It results in ptosis (drooping upper eyelid), miosis (constricted pupil), and occasionally enophthalmos (the impression that the eye is sunk in) and anhidrosis (decreased sweating) on one side of the face.

In children Horner's syndrome sometimes leads to a difference in eye color between the two eyes (heterochromia). This happens because a lack of sympathetic stimulation in childhood interferes with melanin pigmentation of the melanocytes in the superficial stroma of the iris.

History

It is named after Dr Johann Friedrich Horner (1831-1886), the Swiss ophthalmologist who first described the syndrome in 1869. Several others had previously described cases, but "Horner's syndrome" is most prevalent. In France, Claude Bernard is also eponymised with the condition being called "syndrome Bernard-Horner".

Causes

Horner's syndrome is usually acquired but may also be congenital. Although most causes are relatively benign, Horner's syndrome may reflect serious pathology in the neck or chest (such as a Pancoast tumor) and hence requires workup.

Horner's Syndrome is due to a deficiency of sympathetic activity. The site of lesion to the sympathetic outflow is on the ipsilateral side that the symptoms are on. The following are examples of conditions that cause the clinical appearance of Horner's Syndrome:

First-order neuron disorder: Central lesions that involve the hypothalamospinal pathway (e.g. transection of the cervical spinal cord).
Second-order neuron disorder: Preganglionic lesions (e.g. compression of the sympathetic chain by a lung tumor).
Third-order neuron disorder: Postganglionic lesions at the level of the internal carotid artery (e.g. a tumor in the cavernous sinus).

Diagnosis

Three tests are useful in confirming the presence and severity of Horner's syndrome:

Cocaine test - Cocaine blocks the reuptake of norepinephrine resulting in the dilation of a normal pupil. The pupil will fail to dilate in Horner's syndrome.
Paredrine test
Dilation lag test

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Chiropractic manipulation of the neck with Horner's syndrome and internal carotid artery dissection
From Journal of the American Chiropractic Association, 4/1/02 by Haneline, Michael T

The April 2001 issue of the American Journal of Ophthalmology contained a report by Parwar et al.1 implicating chiropractic manipulation therapy (CMT) of the cervical spine in Homer's Syndrome and internal carotid artery dissection (ICAD). The article related that a 44-year-old female received cervical CMT on nine occasions, followed by the manifestation of ICAD symptoms three days after her last chiropractic visit. The patient indicated that each chiropractic session included neck massage, followed by sharp rotation of her head to either side until she felt a "crack." She presented to the emergency room complaining of a severe headache, right-sided neck pain, ptosis, and miosis. Physical examination confirmed the Homer's Syndrome and she was accordingly given a magnetic resonance angiogram (MRA) to evaluate the cervical arteries. MRA revealed dissection of the right internal carotid artery (ICA) within the petrous portion of the temporal bone. There was also an intimal flap in the superior cervical portion of the right vertebral artery (VA). The patient was treated with anticoagulants, and no further neurologic abnormalities developed.

There are several problems with the supposition that Parwar et al. have made in this case. The first concerns the location of the ICAD, which was within the petrous portion of the temporal bone. In our estimation, this is not a region of the artery that is susceptible to CMT-related injury. The theoretical mechanism of cervical CMT causing ICAD involves the cervical portion of the ICA, where it lies in close proximity to the upper-cervical vertebrae.2

The cervical portion of the ICA is freely moveable,3 but as the head and neck are hyperextended, and either rotated or laterally flexed to the opposite side, the ICA becomes fixed to the anterior surface of the upper cervical vertebrae. In this position, a sudden severe stretch to the artery is purported to tear the arterial intima and result in ICAD.4 In contrast, the ICA is not moveable within the temporal bone, and, at that level, has not been associated with the previously mentioned mechanism of injury. There have been reports of intracranial artery dissection following head trauma, but a similar mechanism of causation bv cervical CMT does not occur. Pelkonen et al.5 described a case of ICAD related to vigorous head movements while dancing, but cervical CMT does not involve similar amplitudes or accelerations of head movement, and thus cannot reasonably be compared.

The association of cervical ICAD with CMT is tenuous, though there have been rare reports in the literature. To our knowledge, there have only been three case reports attributing ICAD to cervical CMT in American medical history. Three other incidents were reported by Lee et al.6, which were derived from a survey of California neurologists. They indicated that three of the 56 strokes reported in the survey involved the carotid distribution. This survey, however, was based only on physician recall, and no verification of cause and effect between CMT and the various disorders attributed to it was required. Other limitations to the study were acknowledged by the study's authors. Furthermore, only 36 percent of the neurologists that were sampled actually returned the survey, which may have biased the results. The medical literature is equivocal regarding the relationship of cervical CMT to ICAD, and it reflects a widespread uncertainty concerning the etiology of ICAD7,8,9.

The CMT-related ICAD case that the authors cited, by Peters et al.10, described a case of ICAD that followed cervical CMT by three hours. Prior to the manipulation, the patient exhibited symptoms that may have been prodromal to the ICAD, including increasing neck pain and five episodes of vertigo. The patient had possible predisposing factors that included smoking and the use of oral contraceptives. However, the most likely basis for the dissection was a segmental mediolytic arteropathy, with CMT acting as a triggering mechanism. Notwithstanding, the Peters et al. case represents the closest that any reported occurrence of ICAD following CMT comes to assigning causation. The connection was based on a temporal relationship of the CMT to ICAD. Given the presence of significant predisposing factors, however, the issue of causation was not at all clear.

Although trauma may cause ICAD, most patients present with no history of injury. For that reason, most ICADs are considered spontaneous.11 Many other trivial events have been implicated as causing ICAD, such as pushing a car, scuba diving, turning the head, coughing, vomiting, rhythmic movement of the head and neck to music, and childbirth.12,13 Undoubtedly, most patients, if diligently queried, could recall events in the hours and days prior to presentation similar to those described in the literature as potential ICAD triggers.

While cervical CMT might be a contributing factor in ICAD that develops within hours or possibly even days after manipulation, it is not appropriate to attribute causation based solely on a temporal relationship. Moreover, doctors of chiropractic are not trained to perform cervical CMT with concomitant cervical hyperextension and either rotation or lateral flexion, the mechanism hypothetically required to jeopardize the ICA A high percentage of cervical artery dissection occurrences have been ascribed to non-chiropractic practitioners," who are usually not adequately trained in manipulation" and may employ unsafe techniques.

In closing, we consider that the case presented by Parwar et al. does not support the conclusion that "Acute, painful Homer's Syndrome as a manifestation of vascular dissection may be associated with chiropractic manipulation of the neck." The medical evidence does not support the causal relationship between cervical CMT and ICAD, and putative biomechanical mechanisms are lacking.

References

1. Parwar BL, Fawzi AA, Arnold AC, Schwartz SD. Horner's syndrome and dissection of the internal carotid artery after chiropractic manipulation of the neck. Am J Ophthalmol 2001 Apr; 131(4):523-4.

2. Davie J, Zimerman R. Injury of the carotid and vertebral arteries. Neuroradiology 1983;25:55-69.

3. Ringel S, Harrison S, Norenberg M, Austin J. Fibromuscular dysplasia: multiple "spontaneous" dissecting aneurysms of the major cervical arteries. Ann Neurol 1977;1:301-304.

4. Stringer W, Kelly D. Traumatic dissection of the extracranial internal carotid artery. Neurosurgery 1980;6:123-30.

5. Pelkonen O, Tikkakoski T, Leinonen S, Pyhtinen J, Sotaniemi K. Intracranial arterial dissections. Neuroradiology 1998;40:442-447.

6. Lee K' Carlini W, McCormick G, Albers G. Neurologic complications following chiropractic manipulation: a survey of California neurologists. Neurology 1995;45(6):1213-1215.

7. Desfontaines P, Despland PA. Dissection of the internal carotid artery and treatment in 60 consecutive cases. Acta Neuol Belg 1995;95:226-234.

8. Hart RG, Easton JD. Dissections of cervical and cerebral arteries. Neurol Clin 1983;1:155-182.

9. Brandt T, Hausser I, Orberk E, Grau A, Hartschuh W; Anton-- Lamprecht I, Hacke W. Ultrastructural connective tidue abnormalities in patients with spontaneous cervicocerebral artery dissctions. Ann Neural 1998;44(2)281-285.

10. Peters M, Bohl J, Thomke F, Kallen K, Mahlzahn K, Wandel E, Meyer zum Buschenfeld K Dissection of the internal carotid artery after chiropractic manipulation of the neck. Neurology 1995,45:2284-2286.

11. Ozdoba C, Sturzenegger M, Schroth G. internal carotid artery dissection: MR imaging features and clinical-radiologic correlation. Radiology 1996 Apr; 199(1):191-8.

12. Ast G, Woimant F, Georges B, Laurian C, Haguenau M. Spontaneous dissection of the internal carotid artery in patients. Eur J Med 1993;2(8):466-472.

13. Sturzenegger M. Spontaneous internal carotid artery dissection: early diagnasis and management in 44 patients. J Neurol 1995; 242(4)231-238.

14 Terrett A Veltebrobasilar Stroke Following Manipulation West Des Moines: National Chiropractic Mutual Insurance Company; 1996.16, 67.

15. winkler K, Maurer F, Hegetschweiler-Goertz C, McClelland G, Meeker W, Padgett K, Jackson P. Policy Statement on Spinal Manipulation. American Chiropractic Association. Aug 1999:6.

BY MICHAEL T. HANELINE, DC, FICR, AND ARTHUR C. CROFT, DC, MS, MPH

Drs. Haneline and Croft may be contacted at the Spine Research Institute of San Diego Center for research into Automotive Safety and Health (CRASH), 2731 Via Orange Way, Suite 105, Spring Valley, CA 91978, Tel 619/660-8802.

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