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.
Biopsy ; Cleft Lip ; Conjunctiva ; Duane Retraction Syndrome* ; Exotropia ; Humans ; Infant ; Male ; Ocular Motility Disorders ; Parturition ; Skin ; Strabismus

We evaluated retrospectively the clinical records of 46 cases of Duanes retraction syndrome. The purpose of this study is to demonstrate a clinical characteristics of this syndrome and improvement of abnormal head posture and degree of deviation in 14 cases undergoing operation. Unilateral manifestation was in 42 cases and 4 cases were involved bilaterally. Left eye was involved predominantly in 29 cases (63.0%). The frequencies analyzed according to Hubers classification were that Type I was 64.3%, Type II 7.1%, and Type III 28.6% in unilateral cases. Of unilateral cases, compensatory face turn was exhibited in 65.2% of Type I, 100% of Type II, and 60.0% of Type III. In primary position, 44.4% of Type I had esodeviation, and 100% of Type II ad 83.3% of type III had exodeviation. The major concerns of most patients or their parents were the correction of strabismus and gaze limitation, but they hardly recognized abnormal head posture. The correction of abnormal head posture and strabismus was achieved in 78.6% postoperatively.
Classification ; Duane Retraction Syndrome* ; Esotropia ; Exotropia ; Head ; Humans ; Parents ; Posture* ; Retrospective Studies ; Strabismus

To investigate the clinical similarity between the siblings, we retrospectively studied the medical records of 64 patients, 32 pairs. 31 pairs showed the similar entity of strabismus between siblings. There was exotropia in 22 pairs, esotropia in 5 pairs, bilateral superior obliqre palsy in 2 pairs, exotropia, esotropia and Duane`s retraction syndrome in 1 pair. One was exotropia and the other was exotropia and Duane`s retraction syndrome in one pair. One pair was bilateral supperior obliqre palsy and esotropia and the other was right gypertropia and exotropia. The difference in the amount of deviation was less than 10 prism diopter in 23 pairs. There was no association between the strabismic siblings in refractive anomaly, amblyopia, and anisometropea. The surgical results were similar in 7 pairs of siblings among 8 pairs who underwent corrective operations.
Amblyopia ; Duane Retraction Syndrome ; Esotropia ; Exotropia ; Humans ; Medical Records ; Paralysis ; Retrospective Studies ; Siblings* ; Strabismus*

To investigate the clinical similarity between the siblings, we retrospectively studied the medical records of 64 patients, 32 pairs. 31 pairs showed the similar entity of strabismus between siblings. There was exotropia in 22 pairs, esotropia in 5 pairs, bilateral superior obliqre palsy in 2 pairs, exotropia, esotropia and Duane`s retraction syndrome in 1 pair. One was exotropia and the other was exotropia and Duane`s retraction syndrome in one pair. One pair was bilateral supperior obliqre palsy and esotropia and the other was right gypertropia and exotropia. The difference in the amount of deviation was less than 10 prism diopter in 23 pairs. There was no association between the strabismic siblings in refractive anomaly, amblyopia, and anisometropea. The surgical results were similar in 7 pairs of siblings among 8 pairs who underwent corrective operations.
Amblyopia ; Duane Retraction Syndrome ; Esotropia ; Exotropia ; Humans ; Medical Records ; Paralysis ; Retrospective Studies ; Siblings* ; Strabismus*

The retraction syndrome in its classic form is characterized that severe limitation of abduction, slightly limitation of adduction, globe retraction and narrowing of the palpebral fissure on adduction, frequently associated elevation or depression in adduction, and convergency insufficiency. While many authors reported this type, there were few reports about inverse Duane's retraction syndrome, as exotropia in primary position, severe limitation of adduction. globe retraction and narrowing palpebral fissure on abduction. So the authors present a case of inversed Duane's retraction syndrome.
Depression ; Duane Retraction Syndrome* ; Exotropia

Alternating recurrent painful ophthalmoplegia is caused by various neurological conditions including Tolosa-Hunt syndrome, sellar mass, and parasagittal meningioma. We experienced a rare case of recurrent painful ophthalmoplegia occurring on the contralateral side as a manifestation of idiopathic hypertrophic tentorial pachymeningitis. We propose that idiopathic hypertrophic pachymeningitis should be considered in the differential diagnosis of alternating recurrent painful ophthalmoplegia.
Diagnosis, Differential ; Meningioma ; Meningitis* ; Ophthalmoplegia* ; Tolosa-Hunt Syndrome

PURPOSE: To assess the MR findings of Tolosa-Hunt syndrome and to evaluat differential point of cavernous sinus lesions. MATERIALS AND METHODS: Eleven cases of Tolosa-Hunt syndrome were evaluated by MRI with specific regard to the shape of cavernous sinus, signal intensity, and pattern of enhancement. Other associated findings were also anlaysed. RESULTS: Two patients had normal MR features of the cavernous sinus. When compared with the contralateral normal cavernous sinus, the involved cavernous sinus was enlarged in six of the nine patients. The outer dural margin was convex and bulged laterally in 6 cases, flat in 2 cases, and concave in 1 case. Of the nine patients, five had iso-signal intensity and four had low signal intensity relative to gray matter on short TR/short TE. Three had isosignal intensity and 6 were not detectale on long TR/short and long TE pulse sequence. Contrast enhancement was seen in 8 cases ;7 cases showed homogeneous enhancement, 1 case heterogeneous enhancement. CONCLUSION: In the appropriate clinical setting of painful ophthalmoplegia, MR findings of cavernous sinus abnormality that consist of iso to low signal intensity on short TR/short TE images and isointense or undetectale mass on long TR/short TE or long TR/Iong TE images may suggest the dignosis of Tolosa-Hunt syndrome.
Cavernous Sinus ; Humans ; Magnetic Resonance Imaging ; Ophthalmoplegia ; Tolosa-Hunt Syndrome*

The authors reports one case of Tolosa-Hunt syndrome on a housewife, which was differentiated from other causes of painful ophthalmoplegia by several tests including routine labs, carotid angiogram and brain CT. In orbital venography, 3rd portion of the ipsilateral ophthalmic vein was not visualized. There was a prompt remission on steroid theraphy.
Brain ; Ophthalmoplegia ; Orbit ; Phlebography ; Tolosa-Hunt Syndrome* ; Veins

Of 22 cases of strabismus having surgical operations designed on the basis of A-scan ultrasonography, 19(86%) had less than 10 prism diopters eso-or exodeviation at least 2 months postoperatively; 19 exo-and 3 esodeviations were included in the series. Among 3 cases which had more than 10 prism diopters deviation at final follow up, a true divergence excess type exotropia and a nonaccommodative convergence excess type esotropia were included. With the exception of these 2 cases, 19 among 20 basic tropias(95%) had less than 10 prism diopters deviation. A-scan ultrasonography was done preoperatively to find the diameter and thus the circumference of the globe. This was used with the angle of deviation to estimate the amount of recession and/or resection necessary to correct the deviation. Attention was drawn not to exceed the safe range of recession by considering the oculomotor equator.
Esotropia ; Exotropia ; Follow-Up Studies ; Ocular Motility Disorders ; Strabismus* ; Ultrasonography*

In this study, we reviewed 46 consecutive patients with diagnosis of Duane's retraction syndrome at the Seoul National University Hospital from April, 1987 to October, 1995. The following characteristics were reviewed - sex, laterality, clinical type, primary position alignment, anomalous vertical movements, head turn, fusion and amblyopia. The incidence in each sex showed no difference(male: 54%, female:46%). Ninety-six percent of cases had a unilateral manifestation and 4% of cases had bilaterality. The left eyes were involved in 70% of cases and the right eyes in the 26% of cases. Type I was the most common type(96%) and the esodeviations were the most frequent in primary position. Head turns were observed in 77% of cases and the direction of head turns were toward the involved eyes in esodeviations, and away from the involved eyes in exodeviations regardless of the clinical types. Among 26 patients who underwent the fusion test, seventeen patients(65%) had fusion and that was more common in patients with exodeviation(83%) than others.
Amblyopia ; Diagnosis ; Duane Retraction Syndrome* ; Esotropia ; Exotropia ; Head ; Head Movements ; Humans ; Incidence ; Seoul