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   Table of Contents      
BRIEF REPORT
Year : 2006  |  Volume : 54  |  Issue : 3  |  Page : 197-199

Congenital Horner's syndrome and the usefulness of the apraclonidine test in its diagnosis


1 Department of Neurosurgery, Celal Bayar University School of Medicine, Manisa, Turkey
2 Department of Ophthalmology, Celal Bayar University School of Medicine, Manisa, Turkey

Correspondence Address:
Hasan Mirzai
2040 Sok. Pamukkale 4/60 D:67, Mavisehir, Atakent, 35540, Izmir
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0301-4738.27073

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  Abstract 

We present a seven-month-old baby with miosis of the left pupil, left hypochromia, mild ipsilateral ptosis, left hemifacial anhidrosis and asymmetrical facial flushing. A diagnosis of Horner's syndrome (HS) was presumed and was confirmed by instillation of apraclonidine eye drops. Miosis was reversed upon apraclonidine instillation. Magnetic resonance imaging of the head, neck and thorax and ultrasonography of the neck and abdomen did not reveal any pathological conditions. Although delivery-related brachial plexus injury is known as the most common cause of congenital HS, it should be investigated and should include neuroimaging of the sympathetic pathway, to exclude a serious underlying disease. As in our case, a specific etiology may not always be elicited. Pharmacological testing with apraclonidine may be a practical alternative to cocaine in the diagnosis of HS.

Keywords: Anhidrosis, anisocoria, apraclonidine, heterochromia, Horner′s syndrome, miosis, ptosis


How to cite this article:
Mirzai H, Baser EF. Congenital Horner's syndrome and the usefulness of the apraclonidine test in its diagnosis. Indian J Ophthalmol 2006;54:197-9

How to cite this URL:
Mirzai H, Baser EF. Congenital Horner's syndrome and the usefulness of the apraclonidine test in its diagnosis. Indian J Ophthalmol [serial online] 2006 [cited 2023 Dec 9];54:197-9. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?2006/54/3/197/27073

Horner's syndrome (HS) or oculosympathoparesis classically presents with ipsilateral ptosis, miosis of the pupil and facial anhidrosis. The syndrome results from disruption of the sympathetic innervation to the eye and its adnexa. Congenital HS is most commonly idiopathic or due to birth trauma.[1] The pathological processes responsible for acquired HS, range from simple migraines, to life-threatening malignancy.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10] Therefore appropriate evaluation and timely diagnosis may be life-saving.


  Case Report Top


A seven-month-old baby presented with hypochromia of the left iris, ipsilateral mild ptosis and left hemifacial anhidrosis noticed by her parents since birth [Figure - 1]. The patient was born after a full term normal pregnancy by Cesarean section and was released from the nursery with a reported normal examination. She had no history of any surgical interventions.

Examination of the baby showed mild ptosis of the left upper eyelid and elevation of the left lower eyelid, causing a narrowing of her interpalpebral fissure. She had subtle anisocoria of approximately 2 mm, with left pupillary miosis. The amount of anisocoria was greater (3 mm) in dim illumination and lesser in bright illumination (1 mm).

The color of right iris was brown and the left iris was greenish (iris heterochromia). Eyes were orthophoric and had normal ophthalmoscopic findings. The baby was noticed to have hemifacial anhidrosis of the entire left side of the face.

A diagnosis of HS was presumed and was confirmed by instillation of topical 0.5% apraclonidine eye drops (Iopidine; Alcon, Fort Worth, Texas.[3],[1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14] Topical cocaine and hydroxyamphetamine are not available in our country. First, the baseline pupil diameters were recorded in both dark and light ambient illumination, measured to the nearest 0.5 mm. Then one drop of apraclonidine was instilled into each eye. After 60 minutes, the right pupil had not changed at all, while the left pupil dilated by about 2 mm. In addition, the left upper eye lid was elevated, eliminating the ptosis [Figure - 2].

Magnetic resonance imaging (MRI) of the head, neck and thoracic regions were completely normal. Ultrasonography of the neck and abdomen did not show any pathological conditions. Physical examination of the baby by a pediatrician was reported as normal.


  Discussion Top


Congenital HS is an unusual, but important presentation. It has been estimated that less than 5% of HS can be classified as congenital.[15] In a pediatric population with HS, Jeffery et al .[1] reported that 42% were congenital, 15% were acquired without surgical intervention and 42% were acquired after a surgical procedure of the thorax, neck or central nervous system. In another pediatric population, Woodfruff et al .[2] reported neuroblastoma, previous cardiothoracic surgery and major congenital abnormalities for half of their patients, whereas no cause could be identified in the other half. We could not elicit any history of birth trauma in our patient. As a result of history, clinical examination, pharmacological testing and neuroimaging, we assumed that HS in this baby was congenital.

Sympathetic innervation to the eye and ocular adnexae follows a three-neuron pathway. The first-order neurons start from the hypothalamus and terminate in the ciliospinal center of Budge, while the second-order neurons synapse in the superior cervical ganglion. The third-order neurons ascend along the internal carotid artery to enter the skull and join the ophthalmic division of the trigeminal nerve in the cavernous sinus. The sympathetic fibers reach the ciliary body and the dilatator pupillae muscle via the nasociliary nerve.[3]

Delivery-related brachial plexus injury is known as the most common cause of HS. However, any lesion along the sympathetic chain may result in HS. Of considerable importance is primary thoracic and cervical neuroblastoma, where timely diagnosis is crucial.[1],[9],[10] Other reported causes of congenital HS are agenesis of the internal carotid artery,[4] complication of various surgical procedures involving the thorax and the neck,[7],[8] carotid artery aneurysm[6] and traumatic carotid dissection.[6]

Confirmation of HS is typically made by instilling cocaine to both eyes. Cocaine blocks the reuptake of nor-epinephrine from the synaptic junctions. The normal pupillary response to topical cocaine is dilatation. After confirmation of HS with cocaine, hydroxyamphetamine can be helpful to distinguish central and preganglionic sympathetic lesions from postganglionic lesions. Hydroxyamphetamine releases norepinephrine from the nerve endings, producing pupillary mydriasis in preganglionic lesions.

An interesting alternative to cocaine testing is the use of apraclonidine, an a 2 receptor agonist, primarily used as an intraocular pressure lowering agent. Apraclonidine also has some weak a 1 affinity. Therefore, reversal of anisocoria in HS can be due to denervation hypersensitivity of postsynaptic a 1 receptors in the pupillary dilator muscle.[11],[12],[13],[14],[15] Patients with a greater degree of anisocoria, generally show a greater degree of reversal of anisocoria after apraclonidine, which may reflect a greater or more long-standing degree of denervation. However, a false-negative result may occur in very recently acquired HS, because the a 1 receptors have not yet up-regulated.[11] Apraclonidine may elevate the ptotic eyelid, which merely reflects the typical effect of most topical sympathomimetic drugs.[16]

Since it is not possible to localize the sympathetic lesion by using apraclonidine, we had to predict the site of the lesion by assessment of other features. Our patient had very definite reduced ipsilateral sweating of the entire left side of the face, which implied a preganglionic lesion.

Iris heterochromia occasionally accompanies HS, particularly in congenital lesions.[3] In brown eyed patients, the eye with the lighter eye color corresponds to the abnormal pupil. The cause of iris heterochromia seen in congenital HS may also be related to the fact that sympathetic ganglion cells, as well as iris melanocytes are derivatives of neural crest cells.

In conclusion, although delivery-related brachial plexus injury is known as the most common cause of congenital HS, imaging of the entire sympathetic pathway is warranted, since life-threatening malignancy and abnormalities may be revealed. Pharmacological testing with apraclonidine may be a practical alternative to cocaine in the diagnosis of HS.

 
  References Top

1.
Jeffery AR, Ellis FJ, Repka MX, Buncic JR. Pediatric Horner Syndrome. J AAPOS 1998;2:159-67.  Back to cited text no. 1
[PUBMED]    
2.
Woodruff G, Buncic JR, Morin JD. Horner's syndrome in children. J Pediatr Ophthalmol Strabismus 1988;25:40-4.   Back to cited text no. 2
    
3.
Walton KA, Lawrence MB. Horner syndrome. Curr Opin Ophthalmol 2003;14:357-63.  Back to cited text no. 3
    
4.
Ryan FH, Kline LB, Gomez C. Congenital Horner's syndrome resulting from agenesis of the internal carotid. Ophthalmology 2000;107:185-8.  Back to cited text no. 4
    
5.
Liu JK, Gottfried ON, Amini A, Couldwell WT. Aneurysm of the petrous internal carotid artery: anatomy origins and treatment. Neurosurg Focus 2004;17:E13.  Back to cited text no. 5
    
6.
Robertson WC, Pettigrew LC. Congenital Horner's syndrome and carotis dissection. J Neuroimaging 2003;13:367-70.  Back to cited text no. 6
    
7.
Naimer SA, Weinstein O, Rosenthal G. Congenital Horner Syndrome: A rare though significant complication of subclavian aortoplasty. J Thorac Cardiovasc Surg 2000;120:419-21.  Back to cited text no. 7
    
8.
Rosegger H, Fritsch G. Horner's syndrome after treatment of tension pneumothorax with tube thoracostomy in a newborn infant. Eur J Pediatr 1980;133:67-8.  Back to cited text no. 8
    
9.
Cardesa-Salzmann Tmi Mora-Graupera J, Claret G, Agut T. Congenital cervical neuroblastoma. Pediatr Blood Cancer 2004;43:785-7.   Back to cited text no. 9
    
10.
Beckerman BL, Seaver R. Congenital Horner's syndrome and thoracic neuroblastoma. J Pediatr Ophthalmol Strabismus 1978;15:24-5.  Back to cited text no. 10
    
11.
Freedman KA, Brown S. Topical apraclonidine in the diagnosis of suspected Horner Syndrome. J Neuro Ophthalmol 2005;25:83-5.  Back to cited text no. 11
    
12.
Morales J, Brown SM, Abdul-Rahim AS, Crosson CE. Ocular effects of apraclonidine in Horner syndrome. Arch Ophthalmol 2000;118:951-4.  Back to cited text no. 12
    
13.
Brown SM, Aouchiche R, Freedman KA. The utility of 0.5% apraclonidine in the diagnosis of Horner syndrome. Arch Ophthalmol 2003;121:1201-3.  Back to cited text no. 13
    
14.
Bacal DA, Levy SR. The use of apraclonidine in the diagnosis of Horner syndrome in pediatric patients. Arch Ophthalmol 2004;122:276-9.  Back to cited text no. 14
    
15.
Weinstein JM, Zweifel TJ, Thompson HS. Congenital Horner's syndrome. Arch Ophthalmol 1980;98:1074-8.   Back to cited text no. 15
    
16.
Munden PM, Kadron RH, Denison CE, Carter KD. Palpebral fissure responses to topical adrenergic drugs. Am J Ophthalmol 1991;111:706-10.  Back to cited text no. 16
    


    Figures

  [Figure - 1], [Figure - 2]


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