Diffuse preretinal infiltrates in a patient with orbital atypical T-cell lymphoproliferative infiltration masquerading posterior uveitis.

Purpose: To report an aggressive and rapidly progressive case of atypical T-cell lymphoproliferative infiltration both with intraocular and orbital involvement and preretinal infiltrates. Methods: Medical records and imaging of the patient were retrospectively reviewed. Case presentation: A 25-year-old woman presented first with preretinal infiltrates resembling uveitis and developed orbital and intracranial signs eventually during her evaluation. Clinical presentation worsened gradually. The patient developed bilateral proptosis, pupillary dilation and uvula deviation. Diagnostic orbital incision biopsy revealed T-cell lymphoproliferative disease. Conclusion: This case gives evidence that intraocular involvement due to T-cell lymphoproliferative disease may present as a masquerade syndrome and should be kept in mind in patients with extraordinary presentation.

Penetrating Keratoplasty in Congenital Glaucoma.

BACKGROUND: Childhood glaucoma is one of the most common causes of corneal opacity in childhood and is associated with various pathological corneal changes, including corneal enlargement, corneal clouding, and edema. Congenital glaucoma (CG) may cause a decrease in vision outcomes due to corneal opacity or clouding, which is often associated with stimulus deprivation amblyopia. Therefore, to create a balance between preventing amblyopia and sustaining corneal clearance, patients with CG can be managed with early penetrating corneal transplantation surgery along with advanced glaucoma management. AIM: To investigate the graft survival rate and factors affecting graft survival in patients with congenital glaucoma who underwent penetrating keratoplasty (PKP). STUDY DESIGN: Cross-sectional. MATERIALS AND METHODS: Patients with congenital glaucoma who underwent PKP were retrospectively evaluated. The associations between age, corneal diameter, presence of ocular comorbidities, concurrent ocular surgeries with corneal graft, and visual outcomes were assessed. RESULTS: Among the 30 eyes enrolled in the study, 6 (20%) had aniridia, 6 (20%) had Axenfeld-Rieger syndrome, and 18 (60%) were diagnosed with primary congenital glaucoma. Graft survival rates were 66.6% and 63.33% at 12 and 24 months, respectively. At the end of the follow-up, the overall graft survival rate was 60%. Statistical significance was observed between patient age at the time of surgery and graft failure (p = 0.02). Graft failure was associated with a younger patient age. Functional vision was achieved in 53.3% of patients. CONCLUSIONS: The management of congenital glaucoma and its corneal complications is a delicate issue that requires great effort. PKP in congenital glaucoma was moderately successful in the present study. To provide functional vision, PKP could be the treatment of choice.

Pseudomelanoma: occult intraocular foreign body mimicking choroidal melanoma.

Purpose: To report an occult intraocular foreign body mimicking choroidal melanoma. Methods: Medical records and imagings of the patient were retrospectively reviewed. Case description: A 76-year-old male was referred to our ocular oncology clinic with a suspicious hyperpigmented retinal lesion in the left eye. Biomicroscopy showed aphakia and peripheral iridectomy in the left eye. Fundoscopy revealed a pigmented, slightly elevated lesion on the macula of the left eye surrounded by diffuse atrophy. B-scan ultrasonography showed a preretinal hyperechoic lesion with posterior shadowing. There was no choroidal mass in B-scan or optical coherence tomography (OCT) imaging. On further questioning, it was disclosed that the patient had been hit by an iron fragment in the left eye forty years ago. Conclusion: Choroidal melanoma is a vision- and life-threatening intraocular malignant tumour. Various neoplastic, degenerative, and inflammatory conditions can simulate choroidal melanoma. A previous history of penetrating ocular trauma should lead the surgeon to re-evaluate a diagnosis of melanoma.

Multimodal imaging of a sporadic retinal astrocytic hamartoma simulating retinoblastoma in a newborn.

Introduction: To report a sporadic astrocytic hamartoma simulating retinoblastoma in a newborn. Methods: Clinical data was reviewed retrospectively. Results: A 3-month-old baby with a history of perinatal asphyxia was referred to our ocular oncology clinic with suspected retinoblastoma in the left eye. Dilated fundoscopy revealed a solitary tumor covering the optic disc at the left eye. The whitish-yellow lesion was well-defined, opaque, and minimally calcified. High internal reflectivity and posterior shadowing due to the intralesional calcification, and intratumoral cystic spaces were observed in B-scan ultrasound imaging. Optical coherence tomography imaging showed an intraretinal tumor with cystic spaces and posterior shadowing. The tumor was diagnosed as an astrocytic hamartoma. The systemic evaluation was negative for phacomatoses. The lesion has been observed with multimodal imaging for six years without significant changes. Conclusions: Retinal astrocytic hamartomas are benign tumors that arise within the retinal nerve fiber layer. Differential diagnosis constitutes high importance since they may be misdiagnosed as retinoblastoma, and therefore may be overtreated. Whereas retinoblastoma requires immediate treatment, retinal astrocytic hamartomas are commonly followed-up. Multimodal imaging with B-scan ultrasonography and optical coherence tomography are useful in distinguishing those two entities.

Corneal Endothelial Changes in Patients With Primary Congenital Glaucoma.

PRCIS: Specular microscopic parameters were found to be decreased in patients with primary congenital glaucoma (PCG). Patients with PCG and Haab striae had lower endothelial cell density (ECD) and central corneal thickness (CCT) than those without Haab striae did. The type of surgery (viscogoniotomy, viscotrabeculotomy, or combined viscotrabeculotomy and trabeculectomy) did not affect specular microscopic parameters. PURPOSE: The purpose of this study was to compare specular microscopic parameters such as corneal ECD, coefficient of variation (CV), hexagonal cell percentage (HEX), and CCT between patients with PCG and healthy controls; to determine the predictive ability of Haab striae for endothelial cell changes PCG by comparing patients with and without Haab striae; and to investigate if the type of surgery used to treat glaucoma (viscogoniotomy, viscotrabeculotomy, or combined viscotrabeculotomy and trabeculectomy) affects specular microscopic parameters. METHODS: A cross-sectional specular microscopic analysis of patients with PCG and healthy controls was performed. One hundred eyes of 62 patients with PCG with and without Haab striae and 101 eyes of 101 healthy controls were enrolled in this study. Specular microscopic parameters, including ECD, HEX, CV, and CCT, of all subjects were evaluated. RESULTS: Patients with PCG showed significantly lower ECD and CCT than healthy controls did. Those with Haab striae had lower ECD and CCT but no differences in age, HEX, and CV compared with those without Haab striae. ECD, CV, HEX, or CCT did not significantly differ among patients treated with the 3 different types of surgery. CONCLUSIONS: Prolonged exposure to elevated intraocular pressure during prenatal and/or early postnatal life results in structural changes in immature infant corneas. Specular microscopic parameters differed significantly between healthy controls and patients with PCG. The type of surgery had no effect on these parameters. Among patients with PCG, those with Haab striae had isolated corneal endothelial cell loss without morphologic changes within the endothelium.

An unusual ophthalmic presentation of Wolf-Hirschhorn syndrome.

Purpose: Wolf-Hirschhorn syndrome (WHS) is a rare inherited disease caused by the deletion in short arm of 4th chromosome. Various ocular manifestations in WHS have been described previously. We present an extraordinary clinical case of WHS associated with optic nerve head malformation and optic nerve sheath enlargement in the same eye.Methods: Case reportResults: A male infant was delivered by Caesarean section at 38 weeks with a birth weight of 2040 gr and admitted to neonatal intensive care unit due to multi-systemic abnormalities. The infant had multiple congenital anomalies; a cleft palate, microcephalia, micrognathia, renal pelvicalyceal ectasia, atrial septal defect, transvers arcus hypoplasia, patent ductus arteriosus, hypospadias and undescended testicle. Fundus examination revealed optic disc coloboma of both eyes. Two weeks later, at the second examination, the left optic disc margins were indistinct with vessels radiating from the disc margins which resembles morning glory disc anomaly (MGDA). The MRI demonstrated corpus callosum agenesis and a T1 hypointense, T2 hyperintense, 12 × 9 mm optic nerve sheath enlargement in the retrobulbar area.Conclusion: The case presented here demonstrates that, the optic nerve head malformations and optic nerve sheath enlargement may be due to incomplete closure of choroidal fissure and subsequent accumulation of cerebrospinal fluid may result in a spectrum of optic nerve head malformations.

Superselective intra-arterial chemotherapy treatment for retinoblastoma: clinical experience from a tertiary referral centre.

OBJECTIVE: To evaluate the treatment outcomes of an alternative retrograde superselective ophthalmic artery catheterization (intra-arterial chemotherapy [IAC]), while treating retinoblastoma patients. METHODS: A retrospective review of IAC for 21 treatment-naïve eyes (21 patients, primary group) and 10 eyes of previously treated 9 patients (secondary group). Statistical analysis was performed using Number Cruncher Statistical System 2007, Kaplan-Meier survival analysis, and Fisher's exact test. RESULTS: Total fluoroscopy time ranged from 3 to 12 minutes. Globe salvage was 71.4% (15/21 eyes) and 80% (8/10 eyes) in the primary and secondary groups, respectively. Globe salvage rates were recorded as 100%, 100%, 70%, and 0% for group B, C, D, and E patients, respectively. CONCLUSIONS: Retrograde IAC is effective in tumour control with shorter fluoroscopy time and acceptable complication rates both for naïve and treated patients. Furthermore, controlling retinoblastoma in advanced group D eyes was efficacious.