Education

Case Report: Case 6

This is the 1st in a series of Strabos Institute & WSPOS collaboration cases.
Case Presenters

Dr. Bruna Lana Ducca de Andrade obtained her medical degree from the Faculdade de Ciências Médicas de Santos, São Paulo in 2006. After medical school, Dr. de Andrade completed her residency in Ophthalmology at Santa Casa de Misericórdia de São Paulo in 2011 and in 2013 she completed a Strabismus fellowship at the same institution. In 2015 Dr. de Andrade was selected for a scholarship through the PAAO and completed a one year handson Paediatric Ophthalmology and Strabismus fellowship at Children’s Medical Center, affiliated to the University of Texas Southwestern in Dallas.

After completing her fellowship, Dr. de Andrade returned to Brazil and currently works at her own practice. She is also a volunteer attending at the Strabismus service, at the Ophthalmology Department of the Universidade Federal de São Paulo (UNIFESP) and a member of the Scientific Committee of the Strabos Institute, in São Paulo

Status: CASE CLOSED

Members’ Responses:

When asked (1) Do you feel recommending orbital imaging studies in suspected muscle injuries is Mandatory / Useful but not mandatory / You would not recommend prior imaging studies because the findings during surgical exploration are the key to the surgical plan; 85.71% felt it was Mandatory while 14.29% felt it was Useful but not mandatory. No one replied saying they would not recommend prior imaging studies because the findings during surgical exploration are the key to the surgical plan.

When asked (2) What would you do as an initial approach to this case? 4.76% replied saying they would wait for recovery of the muscle function, 9.52% said they would give a short trial of systemic corticosteroids to reduce inflammation and scarring, 14.29% would opt for Botulinum toxin injection to the antagonist lateral rectus muscle and 71.43% preferred Surgical exploration.

When asked (3) Which approach would you choose to treat medial rectus muscle injuries after endoscopic sinus surgery? 9.52% replied saying they would opt for Vertical rectus transposition, 14.29% would inject Botulinum toxin into the antagonist lateral rectus muscle, 47.26% would opt for Anterior orbitotomy and medial rectus exploration and 28.57% would prefer a Transnasal endoscopic assessment.

Experts Opinion

In addition to answering the questions posed to our members, our Experts were also asked the following questions : 1) Assuming the medial rectus was adherent to the orbital defect, would you expect a positive forced duction test? 2) What would be the optimal time for surgery? 3) If the muscle had been transected and a large segment were missing or destroyed, what would be your surgical approach?

Cumhur Sener

Dr. Cumhur Sener Graduated from the Hacettepe University Medical School, and finished the residency at the Hacettepe University Department of Ophthalmology in Ankara, Turkey. His
Postgraduate research was (a) in vision research at the Netherlands Ophthalmic Research Institute with Henk Spekreijse at Amsterdam, the Netherlands and (b) in strabismus at the Wilmer Institute with David Guyton at Baltimore, USA. He worked at the Hacettepe University Department of Ophthalmology from 1991 – 2014. He volunteered as a visiting faculty in the ORBIS project at Chennai, India in 2002, and also multiple times at Baku, Azerbeijan from 2008 – 2013. He is currently engaged in private practice in Ankara, Turkey. His main fields of interest are Adult and Pediatric Strabismus and Pediatric Ophthalmology.

Dr. Sener began by congratulating the surgeons for this difficult case with an excellent outcome. Here are his answers to our questions :
(1) Do you feel recommending orbital imaging studies in suspected muscle injuries is?

Mandatory. I think orbital imaging is mandatory for this kind of an obvious medial rectus injury presenting with immediate exotropia. It will not only provide invaluable information about the site and extent of the injury but also mechanical and innervational consequences by combining the kinetic mode.

(2) What would you do as an initial approach to this case?

This kind of a case needs immediate intervention at the moment of recognizing a transected muscle, if possible right on the table. The first measure should be placing a temporary stay suture in order to keep the torn sides as close as possible and preventing the contracture of the antagonist muscle. I use a nonabsorbable 5.0 spatulated needle in order to pass from the nasal limbus, engaging the episcleral fibers and pulling the eye towards the nose then suturing with a bolster at the nasal skin. If cheese-wiring occurs, one can pass the suture from either or both of the vertical recti. In this case special attention must be exercised in order to protect the cornea from chronic suture contact. Botulinum toxin injection (BTI) might seem handy, however there are two important caveats. First, its required action is delayed –a few days or a week- and second during or following a strabismus repair any adjustment to a desired target can be erroneous because the real magnitude of the deviation would be disguised. BTI would follow my primary strabismus repair if needed. The goal should be trying to bring back together the transected parts. The regenerative capacity of the extraocular muscles can be surprising. I have seen an originally exotropic case whose transected portion of the lateral rectus was sewn back to the stump 8 mm from the insertion, upon when the surgeon noticed that it should in fact needed recession not resection, and the outcome was surprisingly good. The injury in this case may not be limited to a straightforward transection but a segment of muscle may actually be missing or destroyed and at this far posterior location there might be an associated nerve injury. Parenteral corticosteroid injection under antibiotic coverage and gastric protection should be useful in diminishing the inflammation and protecting the orbital content against bacterial contamination from the sinusitis.

(3) Which approach would you choose to treat medial rectus muscle injuries after endoscopic sinus surgery?

The second step in this particular case is to form a team with an orbital surgeon and an ENT specialist with significant experience in endoscopic sinus surgery. The MR indicates that the proximal end of the muscle is way behind the globe near the orbital apex. The orbital surgeon would most likely use a transcaruncular incision, however this will give limited access to the far posterior compared to an endoscopic transethmoidal approach which can provide exposure up towards the optic canal. The major problem facing either of the specialists is in fact the disturbed orbital anatomy especially the intraorbital fat tissue of both the intra and extraconal space and hematoma. We had to go through with such a procedure in order to expose and transect the fibrotic bands of a severe CFEOM infantile case (Strabismus surgery in congenital fibrosis of the extraocular muscles: a paradigm. Sener EC, Taylan Sekeroglu H, Ural O, Oztürk BT, Sanaç AS. Ophthalmic Genet. 2014 Dec;35(4):208-25; Case 15). Although our goal was to create a controlled medial rectus transection through endoscopic sinus surgery, the fat tissue was completely obscuring our sight and the transection had to be carried on with blind maneuvers. In this aspect, the surgeons of this case have to be congratulated in being able to isolate the proximal end and effectively passing the suture in a very tight place. I was wondering whether this kind of a repair would as well be effective by suturing the distal end of the transected medial rectus to the posterior orbital periosteum as long as there is enough innervation at the distal portion. If suturing of the transected ends turns out to be successful, I would leave the globe at about 15 pd of esotropic state. BTI can be added at any time following the surgery, especially if there is no sign of recovering adduction in a few weeks. One may not be lucky enough in isolating the torn muscle, or the muscle may not recover with the appropriate innervation. Then ciliary vessel sparing monovertical transposition with 3-4 mm resection and moderate to large lateral rectus recession would be my surgical option. Unfortunately, BTI alone in this magnitude of exotropia has not been successful in my hands regardless of the dose increments. The contralateral lateral rectus recession may be added in order to match the left gaze deficit. I would leave the second vertical rectus as the last step as a protection against anterior segment ischemia. As far as circulation concerned, I do not have any experience with Nishida transposition technique, however I suspect this technique or a partial tendon thickness transposition should leave more scar tissue than a controlled cilary vessel sparing full tendon transposition, and this will make things even more complicated if and when a revision is needed. When everything else fails in correcting the exotropia, and cosmesis remains as a significant issue, I found the nonabsorbable suture fixation of the medial rectus insertion to the posterior lacrimal crest through caruncular incision being very useful in maintaining alignment at the primary position although this will obviously restrict the lateral gaze.

(4) Assuming the medial rectus was adherent to the orbital defect, would you expect a positive forced duction test?

One must consider the fact that extensive scar throughout the medial orbit is unavoidable in this case and the forced duction test would be positive if enough time has passed for fibrosis of the orbital soft tissue to take over.

(5) What would be the optimal time for surgery? & (6) If the muscle had been transected and a large segment were missing or destroyed, what would be your surgical approach?

I believe I tried to explain these points in 2, and 3.

Venkateshwar Rao

Dr. Venkateshwar Rao pursued his Ophthalmology training at the R.P. Center, All India Institute of Medical Sciences (AIIMS), New Delhi; following which he underwent a fellowship in Strabismus and Neuro-Ophthalmology under Prof. Prem Prakash from the same institute. He then went on to do a 2nd Fellowship in Pediatric Ophthalmology & Strabismus under the tutelage of Prof. Eugene Helveston from the Indiana University Medical Center, Indianapolis, USA. Dr. Rao is a specialist in Pediatric Strabismus, Adult Strabismus and Neuro-Ophthalmology. In addition to his fellowship training, he also visited various Hospitals in the USA. He served as Head of the Pediatric Ophthalmology, Strabismus and Neuro-Ophthalmology service at L. V. Prasad Eye Institute, Hyderabad, India right from the unit’s inception in January 1998 & was with L. V. Prasad Eye Institute till February 2006.

With his vast clinical and academic experience, Dr. Rao started Shreya Eye Care Center in March 2006 with a mission of providing quality care with compassion in the field of Strabismus, Pediatric eye problems and Neuro-Ophthalmology & is presently the Director of the same. He is a present and past member of several prestigious international organizations including AAO, AAPOS, ISA, AIOS, SPOSI, WSPOS & APAO.

Dr. Rao has lectured at various Universities in India, USA, Indonesia, Sri Lanka & Bangladesh, covering several topics of Strabismus, Amblyopia, Congenital Cataract and Neuro-Ophthalmology in
addition to having published articles in peer reviewed international journals and presented at various international meetings such as AAO, AAPOS, ESA, ISA, WSPOS, APAO & IOA.

Do you feel recommending orbital imaging studies in suspected muscle injuries is?
Mandatory.

(2) What would you do as an initial approach to this case?

Surgical exploration

(3) Which approach would you choose to treat medial rectus muscle injuries after endoscopic sinus surgery?

Transnasal endoscopic assessment.

(4) Assuming the medial rectus was adherent to the orbital defect, would you expect a positive forced duction test?

If the muscle has laceration and is adherent (entrapment) to the orbital wall defect, the patient will show variable degrees of limitation when attempts abduction and a great adduction deficit. This limited abduction has a restricted origin and the forced duction test must be positive. Others features may be the retraction of the globe in the orbit when abduction is attempted with lid fissure narrowing and crepitation of the lids skin. If the muscle is transected the result of the forced duction test depends on whether the anterior segment of the muscle is entrapped (abduction deficit with positive forced duction test) or not (full abduction without restriction).

(5) What would be the optimal time for surgery?

Immediate repair of the muscle laceration or transection is indicated as soon as possible. As time goes by, both segments of the transected muscle begin to lose vitality and the fibrosis process of the wounds could shorten muscle length making it impossible for the surgeon to suture both segments. The same argument could be applied if restoring blood flow and innervation is expected. On the other hand, mild or extreme injuries with or without transection may be approached later on.

(6) If the muscle had been transected and a large segment were missing or destroyed, what would be your surgical approach?

If the muscle had been transected and a large segment were missing or destroyed, the best functional surgery to recover adducting forces would be the transposition of the nasal halves of the vertical rectus muscles, with or without Foster-like sutures. In addition, a mild resection of these halves would be performed to reinforce the lack of adducting forces to maintain the eye in primary position. This procedure may be complement with botulinum toxin injection of the antagonist lateral rectus muscle. If transposition results in poor recovery, we could plan to perform a fixation procedure on the lacrimal caruncle or orbital wall.

Fernando Prieto-Díaz

Dr. Fernando Prieto-Díaz is currently the Medical Chief of Instituto Oftalmológico Prieto-Díaz, La Plata, Argentina. He was the General Secretary of the Latin-American Council of Strabismus (CLADE) from 2003 – 2010 in addition to being the President of the Argentine Centre of Strabismus (CAE) from 2004 – 2006. He is the Editor in Chief of the REOP (Revista de Estrabismo & Oftalmología Pediátrica) Journal of Strabismus & Paediatric Ophthalmology since 2014. He is also the Strabismus Section Head of the WSPOS Global Consult Bureau.

(1) Do you feel recommending orbital imaging studies in suspected muscle injuries is?
Mandatory.

(2) What would you do as an initial approach to this case?

Surgical exploration

(3) Which approach would you choose to treat medial rectus muscle injuries after endoscopic sinus surgery?

Transnasal endoscopic assessment.

(4) Assuming the medial rectus was adherent to the orbital defect, would you expect a positive forced duction test?

If the muscle has laceration and is adherent (entrapment) to the orbital wall defect, the patient will show variable degrees of limitation when attempts abduction and a great adduction deficit. This limited abduction has a restricted origin and the forced duction test must be positive. Others features may be the retraction of the globe in the orbit when abduction is attempted with lid fissure narrowing and crepitation of the lids skin. If the muscle is transected the result of the forced duction test depends on whether the anterior segment of the muscle is entrapped (abduction deficit with positive forced duction test) or not (full abduction without restriction).

(5) What would be the optimal time for surgery?

Immediate repair of the muscle laceration or transection is indicated as soon as possible. As time goes by, both segments of the transected muscle begin to lose vitality and the fibrosis process of the wounds could shorten muscle length making it impossible for the surgeon to suture both segments. The same argument could be applied if restoring blood flow and innervation is expected. On the other hand, mild or extreme injuries with or without transection may be approached later on.

(6) If the muscle had been transected and a large segment were missing or destroyed, what would be your surgical approach?

If the muscle had been transected and a large segment were missing or destroyed, the best functional surgery to recover adducting forces would be the transposition of the nasal halves of the vertical rectus muscles, with or without Foster-like sutures. In addition, a mild resection of these halves would be performed to reinforce the lack of adducting forces to maintain the eye in primary position. This procedure may be complement with botulinum toxin injection of the antagonist lateral rectus muscle. If transposition results in poor recovery, we could plan to perform a fixation procedure on the lacrimal caruncle or orbital wall.

Monte Del Monte

Dr. Monte Del Monte received his medical degree from John’s Hopkins University in 1974. He completed a residency in Pediatrics at the Harvard Boston Children’s Hospital Medical Center and a
residency in Ophthalmology at the Johns Hopkins Wilmer Eye Institute. He also completed a postdoctoral fellowship training in pediatric ophthalmology and strabismus at the National Children’s
Hospital Medical Center in Washington D.C. Dr. Del Monte has presented 45 invited, guest, keynote and named lectures and published over 100 scientific papers in peer-reviewed journals. He has been the director of the Pediatric Ophthalmology Fellowship training program at the University of Michigan for the past 30 years He is currently Director of Pediatric Ophthalmology and Adult Strabismus, Skillman Professor of Pediatric Ophthalmology and Professor of Pediatrics and Communicable Diseases at the University of Michigan, in addition to being the Director of Ophthalmology at the Mott Children’s Hospital.

(1) Do you feel recommending orbital imaging studies in suspected muscle injuries is:
Mandatory : In a case of XT with absent Adduction following endoscopic sinus surgery orbital imaging is very useful to document the location and extent of the injury. Dynamic orbital MRI can also demonstrate the presence or absence of nerve involvement and contracture of the proximal stump. However, I do not find orbital imaging is useful in anterior orbital laceration as the injury is usually too far anterior to see on any but surface coil studies- the posterior orbital muscle position is usually unremarkable. There only surgical exploration is indicated.

(2) What would you do as an initial approach to this case?

Wait for recovery of the muscle function : I have seen 2 cases with minimal residual slips of intact MR muscle that appeared to be completely transected on MRI. The tipoff was a short Adduction jerk on attempted Adduction and a tiny positive active force generation on attempted Adduction. In both cases, very large initial XTs in primary and -5 Adduction recovered significant function over 5-6 weeks leaving only a small residual xt in primary gaze increasing in Adducting gaze which could be fixed with incommitance contralateral LR recession. If no improvement in Adducting saccade is seen in 2-3 weeks, then surgical exploration and attempted multidisciplinary repair as demonstrated here is indicated

(3) Which approach would you choose to treat medial rectus muscle injuries after endoscopic sinus surgery?

Transnasal endoscopic assessment : As demonstrated here, combined endoscopic and transconjunctival isolation of both transected ends of the MR muscle and then reanastamosis is by far the optimal procedure for best alignment, range of movement and possibly some SBVF as noted here.

(4) Assuming the medial rectus was adherent to the orbital defect, would you expect a positive forced duction test?

Early, before significant scarring and fibrosis, I would not expect significant positive forced Abduction. I would also test for any residual positive active force generation of the MR to suggest some residual un-transected muscle slip which could recover force with healing. However, later when the medial rectus distal stump became adherent there may be restricted Abduction.

(5) What would be the optimal time for surgery?

If I was relatively certain that the muscle was completely transected, I would allow 1-2 weeks for resolution of edema and hematoma. If I was not certain as suggested above based on saccades and active force generation, I would wait 5-6 weeks for maximum recovery and restoration of anatomy. Then reassess motility and plan primary repair as done here or alternative incommitance surgery. This approach has been effective for me in these cases.

(6) If the muscle had been transected and a large segment were missing or destroyed, what would be your surgical approach?

It is still reasonable to attempt a primary re-anastomosis posteriorly using the combined endoscopic/ anterior approach. If the reconnected muscle is too tight, then the medial rectus can be recessed at the sclera using an adjustable suture for best alignment in primary position. As with complete 3rd nerve palsy, if the range of motion of the MR does not allow useful fusion, then fogging of the effected eye may be required. Transposition procedures of vertical muscles to the MR insertion are not powerful enough unlike for lateral rectus paralysis and Botox is only temporary. Alternatively, the MR stump can be fixed to the nasal bone with nonabsorbable suture or the new LR.