JAMA Ophthalmol. Bilateral involvement is rare in non-traumatic cases but is relatively more frequent after trauma (crossed, dorsal exit). due to a paresis of another vertical muscle, it may give rise to a V pattern, with additional convergence in downgaze. 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]. Surgery can be considered in the following circumstances: The following surgical procedures can be performed: Image added in courtesy of Dr Agathi Kouri, MD, FRCS, Panagiotis and Aglaia Kiriakou Children's Hospital, Athens, Greece. Mourits M, Koornneef L, Wiersinga M,Prummel. Spoor TC, Shippman S. Myasthenia Gravis Presenting as an Isolated Inferior Rectus Paresis. Microvascular disease can involve CN IV and usually in older patients with cardiovascular risk factors. It is reported in 70% of patients with esotropia and 30% of patients with exotropia. ), Innervational anomaly of the superior division of the III cranial nerve, Neoplastic (ex. Brown syndrome refers to the apparent weakness of the inferior oblique muscle (i.e., limited upgaze, particularly in adduction) secondary to pathology of the superior oblique tendon sheath, usually at the trochlea. If the hypertropia is worse in ipsilateral tilt this implicates the ipsilateral superior oblique as the intorsional ability of the superior oblique is weakened. 2008 Sep-Oct;23(5):291-3. Thacker NM, Velez FG, Demer JL, Rosenbaum AL. https://doi.org/10.1007/978-3-319-63019-9_15, DOI: https://doi.org/10.1007/978-3-319-63019-9_15. 2017 Aug 25;17(1):159. The 2 most commonly performed surgeries for correction of vertical incomitance in a horizontal strabismus are: Video 1: Inferior Oblique Recession Procedures. Brown syndrome is caused by a malfunction of the superior oblique muscle, causing the eye to have difficulty moving up, particularly during adduction (when eye turns towards the nose). If horizontal recti are displaced superior- or inferiorly, they act as additional elevators or depressors. A longitudinal long-term study of spontaneous course. Note convergence in straight upgaze, an important point of differentiation from Brown syndrome. American Academy of Ophthalmology. CrossRef Could demonstrate that the fundus of the affected eye is excyclotorted. Strabismus Surgery: Basic and Advanced Strategies. government site. Romano P, Roholt P. Measured graduated recession of the superior oblique muscle. The trochlear nerve has the longest intracranial course of all of the cranial nerves. Vertical Strabismus. Taylor & Hoyt's Pediatric Ophthalmology and Strabismus, by Scott R. Lambert and Christopher J. Lyons, Elsevier, 2017, pp. Systemic steroids and non-steroidal anti-inflammatory agents have also been utilized with variable success. [4], Most frequently both eyes are affected, although it may be asymmetrical . If cosmetically intolerable or if noticeable: If associated with an IO overaction: Sole IO graded anteriorization, If associated with an SO overaction: Treat the A pattern with horizontal muscle transpositions, or an undercorrected SO weakening procedure, since the latter may aggravate the symptoms of DVD, If both eyes can fixate: Bilateral SR recessions, with asymmetric recessions if asymmetric, If overcorrected: Associate an IR plication or resection. In fourth nerve palsy the Double Maddox rod should demonstrate unilateral excyclotorsion. The trochlear nucleus is in the midbrain, dorsal to the medial longitudinal fasciculus at the level of the inferior colliculus. Inferior oblique muscle overaction (IOOA) manifests by overelevation of the eye in adduction and is frequently associated with horizontal deviations. 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 right superior oblique example to the right, the right eye is hypertropic and the deviation is worse in left gaze and right tilt. Intraocular Pressure: Restrictions may lead to increase IOPs when the eye is moving against the restriction. Duane1 introduced the concept of pattern in strabismus in 1897 when he described V pattern in bilateral superior oblique palsy. Pseudo V-esotropia may be seen in accommodative esotropias with uncorrected hyperopic refractive error. [2][39][40], A dissociated vertical deviation is an upward drift of one eye when binocular fusion is interrupted (such as with alternate cover testing) that is not associated with a compensatory downward shift of the fellow eye when attention if focused on the drifting eye. : Left superior oblique paresis causes a left hypertropia on right gaze and head tilt to the left. Harrad R. Management of strabismus in thyroid eye disease. This hypothesis has gained support from the confluence of evidence from a number of independent studies. The oblique muscles abduct the eye and the vertical recti muscles adduct the eye. A co-innervation of the superior oblique and medial rectus muscles is not implausible, as . Dr. Harold Brown first described eight cases of a new ocular motility condition, which presented with restricted elevation in adduction, among other features in 1949. Errors in the Three-step Test in the Diagnosis of Vertical Strabismus. Furthermore, careful history including associated symptoms and other past medical history can help distinguish a CN 4 palsy from other items on the differential. If inflammatory: systemic nonsteroidal antiinflammatory agents, local steroid injection to the trochlea. Wright KW, Brown's syndrome: diagnosis and management, Trans Am Ophthalmol Soc. : Rheumatoid arthritis; systemic lupus erythematosus), Tight superior oblique muscle (Ex. Mims JL 3rd, Wood R.Bilateral anterior transposition of the inferior oblique. Pearls and oy-sters: Central fourth nerve palsies. Patients may develop a compensatory head tilt to the contralateral side to reduce their diplopia. Various inferior oblique weakening procedures are: Various superior oblique weakening procedures are: Video 2: Posterior Tenectomy of Superior Oblique, Figure 10. It most often occurs as a congenital condition. Arch Ophthalmol. Forced Duction Test: Forced duction testing can evaluate for evidence of restriction and possibly of laxity in the setting of a muscle palsy, Saccadic Eye Movements: In the case of a restriction, normal saccadic eye movements can be observed until the full restrictive amplitude is achieved, where it stops abruptly. Gobin MH. Springer, Cham. Late overcorrections are frequent. Kushner, Burton J. It is seen in bilateral inferior oblique overaction, Brown syndrome, or Duane syndrome (DS). VS often limited to adduction, Depression deficit and VS worst in abduction, Decreased force generation and saccadic velocity, Elevation deficit and VS worst in abduction, Decreased force generation and saccadic velocity, Elevation deficit and VS worst in abduction, Depression deficit and VS worst in abduction, Alternate cover testing shows an upward drift when the eye is covered, without a compensatory upward refixation of the fellow up. MRI may show an infarction in the tegmentum of the midbrain, affecting the fascicle of the fourth nerve. Increased intracranial pressure has also been known to cause CN 4.[8]. Das VE, Fu LN, Mustari MJ, Tusa RJ. Further workup may be needed in acquired Brown syndrome and often depends on the suspected underlying etiology. PMC Nineteen patients were adults over the age of 21 years, and six were children under the age of 10 years. Vertical deviation, that increases on adduction of the affected eye. Spielmann A. 2015 Jul;26(5):357-61. Magnetic resonance imaging of the head (MRI) is often unremarkable in CNV IV palsy but may show a dorsal midbrain contusion or hemorrhage.[5]. syndrome should be differentiated from the following conditions: Management of Brown syndrome depends on symptomatology, etiology, and the course of the disease. A compensatory abnormal head position may be present, often patients adopt a chin up position or a head turn away from the affected eye (to keep the affected eye abducted, avoid hypotropia, and promote binocular fusion). Nearly three fourths (71.4%) of the children had a IVth cranial nerve palsy, primary inferior oblique overaction, Brown syndrome, or a vertical tropia in the setting of an abnormal central nervous . - 89.22.67.240. : Thyroid ophthalmopathy; secondary to superior oblique overaction). due to a paresis of another vertical muscle, it may give rise to a V pattern, with additional convergence in downgaze. 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. [42], Patients with Browns syndrome will have a positive forced-duction test especially evident on the Guytons exaggerated forced-duction test. Combined Brown syndrome and superior oblique palsy without a trochlear nerve: case report. [7] Fourth nerve palsy secondary to microvascular disease will frequently resolve within 4-6 months spontaneously. Conclusions: Based on . In the case of orbital floor fracture with IR affection: If 8-15PD in primary position: Unilateral IR recession. 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. Prendiville P, Chopra M, Gauderman WJ, Feldon SE. 2012 Jun;90(4):e310-3. JS Crawford, Surgical treatment of true Brown's syndrome, American journal of ophthalmology, 1976. The amount of suppression, which can vary from small suppression scotomas in binocular fusion to large suppression areas on the affected side and amblyopia, depends on various factors such as the size of the strabismus and age of onset. Parks MM, Eustis HS. J AAPOS. The IV nerve then courses around the cerebellar peduncle and travels between the superior cerebellar and posterior cerebral arteries in the subarachnoid space. 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. A spontaneous resolution of congenital Browns syndrome has been reported. Fever, headache, neck stiffness may be associated with meningitis. - Morning glory syndrome Term/Front. Kushner BJ. https://www.ophthalmologytimes.com/article/seven-easy-steps-evaluation-fourth-nerve-palsy-adults, https://eyewiki.org/w/index.php?title=Cranial_Nerve_4_Palsy&oldid=90774, Hemisensory loss, ataxia, internuclear ophthalmoplegia, hemiparesis, central Horner syndrome, cranial nerve III palsy, Frequently due to infarction or hemorrhage. Pusateri TJ, Sedwick LA, Margo CE. Skew deviation may demonstrate decreasing vertical strabismus with position change from upright to supine. 1985. doi:10.1136/bjo.69.7.508. True and simulated superior oblique tendon sheath syndromes. oblique palsy after surgery for true Brown's syndrome Jan 1958 82-86 oblique palsy after surgery for true Brown's syndrome. nerve palsy and Brown syndrome, it is instructive to briefly review the evolution in our understanding of Duane retrac-tion syndrome, the prototypical CCDD. Smith TJ Thyroid-associated Ophthalmopathy: Emergence of Teprotumumab as a Promising Medical Therapy. The Parks-three-step-test can be used to help determine the cause of a vertical misalignment caused by a single muscle paresis. 20 However, results for pattern XT and with Duane syndrome-related upshoot were variable. Brown H. Isolated Inferior Oblique Paralysis: An Analysis of 97 Cases. On version testing Brown syndrome might be confused with an inferior oblique muscle (IO) palsy. Trochlear nerve palsy can also occur as part of a broader syndrome related to causes like trauma, neoplasm, infection, and inflammation. When the head is tilted, extorsion and intorsion movements are executed. Ventura MP, Vianna R , SouzaJ, Solari HPand Curi RLN. Thyroid eye disease leads to enlargement of the extraocular muscles and restrictive strabismus. Crouzon syndrome: relationship of rectus muscle pulley location to pattern strabismus. It may be addressed surgically with a Y-splitting procedure of the ipsilateral lateral rectus muscle. Ophthalmol Times. Improvement of congenital Brown syndrome has been described in up to 75% of cases. In the case of forced duction limitation, add an inferior rectus recession to the former. 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). Trans Am Ophthalmol Soc. -, Coats DK, Paysse EA, Orenga-Nania S. Acquired Pseudo-Brown's syndrome immediately following Ahmed valve glaucoma implant. Hypertropia or hypotropia in in adduction. (Courtesy of Vinay Gupta, BSc Optometry). Kushner BJ. Pineles SL, Velez FG, Elliot RL, Rosenbaum AL. An inverse Knapp procedure may be necessary. Superior oblique muscle paresis and restriction secondary to orbital mucocele. Microvascular causes may spontaneously resolve over the course of weeks or months. Determining the onset, severity, and chronicity of symptoms can be vital in delineating between the various etiologies of a CN 4 palsy. Careful examination is necessary in traumatic cases as the CN IV palsies can by asymmetric if bilateral and can be masked or become apparent after strabismus surgery for a presumed unilateral CN IV palsy. (Courtesy of Vinay Gupta, BSc Optometry). [43], In inferior oblique overaction there is an increase of ipsilateral hypertropia in adduction to the contralateral side with a contralateral hypotropia, whereas in DVD, there is a hypertropia in adduction as well as in and abduction without a true contralateral hypotropia, when binocular fusion is interrupted. Bilateral CN IV palsy might show bilateral excyclotorsion. Diagnostic Criteria for Graves' Ophthalmopathy. Limitation of elevation with contralateral hypertropia, previously called double elevator palsy. Oblique muscle weakening is the preferred approach in the presence of oblique muscle overactions. 2015;19:e14. Dysfunction of the fourth cranial nerve (trochlear nerve), which innervates the superior oblique muscle (SOM), is one cause of paralytic strabismus. adalimumab) have been used in refractory cases. Graves' ophthalmopathy. Brown Syndrome. Overelevation or overdepression in adduction (measuring oblique muscle overaction). Simultaneous superior oblique tenotomy and inferior oblique recession in Brown's syndrome. A next step in naming and classification of eye movement disorders and strabismus. Urrets-Zavalia2 first described the need to identify vertical incomitance in a comitant horizontal strabismus in 1948. As it is a painful test, it is difficult to perform in children without general anesthesia. Lid fissure: Restrictions may cause lid fissure narrowing, while a paresis causes lid fissure widening.[4]. The risk in this procedure is that the sutures may cut through the thin superior oblique tendon. Copyright 2023, StatPearls Publishing LLC. We would like to extend sincere thanks to Mr. Vinay Gupta, BSc Optometry, for the contribution of figures in this chapter. In a patient with hypertropia that worsens in left gaze and right head tilt is most compatible with a right superior oblique palsy. 1999;97:1023-109. Secondary to an ipsilateral superior oblique paresis or a contralateral superior rectus paresis. Arch Ophthalmol. 2013. doi:10.1016/j.ophtha.2013.04.009, Lee AG. Other features: Intorsion and abduction in downgaze. Jack J. Kanski- Brad Bowling, Clinical Ophthalmology- A systematic approach, Seventh Edition, Elsevier, 2011. Instruction Courses and Skills Transfer Labs, Program Participant and Faculty Guidelines, LEO Continuing Education Recognition Award, What Practices Are Saying About the Registry, Provider Enrollment, Chain and Ownership System (PECOS), Subspecialty/Specialized Interest Society Directory, Subspecialty/Specialized Interest Society Meetings, Minority Ophthalmology Mentoring Campaign, Global Programs and Resources for National Societies, Patient-Reported Outcomes with LASIK Symptoms and Satisfaction, Steeper corneas and allergies may lead to faster keratoconus progression in kids, ROP treated with ranibizumab or low-dose bevacizumab may need re-treatment, Effect of Overminus Lens Therapy on Myopia Progression, Update on Atropine in Pediatric Ophthalmology, Peripheral Defocus Contact Lenses for Myopia Progression, International Society of Refractive Surgery. Patients can also develop a compensatory head tilt in the direction away from the affected muscle. Likewise, pseudo V-exotropia may be seen in intermittent divergent strabismus, wherein the patient fuses for downgaze and breaks in upgaze, manifesting exodeviation. (Courtesy of Vinay Gupta, BSc Optometry), Figure 6. [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. Leibovitch I, Wormald P, Iatrogenic Brown's Syndrome During Endoscopic Sinus Surgery With Powered Instruments. This procedure may cause iatrogenic Brown syndrome. Castro O, Johnson LD, Mamourian AC. 2020 Jan;117(1):1-18. doi: 10.1007/s00347-019-00988-4. If the pattern is significant, or the patient is symptomatic, it necessitates intervention. Miller MM, Guyton DL. 2011. doi:10.1001/archophthalmol.2011.335, Parulekar M V, Dai S, Buncic JR, Wong AMF. By convention, the misalignment is typically labelled by the higher, or hypertropic, eye. The Academy uses cookies to analyze performance and provide relevant personalized content to users of our website. [2] Some advocate labelling vertical misalignments based on if the deviated eye manifests as a hypertropia or hypotropia. Evaluation of ocular torsion and principles of management. In the case of a coexisting DVD, particular care has to be taken since SO weakening procedures may worsen this entity. It manifests when binocular fusion is interrupted either by occlusion or by spontaneous dissociation. Google Scholar. Subjects: We studied 33 eyes with oblique dysfunction (9 with presumed congenital superior oblique palsy [SOP], 13 with acquired SOP, 7 with Brown syndrome, and 4 with inverted Brown . The procedure of choice is the recession of affected muscles. When these palsies persist, they are typically responsive to prism treatment as they tend to cause comitant deviations. : 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. CN IV has the longest intracranial course and is vulnerable to damage, even with relatively mild trauma. Superior oblique tightening procedures - "tucks"- are indicated in congenital SO palsy with tendon laxity tested through forced duction or when there is minimal IO overaction with the vertical deviation being greatest in downgaze. Clark RA, Miller MJ, Rosenbaum AL, Demer JL. When the eye is adducted, the muscle plane and the visual axis align and the primary action is as a depressor. Ophthalmologe. Miller JE. The majority of patients have a congenital form of the syndrome but acquired inflammatory cases have been . Pediatric Ophthalmology and Strabismus BCSC, Leo, 2011-2012. Etiology and outcomes of adult superior oblique palsies: a modern series. [4], Trauma This disorder results from a dysfunction in the tendon of the superior oblique muscle ( Hargrove, Fleming, & Kerr, 2004 ). The PubMed wordmark and PubMed logo are registered trademarks of the U.S. Department of Health and Human Services (HHS). If there is a large hypotropia in upgaze even in the case of a <8PD deviation in primary position: IR recession and an additional contralateral asymmetrical IR recession or contralateral SR recession may be indicated. official website and that any information you provide is encrypted Unauthorized use of these marks is strictly prohibited. Kim JH, Hwang JM. Gregersen E, Rindziunski E. Brown's syndrome. The pathophysiology is varied, with no clear consensus. The tree-step-test is not diagnostic when more than one muscle is affected or there is a restrictive cause; there are some situations where a false positive result can lead to a misdiagnosis: A paresis of more than one vertical muscle, contracture of the vertical recti, previous vertical muscle surgery, skew deviation, myasthenia gravis, dissociated vertical deviation and small vertical deviations associated with horizontal strabismus. Duane retraction . The key finding in Brown syndrome is limited elevation in AD-duction. Sharma P, Halder M, Prakash P. Torsional changes in surgery for A-V phenomena.