Coexisting Harlequin and Horner's syndromes
*E-mail: anjalina{at}mac.comEditor—We would like to correspond further with regard to the article entitled ‘Coexisting Harlequin and Horner's syndromes after high thoracic paravertebral anaesthesia’.1 2 A combined technique of general anaesthesia and a thoracic paravertebral block was performed. In this case a well-demarcated contralateral hemifacial flushing and ipsilateral pallor developed, without the distinctive ipsilateral Horner's syndrome.
After induction of general anaesthesia, a left paravertebral block was performed at T3/T4 using a 20G spinal needle and loss of resistance to saline technique. A total of 40 ml of local anaesthetic, bupivacaine 0.25% with 1 in 200 000 adrenaline, was injected into the paravertebral space. The operation lasted approximately 4 h and entailed patient repositioning from right lateral to supine. Cardiovascular stability was maintained throughout and the operation was completed without incident.
In the recovery room the patient was noted to have a marked hemifacial flushing of the right side (contralateral to the block) and marked pallor on the left side, in the absence of Horner's syndrome. The colour change persisted for approximately 5 h after operation and resolved without consequence.
Perioperative Harlequin syndrome is caused by sympathetic block of the thermal and emotional flushing response on the pale side, with normal or excessive flushing on the contralateral side.2 This case demonstrates that Harlequin syndrome can occur without Horner's syndrome after high volume paravertebral block (denoting T2/T3 sympathetic interruption with sparing of T1 oculomotor braches). Contrary to a previous report of harlequin syndrome without oculomotor signs1 following a T10/11 thoracic epidural, intraoperative positioning was not a factor.
Given that thoracic paravertebral injection of bupivacaine 0.5%, 15 ml causes somatic and sympathetic block over 5–8 dermatomes,3 it is perhaps surprising that Harlequin syndrome, is not a common occurrence. Only three perioperative adult cases following local anaesthetic administration have been described.1 2 4 This would imply that it is not dependent on a normal or excessive contralateral thermal and emotional flushing response2 but that this preserved response must be excessive and rare. Perioperative, local anaesthetic-induced Harlequin syndrome therefore may be different in mechanism to others described in the literature.5
London, UK
Editor—We would like to thank Drs Majumder and Farquhar-Smith for contributing with a further case report to the available literature regarding the perioperative Harlequin syndrome, and also for their valuable comments regarding the mechanism underlying this syndrome. In regard to the prevalence of Harlequin syndrome in patients exposed to regional anaesthesia in the vicinity of thoracic and cervical sympathetic system, there are few reported encounters, indeed. At the date of our report publication,2 there was only one other published case of Harlequin appearance in adults after an internal jugular central venous cannulation.6 Other cases were recently described,1 4 all in patients where migration of local anaesthetic at the level of preganglionic sympathetic fibres originating from T2 to T4 spinal segments was highly likely. Ever since we published our first case report,2 we encountered a further similar postoperative Harlequin syndrome in a 48-yr-old ASA I female patient after high thoracic (T3) unilateral continuous paravertebral analgesia for breast reconstruction surgery. She presented contralateral hemifacial flushing and sweating well-demarcated in the midline without the distinctive ipsilateral Horner syndrome. We were also anecdotally told of at least two other similar presentations after central venous line insertion. In all cases the hemifacial flushing and sweating was transient and not complicated. The question that arises is whether the prevalence of Harlequin syndrome after high thoracic regional anaesthesia is low or the conditions that trigger the presentation, such as heat, emotion, gustatory stimuli or exercise, are not always met, especially in the immediate perioperative period.
We cannot exclude that some patients are responding with disproportionate vasodilatation and sweating of the contralateral hemiface in response to stimuli such as heat or emotion. Experimental studies in rabbit models have described a closer proximity of endothelial beta- than alpha-adrenoceptors in relation to the sympathetic nerve terminals to the face.7 In vitro research on human facial veins has demonstrated a large inter-individual variability in the relative density and sensitivity of alpha- and beta-adrenoreceptors.8 It is possible therefore that, in some patients, the adrenergic receptors abundance and disposition may facilitate excessive beta-adrenergic vasodilatation in response to environmental stimuli.
As Richardson and Cheema recently affirmed,9 there is more fascinating matter to be learnt about the interaction between the paravertebral block and the afferent sympathetic supply to the face.
Dublin, Ireland
*E-mail: crina{at}ireland.com
References
1 Crawly SM. Coexisting Harlequin and Horner syndromes after high thoracic paravertebral block. Br J Anaesth 2006; 96:537–8
2 Burlacu CL and Buggy DJ. Coexisting Harlequin and Horner syndromes after high thoracic paravertebral anaesthesia. Br J Anaesth 2005; 95:822–4
3 Karamakar MK. Thoracic paravertebral block. Anesthesiology 2001; 95:771–80[CrossRef][Web of Science][Medline]
4 Mashour GA, Levine W, Ortiz VE. Intraoperative Harlequin syndrome. Anesth Analg 2006; 102:655
5 Lance JW, Drummond PD, Gandevia SC, Morris JG. Harlequin syndrome: the sudden onset of unilateral flushing and sweating. J Neurol Neurosurg Psychiatry 1988; 51:635–42
6 Coleman P and Goddard JM. Harlequin syndrome following internal jugular vein catheterisation in an adult under general anesthetic. Anesthesiology 2002; 97:1041[CrossRef][Web of Science][Medline]
7 Winquist RJ and Bevan JA. Relative location of alpha- and beta-adrenoceptors to sites of release of sympathetic transmitter in the rabbit facial vein. Circ Res 1981; 49:486–92
8 Mellander S, Andersson PO, Afzelius LE, Hellstrand P. Neural beta- adrenergic dilatation of the facial vein in man. Possible mechanism in emotional blushing. Acta Physiol Scand 1982; 114:393–9[Web of Science][Medline]
9 Richardson J and Cheema S. Thoracic paravertebral nerve block. Br J Anaesth 2006; 96:537
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