History

Fact Explanation
Hemisensory loss Occurs when there is a lesion in the brainstem involving the sympathetic and cortico spinal nerve pathways, resulting in Horner syndrome and contralateral motor/sensory signs[1]
Dysarthria Can occur in first order neuron Horner syndrome(HS), due to lateral medullary syndrome that occurs as a result of posterior inferior cerebellar artery occlusion. In this condition there is involvement of the first order sympathetic neuron pathway, spinothalamic tract, trigeminal nerve tract, tenth cranial nerve and cerebellar pathways. Dysarthria results from damage to vestibulo-floccular connections. Damage to trigeminal nerve tract can produce ipsilateral sensory loss of face and damage to spinothalamic tract can cause contralateral sensory loss of limbs and trunk[3][4]
Dysphagia Can occur in first order neuron HS which is a component of lateral medullary syndrome as described above[3][4]
Ataxia If occurs together with HS, is suggestive of lateral medullar syndrome[3][4]
Vertigo Occurs with HS when the disruption to nerve pathway is at the medulla, likely to be due to lateral medullary syndrome involving both sympathetic nerve pathways and vestibulo-floccular connections[1][3][4]
Axillary, shoulder or arm pain Occurs when the nerve disruption is at the C8-T2 ventral nerve root level[1]
Neck, facial, and head pain If a patient presents with Horner syndrome associated with ipsilateral orbital, face, or neck pain of acute onset, internal carotid artery dissection should always be excluded until proven otherwise. Pain occurs because of ischemia or stretching of the trigeminal pain fibers surrounding the carotid arteries. [1][2]
History of central venous catheter placement Invasive procedures involving neck region could cause damage to the cervical sympathetic chain[1]
History of chest tube insertion Could have caused damage to the sympathetic nerve pathways in the region of insertion[1]
previous thoracic or neck surgery Could have caused accidental damage to the sympathetic nerve pathways[1]
Hemoptysis and symptoms of lung disease These would suggest the presence of a lung cancer particularly an apical Pancoast tumour which can spread to the cervical sympathetic chain[1]
History of trauma Trauma involving head and neck could have caused damage to the sympathetic nerve pathways[1]
Diplopia Occurs when there is a lesion in the cavernous sinus involving the sympathetic nerves and also causing abducens nerve palsy[1] It can also occur in anterior inferior cerebellar artery occlusion but then in addition to ipsilateral HS and lateral gaze palsy, it also causes ipsilateral sensory loss and weakness of face and contralateral sensory loss of limbs and trunk[4]
Numbness in the distribution of the first or second division of the trigeminal nerve (cranial nerve [CN] V), and pain Occurs when the disruption to sympathetic nerve pathway occurs at the brainstem[1]
Nystagmus Also occurs due to damage to vestibulo-floccular connections that occurs in lateral medullary syndrome[3][4]
References
  1. DAVAGNANAM I, FRASER CL, MISZKIEL K, DANIEL CS, PLANT GT. Adult Horner's syndrome: a combined clinical, pharmacological, and imaging algorithm Eye (Lond) [online] 2013 Mar, 27(3):291-298 [viewed 26 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3597883/
  2. NAUTIYAL A, SINGH S, DISALLE M, O'SULLIVAN J. Painful Horner Syndrome as a Harbinger of Silent Carotid Dissection PLoS Med [online] 2005 Jan, 2(1):e19 [viewed 27 May 2014] Available from: doi:10.1371/journal.pmed.0020019
  3. JOHNSON D, SHARMA S. Answer: Can you identify this condition? Can Fam Physician [online] 2010 May, 56(5):443 [viewed 28 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2868616
  4. LINDSAY KW, BONE I, CALLANDER R, FULLER G, Neurology and neurosurgery illustrated. Fifth edition. Churchill Livingstone. 2011

Examination

Fact Explanation
Ptosis/Partial ptosis Is considered a classical sign of Horner syndrome that occur secondary to interruption of the sympathetic pathway that supplies the superior tarsal muscle of the upper eye lid[1][3][4][5]
Fixed constricted pupil not reacting to light (miosis) This is also a classical sign of Horner syndrome. It results from the unopposed activity of the pupillary constrictors supplied by the parasympathetic nerves, because the pupillary dilators supplied by the sympathetic nerves is not active[1][3][4][5]
Dry skin (anhidrosis) One of the classic triad symptoms of Horner syndrome that occurs because of the disrupted sympathetic supply to the sweat glands on ipsilateral side of face. Occurs when the lesion is proximal to the fiber separation at the carotid arteries [1][3][4][5]
Anisocoria Anisocoria (unequal size of the pupils) is more prominent in the dark, may be most obvious within the first 5 s of darkness when the iris dilators are supposed to be acting. After 10–15 s in the dark, this becomes less apparent (known as dilation lag)[1][3]
Enophthalmos The lower eye lid also has a muscle innervated by sympathetic nerves. When there is disruption to sympathetic nerve supply, the lower lid may be elevated, with overall palpebral aperture reduction making the globe appear sunken in[1][2]
Red conjunctivae Conjunctival hyperemia can occur in acute Horner syndrome[1][4]
Asymmetric facial flushing Is sometimes seen with congenital Horner syndrome[4]
Iris heterochromia Is associated with congenital Horner syndrome[4]
References
  1. DAVAGNANAM I, FRASER CL, MISZKIEL K, DANIEL CS, PLANT GT. Adult Horner's syndrome: a combined clinical, pharmacological, and imaging algorithm Eye (Lond) [online] 2013 Mar, 27(3):291-298 [viewed 26 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3597883/
  2. DAROFF R. Enophthalmos Is Not Present in Horner Syndrome PLoS Med [online] 2005 Apr, 2(4):e120 [viewed 27 May 2014] Available from: doi:10.1371/journal.pmed.0020120
  3. NAUTIYAL A, SINGH S, DISALLE M, O'SULLIVAN J. Painful Horner Syndrome as a Harbinger of Silent Carotid Dissection PLoS Med [online] 2005 Jan, 2(1):e19 [viewed 27 May 2014] Available from: doi:10.1371/journal.pmed.0020019
  4. JOHNSON D, SHARMA S. Answer: Can you identify this condition? Can Fam Physician [online] 2010 May, 56(5):443 [viewed 27 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2868616
  5. LINDSAY KW, BONE I, CALLANDER R, FULLER G, Neurology and neurosurgery illustrated. Fifth edition. Churchill Livingstone. 2011

Differential Diagnoses

Fact Explanation
Pancoast tumor This is an apical lung tumor that can invade the cervical sympathetic plexus and give rise to Horner syndrome [7][8]
Internal carotid artery (ICA) dissection Should be considered the diagnosis until proven otherwise, when the patient presents with acute ipsilateral pain in the orbit, face or neck. HS in ICA dissection results from stretching and ischaemia of the sympathetic fibres surrounding the internal carotid artery.[1][2][5][7]
Cluster headaches Can present with unilateral acute severe pain around eye, ipsilateral conjunctival redness, miosis and ptosis which arouses the suspicion of HS following ICA dissection[8]
Neuroblastoma Is an impotant differential diagnosis of HS in the paediatric population[3][4]
Holmes-Adie pupil (contralateral) This is a benign condition which presents with a unilateral dilated pupil, which can lead to the misdiagnosis of miosis in the contralateral pupil[8]
Argyll Robertson pupil Presents with a constricted pupil which is unreactive to light similar to HS. Seen mainly in patients with neurosyphilis[8]
Sympathetic schwannomas This is a second order neuron lesion which produces the classic triad of ptosis, miosis, and ipsilateral anhidrosis of the face and neck[7]
Unilateral use of miotic drugs Use of a topical mitotic drug can induce pupillary constriction[9]
Jugular vein thrombosis Can present with Horner syndrome[6]
Wallenberg syndrome Lesions involving the lateral medulla gives rise to Wallenberg syndrome. This will be associated with symptoms such as problems with swallowing, hoarseness, dizziness, nausea and vomiting, nystagmus, problems with balance, gait, coordination etc.in addition to miosis and ptosis[7]
References
  1. DAVAGNANAM I, FRASER CL, MISZKIEL K, DANIEL CS, PLANT GT. Adult Horner's syndrome: a combined clinical, pharmacological, and imaging algorithm Eye (Lond) [online] 2013 Mar, 27(3):291-298 [viewed 26 May 2014] Available from: doi:10.1038/eye.2012.281
  2. ARDHALAPUDI S, ADDY V, DA COSTA D. Spontaneous bilateral internal carotid artery dissection BMJ Case Rep [online] :bcr07.2009.2125 [viewed 26 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC30291
  3. SMITH SJ, DIEHL N, LEAVITT JA, MOHNEY BG. Incidence of Pediatric Horner Syndrome and the Risk of Neuroblastoma: A Population-Based Study Arch Ophthalmol [online] 2010 Mar, 128(3):324-329 [viewed 27 May 2014] Available from: doi:10.1001/archophthalmol.2010.6
  4. NICHOLAS R. MAHONEY NR, LIU GT,MENACKER SJ, WILSON MC, HOGARTY MD,MARIS JM. Pediatric Horner Syndrome: Etiologies and Roles of Imaging and Urine Studies to Detect Neuroblastoma and Other Responsible Mass Lesions.Am J Ophthalmol [online] 2006;142:651–659.[viewed on 26 May 2014] Available from; http://www.rima.org/web/medline_pdf/AmJOphthalmol2006_651_9.pdf
  5. NAUTIYAL A, SINGH S, DISALLE M, O'SULLIVAN J. Painful Horner Syndrome as a Harbinger of Silent Carotid Dissection PLoS Med [online] 2005 Jan, 2(1):e19 [viewed 27 May 2014] Available from: doi:10.1371/journal.pmed.0020019
  6. HABEK M, PETRAVIć D, OZRETIć D, BRINAR VV. Horner syndrome due to jugular vein thrombosis (Lemierre syndrome) BMJ Case Rep [online] :bcr2007124479 [viewed 27 May 2014] Available from: doi:10.1136/bcr.2007.124479
  7. JOHNSON D, SHARMA S. Answer: Can you identify this condition? Can Fam Physician [online] 2010 May, 56(5):443 [viewed 27 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2868616
  8. LONGMORE M, AUTORES V, WILKINSON IB, DAVIDSON EH, FOULKES A, MAFI AR. Oxford Handbook of Clinical Medicine. Eighth edition. Oxford University Press; 2012.
  9. BENNET P, BROWN M, SHARMA P. Clinical Pharmacology. Eleventh edition. Churchill Livingstone; 2012

Investigations - for Diagnosis

Fact Explanation
Chest radiograph Done to exclude mediastinal masses and apical lung tumors when such is suspected[1][6]
Cocaine test Can be used to confirm diagnosis of HS, but cannot localize the lesion[1][6]
Apraclonidine test Can be used fro confirming clinical diagnosis of HS but cannot localize the site of lesion[1]
Computed tomography(CT) Done to assess the head, neck and thoracic structures in order to localize the lesion[1]
Magnetic resonance imaging (MRI) Also done to localize the lesion. Gives a better contrast resolution than CT [1][4][5]
Magnetic resonance angiography (MRA) Done to assess the the arterial system of head, neck and thorax[1][4]
Random urine studies for vanillylmandelic acid [VMA] and homovanillic acid [HVA] Done in children with HS to exclude neuroblastoma[2][3]
Color Doppler of neck vasculature Done to see the blood flow through neck vessels[5]
References
  1. DAVAGNANAM I, FRASER CL, MISZKIEL K, DANIEL CS, PLANT GT. Adult Horner's syndrome: a combined clinical, pharmacological, and imaging algorithm Eye (Lond) [online] 2013 Mar, 27(3):291-298 [viewed 26 May 2014] Available from: doi:10.1038/eye.2012.281
  2. NICHOLAS R. MAHONEY NR, LIU GT,MENACKER SJ, WILSON MC, HOGARTY MD,MARIS JM. Pediatric Horner Syndrome: Etiologies and Roles of Imaging and Urine Studies to Detect Neuroblastoma and Other Responsible Mass Lesions.Am J Ophthalmol [online] 2006;142:651–659.[viewed on 26 May 2014] Available from; http://www.rima.org/web/medline_pdf/AmJOphthalmol2006_651_9.pdf
  3. SMITH SJ, DIEHL N, LEAVITT JA, MOHNEY BG. Incidence of Pediatric Horner Syndrome and the Risk of Neuroblastoma: A Population-Based Study Arch Ophthalmol [online] 2010 Mar, 128(3):324-329 [viewed 27 May 2014] Available from: doi:10.1001/archophthalmol.2010.6
  4. NAUTIYAL A, SINGH S, DISALLE M, O'SULLIVAN J. Painful Horner Syndrome as a Harbinger of Silent Carotid Dissection PLoS Med [online] 2005 Jan, 2(1):e19 [viewed 27 May 2014] Available from: doi:10.1371/journal.pmed.0020019
  5. HABEK M, PETRAVIć D, OZRETIć D, BRINAR VV. Horner syndrome due to jugular vein thrombosis (Lemierre syndrome) BMJ Case Rep [online] :bcr2007124479 [viewed 27 May 2014] Available from: doi:10.1136/bcr.2007.124479
  6. LINDSAY KW, BONE I, CALLANDER R, FULLER G, Neurology and neurosurgery illustrated. Fifth edition. Churchill Livingstone. 2011; pg 143

Management - Specific Treatments

Fact Explanation
Neurosurgical care Should be offered for those who have HS due to aneurysms etc that needs neurosurgical intervention[1]
Vascular surgical care Should be offered when there are vascular conditions causing HS which may require surgical intervention[1]
Oncology referal Is needed for patients presenting with HS due to pancoast tumor.These patients will be given the option of chemo-radiotherapy or surgery depending on the stage of the disease[3]
Anticoagulants Anticoagulant treatment is given for 3 to 6 months to those with carotid artery dissection to prevent thromboembolism[2]
References
  1. JOHNSON D, SHARMA S. Answer: Can you identify this condition? Can Fam Physician [online] 2010 May, 56(5):443 [viewed 27 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2868616
  2. NAUTIYAL A, SINGH S, DISALLE M, O'SULLIVAN J. Painful Horner Syndrome as a Harbinger of Silent Carotid Dissection PLoS Med [online] 2005 Jan, 2(1):e19 [viewed 27 May 2014] Available from: doi:10.1371/journal.pmed.0020019
  3. FOROULIS CN, ZAROGOULIDIS P, DARWICHE K, KATSIKOGIANNIS N, MACHAIRIOTIS N, KARAPANTZOS I, TSAKIRIDIS K, HUANG H, ZAROGOULIDIS K. Superior sulcus (Pancoast) tumors: current evidence on diagnosis and radical treatment. J Thorac Dis [online] 2013 Sep, 5(Suppl 4):S342-S358 [viewed 28 May 2014] Available from: doi:10.3978/j.issn.2072-1439.2013.04.08