Quantitative grading evaluation of the therapeutic efficacy of asymmetric bilateral lateral rectus recession in type Ⅲ Duane retraction syndrome / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To quantitatively evaluate the therapeutic efficacy of the asymmetric recession of bilateral lateral rectus in patients with unilateral type III Duane retraction syndrome.METHOD:Retrospective analysis of clinical data. The clinical data of 12 cases of type III Duane retraction syndrome with unilateral lateral rectus recession were analyzed. All subjects had restricted internal rotation, with narrowing of the palpebral fissure and vertical deviation, and restricted external rotation with widening of the palpebral fissure. All patients had abnormal head positions. The degree of strabismus was measured while maintaining the head in a neutral position. Asymmetric recession of bilateral lateral rectus was selected based on the degree of strabismus of affected eye or 1～3mm longer, with a smaller recession and Y-splitting in the affected eye and a larger recession in the healthy eye.RESULT:The difference in the degree of strabismus in the healthy eyes before and after surgery was statistically significant(Z=-4.158, P<0.01), as was the difference in the degree of restricted internal rotation(Z=-2.640, P=0.008). The difference in the degree of restricted external rotation was not statistically significant(Z=-1.732, P=0.083). The difference in abnormal head position was statistically significant(Z=-4.181, P<0.01), while the difference in eyeball recession was not statistically significant(Z=-1.414, P=0.157). The difference in vertical deviation was statistically significant(Z=-3.115, P=0.002). A total of 10 patients(83%)had stereoscopic vision before surgery, and 11 patients(92%)had recovered stereoscopic vision after surgery, with a further improvement in stereoacuity in 50% of patients.CONCLUSION:Asymmetric recession of bilateral lateral rectus combined with Y-splitting of the affected lateral rectus can effectively improve the degree of strabismus, abnormal head position, vertical deviation and stereoacuity without aggravating the degree of restriction of external rotation or eyeball recession.

Does head tilt influence facial appearance more than head turn?

Purpose: To evaluate the frequency of facial asymmetry parameters in patients with head tilt versus those with head turn. Methods: This cross-sectional comparative study was performed on 155 cases, including 58 patients with congenital pure head turn due to Duane retraction syndrome (DRS), 33 patients with congenital pure head tilt due to upshoot in adduction or DRS, and 64 orthotropic subjects as the control group. The facial appearance was evaluated by computerized analysis of digital photographs of patients' faces. Relative facial size (the ratio of the distance between the external canthus and the corner of the lips of both face sides) and facial angle (the angular difference between a line that connects two external canthi and another line that connects the two corners of the lips) measured as quantitative facial parameters. Qualitative parameters were evaluated by the presence of one-sided face, cheek, and nostril compression; and columella deviation. Results: The facial asymmetry frequency in patients with head tilt, head turn, and orthotropic subjects was observed in 32 (97%), 50 (86.2%), and 22 (34.3%), respectively (P < 0.001). In patients with head tilt and head turn, the mean facial angle was 1.78º ± 1.01º and 1.19º ± 0.84º, respectively (P = 0.004) and the mean relative facial size was 1.027 ± 0.018 and 1.018 ± 0.014, respectively (P = 0.018). The frequencies of one-sided nostril compression, cheek compression, face compression, and columella deviation in patients with pure head tilt were found in 19 (58%), 21 (64%), 19 (58%), and 19 (58%) patients, respectively, and in patients with pure head turn the frequencies were observed in 42 (72%), 37 (63%), 27 (47%), and 43 (74%), respectively. All quantitative and qualitative facial asymmetry parameters and facial asymmetry frequencies were significantly higher in head tilt and head turn patients as compared to the control group (P < 0.001). Conclusion: All facial asymmetry parameters in patients with head tilt and head turn were significantly higher than orthotropic subjects. The quantitative parameters such as relative facial size and facial angle were significantly higher in patients with pure head tilt than pure head turn. The results revealed that pure head tilt was associated with a higher prevalence of facial asymmetry than pure head turn.

Management of Duane retraction syndrome: A simplified approach

Duane retraction (or co-contraction) syndrome is a congenital restrictive strabismus which can occur either as an isolated entity or in conjunction with other congenital anomalies and is now listed as a congenital cranial dysinnervation disorder. It is characterized by co-contraction of horizontal recti on attempted adduction causing globe retraction along with variable amounts of upshoots or downshoots. It may have limited abduction or adduction or both and present as esotropic, exotropic, or orthotropic Duane. The diagnosis of this disease is usually clinical. However, recent research has provided a greater insight into the genetic basis of this disease paving a way for a greater role of genetics in the diagnosis and management. This disease can have a varied presentation and hence the treatment plan should be tailor-made for every patient. The indications for surgery are abnormal head posture, deviations in the primary position, retraction and narrowing of palpebral aperture and up- or downshoots during adduction, and sometimes also to improve abduction. The arrival of newer surgical techniques of periosteal fixation (PF) of lateral rectus (LR), partial vertical rectus transposition, or superior or inferior rectus transposition in addition to LR recession with Y-split has vastly improved the management outcomes, providing not only primary position orthophoria but also increased binocular visual fields as well.

Clinical features and surgical outcomes of Duane retraction syndrome in Turkish patients / 国际眼科杂志(Guoji Yanke Zazhi)

@#AIM: To analyze the clinical features and surgical outcomes of Duane retraction syndrome(DRS)patients.<p>METHODS: The clinical data of 94 DRS patients were evaluated. Complete ophthalmic data including age, sex, laterality, the type of DRS, angle of deviation, abnormal head posture(AHP), globe retraction, overshoots and the type of surgical approach were recorded.<p>RESULTS: The average age of patients was 15.4±9.18 years, and there were 78 males and 16 females. Unilateral and bilateral involvement were found in 87% and 13% of patients, respectively. In unilateral DRS patients, 65% left eyes predominance were observed. Of the 94 patients, 59.5% of the patients were type I, 29.7% of the patients were type II, 8.5% of the patients were type III and 2.1% of the patients were type IV. Most of the patients were 36(38%)esotropic and AHP over 10° was noted in 43(96%)patients, preoperatively. There were 40(43%)patients with overshoots which were more common in type II. Surgery was performed to 45(48%)patients.<p>CONCLUSION: An excellent surgical outcome was obtained in 32(78%)patients that underwent horizontal rectus surgery and AHP improved in 24(53%)patients. Y-splitting and posterior tenon fixation of lateral rectus both have satisfactory outcomes in the presence of significant overshoots. Foster augmentation with vertical rectus transposition achieved optimal results in abduction limitation.

Surgical effects in patients with Duane retraction syndrome / 国际眼科杂志(Guoji Yanke Zazhi)

@#AIM: To investigate the clinical characteristics and surgical effects in patients with Duane retraction syndrome(DRS).<p>METHODS: Totally 13 patients with DRS during June 2011 to December 2015 were analyzed retrospectively. The data including clinical types and manifestations, surgical methods and outcomes were reviewed and analyzed. <p>RESULTS: There were 11 male cases and 2 female cases who all had no ocular and systemic anomalies. The left eye was involved in 9 cases, the right eye was involved in 3 cases and 1 case involved in both eyes. Six cases were type Ⅰ,1 case was typeⅡand 6 cases were type Ⅲ. Eleven cases had abnormal head posture(AHP), 9 cases had the up- or down-shoot phenomenon. The surgical treatment was designed according to subtypes and clinical features which included medial rectus recession, lateral rectus recession, recession of both horizontal rectus muscles and lateral rectus recession combined with Y splitting. After surgery, horizontal deviation was less than ±10^△in all patients, and AHP disappeared in 4 cases and improved in 7 cases. The up- or down-shoot and global retraction disappeared in 5 cases and improved in 4 cases. Simultaneously, the restriction of ocular motility was improved in all patients. <p>CONCLUSION: The clinical features of DRS are variant in different types. Detailed examination before surgery and reasonable surgical design are important in treatment of patients with DRS.

Imaging of Cranial Nerves III, IV, VI in Congenital Cranial Dysinnervation Disorders

Congenital cranial dysinnervation disorders are a group of diseases caused by abnormal development of cranial nerve nuclei or their axonal connections, resulting in aberrant innervation of the ocular and facial musculature. Its diagnosis could be facilitated by the development of high resolution thin-section magnetic resonance imaging. The purpose of this review is to describe the method to visualize cranial nerves III, IV, and VI and to present the imaging findings of congenital cranial dysinnervation disorders including congenital oculomotor nerve palsy, congenital trochlear nerve palsy, Duane retraction syndrome, Möbius syndrome, congenital fibrosis of the extraocular muscles, synergistic divergence, and synergistic convergence.

Evaluation of periosteal fixation of lateral rectus and partial VRT for cases of exotropic Duane retraction syndrome.

Purpose: The purpose of this study is to evaluate the lateral rectus periosteal fixation and partial vertical rectus transpositioning (VRT) as treatment modalities to correct exotropic Duane retraction syndrome (Exo‑DRS). Materials and Methods: Prospective interventional case study of cases of Exo‑DRS with limitation of adduction. A total of 13 patients were subdivided into two groups. Six patients underwent only lateral rectus periosteal fixation (group A) and seven patients also underwent partial VRT (group B). Assessment involved prism bar cover test, abduction and adduction range, extent of binocular single visual field and exophthalmometry. These tests were repeated at 1 week, 1 month and 3 months post‑operatively and data analyzed. Results: The pre‑operative mean values and ranges were 26.2 Δ (22‑35) exotropia for group A and −21.3 Δ (14‑30) exotropia for group B. The post‑operative mean and range was +0.6 Δ esotropia (+20 to −8) for group A and 8 Δ (−2 to −20) exotropia for group B. Mean grade of limitation of abduction changed from −3.8 to −3.6 versus −3.6 to −2.8 and mean grade of limitation of adduction changed from −1.9 to −0.7 versus −1.5 to −0.5 in the groups A and B respectively. Mean binocular single visual field changed from 14.7° to 23.3° in group A and 11.8° to 26.4° in the group B respectively. Conclusion: Lateral rectus periosteal fixation is an effective surgery to correct the exodeviation, anomalous head posture and improving adduction in Exo‑DRS and partial VRT in addition is effective in improving abduction and binocular single visual fields.

Surgical Treatment of Face Turn, and Up and Down Shoot in Duane Retraction Syndrome

PURPOSE: To present the results of patients undergoing surgical treatment and determine clinical guidelines for the face turn, and up and down shoot in Duane retraction syndrome (DRS). METHODS: Thirty-four patients with DRS were treated with single horizontal rectus muscle recession, lateral rectus (LR) recession with Y-splitting, or LR recession with Y-splitting combined with medial rectus (MR) recession. The different treatment approaches were based on the angle of deviation in the primary position, versions, and ductions. In all patients, ocular alignment, face turn and up and down shoot were assessed preoperatively and postoperatively. RESULTS: The average standard deviation reduced from 18.5 +/- 6.5 prism diopters (PD) to 4.6 +/- 5.8 PD in horizontal rectus muscle recession and 14.4 +/- 5.5 PD to 1.6 +/- 2.8 PD in LR recession with Y-splitting. LR recession with Y-splitting combined with MR recession was effective in reducing globe retraction. Postoperative 4-mm LR recession with Y-splitting did not change ocular alignment; 7-mm LR recession with Y-splitting showed an average correction of 12.8 PD. Postoperatively, all patients presented a reduction in face turn; however, there was no statistically significant difference between the 2 procedures (Mann-Whitney U test, p = 0.620). CONCLUSIONS: Decisions regarding surgical approaches in DRS should be based on the amount of deviation in primary eye position, the expression of up and down shoot, and the degree of face turn. Surgeons planning Y-splitting of LR with MR recession must consider modifying the MR recession amount due to the smaller LR recession effect of Y-splitting compared with conventional LR recession.

Wildervanck syndrome associated with cleft palate and short stature.

We report a case of Wildervanck syndrome exhibiting Klippel-Feil anomaly, Duane retraction syndrome and deafness. Since the first case was reported in 1952, there have been more reports describing this triad, either complete or incomplete. Our patient had the complete triad of the syndrome along with cleft palate and short stature. Also, a review of the literature regarding this syndrome is presented here.

New considerations about Duane's syndrome: [revision] / Novas considerações sobre a síndrome de Duane: [revisão]

The author presents his arguments to state that the Duane's syndrome type III of the Huber's classification does not exist. He takes the chance of those arguments to show why the medial rectus muscle recession in Duane's syndrome with esotropia cripples the adduction more that it does in esotropias of other origins. He shows also why one must recess also the sound eye's medial rectus in Duane's syndrome with esotropia.

O autor expõe argumentos para afirmar que não existe a síndrome de Duane tipo III da classificação de Huber. Aproveitando esses argumentos, mostra por que o retrocesso do músculo reto medial em síndrome de Duane com esotropia prejudica a adução mais do que o faz em esotropias de outras origens. Mostra, também, por que se deve retroceder também o músculo reto medial do olho não afetado em síndrome de Duane com esotropia.

Surgical Results in Duane Retraction Syndrome

PURPOSE: To report the clinical characteristics and the results of a series of patients treated with various strabismus surgery techniques for Duane retraction syndrome (DRS). METHODS: Thirty-eight patients with DRS undergoing surgical treatment were retrospectively reviewed. In all patients, ocular alignment, abnormal head posture and ocular motility disturbance were assessed both preoperatively and postoperatively. The patients were treated with appropriate horizontal muscle recession, Y-splitting combined with horizontal muscle recession, medial rectus recession and lateral rectus resection. RESULTS: The incidence of DRS was greater in females and in the left eye. Type 1 was the most common, and esodeviation was seen most frequently in the primary position. The esotropic patients with DRS turned their faces toward the affected eyes, while the exotropic patients with DRS turned away from the affected eyes. The deviation in the primary position was reduced from an average of 15.0 prism diopters (PD) to 1.5PD. The face turn was reduced from an average of 17.5 degrees to 1.2 degrees. CONCLUSIONS: The primary deviation and abnormal head posture found in DRS can be improved by proper preoperative evaluation and adequate choice of surgical methods.

A Case of Pseudo-Duane's Retraction Syndrome With Old Medial Orbital Wall Fracture

We report a case of pseudo-Duane's retraction syndrome with entrapment of the medial rectus muscle in an old medial orbital wall fracture presenting identical clinical symptoms as Duane's retraction syndrome. A 15-year-old boy presented with persistent limited right eye movement since a young age. Examination showed marked limited abduction, mildly limited adduction, and globe retraction accompanied by narrowing of the palpebral fissure during attempted adduction in the right eye. He showed a right esotropia of 16 prism diopters and his head turned slightly to the right. A slight enophthalmos was noted in his right eye. A computed tomography scan demonstrated entrapment of the medial rectus muscle and surrounding tissues in an old medial orbital wall fracture. A forced duction test revealed a marked restriction of abduction in the right eye. A 5 mm recession of the right medial rectus muscle was performed. Postoperatively, the patient's head turn and esotropia in the primary position were successfully corrected, but there was still some limitations to his ocular movement. The importance of several tests such as the forced duction test and an imaging study should be emphasized in making a diagnosis for limitation of eye movement.

Síndrome de Wildervanck (Síndrome cérvico-óculo-acústica): [relato de caso] / Wildervanck syndrome: [case report]

O artigo relata o caso clínico de uma criança do sexo feminino, cinco anos de idade que apresenta a rara síndrome de Wilderwanck que consiste da síndrome de Klippel-Feil associada à síndrome de Duane e deficiência auditiva. O exame revelou alterações marcadas da coluna cervical, limitação bilateral da abdução, limitação da adução do olho direito associada à enoftalmia, diminuição da fenda palpebral, anisotropia em A, esotropia nas lateroversões e hipoacusia auditiva neurosensorial bilateral. Nossa paciente revelou a associação de síndrome de Klippel-Feil do tipo II com síndrome de Duane bilateral.

This article reports a case of a female five years old child with rare syndrome of Wilderwanck wich consists in a association of KlippelFeil syndrome and Duane syndrome and auditive hypoacusia. Ocular examination reveals alterations of cervical column, bilateral abduction limitations, aduction limitation of right eye associated with enophthalmos, palpebral fissure decrease, anisotropia in A, lateroversions esotropia and bilateral neurosensorial auditive hypoacusia. Our patient reveal an association of Klippel-Feil Syndrome type II with bilateral Duane Syndrome.

Duane's retraction syndrome and karyotypic abnormalities / 国际眼科杂志(Guoji Yanke Zazhi)

AIM:TO evaluate chromosome 8 abnormalities in Duane's retraction syndrome(DRS)type 1.METHODS:We evaluated chromosome 8 abnormalities in 29 consecutive cases of DRS type Ⅰ.DNA was isolated from the peripheral leukocytes of patients using a genomic DNA extraction kit,then D8S553 and D8S1797 markers used for polymerase chain reaction(PCR).RESULTS:None of the cases were positive for the two markers D8S553 and D8S1797 on chromosome 8 which were tested in our study.CONCLUSION:The possible cause of this finding is that DRS in our patients is more commonly sporadic rather than familial.We recommend study with more cases,other markers,and different chromosomes.

Bilateral Duane's Retraction Syndrome Treated with Bilateral Horizontal Transposition of Vertical Recti

PURPOSE: To report a male infant with bilateral Duane's retraction syndrome treated with bilateral horizontal transpositions of vertical recti to the lateral rectus muscle. METHODS: An 8-month-old boy showed bilateral Duane's retraction syndrome with esotropia of 45PD, severe limitation of abduction (-4 and -3.5 in each eye) and mild globe retraction in both eyes. It was assumed that he inherited the condition from his father through an autosomal dominant pattern. His father showed esotropia of 25PD with -4 limitation of abduction in the left eye and a left head-turn of 10 degrees. He also had +2 globe retraction, +1 upshoot and +2 downshoot in adduction of the left eye. Transposition of two vertical recti to the lateral rectus muscle was performed in both eyes of the boy. RESULTS: Nine months after surgery, the boy had achieved a microesotropia of 6PD in primary gaze with stereopsis of 3000 seconds of arc and considerable improvement in abduction (-2, -1.5 in each eye). The amount of correction of esodeviation was 39PD. This favorable state was maintained at follow-up 3.5 years after surgery. CONCLUSIONS: An 8-month-old boy who had esotropia of 45PD caused by bilateral Duane's retraction syndrome, underwent bilateral horizontal transpositions of vertical recti to the lateral rectus muscle. He achieved a microesotropia of 6PD in primary gaze and considerable improvement of abduction with only mild eyeball retraction.

A case of Mobius Syndrome with Duane's Retraction Syndrome

No abstract available.

A Case of Klippel-Feil Syndrome combined with Duane Retraction Syndrome: Incomplete Form of Wildervanck Syndrome

PURPOSE: Klippel-Feil syndrome is defined as the congenital fusion of two or more cervical vertebrae. The clinical features are low posterior hair line, short neck, and limitation of the movement of the head and neck. Wildervanck syndrome, also known as cervicooculoacoustic syndrome, is a rare genetic disorder that primarily affects females. The disorder is characterized by Klippel-Feil syndrome, Duane syndrome and hearing impairment at birth, although one of these symptoms may be lacking. This report describes a case of Klippel-Feil syndrome combined with Duane retraction syndrome, which can be defined as an incomplete form of Wildervanck syndrome. METHODS: A 15-year-old girl with congenitally fused cervical vertebrae at two levels, C2-C4 vertebrae and, C5-C7 vertebrae, was diagnosed as Klippel-Feil syndrome. Ophthalmologic evaluation was needed due to abnormality in ocular motility. RESULTS: Ophthalmologic examination revealed a visual acuity of 0.9 without correction in both eyes. Slit-lamp and fundus examination were normal. Ocular motility examination showed 14 prism diopters right esotropia in primary gaze, limited abduction, globe retraction, and narrowing of the palpebral fissure on adduction of the right eye.

The Clinical Features of Korean Patients with Duane's Retraction Syndrome

PURPOSE: To describe the clinical features of Duane's retraction syndrome (DRS) in Korean patients. METHODS: We retrospectively analyzed the 78 DRS cases that presented to our department between 1995 and 2004. The clinical features investigated included sex distribution, laterality, type of presentation, deviation in primary position, anomalous vertical movements, face turn, amblyopia and anisometropia. RESULTS: There were 38 (48.7%) affected males and 40 (51.3%) females. Left eye predominance (83.3%) was observed, as was type I presentation (85.9%). Orthotropia was found to be the most common primary position in 46 cases (59.0%). Face turn in unilateral DRS was noted in 13 patients (17.1%). There were 6 cases (7.7%) with anisometropia and 4 (5.1%) with amblyopia. CONCLUSIONS: The clinical manifestations of DRS in our study were different from those of equivalent Caucasian studies yet similar to those previously reported for Asian groups. Racial and regional differences were noted, for which further research is needed to elaborate the reasons and mechanisms.

A Case of Bilateral Duane's Retraction Syndrome Associated with Goldenhar's Syndrome

PURPOSE: Duane's retraction syndrome is a congenital eye movement disorder caused by innervational disturbance. It is rare that Duane's retraction syndrome is associated with Goldenhar's syndrome which shows systemic congenital malformations. We report a case of bilateral Duane's retraction syndrome associated with Goldenhar's syndrome, which was treated with strabismus surgery and excision of epibulbar lipodermoid. METHODS: A 14-month-old boy with exotropia showed severe limitation of adduction, mild limitation of abduction, globe retraction and palpabral fissure narrowing on attempted adduction in both eyes. He also had a diffuse small epibulabar mass under the lateral conjunctiva in the left eye. The angle of exodeviation was 45PD on alternate prism cover and uncover test. He had had cleft lip, multiple skin tags on the face and preauricular appendages since birth and undergone an operation for these abnormalities at age of 4 months. Bilateral 7.5mm recession of the lateral recti and excisional biopsy of epibulbar mass of the left eye were performed. RESULTS: A case of Duane's retraction syndrome associated with Goldenhar's syndrome was found. The patient obtained orthophoria in his primary gaze after bilateral recession of lateral recti. The epibulbar mass was consistent with lipodermoid on pathologic examination.

A case of congenital inverse Duane's retraction syndrome

Inverse Duane's retraction syndrome is very uncommon. Congenital cases are even more unusual. A 6-year-old girl with convergent squint along with severe restriction on abduction is described. On attempted abduction, a narrowing of the palpebral fissure, upshoot and retraction of the eyeball were observed. Brain and orbit MRI demonstrated no intracranial or intraorbital mass, fracture, or entrapment of the medial rectus. Forced duction test was strongly positive. The primary lesion was found to be a tight medial rectus with shortening and soft tissue contracture. Surgical tenotomy of the medial rectus led to successful postoperative motility, but some limitation at full adduction and abduction persisted. This is a case reported with congenital medial rectus shortening, suggesting that this condition may be one of the etiologies of the rare inverse Duane's retraction syndrome.