|Year : 2015 | Volume
| Issue : 1 | Page : 65-67
Disseminated hydatid disease presenting as fever of unknown origin: A case report and review of literature
Nikhil Gupta1, Vishnu Vasudevan1, Sarthak Malik1, Nikhil Nair2
1 Department of Medicine, UCMS, Dilshad Garden, New Delhi, India
2 Department of Radio-Diagnosis, LHMC, New Delhi, India
|Date of Web Publication||8-Jan-2015|
UCMS, Dilshad Garden, New Delhi - 110 095
Source of Support: None, Conflict of Interest: None
Human hydatid disease occurs due to infection with larval form of Echinococcus granulosus. The disseminated hydatid disease is a very rare finding. Disseminated hydatid disease presenting as a cause of fever of unknown origin is a rare phenomenon. We present to you such a rare case.
Keywords: Disseminated hydatid disease, fever of unknown origin, hydatid cyst
|How to cite this article:|
Gupta N, Vasudevan V, Malik S, Nair N. Disseminated hydatid disease presenting as fever of unknown origin: A case report and review of literature. Med J DY Patil Univ 2015;8:65-7
|How to cite this URL:|
Gupta N, Vasudevan V, Malik S, Nair N. Disseminated hydatid disease presenting as fever of unknown origin: A case report and review of literature. Med J DY Patil Univ [serial online] 2015 [cited 2019 Mar 24];8:65-7. Available from: http://www.mjdrdypu.org/text.asp?2015/8/1/65/148852
| Introduction|| |
Indian subcontinent also has a high prevalence of Echinococcus.  The incidence of Echinococcus infection is 0.4/100,000 persons.  FUOs is deﬁned as disorders with temperatures ≥101°F that have persisted for at least 3 weeks that were not diagnosed after three outpatient visits or 3 days in-hospital without elucidation of a cause or 1 week of intelligent and invasive ambulatory investigation. To the best of our knowledge, disseminated hydatid disease as a cause of fever of unknown origin (FUO) has been rarely described in literature. We present to you such a rare case.
| Case Report|| |
A 30-year-old man presented with a 4 weeks history of high grade fever up to 103°F accompanied by rigors and malaise. Risk factors for brucellosis, endocarditis, tuberculosis, and retrovirus infection were negative. There was no history of night sweats, anorexia and weight loss. There was no significant past or family history. A complete physical examination was normal.
Urine routine and cultures were negative for growth. Full blood count and blood film analysis, blood cultures, kidney and liver functions test, creatine kinase, and lymphocyte subset analysis, as well as tests of autoimmunity were negative. There was also no evidence of antinuclear antibodies, rheumatoid factor, and antineutrophil cytoplasmic antibodies. Serum immunoglobulins were normal, and there was no abnormality on serum immunoelectrophoresis. Serum-angiotensin-converting enzyme, Mantoux for mycobacterial infection, HIV as well as lactate dehydrogenase were also either normal or negative. Axial contrast-enhanced computed tomography (CECT) scan of abdomen revealed an oval well-defined cystic lesion in segments 7 and 8 of liver with heterogeneous contents and enhancing internal septations with few air foci in the wall as shown in [Figure 1]. Axial CECT images of chest showed similar but smaller sized lesions in bilateral lung fields as shown in [Figure 2]. CECT scan of head and fundus examination was normal.
|Figure 1: Contrast-enhanced computed tomography scan of abdomen revealing an oval well-defi ned cystic lesion in segments 7 and 8 of liver with heterogeneous contents and enhancing internal septations with few air foci in the wall|
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|Figure 2: Axial contrast-enhanced computed tomography images of chest showing smaller sized cystic lesions in bilateral lung fields suggestive of hydatid cyst|
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Patient was initially treated empirically with cefixime 400 mg BD, but failure of fever to respond led to a detailed workup of patient. After CECT scan of abdomen and chest, the patient was diagnosed with disseminated hydatid cyst disease. Patient underwent surgery, after which his symptoms disappeared and patient became well.
| Discussion|| |
Hydatid cyst is a disease caused by larval stages of dog tapeworm called Echinococcus. This parasite can occur almost anywhere in the body. It demonstrates a spectrum of imaging features that vary according to growth stage, associated complications, and affected tissue.  Hydatid disease in humans most commonly occurs in the liver (55-70%) followed by the lung (18-35%). The two organs can be affected simultaneously in about 5-13% of cases. 
Petersdorf and Beeson  in 1961 were the ﬁrst to deﬁne FUO in terms of time-based diagnostic criteria. These have since been termed ''classic'' FUOs and may be deﬁned as disorders with temperatures ≥101°F that have persisted for at least 3 weeks that were not diagnosed after a week of intensive in-hospital testing. Now, the definition has been modified to three outpatient visits or 3 days in-hospital without elucidation of a cause or 1 week of intelligent and invasive ambulatory investigation. Infections are an important cause of FUO. According to Petersdorf and Beeson's original report, FUOs were caused by infection in 36% of patients.  To the best of our knowledge, hydatid disease has not been reported as a cause of FUO.
Clinical features of hydatid disease depend on the organ involved. Liver involvement by the cyst may lead to hepatomegaly, jaundice, biliary colic-like symptoms, cholangitis, pancreatitis, liver abscess, portal hypertension, ascites, inferior vena cava compression or thrombosis, Budd-Chiari syndrome, cyst rupture, peritoneal spread, and peritonitis. Right lobe is the most common involved part of liver. Infection in hydatid disease of liver occurs only after rupture of both the pericyst and endocyst (communicating and direct rupture). This allows bacteria to pass easily into the cyst (5-8% of cases).  Thus, infection in a ruptured hydatid cyst is a cause of fever. However, the cysts in our case were intact and thus the chance of getting them infected was negligible.
If the cyst settles in lung then the patient may present with tumor of chest, chest pain, chronic cough, expectoration, dyspnea, pneumothorax, eosinophilic pneumonitis, pleural effusion, parasitic lung embolism, and hemoptysis. Secondary infection of the cyst is commonly seen after rupture.  However, the cysts in our case were intact and thus the chance of them getting infected were negligible.
The diagnosis of hydatid cyst disease is often challenging. Combination of serology and imaging leads to diagnosis. Ultrasonography (USG) is the first line of screening for abdominal hydatid cyst. USG best detects cystic membrane, septa, and hydatid sand. CT scan best demonstrates cyst wall calcification and cyst infection.  Imaging in case of hydatid cyst disease may demonstrate wide variation from cystic lesion to solid masses. "Floating membranes" inside the cavity caused by detachment of endocyst is the most specific sign for hydatid disease.
Eosinophilia is seen in few percentages of patients. Serum IgG antibodies to hydatid cyst fluid derived native or recombinant antigen B unit performed by enzyme-linked immunosorbent assay (ELISA) is the gold standard test for diagnosis. It is highly sensitive test with a sensitivity of 95% though the specificity is only around 61%.  Immunoelectrophoresis, ELISA, latex agglutination and indirect hemagglutination test are serological tests for the diagnosis, screening and postoperative follow-up for recurrence.  Species of Echinococcus can be detected by polymerase chain reaction. CT scan is better than magnetic resonance imaging for diagnosis. "Falling snowflake pattern" of hydatid sand can be seen under real time scanner on changing posture of patient. ,,
Management of hepatic cysts includes albendazole or mebendazole therapy in combination with either surgical resection or the puncture aspiration injection re-aspiration procedure. Larger cysts (diameter >10 cm) should undergo surgical resection. , Our patient had cysts in liver and lung. Similar reports of disseminated hydatid cysts have been published before by Geraci et al. 
| Conclusion|| |
Hydatid cyst is a common infection in our country. It often is under diagnosed. It can present with varied manifestations depending on the organ it involves. It can present as PUO. High index of suspicion is required for diagnosis in such cases.
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[Figure 1], [Figure 2]