Congenital subglottic stenosis in two unrelated pairs of siblings

Subglottic stenosis in infants is mostly acquired (secondary) and caused by granulation tissue or submucosal mucous gland hyperplasia after prolonged endotracheal intubation. Subglottic stenosis may also be congenital (primary), and it usually occurs sporadically. There are some reports of its association with inherited anomalies, but there are no previous reports of its familial occurrence in otherwise healthy children. This report describes two pairs of siblings referred for acute inspiratory stridor in whom subglottic stenosis was diagnosed by endoscopy. They were all born at term, and their parents were unrelated. One child had an anteriorly located anus but no other abnormalities. CONCLUSION: Without any surgical intervention all had normal breathing at rest, but inspiratory stridor during respiratory infections and upon physical exercise at follow-up 4-9 y later.     

Essentials of shoulder MR arthrography: a practical guide for the general radiologist

Lesions of the glenoid labrum and ligamentous structures commonly occur secondary to shoulder trauma and are a frequent cause of shoulder joint instability. Numerous eponyms, acronyms and subclassifications are used to describe the often confusing array of bony and labro-ligamentous abnormalities of the shoulder. This aim of this review is to illustrate the relevant features of these lesions and to provide a systematic and practical approach to imaging of the shoulder using MR arthrography.     

Congenital anterior urethrocutaneous fistula associated with urethral duplication

Congenital anterior penile urethral fistula is a rare anomaly; only seven cases have been reported to date. This report outlines the clinical presentation and the embryologic mechanism of a case associated with urethral duplication.     

小儿孟氏骨折脱位扣眼畸形的病理认识以及环状韧带重建术的探讨

目的 对小儿盂氏骨折脱位及陈旧性盂氏骨折的传统治疗方法、创伤病理解剖、治疗结果及发生漏、误诊、误治的病例进行研究,以找到解决问题的方法,提高疗效。方法 对170例盂氏骨折脱位患儿的致病原因、治疗方法、术中所见、手术方式以及治疗效果等进行研究、分析。结果 83例中,优59例,良15例,一般9例,差5例,结论 （1）盂氏骨折脱位的定义应为：尺骨干骨折合并桡-肱及桡-尺脱位,合并/或不合并桡骨上1/3骨折、桡骨颈骨折、桡骨头骨折脱位;（2）尺骨鹰嘴骨折合并肘关节脱位不应诊断为盂氏骨折;（3）提出“超伸直型”盂氏骨折的概念;（4）尺骨骨折的稳妥治疗及坚强内固定是治疗盂氏骨折的关键;（5）桡骨颈骨折既要对位好,更要对线好,以免以后发生旋转障碍;（6）急诊患儿可行环状带修补术及关节囊紧缩术,晚期患儿应行关节囊紧缩术,不主张行环状韧带重建术。     

新生儿外科畸形的早期综合干预

目的通过产房外科等早期综合干预以提高新生儿外科畸形的临床诊治水平。方法对20例产前超声检查存在异常表现的胎儿进行B超和MRI随访观察，发现脐膨出6例，腹裂5例，消化道畸形6例，其他3例。18例行手术治疗，其中产房外科11例。术后多途径营养支持14例。采集脐带血20例。结果20例中治愈14例，因多发严重畸形和家属放弃治疗死亡6例。11例施行产房外科，其中腹裂5例，治愈4例；巨型脐膨出4例，治愈1例，术后31d意外死亡1例，放弃治疗死亡2例，普通型脐膨出2例Ⅰ期手术治愈。消化道畸形5例治愈，1例好转。脐带血成功回输12例。结论对新生儿外科畸形早期诊断和综合治疗，有助于避免严重并发症，降低病死率。产房外科的实施，能在最短时间内中断疾病对患儿的损害。产时脐带血采集和自体回输，提高了输血安全性。     

超声检测中晚期孕产妇妊娠异常分析

目的探讨彩色多普勒超声诊断胎儿先天性畸形的声像图特征和临床实用价值。方法用彩色多普勒超声常规检查胎儿畸形情况。结果检查孕妇3899例中，共查出胎儿畸形26例，其中神经管畸形10例最多。结论超声检查对诊断胎儿畸形有重要价值，是产前诊断的首选方法。     

64排螺旋CT内耳结构三维重建及临床应用

目的探讨64排螺旋CT对内耳结构的重建方法及典型发育畸形的诊断价值。方法选取怀疑内耳病变的患者40例，经64排螺旋CT扫描，将原始图像采用骨算法重建为层厚0．6mm、层间隔0．4mm的薄层图像，然后采用多平面重建（MPR）的方法．显示半规管、耳蜗、前庭结构等结构。结果40例患者．共80耳，经MPR重建后，清晰显示水平半规管78个，后半规管79个．前半规管78个．前庭80个。发现半规管畸形6个。耳蜗畸形8个。结论64排螺旋CT能够清晰地显示内耳正常结构及发育畸形。     

特重度烧伤后伴多处严重畸形的功能重建

To explore new measures for functional reconstruction of multiple severe deformities as a result of extensive deep burn( total burn surface area ≥90% TBSA, including deep burn ≥ 70% TBSA)in late stage. Twelve severe bum patients with above-mentioned deformities were hospitalized in our ward during 1960 ～ 2005,the sears resulted from bums were distributed from head to foot with 173 deformities, including 27 scar ulcers. All patients lacked of self-care ability, among them some could not stand. Due to inadequate skin source, deformities were corrected by skin from matured scars expanded with subcutaneous balloon at late postburn stage. Following our former clinical experience, anatomic investigation and experimental re search, we chose the following methods to correct deformities and restore functions: application of split-thickness scar skin after expansion(88 wounds) ; use of sear skin flap/scar-Achilles tendon flaps (59 wounds) ; combination of thin split-thickness skin grafts from scar and allogeneic acellular dermal matrix (composite skin,40 wounds).All grafts survived, the appear ance and function were improved obviously without complica tions. Follow-up 1～ 40 years, all patients could take care them selves with satisfactory function and appearance, and among them 8 patients returned to work ( one had worked for 40 years),2 pa tients married and had children. The above-mentioned measures are safe, reliable and effective for functional reconstruction of deformities.     

暴力手法松解粘连致股骨髁上骨折畸形愈合1例

患者男性，21岁，因“左股骨干骨折内固定取出术后膝部畸形、活动受限2个月”入院。询问病史，患者于2006年2月曾因车祸致左股骨干骨折、髌骨骨折，在当地医院行手术治疗。髌骨骨折给予石膏固定。3个月后出现膝关节屈曲功能障碍，本次入院12个月前患者于当地医院行内固定取出，同时在麻醉下行手法松解。术后患者出现膝关节疼痛、屈伸功能障碍，查体见左膝关节呈屈曲畸形，膝关节无法伸直，主被动活动明显受限，     

Use of computed tomography to categorize the type of tracheal agenesis

We report a case of type I tracheal agenesis with vertebral defects, anal atresia, tracheoesophageal fistula and/or esophageal atresia, radial dysplasia, renal defects, and cardiovascular defects association in which computed tomography was helpful in categorizing the diagnosis. This rare and fatal condition should always be considered when confronted with a neonate impossible to resuscitate at birth.