先天性脊柱侧凸患者中的脊髓畸形和脊椎畸形

目的:探讨先天性脊柱侧凸患者的各类脊髓畸形与脊椎畸形之间的关系。方法:对我院2003年～2005年收治的211例已确诊的先天性脊柱侧凸患者的临床资料进行回顾性分析,统计先天性脊柱侧凸患者脊髓畸形、脊椎畸形的发生率及其好发部位,观察两者间的关系。结果:211例先天性脊柱侧凸患者中脊髓畸形44例(20.85%),其中脊髓空洞症25例次(11.85%),脊髓纵裂30例次(14.22%),马尾终丝栓系28例次(13.27%),低位圆锥12例次(5.69%),其他类型少见,多种畸形常并发存在;脊髓畸形好发于脊柱胸段、胸腰段、腰段、腰骶段。44例伴有脊髓畸形的先天性脊柱侧凸病例中不良分节34例(77.27%),孤立性半椎体1例(2.27%),非孤立性半椎体21例(47.73%),脊柱裂30例(68.18%),混合型41例(93.18%);脊椎畸形好发于脊柱胸段、腰段、腰骶段。结论:先天性脊椎畸形常合并脊髓畸形,总体上脊髓畸形与脊椎畸形的好发部位相似,但各种脊髓畸形、脊椎畸形间缺少显著的、特定的对应关系。     

350例脊髓血管畸形介入治疗的护理分析

目的探讨护理工作在脊髓血管畸形介入治疗中的价值。方法通过对我院介入中心350例脊髓血管畸形患者介入治疗,针对脊髓血管畸形患者的临床特点和介入治疗方法的适应证、操作过程和预后等特点,从护理学的角度对术前、术中、术后等阶段进行讨论分析。结果术前进行有效的心理护理和健康教育;术中与介入医生及技术操作人员进行有机的配合、熟练使用输液微量泵、准确使用相关药物及认真观察病情变化;术后对患者进行严密的护理观察,可减少并发症的发生,将有助于该手术的成功。结论介入护理工作对脊髓血管畸形介入治疗手术的成败起着至关重要的作用。     

白消安诱导构建胎鼠肢体畸形模型的研究

目的用易获得的化学药物建立大鼠四肢畸形发生率稳定、畸形类型特异的动物模型。方法采用抗肿瘤类致畸药物白消安作为受试物,观察不同剂量和不同给药时间的胎仔畸形率、畸形类型及特征。结果在大鼠受孕第12天(GD12),一次经口给予白消安25mg/kg时,胎仔畸形类型主要为肢体畸形。肢体畸形率以活胎计为37.9%(33/87),以窝计为61.5%(8/13)。畸形类型常见于多指(趾)和缺指(趾),掌跖骨缺失和骨化不全发生率也较高。此外,还发生胫骨缺失和骨化不全,在观察大体形态时所见的短肢是由胫腓骨缺失和发育不全所致。四肢畸形的发生率和严重程度存在着不对称性,后肢较前肢出现率高,缺指(趾)畸形较其他畸形出现率高。结论成功建立大鼠肢体畸形动物模型,为进一步分析研究肢体发育畸形的分子机制和潜在原因奠定了基础。     

Extended Lateral Supramalleolar Flap for Very Distal Foot Coverage: A Case With Arteriovenous Malformation

Arteriovenous malformation of the foot is very uncommon, and surgical closure after its treatment with embolization and total excision may be challenging for the foot surgeon, particularly in distally localized lesions. A popular method to cover these difficult wounds is free-tissue transfer, which is a highly demanding procedure. Alternatively, distally based regional flaps have been occasionally reported for clinical use in such distant foot defects. Herein, we present a 36-year-old female patient with a diagnosis of arteriovenous malformation arising in the distal medial plantar and dorsal surfaces of the right foot. After surgical resection of the vascular lesion preceded by a misapplied embolization procedure, an extended lateral supramalleolar flap was successfully transferred to the defect area, covering it completely. Functional and aesthetic outcome was satisfactory after 6 months follow-up. Extended lateral supramalleolar flap is a useful and reliable choice for distal foot reconstructions.     

Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma?

Background

The relationship between congenital cystic adenomatoid malformation (CCAM) and pleuropulmonary blastoma (PPB), whether causal, correlational, or coincidental, remains controversial. There is a lack of consensus as to the optimal treatment of patients with asymptomatic CCAM.

Method

We reviewed all cases of CCAM and PPB seen at our institution from 1999 to 2008. Institutional Research Ethics Board approval was obtained. The incidence of CCAM and PPB, respectively, was calculated based on birth numbers during the study period.

Results

Seventy-four CCAMs were resected over the study period in 129 children diagnosed with CCAM. Five PPBs were diagnosed during the study period. Three of the 5 PPB cases were initially diagnosed as CCAMs. These PPBs were not clinically or radiologically distinguishable from CCAMs. In our referral area, the incidence of CCAM was 1 in 12,000; and the incidence of PPB was 1 in 250,000 live births. The mortality rate for PPB in this cohort was 20%.

Conclusion

Asymptomatic cystic lung malformations represent a therapeutic dilemma. In this cohort, the incidence of PPB among apparently benign lung lesions was 4%. No clinical or radiological markers differentiated benign CCAMs from PPBs. Our experience provides further justification for resection of all CCAMs. This should be discussed with parents until CCAMs and PPBs can be clearly distinguished preoperatively.     

可控性丝线留置术治疗体表难治性海绵状静脉畸形的实验及临床研究

目的研究可控性丝线的致栓效果及其治疗体表难治性海绵状静脉畸形的初步疗效。方法将丝线一端留置于不同管腔大小的静脉内,另一端挂于血管壁埋于皮下,实现其可控性,用经过不同处置的丝线,分别留置于兔耳缘静脉、股静脉,观察其引起血管栓塞的效果。选择致栓效果较好的丝线组,用于临床治疗难治性体表海绵状静脉畸形的患者。结果几种经不同处置的丝线,均能不同程度地起到闭塞血管的作用,对于管腔稍大,血流较快的静脉(兔股静脉),浸泡丝裂霉素并打结的丝线组,致栓效果最好;临床初步应用证实,可控性丝线治疗体表难治性静脉畸形,安全有效。结论可控性丝线静脉内留置术,是一种安全、有效、损伤小、操作简便的治疗体表难治性海绵状静脉畸形的新方法。     

CT血管造影在颅内静脉畸形诊断中的应用

目的 评价CT血管造影在诊断颅内静脉畸形中的价值与限度。材料与方法 5例经DSA证实的颅内静脉畸形病例,幕上1例,幕下4例。分别在动脉期（CTA）和静脉期（CTV）扫描采样,运用SSD和MIP进行重建。结果 CTA显示所有病灶,但没有显示颅内静脉畸形的“海蛇头”即引流静脉影像特征,而CTV不仅显示病灶,而且还显示了引流静脉“海蛇头”影像特征。结论 静脉期的CT血管造影（CTV）是诊断和随访颅内静脉畸形的有效方法,优于动脉期的CT血管造影（CTA）。     

Gamma Knife Outcome Models as a Reference Standard in the Embolisation of Cerebral Arteriovenous Malformations

Summary. Summary. Background: We sought to utilise outcome models from Gamma Knife radiosurgery (GKRS) to cerebral arteriovenous malformations (AVM) as a reference standard in assessing the clinical outcome of embolisation, thus comparing the outcomes of two different management alternatives, in the same patients. Methods: 87 consecutive patients with 88 AVM were admitted during 1997–1999 for initial embolisation of an AVM. The clinical outcomes were recorded prospectively. Angiography under stereotactic conditions with measurement of AVM volume was performed before and after embolisation. GKRS outcome models were used to predict obliteration rate, complication rate and risk of haemorrhage before and after embolisation. The clinical outcome of embolisation followed by predicted outcome of adjunct GKRS was then compared with the predicted outcome of GKRS as the only treatment. Findings: Eight patients were subjected to microcatheterisation but not to embolisation. By the end of the study period, embolisation had been terminated in 55 patients out of 80 (69%). The predicted outcome of GKRS alone was 58 obliterations and 12 complications while that of the combined management was 58 obliterations and 15 complications. The difference was not significant on the p<0.1 level. Interpretation: Volume measurement from angiography and outcome models from Gamma Knife radiosurgery are useful as a reference standard in the management of AVM. Absolute volume reduction from embolisation is most prominent for AVM>10 ml and thus facilitates subsequent radiosurgery. For AVM≤10 ml, GKRS as the only treatment can be an alternative to primary embolisation, particularly if no significant volume reduction or obviously beneficial effect of targeted embolisation is expected. Further prospective studies are needed to identify subgroups in which one treatment has advantages over the other.     

Postoperative Fecoflowmetric Analysis in Patients with Anorectal Malformation

Because conventional methods of evaluating anorectal function do not necessarily provide good correlations between investigative results and symptoms in patients who have undergone surgery for an anorectal malformation (ARM), we recently introduced feco-flowmetry (FFM) to simulate natural anorectal evacuation. The purpose of this study was to embody significant parameters to elucidate the dynamics of anorectal activity on FFM. The parameters of FFM were compared with those of manometry and Kelly's clinical score (KCS) in 24 patients who underwent surgery for an ARM. There were three fecoflow patterns, namely, block (B) type, segmental (S) type, and flat (F) type. The B-type or S-type patterns were seen in patients classified as "clinically good." There were close relationships between the fecoflow pattern and both the operative procedure and the KCS (P = 0.01 and 0.001, respectively). Maximum fecal stream flow rate (Fmax) precisely reflected the tolerance rate of intended normal saline solution in the colorectum (TR), the evacuative rate (ER), and KCS. Fmax > 45 ml/s or TR > 70% or ER > 50% was statistically regarded as the borderline of fecal continence. Thus, the fecoflow pattern might reflect the motor activity of the pelvic floor muscle. FFM provided quantiative and qualitative evaluations concerning anorectal motor activity in patients who had undergone surgery for an ARM.