RESUMEN
INTRODUCTION: Optic pathway gliomas (OPGs) presenting with acute hydrocephalus represent a true neurosurgical and neurooncological challenge. Two main strategies are currently used: microsurgical removal of the tumor, restoring CSF pathways, and endoscopic biopsy associated with ventriculo-peritoneal shunt implantation. Since the availability of an ultrasonic aspirator handpiece, that can be used inside the working channel of a neuroendoscope, a different less invasive surgical strategy can be used. METHODS: Four pediatric patients were treated by endoscopic ultrasonic aspiration, in order to remove the upper pole of the tumor, obtaining tissue for diagnosis and restoring CSF pathways as initial treatment of OPG invading the third ventricle and causing hydrocephalus due to simultaneous blockage of both Monro foramina and of the Sylvian aqueduct. Surgical technique is described. Pre-operative and post-operative volumes were calculated on magnetic resonance imaging. RESULTS: In all cases, the surgical procedure was uneventful, the biopsy was diagnostic, and CSF pathways were restored. The amount of tumor removed ranged between 31 and 76%. All patients underwent oncological treatment of their tumors. One patient received V-P shunt only 1 year after endoscopic decompression due to tumor progression. CONCLUSION: Our preliminary results show that the use of endoscopic cavitron aspirator is safe and effective to obtain CSF flow restoration and tumor biopsy (for histological and molecular purposes), avoiding CSF shunt implant in the acute phase and offering the chance to obtain a consistent tumor debulking in a minimally invasive fashion. Neuroendoscopic approach together with modern target therapy offers the opportunity to avoid or delay major surgery.
Asunto(s)
Hidrocefalia , Neuroendoscopía , Glioma del Nervio Óptico , Trastornos Respiratorios , Niño , Descompresión Quirúrgica/efectos adversos , Humanos , Hidrocefalia/cirugía , Vértebras Lumbares/cirugía , Neuroendoscopía/métodos , Glioma del Nervio Óptico/complicaciones , Estudios Retrospectivos , Resultado del Tratamiento , Ultrasonido , Ventriculostomía/métodosRESUMEN
BACKGROUND: Chiari malformation type II is present in almost all patients with myelomeningocele but usually remains asymptomatic. Symptoms are generally more severe in neonates, who have the worst prognosis. The association symptoms/hydrocephalus is well known, and first treatment usually consists of ensuring adequate ventricular drainage. Craniovertebral decompression may be required in patients who do not improve after drainage. However, mechanisms of symptom development are not yet completely understood, timing and techniques of surgery are not codified, long-term evolution is poorly reported, and there are few paper reporting clinical onset and treatment in older patients. METHODS: We reviewed our personal series of 42 consecutive symptomatic patients that required surgical treatment. Age at surgery ranged from 1 week to 44 years (mean 6.6 years). Surgical timing strictly depended on clinical conditions: urgent management in the more compromised patients (usually infants) and elective treatment before severe deterioration in patients with less severe conditions. All patients first underwent external ventricular drainage, which resolved the symptomatology in 17 cases (40%). Craniocervical decompression was required by 25 patients (60%) who received no benefit from the ventricular drainage. RESULTS: Early mortality (2 cases = 4.7%) occurred only in neonates. Clinical improvement was achieved in 37 of 40 survivors (92%). During a follow-up ranging from 2 to 20 years (mean 10.3 years), late mortality consisted of 4 cases (10%), mainly due to cardio-respiratory arrest. Twenty-two patients (55%) required surgery for shunt malfunction and 4 for cord detethering. Six patients (15%) required reoperation owing to symptom recurrence. CONCLUSION: Early treatment of symptomatic Chiari II malformations may warrant satisfactory results in a significant number of patients, even in neonates. Nevertheless, overall mortality remains relatively high, throughout the patient life. Formal transition programs and adult spina bifida care processes have become crucial.
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Malformación de Arnold-Chiari , Hidrocefalia , Meningomielocele , Disrafia Espinal , Adulto , Anciano , Malformación de Arnold-Chiari/diagnóstico por imagen , Malformación de Arnold-Chiari/cirugía , Descompresión Quirúrgica , Humanos , Hidrocefalia/cirugía , Lactante , Recién Nacido , Meningomielocele/cirugía , Reoperación , Resultado del TratamientoRESUMEN
INTRODUCTION: Intrathecal baclofen (ITB) is an effective treatment for managing primary and secondary dystonia. Intraventricular baclofen (IVB) was first developed to allow treating patients in which the use of ITB was difficult due to anatomic anomalies. After that, several studies indicate that intraventricular administration of baclofen, is more effective than ITB in refractory dystonia. CLINICAL MATERIAL: We report three cases of children with acute dystonic and dysautonomic storm, treated with IVB. The clinical outcome was satisfactory. The response to the treatment continued after the pump disconnection, suggesting that in this kind of cerebral dysregulations, short-term IVB is an effective treatment. CONCLUSION: Early treatment with IVB may be an effective option in patients with post-anoxic dysautonomic and dystonic storm.
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Enfermedades del Sistema Nervioso Autónomo/tratamiento farmacológico , Baclofeno/administración & dosificación , Baclofeno/uso terapéutico , Distonía/tratamiento farmacológico , Relajantes Musculares Centrales/administración & dosificación , Relajantes Musculares Centrales/uso terapéutico , Adolescente , Enfermedades del Sistema Nervioso Autónomo/complicaciones , Baclofeno/efectos adversos , Niño , Distonía/complicaciones , Femenino , Humanos , Hipoxia Encefálica/complicaciones , Bombas de Infusión Implantables , Inyecciones Intraventriculares , Masculino , Relajantes Musculares Centrales/efectos adversos , Prevención Secundaria , Resultado del TratamientoRESUMEN
OBJECTIVE: Recent trials have shown the safety and benefits of fetoscopic treatment of myelomeningocele (MMC). The authors' aim was to report their preliminary results of prenatal fetoscopic treatment of MMC using a biocellulose patch, focusing on neurological outcomes, fetal and maternal complications, neonatal CSF leakage, postnatal hydrocephalus, and radiological outcomes. METHODS: Preoperative assessment included clinical examination, ultrasound imaging, and MRI of the fetus. Patients underwent purely fetoscopic in utero MMC repair, followed by postoperative in utero and postnatal MRI. All participants received multidisciplinary follow-up. RESULTS: Five pregnant women carrying fetuses affected by MMC signed informed consent for the fetoscopic treatment of the defect. The mean MMC size was 30.4 mm (range 19-49 mm). Defect locations were L1 (2 cases), L5 (2 cases), and L4 (1 case). Hindbrain herniation and ventriculomegaly were documented in all cases. The mean gestational age at surgery was 28.2 weeks (range 27.8-28.8 weeks). Fetoscopic repair was performed in all cases. The mean gestational age at delivery was 33.9 weeks (range 29.3-37.4 weeks). After surgery, reversal of hindbrain herniation was documented in all cases. Three newborns developed signs of hydrocephalus requiring CSF diversion. Neurological outcomes in terms of motor level were favorable in all cases, but a premature newborn died due to CSF infection and sepsis. CONCLUSIONS: The authors' preliminary results suggest that fetoscopic treatment of MMC is feasible, reproducible, and safe for mothers and their babies. Neurological outcomes were favorable and similar to those in the available literature. As known, prematurity was the greatest complication.
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Hidrocefalia/cirugía , Meningomielocele/cirugía , Procedimientos Quirúrgicos Mínimamente Invasivos , Adulto , Femenino , Fetoscopía/métodos , Edad Gestacional , Humanos , Recién Nacido , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Embarazo , Resultado del TratamientoRESUMEN
OBJECTIVE: Severe craniofacial fractures may present different needs in treating intracranial lesions over facial injuries. This paper examines the results of our strategy, consisting of a single-stage combined neurosurgical-maxillofacial treatment. METHODS: A retrospective review was conducted of 33 consecutive patients with complex fractures of the anterior cranial fossa and facial skeleton, who required elective surgery for craniofacial reconstruction. Patients who required emergency surgery for intracranial clots or penetrating wounds were excluded. In all cases, all or almost all the anterior skull-base was injured with compound fractures of the frontal sinus, the orbital roofs, the lamina cribrosa, and the planum sphenoidale. In all cases, the prioritization of treatment was carefully discussed, and surgical timing and strategy were agreed. RESULTS: There was 1 dead. Olfactory injuries were always found intraoperatively. There were no mucoceles, CSF-leak recurrences, cranial infections, or neurologic worsening. The functional and neurologic results were highly satisfactory. CONCLUSIONS: The one-stage surgical treatment of complex craniofacial fractures has numerous advantages, including the possibility of reducing facial fractures without the risk of CSF leaks. It also eliminates the need for repeated procedures in fragile patients, and the need to dismantle the facial reconstruction if the skull base repair is performed later. The main issue is the surgical timing, considering that the maxillofacial surgeon usually favors early facial repair, whereas the neurosurgeon generally prefers delayed manipulation of the contused frontal lobes. A timeframe of 10-14 days after trauma may be a good compromise for safe procedures with excellent neurologic and functional outcomes.
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Procedimientos de Cirugía Plástica , Fracturas Craneales , Humanos , Base del Cráneo/cirugía , Fracturas Craneales/cirugía , Hueso Etmoides/cirugía , Fosa Craneal Anterior/cirugíaRESUMEN
(1) Background: The issue of unsuccessful surgery for Chiari-1 malformation (CM-1), as well as its potential causes and possible solutions, remains poorly documented and studied. (2) Methods: From a retrospective review of a personal series of 98 patients undergoing treatment for CM-1 during the past 10 years, we created two study groups. Group 1: 8 patients (8.1%) requiring additional surgeries owing to postoperative complications (7 cerebrospinal fluid leakage, 1 extradural hematoma); 7 patients (7.1%) undergoing reoperations for failed decompression during the follow-up. Group 2: During the same period, we also managed 19 patients who had previously been operated on elsewhere: 8 patients who required adequate CM-1 treatment following extradural section of the filum terminale; 11 patients requiring reoperations for failed decompression. Failed decompression was managed by adequate osteodural decompression, which was associated with tonsillectomy (6 cases), subarachnoid exploration (8 cases), graft substitution (6 cases), and occipito-cervical fixation/revision (1 case). (3) Results: There was no mortality or surgical morbidity in Group 1. However, one patient's condition worsened due to untreatable syrinx. In Group 2, there were two cases of mortality, and surgical morbidity was represented by functional limitation and pain in the patient who needed revision of the occipitocervical fixation. Twenty patients improved (58.8%), 6 remained unchanged (32.3%), 1 worsened (2.9%) and 2 died (5.9%). (4) Conclusions: The rate of complications remains high in CM-1 treatment. Unfortunately, a certain rate of treatment failure is unavoidable, but it appears that a significant number of re-operations could have been avoided using proper indications and careful technique.