|Year : 2014 | Volume
| Issue : 2 | Page : 174-176
Acquired Horner's syndrome in an infant: A case report
Sumana J Kamath1, Madhurima A Nayak1, Flora D Lobo2
1 Department of Ophthalmology, Kasturba Medical College, Mangalore, Karnataka, India
2 Department of Pathology, Kasturba Medical College, Mangalore, Karnataka, India
|Date of Web Publication||1-Jul-2014|
Madhurima A Nayak
C II 12, KMC Staff Quarters, Light House Hill Road, Mangalore - 575 003, Karnataka
Source of Support: None, Conflict of Interest: None
Horner's syndrome has varied etiology. We report a case of acquired Horner's syndrome in an infant. A 3-month-old female child was referred for drooping of right eye upper lid on the second postoperative day following neck surgery. On examination, she had mild ptosis of right eye; with enophthalmos and miosis. A clinical diagnosis of Horner's syndrome was made. One percent phenylephrine test was done which dilated right pupil, suggestive of a postganglionic lesion. The sample was biopsied and revealed an infected lymphatic cyst. Horner's syndrome is a collection of signs due to interruption of sympathetic innervation to eye and face, characterized by miosis, mild ptosis, enophthalmos, and anhydrosis. The sympathetic pathway is a three neuron pathway. The present lesion is in the third order fibers which form plexus around the internal carotid artery. Dissection along the carotid can be an important cause for interruption of the sympathetic pathway.
Keywords: Infant, neck surgery, postganglionic Horner′s syndrome
|How to cite this article:|
Kamath SJ, Nayak MA, Lobo FD. Acquired Horner's syndrome in an infant: A case report. Muller J Med Sci Res 2014;5:174-6
| Introduction|| |
Horner's syndrome is a clinical diagnosis with varied etiology. The signs are mild ptosis, enophthalmos, miosis, anhydrosis, and loss of ciliospinal reflex. When it is congenital, ipsilateral heterochromic iris also accompanies. First described by Friedrich Horner in 1869,  this syndrome occurs due to the interruption of the sympathetic supply to the face. Although the signs and symptoms are subtle, Horner's syndrome may be the only manifestation of ominous conditions. We present a case of acquired Horner's syndrome in an infant following neck surgery.
| Case Report|| |
A 3-month-old female child was referred to us for drooping of the right upper lid noticed on the second postoperative day, following excision of a cyst in the neck [Figure 1]. She had presented to the department of Pediatrics with difficulty in feeding and hoarse cry since the age of 20 days. On examination, she had stridor and lower chest retraction. She was advised computed tomography (CT) of the neck which showed features suggestive of a branchial cyst [Figure 2]. The child was operated for the same and a cyst-like mass was found extending from just above the clavicle to the base of the skull, in close proximity to the great vessels [Figure 3]. On examination, child was fixing and following light. Gaze was orthophoric. Palpebral aperture was 7 mm (OD) and 9 mm (OS). Extraocular movements were full and normal. Cornea and anterior chamber were normal. Pupil in the right eye was 2 mm, reacting to direct and consensual light. Pupil in the left eye was 4 mm, reacting to direct and consensual light [Figure 4]. Fundus was normal in both eyes. One percent phenylephrine eyedrops were instilled in both eyes, and examined after 15 minutes. The right pupil was 5 mm while left remained 4 mm. A clinical diagnosis of postganglionic Horner's syndrome was made. The biopsy of the excised cyst revealed acute inflammatory exudate with granulation tissue with polymorphous population of small and large lymphoid cells suggestive of an infected lymphatic cyst [Figure 5].
|Figure 5: Histopathology of the cyst wall showing lymphocytic aggregation|
Click here to view
| Discussion|| |
The sympathetic pathway begins in the posterior hypothalamus and descends along the brainstem, uncrossed and relays in the ciliospinal center of Budge at the level of C8-T1. These are the first order neurons. Lesions affecting this pathway lead to central Horner's syndrome. The causes are posterior inferior cerebellar artery (PICA) occlusion,  syringomyelia,  and demyelinating disorders.  The second order neurons (preganglionic fibers) begin in the ciliospinal center and terminate in the superior cervical ganglion. They are closely associated with the superior apex of pleura. Hence, superior sulcus tumor of lung can present with Horner's syndrome and accompanied by shoulder pain and arm pain  and are seen in 14-50% of cases.  Other causes are aortic aneurysms, birth trauma in the neck, and lesions in the neck. The postganglionic fibers from the superior cervical ganglion travel along the internal carotid artery to enter the skull where they run along the ophthalmic division of the trigeminal nerve. Ultimately, they reach the ciliary body, dilator pupillae, and Muller muscle. Carotid artery dissection, benign vascular headache affecting the internal carotid artery, cavernous sinus lesions, and orbital apex lesions can lead to postganglionic Horner's syndrome. The present case is a postganglionic lesion due to dissection of the cyst along the carotids. The procedures which can be associated with Horner's syndrome are coronary artery bypass surgery with neck catheter, carotid endarterectomy, cervical and thoracic sympathectomy, parathyroid surgery, and cervical spine fusion surgery. 
Horner's syndrome in the pediatric age group can be congenital, due to birth trauma to the neck, rarely agenesis of the internal carotid artery,  and neuroblastomas. 
Horner's syndrome is a clinical diagnosis and is only confirmed by pharmacological agents. In the test, we used 1% phenylephrine eyedrops. One drop was instilled in both eyes and pupils were examined after 15 minutes. The affected pupil dilated to 5 mm due to denervation supersensitivity and thus proved postganglionic affection of the sympathetic system. Four percent cocaine is used to confirm Horner's syndrome, though not commonly used. All pupils affected by oculosympathetic paresis do not dilate with cocaine, whereas normal pupils dilate. This test does not help in localizing the lesion. Ten percent hydroxyamphetamine drops are used to localize the lesion. In a patient with preganglionic lesion, pupils will dilate on instillation of hydroxyamphetamine drops, whereas pupil in postganglionic Horner's syndrome, will not.
| Conclusion|| |
Horner's syndrome is a clinical entity, caused by an array of lesions. Neck surgeries and dissection along the carotids is an important cause. Pharmacologic testing aids in localizing the site of the lesion.
| References|| |
|1.||Horner JF. Übereine Form von Ptosis. Klin Monatsbl Augenheilk 1869;7:193-8. |
|2.||Sacco RL, Freddo L, Bello JA, Odel JG, Onesti ST, Mohr JP. Wallenberg′s lateral medullary syndrome. Clinical-magnetic resonance imaging correlations. Arch Neurol 1993;50:609-14. |
|3.||Kerrison JB, Biousse V, Newman NJ. Isolated Horner′s syndrome and syringomyelia. J Neurol Neurosurg Psychiatry 2000;69:131-2. |
|4.||Pomeranz H. Isolated Horner syndrome and syrinx of the cervical spinal cord. Am J Ophthalmol 2002;133:702-4. |
|5.||Kong XY, Wright G, Pesudovs K, O′Day J, Wainer Z, Weisinge HS. Horner syndrome. Clin Exp Optom 2007;90:336-44. |
|6.||Arcasoy SM, Jett JR. Superior pulmonary sulcus tumors and Pancoast′s syndrome. N Engl J Med 1997;337:1370-6. |
|7.||Allen AY, Meyer DR. Neck procedures resulting in Horner syndrome. Ophthal Plast Reconstr Surg 2009;25:16-8. |
|8.||Ryan FH, Kline LB, Gomez C. Congenital Horner′s syndrome resulting from agenesis of the internal carotid artery. Ophthalmology 2000;107:185-8. |
|9.||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 2006;142:651-9. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]