Management Of Congenital Pulmonary Airway Malformation Research Articles

Fetal Congenital Pulmonary Airway Malformation: The Role of an Objective Measurement of Cardiomediastinal Shift.

To examine the relationship between cardiomediastinal shift angle (CMSA) and adverse perinatal outcomes and hydrops in cases of congenital pulmonary airway malformation (CPAM). This retrospective study evaluated CPAM cases referred to our institution from 2008 to 2015. The primary outcome was a composite score for adverse perinatal outcome. CMSA was measured for each case and evaluated for its association with the primary outcome. The prediction accuracy of CMSA for adverse perinatal outcome was assessed using receiver operator characteristic (ROC) curves. Eighteen (21.2%) of the 85 cases experienced an adverse perinatal outcome. Increases in CMSA were associated with adverse perinatal outcomes and hydrops in bivariate analyses. Adjusted analyses found each 10-degree increase in CMSA to be associated with increased odds of an adverse perinatal outcome (adjusted odds ratio [aOR] 2.2, 95% confidence interval [CI]: 1.4-3.3) and hydrops (aOR 3.0, 95% CI: 1.5-6.1). CMSA performed well and was comparable to CPAM volume ratio in predicting adverse perinatal outcomes (area under the curve 0.81 and 0.84, respectively). We describe a novel measurement of mediastinal shift in cases of CPAM and its relationship with adverse perinatal outcomes and hydrops. These findings may shape the evaluation and management of CPAMs, improve our understanding of their prognosis, and influence patient counseling.

Lung Ultrasound Findings in Congenital Pulmonary Airway Malformation.

Congenital pulmonary airway malformation (CPAM) is a group of rare congenital malformations of the lung and airways. Lung ultrasound (LU) is increasingly used to diagnose neonatal respiratory diseases since it is quick, easy to learn, and radiation-free, but no formal data exist for congenital lung malformations. We aimed to describe LU findings in CPAM neonates needing neonatal intensive care unit (NICU) admission and to compare them with a control population. A retrospective review of CPAM cases from three tertiary academic NICUs over 3 years (2014-2016) identified five patients with CPAM who had undergone LU examination. LU was compared with chest radiograms and computed tomography (CT) scans that were used as references. CPAM lesions were easily identified and corresponded well with CT scans; they varied from a single large cystic lesion, multiple hypoechoic lesions, and/or consolidation. The first two LU findings have not been described in other respiratory conditions and were not found in controls. We provide the first description of LU findings in neonates with CPAM. LU may be used to confirm antenatally diagnosed CPAM and to suspect CPAM in infants with respiratory distress if cystic lung lesions are revealed. Further studies are necessary to define the place of LU in the management of CPAM.

Erratum to: Current Management of Congenital Pulmonary Airway Malformations: A "European Pediatric Surgeons' Association" Survey.

Aim To define current management of congenital pulmonary airway malformation (CPAM). Methods A total of 181 European Pediatric Surgeons' Association members (91% senior) from 48 countries completed an online questionnaire. Main Results Prenatal: 93% respondents work in centers with prenatal diagnosis facilities, and 27% in centers offering in utero surgery. Prenatal counseling is performed by 86% respondents, 22% of whom see >10 cases per year. Risk of single pre-/postnatal complications is deemed low (<5%) by more than 60% of respondents. Eighty-six percent respondents do not offer pregnancy termination for prenatally diagnosed CPAM. Fetal hydrops is the most frequent indication for termination (87%), followed by parental willingness (52%). Prenatal surgery is an option for 44% respondents, preferring thoracoamniotic shunt (82%). Postnatal: 75% respondents operate on asymptomatic patients, 18% before 6 months of age, 62% between 6 and 12 months of age, and 20% after 12 months of age. Risk of infection (86%), cancer (63%), and symptoms development (62%) are indications for surgery in asymptomatic CPAM. Sixty-three percent prefer a thoracotomy. Lobectomy is the preferred procedure (58% respondents). Motivations against surgery include lesion <1 cm (64%), risk of postoperative complications (37%), and lack of evidence favoring surgery (27%). Seventeen percent respondents have seen at least one patient with CPAM with lung cancer, in 89% of the cases within the CPAM. Of all the respondents, 83% and 22% offered dedicated follow-up and genetic screening, respectively. Conclusion Current pre- and postnatal management of CPAM lacks uniformity, particularly for surgical indication, timing, and approach. Efforts should be made toward standardization. Risk of CPAM-associated cancer is not clear.

A REVIEW OF POSTNATAL MANAGEMENT OF CONGENITAL PULMONARY AIRWAY MALFORMATIONS

Congenital cystic adenomatoid malformations of the lung (CCAMs) were originally described by Ch'In and Tang in 1949 as rare lung lesions occurring in premature or stillborn infants with anasarca. In 1977 Stocker et al divided CCAM into three types based on their clinical and pathological features. The nomenclature has since changed to congenital pulmonary airway malformations (CPAMs) to reflect the possible absence of cystic changes and the probable underlying pathogenesis of different types. CPAMs are relatively rare congenital abnormalities with a reported incidence varying from 1 in 10,000 to 1 in 35,000. There is a slight male predominance, but no racial predilection has been noted. This review will outline the current nomenclature and features of different types of CPAMs, highlight possible mechanisms underlying the development of CPAMs, review current antenatal imaging modalities and interventions, address the debate surrounding the postnatal management of CPAMs, and suggest areas for future research.

Perinatale aanpak bij congenitale luchtwegmalformaties

AbstractPerinatal diagnosis and management ofcongenital pulmonary airway malformations This review on congenital pulmonary airwaymalformations intends to illustrate the positiveimpact of prenatal diagnosis on their postnatalmanagement. For the gynaecologist these fetalmalformations present as uni- or bilateral hyper-dense or so-called „bright” lungs on fetal ultra-sound. The underlying cause for bilateral hyper-dense fetal lungs is mostly related to a CongenitalHigh Airway Obstruction Syndrome (CHAOS)and exceptionally to a bilateral form of Congeni-tal Cystic Adenomatoid Malformation (CCAM).Outcome is globally poor, although specifictherapeutic procedures may be considered forCHAOS if a reasonable chance for integralrepair of the airways exists. On the other hand,overall outcome for most of the unilateral typesof congenital pulmonary airway malformationshould be considered as fairly good to excellent.Therefore reticence upon termination of preg-nancy is a major message. It is also importantto underscore that in utero regression of theseunilateral bright lungs is rather the rule and notthe exception. Still, these „vanishing lesions”should be thoroughly evaluated after birth.The newborn infant with congenital pulmo-nary airway malformation is often asymptomatic