Laparoscopic gastrostomy in a patient with asphyxiating thoracic dystrophy

Introduction: Asphyxiating thoracic dystrophy (ATD), also known as Jeune syndrome, is an autosomal recessive disease which causes thoracic insufficiency. Complications and life expectancy vary based on specific mutations. Infants with this condition may require operative feeding access in addition t...

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Autores principales:	Ediana Hoxhallari, Elliot C. Pennington
Formato:	article
Lenguaje:	EN
Publicado:	Elsevier 2022
Materias:	Asphyxiating thoracic dystrophy Jeune syndrome Laparoscopic pediatric surgery Gastrostomy Pediatrics RJ1-570 Surgery RD1-811
Acceso en línea:	https://doaj.org/article/b7e610745eb44f4a8a7d63add93e416f
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spelling	oai:doaj.org-article:b7e610745eb44f4a8a7d63add93e416f2021-11-12T04:33:24ZLaparoscopic gastrostomy in a patient with asphyxiating thoracic dystrophy2213-576610.1016/j.epsc.2021.102117https://doaj.org/article/b7e610745eb44f4a8a7d63add93e416f2022-01-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S2213576621003389https://doaj.org/toc/2213-5766Introduction: Asphyxiating thoracic dystrophy (ATD), also known as Jeune syndrome, is an autosomal recessive disease which causes thoracic insufficiency. Complications and life expectancy vary based on specific mutations. Infants with this condition may require operative feeding access in addition to surgery for other associated anomalies. No prior cases of laparoscopic surgery in ATD have been recorded in the literature. This case demonstrates that with proper technique, laparoscopic surgery can be accomplished safely. Case presentation: Newborn diagnosed with ATD prenatally with anatomical 20-week ultrasound who required long term enteral feeding access due to inability to tolerate oral feeds secondary to tachypnea. Management and outcome: Patient underwent laparoscopic gastrostomy tube placement at 2 weeks of age. This was well tolerated, and no complications arose from insufflation pressure despite small thoracic volumes. Pressure control ventilation was used along with frequent desufflation breaks. Patient tolerated tube feeds at goal until expiration at 8 weeks of age. Discussion: Laparoscopic surgery can be safely performed in patients with ATD with appropriate monitoring and ventilatory support. Although ATD is commonly fatal, this type of palliative surgery can improve patients’ quality of life and allow them to discharge home safely so they can spend more time with family.Ediana HoxhallariElliot C. PenningtonElsevierarticleAsphyxiating thoracic dystrophyJeune syndromeLaparoscopic pediatric surgeryGastrostomyPediatricsRJ1-570SurgeryRD1-811ENJournal of Pediatric Surgery Case Reports, Vol 76, Iss , Pp 102117- (2022)
institution	DOAJ
collection	DOAJ
language	EN
topic	Asphyxiating thoracic dystrophy Jeune syndrome Laparoscopic pediatric surgery Gastrostomy Pediatrics RJ1-570 Surgery RD1-811
spellingShingle	Asphyxiating thoracic dystrophy Jeune syndrome Laparoscopic pediatric surgery Gastrostomy Pediatrics RJ1-570 Surgery RD1-811 Ediana Hoxhallari Elliot C. Pennington Laparoscopic gastrostomy in a patient with asphyxiating thoracic dystrophy
description	Introduction: Asphyxiating thoracic dystrophy (ATD), also known as Jeune syndrome, is an autosomal recessive disease which causes thoracic insufficiency. Complications and life expectancy vary based on specific mutations. Infants with this condition may require operative feeding access in addition to surgery for other associated anomalies. No prior cases of laparoscopic surgery in ATD have been recorded in the literature. This case demonstrates that with proper technique, laparoscopic surgery can be accomplished safely. Case presentation: Newborn diagnosed with ATD prenatally with anatomical 20-week ultrasound who required long term enteral feeding access due to inability to tolerate oral feeds secondary to tachypnea. Management and outcome: Patient underwent laparoscopic gastrostomy tube placement at 2 weeks of age. This was well tolerated, and no complications arose from insufflation pressure despite small thoracic volumes. Pressure control ventilation was used along with frequent desufflation breaks. Patient tolerated tube feeds at goal until expiration at 8 weeks of age. Discussion: Laparoscopic surgery can be safely performed in patients with ATD with appropriate monitoring and ventilatory support. Although ATD is commonly fatal, this type of palliative surgery can improve patients’ quality of life and allow them to discharge home safely so they can spend more time with family.
format	article
author	Ediana Hoxhallari Elliot C. Pennington
author_facet	Ediana Hoxhallari Elliot C. Pennington
author_sort	Ediana Hoxhallari
title	Laparoscopic gastrostomy in a patient with asphyxiating thoracic dystrophy
title_short	Laparoscopic gastrostomy in a patient with asphyxiating thoracic dystrophy
title_full	Laparoscopic gastrostomy in a patient with asphyxiating thoracic dystrophy
title_fullStr	Laparoscopic gastrostomy in a patient with asphyxiating thoracic dystrophy
title_full_unstemmed	Laparoscopic gastrostomy in a patient with asphyxiating thoracic dystrophy
title_sort	laparoscopic gastrostomy in a patient with asphyxiating thoracic dystrophy
publisher	Elsevier
publishDate	2022
url	https://doaj.org/article/b7e610745eb44f4a8a7d63add93e416f
work_keys_str_mv	AT edianahoxhallari laparoscopicgastrostomyinapatientwithasphyxiatingthoracicdystrophy AT elliotcpennington laparoscopicgastrostomyinapatientwithasphyxiatingthoracicdystrophy
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Laparoscopic gastrostomy in a patient with asphyxiating thoracic dystrophy

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