Successful treatment for a severe case of fetal lung disorder CPAM
A successful treatment has now been conducted for the fetal lung disorder Congenital Pulmonary Airway Malformattion (CPAM), also known as Congenital Cystic Adenomatoid Malformation (CCAM). By performing a series of prenatal and postnatal treatments, the infant was discharged from the hospital without subsequent complications. This series of treatments potentially provides another option for families with unborn babies with the same disorder.
We describe a case of a large, predominantly microcystic CCAM in a hydropic fetus treated successfully with direct interstitial injection of a sclerosant agent (3% sodium tetradecyl sulfate) at 23+3 weeks gestation, after multiple failed courses of steroids. Elective thoracoscopic right lower lobectomy was performed at 1 year of life and there have been no respiratory or other medical morbidities since. A literature review of fetal lung masses treated with sclerosants antenatally reveals that sclerotherapy may represent a novel treatment option for large hydropic microcystic CCAMs, which are unresponsive to corticosteroids...
We report for the first time one case of pulmonary glial heterotopia occured in a one-month baby free from any malformation. She has the particularity of being born from monozygotic monochorionic twin pregnancy where her anencephalic exencephalic sister suffered a medical termination of pregnancy. She presented neonatal respiratory distress, which recurred one month later. Chest X-ray revealed bilateral cystic pulmonary lesions mainly located in the right lung. Given the suspicion of congenital cystic adenomatoid malformation (CCAM), she underwent an upper and a lower right lung lobectomy at four months old. The pathologic...
Conclusion: CLO in adults is rare and is associated with a broad spectrum of symptoms. Precise anamnesis and radiological findings confirm the diagnosis. Surgical resection is the treatment of choice in symptomatic patients.
Conclusions: Markers of evolution of CLM are difficult to homogenize. Clinical registries may help design more comprehensive care.
Ultrasound in Obstetrics&Gynecology,Volume 52, Issue S1, Page 56-57, October 2018.
We report two cases of peripheral bronchial atresia diagnosed by prenatal ultrasonography (US) and magnetic resonance imaging (MRI). Evaluating an enlarged lung mass that is homogeneously hyperechoic on US and hyperintense on T2-weighted MRI can help in determining whether bronchial atresia is present. Proximal type is suggested when a dilated main bronchus is observed as a tubule structure of an involved lung hilum. In our cases, T2-weighted MRI revealed homogeneously hyperintense lung lesion with decreased signal intensity of adjacent lobe, flattening diaphragm, and mediastinal shift. Dilatation of the main bronchus was ...
Conclusion: Pulmonary pathology is the most common finding in fetal deaths with aspiration pneumonitis being the most common avoidable cause. Accurate fetal autopsy along with clinical data are important in evaluating fetal deaths and can help in reduction of unexplained stillbirths. PMID: 30303136 [PubMed - in process]
The purpose of this study was to clarify the relationship between congenital pulmonary airway malformation volume ratio (CVR) of bronchial atresia (BA), CVR of congenital cystic adenomatoid malformations (CCAM), and time of surgery after birth.
Respirology Case Reports,Volume 6, Issue 8, November 2018.
ConclusionsPreoperative 3D-CT simulation and intraoperative navigation for pediatric minimally invasive surgery were useful and feasible.