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DUANE RETRACTION SYNDROME in Nepal

DUANE RETRACTION SYNDROME

Duane retraction syndrome is characterized by retraction of globe (eyeball) on attempted abduction (inward movement) caused due to co-contraction of both medial and lateral rectus muscle of the involved eye.

Duane retraction syndrome is a non-progressive congenital disorder of eye movement, which may occur either in isolation or in the form of syndrome associated with other congenital defects, most common is perceptive deafness with associated speech disorder. It may be associated with strabismus in primary gaze.

Horizontal eye movement is governed by lateral rectus muscle, which pulls the eye outward and by medial rectus muscle, which pulls the eye inward. When lateral rectus muscle of an eye contracts and pull the eyeball outward, the medial rectus of the same eye relaxes. The contra-lateral medial rectus muscle contracts and the lateral rectus muscle relaxes to a similar extent for smooth movement of eyeballs.

CLASSIFICATION:

THERE ARE THREE MAIN TYPES (HUBER CLASSIFICATION):

Type 1: This is characterized by limitation or absence of abduction (outward movement) with normal or mildly restricted adduction. This is the most common type. The abduction or ability to move the eye outward is limited, but adduction or ability to move the eye inward is normal or nearly so. There is retraction of the eyeball (enophthalmos) with narrowing of the palpebral fissure on adduction. The eyeball assumes its normal position and the palpebral fissure widens again on attempted abduction.

Type 2: This is characterized by limited adduction with normal or mildly restricted abduction. This is the least common type, in this type, affected eye shows limitation of adduction but the abduction is normal or nearly so. On attempted abduction, there is retraction of the globe and narrowing of the palpebral fissure.

Type 3: This type shows limitation of both abduction and adduction, there is limitation of both adduction and abduction. The eyeball retracts and the palpebral fissure narrows on attempted abduction.

SYMPTOMS

Duane retraction syndrome may be associated with some or all of the following features:

  • Retraction of globe on adduction.
  • Complete or partial limitation of abduction.
  • Narrowing of palpebral fissure on adduction.
  • Widening of palpebral fissure on attempted abduction.
  • Partial limitation of adduction.
  • Convergence insufficiency.
  • Oblique movement of globe on attempted abduction.
  • Upshoot or downshoot of globe with adduction (Leash or Bridle Phenomenon)

Patient may present with:

  • Manifest squint.
  • Reduced abduction in affected eye.
  • Loss of binocularity.
  • Head turn to maintain fusion.
  • Certain associated eye conditions like heterochromia, pupillary abnormalities, cataract or microphthalmos (small eyeball).

Patient may carry an old picture of strabismus.

ITIOLOGY:

Duane retraction syndrome is thought to be due to aberrant innervations of medial and lateral rectus muscles. In addition, structural anomalies of the muscle or primary anomaly of the brainstem may be contributing as aetiological factors. Most likely, both genetic and environmental factors play a role in the development of this syndrome.

Duane retraction syndrome may occur as an isolated phenomenon or present as a syndrome.

Isolated form: This comprises 90% of cases which are sporadic and unilateral. Remaining 10% may be inherited, bilateral and may have vertical movement abnormalities. These may be autosomal dominant or autosomal recessive.

ASSOCIATION WITH SYNDROMES:

This may be associated with different syndromes like:

  • Goldenhar syndrome: It is characterised by malformation of jaw, cheek and ear usually on one side of the face.
  • Morning glory syndrome: It is characterised by abnormalities of the optic disc.
  • Moebius syndrome: This may show congenital paresis of facial (VIIth) and abducent (VIth) cranial nerves.

MANAGEMENT:  Associated refractive error may be corrected with suitable spectacles or contact lenses. Compensatory head turn patients may be treated by incorporating prisms in glasses.

-  Similarly, any associated amblyopia is treated Surgical management:

HOMOEOPATHIC MANAGEMENT:

Constitutional remedies are required in this cases.

Few homoeopathic medicines which can be helpful in this case are as followed.

CINERARIA MARITIMA - Cure of cataract and corneal opacities. Is used externally, by instilling into the eye one drop four or five times a day. This must be kept up for several months. Most effective in traumatic cases. Compare in cataract

ACONITE - Red, inflamed. Feel dry and hot, as if sand in them. Lids swollen, hard and red. Aversion to light. Profuse watering after exposure to dry, cold winds, reflection from snow, after extraction of cinders and other foreign bodies.

EUPHRASIA- the eyes water all the time. Acrid lachrymation; bland coryza (Opposite: Cepa). Discharge thick and excoriating (Mercur thin and acrid). Burning and swelling of the lids. Frequent inclination to blink. Free discharge of acrid matter. Sticky mucus on cornea; must wink to remove it. Pressure in eyes. Little blisters on cornea, Opacities, Rheumatic iritis

THUJA OCCIDENTALIS - Ciliary neuralgia; iritis. Eyelids agglutinated at night; dry, scaly. Styes and tarsal tumors (Staph). Acute and subacute inflammation of sclera. Sclera raised in patches, and looks bluish-red. Large, flat phlyctenules, indolent. Recurring episcleritis. Chronic scleritis.

CALCAREA FLUOR - Flickering and sparks before the eyes, spots on the cornea; conjunctivitis; cataract. Strumous phlyctemular keratitis. Subcutaneous palpebral cysts.

SILICEA - Swelling of lachrymal duct. Aversion to light, especially daylight; it produces dazzling, sharp pain through eyes; eyes tender to touch; worse when closed. Vision confused; letters run together on reading. Styes. Iritis and irido-choroiditis, with pus in anterior chamber. Perforating or sloughing ulcer of cornea. Abscess in cornea after traumatic injury. Cataract in office workers. After-effects of keratitis and ulcus cornæ, clearing the opacity.

ARNICA - Diplopia from traumatism, muscular paralysis, retinal hemorrhage. Bruised, sore feeling in eyes after close work. Must keep eyes open. Dizzy on closing them. Feel tired and weary after sight-seeing, moving pictures, etc.

RUTA G- Eyes-strain followed by headache. Eyes red, hot, and painful from sewing or reading fine print. Disturbances of accommodation. Weary pain while reading. Pressure deep in orbits. Tarsal cartilage feels bruised. Pressure over eyebrow.