Suture dehiscence in patients with connective tissue disease: Marfan and Weill-Marchesani syndromes

Marfan and Weill-Marchesani syndromes have a mutation of the fibrillin gene (FBN1), producing alteration of connective tissue, within ophthalmology, it is important to take into consideration this type of diseases in cogenetic alterations in this system will produce modifications at the level of the...

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Veröffentlicht in:Archivos de la Sociedad Española de Oftalmología (English ed.) 2024-11, Vol.99 (11), p.512-516
Hauptverfasser: Valentín-Pastrana Aguilar, M.M., Platas Moreno, I., Muñoz Sanz, N., Sandoval Cortés, B., Herrera Pereiro, J., Jiménez-Alfaro Morote, I.
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container_end_page 516
container_issue 11
container_start_page 512
container_title Archivos de la Sociedad Española de Oftalmología (English ed.)
container_volume 99
creator Valentín-Pastrana Aguilar, M.M.
Platas Moreno, I.
Muñoz Sanz, N.
Sandoval Cortés, B.
Herrera Pereiro, J.
Jiménez-Alfaro Morote, I.
description Marfan and Weill-Marchesani syndromes have a mutation of the fibrillin gene (FBN1), producing alteration of connective tissue, within ophthalmology, it is important to take into consideration this type of diseases in cogenetic alterations in this system will produce modifications at the level of the ocular structures generating problems of various types, Most of the literature refers to ectopia lentis and the complications derived from it, as secondary glaucoma. We present two patients, one with Marfan syndrome and the other with Weill-Marchesani syndrome, who developed lens dislocation, typical of their pathologies, which led them to undergo different surgeries. Both developed suture dehiscence after these surgeries, as a complication derived from their connective tissue involvement. We thus highlight the need for vigilance and extreme caution in the postoperative period of patients affected by this type of syndromes. Los síndromes de Marfan y de Weill-Marchesani presentan mutación del gen de la fibrilina (FBN1), lo que produce alteración del tejido conectivo. Dentro de la oftalmología es importante tener en consideración a este tipo de enfermedades, ya que alteraciones genéticas en este sistema van a producir modificaciones a nivel de las estructuras oculares generando problemas de diversos tipos, en la mayoría de la literatura se hace referencia a la ectopia lentis, y a las complicaciones derivadas de ésta, como glaucoma secundario. Se presentan dos pacientes, uno con síndrome de Marfan y otro con síndrome de Weill-Marchesani, que desarrollaron luxación del cristalino,típicadeestas patologías, que los llevaron a someterse a distintas intervenciones. Ambos tras dichas cirugías desarrollaron dehiscencia de las suturas, como complicación derivada de su afectación del tejido conectivo. Resaltamos así la necesidad de vigilancia y cautela extrema en el postoperatorio de pacientes afectos con este tipo de síndromes.
doi_str_mv 10.1016/j.oftale.2024.09.002
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We present two patients, one with Marfan syndrome and the other with Weill-Marchesani syndrome, who developed lens dislocation, typical of their pathologies, which led them to undergo different surgeries. Both developed suture dehiscence after these surgeries, as a complication derived from their connective tissue involvement. We thus highlight the need for vigilance and extreme caution in the postoperative period of patients affected by this type of syndromes. Los síndromes de Marfan y de Weill-Marchesani presentan mutación del gen de la fibrilina (FBN1), lo que produce alteración del tejido conectivo. Dentro de la oftalmología es importante tener en consideración a este tipo de enfermedades, ya que alteraciones genéticas en este sistema van a producir modificaciones a nivel de las estructuras oculares generando problemas de diversos tipos, en la mayoría de la literatura se hace referencia a la ectopia lentis, y a las complicaciones derivadas de ésta, como glaucoma secundario. 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subjects Adult
Conjunctiva
Conjuntiva
Dehiscencia de suturas
Ectopia lentis
Female
Fibrilina
Fibrillin
Humans
Lens Subluxation - etiology
Lens Subluxation - surgery
Male
Marfan Syndrome - complications
Marfan Syndrome - genetics
Postoperative Complications - etiology
Surgical Wound Dehiscence - etiology
Suture dehiscence
Weill-Marchesani Syndrome - etiology
Weill-Marchesani Syndrome - genetics
title Suture dehiscence in patients with connective tissue disease: Marfan and Weill-Marchesani syndromes
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