Inverse Bell's Phenomenon

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Article initiated by:
Caroline.H.Kim
All contributors:
Andrew Go Lee, MD, Noor Laylani, M.D., Caroline.H.Kim, Nagham Al-Zubidi, Nagham Al-Zubidi, MD, Noor Laylani, M.D., Mary Labowsky, MD
Assigned editor:
Mary Labowsky, MD
Review:
Assigned status Up to Date
 by Mary Labowsky, MD on November 6, 2024.


Inverse Bell’s phenomenon:

Disease Entity

Bell’s phenomenon (also known as Bell’s reflex, or palpebral oculogyric reflex) is normally an upward and lateral deviation of the eyes upon forced eyelid closure to avoid corneal exposure. An inverse Bell’s phenomenon refers to a paradoxical downwards and inwards movement of the eyeball upon forced eyelid closure (reflexive globe hypotonia). Inverse Bell’s phenomenon is a rare ophthalmic phenomenon that can be found in both children and adults. It is associated with numerous ocular and systemic conditions, including peripheral facial nerve palsy, conjunctival scarring, trauma, tabes dorsalis, eyelid swelling, ectropion of the upper lids, and extensive ptosis surgery; however, it can also be seen as part of the physiologic response to eyelid closure.[1] [2]

Levator resection to treat ptosis is one of the most frequently performed surgeries in ophthalmology with a potential early complication of a transient inverse Bell’s phenomenon. Typically, inverse Bell’s phenomenon is seen bilaterally and returns to normal in days to months without intervention following recovery postoperatively. Overall, the prognosis of inverse Bell’s Phenomenon is good with recovery, to normal.[3] Nevertheless, lubrication of the eye is recommended to prevent corneal damage.[4]

Neuroanatomy

The oculomotor nerve (cranial nerve III) has three primary motor functions and no sensory function:

  1. Innervation to the pupil and lens (autonomic: parasympathetic)
  2. Innervation to the upper eyelid (somatic)
  3. Innervation of the eye muscles that allow for visual tracking and gaze fixation (somatic)

The oculomotor nerve exits the brainstem near the midline at the base of the midbrain just caudal to the mammillary bodies. It passes through the cavernous sinus and proceeds through the supraorbital fissure to reach the orbit.[5] The oculomotor nerve elevates the upper eyelid by innervating the levator palpebrae superioris muscle with supplementation by the superior rectus muscle. Innervation to medial rectus, inferior rectus, and inferior oblique muscles adduct, retracts, and elevates the eye during adduction, respectively.[5]

Additionally, cranial nerves IV (trochlear), VI (abducens), and VII (facial) are also associated with the misdirection of oculomotor movements. Isolated injury to CN III nerve or its nucleus in the midbrain are rare, so most lesions result in complete limitation of eye movement, dilated pupil, and ptosis. CN IV or trochlear nerve similarly has its nucleus in the midbrain, innervating the contralateral superior oblique muscle and allowing for movement of the eye inferiorly when the eye is adducted. A lesion to CN IV results in a hypertropia. The final cranial nerve responsible for ocular movement is CN VI or abducens, which originates in the pons and innervates the lateral rectus muscle. An abducens lesion presents with esotropia. The median longitudinal fasciculus allows the oculomotor nuclei to communicate, allowing for the extraocular muscles to coordinate.[6][7] [8] Injury to any of these nerves, nuclei, or tracts could lead to abnormal eye movements.

Etiology

There are numerous etiologies of inverse Bell’s phenomenon including peripheral facial nerve palsy, conjunctival scarring, and ptosis surgery, with ptosis repair being the most well-documented and common cause.[9] Additionally causes include cicatricial entropion of the upper eyelids, Salzmann nodular disease, thyroid disease, or following surgical correction of monocular elevation deficiency (MED) and hypotropia.

Inverse Bell’s phenomenon may be related to the location of Salzmann’s nodules. Patients presenting with superior Salzmann nodules have been observed to exhibit inverse Bell’s phenomenon, whereas a normal Bell’s phenomenon may be preserved with inferior nodules.[10]

Pathogenesis

A proposed explanation for inverse Bell’s phenomenon is injury to the oculomotor nerve innervating the superior rectus muscle causing malfunction of the trigemino-oculomotor projection. Hyperemia or inflammation of the superior fornix can also result in abnormal function of the superior rectus and the eyelid.[4]

Similarly, an abnormality in the relationship between CN IV and VII cranial nerve nuclei could result in an abnormal response from the superior oblique and inferior rectus muscles.[5]

Nevertheless, damage to the soft tissue, edema, or inflammation relation to the superior rectus could also be a potential cause for the inverse Bell’s phenomenon considering levator resection causes some degree of intraoperative tissue manipulation.[1] Typically, inverse Bell’s phenomenon resolves in a similar timeline to the resolution of postoperative lid edema and ecchymosis following levator resection or frontalis sling suspension procedure.[1]

Epidemiology

Bell’s phenomenon has variability across the population, with about 10% of normal individuals exhibiting some deviation from the norm.[2] Approximately 8% of individuals have a downwards or inverse Bell’s phenomenon response naturally.[2] The variation among the downwards ocular movements is drawn below in Figure 4.[2]

The youngest documented case occurred in a six-year- old kid following congenital levator resection.[1] This suggests that inverse Bell’s phenomenon can affect any age ranging from childhood to adulthood.

Risk Factors

In approximately 17% of people, an inverse Bell’s phenomenon has been associated with ocular surface abnormalities (e.g., . inferior keratitis, reduced tear production, redundant conjunctiva, and severe corneal ulcer resulting in evisceration).[2] Furthermore, it may present as a complication of other ocular diseases including, but not limited to, peripheral seventh nerve palsies, tabes dorsalis, eyelid swelling, scarring of conjunctiva, ectropion of upper lid, and most commonly after ptosis repair.[11][10]

Additionally causes include cicatricial entropion of the upper eyelids, Salzmann nodular degeneration, Thyroid eye disease, or following surgical correction of monocular elevation deficiency (MED) and Hypotropia in Med Type II.

Ptosis repair for congenital ptosis, residual ptosis, or repeat surgical attempts to correct ptosis are more likely to develop inverse Bell’s phenomenon.[4] According to a 1965 study, the overall prevalence of inverse Bell’s phenomenon is 2% in normal ptosis repair postoperatively.[9] However, other studies suggest that inverse Bell’s phenomenon is correlated with the amount of levator resection.[12] In a study of large levator resections spanning from 18mm - 28mm, only two of the 32 patients presented with inverse Bell’s phenomenon.[12]

Diagnosis

History

Inverse Bell’s phenomenon is often seen in the immediate postoperative period of extensive levator resection. A detailed history of the patient’s past procedures as well as a preoperative assessment of their Bell’s reflex would be crucial to determine whether the inverse Bell’s phenomenon is deviation for the norm for that patient, or if it was part of the normal variation of extraocular movements during forced eyelid closure.

Physical examination

Gross physical examination of the Bell’s phenomenon can be observed when the examiner ask the patient to close his eyes while he simultaneously elevates the patient’s upper eyelids with his finger and thumb.[2] If the exam is positive for inverse Bell’s phenomenon, the examiner will observe the downward rolling of the eyeball during forced eyelid closure.[4] There should be no restriction of eye movement on any gaze.[9]

Clinical Diagnosis

Diagnosis of inverse Bell’s phenomenon is typically a clinical diagnosis. Pre and postoperative assessment of the Bell’s reflex is paramount following ptosis surgery and also for diseases involving progressive external ophthalmoplegia and oculomotor palsies, complications of lagophthalmos following corneal abnormality.[2]

Diagnostic procedures

Following physical postoperative ocular examination of globe movements upon eyelid closure various imaging studies can be used to determine whether the positive inverse Bell’s phenomenon is due to a neuro reflexive, iatrogenic, or other cause.

An Magnetic resonance imaging (MRI) could be performed for the following:

  • Patients younger than 50 years
  • History of cancer
  • CN III, IV, and/or VI nerve palsy
  • Optic nerve edema
  • If no marked improvement is seen after 3 months or other nerves become involved


Other testing to be considered includes Electrodiagnostic testing to stimulate CN III, IV, and VI to determine the level of oculomotor nerve injury, Electronystagmography

Management

General treatment

No proven treatment or medical therapy for inverse Bell’s phenomenon. Typically, inverse Bell’s phenomenon will spontaneously resolve in a few days to a few months following ptosis surgery.[1] An article published in 2017 found that spontaneous resolution of inverse Bell’s phenomenon three weeks following surgery potentially correlates to the decrease in tissue edema and inflammation.[12]

However, “copious lubricating eye drops, and frequent examination of the eye is required until it gets resolved.”[4]

Complications

The most likely complication due to inverse Bell’s phenomenon is the appearance of exposure keratitis following corneal exposure post-levator resection due to inadequate protection by the lower eyelid. This complication can be managed through heavy lubrication in the postoperative period.[4][13][12]

Prognosis

The prognosis for inverse Bell’s phenomenon depends on the underlying etiology. In most cases of inverse Bell’s phenomenon patients will return to normal following days to months without medical intervention.

References

  1. ↑ 1.0 1.1 1.2 1.3 1.4 Liakopoulos, D. A., Bontzos, G., & Detorakis, E. T. (2015, July 15). Late-onset inverse Bell's phenomenon after upper eyelid trauma. National Journal of Maxillofacial Surgery, 12(2), https://doi.org/131-132. 10.4103/njms.NJMS_283_20
  2. ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 Francis, I. C., & Loughhead, J. A. (1984). BELL’S PHENOMENON: A STUDY OF 508 PATIENTS. Australian Journal of Ophthalmology, 12, 15-21.
  3. ↑ Betharia, S. M., & Sharma, V. (2006). Inverse Bell’s phenomenon observed following levator resection for blepharoptosis. Graefe's Archive for Clinical and Experimental Ophthalmology, 244, 868-870. https://doi.org/10.1007/s00417-005-0122-4
  4. ↑ 4.0 4.1 4.2 4.3 4.4 4.5 Shitole, S., Jakkal, T., & Khaire, B. (2015, Mar 1). Inverse Bell’s Phenomenon: Rare Ophthalmic Finding Following Ptosis Surgery. Journal of Clinical and Diagnostic Research, 9(3). ND01–ND2. https://doi.org/10.7860/JCDR/2015/11340.5686
  5. ↑ 5.0 5.1 5.2 Joyce, C., Le, P. H., & Peterson, D. C. (2023, March 27). Neuroanatomy, Cranial Nerve 3 (Oculomotor) - StatPearls. NCBI. Retrieved June 21, 2023, from https://www.ncbi.nlm.nih.gov/books/NBK537126/
  6. ↑ Buckley, E. G., Ellis, F. D., Postel, E., & Saunders, T. (2005, Feb). Posttraumatic abducens to oculomotor nerve misdirection. Journal of AAPOS, 9(1). https://doi.org/10.1016/j.jaapos.2004.11.011.
  7. ↑ Kaufman, D. M., Geyer, H. L., Milstein, M. J., & Rosengard, J. (2022). Kaufman's Clinical Neurology for Psychiatrists. Elsevier.
  8. ↑ McGee, S. R. (2012). Evidence-based Physical Diagnosis. Elsevier/Saunders.
  9. ↑ 9.0 9.1 9.2 Pandey, T. R., Limbu, B., Sthapit, P. R., Gurung, H. B., & Saiju, R. (2019). Transient Inverse Bell’s Phenomenon Following Frontalis Sling–Suspension Ptosis Surgery: A Rare Ophthalmic Phenomenon. International Medical Case Reports Journal, 12, 325-327. https://doi.org/10.2147/IMCRJ.S216805
  10. ↑ 10.0 10.1 Nissirios, N. J., Barsam, A., Nadji, E., Donnenfeld, E., & Perry, H. D. (2013, July). Relationship of Bell Phenomenon With Salzmann Nodular Degeneration. The Journal of Cornea and External Disease, 32(7), 939-942. https://doi.org/10.1097/ICO.0b013e318281722b
  11. ↑ Dortzbach, R. K., & Callahan, A. (1971, Jan 1). Repair of Cicatricial Entropion of Upper Eyelids. JAMA Ophthalmology, 85(1), 82-89. https://doi.org/10.1001/archopht.1971.00990050084013
  12. ↑ 12.0 12.1 12.2 12.3 Goel, R., Kishore, D., Nagpal, S., Jain, S., & Agarwal, T. (2017, Feb 28). The Relationship of Amount of Resection and Time for Recovery of Bell’s Phenomenon after Levator Resection in Congenital Ptosis. The Open Ophthalmology Journal, 11, 24-30. https://doi.org/10.2174/1874364101711010024
  13. ↑ Betharia, S., & Kalra, B. (1985). Observations on Bell's phenomenon after levator surgery. Indian Journal of Ophthalmology, 33(2), 109-111.
  1. Patel, D. K., & Levin, K. H. (2015, July). Bell palsy: Clinical examination and management. Cleveland Clinic Journal of Medicine, 82(7), 419-426. https://doi.org/10.3949/ccjm.82a.14101
  2. Sanders, R. D. (2009, Nov). Cranial Nerves III, IV, and VI: Oculomotor Function. Psychiatry (Edgmont (Pa. : Township)), 6(11), 34-39.
  3. Deb, I. (2015). The Importance of Monocular Elevation Deficiency (MED) in Congenital Ptosis. AIOC, 73.
  4. Yoon, J. S., Lew, H., & Lee, S. Y. (2008, Nov 1). Bell’s Phenomenon Protects the Tear Film and Ocular Surface After Frontalis Suspension Surgery for Congenital Ptosis. Journal of Pediatric Ophthalmology & Strabismus, 45(6), 350-355. https://doi.org/10.3928/01913913-20081101-17
  5. Brown, A. C., & Nataneli, N. (2023, April 20). Salzmanns Nodular Corneal Degeneration - StatPearls. NCBI. Retrieved June 21, 2023, from https://www.ncbi.nlm.nih.gov/books/NBK560684/
  6. Na, K. S., Shin, S. J., & Yang, S.-W. (2008, August 31). Inverse Bell's Phenomenon Following Repeated Levator Resection Surgery. Journal of Korean Ophthalmology Society, 49(2), 352-356. https://doi.org/10.3341/jkos.2008.49.2.352
  7. Na, K.-S., & Yang, S.-W. (2009). Two cases of inverse Bell's phenomenon following levator resection: a contemplation of the mechanism. European Journal of Ophthalmology, 19(2), 285-287. https://doi.org/10.1177/112067210901900218
  8. Dhiman, S., Kamlesh, & Thacker, P. (2015). Efficacy of Vertical Muscle Surgery for Management of Hypotroia in Med Type II. AIOC, 73.
  9. Yang, M. C., Al-Hashimi, S., & Rootman, D. B. (2018, Aug 27). Salzmann’s nodular degeneration of cornea associated with thyroid eye disease. The International Journal on Orbital Disorders, Oculoplastic and Lacrimal Surgery, 38(4), 325-327. https://doi.org/10.1080/01676830.2018.1505921
  10. Mizukoshi, K., Watanabe, Y., Aso, S., & Asai, M. (1988). Prognostic value of blink test in patients with facial paralysis. Acta oto-laryngologica. Supplementum, 446, 70-75. https://doi.org/10.3109/00016488709121844
  11. Abraham, S. V. (1931, July). Bell's Phenomenon and the Fallacy of the Occlusion Test. American Journal of Ophthalmology, 14(7), 656-664. https://doi.org/10.1016/S0002-9394(31)91154-9.
  12. Deb, I. (2015). The Importance of Monocular Elevation Deficiency (MED) in Congenital Ptosis. AIOC, 73.
  13. Gupta, J. S., Chatterjee, A., & Kumar, K. (1965, May). Inverse Bell's Phenomenon as a Protective Mechanism. American Journal of Ophthalmology, 59, 931-933.
  14. Morawala, A., Sharma, A., & Naik, M. N. (2019). Inverse Bell’s phenomenon: a rare complication of levator resection surgery in a case of congenital ptosis. BMJ Case Reports, 12(11). https://doi.org/10.1136/bcr-2019- 232451
  15. Victoria, A. C., Chuck, R. s., Rosenberg, J., & Schwarcz, R. M. (2011, July). Timing of eyelid surgery in the setting of refractive surgery: preoperative and postoperative considerations. Current Opinion in Ophthalmology, 22(4), 226-232. https://doi.org/10.1097/ICU.0b013e328347b275
  16. Yum, C. L., Kwok, T. Y., & Yuen, H. K. (2022, 12 29). Reversal of inverse Bell’s reflex after sling removal and scar lysis in an 11-year-old patient: a case report. Hong Kong Journal of Ophthalmology, 26(2). https://www.hkjo.hk/index.php/hkjo/article/view/328
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