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Duane retraction syndrome mimicking orbital fracture
Available online at www.sciencedirect.com
British Journal of Oral and Maxillofacial Surgery 49 (2011) e76–e78
Short communication
Duane retraction syndrome mimicking orbital fracture Simon Toledano ∗ , Alan Parbhoo 1 , Derek Von Arx 1 Bedfordshire, Hertfordshire & Buckinghamshire Network, Luton LU4 0DZ, United Kingdom Accepted 27 March 2011 Available online 6 May 2011
Abstract We describe the case of a 20-year-old patient who presented at the accident and emergency department with symptoms and signs of fractures of the zygomatic complex and orbit following an assault. He was later discovered to have Duane retraction syndrome, a rare congenital disorder of eye movement that has some of the clinical characteristics of fractures of the zygomatic complex and orbit. The fracture was not displaced, and without knowledge of the syndrome a surgeon might have been forced to investigate further. © 2011 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Keywords: Duane retraction syndrome; Orbital fracture; Trauma; Zygomatic complex fracture; Diplopia
Case report A 20-year-old man attended the accident and emergency department with pain and bruising around his right eye following an assault when his face had been kicked and punched numerous times. His head was not injured and his Glasgow Coma Score was 15. He was fit and healthy with no noteworthy medical history. On examination he had right periorbital bruising and swelling, right temporal abrasions, evidence of epistaxis, which had ceased, and paraesthesia of the right infraorbital region. There was tenderness on palpation of the right infraorbital rim and zygomaticofrontal suture. No step deformities were noted. Ocular examination showed diplopia in right lateral gaze with limitation of abduction, and enophthalmos of the right eye (Figs. 1 and 2). Direct and consensual pupillary reflexes were normal. Radiographs showed a probable undisplaced fracture of the right zygomatic complex with an opaque right antrum.
∗
Corresponding author. Tel.: +44 01582718062. E-mail addresses: [email protected], [email protected] (S. Toledano), [email protected] (A. Parbhoo), [email protected] (D. Von Arx). 1 Tel.: +44 01582718062.
When the patient was informed that some of his ocular movements were limited and that this could be caused by a fracture of the orbital floor, he reported that a diagnosis of Duane retraction syndrome in the right eye had been made by an optician a month previously. He was given instructions for the non-surgical management of his injuries and was asked to return for a review appointment. No further ophthalmic or orthoptic input was required.
Discussion Duane retraction syndrome is a rare congenital disorder of eye movement with an incidence of about 0.1%, and accounts for 1–5% of all cases of strabismus. It is characterised by marked limitation or absence of abduction, variable limitation of adduction, narrowing of the palpebral fissure, and retraction of the globe on attempted adduction of the affected eye (Fig. 3).1 It is named after Alexander Duane, an American ophthalmologist.1 In 85% of cases it is unilateral, and it mainly affects the left eye.2 The syndrome is a congenital anomaly of the nucleus of the sixth cranial nerve with aberrant innervations from the third cranial nerve, which results in the co-contraction of the medial and lateral rectus on adduction. Breinin3 described this co-contraction as the cause of the globe
0266-4356/$ – see front matter © 2011 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
doi:10.1016/j.bjoms.2011.03.007
S. Toledano et al. / British Journal of Oral and Maxillofacial Surgery 49 (2011) e76–e78
Fig. 1. Photograph shows the patient’s appearance at review after most of the bruising and swelling had resolved (published with the patient’s consent): forward gaze.
Fig. 2. Photograph shows the patient’s appearance at review after most of the bruising and swelling had resolved (published with the patient’s consent): right lateral gaze.
being retracted on adduction. In normal ocular function, when a muscle contracts its direct antagonist relaxes to allow smooth movement. This has been defined as Sherrington’s law of reciprocal innervation. Both genetic and environmental factors are thought to be involved when the
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cranial nerves and ocular muscles are developing between the fourth and eighth week of gestation. Interestingly, most patients remain asymptomatic and are able to maintain binocularity with only a slightly abnormal position of the head.4 Many attempts to classify the syndrome have been attempted. Huber5 developed a system based on electromyographic findings, which is still widely used today. Type I shows marked limitation of abduction with minimally defective or normal adduction, retraction of the globe and narrowing of the palpebral fissure in adduction, and widening of the fissure in abduction. Type II shows marked limitation of adduction, normal or slightly limited abduction, and retraction of the globe and narrowing of the palpebral fissure in adduction. Type III shows combined limitation or absence of both abduction and adduction, and retraction of the globe and narrowing of the palpebral fissure in adduction. Most patients are diagnosed with the condition during childhood but it can easily be mistaken for common childhood squint. When making a diagnosis it is imperative that abduction or adduction are carefully examined, as well as any changes in the palpebral fissure and position of the head. Similar clinical features can also be caused by inflammation of orbital soft tissue, or injury,6 orbital surgery,7 pterygium excision surgery,8 localised infection,9 or neoplasm.10 When assessing ocular function in patients who have been injured, it is important to consider other causes of reduced ocular mobility. Duane retraction syndrome, although rare, can confuse the clinical picture and it is imperative that such injuries are investigated fully before embarking on treatment. All patients with orbital injuries in our department have ophthalmic, and orthoptic assessment with HESS charting and fields of binocular single vision before operation is considered. We find this multidisciplinary approach extremely helpful.
References
Fig. 3. Photograph shows the patient’s appearance at review after most of the bruising and swelling had resolved (published with the patient’s consent): left upward gaze.
1. Duane A. Congenital deficiency of abduction associated with impairment of adduction, retraction movement, contraction of the palpebral fissure and oblique movements of the eye. Arch Ophthalmol 1905;34:133–45. 2. Zanin E, Gambarelli N, Denis D. Distinctive clinical features of bilateral Duane retraction syndrome. J AAPOS 2010;14:293–7. 3. Breinin GM. Electromyography; a tool in ocular and neurologic diagnosis. II. Muscle palsies. AMA Arch Ophthalmol 1957;57: 165–75. 4. Yüksel D, Orban de Xivry JJ, Lefèvre P. Review of the major findings about Duane retraction syndrome (DRS) leading to an updated form of classification. Vision Res 2010;50:2334–47. 5. Huber A. Electrophysiology of the retraction syndromes. Br J Ophthalmol 1974;58:293–300. 6. Duane TD, Schatz NJ, Caputo AR. Pseudo-Duane’s retraction syndrome. Trans Am Ophthalmol Soc 1976;74:122–32.
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S. Toledano et al. / British Journal of Oral and Maxillofacial Surgery 49 (2011) e76–e78
7. Sood GC, Srinath BS, Krishnamurthy G. Acquired Duane’s retraction syndrome following Kronlein’s operation. Eye Ear Nose Throat Mon 1975;54:308–10. 8. Khan AO. Inverse globe retraction syndrome complicating recurrent pterygium. Br J Ophthalmol 2005;89:640–1.
9. Murthy R. Inverse Duane’s retraction syndrome following myocysticercosis. Indian J Ophthalmol 2008;56:89–90. 10. Kivlin JD, Lundergan MK. Acquired retraction syndrome associated with orbital metastasis. J Pediatr Ophthalmol Strabismus 1985;22:109–12.
British Journal of Oral and Maxillofacial Surgery 49 (2011) e76–e78
Short communication
Duane retraction syndrome mimicking orbital fracture Simon Toledano ∗ , Alan Parbhoo 1 , Derek Von Arx 1 Bedfordshire, Hertfordshire & Buckinghamshire Network, Luton LU4 0DZ, United Kingdom Accepted 27 March 2011 Available online 6 May 2011
Abstract We describe the case of a 20-year-old patient who presented at the accident and emergency department with symptoms and signs of fractures of the zygomatic complex and orbit following an assault. He was later discovered to have Duane retraction syndrome, a rare congenital disorder of eye movement that has some of the clinical characteristics of fractures of the zygomatic complex and orbit. The fracture was not displaced, and without knowledge of the syndrome a surgeon might have been forced to investigate further. © 2011 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Keywords: Duane retraction syndrome; Orbital fracture; Trauma; Zygomatic complex fracture; Diplopia
Case report A 20-year-old man attended the accident and emergency department with pain and bruising around his right eye following an assault when his face had been kicked and punched numerous times. His head was not injured and his Glasgow Coma Score was 15. He was fit and healthy with no noteworthy medical history. On examination he had right periorbital bruising and swelling, right temporal abrasions, evidence of epistaxis, which had ceased, and paraesthesia of the right infraorbital region. There was tenderness on palpation of the right infraorbital rim and zygomaticofrontal suture. No step deformities were noted. Ocular examination showed diplopia in right lateral gaze with limitation of abduction, and enophthalmos of the right eye (Figs. 1 and 2). Direct and consensual pupillary reflexes were normal. Radiographs showed a probable undisplaced fracture of the right zygomatic complex with an opaque right antrum.
∗
Corresponding author. Tel.: +44 01582718062. E-mail addresses: [email protected], [email protected] (S. Toledano), [email protected] (A. Parbhoo), [email protected] (D. Von Arx). 1 Tel.: +44 01582718062.
When the patient was informed that some of his ocular movements were limited and that this could be caused by a fracture of the orbital floor, he reported that a diagnosis of Duane retraction syndrome in the right eye had been made by an optician a month previously. He was given instructions for the non-surgical management of his injuries and was asked to return for a review appointment. No further ophthalmic or orthoptic input was required.
Discussion Duane retraction syndrome is a rare congenital disorder of eye movement with an incidence of about 0.1%, and accounts for 1–5% of all cases of strabismus. It is characterised by marked limitation or absence of abduction, variable limitation of adduction, narrowing of the palpebral fissure, and retraction of the globe on attempted adduction of the affected eye (Fig. 3).1 It is named after Alexander Duane, an American ophthalmologist.1 In 85% of cases it is unilateral, and it mainly affects the left eye.2 The syndrome is a congenital anomaly of the nucleus of the sixth cranial nerve with aberrant innervations from the third cranial nerve, which results in the co-contraction of the medial and lateral rectus on adduction. Breinin3 described this co-contraction as the cause of the globe
0266-4356/$ – see front matter © 2011 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
doi:10.1016/j.bjoms.2011.03.007
S. Toledano et al. / British Journal of Oral and Maxillofacial Surgery 49 (2011) e76–e78
Fig. 1. Photograph shows the patient’s appearance at review after most of the bruising and swelling had resolved (published with the patient’s consent): forward gaze.
Fig. 2. Photograph shows the patient’s appearance at review after most of the bruising and swelling had resolved (published with the patient’s consent): right lateral gaze.
being retracted on adduction. In normal ocular function, when a muscle contracts its direct antagonist relaxes to allow smooth movement. This has been defined as Sherrington’s law of reciprocal innervation. Both genetic and environmental factors are thought to be involved when the
e77
cranial nerves and ocular muscles are developing between the fourth and eighth week of gestation. Interestingly, most patients remain asymptomatic and are able to maintain binocularity with only a slightly abnormal position of the head.4 Many attempts to classify the syndrome have been attempted. Huber5 developed a system based on electromyographic findings, which is still widely used today. Type I shows marked limitation of abduction with minimally defective or normal adduction, retraction of the globe and narrowing of the palpebral fissure in adduction, and widening of the fissure in abduction. Type II shows marked limitation of adduction, normal or slightly limited abduction, and retraction of the globe and narrowing of the palpebral fissure in adduction. Type III shows combined limitation or absence of both abduction and adduction, and retraction of the globe and narrowing of the palpebral fissure in adduction. Most patients are diagnosed with the condition during childhood but it can easily be mistaken for common childhood squint. When making a diagnosis it is imperative that abduction or adduction are carefully examined, as well as any changes in the palpebral fissure and position of the head. Similar clinical features can also be caused by inflammation of orbital soft tissue, or injury,6 orbital surgery,7 pterygium excision surgery,8 localised infection,9 or neoplasm.10 When assessing ocular function in patients who have been injured, it is important to consider other causes of reduced ocular mobility. Duane retraction syndrome, although rare, can confuse the clinical picture and it is imperative that such injuries are investigated fully before embarking on treatment. All patients with orbital injuries in our department have ophthalmic, and orthoptic assessment with HESS charting and fields of binocular single vision before operation is considered. We find this multidisciplinary approach extremely helpful.
References
Fig. 3. Photograph shows the patient’s appearance at review after most of the bruising and swelling had resolved (published with the patient’s consent): left upward gaze.
1. Duane A. Congenital deficiency of abduction associated with impairment of adduction, retraction movement, contraction of the palpebral fissure and oblique movements of the eye. Arch Ophthalmol 1905;34:133–45. 2. Zanin E, Gambarelli N, Denis D. Distinctive clinical features of bilateral Duane retraction syndrome. J AAPOS 2010;14:293–7. 3. Breinin GM. Electromyography; a tool in ocular and neurologic diagnosis. II. Muscle palsies. AMA Arch Ophthalmol 1957;57: 165–75. 4. Yüksel D, Orban de Xivry JJ, Lefèvre P. Review of the major findings about Duane retraction syndrome (DRS) leading to an updated form of classification. Vision Res 2010;50:2334–47. 5. Huber A. Electrophysiology of the retraction syndromes. Br J Ophthalmol 1974;58:293–300. 6. Duane TD, Schatz NJ, Caputo AR. Pseudo-Duane’s retraction syndrome. Trans Am Ophthalmol Soc 1976;74:122–32.
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S. Toledano et al. / British Journal of Oral and Maxillofacial Surgery 49 (2011) e76–e78
7. Sood GC, Srinath BS, Krishnamurthy G. Acquired Duane’s retraction syndrome following Kronlein’s operation. Eye Ear Nose Throat Mon 1975;54:308–10. 8. Khan AO. Inverse globe retraction syndrome complicating recurrent pterygium. Br J Ophthalmol 2005;89:640–1.
9. Murthy R. Inverse Duane’s retraction syndrome following myocysticercosis. Indian J Ophthalmol 2008;56:89–90. 10. Kivlin JD, Lundergan MK. Acquired retraction syndrome associated with orbital metastasis. J Pediatr Ophthalmol Strabismus 1985;22:109–12.