Hydatid cyst presenting as a quinsy* By
AHMED BASSYOUNI
(Cairo)t and
AMR MAHER
(Alexandria)!
Introduction HYDATID disease has a world-wide distribution but is most prevalent in sheeparid cattle-raising countries such as Australia, New Zealand, South America and Central Europe. The disease is also relatively common in the Middle East; in Iran, it comprises 1 per cent of admissions to the surgical wards (Lofti and Hashemian, 1973). Hydatidosis is a disease which often presents with diagnostic difficulties and sometimes a threat to life. It is primarily a surgical disease as there is no suitable medical treatment to date. Amir-Jahed et al. (1975) found the mortality in cases not treated by surgery to be about 60 per cent and in surgically treated cases to be less than 4 per cent. To our knowledge, from a search of the available literature, this is the first • case to be reported in O.R.L. practice. A discussion of the salient features of the diagnosis and treatment is presented to acquaint the otolaryngologist with this potentially fatal condition.
Parasitology
In hydatid disease, man is usually infected by the accidental ingestion of the fertile ova of the minute tapeworm Echinococcus granulosus, passed in millions within the faeces of dogs, wolves and foxes which act as its definitive hosts. When the eggs are swallowed by the intermediate host such as man, sheep, cattle or pigs, they hatch and the embryo passes through the intestinal wall and enters the portal circulation where most are trapped in the liver but some pass to the lungs and rarely to the heart, kidney, spleen, bones, brain, orbit, salivary glands and tongue. In these sites, the embryo transforms into a cyst which develops the germinal epithelium that produces brood capsules, larval forms (proscolices) and eventually the scolices. The life cycle is completed when dogs consume infected viscera of the intermediate host, usually the liver or lungs. Case report
S.A., a 30-year-old Jordanian housewife, presented in the E.N.T. out-patient clinic with a slight impairment of speech, discomfort in the throat and dysphagia of two weeks' duration, with no improvement on taking antibiotics. She gave a history of having had a right quinsy the previous year which was relieved by incision. Three months later, she developed left empyema which was treated by repeated aspirations and followed later by a left lower lobe lobectomy in Hadassa Hospital. * This work has been done in Dr. Fakhry Hospital, AlKhobar, Kingdom of Saudi Arabia. t Assistant Professor, E.N.T., Cairo University. + Assistant Professor, E.N.T., Alexandria University. 729
730
A. BASSYOUNI AND A. MAHER
Clinical examination revealed a firm rounded swelling, one inch in diameter, in the right side of the soft palate above and lateral to the tonsil with slight congestion and oedema of the intact covering mucosa. A preliminary diagnosis of quinsy or mixed salivary tumour was made. The routine blood picture showed a leucocytosis with a 5 per cent eosinophilia. On introducing a spinal puncture needle fitted onto a syringe, 20 ml of yellow pus gushed out and the swelling collapsed. A culture and specific antibiogram of the aspirated fluid showed coagulase-positive staphylococci, sensitive to Garamycin. Four days later, the 'abscess' had reformed and on this occasion was incised under surface anaesthesia only to recur after two days to reach its previous size. The patient was admitted to hospital for an abscess tonsillectomy. The routine pre-operative investigations were normal and the eosinophilic count remained at 5 per cent. A straight chest X-ray showed a slight left basal pleural veiling. At operation, about 15 ml of pus were first aspirated and then the thick cyst wall was stripped away with surprising ease in four pieces. The histopathology report stated: 'a cyst wall lined by granulation tissue heavily permeated with neutrophils and eosinophils,' After an uneventful post-operative period of 16 days, the patient returned complaining of a sense of suffocation and with continuous gagging. When the mouth was opened for inspection, a cyst was seen emerging through the operation site (Fig. 1). It was two inches in diameter and closely resembled a shelled hard-boiled egg (Fig. 2). The wound was first painted with formalin solution and then the patient was made to gargle with hydrogen peroxide. The clinical diagnosis of hydatid disease was now certain. Further interroga*
FIG. 1 Hydatid cyst emerging from the operation site.
:-••'#",-
CLINICAL RECORDS
731
FIG. 2 Complete hydatid cyst, after expulsion.
tion of the patient showed her to be in the habit of eating spiced raw livers of home-slaughtered sheep 'after removing the white patches in them'. She denied keeping pet dogs but mentioned casually that her neighbours did. The latest histopathology report was consistent with that of an Echinococcus cyst containing many acute inflammatory cells, but no scolices. Further X-rays of the chest showed a small round homogeneous opacity with well-defined borders in the left lung (Fig. 3). A radiological survey of the body failed to show any other cysts. Diagnosis Hydatid cysts are liable to be overlooked in the differential diagnosis of space-occupying lesions in endemic areas (Nourmand, 1976). They may remain silent for up to 40 years until adjacent structures are compressed producing pain or organ system dysfunction, lntracystic infection or haemorrhage may produce a sudden exacerbation of symptoms (Lewis et al., 1975; Chandra and Prakash, 1965). Rupture of the cyst which is filled with highly antigenic fluid may result in an anaphylactic reaction (Lewis et al., 1975). In O.R.L. practice hydatid disease is rarely encountered even in endemic areas. Hydatid cysts in the thyroid may present with symptoms of hoarseness, stridor or dysphagia due to pressure on the recurrent laryngeal nerve, trachea or oesophagus (Skalkeas and Sechas, 1967; Chandra and Prakash, 1965). A hydatid cyst of the temporal bone was reported by Fenu (1968), two cases in the tongue by Perl et al. (1972) and Gracanin (1963), and one in the orbit presenting with proptosis (Tonjum, 1963).
732
A. BASSYOUNI AND A. MAHER
FIG.
3a
Laboratory investigations
Eosinophilia is suggestive of hydatid disease but non-specific (Lewis et al., 1975). The Casoni intradermal test is now considered obsolete (Shantz et al., 1975). Serological tests are important but fallible. The immuno-electrophoresis test introduced by Capron et al. (1967) is the only test that is absolutely specific for hydatid disease. Chest X-ray patterns, distinctive liver scintillation scan appearances and angiographic abnormalities have been reported as helpful. Far more important than any test, however, is an awareness that the condition may arise in any individual from an endemic area. Treatment
No effective medical treatment exists (Lewis et al., 1975). A broad spectrum antihclmintic, Mebendazole, kills hydatids in mice but not in man (Heath and Chevis, 1974). The principle of surgical treatment is the total excision of the cyst; cryosurgery may be helpful especially in large cysts (Amir-Jahed et al., 1975). Extensive packing of the operative field is essential to avoid seeding and anaphylactic shock. Scolicidal agents, such as hypertonic saline, absolute alcohol, hydrogen peroxide
CLINICAL RECORDS
FIG.
733
3b
FIG. 3 X-ray chest (ij Postero-amerior view and (ii) Lateral view, showing dense round homogeneous opacity in the left lung.
or formalin, may be injected into the cyst and retained for 5-10 minutes prior to removal. The anaesthetist must anticipate the possibility of anaphylactic shock (Lewis et a!., 1975). Discussion In this case, contact with dogs is the most likely route of infestation. An alternative possibility is the direct invasion of a tonsillar crypt by a larval protoscolex during ingestion of raw infected liver. In hydatid cysts that rupture during surgical removal, seeding of the contents is known to occur. Recently, Beard (1976), a member of the Australian Hydatid Control Council postulated that 'since the Lebanese eat raw liver, a hydatid of the tonsil might arise from the implantation of a protoscolex in a tonsillar crypt'. This may be compared to the escape of liver flukes during mastication of raw liver introducing nasopharyngeai fascioliasis (Schwabe, 1976). Primary tonsillar hydatidosis is supported by the fact that the human intestinal digestive juices contain bile which destroys the larval scolices (Smyth, 1962),
734
A. BASSYOUNI AND A. MAHER
whereas the tonsil lacks any digestive or lytic properties. The natural liability of dogs to the disease is explained by the fact that the Intestinal juices of the dog do not affect the larval scolices. The theory of direct implantation is supported by the case of a laboratory worker who had a small, histologically confirmed hydatid cyst removed from the conjunctival sac a few months after a cyst had burst in his face (Bates, 1976). The radiological picture and previous thoracotomy are highly suggestive of pulmonary hydatidosis. Amir-Jahed et ah (1975) reported a definite diagnostic pattern in 91-5 per cent of plain chest X-rays. If the concept of primary hydatidosis of the peritonsillar region stands, then the only logic correlation between it and the pulmonary hydatid cyst, in our case, is the inhalation of hydatid sand during incision of the first quinsy, one year earlier. An alternative explanation, however, is the development of multiple hydatid infestation by ingestion of fertile ova. REFERENCES AMIR-JAHED, A. K.. FARDIN, R., FARZAD, A., and BAKSHANDLH, K. (1975) Annals of Surgery,
182,541. BATES, S. E. M. (1976) Quoted from Beard, T. C. (1976) Lancet, 2, 811. Personal communication. BEARD, T, C. (1976) Lancet, 2, 811. CAPRON, A., VERNES, A., and BIGUET, J. (1967) In 'Le Kyste Hydatique du Foie', pp. 27-40, Journees Lyonnaises d'Hydatidologie. Simep., ed. Lyort. CHANDRA, T., and PRAKASH, A. (1965) British Journal of Surgery, 52, 235. FENU, G. (1968) Clinka Oto-Rino-Laringaiatrica (Roma), 20, 125. GRACANIN, S. (1963) Journal of Laryngology and Otology, 11, 624. HEATH, D. D., and CHEVIS, R. A. F. (1974) Lancet, 2, 218.
LEWIS, J. W., Koss, N., and KERSTEIN, M. D. (1975) Annals of Surgery, 181, 390. LOFTI, M , and HASHEMIAN, H. (1973) International Surgery, 58, 166. NOURMAND, A. (1976) American Journal of Tropical Medicine and Hygiene, 25, 845. PERL, P., PERL, T., and GOLDBERG, B. (1972) Oral Surgery, Oral Medicine and Oral Pathology, 33, 579. SKALKEAS, G. D., and SECHAS, M. N. (1967) Journal of the American Medical Association, 200, 188. SCHWABE, C. W. (1976) Quoted from Beard, T. C. (1976) Lancet, 2, 811. SHANTZ, P. M., ORTIZ-VALQUI, R. E., and LUMBRERAS, H. (1975) American Journal of Tropical Medicine and Hygiene, 24, 849. SMYTH, J. D. (1962) Quoted from Yamashita, J. (1968) Bulletin of the World Health Organisation (Geneva), 39, 121. TONJUM, A. M. (1963) Ada Ophthalmologica (Kobenhavn), 41, 445.
AHMED BASSYOUNI
(Cairo)t and
AMR MAHER
(Alexandria)!
Introduction HYDATID disease has a world-wide distribution but is most prevalent in sheeparid cattle-raising countries such as Australia, New Zealand, South America and Central Europe. The disease is also relatively common in the Middle East; in Iran, it comprises 1 per cent of admissions to the surgical wards (Lofti and Hashemian, 1973). Hydatidosis is a disease which often presents with diagnostic difficulties and sometimes a threat to life. It is primarily a surgical disease as there is no suitable medical treatment to date. Amir-Jahed et al. (1975) found the mortality in cases not treated by surgery to be about 60 per cent and in surgically treated cases to be less than 4 per cent. To our knowledge, from a search of the available literature, this is the first • case to be reported in O.R.L. practice. A discussion of the salient features of the diagnosis and treatment is presented to acquaint the otolaryngologist with this potentially fatal condition.
Parasitology
In hydatid disease, man is usually infected by the accidental ingestion of the fertile ova of the minute tapeworm Echinococcus granulosus, passed in millions within the faeces of dogs, wolves and foxes which act as its definitive hosts. When the eggs are swallowed by the intermediate host such as man, sheep, cattle or pigs, they hatch and the embryo passes through the intestinal wall and enters the portal circulation where most are trapped in the liver but some pass to the lungs and rarely to the heart, kidney, spleen, bones, brain, orbit, salivary glands and tongue. In these sites, the embryo transforms into a cyst which develops the germinal epithelium that produces brood capsules, larval forms (proscolices) and eventually the scolices. The life cycle is completed when dogs consume infected viscera of the intermediate host, usually the liver or lungs. Case report
S.A., a 30-year-old Jordanian housewife, presented in the E.N.T. out-patient clinic with a slight impairment of speech, discomfort in the throat and dysphagia of two weeks' duration, with no improvement on taking antibiotics. She gave a history of having had a right quinsy the previous year which was relieved by incision. Three months later, she developed left empyema which was treated by repeated aspirations and followed later by a left lower lobe lobectomy in Hadassa Hospital. * This work has been done in Dr. Fakhry Hospital, AlKhobar, Kingdom of Saudi Arabia. t Assistant Professor, E.N.T., Cairo University. + Assistant Professor, E.N.T., Alexandria University. 729
730
A. BASSYOUNI AND A. MAHER
Clinical examination revealed a firm rounded swelling, one inch in diameter, in the right side of the soft palate above and lateral to the tonsil with slight congestion and oedema of the intact covering mucosa. A preliminary diagnosis of quinsy or mixed salivary tumour was made. The routine blood picture showed a leucocytosis with a 5 per cent eosinophilia. On introducing a spinal puncture needle fitted onto a syringe, 20 ml of yellow pus gushed out and the swelling collapsed. A culture and specific antibiogram of the aspirated fluid showed coagulase-positive staphylococci, sensitive to Garamycin. Four days later, the 'abscess' had reformed and on this occasion was incised under surface anaesthesia only to recur after two days to reach its previous size. The patient was admitted to hospital for an abscess tonsillectomy. The routine pre-operative investigations were normal and the eosinophilic count remained at 5 per cent. A straight chest X-ray showed a slight left basal pleural veiling. At operation, about 15 ml of pus were first aspirated and then the thick cyst wall was stripped away with surprising ease in four pieces. The histopathology report stated: 'a cyst wall lined by granulation tissue heavily permeated with neutrophils and eosinophils,' After an uneventful post-operative period of 16 days, the patient returned complaining of a sense of suffocation and with continuous gagging. When the mouth was opened for inspection, a cyst was seen emerging through the operation site (Fig. 1). It was two inches in diameter and closely resembled a shelled hard-boiled egg (Fig. 2). The wound was first painted with formalin solution and then the patient was made to gargle with hydrogen peroxide. The clinical diagnosis of hydatid disease was now certain. Further interroga*
FIG. 1 Hydatid cyst emerging from the operation site.
:-••'#",-
CLINICAL RECORDS
731
FIG. 2 Complete hydatid cyst, after expulsion.
tion of the patient showed her to be in the habit of eating spiced raw livers of home-slaughtered sheep 'after removing the white patches in them'. She denied keeping pet dogs but mentioned casually that her neighbours did. The latest histopathology report was consistent with that of an Echinococcus cyst containing many acute inflammatory cells, but no scolices. Further X-rays of the chest showed a small round homogeneous opacity with well-defined borders in the left lung (Fig. 3). A radiological survey of the body failed to show any other cysts. Diagnosis Hydatid cysts are liable to be overlooked in the differential diagnosis of space-occupying lesions in endemic areas (Nourmand, 1976). They may remain silent for up to 40 years until adjacent structures are compressed producing pain or organ system dysfunction, lntracystic infection or haemorrhage may produce a sudden exacerbation of symptoms (Lewis et al., 1975; Chandra and Prakash, 1965). Rupture of the cyst which is filled with highly antigenic fluid may result in an anaphylactic reaction (Lewis et al., 1975). In O.R.L. practice hydatid disease is rarely encountered even in endemic areas. Hydatid cysts in the thyroid may present with symptoms of hoarseness, stridor or dysphagia due to pressure on the recurrent laryngeal nerve, trachea or oesophagus (Skalkeas and Sechas, 1967; Chandra and Prakash, 1965). A hydatid cyst of the temporal bone was reported by Fenu (1968), two cases in the tongue by Perl et al. (1972) and Gracanin (1963), and one in the orbit presenting with proptosis (Tonjum, 1963).
732
A. BASSYOUNI AND A. MAHER
FIG.
3a
Laboratory investigations
Eosinophilia is suggestive of hydatid disease but non-specific (Lewis et al., 1975). The Casoni intradermal test is now considered obsolete (Shantz et al., 1975). Serological tests are important but fallible. The immuno-electrophoresis test introduced by Capron et al. (1967) is the only test that is absolutely specific for hydatid disease. Chest X-ray patterns, distinctive liver scintillation scan appearances and angiographic abnormalities have been reported as helpful. Far more important than any test, however, is an awareness that the condition may arise in any individual from an endemic area. Treatment
No effective medical treatment exists (Lewis et al., 1975). A broad spectrum antihclmintic, Mebendazole, kills hydatids in mice but not in man (Heath and Chevis, 1974). The principle of surgical treatment is the total excision of the cyst; cryosurgery may be helpful especially in large cysts (Amir-Jahed et al., 1975). Extensive packing of the operative field is essential to avoid seeding and anaphylactic shock. Scolicidal agents, such as hypertonic saline, absolute alcohol, hydrogen peroxide
CLINICAL RECORDS
FIG.
733
3b
FIG. 3 X-ray chest (ij Postero-amerior view and (ii) Lateral view, showing dense round homogeneous opacity in the left lung.
or formalin, may be injected into the cyst and retained for 5-10 minutes prior to removal. The anaesthetist must anticipate the possibility of anaphylactic shock (Lewis et a!., 1975). Discussion In this case, contact with dogs is the most likely route of infestation. An alternative possibility is the direct invasion of a tonsillar crypt by a larval protoscolex during ingestion of raw infected liver. In hydatid cysts that rupture during surgical removal, seeding of the contents is known to occur. Recently, Beard (1976), a member of the Australian Hydatid Control Council postulated that 'since the Lebanese eat raw liver, a hydatid of the tonsil might arise from the implantation of a protoscolex in a tonsillar crypt'. This may be compared to the escape of liver flukes during mastication of raw liver introducing nasopharyngeai fascioliasis (Schwabe, 1976). Primary tonsillar hydatidosis is supported by the fact that the human intestinal digestive juices contain bile which destroys the larval scolices (Smyth, 1962),
734
A. BASSYOUNI AND A. MAHER
whereas the tonsil lacks any digestive or lytic properties. The natural liability of dogs to the disease is explained by the fact that the Intestinal juices of the dog do not affect the larval scolices. The theory of direct implantation is supported by the case of a laboratory worker who had a small, histologically confirmed hydatid cyst removed from the conjunctival sac a few months after a cyst had burst in his face (Bates, 1976). The radiological picture and previous thoracotomy are highly suggestive of pulmonary hydatidosis. Amir-Jahed et ah (1975) reported a definite diagnostic pattern in 91-5 per cent of plain chest X-rays. If the concept of primary hydatidosis of the peritonsillar region stands, then the only logic correlation between it and the pulmonary hydatid cyst, in our case, is the inhalation of hydatid sand during incision of the first quinsy, one year earlier. An alternative explanation, however, is the development of multiple hydatid infestation by ingestion of fertile ova. REFERENCES AMIR-JAHED, A. K.. FARDIN, R., FARZAD, A., and BAKSHANDLH, K. (1975) Annals of Surgery,
182,541. BATES, S. E. M. (1976) Quoted from Beard, T. C. (1976) Lancet, 2, 811. Personal communication. BEARD, T, C. (1976) Lancet, 2, 811. CAPRON, A., VERNES, A., and BIGUET, J. (1967) In 'Le Kyste Hydatique du Foie', pp. 27-40, Journees Lyonnaises d'Hydatidologie. Simep., ed. Lyort. CHANDRA, T., and PRAKASH, A. (1965) British Journal of Surgery, 52, 235. FENU, G. (1968) Clinka Oto-Rino-Laringaiatrica (Roma), 20, 125. GRACANIN, S. (1963) Journal of Laryngology and Otology, 11, 624. HEATH, D. D., and CHEVIS, R. A. F. (1974) Lancet, 2, 218.
LEWIS, J. W., Koss, N., and KERSTEIN, M. D. (1975) Annals of Surgery, 181, 390. LOFTI, M , and HASHEMIAN, H. (1973) International Surgery, 58, 166. NOURMAND, A. (1976) American Journal of Tropical Medicine and Hygiene, 25, 845. PERL, P., PERL, T., and GOLDBERG, B. (1972) Oral Surgery, Oral Medicine and Oral Pathology, 33, 579. SKALKEAS, G. D., and SECHAS, M. N. (1967) Journal of the American Medical Association, 200, 188. SCHWABE, C. W. (1976) Quoted from Beard, T. C. (1976) Lancet, 2, 811. SHANTZ, P. M., ORTIZ-VALQUI, R. E., and LUMBRERAS, H. (1975) American Journal of Tropical Medicine and Hygiene, 24, 849. SMYTH, J. D. (1962) Quoted from Yamashita, J. (1968) Bulletin of the World Health Organisation (Geneva), 39, 121. TONJUM, A. M. (1963) Ada Ophthalmologica (Kobenhavn), 41, 445.
