Harlequin syndrome in a young patient with osteosarcoma after pleural metastasectomy: A case report and a mini review of the literature

Moutafi,Myrto; Kyriazoglou,Anastasios; Kotsantis,Ioannis; Economopoulou,Panagiota; Efthymiou,Evgenia; Kyrkasiadou,Maria; Boulouta,Anna; Gavrielatou,Niki; Zazas,Evangelos; Markakis,Konstantinos; Psyrri,Amanda

doi:10.3892/etm.2025.12812

Harlequin syndrome in a young patient with osteosarcoma after pleural metastasectomy: A case report and a mini review of the literature

Authors:
- Myrto Moutafi
- Anastasios Kyriazoglou
- Ioannis Kotsantis
- Panagiota Economopoulou
- Evgenia Efthymiou
- Maria Kyrkasiadou
- Anna Boulouta
- Niki Gavrielatou
- Evangelos Zazas
- Konstantinos Markakis
- Amanda Psyrri
View Affiliations

Affiliations: Section of Medical Oncology, Second Department of Internal Medicine, School of Medicine, National and Kapodistrian University of Athens, Attikon University Hospital, Athens 12462, Greece, Second Department of Radiology, Attikon University Hospital, Athens 12462, Greece, Section of Internal Medicine, Second Department of Internal Medicine, School of Medicine, National and Kapodistrian University of Athens, Attikon University Hospital, Athens 12462, Greece
Published online on: January 30, 2025 https://doi.org/10.3892/etm.2025.12812
Article Number: 62

Metrics: Total Views: 0 (Spandidos Publications: | PMC Statistics: )

Metrics: Total PDF Downloads: 0 (Spandidos Publications: | PMC Statistics: )

Cited By (CrossRef): 0 citations Loading Articles...

Abstract

Harlequin syndrome is a rare autonomic disorder with at least 83 reported cases in literature, 6% of which are congenital. When the fibers responsible for sudomotor and vasomotor supply to the face at the T2‑T3 level are unilaterally blocked, it leads to hemifacial discoloration. This results in one‑half of the face appearing flushed and hyperemic, sharply contrasting with the pale appearance of the other half. The cause of this syndrome is unknown; however, it appears to involve an autonomic nervous system dysfunction. It can be caused by an injury, compression, or blockade of sympathetic fibers along the pathway. The present study was a case of a metastatic osteosarcoma patient with a history of video‑assisted thoracic surgery pleurectomy that presented in Attikon University Hospital (Athens, Greece) with worsening dyspnea and right‑sided facial flushing.

View Figures

View References

1	Cheshire WP Jr and Low PA: Harlequin syndrome: Still only half understood. J Neuroophthalmol. 28:169–170. 2008.PubMed/NCBI View Article : Google Scholar
2	Bremner F and Smith S: Pupillographic findings in 39 consecutive cases of harlequin syndrome. J Neuroophthalmol. 28:171–177. 2008.PubMed/NCBI View Article : Google Scholar
3	Lance JW, Drummond PD, Gandevia SC and Morris JG: Harlequin syndrome: The sudden onset of unilateral flushing and sweating. J Neurol Neurosurg Psychiatry. 51:635–642. 1988.PubMed/NCBI View Article : Google Scholar
4	Tascilar N, Tekin NS, Erdem Z, Alpay A and Emre U: Unnoticed dysautonomic syndrome of the face: Harlequin syndrome. Auton Neurosci. 137:1–9. 2007.PubMed/NCBI View Article : Google Scholar
5	Drummond PD and Finch PM: Reflex control of facial flushing during body heating in man. Brain. 112 (Pt 5):1351–1358. 1989.PubMed/NCBI View Article : Google Scholar
6	Drummond PD and Edis RH: Loss of facial sweating and flushing in Holmes-Adie syndrome. Neurology. 40:847–849. 1990.PubMed/NCBI View Article : Google Scholar
7	Yu D, Zhang S, Feng A, Xu D, Zhu Q, Mao Y, Zhao Y, Lv Y, Han C, Liu R and Tian Y: Methotrexate, doxorubicin, and cisplatinum regimen is still the preferred option for osteosarcoma chemotherapy: A meta-analysis and clinical observation. Medicine (Baltimore). 98(e15582)2019.PubMed/NCBI View Article : Google Scholar
8	Sanfilippo R, Bertulli R, Marrari A, Fumagalli E, Pilotti S, Morosi C, Messina A, Dei Tos AP, Gronchi A and Casali PG: High-dose continuous-infusion ifosfamide in advanced well-differentiated/dedifferentiated liposarcoma. Clin Sarcoma Res. 4(16)2014.PubMed/NCBI View Article : Google Scholar
9	Micheau A HD. e-Anatomy.
10	Vidal Esteban A, Natera-de Benito D, Martínez Sánchez D, Reche Sainz A, Rodríguez Díaz MR, Alfaro Iznaola CM and de Santos Moreno MT: Congenital Harlequin syndrome as an isolated phenomenon: A case report and review of the literature. Eur J Paediatr Neurol. 20:426–430. 2016.PubMed/NCBI View Article : Google Scholar
11	van Brakel TJ and Barendregt WB: Wet and dry hands after video-assisted thoracoscopic pleurectomy. Interact Cardiovasc Thorac Surg. 16:563–564. 2013.PubMed/NCBI View Article : Google Scholar
12	Wagner R, Lacher M, Merkenschlager A and Markel M: Harlequin syndrome after thoracoscopic repair of a child with tracheoesophageal fistula (TEF). European J Pediatr Surg Rep. 7:e63–e65. 2019.PubMed/NCBI View Article : Google Scholar
13	Sugimura H, Spratt EH, Compeau CG, Kattail D and Shargall Y: Thoracoscopic sympathetic clipping for hyperhidrosis: long-term results and reversibility. J Thorac Cardiovasc Surg. 137:1370–6; discussion 1376-7. 2009.PubMed/NCBI View Article : Google Scholar
14	Mavroudis I, Balmus IM, Ciobica A, Luca AC, Chowdhury R, Iordache AC, Gorgan DL and Radu I: Mini-review on the harlequin syndrome-a rare dysautonomic manifestation requiring attention. Medicina (Kaunas). 58(938)2022.PubMed/NCBI View Article : Google Scholar
15	Willaert WI, Scheltinga MR, Steenhuisen SF and Hiel JA: Harlequin syndrome: Two new cases and a management proposal. Acta Neurol Belg. 109:214–220. 2009.PubMed/NCBI
16	Fringeli Y, Humm AM, Ansorge A and Maestretti G: Harlequin sign concomitant with Horner syndrome after anterior cervical discectomy: A case of intrusion into the cervical sympathetic system. J Neurosurg Spine. 26:684–687. 2017.PubMed/NCBI View Article : Google Scholar
17	Sullivan TR, Kanda P, Gagne S and Costache I: Harlequin Syndrome associated with erector spinae plane block. Anesthesiology. 131(665)2019.PubMed/NCBI View Article : Google Scholar
18	Burlacu CL and Buggy DJ: Coexisting harlequin and Horner syndromes after high thoracic paravertebral anaesthesia. Br J Anaesth. 95:822–824. 2005.PubMed/NCBI View Article : Google Scholar
19	Biondi A, Persiani R, Zoccali M, Rausei S, Cananzi F and D'Ugo D: Harlequin syndrome. Ann Thorac Surg. 88(304)2009.PubMed/NCBI View Article : Google Scholar
20	Tubbs RS, Salter G, Wellons JC III and Oakes WJ: Blood supply of the human cervical sympathetic chain and ganglia. Eur J Morphol. 40:283–288. 2002.PubMed/NCBI View Article : Google Scholar
21	Van Slycke S, Stockman A, Dionigi G, Carette R, Gillardin JP, Brusselaers N and Vermeersch H: Harlequin syndrome after thyroidectomy for compressive retrosternal goiter. case report and review of the literature. Acta Chir Belg. 114:212–214. 2014.PubMed/NCBI View Article : Google Scholar
22	Reddy H, Fatah S, Gulve A and Carmichael AJ: Novel management of harlequin syndrome with stellate ganglion block. Br J Dermatol. 169:954–956. 2013.PubMed/NCBI View Article : Google Scholar
23	Manhães RK, Spitz M and Vasconcellos LF: Botulinum toxin for treatment of Harlequin syndrome. Parkinsonism Relat Disord. 23:112–113. 2016.PubMed/NCBI View Article : Google Scholar
24	Kirk F, Crathern K and Stroebel A: Video-assisted thoracoscopic sympathectomy for Harlequin syndrome. Eur J Cardiothorac Surg. 63(ezac577)2023.PubMed/NCBI View Article : Google Scholar
25	Sharma B, Sharma P, Ahuja I and Pemawat A: Harlequin syndrome and autonomic seizures-A rare association. Ann Indian Acad Neurol. 26:839–841. 2023.PubMed/NCBI View Article : Google Scholar
26	Navickaitė I, Ališauskienė M, Petrauskienė S and Žemgulytė G: Sarcoidosis-associated sensory ganglionopathy and harlequin syndrome: A case report. Medicina (Kaunas). 59(1495)2023.PubMed/NCBI View Article : Google Scholar
27	Karam C, Abou Nafeh N, Aouad MT, Siddik-Sayyid S, Kaddoum R, Zeeni C, Anka S, Shaya B and Khalili A: Harlequin syndrome during peripheral cardiopulmonary bypass in a patient with an obstructing tracheal schwannoma: A case report. Clin Case Rep. 11(e7509)2023.PubMed/NCBI View Article : Google Scholar
28	Melka D and Zebenigus M: Idiopathic harlequin syndrome in a patient from Ethiopia: A case report. Ethiop J Health Sci. 33:383–386. 2023.PubMed/NCBI View Article : Google Scholar
29	Giunta M, Recchia EG, Capuano P, Toscano A, Attisani M, Rinaldi M and Brazzi L: Management of harlequin syndrome under ECPELLA support: A report of two cases and a proposed approach. Ann Card Anaesth. 26:97–101. 2023.PubMed/NCBI View Article : Google Scholar
30	Hylton JRE: Harlequin syndrome as a complication of epidural anaesthesia in an infant: Do adjunct medications play a role? Indian J Anaesth. 66:669–672. 2022.PubMed/NCBI View Article : Google Scholar
31	Dalldorf DA, Hart A, Grant SA and Teeter EG: Harlequin syndrome following regional liposomal bupivacaine use in a partial sternectomy. Cureus. 14(e28005)2022.PubMed/NCBI View Article : Google Scholar
32	Yan R, Dang C and Yuan W: Harlequin syndrome induced by intraspinal analgesia in patients with advanced cancer: A case report. Transl Cancer Res. 11:2457–2461. 2022.PubMed/NCBI View Article : Google Scholar
33	Korbi M, Boumaiza S, Achour A, Belhadjali H and Zili J: Harlequin syndrome: An asymmetric face. Clin Case Rep. 10(e05833)2022.PubMed/NCBI View Article : Google Scholar
34	Persson RM, Tellnes K, Hoven H, Haaverstad R and Svendsen ØS: Harlequin syndrome associated with thoracic epidural anaesthesia. Anaesth Rep. 10(10.1002/anr3.12144)2022.PubMed/NCBI View Article : Google Scholar
35	Schultz L, Mackarey A, Oh C and Kent P: Harlequin syndrome following microwave ablation in a child with a symptomatic paraspinal mass. BMJ Case Rep. 13(e232700)2020.PubMed/NCBI View Article : Google Scholar
36	Tanaka Y and Satomi K: Cardiac sympathetic hyperactivity of lung cancer-associated harlequin syndrome. JMA J. 2:190–191. 2019.PubMed/NCBI View Article : Google Scholar
37	Elboukhari K, Baybay H, Elloudi S, Douhi Z and Mernissi FZ: Idiopathic harlequin syndrome: A case report and literature review. Pan Afr Med J. 33(141)2019.PubMed/NCBI View Article : Google Scholar
38	Pasrija C, Bedeir K, Jeudy J and Kon ZN: Harlequin syndrome during venoarterial extracorporeal membrane oxygenation. Radiol Cardiothorac Imaging. 1(e190031)2019.PubMed/NCBI View Article : Google Scholar
39	Hans-Bittner NR, Bittner GC and Hans Filho G: Do you know this syndrome? Harlequin syndrome. An Bras Dermatol. 93:585–586. 2018.PubMed/NCBI View Article : Google Scholar
40	Lee DH, Seong JY, Yoon TM, Lee JK and Lim SC: Harlequin syndrome and Horner syndrome after neck schwannoma excision in a pediatric patient: A case report. Medicine (Baltimore). 96(e8548)2017.PubMed/NCBI View Article : Google Scholar
41	Lefevre A and Schnepper G: Development of harlequin syndrome following placement of thoracic epidural anesthesia in a pediatric patient undergoing Nuss procedure. Clin Case Rep. 5:1523–1525. 2017.PubMed/NCBI View Article : Google Scholar
42	Jeon YJ, Son J and Cho JH: Harlequin syndrome following resection of mediastinal ganglioneuroma. Korean J Thorac Cardiovasc Surg. 50:130–132. 2017.PubMed/NCBI View Article : Google Scholar
43	Algahtani H, Shirah B, Algahtani R and Alkahtani A: Idiopathic harlequin syndrome manifesting during exercise: A case report and review of the literature. Case Rep Med. 2017(5342593)2017.PubMed/NCBI View Article : Google Scholar
44	Al Hanshi SAM and Al Othmani F: A case study of Harlequin syndrome in VA-ECMO. Qatar Med J. 2017(39)2017.
45	Kim JY, Lee MS, Kim SY, Kim HJ, Lee SJ, You CW, Kim JS and Kang JH: A pediatric case of idiopathic Harlequin syndrome. Korean J Pediatr. 59 (Suppl 1):S125–S128. 2016.PubMed/NCBI View Article : Google Scholar
46	Naqvi SY, Zainal A, Flood SP and Kinniry P: Harlequin syndrome with contralateral anhidrosis after an upper chest gunshot wound. BMJ Case Rep. 2016(bcr2016216931)2016.PubMed/NCBI View Article : Google Scholar
47	Jung JM, Lee MH, Won CH, Chang SE, Lee MW, Choi JH and Moon KC: Iatrogenic harlequin syndrome: A new case. Ann Dermatol. 27:101–102. 2015.PubMed/NCBI View Article : Google Scholar
48	Emsley HC: Postexertional harlequin syndrome with spontaneous improvement. BMJ Case Rep. 2013(bcr2013200516)2013.PubMed/NCBI View Article : Google Scholar
49	Breunig Jde A, Hartmann M, Freire CF and de Almeida HL Jr: Harlequin syndrome in childhood-case report. An Bras Dermatol. 87:907–909. 2012.PubMed/NCBI View Article : Google Scholar
50	Pradeep PV, Benede AK, Harshita SS and Jayashree B: Harlequin syndrome in a case of toxic goitre: A rare association. Case Rep Med. 2011(293076)2011.PubMed/NCBI View Article : Google Scholar
51	Moon SY, Shin DI, Park SH and Kim JS: Harlequin syndrome with crossed sympathetic deficit of the face and arm. J Korean Med Sci. 20:329–330. 2005.PubMed/NCBI View Article : Google Scholar
52	Fallon KE and May JJ: Harlequin syndrome in two athletes. Br J Sports Med. 39(e1)2005.PubMed/NCBI View Article : Google Scholar
53	Lombardi C, Vetrugno R, Provini F, Plazzi G, Pierangeli G, Coccagna G, Lugaresi E, Montagna P and Cortelli P: Harlequin syndrome: An association with overlap parasomnia. J Neurol Neurosurg Psychiatry. 75:341–342. 2004.PubMed/NCBI
54	Swan MC, Nicolaou M and Paes TR: Iatrogenic harlequin syndrome. Postgrad Med J. 79(278)2003.PubMed/NCBI View Article : Google Scholar
55	Corbett M and Abernethy DA: Harlequin syndrome. J Neurol Neurosurg Psychiatry. 66(544)1999.PubMed/NCBI View Article : Google Scholar
56	Noda S: Harlequin syndrome due to superior mediastinal neurinoma. J Neurol Neurosurg Psychiatry. 54(744)1991.PubMed/NCBI View Article : Google Scholar