Mridula Mehta

Orbital schwannoma with cystic degeneration.

Background:  Schwannoma is a benign, slowly growing, painless, peripheral nerve sheath tumour that is uncommon in orbit. Longstanding tumours may develop degenerative changes. Extensive cystic change in orbital schwannoma is quite rare. We conducted this study to analyse the cases of orbital schwannoma, which were predominantly cystic and describe their clinical, imaging and histopathological features.

Clinical and pathologic profile of angiomyxomas of the orbit.

Purpose: To describe clinical, imaging, and histopathologic features of angiomyxomas of the orbit, which are extremely rare tumors in the orbit. Methods: A retrospective review of clinical case records and imaging findings of histopathologically diagnosed cases of angiomyxoma over a period of 8 years (2001–2008). The histopathologic features were studied by routine hematoxylin-eosin staining, special stain (Alcian blue), and immunohistochemistry. Results: Four cases (2 male and 2 female) with a mean age of 35 years (range, 28–40 years) were diagnosed as angiomyxoma. Two of these were angiomyxomas, and 2 were aggressive angiomyxomas. All patients presented with gradual progressive proptosis. The mean duration of symptoms was 10.5 months (range, 5–24 months). There was associated reduction in the visual acuity in 2 cases. The superior orbit was involved in all 4 patients. CT scan showed a heterogeneously enhancing irregular mass confined to the superior orbit. Surgical removal of the mass was performed in all 4 cases. On follow-up, there was recurrence at 6 months in 1 case, which was histopathologically diagnosed as an angiomyxoma. Conclusions: Angiomyxoma is an extremely rare, locally aggressive orbital tumor, occurring in the third to fourth decade of life. Complete excision is the treatment of choice. These tumors are likely to have a recurrence due to their infilterative growth and require long-term follow-up.

Medulloepithelioma of the ciliary body associated with massive intravitreal hemorrhage in an adult

Intraocular medulloepithelioma is a rare tumor of embryonal origin arising from primitive neuroepithelial cells. It occurs mainly in children and, to the best of our knowledge, only 13 cases have been reported in adults. None of these cases was associated with a large intravitreal hemorrhage. We herein report a case of malignant nonteratoid medulloepithelioma presenting in an adult with massive intraocular hemorrhage. A 38-year-old woman presented with pain, redness, and loss of vision in her left eye for 6 months. She had a past history of surgery for complicated cataract in both eyes, 2 years previously. The previous records revealed normal posterior segments. On examination, the vision was no light perception OS and 6/9 OD. A sentinel vessel was seen nasally, the iris was plastered to the cornea, and the intraocular pressure was raised. The right eye was normal. Ultrasonography revealed a mass of 12.7 mm × 8.8 mm superonasal to the disc, with choroidal excavation and vitreous hemorrhage. Magnetic resonance imaging (MRI) of the orbits showed 2 intraocular masses that were continuous in a few sections. One was in the region of the ciliary body and the second was posteronasal to it, involving the retina. No extraocular spread was noted (Fig. 1). Based on these findings, a presumptive diagnosis of choroidal melanoma was made, for which the patient underwent enucleation. Gross examination of the enucleated globe revealed a grayish mass in the vitreous cavity measuring 18 mm × 15 mm × 10 mm near the ciliary body that extended anteriorly up to the iris and posteriorly along the retinal surface. Posteriorly, it was joined by a second, slightly smaller mass. The entire vitreous cavity was filled with hemorrhage (Fig. 2). Microscopically, a tumor consisting of epithelial

Orbital teratoma: a rare cause of congenital proptosis

be associated with other ocular conditions like retinitis pigmentosa. Orellana et al. hypothesized that cysts arise from displacement and proliferation of the pigment epithelium originating from the pars plana and not from the iris. Asiyo-Vogel et al. postulated that the cysts formed from displaced retinal tissue, after retinal detachment surgery. In our patient the iris transillumination suggested that the iris cyst was formed from tissue originating from the iris root and dislodged into the vitreous cavity probably through the zonules; this was confirmed histopathologically. We believe that the occurrence of the full thickness macular hole was completely coincidental. Dislodged intravitreal iris cysts are a rare entity usually asymptomatic. Because of their rarity they may represent a diagnostic challenge to the ophthalmologist, especially when combined with other ocular disease.

Extraocular retinoblastoma in Indian children: clinical, imaging and histopathological features.

AIM TO STUDY EYES WITH EXTRAOCULAR DISSEMINATION (EORB), WITH THE FOLLOWING AIMS: first to establish the mean lag period and to understand various reasons for delayed presentation, second to study their imaging profiles and third to analyze histopathological features of eyes enucleated after neoadjuvant chemotherapy. METHODS Prospective study of clinical and imaging features of EORBs (stage III and IV International Retinoblastoma Staging System) presenting to a tertiary eye care centre. Histopathological features of eyes enucleated after receiving neoadjuvant chemotherapy were analyzed. A pictorial illustration of the varied imaging profile of EORB was also presented. RESULTS Over a period of one year, 97 eyes were diagnosed with retinoblastoma; 32 children (36 eyes) (37.1%) had EORB. Mean age 3.6±1.9 years, 71.9% males, 71.9% unilateral, 3.1% with positive family history and 40.6% with metastasis. On imaging, there was extrascleral involvement in 22.2%, involvement of orbital part of optic nerve in 33.3%, involvement of central nervous system in 27.8% and orbital wall involvement in 2.9% eyes. On histopathological analysis of eyes enucleated after neoadjuvant chemotherapy, 25.0% had no residual viable tumour tissue and rest all tumours were poorly differentiated. CONCLUSION There are very few human malignancies where definitive treatment is started without any confirmed histopathological diagnosis and imaging plays an important role in diagnosis and appropriate staging of the disease. Chemotherapy has a variable effect on EORB, 75.0% of eyes with EORB had residual viable tumour tissue when enucleated after receiving neoadjuvant chemotherapy.

Congenital horizontal tarsal kink with microphthalmos corrected by intermarginal suture tarsorrhaphy.

Congenital horizontal tarsal kink is extremely rare. Only one case of associated microphthalmos has been reported. Various surgical techniques have been described with successful results. Few reported cases have been managed with intermarginal suture tarsorrhaphy. The current case is probably the third patient with severe tarsal kink to be managed with this simple technique.

Retinoblastoma: still a challenge in developing countries

Editor, W e read with interest the editorial by Tero Kivelä ‘200 years of success initiated by James Wardrop’s 1809 monograph on retinoblastoma’ (Kivelä 2009). It has been indeed a long but fruitful journey from enucleation and external beam radiation therapy (EBRT) in the 1990s to the present era of chemoreduction. However, even after 200 years of Wardrop’s prediction of the importance of an early enucleation, we in the developing countries frequently come across the more deadly presentation of this morbid tumour, the extraocular retinoblastoma. At our hospital, which is a leading tertiary level referral hospital in northern India, of the total retinoblastoma cases registered in the year 2009, approximately 50% presented with extraocular spread (M. Mehta, S. Sethi, N. Pushker, B. Chawla and S. Ghose, unpublished data). Multiple factors could explain delayed presentation of extraocular retinoblastoma in developing countries (Antoneli et al. 2003; Orellana et al. 2009) of which late referral because of lack of education of the parents and awareness among practitioners might account for the delayed diagnosis (Antoneli et al. 2003). Extraocular retinoblastoma is a challenge for the ophthalmologist and the oncologist. Until recently, exenteration was a common mode of management for extraocular retinoblastoma, which is largely a mutilating surgery with major disfiguring sequelae. In the 1980s, considering the common neuroectodermal origin, this tumour shares with other embryonal tumours and the chemotherapeutic agents that were effective in neuroblastoma were given in extraocular retinoblastoma (Doz et al. 1994). However, the current concept is that of neoadjuvant chemotherapy to allow tumour reduction followed by enucleation or resection of residual mass followed by adjuvant chemotherapy and radiotherapy. Although chemotherapy and EBRT continue to be the mainstay in management of patients with extraocular retinoblastoma, the current data suggest that even with aggressive chemotherapy, survival rates are poor (Antoneli et al. 2003; Leal-Leal et al. 2006). Patients with dissemination into the central nervous system or those with systemic metastatic disease continue to die with disease progression and remain incurable despite aggressive management. Our experience shows that late referral, refusal of enucleation and incomplete treatment predict extraocular retinoblastoma. To improve survival and to improve vision, it is important to improve the referral system; this can be achieved by increasing awareness among general ophthalmologists and paediatricians of the early signs of retinoblastoma and the importance of timely referral and, above all, by outreach to the community and by increasing parent awareness.

Disseminated cysticercosis with multiple bilateral orbital cysts.

The authors report 2 cases of cysticercosis with widespread involvement of both orbits and subcutaneous tissue and muscles of the head, face, and neck. These cases highlight the severity of orbital involvement in patients with disseminated disease without signs of orbital infection. To the best of our knowledge, such an extensive involvement of both orbits has never been reported.

Malignant non-teratoid medulloepithelioma of the optic nerve with intraocular extension

Medulloepithelioma is an uncommon tumour. These tumours most commonly arise in the brain and spinal cord; however, they also may arise from the optic nerve. We, herein, report a rare case of malignant non‐teratoid medulloepithelioma of the optic nerve with intraocular extension.

Large orbital hydatid cyst presenting as subconjunctival mass

Cystic echinococcosis (CE; previously known as hydatid disease) represents one of the most common helminth infections of humans and usually involves parenchymatous organs such as the liver and lungs (Lewall, 1998; Kiratli et al., 2003; Stamatakos et al., 2009). Orbital involvement is extremely rare, only accounting for about 1% of all cysts (Gomez Morales et al., 1988; Gökcek et al., 1997). The correct preoperative diagnosis of CE is crucial in the surgical management of the disease. A patient with a large and isolated orbital hydatid cyst, appearing as a subconjunctival mass, is reported below, with the clinical, imaging and histopathological features of this rare presentation discussed in detail.