Síndrome de Duane: estudo retrospectivo de 41 casos / Fourty-one cases of Duane's Syndrome: retrospective study

Objetivos: Descrever as características de 41 pacientes acompanhados no departamento de Motilidade Ocular. Local: Hospital das Clínicas da Faculdade de Medicina da USP. Pacientes e Métodos: Estudo retrospectivo de 41 casos de Síndrome de Duane quanto às características clínicas oculares. Resultados: 22 pacientes do sexo masculino e 19 do feminino. Predominância do tipo I (75,6 por cento) e de acometimento do olho esquerdo (70,7 por cento). 92,6 por cento dos casos era bilateral. 24,4 por cento dos pacientes foram submetidos à cirurgia, com 70 por cento de bons resultados. Conclusão: Nosso estudo condiz com o relato na literatura quanto às características clínicas desta síndrome.

Duane's retraction syndrome: literature review.

BACKGROUND: Duane's retraction syndrome (DRS), also known as Stilling-Turk-Duane syndrome, is defined as a congenital miswiring of the lateral and medial recti muscles, resulting in an impaired ocular motility syndrome that includes palpebral fissure narrowing. The incidence of DRS is approximately 1% of the total cases of strabismus. Eighty percent of cases are unilateral and characterized by either limited abduction, limited adduction, or both. CASE REPORT: A 21-year-old man came to the clinic for a routine ocular examination without symptoms. A review of the history uncovered the presence of congenital, type I Duane's retraction syndrome. The examination demonstrated orthophoria in primary gaze, an abduction deficit O.S., and left globe retraction with palpebral fissure narrowing on right gaze O.S. MANAGEMENT: In most cases of DRS the eyes are straight in primary position and there is no amblyopia. Amblyopia, when present, is usually the result of anisometropia and not strabismus. Because our patient had no symptoms of diplopia in primary gaze (orthophoria) or in attempted right gaze (due to suppression of the left eye with abduction), prismatic and/or surgical management were not indicated, since the patient was free from binocular and cosmetic abnormalities. CONCLUSION: DRS is characterized by abnormal development of the cells in the abducens nucleus (CN VI), resulting in restricted or absent abduction and erroneous innervation of the lateral rectus by branches emanating from oculomotor nuclei (CN III). Management may include orthoptics, surgery, or monitoring.

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.

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.

A case of Duane's retraction syndrome with multiple congenital malformations.

Duane's retraction syndrome is associated with various ocular and systemic abnormalities. We report a case with bilateral Duane's retraction syndrome type I accompanied by renal agenesis, vesico-ureteral reflux, patent ductus arteriosus, and external ear malpositioning. Because of disabling consequences, we recommend a thorough physical examination and screening for renal and cardiac abnormalities in all patients presenting with Duane's reaction syndrome.

[Crocodile tears and retraction syndrome]. / Krokodilstränen und Retraktionssyndrom.

BACKGROUND: An association between crocodile tears and Duane's syndrome has been described in more than 50 cases. PATIENT: A 29-year-old man had tearing of his right eye whenever he ate something sour. At psychic weeping his right eye remained dry. In addition, he had Duane's retraction syndrome, also in the right eye. CONCLUSION: In this case of congenital crocodile tears with an ipsilateral Duane's syndrome the normal function of the facial muscles suggests that the abnormal coupling between the gustatory nucleus and the lacrimal gland is not located in the facial nerve proximal to the Ganglion geniculi, as in most acquired cases. Rather, the abnormal coupling may be located in the brainstem. The association with Duane's syndrome indicates that abnormal innervational coupling is present also in the ocular motor domaine.

[Stilling-Duane retraction syndrome; surgical options]. / Le syndrome de rétraction de Stilling-Duane; options chirurgicales.

BACKGROUND: The Stilling-Duane's retraction syndrome is a congenital abnormality of ocular movements. In order to maintain simple binocular vision, most patients adopt an abnormal head posture; this can be corrected by surgery. MATERIALS AND METHODS: We carried out a retrospective study of the clinical files of 56 patients who presented with Duane's syndrome and had been examined at the Strabological Department of the Lausanne Eye Clinic between 1974 and 1993. RESULTS: Our group study is comparable to that found in the literature as regards the distribution according to sex (45% of males, 55% of females), laterality (75% of the cases with disease predominance in the left eye, 18% in the right eye, and 7% with bilateral, symmetrical disease), and associated anisometropia (18%) or amblyopia (12.5%). Approximately 1 of 5 patients sought medical advice only after 20 years of age. A compensating head turn (towards the ill side in patients with esotropia and towards the healthy side in patients with exotropia) was found in 71% of the cases. Our surgery (10 patients with a mean follow-up of 8 months) resulted in a decreased head turn in 7 cases. CONCLUSIONS: The purpose of this surgery is to improve the head turn without worsening retraction. This can be achieved with simple muscle recessions.

[Unusual forced head postures in congenital paralysis of the superior oblique muscle]. / Ungewöhnliche Kopfzwangshaltungen bei kongenitaler Parese des M. obliquus superior.

BACKGROUND: With congenital superior oblique palsy, an abnormal head posture which is different from the usual one (head tilt) has hardly been described. We performed this study to point out the spectrum of atypical head posture and its operative treatment procedures. PATIENTS: The charts of all 103 patients with congenital superior oblique palsy which were seen at our clinic between 1983 and 1993 were reviewed. RESULTS: 13 patients (13%) had an atypical head posture. Group 1: patients with a face turn to the non-involved side (n = 5). The vertical deviation increased abruptly starting from the primary position; is was comitant in adduction. Combined surgery of the obliques muscles was most often performed (n = 3). Group 2: patients with a vertical abnormal head posture. 3 Patients presented with a chin elevation. Their vertical deviation was incomitant, it was smallest in downgaze. An isolated recession of the inferior oblique muscle was performed in all cases. One patient had a chin depression; she also had an esotropia in downgaze. A recession of the inferior oblique muscle was performed. Group 3: patients with a face turn and a chin elevation (n = 4). Vertical deviation was maximal in adduction and was smallest in the lower and temporal field of gaze. We performed first a recession of the inferior oblique muscle and then-if necessary-a tuck of the superior oblique muscle or a recession of the contralateral inferior rectus muscle. CONCLUSIONS: An atypical head posture can occur in about 10% of cases. Its cause can be explained after checking the incomitance of the vertical deviation and the motility disorder. These parameters also determine the operative procedures.

Inervación paradóxica: casos curiosos / Paradoxical Innervation: curious cases

Cinco casos curiosos de inervación paradóxica con compromiso de los movimientos oculares, palpebrales y pupilares permiten teorizar acerca de su etiología. Un Síndrome Disquinético (Misdirection Syndrome) completo, con alteraciones de la motilidad ocular, de los movimientos palpebrales y pupilares, a consecuencias de una herida penetrante orbitaria, aporta sólidos argumentos en favor de un mecanismo periférico de regeneración aberrante del III Par. Se analizan además los conceptos de transmisión neuronal efáptica y de cromatolisis que induciría una reorganización de las sinapsis nucleares. Un caso de retracción paradóxica del párpado superior derecho durante la infralevoversión permite suponer la existencia de un escape supranuclear del estímulo inervacional. Un paciente con ptosis bilateral y retracción del globo ocular derecho durante la elevación sugiere una alteración inervacional supranuclear que compromete grupos musculares diferentes. Otro paciente con retracción del párpado superior izquierdo, hipofunción del oblicuo inferior izquierdo y retracción del globo ocular durante la supradextroversión seguida de ptosis, sobreacción del oblicuo superior y protrusión del globo ocular izquierdo en infradextroversión recuerda un tipo de inervación paradóxica similar a la del Síndrome de Duane, pero comprometiendo grupos musculares diferentes. Un caso curiosísimo de endotropia bilateral al abrir la boca que cede solamente al cerrar la mandíbula hace pensar en un escape inervacional de una vía normal hacia otra o la existencia de un padrón oculomotor diferente, sugerente de regresión a un esquema motor atávico

Congenital unilateral adduction deficit with simultaneous abduction. A variant of Duane's retraction syndrome.

Congenital adduction deficit not associated with other signs of oculomotor nerve weakness occurs infrequently. Type 2 Duane's retraction syndrome accounts for some of these cases. The authors identified three children with unilateral adduction deficits and simultaneous abduction of the eyes on attempted lateral gaze into the field of action of the apparently paretic medial rectus muscle. This is the first report of a series of patients with this condition previously termed simultaneous or synergistic divergence. All patients had a large exotropia, and two had a horizontal face turn away from the involved eye. A large (14 mm) lateral rectus muscle recession done on one of these children reduced the face turn but did not eliminate the simultaneous abduction. Clinical, intraoperative, and electromyographic data suggest that this condition is an unusual variant of type 2 Duane's syndrome.

Further observations on the auditory brainstem response in Duane's syndrome.

Auditory brainstem responses were recorded in 16 patients with Duane's syndrome. There were no significant differences in the mean wave and interwave latencies between the patients and 16 neurologically and audiologically normal control subjects, although 2 patients had responses from one side that were of increased latency. A consistent relation between the auditory brainstem response and the affected eye was not found; therefore, the data from this study do not support the findings of a previous report on this subject.