Study design: case series of 19 patients operated for severe scoliosis and diastematomyelia / Série de casos de 19 pacientes operados para escoliose severa e diastematomielia / Diseño del estudio: serie de casos de 19 pacientes operados por escoliosis grave y diastematomielia

ABSTRACT Objective: Diastematomyelia is a rare congenital spine and spinal cord malformation in which the spinal cord is divided into two parts by the osseous or fibrous septum. The incidence of diastematomyelia in patients with the most severe forms of congenital scoliosis is much higher than its general incidence in the population. When performing surgeries to correct scoliotic deformities, the question arises regarding the choice of a strategy for managing the septum. An unambiguous answer to this question does not exist, since the disease is very rare and heterogeneous. The aim was to summarize the data on different surgical strategies for detecting diastematomyelia. Methods: Literature review and retrospective analysis of our own clinical data. Results: We present our own experience of treating 19 patients with diastematomyelia and severe congenital scoliosis. Posture disorder was corrected in all cases; the septum was removed in none of the cases. Significant correction was achieved for all patients, and no neurological complications were observed in the short- and long-term follow-up. Conclusions: Surgical nonremoval of the spur enables compensation to be achieved, without neurological complications either in the immediate postoperative period or in the long-term (more than 2 years) follow-up. Level of Evidence IV; Case seriesh.

RESUMO Objetivo: A diastematomielia é uma má formação rara da espinha dorsal e medula congênita, quando a medula espinhal é dividida em duas partes pelo septo ósseo ou fibroso. A incidência de diastematomielia em pacientes com formas mais graves de escoliose congênita é muito maior do que a incidência geral em uma população. Ao realizar cirurgias para corrigir deformidades escolióticas, surge a questão sobre a escolha de uma estratégia para o manuseio do septo. Uma resposta inequívoca à essa questão não existe, pois a doença é muito rara e heterogênea. O objetivo foi resumir os dados sobre diferentes estratégias cirúrgicas para detecção da diastematomielia. Métodos: Revisão de literatura e análise retrospectiva de nossos próprios dados clínicos. Resultados: Apresentamos nossa própria experiência no tratamento de 19 pacientes com diastematomielia e escoliose congênita grave. Transtorno de postura foi corrigido em todos os casos; em nenhum dos casos o septo foi removido. Correção significativa foi alcançada para todos os pacientes e nenhuma complicação neurológica foi observada a curto e longo prazo de acompanhamento. Conclusões: A não remoção cirúrgica do esporão permite obter uma compensação e ter a falta de complicações neurológicas, tanto no período pós-operatório imediato quanto a longo prazo (mais de 2 anos) de acompanhamento. Nível de Evidência IV; Série de casosh.

RESUMEN Objetivo: La diastematomielia es una malformación congénita rara de la columna vertebral y la médula espinal en la cual la médula espinal se divide en dos partes por el tabique óseo o fibroso. La incidencia de diastematomielia en pacientes con las formas más graves de escoliosis congénita es mucho mayor que su incidencia general en una población. Cuando se realizan cirugías para corregir deformidades escolióticas, surge la pregunta con respecto a la elección de una estrategia para el manejo del tabique. No existe una respuesta inequívoca a esta pregunta, ya que la enfermedad es muy rara y heterogénea. El objetivo fue resumir los datos sobre diferentes estrategias quirúrgicas para la detección de diastematomielia. Métodos: Revisión de la literatura y análisis retrospectivo de nuestros propios datos clínicos. Resultados: Presentamos nuestra propia experiencia en el tratamiento de 19 pacientes con diastematomielia y escoliosis congénita grave. El trastorno postural fue corregido en todos los casos. El tabique no fue removido en ninguno de los casos. Se logró una corrección significativa en todos los pacientes y no se observaron complicaciones neurológicas en el seguimiento a corto y largo plazo. Conclusiones: La no remoción quirúrgica del espolón permite lograr una compensación sin complicaciones neurológicas, ya sea en el postoperatorio inmediato o en el seguimiento a largo plazo (más de 2 años). Nivel de Evidencia IV; Serie de casosh.

Caso Clínico: Diastomatomielia, una Forma Rara de Disrafia Espinal Oculta / Case Report: Diastomatomyelia, a Rare Form of Hidden Spinal Disraphy

INTRODUCCIÓN: La diastematomielia es una malformación raquimedular, un desdoblamiento sagital de la médula espinal, frecuentemente por debajo de la quinta vértebra dorsal; siendo una presentación rara de disrafismo espinal (menos del 3 %), más frecuente en el sexo femenino (3:1), se presenta entre los 10 y 76 años de edad. Puede ser asintomática y un descubrimiento incidental. Su diagnóstico es básicamente radiológico. CASO CLÍNICO: Paciente de sexo masculino de 20 años de edad, con cuadro de lumbalgia crónica, habiendo sido tratado por diferentes especialistas y tratamientos. Se solicitó estudios de imagen con la finalidad de esclarecer el diagnóstico; en la exploración física reportó zona de hipertricosis localizada a nivel lumbosacro y escoliosis dorsolumbar leve. EVOLUCIÓN: Los estudios radiológicos, tomográficos y de resonancia magnética evidenciaron la fusión de los cuerpos vertebrales L2 - L3 (espolón óseo), asociando anomalías de fusión de los elementos posteriores con división del canal raquídeo, y en médula espinal dos hemimédulas. Al momento el paciente se encuentra en controles periódicos por el servicio de traumatología con tratamiento clínico. CONCLUSIONES: No es sencillo el diagnóstico, frecuentemente se realiza en etapas tardías, cuando las alteraciones neurológicas son explícitas. Es preciso permanecer alerta ante alteraciones cutáneas de la línea media; dado que puede ser indicadora de diastematomielia o de cualquier disrafismo oculto.

BACKGROUND: Diastematomyelia is a spinal cord malformation consisting of a sagittal split spinal cord, often below the fifth thoracic vertebra and being a rare presentation of occult spinal dysraphism (less than 3 %), and it is more common in females (3:1), in the age of 10 and 76 years old. It can be asymptomatic and be discovered incidentally. It is basically radiological diagnosis. CASE REPORT: A 20 - years old, male patient presents chronic and unwilling back pain treatment, which has been treated by different specialists and treatments. If was requested image exams in order to clarify the diagnosis. Physical examination zone localized hypertrichosis and lumbosacral thoracolumbar scoliosis reported. EVOLUTION: The computed tomography and magnetic resonance showed the fusion of the L2 - L3 (bone spurs) associated with abnormalities fusion of the posterior elements with division of the spinal canal into two compartments, vertebral bodies and spinal cord in two hemicords. At present, the patient is under periodic controls by the traumatology service with clinical treatment. CONCLUSIONS: It is not easy diagnosis diastematomyelia often is performed in later stages, when neurological disorders are already flowered. Because of this, must remain alert to any skin disorder midline, it can be indicative of diastematomyelia or any of the hidden dysraphisms.

Fractura de L2 (AOSpine A3) en un paciente con diastematomielia asintomática. Reporte de caso / L2 (AOSpine A3) fracture in a patient with asymptomatic diastematomyelia. Case report

La diastematomielia es una anomalía del desarrollo del tubo neural, caracterizada por una división sagital de la médula espinal. Los síntomas son variados y puede cursar con dolor, trastornos de la marcha, escoliosis, deformidad de los pies, alteraciones del control de esfínteres, estigmas cutáneos, aumento de la tensión muscular e hidrocefalia, entre otros. En ocasiones es asintomática. Se presenta un caso clínico de un varón de 24 años, que sufre caída desde 4 m de altura y sufre fractura en columna lumbar L2 (AOSpine A3, N0), con diastematomielia en L1 de tipo I como hallazgo incidental y hemivértebra en T12. Se manejó con estabilización con tornillos pediculares con buena evolución. El manejo de estos pacientes debe ser individualizado. Los tornillos pediculares ofrecen resultados satisfactorios con bajo riesgo de lesión neurológica. Nivel de evidencia clínica Nivel IV.

Diastematomyelia is an abnormality of the development of the neural tube, and is characterised by a sagittal division of the spinal cord. is the symptoms vary and may include pain, gait disorders, scoliosis, deformity of the feet, changes in sphincter control, skin stigmas, increased muscle tension, and hydrocephalus, among others. It is occasionally asymptomatic. The case is reported of a 24-year-old male, suffering a 4-metre fall and a lumbar spine fracture L2 (AOSpine A3, N0), with L1 type I diastematomyelia as an incidental finding and hemivertebra in T12. It was managed with stabilisation with pedicle screws, with a good outcome. The management of these patients must be individualised, the pedicle screws offer satisfactory results with low risk of neurological damage.

Asociación entre diastematomielia y meduloepitelioma: reporte de un caso y revisión de la literatura / Association between diastematomyelia and medullo epithelioma: case report and literature review

Aim: Case report of association between diastematomyelia and medulloepithelioma. Method: 14-year-old patient with lower back pain and recent neurological deficit in extremities. CT and MRI scans of the thoracolumbar spine revealed a diastematomyelia. Intraoperative examination confirmed the presence of a spinal dysraphism and associated tumor, which was almost completely resected. Result: Histopathological and immunohistochemical findings were consistent with medulloepithelioma. Her postoperative course was uneventful. Conclusion: Diastematomyelia may manifest during adolescence as lower back pain and neurological deficit. The association of this malformation with a neoplasia is extremely rare; the present case describes concomitance with medulloepithelioma.

Reporte de un caso de asociación de diastematomielia y meduloepitelioma. Método: Paciente de 14 años con dolor lumbar y déficit neurológico en extremidades de reciente instalación. En TAC y RNM de columna dorsolumbar se pesquisa una diastematomielia. La exploración intraoperatoria, comprueba la presencia de una disrafia espinal y un tumor asociado al defecto, que se reseca casi en su totalidad. Resultado: Los hallazgos histopatológicos e inmunohistoquímicos son concordante con meduloepitelioma. La paciente tuvo un postoperatorio satisfactorio. Conclusión: La diastematomielia se puede manifestar durante la adolescencia como dolor lumbar y déficit neurológico. La asociación de esta malformación, con una neoplasia es muy poco frecuente, el presente caso describe la concomitancia con un meduloepitelioma.

Posterior spinal osteotomy of severe and rigid congenital scoliosis with diastematomyelia / 中国综合临床

Objective To evaluate the effectiveness and safety of one stage posterior vertebral osteotomy for correction of severe and rigid congenital scoliosis associated with Ⅰ, Ⅱ type of diastematomyelia.Methods According to the diastematomyelia packet,52 patients were divided into type Ⅰ group performed with mediastinum resection combined with spinal osteotomy, group Ⅱ without treatment of diastematomyelia direct spinal osteotomy.Group Ⅲ spinal osteotomy directly without diastematomyelia.Results The mean operation time was (548.6±113.2) min,the average amount of bleeding was (3 728.6±1 436.5) ml.In group Ⅰ,the mean operation time was (608.6± 123.2) min, significantly longer than those of group Ⅱ ((521.3 ±102.4) min,t=2.787,P＜0.01).In group Ⅰ the average amount of bleeding was (5 018.3 ±2 174.2) ml, significant more than that of group Ⅱ((2 615.3± 1 132.8) ml,t=5.182,P＜0.01).Patients with preoperative Cobb angle measurement for (95.2± 14.3) degrees, postoperative for (35.2± 14.8) degrees, follow-up of 2 years for (37.6± 16.1) degrees, group Ⅰ included preoperative (92.3 ± 12.8) degrees, postoperative (32.6 ± 15.8)degrees, 2 years later (35.8 ± 17.2) degrees;group Ⅱ before operation (99.2 ± 17.3) degrees, postoperative (37.3±14.3)degrees, 2 years later (40.2± 15.3) degrees.The postoperative Cobb angle correction rate and correction loss rate showed no significant difference between two groups (P ＞0.05), a posterior spinal osteotomy for the treatment of type Ⅰ and type Ⅱ with diastematomyelia severe rigid congenital scoliosis has good correction effect.This group of patients, the complication rate was 21.2％ (11/52);where in Ⅰ group the incidence rate of 36.4％ (8/22) was significantly higher than that of Ⅱ group 10.0％ (3/30) (P =0.021).Conclusion One stage posterior vertebral osteotomy for severe rigid with diastematomyelia of congenital scoliosis with the feasibility, effectiveness and safety, patients with type Ⅰ diastematomyelia should first bony mediastinum resection, Ⅱ type of diastematomyelia there is no need for treatment of diastematomyelia.

Diastematomyelia in Adult: A Case Report

STUDY DESIGN: Case report. OBJECTIVES: We report an adult patient with diastematomyelia. SUMMARY OF LITERATURE REVIEW: Diastematomyelia is an uncommon congenital malformation of the vertebral axis, characterized by a separation of the spinal cord with an interposed bony, cartilaginous or fibrous septum. Most of the patients present this condition in childhood. The cases in adulthood are extremely rare. MATERIALS AND METHODS: The authors experienced a 46-year old female patient with diastematomyelia presenting a gradual onset of neurologic claudication. We treated with decompressive laminectomy, septum removal and posterior instrumentation. RESULTS: We had satisfactory surgical results. CONCLUSIONS: We report an extremely rare case of diastematomyelia in adulthood.

Surgical treatment of scoliosis of thoracic hemivertebra and diastematomyelia of adolescent / 中国矫形外科杂志

[Objective] To discuss the operation and clinical results about surgical treatment of scoliosis of thoracic hemivertebra and diastematomyelia of adolescent.[Methods]From Jan 2001 to June 2007,15 patients were treated with one-stage posterior hemivertebrae and osseous divide resection combined with transpedicular instrumentation and bone graft.There were 6 males and 9 females with an average age of 21.2 years(range 16 to 24 years).All cases were segmented hemivertebrae.Hemivertebrae were located at T11(n=3)and T12(n=12).The status of the spinal fusion,correction rate and instrumentation were evaluated after surgery.[Results]All cases were followed up for 19 to 45 months with an average of 34 months.Cobb's angles of the main curve were 52.3??3.8?before surgery,10.2??1.4? after surgery.At the final follow-up there was 1.6? loss of correction.The mean height was increased by 3.76 cm.The district of bone graft showed good bone fusion.The time of vertebra fusion was 3 to 5 months(mean 3.6 months).No instrumentation,spinal fusion failure or other severe complications were noted.[Conclusion]One-stage posterior hemivertebrae and osseous divide resection combined with transpedicular instrumentation and bone graft can achieve a satisfactory result for the treatment of scoliosis of thoracic hemivertebra and diastematomyelia of adolescent.It can be recommended in clinical practice because of a good stability of fixation and fine spinal fusion.

Treatment of diastematomyelia by operation / 中国矫形外科杂志

[Objective]To evaluate the clinical treatment results for diastematomyelia treated by operation. [Method]Data from patients with diastematomyelia admitted to our hospital from May 1978 to January 2006 were reviewed. According to the classification, there were single-tube in 25 and double-tube in 96 patients. One hundred and two patients with diastematomyelia were treated by operation and ninteen by conservative treatment. The clinical results were retrospectively analyzed according to scoring neural function and monitoring the peak P40 of posterior trivial nerve cortical somatosensory evoked potential (PTNCSEP) before and after treatment.[Result]The total operative effective rate of double-tube diastematomyelia amounted to 86% during a follow-up period of 6 months to 15 years,more significant in patients with pain.The scores of neural function and PTNCSEP were significantly improved. But patients with single-tube diastematomyelia had no obvious change after operation.[Conclusion]The spinal cord is damaged severely in double-tube diastematomyelia and gradually aggravated.Operation can get symptomatic relief in many patients and terminate spinal cord lesion. So operation should be performed as soon as the diagnosis is made. The spinal cord is damaged slightly in single-tube diastematomyelia and surgery is not indicated.

Diastematomyelia Combined with Intradural Cystic Teratoma

Diastematomyelia is an uncommon dysraphic condition in which the spinal cord is split, with or without a fibrous, cartilaginous, or bone septum. The septum usually occurs in the lower thoracic or lumbar regions. Most of the patients with this condition are children of less than the years old. The condition is much less common in adults. Congenital tumor associated with diastematomyelia is more uncommon finding. Now, authors report one case of diastematomyelia combined with intradural cystic teratoma who is eighteen years old female patient.

SURGICAL TREATMENT OF CONGENITAL SCOLIOSIS WITH DIASTEMATOMYELIA / 解放军医学杂志

To study the clinical characteristics and surgical treatment of congenital scoliosis with diastematomyelia, four patients were found to have scoliosis with diastematomyelia by CT and MRI.The first step of surgery was to excise the osteal compartion in spinal cord, the second step was the posterior scoliosis correction and fusion after two weeks. All the patients had successful surgery. The body height of the patients increased 4 75cm and the Cobb angle deceased 43? in average. No complication was found in the nervous system. This method is very effective, and is worth popularizing its application.

Congenital Scoliosis Associated with Diastematomyelia / 대한정형외과학회잡지

Diastematomyelia is an uncommon congenital malformation of the vertebral axis characterized by a localized longitudinal separation of the spinal cord with an interposed septum. It may be found in association with congenital scoliosis. The Authors experienced a case of congenital scoliosis with diastematomyelia. Diastematomyelia was surgicallv resected and scoliosis was corrected by Dwyer operation 5 months later. The results of both operations were satisfactory in 10 months follow up.