Single Burr-Hole Extended Transforaminal Approach for Concurrent Endoscopic Surgery in the Third Ventricle Posterior to the Foramen of Monro and Ventriculostomy: Clinical Series and Planning Steps.

OBJECTIVE: For endoscopic surgery of third ventricular lesions posterior to the foramen of Monro that frequently require a third ventriculostomy during the same procedure, the extended transforaminal approach (ETFA) through the choroid fissure has been proposed. This study reports clinical results and provides anatomic background and guidelines for individual planning of a single burr-hole approach and a safe transchoroid entry zone. METHODS: A retrospective review was undertaken of 25 cases of concurrent third ventricle surgery and third ventriculostomy via ETFA. Assessment was made of a safe transchoroidal entry zone on cadavers (6 hemispheres) and of planning guidelines on magnetic resonance imaging showing occlusive hydrocephalus (30 sides). RESULTS: ETFA was feasible in all 25 cases. The safe transchoroid entry zone was sufficient in 16 cases; in 9 cases, additional transchoroid opening with transection of the anterior septal vein was required without clinical consequences. The anatomic study showed a safe transchoroid entry zone of 5 mm (3-6 mm) for posterior enlargement of the foramen of Monro. Individual planning on magnetic resonance imaging of patients with enlarged third ventricles showed an optimal burr-hole position 22 mm (10-30 mm) lateral to the midline and 8 mm (27 to -23 mm) precoronal; a foramen of Monro diameter of 7 mm (3-11 mm) and a safe transchoroid entry zone of 6 mm (3-12 mm). CONCLUSIONS: According to our data, concurrent endoscopic surgery of third ventricular lesions posterior to the foramen of Monro and ventriculostomy are feasible through a single burr hole and a transchoroid extension of the transforaminal approach. Precise preoperative planning is recommended for anticipating the individual anatomic nuances.

Gómez-López-Hernández syndrome in a child born to consanguineous parents: new evidence for an autosomal-recessive pattern of inheritance?

BACKGROUND: Gómez-López-Hernández syndrome is a rare genetic disease characterized by scalp alopecia with trigeminal anesthesia, brachycephaly or turribrachycephaly, midface retrusion, and rhombencephalosynapsis. We report the second case with this condition who presented with consanguineous parents. PATIENT: This boy was evaluated shortly after birth because of suspected craniosynostosis. He was the only son of healthy, consanguineous parents (his maternal grandmother and his paternal great-grandfather were siblings). His examination was notable for turribrachycephaly, prominent forehead, bilateral parietotemporal alopecia, midfacial retrusion, anteverted nostrils, micrognathia, low-set and posteriorly rotated ears, and short neck with redundant skin. Radiographs and tridimensional computed tomography scan of skull revealed lambdoid craniosynostosis. Brain magnetic resonance imaging revealed complete rhombencephalosynapsis, aqueductal stenosis, fused colliculi, abnormal superior cerebellar penducle, mild ventriculomegaly, and dysgenesis of the corpus callosum. CONCLUSIONS: Since its first description, 34 patients with this condition have been reported. The etiology of Gómez-López-Hernández syndrome is unknown. However, it is noteworthy that the patient in this report presented with a family history of consanguinity because this finding reinforces the possibility of an autosomal-recessive inheritance for this condition.

Quantitative computed tomography and cranial burr holes: a model to evaluate the quality of cranial reconstruction in humans.

Current methods to evaluate the biologic development of bone grafts in human beings do not quantify results accurately. Cranial burr holes are standardized critical bone defects, and the differences between bone powder and bone grafts have been determined in numerous experimental studies. This study evaluated quantitative computed tomography (QCT) as a method to objectively measure cranial bone density after cranial reconstruction with autografts. In each of 8 patients, 2 of 4 surgical burr holes were reconstructed with autogenous wet bone powder collected during skull trephination, and the other 2 holes, with a circular cortical bone fragment removed from the inner table of the cranial bone flap. After 12 months, the reconstructed areas and a sample of normal bone were studied using three-dimensional QCT; bone density was measured in Hounsfield units (HU). Mean (SD) bone density was 1535.89 (141) HU for normal bone (P < 0.0001), 964 (176) HU for bone fragments, and 453 (241) HU for bone powder (P < 0.001). As expected, the density of the bone fragment graft was consistently greater than that of bone powder. Results confirm the accuracy and reproducibility of QCT, already demonstrated for bone in other locations, and suggest that it is an adequate tool to evaluate cranial reconstructions. The combination of QCT and cranial burr holes is an excellent model to accurately measure the quality of new bone in cranial reconstructions and also seems to be an appropriate choice of experimental model to clinically test any cranial bone or bone substitute reconstruction.

Central nervous system abnormalities in patients with oculo-auriculo-vertebral spectrum (Goldenhar syndrome).

OBJECTIVE: To describe the central nervous system (CNS) alterations present in a sample of oculo-auriculo-vertebral spectrum (OAVS) patients, trying to correlate them with other clinical features. METHOD: Seventeen patients with diagnosis of OAVS were evaluated. All presented radiological evaluation of the CNS, normal GTG-Banding karyotype and clinical features involving at least two from the four following areas: oro-cranio-facial, ocular, auricular and vertebral. RESULTS: CNS alterations were verified in eight from seventeen patients (47%). Diffuse cerebral hypoplasia, dilated lateral cerebral ventricles (asymptomatic hydrocephalus), corpus callosum dysgenesis and frontal hypodensities were the most frequent abnormalities. Presence of ophthalmologic abnormalities was the only clinical association observed, being significantly more frequent among patients with cerebral alterations (63% versus 11%). CONCLUSION: CNS abnormalities are frequent in patients with OAVS, especially in carriers of ophthalmologic alterations. However, the absence of detectable cerebral abnormalities did not exclude the possibility that these subjects will subsequently present neurological symptoms.

Central nervous system abnormalities in patients with oculo-auriculo-vertebral spectrum (Goldenhar syndrome) / Anormalidades do sistema nervoso central em pacientes com espectro óculo-aurículo-vertebral (síndrome de Goldenhar)

Objective: To describe the central nervous system (CNS) alterations present in a sample of oculo-auriculo-vertebral spectrum (OAVS) patients, trying to correlate them with other clinical features. Method: Seventeen patients with diagnosis of OAVS were evaluated. All presented radiological evaluation of the CNS, normal GTG-Banding karyotype and clinical features involving at least two from the four following areas: oro-cranio-facial, ocular, auricular and vertebral. Results: CNS alterations were verified in eight from seventeen patients (47 percent). Diffuse cerebral hypoplasia, dilated lateral cerebral ventricles (asymptomatic hydrocephalus), corpus callosum dysgenesis and frontal hypodensities were the most frequent abnormalities. Presence of ophthalmologic abnormalities was the only clinical association observed, being significantly more frequent among patients with cerebral alterations (63 percent versus 11 percent). Conclusion: CNS abnormalities are frequent in patients with OAVS, especially in carriers of ophthalmologic alterations. However, the absence of detectable cerebral abnormalities did not exclude the possibility that these subjects will subsequently present neurological symptoms.

Objetivo: Descrever as alterações do sistema nervoso central (SNC) presentes em uma amostra de pacientes com espectro óculo-aurículo-vertebral (EOAV), tentando correlacioná-las com os demais achados clínicos. Método: Foram avaliados dezessete pacientes com diagnóstico de EOAV. Todos apresentavam avaliação radiológica do SNC, cariótipo por bandas GTG normal e achados clínicos em pelo menos duas das quatro das seguintes áreas: oro-crânio-facial, ocular, auricular e vertebral. Resultados: Alterações do SNC foram verificadas em oito dos dezessete pacientes (47 por cento). Hipoplasia cerebral difusa, dilatação dos ventrículos cerebrais laterais (hidrocefalia assintomática), disgenesia do corpo caloso e hipondesidades frontais foram as anormalidades mais frequentes. A presença de anormalidades oftalmológicas foi a única associação clínica observada, sendo significativamente mais comum entre pacientes com alterações cerebrais (63 por cento versus 11 por cento). Conclusão: Anormalidades do SNC são frequentes entre pacientes com EOAV, especialmente em portadores de alterações oftalmológicas. Entretanto, a ausência de anormalidades cerebrais detectáveis não exclui a possibilidade de que estes indivíduos venham subseqüentemente a apresentar sintomas neurológicos.

Migraçäo dos sistemas de derivaçäo ventrículoperitoneal para o ventrículo lateral. Relato de caso / Migration of netriculoperitoneal shunt system to left ventricular cavity: a case report

É relatado o caso de uma criança submetida à derivaçäo ventriculoperitoneal em que houve migraçäo de todo o sistema de derivaçäo para a cavidade ventricular. A permanência do corpo estranho colonizado dentro do ventrículo lateral impedia a cura da ventriculite

Saída do cateter de derivaçäo ventrículoperitoneal pelo ânus: relato de caso / Extrusion of ventriculoperitoneal shunt through the anus: a case report

É estudado o caso de um lactente que foi sumetido a tratamento cirúrgico de meningomielocele e derivaçäo ventriculoperitoneal (DVP) para tratamento de hidrocefalia descompensada no período neo-natal. Cinco meses após, ocorreu extrusäo do cateter peritoneal através do ânus. Após sua desconexäo ao nível do pescoço, mediante traçöes suaves e repetidas de sua porçäo extrusa, foi realizada remoçäo do cateter. Essa manobra simples evita a necessidade de cirurgia abdominal para a remoçäo do cateter contaminado na porçäo distal

Lipoma of the cerebellopontine angle: case reports and literature review

Säo estudados dois pacientes com lipoma no ângulo pontocerebelar (APC), submetidos a tratamento cirúrgico. A literatura disponível foi revisada, tendo sido identificados 29 casos submetidos a tratamento cirúrgico, com a mesma lesäo. As manifestaçöes clínicas, possibilidades dos métodos diagnósticos, indicaçöes cirúrgicas e estratégias operatórias säo discutidas. Chama-se a atençäo para as peculiaridades dos lipomas do APC e as discutível validade da tentativa de remoçäo completa em todos os casos