Disseminated Hydatid Cyst Disease Presenting as Acute ... - MedIND

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Disseminated Hydatid Cyst Disease Presenting as Acute Respiratory Distress MK Sundarka*, R Bansal*, V Talwar*, R Jindal**, M Saini**, HL Gupta***

Case report

was unremarkable except for tachycardia.

A 30-year-old woman presented in the medical emergency of Smt. Sucheta Kripalani hospital, New Delhi with acute breathlessness and dry cough of one hour duration without any chest pain. There was no history suggestive of bronchial asthma, drug intake, allergy, or chest trauma. Physical examination revealed tachypnoea (RR-48/ min.), tachycardia (110/min), and a blood pressure of 110/70 mm of Hg. There was no cyanosis and her temperature was 370C. Chest examination revealed diminished air entry, polyphonic wheeze and bibasilar fine endinspiratory crepts. Cardiovascular examination

Emergency biochemical investigations revealed a haemoglobin of 12g%, TLC of 14,700 cells/uL, (P80, L15, M4, E1, B0). ABG showed hypoxia with type I respiratory failure. Electrocardiogram showed sinus tachycardia. A provisional diagnosis of non-cardiogenic pulmonary oedema was made and management started on the same lines. Emergency chest radiograph (Fig. 1) showed a round partially filled cystic opacity of >4 cm. diameter in right lower zone. Emergency abdominal ultrasound (Fig. 2) was suggestive of multiple thin walled cysts in the liver. A possibility of disseminated hydatid disease was considered

Fig. 1 : Hydatid cyst (water-lilly appearance) in chest–X-ray.

* Senior Resident, ** Postgraduate Resident, *** Director, Professor and Head, Department of Medicine, Lady Hardinge Medical College & Associated Smt. Sucheta Kripalani Hospital, New Delhi 110001.

Fig. 2 : Ultrasonographic picture of hydatid cysts (Liver).

and further evaluation was done to confirm it.Serology for hydatid cyst disease was significantly positive by ELISA. Her clinical presentation most likely was due to rupture of hydatid cyst in the lung.Her condition markedly improved after the inhaled salbutamol and intravenous hydrocortisone sodium therapy. Patient was started on oral albendazole therapy (15 mg/kg/day) for 4 weeks. Another course of albendazole was administered after an interval of three weeks. Follow up of this patient revealed diminution in the size of cysts , both on X-ray chest PA view and on ultrasound examination of abdomen. Symptomatically also there was marked improvement. Liver function tests and other biochemical parameters were normal.

Discussion Q. What is hydatid cyst disease? A. Hydatid cyst disease is a zoonotic disease caused by the larval stage of Echinococcus granulosus (dog tapeworm), E. multilocularis, or E. vogeli. This disease occurs when humans ingest the hexacanth embryos of the dog tape worm. It is endemic in cattle-and sheep-raising regions of the world such as Central Europe, the Mediterranean countries, the Middle East, South America, Australia, New Zealand, and South 1,2,3 Africa . Hydatid disease is prevalent in the Northern part of our country especially in sheep grazing areas. E. granulosus being the most common parasite in our settings. Q. What are the clinical features of echinococcosis? A. The clinical presentation of hydatid disease is often non-specific and many patients may be asymptomatic. The symptoms depend on the size and site of the lesion and the accessibility of the organ involved for clinical examination. It can affect any organ of body including liver (60%), lungs (20%), kidneys (3%), brain (1%) and rarely heart, ureter, spleen, uterus, fallopian tube,

Journal, Indian Academy of Clinical Medicine

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mesentery, pancreas, diaphragm, and muscles . Cardiac involvement with echinococcosis is uncommon (0.02%-2%). Infestation by 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 5 about 5-13% of cases . In our case lung and liver involvement has been demonstrated simultaneously. Slowly enlarging echinococcal cysts generally remain asymptomatic until their expanding size or their space occupying effect in an involved organ elicits symptoms. Liver and lung are the most common sites. Five to twenty years may elapse before cysts enlarge sufficiently to cause symptoms and they may be discovered as an incidental finding on a routine x-ray or ultrasound study. Patients with hepatic echinococcosis, who are symptomatic, most often present with abdominal pain or a palpable mass in the right upper quadrant. Compression of a bile duct or leakage of cyst fluid into the biliary tree may mimic recurrent cholelithiasis, and biliary obstruction can result in jaundice (seen in 15% cases). Rupture or episodic leakage from a hydatid cyst may produce fever, pruritis, urticaria, eosinophilia, or fatal anaphylaxis. Pulmonary hydatid cyst may rupture into the bronchial tree or peritoneal cavity and produce cough, chest pain, or haemoptysis. Cysts may involve any organ, and other presentations are due to those in bone (invasion of the medullary cavity with slow bone erosion producing pathologic fractures), the central nervous system (space occupying lesions) and the heart (conduction 6 defects, pericarditis) . Cystic echinococcosis in humans is one of the three most impartant zoonotic diseases next only to rabies and brucellosis. Q. Describe the life cycle and mode of transmission of dog tape worm. A. Hydatid disease is caused by the larvae of a flat tapeworm, Echinococcus granulosus, in humans. The life cycle of this parasite exists between carnivores and herbivores, like dogs and

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sheep; man is an accidental intermediate host and an end-point in the parasite’s life cycle. Disease frequency in man depends on the presence of a definitive host, such as a dog in his environment. The mature worm has a head and three proglottids, measuring less than 1 cm in length, and inhabits the intestines of carnivorous animals, especially dogs. The last proglottid contains about 500 eggs, passing with animal stool when the gravid proglottid is ruptured. Sometimes the proglottid detaches completely from the worm. Herbivorous animals like sheep and cows become infested by eating contaminated grass. Contaminated vegetables are the culprit for human infestations. Larvae are released from eggs in the gastrointestinal tract of man and other intermediate hosts, passing through the intestinal wall, and reaching the portal vein. Thus, the liver is the first and most common site of the disease. Some larvae may even pass into the lungs, reach the left side of the heart and the systemic circulation, and then they may lodge in any tissue except hair, nails and teeth. Q. What are the diagnostic modalities? A. Radiological and related studies are important in detecting and evaluating echinococcal cysts. Plain films will define pulmonary cysts, usually as rounded, irregular masses of uniform density, but may miss cysts in other organs unless there is cyst wall calcification, as occurs in the liver. Ultrasonography, echocardiography and magnetic resonance imaging (MRI) are of great value in diagnosing and determining the anatomic extent and relationship of the cyst. Eosinophilia in peripheral blood is seen only in 30% of patients. Specific diagnosis could be made by examination of the aspirated fluids for the presence of scoliceal hooklets, but diagnostic aspiration is not conventionally recommended because of the risks from fluid leakage. However, CT guided aspiration of hydatid cyst for diagnosis has been utilised successfully in some centres in

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conjunction with instillation of ethanol after aspiration. Different serological tests are being carried out for the diagnosis, screening and post-operative follow up for recurrence. These include the hydatid immunoelectrophoresis, enzyme-linked immunosorbent assay (ELISA), latex agglutination 6 and indirect haemagglutination (IHA) test . Q. What is the role of serology in diagnosis? A. Sero-diagnostic assays can be useful, although a negative test does not exclude the diagnosis of echinococcosis. While cysts in the liver are more likely to elicit positive antibody responses than those in the lungs, up to 50% of infected individuals may have negative serology. Detection of antibody to a specific echinococcal antigen [Antigen 5 or arc 5] has the highest degree of specificity, although false positive findings may be present in 6 cysticercosis . Q. Describe key points related to Casoni’s test. A. The Casoni’s test is an immediate hypersensitivity test originally introduced by Casoni in 1911. The antigen is hydatid fluid collected from animal or human cysts and sterilised by Seitz or membrane filtration. 0.2 ml of the antigen is injected intradermally on one arm and an equal volume of saline as control on the other arm. In positive cases a large wheal, about 5 cm in diameter, with multiple pseudopodial projections appears within 20-30 minutes at the test side and fades in an hour. The test is very sensitive, but false positive reactions may appear in a number 7 of other conditions . The test itself can sensitize the patient or result in anaphylaxis in an already sensitised patient and is no longer recommended 8 as a diagnostic procedure . Q. What is the treatment of hydatid cyst disease ? A. Therapy for echinococcosis is based on considerations of the size, location and manifestations of cysts and the overall health of


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the patient. The treatment of hydatid cysts is principally surgical. Surgery can be performed with removal of the cyst intact if possible after first sterilising the cyst with formalin or alcohol. However, pre- and post-operative 1-month courses of albendazole or 2 weeks of praziquantel should be considered in order to sterilise the cyst, decrease the chance of anaphylaxis, decrease the tension in the cyst wall (thus reducing the risk of spillage during surgery) and to reduce the 6 recurrence rate post-operative . Intra-operatively, the use of hypertonic saline or 0.5% silver nitrate solutions before opening the cavities tends to kill the daughter cysts and therefore prevent further spread or anaphylactic reaction. Medical treatment (e. g., with albendazole) can result in reduction of the cyst size. As medical therapy, albendazole, given at 400 mg twice a day for 28 days and repeated from 1 to 8 times, separated by the drug free interval of 2 to 3 weeks, is most efficacious for those with hepatic and/or pulmonary cysts. Chronic calcified cysts can be 6 left . Preventive measures are important and include washing of hands with soap and warm water, washing of fruits and vegetables before consumption, and de-worming of pet dogs. In endemic areas infected dogs can be treated by praziquantel treatment. Limitation of the number


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of stray dogs is helpful in reducing the prevalence 6 of human infection . The prognosis without any complication is good 4 although there is always the risk of rupture .

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Shields R, Johnson AG. The Liver. In : Bailey H, Love M, (editors). Bailey and Love’s Short Practice of Surgery :19th ed. London: HK Lewis & Co, Ltd, 1984; 853-6.

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Saidi F. Surgery of Hydatid Disease. 1st ed. London: WB Saunders Company, Ltd., 1976: 22-5.

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Schwartz SI. Liver. In: Schwartz SI, Shire GT, Spencer FC, (editors). Principles of Surgery, 6th ed. New York : McGraw-Hill, Inc, 1994; 1332-4.

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Farthing MJG, Jeffries DJ, Anderson J. Infectious diseases, tropical medicine and sexually transmitted diseases In Clinical Medicine , Kumar P, Clark M, (editors). 4th edition: WB Saunders 1998; 96-7.

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Saidi F. Treatment of Echinococcal Cysts In : Nyhus LM, Baker RJ, Sabiston DC, (editors). Mastery of Surgery. 2nd ed. London: Little, Brown and Company, 1992; 81837.

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Thomas B Nutman, Peter F Weller. Cestodes In : Isselbacher Kurt J, Braunwald E, Wilson Jean D, Martin Joseeph B et al, (editors). Harrison’s Principles of Internal Medicine Vol 1, 13th edition : McGraw -Hill, Inc, 1994; 931-3.

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Paniker Jayaram CK. Textbook of Parasitology, 2nd edition : Jaypee Brothers, 1991; 135-40.

8.

Mazumdar A, Singh Gurpreet, Gupta NM. Primary splenic hydatids in adults. Tropical Gastroenterology 2000; XXI (2): www. tropical gastroenterology.com.


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