Makindo Medical Notes.com

Mirizzi Syndrome is a rare condition that occurs when a gallstone becomes impacted in the cystic duct or neck of the gallbladder, leading to compression or obstruction of the common bile duct (CBD)

About

• Rare cause of obstructive jaundice

Aetiology

• Gallstone Impaction: gallstone lodged in the cystic duct or the neck of the gallbladder.
• Bile Duct Compression due to impacted stone either directly or indirectly, causing partial or complete obstruction.
• Chronic Inflammation can exacerbate the compression of the bile duct and lead to further complications.

Clinical

• Jaundice: Yellowing of the skin and eyes due to the buildup of bilirubin in the blood.
• Right Upper Quadrant Pain: Pain in the upper right abdomen, which can be severe and persistent.
• Fever and Chills: Often indicative of a secondary infection such as cholangitis (infection of the bile duct).
• Nausea and Vomiting: Common gastrointestinal symptoms associated with biliary obstruction.
• Pruritus: Itching, due to the accumulation of bile acids in the skin.
• Dark Urine and Pale Stools: Resulting from the obstruction of bile flow.

Classification

• Type I - No fistula present
• Type IA - Presence of the cystic duct
• Type IB - Obliteration of the cystic duct
• Types II-IV - Fistula present
• Type II - Defect smaller than 33% of the CBD diameter
• Type III - Defect 33-66% of the CBD diameter
• Type IV - Defect larger than 66% of the CBD diameter

Investigations

• Liver Function Tests (LFTs): Elevated bilirubin and alkaline phosphatase levels are common, indicating bile duct obstruction.
• Ultrasound: The initial imaging modality of choice, which may show gallstones and dilation of the bile ducts.
• Magnetic Resonance Cholangiopancreatography (MRCP): A non-invasive imaging technique that provides detailed images of the bile ducts and can help in visualizing the site of obstruction and any associated fistulas.
• Endoscopic Retrograde Cholangiopancreatography (ERCP): This procedure allows direct visualization of the bile ducts and can be both diagnostic and therapeutic, particularly in cases where intervention is needed to remove stones or place stents.
• CT Scan: May be used to assess the extent of inflammation and any complications.

Management

• Cholecystectomy: Removal of the gallbladder relieves the obstruction and prevent further complications.
• ERCP with Stone Removal or Stenting: ERCP can remove the impacted stone or place a stent to relieve the obstruction temporarily.
• Surgical Bile Duct Repair: If a fistula has formed, surgical repair of the bile duct may be required, which can range from simple closure to more complex reconstructive surgery, such as a Roux-en-Y hepaticojejunostomy.
• Antibiotics: If cholangitis is present before surgical intervention.
• Delayed diagnosis or treatment can lead to complications such as cholangitis, biliary cirrhosis, or even biliary tract cancer in long-standing cases.