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Trochlear Nerve Palsy Treatment & Management

  • Author: Zafar A Sheik, MD, MD; Chief Editor: Andrew G Lee, MD  more...
 
Updated: Feb 17, 2016
 

Medical Therapy

Prisms may be used for patients with small deviations and diplopia without torsional component. Incomitance of deviation often limits usefulness of this therapy.

Botulinum toxin also has been studied in treatment of fourth nerve palsy.[17] It is a neuromuscular agent that acts presynaptically to block neurotransmitter release and results in muscle weakening. Use of this agent as primary therapy for fourth nerve palsy has been discouraging. However, it may be used best to correct residual deviation after strabismus surgery to delay or avoid further surgery.

BOTOX® is purified botulinum toxin A, derived from a culture of the Hall strain of Clostridium botulinum. It acts by binding to receptor sites on motor nerve terminals and inhibiting the release of acetylcholine. BOTOX® may be used for the treatment of strabismus and blepharospasm in patients 12 years and older. It is pregnancy category C.

Side effects for use in strabismus include ptosis and vertical deviation by action at extraocular muscles close to the site of injection. Injection should be performed under direct visualization during a surgical procedure or with the aid of electromyography.

Each vial of BOTOX® contains 100 units of botulinum toxin A in a vacuum-dried form. It needs to be reconstituted using preservative free 0.9% sodium chloride as the diluent. Doses used in strabismus range from 1.25-5 units, depending on the amount of deviation.

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Surgical Therapy

In 1970s, Knapp developed a surgical approach for superior oblique palsy.[1] He classified superior oblique palsy by determining field of gaze in which deviation was greatest. Based on this classification, he recommended operation on the muscle or muscles that acted in this direction of gaze.

See the images below.

A 2-year-old girl with compensatory left head tilt A 2-year-old girl with compensatory left head tilt due to congenital right superior oblique palsy.
Postoperative photo of same girl; note marked impr Postoperative photo of same girl; note marked improvement of head tilt.

Plager described a tailored treatment plan that evolved from Knapp's recommendations, with some additions based on more recent operative algorithms.[18] For a deviation of less than 15 prism diopters, single muscle surgery may suffice. If there is any inferior oblique overaction, inferior oblique weakening by myectomy or recession is the procedure of choice. Without any evidence of inferior oblique overaction, another muscle may be chosen. In case of ipsilateral superior rectus restriction, a superior rectus recession would be indicated. Superior oblique tendon tuck is preferred if significant tendon laxity is present, as has been described in congenital cases. Contralateral inferior rectus recession is chosen if there is no evidence of superior rectus restriction or superior oblique tendon laxity. This is an especially useful procedure when deviation is greatest in downgaze.

For deviation greater than 15 prism diopters, 2-3 muscle surgeries probably will be required. Two muscle surgery generally includes weakening of ipsilateral inferior oblique, as well as a procedure on ipsilateral superior rectus, superior oblique, or contralateral inferior rectus. For large deviations, 3-muscle surgery should be considered. Inferior oblique and contralateral inferior rectus should be weakened. Then, the surgeon may choose to operate on superior oblique or superior rectus, based on intraoperative findings.

Modified Harada-Ito procedure is useful for patients with large excyclotorsional deviation. This is likely to be the case for patients with bilateral superior oblique palsy, and bilateral surgery should be performed.[19] In this procedure, the superior oblique tendon is split and anterior fibers are advanced anteriorly and laterally.

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Preoperative Details

Careful assessment of deviation in all fields of gaze should be performed.

Multiple measurements should be taken to ensure that deviations are stable.

Ductions should be evaluated to determine if there is inferior oblique overaction.[20]

Presence of V-pattern esotropia is highly suggestive of bilateral superior oblique palsy.

It may not be possible to determine if there is superior rectus restriction in clinic, and this test may be performed in operating suite.

Photographs that show head position and ocular motility findings, including head tilts, are useful for documentation.

Infants presenting with torticollis may be suspected of having superior oblique palsy.

To differentiate true cases of strabismus from neuromuscular causes of torticollis, patch test may be performed in the office. After 20 minutes of monocular occlusion, the child is reevaluated, still wearing the patch. If head tilt was adopted for fusional purposes, it will be reduced after patching.

There is a low risk of amblyopia in affected children presumably because they can achieve intermittent fusion by using head tilt and large fusional amplitudes. Loss of compensatory head position by a child suggests loss of fusion and may be associated with development of amblyopia.

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Intraoperative Details

Patients with congenital superior oblique palsy often have abnormally lax superior oblique tendon. Exaggerated, forced duction test described by Guyton can be performed intraoperatively to determine if there is any degree of tendon laxity relative to normal eye, as follows:[21]

  • This test is performed by grasping the eye obliquely at 2- and 8-o'clock positions for the left eye and at 4- and 10-o'clock positions for the right eye.
  • Eye is rotated superiorly and medially while simultaneously depressing the eye into the orbit. This places superior oblique tendon on maximal tension.
  • Eye is rolled back and forth over the tendon to ascertain its tension.
  • Performing this test prior to the start of case will guide the surgeon to determine eyes that will benefit from a superior oblique tendon tuck.
  • Of equal importance is that it will identify those patients who are at risk for developing a postoperative Brown syndrome.
  • Tendon tucks should be performed only for markedly lax tendons. Tuck should be enough to match tension of the normal eye's tendon.

Any surgeon who performs oblique muscle surgery should be familiar with anatomy, landmarks, and appropriate approaches to these muscles.

Visualization is more difficult than with rectus muscle surgery, and injury to adjacent nerves, blood vessels, and other extraocular muscles may occur. Use of headlight can improve visualization.

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Postoperative Details

Without careful preoperative assessment, bilateral asymmetric superior oblique palsy may be mistaken for unilateral palsy. After surgery for unilateral palsy, contralateral superior oblique weakness becomes unmasked; unfortunately, then, a second surgery is required.

Patients with torsional complaints are among the most difficult to treat. Considerations are as follows:

  • In general, patients can fuse up to about 8° of cyclotropia before becoming symptomatic.
  • Patients with bilateral fourth nerve palsies from head trauma should be warned about the likelihood of persistent diplopia after surgery.
  • Many of these patients also may have central disruption of trauma from severe head injury and will be unable to fuse even after excellent surgical alignment.
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Complications

As with any strabismus surgery, undercorrections and overcorrections may occur. It is generally better to undercorrect a patient than to overcorrect a patient.

For patients with long-standing disease and large fusional amplitudes, a small residual deviation may be perfectly well controlled, but an overcorrection will be intolerable.

Adjustable suture surgery minimizes risk of overcorrection and undercorrection.

Perhaps the most troublesome complication is that of iatrogenic Brown syndrome, resulting in severe limitation of elevation.[22] Assessing superior oblique tendon intraoperatively should make this less likely.

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Outcome and Prognosis

Prognosis of trochlear nerve palsy varies depending on etiology. Best information regarding outcome comes from cases collected at the Mayo Clinic over the past 40 years, as follows:

  • Recovery is most likely in patients whose superior oblique palsy is secondary to microvascular disease.
  • Idiopathic cases also have greater than 50% likelihood of spontaneous recovery.
  • Most cases resolve within weeks to months, with the vast majority completely recovering by 6 months.
  • Some cases may resolve slowly over the course of a year.
  • Patients with head trauma were less likely to recover, yet, nearly 50% of these patients showed some degree of improvement.
  • Cases due to aneurysm or neoplasm were least likely to have functional recovery.

Because patients have good fusional abilities, surgery generally produces excellent results. Plager reported a nearly 90% success rate with his surgical algorithm.[18] Mitchell and Parks also reported excellent results in correcting excyclotorsion using modified Harada-Ito procedure.[19]

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Contributor Information and Disclosures
Author

Zafar A Sheik, MD, MD 

Zafar A Sheik, MD, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, International Society of Refractive Surgery

Disclosure: Nothing to disclose.

Coauthor(s)

Kelly A Hutcheson, MD, MBA Associate Professor, Department of Ophthalmology, George Washington University School of Medicine, Children's National Medical Center

Kelly A Hutcheson, MD, MBA is a member of the following medical societies: Alpha Omega Alpha, Association for Research in Vision and Ophthalmology, American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus

Disclosure: Nothing to disclose.

Specialty Editor Board

Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

Chief Editor

Andrew G Lee, MD Chair, Department of Ophthalmology, Houston Methodist Hospital; Clinical Professor, Associate Program Director, Department of Ophthalmology and Visual Sciences, The University of Texas Medical Branch; Clinical Professor, Department of Surgery, Division of Head and Neck Surgery, University of Texas MD Anderson Cancer Center; Professor of Ophthalmology, Neurology, and Neurological Surgery, Weill Medical College of Cornell University; Clinical Associate Professor, University of Buffalo, State University of New York School of Medicine

Andrew G Lee, MD is a member of the following medical societies: American Academy of Ophthalmology, Association of University Professors of Ophthalmology, American Geriatrics Society, Houston Neurological Society, Houston Ophthalmological Society, International Council of Ophthalmology, North American Neuro-Ophthalmology Society, Pan-American Association of Ophthalmology, Texas Ophthalmological Association

Disclosure: Received ownership interest from Credential Protection for other.

Additional Contributors

Edsel Ing, MD, FRCSC Associate Professor, Department of Ophthalmology and Vision Sciences, University of Toronto Faculty of Medicine; Consulting Staff, Hospital for Sick Children and Sunnybrook Hospital

Edsel Ing, MD, FRCSC is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Society of Ophthalmic Plastic and Reconstructive Surgery, Royal College of Physicians and Surgeons of Canada, Canadian Ophthalmological Society, North American Neuro-Ophthalmology Society, Canadian Society of Oculoplastic Surgery, European Society of Ophthalmic Plastic and Reconstructive Surgery, Canadian Medical Association, Ontario Medical Association, Statistical Society of Canada, Chinese Canadian Medical Society

Disclosure: Nothing to disclose.

Acknowledgements

Brian R Younge, MD Professor of Ophthalmology, Mayo Clinic School of Medicine

Brian R Younge, MD is a member of the following medical societies: American Medical Association, American Ophthalmological Society, and North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

References
  1. Knapp P. Classification and treatment of superior oblique palsy. Am Orthopt J. 1974. 24:18-22. [Medline].

  2. Miller NR, Newman NJ. Walsh and Hoyt's Clinical Neuro-Ophthalmology. 5th ed. 1998. 1227-1237.

  3. Madigan WP, Zein WM. Recent developments in the field of superior oblique palsies. Curr Opin Ophthalmol. 2008 Sep. 19(5):379-83. [Medline].

  4. Richards BW, Jones FR Jr, Younge BR. Causes and prognosis in 4,278 cases of paralysis of the oculomotor, trochlear, and abducens cranial nerves. Am J Ophthalmol. 1992 May 15. 113(5):489-96. [Medline].

  5. Holmes JM, Mutyala S, Maus TL, et al. Pediatric third, fourth, and sixth nerve palsies: a population-based study. Am J Ophthalmol. 1999 Apr. 127(4):388-92. [Medline].

  6. Kodsi SR, Younge BR. Acquired oculomotor, trochlear, and abducent cranial nerve palsies in pediatric patients. Am J Ophthalmol. 1992 Nov 15. 114(5):568-74. [Medline].

  7. Brazis PW. Palsies of the trochlear nerve: diagnosis and localization--recent concepts. Mayo Clin Proc. 1993 May. 68(5):501-9. [Medline].

  8. Keane JR. Fourth nerve palsy: historical review and study of 215 inpatients. Neurology. 1993 Dec. 43(12):2439-43. [Medline].

  9. Robb RM. Idiopathic superior oblique palsies in children. J Pediatr Ophthalmol Strabismus. 1990 Mar-Apr. 27(2):66-9. [Medline].

  10. Rush JA, Younge BR. Paralysis of cranial nerves III, IV, and VI. Cause and prognosis in 1,000 cases. Arch Ophthalmol. 1981 Jan. 99(1):76-9. [Medline].

  11. Son S, Park CW, Yoo CJ, Kim EY, Kim JM. Isolated, contralateral trochlear nerve palsy associated with a ruptured right posterior communicating artery aneurysm. J Korean Neurosurg Soc. 2010 May. 47(5):392-4. [Medline]. [Full Text].

  12. Helveston EM, Krach D, Plager DA, Ellis FD. A new classification of superior oblique palsy based on congenital variations in the tendon. Ophthalmology. 1992 Oct. 99(10):1609-15. [Medline].

  13. Phillips PH, Hunter DG. Evaluation of ocular torsion and principles of management. In: Rosenbaum AL, Santiago AP, eds. Clinical Strabismus Management. WB Saunders. 1999:52-72.

  14. Kono R, Okanobu H, Ohtsuki H, Demer JL. Absence of relationship between oblique muscle size and bielschowsky head tilt phenomenon in clinically diagnosed superior oblique palsy. Invest Ophthalmol Vis Sci. 2009 Jan. 50(1):175-9. [Medline].

  15. von Noorden GK, Murray E, Wong SY. Superior oblique paralysis. A review of 270 cases. Arch Ophthalmol. 1986 Dec. 104(12):1771-6. [Medline].

  16. Graf M, Weihs J. Effect of diagnostic occlusion in acquired trochlear nerve palsy. Graefes Arch Clin Exp Ophthalmol. 2009 Feb. 247(2):253-9. [Medline].

  17. Garnham L, Lawson JM, O'Neill D, Lee JP. Botulinum toxin in fourth nerve palsies. Aust N Z J Ophthalmol. 1997 Feb. 25(1):31-5. [Medline].

  18. Plager DA. Superior oblique palsy and superior oblique myokymia. In: Clinical Strabismus Management: Principles and Surgical Techniques. 1999:219-229.

  19. Mitchell PR, Parks MM. Surgery of bilateral superior oblique palsy. Ophthalmology. 1982 May. 89(5):484-8. [Medline].

  20. Kushner BJ. Overaction of the inferior oblique muscle in 4th nerve palsy. Binocul Vis Strabismus Q. 2008. 23(4):198-9. [Medline].

  21. Guyton DL. Exaggerated traction test for the oblique muscles. Ophthalmology. 1981 Oct. 88(10):1035-40. [Medline].

  22. Lee J. Management of Brown syndrome. Semin Ophthalmol. 2008 Sep-Oct. 23(5):291-3. [Medline].

 
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A 2-year-old girl with compensatory left head tilt due to congenital right superior oblique palsy.
Postoperative photo of same girl; note marked improvement of head tilt.
Patient with traumatic bilateral superior oblique palsy; note right hypertropia on right head tilt and left hypertropia on left head tilt.
 
 
 
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