Síndrome de Duane: a propósito de un caso / Duane syndrome: a case report

El síndrome de Duane es una enfermedad poco frecuente en la cual se observa retracción del globo ocular y limitación de los movimientos horizontales. Presentamos el caso de un paciente de 11 años que acudió a Urgencias debido a una desviación ocular supuestamente de reciente instauración. En la exploración se objetiva un déficit en la abducción y la aducción, así como cierre de la fisura palpebral en la aducción forzada. Se diagnostica de síndrome de Duane tipo III y se decide manejo conservador con observación y seguimiento (AU)

Duane syndrome is a rare condition, whose clinical presentation involves retraction of the eyeball upon attempted abduction, and limitation of horizontal movements. We present the case of an 11-year-old patient who presented to the Emergency Department with a recent-onset ocular deviation. Examination revealed a deficit in abduction and adduction, as well as closure of the palpebral fissure in forced adduction. The patient was diagnosed with Duane syndrome type III, and conservative management with observation and follow up was decided. (AU)

Afecciones de la conjuntiva en pacientes con prótesis ocular / Conjunctival diseases of patients with ocular prothesis

OBJETIVOS: determinar las afecciones conjuntivales en pacientes con prótesis ocular. MÉTODOS: estudio observacional descriptivo longitudinal prospectivo, conformado por 62 pacientes (65 cavidades anoftálmicas) quienes acudieron al Servicio de Oculoplastia del Instituto Cubano de Oftalmología "Ramón Pando Ferrer" en el período de mayo a diciembre de 2010. Se analizaron la edad, el sexo, las causas de la pérdida ocular, el ojo afectado, la afección conjuntival, el tipo de cavidad, el tiempo de uso protésico, el resultado microbiológico, el manejo higiénico y la respuesta al tratamiento. RESULTADOS: se presentaron 23 pacientes de 40 a 59 años (37,1 %), de los cuales el sexo masculino mostró el 66,1 %. El traumatismo reveló el 40,3 % y el ojo derecho el 50 %. El 33,8 % presentó conjuntivitis alérgica; las retracciones de la conjuntiva reportaron el 18,5 %. El 67,5 % correspondió a cavidades atípicas con un tiempo de más de 366 días en el uso de la prótesis ocular. El resultado microbiológico positivo en 55 cavidades fue de 84,6 %. Se aisló el Staphylococcus aureus en el 70,9 % y de ellos el 41,5 % se efectuó con manejo diario de la prótesis. La respuesta al tratamiento fue buena en el 76,4 %. CONCLUSIONES: las afecciones conjuntivales de los pacientes con prótesis ocular constituyen un problema de salud. Asimismo, la mayoría de las cavidades anoftálmicas son atípicas en adultos, con pérdida del globo ocular por traumatismo. La conjuntivitis alérgica y la retracción conjuntival son las afecciones más frecuentes en los pacientes con prótesis ocular de más de un año de uso, por la presencia de Staphylococcus aureus a consecuencia de mayor manipulación higiénico protésica, aunque en la mayoría de los casos existe buena respuesta al tratamiento.

OBJECTIVES: to determine the conjunctival diseases of patients with ocular prosthesis. METHODS: prospective, longitudinal, descriptive and observational study of 62 patients (65 anophthalmic cavities) who went to the Oculoplasty service of "Ramon Pando Ferrer" Cuban Institute of Ophthalmology from May to December 2010. Age, sex, causes of eye loss, affected eye, conjunctival disease, type of cavity, length of time of prosthesis use, microbiological result, hygienic handling and response to treatment were the analyzed variables. RESULTS: in the studied group, the 40-59 y age group accounted for 37,1 % of patients; males were predominant with 66,1 %. Traumatism was observed in 40,3 %, being the right eye the most affected in 50 % of cases. Likewise, 33,8 % presented with allergic conjunctivitis and 18,5 % had conjunctival retractions. Atypical cavities represented 67,5 % with over 366 days of using the ocular prosthesis. The positive microbiological result was found in 55 cavities for 84,6 %. Staphylococcus aureus was isolated in 70,9 % of cavities and 41,5 % with daily handling of the ocular prosthesis. The response to treatment was good in 76,4 % of patients. CONCLUSIONS: conjunctival diseases in patients with ocular prosthesis are a health problem. Similarly, most of the anophthalmic cavities are atypical in adults with loss of eyeball from traumatism. Allergic conjunctivitis and conjunctival retraction are the most frequent illnesses in the patients wearing ocular prosthesis for more than one year, due to Staphylococcus aureus presence resulting from frequent hygienic handling of the prosthesis. The majority of patients positively responded to treatment.

Afecciones de la conjuntiva en pacientes con prótesis ocular / Conjunctival diseases of patients with ocular prothesis

Objetivos: determinar las afecciones conjuntivales en pacientes con prótesis ocular. Métodos: estudio observacional descriptivo longitudinal prospectivo, conformado por 62 pacientes (65 cavidades anoftálmicas) quienes acudieron al Servicio de Oculoplastia del Instituto Cubano de Oftalmología Ramón Pando Ferrer en el período de mayo a diciembre de 2010. Se analizaron la edad, el sexo, las causas de la pérdida ocular, el ojo afectado, la afección conjuntival, el tipo de cavidad, el tiempo de uso protésico, el resultado microbiológico, el manejo higiénico y la respuesta al tratamiento.Resultados: se presentaron 23 pacientes de 40 a 59 años (37,1 por ciento), de los cuales el sexo masculino mostró el 66,1 por ciento. El traumatismo reveló el 40,3 por ciento y el ojo derecho el 50 por ciento. El 33,8 por ciento presentó conjuntivitis alérgica; las retracciones de la conjuntiva reportaron el 18,5 por ciento. El 67,5 por ciento correspondió a cavidades atípicas con un tiempo de más de 366 días en el uso de la prótesis ocular. El resultado microbiológico positivo en 55 cavidades fue de 84,6 por ciento. Se aisló el Staphylococcus aureus en el 70,9 por ciento y de ellos el 41,5 por ciento se efectuó con manejo diario de la prótesis. La respuesta al tratamiento fue buena en el 76,4 por ciento. Conclusiones: las afecciones conjuntivales de los pacientes con prótesis ocular constituyen un problema de salud. Asimismo, la mayoría de las cavidades anoftálmicas son atípicas en adultos, con pérdida del globo ocular por traumatismo. La conjuntivitis alérgica y la retracción conjuntival son las afecciones más frecuentes en los pacientes con prótesis ocular de más de un año de uso, por la presencia de Staphylococcus aureus a consecuencia de mayor manipulación higiénico protésica, aunque en la mayoría de los casos existe buena respuesta al tratamiento(AU)

Objectives: to determine the conjunctival diseases of patients with ocular prosthesis.Methods: prospective, longitudinal, descriptive and observational study of 62 patients (65 anophthalmic cavities) who went to the Oculoplasty service of Ramon Pando Ferrer Cuban Institute of Ophthalmology from May to December 2010. Age, sex, causes of eye loss, affected eye, conjunctival disease, type of cavity, length of time of prosthesis use, microbiological result, hygienic handling and response to treatment were the analyzed variables. Results: in the studied group, the 40-59 y age group accounted for 37,1 percent of patients; males were predominant with 66,1 percent. Traumatism was observed in 40,3 percent, being the right eye the most affected in 50 percent of cases. Likewise, 33,8 percent presented with allergic conjunctivitis and 18,5 percent had conjunctival retractions. Atypical cavities represented 67,5 percent with over 366 days of using the ocular prosthesis. The positive microbiological result was found in 55 cavities for 84,6 percent. Staphylococcus aureus was isolated in 70,9 percent of cavities and 41,5 percent with daily handling of the ocular prosthesis. The response to treatment was good in 76,4 percent of patients. Conclusions: conjunctival diseases in patients with ocular prosthesis are a health problem. Similarly, most of the anophthalmic cavities are atypical in adults with loss of eyeball from traumatism. Allergic conjunctivitis and conjunctival retraction are the most frequent illnesses in the patients wearing ocular prosthesis for more than one year, due to Staphylococcus aureus presence resulting from frequent hygienic handling of the prosthesis. The majority of patients positively responded to treatment(AU)

Diagnosis and management of strabismus using telemedicine.

This study was undertaken to measure the effect of telemedicine consultation for diagnosis and treatment planning in cases of strabismus. Telemedicine consultation records of all patients on ORBIS Telemedicine Cyber-Sight that resulted in a final diagnosis of superior oblique palsy (SOP), Duane syndrome (DS), and Brown syndrome (BS) were collected. The following were then determined: (1) the clinical characteristics of patients in each category, (2) the diagnosis submitted by the doctor requesting consultation compared to the diagnosis determined by the mentor, and (3) the treatment plan submitted with the consultation request compared to the mentor's suggested plan. The clinical characteristics of patients with SOP, DS, and BS were similar to those reported in the literature. There were 89 with SOP, 131 with DS, and 50 with BS. Partners and mentors agreed on the diagnosis of SOP in 81% of cases, DS in 79% of cases, and BS in 72% of cases. Mentors agreed with the partners' proposed treatment plan in SOP 35%, DS 55%, and BS 52%. Mentors are likely to change the diagnosis proposed by doctors seeking consultation for strabismus in 25% of cases and provide a new treatment plan in more than 50% of cases. The clinical characteristics of those strabismus entities selected matched those found in the literature, making it likely that the cases as viewed on telemedicine presented a true clinical picture.

Fixation preference for the affected eye in patients with unilateral Duane syndrome.

Duane retraction syndrome is a congenital incomitant strabismus caused by dysinnervation of the medial and lateral rectus muscles. Patients with unilateral Duane syndrome (80-90% of cases) who exhibit a fixation preference tend to prefer the unaffected eye. We describe 8 patients with unilateral Duane syndrome who prefer the affected eye. The most frequent associated ophthalmic finding was decreased vision in the unaffected eye from anisometropia and/or amblyopia. An additional associated finding was decompensated intermittent exotropia in 2 patients.

Factors predicting upshoots and downshoots in Duane's retraction syndrome.

PURPOSE: Factors predicting mechanical and innervational types of upshoots and downshoots in Duane's retraction syndrome were evaluated. METHODS: This retrospective study evaluated upshoots and downshoots in 196 patients (222 eyes) with Duane's retraction syndrome seen between January 1990 and July 2001. On the basis of the clinical characteristics, upshoots and downshoots were classified as mechanical type, innervational type, or both. Factors potentially predicting the upshoot-downshoot phenomenon that were studied included patient age, type of Duane's retraction syndrome, horizontal strabismus, and vertical tropia in the primary position of gaze. RESULTS: Upshoots and downshoots were present in 39% of the eyes. The mechanical type was more common than the innervational type (26% vs 12%). Overall, upshoots and downshoots were statistically significantly more common in type III Duane's retraction syndrome, with exotropia, and with vertical tropia in primary position. Patient age had no correlation with upshoots or downshoots. Both types of upshoots and downshoots were significantly more common in type III Duane's retraction syndrome. A statistically significant association was found between exotropia and the mechanical type, and between vertical tropia in primary position and the innervational type. CONCLUSIONS: All patients with type III Duane's retraction syndrome, exotropia, and vertical tropia in the primary position of gaze should be examined for upshoots and downshoots. One should look specifically for the mechanical type when there is exotropia and for the innervational type when there is a large vertical tropia in the primary position of gaze.

Pseudotumor cerebri in a patient with Goldenhar's and Duane's syndromes.

A 4-year-old boy presented with Goldenhar's syndrome, Duane's syndrome and bilateral papilledema. Magnetic resonance imaging of the brain was unremarkable. On lumbar puncture, the cerebrospinal fluid (CSF) pressure measured 36 cm H(2)O. CSF examination was normal. The diagnosis of pseudotumor cerebri was made, and treatment with acetazolamide was started. As the papilledema did not resolve, steroids were added to the treatment. Lumbar puncture was repeated after 1 month, and pressure was found to be 30 cm H(2)O. Because medical treatment was not effective in lowering the CSF pressure, optic nerve sheath fenestration was performed. Papilledema resolved over the next 2 months. To the best of our knowledge, this is the first case of Goldenhar's syndrome associated with pseudotumor cerebri.

Duane retraction syndrome.

Duane retraction syndrome (DRS) consists of deficient horizontal eye movements, eyelid retraction, palpebral fissure narrowing, and abnormal vertical eye movements. Most cases are sporadic and unilateral (usually left side) with a slight female predominance. Several associated ocular and systemic conditions have been described in DRS patients. In most cases, the abducens nucleus and nerve are absent or hypoplastic, and the lateral rectus muscle is innervated by a branch of the oculomotor nerve. However, there may be contributing mechanical abnormalities. Type I DRS (primary gaze position esotropia with limitation of abduction) comprises the majority of cases. Approximately 50% of type I DRS patients are orthophoric in primary gaze. Esotropia is the most common type of strabismus encountered, and characteristic up shoots and down shoots occur in adduction. Surgical intervention has gradually become more popular in order to improve the primary gaze alignment and mitigate some of the associated abnormalities in ocular motility. However, patients are rarely rendered clinically normal, and limited expectations are appropriate.

Duane's syndrome.

Duane's syndrome is an unusual congenital form of strabismus where there is paradoxical anomalous lateral rectus innervation of the affected eye due to misdirection of axons destined for the medial rectus. Three types of Duane's syndrome are recognized. Most cases of Duane's syndrome are sporadic but up to 10% are familial, usually with autosomal dominant inheritance. Several autosomal dominant syndromes with dysmorphic features are associated with Duane's syndrome. Chromosomal loci for genes contributing to Duane's syndrome have been suggested at 4q, 8q and 22q. Duane's syndrome is heterogeneous at multiple levels with variations in its ocular manifestations, accompanying systemic manifestations and in the chromosomal loci with which it may be associated. The definition clinically and genetically of the various subgroups of Duane's syndrome will provide a valuable insight into brainstem axonal guidance to the extraocular muscles during human development.

Paralytic and incomitant strabismus.

During the past year, there have been interesting reports on the association of head tilt, facial asymmetry, and congenital superior oblique palsy. The etiology of sixth nerve palsy and its outcome in adults and children have been studied in two large series, and an orbital surgical approach has been reported to be valuable in cases of Brown's syndrome and superior oblique overaction.

[Congenital syndromes of oculomotor disturbances--diagnosis and results of surgical treatment]. / Wrodzone zespoly zaburzen narzadu ruchowego oczu--rozpoznawanie i wyniki operacyjnego leczenia.

During the period of 30 years (1964-1994), 363 children with different congenital syndromes of oculomotor disturbances were treated: 300 with Duane's retraction syndrome, 29 with Moebius syndrome, 34 with the superior oblique tendon sheath syndrome of Brown. Symptoms of these syndromes are presented. The choice of appropriate therapy of oculomotor disturbances are described. For aesthetic indications surgical treatment was carried out in 142 (47.3%) patients with retraction syndrome, in 20 (68.7%) with Moebius syndrome and in 23 (67.75%) with Brown's syndrome. Marked reduction of deviation in primary position and reduction of anomalous head posture were obtained.

[Injection of botulinum toxin into the oculomotor muscles in disorders of ocular motility]. / Wstrzykiwanie toksyny botulinowej do miesni okoruchowych w zaburzeniach ruchomosci galki ocznej.

Injections of botulinum toxin into the oculomotor muscles was used in incorrectible diplopia, ocular torticollis, Duane's syndrome and congenital nystagmus. Favourable results were obtained and the sole complications which could be observed were a transitory ptosis and subconjunctival haemorrhages. Frequently 2 to 3 injections were sufficient for a permanent effect.

Duane's retraction syndrome.

Duane's retraction syndrome (DRS) is a congenital abnormality of ocular motility that occurs in about 1% of strabismic patients. Three types have been described and the clinical features include incomitant horizontal strabismus, restricted motility and globe retraction. Head turn, upshoot or downshoot of the adducted eye, and bilaterality may occasionally be present. Since strabismus is invariably present in DRS, careful assessment of extraocular motility should be performed on all children with a suspected or confirmed eye turn. Early diagnosis may save practitioners and parents hours of time and financial investment with orthoptic and surgical management attempts. A case of DRS is presented.