Strabismus

ISSN: 0927-3972 (Print) 1744-5132 (Online) Journal homepage: http://www.tandfonline.com/loi/istr20

Surgery for Supranuclear Monocular Elevation

Deficiency

Michael C. Struck MD & Jennifer C. Larson MD

To cite this article: Michael C. Struck MD & Jennifer C. Larson MD (2015) Surgery
for Supranuclear Monocular Elevation Deficiency, Strabismus, 23:4, 176-181, DOI:
10.3109/09273972.2015.1099710

To link to this article: http://dx.doi.org/10.3109/09273972.2015.1099710

Published online: 15 Dec 2015.

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 Strabismus, 2015; 23(4): 176–181
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ISSN: 0927-3972 print / 1744-5132 online
DOI: 10.3109/09273972.2015.1099710

ORIGINAL ARTICLE

Surgery for Supranuclear Monocular Elevation

Deficiency
Michael C. Struck, MD and Jennifer C. Larson, MD

Department of Ophthalmology, University of Wisconsin – Madison, Madison, WI, USA

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ABSTRACT
Purpose: We report a novel approach to surgery for monocular elevation deficiency (MED).
Methods: A retrospective review of 5 patients undergoing surgery for supranuclear MED between 2003 and
2014. All patients had intact Bell’s phenomenon, hypotropia, limited elevation above the primary position, and
negative forced duction testing of the paretic eye. Preoperatively all patients preferred chin-up head posture
and three had pseudoptosis or ptosis. One of the 5 had prior vertical muscle surgery. Surgery correction for the
MED consisted of near maximal superior rectus recession on the contralateral sound eye.
Results: Compensatory chin-up head position and alignment in primary position was improved in all patients.
Average age at surgery was 5.3 years. Average superior rectus recession was 9.7 mm. Mean follow-up was 4.8
years (range 12 months to 11.5 years). The vertical deviation of the paretic eye in primary position
postoperatively was orthotropic for 2, hypotropic for 2, and overcorrected for 1.
Conclusions: In cases of supranuclear MED (double elevator palsy) contralateral superior rectus recession based
on the innervational principle is a simple and reliable alternative surgical approach compared to published
results of the Knapp transposition procedure. Additionally, it holds the possibility for decreased complications
and less complicated future surgical treatment options.
Keywords: Double elevator palsy, hypertropia, innervational surgery, Knapp procedure, monocular elevation
deficiency, strabismus surgery, supranuclear palsy

INTRODUCTION between the different etiologies of MED can be

Monocular elevation deficiency (MED) is defined as
the inability to elevate one eye equally in abduction,
adduction, and primary gaze. MED was originally
termed double elevator palsy (DEP) when the dis-
order was thought to be caused by paresis of the two
eye elevators, the superior rectus and inferior obli-
que.1 Currently MED is categorized into 3 major
pathophysiologic disorders: superior rectus paresis,
inferior rectus or superior oblique restriction, and
supranuclear disturbance.2–6 We report here only on
patients with supranuclear (SNP) MED.
The affected eye in MED typically presents with
hypotropia, ptosis, or pseudoptosis, and when the
affected eye fixates, a large secondary vertical devi-
ation of the sound eye (Figure 1b).7 Distinguishing
achieved by examining for the presence of Bell’s
phenomenon bilaterally. An intact Bell’s phenomenon
is pathognomic for a supranuclear disturbance.7 An
absent Bell’s phenomenon infers a different etiology,
such as an inferior rectus restriction or superior rectus
palsy. In a cooperative patient, inferior rectus restric-
tion, Brown syndrome, and superior rectus palsy can
also be differentiated by saccadic velocity analysis,
forced ductions, and/or active force generation.7
Innervational surgery is based on the innervation
effect, which was first described by Guibor in 1958.8
He explained how placing a base out prism in front of
the sound eye prevented contracture of the medial
rectus of the contralateral, paretic eye in patients with
unilateral sixth nerve palsy. Later this technique was
applied to the surgical treatment of esotropia in 6th

Received 4 March 2015; Revised 31 July 2015; Accepted 19 August 2015; Published online 8 December 2015
Correspondence: Michael C. Struck, MD, Associate Professor, Department of Ophthalmology, University of Wisconsin – Madison, 2870
University Ave. #206, Madison, WI 53705. Tel: 608-263-9859. E-mail: mcstruck@wisc.edu

176

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FIGURE 1. (1a) Pre-operation compensatory head posture with
chin-up positioning. (1b) Pre-operation force primary position.
(1c) After the final surgery at age 2 years.
nerve palsy.9 In 1978, Horta-Barbosa applied this
innervational principle to the surgical management of
unilateral third nerve palsy.10 The concept invokes
stimulation of the affected contralateral agonist
and/or inhibition the contralateral antagonist by the
accepted principles of Herring and Sherrington’s laws
of innervation. SNP MED attempts to increase the
resting tonus of the elevators of the affected eye by
surgically weakening the contralateral agonist,
thereby increasing the required innervational input
to both. Prieto-Diaz and Gamio11 employed this
technique in MED. We report our results using this
technique in SNP MED.
METHODS
Surgical records for all children under 18 years,
without 3rd nerve palsy, who underwent surgery for
MED were reviewed between 2003 and 2014. Thirteen
records were recovered; of these, 3 patients had
confirmed congenital fibrosis of the extraocular
muscles and 5 had restrictive elevation. The remain-
ing 5 subjects were reviewed retrospectively after
contralateral superior rectus recession for SNP MED
by the senior author (MCS). The diagnosis of mon-
ocular elevation deficiency was made based on the
history of paretic eye hypotropia and sound eye
hypertropia when the paretic eye fixates, as well as a
intact Bell’s phenomenon and negative passive forced
! 2015 Taylor & Francis Group, LLC
Surgery for MED 177
duction testing. Patients were excluded if they had
restrictions on forced duction testing, and/or absent
Bell’s phenomenon.
Data collected included preoperative and post-
operative assessment of ocular motility and head
posture, and measurements at forced primary pos-
ition using the Krimsky test or the alternating
cover test, with the prism over the MED eye. Two
patients were cooperative for preoperative provoca-
tive prism testing, illustrated in case 2 below. In
this test, previously described (Steve Archer, per-
sonal communication, 2013) in Duane syndrome
for similar preoperative assessment of surgical pre-
dictability, the prism is placed over the sound eye,
which remains the fixing eye, and the change in devi-
ation of the contralateral eye is assessed for the poten-
tial response to surgical correction of the sound
eye. For all patients the surgery consisted of treat-
ment of contralateral (sound eye) superior rectus
recession. Maximum surgery was attempted, not
based on preoperative measurements, without
interfering with the position of the superior oblique
tendon, transection of the superior oblique to super-
ior rectus frenulum, and directly suturing the
muscle to the sclera 9 to 10.5 mm from the original
insertion.
This study was approved by the Institutional
Review Board of University of Wisconsin and was
compliant with the Health Insurance Portability and
Accountability Act, and was in adherence to the
Declaration of Helsinki.
RESULTS
Compensatory chin-up head position and alignment
in primary position were improved in all patients.
Likewise the pseudo-ptosis improved in all patients,
but was not eliminated. Table 1 shows the results on
an individual basis. Average age at surgery was 5.3
years, range 7 months to 9 years. Average superior
rectus recession was 9.7 mm. Mean follow-up was 4.8
years (range 12 months to 11.5 years). Average
surgical effect was 21 prism diopters of vertical
shift for superior rectus surgery alone. The vertical
deviation of the paretic eye in primary position
postoperatively was orthotropic for 2 and a small
residual hypotropia for 2. One patient (#4), with
the smallest preoperative deviation, initially had a
small undercorrection but over time developed a
stable overcorrection. One patient (#1) initially was
well corrected, but one year after initial surgery,
required a second surgery for recurrence. One
patient had had a prior vertical rectus surgery for
MED at 16 months of age, with no restriction of the
ipsilateral IR muscle noted at the time of either
surgery, and at 8 years underwent the procedure
described.
 178 M. C. Struck and J. C. Larson

TABLE 1. Characteristics of case study subjects.

Case No. 1 Case No. 2 Case No. 3 Case No. 4 Case No. 5 Average

Age at surgery 11 months 8 years 9 years 7 months 8 years* 5.3 years

Length SR recession 10 mm 10.5 mm 9.0 mm 9.0 mm 10 mm 9.7 mm
Length follow-up 2.2 years 2.5 years 11.5 years 7.25 years 1 year 4.8 years
Prior surgery None None MROU None IROD 4 mm rec
recession SROD 4 mm
resect
Pre-op hypertropia in 1 Rhypo 25 Rhypo 30 LHypo 30 Rhypo 14 Rhypo 22 24 PD
1 month post-op hypertropia in 1 orthophoria Rhypo 8 Lhypo 12 RHT 6 orthotropic 5 PD
Correction of vertical deviation 25 PD 22 PD 18 PD 20 PD 22 PD 21 PD

*Initial VR muscle surgery at 16 months of age for MED.

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FIGURE 2. An intact Bell’s phenomenon illustrated by the eye’s

ability to elevate indicating a supranuclear etiology of MED.

ILLUSTRATIVE CASE REPORT

Case 1

A 6-month-old boy presented with right eye SNP

MED illustrated by secondary deviation (Figure 1) of
greater than 25 PD left hypertropia. He also presented
with a preferred chin-up head position of at least 20
degrees and pseudoptosis. At 11 months, he under-
went left superior rectus recession of 10 mm. In the FIGURE 3. (3a) Pre-operation forced primary position. (3b) Pre-
first 6 months postoperative he was orthotropic, operation prism test, left eye fixing through a 35-PD base down
however at the age of 2 years he had recurrent right prism. (3c) Post-operation forced primary position, 8pd right
hypotropia and chin up compensatory head posture hypotropia, improved ptosis.
and underwent ipsilateral IR recession of 5.5 mm.
Currently, he is orthophoric in primary position, and
has not required eyelid surgery. DISCUSSION

Standard surgical treatment for MED, with an eti-

Case 2
An 8-year-old boy was followed since birth for a right
eye SNP MED characterized by 30 PD right hypo-
tropia, 25-degree chin-up head posture, and right
ptosis. His intact Bell’s phenomenon is illustrated
below (Figure 2). Provocative preoperation prism
testing was conducted in an attempt to predict
surgical response (Figure 3). Surgical correction con-
sisted solely of a left superior rectus recession of
10.5 mm. He has been followed postoperatively for 2.5
years. He has residual 8 PD right hypotropia when
fixating with his left eye. He has a reduced chin-up
head position of approximately 4 degrees.
ology of SNP deficit, relies on full tendon superior
transposition of the horizontal rectus muscles (Knapp
procedure).12,13 The original Knapp procedure today
is usually modified so that the horizontal rectus
muscles are attached along the spiral of Tillaux.14
Inferior rectus recession has supplemented the Knapp
procedure for cases with either concurrent inferior
rectus restriction, recurrence (undercorrection), or to
augment the effect.4,8,9,14–19
Table 2 is an analysis of the reported results
following Knapp procedure. This represents the out-
comes for different MED etiologies following trad-
itional Knapp procedures (full tendon transposition)
and modified Knapp procedures (partial tendon
Strabismus
 Surgery for MED 179

TABLE 2. Summary of horizontal rectus muscle transposition procedures (Knapp procedure).

Number of Average vertical

Author / year cases correction (PD) Procedure

Watson / 196220 2 30 Knapp

Knapp / 196912 15 38 Knapp (13 cases) and Knapp + simultaneous IRc (2 cases)
Harley / 197113 4 25-40 Knapp
Cooper / 197121 6 26 Knapp
Dunlap / 197128 2 35 Knapp partial transposition  10mm
Scott / 19774 5 37 Prior IRc followed by Knapp
1 0 Knapp
Barsoum-Homsy / 198323 2 29 Knapp (full tendon transposition)
2 17 Modified Knapp (partial transposition)
Lee / 198618 4 17 Knapp
3 35 Knapp + simultaneous IRc
Burke / 199217 13 21 Knapp
6 38 Prior IRc followed by Knapp
Kocak / 200015 5 20 Knapp (full transposition)
3 17 Modified Knapp (partial transposition)
3 22 Prior IRc followed by Knapp (full tendon transposition)
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2 8 Prior IRc followed by modified Knapp (partial transposition)

Caldeira / 200024 10 36 Knapp
Snir / 200522 6 9 Knapp
Bagheri / 200816 8 29 Knapp
5 32 Knapp + IRc
Zafar / 201219 12 26 Knapp
10 32 Prior IRc followed by Knapp

Note: Reported average vertical correction following Knapp procedure.

transposition) alone or in combination with inferior
rectus recession at varying lengths of follow-up.
Complications of the Knapp procedure include under-
correction with the need for subsequent ipsilateral
IR recession, overcorrection, and residual ptosis.
A significant and difficult complication of Knapp
procedure includes overcorrection.17,19,24 Burke et al.
were the first to describe how the magnitude of
vertical correction after Knapp procedure may pro-
gress. Reversal of a full tendon transposition proced-
ure has been reported to be difficult and
unpredictable.25 Zafar reported 3 patients who were
overcorrected with the Knapp procedure, and despite
reversal of the Knapp, the overcorrection persisted.19
We have had the same experience in one case.
Additionally, Knapp procedure associated with sim-
ultaneous recession-resection for a horizontal devi-
ation has been associated with cyclodeviation.15,26
Contralateral superior rectus muscle provides an
appealing alternative treatment for cases of supra-
nuclear MED. In our study, the mean vertical devi-
ation correction was 21 PD, similar to results for
Knapp alone. Comparing postoperative results of the
innervational method to the published results of the
Knapp procedure is difficult because our study is
limited to 5 patients and studies listed in Table 2 do
not segregate the mixture of etiologies for MED. The
preoperative and postoperative vertical deviations
weren’t reported in Preito-Diaz’s study, preventing
comparison.
Essentially the innervational principle involves
functionally weakening the agonist muscle in the
! 2015 Taylor & Francis Group, LLC
sound eye with surgery, prisms, or botulinum toxin in
order to increase the innervation to, and thus function
of, its paretic yoke muscle via Hering’s Law.
Additionally, the increased innervation will poten-
tially weaken ipsilateral antagonists via Sherrington’s
law resulting in potential further improved function
of the paretic muscle.9,27 Importantly the weak muscle
must be paretic and not paralyzed for the innerva-
tional effect to work. The nerve and muscle of the
affected eye in supranuclear MED is functional, as
noted by the intact Bell’s reflex.
Applying this principle to SNP MED, the sound
eye superior rectus is recessed, therefore stimulating
an ipsilateral increased innervation required to main-
tain primary position, which then results in a contra-
lateral increased innervation transmitted to the yoked,
SNP superior rectus via Hering’s law. This results in
increased tonic stimulation of the SNP muscle and
elevation of the affected eye. The increased innerv-
ation to the SNP superior rectus may also provide
additional benefit of stimulation of the levator
palpebre and facilitate relaxation of the antagonist
inferior rectus via Sherrington’s law, which may
further improve the elevation of the paretic eye. This
treatment was first described Preito-Diaz,11 however,
it is unclear if the patients included in his study had
SNP MED because they were reported as not having
intact Bell’s phenomenon.
Predicting surgical effects of innervational surgery
in a cooperative patient with a simple prism test is
helpful as demonstrated in case 3 (Figure 2). This test
involves placing a prism over the sound eye, while it
 180 M. C. Struck and J. C. Larson
remains the fixing eye, in order to estimate the
innervational effects surgery will create in the affected
eye. Although it is difficult to overcorrect the devi-
ation because the underlying pathology is a SNP
disturbance and innervational surgery will not result
in overstimulation of the paretic muscle, we did see
progressive overcorrection in one case. Only 2 of our
patients were able to cooperate with this test. In
general, the maximum amount of recession feasible
should be performed, avoiding the superior oblique
tendon.
In both our study and in Prieto-Diaz’s study, all
patients demonstrated improvement of ptosis post-
operatively. Several studies have shown limited
improvement, no improvement, or worsening of
ptosis following the Knapp procedure.12,17,28–30 This
difference can be explained by the different principles
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used in the surgeries. In the Knapp procedure, the
globe elevation is achieved only by static vector
forces. In contrast, in the innervational procedure,
innervation is actually being increased to the superior
rectus and as a corollary the levator palpebrae of the
paretic eye.
In conclusion, compared to the Knapp transpos-
ition procedure, the innervational method provides an
active physiologic response rather than a static eleva-
tion force. It achieves a similar amount of vertical
shift, is technically easier to perform, and is associated
with better ptosis correction. It leaves more options
for further strabismus treatment, is potentially revers-
ible, and potential decreased complications may
decrease the need for future muscle surgeries.
DECLARATION OF INTEREST
The authors declare no conflicts of interest.
Literature Search: PubMed and MEDLINE were
searched up to May 2014 for monocular elevation
deficiency AND each of the following: treatment,
surgery, Knapp procedure, innervational surgery, and
natural history.
Funding: This work was supported in part by an
unrestricted grant from Research to Prevent Blindness.
Contributors: MCS: conception and design, collection
of data, data analysis, interpretation of data,
drafting the manuscript, approval of manuscript;
JCL: collection of data, data analysis, drafting the
manuscript.
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