Cargando…
Vascular Injury After Scoliosis Correction in Ehlers-Danlos Syndrome: Proceed With Caution
Ehlers-Danlos syndrome (EDS) is a rare inherited connective tissue disorder characterized by collagen synthesis disruption, resulting in joint hyperlaxity, skin and vascular fragility, and bleeding diathesis. Patients with EDS are susceptible to spinal deformities, with scoliosis accounting for up t...
Autores principales: | , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Wolters Kluwer
2023
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10431467/ https://www.ncbi.nlm.nih.gov/pubmed/37581952 http://dx.doi.org/10.5435/JAAOSGlobal-D-23-00061 |
_version_ | 1785091209726787584 |
---|---|
author | Prabhakar, Gautham Gonuguntla, Rishi K. Momtaz, David Chaput, Christopher Hogue, Grant D. |
author_facet | Prabhakar, Gautham Gonuguntla, Rishi K. Momtaz, David Chaput, Christopher Hogue, Grant D. |
author_sort | Prabhakar, Gautham |
collection | PubMed |
description | Ehlers-Danlos syndrome (EDS) is a rare inherited connective tissue disorder characterized by collagen synthesis disruption, resulting in joint hyperlaxity, skin and vascular fragility, and bleeding diathesis. Patients with EDS are susceptible to spinal deformities, with scoliosis accounting for up to 23.4% of musculoskeletal abnormalities. Conservative management is often trialed initially; however, severe scoliosis can lead to significant sagittal imbalance and cardiopulmonary compromise. Surgical intervention for scoliosis correction in patients with EDS presents unique challenges because of tissue fragility and an increased risk of vascular and wound complications. This case report discusses a 20-year-old man with type II EDS and scoliosis, who experienced retroperitoneal compartment syndrome, significant left lower extremity weakness, and loss of sensation after scoliosis correction surgery. The report also provides an overview of the existing literature on scoliosis surgery outcomes in patients with EDS, highlighting the need for heightened vigilance and cautious surgical approaches. |
format | Online Article Text |
id | pubmed-10431467 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Wolters Kluwer |
record_format | MEDLINE/PubMed |
spelling | pubmed-104314672023-08-17 Vascular Injury After Scoliosis Correction in Ehlers-Danlos Syndrome: Proceed With Caution Prabhakar, Gautham Gonuguntla, Rishi K. Momtaz, David Chaput, Christopher Hogue, Grant D. J Am Acad Orthop Surg Glob Res Rev Case Report Ehlers-Danlos syndrome (EDS) is a rare inherited connective tissue disorder characterized by collagen synthesis disruption, resulting in joint hyperlaxity, skin and vascular fragility, and bleeding diathesis. Patients with EDS are susceptible to spinal deformities, with scoliosis accounting for up to 23.4% of musculoskeletal abnormalities. Conservative management is often trialed initially; however, severe scoliosis can lead to significant sagittal imbalance and cardiopulmonary compromise. Surgical intervention for scoliosis correction in patients with EDS presents unique challenges because of tissue fragility and an increased risk of vascular and wound complications. This case report discusses a 20-year-old man with type II EDS and scoliosis, who experienced retroperitoneal compartment syndrome, significant left lower extremity weakness, and loss of sensation after scoliosis correction surgery. The report also provides an overview of the existing literature on scoliosis surgery outcomes in patients with EDS, highlighting the need for heightened vigilance and cautious surgical approaches. Wolters Kluwer 2023-08-15 /pmc/articles/PMC10431467/ /pubmed/37581952 http://dx.doi.org/10.5435/JAAOSGlobal-D-23-00061 Text en Copyright © 2023 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Orthopaedic Surgeons. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY) (https://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Prabhakar, Gautham Gonuguntla, Rishi K. Momtaz, David Chaput, Christopher Hogue, Grant D. Vascular Injury After Scoliosis Correction in Ehlers-Danlos Syndrome: Proceed With Caution |
title | Vascular Injury After Scoliosis Correction in Ehlers-Danlos Syndrome: Proceed With Caution |
title_full | Vascular Injury After Scoliosis Correction in Ehlers-Danlos Syndrome: Proceed With Caution |
title_fullStr | Vascular Injury After Scoliosis Correction in Ehlers-Danlos Syndrome: Proceed With Caution |
title_full_unstemmed | Vascular Injury After Scoliosis Correction in Ehlers-Danlos Syndrome: Proceed With Caution |
title_short | Vascular Injury After Scoliosis Correction in Ehlers-Danlos Syndrome: Proceed With Caution |
title_sort | vascular injury after scoliosis correction in ehlers-danlos syndrome: proceed with caution |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10431467/ https://www.ncbi.nlm.nih.gov/pubmed/37581952 http://dx.doi.org/10.5435/JAAOSGlobal-D-23-00061 |
work_keys_str_mv | AT prabhakargautham vascularinjuryafterscoliosiscorrectioninehlersdanlossyndromeproceedwithcaution AT gonuguntlarishik vascularinjuryafterscoliosiscorrectioninehlersdanlossyndromeproceedwithcaution AT momtazdavid vascularinjuryafterscoliosiscorrectioninehlersdanlossyndromeproceedwithcaution AT chaputchristopher vascularinjuryafterscoliosiscorrectioninehlersdanlossyndromeproceedwithcaution AT hoguegrantd vascularinjuryafterscoliosiscorrectioninehlersdanlossyndromeproceedwithcaution |