Marys Medicine


Coexisting harlequin and horner syndromes after paediatric neck dissection: a case report and a review of the literature

Journal of Plastic, Reconstructive & Aesthetic Surgery (2008) 61, 1382e1384 Coexisting harlequin and Horner syndromes afterpaediatric neck dissection: a case report anda review of the literature Jai N. Darvall , Adel W. Morsi, Anthony Penington Department of Plastic Surgery, Royal Children's Hospital, Flemington Road, Parkville 3052, Victoria, Australia Received 17 July 2006; accepted 20 April 2007 Harlequin syndrome, the presentation of hemifacial flushing and sweating, is a well Cervical sympathetic recognized, though rarely reported, phenomenon associated with cervical sympathetic trauma. It is thought to result from disruption to sudomotor and vasomotor neurons present Harlequin syndrome; in the cervical sympathetic chain. The more common Horner's syndrome classically comprises Horner's syndrome; the triad of unilateral miosis, ptosis and ipsilateral facial anhydrosis, and may also present as a sequela of cervical sympathetic denervation. We report a 26-month-old child with concom- itant Horner's and harlequin syndromes, following neck dissection to address a large cervical lymphatic malformation. To our knowledge this is the first reported case of both syndromesresulting from surgery, and illustrates the particular challenge of lymphatic malformationsin neck surgery due to their non-adherence to anatomical planes.
ª 2007 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published byElsevier Ltd. All rights reserved.
Magnetic resonance image and ultrasound scan revealeda 5.3  3.5 cm multiseptated, cystic malformation deep tothe left sternocleidomastoid muscle, displacing it laterally.
A 26-month-old boy weighing 15 kg underwent elective It was seen to wrap around the carotid vessels and internal neck dissection for a left cervical lymphatic malformation.
jugular vein with displacement of trachea and oesophagus Clinically, a fluctuant mass was palpable in the left anterior to the right. Attempted percutaneous sclerotherapy with cervical triangle, with minor tracheal deviation to the right.
doxycycline 3 months prior to surgery was followed byincreasing neck swelling, with worsening noisy breathingat night. Further assessment by way of nasoendoscopy * Corresponding author. Address: Department of Plastic Surgery, demonstrated incipient airway obstruction, and it was Royal Children's Hospital, Flemington Road, Parkville, Victoria3052, Australia.
decided to proceed to early surgical resection to avoid E-mail address: (J.N. Darvall).
the risk of complete tracheal obstruction with further 1748-6815/$ - see front matter ª 2007 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

Coexisting harlequin and Horner syndromes after paediatric neck dissection sclerotherapy. Past history included mild asthma from 6 nor any demonstrable motor deficit. Of note, erythema months of age, well controlled on Ventolin and Flixotide.
was confined to the face, with no involvement of limbs or At operation, the mass was found to be densely adherent trunk. The hemifacial flushing was found to be well related to surrounding structures, including the trachea and to emotion, i.e. it was more florid during crying spells.
oesophagus. The vagus, recurrent laryngeal and hypoglossal Over the next 2 weeks, the right facial plethora intermit- nerves were all seen and preserved. Two drains were tently appeared, less pronounced as time progressed. At the inserted and the wound closed in layers.
time of report (2 months following the second operation) The patient remained intubated and ventilated for 24 h the harlequin syndrome persists subtly, with no change in postoperatively in the intensive care unit. Extubation the the severity of the left Horner's syndrome.
next evening proceeded uneventfully, however the drainswere accidentally pulled out by the patient. The childwas transferred the following morning to the ward, where the left side of the neck was noted to have become moreswollen. This coincided with symptoms consistent with The sudden presentation of hemifacial flushing and a left Horner's syndrome e namely a miotic left pupil sweating was first termed ‘harlequin syndrome' by Lance with left sided ptosis. No other symptoms, nor any other et al. in 1988, named after the similar though separate neurological change were evident at that stage. After further increase in the size of the left neck over the next newborns in the dependent half of the body.Since then, 2 days, the patient was returned to theatre on the fourth the syndrome has been well represented in the literature.
postoperative morning. A drain was reinserted, with a small Acquired harlequin syndrome, due to sympathetic denerva- amount of clear lymphatic fluid removed.
tion, has been associated with central autonomic disruption Two hours after returning to the ward, the patient was in cases of stroke, as well as linked to more peripheral noted to have developed a typical harlequin appearance, autonomic denervation due to trauma, anaesthesia and with facial flushing on the right (non-operative) side, and tumours.It is important to recognise that the flushed a clear demarcation at the midline (The right side ‘harlequin' side represents the normally innervated half of the face was clinically warmer to touch, though there of the face. The pathological appearance is due to an was no obvious disparity in sweating patterns. There was inability on the ipsilateral, affected side to mount a vaso- no change in the previously noted left Horner's syndrome, motor response to sympathetic stimuli, resulting in unequalflushing on the contralateral side of the face.
The sympathetic innervation to the face derives from pupillomotor fibres that leave the cord at T1 level, withsudomotor and vasomotor fibres exiting at T2 and T3. Fromhere, preganglionic fibres enter the cervical chain,ascending to synapse in the superior cervical ganglion,which lies just anterior to the C1 and C2 vertebrae. Thirdorder neurons exit this ganglion rostrally to pass along theinternal carotid artery to supply the eye and forehead,whilst fibres from the caudal aspect of the ganglion travelwith the external carotid nerve to supply the rest of theface. Postganglionic fibres also project more proximallyfrom the stellate ganglion, located more inferiorly justanterior to the C7 vertebrae, to innervate the upper arm.
The presence, then, of coexisting Horner's and harlequinsyndromes in this patient localises the lesion to, orimmediately proximal to, the superior cervical ganglion.
Importantly, there were no colour changes observed in theupper limbs, indicating sympathetic denervation distal tothe stellate ganglion.
Reports of harlequin syndrome in the perioperative setting are uncommon, particularly in the paediatricpopulation. We believe this represents the first reportedexample of concomitant Horner's and harlequin syndromedue to surgery.
A similar case was reported in 1996, in which a 2-year-old child underwent right neck dissection to remove a lymphaticImmediately postoperatively, contralateralfacial harlequin changes were noticed which persisted for3 hours, worsened by crying or coughing. In a second caselinked to neck dissection, a 3-month-old child had a neuro-blastoma of the left cervical sympathetic chain excised.
Postoperative photograph showing flushing of the Initially this tumour had presented with an ipsilateral Horn- right side of the face with clear transition at the midline.
er's syndrome, preceding surgery, but postoperatively the

J.N. Darvall et al.
patient developed typical harlequin plethora on the contra- lateral (right) side, attributed to further operative sympa-thetic damage. Intraoperative right facial erythema was 1. Lance JW, Drummond PD, Gandevia SC, et al. Harlequin also noted in a 9-month-old child during Nissen fundoplica- syndrome: the sudden onset of unilateral flushing and sweating.
tion and gastrostomy to address aspiration pneumonitis.
J Neurol Neurosurg Psychiatry 1988;51:635e42.
No surgical nor anaesthetic causes were ascertained, and 2. Rousseaux M, Hurtevent JF, Benaim C, et al. Late contralateral the changes resolved completely within 1 hour.
hyperhidrosis in lateral medullary infarcts. Stroke 1996;27: An adult case of perioperative harlequin syndrome involved a 46-year-old woman undergoing laparotomy for 3. Burlacu CL, Buggy DJ. Coexisting harlequin and Horner syndromes after high thoracic paravertebral anaesthesia. Br J an abdominal abscess, in which transient harlequin Anaesthesia 2005;96:822 syndrome was observed after right internal jugular vein 4. Lombardi C, Vetrugno R, Provini F, et al. Harlequin syndrome: an cannulation.The proposed mechanism was neuropraxia of association with overlap parasomnia. J Neurol Neurosurg Psychi- the right cervical sympathetics due to direct trauma.
atry 2004;75:341e2.
In summary, a case is presented of concurrent Horner's 5. Noda S. Harlequin syndrome due to superior mediastinal syndrome and harlequin syndrome. This has been caused by neurinoma. J Neurol Neurosurg Psychiatry 1991;54:744.
presumed surgical injury to the sympathetic chain between 6. Turco GR, Farber NE. Postoperative autonomic deficit: a case of the stellate and superior cervical ganglia. Lymphatic harlequin syndrome. Anesthesiology 1996;85:1197e9.
malformations present a special challenge in neck surgery 7. Padda GS, Cruz OA, Silen ML, et al. Skin conductance responses in because of the extent and size of the lesions and their lack paediatric Harlequin syndrome. Paediatr Anaesth 1999;9:159e62.
8. Wagner DL, Sewell AD. Harlequin color changes in an infant of respect for normal anatomical boundaries. This predis- during anesthesia. Anesthesiology 1985;62:695.
poses them to surgical complications, including damage to 9. Coleman P, Goddard J. Harlequin syndrome following internal the cervical sympathetic chain, beyond those seen in jugular vein catheterization in an adult under general anaes- routine neck dissection.
thetic. Anesthesiology 2002;97:1041.
Template for the male areola of the round nipple-areola complex in the EuropeanmaleSuch a template may be useful in planning areolarreduction in gynaecomastia and in bilateral areolar recon- The plastic cap from lignocaine vials (AstraZeneca) has struction ).
a diameter of 23 mm which equates to the mean diameter 1. Beer GM, Budi S, Seifert B, et al. Configuration and localiza- tion of the nipple-areola complex in men. Plast Reconstr Surg2001;108:1947e52.
Leicester Royal Infirmary, Leicester, UK ª 2008 Published by Elsevier Ltd on behalf of British Associationof Plastic, Reconstructive and Aesthetic Surgeons.
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