Congenital Thoracic Malformations Research Articles

Clinical Presentation and Outcome of Congenital Thoracic Malformations in Children Beyond Neonatal Period

Objective: To describe the clinical spectrum of presentation and outcome of children with congenital thoracic malformations beyond neonatal age. Methodology: Cross sectional study conducted at inpatient department of National institute of child health from Jan-Dec 2021. All patients hospitalized in study duration with diagnosis of congenital thoracic formations from 1 month till 12 years of age were enrolled. History, examination, laboratory tests, treatment and outcome were recorded. Results: Total 44 children were enrolled with mean age of 7.7+10.8 months. Common congenital thoracic malformations were congenital diaphragmatic hernia 24(54.5%), congenital cystic adenomatoid malformation 6(13.6%), congenital lobar emphysema5(11.4%) and eventration of diaphragm 4(9.1%). Three (6.9%) patients had associated cardiac anomalies. Surgical intervention was done in 33 (75%) patients with a postoperative survival rate of 95.4%. Conclusion: Congenital diaphragmatic hernia and cystic adenomatoid malformation are common congenital thoracic malformations that present beyond neonatal age and are associated with good postsurgical outcome. Keywords: congenital diaphragmatic hernia, congenital cystic adenomatoid malformation, beyond neonate

Malformations thoraciques congénitales et implants sur mesure 3D. Nouvelle classification basée sur une série de 789 cas traités

The authors present a new study on 789 cases of congenital thoracic malformations including 638 pectus excavatum and 151 Poland syndromes, according to a new classification which completes Chin's one. All these malformations were treated with silicone elastomer implants. The contribution of computer-aided design and manufacturing (CAD/CAM) since 2008 is essential. The one-stage surgical protocol is precisely described. The results are impressive, permanent, for life, and complications are rare. The authors evoke a common vascular etiopathogenesis theory at the embryonic stage and question the heavy techniques of invasive remodeling that are most often unjustified.

Chirurgie réparatrice des malformations congénitales du sein et du thorax “Changer la vie”

The life of young girls and boys with congenital breast and thoracic malformations is more a matter of survival than of living, burdened by severe body image damage. Positively changing their lives is what these girls or young men are calling us to do. Our duty is certainly to understand their malformation, but also to understand its repercussions and thus to draw the most appropriate treatment. It is in this perspective that this report should be read.

Persistent Pulmonary Interstitial Emphysema With Respiratory Infection: A Clinicopathological Analysis of Six Cases and Detection of Infectious Pathogens by Metagenomic Next-Generation Sequencing (mNGS).

BackgroundPersistent pulmonary interstitial emphysema (PPIE) is known to be related to mechanical ventilation and preterm. However, PPIE is also reported rarely in non-ventilated and full-term infants. Its relationship with respiratory infection is rarely reported in the literature. PPIE is difficult to diagnose and always mimics other congenital thoracic malformations (CTMs), such as congenital cystic adenomatoid malformation (CCAM).ObjectiveThe objective of this study was to evaluate clinicopathological and radiographic features of PPIE with respiratory infection and to detect the possible infectious pathogens.MethodsFrom January 2011 to December 2019, six cases were confirmed pathologically with PPIE from a large cohort of 477 resected CTMs in West China Hospital of Sichuan University. Clinical and radiographic features were obtained from patients' medical records and follow-up. The present study aimed to analyze clinicopathological and radiographic features and to detect the infectious pathogens by metagenomic next-generation sequencing (mNGS).ResultsThe six PPIE cases included four girls and two boys, ranging from 2 months to 5 years; 100% (5/5) of the available cases were full-term and without mechanical ventilation. CCAM were suspected in 66.7% (4/6) patients; 66.7% (4/6) cases affected a single lobe, and 33.3% (2/6) cases affected both lung lobes. Clinically, all six PPIEs were presented with symptoms of respiratory infection and diagnosed with pneumonia. All six patients were treated by surgery after anti-infective treatment. The pathologic characteristics showed lung cysts with variable size along the bronchovascular bundles, the cysts had a discontinuous fibrotic wall with a smooth inner surface lined with uninucleated and/or multinucleated macrophages. Streptococcus pneumoniae was detected in patient No. 1. Human beta-herpesvirus 5 was detected in patient No. 2. Neisseria mucosa, Neisseria sicca, Prevotella melaninogenica, Prevotella histicola, and Fusobacterium nucleatum were detected in patient No. 5, and no infectious pathogen was detected in 50% (3/6, No. 3, No. 4, and No. 6) of cases.ConclusionSix rare cases of PPIE with respiratory infection were treated by surgery after anti-infective treatment. All five available cases were full-term infants without mechanical ventilation. The histological characteristics of PPIE were the wall of cysts composed of a thin layer of discontinuous fibrous tissue and lined with uninucleated or/and multinucleated macrophages.

Musculoskeletal deformities in children with congenital thoracic malformations: a population-based cohort study.

It is unclear if musculoskeletal deformities observed in patients with congenital diaphragmatic hernia (CDH), congenital lung lesion (CLL) and esophageal atresia/tracheoesophageal fistula (EA/TEF) are associated with the anomaly or are a result of the surgery required to treat the anomaly. This study compared the prevalence of musculoskeletal deformities for: (1) children with congenital thoracic anomalies to controls; (2) CLL to EA/TEF both repaired via thoracotomy; and (3) CLL and EA/TEF to CDH repaired via laparotomy. We performed a retrospective study of children with CLL, CDH or EA/TEF between 1990 and 2016. Date-of-birth-matched control groups were generated from a population-based dataset. International Classification of Disease codes were used to identify scoliosis and pectus anomalies. We determined Hazard ratios (HR) for cases versus controls. We included 167 cases (CDH n = 82; CLL n = 29; EA/TEF n = 56) and 1670 controls. EA/TEF had a greater risk of scoliosis (HR 5.52, 95%CI 1.49,13.73) and pectus deformities (HR 4.07, 95%CI 1.96,8.45). CDH showed more scoliosis (HR 5.03, 95%CI 1.99,12.74) but not pectus anomalies. Musculoskeletal deformities were not more common in CLL. Children born with CDH or EA/TEF, but not CLL, had more musculoskeletal deformities than controls. The inconsistent association between musculoskeletal deformities and the surgical approach suggested a congenital predisposition.

Antenatal diagnosis of congenital pulmonary airway malformation – two case report

BackgroundCongenital Pulmonary Airway Malformation (CPAM), is the most common Congenital Thoracic Malformation, accounting for 95% of congenital echogenic lung lesions detected antenatally on ultrasonography (USG).
 Antenatally diagnosed CPAM may regress or result in hydrops fetalis. Management options include expectant fetal follow-up, maternal administration of steroids and fetal surgery. We present two cases of antenatally diagnosed CPAM with both mothers consenting to and receiving antenatal steroids.
 Case 1
 A 31-year-old primigravida was noted with homogeneous highly hyperechoic right lung mass measuring 3.0 x 3.1 x 2.5cm at 24 weeks gestation on USG. The mass-to-thorax ratio (MTR) was 0.72, with no systemic feeding artery noted, stomach bubble was normally situated, there was no hydrops. An initial diagnosis of CPAM of the right lung was made. At 38 weeks’ gestation, USG showed mass was unmeasurable. Delivery was via CS at 39 weeks. Baby was well at birth and discharged home after 48 hours of monitoring, baby is 4 months old and growing well
 Case 2
 A 33-year-old G4P3AA was diagnosed with CPAM on USG at 22 weeks’ gestation. There was displacement of fetal heart to the right hemi-thorax. Colour Doppler did not show a systemic feeding arterial supply, or hydrops, and no associated structural anomaly. Serial USG for MTR at 28- and 32-weeks’ gestation showed increase of 0.72 and 0.86 respectively, with polyhydramnios. Further USG noted MTR of 0.57. Delivery was by emergency caesarean section (CS) for prolonged labour at term. Baby required admission due to hypoxia and respiratory distress on the first day of life, baby was discharged on day 14 post-delivery. Chest computed tomographic angiogram noted a feeding vessel to part of the right lung. Patient is 6 months old and has undergone a successful surgery.
 Conclusion
 Antenatal USG diagnosis and multidisciplinary approach to management in Congenital Thoracic Malformations is essential and improves antenatal and post-natal outcomes.

Transthoracic Vertebral Distraction and Stabilization in 10 Dogs with Congenital Thoracic Vertebral Malformations.

The aim of this study was to describe the surgical technique and outcomes of dogs with congenital thoracic vertebral body malformations (CTVBM) treated with unilateral vertebral distraction and stabilization with monocortical screws and polymethylmethacrylate (PMMA). Medical records of 10 client-owned dogs with CTVBM were retrospectively searched for signalment, history, neurological examination, neurological grade based on the modified Frankel scale (MFS), diagnostic method, CTVBM location, Cobb's angle, and vertebral canal angle (VCA), surgical procedure, complications and long-term follow-up. One dog suffered cardiac arrest 24 hours post-surgery and died. Median follow-up in nine dogs was 26.5 months (12-50 months). Cobb's angle improved from 33-83 to 10-46 degrees and the VCA ratio from 97-138 to 122-164 degrees. The MFS improved from 3 (n = 3)-4 (n = 7) to 4 (n = 2)-6 (n = 7). Seven dogs recovered full function. Nine dogs improved after surgery compared with preoperative status, recovering ambulation when lost or achieving normal neurological examination in seven cases. No complications were reported during the follow-up period (12-50 months). Unilateral transthoracic vertebral distraction and stabilization with monocortical screws and PMMA for treatment of compressive myelopathy due to CTVBM achieved long-term neurological improvement in 9 of 10 dogs.

They SHALL grow old: a UK rare disease clinical network for adult congenital thoracic malformations

Alexander Pope’s line ‘A little learning is a dangerous thing’ (note: NOT knowledge) is probably one of the most misquoted in all literature, but certainly applies today to many rare...

Reference range of fetal thorax using two-dimensional and three-dimensional ultrasound VOCAL technique and application in fetal thoracic malformations

BackgroundTo establish the normal reference range of fetal thorax by two-dimensional (2D) and three-dimensional (3D) ultrasound VOCAL technique and evaluate the application in diagnosing fetal thoracic malformations.MethodsA prospective cross-sectional study was undertaken involving 1077 women who have a normal singleton pregnancy at 13–40 weeks gestational age (GA). 2D ultrasound and 3D ultrasound VOCAL technique were utilized to assess fetal thoracic transverse diameter, thoracic anteroposterior diameter, thoracic circumference, thoracic area, lung volume, thoracic volume and lung-to-thoracic volume ratio. The nomograms of 2D and 3D fetal thoracic measurements were created to GA. 50 cases were randomly selected to calculate intra- and inter-observer reliability and agreement. In addition, the case groups including congenital skeletal dysplasia (SD) (15), congenital diaphragmatic hernia (CDH) (30), pulmonary sequestration (PS) (25) and congenital cystic adenomatoid malformation (CCAM) (36) were assessed by the nomograms and followed up subsequently.ResultsBoth 2D and 3D fetal thoracic parameters increased with GA using a quadratic regression equation. The intra- and inter-observer reliability and agreement of each thoracic parameter were excellent. 2D fetal thoracic parameters could initially evaluate the fetal thoracic development and diagnose the skeletal thoracic deformity, and lung volume, thoracic volume and lung-to-thorax volume ratio were practical to diagnose and differentiate CDH, PS and CCAM.ConclusionWe have established the normal fetal thoracic reference range at 13–40 weeks, which has a high value in diagnosing congenital thoracic malformations.

Structural and perfusion magnetic resonance imaging of congenital lung malformations

BackgroundA radiation-free advanced imaging modality is desirable for investigating congenital thoracic malformations in young children.ObjectiveTo describe magnetic resonance imaging (MRI) findings of congenital bronchopulmonary foregut malformations and investigate the ability of lung MRI for their classification.Materials and methodsThis is a retrospective analysis of consecutive MRI examinations performed for suspected congenital lung anomalies in 39 children (median age: 3.8 months, range: 2 days–15 years). Morphological and perfusion findings were characterised on respiratory-gated fast spin echo and dynamic contrast-enhanced sequences obtained at 1.5 tesla. Abnormalities were classified independently by two readers and compared to an expert diagnosis based on pathology, surgery and/or other imaging.ResultsMain diagnoses included bronchopulmonary lesions in 33 patients, scimitar syndrome in 4 patients, pulmonary arteriovenous malformation and oesophageal duplication cyst in one patient each. Of 46 observed abnormalities, 44 (96%) were classified correctly with very good interobserver agreement (96% concordance rate). The 39 detected lung lesions included isolated overinflation (17/39, 44%), cystic pulmonary airway malformation (8/39, 21%), bronchopulmonary sequestration (7/39, 18%), bronchogenic cyst (4/39, 10%) and hybrid lesion (3/39, 8%). All lung lesions presented as perfusion defect at peak pulmonary enhancement. Non-cystic lesions showed a delayed peak (median delay: 2.8 s, interquartile range: 0.5 to 4.0 s) in relation to normal lung parenchyma.ConclusionA dedicated lung MRI protocol including respiratory compensated sequences, dynamic angiography and perfusion is able to reliably delineate parenchymal and vascular components of congenital bronchopulmonary foregut malformations. Therefore, MRI may be considered for comprehensive postnatal evaluation of congenital thoracic malformations.

Incidence of congenital thoracic malformations detected by prenatal ultrasound.

The increased and earlier use of prenatal ultrasound has facilitated the detection of congenital thoracic malformations (CTMs). Our Pediatric Pulmonology Institute follows an increasing number of patients with CTMs. Thus, we aimed to evaluate and describe prenatal sonographic findings of CTM, and to estimate changes in detection rates of CTMs over a period of 16years. A retrospective, cross-section analysis of prenatal ultrasound (US) screening tests carried out in a large community-based clinic, comparing two periods: 2001-2007 and 2007-2017. A total of 34716 prenatal US were performed at a median gestational age of 15.4 weeks (range, 11.6-23.9) and 15.7 weeks (range, 12-33.6) in 2001-2007 and 2007-2017, respectively. In 2001-2007, 12016 prenatal US tests detected 19 CTMs, compared to 30 CTMs in 22 700 tests in 2007-2017. Detection rates did not change (1.58/1,000 in 2001-2007 versus 1.32/1,000 in 2007-2017, P = 0.64). The most common abnormality was congenital pleural effusion (CPE) (17 cases, 34.7%), followed by congenital pulmonary airway malformation) and congenital diaphragmatic hernia; 13 cases each, 26.5%. Twenty CTMs, mainly congenital diaphragmatic hernia and CPE, were associated with other fetal lesions. Congenital diaphragmatic hernia and CPE tend to appear with multiple lesions and warrant further attention. The incidence rates stayed stable when comparing the last decade to previous years. Thus, the increased referral of CTM can be attributed to an increase in the number of prenatal screening studies performed, rather than a true higher incidence.

Utility of open surgical lung biopsy in children

IntroductionOpen lung biopsy (OLB) is commonly used to obtain a definitive histological diagnosis in children with respiratory disorders. This allows correct treatment pathways to be followed and guides discussions surrounding prognosis. Our aim was to determine if OLB is useful in obtaining a conclusive diagnosis in a child with complex respiratory disease. Materials and methodsIn total, 179 OLB episodes were identified in children under 18 years from 2006 to 2016. Biopsies confirming congenital thoracic malformations or pulmonary metastatic disease were excluded. Results42 patients had 44 episodes of OLB in the period studied. A definitive histological diagnosis was reached in 35 (79%) cases. There were no deaths directly attributable to OLB surgery. ConclusionOLB contributed substantially in obtaining a definitive diagnosis for our patient population, no increase in mortality. It allowed targeted treatment and provided valuable prognostic information on the likely progression of the disease so families could plan for palliation where appropriate.

Congenital Lung Malformations: Unresolved Issues and Unanswered Questions.

Advances in prenatal and postnatal diagnosis, perioperative management, and postoperative care have dramatically increased the number of scientific reports on congenital thoracic malformations (CTM). Nearly all CTM are detected prior to birth, generally by antenatal ultrasound. After delivery, most infants do well and remain asymptomatic for a long time. However, complications may occur beyond infancy, including in adolescence and adulthood. Prenatal diagnosis is sometimes missed and detection may occur later, either by chance or because of unexplained recurrent or persistent respiratory symptoms or signs, with difficult implications for family counseling and substantial delay in surgical planning. Although landmark studies have been published, postnatal management of asymptomatic children is still controversial and needs a resolution. Our aim is to provide a focused overview on a number of unresolved issues arising from the lack of an evidence-based consensus on the management of patients with CTM. We summarized findings from current literature, with a particular emphasis on the vigorous controversies on the type and timing of diagnostic procedures, treatments and the still obscure relationship between CTM and malignancies, a matter of great concern for both families and physicians. We also present an algorithm for the assessment and follow-up of CTM detected either in the antenatal or postnatal period. A standardized approach across Europe, based on a multidisciplinary team, is urgently needed for achieving an evidence-based management protocol for CTM.

Antenatally Detected Congenital Thoracic Malformations and Its Postnatal Follow-Up in Foetal Surgical Congenital Malformations Counseling Center: A Retrospective Study

Antenatally Detected Congenital Thoracic Malformations and Its Postnatal Follow-Up in Foetal Surgical Congenital Malformations Counseling Center: A Retrospective Study

Outcomes of nonsurgical treatment for congenital thoracic vertebral body malformations in dogs: 13 cases (2009-2016).

OBJECTIVE To characterize outcomes following nonsurgical treatment of congenital thoracic vertebral body malformations causing neurologic deficits in dogs. DESIGN Retrospective case series. ANIMALS 13 client-owned dogs treated nonsurgically for congenital thoracic vertebral body malformations at 3 veterinary referral hospitals from June 2009 through May 2016. PROCEDURES Data were extracted from the medical records regarding dog signalment, duration and type of clinical signs before referral, general physical and neurologic examination findings, radiographic and MRI findings, and treatments provided after diagnosis. Follow-up data were obtained from records of recheck examinations and via a standardized owner questionnaire. RESULTS All included dogs were screw-tail brachycephalic breeds with a median age of 6 months. All dogs had ambulatory paraparesis and ataxia, and in 1 dog, signs of spinal hyperesthesia could be elicited. Nonsurgical treatments consisted of restricted exercise without (n = 5) or with (3) physiotherapy, physiotherapy without restricted exercise (3), and no exercise modification (2). Seven dogs received additional nonsurgical treatment consisting of prednisolone (n = 5) or gabapentin (2). Four dogs were eventually euthanized because of progressive neurologic deterioration, 2 underwent surgery for the same reason, and the remaining 7 dogs survived for ≥ 170 days after diagnosis, despite progressive neurologic deterioration. CONCLUSIONS AND CLINICAL RELEVANCE Nonsurgical treatment of congenital thoracic vertebral body malformations was associated with an unfavorable outcome in this group of dogs. Despite this treatment, all dogs had progression of clinical signs.

Radiographic assessment of the congenital thoracic vertebral malformations in French bulldogs

Radiographic assessment of the congenital thoracic vertebral malformations in French bulldogs

Early prenatal ultrasound diagnosis of congenital thoracic malformations

Objectives: To evaluate and describe the spectrum and rate of congenital thoracic malformations (CTMs) diagnosed by early prenatal sonography (gestational age (GA) less than 16 weeks).Methods: A retrospective, cross-sectional analysis of prenatal ultrasound screening tests in a community-based clinic.Results: In 2001–2017, 31 261 prenatal ultrasound tests detected 31 CTMs at a gestational age of 15.2 (range, 11.6–16.0) weeks. The most common malformation was congenital pleural effusion (CPE) (15 fetuses, 0.48/1000), followed by congenital diaphragmatic hernia (CDH) (10 fetuses, 0.32/1000). Pulmonary hypoplasia (PH), congenital pulmonary airway malformation and broncho-pulmonary sequestration appeared in much smaller proportions (three, two and one fetuses, respectively). Most CTMs were associated with additional fetal lesions (15 fetuses, 48%). All early CDH (10 fetuses) and PH (three fetuses) and 6/15 with CPE had termination of pregnancy or missed abortions.Conclusions: Prenatal ultrasound before 16 GA was able to detect CTMs in 0.99/1000 of screening ultrasound (US) performed. Most CTMs tended to appear with multiple lesions and were associated with unfavorable outcomes. Earlier prenatal diagnosis may enable early termination of pregnancy in fetuses with lethal malformations.

Exercise capacity is not decreased in children who have undergone lung resection early in life for congenital thoracic malformations compared to healthy age-matched children.

The purpose of this study was to compare (i) the exercise capacity and (ii) lung function prior to and immediately post cardiopulmonary exercise tests (CPET) of children who underwent early life lung resection for Congenital Pulmonary Airway Malformations (CPAM) to healthy control children. Eight children with CPAM (four males, age 9.6 ± 1.8 years) and eight control children without respiratory disease (three males, age 9.4 ± 1.4 years) performed a CPET on a cycle ergometer, during which maximal oxygen consumption (V̇O2max ) and heart rate were measured. Prior to and immediately post CPET, lung function measures including Nitrogen Multiple Breath Washout (MBW) and spirometry were performed. There were no significant between group differences in pre CPET lung function (P > 0.05) or maximal exercise capacity (V̇O2max CPAM: 39.4 mL. kg-1. min-1 , Control: 40.5 mL. kg-1. min-1 ). Post CPET, FEV1 was significantly lower in the CPAM group, with two participants diagnosed subsequently with exercise induced bronchospasm based on post-CPET spirometry and follow-up clinical investigations. Early life lung resection for CPAM does not appear to have negative implications for exercise capacity later in childhood. Clinicians should be aware that dyspnoea following exercise may be due to asthma rather than residual effects of CPAM in these children.

Editorial: Old Problems and New Solutions in Pediatric Pulmonology

The series on pediatric pulmonology continues in this issue of the Journal, with four manuscripts that illustrate the new problems of our burgeoning speciality [1–4]. These are how the management of old problems (in this case TB) is being transformed by cutting edge science [1]; how the application of new technologies are creating challenging problems (congenital lung disease) [2]; and how new technologies are transforming the lives of children (neuromuscular disease and NIV) and taking mechanical ventilation, what used to be the prerogative of intensive care into the home, transforming the prognosis of children [3, 4]. However, as we shall see, the more the speciality advances, the more the basic truths we learned as students remain the bedrock of our practice. Old disease, new technology: TB, the captain of the men of death, used to be simple – the tuberculin skin test, sputum (or other biological sample) microscopy and culture, and evidence based treatment with quadruple therapy for 2 mo, and rifampicin and isoniazid for a further 4 mo. Problem solved, even though diagnostics and determining sensitivities might take weeks – treatment could confidently be started while waiting. In children, admittedly diagnosis often had to be on clinical suspicion, because it was difficult to obtain samples. Enter the Dragon! Or rather, multidrug-resistant TB, extremely drug-resistant TB and totally drug resistant TB [5]. Perspectives have had to change rapidly; now we need rapid diagnosis and rapid sensitivity testing, for fear of starting drugs to which the organism is resistant, effectively but inadvertently proposing a single agent regime which will lead to worse problems of drug resistance. The problem has been made worse with the advent of HIVand the consequent worsening of the manifestations of TB. The modern approach is firstly to use novel tests to obtain specimens, such as sputum induction which can be taken in very young babies, and the string test. The second approach is newer microscopy and culture techniques, and specific molecular diagnostics, which are reviewed [1]. Molecular tests not merely detect MTB rapidly in sputum or gastric aspirates, but also allow drug sensitivities to be determined, a major management advance. The future will undoubtedly include molecular handprints on blood samples, as sophisticated techniques become cheaper and more accessible. New technology, new problems: Increased monitoring of pregnant women with ultrasound scanning, and increasingly MRI, has identified abnormalities which previously passed us by. Congenital lung malformations come into just this category. In this issue, a simple clinical classification of congenital thoracic malformations (CTM), which is applicable preand post-natally, is described. However, description is one thing, evidence based management is another. How should you counsel a woman with a fetus with a large CTM at 20 wk of the pregnancy? If the baby is born well, what further imaging should be done and when? What is the risk of complications, and should well babies be submitted to surgery to reduce the risk? And when should surgery be performed? Finally, the biggest elephant in the room, what is the risk of primary lung malignancy in a baby with a CTM? High risk groups can be delineated, but most babies with CTMs are low risk. * Andrew Bush A.Bush@rbht.nhs.uk

Rare Lung Diseases: Congenital Malformations.

Increasingly, congenital thoracic malformations (CTMs) are diagnosed on antenatal ultrasound, but we lack the evidence to suggest rational management, not least because descriptive terms are used inconsistently. This review describes a simplified clinical classification of CTMs, and contrasts it with pathological descriptions. The age related presentations of CTM are described, together with the differential diagnoses of cystic masses presenting both antenatally and postnatally. Antenatally diagnosed CTMs rarely require intervention before birth; and urgent treatment is only required postnatally if the baby is symptomatic and does not respond to medical management. The asymptomatic baby with an antenatal diagnosis of a CTM presents a management conundrum. Definitive imaging is with high-resolution computed tomography (HRCT), but the optimal timing of imaging is unclear. Whether surgery should be offered to asymptomatic infants is also unclear; in the medium term, 5 % of asymptomatic babies will require surgery for complications of the disease. The most vexed question is malignant change; the risk in the medium term is probably less than 5 %, but we have no way of delineating a high-risk group. Indeed, malignancy has been described even after complete resection of a CTM. The author's personal management is to advocate surgery in the second year of life for all except for the most trivial CTMs, but many would differ and advocate conservative management. More data are needed if we are to rationalise our approach to these infants.