BASICS
DESCRIPTION
• An inability to elevate the eye in all fields of gaze often resulting in a large hypotropia, with associated ptosis
• Also called monocular elevation deficiency
EPIDEMIOLOGY
Incidence
Very rare
Prevalence
Unknown
RISK FACTORS
None Known
Genetics
No known genetic association (although monocular elevation deficit may also be seen in Congenital cranial dysinnervation disorders and some craniosynostosis syndromes)
PATHOPHYSIOLOGY
Neurologic (cranial nerve, nuclear, or supranuclear deficits and mechanical restriction (or both) can contribute.
ETIOLOGY
• Classified into 3 subgroups with different underlying mechanisms:
– Primary inferior rectus restriction
– Primary paresis or of the superior rectus
– Supranuclear deficit (1)[A]
COMMONLY ASSOCIATED CONDITIONS
• Marcus Gunn jaw-winking ptosis is seen in 25% of patients
• Hypotropia
• Ptosis
• Chin lift (2)[A]
DIAGNOSIS
HISTORY
Family notices limited elevation and ptosis of the involved eye, along with an abnormal chin up head position.
PHYSICAL EXAM
• Reduced elevation of the eye in all positions of gaze
• When fixing with the non-paretic/restricted eye, the involved eye is hypotropic and the lid becomes ptotic.
• Fixation with the involved eye results in a hypertropia of the non-paretic eye (which is often of greater magnitude than the baseline hypotropia) and often, resolution of the ptosis.
• Many patients present with a large chin up position, used to maintain binocular vision.
• Full ocular examination including dilated retinal examination and refraction.
• Evaluate visual acuity for amblyopia.
DIAGNOSTIC TESTS & INTERPRETATION
Lab
None
Imaging
Can be helpful in differential diagnosis, but is not necessary if clinical diagnosis is clear.
Diagnostic Procedures/Other
• Forced duction testing is necessary to establish etiology and best surgical approach.
• If positive, indicates inferior rectus restricted.
• If negative, indicates no inferior rectus restriction.
• Assessment of Bell’s phenomenon:
– When present, cause is likely supranuclear
– When absent, consider inferior rectus restriction (3)[A]
DIFFERENTIAL DIAGNOSIS
• Brown’s syndrome
• Orbital blowout fracture
• Thyroid ophthalmopathy
• Orbital fibrosis syndrome
• Congenital cranial dysinnervation disorders
• Anomalous/absent muscles (as in craniosynostosis syndrome)
• Superior orbital tumor or hemorrhage
• Parinaud syndrome
– Hydrocephalus
– Abnormality in the midbrain region of the quadrigeminal plate
• Cranial Nerve III palsy (4)[A]
TREATMENT
MEDICATION
None
ADDITIONAL TREATMENT
General Measures
• Correct refractive error
• Treat amblyopia
Issues for Referral
• Consider neurology consult if concern about intracranial cause
• Consider endocrinology consult if concern about thyroid eye disease
Additional Therapies
Ptosis surgery should not be done until final alignment is established (5)[A]
COMPLEMENTARY & ALTERNATIVE THERAPIES
None
SURGERY/OTHER PROCEDURES
• Surgery is indicated for relief of strabismus and/or limiting head position.
• Some patients are orthophoric in primary and do not require surgery.
• Forced duction
– If positive, include inferior rectus recession in surgical plan
– If negative:
And large hypotropia present, a transposition procedure should be done, moving all or a portion of the medial and lateral rectus muscles to the superior rectus insertion
With a smaller hypotropia, resection of the superior rectus and recession of the inferior rectus can be done (5)[A], (6)[A], (7)[A]
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
More frequently in young children to rule out amblyopia.
Patient Monitoring
• For visual acuity to rule out amblyopia
• For recurrent strabismus and diplopia, or head position
PATIENT EDUCATION
Importance of regular follow-up to monitor for amblyopia
PROGNOSIS
• Reoperation frequently needed
• Good visual outcome with refractive correction and treatment of amblyopia
COMPLICATIONS
• Recurrent strabismus
• Persistent/recurrent true ptosis
• Amblyopia
REFERENCES
1. Cadera W, Bloom JN, Karlik S. An MRI study of double elevator palsy. Ophthalmology 1997;32:250–253.
2. Mims JK 3rd. “Double elevator palsy” eye supraducts during stage II general anesthesia supporting hypothesis of (supra) nuclear etiology. Binocul Vis Strabismus Q 2005;20:199–204.
3. Zafar SN, Khan A, Azad N, et al. Ptosis associated with monocular elevation deficiency. J Pak Med Assoc 2009;59:522–524.
4. Mets HS. Double elevator palsy. J Pediatr Ophthalmol Strabismus 1991;18:31–35.
5. Foster RS. Vertical muscle transposition augmented with lateral fixation. J AAPOS 1997;1(1):20–30.
6. Knapp P. The surgical treatment of double-elevator paralysis. Trans Am Ophthalmol Soc 1969;67:304–323.
7. Burke JP, Ruben JB, Scott WE. Vertical transposition of the horizontal recti (Knapp procedure) for the treatment of double elevator palsy: Effectiveness and long-term stability. Br J Ophthalmol 1992;76(12):734–737.
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