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Dissociative phenomena in congenital monocular elevation deficiency
Dissociative Phenomena in Congenital Monocular Elevation Deficiency Richard
1. Olson!
MD,a
and William
E. Scott,
MDb
Introduction: Monocular elevation deficiency is characterized by unilateral limitation of elevation in both adduction and abduction and is usually present at birth. Dissociative phenomena such as dissociated vertical deviation are well recognized in association with conditions such as congenital esotropia but much less so in association with conditions such as congenital monocular elevation deficiency. Mefhods:All 129 patients given the diagnosis of monocular elevation deficiency or double elevator palsy in the Pediatric Ophthalmology and Strabismus Clinic at the University of Iowa Hospitals and Clinics between 1971 and 1995 were reviewed. After those with history of trauma, myasthenia gravis, thyroid eye disease, orbital lesions, Brown syndrome, or monocular elevation deficiency with acquired onset were excluded, 31 patients with congenital monocular elevation deficiency remained for retrospective study. Resulfs: First diagnosed at median age 2.6 years (although all were noted by parents at less than 6 months of age) with mean follow-up of 5.0 years (up to 15.5 years), 9 of 31 (29%) developed dissociated vertical deviation in the eye with monocular elevation deficiency, all of whom had undergone strabismus surgery 0 to 9.7 years previously (mean 3.5 years). Those who developed dissociated vertical deviation were generally younger, were followed up longer, and had more accompanying horizontal strabismus than did those who did not develop dissociated vertical deviation. The results did not reach significance. Conc/usion:The current study demonstrates that dissociated vertical deviation occurs in association with monocular elevation deficiency. (J AAPOS 1998;2:72-8)
D
issociative phenomena in strabismus have been described for more than a century1 and under many different names, encompassing vertical, horizontal, torsional strabismus noted during inattention or under cover, which is dissociated (i.e., does not follow Hering’s law).2 The currently accepted clinical term is dissociated vertical deviation.3 Authors have pointed out the inadequacy of the term dissociated vertical deviation because the name seems to exclude the horizontal and torsional components of the complex.4 Wilson and McClatchef advocate the term “dissociated strabismus complex.” However, in accordance with common usage, throughout this article we will use the term dissociated vertical deviation to encompass vertical as well as horizontal and torsional dissociative strabismus. Dissociated vertical deviation has been noted to accompany congenital esotropia and other forms of strabismus.3-11 Raab3 stated that dissociated vertical deviation “can coexist
From the Moran Eye Centw, University of Utah, Salt Lake City, Utah,& and the Department of Opbtbahology, Univwsiy ofha Horpitals and Clinti, Iowa City, IOWAN Supponed in pmt by an unrestricted grant from Research to Prevent Blindness, Inc., New %k (R.3.0.). Presented at the annual meeting of the American Association for Pediatric Ophthalmology and Swabimus, Cbnrleston, South Carolina, April 6, 1997. Submitted on April 6, 1997. Revision accepted on October 13, 1997. Reprints not available 90% the authors. Copyright 0 1998 by the American Awxiation for Pediatric Opbtbalmology and Stmbinnm 1091-8F31/98 $1.00 + 0 75/l/88077
72
April 1998
with other types of vertical anomaly,” and Helvestons noted that dissociated vertical deviation “may also occur with exodeviations and hyperdeviations,” although it is most commonly associated with congenital esotropia. Recently, Kushnertl described a case of dissociated vertical deviation with monocular elevation deficiency, Ku&k et al.6 described dissociated vertical deviation with sensory heterotropias, and Wilson et al.7 described dissociated vertical deviation with accommodative esotropia. Monocular elevation deficiency, more commonly known as double elevator palsy, was first described by White13 in 1942. The clinical characteristics of monocular elevation deficiency, including amblyopia, have been described in several series,l3-21 and the surgical treatment and results of treatment have been documented by Knapp22 and Burke et al.,23 among others. Several different classification schemes, based on clinical i?ndings and hypotheses of etiology, have been advanced. A supranuclear etiology was suggested by Jampel and Fells.24 Scott and Jackson15 pointed out the frequent presence of inferior rectus restriction, either as a primary cause of monocular elevation deficiency or as a result of it. More recently, Ziffer et al.20 have proposed at least three groups of patients with congenital monocular elevation deficiency: primary inferior recurs restriction, primary superior rectus paresis or palsy, and congenital supranuclear elevation deficiency. Having seen several congenital monocular elevation deficiency patients at the University of Iowa Hospitals and Clinics who clearly demonstrated dissociated vertical deviation in the affected eye, we endeavored to review all casesof monocular Journal of AAPOS
Journal of AAPOS Volume 2 Number 2 Apd
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1998
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TABLE 1. Patients with monocular elevation deficiency who developed dissociated vertical deviation Patient NO.
Sex
MED eye
Age at first visit (v)
Vertical deviation W
Horizontal deviation (PD)
Age at first MED surgery (vr)
1 F OS 0.8 18 20 ET 2 M OD 1.8 18 20 XT 3 F OD 0.9 25 14XT 4.0 35 IZXT 4 F OD 5 F OS 0.9 3; 20 ET 6 F OD 2.6 35 14 ET 9.2 20 16 ET 7 M OS 0.6 15 4XT 8 M OD 0.9 30 60 ET 9 F OD MED,Monocular elevation deficiency;6 female; OS,left eye; i? esotropia;/, inferior rectus recession; M,
Sex (Female)
1.0 2.9 1.0 4.7 3.8 2.7 9.7 1.1 4.9 male; 00,
MED surgery Me
Age DVD first noted in clinic (yr)
I I K K K K. I I K I
Age at last visit iv)
2.1 3.0 2.1 6.3 5.9 11.2 13.6 5.8 5.0
2.1 5.9 5.2 8.7 8.9 12.3 20.0 15.2 14.8
righteye;X7;exotropia; ,Y,Knappprocedure.
Nystagmus
FIG. 1. Relative prevalence of female gender, right eye lOOl as eye with monocular elevation deficiency, and presence of nystagmus in both subset of congenital monocular elevation deficiency that developed dissociated vertical deviation (DVD) (n= 9) and subset that did not (n = 22).
elevation deficiency diagnosed at the University of Iowa Hospitals and Clinics in the last quarter century to determine the frequency and characteristics of this association.
SUBJECTS AND METHODS The computerized database of all patients seen by the Pediatric Ophthalmology and Strabismus Service of the Department of Ophthalmology, University of Iowa Hospitals and Clinics, between 1971 and 1996 was searched to reveal all patients given a diagnosis of either monocular elevation deficiency or double elevator palsy. A total of 129 consecutive patients were identified, and charts were available for 126. Inclusion criteria included onset at younger than 1 year old, unilateral elevation deficit, and elevation deficit present in both abduction and adduction. Exclusion criteria included trauma, thyroid eye disease, myasthenia gravis, presence of
orbital lesions, Brown syndrome, Duane syndrome, superior oblique palsy, third cranial nerve palsy, and previous extraocular muscle or intraocular surgery. By these criteria 95 were excluded and 3 1 remained. All patients underwent complete ophthalmologic examination. Visual acuity, fixation, alignment at distance and near, versions, ductions, cycloplegic refraction, and anterior and posterior examinations were all performed. Each patient was evaluated for nystagmus, abnormal head position, and presence of ptosis or pseudoptosis. In accordance with patient ability, sensory testing at distance and near with the Worth four dot was also done. Where possible, the Bell’s phenomenon was tested. In most patients the presence or absence of an accentuated lower lid crease on the side of the monocular elevation deficiency was noted. Those patients who underwent surgery had forced duction testing performed in the operating room. Data for the initial visit, preoperative and postop-
74
Journal of AAPOS Volwne2 Number 2 April 1998
Olson and Scott
Initial Visit
1st MED Surgery
DVD 1st Documented
Last Visit
FIG. 2. Age (in years) for initial clinic visit, first surgery for monocular elevation deficiency (MD), first documentation of dissociated vertical deviation @VD), in ipsilateral eye, and final clinic visit for subset of congenital monocular elevation deficiency that developed dissociated vertical deviation (n = 9) and subset that did not (n = 22).
erative visits (where applicable), and the final visit were recorded. The first documentation and characterization of dissociated vertical deviation was noted. Statistical analysis was carried out with use of chisquare and paired t tests, as appropriate.
RESULTS Of the 3 1 patients with congenital monocular elevation deficiency, 19 were male and 12 were female. The mean age at the initial visit was 3.4 years (range 0.3 to 10.5 years), with a mean follow-up of 5.0 years (range 0 to 15.5 years). Twenty-one were right eyes and 10 were left eyes. Twenty-one of the 31 underwent surgery for monocular elevation deficiency, and 8 of the 21 underwent one to three additional monocular elevation deficiency surgeries. The mean age for the first monocular elevation deficiency surgery was 4.9 years (range 1.O to 16.1 years). Ocular alignment in primary position at the initial examination was as follows: a mean vertical deviation of 19.8 PD (0 to 40 PD of hypotropia) and a mean horizontal deviation (whether exo or eso) of 10.0 PD. Nine had exotropia with a maximum of 20 PD, 16 had esotropia with a maximum of 60 PD, and 6 had no horizontal deviation. Versions at the initial examination revealed a mean limitation to elevation in the affected eye of -3.3 in adduction and -3.2 in abduction on a scale of -1 (minimal) to -4 (no elevation past the midline). At the initial visit, 19 of 3 1 had amblyopia or had previously been treated successfully for it. Twenty-four of 31 had a chin-up head position. Five of 3 1 had nystagmus. Of the 16 who could perform Worth four dot testing, 3 had
central fusion and 7 had peripheral fusion. Six of 31 had anisometropia. Pseudoptosis was present in 27 and real ptosis in 20 (real ptosis was defined as that ptosis that remained after the hypotropic eye was forced to pick up fixation). Eight of 3 1 had Marcus-Gunn jaw wink. Nine of the 20 with ptosis eventually underwent ptosis repair. Nine of the 21 who underwent surgery for monocular elevation deficiency had positive forced ductions to elevation in the affected eye. At the initial visit 2 1 of 28 were felt to have absent or reduced Bell’s phenomenon in the affected eye. Fifteen of 28 were felt to have an accentuated lower lid crease on the affected side. Of the 2 1 who eventually underwent one or more surgeries for monocular elevation deficiency, 13 had Knapp procedures and 9 had inferior rectus recessions. The following data comparing the subset of monocular elevation deficiency patients who developed dissociated vertical deviation (hereafter referred to as the DVD group) with those who did not (hereafter referred to as the nonDVD group). These data should be seen only as indicating possible trends. Because of the small sample size, none of the statistical studies reached significance. The DVD group numbered 9 Of 3 1, all with dissociated vertical deviation in the same eye that had monocular elevation deficiency (Table 1). All 9 had previously undergone one or more surgeries for monocular elevation deficiency. Sixtyseven percent of the DVD group were female compared with 27% of the non-DID group. The right eye was the hypotropic eye most often in both groups: 67% versus 68%, respectively (Figure 1).
Journal of AAPOS Volume 2 Number 2 April 1998
Olsonand Scott 7J
45
40
35
c 30 3 E 5 E 25 .” h .= 6 20 P .I $ 15
10
5
0 Hypotropia
Esotropia
Exotropia
No Horizontal
Deviation
FIG.3. Mean deviation (in prism diopters) (with errorbarssignifying SDS) listed for hypotropia, esotropia, exotropia, or no horizontal deviation for groups subset of congenital monocular elevation deficiencythat developed dissociated vertical lOV’LJj deviation (n = 9) and subset that did not (n = 22). Relative prevalence of different horizontal deviations can be determined from numbers at base of columns. 1 q DVD
1 r-lI?3
0.9
q No DVD
/
0.8 0.7
0.68-
I
0.3
0.32
0.33
0.2 0.1 0 Ptosis
Pseudoptosis
Jaw Winking
Ptosis Surgery
FIG. 4. Relative presence of true ptosis, pseudoptosis (i.e., ptosis that disappears when hypotropic eye is forced to take up fixation), and Marcus-Gunn jaw winking as well as those who underwent ptosis repair surgery during study, for subset of congenital monocular elevation deficiencythat developed dissociated vertical (OWj deviation (n = 9) and subset that did not (n = 22).
The mean age at which dissociated vertical deviation was first noted was 6.1 years, an average of 3.7 years after the initial visit (range 1.2 to 8.6 years). Dissociated vertical deviation was first noted a mean of 2.5 years after the first surgery for monocular elevation deficiency (range 0.1 to 8.5 years).
The DVD group was slightly younger than the nonDVD group when they first came to attention, with a mean age at the initial visit of 2.4 years (range 7 months to 9.2 years) versus 3.8 years (range 4 months to 10.5 years). Of those who underwent surgery for monocular elevation deficiency, the DVD group was generally also younger,
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Journal of AAPOS Volume 2 Number 2 April 1998
Olson and Scott
Chin Up
Amblyopia
Anisometropia
Peripheral Fusion
Central Fusion
FIG. 5. Relative prevalence of chin-up head position, amblyopia, anisometropia, and peripheral and central fusion (both of which were tested on Worth four dot at near and distance) for subset of congenital monocular elevation deficiency that developed dissociated vertical deviation KIVD) (n = 9) and subset that did not (n = 22). Note that n for Worth four dot testing was low for both groups.
with a mean age at first surgery of 3.5 years (range 1.O to 9.7 years) versus 6.0 years (range 1.0 to 16.1 years). The DVD group was followed up longer: total time between initial and final visits was a mean of 7.8 years (range 1.3 to 13.8 years) versus 3 8 years (range 0 to 15.5 years) for the non-DVD group (Figure 2). During the time of the study, 4 of 9 in the DVD group underwent more than one surgery for monocular elevation deficiency versus 4 of 12 in the non-DVD group. The Knapp procedure was eventually performed on 56% (5/9) of the DVD group versus 67% (8/12) of the non-DVD group. Inferior rectns recession was performed on 56% (5/9) versus 3 3 % (4/12), respectively. The following data reflect the initial examination findings before any of the patients had undergone any extraocular or intraocular surgery or had developed dissociated vertical deviation. The DVD group had more pronounced vertical and horizontal deviations. The difference in vertical alignment was small: the mean hypotropia in the DVD group (n = 9) was 25.1 PD (range 15 to 35 PD) compared with 18.5 PD (range 0 to 40 PD) in the non-DVD group (n = 22). More striking was the horizontal deviation: all 9 of the DVD group had some degree of horizontal deviation compared with 16 of 22 in the non-DVD group. Five of 9 of those with dissociated vertical deviation were esotropic (mean 26.0 PD, range 14 to 60 PD) and 4 were exotropic (mean 12.5 PD, range 4 to 20 PD). In comparison, 11 of 22 of the non-DVD group were esotropic (mean only 9.6 PD, range 2 to 18 PD) and 5 of 22 were exotropic (mean only 5.2 PD, range 2 to 6 PD) (Figure 3).
Both subsets were generally the same in eyelid-related features of monocular elevation deficiency, except that Marcus-Gunn jaw winking was less common among the DVD group. Jaw winking was seen in 11% (l/9) of the DVD group versus 32% (7/22) in the non-DVD group. Pseudoptosis was seen in 100% of the DVD group and in 82% of the non-DVD group, whereas true ptosis was seen in 56% and 68%, respectively. Approximately equal percentages eventually underwent ptosis repair as well: 33 % of the group with and 27% of the group without dissociated vertical deviation (Figure 4). Either a history of treated amblyopia or amblyopia present at the initial examination was noted in 56% of the DVD group compared with 64% of the non-DVD group. Nystagmus (11% vs 18%, respectively), anisometropia (22% vs 18%, respectively), and presence of a chin-up head position (75% vs 86%, respectively) were all fairly evenly distributed. Sensory testing with the Worth four dot could only be performed on a small portion of the patients. Of note, however, none of the 4 in the DVD group who were tested demonstrated central fusion, whereas 3 of the 12 tested in the non-DVD group did demonstrate central fusion. Three of the 4 in the group with dissociated vertical deviation demonstrated peripheral fusion versus 4 of 12 in the group without (Figure 5). Forced duction testing was performed on all patients who underwent surgical correction of monocular elevation deficiency. Fifty-six percent (5/9) of the DVD group had positive forced ductions to elevation of the affected eye compared with 68% (4112) of the non-DVD group. Preoperatively, 57% of the DVD group compared with
Journal of AAPOS tilume 2 Number 2 April 1998
Olson and Scott
Forced Ductions
Abnormal
Bell’s
77
Lid Crease Sign
FIG. 6. Relative prevalence of positive forced ductions (restricted elevation of affected eye), absent or reduced Bell’s phenomenon on affected side, and accentuated lower lid crease on affected side for subset of congenital monocular elevation deficiency that developed dissociated vertical deviation (DVD) (n = 9) and subset that did not (n = 221
81% (17/22) of the non-DVD group were felt to have decreased or absent Bell’s phenomenon on the affected side. An accentuated lower lid crease was in 44% (4/9) of the DVD group versus 58% (1 l/19) of the non-DVD group (Figure 6).
DISCUSSION Dissociated vertical deviation occurs most frequently in association with congenital esotropia but has been reported in association with accommodative esotropia, exotropias, sensory heterophorias, orthophoria, the general category of hyperdeviations,l-lo> 12 and monocular elevation deficiency. 11 The current study is the largest yet reported regarding dissociated vertical deviation with monocular elevation deficiency or double elevator palsy. We prefer the term monocular elevation deficiency to the more common term double elevator palsy because the term double elevator palsy is misleading. Both elevators do not have to be involved to have a “double elevator palsy”@ 17~20~21 and neither elevator need be paretic if inferior rectus restriction alone exists.t5 We agree with Wilson that the term dissociated vertical deviation is oversimplified 2~7 because the phenomenon also encompasses horizontal and torsional components. Wilson has proposed the term “dissociated strabismus complex. “22427 In this article dissociated vertical deviation refers to horizontal, torsional, and vertical components. Scott and Jackson15 proposed a classification of monocular elevation deficiency in 1977 as follows: (1) supranuclear palsy of the elevators, as in the supranuclear theory of Jampel and Fells, 24 (2) isolated inferior rectus
restriction, and (3) combination palsy and inferior rectus restriction. More recently, Ziffer et al.20 proposed at least three distinct subgroups of clinical double elevator palsy: primary inferior rectus restriction, primary superior rectus paresis or palsy, and congenital supranuclear elevation deficiency. We did not perform vertical saccadic velocity testing and thus cannot make the distinction between muscle paresis and supranuclear elevation deficiency in this study. We feel that an additional category combining elevation deficiency (whether muscle palsy or supranuclear lesion) with inferior rectus restriction is clinically useful. Several of our patients required inferior rectus recessions for restriction, followed later by Knapp procedures after the elevation weakness persisted. Our findings for congenital monocular elevation deliciency taken as a whole are not dissimilar to those of previous series.l5-17 This is the largest series of patients with congenital monocular elevation deficiency reported to date. We found pseudoptosis in 90%, true ptosis in 64%, chin-up head position in 77%) amblyopia in 61%) hypotropia in primary gaze in 97% with a mean of 20 PD, and significantly reduced upgaze in both adduction and abduction in the affected eye. We also found MarcusGunn jaw winking in 28%, a horizontal deviation greater than 8 PD in 42 %, and anisometropia in 19%. Bell’s phenomenon was reduced or absent in 7.5%, the lower lid crease was accentuated in 56%, and forced ductions were positive for restriction to elevation in the affected eye in 42 % of those tested. Taking intraoperative forced ductions as the gold standard for restriction to elevation, neither abnormal Bell’s phenomenon or an accentuated lid
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Journal of AAPOS Volume 2 Number 2 April 1998
Olson and Scott
crease predicted restriction very well, with a sensitivity and specificity of 100% and 42% for abnormal Bell’s phenomenon and 67% and 58% for an accentuated lid crease. Nearly one third (29%) of our patients with congenital monocular elevation deficiency developed dissociated vertical deviation in the ipsilateral eye over a mean follow-up of 5.0 years (up to 15.5 years). What set them apart from the 7 1% who did not develop dissociated vertical deviation? The small sample size precluded statistical significance, but the n-ends suggest that those patients with congenital monocular elevation deficiency who developed dissociated vertical deviation had (1) somewhat more severe hypotropia with a (2) larger associated horizontal deviation and (3) imperfect fusion and (4) came to surgical attention somewhat earlier than those who did not develop dissociated vertical deviation. In our study the group with dissociated vertical deviation was also followed up longer, suggesting that with longer follow-up we may see a higher prevalence of dissociated vertical deviation in monocular elevation deficiency than we report. This is consistent with Helveston’ss observation that dissociated vertical deviation “occurs in people who have some underlying fusion anomaly” and is more likely to occur “the denser the fusion anomaly and the earlier the onset.” We thank Rita Hanover for technical assistance.
10. 11. 12. 13. 14. 15. 16. 17. 18. 19.
References
20.
1. Stevens GT On double vertical strabismus. Annales d’ocularistique (Paris) 1895;113:225-32. 2. Wilson ME. Dissociated vertical deviation. In: Margo CE, editor. Diagnostic problems in clinical ophthalmology Philadelphia: WB Saunders; 1994. p. 769-72. 3. Raab EL. Dissociative vertical deviation. J Pediatr Ophthalmol Strabismus 1970;7:146-51. 4. Wilson ME, McClatchey SK. Dissociated vertical deviation. J Pediatr Ophthalmol Strabismus 1991;28:90-5. 5. Helveston EM. Dissociated vertical deviation: a clinical and laboratory study. Trans Am Ophthalmol Sot 1980;78:734-79. 6. Kutluk S, Avilla CW, von Noorden GK. The prevalence of dissociated vertical deviation in patients with sensory heterotropia. Am J
21.
22. 23.
24.
Ophthalmol 1995;119:744-7. Wilson ME, Saunders RA, Berland JE. Dissociated horizontal deviation and accommodative esotropia: treatment options when an esoand an exodeviation co-exist. J Pediatr Ophthalmol Strabismus 1995;32:228:30. Enke ES, Stewart SA, Scott WE, Wheeler DT The prevalence of dissociated horizontal deviations in congenital esotropia. Am Or&optic J 1994;44:109-11. Parks MM, Mitchell PR. Dissociated vertical deviations. In: Tasman W, Jaeger EA, editors. Duane’s clinical ophthalmology. Philadelphia: JB Lippincott; 1993. p. l-6. Biglan AW, Davis JS, Cheng KP, Pettapiece MC. Infantile exotropia. J Pediatr Ophthalmol Strabismus 1996;33:79-84. Kushner BJ. A case of dissociated hypertropia after surgery for a double elevator palsy. Binocular Vision Q 1990;5:88-92. Stewart SA, Scott WE. The age of onset of dissociated vertical deviation (DVD). Am J O&opt 1991;41:85-9. White JW. Paralysis of the superior rectus and the inferior oblique muscle of the same eye. Arch Ophthalmol 1942;27:366-71. Rosner RS. Double elevator paralysis. Am J Ophthalmol 1963; 55:87-93. Scott WE, Jackson OB. Double elevator palsy: the significance of inferior rectus restriction. Am Or&optic J 1977;27:5-10. Metz HS. Double elevator palsy. Arch Ophthalmol 1979;97:901-3. Metz HS. Double elevator palsy. J Pediatr Ophthalmol Strabismus 1981;18:31-5. Barsoum-Homsy M. Congenital double elevator palsy. J Pediatr Ophthalmol Strabismus 1983;20:185-91. Bell JA, Fielder AR, Viney S. Congenital double elevator palsy in identical twins. J Clin Neuro-ophthahnol 1990;10:32-4. Ziffer AJ, Rosenbaum AL, Demer JL, Yee RD. Congenital double elevator palsy: vertical saccadic velocity utilizing the scleral search coil technique. J Pediatr Ophthalmol Strabismus 1992;29:142-9. Ruben JB. Congenital double elevator palsy: vertical saccadic velocity utilizing the scleral search coil technique [letter]. J Pediatr Ophthalmol Strabismus 1993;30:399. Knapp I? The surgical treatment of double-elevator paralysis. Tram Am Ophthahnol Sot 1969;67:304-23. Burke JP, Ruben MB, Scott WE. Vertical transposition of the horizontal recti (Knapp procedure) for the treatment of double elevator palsy: effectiveness and long-term stability. Br J Ophthalmol 1992;76:734-7. Jampel RS, Fells P Monocular elevation paresis caused by a central nervous system lesion. Arch Ophthalmol 1968;80:45-7.
CORRECTION The Effect of Anterior Transposition of the Inferior Oblique Muscle on Ocular Torsion (Santiago AR, Isenberg SJ, Apt L, Roh YB. 1997;1:191-6) On page 194, in Figure 2, the middle and bottom figures in each column are transposed. Therefore the right middle should be the left middle and the right bottom should be the left bottom.
1. Olson!
MD,a
and William
E. Scott,
MDb
Introduction: Monocular elevation deficiency is characterized by unilateral limitation of elevation in both adduction and abduction and is usually present at birth. Dissociative phenomena such as dissociated vertical deviation are well recognized in association with conditions such as congenital esotropia but much less so in association with conditions such as congenital monocular elevation deficiency. Mefhods:All 129 patients given the diagnosis of monocular elevation deficiency or double elevator palsy in the Pediatric Ophthalmology and Strabismus Clinic at the University of Iowa Hospitals and Clinics between 1971 and 1995 were reviewed. After those with history of trauma, myasthenia gravis, thyroid eye disease, orbital lesions, Brown syndrome, or monocular elevation deficiency with acquired onset were excluded, 31 patients with congenital monocular elevation deficiency remained for retrospective study. Resulfs: First diagnosed at median age 2.6 years (although all were noted by parents at less than 6 months of age) with mean follow-up of 5.0 years (up to 15.5 years), 9 of 31 (29%) developed dissociated vertical deviation in the eye with monocular elevation deficiency, all of whom had undergone strabismus surgery 0 to 9.7 years previously (mean 3.5 years). Those who developed dissociated vertical deviation were generally younger, were followed up longer, and had more accompanying horizontal strabismus than did those who did not develop dissociated vertical deviation. The results did not reach significance. Conc/usion:The current study demonstrates that dissociated vertical deviation occurs in association with monocular elevation deficiency. (J AAPOS 1998;2:72-8)
D
issociative phenomena in strabismus have been described for more than a century1 and under many different names, encompassing vertical, horizontal, torsional strabismus noted during inattention or under cover, which is dissociated (i.e., does not follow Hering’s law).2 The currently accepted clinical term is dissociated vertical deviation.3 Authors have pointed out the inadequacy of the term dissociated vertical deviation because the name seems to exclude the horizontal and torsional components of the complex.4 Wilson and McClatchef advocate the term “dissociated strabismus complex.” However, in accordance with common usage, throughout this article we will use the term dissociated vertical deviation to encompass vertical as well as horizontal and torsional dissociative strabismus. Dissociated vertical deviation has been noted to accompany congenital esotropia and other forms of strabismus.3-11 Raab3 stated that dissociated vertical deviation “can coexist
From the Moran Eye Centw, University of Utah, Salt Lake City, Utah,& and the Department of Opbtbahology, Univwsiy ofha Horpitals and Clinti, Iowa City, IOWAN Supponed in pmt by an unrestricted grant from Research to Prevent Blindness, Inc., New %k (R.3.0.). Presented at the annual meeting of the American Association for Pediatric Ophthalmology and Swabimus, Cbnrleston, South Carolina, April 6, 1997. Submitted on April 6, 1997. Revision accepted on October 13, 1997. Reprints not available 90% the authors. Copyright 0 1998 by the American Awxiation for Pediatric Opbtbalmology and Stmbinnm 1091-8F31/98 $1.00 + 0 75/l/88077
72
April 1998
with other types of vertical anomaly,” and Helvestons noted that dissociated vertical deviation “may also occur with exodeviations and hyperdeviations,” although it is most commonly associated with congenital esotropia. Recently, Kushnertl described a case of dissociated vertical deviation with monocular elevation deficiency, Ku&k et al.6 described dissociated vertical deviation with sensory heterotropias, and Wilson et al.7 described dissociated vertical deviation with accommodative esotropia. Monocular elevation deficiency, more commonly known as double elevator palsy, was first described by White13 in 1942. The clinical characteristics of monocular elevation deficiency, including amblyopia, have been described in several series,l3-21 and the surgical treatment and results of treatment have been documented by Knapp22 and Burke et al.,23 among others. Several different classification schemes, based on clinical i?ndings and hypotheses of etiology, have been advanced. A supranuclear etiology was suggested by Jampel and Fells.24 Scott and Jackson15 pointed out the frequent presence of inferior rectus restriction, either as a primary cause of monocular elevation deficiency or as a result of it. More recently, Ziffer et al.20 have proposed at least three groups of patients with congenital monocular elevation deficiency: primary inferior recurs restriction, primary superior rectus paresis or palsy, and congenital supranuclear elevation deficiency. Having seen several congenital monocular elevation deficiency patients at the University of Iowa Hospitals and Clinics who clearly demonstrated dissociated vertical deviation in the affected eye, we endeavored to review all casesof monocular Journal of AAPOS
Journal of AAPOS Volume 2 Number 2 Apd
Olson and Scott
1998
73
TABLE 1. Patients with monocular elevation deficiency who developed dissociated vertical deviation Patient NO.
Sex
MED eye
Age at first visit (v)
Vertical deviation W
Horizontal deviation (PD)
Age at first MED surgery (vr)
1 F OS 0.8 18 20 ET 2 M OD 1.8 18 20 XT 3 F OD 0.9 25 14XT 4.0 35 IZXT 4 F OD 5 F OS 0.9 3; 20 ET 6 F OD 2.6 35 14 ET 9.2 20 16 ET 7 M OS 0.6 15 4XT 8 M OD 0.9 30 60 ET 9 F OD MED,Monocular elevation deficiency;6 female; OS,left eye; i? esotropia;/, inferior rectus recession; M,
Sex (Female)
1.0 2.9 1.0 4.7 3.8 2.7 9.7 1.1 4.9 male; 00,
MED surgery Me
Age DVD first noted in clinic (yr)
I I K K K K. I I K I
Age at last visit iv)
2.1 3.0 2.1 6.3 5.9 11.2 13.6 5.8 5.0
2.1 5.9 5.2 8.7 8.9 12.3 20.0 15.2 14.8
righteye;X7;exotropia; ,Y,Knappprocedure.
Nystagmus
FIG. 1. Relative prevalence of female gender, right eye lOOl as eye with monocular elevation deficiency, and presence of nystagmus in both subset of congenital monocular elevation deficiency that developed dissociated vertical deviation (DVD) (n= 9) and subset that did not (n = 22).
elevation deficiency diagnosed at the University of Iowa Hospitals and Clinics in the last quarter century to determine the frequency and characteristics of this association.
SUBJECTS AND METHODS The computerized database of all patients seen by the Pediatric Ophthalmology and Strabismus Service of the Department of Ophthalmology, University of Iowa Hospitals and Clinics, between 1971 and 1996 was searched to reveal all patients given a diagnosis of either monocular elevation deficiency or double elevator palsy. A total of 129 consecutive patients were identified, and charts were available for 126. Inclusion criteria included onset at younger than 1 year old, unilateral elevation deficit, and elevation deficit present in both abduction and adduction. Exclusion criteria included trauma, thyroid eye disease, myasthenia gravis, presence of
orbital lesions, Brown syndrome, Duane syndrome, superior oblique palsy, third cranial nerve palsy, and previous extraocular muscle or intraocular surgery. By these criteria 95 were excluded and 3 1 remained. All patients underwent complete ophthalmologic examination. Visual acuity, fixation, alignment at distance and near, versions, ductions, cycloplegic refraction, and anterior and posterior examinations were all performed. Each patient was evaluated for nystagmus, abnormal head position, and presence of ptosis or pseudoptosis. In accordance with patient ability, sensory testing at distance and near with the Worth four dot was also done. Where possible, the Bell’s phenomenon was tested. In most patients the presence or absence of an accentuated lower lid crease on the side of the monocular elevation deficiency was noted. Those patients who underwent surgery had forced duction testing performed in the operating room. Data for the initial visit, preoperative and postop-
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Journal of AAPOS Volwne2 Number 2 April 1998
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Initial Visit
1st MED Surgery
DVD 1st Documented
Last Visit
FIG. 2. Age (in years) for initial clinic visit, first surgery for monocular elevation deficiency (MD), first documentation of dissociated vertical deviation @VD), in ipsilateral eye, and final clinic visit for subset of congenital monocular elevation deficiency that developed dissociated vertical deviation (n = 9) and subset that did not (n = 22).
erative visits (where applicable), and the final visit were recorded. The first documentation and characterization of dissociated vertical deviation was noted. Statistical analysis was carried out with use of chisquare and paired t tests, as appropriate.
RESULTS Of the 3 1 patients with congenital monocular elevation deficiency, 19 were male and 12 were female. The mean age at the initial visit was 3.4 years (range 0.3 to 10.5 years), with a mean follow-up of 5.0 years (range 0 to 15.5 years). Twenty-one were right eyes and 10 were left eyes. Twenty-one of the 31 underwent surgery for monocular elevation deficiency, and 8 of the 21 underwent one to three additional monocular elevation deficiency surgeries. The mean age for the first monocular elevation deficiency surgery was 4.9 years (range 1.O to 16.1 years). Ocular alignment in primary position at the initial examination was as follows: a mean vertical deviation of 19.8 PD (0 to 40 PD of hypotropia) and a mean horizontal deviation (whether exo or eso) of 10.0 PD. Nine had exotropia with a maximum of 20 PD, 16 had esotropia with a maximum of 60 PD, and 6 had no horizontal deviation. Versions at the initial examination revealed a mean limitation to elevation in the affected eye of -3.3 in adduction and -3.2 in abduction on a scale of -1 (minimal) to -4 (no elevation past the midline). At the initial visit, 19 of 3 1 had amblyopia or had previously been treated successfully for it. Twenty-four of 31 had a chin-up head position. Five of 3 1 had nystagmus. Of the 16 who could perform Worth four dot testing, 3 had
central fusion and 7 had peripheral fusion. Six of 31 had anisometropia. Pseudoptosis was present in 27 and real ptosis in 20 (real ptosis was defined as that ptosis that remained after the hypotropic eye was forced to pick up fixation). Eight of 3 1 had Marcus-Gunn jaw wink. Nine of the 20 with ptosis eventually underwent ptosis repair. Nine of the 21 who underwent surgery for monocular elevation deficiency had positive forced ductions to elevation in the affected eye. At the initial visit 2 1 of 28 were felt to have absent or reduced Bell’s phenomenon in the affected eye. Fifteen of 28 were felt to have an accentuated lower lid crease on the affected side. Of the 2 1 who eventually underwent one or more surgeries for monocular elevation deficiency, 13 had Knapp procedures and 9 had inferior rectus recessions. The following data comparing the subset of monocular elevation deficiency patients who developed dissociated vertical deviation (hereafter referred to as the DVD group) with those who did not (hereafter referred to as the nonDVD group). These data should be seen only as indicating possible trends. Because of the small sample size, none of the statistical studies reached significance. The DVD group numbered 9 Of 3 1, all with dissociated vertical deviation in the same eye that had monocular elevation deficiency (Table 1). All 9 had previously undergone one or more surgeries for monocular elevation deficiency. Sixtyseven percent of the DVD group were female compared with 27% of the non-DID group. The right eye was the hypotropic eye most often in both groups: 67% versus 68%, respectively (Figure 1).
Journal of AAPOS Volume 2 Number 2 April 1998
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45
40
35
c 30 3 E 5 E 25 .” h .= 6 20 P .I $ 15
10
5
0 Hypotropia
Esotropia
Exotropia
No Horizontal
Deviation
FIG.3. Mean deviation (in prism diopters) (with errorbarssignifying SDS) listed for hypotropia, esotropia, exotropia, or no horizontal deviation for groups subset of congenital monocular elevation deficiencythat developed dissociated vertical lOV’LJj deviation (n = 9) and subset that did not (n = 22). Relative prevalence of different horizontal deviations can be determined from numbers at base of columns. 1 q DVD
1 r-lI?3
0.9
q No DVD
/
0.8 0.7
0.68-
I
0.3
0.32
0.33
0.2 0.1 0 Ptosis
Pseudoptosis
Jaw Winking
Ptosis Surgery
FIG. 4. Relative presence of true ptosis, pseudoptosis (i.e., ptosis that disappears when hypotropic eye is forced to take up fixation), and Marcus-Gunn jaw winking as well as those who underwent ptosis repair surgery during study, for subset of congenital monocular elevation deficiencythat developed dissociated vertical (OWj deviation (n = 9) and subset that did not (n = 22).
The mean age at which dissociated vertical deviation was first noted was 6.1 years, an average of 3.7 years after the initial visit (range 1.2 to 8.6 years). Dissociated vertical deviation was first noted a mean of 2.5 years after the first surgery for monocular elevation deficiency (range 0.1 to 8.5 years).
The DVD group was slightly younger than the nonDVD group when they first came to attention, with a mean age at the initial visit of 2.4 years (range 7 months to 9.2 years) versus 3.8 years (range 4 months to 10.5 years). Of those who underwent surgery for monocular elevation deficiency, the DVD group was generally also younger,
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Journal of AAPOS Volume 2 Number 2 April 1998
Olson and Scott
Chin Up
Amblyopia
Anisometropia
Peripheral Fusion
Central Fusion
FIG. 5. Relative prevalence of chin-up head position, amblyopia, anisometropia, and peripheral and central fusion (both of which were tested on Worth four dot at near and distance) for subset of congenital monocular elevation deficiency that developed dissociated vertical deviation KIVD) (n = 9) and subset that did not (n = 22). Note that n for Worth four dot testing was low for both groups.
with a mean age at first surgery of 3.5 years (range 1.O to 9.7 years) versus 6.0 years (range 1.0 to 16.1 years). The DVD group was followed up longer: total time between initial and final visits was a mean of 7.8 years (range 1.3 to 13.8 years) versus 3 8 years (range 0 to 15.5 years) for the non-DVD group (Figure 2). During the time of the study, 4 of 9 in the DVD group underwent more than one surgery for monocular elevation deficiency versus 4 of 12 in the non-DVD group. The Knapp procedure was eventually performed on 56% (5/9) of the DVD group versus 67% (8/12) of the non-DVD group. Inferior rectns recession was performed on 56% (5/9) versus 3 3 % (4/12), respectively. The following data reflect the initial examination findings before any of the patients had undergone any extraocular or intraocular surgery or had developed dissociated vertical deviation. The DVD group had more pronounced vertical and horizontal deviations. The difference in vertical alignment was small: the mean hypotropia in the DVD group (n = 9) was 25.1 PD (range 15 to 35 PD) compared with 18.5 PD (range 0 to 40 PD) in the non-DVD group (n = 22). More striking was the horizontal deviation: all 9 of the DVD group had some degree of horizontal deviation compared with 16 of 22 in the non-DVD group. Five of 9 of those with dissociated vertical deviation were esotropic (mean 26.0 PD, range 14 to 60 PD) and 4 were exotropic (mean 12.5 PD, range 4 to 20 PD). In comparison, 11 of 22 of the non-DVD group were esotropic (mean only 9.6 PD, range 2 to 18 PD) and 5 of 22 were exotropic (mean only 5.2 PD, range 2 to 6 PD) (Figure 3).
Both subsets were generally the same in eyelid-related features of monocular elevation deficiency, except that Marcus-Gunn jaw winking was less common among the DVD group. Jaw winking was seen in 11% (l/9) of the DVD group versus 32% (7/22) in the non-DVD group. Pseudoptosis was seen in 100% of the DVD group and in 82% of the non-DVD group, whereas true ptosis was seen in 56% and 68%, respectively. Approximately equal percentages eventually underwent ptosis repair as well: 33 % of the group with and 27% of the group without dissociated vertical deviation (Figure 4). Either a history of treated amblyopia or amblyopia present at the initial examination was noted in 56% of the DVD group compared with 64% of the non-DVD group. Nystagmus (11% vs 18%, respectively), anisometropia (22% vs 18%, respectively), and presence of a chin-up head position (75% vs 86%, respectively) were all fairly evenly distributed. Sensory testing with the Worth four dot could only be performed on a small portion of the patients. Of note, however, none of the 4 in the DVD group who were tested demonstrated central fusion, whereas 3 of the 12 tested in the non-DVD group did demonstrate central fusion. Three of the 4 in the group with dissociated vertical deviation demonstrated peripheral fusion versus 4 of 12 in the group without (Figure 5). Forced duction testing was performed on all patients who underwent surgical correction of monocular elevation deficiency. Fifty-six percent (5/9) of the DVD group had positive forced ductions to elevation of the affected eye compared with 68% (4112) of the non-DVD group. Preoperatively, 57% of the DVD group compared with
Journal of AAPOS tilume 2 Number 2 April 1998
Olson and Scott
Forced Ductions
Abnormal
Bell’s
77
Lid Crease Sign
FIG. 6. Relative prevalence of positive forced ductions (restricted elevation of affected eye), absent or reduced Bell’s phenomenon on affected side, and accentuated lower lid crease on affected side for subset of congenital monocular elevation deficiency that developed dissociated vertical deviation (DVD) (n = 9) and subset that did not (n = 221
81% (17/22) of the non-DVD group were felt to have decreased or absent Bell’s phenomenon on the affected side. An accentuated lower lid crease was in 44% (4/9) of the DVD group versus 58% (1 l/19) of the non-DVD group (Figure 6).
DISCUSSION Dissociated vertical deviation occurs most frequently in association with congenital esotropia but has been reported in association with accommodative esotropia, exotropias, sensory heterophorias, orthophoria, the general category of hyperdeviations,l-lo> 12 and monocular elevation deficiency. 11 The current study is the largest yet reported regarding dissociated vertical deviation with monocular elevation deficiency or double elevator palsy. We prefer the term monocular elevation deficiency to the more common term double elevator palsy because the term double elevator palsy is misleading. Both elevators do not have to be involved to have a “double elevator palsy”@ 17~20~21 and neither elevator need be paretic if inferior rectus restriction alone exists.t5 We agree with Wilson that the term dissociated vertical deviation is oversimplified 2~7 because the phenomenon also encompasses horizontal and torsional components. Wilson has proposed the term “dissociated strabismus complex. “22427 In this article dissociated vertical deviation refers to horizontal, torsional, and vertical components. Scott and Jackson15 proposed a classification of monocular elevation deficiency in 1977 as follows: (1) supranuclear palsy of the elevators, as in the supranuclear theory of Jampel and Fells, 24 (2) isolated inferior rectus
restriction, and (3) combination palsy and inferior rectus restriction. More recently, Ziffer et al.20 proposed at least three distinct subgroups of clinical double elevator palsy: primary inferior rectus restriction, primary superior rectus paresis or palsy, and congenital supranuclear elevation deficiency. We did not perform vertical saccadic velocity testing and thus cannot make the distinction between muscle paresis and supranuclear elevation deficiency in this study. We feel that an additional category combining elevation deficiency (whether muscle palsy or supranuclear lesion) with inferior rectus restriction is clinically useful. Several of our patients required inferior rectus recessions for restriction, followed later by Knapp procedures after the elevation weakness persisted. Our findings for congenital monocular elevation deliciency taken as a whole are not dissimilar to those of previous series.l5-17 This is the largest series of patients with congenital monocular elevation deficiency reported to date. We found pseudoptosis in 90%, true ptosis in 64%, chin-up head position in 77%) amblyopia in 61%) hypotropia in primary gaze in 97% with a mean of 20 PD, and significantly reduced upgaze in both adduction and abduction in the affected eye. We also found MarcusGunn jaw winking in 28%, a horizontal deviation greater than 8 PD in 42 %, and anisometropia in 19%. Bell’s phenomenon was reduced or absent in 7.5%, the lower lid crease was accentuated in 56%, and forced ductions were positive for restriction to elevation in the affected eye in 42 % of those tested. Taking intraoperative forced ductions as the gold standard for restriction to elevation, neither abnormal Bell’s phenomenon or an accentuated lid
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Journal of AAPOS Volume 2 Number 2 April 1998
Olson and Scott
crease predicted restriction very well, with a sensitivity and specificity of 100% and 42% for abnormal Bell’s phenomenon and 67% and 58% for an accentuated lid crease. Nearly one third (29%) of our patients with congenital monocular elevation deficiency developed dissociated vertical deviation in the ipsilateral eye over a mean follow-up of 5.0 years (up to 15.5 years). What set them apart from the 7 1% who did not develop dissociated vertical deviation? The small sample size precluded statistical significance, but the n-ends suggest that those patients with congenital monocular elevation deficiency who developed dissociated vertical deviation had (1) somewhat more severe hypotropia with a (2) larger associated horizontal deviation and (3) imperfect fusion and (4) came to surgical attention somewhat earlier than those who did not develop dissociated vertical deviation. In our study the group with dissociated vertical deviation was also followed up longer, suggesting that with longer follow-up we may see a higher prevalence of dissociated vertical deviation in monocular elevation deficiency than we report. This is consistent with Helveston’ss observation that dissociated vertical deviation “occurs in people who have some underlying fusion anomaly” and is more likely to occur “the denser the fusion anomaly and the earlier the onset.” We thank Rita Hanover for technical assistance.
10. 11. 12. 13. 14. 15. 16. 17. 18. 19.
References
20.
1. Stevens GT On double vertical strabismus. Annales d’ocularistique (Paris) 1895;113:225-32. 2. Wilson ME. Dissociated vertical deviation. In: Margo CE, editor. Diagnostic problems in clinical ophthalmology Philadelphia: WB Saunders; 1994. p. 769-72. 3. Raab EL. Dissociative vertical deviation. J Pediatr Ophthalmol Strabismus 1970;7:146-51. 4. Wilson ME, McClatchey SK. Dissociated vertical deviation. J Pediatr Ophthalmol Strabismus 1991;28:90-5. 5. Helveston EM. Dissociated vertical deviation: a clinical and laboratory study. Trans Am Ophthalmol Sot 1980;78:734-79. 6. Kutluk S, Avilla CW, von Noorden GK. The prevalence of dissociated vertical deviation in patients with sensory heterotropia. Am J
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Ophthalmol 1995;119:744-7. Wilson ME, Saunders RA, Berland JE. Dissociated horizontal deviation and accommodative esotropia: treatment options when an esoand an exodeviation co-exist. J Pediatr Ophthalmol Strabismus 1995;32:228:30. Enke ES, Stewart SA, Scott WE, Wheeler DT The prevalence of dissociated horizontal deviations in congenital esotropia. Am Or&optic J 1994;44:109-11. Parks MM, Mitchell PR. Dissociated vertical deviations. In: Tasman W, Jaeger EA, editors. Duane’s clinical ophthalmology. Philadelphia: JB Lippincott; 1993. p. l-6. Biglan AW, Davis JS, Cheng KP, Pettapiece MC. Infantile exotropia. J Pediatr Ophthalmol Strabismus 1996;33:79-84. Kushner BJ. A case of dissociated hypertropia after surgery for a double elevator palsy. Binocular Vision Q 1990;5:88-92. Stewart SA, Scott WE. The age of onset of dissociated vertical deviation (DVD). Am J O&opt 1991;41:85-9. White JW. Paralysis of the superior rectus and the inferior oblique muscle of the same eye. Arch Ophthalmol 1942;27:366-71. Rosner RS. Double elevator paralysis. Am J Ophthalmol 1963; 55:87-93. Scott WE, Jackson OB. Double elevator palsy: the significance of inferior rectus restriction. Am Or&optic J 1977;27:5-10. Metz HS. Double elevator palsy. Arch Ophthalmol 1979;97:901-3. Metz HS. Double elevator palsy. J Pediatr Ophthalmol Strabismus 1981;18:31-5. Barsoum-Homsy M. Congenital double elevator palsy. J Pediatr Ophthalmol Strabismus 1983;20:185-91. Bell JA, Fielder AR, Viney S. Congenital double elevator palsy in identical twins. J Clin Neuro-ophthahnol 1990;10:32-4. Ziffer AJ, Rosenbaum AL, Demer JL, Yee RD. Congenital double elevator palsy: vertical saccadic velocity utilizing the scleral search coil technique. J Pediatr Ophthalmol Strabismus 1992;29:142-9. Ruben JB. Congenital double elevator palsy: vertical saccadic velocity utilizing the scleral search coil technique [letter]. J Pediatr Ophthalmol Strabismus 1993;30:399. Knapp I? The surgical treatment of double-elevator paralysis. Tram Am Ophthahnol Sot 1969;67:304-23. Burke JP, Ruben MB, Scott WE. Vertical transposition of the horizontal recti (Knapp procedure) for the treatment of double elevator palsy: effectiveness and long-term stability. Br J Ophthalmol 1992;76:734-7. Jampel RS, Fells P Monocular elevation paresis caused by a central nervous system lesion. Arch Ophthalmol 1968;80:45-7.
CORRECTION The Effect of Anterior Transposition of the Inferior Oblique Muscle on Ocular Torsion (Santiago AR, Isenberg SJ, Apt L, Roh YB. 1997;1:191-6) On page 194, in Figure 2, the middle and bottom figures in each column are transposed. Therefore the right middle should be the left middle and the right bottom should be the left bottom.