Congenital Cystic Adenomatoid Malformation in the Fetus: A Hypothesis of Its Development

Kulwa, Ema; Tharakan, Tessie; Baxi, Laxmi

doi:10.1159/000086836

Cited by 7 publications

(5 citation statements)

References 23 publications

(15 reference statements)

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“…One study has attributed their development to the effect of alcohol consumption on retinoic acid levels [8] . However, recent studies have indicated that bronchial atresia plays a role in the etiology of microcystic maldevelopment present in the small cyst-type CCAM [1] .…”

Section: Introductionmentioning

confidence: 99%

Prenatal Course and Postnatal Management of Peripheral Bronchial Atresia: Association with Congenital Cystic Adenomatoid Malformation of the Lung

et al. 2008

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Objective: Peripheral bronchial atresia (PBA), a newly identified fetal lung lesion, is often asymptomatic and managed nonoperatively. However, recent studies suggest that bronchial atresia plays a role in the etiology of microcystic maldevelopment present in congenital cystic adenomatoid malformations (CCAM) which require resection to decrease the risk of infection, pneumothorax and malignant degeneration. The purpose of this study was to evaluate the prenatal radiographic and postnatal computed tomography (CT) scan/pathology findings with attention to the pathologic diagnosis of microcystic maldevelopment in infants with the presumptive diagnosis of PBA. Methods: A retrospective review of prenatal and postnatal records of patients diagnosed with fetal lung lesions was performed. Two groups of patients were identified: (1) patients diagnosed with PBA on postnatal CT scan (n = 16), and (2) patients with the pathologically confirmed diagnosis of PBA independent of postnatal CT findings (n = 23). Results: Prenatal ultrasound diagnosis of these lesions included CCAMs, hybrid lesions, bronchopulmonary sequestrations and bronchial atresia. Eleven of the 16 patients in group 1 with the postnatal radiologic diagnosis of PBA underwent surgical resection, 6 of which were found to have microcystic changes consistent with CCAM. Evaluation of the 23 patients in group 2 with pathologically confirmed PBA identified 65% that had evidence of microcystic maldevelopment consistent with the small cyst type of CCAM. Conclusion: Radiographically diagnosed PBA as well as pathologically confirmed PBA is frequently associated with microcystic changes consistent with the small cyst type of CCAM. Thus, operative management should be considered for PBA to decrease CCAM-associated risks.

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Section: Introductionmentioning

confidence: 99%

Prenatal Course and Postnatal Management of Peripheral Bronchial Atresia: Association with Congenital Cystic Adenomatoid Malformation of the Lung

et al. 2008

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“…Knowledge of the regulation of WT1 expression is scant however an enhancer located 4Kb upstream of the transcription start site in zebra fish is highly conserved and is responsive to the retinoic acid receptor family of transcription factors [18]. Interestingly in humans, retinoic acid deficiency, for example due to high alcohol intake during pregnancy, may predispose to CPAM [19]. In both the abnormal fetuses we describe, normal testes were present.…”

Section: Discussionmentioning

confidence: 99%

Possible Role of WT1 in a Human Fetus with Evolving Bronchial Atresia, Pulmonary Malformation and Renal Agenesis

et al. 2012

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The association of peripheral bronchial atresia and congenital pulmonary airway malformation (CPAM) has recently been recognised, but the pathology of the lesions evolving together has not been described. We present autopsy findings in a 20 week fetus showing areas of peripheral bronchial destruction and airway malformation consistent with developing CPAM in the right lung supporting a causal relationship between these lesions. This fetus also had congenital heart defect, bilateral renal agenesis and syndactyly. We identified another fetus from our autopsy files, with bilateral renal agenesis, similar right sided pulmonary malformation and cardiac defects. Similar bilateral renal agenesis and defects of the heart and lungs are found in wt1(-/-) mice and we have investigated the expression of WT1 in these fetuses. We hypothesise that the cardiac, liver, renal and possibly lung lesions in these two cases may arise due to mesenchymal defects consequent to WT1 misexpression and discuss evidence for this from the scientific literature. We used immunoperoxidase stains to analyse WT1 expression in autopsy hepatic tissue in both fetuses. We also investigated the expression of α-smooth muscle actin (α-SMA), a marker of activated hepatic stellate cells/myofibroblasts, and desmin in hepatic mesenchyme and compare these findings with control fetuses, without congenital malformations. We found reduced WT1 expression in hepatic mesothelium in both fetuses with malformations. There was also increased expression of α-SMA in liver perisinusoidal cells, as seen in the wt1(-/-) mouse model. We therefore propose that abnormality of WT1 signalling may be an underlying factor, as WT1 is expressed in coelomic lining cells from which mesenchyme is derived in many organs.

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“…Pneumonectomy is the most commonly used surgical treatment for patients with CPAM, and it has a high success rate 11,22) . A previous study that assessed 1,120 infants demonstrated that the thoracoscopic approach induced fewer complications and resulted in shorter hospitalization 23) . In neonates, thoracoscopic intervention may be difficult because of the limited operative space within their small bodies 24) .…”

Section: Surgery and Complicationsmentioning

confidence: 99%

“…Congenital pulmonary airway malformation (CPAM) is a de velopmental anomaly of the lungs that occurs in 1 in 10,000 to 35,000 newborn infants. It originates from the bronchopulmonary foregut and is caused by delayed pulmonary development at 4 to 7 weeks of gestation 1) . CPAM accounts for 95% of congenital cystic pulmonary lesions, most commonly found via prenatal screening at 18 to 20 weeks of gestation 2) .…”

Section: Introductionmentioning

confidence: 99%

Evaluation of Newborn Infants with Prenatally Diagnosed Congenital Pulmonary Airway Malformation: A Single-Center Experience

Lim¹,

Han²,

Shin³

et al. 2019

Neonatal Med

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Purpose: Congenital pulmonary airway malformation (CPAM)-a rare developmental anomaly-affects the lower respiratory tract in newborns. By comparing the reliability of diagnostic tools and identifying predictive factors for symptoms, we provide com prehensive clinical data for the proper management of CPAM. Methods: We reviewed the medical records of 66 patients with prenatally diagnosed CPAM delivered at Severance Children's Hospital between January 2005 and July 2017. Results: We enrolled 33 boys and 33 girls. Their mean gestational age and birth weight were 38.8 weeks and 3,050 g, respectively. Prenatal ultrasonography and postnatal radiography, lung ultrasonography, and chest computed tomography (CT) showed inconsistent findings. Chest CT showed superior sensitivity (100%) and positive pre dictive value (90%). Among the 66 patients, 59 had postnatally confirmed CPAM, three had pulmonary sequestration, one had cystic teratoma, and one had a normal lung. Of the 59 patients with CPAM, 21 (35%; mean age, 23.4 months) underwent surgery, including 15 who underwent videoassisted thoracoscopy. Twentyfive and 12 patients exhibited respiratory symptoms at birth and during infancy, respectively. Apgar scores and mediastinal shift on radiography were significantly associated with respiratory symptoms at birth. However, none of the factors could predict respiratory symptoms during infancy. Conclusion: Radiography or ultrasonography combined with chest CT can confirm an unclear or inconsistent lesion. Apgar scores and mediastinal shift on radiography can predict respiratory symptoms at birth. However, symptoms during infancy are not associated with prenatal and postnatal factors. Chest CT combined with periodic symptom monitoring is important for diagnosing and managing patients with pre natally diagnosed CPAM and to guide appropriate timing of surgery.

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Congenital Cystic Adenomatoid Malformation in the Fetus: A Hypothesis of Its Development

Cited by 7 publications

References 23 publications

Prenatal Course and Postnatal Management of Peripheral Bronchial Atresia: Association with Congenital Cystic Adenomatoid Malformation of the Lung

Prenatal Course and Postnatal Management of Peripheral Bronchial Atresia: Association with Congenital Cystic Adenomatoid Malformation of the Lung

Possible Role of WT1 in a Human Fetus with Evolving Bronchial Atresia, Pulmonary Malformation and Renal Agenesis

Evaluation of Newborn Infants with Prenatally Diagnosed Congenital Pulmonary Airway Malformation: A Single-Center Experience

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