主肺动脉窗25例临床分析

目的 分析主肺动脉窗（APW）的发病、分型和伴发畸形情况,总结诊断及治疗经验。方法 回顾性分析复旦大学附属儿科医院2003至2013年诊治的25例APW患儿的临床症状、病理分型、合并畸形、诊断、治疗和预后资料。结果 25例APW患儿进入分析,男14例,女11例,年龄5 d至13岁（6月龄以下12例）,占同期住院先天性心脏病患儿的0.18%。Ⅰ型5例（20%）,Ⅱ型16例（60%）,Ⅲ型1例（4%）,Ⅴ型3例（12%）。单纯APW 10例,APW合并单纯卵圆孔未闭3例;余12例（48.0%）均合并其他需纠正的心血管畸形,其中合并单纯房间隔缺损（ASD）1例,单纯动脉导管未闭（PDA）2例,单纯室间隔缺损(VSD)3例,右室双出口和ASD 1例,主动脉瓣狭窄和右位主动脉弓1例,肺动脉瓣闭锁伴VSD、右位主动脉弓、永存左上腔静脉1例, 主动脉弓离断及PDA 3例。超声心动图诊断17例,误诊或漏诊8例（32%）中5例通过心导管检查及造影诊断,3例外科术中诊断。25例均有不同程度肺动脉高压,其中2例心导管检查评估为阻力性肺动脉高压,4例家长放弃诊治,其余19例施行一期根治术,术后死亡2例（10.5%）。17例术后随访3个月至9年3个月,平均（23±27）个月,其中15例临床无症状,复查超声心动图APW修补无残余,肺动脉压力逐渐下降至正常。结论 APW缺损主要发生在主动脉远端,常伴有间隔缺损、PDA和主动脉弓病变等;超声心动图为首选检查方法,但必要时需行心导管检查及造影。APW患儿易早期发生肺动脉高压,一经诊断尽早手术,可获得较好的中长期疗效。     

儿童右肺动脉异常起源于主动脉的诊断与外科治疗

目的 评价儿童右肺动脉异常起源于主动脉(aortic origin of the right pulmonary artery,AORPA)的治疗方法及效果,并探讨AORPA的诊断与鉴别诊断方法.方法 回顾性分析1993年5月至2007年7月11例儿童AORPA术前诊断及手术治疗的围术期疗效及中长期随访结果.分别采用右肺动脉经人工血管(2例)或同种主动脉(2例)与主肺动脉吻合术、体外循环下右肺动脉与主肺动脉直接吻合术(3例)、体外循环下(主动脉环及肺动脉环片)双片法右肺动脉与主肺动脉吻合术(2例)、非体外循环下右肺动脉经人工血管与主肺动脉吻合术(2例).结果 11例中10例术前确诊,1例术前漏诊而在术中发现.5例通过右室造影及主动脉造影或多排CT/MRI检查确诊,6例单纯超声心动图检查患儿中确诊5例,1例漏诊.11例手术患儿围术期死亡2例.术后随访2.2～13年,1例于术后4年死于右心功能衰竭.生存8例患儿术后超声心动图复查射血分数(EF)值43%～52%,心功能I级5例,Ⅱ级3例.结论 右室造影及主动脉造影或多排CT/MRI检查可减少漏诊率.手术是治疗AORPA的有效方法,但应尽早手术以进一步提高手术疗效.应用自体组织加宽加长右肺动脉可减少吻合口狭窄的可能性.     

一侧肺动脉起源于升主动脉的诊治特点探讨

目的 探讨一侧肺动脉起源于升主动脉(AOPA)的临床诊治特点.方法 对12例AOPA患儿的临床特点、超声心动图、心血管造影及于术治疗结果 进行总结分析.结果 12例中男7例,女5例;平均年龄23个月;均有不同程度的气促、喂养困难及反复呼吸道感染史.经心导管检查证实诊断,其中9例为右肺动脉异常起源于升主动脉,为近端型;3例左肺动脉异常起源于升主动脉,1例为近端型,2例为远端型.12例患儿均合并有不同程度的肺动脉高压.超声心动图检查的符合率为83%.9例经手术治疗,其中1例术后4个月死亡.结论 超声心动图对于AOPA的诊断具有重要价值,结合心导管造影检查可确诊,心导管造影对大龄患儿尤其需要;由于存在发展为器质性肺高压的风险,本病一经诊断应尽早行于术治疗.     

肺动脉吊带畸形三例临床分析

目的 总结3例肺动脉吊带畸形患儿的临床表现和诊治经验,减少其误诊率.方法 回顾分析3例肺动脉吊带畸形病例诊治的临床资料及实验室检查资料的特点.结果 (1)临床表现:3例患儿的初次发病年龄<3个月,主要临床表现均为反复咳嗽、喘息,均因呼吸困难加剧急诊入院,均有多次门诊及住院治疗史,确诊前均被诊断为重症肺炎、先天性喉喘鸣,例2还诊断为急性喉炎,例3合并先天性心脏病(房间隔缺损).(2)CT检查:3例患儿均表现为左肺动脉起自右肺动脉,绕行于气管后方进入肺门,气管都有明显狭窄.心脏超声及纤维支气管镜对本病的诊断有提示作用.(3)治疗:3例均行手术治疗,2例于术后7～10 d成功脱离呼吸机,控制肺部感染后出院,1例患儿因气管内肉芽组织形成,不能脱离呼吸机而放弃治疗.结论 肺动脉吊带畸形婴幼儿可表现为反复咳喘,因呼吸困难急诊入PICU.肺动脉吊带畸形的诊断主要依靠CT、超声心动图及纤维支气管镜检查.早期诊断、最佳时机手术和围手术期监护是成功治疗的关键.     

肺动脉吊带的诊断与治疗

目的总结肺动脉吊带的诊断和治疗。方法对7例患儿的临床资料、心血管造影、磁共振与单排螺旋CT、彩色多普勒超声心动图和手术资料进行分析。结果患儿早期即有典型的喘息和喘鸣等症状;5例右心室造影、7例心血管磁共振成像和4例单排螺旋CT检查显示,左肺动脉起源于右肺动脉及受压的气道;5例行胸骨切开及在体外循环下作肺动脉吊带矫治术,同时处理合并的气道及心脏畸形。结论对有反复发作喘鸣等呼吸道症状的患儿,要考虑有肺动脉吊带的可能;选择性心血管造影、磁共振成像和螺旋CT为明确肺动脉吊带诊断的最佳方法,可互为补充;对合并有先心病或有症状的肺动脉吊带患儿应早期手术治疗。     

单侧肺动脉起源于升主动脉的外科治疗

目的 探讨单侧肺动脉起源于升主动脉(AOPA)的外科治疗方法.方法 2006年5月-2008年8月共8例AOPA患儿接受外科治疗.男女各4例;年龄1～96个月;体质量4～24 kg.7例为右肺动脉起源于主动脉,同时并动脉导管未闭、肺动脉高压,其中4例并卵圆孔未闭.1例左肺动脉起源于主动脉,同时并法洛四联症型肺动脉闭锁.病例术前均经心血管增强CT确诊.其中7例右肺动脉起源于主动脉的病例,在体外循环心脏停跳下,游离并切断右肺动脉,将切断的右肺动脉直接吻合于主肺动脉.1例左肺动脉起源异常,由于并法洛四联症型肺动脉闭锁,肺动脉发育较差,因此游离左肺动脉后采用7 mm Goretex管道行中央分流.结果 本组患儿均成活,术后恢复顺利,手术效果良好.术后1个月超声复查右肺动脉与主肺动脉吻合口流速(1.71±0.24)m/s,压差(1.60±0.45)kPa,均未见明显狭窄.结论 AOPA肺动脉高压产生快,应早期诊断,早期手术治疗,近期手术效果良好.但仍需远期观察,以评估手术效果.     

儿童先天性体动脉-肺动脉瘘4例并文献复习

目的总结临床表现为大量咯血的先天性体动脉-肺动脉瘘患儿的临床特点及诊治经 验。方法回顾性收集首都医科大学附属北京儿童医院2007年3月至2008年2月诊断为先 天性体动脉-肺动脉瘘4例患儿的临床资料,总结其临床表现、胸部X线片、胸部64 排CT增强扫描三维容积再现(3D-VR)、数字减影血管造影（DSA）、治疗及随访情 况。结果男1例,女3例,最大发病年龄为11岁,最小发病年龄为2个月,主要表现为咯 血。1例有杵状指,3例未见特异性体征。胸部X线片检查：3例未见异常,1例示肺 间实质浸润。胸部64排CT增强扫描3D-VR检查：1例提示支气管动脉迂曲,但未见 异常交通;1例导管栓塞治疗（TCE）后示右侧支气管动脉扩张迂曲,并与肺动脉 相通可能性大,考虑支气管动脉-肺动脉瘘;1例提示支气管动脉-肺动脉瘘;1例 未见异常。3例DSA提示为支气管动脉-肺动脉瘘,病变部位均位于右下肺,并行 TCE。随访至2009年2～5月,1例复发,表现为痰中带血,胸部64排CT增强扫描未 见异常,余3例未复发。3例怀疑为遗传性出血性毛细血管扩张症(HHT),1例考虑 为HHT高度危险者。结论体动脉-肺动脉瘘可造成大量咯血。胸部X线片一般无特异性表现,胸部64排 CT增强扫描3D-VR可显示病变部位,明确诊断需行DSA。可采用TCE治疗。TCE远期 效果应进行长期随访予以明确。     

二次手术治疗肺动脉吊带矫治术后气管狭窄

目的探讨肺动脉吊带合并气管狭窄的治疗方法。方法分析2017年2月至2022年10月山东大学附属儿童医院收治的26例肺动脉吊带矫治术后因气管狭窄接受二次手术治疗的患儿资料。其中男14例, 女12例;中位年龄为31.3个月;中位体重为13.7 kg;两次手术间隔中位时间为12.5个月。1级狭窄3例, 2级狭窄15例, 3级狭窄8例。合并右位心3例, 合并左肺动脉狭窄或闭锁3例, 合并迷走左锁骨下动脉畸形及Kommerell憩室1例。患儿均采用Slide气管成形术加宽气道。术后定期复查电子支气管镜。非正态分布数据采用Wilcoxon符号秩检验(配对样本)。结果 26例患儿气管狭窄段长度中位数为4 cm, 范围为2.5～6.6 cm。本研究病例无手术死亡, 无住院期间死亡。术后呼吸机辅助中位通气时间为19.1 h。术后并发症包括吻合口漏合并胸骨哆开1例, 声带麻痹3例, 气管软化1例, 乳糜胸1例, 脑损伤2例。术后中位随访时间为7.0个月。活动耐力略差3例, 其余患儿无呼吸道症状。3例行左肺动脉加宽术的患儿术后定期复查心脏彩色多普勒超声检查提示左肺动脉血流通畅。3例声带麻痹患儿术后3个月症状...     

大型室间隔缺损合并肺动脉高压术后远期疗效的随访

先天性心脏病 (简称先心病 )左向右分流合并重度肺动脉高压术后可复性问题一直是临床关注的焦点。手术矫正心内畸形后 ,如肺动脉高压不能恢复 ,甚至加重则失去了外科治疗的意义。有关手术的疗效和手术适应证的选择 ,国内外均有些报道 ,但缺乏肺动脉高压血流动力学长期随访的资料。我们通过对 2 4例大型室间隔缺损合并重度肺动脉高压(全肺阻力增高 )患儿进行术前及术后长期血流动力学的随访 ,旨在了解手术对这些患儿肺动脉高压的远期疗效。对象本组患儿 2 4例 ,男 11例 ,女 13例 ;年龄 6个月～ 14岁 ,平均 (5 8± 4 7)岁。≤ 2岁者 11例 ,>2…     

先天性支气管动脉-肺动脉瘘导致的隐源性咯血三例诊治分析

目的 加强对先天性支气管动脉-肺动脉瘘导致的隐源性咯血的认识.方法 回顾性分析我院收住的3例先天性支气管动脉-肺动脉瘘导致的隐源性咯血患儿的临床、实验室及影像学资料.结果 3例患儿均为年长儿,以咯血为首发症状,除合并轻微咳嗽外无其他典型临床症状;3例患儿均行胸部CT、胸主动脉CT血管造影及支气管镜检查,例1和例2患儿胸部CT均提示肺内片状阴影,例3未见异常;例1患儿胸主动脉CT血管造影提示右侧支气管内异常结节影,例2、3患儿未见异常;3例患儿支气管镜检查结果均未能明确咯血原因;最后均通过主动脉及选择性支气管动脉造影确诊,并予支气管动脉栓塞治疗.结论 年长儿童的隐源性咯血要警惕先天性支气管动脉-肺动脉瘘,及时行数字减影血管造影检查明确诊断并予治疗.     

Prolapsed valve of the foramen ovale in newborns and infants with coarctation of the aorta

Summary Out of 60 newborns and infants with symptomatic coarctation of the aorta, 34 had a significant left-to-right shunt at atrial level. In 20 of these 34 children, the atrial shunt had disappeared after surgical correction of the coarctation. All patients with this reversible shunt, thought to be secondary to an incompetent valve of the foramen ovale, were younger than 35 days at first cardiac catheterization. Left atrial pressure and left atrial volume of these patients did not differ significantly from those in a control group without atrial shunt. The conditions necessary for the development of a valve-incompetent foramen ovale include not only an increased left ventricular work load, but also a thin and pliable valve of the foramen ovale. It is only in infants younger than one month of age that the valve is thin and pliable enough to prolapse through the foramen ovale and form a channel between the two atria, which results in a left-to-right atrial shunt.     

Malignant intracranial chordoma and sarcoma of the clivus in infancy

Neuroradiological and clinical findings suggesting chordoma of the clivus are described in three children. They presented with torticollis as the primary and predominant symptom. Progressive deterioration with development of slurred speech, signs of intracranial pressure and further cranial nerve involvement led to more extensive neuroradiological investigation. The clinical and neuroradiological findings in these three children were similar; the prognosis was unfavourable for two of the children. The histological diagnosis in two cases was malignant chordoma; in the third case it was undifferentiated sarcoma of the clivus.This paper was presented in modified form to the ESPR-Meeting at Lucerne, May 1977     

Developmental dysplasia of the hip. A population-based comparison of ultrasound and clinical findings

Clinical and ultrasound findings were compared in 3613 newborns examined for developmental dysplasia of the hip (DDH) within 48 hours of delivery. Clinical and sonographic hip stability was described as stable, borderline unstable, dislocatable or dislocated, and the morphology on ultrasound as normal, immature or dysplastic. Persistent clinical or sonographic dislocatability or dislocation. major dysplasia or minor dysplasia combined with an unstable femoral head were indications for early treatment. A total of 123 (3.4%) infants were subjected to early treatment. of which 55 (45%) fulfilled the criteria for treatment on both clinical and ultrasound examinations, 52 (42%) were treated on the basis of ultrasound findings alone, and 16 (13%) on the basis of clinical findings alone. Thirty percent of the infants with clinically dislocated or dislocatable hips were judged to have stable or just borderline unstable hips on the first clinical examination. Of 486 (13.5%) infants with sonographically immature or minor dysplastic but stable hips, 472 (97%) normalized spontaneously, while treatment was initiated in 14 (3%) of them at 1-3 months of age because of lack of sonographic improvement. Only one infant presented with late DDH during an observation period of 3 years. Accepting sonographic dysplasia as a criterion for early splinting may result in a treatment rate which is almost twice the rate based on clinical criteria, but late dislocation may be virtually eliminated.     

Hypercalcemia and carcinoma of the penis

Hypercalcemia is the most common metabolic disorder associated with malignancies. Squamous cell carcinoma of the penis is a tumor for which this abnormality has rarely been described. This report presents a case of hypercalcemia seen in a patient with advanced penile cancer. A chemotherapy regimen of intravenous cisplatin and fluorouracil caused regression of the primary tumor and normalization of the serum calcium. A literature review supported an association between squamous cell carcinoma of the penis and hypercalcemia. Med. Pediatr. Oncol. 29:576–577, 1997. © 1997 Wiley-Liss, Inc.     

Correlation between severity of mucopolysaccharidoses and combination of the residual enzyme activity and efficiency of glycosaminoglycan synthesis

Aim: To develop a method for prediction of severity and clinical course of mucopolysaccharidoses (MPS), a group of inherited metabolic diseases.
Methods: Various biochemical and clinical parameters (including estimation of the level of clinical severity, presence of specific mutations, residual enzyme activity, urinary glycosaminoglycan (GAG) excretion, storage of GAG in fibroblasts and efficiency of GAG synthesis) of patients suffering from MPS types II, IIIA and IIIB were determined. Correlations between genetic, biochemical and clinical parameters were tested.
Results: We found that efficiency of GAG synthesis may contribute to the level of severity of MPS. It appears that (i) combination of low or average efficiency of GAG synthesis and the presence of residual activity of the enzyme is responsible for an attenuated phenotype, (ii) a lack of detectable residual enzyme activity causes a severe phenotype, irrespective of the efficiency of GAG synthesis and (iii) high efficiency of GAG synthesis leads to a severe phenotype, even if residual enzyme activity is detected. This correlation was found to be valid in 15 out of 17 patients tested.
Conclusion: Analysis of efficiency of GAG synthesis and residual activity of the enzyme may be considered for prediction of severity of MPS patients' clinical phenotypes.     

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     

Torsion of the penis

Torsion of the penis, with or without a minor degree of hypospadias, is a congenital abnormality that is being diagnosed more frequently and referred to pediatric surgeons. In the 5-year period 1988–1992, 16 boys, 9 with glanular or juxtaglanular hypospadias and 7 with isolated torsion of the penis, were operated upon for penile torsion in our clinic using either a circular incision at the subcoronal sulcus or Johnston's procedure. The functional and cosmetic results in the two groups are compared and specific indications for the two techniques are discussed. The first technique is suggested as the procedure of choice because it involves minimal complications and gives the best cosmetic results.     

应用高分子绷带与Pavlik挽具治疗新生儿先天性膝关节脱位

目的介绍利用高分子绷带与Pavlik挽具治疗新生儿先天性膝关节脱位的方法。方法2004年6月～2005年6月收治6例(8膝)新生儿先天性膝关节脱位,其中4例(5髋)并有发育性髋关节脱位,均采用闭合复位、阶段性高分子绷带固定与Pavlik挽具治疗。结果6例(8膝)膝关节过伸消失、屈曲功能完全恢复,平均屈曲120°。结论本法对新生儿先天性膝关节脱位,尤其是合并有发育性髋关节脱位的患儿,疗效满意。     

婴儿肺炎呼吸系统顺应性及阻力的被动流速容量测定

目的：探讨婴儿肺炎时呼吸系统顺应性(CRS)及阻力(RRS)的变化。方法：79例婴儿肺炎分为1～6月和7～12月2个年龄组。其中重症肺炎27例(1～6个月13例,7～12个月14例),普通肺炎52例(1～6个月28例,7～12个月24例)及37例正常婴儿(1～6个月18例,7～12个月19例)。采用美国森迪公司2600型肺功能仪,利用被动流速容量技术(PFV)进行CRS及RRS的测定。结果：1～6个月正常对照组、普通肺炎组和重症肺炎组的CRS分别为(0.017±0.005),(0.004±0.001),(0.003±0.0008) L/kPa,RRS分别为(3.61±0.76),(9.09±2.32),(10.14±1.01) kPa·s/L。7～12个月正常对照组、普通肺炎组和重症肺炎组的CRS分别为(0.020±0.005),(0.011±0.002),(0.006±0.002) L/kPa,RRS分别为(1.11±0.23),(1.61±0.37),(2.05±0.55) kPa·s/L。3组间CRS及RRS差异有显著性意义(P<0.05)。结论：婴儿肺炎时CRS降低,RRS增高,且病情愈严重改变愈明显。