Statement of Ethics Written informed consent was obtained from the patient for publication of this report and any presented images. [karger.com]

To date, there has been no report of a child with ectopia lentis as the only presentation of an intraocular tumor. We present 2 children with malignant medulloepitheliomas who presented in this fashion. [ncbi.nlm.nih.gov]

Lally, M.D., and Julia Monsonego, C.R.A. 2 Citing Articles A 27-year-old woman with a diagnosis of Marfan's syndrome presented with worsening vision and glare in each eye for 1 month. On examination, visual acuity was 20/30 in each eye. [nejm.org]

• Pain

  Fever and severe neck pain An 85-year-old man with a 5-day history of persistent fever and severe neck pain presented to our hospital. No bacteria were identified in the culture test including cerebrospinal fluid. [thelancet.com]

  After that, intermittent ocular pain and visual acuity of the left eye were improved. Genetic testing confirmed an FBN1 gene mutation in the patient. [synapse.koreamed.org]

  He is unsure if he lost consciousness but complains of severe pain on the back of his head, left side of his face, and in his left eye. His initial Glasgow Coma Scale was 15 per paramedic report. [tamingthesru.com]

  Pain Medications Your doctor may prescribe medications to treat joint pain or the pain of dural ectasia, a swelling of the membrane that surrounds the spinal cord. Sometimes, an over-the-counter pain medication, such as ibuprofen, is recommended. [nyulangone.org]

  A 56-year-old female previously diagnosed with SWS presented to the outpatient clinic complaining of right-sided decreased visual acuity and pain after a fall. [karger.com]

• Tall Stature

  MARFAN SYNDROME  Salient features:  Skeletal Manifestations  Tall stature  Increased arm span in relation to body height and elongated lower segment  Arachnodactyly  Joint laxity, scoliosis 11. [slideshare.net]

  Other features include tall stature with increased arm span, arachnodactyly, mitral valve prolapse, aortic dilation and dissection, scoliosis, and hyperextensible joints. [medilib.ir]

  All patients presented with typical features of Marfan’s syndrome like tall-statured with long, thin extremities; arachnodactly, prognathis and a high arched palate. [djo.org.in]

• Short Finger

  The systemic features can be short stature, short fingers of hands and toes, brachydactyly, and delayed milestones resulting in learning difficulty.[14] Homocystinuria This is the second most common etiology for inheritable ectopia lentis. [statpearls.com]

• Eruptions

  Additional clinical manifestations include delayed dental eruption and neurologic abnormalities [9]. [medilib.ir]

• Brachydactyly

  Weill-Marchesani syndrome: WMS is a connective tissue disorder characterized by abnormalities of the lens of the eye, proportionate short stature, brachydactyly, and joint stiffness. [entokey.com]

  Weill-Marchesani syndrome More Details is a rare connective tissue disorder either as autosomal dominant or recessive with short stature, brachydactyly, and EL. [9] Very few cases of autosomal recessive EL with hereditary chorioretinal disorders have [annalsofian.org]

  Joint stiffness WMS-like syndrome (OMIM 613195 ) ADAMTS17 AR Brachydactyly MSPKA (OMIM 251750 ) LTBP2 AR Megalocornea Glaucoma Microspherophakia Axial myopia Marfanoid features Aniridia PAX6 AD Iris hypoplasia Foveal dysplasia Optic nerve hypoplasia [ncbi.nlm.nih.gov]

  Spherophakia with brachydactyly. Comparison with Marfan's syndrome. Am J Ophthalmol. 1953; 36: 1571-1574 PubMed Google Scholar Prockop D.J. Sjoersdsma A Significance of urinary hydroxy-proline in man. [surveyophthalmol.com]

  The systemic features can be short stature, short fingers of hands and toes, brachydactyly, and delayed milestones resulting in learning difficulty.[14] Homocystinuria This is the second most common etiology for inheritable ectopia lentis. [statpearls.com]

• Contusion

  Luxation occurs in contusion of the globe, in many ocular (e.g. buphthalmos) and other diseases (e.g. syphilis) or it can be inherited (e.g. the bilateral, symmetrical, superior subluxation commonly found in Marfan's syndrome or homocystinuria). [medical-dictionary.thefreedictionary.com]

  Related pages: aetiology complications treatment contusion injury to the eye [gpnotebook.com]

  Arch Ophthalmol. 1969; 82: 267-268 PubMed Crossref Google Scholar Wolf SM Zimmerman LE Chronic secondary glaucoma associated with retrodisplacement of iris root and deepening of the anterior chamber angle secondary to contusion. [surveyophthalmol.com]

• Fracture

  (See "Orbital fractures" and "Open globe injuries: Emergency evaluation and initial management" and "Traumatic hyphema: Clinical features and diagnosis".) [medilib.ir]

  […] remainder of his examination is within normal limits. imaging CT scan of the head, face, and cervical spine demonstrate a left lens dislocation with pre-septal soft tissue swelling surrounding the left globe and overlying the left maxilla without underlying fracture [tamingthesru.com]

• Iridodonesis

  L21) See corectopia ; iridodonesis ; pupillary block. Fig. [medical-dictionary.thefreedictionary.com]

  In younger children, examination with a penlight may demonstrate tremulousness of the iris (iridodonesis). After dilation of the pupils, the edge of the lens may be seen directly or on retroillumination. [medilib.ir]

  Megalocornea, With Ectopia Lentis And With Or Without Secondary Glaucoma Synonyms - Classification developmental, eye, genetic Phenotypes Autosomal recessive inheritance ; Deep anterior chamber ; Ectopia lentis ; Glaucoma ; High palate ; Hypermetropia ; Iridodonesis [mousephenotype.org]

  In the setting of lens dislocation, the patient may have trembling or shimmering of either the lens or iris, phakodenesis or iridodonesis, respectively. [tamingthesru.com]

• Strabismus

  J Pediatr Ophthalmol Strabismus. 2008 Jan-Feb. 45(1):12. [QxMD MEDLINE Link]. Omulecki W, Wilczynski M, Gerkowicz M. Management of bilateral ectopia lentis et pupillae syndrome. Ophthalmic Surg Lasers Imaging. 2006 Jan-Feb. 37(1):68-71. [emedicine.medscape.com]

  Pediatric ophthalmology and strabismus. 2nd ed. New York: Springer; 2003. p. 584–95. CrossRef Google Scholar 39. Simon JW. Basic and clinical science course. In: Section 6: pediatric ophthalmology and strabismus. [link.springer.com]

  J Pediatr Ophthalmol Strabismus. 2008 Jan-Feb. 45(1):12. [Medline]. Omulecki W, Wilczynski M, Gerkowicz M. Management of bilateral ectopia lentis et pupillae syndrome. Ophthalmic Surg Lasers Imaging. 2006 Jan-Feb. 37(1):68-71. [Medline]. [emedicine.com]

• Diplopia

  Subluxation is one of the causes of monocular diplopia. If the luxation is complete the eye becomes markedly hyperopic and is unable to accommodate. [medical-dictionary.thefreedictionary.com]

  While most clinicians immediately think of neurological problems when diplopia is mentioned, monocular diplopia is always attributed to some type of media issue, such as a cataract, corneal opacity or subluxated lens. [reviewofoptometry.com]

  Patients may also complain of monocular diplopia and are at risk of developing amblyopia, glaucoma, and retinal detachment. Ectopia lentis can be inherited as an isolated condition, usually autosomal dominant (simple ectopia lentis). [visualdx.com]

  Symptoms of ectopia lentis include monocular diplopia and visual distortion. Symptoms vary based on the extent of displacement, but complete displacement may result in marked visual blurring. [tamingthesru.com]

• Blepharoptosis

  NIH GARD Information: Blepharoptosis myopia ectopia lentis This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD). [rarediseases.org]

  110150 BLEPHAROPTOSIS, MYOPIA, AND ECTOPIA LENTIS ORPHA: 1259; TEXT Gillum and Anderson (1982) described a family in which a 72-year-old woman and 2 of her daughters showed blepharoptosis from birth, high grade myopia, and ectopia lentis, present in one [omim.org]

  A previously undescribed syndrome of bilateral blepharoptosis, ectopia lentis, and high myopia apparently was dominantly inherited and originated from a spontaneous genetic mutation. [ncbi.nlm.nih.gov]

  3MC syndrome 1 acrocephalosyndactylia + Arthrogryposis, Cleft Palate, Craniosynostosis, and Impaired Intellectual Development Aurocephalosyndactyly Baller-Gerold syndrome Baraitser Rodeck Garner syndrome Beare-Stevenson cutis gyrata syndrome Blepharoptosis [rgd.mcw.edu]

  Ocular Associations  Persistent pupillary membrane  Aniridia  Rieger’s syndrome  Dominantly inherited blepharoptosis  High myopia  Congenital glaucoma/buphthalmos  Pseudoexfoliation  Retinitis pigmentosa  Hypermature cataract  Intraocular tumor [slideshare.net]

• Eye Pain

  If a child has a history of intermittent eye pain and a condition associated with lens subluxation, the eye pain may represent intermittent angle closure glaucoma secondary to lens subluxation into the anterior chamber. [medilib.ir]

• Pierre Robin Syndrome

  Crouzon syndrome Pierre Robin syndrome Sprengel deformity Video Ectopia Lentis Surgery This 46 year old woman has had progressive vision loss from ectopia lentis which is more difficult to correct. [retinavitreous.com]

  […] dysostosis; Sturge-Weber syndrome; Conradi syndrome; Pfaundler syndrome; Pierre Robin syndrome; Wildervanck syndrome; Sprengel deformity. [emergency-live.com]

  Ehlers-Danlos syndrome Sturge-Weber syndrome Mandibulofacial dysostosis Wildervanck syndrome Conradi syndrome Pfaundler syndrome Crouzon syndrome Pierre Robin syndrome Sprengel deformity History A history of any recent trauma is of principal importance [eyewiki.aao.org]

• Tremulousness

  In younger children, examination with a penlight may demonstrate tremulousness of the iris (iridodonesis). After dilation of the pupils, the edge of the lens may be seen directly or on retroillumination. [medilib.ir]

  Freed from its ligamentous attachment, the lens, besides getting dislocated assumes a more spherical form, becomes tremulous and gets tilted. [ijo.in]

  In younger children less cooperative with a slit lamp examination, examination with a penlight may demonstrate tremulousness of the iris (iridodonesis) [14] Grading systems for ectopia lentis have been proposed to facilitate and standardize studies, as [eyewiki.aao.org]

• Neglect

  A neglected cause of secondary glaucoma in eyes in which the lens is absent or subluxated. Arch Ophthalmol. 1947; 47: 740-771 Crossref Google Scholar Chase H.P. Goodman S.I. O'Brien D Treatment of homocystinuria. [surveyophthalmol.com]

• Paresis

  Pediatrics. 1967; 40: 586-589 PubMed Google Scholar Stadlin W Klein D Ectopie congenitale du crystallin avec spherophaquie et brachymorphie accompagnee de paresis du regard. [surveyophthalmol.com]

Workup  Cardiac evaluation for Marfan syndrome  Serum and urine levels of homocysteine or methionine for homocystinuria  Axial length measurement 32. [slideshare.net]

The patient received topical bromfenac 0.09% three times a day for 6 weeks for CME treatment. After treatment, macular central thickness decreased to 220 µm and BCVA improved significantly to 20/30 after contact lens rehabilitation. [karger.com]

Prophylactic lensectomy has proven to be the most reliable treatment in our experience. [ncbi.nlm.nih.gov]

Treatment Treatment Options: Glaucoma, retinal detachments, and cataracts may require surgery. References Christensen AE, Fiskerstrand T, Knappskog PM, Boman H, Rodahl E. A novel ADAMTSL4 mutation in autosomal recessive ectopia lentis et pupillae. [disorders.eyes.arizona.edu]

Treatment and prognosis Complications An anterior dislocation/subluxation carries greater complications than a posterior one. Such complications include: acute glaucoma corneal injury iridal injury Promoted articles (advertising) [radiopaedia.org]

Prognosis Depending on the degree of lens dislocation, the age of onset, and its associated secondary complications, most patients do well. [emedicine.medscape.com]

Prognosis Visual prognosis depends on the degree of lens dislocation, the age of onset, age of treatment and complications. [orpha.net]

Prognosis Most patients do well and at least 85% have 20/40 or better visual acuity after treatment. [bceye.com]

Mortality/Morbidity Ectopia lentis may cause marked visual disturbance, which varies with the degree of lens displacement and the underlying etiologic abnormality. [emedicine.medscape.com]

The commonest atraumatic etiologies are Marfan syndrome and homocystinuria. [radiopaedia.org]

This was recently reported in monozygotic twin sisters, supporting a genetic etiology for this syndromic association. We report on female first cousins once removed who were born with unilateral coronal synostosis. [ncbi.nlm.nih.gov]

As ectopia lentis may be the first sign of a more serious systemic disease, it is important to determine the etiology for appropriate patient management. [eyewiki.aao.org]

Epidemiology Frequency United States Ectopia lentis is a rare condition. Incidence in the general population is unknown. The most common cause of ectopia lentis is trauma, which accounts for nearly one half of all cases of lens dislocation. [emedicine.medscape.com]

Keywords: anterior segment • clinical (human) or epidemiologic studies: pre • linkage analysis © 2003, The Association for Research in Vision and Ophthalmology, Inc., all rights reserved. [iovs.arvojournals.org]

Pathophysiology Disruption or dysfunction of the zonular fibers of the lens, regardless of cause (trauma or heritable condition), is the underlying pathophysiology of ectopia lentis. [emedicine.medscape.com]

These findings should encourage earlier surgery when indicated to prevent amblyopia and improve visual acuity. [ncbi.nlm.nih.gov]

A nonsurgical alternative involves the use of a miotic to constrict the pupil and prevent the lens from luxating into the anterior chamber. [premilife.com]

Beta Blockers These medications, taken by mouth, lower blood pressure, which can prevent or slow the enlargement of the aorta. [nyulangone.org]

Early treatment can prevent secondary glaucoma. Lens subluxation Lens subluxation is also observed in dogs and is characterised by partial displacement of the lens. [emergency-live.com]