Serial lung mass volume ratios as prognostic indicators of neonatal respiratory morbidity in fetal pulmonary malformations.

BACKGROUND: Several studies have shown that the congenital pulmonary airway malformation volume ratio is a useful prognosticator of neonatal outcome in prenatally diagnosed lung lesions. However, there remains a lack of consensus on which congenital pulmonary airway malformation volume ratio values have the best predictive value because of operator dependence, inherent changes in lung lesion size throughout gestation, and the widespread use of maternal steroids. OBJECTIVE: This study sought to determine the association between serial congenital pulmonary airway malformation volume ratio measurements and neonatal outcomes among fetuses with lung malformations. STUDY DESIGN: This was a retrospective cohort study of fetuses with a prenatally diagnosed lung malformation managed at 2 major fetal centers from January 2010 to December 2021. Prenatal variables, including prospectively measured congenital pulmonary airway malformation volume ratio measurements (initial, maximum, and final), were analyzed. The results were correlated with 3 outcome measures, namely surgical resection before 30 days of life, a need for supplemental O2 at birth, and endotracheal intubation at birth. Statistical analyses were performed using receiver operating characteristic curve analyses, Welch 2 sample t tests, and multivariable logistic regressions (P<.05). RESULTS: There were 123 fetuses with isolated lung lesions identified. Eight (6.5%) had hydrops. The mean initial congenital pulmonary airway malformation volume ratio was 0.67±0.61 cm2 at 22.9±3.9 weeks' gestation. The mean maximum congenital pulmonary airway malformation volume ratio was 1.08 ± 0.94 cm2 at 27.0 ± 4.0 weeks' gestation. The mean final congenital pulmonary airway malformation volume ratio was 0.58±0.60 cm2 at 33.2±4.1 weeks' gestation. At a mean gestational age at delivery of 38.3±2.6 weeks, 15 (12.2%) underwent neonatal lung resection for symptomatic disease. In a multivariable regression, all 3 congenital pulmonary airway malformation volume ratio measurements showed a significant correlation with neonatal lung resection (P<.001). Optimal congenital pulmonary airway malformation volume ratio cutoffs were established based on an initial congenital pulmonary airway malformation volume ratio of ≥0.8 cm2, maximum congenital pulmonary airway malformation volume ratio of ≥1.5 cm2, and a final congenital pulmonary airway malformation volume ratio of ≥1.3 cm2 with associated areas under the curve of 0.89, 0.97, and 0.93, respectively. The final congenital pulmonary airway malformation volume ratio had the highest specificity for predicting surgical lung resection in the early postnatal period. CONCLUSION: Measuring congenital pulmonary airway malformation volume ratios throughout pregnancy in fetuses with pulmonary malformations has clinical value for prenatal counseling and planning care transition after delivery. Fetuses with a final congenital pulmonary airway malformation volume ratio of more than 1.3 cm2 are likely to require neonatal surgery and therefore should be delivered at tertiary care centers with a neonatal intensive care unit and pediatric surgical expertise.

Congenital lung malformation patients experience respiratory infections after resection: A population-based cohort study.

PURPOSE: The benefit of elective resection of congenital lung malformations continues to be debated. Proponents of resection endorse a decreased risk of respiratory complications as one indication for surgery. Our study aimed to compare the prevalence of respiratory infections in cases, before and after resection of congenital lung malformations, to controls without a history of congenital lung malformation. METHODS: We performed a retrospective cohort study of children born from 1991 to 2007 who underwent congenital lung malformation resection. Patients were identified from Winnipeg´s Surgical Database of Outcomes and Management (WiSDOM), and a 10:1 date-of-birth matched control group was generated from a population-based administrative data repository. International Classification of Disease codes were used to assess pulmonary infection outcomes. Relative rates (RR) were calculated to compare the frequency of pneumonia, respiratory infections and influenza between cases and controls. RESULTS: We included 31 congenital lung malformation cases and 310 controls. Cases consisted of 14 (45.16%) congenital pulmonary airway malformations, 9 (29.03%) bronchopulmonary sequestrations and 8 (25.81%) hybrid lesions. Before resection, pneumonia was more common in cases than controls (RR 6.85; 95%CI 3.89, 11.9), while the risk of acute respiratory infections (RR 1.21; 95%CI 0.79, 1.79) and influenza (RR 0.46; 95%CI 0.01, 3.22) were similar to controls. Post-resection, the risk of pneumonia (RR 9.75; 5.06, 18.50) was still higher in cases than controls, and respiratory infections (RR 1.77; 95%CI 1.20, 2.53) and influenza (RR 3.98; 95%CI 1.48, 9.36) were more common in cases than controls. CONCLUSION: Our study demonstrated that after resection of congenital lung malformations, children experience more frequent respiratory infections compared to the general population. Resection does not eliminate the increased risk of pneumonia.

Incidence of congenital thoracic malformations detected by prenatal ultrasound.

BACKGROUND: 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 16 years. METHODS: 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. RESULTS: A total of 34 716 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, 12 016 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. CONCLUSIONS: 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.

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.

Malformaciones congénitas pulmonares diagnosticadas en un periodo de 10 años / Congenital Pulmonary Malformations Diagnosed Over a Period of 10 Years

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Is congenital pulmonary airway malformation really a rare disease? Result of a prospective registry with universal antenatal screening program.

BACKGROUND: Congenital pulmonary airway malformation (CPAM) is an increasingly recognized disease with potential mortality. Owing to limited published studies, the true incidence is yet to be determined. We carried out this prospective study with the aim to estimate its true incidence on a population basis. METHODS: An antenatal ultrasonography program was implemented since 2009. Fetuses with suspected intra-thoracic lesions were monitored by regular follow-ups. Antenatal course, postnatal outcomes, and other demographics were compared to those of patients with CPAM in the previous decades (1989-2008). The incidence of CPAM was calculated in different periods. RESULTS: 66 CPAM patients were identified between 2009 and 2014 with 62 patients being detected by antenatal scan. In contrast, 45 patients were identified between 1989 and 2008 with 27 patients being detected antenatally. The incidence rate during the past and recent period was estimated as ~1 in 27,400 and ~1 in 7200 live births, respectively (p = 0.024). CONCLUSION: With increasing awareness of clinicians and the universal use of latest ultrasound technology, it is likely that more CPAM cases will be detected in the future. Here, we presented our best estimated incidence rate of CPAM, yet only a larger scale study can reveal its true incidence.

The Association of H1N1 Pandemic Influenza with Congenital Anomaly Prevalence in Europe: An Ecological Time Series Study.

BACKGROUND: In the context of the European Surveillance of Congenital Anomalies (EUROCAT) surveillance response to the 2009 influenza pandemic, we sought to establish whether there was a detectable increase of congenital anomaly prevalence among pregnancies exposed to influenza seasons in general, and whether any increase was greater during the 2009 pandemic than during other seasons. METHODS: We performed an ecologic time series analysis based on 26,967 pregnancies with nonchromosomal congenital anomaly conceived from January 2007 to March 2011, reported by 15 EUROCAT registries. Analysis was performed for EUROCAT-defined anomaly subgroups, divided by whether there was a prior hypothesis of association with influenza. Influenza season exposure was based on World Health Organization data. Prevalence rate ratios were calculated comparing pregnancies exposed to influenza season during the congenital anomaly-specific critical period for embryo-fetal development to nonexposed pregnancies. RESULTS: There was no evidence for an increased overall prevalence of congenital anomalies among pregnancies exposed to influenza season. We detected an increased prevalence of ventricular septal defect and tricuspid atresia and stenosis during pandemic influenza season 2009, but not during 2007-2011 influenza seasons. For congenital anomalies, where there was no prior hypothesis, the prevalence of tetralogy of Fallot was strongly reduced during influenza seasons. CONCLUSIONS: Our data do not suggest an overall association of pandemic or seasonal influenza with congenital anomaly prevalence. One interpretation is that apparent influenza effects found in previous individual-based studies were confounded by or interacting with other risk factors. The associations of heart anomalies with pandemic influenza could be strain specific.

Surgical outcomes for congenital lung malformations: 10 years experience at a single center.

OBJECTIVE: To study the surgical outcome of congenital lung malformation and natural history of the disease at Srinagarind Hospital. MATERIAL AND METHOD: Retrospectively review the medical records of 25 patients diagnosed with congenital lung malformation who underwent surgical treatment between January 2001 and December 2011. RESULTS: Twenty-five patients diagnosed with congenital lung malformation underwent surgery: 16 males (64%), 9 females (36%), median age seven months, median body weight 6 kg. Twelve (48%) had congenital cystic adenomatoid malformation congenital cystic adenomatoidmaflormation (CCAM), seven (28%) pulmonary sequestration, four (16%) congenital lobar emphysema, and one a bronchogenic cyst (4%). The most common presenting symptoms were respiratory tract infection (14, 56%), respiratory distress (7, 28%), lung abscess (1, 4%), hemoptysis (1, 40%), and asymptomatic (2, 8%). Post-operative mortality over 30 days was 0. Complications included lung infection, atelectasis, post-operative bleeding, and wound infection. Post-operative follow-up (lasting between 1-8 years) revealed normal tolerance to daily activity without any respiratory problems. CONCLUSION: Surgical treatment for congenital lung malformations had a good result with few complications.

Pre and perinatal aspects of congenital cystic adenomatoid malformation of the lung.

OBJECTIVE: To identify the incidence of congenital cystic adenomatoid malformation of the lung (CCAM) at birth; to evaluate prenatal and perinatal history, association with clinical and sociodemographic variables and concordance between CT scan results and anatomopathology studies. METHOD: Descriptive study based on the registry of malformed newborns, deliveries and patients records between August 1990 and November 2010. Ultrasonic, clinical, imaging and anatomopathologic information were studied. Association studies were made using chi-square test. Kappa was used to correlate CT scan to anatomopathology results. RESULTS: The incidence was 1:1980 (25/49 503). The mean gestational age for detection by ultrasonography was 24 ± 3.7 weeks. There were progression of the lesions in 11 cases (44%), stability in 6 (24%) and regression in 8 (32%). Three cases of CCAM followed due to polyhydramnios/hydrops died. There were neither familial cases nor association with sex, weight, age or maternal parity (p > 0.15). Radiographic abnormalities were found in 22/23 studied patients. The correspondence between CT scan and anatomopathologic was 0.77 (Kappa). CONCLUSIONS: The incidence was higher than the one described in the literature, probably, because it is a reference center in fetal medicine. The prenatal lesion involution rate was 32%, an intermediate proportion. There was good concordance between CT scan and anatomopathologic results. The polyhydramnios/hydrops were predictive of worst prognosis.

Malformación adenomatoidea quística congénita / Congenital cystic adenomatous malformation

Introduction: Congenital Cystic Adenomatous Malformation (CCAM) is an infrequent entity due to an alteration in alveolar-pulmonary development. Material and Methods: A descriptive, retrospective study of newborns presenting CCAM in a tertiary care hospital in Madrid, Spain. Results: Seven patients were found. All were full term, normal weight births. Two patients showed respiratory distress at birth. Two chest x-rays were normal. CT scans showed three clear CCAM lesions, four suggestive of hybrid lesions. All were referred to medical centers with pediatric surgery for followup. Discussion: At birth, this pathology may be asymptomatic and appear as a chest x-ray finding. Long term management is complicated by infection and malignization. Conservative treatment of asymptomatic patients includes regular follow up of lesions. Surgical treatment is reserved for symptomatic or complicated patients. Conclusions: Prenatal suspicion of CCAM is important since clinical exam and radiology may be normal in neonatal period. Chest CT scans are important in confirming diagnosis and determining future surgery. More studies are necessary for the proper diagnosis and management of this disorder.

Introducción: La malformación adenomatoidea quística (MAQ) es una entidad congénita poco frecuente debida a una alteración en el desarrollo alveolar pulmonar. Pacientes y Método: Se realizó un estudio descriptivo y retrospectivo de los recién nacidos con el diagnóstico prenatal de MAQ durante 6 años en un hospital terciario de Madrid (España). Resultados: Se encontraron un total de siete pacientes. Todos fueron recién nacidos a término de peso adecuado. Dos pacientes presentaron distress respiratorio al nacimiento. Dos radiografías de tórax fueron normales. En la tomografía axial (TAC), tres lesiones fueron MAQ y cuatro fueron su-gerentes de lesión híbrida. Todos se derivaron a centro con cirugía pediátrica para seguimiento. Discusión: Al nacimiento, esta patología puede permanecer asintomática y ser un hallazgo casual en una radiografía torácica. A largo plazo el riesgo de infección y malignización complican el manejo. El tratamiento conservador, que se dirige a pacientes asintomáticos, obliga a realizar controles seriados de las lesiones. El tratamiento quirúrgico se reserva para los pacientes con sintomatología o complicaciones postnatales. Conclusiones: El diagnóstico de sospecha prenatal de MAQ es fundamental dado que la clínica y radiología pueden ser normales en el período neonatal. Se debe realizar TAC torácico para confirmar la lesión y valorar futura cirugía. Se necesitan más estudios sobre el correcto diagnóstico y manejo de esta patología.

Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma?

BACKGROUND: The relationship between congenital cystic adenomatoid malformation (CCAM) and pleuropulmonary blastoma (PPB), whether causal, correlational, or coincidental, remains controversial. There is a lack of consensus as to the optimal treatment of patients with asymptomatic CCAM. METHOD: We reviewed all cases of CCAM and PPB seen at our institution from 1999 to 2008. Institutional Research Ethics Board approval was obtained. The incidence of CCAM and PPB, respectively, was calculated based on birth numbers during the study period. RESULTS: Seventy-four CCAMs were resected over the study period in 129 children diagnosed with CCAM. Five PPBs were diagnosed during the study period. Three of the 5 PPB cases were initially diagnosed as CCAMs. These PPBs were not clinically or radiologically distinguishable from CCAMs. In our referral area, the incidence of CCAM was 1 in 12,000; and the incidence of PPB was 1 in 250,000 live births. The mortality rate for PPB in this cohort was 20%. CONCLUSION: Asymptomatic cystic lung malformations represent a therapeutic dilemma. In this cohort, the incidence of PPB among apparently benign lung lesions was 4%. No clinical or radiological markers differentiated benign CCAMs from PPBs. Our experience provides further justification for resection of all CCAMs. This should be discussed with parents until CCAMs and PPBs can be clearly distinguished preoperatively.

Congenital cystic adenomatoid malformation of lung type 1.

Congenital cystic adenomatoid malformation, a rare developmental, hamartomatous abnormality of the lung, usually is unilateral, is localized, and presents in early infancy. Delayed occurrence in older children and multilobar involvement are rare. We describe a case of congenital cystic adenomatoid malformation type 1 with multilobar involvement, associated emphysema, and coexistent tracheobronchopathia osteochondroplastica in an adolescent girl for whom the correct diagnosis was achieved only on histologic examination. The importance of an accurate diagnosis of this entity enables proper subtyping, management to minimize the risk of infections and malignancy, and exclusion of associated malformations.

Lack of consensus among Canadian pediatric surgeons regarding the management of congenital cystic adenomatoid malformation of the lung.

PURPOSE: Review of the literature suggests that there is lack of consensus regarding the management of antenatally diagnosed congenital cystic adenomatoid malformation of the lung (CCAM) that is asymptomatic at birth. This study aims to describe the variability among Canadian pediatric surgeons in how this pathologic finding is managed. METHODS: Surveys were sent to all practicing Canadian members of Canadian Association of Pediatric Surgeons. Responders were asked to state whether they recommend resection or nonoperative management and to describe the follow-up imaging type and frequency used. RESULTS: A 69% response rate was obtained. There was no consistency regarding the imaging modality used to detect asymptomatic CCAM. Sixty-seven percent of responders recommend resection of persistent but asymptomatic CCAM; there was neither consensus with regard to age at which resection is performed (2-18 months) nor technical considerations at resection (61% open, 83% lobectomy). Among the responders who do not recommend resection, the frequency of follow-up was variable (every 3 months to every year), as were the imaging modality used and the length of follow-up (3 years to indefinitely); 80% of neonates in whom nonoperative management was recommended initially ultimately underwent resection. CONCLUSIONS: Lack of consensus among Canadian pediatric surgeons, and even within institutions, regarding the management of antenatally diagnosed CCAM in the asymptomatic neonate, is demonstrated. This clearly highlights the need for prospective studies.

Congenital malformations of the lung.

Congenital malformations of the lung are rare and vary widely in their presentation and severity. The most common manifestation of the congenital cystic disease of the lung at newborn and early infancy is respiratory distress. Later on in life, cysts usually lose this compressive character and may remain asymptomatic until infection occurs, while producing cough, dyspnea and thoracic pain. The purpose of this study is to review authors institutional experience of congenital cystic lung disease, with specific reference to diagnosis, treatment, as well as outcome, furthermore, to present some cases with unusual clinical manifestations.

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.

Outcome predictors for infants with cystic lung disease.

BACKGROUND/PURPOSE: This study aimed at identifying characteristic features indicating congenital cystic adenomatoid malformation of the lung (CCAM) and evaluating the outcome predictors to identify prenatally subgroups of fetuses with significantly different probabilities of mortality or severe respiratory difficulty. METHODS: Twenty-eight neonates who had undergone antenatal evaluation for cystic lung disease (CLD) were reviewed retrospectively. The patients were divided into 3 groups according to the severity of their clinical course; mild (n = 7), moderate (n = 13), and severe (n = 8). Ultrasonographic findings in the fetus and their pulmonary lesion were evaluated. The normal lung to thorax transverse area ratio (L/T) was measured by ultrasonography. RESULTS: High echogenicity of the lesion throughout pregnancy and polyhydramnios were frequently seen in CCAM. All of the patients with other CLD showed isoechogenicity at the end of pregnancy. All patients in the severe group had both polyhydramnios and fetal hydrops. L/T was increased in mild and moderate groups, whereas no patient in the severe group had an increase in L/T at the final measurement. Each value of final L/T in the severe group was less than 0.25. CONCLUSIONS: The subgroup of fetuses with an increased probability of mortality or severe respiratory difficulty could be predicted from the combination of polyhydramnios, fetal hydrops, and a final L/T value of less than 0.25.

Congenital cystic adenomatoid malformation: accuracy of prenatal diagnosis, prevalence and outcome in a general population.

OBJECTIVES: Most available data regarding accuracy of prenatal diagnosis, prevalence and outcome of congenital cystic adenomatoid malformation (CCAM) are derived largely from tertiary referral centres and may not reflect general population rates. We aimed to describe the accuracy of prenatal diagnosis, ascertain the population prevalence and post-natal outcome for cases of suspected CCAM. METHODS: Retrospective collection of prenatal and paediatric data for cases of suspected CCAM notified to the Trent Congenital Anomalies Register 1997 to 2001. RESULTS: Thirty-seven cases of CCAM were suspected prenatally. Twenty-one cases were confirmed post-natally as having a CCAM (positive predictive value 57%). Eighteen of the 21 cases were delivered at term as live births, 15 of which have undergone successful surgery to date. Thirteen of the 37 cases had apparently resolved by delivery. Three further cases were subsequently found to be cases of lung sequestration or lobar emphysema. Five cases of CCAM were detected after delivery (sensitivity of prenatal detection 81%). The population prevalence at delivery was 9.0 per 1,00,000 total births. Five confirmed cases of CCAM developed hydrops, three required in utero intervention and delivered as live births at term, one was terminated and one died in utero. The overall mortality in the confirmed cases of CCAM was 23% of which the majority were terminations of pregnancy. CONCLUSIONS: Problems of diagnostic accuracy and apparent resolution of CCAM render counselling difficult, although our data suggest that the prognosis is better than others have reported. Confirmation of the diagnosis in the neonatal period is vital in order to obtain the true population prevalence figures and to interpret outcome data.

Management of asymptomatic neonatal cystic adenomatoid malformations.

BACKGROUND/PURPOSE: Although much is known about the prenatal course of cystic adenomatoid malformations (CCAM), the postnatal course of asymptomatic lesion is less well documented. The authors studied the pre- and postnatal course and treatment of asymptomatic CCAM. METHODS: The authors reviewed the files of all patients referred to Necker-Enfants Malades hospital with an antenatal diagnosis of CCAM and asymptomatic at birth. RESULTS: Files of 29 patients were studied. The first x-ray film was considered normal in 12 cases (41.3%). Computed tomography was normal in 4 cases and showed cystic lung malformations in the other patients. Postnatally, clinical manifestations occurred in 3 patients (10.3%). CCAM vanished in 6 cases. Surgical resection of CCAM was performed in 17 cases (58.6%). All the patients currently are asymptomatic. CONCLUSIONS: CCAM can shrink or vanish during pregnancy and antenatal ultrasound findings are not predictive of the postnatal course. Thus, all infants with prenatal diagnosis of CCAM require postnatal evaluation. Normal radiographic findings at birth do not rule out CCAM persistence on CT. The treatment of asymptomatic CCAM is controversial. Surgery may be advocated because of the low morbidity and the prevention of late complications, above all, cancer. The surgical indications of small (<3 cm) and asymptomatic lesions should be discussed on a case-by-case basis with the parents.