515
SHORT SCIENTIFIC ARTICLE
Surgical treatment of a patient with congenital nystagmus with compensatory head posture: A case report
Department of 
Ophthalmology, University 
Medical Centre Ljubljana, 
Ljubljana, Slovenia
Correspondence/
Korespondenca:
Alma Kurent, e: alma.
kurent@gmail.com
Key words:
nystagmus; null zone; 
compensatory head 
posture; treatment; 
Kestenbaum procedure
Ključne besede:
nistagmus; ničta točka; 
kompenzatorna drža 
glave; zdravljenje; 
Kestenbaumova operacija
Received: 21. 1. 2019
Accepted: 2. 6. 2020
eng slo element
en article-lang
10.6016/ZdravVestn.2918 doi
21.1.2019 date-received
2.6.2020 date-accepted
Neurobiology Nevrobiologija discipline
Short scientific article Klinični primer article-type
Surgical treatment of a patient with congenital 
nystagmus with compensatory head posture - 
a case report
Kirurško zdravljenje bolnice s prirojenim nistag-
musom s kompenzacijsko držo glave – prikaz 
primera
article-title
Surgical treatment of a patient with congenital 
nystagmus with compensatory head posture - 
a case report
Kirurško zdravljenje bolnice s prirojenim nistag-
musom s kompenzacijsko držo glave – prikaz 
primera
alt-title
nystagmus, null zone, compensatory head 
posture, treatment, Kestenbaum procedure
nistagmus, ničta točka, kompenzatorna drža 
glave, zdravljenje, Kestenbaumova operacija kwd-group
The authors declare that there are no conflicts 
of interest present.
Avtorji so izjavili, da ne obstajajo nobeni 
konkurenčni interesi. conflict
year volume first month last month first page last page
2020 89 9 10 515 520
name surname aff email
Alma Kurent 1 alma.kurent@gmail.com
name surname aff
Dragica Kosec 1
eng slo aff-id
Department of Ophthalmology, 
University Medical Centre 
Ljubljana, Ljubljana, Slovenia
Očesna klinika, Univerzitetni 
klinični center Ljubljana, 
Ljubljana, Slovenija
1
Surgical treatment of a patient with 
congenital nystagmus with compensatory 
head posture: A case report
Kirurško zdravljenje bolnice s prirojenim nistagmusom 
s kompenzatorno držo glave – prikaz primera
Alma Kurent, Dragica Kosec
Abstract
The correction of compensatory head posture in a congenital nystagmus involves surgical treat-
ment that includes recession and resection of extraocular muscles to move the eccentric null 
zone to a primary position.
A 39-year-old patient presented with an impaired visual acuity and nystagmus that was present 
since childhood. She had a left head turn with permanent neck pain. At the examination, best 
corrected visual acuity was 0.4 in both eyes with her glasses. Correction in the right eye was 
-6.50-1.50/180° and in the left eye -5.50-2.50/180°. Measured objective and subjective angles of 
squint were +4°, fusion from -3° to +29°, with the presence of stereo vision. During the cover test 
the nystagmus was present and it enhanced while covering the eye. Ocular motility was not lim-
ited. Fundus examination revealed myopic changes in both eyes. Prisms were prescribed, which 
were well tolerated by the patient. Also, no apparent head turn was noticed while wearing the 
prisms.
Nine months later, the patient underwent a Kestenbaum procedure. Retroposition of the lateral 
rectus muscle with resection of the medial rectus muscle in the right eye and retroposition of the 
medial rectus muscle with resection of the lateral rectus muscle in the left eye were performed. 
After the procedure nystagmus dampened the most in the minimal left position, the head was in 
a straight position. Two years after the procedure nystagmus dampened the most in the primary 
position, the head was in a straight position. Nine years after surgery and refractive correction 
with contact lenses, the visual acuity was 0.8-0.9p in both eyes.
The presented case showed that adequate functional and surgical treatment led to a good mor-
phological outcome with improved visual acuity in a patient with congenital nystagmus and a 
compensatory head posture even in adulthood.
Izvleček
Korekcija kompenzatorne drže glave pri prirojenem nistagmusu zahteva kirurško zdravljenje, ki 
vključuje recesijo in resekcijo zunajočesnih mišic zaradi premika ničte točke v primarni položaj.
39-letna bolnica je bila obravnavana zaradi slabovidnosti in nistagmusa, ki je bil prisoten od otro-
štva. Glavo je od otroštva obračala v levo in imela stalne bolečine v vratu. Ob prvem pregledu je 
bila najboljša korigirana vidna ostrina 0,4 obojestransko z očali. Korekcija na desnem očesu je 
bila –6,50–1,50/180° in na levem očesu –5,50–2,50/180°. Izmerjena objektivni in subjektivni škilni 
kot sta bila +4°, fuzija od –3° do +29° s prisotnim stereo vidom. Ob izvajanju testa cover je bil pri-
soten nistagmus, ki se je med pokrivanjem okrepil. Gibljivost zrkel ni bila omejena. Na očesnem 
Slovenian
Medical
Journal
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and 15Δ base temporally in front of the 
left eye. Prisms were well tolerated by the 
patient. Also, no apparent head turn was 
noticed while wearing the prisms.
Nine months after the first examina-
tion, the patient underwent surgery - 
Kestenbaum procedure. Retroposition of 
the lateral rectus muscle (6.5 mm) with 
resection of the medial rectus muscle 
(5.5 mm) in the right eye and retropo-
sition of the medial rectus muscle (4.5 
mm) with resection of the lateral rectus 
muscle (7.5 mm) in the left eye were per-
formed. One week after the surgery, the 
nystagmus dampened the most in the 
slight left gaze, the head position was 
normal. Two years after the procedure 
the nystagmus dampened the most in the 
primary position. Contact lenses which 
she did not wear before, were prescribed 
to the patient one year after the proce-
dure. Her visual acuity improved slowly 
during following years. Nine years after 
Figure 1: A left head turn and some left tilt adopted by the patient since 
her childhood.
Figure 2: The nine cardinal positions of gaze in a patient 9 years after surgery. In the primary 
position (image in the middle) the patient looks straight and nystagmus is almost absent. In 
other cardinal positions of gaze nystagmus is still present.
1 Introduction
Congenital nystagmus is an eye 
movement disorder characterized by 
involuntary oscillations. Patients with 
congenital nystagmus often turn the 
head to place the eyes in a direction 
of gaze with the least nystagmus (null 
zone). Children with congenital nystag-
mus tend to prefer gaze in the direction 
of null zone to gain optimal visual acuity. 
When the null zone is located eccentric 
ozadju so bile ugotovljene degenerativne spremembe zaradi kratkovidnosti. Ob prvem pregledu 
so ji bile predpisane prizme, ki jih je dobro prenašala. Tudi položaj glave je bil ob nošnji prizem 
poravnan.
Devet mesecev po prvem pregledu je bila pri bolnici opravljena Kestenbaumova operacija. Nap-
ravljena je bila retropozicija zunanje preme mišice in resekcija notranje preme mišice na desnem 
očesu in retropozicija notranje preme mišice in resekcija zunanje preme mišice na levem očesu. 
Po posegu je bil nistagmus najbolj umirjen v rahlo levi poziciji, položaj glave je bil poravnan. Dve 
leti po posegu pa je bil nistagmus najbolj umirjen v primarni poziciji, glava pa je ostala izravnana. 
Pri bolnici je bila 9 let po operaciji in s korekcijo s kontaktnimi lečami vidna ostrina obojestransko 
0,8–0,9p.
Primer je prikazal, da sta ustrezna funkcionalna in kirurška obravnava omogočili dober morfolo-
ški rezultat z izboljšanjem vidne ostrine pri prirojenem nistagmusu in kompenzatorni drži glave 
tudi v odrasli dobi.
Cite as/Citirajte kot: Kurent A, Kosec D. Surgical treatment of a patient with congenital nystagmus with 
compensatory head posture: A case report. Zdrav Vestn. 2020;89(9–10):515–20.
DOI: https://doi.org/10.6016/ZdravVestn.2918
Copyright (c) 2020 Slovenian Medical Journal. This work is licensed under a
Creative Commons Attribution-NonCommercial 4.0 International License.
to the primary gaze, a compensatory 
head posture is frequently adopted (1-
5). The surgical correction of compen-
satory head posture involves extraocular 
muscle surgery in each eye to move the 
eccentric null zone to the primary or 
straight ahead position (2,6-9), but such 
surgery is probably carried out by a mi-
nority of ophthalmologists (10).
The purpose of the case report is to 
present surgical and functional manage-
ment in an adult patient with congeni-
tal nystagmus and a compensatory head 
posture which led to a good and stable 
morphological result with improved vi-
sual acuity.
2 Case presentation
A 39-year-old patient presented to 
the Eye Hospital, University Medical 
Centre Ljubljana with an impaired visual 
acuity and a horizontal nystagmus that 
was present since childhood. Since her 
517
SHORT SCIENTIFIC ARTICLE
Surgical treatment of a patient with congenital nystagmus with compensatory head posture: A case report
childhood, her head was turned to the 
left and she had permanent neck pain. 
She did not report any other health prob-
lems. At the first examination, best cor-
rected visual acuity was 0.4 in both eyes 
with her glasses with a left head turn and 
some left tilt. Correction in the right eye 
was -6.50-1.50/180° and in the left eye 
-5.50-2.50/180°. Measured objective and 
subjective angles of squint were +4°, fu-
sion from -3° to +29°, with the presence 
of stereo vision. The patient adopted a left 
head turn and some left tilt (Figure 1), 
which allowed her to use her null zone in 
the right gaze in which nystagmus damp-
ened the most and visual acuity was the 
best. During the cover test, the nystag-
mus was present and it enhanced while 
covering the eye. Ocular motility was not 
limited. Fundus examination revealed 
myopic changes in both eyes. At the first 
examination, prisms were prescribed 
15Δ base nasally in front of the right eye 
and 15Δ base temporally in front of the 
left eye. Prisms were well tolerated by the 
patient. Also, no apparent head turn was 
noticed while wearing the prisms.
Nine months after the first examina-
tion, the patient underwent surgery - 
Kestenbaum procedure. Retroposition of 
the lateral rectus muscle (6.5 mm) with 
resection of the medial rectus muscle 
(5.5 mm) in the right eye and retropo-
sition of the medial rectus muscle (4.5 
mm) with resection of the lateral rectus 
muscle (7.5 mm) in the left eye were per-
formed. One week after the surgery, the 
nystagmus dampened the most in the 
slight left gaze, the head position was 
normal. Two years after the procedure 
the nystagmus dampened the most in the 
primary position. Contact lenses which 
she did not wear before, were prescribed 
to the patient one year after the proce-
dure. Her visual acuity improved slowly 
during following years. Nine years after 
Figure 1: A left head turn and some left tilt adopted by the patient since 
her childhood.
Figure 2: The nine cardinal positions of gaze in a patient 9 years after surgery. In the primary 
position (image in the middle) the patient looks straight and nystagmus is almost absent. In 
other cardinal positions of gaze nystagmus is still present.
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surgery and refractive correction with 
contact lenses, the visual acuity was 0.8-
0.9p in the right and left eye. Null zone 
was in the primary position (Figure 2).
3 Discussion
In the presented adult case, head po-
sition corresponded to the null zone, 
similarly as in most patients with infan-
tile nystagmus (4). Attempts to eliminate 
compensatory head posture in infantile 
nystagmus with an eccentric null zone 
with various extraocular muscle surger-
ies have been tried, but the Kestenbaum 
procedure as modified by other authors, 
is probably now the most commonly 
performed (11). 
Surgical procedures for the correc-
tion of compensatory head positions 
in patients with congenital nystagmus 
began with the independent reports of 
Anderson, Goto and Kestenbaum in 
the early 1950s (7,12-15). Since its ini-
tial description, the procedure has been 
modified by Parks, Calhoun and Harley, 
and Taylor and Jesse (7,15). Anderson 
proposed to weaken the horizontal rec-
tus muscles that are activated during the 
slow phase of the nystagmus because 
they were thought to have a greater tone 
than their antagonists. Therefore, in a 
patient with a concordant left head turn, 
an Anderson-like procedure would con-
sist of a supralarge recession of the right 
lateral rectus and left medial rectus mus-
cles (4,13,16). A Kestenbaum (4,12) pro-
cedure combined 5 mm recessions with 
resections of the antagonist muscles; 
however, the initial procedure led to a 
high rate of hypocorrections and Pratt-
Johnson increased the amount of mus-
cle surgery. The former 5 mm recessions 
and resections were increased to 10 mm 
(4). Parks modified the Kestenbaum 
technique to the 5-6-7-8 or the “Classic 
Maximum” procedure in which a re-
cession of 7 mm was performed to the 
lateral rectus, a resection of 6 mm to 
the medial rectus of the abducted eye, a 
recession of 5 mm to the medial rectus, 
and finally a resection of 8 mm to the 
lateral rectus of the adducted eye (4,17). 
Similar surgery was performed in the 
presented case. 
It has been demonstrated that sur-
gery on all four horizontal muscles 
was a safe and effective method for al-
tering the compensatory head position 
in congenital nystagmus (7,8,18,19). It 
was indicated that varying degrees of 
improvement may be expected in the 
studies that included mostly paediatric 
patients as well as some adults (6-9,18), 
but complete elimination of the head 
turn occurred in only 6 of 19 patients 
(32%) in one older study (18). Newer 
studies report 26 of 36 patients (72%) 
with a residual head turn not exceeding 
10° after surgery (6) and 34 of 44 (77%) 
cases with a normal head posture or 
head turn of less than 5° after surgery 
(9). The most common anomalous head 
position following Kestenbaum surgery 
was usually in the same direction as was 
present prior to surgery (15). Similarly, 
in the presented case nystagmus damp-
ened the most in the slight left gaze af-
ter surgery and the patient reported to 
be confused about the head position. 
Probably the confusion was due to the 
discrepancy between neck propriocep-
tion and new eyeball position, but later 
this discrepancy has been resolved. It is 
possible for new or recurrent compen-
satory head postures to reappear years 
after the initial treatment, which was 
reported in the study of four children 
after Anderson-Kestenbaum surgery 
(15). But since the head position was 
still stable 9 years after surgery and the 
visual acuity improved, new null zone 
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SHORT SCIENTIFIC ARTICLE
Surgical treatment of a patient with congenital nystagmus with compensatory head posture: A case report
development in the presented case is 
probably unlikely. 
Compensatory head position was 
more often reported as horizontal in the 
literature, but it may also be vertical or 
take the form of a tilt even though the 
nystagmus itself is horizontal (10). In 
the presented case, there was a combi-
nation of predominant left head turn 
and some left tilt, which both improved 
after surgery on the horizontal rectus 
muscles. Head tilt and vertical head 
turns may improve with only weakening 
surgery of the horizontal rectus muscles 
in those cases in which the horizontal 
head turn predominates, at least when 
other components are not severe. This 
approach is thought to work by moving 
the blockage point to the primary posi-
tion where the cyclovertical muscles’ ac-
tions are weaker (4,20). Arroyo-Yllanes 
et al. treated 21 patients with horizontal 
nystagmus and compensatory head po-
sition in all three axes with a predomi-
nant head turn. In all cases a modified 
Anderson procedure was performed - 
that is, 2 mm retroequatorial recessions 
of the horizontal yoke rectus muscles 
responsible for the blockage position, 
plus corrective surgery for strabismus 
when needed. The three components of 
the compensatory head position were 
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and improved cosmesis. Additionally, 
refractive correction with contact lenses 
led to improved visual acuity.
Consent was obtained from the pa-
tient for the publication of this case 
presentation.
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