Transactions 29th European Strabismological Association Meeting – de Faber (ed) © 2005 European Strabismological Association, ISBN 04 1537 211 9

Globe retraction in a patient with nanophthalmos

S. Öner, A.F. Nohutçu & H.N. Ortak

Haydarpasa Numune Hospital for Education and Research, Istanbul, Turkey

ABSTRACT: We present a 14 year-old female patient with bilateral nanophthalmos and globe retraction with narrowing of the palpebral fissure on adduction of the left eye. There was ortotropia in primary position, and no restriction of ocular motility. Magnetic resonance imaging showed bilateral normal nervi abducens. One brother of the patient had bilateral nanophthalmos without ocular motility disorder. There is no report of nanophthalmos associated with globe retraction in the literature. Globe retraction on adduction is a typical component of Duane Syndrome Type I, which has not been reported in association with nanophthalmos. We suggest that the phenomenon of globe retraction in this case may be related to mechanical factors rather than innervational abnormalities.

1INTRODUCTION

The characteristic features of nanophthalmos are narrow palpebral fissures in combination with a deep set globe in a small orbit, hypermetropia, short axial length, reduced volume of the globe with normal lens volume, and normal or reduced corneal diameter. Complications of this disorder are angle closure glaucoma, uveal effusion, choroidal detachment, and non-rhegmatogenous retinal detachment (Singh et al. 1994). Associated strabismus, in most cases nonaccomodative esotropia, and with lower incidence microesotropia and exotropia, can be observed (Sener et al. 2003). Globe retraction and narrowing of the palpebral fissure on adduction are typical components of the Duane retraction syndrome (DRS). Numerous systemic and ocular disorders have been reported in association with DRS (DeRespinis et al. 1993). We report a case of bilateral nanophthalmos with globe retraction and narrowing of the palpebral fissure of the left eye on attempted adduction. To our best knowledge, a similar case has not been described yet.

2CASE REPORT

The 14 year-old girl presented in our clinic in August 2003 for spectacle renewal. Anamnesis revealed no problem during pregnancy; normal delivery, and no history of disease. She had bilateral narrow palpebral fissures and deeply set globes (Fig. 1).

Best corrected visual acuity (BCVA) was 0.4 ( 13.0) OD, and 0.5 ( 11.0) OS. She has been wearing spectacles since the age of 1. On biomicroscopic examination, horizontal corneal diameter

Figure 1. Photographs demonstrate bilateral deep set globes with narrow palpebral fissures, and retraction of the left globe with narrowing of the palpebral fissure on right gaze.

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Table 1. Orbital B-scan ultrasonography values.

of bilateral 11.0 mm, and thick irises with a convexity towards the cornea were observed. Gonioscopy revealed bilateral Shaffer grade II anterior chamber angle, periferal anterior synechiae and prominent iris convexity. Intraocular pressure was 11 mmHg in both eyes. Visual field testing showed bilateral arcuate scotoma, more extensive in the right eye. On fundoscopy bilateral pseudopapiledema and macular folds were observed. Orbital B-scan ultrasonography values are shown in Table 1.

Magnetic resonance imaging of the brain stem showed bilateral normal nervi abducens. There was ortotropia in primary position and no restriction of ductions and versions. Diplopia, head turn or nystagmus were not observed. On attempted adduction of the left eye there was globe retraction and narrowing of the palpebral fissure (Fig. 1).

Nanophthalmos was present in 2 of 3 children of this family. The oldest child, a girl, was reported as being healthy and having no ocular problems, the youngest child, an otherwise healthy 11 year-old boy without a history of abnormalities during pregnancy or birth, had bilateral nanophthalmos without ocular motility disorder. Mother and father are secondary degree relatives. Ocular problems have not been reported in any other member of the family.

3DISCUSSION

Our case showed features of typical nanophthalmos, and globe retraction with narrowing of the palpebral fissure of the left eye on attempted right gaze. Globe retraction and narrowing of the palpebral fissure on adduction are typical components of DRS, and have been related to the paradoxical innervation of the lateral rectus muscle, leading to co-contraction of the horizontal rectus muscles. DRS is more frequently found on the left eye and in females (DeRespinis et al. 1993). Magnetic resonance imaging can show abscence of the nervus abducens (Ozkurt et al. 2003). Gross et al. (Gross et al. 1994) report a case of DRS type II with synchroneous innervation of the ipsilateral horizontal rectus muscles without globe retraction. They propose that in DRS both innervational and mechanical factors are involved. In our case, we propose a mechanical factor for the globe retraction, because neither strabismus, nor restriction of ocular motility was present.

REFERENCES

DeRespinis, P.A., Caputo, A.R., Wagner, R.S. & Guo, S. 1993. Duane’s Retraction syndrome. Surv Ophthalmol 38(3): 257–288.

Gross, S.A., Tien, D.R. & Breinin, G.M. 1994. Aberrant innervational pattern in Duane’s syndrome type II without globe retraction. Am J Ophthalmol 117(3): 348–351.

Ozkurt, H., Basak, M., Oral, Y. & Ozkurt, Y. 2003. Magnetic resonance imaging in Duane’s retractionsyndrome. J Pediatr Ophthalmol Strabismus 40(1): 19–22.

Sener, E.C., Mocan, M.C., Sarac, O.I., Gedik, S. & Sanac, A.S. 2003. Management of strabismus in nanophthalmic patients. Ophthalmology 110(6): 1230–1236.

Singh, O.S. & Sofinski, S.J. 1994. Nanophthalmos: Guidelines for diagnosis and therapy. In: D.M. Albert & F.A. Jacobiec (eds). Principles and Practice of Ophthalmology: 1528–1540. Philadephia: W.B. Saunders.

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Transactions 29th European Strabismological Association Meeting – de Faber (ed) © 2005 European Strabismological Association, ISBN 04 1537 211 9

Surgical treatment of consecutive exotropia

Aslıhan Öztürk, Serpil Akar, Birsen Gökyig˘it, Ömer Faruk Öge, Zerrin Bayraktar Ömer Faruk Yılmaz

Beyog˘lu Eye Research and Education Hospital

ABSTRACT:

Introduction: Consecutive exotropia (CXT) is a well-known phenomenon found after various surgeries for esotropia (ET). We investigated the characteristics of these patients and the results of the surgery for CXT in this study.

Materials and methods: We reviewed the files of patients who underwent surgery for CXT between 1995–2002 at Beyog˘lu Eye Research and Education Hospital. We used Wilcoxon signed Ranks tests as statistical method for evaluation.

Results: There was 28 patients who underwent surgery for CXT. Mean age was 18,21 17,07 (range 3–73) years. The average preoperative deviation was (29,29 13,35) prism diopters (pd) (range 10–58 pd XT ) at near fixation and (25,39 15,7) pd (range 0–58 pd XT) at distance fixation. The postoperative average deviation was ( 1,18 7,11) pd (range 15 ET–18 pd XT) at near fixation and ( 0,96 7,5) pd (range 25 ET–16 pd XT) at distance fixation. 3 patients had 10 pd of exotropia while 1 had 10 pd of esotropia. One surgery had enough at 24 (85,7%) patients 4 (14,2%) patients required 2 surgeries for CXT. Mean follow up was 9,43 5,6 (range 6–28) months. There are statistically significant difference between preoperative and postoperative near deviations and between preoperative and postoperative distance deviations (p 0.001, p 0.001).

Conclusions: Good alignment can be achieved after surgery for CXT.

1INTRODUCTION

Consecutive exotropia (CXT) is a frequent problem found after varies surgeries for esotropia (ET). Factors of development of CXT in reported series are excessive amount of surgery, amblyopia, high hyperopia and failure to evaluate of patients preoperatively (Cooper EL. 1961, Brown R.M. 1976).

In this study, we examined the characteristics of these patients and the factors involved in the development of CXT and the results of the surgical procedures used for its treatment.

2 MATERIALS AND EXPERIMENTAL METHODS

We reviewed the medical records of patients who underwent surgery for CXT between 1995–2002 at strabismus department of Beyog˘lu Eye Research and Education Hospital.

Age onset of ET, age at surgery for ET, age CXT noted, age at time of surgery for XT, amount of XT , type of XT surgery, number of XT surgeries, presence of amblyopia, versions, amount of deviation after surgery for CXT were recorded. The surgical technique used was individually considered for each patient. Mean follow up was 9,43 5,6 (range 6–28) months. We used Wilcoxon signed Ranks tests as statistical method for evaluation.

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Table 1. Age and time.

Table 2. Preoperative findings.

Table 3. Preoperative and postoperative deviations.

3RESULTS

There was 28 patients who underwent surgery for CXT (11 female and 17 male). Mean age of onset ET was 1,63 years (range 0–6 years). 15 (53.5%) out of 28 patients were younger than 1 year at the onset of the ET. The children with early onset ET are less likely to have stable binocular vision and are more likely to develop CXT. (Eugene R.F. 1983).

We research the age of the patient at the time of the ET surgery and the interval between the onset of the CXT. Mean age at ETsurgery was 6,83 0,77 (range 0,5–17) years and mean age that XT noted was 14,3 2,48 (0,5–55) years. Mean age at CXT surgery was 18,21 17,07 (range 3–73) years. The time between surgery for esotropia and exotropia was between 3 days and 38 years (average 7,7 years) (Table 1).

Ambliyopia has been identified as one of the factors important in the development of CXT.19 out of 28 patients had ambliyopia and 16 out of 28 patients had A/V/X patterns. (Table 2)

Only 5 of 28 patients showed a hyperopia in excess of 2,5 diyoptres and 1 patient showed a myopia in excess of 9,5 diyoptres. The rest of the patients had a refractive error between 2,50 and 1,50.

The average preoperative deviation was (29,29 13,35) prism diopters (pd) (range 10–58 pd XT ) at near fixation and (25,39 15,7) pd (range 0–58 pd XT) at distance fixation (Table 3). One surgery had enough at 24 (85,7%) patients , 4 (14,2%) patients required 2 surgeries for CXT.

We performed Medial rectus (MR) advancement in 9 patients (MR advancement with infra position in 1 patient), MR advancement LR recession in 13 patients (MR advancement with supra position LR recession in 1 patient), MR resection MR advancement in 2 patients, MR advancement LR myotomi at inferior side in 1 patient, MR advancement bilateral IO recession in 1 patient, MR advancement bilateral LR recession in 2 patients at the first operation. (Table 4)

The postoperative average deviation was ( 1,18 7,11) pd (range 15 ET–18 pd XT) at near fixation and ( 0,96 7,5) pd (range 25 ET–16 XT) at distance fixation. There are statistically significant difference between preoperative and postoperative near deviations and between preoperative and postoperative distance deviations (p 0.001, p 0.001), 3 patients had 10 prism diopters of exotropia while 1 had 10 PD of esotropia.

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4DISCUSSION

Consecutive XT is not infrequently confronted after surgery for ET. The incidence in reported series of CXT ranged from 4% to 20% (Bietti GB 1965, Dunnington JH 1950). The incidence was found 2,4% in this study.

The time of onset of CXT varies greatly but there has been a noted progression towards exotropic drift in long term follow up of patients in previous studies (Caputo AR 1990). This study indicate that most of the deviations occurred in the immediate postoperative period. But CXT may develop many years later after ET surgery. Therefore, the success of surgery for ET can not be evaluated within a short follow up period.

A high degree of hyperopia has been thought to be responsible producing CXT in previous studies (Cooper EL 1961, Brown RM 1976). Our study did not prove this. In our study, only 5 (17%) patient showed a hyperopia in excess of 2,5 dioptres.

It is important to properly evaluate the presence of amblyopia and A/V/X patterns (Eugene RF 1983, Bradbury JA 1993). The presence of amblyopia and A/V/X patterns were found to be the most common factors in the development of CXT in our study. These findings may help in the planning of the most appropriate surgery for CXT. Amblyopia must be fully corrected. After surgery patient should continue with amblyopia therapy.

5CONCLUSION

The final deviation was noted to be closer to orthophoria in our study. Good alignment can be achieved after surgery for CXT.

REFERENCES

1.Cooper EL. The surgical management of secondary exotropia. Trans Am Acad Ophthalmol Otolaryngol, 1961; 65: 595–608.

2.Brown RM, Cooper BM. An assesment of the rule of secondary and consecutive factors in overcorrected esotropia, orthoptics past, present, future. New York: Stratton Intercontinental Medical Book Corporation, 1976: 515–22.

3.Eugene RF. Consecutive exotropia following surgery. British Journal of Ophthalmology, 1983; 67: 546–548.

4.Bradbury JA, et al. Secondary exotropia: a retrospective analysis of matched cases. Journal of Pediatric Ophthalmology and Strabismus, 1993; 30(3): 163–166.

5.Caputo AR, et al. Preferred postoperative alignment after congenital esotropia surgery. Annals of Ophthalmology, 1990; 22(7): 269–272.

6.Bietti GB, Bagloni B. Problems related to surgical overcorrections in strabismus surgery. J Pediatr Ophthalmol, 1965; 2: 11–14.

7.Dunnington JH, Reagan EF. Factors influencing the postoperative result in concomitant convergent strabismus. Arch Ophthalmol, 1950; 33: 380–7.

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