Perioperative Anesthetic Management of A Patient with Turner Syndrome Undergoing Two- Flap Palatoplasty Surgical Repair: A Case Report

Abstract Turner's syndrome (TS) is one of the most common sex chromosomal abnormalities; monosomy (XO). The prevalence of TS is about 1 in 2000 to 1 in 2500 live female births. [1] Turner's syndrome can cause a variety of medical and developmental problems, including short height, webbed neck, broad chest with widely spaced nipples, low line hair, low-set ear, pubertal failure, infertility, and failure of ovaries to develop. TS may be associated with heart defects like bicuspid aortic valve (BAV), coarctation of the aorta (COA) and pulmonary arterial hypertension (PAH).[2] Such a combination of TS with cardiac and non-cardiac defects with a wide range of clinical presentations poses a challenge during perioperative management. TS with cleft palate can have a short trachea with a high location of the carina, risking endobronchial intubation and accidental extubation of the endotracheal tube. [3] The prevalence of cleft palate in patients with TS has not been described in the literature, as not many cases have been reported on the perioperative management of a patient with TS with cleft palate undergoing palatoplasty. We describe the perioperative anaesthetic management of 14 years-6 months, short statured female with TS undergoing bardach,s two-flap palatoplasty repair after obtaining informed consent from the parents.

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Publication Details

Journal
Zenodo (CERN European Organization for Nuclear Research)
Published
2024-11-30
DOI
https://doi.org/10.5281/zenodo.15448859
Primary Topic
Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities
Type
article
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article

Perioperative Anesthetic Management of A Patient with Turner Syndrome Undergoing Two- Flap Palatoplasty Surgical Repair: A Case Report

Zenodo (CERN European Organization for Nuclear Research)
Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities
article

Perioperative Anesthetic Management of A Patient with Turner Syndrome Undergoing Two- Flap Palatoplasty Surgical Repair: A Case Report

article en

Abstract

Abstract Turner's syndrome (TS) is one of the most common sex chromosomal abnormalities; monosomy (XO). The prevalence of TS is about 1 in 2000 to 1 in 2500 live female births. [1] Turner's syndrome can cause a variety of medical and developmental problems, including short height, webbed neck, broad chest with widely spaced nipples, low line hair, low-set ear, pubertal failure, infertility, and failure of ovaries to develop. TS may be associated with heart defects like bicuspid aortic valve (BAV), coarctation of the aorta (COA) and pulmonary arterial hypertension (PAH).[2] Such a combination of TS with cardiac and non-cardiac defects with a wide range of clinical presentations poses a challenge during perioperative management. TS with cleft palate can have a short trachea with a high location of the carina, risking endobronchial intubation and accidental extubation of the endotracheal tube. [3] The prevalence of cleft palate in patients with TS has not been described in the literature, as not many cases have been reported on the perioperative management of a patient with TS with cleft palate undergoing palatoplasty. We describe the perioperative anaesthetic management of 14 years-6 months, short statured female with TS undergoing bardach,s two-flap palatoplasty repair after obtaining informed consent from the parents.

Zenodo (CERN European Organization for Nuclear Research)
Innovative Research (United States) (US)
Good health and well-being
Openalex Percentile: Top 67%
Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities
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