-
Journal of AAPOS : the Official... Feb 2021To determine the prevalence of anomalous extraocular bands in patients who underwent surgery for Duane syndrome and to compare the clinical findings in patients with and...
PURPOSE
To determine the prevalence of anomalous extraocular bands in patients who underwent surgery for Duane syndrome and to compare the clinical findings in patients with and without bands.
METHODS
Thirty-one patients with Duane syndrome who had their first surgery on rectus muscles to correct the primary deviation and abnormal head posture were included in this retrospective study. Patients were divided into two groups depending on the identification of anomalous extraocular bands intraoperatively. Baseline clinical characteristics were compared between the groups.
RESULTS
A total of 31 patients were included. Anomalous bands were found in 6 of 19 (32%) patients with esotropic Duane syndrome and 9 of 12 (75%) with exotropic Duane syndrome (P = 0.02). In esotropic Duane syndrome, the bands were localized under the medial rectus muscle in 5 patients and under the lateral rectus muscle in 1 patient. All of the bands in patients with exotropic Duane syndrome were under the lateral rectus muscle. The amount of preoperative primary deviation, globe retraction, and up- or downshoot were similar between groups. All of the bands had distinct tight insertion on the sclera, requiring a sharp dissection for disinsertion. In 7 cases, the anomalous band was a translucent structure that could be identified under the surgical microscope as scleral indentation during forced duction testing. Histological examination of 6 cases revealed only fibrous tissue in 4 and accompanying striated muscle tissue in 2 patients.
CONCLUSIONS
The present study highlights the incidence of anomalous bands in Duane syndrome. Repeating forced duction testing after disinsertion of the affected muscle and excision of the anomalous band is helpful for intraoperative identification of these structures.
Topics: Duane Retraction Syndrome; Humans; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Retrospective Studies; Sclera
PubMed: 33601039
DOI: 10.1016/j.jaapos.2020.10.006 -
Journal of Pediatric Ophthalmology and... Jan 2021To report surgical outcomes of patients with exotropic Duane retraction syndrome.
PURPOSE
To report surgical outcomes of patients with exotropic Duane retraction syndrome.
METHODS
A retrospective review of the medical records from patients with exotropic Duane retraction syndrome who underwent strabismus surgery and had at least 2 months of postoperative follow-up was conducted. Surgical success was defined as a postoperative horizontal deviation within 8 prism diopters (PD), abnormal head posture (AHP) less than 5 degrees, and a two-step decrease in overshoots.
RESULTS
Seventy-three patients with exotropic Duane retraction syndrome (38, 52% male, mean age 14 ± 7.9 years) met the study criteria. Unilateral type 3 Duane retraction syndrome was seen in 56 (77%) patients and type 1 in 14 (19%) patients. The mean follow-up period was 22 weeks (range: 8 to 209 weeks). Unilateral and bilateral lateral rectus recession were performed in 48 (65%) and 25 (34%) patients, respectively. In addition to recession, a lateral rectus Y-splitting was performed in 42 (56%) patients. Five patients underwent differential medial rectus and (larger) lateral rectus recession to alleviate severe globe retraction. The mean primary position reduced from 23 PD (CI: 19.6 to 26 PD) preoperatively to 9 PD (CI: 7.02 to 11.02 PD) postoperatively (P < .0001). Postoperatively 50% of patients were orthotropic in the primary position. Surgical success for primary position deviation, AHP, and overshoots were achieved in 74%, 81%, and 71% of patients, respectively.
CONCLUSIONS
In the current study, type 3 Duane retraction syndrome was the most common subtype necessitating surgery for exotropic Duane retraction syndrome. Good surgical outcomes were obtained for various indications. Unilateral or bilateral lateral rectus recession was successful in improving motor alignment and AHP. The addition of lateral rectus Y-splitting corrected overshoots greater than grade 2 in most patients with exotropic Duane retraction syndrome. [J Pediatr Ophthalmol Strabismus. 2021;58(1):9-16.].
Topics: Adolescent; Adult; Child; Duane Retraction Syndrome; Female; Humans; Male; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Retrospective Studies; Treatment Outcome; Young Adult
PubMed: 33495792
DOI: 10.3928/01913913-20200910-02 -
Strabismus Mar 2021A 10-year-old boy presented with complaints of abnormal eye movements and face turn since early There was a limitation of adduction in LE with narrowing of palpebral...
A 10-year-old boy presented with complaints of abnormal eye movements and face turn since early There was a limitation of adduction in LE with narrowing of palpebral fissure and downshoot characteristic of type 2 Duane Syndrome. He was a known case of Arthrogryposis multiplex congenita (AMC) and had undergone multiple orthopedic surgeries. The literature on Duane Retraction Syndrome (DRS) in AMC is limited. Here, we report in brief the association of DRS type 2 in AMC.
Topics: Arthrogryposis; Child; Duane Retraction Syndrome; Humans; Male
PubMed: 33480805
DOI: 10.1080/09273972.2020.1871380 -
BMC Ophthalmology Jan 2021Superior rectus muscle transposition (SRT) is one of the proposed transposition techniques in the management of defective ocular abduction secondary to chronic sixth...
BACKGROUND
Superior rectus muscle transposition (SRT) is one of the proposed transposition techniques in the management of defective ocular abduction secondary to chronic sixth nerve palsy and esotropic Duane retraction syndrome (Eso-DRS). The aim of the current study is to report the outcomes of augmented SRT in treatment of Eso-DRS and chronic sixth nerve palsy.
METHODS
a retrospective review of medical records of patients with Eso-DRS and complete chronic sixth nerve palsy who were treated by augmented full tendon SRT combined with medial rectus recession (MRc) when intraoperative forced duction test yielded a significant contracture. Effect on primary position esotropia (ET), abnormal head posture (AHP), limitation of ocular ductions as well as complications were reported and analyzed.
RESULTS
a total of 21 patients were identified: 10 patients with 6th nerve palsy and 11 patients with Eso-DRS. In both groups, SRT was combined with ipsilateral MRc in 18 cases. ET, AHP and limited abduction were improved by means of 33.8PD, 26.5°, and 2.6 units in 6th nerve palsy group and by 31.1PD, 28.6°, and 2 units in Eso-DRS group respectively. Surgical success which was defined as within 10 PD of horizontal orthotropia and within 4 PD of vertical orthotropia was achieved in 15 cases (71.4%). Significant induced hypertropia of more than 4 PD was reported in 3 patients (30%) and in 2 patients (18%) in both groups, respectively.
CONCLUSION
augmented SRT with or without MRc is an effective tool for management of ET, AHP and limited abduction secondary to sixth nerve palsy and Eso-DRS. However, this form of augmented superior rectus muscle transposition could result in high rates of induced vertical deviation.
Topics: Abducens Nerve Diseases; Duane Retraction Syndrome; Esotropia; Eye Movements; Humans; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Retrospective Studies; Vision, Binocular
PubMed: 33472581
DOI: 10.1186/s12886-020-01779-1 -
Strabismus Mar 2021A fixation preference for the affected eye is uncommon in patients with unilateral Duane retraction syndrome (DRS), and surgery on the fellow eye is rarely advocated. We...
A fixation preference for the affected eye is uncommon in patients with unilateral Duane retraction syndrome (DRS), and surgery on the fellow eye is rarely advocated. We are presenting a case report of a 9-year-old boy with unilateral DRS type II in the left eye who received lateral rectus muscle recession in his right amblyopic eye. The patient was orthophoric and his face turn was gone 6 months postoperatively. Surgery on the fellow amblyopic eye is a good choice for unilateral DRS where the affected eye dominants the fixation, and the satisfactory outcome suggests that alignment in the primary position can correct the face turn effectively despite the muscle duction deficit in the affected eye and further extend the binocular single visual field.
Topics: Child; Duane Retraction Syndrome; Humans; Male; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Retrospective Studies; Treatment Outcome; Vision, Binocular
PubMed: 33455502
DOI: 10.1080/09273972.2020.1871377 -
Medical Hypotheses Nov 2020Marcus Gunn Syndrome (MGS), mostly occurring in congenital ptosis patients, is characterized by jaw movement evoking ptotic eyelid retraction, followed by collapse....
Marcus Gunn Syndrome (MGS), mostly occurring in congenital ptosis patients, is characterized by jaw movement evoking ptotic eyelid retraction, followed by collapse. Inverted, bilateral and acquired MGS were also reported. Some cases manifest MGS only temporarily in life. These features suggest MGS may be due to multiple pathogeneses, which are still unclear. People also classify MGS as a kind of trigeminal oculomotor synkenesis (TOS), like Duane syndrome (DS), in which ocular adduction prompts eyelid moving or eyeball retraction. The most popular hypothesis for TOS is congenital miswiring, as evidence supporting this hypothesis is found in DS cases: hypoplasia abducens nerve fusing with a branch of oculomotor nerve is observed. Seven mutant genes have been identified associated with TOS and two of them are found among MGS cases. Accordingly, these mutant genes may dominate cranial nerve misconnection and generate TOS. However, unlike in DS case, evidence of miswiring is not encountered in most MGS cases. The fact is that two "MGS genes" are from congenital fibrosis of extraocular muscle (CFEOM) cases presenting with associated MGS. But most of MGS cases do not suffer CFEOM. Thus, mutant genes dominated congenital miswiring may not be the pathogenesis for the majority of MGS. As an alternate pathogenic pathway, a "release hypothesis" proposed that MGS is a primitive physiologic reflex that became suppressed during phylogenetic development but could be released under certain pathologic conditions. This hypothesis was and is overlooked because the hypothesized reflex arc has not been defined. Decades ago, a neural tract tracing study in Xenopus revealed a direct projection from masticator afferent mesencephalic trigeminal nucleus (Vme) neurons to oculomotor and trochlear nucleus (III/IV). In clinical studies, co-firing of pterygoid muscle and levator palpebrae was recorded by electromyography during onset of MGS, and stimulating pterygoid muscle nerve elicited eyelid retraction. Recently, retraction of the ipsilateral eyelid by stimulating the trigeminal motor root was even observed in cases without congenital ptosis and MGS, highlighting the existence of a latent pathway. In rats, recently we demonstrated projections from the Vme neurons to the III/IV, and to their premotor neurons in interstitial nucleus of Cajal by neural tract tracing and electrophysiologic studies. Fos expression in pre-oculomotor neurons was induced by repeated down stretching the lower jaw. Combining previous and our own studies, we assumed the Vme neurons is excited when jaw moves and in turn, some eyelid activity related III motoneurons are activated through projections of Vme to oculomotor system, like in Xenopus. Genetic factors may dominate to what extent this primitive reflex-arc is preserved, which consequently determines phenotype.
Topics: Animals; Blepharoptosis; Humans; Jaw Abnormalities; Oculomotor Muscles; Phylogeny; Rats; Rats, Gunn; Trigeminal Nerve
PubMed: 33254517
DOI: 10.1016/j.mehy.2020.110210 -
European Journal of Ophthalmology Jan 2022To evaluate the effect of half tendon vertical rectus muscle transposition (HVRT) combined with medial rectus muscle recession (MRrec) in complete sixth nerve palsy...
PURPOSE
To evaluate the effect of half tendon vertical rectus muscle transposition (HVRT) combined with medial rectus muscle recession (MRrec) in complete sixth nerve palsy (CSNP) and esotropic Duane Retraction Syndrome (eDRS).
METHODS
A retrospective study of patients with unilateral CSNP or eDRS who underwent HVRT combined with medial rectus recession over the course of 18 years. The patients with previous strabismus surgery and follow up less than 3 months were excluded. Overcorrection was defined as any amount of exotropia, and undercorrection was defined as residual esotropia of ⩾10 prism diopter (PD).
RESULTS
A total of 39 patients were enrolled including 22 CSNP and 17 eDRS patients. Esotropia improved from 45.8 ± 22 and 22.5 ± 6.4 PD to 0.8 ± 2.5 and 0.3 ± 1 PD in CSNP group and eDRS group respectively. The angle of face turn improved from 34.3 ± 8.4° and 26.5 ± 9° to 0.6 ± 2.4° and 0.8 ± 1.6° in the CSNP group and eDRS group respectively. Abduction limitation improved from -4.5 ± 0.5 and -4 ± 0 units to -2.9 ± 0.5 and -2.7 ± 0.5 units in CSNP group and eDRS group respectively. No patient developed a new vertical deviation. An overcorrection occurred in one patient of the eDRS group who improved after botulinum toxin injection in the ipsilateral lateral rectus muscle. Undercorrection was seen in two patients.
CONCLUSION
HVRT combined with MRrec is an effective procedure to improve esotropia, face turn, and abduction limitation in CSNP and eDRS.
Topics: Abducens Nerve Diseases; Duane Retraction Syndrome; Esotropia; Eye Movements; Humans; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Retrospective Studies; Vision, Binocular
PubMed: 33228408
DOI: 10.1177/1120672120973612 -
Clinical Imaging Mar 2021We aim to review the imaging features of congenital cranial dysinnervation disorders. Characteristic imaging findings can define subtypes of these disorders through... (Review)
Review
We aim to review the imaging features of congenital cranial dysinnervation disorders. Characteristic imaging findings can define subtypes of these disorders through assessment of cranial nerves, extraocular muscles, orbital, and brain abnormalities. Duane retraction syndrome shows absent or hypoplasic 6th cranial nerve and preserved extraocular muscles (EOM). Mobius syndrome shows absent 7th and 6th cranial nerves, absence of facial colliculus, flattening of the dorsal aspect of the pons, hypoplasia of the pons and medulla, and flattening of the 4th ventricular floor. Congenital fibrosis of the extraocular muscles reveals unilateral or bilateral hypoplasia or aplasia of the 3rd cranial nerve, atrophy of superior rectus and levator palpebrae superioris muscles, and atrophy of the brainstem and cerebellar hemispheres. Horizontal gaze palsy and progressive scoliosis show characteristic split pons sign, butterfly medulla, absent facial colliculi, and spinal scoliosis. HOXA1 Mutations show a bilateral absence of 6th cranial nerves with the underdeveloped inner ear. Pontine Cap Tegmental Dysplasia shows ventral pontine hypoplasia, dorsal tegmental projection into the 4th ventricle, and variable cranial nerve deficits.
Topics: Cranial Nerves; Duane Retraction Syndrome; Humans; Magnetic Resonance Imaging; Oculomotor Muscles; Radiologists
PubMed: 33189029
DOI: 10.1016/j.clinimag.2020.10.055 -
International Ophthalmology Mar 2021To assess the effect of lateral rectus muscle resection on abduction in Duane retraction syndrome (DRS) type 1.
PURPOSE
To assess the effect of lateral rectus muscle resection on abduction in Duane retraction syndrome (DRS) type 1.
METHODS
The medical records of patients with DRS type 1 were reviewed retrospectively. Fifteen patients who underwent lateral rectus resection were included. Prism and cover test and the Krimsky test were used to detect deviations. Ocular ductions, abnormal head position (AHP), and globe retraction were recorded.
RESULTS
Nine (60.0%) patients were female. The mean age was 13.1 ± 2.3 (range, 2-34) years. Left eyes were included in ten (66.7%) patients. Mean follow-up time was 37.6 ± 16.6 (range, 6-70) months. All patients had AHP, 13 patients had mild globe retraction, and 2 patients had no globe retraction preoperatively. Mean lateral rectus resection was 3.1 ± 0.7 (range, 2.0-4.5) mm, and the mean medial rectus recession was 4.4 ± 0.6 (range, 3.0-5.0) mm. The mean preoperative deviation decreased from 23.3 ± 6.9 (range, 14-35) prism diopters (pd) to 2.2 ± 4.1 (range, 0-10) pd at near, and from 23.6 ± 7.1 (range, 14-35) pd to 1.8 ± 3.5 (range, 0-10) pd at distance, at 6 months postoperatively (p = 0.01). The mean limitation in abduction decreased from - 3.2 ± 0.9 to - 1.3 ± 1.1 postoperatively (p < 0.001). AHP resolved in all patients. There was not a limitation in adduction or a worsening of globe retraction in any patient.
CONCLUSION
Lateral rectus resection can be used to improve abduction in patients with DRS type 1 who have mild globe retraction. We assume that this procedure has no worsening effect on globe retraction in appropriate cases.
Topics: Adolescent; Child; Duane Retraction Syndrome; Eye Movements; Female; Humans; Male; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Retrospective Studies
PubMed: 33185820
DOI: 10.1007/s10792-020-01632-w -
European Journal of Ophthalmology Sep 2021To evaluate the efficacy of symmetric bilateral medial rectus recession in large-angle esotropic Duane retraction syndrome (DRS) with moderate to severe globe retraction.
PURPOSE
To evaluate the efficacy of symmetric bilateral medial rectus recession in large-angle esotropic Duane retraction syndrome (DRS) with moderate to severe globe retraction.
METHODS
In a retrospective study, medical reports of 30 patients with esotropia of equal or more than 20 prism diopters (pd) and moderate to serve globe retraction due to unilateral DRS who underwent symmetric bilateral medial rectus recession were reviewed. Age, gender, laterality, amblyopia, length of follow-up, pre- and postoperative measurements of primary position deviation, ocular ductions and severity of globe retraction and abnormal head posture were evaluated. A successful result was defined as decreasing esotropia to equal or less than 8 pd or equal or less than 8 pd of consecutive exotropia.
RESULTS
The mean age of patients at surgery was 13.7 ± 8.5 years old (range: 3-38). The mean preoperative esotropia measured 28.9 ± 9.1 pd in distance and 25.7 ± 7.2 pd in near, which decreased to 4.9 ± 6.1 pd in distance and 3.9 ± 8.8 pd in near postoperatively. The mean bilateral medial rectus recession was 4.9 ± 0.9 mm (range: 3-6 mm). The mean abnormal head posture improved from 19.1 ± 6.9 degrees (range: 10-30 degrees) to 3.3 ± 4.7 degrees (range: 0-15 degrees) postoperatively. At the last follow-up visit, 23 patients (76.7%) had a successful outcome.
CONCLUSIONS
In the large-angle esotropic DRS patients with moderate to severe globe retraction, symmetric bilateral medial rectus recession, can be conducted to successfully resolve primary position deviation and abnormal head posture.
Topics: Adolescent; Adult; Child; Child, Preschool; Duane Retraction Syndrome; Esotropia; Follow-Up Studies; Humans; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Retrospective Studies; Treatment Outcome; Vision, Binocular; Young Adult
PubMed: 33148050
DOI: 10.1177/1120672120968731