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Brown Syndrome

Sup Oblique Tendon Sheath Synd

Brown syndrome is a rare disorder of the eye distinguished by pathological changes of the superior oblique muscle and its tendon, resulting in the inability to elevate the eye while being in adduction. Both congenital and acquired forms are described, and the diagnosis can be made only after a thorough physical examination and a detailed patient history.


Presentation

Brown syndrome is a relatively rare condition of the eye apparatus, in which various factors render the superior oblique muscle unable to perform its respective function - elevation of the eye while being in adduction, principally as a result of mechanical limitation [1] [2] [3] [4]. Numerous conditions have been implicated in the pathogenesis of this ocular disorder, including juvenile and rheumatoid arthritis, systemic diseases (scleroderma, Sjogren's syndrome, systemic lupus erythematosus), orbital metastatic deposits, but also trauma, inflammation, and surgery, all leading to restriction of superior oblique tendon movement [1] [2] [3] [5]. On the other hand, many individuals develop Brown syndrome from birth, indicating that congenital forms of the disease also exist [1] [2] [4] [5]. Furthermore, familial Brown syndrome, estimated to occur in approximately 1 in 20,000 live births, was also recognized in the literature [1] [3]. The clinical presentation is characterized by the inability of patients to elevate the eye in adduction, producing predominantly unilateral (although 10% of cases exhibit bilateral) changes in eye movement [1] [2] [3]. Consequently, a slight downward deviation of the eye on adduction is noted [1] Studies did not show a predilection toward gender or either of the eyes [1]. In the setting of the congenital Brown syndrome, spontaneous resolution is seen in 11-76% of children, but complications such as hypertropia, amblyopia, and other visual deficits may develop [1] [4] [6].

Localized Edema
Abnormal Eye Movement
  • Does Brown syndrome cause eye problems besides abnormal eye movements? In the more severely affected cases of Brown syndrome, some children will have poor binocular vision which can result in poor depth perception and/or amblyopia.[aapos.org]
  • While most patients are asymptomatic, some adopt an anomalous head position and others have a very noticeable abnormal eye movement pattern that necessitates the lengthening of the superior oblique tendon, generally with good outcomes.[link.springer.com]
Eye Pain
Cranial Neuropathy
  • Herpes zoster ophthalmicus can be associated with a variety of ocular and visual sequelae, including isolated or even multiple cranial neuropathies, potentially affecting the oculomotor, trochlear, or abducens nerves.[ncbi.nlm.nih.gov]
Frontal Headache
  • By age 5 years, he complained to his mother of daily, intermittent diplopia, eye pain in both eyes (OU), and frontal headaches. Additional strabismus surgeries OU were done at ages 6¾ years and 8¼ years.[ncbi.nlm.nih.gov]

Workup

The diagnosis of Brown syndrome can be made only after an extensive workup. Because the initial diagnosis of an eye-related pathology might be made by a simple evaluation of eye movement, the role of a properly performed physical examination is pivotal in recognizing this condition. Apart from the obvious inability to perform eye elevation while being in adduction, several other features that are seen during the exam are a downward deviation in adduction, widening of the palpebral fissure when the patient adducts the eye and the absence of over-activation of the superior oblique muscle [1]. Additionally, the forced duction test, during which the limbus is grasped by a forceps with the attempt to move the eye mechanically, will be positive - restricted movement will be observed due to the mechanical nature of the disease. In that case, the diagnosis of Brown syndrome is almost definite [1]. To identify the exact cause of this condition, a thorough patient history is helpful in distinguishing between congenital and acquired forms. Age of onset, as well as a history of previous conditions or events that could lead to Brown syndrome, should be assessed. Imaging studies, such as computed tomography (CT) and magnetic resonance imaging (MRI) have been regarded as potentially useful tools in solidifying the diagnosis [5]. Thickening of the superior oblique tendon on T1 and high signal intensity on T2-weighed imaging have been reported in this condition [5].

Treatment

  • Techniques and results of treatment for Brown syndrome at two institutions were reviewed.[ncbi.nlm.nih.gov]
  • BACKGROUND: The treatment of Brown syndrome has been undergoing an evolution toward more effective procedures with fewer operative interventions.[ncbi.nlm.nih.gov]
  • Antibiotic treatment is often insufficient for resolution. Surgical correction can effectively prevent amblyopia and restore binocular vision. [J Pediatr Ophthalmol Strabismus. 2019;56:e17-e19.].[ncbi.nlm.nih.gov]
  • Because of the chance for spontaneous resolution, conservative management, not surgery, should be the first line of treatment for acquired Brown's syndrome.[ncbi.nlm.nih.gov]
  • Its recognition may be a critical step during the treatment planning and may determine presumably the success of future therapies.[ncbi.nlm.nih.gov]

Prognosis

  • If the cervical spinal cord lesion is incomplete, such as central cord or Brown-Séquard syndrome, younger patients with have a more favorable prognosis for recovery.[emedicine.medscape.com]
  • Prognosis The prognosis for Brown-Séquard's syndrome is generally poor although it may be better than other forms of spinal cord injury. [ 13 ] Aetiology can have a bearing on prognosis.[patient.info]
  • Preoperative 1 month 12 month P AER (0-3) 2,88 0,92 0,59 Torticollis (%) 79.1 8 4 Triamcinolone injection in trochlea was not related with the prognosis, the existence of torticollis, or the value of AER (P 0.05).[article.sciencepublishinggroup.com]
  • PMID: 19085429 Prognosis Dotan G, Eiger-Moscovich M, Snir M, Ehrenberg M, Friling R J Pediatr Ophthalmol Strabismus 2018 Mar 1;55(2):113-116. Epub 2017 Dec 19 doi: 10.3928/01913913-20171101-02.[ncbi.nlm.nih.gov]
  • Treatment and prognosis Prognosis of anterior cord syndrome is worst among all other spinal cord injury syndromes 5. It is associated with high mortality and poor functional outcome in terms of poor recovery of motor power and coordination.[radiopaedia.org]

Etiology

  • Acquired nonsuperior oblique Brown syndrome Etiology of acquired nonsuperior oblique Brown syndrome Acquired nonsuperior oblique etiologies: Inferior orbital fibrous adhesions to the posterior globe are caused by the following: orbital floor fracture[emedicine.com]
  • Congenital Brown syndrome may be associated with an absent trochlear nerve and hypoplastic superior oblique muscle suggesting an etiologic mechanism of congenital cranial dysinnervation disorder.[ncbi.nlm.nih.gov]
  • Occurrences of Brown syndrome are rare, hence the difficulty in establishing the exact etiology.[ncbi.nlm.nih.gov]
  • AIMS: Our aim was to elucidate the etiology of Brown syndrome by evaluating the trochlea position, morphologic characteristics of the extraocular muscles including superior oblique muscle/tendon complex, and the presence of the cranial nerves (CN) III[ncbi.nlm.nih.gov]

Epidemiology

  • Epidemiology [ edit ] In Brown's original series there was a 3:2 predominance of women to men and nearly twice as many cases involved the right eye as the left. 10% of cases showed bilaterality.[en.wikipedia.org]
  • Epidemiology [ edit ] Brown-Séquard syndrome is rare as the trauma would have to be something that damaged the nerve fibres on just one half of the spinal cord. [3] History [ edit ] Charles-Édouard Brown-Séquard studied the anatomy and physiology of the[en.wikipedia.org]
Sex distribution
Age distribution

Pathophysiology

  • Management in Brown syndrome should be tailored to the pathophysiology of the individual patient.[ncbi.nlm.nih.gov]
  • CT is a valuable tool in understanding the pathophysiology and management of acquired Brown syndrome, showing thickening and inflammatory changes of the reflected portion of the superior oblique tendon.[ncbi.nlm.nih.gov]
  • The pathophysiology of Brown-McLean syndrome is not fully understood.[ncbi.nlm.nih.gov]
  • Pathophysiology The pure Brown-Séquard's syndrome reflecting hemisection of the cord is rarely seen. However, a clinical picture with some of the features of the syndrome is more common.[patient.info]
  • […] blepharoplasty, trauma, focal metastatic lesions, preseptal cellulitis, hypogammaglobulinaemia, Hurler Schies, combined lichen sclerosus et atrophicus and morphea, psoriasis. [2] , [3] , [4] , [5] , [6] , [7] , [8] , [9] It has been suggested that the pathophysiology[ijo.in]

Prevention

  • Surgical correction can effectively prevent amblyopia and restore binocular vision. [J Pediatr Ophthalmol Strabismus. 2019;56:e17-e19.].[ncbi.nlm.nih.gov]
  • Surgical exploration revealed adhesions that prevented the normal sliding of the superior oblique tendon beneath the superior rectus muscle. Forced ductions became normal and downgaze improved after removal of the silicone band.[ncbi.nlm.nih.gov]
  • In all cases, the child’s vision is closely monitored to prevent other problems down the road. Takeaways Having one eye that cannot move and ends up stuck in the upward position could be diagnosed as Brown syndrome.[bundoo.com]
  • This prevents the lower eye from raising the eye inward. Brown’s Syndrome can be congenital or acquired in adulthood. It may be constant or intermittent. In 90% of cases, only one eye is affected, more often, the right one.[mariaelisascarale.it]
  • The combination of a sheath-stenosis and tendon swelling prevents movements of the tendon.[emedicine.com]

References

Article

  1. Olusanya BA. Brown syndrome with severe amblyopia: a case report from Africa. Pan Afr Med J. 2015;20:56.
  2. Cho YA, Kim S, Graef MH. Surgical Outcomes in Correction of Brown Syndrome. Korean J Ophthalmol. 2006;20(1):33-40.
  3. Kenawy N, Pilz DT, Watts P. Familial unilateral Brown syndrome. Indian J Ophthalmol. 2008;56(5):430-434.
  4. Lambert SR. Late spontaneous resolution of congenital Brown syndrome. J AAPOS. 2010;14(4):373-375.
  5. Currie S, Goddard T. MR imaging features of acquired Brown syndrome. AJNR Am J Neuroradiol. 2009;30(9):1778-1779.
  6. Kent JS, Makar I. Hypertropia following Spontaneous Resolution of Brown’s Syndrome. Case Rep Ophthalmol. 2012;3(2):230-235.

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Last updated: 2019-07-11 21:56