Dynamic aspects of expanding cava septi pellucidi et Vergae

Summary Two paediatric patients with expanding cysts of the cava Vergae et septi pellucidi are presented. In the first patient, consecutive CT scans showed agrowing cavum thought to be responsible for his dramatic increase in head circumference. In the other patient, the expanding cavum was discovered because a routine skull X-ray after minor head trauma revealed marked impressiones digitatae.Both patients were successfully treated with stereotactically placed internal shunts from the cysts via the lateral ventricle to the subarachnoid space. During this procedure, contrast medium was instilled, and the cysts were visualized on postoperative CT scans.Some dynamic aspects of such expanding cava are discussed.     

Cardiovascular autonomic dysfunction in normotensive awake subjects with obstructive sleep apnoea syndrome

Cardiovascular autonomic function in normotensive awake patients with obstructive sleep apnoea syndrome was studied in 21 normotensive (mean age 48 ± 14 years), drug-free men with obstructive sleep apnoea syndrome. Cardiovascular reflex tests with continuous blood pressure monitoring and biochemical indices were performed the morning after a standard polygraphic sleep recording. A group of 20 agematched (mean age 49 ± 19 years) normal subjects was used as controls. The obstructive sleep apnoea syndrome patients showed higher heart rate and noradrenaline plasma levels (p < 0.05) at rest and a higher blood pressure response to head-up tilt (p < 0.01), suggesting sympathetic overactivity. Respiratory arrhythmia, baroreflex sensitivity index and Valsalva ratio were significantly lower in the obstructive sleep apnoea syndrome group (p < 0.01) whereas the decrease in heart rate induced by the cold face test was significantly higher (p < 0.05) showing a blunting of reflexes dependent on baroreceptor or pulmonary afferents with normal or increased cardiac vagal efferent activity. These abnormalities in autonomic regulation may predispose obstructive sleep apnoea syndrome patients to cardiovascular complications like hypertension and cardiac arrhythmias.     

Molecular breakpoint analysis and relevance of variable mosaicism in a woman with short stature,primary amenorrhea,unilateral gonadoblastoma,and a 46,X,del(Y)(q11)/45,X karyotype

Congenital hydrocephalus associated with aqueductal stenosis and/or agenesis of the corpus callosum has been described in newborn males with mutations in L1CAM, a gene that encodes a neural cell adhesion molecule. These males usually have severe mental retardation and may have spastic paraplegia and adducted thumbs. In contrast, Hirschsprung disease, or absence of ganglion cells in the distal gut, has rarely been described in such individuals. We report a male infant who had severe hydrocephalus identified in the prenatal period with evidence of aqueductal stenosis and adducted thumbs at birth. He developed chronic constipation, and rectal biopsy confirmed the diagnosis of Hirschsprung disease. Molecular testing of the L1CAM gene revealed a G2254A mutation, resulting in a V752M amino acid substitution. A common polymorphism in RET, but no mutation, was identified. Our patient represents the third example of coincident hydrocephalus and Hirschsprung disease in an individual with an identified L1CAM mutation. We hypothesize that L1CAM‐mediated cell adhesion may be important for the ability of ganglion cell precursors to populate the gut, and that L1CAM may modify the effects of a Hirschsprung disease–associated gene to cause intestinal aganglionosis. © 2002 Wiley‐Liss, Inc.     

阻塞性黄疸:PTC下胆管钳夹活检的技术方法学研究

目的探索切实可行的胆管病理学检查新途径.资料与方法连续92例阻塞性黄疸患者接受经皮肝穿刺胆管造影(PTC)和经皮肝穿胆管引流(PTCD)治疗.PTCD过程中,影像监测下经皮经肝胆管穿刺,向胆管内引入活检钳对梗阻段钳夹活检,行组织病理学检查.统计学分析用χ2检验或Fisher确切概率计算法,以α=0.05作为检验水准.结果 92例钳夹活检患者90例成功获得组织块,技术成功率97.83%(90/92).钳夹活检敏感性为88.04%,63例胆管癌性恶性肿瘤钳夹活检敏感性较25例非胆管癌性恶性肿瘤高(93.65%比72.00%,P＜0.05).结论PTC下胆管钳夹活检操作简单,创伤小,敏感性高,是一种值得推广的胆管病理学诊断新途径.     

阻塞性睡眠呼吸暂停综合征患者肾功能和心钠素的变化分析

目的探讨病人肾功能、心钠素(ANP)的含量变化及经鼻持续气道正压(nCPAP)治疗对阻塞性睡眠呼吸暂停综合征(OSAS)患者的影响。方法选择经多导睡眠图(PSG)确诊为中、重度的OSAS患者11例为试验组,PSG检查正常者14例为对照组，试验组给予nCPAP治疗，分别测定其血尿肌酐、尿量、尿β2-微球蛋白(β2-MG)、血浆心钠素(ANP)，并计算出内生肌酐清除率(Ccr)，比较试验组与对照组及试验组治疗前后各项指标的差异。结果试验组与对照组比较，Ccr,尿β2-MG差异无显著性，经nCPAP治疗后两指标也无明显改变；试验组的夜尿量、血ANP含量显著高于对照组，nCPAP治疗后上述指标均较治疗前明显下降。结论OSAS患者夜尿量增多可能与ANP增加有关，这种增高可以被nCPAP治疗所纠正。     

Ventriculosubcutaneous shunt for temporary treatment of neonatal post-IVH hydrocephalus: a technical note

A technique for temporary ventricular drainage using a subcutaneous pouch is described for use in neonatal hydrocephalus after intraventricular hemorrhage. The advantages include continuous, valve-regulated ventricular decompression, no exposed tubing, avoidance of electrolyte imbalance and of the loss of CSF, obviation of the need for repeated tapping, and the ability to convert to a peritoneal shunt on an elective basis.     

Optic chiasm glioma associated with inappropriate secretion of antidiuretic hormone,cerebral ischemia,nonobstructive hydrocephalus and chronic ascites following ventriculoperitoneal shunting

An optic chiasm glioma may cause loss of vision, endocrine disturbances, hydrocephalus and cerebral ischemia due to its proximity to the pituitary, hypothalamus, III ventricle and internal carotids. A 3-month-old infant with optic chiasm glioma developed hypopituitarism and inappropriate secretion of antidiuretic hormone with plasma hypo-osmolality. The cerebrospinal fluid (CSF) protein concentration was markedly elevated. The impairment of fluid absorption via arachnoid villi and peritoneum by the high protein content, and reversed osmotic gradient between protein-rich CSF and hypo-osmolar plasma may have contributed to both nonobstructive hydrocephalus and recurrent ascites following ventriculoperitoneal shunting. Cerebral ischemia from carotid compression may have led to cerebral atrophy.     

Experimental Hydrocephalus in Suckling Hamster Induced by Myxovirus Infection

Abstract This study was undertaken to elucidate the pathogenesis of the hydrocephalus and aqueductal stenosis induced by intracerebral mumps virus inoculation in suckling hamsters.
Mild ventricular dilatation became apparent after 5 days of inoculation. Focal denuding of the ependymal layer and subsequent aqueductal stenosis were observed by 14 days after inoculation. The virus antigen was detected not only in the ependymal cells and choroid plexus, but also in some neurons in the cerebral cortex, hippocampus, midbrain and cerebellum. In the cerebral aqueduct, the orderly arrangement of the cilialy clusters was destroyed on the 5th day after inoculation. After 10 days, proliferation of GFAP positive cells was noticed around the cerebral aqueduct and subsequently caused aqueductal stenosis. In the advanced state of hydrocephalus, the cerebellum was displaced downward and showed an elongated, atrophic and sleevelike structure similar to the Arnold-Chiari malformation. It was suggested that the extensive damage of the ependymal cilia may account for early ventricular dilatation, and subsequent aqueductal stenosis with glial proliferation is the main cause of the advanced hydrocephalus. It has not yet been determined whether the mumps virus can pass through the human placenta or not. If it can, however, our results strongly suggest that mumps virus infection in the human fetus will cause congenital hydrocephalus.     

悬雍垂保留在悬雍垂腭咽成形术中的作用

目的 探讨悬雍垂保留在悬雍垂腭咽成形术(UPPP)中的作用。方法 比较43例UPPP与43例保留悬雍垂的改良UPPP的术中术后并发症、术后症状改善程度以及术后咽腔测量结果。结果 术中术后大出血、开放性鼻音、进流食返呛人鼻腔等并发症：改良UPPP明显低于UPPP(P＜0．05)；咽异物感：改良UPPP极明显低于UPPP(P＜0．01)。术后1年患者主观症状改善程度：明显改善者，改良UPPP高于UPPP，无改善者，UPPP高于改良UPPP，有显著性差异(P＜0.05)。术后1年咽腔扩大程度：下腭咽横径、前后径，改良UPPP较UPPP两者均明显扩大(P＜0.05)。结论 UPPP中保留悬雍垂可明显降低术中术后大出血、开放性鼻音、进流食返呛人鼻腔、咽异物感等并发症，术后症状明显改善，咽腔得到明显扩大。