Figure 4 (postoperative MRI T2 sagital view)
Discussion:
The first description of Harlequin syndrome was in 1988 by Lance at
al.1They described five cases of adult patients with
unilateral flushing and sweating with contralateral anhidrosis. In years
that followed (1993) this phenomenon was described as a dysfunction of
the pregangliotic and postganglioticcervical sympathetic nerve fibers
and parasympathetic neurons of ciliary ganglion5.
The anatomy of sympathetic fibers innervating the face are made of a
three- neuron chain pathway: first neurons originate in hypothalamus and
synapse in the intermediolateral cell column of the upper thoracic
spinal cord with preganglionic fibers (second neurons) - somewhere
between Th1 – Th3 9. Preganglionic fibers leave the
spinal cord at Th2 -Th3, synapsing with the postganglionic fibers (third
neurons) in superior cervical ganglion. Then, postganglionic fibers
leave cervical ganglion passing either along internal carotid artery to
supply forehead, nose and eye, or along external carotid artery to
supply the rest of the face. Preganglionic oculosympathetic neurons
originate at the level of Th1.
In our case the lesion is at the level of inferior cervical ganglion
(stellate ganglion) given the symptoms – unilateral facial flushing and
upper extremity and trunk, without Horner’s syndrome. Harlequin sign in
our case presented probably due to resection of sympathetic nerves while
removing meningioma.
The PubMed database was searched online (PubMed, http:// pubmed.com). A
search query using Harlequin syndrome revealed 129 published cases of
which 23 was iatrogenic. (Table 1) Of 23 patients female sex was
predominant -15:8 ratio in which 8 were pediatric patients.
Most of the patients had undergone a thoracic surgery (8 patients),
Intrathecal pump in 3 patients, total thyroidectomy and neck surgery (3
patients), five patients underwent spinal nerve block, and only two had
neurosurgical procedure.
Conclusion:
Harlequin syndrome as a condition often frightens the patients since it
happens after the operation, while they perform their usual activities.
In most cases (about 80%) it resolves by itself within few hours. The
patient fear and anxiety can be calmed by explaining the benign nature
of the condition. If the patient has long term sequelae and the symptoms
aren’t tolerable, a contralateral sympathectomy or stellate ganglion
block are options for symptom relief32,33.