Harlequin Syndrome after Thoracoscopic Repair of a Child with Tracheoesophageal Fistula (TEF)

Harlequin syndrome (HS) is a rare dysautonomia of the sympathetic nervous system leading to asymmetric facial flushing and sweating. In the literature, only a few cases of HS after thoracoscopic tracheoesophageal fistula (TEF) repair are reported. We report on a newborn with TEF who developed HS aft...

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Main Authors: Richard Wagner, Martin Lacher, Andreas Merkenschlager, Moritz Markel
Format: Article
Language:English
Published: Georg Thieme Verlag KG 2019-01-01
Series:European Journal of Pediatric Surgery Reports
Subjects:
Online Access:http://www.thieme-connect.de/DOI/DOI?10.1055/s-0039-1697667
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author Richard Wagner
Martin Lacher
Andreas Merkenschlager
Moritz Markel
author_facet Richard Wagner
Martin Lacher
Andreas Merkenschlager
Moritz Markel
author_sort Richard Wagner
collection DOAJ
description Harlequin syndrome (HS) is a rare dysautonomia of the sympathetic nervous system leading to asymmetric facial flushing and sweating. In the literature, only a few cases of HS after thoracoscopic tracheoesophageal fistula (TEF) repair are reported. We report on a newborn with TEF who developed HS after thoracoscopic repair. On the first day of life, the girl (3,480 g, gestation age: 41 week) underwent thoracoscopic repair of a type C esophageal atresia (TEF; OR time 105 minute) without complications. The postoperative course was uneventful, the patient swallowed and thrived well and did not require esophageal dilatations. At 2 years of age, missing facial flushing, transpiration, and warming on the right side of her face during agitation were noticed. As no further intervention was required, the girl and her parents adapted well to the symptoms. Our report shows that the late onset of HS after the surgical procedure is unlikely a direct causal relation to the thoracoscopic operation but rather a shared embryological pathogenesis, like a neurocristopathy.
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spelling doaj.art-4cfe4970bcfd4dcb80644e23cf6753672022-12-22T00:04:18ZengGeorg Thieme Verlag KGEuropean Journal of Pediatric Surgery Reports2194-76192194-76272019-01-010701e63e6510.1055/s-0039-1697667Harlequin Syndrome after Thoracoscopic Repair of a Child with Tracheoesophageal Fistula (TEF)Richard Wagner0Martin Lacher1Andreas Merkenschlager2Moritz Markel3Klinik und Poliklinik für Kinderchirurgie, Universitätsklinikum Leipzig, Leipzig, Sachsen, GermanyKlinik und Poliklinik für Kinderchirurgie, Universitätsklinikum Leipzig, Leipzig, Sachsen, GermanyKlinik für Neuropädiatrie, Universitätsklinikum Leipzig Klinik und Poliklinik für Kinder- und Jugendmedizin, Leipzig, Sachsen, GermanyKlinik und Poliklinik für Kinderchirurgie, Universitätsklinikum Leipzig, Leipzig, Sachsen, GermanyHarlequin syndrome (HS) is a rare dysautonomia of the sympathetic nervous system leading to asymmetric facial flushing and sweating. In the literature, only a few cases of HS after thoracoscopic tracheoesophageal fistula (TEF) repair are reported. We report on a newborn with TEF who developed HS after thoracoscopic repair. On the first day of life, the girl (3,480 g, gestation age: 41 week) underwent thoracoscopic repair of a type C esophageal atresia (TEF; OR time 105 minute) without complications. The postoperative course was uneventful, the patient swallowed and thrived well and did not require esophageal dilatations. At 2 years of age, missing facial flushing, transpiration, and warming on the right side of her face during agitation were noticed. As no further intervention was required, the girl and her parents adapted well to the symptoms. Our report shows that the late onset of HS after the surgical procedure is unlikely a direct causal relation to the thoracoscopic operation but rather a shared embryological pathogenesis, like a neurocristopathy.http://www.thieme-connect.de/DOI/DOI?10.1055/s-0039-1697667harlequin syndromeesophageal atresiathoracoscopic repairneurocristopathy
spellingShingle Richard Wagner
Martin Lacher
Andreas Merkenschlager
Moritz Markel
Harlequin Syndrome after Thoracoscopic Repair of a Child with Tracheoesophageal Fistula (TEF)
European Journal of Pediatric Surgery Reports
harlequin syndrome
esophageal atresia
thoracoscopic repair
neurocristopathy
title Harlequin Syndrome after Thoracoscopic Repair of a Child with Tracheoesophageal Fistula (TEF)
title_full Harlequin Syndrome after Thoracoscopic Repair of a Child with Tracheoesophageal Fistula (TEF)
title_fullStr Harlequin Syndrome after Thoracoscopic Repair of a Child with Tracheoesophageal Fistula (TEF)
title_full_unstemmed Harlequin Syndrome after Thoracoscopic Repair of a Child with Tracheoesophageal Fistula (TEF)
title_short Harlequin Syndrome after Thoracoscopic Repair of a Child with Tracheoesophageal Fistula (TEF)
title_sort harlequin syndrome after thoracoscopic repair of a child with tracheoesophageal fistula tef
topic harlequin syndrome
esophageal atresia
thoracoscopic repair
neurocristopathy
url http://www.thieme-connect.de/DOI/DOI?10.1055/s-0039-1697667
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AT andreasmerkenschlager harlequinsyndromeafterthoracoscopicrepairofachildwithtracheoesophagealfistulatef
AT moritzmarkel harlequinsyndromeafterthoracoscopicrepairofachildwithtracheoesophagealfistulatef