China
Africa
Explore chapters and articles related to this topic
An Approach to Oculomotor Anomalies in a Child
Published in Vivek Lal, A Clinical Approach to Neuro-Ophthalmic Disorders, 2023
The most common presentation of Duane syndrome is Type I (Figure 21B.2), which is characterized by a unilateral complete lack of abduction, palpebral fissure narrowing on adduction, and esotropia. Approximately half of children with Duane syndrome are orthotropic in primary position, while the other half typically require a face turn to maintain binocular single vision.19 Duane syndrome can be differentiated from sixth nerve palsy by the size of the esotropia in primary position relative to the abduction weakness. In a patient with such severe limitation to abduction, one would expect a large angle esotropia; if the esotropia is very small, then Duane syndrome should be considered.
The eye
Published in Angus Clarke, Alex Murray, Julian Sampson, Harper's Practical Genetic Counselling, 2019
Strabismus is a frequent feature of many generalised neuromuscular disorders, which may follow Mendelian inheritance (see Chapter 13). Duane syndrome is due to aberrant innervation of ocular muscles; it is generally sporadic, but a few families follow autosomal dominant inheritance. Isolated strabismus, whether classified as convergent or divergent, fits a polygenic pattern. Variation between studies results, in part, from the extent to which minor deviations are classed as abnormal. From the viewpoint of counselling, it seems that where parents are normal and one child is affected, the risk for subsequent children is around 15%. Where one parent is also affected, the risk is around 40%.
Torticollis
Published in Benjamin Joseph, Selvadurai Nayagam, Randall Loder, Ian Torode, Paediatric Orthopaedics, 2016
A child presenting with predominantly a head tilt but without pain and without contracture of the sternocleidomastoid and with a good range of motion in the cervical spine should alert the examiner to consider an ocular cause for the posture. One should look for strabismus (misalignment of the eyes) or nystagmus (rapid jerking movement of the eye). Weakness of the superior oblique muscle is a common cause of ocular torticollis in children. Duane syndrome is a rare ocular cause of torticollis due to a congenital disorder of eye movement.1 Referral to an ophthalmologist is appropriate.
Don’t Miss This! Red Flags in the Pediatric Eye Examination: Ophthalmoplegia in Childhood
Published in Journal of Binocular Vision and Ocular Motility, 2019
If the esodeviation is new, suspicion of an abducens nerve paresis is increased with demonstrable abduction deficits in one or both eyes. Ideally, prism and alternate cover measurements should verify significant lateral incomitance in lateral gaze. An important differential diagnosis is the Duane syndrome. In the Duane syndrome, the lateral incomitance is there, but the key finding is that the esodeviation usually measures less to no deviation in primary position compared to an intermediate-sized esotropia in abducens nerve paresis. Infants with early onset large-angle esotropia often appear to have incomplete ductions. Have the examiner assess the ductions using the oculocephalic reflex by rotating the child with outstretched arms as the eyes rotate in the direction of the rotation. Rule out an accommodative or partially accommodative esotropia with a cycloplegic refraction. Family history of comitant strabismus can help reduce concern for an intracranial pathology. Lastly, a dilated fundus examination is mandatory to evaluate the possibility of optic disc swelling from increased intracranial pressure (ICP) as well as optic nerve atrophy or a macular lesion that caused a sensory esotropia.
Binocular Function in Subjects with Orthotropic Duane Retraction Syndrome
Published in Journal of Binocular Vision and Ocular Motility, 2019
Bhagya Lakshmi Marella, Ruby Moharana, Ramesh Kekunnaya
Duane retraction syndrome (DRS), also known as Stilling–Turk–Duane syndrome, is a rare form of congenital ocular motor disorder. Prevalence of DRS in the general population is 0.1% and it peaks to 1–4% of all strabismus cases.1,2 In the literature, various theories have been proposed to explain the pathogenesis of DRS. First, studies put forward myogenic theory. Studies advocating myogenic theory believed that fibrosis of the lateral rectus muscle leads to the dual insertion of the medial rectus, which causes movement restriction and the retraction of the globe on adduction.3 Further, electrophysiological studies revealed that absence of the abducens motor neurons on the ipsilateral side of the affected eye results in the aberrant innervation of the lateral rectus by the third cranial nerve.4,5 The concept of the congenital cranial dysinnervation syndrome (CCDS) has been evolved in last decade. Parsa et al. in 1998 showed the absence of sixth nerve in cases of unilateral Type 1 DRS which subsequently led to fibrosis of the extraocular muscles and the concept of CCDS.6,7
The Use of Botulinum Toxin in Strabismus Treatment
Published in Journal of Binocular Vision and Ocular Motility, 2019
ET associated to Duane syndrome should be treated with surgery but there are situations in young children with ET and hyperopia that respond to BoNT- A. In a series of eight such patients, 50% of them were able to avoid surgery, and in two patients the deviation was reduced considerably resulting in surgery to be postponed to an older age.102 Dawson and Lee also used BoNT-A in Duane patients diagnostically and found that 53% of the patients were aligned. The remainder had some reduction of the deviation.103 Ameri et al. found a significant relationship between the success rate and the amount of tightness felt on forced duction test (FDT). Better responses were observed in the presence of+1 or+2 restriction on FDT.104