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• W2042446908 abstract "Congenital lobar emphysema (CLE) is a malformation of the lung that is usually diagnosed postnatally. It is characterized by air trapping and/or overdistension of lung segments and lobes. In some cases there is respiratory distress, mediastinal shift and wheezing due to spontaneous overinflation of the affected areas. Prenatal diagnosis has been made in a number of cases, and such fetuses present with a congenital echogenic mass, including mediastinal shift and displacement of the heart, resulting in compression of the contralateral lung. Both macro- and microcystic lesions have been reported. While the association with increased nuchal translucency (NT) has been made for lesions that lead to high intrathoracic pressure such as congenital diaphragmatic hernia (CDH)1, Fryns' syndrome2 and congenital cystic adenomatoid malformation (CCAM)3, this is the first report of the association between CLE and increased NT. A 32-year-old primigravida, at 12 + 2 weeks, was referred to our fetal medicine unit because of an increased NT of 4.5 mm. Ultrasound examination was performed using an ATL HDI 5000 machine (software version HDI® 5000 4252-0901-05 185.13, copyright 1997, Advanced Technology Laboratories, Solingen, Germany) with a 5-MHz transabdominal probe and this confirmed the findings. The results of first-trimester biochemistry (free beta human chorionic gonadotropin and pregnancy-associated plasma protein-A) were normal. Chorionic villus sampling showed a normal karyotype. A follow-up scan at 20 weeks' gestation was performed, which showed an echogenic mass in the left upper lobe of the lung. The increased NT had resolved. Biometry was within normal ranges. Sagittal and transverse sections of the fetal thorax showed bright lung tissue of microcystic appearance (Figure 1). No macrocystic lesions, mediastinal shift or associated anomalies were observed and there was no sign of fetal compromise, features which together raised the suspicion of CCAM Type III4. Serial follow-up scans to exclude hydrops or mediastinal shift were normal. Spontaneous delivery occurred at term. Postnatal imaging using chest X-rays and both magnetic resonance imaging and computed tomography scans were also suggestive of CCAM. Histological studies, performed for suspected malignancy, were however consistent with the diagnosis of CLE. Fetal ultrasound scan at 20 weeks' gestation. (a) Sagittal and (b) transverse sections of the fetal thorax showing bright lung tissue with a microcystic appearance resembling that associated with congenital cystic adenomatoid malformation Type III. The differential diagnosis of a congenital chest mass includes CCAM, CLE, pulmonary sequestration and bronchogenic cysts5. Histological studies of lung masses obtained from postnatal thoracic surgery have shown that there may be more than one diagnosis attributed to the same lesion5. This has led to the assumption that the above-mentioned four diagnoses may in fact be the same interrelated abnormality of a hybrid lung lesion6, 7. The presumed mechanism leading to increased NT is increased superior mediastinal compression, which is commonly associated with fetal structural defects such as CDH, Fryns' syndrome and CCAM, or narrow chest in skeletal dysplasias such as osteogenesis Type II, thanatophoric dysplasia or asphyxiating thoracic dystrophy. In CDH, increased NT is present in 37% (7/19) of cases1. These include about 83% of the cases that result in neonatal death due to pulmonary hypoplasia and about 22% of the survivors1. In fetuses with CDH and increased NT, the intrathoracic herniation of the abdominal viscera may occur in the first trimester and prolonged compression of the lungs causes pulmonary hypoplasia. The same mechanism may account for the poor prognosis in Fryns' syndrome, characterized by diaphragmatic hernia, digital defects and short, webbed neck. One case demonstrated a NT of >6.4 mm in the first trimester3. CCAM is a pulmonary lesion involving large or small cysts4, which has been described in association with an increased NT of >6.4 mm in the first trimester3. The underlying mechanism may be superior mediastinal compression, and in CLE the mechanism may be identical. A common feature in skeletal dysplasias is thoracic constriction or narrow thorax, and death is secondary to pulmonary hypoplasia. In osteogenesis Type II, the ribs and limbs are short with multiple fractures and death follows respiratory failure. Two cases in the first trimester both demonstrated an increased NT of 3.5–4.4 mm3. In thanatophoric dysplasia, the thorax is narrow and there is severe limb shortening and a prominent forehead. One case in the first trimester was reported with an increased NT of 3.0 mm3. In asphyxiating thoracic dystrophy, there is a narrow chest and rhizomelic limb shortening and one reported case in the first trimester demonstrated an increased NT of 5.8 mm8. The finding of congenital lung masses following identification of increased NT in the first trimester requires follow-up to monitor the development of both hydrops, which is the main prognostic factor, and malignancy. Superior mediastinal compression leading to increased NT may be the consequence of both CCAM and CLE. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article. C. S. von Kaisenberg*, S. Engler , T. Ankermann , * Department of Obstetrics and Gynecology, University of Schleswig-Holstein, Campus Kiel, Michaelisstrasse 16, 24105 Kiel, Germany, Department of Surgery, University of Schleswig-Holstein, Campus Kiel, Michaelisstrasse 16, 24105 Kiel, Germany, Department of General Pediatrics, University of Schleswig-Holstein, Campus Kiel, Michaelisstrasse 16, 24105 Kiel, Germany" @default.
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• W2042446908 title "Congenital lobar emphysema and increased nuchal translucency" @default.
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