Horner syndrome

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Classification according to ICD-10
G90.2 Horner syndrome
ICD-10 online (WHO version 2019)

Horner syndrome (also known as Horner's triad ) is the specific form of nerve damage caused by the failure of the sympathetic head or neck sympathetic, a part of the involuntary nervous system . It has a mostly one-sided, three-part symptom complex (triad), consisting of a pupillary constriction ( miosis ), the drooping of the upper eyelid ( ptosis ) and an eyeball that has sunk slightly into the eye socket ( enophthalmos ).

The clinical picture is named after the Swiss ophthalmologist Johann Friedrich Horner and his description from 1869. With reference to an earlier description by the French physiologist Claude Bernard , the terms Bernard-Horner syndrome or Claude Bernard-Horner syndrome are used synonymously.

Symptoms

Left-sided Horner syndrome

Constriction of the pupil

Pupillary constriction ( miosis ) occurs as a result of the failure of the sympathetically innervated dilatator pupillae muscle (pupil widener) in the iris . This results in a dilation deficit in the dark, i.e. H. the pupil does not dilate as it normally does in the dark. Those affected sometimes report problems with their eyesight in dark surroundings.

Retraction of the eyeball

The eyeball ( enophthalmos ), which has sunk back into the eye socket, is caused by the failure of the sympathetically innervated orbital muscle . These are smooth muscles in the periorbita , the outer connective tissue covering of the eye. The tone of this muscle usually ensures that the eye is pushed slightly outward. This form of enophthalmus is, in contrast to the cramps of the outer striated eye muscles ( musculus retractor bulbi , musculi recti bulbi ), hardly visible. In the specialist literature, there are different opinions as to whether the enophthalmos in Horner's syndrome is merely feigned by the ptosis (pseudoenophthalmos) or is actually present due to the impairment of the orbital muscle.

In animals, a partial prolapse of the nictitating membrane (also known as the "third eyelid") can occur.

Drooping of the upper eyelid

The drooping of the upper eyelid ( ptosis ) is caused by the failure of the sympathetically innervated tarsalis muscle (also Müllerian muscle ), which also consists of smooth muscles . In the healthy eye, this muscle gathers the eyelid in a vertical direction.

The actual lifter muscle of the upper eyelid, the levator palpebrae superioris , is significantly stronger and consists of striated muscles. It is somatomotorically innervated by the oculomotor nerve and is therefore not affected in Horner's syndrome. The ptosis is less pronounced in Horner's syndrome than in a failure of the oculomotor nerve. While there is also incomitating ptosis in levator paralysis, the extent of ptosis in Horners syndrome is almost the same in all directions.

More signs

In addition to these classic three main symptoms (triad) in the eye, further symptoms such as vasodilation ( vasodilation ) and reduced sweat secretion in the affected part of the face ( anhidrosis ) occur in the event of a sympathetic failure . Pigment spots in the eye, different pupil sizes and different colors of the iris are further indicators.

Pathophysiology

The individual symptoms of Horner's syndrome can be explained by failures along the anatomical course of the sympathetic innervation of the head region and are shown below for the peripheral part, i.e. not the central nervous part:

  • First neuron (first nerve cell): The cell bodies ( somata ) of the sympathetic nervous system are located in the upper thoracic cord (chest part of the spinal cord ). From there, axons (processes of neurons) move in the direction of the corresponding ganglia .
  • Stellate ganglion : The axons of the nerve cells of the sympathetic nervous system extend inside the sympathetic trunk headfirst. The nerve cell processes relevant for the Horner triad cross the stellate ganglion without being switched there. Horner syndrome, for example, can be an indication of a successful stellate blockade .
  • Neck part of the sympathetic nerve: in most mammals it occurs as the vagosympathetic trunk . This trunk pulls at the neck together with the common carotid artery dorsally (on the side facing the back) from the esophagus and trachea towards the head.
  • Second neuron : The switch takes place in humans in the ganglion cervicale superius , in animals called ganglion cervicale craniale . This ganglion is located ventrally (ventrally) to the upper cervical vertebrae. In animals, it is found caudally (towards the tail), near the base of the skull.
  • "Internal carotid plexus": On the further way, the sympathetic fibers can be damaged by a dissection of the internal carotid artery during their passage (without switching) through the internal carotid plexus.
  • Ganglion ciliare : The sympathetic nerve fibers pass through this parasympathetic ganglion behind the eye without further switching and thus reach the eye.

In relation to the superior cervical ganglion, a distinction is made between preganglionic and postganglionic damage to the sympathetic innervation of the head region. The latter is generally classified as less dangerous.

Causal diseases

A Horner symptom complex can occur in the following diseases and conditions, among others:

Diagnosis

  1. The pupil size is measured with a pupillometer in the light and in the dark.
  2. Cocaine test : one hour after instillation of cocaine , an indirect sympathomimetic , the pupil size is read again. If the difference in pupil size ( anisocoria ) is still greater than one millimeter, the presence of Horner's syndrome is very likely.
  3. Pholedrine test : If Pholedrine dilates the pupil in Horners syndrome, the damage must affect the first neuron in the hypothalamus or the second neuron in the nucleus intermediolateralis. If there is no enlargement, the third neuron, the cell body of which is located in the superior cervical ganglion, is affected. However, this test cannot differentiate between lesions of the first and second neurons.

therapy

There is no known therapy. In individual cases it has been reported that, depending on the cause, the external symptoms partially subside by themselves after a few months.

Individual evidence

  1. ^ Theodor Axenfeld (founder), Hans Pau (ed.): Textbook and atlas of ophthalmology. With the collaboration of Rudolf Sachsenweger a . a. 12th, completely revised edition. Gustav Fischer, Stuttgart a. a. 1980, ISBN 3-437-00255-4 , pp. 145, 150.
  2. ↑ JF Horner: About a form of ptosis. In: Clinical monthly sheets for ophthalmology , Stuttgart, 1869, Volume 7, pp. 193-198.
  3. ^ Bernard C .: Sur les effets de la section de la portion céphalique du grand sympathique . In: Comptes Rendus. Société de Biologie (Paris) . tape 4 , 1852, p. 168-169 .
  4. ^ Who named it Claude Bernard-Horner syndrome
  5. ^ Albert J. Augustin: Ophthalmology. 3rd, completely revised and expanded edition. Springer, Berlin a. a. 2007, ISBN 978-3-540-30454-8 , p. 1382.
  6. ↑ Herbert Kaufmann (Ed.): Strabismus. 3rd, fundamentally revised and expanded edition. Georg Thieme, Stuttgart a. a. 2003, ISBN 3-13-129723-9 , p. 486.
  7. ↑ B. Biedermann, M. Sojer, H. Stockner, M. Spiegel, C. Schmidauer: Dissections of the internal and vertebral carotid arteries: causes, symptoms, diagnosis and therapy. (PDF; 647 kB) In: Journal for Neurology, Neurosurgery and Psychiatry. Volume 8, No. 2, 2007, pp. 7-18, ISSN  1608-1587 .
  8. ↑ U. Lips, I. Conrad, F. Zevounou, B. Schappler-Scheele: Report on 2 cases of irreversible Horner syndrome after puncture of the internal jugular vein. In: Anesthesiology, Intensive Care Medicine, Emergency Medicine , Volume 17, No. 5, September 1982, pp. 301-302, ISSN  0174-1837 , PMID 6816087 .
  9. ↑ G. Reddy, A. Coombes, AD Hubbard: Horner's syndrome following internal jugular vein cannulation. In: Intensive Care Medicine , Volume 24, No. 2, February 1998, pp. 194-196, ISSN  0342-4642 , PMID 9539082 , doi: 10.1055 / s-2007-1003901 .
  10. ^ Pertti K. Suominen, Anna-Maija Korhonen, Sonia J. Vaida, Arja S. Hiller: Horner's syndrome secondary to internal jugular venous cannulation. In: Journal of Clinical Anesthesia , Volume 20, No. 4, June 2008, pp. 304-306, ISSN  0952-8180 , PMID 18617132 , doi: 10.1016 / j.jclinane.2007.10.016 .
  11. ↑ Recommendations for examination and diagnostic procedures in the case of pupillary disorders. (PDF; 106 kB) BVA and DOG , accessed on June 18, 2015 .
  12. ↑ Helmut Wilhelm, Evelina Schäffer: Pholedrin for the localization of Horner's syndrome . In: Klinische Monatsblätter Augenheilkunde , 1994, 204 (3), pp. 169-175, doi: 10.1055 / s-2008-1035515 .