Utilidad práctica de la dinámica cuantitativa de LCR en la malformación de Chiari tipo I y la siringomielia. Reporte de 5 casos / Practical utility of quantitative CSF dynamics in type I Chiari malformation and syringomyelia. Report of 5 cases

Introducción: el tratamiento de la malformación de Chiari I (MCI) y/o la siringomielia (SM) es controversial. La dinámica cuantitativa del LCR a nivel cráneo espinal es una alternativa que podría orientar la terapéutica. El objetivo de esta publicación es describir 5 casos en donde la utilización de la dinámica de LCR permitió guiar el tratamiento. Material y método: se revisaron las historias clínicas de 5 casos (edad media: 39 años / 3 mujeres y 2 varones). Todos fueron estudiados con RM en contraste de fase. El diagnóstico fue de MCI (1 caso) y SM (3 casos) o solo SM (1 caso). Sólo 2 casos con MCI+SM fueron intervenidos (descompresión + plástica dural). Todos fueron seguidos entre 1,5 y 6 años. Resultados: caso 1 (MCI) la velocidad del LCR fue normal por lo que su cefalea fue tratada médicamente con buenos resultados; caso 2 (MCI+SM) la velocidad estuvo aumentada por lo que fue intervenida controlándose los síntomas y la SM; caso 3 (MCI +SM) la velocidad fue normal siendo su diagnóstico compatible con síndrome post-siringomiélico; caso 4 (SM) la velocidad estuvo aumentada a nivel C5-C6 siendo su diagnóstico compatible con una SM espinal primaria; caso 5 (CMI + SM) luego de la intervención se observó que las velocidades y la SM tardaron 16 meses en normalizarse. Conclusión: en los casos descriptos la dinámica de LCR permitió realizar el diagnóstico correcto, determinar la conveniencia de realizar la cirugía, encontrar la causa y controlar la evolución postoperatoria(AU)

Background: the treatment of Chiari malformation I (CMI) and/or syringomyelia (SM) is controversial. The quantitative dynamics of CSF at the craniospinal level is an alternative that could guide therapy. The objective of this publication is to describe 5 cases in which the use of CSF dynamics allowed guiding the treatment. Methods: the medical records of 5 cases (mean age: 39 years / 3 women and 2 men) were reviewed. All were studied with MRI in phase contrast. The diagnosis was CMI (1 case) and SM (3 cases) or only SM (1 case). Only 2 cases with CMI+SM underwent surgery (decompression + duraplasty). All were followed between 1.5 and 6 years. Results: case 1 (CMI) the velocity of the CSF was normal, so his headache was treated medically with good results; case 2 (CMI+SM) the velocity was increased so it was intervened controlling the symptoms and the SM; case 3 (CMI +SM) the velocity was normal, its diagnosis being compatible with post-syringomyelic syndrome; case 4 (SM) the velocity was increased at the C5-C6 level, its diagnosis being compatible with a primary spinal SM; case 5 (CMI + SM) after the intervention it was observed that the velocities and the SM took 16 months to normalize. Conclusion: in the cases described, the CSF dynamics allowed the correct diagnosis to be made, to determine the advisability of performing surgery, to find the cause and to control the postoperative evolution(AU)

Arnold Chiari I Malformation with Tip-Toe Gait: A Case Report

The Arnold-Chiari type I malformation has many symptoms such as headache, neck pain, gait impairment, abnormal movements or postures. But a few cases reported association of specific symptom including headache, neck pain, vertigo or ataxia. We report a case of 12 year-old boy presenting with tip-toe gait. Magnetic resonance imaging (MRI) study of brain and spine revealed underlying Arnold-Chiari type I malformation. This case shows that it is need for central nervous system evaluation in patients with changes of lower extremities tone.

Manifestações otoneurológicas tardia da malformação de Chiari I / Late otoneurologic manifestations of Chiari I malformation

INTRODUÇÃO: A malformação de Chiari (MAC) pertence a um grupo de anomalias que envolvem as estruturas da junção crâniocerebelomedular. O tipo I (MAC I) é caracterizado pela descida das tonsilas cerebelares e da porção medial do lobo inferior do cerebelo pelo canal cervical. Na literatura, têm sido descritas manifestações motoras, sensoriais e autonômicas (5). É encontrada com mais frequência em mulheres. Sua prevalência é difícil de definir, pois existem muitos casos assintomáticos, o que torna escassa a informação epidemiológica (3). OBJETIVO: O objetivo do trabalho foi relatar um caso de malformação de Chiari tipo I em uma paciente de 66 anos. RELATO DE CASO: Os autores relatam um caso de malformação de Chiari tipo I, em paciente de 66 anos, com sintomas de zumbido, hipoacusia e cefaleia occipital. COMENTÁRIOS FINAIS: A hipótese diagnóstica de Chiari tipo I deve ser embasada nas queixas do paciente, no exame clínico e de imagens, sendo a prevalência desta enfermidade de difícil definição, podendo haver diagnóstico em raras faixas etárias.

INTRODUCTION: Chiari malformation (CM) belongs to an anomaly group comprised of the structures in the cranial-cerebellarmedullary junctions. Type I (CM I) is characterized by the descent of the cerebellar tonsils and the medial portion of the lower cerebellar lobe through the cervical spinal canal. In literature, motor, sensorial and autonomous manifestations have been described. It is mostly found in women. Its prevalence is hard to determine, since there are many asymptomatic cases, hence, making the epidemiologic information scarce. OBJECTIVE: The objective of this work was to report a Chiari I malformation case in a 66-year-old female patient. CASE REPORT: Authors report a Chiari I malformation case in a 66-year-old female patient, showing tinnitus, hearing loss and occipital headache symptoms. FINAL COMMENTS: Chiari I's diagnostic hypothesis must be based on the patient's complaints, clinical and image examinations, and since the prevalence of this disease is difficult to determine, there can be diagnoses in rare age groups.

Treatment of Arnold-chiari I malformation by limited posterior fossa decompression / 中华神经医学杂志

Objective To explore the effect of limited posterior fossa decompression(LPFD)in the treatment of Amold-chiari I malformation.Methods A retrospective analysis was conducted among 29 patients undergoing LPFD from 2004 to 2008.The standard surgical procedures included small osseous decompression of the occipital bone above the forarnen magnum,removal of the posterior arch of the atlas,separation of the arachnoid adhesions,and reduction of the inferior cerebellar tonsils,a dural graft for duraplasty.The outcomes of the surgeries were evaluated using the Tator criteria.Results Excellent results were obtained in 23(79.3%)patients according to the Tator scores,and good results were achieved in 6(20.7%)patients.During the follow-up of 15 patients,the syrmgomyelia was found to be further reduced in 9 patients,and 1 patient experienced recurrence.Conclusion Limited posterior fossa decompression is effective for management of Amold-chiari I malformation with minimal invasiveness and complications.

Arnold-Chiari Type I Malformation with Hemihypertrophy: A case report

The association between Arnold-Chiari type 1 malformation and hemihypertrophy has not been appreciated but a few case reports have suggested their association and proposed a common pathogenesis of dysembryoplasia of mesoderm. We report a case of 17 year-old girl presenting with left side hemihypertrophy and scoliosis. Magnetic resonance imaging (MRI) study of brain and spine revealed underlying Arnold- Chiari type 1 malformation and syringomyelia. The purpose of this paper is to emphasize the need for central nervous system evaluation in patients with hemihypertrophy.

Amigdalopexia cerebelosa en el manejo de la malformación de chiari de tipo I / Cerebellar amygdalopexy in the management of Chiari I malformation

Antecedentes. El tratamiento de la malformación de Chiari de tipo I asociada a siringomielia es controvertido. En este trabajo se presenta un análisis clínico, quirúrgico y radiológico de los pacientes con esta afección que fueron manejados durante un periodo de doce años. Material y métodos. Se incluyeron 48 pacientes, donde se encontró un discreto predominio en el sexo femenino. El cuadro clínico estuvo dominado por cefalea, dolor cervical, signos y síntomas cerebelosos, afección de nervios craneales bajos y lesión de la vía piramidal. Resultados. Tanto el grado de descenso amigdalino como el tamaño de la siringomielia fueron muy variables, sin encontrar correlación entre ambos. La cirugía consistió en una craniectomía occipital, laminectomía de C1, ascenso de amígdalas cerebelosas mediante coagulación bipolar y plastía de duramadre. Los mejores resultados clínicos se obtuvieron en el dolor y los síntomas cerebelosos, mientras que los peores fueron en la afección de los nervios craneales bajos y de la vía piramidal, sin embargo, en la gran mayoría de los pacientes se logró detener la progresión de los síntomas. No se presentaron complicaciones serias en el presente estudio. Conclusión. El procedimiento propuesto ofrece una alternativa segura, efectiva y comparable con otros métodos más riesgosos para el manejo de esta malformación.

BACKGROUND: The treatment of Chiari I malformation associated with syringomyelia is controversial. OBJECTIVE: We describe a series of patients with this disease treated during a twelve-year period. We also present clinical, surgical and radiological findings. MATERIAL AND METHODS: Forty eight patients were included; a non significant female predominance was found. Clinical course was characterized by headache, cerebellar signs and symptoms, neck pain and involvement of lower cranial nerves and pyramidal tract. The degree of tonsillar descent and syringomyelia size varied and a correlation between them was not found. Surgery consisted in an occipital craniectomy, C1 laminectomy and tonsillar elevation through bipolar coagulation and duraplasty. RESULTS: The best results were observed in pain and cerebellar symptoms, while a deficit of lower cranial nerves and pyramidal tract were observed. However, in most patients we were able to slow symptom progression. No adverse effects were documented. CONCLUSIONS: The surgicalprocedureproposed herein is an effective and safe treatment alternative for this malformation, and its results are comparable to other riskier procedures.

Neuropathic Arthropathy Induced by Syringomyelia due to Arnold-Chiari I Malformation: A case report

Neuropathic arthropathy is a chronic and progressive disease of bone and joints. One of the most common causes of neuropathic arthropathy is syringomyelia. Syringomyelia associated with Arnold-Chiari I malformation has been well documented in many reports. We report a case of 76 year-old woman presented with the right elbow joint pain and stiffness. Her symptom was caused by neuropathic arthropathy associated with Arnold-Chiari I malformation and syringomyelia. The purpose of this paper is to emphasize that neuropathic arthropathy requires the evaluation of central nervous system to assess for occult causal lesion.

Application of ventriculoperitoneal shunt as a treatment for hydrocephalus in a dog with syringomyelia and Chiari I malformation

A twenty-month-old Chihuahua male dog was presented to us suffering with ataxia. Based on the physical examination, X-ray and magnetic resonance imaging (MRI) examinations, we diagnosed the dog with hydrocephalus, Chiari I malformation and syringomyelia. Treatment consisted of internal medical treatment and the placement of a ventriculoperitoneal (VP) shunt. The ventricular dilatation was relieved and the dog improved neurologically; however, the Chiari I malformation and syringomyelia remained after surgically positioning the VP shunt.

Clinical observations of duraplasty using synthetic dural substitute or autologous fascia lata for treating Chiari I malformation complicated with syringomyelia / 中国微创外科杂志

Objective To evaluate the curative effects and complications of synthetic dural substitute (Neuro-Patch) in the surgical treatment of Chiari type I malformation (CMI) complicated with syringomyelia (SM). Methods .Forty patients suffered from CMI with SM were given foramen magnum decompression and duraplasty between June 2002 and June 2004. The duraplasty was performed using either synthetic dural substitute (Neuro-Patch Group, n=20) or autologous fascia lata (Autologous Group, n= 20). Results .Symptoms were improved in 17 patients in both of groups (85.0%) at 6 months postoperatively, and no deterioration of symptoms was seen. Postoperative pyrexia occurred in 12 patients in the Neuro-Patch Group (60.0%) and 9 patients in the Autologous Group (45.0%), without significant differences (?2=0.902,P=0.342). No statistically significant differences were observed between the Neuro-Patch Group and the Autologous Group in the operating time (3.6?0.7 h vs. 3.4?0.4 h; t=1.109,P=0.274), the time to the onset of postoperative pyrexia (7.3?3.4 d vs. 9.4?2.5 d;t=-1.560,P=0.135), the length of duration of postoperative pyrexia (range, 1~19 d vs. 1~8 d, median, 4.5 d vs. 2 d;z=-1.643,P=0.100), the drainage time (1.3?0.5 d vs. 1.2?0.4 d; t=0.230, P=0.820), the drainage volume (range, 15~300 ml vs. 20~250 ml, median, 80 ml vs. 37.5 ml; z=-1.359,P=0.174), the duration of antibiotic administration (15.8?4.8 d vs. 13.7?1.5 d;t=1.260,P=0.223), and the assessment outcomes of curative effects. The length of duration of hormone requirement was longer in the Neuro-Patch Group (12.8?4.1 d) than in the Autologous Group (7.8?3.1 d) (t=3.055, P=0.007). On MRI examinations at 6 postoperative months, no posterior cranial fossa effusion was detected in both of groups. Follow-up checkups for 9 months ~ 2 years in the 40 patients showed delayed infectious granuloma on the wound in 1 patient in the Autologous Group, which was cured by debridement. Conclusions .The Neuro-Patch is a reliable dural substitute for repairing of dural defects in the treatment of CMI associated with syringomyelia.

Chiari I Malformation Associated with Syringomyelia Treated with Foramen Magnum Decompression

The authors report two cases of Chiari I malformation associated with syringomyelia. The first is that of a 45-year-old woman with neck and shoulder pain and the other, a 24-year-old man with neck pain. In the former, MRI revealed caudal descent of the cerebellar tonsil to the C2 level and a large eccentric syrinx from the C2-T6 levels ; in the man, MRI showed caudal descent of the cerebellar tonsil to the C2 level and a large eccentric syrinx from the C2-T4 levels. We successfully treated these patients by foramen magnum decompression and duroplasty. Theories of Chiari I malformation associated with syringomyelia, as well as clinical signs and symptoms, and management techniques, are discussed.