Raras
Buscar doenças, sintomas, genes...
Queratopatia neurotrófica
ORPHA:137596CID-10 · H16.2CID-11 · 1F00.10DOENÇA RARA

A Queratopatia Neurotrófica é uma doença rara e progressiva que afeta a córnea (a parte transparente na frente do olho). Nela, a córnea perde parte ou toda a sua sensibilidade. Pode não apresentar sintomas no início ou causar vermelhidão nos olhos e uma leve diminuição da visão. Com o tempo, pode levar à perda total da visão.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

A Queratopatia Neurotrófica é uma doença rara e progressiva que afeta a córnea (a parte transparente na frente do olho). Nela, a córnea perde parte ou toda a sua sensibilidade. Pode não apresentar sintomas no início ou causar vermelhidão nos olhos e uma leve diminuição da visão. Com o tempo, pode levar à perda total da visão.

Pesquisas ativas
4 ensaios
43 total registrados no ClinicalTrials.gov
Publicações científicas
455 artigos
Último publicado: 2026 Mar 17

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Adult
+ childhood
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: H16.2
Você se identifica com essa condição?
O Raras está aqui pra te apoiar — com ou sem diagnóstico

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

👁️
Olhos
7 sintomas
🫘
Rins
1 sintomas
📏
Crescimento
1 sintomas

+ 6 sintomas em outras categorias

Características mais comuns

90%prev.
Morfologia anormal do quinto nervo craniano
Muito frequente (99-80%)
90%prev.
Sensibilidade corneana diminuída
Muito frequente (99-80%)
55%prev.
Diminuição lenta da acuidade visual
Frequente (79-30%)
17%prev.
Cicatriz corneana
Ocasional (29-5%)
17%prev.
Ulceração corneana
Ocasional (29-5%)
17%prev.
Astigmatismo
Ocasional (29-5%)
15sintomas
Muito frequente (2)
Frequente (1)
Ocasional (9)
Muito raro (3)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 15 características clínicas mais associadas, ordenadas por frequência.

Morfologia anormal do quinto nervo cranianoAbnormal fifth cranial nerve morphology
Muito frequente (99-80%)90%
Sensibilidade corneana diminuídaDecreased corneal sensation
Muito frequente (99-80%)90%
Diminuição lenta da acuidade visualSlow decrease in visual acuity
Frequente (79-30%)55%
Cicatriz corneanaCorneal scarring
Ocasional (29-5%)17%
Ulceração corneanaCorneal ulceration
Ocasional (29-5%)17%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico455PubMed
Últimos 10 anos200publicações
Pico202565 papers
Linha do tempo
2026Hoje · 2026🧪 2005Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado3
3Fase 32
2Fase 22
·Pré-clínico3
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 10 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Queratopatia neurotrófica

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

3 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

43 ensaios clínicos encontrados, 4 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
🧪 Está conduzindo uma pesquisa?
Divulgue para pacientes e familiares que acompanham esta doença.
Divulgar pesquisa →

Publicações mais relevantes

📖Melhor nível de evidência: Revisão
Timeline de publicações
368 papers (10 anos)
#1

Corneal thinning and perforation: a narrative review of diagnostic and stepwise management strategies.

International ophthalmology2026 Mar 24

Corneal perforation is a sight-threatening ophthalmic emergency caused by trauma, infection, autoimmune disease, or ocular surface disorders. Early recognition and timely management are critical to maintain globe integrity and prevent complications. This review synthesizes current knowledge on the diagnosis and management of corneal perforations. A narrative review of the literature was conducted using PubMed, Scopus, Web of Science, and Google Scholar through 2025. Keywords included "corneal perforation," "corneal thinning," "corneal melt," "ulcerative keratitis," "tissue adhesive," "amniotic membrane transplantation," and "keratoplasty." English-language original studies, case series, and reviews were included, and unrelated reports were excluded. Data were qualitatively synthesized due to heterogeneity in study designs. Illustrative clinical cases from King Khaled Eye Specialist Hospital were included to demonstrate real-world diagnostic and therapeutic approaches. Major causes of corneal perforation include infectious keratitis, autoimmune/inflammatory diseases, neurotrophic keratopathy, trauma (including iatrogenic), and degenerative or exposure-related conditions. Common clinical features are rapid vision loss, severe ocular pain, and tearing. Evaluation requires a careful, minimally manipulative examination with adjunctive imaging and systemic assessment when indicated. Management options include tissue adhesives, amniotic membrane grafts, platelet-rich plasma, lamellar or penetrating keratoplasty, and conjunctival flaps. Recent advances in biological adhesives, amniotic membrane techniques, and anterior segment imaging have expanded treatment options and improved outcomes. Follow-up is guided by slit lamp examinations and imaging. This review integrates recent evidence and evolving strategies to provide clinicians with a structured, stepwise framework for managing corneal perforations.

#2

Systematic Literature Review on the Effect of Cenegermin on Corneal Sensation and Innervation in Patients with Neurotrophic Keratopathy.

Journal of ocular pharmacology and therapeutics : the official journal of the Association for Ocular Pharmacology and Therapeutics2026 Mar 18

Neurotrophic keratopathy (NK) results from trigeminal nerve impairment, leading to loss of corneal sensation (CS), epithelial breakdown, and in advanced stages, stromal ulceration or perforation. Cenegermin, a recombinant human nerve growth factor, is the first and only FDA-approved therapy for NK, although existing studies are limited by small sample sizes. This systematic literature review evaluated cenegermin's effects on CS and corneal innervation (CI) in patients with NK. English-language studies published between August 2018 and June 2024 measuring CS or CI after cenegermin treatment for NK were identified through PubMed and Embase. For studies reporting quantitative data, sample size-based weighting was applied to each study's reported or derived mean change. Weighted means were calculated by NK stage or overall, where applicable. A total of 25 studies met the inclusion criteria. The pooled mean age was 61.8 years (range: 2-93 years). Most included patients with stage II (moderate; 19 studies) and stage III (severe; 17 studies) NK. Across 8 studies reporting quantitative CS outcomes and 3 studies reporting quantitative CI outcomes, the weighted mean improvement from baseline to 8 weeks post-treatment was 116.5% for CS (range: 78.3%-259.3%) and 64.5% for CI (range: 39.1%-188.9%). An 8-week course of cenegermin led to improvements in CS and CI in patients with NK, with greater relative gains observed among those patients with more severe disease. While study design heterogeneity and small sample sizes may limit the generalizability of these findings, the data support cenegermin's role in promoting corneal nerve regeneration.

#3

The Role of Innervation in Ocular Surface Homeostasis, Tissue Remodeling Following Nerve Injury, and the Therapeutic Potential of Hemocomponents in Neuronal and Cicatricial Pathologies.

Journal of clinical medicine2026 Mar 06

The ocular surface is a neuro-epithelial-immune unit in which corneal innervation is essential for maintaining tissue integrity and visual function. Sensory nerves regulate reflex tearing and blinking, provide trophic support, and modulate local immune responses. Nerve injury resulting from trauma, surgery, infection, systemic disease, or chronic inflammation disrupts epithelial homeostasis and may lead to neurotrophic keratopathy, neuropathic pain, and pathological remodeling. Beyond classical neurotrophic disease, nerve dysfunction contributes to severe dry eye and immune-mediated cicatricial disorders. Depending on the neuro-inflammatory context, remodeling may evolve toward stromal thinning, as in keratoconus, or progressive fibrosis, as in ocular cicatricial pemphigoid. Blood-derived eye drops, including serum- and platelet-based formulations, represent biologically active therapies that support epithelial repair and nerve regeneration, although greater standardization is needed.

#4

[Recurrence of herpetic eye disease following cataract surgery: risk factors and prevention].

Die Ophthalmologie2026 Mar 10

Herpetic eye disease poses a significant challenge in cataract surgery. On the one hand, patients with a history of herpes infections develop cataracts more rapidly and the surgery itself is associated with a higher rate of intraoperative complications. On the other hand, the risk of herpetic recurrence after cataract surgery is markedly increased (46% for herpes simplex virus and up to 40% for herpes zoster virus). The prognosis is significantly worse in the event of a recurrence, which is why reducing the recurrence rate is of great clinical interest. Preoperatively, attention should be paid to signs of prior herpes infections, such as corneal scars, neovascularization, synechiae and neurotrophic keratopathy. Risk factors for recurrence include the duration of quiescence, the number of previous episodes, a temporal tunnel incision, immunosuppressive therapy, diabetes mellitus and atopy. Prophylactic perioperative antiviral therapy is frequently used; however, randomized data specifically addressing cataract surgery are lacking, so that current recommendations are largely based on indirect evidence and expert consensus. Herpesvirus-assoziierte Augenerkrankungen stellen eine relevante Herausforderung in der Kataraktchirurgie dar. Einerseits entwickeln Patienten mit vorausgegangener Herpesinfektionen schneller eine Katarakt, wobei die Operation selbst mit einer erhöhten intraoperativen Komplikationsrate einhergeht. Zum anderen ist das Rezidivrisiko nach Kataraktoperation deutlich erhöht (46 % bei dem Herpes-simplex-Virus und bis zu 40 % bei dem Herpes-zoster-Virus). Die Prognose ist bei einem Rezidiv deutlich schlechter, weswegen die Reduktion der Rezidivrate von hohem Interesse ist. Präoperativ sollte auf Zeichen früherer Herpesinfektionen wie Hornhautnarben, Neovaskularisationen, Synechien und eine neurotrophe Keratopathie geachtet werden. Zu den Risikofaktoren für ein Rezidiv zählen die Dauer der Schubfreiheit, die Anzahl an früheren Schüben, ein Tunnelschnitt von temporal, immunsuppressive Therapie, Diabetes mellitus und Atopie. Eine prophylaktische perioperative antivirale Therapie wird häufig eingesetzt, randomisierte Daten speziell für die Kataraktoperation fehlen jedoch, sodass die Empfehlung derzeit überwiegend auf indirekter Evidenz und Expertenkonsens beruht.

#5

Clinical evidence on allogeneic blood-derived eye drops in ocular surface and corneal diseases.

The ocular surface2026 Mar 05

To systematically review clinical studies on allogeneic blood-derived eye drops in ocular surface and corneal diseases. A PRISMA-guided search of PubMed, Google Scholar, Scopus, Web of Science, and Embase (1985-September 2025) identified eligible clinical studies on allogeneic eye drops for ocular surface and corneal diseases. Two reviewers independently extracted data and assessed study quality using Joanna Briggs Institute tools. Sixty studies (1733 participants; 2374 eyes) met inclusion criteria, with 1423 patients (1738 eyes) receiving allogeneic eye drops. The cohort included 929 females (53.6%), 562 males (32.4%), and 242 unspecified (14.0%), with a mean age of 42.3 ± 21.8 years. Indications were dry eye disease (30.0%), mixed ocular surface disorders (21.7%), ligneous conjunctivitis (20.0%), persistent epithelial defects, erosions, and ulcers (18.3%), neurotrophic keratopathy (5.0%), and chemical or toxin-related injuries (5.0%). Treatments comprised allogeneic serum (47.4%), umbilical cord serum (43.2%), cord blood platelet lysate (10.1%), platelet-poor plasma/fresh frozen plasma (6.0%), plasma rich in growth factors (2.1%), and platelet-rich plasma (0.5%). Across 1738 treated eyes, reported outcomes were generally consistent, with improvements observed in patient-reported symptoms, epithelial healing, visual outcomes, and adverse events reported in <2% of cases. In dry eye disease, the largest relative improvements were reported for symptom scores (28.3%) and epithelial healing (24.9%), with smaller gains in tear-based measures (14-17%). Mixed ocular surface disorders showed symptom relief (28.5%), epithelial healing (16.9%), and BCVA improvement (7.7%). Ligneous conjunctivitis achieved complete/near remission in 1.5%, persistent epithelial defects healed in 10.2% with 0.5% recurrence, neurotrophic keratopathy showed 2.1% resolution with improved sensitivity, and chemical burns achieved 2.0% complete closure with better clarity and vision. Overall, patient-reported symptoms and epithelial healing emerged as the most consistently responsive outcomes, while reported safety profiles remained favorable. While many studies demonstrated acceptable methodological quality within their respective designs, the overall evidence base was predominantly composed of observational and non-comparative studies, which limits overall certainty. Allogeneic blood-derived eye drops are associated with meaningful clinical improvement across ocular surface diseases and demonstrate favorable short-to mid-term safety. Further controlled studies with standardized protocols, longer follow-up, and cost-effectiveness analyses are needed.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC232 artigos no totalmostrando 199

2026

Corneal thinning and perforation: a narrative review of diagnostic and stepwise management strategies.

International ophthalmology
2026

Systematic Literature Review on the Effect of Cenegermin on Corneal Sensation and Innervation in Patients with Neurotrophic Keratopathy.

Journal of ocular pharmacology and therapeutics : the official journal of the Association for Ocular Pharmacology and Therapeutics
2026

The Role of Innervation in Ocular Surface Homeostasis, Tissue Remodeling Following Nerve Injury, and the Therapeutic Potential of Hemocomponents in Neuronal and Cicatricial Pathologies.

Journal of clinical medicine
2026

[Recurrence of herpetic eye disease following cataract surgery: risk factors and prevention].

Die Ophthalmologie
2026

Clinical evidence on allogeneic blood-derived eye drops in ocular surface and corneal diseases.

The ocular surface
2026

Cross face nerve grafting for reinnervation of nasal and tongue mucosa following trigeminal nerve injury.

JPRAS open
2026

Recovery of corneal sensation following eyelid surgery for neurotrophic keratopathy and preganglionic facial palsy.

The British journal of ophthalmology
2026

Neurotrophic keratopathy in childhood: advances in understanding of pathogenesis and management.

Eye (London, England)
2026

Proteomic analysis of corneal epithelial cells in patients with neurotrophic keratopathy.

Experimental eye research
2026

Molecular landscape connecting complications of dry eye disease: mechanisms, therapeutic targets, biomarkers, and future innovations.

International ophthalmology
2026

Association Between Neurotrophic Keratitis and Limbal Stem Cell Deficiency.

American journal of ophthalmology
2026

Management of persistent corneal epithelial defects: role of insulin.

Current opinion in ophthalmology
2026

Neurotrophic keratopathy patients after minimally invasive corneal neurotization treated with RhNGF eyedrops: a case series.

Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie
2026

Letter to Editor: Prospective consecutive case series of patients with neurotrophic keratopathy associated with unilateral total limbal stem cell deficiency caused by severe ocular surface burns.

The ocular surface
2025

Shelf-Stable, Cryopreserved Amniotic Membrane for the Management of Ocular Surface Disease: A Retrospective Assessment.

Clinical optometry
2026

Real-world effectiveness of topical insulin for neurotrophic keratopathy: retrospective case series of 29 eyes.

Canadian journal of ophthalmology. Journal canadien d'ophtalmologie
2025

Neurotrophic Keratopathy in Herpes Zoster Ophthalmicus.

Eye &amp; contact lens
2025

Long-term outcomes of corneal neurotization for neurotrophic keratopathy: a dual center, single surgeon retrospective study.

Plastic and reconstructive surgery
2025

Indirect Corneal Neurotization Using the Infraorbital Nerve and Sural Nerve Interposition Graft: A Case Report.

The Journal of craniofacial surgery
2025

Therapeutic Outcomes of Shelf-Stable, Cryopreserved Amniotic Membrane in Recalcitrant Keratopathy.

Clinical ophthalmology (Auckland, N.Z.)
2025

Medical and surgical interventions for neurotrophic keratopathy.

The Cochrane database of systematic reviews
2025

In vivo confocal microscopy (IVCM) analysis of corneal sub-basal nerve plexus (SNP) and corneal sensitivity after micropulse transscleral cyclophotocoagulation (MP-TSCPC) in glaucoma patients.

International ophthalmology
2025

Alterations of the ocular surface during diabetes: molecular mechanisms and therapeutic strategies.

International ophthalmology
2025

Polyneuritis cranialis following actinomycosis of a wisdom tooth: a case report.

Journal of medical case reports
2025

Outcome of Cenegermin in the Treatment of Neurotrophic Keratopathy.

Cornea
2025

Aging and Corneal Nerve Health: Mechanisms of Degeneration and Emerging Therapies for the Cornea.

Cells
2025

AQP5 Regulates Corneal Nerve Distribution Through NETRIN-1 and SLIT3.

Investigative ophthalmology &amp; visual science
2025

Anesthetic Considerations in Pediatric Corneal Neurotization: A Case Report.

Cureus
2025

New Phenotype in Two Siblings with Familial FLVCR1 Mutation: Neurotrophic Keratopathy.

Turkish journal of ophthalmology
2025

Clinical Outcomes of Corneal Neurotization in Patients With Herpetic Neurotrophic Keratopathy.

Annals of plastic surgery
2025

Efficacy of Corneal Neurotization Surgery in Eyes With Neurotrophic Keratopathy: A Systematic Review and Meta-Analysis.

Annals of plastic surgery
2025

Cenegermin for the Treatment of Moderate or Severe Neurotrophic Keratopathy: Results from a Prospective, Phase IV, Open-Label Study in China.

Ophthalmology and therapy
2025

Management of refractory persistent epithelial defect treatment with topical insulin: An Indian pilot study.

Indian journal of ophthalmology
2025

The role of limbal stem cells on neurotrophic cornea.

Clinical &amp; experimental optometry
2025

Severe Aqueous-Deficient Dry Eye Following Herpes Zoster Ophthalmicus With Periocular Inflammation: A Case Series.

Cureus
2025

Indirect Corneal Neurotization: Long-Term Outcomes Using a Sural Nerve Graft in Patients With Facial Palsy and Neurotrophic Keratopathy.

Cornea
2025

Topical Cenegermin for Pediatric Neurotrophic Keratopathy: A Review of the Literature and Systematic Review.

Cornea
2025

Congenital Corneal Anesthesia Secondary to Brainstem Ischemia as a Rare Complication of Neonatal Hypoxic-Ischemic Encephalopathy.

Cureus
2025

Corneal neurotization meets COVID-19: a case report of minimally invasive corneal neurotization complicated by COVID-19-related keratitis.

Journal of ophthalmic inflammation and infection
2025

Oculoplastic Interventions in the Management of Ocular Surface Diseases: A Comprehensive Review.

Life (Basel, Switzerland)
2025

The Therapeutic Potential of Insulin Eye Drops in Neurotrophic Keratopathy: A Comprehensive Review.

Biomedicines
2025

Evaluation of Corneal Sensitivity: Tools We Have.

Diagnostics (Basel, Switzerland)
2025

Prospective consecutive case series of patients with neurotrophic keratopathy associated with unilateral total limbal stem cell deficiency caused by severe ocular surface burns.

The ocular surface
2025

Real-World Outcomes of Allogeneic Immunosafe Plasma Rich in Growth Factors Eye Drops for Refractory Ocular Surface Diseases: A Prospective Observational Study.

Ophthalmology and therapy
2025

Impact of topical insulin as a therapeutic intervention for corneal wounds: A comprehensive systematic review and meta-analysis.

European journal of ophthalmology
2026

Cenegermin treatment for pediatric neurotrophic keratopathy: a systematic review.

Canadian journal of ophthalmology. Journal canadien d'ophtalmologie
2025

Utility of cenegermin for the management of neurotrophic keratopathy after penetrating keratoplasty.

Oman journal of ophthalmology
2025

Minimally invasive corneal neurotization surgery and outcomes in a rural population.

International ophthalmology
2025

The Role of Nerve Growth Factor on the Ocular Surface: A Review of the Current Experimental Research and Clinical Practices.

International journal of molecular sciences
2025

Corneal Neurotization Using the Supratrochlear Nerve With a Great Auricular Nerve Graft for Neurotrophic Keratopathy: Long-Term Results.

American journal of ophthalmology
2025

Periocular Management of Pediatric Facial Nerve Palsy.

American journal of ophthalmology
2025

Corneal Sensory Denervation Causes Epithelial Ferroptosis and Delayed Healing in Mice.

Investigative ophthalmology &amp; visual science
2025

Corneal Neurotization in Elderly Patients with Multimorbidity.

Current eye research
2025

Topical insulin as a novel treatment for persistent epithelial defects and other ocular surface disorders: a systematic review.

Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie
2025

Impact of neurotrophic keratopathy on quality of life: a utility analysis.

Canadian journal of ophthalmology. Journal canadien d'ophtalmologie
2026

Outcomes of Corneal Transplantation After Minimally Invasive Corneal Neurotization in Children.

Cornea
2025

Corneal Neurotization: Paving the Path to Reinnervation.

Otolaryngologic clinics of North America
2025

Corneal Neurotization, Recent Progress, and Future Perspectives.

Biomedicines
2025

Neurotrophic Keratopathy in Pediatric Population: An IRIS® Registry Report.

Ophthalmology
2025

Corneal neurotization for the treatment of neurotrophic keratopathy.

Current opinion in ophthalmology
2025

A Narrative Medicine Approach to Navigating Barriers to the Diagnosis of Pediatric Neurotrophic Keratopathy.

American journal of ophthalmology
2025

Biological topicals in ocular surface disorders.

Indian journal of ophthalmology
2025

Neurotrophic keratopathy: Update in diagnosis and management.

Indian journal of ophthalmology
2025

Bilateral In Vivo Confocal Microscopic Changes of the Corneal Subbasal Nerve Plexus in Patients with Acute Herpes Zoster Ophthalmicus.

Ophthalmology and therapy
2025

In Vitro Evaluation of Gelatin-Based Hydrogels as Potential Fillers for Corneal Wounds.

Biomacromolecules
2025

[Neurotrophic keratopathy and corneal ulcers in diabetes mellitus].

Die Ophthalmologie
2025

Corneal Recovery After Neurotization for Neurotrophic Keratopathy.

JAMA ophthalmology
2025

Preliminary report of minimally invasive corneal neurotization in patients with neurotrophic keratopathy in Southern China.

BMC ophthalmology
2023

Sandwich Technique and Amniotic Membrane Transplantation as an Effective Method in Repairing Corneal Perforations.

Middle East African journal of ophthalmology
2025

Corneal Ring Infiltrate Following Micropulse Cyclophotocoagulation.

The American journal of case reports
2025

Disrupted Mitochondrial Dynamics Impair Corneal Epithelial Healing in Neurotrophic Keratopathy.

International journal of molecular sciences
2025

Techniques in Corneal Neurotization.

Atlas of the oral and maxillofacial surgery clinics of North America
2025

Topical insulin in pediatric neurotrophic keratopathy associated with CIPA syndrome.

Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus
2025

Nerve growth factor loaded hypotonic eye drops for corneal nerve repair.

Journal of controlled release : official journal of the Controlled Release Society
2025

Incidence of corneal perforation and lagophthalmos in patients with leprosy in Japan based on 40-year observations.

Japanese journal of ophthalmology
2025

Substance P- and Insulin-like Growth Factor 1-derived Tetrapeptides for Neurotrophic Keratopathy Related to Leprosy: A Clinical Trial.

Ophthalmology science
2024

Autologous blood in the management of ocular surface disorders.

World journal of experimental medicine
2025

The role of lysophosphatidic acid and its receptors in corneal nerve regeneration.

The ocular surface
2024

Topical insulin used alone or in combination with drug-depository contact lens for refractory cases of neurotrophic keratopathy.

American journal of ophthalmology case reports
2025

Conjunctival Neuroma After Corneal Neurotization in a Patient With Neurotrophic Keratopathy.

Cornea
2024

The THINK Study: Testing Hypoesthesia and the Incidence of Neurotrophic Keratopathy in Cataract Patients with Dry Eye.

Clinical ophthalmology (Auckland, N.Z.)
2024

Neuronal Intranuclear Inclusion Disease with a Corneal Disorder: A Case Report.

Medicina (Kaunas, Lithuania)
2024

Comparison of Cochet-Bonnet, Dental Floss, and Cotton Wisp With Applications to Corneal Sensation Testing.

Cornea
2025

Long-term outcomes of sural nerve vertical cross-face graft for lacrimal gland neurotization in neurodeprivative dry eye.

Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie
2024

Scleritis and episcleritis in patients with idiopathic small fiber neuropathy.

American journal of ophthalmology case reports
2025

Concurrent Limbal Stem Cell Deficiency and Mild Neurotrophic Keratopathy in Graft-Vs-Host Disease.

Cornea
2024

Can Inactivated Coronavirus Disease 2019 Vaccine Cause Bilateral Neurotrophic Keratopathy?

Journal of current ophthalmology
2024

Topical Insulin for Neurotrophic-Related Epithelial Defects: Where do We Stand? A Systematic Review.

Journal of current ophthalmology
2025

Nerve Growth Factor Treatment for Neurotrophic Keratopathy in the IRIS® Registry.

Ophthalmology
2024

Topical Application of 0.05% Cyclosporine for the Treatment of Neurotrophic Keratopathy Secondary to Herpes Simplex Keratitis.

Clinical therapeutics
2024

Preventing and treating neurotrophic keratopathy by a single intrastromal injection of AAV-mediated gene therapy.

The ocular surface
2024

Current perspectives and concerns in corneal neurotization.

Indian journal of ophthalmology
2024

Single-Stage Bilateral Corneal Neurotization for Neurotrophic Keratopathy in Stüve-Wiedemann Syndrome: A Case Report and Literature Review.

Journal of pediatric ophthalmology and strabismus
2025

Long-Term Evaluation of Patients with Neurotrophic Keratopathy Undergoing Staged Keratoplasty After Corneal Neurotization.

Current eye research
2024

Corneal reinnervation in patients with severe neurotrophic keratopathy secondary to herpes zoster ophthalmicus after treatment with autologous serum tear drops.

Cornea open
2025

Clodronate liposome treatment contributes to the nerve regeneration in corneal nerve involvement of diabetic mice.

Experimental animals
2024

The use of rh-NGF in the management of neurotrophic keratopathy.

Frontiers in ophthalmology
2024

Detection of Subclinical Neurotrophic Keratopathy by Noncontact Esthesiometry.

Ophthalmology and therapy
2024

The Utilization of Topical Insulin for Ocular Surface Diseases: A Narrative Review.

Cureus
2024

Tear Fluid Progranulin as a Noninvasive Biomarker for the Monitoring of Corneal Innervation Changes in Patients With Type 2 Diabetes Mellitus.

Translational vision science &amp; technology
2024

Co-existence of Neurotrophic Keratopathy in Eyes With Limbal Stem Cell Deficiency.

American journal of ophthalmology
2024

Development and Characterization of a Photocrosslinkable, Chitosan-Based, Nerve Growth Factor-Eluting Hydrogel for the Ocular Surface.

Translational vision science &amp; technology
2024

Corneal Nerve Assessment by Aesthesiometry: History, Advancements, and Future Directions.

Vision (Basel, Switzerland)
2024

Topical insulin in neurotrophic keratopathy after diabetic vitrectomy.

Scientific reports
2024

Neurotrophic keratopathy: General features and new therapies.

Survey of ophthalmology
2024

Insights on the Choice and Preparation of the Donor Nerve in Corneal Neurotization for Neurotrophic Keratopathy: A Narrative Review.

Journal of clinical medicine
2024

Iris neovascularization and neurotrophic keratopathy following ultrasound cycloplasty in refractory glaucoma: case series.

BMC ophthalmology
2024

Corneal Neurotization for Neurotrophic Keratopathy: A Multicenter Experience.

Ophthalmic plastic and reconstructive surgery
2024

The role of topical insulin in ocular surface restoration: A review.

Survey of ophthalmology
2024

Predictive performance of corneal and lid margin sensitivity for dry eye disease: An investigator-masked, prospective, prognostic accuracy study.

The ocular surface
2024

Substance P promotes transforming growth factor-β-induced collagen synthesis in human corneal fibroblasts.

American journal of physiology. Cell physiology
2024

Outcomes of Boston Keratoprosthesis Type I Implantation in Poland: A Retrospective Study on 118 Patients.

Journal of clinical medicine
2024

Neurotrophic Keratopathy in Marfan Syndrome Patient After Micropulse Transscleral Cyclophotocoagulation: A Call for Risk Stratification.

The American journal of case reports
2024

Corneal Neurotization: Essentials for The Facial Paralysis Surgeon.

Facial plastic surgery : FPS
2024

Establishment of mouse model of neurotrophic keratopathy through TRPV1 neuronal ablation.

Experimental eye research
2023

Topical Insulin in Neurotrophic Keratopathy: A Review of Current Understanding of the Mechanism of Action and Therapeutic Approach.

Pharmaceutics
2024

Neurotrophic Keratopathy after wide retinal endolaser and postoperative Ketorolac eye drops: A case series.

European journal of ophthalmology
2024

Minimally Invasive Corneal Neurotization: 10-Year Update in Technique Including Novel Donor Transfer of the Great Auricular Nerve.

Plastic and reconstructive surgery
2024

Phase IV Multicenter, Prospective, Open-Label Clinical Trial of Cenegermin (rhNGF) for Stage 1 Neurotrophic Keratopathy (DEFENDO).

Ophthalmology and therapy
2024

Neurotrophic keratopathy: Clinical presentation and outcomes in 354 eyes in a community-based population.

European journal of ophthalmology
2024

A New Treatment for Recalcitrant Neurotrophic Keratopathy of Ocular Graft-Versus-Host Disease with Virus Infection.

Ophthalmology and therapy
2023

Diagnostic performance of real-time quantitative PCR in tear samples in various subtypes of herpes simplex keratitis.

Journal of clinical microbiology
2024

Corneal Superficial Plaque Formation After Recombinant Human Nerve Growth Factor Use in a Patient With Neurotrophic Keratopathy and Limbal Stem Cell Deficiency From Mucous Membrane Pemphigoid.

Cornea
2023

Clinical outcomes of corneal neurotization using sural nerve graft in neurotrophic keratopathy.

PloS one
2023

Effect of recombinant human nerve growth factor treatment on corneal nerve regeneration in patients with neurotrophic keratopathy.

Frontiers in neuroscience
2023

Neurotrophic Keratopathy and Topical Insulin Therapy: A Case Report.

Cureus
2024

Outcomes and success of amniotic membrane transplantation for the treatment of corneal diseases.

Cutaneous and ocular toxicology
2023

Corneal Neurotization: Preoperative Patient Workup and Surgical Decision-making.

Plastic and reconstructive surgery. Global open
2023

Indications and outcomes of keratoplasty ≤ 5.5 mm diameter ("mini-keratoplasty").

BMC ophthalmology
2024

Chitosan-based thermosensitive hydrogel with long-term release of murine nerve growth factor for neurotrophic keratopathy.

Neural regeneration research
2023

Use of PROSE for long-term ocular surface support in patients with a permanent keratoprosthesis.

American journal of ophthalmology case reports
2023

Herpes simplex virus keratitis: electronic medical records driven big data analytics report from a tertiary eye institute of South India.

International ophthalmology
2023

Neurotrophic keratopathy: An updated understanding.

The ocular surface
2024

Corneal Sensitivity Is Inversely Correlated With Severity of Diabetic Retinopathy in a Predominantly Underrepresented Population.

American journal of ophthalmology
2023

The Role of Sensory Innervation in Homeostatic and Injury-Induced Corneal Epithelial Renewal.

International journal of molecular sciences
2023

Semiscleral Contact Lens Use After Direct Corneal Neurotization for Neurotrophic Keratopathy.

Eye &amp; contact lens
2023

Efficacy of the Buried Temporary Suture Tarsorrhaphy: A Novel Technique.

Ophthalmic plastic and reconstructive surgery
2024

Ocular adverse events of cenegermin used in neurotrophic keratopathy: an analysis of the FDA adverse event reporting system database.

Expert opinion on drug safety
2024

Combined Use of Therapeutic Hyper-CL Soft Contact Lens and Insulin Eye Drops for the Treatment of Recalcitrant Neurotrophic Keratopathy.

Cornea
2023

Neurotrophic keratopathy associated with cancer immunotherapy targeting programmed death 1.

The ocular surface
2023

Neurotrophic Keratopathy After Slow Coagulation Transscleral Cyclophotocoagulation.

Cornea
2023

Evaluation of Corneal Nerve Regeneration After Minimally Invasive Corneal Neurotization.

Asia-Pacific journal of ophthalmology (Philadelphia, Pa.)
2024

Clinical associations of corneal neuromas with ocular surface diseases.

Neural regeneration research
2023

Urokinase-type plasminogen activator promotes corneal epithelial migration and nerve regeneration.

Experimental eye research
2023

Ocular-Surface Regeneration Therapies for Eye Disorders: The State of the Art.

Biotech (Basel (Switzerland))
2023

New Promising Therapeutic Approach for Refractory Corneal Epithelial Defects.

Cureus
2024

Neurotrophic keratopathy following rhegmatogenous retinal detachment surgery.

Canadian journal of ophthalmology. Journal canadien d'ophtalmologie
2023

Detection of Subclinical Neurotrophic Keratopathy by Non-Contact Esthesiometry.

Research square
2023

Trigeminal nerve-derived substance P regulates limbal stem cells by the PI3K-AKT pathway.

iScience
2024

Guillain-Barré Syndrome With Associated Bilateral Neurotrophic Keratopathy.

Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
2023

Prevalence and clinical characteristics of neurotrophic keratopathy in hispanic population in northeastern Mexico.

International ophthalmology
2023

Autologous serum eye drops in dry eye disease: Preferred practice pattern guidelines.

Indian journal of ophthalmology
2023

[Advancements in corneal sensory reconstruction for the treatment of neurotrophic keratopathy].

[Zhonghua yan ke za zhi] Chinese journal of ophthalmology
2023

Corneal Neurotization-Indications, Surgical Techniques and Outcomes.

Journal of clinical medicine
2023

Cenegermin for the treatment of dry eye disease.

Drugs of today (Barcelona, Spain : 1998)
2023

Cost and Prescriber and Patient Characteristics of Cenegermin Use in the Medicare Population.

American journal of ophthalmology
2023

Noxious effects of riot control agents on the ocular surface: Pathogenic mechanisms and management.

Frontiers in toxicology
2023

Umbilical Cord Graft for Refractory Neurotrophic Keratopathy.

Cornea
2023

Applications of mesenchymal stem cells in ocular surface diseases: sources and routes of delivery.

Expert opinion on biological therapy
2023

Minimally invasive corneal neurotization for neurotrophic keratopathy: The potential effect of age, denervation chronicity and lesion location.

American journal of ophthalmology case reports
2023

Phialophora chinensis fungal keratitis: An initial case report and species identification.

American journal of ophthalmology case reports
2023

Can Nerve Growth Factor (NGF) Be a Treatment Option for Pediatric Eye Diseases?

Seminars in ophthalmology
2023

Corneal nerve regeneration is affected by scar location in herpes simplex keratitis: A longitudinal in vivo confocal microscopy study.

The ocular surface
2022

0.1% RGN-259 (Thymosin ß4) Ophthalmic Solution Promotes Healing and Improves Comfort in Neurotrophic Keratopathy Patients in a Randomized, Placebo-Controlled, Double-Masked Phase III Clinical Trial.

International journal of molecular sciences
2022

[Modern capabilities in diagnosis and treatment of neurotrophic keratopathy].

Vestnik oftalmologii
2023

Corneal neurotization in the management of neurotrophic keratopathy: A review of the literature.

Journal francais d'ophtalmologie
2022

Observational study of cenegermin for the treatment of limbal stem cell deficiency associated with neurotrophic keratopathy.

Therapeutic advances in ophthalmology
2023

Omega-3 polyunsaturated fatty acids and corneal nerve health: Current evidence and future directions.

The ocular surface
2023

Bilateral Corneal Neurotization for Ramos-Arroyo Syndrome and Developmental Neurotrophic Keratopathy: Case Report and Literature Review.

Cornea
2022

[CORNEAL NEUROTIZATION FOR NEUROTROPHIC KERATOPATHY].

Harefuah
2022

Neurotrophic Keratopathy Treated with Topical Recombinant Human Nerve Growth Factor (Cenegermin): Case Series Study with Long-Term Follow-Up.

Case reports in ophthalmology
2022

Ophthalmic findings of congenital insensitivity to pain with anhidrosis with a novel neurotrophic tyrosine kinase receptor type 1 gene mutation: A case report.

Frontiers in medicine
2022

Waardenburg syndrome-associated neurotrophic keratopathy in a child treated with neurotization surgery.

Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus
2022

Medium-term Outcomes of Micropulse Transscleral Cyclophotocoagulation in Refractory Glaucoma.

Journal of current glaucoma practice
2022

Ocular manifestations among patients with congenital insensitivity to pain due to variants in PRDM12 and SCN9A genes.

American journal of medical genetics. Part A
2022

Sustained Release of Tacrolimus From a Topical Drug Delivery System Promotes Corneal Reinnervation.

Translational vision science &amp; technology
2022

Efficacy of Recombinant Human Nerve Growth Factor in Stage 1 Neurotrophic Keratopathy.

Ophthalmology
2022

Exploration of biomimetic poly(γ-benzyl-L-glutamate) fibrous scaffolds for corneal nerve regeneration.

Journal of materials chemistry. B
2022

Ocular pain in ocular graft-versus-host disease patients with neurotrophic keratopathy.

The ocular surface
2023

[Autologous serum eye drops for therapy-resistant epithelial defects of the cornea : Impact of underlying disease and simultaneous amniotic transplantation in 990 applications].

Die Ophthalmologie
2022

Physicochemical and microbiological stability of insulin eye drops in an artificial tear vehicle used in the treatment of refractory neurotrophic keratopathy.

Journal francais d'ophtalmologie
2022

Prevalence of neurotrophic keratopathy in patients with chronic ocular graft-versus-host disease.

The ocular surface
2022

Aberrant Corneal Homeostasis in Neurosurgery-Induced Neurotrophic Keratopathy.

Journal of clinical medicine
2022

[Treatment of neurotrophic keratopathy in combined lesions of the trigeminal and facial nerves].

Vestnik oftalmologii
2022

Neurotrophic Keratopathy in the United States: An Intelligent Research in Sight Registry Analysis.

Ophthalmology
2022

Prophylactic Laser Treatment Posterior to Pars Plana Vitrectomy Sclerotomy Wounds During Macular Surgery.

Kansas journal of medicine
2022

Complications in Retinal Surgery: A Review of Corneal Changes Following Vitreoretinal Procedures.

International ophthalmology clinics
2022

Recombinant Human Nerve Growth Factor for Pediatric Neurotrophic Keratopathy.

Eye &amp; contact lens
2022

Neurotrophic Keratopathy in Systemic Diseases: A Case Series on Patients Treated With rh-NGF.

Frontiers in medicine
2022

Minimally invasive, indirect corneal neurotization using an ipsilateral sural nerve graft for early neurotrophic keratopathy.

American journal of ophthalmology case reports
2022

Neurotization of the human cornea - A comprehensive review and an interim report.

Indian journal of ophthalmology
2022

Neurotrophic Keratitis Due to Congenital Corneal Anesthesia with Deafness, Hypotonia, Intellectual Disability, Face Abnormality and Metabolic Disorder: A New Syndrome?

Medicina (Kaunas, Lithuania)
2022

Minimally Invasive Corneal Neurotization Provides Sensory Function, Protects Against Recurrent Ulceration, and Improves Visual Acuity.

American journal of ophthalmology
2022

A multicenter report of the use of plasma rich in growth factors (PRGF) for the treatment of patients with ocular surface diseases in North America.

The ocular surface
2022

Clinical and instrumental assessment of the corneal healing in moderate and severe neurotrophic keratopathy treated with rh-NGF (Cenegermin).

European journal of ophthalmology
2022

Topical cenegermin 0.002% for pediatric neurotrophic keratopathy.

European journal of ophthalmology
2022

Neurotrophic keratopathy: Clinical presentation and effects of cenegermin.

American journal of ophthalmology case reports
2022

The Role of Hi-Tech Devices in Assessment of Corneal Healing in Patients with Neurotrophic Keratopathy.

Journal of clinical medicine
2022

Pillar tarsoconjunctival flap: An alternative approach for the management of refractory corneal ulcer.

European journal of ophthalmology
Ver todos os 232 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

Ainda não temos associações cadastradas para Queratopatia neurotrófica.

É de uma associação que acompanha esta doença? Fale com a gente →

Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Queratopatia neurotrófica

Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.

Tire suas dúvidas

Perguntas, dicas e experiências compartilhadas aqui na página

Participe da discussão

Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.

Fazer login

Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Corneal thinning and perforation: a narrative review of diagnostic and stepwise management strategies.
    International ophthalmology· 2026· PMID 41874780mais citado
  2. Systematic Literature Review on the Effect of Cenegermin on Corneal Sensation and Innervation in Patients with Neurotrophic Keratopathy.
    Journal of ocular pharmacology and therapeutics : the official journal of the Association for Ocular Pharmacology and Therapeutics· 2026· PMID 41852024mais citado
  3. The Role of Innervation in Ocular Surface Homeostasis, Tissue Remodeling Following Nerve Injury, and the Therapeutic Potential of Hemocomponents in Neuronal and Cicatricial Pathologies.
    Journal of clinical medicine· 2026· PMID 41827440mais citado
  4. [Recurrence of herpetic eye disease following cataract surgery: risk factors and prevention].
    Die Ophthalmologie· 2026· PMID 41805863mais citado
  5. Clinical evidence on allogeneic blood-derived eye drops in ocular surface and corneal diseases.
    The ocular surface· 2026· PMID 41794131mais citado
  6. Descemet Membrane Anterior Keratoplasty With BrightMEM Corneal Allograft: An Innovative Technique for Treatment of Nonhealing Diseases of the Ocular Surface.
    Cornea· 2026· PMID 41925731recente
  7. The Therapeutic Approach and Mechanism of Action of Insulin Eye Drops for the Treatment of Neurotrophic Keratitis.
    Drug Des Devel Ther· 2026· PMID 41913732recente
  8. Concomitant Tarsorrhaphy During Penetrating Keratoplasty for Promoting Epithelial Healing in Neurotrophic Keratopathy.
    Am J Ophthalmol· 2026· PMID 41887389recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:137596(Orphanet)
  2. MONDO:0015290(MONDO)
  3. GARD:19878(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q1739188(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Queratopatia neurotrófica
Compêndio · Raras BR

Queratopatia neurotrófica

ORPHA:137596 · MONDO:0015290
Prevalência
Unknown
Herança
Not applicable
CID-10
H16.2 · Ceratoconjuntivite
CID-11
Ensaios
4 ativos
Início
Adult, Childhood
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0339296
EuropePMC
Wikidata
Papers 10a
DiscussaoAtiva

Nenhuma novidade ainda. O agente esta monitorando.

0membros
0novidades