Anisometropia and refractive status in children with unilateral congenital nasolacrimal duct obstruction.

OBJECTIVE: The objective of the study was to evaluate the refractive status and thereby assess anisometropia in children with unilateral congenital nasolacrimal duct obstruction (CNLDO). STUDY DESIGN: This study design was a descriptive cross-sectional study. PLACE AND DURATION: this study was conducted at the Department of Pediatric Ophthalmology and Strabismology, Al-Shifa Trust Eye Hospital, Rawalpindi; from August 2013 to July 2014. METHODOLOGY: This study assessed consecutive children with unilateral CNLDO. Cycloplegic refraction on all children with CNLDO was performed followed by appropriate intervention. Refractive errors of the affected and normal eyes were compared. RESULTS: One hundred and twenty-four children with a mean age of 29.69 ± 21.12 months (range, 2 months to 8 years) were studied. Based on spherical equivalent (SE), hypermetropia was more common in the affected eyes (P < 0.001). Anisometropia of >1.5 diopters (D) was present in n = 17 (13.7%). Interocular difference was significant for spherical error and SE (P < 0.001) but not cylindrical errors. CONCLUSION: Unilateral CNLDO is associated with statistically significant anisometropia, especially anisohypermetropia which has amblyogenic potential. It is vital to perform cycloplegic refraction routinely and counsel parents regarding prognosis and regular follow-ups.

Comparison of Anisometropia and Refractive Status in Children With Unilateral and Bilateral Congenital Nasolacrimal Duct Obstruction.

PURPOSE: To evaluate and compare the refractive state in children diagnosed as having unilateral or bilateral congenital nasolacrimal duct obstruction (CNLDO). This study also compares how the laterality of CNLDO affects the refractive state of the patients. METHODS: This descriptive cross-sectional study includes consecutive children with unilateral and bilateral CNLDO over a period of 1 year. Cycloplegic refraction was performed on each child who presented with CNLDO followed by appropriate plans for treatment. The refractive errors of patients with unilateral and bilateral CNLDO were compared. RESULTS: One hundred sixty-one patients with unilateral CNLDO (mean age: 29 ± 19.93 months) and 46 with bilateral CNLDO (mean age: 30 ± 16.21 months) were enrolled from August 2013 to July 2014. The rate of the anisometropia (≥ 1 diopters [D] difference between the two eyes) was 13.7% (n = 22) and 8.6% (n = 4) in patients with unilateral and bilateral CNLDO, respectively. Interocular difference was significant in terms of spherical equivalent (P < .01) but not cylindrical refractive error in patients with unilateral CNLDO. Patients with bilateral CNLDO had insignificant interocular differences in terms of spherical equivalent and cylindrical refractive errors (P > .05). CONCLUSIONS: Unilateral CNLDO is associated with statistically significant anisometropia compared to bilateral CNLDO, which predisposes children with unilateral CNLDO to amblyopia. It is vital to perform cycloplegic refraction routinely and counsel parents regarding regular follow-ups. [J Pediatr Ophthalmol Strabismus. 2016;53(3):168-172.].

Fraser Syndrome.

Fraser's Syndrome (FS) is a rare autosomal recessive disorder with a spectrum of malformations. The most consistent features are Cryptophthalmos (CO), syndactyly, genitourinary tract abnormalities, laryngeal and tracheal anomalies, craniofacial dysmorphism, malformations of the ear and nose, orofacial clefting and musculoskeletal defects. FS is genetically heterogeneous; so far mutations in FRAS1, FREM2 and GRIP1 genes have been linked to FS. FS can be diagnosed on clinical examination, pre-natal ultrasound or perinatal autopsy. We present a case of a 3 months old child born to consanguineous healthy parents with bilateral complete CO, unilateral microphthalmia, hypertelorism, syndactyly (hands and feet bilaterally), ambiguous genitalia with cryptorchidism and an umbilical hernia. We also present the criteria for diagnosing FS and the significant features on pre-natal ultrasonography. Around 200 case reports of patients with FS and CO have been published. To our knowledge, this is the first reported case of FS in Pakistan.

Iridocorneal endothelial syndrome.

A 19 years boy with a 2 years history of reduced and fluctuating vision along with change in pupillary shape and iris colour in his left eye presented to the glaucoma clinic. Ocular examination revealed distinct unilateral stretch holes, iris architecture changes and localized iris atrophy. Intraocular pressure was 16 mmHg in the right and 36 mmHg in the left eye. Gonioscopy of the left angle revealed broad based peripheral anterior synechiae at approximately 2 O'clock to 10 O'clock hours. The optic disc of the left eye had a vertical cup of 0.9 which manifest as superior and inferior arcuate scotoma. Specular microscopy showed unilateral abnormal endothelium with irregular cells of variable shape and size. To our knowledge, this is only the second reported case of iridocorneal endothelial syndrome in a male teenager.