Horner's syndrome, also known as oculosympathetic paresis, is a combination of symptoms that arises when a group of nerves known as the sympathetic trunk is damaged. The signs and symptoms occur on the same side (ipsilateral) as it is a lesion of the sympathetic trunk. It is characterized by miosis (a constricted pupil), partial ptosis (a weak, droopy eyelid), apparent anhidrosis (decreased sweating), with apparent enophthalmos (inset eyeball). The nerves of the sympathetic trunk arise from the spinal cord in the chest, and from there ascend to the neck and face. The nerves are part of the sympathetic nervous system, a division of the autonomic (or involuntary) nervous system. Once the syndrome has been recognized, medical imaging and response to particular eye drops may be required to identify the location of the problem and the underlying cause.
Signs and symptoms Signs that are found in people with Horner's syndrome on the affected side of the face include the following:
Ptosis Anhidrosis: inability to sweat in a normal way. Miosis Enophthalmos Inability to completely close or open the eyelid Headaches Loss of ciliospinal reflex: the pupils do not get bigger when the neck or face is hurt or pinched. Bloodshot conjunctiva: The white part of the eye (the conjunctiva) looks red. How red it is can change depending on where the problem or injury is in the eye. Unilateral straight hair: In congenital Horner's syndrome, the hair on the affected side of the head may be straight, whereas on the contralateral side, it is curly. Heterochromia iridum Damage to the sympathetic nerves can cause several problems. The dilator muscle, which normally makes the pupil bigger, stops working. This makes the pupil smaller (miosis). The superior tarsal muscle is in the upper eyelid and helps lift it. It stops working so the upper eyelid droops (ptosis). Sweating on the face is reduced. The eye may look sunken (enophthalmos), but this is usually just because of the drooping eyelid. True sunken eyes are rare in humans but can be seen in cats, rats, and dogs with Horner's syndrome. The reaction of the pupils to light is normal because it does not depend on sympathetic nerve supply. Heterochromia can happen in children under two years old. If the sympathetic nerves do not send signals, the iris cannot make its natural color (melanin). This can make the two eyes different colors. In animals, Horner's syndrome can make the third eyelid, a small extra eyelid, cover part of the eye. This eyelid usually helps protect and moisten the eye.
Causes
Horner's syndrome is usually an acquired medical condition that results from a disease, but may also be congenital (inborn, associated with heterochromatic iris, birth trauma or injury to the nerves or carotid artery during delivery, lack of development (agenesis) of the carotid artery) or iatrogenic (caused by medical treatment or medical procedures). In rare cases, Horner's syndrome may be caused by repeated, minor head trauma, such as being hit with a soccer ball. Although most causes are relatively benign, Horner's syndrome may reflect serious disease in the neck or chest (such as a Pancoast tumor or thyrocervical venous dilatation). Causes can be divided according to the presence and location of anhidrosis (inability to sweat):
Central (anhidrosis of face, arm and trunk) Syringomyelia Multiple sclerosis Encephalitis Brain tumors: Brainstem tumors (such as gliomas) Hypothalamic tumors Pituitary tumors (rare, if they compress nearby structures) Lateral medullary syndrome Preganglionic (anhidrosis of face) Cervical rib traction on stellate ganglion Thyroid carcinoma Thyroidectomy – surgical removal of all or part of the thyroid gland Goiter – swelling in the neck due to an enlarged thyroid gland Bronchogenic carcinoma of the superior fissure (Pancoast tumor) on apex of lung Klumpke paralysis Trauma – base of neck, usually blunt trauma or trauma due to surgery (e.g., tube thoracostomy) Thoracic aortic aneurysm Postganglionic (no anhidrosis) Cluster headache – combination termed Horton's headache Horner's syndrome may occur temporarily during a migraine attack and be relieved afterwards Cavernous sinus thrombosis Middle ear infection Sympathectomy Nerve blocks, such as cervical plexus block, stellate ganglion or interscalene block
Pathophysiology Horner's syndrome results from any disruption of the sympathetic innervation of the eye. The site of the lesion in the sympathetic outflow is on the ipsilateral side of the symptoms. The sympathetic outflow from the brain to the eye and face consists of three routes, as follows:
First-order neuron (central neuron): Originates in the posterolateral side of the hypothalamus. Runs through the brainstem. Exits at the levels of C8, T1, and T2 of spinal segments, where it synapses with the second-order neuron. e.g., Central lesions affecting the Hypothalamospinal tract, like the transection of cervical spinal cord. Second-order neuron: Originates at the C8, T1, and T2 spinal segments Passes over the apex of the lung. Ascends through the cervical sympathetic chain. Ends in the superior cervical ganglion, where it synapses with the third-order neuron, i.e., preganglionic lesions that cause compression of the sympathetic chain, like a lung tumor. Third-order neuron: Starts at the superior cervical ganglion. Travels with the internal carotid artery. Reaches the cavernous sinus to attach to the abducens nerve (CN VI). Travels with the ophthalmic division of the trigeminal nerve (CN V1). Joins the nasociliary nerve to reach the ciliary ganglion. Ends with the long and short ciliary nerves. Supplies the pupillary dilator muscle, Müller's muscle, and lacrimal glands, i.e., postganglionic lesions that occur at the level of the internal carotid artery, like carotid artery dissection. Lesions in the first- and second-order neurons cause the classic Horner's syndrome. Other symptoms in these cases relate to the site of the lesion. In contrast, those of the third-order neuron may not show anhidrosis. Lesions distal to the carotid bifurcation do not cause anhidrosis. This happens because the facial sweat nerves travel with the external carotid artery. In such lesions, only the area above the brow shows anhidrosis. This results in partial Horner's syndrome.
Diagnosis
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