External Total Chronic Progressive Ophthalmoplegia

DEFINITION:

External, total, chronic progressive ophthalmoplegia is a rare disorder affecting ocular motility and functioning of the levator palpebrae muscles.

SIGNS AND SYMPTOMS:

The signs and symptoms of external, total, chronic progressive ophthalmoplegia may include, but are not limited to, the following:

1. bilateral ptosis
2. decreased ocular motility in all positions of gaze.
3. abnormal head or neck posture (i.e. patient’s chin may be maximally elevated to permit vision in advanced stages of ophthalmoplegia)
4. head tilt/turn
5. diplopia (rare)
6. abnormal postural adaptation/abnormal working distance (ICD: R29.3)
7. spatial disorientation/incoordination/clumsiness (ICD: R27.8)
8. muscles of mastication may become involved in addition to extra-ocular muscles and levator muscles

DIAGNOSTIC FACTORS:

External, total, chronic progressive ophthalmoplegia is characterized by one or more of the following:

1. bilateral ptosis
2. may be myogenic or nuclear in etiology
3. non-comitant ocular motility

THERAPEUTIC MANAGEMENT CONSIDERATIONS:

The doctor of optometry determines appropriate diagnostic and therapeutic modalities, and frequency of evaluation and follow-up, based on the urgency and nature of the patient’s conditions and unique needs. Vision disorders that are not totally cured through vision therapy may still be ameliorated with

significant improvement in visual function and quality of life. In cases of external, total, chronic progressive ophthalmoplegia, co-management with medicine (i.e. internists, neuro-ophthalmology and/or ophthalmology) is often in order due to systemic complications. The management of the case and duration of treatment would be affected by:

1. the severity of symptoms and diagnostic factors, including onset and duration of the problem
2. the complications of associated visual conditions
3. implications of patient’s general health, cognitive development, physical development, and effects of medications taken
4. etiological factors
5. extent of visual demands placed upon the individual
6. patient compliance and involvement in the prescribed therapy regimen
7. type, scope, and results of prior interventions

PRESCRIBED TREATMENT REGIMEN:

Some cases are successfully managed by the prescription of therapeutic lenses and/or prisms and, possibly, a ptosis crutch and/or lid taping. Many external, total, chronic progressive ophthalmoplegia cases benefit from optometric vision therapy, which incorporates the prescription of specific treatments in order to:

1. to decrease the ptosis using a ptosis crutch and/or neuromuscular exercises
2. develop adequate fusional vergence ranges and stability in all positions of gaze at distance and near
3. enhance accommodative/convergence relationships
4. enhance depth judgments and/or stereopsis
5. integrate binocular function with information processing
6. enhance fusional vergence facility and flexibility
7. integrate binocular skills with accurate motor responses
8. integrate binocular skills with other sensory skills (vestibular, kinesthetic, tactile, and auditory)
9. increase visual stamina/integrate newly established skills with information processing

DURATION OF TREATMENT:

The following treatment ranges are provided as a guide. Treatment duration will depend upon the particular patient’s condition and associated factors. When duration of treatment beyond these ranges is required, documentation of the medical necessity for additional treatment services may be warranted for third-party claims processing and review purposes.

1. Full treatment requires resolution of associated visual conditions.
2. The most commonly encountered external, total, or chronic progressive ophthalmoplegia usually requires 32 to 40 hours of office therapy.
3. Associated factors such as cerebral vascular accident, head trauma, and/or systemic conditions may warrant an increase in treatment period.

FOLLOW-UP CARE:

At the conclusion of the active treatment regimen, periodic follow-up evaluation is required. Should signs, symptoms, or other diagnostic factors recur, further therapy may be medically necessary. Therapeutic lenses may be prescribed during or at the conclusion of active vision therapy to assist in the maintenance of long-term stability.