Med. J. Cairo Univ., Vol. 78, No. 1, June: 331-336, 2010 www.medicaljournalofcairouniversity.com Duane Retraction Syndrome KARIMA L. SHALABY, M.D.* and MOSTAFA BAHGAT, M.D.** The Pediatric Ophthalmology Section*, Tripoli Eye Hospital and the Department of Ophthalmology**, Faculty of Medicine, Cairo University. Abstract Electromyography studies have shown paradox- ical innervations of Lateral rectus muscle and Purpose of Study: To evaluate and to manage if manage- anomalous synergistic innervations of medial rec- ment indicated for cases of Duane retraction syndrome. tus, inferior rectus, superior rectus and oblique Patients and Methods: 15 Duane retraction syndrome muscles [7,8] . (DRS) patients seen in Pediatric clinic in Tripoli Eye Hospital in period from January 2006-December 2006. Complete In most cases of DRS the entire 6 th nerve atro- ophthalmic examination including ortho-optic assessment for phy instead of post half of 6 th nerve (without all cases. specific teratogenic stimulus) 95% of DRS cases Results: Patients age ranged 1 year to 20 years in this this is the only initial abnormality. In about 5% of group of study, females were affected more than males with cases other abnormalities seen (e.g. nerve deafness). 2 to 1 ratio. Type 1 (esotropic) is the most common 80% of cases. Left eye was affected more than right eye. Bilateral in Most cases of DRS are sporadic [5,9] . 13.3% of cases. DRS clinical picture varies widely, surgical intervention will not eliminate the abnormality but will lessen Etiology: it. Two cases were operated upon to improve alignment in Etiology of DRS has been proposed by several primary position. Medial rectus muscle was found too tight on forced-duction test surgery included bilateral medial rectus investigators to be fibrosis of lateral rectus (LR)- muscle recession. deficient abduction and retraction of the globe on adduction, due to the pull of medial rectus (MR) Conclusion: DRS picture varies widely, no treatment is available to eliminate the abnormalities but only to lessen against the tight LR [7] . them. The limitations in treatment are due to poor correction of ductions and versions. Also, up shoot, down shoot and Abnormal innervation of LR-co-correction of enophthalmos correction is not complete. LR and MR on adduction-retraction [10] . Key Words: Duane syndrome – Retraction – Upshoot and downshoot – Electromyography – Teratogenic – Co-contraction of vertical recti on adduction- Esotropia – Exotropia – Orthoptic. retraction of the globe. Introduction Protrusion of globe on attempted abduction, due to co-contraction of oblique muscles [11] . DUANE Retraction Syndrome (DRS) is an abnor- mal pattern of ocular motility characterized by So, the etiology of DRS may be a mixture of retraction of globe with narrowing of palpebral anatomical and neurological anomalies. Therefore, fissure on attempted adduction and a variety of Fibrosis of LR may be secondary to abnormal other abnormal movement of the affected eye when innervation. Electromyographic studies show co- the other eye fixates in various cardinal positions contraction of horizontal recti, also show co- [1,2,3] . contraction of vertical recti on adduction [13] . The abnormal pattern of ocular motility of DRS Classification is the result of developmental adaptation that occurs in the embryo as consequence of the absence of HUBER Classification: 6th cranial nerve [4,5,6] . • Type (1): o Esotropia in PP, restrict abduction. Correspondence to: Dr. Mostafa Bahgat, Ophthalmology Dept, Faculty of Medicine, Cairo University, Cairo, Egypt. o Most common type. 331 332 Duane Retraction Syndrome • Type (2): 2- Ocular examination: o Poor adduction and exotropia. a- Vision and refraction under cycloplegia to reach best corrected vision. • Type (3): b- Ortho-optic status. o Limited adduction and abduction [1,3,4,7,8] . c- Ocular motility. Associated developmental abnormalities: d- Anterior segment. Ocular, auditory, CNS, skeletal, genitourinary e- Fundus examination. and skin abnormalities [12] . f- Systemic examination for any associated Diagnosis of Duane’s retraction syndrome: anomaly. By testing ocular motility: Results Positive history of deviation, abnormal motility in all patients. No history of similar problem in the family Primary position their families. DRS was seen more in female patients in this group of study 66.7% females, 33.3% males (Fig. 1). Male 33.3% Gaze to the right Gaze to the left Female Differential diagnosis of Duane ’s retraction syn- 66.7% drome: Fig. (1): Gender distribution in DRS cases in Pediatric Clinic 1- Abducent nerve palsy: There is no retraction of 2006. the globe on attempted adduction. No widening of palpebral fissure nor protrusion of the globe DRS distribution according to type: (Fig. 2) on attempted abduction. • Type (1) 80%. 2- Pseudo-Duane: Due to fracture of the medial • Type (2) 13%. orbital wall-entrapment of medial rectus muscle. • Type (3) 7%. CT scan will reveal the problem [12,13] . 100 Aim of study: 80 To evaluate the clinical findings in DRS cases 80 seen in year 2006 in Pediatric Clinic in Tripoli Eye hospital and to manage cases if management indi- 60 cated. Percent 40 Patients and Methods 20 13 This study included 15 patients in period (Jan- 7 uary 2006-December 2006) in Tripoli Eye Hospital 0 (Pediatric Clinic). Age range from 1 year to 20 1 2 3 years. All cases were subjected to the following: Type 1- History: About onset, face turn, progression and Fig. (2): Distribution of Duane syndrome according to the family history. type. Karima L. Shalaby & Mostafa Bahgat 333 Type 1: • In 80% of this group of study. • Esotropia with head straight or no deviation in primary position. • Face turn in 2 cases of this type. • Limited abduction. • Narrowing of palperal fissure on adduction. • Enophthalmos on adduction. Picture (3): L (esotropic in pp, limited abduction). Picture (1): No deviation in pp, limited abduction and retraction. Picture (2): L (esotropic in pp, defective abduction, retraction). Picture (4): Marked retraction on adduction. 334 Duane Retraction Syndrome Type 2: • DRS seen more in left eye in this group of study 53.33%. • 13% in this group of study exotropia. • DRS seen in right eye in 5 patients 33.33%. • Limited adduction, full to slight limited abduction. • Bilateral in 2 patients 13.33% (Fig. 3). • Narrowing of palperal fissure & enophthalmos. 10 Eye Left 8 Bilateral Right 6 Count 4 2 0 Unilateral Bilateral Bilateral Fig. (3): Bilaterality of Duane syndrome in Pediatric Clinic 2006. • Good fusion in 2 patients 13.3%. Picture (5): Type 2 (R limited adduction and retraction). • Due to anisometropia amblyopia seen in 6 patients 40%. Type 3: • Occlusion therapy trial failed in 1 patient (7 years • In 7% in the group of study. old, type 1) patient operated. • Eyes are aligned in Primary position, with head • Anterior segment and fundus normal in all pa- straight, with fusion. tients. • Limited abduction and adduction. • 2 patients: Pre-auricular skin tag (Picture 6). • Severe up and down shoot. • (Ear-Nose-Throat examination: normal). • 1 patient has hand anomaly. • No other abnormality detected. Picture (6): (No deviation in pp, L marked up shoot on attempted adduction). Picture (7): (Auricular skin tag in Duane syndrome patient). Karima L. Shalaby & Mostafa Bahgat 335 Picture (8): Post operative results. Picture (9) Discussion o Reduction of up and down shoot. o Reduction of enophthalmos. • The clinical picture of DRS varies widely [2] . o Alignment of eyes in PP [6,7,10] . • Careful assessment of ocular motility is important [1,3,5] . • No treatment available will eliminate the abnor- mality of DRS but will lessen them [10] . • Each case of DRS requires unique approach to treatment if treatment is even considered. • The limitation of treatment: • The goals of treatment of DRS are: o Normal ductions and versions cannot be o Improve head posture. achieved. 336 Duane Retraction Syndrome o Up shoot, down shoot & enophthalmos can be therapy; Saunders Company; Pennsylvania, 398-400, 2000. greatly reduced but not eliminated [10] . 6- HELVESTON E. and F. ELLIS: Pediatric Ophthalmology • Two patients operated to improve alignment in Practice. Mosby Company, Chapter 2: 44-45, 1980. primary position in this group of study (13.3%). 7- PLAGER D.: Strabismus Surgery; Oxford University Medial rectus muscle were too tight on forced Press, Chapter 1: 9, Chapter 2: 31-32, 2004. duction test. Both Medial rectus muscle were 8- KANSKI J.: Clinical Ophthalmology; Elsevier Science, recessed with acceptable improvement of head Chapter 16: 548, 2003. posture, reduction in enophthalmos. 9- Duane Clinical Ophthalmology, Volume 1, Chapter 20, References 2003. 1- HELVESTON E.: Surgical management of strabismus 5: 10- HELVESTON E.: The Strabismus Minute, Cybersight, Wayenborgh, Belgium, Chapter 5: 149-150, 2005. Volume 2: 105-110, 2000. 2- RUESSMANN W.: Surgical Ophthalmology, 1: 357-358, 11- CHUNG M. and STOUT J.: Clinical diversity of hereditary 1991. Duane retraction syndrome. Ophthalmology, 107: 500-3, 3- KAUFMANN H. STRABISMUS: G. Kommerell Enke, 2000. stutgart, Augenmuscle lahmung 4.1 Retraktion syndrome 12- MOSTAFA A.: Strabismus for Postgraduate Students. 520, 1995. Egypt, Cairo, 128-130, 2004. 4- American Academy of Ophthalmology: BCSC. Section 13- WRIGHT K.W.: Color Atlas of Strabismus Surgery. 5-6; 229-236; 137-140, 2004-2005. Strategies and Techniques. Republic of Panama, Wright 5- FRAUENFELDER F. and ROY F.H.: Current ocular Publishing, 2000. .
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