Duane's Retraction Syndrome

ABSTRACT: The condition of Duane's retraction syndrome is reviewed, and data presented from 5 cases.     

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.     

Midline orofacial cleft defects in association with type 1 Duane's retraction syndrome

Anomalies of ocular motility associated with Duane's retraction syndrome (DRS) have often been perceived as isolated phenomena arising as a result of congenital ocular miswiring between the lateral and medial recti muscles. Interestingly, the reporting of concomitant ocular and systemic anomalies arising in the setting of DRS discounts this narrow perception and highlights the importance of a thorough investigation for non‐ocular comorbidities. A 37‐year‐old Caucasian male presented for a routine ocular examination with complaints of gradual reduction in uncorrected near vision. Our testing confirmed the characteristic deficit of abduction, palpebral fissure narrowing and globe retraction consistent with a type 1 Duane's defect of the right eye. A midline cleft lip and palate were also noted in the absence of signs of nuchal rigidity/reduced range of motion in the cervical spine, spinal misalignment, deafness, optic nerve anomalies, hypertelorism or altered mental state. Awareness of key comorbidities and the importance of thorough clinical evaluation with appropriate supplemental testing and consultation with a paediatric ophthalmologist are warranted in non‐isolated presentations of DRS.     

Duane's retraction syndrome and spastic paraparesis

Duane's retraction syndrome (DRS) is a neurogenic eye movement abnormality frequently associated with other congenital defects involving ocular, skeletal and neural structures. The authors report on a patient who had DRS associated with a severe spastic paraparesis, which progressed from childhood until young adulthood with subsequent stabilization. The patient had severe limitation of abduction of the left eye, narrowing of the palpebral fissure, and globe retraction in adduction. Neurological examination revealed a severe spastic paraparesis. Extensive clinical neurophysio-logical evaluation, including electromyography, nerve conduction studies, somatosensory evoked responses and transcranial magnetic stimulation, was consistent with a severe myelopathy. Magnetic resonance imaging of the spinal cord demonstrated significant thinning of the cervical and thoracic spinal cord. This previously unreported association is unlikely coincidental, given the rarity of both findings, and may be explained by the abnormal development of neural structures during a critical period of gestation.     

Congenital innervation dysgenesis syndrome (CID)/congenital cranial dysinnervation disorders (CCDDs)

Congenital loss of innervation to the extra-ocular muscles (EOMs) can have a profound effect on the target muscle. This has been well recognised in Duane''s retraction syndrome. However, it has been less emphasised in other congenital oculo-motor disorders. Such congenital ocular motor defects have been expanded to include DRS, congenital fibrosis of EOMs, monocular elevation defect, Möbius syndrome, as well as several other non-ocular muscles supplied by cranial nerves such as facial muscles. Such loss of innervation to motor muscles can be unified as a defined clinical entity, which can be labelled as congenital innervation dysgenesis syndrome or CID for short. CID may also affect other muscles supplied by nerves other than the cranial nerves and may be sensory as well as motor.     

Electron microscopic study of Duane's syndrome,endocrine ophthalmopathy and other myopathies with strabismus

The lateral and the medial rectus muscles from children with special forms of strabismus are described ultrastructurally. In Duane's retraction syndrome, the lateral rectus muscle was composed of mature and immature collagen fibrils and no nerve structures could be identified in the whole bulk of tissue. the medial rectus was partly fibrotic and partly composed of muscle fibers showing various stages of degeneration. Nerve axons were seen adiacent to the muscle cells. In endocrine ophthalmopathy and rod myopathy, the muscle fibers showed myofibrillar degeneration and abnormalities of the Z-band and also an increase in the amount of the interstitial collagen tissue. It is concluded that the absence of nerve structures in the area of the totally fibrotic lateral rectus muscle supports the view that Duane's syndrome is related to a neurogenic abnormality.     

Unilateral acquired Brown's syndrome in systemic scleroderma: An unusual cause for diplopia

Brown''s syndrome can be congenital or acquired with multiple causes. It has been described as a ocular complication in various rheumatic and nonrheumatic diseases. We describe a case of 27-year-old female patient with 5 years old history of systemic scleroderma who developed vertical diplopia, a left head tilt, and restriction of left eye on elevation in adduction. The patient responded to systemic steroids with resolution of diplopia.     

Von Pflugk (Dresden): Contribution to the Technique of Strabismus Operations,Suturing Anteriorly and Posteriorly

Up to 25% of the eyes affected with Duane's syndrome show upshoot and/or downshoot in attempted adduction. This is thought to be caused by the tight horizontal muscles both contracting in attempted adduction (leash or bridle effect) due to abnormal firing of the lateral rectus muscle through which the globe slips beneath the muscle. Surgical procedures successful in correcting the very disturbing cosmesis of gaze are posterior fixation, recession of the horizontal rectus muscles or splitting of lateral recti muscles insertion. The authors report on a 27-year-old male with Duane's syndrome type III with extreme upshoot in adduction. After recession of both horizontal muscles the upshoot on attempted adduction was no longer present postoperatively. At one year follow-up a very discrete upshoot in attempted adduction could be documented. The resolving of the upshoot postoperatively could be explained by the bridle mechanism alone. The low grade upshoot in attempted adduction at one year follow-up could have been caused by new adhesions of the check ligaments and Tenon's capsule at the level of the lateral rectus muscle resulting from surgery itself. The possibility of an anomalous cyclovertical innervation playing a role in the vertical anomalous movements as Duane originally hypothetized cannot be excluded.     

Effects of Duane's retraction syndrome on sensory visual development

PURPOSE: To study the effects of Duane's retraction syndrome on sensory visual development. METHODS: Monocular and binocular visual function and ocular motility have been studied and pattern reversal visual evoked potentials recorded from 22 patients with Duane's syndrome aged from 4 to 55 years. RESULTS: Sixteen of the patients maintained binocular single vision using an abnormal head posture. All had normal visual acuities in both eyes. The 12 adults in this group had a mean stereoacuity of 78 seconds of arc with the mus stereotest and 101 seconds of arc with the TNO test. Both these values were significantly worse than for normal adults with a similar age range. The binocular VEPs from these patients showed enhancement of the binocular P100 VEP amplitude compared to the mean monocular P100 amplitude when they used their head posture but, unlike in normal subjects, this binocular enhancement was not reduced significantly after the age of 5. Six patients had lost binocular function and had a manifest convergent squint. Of these, 4 were anisometropic. VEPs in this group showed mildly delayed P100 latencies in the affected eye with asymmetric amplitudes even though their amblyopia had been successfully treated by patching and only one patient had a substantially reduced acuity. In neither group was there any relationship between the degree of restriction of ocular motility and the sensory changes. CONCLUSION: Patients with Duane's syndrome who retain binocular single vision show abnormal binocular visual maturation after the age of 5 years. This results in reduced stereoacuity in the absence of amblyopia.     

Hypertropia following Spontaneous Resolution of Brown's Syndrome

Purpose

To report a case of bilateral Brown''s syndrome with unilateral spontaneous resolution causing hypertropia and significant head tilt.

Case Report

A 3 ½-year-old girl presented with bilateral typical Brown''s syndrome and orthophoria in the primary position; she presented with unilateral resolution of right Brown''s syndrome 6 months later, causing right hypertropia and gradually deteriorating left head tilt. She benefited from right superior rectus muscle recession to help correct her head posture.

Conclusion

This is the first report of a patient presenting with known bilateral Brown''s syndrome with subsequent documented unilateral resolution causing a significant hypertropia of the resolved side and contralateral head tilt. Our case provides evidence in support of Clark and Noël''s [Can J Ophthalmol 1993;28:213–216] hypothesis that patients who present with unilateral Brown''s syndrome and contralateral inferior oblique muscle overaction might originally have had bilateral Brown''s syndrome with spontaneous resolution of 1 side only.Key Words: Brown''s syndrome, Hypertropia, Spontaneous resolution of Brown''s syndrome     

Optic Tract Syndrome,Horner's Syndrome,and Trochlear Nerve Palsy due to Suprasellar Germinoma

We report a 23-year-old man with suprasellar germinoma accompanied by optic tract syndrome, Horner's syndrome, and trochlear nerve palsy. Neuroimaging demonstrated that the optic tract, the hypothalamus on the left side, and the trochlear nerve in the subarachnoid space were involved by a germinoma. In addition to neuroimaging findings, detailed observation of various ocular symptoms was highly useful for determining the localization of the germinoma.     

Ophthalmic complications including retinal detachment in hyperimmunoglobulinemia E (Job's) syndrome: Case report and review of literature

Hyperimmunoglobulinemia E (Job''s) syndrome is characterized by markedly increased levels of immunoglobulin E, recurrent cutaneous and systemic pyogenic infections, atopic dermatitis, and peripheral eosinophilia. Although ocular involvement in Job''s syndrome is rare, there are reports of keratoconus, staphylococcal chalazia with blepharitis, and Candida endophthalmitis by various authors. We present the first case report of retinal detachment with complicated cataract in Job''s syndrome.     

Anterior Segment and Retinal Pigmentary Abnormalities in Arteriohepatic Dysplasia

Arteriohepatic dysplasia (AHD, Alagille's syndrome) is presumed to be one of the six known familial intrahepatic cholestatic syndromes, all of which present with neonatal jaundice or failure to thrive, or both. Accurate early diagnosis of the proper syndrome is important, as arteriohepatic dysplasia has a good prognosis, whereas the other syndromes usually lead to death in infancy or early adulthood. Posterior embryotoxon was found in all five of our patients and may be one of the hallmarks of this syndrome. Axenfeld's anomaly was present in three of five patients, and retinal pigmentary abnormalities were found in four of five patients. Variable abnormalities were found in the cardiovascular system, bones, central nervous system, kidneys, endocrine system, and body habitus. Inheritance may be autosomal dominant as vertical transmission was documented in one family. Liver function improves during the first five years of life so that only the ocular and skeletal signs may be present in adults.     

The Spectrum of Associated Ocular and Systemic Malformations

The cases of Peters' anomaly seen over the past decade, at Hospital Ste-Justine in Montreal, are reviewed. Associated ocular anomalies were observed in 50% of cases while 60% of patients presented with associated systemic defects. It is clear, from these patients and those reported in the literature, that Peters' anomaly can be an isolated condition, or part of distinct syndromes: the Krause-Kivlin syndrome or the Peters'-plus syndrome. The authors emphasize the importance for the ophthalmologist to recognize these possibilities if proper management is to be provided.     

Modified thin manual Descemet stripping endothelial keratoplasty with air-guided, non-pachymetric donor lenticule dissection: outcomes of graft thickness and complication rate

AIM: To study the different treatment modalities needed in cases of Duane''s Retraction Syndrome. METHODS: This prospective study undergone in more than four years, in Alexandria, included 238 patients of Duane''s Retraction Syndrome, including type I, 162 patients (68%), type II 12 patients (5%) and type III 64 patients (27%). Surgery was indicated in 98 (41%) of them, to eliminate abnormal head posture, deviation of the eye in primary position, severe retraction of the globe or cosmetically unacceptable upshoot with attempted adduction. RESULTS: Type I was the most common and type II was the least. Females were predominant in this study, constituting 125 patients (52.5%), and males 113 patients (47.5%). Left eye was more affected, in 110 patients (46.2%), right eye in 91 patients (38.2%) and bilateral in 37 patients (15.6%). Amblyopia was found in 27 patients (11.3%) and treated in 13 patients under 10 years of age, by patching the normal eye. Ninety eight patients (41%) were operated , the results were most satisfactory and a nomogram is followed in the surgical plan CONCLUSION: The surgical management was needed in less than half of the cases and should be planned for every case individually according to the clinical findings, planned nomogram and modified intraoperatively according to the anatomical findings during surgery.     

Duane's retraction syndrome and juvenile Batten's disease: A new association?

Background: Although Duane's retraction syndrome (DRS) represents less than 5% of strabismus patients presenting to an ophthalmology department, it is a difficult management problem that is often poorly treated. The developmental defect has been isolated to early in the embryonic period, but to date a chromosomal location is still uncertain. Neuronal ceroid lipofuscinosis (NCL) or Batten's disease is a lysosomal storage disease with autosomal recessive inheritance, which has been categorized according to the age of onset of symptoms. Methods/Results: We report on a patient with DRS who developed juvenile Batten's disease. Conclusions: These two abnormalities can both be inherited, but their association has not been previously documented.     

Overview and Diagnosis of Acute Retinal Necrosis Syndrome

Acute retinal necrosis (ARN) syndrome, also known as Kirisawa's uveitis, is one of the most serious ocular diseases, and is characterized by a combination of peripheral, confluent, necrotizing retinitis, retinal arteritis, and intraocular inflammation. ARN syndrome is caused by the herpesvirus family, including herpes simplex virus (HSV) and varicella-zoster virus (VZV). The diagnosis of ARN syndrome is fundamentally based on clinical appearance and the demonstration of viral infection. Recently, polymerase chain reaction techniques permit detection of very small amounts of viral DNA in intraocular specimens. This knowledge can help in both the diagnosis and design of therapeutic strategy for ARN syndrome. Here we review the clinical presentation and the current advances in the diagnosis of ARN syndrome.     

Polytetrafluoroethylene in the surgery of cases with severe limitation of abduction Long-term results

Background: In cases of severe palsy or paralysis of eye muscles, it is sometimes necessary to extensively weaken the homolateral antagonist. Large recessions reduce the ocular motility by shortening the arc of contact between muscle and sclera. The lengthening of the muscle tendon with Fascia lata, Lyo-Dura®, or the free transplantation of an excided piece of autologous eye muscle tendon does not ensure predictable precise results. We used polytetrafluoroethylene (PTFE, Gore-Tex®) for seven years and report on our results. Method: A new technique is introduced in which the muscle is lengthened by PTFE. Ten patients (7 sixth nerve palsies, 1 strabismus fixus, 2 Duane’s retraction syndromes) underwent lengthening of the medial rectus muscle with a Gore-Tex® soft tissue patch between 8 and 14mm in size. The two patients with the Duane's retraction syndrome were not operated on the rectus externus muscle. All other patients had a resection of the paralytic muscle. Results: In most cases, good results were obtained: binocular single vision in primary head position within a small field of fusion. Histological examination of a specimen of Gore-Tex® four years after surgery showed an excellent integration of the graft into the tissue. Fibrous connective tissue wrapped the Gore-Tex® like a capsule. There were no signs of inflammation or graft reaction. Conclusions: PTFE (Gore-Tex® Soft Tissue Patch) is a suitable material for complicated strabismus surgery. Main advantages: availability at any time in the operation theater; high biocompatibility; form stability; and no diminution of operative results.     

Parinaud's Oculoglandular Syndrome Due to Herpes Simplex Virus Type 1

Purpose: To describe the clinical findings in a patient with Parinaud's oculoglandular syndrome as an uncommon manifestation of primary herpes simplex virus type 1 (HSV-1) infection. Methods: The clinical course, the laboratory findings, the therapy, and the outcome regarding a 14-year-old girl are described. Results: The culture and PCR detection of HSV-1 on conjunctiva and skin scrapings, along with seroconversion to HSV, confirmed the etiology. The oral and local acyclovir therapy led to a prompt improvement in the patient's symptoms. Conclusion: The solitary ocular-glandular syndrome due to HSV-1 primary infection has never been reported before. Parinaud's oculoglandular syndrome is found in 5% of patients with cat-scratch disease and only on rare occasion associated with other conditions. Herpetic infection should be considered in the differential diagnosis of young patients with conjunctivitis, periorbital swelling, and painful preauricular and submandibular lymphadenopathy, combined with systemic symptoms of malaise and fever.     

Fotofobia y dolor neuropático en el síndrome de Sudeck

The case is presented of a 14 year-old patient diagnosed with Sudeck's syndrome secondary to uneventful foot trauma. The patient complained of decreased visual acuity along with photophobia and intense ocular pain not correlated with the exploratory findings.Sudeck's syndrome is an idiopathic neuropathic inflammatory disease characterised by disproportionate pain, unrelated to a previous traumatic event, which can evolve to severe and generalised pain. A new explanation has recently described this as “neuropathic eye pain” for those patients with severe eye pain that do not correlate with clinical signs. In the case presented here, the pain became widespread and led to photophobia and very intense ocular neuropathic pain. It is believed that this was the cause of the visual decrease presented by this patient. It is proposed that the Sudeck syndrome should become part of the differential diagnosis of neuropathic eye pain.