产前超声诊断先天性肺囊性腺瘤样畸形分析

目的探讨产前超声诊断先天性肺囊性腺瘤样畸形的临床应用价值。方法回顾性分析行产前超声诊断5例先天性肺囊性腺瘤样畸形患者的临床资料。结果本组5例中2例患者出生后证实,3例尸检病理证实。其中Ⅱ型3例,Ⅲ型2例。结论完善孕妇产前检查可有效筛查先天性肺囊性腺瘤样畸形等疾患,降低缺陷儿出生率,效果肯定。     

囊性听神经瘤的诊断与治疗

目的 总结囊性听神经瘤的临床特点及治疗效果。方法 22例患者术前均应用CT和MRI进行诊断,应用乙状窦后入路手术切除肿瘤。术中证实,术后病理确诊囊性听神经瘤。结果 22例肿瘤全切除18例,术后面神经功能分级：Ⅱ级4例、Ⅲ级7例、Ⅳ级3例、Ⅴ级2例、Ⅵ级2例;次全切除4例,面神经功能Ⅱ级。结论 由于囊性听神经瘤的临床特点及疗效差,应把它作为一种特殊亚型来处理。     

家族性腺瘤样息肉病的治疗(附一家族病例分析)

临床资料家族性腺瘤样息肉病 (ＦＡＰ)是常染色体显性遗传性疾病[1] 。近年发现 4 0 %～ 90 %病人伴有上消化道息肉[2 ] 。该病治疗比较困难。通过对我科近年诊治的一家族情况随访 ,结合文献讨论、分析如下。采取回顾性分析法 ,对一ＦＡＰ家族 3人进行随访研究。父亲 5 3岁 ,2 5年前因粘液血便于我院诊断为全结肠多发息肉病伴部分结肠、直肠癌变 ,行全大肠切除术、回肠永久性造口术 ,术后恢复良好 ,每日大便 10余次 ,但不能自控 ,且伴有性功能障碍 ,现健在。其有两个孪生儿子 ,现年 2 7岁 ,均于13年前因粘液血便诊断为ＦＡＰ ,长子于 13年前…     

肺先天性囊性腺瘤样畸形一例

肺先天性囊性腺瘤样畸形一例印洪林，马恒辉患者女，１６岁。１９８９年因发热，咳嗽，以上呼吸道感染行胸片检查，发现右肺内阴影，诊断“肺炎”。给抗炎治疗，体温恢复正常，但复查胸片肺内阴影未见消散。在多家医院仍按肺结核治疗５个月，再次复查胸片见肺内阴影大小形...     

枕颈结合部先天性畸形的诊断和治疗

枕颈结合部畸形是指由于先天发育因素造成的寰枢椎及其附属结构和与之相连的枕骨及其包含其中的神经组织等的解剖结构异常,有时伴发远部畸形。临床上并非少见。该部位解剖结构复杂,以往限于影像学检查手段及对胚胎发育研究不够等因素,导致对各种复合畸形认识水平的不足。随着影像诊断技术和医疗水平的不断提高,该类畸形正逐渐被人们关注和重视,兹将有关的诊断和治疗综述如下。1病因枕颈结合部先天性畸形的确切病因还不清楚。近年来,随着人们对脊椎发育分子遗传调控的理解和认识,对枕颈结合部和颈椎骨性发育畸形根源有新的解释[1、2]。另外,…     

先天性上颈椎畸形的诊断与治疗分析

[目的]分析先天性上颈椎畸形造成上颈椎不稳的病因,探讨其手术方法的选择.[方法]2003年1月～2007年6月收治65例先天性上颈椎畸形,男39例,女26例,术前常规进行X线片、CT和MRI检查,根据影像学分为发育不全畸形32例,分节不全畸形18例,结构畸形15例.37例可以复位并且脊髓前方无明显受压患者采用后路固定、植骨融合,其他28例上颈椎脱位不可复位或者脊髓前方存在压迫患者,首先采用前方齿状突切除、减压,然后经后路内固定、取自体松质骨植骨融合.[结果]所有患者均顺利完成手术,未出现脊髓血管损伤等严重并发症.65例患者中47例获得随访,随访12～24个月,平均随访(15±6)个月.1例患者后路钛棒断裂,再次手术更换断裂钛棒,其余患者后方植骨形成骨性融合.34例上颈椎局部疼痛、活动受限患者中28例获得随访,患者疼痛均明显减轻或者消失.31例四肢麻木、上运动神经元损伤患者中19例患者获得随访,采用Frankel脊髓评分,术前患者评分5例B,8例C,17例D;获得随访患者术后Frankel评分5例C,6例D,8例E,与术前相比神经功能改善1～2级.[结论]根据先天性上颈椎畸形的病因、环枢椎复位和脊髓受压情况,分别选择后路或者前后路联合手术治疗.     

先天性肺囊腺瘤样畸形手术麻醉一例

患儿,男,16个月,体重9.5kg,因"活动后气喘4个月"入院。胸部X线片及CT提示左肺体积增大伴多发囊状影,左下肺膨胀不全。初步诊断为先天性囊腺瘤样畸形。超声心动图显示右位心(心脏位于右侧胸腔),微量心包积液,左心射血分数(LVEF)77%。拟行肺囊腺瘤切除及左全肺切除术。患儿入室生命体征平稳,BP100/60mmHg,HR162次/分,SpO298%。面罩吸氧,吸入8%七氟醚。患儿入睡后,建立外周静脉通道,给予盐酸戊乙奎醚0.01mg/kg。氯     

先天性多指畸形的诊治

先天性多指畸形的诊治杨钧路来金刘志刚多指畸形在手的先天性畸形中最为多见。津下报告１１５９例先天性畸形中，多指畸形占２８６例，其中拇指占２５９例。由于此畸形的症状典型，治疗容易，故临床上系统地观察和报道较少。近１０年来，本院及笔者在日本名古屋大学研修时...     

Successful treatment of an adult patient with pulmonary abscess secondary to congenital cystic adenomatoid malformation

We report a case of a 29 year old male who at age 25, developed a pulmonary abscess in the left lower lobe. A left lower lobectomy was performed following two recurrences on antibiotic treatment. The result of histopathological examination confirmed congenital cystic adenomatoid malformation (CCAM), a congenital disease characterized by multiple cysts resulting from adenomatous hyperplasia of the bronchial epithelia. In many cases, respiratory distress occurs during the neonatal period, and in about 80–85% of patients, CCAM is diagnosed before the age of two years due to respiratory infection. CCAM which was diagnosed in adulthood is very rare.     

Antenatal ultrasound diagnosis in cystic adenomatoid malformation of the lung--case report

Antenatal ultrasound diagnosis of cystic adenomatoid malformation was made in a fetus of 27 weeks gestation. The child was born at term and successfully operated upon 30 hours later. Early recognition by ultrasound allowed for adequate planning and preparation of management between obstetricians, neonatologists , and pediatric surgeons. The present case appears to be the fourth one reported with antenatal ultrasound diagnosis and the first one with successful operation, long-term survival and cure.     

Pulmonary arteriovenous malformation mimicking congenital cystic adenomatoid malformation in a newborn

Congenital pulmonary arteriovenous malformations (AVMs) are rare lesions, usually asymptomatic. We report on the case of a baby who was thought to have a congenital cystic adenomatoid malformation of the left upper lobe based on prenatal and postnatal imaging. Final pathology revealed a congenital pulmonary AVM. Neither the child nor her family have any evidence of hereditary hemorrhagic telangiectasia. To our knowledge, this is the first reported case of a pulmonary AVM mimicking a congenital cystic adenomatoid malformation.     

Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history

We studied the natural history and pathophysiology of congenital cystic adenomatoid malformation (CCAM) detected prenatally by ultrasound in twelve fetuses. Two types of fetal CCAM can be distinguished by gross anatomy, ultrasound findings, and prognosis. Microcystic lesions are usually associated with fetal hydrops and have a poor prognosis (five cases with one survivor). Antenatal diagnosis, maternal transport, and immediate thoracotomy after birth allowed the first reported survival of a newborn with a large microcystic CCAM. Macrocystic lesions are not usually associated with hydrops and have a favorable prognosis (five of seven survived). We conclude that fetuses with hydrops are at high risk for fetal or neonatal demise without intervention. Fetuses with CCAM but without hydrops have a good chance for survival with maternal transport, planned delivery, and immediate neonatal resuscitation and surgery.     

Thoracoscopic versus open resection for congenital cystic adenomatoid malformations of the lung

Purpose

This study evaluated the potential advantages of thoracoscopy compared to thoracotomy for resection of congenital cystic adenomatoid malformations (CCAM).

Methods

We conducted a retrospective chart review of consecutive cases of CCAM resection at University of California San Francisco Children's Hospital from January 1996 to December 2006.

Results

Thirty-six cases of postnatal CCAM resections were done over the past 10 years; 12 patients had thoracoscopic resections, whereas 24 patients had open resections. Patients in the thoracoscopic group had significantly longer operative time (mean difference of 61.3 minutes; 95% confidence interval [CI], 30.5-92.1) but shorter postoperative hospital stay (mean difference of 5.7 days; 95% CI, 0.9-10.4) and duration of tube thoracostomy (mean difference of 2.6 days; 95% CI, 0.7-4.5) and lower odds of postoperative complications (odds ratio of 9.0 × 10⁻⁴; 95% CI, 8.0 × 10⁻⁶-0.1). In the subgroup analysis of only asymptomatic patients, the thoracoscopy group still had a significantly shorter hospital stay (mean difference of 2.8 days; 95% CI, 0.7-4.8). There was also a pattern for reduced complications in the thoracoscopy group (OR, 0.13; 95% CI, 0.02-1.0; P = .05). The average hospital costs were similar in both groups. With a conversion rate of 33% (6/18), patients with a history of preoperative respiratory symptoms had a higher incidence of conversion than those who were asymptomatic (66.7% vs 0%, P = .005). These four patients had a history of pneumonia.

Conclusion

Minimally invasive resection of CCAM results in longer operative time but shorter hospital stay, potentially reduced complications, and no additional hospital costs. Thoracoscopic lobectomy in patients with a history of pneumonia is challenging and a risk factor for conversion to thoracotomy.     

Syndromic presentation of a pleuropulmonary blastoma associated with congenital cystic adenomatoid malformation. A case report

Pleuropulmonary blastoma (PPB) is a rare malignant mesenchymal pediatric tumor with a well-recognized association with congenital cystic adenomatoid malformation (CCAM). Recently, it has been described in a patient with CCAM, multiple jejunal polyps, and cystic nephroma. We describe a similar case of a unique presentation of PPB, arising in association with CCAM and with a history of intussception caused by multiple small bowel polyps.     

Intralobar sequestration associated with cystic adenomatoid malformation: diagnostic and thoracoscopic pitfalls

Background  Intralobar sequestration (ILS) associated with congenital cystic adenomatoid malformation (CCAM) is a well-known entity. This hybrid form has many considerations for an appropriate management. This report aims to analyze pitfalls in the diagnosis, treatment, and thoracoscopic procedure pertaining to this association. Methods  Between May 2004 and September 2007, 23 fetal lung lesions were diagnosed prenatally and managed at the authors’ institution. Seven of these lesions were the hybrid form of ILS-CCAM. The records for these lesions were reviewed retrospectively. Results  In all seven fetuses, prenatal ultrasound diagnosed congenital lung abnormalities. In three cases, fetal magnetic resonance imaging (MRI) demonstrated a macrocystic lesion supplied by an aberrant vessel. In all cases, the intrauterine evolution remained uncomplicated. All the newborns were asymptomatic at birth. Chest computed tomography (CT) performed at the age of 1 month showed the aberrant vessel in five cases. A regression of the lesion was noted in one patient and that of the aberrant vessel in another patient. Elective thoracoscopic lobectomy of the affected lobe was performed for six patients. The average age at the time of intervention was 5 months. At this writing, one patient is under observation due to resolution of the lesion. The operating time ranged from 80 to 160 min. One conversion to thoracotomy was required. All surgically treated patients had an uneventful hospital course. Histopathologic examination confirmed the association of intralobar sequestration with Stocker type 2 CCAM in five cases and with CCAM type 1 in one case. Conclusions  Prenatal diagnosis of the ILS-CCAM association was possible, as was resolution of one or both components of this lesion. Preoperative identification of the aberrant vessel is important for prevention of operative morbidity. This should be controlled before the lobectomy is begun.     

Video-assisted thoracoscopic resection of type I cystic adenomatoid malformation in a 3-month-old girl

A 3-month-old Chinese girl with type I cystic adenomatoid malformation involving both lobes of her left lung was successfully operated upon using the video-assisted thoracoscopic surgical (VATS) approach. The conventional endoscopic stapling device was used for resection without complications.     

Characteristics of congenital cystic adenomatoid malformations associated with nonimmune hydrops and outcome

Background/Purpose

In fetuses with congenital cystic adenomatoid malformations of the lung (CCAMs), hydrops fetalis and large masses are associated with poor outcomes. This study attempts to (1) determine sonographic features (in addition to large size) that correlate with hydrops and (2) characterize the features that correlate with outcome among hydropic fetuses.

Method

Charts and sonograms of fetuses with large, unilateral CCAMs were retrospectively reviewed. Mass features evaluated included laterality, macrocystic/microcystic, cystic/solid predominance, degree of mediastinal shift, retrocardiac component, diaphragm eversion, polyhydramnios, and mass-thorax ratio (MTR). Features of hydrops included degree of ascites, scalp and integumentary edema, pleural/pericardial effusion, and placentomegaly.

Results

Thirty-six fetuses with large CCAMs were studied: 27 with and 9 without hydrops. Three sonographic features were significantly associated with hydrops: MTR of at least 0.56, cystic predominance of mass, and eversion of hemidiaphragm. Of 27 fetuses with hydrops, 10 (37%) demonstrated all 3 features compared with none in those without hydrops (P = .04). All 9 nonhydropic fetuses were expectantly managed, and 100% survived. In the hydropic group, none of the expectantly managed fetuses survived, and 10 (43%) of the 21 fetuses who underwent fetal intervention survived.

Conclusion

Three features of large CCAMs were significantly associated with hydrops: MTR, cystic predominance, and diaphragm eversion. Identification of these features will allow clinicians to accurately predict which fetuses may warrant closer follow-up and possible treatment.