Comparison of inferior oblique myectomy to recession for the treatment of superior oblique palsy.

PURPOSE: To compare inferior oblique (IO) myectomy with recession for the treatment of superior oblique (SO) palsy. METHODS: A retrospective review of medical records identified all patients with SO palsy who underwent IO weakening procedures. Patients were excluded if IO muscle surgery was bilateral, combined with other vertical muscle surgery and if follow up was less than 4 weeks. Good outcome parameters were: improvement of deviation in primary gaze, resolution of diplopia in primary and reading gazes and correction of head tilt to the contralateral side. We analysed subsets of subjects with large preoperative vertical deviations (>15Δ) and those with small-moderate deviations (≤15Δ). Comparisons were made using Wilcoxon rank sum and Fisher's exact tests. RESULTS: Eighty-five patients met the study criteria. Forty-three eyes underwent myectomy, and 42 underwent recession of the IO. Those patients who underwent myectomy compared with recession had less postoperative hypertropia (HT) in primary gaze (p<0.001) and were more likely to meet criteria for success as measured by ≤4Δ residual HT (p=0.056). This difference was pronounced (p=0.005) in patients with preoperative small-moderate deviations, but statistical significance was lost in patients with large preoperative deviations. There was no difference between the two groups with respect to resolution of diplopia or anomalous head tilt. CONCLUSIONS: IO weakening procedures, including both recession and myectomy, are effective in the treatment of SO palsy. While the two procedures were similar in treatment of diplopia and abnormal head tilt, our findings support myectomy as slightly more effective than recession in improving HT in primary gaze; this difference is more pronounced in individuals with small-moderate preoperative hyperdeviations.

Retinoblastoma with autoinfarction presenting as orbital cellulitis.

We describe a case of unilateral retinoblastoma in a 13-month-old boy who presented with signs of orbital cellulitis. Heterochromia, hyphema, and vitreous hemorrhage were observed. Initial computed tomography (CT) imaging failed to reveal the calcified intraocular mass that was later identified on ultrasonography. Histopathology of the enucleated eye revealed diffuse inflammation and tumor necrosis but with absence of malignant retinoblastoma cells. Genetic testing was equivocal. The patient is presumed to have had retinoblastoma that underwent autoinfarction, leading to secondary inflammation. This case highlights the importance of complete ocular evaluation and proper imaging modalities in children presenting with orbital cellulitis.

Oculodentodigital dysplasia: new ocular findings and a novel connexin 43 mutation.

OBJECTIVES: To describe new ocular findings associated with oculodentodigital dysplasia (ODDD) and a novel mutation in the connexin 43 transmembrane domain. DESIGN: Oculodentodigital dysplasia is a rare autosomal dominant disease characterized by multiple systemic abnormalities, most commonly of the ocular, nasal, dental, and limb structures. Herein, we studied 2 patients with ODDD. We describe their clinical findings and 2 ocular abnormalities not previously emphasized or reported. RESULTS: Optic nerve and retinal dysplasia was observed in both patients, and ciliary body cysts were observed in 1 patient. Both patients carried isolated in-frame deletion and missense mutations of the GJA1 gene on chromosome 6. CONCLUSIONS: Optic nerve and retinal dysplasia had not been emphasized as ocular manifestations of ODDD. Ciliary body cysts have not previously been reported in association with ODDD. CLINICAL RELEVANCE: Our findings support the potential significance of connexin 43 in the retina, optic nerve, and ciliary body. Retinal and optic nerve dysplasia may be more common than previously appreciated and may be associated with reduced vision. In addition, the ciliary body cysts observed in 1 patient may be secondary to weakened cellular adhesions between ciliary body pigmented and nonpigmented epithelium associated with the in-frame deletion identified in the affected patient. The presence of these cysts may exacerbate glaucoma or complicate its management.

Descemet stripping automated endothelial keratoplasty in a 2-year-old child.

Endothelial transplantation in the form of Descemet stripping endothelial keratoplasty (DSEK) or Descemet stripping automated endothelial keratoplasty (DSAEK) has rapidly become a popular, if not the preferred, method of treating endothelial failure. Although the need to perform corneal transplantation in children with endothelial dysfunction is relatively rare, this situation does arise, and in children still in the amblyogenic age group, rapid recovery from an essentially refractive-neutral corneal procedure carries significant advantages. We describe the use of DSAEK in a 2-year-old child who exhibited irreversible corneal edema from endothelial damage secondary to complicated cataract surgery. The rapid recovery and lack of induced astigmatism from DSAEK allowed for prompt institution of amblyopia therapy.

Optic disc coloboma and localized chorioretinal defects in constitutional partial trisomy 8 mosaicism.

Constitutional partial trisomy 8 mosaicism is a rare chromosomal multisystem disorder with systemic and ophthalmologic manifestations. We report the case of a 15-year-old female mosaic for trisomy 8 who has unilateral colobomatous microphthalmia and multiple chorioretinal defects. These congenital anomalies have not been previously reported in association with constitutional partial trisomy 8 mosaicism.

Vitrectomy for dense vitreous hemorrhage in infancy.

PURPOSE: To report clinical data, including etiology and visual outcome, in newborns requiring vitrectomy for dense vitreous hemorrhage. METHODS: In this retrospective case series, we surveyed subscribers to the American Association for Pediatric Ophthalmology and Strabismus ListServe regarding patients under their care. RESULTS: A total of 28 eyes of 21 patients were included. Most common etiologies were thrombocytopenia, shaken baby syndrome, and birth trauma. In 9 cases (12 eyes), the vitreous hemorrhage was idiopathic. Mean time between diagnosis and surgery was 1.4 months. Complications included strabismus, cataract, glaucoma, high myopia, and retinal detachment. Recognition visual acuities were available for 8 eyes: 20/25 (2 eyes), 20/30, 20/40 (2 eyes), 20/60 (2 eyes), and 20/100. One eye had no light perception. CONCLUSIONS: The etiologies encountered in our patients were similar to those reported previously. Visual outcomes were much worse in cases with retinal complications. Other patients had better visual outcomes. Despite potential surgical and postoperative complications, this series demonstrates favorable visual outcomes can be achieved following early vitrectomy in this setting.

Observations on the development and progression of unilateral high myopia.

PURPOSE: The purpose of this article is to document the rare observation of the development, progression, retinal changes, and results of visual rehabilitation in a young child with unilateral high myopia, as well as additional clinical observations in four very young patients with this condition. We also examine the role of factors such as amount of astigmatism, presence of strabismus, degree of anisometropia, and penalization in the development of optimal vision in these patients. MATERIALS: A review of patients from 1997-2002 disclosed 33 individuals with a diagnosis of unilateral high myopia. Five children with adequate information on the progression of myopia and/or in whom a final visual outcome could be determined were included in this study. We excluded patients with insufficient follow-up, those who did not comply with occlusion, and those with co-existing ocular conditions such as retinopathy of prematurity. RESULTS: Three females and two males (four right eyes and one left) were followed for a period of 16 months to 7 years. Myopic fundus changes were present in three patients. Glasses were the preferred method of optical correction in four patients; one patient was prescribed a contact lens. Occlusion therapy was used in four of the five patients. Strabismus was present in four. Ipsilateral astigmatism of +2.00 D or more was present in three of the affected eyes. The difference in the spherical equivalent refraction between both eyes in each patient ranged from 5 to 20 diopters. In one patient, we observed the progressive development of unilateral myopia from an initial hypermetropia of +2.00 at age 2 months to myopia of -9.00 at the age of 5 years. CONCLUSIONS: Unilateral high myopia is probably not present at birth. Our observations support its development in the first few years of life. The results of amblyopia therapy depend on the degree of anisometropia, the co-existence of strabismus, the compliance with penalization, and the presence of retinal abnormalities.