Paralytic Strabismus Chapter 20. ORBIS Telemedicine. 2003. http://telemedicine.orbis.org/bins/content_page.asp?

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Cranial Nerve IV Paralysis ETIOLOGY. In our practice superior oblique paralysis is the most common form of paralytic strabismus. In a review of 270 patients with superior oblique paralysis treated by us during a 10-year period, a congenital paralysis was encountered most often (39.5%). This was followed in order of frequency by traumatic (34%), idiopathic (23.2%), and neurologic (2.9%) paralyses.202 This etiologic distribution is similar to that reported by other strabismologists, 67, 136, 142 but differs in a neuroophthalmologic practice where trauma and vascular disorders predominate and congenital paralysis is only infrequently diagnosed. 217 Blunt head trauma, often only a mild concussion without loss of consciousness, is among the most frequent causes, but direct injury to the trochlea143, 159 or to the tendon during blepharoplasty 273 has also been reported. Congenital superior oblique paralysis may also follow an autosomal dominant mode of inheritance29 but this occurrence is rare. It is of historical interest that in the preantibiotic era iatrogenic damage to the trochlea during ethmoidectomy was probably the most common cause of a cranial nerve IV palsy. A superior oblique paralysis of sudden onset and without a history of trauma, while in most instances caused by spontaneous decompensation of a congenital paralysis, may also signal an intracranial process.17, 49, 151 Myasthenia gravis226 and multiple sclerosis120 may present as an isolated unilateral superior oblique paralysis with an insidious onset and may, therefore, easily be confused with a congenital paralysis. Of 221 cases with trochlear paralysis 6 patients had a unilateral recently acquired cisternal schwannoma of the trochlear nerve as diagnosed with neural imaging. 72 None developed additional symptoms or signs of cranial nerve or central nervous system involvement. Helveston and coworkers108 drew attention to the fact that the superior oblique tendon is different in congenital as compared to acquired paralysis. A redundancy of the tendon or an abnormal posterior insertion of the tendon into Tenons capsule was noted in most congenital but not in acquired palsies, and Plager214, 215 confirmed the laxity of the tendon in congenital cases at the time of surgery by traction testing of the superior oblique. Slight ultrastructural differences in superior oblique tendons from patients with congenital and acquired paralyses have been observed108 and the question arose whether patients with the congenital variety have a true paralysis of that muscle. This speculation gained further substance by the findings of Tian and Lennerstrand260 who reported that the peak saccadic velocity of the eye during downward movement in adduction was more reduced in acquired than in congenital paralysis. On the other hand, magnetic resonance of congenital paralysis in which a patient was aware of image tilting under casual conditions of seeing. This should not distract from the fact that after dissociation of the eyes with Maddox rods, cyclotropia can be diagnosed in congenital cases as well because cyclofusion or other sensory or psychological mechanisms to eliminate image tilting under casual conditions of seeing are disrupted with this test (see Chapter 18). Excyclotropia, when measured with the

imaging (MRI) of the superior oblique muscle has shown a more pronounced volume reduction of the muscle in congenital vs. acquired cases.1, 231, 232 Whether this constitutes a denervation atrophy or represents a primary anomaly, as suggested by Sato,231 is not clear at this time. Be this as it may, the recognition of differences in the physical characteristics of the tendon in congenital and acquired cases has important therapeutic implications (see p. 450).

Maddox double rod test, may occur in the nonparalyzed eye in patients who habitually fixate with their paralyzed eye because of a monocular sensorial adaptation to the cyclodeviation that has taken place in that eye.208

DIAGNOSIS. Diagnosis of superior oblique paralysis is based on the presence of a hypertropia, usually greatest in the nasal field of the involved eye, but not necessarily in the field of action of the paralyzed muscle. Overaction of the We discussed in Chapter 18 that apparent unopposed antagonistic inferior oblique oblique dysfunction, including decreased commonly causes the hypertropia to be depression in adduction, may actually be greatest in the field of action of that caused by heterotopic muscle pulleys.47 muscle. Kommerell and coworkers147 MRI may be of help in differentiating true described two most unusual patients with from pseudoparalysis of the superior the clinical signs of superior oblique palsy oblique muscle.60 In the latter instance the who were able to vary their vertical angle volume and the contractile function of the of strabismus at will. muscle will be normal. In view of the difficulties encountered by SYMPTOMS. The symptoms of superior the student of ocular motility in oblique palsy may consist of asthenopia, diagnosing a superior oblique paralysis vertical diplopia, image tilting, and an and in view of its being confused with a anomalous head posture. The question superior rectus paralysis of the fellow eye arises whether the presence or absence of and its high prevalence, the principal diplopia, with or without image tilting, is a diagnostic and clinical features in a helpful symptom in distinguishing patient with a left superior oblique between recently acquired and congenital paralysis are shown in detail in Figure 20 paralysis. It is essential to determine the 20. With a spread of comitance and with age of onset whenever possible since a secondary contracture of the ipsilateral recently acquired superior oblique palsy superior rectus muscle, the hypertropia of nontraumatic origin requires a medical may involve the entire lower field of gaze. workup. Conversely, when the onset is This contracture of the superior rectus is clearly congenital, treatment, if indicated, easily diagnosed with the forced duction may commence without further test and may cause pseudo-overaction of evaluation of the patient by costly and the superior oblique in the uninvolved eye unnecessary procedures. Although (secondary deviation).124, 202, 248 Knapp142 and vertical diplopia occurred in our series Knapp and Moore143 introduced a more commonly in patients with acquired classification that describes the most superior oblique palsy, 25% of patients common manifestations of superior with congenital palsy also complained oblique paralysis. Although modifications about diplopia. Therefore, the presence or of this classification have been absence of diplopia is not a reliable sign suggested108, 243, 244 we have adopted in determining the onset. However, image Knapps as being the practical one. tilting as an isolated symptom or Depending on the magnitude of combined with vertical diplopia occurred hypertropia in the diagnostic positions of only in acquired paralysis and thus gaze, seven classes are distinguished. emerges as a valid differential diagnostic criterion.202 We do not know of one A description of each class and its instance prevalence in a group of 202 patients

seen in our practice in

FIGURE 2020. Clinical findings in a patient with a long-standing traumatic left superior oblique paralysis. The head is tilted to the right shoulder and the face is slightly turned to the right (A). In primary position this patient had a left hypertropia of 20 prism diopters (F), increasing to 30 prism diopters in dextroversion (E), with the greatest deviation (35 prism diopters) when the patient was looking up and to the right (B). The hyperdeviation was also present in the left field of gaze (D, G) where it measured 10 prism diopters (spread of comitance). Note secondary overaction of the left inferior oblique muscle (B, E) and only minimal limitation of depression when looking down and to the right (H). The Bielschowsky head tilt test is diagnostic for a left superior oblique paralysis with increase of the left hypertropia on tilting the head to the left shoulder (K, L).

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whom preoperative diagnostic positions head tilt toward the paralyzed side. could be determined on the deviometer Whereas this occurred in only 7 of 270

is presented in Table 205.202 The right eye is used here as an example. Examples: in class 1 the hypertropia is greatest when the right eye is elevated and adducted (27% of our patients). The deviation is categorized as class 3 when it is of equal magnitude in the entire paralyzed field of gaze (21% of our patients).

patients,202 unawareness of the existence of a paradoxical head tilt may easily confound the diagnosis. A comparison of the clinical findings obtained in patients with a paradoxical head tilt and in those with a head tilt that "conformed to the rule" showed that in the former group intermittent and unstable fusion was present with the head tilted toward the uninvolved Whereas the distribution of hypertropia side, but alternating suppression and in the different gaze positions may vary diplopia occurred when holding the head toward the paralyzed side.202 Thus because of a spread of comitance, an these patients preferred a head exception exists in class 7. In these position that disrupted fusion, caused a patients a classic superior oblique wide separation of the double images, paralysis is associated with restriction and thus eliminated the discomfort that of elevation in adduction (pseudomay have been associated with the Brown syndrome) and direct trochlear trauma is the cause. Knapp and Moore143 constant effort to maintain single binocular vision in the presence of a mentioned dog bites as a common superior oblique weakness. cause, to which we add frontal sinus surgery. Unless a history of recent trauma or old Auxiliary diagnostic features include a photographs clearly establish the traumatic or congenital nature of a positive Bielschowsky head tilt test, superior oblique paralysis, the clinician which is nearly always present and a head tilt toward the nonparalyzed side, may be in a quandary in deciding which is present in only approximately whether to order neuroimaging. A recently established guide for the cost70% of the patients.202 Burian and 38 coworkers explained the absence of a effective evaluation of patients with superior oblique paralysis indicates that head tilt by the patients inability to isolated congenital, old traumatic, or obtain single binocular vision by any vicarious head position, by the presence vasculopathic cases do not require neuroimaging.155 Patients with of large fusional amplitudes, or by nonisolated palsies require directed reduced visual acuity in one eye. neuroimaging studies based upon the However, we have been unable to confirm this since visual acuity of each results of the nonocular symptomatology. eye, the ability to fuse in primary position, and the magnitude of hypertropia and cyclotropia were the CONGENITAL ABSENCE OF THE same in patients with and without a SUPERIOR OBLIQUE MUSCLE. This head tilt.202 Of special interest are mostly unexpected finding at the time patients with a paradoxical of surgery presents a special challenge to the surgeon. Only in rare instances is this anomaly diagnosed prior to the operation. Helveston and coworkers107 noted the association of amblyopia and TABLE 205. Classification According to Amount of of a horizontal deviation in these cases. Hypertropia in Diagnostic Positions (N 202) To this we added a large hypertropia in primary position, spread of comitance, and pseudo-overaction of the contralateral superior oblique muscle as additional clinical features.268 Neural imaging may demonstrate congenital

absence of the superior oblique muscle preoperatively and thus facilitate planning of effective surgical correction. SPONTANEOUS LIMITATION OF ELEVATION (BROWN SYNDROME) FOLLOWING ACQUIRED SUPERIOR OBLIQUE PARALYSIS. The first patient with this intriguing anomaly following mostly traumatic superior oblique paralysis was described by Fox77 in 1981 and not more than 12

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other cases have been reported since.192, 235, 249 The onset of limitation of elevation in adduction is gradual, progressive, and may mimic Brown syndrome (see Chapter 21). The forced duction test was positive in some but not in all patients. The etiology is entirely speculative and fibrotic reaction of the superior oblique tendon or adjacent structures235 or a secondary contracture of the tendon192 have been mentioned. UNILATERAL VS. BILATERAL PARALYSIS. It is most important to carefully examine the patient for involvement of the fellow eye when superior oblique paralysis is of traumatic origin. Bilateral involvement was present in 19 (21%) of 92 traumatic cases observed in our clinic,202 which is in contrast to the 88% of cases reported by other observers.193 The severity of the paralysis is often asymmetrical, and the involvement of the second eye may not become apparent until the eye with the more severe defect has been operated on (masked bilateral superior oblique paresis).109, 117, 148, 153, 264 A right hypertropia in left gaze and a left hypertropia in

may be accompanied by chin depression. Finally, in patients with bilateral paralysis the vertical deviation in primary position is usually smaller than with unilateral involvement since the loss of the depressing function in one eye tends to balance the same loss in the fellow eye.136, 202 The differential diagnostic points between unilateral and bilateral involvement are summarized in Table 206. Ellis and coworkers68 made the interesting observation that surgical overcorrection of a unilateral superior oblique paralysis may masquerade as an apparent contralateral superior oblique paresis. PARALYSIS VS. PSEUDOPARALYSIS. Premature unilateral stenosis of the coronal sutures (plagiocephaly) may cause a pseudoparalysis of the superior oblique muscle with upshoot in adduction because of desagittalization of the planes of these muscles.10, 218a The retroplacement of the trochlea in these

right gaze, as well as a positive Bielschowsky test with the head tilted to either side, are the only signs we consider diagnostic for bilateral involvement because neither occurred in unilateral cases.202 However, absence of these two features does not exclude bilateral involvement. Several authors have stated that when excyclotropia is in excess of 10 to 15 a bilateral paralysis should be suspected.67, 149, 183 However, in reviewing 203 patients with unilateral and bilateral paralysis we were unable to confirm this widely held view: the mean excyclotropia was 7 (range, 1 to 25) in patients with unilateral and 8 (range, 3 to 20) in patients with bilateral paralysis.202 Kushner153 added bilateral objective excyclotorsion of the globes on fundus examination as another sign in distinguishing unilateral from bilateral paralysis. It has been claimed that bilaterality should be suspected when excyclotropia increases significantly in downward gaze.140 However, it is our experience that this occurs also in unilateral cases. Since the etiology of bilaterality of the paralyses is often traumatic the cyclotropia may be symptomatic. A significant V pattern (15 or more difference between upward and downward gaze), commonly accompanied by chin depression, occurred in 48% of our patients with bilateral, but also in 5% of patients with unilateral paralysis.202 The V pattern is caused by a decrease in the abducting effect of the superior oblique(s) in depression and overaction of the inferior oblique muscle(s) and

cases increases the angle between the reflected part of the superior oblique tendon and the plane of the inferior oblique muscle (Fig. 20 21). This reduces the vertical effect of the superior oblique muscle while increasing its incyclotorsional effect and causes an imbalance of opposing muscle forces in favor of the inferior oblique muscle. Figure 2022 shows the rather characteristic appearance of a patient with premature closure of the left coronal suture and marked hypertropia of the left eye. Limn de Brown and coworkers163 studied with anthropometric methods the relationship between orbital malpositioning and strabismus in plagiocephalic children and established a
TABLE 206. Diagnosis of Bilateral Superior Oblique Paralysis

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FIGURE 2021. Left plagiocephaly. Recession of the left trochlea reduces the length of the unreflected part of the left superior oblique (SO) muscle thus decreasing its contracting power and causing imbalance in relation to the contracting power of the ipsilateral inferior oblique (IO). Angle alpha is greater than angle beta, causing diminished vertical effect of SO. RE, Right eye; LE, left eye; a, distance between the sagittal axis of two muscles in orbit. (From Bagolini B, Campos EC, Chiesi C: Plagiocephaly causing superior oblique deficiency and ocular torticollis. A new clinical entity. Arch Ophthalmol 100:1093, 1982.)

quantitative correlation between the degree of orbital anomalies (vertical displacement, intorsion, and frontodisclination) and the hypertropia in the nasal field of the involved side.