Agenesis of the right internal carotid artery and aqueductal stenosis in a child with hydrocephalus, the role of endoscopic third ventriculostomy: a case report.

Agenesis of the internal carotid artery (aICA) is a rare congenital vascular condition that can affect one or both sides of the patient. Most patients remain asymptomatic, but ischemic/hemorrhagic stroke, intracranial aneurysm, and other neurologic findings can occur. CT scan can demonstrate the absence of the bony carotid canal and helps to differentiate a complete aICA from aplasia or hypoplasia. The association of aICA and aqueductal stenosis (AS) has never been reported in the literature. We report the case of a 9-year-old with agenesis of the right ICA associated with AS and hydrocephalus, which was treated successfully with an endoscopic third ventriculostomy (ETV). We review the literature looking for the association of the clinical findings and the evolution of the patient.

Proposed radiological score for the evaluation of isolated fourth ventricle treated by endoscopic aqueductoplasty.

PURPOSE: Evidence supporting the effectiveness of endoscopic aqueductoplasty (EA) for the treatment of isolated fourth ventricle (IFV) is limited to small surgical series of cases. Additionally, studies adopted different radiological outcome criteria, which makes it difficult to compare outcomes accurately. Thus, we aimed to develop a radiological score (RS) as an alternative assessment method for EA. METHODS: The cases of 20 consecutive pediatric patients harboring IFV and treated by EA were retrospectively reviewed. Clinical data and pre- and 1-year postoperative brain images were analyzed. The RS was based on the enlargement of the fourth ventricle and deformation of the cerebellum and brainstem. After randomization, three experts, blinded to patient outcomes, analyzed the brain images and established a consensus for the values of the score. Outcomes were validated by comparing the maximum anteroposterior distance of the fourth ventricle using the RS, pediatric functional status score, and clinical symptoms. RESULTS: The RS was strongly correlated with the anteroposterior distance of the fourth ventricle (Pearson's coefficient = 0.78), and the mean RS dropped from 6.15 to 3.90 (p < 0.001) 1 year after EA. Upward extension (p = 0.021) and brainstem deformation (p = 0.010) were the most significant improved features. There was agreement among RS and symptom improvement in 16 children (80%) and the pediatric functional status score in 14 children (70%). CONCLUSION: In this study, the proposed radiological score proved to be an accurate tool for the evaluation of IFV treatment with EA.

Estenosis acueductal en acondroplasia: a propósito de un caso / Aqueductal stenosis in achondroplasia: report of a case

Achondroplasia is the most frequent cause of disproportionate short stature. Characterized by abnormal growth of long bones, it renders a short-limbed individual of normal intelligence. A serious potential complication is spinal compression, which can happen at any level but is particularly common at the craniocervical junction. It can cause important morbility during the first few years of life, including sudden death. We present a 22-month-old patient diagnosed with achondroplasia, who developed aqueductal stenosis with symptomatic spinal cord compression, diagnosed during a routine consultation, requiring decompressive surgery with excellent results.

La acondroplasia es la condición asociada a talla baja desproporcionada más frecuente, caracterizada por un crecimiento óseo anormal, que resulta en talla baja con extremidades cortas e inteligencia normal. Una de las complicaciones más habituales es la compresión medular, que puede ocurrir a cualquier nivel, siendo más frecuente en la unión cráneo cervical, generando alta morbimortalidad en los primeros años de vida, principalmente por muerte súbita. Presentamos una paciente de 1 año 10 meses con diagnóstico precoz de acondroplasia, que presentó en su evolución estenosis acueductal con compresión medular, sintomática, pesquisada en control rutinario, que requirió cirugía descompresiva con buena evolución posterior.

Endoscopic management of hydrocephalus due to neurocysticercosis.

OBJECTIVE: Hydrocephalus due to neurocysticercosis usually shows poor prognosis and shunt failure is a common complication. Neuroendoscopy has been suggested as treatment, but the indications remain unclear. METHODS: A cohort of patients with clinical/radiological diagnosis of hydrocephalus due to NCC, treated between January 2002 and September 2006, were the subjects of the study. We excluded patients with tumors or those in whom diagnosis was not confirmed (histology/positive ELISA in CSF). Neuroendoscopy was offered as the first line of treatment. Shunt failure rate and Karnofsky index at 12 months were assessed. RESULTS: Eighty-six patients (47 male) with a median age of 38 (9-79) were included in the study. Of them, 36.1% had a shunt before endoscopy and 97.7% had a Karnofsky index <80. We did not find the parasite in 18.6%, extraction was achieved in 79%, and in 87.2% an endoscopic third ventriculostomy (ETV) was performed. The median follow-up time was 43 months (1-72). Shunt failure was seen in 6.6% of patients with ETV in comparison to 27.2% in those without ETV. A hazard ratio of 0.22 (95% CI, 0.05-0.93) for shunt failure after ETV was calculated. At 12 months, 20.9% had a Karnofsky index <80. CONCLUSION: Early extraction of parasite plus ETV might allow improving outcome and reducing shunt failure. Limitation of inflammatory stimulation by parasite antigens and improvement of CSF dynamics could be an explanation for these findings.

Venous lesions as a cause of sylvian aqueductal obstruction: case report.

OBJECTIVE: The cases of two patients who presented with sylvian aqueductal obstruction caused by venous lesions (an ectatic vein and a developmental venous anomaly, which are uncommon causes of obstructive hydrocephalus) are reported. CLINICAL PRESENTATION: A 42-year-old man presented with chronic headache and behavior abnormalities. Magnetic resonance imaging revealed supratentorial ventricular dilation caused by an obstruction of the sylvian aqueduct by a developmental venous anomaly. An 18-year-old man complained of intermittent headaches. The magnetic resonance imaging scan disclosed aqueductal stenosis-type hydrocephalus secondary to an ectatic vein. INTERVENTION: Both patients underwent endoscopic third ventriculostomy and recovered well. During the procedures, the aqueductal obstruction by venous elements could be seen clearly. CONCLUSION: Venous anomalies may cause obstructive hydrocephalus and can be suspected in cases of chronic and intermittent headaches. Endoscopic third ventriculostomy is an effective treatment.

Endoscopic aqueductoplasty in the treatment of aqueductal stenosis.

OBJECTIVE: Endoscopic aqueductoplasty is an option of treatment of obstructive hydrocephalus caused by aqueductal stenosis. We report on our experience with this endoscopic technique. MATERIALS AND METHODS: Eighteen patients with primary or secondary aqueductal stenosis underwent endoscopic aqueductoplasty (EA) with or without stenting between July 2004 and January 2007. EA, EA with a stent, EA with endoscopic third ventriculostomy (ETV), and EA with stenting in addition to ETV were performed in eight, five, three, and two patients, respectively. A repeat endoscopic procedure was done in one patient. EA with a stent was performed in case 1, 8 months after first endoscopic procedure. In four cases, aqueductoplasty with stent was performed through a suboccipital approach. RESULTS: There were no deaths due to the neuroendoscopic procedures. All of the patients showed improvement or resolution of their preoperative symptoms, although in case 1 a new endoscopic procedure was performed: EA with a stent. CONCLUSION: Cerebral aqueductoplasty is an effective and successful treatment for membranous and/or short-segment stenosis of the sylvian aqueduct. Endoscopic aqueductoplasty candidates must be selected very carefully but longer follow-up periods are necessary to evaluate long-term aqueductal patency after aqueductoplasty.

Bruns syndrome caused by intraventricular neurocysticercosis treated using flexible endoscopy.

OBJECT: Neurocysticercosis is the most frequent cause of hydrocephalus in adults in regions where the disease is endemic, including Latin America. The prognosis for intraventricular neurocysticercosis is worse than that for the intraparenchymal form of the disease, making treatment especially important. Although active and viable intraventricular cysts produce no reaction in the host, they can cause noncommunicating hydrocephalus, whose onset is frequently abrupt. Sometimes the increasing intracranial pressure due to obstruction of the cerebral aqueduct (ball-valve mechanism) is intermittent, producing relapsing/remitting symptoms; this life-threatening phenomenon is called "Bruns syndrome." METHODS: Between 1996 and 2004, among a group of 285 patients with neurocysticercosis and Bruns syndrome caused by cysticercal cysts of the third ventricle was diagnosed in seven patients by using magnetic resonance imaging. An endoscopic procedure with a flexible cerebral endoscope was performed, intact parasitic cysts were removed, and a complete exploration was undertaken to look for more cysticercal cysts in the whole ventricular system and the subarachnoid basal cisterns. There were no deaths or complications. All seven patients were asymptomatic during a follow-up period ranging from 1 to 5 years. CONCLUSIONS: Flexible cerebral endoscopy allows one, in a minimally invasive manner, to approach the ventricular system and subarachnoid basal cisterns and to remove intraventricular neurocysticercal cysts. Flexible endoscopy is an alternative treatment for Bruns syndrome caused by neurocysticercosis of the third ventricle.

Anatomía quirúrgica endoscópica del acueducto mesencefálico / Surgical endoscopic anatomy of the mesancephalic aqueduct

Objetivos: Describir la anatomía endoscópica del acueducto mesencefálico. Detallar la técnica para el abordaje con éxito de la reción. Enumerar los reparos anatómicos endoscópicos que el cirujano debe conocer para llevar a cabo con éxito el abordaje endoscópico. Método: Se utilizaron diez cadáverez adultos, obteniendose imágenes de 14 cirugías. Utilizamos endoscopios rígidos de cero grados para la inspección del acueducto mesencefálico. En aquellos casos en los que se observó una estenosis del acueducto llevamos a cabo una acueductoplastia con balón. Con la ayuda de un endoscopio flexible se exploró el acueducto y el cuarto ventrículo y se preforaron las obstrucciones membranosas. en un caso fue necesario insertar un stent en el acueducto. Resultados: El acueducto mesencefálico, también llamado acueducto de Silvio, comunica el tercer ventrículo con el cuarto. La entrada al mismo se encuentra en la pared posterior del tercer ventrículo. Esta pared es muy estrecha y se pueden identificar en ella varias estructuras, que en sentido cráneo-caudal son: el receso suprapineal, la comisura habenular, el receso pineal, la comisura posterior, y la entrada al acueducto. En condiciones normales, la entrada al acueducto en el piso del tercer ventrículo es de un ancho aproximado de 1 mm, por lo tanto, el escaso diámetro impide su canulación segura con elementos endoscópicos. En los casos en que el acueducto se encuentra dilatado se constituye en el camino ideal para llegar al cuarto ventrículo y navegar a través del mismo sin dificultad. Conclusión: La región del acueducto es de difícil abordaje, por lo tanto es imprescindible conocer la anatomía endoscópica para poder llevar a cabo con éxito los diversos procedimientos quirúrgicos. (AU)

Derivaçäo ventricular cerebral: experiência com 35 pacientes / Cerebral ventricular shunt: experience with 35 patients

Os autores apresentam a experiência adquirida com parte dos pacientes submetidos às derivaçoes ventricular, peritoneal e atrial, desde o início do Serviço de Neurocirurgia, em 1983. A técnica operatória, apesar de simples, tem ainda muitas controvérsias, em virtude do número elevado de revisoes que os sistemas de inúmeras procedências necessitam, levando ao aparecimento de complicaçoes, algumas vezes graves. Com estas ressalvas, a mortalidade foi de 14,28 por cento, comparável a outras séries. O primeiro shunt peritoneal é creditado a Ferguson, em 1898. Foi feito com agulha de prata, comunicando o espaço subaracnóide com o peritônio, através do corpo vertebral de L5. Este e outros métodos utilizados em tempos mais remotos falharam e novas tentativas somente foram feitas e publicadas 25 anos após. Quando a válvula Spitz Holter e os cateteres flexíveis começaram a ser usados, a cirurgia do shunting começou a ficar mais popular. Desde entao vem-se notando que as complicaçoes com a DVA usada na época nao eram tao infreqüentes. A partir de 1967, Ames & Murgathi chamaram a atençao para a possibilidade de utilizar a cavidade peritoneal para o desvio ventricular. Trabalhos mais recentes têm mostrado que tanto a DVA quanto a DVP sao eficientes para o tratamento dos hidrocéfalos, existindo significantes vantagens para o sistema peritoneal quando se leva em conta a gravidade das complicaçoes com a DVA.