Craniofacial Microsomia: Goldenhar Syndrome in Association with Bilateral Congenital Cataract.

Craniofacial microsomia (CFM) includes a spectrum of malformations primarily involving structures derived from the first and second branchial arches. Patients with hemifacial microsomia and epibulbar dermoids are said to have Goldenhar syndrome (GHS). Four-month-old boy with whitish pupillary reflex presented with the features of GHS in pediatric ophthalmology clinic. The child had ocular and auricular manifestations. There were no vertebral anomalies, but he had bilateral congenital cataract. The peculiarity of this case is the presence of the bilateral total congenital cataract, in association with CFM. There is absence of epibulbar dermoid or lipodermoid in the eyes, although the child had features of GHS. In addition to it, anesthetic intubation was smooth in this case. Any case diagnosed with CFM and/or GHS needs treatment through multidisciplinary approach, consultation in ophthalmology department is one of them.

Visual Axis Opacification in Children Following Paediatric Cataract Surgery.

Visual axis opacification (VAO) occurs in up to 40% of pediatric patients after cataract surgery withintraocular lens implantation(IOL) even with a primary posterior capsulotomy (PPC). In both children and adult group, opacification does obscure the visual axis. However, in children after PPC, there is no capsule. Hence, the terminology VAO is used in children rather than posterior capsular opacification. This opacification is caused by a proliferation of epithelial cells on the posterior capsule or anterior vitreous face and can hinder the optical image quality needed for normal visual development. The rate of epithelial mitotic activity is higher in children compared to adult. It can be managed by Yag laser or surgical membranectomy, the latter is preferable. International and national published articles were systematically reviewed on aetio-pathogenesis, surgical techniques and equipment, type and material of IOL, and management of VAO in children operated for cataract. Author's experience was also included to write manuscript. VAO is frequent complication following cataract surgery in pediatric patients. Age of cataract patients, surgical technique and type and materials of IOL are most common influencing factor for VAO. Immediate management with advance equipment of VAO reduces the incidence for development of irreversible stimulation deprivation amblyopia.

Bruckner red light reflex test in a hospital setting.

BACKGROUND: Childhood blindness is a major problem in developing world including Nepal that needs to be addressed. OBJECTIVE: The objective of study was to measure the effectiveness of the Bruckner red reflex test for screening of posterior segment opacities in children. METHODS: Patients aged less than five years who came to the Tilganga Institute of Ophthalmology, Kathmandu from March to August, 2009 were recruited in this cross-sectional descriptive study. Children were screened with the Bruckner red light reflex test in a dark room. Patients with abnormal Bruckner tests had their eyes dilated for further investigations. Abnormalities were recorded. The number of patients with an abnormal Bruckner test was recorded and correlated with their potential pathology. RESULTS: A total of 172 patients with 97 boys and 75 girls were included. Twenty-three had abnormal Bruckner test results in either one or both eyes. Four of these twentythree patients had no pathology diagnosed; hence the sensitivity of the Bruckner screen to diagnose significant pathology was calculated at 82.6%. Of the 153 patients with normal results, two patients were found to have pathology detected, which included cataract and strabismus. Hence, the specificity of this test was 98.7%. The screening test had a positive predictive value of 90.5% and a negative predictive value of 97.4%. CONCLUSION: Bruckner test screening is a sensitive and specific marker for detecting the posterior segment opacities in children. We recommend utilizing the test to evaluate these problems where other technologies are rare.

Cataract surgery in children: controversies and practices.

Pediatric cataract is totally different from adult cataract. The objective of this review article is to describe the peculiarities of pediatric cataract and the controversies and practices in pediatric cataract surgery. The differences in the surgical technique are discussed in the article. There are lots of controversies regarding IOL implantation in children. The result of the review is that the pediatric cataract surgery should be performed in between 6-8 weeks of age. There is no contra-indication for IOL implantation. Surgeons can implant either polymethylmethacrylate (PMMA) or Acrysoft foldable IOL.

Aberrant regeneration of the third cranial nerve.

BACKGROUND: Aberrant regeneration of the third cranial nerve is most commonly due to its damage by trauma. CASE: A ten-month old child presented with the history of a fall from a four-storey building. She developed traumatic third nerve palsy and eventually the clinical features of aberrant regeneration of the third cranial nerve. The adduction of the eye improved over time. She was advised for patching for the strabismic amblyopia as well. CONCLUSION: Traumatic third nerve palsy may result in aberrant regeneration of the third cranial nerve. In younger patients, motility of the eye in different gazes may improve over time.

Goldenhar syndrome in association with Duane syndrome.

Goldenhar syndrome (GHS) is also known as Oculo-Auriculo-Vertebral (OAV) syndrome or Branchial arch syndrome. Duane retraction syndrome (DRS) is a congenital disorder of ocular motility characterized by limited abduction, adduction or both. It is unilateral in 80% of cases. The important and interesting part of this eight months old child is presence of GHS with DRS. She has bilateral invol-vement, which is seen in only 5-8% of GHS, as compared to high incidence of unilateral involve-ment. This child also had refractive error of + 6.00/ - 1.5 * 180. At four year of age her vision with glass was 6/9. Children with GHS and DRS should have early eye examination done to treat the problem of refractive error.

Axial length in eyes with bilateral pediatric cataract at tertiary eye care center in Nepal: a preliminary report.

The aim of the study was to determine the average axial length reading of the bilateral pediatric cataract undergoing cataract surgery. Pre-operative axial length measurement was done in 80 children below 15 years who had bilateral pediatric cataract. Axial length measurement was done in 56 fellow eyes. The axial length was measured under general anesthesia with the Accutome A-scan. The mean age was 69.7 months (SD=52.6), range from one month to 168 months. The mean axial length reading was 21.3 mm in operated eyes and 21.1 mm in fellow eyes. The range of axial length reading was 16.2 -31.5 mm in operated eyes and it was 16.5 -31.5 mm in fellow eyes. This short observation found that in bilateral pediatric cataract, the axial length value is similar in both operated and fellow eyes. Based on the axial length value of the operated eye, the patients with bilateral congenital cataract can undergo surgery in those eye hospitals where facilities of measurement of axial length is not available.

Tertiary eye care centre model for development of paediatric cataract surgery services in developing countries.

It is estimated that every minute a child becomes bilaterally blind in developing nations. Paediatric cataract blindness presents an enormous problem to developing countries in terms of the human social burden, economic loss, and morbidity. The management of the paediatric cataract is lens aspiration with intra ocular lens implantation with anterior vitrectomy. The surgery should be performed by paediatric ophthalmologist in tertiary eye care centres. Facilities of general anaesthesia should be available in these centres and the operating theatres should be well-equipped with vitrectomy machine, biometry and portable keratometer.

Keratometry in eyes with bilateral pediatric cataract at tertiary eye care center in Nepal: a preliminary report.

Pre-operative keratometry measurement of the subjects was done in 80 children with bilateral congenital cataract below 15 years who had undergone pediatric cataract surgery. The objective of the study was to evaluate the average keratometry reading in Nepalese children with bilateral cataract. The keratometry reading was taken under general anesthesia with the Nidek Auto Keratometer, model KM-500. The mean age was 69.7 months (SD = 52.6), range from one month to 168 months. The mean keratometry reading was 44.8 (SD = 2.7) in operated eyes and 44.7 (SD = 2.6) in fellow eyes. The minimum and the maximum values of keratometry reading in operated eyes were 39.4 and 51.5 respectively where as it was 40.3 and 51.5 in fellow eyes. We concluded that in bilateral pediatric cataract the keratometry value is similar in both operated and fellow eyes. Based on the keratometry value of the operated eye, the patients with bilateral congenital cataract can undergo surgery in those eye hospitals where facilities of the keratometry is not available under general anesthesia. We also recommended that additional study on axial length will be needed to calculate the Intra Ocular Lens power in fellow eye.