Chiari畸形合并脊髓空洞症术中不同大小后颅窝减压窗近期疗效的比较

目的 探讨不同大小后颅窝骨性减压窗对Chiari畸形合并脊髓空洞症患者手术近期疗效的影响。方法 回顾性分析2007~2010年收治的117例Chiari畸形合并脊髓空洞症患者的临床资料,均采用环枕减压硬膜成形术,空洞横径>脊髓50%者行空洞穿刺减压术。根据后颅窝减压窗面积分为2组:Ⅰ组,87例,减压面积为12 cm2（3 cm×4 cm）;Ⅱ组,30例,减压面积<6 cm2。术后2周采用Tater评分评定疗效。结果 117例患者手术后症状均无加重,症状改善97例（82.9%）,无变化20例。Ⅰ组症状改善76例（87.4%）,Ⅱ组为21例（70%）;两组症状改善率差异显著（P<0.05）。>结论 Chiari畸形合并脊髓空洞症患者的后颅窝减压面积以不小于12 cm2的近期疗效较好。     

Posterior cranial fossa dimensions in the Chiari I malformation: Relation to pathogenesis and clinical presentation

Summary Skull dimensions were measured on lateral skull radiographs in 33 adult patients with MRI-verified Chiari I malformations and in 40 controls. The posterior cranial fossa was significantly smaller and shallower in patients than in controls. In the patients, there was a positive correlation between posterior fossa size and the degree of the cerebellar ectopia, which might indicate that a posterior cranial fossa which was originally too small had been expanded by the herniation of hindbrain structures at an early stage. Pyramidal signs and cerebellar symptoms and signs, which may be due to compression of neural structures, were associated with a large degree of ectopia and a relatively large posterior cranial fossa. Syringomyelia and headache, which may be due to the valve action of the herniated cerebellar tissue, were not associated with a particularly large posterior fossa or herniation. No special clinical presentation was associated with a very small posterior cranial fossa, which may indicate that a small posterior cranial fossa per se has little or no clinical significance, although it may be the primary developmental anomaly.     

Treatment of failed Adult Chiari Malformation decompression with CSF drainage: observations in six patients

OBJECTIVE: We report the use of CSF drainage for the management of failed Adult Chiari Malformation (ACM) decompression. METHODS: All patients with more than one year follow-up after treatment of their failed ACM were included in this study. They underwent initial decompression between September 1998 and April 2000. Clinical and radiological data were collected initially and at recurrence. Lumbar punctures (LP) were done at recurrence for diagnostic and therapeutic purposes. Opening pressures and symptomatic relief were recorded. Therapeutic options included intermittent LP and ventriculo-peritoneal shunting (VPS). RESULTS: There were 6 patients (5 females and one male). Their age ranged from 19 to 43 years. Tonsillar descent ranged from 5 to 21 mm. The symptoms recurred 1.5 to 9 months postoperatively (average 5.6 months). Postoperative imaging revealed the presence of CSF flow behind the tonsils and the formation of a retrotonsillar neocistern in all patients. On LP, the opening pressure ranged from 17 to 31 cm of water (average 23 cm). All patients improved after CSF drainage, and four patients underwent VPS. The other patients were treated with repeat LP+/-Acetazolamide. There was significant improvement in all patients, with 18 months follow-up after CSF drainage (range 16-21 months). CONCLUSIONS: Our results suggest a role for CSF drainage in the treatment of some patients with failed ACM surgery. Possible explanations for the failure of ACM surgery in this subgroup include: surgical complications leading to neural hydrodynamic alteration, inadequate initial surgery, and coexistence with another pathology, possibly a mild form of intracranial hypertension. More prospective and hydrodynamic studies are needed to further clarify these issues.     

Normal Anatomic Variants on Transthoracic Echocardiogram

Apart from their existence as medical curiosities, anatomic variants also double as diagnostic dilemmas. In the heart, more than in any other location in the body, misinterpretation of normal anatomic variants as pathologic entities can have a profound impact on treatment decisions and clinical consequences. Echocardiography is an easily accessible tool these days and is used routinely in most cardiac evaluations. Thus it becomes imperative for the echocardiographer to be cognizant of normal anatomic variants. Furthermore, echocardiographic findings should always be evaluated in their proper clinical context and diagnoses should never be entertained in a clinical vacuum. The literature is replete with numerous case reports and vignettes on these fascinating structures but is lacking in a formal review of normal anatomic variants. In this article, we have attempted a systemic review of normal variants, their embryologic origins, echocardiographic characteristics, and common pitfalls encountered in their evaluation.