papillotomy, aiming at cutting the sphincter muscle to relieve biliary obstruction. The likely response will be good in Type I cases but questionable in Type III patients.

The clinical presentation of bile duct stones can be similar with pain and impaired LFTs if the stone impacts or obstructs the bile duct. Distal stone obstruction in the common channel can also cause acute biliary pancreatitis. The diagnosis of biliary stone(s) is more straightforward if there is imaging evidence of a dilated duct, presence of ductal stones and/or gallbladder stones with elevated alkaline phosphatase (ALKP), transaminases, and possibly bilirubin. The presence of cholangitis is more serious and warrants urgent attention and treatment. Imaging includes abdominal ultrasound or a computed tomography scan, which can help to define the presence of any ductal dilation or stones; although the presence of very small stones in the distal bile duct may not be readily detected, even with magnetic resonance cholangiopancreatography (MRCP). Endoscopic ultrasound (EUS) remains the most sensitive test for diagnosing distal bile duct stones. Endoscopic retrograde cholangiopancreatography (ERCP) is more invasive, but offers a therapeutic option if stones are detected in the bile duct. The positive predictive factors for the presence of bile duct stones include positive imaging results, a dilated bile duct with impaired LFTs, concurrent cholangitis, or acute biliary pancreatitis. A past history of biliary pancreatitis may not predict the presence of bile duct stones because the impacted stone can pass after a few days.

Figure 1. Cholangiogram showing distal CBD stone. Extrinsic compression on common hepatic duct is suggestive of Mirrizzi’s syndrome.

Figure 2. (A) Stenotic biliary orifice and evidence of a prior papillotomy (note periampullary diverticulum). (B) Stone extraction after extension of prior papillotomy (guidewire in place).

Figure 3. A different patient with postpapillotomy stenosis.

Figure 4. (A) Six-mm biliary dilation balloon inflated at the stenotic orifice. (B) Wide opened biliary orifice after balloon dilation.

However, the clinical situation may change if the patient has undergone prior biliary papillotomy. One thing to consider is whether the symptoms are related to recurrent bile duct stones, especially in patients who still retain their gallbladder (Figure 1). Ascending reflux with bacterial contamination can predispose to de novo bile duct stone formation, which can cause subsequent cholangitis. Foreign bodies, including surgical clips, similarly can be a nidus of stone formation. A less likely possibility is postpapillotomy stenosis secondary to scarring of the papillary orifice (Figure 2A). This may present with a similar problem of biliary obstruction with pain and impaired LFTs, with or without symptoms of infection and cholangitis.

The investigation will be similar to the other conditions, with conventional imaging of the bile ducts to rule out any obstructing factor and to document the size of the bile ducts. Serial LFTs can indicate whether there is evidence of persistent biliary obstruction. Ethyl hepatic iminodiacetic acid imaging (EHIDA) can demonstrate the patency of the biliary system and flow of the isotope into the duodenum. Early or urgent intervention is indicated if the patient has signs and symptoms of cholangitis or if there is evidence to suggest delayed emptying of the biliary system. The aim of intervention is to provide drainage, and ERCP is the first line of treatment. ERCP can demonstrate the status of the prior papillotomy, whether it is patent, and the presence or absence of stones in the biliary system. If stones are demonstrated, the papillotomy can be extended if it can be performed safely and if the stones can be removed with a basket or balloon (Figure 2B).

In the unusual situation of postpapillotomy stenosis, a number of treatment options are available. A lot depends on the size of the remaining papilla (if a small papillotomy was performed) or if the orifice is stenosed. Late postpapillotomy stenosis can occur in up to 10% of cases after a prior papillotomy, especially where the prior papillotomy was suboptimal. A tight pinhole opening may sometimes be seen to represent the biliary orifice (Figure 3). Extension of a prior papillotomy can be risky if there is not much remaining papilla. Further cutting will significantly increase the risk for perforation. Following a diagnostic cholangiogram to rule out ductal filling defects, a guidewire can be inserted in the (possible) dilated bile duct. A dilation balloon (size ranging from 4 to 8 mm) can be selected, inserted over the guidewire, and fully inflated for 3 to 5 minutes to perform dilation of the orifice (sphincteroplasty) to improve drainage from the biliary system (Figure 4). Bile should be taken for culture and prophylactic antibiotics should be given if there is clinical suspicion of cholangitis. Depending on the findings and whether there is any residual edema that may block the biliary orifice, a 7- or 8-cm 10-Fr stent can be inserted to endure biliary drainage and removed after 1 week when tissue edema settles. Clinical follow-up is necessary, as we have seen patients returning after a period of months to a year with recurrent symptoms secondary to restenosis following sphincteroplasty. We have found that the addition of steroid injection around the dilated orifice (Kenalog 40-mg solution injected in divided doses into 4 quadrants around the stent) helps to delay the onset of restenosis.

Excerpted from:

References:
Leung J. Fundamentals of ERCP. In: Cotton P, Leung LW, eds. Advanced Digestive Endoscopy: ERCP. Boston, MA: Blackwell Science; 2005.
Liao WC, et al. Gastrointest Endosc. 2010;72:1154-1162.

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