Fetal echogenic lung lesions: Prenatal ultrasound diagnosis and outcome

The differential diagnosis of echogenic areas in the fetal chest include congenital diaphragmatic hernia (CDH), cystic adenomatoid malformation (CAM), sequestrated lung and tracheal or bronchial atresia. The purpose of this study was to evaluate the accuracy of prenatal diagnosis and document outcome in fetuses with echogenic chest lesions. Seventeen fetuses with echogenic chest masses were seen in our unit between 17 and 36 weeks' gestation over a 5-year period. We reviewed these cases retrospectively for prenatal diagnosis, postnatal diagnosis and outcome. Prenatal diagnosis was correct in 13 fetuses, with CDH in 8, sequestrated lung in 4 and tracheal atresia in 1. Four fetuses had incorrect or uncertain prenatal diagnoses. In three fetuses CDH and CAM could not be differentiated. After delivery two of these had CDH and one had sequestrated lung. One fetus with bilateral lesions had prenatal diagnosis of bilateral CAM. Post-mortem examination revealed tracheal atresia as part of Fraser syndrome. All five babies with sequestrated lung are well and none required surgery. Ten fetuses had CDH, two pregnancies were terminated, one died in utero, five died as neonates and two babies survived following surgery. The study reveals that in a minority of fetuses CDH and CAM could not be differentiated prenatally. We agree with recent reports of fetal sequestrated lung describing sonographic improvement in utero. A large lesion on initial scan does not necessarily predict a poor neonatal outcome in this condition. This, together with the poor outcome in fetuses with echogenic CDH and tracheal atresia, has important implications for prenatal counselling.     

Imaging of congenital cystic lung lesions

Congenital cystic lung lesions are a rare but clinically significant group of anomalies, including congenital cystic adenomatoid malformation (CCAM), pulmonary sequestration, congenital lobar emphysema (CLE) and bronchogenic cysts. These conditions can all present on imaging studies as air or fluid filled cysts. Widespread use of antenatal ultrasound has led to increased detection of infants with congenital thoracic abnormalities in utero, resulting in a better understanding of the natural history of many of these lesions and also allowing provision to be made for delivery and postnatal management. More recently antenatal magnetic resonance (MR) imaging has provided further information on the nature of many of these lesions and helped to differentiate them from extrathoracic abnormalities such as congenital diaphragmatic hernia (CDH), which is important in parental counselling. Many children with congenital cystic lung lesions will present with symptoms resulting in the need for prompt diagnosis and treatment and imaging has an essential role in the management of these children. Some congenital lung lesions are treated surgically, whilst others are managed conservatively.     

Outcome of antenatally suspected congenital cystic adenomatoid malformation of the lung: 10 years' experience 1991-2001

OBJECTIVE: To determine the outcome of antenatally suspected congenital cystic adenomatoid malformation of the lung (CCAM) over a 10 year period. METHODS: This is a retrospective study of all babies diagnosed antenatally in the Prenatal Diagnosis Unit and delivered in Oxford between 1991 and 2001. Data were obtained from the Oxford Congenital Anomaly Register, theatre records, and histopathology reports. RESULTS: Twenty eight cases of CCAM were diagnosed antenatally. Five pregnancies were terminated. Data are available on all 23 of the pregnancies that continued and resulted in two neonatal deaths and 21 surviving babies. Eleven of the 23 cases (48%) showed some regression of the lesion antenatally, and four of these cases appeared to resolve completely on prenatal ultrasound. Three of the 23 babies (13%) were symptomatic in the early neonatal period, and three developed symptoms shortly afterwards. Seventeen of the 23 babies (74%) were asymptomatic, of whom 12 had abnormalities on chest radiograph or computed tomography scan and had elective surgery. Two babies (8%) had completely normal postnatal imaging, and three had abnormalities which resolved in the first year of life. Seventeen of the 23 babies (74%) had surgery. Histology at surgery was heterogeneous. Of the 23 live births, all 21 survivors (91%) are well at follow up or have been discharged. CONCLUSIONS: All babies diagnosed antenatally with CCAM require postnatal imaging with computed tomography irrespective of signs of antenatal resolution. In asymptomatic infants, the recommendations are close follow up and elective surgery for persistent lesions within the first year of life. Histology at surgery was heterogeneous, and this should be considered when counselling parents.     

Antenatal diagnosis of pulmonary tumours: report of two cases

We report two cases of antenatal diagnosis of pulmonary tumours. In one case a bronchogenic cyst was connected with the bronchopulmonary tree and grew rapidly after birth. Thanks to the antenatal diagnosis, it was resected before any complication appeared. In the second case, congenital cystic adenomatoid malformation of the lung was associated with fetal anasarca. Initially cyst aspiration was carried out; then long-term drainage was attempted. These cases illustrate the impact of antenatal diagnosis on perinatal management.     

The role of emergency thoracoscopy in the management of congenital cystic adenomatoid malformation of the lung associated with oesophageal atresia.

Congenital cystic adenomatoid malformation of the lung associated with oesophageal atresia is exceptional. The authors describe a case of a mixed type I - II congenital cystic adenomatoid malformation of the left lung associated with oesophageal atresia and tracheooesophageal fistula in a male infant. The interesting aspect of this case is not only the extremely rare association - only two reports in the literature - but the surgical choices. In fact, two weeks after surgical repair of the oesophageal atresia, the growth of the cystic volume of the congenital adenomatoid malformation made respiratory weaning very difficult, and it was therefore decided to solve the respiratory distress by opening the tensional cysts using a thoracoscopic access. The advantage of this treatment was that it decompressed the underlying healthy lung tissue and permitted the expansion of the normal lobar parenchyma. This is a palliative approach that allows the mandatory definitive resection of the affected lung lobe to be postponed until a later time.     

Fetal laryngoscopy and lung biopsy in a case of bilateral lethal congenital cystic adenomatoid malformation of the lung

A case of prenatal diagnosis of bilateral congenital cystic adenomatoid malformation of the lung (CCAM) is presented. The differential diagnosis among CCAM, pulmonary sequestration, bronchial obstruction, and laryngeal atresia is discussed. The role of diagnostic fetoscopy and fetal lung biopsy as an adjunct to ultrasound is addressed.     

FETAL LARYNGOSCOPY AND LUNG BIOPSY IN A CASE OF BILATERAL LETHAL CONGENITAL CYSTIC ADENOMATOID MALFORMATION OF THE LUNG

A case of prenatal diagnosis of bilateral congenital cystic adenomatoid malformation of the lung (CCAM) is presented. The differential diagnosis among CCAM, pulmonary sequestration, bronchial obstruction, and laryngeal atresia is discussed. The role of diagnostic fetoscopy and fetal lung biopsy as an adjunct to ultrasound is addressed.     

Pulmonary sequestration with histologic changes of cystic adenomatoid malformation

Pulmonary sequestration and congenital cystic adenomatoid malformation (CCAM) are two infrequent congenital pulmonary diseases. The combination of these two entities is rare. We report a case where the antenatal ultrasonography showed a left pulmonary mass suggesting CCAM. The US done after birth revealed an aberrant vascularisation. Pathologic examination confirmed the association of both lesions.     

Intralobar Sequestration of the Lung Is a Congenital Anomaly: Anatomopathological Analysis of Four Cases Diagnosed in Fetal Life

The origin of intralobar sequestration of the lung (ILS)—whether congenital or acquired—has often been controversial, since firm anatomical evidence of prenatal origin has been sparse. We describe four cases in which anomalous arterial supply from the aorta to abnormally echogenic lower lung lobes was demonstrated on routine antenatal ultrasound examination, and the diagnosis of ILS was subsequently confirmed by postmortem examination including bronchography of the fetuses at between 20 and 23 wk. One right-sided lesion was supplied from a branch of the coeliac axis, and three left-sided lesions were supplied from the thoracic aorta. In two cases, the arterial supply was shown to be multiple. All lesions were situated in the lower lobes. They showed a cystic transformation of the affected lung tissue which was histologically indistinguishable from cases diagnosed as congenital cystic adenomatoid lesion (CCAM) at the same gestational age, but clearly different from that seen in bronchial obstruction due to laryngeal stenosis at that age. Similar vascular lesions have been reported in otherwise normal lungs. The character of the anomalous arterial vessels is consistent with origin from the embryonic splanchnic plexus. The precise sequence of pathogenetic events is unclear but there appears to be a close relationship of this condition not only with CCAM but also with the condition of anomalous arterial supply to the lower lobe.     

Antenatal ultrasound findings in cystic adenomatoid malformation

A case of congenital cystic adenomatoid malformation of the lung is presented with prenatal ultrasound findings. The present case appears to be the third instance reported in which the diagnosis was made antenatally by ultrasound, and the first which resulted in a live birth, although the patient succumbed to pulmonary hypoplasia.     

Perinatal management of common neonatal thoracic lesions

Esophageal atresia, congenital diaphragmatic hernia, bronchopulmonary malformations and cystic lung diseases are the common neonatal thoracic surgical lesions encountered in practice. The availability of antenatal ultrasonography has lead to these lesions being detected before birth. Antenatal diagnosis can be made with a fair degree of accuracy in tertiary fetal medicine centres. Antenatal intervention is limited in a very few centres in the western world and not being done in India at present. The outcome of these babies with antenatal diagnosis of thoracic lesions has changed in the last decade. Earlier intervention is now possible in cystic lung disease before infectious complication has set in. All these lesions are managed exclusively in well developed neonatal surgery units with excellent outcome in the western world. The present study reviews the antenatal detection, clinical presentation, interventional/surgical procedures [antenatally and postnatally] and outcome of these common neonatal thoracic surgical lesions.     

产时子宫外处理技术治疗胎儿先天性肺囊性腺瘤样畸形的初步探讨

目的 初步探讨采用产时子宫外处理技术（EXIT）治疗胎儿先天性肺囊腺瘤样畸形的可行性.方法 2010年10月至2012年6月我们对3例胎儿先天性肺囊腺瘤样畸形采用产时子宫外处理技术切除.3例均经2次以上产前彩超诊断为先天性肺囊腺样畸形,且巨大畸形导致胎儿心脏、纵隔部分或全部移位,术前计算CVR值均大于1.6.结果 3例术后均存活,其中男1例,女2例,手术时胎龄36 ～37＋周,平均（36.86±0.79）周,体重2 700～3 000 g,平均（2 866.67±152.75）g;麻醉时间130～134 min,平均（133.67 ±3.51）min;总手术时间92～98 min,平均（94.33 ±3.21） min;手术时间33～49 min,平均（42.67±8.50） min;术中出血量2～20 mL;平均（10.67±9.02） mL.术后病理检查结果均为肺囊性腺瘤样畸形,左肺2例,右肺1例.结论 产时子宫外处理可以作为治疗高风险先天性肺囊腺样畸形的方法之一,但因手术风险大,操作较复杂,需多学科团队进行合作,需严格把握该技术的适应证,结合各方面条件,谨慎选择.     

先天性肺囊性疾病

先天性肺囊性疾病是较少见的先天性肺部疾病,是肺组织胚胎发育异常所形成的畸形。囊性病变包括囊性腺瘤样畸形、支气管源性肺囊肿、先天性大叶性肺气肿、肺隔离症。此类疾病的临床表现相似,但发生机制及病理改变有各自的特点。囊性腺瘤样畸形是由于细支气管发育停滞,以肺泡不发育为代价引起的肺间质大量增生,目前根据病理类型分成5型。支气管源性肺囊肿是胚胎发育时期支气管树某段的异常出芽形成的。先天性大叶性肺气肿是支气管内阻外压以及支气管壁的病变造成的气体陷闭,肺泡腔扩张。肺隔离症是部分肺组织与正常的支气管肺组织无交通,供血来自体循环,病变肺无正常功能,分叶内型和叶外型。     

Cystic adenomatoid malformation of the lung: neonatal management of 21 cases

Currently, congenital cystic adenomatoid malformation of the lung (CCAM) is often diagnosed antenatally by ultrasound, allowing prompt and appropriate medical and surgical management after birth. The authors report 21 cases of CCAM admitted from 1988 to 1997 to a neonatal intensive care unit and treated by high-frequency oscillation (HFO) and early surgery. Six infants developed respiratory distress, of whom 4 required ventilation by HFO. HFO was also the mode of ventilation used in all cases except 1 during the perioperative period. There was no death from respiratory failure. The authors emphasize the usefulness of antenatal diagnosis, the efficiency of HFO in cases with severe respiratory failure, and well-tolerated early surgery. Accepted: 5 January 1999     

Cystic intrapulmonary lymphangioma: HRCT findings

We report a rare case of cystic intrapulmonary lymphangioma involving the left lung, which presented with pneumothorax and respiratory distress in a 6-month-old infant. Chest radiographs showed a multicystic lesion in the left lung mimicking the features of congenital cystic adenomatoid malformation of the lung. The lesion appeared on high-resolution CT (HRCT) as a multiseptate, air-filled cystic lesion in the left hilar area. Associated HRCT findings were thickening of interlobular septa and bronchovascular bundles in the left lung and the presence of peripheral pulmonary vessels within cystic lesions in the apex of the left lung. HRCT findings correlated well with histopathologic findings. We suggest that these associated findings may be helpful in distinguishing this condition from other cystic lung diseases and that this entity should be included in the differential diagnosis of multicystic lung lesions.     

Polyalveolar Lobe and Congenital Cystic Adenomatoid Malformation Type II: Are They Related?

A left lower polyalveolar lobe in a 28-day-old infant and a right lung with congenital cystic adenomatoid malformation type II in a 17-day-old infant are described. The adenomatoid malformation involved all lobes, but the lower lobe appeared to be mostly replaced by a polyalveolar area, a complication hitherto undescribed. The pronounced increase in number of alveoli in both cases was established by two different morphometric methods and was compared with three normal controls of the same age. The simultaneous occurrence of polyalveolar lobe and congenital cystic adenomatoid malformation type II in the same lung suggests a causal relation between both anomalies. In the grossly abnormal lung tissue of an adenomatoid malformation, polyalveolar areas can easily be overlooked.     

Cystic adenomatoid malformation of the lung. Importance of prenatal diagnosis

Cystic adenomatoid malformation of the lung is a rare malformation of the lung airway which often performed diagnosed in the prenatal period by ultrasound. Ultrasound monitoring should be performed during pregnancy to assess lung development. We report the case of a 4-year-old patient with prenatal diagnosis of cystic adenomatoid malformation of the lung, not confirmed by chest radiograph at birth. The patient underwent surgery at 4 years of age after diagnosis was made for presenting recurrent pneumonia. A normal chest radiograph at birth does not exclude this malformation and a computerized tomography at 4 weeks of birth must be done to confirm or rule out this anomaly. Once the diagnosis is made, surgical treatment should be prompted to avoid complications.     

CONGENITAL CYSTIC ADENOMATOID MALFORMATION OF THE LUNG: A Report of Two Cases

ABSTRACT. Michalsen, H. (Department of Paediatrics, Ullevål Hospital, Oslo, Narway). Congenital cystic adenomatdd malformation of the lung. Acta Paediatr Scand, 63: 793, 1974.—The clinical picture of the congenital cystic adenomatoid malformation of the lung is reviewed. Two patients suffering from this entity are presented, the first patient was a newborn girl with acute respiratory distress shortly after birth, and the seeond patient was 12 months old and had a history of repeated pulmonary infections. The importance of, and difficulty in, differentiation between diaphragmatic hernia and the congenital cysts of this type in newborn infants is emphasized.     

Cystic adenomatoid malformation of the lung: clinical evolution and management

Cystic adenomatoid malformation of the lung (CAML) is a rare pulmonary maldevelopment resulting from an abnormal growth of the terminal bronchial structures. This study proposes a possible management of prenatally diagnosed CAML. A group of nine cases of CAML diagnosed prenatally between January 1990 and December 1995 was studied retrospectively. The evolution of lesions was followed in utero by serial ultrasound monitoring. Chest X-ray was performed at birth in all neonates and CT only in the symptomatic ones. CAML was macrocystic in three cases and microcystic in six. No polyhydramnios, hydrops or associated malformations were seen. In four cases, CAML was confirmed at birth and required surgery. The lesion decreased in size prenatally in five fetuses, of whom only one underwent surgery. In two further cases a prenatal diagnosis of CAML was changed to diaphragmatic hernia. Conclusion Given the possible clinical disappearance or resolution of cystic adenomatoid malformation of the lung, we believe that surgery is justified at birth only in symptomatic and radiologically positive neonates. It could be safely delayed in those asymptomatic patients with either positive or negative chest X-ray. The former need computed tomography at birth, whereas, in the latter, it should be performed at 6 months of age for a more definitive assessment of the patient. Received: 22 September 1998 / Accepted: 20 April 1999     

Pulmonary interstitial emphysema due to respiratory syncytial virus infection

Pulmonary interstitial emphysema (PIE) primarily affects premature infants on positive pressure ventilation. PIE is rarely reported in infants and children in the absence of mechanical ventilation and/or associated respiratory infection. We report a case of PIE in a 22‐month‐old girl who had severe respiratory distress due to respiratory syncytial virus infection. Chest computed tomography showed cystic lung lesions mimicking congenital cystic adenomatoid malformation. The cystic lesions spontaneously resolved after conservative treatment. Based on the clinical course and the chronological changes on imaging, the cystic lung lesions were diagnosed as localized persistent PIE.