Management and Outcome of a Neurologically Intact Infant with Pierre Robin Sequence and Dandy-Walker Variant Diagnosed with Large Hemispheric Brain Abscess ­ Case Report

Pierre Robin sequence (PRS) is a condition consisting of three essential components: micrognathia or retrognathia, cleft palate, and glossoptosis. It can be part of multiple congenital anomalies. We present the case and outcome of a 3-month-old clinically stable patient who has PRS with Dandy-Walker variant ­ which is a rare presentation in the literature ­ with a large right hemispheric brain abscess, treated with multiple minimally-invasive surgical drainage procedures with adjuvant antibiotics.

Perioperative management of a patient with Dandy Walker malformation with tetralogy of Fallot undergoing total correction and fresh homologous pericardial pulmonary valve conduit implantation: Report of a rare case.

Perioperative management of a patient with Dandy–Walker malformation (DWM) with tetralogy of Fallot (TOF), patent ductus arteriosus, and pulmonary artery stenosis is a great challenge to the anesthesiologist. Anesthetic management in such patients can trigger tet spells that might rapidly increase intracranial pressure (ICP), conning and even death. The increase in ICP can precipitate tet spells and further brain hypoxia. To avoid an increase in ICP during TOF corrective surgery ventriculo‑peritoneal (VP) shunt should be performed before cardiac surgery. We present the first case report of a 11‑month‑old male baby afflicted with DWM and TOF who underwent successful TOF total corrective surgery and fresh autologous pericardial pulmonary valve conduit implantation under cardiopulmonary bypass after 1 week of VP shunt insertion.

Malformación de Dandy-Walker de diagnóstico tardío revelada por hipertensión intracraneal / Late diagnosis of Dandy-Walker’s malformation revealed by intracranial hypertension

La malformación de Dandy-Walker se ha descrito clásicamente por la triada de dilatación quística del cuarto ventrículo, hipoplasia del vermis cerebeloso e hidrocefalia. Los síntomas suelen aparecer en la infancia precoz. La presentación en la edad adulta es extremadamente rara, aunque puede ocurrir de forma espontánea o tras un traumatismo craneal. Caso clínico. Varón de 24 años que acude a urgencias por un cuadro compatible con hipertensión intracraneal. El TAC craneal reveló una malformación de Dandy-Walker. Comentario y Conclusiones. Aproximadamente el 80% de los pacientes con esta malformación se presentan durante le primer año de vida, principalmente con marcrocrania secundaria a la hidrocefalia. Por el contrario, en la edad adulta suelen presentarse con síntomas de disfunción de estructuras posteriores dando lugar a nistagmus, alteración de la marcha y ataxia, aunque también podrían presentar síntomas de hipertensión intracraneal...

Endoscopic third ventriculostomy is a safe and effective procedure for the treatment of Blake's pouch cyst / Terceiroventriculostomia endoscopica e um procedimento efetivo e seguro para o tratamento dos cistos da bolsa de Blake

OBJECTIVE: Blake's pouch cyst (BPC) is a midline cystic malformation of the posterior fossa, within Dandy-Walker's complex (DWC), often associated with hydrocephalus. Endoscopic third ventriculostomy (ETV) has been an alternative to conventional methods for BPC treatment. This study aimed at reporting our experience with ETV in a series of patients with BPC. METHODS: Of 33 patients diagnosed with midline posterior fossa cyst, 26 met the protocol criteria for DWC, and eight subjects with BPC were selected (aged one month to two years old). All cases were treated with ETV. RESULTS: Five patients were male; and three were prenatally diagnosed. They had hydrocephalus and motor deficiencies. Motor assessment at a five-year follow-up yielded normal findings. All patients improved, and only one had residual cognitive dysfunction, despite overall neurological improvement. There were no complications. CONCLUSIONS: ETV was a safe and effective procedure, reducing risks and morbidity associated with open surgery and shunt-related problems. .

OBJETIVO: O cisto da bolsa de Blake (CBB) é uma malformação cística mediana da fossa posterior, dentro do complexo de Dandy-Walker (CDW), frequentemente associada à hidrocefalia. A terceiroventriculostomia endoscópica (TVE) tem sido considerada uma alternativa aos métodos convencionais de tratamento do CBB. Este estudo teve como objetivo relatar nossa experiência com TVE em uma série de pacientes com CBB. MÉTODOS: Dos 33 pacientes diagnosticados com cisto mediano da fossa posterior, 26 preencheram os critérios do protocolo para CDW, dos quais oito foram selecionados com CBB (idades variando entre um mês a dois anos). Todos os casos foram tratados com TVE. RESULTADOS: Cinco pacientes eram do sexo masculino, três tiveram diagnóstico no pré-natal; e todos apresentavam hidrocefalia e alterações motoras. A avaliação motora após cinco anos apresentou resultados normais. Todos os pacientes melhoraram, e apenas um apresentou um leve déficit cognitivo, apesar da melhora neurológica geral. Não houve complicações. CONCLUSÕES: A TVE foi um procedimento seguro e eficaz, reduzindo riscos e morbidade associados à cirurgia aberta e a problemas relacionados aos shunts.

Sindrome de Dandy-Walker: conceptos neuroquirúrgicos

Se presenta un caso con Síndrome de Dandy0Walker. La tomografia axial computarizada del cráneo y cerebro así como el neurosonograma, demostraron claramente la hidrocefalia, la presencia de un quiste de la fosa posterior contínuo con el 4§ ventrículo y la agenesia del cuerpo calloso. Se concluye que el diagnóstico es radiológico. Nuestro método preferencial de tratamento quirúrgico es la derivación de la fosa posterior que ofrece ciertas ventajas técnicas, donde no es necesario combinar la derivación supratentorial