| Congenital fourth nerve palsy | |
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| Trochlear nerve | |
| Specialty | Neurology |
Congenital fourth nerve palsy is a condition present at birth characterized by a vertical misalignment of the eyes due to a weakness or paralysis of the superior oblique muscle.
Other names for fourth nerve palsy include superior oblique palsy and trochlear nerve palsy. [1] When looking to the right/left the nerve/muscle is not strong enough or is too long and the eye drifts up.
Though present from birth, symptoms of congenital fourth cranial nerve palsy may start as subtle and increase with age. Hence, diagnosis by a healthcare practitioner may not be made until later childhood or adulthood. Young children adopt a compensatory head position in order to compensate for the underacting superior oblique muscle, [2] or have a tendency to close one eye whilst reading. [3] The characteristic head tilt is usually away from the affected side to reduce eye strain and prevent double vision (diplopia). Old photographs may reveal the presence of a consistent head tilt (ocular torticollis) from an early age. Most people with congenital CN IV palsy have facial asymmetry due to the chronic head tilt. Other compensatory measures for congenital fourth nerve palsy are development of large vertical fusional amplitudes and lack of subjective symptoms of torsion, even in the presence of great ocular rotation.
Congenital fourth nerve palsy may remain undetected until adulthood, when intermittent diplopia may arise, due to decompensated ability to overcome the vertical deviation. Until this occurs, many ophthalmologists and optometrists may miss the other signs and symptoms. Reduced vertical fusional reserves result from fatigue (stress, fever, other illnesses, a lot of near work) or simply the effects of old age. Diplopia from congenital fourth nerve palsy has occasionally been reported to manifest transiently during pregnancy. Congenital fourth nerve palsy may also become evident following cataract surgery once binocular vision is restored after a long period of progressive monocular visual loss and accompanying vergence decompensation. People may complain of neck pain, after years of chronic head tilting (ocular torticollis), but this is also encountered in children. [4]
Congenital fourth nerve palsy can affect reading comprehension (and concentration during other near tasks) due to the increased vertical fusional demands and head tilting required to maintain single vision and prevent vertical diplopia. Some people find they lose their place easily while reading, and find a marker or using a finger to guide them helpful. A three step test is useful to determine whether the symptoms conform to the pattern of a fourth nerve palsy. [3]
The cause of congenital fourth nerve palsy is unclear in most cases. It may be neurogenic in origin, due to a dysgenesis of the CN IV nucleus or nerve, but a clinically similar palsy may result from absence or mechanical dysfunction (e.g., abnormal laxity) of the superior oblique tendon. Usually unilateral, congenital fourth nerve palsies can also occur bilaterally. [5] Bilateral congenital fourth nerve palsy may be unmasked only after corrective surgery of one eye for what was thought to be a unilateral palsy.
The fourth cranial nerve innervates the superior oblique muscle for each eye. The superior oblique muscle is one of the six extraocular muscles that allow movement of eye. Specifically, the superior oblique muscle primarily intorts the eye (such that the top of the eye rolls toward the nose), with secondary actions of depression (downgaze) and abduction (looking away from the nose). When this muscle's function is diminished due to a fourth cranial nerve (CN IV) palsy, the affected eye will extort, deviate upward (hypertropia), and, to a smaller extent, drift inward.
Congenital fourth cranial nerve palsy can be treated with strabismus surgery, where muscle attachment sites on the globe are modified to realign the eyes. Some eye doctors prefer conservative or no management of congenital fourth nerve palsy. Other eye doctors recommend surgery early in a patient's life to prevent the compensatory torticollis and facial asymmetry that develop with age.
Prism lenses set to make minor optical changes in the vertical alignment may be prescribed instead of or after surgery to fine-tune the correction. Prism lenses do not address torsional misalignment and this may limit their use in certain cases. An additional consideration of prism lenses is that they must be worn at all times. Prism lenses reduce vertical fusional demands by allowing the eyes to rest in their vertically misaligned state. When they are removed the patient may experience vertical diplopia they find hard to resolve due to the rested state of their eyes.
Cases of congenital fourth nerve palsy vary in magnitude and way they affect the motion of the superior oblique muscle. Therefore, different surgeries are available dependent upon the type of misalignment. Sometimes surgery on more than one eye muscle is required. In some simpler, unilateral cases a single surgery may suffice. In these cases the main problem is that the inferior oblique muscle of the same eye acts unopposed by the weakened superior oblique muscle, pulling the eye up. An example of a safe and effective procedure is a disinsertion of the inferior oblique muscle to allow it to reattach itself further down the globe of the eye. This acts to 'weaken' its action and allow the eye to move back into a more neutral alignment. [6]
In all cases of congenital fourth nerve palsy, it is important to see an experienced strabismologist about management/treatment options. A strabismologist is an ophthalmologist (eye doctor) specialising in eye movement disorders.
A Cochrane Systematic Review compared several surgical treatments (myectomy, recession, anterior transposition, disinsertion) in people with fourth nerve palsy. [7] While there was not enough high-quality evidence to recommend the best surgical treatment, all four types of surgery did result in a reduction of hypertropia. [7] One study comparing inferior oblique anterior transposition with disinsertion found that participants who underwent anterior transposition had a greater proportion of participants who had resolved head tilt, and a lesser proportion of participants requiring a second surgery, compared to disinsertion. [8] [7] However, all participants who underwent anterior transposition developed elevation deficiency. [8] [7]
The oculomotor nerve, also known as the third cranial nerve, cranial nerve III, or simply CN III, is a cranial nerve that enters the orbit through the superior orbital fissure and innervates extraocular muscles that enable most movements of the eye and that raise the eyelid. The nerve also contains fibers that innervate the intrinsic eye muscles that enable pupillary constriction and accommodation. The oculomotor nerve is derived from the basal plate of the embryonic midbrain. Cranial nerves IV and VI also participate in control of eye movement.
The trochlear nerve, also known as the fourth cranial nerve, cranial nerve IV, or CN IV, is a cranial nerve that innervates a single muscle - the superior oblique muscle of the eye. Unlike most other cranial nerves, the trochlear nerve is exclusively a motor nerve.
Torticollis, also known as wry neck, is a painful, dystonic condition defined by an abnormal, asymmetrical head or neck position, which may be due to a variety of causes. The term torticollis is derived from the Latin words tortus, meaning "twisted", and collum, meaning "neck".
Strabismus is a vision disorder in which the eyes do not properly align with each other when looking at an object. The eye that is pointed at an object can alternate. The condition may be present occasionally or constantly. If present during a large part of childhood, it may result in amblyopia, or lazy eyes, and loss of depth perception. If onset is during adulthood, it is more likely to result in double vision.
Diplopia is the simultaneous perception of two images of a single object that may be displaced horizontally or vertically in relation to each other. Also called double vision, it is a loss of visual focus under regular conditions, and is often voluntary. However, when occurring involuntarily, it results in impaired function of the extraocular muscles, where both eyes are still functional, but they cannot turn to target the desired object. Problems with these muscles may be due to mechanical problems, disorders of the neuromuscular junction, disorders of the cranial nerves that innervate the muscles, and occasionally disorders involving the supranuclear oculomotor pathways or ingestion of toxins.
Duane syndrome is a congenital rare type of strabismus most commonly characterized by the inability of the eye to move outward. The syndrome was first described by ophthalmologists Jakob Stilling (1887) and Siegmund Türk (1896), and subsequently named after Alexander Duane, who discussed the disorder in more detail in 1905.
Eye movement includes the voluntary or involuntary movement of the eyes. Eye movements are used by a number of organisms to fixate, inspect and track visual objects of interests. A special type of eye movement, rapid eye movement, occurs during REM sleep.
The medial rectus muscle is a muscle in the orbit near the eye. It is one of the extraocular muscles. It originates from the common tendinous ring, and inserts into the anteromedial surface of the eye. It is supplied by the inferior division of the oculomotor nerve (III). It rotates the eye medially (adduction).
The inferior oblique muscle or obliquus oculi inferior is a thin, narrow muscle placed near the anterior margin of the floor of the orbit. The inferior oblique is one of the extraocular muscles, and is attached to the maxillary bone (origin) and the posterior, inferior, lateral surface of the eye (insertion). The inferior oblique is innervated by the inferior branch of the oculomotor nerve.
The extraocular muscles, or extrinsic ocular muscles, are the seven extrinsic muscles of the human eye. Six of the extraocular muscles, the four recti muscles, and the superior and inferior oblique muscles, control movement of the eye and the other muscle, the levator palpebrae superioris, controls eyelid elevation. The actions of the six muscles responsible for eye movement depend on the position of the eye at the time of muscle contraction.
Sixth nerve palsy, or abducens nerve palsy, is a disorder associated with dysfunction of cranial nerve VI, which is responsible for causing contraction of the lateral rectus muscle to abduct the eye. The inability of an eye to turn outward, results in a convergent strabismus or esotropia of which the primary symptom is diplopia in which the two images appear side-by-side. Thus, the diplopia is horizontal and worse in the distance. Diplopia is also increased on looking to the affected side and is partly caused by overaction of the medial rectus on the unaffected side as it tries to provide the extra innervation to the affected lateral rectus. These two muscles are synergists or "yoke muscles" as both attempt to move the eye over to the left or right. The condition is commonly unilateral but can also occur bilaterally.
In the fields of optometry and ophthalmology, the Hirschberg test, also Hirschberg corneal reflex test, is a screening test that can be used to assess whether a person has strabismus.
Hypertropia is a condition of misalignment of the eyes (strabismus), whereby the visual axis of one eye is higher than the fellow fixating eye. Hypotropia is the similar condition, focus being on the eye with the visual axis lower than the fellow fixating eye. Dissociated vertical deviation is a special type of hypertropia leading to slow upward drift of one or rarely both eyes, usually when the patient is inattentive.
The Harada–Ito procedure is an eye muscle operation designed to improve the excyclotorsion experienced by some patients with cranial nerve IV palsy. In this procedure, the superior oblique tendon is split, and the anterior fibers – the fibers most responsible for incyclotorsion – are moved anteriorly and laterally. This selectively stretches and tightens these fibers, enhancing the incyclotorsion power of the superior oblique.
Strabismus surgery is surgery on the extraocular muscles to correct strabismus, the misalignment of the eyes. Strabismus surgery is a one-day procedure that is usually performed under general anesthesia most commonly by either a neuro- or pediatric ophthalmologist. The patient spends only a few hours in the hospital with minimal preoperative preparation. After surgery, the patient should expect soreness and redness but is generally free to return home.
Fourth cranial nerve palsy or trochlear nerve palsy, is a condition affecting cranial nerve 4 (IV), the trochlear nerve, which is one of the cranial nerves. It causes weakness or paralysis of the superior oblique muscle that it innervates. This condition often causes vertical or near vertical double vision as the weakened muscle prevents the eyes from moving in the same direction together.
Brown syndrome is a rare form of strabismus characterized by limited elevation of the affected eye. The disorder may be congenital, or acquired. Brown syndrome is caused by a malfunction of the superior oblique muscle, causing the eye to have difficulty moving up, particularly during adduction. Harold W. Brown first described the disorder in 1950 and initially named it the "superior oblique tendon sheath syndrome".
Oculomotor nerve palsy or oculomotor neuropathy is an eye condition resulting from damage to the third cranial nerve or a branch thereof. As the name suggests, the oculomotor nerve supplies the majority of the muscles controlling eye movements. Damage to this nerve will result in an inability to move the eye normally. The nerve also supplies the upper eyelid muscle and is accompanied by parasympathetic fibers innervating the muscles responsible for pupil constriction. The limitations of eye movement resulting from the condition are generally so severe that patients are often unable to maintain normal eye alignment when gazing straight ahead, leading to strabismus and, as a consequence, double vision (diplopia).
Dissociated vertical deviation (DVD) is an eye condition which occurs in association with a squint, typically infantile esotropia. The exact cause is unknown, although it is logical to assume it is from faulty innervation of eye muscles.
The Parks–Bielschowsky three-step test, also known as Park's three-step test or Bielschowsky head tilt test, is a method used to isolate the paretic extraocular muscle, particularly superior oblique muscle and trochlear nerve, in acquired vertical double vision. It was originally described by Marshall M. Parks.