Double Elevator Palsy

Double Elevator Palsy refers to limited elevation of one eye in adduction, primary gaze, and abduction. The term double elevator refers to both the elevators of an eye i.e. superior rectus and inferior oblique muscle. However, there are cases in which deficient elevation is produced by tight inferior rectus muscle. Due to this, Double elevator palsy is included under restrictive strabismus. Now Double elevator palsy is known by a more descriptive term as Monocular Elevation Deficit Syndrome (MED).This is distinguished from Brown’s syndrome in which elevation is restricted much more in adduction rather than in abduction.

This condition presents as hypotropia when the patient fixates with normal non-paretic eye. Chin elevation and ptosis/pseudoptosis are often present. Fixation with the paretic eye produces hypertropia of the normal non-paretic eye. Ptosis also disappears since it is not due to paresis of levator palpebrae superioris muscle.

White (1942) described a congenital deficiency of upgaze associated with hypotropia and ptosis of the affected paretic eye. Dunlap (1952) to describe the weakness affecting both elevators of involved eye coined the term Double elevator palsy.

Double elevator palsy may be associated with certain innervational conditions such as

  • Jaw-winking ptosis
  • Duane’s retraction syndrome
  • Other misdirection strabismus syndromes

 

References

Wright Kenneth W. Color Atlas of Strabismus Surgery- Strategies and Techniques. Springer Science+Business Media, LLC 2007. P 69-70.

Nelson Leonard B, Olitsky Scott E. Harley’s Pediatric Ophthalmology Fifth Edition. Lippincott Williams & Wilkins 2005. P 181-182.

Wright Kenneth W, Spiegel Peter H. Pediatric Ophthalmology and Strabismus Second Edition. Springer- Verlag New York, Inc. 2003. P 257- 258.

Lorenz Brigit, Brodsky Michael C. Pediatric Ophthalmology, Neurophthalmology, Genetics; Strabismus-New Concepts in Pathophysiology, Diagnosis, and Treatment. Springer- Verlag Berlin Heidelberg 2010. P 87- 88.

Rosenbaum Arthur L, Santiago Alvina Pauline. Clinical Strabismus Management- Principles and Surgical Techniques. W.B. Saunders Company 1999. P 272- 279.

Kaynak-Hekimhan Pelin, Grover A K, Toukhy Essam El, Nassaralla Belquiz A, Murthy Ramesh, Morekar Sunil. Surgical Techniques in Ophthalmology- Oculoplasty and Reconstructive Surgery. Jaypee Brothers Medical Publishers (P) Ltd 2010. P 219.

Bowling Brad. Kanski’s Clinical Ophthalmology- A Systematic Approach Eighth Edition. Elsevier Limited 2016. P 766.

http://www.djo.org.in/articles/26/1/monocular-elevation-deficit-simplified.html

https://www.sankaranethralaya.org/insight/PDF%20Files/october2016/case-report-3.pdf

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3589222/

http://www.ijo.cn/en_publish/2018/8/20180816.pdf

White JW. Paralysis of the superior rectus and inferior oblique muscles of the same eye. Arch Ophthalmol. 1942; 27: 366–371.

Dunlap EA. Vertical displacement of horizontal recti; Symposium on strabismus transactions of the new Orleans Academy of Ophthalmology; St Louis: Mosby; 1971. pp. 307–329.

 

 

Symptoms of MED includes

  • Vertical misalignment of eyes
  • Profound restriction of elevation across horizontal plane, from adduction to abduction with orthophoria in primary position
  • Abnormal head posture with chin elevation
  • Ptosis
  • Amblyopia
  • Diplopia

 

 

Causes may include congenital and acquired cases.

Congenital cases are characterised by orthotropia or hypotropia in primary position of gaze, true ptosis or pseudoptosis in majority of cases. A large number of cases on forced duction test, show restriction of eyeball in elevation. Jaw movement as in yawning may show paradoxical movements of eyelids (Marcus Gunn phenomenon). Dissociated vertical deviation (DVD) may be present. Upward and outward rolling of eyeballs or Bell’s phenomenon is often preserved.

Since elevation is preserved in Bell’s phenomenon, DVD or under anaesthesia, several authors believe it to be a supranuclear disorder. Others discuss it to be a fascicular lesion. Long lasting palsy of superior rectus alone may impede elevation in adduction, thus nuclear origin of superior rectus may be a factor. Even primary fibrotic origin is presumed in cases showing resistance to forced duction test.

Thus supranuclear, nuclear, fascicular and muscular causes are discussed for double elevator palsy.

Cerebrovascular diseases such as hypertension, thromboembolism, and arteritis usually cause acquired cases. Other causes of double elevator palsy include

  • Infectious disease
  • Tumours
  • Sarcoidosis

 

Diagnosis depends upon history and clinical examination.

 

History

In congenital MED, history of onset of symptoms and its association with other features is important. The presence of associated amblyopia points to congenital aetiology.

In acquired MED, history of associated neurological features (ataxia, vertigo, tinnitus, loss of consciousness) is important. History of malignancy or cardiovascular disorder is also important.

 

Clinical examination

Clinical examination distinguishes between MED due to primary superior rectus palsy, any cause of supranuclear superior rectus weakness, or primary/secondary inferior rectus restriction.

Primary superior rectus palsy or paresis: It is characterised by a forced duction test showing no restriction to full upward rotation. Bell’s phenomenon is not present.

Supranuclear superior rectus weakness: This is usually congenital and is characterised by monocular absence of vertical eye movements in superior field of gaze. There is no resistance to upward gaze in forced duction test. Bell’s phenomenon is present.

Patients with primary inferior rectus restriction or fibrosis: This group of patients often do not have hypotropia in primary gaze. Forced duction test shows restriction to upward rotation because of inferior rectus restriction. Superior rectus palsy or supranuclear disorders may produce secondary contracture in inferior rectus. Hypotropia and ptosis are usually present. Forced duction test shows inferior rectus restriction.

 

Congenital MED

  • Eyes: Classically MED present with unilateral limitation of upgaze above horizontal midline with accompanying ptosis. Limitation in upgaze is same whether in adduction or abduction. The affected eye is hypotropic. Ptosis is most noticeable when the nonparetic eye fixes and the paretic eye is hypotropic. Pseudoptosis occurs when the eyelid is passively drawn down in hypotropic eye. It resolves when paretic eye takes up fixation. True ptosis does not resolve when paretic eye takes up fixation. In cases with associated ptosis and pseudoptosis together, ptosis improves in part when the paretic eye takes up fixation in primary position.
  • Head posture: Depending upon degree of binocularity and fixation preference, variable head postures are observed in MED. A patient with hypotropia and having some degree of fusion, usually tilts head back in chin up position to maintain binocularity. Cases with no hypotropia or hypotropic eye is also amblyopic, adopts a normal head posture. Rarely, patient may fixate with affected eye, producing large secondary hypertropia in the nonparetic eye.

 

Acquired MED

  • Eyes: It is characterised by acute onset of diplopia in both primary position and upward gaze. Limitation of upgaze is same in both adduction and abduction. Ptosis is usually absent. There may be associated abnormalities of pupil, paresis in downgaze, and convergence weakness. Bell’s phenomenon is usually present unless it is affected by secondary restriction of inferior rectus muscle.
  • Head position: Often patient assumes chin up position to maintain binocularity and thus decreasing diplopia.

 

Investigations

Besides regular blood tests, following tests may be done

  • Chest radiography
  • Computed tomography
  • Cerebral angiography
  • Magnetic resonance imaging of the brain stem

 

Differential diagnosis

Differential diagnosis of congenital MED includes conditions such as

  • Brown superior oblique syndrome
  • Congenital fibrosis of inferior rectus muscle
  • Congenital absence of superior rectus muscle with or without absence of inferior oblique muscle
  • Vertical Duane co-contraction syndrome
  • Third cranial nerve palsy with involvement of superior branch
  • Anomalous insertion of superior or inferior rectus muscle

Differential diagnosis of acquired MED includes conditions such as

  • Dysthyroid orbitopathy
  • Myasthenia gravis
  • Fracture of orbital floor with muscle entrapment
  • Lesions affecting superior branch of oculomotor nerve
  • Progressive external ophthalmoplegia
  • Orbital cellulitis
  • Orbital inflammatory disease
  • Systemic amyloidosis with extraocular muscle infiltration
  • Labyrinthine disorders
  • Cerebellar tumours

 

 

Management is not required in all cases of MED.

Patients with orthophoria in primary position of gaze or with limited changes in head position may be just observed.

 

Medical therapy

  • Refractive error correction: Any associated refractive error is corrected
  • Amblyopia treatment: If amblyopia is noticed in congenital MED, it needs to be treated.

 

Surgical therapy

Indications for surgery are

  • Deviation of eyes producing amblyopia and suppression
  • Vertical deviation in primary position of gaze
  • Diplopia in primary position of gaze
  • Contracted binocular fields
  • Ptosis surgery if significant ptosis persist after strabismus surgery

 

Surgical procedures

Patient and the family members should be clear that surgery does not cure MED, particularly cases with superior rectus paresis or with supranuclear aetiology. The goal of surgery is to improve position of affected paretic eye in primary position of gaze, to improve binocularity.

  • Inferior rectus recession with conjunctival recession: Inferior rectus recession with conjunctival recession is done in cases with inferior rectus restriction demonstrated by forced duction test.
  • Superior rectus resection along with recession of inferior rectus: Patients with large hypotropia in primary position may require superior rectus resection in addition to the usual recession of inferior rectus muscle.
  • Knapp procedure: It is done in cases with secondary inferior rectus restriction. In these cases, hypotropia persist after inferior rectus recession because of primary superior rectus palsy. Therefore, Knapp procedure is performed in these cases. This procedure involves vertical transposition of the horizontal recti muscles in addition to recession of inferior rectus muscle.
  • Modified Knapp procedure: In this procedure, partial tendon transposition is done after inferior rectus recession. This is beneficial in cases with lesser hypotropia in primary position.
  • Posterior fixation suture: This may be done in cases with large hypotropia in primary position of gaze with no inferior rectus restriction.
  • Superior rectus resection: Patients with small hypotropia with ability of upgaze above midline benefit from superior rectus resection, as an alternative to horizontal rectus transposition.
  • Ptosis surgery: This surgery may be required after strabismus surgery to decrease residual ptosis.

 

  • PUBLISHED DATE : Apr 23, 2019
  • PUBLISHED BY : NHP Admin
  • CREATED / VALIDATED BY : Dr. S. C. Gupta
  • LAST UPDATED ON : Apr 23, 2019

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