Printer Friendly

Orbital hydatid cyst in childhood: a report of two cases.

Abstract: Intraorbital hydatid disease (IHD) is quite rare and represents < 1% of the Echinococcus cases. In our current communication, we report two children with solitary, primary intraorbital hydatid cysts. Both children presented with nontender, nonpulsatile proptosis. Imaging workup, including CT and MRI scans of the head and the orbits, revealed a retro-bulbar cyst in both patients. Surgical resection was performed by employing a fronto-temporo-orbito-zygomatic (FTOZ) approach. Accidental intraoperative rupture occurred in one case with no further consequences. Albendazole was postoperatively employed for 12 weeks. Outcome was excellent in both cases with complete resolution of their symptoms and complete visual recovery. Pertinent literature was reviewed with this opportunity.

Key Words: albendazole, exophthalmos, hydatid cyst, orbital, outcome


Echinococcosis represents one of the most common human parasitoses in certain geographical areas; its incidence, clinical manifestations, outcome and treatment have been adequately described in the literature. (1) Although liver and lungs are usually involved in the vast majority of cases, the presence of hydatid cysts in other organs has also been extensively reported. (1) The incidence of orbital hydatid disease has been reported to range from 0.3% to 1.0% among patients with Echinococcosis, while in highly endemic areas orbital hydatid cysts represent 5 to 18% of patients referred to hospitals with ocular orbital space occupying lesions. (2-7) Increased migration and world-wide traveling have made the detection of orbital hydatid cyst cases possible in geographical areas such as Northern America and Northern Europe.

In our current communication we present two cases of orbital hydatid cysts in children, and with this opportunity, we review the pertinent literature regarding the diagnosis and the therapeutic management of patients harboring orbital hydatid disease.

Case Report

Patient 1

A 15 year old girl was admitted to the neurosurgical service with severe proptosis of the left eye after sustaining a minor head injury 48 hours earlier. Her physical examination revealed moderate left eye proptosis, which according to the patient was firstly noted approximately two weeks ago. Her left eye was non tender to palpation while no bruits were audible. Ocular motion was within normal limits. Fundoscopic examination revealed left-sided Grade II pupillary edema. A head CT scan was obtained, which showed a well-defined, round, hypo-dense mass with a peri-lesional rim of hyperdensity; the adjacent bony structures appeared intact (Fig. 1). A MRI study demonstrated a homogeneous unilocular lesion, which was hypointense on T1 weighted images and hyperintense on T2 weighted images (Fig. 2).

At that point, systemic diagnostic workup thought to be necessary for ruling out disseminated hydatidosis. This included thorax and abdominal CT scans as well as echocardiogram. All of the obtained studies revealed no abnormalities. Surgical resection via a left-sided Fronto-Temporo-Zygomatic (FTOZ) craniotomy was undertaken, and with employing microsurgical dissection, the external capsule of the cyst was dissected from the intraorbital structures and the cyst was resected en toto.

No intraoperative or postoperative complications occurred. A postoperative CT-scan confirmed extirpation of the cyst (Fig. 3). Postoperatively, Albendazole (10 mg/kg twice a day) was administered to the patient for 12 weeks.

The patient was followed for 2 years. Her left-side proptosis was totally resolved within 2 weeks postoperatively, and her fundoscopic examination returned to normal within 6 weeks postoperatively. She remained totally asymptomatic, and she was finally discharged from our outpatient clinic.

Patient 2

A 4 year old male was admitted to the neurosurgical service with symptoms of progressive axial proptosis of the left eye and visual disturbances of four week duration. His physical examination revealed a non tender to palpation left eye proptosis, which was nonpulsatile; ipsilateral IVth and VIth nerve paresis was also noted. The rest of his physical examination was unremarkable.

Head and orbital CT and MRI scans were obtained revealing a moderate size cyst within the left orbital cavity, which was compressing and was anteriorly displacing the ocular globe; the cyst demonstrated homogenous intensity and there was no contrast enhancement (Fig. 4). The rest of his imaging workup, including cardiac echo and thorax and abdominal CT-scans, revealed no other lesions.

The patient underwent surgical resection of the retrobulbar cyst via a left FTOZ approach. Intraoperatively, the outer capsule of the exposed cyst was identified, and an effort was made to dissect it from the adjacent, tightly-adherent neural structures. During the micro-dissection, a tear of the outer membrane occurred, with subsequent cystic fluid leakage. The operative field was thoroughly irrigated with 9% hypertonic saline solution, while the cystic content was meticulously removed. The cyst membrane was dissected and finally, completely removed (Figs. 5 & 6).

The patient was maintained on Albendazole (10 mg/kg twice daily for 12 wk) and his postoperative period was uneventful. His clinical examination improved with total resolution of his preoperative proptosis and cranial nerve pareses within three months postoperatively. His follow-up (3 years) revealed no other complications or any other systematic lesions, while the patient remained totally asymptomatic and was finally discharged from our outpatient clinic.


Orbital hydatidosis represents a quite rare clinicopathologic entity. (2-7) It can be differentiated to primary, where cyst or cysts are solely located into the orbital cavity (as in our cases) and secondary, when orbital lesions represent a component of disseminated multi-organ systemic disease. (8,9) Its incidence varies from 0.8 to 1.0% of all hydatid disease cases, but in highly endemic areas, orbital hydatid cysts have been reported to represent up to 25.8% of all cystic orbital lesions. (2-7,10,11) Orbital Echinococcal cysts are significantly more common in children (12-15); however, cases of elderly patients with isolated primary orbital cysts have been reported. (16-19) Orbital hydatid cysts are solitary lesions in the vast majority of cases, but there are reports in the literature of multiple intraorbital cysts, a phenomenon which occurs in less than 5% of patients with orbital hydatid cysts. (7) The usual strains responsible for orbital cysts are E granulosus and E multicularis, although E oligarthrus has been isolated in cases from South and Central America. (1,20,21)

Even though intraorbital hydatid cyst is quite rare, especially in nonendemic areas, it should always be included in the differential diagnosis of orbital space-occupying lesions. The most commonly presenting clinical symptoms and signs of orbital Echinococcosis are: nonpulsatile, nontender exophthalmos (as in our cases), visual disturbances, papilledema, diplopia, chemosis, eyelid edema, conjunctivitis and hypopyon. (22) The mean duration of symptoms has been reported to vary between 3 months and 2 years. (23) In our cases, the duration of symptoms was less than 4 weeks. There are reports in the literature, mostly from developing countries, in which patients presented at very late stages of their disease with subsequent unfavorable outcome. (24,25) However, a few cases with acute onset visual acuity loss have been reported, due to retinal detachment. (26,27) Exudative retinal detachment can be explained on the basis of toxins liberated from the semipermeable cyst wall, causing local inflammation. (27)

In regards to the anatomic location of the intraorbital hydatid cysts, various orbital locations have been described. (4,28-36) It is well accepted that there is a predilection for the superomedial and superolateral orbital areas. (37) More uncommon locations, such as within the medial rectus muscle, subretinally in the vitreous body in the anterior chamber, (29) and retro-orbitally (35) have also been reported. Interestingly, Talib, in his report, stated that left-sided lesions were more common than right-sided ones; due to the fact that left, common carotid artery arises directly from the summit of the aortic arch. (3) Another interesting observation is the presentation of several cases in association with a minor head injury, as in one of our cases. (17) This association might be explained by the fact that the sustained head injury alarms the patients and their families and makes them seek medical help so their long-standing ocular symptoms are discovered; however, etiologic association cannot be ruled out.


The diagnostic imaging approach of these lesions has been thoroughly investigated and adequately described in previous series. (13,29,32,37-39,40-42) However, the absence of the "water-lily" sign in the orbital cysts, which represents a typical characteristic of the Echinococcal cysts in other locations, is a point that needs to be emphasized; there is only one orbital case reported with a positive "water-lily" sign. (42) The superiority of the MRI in the detection of the lesion and the delineation of its relationship to the adjacent ocular structures cannot be overemphasized. (29,32,38) It is also widely accepted that none of the currently employed serologic tests can rule out the existence of a solitary orbital hydatid cyst. (3,17,38,43)



In regards to the selection of the most appropriate surgical approach for resecting an orbital hydatid cyst, several reports exist in the literature. (5,9,44-46) Each approach carries certain advantages and disadvantages. The size of the cyst, its anatomic location, its extension into the cranial cavity, the patient's general medical condition, the extent of the disease and the surgeon's familiarity with each surgical approach are a few among other factors that will dictate the most suitable approach. We selected the FTOZ approach in our cases because it provides adequate exposure for meticulous microsurgical dissection of the cyst and is very frequently employed in our department in comparison to some purely orbital approaches. The major disadvantage of this approach is the risk of intraoperative cyst rupture and spillage of its content into the cranial cavity. Meticulous microsurgical dissection, continuous irrigation during dissection and adequate visualization are key elements in successful extirpation of these cysts. Postoperative adjuvant chemotherapy with albendazole is recommended by previous reports. (7,47) The outcome of these procedures has been reported excellent in the vast majority of cases (4,5,44,45); however, in late stage cases with extensive optic atrophy and global compression, enucleation of the globe has been reported. (24,25,48)




The early diagnosis of orbital hydatidosis and its prompt surgical management has excellent results in the vast majority of these patients. This makes the importance of its appropriate diagnosis even more crucial. The clinician should always include the diagnosis of hydatid cyst in the differential diagnosis list of orbital mass, especially in children. Increases of world tourism and migration should make the clinician to include this quite rare diagnosis in his list even in nonendemic areas.


1. Plorde JJ, Ramsey PG: Nematodes, Cestodes, and Hermaphroditic Trematodes. In Wilson JD, Braunwald E., Isselbacher KJ, et al (eds): Harrison's Principles of Internal Medicine, 12th ed. New York, McGraw-Hill Inc, 1991, pp 827-828.

2. Shah A, Kirtane MV, D'Souza CE, et al. Hydatid cyst of the orbit (a case report). J Postgrad Med 1998;34;43-44A.

3. Talib H. Orbital hydatid disease in Iraq. Br J Surg 1972;59:391-394.

4. Gomez Morales A, Croxatto JO, Crovetto L, et al. Hydatid cysts of the orbit. A review of 35 cases. Ophthalmology 1988;95:1027-1032.

5. Xiao A, Xueyi C. Hydatid cysts of the orbit in Xinjiang: a review of 18 cases. Orbit 1999;18:151-155.

6. Klauss V, Chana HS. Ocular tumors in Africa. Soc Sci Med 1983:17: 1743-1750.

7. Jimenenez-Mejias ME, Alarcon-Cruz JC, Marquez-Rivas FJ, et al. Orbital hydatid cyst: treatment and prevention of recurrences with albendazole plus praziquantel. J Infect 2000;105-107.

8. Staindl O, Kenkel C. Echinokokkose der Orbita [Echinococcosis of the orbit]. HNO 1985;33:404-408.

9. Betharia SM, Pushker N, Sharma V, et al. Disseminated hydatid disease involving orbit, spleen, lung and liver. Ophthalmologica 2002;216:300-304.

10. Belmekki M, El Bakkali M, Abdellah H, et al. Epidemiologie des processus orbitaires chez l'enfant. A propos de 54 cas. J Fr Ophthalmol 1999;22:394-398.

11. Cooney RM, Flanagan KP, Zehyle E. Review of surgical management of cystic hydatid disease in a resource limited setting: Turkana, Kenya. Eur J Gastroenterol Hepatol 2004;16:1233-1236.

12. Reddy MV, Satyndran OM, Krishna KS. Orbital hydatid cyst. J Indian Med Assoc 1970;55:203-204.

13. Danziger A, Price H. Computed tomographic findings in orbital echinococciasis. J Comp Assist Tomogr 1980;4:128-129.

14. Zahawi HM, Hameed OK, Abalkhail AA. The possible role of the age of the human host in determining the localization of hydatid cysts. Ann Trop Med Parasitol 1999;93:621-627.

15. Flament J, Boukoffa SO, Rahmani A, et al. Place du kyste hydatique dans la pathologie orbitaire de l'est algerien. Rev Otoneuroophtalmol 1980;52:147-152.

16. Liegl O, Werner H, Janitschke K. Echinokokkus-Zyste in der Orbita. Klin Monatsbl Augenheilkd 1980;177:80-84.

17. Crompton JL, Iyer PV, Merry DJ, et al. Hydatid cyst: an unusual cause of diplopia. Aust N Z J Ophthalmol 1985;13:195-203.

18. Fink AM, Newman DE, Stringer DA. Pediatric case of the day. Orbital hydatid cyst. Radiographics 1995;15:731-744.

19. Lerner SF, Gomez Morales A, Croxatto JO. Hydatid cyst of the orbit. Arch Ophthalmol 1991;109:285.

20. Lopera RD, Melendez RD, Fernandez I, et al. Orbital hydatid cyst of Echinococcus oligarthrus in a human in Venezuela. J Parasitol 1989; 75:467-470.

21. Basset D, Girou C, Nozais IP, et al. Neotropical echinococcosis in Suriname: Echinococcus oligarthrus in the orbit and Echinococcus vogeli in the abdomen. Am J Trop Med Hyg 1998;59;787-790.

22. Gokcek C, Bayar N, Buharai Z. Total removal of an unruptured orbital hydatid cyst. Can J Ophthalmol 2001;36:218-220.

23. Ozek MM, Pamir MN, Sav A. Spontaneous rupture of an intraorbital hydatid cyst. A rare cause of acute visual loss. J Clin Neuroophthalmol 1993;13:135-137.

24. Gola R, Cheynet F, Ourgaud M, et al. Kyste hydatique orbitaire. A propos de 2 cas. Rev Stomatol Chir Maxillofac 1990;91 (Suppl 1):59-62.

25. Huilgol AV. Orbital hydatid cyst. J All India Ophthalmol Soc 1963;11:79-81.

26. Gelisken F, Erda S, Kreissig I. Exsudative Netzhautablosung bei intraor-bitaler Echinokokkuszyste. Klin Monatsbl Augenheilkd 1994;205:109-111.

27. Jain GC, Gupta AP, Purohit BN, et al. Hydatid cyst of orbit. An unusual cause of proptosis. Indian Pediatr 1983;20:605-607.

28. Okelo GB, Kyobe J, Shah AK, et al. Retro-orbital hydatid: case report. East Afr Med J 1980;57:285-288.

29. Gokcek C, Gokcek A, Akif Bayar M, et al. Orbital hydatid cyst: CT and MRI. Neuroradiology 1997;39:512-515.

30. Amaya C, Villarejo F, Izquierdo JM, et al. Hydatid cyst: unusual cause of unilateral exophthalmos. Surg Neurol 1980;14:351-354.

31. Kiratli H, Bilgic S, Ozturkmen C, et al. Intramuscular hydatid cyst of the medial rectus muscle. Am J Ophthalmol 2003;135:98-99.

32. Aksoy FG, Tanrikulu S, Kosar U. Inferiorly located retrobulbar hydatid cyst: CT and MRI features. Comp Med Imaging Graph 2001;25:535-540.

33. Benrabah R, Aouchiche M, Hamza R, et al. Localisation orbitaire du kyste hydatique (10 observations) tomodensitometrie. Bull Soc Ophthalmol Fr 1984;84:29-34.

34. Alparslan L, Kanberoglu K, Peksayar G, et al. Orbital hydatid cyst: assessment of two cases. Neuroradiology 1990;32:163-165.

35. Agrawal R, Agrawal A. Retro-orbital hydatid cyst. J Indian Med Assoc 1999;97:525.

36. Lamba PA, Bhatia PC, Jain M, et al. Hydatid cyst of orbit. Indian J Ophthalmol 1983;31:23-25.

37. Diren HB, Ozcanli H, Boluk M, et al. Unilocular orbital, cerebral and intraventricular hydatid cysts: CT diagnosis. Neuroradiology 1993;35: 149-150.

38. Hanioglu S Saygi S, Yazar Z, et al. Orbital hydatid cyst. Can J Ophthalmol 1997;32:334-337.

39. Khalili AH. CT values in orbital hydatid cyst. Comput Radiol 1987;11:193-197.

40. Betharia SM, Sharma V, Pushker N. Ultrasound findings in orbital hydatid cysts. Am J Ophthalmol 2003;135:568-570.

41. Sperryn CW, Corr PD. CT evaluation of orbital hydatid disease: a review of 10 cases. Clin Radiol 1994;49:703-704.

42. Malde HM, Gadkari SS, Chadha D, et al. Water lily sign in an orbital hydatid cyst. J Clin Ultrasound 1993;21:458-459.

43. El-Arousy MH, Ismail SA. Cerebrospinal echinococcosis: serodiagnosis using different hydatid cyst fluid antigens. J Egypt Soc Parasitol 2005; 35:193-204.

44. Ergin M, Okian AML, Yuksel M, et al. Orbital hydatid cysts: report of four cases. Neurosurg Rev 1987;20:35-37.

45. Nahri GE. A simplified technique for removal of orbital hydatid cysts. Br J Ophthalmol 1991;75:743-745.

46. Akhan O, Bilgic S, Akata D, et al. Percutaneous treatment of an orbital hydatid cyst: a new therapeutic approach. Am J Ophthalmol 1998;125: 877-879.

47. Sihota R, Sharma T. Albendazole therapy for a recurrent orbital hydatid cyst. Indian J Ophthalmol 2000;48:142-143.

48. Sevel D, Sapeika RJ. Hydatid cyst of the orbit. Surv Ophthalmol 1997; 22:101-105.

Alexandru Vlad Ciurea, MD, Gambardella Giuseppe, MD, Theofilos G. Machinis, MD, Teodora Camelia Coman, MD, and Kostas N. Fountas, MD, PHD

From the Department of Neurosurgery, Clinic Hospital, Bagdasar-Arseni, Bucharest, Hungary; the Department of Microsurgery, Hospital Casa Di Cura Cristo Re, Messina, Italy; and the Department of Neurosurgery, The Medical Center of Central Georgia, Mercer University School of Medicine, Macon. GA.

Reprint requests to Kostas N. Fountas, 840 Pine Street, Suite 880, Macon, GA 31201. Email:

Accepted February 24, 2006.


* Orbital hydatid cyst represents a quite rare location of Echinococcosis.

* Orbital cysts occur most commonly in children.

* Early detection and prompt surgical resection of these orbital cysts result in excellent functional outcome in most patients.

* Albendazole is recommended as an adjunctive treatment.

* Inclusion of orbital hydatid cyst in the differential diagnosis of orbital mass is of paramount importance for their prompt treatment.
COPYRIGHT 2006 Southern Medical Association
No portion of this article can be reproduced without the express written permission from the copyright holder.
Copyright 2006, Gale Group. All rights reserved. Gale Group is a Thomson Corporation Company.

Article Details
Printer friendly Cite/link Email Feedback
Title Annotation:Case Report
Author:Fountas, Kostas N.
Publication:Southern Medical Journal
Date:Jun 1, 2006
Previous Article:A newborn with Beals syndrome.
Next Article:Ceftriaxone-resistant salmonella septicemia and osteomyelitis in sickle cell disease adults.

Related Articles
Is High Prevalence of Echinococcus multilocularis in Wild and Domestic Animals Associated with Disease Incidence in Humans?
Primary cardiac hydatid disease: cross-sectional imaging features.
Giant intrathoracic extrapulmonary hydatid cyst manifested as unilateral pectus carinatum.
Reemergence of canine Echinococcus granulosus infection, Wales.
Echinococcosis, Ningxia, China.
Primary hydatid cyst of the head and neck diagnosed with ultrasound and computed tomography: a report of two cases.
Echinococcosis in Tibetan populations, Western Sichuan Province, China.
Global socioeconomic impact of cystic echinococcosis.
Spinal hydatid disease.
Spinal hydatid disease, a rare but existent pathological entity: case report and review of the literature.

Terms of use | Copyright © 2017 Farlex, Inc. | Feedback | For webmasters