Orbital schwannoma: a clinicopathologic study.

The aim of the study was to study the clinical, radiological and histopathological characteristics of orbital schwannomas. It is a retrospective study conducted at a tertiary eye care hospital. A review of histopathological records of the orbital tumors operated between 1993 and 2011 was done. The clinical, imaging and histopathological details of cases of orbital schwannoma were analyzed. Forty-nine cases of orbital schwannomas identified. The age ranged from 8 to 65 years with a female preponderance. The median duration of symptoms was 3 years. Computed tomography findings varied from a hypodense to hyperdense lesion with nil to marked contrast enhancement. USG demonstrated a defined lesion with variable internal reflectivity. Varied proportions of Antoni A and Antoni B areas were found on histopathology of the masses. Hypodense or cystic areas on imaging significantly correlated with Antoni B areas on histopathology. Orbital schwannoma is a rare tumor. The incidence of schwannoma in our institution is 6.5 %. Variable imaging features were found. The definite diagnosis can be established on the basis of histopathological and immunohistochemical studies.

Giant cell reparative granuloma of the orbit.

Giant cell reparative granuloma is a rare fibro-osseous benign lesion. Only a few isolated cases of giant cell reparative granuloma of the orbit have been reported previously. The authors report an interesting case of giant cell reparative granuloma of the orbit in an 8-year-old girl. Surgical resection was performed, and no recurrence was noted at 18-month follow up.

Bilateral microphthalmos with unilateral superior cyst in a child with autism and CHARGE syndrome.

A case of autism with CHARGE syndrome with microphthalmos and a superior colobomatous cyst arising from the optic disc is reported. A 7-year-old boy presented with a gradually increasing mass, involving the superior orbit and upper eyelid of right eye of 3 years' duration. Clinical examination revealed bilateral microphthalmos with typical iris coloboma, posterior synechiae, and cataractous lens. Imaging revealed bilateral optic disc colobomas with a superior cyst in the right orbit. Intraoperatively, a single cystic lesion was seen in the superior orbit arising from the optic disc region. Histopathology was suggestive of a colobomatous cyst, positive for glial tissue [glial fibrillary acidic protein (GFAP) positive] and neuroretinal elements (synaptophysin positive) on immunohistochemistry. Genetic analysis revealed a normal karyotype (46, XY).

Ectopic meningioma of the orbit.

Ectopic meningiomas within the orbit are very rare. Most of the previously reported cases were located along the medial wall. Here we report on three cases of ectopic meningiomas presenting as superomedial orbital masses along with their radiological and histopathological features. All three patients underwent surgical excision of the tumor via anterior orbitotomy.

Conjunctivodacryocystorhinostomy using a high-density porous polyethylene-coated tear drain tube.

To evaluate the outcome of conjunctivodacryocystorhinostomy using a high-density porous polyethylene (HDPP)-coated tear drain tube. Patients with epiphora due to a proximal lacrimal system block were included in a prospective interventional case study. A total of 22 eyes were treated with lacrimal bypass surgery using the HDPP-coated tube. On follow-up (12-41 months), 21 eyes had a patent well-positioned tube with subjective relief of epiphora. In one eye, a loose sleeve was noted during surgery. The tube dislodged postoperatively and was removed. A high success rate with only a few minor complications is achievable using a HDPP-coated tear drain tube for lacrimal bypass surgery. Long-term follow-up is required to look for tube blockage due to conjunctival or nasal mucosal overgrowth.

Comparison of olopatadine and fluorometholone in contact lens-induced papillary conjunctivitis.

PURPOSE: The purpose of this study was to compare the efficacy of olopatadine with fluorometholone in contact lens-induced mild to moderate papillary conjunctivitis. METHODS: A randomized, double-masked study was conducted. Eighty-five (n = 170 eyes) soft contact lens users with mild to moderate papillary conjunctivitis were enrolled. Patients were randomly assigned to three groups to receive olopatadine 0.1%, fluorometholone 0.1%, or both. All drugs were instilled twice daily for 8 weeks. Contact lens use was discontinued during initial 4 weeks of therapy and subsequently patients were prescribed monthly disposable lenses. Patients were followed up every 2 weeks, and variables assessed were symptoms and signs, tear film status, and intraocular pressures. RESULTS: Decrease in ocular redness, itching, and tearing along with improvement in contact lens tolerance was comparable in all the three groups. Olopatadine was more effective in reducing redness than fluorometholone at 8 weeks (P=0.01). Improvement in congestion and papillary reaction was comparable in all groups. There was a significant increase in tear break up time of more than 2 sec for fluorometholone and no significant increase for olopatadine. The olopatadine and fluorometholone groups had significant increase of more than 2 mm in Schirmer test and more than 3 sec in tear break up time. In patients with subnormal and borderline tear functions, significant improvement was observed with both drugs. After 8 weeks of use of fluorometholone, there was a significant increase in intraocular pressure (P=0.003). CONCLUSIONS: Olopatadine and fluorometholone were the most effective for papillary conjunctivitis followed by olopatadine monotherapy and then fluorometholone monotherapy. Olopatadine is effective in alleviating signs and symptoms of contact lens-induced mild to moderate papillary conjunctivitis and is comparable with fluorometholone in efficacy.

Periorbital necrotising fasciitis in infants: Presentation and management of six cases.

PURPOSE: To present the clinical features and management of infants presenting with periorbital necrotising fasciitis (NF). METHODS: Retrospective case series. RESULTS: Six children were studied. The age at presentation was in the range of 5-11 months (median, 8 months). All children presented with acute onset eyelid inflammation and necrosis with fever, lethargy and poor oral intake. The management included intravenous antibiotics and repeated surgical debridement. The infection healed by 2-3 weeks in all cases, resulting in cicatricial ectropion and lagophthalmos. Full thickness skin grafting (with a Hughes tarso-conjunctival graft in one child) was performed in all patients at 3-5 weeks subsequently. Repeat surgery was required in three children. Adequate globe coverage and cosmesis was achieved in five children. CONCLUSION: NF of eyelids is a potentially fatal infection that requires urgent and vigorous management and heals with sequelae that may need more than one surgical intervention over a period of time. Adequate cosmetic and functional outcomes can be achieved.

Clinical misdiagnosis of retinoblastoma in Indian children.

AIM: To determine the rate of clinical misdiagnosis in paediatric patients who were enucleated for retinoblastoma and report the clinicopathological features of cases that were misdiagnosed. METHODS: Retrospective review of medical records of children who underwent a primary enucleation for advanced retinoblastoma was done. In all cases, the diagnosis of retinoblastoma was made on the basis of clinical presentation and imaging modalities. Clinicopathological features of eyes with discordant clinical and histopathological diagnosis were studied in detail. RESULTS: Of 280 eyes (280 patients) that were enucleated over a 4-year period, histopathological diagnosis was consistent with retinoblastoma in 276 (98.6%) eyes. In 4 (1.4%) eyes, clinical and histopathological diagnoses were discordant. Histopathological features in misdiagnosed cases included one case each of granulomatous endophthalmitis, retinal astrocytoma, Coats' disease and persistent hyperplastic primary vitreous. CONCLUSIONS: To the best of our knowledge, this is the first study to examine the rate of clinical misdiagnosis of retinoblastoma from South Asia. Despite the use of modern preoperative imaging modalities including MRI scans, benign lesions in end-stage conditions simulated retinoblastoma, resulting in potentially avoidable enucleation.

Primary liposarcoma of the orbit.

Liposarcoma is the most common sarcoma in adults, but is an extremely rare tumor of the orbit. We report 4 cases of primary orbital liposarcoma treated at our center. All cases had tumor localized to the orbit and underwent exenteration followed by radiotherapy in one patient. None of the patients had a recurrence until a follow-up of 1-5 years (mean: 4 years).

Orbital tuberculosis in a paediatric population.

PURPOSE: To report the clinical profile and management of orbital tuberculosis (TB) in children. METHODS: Eight cases were studied retrospectively. RESULTS: Ages in the range of 3-16 years. Three cases presented with discharging sinus in upper lid, three with a cystic mass and two with lid necrosis. Underlying bony changes were found in five patients. Drainage and curettage was done for five patients, needle drainage of fluid was done in one patient and in two patients local debridement was done. Polymerase chain reaction for tuberculosis was positive in four cases and acid fast bacilli (AFB) were isolated on culture in three cases. On histopathology, six cases had granulomatous inflammation with caseating necrosis in one, though AFB could not be found. A favourable response to anti-tubercular treatment was achieved in all cases. CONCLUSION: High index of suspicion is required for diagnosis of orbital TB. Microbiological and pathological diagnosis may not be achieved in all cases.

Role of oral corticosteroids in orbital cellulitis.

PURPOSE: To evaluate the role of oral corticosteroids as an anti-inflammatory adjunct in the treatment of orbital cellulitis. DESIGN: Prospective, comparative, single-masked, interventional clinical study. METHODS: setting: Tertiary eye care center (All India Institute of Medical Sciences). study population: Patients with acute onset (within 14 days) of orbital cellulitis with or without abscess. intervention: Patients were randomized into 2 groups in the ratio of 1:2. Both groups received initial intravenous antibiotics. In Group 2, oral steroids were added after an initial response to intravenous antibiotics. main outcome measures: Resolution of signs and symptoms, duration of intravenous antibiotics, length of hospital stay, and sequelae of disease (ptosis, proptosis, and movement restriction) were evaluated and compared between the 2 groups. RESULTS: A total of 21 patients (age range, 11-59 years) with orbital cellulitis were studied. There were 7 patients in Group 1, who received standard intravenous antibiotics, and 14 in Group 2, who received adjuvant steroids. Patients in Group 2 showed an earlier resolution of inflammation in terms of periorbital edema (P = .002 at day 7), conjunctival chemosis (P < .001 at day 10), and pain (P = .012 at day 7). They also attained vision of 0.02 on logMAR earlier than Group 1 patients. Decrease in proptosis and improvement in extraocular movements were also significantly better with the use of steroids (P = .027 at day 10, P = .003 at day 14, respectively). While a significant number of patients in Group 1 had mild residual ptosis, proptosis, and movement restriction at 12 weeks, none of the patients treated with steroids had any residual changes (P = .023, P = .001, and P = .001, respectively). The durations of intravenous antibiotics and hospital stay were significantly less in Group 2. CONCLUSION: Use of oral steroids as an adjunct to intravenous antibiotic therapy for orbital cellulitis may hasten resolution of inflammation with a low risk of exacerbating infection.

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.

Sentinel lymph node biopsy in malignant eyelid tumor: hybrid single photon emission computed tomography/computed tomography and dual dye technique.

PURPOSE: To study the utility of hybrid single photon emission computed tomography / computed tomography (SPECT/CT) scan and dual-dye technique in identification of the sentinel lymph node (SLN) in patients with an advanced malignant eyelid tumor. DESIGN: Nonrandomized prospective interventional study. METHODS: setting: A tertiary eye care center. study population: Patients with an advanced malignant eyelid tumor without clinically involved regional lymph nodes. intervention: SLN biopsy was performed using dual-dye technique (a combination of radiotracer and vital blue dye) following localization by SPECT/CT. main outcome measures: Localization of SLN in the regional node basin by hybrid SPECT/CT scan; SLN identification rate using dual-dye technique; SLN positivity rate; false-negative rate; and complications, if any, of SLN biopsy. RESULTS: Sixteen patients of biopsy-proven eyelid malignancy (7 squamous cell carcinomas [43.75%], 5 sebaceous cell carcinomas [31.25%], and 4 malignant melanomas [25%]) were included in the study. Preoperative localization of SLN was performed using SPECT/CT in 12 patients. SLN biopsy using dual-dye technique was performed in 16 patients. SPECT/CT accurately localized SLN in 11 out of 12 patients. The preauricular region was the most common site of SLN. SLN identification rates for dual-dye, radiotracer, and blue dye techniques were 100% (16/16 patients), 100% (16/16 patients), and 87.5% (14/16 patients), respectively. SLN showed metastasis in 2 patients (12.5%). On follow-up, 1 patient developed cervical lymph node metastasis, thus giving a false-negative rate of 7.14%. There were no complications associated with SLN biopsy. CONCLUSION: Accurate preoperative localization of SLN in relation to adjacent anatomic structures using SPECT/CT aids in intraoperative identification of SLN. SLN biopsy should be considered in patients with eyelid tumors at significant risk for metastasis who have clinically negative nodal basins. Dual-dye technique is safe and feasible in advanced eyelid tumors. Blue dye technique can be used for SLN biopsy in settings where nuclear medicine facilities are not available, albeit with a lower SLN identification rate. Detection of metastasis in SLNs in ∼12% of cases emphasizes the utility of SLN biopsy in accurate staging and treatment of eyelid malignancies.

Infantile hemangiopericytoma of the orbit treated with primary chemotherapy.

A 7-month-old female infant presented with proptosis of the left eye. Imaging showed an intraconal mass with marked post-contrast enhancement. Histopathology revealed a diagnosis of infantile hemangiopericytoma. The patient was treated with primary chemotherapy with no recurrence until 14 months after completion of treatment.