The influence of congenital corneal opacity on ERGs obtained using an abbreviated protocol.

BACKGROUND: 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). METHODS: 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. RESULTS: 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). CONCLUSION: 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.

Variable phenotype of secondary congenital corneal opacities associated with microphthalmia with linear skin defects syndrome.

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.

Penetrating keratoplasty in children under 3 years old with congenital corneal opacities.

AIM: 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. CONCLUSION: 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.

Resultados visuales y sobrevida de injerto obtenidos en una población pediátrica sometida a queratoplastia / Visual outcomes and graft survival in pediatric patients underwent penetrating keratoplasty

Objetivo: Describir resultados visuales y complicaciones de una serie de casos de pacientes pediátricos a los que se les realizó queratoplastia penetrante en el Hospital de San José, en Bogotá, Colombia. Diseño: Estudio observacional descriptivo, retrospectivo, serie de casos. Método: Se evaluaron 14 ojos de 12 pacientes menores de 16 años a quienes se les realizó queratoplastia penetrante entre los años 1999-2014. Se hizo una revisión de la literatura sobre etiología, manejo quirúrgico, seguimiento y sobrevida de injerto. Resultados: De los 14 ojos estudiados 8 ojos (57,1%) tenían patología congénita con injerto claro en 6 ojos (75%) entre los 3 y 9 meses, 3 ojos (21,4%) tenían etiología traumática, de estos, todos los injertos permanecieron claros en un rango de 3 meses a 11 años y 3 ojos (21,4%) tenían leucomas, queratitis o inminencia de perforación, de estos el injerto permaneció claro entre los 3 y 18 meses. Se encontró una mejoría de visión en 8 de los ojos evaluados (57,1%). Las complicaciones encontradas en estos pacientes fueron: glaucoma en 5 ojos (35.7%), falla de injerto en 6 ojos (42.8%), rechazo endotelial en 7 ojos (50%) y desprendimiento de retina en 1 ojo (7.14%). Conclusión: En el estudio, la mejoría visual fue ligeramente menor a la reportada en la literatura. Las complicaciones presentadas fueron principalmente rechazo endotelial y falla de injerto, lo que difiere de la literatura donde la catarata es la principal complicación.

Purpose: To describe visual results and complications in a case series of paediatric patients who underwent penetrating keratoplasty at Hospital de San José in Bogotá, Colombia. Design: Observational, descriptive and retrospective study, series of cases. Method: 14 eyes of 12 patients under 16 years (2 months - 16 years) who underwent penetrating keratoplasty in Hospital de San Jose from 1999 to 2014 were evaluated. A literature review of etiology, surgical management, monitoring and graft survival of pediatric patients was done. Results: Of the 14 eyes studied, 8 eyes (57.1%) had congenital pathology with clear graft in six eyes between 3 and 9 months, 3 eyes (21.4%) had traumatic aetiology, of these, all graft s remained clear in a range of 3 months to 11 years and 3 eyes (21.4%) had leucoma, keratitis or impending perforation, of these, the graft remained clear between 3 and 18 months. An improvement in vision was found in eight of the evaluated eyes (57.1%). The complications found in these patients were: glaucoma in 5 eyes (35.7%), graft failure in 6 eyes (42.8%), endothelial rejection in 7 eyes (50%) and retinal detachment in 1 eye (7.14%). Conclusion: In our study, the visual improvement was slightly lower than that reported in the literature. The complications presented were mainly endothelial rejection and graft failure, which differs from the literature where cataract is the main complication.