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Case Report
Revised

Case Report: Portal cavernoma related to multiple liver hydatidosis: A rare case of fatal cataclysmic haemorrhage

[version 2; peer review: 2 approved]
Previous title: Case Report: Portal cavernoma secondary to multiple liver hydatidosis: A rare cause of cataclysmic haemorrhage in a young adult
PUBLISHED 25 Nov 2021
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Abstract

Clinical presentation of liver hydatidosis can vary from asymptomatic forms to lethal complications. We report herein a rare case of a 27-year-old male from a rural Tunisian region who presented with large-abundance haematemesis, haemodynamic instability, and marked biological data of hypersplenism. Endoscopy showed bleeding esophageal varicose veins that were ligated. Abdominal ultrasound concluded the presence of three type CE2 hydatic liver cysts causing portal cavernoma with signs of portal hypertension. Despite resuscitation, the patient died of massive rebleeding leading to haemorrhagic shock. Hepatic hydatid cyst should be considered as an indirect cause of gastrointestinal bleeding in endemic countries. Early abdominal ultrasound in varicose haemorrhage is essential in orienting the diagnosis.

Keywords

Case report, portal cavernoma, variceal bleeding, hydatidosis

Revised Amendments from Version 1

Changes consisted mainly in adding two more references in the introduction and discussion parts. Grammatical changes were also made in some parts. Additionally, the title and abstract were amended.

See the authors' detailed response to the review by Souheil Zayet

Introduction

Echinococcosis liver hydatidosis is endemic in the Maghreb countries.1 Vascular complications are exceedingly rare,2 however, its manifestations can be critical. The clinical presentation depends on the cyst’s segment localization. Compression or invasion of the hydatic cyst in the portal vein can lead to portal vein thrombosis or extrahepatic portal vein obstruction (EPVHO).3 This can rarely lead to cavernous transformation and portal hypertension explaining the origin of symptoms. Prognosis is generally good. Cataclysmic presentations have not been described in the literature.

Case presentation

A 27-year-old male patient, from Mornag, a rural area of Tunisia, without any medical history, presented to the Habib Thameur Hospital emergency room with massive upper gastrointestinal bleeding. On admission (day one) in August 2020, physical examination revealed diffuse mucocutaneous pallor, lesions of old scarifications in the left upper limb, a Glasgow Coma Score (GCS) of 15/15, tachycardia of 105 beats per minute, hypotension of 80/50 mmHg without signs of peripheral hypoperfusion. Abdominal examination revealed slight epigastric tenderness and enlarged spleen without hepatomegaly or skin signs of hepatocellular failure. The rectal digital examination came back stained with melena.

Laboratory investigation showed signs of hypersplenism including decreased count of white blood count of 2870 cells/mm3, thrombocytopenia of 46,000 cells/mm3, and normochromic normocytic anemia of 5.6 g/dL. Minor signs of hepatocellular insufficiency were also displayed including a low rate of prothrombin ratio of 60% and hypocholesterolemia of 2.87 mmol/L. He had neither cholestasis nor cytolysis. Albuminemia was normal at the value of 36 g/L. Acute kidney failure was noted (urea of 11.6 mmol/L) with a normal blood electrolytes. Anti-hepatitis C virus antibody, hepatitis B surface antigen and hepatitis B core antibody were all negative.

On day two, the patient was stabilized following fluid resuscitation and blood transfusion of two red blood cell concentrates. The patience was treated with a proton-pump inhibitor (omeprazole) and octreotide. Upper gastroduodenoscopy showed the presence of oesophageal varices with massive active bleeding, moderate hypertensive gastropathy and gastric varicose veins (Figure 1). Four elastics with a first kit of ligature were put in place but did not allow the control of bleeding. Five supplementary elastics in a second ligature kit allowed a reduction in bleeding but without total control of the haemorrhage.

70ff593b-a947-4783-86b4-6fb5af799fa8_figure1.gif

Figure 1. An upper gastroduodenoscopy demonstrating the presence of esophageal varices complicated by massive active bleeding, and gastric varicose veins.

An abdominal ultrasound (US) concluded the presence of multiple multiloculated cystic formations evoking hydatic cysts type CE2 of the WHO classification of segments I, IV, and V with the largest cyst measuring 40 mm (Figures 2a and 2b). A portal cavernoma with a dilated splenic vein and splenomegaly of 22 cm was described. Hepatic veins were permeable with a normal caliber. Diagnosis of multiple liver hydatidosis was retained based on the epidemiological context and the typical aspect in abdominal US.

70ff593b-a947-4783-86b4-6fb5af799fa8_figure2.gif

Figure 2. a and b: An abdominal ultrasound showing the presence of multiple multiloculated cystic formations evoking hydatic cysts type CE2 of the WHO classification of segments I, IV, and V.

After a week, the patient had a cataclysmic re-bleeding causing refractory hemorrhagic shock and disseminated intravenous coagulation leading to death.

Discussion

Hepatic hydatid cyst can invade or compress the portal vein and results in EPVHO. Portal cavernoma is the outcome of chronic portal vein obstruction. It results in the creation of collateral circulation.

As soon as portal hypertension is established, blood can run hepatopetal or hepatofugal from the liver through portosystemic collaterals. This leads to the development of gastroesophageal or ectopic rectal variceal bleeding which represents the most typical clinical feature as in our case.5 According to a prospective study by Noronha et al., (2016), 71% of patients with chronic EPVHO showed gastroesophageal varices in endoscopy.5

EPVHO is an uncommon entity in adults. Indeed, the incidence of having EPVHO for the general population does not exceed 1% with a very low mortality rate.6 Hepatic hydatid cyst can results in EPVHO whether by compressing or directly invading the portal vein2,7 Computerized tomography can help indicate the mechanism of thrombosis. Venous compressions are among the rarest complications of hydatid liver cysts even in endemic countries as shown in Table 1. Size and perivascular topography are two essential elements for its occurrence (tunisiechirurgicale.com).

Table 1. Incidence of vascular complications in liver hydatidosis in the literature.

SeriesSaleh et al.,8Eddeghai et al.,9Hafi et al.,10Ben Ameur et al., [tunisiechirurgicale.com]
Incidence0.8 %1 %0.8%1.8%

From a pathophysiological point of view, EPVHO is the consequence of the ‘Virchow’s Triad’: the extrinsic chronic compression of the vascular wall by the hydatic cysts decreases the blood flow rate thus creating endothelial injuries. This can lead to the formation of a bland thrombus.11

To our knowledge, few cases of hepatic hydatidosis revealed by portal hypertension have been reported in the medical literature since the publication of the first case in 1990.810 In a large Spanish cohort of 506 patients followed over 20 years, only two patients presented portal hypertension and variceal haemorrhage.12 This demonstrates how seldomly described is this complication in the literature.

Our case highlighted an unusual presentation. The portal vein thrombosis and the cavernous transformation remained undiagnosed until hematemesis due to varices rupture. Our young patient had no history of inflammatory bowel diseases, pancreatitis, cirrhosis, neoplastic condition or coagulopathy to explain the cavernoma. The only convincing cause was the compression of the hydatid cysts respectively in segments I, IV, and V of the portal vein leading to chronic thrombosis. The minor biological signs of hepatocellular insufficiency were probably secondary to the chronic evolution of the cavernoma, but the ultrasound did not show any signs of chronic hepatopathy. Ultrasound was an indispensable tool to orienting diagnosis as proved in the literature.13 In this particular case, our patient's prognosis was poor, unlike previous ones.

We summarize in Table 2 different cases of hepatic hydatid cyst causing portal cavernoma. The mean age was 58.2 years old. No signs of gastrointestinal bleeding were reported before diagnosis. Portal thromboses were mainly secondary to direct compression or invasion of the portal vein. The outcome was generally positive except in our case. Treatment was generally based on surgery and the drug albendazole.

Table 2. Cases of hepatic hydatid cyst causing portal cavernoma.

CaseAgeClinical presentationEndoscopyMechanism of thrombosisPrognosisTreatment
Ertan et al.,1477Fever and generalized weaknessNot doneDirect communication with the portal veinOn follow-upAlbendazole
Kirmizi et al.,1533Abdominal distension after mealsOesophageal and gastric fundal varices with no bleedingCompression of the hepatic hilumPreservedSurgery
Moisan et al.,1662Abdominal pain
Hepatomegaly
Grade I oesophageal varicesInvasion of the portal vein by daughter vesiclesPreservedAlbendazole + Propranolol
Colovic et al.,1756Right upper quadrant abdominal painNormalCompression of the hepatoduodenal ligamentPreservedSurgery
Kayecetin et al.,1863Fever, anorexia, jaundice and epigastric massNot doneAbdominal sepsis after the rupture of the liver cystPreservedSurgery, Albendazole

Our case illustrates the importance of considering non-cirrhotic portal hypertension secondary to liver hydatidosis especially in young patients in endemic countries. Urgent therapy and diagnostic investigations should be pursued simultaneously to ensure on-time intervention. Our case lacks abdominal computed tomography or magnetic resonance imaging which could have given a more accurate demonstration of the thrombosis mechanism. Our patient could have been a candidate for transjugular intrahepatic portosystemic shunt.

Conclusion

Liver hydatidosis remains a public health issue in Tunisia because of its high morbidity and, exceptionally, possible mortality. Our case highlights an unusual presentation due to the young age, symptomatic portal cavernoma, and fatal death of the patient. It shows the importance of considering hepatic hydatid cyst as a cause of portal hypertension and portal cavernoma on presentation of gastrointestinal bleeding. Early abdominal ultrasound has a valuable contribution to orienting diagnosis, especially in endemic low-middle income countries.

Data availability

All data underlying the results are available as part of the article and no additional source data are required.

Consent

Written informed consent for publication of their clinical details and clinical images was obtained from the patient’s family.

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Zouaghi A, Bellil N, Ben Abdallah K et al. Case Report: Portal cavernoma related to multiple liver hydatidosis: A rare case of fatal cataclysmic haemorrhage [version 2; peer review: 2 approved]. F1000Research 2021, 10:1097 (https://doi.org/10.12688/f1000research.74012.2)
NOTE: If applicable, it is important to ensure the information in square brackets after the title is included in all citations of this article.
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ApprovedThe paper is scientifically sound in its current form and only minor, if any, improvements are suggested
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Version 2
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PUBLISHED 25 Nov 2021
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Reviewer Report 26 Nov 2021
Souheil Zayet, Infectious Disease Department, Nord Franche-Comté Hospital, Trévenans, France 
Approved
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The authors responded ... Continue reading
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Zayet S. Reviewer Report For: Case Report: Portal cavernoma related to multiple liver hydatidosis: A rare case of fatal cataclysmic haemorrhage [version 2; peer review: 2 approved]. F1000Research 2021, 10:1097 (https://doi.org/10.5256/f1000research.79569.r101158)
NOTE: it is important to ensure the information in square brackets after the title is included in all citations of this article.
Version 1
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PUBLISHED 29 Oct 2021
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Reviewer Report 16 Nov 2021
Khaoula Chabbouh, Gastroenterology B department, Faculty of Medicine, La Rabta Hospital, University Tunis-El-Manar, Tunis, Tunisia 
Approved
VIEWS 8
This is an interesting case as it illustrates a rare complication of Echinococcosis liver hydatidosis which is still endemic in the Maghreb countries.

However, some remarks should be mentioned:
  1. The diagnosis was based
... Continue reading
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Chabbouh K. Reviewer Report For: Case Report: Portal cavernoma related to multiple liver hydatidosis: A rare case of fatal cataclysmic haemorrhage [version 2; peer review: 2 approved]. F1000Research 2021, 10:1097 (https://doi.org/10.5256/f1000research.77717.r98517)
NOTE: it is important to ensure the information in square brackets after the title is included in all citations of this article.
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Reviewer Report 03 Nov 2021
Souheil Zayet, Infectious Disease Department, Nord Franche-Comté Hospital, Trévenans, France 
Approved with Reservations
VIEWS 24
This submission is an observation of a patient with a rare presentation of portal cavernoma related to multiple liver hydatidosis.

This original document is well treated in its entirety and is of academic and scientific interest, although ... Continue reading
CITE
CITE
HOW TO CITE THIS REPORT
Zayet S. Reviewer Report For: Case Report: Portal cavernoma related to multiple liver hydatidosis: A rare case of fatal cataclysmic haemorrhage [version 2; peer review: 2 approved]. F1000Research 2021, 10:1097 (https://doi.org/10.5256/f1000research.77717.r98522)
NOTE: it is important to ensure the information in square brackets after the title is included in all citations of this article.
  • Author Response 08 Dec 2021
    Khalaf Ben Abdallah, Department of General Surgery, Habib Thameur Hospital, Tunis, 1008, Tunisia
    08 Dec 2021
    Author Response
    Thank you for your valuable comments.

    Your remarks were gladly taken into consideration. Needed changes were adressed.

    When it comes to surgical treatment, there is no consensus on ... Continue reading
COMMENTS ON THIS REPORT
  • Author Response 08 Dec 2021
    Khalaf Ben Abdallah, Department of General Surgery, Habib Thameur Hospital, Tunis, 1008, Tunisia
    08 Dec 2021
    Author Response
    Thank you for your valuable comments.

    Your remarks were gladly taken into consideration. Needed changes were adressed.

    When it comes to surgical treatment, there is no consensus on ... Continue reading

Comments on this article Comments (0)

Version 2
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Comment
Alongside their report, reviewers assign a status to the article:
Approved - the paper is scientifically sound in its current form and only minor, if any, improvements are suggested
Approved with reservations - A number of small changes, sometimes more significant revisions are required to address specific details and improve the papers academic merit.
Not approved - fundamental flaws in the paper seriously undermine the findings and conclusions
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