Abstract:Pulmonary agenesis is a rare congenital anomaly, estimated to complicate around 1 per 15,000 pregnancies, in which there is complete absence or severe hypoplasia of one or both lungs, frequently associated with other abnormalities. A prospective prenatal diagnosis is a challenge, and a substantial proportion of cases are diagnosed by fetal magnetic resonance imaging, postnatal computed tomography, or postmortem. Thus, there are only a few reported cases of prenatal diagnosis in the literature. We report the pr… Show more
“…In recent years, progress achieved in obstetrical imaging has made early antenatal diagnosis possible with largest case series of 18 cases reported by Zhang et al 17 Cases have also been diagnosed antenatally as early as first, 18 second, [19][20][21] and third 22 trimester of pregnancy. In our study, cases 1 and 2 were diagnosed at the second and third trimester, respectively.…”
Pulmonary agenesis is a rare congenital malformation of lung development defined as complete absence of lung tissues, bronchi, and pulmonary vessels; it may be uni- or bilateral. The right-sided form carries the poorest prognosis due to severity of co-existent anomalies. Its diagnostic circumstances are variables: first reported cases were diagnosed at autopsy, but early postnatal as well as fortuitous discovery have been reported. In recent years, progress in obstetrical imaging has made antenatal diagnosis possible so that fetal ultrasound and MRI allow early diagnosis and refinement by permitting the elimination of differential diagnoses (diaphragmatic hernia, cystic adenomatoid malformation of the lung, giant lobar emphysema, and situs inversus). This anomaly is compatible with normal life provided co-existent malformations are thoroughly investigated and managed in a multidisciplinary setting. We report four cases of lung agenesis two of which were diagnosed antenatally at 23rd and 30th weeks of gestation respectively. Our aim is to describe the circumstances having led to diagnosis and report both follow-up and outcome of our patients.
“…In recent years, progress achieved in obstetrical imaging has made early antenatal diagnosis possible with largest case series of 18 cases reported by Zhang et al 17 Cases have also been diagnosed antenatally as early as first, 18 second, [19][20][21] and third 22 trimester of pregnancy. In our study, cases 1 and 2 were diagnosed at the second and third trimester, respectively.…”
Pulmonary agenesis is a rare congenital malformation of lung development defined as complete absence of lung tissues, bronchi, and pulmonary vessels; it may be uni- or bilateral. The right-sided form carries the poorest prognosis due to severity of co-existent anomalies. Its diagnostic circumstances are variables: first reported cases were diagnosed at autopsy, but early postnatal as well as fortuitous discovery have been reported. In recent years, progress in obstetrical imaging has made antenatal diagnosis possible so that fetal ultrasound and MRI allow early diagnosis and refinement by permitting the elimination of differential diagnoses (diaphragmatic hernia, cystic adenomatoid malformation of the lung, giant lobar emphysema, and situs inversus). This anomaly is compatible with normal life provided co-existent malformations are thoroughly investigated and managed in a multidisciplinary setting. We report four cases of lung agenesis two of which were diagnosed antenatally at 23rd and 30th weeks of gestation respectively. Our aim is to describe the circumstances having led to diagnosis and report both follow-up and outcome of our patients.
“…In the review by Maltz and Nadas (1968), cardiac and vascular defects were noted in 23% of the 156 reported patients [17]. A more recent review [18] by Meller et al (2012) suggested that left lung agenesis was more frequently isolated than right lung agenesis. They found that only 1 in 6 cases of left lung agenesis had a cardiovascular anomaly, whereas 19 of 25 cases with right-sided presented with a cardiovascular anomaly, the most common of which was scimitar syndrome (15 cases).…”
BackgroundFetal unilateral lung agenesis, complicated with cardiac shifting, is a rare anomaly, the diagnosis of which remains a challenge for many sonographers in routine screening programs. The present study describes a systematic approach for the diagnosis of fetal unilateral lung agenesis and cardiac malpositions in routine prenatal screening.MethodsA total of 18 cases of fetal unilateral lung agenesis complicated with cardiac malposition were reviewed. A systematic method was proposed to identify the fetal left side and right side according to the fetal head position and posture by acquiring a long axis and transverse view of the fetus. Fetal unilateral lung agenesis was diagnosed by evaluation of the ipsilateral pulmonary artery. The diagnosis was confirmed by postnatal echocardiography, digital radiology, and computed tomography after birth or by autopsy findings.ResultsThe left-sided fetal heart with the cardiac apex rotating to the left and posterior were confirmed in all 7 left lung agenesis cases, while the rightward shifting of the fetal heart together with the cardiac axis deviating to the right were confirmed in all 11 cases of right lung agenesis. The disappearance of the ipsilateral pulmonary artery was confirmed in all 18 cases of unilateral lung agenesis. Cardiac anomalies were present in a total of 7 of the18 cases of lung agenesis with 4 of 7 in cases of left lung agenesis and 3 of 11 in cases of right agenesis.ConclusionsThe systematic approach introduced in the current report is helpful in the diagnosis of fetal unilateral lung agenesis complicated with cardiac malposition. The information provided by this study may be helpful to better understand unilateral lung agenesis anatomically and to facilitate its potential examination.
“…44,4850 It is important to note that cardiovascular abnormalities are more frequent in right pulmonary agenesis than in left pulmonary agenesis (76% and 17% respectively). 46 Pulmonary agenesis is not associated with an increased incidence of the chromosomal abnormalities. 44,47 The prognostic in right pulmonary agenesis is poorer than in left pulmonary agenesis; this is explained by the important shift of the mediastinum and by a higher frequency of associated pathologies in right pulmonary agenesis.…”
Section: Dsjuogmentioning
confidence: 94%
“…47,48 The mediastinal shift will bring the problem of a differential diagnosis with the more frequent pathologies, congenital diaphragmatic hernia, CCAM, BPS, or less common malformations, such as pulmonary emphy sema, mediastinal teratoma, neuroblastoma, hetero taxy syndrome. 46 …”
Section: Right Pulmonary Agenesismentioning
confidence: 97%
“…44,47 The prognostic in right pulmonary agenesis is poorer than in left pulmonary agenesis; this is explained by the important shift of the mediastinum and by a higher frequency of associated pathologies in right pulmonary agenesis. 44,46 We diagnosed a case with right pulmonary agenesis at 24 weeks. The first sonographic sign was the shift of the mediastinum toward right and the fetal dextrocardia in the absence of a thoracic tumor formation.…”
Purpose:We present our experience in prenatal diagnosis and obstetrical management of congenital lung malformations (CLM).
Materials and methods:The diagnosis of CLM was performed during routine second and third trimester fetal morphology assess ment. The extent of the disease was established accor ding to the type, localization and size of the pulmonary lesion and the presence of fetal complications (mediastinal shift, fetal hydrops, hydramnios). Termination of pregnancy (TOP) was indicated in cases associated with extrapulmonary anomalies, untreatable cases and fetal hydrops. After delivery a pulmonary Xray and surgical examination was performed in all newborns.
Results:We diagnosed 15 cases with congenital lung mal for ma tions. Of these, 9 had congenital cystic adenomatoid malfor mation (CCAM). TOP was performed in 6 cases with CCAM. Three cases had a favorable pre/postnatal evolution. Bronchopulmo nary sequestration (BPS) was diagnosed in 3 cases, all with favorable perinatal evolution. Right pulmonary agenesis was diagnosed in one case and the outcome was neonatal death. One case of congenital high airway obstruction syn drome was followed by TOP. One case of severe bilateral pul monary hy poplasia (secondary to a severe bilateral hydrothorax) resulted in neonatal death.
Conclusion:Obstetrical management is established individually depending on the severity of the cases.
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