Ординатура / Офтальмология / Английские материалы / Surgical Atlas of Orbital Diseases_Mallajosyula_2009

a complaint of headache and orbital discomfort. The mucocele arises in the sphenoidal sinus and postethmoidal air cells and may mimic retrobulbar neuritis, or cause ophthalmoplegia and ptosis by affecting branches of 3rd cranial nerve along with axial proptosis.30,31

The differential diagnosis of cystic medial orbital lesion must also include encephalocele with skull base deformity.

Appearance of a typical mucocele on a CT scan includes bowing of the sinus wall into the orbit, with attenuation or even erosion of bone with a cystic cavity. MRI shows highly variable signal intensities.

Treatment of mucocele is surgical for reestablishment of normal drainage, removal of the lining of the cyst. Frontal and ethmoidal mucocele are approached sub-periosteally with elevation of an osteoplastic flap32; eradication and extirpation of all diseased mucosa is done. Successful endoscopic sinus obliteration and or re-establishing sinus drainage protects against recurrence.33 Sphenoidal sinus mucocele is approached intranasally. Neurosurgical consultation is indicated for those mucoceles with intracranial extension.34,35

CYSTS OF THE OPTIC NERVE SHEATH

Dilatation and expansion of the optic nerve sheath has been observed in variety of neoplastic and nonneoplastic condition. Such a cystic growth in a optic nerve sheath filled with CSF has been perioptic hygroma, arachnoid cyst of the optic nerve sheath, optic hydrops and meningocele.36,37 Most perioptic hygromas present in patients between the ages of 30-60yrs with complaints of headache and visual disturbance. Optic nerve signs are defective vision, RAPD, optic disc edema, optic atrophy and visual field defects. The cyst can be well delineated in MRI with fat suppression technique and gadolinium contrast.

Cystic Lesions of Orbit 203

HEMATIC CYST

Hematic cyst refers to the accumulation of hematogenous debris within a cavity lined with fibrous tissue but not epithelial or endothelial tissue.40,41

It can occur at any age. It should be distinguished from endothelial-lined blood containing cyst such as chocolate cyst associated with lymphangioma, venous varices and hemangioma. Hematic cysts are uncommon. Proptosis, globe displacement, motility disturbance may occur due to chronic hematic cyst (Figure 15.6). Spontaneous eyelid ecchymosis and edema may suggest this diagnosis. In acute cases there may be decrease in vision, RAPD and choroidal folds.42, 43

Hematic cyst may be due to trauma and an incompletely reabsorbed orbital hemorrhage, wherein the presence of old blood and blood breakdown product incite a granulomatous inflammation and fibrous encapsulation. Others believe that a cyst arises from bleeding within the bony diploes that breaks out into the peripheral space.44

Investigations

Plain film may show bony erosion when cyst arises in the superior orbit and involve the orbital portion of the frontal bone. CT shows well-defined nonenlarging mass (Figure 15.7) having the same density as the brain. On MRI T1 and T2 weighted signal hyper intensity are constant with blood.45

Treatment

Surgical evacuation of the cyst content, removal of the fibrous wall lining and establishment of meticulous hemostasis to prevent recurrence. Removal of one wall of the cyst often is all that is necessary and all that is attached to the orbital tissue can be left intact (Figures 15.8 and 15.9).

Figure 15.7: CT scan shows well defined mass in the orbital apex

Figure 15.8: Surgical approach for removal of hematic cyst

Figure 15.9: Postoperative appearance 3 weeks after the operation

Simple Cyst

Simple cyst may include serous, retention and implantation cyst. These are rare in the orbit but are slightly more common in the eyelid and conjunctiva. This cyst is lined by simple epithelium.

Retention Cyst

It originates in the glandular appendages of the conjunctiva and adnexal structure. Obstruction of the orifice of the lacrimal gland or accessory lacrimal gland from trauma or cicatrix may result in a thin walled epithelium lined cyst in the superior fornix that can cause mass effect.

Lacrimal Ductal Cyst

It arises often in the supero-lateral orbit and can be easily seen by everting the upper lid. These are frequently bilateral,46 they present as round lesions originating from the palpebral portion of the lacrimal gland which protrudes into the superior fornix. Marsupialization may be preferable to complete excision, because attempt to excise the cyst may unnecessarily close the remaining lacrimal ductal cyst.47,48 Ductal cysts can rarely arise from accessory lacrimal glands.49

Implantation Cyst

It arises from misplacement of surface epithelium into the orbit as a result of trauma or surgery. It is difficult to eradicate due to variable depth. Removal of the entire cyst or electrodessication of the epithelial lining effects a cure.50

DACRYOCELE

Distension of the nasolacrimal sac by entrapped mucoid material results in the formation of a dacryocele due to obstruction of the nasolacrimal duct. A congenital dacryocele manifests at birth as a firm bluish swelling in the medial canthal area at or below the medial canthal tendon. Lacrimal sac is tense and filled with mucous and dacryocystitis with or without cellulitis may be a frequent presentation.51

Surgical indications are dacryocystitis, cellulitis, recurrent dacryocystitis, difficulties in breathing in large nasal cyst, failure to respond to conservative treatment. Difficulties in diagnosis of congenital dacryocele include - encephalocele, mucocele, dermoid cyst, and capillary hemangioma. Congenital dacryocele should be conservatively managed initially and if there is no response probing should not be delayed.52,53 Adult dacryocele needs dacryo- cysto-rhinostomy (DCR) surgery.53, 54

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2.Shields JA, Shields CL. Orbital cysts of childhood classification, clinical features and management. Surv Ophthalmol. 2004;49(3):281-99.

3.Stefanyszyn MA, Handler SD, Wright JE: Pediatric orbital tumors. Otolaryngol Clin North Am 1988;21:103.

4.Pfeiffer RL, Nicholl R J: Dermoid and epidermoid tumors of the orbit. Arch Ophthalmol 1948;40:639.

5.Ahuja R, Azar NF. Orbital dermoids in children. Semin Ophthalmol. 2006;21(3):207-11.

6.Yanoff M, Fine BS: Ocular Pathology: A Text and Atlas, 3rd ed. Philadelphia, JB L ippincott, 1989.

7.Rootman J: Diseases of the Orbit: A Multidisciplinary Approach. Philadelphia, JB Lippincott, 1988.

8.Bullock JD, Bartley GB: Dynamic proptosis. Am J Ophthalmol 1986;102:104.

9.Yen KG, Yen MT. Current trends in the surgical management of orbital dermoid cysts among pediatric ophthalmologists. J Pediatr Ophthalmol Strabismus. 2006;43(6):337-40; quiz 363-4.

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10.Pryor SG, Lewis JE, Weaver AL, Orvidas LJ. Pediatric dermoid cysts of the head and neck. Otolaryngol Head Neck Surg. 2005;132(6):938-42.

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12.Yuen HK, Chong YH, Chan SK, Tse KK, Chan N, Lam DS. Modified lateral orbitotomy for intact removal of orbital dumbbell dermoid cyst. Ophthal Plast Reconstr Surg. 2004;20(4):327-9.

13.Duke-Elder S: System of Ophthalmology, Vol XIII, Normal and Abnormal Development: Part II. Congenital Deformation. St. Louis, CV Mosby, 1963.

14.Levin ML, Leone CR Jr, Kincaid MC: Congenital orbital teratomas. Am J Ophthalmol 1986;102:476.

15.Thomas J, Gregory L, Louis B. Orbital Neoplasm. Orbit, Eyelid and Lacrimal System. American Academy of Ophthalmology, 2004-2005;Section 7.64.

16.Lee GA, Sullivan TJ, Tsikleas GP, Davis NG Congenital orbital teratoma. Aust N Z J Ophthalmol. 1997;25(1): 63-6.

17.Gnanaraj L, Skibell BC, Coret-Simon J, Halliday W, Forrest C, DeAngelis DD. Massive congenital orbital teratoma. Ophthal Plast Reconstr Surg. 2005;21(6):445-7.

18.Chang DF, Dallow RL, Walton DS: Congenital orbital teratoma: Report of a case with visual preservation. J Pediatr Ophthalmol Strabismus 1980;17:88.

19.Mahapatra AK, Agrawal D. Anterior encephaloceles: a series of 103 cases over 32 years. J Clin Neurosci. 2006;13(5):536-9. Epub 2006 May 6.

20.Mahapatra AK, Suri A. Anterior encephaloceles: a study of 92 cases. Pediatr Neurosurg. 2002;36(3):113-8.

21.Macfarlane R, Rutka JT, Armstrong D, Phillips J, Posnick J, Forte V, Humphreys RP, Drake J, Hoffman HJ. Encephaloceles of the anterior cranial fossa. Pediatr Neurosurg. 1995;23(3):148-58.

22.Raman Sharma R, Mahapatra AK, Pawar SJ, Thomas C, AlIsmaily M. Trans-sellar trans-sphenoidal encephaloceles: report of two cases. J Clin Neurosci. 2002;9(1):89-92.

23.Waring GO III, Roth AM, Rodrigues MM: Clinicopathologic correlation of microphthalmos with cyst. Am J Ophthalmol 1976;82:714.

24.Lieb W, Rochels R, Gronemeyer U: Microphthalmos with colobomatous orbital cyst: Clinical, histological, immunohistological, and electron microscopic findings. Br J Ophthalmol 1990;74:59.

25.Chaudhry IA, Shamsi FA, Elzaridi E, Arat YO, Riley FC. Congenital cystic eye with intracranial anomalies: a clinicopathologic study. Int Ophthalmol. 2007;27(4):223-33. Epub 2007 Apr 24.

26.Brodic G E. Cystic Lesion of the Orbit In:Principle and Practice of Ophthalmology 2nd ed. WB Saunders Company, 2000 pp 3072.

27.Doglietto F, Massimi L, Dickmann A, Tamburrini G, Caldarelli M, Di Rocco C. Microphthalmia and colobomatous cyst of the orbit. Acta Neurochir (Wien). 2006;148(10):1123-5. Epub 2006 Sep 8.

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28.Naik MN, Murthy RK, Raizada K, Honavar SG. Ethanolamine oleate sclerotherapy in the management of orbito-palpebral cyst associated with congenital microphthalmos. Am J Ophthalmol. 2005;139(5): 939-41.

29.Naik MN, Honavar SG, Murthy RK, Raizada K, Thomas R. Ethanolamine Oleate Sclerotherapy Versus Simple Cyst Aspiration in the Management of Orbitopalpebral Cyst Associated With Congenital Microphthalmos. Ophthal Plast Reconstr Surg. 2007;23(4):307-11.

30.Ajaiyeoba A, Kokong D, Onakoya A. Clinicopathologic, ophthalmic, visual profiles and management of mucoceles. J Natl Med Assoc. 2006;98(1):63-6.

31.Herndon M, McMains KC, Kountakis SE. Presentation and management of extensive fronto-orbital-ethmoid mucoceles. Am J Otolaryngol. 2007;28(3):145-7.

32.Ulualp SO, Carlson TK, Toohill RJ. Osteoplastic flap versus modified endoscopic Lothrop procedure in patients with frontal sinus disease. Am J Rhinol. 2000;14(1):21-6.

33.Har-El G. Endoscopic management of 108 sinus mucoceles. Laryngoscope. 2001;111(12):2131-4.

34.Peral Cagigal B, Barrientos Lezcano J, Floriano Blanco R, García Cantera JM, Sánchez Cuéllar LA, Verrier Hernández A. Frontal sinus mucocele with intracranial and intraorbital extension. Med Oral Patol Oral Cir Bucal. 2006;1;11(6):E52730.

35.Shah A, Meyer DR, Parnes S. Management of frontoethmoidal mucoceles with orbital extension: is primary orbital reconstruction necessary? Ophthal Plast Reconstr Surg. 2007;23(4):267-71.

36.Harris GJ, Sacks JG, Weinberg PE, O'Grady RB: Cyst of the intraorbital optic nerve sheaths. Am J Ophthalmol 1976;81:656.

37.Miller NR, Green WR: Arachnoid cysts involving a portion of the intraorbital optic nerve. Arch Ophthalmol 1975;93:1117.

38.Moschos MM, Lymberopoulos C, Moschos M. Arachnoid cyst of the optic nerve: a case report. Klin Monatsbl Augenheilkd. 2004;221(5):408-9.

39.Akor C, Wojno TH, Newman NJ, Grossniklaus HE. Arachnoid cyst of the optic nerve: report of two cases and review of the literature. Ophthal Plast Reconstr Surg. 2003;19(6):466-9.

40.Krohel GB, Wright JE: Orbital hemorrhage. Am J Ophthalmol 1979;88:254.

41.Pearson PA, Rakes SM, Bullock JD: Letter: Clinicopathologic study of hematic cysts of the orbit. Am J Ophthalmol 1986;102:804.

42.Milne HL 3rd, Leone CR, Kincaid MC, Brennan MW. Chronic hematic cyst of the orbit. Ophthalmology. 1987;94(3):271-7.

43.Privat C, Bellamy J, Courthaliac C, Kinn T, Ravel A, Mondie J, Bacin F, Boyer L. Chronic hematic cyst of the orbit (orbital subperiosteal hematoma). J Radiol. 2000;81(7):811-4.

44.Cameron JD, Letson RD, Summers CG: Clinical significance of hematic cyst of the orbit. Ophthalmic Plast Reconstr Surg 1988;4(2):95.

45.Kersten RC, Kersten JL, Bloom HR, Kulwin DR. Chronic hematic cyst of the orbit. Role of magnetic resonance imaging in diagnosis. Ophthalmology. 1988;95 (11): 1549-53.

46.Tsiouris AJ, Deshmukh M, Sanelli PC, Brazzo BG. Bilateral dacryops: correlation of clinical, radiologic, and histopathologic features. AJR Am J Roentgenol. 2005;184(1):321-3.

47.Bullock JD, Fleishman JA, Rosset JS. Lacrimal ductal cysts. Ophthalmology. 1986;93(10):1355-60.

48.Brownstein S, Belin MW, Krohel GB, Smith RS, Condon G, Codere F. Orbital dacryops. Ophthalmology. 1984;91(11):1424-8.

49.Khoury NJ, Haddad MC, Tawil AN, Ma'luf RN. Ductal cysts of the accessory lacrimal glands: CT findings. AJNR Am J Neuroradiol. 1999;20(6):1140-2.

50.Wuebbolt GE, Zuercher M, O'Donnell B, Collin R. Epithelial implantation cysts of the upper eyelid after lid-lowering procedures. Ophthalmology. 1993;100(9):1289-92.

51.Mansour AM, Cheng KP, Mumma JV, Stager DR, Harris GJ, Patrinely JR, Lavery MA, Wang FM, Steinkuller PG Congenital dacryocele. A collaborative review. Ophthalmology. 1991;98(11):1744-51.

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The prevalence of parasitic cysts of orbit varies greatly in different regions of the world. For example cysticercosis which is endemic in Asia, Latin America and Africa,1 is rare in North America and European Countries. Nearly 15% of proptosis we treat is due to cysticercosis! Hydatid is the next common parasitic cyst. However, in view of the wide spread international travel, it is essential to know about these conditions, as revealed by the fact that many cases of neurocysticercosis were reported in USA.2,3

Cysticercosis: Cysticercosis is the larval form of Taenia Solium. Man is the definitive host for Taenia Solium. The larval form of cysticercosis occurs in infested pig, which is the intermediate host. Cysticercosis occurs in humans when they take contaminated food like improperly cooked or uncooked vegetables or through water.

Cysticercosis can involve many organs and subcutaneous tissues. Neurocysticercosis is very common. Extraocular cysticercosis is more frequent than intraocular. We see 40-50 new cases of myocysticercosis per year while my vitreo-retinal colleagues see 4-6 cases a year. Most often we see solitary cysts. Multiple cysts or association with neurocysticercosis is rare.4,5 Very few cases of optic nerve involvement are reported.6,7,8

The clinical presentation of orbital cysticercosis is varied. The onset can be acute/sub-acute or intermittent. Pain and swelling can vary from severe to minimal. Ocular motility restriction and diplopia are very common. FDT is positive. The horizontal recti and superior rectus/LPS complex are commonly

involved. Most of the patients are young with an average age of 16.5 years. Myocysticercosis is the most common cause of acquired ptosis in the age group of 10-20 years in our series. A high degree of suspicion is needed to investigate and diagnose this condition. Anticysticercosis antibodies are positive in about 60% of cases. CT scan or Ultrasound B scan of orbit9,10 reveal enlarged muscle with a cyst showing a hyper dense spot within (which represents scolex). We prefer CT scan to B scan for the diagnosis, since we want to know if there is associated neurocysticercosis.

If neurocysticercosis also coexists, we refer the patient to neurologist. If there is no neurocysticercosis, we treat the patient with oral Albendazole 15 mg/ kg.body.wt/ day in 2 divided doses for 4 weeks along with prednisolone. In those with severe inflammatory signs, we prescribe Prednisolone at a dose of 2 mg/ kg.body wt/ day and in others at 1 mg /kg.body wt/ day, and will taper over a period of 4 weeks. We follow the patients with B scan, since it is less expensive and easily available.

Awareness of the disease, high index of suspicion and imaging are necessary to diagnose this condition. When the disease is for several months, and is not diagnosed and treated properly, the cyst migrates anteriorly . It can be seen in subtenon’s space, usually in relation to an extraocular muscle. Such cysts are excised, which on histopathology show a highly convoluted membrane with scolex. These patients are investigated for the presence of cysticercosis elsewhere and treated acoordingly.Rarely spontaneous extrusion may11 occur.

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When to suspect Myocysticercosis?

Young patient (5-20 yrs) Stay/visit to endemic region

Onset: Acute/Subacute/Intermittent Pain: Severe/moderate

Proptosis: Mild to moderate and eccentric Ocular motility: Restricted, FDT positive, Diplopia common

Soft tissue changes: Severe/minimal.

Investigations

Serum Anticysticercosis: Positive in about 60% cases only.

Imaging: CT/MRI preferred; Enlarged extraocular muscle with a cyst and scolex.

Treatment

Albendazole: 15mg/kg body wt/day in 2 divided doses × 4 weeks along with Prednisolone 1-2 mg/kg body wt/ day, tapered over 4 weeks.

in short time, symptoms not in proportion to the signs, myocysticercosis was our clinical diagnosis. Plain CT scan of Orbits revealed enlarged LPS-SR complex with a cyst and scolex (Figures 16.3 and 16.4), confirming our clinical diagnosis.

She was treated with Albendazole 400 mg bid for 4 weeks along with Prednisolone 90 mg/day (Her wt. was 45 kg) Since the inflammation was fairly severe as evidenced by severe edema of lids and markedly enlarged. LPS-SR complex. We started her on Prednisolone at 2 mg/kg.body wt./day and tapered over a months time. The response was quite dramatic as you can see in the Figure 16.5 which was taken 1 week after starting the medication.

CASE ILLUSTRATIONS

Case 1

Miss S, an young girl of 16 years in age was referred to us with the history of pain and swelling of right eye since 2 days. She had a similar episode 2 weeks back for which the referring doctor treated her with systemic antibiotics and she responded.

As you could see, (Figure 16.1) she presented with marked swelling of both the lids of her right eye, upper lid being more severe. She complains of pain of moderate intensity. There is a slight raise of local temperature, mild tenderness, minimal chemosis, mild proptosis and normal ocular motility (Figure 16.2).

We felt the chances of orbital infection are remote since it is difficult to explain why an infection recurs in such a short time in an otherwise healthy young woman. In view of the age, intermittency/ recurrence

Figure 16.1: Miss S, F16 presenting with severe edema of lids and swelling extending all round into periocular region of right eye

Figure 16.2: On elevating the eye lid, the eye looks normal but for mild chemosis and minimal proptosis

Figure 16.3: Axial CT image shows enlarged

LPS-SR complex enclosing a cyst

Figure 16.4: Coronal CT image showing a markedly enlarged LPSSR complex enclosing a cyst. The scolex( hyper-dense spot) within the cyst can be easily made-out

Figure 16.5: One week post treatment, the edema has almost subsided, the chemosis disappeared and proptosis reduced. There is a very marked clinical improvement

Case 2

Master V, a boy of 8 years presented with a swelling of right lower lid of 4 months duration, associated with mild pain. He gives history of occasional episodes of dull retrobulbar pain for which he did not consult any doctor.

On examination a cystic lesion, 15 mm × 12 mm in size and involving lower eyelid of right eye was noticed (Figure 16.6). It is getting more prominent when the child is looking up, and less prominent when the child looks downwards, demonstrating its relation to inferior rectus.

Transillumination was positive (Figure 16.7). The eye was very quiet. Ocular motility was normal CT scan of orbit revealed cysticercosis cyst with scolex (Figure 16.8). Since the cyst is anteriorly located, and is very easily accessed, it was excised through conjunctival approach. We found that most often the cysticercosis is encased in a wall which is about 2 mm thick and is very closely related to the extraocular

Parasitic Cysts of Orbit 209

muscle. It is not necessary to remove the cyst along with the casing. We prefer to de-roof the surrounding casing. Live cysticercus cyst usually shows motility. In fact, most often, it tries to come-out after an incision is made in the casing and tries to wriggle-out! These movements can be very well appreciated with the operating microscope.

Normally there is no need to use a cryo for the extraction of the cysticercus, unlike in hydatid cyst, wherein the use of cryo is mandatory. The excised cyst showed a dense spot which was the scolex (Figure 16.9).

Histopathology revealed convoluted membrane (Figure 16.10)

Figure 16.6: Large cyst involving right lower lid

Figure 16.7: Transillumination of the cyst

210 Surgical Atlas of Orbital Diseases

Figure 16.8: CT Orbits showing cyst with scolex

Case 3

Baby H, a girl of 8 years presented with painful eccentric proptosis of left eye of 2 weeks duration. On examination fullness in supero-medial space with the globe pushed down and out and severe tenderness was present. Abduction was restricted (Figure 16.11).

In view of short duration, pain and inflammatory signs, inflammatory /infective etiology was suspected.

CT scan of the orbit revealed sub-periosteal abscess with a cystic lesion within (Figure 16.12).

Careful examination revealed a white spot within it representing scolex (Figure 16.13).

The subperiosteal abscess was drained through superior Lynch incision. Along with pus, a cysticercosis cyst also was removed (Figures 16.14 and 16.15). It is the first and the only case of cysticercosis involving the subperiosteal space that we encountered. It is one of the rarest presentations of

Figure 16.13: Cyst with scolex

Figure 16.14: Incision mark on first postoperative day

Figure 16.15: Excised cysticercosis

Figure 16.16: Microphotograph showing cysticercosis with suckers

Figure 16.17: One month postoperative picture showing normal restoration of movement

cysticercosis and the only one to the best of our knowledge and pub med search. The diagnosis was confirmed by histopathology (Figure 16.16).

The postoperative recovery was uneventful. The ocular motility was restored (Figure 16.17). Very faint incisional scar can be made out.

Case 4

Mrs V, female 32 years, presented with sudden diminution of vision of her right eye since 2 weeks, associated with mild pain. Examination revealed mild fullness of superior sulcus, RAPD, and normal ocular motility. Optic disc was normal. Vision was 20/400 (Figures 16.18, 16.19 and 16.20).

The possibility of retro bulbar neuritis was thought of. But to exclude any other pathology, CT imaging of orbit was performed which surprisingly revealed

212 Surgical Atlas of Orbital Diseases

cysticercosis involving the lateral rectus, abetting optic nerve at orbital apex (Figures 16.21 and 16.22). This is an unexpected diagnosis, since cysticercosis is rare after 20 years, and presenting like retro bulbar neuritis is very unusual.

She was treated with oral Albendazole(15 mg/kg. body wt. in 2 divided doses /day) for 4 weeks and Prednisolone at a dose of 2 mg/kg.body wt./day tapered over a period of 4 weeks. This high dose of prednisolone was used because of associated optic neuropathy.

The patient recovered very well. The patient's periocular fullness disappeared. The pain subsided (Figure 16.23). Her vision improved to 20/30. How ever she had a inferior arcuate scotoma (Figure 16.24).

CT imaging after 4 months showed the presence of a shrunken cyst and scolex could not be seen

(Figure 16.25), which means that the cyst died. The patient is doing well for the past 2 years.

This is a unique case scenario. Cysticercosis very rarely causes a toxic optic neuropathy. It is rare after 25 years of age. In fact at the age of 32 years, this patient is the oldest patient of cysticercosis we have come across so far. We never suspected the possibility of cysticercosis in this patient, but the hunch to get a CT helped in making correct diagnosis and provide correct treatment for this lady. We could restore her vision.

This case clearly illustrates that even those who regularly come across cysticercosis can also miss a case, unless they are extra careful.

Figure 16.18: Shows mild periocular fullness. Her vision was 20/ 400. Pupil was dilated and sluggish with a significant RAPD

Figure 16.21: Cyst with hyper dense spot representing scolex, involving lateral rectus at the orbital apex