Pediatric oculomotor nerve palsies are rare disorders and differ from adult oculomotor nerve palsies, in which the leading causes are aneurysm and microvascular diseases. In childhood, congenital, trauma, or neoplasm are the more common causes of cranial nerve III palsy. In addition, amblyopia is common and difficult to treat successfully. The overall visual prognosis for children with oculomotor nerve palsy is worse than those with other oculomotor nerve dysfunctions. The choice of surgical procedure for the treatment of cranial nerve III palsy depends on the specific nature and the severity of the paresis or palsy. Many procedures have been advocated for depending on the remaining function of the four extraocular muscles supplied by cranial nerve III. In complete palsy, the surgeon is limited to using only the superior oblique and/or lateral rectus muscles. These muscles are operated on in various ways to reposition the eye around primary position. The operation described in this article moves the static abducted eye into a static primary positon.
A 15-year-old girl diagnosed as having left congenital complete cranial nerve III palsy presented with best corrected visual acuity of 20/20 in the right eye and 20/100 in the left eye, binocular suppression of the left eye, normal anterior and posterior segment examinations, left eye fixed, and mydriatic pupil with hypoaccommodation and complete ptosis. The patient had 50 prism diopters (PD) of left exotropia, a -6 adduction deficit, a -3 elevation deficit, and a -4 depression deficit. She had undergone multiple (5) previous procedures, including bilateral lateral rectus recession, bilateral medial rectus resection, bilateral lateral rectus re-recession, another left lateral rectus re-recession and suture to the orbital rim, and a sharing procedure of the left superior and inferior rectus muscles to the left medial rectus insertion. Her medical history consisted of scoliosis, myopia in both eyes, and strabismic amblyopia in the left eye. A recent orbital magnetic resonance imaging scan showed severe atrophy of the medial, lateral, and inferior rectus muscles.
Under general anesthesia, a forced duction test showed a severe restriction to adduction with contraction and restriction from scar tissue laterally.
Stage 1. A lateral fornix conjunctival incision was made. The inferotemporal quadrant was explored and the inferior oblique muscle was isolated. A single-armed 6-0 polypropylene suture was placed in locked fashion across the muscle near its insertion site. The muscle was then cut anterior to the suture line and the sutures were temporarily taped to the drape of the inferior orbit with one ¼-inch Steri-Strip (3M Co., Maplewood, MN) (Figure 1A).
The operational stages of the surgical procedure. (A) The suture (arrow) isolating the inferior oblique muscle lateral to the inferior rectus muscle. (B) Injection of botulinum toxin (arrow) into the lateral rectus muscle. (C) Isolation of, and suture through, the superior oblique tendon medial to the superior rectus muscle (arrow). (D) A hemostat (arrow) grabbing the inferior oblique muscle by the previously placed suture and bringing it to the inferonasal quadrant of the eye under the inferior rectus muscle. (E) The joined inferior oblique muscle and superior oblique tendon (arrow) at the insertion of the medial rectus muscle. (F) The bolsters (arrow) securing two 5-0 polyglactin 910 sutures through the skin, periorbita, and episclera of the inferior and superior medial orbit and eye.
Stage 2. The lateral rectus muscle was isolated and found 24 to 26 mm posterior to the limbus and extremely taut. Five to seven units of botulinum toxin were injected into the lateral rectus muscle (Figure 1B).
Stage 3. A superonasal fornix conjunctival incision was made and the superior oblique tendon was isolated. A single-armed 6-0 polypropylene suture was then placed through the middle of the tendon in a multiple locked fashion and the tendon anterior to the suture was released from underlying scar tissue and the overlying superior rectus muscle (Figure 1C).
Stage 4. An inferonasal fornix incision was made and a hemostat was placed from the inferonasal wound to the inferotemporal wound underneath the inferior rectus muscle, where it grabbed the inferior oblique muscle via its previously placed polypropylene suture. The inferior oblique muscle was then brought under the inferior rectus muscle and into the medial quadrant (Figure 1D). The needles were then brought through the episclera at the inferior half of the medial rectus insertion. The superior oblique muscle was then brought inferiorly to the medial rectus insertion area and sutured through the episclera at the superior half of the medial rectus insertion. Both the transposed superior and inferior oblique muscles were then further sutured together across the insertional width of the medial rectus muscle (Figure 1E).
Stage 5. Two double-armed 5-0 polyglactin 910 sutures were placed through the episcleral and scleral tissues at 6 mm posterior to the limbus at the 8- and 10-o'clock positions. These sutures were then passed through the orbital tissues and periorbita inferonasally and superonasally to exit through the skin of the medial eyelid, where they were passed through a 5-mm bolster (Figure 1F). These two sutures were pulled until the eye was in approximately 5 to 10 degrees of adduction and then tied over the bolster. Conjunctivoplasty was performed medially due to the bulky and redundant conjunctiva resulting from bringing the eye into adduction. The conjunctiva was then closed with multiple interrupted 6-0 polyglactin 910 sutures.
Immediately postoperatively and 6 months after surgery, the incomitant primary position of 50 PD of the left exotropia changed to a 12 PD primary positon of the exotropia with no significant change in the associated vertical incomitant deviation (Figures 2–3).
Operating room photographs showing the left eye position preoperatively and immediately postoperatively.
Eye position and horizontal version at 2 months after surgery.
Congenital cranial nerve III palsy usually presents in the newborn or early infantile period with a large angle exotropia, duction limitations, ptosis, and anisocoria (either miotic or mydriatic). The palsy can be complete or partial and often results in deep amblyopia and loss of binocular function.
The most common reported etiologies are orbital or intracranial tumors, trauma, inflammatory and microvascular diseases, complications of neurosurgery, and unknown.1–4 Other, less common etiologies include nonaneurysmal, subarachnoid hemorrhage, polycythemia, sphenoiditis, neurobrucellosis, interpeduncular fossa lipoma, metastatic pancreatic cancer, leukemia, and lymphoma.5
Both medical and surgical treatments are required. Medical treatments consist of patching, atropine drops, and glasses. Prisms and vision therapy may also be necessary. Multiple surgical procedures are usually required due to the large oculomotor responsibility of cranial nerve III. Numerous surgical techniques have been described and include injection of botulinum toxin into the lateral rectus muscle,6 horizontal recti recession and resection,7–9 horizontal recti recession and resection with vertical transposition,7,9 superior oblique weakening,9 transplantation of the lateral rectus muscle to the medial side of the globe,10 recession and vertical transposition of the inferior rectus muscle combined with medial rectus resection,10 nasal transposition of two halves of the lateral rectus muscle combined with medial rectus resection,11 augmented Hummelsheim procedure combined with lateral rectus recession,12 and modified Knap procedure and superior oblique tendon transposition above the medial rectus muscle combined with a large lateral rectus recession.13
Globe fixation procedures with different techniques have been described for the treatment of cranial nerve III palsy and are used after several previous procedures. Srivastava et al.14 described a technique that involved fixation of the globe at the medial rectus insertion to the medial palpebral ligament insertion at the anterior lacrimal crest with incorporation of the periosteum and combined with a large recession of the lateral rectus muscle. Mora15 described a fixation technique in which two intraoperatively adjustable, nonabsorbable double-armed 5-0 polyester fiber traction sutures were placed one above the other through the periorbita of the crest and passed through one superior and one inferior medial rectus insertion. Intraoperative adjustment was performed by tightening each of the sutures until the eye appeared to be in the primary position and the sutures were then secured.15 Sharma et al.16 described a fixation technique in which a large lateral rectus recession was combined with the passing of a 5-0 double-armed polyester suture on spatulated needles through the periosteum overlying the anterior lacrimal crest at its superior part, and then the needles were brought anterior to the medial rectus insertion and tightened enough to align the eye in the 8 to 10 PD adducted position. Velez et al.17 described a technique in which the lateral rectus muscle was disinserted and attached to the adjacent orbital periosteum with two periosteal bites using pre-placed nonabsorbable sutures, combined with medial rectus resection and superior oblique tenotomy.
Salazar-León et al.18 described a technique of ocular fixation using fascia lata combined with a large lateral rectus recession. The ocular fixation was accomplished by suturing one end of the fascia lata to the nasal bone periosteum and the other end was passed under the conjunctiva to the medial rectus insertion. After rotating the globe to a straight position, the fascia lata was sutured and tightened with 6-0 monofilament sutures to the medial rectus insertion.18
Another fixation technique described a large lateral rectus recession and medial rectus resection in conjunction with traction sutures that were passed through the insertion of the superior and inferior rectus muscles right next to the sclera and taken through the upper and lower fornices to the medial canthus, where they were brought to the surface through the eyelid skin by means of a large half-circle cutting-edge eyed needle. The traction sutures were then pulled up to bring the eye into adduction and were tied over plastic bolsters. Traction sutures were left in situ for 6 weeks.1,19 Villaseñor Solares et al.20 described a technique in which a superior oblique tenectomy was performed and the superior oblique tendon fragment was used for ocular fixation to the nasal periosteum. The tendon was sutured to the medial rectus insertion site. Saxena et al.21 described a fixation technique in which a large lateral rectus recession was performed along with a precaruncular anchoring procedure. The anchoring procedure was performed by passing nonabsorbable sutures through the periosteum posterior to the posterior lacrimal crest and then sutures were passed on the sclera on either end of the medial rectus muscle.21
Our addition to this large list of procedures consisted of three parts: botulinum toxin injection to the lateral rectus muscle, transposition of the superior and inferior oblique muscles nasally to the medial rectus insertion, and globe fixation to the eyelids through the periorbita and eyelids. The surgical result was satisfactory. The success of this procedure may have been due to the additional constructed adduction forces from the multiple previous procedures. This new globe fixation procedure may be added to the surgical armamentarium promoting ocular alignment due to the disfiguring effects of cranial nerve III palsy.
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- Schumacher-Feero LA, Yoo KW, Solari FM, Biglan AW. Third cranial nerve palsy in children. Am J Ophthalmol. 1999;128:216–221. doi:10.1016/S0002-9394(99)00128-2 [CrossRef]
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- Bagheri A, Borhani M, Tavakoli M, Salehirad S. Clinical features and outcomes of strabismus treatment in third cranial nerve palsy during a 10-year period. J Ophthalmic Vis Res. 2014;9:343–349.
- Sadagopan KA, Wasserman BN. Managing the patient with oculomotor nerve palsy. Curr Opin Ophthalmol. 2013;24:438–447. doi:10.1097/ICU.0b013e3283645a9b [CrossRef]
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- Köse S, Uretmen O, Pamukçu K. An approach to the surgical management of total oculomotor nerve palsy. Strabismus. 2001;9:1–8. doi:10.1076/stra.220.127.116.112 [CrossRef]
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- Lee V, Bentley CR, Lee JP. Strabismus surgery in congenital third nerve palsy. Strabismus. 2001;9:91–99. doi:10.1076/stra.18.104.22.1689 [CrossRef]
- Taylor JN. Surgical management of oculomotor nerve palsy with lateral rectus transplantation to the medial side of globe. Aust N Z J Ophthalmol. 1989;17:27–31. doi:10.1111/j.1442-9071.1989.tb00486.x [CrossRef]
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- Metz HS, Yee D. Third nerve palsy: superior oblique transposition surgery. Ann Ophthalmol. 1973;5:215–218.
- Srivastava KK, Sundaresh K, Vijayalakshmi P. A new surgical technique for ocular fixation in congenital third nerve palsy. J AAPOS. 2004;8:371–377. doi:10.1016/j.jaapos.2004.01.015 [CrossRef]
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- Saxena R, Sinha A, Sharma P, Phuljhele S, Menon V. Precaruncular approach for medial orbital wall periosteal anchoring of the globe in oculomotor nerve palsy. J AAPOS. 2009;13:578–582. doi:10.1016/j.jaapos.2009.11.003 [CrossRef]