Tratamiento quirúrgico en malformación de chiari I en la edad pediátrica: estudio y resultados en serie de 57 casos / Surgical treatment in chiari I malformation in the pediatric age: study and results in a series of 57 cases

Introducción: Las malformaciones de Chiari constituyen un grupo heterogéneo de patologías que ocasionan la herniación descendente de estructuras de la fosa posterior. La malformación de Chiari tipo I (MCI) se define como la herniación amigdalina de al menos 3 a 5 mm por debajo del nivel del foramen magno. La resonancia magnética ha aumentado el número de casos diagnosticados, inclusive en la edad pediátrica. Este trabajo tiene por objetivo presentar los resultados en cuanto a hallazgos clínicos y evolución postoperatoria en una serie pediátrica y se realiza una revisión del tema. Pacientes y métodos: Se efectuó un análisis retrospectivo de cohorte única de 57 pacientes operados a lo largo de 20 años por el mismo equipo neuroquirúrgico. Se revisaron los registros de uno de los autores (ES) para obtener datos sobre presentación clínica, diagnóstico, tratamiento y evolución postoperatoria, con un seguimiento de al menos 6 meses en cada paciente. Resultados: Se operaron 57 pacientes de entre 3 a 13 años de edad. El 100% de las malformaciones de Chiari I operadas eran sintomáticas. El síntoma más frecuente fue la cefalea (90%). En todos los casos se efectuó descompresión de la fosa posterior con duroplastia. El 100% de los pacientes presentaron mejoría clínica postoperatoria que se mantuvo a los 6 meses de seguimiento. Se registraron 3 casos de pseudomeningocele y un solo caso de fístula externa de LCR (7%). Conclusiones: La descompresión de fosa posterior con duroplastia es una opción quirúrgica muy efectiva y con baja tasa de complicaciones.

Introduction: Chiari malformations constitute a heterogeneous group of disorders characterized by a maldevelopment of the hindbrain and posterior cranial fossa that determines downward herniation of the cerebellar tonsils. Chiari malformation type I is defined as a 3 to 5 mm tonsillar herniation below the level of the foramen magnum. MRI has increased the number of cases diagnosed even in the pediatric age. Hence, the aim of this article is to present our results concerning clinical findings and outcomes on an exclusively pediatric series and to perform a review of the subject.Patients and methods:this is a retrospective, single-cohort study involving 57 patients operated in a 20-year period by the same neurosurgical team. Clinical records of one of the authors (ES) were reviewed in order to obtain data about symptoms, diagnosis, treatment and postoperative outcome at 6-month follow up.Results: 57 patients between 3 and 13 years of age were operated on. All of the patients were symptomatic at the time of diagnosis. The most frequent symptom was headache (90. All of the patients underwent posterior fossa decompression with duroplasty. 100% of the patients experienced sustained clinical improvement even at 6 month-follow up. Three cases of pseudomeningocele and only one case of external CSF leak were observed (7%).Conclusions:according to our findings and international literature, posterior fossa decompression with duroplasty is an effective surgical treatment with a low rate of complications.

Resultados clínicos de la resincronización ventricular en pacientes con insuficiencia cardíaca severa / Results of intraventricular resynchronization in patients with severe cardiac failure

BACKGROUND: Intraventricular resynchronization with pacemakers is a promising therapy for patients with refractory cardiac failure and intraventricular conductions delay. However its long term effects are not well known. AIM: To report the results of this therapy in patients with cardiac failure. PATIENTS AND METHODS: Fourteen patients (11 male), whose mean age was 68 years, with a severe and refractory cardiac failure, have been treated in our unit using intraventricular resynchronization with pacemakers. Eight had a coronary heart disease and six a dilated myocardiopathy. The pacemaker was implanted transvenously, with conventional stimulation in atrium and right ventricle. The left ventricle was stimulated through an epicardial vein, accessed through the coronary sinus. RESULTS: In one patient the high thresholds did not allow a left ventricular stimulation. In the other 13 patients, a clinical improvement was observed in 11 (85 per cent), that has been sustained for a mean of 8.2 months. The ejection fraction improved form 23.5 to 32.4 per cent (p < 0.001), the 6 min walking test improved from 347 to 437 m (p = 0.003) and the functional capacity changes from 3.3 to 2.7 (p < 0.001). Three patients died during follow up. One was the patient in whom the stimulation failed and two had a sudden death. No complications of the procedure were observed. CONCLUSIONS: In this series, intraventricular resynchronization with pacemakers was effective in 11 of 13 patients, improving functional capacity and ejection fraction. Sudden death could be avoided adding a defibrillator to the pacemaker system.