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.