lenticonus

円錐水晶体

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/06/23 11:51:05」(JST)

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Lenticonus (/len·ti·co·nus/ (len″tĭ-ko´nus)) [lens + L. conus, cone] is a rare congenital anomaly of the eye characterized by a conical protrusion on the crystalline lens capsule and the underlying cortex. It can reach a diameter of 2 to 7 mm. The conus may occur anteriorly or posteriorly. If the bulging is spherical, instead of conical, the condition is referred to as lentiglobus. It produces a decrease in visual acuity and irregular refraction that cannot be corrected by either spectacle or contact lenses.

Biomicroscopically lenticonus is characterized by a transparent, localized, sharply demarcated conical projection of the lens capsule and cortex, usually axial in localization. In an early stage, retro-illumination shows an «oil droplet» configuration. Using a narrow slit, the image of a conus is observed. In a more advanced stage associated subcapsular and cortical opacities appear. Retinoscopically the oil droplet produces a pathognomonic scissors movement of the light reflex. This phenomenon is due to the different refraction in the central and the peripheral area of the lens. Ultrasonography also can illustrate the existence of a lenticonus. A-scan ultrasonography may reveal an increased lens thickness and B- scanultrasonography may show herniated lenticular material, suggestive of a lenticonus. Amblyopia, cataract, strabismus and loss of central fixation may be observed in association with lenticonus posterior. Cataract,flecked retinopathy, posterior polymorphous dystrophy and corneal arcus juvenilis may be encountered in association with lenticonus anterior that occurs as a part of the Alport syndrome.

Exist two distinct types of lenticonus based on the face of the lens affected.

Types

1. Lenticonus anterior; lenticonus anterior is part of the Alport syndrome
2. Lenticonus posterior; lenticonus posterior is more common than lenticonus anterior and is sometimes found in Lowe syndrome

References

UpToDate Contents

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• 1. 遺伝性腎炎（アルポート症候群）の臨床症状、診断および治療 clinical manifestations diagnosis and treatment of hereditary nephritis alport syndrome
• 2. 遺伝性腎炎（アルポート症候群）の遺伝学、病因、および病理 genetics pathogenesis and pathology of hereditary nephritis alport syndrome
• 3. 慢性腎疾患関連眼疾患 eye disorders associated with chronic kidney disease
• 4. 小児における白内障 cataract in children
• 5. 小児における顕微鏡的血尿の評価 evaluation of microscopic hematuria in children

English Journal

• The 2014 international workshop on alport syndrome.

• Miner JH1, Baigent C2, Flinter F3, Gross O4, Judge P2, Kashtan CE5, Lagas S6, Savige J7, Blatt D8, Ding J9, Gale DP10, Midgley JP11, Povey S12, Prunotto M13, Renault D14, Skelding J15, Turner AN16, Gear S15.
• Kidney international.Kidney Int.2014 Oct;86(4):679-84. doi: 10.1038/ki.2014.229. Epub 2014 Jul 2.
• Alport syndrome, historically referred to as hereditary glomerulonephritis with sensorineural deafness and anterior lenticonus, is a genetic disease of collagen α3α4α5(IV) resulting in renal failure. The collagen α3α4α5(IV) heterotrimer forms a network that is a major component of the kidney g
• PMID 24988067

• Phacoemulsification in a Rare Case of Alport's Syndrome.

• Mohan S1, Gupta P, Sahai K, Sachan SK.
• Seminars in ophthalmology.Semin Ophthalmol.2014 Jul;29(4):196-8. doi: 10.3109/08820538.2013.810283. Epub 2013 Aug 16.
• AIM: To report a rare case of Alport Syndrome (AS) highlighting the precautions and surgical complications during phacoemulsification.METHOD: A 35 year old female presented with bilateral painless progressive diminution of vision since 6 months and had bilateral deafness since 12 years. Best correct
• PMID 23952035

• Bilateral anterior lenticonus in a case of Alport syndrome: a clinical and histopathological correlation after successful clear lens extraction.

• Sonarkhan S1, Ramappa M2, Chaurasia S2, Mulay K3.
• BMJ case reports.BMJ Case Rep.2014 Jun 26;2014. pii: bcr2013202036. doi: 10.1136/bcr-2013-202036.
• A 29-year-old woman presented with symptoms of gradual, progressive, painless diminution of vision in both eyes for past 8-10 years. On examination, uncorrected visual acuity was 20/60 in the right eye and 20/126 in the left eye. Anterior segment examination was essentially normal except for a con
• PMID 24969069

Japanese Journal

• 水晶体疾患 (特集 眼科における先天異常)

• 野村 耕治
• 眼科 51(6), 747-754, 2009-06
• NAID 40016644868

• 片眼性の成熟白内障でみつかった両眼性前部円錐水晶体の家系

• 鈴木 香,鈴木 幸彦,中澤 満
• 眼科手術 = Journal of ophthalmic surgery 21(4), 507-511, 2008-10-30
• NAID 10024370819

Related Links

• lenticonus - definition of lenticonus in the Medical dictionary ...

lenticonus /len·ti·co·nus/ (len″tĭ-ko´nus) a congenital conical bulging, anteriorly or posteriorly, of the lens of the eye. len·ti·co·nus (lĕn′tĭ-kō′nəs) n. A conical projection of the anterior or posterior surface of the lens, occurring as a ...

• Lenticonus | Define Lenticonus at Dictionary.com

Medical Dictionary lenticonus len·ti·co·nus (lěn'tĭ-kō'nəs) n. A conical projection of the anterior or posterior surface of the lens, occurring as a developmental anomaly. The American Heritage® Stedman's Medical Dictionary ...

Related Pictures

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★リンクテーブル★

リンク元	「アルポート症候群」

「アルポート症候群」

　　[★]

英

Alport syndrome, Alport's syndrome, AS

同

遺伝性腎炎、Alport症候群、難聴遺伝性腎炎 hereditary nephritis with deafness

概念

• 主にX連鎖劣性遺伝の遺伝性腎炎であり、血尿を伴う腎炎のほかに、感音難聴、眼症状を呈する。IV型コラーゲンの異常と考えられている。進行型であり、やがて蛋白尿、高血圧を来たし腎不全に陥る。

病因

SPE.603

• 孤発性(10-15)
• 遺伝性：IV型コラーゲンの異常

• X-linked(70-80%)
• AR(10-15%)
• AD

遺伝形式

• 1. XD : 血尿、感音性難聴、水晶体の変形(円錐水晶体 lenticonus)
• 2. X-linked form : 1. + びまん性平滑筋腫(diffuse keiomyomatosis)
• 3. AR : 腎病変のみ
• 4. AD : 腎病変のみ

身体所見

• 円錐水晶

症候

(XD型)男性の場合、幼少期に無症候性血尿として発見され、肉眼的血尿から、次第に蛋白尿(難治性ネフローゼ)を呈するようになり、20歳頃に腎不全に至る。女性の場合のまま緩徐に経過する。

• 1. 感音性難聴(30-40%の症例。両側。(SPE.604))
• 2. 血尿：初発
• 3. 腎炎
• 4. 眼症状(円錐角膜、球状水晶体、白内障など)(15%の症例(SPE.604))

検査

SPE.604

光学顕微鏡

• メサンギウム細胞、基質の増加。進行例では巣状・分節状糸球体硬化

電子顕微鏡

• 糸球体基底膜の不規則な肥厚、層状化、断裂、菲薄化

蛍光顕微鏡

• IV型コラーゲン抗α鎖抗体で免疫蛍光染色すると、XD男性の糸球体基底膜、皮膚基底膜は染色されない。