Management of NF-1 dystrophic scoliosis associated with rib heads dislocation into the spinal canal in neurological intact patients: a systematic literature review.

PURPOSE: The management of scoliosis and kyphoscoliosis in patients with Type 1 Neurofibromatosis (NF-1) among spinal surgeons is still challenging due to the severity of the deformity especially in dystrophic deformity types. This rapid and progressive condition is likely to be associated with dislocated rib heads into the spinal canal, hence representing a real dilemma on the decision making between its resection versus not resection during the corrective surgery, especially in patients with normal neurological status. The objective of this publication is to discuss the management options in this patient population through a literature review. METHODS: A comprehensive systematic literature search was performed for relevant studies using PubMed, Web of Science, and Scopus databases. Previous publications depicting neurologically intact patients with NF-1 and rib dislocation into the canal were reviewed. Articles reporting individual cases or case series/cohorts with patient-discriminated findings were included. RESULTS: The data collection retrieved a total of 55 neurologically intact patients with NF-1 dystrophic scoliosis and rib penetration into the canal who underwent spinal surgery. Among them, 37 patients underwent surgery without head rib resection and 18 patients with rib excision. No patient presented postoperative neurological deficit except for one case of late postoperative neurological deterioration reported in a patient within situ fusion in which the surgeons ignored the presence of previous spinal cord compression. CONCLUSION: Corrective surgery for patients with NF-1 and rib penetration into the canal in neurologically intact patients can be safely performed without the resection of the dislocated rib heads without a higher risk of neurological compromise.

[Coffin-Lowry syndrome. Its association with congenitally narrow cervical canal and myelomalacia]. / Síndrome de Coffin-Lowry. Su asociación con canal cervical estrecho congénito y mielomalacia.

BACKGROUND: Typical clinical features of the Coffin-Lowry syndrome include facies with hypertelorism, small nose, wide mouth, full and everted lips; short stature, mental retardation, pectus deformity, mitral valve dysfunction, hippocampal and cerebellar involvement, hearing loss and spinal disorders such as kyphosis and scoliosis. Due to its scarce incidence, it is difficult making an early diagnosis. The aim of this report was to document the anatomical peculiarities identified during the surgical treatment of a patient with this syndrome. CLINICAL CASE: Male patient with Coffin-Lowry syndrome who evolved with narrow cervical canal plus myelomalacia at short age, making decompression from C3 to C6 and instrumentation from C2 to C7 necessary. During the surgery, in addition to calcification of the yellow ligament, adhesions on the dura mater from C4 to C4, dark purplish color in this area and hourglass-shaped thinning were found; the ends at C3 and C6 were normal. The purpose of the surgery was to stop the myopathy. Post-operatively, the patient had pulmonary complications; at the sixth day he passed away due to ventilatory complications and inadequate secretion control. CONCLUSIONS: The Coffin-Lowry syndrome is a rare diagnosis in our country; neurological involvement at the spinal level is characterized by kyphosis or scoliosis; for its diagnosis, an adequate medical history and a karyotype are necessary.

INTRODUCCIÓN: Las características clínicas típicas del síndrome de Coffin-Lowry son facies con hipertelorismo, nariz pequeña, boca amplia, labios amplios y evertidos; estatura corta, retardo mental, deformidad del pectus, disfunción de la válvula mitral, afectación de hipocampo y cerebelo, pérdida de la audición y trastornos de la columna, como cifosis o escoliosis. Debido a su escasa incidencia es difícil realizar el diagnóstico temprano. El objetivo de este informe fue documentar las peculiaridades anatómicas identificadas durante el tratamiento quirúrgico de un paciente con este síndrome. CASO CLÍNICO: varón con síndrome de Coffin-Lowry quien evolucionó con canal cervical estrecho más mielomalacia a corta edad, por lo que fue necesaria descompresión de C3 a C6 e instrumentación de C2 a C7. Durante la cirugía se encontró, además de la calcificación del ligamento amarillo, adherencias a la duramadre desde C4 a C5, color violáceo obscuro en esta área y adelgazamiento en forma de reloj de arena; los extremos en C3 y C6 eran normales. El objetivo de la cirugía fue detener la miopatía. En el posquirúrgico, el paciente presentó complicaciones pulmonares; al sexto día falleció por complicaciones ventilatorias y mal manejo de secreciones. CONCLUSIONES: el síndrome de Coffin-Lowry es un diagnóstico raro en nuestro país, la afección neurológica a nivel de la columna se caracteriza por cifosis o escoliosis, para su diagnóstico es necesario una adecuada historia clínica y un cariotipo.

Posterior-only spinal fusion without rib head resection for treating type I neurofibromatosis with intra-canal rib head dislocation

OBJECTIVES: Patients with Type I neurofibromatosis scoliosis with intra-canal rib head protrusion are extremely rare. Current knowledge regarding the diagnosis and treatment for this situation are insufficient. The purpose of this study is to share our experience in the diagnosis and surgical treatments for such unique deformities. METHODS: Six patients with Type I neurofibromatosis scoliosis with rib head dislocation into the spinal canal were diagnosed at our institution. Posterior instrumentation and spinal fusion without intra-canal rib head resection via a posterior-only approach was performed for deformity correction and rib head extraction. The efficacy and outcomes of the surgery were evaluated by measurements before, immediately and 24 months after the surgery using the following parameters: coronal spinal Cobb angle, apex rotation and kyphosis of the spine and the intra-canal rib head position. Post-operative complications, surgery time and blood loss were also evaluated. RESULTS: Patients were followed up for at least 24 months post-operatively. The three dimensional spinal deformity was significantly improved and the intra-canal rib head was significantly extracted from the canal immediately after the surgery. At follow-up 24 months after surgery, solid fusions were achieved along the fusion segments, and the deformity corrections and rib head positions were well maintained. There were no surgery-related complications any time after the surgery. CONCLUSIONS: Systematic examinations are needed to identify patients with Type I neurofibromatosis scoliosis with rib head dislocation into the canal who can be treated by posterior-only spinal fusion without rib head resection. .

Posterior-only spinal fusion without rib head resection for treating type I neurofibromatosis with intra-canal rib head dislocation.

OBJECTIVES: Patients with Type I neurofibromatosis scoliosis with intra-canal rib head protrusion are extremely rare. Current knowledge regarding the diagnosis and treatment for this situation are insufficient. The purpose of this study is to share our experience in the diagnosis and surgical treatments for such unique deformities. METHODS: Six patients with Type I neurofibromatosis scoliosis with rib head dislocation into the spinal canal were diagnosed at our institution. Posterior instrumentation and spinal fusion without intra-canal rib head resection via a posterior-only approach was performed for deformity correction and rib head extraction. The efficacy and outcomes of the surgery were evaluated by measurements before, immediately and 24 months after the surgery using the following parameters: coronal spinal Cobb angle, apex rotation and kyphosis of the spine and the intra-canal rib head position. Post-operative complications, surgery time and blood loss were also evaluated. RESULTS: Patients were followed up for at least 24 months post-operatively. The three dimensional spinal deformity was significantly improved and the intra-canal rib head was significantly extracted from the canal immediately after the surgery. At follow-up 24 months after surgery, solid fusions were achieved along the fusion segments, and the deformity corrections and rib head positions were well maintained. There were no surgery-related complications any time after the surgery. CONCLUSIONS: Systematic examinations are needed to identify patients with Type I neurofibromatosis scoliosis with rib head dislocation into the canal who can be treated by posterior-only spinal fusion without rib head resection.

Postoperative spinal deformity after treatment of intracanal spine lesions.

BACKGROUND CONTEXT: Surgical treatment of intracanal (both intramedullary and extramedullary) spine lesions requires posterior decompressive techniques in nearly all instances. Postoperative spinal deformities, most notably sagittal and coronal decompensation, are of significant concern for both the patient and the spinal surgeon. PURPOSE: To review and define principles and features of spinal deformities after posterior spinal decompression for intracanal spinal lesions, and to define patients who may benefit from the concomitant spinal fusion. METHODS: A systematic review of MEDLINE was conducted, including articles published between 1980 and 2011. Articles related to spinal deformities after posterior decompression for the treatment of intracanal spine lesions were identified. RESULTS: Ten articles met all inclusion and exclusion criteria. All were case series with limited evidence (Level IV). Many risk factors to deformity were implied but with limited evidence. Young age was the most commonly identified risk in these articles. CONCLUSIONS: Spinal deformity after posterior decompression is a common complication, most notably in children and young adults, after the removal of intramedullary tumors. Many risk factors have been implied to increase the postoperative development of spinal deformity, including young age, laminectomy extension, preoperative deformity, and extensive facet resection, among others. However, there is a lack of high-quality evidence to propose an algorithm for treatment or preventive measures. New studies with larger series of patients and standardized clinical outcomes are necessary to establish optimal treatment protocols.

Experiencia en tratamiento quirúrgico de cinco casos de siringomielia / Experience in surgical treatment of five cases of syringomyelia

Siringomielia, cavidades quísticas dentro del cordón espinal. Con incidencia baja de 8.4 por 100000 por año, Presentamos nuestra experiencia en tratamiento quirúrgico de 5 casos de siringomielia. Se presenta serie clínica, sin distinción de edad ni sexo, con diagnostico de siringomielia y que cumplan con los criterios quirúrgicos establecidos por el grupo. Estudiamos 5 pacientes, la totalidad de los casos presentaron síndrome disociativo medular por siringomielia, 4 casos sexo femenino, promedio de edad de 40,8 años, rango de seguimiento de 6 años a 6 meses, 5 casos procedentes del estado Mérida, 100% diagnostico por resonancia, 3 caso localización cervical, 1 caso la etiología es hemangioma capilar, a los 5 se les realizo derivación siringosubaracnoidea con mejoría clínica y en el postoperatorio tardío un 20% presento recidiva, manejada conservadoramente.

Syringomyelia, cystic cavities within the spinal cord. With low incidence of 8.4 per 100000 per year, present our experience in surgical treatment of 5 cases of syringomyelia. We present clinical series, regardless of age, sex diagnosed with syringomyelia and meet surgical criteria established by the grupo. We studied 5 patients, all cases had spinal cord syndrome and syringomyelia dissociative 4 cases female, mean age of 40.8 years, range 6-year follow-up to 6 months, 5 cases from the state of Merida, 100% resonance diagnosis, 3 cases cervical lesion, 1 case of capillary hemangioma etiology, to 5 were conducted with clinical improvement siringosubaracnoidea bypass and postoperative period by 20% for 1 case of recurrent, conservatively managed.

Experiencia en cirugía lumbar endoscópia / Experience in lumbar endoscopic surgery

La cirugía endoscópica de columna, resultado de la inquietud de múltiples investigadores en encontrar técnicas menos invasivas para el tratamiento de problemas quirúrgicos. Serie clínica de 33 pacientes, sometidos a cirugía lumbar endoscópica y evolución clínica (2009–2011). 33 pacientes, el 91% mínimo 6 meses de evolución, 100% fueron manejados ambulatorio, 52% femenino, 48% masculinos, promedio de 49 años de edad, 60% diagnostico de Lumbalgia discogenica y 40% síndrome de receso lateral y radiculalgia, 30% empleados públicos, 50% Discectomía endoscopia un nivel, 30% dos niveles, 17% tres niveles y 3% cuatro niveles, 21% tenía signos radiculares preoperatorios, un paciente (3%) espondilodiscitis, 37% reagudización clínica e inestabilidad lumbar, 15% requirió otra cirugía y 9% se le plantea nueva cirugía. Se evaluaron con la escala visual análoga del dolor, test de incapacidad de Oswestry, criterios de Macnab. La cirugía endoscópica lumbar, técnica atractiva, al reducir la vía de abordaje, reduce las complicaciones de cicatrización y la temprana incorporación a sus actividades. En nuestra experiencia, obtuvimos un 76% entre buenos y aceptables resultados y 24% malos según Macnab, lo que obliga a ser acuciosos para seleccionar al paciente.

Endoscopic surgery of the spine, resulting from the concern of many researchers to find less invasive techniques for the treatment of surgical problems. A case series of 33 patients who underwent lumbar endoscopic surgery and clinical course (2009 - 2011). 33 patients, 91% at least 6 months duration, 100% were ambulatory, 52% female, 48% male, average age 49 years, 60% diagnosis of discogenic low back pain and 40% lateral recess syndrome and radiculalgia, 30% civil servants, 50% endoscopic discectomy level, 30% two levels, three levels 17% and 3% four levels, 21% had preoperative radicular findings, one patient (3%) spondylodiscitis, 37% and clinical worsening lumbar instability, 15% required another surgery and 9% is facednew surgery. Were evaluated with visual analog pain scale, Oswestry Disability test, Macnab criteria. The lumbar endoscopic surgery, technically attractive, reducing the surgical approach, reduces the complications of early healing and incorporation into its activities. In our experience, we obtained 76% between good and acceptable and 24% poor results according to Macnab, making it necessary to be diligent to select the patient.

Endoscopic approaches to the spinal cord.

Minimally invasive procedures have been used to treat various diseases in medicine. Great improvements in these techniques have provided intraventricular, transnasal and more recently cisternal intracranial accesses used to treat different conditions. Endoscopic approaches have been proposed for the treatment of disk herniation or degenerative disease of the spine with great progress in the recent years. However the spinal cord has not yet been reached by video-assisted procedures. This article describes our recent experience in procedures to approach the spinal cord itself in order to provide either diagnosis by tissue biopsies or inducing radiofrequency spinal ablation to treat chronic pain syndromes. We describe three different approaches proposed to provide access to the entire length of the spinal canal from the cranium-cervical transition, cervico-thoracic canal (spinal cord and radiculi) to the lumbar-sacral intraraquidian structures (conus medularis and sacral roots). We idealized the use of endoscopy to assist cervical anterolateral cordotomies and trigeminal nucleotractotomies, avoiding the use of contrast medium as well as vascular injuries and consequent unpredictable neurological deficits. This technique can also provide minimally invasive procedures to possibly treat spasticity through selective rhizotomies, assist catheter placements in the lumbar canal or debridation of adherences in cystic syringomyelia and arachnoid cysts, providing normalization of CSF flow.

Intraosseous sacral paraganglioma with extradural extension: case report.

BACKGROUND: Paragangliomas are tumors that arise from the paraganglion system, which is a component of the neuroendocrine system. Approximately 10% are located in the extra-adrenal paraganglion system. Paragangliomas of the spine, however, are rare. They usually present as an intradural tumor in the cauda equina. There are only three reports of primary intraosseous paragangliomas of the sacrum. CASE DESCRIPTION: A 69-year-old man presented with low back pain and urinary incontinence. Imaging revealed a large intraosseous mass at S2, S3 and S4. Surgical resection was accomplished through a posterior midline incision exposing the spine from L5 to the coccyx. The tumor was located in the extradural space. It was friable, grayish and bleeding. Total tumor removal was performed, with normal bone margins. Follow-up at 2 years showed complete resolution of the preoperative symptoms and no evidence of local recurrence. CONCLUSION: Although rare, the possibility of paraganglioma should be included in the differential diagnosis of sacral tumors. The majority of the spinal paragangliomas are benign, slowly growing tumors with low proliferative activity. Despite these characteristics, local recurrence has been reported in cases of both macroscopically total and subtotal resection. Postoperative radiation therapy for patients with incomplete excision may not prevent recurrence, so gross tumor removal should be the goal of surgery.

Outcome following lumbar disc surgery: the role of fibrosis.

BACKGROUND: Failed-back surgery syndrome remains a challenge for spinal surgeons. It can be related to several causes, including poor surgical indication, misdiagnosis, surgical technique failure, spondilodiscitis and fibrosis. Fibrosis has been associated with a poorer outcome in lumbar disc surgery, although its role in the generation of symptoms is not yet clear. In this study, the authors have analyzed any possible correlation between the clinical outcome and the degree of fibrosis. METHOD: Forty consecutive patients were enrolled in a prospective study. All of them had operations in the lower lumbar disc in a single level for the first time. Three months after the operation they were submitted to clinical outcome evaluations and questionnaires, including Numeric Pain Rating scales (NPR) for lumbar and leg pain, the McGill Pain Questionnaire, The Quebec Back Pain Disability scale (QBPD) and Straight Leg Raising test. These data were correlated with the degree of fibrosis as revealed by Magnetic Resonance Imaging (MRI). FINDINGS: After 3 months, the NPR values for lumbar and leg pain ranged from 0 to 8 (mean 2.32 and 1.67 respectively). The values of the post-operative QBPD scale ranged from 1 to 71 (mean 25.9). Every patient showed a varied degree of fibrosis on MRI. However, statistical analysis depicted no significant correlation between fibrosis and a poorer clinical outcome for pain and disability. CONCLUSIONS: The authors found no correlation between excessive fibrosis with lumbar and leg pain, disability or straight leg resistance. The role of fibrosis in the generation of symptoms in patients who have had lumbar disc surgery should be reevaluated.

[Lumbar neuroendocrine carcinoma with invasion of the neural canal]. / Carcinoma neuroendocrino lumbar con invasion al canal neural.

INTRODUCTION: Extraspinal tumors are a rare cause of lumbar root compression and neuroendocrine origin are one of the least frequent of this group. CASE REPORT: A 70 years-old woman complained of pain in low back and her right leg. Following image studies a tumor at the level of the L2 vertebrae was diagnosed. This was operated on and a neuroendocrine carcinoma was removed. CONCLUSION: The neuroendocrine tumors show a slow grows typically and are located in the gastrointestinal tract and lung. These are very rare in others localizations.

Avaliaçäo de pacientes com estenose do canal cervical submetido a laminoplastia / Evaluation of patients with stenosis of the cervical canal submitted to laminoplasty

Os autores apresentam os resultados de revisäo clínica e por imagem de nove pacientes com estenose do canal cervical e mielopatia, submetidos a laminoplastia do tipo "porta aberta", com seguimento médio de três anos e sete meses e idade média de 61,8 anos. Concluem que, em funçäo dos resultados obtidos, a técnica descrita é uma opçäo a ser considerada nos casos de estenose do canal cervical que necessitam de descompressäo acima de três níveis.