Original Investigation

Blepharoptosis Associated With Third Cranial Nerve Palsy Abbas Bagheri, M.D., Morteza Borhani, M.D., Shahram Salehirad, M.D., Shahin Yazdani, M.D., and Mehdi Tavakoli, M.D., F.I.C.O. Ophthalmic Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran

Purpose: To evaluate the demographics and outcomes of management for blepharoptosis associated with third cranial nerve palsy at a tertiary eye center. Methods: This retrospective cross-sectional and interventional study was performed on hospital records of patients with ptosis associated with third cranial nerve palsy who were referred to Labbafinejad Medical Center from January 1999 to January 2009. The authors evaluated age, sex, laterality, severity of involvement, etiology, clinical findings, and treatment modalities and outcomes in patients with blepharoptosis due to third cranial nerve palsy over a 10-year period. Results: Of a total of 45 subjects, 25 cases (55.6%) were males and 20 subjects (44.4%) were females. Mean age of enrolled subjects was 21.1 ± 15.5 years. Etiologies included trauma in 21 (46.7%), congenital in 16 (35.6%), and other causes in 8 (17.7%) cases. Overall, 18 of 45 patients (40%) underwent ptosis surgery: of these, 15 (83.3%) subjects improved with one procedure, whereas 3 (16.7%) subjects required more than one operation. For initial ptosis surgery, 5 (27.7%) patients underwent levator resection and 13 (72.3%) cases underwent frontalis sling; all second and third procedures were frontalis sling. One-step, 2-step, and 3-step ptosis surgery was accomplished in 15 (83.3%), 1 (5.6%), and 2 (11.1%) patients, respectively. Eventually, an open visual axis was attained in all cases following 1–3 operations. Conclusions: Trauma was the most common cause of ptosis associated with third cranial nerve palsy in the current series. Surgical management of ptosis in patients with third nerve palsy may be challenging but most subjects achieve good results following appropriate and stepwise surgical plans. (Ophthal Plast Reconstr Surg 2015;31:357–360)

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lephroptosis is a common consequence of third cranial nerve palsy and can be difficult to treat.1–3 The ptotic eyelid may cover the pupil, causing or exacerbating amblyopia in pediatric patients and give rise to functional and cosmetic concerns in adults; on the other hand, management and repair of this condition may be complicated by exposure keratitis because of a poor Bell’s phenomenon due to dysfunction of the eye levator muscles.4 The success of surgical intervention in this condition depends on patient selection, surgical expectations, and appropriate management of complications. Because of the neurologic characteristics of oculomotor nerve palsy, not all affected Accepted for publication April 16, 2014. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Mehdi Tavakoli, M.D., F.I.C.O., Ophthalmic Research Center, Shahid Beheshti University of Medical Sciences, Labbafinejad Medical Center, 23, Paydarifard Street, Pasdaran Avenue, Tehran 16666, Iran. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000336

Ophthal Plast Reconstr Surg, Vol. 31, No. 5, 2015

subjects may benefit from surgery and the selection of suitable candidates is restricted to carefully chosen cases whose functional and cosmetic disability may improve from surgical intervention.3 The presence of amblyopia, visual deficits, involvement of other cranial nerves (particularly the trigeminal and facial nerves), alternation of paretic and spastic function in cyclic oculomotor palsy, and recovery with or without aberrant regeneration further affect patient selection and outcomes of treatment.3 The current study was performed to determine demographic characteristics and therapeutic results of blepharoptosis in patients with third cranial nerve palsy referred to the authors’ tertiary eye center.

METHODS This retrospective study was performed on hospital records of patients with ptosis associated with third cranial nerve palsy who were referred to Labbafinejad Medical Center from January 1999 to January 2009. The study was approved by the Review Board and ethics Committee of Ophthalmic Research Center affiliated to Shahid Beheshti University of Medical Sciences. In addition, the research adhered to the tenets of the Declaration of Helsinki. Variables included gender, age at onset of the palsy and age at presentation, length of follow up, and the etiology of the palsy; the authors also noted whether the palsy was complete or partial and if signs of aberrant regeneration were present. Appropriate systemic evaluations, including fasting plasma glucose levels, blood pressure measurement, lipid profile, thyroid function tests, and general laboratory tests, including erythrocyte sedimentation rate to rule out giant cell arteritis, had been performed for acquired cases based on clinical findings. Neuroimaging had been performed in all acquired cases. Subjects with acquired nontraumatic pupil-sparing palsy and normal neuroimaging were also evaluated for myasthenia gravis by Tensilon test and electromyography. The authors classified the ptosis in unilateral cases into 3 groups based on the difference between palpebral fissure heights of 2 eyes: mild (less than 2 mm), moderate (between 2 and 4 mm), and severe (more than 4 mm).

TABLE 1.  Classification of third cranial nerve palsy and accompanying secondary diagnoses Type of 3rd nerve palsy Isolated Nonisolated

Accompanying conditions — 4th nerve palsy 6th nerve palsy 7th nerve palsy Neurofibromatosis type 1 Brown syndrome Benedikt syndrome

Total

Frequency (%) 33 (73.3) 12 (26.7) 5 (11.1) 3 (6.7) 1 (2.2) 1 (2.2) 1 (2.2) 1 (2.2) 45 (100)

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FIG. 1.  Changes in eyelid parameters following initial ptosis surgery. MRD 1, upper eyelid margin reflex distance-1; PFH, palpebral fissure height. In bilateral cases, blepharoptosis was categorized based on palpebral fissure height: mild (more than 8 mm), moderate (between 4 and 8 mm), and severe (less than 4 mm). Levator muscle function was classified into 3 groups: good (more than 8 mm of excursion), fair (between 4 and 8 mm of excursion), and weak (less than 4 mm of excursion). Surgical outcomes were defined according to inter-eye asymmetry and were also divided into 3 categories: optimal (asymmetry less than 1 mm), suboptimal (asymmetry between 1 and 2 mm), and poor (asymmetry more than 2 mm). Patients with recent onset palsy (less than 12 months) received conservative treatment such as eyelid crutches; surgery was considered if the palsy was stable for at least 12 months. In subjects with fair to good levator muscle function, the authors performed levator resection, and in cases with fair to poor levator muscle function, the authors performed the frontalis sling procedure. All surgical results data gathered 6 months following each operation were analyzed for the purpose of the study.

RESULTS This study included 45 patients, consisting of 25 (55.6%) male and 20 (44.4%) female subjects with mean age of 21.1 ± 15.5 years (range10 months to 60 years). Mean follow-up duration was

TABLE 2.  Results of initial ptosis surgery on 18 patients according to type of operation Operation

Optimal

Suboptimal

Poor

Total

Levator resection Frontalis sling Total

3 (60%) 2 (15.4%) 5

2 (40%) 3 (23.1%) 5

0 8 (61.5%) 8

5 13

P = 0.006 based on Mann-Whitney test.

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27.3 months (range 6 months to 10 years). All cases of oculomotor palsy were unilateral; the right eye was involved in 21 cases (46.7%) and the left eye was involved in 24 (51.3%) subjects. The condition was congenital in 16 (35.6%) and acquired in 29 (64.4%) patients and causes included trauma in 21 (46.7%), tumors in 2 (4.4%), ischemia in 2 (4.4%), viral meningitis in 1 (2.2%), and unknown in 3 (6.7%) cases. The authors observed no case of multiple sclerosis, myasthenia gravis, or cyclic oculomotor palsy in this series. The third cranial nerve palsy was isolated in 33 (73.3%) subjects and accompanied by other cranial nerve palsies or neurologic conditions in 12 (26.7%) patients (Table 1). The palsy was complete in 22 cases (48.9%) and incomplete in 23 cases (51.1%), including 2 patients with isolated superior divisional involvement. Aberrant regeneration was present in 6 cases (13.3%) and 2 patients had associated jaw winking. Ptosis was mild in 5 (11.1%), moderate in 11 (24.4%), and severe in 29 (64.5%) cases. Mean levator muscle function was 7 ± 6 mm (range 0–17 mm) and was classified as good in 13 (28.8%), fair in 5 (11.2%), and weak in 27 (60%) cases. Mean palpebral fissure height was 5 ± 4 (range 0–10) mm and mean marginal reflex distance-1 was –1 ± 4 (range –4 to +4) mm. In 8 patients (17.7%), eyelid crutches were prescribed because of impaired Bell’s reflex. Two (4.4%) patients had history of frontalis sling procedure some years ago. During follow up, 18 of 45 patients (40%) underwent ptosis surgery. Among operated patients, levator muscle function was good in 4 (22.2%), fair in 3 (16.7%), and weak in 11 (61.6%) cases. One-step, 2-step, and 3-step ptosis surgery was accomplished in 15 (83.3%), 1 (5.6%), and 2 (11.1%) patients, respectively. As initial ptosis surgery, 5 (27.7%) patients underwent levator resection, whereas 13 (72.3%) subjects underwent a frontalis sling procedure (including sling with silicone rod, supramid or fascia lata). In patients who underwent surgery, mean preoperative palpebral fissure height was 4 ± 3 (range 0–8) mm, which was increased to 7 ± 3 (range 0–12) mm, and mean marginal

© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.

Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.

Ophthal Plast Reconstr Surg, Vol. 31, No. 5, 2015

Blepharoptosis in Third Nerve Palsy

reflex distance-1 was increased from –3 ± 3 (range –4 to +2) mm to 1 ± 3 (range –4 to +5) mm following the first operation (Fig. 1). Outcomes of initial ptosis surgery on 18 patients were optimal in 5 (27.8%), suboptimal in 5 (27.8%), and poor in 8 (44.4%) cases. In patients who underwent levator resection, the results were optimal in 3 (60%) and suboptimal in 2 (40%); poor results were not observed in this group. In patients who underwent frontalis sling, the results were optimal in 2 (15.4%), suboptimal in 3 (23.1%), and poor in 8 (61.5%) patients (Table 2). Outcomes of surgery in 3 patients who needed more than one operation are detailed in Table 3. All second and third ptosis operations were frontalis sling procedures (including sling with silicone rod and fascia lata). The second operation gave rise to optimal results in 1 and poor results in 2 cases. Outcomes of the third operation were suboptimal in one and poor in another patient. The visual axis was open before surgery in 2 (40%) patients in the levator resection group, whereas all 5 (100%) cases had an open visual axis after the first operation. In the frontalis sling group, the visual axis was open in only 1 (7.7%) case before surgery increasing to 6 (46.2%), 9 (69%), and 13 (100%) cases following the first, second, and third operations, respectively. In the frontalis sling group, patients with occluded visual axes required a mean number of 1.9 ± 0.9 operations to reach open axes but in the levator resection group, patients achieved open visual axes with 1 corrective operation (Figs. 2 and 3). The most common complications following initial operation were lagophthalmos in both groups and undercorrection in the frontalis sling group (Table 4). Other complications, especially exposure keratitis, were not encountered in this series.

DISCUSSION Acquired third cranial nerve palsy represents approximately 30% of ocular motor cranial nerve palsies being less common than acquired sixth nerve palsy and more common than acquired fourth nerve palsy.5,6 In large retrospective series of third nerve palsies with defined causes, microvascular ischemia was among the most common etiologies representing 17% to 35% of cases.5–7 Aneurysmal and neoplastic etiologies were approximately equal (up to 19%), and a large percentage of patients in each series had undetermined etiology.8,9 Trauma was the most common etiology of third cranial nerve palsy in this study. This large portion of traumatic cases could be related to the fact that the authors’ center is a tertiary eye center and a large number of vascular cases with spontaneous resolution may not be referred to us. The list of common etiologies of third nerve palsy varies in different reports. This variety may be attributable to different study settings and inclusion criteria.4,10–13Congenital third cranial nerve palsy has been reported as the most common cause in several studies, especially in pediatric series.4,10Vascular causes followed by trauma were the most common etiologies in acquired third nerve palsy reported by Park et al.11

FIG. 2.  A 16-year-old boy with traumatic third nerve palsy before (A) and after (B) one step levator resection surgery and also strabismus operation.

Blepharoptosis is a common finding in patients with third cranial nerve palsy.2 In the current series, ptosis surgery in patients who initially underwent levator resection had acceptable results in almost all cases; however, in patients who underwent the frontalis sling procedure, outcomes of the first operation were poor in 61.5%, which may be due to more

TABLE 3.  Surgical outcomes in 3 patients requiring more than 1 surgery MRD1 (PFH)*

Patient 1 Patient 2 Patient 3

Preoperative

Postop 1

Postop 2

Postop 3

–2 (5) 0 (5) –4 (2)

0 (6) 1 (5) 0 (5)

4 (12) 1 (5) 1 (5)

— 2 (6) 3 (8)

*Values are in millimeters. MRD1, marginal reflex distance-1; PFH, palpebral fissure height; Postop, postoperative.

FIG. 3.  A 52-year-old man with congenital third nerve palsy before (A) and after first (B) and second (C) frontalis sling operations.

© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.

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TABLE 4.  Complications of ptosis surgery according to type of operation Ptosis surgery Levator resection Frontalis sling Total p* *

Overcorrection

Undercorrection

Lagophthalmos

Total

0 0 0 1

2 (40%) 11 (84.6%) 13 0.1

2 (40%) 2 (15.4%) 4 0.5

4 13 18

Based on Wilcoxon test.

severe palsy in this group. The visual axis was open (marginal reflex distance-1 ≥ 1 mm) in 40% of patients preoperatively in the levator resection group, which increased to 100% after one surgery, but in the frontalis sling group, 7.7% of patients had open visual axis preoperatively, which increased to 46.2% postoperatively; only following 3 operations, this figure reached 100%. Schumacher-Feero et al.4 reported surgical results in 23 children (34 eyes) with ptosis due to congenital third nerve palsy treated with levator resection or frontalis sling. After a mean of 1.4 procedures, 17 of 18 eyes with partial palsy and 14 of 16 eyes with complete palsy had good final eyelid position. In another study by Malone and Nerad3 on 170 patients with oculomotor palsy, 20 patients underwent sling procedure or levator advancement for treatment of blepharoptosis. Functional and cosmetic disabilities were relieved in 83% and 100% of cases, respectively. Undercorrection was the most common complication of ptosis surgery in this series with a frequency of 40% in the levator resection group and 85% in the frontalis sling group, which may be attributable to more severe ptosis and levator muscle dysfunction in the latter group. Undercorrection and need for reoperation is a common complication of frontalis suspension procedures using various materials. Recurrence rates of 8 to 53% have been reported in patients undergoing frontalis sling with autogenous or banked fascia lata in different series.14–17 Such results have also been described with synthetic materials.16–18 In conclusion, in the current series, most patients had traumatic etiology followed by congenital causes. A sizable portion of the patients required more than 1 surgery. Ptosis correction in such patients is challenging and a considerable number of patients need multiple interventions. Although the surgical treatment of ptosis due to third cranial nerve palsy is difficult, an appropriate approach and multistep surgical plan might achieve acceptable results in most patients.

REFERENCES 1. Krohel GB. Blepharoptosis after traumatic third-nerve palsies. Am J Ophthalmol 1979;88(3 Pt 2):598–601.

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© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.

Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.

Blepharoptosis Associated With Third Cranial Nerve Palsy.

To evaluate the demographics and outcomes of management for blepharoptosis associated with third cranial nerve palsy at a tertiary eye center...
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