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Syndrome congénital d´obstruction des voies aériennes supérieures (syndrome du CHAOS): à propos d’un cas

Congenital upper airway obstruction syndrome is a rare malformation that can be fatal to a newborn baby. It is defined as a complete or almost complete obstruction of the upper airways. CHAOS rate is unknown. We here report the case of a 24-year-old primiparous patient with no medical or surgical hi...

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Autores principales: Moussaoui, Kamal El, Slaoui, Aziz, Baidada, Aziz, Kharabch, Aicha
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The African Field Epidemiology Network 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7825376/
https://www.ncbi.nlm.nih.gov/pubmed/33520070
http://dx.doi.org/10.11604/pamj.2021.38.1.27283
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author Moussaoui, Kamal El
Slaoui, Aziz
Baidada, Aziz
Kharabch, Aicha
author_facet Moussaoui, Kamal El
Slaoui, Aziz
Baidada, Aziz
Kharabch, Aicha
author_sort Moussaoui, Kamal El
collection PubMed
description Congenital upper airway obstruction syndrome is a rare malformation that can be fatal to a newborn baby. It is defined as a complete or almost complete obstruction of the upper airways. CHAOS rate is unknown. We here report the case of a 24-year-old primiparous patient with no medical or surgical history. First-trimester ultrasound didn’t show any abnormality. During the second trimester, ultrasonography showed extensive subcutaneous edema, hypertrophied lung with hyperechoic appearance, hypoplastic heart and large-volume ascites associated with severe oligoamnios. After having excluded the most common causes, including isoimmunization (indirect negative Coombs), infections (negative serologies) kariotiping was not performed because the patient refused it. The results of ultrasonography suggested fetal hydrops secondary to CHAOS syndrome, because of pathognomonic signs of pulmonary hypertrophy with inverted or convex diaphragm. MRI was requested; it suggested CHAOS syndrome associated with other malformations: laryngeal atresia, microphthalmia with hypertelorism and deviation of the nasal septum with lack of visualization of the thymic tissue, no clear identification of the bladder, absence of right kidney and hypoplastic left kidney. The outcome of pregnancy was marked by premature labour at the 24(th) week of amenorrhea. The baby had a birth weight of 1475g, polimalformations such as sexual ambiguity, distended abdomen and polydactyly . The newborn died 3 min after birth. Karyotyping was performed which showed 46XX.
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spelling pubmed-78253762021-01-29 Syndrome congénital d´obstruction des voies aériennes supérieures (syndrome du CHAOS): à propos d’un cas Moussaoui, Kamal El Slaoui, Aziz Baidada, Aziz Kharabch, Aicha Pan Afr Med J Case Report Congenital upper airway obstruction syndrome is a rare malformation that can be fatal to a newborn baby. It is defined as a complete or almost complete obstruction of the upper airways. CHAOS rate is unknown. We here report the case of a 24-year-old primiparous patient with no medical or surgical history. First-trimester ultrasound didn’t show any abnormality. During the second trimester, ultrasonography showed extensive subcutaneous edema, hypertrophied lung with hyperechoic appearance, hypoplastic heart and large-volume ascites associated with severe oligoamnios. After having excluded the most common causes, including isoimmunization (indirect negative Coombs), infections (negative serologies) kariotiping was not performed because the patient refused it. The results of ultrasonography suggested fetal hydrops secondary to CHAOS syndrome, because of pathognomonic signs of pulmonary hypertrophy with inverted or convex diaphragm. MRI was requested; it suggested CHAOS syndrome associated with other malformations: laryngeal atresia, microphthalmia with hypertelorism and deviation of the nasal septum with lack of visualization of the thymic tissue, no clear identification of the bladder, absence of right kidney and hypoplastic left kidney. The outcome of pregnancy was marked by premature labour at the 24(th) week of amenorrhea. The baby had a birth weight of 1475g, polimalformations such as sexual ambiguity, distended abdomen and polydactyly . The newborn died 3 min after birth. Karyotyping was performed which showed 46XX. The African Field Epidemiology Network 2021-01-01 /pmc/articles/PMC7825376/ /pubmed/33520070 http://dx.doi.org/10.11604/pamj.2021.38.1.27283 Text en Copyright: Kamal El Moussaoui et al. https://creativecommons.org/licenses/by/4.0 The Pan African Medical Journal (ISSN: 1937-8688). This is an Open Access article distributed under the terms of the Creative Commons Attribution International 4.0 License (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
Moussaoui, Kamal El
Slaoui, Aziz
Baidada, Aziz
Kharabch, Aicha
Syndrome congénital d´obstruction des voies aériennes supérieures (syndrome du CHAOS): à propos d’un cas
title Syndrome congénital d´obstruction des voies aériennes supérieures (syndrome du CHAOS): à propos d’un cas
title_full Syndrome congénital d´obstruction des voies aériennes supérieures (syndrome du CHAOS): à propos d’un cas
title_fullStr Syndrome congénital d´obstruction des voies aériennes supérieures (syndrome du CHAOS): à propos d’un cas
title_full_unstemmed Syndrome congénital d´obstruction des voies aériennes supérieures (syndrome du CHAOS): à propos d’un cas
title_short Syndrome congénital d´obstruction des voies aériennes supérieures (syndrome du CHAOS): à propos d’un cas
title_sort syndrome congénital d´obstruction des voies aériennes supérieures (syndrome du chaos): à propos d’un cas
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7825376/
https://www.ncbi.nlm.nih.gov/pubmed/33520070
http://dx.doi.org/10.11604/pamj.2021.38.1.27283
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