小儿先天性肺腺瘤样畸形的诊断及外科治疗

目的 总结小儿先天性肺腺瘤样畸形(CPAM)诊治经验.方法 回顾2006年11月至2009年11月手术的13例CPAM临床资料,男6例,女7例,手术年龄50d至13岁.多数患儿有临床症状,经胸部X线和CT检查确诊.3例小于2个月者急诊手术,其余患儿择期手术.术式有单肺叶切除10例,肺叶+不规则肺段切除2例,囊肿剥离术1例.病理示Ⅰ型8例,Ⅱ型和Ⅲ型各2例,Ⅳ型1例.结果 无手术死亡.2例术后早期出现支气管胸膜瘘,1例经保守治疗,1例在胸腔镜下瘘口缝合后治愈.术后平均住院9.7 d,平均随访12.3个月,2例肺叶+不规则肺段切除者活动量轻度受限,其余患儿恢复良好.结论 胎儿超声是CPAM产前诊断的主要手段,胸部CT检查对产后诊断有高度特异性.     

小儿先天性肺囊性腺瘤样畸形四例

先天性肺囊性腺瘤样畸形 (ｃｏｎｇｅｎｉ ｔａｌｃｙｓｔｉｃａｄｅｎｏｍａｔｏｉｄｍａｌｆｏｒｍａｔｉｏｎｏｆｔｈｅｌｕｎｇ ,ＣＣＡＭ)是一种少见的小儿的先天性支气管 -肺发育畸形。由Ｃｈｉｎ等首次报道 (ＡｒｃｈＰａｔｈｏｌ,1949)。 1996～2 0 0 0年我院共收治 4例 ,均经临床病理证实 ,现报道如下。临床资料本组 4例 ,男 2例 ,年龄分别为 5、8个月 ;女 2例 ,年龄分别为 4、8个月。均以长期咳嗽、气喘症状就诊。 2例以肺部感染入呼吸内科 ,经抗感染治疗肺部病变不改善而转入外科。另 2例因胸部透视示支气管不全阻塞误诊为支气管…     

先天性肺囊腺瘤样畸形40例诊疗分析

目的 分析先天性肺囊腺瘤样畸形胎儿-新生儿的的围产危险因素,探讨其手术时机以及风险管理方法.方法 回顾性分析自2008年10月至2013年1月,来本院就诊40例先天性肺囊腺瘤样畸形病例资料.结合产前诊断以及出生后的胸片、CT检查,分析围产风险因素和手术时机. 结果临床诊断先天性肺囊腺瘤样畸形40例中,顺产17例,剖腹产23例;早产儿3例.出生后有呼吸道症状11例,占27.5％,新生儿期出现呼吸道症状14例,占35.0％.40例中,手术治疗32例;1例放弃治疗,后来死亡;7例家属选择等待.32例手术患儿均治愈出院.平均手术年龄5.6个月.结论 先天性肺囊腺瘤样畸形在新生儿期存在呼吸道风险,应选择在有新生儿外科手术条件的医院生产,出生后需注意预防呼吸道风险,为手术创造条件.     

小儿先天性肺囊肿的外科治疗

目的 总结小儿先天性肺囊肿的外科治疗经验。方法 本组患儿均在全麻下行外科手术治疗,其中占据一侧胸腔的巨大张力性囊肿６例,２例术前放管引流减压。单纯囊肿摘除９例,肺段及肺楔形切除各２例,肺叶切除３６例,全肺切除１例。结果 本组无手术死亡,术后并发支气管胸膜瘘１例。１１例肺叶切除患儿随访１～１０年,肺功能各项指标均正常。结论先天性肿囊肿应早期诊断、早期治疗。对巨大张力性肺囊尖急诊手术,必要时术前放管囊     

小儿先天性肺囊性腺瘤样畸形23例诊治分析

目的 总结小儿先天性肺囊性腺瘤样畸形(CCAM)的临床特点及诊治经验.方法 回顾性分析2001年3月至2007年12月在北京儿童医院经手术和病理检查确诊为CCAM的23例患儿的临床资料.所有患儿术前均摄X线胸片并行胸部CT扫描检查.结果 23例患儿中男15例,女8例.进行手术治疗时的平均年龄4.5岁,其中包括新生儿2例.术前诊断主要依靠病史和影像学检查.术前误诊4例,术前影像学检查确诊率为82.6%.依照Stocker组织病理学分型,23例患儿中Ⅰ型15例,Ⅱ型8例,没有恶变.23例均经手术治疗,证实1例为双侧痛变,余均为单侧病变和累及单叶.全部病例痊愈出院,随访结果显示23例均恢复良好.结论 小儿CCAM临床表现无特异性.影像学检查是发现该病的有效手段,确诊依据组织学检查,组织病理学分型可依据胸部CT结果得出大致判断.手术治疗是CCAM主要治疗手段,肺叶切除是标准术式并可取得良好的效果.     

儿童先天性肺囊性腺瘤样畸形四例临床特点分析

目的探讨儿童先天性肺囊性腺瘤样畸形(congenital cystic adenomatoid malformation of lung,CCAM)的临床、影像及组织病理特点,提高临床对该病的认识。方法收集新乡医学院第一附属医院经病理证实为CCAM的4例患儿病例资料,对其临床表现、影像学及病理特点、治疗方法进行分析。结果3例患儿有反复呼吸道感染,1例自发性气胸;2例胸部X线见到囊性改变,2例胸部CT提示存在CCAM,1例提示肺脓肿,1例提示不除外肺隔离症;4例均手术切除病变肺叶,术后病理证实为CCAM,均预后良好。结论反复呼吸道感染者需注意该病可能,需结合临床表现、CT检查、病理结果共同诊断CCAM,手术切除是有效治疗手段。     

胎儿-新生儿先天性肺囊腺瘤样畸形手术初步结果分析

目的总结胎儿及新生儿先天性肺囊腺瘤样畸形的手术情况,初步比较两种手术的治疗结果。方法回顾性分析我们于2009年1月至2014年12月收治的13例先天性肺囊腺瘤样畸形患儿临床资料,均采取产时子宫外手术或新生儿期手术,术后均获得病理检查证实。按照手术方式将患儿分为两组,即胎儿手术组(n=6)、新生儿手术组(n=7),比较两组治疗结果。结果13例均存活,胎儿手术组与新生儿手术组手术时胎龄分别为(38.00±1.44)周、(39.27±1.42)周,P=0.138;出生体重分别为(3 060.00±342.05)g、(3 354.29±312.78)g,P=0.133;手术出血量分别为(8.67±6.38)mL、(6.29±3.04)mL,P=0.396;术后呼吸机支持天数分别为(3.67±1.75)d、(2.57±1.13)d,P=0.201;术后住院天数分别为(18.33±9.20)d、(11.43±2.43)d,P=0.081;差异均无统计学意义。肿物大小比较,P=0.000[(124.82±40.70)cm~3 vs(23.76±5.84)cm~3];手术时间比较P=0.000,[(43.16±6.6)min vs(92.86±24.58)min],差异有统计学意义。结论产时子宫外处理技术可以作为治疗高风险的先天性肺囊腺瘤样畸形的方法之一,但手术风险大,操作复杂,需多学科合作,把握手术适应证,谨慎选择。     

70例婴幼儿先天性肺囊腺瘤样畸形的围术期分析

目的：总结婴幼儿先天性肺囊腺瘤样畸形的诊断和手术治疗方法。方法回顾性分析自2008年8月至2013年7月于本院就诊的70例先天性肺囊腺瘤样畸形患儿临床资料,比较出生后X线检查、CT扫描检查及手术治疗结果,并进行随访。结果70例患儿中,男39例,女31例。入院时最小年龄为30 min,最大年龄为13岁。手术时期：新生儿期40例,1个月至1岁26例,1～13岁4例,平均手术年龄（4.3±0.6）个月。分型：Ⅰ型18例,Ⅱ型35例,Ⅲ型6例,混合型6例,存在大叶性肺气肿5例。4例合并其他畸形,其中2例合并纵隔囊肿;1例混合型合并骶尾部畸胎瘤;1例左侧Ⅲ型合并左侧膈疝。病变位置：1例为双侧,其余均为单侧。右侧21例,左侧18例。术前X线检查均无特异性。70例均采用开胸手术治疗,其中肺叶切除术59例,肺段切除术6例,肺叶＋肺段切除术3例;2例行右肺两肺叶切除术。术后并发症包括气胸、咳嗽、伤口感染、胸腔粘连、膈肌膨升,经治疗后痊愈。随访2个月至5年,均存活,无一例死亡,且无术后复发现象。结论出生后增强CT检查对于先天性肺囊腺瘤样畸形的确诊及手术方案的制定极其重要,建议确诊后早期手术切除。     

先天性颈椎畸形的诊断与治疗

先天性颈椎畸形的诊断与治疗李祁伟综述吉士俊审校先天性颈椎畸形发病率低，病种多，其中常见的有：颅底凹陷、Ｋｌｉｐｐｅｌ－Ｆｅｉｌ综合征、先天性齿突畸形、枕颈融合、环椎畸形、环椎横韧带松弛等［１］。因其命名各异，诊断标准不一，治疗方案尚未统一。本文综合国...     

Antenatal diagnosis and management of congenital cystic adenomatoid malformation

One of the most enigmatic pulmonary lesions encountered in the prenatal period is the congenital cystic adenomatoid malformation (CCAM). This review presence current thinking on pathogenesis, prenatal assessment, fetal intervention, and management for this pulmonary malformation. Careful delivery planning by utilizing a multidisciplinary approach will optimize neonatal outcomes.     

Long-term outcome of congenital cystic adenomatoid malformation

To determine the long-term outcome of congenital cystic adenomatoid malformation (CCAM), the records of all patients with CCAM treated in three Hungarian paediatric surgical centers between 1977 and 1996 were reviewed. Patients were followed for up to 20 years following diagnosis and treatment. In 20 patients CCAM was diagnosed postnatally and in 3 prenatally. Biodata including the operative procedures are presented. Follow-up findings between 18 months and 20 years after diagnosis showed better height and weight growth in patients operated upon in later childhood compared with those operated upon in infancy. The older the patient at the time of diagnosis, the better was the long-term outcome. Accepted: 5 January 1999     

Managing prenatally diagnosed asymptomatic congenital cystic adenomatoid malformation

Aim  To review our cases of congenital cystic adenomatoid malformation (CCAM) with special emphasis on the management of prenatally diagnosed asymptomatic CCAM (PDA-CCAM). Methods  A total of 42 consecutive CCAM patients treated between 1990 and 2008 at our institution and affiliated hospitals were divided into four groups, according to whether prenatal diagnosis was made [PD (+) or (−)], whether patients were symptomatic [S (+) or (−)], whether there was any increase in size observed radiologically [R (+) or (−)], and whether surgical resection was performed [X (+) or (−)], to give a symptomatic early surgery group diagnosed prenatally (group A; n = 15): PD (+), S (+), R (+), X (+); an asymptomatic early surgery group diagnosed prenatally (group B; n = 8): PD (+), S (−), R (−), X (+); a conservative observation group diagnosed prenatally (group C; n = 6): PD (+), S (−), R (−), X (−); and a symptomatic surgery group diagnosed postnatally (group D; n = 13): PD (−), S (+), R (unknown), X (+). Patient demographics, effects of surgical stress, histopathology, and outcome were compared between the four groups. Results  Groups A and B had surgery as neonates and mean duration of post-operative follow-up has been 15.7 months for group A and 63.3 months for group B. Group C has been under observation for a mean of 21.6 months (range: 10–40 months) with no incidence of infection. In group D, four had surgery as neonates after developing respiratory distress, and nine had surgery later (mean age: 4.1 years) after developing pneumonia (late onset group). Mean duration of follow-up for group D has been 48.0 months. In the late-onset group, pneumonia was successfully treated medically, and all nine had elective surgery with no intra- or post-operative complications. However, compared with group B (asymptomatic early surgery group diagnosed prenatally) the late-onset group had significantly longer duration of surgery (P < 0.05), significantly greater intraoperative blood loss (P < 0.01), and significantly higher peak post-operative C-reactive protein (P < 0.01), although there were no statistical differences for increase in white blood cell count ratios or length of hospitalization. Malignancy has not developed to date in any subject. Conclusion  Our data suggest that patients with PDA-CCAM may be observed safely until they become symptomatic or changes in size are observed radiologically.     

Long-term outcome of asymptomatic patients with congenital cystic adenomatoid malformation

Purpose  Congenital cystic adenomatoid malformation (CCAM) represents a rare congenital anomaly of the lung. It remains controversial whether patients with asymptomatic lesions warrant early surgical intervention. Our aim was to review the outcome of asymptomatic CCAM patients at a paediatric tertiary centre. Methods  The medical case notes of all children with CCAM presenting to our institution between 1986 and 2007 were reviewed. Data on pre- and post-natal investigations, clinical presentation, lesion site, type of surgical procedure, timing, and outcomes of surgery were reviewed. Results  A total of 35 patients were diagnosed with CCAM during the 21-year study period (1986–2007). Sixty percent (n = 21) were asymptomatic at birth including eight patients with prenatal ultrasound scan confirming CCAM. In this group, 18 patients (86%) subsequently developed symptoms (median age 2 years, range 1 month–13 years) and required surgery. Symptoms included pneumonia with or without infected CCAM (43%), respiratory distress (14%) and spontaneous pneumothorax (14%). Eight patients underwent multiple hospital presentations with complications related to CCAM. Of the 21 initially asymptomatic patients, 17 (81%) underwent surgical resection. Only one of these patients was completely asymptomatic prior to surgery. There were eight post-operative complications and no mortality. One patient underwent a second thoracotomy for residual CCAM. The median length of hospital stay was 9 days (range 3–32 days). Conclusion  This study suggests patients who present with asymptomatic CCAM will subsequently become symptomatic. Early surgical referral and intervention may be beneficial to avoid the development of complications.     

Intralobar pulmonary sequestration with congenital cystic adematous malformation and rhabdomyomatous dysplasia

Pulmonary sequestration and congenital cystic adenomatous malformations (CCAM) are well known but still uncommon anomalies of the lung. Extralobar sequestrations are well described in association with CCAM, but fewer intralobar lesions are found with this association. The existence of striated muscle within CCAM is described, and we have evidence of dysplastic changes within the various cellular components occurring, with rhabdomyomatous dysplasia being one of these. A literature review shows no previous evidence of a reported intralobar sequestration associated with CCAM and rhabdomyomatous dysplastic changes.     

Rhabdomyosarcoma arising within congenital cystic adenomatoid malformation

Rhabdomyosarcoma arising within a congenital cystic adenomatoid malformation (CCAM) is an unusual entity. The patient underwent a lobectomy of his right lower lobe of lung due to a CCAM at the age of two. One year later, he developed a solid embryonal rhabdomyosarcoma at the same location. He received 1-year period chemotherapy and when the tumor reduced to a resectable size, surgical excision was done. The tumor cells appeared more differentiated after chemotherapy. The patient remains disease free to date, 16 months after surgery.     

小儿先天性肺隔离症的诊断与治疗

目的 探讨小儿先天性肺隔离症的诊断与治疗.方法 回顾性分析2001年3月至2011年3月收治的小儿先天性肺隔离症20例的临床资料.其中,男11例,女9例,年龄4 d～9岁(平均5.3岁),体重4～28 kg(平均11.3 kg).术前20例均行X线平片检查,15例加行普通CT平扫检查.误诊为肺囊肿9例,肺内肿瘤2例,漏诊4例在行其他手术时发现本病;另5例加行64层高速螺旋CT三维血管成像检查,发现有异常体动脉分支血管入隔离肺组织4例,高度疑诊本病1例.结果 全部20例均接受手术治疗,术后病理检查均证实为本病.术中发现病变肺组织(隔离肺)均有起自体动脉分支血管供血.本组均治愈,术后随访15例,随访2～7年,生长发育良好无复发,失访5例.结论 本病较少见,临床表现缺乏特异性,易被误诊和漏诊.高速螺旋CT 三维血管成像检查是术前确诊本病的重要手段.术中准确处理来自体动脉的分支血管是手术成功的关键.     

Congenital cystic adenomatoid malformation associated with ipsilateral eventration of the diaphragm

A two-month-old girl was operated for a left-sided eventration of the diaphragm. At surgery, a mass representing an extralobar pulmonary sequenstration (EPS) was found inferior to the left lobe of the lung and was excised. Histopathological examination of the resected specimen showed presence of a type II congenital cystic adenomatoid malformation (CCAM) within it. A review of the relevant literature is presented.     

Coincidence of congenital cystic adenomatoid malformation and extralobar sequestration of the lung in a newborn

We present a rare case of coincidence of an extralobar sequestration with a congenital cystic adenomatoid malformation of the lung in a newborn. The symptoms, diagnostic features, and therapy are described and the etiology and classification are briefly discussed.