Incidence, Management, and Outcome of Pulled-in-Two Syndrome Associated With Strabismus Surgery
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Incidence, Management, and Outcome of Pulled-in-Two
Syndrome Associated With Strabismus Surgery
Miao Kong, MD; Yuanyuan Han, MD; Junhong Li, MD; Xueliang Feng, MD; Lijun Zhang, MD; Hong Zhang, MD
ABSTRACT Conclusions: Pulled-in-two syndrome is one of the rar-
Purpose: To report the incidence and outcomes of pulled- est and most severe complications of strabismus sur-
in-two syndrome during strabismus surgery and investi- gery. Cranial nerve palsy, advanced age, prior surgery,
gate the clinical features and management of the disease. thyroid-associated ophthalmopathy, and degenerative
conditions of the extraocular muscle are risk factors for
Methods: The medical records of patients who under- pulled-in-two syndrome. Therefore, the prevention of
went strabismus surgery and developed pulled-in-two pulled-in-two syndrome in patients with these risk fac-
syndrome between July 2013 and October 2020 were re- tors should be investigated further.
viewed retrospectively. The demographic characteristics,
intraoperative details (including surgery type and man- [J Pediatr Ophthalmol Strabismus. 20XX;X(X):XX-XX.]
agement), and subsequent and final outcomes were ex-
tracted from the records.
INTRODUCTION
Results: Of the 11,824 strabismus surgeries during the Pulled-in-two syndrome, or sudden rupture
study period, 4 cases of pulled-in-two syndrome were of the extraocular muscle under minimal tension
documented, accounting for an overall incidence of intraoperatively during strabismus surgery, is an
0.034%. The average age of the patients with pulled- uncommon but potentially catastrophic surgical
in-two syndrome was 61.75 ± 8.99 years. All 4 patients complication. It was reported first and named by
were women; 2 had abducens nerve palsy and the Greenwald in 1990.1 Pulled-in-two syndrome usu-
other 2 had myopic strabismus fixus. The inferior rectus ally occurs when extraocular muscles are hooked by
muscle was involved in 1 patient and the medial rectus strabismus hooks during strabismus surgery without
muscle was involved in 3 patients. All of the involved excessive force. Pulled-in-two syndrome is extremely
extraocular muscle was lost. One patient achieved or- rare, and the incidence has been estimated to be 1 in
thotropia, and the others were undercorrected after the 100,000 following strabismus surgery.2,3 Hitherto,
surgery. only a small number of cases have been published
From Tianjin Medical University, Tianjin, China (MK, YH, JL); the Departments of Strabismus and Pediatric Ophthalmology (MK, JL, XF, LZ) and
Orbit and Ocular Tumor (YH), Shanxi Provincial Eye Hospital, Taiyuan, China; and the Department of Ophthalmology, Sino-Singapore Eco-City Hospital
of Tianjin Medical University, Sino-Singapore Eco-City, Tianjin, China (HZ).
© 2022 Kong, Han, Li, et al; licensee SLACK Incorporated. This is an Open Access article distributed under the terms of the Creative Commons Attribu-
tion 4.0 International (https://creativecommons.org/licenses/by/4.0). This license allows users to copy and distribute, to remix, transform, and build upon the
article, for any purpose, even commercially, provided the author is attributed and is not represented as endorsing the use made of the work.
Submitted: April 9, 2022; Accepted: June 23, 2022; Posted online: August 8, 2022
Supported by the Research Foundation of Health Commission of Shanxi Province, Taiyuan, China (Grant No. 2017100).
Disclosure: The authors have no financial or proprietary interest in the materials presented herein.
Drs. Kong and Han contributed equally to this work and should be considered as equal first authors.
Correspondence: Hong Zhang, MD, Department of Ophthalmology, Sino-Singapore Eco-City Hospital of Tianjin Medical University, No. 3333 Hechang
Road, Sino-Singapore Eco-City, Tianjin 300467, China. Email: eyezhanghong@163.com
doi:10.3928/01913913-20220627-01
Journal of Pediatric Ophthalmology & Strabismus • Vol. XX, No. X, 20XX 1
TABLE 1
Clinical Summary of Patients With Pulled-in-Two Syndrome
Characteristic Case 1 Case 2 Case 3 Case 4
Gender Female Female Female Female
Age (years) 71 51 58 67
Chief claim Left eye esotropia for Right eye esotropia Right eye esotropia Bilateral eye esotro-
40 years for 50 years for 40 years pia for 40 years
Ocular history Abducens nerve Abducens nerve High myopia and High myopia, am-
palsy in left eye and palsy and amblyopia amblyopia in right blyopia, and cataract
cataract in both eyes in right eye eye in both eyes
Ocular alignment Left eye esotropia Right eye esotro- Right eye esotropia Both eyes fixed in
and motility preop- of 110 PD with com- pia of 100 PD with of 120 PD and hypo- nasal-inferior posi-
erative plete limitation of complete limitation tropia of 20 PD with tion with movement
abduction of abduction significantly limited limitation in all direc-
duction tions
Diagnosis Abducens nerve Abducens nerve Myopic strabismus Myopic strabismus
palsy of left eye palsy of right eye fixus of right eye fixus of both eyes
Plan LMR RES+Nishida RMR RES+Nishida RMR RES+Yokoyama BMR RES+Yokoyama
procedure procedure procedure procedure
Intraoperative com- Left IR ruptured ap- Right MR ruptured Right MR ruptured Right MR ruptured
plication proximately 4 mm approximately 5 mm approximately 4 mm approximately 4 mm
posterior to the in- posterior to the in- posterior to the in- posterior to the in-
sertion and was lost sertion and was lost sertion and was lost sertion and was lost
Management LMR RES+IO anterior RMR free+Nishida RMR free+Yokoyama RMR free+LMR
transposition procedure procedure RES+Yokoyama
procedure
Histopathological Muscle tissue fibrosis Muscle tissue fibrosis Muscle tissue atro- Muscle tissue sig-
examination with significant with hypertrophy phied with fibrosis nificant interstitial
hyaline degeneration fibrosis
and scarring
Ocular alignment +15 PD Ortho +25 PD +10 PD
postoperative
BMR = bilateral medial rectus; IO = inferior oblique; IR = inferior rectus; LMR = left medial rectus; MR = medial rectus; ortho = orthotropia; PD = prism
diopters; RES = recession; RMR = right medial rectus
on this condition. Thus, the current study aimed to extracted. The following data were collected: de-
evaluate the incidence and outcome of pulled-in- mographic profile, medical history, preoperative
two syndrome associated with strabismus surgery in and postoperative ocular deviation in primary gaze,
our center. preoperative and postoperative ocular motility, limi-
tation of ductions in the affected eye, type of stra-
PATIENTS AND METHODS bismus surgery performed, intraoperative and post-
This study was approved by the Shanxi Provincial operative complications, slit-lamp examination for
Eye Hospital Ethics Committee (SXYYLL20210531) signs of anterior segment ischemia, and duration of
and conducted according to the tenets of the Dec- postoperative follow-up.
laration of Helsinki. Informed consent was obtained
from all patients prior to participation. RESULTS
The medical records of all patients with pulled- A total of 11,824 strabismus surgeries were
in-two syndrome who underwent strabismus sur- performed during the study period. Subsequently,
gery in our department between 2013 and 2020 4 patients experienced pulled-in-two syndrome,
were reviewed, and the relevant information was accounting for an overall incidence of 0.034%.
2
A B
Figure 1. Case 1. (A) Preoperative photograph showing a large-angle esotropia. (B) Postoperative photograph 2 weeks after left medial
rectus recession, inferior rectus muscle tenotomy (pulled-in-two syndrome with unrecovered muscle), and inferior oblique muscle anterior
transposition.
A B C
Figure 2. Histopathology of three different patients shows (A) significant muscle fiber atrophy, (B) fibrous tissue hyperplasia, and (C) ocular
muscle fibrosis with significant hyaline degeneration (hematoxylin–eosin staining; original magnification ×200).
The data of the patients who developed pulled-in- the snapped inferior oblique muscle were excised for
two syndrome are summarized in Table 1. All 4 pathological examination (Figure 1). Strikingly, the
cases were women, and the mean age at surgery was medial rectus muscle ruptured at the time of being
61.75 ± 8.99 years (range: 51 to 71 years). None of sutured during surgery in the remaining cases. Re-
these cases had undergone previous ocular surgery. peated forced duction testing was performed after
One patient (case 1) had depression, whereas that and showed the limitation in ocular motility
others had no history of systemic disease. In addi- was reduced but still persistent. Free myotomy of
tion, these patients had no history of ocular trauma the medial rectus muscle was allowed given the re-
or surgery. Cases 1 and 2 were diagnosed as having striction, and we proceeded with the Nishida proce-
abducens nerve palsy, and cases 3 and 4 were diag- dure (case 2) or Yokoyama procedure (case 3 and 4).
nosed as having myopic strabismus fixus. None of The involved extraocular muscles ruptured approxi-
the patients reported diplopia prior to surgery. The mately 4 mm posterior to the insertion in cases 1, 3,
surgery aimed to improve the cosmetic appearance. and 4, and 5 mm posterior to the insertion in case 2.
The forced duction test administered intraopera- All of the proximal portions of the muscles retracted
tively showed a marked restriction. During surgery into the orbit and could not be recovered after care-
on case 1, the left medial rectus muscle was recessed ful exploration; the distal ends of the snapped ocular
by 8 mm first and the left inferior rectus muscle was muscle also were excised for pathological examina-
then isolated. As the muscle was being held on a tion. The data are shown in Table 1. Histopatho-
strabismus hook without undue force, it ruptured logical abnormalities were noted in these patients
approximately 4 mm posterior to the insertion and (Figure 2).
the proximal end was retracted into the orbit. Si-
multaneously, the inferior oblique muscle was trans- DISCUSSION
posed to the lateral edge of the inferior rectus inser- Pulled-in-two syndrome is a rare and devastat-
tion with 6-0 polyglactin 910. The distal ends of ing complication of strabismus surgery. Consider-
Journal of Pediatric Ophthalmology & Strabismus • Vol. XX, No. X, 20XX 3
ing the differences in anatomy, physiology, report- muscle insertion, suggesting that the most suscep-
ing bias, investigation methods, and geographical tible point is the muscle–tendon junction.3,5,8,9 Ellis
distribution of strabismus in different populations, et al3 reported that the rupture mostly occurred 4
the incidence rate of pulled-in-two syndrome var- to 9 mm from the point of insertion (24 [60%], ie,
ies widely. Erzurum and Greenwald2 reported a 1 in the muscle–tendon junction). In the current cases,
5,000 incidence of pulled-in-two syndrome based on all muscles ruptured approximately 4 to 6 mm from
a multicenter questionnaire survey of 60 members of their insertion.
the American Association for Pediatric Ophthalmol- Based on the previous reports, it was inferred
ogy and Strabismus (AAPOS). Ellis et al3 reported that the most commonly associated risk factors were
51 cases of pulled-in-two syndrome collected from previous ocular surgery and cranial nerve palsy, fol-
29 surgeons at 28 institutions or practices across six lowed by thyroid disease and scleral buckle. The other
countries and estimated that a strabismus surgeon potential risk factors included advanced age, ocular
might encounter one case of pulled-in-two syndrome and orbital trauma, pathological myopia, congenital
over 10 years of practice. Another prospective study exotropia, strabismus fixus, myasthenia gravis, Down
conducted by Bradbury and Taylor4 comprehensive- syndrome, mitochondrial myopathy, and congenital
ly investigated the complications of approximately fibrosis of the extraocular muscles.3,5-10 In the current
24,000 strabismus surgeries performed in the United study, 2 cases presented abducens nerve palsy, and
Kingdom over 2 years; during this time, only 5 cases the other 2 cases had high myopic strabismus fixus.
with pulled-in-two syndrome were reported. Herein, The histopathological abnormalities in these muscles
we reviewed the patient records of 11,824 strabismus included degenerative changes, increased connec-
surgeries performed in our department over a 7-year tive tissue, and abnormal muscle fibers,11 similar to
period; of these, 4 cases of pulled-in-two syndrome the previous reports. Another risk factor for pulled-
were documented. Thus, the overall incidence of in-two syndrome is advanced age. Previous reports
0.034% was consistent with that reported previously. showed that pulled-in-two syndrome can occur at
Erzurum and Greenwald2 and Ellis et al3 re- any age but is more likely to occur in older patients,
ported that the most commonly involved muscles with the peak age range from 60 to 69 years.3 In our
in pulled-in-two syndrome were the medial rectus cases, the mean age was 61.75 ± 8.99 years (range:
and inferior rectus. Among the 40 cases of pulled- 51 to 71 years). Although the gender differences in
in-two syndrome reported by Ellis et al, medial rec- pulled-in-two syndrome have not been reported, the
tus and inferior rectus muscle rupture accounted results of 26 of 40 (65%) female cases in the study
for more than 85% of the cases, whereas no case by Ellis et al3 and all 4 female cases in our series indi-
of superior oblique muscle rupture was reported. In cated that pulled-in-two syndrome is likely to occur
some studies, pulled-in-two syndrome also occurred in older women.
in the medial, inferior, lateral, and superior rectus The preferred management for pulled-in-two
muscles.4-8 In our case series, the medial rectus mus- syndrome is surgical recovery and reattachment of
cle was involved in 3 cases and the inferior rectus the snapped muscle; however, if the muscle is lost,
muscle was involved in the remaining 1 case. This either transposition surgery can be performed or
finding was consistent with previous studies. Ellis the patient may be observed. In the study by Ellis
et al3 speculated that the occurrence of pulled-in- et al,3 the snapped muscles in 27 of 40 (68%) cases
two syndrome for a specific extraocular muscle was with pulled-in-two syndrome were identified and
dependent on how commonly that muscle is oper- retrieved to the insertion, one-half of the other cas-
ated on. Intriguingly, the surgery on the superior es with lost muscles achieved acceptable results in
oblique muscle is relatively uncommon compared primary gaze through the transposition procedures,
to other extraocular muscles, and the anatomy or and the remaining cases were monitored by a close
intrinsic characteristics might contribute to the rate follow-up postoperatively. Transposition surgery is
of pulled-in-two syndrome. an option in patients whose muscle cannot be found
Notably, pulled-in-two syndrome can occur in or may be too friable to retrieve, but it may carry
any part of the extraocular muscle, including the the risk of anterior segment ischemia, especially full-
muscle tendon and belly. However, the specific loca- tendon transposition surgery.5-10,12 An alternative to
tion of the dehiscence is several millimeters from the transposition surgery is the Nishida procedure. The
4
Nishida procedure without tenotomy or splitting 3. Ellis EM, Kinori M, Robbins SL, Granet DB. Pulled-in-two syn-
drome: a multicenter survey of risk factors, management and out-
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sy, Duane syndrome, and medial rectus transection jaapos.2016.06.004 PMID:27647114
4. Bradbury JA, Taylor RH. Severe complications of strabismus
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satisfactory results.13-15 Some surgeons5 also report- jaapos.2012.10.016 PMID:23352718
5. Wallace DK, Virata SR, Mukherji SK. Strabismus surgery
ed good outcomes with no intervention because the complicated by “pulled in two syndrome” in a case of breast
retracted muscle might retain some function. Wan carcinoma metastatic to the medial rectus muscle. J AAPOS.
2000;4(2):117-119. https://doi.org/10.1067/mpa.2000.103438
and Hunter16 suggested that transposition surgery PMID:10773811
should be performed after the patient recovers. 6. Jones AM, Starte J, Dunn H, Ahmad K, Tan K. Surgical technique
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an inferior oblique transposition procedure, whereas S. Pulled into two syndrome (PITS) in a case of congenital fibrosis
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1230. https://doi.org/10.4103/ijo.IJO_1542_18 PMID:31238477
and no additional surgery was performed. However, 8. Palko J, Yandrick M, Shepherd JB. A case of indirect superior
all cases achieved an acceptable appearance. Thus, rectus pulled-in-two syndrome during superior oblique tenec-
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it is recommended that these patients be observed jaapos.2018.04.012 PMID:30120983
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