Intrahepatic lithiasis is rare in the western countries. On the contrary it is frequent in Southeast Asia (China, Japan, South Korea). It is characterized by gallstones within the bile ducts proximal to the confluence of the right and left hepatic ducts, with or without stones in the gallbladder or in the main bile duct. This disease is usually associated with repeated episodes of acute cholangitis, liver atrophy, bile ducts stenosis, secondary biliary cirrhosis, and intrahepatic cholangiocarcinoma. While in med school all surgeons read about this disease and its link to Clonorchis sinensis and ascariasis. During my 6 years of surgery residency, I saw none. Neither during the first 8 years of general surgeon I was presented with one of such cases. Although its rarity, we must know how to diagnose and treat this entity. Experience should be a way to settle knowledge, not the ultimate way of learning. Learning comes first, experience comes after.
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Do you know how to treat intrahepatic lithiasis? Do you know which are the drawbacks of its treatment? What is the recurrence rate? The idea of this post followed a paper published by Prof. Dr. Orlando Torres, Brazil, in the Asian Journal of Surgery, where the results of a group of 127 non-Asian patients treated for intrahepatic lithiasis in 6 Brazilian Centers are presented. Since this entity is also a rarity in America, the authors analyzed the data. Clinical presentation in Brazil was like other studies. Most patients with intrahepatic lithiasis are symptomatic and present with abdominal pain, jaundice, fever, nausea and vomiting. Acute cholangitis is common and irreversible lesions can arise (biliary duct stenosis, liver atrophy, cirrhosis). This is the group of patients who benefit the most with resection. Surgery must remove all stones, the entire diseased liver parenchyma (infected), all stenotic intrahepatic ducts, and promote an adequate biliary drainage. Only by doing so we can reduce recurrence and avoid intrahepatic cholangiocarcinoma. The authors say. This is what Prof. Dr. Orlando Torres et al offered to their patients, and with good outcomes. Other nonsurgical treatments can be offered to asymptomatic patients or to those not suitable for surgery.
Resection is advised in symptomatic patients.
The authors achieved 88,2% of good long-term results.
According to the authors' data, most patients presented with intrahepatic lithiasis in the left lobe (49,6%). This is consistent with the remaining literature. One possible reason is the angle between the left hepatic duct and the main bile duct, which can promote bile stasis and gallstones formation. Bilobar stones were found in 28,3% of cases. Interesting to read is the biochemical results of this group of Brazilian patients. Serum bilirubin was elevated only in 19,7% of patients, alkaline phosphatase in 15% and aminotransferases in 7,8%. I believe this is particularly important for diagnosis. It means we cannot support our diagnosis in blood tests but that clinical evaluation is paramount for diagnosis. All doctors must have a low threshold of suspicion. The most frequently performed procedures were left lateral sectionectomy (segments 2 and 3) in a total of 63, left hepatectomy (segments 2, 3 and 4) in 36 patients, and 19 right hepatectomies (segments 5, 6, 7 and 8). Additional biliary drainage was performed in 28 patients (22%) by a Roux-en-Y hepaticojejunostomy. What surprised me was the low rate of laparoscopic procedures, only four patients. According to authors the fibrosis and the past medical history of recurrent infection of both the bile ducts and parenchyma, justifies this low rate of minimally invasive surgery. I believe in the future this will probably change as the outcomes become identical.
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Recurrence was only found in 10 patients (7,8%). Eight of them were patients with bilobar stones and hepaticojejunostomy. These data are crucial to understand how to reduce recurrence. Is hepaticojejunostomy a risk factor? According to the authors and worldwide literature, bilioenteric anastomosis is associated with higher rate of biliary fistula and recurrence. This technique is indicated for patients with dilated bile duct > 2 cm. However, when it is necessary the outcome is worse. Because of this conclusion the authors state that recurrence of symptoms revealed that bilioenteric anastomosis is not the ideal solution for intrahepatic lithiasis“. What can we do differently? No answer… Treating intrahepatic lithiasis with resection allowed the authors to achieved good long-term results in 88,2% of patients. Only two had a cholangiocarcinoma in the left atrophic lobe. Excellent results in a difficult entity, I would say.
Another interesting idea from this paper is the fact that biliary fistula (the most common complication) is more frequent following a resection because of intrahepatic lithiasis than because of another condition. I ask: Should we use a drainage? Yes, we should. It will do no harm and can soften consequences from a biliary fistula. The authors do not talk about the use of a sealant on the raw surface to decrease fistula rate. Would it help? I personally use it. If we can avoid one fistula, it is worth the money. Must not forget that in liver surgery the most important is not what we take but what we left. A good remaining liver must be our goal. For a non-cirrhotic liver: at least two adjacent segments, with a good blood inflow and outflow, with adequate biliary drainage. That is why treating bilobar intrahepatic stones is not easy. In a case of bilobar disease, resection of the atrophic liver and stenotic ducts must be complemented with intraoperative gallstones' clearance of the remaining liver.
The take home messages I highlight from this paper are:
most patients are symptomatic (previous history of acute cholangitis is common)
CT scan and/or MRCP (magnetic resonance) are crucial to identify stones and decide best surgical treatment
resection is advised in symptomatic patients and/or with irreversible lesions to parenchyma and bile ducts
resection must remove all stones, the entire diseased parenchyma and all stenotic ducts
only by doing so we can reduce recurrence, residual stones and cholangiocarcinoma
resection has better results comparing to choledocholithotomy or bilioenteric anastomosis alone or other nonsurgical approaches
biliary fistula is the most frequent complication
Keep learning everything you can. Never know when you're going to need it...
I advise reading this paper to answer some doubts you may have.
Thank you to Prof. Orlando Torres for sharing this paper.
Link to article:
Dr. Carlos Eduardo Costa Almeida
General Surgeon
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