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Query: UMLS:C0016382 (flushing)
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We report on a case demonstrating unilateral Horner's syndrome (HS) after lumbar epidural obstetric anesthesia. A healthy, 32-year-old woman with a breech presentation was scheduled for an elective Cesarean section. The patient had normal vital signs throughout the surgical procedure. The operation lasted for 50 min. In the recovery room, she complained of left nasal stuffiness, left cheek numbness, and heaviness in her left eye. Meanwhile, left nipple sensory loss was noted during baby suckling training. On physical examination, her left eyelid was droopy along with left-side ptosis and facial flushing. Reduced sensation over the left hemifacial region and upper arm was also noted, which resolved completely over the next 110 min. A diagnosis of unilateral HS was then made. Although typically a benign side effect which often spontaneously resolves, HS is likely to cause anxiety in both the patient and the doctor. Prompt recognition of this syndrome and determination of its cause from lumbar epidural anesthesia can prevent unnecessary and potentially dangerous diagnostic workup and can reassure both patients and clinicians. The patient was discharged from the hospital 5 days after onset with a good outcome.
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PMID:Postpartum unilateral Horner's syndrome following lumbar epidural anesthesia after a Cesarean delivery. 1555 11

Horner's syndrome is a disorder of the sympathetic nerve supplying the eye. Infrequently, Horner's syndrome can arise as a complication of epidural anesthesia, but its clinical course is favorable. The incidence increases when epidural analgesia is used in obstetrics because of physiological and anatomic changes in obstetric patients that favor spread of the local anesthetic. We report the case of a 31-year-old woman requiring epidural analgesia for labor. She received 10 mL of 0.15% ropivacaine with a bolus dose of 50 microg of fentanyl, followed by epidural catheter infusion of 0.15% and 0.001% fentanyl at a rate of 10 mL/h. Two hours after starting the infusion, the patient's right eye presented a contracted pupil, a drooping eyelid, and enophthalmos, accompanied by flushing on the same side of the face. Horner's syndrome was diagnosed. Signs resolved over the next hour without treatment. The literature contains reports of widely differing incidences of Horner's Syndrome ranging from 1.3% to 75%. The case we report was the only one in our hospital over a period of 4 years during which 12,796 epidural procedures were performed. These data suggest to us that Horner's syndrome often passes undetected because clinical manifestations are not remarkable. Nevertheless, the diagnosis should be kept in mind so that unnecessary treatment is avoided, given that the clinical course is favorable with spontaneous resolution.
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PMID:[Horner's syndrome following epidural analgesia for labor]. 1558 41

We report a Japanese infant with Horner syndrome whose clinical examination and testing suggested the location of the causative lesion. A 4-year-old Japanese girl had an acquired right ocular ptosis and unequal pupils presenting shortly after birth. She also exhibited left hemifacial flushing and loss of sweating on the contralateral side (harlequin sign). Physical examination demonstrated 2.0 mm of ptosis of the right upper lid with normal elevator function. The diameters of the pupils were 4 mm on the left and 2.5 mm on the right. No sweating was induced in the right frontal region at 40 degrees C for 15 minutes of sweat challenge test. Otherwise, no abnormalities were found by the neurophysiologic examinations or magnetic resonance imaging of the brain. Based on the clinical examination, we speculated that the responsible lesion might be in the preganglionic areas. Harlequin sign was informative for making the diagnosis of Horner syndrome.
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PMID:Harlequin sign (hemifacial flushing and contralateral hypohidrosis) in a 4-year-old girl with Horner syndrome. 1691 33

We present a seven-month-old baby with miosis of the left pupil, left hypochromia, mild ipsilateral ptosis, left hemifacial anhidrosis and asymmetrical facial flushing. A diagnosis of Horner's syndrome (HS) was presumed and was confirmed by instillation of apraclonidine eye drops. Miosis was reversed upon apraclonidine instillation. Magnetic resonance imaging of the head, neck and thorax and ultrasonography of the neck and abdomen did not reveal any pathological conditions. Although delivery-related brachial plexus injury is known as the most common cause of congenital HS, it should be investigated and should include neuroimaging of the sympathetic pathway, to exclude a serious underlying disease. As in our case, a specific etiology may not always be elicited. Pharmacological testing with apraclonidine may be a practical alternative to cocaine in the diagnosis of HS.
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PMID:Congenital Horner's syndrome and the usefulness of the apraclonidine test in its diagnosis. 1692 Dec 19

Harlequin syndrome, the presentation of hemifacial flushing and sweating, is a well recognized, though rarely reported, phenomenon associated with cervical sympathetic trauma. It is thought to result from disruption to sudomotor and vasomotor neurons present in the cervical sympathetic chain. The more common Horner's syndrome classically comprises 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 concomitant 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 syndromes resulting from surgery, and illustrates the particular challenge of lymphatic malformations in neck surgery due to their non-adherence to anatomical planes.
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PMID:Coexisting harlequin and Horner syndromes after paediatric neck dissection: a case report and a review of the literature. 1908 11

Harlequin sign and harlequin syndrome, which are used interchangeably in the literature, are characterized by sudden onset of hemifacial sweating and flushing, induced by exercise and heat. Hemifacial sweating and flushing with normal ocular sympathetic innervation, known as harlequin syndrome, is rarely associated with tonic pupils, parasympathetic oculomotor lesion and pre- or postganglionic sudomotor sympathetic deficit. In the literature, hemifacial sweating and flushing in patients with apparently abnormal ocular sympathetic innervation has been defined as harlequin sign. To date, a few reports of excessive hemifacial sweating and flushing in structural lesion have been documented. Herein, we report five patients with excessive hemifacial sweating and flushing, two of whom had a syrinx. In presenting the patients, we have attempted to distinguish harlequin syndrome from harlequin sign. With this in mind, Case 1 can be described as harlequin syndrome resembling Ross syndrome, Case 2 as harlequin syndrome with normal ocular sympathetic innervation, Case 3 as harlequin sign with congenital Horner syndrome, Case 4 as harlequin sign with sympathetic and parasympathetic denervation sensitivity, and Case 5 as harlequin syndrome associated with occult sympathetic denervation sensitivity. These cases are discussed together with a review of the literature.
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PMID:Unnoticed dysautonomic syndrome of the face: Harlequin syndrome. 1756 97

Horner's syndrome is characterized by a classic triad of ipsilateral pupillary miosis, partial eyelid ptosis, and facial anhydrosis. This case study reports a 7-year-old boy with right miosis, mild blepharoptosis, and iris hypopigmentation detected in a routine pediatric follow-up without ipsilateral facial anhydrosis, flushing, or pain. There was no history of birth trauma and test with cocaine provoked no response of the right pupil, suggesting right Horner's syndrome. Mediastinal tumor was ruled out and brain magnetic resonance imaging incidentally showed absence of flow in the right internal carotid artery. Subsequent magnetic resonance angiography demonstrated agenesis of the right internal carotid artery without other vascular-associated malformations. The final diagnosis was right, congenital Horner's syndrome due to ipsilateral internal carotid agenesis. We describe in detail the radiological findings and pathophysiological mechanisms of this unusual association.
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PMID:Agenesis of internal carotid artery in a child with ipsilateral Horner's syndrome. 1916 24

Harlequin syndrome is rare and typically characterized by asymmetric flushing and sweating. Although it is usually considered idiopathic, literature review shows that it may be caused by lesion over lung apex or after central venous catheterization in the internal jugular vein. We present a 74-year-old woman who had been experiencing recurrent chest pain and right shoulder pain since 2 weeks ago. The tentative diagnosis was made by the emergency physician (EP) as acute coronary syndrome. The patient was given nitroglycerin treatment. Twelve hours later, the patient developed another episode of chest pain. The electrocardiogram and cardiac enzyme study results were, however, both normal. Further evaluation showed intermittent flushing over the left side of her face, as well as right-eye ptosis. A chest computed tomography (CT) was conducted, under the suspicion of Harlequin syndrome in combination with Horner syndrome, to derive the diagnosis of a right lung apex tumor. This case showed that history taking and physical examination are very important in the emergency department. It is particularly vital to observe the microchanges in the patient's symptoms and signs. It is also imperative to reassess the patient whose symptoms fail to improve under treatment, to look for other underlying lesions.
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PMID:An old lady with anterior chest pain and unilateral facial flushing. 2097 94

Agenesis of the internal carotid artery is a rare, usually asymptomatic congenital anomaly. Patients may remain asymptomatic because a network of collaterals develops in response to agenesis of the internal carotid artery, which is usually diagnosed as an incidental finding after magnetic resonance imaging. The collateral circulation is prone to developing aneurysms and subarachnoid hemorrhages. We report on a 16-year-old boy with a 1-year history of chronic daily headache. He manifested left-sided miosis and ptosis, with no evidence of anhidrosis, flushing, or pain. He was diagnosed with agenesis of the internal carotid artery with established collateral circulation. The association of Horner syndrome with agenesis of the internal carotid artery is not well reported. Agenesis of the internal carotid artery should be further evaluated to delineate the underlying mechanism, using computed tomography to examine the integrity of the bony carotid canal. Hypoplasia or agenesis of the carotid canal will confirm the congenital cause of the condition. If the canal is intact, dissection of the internal carotid artery should be considered.
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PMID:Agenesis of the internal carotid artery associated with ipsilateral Horner syndrome in a child. 2270 23

Harlequin syndrome is a rare neurological condition that results in unilateral facial flushing and sweating. Although the syndrome is generally a benign condition with complete resolution if appropriate treatment is initiated, unilateral facial flushing can be a sign of several serious conditions and should be thoroughly investigated. Sudden onset of facial flushing related to harlequin syndrome developed in a patient who had bilateral lung transplant with postoperative epidural anesthesia for pain control. Differential diagnosis includes neurovascular disease (acute stroke), malignant neoplasm of brain or lung, Horner syndrome, idiopathic hyperhidrosis, and Frey syndrome. Harlequin syndrome is often easily treated by discontinuing the anesthetic or adjusting placement of the epidural catheter.
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PMID:Harlequin syndrome as a complication of epidural anesthesia. 2488 29


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