Visual electrophysiology may be used to assess visual potential in infants with congenital corneal opacities (CCO). It is essential to recognize confounding effects from these opacities on the flash electroretinogram (ERG).
ERGs were recorded in awake children employing skin electrodes placed at the lower eyelid crease, both referred to a midfrontal electrode (Fz). A hand-held stimulator was used to present a mixed rod-cone and a dim white stimulus. Recordings were carried out before and after penetrating keratoplasty (PK), when performed.
Five infants under the age of 12 months with visually significant CCO were evaluated. In all cases, initial ERGs employing the mixed rod-cone stimulus showed well-defined a-wave with reduced amplitude b-wave. Reduction of stimulus intensity resulted in an increase in the b-wave and normalization of the b:a ratio from 1.1 (range 0.7 to 1.3) to 2.8 (range 1.5 to 4.3). In three cases who underwent PK, the postoperative ERGs recorded with a mixed rod-cone stimulus were normal in waveform shape with a mean b:a ratio of 2.0 (range 1.7 to 3.0).
Selective reduction of the scotopic bright flash ERG b-wave is typically caused by retinal dysfunction that is post-phototransduction or inner retinal. In infants with CCO, scotopic ERGs to bright flashes can show a reduced b:a ratio that improves or normalizes either after PK or stimulus intensity reduction. The study highlights that media opacity can contribute to the generation of an ERG with reduced b-wave in the absence of inner retinal dysfunction.

To describe the anterior segment (AS) findings in patients with microphthalmia with linear skin defects syndrome (MLS), also known as microphthalmia, dermal aplasia, and sclerocornea (MIDAS). A retrospective chart review was conducted to identify patients with a diagnosis of MLS syndrome seen at UPMC Children\'s Hospital of Pittsburgh. Ophthalmic examination, high-frequency ultrasound, AS optical coherence tomography, and molecular testing were reviewed. Five female patients (10 eyes) were identified. One eye was anophthalmic, one was in a status post penetrating keratoplasty, and eight eyes presented with congenital corneal opacity (CCO). Of these, one showed a normal lens and a very small faint CCO; five showed congenital aphakia and characteristic silvery appearance of the cornea with vascularization; and two showed irido-corneal adhesions in association with normal or abnormal lens and localized avascular CCO. Genetic testing was performed and revealed involvement of HCCS in four patients. In MLS patients, kerato-irido-lenticular dysgenesis can be associated with secondary CCO. It is important to distinguish these CCO from sclerocornea, in order to refine the appropriate management and counseling the parents about the prognosis.

OBJECTIVE: To evaluate the graft rejection and visual outcomes after penetrating keratoplasty (PK) in the presence of various congenital corneal opacities in children.
METHODS: In this retrospective cohort study, children who underwent PK were then followed for 5y. The patient\'s medical records were collected from June 2014 until June 2019 and analyzed in December 2019. All patients were children under three years old with congenital corneal opacities with or without microcornea who came to a pediatric ophthalmologist and underwent PK in Jakarta Eye Center (JEC). Beforehand, all children have participated in a thorough evaluation for PK. In the case of severe microcornea was not advised to undergo surgery. The visual outcomes and graft survival rate were described in percentages. The graft survival plot was presented with Kaplan-Meier, while the visual acuity was analyzed using the Wilcoxon signed ranks test.
RESULTS: Sixteen eyes from eleven patients (seven girls and four boys) underwent PK. The graft survival rate of the first 6, 12, and 18 mo later of keratoplasty was 100%, 83.3%, and 66.7%, respectively. The overall mean survival time is 22mo (standard error 2.419), and no significant difference between the patients underwent PK before and after 36mo of their age (P=0.52). The graft failure was 50%, and post-surgery complications included cataract 43.7%, band keratopathy 12.5%, and scleromalasia 6.25%. Wilcoxon test analysis of visual acuity post keratoplasty was not statistically significant (P=0.34), while overall showed 44% improvements of visual outcome for 5y of follow-up. With a good survival at one year up to 22mo (83.3%), the visual acuity could be achieved (63%), and showed improvements (44%) during follow-up.
CONCLUSIONS: The complications are frequent for pediatric PK. Thus, corneal surgery on infants requires careful case selection, adequate pre-operative evaluation, skilled surgery (optical correction), very close cooperation family-physician, intensive post-operation care, and amblyopia management in the future.