先天性右膝关节脱位2例报告

例1:女2天。右膝关节活动少伴肿胀2天。患儿系第1胎第1产,孕37周,顺产,生后无窒息。其母孕期无明显外伤史。查体:体温36、,呼吸40次/分,脉搏120次/分,体重3.37kg。精神反应好,心、肺、腹无异常。右膝半屈位,略肿,髌骨可触及,膝关节被动伸展可达60°位,屈曲100°位,完全伸直有侧向活动,浮髌试验阴性,右肢色泽正常,各足趾可主动屈伸。左膝关节检查正常。余无畸形。X线:双膝关节所示诸骨骨质清晰,骨小梁、关节、关节间隙正常,股骨及胫腓骨未见异常。诊断:先天性右膝关节脱位。给予石膏绷带屈膝15°功能位固定。例2:女,1天,右膝关节过度伸展1天…     

同侧先天性髋及膝关节脱位一例

同侧先天性髋及膝关节脱位国内未见报道。我院 2 0 0 2年 8月收治 1例 ,治愈 ,报告如下。患儿 :女 ,第一胎 ,足月顺产 ,体重30 0 0 g。产前及产时检查胎位未发现异常。产后发现左髋屈曲、左膝反曲畸形 ,左足可接触左肩部 ,足趾活动正常 ,被动屈膝时有弹性抵抗 ,膝前部皮肤有皱褶 ,肥国窝皮肤光滑 ,左膝呈过伸 4 5°位 ,被动屈曲约 30°。可接触髌骨 ,较对侧明显减小 ,可活动。作抽屉试验及分离试验 ,亦未见异常。双下肢等长 ,臀纹对称。因患儿家长拒绝拍片 ,未获早期资料。即日给患儿手法复位 ,用铝板固定左膝屈曲 30°位。 2周后给患儿手法…     

Pavlik挽具治疗新生儿及婴儿先天性髋关节脱位

Ｐａｖｌｉｋ挽具治疗新生儿及婴儿先天性髋关节脱位朱葆伦，董馥荣１９５７年捷克的Ｐａｖｌｉｋ首先设计介绍了Ｐａｖｌｉｋ装置，以后国外有不少报道。我院自１９８９年５月～１９９１年６月应用Ｐａｖｌｉｋ挽具治疗新生儿及婴儿先天性髋关节脱位（ＣＤＨ）共１００例...     

Pavlik挽具有限制动治疗新生儿股骨干骨折

目的探讨Pavlik挽具有限制动治疗新生儿股骨干骨折的临床疗效。方法 2005年1月-2010年1月,应用Pavlik挽具有限制动治疗新生儿股骨干骨折47例。其中男26例,女21例;左侧16例,右侧31例。臀产位13例,剖宫产29例,正常分娩5例。治疗时年龄12 h～23 d。骨折发生于股骨干中上1/3段38例,中1/3段骨折9例;斜形或螺旋形33例,横行骨折14例。大腿外观有明显向外成角及重叠缩短畸形。成角移位平均45°(22°～80°),短缩移位平均1.0 cm(0.5～2.0 cm)。患儿下肢足趾血运好,无其他合并伤。治疗采用Pavlik挽具,双膝屈曲、双侧髋屈曲外展有限制动。定期随访复查X线片及适时调整挽具。结果 Pavlik挽具应用最短3周,最长6周,平均4周。X线片示骨折线模糊,连续性骨痂形成后去除Pavlik挽具,并定期随访至学步期。平均随访20个月(8个月～4 a),47例骨折均一期治愈,患侧肢体外形好,无短缩,无跛行步态。结论 Pavlik挽具有限制动治疗新生儿股骨干骨折方便简捷、安全可靠、疗效好、并发症少,不需要住院或住院时间短。     

先天性膝关节脱位一例

患儿 :男 ,1岁 6个月 ,足月顺产 ,出生后左膝关节过伸位不能弯曲。体检 :左膝关节过伸位不能活动 ,股骨髁向后 ,胫骨向前移位 ,膑骨形态正常 ,被动活动膝关节过伸 30°以上 ,胫骨可前后推移 ,股四头肌无挛缩 ,膑上囊触不清 ,行膝关节复位后外形肿胀 ,无其他畸形。Ｘ线片示 :胫骨向前移位。家族史无类似病例 ,其母亲无致畸药物服用史。治疗 :左膝关节复位屈曲 30° ,绷带固定 2周 ,解除固定后关节活动正常。随访结果 :一年半时关节外形无异常 ,活动自如 ,行走如同龄儿。讨　　论先天性膝关节脱位是一种少见的先天性畸形 ,病因不明 ,少数有家…     

改良Langenskiold术和Grammont术联合治疗先天性及习惯性髌骨脱位的短期疗效观察

目的介绍一种治疗先天性及习惯性髌骨脱位的新手术方式,并通过对短期疗效的观察,评估此手术方式的可行性。方法收集2018年8月至2019年1月复旦大学附属儿科医院小儿骨科通过改良Langenskiold和Grammont手术方法联合治疗的先天性或习惯性髌骨脱位患儿病史资料,对术后膝关节功能进行评价,评估上述手术方式的可行性。结果期间共治疗8例9膝,其中先天性髌骨脱位5例,习惯性髌骨脱位3例。8例中男2例,女6例;左侧6例,右侧1例,双侧1例;平均手术年龄5.9岁(1.4～10.7岁)。术后根据患儿髌骨有无再脱位、膝关节屈伸功能、走路有无疼痛评估手术疗效。术后24周随访所有患儿膝关节活动正常。8例至最后一次随访均未发生髌骨再脱位;其中7例膝关节功能评价为优(87.5%),1例因术前术后均无法行走,故无法评价术后膝关节功能等级。结论从短期结果看,改良Langenskiold和Grammont方法联合治疗先天性及习惯性髌骨脱位是可行的,但长期疗效有待进一步随访观察。     

先天性髋关节脱位闭合复位的治疗体会

目的总结1994年1月￣2004年12月采用闭合复位治疗的先天性髋关节脱位262例(316髋)的疗效,探讨其影响因素。方法于全麻下行内收肌松解,手法复位,蛙式位或人体位支架固定,复位后定期作X线检查。结果262例患儿平均随访5年3个月(2￣8.5年),优良率为93%。复位后因髋臼发育不良仍有半脱位的13髋经再次手术痊愈。26髋发生股骨头骨骺发育不良(即无菌性坏死),占8%,其中14个髋术前股骨头骺未出现。结论先天性髋关节脱位早期治疗是成功的关键,闭合复位后支架固定是减少股骨头坏死的重要因素。     

Pavlik支具在新生儿股骨干骨折的应用

目的 选择更为简便有效的新生儿股骨干骨折的治疗措施。方法 对12例12侧新生儿股骨干骨折应用Pavlik支具固定四周。结果 所有患儿均获治愈，对线良好，无并发症发生。结论 应用Pavlik支具治疗新生儿股骨干骨折方法简便，效果可靠，容易护理，没有不良后果或并发症。     

骨盆股骨短缩去旋转内翻截骨治疗先天性髋脱位

目的 探讨骨盆截骨结合股骨短缩去旋转内翻截骨治疗先天性髋关节脱位的疗效。方法 手术治疗先天性髋关节脱位15例16髋，全部均为女性，年龄平均4岁6个月(3岁～6岁6个月)，采用髋关节切开复位、骨盆截骨及股骨短缩去旋转内翻截骨术。结果 优13个髋，良3个髋，没有再脱位。X线片结果优12个髋，良4个髋，没有发生股骨头缺血性坏死。结论 骨盆截骨结合股骨短缩去旋转内翻截骨术治疗先天性髋关节脱位能够获得满意的结果。     

先天性髋关节脱位术后再脱位原因分析

目的 探讨先天性髋关节脱位术后再脱位的原因。 方法 分析18例先天性髋关节脱位术后再脱位的临床资料,其中髋臼造顶术7例,Salter骨盆截骨术6例,Pemberton关节囊周围截骨术4例,Staheli髋臼延伸术1例。 结果 再脱位的原因中,前倾角>60°未矫正6例,髋臼造顶位置过高5例,手术适应证选择相对不合理3例,植骨被吸收2例,假臼误认为真臼1例,术后过早下地1例。结论 病理改变了解不充分和手术适应证选择不当等因素是造成先天性髋关节脱位术后再脱位的主要原因。     

先天性髋脱位闭合复位551例报告

先天性髋脱位闭合复位５５１例，其中３９１例获随访２～１８年。通过对内收肌切断组和内收肌十髂腰肌切断组两种不同治疗方式的观察，结果发现内收肌十髂腰肌组治疗结果优良率为９０．５％，高于内收肌组；而股骨头坏死率为８．５％，低于内收肌组。强调闭合复位时选择手术方式的重要性，并对复位后髋臼的发育潜力及复位时年龄、股骨头坏死及其治疗进行了讨论。     

Congenital cutaneous candidiasis: Report of four cases and review of the literature

Congenital cutaneous candidiasis (CCC) is a rare disease acquired by an ascending route, liable to affect the offspring of pregnant women suffering from vulvovaginitis. The cutaneous lesions are present at birth or within the first hours of life. Some infants may present with respiratory distress or clinical signs of sepsis during the first 2 days of life. We report four new cases of CCC, three of which presented transient respiratory distress and clinical signs of sepsis with hepatosplenomegaly. The evolution was favourable in all three cases with topical and oral therapy. We emphasize the self-limited character of this disease, although preterm infants may be at risk of systemic spread. Only one infant presented paronychia as a late complication.     

Congenital cyst of the pancreas in a newborn

We report a case of congenital pancreatic cyst in a newborn detected antenatally by ultrasound scanning. The cyst was resected and pancreatico-enterostomy performed. The postoperative course was uneventful. Histology of the cyst showed a hamartoma. Correspondence to: M. S. Fleet     

Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and cystic dysplasia of the kidney

Congenital absence of portal vein is a rare malformation. To date, 16 cases have been reported – all in association with other anomalies, i.e. benign or malignant hepatic neoplasms in 6 cases and cardiac malformations in 12. This case report described a girl with congenital absence of portal vein, focal nodular hyperplasia of the liver and cystic kidney dysplasia. Angiography showed the splenic vein and superior mesenteric vein joining to form a common trunk that entered the inferior vena cava directly above the liver. A review of the other cases in the literature is provided and the clinical aspects of our patient are discussed. Received: 3 December 1996 / Accepted in revised form: 9 September 1997     

全国医院感染监控网儿科和产科新生儿室院内感染监测报告

目的：了解儿科和新生儿室医院感染发生的特点,为控制医院感染提供依据。方法：全国医院感染监控网135家医院专职医院感染控制人员按照统一的要求对综合性医院儿科住院病人和产科新生儿室的医院感染进行监测,按月报告监测结果。结果：2000年1～12月共监测儿科和产科新生儿室155 975人次,感染4 310人次,发病率2.8％;感染4 699例次,例次发病率3.0％。儿科新生儿组感染发病率最高为4.1％,较儿科非新生儿组(2.5％)和产科新生儿组(3.2％)高(P<0.01)。医院感染以呼吸道感染和皮肤软组织感染为主。结论：病理新生儿的医院感染发病率较高,应加强监测,合理的预防和治疗医院感染,尤其应加强呼吸道感染的控制,强调隔离及无菌操作,避免接触传播,以降低医院感染的发生率。     

Congenital Aneurysm of the Muscular Interventricular Septum in a Fraternal Case Diagnosed by Fetal Echocardiography

Two siblings with aneurysms of the muscular ventricular septum are presented. Case 1 (the older brother) underwent fetal echocardiography at 26 weeks of gestation. Initially, it was thought that he had a muscular ventricular septal defect; however, postnatal echocardiography revealed an aneurysm of the muscular portion of the ventricular septum. Retrospective review of the 26-weeks gestation fetal echocardiogram confirmed this diagnosis. Case 2 (a younger brother of the first case) was also diagnosed as having an aneurysm of the ventricular septum by fetal echocardiography at 28 weeks of gestation. Postnatal echocardiography confirmed this diagnosis.     

Congenital hypothyroidism and partial thyroid hormone unresponsiveness of the pituitary in a patient with congenital thyroxine binding albumin elevation

We describe a girl who presented at the age of 6 weeks with cardiogenic shock due to congenital hypothyroidism (serum thyroxine (T4) <12 nmol/l). Thyroxine replacement therapy was instituted. In spite of high total serum T4 levels, thyroid stimulating hormone (TSH) serum values remained elevated. The raised serum T4 levels were the result of congenital elevation of thyroid binding albumin (TBA). Toxic doses of both T4 and triiodothyronine (T3) normalized the elevated TSH levels indicating that the pituitary is responsive to thyroid hormone, albeit at a higher threshold. In patients with congenital TBA elevation and an altered T4 pituitary response requiring thyroid replacement therapy, the measurement of serum free T4 levels is the parameter of choice to monitor treatment.     

Transient Postnatal Heart Failure Caused by Noncompaction of the Right Ventricular Myocardium

Isolated noncompaction of the ventricular myocardium is a cardiomyopathy influencing almost exclusively the left ventricle in children and adults. We report a case of a neonate presenting with right ventricular failure caused by a noncompaction of the right ventricle. However, later course of the disease demonstrated biventricular involvement. Right ventricular noncompaction can be a cause of early postnatal right ventricular failure.