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Horner's Syndrome

Congenital Horner Syndrome

Horner's syndrome (Bernard-Horner syndrome, Claude Bernard-Horner syndrome) is caused by a lesion in the sympathetic nervous system and characterized by the classic triad of miosis, ptosis and decreased hemifacial sweating.


Presentation

Horner’s syndrome affects one side of the face. The following are the signs and symptoms experienced by individuals suffering from this syndrome:

  • Onset of miosis, a condition wherein there is decrease in the size of the pupil.
  • Anisocoria, wherein there is considerable difference between both pupils.
  • Delayed dilation of pupil in dim light.
  • Onset of upside down ptosis, a condition characterized by elevation of lower eye lid.
  • Dropping eye lids, especially the upper one.
  • Affected side experiences little or no sweating.
  • Children affected by Horner’s syndrome, do not experience the flushing or redness of face in the affected side as one would normally experience after exposure to heat or after physical exertion. In addition, the color of the iris in affected children is lighter than the normal eye [6].
Euthyroid
  • The postoperative work-up revealed a eutopic and euthyroid thyroid gland. The postoperative course was marked by Horner's syndrome that persisted at 1-year follow-up.[ncbi.nlm.nih.gov]
Intravenous Drugs
  • This case highlights that with the increasing intravenous drug use, this rare presentation of HS may become more common.[ncbi.nlm.nih.gov]
Hypotension
  • .  Constricts with weak Pilocarpine  Holmes-Adie syndrome  Reduced tendon reflexes (Knee, ankle) - Orthostatic hypotension Afferent & efferent defects 28. Adie’s tonic pupil (OD) 29. Argyll-Robertson pupil 30. Horner’s pupil (OS) 31.[slideshare.net]
  • Vitals remained stable except for two episodes of hypotension (blood pressure - 89/54 mm Hg and 90/50 mm Hg) late in intraoperative period and managed with 6 mg injection mephentermine.[ijaweb.org]
  • Cardiac tamponade: an unlikely cause of unexplained hypotension in an isolated "minor" blunt chest injury. Singapore Med J 1997; 38:35-36. [ Links ] 14. Geusens E, Pans S, Prinsloo J, Fourneau I. The widened mediastinum in trauma patients.[scielo.br]
  • […] apraclonidine test The topical apraclonidine test is a practical and reliable alternative to the topical cocaine test; it is readily available and adequately sensitive (87%) and is currently the test of choice. [ 22 , 23, 24 ] Apraclonidine is an ocular hypotensive[emedicine.medscape.com]
Ptosis
  • Besides its diagnostic value in Horner’s syndrome, topical apraclonidine could correct ptosis for the sake of esthetics or when ptosis reduces the superior visual field.[ncbi.nlm.nih.gov]
  • The ptosis of Horner syndrome can be quite mild or barely noticeable (partial ptosis). [ citation needed ] 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[en.wikipedia.org]
  • Postoperatively, left ptosis and miosis had improved slightly. To our knowledge, this is the first report regarding mediastinal GCT presenting with preoperative Horner's syndrome.[ncbi.nlm.nih.gov]
  • Rarely, the ptosis may be permanent and require surgical intervention.[ncbi.nlm.nih.gov]
  • Patient 2 was a 42-year-old man who presented to our emergency department with persistent headache and ptosis over several weeks.[ncbi.nlm.nih.gov]
Miosis
  • Postoperatively, left ptosis and miosis had improved slightly. To our knowledge, this is the first report regarding mediastinal GCT presenting with preoperative Horner's syndrome.[ncbi.nlm.nih.gov]
  • Horner's syndrome is characterized by a classic triad of ipsilateral pupillary miosis, partial eyelid ptosis, and facial anhydrosis.[ncbi.nlm.nih.gov]
  • Diagnosis [ edit ] Left-sided Horner's syndrome in a cat as a result of trauma, demonstrating miosis in left pupil.[en.wikipedia.org]
  • Horner's syndrome (oculosympathetic paresis) is characterised by the classic triad of ipsilateral palpebral ptosis, pupillary miosis and facial anhidrosis.[ncbi.nlm.nih.gov]
  • Right upper lid ptosis, right miosis and anisocoria were recognized on the first postoperative day. Findings of Horner's syndrome were ameliorated completely at the 6th month.[ncbi.nlm.nih.gov]
Enophthalmos
  • A 36-year-old Sri Lankan Sinhalese woman developed unilateral partial ptosis with enophthalmos and myosis one week after total thyroidectomy for a benign multinodular goiter. A clinical diagnosis of Horner's syndrome was made.[ncbi.nlm.nih.gov]
  • Horner's syndrome is characterized by myosis, eyelid ptosis, enophthalmos, and lack of sweating, with vascular dilatation of the lateral part of the face, caused by damage of the cervical sympathetic chain.[ncbi.nlm.nih.gov]
  • It is characterized by miosis (a constricted pupil), partial ptosis (a weak, droopy eyelid), apparent anhidrosis (decreased sweating), with or without enophthalmos (inset eyeball ).[en.wikipedia.org]
  • Evidence favors idea that muscle of Muller although important in animals is vestigal in man and does not contribute to either enophthalmos by paralysis or exophthalmas by stimulation.[ghorayeb.com]
  • Enophthalmos (the impression that the eye is sunk in) and anhydrosis (decreased sweating) on the affected side of the face, loss of ciliospinal reflex and blood shot conjunctiva might occur depending on the site of lesion.[eyeplastics.com]
Blepharoptosis
  • Two weeks later, she developed blepharoptosis and anisocoria with a smaller pupil in the right eye, which increased in the darkness.[ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]
  • Clinical features include miosis; mild blepharoptosis; and hemifacial anhidrosis (decreased sweating)(see hypohidrosis).[icd10data.com]
  • Clinical features include MIOSIS; mild BLEPHAROPTOSIS; and hemifacial ANHIDROSIS (decreased sweating)(see HYPOHIDROSIS).[fpnotebook.com]
Pupillary Abnormality
  • Pupillary abnormalities . Also consider unilateral use of miotic drugs. Investigations Investigations will be guided by suspected aetiology. For example: [ 8 ] CXR may show an apical carcinoma of the lung.[patient.info]
  • Finally, an abnormality of the third cranial nerve (a nerve that comes from the brain to the eye socket and controls eyelid position, eye movement, and pupil size) can cause a pupillary abnormality.[aapos.org]
  • Methods Nineteen patients with unilateral Horner syndrome (Horner group) and age-matched controls of 30 healthy individuals with normal vision and neither optic nerve dysfunction nor pupillary abnormalities were included.[journals.plos.org]
Stroke
  • […] not in the course of PCA stroke.[stroke.ahajournals.org]
  • (hemispheric) weakness, sensory deficit, homonymous hemianopia, diplopia, or ataxia No sensory or motor level (instead, hemiplegia) Brainstem stroke (lateral medullary syndrome) Contralateral pain and temperature sensory loss Ipsilateral facial sensory[derangedphysiology.com]
  • Discussion Carotid artery dissection (CAD) causes ischemic stroke in 2% of the general population, but accounts for 10-25% of ischemic strokes in the young to middle-aged population. 1 Though no predilection for sex has been documented, the condition[reviewofoptometry.com]
  • – while stroke is the end consequence of untreated carotid dissection, it’s important to know to make the diagnosis as you need contrast for the scan to pick it up headache – a headache and horner’s should make you at least think about a diagnosis of[emergencymedicineireland.com]
Hyperesthesia
  • As the injury was not a complete hemisection of the spinal cord, the patient presented with ipsilateral motor deficit and hyperesthesia and diminished contralateral fine touch sensation; however, proprioception, vibration and temperature were all initially[ncbi.nlm.nih.gov]
Homonymous Hemianopsia
  • Furthermore, left homonymous hemianopsia, left motor and sensory neglect, left hyperhidrosis, right Horner’s syndrome (Fig 1 ), and visuospatial apraxia were noted. Excessive sweating was particularly marked on the face, thorax, and hand.[stroke.ahajournals.org]

Workup

A general medical examination would be carried out in the preliminary stages, followed by other tests to identify the nature of the symptoms and exact cause behind development of Horner’s syndrome. Typically the following tests would be done:

  • Eye examination: In this test, eye drops will be administered to both eyes; the pupil of the healthy eye will get dilated, whereas the eye drops will constrict the pupil of the affected eye. This will help in confirming the diagnosis of Horner’s syndrome.
  • Imaging studies: Imaging studies such as CT scan, MRI and X-ray will be done in order to locate the site of nerve damage. A chest radiograph would provide insight about the development of apical bronchogenic carcinoma which is considered as one of the most potential causes of Horner’s syndrome. In addition, MRI of the brain and CT scan of the head are also indicated [7].
  • Blood and urine tests would also form an important part of the diagnostic procedure and are done if children are suspected to be suffering from Horner’s syndrome. These tests are necessary to diagnose the presence of neuroblastoma [8].
  • Pharmacologic testing: This method is very helpful in diagnosis of Horner’s syndrome. These include the topical cocaine test, topical apraclonidine test and topical hydroxyamphetamine test [9].

Treatment

There is no specific treatment regime for Horner’s syndrome. The major goal of treatment is to successfully treat the underlying disease condition, which would in turn help in appropriate management of the condition.

Surgical intervention may also be required when the carotid artery is involved. The types of surgical procedures indicated in treatment of Horner’s syndrome include neurosurgical procedures, and vascular surgical methods for aneurysm or dissection of the carotid artery [10].

Prognosis

The prognosis of the condition majorly depends on the underlying causative factor. If the underlying causative factor can be successfully treated, then the prognosis of Horner’s syndrome is usually favorable.

Etiology

Horner’s syndrome occurs when certain groups of nerves of the sympathetic trunk are damaged. The sympathetic nerves are responsible for regulation of the heart rate, blood pressure, perspiration and size of the pupil. Injury or damage to the nerve fibers can stem from the following factors [2]:

  • Injury to the carotid artery
  • Individuals suffering from migraine [3]
  • Development of tumor in the lungs
  • Development of tumor in the brainstem
  • Stroke
  • Sympathectomy, wherein surgery is done to relieve pain by interrupting the nerve fibers
  • Injury to the nerves of the brachial plexus
  • In many instances, the cause of Horner’s syndrome is unknown and cannot be identified. Such a type of condition is then termed as idiopathic Horner’s syndrome.

Epidemiology

Horner’s syndrome is a rare disorder affecting about 1 in 6250 newborns. The condition can also occur later in life; but the exact incidence in such cases is unknown [4].

Sex distribution
Age distribution

Pathophysiology

Horner’s syndrome develops as a result of a lesion which occurs at any point in the pathway of the sympathetic trunk. Due to such a kind of phenomenon, all patients experience abnormalities such as ptosis, elevation of the lower eye lid, development of dilation lag and miosis and impaired flushing and sweating. The degree of severity of these abnormalities depends on the extent of damage to the nerve fibers.

Anatomically, 3 groups of nerves responsible for the sympathetic function of the eye are the first order, second order and third order neurons. The first order neurons travel from the hypothalamus to the cervical spine. The second order neurons travel from neck to the outer side of the spinal region. Lastly, the third order neurons, travel through the outer side of the spinal cord to the eye [5].

Prevention

Prevention of Horner’s syndrome is geared towards preventing the onset of trigger factors. This would include prevention of trauma of the head, avoiding smoking and controlling various cardiovascular risk factors. All these approaches can help prevent the onset of Horner’s syndrome.

Summary

Horner’s syndrome is characterized by development of various kinds of signs and symptoms that occur as result of damage to the sympathetic trunk. Such a type of condition affects the face and eyes. This syndrome produces signs and symptoms that affect one side of the body where there is lesion of the sympathetic trunk. Horner’s syndrome causes decrease in the size of the pupil, drooping eyelid and reduced sweating particularly on the side of the face that has been affected. The condition is also known as oculosympathetic palsy or Horner-Bernard syndrome [1].

Patient Information

  • Definition: Horner’s syndrome occurs when the nerve pathway that travels through brain to the face and then reaches the eye, gets damaged. This nerve pathway is disrupted only one side of the body. In this condition, a specific group of symptoms mark the onset of the syndrome.
  • Cause: Horner’s syndrome mainly occurs due to underlying disease condition such as tumors, injury to the spinal cord or a stroke. In many cases, no cause can be identified and in such situations, the syndrome is termed as idiopathic Horner’s syndrome.
  • Symptoms: Symptoms of Horner’s syndrome include decrease in the size of the pupil, delayed dilation of the pupil in dim light, and reduced sweating on one side of the face that is affected. In children with Horner’s syndrome, the color of the iris of the affected eye is different from the normal. In addition, the part of the face that is affected does not turn red after physical exertion or exposure to hot weather.
  • Diagnosis: A preliminary medical examination of the signs and symptoms experienced by the individual forms basis of the diagnostic procedure. An eye examination is also carried out to evaluate the dilation of pupils in response to eye drops. Imaging studies such as chest X-ray, MRI of the brain and CT scan of the head would also be necessary.
  • Treatment: Treatment of Horner’s syndrome largely depends on the underlying cause. If the causative factor can be treated then, the syndrome gets corrected by itself.

References

Article

  1. Reede DL, Garcon E, Smoker WR, Kardon R. Horner's syndrome: clinical and radiographic evaluation.Neuroimaging Clin N Am. May 2008;18(2):369-85, xi
  2. Walton KA, Buono LM. Horner syndrome. Curr Opin Ophthalmol 2003; 14:357.
  3. Havelius UA. Horner-like syndrome and cluster headache. What comes first? Acta Ophthalmol Scand 2001; 79:374.
  4. Woodruff G, Buncic JR, Morin JD.Horner's syndrome in children. J Pediatr Ophthalmol Strabismus 1988; 25:40.
  5. Allen AY, Meyer DR. Neck procedures resulting in Horner syndrome. Ophthal Plast Reconstr Surg. Jan-Feb 2009;25(1):16-8.
  6. George ND, Gonzalez G, Hoyt CS. Does Horner's syndrome in infancy require investigation? Br J Ophthalmol 1998; 82:51.
  7. Almog Y, Gepstein R, Kesler A. Diagnostic value of imaging in horner syndrome in adults. J Neuroophthalmol. Mar 2010;30(1):7-11.
  8. Davies RP, Slavotinek JP, Dorney SF. VIP secreting tumours in infancy. A review of radiological appearances. Pediatr Radiol 1990; 20:504.
  9. Watts P, Satterfield D, Lim MK. Adverse effects of apraclonidine used in the diagnosis of Horner syndrome in infants. J AAPOS. Jun 2007;11(3):282-3.
  10. Selim M, Caplan LR. Carotid Artery Dissection.Curr Treat Options Cardiovasc Med 2004; 6:249.

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Last updated: 2018-06-22 07:58