Treatment of Cavernous Transformation of Portal Vein Caused by Hepatic Cystic Echinococcosis Using Ex Vivo Liver Resection and Autotransplantation.

BACKGROUND Hepatic cystic echinococcosis (HCE) is a frequently overlooked parasitic liver disease, for which the commonly recommended treatment is radical resection. However, this approach is often associated with severe comorbidities such as HBV/HCV, cirrhosis, and hepatic carcinoma, among others. CASE REPORT In this report, we present a case successfully managed by ex vivo liver resection and autologous liver transplantation (ELRA). In the described case, ex vivo resection was not feasible due to recurrent lesions and infections invading the portal vein, which resulted in portal vein cavernous transformation. CONCLUSIONS Through this paper, we aim to detail the treatment process, showcasing the feasibility and advantages of ELRA. Additionally, we propose a novel approach for the treatment of this disease, while emphasizing the importance of radical resection surgery to prevent long-term complications.

Concomitant Polymyalgia Rheumatica and Giant Cell Arteritis Associated with Cystic Echinococcosis: A Rare Geriatric Case.

INTRODUCTION: Parasitic infections could be an important triggering factor for autoimmune diseases. We present a clinical case of concomitant polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) induced with cystic echinococcosis (CE). CASE PRESENTATION: A 74-year-old male was admitted with a 2-month history of progressive pain at the shoulders and hip, movement restriction, and constitutional symptoms. As a result of the examinations performed due to rheumatological complaints, PMR and GCA were diagnosed. The cystic appearance, which was incidentally detected in the liver 6 months ago and not examined at that time, was found to be hydatid cyst. Medical treatment was initiated for all three conditions and the patient's symptoms improved significantly. DISCUSSION: Parasite infections may cause various autoimmune diseases because of molecular mimicry or sustained immune activation. Echinococcus granulosus is a very complex multicellular parasite and highly immunogenic for humans. Some body parts of the parasite, the outer surface and secreted particles, stimulate the host immune system strongly. CONCLUSION: The first case in the literature of coexistence of PMR and GCA associated with CE. Autoimmune diseases should be evaluated in patients with CE. Furthermore, CE should be considered in patients with autoimmune diseases in the presence of a cyst.

Early postoperative intra-axial dissemination of a pediatric extradural and complicated hydatid cyst.

Hydatid disease is very common around the Mediterranean basin and endemic in some parts of the world. Cerebral involvement remains rare, represents only about 2% of all hydatid localizations and mainly affects the pediatric population. Extradural hydatid cyst is very rare or even exceptional when it is associated with or followed by intracerebral disseminations. Here, the authors report a new exceptional case of an early multiple intra-axial hydatid dissemination in a 5-year-old North African male patient from a rural area who underwent surgery 3 months after a primary osteolytic extradural and complicated hydatid cyst with good clinical and radiological outcomes.

A case of pediatric primary osteolytic extradural and complicated hydatid cyst revealed by a skull vault swelling.

Hydatidosis is a parasitic infestation whose etiological agent is the larva of the cestode Echinococcus granulosus. It is a zoonosis, and the human being behaves as an accidental intermediate host in the parasitic cycle with pediatric predominance. The most frequent clinical presentation is hepatic, followed by pulmonary, with cerebral hydatidosis being extremely rare. Imaging is characteristic, generally dealing with single cystic lesion, usually unilocular and less frequently multilocular, located mainly intraaxially. Extradural hydatid cyst, whether primary or secondary, remains very rare or even exceptional. The primary disease remains extremely rare, and its clinical picture is related to the number, size, and location of the lesions. Infection within these cerebral hydatid cysts remains an extremely rare occurrence, and only few cases were reported previously in the literature. The authors report the nosological review of the clinical, imaging, surgical, and histopathological records of a pediatric primary osteolytic extradural and complicated hydatid cyst in a 5-year-old North African male patient coming from a rural area who presented for progressive onset of a painless left parieto-occipital soft swelling without any neurological disorder with good outcomes after surgery. The authors report this case due the fact that it had not been documented before in the pediatric population and to the success of the specialized treatment.

A rare case of hydatid cyst-associated AA amyloidosis and literature review of hydatid cyst-associated nephropathy.

Hydatid disease is an infective picture caused by echinococcus, which progresses with cysts in various organs, especially in the liver. Renal involvement is an unusual location in the course of the disease. Although mostly asymptomatic renal cysts are seen, rarely glomerular or tubular associated nephropathy develops. In addition, the development of amyloidosis has been shown previously in patients with untreated chronic hydatid cysts. We wanted to bring a 27-year-old female patient with a 10-year history of hydatid cyst and AA amyloidosis to the literature. In addition, in our literature review for hydatid disease-associated nephropathies, we brought together data from 12 studies involving a total of 21 cases. Of these cases, 3 were membranous glomerulonephritis (MGN), 5 were, membranoproliferative glomerulonephritis (MPGN), 1 was minimally change disease (MCD), 5 were AA amyloidosis (including our case), 3 were immunoglobulin A nephropathy (IgAN), 1 was tubulointerstitial nephritis (TIN), 1 was chronic kidney disease (CKD), 1 was TIN with mesangioproliferative glomerulonephritis (MesPGN), 1 was TIN with IgAN, 1 was MPGN with immunoglobulin M nephropathy (IgMN). In this way, we wanted to shed light on the relationship between Echinococcus and nephropathy. In this way, we wanted to emphasise the necessity of doing renal examinations in the follow-up of hydatid cyst patients.

Evaluation of the features of cystic echinococcosis with concurrent super-infection: a retrospective study in Southern Iran.

OBJECTIVE: Superinfection of cystic echinococcosis (CE) is a life-threatening complication with significant morbidities, which can be prevented with early diagnosis and treatment. This study aims to examine the clinical characteristics, diagnostic methods, and treatment options for superinfected CE, as there is currently limited information available on the differences between superinfected and non-infected CE in terms of clinical features, serological and radiologic findings. METHODS: This cross-sectional study was conducted on hospital records of patients who were diagnosed with hydatid cysts in a 15-year period (2004 to 2018) in two main university-affiliated referral centers in Fars province, southern Iran. Patients' information regarding the demographical and clinical features related to CE, age, sex, previous history of CE or recurrence, size and location of CE, and length of hospital stay were collected. Moreover, the characteristics of concurrent infections with hydatid cysts were recorded. RESULTS: A total of 586 surgeries due to CE were performed on 501 patients, of which 67 (11.43%) had reoperations due to the recurrence of the disease. A total of 30 (5.99%) incidences of superinfection were observed. There were no statistically significant differences in terms of laboratory and imaging findings between CE patients with concurrent infections and other CE patients (p-value > 0.05). Among the patients with super-infection, four had fungal infections of the lungs. Aspergillus fumigatus was the causative pathogen in all four patients that were diagnosed with fungal superinfection. All patients underwent surgical excision with favorable long-term outcomes. CONCLUSION: Our study revealed a 5.99% incident rate of CE superinfection. Regarding the concurrent fungal infections in hydatid cysts, the patient's symptoms and laboratory and imaging findings are inconclusive and histopathological evaluation seems to be the most reliable option. Surgical resection is the gold-standard treatment option with favorable outcomes and potentially can be curative.

Rupture of a liver hydatid cyst into the right portal vein leading to right portal vein thrombosis: a case report and literature review.

Hydatid disease (HD) is a worldwide parasitic disease. HD is endemic in many sheep- and cattle-raising areas, with a high prevalence of 5% to 10% in the Mediterranean region. Fistulation of liver hydatid cysts (LHC) in the bile ducts is the most common complication, followed by rupture of cysts in the peritoneal and thoracic cavities. Vascular complications are a rare complication of HD. We describe the case of a 70-year-old woman who was admitted with the chief complaint of pain in the abdominal right upper quadrant for 6 months. Abdominal computed tomography revealed a large LHC in the right liver that had ruptured into the right portal vein branch, with venous thrombosis. Intraoperatively, the right portal vein was opened longitudinally, and the hydatid contents were evacuated. Right hepatectomy was performed to completely excise the LHC. The penetration of a cyst into an adjoining vessel is very rare, and portal vein invasion by HD is extremely rare, with only 10 cases published in the literature, to the best of our knowledge.

Precordial pain induced by the isolated cardiac hydatid cyst in interventricular septum: a case report.

BACKGROUND: Human hydatid disease occurs after infection with Echinococcus granulosus, mainly involves liver and lung, while hydatid involves heart is infrequent. A great majority of hydatid diseases could be asymptomatic, and incidentally found through examination. Here, we reported a woman who suffered an isolated cardiac hydatid cyst located at the interventricular septum. CASE PRESENTATION: A 48-year-old woman presented intermittent chest pain was admitted to the hospital. Imaging examination revealed a cyst located at the interventricular septum near the right ventricular apex. Considering medical history, radiological findings and serological results, cardiac hydatid disease was suspected. The cyst was successfully removed, while pathological biopsy confirmed the diagnosis of infection of Echinococcus granulosus. Postoperative course was uneventful, the patient was discharged from hospital without complications. CONCLUSION: For symptomatic cardiac hydatid cyst, surgical resection is necessary to avoid progression of disease. During surgical procedure, appropriate methods to reduce the potential risk of hydatid cyst metastasis are essential. Besides surgery, combined with regular drug therapy is an effective strategy to prevent reappearance.

Diagnosis and surgical treatment of cysto-gastric fistula out of an hepatic hydatid cyst. Unusual case.

BACKGROUND: Hydatid cyst is an endemic zoonotic infection that annual incidence ranges from <1 to 200 per 100,000 individuals. The most common complication of hepatic hydatid cyst reported is rupture of the cysts, most commonly intrabiliary rupture. Direct rupture to hollow visceral organs is rarely seen. We describe here an unusual cystogastric fistula in a patient with liver hydatid cyst. CASE PRESENTATION: The 55-year-old male patient presented with right upper quadrant abdominal pain. After radiological imaging studies, the diagnose was of hydatid cyst involving the left lateral segment of the liver ruptured into the gastric lumen and resulted in a cystogastric fistula. Gastroscopy revealed that the cyst and its contents protruding from anterior wall to the gastric lumen. Partial pericystectomy and omentopexy were performed and the gastric wall was primarily repaired. There were no complications in the postoperative period and 3-month follow up. CONCLUSION: This case, to our knowledge, is the first reported case of cystogastric fistula surgically treated in a patient with liver hydatid cyst in the literature. Our clinical experience shows that, although it is a benign disease, complicated hydatid cysts should be evaluated in detail preoperatively, and after the detailed diagnostic work-up, surgical therapy might be planned individually for each case. KEY WORDS: Cysto-gastric fistula, Hydatid Cyst, Liver hydatidosis.

Cardiac Hydatid Cyst: An Uncommon Cause of Complete Atrioventricular Block. / Cisto Hidático Cardíaco: Uma Causa Incomum de Bloqueio Atrioventricular Total.

Hydatidosis is a zoonosis caused by Echinococcus granulosus, leading to the formation of cysts on involved organs. Cardiac involvement is rare and can cause a wide range of complications secondary to rupture, embolization, or compression. Its diagnosis is challenging, and is generally confirmed through data related to clinical manifestations, environmental exposure, and laboratory and imaging exams. Surgical removal is necessary in most cases, in which an association with antiparasite therapy is recommended. The present article describes a case of a cardiac hydatid cyst associated with a complete atrioventricular block (AVB) in a young adult patient, with the need for a pacemaker implant, an atypical presentation, and scarce reports in the literature.

A hidatidose é uma zoonose causada pelo Echinococcus granulosus, levando à formação de cistos nos órgãos acometidos. O envolvimento cardíaco é raro e pode causar diversas complicações secundárias à ruptura, embolização ou compressão. Seu diagnóstico é desafiador, sendo confirmado por meio de dados relacionados a manifestações clínicas, exposição ambiental, exames laboratoriais e de imagem. A ressecção cirúrgica é necessária na maioria dos casos, sendo indicada a associação com terapia antiparasitária. No presente artigo, descreve-se um caso de cisto hidático cardíaco associado a bloqueio atrioventricular total em paciente jovem, com necessidade de implante de marcapasso, uma apresentação atípica e pouco relatada na literatura.

NOTCH signaling inhibition after DAPT treatment exacerbates alveolar echinococcosis hepatic fibrosis by blocking M1 and enhancing M2 polarization.

Alveolar echinococcosis (AE) is a lethal helminthic liver disease caused by persistent infection with Echinococcus multilocularis (E. multilocularis). Although more and more attention has been paid to the macrophages in E. multilocularis infection, the mechanism of macrophage polarization, a critical player in liver immunity, is seldom studied. NOTCH signaling is involved in cell survival and macrophage-mediated inflammation, but the role of NOTCH signaling in AE has been equally elusive. In this study, liver tissue samples from AE patients were collected and an E. multilocularis infected mouse model with or without blocking NOTCH signaling was established to analyze the NOTCH signaling, fibrotic and inflammatory response of the liver after E. multilocularis infection. Changes in polarization and origin of hepatic macrophages were analyzed by flow cytometry. In vitro qRT-PCR and Western blot assays were performed to analyze key receptors and ligands in NOTCH signaling. Our data demonstrated that hepatic fibrosis develops after AE, and the overall blockade of NOTCH signaling caused by DAPT treatment exacerbates the levels of hepatic fibrosis and alters the polarization and origin of hepatic macrophages. Blocking NOTCH signaling in macrophages after E. multilocularis infection downregulates M1 and upregulates M2 expression. The downregulation of NTCH3 and DLL-3 in the NOTCH signaling pathway is significant. Therefore, NOTCH3/DLL3 may be the key pathway in NOTCH signaling regulating macrophage polarization affecting fibrosis caused by AE.

Percutaneous Treatment of Giant Hydatid Cysts and Cystobiliary Fistula Management.

RATIONALE AND OBJECTIVES: To investigate the efficacy and safety of percutaneous treatment in cystic echinococcosis (CE) type 1 and 3a giant cysts (with at least one diameter>10 cm) according to the World Health Organization classification and to evaluate the management of complications, especially cystobiliary fistulas (CBFs). MATERIALS AND METHODS: This retrospective study included 66 patients with 68 CE1 and CE3a giant cysts treated with percutaneous catheterization between January 2016 and December 2021. The characteristics of the cysts, major and minor complications, time to catheter removal, and length of hospital stay were recorded. RESULTS: Among the 68 cysts, CBFs occurred in 35 (51.5%), cavity infections in 11 (16.1%), recollection in five (7.4%), and anaphylaxis in three (4.5%). There was no mortality. Biliary drainage was observed intraoperatively in 20 (29.4%) and only postoperatively in 15 (22.1%) of the 35 cysts with CBFs. A plastic biliary stent was placed in 18 (51.5%) of the 35 cysts with CBFs. The patients with CBFs had a longer hospital stay and time to catheter removal than those without fistulas (15.3 ± 10.9 vs. 6.1 ± 2.6 days and 32.7 ± 51.8 vs. 6.2 ± 3.1 days, respectively; P < 0.001). Of the patients who developed recollection, three were treated with secondary catheterization, and two underwent surgery. In total, three patients underwent surgery. The rate of clinical success was 95.4%. All cysts were followed up for an average of 19.1 (range, 12-60) months, and there was an average 88.8% reduction in cyst volume compared to the initial evaluation. CONCLUSION: CE1 and CE3a giant cysts can be treated effectively and safely with high clinical success using the catheterization technique. Contrary to what has previously been reported for these patients, the rate of CBFs is high, but these patients can successfully be treated with percutaneous drainage and/or endoscopic retrograde cholangiopancreatography without the requirement of surgery.

[Rare complication of echinococcosis in clinical practice]. / Redkoe oslozhnenie ekhinokokkoza.

The authors report retroperitoneal echinococcosis with destruction of the bodies and left transverse processes of L4-5 vertebrae, recurrence and pathological fracture of L4-5 vertebrae with secondary spinal stenosis and left-sided monoparesis. Retroperitoneal echinococcectomy, pericystectomy, decompressive laminectomy L5 and foraminotomy L5-S1 on the left were performed. Therapy with albendazole was prescribed in postoperative period.

Hidatidosis pancreática en una niña de 5 años / Pancreatic echinococcosis in a 5-year-old girl

La hidatidosis pancreática representa el 0,2-0,6 % de los casos, siendo la población pediátrica la de mayor riesgo. Las lesiones suelen localizarse en cabeza del páncreas (50-58 %); la localización en cuerpo y cola del páncreas se encuentra en el 24-34 % y el 19 %, respectivamente. Dada la posibilidad de complicaciones, suele realizarse tratamiento quirúrgico. Se sugiriere indicar albendazol antes y después del acto quirúrgico por los riesgos de ruptura y diseminación de los protoescólices. Se presenta el caso de una niña de 5 años de edad con dolor abdominal progresivo y lesión quística en páncreas compatible con hidatidosis en la ultrasonografía. En la tomografía computada se observa compresión de la vía biliar. La hemoaglutinación indirecta fue negativa. Presentó elevación de la bilirrubina total, con franco predominio de bilirrubina directa, y aumento de enzimas hepáticas. Se realizó laparotomía exploradora, colecistectomía y destechamiento del quiste. Evolucionó favorablemente, continuó con albendazol durante 3 meses luego de la cirugía.

Pancreatic echinococcosis accounts for 0.2­0.6% of cases, with the pediatric population being at a higher risk. Most commonly, pancreatic lesions occur in the head of the pancreas (50­58%); and in the body and tail in 24­34% and 19% of cases, respectively. Given the potential complications, surgery is usually performed. Albendazole is recommended before and after the surgery due to the risks for rupture and dissemination of protoscolices. Here we describe the case of a 5-year-old girl with progressive abdominal pain and cystic lesion in the pancreas compatible with echinococcosis in the ultrasound. The computed tomography showed bile duct compression. Indirect hemagglutination was negative. She had elevated total bilirubin, with a clear predominance of direct bilirubin, and high liver enzymes. Exploratory laparotomy, cholecystectomy, and unroofing of the cyst were performed. The patient had a favorable course and continued with albendazole for 3 months after the surgery.

THERAPEUTIC APPROACH OF COMPLICATED HYDATID DISEASE: ROLE OF ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY IN CHOLANGIOHYDATIDOSIS.

BACKGROUND: Hydatid disease, a parasitic infestation caused by Echinococcus granulosus larvae, is an infectious disease endemic in different areas, such as India, Australia, and South America. The liver is well known as the organ most commonly affected by hydatid disease and may present a wide variety of complications such as hepatothoracic hydatid transit, cyst superinfection, intra-abdominal dissemination, and communication of the biliary cyst with extravasation of parasitic material into the bile duct, also called cholangiohydatidosis. Humans are considered an intermediate host, exposed to these larvae by hand-to-mouth contamination of the feces of infected dogs. AIM: This study aimed to highlight the role of endoscopic retrograde cholangiopancreatography in patients with acute cholangitis secondary to cholangiohydatidosis. METHODS: Considering the imaging findings in a 36-year-old female patient with computed tomography and magnetic resonance imaging showing a complex cystic lesion in liver segment VI, with multiple internal vesicles and a wall defect cyst that communicates with the intrahepatic biliary tree, endoscopic biliary drainage was performed by endoscopic retrograde cholangiopancreatography with papillotomy, leading to the discharge of multiple obstructive cysts and hydatid sand from the main bile duct. RESULTS: Clinical and laboratory findings improved after drainage, with hospital discharge under oral antiparasitic treatment before complete surgical resection of the hepatic hydatid cyst. CONCLUSIONS: Endoscopic retrograde cholangiopancreatography is a safe and useful method for the treatment of biliary complications of hepatic hydatid disease and should be considered the first-line procedure for biliary drainage in cases of cholangiohydatid disease involving secondary acute cholangitis.

Non-vertebral hydatidosis in bone: Difficulties in management.

OBJECTIVES: This study presents our experience in surgical treatment of extravertebral bone hydatidosis and aims to investigate the utility of specific immunoglobulin E (IgE) in diagnosis and prognosis of the disease. PATIENTS AND METHODS: Between January 1990 and December 2019, a total of 10 patients (6 males, 4 females; mean age: 47.2±14.7 years; range, 27 to 71 years) with non-vertebral bone hydatidosis surgically treated in our hospital were retrospectively included. Curettage or wide resection was performed in all cases, followed by medical antihelminthic therapy. Specific IgE p2 was studied in seven patients during and at final follow-up. RESULTS: At the time of diagnosis, secondary infection of the cyst was observed as the initial symptom in two patients mimicking an abscess and, in both cases, more surgeries were required without final healing. In two cases, over five specific IgE presented a false negative at the time of diagnosis and it was not correlated with clinical evolution in three cases over seven. In six cases, diagnosis was obtained before surgery. In treatment, pelvic disease had the worst prognosis (none healed) and bacterial overinfection was a common complication after surgery. At the final follow-up, only two femoral cases (20%) were free of disease. Other four cases (three in iliac bone, one in proximal femur) needed several surgeries without healing. The other four patients showed no progression or refused a new surgical treatment. CONCLUSION: Location, bone defect, when it is possible to perform a radical surgery, and associated bacterial overinfection after surgery make cystic hydatidosis in bone an infection very difficult to treat definitively in humans. Negative specific IgE does not exclude bone hydatidosis.

Pancreatic echinococcosis in a 5-year-old girl. / Hidatidosis pancreática en una niña de 5 años.

Pancreatic echinococcosis accounts for 0.2-0.6% of cases, with the pediatric population being at a higher risk. Most commonly, pancreatic lesions occur in the head of the pancreas (50-58%); and in the body and tail in 24-34% and 19% of cases, respectively. Given the potential complications, surgery is usually performed. Albendazole is recommended before and after the surgery due to the risks for rupture and dissemination of protoscolices. Here we describe the case of a 5-year-old girl with progressive abdominalpain and cystic lesion in the pancreas compatible with echinococcosis in the ultrasound. The computed tomography showed bile duct compression. Indirect hemagglutination was negative. She had elevated total bilirubin, with a clear predominance of direct bilirubin, and high liver enzymes. Exploratory laparotomy, cholecystectomy, and unroofing of the cyst were performed. The patient had a favorable course and continued with albendazole for 3 months after the surgery.

La hidatidosis pancreática representa el 0,2-0,6 % de los casos, siendo la población pediátrica la de mayor riesgo. Las lesiones suelen localizarse en cabeza del páncreas (50-58 %); la localización en cuerpo y cola del páncreas se encuentra en el 24-34 % y el 19 %, respectivamente. Dada la posibilidad de complicaciones, suele realizarse tratamiento quirúrgico. Se sugiriere indicar albendazol antes y después del acto quirúrgico por los riesgos de ruptura y diseminación de los protoescólices. Se presenta el caso de una niña de 5 años de edad con dolor abdominal progresivo y lesión quística en páncreas compatible con hidatidosis en la ultrasonografía. En la tomografía computada se observa compresión de la vía biliar. La hemoaglutinación indirecta fue negativa. Presentó elevación de la bilirrubina total, con franco predominio de bilirrubina directa, y aumento de enzimas hepáticas. Se realizó laparotomía exploradora, colecistectomía y destechamiento del quiste. Evolucionó favorablemente, continuó con albendazol durante 3 meses luego de la cirugía.