
ResumenObjetivoDescribir los diferentes hallazgos presentados por el síndrome de displasia ectodérmica, ectrodactilia y paladar hendido y proporcionar información que ayude a conocer qué cambios están relacionados con la displasia en sí y cuáles son secundarios.Reporte de casoSe revisaron a una madre e hija con diagnóstico sistémico de síndrome de displasia ectodérmica, ectrodactilia y paladar hendido, ambas presentaron una ausencia de los orificios de salida de las glándulas de Meibomio, ojo seco severo de predominio evaporativo, y además la madre presenta un leucoma corneal central con neovascularización superficial.ConclusionesEl síndrome de displasia ectodérmica, ectrodactilia y paladar hendido puede causar vascularización corneal secundaria a alteraciones crónicas de la superficie ocular, secundaria a la ausencia de los orificios de las glándulas de Meibomio con la consecuente deficiencia de la capa lipídica en la película lagrimal, la cicatrización conjuntival progresiva y la insuficiencia de células límbicas, con afectación de la agudeza visual usualmente en la etapa adulta.AbstractObjectiveDescribe the different findings presented by the ectodermal dysplasia, ectrodactyly and clefting syndrome and provide information to help you know what changes are related to the dysplasia itself and which are secondary.Case reportA mother and daughter with a diagnosis of ectodermal dysplasia, ectrodactyly and clefting syndrome was reviewed, both showed an absence of the ducts of Meibomian glands with a predominantly evaporative dry eye. In addition, the mother has a central leucoma with superficial vascularization.ConclusionsEctodermal dysplasia, ectrodactyly and clefting syndrome can cause corneal vascularization secondary to chronic alterations of the ocular surface associated to absence of Meibomian gland orifices with tear film lipid layer deficiency, cicatricial conjuntivitis, and limbal stem cell deficiency; causing decreased visual acuity in early adulthood.
Enfermedad corneal, Vascularización corneal, Corneal diseases, Cleft lip, Corneal neovascularization, Displasia ectodérmica, Ectodermal dysplasia, RE1-994, Paladar hendido, Síndrome de displasia ectodérmica, ectrodactilia y paladar hendido, Ophthalmology, Cleft palate, Ectodermal dysplasia, ectrodactyl and clefting syndrome
Enfermedad corneal, Vascularización corneal, Corneal diseases, Cleft lip, Corneal neovascularization, Displasia ectodérmica, Ectodermal dysplasia, RE1-994, Paladar hendido, Síndrome de displasia ectodérmica, ectrodactilia y paladar hendido, Ophthalmology, Cleft palate, Ectodermal dysplasia, ectrodactyl and clefting syndrome
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