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Vol. 2. Issue 4.
Pages 329-336 (January 1991)
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Vol. 2. Issue 4.
Pages 329-336 (January 1991)
Lipomielomeningoceles
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J. Broseta, G. García-March, Mª.J. Sánchez-Ledesma, J. Gonçalves, J.A. Barcia-Albacar, A. García-Palomo, I. Silva
Servicio y Cátedra de Neurocirugía, Complejo Hospitalario y Universidad de Salamanca, Salamanca
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Resumen

En el último período se han recogido 5 adultos y 3 niños con estados disráficos ocultos, en forma de lipomielomeningoceles lumbosacros, asociados o no a médula anclada, cuya afectación neurológica y ortopédica fue atribuida inicialmente a otros procesos. Siguiendo la escala clínica para estas lesiones del Sick Children Hospital de Toronto, sólo 1 caso se encontraba en el grado 0, al mostrar únicamente problemas esfinterianos vesicales ocasionales; el resto presentaba algún tipo de estigma externo, como lipomas subcutáneos, senos dérmicos e hipertricosis, diversos grados de debilidad y alteraciones sensitivas en miembros inferiores, deformidades otopédicas generalmente en pie y cadera, alteraciones en la marcha y, finalmente, trastornos esfinterianos. En los primeros casos de la serie el diagnóstico se basó exclusivamente en el estudio simple, mielográfico y mediante TC del raquis lumbosacro; en los cuatro últimos, el estudio de la lesión se completó con RM. Con esta batería de pruebas se confirmó siempre la presencia de una masa lipomatosa de diferente entidad, y generalmente con extensión extrarraquídea, en contacto con las estructuras neurales segmentarias, asociándose a dilatación local y oclusión parcial o total del fondo de saco dural en 5 casos, a diastematomielia en 1, a filum terminale engrosado en 2 y a médula anclada en 4.

En los 7 çasos sintomáticos se intentó paliar el cuadro mediante la cirugía, con resección subtotal de la masa lipomatosa, sección del filum terminale y reparación del fondo de saco dural. Sólo en 3 casos, 1 adulto y 2 niños, se observó alguna mejoría significativa, principalmente en la actividad esfinteriana; en el resto no se detectó ningún cambio.

Descriptores:
Lipomielomeningocele
Médula anclada
Espina bífida oculta
Paraparesia
Disfunción vesical neurogénica
Summary

Three children and five adults with occult spinal dysraphism, namely lumbosacral lipomyelomeningoceles usually with tethered spinal cord, whose neurological symptoms and orthopedic deformities were initially attributed to other processes, were lastly treated. Following the Hospital for Sick Children of Toronto clinical scale, only 1 case presenting with occasional bladder dysfunction was graded as 0; the rest showed sorne type of cutaneous lesions as subcutaneous lipomas, dermal sinus, tail-like appendages or hypertrichosis, diverse degrees of weakness and sensation alterations in the lower limbs, orthopedic deformities generally in the hip and/or the foot, gait impairment and finally sphincterian disturbances. In the first cases, definitive diagnosis was confirmed by X-ray, myelographic and CT-scanning studies of the lumbrosacral region; in the last 4 cases, the lesion was also detected by MR. With these imaging procedures, a lipomatous mass with extraspinal extension and in contact with the segmentary neural structures was constantly found associated to local widdening and partial or total occlusion of the dural sac in 5 cases, to diastematomyelia in 1, to thickened filum terminale in 2, and to tethered spinal cord in 4. In the 7 symptomatic cases, surgical approach to the lesion was attempted carrying through subtotal mass removal, filum terminale section and dural sac repairingo In 3 cases, 1 adult an 2 children, a ligth improvement was observed, mainly in regarding to urinary incontinence; the rest remained unchanged.

Key words:
Lipomyelomeningocele
Tethered spinal cord
Occult spinal dysraphism
Paraparesis
Neurogenic bladder dysfunction

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