Type 1 Mirizzi Syndrome- Cholelithiasis with Choledocholithiasis

Abstract

An uncommon consequence of cholelithiasis, Mirizzi syndrome is characterized by blockage of the typical bile duct as a result of an impacted gall stone in the cystic duct or gallbladder neck. We describe the scenario of a 40-year-old woman who has had epigastric discomfort for a year and a recent fever. Type I Mirizzi disease with choledocholithiasis was verified by imaging and ERCP, while laboratory tests showed increased bilirubin and liver enzymes. Common bile duct stenting was used to treat the patient at first, and then laparoscopic cholecystectomy. Symptoms resolved and metabolic indicators returned to normal during the uncomplicated postoperative recovery period. This example emphasizes how crucial it is to identify Mirizzi syndrome early and treat it multidisciplinaryly in order to lower the risk for complications and enhance patient outcomes.

Keywords

Introduction

Mirizzi Syndrome (MS) is an unusual complication arising from gallstones, marked by compression of the common bile duct (CBD) because of a stone lodged in the neck of the gallbladder or cystic duct. This is an account of Type I Mirizzi Syndrome in a female patient in his 40s complaining of abdominal pain for the past one year and fever for the past few days. Diagnostic imaging, along with endoscopic retrograde cholangiopancreatography (ERCP), demonstrated the existence of choledocholithiasis and biliary obstruction. After treating the patient with CBD stenting and laparoscopic cholecystectomy, the patient’s symptoms resolved. She had an uneventful recovery and there was a decrease in her bilirubin level and improvement in her clinical status over the course of her recovery. This case illustrates the need for early diagnosis and multidisciplinary management of Mirizzi Syndrome to prevent complications and improve the prognosis for the patient. In 1948, it was discovered that a rare side effect of chronic gallbladder stones was Mirizzi syndrome (MS). It refers to the set of symptoms caused by chronic gallstones that obstruct and lead to the formation of fistulas, the common bile duct (CBD). It could be attributable to extrinsic tension deriving out of a lodged gallstone in gallbladder neck and cystic duct as a consequence of an edema response brought on by persistent gallstone cholecystitis. When a patient presents with obstructive jaundice or ascending cholangitis, it is crucial to possess an elevated degree of suspicion for MS because it may go undiagnosed ^[1]. Early management is crucial to prevent future problems due to the distinctive presentation of this condition. MS usually peaks in late adulthood, and it seems that women are more likely than males to be affected by MS ^[2]. The most common standard treatment for mirizzi syndrome is either a subtotal or cholecystectomy. Although laparoscopy offers benefits, it is frequently converted to an open laparotomy, which can lead to a significant risk of complications ^[3].

Case Presentation

A female patient in his 40s, presented with a 1-year history of epigastric pain and a recent 2-day history of fever. She didn’t show any signs of jaundice, or gastrointestinal distress. His clinical and laboratory evaluation showed elevated bilirubin and liver enzymes. Imaging/ ERCP confirmed choledocholithiasis and Type I Mirizzi Syndrome. She was stented with CBD stent, and then laparoscopic cholecystectomy was done, which relieved her symptoms and normalised her biochemical parameters.

Investigation

In examination, she was conscious, oriented, and afebrile, without signs of pallor, pedal edema, icterus, cyanosis, clubbing, or lymphadenopathy. vital signs were stable: PR-84/min, BP-120/70 mmHg, RR-22/min, and SPO₂ 99% at ambient air. Abdominal examination revealed sensitivity in the epigastrium and right hypochondrium without organomegaly, unbound hernial orifices, normal bowel sounds, and normal external genitalia. Systemic examination showed a normal cardiovascular system with S1S2 sounds and no murmurs, a normal respiratory system with no added sounds, and a non-focal neurological examination. Biochemical analysis showed elevated bilirubin levels: indirect 2.8mg/dL, direct 6.6mg/dL, and total 9.4mg/dL. Examination revealed tenderness in the epigastrium and right hypochondrium, with stable vital signs. Laboratory results showed elevated bilirubin, ALT at 40U/L, AST at 54U/L, and alkaline phosphatase at 324U/L. Hematological findings included a total RBC count of 4.16x10^6/uL, hemoglobin 11.3g/dL, WBC count 7.36x10^3/uL, and differential counts within normal limits. A pus culture was sterile after 48 hours. ERCP findings indicated cholangitis, Mirizzi syndrome, and cystic duct calculi. CBD stenting was performed, and bile was sent for culture. Cholangiogram revealed dilated bile ducts with extrinsic compression in the upper CBD and minimal narrowing in the farthest CBD. Biliary dilatation was executed, and aneurysm was inserted. The patient underwent a normal Pan Endoscopy with no complications recorded. Subsequent cholangiography revealed dilation in the common hepatic duct, intrahepatic bile ducts on the right and left sides, and extrinsic compression on the upper part of CBD. Additionally, there was minimal narrowing observed in the farthest part of CBD. Further investigations included balloon trawling to extract any potential stones, which yielded negative results. Biliary dilatation was then carried out using a series of dilators, including 7 Fr, 8.5 Fr, and 10 Fr sizes. To ensure appropriate drainage and stenting, a A twin pigtail stent measuring 7 Fr x 5 cm was inserted across the left liver duct that is connected to the common bile duct. Notably, a pancreatogram was not conducted during the procedure. Post-procedure care and follow-up were handed over to the gastroenterology team for continued management and monitoring of the patient's condition.

Treatment

The surgical procedure was conducted under stringent aseptic measures and general anesthesia was used with the operating area being painted and draped with extreme care. The gallbladder was extracted through the 10mm epigastric port in a piecemeal fashion. Following the removal, a thorough wound wash was performed to ensure cleanliness, and complete hemostasis was achieved. A 24Fr drain was securely fixed, the 10 mm port site was closed with 1 Proline suture, and the skin was closed using 2-0 Ethilon sutures. Postoperative care instructions were provided, and the patient was monitored closely for any signs of complications.  Postoperatively, the patient was managed with Injection Pipzo 4.5g for a duration of 5 days and is administered to combat bacterial infections. Injection Xone 2g, to be taken once, serves as a potent antibiotic for a severe infection. Injection Vitamin K 1 ampoule, to be taken thrice daily for 3 days, is provided to address any potential vitamin K deficiency. Injection Fortwin 30mg, for as-needed use once daily, helps manage severe pain. Injection Phenergan 12.5mg, once daily, is prescribed for the management of nausea. Injection Fevastin 600mg, to be taken thrice daily, aids in treating specific medical conditions. Tablet Pan 40 mg, to be taken once daily, is prescribed for gastric acid suppression. Tablet Ursocol 300 mg, to be taken three times daily, helps in managing liver and gallbladder conditions. Tablet Thyronorm 100mcg, to be taken once daily, is prescribed for thyroid hormone replacement therapy.

Complications

Most common postoperative complication in this patient was Calot’s triangle was frozen with dense adhesions and inflammation further complicating surgical approach making anatomical dissection difficult and risky. However, feasible intraoperative measures allowed a simple and secure cholecystectomy without conversion to open surgery. There were no significant perioperative complications, although the complex anatomy necessitated a significant amount of surgical expertise and adaptation.

Outcome and follow up

The patient had an uneventful postoperative recovery. Her symptoms went away and her liver-function tests returned to normal. She was sent home on a regimen of antibiotics, analgesics and supportive therapy. Overall, a gastroenterology follow-up was planned with biliary stent removal at a later date and treatment of hypothyroidism.

DISCUSSION

A rare side effect of cholelithiasis called Mirizzi syndrome involves stones stuck in the cystic duct pinching the normal hepatic duct externally is known as Hartmann's pouch. Type I causes inflammation of the Calot triangle when a stone lodges in the Hartmann pouch or the cystic duct, obstructing the usual hepatic duct externally^[3,4-5] and external compression of the bile duct by an impacted gallstone in the infundibulum or cystic duct^[6].Gallstones are more common in women, people over 40, obese people, people who eat a high-fat diet, people who have relatives with a history of gallstones, people with diabetes, and people on estrogen-containing drugs. The majority of gallstones, often known as silent gallstones, are asymptomatic. Gallstones can vary in number, size, and location, and about one in five people will experience symptoms ^[7]. Imaging studies confirmed the presence of cholelithiasis, choledocholithiasis, and biliary dilatation. The patient underwent ERCP with CBD stenting followed by laparoscopic cholecystectomy, leading to a decline in bilirubin levels postoperatively. The successful resolution of the biliary obstruction and symptomatic improvement post-surgery indicate a positive outcome. The choice of procedures was appropriate given the biliary nature of the pathology. The prompt consultation with a medical gastroenterologist ensured a multidisciplinary approach to management.

Learning points/Take Home Message

• Mirizzi Syndrome is a rare entity which can present as acute cholecystitis and thus early imaging and diagnosis are paramount to prevent clinical delays and complications.
• A laparoscopic cholecystectomy after ERCP is an effective management for type I MS.
• Inflammation and anatomical distortion increased surgical difficulty; a high degree of intraoperative caution was required.
• Comprehensive care through an interdisciplinary approach involving gastroenterology and surgical management is critical for successful treatment outcome.
• Stent management and monitoring for any residual or recurrent disease require regular follow-up.

Patient’s Perspective

The patient expressed relief at the resolution of his symptoms and emphasized the importance of seeking medical attention for persistent respiratory complaints. He was satisfied with the treatment outcome and acknowledged the need for continued follow-up

REFERENCE

1. Murphy PB, Atwater AR, Mueller M. Allergic Contact Dermatitis. 2023 Jul 13. In: Stat Pearls [Internet]. Treasure Island (FL): Stat Pearls Publishing; 2025 Jan–. PMID: 30422461.
2. Lawrence DT, Anand RJ, Girish KJ, Tripaty TB. Ayurvedic management of Vicharchika (Eczema)-A Case Report. Journal of Ayurveda and Integrated Medical Sciences. 2023 Jul 29;8(6):240-4.
3. Tiwari VK. Ayurvedic management of vicharchika (eczema).
4. Anwar Niyaz O. Management of acne vulgaris with Dooshivishari Agada – a research article. World Journal of Pharmaceutical Research. 2024;13(5):1573. doi:10.20959/wjpr20245-31735.
5. Srikanthamurthy KR. Ashtanga Hridaya, Vol. 3. 1st ed. Varanasi: Chaukhambha Orientalia; 1997. Uttarasthana, Chapter 35, verses 39–40. p. 370.
6. Krishnamurthy MS. Basavarajeeyam. 1st ed. Reprint 2019. Chapter 18, verses 212–214. Varanasi: Chaukhambha Orientalia; 2014. p. 475. ISBN: 978-81-7637-303-6
7. Rasa Tantra Sara va Siddha Yoga Sangraha. Kharaliya Rasayana. Varanasi: Krishna Gopala Ayurveda Bhavana; p. 274.