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Encyclopedia > Duane syndrome
Duane syndrome
Classification & external resources
An individual diagnosed with Duane syndrome in the left eye. In this image, the affected person is attempting to look far right. Notice the afflicted left eye faces straight and up (an "upshoot"), rather than following the right eye to the right.
ICD-10 H50.8
ICD-9 378.71
OMIM 126800 604356
DiseasesDB 30810
eMedicine oph/326 
MeSH D004370

Duane syndrome (DS) is a rare, congenital eye movement disorder most commonly characterized by the inability of the eye to turn out. The syndrome was first described by Stilling (1887) and Turk (1896), and subsequently named for Alexander Duane who discussed the disorder in more detail in 1905[1] Image File history File linksMetadata Duane_syndrome. ... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // H00-H59 - Diseases of the eye and adnexa (H00-H06) Disorders of eyelid, lacrimal system and orbit (H00) Hordeolum and chalazion (H000) Hordeolum and other deep inflammation of eyelid (H001) Chalazion (H01) Other inflammation of eyelid (H010) Blepharitis (H011) Noninfectious dermatoses of eyelid (H02) Other disorders of eyelid (H020) Entropion... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... A congenital disorder is a medical condition or defect that is present at or before birth (for example, congenital heart disease). ... An eye is an organ that detects light. ... This article is about the medical term. ... For other uses, see Eye (disambiguation). ... Alexander Duane (September 1, 1858 - June 10, 1926) was an American ophthalmologist who was a native of Malone, New York. ... For other uses, see 1905 (disambiguation). ...


Other names for this condition include: Duane's Retraction Syndrome (or DR syndrome), Eye Retraction Syndrome, Retraction Syndrome, Congenital retraction syndrome and Stilling-Turk-Duane Syndrome.[2]

Contents

Characteristics

As described by Duane, the characteristic features of the syndrome are:
1. Limitation of abduction (outward movement) of the affected eye.
2. Less marked limitation of adduction (inward movement) of the same eye.
3. Retraction of the eyeball into the socket on adduction, with associated narrowing of the palpebral fissure (eye opening)
4. Widening of the palpebral fissure on attempted abduction. (N.B. Mein and Trimble [3] point out that this is "probably of no significance" as the phenomenon also occurs in other conditions in which abduction is limited).
5. Poor convergence
6. A face turn to the side of the affected eye to compensate for the movement limitations of the eye(s) and to maintain binocular vision. For other uses, see Eye (disambiguation). ... A socket generally designates a cavity or region used for fitting and connecting some specific device. ... Binocular vision is vision in which both eyes are used synchronously to produce a single image. ...



In addition to the above, other characteristics may or may not be present, and these include:
1. Elevation or 'upshoot' of the affected eye on adduction. More rarely 'down shoots' can also occur.
2. Head movements to compensate for loss of eye movement when attempting to view an object outside of binocular viewing range (which may be very narrow).
3. Absence of diplopia: Few patients with the syndrome complain of diplopia when their eyes are misaligned. Diplopia, commonly known as double vision, is the perception of two images from a single object. ...


In about 80 per cent of cases, only one eye is affected, most often the left. However, in some cases, both eyes are affected, with one eye usually more affected than the other.


Causes

DS is a miswiring of the eye muscles, causing some eye muscles to contract when they shouldn't and other eye muscles not to contract when they should.[2] For other uses of Muscles, see Muscles (disambiguation). ...


Alexandrakis G & Saunders RA [4] state that:

In most cases, the abducens nucleus and nerve are absent or hypoplastic, and the lateral rectus muscle is innervated by a branch of the oculomotor nerve. However, there may be contributing mechanical abnormalities

This view is supported by the earlier work of Hotchkiss et al[5] who reported on the autopsy findings of two patients with Duanes syndrome. In both cases the sixth cranial nerve nucleus was absent, as was the sixth nerve, and the lateral rectus muscle was innervated by the inferior division of the third or oculomotor cranial nerve. This misdirection of nerve fibres results in opposing muscles being innervated by the same nerve. Thus, on attempted abduction, stimulation of the lateral rectus via the oculomotor nerve will be accompanied by stimulation of the opposing medial rectus via the same nerve; a muscle which works to adduct the eye. Thus, co-contraction of the muscles takes place, limiting the amount of movement achievable and also resulting in retraction of the eye into the socket. The oculomotor nerve () is the third of twelve paired cranial nerves. ...


The mechanical factors noted by Miller and Clark above are generally regarded as arising secondary to loss of innervation. During corrective surgery fibrous attachments have been found connecting the horizontal recti and the orbital walls and fibrosis of the lateral rectus has been confirmed by biopsy. This fibrosis can result in the lateral rectus being 'tight' and acting as a tether or leash. Co-contraction of the medial and lateral recti allows the globe to slip up or down under the tight lateral rectus producing the up and down shoots characteristic of the condition.


Epidemiology

Most patients are diagnosed by the age of 10 years and DS is more common in girls (60 percent of the cases) than boys (40 percent of the cases). A recent french study [6] reports the following findings:

The incidence of this syndrome in the population of strabismic patients was 1.9%. The number of women affected was 83 (53.5%). The syndrome was unilateral in 121 cases (78.1%). The left eye (71.9%) was affected more frequently than the right.

Around 10-20% of cases are familial; these are more likely to be bilateral than non-familial Duane syndrome. Duane syndrome has no particular race predilection. While usually isolated to the eye abnormalities, Duane syndrome can be associated with extraocular problems (so-called "Duane's Plus"), including cervical spine abnormalities (Klippel-Feil syndrome), Goldenhar syndrome, autism, heterochromia,and thalidomide-induced embryopathy. A family of Ouagadougou, Burkina Faso in 1997 A family consists of a domestic group of people (or a number of domestic groups), typically affiliated by birth or marriage, or by comparable legal relationships — including domestic partnership, adoption, surname and (in some cases) ownership (as occurred in the Roman Empire). ... Klippel-Feil syndrome is a rare disorder characterized by the congenital fusion of any 2 of the 7 cervical (neck) vertebrae. ... Goldenhar syndrome (also known as Oculo-Auriculo-Vertebral/OAV syndrome) - sometimes used interchangeably with Hemifacial Microsomia (although this definition is usually reserved for cases without internal organ/verterbrae disruption) - is a congenital defect documented in 1952 by Maurice Goldenhar and affecting between 1/3500 to 1/26000 live births in... Autism is a brain development disorder characterized by impairments in social interaction and communication, and restricted and repetitive behavior, all exhibited before a child is three years old. ... For the tightly packed form of DNA, see Heterochromatin. ... This article is about the drug. ...

Treatment

Duane syndrome cannot be cured, as the "missing" cranial nerve cannot be replaced. Treatment, therefore, is properly directed toward: correcting refractive errors, normalizing head position, expanding field of binocularity, and minimizing up- and downshoots. Strabismus surgery is often helpful; occasionally this is not needed during childhood, but becomes appropriate later in life, as the head position changes (presumably due to progressive muscle contracture). Cranial nerves are nerves which start directly from the brainstem instead of the spinal cord. ... // A refractive error, or refraction error, is an error in the focusing of light by the eye and a frequent reason for reduced visual acuity. ... This article or section does not cite its references or sources. ... Isolating the inferior rectus muscle Disinserting the medial rectus muscle Strabismus surgery is surgery on the extraocular muscles to correct the misalignment of the eyes. ... Contracture can refer to: Dupuytrens contracture Volkmanns contracture Capsular contracture This is a disambiguation page: a list of articles associated with the same title. ...


Prominent down- or upshoots (such as that pictured above) may be treated with a special form of muscle surgery, a "Y-splitting" procedure, often combined with a recession. In macroeconomics, a Recession is a decline in any countrys Gross Domestic Product (GDP), or negative real economic growth, for two or more successive quarters of a year. ...


Vertical Rectus Transposition sugery has achieved increased lateral motility in many cases. The VRT realigned muscles work with the good medial muscle to provide a "tripod" of musculature for the eye; the newly moved muscles provide torque and tension against the medial muscle, which is what allows for the central alignment. And, because they are "working" muscles, they also may allow for some range of movement (lateral) in their new position. Dr. Arthur Rosenbaum at UCLA is a leading practitioner of this technique.


References

  1. ^ Duane, A. Congenital Deficiency of Abduction associated with impairment of adduction, retraction movements, contraction of the palpebral fissure and oblique movements of the eye. Archives of ophthalmology (Chicago) 1905 34, 133-50
  2. ^ a b genome.gov. Retrieved on 2007-06-06.
  3. ^ Mein, J. & Trimble, R. Diagnosis and management of ocular motility disorders. 2nd ed. Blackwells 1991
  4. ^ Duane retraction syndrome. Ophthalmol Clin North Am. 2001 Sep;14(3):407-17
  5. ^ Hotchkiss M.G., Miller N.R., Clark, A.W., & Green W. R., (1980) Bilateral Duane's retraction syndrome. A clinico-pathologic case report. Arch of Ophthalmology 98, 870-4
  6. ^ Mehel E, Quére MA, Lavenant F, Pechereau A. Epidemiological and clinical aspects of Stilling-Turk-Duane syndrome. J Fr Ophtalmol. 1996;19(8-9):533-42.

Year 2007 (MMVII) is the current year, a common year starting on Monday of the Gregorian calendar and the AD/CE era in the 21st century. ... is the 157th day of the year (158th in leap years) in the Gregorian calendar. ...

External links

  • Duanes Syndrome Support and Discussion Group
  • Animation at mrcophth.com
  • SEE-KID: Computer-assisted Simulation and Treatment of Eye Motility Disorders
v • d • e
Pathology of the eye (primarily H00-H59, 360-379)
Eyelid, lacrimal system and orbit Stye - Chalazion - Blepharitis - Entropion - Ectropion - Lagophthalmos - Blepharochalasis - Ptosis - Xanthelasma - Trichiasis - Dacryoadenitis - Epiphora - Exophthalmos - Enophthalmos
Conjunctiva Conjunctivitis - Pterygium - Pinguecula - Subconjunctival hemorrhage
Sclera and cornea Scleritis - Keratitis - Corneal ulcer - Snow blindness - Thygeson's superficial punctate keratopathy - Fuchs' dystrophy - Keratoconus - Keratoconjunctivitis sicca - Arc eye - Keratoconjunctivitis - Corneal neovascularization - Kayser-Fleischer ring - Arcus senilis - Band keratopathy
Iris and ciliary body Iritis - Uveitis - Iridocyclitis - Hyphema - Persistent pupillary membrane - Iridodialysis - Synechia
Lens Cataract - Aphakia
Choroid and retina Chorioretinitis - Choroideremia - Retinal detachment - Retinoschisis - Retinopathy (Hypertensive retinopathy, Diabetic retinopathy, Retinopathy of prematurity) - Macular degeneration - Retinitis pigmentosa - Retinal haemorrhage - Central serous retinopathy - Macular edema - Epiretinal membrane - Macular pucker
Optic nerve and visual pathways Optic neuritis - Papilledema - Optic atrophy - Leber's hereditary optic neuropathy
Ocular muscles,
binocular movement,
accommodation and refraction		 Paralytic strabismus: Ophthalmoparesis - Progressive external ophthalmoplegia - Palsy (III, IV, VI) - Kearns-Sayre syndrome

Other strabismus: Esotropia/Exotropia - Hypertropia - Heterophoria (Esophoria, Exophoria) - Brown's syndrome - Duane syndrome
Other binocular: Conjugate gaze palsy - Convergence insufficiency - Internuclear ophthalmoplegia - One and a half syndrome
A renal cell carcinoma (chromophobe type) viewed on a hematoxylin & eosin stained slide Pathologist redirects here. ... For other uses, see Eye (disambiguation). ... An eyelid is a thin fold of skin that covers and protects an eye. ... Tears are a liquid produced by the bodys process of lacrimation to clean and lubricate the eyes. ... In anatomy, the orbit is the cavity or socket of the skull in which the eye and its appendages are situated. ... A stye (IPA: ) or hordeolum () is an infection of the sebaceous glands at the base of the eyelashes. ... A chalazion, also known as a Meibomian gland lipogranuloma, is a cyst in the eyelid that is caused by inflammation of the meibomian gland, usually on the upper eyelid. ... Blepharitis is inflammation of the eyelids. ... Entropion is a medical condition in which the eyelids fold inward. ... Ectropion is a condition of loose eyelids. ... Lagophthalmos describes difficulty in complete closure of the eyelid over the eyeball. ... Blepharochalasis is an inflammation of the eyelid that is characterized by exacerbations and remissions of eyelid edema, which results in a stretching and subsequent atrophy of the eyelid tissue. ... In ophthalmology, ptosis is an abnormally low position (drooping) of the upper eyelid which may grow more or less severe during the day. ... Xanthelasma (or xanthelasma palpebrarum) are sharply demarcated yellowish collections of cholesterol underneath the skin, usually around the eyes. ... Trichiasis is a medical term for ingrown eyelashes. ... Dacryoadenitis is inflammation of the lacrimal glands (the tear-producing glands). ... Epiphora is excessive tear production, usually a result from an irritation of the eye. ... Exophthalmos (or proptosis) is a bulging of the eye anteriorly out of the orbit. ... Enophthalmos is recession of the eyeball within the orbit. ... The conjunctiva is a membrane that covers the sclera (white part of the eye) and lines the inside of the eyelids. ... Pterygium usually refers to a benign growth of the conjunctiva. ... A Pinguecula is a type of conjunctival degeneration in the eye. ... A subconjunctival hemorrhage is a common and relatively minor post-LASIK complication. ... Schematic diagram of the human eye. ... The cornea is the transparent front part of the eye that covers the iris, pupil, and anterior chamber, providing most of an eyes optical power [1]. Together with the lens, the cornea refracts light and, as a result, helps the eye to focus. ... Scleritis is a serious inflammatory disease that affects the white outer coating of the eye, known as the sclera. ... A corneal ulcer is an inflammatory condition of the cornea involving loss of its outer layer. ... For other meanings see Snowblind. ... Thygesons superficial punctate keratopathy (TSPK) is a disease of the eyes. ... Fuchs dystrophy is a slowly progressing corneal disease that usually affects both eyes and is slightly more common in women than in men. ... Keratoconus (from Greek: kerato- horn, cornea; and konos cone), is a degenerative non-inflammatory disorder of the eye in which structural changes within the cornea cause it to thin and change to a more conical shape than its normal gradual curve. ... Keratoconjunctivitis sicca (KCS), also called keratitis sicca,[1] sicca syndrome,[1] xerophthalmia,[1] dry eye syndrome (DES),[1] or simply dry eyes,[1] is an eye disease caused by decreased tear production or increased tear film evaporation commonly found in humans and some animals[2]. Keratoconjunctivitis sicca is Latin and... Arc eye, also known as arc flash, welders flash, corneal flash burns, or flash burns, is a painful ocular condition sometimes experienced by welders who have failed to use adequate eye protection. ... Keratoconjunctivitis sicca or KCS is an eye disease caused by decreased tear production or increased tear film evaporation commonly found in people and small animals. ... Corneal neovascularization is the excessive ingrowth of blood vessels from the limbal vascular plexus into the cornea. ... Kayser-Fleischer rings are pigmented rings in the peripheral cornea, resulting from copper deposition in Descemets membrane. ... Arcus senilis (or Arcus senilis corneae. ... Band keratopathy is a corneal disease derived from the appearance of calcium on the central cornea. ... In anatomy, the iris (plural irises or irides) is the most visible part of the eye of vertebrates, including humans. ... Schematic diagram of the human eye The ciliary body is the part of the eye containing the ciliary muscle and ciliary processes. ... Iritis is a form of anterior uveitis and refers to the inflammation of the iris of the eye. ... Uveitis specifically refers to inflammation of the middle layer of the eye, termed the uvea but in common usage may refer to any inflammatory process involving the interior of the eye. ... Iridocyclitis, also known as anterior uveitis, is a condition in which the uvea of the eye suffers inflammation. ... Caused by a blunt blow to the eye. ... Persistent pupillary membrane (PPM) is a condition of the eye involving remnants of a fetal membrane that persist as strands of tissue crossing the pupil. ... Iridodialysis, sometimes known as a coredialysis, is a localized separation or tearing away of the iris from its attachment to the ciliary body. ... A synechia is an eye condition where the iris adheres to the either the cornea (i. ... Light from a single point of a distant object and light from a single point of a near object being brought to a focus by changing the curvature of the lens. ... Human eye cross-sectional view, showing position of human lens. ... Aphakia is the absence of the lens of the eye, due to surgical removal, perforating wound or ulcer, or congenital anomaly; causes a loss of accommodation, hyperopia, and a deep anterior chamber. ... The choroid, also known as the choroidea or choroid coat, is the vascular layer of the eye lying between the retina and the sclera. ... Human eye cross-sectional view. ... In medicine, Chorioretinitis is an inflammation of the choroid and retina of the eye. ... Choroideremia is an X-linked recessive retinal degenerative disease that leads to the degeneration of the choriocapillaris, the retinal pigment epithelium, and the photoreceptor of the eye. ... Retinal detachment is a disorder of the eye in which the retina peels away from its underlying layer of support tissue. ... Retinoschisis is an uncommon eye disease characterized by the abnormal splitting of the retinas sensory layers, usually in the outer plexiform layer, with resulting loss of visual function [1]. The retina, which consists of multiple layers of interconnected nerve and pigment cells, separates into separate layers resulting in a... Retinopathy is a general term that refers to some form of non-inflammatory damage to the retina of the eye. ... Hypertension, or high blood pressure that does not respond to treatment, has several ocular manifestations. ... Diabetic retinopathy is retinopathy (damage to the retina) caused by complications of diabetes mellitus, which could eventually lead to blindness. ... Retinopathy of prematurity (ROP), previously known as retrolental fibroplasia (RLF), is a disease of the eye that affects prematurely born babies. ... Listen to this article ( info/dl) This audio file was created from an article revision dated 2005-07-19, and may not reflect subsequent edits to the article. ... Normal vision. ... Retinal haemorrhage is a disorder of the eye in which bleeding occurs into the retina. ... Central serous retinopathy or CSR is a visual impairment, often temporary, usually in one eye, affecting males in the age group 20 to 50. ... Macular edema occurs when fluid and protein deposits collect on or under the macula, causing it to thicken and swell. ... Human eye cross-sectional view. ... Human eye cross-sectional view. ... This article is about the anatomical structure. ... The visual system is the part of the nervous system which allows organisms to see. ... Optic neuritis, sometimes called retrobulbar neuritis, is the inflammation of the optic nerve that may cause a complete or partial loss of vision. ... Papilledema is optic disc swelling that is caused by increased intracranial pressure. ... Optic atrophy is a pathological term and somewhat misleading. ... Leber’s hereditary optic neuropathy (LHON) or Leber optic atrophy is a mitochondrially inherited (mother to all offspring) form of acute or subacute loss of central vision that may lead to degeneration of retinal ganglion cells (RGCs) and their axons; this affects predominantly young adult males. ... MRI scan showing lateral and medial rectus muscles. ... Binocular vision is vision in which both eyes are used together. ... Light from a single point of a distant object and light from a single point of a near object being brought to a focus by changing the curvature of the lens. ... For the property of metals, see refraction (metallurgy). ... Ophthalmoparesis is a physical finding in certain neurologic illnesses. ... Progressive external ophthalmoplegia is a disorder of the mitochondria. ... Oculomotor nerve palsy is an eye condition resulting from damage to the third cranial nerve or a branch thereof. ... Fourth nerve palsy is a condition present at birth characterized by a vertical misalignment of the eyes due to a weakness or paralysis of the superior oblique muscle. ... Sixth nerve palsy, or abducens nerve palsy, is a disorder associated with dysfunction of cranial nerve VI (the abducens nerve) which is responsible for contracting the lateral rectus muscle to abduct (i. ... Kearns-Sayre syndrome (abbreviated KSS) is a disease caused by a 5,000 base deletion in the mitochondrial DNA. As such, it is a rare genetic disease in that it can be heteroplasmic, that is, more than one genome can be in a cell at any given time. ... Strabismus (from Greek: στραβισμός strabismos, from στραβίζειν strabizein to squint, from στραβός strabos squinting, squint-eyed[1]) is a condition in which the eyes are not properly aligned with each other. ... Person exhibiting esotropia of the right eye Esotropia is a form of strabismus, or squint, in which one or both eyes turns inward. ... Person exhibiting exotropia of the right eye Exotropia is a form of strabismus where the eyes are deviated outward. ... Hypertropia is a condition of misalignment of the eyes (strabismus), whereby the visual axis of one eye is higher than the fellow fixating eye. ... Heterophoria is a type of eye condition where the motion of the eyes is not parallel to each other. ... Esophoria is characterised by inward deviation of the eye usually due to extra-ocular muscle imbalance. ... This article or section does not cite any references or sources. ... Browns syndrome is due to fibrous adhesions in the upper medial quadrant of the orbit. ... Conjugate gaze palsy refers to an inability of both eyes to move in the same direction at the same time. ... Convergence insufficiency is a sensory and neuromuscular anomaly of the binocular vision system, characterized by an inability to converge the eyes or sustain convergence. ... Internuclear ophthalmoplegia is a physical finding, or sign, that is a particular form of [[[ophthalmoparesis]]. It can affect either the right or left eye. ... Schematic representation of most common extra-ocular movement abnormality in one and a half syndrome. ...

Refractive error: Hyperopia/Myopia - Astigmatism - Anisometropia/Aniseikonia - Presbyopia
Visual disturbances and blindness Amblyopia - Leber's congenital amaurosis - Subjective (Asthenopia, Hemeralopia, Photophobia, Scintillating scotoma) - Diplopia - Scotoma - Anopsia (Binasal hemianopsia, Bitemporal hemianopsia, Homonymous hemianopsia, Quadrantanopia) - Color blindness (Achromatopsia) - Nyctalopia - Blindness/Low vision
Pupil Anisocoria - Argyll Robertson pupil - Marcus Gunn pupil/Marcus Gunn phenomenon - Adie syndrome
Infectious diseases Trachoma - Onchocerciasis
Other Nystagmus - Miosis - Mydriasis - Glaucoma - Floater - Leber's hereditary optic neuropathy - Red eye - Keratomycosis - Xerophthalmia - Aniridia

  Results from FactBites:
 
eMedicine - Duane Syndrome : Article by Arun Verma, MD (7501 words)
Duane syndrome (DS) is a rare, congenital disorder of eye movement.
In 1976, Duane and coauthors described entirely unrelated pseudo-DS in which abduction is restricted and narrowing of the palpebral fissure and retraction of the globe occur after fracture of the medial orbital wall with entrapment of the medial rectus muscle.
Duane A: Congenital deficiency of abduction, associated with impairment of adduction, retraction movements, contraction of the palpebral fissure and oblique movements of the eye.
  More results at FactBites »


 
 

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