Published 29 December 2008, doi:10.1136/bmj.a3090
Cite this as: BMJ 2008;337:a3090

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Picture quiz

A case of hepatomegaly

¹ Basildon University Hospital, Basildon SS16 5NL

A 27 year old man from Turkey presented with sudden onset epigastric pain that woke him from sleep. He had no other symptoms. On examination he had right upper quadrant abdominal tenderness and a palpable hepatomegaly of 10 cm below the right subcostal margin. Blood results showed a white cell count of 12.6x10⁹ cells/l and C reactive protein of 366 µg/ml, and liver function was normal.

Questions

1 Describe the abnormalities in the figures

2 What is the likely diagnosis?

3 How would you establish the diagnosis?

4 What is the treatment?

View larger version (45K): [in this window] [in a new window] [PowerPoint Slide for Teaching]   Fig 1 Ultrasound scan (top) and computed tomograms

 

Short answers

1 The ultrasound scan of the liver (top), shows a multicystic, multiloculated mass of mixed echogenicity. The abdominal computed tomography scans, show a large well defined mass within the liver; from the evidence of the ultrasound scan, this mass is cystic.

View larger version (46K): [in this window] [in a new window] [PowerPoint Slide for Teaching]   Fig 2 Ultrasound scan of the liver shows a multicystic, multiloculated mass of mixed echogenicity (top). Below, the coronal and sagittal views of an abdominal CT scan show a large well defined mass within the liver

 

2 Hydatid liver disease.

3 Enzyme linked immunosorbent assay (ELISA) to check for antibodies to hydatid antigen in the serum.¹

4 Prevention of cyst rupture and surgical removal of cysts.

Long answers
Diagnosis
Hydatid liver disease results from infection by tapeworm larvae of the genus Echinococcus. There are four main types: for E granulosus the definitive host is the dog: for E multilocularis the definitive host is the fox; for E vogeli the definitive host is the bush dog (found commonly in South and Central America);² and for E oligarthrus felids (carnivorous mammals, including the domesticated cat and big cats such as lions, tigers, panthers, lynxes, leopards, pumas, and cheetahs) are the definitive hosts.³

As well as the definitive hosts, the life cycle of tapeworm requires intermediate hosts: humans, sheep, goats, swine, cattle, horses, camels, and rodents. The adult tapeworm (E granulosus) infects the intestinal tract of the definitive host; it produces eggs, which are expelled within the host’s faeces. The intermediate host ingests the cyst-containing organs of the infected host. The eggs hatch, and the tiny embryos travel in the bloodstream and lodge in organs such as the brain, liver, spleen, lungs, and kidneys; these form hydatid cysts.⁴

Hydatid disease is found mainly in sheep farming areas, notably in Greece and Turkey, and 10-20 cases are reported in the UK each year. Prevention is targeted at deworming dogs that might be carrying this parasite, as well as good hygiene after contact with dogs and sheep.

Treatment
The mainstay of treatment is prevention of cyst rupture, which could cause anaphylaxis. This is done with albendazole to reduce the size of the cyst. Albendazole acts to reduce glucose uptake by the tapeworm microtubules and causes the tapeworm to die. Praziquantil reduces contamination of the peritoneal cavity by increasing permeability of the parasite cells to calcium, thereby inducing contraction and paralysis of the parasite cells.⁵ This initial medical management sterilises the cyst, reduces the cyst wall tension, which is important intraoperatively, and reduces recurrence.⁶ Subsequent surgical management involves removal of the cysts without rupturing or puncturing them, guided by computed tomography; aspiration of fluid; and injection of hypertonic saline solution as a scolicidal agent.⁴ In cases of recurrent cysts, liver resection may be needed.⁷

Cite this as: BMJ 2008;337:a3090

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Competing interests: None declared.

Provenance and peer review: Not commissioned; externally peer reviewed.

Patient consent obtained.

References

1. Sayek I, Onat D. Diagnosis and treatment of uncomplicated hydatid cyst of the liver. World J Surg 2001;25:21-7.[CrossRef][Web of Science][Medline]
2. D’Alessandro A, Rausch RL, Cuello C, Aristizabal N. Echinococcus vogeli in man, with a review of polycystic hydatid disease in Colombia and neighboring countries. Am J Trop Med Hyg 1979;28:303-17.[Abstract/Free Full Text]
3. D’Alessandro A, Ramirez LE, Chapadeiro E, Lopes ER, Mesquita PM. Second recorded case of human infection by Echinococcus oligarthrus. Am J Trop Med Hyg 1995;15:29-33. Khanfar N. Hydatid disease: a review and update. Curr Anaesth Crit Care 2004;15:173-83.
4. Peláez V, Kugler C, Correa D, Del Carpio M, Guangiroli M, Molina J, et al. PAIR as percutaneous treatment of hydatid liver cysts. Acta Trop 2000;75:197-202.[CrossRef][Web of Science][Medline]
5. Greenberg RM. Molecular target of the antischistosomal drug praziquantel. Future Microbiol 2007;2:265-8.[CrossRef][Web of Science]
6. Abu-eshy SA. Some rare presentations of hydatid disease (Echinococcus granulosus). J R Coll Edin Surg 1998;43:347-52.
7. Ayles HN, Corbett EL, Taylor I, Cowie AG, Bligh J, Walmsley K, et al. A combined medical and surgical approach to hydatid disease: 12 years experience at the Hospital for Tropical Diseases, London. Ann R Coll Surg Eng 2002;84:100-5.[Web of Science][Medline]

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