Design: Comparative case series. Limited elevation in straight-up gaze and abduction can also be present, but are more subtle. -, Yang HK, Kim JH, Kim JS, Hwang JM. The etiology of the so-called A and V syndromes. Some authors recommend following such patients for resolution over time and control of the vasculopathic risk factors alone. Stager DR Jr, Beauchamp GR, Wright WW, Felius J, Stager D Sr. Anterior and nasal transposition of the inferior oblique muscles. About 17 eyes of 17 children with congenital Brown's syndrome underwent superior oblique split tendon elongation between January 2012 and March 2020 by a single surgeon. syndrome is a vertical strabismus syndrome characterized by limited elevation of the eye in an adducted position, most often secondary to mechanical restriction of the superior oblique tendon/trochlea complex. -, Coats DK, Paysse EA, Orenga-Nania S. Acquired Pseudo-Brown's syndrome immediately following Ahmed valve glaucoma implant. In mild cases, there is no vertical deviation in primary position or downshoot in adduction. By convention, the misalignment is typically labelled by the higher, or hypertropic, eye. Relocate horizontal rectus muscle. Strabismus Surgery: Basic and Advanced Strategies. Optic pit Definition/Back - Coloboma, small recess at disc rim Restriction of elevation in abduction after inferior oblique anteriorization. Bilateral CN IV palsy may have large degree of bilateral excylotorsion (e.g., > 10 degrees) on the Double Maddox rod test. Dissociated vertical deviation: Etiology, mechanism, and associated phenomena.J. Patients can present with binocular, vertical or torsional diplopia. Loss of fusion and the development of A or V patterns. For example, on alternate cover testing, the right eye would drift upward when covered and be seen to come down when the left eye is covered. Additional fourth step to distinguish from skew deviation. Strabismus. due to a paresis of another vertical muscle, it may give rise to a V pattern, with additional convergence in downgaze. An inverse Knapp procedure may be necessary. Systemic steroids and non-steroidal anti-inflammatory agents have also been utilized with variable success. [4], Other features: Abduction and extorsion. The oblique muscles abduct the eye and the vertical recti muscles adduct the eye. Esmail F, Flanders M. Masked bilateral superior oblique palsy. This page has been accessed 120,859 times. Clipboard, Search History, and several other advanced features are temporarily unavailable. For uncertain reasons, Brown syndrome is more commonly found in the right eye than the left eye. When bilateral, the vertical deviation of each eye is not related to the other, as in true hypertropia (no yoke muscle overaction is present).[4][41]. Courtesy of Federico G. Velez, MD. Brown syndrome (BS) is a rare ocular motility disorder characterized by a limitation of elevation in adduction of the eye. Clinical photograph of the patient showing A-pattern esotropia. 2020;101383. For example, Brown's syndrome (superior oblique tendon sheath syndrome), which causes tethering of the superior oblique muscle, has a similar eye movement pattern to an inferior oblique paresis. It requires not only the correction of the horizontal deviation, but also of the vertical pattern. Arrow pattern is another variant of Y-pattern, where a relative convergence is seen from midline primary position to downgaze. SO weakening procedures: SO expander, tenotomy, tenectomy or recession. Brown Syndrome. Congenital (Ex. -. It is the thinnest, and longest cranial nerve. predisposition to congenital Brown syndrome, however, most cases are sporadic in nature. Brown syndrome is a rare form of strabismus characterized by limited elevation of the affected eye. A new treatment for A and V patterns in strabismus by slanting muscle insertions. It is frequently bilateral and associated with a horizontal strabismus, although it may be isolated. Other features: Chin elevation[2]and ipsilateral true or pseudo-ptosis. Bethesda, MD 20894, Web Policies Nineteen patients were adults over the age of 21 years, and six were children under the age of 10 years. Cooper C,Kirwan JR,McGill NW,Dieppe PA. Brown's syndrome: an unusual ocular complication of rheumatoid arthritis. Arch Ophthalmol. [6] Sudden onset, of a painless, neurologically isolated CN IV without a history of head trauma or congenital CN IV palsy in a patient with risk factors for small vessel disease implies an ischemic etiology. Vertical deviation, that increases on adduction of the affected eye. High-resolution MRI demonstrated varied abnormalities in both congenital and acquired Brown syndrome such as traumatic or iatrogenic scarring, avulsion of the trochlea, cyst in the superior oblique tendon, inferior displacement of the lateral rectus pulley and fibrous restrictive bands extending from the trochlea to the globe (Bhola et al, 2005). Before Strabismus secondary to implantation of glaucoma drainage device. Yang HK, Kim JH, Hwang JM. Special focus should be given to the sensory-motor examination, including strabismus measurements in all cardinal positions of gaze, ocular motility, and binocular function/stereopsis. FOIA The incidence of Brown's Syndrome was unrelated to tuck size. Spoor TC, Shippman S. Myasthenia Gravis Presenting as an Isolated Inferior Rectus Paresis. [4] Sometimes bilateral involvement can be masked due to an asymmetrical involvement. It is seen in bilateral inferior oblique overaction, Brown syndrome, or Duane syndrome (DS). Conclusions: Based on . With a bilateral dissociated vertical deviation, both eyes are seen to drift up when covered and re-fixate with a downward movement when uncovered. There are several clinically significant features of the trochlear nerve anatomy. When the head is tilted, extorsion and intorsion movements are executed. An official website of the United States government. So, in a patient with right hypertropia that worsens in left gaze, this suggests either right superior oblique or a left superior rectus involvement. In the case of orbital floor fracture with IR affection: If 8-15PD in primary position: Unilateral IR recession. : Thyroid ophthalmopathy; secondary to superior oblique overaction). There are eight possible muscles that could cause a hypertropia -- the bilateral superior recti, inferior recti, superior obliques and inferior obliques. Skew deviation may demonstrate decreasing vertical strabismus with position change from upright to supine. PubMedGoogle Scholar, 2017 Springer International Publishing AG, Kushner, B.J. It manifests when binocular fusion is interrupted either by occlusion or by spontaneous dissociation. Disclaimer. To distinguish between a IO paresis and a SO overaction see head-tilt-test above. For example, workup for a suspected inflammatory etiology may require laboratory testing, while suspected trauma may prompt additional imaging. -, Kaeser PF, Kress B, Rohde S, Kolling G. Absence of the fourth cranial nerve in congenital Brown syndrome. 1993;68(5):501-509. doi:10.1016/S0025-6196(12)60201-8, Dosunmu EO, Hatt SR, Leske DA, Hodge DO, Holmes JM. The trochlear nerve has the longest intracranial course of all of the cranial nerves. The trochlear nerve passes adjacent to the ophthalmic division of the trigeminal nerve and the two share a connective tissue sheath. ANATOMY. Pseudo patterns must be ruled out by measuring the deviations after prescribing appropriate refractive correction or observing the deviation under cover to prevent fusion. https://doi.org/10.1007/978-3-319-63019-9_15, DOI: https://doi.org/10.1007/978-3-319-63019-9_15. A and V patterns seen in exodeviation and esodeviation. JAMA Ophthalmol. Introduction. If the hypertropia is worse in ipsilateral tilt this implicates the ipsilateral superior oblique as the intorsional ability of the superior oblique is weakened. Kushner BJ. Urrets-Zavalia A. Abduction en la elevacion. Congenital Brown's Syndrome: Intraoperative Findings Surgical Procedures and Postoperative Results Andreea Ciubotaru Brave Inferior Oblique Vincent Paris Early Strabismus Surgery can improve Facial Asymmetry in Anterior PlagiocephalyLeila S Mohan Superior Oblique Tendon Elongation with Bovine Pericardium (Tutopatch) for Brown Syndrome. Brown syndrome is attributed to a disturbance of free tendon movement through the trochlear pulley. It is a rare and a bilateral involvement is very uncommon. (Courtesy of Vinay Gupta, BSc Optometry), Figure 7. The key feature is inability to elevate the adducted eye. The Academy uses cookies to analyze performance and provide relevant personalized content to users of our website. https://eyewiki.org/w/index.php?title=Hypertropia&oldid=91972, Elevation deficit and VS worst in adduction, occasional over-depression in adduction, Elevation deficit and VS worst in adduction, Depression deficit and VS worst in adduction, Worse with ipsilateral tilt, alternates if bilateral, Over-elevation in adduction. The terminology regarding Brown syndrome has varied and was often confusing. A waiting period of 6 to 12 month following thyroid function test stabilization is recommended. Several theories have been put forth to explain the occurrence of pattern in horizontal strabismus. If a vertical deviation in primary position, abnormal head posture or diplopia: If vertical deviation <10DP: Ipsilateral SO weakening (see superior oblique overaction). In the presence of a significant Y pattern in upgaze, even if there is no significant deviation in primary position or sidegaze: Bilateral IO weakening procedures. In this chapter, we will discuss in detail the various types of pattern strabismus, its mechanisms, and the appropriate surgical intervention for the same. The pathophysiology of this phenomenon is multifactorial and has been attributed to factors including oblique muscle dysfunction, horizontal or vertical recti anomaly, displacement of muscle pulleys, and orbital anomalies. In the case of a traumatic cause, it is advised to wait for 6 months and reevaluate for a potential recovery. However, oblique muscles have the greatest effect on vertical alignment when the eye is adducted and so are tested in adduction. Furthermore, careful history including associated symptoms and other past medical history can help distinguish a CN 4 palsy from other items on the differential. Romano P, Roholt P. Measured graduated recession of the superior oblique muscle. (Bielschowsky head tilt test). In pseudo-inferior rectus palsy with hypertropia in primary position: Ipsilateral muscle slack reduction through a plication + contralateral IR recession. Conversely, when an eye with a normal SO elevates in adduction, the SO insertion moves posteriorly, pulling the SO tendon through the trochlea. - Oblique palpebral fissures - Prominent epicanthal folds - Brush field spots . Incomitant strabismus associated with instability of rectus pulleys. Does the hypertropia worsen in left or right head tilt? The first challenge for the clinician is to diagnose the pattern and the second is to identify the cause. Flowchart showing various theories for pattern strabismus. The superior rectus and inferior oblique muscles elevate the eye and the inferior rectus and superior oblique muscles depress the eye. (Courtesy of Vinay Gupta, BSc Optometry), Figure 4. If horizontal recti are displaced superior- or inferiorly, they act as additional elevators or depressors. Diagnostic Criteria for Graves' Ophthalmopathy. After extensive further investigation, it was demonstrated that key clinical features were a V or Y pattern strabismus, divergence in upgaze, downdrift in adduction, and a positive forced duction test for ocular elevation in the nasal field. To make everything a bit more confusing, a Y pattern can also be present when there is an aberrant innervation of the lateral recti, in upgaze,[42] or in the case of a bilateral inferior oblique overaction (see above). Brown Syndrome secondary to an inflammatory condition is frequently associated with orbital pain and tenderness on movement or palpation of the trochlea. In cases of acquired Brown syndrome, a thorough orbital examination should be performed with special attention to the trochlear area. (Courtesy of Vinay Gupta, BSc Optometry), Figure 6. Vertical strabismus describes a vertical misalignment of the eyes. In the case of a coexisting DVD, particular care has to be taken since SO weakening procedures may worsen this entity. Although A or V patterns are the most common patterns observed (Figure 1), there are several other patterns that can be seen in a comitant strabismus. : Strabismus surgery; glaucoma surgery, especially with the Baerveldt device or due to a mass effect caused by the bubble, The impacted muscle will be a depressor of the higher eye (inferior rectus or superior oblique) or a elevator of the lower eye (superior rectus or inferior oblique), Determine in which horizontal gaze the hypertropia is worse, If worse in left gaze, the oblique muscles in the right eye or the vertical recti in the left eye are affected, If worse in right gaze, the oblique muscles in the left eye or vertical recti in the right eye are affected, Determine in which head tilt the deviation is the worse, If worse in right tilt, the right eye intorters (superior oblique and superior rectus) or left eye extorters (inferior oblique and inferior rectus) are affected, If worse in left tilt, the left eye intorters (superior oblique and superior rectus) or right eye extorters (inferior oblique and inferior rectus) are affected. - 89.22.67.240. Bilateral superior oblique palsies. This patient had no abnormal neurologic findings. ptosis,miosis, etc.). The 2 most commonly performed surgeries for correction of vertical incomitance in a horizontal strabismus are: Video 1: Inferior Oblique Recession Procedures. Signs and symptoms associated with CN II,III, V, VI and II. Note convergence in straight upgaze, an important point of differentiation from Brown syndrome. Etiology and outcomes of adult superior oblique palsies: a modern series. 2015;19:e14. Dawson E, Barry J, Lee J. Spontaneous resolution in patients with congenital Brown syndrome. 2015 Jul;26(5):357-61. In this procedure it is important to keep the anterior IO fibres posterior to the IR insertion in order to avoid a hypercorrection and consequent hypodeviation. (Courtesy of Vinay Gupta, BSc Optometry), Figure 2. 2011. doi:10.1001/archophthalmol.2011.335, Parulekar M V, Dai S, Buncic JR, Wong AMF. [4] A vertical deviation in primary position is more frequently associated with a unilateral or asymmetric SO paresis. Strabismus surgery can be used in patients who do not respond or tolerate prisms. Brown syndrome, in simplest terms, is characterized by restriction of the superior oblique trochlea-tendon complex [ 1] such that the affected eye does not elevate in adduction. Previously referred to as "superior oblique tendon A translucent occluder for study of eye position under unilateral or bilateral cover test. The site is secure. Computed tomography (CT) scan is generally the first line imaging study in trauma but is often normal. Idiopathic : Following strabismus surgery). Munoz M, Parrish Rk. Bartley GB, Gorman CA. Manley, DR and Rizwan, AA. [Brown's atavistic superior oblique syndrome: etiology of different types of motility disorders in congenital Brown's syndrome]. Vertical misalignments of the eyes typically results from dysfunction of the vertical recti muscles (inferior and superior rectus) or of the oblique muscles (the inferior oblique and superior oblique). Fourth cranial nerve palsies can affect patients of any age or gender. Increased vertical deviation on head tilt to the ipsilateral side. Brown Syndrome. The clinical features were similar to those of an inferior oblique palsy, although there was minimal superior oblique muscle overaction. The three questions to ask in evaluation of the CN IV palsy are as follows: Features suggestive of a bilateral fourth nerve palsy include: The management of a trochlear nerve palsy depends on the etiology of the palsy. Isolated third, fourth, and sixth cranial nerve palsies from presumed microvascular versus other causes: A prospective study. When the cover is switched back to the right eye again, there is NO upward refixation movement of the left eye. Trochlear nerve palsy is a common cause of congenital cranial nerve (CN) palsy. a #240 retinal silicone band), a non-absorbable "Chicken suture", or a superior oblique split tendon lengthening procedure, Iatrogenic Brown syndrome secondary to muscle plication may require reversal of the plication, In case the primary cause is a tendon cyst, removal of the cyst may be indicated. A tendon cyst or a mass may be palpable in the superonasal orbital. of Brown syndrome. and transmitted securely. The majority of patients have a congenital form of the syndrome but acquired inflammatory cases have been . If the deviation has become comitant due to superior and inferior rectus contractures, respective recessions should be performed. Muscle disfunction may result from paresis, restriction, over-action, muscle malpositioning, and dysinnervation. [7] Fourth nerve palsy secondary to microvascular disease will frequently resolve within 4-6 months spontaneously. Neurology. [1] Contents 1Disease Entity The disorder can be distinguished clinically from an inferior oblique palsy by the presence of positive forced duction testing, the absence of superior oblique overaction, and, typically, normal alignment in primary gaze. Patients can also develop a compensatory head tilt in the direction away from the affected muscle. Examiners should consider obtaining the following: visual acuity, motility evaluation, binocular function and stereopsis, strabismus measurements at near, distance, and in the cardinal positions of gaze, and evaluation of ocular structures in the anterior and posterior segments.
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