Emergency cesarean section in a pregnant woman with Freeman-Sheldon syndrome and acute respiratory distress. A Case report

Freeman-Sheldon syndrome is a rare genetic disorder characterized by a wide spectrum of bone abnormalities, joint contractures, and typical facial features. Facial abnormalities of microstomia, micrognathia, and macroglossia constitute an impediment to accessing the airway. On the other hand, skelet...

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Main Author: Viviana de la Caridad Sáez Cantero
Format: Article
Language:Spanish
Published: Centro Provincial de Información de Ciencias Médicas. Cienfuegos 2025-01-01
Series:Medisur
Subjects:
Online Access:http://medisur.sld.cu/index.php/medisur/article/view/45305
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author Viviana de la Caridad Sáez Cantero
author_facet Viviana de la Caridad Sáez Cantero
author_sort Viviana de la Caridad Sáez Cantero
collection DOAJ
description Freeman-Sheldon syndrome is a rare genetic disorder characterized by a wide spectrum of bone abnormalities, joint contractures, and typical facial features. Facial abnormalities of microstomia, micrognathia, and macroglossia constitute an impediment to accessing the airway. On the other hand, skeletal alterations such as joint contractures and scoliosis are a restrictive component for both respiratory function and the evolution of pregnancy. The case of a pregnant woman with Freeman-Sheldon syndrome and a genetic diagnosis of a fetus with stigmata of the syndrome is presented. This patient required termination of pregnancy by emergency cesarean section due to acute respiratory distress secondary to chronic polyhydramnios. Planning the course of action to follow, whether anticipated or emergency, accompanied by a multidisciplinary team, is decisive in the proper management of patients with these characteristics.
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institution Kabale University
issn 1727-897X
language Spanish
publishDate 2025-01-01
publisher Centro Provincial de Información de Ciencias Médicas. Cienfuegos
record_format Article
series Medisur
spelling doaj-art-7ab5eaa6163e4688aab92e6c8bf8c9e22025-01-30T21:29:04ZspaCentro Provincial de Información de Ciencias Médicas. CienfuegosMedisur1727-897X2025-01-01230e45305e453052592Emergency cesarean section in a pregnant woman with Freeman-Sheldon syndrome and acute respiratory distress. A Case reportViviana de la Caridad Sáez Cantero0Hospital General Docente Enrique Cabrera. Boyeros, La habanaFreeman-Sheldon syndrome is a rare genetic disorder characterized by a wide spectrum of bone abnormalities, joint contractures, and typical facial features. Facial abnormalities of microstomia, micrognathia, and macroglossia constitute an impediment to accessing the airway. On the other hand, skeletal alterations such as joint contractures and scoliosis are a restrictive component for both respiratory function and the evolution of pregnancy. The case of a pregnant woman with Freeman-Sheldon syndrome and a genetic diagnosis of a fetus with stigmata of the syndrome is presented. This patient required termination of pregnancy by emergency cesarean section due to acute respiratory distress secondary to chronic polyhydramnios. Planning the course of action to follow, whether anticipated or emergency, accompanied by a multidisciplinary team, is decisive in the proper management of patients with these characteristics.http://medisur.sld.cu/index.php/medisur/article/view/45305artrogriposiscesárea, manejo de la vía aéreainsuficiencia respiratoriapolihidramnios
spellingShingle Viviana de la Caridad Sáez Cantero
Emergency cesarean section in a pregnant woman with Freeman-Sheldon syndrome and acute respiratory distress. A Case report
Medisur
artrogriposis
cesárea, manejo de la vía aérea
insuficiencia respiratoria
polihidramnios
title Emergency cesarean section in a pregnant woman with Freeman-Sheldon syndrome and acute respiratory distress. A Case report
title_full Emergency cesarean section in a pregnant woman with Freeman-Sheldon syndrome and acute respiratory distress. A Case report
title_fullStr Emergency cesarean section in a pregnant woman with Freeman-Sheldon syndrome and acute respiratory distress. A Case report
title_full_unstemmed Emergency cesarean section in a pregnant woman with Freeman-Sheldon syndrome and acute respiratory distress. A Case report
title_short Emergency cesarean section in a pregnant woman with Freeman-Sheldon syndrome and acute respiratory distress. A Case report
title_sort emergency cesarean section in a pregnant woman with freeman sheldon syndrome and acute respiratory distress a case report
topic artrogriposis
cesárea, manejo de la vía aérea
insuficiencia respiratoria
polihidramnios
url http://medisur.sld.cu/index.php/medisur/article/view/45305
work_keys_str_mv AT vivianadelacaridadsaezcantero emergencycesareansectioninapregnantwomanwithfreemansheldonsyndromeandacuterespiratorydistressacasereport