Background
Case summary: 36 years old male with back pain with paresthesia
of the right leg for over a one-year period. Magnetic resonance imaging
(MRI) showed intraduralextramedullary tumor. Tumor resection was
performed. Control magnetic resonance imaging (MRI) showed no signs of
residual tumor, recurrence of tumor or signs of ischemia. Six months
after the operation, the patient developed Harlequin syndrome with no
Horner’s syndrome.
Method: 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. Of 23 patients female
sex was predominant -15:8 ratio in which 8 were pediatric patients.
Objective: Harlequin syndrome in neurosurgical procedure is
rare, but we think that the surgeons must be aware of this condition as
a possible complication after the 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.
Keywords : Case report, harlequin syndrome, thoracic spinal
cord, autonomic disorder.
Introduction :
Harlequin syndrome is still a rare, uncommon autonomic disorder caused
by dysfunction of sympathetic innervation of the face. The symptoms that
characterize it are unilateral loss of flushing of the face and neck and
anhidrosis with compensatory flushing and sweating on the contralateral
side. Horner’s syndrome may be present.1
In most of the casesit is primarily idiopathic, but it can be associated
with diabetic neuropathy, GuillianBarre syndrome, Bradbury-Eggleston
syndrome, brain stem infarction,
superior mediastinal neurinoma and internal carotid artery dissection
(secondary Harlequin syndrome) or as accidental injury to the
sympathetic nervous system after invasive procedure - surgical
procedures, central line insertion, or a peripheral nerve block
(iatrogenic Harlequin syndrome) 2,3,4,This syndrome
may be transient or permanent.5,6,7
To the best of our knowledge the syndrome has been associated with
invasive procedures such as thoracotomy, cervical discectomy or
paravertebral nerve block. Here we present a unique case of Harlequin
syndrome without Horner syndrome after contralateral Th3 intradural
tumor resection.
Case report:
36 years old male presented with the occasional back pain with
paresthesia of the right leg for over a one-year period. Magnetic
resonance imaging (MRI) showed intraduralextramedullary tumor,
hyperintense mass on T1 - weighted contrast images, ventrolateral to the
right in the level of Th3 corpus, dimensions 1.7mm x 1mm (figure 1).
Neurological examination on admission was entirely normal,without any
neurological deficit, without sensory level. The decision was made to
proceed with the resection of the tumor.
Right sided hemilaminectomy was performed at the level of Th3. After the
initial exposure of the tumor, which was solid, adherent to adjacent
nerves, the tumor was totally resected. No intraoperative complications
were noted.After the operation patient recovered well, without any
neurological deficit. Subsequent histological analysis revealed that the
tumor was transitional meningioma WHO grade I.
The patient was doing well, he returned to his regular activities. Six
months after the operation, during the exercise the patient developed
left side facial flushing, following sweating on the left side of the
torso and face. Neurological examination was normal; there were neither
Horner’s syndrome nor any history of this occurring previously. No other
symptoms were evident.(figure 2 was provided by patient).Control
magnetic resonance imaging (MRI) showed no signs of residual tumor,
recurrence of tumor or signs of ischemia (figure 3 and 4). On follow-up
there was no recurrence of Harlequin syndrome.