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abnormal position of a part or organ (especially at the time of birth)

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lendの過去・過去分詞

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/06/30 17:36:26」(JST)

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Anterior lens luxation in a dog

Anterior lens luxation with cataract formation in a cat

Ectopia lentis is a displacement or malposition of the eye's crystalline lens from its normal location. A partial dislocation of a lens is termed lens subluxation or subluxated lens; a complete dislocation of a lens is termed lens luxation or luxated lens.

Ectopia lentis in dogs and cats

Although observed in humans and cats, ectopia lentis is most commonly seen in dogs. Ciliary zonules normally hold the lens in place. Abnormal development of these zonules can lead to primary ectopia lentis, usually a bilateral condition. Luxation can also be a secondary condition, caused by trauma, cataract formation (decrease in lens diameter may stretch and break the zonules), or glaucoma (enlargement of the globe stretches the zonules). Steroid administration weakens the zonules and can lead to luxation as well. Lens luxation in cats can occur secondary to anterior uveitis (inflammation of the inside of the eye).

Anterior lens luxation

With anterior lens luxation, the lens pushes into the iris or actually enters the anterior chamber of the eye. This can cause glaucoma, uveitis, or damage to the cornea. Uveitis (inflammation of the eye) causes the pupil to constrict (miosis) and trap the lens in the anterior chamber, leading to an obstruction of outflow of aqueous humour and subsequent increase in ocular pressure (glaucoma).^[1] Better prognosis is valued in lens replacement surgery (retained vision and normal intraocular pressure) when it is performed before the onset of secondary glaucoma.^[2] Glaucoma secondary to anterior lens luxation is less common in cats than dogs due to their naturally deeper anterior chamber and the liquification of the vitreous humour secondary to chronic inflammation.^[3] Anterior lens luxation is considered to be an ophthalmological emergency.

Posterior lens luxation

With posterior lens luxation, the lens falls back into the vitreous humour and lies on the floor of the eye. This type causes fewer problems than anterior lens luxation, although glaucoma or ocular inflammation may occur. Surgery is used to treat dogs with significant symptoms. Removal of the lens before it moves to the anterior chamber may prevent secondary glaucoma.^[2]

Lens subluxation

Lens subluxation is also seen in dogs and is characterized by a partial displacement of the lens. It can be recognized by trembling of the iris (iridodonesis) or lens (phacodonesis) and the presence of an aphakic crescent (an area of the pupil where the lens is absent).^[4] Other signs of lens subluxation include mild conjunctival redness, vitreous humour degeneration, prolapse of the vitreous into the anterior chamber, and an increase or decrease of anterior chamber depth.^[5] Removal of the lens before it completely luxates into the anterior chamber may prevent secondary glaucoma.^[2] A nonsurgical alternative involves the use of a miotic to constrict the pupil and prevent the lens from luxating into the anterior chamber.^[6]

Breed predisposition

Terrier breeds are predisposed to lens luxation, and it is probably inherited in the Sealyham Terrier, Jack Russell Terrier, Wirehaired Fox Terrier, Rat Terrier, Teddy Roosevelt Terrier, Tibetan Terrier,^[7] Miniature Bull Terrier, Shar Pei, and Border Collie.^[8] The mode of inheritance in the Tibetan Terrier^[5] and Shar Pei^[9] is likely autosomal recessive. Labrador Retrievers and Australian Cattle Dogs are also predisposed.^[10]

Systemic associations in humans

In humans, there are a number of systemic conditions that are associated with ectopia lentis:^[11]

More common:

Marfan syndrome (upward and outward)^[12]
Homocystinuria (downward and inwards)^[12]
Weill–Marchesani syndrome
Sulfite oxidase deficiency
Hyperlysinemia

Less common:

Ehlers–Danlos syndrome
Crouzon disease
Refsum syndrome
Kniest syndrome
Mandibulofacial dysostosis
Sturge–Weber syndrome
Conradi syndrome
Pfaundler syndrome
Pierre Robin syndrome
Wildervanck syndrome
Sprengel deformity

References

^ Ketring, Kerry I. (2006). "Emergency Treatment for Anterior Lens Luxation" (PDF). Proceedings of the North American Veterinary Conference. Retrieved 2007-02-22.
^ ^a ^b ^c Glover T, Davidson M, Nasisse M, Olivero D (1995). "The intracapsular extraction of displaced lenses in dogs: a retrospective study of 57 cases (1984-1990)". Journal of the American Animal Hospital Association 31 (1): 77–81. PMID 7820769.
^ Peiffer, Robert L., Jr. (2004). "Diseases of the Lens in Dogs and Cats". Proceedings of the 29th World Congress of the World Small Animal Veterinary Association. Retrieved 2007-02-22.
^ "Lens". The Merck Veterinary Manual. 2006. Retrieved 2007-02-22.
^ ^a ^b Grahn B, Storey E, Cullen C (2003). "Diagnostic ophthalmology. Congenital lens luxation and secondary glaucoma". Canadian Veterinary Journal 44 (5): 427, 429–30. PMC 340155. PMID 12757137.
^ Binder DR, Herring IP, Gerhard T (2007). "Outcomes of nonsurgical management and efficacy of demecarium bromide treatment for primary lens instability in dogs: 34 cases (1990-2004)". Journal of the American Veterinary Medical Association 231 (1): 89–93. doi:10.2460/javma.231.1.89. PMID 17605669.
^ Gelatt, Kirk N., ed. (1999). Veterinary Ophthalmology (3rd ed.). Lippincott, Williams & Wilkins. ISBN 0-683-30076-8.
^ Petersen-Jones, Simon M. (2003). "Conditions of the Lens". Proceedings of the 28th World Congress of the World Small Animal Veterinary Association. Retrieved 2007-02-22.
^ Lazarus J, Pickett J, Champagne E (1998). "Primary lens luxation in the Chinese Shar Pei: clinical and hereditary characteristics". Veterinary Ophthalmology 1 (2-3): 101–107. doi:10.1046/j.1463-5224.1998.00021.x. PMID 11397217.
^ Johnsen D, Maggs D, Kass P (2006). "Evaluation of risk factors for development of secondary glaucoma in dogs: 156 cases (1999-2004)". Journal of the American Veterinary Medical Association 229 (8): 1270–4. doi:10.2460/javma.229.8.1270. PMID 17042730.
^ Eifrig CW, Eifrig DE. "Ectopia Lentis". eMedicine.com. November 24, 2004.
^ ^a ^b Peter Nicholas Robinson; Maurice Godfrey (2004). Marfan syndrome: a primer for clinicians and scientists. Springer. pp. 5–. ISBN 978-0-306-48238-0. Retrieved 12 April 2010.

External links

GeneReviews/NCBI/NIH/UW entry on ADAMTSL4-Related Eye Disorders,Autosomal Recessive Isolated Ectopia Lentis,Ectopia Lentis et Pupillae

UpToDate Contents

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1. 小児における水晶体偏位（水晶体脱臼） ectopia lentis dislocated lens in children
2. Overview of eye injuries in the emergency department
3. マルファン症候群および関連疾患の遺伝学、臨床的特徴、および診断 genetics clinical features and diagnosis of marfan syndrome and related disorders
4. Approach to eye injuries in the emergency department
5. マルファン症候群および関連疾患のマネージメント management of marfan syndrome and related disorders

English Journal

Effect of Angiotensin II receptor blocker on experimental periodontitis in a mouse model of Marfan syndrome.

Suda N, Moriyama K, Ganburged G.SourceDivision of Orthodontics, Department of Human Development & Fostering, Meikai University School of Dentistry, Sakado 350-0283, Saitama, Japan.
Infection and immunity.Infect Immun.2012 Oct 31. [Epub ahead of print]
Marfan syndrome is an autosomal dominant disease characterized by aneurysm and dilatation of the aortic root, tall stature, and ectopia lentis. These manifestations reflect excessive signaling of transforming growth factor (TGF)-β. Moreover, patients are frequently associated with severe periodonti
PMID 23115041

Exogenous Activation of BMP-2 Signaling Overcomes TGFβ-Mediated Inhibition of Osteogenesis in Marfan Embryonic Stem Cells and Marfan Patient Specific Induced-Pluripotent Stem Cells.

Quarto N, Li S, Renda A, Longaker MT.SourceHagey Laboratory for Pediatric Regenerative Medicine, Department of Surgery, Stanford University, School of Medicine, Stanford, CA, USA; Dipartimento di Scienze Chirurgiche, Anestesiologiche-Rianimatorie e dell'Emergenza "Giuseppe Zannini", Universita' degli Studi di Napoli Federico II, Napoli, Italy. quarto@unina.it.
Stem cells (Dayton, Ohio).Stem Cells.2012 Oct 4. doi: 10.1002/stem.1250. [Epub ahead of print]
Marfan syndrome (MFS) is a hereditary disease caused by mutations in the gene encoding Fibrillin-1 (FBN1) and characterized by a number of skeletal abnormalities, aortic root dilatation and sometimes ectopia lentis. Although the molecular pathogenesis of MFS was attributed initially to a structural
PMID 23037987

Japanese Journal

生後1ヵ月よりpropranolol，losartanなどの内科的治療を行い，5歳時に僧帽弁置換術を行った新生児Marfan症候群の1例

平野恭悠,村上洋介,佐々木赳,藤野光洋,川崎有希,江原英治,荒木幹太,金谷知潤,石丸和彦,西垣恭一,吉田修一朗,吉田葉子,鈴木嗣敏
日本小児循環器学会雑誌 30(6), 671-675, 2014
新生児Marfan症候群は希少な疾患で，わが国においての文献的報告は12例に留まる．出生直後ないし乳児期早期より特徴的な外表奇形と重篤な心肺機能不全を呈し予後不良である．症例は5歳女児．日齢2に哺乳不良と嘔吐を主訴に他院に入院．クモ様指と老人様顔貌があり，心エコーにて大動脈基部拡大，僧帽弁逆流，僧帽弁逸脱が確認され，新生児Marfan症候群と診断された．日齢26に当科に紹介され，生後1ヵ月よりpr …
NAID 130004879044

小児Marfan症候群における改訂Ghent基準の有用性

小野博,香取竜生,犬塚亮,今井靖,賀藤均
日本小児循環器学会雑誌 30(1), 49-56, 2014
背景：Marfan症候群に対するGhent基準が2010年に改訂された．改訂基準では大動脈病変，水晶体脱臼，遺伝子変異に重点がおかれ，骨格所見はsystemicscoreに一括された．方法：2008年4月～2009年12月に東京大学医学部附属病院小児科マルファン外来を受診し，従来のGhent基準（旧基準）においてMarfan症候群と診断または疑いでフォローされている症例38例について，改訂Ghen …
NAID 130003398237

Clinical Features Differ Substantially Between Caucasian and Asian Populations of Marfan Syndrome

, , , , , , , , , ,
Circulation Journal 77(11), 2793-2798, 2013
… Z-score: 4.5 vs. 3.6, P=0.013), and more often a positive systemic score (75.5% vs. 60.0%, P=0.045), but less frequently ectopia lentis (24.5% vs. 48.1%, P=0.004) compared with the Caucasian population (n=206). …
NAID 130003361857

Related Pictures

★リンクテーブル★

リンク元	「水晶体偏位」「lens dislocation」
関連記事	「ectopia」

「水晶体偏位」

Ectopia lentis
Classification and external resources
Specialty	medical genetics
ICD-10	H27.1, Q12.1
ICD-9-CM	743.37
OMIM	225100
DiseasesDB	29374
eMedicine	oph/55
MeSH	D004479
	[edit on Wikidata]