小儿腹腔内肺隔离症4例并文献复习

Pediatric intra-abdominal extralobar pulmonary sequestration:a case series and literature review

目的探讨儿童腹腔内肺隔离症(intra-abdominal extralobar pulmonary sequestration,IEPS)的临床特征、病理类型、诊疗方法及预后情况,以提高对该病的认识及诊治水平。方法回顾性分析2007年6月至2018年6月首都医科大学附属北京儿童医院收治的4例腹腔内肺隔离症患儿临床资料;检索万方、维普、CNKI、Pubmed、Medline数据库截至2018年6月关于小儿腹腔内肺隔离症的相关文献,并进行分析。结果本研究4例腹腔内肺隔离症患儿,合并先天性肺气道畸形Ⅱ型/Ⅲ型(congenital pulmonary airway malformation type 2/ type 3,CPAM Ⅱ/Ⅲ型)各2例,均行手术治疗,分别随访3个月、6个月、3年半、4年,预后良好。通过文献检索,检索到31篇儿童IEPS相关文献,联合本中心4例患儿分析结果如下:共47例患儿,44例产前发现病变时母亲孕龄为25周(IQR:12~37),46例产前经多普勒B超诊断腹腔肿物,10例怀疑神经母细胞瘤(neuroblastoma,NB),7例怀疑IEPS。5例终止妊娠。产后42例患儿中7例(7/42,16.7%)伴发先天畸形。术前主要经B超(30/38,78.9%)、增强CT(27/38, 71.1%)明确诊断,其中11例误诊为NB,9例诊断为IEPS。5例行保守治疗,3例保守治疗无效后行手术治疗。共40例患儿行手术治疗,25例(25/40,62.5%)出生后6个月内行手术治疗,主要采用开腹手术(25/40,62.5%),3例行腹腔镜中转开腹手术。肿物主要位于左腹(32/40,80.0%)。手术切除肿物平均长径3.0(IQR:2.8~3.9)cm,供血来源主要为腹主动脉及其分支(16/21,76.2%)。术后病理检查均证实为肺隔离症,22例(22/40,55.0%)伴CPAM,其中12例伴CPAM Ⅱ型,2例伴CPAM Ⅲ型。所有患儿预后良好。结论产前超声对于小儿腹腔内肿物具有良好的诊断价值,但明确腹腔内肺隔离症仍需产后随访及综合评估。手术治疗是早期诊断IEPS的唯一方式,应根据病变选择合适的手术方案,完整切除肿物者预后良好。

Objective To explore the clinical presentations,pathology,treatments and outcomes of intra-abdominal extralobar pulmonary sequestration (IEPS). Methods Four cases of IEPS were selected from our center while more clinical cases retrieved from the databases of Wanfang,Weipu,CNKl,PubMed and Medline prior to June 2018. Results All 4 patients diagnosed as IEPS,including 2 patients each of congenital pulmonary airway malformation type 2/ type 3 (CPAM Ⅱ/Ⅲ),were operated.All of them showed normal growth and development during a follow-up period of 3 months,6 months,3.5 years and 4 years,respectively.A total of 47 IEPS cases have been reported.There was a total of 5 aborted fetuses,and an average gestational age of lesion detection at 25(12-37) weeks.Among 46 cases diagnosed as abdominal mass by prenatal ultrasound,there were suspected neuroblastoma (NB, n =10),suspected IEPS ( n =7) and associated anomalies ( n =7).Preoperative diagnosis was made by type B ultrasound (30/38,78.9%) and enhanced CT (27/38,71.1%).The misdiagnosis was NB ( n =11) and IEPS ( n =9).A total of 40 cases were operated by open surgery (25/40,62.5%) and laparoscopic laparotomy ( n =3).And 62.5% of them underwent surgery within 6 months.The mass was located predominantly in left abdomen (32/40,80.0%).The mean length and diameter of tumor after surgical resection were 3.0 (IQR:2.8 ~ 3.9) cm and major blood supply came from abdominal aorta and its branches (16/21,76.2%).Postoperative pathology confirmed IEPS with CPAM ( n =22),CPAM Ⅱ( n =12) and CPAM Ⅲ( n =2).All children had an excellent prognosis. Conclusion Intra-abdominal extralobar pulmonary sequestration should be evaluated comprehensively postpartum although prenatal ultrasound has an excellent diagnostic accuracy of intraperitoneal masses in children.Surgery is the only method of making an early definite diagnosis.Surgical approach should be selected properly according to the lesions.Postsurgical outcomes are favorable.