Dr. Prasad Wagle
HPB Surgeon

Background:

Postcholecystectomy syndrome (PCS) commonly occurs due to extrabiliary causes such as peptic ulcer disease, reflux oesophagitis, irritable bowel syndrome, hepatitis and chronic pancreatitis. Biliary causes such as retained or recurrent biliary calculi with/out cholecystitis, biliary stricture, biliary dyskinesia and biliary leaks cause early or late PCS less frequently as compared with extrabiliary causes. Among all biliary causes of late PCS, postcholecystectomy Mirizzi syndrome (PCMS) is extremely rare.^1–3 It is often misdiagnosed and difficult to manage. Hence, all attempts should be made in the index surgery to prevent PCMS.

PCMS can occur due to cystic duct stump calculus or gall bladder remnant calculus. The role of laparoscopy in its management is controversial.^1–4 We present a case wherein, laparoscopic completion cholecystectomy (LCC) was performed for PCMS due to gall bladder remnant calculus, review the literature and summarise our algorithmic approach to the management of PCS.

Case presentation:

A 48-year-old woman presented with recurrent acute right upper abdominal pain, nausea and vomiting episodes since a month. She had undergone emergency open subtotal cholecystectomy (SC) for acute calculous cholecystitis 18 years ago, mitral valve replacement 7 months ago and was on oral anticoagulation.

Investigations:

On investigation, alkaline phosphatase was elevated. Other liver function tests (LFT) were within normal limits. Transabdominal ultrasound revealed a walled-off collection in gall bladder fossa with a non-obstructing common bile duct (CBD) calculus. MR cholangiopancreatography (MRCP) revealed a gall bladder remnant with a calculus within, compressing the common hepatic duct (CHD) suggestive of the type 1 Mirizzi syndrome (MS) (figure 1).

The major risk factor for biliary PCS is SC, which occurs in up to 13.3% of cases of laparoscopic cholecystectomy (LC). SC is performed intentionally in cases with poor visualisation, severe inflammation or adhesions in Calot’s triangle, cholelithiasis in portal hypertension or cirrhosis, and MS, to avoid bile duct injury and excessive bleeding. It may also be performed unintentionally as a result of congenital duplication or an hourglass configuration due to adenomyomatosis, especially by inexperienced surgeons.^{4 5}

As seen in figure 3, early PCS occurs within 2 years of cholecystectomy. ¹ Among cases of late PCS, remnant stones in cystic duct stump account for 17%–25% cases.⁶ A cystic duct stump >1cm in length after cholecystectomy causing PCS is known as cystic duct stump syndrome.⁷ Palanivelu et al⁸ reported an incidence of remnant cystic duct calculus of 4.19% in LC as compared with 0.02% in open cholecystectomy.⁹

PCMS can occur due to stone within gall bladder remnant or cystic duct stump or due to surgical clip.^{9 10} It can be associated with an atrophic gall bladder remnant with thick/thin walls, impacted gallstones at the infundibulum or Hartmann’s pouch, an obstructed long or short cystic duct with or without a low insertion, partial/complete obstruction by external compression of CHD by a gallstone or surgical clip, and occasional presence of an anomalous communication of gall bladder remnant or cystic duct stump with CBD or small intestine.^{10 11} It can present with any of the biliary symptoms shown in figure 3. Across studies, 75%–80% patients have deranged LFT, most commonly, an elevated gamma-glutamyl transferase.

In one study involving 198 patients, the sensitivity of ultrasound for MS was as high as 77.8%.¹² Though endoscopic

retrograde cholangiopancreatography (ERCP) is the gold standard for MS, MRCP is a highly sensitive non-invasive diagnostic investigation with sensitivity approaching 100% and is preferred over ERCP.³ Heavily T2-weighted axial MR images can easily demonstrate the closely lying cystic duct and CHD which may sometimes be missed on MRCP.⁶ Endoscopic ultrasound can also be performed prior to ERCP.12 Percutaneous transhepatic cholangiography and intraductal ultrasonography for identification of fistula are also options.^13–15

Management of PCMS is complicated by the presence of dense adhesions in Calot’s triangle and subhepatic region due to longstanding inflammation, which increases the risk of bile duct injury or significant bleeding. Complications of MS include biliary cutaneous fistula, secondary biliary cirrhosis and lateonset biliary strictures, which also make the surgical management difficult.¹² The management depends on whether the stone is in cystic duct stump or gall bladder remnant.

For cystic duct stump calculus leading to PCMS, endoscopic management is feasible with options including sphincterotomy and balloon or basket use/holmiumlaser lithotripsy, electrohydraulic lithotripsy, mechanical extracorporeal shockwave lithotripsy for stone fragmentation or chemical dissolution followed by retrieval of stone fragments endoscopically.^{3 6} In remnant gall bladder after SC with calculus in the cystic duct, endoscopic manipulation of stone back into the gall bladder followed by LCC is also an option.⁶ However, endoscopic retrieval of retained gall bladder remnant calculus should not be attempted.³

PCMS due to gall bladder remnant calculus can be managed by a preoperative ERCP with sphincterotomy and stent followed by LCC or open CC. Single-stage LC is also an option, to be attempted by experienced laparoscopic surgeons at advanced laparoscopy centres.³ In a study, preoperative drainage with an endoscopic stent, or a nasobiliary drain placement by ERCP, was used to facilitate surgery.¹² The advantage of preoperative ERCP is that it allows for the acute inflammatory stage to resolve, thereby reducing the difficulty of the surgery, and the resulting morbidity. The disadvantage is the addition of an invasive procedure, with its inherent risks and complications.

CC is feasible in cases of type 1 MS due to any cause and is the treatment of choice to avoid the above-mentioned complications of MS unless the patient is unfit for surgery.^{3 12} In that scenario, endoscopic management is preferred. After SC, intense inflammation and dense adhesions make identification of cystic duct and Calot’s triangle difficult. Hence, LCC is not routinely attempted.4 However, with growing expertise, laparoscopic management is considered safe and feasible for type 1 MS, even in postcholecystectomy patients with MS due to remnant gall bladder calculus. In a recent study by Sanjay Kumar Saroj et al, the re-exploration rates in laparoscopic and open CC for type 1MS were found similar. ¹

During LCC for PCMS, care should be taken to remain above the Rouviere’s sulcus, achieve the critical view of safety, dissect from right to left to avoid the bile duct and minimise the use of energy devices in Calot’s triangle. A low threshold should be kept for conversion to open surgery. In contrast, prevention of PCS due to gall bladder remnant or cystic duct stump stone is possible by meticulous dissection of the cystic duct up to its junction with bile duct followed by the milking of duct before clipping. Liberal use of intraoperative cholangiogram delineates the anatomy well and avoids bile duct injuries, in addition to identifying any residual CBD stones missed on MRCP. Achieving the critical view of safety is of paramount importance to avoid bile duct injury and related causes of PCS. Also, the cystic duct stump should not be kept >5mm long when a cystic duct stump is present.^{3 4}

Learning points:

• Postcholecystectomy Mirizzi syndrome (PCMS), although rare, should be one of the differential diagnoses in patients presenting with postcholecystectomy syndrome.
• Axial MR images should be studied since coronal MR cholangiopancreatography images may miss subtle compression of common hepatic duct.
• Laparoscopic completion cholecystectomy is a safe and feasible option for type 1 PCMS when carried out by experienced surgeons.
• The threshold for conversion to open surgery should be low in such cases.

Contributors GSD, RN and PP: worked for the literature review and the manuscript preparation under the creative inputs, critical evaluation and guidance of PW. All authors have worked on the article generation and editing.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests Not declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.

References:

1. Saroj SK, Kumar S, Afaque Y, et al. The Laparoscopic Re-Exploration in the Management of the Gallbladder Remnant and the Cystic Duct Stump Calculi. J Clin Diagn Res 2016;10:PC06–PC08.
2. Nagorni EA, Kouklakis G, Tsaroucha A, et al. Post-laparoscopic cholecystectomy Mirizzi syndrome induced by polymeric surgical clips: a case report and review of the literature. J Med Case Rep 2016;10:1–7.
3. Amin A, Zhurov Y, Ibrahim G, et al. Combined Endoscopic and Laparoscopic Management of Postcholecystectomy Mirizzi Syndrome from a Remnant Cystic Duct Stone: Case Report and Review of the Literature. Case Rep Surg 2016;2016:1–9.
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