Bilateral Macular Edema: A New Ocular Feature of Dandy–Walker Syndrome

To describe a case of bilateral cystoid macular edema in a patient with Dandy–Walker syndrome. An 18-year-old male was referred to our tertiary referral center for evaluation of his decreased visual acuity. Detailed ophthalmic examination and imaging revealed the presence of bilateral cystoid macular edema, which was successfully treated with intravitreal triamcinolone injections (2 mg in 0.05 ml). Recurrence of macular edema developed after a period of approximately four months. This is an unusual ophthalmic manifestation of Dandy–Walker syndrome. Cystoid macular edema should be included in the differential diagnosis of subjects with Dandy–Walker syndrome presenting with decreased vision. The pathogenetic mechanism for the development macular edema in this case is not clear. Intravitreal triamcinolone is an effective treatment, but edema was recurrent in our case. Other approaches (such as oral Acetazolamide or intravitreal Anti-VEGF) have to be considered as well.     

醉香含笑树皮提取物诱导HepG2细胞凋亡的作用机制

目的 探讨醉香含笑树皮提取物（extrat of barks of Michelia macclurei Dandy.,EBMMD）诱导人肝癌细胞HepG2凋亡的作用及相关机理。方法 采用倒置生物显微镜、流式细胞仪、Western-blotting等技术,考察EBMMD对HepG2细胞凋亡及COX-2蛋白表达的影响。结果 流式细胞术分析可见,EBMMD能明显诱导HepG2细胞凋亡,且呈浓度依赖关系;倒置生物显微镜观察可见细胞凋亡的形态学改变;随EBMMD浓度增高,细胞内COX-2蛋白的表达逐渐减少,且当EBMMD浓度达25.00μg.mL 1时,能明显抑制COX-2蛋白表达。结论 EBMMD可以明显诱导HepG2细胞凋亡,其作用机理可能与下调细胞内COX-2蛋白表达进而诱导其凋亡有关。     

The birth and growth of neuroradiology in the USA

Summary Neuroradiology in the USA was started by the neurosurgeons Harvey Cushing, who personally made the first X-rays used to treat a patient with a neurological disability, and Walter Dandy, who first performed air ventriculography, ventriculoscopy, air encephalography and air myelography. The father of neuroradiology in the USA was Merrill Sosman, Cushing's associate. Modern neuroradiology was introduced into the USA from Europe by Mannie Schechter who was one of the original team of three Chief Editors of Neuroradiology.To commemorate the 50th anniversary of the founding of the Harvey Cushing Society (now called the American Association of Neurological Surgeons), and the completion of 10 years of publication of Neuroradiology     

Initial experience with endoscopic third ventriculostomy in Nigeria, West Africa

Objectives Endoscopic third ventriculostomy (ETV) as an alternative to traditional shunt surgery in the management of hydrocephalus of different etiologies is new in Nigeria and West Africa, with no published data till date. This initial study was done to determine the success rate and complication among our patient population. Materials and methods This series consists of a prospective study of the clinical and radiological features and outcome of the first 25 consecutive patients who underwent ETV. ETV was considered to be successful when there was no event occurring during surgery that resulted in the procedure being aborted, postoperative neurological deficit, or adverse event that resulted in a modification of the normal postoperative care. Patients follow-up was for a minimum of 2 months. Results There were 14 males and 11 females. The median age of the patients was 6 months, ranging from 1 month to 48 years. The study included obstructive hydrocephalus because of aqueductal stenosis, Dandy Walker malformation, and pineal region tumor. ETV was successful in 23 patients. Two patients had cerebrospinal fluid leak and superficial surgical site infection. There was no mortality. Conclusion ETV in this series is safe with comparable surgical outcomes to conventional ventriculoperitoneal shunt surgery, and minimal postoperative morbidity has been observed. A commentary on this paper is available at .     

Sonographic findings in a case of tetrasomy 9p associated with increased nuchal translucency and Dandy‐Walker malformation

We report a case of a 23‐year‐old pregnant woman, who underwent amniocentesis after ultrasound (US) examination in the first trimester which revealed a nuchal translucency thickness of 2.9 mm. Cytogenetic analysis revealed complete tetrasomy of the short arm of chromosome 9. Further US evaluation in the second trimester revealed Dandy‐Walker malformation, ventriculomegaly, bilateral clubfoot, lip and palate clefts, arthrogryposis and hyperechoic kidneys with bilateral pelvic dilatation. At 30 weeks of gestation, a placental abruption was noted and a Cesarean section was performed. The infant died shortly after birth. A review of previous cases of tetrasomy 9p shows that the remarkable sonographic findings are ventriculomegaly, intrauterine growth restriction, genitourinary anomaly, Dandy‐Walker malformation, cleft lip/palate and limb malformation, but the association of tetrasomy 9p and increased nuchal translucency had not been reported. © 2009 Wiley Periodicals, Inc. J Clin Ultrasound, 2009.     

Repeated unbalanced offspring due to a familial translocation involving chromosomes 5 and 6

We identified 10 members of a single family with mental retardation and microcephaly, one member having macrocephaly instead. The pedigree was best compatible with the segregation of a small translocation, despite results of previous cytogenetic studies in several relatives being apparently normal. Eventually high resolution and fluorescence in situ hybridization studies in the parents allowed the detection of a balanced translocation between 5qter and 6qter, and of its unbalanced products in the offspring. The pertinent findings from the family are briefly compared with the clinical findings in patients from the literature with either a duplication or deletion of 5q35-ter, or a duplication or deletion of 6q27-ter. Am. J. Med. Genet. 80:448–453, 1998. © 1998 Wiley-Liss, Inc.     

Dandy–Walker syndrome with duplex kidney abnormalities in trisomy 18 – A rare case report

Objective

Trisomy 18 is one of the major numerical chromosomal disorders. The incidence of trisomy 18 is approximately one in 6000 live births. Dandy–Walker malformation (DWM) is the most common congenital malformation of the cerebellum, with an incidence of about one in 5000 live births. The incidence of trisomy 18 associated with DWM is rare and long-term survival rate is very low.