Splenic torsion in the setting of polysplenia syndrome

Background: Polysplenia syndrome is a rare condition that occurs when polysplenia co-exists with various other congenital anomalies. Splenic torsion in the setting of polysplenia syndrome is extremely rare, having been documented only five times previously in the English literature. Case report: We...

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Autores principales:	Margaret Kennedy, Daniel Farinas Lugo, William Raleigh Thompson
Formato:	article
Lenguaje:	EN
Publicado:	Elsevier 2021
Materias:	Splenic torsion Polysplenia syndrome Malrotation Pediatrics RJ1-570 Surgery RD1-811
Acceso en línea:	https://doaj.org/article/50b0301b6ca049178f4c2f81690dae3f
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spelling	oai:doaj.org-article:50b0301b6ca049178f4c2f81690dae3f2021-11-24T04:29:34ZSplenic torsion in the setting of polysplenia syndrome2213-576610.1016/j.epsc.2021.102070https://doaj.org/article/50b0301b6ca049178f4c2f81690dae3f2021-12-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S2213576621002918https://doaj.org/toc/2213-5766Background: Polysplenia syndrome is a rare condition that occurs when polysplenia co-exists with various other congenital anomalies. Splenic torsion in the setting of polysplenia syndrome is extremely rare, having been documented only five times previously in the English literature. Case report: We present a case of a 9 year-old female who underwent laparoscopic splenectomy for ischemic polysplenia secondary to splenic torsion in the setting of polysplenia syndrome. This previously healthy patient presented to the emergency department with a 3-day history of abdominal pain and emesis. She was found to have leukocytosis and computed tomography (CT) revealed evidence of polysplenia with torsion of multiple splenules, as well as an annular pancreas, intestinal malrotation, and an interrupted inferior vena cava (IVC) with azygous continuation. Conclusion: To our knowledge, this was the first documented case of splenic torsion in the setting of polysplenia syndrome with concurrent anomalies of intestinal malrotation, interruption of the IVC with azygous continuation, and preduodenal portal vein (PDPV). The goal of our case report is to describe this rare phenomenon while detailing the diagnostic and management considerations. Mesh terms: Splenic Torsion, Polysplenia Syndrome, Polysplenia, Malrotation.Margaret KennedyDaniel Farinas LugoWilliam Raleigh ThompsonElsevierarticleSplenic torsionPolysplenia syndromeMalrotationPediatricsRJ1-570SurgeryRD1-811ENJournal of Pediatric Surgery Case Reports, Vol 75, Iss , Pp 102070- (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Splenic torsion Polysplenia syndrome Malrotation Pediatrics RJ1-570 Surgery RD1-811
spellingShingle	Splenic torsion Polysplenia syndrome Malrotation Pediatrics RJ1-570 Surgery RD1-811 Margaret Kennedy Daniel Farinas Lugo William Raleigh Thompson Splenic torsion in the setting of polysplenia syndrome
description	Background: Polysplenia syndrome is a rare condition that occurs when polysplenia co-exists with various other congenital anomalies. Splenic torsion in the setting of polysplenia syndrome is extremely rare, having been documented only five times previously in the English literature. Case report: We present a case of a 9 year-old female who underwent laparoscopic splenectomy for ischemic polysplenia secondary to splenic torsion in the setting of polysplenia syndrome. This previously healthy patient presented to the emergency department with a 3-day history of abdominal pain and emesis. She was found to have leukocytosis and computed tomography (CT) revealed evidence of polysplenia with torsion of multiple splenules, as well as an annular pancreas, intestinal malrotation, and an interrupted inferior vena cava (IVC) with azygous continuation. Conclusion: To our knowledge, this was the first documented case of splenic torsion in the setting of polysplenia syndrome with concurrent anomalies of intestinal malrotation, interruption of the IVC with azygous continuation, and preduodenal portal vein (PDPV). The goal of our case report is to describe this rare phenomenon while detailing the diagnostic and management considerations. Mesh terms: Splenic Torsion, Polysplenia Syndrome, Polysplenia, Malrotation.
format	article
author	Margaret Kennedy Daniel Farinas Lugo William Raleigh Thompson
author_facet	Margaret Kennedy Daniel Farinas Lugo William Raleigh Thompson
author_sort	Margaret Kennedy
title	Splenic torsion in the setting of polysplenia syndrome
title_short	Splenic torsion in the setting of polysplenia syndrome
title_full	Splenic torsion in the setting of polysplenia syndrome
title_fullStr	Splenic torsion in the setting of polysplenia syndrome
title_full_unstemmed	Splenic torsion in the setting of polysplenia syndrome
title_sort	splenic torsion in the setting of polysplenia syndrome
publisher	Elsevier
publishDate	2021
url	https://doaj.org/article/50b0301b6ca049178f4c2f81690dae3f
work_keys_str_mv	AT margaretkennedy splenictorsioninthesettingofpolyspleniasyndrome AT danielfarinaslugo splenictorsioninthesettingofpolyspleniasyndrome AT williamraleighthompson splenictorsioninthesettingofpolyspleniasyndrome
_version_	1718415974153060352

Splenic torsion in the setting of polysplenia syndrome

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