Correspondence: Dr Ament, Department of Ophthal-

mology, Massachusetts Eye and Ear Infirmary, Harvard

Medical School, 243 Charles St, Boston, MA 02114-
4724 (jaredament@post.harvard.edu).
Author Contributions: Drs Ament and Pineda had full
access to all of the data in the study and take responsi-
bility for the integrity of the data and the accuracy of the
data analysis.
Financial Disclosure: The authors do not have any com-
mercial or proprietary interest in the Boston KPro, nor do
they have any financial interest or receive payment as a
consultant, reviewer, or evaluator. Dr Ament is a clinical
research fellow under Claes Dohlman, MD, PhD, creator
of the Boston KPro. Dr Dohlman has no financial interest
in the Boston KPro. He makes no profit from its sales. All
proceeds support continuing research and medical mis-
sions to Africa and other nonindustrialized nations. Figure 1. External photograph of the patient with complete ptosis of the left eye.
Role of the Sponsor: Dr Dohlman was not involved in
the design and conduct of the study; the collection, man-
agement, analysis, and interpretation of the data; or the
preparation, review, or approval of the manuscript.
Additional Contributions: Through a research and de-
velopment fund, Dr Dohlman donated the KPro devices
and provided support for the medical trips. Khalil
Lakho, MD, an administrator in Sudan, provided logis-
tical support, clinic time, operating room time, and co-
ordination of efforts with ophthalmology staff and resi-
dents. Tania Marie Ament, BS, modified and conducted
activities of daily living, instrumental activities of daily
living, and quality-of-life surveys.
1. Cardona H. Mushroom transcorneal keratoprosthesis (bolt and nut). Am J
Ophthalmol. 1969;68(4):604-612.
2. Ament JD, Pineda R, Lawson B, Belau I, Dohlman CH. The Boston Kerato-
prosthesis: international protocol, version 2, June 15, 2009. http://www
.masseyeandear.org/gedownload!/KPro%20International%20Protocol2.pdf
Figure 2. Complete third nerve palsy with pupillary involvement in the left eye.
?item_id=5816015&version_id=5816016. Accessed August 15, 2009.
3. World Health Organization. Blindness. http://www.who.int/topics/blindness palsy, speculate about the neuronal pathways, and educe
/en/. Accessed June 16, 2009.
4. Katz S, Ford AB, Moskowitz RW, Jackson BA, Jaffe MW. Studies of illness in
its diagnostic and therapeutic implications.
the aged: the index of ADL: a standardized measure of biological and psy-
chosocial function. JAMA. 1963;185:914-919. Report of a Case. A 10-year-old girl had complete droop-
5. Boisjoly H, Gresset J, Fontaine N, et al. The VF-14 index of functional visual
impairment in candidates for a corneal graft. Am J Ophthalmol. 1999;128(1): ing of the left eyelid with the globe fixed in abduction
38-44. (Figure 1). Her birth and family history were unremark-
6. Aldave AJ, Kamal KM, Vo RC, Yu F. The Boston type I keratoprosthesis: im- able. Aided visual acuity was 20/20 OD and 20/60 OS. An
proving outcomes and expanding indications. Ophthalmology. 2009;116(4):
640-651. isolated left complete pupillary-involving third nerve palsy
with no signs of aberrant regeneration was noted
(Figure 2). On pulling the left upper eyelid margin down
Proprioceptive Transient Elevation of Ptotic with her finger, her ptotic eyelid reflexively elevated by 6
Eyelid and Lacrimation in Congenital Third to 8 mm, drifting back in 30 to 50 seconds. Profuse lacri-
Nerve Palsy: The Monosynaptic Stretch mation followed (Figure 3 and video, http://www
.archophthalmol.com). The pupil, other muscles sup-
or Hoffmann Reflex Gone Awry?
plied by the third nerve, and the contralateral eyelid were
unaffected. The phenomenon could be repeated immedi-

A lthough proprioceptive structures seen in anti-

gravity muscles like jaw-closing muscles are lack-
ing in levator palpebrae superioris muscle (LPSM),
another antigravity muscle, Müller muscle (MM), may act
ately thereafter and was not abolished by local anesthesia.
There was no jaw wink or associated salivation. Results from
the rest of the examination and magnetic resonance imaging
of the brain and orbits were unremarkable.

Video available online at Comment. Proprioceptive structures, muscle spindles,

www.archophthalmol.com
like one for LPSM.1 We report stretch-induced ephemeral
eyelid elevation of the completely ptotic eyelid followed by
copious lacrimation in a girl with congenital third nerve
and palisade endings exist in the global but not orbital
layer of human extraocular muscles. Distal myotendi-
nous junctions, the areas traumatized in most strabis-
mus procedures, are most richly endowed. The informa-
tion they relay, however, remains controversial.2 Levator

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©2010 American Medical Association. All rights reserved.

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Figure 3. Reflexive left upper eyelid elevation and lacrimation on
proprioceptive stimulation of the left upper eyelid.
palpebrae superioris muscle lacks a global layer as well
as proprioceptive structures.1,3 Its orbital layer has 2 types
of muscle fibers. Most (80%) are fast-twitch, singly in-
nervated, fatigue-resistant fibers and the rest (20%) are
slow-twitch, multiply innervated, fatigable fibers.3 The
latter likely have an MM-mediated proprioceptive role,
reflexively contracting in response to voluntary contrac-
tion of the former.1,4,5 Intraoperative stretching of MM
is known to induce ipsilateral or bilateral involuntary con-
traction of LPSM.1 Thick (proprioceptive) and thin (sym-
pathetic) nerve fibers on proximal and distal MM are
shown to pass through the palpebral lobe of the lacri-
mal gland to join the lacrimal nerve. Electrical stimula-
tion of thick but not thin fibers consistently results in
involuntary retraction of the upper eyelid (Hoffmann
reflex).1 Ban et al4 electromyographically verified the pres-
ence of monosynaptic trigemino-oculomotor reflex os-
tensibly mediated via mesencephalic and central caudal
nuclei of the fifth and third cranial nerves and specu-
lated that it may account for inexplicable ptosis follow-
ing trauma, surgery, or tumor removal in the aponeu-
rotic area of LPSM. The horseradish peroxidase technique
has shown that fibers from the oculomotor nerve enter-
ing the trigeminal (ophthalmic) nerve are afferent in na-
ture.5 A hyperactive monosynaptic trigemino-oculomotor
reflex resulting from stretch by multiply innervated slow-
twitch fibers as elaborated earlier1,4,5 likely gave rise to sus-
tained, fatigable eyelid elevation here.
As the proprioceptive fibers run through the lacrimal
gland and nerve,1 the lacrimation that follows could be
due to cross talk between proprioceptive or sympa-
thetic fibers with secretomotor fibers from superior sali-
vatory nucleus. Sparing of other extraocular muscles sup-
(REPRINTED) ARCH OPHTHALMOL / VOL 128 (NO. 6), JUNE 2010
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©2010 American Medical Association. All rights reserved.
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plied by the third cranial nerve further buttresses
proprioceptive underpinnings. Such a monosynaptic re-
flex, conterminous with a jaw-jerk reflex, is quite dis-
tinct from motor miswiring resulting in a jaw-winking
phenomenon in congenital ptosis.
Proprioceptive ephemeral eyelid elevation and lacri-
mation induced by stretching of the eyelid (MM) has not
been reported to our knowledge. The phenomena, har-
nessed and modulated pharmacologically, may have tre-
mendous diagnostic and therapeutic potential in enti-
ties like dry eye, thyroid eye disease, ocular myasthenia,
pathological eyelid retraction, acquired ptosis, eyelid
trauma, surgery involving the LPSM aponeurosis, and nu-
merous neuromuscular disorders. Alleviation of eyelid
retraction in thyroid eye disease by triamcinolone ace-
tonide injections in MM is a case in point.6
Pramod Kumar Pandey, MD
Subhash Dadeya, MD
Pankaj Vats, MS, DNB
Pooja Jain, MS, DNB
Ashish Amar, MS
Mihir Kumar Sahoo, DOMS
Anupam Singh, MS
Author Affiliations: Department of Ophthalmology, Guru
Nanak Eye Centre, Maulana Azad Medical College, New
Delhi, India.
Correspondence: Dr P. K. Pandey, Department of Oph-
thalmology, Guru Nanak Eye Centre, Maulana Azad Medi-
cal College, New Delhi, Ne 110002, India (pkpandey
_001@yahoo.co.in).
Financial Disclosure: None reported.
Online-Only Material: A video is available at http://www
.archophthalmol.com.
1. Yuzuriha S, Matsuo K, Ishigaki Y, Kikuchi N, Kawagishi K, Moriizumi T.
Efferent and afferent innervations of Mueller’s muscle related to involuntary
contraction of the levator muscle: importance for avoiding injury during eye-
lid surgery. Br J Plast Surg. 2005;58(1):42-52.
2. Weir CR, Knox PC, Dutton GN. Does extraocular muscle proprioception in-
fluence oculomotor control? Br J Ophthalmol. 2000;84(9):1071-1074.
3. Porter JD, Baker RS, Ragusa RJ, Brueckner JK. Extraocular muscles: basic and
clinical aspects of structure and function. Surv Ophthalmol. 1995;39(6):451-
484.
4. Ban R, Matsuo K, Osada Y, Ban M, Yuzuriha S. Reflexive contraction of leva-
tor palpebrae superioris muscle to involuntarily sustain the effective eyelid
retraction through the transverse trigeminal proprioceptive nerve on the proxi-
mal Mueller’s muscle: verification with evoked electromyography. J Plast Re-
constr Aesthet Surg. 2010;63(1):59-64.
5. Atasever A, CÈ elik HH, Durgun B, Yilmaz E. The course of the proprioceptive
afferents from extrinsic eye muscles. Turk Neurosurg. 1992;2(4):183-186.
6. Chee E, Chee S-P. Subconjunctival injection of triamcinolone in the treat-
ment of lid retraction of patients with thyroid eye disease: a case series. Eye
(Lond). 2008;22(2):311-315.
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