Horner's syndrome

Horner's syndrome

Left-sided Horner's syndrome
Classification and external resources
Specialty neurology
ICD-10 G90.2
ICD-9-CM 337.9
OMIM 143000
DiseasesDB 6014
MedlinePlus 000708
eMedicine med/1029 oph/336
Patient UK Horner's syndrome
MeSH D006732

Horner's syndrome (also known as Bernard-Horner syndrome and oculosympathetic palsy) is a combination of symptoms that arises when a group of nerves known as the sympathetic trunk is damaged. The signs and symptoms occur on the same side as the lesion of the sympathetic trunk. It is characterized by miosis (a constricted pupil), ptosis (a weak, droopy eyelid), apparent anhidrosis (decreased sweating), with or without enophthalmos (inset eyeball).

The nerves of the sympathetic trunk arise from the spinal cord in the chest, and from there ascend to the neck and face. The nerves are part of the sympathetic nervous system, a part of the autonomic (or involuntary) nervous system. Once the syndrome has been recognized, medical imaging and response to particular eye drops may be required to identify the location of the problem and the underlying cause.[1]

Signs and symptoms

Signs that are found in patients on the affected side of the face include

Interruption of sympathetic pathways leads to several implications. It inactivates the dilator muscle and thereby produces miosis. It inactivates the superior tarsal muscle which produces ptosis. It inactivates the orbitalis muscle which produces the effect of enophthalmos. It also reduces sweat secretion in the face.

Sometimes there is flushing on the affected side of the face due to dilation of blood vessels under the skin. The pupil's light reflex is maintained as this is controlled via the parasympathetic nervous system.

In children, Horner's syndrome sometimes leads to heterochromia, a difference in eye color between the two eyes.[1] This happens because a lack of sympathetic stimulation in childhood interferes with melanin pigmentation of the melanocytes in the superficial stroma of the iris.

In veterinary medicine, signs can include partial closure of the third eyelid, or nictitating membrane.

Causes

Scheme showing sympathetic and parasympathetic innervation of the pupil and sites of a lesion in Horner's syndrome.

Horner syndrome is acquired as a result of disease, but may also be congenital (inborn, associated with heterochromatic iris) or iatrogenic (caused by medical treatment). Although most causes are relatively benign, Horner syndrome may reflect serious disease in the neck or chest (such as a Pancoast tumor (tumor in the apex of the lung) or thyrocervical venous dilatation).

Causes can be divided according to the presence and location of anhidrosis:

Pathophysiology

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

If someone has impaired sweating above the waist affecting only one side of the body, yet they do not have a clinically apparent Horner's syndrome, then the lesion is just below the stellate ganglion in the sympathetic chain.

Diagnosis

Left-sided Horner's syndrome in a cat as a result of trauma, demonstrating miosis in left pupil.

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

It is important to distinguish the ptosis caused by Horner's syndrome from the ptosis caused by a lesion to the oculomotor nerve. In the former, the ptosis occurs with a constricted pupil (due to a loss of sympathetics to the eye), whereas in the latter, the ptosis occurs with a dilated pupil (due to a loss of innervation to the sphincter pupillae). In a clinical setting, these two ptoses are fairly easy to distinguish. In addition to the blown pupil in a CNIII (oculomotor nerve) lesion, this ptosis is much more severe, occasionally occluding the whole eye. The ptosis of Horner syndrome can be quite mild or barely noticeable (partial ptosis).

When anisocoria occurs and the examiner is unsure whether the abnormal pupil is the constricted or dilated one, if a one-sided ptosis is present then the abnormally sized pupil can be presumed to be on the side of the ptosis.

In children

The most common causes in young children are birth trauma and a type of cancer called neuroblastoma.[4] The cause of about a third of cases in children is unknown.[4]

History

It is named after Johann Friedrich Horner, the Swiss ophthalmologist who first described the syndrome in 1869.[5][6] Several others had previously described cases, but "Horner's syndrome" is most prevalent. In France and Italy, Claude Bernard is also eponymized with the condition ("Claude Bernard-Horner syndrome").

See also

References

  1. 1 2 3 Ropper AH, Brown RH (2005). "14: disorders of ocular movement and pupillary function". In Ropper AH, Brown RH. Adams and Victor's Principles of Neurology (8th ed.). New York: McGraw-Hill Professional. pp. 222–45. doi:10.1036/0071469710. ISBN 0-07-141620-X.
  2. ↑ Laing, C; Thomas, DJ; Mathias, CJ; Unwin, RJ (2000). "Headache, hypertension and Horner's syndrome". Journal of the Royal Society of Medicine 93 (10): 535–536. PMC 1298129. PMID 11064693..
  3. 1 2 Lee JH, Lee HK, Lee DH, Choi CG, Kim SJ, Suh DC (2007). "Neuroimaging strategies for three types of Horner syndrome with emphasis on anatomic location". AJR Am J Roentgenol 188 (1): W74–81. doi:10.2214/AJR.05.1588. PMID 17179330.
  4. 1 2 Kennard, Christopher; Leigh, R. John (2011-06-28). Neuro-ophthalmology: Handbook of Clinical Neurology (Series Editors: Aminoff, Boller and Swaab). Elsevier. p. 452. ISBN 9780702045479.
  5. ↑ Horner JF (1869). "Über eine Form von Ptosis". Klin Monatsbl Augenheilk 7: 193–8.
  6. ↑ synd/1056 at Who Named It?
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