Fourth Cranial Nerve Palsy The superior oblique muscle is innervated by the trochlear nerve and it has 3 functions.

These functions are dependant upon the gaze position that the eyes are in. The primary function of the superior oblique muscle is to depress the globe when the eye is adducted. (This is important for reading). The second function is incyclotorsion of the globe. (This helps to compensate for head tilting). The third function is abduction of the eye in down gaze. The antagonist to the superior oblique muscle is the inferior oblique muscle. This muscle has 3 functions which are also field of gaze dependant. The primary function of the inferior oblique is to elevate the globe when it is in an adducted position. The second function is excyclotorsion and the third function is to abduct the globe when the eyes are in an upgaze position. As the superior oblique muscle passes forward in the orbit, it makes a transition into a tendonous extension. This extension passes through the trochlea which is located in the anteriomedial orbital roof and then it fans out to insert laterally and posteriorally on the superior aspect of the globe. The inferior oblique muscle originates from the anterior inferiomedial orbital wall and inserts laterally and posteriorally on the inferiolateral aspect of the globe. Signs and Symptom Clinical presentation of a trochlear nerve palsy is caused by the relative imbalance of action of the superior oblique muscle and its direct antagonist muscle, the inferior oblique. The affected eye characteristically has a vertical and torsional position, and the elevation of the involved eye increases in adduction. Since the superior oblique is an abductor of the eye in down gaze, the patient may develop an esotropia when looking down. Thus, to preserve binocularity a patient will usually present with a head tilt away from the involved eye combined with a head turn, also away from the involved eye. The chin may also be held in a down position. The tilt and turn of the head places the affected eye in a position where the palsied superior oblique muscle and the overaction of the inferior oblique have minimal input. Holding the chin down helps to compensate for the esotropia in down gaze, (V-pattern esotropia). In infants, the compensatory head position will not be seen until the child has assumed an upright posture. Bilateral cases of trochlear nerve palsy have a hyper deviation of the right eye in left gaze and hyperdeviation of the left eye in right gaze. The head posture caused by a congential fourth nerve palsy can develop in late infancy or in adulthood. In later onset cases, the patient's fusional amplitudes, or his/her ability to control the deviation of the eyes, are thought to "decompensate" resulting in the onset of signs and symptoms. Young children usually don't complain of symptoms, although as the child matures and can verbalize, especially in later childhood or as an adult, they may have asthenopia (visual discomfort), reading difficulties, vertical diplopia or neck strain. Image tilting is not found in congenital fourth verve palsy; although it can be present in acquired adult cases. Rarely a patient with a decompensated trochlear nerve palsy will paradoxically tilt their head toward the involved eye in an effort to maximally separate the diplopic images for visual comfort. ETIOLOGY The two causes of trochlear nerve palsy are congenital and acquired. Acquired cases most commonly result from closed head trauma. The trauma may be slight such as a head bump or severe as seen in patients who are rendered unconscious. Occasionally the trauma may involve the orbit and the superior oblique muscle may be directly injured.

Patients with a congenital trochlear nerve palsy do not have associated pathology and these patients are usually not "worked up" with neuro-imaging. Infants and Children: In this age group the majority of fourth cranial palsies are congenital or observed to be present from birth. Affected children will usually develop a head posture. As previously stated, some of the delayed presentations are thought to be secondary to "decompensation" of a congenital palsy and/or a decrease in the patient's fusional amplitudes. The cause of most congenital fourth nerve palsies is unknown and these children are otherwise normal. Agenesis of the trochlear nerve nucleus has been described but never with an isolated fourth cranial nerve palsy. Acquired palsies most commonly occur as the result of open or closed head trauma. In theses injuries, the trochlear nerves are compressed against the tentorial edge or at other locations along their path. Bilateral cases can be due to disruption of the decussating fibers of the nerve in the anterior medullary velum. Encephalitis, neonatal hypoxia and hydrocephalus may also cause a fourth cranial nerve palsy. Posterior fossa tumors or a pinealoma may also produce a fourth nerve palsy but an isolated fourth cranial palsy is almost never the presenting sign. When a fourth cranial nerve palsy is observed with a tumor, it is usually accompanied by other obvious signs or other cranial nerve palsies. Adults: Trauma is the most common etiology for fourth nerve paralysis in adults. Vascular disease including hypertension, atherosclerosis, aneurysms, and diabetes mellitus are other less common causes. However, the cause of a fourth cranial nerve palsy in adults may be undetermined. In these cases, the palsy is usually classified as a decompensated congenital palsy. EVALUATION The patient is observed paying special attention to any abnormal head posturing. For example, with a left fourth nerve palsy, the patient may maintain a right head tilt to avoid diplopia. An infant or young child may resist attempts to straighten the head. Older children or adults may complain of vertical diplopia when the head is held upright. This diplopia may increase in right gaze or down gaze and improve in left gaze. Characteristically, hyper deviation will develop in the right eye when the head is held in the primary position. The vertical deviation of the eyes will increase with tilting the head to the left and decrease or can be absent with tilting the head to the right. These findings may be subtle and variable, especially when there is a history of previous strabismus surgery or if there are bilateral superior oblique (trochlear nerve) palsies. Occlusion or patching of the affected eye will improve or eliminate the head posture. The fundus may show excyclorotation. Normally, the fovea is located slightly above a horizontal line that passes through the optic nerve. In patients with a fourth cranial nerve palsy, the fovea is displaced downward in relationship to the optic nerve. (the fundus shows excycloratation) To differentiate head postures caused by fourth nerve paralysis from those caused by a muscular etiology such as torticollis, palpate the cervical muscles and rotate the head to the opposite side. A fourth cranial nerve palsy that is recent in its onset will not be associated with tight cervical muscles.

TREATMENT Patients with mild, congenital fourth nerve palsy may have good binocular function, and surgical treatment is recommended only if the head posture is unacceptable. However, if the head tilt is significant or there is pronounced overaction of the ipsilateral inferior oblique muscle, then eye muscle surgery can be performed to improve the head position and correct the ocular alignment. The primary goal of surgery is to eliminate the head posture and promote binocularity in the most important fields of gaze, that is, straight ahead and in down gaze or the reading position. Acquired cases, whether vascular or traumatic, are treated surgically only after a waiting period of 6 months has elapsed. This permits recovery of nerve function. SUMMARY Patients who have congenital or acquired trochlear nerve palsy can be treated with strabismus surgery, when appropriate, to eliminate or improve a head posture or eliminate or reduce symptoms of diplopia. Surgery is advised when the ocular deviation or strabismus is stable. Patients who have an acquired trochlear nerve palsy can be treated with surgery after a waiting period of at least 6 months from the time of insult. When surgery is performed, follow-up care at appropriate intervals is necessary to facilitate binocular function.