Clinical response to surgical decompression in atypical pediatric Bow Hunter's syndrome suggesting alternative pathophysiology: Case report.

Bow Hunter's syndrome is a rare cause of posterior circulation ischemia, produced by the mechanical and reversible occlusion of the vertebral artery during cephalic rotation. Diagnosis requires clinical suspicion and careful inspection of images with three-dimensional reconstruction. The study of choice is dynamic digital subtraction angiography (DSA). Treatment alternatives are: medical, surgical or endovascular. We report the case of an 8-year-old boy with recurrent infarctions of the posterior circulation secondary to the dissection of the vertebral artery, in association with an occipital bone spur. Dynamic DSA was negative. Conservative initial management was elected with cervical immobilization and anticoagulation, but due to persistence of symptoms, surgical decompression was decided. The patient did not repeat symptoms postoperatively and returned to his usual life. This is the first case reported to our knowledge of a surgical pediatric patient with asymptomatic atypical compression of VA secondary to BHS, whose dynamic angiography was negative, suggesting an alternative mechanism of the syndrome.

Metástasis supratentorial de meduloblastoma de fosa posterior / Supratentorial metastasis of P-fosa medulloblastoma

Objective: our objective is to analyze the patients that presented supratentorial metastases. Material and methods: we studied 170 patients with medulloblastoma from the year 1991 to 2005. Twenty seven presentedsupratentorial metastases. We considered age at presentation, pathology, staging, dissemination to other places and outcome. Results: twenty seven patients (15,8%) with medulloblastoma presented supratentorial metastases, the average age was 5,87 years range from 3 to 11 years. 13/27 were staged high risk and 14/27 standard. The average time between surgery and presence of recurrence was 12,65 month. 12/27 presented desmoplasia. 25/27 patients died. Conclusion: 15,8% of patients with medulloblastoma presented supratentorial metastases before 16 months of the first surgery. Allthe patients were younger than 12 years. The appearance of supratentorial metastases is not related with the stage or the presence of desmoplasia. The outcome was unfavorable once the dissemination was diagnosed.

Fistulas piales cerebrales en pediatría / Pial shunts in pediatric patients

Objective: to analyze our experience in brain pial arteriovenous fistulae in paediatric patients and review the modern concepts about this pathology.Materials and method: between 2004 and 2011, 6 cases of pial arteriovenous fistulae were diagnosed and treated at the National Hospital of paediatrics “Juan P.Garrahan”, defining them as arteriovenous malformations with one or more arterial connections with one single venous drainage, without interposed nidus. We reviewed the medical records and neuroimaging. The main method for diagnosis was cerebral angiography. Results: there were 4 male and 2 female patients, ages ranging from 1 month to 14 years old. 3patients presented brain hemorrhage, 2 had seizures as the initial symptom, and one was diagnosed because of his congenital hydrocephalus. 2 were treated with open surgery and 4 with embolisation. There were no important complications or rebleeding. Conclusion: brain pial fistulas are infrequent vascular malformations important for their high risk of bleeding and relative frequency in paediatrics. The management of these patients needs a multidisciplinary team for analysis, discussion, choosing the most suitable treatment, and follow up.

Lesiones intramedulares que simulan tumor / Spinal cord lesions that mimic neoplasms

Objetivo. Analizar las características clínicas y neurorradiológicas de las distintas lesiones intramedulares con la finalidad de poder diferenciar las lesiones no-tumorales de las tumorales y reservar la exploración quirúrgica sólo para aquellos pacientes sin diagnósticoy con sintomatología rápidamente progresiva. Material y métodos. Se revisaron las historias clínicas de 44 pacientes operados consecutivamente con diagnóstico presuntivo de tumor intramedular entre enero de 1988 y diciembre de 2007. No se incluyeron en el análisis los tumores bulbomedulares ni los del cono y filum ni tampoco los lipomas, teratomas y quistes dermoides.Resultados. Se identificaron 4 pacientes (9.1%), 3 masculinos y 1 femenino, con edad media de 9 años, que presentaban deterioro neurológico progresivo e IRM espinal compatible con tumor intramedular. El resultado histopatológico confirmó en los cuatro casos lesiones no tumorales: infiltrado inflamatorio inespecífico, edema, micosis y gliosis respectivamente.Conclusiones. El minucioso análisis clínico, de laboratorio y el estudio de las imágenes en IRM puede ayudar a la correcta distinción entre tumores y lesiones no-tumorales evitando la exploración quirúrgica que sólo está justificada en los casos muy dudososacompañados de rápido deterioro neurológico funcional del paciente.

Objective. To analyze the clinic and neuro-imaging characteristicsof the intra spinal lesions in order to differentiate the non-neoplasticlesions from neoplastic lesions and to spare surgical exploration only for non-diagnosed patients with rapid deterioration. Materials and method. Fourty-four medical charts of patientsoperated on spinal cord lesions between January 1988 and December 2007 have been analyzed. Results. In four patients (3 male, 1 female – mean age: 9 years)who were operated with diagnosis of intra-spinal tumor, their lesions turned out to be non-neoplastic: non-specific inflammation, edema, mycosis and gliosis, respectively. Conclusions. The thorough clinical and laboratory investigation together with the study of the imaging can help distinguishbetween non-neoplastic lesions from neoplastic lesions in order to avoid surgical exploration which is only suitable in very doubtful cases with rapid deterioration.

Lesiones intramedulares que simulan tumor / Spinal cord lesions that mimic neoplasms

Objetivo. Analizar las características clínicas y neurorradiológicas de las distintas lesiones intramedulares con la finalidad de poder diferenciar las lesiones no-tumorales de las tumorales y reservar la exploración quirúrgica sólo para aquellos pacientes sin diagnósticoy con sintomatología rápidamente progresiva. Material y métodos. Se revisaron las historias clínicas de 44 pacientes operados consecutivamente con diagnóstico presuntivo de tumor intramedular entre enero de 1988 y diciembre de 2007. No se incluyeron en el análisis los tumores bulbomedulares ni los del cono y filum ni tampoco los lipomas, teratomas y quistes dermoides.Resultados. Se identificaron 4 pacientes (9.1%), 3 masculinos y 1 femenino, con edad media de 9 años, que presentaban deterioro neurológico progresivo e IRM espinal compatible con tumor intramedular. El resultado histopatológico confirmó en los cuatro casos lesiones no tumorales: infiltrado inflamatorio inespecífico, edema, micosis y gliosis respectivamente.Conclusiones. El minucioso análisis clínico, de laboratorio y el estudio de las imágenes en IRM puede ayudar a la correcta distinción entre tumores y lesiones no-tumorales evitando la exploración quirúrgica que sólo está justificada en los casos muy dudososacompañados de rápido deterioro neurológico funcional del paciente.(AU)

Objective. To analyze the clinic and neuro-imaging characteristicsof the intra spinal lesions in order to differentiate the non-neoplasticlesions from neoplastic lesions and to spare surgical exploration only for non-diagnosed patients with rapid deterioration. Materials and method. Fourty-four medical charts of patientsoperated on spinal cord lesions between January 1988 and December 2007 have been analyzed. Results. In four patients (3 male, 1 female ¹ mean age: 9 years)who were operated with diagnosis of intra-spinal tumor, their lesions turned out to be non-neoplastic: non-specific inflammation, edema, mycosis and gliosis, respectively. Conclusions. The thorough clinical and laboratory investigation together with the study of the imaging can help distinguishbetween non-neoplastic lesions from neoplastic lesions in order to avoid surgical exploration which is only suitable in very doubtful cases with rapid deterioration.(AU)