Three Extremely Rare Findings in the Same Patient: Harlequin Syndrome, Thyrocervical Trunk Aneurysm, and Systemic-Pulmonary Arterio-Arterial Fistula

Góes, Adenauer Marinho de Oliveira; Junior, Adib Koury; Paschoal, Eric Homero Albuquerque; Jeha, Salim Abdon Haber

doi:10.1016/j.avsg.2017.06.146

Cited by 4 publications

(6 citation statements)

References 12 publications

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“…Harlequin syndrome, first described in 1988 by Lance [1], is a dysautonomic syndrome of the face due to a unilateral dysfunction of the sympathetic system. It is more common in women, and most often benign and idiopathic.…”

Section: Introductionmentioning

confidence: 99%

Idiopathic harlequin syndrome: a case report and literature review

Elboukhari¹,

Baybay²,

Elloudi³

et al. 2019

Pan Afr Med J

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Harlequin's syndrome is a rare dysautonomic syndrome of the face characterized by sweating with flush of one side and anhidrosis of the contralateral side. Mostly idiopathic although several secondary cases have been reported in the literature, the purpose of the treatment is mainly aesthetic and functional. We report the case of a patient having harlequin syndrome in its idiopathic form with a literature review.

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Section: Introductionmentioning

confidence: 99%

Idiopathic harlequin syndrome: a case report and literature review

Elboukhari¹,

Baybay²,

Elloudi³

et al. 2019

Pan Afr Med J

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“…The pathogenesis usually involves arterial intimal injury creating discontinuity in the arterial wall, thereby allowing blood into the false arterial lumen, giving rise to pseudoaneurysm formation [24][25][26]. Rupture of a pseudoaneurysm can result in formation of a hematoma and can cause hemodynamic decline or airway compromise and can potentially be fatal [27,28]. Abrokwah et al reported the case of a patient in which cannulation of the subclavian vein was attempted for cardiac pacing; however, the procedure was ultimately abandoned due to bleeding from the puncture site.…”

Section: Discussionmentioning

confidence: 99%

“…Although still suspected, the patient did not report any falls or other sources of trauma in that case [30]. Junior et al described a case of a patient with Harlequin syndrome who was found to have thyrocervical trunk pseudoaneurysm as well as a systemic-pulmonary arterio-arterial fistula [27]. Additionally, 2 cases of thyrocervical trunk and thyrocervical trunk branch pseudoaneurysms have been associated with clavicular fracture [25,31].…”

Section: Discussionmentioning

confidence: 99%

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Superficial Stab Wound to Zone I of the Neck Resulting in Thyrocervical Trunk Pseudoaneurysm Presented as Recurrent Hemothorax and Successfully Managed by Coil Embolization

et al. 2020

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Objective:Rare disease Background:Thyrocervical trunk pseudoaneurysms are rare complications that have been documented after internal jugular or subclavian venous cannulation. Even less common, these pseudoaneurysms can arise after blunt or penetrating trauma. Clinical hallmarks include an expanding supraclavicular mass with local compressive symptoms such as paresthesias, arterial steal syndrome, and Horner's syndrome. Patients may be asymptomatic, however, or present with overlying ecchymosis or the presence of a new bruit or thrill. With the risk of rupture, thyrocervical trunk pseudoaneurysm is associated with significant morbidity and mortality. Case Report:We report the case of a 27-year-old man who presented after sustaining a self-inflicted stab wound to zone I of his neck. Initial examination revealed only a superficial small laceration, but a chest x-ray revealed a pneumothorax, and tube thoracostomy returned 300 mL of bloody output. After resolution of the hemothorax and removal of the thoracostomy tube, the patient reaccumulated blood, requiring a repeat tube thoracostomy. Angiography at that time revealed a pseudoaneurysm of the thyrocervical trunk, and coil embolization was performed to obliterate the pseudoaneurysm. Conclusions:Thyrocervical trunk pseudoaneurysms can be asymptomatic, often have a delayed presentation, and can be life-threatening due to the risk of rupture and subsequent hemodynamic decline or airway compromise. While these pseudoaneurysms are well-known complications of deep penetrating injuries, they can also present following superficial penetrating injury to zone I of the neck. Selective angiography is the imaging modality of choice. Open surgical repair was traditionally the criterion standard for treatment; however, endovascular approaches are minimally invasive, feasible, and safer alternatives with reduced complications and are becoming more common.

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“…12 18 In this context, one case of HS associated with rare cardiovascular anomalies has been described. 19 It is tempting to speculate that both conditions, EA and HS, could be a result of disturbed neural crest migration. Due to the very limited number of reported cases of HS in children and in particular in association with EA, this conclusion is only hypothetical and further research addressing the pathogenesis of HS is needed.…”

Section: Discussionmentioning

confidence: 99%

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

Wagner

Lacher

Merkenschlager

et al. 2019

European J Pediatr Surg Rep

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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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Three Extremely Rare Findings in the Same Patient: Harlequin Syndrome, Thyrocervical Trunk Aneurysm, and Systemic-Pulmonary Arterio-Arterial Fistula

Cited by 4 publications

References 12 publications

Idiopathic harlequin syndrome: a case report and literature review

Idiopathic harlequin syndrome: a case report and literature review

Superficial Stab Wound to Zone I of the Neck Resulting in Thyrocervical Trunk Pseudoaneurysm Presented as Recurrent Hemothorax and Successfully Managed by Coil Embolization

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

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