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Liver transplant outcome in hepatic hydatidosis: A case series

Soheila Milani *1,Shahrzad Maragheh Moghaddam 2 and Nahid Zirak 3

1, 3 Transplant Research Center, Clinical Research Institute, Mashhad University of Medical Sciences, Mashhad, Iran
2Mashhad University of Medical Sciences, Mashhad, Iran
3Department of Anesthesia and Intensive Care, Mashhad University of Medical Sciences, Mashhad, Iran

*Corresponding author

*Soheila Milani, Department of Anesthesia and Intensive Care, Mashhad University of Medical Sciences, Mashhad, Iran

Abstract

Introduction: Hydatidosis is a parasitic infection characterized by the growth of cysts, primarily in the liver, which can result in various complications, including vessel and bile duct compression, liver failure, portal hypertension, and cirrhosis. Liver transplantation (LT) can be lifesaving for patients with end-stage hydatid liver disease.

Method: This is a retrospective study with data collected from medical records. The unique case series aims to highlight the outcome of LT in hepatic hydatidosis. This series was conducted at our Transplant Center from August 2016 to January 2025. All patients who underwent deceased donor LT with a definitive diagnosis of hydatid liver cysts were included in the study.

Results: The series included five cases. The patients' ages ranged from 26 to 67 years, with a predominance of women. The Model for End-Stage Liver Disease score was between 7 and 12. The left lobe of the liver was involved in only one case. Of the five cases, three had good outcomes with no recurrence or complications during follow-up durations of 98, 39, and 15 months.

Conclusion: Although liver transplantation can be a lifesaving option for patients with end-stage hydatid liver disease, it poses risks such as hydatid cyst recurrence, infections, vascular complications, and side effects from immunosuppressive therapy. Proper preoperative and intraoperative management, along with long-term follow-up, is crucial for better outcomes.

Keywords: Liver Transplantation, Echinococcus, Recurrence

Abbreviations: LT: Liver transplantation; MELD: Model for End-Stage Liver Disease; IVC: Inferior vena cava; COVID-19: coronavirus disease of 2019.

Introduction

Hydatid liver disease is caused by infection with Echinococcus granulosus. This infection is prevalent in various regions worldwide, including several parts of Eurasia (such as the Mediterranean, southern and central parts of Russia, Central Asia, and China), Australia, some parts of the Americas, particularly South America, and North and East Africa [1]. Surgical intervention is one of the main therapeutic approaches for hydatid liver. The preferred treatment is the early resection of hepatic lesions, accompanied by adjuvant antiparasitic medications like albendazole and mebendazole. These drugs are thought to have parasitostatic effects, which help slow the growth of the lesions [2]. Liver transplantation (LT) is a viable treatment option for hydatid liver, including patients with advanced stages of disease who do not benefit from cyst resection as well as those with liver failure, portal hypertension, or recurrent cholangitis [3]. However, hydatidosis is a rare indication of LT in the world. In this context, LT may result in various potential complications. The objective of this unique case series is to emphasize the outcomes of liver transplantation for hepatic hydatidosis.

Methods
This retrospective series included five adult patients with a confirmed diagnosis of hydatid liver cysts who underwent deceased donor liver transplants from August 2016 to January 2025 at our transplant center. All patients were evaluated thoroughly with detailed history and clinical examination, ultrasonography, and CT scan of the abdomen. The liver transplant procedure was performed using the classic technique, without venovenous bypass or the piggyback technique. The liver grafts were cold-preserved with the University of Wisconsin solution. All cases were managed using the same anesthetic protocol. After LT, the immunosuppressive regimen typically included tacrolimus and prednisone. Prolonged immunosuppressive therapy following LT can increase the risk of parasite recurrence or reactivation; thus, albendazole was generally administered for six months post-transplant.

Case presentation

Demographic and Perioperative Data of the patients are summarized in Table 1.

Case 1: A 35-year-old woman presented with a history of left lobectomy for a liver hydatid cyst five years earlier. Color Doppler ultrasound showed alterations in portal vein velocity and flow consistent with portal hypertension. She underwent liver transplants in January 2017 due to multi-loculated involvement of the right lobe of the liver. The patient's regular follow-up shows that she has been well without any complications to date.

Case 2: A 26-year-old woman presented with symptoms of anorexia, weakness, fatigue, progressive jaundice, and generalized itching. Ultrasound and CT scan of her abdomen revealed a cystic lesion occupying a significant portion of the right lobe of the liver, as well as another cystic lesion located at the medial margin of the left lobe of the liver. Additionally, the intrahepatic branches of the portal vein and the hepatic veins in the right lobe were completely obstructed. She underwent LT with a deceased donor in August 2016, due to the impossibility of surgical resection of liver hydatid cyst.      In May 2022, the patient underwent a liver re-transplantation due to recurrent hepatic hydatid disease and severe liver failure.

Case 3: A 46-year-old man presented with symptoms of abdominal fullness, early satiety, and vague abdominal pain and underwent further evaluation. Ultrasound revealed hepatomegaly, a heterogeneous cystic mass, chronic portal vein thrombosis, and portal hypertension. A spiral CT scan of the abdomen showed an enlarged liver, with hypodense masses occupying a significant portion of the left lobe and parts of the right lobe adjacent to the Inferior vena cava (IVC). The mass was reported to obstruct the main portal vein and invasion into the IVC. The patient underwent a liver transplant in May 2019 and was discharged in good general condition 13 days later. The patient was disease-free with normal liver function 16 months after the transplant. Unfortunately, he died due to coronavirus disease of 2019 (COVID-19) in September 2020.

Case 4: In 2019, a 63-year-old woman was assessed for abdominal pain and diagnosed with a hydatid liver cyst, which was subsequently surgically resected and treated with albendazole. In November 2021, imaging studies revealed a large mass, approximately 17 cm in diameter, involving most of the inferior segment of the right liver lobe, with strong evidence suggesting hepatic echinococcosis. Color Doppler imaging revealed that the right portal vein branch and the proximal sections of the left portal vein branch traversed through this mass. Additionally, the lesion compressed the superior vena cava in its intrahepatic portion and obstructed the right hepatic vein. The patient underwent liver transplants in December 2021. Follow-up assessments indicate that she is in good overall condition without any complications to date. Figure 1: shows the explanted liver of the patient in case 4.

Case 5: A 63-year-old woman presented with dull abdominal pain, loss of appetite, abdominal distension, and a sensation of fullness. Imaging studies revealed a large mass that completely replaced the right lobe of the liver and extended into the left lobe. Strong evidence indicated the presence of hepatic hydatid disease. The inferior vena cava, intrahepatic segments, hepatic venous branches, and the right branch of the portal vein were not visible, while the main portal vein and the left branch of the portal vein appeared normal. The patient underwent a liver transplant in November 2023. She has been well without any complications to date.

Figure 1:   The explanted liver of the patient in case 4.

Figure 2:   The explanted liver of the patient in case 5.

Table 1: Characteristics and perioperative data of patients.

Discussion

Hydatid liver disease is curable with minimal complications in the early stage.  However, LT can be lifesaving for patients in the end-stage of the disease. Hence, we present this case series to clearly describe the disease spectrum of clinical presentation, liver transplant treatment options, and the associated prognosis. There are only a few reports on this topic in the literature. There has been a case reported of LT in hepatic hydatidosis in a 48-year-old male patient from Northern Brazil, who presented with recurrent episodes of cholangitis. On the 31st day after surgery, the patient died due to a pulmonary embolism, and the autopsy showed an apparent healthy hepatic graft [3]. Notably, to reduce the surgical difficulty and postoperative mortality, intracystic- abscess and cholangitis due to hepatic hydatidosis should be medically and percutaneously managed before LT.

Moreover, two cases have been reported of LT for hepatic hydatidosis in female patients also from northern Brazil. Case 1 involved a 51-year-old with obstructive jaundice and a MELD score of 24, while Case 2 was a 52-year-old with recurrent cholangitis and a MELD score of 20. Both patients underwent successful LT, received post-transplant albendazole therapy, and showed good outcomes with no complications during follow-up periods of 5 and 96 months, respectively [4]. The review of these reports indicates that, the First:  LT for liver hydatidosis is a complex and relatively rare medical procedure. The surgical approach is determined by factors such as the size and location of the cysts, their relation to bile ducts and blood vessels, as well as the clinical condition and complications of the patients, and the experience of the surgical team [5,6]. Second, the Model for End-Stage Liver Disease (MELD) score is essential for prioritizing LT candidates, although its direct impact on the procedure for hepatic hydatidosis is limited. This is shown in Table 1 of the present article.

The MELD score is determined by bilirubin, creatinine, and INR levels to evaluate liver dysfunction, particularly in cirrhosis. In patients with hydatid cysts, LT is often needed due to structural complications rather than hepatic failure. Vascular or biliary involvement may elevate the MELD score, but massive liver involvement without biochemical dysfunction may result in a low score.

Thus, in cases of hepatic hydatidosis, it should be necessary to establish alternative criteria to ensure timely transplantation. Third, in liver hydatidosis post-transplant outcomes rely on infection control and recurrence prevention rather than MELD-based survival estimates. In cases of hepatic hydatidosis, the five-year survival rate following LT has been documented to be 71%, with a disease-free survival rate of 58% [7]. Finally, further research on this topic is needed to establish a consensus on standardized guidelines.

Conclusion

Liver transplantation is a viable treatment option for patients with advanced hepatic hydatidosis, particularly those with liver failure or recurrent cholangitis. A multidisciplinary approach, including surgical intervention, antiparasitic therapy, and long-term follow-up, is essential to ensure optimal outcomes in these patients.

REFERENCES

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JCMCRI
ISSN: 3064-8025

Journal of Clinical & Medical Case Reports, Images (JCMCRI) ISSN: 3064-8025 is a peer-reviewed journal dedicated to publishing clinical images from all sectors of medicine. The goal of this magazine is to disseminate information about new discoveries and treatments in science and medicine.

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