Orbital hydatid cyst: Review of 10 cases

Journal of Cranio-Maxillo-Facial Surgery (2010) 38, 274e278 Ó 2009 European Association for Cranio-Maxillo-Facial Surgery doi:10.1016/j.jcms.2009.10.001, available online at http://www.sciencedirect.com

Orbital hydatid cyst: Review of 10 cases Salma BENAZZOU, Maxillofacial Surgeon, MD1, Yasser ARKHA, Professor of Neurosurgery, MD2, Said DERRAZ, Professor of Neurosurgery, MD2, Abdessamad EL OUAHABI, Professor of Neurosurgery, MD2, Abdessalam EL KHAMLICHI, Professor and Chairman of Neurosurgery, MD2 1 2

Department of ENT and Maxillofacial Surgery, Avicenne University Medical Centre, Rabat, Morocco; Department of Neurosurgery, Avicenne University Medical Centre, Rabat, Morocco

SUMMARY. Background: Echinococcosis represents one of the most common human parasitoses in some geographical areas. Orbital involvement is extremely rare. Materials and methods: In the records of our Medical University Centre between 1984 and 2006, we found 10 cases of orbital hydatid cyst. Results: Among them, 6 males and 4 females; the ages ranged from 2 to 60 years and 7 cases (70%) were less than 12 years of age. The main symptoms of orbital hydatid cyst were slowly progressive unilateral proptosis (100%) with visual loss (90%). The presumptive diagnosis was made on the images obtained from computed tomography and/or magnetic resonance imaging. Surgical removal was the main treatment; although, it is frequently complicated with cyst rupture and spillage of the contents. For this reason, we adopted the technique of cyst puncture with irrigation. A postoperative antihelminthic treatment has always been used. Conclusion: Hydatid cyst is an endemic disease in Morocco. Orbital involvement should be considered in the differential diagnosis of proptosis especially in children known to have been in affected geographical areas. Ó 2009 European Association for Cranio-Maxillo-Facial Surgery

Keywords: hydatid cyst, orbital echinococcosis, diagnosis, surgery

diagnostic studies, treatment and outcomes were evaluated.

INTRODUCTION In humans, the liver and lungs, followed by the central nervous system, are the most frequent sites of hydatid cysts, which are caused by Echinococcus granulosus (Onerci et al., 1991). It is endemic in some cattle- and sheep-raising regions of the world such as Mediterranean countries (Morocco), Central Europe, the Middle East, South America, Australia, and New Zealand (Turgut et al., 1992; Turgut et al., 2004). Orbital hydatid cyst is extremely rare worldwide, accounting for less than 1% of all hydatid cysts (Alparslan et al., 1990). However, in endemic areas, orbital hydatid cyst can account for nearly 20% of orbital masses (Talib, 1972; Gomez Morales et al., 1988). In the present paper, we report 10 new cases of orbital hydatid cysts from the Avicenne University Medical Centre (presenting since 1984).

RESULTS Among the 10 cases of orbital hydatid cysts, there were 6 males and 4 females. 70% were under 12 years of age at the time of initial examination. The median age was 18 years (range, 2e60). There was no contributing medical history in 9 patients, but one was operated upon years earlier for hydatid cyst of the parotid gland. All patients were from rural areas and presented with durations varying from 2 months to 1 year. They had a history of slowly progressive unilateral proptosis, irreducible, non-pulsatile, and non-fluctuant (100%) lesions. There was partial visual loss in 8 patients and a complete visual loss in 1 patient at presentation. Impaired ocular motility was found in 6 patients. Orbital pain was reported in 2 cases, and conjunctival erythema in 1 case. Fundoscopic examination revealed papilloedema with engorged retinal veins in 5 cases and an atrophy of the optic nerve in 1 case. The presumptive diagnosis of hydatid cyst was made on cranioeorbital computed tomography (CT) scan in 9 patients, on ultrasonography (US) in 1 patient and on magnetic resonance imaging (MRI) in 2 patients. They showed a well-defined, intraorbital, unilocular cyst in

MATERIALS AND METHODS A review of the hospital records between 1984 and 2006 revealed that 166 intraorbital tumours have been operated upon in the department of Neurosurgery; some of them having been documented by the ophthalmologists. Central nervous system involvement by hydatid disease was recorded in 135 cases during the same period. Among them, 10 cases of orbital hydatid cyst have been found. The epidemiology, clinical presentations, 274

No recurrence No recurrence Puncture + irrigation Alb Puncture + irrigation Alb ua ( ) 2004 46 years/F 2006 9 years/M 9 10

M, male; F, female; ua, unavailable.

1997 60 years/M 2 years 8

3 months Proptosis, visual loss 10 months Proptosis, visual loss, impairment of ocular motility

1995 36 years/F 7

9 months

( ) ( )

No recurrence Puncture + irrigation Alb (+) (+)

ua Puncture + irrigation Alb ( ) ( ) CT

Atrophy of CT optical nerve ( ) CT, MRI Papilloedema MRI

ua Papilloedema ua Papilloedema ua 6 years/F 2 years/M 7 years/F 6 years/M 3 years/F 1984 1986 1986 1990 1992 2 3 4 5 6

3 months 11 months 2 months 1 years 2 months

Proptosis, visual loss Proptosis, visual loss, impairment ocular motility Proptosis, visual loss, impairment visual motility Proptosis, visual loss Proptosis, impairment ocular motility, conjunctival erythema Proptosis, visual loss, pain, impairment ocular motility Proptosis, pain, complete visual loss

Papilloedema

ua No recurrence No recurrence ua No recurrence ua Meb Meb Meb Alb Removal in toto Rupture Puncture + irrigation Puncture + irrigation Puncture + irrigation ( ) ( ) ( ) ua ua ) ) ) ) ) ( ( ( ( (

ua Rupture (+) ( )

CT (polycystic ecchinicoccosis) CT CT CT CT CT, US Papilloedema Proptosis, visual loss, impaired ocular motility 1984 11 years/M 1 years 1

Organ Hydatid Surgery involvement serology Diagnostic study Fundoscopic examination Clinical finding Time of diagnosis Age Sex Case Year

Orbital hydatidosis is a rare zoonosis caused by the larval stage of the cestode, genus E. granulosus. Its incidence varies from 0.3 to 1.0% of all hydatid disease cases (Alparslan et al., 1990; Shah et al., 1998; Ciurea et al., 2006). But in highly endemic areas, orbital hydatid cyst represents 5e26% of all cystic orbital lesions (Talib, 1972; Gomez Morales et al., 1988; Shah et al., 1998; Belmekki et al., 1999; Ciurea et al., 2006) in our medical university centre, it accounts for 6%. It is primary echinococcosis, and is generally unilateral. Patients are mostly children (70% of our series was under 12 years). It is uncommon to find other simultaneous hydatid cysts elsewhere in the body (Onerci

Table 1 e The ten patients with orbital hydatid cyst

DISCUSSION

Antihelminthic Outcome treatment

all but 1 patient (polycystic) with an average diameter of about 24 mm (range, 18 mme40 mm). All the orbital hydatid cysts were located in retrobulbar tissues, 7 in the superomedial angle and 3 in the superolateral angle. Orbital enlargement was noticed in 2 cases without an extension to neighbouring structures. To exclude disseminated hydatidosis, all the patients had a chest X-ray and abdominal US that revealed no abnormalities. The blood chemistry was normal with eosinophilia in 8 cases and positive serological tests of echinococcal (Casoni’s intradermal test, Ig M Enzyme-linked immunosorbent assay (ELISA), and indirect haemagglutination (IHA)) in only 2 patients. All the patients underwent operations, in the department of Neurosurgery, to remove the orbital hydatid cyst. The FrontoeTemporal approach was undertaken in our cases with a microsurgical dissection. After orbital deroofing and incision of the periorbita, the external capsule of the cyst was dissected from the intraorbital structures; the cyst was resected in toto in 1 case, accidental intraoperative rupture occurred in 2 cases and a cystic aspiration with hypertonic saline irrigation was performed in 7 cases. The cyst was punctured with a 24-gauge needle and the contents were aspirated with extreme care. The cyst cavity was irrigated with hypertonic saline solution. After removing the cyst wall, we irrigated the operation field with the same solution. Histological confirmation of orbital hydatid cyst was available in all the patients, demonstrating an outer laminated periodic acideSchiff positive membrane and an inner membrane with numerous brood capsules and protoscolices attached to it, which confirmed the diagnosis of echinococcosis. In this series, hydatid disease was caused by E. granulosus. As seen in Table 1, 8 patients underwent chemotherapy after surgery. They received albendazole 10 mg/kg or mebendazole 30 mg/kg a day for 28 days, followed by a 2 weeks rest period for 3 months. Among the 10 cases in our series, only 7 were available for follow up. The others had not come to followup examinations. An improvement of proptosis (70%), of visual impairment (37.5%), of motility abnormalities (20%) and of papilloedema (50%) was noted. The patients had no adverse reaction to treatment. They had no evidence of local recurrence after 3 years.

No recurrence

Orbital hydatid cyst 275

276 Journal of Cranio-Maxillo-Facial Surgery

et al., 1991). However, one patient had suffered from hydatid disease of the parotid gland years earlier. The lesion is usually situated behind the globe and causes proptosis. The cysts are slow growing and may cause pressure symptoms such as visual loss, mechanical impairment of ocular motility, pain, chemosis, lid oedema, papilloedema, and optic atrophy (Gomez Morales et al., 1988; Gola et al., 1990; Turgut et al., 2004). The mean duration of symptoms has been reported to vary between 3 months and 2 years (Ozek et al., 1993). In our series, 80% had visual loss, but in one patient, this was complete at the time of initial examination. This high incidence of visual loss in Moroccan the population is probably due to the advanced stage of the disease in most patients. Orbital hydatid cysts are almost located in the superomedial or superolateral angle of the orbit, in or close to the muscle cone. Erosion of the orbital roof may occasionally lead to intracranial infection (Xiao and Xueyi, 1999; Turgut et al., 2004; Ciurea et al., 2006). The diagnostic imaging of these lesions has been thoroughly investigated. CT and MRI use became widespread and US has lost its importance. On CT (Diren et al., 1993; Sperryn and Corr, 1994; Go¨kc¸ek et al., 1997; Aksoy et al., 2001), the orbital hydatid cyst appears as a hypodense, well-defined fluid-filled cyst which is thin-walled and mostly unilocular with densitometric values similar to the globe, and with a fine peripherical ring enhancement of its fibrous capsule after contrast was given (Fig. 1). Polycystic echinococcosis of the orbit appears as closely packed multiple cysts; it occurs in less than 5% of patients with orbital hydatid cysts (Ciurea et al., 2006). Only one patient had this phenomenon (Fig. 2). CT appearances of orbital hydatid cyst can be easily confused with dermoid cyst, abscess, intraobital haematoma and lymphangioma (Fink et al., 1995). Orbital MRI has proved to be a useful, non-invasive diagnostic tool to rule out other cystic lesions in spite of its limited use in endemic areas due to high cost. The cyst appears as a low-intensity signal on T1-weighted images and as high-intensity signal onT2-weighted images

Fig. 1 e Axial CT scan showing a well-defined, thin-walled fluid-filled unilocular cyst located along the medial wall of the left orbit.

(Go¨kc¸ek et al., 1997; Aksoy et al., 2001). After an intravenous injection of Gadolinium, the lesion exhibits capsular contrast enhancement on T1-weighted images (Fig. 3). Eosinophilia appears in 20e25% (in our series, we found 80%). The serological tests may be negative in 50e60% because of the integrity of the cyst wall in the orbit (Kiratli et al., 2003; Murthy et al., 2005). Once the diagnosis of orbital hydatid cyst is made or suspected, the primary treatment is surgical (Talib, 1972; Gomez Morales et al., 1988; Xiao and Xueyi, 1999; Turgut et al., 2004). All the patients have been operated upon in the department of Neurosurgery by using a frontoetemporal craniotomy. Technically, there are various orbital surgical methods that could be used by the ophthalmologists unless there is an extension of the lesion to the cranial cavity (Lamba et al., 1983; Kaymaz et al., 2002; Kiratli et al., 2003; Murthy et al., 2005). The definitive treatment of hydatid cyst is surgical removal without rupture. However, it is almost impossible because of the usually thin wall of young cysts, and restricted access in the orbital cavity (Nahri, 1991; Ergu¨n et al., 1997). For these reasons, we adopted e as reported in the literature (Nahri, 1991; Ersahin et al., 1993; Ergu¨n et al., 1997) e the surgical decompression and irrigation with hypertonic saline in the 7 last cases as an alternative approach to the conventional surgical extirpation. The most important complication of the surgical treatment of orbital hydatid cyst is spillage of the contents of the cyst which leads perioperatively to anaphylaxis and in the follow up to secondary dissemination with local recurrence (Agarwal et al., 1982; Lamba et al., 1983; Ozek et al., 1993). Adjuvant medical treatment with antihelmintics is of great importance in these cases. The first reports regarding medical management of this entity used mebendazole (Agarwal et al., 1982). However, a multicentred clinical trial of albendazole and mebendazole in human cystic echinococcosis demonstrate that albendazole is more effective than mebendazole, with a higher degree of systemic absorption and penetration into the cyst; It also shows that albendazole for a longer period appeared to lead to greater success (Davis et al., 1989; Sihota and Sharma, 2000). We prescribed three-months course of albendazole (10 mg/Kg) or mebendazole

Fig. 2 e Axial CT scan showing closely packed multiple cysts.

Orbital hydatid cyst 277

Fig. 3 e (a): contrast-enhanced T1-weighted axial and coronal MRI demonstrating low signal intensity of the orbital mass and capsular contrast enhancement. (b): T2-weighted axial and coronal MRI of the same patient.

(30 mg/Kg) with good results and no biochemical abnormalities of liver function have been reported. Albendazole is also recommended as preventive therapy for 14e28 days prior to surgery (Fink et al., 1995; Jimene´nez-Mejı´as et al., 2000; Ciurea et al., 2006), but we have no experience of presurgical chemotherapy.

CONCLUSION Orbital hydatid cyst should be considered in the differential diagnosis of proptosis in children living in poor socioeconomic circumstances in regions where echinococcosis is endemic. But, increases in world tourism and migration should make the clinician include this quite rare diagnosis in his list of differential diagnosis, even in non-endemic areas. References Aksoy FG, Tanrikulu S, Kosar U: Inferiorly located retrobulbar hydatid cyst: CT and MRI features. Comp Med Imaging Graph 25: 535e540, 2001

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Dr Salma BENAZZOU, MD BP 6626 Rabat Instituts Madinat Al Irfane Rabat Morocco E-mail: [email protected] Paper received 1 January 2009 Accepted 5 October 2009