Hemifacial Spasm Associated with Chiari Type I Malformation: Surgical Considerations and Case Report

Hemifacial spasm (HS) is a movement disorder characterized by paroxysmal and irregular contractions of the muscles innervated by the facial nerve. Chiari malformation type I (CM I) is a congenital disease characterized by caudal migration of the cerebellar tonsils, and surgical decompression of foramen magnum structures has been used for treatment. The association of HS with CM I is rare, and its pathophysiology and therapeutics are speculative. There are only a few cases reported in the literature concerning this association. The decompression of the posterior fossa for the treatment of CM I has been reported to relieve the symptoms of HS, suggesting a relation between these diseases. However, the possible complications of posterior fossa surgery cannot be underrated. We report the case of a 66-year-old patient, in ambulatory follow-up due to right HS, no longer responding to botulinum toxin treatment. Magnetic resonance imaging (MRI) of the skull revealed compression of the facial nerve and CM I. The patient underwent surgery for HS by neurovascular microdecompression of the facial nerve via right lateral suboccipital craniectomy, but presented significant clinical worsening in the postoperative period even though the cerebellum edema related to surgical manipulation was mild. Due to the clinical worsening, the patient underwent a median suboccipital craniectomy with decompression of the foramenmagnum structures. After this second surgery, the patient had progressive improvement and was discharged from the hospital for ambulatory care.

Type I Chiari malformation presenting orthostatic syncope who treated with decompressive surgery / 소아과

Chiari malformations are a congenital anomaly of the hindbrain. The most common, Chiari malformation type I (CM-I), is characterized by herniation of the cerebellar tonsils extending at least 3 mm below the plane of the foramen magnum. Consequently, CM-I is associated with hydrocephalus and symptoms involving compression of the cervicomedullary junction by ectopic tonsils. Several studies have reported the clinical symptoms associated with CM-I, including suboccipital headache, weakness in the upper extremities, facial numbness, loss of temperature sensation, ataxia, diplopia, dysarthria, dysphagia, vomiting, vertigo, nystagmus, and tinnitus. Syncope is one of the rarest presentations in patients with CM-I. There are many hypotheses regarding the causes of syncope in patients with CM-I; however, the mechanisms are not clearly understood. Although surgical decompression for CM-I in patients with syncope has yielded good clinical results in some studies, such cases are rarely reported. We report a case of orthostatic syncope in a patient with CM-I who was treated with surgical intervention.

Síndrome de Marcapaso en paciente con Síndrome de QT Prolongado / Pacemaker syndrome in long QT syndrome patient

El síndrome de marcapasos es generado por la desincronización de la actividad auricular con la ventricular durante el marcapaso ventricular VVI en pacientes portadores de enfermedad del nodo. La enfermedad evoluciona de meses hasta varios años después del implante del marcapaso, siendo más frecuente en pacientes adultos mayores. El único tratamiento es el implante de un electrodo auricular para una estimulación aurículo ventricular sincrónica. Se presenta el caso de una paciente diagnosticada con enfermedad del nodo sinusal asociada a un QT prolongado, a la cual se le implantó un marcapaso VVIR, luego de algunos meses comenzó a presentar síntomas de insuficiencia cardiaca,a los 3 años del implante presento un episodio sincopal,en cuya evaluación con Holter ECG se registró un episodio de una taquicardia ventricular no sostenida observándose la presencia de ondas P retrogradas tras cada estimulación ventricular con marcapasos. Se consideró la probabilidad de un síncope arrítmico, que se manejó con Amiodarona. Se diagnosticó síndrome de marcapaso, realizando un mejoramiento del marcapaso por un equipo bicameral, con lo cual todos los síntomas de insuficiencia cardiaca desaparecieron. No se repitió el evento sincopal y desde la época la paciente se maneja en una capacidad funcional normal.

Pacemaker syndrome is an entity generated by a desynchronization between the auricular and ventricular activity during the paced ventricleVVI in patients with node disease.This syndrome can develop in moths even years after the pacemaker implant, being more frequent in elderly patients. The only treatment is to implant an auricular electrode for a synchronic atrioventricular stimulation. It presents the case of a patient diagnosed with a sinus node disease associated to long QT, for this reason it was implanted a pacemaker VVIR, few months later the patient started to have symptoms of heart failure, three years after that presented a syncope episodes showed in a Holter ECG study that reported unsustainable ventricular tachycardia episode and retrograde P waves after every ventricular stimulation sent by the pacemaker. The probability of an arrhythmic syncope was considered treated with Amiodarona. Pacemaker syndrome was diagnosed, making an improvement by a dual chamber pacemaker and all symptoms of heart failure disappeared. Syncopal event was not repeated and from the time the patient is operated on a normal functional capacity.

Siringomielia asociado a Malformación de Chiari tipo I / Associated syringomyelia Chiari Malformation type I

La Siringomielia asociada a Malformación de Chiari tipo I, se refiere a una cavidad formada en la médula espinal debido a la obstrucción del canal medular por el desplazamiento de las amígdalas cerebelosas hacia el agujero magno, manifestándose entre los 25-40 años, con cuadros progresivos de dorsalgias, cervicalgias, dolor de extremidades uni-bilateral, pérdida de sensibilidad; dependiendo del tamaño de la cavidad, la medula espinal puede comprimirse y generar la lesión irreversible de la médula espinal. Se presenta un caso clínico de Siringomielia asociada a Malformación de Chiari tipo I; que presentó cuadro clínico de larga data previa a su diagnóstico; caracterizado por dorsalgias recurrentes que fueron tratadas con diclofenaco y complejo B, pensando en problema muscular. Se le diagnostica por resonancia magnética (RM), y el tratamiento fue la descompresión de fosa posterior; un año después de su tratamiento quirúrgico se le realiza una RM de control donde hay ausencia de la Siringomielia.

Syringomyelia associated with Chiari I malformation, refers to a cavity in the spinal cord because obstruction of the spinal canal by the displacement of the cerebellar tonsils into the magnum foramen, manifested during 25-40 years, with progressive frames of back pain, cervical pain, uni-bilateral limb pain, loss of sensitivity, depending on the size of the cavity, can compress the spinal cord and generate irreversible injury of the spinal cord. It is reports a case of syringomyelia associated with Chiari I malformation that presents longstanding clinical disorder prior to the diagnosis, characterized by recurrent back pain treated just with diclofenac and B complex, thinking as a in muscle problem. Diagnosed by magnetic resonance imaging (MRI), and the treatment was a posterior fossa decompression; a year after the surgery treatment a second control MRI scan was performed with absent of syringomyelia.

Anesthetic management of a patient with Arnold-Chiari malformation type I with associated syringomyelia: A case report

Arnold-Chiari malformation type I (ACM I) is anatomically defined as the displacement of the cerebellar tonsils below the level of the foramen magnum. Syringomyelia is a condition in which a cavity called a syrinx develops in the spinal cord and is filled with cerebrospinal fluid. Here we report the anesthetic management of a case of ACM I with associated syringomyelia scheduled for suboccipital craniectomy, cervical laminectomy and duraplasty.

Cerebellar Ectopia Associated with Unilateral Agenesis of Posterior Arch of Atlas

Chiari type I malformation(CM-I) is a congenital disorder recognized by caudal displacement of the cerebellar tonsils through the foramen magnum and into the cervical canal. Though bony anomalies associated with CM including platybasia, small posterior fossa and occipitalization, are relatively frequent, but the incidence rate associated with agenesis of posterior arch of atlas is very low. We report our experience of surgical treatment for patient who had CM-I with unilateral agenesis of posterior arch of atlas.