Bronchopulmonary Malformations Research Articles

Retrospective evaluation of neonates with fatal congenital lung malformation: A single center 15-year forensic autopsy experience.

Congenital lung malformation (CLM) is a leading cause of infant mortality. Clinical methods for diagnosing CLM mainly rely on computed tomography, magnetic resonance imaging, ultrasonography, and Doppler. However, forensic identification of the cause of death in neonates is challenging. Unequivocal classification criteria for CLM are missing as its forensic identification is ambiguous. Therefore, we aimed to analyze neonatal death cases at our center to assist in identifying those with congenital lung malformation. This retrospective study identified and classified the causes of deaths of neonates autopsied between January 2008 and April 2023. All cases born alive and died within 28 days with a clear time of death were selected, and forensic experts reviewed their records. The manner, cause of death, and other characteristics were noted and discussed. This retrospective study reveals a steady increase in autopsy cases from 2008 to 2015, attributed to improved parental consent, heightened awareness of autopsy importance, and enhanced medical resources. However, a subsequent decline post-2015 is observed, potentially influenced by advancements in medical technology and prenatal examination protocols. The top causes of neonatal mortality include respiratory diseases, asphyxia, congenital dysplasia, and fetal distress. Congenital lung malformations, particularly bronchopulmonary malformations, constitute a significant portion of congenital anomalies. This study underscores the importance of standardized autopsies and histopathological examinations in diagnosing and understanding CLM. Future research should focus on expanding case collections and elucidating the genetic basis of CLM to improve forensic management and outcomes.

CYSTIC ADENOMATOID MALFORMATION OF THE LUNG IN AN ADULT: A CASE REPORT

CYSTIC ADENOMATOID MALFORMATION OF THE LUNG IN AN ADULT: A CASE REPORT

Bronchopulmonary malformation with multiple feeding vessels: rare presentation and novel use of indocyanine green

Bronchopulmonary malformation with multiple feeding vessels: rare presentation and novel use of indocyanine green

PREGNANCY MANAGEMENT AND OUTCOME IN A WOMAN WITH CONGENITAL UNILATERAL LUNG AGENESIS: A CASE REPORT

TOPIC: Lung Pathology TYPE: Medical Student/Resident Case Reports INTRODUCTION: Congenital unilateral lung agenesis is a rare condition characterized by the complete absence of a lung and the bronchovascular tree. It is often associated with cardiac and bronchopulmonary malformations, resulting in significant morbidity and mortality. Pregnancy, due to the physiologic changes during gestation and childbirth, poses additional risks to these patients. We report a case of a woman with congenital right lung agenesis (CRLA) whose successful pregnancy could offer guidance to clinicians in the interdisciplinary management of a high-risk condition (1). CASE PRESENTATION: A 26-year-old G1P0 was referred to our Maternal Fetal Medicine (MFM) clinic for her CRLA with resultant dextrocardia and left lung hyperinflation. She was asymptomatic and was able to tolerate daily activities without concerns. Pre-conceptional imaging studies showed results consistent with her medical history.There were no obstetric concerns with normal prenatal labs and normal fetal anatomy ultrasound. She was recommended to undergo cardiopulmonary evaluation and follow up with the relevant specialists.The patient remained clinically stable throughout the gestation. Delivery and postpartum planning was done in the early third trimester at a multidisciplinary conference with the MFM, Anesthesia, Cardiology, Pulmonology, Obstetrics, and Critical Care teams.The patient was electively induced at 39 weeks resulting in an uncomplicated vaginal delivery. Careful monitoring of the patient's hemodynamic status was performed during the intra- and postpartum periods due to the concern of volume overload from intravenous fluid infusions during and autotransfusion following delivery. Her postpartum course was uncomplicated. DISCUSSION: Several cardiopulmonary physiological changes occur in pregnancy to accommodate the growing fetus. An increase in cardiac output with a decrease in pulmonary and systemic vascular resistance leads to an increase in pre-load and increased pulmonary capillary permeability. The risk of pulmonary edema is highest in the second stage of labor due to the autotransfusion into the systemic circulation and the relief of pressure on the inferior vena cava (2). During pregnancy, there is a 40% increased oxygen requirement by the growing fetus, placenta, and maternal organs with a decreased reserve secondary to the increased consumption and decreased functional residual capacity. These changes are often the source of significant morbidity and mortality in women with compromised systems (3). For these reasons, a multidisciplinary care approach was of utmost importance for this patient. CONCLUSIONS: We advocate for the utility of a multidisciplinary approach with frequent follow-up and testing with each specialty to ensure the best care for a successful pregnancy outcome in this patient with CRLA. REFERENCE #1: Wert S. E. (2016). Normal and Abnormal Structural Development of the Lung. In Polin R. A., Abman S. H., Rowitch D. H., Benitz W. E., & Fox W. W., Fetal and Neonatal Physiology (pp. 627-641). Elsevier. https://doi.org/10.1016/B978-0-323-35214-7.00061-5 REFERENCE #2: Silversides C. K. & Colman J. M. (2007). Physiological changes in pregnancy. Oakley, C. & Warnes C. A. (2007). Heart Disease in Pregnancy (pp. 6-16). Blackwell Publishing Limited. REFERENCE #3: Gabbe, S. G., Niebyl, J. R., Simpson, J. L., Landon, M. B., Galan, H. L., Jauniaux, E. R., ... & Grobman, W. A. (2016). Obstetrics: normal and problem pregnancies e-book. Elsevier Health Sciences. DISCLOSURES: No relevant relationships by Kajal Angras, source=Web Response No relevant relationships by Kelly Ca, source=Web Response No relevant relationships by Cherise Hatch, source=Web Response No relevant relationships by VANI MOVVA, source=Web Response

Ectopic bronchogenic cyst in the retroperitoneal region: a case report and literature review of adult patients

BackgroundBronchogenic cyst is congenital aberration of bronchopulmonary malformation, which is rarely encountered in the abdomen and retroperitoneum. We present a case report and literature review of retroperitoneal bronchogenic cyst.Case presentationA 53-year-old female presented to outpatient clinic for a routine checkup of lumbar intervertebral disc herniation. She received a contrast computed tomography scan of the abdomen which revealed a retroperitoneal cystic lesion below the left crura of diaphragm. Afterward, the patient underwent a laparoscopic excision of the cystic lesion and was discharged uneventfully at postoperative day 4. Histopathological findings confirmed the diagnosis of retroperitoneal bronchogenic cyst. Our literature review identified 55 adult cases in recent two decades. The average age at diagnosis was 43.2 (range 17–69) years. 44 (80%) cases had a retroperitoneal cyst on the left side, and 52 (94.5%) cases underwent curative excision through open or laparoscopic surgery. In the available follow up of cases, there was no recurrence after surgery.ConclusionsBronchogenic cyst is rare in the retroperitoneal region. It should be considered as one of the differential diagnoses of a retroperitoneal neoplasm.

Congenital Lobar Emphysema: Perioperative Evaluation and Management

Congenital lobar emphysema (CLE) is a lung malformation characterized by overdistension and air trapping in the affected lobe. It is one of the causes of neonatal and infantile respiratory distress. This study aimed to evaluate our experience regarding perioperative and surgical management in children with CLE. A retrospective observational study was done for all CLE patients who underwent surgery at Menoufia University Hospital. Perioperative data collected included demographic, clinical, and radiological findings, as well as operative and postoperative data. We included 30 neonates and infants who suffered from CLE between January 2013 and December 2020; the mean age was 111.43 ± 65.19 days, and 21 were males. All cases presented with respiratory distress; 19 had cyanosis, and 15 had recurrent pneumonia and fever. Plain chest x-ray and computed tomography (CT) revealed emphysema in all cases. Lobectomy was done in all cases; the mean age at surgery was 147.58 ± 81.49 days. Postoperative complications occurred in 5 patients, and 2 of them needed mechanical ventilation. The follow-up duration ranged from 3 months to 1 year (except 1 case lost to follow-up after 3 months), and all patients were doing well. CLE is a rare bronchopulmonary malformation that requires a high index of clinical suspicion, especially in persistent and recurrent infantile respiratory distress. Chest CT is the most useful diagnostic modality. Early management of CLE improves outcome and prevents life-threatening complications. Surgical management is the treatment of choice in our center, without recorded mortality.

Magnetic resonance imaging for congenital lung malformations

Congenital lung malformations are most often identified on prenatal US screening. Fetal MRI is often performed to further evaluate these lesions. Although some of these lesions might cause prenatal or early postnatal symptoms that require urgent management, the majority are asymptomatic at birth and might be subtle or invisible on chest radiographs. Postnatal imaging is frequently deferred until 3–6 months of age, when surgery or long-term conservative management is contemplated. High-quality imaging and interpretation is needed to assist with appropriate decision-making. Contrast-enhanced chest CT, typically with angiographic technique, has been the usual postnatal imaging choice. In this review, the author discusses and illustrates the indications and use of postnatal MR imaging for bronchopulmonary malformations as well as some differential diagnoses and the advantages and disadvantages of MR versus CT.

‘Hybrid’ Bronchopulmonary Malformation – Lobar Emphysema and Extra Lobar Sequestration

Background: Idiopathic lobar emphysema (ILE) and bronchopulmonary sequestration (BPS) are two of the well-characterized pulmonary malformations. Case report: An antenatally detected case of a left bronchopulmonary malformation (BPM) was clinicoradiologically diagnosed to be a left upper lobar emphysema with isolated dextrocardia in the neonatal period. Besides the emphysematous left upper lobe, an accessory lobe akin to an extra lobar BPS was an operative surprise. Histopathological examination of both excised lobes led to a revised diagnosis of a ‘hybrid’ malformation comprising lobar emphysema and extra lobar BPS. The postoperative recovery was uneventful. Discussion/conclusion: The observations suggest that BPS and ILE may be interim entities in a continuum of abnormal embryogenesis. Such a hybrid malformation has not been hitherto reported.

Isolated systemic arterial supply to normal lung - an unusual cause of extracardiac left-to-right shunt.

Isolated systemic arterial supply to a normal lung, a type of bronchopulmonary vascular malformation, is a rare cause of extracardiac left-to-right shunt. We describe such a case that was successfully managed by transcatheter closure of the anomalous arterial supply to otherwise normal lung.

Clinical and surgical aspects of congenital lobar over-inflation: a single center retrospective study

BackgroundCongenital lobar overinflation (CLOI) is one of the most important causes of infantile respiratory distress (RD). We aim to evaluate our experience in CLOI management emphasizing on clinical features, diagnostic modalities, surgery and outcomes.MethodsThis is a retrospective study for all CLOI cases undergoing surgical management at Qena University Hospital. Demographic data, clinical data, radiographic findings, surgery and postoperative follow-up were reviewed.ResultsA total of 37 neonates and infants with CLOI were presented to our center between January 2015 and January 2019; their mean age was 111.43 ± 65.19 days and 22 were males. All cases presented with RD; and cyanosis in 19 cases. 15 cases presented with recurrent pneumonia and fever. Diminished breath sounds on the affected side and wheezes were the main clinical findings in 30 and 22 cases respectively. On CXR, emphysema was detected in all cases. A confirmatory CT chest was done for all cases. Left upper lobe was affected in 23 cases, right middle lobe in 7 and right upper lobe in 7 cases.Lobectomy was done in thirty-one cases; their mean age at surgery was 147.58 ± 81.49 days and 19 were males. Postoperative complications were noted in 5 cases and postoperative ventilation was required for 2 of them. No morbidity or mortality was reported. The follow-up duration ranged from 3 months to 1 year and all patients were doing well except one case that lost follow up after 3 months.ConclusionCLOI is a rare bronchopulmonary malformation that requires a high index of clinical suspicion, especially in persistent and recurrent infantile RD. CT chest is the most useful diagnostic modality. Early management of CLOI improves outcome and avoid life-threatening complications. Surgical management is the treatment of choice in our center without recorded mortality.

Fetal Bronkopulmoner Malformasyonlar: Prenatal Tanı ve Perinatal Sonuçlar

Fetal Bronkopulmoner Malformasyonlar: Prenatal Tanı ve Perinatal Sonuçlar

P1311 A rare case of asymptomatic cyst in interatrial septum longterm observation

Abstract Background Incidental findings in echocardiography performed from other indications are not always able to explain the patient condition as well as the observed pathology. However, review of literature, helps to find the most possible explanation. We present a case study of a patient with our proposal of possible diagnosis. Case report 28-year old female with hypothyroidism and no other concomitant diseases presented with easy fatigue. Exercise stress test was clinically and ECG negative with 12,8 METs. Transthoracic echocardiography showed normal size of heart structures with preserved systolic and diastolic function of both left and right chamber. The only abnormality was a cystic structure of 44x31x34 cm in interatrial septum which was protruding to both left and right atrium and was not affecting the blood flow with normal gradients. We did not find any signs of inflammation nor infiltration to the heart walls. There was no flow inside the cyst with the use of colour Doppler. The structure had homogenous echolucency with very thin walls and a septum inside. As we did not find any connection between the cyst and the atriums we considered it as not harmful in terms of a risk of embolization. Patient was hemodynamically stable, there was no history of neurological dysfunctions nor atrioventricular conduction disturbances. Taking into account possible diagnoses for that patient we decided to schedule the patient for follow-ups. Ten years observation period confirmed that that our approach was proper. Serial echocardiographic studies showed no change in the cyst. In this year we performed multimodality imaging with CT and MRI, which showed the same views as echocardiography. Discussion The most possible diagnose for our patient is a bronchogenic cyst, a very rare congenital bronchopulmonary malformation of the foregut that is usually found incidentally with mostly asymptomatic benign course. Also the localization of the cyst confirms that diagnosis. Nevertheless the final diagnosis would be possible only in histopathological study. The differential diagnosis should include a cystic myxoma and echinococcosis. However the structure is not polycystic and seems to have homogenous echolucency. We ruled out the blood cyst because of the lack of typical localization and connection to valves. As long as the patient remains asymptomatic surgical resection remains controversial.

The role of multidetector CT angiography and 3D postprocessing imaging in the diagnosis and investigation of bronchopulmonary sequestration.

Key Clinical MessageCongenital bronchopulmonary malformations are usually asymptomatic. Precise multimodality imaging plays an essential role in the identification of rare cardiothoracic entities, offering excellent imaging quality and the decisive diagnosis.

Risk analysis and outcomes of bronchopulmonary sequestrations.

Researched and discussed the risks and outcomes of bronchopulmonary sequestrations, especially the intralobar type. A retrospective review of our experiences with bronchopulmonary sequestrations from January 2012 to April 2015 is reported. The present study researched and discusses the risks and outcomes of bronchopulmonary sequestrations, especially the intralobar type, compared with other types of bronchopulmonary sequestrations in symptoms, surgery, pathology, and excretion. A total of 126 bronchopulmonary sequestrations were diagnosed. All fetal chest cases (18-30weeks) of solid or high-echo masses were diagnosed antenatally and then confirmed by ultrasound. Enhanced computed tomography was used to confirm the diagnosis. Eighty-three boys and 43 girls were included. The mean age at surgery was 4.2±0.5months. There were 103 cases of left, 22 cases of right, and 1 case of bilateral sequestration. There were 62 extralobar cases, 51 intralobar cases, 13 cases within the diaphragm, and a rare bilateral case. A preoperative history of recurrent respiratory tract infection was present in 39 cases, including 10 extralobar and 29 intralobar cases. Operations were completed successfully, and diagnoses were confirmed pathologically. Thirty-seven cases were associated with congenital bronchopulmonary malformation changes, of which 4 were extralobar, 31 were intralobar, and 2 were within the diaphragm. The intralobar type was relatively uncommon among bronchopulmonary sequestrations. However, due to communication with normal lung tissue, infection is common in the intralobar type. Computed tomography examinations are very important immediately after birth. It is necessary to resect the mass in early childhood.

Congenital bronchopulmonary malformation: CT histopathological correlation

This study evaluated the accuracy of postnatal computed tomography (CT) imaging in the identification of congenital bronchopulmonary malformation (BPM) in comparison with histopathological analysis. CT scans of prenatally diagnosed BPMs from 24 patients with available histology were analysed retrospectively. The CT images were reviewed blinded to histological findings by two radiologists. Specific diagnosis was assigned based on predetermined criteria. The accuracy of CT was evaluated. The agreement rate in CT diagnosis between two radiologists was 100%. In 75% the lesions were located in the lower lobes. An overlap of 71% in CT and histopathological diagnoses was reached. The least matching diagnosis was type 2 CPAM. Contrast enhanced chest CT is very accurate in characterizing the BPM spectrum and provides important information on lesion type and structure.

Congenital Pulmonary Sequestration Supplied by the Right Coronary Artery

A 67-year-old patient, with no respiratory symptoms, underwent coronary catheterization for revascularization of stenosis in the right coronary artery, during which we observed a large caliber artery, apparently bronchial, extending toward the left lung base (Fig. 1A). Multislice computed tomography angiography revealed a rounded heterogeneous consolidation, 3–4 cm, in the left cardiophrenic angle. Drainage veins extending toward the left atrium were observed within the consolidation (Fig. 1B). Bronchopulmonary sequestration of the left lower lobe was diagnosed. Pulmonary sequestration (PS) is a rare malformation (0.15%–6.4% of all bronchopulmonary malformations). It is defined as an area of non-functioning lung, unconnected, or poorly connected with the tracheobronchial tree, usually irrigated by the systemic circulation. PS is conventionally classified as intralobar or extralobar. Pryce1 identifies 3 types of PS, depending on its relationship with the bronchial tree and its blood supply. This was extralobar PS type 3, since the patient presented an extrabronchial pulmonary area irrigated by the systemic circulation. Irrigationwassupplied by the right coronary artery; this is an extraordinarily rare observation – only 15 cases have been reported in the medical literature.2

Congenital Lesions of the Lung

1. Clinicians can provide better care for patients with congenital lung abnormalities when they understand the developmental sequence and different types of abnormalities. 2. Knowing the appropriate antenatal and postnatal imaging strategies for congenital lung abnormalities will optimize diagnosis and guide treatment. After completing this article, readers should be able to: 1. Understand the developmental sequence of congenital lung abnormalities (CLA). 2. Distinguish the different types of CLA. 3. Identify the antenatal and postnatal imaging to optimize the diagnosis of CLA. 4. Know the treatment strategies for CLA. Congenital lung abnormalities (CLAs), also termed bronchopulmonary malformation (BPM), are increasingly identified in the perinatal setting. The use of ultrasonography with improved resolution for antenatal screening, along with supplementary fetal magnetic resonance imaging (MRI) has increased the detection of pulmonary lesions. The estimated incidence is 1 in 2,000 to 1 in 2,500 live births. A large percentage of these will remain asymptomatic in the neonatal period and later. An understanding of the pathophysiology and timing of the development of these lesions is essential in the diagnosis and treatment of these infants. CLAs are likely to represent a spectrum of developmental miscues, along with tracheobronchial obstruction, that result in the downstream formation of these lesions. (1) Lung development occurs through 5 distinct stages as follows: Embryonic (4–7 weeks): Lung buds develop off the primitive foregut. Lobar branching occurs along with vascular inflow and outflow development. Pseudoglandular (7–17 weeks): The tracheobronchial tree grows and branches, ultimately forming 20 generations of airways. Vascular ingrowth matches the tracheobronchial tree to the preacinar level and achieves a mature configuration. Arteries follow airway branching with the veins located in the adjacent connective tissue. Cannicular (16–23 weeks): Terminal branching of the bronchial tree occurs, forming respiratory bronchioles and initiating the formation of air-blood interface along with the differentiation of type 1 and 2 pneumocytes. Type …

Perinatal Thoracic Mass Lesions: Pre- and Postnatal Imaging

Chest masses present a common problem in the perinatal period. Advances in prenatal ultrasound, supplemented by fetal magnetic resonance imaging, now allow early detection and detailed characterization of many thoracic lesions in utero. As such, in asymptomatic infants, assessment with postnatal computed tomography or magnetic resonance imaging can often be delayed for several months until the time at which surgery is being contemplated. Bronchopulmonary malformations comprise most of the thoracic masses encountered in clinical practice. However, a variety of other pathologies can mimic their appearances or produce similar effects such as hypoplasia of a lung or both lungs. Understanding of the key differentiating clinical and imaging features can assist in optimizing prognostication and timely management.

Surgical management of bronchopulmonary malformations

BackgroundBronchopulmonary malformations (BPM) are rare conditions, which typically arise below the carina and can result in significant morbidity (infection and/or hemorrhage) and mortality (respiratory failure). Materials and methodsAll children with BPM surgically treated from 2001–2014 at a tertiary care children's hospital were identified. Patient demographics, surgical indications, procedure type, estimated blood loss, pathology, perioperative complications, length of stay, and outcomes were analyzed. ResultsA total of 41 BPM patients underwent surgery with 98% overall survival (one abdominal BPM expired) but 100% for thoracic lesions. Resections were performed thoracoscopically (37%), thoracoscopy converted to open (22%), and via thoracotomy (37%). Poor visualization (67%) or inability to tolerate single lung ventilation (33%) led to conversions. No conversions resulted from hemorrhage or received blood transfusions. Patients with prenatally diagnosed BPM were more likely to undergo thoracoscopic surgery (odds ratio [OR], 18.2) versus nonprenatally diagnosed, P = 0.002. Open/converted patients had longer chest tube days (6.2) versus thoracoscopic (2.9), P = 0.048. Additionally, respiratory distress was a more common indication in patients aged <4 mo (OR, 28.0) versus ≥4 mo and <6 kg (OR, 40.5) versus ≥6 kg, P < 0.001. Open resections were more common in patients aged <4 mo (OR, 26.3) versus ≥4 mo, P = 0.002. Operative time was shorter and estimated blood loss (mL/kg) was greater for <6 versus ≥6 kg, P < 0.05. ConclusionsBPM resections have high overall survival. Chest tube days are shorter among thoracoscopic patients, but conversion to thoracotomy can avoid hemorrhage and need for transfusion. Size and respiratory distress limit use of thoracoscopy in young infants with BPM.

Secuestro pulmonar extralobar asintomático y la importancia del diagnóstico y tratamiento perinatal: El manejo expectante no es una opción terapéutica

Introduction: Prenatal ultrasonography currently represents presentation form of bronchopulmonary malformations. To 90% manifest in neonates with obvious radiological abnormalities and symptomatic progression where surgical resection is clearly indicated. In < 4% causing mass effect with mediastinal shift, hydrops and fetal death and other extreme behave «asymptomatic» with apparent involution and its management has been controversial: early elective resection or radiologic expectant management to document the regression or development complications. It analyzes the literature about the controversy: operate or not to operate. Description of cases: Three fetuses with prenatal diagnosis of intrathoracic solid mass, asymptomatic newborns: one with hydrothorax and other without apparent prenatal involution both with abnormal irrigation compatible with extralobar sequestration, the third with a paracardiac mass where imaging studies did not diagnostic a extralobar sequestration of ectopic location and synchronous with cystic adenomatoid malformation, only transoperative and pathological fi nd- ings confi rmed the diagnosis. Discussion: In asymptomatic patients all bronchopulmonary malformations could be diagnosed and surgically resected for unrecognized their natural history and the low rate of natural regression, radiological proposals are not effective for monitoring.