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Emergent Cesarean Delivery in a Patient With Freeman-Sheldon Syndrome Complicated by Preeclampsia, Acute Pulmonary Embolism, and Pulmonary Edema: A Case Report

Freeman-Sheldon syndrome (FSS) is an exceedingly rare congenital disorder with an unspecified prevalence. FSS is caused by a mutation in the embryonic skeletal muscle myosin heavy chain 3 gene. Patients may have facial abnormalities that put them at risk of difficult airway intubation. These facial...

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Autores principales: Fayed, Mohamed, Giska, Mark A, Shievitz, Rebekah C, Attali, Ami, Younger, Joshua
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8796958/
https://www.ncbi.nlm.nih.gov/pubmed/35141063
http://dx.doi.org/10.7759/cureus.20802
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author Fayed, Mohamed
Giska, Mark A
Shievitz, Rebekah C
Attali, Ami
Younger, Joshua
author_facet Fayed, Mohamed
Giska, Mark A
Shievitz, Rebekah C
Attali, Ami
Younger, Joshua
author_sort Fayed, Mohamed
collection PubMed
description Freeman-Sheldon syndrome (FSS) is an exceedingly rare congenital disorder with an unspecified prevalence. FSS is caused by a mutation in the embryonic skeletal muscle myosin heavy chain 3 gene. Patients may have facial abnormalities that put them at risk of difficult airway intubation. These facial abnormalities include micrognathia, macroglossia, high-arched palate, prominent forehead, and mid-face hypoplasia. Additionally, skeletal abnormalities such as joint contractures, scoliosis with resultant restrictive lung disease, and camptodactyly (bent fingers) can be noted. These features played an important role in the anesthetic management of our FSS patient. Perioperative planning and optimization were crucial in her anesthetic management as she underwent an urgent cesarean section due to preeclampsia with severe features.
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spelling pubmed-87969582022-02-08 Emergent Cesarean Delivery in a Patient With Freeman-Sheldon Syndrome Complicated by Preeclampsia, Acute Pulmonary Embolism, and Pulmonary Edema: A Case Report Fayed, Mohamed Giska, Mark A Shievitz, Rebekah C Attali, Ami Younger, Joshua Cureus Anesthesiology Freeman-Sheldon syndrome (FSS) is an exceedingly rare congenital disorder with an unspecified prevalence. FSS is caused by a mutation in the embryonic skeletal muscle myosin heavy chain 3 gene. Patients may have facial abnormalities that put them at risk of difficult airway intubation. These facial abnormalities include micrognathia, macroglossia, high-arched palate, prominent forehead, and mid-face hypoplasia. Additionally, skeletal abnormalities such as joint contractures, scoliosis with resultant restrictive lung disease, and camptodactyly (bent fingers) can be noted. These features played an important role in the anesthetic management of our FSS patient. Perioperative planning and optimization were crucial in her anesthetic management as she underwent an urgent cesarean section due to preeclampsia with severe features. Cureus 2021-12-29 /pmc/articles/PMC8796958/ /pubmed/35141063 http://dx.doi.org/10.7759/cureus.20802 Text en Copyright © 2021, Fayed et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Anesthesiology
Fayed, Mohamed
Giska, Mark A
Shievitz, Rebekah C
Attali, Ami
Younger, Joshua
Emergent Cesarean Delivery in a Patient With Freeman-Sheldon Syndrome Complicated by Preeclampsia, Acute Pulmonary Embolism, and Pulmonary Edema: A Case Report
title Emergent Cesarean Delivery in a Patient With Freeman-Sheldon Syndrome Complicated by Preeclampsia, Acute Pulmonary Embolism, and Pulmonary Edema: A Case Report
title_full Emergent Cesarean Delivery in a Patient With Freeman-Sheldon Syndrome Complicated by Preeclampsia, Acute Pulmonary Embolism, and Pulmonary Edema: A Case Report
title_fullStr Emergent Cesarean Delivery in a Patient With Freeman-Sheldon Syndrome Complicated by Preeclampsia, Acute Pulmonary Embolism, and Pulmonary Edema: A Case Report
title_full_unstemmed Emergent Cesarean Delivery in a Patient With Freeman-Sheldon Syndrome Complicated by Preeclampsia, Acute Pulmonary Embolism, and Pulmonary Edema: A Case Report
title_short Emergent Cesarean Delivery in a Patient With Freeman-Sheldon Syndrome Complicated by Preeclampsia, Acute Pulmonary Embolism, and Pulmonary Edema: A Case Report
title_sort emergent cesarean delivery in a patient with freeman-sheldon syndrome complicated by preeclampsia, acute pulmonary embolism, and pulmonary edema: a case report
topic Anesthesiology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8796958/
https://www.ncbi.nlm.nih.gov/pubmed/35141063
http://dx.doi.org/10.7759/cureus.20802
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