Congenital Pulmonary Malformation Research Articles

Congenital pulmonary airway malformation in a cat.

Congenital structural anomalies of the lower airways of the respiratory tract are uncommon in cats. We describe here a case of cystic pulmonary lesions in a 6-wk-old domestic shorthair cat consistent with congenital pulmonary airway malformation (CPAM; formerly referred to as cystic adenomatoid malformation of the lung, or congenital pulmonary adenomatoid malformation; Stocker type II). CPAM is rarely reported in veterinary species and, to our knowledge, has not been reported in cats. In humans and veterinary species, individuals with CPAM (Stocker types I-IV) can be asymptomatic at birth but are predisposed to developing respiratory abnormalities that typically manifest clinically in the early years of life. We review the pathologic features of CPAM.

Fetal Therapy for Congenital Pulmonary Malformations: A Prospective Population-Based National Cohort Study.

To assess the frequency of fetal therapy for fetuses with congenital pulmonary malformations (CPMs) and to investigate their short-term outcomes. The study population included 435 singleton fetuses diagnosed with CPMs from a national population-based cohort study in France in 2015-2018. Information was obtained from medical records on CPM volume ratio (CVR), signs of compression, fetal therapy and perinatal outcomes. The characteristics and outcomes of fetuses with and without fetal therapy were compared using a univariate test. Twenty six fetuses (6.0%, 95% CI: 4.1-8.6) received at least one fetal therapy including thoracoamniotic shunts only (n=3), antenatal steroids only (n=12), and a combination of several therapies including thoracentesis and amniodrainage, in addition to shunts and steroids (n=11). Compared with fetuses without fetal therapy, those who did have higher CVR (1.6±0.3 vs. 0.7±0.04, p<0.001) and more severe signs of compression (73.1% vs. 12.8%, p<0.001). The proportion of live births after fetal therapy was 84.6% versus 98.5% (p<0.001) for those without fetal therapy and the hospital mortality rate was 13.6% versus 1.0% (p=0.004), respectively. A small minority of fetuses with CPMs underwent fetal therapy. These patients had a lower survival compared with those who did not receive fetal therapy. NCT02352207.

Extralobar sequestration — not obvious radiologic finding. Illustrated overview. Case report

INTRODUCTION: Bronchopulmonary sequestration is a rare congenital pulmonary malformation. A sequestration is generally defined as non-functioning lung tissue that is not in normal continuity with the tracheobronchial tree and derives its blood supply from systemic vessels. OBJECTIVE: To define the main clinical and radiologic features of intra- and extralobar sequestration of the lung, to study vessel variability and to analyse case of not obvious CT images of intralobar sequestration that was diagnosed intraoperatively. MATERIALS AND METHODS: We analysed literature found in PubMed and ScienceDirect regarding lung sequestration since 1979. The case report of a patient with extralobar sequestration diagnosed intraoperatively.RESULTS: The main clinical and radiologic features of lung sequestration, challenges of differential diagnosis were summarised, including specificity of case report.DISCUSSION: Presence of aberrant vessel requires verification of pathology before operation due to high risk of haemorrhage. The leading role in the verification belongs to radiologic imaging, however in rare cases aberrant vessel may not be visualised due to small diameter or occlusion.CONCLUSION: The anatomy of aberrant vessels in sequestrations is extremely variable. In case report sequestration was seen in an adult. The pathology was not diagnosed prior to the operation due to failure of the vessel visualisation. Knowledge of possible diagnostic challenges decreases the risk of intraoperative complications.

Congenital pulmonary malformations : Diagnosis and treatment

Congenital pulmonary malformations (CPM) are rare and can be associated with high morbidity. Clinical presentation, diagnostic procedures, imaging, and therapy of CPM are discussed. Today, most CPM can be diagnosed prenatally by ultrasound. Postnatally, respiratory symptoms up to respiratory failure and recurrent lower respiratory tract infection are typical findings. Due to low diagnostic accuracy of chest x‑ray in CPM, all children with prenatal diagnosis of CPM or postnatally suspected CPM should undergo cross-sectional imaging. Based on imaging alone, the various subtypes of CPM cannot be definitively differentiated, which is why histological confirmation remains the gold standard. Surgical resection is the standard of care with minimally invasive procedures increasingly being employed. In certain situations, awatch-and-wait approach is possible.

Внутриутробное лечение неиммунной водянки плода при кистозно-аденоматозном пороке развития легкого I типа

Aim. To present the experience of using a thoracoamniotic bypass for the treatment of a fetus diagnosed with nonimmune fetal dropsy, against the background of cystic adenomatous lung malformation type I. Key points. Congenital cystic adenomatous pulmonary malformation (CAPRL) is a benign lung lesion that appears before birth as a cyst or mass in the chest. The macrocystic form of this defect is associated with a significant risk of pulmonary hypoplasia, pleural effusion, and non-immune fetal hydrops. We present our own observation of a patient with type 1 CAPRL in the fetus and signs of non-immune hydrops. Conclusion. The use of intrauterine thoraco-amniotic shunting can stop the manifestations of hydrops in type I CAPRL in the fetus. Keywords: cystic adenomatous malformation of the lungs in the fetus, non-immune hydrops, thoraco-amniotic shunt.

Bilateral Pulmonary Sequestration – A Rare Entity

Abstract Bronchopulmonary sequestration (BPS) is a congenital anomaly within the spectrum of abnormalities that can affect the normal development of airways, lung parenchyma, and vasculature. It accounts for up to 6.4% of all congenital pulmonary malformations. Sequestrated lung is a nonfunctioning mass of lung tissue that lacks communication with the tracheobronchial tree and is supplied by systemic arterial circulation. Pulmonary sequestration is usually a unilateral disease, bilateral pulmonary sequestration is a rare entity. Herein we present the case of a 26year old female, evaluated for cough with expectoration, hemoptysis and loss of weight, whose radiology suggestive of bilateral bronchopulmonary sequestration.

Congenital pulmonary malformations in children in a pediatric hospital in Peru, 2010-2020.

Congenital pulmonary malformations (CPMs) are rare in children. This study aimed to describe the clinical, imaging characteristics, and treatment of patients with this pathology. We conducted a descriptive and retrospective study with data from patients with CPMs diagnosed at Instituto Nacional de Salud del Niño-Breña (Lima-Peru), from January 2010 to December 2020. We described CPM clinical and imaging characteristics, type and treatment. The sample was formed of 70 patients. The median age was 29 months (range 15 days-14 years) and the male/female ratio was 1.4. The chest tomography found parenchymal involvement in 50 (71.4%) cases and mixed involvement (parenchymal and vascular) in 18 (25.7%) cases. Congenital malformation of the pulmonary airway was present in 39 (55.7%) cases, followed by bronchogenic cyst in 10 (14.3%), intralobar pulmonary sequestration in 9 (12.9%), and extralobar pulmonary sequestration in 7 (10%). Lobectomy was performed in 61 (87.1%) cases, cystectomy in 5 (7.1%), segmentectomy in 2 (2.9%), and embolization in 2 (2.9%). The most frequent post-operative complication was pneumonia, found in 9 (12.9%) cases. The median hospital stay was 26 days. No patient died during hospitalization. In our institution, the most frequent CPM was congenital malformation of the pulmonary airway, and lobectomy was the most frequently performed surgical procedure. CPMs represent a diverse group of disorders of lung development with varied imaging patterns and clinical manifestations.

Do More with Less? Lobectomy vs. Segmentectomy for Patients with Congenital Pulmonary Malformations.

Congenital Pulmonary Malformations (CPMs) are rare benign lesions potentially causing infective complications and/or malignant transformation, requiring surgery even when asymptomatic. CPMs are rare in adulthood but potentially detected at any age. There is not a consensus on the correct extent of resection in both adults and paediatrics. This retrospective multicentric study aims to identify the appropriate surgical resection to prevent the recurrence of the related respiratory symptoms. Between 2010 and 2020, a total of 96 patients (adults and pediatrics) underwent surgery for CPMs in 4 centers. A 2:1 propensity score matching (considering sex and lesion side) was performed, identifying 2 groups: 50 patients underwent lobectomy (group A) and 25 sub-lobar resections (group B). Clinical and histopathological characteristics, early and late complications, and symptom recurrence were retrospectively analyzed and compared between the two groups by univariate and multivariate analysis. Patients who underwent lobectomy had a statistically significant lower rate of recurrence (4% vs. 24% of group B, p = 0.014) and a lower rate of intraoperative complications (p = 0.014). Logistic regression identified sub-lobar resection (p = 0.040), intra- and post-operative complications (p = 0.105 and 0.022),and associated developed neoplasm (p = 0.062) as possible risk factors for symptom recurrence after surgery. Pulmonary lobectomy seems to be the most effective surgical treatment for CPMs, guaranteeing the stable remission of symptoms and a lower rate of intra- and postoperative complications. To our knowledge, this is one of the largest studies comparing lobectomy and sub-lobar resections in patients affected by CPMs, considering the low incidence worldwide.

Resolution of Fetal Hydrops-not a Favorable Outcome in Congenital Pulmonary Malformation Type 1

Background: Congenital pulmonary airway malformations (CPAMs) comprise a spectrum of anatomical anomalies of the lungs and respiratory tree. The natural history of prenatally diagnosed CPAM varies from in utero resolution to progression to hydrops and intrauterine death. The majority of fetuses (90%) have a good outcome. However, in a minority (10%), the mass may grow so large that it is associated with complications and becomes life-threatening to the fetus. Clinical Description: Prenatal diagnosis of giant left CPAM type 1 was made at 24 weeks of gestation in a 29-year-old primigravida woman. The lesion consisted of multiple large cysts with a CPAM volume ratio of 4.7. Since the diagnosis, the mass was associated with nonimmune fetal hydrops and polyhydramnios which got worse throughout the following 4 weeks of gestation. Doppler studies remained normal until term. Management: The family received prenatal counseling. Parents refused termination of pregnancy and any other invasive or pharmacological therapy in light of the bad prognosis. The pregnancy was strictly monitored using standard fetal lung parameters. By 31 weeks' gestation, hydrops spontaneously improved to resolution but without any change in the size of the cysts. A baby girl was delivered at term, with birth asphyxia, who, despite all efforts by a multidisciplinary team, could not be revived. Autopsy and histopathological findings were compatible with type 1 CPAM. Conclusion: Hydrops is a well-known complication in fetuses with congenital cystic adenomatoid malformation. The present case reminds the possibility of its spontaneous resolution during pregnancy, even though the cyst size remains unaltered. The eventual neonatal survival depends not on the presence of hydrops but on the cyst volume and underlying lung hypoplasia.

Robotic-assisted thoracoscopic lobectomy for synchronous pulmonary sequestration and adenocarcinoma: A case report.

Pulmonary sequestration (PS) is a congenital pulmonary malformation. Adenocarcinoma arising in PS is extremely rare. We present the first reported case of synchronous intralobar PS and lung adenocarcinoma in the right lower lobe, which was successfully treated using robotic-assisted thoracic surgery (RATS). The robotic system allowed for easy identification, clipping, and dissection of the abnormal artery, highlighting its benefits over traditional surgical approaches. This case underscores the importance of considering the possibility of coexisting lung cancer in patients with a clinical diagnosis of PS and demonstrates the safety and efficacy of RATS in managing this rare condition.

Pulmonary Sequestration in an Adult: A Case Report

Pulmonary sequestration (PS) is a form of congenital pulmonary malformation that is uncommonly diagnosed during adulthood. Pulmonary sequestrations can manifest with variable presentations. It can remain asymptomatic or present with more severe symptoms such as hemoptysis and recurrent pneumonia. The main feature of this disease is that partial lung tissues separate from the main lung during the embryonic period, receiving blood supply from systemic circulation arteries. We report a case of a 39 year old men smoker with medical history characterized by frequent infections since childhood, chest pain and hemoptysis. CT scan showed a partial PS of left lower lobe, supplied by an abnormal artery arising from descending aorta. Surgical treatment through a lung sequestrectomy and basal lobectomy resection was performed.

Clinical-Diagnostic and Morphopathological Correlations in Congenital Pulmonary Malformation of the Respiratory Tract Type I in Newborn with Progressive Respiratory Distress

Clinical-Diagnostic and Morphopathological Correlations in Congenital Pulmonary Malformation of the Respiratory Tract Type I in Newborn with Progressive Respiratory Distress

Fetal Lung Interstitial Tumor (FLIT): Review of The Literature.

Fetal lung interstitial tumor (FLIT) is an extremely rare pediatric lung tumor that shares radiological features with congenital pulmonary malformations (cPAM) and other lung neoplasms. A review of the literature, together with the first European case, are herein reported. A systematic and manual search of the literature using the keyword "fetal lung interstitial tumor" was conducted on PUBMED, Scopus, and SCIE (Web of Science). Following the PRISMA guidelines, 12 articles were retrieved which describe a total of 21 cases of FLIT, and a new European case is presented. A prenatal diagnosis was reported in only 3 out of 22 (13%) cases. The mean age at surgery was 31 days of life (1-150); a lobectomy was performed in most of the cases. No complications or recurrence of disease were reported at a mean follow-up of 49 months. FLIT is rarely diagnosed during pregnancy, may present at birth with different levels of respiratory distress, and requires prompt surgical resection. Histology and immunohistochemistry allow for the differentiation of FLIT from cPAM and other lung tumors with poor prognosis, such as pleuropulmonary blastoma, congenital peri-bronchial myofibroblastic tumor, inflammatory myofibroblastic tumor, and congenital or infantile fibrosarcoma.

Impact of prenatal estimation of the risk of respiratory distress in neonates with congenital pulmonary malformations on the choice of delivery site

BackgroundThe vast majority of prenatally diagnosed congenital pulmonary malformations (CPM) remain asymptomatic at birth. The maximal value of the CPM volume ratio (CVRmax) predicts the risk of neonatal respiratory distress (NRD), and should allow for better assessment of the level of expertise needed at the delivery site. AimThis study evaluated the level of maternity units currently chosen for the delivery of CPMs, and determined the impact of the choice of delivery site based on the CVRmax, with a threshold of 0.4 cm2. MethodsData were extracted from the French prospective MALFPULM cohort, with inclusion between March 2015 and June 2018. ResultsThe final study population consisted of 383 women. Deliveries in level 1 or 2 maternity units (n = 98, 25%) involved CPMs with lower CVRmax (p<0.001), causing fewer signs of prenatal compression (p = 0.025). Among the 62 children (16%) who presented with NRD, only seven (11%) were born in level 1 or 2 units (p = 0.0078). Choosing the maternity level according to the CVRmax would have increased the number of births in level 1 or 2 maternity hospitals by 70%. In these maternity units, the percentage of children with NRD would have increased from 8% in the actual distribution to 10% in the new strategy. ConclusionOur results showed an overuse of level 3 maternity hospitals for the delivery of newborns with a prenatal diagnosis of CPM. The use of CVRmax should enable a reduction in the use of expertise centers without an adverse impact on newborns.

Antenatally Detected Thoracic Lesions: Prognosis, Management and Outcome.

Foetal thoracic lesions are uncommon, with the incidence of 1 in 15,000 live births. Antenatal monitoring of these lesions is required to prognosticate the parents about the postnatal outcome of the lesions and about the well-being of the baby. Foetal ultrasound and magnetic resonance imaging (MRI) are the modalities to detect these lesions and follow-up during pregnancy and postnatally. Congenital pulmonary adenomatoid malformations (CPAM), congenital diaphragmatic hernia (CDH) and bronchopulmonary sequestrations (BPS) are the commonly detected foetal thoracic lesions. Parameters such as congenital cystic adenomatoid malformation (CCAM) volume ratio (CVR) and lung-to-head ratio (LHR) determine the prognosis of these lesions. With this background, we planned to study the prognosis and outcome of antenatally detected thoracic lesions. This was a prospective study carried out for 2 years (January 2017 to December 2018). Pregnant females with foetuses diagnosed to have thoracic lesions on the second- and third-trimester ultrasound and foetal MRI were enrolled for the study. CVR and LHR were noted. Outcomes were analysed in terms of the need of termination of pregnancy, foetal or neonatal demise and need of surgery or conservative management. Of a total of 521 pregnancies, thoracic lesions were detected in 22 (4.22%) cases. Individually, the incidence of each lesion was: CPAM-10 (45.45%), BPS-5 (22.73%), CDH-5 (22.73%) and congenital higher airway obstruction-2 (9.1%). Chromosomal screening was normal in 100% of cases. Follow-up was done for 2 years. Termination of pregnancy was required in 3 (13.64%) cases, postnatal death occurred in 4 (18.2%) cases, surgery was required in three cases of CDH soon after birth and two cases of extralobar BPS at 2 years of age. 10 (45.45%) cases were managed conservatively, who are doing well at 2-year follow-up. CPAMs, BPSs and CDH are the commonly detected antenatal thoracic lesions. Ultrasonography and MRI help detect and prognosticate the parents about the outcomes of these lesions. CVR and LHR with associated congenital malformations are important prognostic markers for these lesions. There is a need of long-term prospective studies to delineate the natural history of these lesions.

Long-Term Results of Pediatric Congenital Pulmonary Malformation: A Population-Based Matched Case-Control Study with a Mean 7-Year Follow-Up.

Symptomatic congenital pulmonary malformations (CPMs) are a group of anomalies involving the lungs. The long-term outcomes of these patients are not well known. The present research aimed to study the pulmonary function, respiratory morbidity, and health-related quality of life (QoL) of patients treated for CPMs. All children (<16 years of age) treated for CPMs in 2002−2012 (in Oulu University Hospital) were invited to the follow-up visit. Altogether, there were 22 patients, out of which 17 (77%) participated. The mean follow-up time was 6.6 (ranged from 3 to 16) years. Pulmonary function tests, diffusing capacity, respiratory morbidity, and QoL were determined as the primary outcomes. Potential residual malformations and lung anatomy were investigated using computer tomography (CT) imaging. The outcomes were compared to the age- and sex-matched healthy controls. The forced expiratory volume at 1 s (FEV1, Z-score) remained lower in operated patients compared to the healthy controls (−1.57 ± SD 1.35 vs. −0.39 ± SD −0.86, p-value 0.005). There were no differences in respiratory morbidity or QoL between the patients and the controls. The surgical approach (lobectomy vs. partial resection) did not affect lung function. A younger age (<1 year of age) at the time of the surgery seemed to result in a higher lung capacity, but the finding was not statistically significant. Patients with CPMs treated with surgery were satisfied with their wellbeing in the long-term. A lower lung function did not have an impact on their wellbeing. However, there was a slight decrease in lung function compared to the healthy controls, and a clinical follow-up of the patients was recommended.

Clinical Presentation and Surgical Management of Five Pediatric Cases with Bronchogenic Cysts: Retrospective Case Series.

Bronchogenic cysts (BCs) refer to congenital lesions that result from primitive or abnormal foregut budding, and can be pulmonary or mediastinal. Their occurrence can take place at any point on the tracheobronchial tree, but they are usually localized in the lung parenchyma and mediastinum, and may be symptomatic or asymptomatic. Bronchogenic cyst symptoms can vary, depending on the size and location of the cyst. A retrospective review of the charts of five patients with a histopathological diagnosis of bronchogenic cysts was performed between 2014 and 2020. The patients reported in this study were diagnosed and managed at Abha Maternity and Children Hospital, Abha, southwest Saudi Arabia. In addition, demographic information, as well as diagnostic and therapeutic information, was provided for each patient, both at discharge and after discharge. All patients had confirmed congenital bronchogenic cysts with different clinical phenotypes and radiological findings. All patients had histopathologically confirmed bronchogenic cysts with different clinical and radiological presentations. Two patients had mediastinal-located cysts; one had a laryngeal cyst; and the last two patients had infected intrapulmonary bronchogenic cysts. All patients underwent complete excision and did not experience recurrence or other postoperative complications during the follow-up period. The latter two patients required lobectomies of the right middle and upper lobes. Although bronchogenic cysts are considered a rare congenital pulmonary malformation, they should be considered in the differential diagnosis of pediatric patients with unusual airway and parenchymal lung manifestations, particularly, persistent stridor, feeding difficulty, and complicated pneumonia. Surgical excision of the cyst is the gold-standard therapy for symptomatic bronchogenic cysts and is highly recommended for asymptomatic ones. Long-term follow-up studies will be required to explore any long-term complications of BCs, particularly regarding the malignancy transformation.

Lung epithelium development and airway regeneration.

The lung is composed of a highly branched airway structure, which humidifies and warms the inhaled air before entering the alveolar compartment. In the alveoli, a thin layer of epithelium is in close proximity with the capillary endothelium, allowing for an efficient exchange of oxygen and carbon dioxide. During development proliferation and differentiation of progenitor cells generates the lung architecture, and in the adult lung a proper function of progenitor cells is needed to regenerate after injury. Malfunctioning of progenitors during development results in various congenital lung disorders, such as Congenital Diaphragmatic Hernia (CDH) and Congenital Pulmonary Adenomatoid Malformation (CPAM). In addition, many premature neonates experience continuous insults on the lung caused by artificial ventilation and supplemental oxygen, which requires a highly controlled mechanism of airway repair. Malfunctioning of airway progenitors during regeneration can result in reduction of respiratory function or (chronic) airway diseases. Pathways that are active during development are frequently re-activated upon damage. Understanding the basic mechanisms of lung development and the behavior of progenitor cell in the ontogeny and regeneration of the lung may help to better understand the underlying cause of lung diseases, especially those occurring in prenatal development or in the immediate postnatal period of life. This review provides an overview of lung development and the cell types involved in repair of lung damage with a focus on the airway.

Hemothorax From an Anomalous Bronchial Artery Bleed in an Infected Intralobar Pulmonary Sequestration

Pulmonary sequestration is a rare congenital malformation. It represents 0.15-6.4% of all congenital pulmonary malformations. It is characterized by non-functional, dysplastic mass of lung tissue that is not in communication with the normal tracheobronchial tree and is associated with a systemic arterial supply. We report a young gentleman in his mid-thirties who presented with community-acquired pneumonia from an infected intralobar pulmonary sequestration which subsequently developed a hemothorax from an anomalous bronchial artery bleed.

Congenital Pulmonary Adenomatoid Malformation in three Infants with Diagnostic and Management Challenges in a Low and Middle-Income Country: A Case Series

Congenital Pulmonary Adenomatoid Malformation in three Infants with Diagnostic and Management Challenges in a Low and Middle-Income Country: A Case Series