ARCH SOC ESP OFTALMOL. 2010;85(12):395–399 ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA
ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA
Vol. 85
Mayo 2010
Núm. 5
Contenido Editorial La retina como marcador biológico de daño neuronal Artículos originales Comparación de tres instrumentos de tomografía de coherencia óptica, un time-domain y dos Fourierdomain, en la estimación del grosor de la capa de fibras nerviosas de la retina Idoneidad de tratamiento en sospechosos de glaucoma. Estudio de concordancia con el grupo de estudio RAND Atrofia de la capa de fibras nerviosas de la retina en pacientes con esclerosis múltiple. Estudio prospectivo con dos años de seguimiento Comunicaciones cortas Hipercorrección secundaria a transposición muscular aumentada Crítica de libros, medios audiovisuales y páginas web oftalmológicos Clinical Neuro-Ophthalmology: The Essentials Sección histórica Del mal de la rosa y la queratoconjuntivitis pelagrosa Sección iconográfica El estrabismo de Rembrandt Sociedades y Reuniones Científicas Ofertas de trabajo
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Original article
Superior oblique sharpening surgery in the treatment of Brown syndrome plus P. Merino,* V. Rivero, P. Gómez de Liaño, G. Franco, J. Yáñez Sección de Motilidad Ocular, Departamento de Oftalmología, Hospital General Universitario Gregorio Marañón, Madrid, Spain
A RT I C L E I N F O R M AT I O N
A B S T R A C T
Article history:
Purpose: To describe superior oblique sharpening in congenital Brown syndrome plus.
Received on May 27, 2010
Material and methods: A retrospective study of 17 Brown syndrome cases that were treated
Accepted on Oct. 11, 2010
with oblique superior sharpening from 1997 to 2007. Vertical deviation in primary position was classified as + to +++, head tilt as: mild (< 10°), moderate (10-20°) and severe (≥ 20°);
Keywords:
elevation in adduction from –1 to –4. A good postoperative result was considered if last
Oblique superior sharpening
elevation limitation in adduction was zero or –1, without head tilt and vertical deviation in
Secondary oblique superior palsy
primary position.
Brown syndrome plus
Results: Mean age was 4.9 years. Limitation elevation in adduction which was –3 in 8 cases
Torticollis
(47.1%) and –4 in 9 (52.9%), which improved completely after surgery in 6, –1 in 9 and –3 in
Vertical deviation
2 patients. Preoperative hypotropia in 15 cases (13 mild, 1 moderate and one severe) was resolved in 13 after surgery. Of 14 patients with torticollis (3 mild, 10 moderate and one severe) it was surgically corrected in 11. Success was achieved in 14 (82.4%), 2 were under corrected (11.8%) and one was overcorrected (5.88%). Mean follow-up was 60.71 months. Conclusions: Oblique superior sharpening as treatment for Brown syndrome plus is an effective procedure. The incidence of secondary oblique palsies has been very low. © 2010 Sociedad Española de Oftalmología. Published by Elsevier España, S.L. All rights reserved.
Resultados del afilamiento del oblicuo superior en el tratamiento del síndrome de Brown plus R E S U M E N
Palabras clave:
Objetivo: Describir los resultados del afilamiento del oblicuo superior (OS) en el síndrome
Afilamiento del oblicuo superior
de Brown congénito plus.
Paresias secundarias del oblicuo
Material y métodos: Estudio retrospectivo de 17 casos con síndrome de Brown operados con
superior
el afilamiento del OS desde 1997 a 2007. La desviación vertical (DV) en posición primaria
Síndrome de Brown congénito plus
de la mirada (PM) se clasificó de + a +++, el tortícolis en leve (< de 10°), moderado (10-20°)
Tortícolis
y severo (≥ 20°); y la limitación de la elevación en aducción (LEA) de –1 a –4. Se consideró
Desviación vertical
*Corresponding author. E-mail:
[email protected] (P. Merino). 0365-6691/$ - see front matter © 2010 Sociedad Española de Oftalmología. Published by Elsevier España, S.L. All rights reserved.
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buen resultado post-operatorio cuando la LEA fue nula o grado –1, sin tortícolis y sin DV en PM al final del periodo de seguimiento. Resultados: La edad media de la cirugía fue de 4,9 años. La LEA fue de –3 en 8 casos (47,1%) y de –4 en 9 (52,9%), normalizándose por completo con la cirugía en 6 casos, y quedando en 9 una LEA de –1 y en 2 de –3. La hipotropía preoperatoria, presente en 15 casos (13 leves, una moderada y una severa), se corrigió con la cirugía en 13. El tortícolis inicial, presente en 14 casos (3 leves, 10 moderados y uno severo), se corrigió en 11. Un buen resultado se obtuvo en 14 pacientes (82,4%), 2 quedaron hipocorregidos (11,8%) y uno hipercorregido (5,88%). El tiempo medio de seguimiento fue de 60,71 meses. Conclusiones: El afilamiento del OS en el síndrome de Brown congénito plus ha obtenido buenos resultados. La incidencia de hipercorrecciones por paresias secundarias de OS ha sido muy escasa. © 2010 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L. Todos los derechos reservados.
Introduction Brown syndrome is a restrictive ocular motility disorder characterized by severe active and passive elevation in adduction. Elevation in abduction may be normal or limited, in any case to a lesser extent than in adduction.1,2 It may be congenital or acquired; constant or intermittent; mild, moderate or severe; true or plus.1,3,4 True Brown syndrome is not associated with vertical deviation in PP, infraversion or torticollis. Brown syndrome plus is rather determined by the presence of vertical deviation in PP and torticollis.3 In the literature, its treatment remains a controversial issue. Several surgical procedures have been described and complications have been found to be commonplace,4 which explains in some instances a preference for conservative and expectant treatments.5 The present study describes the results obtained in a series of patients diagnosed with congenital Brown syndrome plus who underwent surgery using the superior oblique (SO) sharpening technique described by Horta.6
assessing the head tilt with respect to the body axis as the patient’s gaze fixated on the smallest row in the optotype at a distance of 6 meters. Patients were classified as mild (< 10°), moderate (10-20°) and severe (≥ 20°). ELA was classified as –1 thru –4 (–1: when a 75% elevation is attained; –2: at 50%; –3: when the eye does not elevate above the horizontal line; –4: if the eye does not reach the horizontal). Superior oblique hyperfunction was rated as null, mild (+1), moderate (+2) and severe (+3). BV was explored using the TNO test in patients old enough to cooperate, and was defined as positive if ≥ 480”. Intraoperative passive duction was performed before and after surgery. The direct portion of the SO was sharpened by placing the eye downwards and outwards and releasing restriction until turning passive duction negative. Surgery was completed by injecting triamcinolone acetonide (40mg) in the trochlear region. Results were considered good whenever ELA was normal or –1, with no torticollis or DV in PP, upon completion of follow-up.
Results Subjects, material and method The retrospective study covered 17 patients diagnosed with Brown syndrome plus and treated with the oblique superior sharpening technique between 1997 and 2007. Patients whose follow-up lasted less than 3 months, those who underwent other surgical procedures associated or not with sharpening were excluded. Table 1 contains preoperative data, including gender, age at operation, affected eye, vertical deviation (VD) and horizontal deviation (HD) in primary position (PP), torticollis, elevation limitation in adduction (ELA), hyperfunction of the ipsilateral superior oblique, V syndrome and binocular vision (BV). VD was qualitatively classified using the + a +++ cover test, since the age of some patients did not allow for the objective measurement with prisms. HD was measured with prisms and cover test. Torticollis was qualitatively measured
A total of 17 patients diagnosed with congenital and constant Brown syndrome plus underwent surgery using the superior oblique sharpening technique. Out of those 17 patients, 6 were males (35.3%) and 11 females (64.7%), 7 LE (41.2%), 8 RE (47.1%) and 2 bilateral (11.8%). Mean age at the time of surgery was 4.9 years, SD: 2,16 (range: 2.2-9.9). Of the 16 patients explored, 12 had BV, which remained unaltered after surgery, whereas one patient recovered BV after the procedure. SO hyperfunction was only observed in 8 out of 17 cases (7 were mild: 41.2%, and one moderate: 5.9%). V syndrome was observed in 14 (82.4%). Table 2 contains for every patient data on preoperative and postoperative hypotropia in PP, torticollis and ELA respectively. Initial ELA was –3 in 8 cases (47.1%) and –4 in 9 (52.9%). The 2 bilateral cases showed asymmetric ELA, using the eye with greater limitation for statistical recount purposes. Upon completion of follow-up, ELA had improved
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Table 1 – Preoperative data Gender
Eye
Age QX
Hypotropia
HD
T
ELA
SO Hyper
VS
BV
M F F F F F F M F F M F M F M M F
L BE R R R R L R L R L L L R BE L R
8.5 4.33 9.92 3.66 4.66 4.33 6 2.17 6.08 5.25 7.58 2.92 2.33 2.83 4.08 3.67 5.08
+ RE+ + ++ + + + +++ + + + + + + LE+ + No
OT –4dp OT –4dp OT OT OT OT OT OT –8dp OT OT +2dp OT OT OT
MOD No Mild MOD No No MOD MOD MOD MOD Mild MOD SEV No MOD MOD MOD
+++ RE+++/LE++ ++++ ++++ ++++ +++ +++ ++++ +++ +++ ++++ +++ ++++ +++ RE+++/LE++++ ++++ ++++
+ RE+ + ++ No No No No No No + No No + No + +
Yes Yes No Yes Yes Yes Yes Yes No Yes Yes Yes Yes No Yes Yes Yes
Yes No Yes No Yes Yes No Yes Yes Yes Yes Yes Yes NC Yes No Yes
BE: both eyes; R: right eye; HD: horizontal deviation in primary position; Age QX.: age at the time of surgery (years); SO Hyper: superior oblique hyperfunction; L: left eye; ELA: elevation limitation in adduction; F: female; MOD: moderate; NC: uncooperative patient; OT: orthotropia; VS: V syndrome; SEV: severe; T: torticollis; BV: binocular vision; M: male.
in all patients, stabilizing in 6, reaching –1 in 9 and –3 in 2. Preoperative hypotropia present in 15 cases (mild in 13, moderate in one, severe in one) was corrected with surgery in 13. Previous torticollis in 14 cases (3 mild, 10 moderate and one severe) was corrected in all but 3 patients (2 mild and one moderate). Good results were obtained in 14 patients (82.4%). Of the 3 cases (17.6%) which obtained poor results, 2 were associated with hypocorrection (11.8%) and one with hypercorrection (5.88%). The hypocorrected patient was diagnosed two months after surgery upon detection of reverse torticollis,
hypertropia of the affected eye in PP and ipsilateral inferior oblique hyperfunction, clinical signs compatible with SO secondary paresis. All 3 patients with poor results required re-intervention (table 2), which resulted in yet another hypercorrection in one patient previously hypocorrected. Mean follow-up was 60.71 months, SD: 35.23. Even though none of the –3 ELA cases showed poor results unlike the three –4 ELA cases, no statistically significant relation was found between both factors, nor between the results and the initial hypotropia and torticollis (Pearson’s chi-square test. SPSS 15.0 Program).
Table 2 – Postoperative data Hp
T
ELA
HYPO
HYPER
Reintervention
BV
Tfol
Result
No No No No No No No + No No No No No No No + No
No No No No No No No Mild No No No No No No No Mild Mod
+ RE+ + No + + No +++ No + + No + + No +++ No
No No No No No No No Yes No No No No No No No Yes No
No No No No No No No No No No No No No No No No Yes
No No No No No No No Reaf+Tenect.Post. No No No No No No No Reaf/RtSR 5 RtRS5+ResSO RE6 RETROIR LE 4+APTRL RE
Yes No Yes Yes Yes Yes No Yes Yes Yes Yes Yes Yes NC Yes No Yes
26 77 3 33 111 55 113 90 47 13 65 90 46 24 74 50 115
Good Good Good Good Good Good Good Bad Good Good Good Good Good Good Good Bad Bad
HYPER: hypercorrection; HYPO: hypocorrection; Hp: hypotropia; ELA: elevation limitation in adduction; MOD: moderate; NC: uncooperative patient; IO: inferior oblique; SO: superior oblique; Rt: retroinsertion; Reaf: reafilamiento; Res: resection; IR: inferior rectus; SR: superior rectus; Tfol: follow-up time in months; T: torticollis; VS: V syndrome; BV: binocular vision.
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Discussion Brown syndrome is a restrictive ocular motility disorder whose clinical features are well-known and have been reported in the literature since it was first described by Brown in 1950.1,2 The same cannot be said of its etiology and treatment. Apart from the initial etiological hypotheses on the sharpening and contraction of the reflex portion of the SO or its sheath and surrounding tissues1,4 and those more widely accepted theories according to which the underlying cause of most congenital and constant Brown syndromes is a spatial imbalance in the trochlea/tendon complex,1,2,4 there are also cases where the SO may explain a poor response to conventional treatments. Rosembaun et al published a series contributing the theory whereby the inferior glide of the lateral rectus due to alterations in the pulleys of extrinsic ocular muscles could result in congenital or acquired Brown syndrome. Thus, the said author recommends carrying out an NMR study to determine the condition of the pulleys, the SO and the trochlea as to avoid any potential surgical hypocorrections derived from the use of conventional surgical techniques.7 The frequency of this syndrome is estimated at one per every 500 strabismic patients, with a slight predominance of the female gender and RE, almost always unilateral (90%).8 Our results match the figures published, as 64.7% were females and in 47.1% of unilateral cases the RE was affected; 11.8% were bilateral. Several horizontal and vertical deviations have been associated with this syndrome, V-pattern incomitance being the most common form in 70% of cases,8 in our series 82.4%. As far as etiology is concerned, the confusing data available has prompted the use of several surgical techniques to treat this disorder, all of which resulted in a significant number of surgical hypercorrections due to secondary paresis of the SO, thus worsening prognosis as they may alter the existing BV prior to surgery in the majority of Brown’s syndrome patients.1,2,9-11 In our series, 75% had BV, which is the reason why certain authors recommend simple observation in the long run as there are reversible cases that resolve spontaneously: Dawson et al reported that 75% resolved spontaneously in a study of 32 patients with congenital Brown syndrome and a mean follow-up of 4 years. Ninety-one percent of patients had BV, 59% torticollis and in 34% stereopsis improved by the end of this period.5 Surgery is not universally accepted: some authors recommend observation in all cases,5 others recommend surgery for moderate Brown cases, i.e. minimal or no VD in PP and no torticollis but severe down-shoot.12 However, most authors operate only plus or severe Brown cases.3,13-15 Traditionally, tenotomy and tenectomy of the superior oblique muscle were the techniques of choice to improve restriction present in elevation in adduction.9 Nevertheless, these techniques frequently result in SO secondary palsies in 50-85% of cases,4,9,16,17 with a residual elevation limitation that ranges from 37 to 68%.12 Consequently, certain authors recommend combining nasal SO tenotomy and weakening of the inferior oblique.9
Wright published in 1991 his SO elongation technique with silicone band, which reduced by up to 10-11% the incidence of SO iatrogenic palsies.10,18 However, complications have been described as a consequence of both band adhesions to the sclera and the superior rectus19 and explant extrusions; thus, others prefer using non-resorbable sutures to elongate the tendon20 or performing SO resection.21,22 SO sharpening is a technique described by Horta to treat true and plus Brown syndromes whenever the cause is disproportion between the SO and trochlea or an inelastic SO.6 The few articles describing this technique obtained good results, with the disappearance of torticollis and ELA in 70% of cases, hypercorrection in 10-11% and hypocorrection in 10-22%.13-15 Martin et al report that 10% of RS fibrosis,15 which according to Jampolsky3 is caused by limitation of the depression secondary to treatment rather than by SO palsy, and 30% of patients required reintervention. In the present series, good results were obtained in 82.4% of cases using the SO sharpening technique, similar to the results obtained by other authors using the same technique14,15 and also to those attained with other types of procedures performed on the SO.4,9,10,12,18,19 Treatment failure due to hypocorrection accounted for a very small percentage of cases (11.8%), 2 out of 17; in one patient reintervention did not resolve SO paresis. Most authors recommend a slight hypocorrection during post-operative care for patients suffering from Brown syndrome.4,9,12-15,16 Hypercorrection due to SO secondary paresis was less common: one in 17 (5.88%), less than the figures reported for other surgical techniques10,18,19 except for the 15-mm posterior tenectomy in the series by Vélez, which resulted in no complications.12 The percentage of reinterventions was 17.64%, lower than the 30% reported by Martin et al15 using the same technique. ELA immediately after surgery was hypocorrected in all cases except one, which evolved in 2 months into SO palsy, undergoing surgery again on two occasions to address this issue. Upon completion of follow-up, ELA stabilized in 6 and remained slight in 9; thus, we agree with those authors who recommend post-operative hypocorrection,4,9,12-16, and advise against performing simultaneously the sharpening and recession of the SO.13 This study is limited for a number of reasons, such as: the fact that this is a retrospective paper and the small number of patients involved. The fact that no statistically significant relation was found between the result and the elevation limitation in adduction may be due to the limited number of patients included in this sample. Selecting a control group for observation would also be of interest in order to compare final results in treated and untreated patients. Finally, the use of the superior oblique sharpening technique to treat congenital Brown syndrome plus has shown good results in the majority of cases, similar to those attained with other surgical techniques. The incidence of iatrogenic hypercorrection due to SO secondary palsy was smaller than the figures published in other series. The number of reinterventions was low. In view of the risk of SO iatrogenic paresis, we recommend caution when resorting to surgery to treat hypocorrections. No other complications were found.
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Conflict of interest None of the authors has declared any conflict of interest.
R e f e r e n c e s
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11. Véronneau-Troutman S. Simultaneous superior oblique ˘ sheathectomy and inferior oblique tuck in congenital Brownis syndrome. Ann Ophthalmol. 1990;22:406-13. 12. Vélez FG, Vélez G, Thacker N. Superior oblique posterior tenectomy in patients with Brown syndrome with small deviations in the primary position. J AAPOS. 2006;10:214-9. 13. Merino P, Gómez-Liaño P, Valls I. Superior oblique sharpening ˘ syndrome “plus” Results. Arch Soc Esp Oftalmol. in Brownis 2000;75:29-34. 14. Rodríguez JM, Armada F, Rodríguez J, Gómez de Liaño R. Whettting technique in the surgical treatment of Brown syndrome. Arch Soc Esp Oftalmol. 1997;72:489-92. 15. Martín A, Rodríguez JM, Hernáez A, Bustos A. Results in whetting oblique superior tendon. Acta Estrabológica. 2001;30:11-25. 16. Cho YA, Kim S, Graef MH. Surgical outcomes in correction of Brown syndrome. Kor J Ophthalmol. 2006;20:33-40. 17. Armadá F, Rodríguez J.M. Tenotomy complications in surgery treatment of Brown syndrome. Acta Estrabológica. 1995;24:103-10. 18. Wright KW, Min BM, Park C. Comparison of superior oblique tendon expander to superior oblique tenotomy for the management of superior oblique overaction and Brown syndrome. J Pediatr Ophthalmol Strabismus. 1992;29:92-7. 19. Wilson ME, Sinatra RB, Saunders A. Downgaze restriction after placement of superior oblique tendon spacer for Brown syndrome. J Pediatr Ophthalmol Strabismus. 1995;32:29-34. 20. Yazdian Z, Kamali-Alamdari M, Yazdian MA, Taher M. Superior oblique tendon spacer with application of nonabsorbable adjustable suture for treatment of Brown syndrome. J AAPO. 2008;12:405-8. 21. Kraft SP, Nabi UN, Wilson ME, Roarty JD, Budning AS, De Faber ˘ syndrome with delayed JT, et al. Bilateral idiopathic Brownis onset in the second eye. J AAPOS. 2000;4:158-63. 22. Astle WF, Cornock E, Drummond GT. Recession of the superior ˘ oblique tendon for inferior oblique palsy and Brownis syndrome. Can J Ophthalmol. 1993;28:207-12.
