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Cureus Oct 2022Duane retraction syndrome (DRS) with mechanical and innervational upshoot poses a surgical challenge. We discuss a case of DRS with mechanical and innervational upshoot...
Duane retraction syndrome (DRS) with mechanical and innervational upshoot poses a surgical challenge. We discuss a case of DRS with mechanical and innervational upshoot and its surgical management. An 11-year-old boy presented with left eye upward deviation since birth. This deviation was worst on the right gaze. His best corrected visual acuity was 6/6 OD and 6/60 OS. Refraction showed low hyperopia with low astigmatism in both eyes. Stereoacuity was absent and there was suppression on the Worth 4 dot test in the left eye. The left eye had large hypertropia of 50 prism diopter in primary gaze. Extraocular movements showed severe upshoot and narrowing of palpebral fissures on adduction and limited abduction (-2). The patient underwent Y-splitting of the left lateral rectus (LR) muscle of 10 mm, LR recession of 4 mm, and left eye superior rectus recession of 12 mm. A marked reduction in hypertropia in primary gaze was observed on day one and at two months postoperatively with residual upshoot on adduction. His left eye deviation remained stable after six months postoperatively.
PubMed: 36415378
DOI: 10.7759/cureus.30470 -
Archivos Argentinos de Pediatria Jun 2023Wildervanck syndrome (also known as cervico-oculo-acoustic dysplasia) is a very rare disease, characterized by the typical triad of cervical vertebral fusion or...
Wildervanck syndrome (also known as cervico-oculo-acoustic dysplasia) is a very rare disease, characterized by the typical triad of cervical vertebral fusion or Klippel-Feil anomaly, Duane syndrome (paresis of the sixth cranial nerve), and hearing loss. Other vascular, cardiac, and musculoskeletal conditions have also been described. In this case report, we describe a patient who met the cardinal triad and also presented additional clinical data that have not been previously reported, which contribute to broadening the disease phenotype. We have also reviewed the bibliography related to this syndrome.
Topics: Humans; Klippel-Feil Syndrome; Duane Retraction Syndrome; Deafness; Abnormalities, Multiple
PubMed: 36413195
DOI: 10.5546/aap.2022-02624.eng -
Journal of AAPOS : the Official... Oct 2022To investigate whether chronic co-contraction of the medial and lateral rectus muscles leads to higher against-the-rule astigmatism in affected versus fellow eyes in...
PURPOSE
To investigate whether chronic co-contraction of the medial and lateral rectus muscles leads to higher against-the-rule astigmatism in affected versus fellow eyes in Duane syndrome.
METHODS
A database of 4,103 patients of Dr. Arthur Jampolsky was queried for diagnosis of Duane syndrome. Inclusion criteria were unilateral Duane syndrome and complete data set. Refractive error was compared between Duane and fellow eyes.
RESULTS
A total of 84 patients with unilateral Duane syndrome were identified. The spherical equivalent of Duane eyes and fellow eyes was similar (0.4 vs. 0.2 [P = 0.14]). Mean cylinder power was higher in Duane eyes compared to fellow eyes (0.7 vs 0.4 [P = 0.0003]). There was modest evidence of more against-the-rule astigmatism in Duane eyes compared to fellow eyes (P = 0.04) but no difference for with-the-rule astigmatism (P = 0.83). Duane compared to fellow eyes were, however, significantly more likely to have oblique astigmatism (P = 0.004) or any astigmatism at all (P = 0.0005).
CONCLUSIONS
Duane eyes compared to fellow eyes had higher astigmatism that was more likely to be oblique. Our study lends support to the hypothesis that extraocular muscles may influence refractive error.
Topics: Humans; Duane Retraction Syndrome; Astigmatism; Refractive Errors; Refraction, Ocular; Oculomotor Muscles
PubMed: 36122873
DOI: 10.1016/j.jaapos.2022.07.008 -
Oman Journal of Ophthalmology 2022To describe the clinical profile and magnetic resonance imaging findings of the brain in Duane retraction syndrome (DRS) and determine whether there is an association...
PURPOSE
To describe the clinical profile and magnetic resonance imaging findings of the brain in Duane retraction syndrome (DRS) and determine whether there is an association between clinical presentation and magnetic resonance imaging (MRI) brain characteristics.
MATERIALS AND METHODS
This was a cross-sectional study done at a tertiary care center in South India. We recruited and analyzed the clinical characteristics of 54 patients with DRS. MRI of the brain with fast imaging employing steady-state acquisition (FIESTA) was performed in 41 cases, and the cisternal segment of the sixth nerve was studied. Statistical analysis was done to determine any association between the radiological and clinical features.
RESULTS
Type 1 DRS was predominant, followed by Type 3 DRS and Type 2 DRS. 9.3% of cases were bilateral and 11.1% were familial. Orthotropia was most common, followed by esotropia and exotropia. The MRI brain showed the absence of the cisternal part of the sixth nerve on the affected side in 82% of Type 1 and 75% of Type 3 unilateral DRS. Both the abducens nerves were visualized in 19.5% of the patients with unilateral DRS. There was no statistically significant association between MRI brain findings and the clinical features.
CONCLUSIONS
MRI brain with FIESTA shows absent or hypoplastic sixth nerve in most cases of Type 1 and Type 3 DRS. However, around 20% of DRS cases may show the presence of the cisternal part of the sixth nerve. Hence, clinicians must be cautious when ruling out DRS on the basis of MRI brain findings. Although aplasia of the sixth nerve is the most frequent MRI finding, it may not be the sole etiologic factor.
PubMed: 35937749
DOI: 10.4103/ojo.ojo_133_21 -
Oman Journal of Ophthalmology 2022
PubMed: 35937726
DOI: 10.4103/ojo.ojo_141_22 -
Journal of Binocular Vision and Ocular... 2023A 10-year-old boy was introduced with a chief complaint of ocular misalignment from birth. Ocular examination indicated right Brown and left Duane retraction syndrome in...
A 10-year-old boy was introduced with a chief complaint of ocular misalignment from birth. Ocular examination indicated right Brown and left Duane retraction syndrome in a non-familial pattern. We suspect that a teratogenic damage or genetic mutation may be responsible for this combination.
Topics: Male; Humans; Child; Duane Retraction Syndrome; Ocular Motility Disorders
PubMed: 35917503
DOI: No ID Found -
Japanese Journal of Ophthalmology Sep 2022To evaluate the clinical findings of different types of Duane retraction syndrome (DRS).
PURPOSE
To evaluate the clinical findings of different types of Duane retraction syndrome (DRS).
STUDY DESIGN
Retrospective.
METHODS
This study was performed on 691 patients with DRS who underwent surgery. Clinical examinations included laterality, abnormal head posture (AHP), corrected distance visual acuity (CDVA), refractive error, amblyopia, deviation, overshoots, and type of surgery.
RESULTS
The mean age of patients with DRS was 16.7 ± 12.5 (range 1.0-73) years. The patients included 396 (57.3%) women and 295 (42.7%) men (P < 0.001). DRS type I, was observed in 429 (62.1%), II in 168 (24.3%), III in 88 (12.7%) and IV in 6 (0.9%) patients. Unilateral DRS was observed OS in 628 (90.9%) [471 (%78.9) and OD in 157 (21.1%) eyes (P < 0.001)]. O ther clinical findings were AHP (n = 522, 78.1%), overshoot (n = 236, 34.2%) and amblyopia (n = 118, 17.1%). The prevalence of overshoot in types I, II, and III was 17.5% (75/429), 60.7% (102/168) and 64.8% (57/88), respectively (P < 0.001). The prevalence of amblyopia was significantly lower in patients with AHP (80/522, 15.3%) compared to patients with normal head posture (38/169, 22.5%) (P = 0.023). The mean angle of deviation in the primary position (PP) at distance was 21.7 ± 11.5 △ for esotropic group and 17.8 ± 12.4 △ for exotropic group. Sixty-two (9.0%) patients required second surgery for resolving residual misalignment (1.1 surgeries for each patient).
CONCLUSIONS
About two-thirds of DRS patients had AHP, one-third had overshoots, and one-sixth had amblyopia. The results show that different types of DRS are associated with different epidemiological and clinical characteristics.
Topics: Adolescent; Adult; Aged; Amblyopia; Child; Child, Preschool; Duane Retraction Syndrome; Esotropia; Exotropia; Female; Humans; Infant; Male; Middle Aged; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Retrospective Studies; Young Adult
PubMed: 35861933
DOI: 10.1007/s10384-022-00931-2 -
Indian Journal of Ophthalmology Jul 2022
Topics: Duane Retraction Syndrome; Eye Movements; Humans; Oculomotor Muscles
PubMed: 35791235
DOI: 10.4103/ijo.IJO_2821_21 -
Case Reports in Ophthalmology 2022Idiopathic intracranial hypertension (IIH) is a poorly understood condition, and its presentation can coexist with other diseases. Simultaneous IIH and Duane retraction...
Idiopathic intracranial hypertension (IIH) is a poorly understood condition, and its presentation can coexist with other diseases. Simultaneous IIH and Duane retraction syndrome (DRS) type 1 have never been reported to coexist in an adult patient. Herein, we report a 32-year-old obese female with a history of chronic renal failure who had a renal transplant rejection 6 years prior to presentation and was treated with oral steroids and immunosuppressive medications. She began to experience signs and symptoms of increased intracranial pressure (morning headache and binocular horizontal diplopia) and had limited abduction of one eye on examination. The case was later diagnosed as IIH with DRS type 1.
PubMed: 35702521
DOI: 10.1159/000524363 -
Journal of Pediatric Ophthalmology and... 2023To evaluate the role of botulinum toxin A in the treatment of adults with Duane retraction syndrome and its impact on quality of life.
PURPOSE
To evaluate the role of botulinum toxin A in the treatment of adults with Duane retraction syndrome and its impact on quality of life.
METHODS
A total of 25 adults with unilateral Duane retraction syndrome were selected for this interventional case series. Botulinum toxin was injected in the appropriate horizontal recti. The parameters assessed were ocular deviation (▵), overshoots, and abnormal head posture. Patients were followed up at 1 day, 10 days, and 3 months after the injection. The outcome was categorized on the basis of deviation/abnormal head posture/overshoots as: (1) significant improvement (< 8 prism diopters [PD]/< 5 degrees/≤ grade 1); (2) partial improvement (8 to 20 PD/5 to 15 degrees/≤ grade 2); and (3) no improvement (> 20 PD/> 15 degrees/≥ grade 3). Patients with partial/significant improvement were considered to have a favorable outcome. The impact on quality of life was assessed using the Adult Strabismus-20 Questionnaire scores 10 days after injection.
RESULTS
There was a significant reduction in ocular deviation in esotropic and exotropic Duane retraction syndrome at 10 days ( = .001) and 3 months ( = .04) after botulinum toxin injection. The abnormal head posture improved from 11.58 ± 7.43 to 7.86 ± 6.25 degrees at 10 days. Botulinum toxin had a positive impact on the Adult Strabismus-20 Questionnaire scores, which significantly improved ( < .05) at 10 days. A favorable outcome was noted in 21 patients at 10 days but only 4 patients at 3 months. Three patients developed ptosis and 1 patient with orthotropic Duane retraction syndrome developed transient exotropia.
CONCLUSIONS
In adults with Duane retraction syndrome, botulinum toxin can have a useful diagnostic role by providing insight to patients and setting realistic expectations. It can identify patients likely to benefit from further treatment and also has a positive impact on quality of life. .
Topics: Humans; Adult; Botulinum Toxins, Type A; Oculomotor Muscles; Duane Retraction Syndrome; Quality of Life; Retrospective Studies; Strabismus; Treatment Outcome
PubMed: 35446195
DOI: 10.3928/01913913-20220324-01