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].
Entire Body System
- Inflammation
Secondary cases, due to inflammation in the trochlear notch can benefit from steroid treatment. [eyewiki.aao.org]
Magnetic resonance imaging orbit with contrast was advised and did not show any mass lesion or evidence of inflammation in the trochlear region. [ojoonline.org]
Acquired Brown’s Syndrome Acquired Brown’s syndrome is usually caused by an inflammation around the superior oblique tendon and trochlea. There may be pain and tenderness in the superior nasal quadrant, with the condition often being intermittent. [wrighteyecare.com]
In that condition the inflammation can involve tendons and various tissues other than the joint surfaces. [irishhealth.com]
[…] mild: failure of elevation in ADduction - moderate: downshoot of eye in aDduction - severe: hypotropia in primary Causes : - congenital - idiopathic, impaired tendon movement through trochlear - acquired - trauma to trochlear ie dog bite, surgical - inflammation [sites.google.com]
- Weakness
Definition Treatment Prognosis Clinical Trials Organizations Publications Definition Brown-Sequard syndrome (BSS) is a rare neurological condition characterized by a lesion in the spinal cord which results in weakness or paralysis (hemiparaplegia) on [ninds.nih.gov]
Treatment with dexamethasone recovered weakness and sensations. [n.neurology.org]
facial palsy, neck and shoulder weakness, upper motor neuron signs, and ataxia. [pediatricneurosciences.com]
The most frequent presenting symptoms are cranial neuropathy, sensory ataxia, muscle weakness and respiratory insufficiency due to diaphragmatic paralysis. [orpha.net]
Typically, the first symptoms of nerve degeneration are generalised weakness and balance problems. [facialpalsy.org.uk]
- Asymptomatic
Postoperatively, the patient was asymptomatic in all diagnostic gaze positions. [ncbi.nlm.nih.gov]
Diagnostic Criteria Many patients with type 1 ECG pattern are asymptomatic. [aerjournal.com]
No clear-cut recommendations have been defined for risk stratification in asymptomatic patients. [onlinejacc.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]
Admittedly study sizes are pretty small – but one study followed 98 asymptomatic Japanese patients with ‘Brugada sign’ on routine ECG for 7.8 years and found them to have no greater mortality than the rest of a 14000 strong cohort. [litfl.com]
- Head Tilted Backward
Brown characterized the syndrome in many ways such as: Limited elevation in the eye when head is straight up Eyes point out in a straight up gaze (divergence in up gaze) Widening of the eyelids in the affected eye on adduction Head tilts backwards (compensatory [ophthnotes.com]
Brown characterized the syndrome in many ways such as:[2] Limited elevation in the eye when head is straight up Eyes point out in a straight up gaze (divergence in up gaze) Widening of the eyelids in the affected eye on adduction Head tilts backwards [en.wikipedia.org]
- Short Stature
Males may develop large calcium-containing Sertoli cell tumors (LCST) of the testicles, which secrete estrogen and can lead to enlarged breasts (gynecomastia), advanced skeletal age, and ultimately short stature, if untreated. [cancer.net]
Gastrointestinal
- Vomiting
Other signs and symptoms in newborns may include: Swollen belly Vomiting, including vomiting a green or brown substance Constipation or gas, which might make a newborn fussy Diarrhea In older children, signs and symptoms can include: Swollen belly Chronic [mayoclinic.org]
The infant is cyanosed, acidotic, has cold peripheries and has the signs of all of marked hyponia, poor feeding, vomiting, loose stools and a distended abdomen. Fig. 1 Brownish pigmentation seen over the abdomen. [indianpediatrics.net]
Monitor your newborn for vomiting or stomach upset. Keep an eye on the situation to be sure that it's getting better. Call your doctor if it does not begin to clear up within a few days. [verywellfamily.com]
[…] painkillers such as paracetamol to bring it down; get medical advice as soon as possible if this doesn't help drinking too much alcohol – avoid drinking lots of alcohol in a short space of time dehydration – get medical advice if you have diarrhoea or vomiting [nhs.uk]
- Abdominal Pain
Signs and symptoms include cramping, abdominal pain, bloating, gas, and diarrhea or constipation, or both. IBS is a chronic condition that you'll need to manage long term. Only a small number of people with IBS have severe signs and symptoms. [mayoclinic.org]
Other symptoms are sometimes reported, including gastrointestinal complaints, fatigue, weakness or abdominal pain. However, it is not clear that these symptoms are directly related to higher levels of bilirubin. [betterhealth.vic.gov.au]
Some EDS patients experience abdominal pain and gut abnormalities, Sarmad Almansour, M.D., an internal medicine and rheumatology specialist at Detroit Medical Center's Sinai-Grace Hospital, tells SELF. [self.com]
No fever, loss of appetite, abdominal pain, jaundice, behavioral changes were noted. There were no other constitutional symptoms like night sweats, chills, chest pain and easy fatigability suggestive of Lymphoma. [clinmedjournals.org]
- Nausea
Case presentation A 13-year-old girl, presented to the Emergency Department with a 4-day history of increasing right lower quadrant (RLQ) and hypogastric pain, nausea and sporadic vomiting without fever, diarrhoea or urinary symptoms. [ncbi.nlm.nih.gov]
Headaches were associated with increased discoloration of facial PWS, auras, nausea/vomiting, dysarthria, dizziness, and feelings of facial pulsation. Table 2. [emedicine.medscape.com]
Musculoskeletal
- Fracture
When repairing medial orbital wall fracture that causes Brown syndrome, surgeons should always be careful of entrapment of the superior oblique muscle if the implant is inserted without identifying the superior and posterior margin of the orbital fracture [ncbi.nlm.nih.gov]
None has been observed in such a high-energy fracture/dislocation pattern. Despite the severity of the fracture, the patient recovered fully at 6 months follow-up. [asianjns.org]
- Torticollis
Preoperative torticollis was observed in 79.1% patients. The most common type was torsional torticollis (66.6%). Horizontal torticollis occurred in 5 cases, 3 of them associated with torsional torticollis. [article.sciencepublishinggroup.com]
optical torticollis and other things we can barely pronounce. [jendawnscowgirlup.blogspot.com]
Keywords Brown syndrome Ocular torticollis Anomalous head posture Inferior oblique palsy Fibrosis of extraocular muscles References 1. Brown HW. Congenital structural muscle anomalies. In: Allen JH, editor. Symposium on strabismus. [link.springer.com]
Apparent inferior rectus palsy with gaze-dependent sensory adaptations. 61 Kimlin JA...Sampson GP 24811845 2014 47 Clinical progression of untreated bilateral Brown syndrome. 61 Sorrentino D...Warman R 24698613 2014 48 Effect of strabismus surgery on torticollis [malacards.org]
Eyes
- Strabismus
Disease In 90% of patients the disease is unilateral, while hereditary predisposition is present in some cases, like most cases of childhood strabismus. [eyewiki.aao.org]
Postoperative effect on abnormal head posture, vertical strabismus in primary gaze, vertical strabismus into affected side gaze, and elevation in adduction. RESULTS: The mean duration of follow-up was 30 months (range, 10-72 months). [ncbi.nlm.nih.gov]
Symposium on strabismus. Trans New Orleans Acad Ophthalmol. St Louis: CV Mosby; 1950. p. 205–36. Google Scholar 2. von Noorden GK, Campos EC. Special forms of strabismus. In: von Noorden GK, Campos EC, editors. [link.springer.com]
H50.61 Brown's sheath syndrome H50.612 …… left eye H50.69 Other mechanical strabismus H50.8 Other specified strabismus H50.89 Other specified strabismus H50.9 Unspecified strabismus H51 Other disorders of binocular movement H51.0 Palsy (spasm) of conjugate [icd10data.com]
The presence of inelastic or tethered superior oblique muscle-tendon can be diagnosed without forced duction testing by observing the pattern of strabismus including torsion. [scienceopen.com]
- Diplopia
Also, it may be attempted in other incomitant strabismic deviations with bothersome diplopia limited to specific positions of gaze. [ncbi.nlm.nih.gov]
One day postoperative The patient reported blur in the right eye, but no diplopia. Cover test revealed a minimal left hypotropia without diplopia, which was controlled to a phoria with a slight head tilt left AHP. [bioj-online.com]
- Esotropia
In the primary gaze, she presented with 10PD esotropia (ET) and 6PD HOT in the left eye at distance, and 35PD ET' and 6PD HOT' at near. A diagnosis of Brown's syndrome and accommodative esotropia was made. [synapse.koreamed.org]
At the most recent follow-up visit, 6 patients remained orthophoric in primary position; 1 patient had a micro-esotropia; 1 had developed an exotropia of 30 D; and 1 had developed a right hyperphoria. [ncbi.nlm.nih.gov]
Methods: A 41-year-old female patient presented with right hypertropia and esotropia. We reviewed her history, conducted an ophthalmic examination and performed surgery. [ophthalmology.org]
Our purpose here was to determine long term binocular sensory adaptation in Brown’s syndrome using data obtained from motion symmetry visually evoked potentials (MSVEP ) (Norcia, et al, Anomalous motion VEPs in infants and infantile esotropia. [iovs.arvojournals.org]
- Iridodonesis
Earlier reports hypothesised that iridodonesis in aphakic patients could result in intermittent peripheral endothelial abrasion, hence a superior iridectomy is protective of the superior cornea. 3 5 However Almousa et al 7 and Lim et al separately reported [ncbi.nlm.nih.gov]
Neurologic
- Hyperactivity
This however is not the case since during upwards turn we observe "V" phenomenon and not "A" as it would have been the case in an actual superior oblique hyperactivity. [eyewiki.aao.org]
They might: have trouble learning have attention problems and hyperactivity be shorter than other children their age The signs and symptoms of Legius syndrome can look like those of a more serious genetic problem called neurofibromatosis. [kidshealth.org]
Symptoms of hyperthyroidism can include: hyperactivity difficulty breathing mood swings difficulty sleeping lethargy (lack of energy) muscle weakness the need to pass stools or urine frequently Increased susceptibility to infection People with Down's [hse.ie]
These include moderate to severe intellectual disability, epilepsy, inability to walk and talk or difficulty doing so, attenuated head and brain growth, and hyperactivity. [news.brown.edu]
Spasticity and hyperactive reflexes may not be present with an acute lesion. Aetiology The causes of this syndrome are: Most commonly, trauma (penetrating or blunt).[2] Neoplasia (spinal cord tumour - either metastatic or primary). [patient.info]
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
Secondary cases, due to inflammation in the trochlear notch can benefit from steroid treatment. [eyewiki.aao.org]
Techniques and results of treatment for Brown syndrome at two institutions were reviewed. [ncbi.nlm.nih.gov]
Other treatment is symptomatic and supportive. × Treatment Generally treatment for individuals with BSS focuses on the underlying cause of the disorder. Early treatment with high-dose steroids may be beneficial in many cases. [ninds.nih.gov]
The various etiologic theories are reviewed and the spectrum of medical and surgical treatments are described and evaluated. [scienceopen.com]
Prognosis
Prognosis The prognosis for individuals with BSS varies depending on the cause of the disorder. x Prognosis The prognosis for individuals with BSS varies depending on the cause of the disorder. [ninds.nih.gov]
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]
10–15% functional recovery; if no recovery is evident and progressive after 24 hours, prognosis is poor Vibration and proprioception are typically spared because of an intact dorsal column! [amboss.com]
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]
In terms of etiology, Brown syndrome has two distinct forms – congenital and acquired. [ojoonline.org]
Occurrences of Brown syndrome are rare, hence the difficulty in establishing the exact etiology. [ncbi.nlm.nih.gov]
Further workup may be needed in acquired Brown syndrome and often depends on the suspected underlying etiology. [eyewiki.aao.org]
Epidemiology
Terminology Also called sideropenic dysphagia Epidemiology Occurs mostly in Scandinavia and Great Britain Usually women ages 30+ years Treatment Iron supplements, mechanical dilation and monitor for upper GI carcinoma Clinical images Images hosted on [pathologyoutlines.com]
To mark the occasion of World Down Syndrome Day, the JRC publishes a report describing the epidemiology of Down syndrome in Europe over the last 25 years. [ec.europa.eu]
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]
Pathophysiology
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]
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. Article History Published in print: 1985 [pubs.rsna.org]
In: Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, Management. 10th ed. Philadelphia, Pa.: Saunders Elsevier; 2016. https://www.clinicalkey.com. Accessed May 12, 2017. AskMayoExpert. [mayoclinic.org]
Prevention
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]
Prognosis and prevention[3, 12] Correction of iron deficiency will correct the condition if malignancy has not yet occurred. Prevention of iron deficiency prevents the disease. Historical notes[2] Henry Plummer (1874-1937) was an American internist. [patient.info]
Things You Can Do For Hand-Foot Syndrome: Prevention: Prevention is very important in trying to reduce the development of hand-foot syndrome. Actions taken to prevent hand-foot syndrome will help reduce the severity of symptoms should they develop. [chemocare.com]
[…] excavatum, pectus carinatum, and slipping rib syndrome Research Interests Pediatric trauma; injury prevention; chest wall deformities Academic Affiliation Professor, UC Department of Surgery Departments Colorectal Disorders, Injury Prevention, Surgery [cincinnatichildrens.org]
References
- Olusanya BA. Brown syndrome with severe amblyopia: a case report from Africa. Pan Afr Med J. 2015;20:56.
- Cho YA, Kim S, Graef MH. Surgical Outcomes in Correction of Brown Syndrome. Korean J Ophthalmol. 2006;20(1):33-40.
- Kenawy N, Pilz DT, Watts P. Familial unilateral Brown syndrome. Indian J Ophthalmol. 2008;56(5):430-434.
- Lambert SR. Late spontaneous resolution of congenital Brown syndrome. J AAPOS. 2010;14(4):373-375.
- Currie S, Goddard T. MR imaging features of acquired Brown syndrome. AJNR Am J Neuroradiol. 2009;30(9):1778-1779.
- Kent JS, Makar I. Hypertropia following Spontaneous Resolution of Brown’s Syndrome. Case Rep Ophthalmol. 2012;3(2):230-235.