Correspondence were found to be associated with progressive peripheral corneal thinning and astigmatism in the axis of the lesions.9 Ring pigmentation in the peripheral cornea may also arise secondary to Wilson disease, non-Wilsonian chronic liver disease, hypercarotenemia (hypervitaminosis A), or ocular chrysiasis.10–12 Typically, hypercarotenemia presents with circumferential involvement of the peripheral deep stroma with yellow-brown pigment.11 Corneal involvement in Wilson disease is characterized by bilateral peripheral golden-brown, blue-green, or ruby red homogeneous deposits at the level of Descemet membrane.10 Impaired copper metabolism may also lead to similar deposits in non-Wilson hepatic disease.10 In contrast, diffusely scattered dotlike deposits in peripheral and central stroma is observed in ocular chrysiasis.13 By providing microscopic details of corneal deposition, IVCM may be helpful in the differential diagnosis of such disorders. Our case describes a patient with bilateral conjunctival pigmentation and annular corneal pigmentation in addition to arthropathic changes and skin pigmentation. Using IVCM, we were able to demonstrate the extent, level, and nature of corneal involvement in ochronosis. IVCM revealed highly reﬂective, homogeneous deposits most dense at the level of Descemet membrane and scattered microdeposits at Bowman layer with sparing of remaining corneal layers, including epithelium, stroma, and endothelium. To the best of our knowledge, our report is the ﬁrst one in the literature to detail the in vivo ultrastructural corneal characteristics of ochronosis using IVCM. Because ocular involvement may be the ﬁrst sign of alkaptonuric ochronosis, it is important to make the differentiation from other pigmented lesions of the eye. In these settings, IVCM is a valuable diagnostic tool that may assist in detection and exact localization of pathologic deposits in the cornea and help to establish the correct diagnosis in such patients.
Intraosseous orbital hydatid cyst: report of a rare case A 69-year-old female came to Oculoplastic Clinic, Farabi Eye Hospital, Tehran, Iran, with the complaint of newonset proptosis and loss of vision in her left eye from 4 months ago. She had no history of any trauma. On examination, there was no visible skin scar of any previous surgery around the orbit. The right eye had best corrected visual acuity (BCVA) of 9/10, and slit-lamp examination of its anterior segment and funduscopy showed no pathology except mild cataract. In her left eye, she had BCVA of 2/10, mild conjunctival injection and chemosis, severe proptosis (10-mm difference in exophthalmometry; Fig. 1A), lateral gaze limitation, and a reactive pupil with 2þ relative afferent pupillary defect.
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Sibel Kocabeyoglu, Duygu Sevim, Mehmet C. Mocan, Murat Irkec Hacettepe University School of Medicine, Ankara, Turkey Correspondence to: Murat Irkec, MD: [email protected]
REFERENCES 1. La Du BN, Zannoni VG, Laster L, et al. The nature of the defect in tyrosine metabolism in alcaptonuria. J Biol Chem. 1958;230:251-60. 2. Albers SE, Brozena SJ, Glass LF, Fenske NA. Alkaptonuria and ochronosis: a case report and review. J Am Acad Dermatol. 1992;27:609-14. 3. Gaines JJ. The pathology of alkaptonuric ochronosis. Human Pathol. 1989;20:40-6. 4. Kenny D, Ptacin MJ, Bamrah VS, Almagro U. Cardiovascular ochronosis: a case report and review of the medical literature. Cardiology. 1990;77:477-83. 5. Duke-Elder. Tomo VIII System of Ophthalmology. Part 2. London: Henry Kimpton; 1965: 1064-8. 6. Chévez Barrios P, Font RL. Pigmented conjunctival lesions as initial manifestations of ochronosis. Arch Ophthalmol. 2004;122:1060-3. 7. Skinsnes OK. Generalized ochronosis. Report of an instance in which it was misdiagnosed as melanosarcoma, with resultant enucleation of an eye. Arch Pathol (Chic). 1948;45:552-8. 8. Carlson DM, Helgeson MK, Hiett JA. Ocular ochronosis from alkaptonuria. J Am Optom Assoc. 1991;62:854-6. 9. Cheskes J, Buettner H. Ocular manifestations of alkaptonuric ochronosis. Arch Ophthalmol. 2000;118:724-5. 10. Liu M, Cohen EJ, Brewer GJ, Laibson PR. Kayser-Fleischer ring as the presenting sign of Wilson disease. Am J Ophthalmol. 2002;133:832-4. 11. Rahmani B, Jampol LM, Feder RS. Clinicopathologic reports, case reports, and small case series: peripheral pigmented corneal ring: a new ﬁnding in hypercarotenemia. Arch Ophthalmol. 2003;121: 403-7. 12. Kincaid MC, Green WR, Hoover RE, Schenck PH. Ocular chrysiasis. Arch Ophthalmol. 1982;100:1791-4. 13. Lopez JD, del Castillo JM, Lopez CD, Sanchez JG. Confocal microscopy in ocular chrysiasis. Cornea. 2003;22:573-5. Can J Ophthalmol 2014;49:e38–e40 0008-4182/14/$-see front matter & 2014 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2013.12.005
The left eye had also mild cataract, and its funduscopy showed no signiﬁcant abnormal ﬁnding. Radiologic evaluation for the eye was started with CT scan (Fig. 1B). CT scan revealed a well-deﬁned, dumbbellshaped cystic lesion—internal density similar to water, delineated by a perceptible hyperdense rim—in lateral wall of orbit, with destruction of the bone and extension into the orbit, compressing orbital contents and pushing the globe forward. For further evaluation of the lesion, magnetic resonance imaging (MRI) was performed (Fig. 1C, 1F). MRI showed a multiloculated cystic lesion with better depicting peripheral ﬁbrous capsule and internal septations. The lesion had low signal intensity in T1-wighted imaging without enhancement and high signal intensity in T2-weighted images.
Fig. 1 — Patient photograph showing left eye proptosis (A). Orbital axial CT scan showing a well-defined homogeneous hypodense (similar to water or CSF density) lesion and its peripheral capsule with increased density (similar to soft tissue). The lesion is located inside the zygomatic bone in lateral wall of orbit, extended into orbit pushing the globe forward (B). T2weighted axial (C) and sagittal (D) MRI showing the same well-defined lesion that has hypersignal intensity similar to water or CSF. Sometimes the fibrotic capsule can be seen as a peripheral hypointense ring around the lesion (D). T1-wighted axial MRI showing the lesion with hyposignal intensity (E). The lesion shows some internal septations. T1-weighted coronal MRI with contrast (F), which does not have complete fat suppression, so an appropriate judgement is not possible about any enhancement in the lesion’s capsule. The lesion shows no internal enhancement.
The signal intensity of the lesion was consistent with an isolated multiloculated cystic lesion with content density similar to water or cerebrospinal ﬂuid (CSF), with no internal enhancement, which could be a hydatid cyst, a dermoid cyst, an abscess, a mucocele, a hematocele, or an encephalocele. However, in T1-weighted images, hypersignal intensity is expected in dermoid cysts (because of their fat content) and hematocele (because of methemoglobin inside them). Moreover, abscess usually has a notable wall enhancement in
contrast studies, whereas thin peripheral enhancement is sometimes evident in mucocele and hydatid cyst, mostly as a result of superinfection. In the case of encephalocele, a cranial defect, and hence a connection with intracranial extra-axial CSF space, could be depicted. With these ﬁndings, the most probable diagnosis was an intraosseous hydatid cyst. Considering its compressive effect, the lesion had to be excised as soon as possible. Surgical en bloc excision of the cyst is the mainstay of treatment. To avoid intraorbital
Fig. 2 — Surgical approach through an incision 1.5 cm posterior to lateral orbital wall rim (A). Completely removed hydatid cyst (B). CAN J OPHTHALMOL — VOL. 49, NO. 2, APRIL 2014
Fig. 3 — Histopathologic view of the patient’s lesion showing laminated hyalinized cystic wall of hydatid cyst (A) attached to some scolexes (B). Hematoxylin and eosin stains. Original magnification 100 (A); 400 (B).
dissemination of the cyst’s content, considering its location, we approached through an incision 1.5 cm posterior to lateral orbital rim (Fig. 2A). Although the cyst ruptured during last step, there was no spillage into orbital space and it could be totally removed (Fig. 2B). Pathology conﬁrmed hydatid cyst (Fig. 3A, 3B). After surgery, albendazole was prescribed for the patient for 3 months. After 2 years of follow-up, no sign of recurrence has been detected in this patient. Brain imaging was performed for evaluation of any intracranial extension or dissemination, which was negative. To assess whether the patient had pulmonary hydatidosis, we performed a chest x-ray, which showed no ﬁnding. Also, abdominal ultrasonography was performed to evaluate the liver, which showed no abnormal ﬁnding. Echinococcosis is one of the most common parasitoses that affect humans in its endemic areas. When contaminated, it mostly affects liver, lung, and kidneys.1,2 Orbital involvement is rare, and only about 1% of hydatidoses occur in orbit.3,4 It has been reported that hydatidosis accounts for only 1% of orbital tumours2; but among orbital cystic lesions, it has been the second most frequent lesion (25.8%) after dermoid cysts (29.7%).5 Although in endemic areas such as Spain, Lebanon, Iraq, and Turkey, the incidence rate of hydatid cyst has been reported to be 13.6%, 6%, 20%, and 6.6% among orbital tumours, respectively.6 In North America, Echinococcus granulosus is rarely reported in Canada and Alaska, and a few human cases have also been reported in Arizona and New Mexico in sheep-raising areas. In the United States, most infections are diagnosed in immigrants from countries where cystic echinococcosis is endemic.7 Orbital hydatidosis mostly affects children and young adults. This occurs most frequently when individuals handle or contact infected dogs or other infected carnivores, or inadvertently ingest food or drink contaminated
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with fecal material that contains tapeworm eggs. In our case, we think that she has ingested vegetables possibly contaminated with fecal material of an infected dog. It is usually unilateral with a trend for left eye involvement,3 and occurs with an insidious onset, causing slowly progressive proptosis, which mostly leads to visual loss and limited eye movements.1,3,4,8 There can be sudden symptoms, for example, complete loss of vision, proptosis, pain, among others, caused by spontaneous cyst rupture. These lesions are usually not associated with hydatid cysts of other organs3; however, accompanied involvement of liver, lung, brain, spleen, and thyroid has been reported.9 Thus, chest x-ray and abdominal ultrasonography should be performed as a routine work-up for all patients.1 About 2% of hydatid cysts have been reported to be located in skeleton,9 and there is small number of case reports on intraosseous hydatid cysts,10–13 although periorbital bone involvement has not been reported. Eosinophilia can be seen in a few patients. Although serologic tests are generally positive in systemic hydatidosis, they are almost always negative in orbital hydatidosis, and thus are not reliable.1,9 Imaging studies including CT scan and MRI are now standard diagnostic evaluation for these lesions.1,3 On CT scan, they are shown as welldeﬁned encapsulated cystic lesions with internal density of water or CSF, which can show increased peripheral density at its capsule area (a hyperdense rim); this can show rim enhancement with contrast.6,9 These lesions can result in bone destruction (mostly medial wall into nasal cavity).6,14 On MRI, we can see the lesion as a well-deﬁned cystic mass with low-intensity signal in T1-weighted imaging, which can show peripheral rim enhancement with contrast and high internal signal intensity, and a hyposignal peripheral rim (caused by ﬁbrous component of the capsule) in T2-weighted images.9
Correspondence Surgical en bloc resection of the cyst is the mainstay of treatment and it can be curative if we are sure there has been no spillage of the contents intraoperatively3; however, it frequently has leakage through small punctures in the cyst wall, made during the procedure, which necessitates antihelmintic therapy, albendazole or mebendazole, postoperatively to reduce recurrence.1,3,8,9 Also, preoperative medical therapy has been advised to sterilize the lesion before the procedure, causing reduced risk for complications such as anaphylaxis and recurrence.1 For intraosseous lesions, extensive bony curettage is suggested.12 Irrigation of the affected area with hypertonic saline has also been advised, especially in the case of spillage.8,9,11 One important key in managing these patients is to have regular follow-up visits to detect any recurrence, especially in adjacent organs.1,11,12 In conclusion, we should consider the hydatidosis as a cause of progressive unilateral proptosis and visual loss in its endemic areas, which can be diagnosed with the help of imaging studies. Immediate intervention should be performed because of its compressive effects, and its recurrence must always be kept in mind, which can result in poor visual outcomes. Mohammad Taher Rajabi, * Marjan AkbariKamrani, * Mohammad Hadi Gharib, * Mohammad Bagher Rajabi, * Fahimeh Asadi Amoli, * Yalda Abrishami, * James Oestreicher †
* Eye Research Center, Farabi Eye Hospital, Tehran University of Medical Sciences, Tehran, Iran; †Department of Ophthalmology and Vision Sciences, University of Toronto, Toronto, Ont.
Correspondence to: Yalda Abrishami: [email protected]
Conjunctival squamous cell carcinoma with intraocular invasion after radiotherapy in epidermodysplasia verruciformis Conjunctival invasive squamous cell carcinoma (SCC) is the most common malignancy of the conjunctiva in the world.1 Incidence of this disease varies from 0.02 to 3.5 cases per 100,000 population per year.2 Although relatively uncommon, the disease carries potentially devastating ocular morbidity, and if neglected and not treated promptly can cause mortality. Epidermodysplasia verruciformis (EV) is a rare genetic lifelong disease that was ﬁrst described by Lewandowsky and Lutz3 in 1922 and is associated with a profound susceptibility to cutaneous infection with certain types of human papillomavirus (HPV). These strains of HPV were termed EV-HPV types because they were originally believed to occur only in association with the disease. Several HPVs, such as HPV-5, -8, -9,-12, -14, -15, -17, -19, -25, -36, -38, -47, and -50, have been identiﬁed, and
REFERENCES 1. Siddiqui MA, Rizvi SW, Rizvi SA, et al. Atypical multifocal hydatid disease of cranial vault: simultaneous orbital and extradural meningeal involvement. Emerg Radiol. 2010;17:427-30. 2. Aouchiche M, Benrabah R, Abanou A, et al. [Computed x-ray tomographic aspects of intra-orbital hydatid cyst. Apropos of 10 cases]. [Article in French] J Fr Ophtalmol. 1983;6:901-16. 3. Limaiem F, Bellil S, Bellil K, et al. Primary orbital hydatid cyst in an elderly patient. Surg Infect (Larchmt). 2010;11:393-5. 4. Danziger A, Price H. Computed tomographic ﬁndings in orbital echinococciasis. J Comp Assist Tomogr. 1980;4:128-9. 5. Günalp I, Gündüz K. Cystic lesions of the orbit. Int Ophthalmol. 1996-1997;20:273-7. 6. Kars Z, Kanzu T, Ozcan O, et al. Orbital echinococcosis, report of two cases studied by computerized tomography. J Clin Neuroophthalmol. 1982;2:197-9. 7. Centers for Disease Control and Prevention. Cystic echinococcosis. http://www.cdc.gov/parasites/echinococcosis/gen_info/ce-faqs.html. 8. Benazzou S, Arkha Y, Derraz S, et al. Orbital hydatid cyst: review of 10 cases. J Craniomaxillofac Surg. 2010;38:274-8. 9. Turgut A, Turgut M, Kosar U. Hydatidosis of the orbit in Turkey: results from review of the literature 1963–2001. Int Ophthalmol. 2004;25:193-200. 10. Erman T, Tuna M, Göçer I, et al. Intracranial intraosseous hydatid cyst. Case report and review of literature. Neurosurg Focus. 2001;11: ECP1. 11. Yazdani N, Basam A, Heidarali M, et al. Infratemporal hydatid cyst: a case presenting with blindness. J Laryngol Otol. 2010;124:456-9. 12. Pelegri C, Gaertner E, Bernard E, et al. Recurrence of femoral echinococcosis 5 years after a primary surgical procedure. Orthop Traumatol Surg Res. 2010;96:94-6. 13. Fyfe B, Amazon K, Poppiti RJ Jr, et al. Intraosseous echinococcosis: a rare manifestation of echinococcal disease. South Med J. 1990;83:66-8. 14. Murthy R, Honavar SG, Vemuganti GK, et al. Polycystic echinococcosis of the orbit. Am J Ophthalmol. 2005;140:561-3. Can J Ophthalmol 2014;49:e40–e43 0008-4182/14/$-see front matter & 2014 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2013.12.006
they are now known to be present both in several types of benign and malignant cutaneous lesions and in normal skin in the unaffected population.4 Only a few types of HPV, speciﬁcally HPV-5 and HPV-8, as well as HPV-14, have been implicated in malignant transformation of EV lesions.5 It appears that T cells and natural killer cells in patients with EV may have a decreased response to speciﬁc HPVs. Interestingly, these patients do not appear to be at increased risk for development of other viral or bacterial infections.6 For affected individuals, classic EV ﬁrst appears during early childhood. Polymorphic cutaneous lesions, including ﬂat, wartlike papules and pityriasis versicolour-like macules, persist. Approximately half of all patient conditions may transform to cutaneous malignancies such as Bowen’s type carcinoma in situ and invasive SCC, usually in sunexposed areas, in the fourth or ﬁfth decade of life.7 These malignancies have been reported to occur on the forehead, lips, and periorbital areas, and tend to be locally CAN J OPHTHALMOL — VOL. 49, NO. 2, APRIL 2014