A C T A 0 P H T H A L M 0 L O G IC A
69 (1991) 802-804
CASE REPORT
A primary intraocular hydatid cyst Satvet Sinavl, Ali Demirc?, Bahar Sinav3, Fatma t)ge’, Yuksel Sullu’ and Bedri Kandemir4 Ondokuz Mayis University, Department of Ophthalmology’,Department of Radiology2, Department of Biochemistry3,Department of Pathology4, Faculty of Medicine, Samsun,Turkey
Abstract. Ultrasonography and computerized tomography (CT) of the painful and blind right eye of a 13year-old girl showed a cystic mass almost filling the vitreous cavity and narrowing the anterior chamber. The histopathologic diagnosis of the enucleated eye was a n echinococcus cyst. Abdominal ultrasonography, brain CT and chest X-rays gave no abnormal findings when first examined, nor during the follow-up 18 months after surgery. Indirect hemagglutination test was negative at the last control. The case was accepted as a primary hydatid cyst (echinococcosis) of the eyeball. Key words: echinococcosis - hydatid cyst - intraocular tumor - ultrasonography - computerized tomography.
The tapeworm Echinococcus granulosus exists in nature in infected dogs, which house the adult worm in their intestinal tract. Sheep or cattle become the intermediate hosts by ingesting the contaminated dog feces and thereby become the hosts for the gradually enlarging cyst, the hydatid. In humans, the liver (60%)and lungs are the most common sites of hydatid cyst development, but all organs may be affected. The small oncosphere undergoes dramatic changes after implantation, increasing in size to two or three centimeters in a few months. The embryo develops a central cyst, which has an inner germinal layer containing numerous nuclei (Jones 1990). Echinococcus is seen in most sheep and cattleraising areas of the world including Australia, East Africa, South America, Eastern Europe and the Middle East. The relatively benign type E.granulosus is the more common type, but E.alveolaris may be fatal (Williams et al. 1987). 802
Case Report A 13-year-oldgirl was referred to our department hom another eye clinic where she had been followed for three years. In 1986 she had lost the vision in her right eye under the diagnosis of nonregmatogenous retinal detachment. There was only light perception. No treatment had been given. When first seen by us in April 1989 she was suffering from pain in her right eye. The eye was red and tender, with a flat anterior chamber. The lensiris diaphragm was pushed forward by a vascularked gray coloured mass, hardly seen through a cloudy cataractous lens. Her iris was severely vascularized also. Applanation tonometry value was over 50 mmHg. Orbital ultrasonography and orbital CT showed an irregular mass occupying most of the vitreous cavity (Figs. 1 and 2). Brain CT, chest X-ray, abdominal ultrasonography and detailed physical examination revealed no other lesion. The painful eye was enucleated. In gross appearance of the specimen the sclera was intact; the vitreous cavity was filled with a rough cystic structure (Fig. 3). In histopathologicsections of the specimen a cuticular membrane of a hydatid cyst was seen attached to retinal tissue at some sites (Fig. 4). A germinal epithelium covered the internal surface of the cyst wall, but no scolexes were observed. The cystic space contained a clear liquid. Following the diagnosis intraocular hydatid cyst or echinococcosis, orbital ultrasonography, cerebral CT, chest X-ray and abdominal ultrasonography were repeated, in search of a primary site, at each follow-
Fig. I . Ultrasonography of the right eye shows an irregular mass in the vitreous cavity.
up visit. After 1.5 years there still was no evidence of cystic pathology elsewhere. Further, there was a negative indirect hemagglutination test.
Fig. 3. Only a small space or vitreous is free behind the lens, which is pushed forward to be in contact with cornea.
Discussion Orbital echinococcosis is seen sporadically in underdeveloped countriesbut intraocular hydatid
Fig. 2. Computerized tomography of the orbit: the right eye is filled with the lesion. 51*
803
Fig. 4. Histopathologic section of the enucleated eye. The cyst wall adheres to retina (R) and the germinative membrane (GM) covers the inner surface of the lamina cuticularis (LC) of the hydatic cyst (HE, 100X).
cyst is extremely rare (Benrabah et al. 1984). In an Iraqi series of orbital tumours (Talib 1972) reported a 20 percent rate, while orbital implantation of the oncosphere appears in about 1%(ha1& Andi 1989; Morales et al. 1988; Talmud et al. 1985). Intraocular hydatid cysts may be present beneath the retina or may lie free, floating within the vitreous cavity (Listricin 1953). The cyst increases in size at the rate of about one centimeter per year (Jones 1990). Our patient had a history of nearly three years prior to enucleation and a subsequent follow-up of 1% year. Although the indirect hemagglutination test is reported to be positive in less than 50%of the cases (Morals et al. 1988), the negative result at the last visit is taken as support for the infestation being a primary intraocular settlement. Other available serologic tests like complement fixation, latex a g glutination or bentonite flocculation tests are not pathognomonic for echinococcosis. Computerized brain tomography, chest X-rays, orbital and abdominal ultrasonographies remaining negative after 1.5 years further support the case as one of primary intraocular echinococcosis.
References Benrabah R, Aouchiche M & Hamza R (1984): Localisation orbitaire du kyste hydatique tomodansitometrie. Bull Soc Ophthalmol Fr 84: 29-34. Inal A & Andi M (1989):Orbita hidatid kisti. TOD XXIII. Ulusal Kongresi Bulteni 2: 800-803. Jones T C (1990):Cestodes. In: Mandell G L, Douglas R G & Bennett J E (eds). Principles and Practice of Infectious Diseases, 3rd ed, pp 2155-2156. Churchill Livingstone, Edinburgh. Litricin 0 (1953):Echinococcus cyst of the eyeball. Arch Ophthalmol50: 506-508. Morales A G, Croxatto J 0,Crovetto L & Ebner R (1988): Hydatid cysts of the orbit, a review of 35 cases. Ophthalmology 95: 1027-1032. Talib H (1972):Orbital hydatid disease in Iraq. Br J Surg 59: 391-399. Talmud M, Malbrel C & Mutte J (1985): Retentissement oculaire d'un kyste hydatique cerebral. Bull Soc Ophthalmol Fr 88: 175-177. Williams D F, Williams G A & CayaJ G (1987): Intraocular Echinococcus multilocularis. Arch Ophthalmol 105: 1106-1109. Received on February 8th, 1991. Author's address:
Dr. Satvet Sinav, Ondokuz Mayis Universitesi Tip Fakultesi, Goz Anabilim Dali, Samsun, Turkey.
804
69 (1991) 802-804
CASE REPORT
A primary intraocular hydatid cyst Satvet Sinavl, Ali Demirc?, Bahar Sinav3, Fatma t)ge’, Yuksel Sullu’ and Bedri Kandemir4 Ondokuz Mayis University, Department of Ophthalmology’,Department of Radiology2, Department of Biochemistry3,Department of Pathology4, Faculty of Medicine, Samsun,Turkey
Abstract. Ultrasonography and computerized tomography (CT) of the painful and blind right eye of a 13year-old girl showed a cystic mass almost filling the vitreous cavity and narrowing the anterior chamber. The histopathologic diagnosis of the enucleated eye was a n echinococcus cyst. Abdominal ultrasonography, brain CT and chest X-rays gave no abnormal findings when first examined, nor during the follow-up 18 months after surgery. Indirect hemagglutination test was negative at the last control. The case was accepted as a primary hydatid cyst (echinococcosis) of the eyeball. Key words: echinococcosis - hydatid cyst - intraocular tumor - ultrasonography - computerized tomography.
The tapeworm Echinococcus granulosus exists in nature in infected dogs, which house the adult worm in their intestinal tract. Sheep or cattle become the intermediate hosts by ingesting the contaminated dog feces and thereby become the hosts for the gradually enlarging cyst, the hydatid. In humans, the liver (60%)and lungs are the most common sites of hydatid cyst development, but all organs may be affected. The small oncosphere undergoes dramatic changes after implantation, increasing in size to two or three centimeters in a few months. The embryo develops a central cyst, which has an inner germinal layer containing numerous nuclei (Jones 1990). Echinococcus is seen in most sheep and cattleraising areas of the world including Australia, East Africa, South America, Eastern Europe and the Middle East. The relatively benign type E.granulosus is the more common type, but E.alveolaris may be fatal (Williams et al. 1987). 802
Case Report A 13-year-oldgirl was referred to our department hom another eye clinic where she had been followed for three years. In 1986 she had lost the vision in her right eye under the diagnosis of nonregmatogenous retinal detachment. There was only light perception. No treatment had been given. When first seen by us in April 1989 she was suffering from pain in her right eye. The eye was red and tender, with a flat anterior chamber. The lensiris diaphragm was pushed forward by a vascularked gray coloured mass, hardly seen through a cloudy cataractous lens. Her iris was severely vascularized also. Applanation tonometry value was over 50 mmHg. Orbital ultrasonography and orbital CT showed an irregular mass occupying most of the vitreous cavity (Figs. 1 and 2). Brain CT, chest X-ray, abdominal ultrasonography and detailed physical examination revealed no other lesion. The painful eye was enucleated. In gross appearance of the specimen the sclera was intact; the vitreous cavity was filled with a rough cystic structure (Fig. 3). In histopathologicsections of the specimen a cuticular membrane of a hydatid cyst was seen attached to retinal tissue at some sites (Fig. 4). A germinal epithelium covered the internal surface of the cyst wall, but no scolexes were observed. The cystic space contained a clear liquid. Following the diagnosis intraocular hydatid cyst or echinococcosis, orbital ultrasonography, cerebral CT, chest X-ray and abdominal ultrasonography were repeated, in search of a primary site, at each follow-
Fig. I . Ultrasonography of the right eye shows an irregular mass in the vitreous cavity.
up visit. After 1.5 years there still was no evidence of cystic pathology elsewhere. Further, there was a negative indirect hemagglutination test.
Fig. 3. Only a small space or vitreous is free behind the lens, which is pushed forward to be in contact with cornea.
Discussion Orbital echinococcosis is seen sporadically in underdeveloped countriesbut intraocular hydatid
Fig. 2. Computerized tomography of the orbit: the right eye is filled with the lesion. 51*
803
Fig. 4. Histopathologic section of the enucleated eye. The cyst wall adheres to retina (R) and the germinative membrane (GM) covers the inner surface of the lamina cuticularis (LC) of the hydatic cyst (HE, 100X).
cyst is extremely rare (Benrabah et al. 1984). In an Iraqi series of orbital tumours (Talib 1972) reported a 20 percent rate, while orbital implantation of the oncosphere appears in about 1%(ha1& Andi 1989; Morales et al. 1988; Talmud et al. 1985). Intraocular hydatid cysts may be present beneath the retina or may lie free, floating within the vitreous cavity (Listricin 1953). The cyst increases in size at the rate of about one centimeter per year (Jones 1990). Our patient had a history of nearly three years prior to enucleation and a subsequent follow-up of 1% year. Although the indirect hemagglutination test is reported to be positive in less than 50%of the cases (Morals et al. 1988), the negative result at the last visit is taken as support for the infestation being a primary intraocular settlement. Other available serologic tests like complement fixation, latex a g glutination or bentonite flocculation tests are not pathognomonic for echinococcosis. Computerized brain tomography, chest X-rays, orbital and abdominal ultrasonographies remaining negative after 1.5 years further support the case as one of primary intraocular echinococcosis.
References Benrabah R, Aouchiche M & Hamza R (1984): Localisation orbitaire du kyste hydatique tomodansitometrie. Bull Soc Ophthalmol Fr 84: 29-34. Inal A & Andi M (1989):Orbita hidatid kisti. TOD XXIII. Ulusal Kongresi Bulteni 2: 800-803. Jones T C (1990):Cestodes. In: Mandell G L, Douglas R G & Bennett J E (eds). Principles and Practice of Infectious Diseases, 3rd ed, pp 2155-2156. Churchill Livingstone, Edinburgh. Litricin 0 (1953):Echinococcus cyst of the eyeball. Arch Ophthalmol50: 506-508. Morales A G, Croxatto J 0,Crovetto L & Ebner R (1988): Hydatid cysts of the orbit, a review of 35 cases. Ophthalmology 95: 1027-1032. Talib H (1972):Orbital hydatid disease in Iraq. Br J Surg 59: 391-399. Talmud M, Malbrel C & Mutte J (1985): Retentissement oculaire d'un kyste hydatique cerebral. Bull Soc Ophthalmol Fr 88: 175-177. Williams D F, Williams G A & CayaJ G (1987): Intraocular Echinococcus multilocularis. Arch Ophthalmol 105: 1106-1109. Received on February 8th, 1991. Author's address:
Dr. Satvet Sinav, Ondokuz Mayis Universitesi Tip Fakultesi, Goz Anabilim Dali, Samsun, Turkey.
804
