A 43-year-old man developed double vision, esotropia, and abduction limitation after excision of a doubleheaded pterygium. Exploration during strabismus surgery 4 months later revealed a partially severed lateral rectus muscle and extensive conjunctival scarring. Direct injury to the lateral rectus muscle during pterygium surgery is extremely rare. Prevention and management strategies are discussed. [ J Pediatr Ophthalmol Strabismus . 2020;57:e96–e99.]
Purpose: The aim of reporting this case is to describe a rare combination of blepharophimosis–ptosis–epicanthus inversus syndrome with congenital nasolacrimal duct obstruction. A variety of lacrimal anomalies have been seen in blepharophimosis–ptosis–epicanthus inversus syndrome but the occurrence of nasolacrimal duct obstruction is rare. Method: The blepharophimosis–ptosis–epicanthus inversus syndrome is an autosomal dominant rare genetic defect with clinical manifestation of dysplasia of the eyelids, palpebral fissures, flat nasal bridge, and ptosis. A 20-month-old boy was referred with the complaints of watering and discharge from his right eyes since birth. On examination, the child had all the features of blepharophimosis–ptosis–epicanthus inversus syndrome with right congenital nasolacrimal duct obstruction in line with the published reports. Result: On endoscopic probing and irrigation, the probe could not be visualized into the inferior meatus. On dacryoendoscopy, the membranous part of the nasolacrimal duct was found to be completely obliterated with no light transmission into the nose indicating a malformed nasolacrimal duct. The child was managed by endoscopic dacryocystorhinostomy. We could find only one case report published so far on the combination of congenital nasolacrimal duct obstruction with blepharophimosis–ptosis–epicanthus inversus syndrome. This study adds one more case of blepharophimosis–ptosis–epicanthus inversus syndrome with congenital nasolacrimal duct obstruction and adjuvant use of dacryoendoscopy.
Congenital aniridia is a pan ocular disorder characterized by partial or total loss of iris tissue as the defining feature. Classic aniridia, however, has a spectrum of ocular findings, including foveal hypoplasia, optic nerve hypoplasia, nystagmus, late-onset cataract, glaucoma, and keratopathy. The latter three are reasons for further visual compromise in such patients. This entity is often due to mutations in the PAX6 (Paired box protein Pax-6) gene. Recently, aniridia-like phenotypes have been reported due to non- PAX6 mutations as in PITX2, FOXC1, FOXD3, TRIM44 , and CYP1B1 as well wherein there is an overlap of aniridia, such as iris defects with congenital glaucoma or anterior segment dysgenesis. In this review, we describe the various clinical features of classic aniridia, the comorbidities and their management, the mutation spectrum of the genes involved, genotype-phenotype correlation of PAX6 and non- PAX6 mutations, and the genetic testing plan. The various systemic associations and their implications in screening and genetic testing have been discussed. Finally, the future course of aniridia treatment in the form of drugs (such as ataluren) and targeted gene therapy has been discussed.
Purpose: To highlight the importance of dacryoendoscopy in recognizing the factors responsible for failure with successful recanalization obviating the need for dacryocystorhinostomy in a few selective cases.Method: A retrospective study of 13 children undergoing dacryoendoscopy for refractory congenital nasolacrimal duct obstruction (CNLDO) was carried out during a period of 3 years from 2016 to 2018. Children with single or multiple failed probings were included in the study.Results: Out of the total 13 cases included in the study there 9 males and 4 females. The age ranged from 9 months to 36 months with the involvement of the right side in 7 cases and the left side in 6 cases. Four cases had dysgenesis of bony nasolacrimal duct (NLD), 4 cases had dacryolith, 3 cases had an intact membrane at the lower end of NLD and 2 cases had fibrosis of the lower end of NLD and the surrounding area in the inferior meatus (IM) following multiple interventions, (Table 1). Dacryoendoscopic recanalization was done in 7 cases while the endoscopic dacryocystorhinostomy (DCR) was done in 6 cases. Asuccessful outcome was achieved in all the cases and at the end of 6 months, all the children remained asymptomatic.Conclusions: Dacryoendoscopy (DEN) facilitates direct examination of the nasolacrimal system and thus has an added advantage over nasal endoscopy assisted probing in the refractory cases of CNLDO.
To evaluate the outcome of bilateral symmetric and asymmetric superior rectus (SR) recessions in patients with bilateral dissociated vertical deviation (DVD) without oblique dysfunction and determine factors that might influence the surgical outcome.
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