congenital lung lesions

Although airway foreign body aspiration (FBA) is a common occurrence in any age group, unrecognized and retained foreign bodies in lungs may result in severe complications, such as lung abscess or bronchiectasis. In rare cases, FBA may present with similar clinical features as many other diseases (e.g. asthma, tumor, pulmonary eosinophilia). Here, we report a rare case of missed FBA in a nine-year-old boy, whose chest CT scan was suggestive of a cavitary lesion in the left lower lobe mimicking congenital pulmonary airway malformation (CPAM)...

Congenital lung malformations are a constellation of pathologies that can be diagnosed antenatally by ultrasound and fetal MRI. Ultrasound is considered the modality of choice for a routine assessment of second-trimester scans worldwide. Bronchopulmonary sequestration (BPS) and congenital pulmonary airway malformation (CPAM) are the 2 most common echogenic chest masses discovered incidentally during routine ultrasound scans in the second trimester. This paper describes BPS and differentiates it from CPAM sonographically in utero...

INTRODUCTION: Congenital lung malformations (CLMs) have a variable natural history. Larger lesions with CLM volume ratio (CVR) ≥ 1.6 are associated with hydrops and fetal mortality. The purpose of this study is to describe the management and outcomes of high-risk (CVR ≥ 1.6) CLM patients. METHODS: A retrospective cohort study was performed for all fetuses evaluated between May 2015 and May 2022. Demographics, prenatal imaging factors, prenatal and postnatal treatment, and outcomes were collected...

BACKGROUND: Open fetal resection for large lung lesions has virtually been replaced by maternal steroid administration. Despite this paradigm shift, little is known about the effects steroids have on lung lesion growth in utero. METHODS: A 10-year retrospective review of all prenatally diagnosed lung lesions cared for at our fetal care center was performed. We evaluated the effects of prenatal steroids on congenital pulmonary airway malformation (CPAM)-volume-ratio (CVR), distinguishing change in CVR among CPAMs, bronchopulmonary sequestrations (BPS), and bronchial atresias...

The bronchogenic cyst (BC) is a type of congenital anomaly that is most usually found in the mediastinum and lung, and rarely in the diaphragm. We report two cases of bronchogenic cysts originating from the left diaphragm. Case 1 involved a 50-year-old man who underwent computed tomography (CT) of the adrenal glands for hypertension, showing left adrenal changes. An adrenal CT at our hospital showed a space-occupying lesion above the left diaphragm. We performed a left-sided thoracotomy in the seventh intercostal space and found that the mass was entirely located in the diaphragm...

RATIONALE: Congenital pulmonary airway malformation (CPAM) is a rare congenital dysplastic malformation and accounts for 25% of congenital lung lesions. Commonly, it is diagnosed prenatally in ultrasound. The CPAM volume ratio (CVR) is a well-recognized predictor of fetal prognosis, and when the CVR is >1.6 cm2, the fetus is very likely to develop hydrops and even intrauterine deaths. However, the association of CVR with a wide range of complications and neonatal prognosis is unclear...

INTRODUCTION: Three-dimensional (3D) printed cardiac models are increasingly being used for medical education, simulation and training, communication, surgical planning and research. Given the complexities of congenital cardiac anatomy, 3D printing is well suited as an adjunct to traditional teaching methods. This study aims to explore the influence of 3D printed cardiac models as a teaching aid for nurses and paediatric trainees. We hypothesise that using 3D models as an adjunct to didactic teaching methods improves knowledge and confidence levels of participants, regardless of their cardiology experience...

BACKGROUND: Distinguishing congenital pulmonary airway malformations (CPAMs) from pleuropulmonary blastoma (PPB) can be challenging. Previously diagnosed patients with CPAM may have been misdiagnosed and we may have missed DICER1-associated PPBs, a diagnosis with important clinical implications for patients and their families. To gain insight in potential misdiagnoses, we systematically assessed somatic DICER1 gene mutation status in an unselected, retrospective cohort of patients with a CPAM diagnosis...

In the last few years, current evidence has supported the use of point-of-care ultrasound (POCUS) for a number of diagnostic and procedural applications. Considering the valuable information that POCUS can give, we propose a standardized protocol for the management of neonates with a congenital diaphragmatic hernia (CDH-POCUS protocol) in the neonatal intensive care unit. Indeed, POCUS could be a valid tool for the neonatologist through the evaluation of 1) cardiac function and pulmonary hypertension; 2) lung volumes, postoperative pleural effusion or pneumothorax; 3) splanchnic and renal perfusion, malrotations, and/or signs of necrotizing enterocolitis; 4) cerebral perfusion and eventual brain lesions that could contribute to neurodevelopmental impairment...

OBJECTIVES: This study evaluates the safety and efficacy of thoracoscopic lobectomy for congenital lung lesions in infants less then 4 months of age. MATERIALS AND METHODS: From January 1997 to October 2022, 194 patients under 4 months of age and weight less then 5.6 Kg underwent video-assisted thoracoscopic lobe resection for CPAM, Sequestration, and CLE. All procedures were performed by or under the direct guidance of a single surgeon. RESULTS: 195 of 196 procedures were completed thoracoscopically...

INTRODUCTION: Pleuropulmonary blastoma (PPB) is a rare, aggressive, primary intrathoracic malignancy typically seen in infancy and early childhood. Accurate distinction from congenital cystic lung lesions is crucial due to significant prognostic and therapeutic differences. Cytologic features have rarely been described. Establishing a cytodiagnosis is challenging owing to its rarity, lack of awareness, and multiple morphologic mimics. MATERIALS AND METHODS: This was a retrospective study conducted over 8 years...

Pulmonary sequestration is a congenital lung malformation characterized by a mass of nonfunctioning lung tissue that receives its arterial supply from an aberrant systemic artery. If symptomatic, most newborns present with respiratory distress. Recurrent infection is the most common presentation after the neonatal period. It is often diagnosed prenatally and is treated with elective surgical resection between ages six and twelve months. We present a case of an infant diagnosed with congenital pulmonary airway malformation prenatally revealed to be pulmonary sequestration at the age of six months, emphasizing the need for appropriate postnatal imaging...

BACKGROUND: Hereditary haemorrhagic telangiectasia (HHT), or Rendu-Osler-Weber syndrome, is a rare genetic disorder characterized by the development of telangiectasias and arteriovenous malformations (AVMs) throughout the body. We present a case of percutaneous embolization of pulmonary AVMs in an adult patient. CASE SUMMARY: A 26-year-old male patient with polycythaemia of unknown origin and a family history of secundum atrial septal defect underwent cardiac evaluation which revealed clubbing as a sign of peripheral cyanosis...

Congenital lung and lower airway abnormalities are rare, but they are an important differential diagnosis in children with respiratory diseases, especially if the disease is recurrent or does not resolve. The factors determining the time of presentation of congenital airway pathologies include the severity of narrowing, association with other lesions and the presence or absence of congenital heart disease (CHD). Bronchoscopy is required in these cases to assess the airway early after birth or when intubation and ventilation are difficult or not possible...

BACKGROUND: Pleuropulmonary blastoma (PPB) is an extremely rare and malignant pediatric lung tumor. Purely cystic PPB has a more favorable prognosis than solid PPB, but may be difficult to distinguish from a certain type of "benign" congenital pulmonary airway malformation before and during surgery. The influence of tumor rupture on long life prognosis has not been clarified in detail. CASE PRESENTATION: A 5-month-old boy underwent emergency transfer from another hospital due to a left thoracic cystic lesion and left pneumothorax detected on chest radiography performed for persistent wheeze and cough...

Langerhans cell histiocytosis is an uncommon proliferative disorder that may influence many organs; so, the clinical presentations vary. Here we describe an 85-day-old female who was born with In vitro fertilization after 10 years of infertility. She referred to us due to severe pulmonary insufficiency and congenital progressive maculopapular rash with desquamation. There were significant cystic changes in chest imaging studies. Further evaluation demonstrated lytic lesions in cranial, femoral, and humorous bones...

The heart and aortic arch arteries in amniotes form a double circulation, taking oxygenated blood from the heart to the body and deoxygenated blood to the lungs. These major vessels are formed in embryonic development from a series of paired and symmetrical arteries that undergo a complex remodelling process to form the asymmetric arch arteries in the adult. These embryonic arteries form in the pharyngeal arches, which are symmetrical bulges on the lateral surface of the head. The pharyngeal arches, and their associated arteries, are found in all classes of vertebrates, but the number varies, typically with the number of arches reducing through evolution...

INTRODUCTION: The aim of this study was to evaluate the accuracy of imaging tests (prenatal ultrasound [US] and postnatal computed tomography [CT]) in comparison to histology for diagnosis of congenital lung malformations (CLMs). MATERIAL AND METHODS: Retrospective study of patients with a prenatal diagnosis of CLM whose postnatal follow-up included thoracic CT scan and histological examination of the lesion. We collected data on demographic variables, gestational age at diagnosis, US findings and the history of multiple gestation...

The differential diagnosis for neonatal primary lung masses includes developmental anomalies and congenital lung tumors. Fetal lung interstitial tumor (FLIT) is a rare benign mesenchymal lesion which presents either antenatally or within the first 3 months of age. FLIT is a circumscribed solid-cystic mass which histologically resembles the fetal lung during the canalicular stage at 20-24 weeks of gestation. It is composed of immature mesenchymal cells expanding the interstitium and irregular airspace-like structures...

INTRODUCTION: Fetuses with large lung lesions including congenital cystic adenomatoid malformations (CCAMs) are at risk for cardiopulmonary compromise. Prenatal maternal betamethasone and cyst drainage for micro- and macrocystic lesions respectively have improved outcomes yet some lesions remain large and require resection before birth (open fetal surgery, OFS), at delivery via an Ex Utero Intrapartum Treatment (EXIT), or immediately post cesarean section (section-to-resection, STR). We sought to compare prenatal characteristics and outcomes in fetuses undergoing OFS, EXIT, or STR to inform decision-making and prenatal counseling...