Keratoconus and Corneal Ectasia with Relatively Low Keratometry.

INTRODUCTION: The study aims to demonstrate and estimate the prevalence of clinical corneal ectasia and keratoconus (KC) in patients with relatively low keratometry (low-K KC). METHODS: In a retrospective, analytical, and non-interventionist study, one eye was randomly selected from 1054 patients from the original Tomographic Biomechanical Index (TBIv1) study and the external validation (from Rio de Janeiro, Brazil, and Milan, Italy clinics). Patients were stratified into three groups. Group 1 included 736 normal patients, and groups 2 and 3 included 318 patients with clinical KC in both eyes, divided into low-K KC (90 patients) and high-K KC (228 patients), respectively. All patients underwent a comprehensive ophthalmological evaluation along with Pentacam and Corvis ST (Oculus, Wetzlar, Germany) examinations. Cases with maximum mean zone 3 mm keratometry (Kmax zone mean 3 mm) lower than 47.6 diopters (D) were considered as low-keratometry keratoconus, and cases with Kmax zone mean 3 mm higher than 47.6 D were regarded as high-keratometry keratoconus. RESULTS: Ninety (28.30%) of the 318 KC group presented ectasia with low-keratometric values (low-Kmax). The average age in the normal group was 39.28 years (range 6.99-90.12), in the low-Kmax KC group it was 37.49 (range 13.35-78.45), and in the high-Kmax KC group it was 34.22 years (range 12.7-80.34). Mean and SD values and median (range), respectively, of some corneal tomographic and biomechanical parameters evaluated from the low-Kmax KC group were as follows: Belin-Ambrósio enhanced ectasia display (BAD-D) 3.79 ± 1.62 and 3.66 (0.83-9.73); Pentacam random forest index (PRFI) 0.78 ± 0.25 and 0.91 (0.05-1); corneal biomechanical index (CBI) 0.58 ± 0.43 and 0.75 (0-1); TBI 0.93 ± 0.17 and 1 (0.35-1); and stiffness parameter at A1 (SP-A1) 86.16 ± 19.62 and 86.05 (42.94-141.66). CONCLUSION: Relatively low keratometry, with a Kmax lower than 47.6 D, can occur in up to 28.30% of clinical keratoconus. These cases have a less severe presentation of the disease. Future studies involving larger populations and prospective designs are necessary to confirm the prevalence of keratoconus with low keratometry and define prognostic factors in such cases.

Multimodal diagnostics for keratoconus and ectatic corneal diseases: a paradigm shift.

Different diagnostic approaches for ectatic corneal diseases (ECD) include screening, diagnosis confirmation, classification of the ECD type, severity staging, prognostic evaluation, and clinical follow-up. The comprehensive assessment must start with a directed clinical history. However, multimodal imaging tools, including Placido-disk topography, Scheimpflug three-dimensional (3D) tomography, corneal biomechanical evaluations, and layered (or segmental) tomography with epithelial thickness by optical coherence tomography (OCT), or digital very high-frequency ultrasound (dVHF-US) serve as fundamental complementary exams for measuring different characteristics of the cornea. Also, ocular wavefront analysis, axial length measurements, corneal specular or confocal microscopy, and genetic or molecular biology tests are relevant for clinical decisions. Artificial intelligence enhances interpretation and enables combining such a plethora of data, boosting accuracy and facilitating clinical decisions. The applications of diagnostic information for individualized treatments became relevant concerning the therapeutic refractive procedures that emerged as alternatives to keratoplasty. The first paradigm shift concerns the surgical management of patients with ECD with different techniques, such as crosslinking and intrastromal corneal ring segments. A second paradigm shift involved the quest for identifying patients at higher risk of progressive iatrogenic ectasia after elective refractive corrections on the cornea. Beyond augmenting the sensitivity to detect very mild (subclinical or fruste) forms of ECD, ectasia risk assessment evolved to characterize the inherent susceptibility for ectasia development and progression. Furthermore, ectasia risk is also related to environmental factors, including eye rubbing and the relational impact of the surgical procedure on the cornea.

Biomechanics in Keratoconus Diagnosis.

Purpose: To prospectively review the importance of biomechanical assessment in the screening, diagnosis, prognosis, individualized planning, and clinical follow-up for ectatic corneal diseases.Methods: We demonstrate two commercially available devices to assess the corneal biomechanics in vivo, the Ocular Response Analyzer (ORA, Reichester, NY, USA) and the Corvis ST (Oculus, Wetzlar, Germany). Novel devices have been demonstrated to provide in vivo biomechanical measurements, including Brillouin optical microscopy and OCT elastography. Conclusion: The integration of biomechanical data and other data from multimodal refractive imaging using artificial intelligence demonstrated the ability to enhance accuracy in diagnosing ectatic corneal diseases.

Optimized Artificial Intelligence for Enhanced Ectasia Detection Using Scheimpflug-Based Corneal Tomography and Biomechanical Data.

PURPOSE: To optimize artificial intelligence (AI) algorithms to integrate Scheimpflug-based corneal tomography and biomechanics to enhance ectasia detection. DESIGN: Multicenter cross-sectional case-control retrospective study. METHODS: A total of 3886 unoperated eyes from 3412 patients had Pentacam and Corvis ST (Oculus Optikgeräte GmbH) examinations. The database included 1 eye randomly selected from 1680 normal patients (N) and from 1181 "bilateral" keratoconus (KC) patients, along with 551 normal topography eyes from patients with very asymmetric ectasia (VAE-NT), and their 474 unoperated ectatic (VAE-E) eyes. The current TBIv1 (tomographic-biomechanical index) was tested, and an optimized AI algorithm was developed for augmenting accuracy. RESULTS: The area under the receiver operating characteristic curve (AUC) of the TBIv1 for discriminating clinical ectasia (KC and VAE-E) was 0.999 (98.5% sensitivity; 98.6% specificity [cutoff: 0.5]), and for VAE-NT, 0.899 (76% sensitivity; 89.1% specificity [cutoff: 0.29]). A novel random forest algorithm (TBIv2), developed with 18 features in 156 trees using 10-fold cross-validation, had a significantly higher AUC (0.945; DeLong, P < .0001) for detecting VAE-NT (84.4% sensitivity and 90.1% specificity; cutoff: 0.43; DeLong, P < .0001) and a similar AUC for clinical ectasia (0.999; DeLong, P = .818; 98.7% sensitivity; 99.2% specificity [cutoff: 0.8]). Considering all cases, the TBIv2 had a higher AUC (0.985) than TBIv1 (0.974; DeLong, P < .0001). CONCLUSIONS: AI optimization to integrate Scheimpflug-based corneal tomography and biomechanical assessments augments accuracy for ectasia detection, characterizing ectasia susceptibility in the diverse VAE-NT group. Some patients with VAE may have true unilateral ectasia. Machine learning considering additional data, including epithelial thickness or other parameters from multimodal refractive imaging, will continuously enhance accuracy. NOTE: Publication of this article is sponsored by the American Ophthalmological Society.

Corneal ectasia detection by epithelial pattern standard deviation from OCT.

PURPOSE: To test the ability of the corneal epithelial pattern standard deviation (PSD) to distinguish between normal and cases with corneal ectatic condition. SETTING: Instituto de Olhos Renato Ambrósio, Rio de Janeiro, Brazil. DESIGN: Cross-sectional retrospective study. METHODS: Patients were stratified into 4 groups based on clinical data and corneal tomography. Groups 1 and 2 comprised 1 eye randomly selected from 105 patients with normal corneas (N) and 86 patients with bilateral keratoconus (KC). Groups 3 and 4, respectively, comprised 11 ectatic eyes with no surgical treatment for KC (very asymmetric ectasia [VAE]-E) from patients whose fellow eyes (61) presented with normal topographic patterns (VAE-NT). Corneas were scanned using an OCT system (RT Vue) and Scheimpflug corneal tomography (Pentacam) and also had biomechanical assessment through the Corvis ST. Corneal epithelial thickness maps were analyzed, and the PSD value was calculated. The area under the receiver operating characteristic curve analysis was used to evaluate the diagnostic accuracy of the indices. RESULTS: A total of 105 normal eyes, 86 keratoconic eyes, and 11 ectatic eyes whose fellow eyes (61) presented normal topographic patterns were evaluated. Epithelial PSD was significantly different across the 4 groups ( P < .0001). The pairwise comparison revealed that the normal group presented significantly lower values than both ectasia groups (KC and VAE-E, P < .0001) and the VAE-NT group ( P = .0008). There was no statistical significant difference between KC and VAE-E ( P = .4284), while they were significantly higher than the VAE-NT group ( P < .0001 and P = .0004). CONCLUSIONS: Epithelial PSD can be used to detect abnormal epithelial thickness patterns. Corneal epithelial thickness changes could be detected accurately in patients with KC, even in the form fruste of the disease.

Diffuse Unilateral Subacute Neuroretinitis Evolving With Submacular Granuloma.

DUSN is an infectious ocular disease that can lead to severe visual impairment and blindness. It usually occurs in young healthy individuals and depending on the stage of the disease, clinical presentation may range from mild vitritis and multifocal gray-white lesions in outer retina to optic atrophy.Parasites of different sizes and species have been proposed as the etiological agent of this disease. Thus, it is hypothesized that different infectious worms may be considered as the likely cause of a both autoimmune and toxic form of nematode retinopathy.Most patients present with already severe visual impairment and in the later stages of the disease, where the likelihood of improvement is low, despite therapy. In cases of early diagnosis, prompt treatment, whether with oral antihelmintic or direct photocoagulation of the worm, patients may show considerable visual improvement and have a more favorable prognosis.

Ectatic diseases.

Ectatic corneal disease (ECD) comprises a group of disorders characterized by progressive thinning and subsequent bulging of the corneal structure. Different phenotypes have been recognized, including keratoglobus, pellucid marginal degeneration (PMD), and keratoconus (KC). Keratoconus has been widely investigated throughout the years, but the advent of laser refractive surgery boosted an immediate need for more knowledge and research about ectatic diseases. This article discusses nomenclature of ectatic disease, etiology and pathogenesis, along with treatment options, with special focus ok KC and forme fruste keratoconus.

Advanced Surface Ablation in Mild (Fruste) Keratoconus: A Case Report.

Corneal ectasia is a complication of refractive surgery, and keratoconus is a contraindication to this type of procedure. Surface ablation may be an option for selected cases of mild keratoconus, with patient education being fundamental to this treatment as well as a complete evaluation of the cornea and optical properties of the patient. Here we report the clinical outcome of a patient 15 years after advanced surface ablation in a case of mild (fruste) keratoconus.

The Role of Corneal Biomechanics for the Evaluation of Ectasia Patients.

PURPOSE: To review the role of corneal biomechanics for the clinical evaluation of patients with ectatic corneal diseases. METHODS: A total of 1295 eyes were included for analysis in this study. The normal healthy group (group N) included one eye randomly selected from 736 patients with healthy corneas, the keratoconus group (group KC) included one eye randomly selected from 321 patients with keratoconus. The 113 nonoperated ectatic eyes from 125 patients with very asymmetric ectasia (group VAE-E), whose fellow eyes presented relatively normal topography (group VAE-NT), were also included. The parameters from corneal tomography and biomechanics were obtained using the Pentacam HR and Corvis ST (Oculus Optikgeräte GmbH, Wetzlar, Germany). The accuracies of the tested variables for distinguishing all cases (KC, VAE-E, and VAE-NT), for detecting clinical ectasia (KC + VAE-E) and for identifying abnormalities among the VAE-NT, were investigated. A comparison was performed considering the areas under the receiver operating characteristic curve (AUC; DeLong's method). RESULTS: Considering all cases (KC, VAE-E, and VAE-NT), the AUC of the tomographic-biomechanical parameter (TBI) was 0.992, which was statistically higher than all individual parameters (DeLong's; p < 0.05): PRFI- Pentacam Random Forest Index (0.982), BAD-D- Belin -Ambrosio D value (0.959), CBI -corneal biomechanical index (0.91), and IS Abs- Inferior-superior value (0.91). The AUC of the TBI for detecting clinical ectasia (KC + VAE-E) was 0.999, and this was again statistically higher than all parameters (DeLong's; p < 0.05): PRFI (0.996), BAD-D (0.995), CBI (0.949), and IS Abs (0.977). Considering the VAE-NT group, the AUC of the TBI was 0.966, which was also statistically higher than all parameters (DeLong's; p < 0.05): PRFI (0.934), BAD- D (0.834), CBI (0.774), and IS Abs (0.677). CONCLUSIONS: Corneal biomechanical data enhances the evaluation of patients with corneal ectasia and meaningfully adds to the multimodal diagnostic armamentarium. The integration of biomechanical data and corneal tomography with artificial intelligence data augments the sensitivity and specificity for screening and enhancing early diagnosis. Besides, corneal biomechanics may be relevant for determining the prognosis and staging the disease.

Hepatobiliary disease in children and adolescents with cystic fibrosis / Doença hepatobiliar em crianças e adolescentes com fibrose cística

Abstract Objectives: The aims of the study were to determine the frequency of hepatobiliary disease in patients with cystic fibrosis and to describe the sociodemographic, clinical, and laboratory profile of these patients. Methods: This was a retrospective, descriptive, and analytical study of 55 patients diagnosed with cystic fibrosis, aged between 3 months and 21 years, followed-up from January 2008 to June 2016 in a referral center. Medical records were consulted and sociodemographic, clinical and laboratory data, including hepatobiliary alterations, imaging studies, genetic studies, liver biopsies, and upper digestive endoscopies were registered. Results: Hepatobiliary disease was diagnosed in 16.4% of the patients and occurred as an initial manifestation of cystic fibrosis in 55.6% of these cases. The diagnosis of hepatopathy occurred before or concomitantly with the diagnosis of cystic fibrosis in 88.9% of the children. All patients with hepatobiliary disease were considered non-white, with a predominance of females (77.8%) and median (IQR) of 54 (27-91) months. Compared with the group without hepatobiliary disease, children with liver disease had a higher frequency of severe mutations identified in the CFTR gene (77.8% vs. 39.6%, p = 0.033) and severe pancreatic insufficiency (88.9% vs. 31.6%, p = 0.007). Conclusion: The frequency of hepatobiliary disease was high, with a very early diagnosis of the disease and its complications in the studied series. A statistical association was observed between the occurrence of hepatobiliary disease and the presence of pancreatic insufficiency and severe mutations in the CFTR gene. It is emphasized that cystic fibrosis is an important differential diagnosis of liver diseases in childhood.

Resumo Objetivos: Os objetivos do estudo foram determinar a frequência da doença hepatobiliar em pacientes com fibrose cística e descrever o perfil sociodemográfico, clínico e laboratorial destes. Métodos: Estudo retrospectivo, descritivo e analítico de 55 pacientes com diagnóstico de fibrose cística, entre três meses e 21 anos, acompanhados de janeiro de 2008 a junho de 2016 em um centro de referência. Foi realizada consulta aos prontuários médicos, registrando-se os dados sociodemográficos, clínicos e laboratoriais, incluindo-se alterações hepatobiliares, exames de imagem, estudos genéticos, biópsias hepáticas e endoscopias digestivas altas. Resultados: A doença hepatobiliar foi diagnosticada em 16,4% dos pacientes e ocorreu como manifestação inicial da fibrose cística em 55,6% destes casos. O diagnóstico da hepatopatia ocorreu antes ou concomitante ao diagnóstico da fibrose cística em 88,9% das crianças. Todos os pacientes com doença hepatobiliar foram considerados não brancos, havendo predominância do sexo feminino (77,8%) e mediana (I.I.Q) de idade de 54 (27-91) meses. Em comparação com o grupo sem doença hepatobiliar, as crianças com hepatopatia tiveram maior frequência de mutações graves no gene CFTR identificadas (77,8% vs 39,6%; p = 0,033) e de insuficiência pancreática grave (88,9% vs 31,6%; p = 0,007). Conclusão: A frequência de doença hepatobiliar foi elevada, observando-se um diagnóstico muito precoce da mesma e de suas complicações na casuística estudada. Houve associação estatística entre a ocorrência de doença hepatobiliar e a presença de insuficiência pancreática e de mutações graves do gene CFTR. Enfatiza-se que a fibrose cística represente um importante diagnóstico diferencial de hepatopatias na infância.

Hepatobiliary disease in children and adolescents with cystic fibrosis.

OBJECTIVES: The aims of the study were to determine the frequency of hepatobiliary disease in patients with cystic fibrosis and to describe the sociodemographic, clinical, and laboratory profile of these patients. METHODS: This was a retrospective, descriptive, and analytical study of 55 patients diagnosed with cystic fibrosis, aged between 3 months and 21 years, followed-up from January 2008 to June 2016 in a referral center. Medical records were consulted and sociodemographic, clinical and laboratory data, including hepatobiliary alterations, imaging studies, genetic studies, liver biopsies, and upper digestive endoscopies were registered. RESULTS: Hepatobiliary disease was diagnosed in 16.4% of the patients and occurred as an initial manifestation of cystic fibrosis in 55.6% of these cases. The diagnosis of hepatopathy occurred before or concomitantly with the diagnosis of cystic fibrosis in 88.9% of the children. All patients with hepatobiliary disease were considered non-white, with a predominance of females (77.8%) and median (IQR) of 54 (27-91) months. Compared with the group without hepatobiliary disease, children with liver disease had a higher frequency of severe mutations identified in the CFTR gene (77.8% vs. 39.6%, p=0.033) and severe pancreatic insufficiency (88.9% vs. 31.6%, p=0.007). CONCLUSION: The frequency of hepatobiliary disease was high, with a very early diagnosis of the disease and its complications in the studied series. A statistical association was observed between the occurrence of hepatobiliary disease and the presence of pancreatic insufficiency and severe mutations in the CFTR gene. It is emphasized that cystic fibrosis is an important differential diagnosis of liver diseases in childhood.

Luxaçäo isolada do escafóide. Relato de caso / Isoleted dislocation of scaphoid. Case report

Os autores relatam raro caso de luxaçao traumática isolada do escafóide, com seguimento de 17 meses. Sao descritos os quadros clínico e radiológico, assim como o tratamento realizado e o resultado obtido.

Avaliaçäo clínica e radiológica da artroplastia do quadril com prótese bipolar näo cimentada / Clinical and radiological evaluation of hip arthroplasty with cementless bipolar prosthesis

Este trabalho tem por objetivo realizar avaliaçäo clínica e radiológica de 50 pacientes, num total de 63 quadris, submetidos a artroplastias com prótese bipolar näo cimentada no período de junho de 1989 a junho de 1993. A avaliaçäo clínica baseou-se no protocolo de D'Aubigné e Postel modificado por Charnley e a radiológica, no protocolo de Charles Engh. os resultados foram considerados como bons e excelentes em 77,77 porcento dos casos em relaçäo à dor, 74,59 porcento para a marcha e 92,05 porcento para mobilidade. Na avaliaçäo radiológica, obtivemos 92,06 porcento de bons resultados num período médio de acompanhamento pós-operatório de 38 meses