Diagnosis of Nephropathic Cystinosis in a Child During Routine Eye Exam.

We present a 7-year-old patient who was diagnosed with asymptomatic nephropathic cystinosis following the detection of the pathognomonic corneal white crystalline opacities during a routine eye examination.

Bilateral Subperiosteal Hematoma and Orbital Compression Syndrome in Sickle Cell Disease.

A 14-year-old boy with sickle cell disease presented with preseptal cellulitis findings as proptosis, eyelid edema, and hyperemia. His best corrected visual acuity in the right eye was 20/20 and 16/20 in the left eye. He had limited ductions in vertical and lateral gazes in both eyes. Bilateral venous tortuosity was observed in posterior segment examination. Orbital bone infarction and subperiosteal hematoma were seen in magnetic resonance imaging. He was diagnosed as having orbital compression syndrome secondary to vaso-occlusive crisis of sickle cell disease and was treated with intravenous ampicilin-sulbactam and methylprednisolone.

A rare cause of intermittent respiratory distress and epiphora in the newborn: congenital dacryocystocele.

Congenital dacryocystocele is a malformation of the nasolacrimal system. Most of the cases can be successfully treated with conservative treatment options such as sac massage. Congenital dacryocystoceles with concomitant intranasal cysts causing respiratory distress in the newborns are rarely published. In this case report we presented a 2-week-old newborn with bilateral congenital dacryocystoceles causing intermittent respiratory distress. We discuss the importance of nasal endoscopic treatment and multidisciplinary approach for these rare malformations in the light of the current literature.

A Rare Cause of Proptosis in Childhood: Langerhans Cell Histiocytosis.

A three-year-old male patient was admitted to the clinic with proptosis in his right eye. He had a history of fever with an unknown etiology. In examination, right proptosis was observed and an immobile mass was palpated at the lateral wall of the right orbita. Eye movement was unrestricted in all directions and anterior and posterior segment examination was normal in both eyes. On computed tomography, diffuse bone destruction and expansion was observed in the right orbital lateral wall and other cranial bones. Langerhans cell histiocytosis was diagnosed by bone biopsy. Malignancy is an important cause of proptosis in childhood. Pediatric patients who are admitted to clinic with proptosis should be carefully examined and Langerhans cell histiocytosis should also be considered as an etiology.

Simultaneous papilledema and optic disc drusen in a child.

Idiopathic intracranial hypertension is a headache syndrome characterized by elevated intracranial pressure with normal cerebrospinal fluid content, normal cranial imaging, and elevated appearance of the optic disc. We report on a 6.5-year-old boy with complaints of headache and right esotropia causing diplopia. A lumbar puncture indicated an opening cerebrospinal fluid pressure of 28 cm H(2)O. The headache, diplopia, and esodeviation resolved after the lumbar puncture. However, at 2-week follow-up, the elevated appearance of the optic disc continued despite normal cerebrospinal fluid pressure. A second ophthalmologic consultation revealed optic disc drusen, as also demonstrated by ocular ultrasonography. To date, two such cases have been reported in the literature. To our knowledge, this patient is the youngest with coexisting optic disc drusen and idiopathic intracranial hypertension.

Inducing ocular allergy with beta-lactoglobulin: a new experimental model.

PURPOSE: To develop a new experimental ocular allergy animal model induced by beta-lactoglobulin (BLG), a major cow's milk allergen, and to discuss the clinical, histopathological and immunohistochemical findings. METHODS: Forty Balb/c mice were randomized and separated into groups of 10. Groups were determined according to the different concentrations of BLG drops used. Study groups were immunized with 2.5, 5 or 10 mg/ml topical BLG (groups 2, 3 and 4, respectively) following intraperitoneal injection for the systemic immunization. The control group (group 1) was immunized with aluminium hydroxide (alum) alone within the same intervals. After ocular challenge, all the animals were evaluated clinically, histopathologically (mast cell and eosinophil infiltration) and immunhistochemically in terms of both T helper type 1- (IFNgamma, TNFalpha) and T helper type 2- (IL-3, IL-4, IL-5, IL-10) specific cytokines. RESULTS: Both clinical and immunohistochemical findings showed that an allergic conjunctivitis was induced in all study groups, with an optimized dosage of topical 5 mg/ml BLG. The conjunctivitis was associated with both Th1 and Th2 response, with a slight predominance of Th1 reaction. CONCLUSION: We describe a new murine model of acute allergic conjunctivitis induced by BLG. We believe that this new preliminary model has the immune parameters of the late phase of acute allergic conjunctivitis and it provides an alternative means for studying the pathogenesis and future treatments of ocular allergy. Our results should be enhanced with more detailed cellular and humoral parameters.

Effects of intravitreal bevacizumab in repeated doses: an experimental study.

PURPOSE: To evaluate the effects of repeated 1.25-mg intravitreal bevacizumab injections on cornea and uveoretinal tissues using histologic and biochemical analyses. METHODS: Twenty-four New Zealand albino rabbits were used. Twelve rabbits received an injection of bevacizumab in their right eyes three times with an interval of 25 days (Group 1); their contralateral eyes served as controls (Group 2). Six rabbits had an injection of vehicle in both eyes (Group 3), with the same regimen as bevacizumab, and six rabbits' eyes were used as a sham group (Group 4). Enucleated eyes were used for histologic and biochemical analyses, which included the activities of caspase 3 and 8 enzymes, glutathione content, catalase activity, and malondialdehyde content. RESULTS: No inflammation in aqueous humor and no sign of corneal or uveoretinal toxicity was found in bevacizumab-injected eyes. The difference of activity of corneal caspase 8 enzyme between Groups 1 and 2 and between Groups 1 and 4 was statistically significant (P < 0.05). In the uveoretinal tissue, in Group 1, the activities of caspase 3 and 8 enzymes were the lowest, and uveoretinal malondialdehyde content was also significantly lower than Group 4. CONCLUSION: A repeated dose of intravitreal bevacizumab injection did not cause a toxic effect on cornea and uveoretinal tissue. Biochemically, it also did not cause any apoptosis, oxidative reaction, or lipid peroxidation. Instead, bevacizumab injection caused a considerable decrease in the apoptotic enzyme activities and lipid peroxidation in the uveoretinal tissue. Further studies are needed to be conducted for possible detrimental side effects and apoptotic and oxidative effects of repeated bevacizumab injections on both the injected and the contralateral eyes.

Surgical treatment of an exposed orbital implant with vascularized superficial temporal fascia flap.

Orbital implants are often used for the correction of volume deficit after enucleation or evisceration. An orbital implant enhances aesthetics and improves mobility of the subsequent prosthetic eye. With advancements in technology and techniques, implant-related complication rates have been decreased. However, implant exposition as one of the most common complications of socket surgery is still a problem for the oculoplastic surgeon. Many factors are thought to cause orbital implant exposure: Large implants, tension on the wound, and textured surface of the implant may cause a breakdown of the covering layers over the implant. Inadequate fibrovascular ingrowth into the porous implant is the most important factor. Various surgical methods have been described to cover the defect, most offering the use of various tissues as a graft, which are not always satisfactory. We describe a case with silicone implant exposition that was managed with a vascularized superficial temporal fascia flap. According to our knowledge, this is the first article reporting the usage of a vascular tissue in the management of an orbital implant exposure.

Uncommon associations with ataxia-telangiectasia: vitiligo and optic disc drusen.

Ataxia-telangiectasia (A-T) is an autosomal recessive condition presented by progressive cerebellar ataxia, oculocutaneous telangiectasia, humoral and cellular immunodeficiencies and a predisposition to malignancy. We report on a 13 years old male patient with the diagnosis of A-T associated with uncommon clinical features; optic disc drusen and vitiligo. To our knowledge, this is the first report of A-T associated with these findings.

An unexpected outcome of blunt ocular trauma: rupture of three muscles.

INTRODUCTION: Traumatic strabismus due to isolated extraocular muscle rupture is uncommon. Treatment usually depends on the severity of both the subjective and objective findings. METHODS: We report a male patient with restricted abduction and supraduction in the right eye follow ing a blunt ocular trauma. The exploration revealed the rupture of superior rectus, superior oblique, and lateral rectus muscles. Only lateral rectus muscle could be sutured to the proximal segment. Superior rectus and superior oblique muscles were severed brutally, so that repairing was not possible. RESULTS: On the day after exploration and primary suturation, there was 25 prism diopters (PD) hypotropia and 15 PD esotropia in his right eye with severe limited supraduction and abduction. His major complaint was a large vertical diplopia which resolved partially with the prismatic glasses prescribed. After 6 months follow-up, medial rectus and inferior rectus recession was performed in the right eye. The patient had a limited but improved abduction after the operation. He was orthotropic and had a single binocular vision in the primary position. DISCUSSION: In suspected extraocular muscle ruptures, orbital imaging methods and surgical exploration should be considered promptly. MRI may be mandatory to demonstrate the severed muscles in cases with persistent diplopia and normal CT. Prognosis is usually better in patients having partial extraocular muscle damage and treatment options should be evaluated on patient basis.

Persistent pupillary membranes in 3 siblings.

We report 3 siblings from a nonconsanguineous white family with bilateral persistent pupillary membranes with dissimilar forms of severity. Two of the patients required no treatment; 1 was treated surgically for occlusion of the pupillary axis and moderate visual acuity decrease. Anterior segment examination was otherwise normal in the siblings. Although persistent pupillary membranes are usually sporadic, cases in which they are associated with other anterior chamber abnormalities might be inherited autosomal dominant. To our knowledge, this is the first report of a familial isolated persistent papillary membrane case with no other anterior chamber abnormality.

Intravitreal triamcinolone acetonide treatment for serpiginous choroiditis.

PURPOSE: To report the efficacy of intravitreal triamcinolone acetonide injection for acute treatment of a patient with serpiginous choroiditis. METHODS: A 50-year-old male patient with serpiginous choroiditis presenting with the complaint of decreased visual acuity in his right eye for the last 10 days. The best corrected visual acuity (BCVA) of the patient was counting finger from 1 meter. Fundus examination and fundus fluorescein angiography of right eye revealed active macular choroiditis in right eye. Intravitreal triamcinolone acetonide (4 mg/0.1 ml) was injected into vitreous, and the patient was followed with visual acuity testing, intraocular pressure measurement, and fundus examination, including fundus fluorescein angiography. RESULTS: Visual acuity of the patient improved to 20/100 after 2 weeks in spite of the triamcinolone crystals, and to 20/50 after 4 weeks with a single dose intravitreal triamcinolone acetonide injection. Complete resolution of the active lesion has been maintained during the 6 months of follow-up. CONCLUSIONS: Single dose intravitreal triamcinolone acetonide injection is sufficient for controlling the active lesions in serpiginous choroiditis. It needs further evaluation as an alternative treatment for achieving rapid and significant visual acuity recovery.

Excimer laser-assisted anterior lamellar keratoplasty for keratoconus, corneal problems after laser in situ keratomileusis, and corneal stromal opacities.

PURPOSE: To evaluate excimer laser-assisted anterior lamellar keratoplasty to augment thin corneas as in keratoconus (<350 microm) and corneal ectasia after laser in situ keratomileusis (LASIK) and to treat anterior stromal opacities. SETTING: Ophthalmology Department, School of Medicine, Gazi University, Ankara, Turkey. METHODS: Thirteen eyes (5 keratoconus, 3 macular dystrophies, 1 post-LASIK ectasia, 1 post-LASIK interstitial keratitis, 3 post-herpetic keratitis sequelae) of 13 patients were included in this prospective study. The treatment group was divided into corneal ectasia and stromal opacity groups. A donor stromal button approximately 350 microm thick received a 100 microm excimer laser ablation on the endothelium. The remaining cornea (epithelium, Bowman's membrane, and stroma) was punched with a 7.5 or 7.7 mm trephine. After transepithelial ablation of the host cornea to 200 mum thickness, the corneal button was sutured with interrupted 10-0 monofilament nylon. Sutures were removed between 3 months and 6 months postoperatively. Preoperative and postoperative simulated keratometric cylinders and corneal thickness values were compared using the Wilcoxon signed rank test. The postoperative spherical equivalent refraction and best spectacle-corrected visual acuity (BSCVA) between the groups were compared using the Mann-Whitney U test. RESULTS: The mean follow-up was 27.6 months +/- 8.3 (SD). All patients gained 2 lines or more of BSCVA, and no patient lost a line. The mean corneal thickness was 381.2 +/- 88.2 microm preoperatively, which significantly increased to 534.9 +/- 96.6 microm postoperatively (P < .05). The mean preoperative simulated keratometric cylinder was 7.44 +/- 7.18 diopters (D); postoperatively, it decreased to 2.61 +/- 1.73 D (P < .05). There was no significant difference in postoperative spherical equivalent refraction or BSCVA between the groups (P > .05). CONCLUSIONS: This technique presents a different modality for the treatment of keratoconus, post-LASIK corneal problems, and other corneal stromal opacities with anterior lamellar keratoplasty. Additional studies with more patients and longer follow-up will help determine the role of this technique as a substitute for penetrating keratoplasty in these patients.

Effect of mitomycin C on ciliary body and intraocular pressure with various application depths: an experimental study.

BACKGROUND: To evaluate the effects of mitomycin C (MMC) on intraocular pressure (IOP) and ciliary body via transmission electron microscopy when applied under conjunctiva or different depths of sclera, without performing any filtering surgery. METHODS: Thirty-six eyes of 36 New Zealand albino rabbits were used in this study. MMC was prepared in a concentration of 0.4 mg/mL and 0.05 cc (20 microg) was soaked in preprepared sterile surgical sponges. Six groups each consisting of six eyes were formed and IOP was measured preoperatively. Group 1 was the control group: the superior conjunctiva was opened and only irrigated with balanced salt solution (BSS). In group 2, MMC soaked sponges were applied under the conjunctiva. In groups 3 and 4, a scleral flap of approximately 1/3 scleral thickness was prepared and in groups 5 and 6, and a scleral flap of approximately 2/3 scleral thickness was prepared, all with a standard size of 4 x 4 mm. MMC soaked sponges were applied under these areas for 5 min in eyes in groups 3 and 5 followed by an irrigation of the relevant areas with 10 cc BSS, whereas only irrigation with BSS was done in groups 4 and 6 as control groups. No filtering procedure was performed in any of the eyes. Eyes were enucleated on the 30th day following measurement of IOP and the ciliary body regions were evaluated using transmission electron microscopy. Kruskal-Wallis test was used for the statistical assessment of IOP between groups. RESULTS: The deep scleral flap group (group 5) showed statistically significantly more IOP reduction than both the superficial scleral flap group (group 3; P = 0.004) and the subconjunctival group (group 2; P = 0.002) on postoperative day 30. Electron microscopic evaluation of the surgical groups revealed a wide range of different histopathological effects due to different MMC application methods. The histopathological changes were more evident in the group 5, where MMC was applied under deep scleral flap. CONCLUSIONS: Subscleral application of MMC seems to provide greater IOP decrease than subconjunctival application, possibly caused by a more significant ciliary body toxicity. This may be the beginning of a non-penetrating, easy to perform and safe method to decrease IOP in glaucoma patients, which the authors call 'toxic ciliary ablation surgery'. However, the long-term results and complications must be assessed with further studies.

Bilateral giant macular hole in a patient with chronic renal failure.

A 32-year-old woman with a medical history of chronic hypertension, chronic renal failure, and hearing deficit presented with bilateral giant macular holes. Possible mechanisms that cause giant macular holes and the relationship between renal failure and giant macular hole formation are discussed.

Microkeratome-assisted lamellar keratoplasty for keratoconus: stromal sandwich.

PURPOSE: To evaluate microkeratome-assisted lamellar keratoplasty for the treatment of keratoconus when it is not possible to correct the astigmatic ametropia with contact lenses. SETTING: Ophthalmology Department, School of Medicine, Gazi University, Ankara, Turkey. METHODS: This prospective study comprised 9 eyes of 7 keratoconus patients with contact lens intolerance. The donor cornea was prepared with a microkeratome and punched with a 7.25 mm or 7.50 mm trephine. Following the creation of a standard 9.0 mm corneal flap in the host cornea, the donor stromal button was implanted under this corneal flap like a sandwich. Transepithelial photorefractive keratectomy or laser in situ keratomileusis was performed when the corneal topography and refraction stabilized by the end of the sixth postoperative month. Follow-up ranged from 7 to 22 months. RESULTS: All patients gained 5 or more lines (mean 7.2 lines +/- 1.6 [SD]), and no patient lost a line of vision. The mean corneal thickness was 432.7 +/- 36.1 micrometers preoperatively and 578.1 +/- 45.1 micrometers after refractive surgery. CONCLUSION: The early visual results of this surgical technique are promising and seem to be comparable to those with penetrating keratoplasty.