新生儿支气管肺发育不良诊治进展

支气管肺发育不良（bronchopulmonarydy splasia,BPD）是一种慢性肺部疾病,常见于长期氧疗和机械通气的早产儿。BPD由Northway于1967年首次报道,近年来其发生率有逐年增加的趋势,并成为NICU最为棘手的问题之一以及婴儿期慢性肺疾病（CLD）的主要病因。产前糖皮质激素和出生后外源性表面活性物质的应用,以及保护性通气策略实施,使BPD表现形式发生了很大变化,更为常见的是一种轻型BPD（又称为“neWBPD”）,这种“newBPD”与40年前的“oldBPD”从病因、病理改变及临床表现等方面均有很大区别。文章对BPD最新定义、诊断标准及治疗进展作一介绍。     

Clinical outcomes of congenital lung abnormalities

Individually, congenital abnormalities of the lung are rare but collectively they form an important group of conditions. The range of malformations is broad and the clinical impact is very variable, depending on the degree of malformation. This article groups these conditions under their traditional headings and considers their management. However, in conditions that are atypical, it is often useful to adopt a clear anatomical approach. Consider investigations that will clearly identify the structure of the bronchial tree, lung parenchyma and the vascular supply and drainage. Describe each in logical and simple terms. Similarly, an individual assessment of function will assist in planning appropriate management. The natural history of some of the congenital lung lesions is often unknown. This gap in our knowledge has widened with the advent of detailed antenatal ultrasound scanning. Paediatricians are now faced with an increasing cohort of completely asymptomatic antenatally diagnosed congenital lung lesions and the best post-natal management is unclear. Long-term follow-up studies are required to document their natural history.     

Thyroglossal duct cyst accompanied by laryngomalacia and pulmonary sequestration

A 2‐month‐old full‐term female infant developed nasal stridor, which progressed to respiratory distress and poor sucking ability. Direct pharyngoscopy showed laryngomalacia and a midline cystic mass in the lingual region. The mass pressed on the epiglottis, causing dyspnea. Computed tomography incidentally revealed extralobar pulmonary sequestration. Direct deroofing of the lingual cyst and plication of the epiglottis were performed at 3 months of age, and the patient recovered from the respiratory distress. Histopathology of the cystic mass showed a thyroglossal duct cyst. Thoracoscopic resection of the pulmonary sequestration was then done at 17 months of age. Thyroglossal duct cysts in the lingual region may cause destabilization of the epiglottis and laryngomalacia, resulting in acquired respiratory obstruction. The combination of thyroglossal duct cyst, laryngomalacia, and pulmonary sequestration is rare; therefore, reports must be accumulated in order to explore the embryological origins of such cases.     

Identical twins concordant for pulmonary sequestration communicating with the esophagus and discordant for the VACTERL association

Communicating bronchopulmonary foregut malformations (CBPFMs) are unusual congenital structures composed of a segment of lung tissue connected to the foregut. We present what we believe is the first reported case of identical twins concordant for CBPFM who are discordant for the VACTERL association. Their nonfunctional lung tissue was successfully removed and the fistulae were corrected, and they are expected to live normal life spans. We review the literature concerning these malformations and the proposed theories of their etiology. This case report of concordance in identical twins suggests that a possible genetic component to CBPFMs cannot be ruled out. The discordance for the VACTERL association implies that the etiology is most likely multifactorial.     

Non-immune hydrops fetalis and bilateral pulmonary hypoplasia in a newborn infant with extralobar pulmonary sequestration

Extralobar pulmonary sequestration was found in a newborn premature infait that presented with non-immune hydrops fetalis, massive bilateral hydrothorax and polyhydramnios in utero. The baby died of severe respiratory insufficiency 15 h after birth. Postmortem examination revealed distended lymphatic vessels in the sequestered lung tissue probably due to impeded lymph drainage. We suggest that not extralobar pulmonary sequestration itself but a subsequent massive unilateral hydrothorax due to severe obstruction of lymph drainage was the cause of the non-immune hydrops fetalis, pulmonary hypoplasia and polyhydramnios. If these symptoms are diagnosed before delivery, a search for extralobar pulmonary sequestration is indicated.     

Congenital cystic adenomatoid malformation and extralobar sequestration occurring independently in the ipsilateral hemithorax

We report a case of congenital cystic adenomatoid malformation (CCAM) and extralobar pulmonary sequestration (EPS) occurring independently in the ipsilateral hemithorax. A literature search using Medline, Winspirs 2.0, found 14 previously reported cases of CCAM within an EPS. There are no reports of each form of congenital pulmonary abnormality occurring separately in the same patient. Accepted: 17 December 1998     

Intra-abdominal extralobar pulmonary sequestration presenting antenatally as a suprarenal mass

Extralobar pulmonary sequestration is a rare pulmonary parenchymal anomaly which rarely may be present in an intra-abdominal location. The authors report a case of intra-abdominal extralobar pulmonary sequestration which presented to us as an antenatally diagnosed suprarenal mass and was worked up as such. The diagnosis was revealed only at laparotomy. Intra-abdominal extralobar pulmonary sequestration should also be kept in differential diagnosis in cases of masses in the suprarenal location, especially on the left side.     

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.     

Infradiaphragmatic extralobar pulmonary sequestration in an infant

A rare case of infradiaphragmatic pulmonary sequestration with an antenatally diagnosed abdominal tumor is reported. Extirpation of the tumor was performed 2 months after delivery; the histological examination showed alveolar and bronchial tissue. Offprint requests to: M. Yagi     

机械通气新生儿肺功能的随访研究

目的探讨新生儿期机械通气对婴幼儿肺功能的影响。方法对42例生后接受机械通气治疗的患儿 ,于3～18月龄时进行肺功能随访。测定潮气量 (VT) ,吸呼比 (吸气时间/呼气时间 ,Ti/Te) ,达峰时间比 (到达潮气呼气峰流速的时间/呼气时间 ,TPTEF/TE) ,达峰容积比 (到达潮气呼气峰流速时的呼出气量/潮气量 ,VPEF/VE) ,呼气峰流速 (PEF) ,呼吸系统阻力 (Rrs)及顺应性 (Crs)。结果①机械通气组婴儿各项肺功能测定值与对照组相比 ,除Rrs外 ,差异均无显著意义(P>0.05) ,机械通气组Rrs为 (6.60±2.78)Kpa/(L·S) ,明显高于对照组 (4.91±1.97)Kpa/(L·S)(P<0.01)。②阻力与机械通气天数呈显著正相关(r=0.42,P=0.03)。结论新生儿期接受机械通气治疗的患儿 ,至婴幼儿期 ,除阻力外肺功能指标均已基本恢复正常。而阻力增高主要与机械通气时间过长有关。     

新生儿皮下脂肪坏死1例报告及文献回顾

目的探讨新生儿皮下脂肪坏死(Sc FN)的诊断与治疗。方法回顾性分析1例Sc FN患儿的临床资料,并结合文献分析Sc FN的病因、发病机制、鉴别诊断等。结果女性患儿,足月剖宫产,生后2 d因背部、肩部皮下硬结半天入院,抗感染治疗不佳。入院第10天患儿背部硬结局部出现软化、波动,局部穿刺液镜检示少量炎症细胞浸润,脂肪细胞变性,诊断为Sc FN。随访2月肿块消退,全身状况良好。结论 Sc FN是一种良性脂肪疾病,大多发生在有异常分娩史的足月新生儿,常发生于生后4周内,为自限性疾患,预后良好。     

Right pulmonary aplasia, aberrant left pulmonary artery, and bronchopulmonary sequestration with an esophageal bronchus

Pulmonary aplasia and bronchopulmonary foregut malformations in which a patent communication between the foregut and the pulmonary system is present are rare congenital abnormalities. Pulmonary aplasia associated with a pulmonary sling is an even rarer abnormality. We report a unique case of right pulmonary aplasia, aberrant left pulmonary artery, and bronchopulmonary sequestration with an esophageal bronchus diagnosed by multidetector helical CT.     

Pre- and postnatal treatment of a pulmonary sequestration presenting as a unilateral hydrothorax

We report a case of a neonatal sequestration diagnosed antenatally as a progressive left hydrothorax when ultrasound (US) was done at 30 weeks' gestation for polyhydramnios. Postnatal US and computed tomography failed to demonstrate any lesion; magnetic resonance imaging showed a spherical mass in the left chest adjacent to the left lower lobe. At the age of 4 weeks a thoracotomy was performed and the mass was excised. Pulmonary sequestrations may present with a pleural effusion and polyhydramnios as a part of non-immune hydrops fetalis. In our case hydrops was prevented by insertion of a pleuro-amniotic shunt.     

Torsion and infarction of an extralobar pulmonary sequestration

An 8-year-old girl presented with rapid onset of haemorrhagic pleural effusion due to torsion and infarction of an extralobar sequestration of the lung. A literature survey has failed to reveal a similar case.     

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.     

Familial severe congenital diaphragmatic hernia: Left herniation in one sibling and bilateral herniation in another

Familial congenital diaphragmatic hernia (CDH) is extremely rare; it comprises about 2% of all CDH cases. The empirical risk is about 2%, increasing to 10% in a family with two affected children. This report describes severe CDH in two siblings who had been diagnosed prenatally. The female newborn diagnosed with left CDH prenatally was born at 38 weeks of gestation. Despite surgical repair and intensive treatment, she died 10 days after birth. Her younger brother was born at 39 weeks of gestation after being diagnosed with bilateral CDH prenatally, and died 75 min after birth. Both infants had neither other congenital anomaly nor chromosomal abnormalities. Their parents are healthy without consanguinity. Their first daughter and the fourth child have no congenital anomalies.     

Prevalence and distribution of congenital abnormalities in Turkey: Differences between the prenatal and postnatal periods

The aim of this study was to determine the distribution of cases associated with congenital abnormalities during the following three periods: pregnancy, birth, and the neonatal period. This was a retrospective study of cases between 2002 and 2006. All abnormal pregnancies, elective terminations of pregnancies, stillbirths, and births with congenital abnormalities managed in the Neonatology Unit were classified based on the above distribution scheme. During the 5‐year study period, 1906 cases with congenital abnormalities were recruited, as follows: 640 prenatally detected and terminated cases, with most abnormalities related to the central nervous system, chromosomes, and urogenital system (56.7%, 12.7%, and 8.9%, respectively); 712 neonates with congenital abnormalities (congenital heart disease [49.2%], central nervous system abnormalities [14.7%], and urogenital system abnormalities [12.9%]); and hospital stillbirths, of which 34.2% had malformations (220 prenatal cases [34.4%] had multiple abnormalities, whereas 188 liveborn cases [26.4%] had multiple abnormalities). The congenital abnormalities rate between 2002 and 2006 was 2.07%. Systematic screening for fetal anomalies is the primary means for identification of affected pregnancies.