congenital cystic lung

Erythrocytosis or polycythemia is defined as an increase in red blood cell concentration above the age- and sex-specific normal levels. Unlike anemia that is very common in chronic kidney disease (CKD) patients, erythrocytosis is less frequent but requires specific understanding by healthcare professionals in order to provide the best care. Erythrocytosis, especially when undiagnosed and untreated, can lead to serious thrombotic events and higher mortality. Classical causes of erythrocytosis associated with CKD include cystic kidney diseases, kidney or other erythropoietin-secreting neoplasms, high-altitude renal syndrome, overdosage of erythropoietin-stimulating agents, androgen therapy, heavy smoking, chronic lung disease, obstructive sleep apnea, IgA nephropathy, post-kidney transplant erythrocytosis, renal artery stenosis and congenital etiologies...

BACKGROUND: Surgical intervention is advisable for both asymptomatic and symptomatic CCAM children. This study aims to compare and analyze the efficacy of thoracoscopic and Da Vinci robot-assisted procedures in the management of CCAM among pediatric patients. METHODS: The clinical data of 188 pediatric patients diagnosed with CCAM and admitted to the Children's Hospital, Zhejiang University School of Medicine, from April 2019 to April 2023 were retrospectively analyzed...

BACKGROUND: 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...

Pulmonary sequestration and cystic pulmonary adenomatoid malformation are rare congenital cystic disorders of the lungs. The presence of both the diseases in the same individual is therefore very uncommon. Pulmonary sequestration is a nonfunctional pulmonary tissue mass that derives its blood supply from systemic blood supply other than pulmonary circulation. Congenital cystic pulmonary adenomatoid malformation represents a mass consisting of abnormal bronchiolar air spaces and a deficiency of functional alveoli...

OBJECTIVE: Bronchogenic cysts are rare congenital abnormalities, and usually asymptomatic until adulthood. We present a fetus prenatally diagnosed with a bronchogenic cyst, experiencing compression symptoms immediately after birth and underwent thoracoscopic surgery at 14 days old. CASE REPORT: A 33-year-old primigravida had a suspicion of fetal tracheal cyst. Prenatal ultrasound scan revealed a cyst near the trachea at 23 weeks' gestation. Fetal MRI defined a cystic lesion in the upper mediastinum, displacing surrounding vessels...

Pulmonary sequestration (PS) is a rare congenital anomaly that accounts for 1% to 6% of all pulmonary malformations at birth. It is characterized by a focal area of pulmonary tissue that does not have direct communication with the tracheobronchial tree and does not get blood supply from the pulmonary circulation. We present the case of a 28-year-old female with a history of recurrent pulmonary infections who was found to have intralobar sequestration and underwent curative surgical excision. Because pulmonary sequestration is commonly misdiagnosed, as it can mimic other conditions on chest X-rays, this case illustrates the importance of recognizing pulmonary sequestration as a separate entity and diagnosing/treating it appropriately...

A non-smoker man in his second decade presented to a medical centre with intermittent haemoptysis over 2 years. The haemoptysis was infrequent initially to be ignored, but later, the episodes increased in amount and frequency. Routine blood tests including coagulation profile showed normal results. The chest radiography and echocardiography were normal. The contrast-enhanced CT scan of the chest showed a cystic lesion with intracystic abnormality and surrounding ground-glass opacity in the left upper lobe. The CT pulmonary angiography and invasive pulmonary angiography showed the abnormality to be a dilated pulmonary vessel draining into the left atrium, thereby confirming the diagnosis of congenital pulmonary varix contained within a lung cyst...

INTRODUCTION: Children's rare lung diseases are a heterogeneous group of rare lung diseases with significant morbidity and mortality. There is very limited information on the incidence and prevalence of children's rare lung diseases in Asia. We investigated the nationwide incidence, prevalence, and pattern of medical service utilization of children's rare lung diseases in Korea. METHODS: We studied patients who were diagnosed with rare lung diseases coded per International Statistical Classification of Diseases and Related Health Problems, 10th Edition and registered in the national rare diseases database of confirmed patients...

UNLABELLED: Pleuropulmonary blastoma (PPB) is the most common pediatric malignant primary lung tumor. It's associated with the DICER1 gene pathogenic germline variants. Antenatal presentation is infrequent and poses a challenge in the differential diagnosis of congenital pulmonary airway malformation (CPAM). OBJECTIVE: to report a case of unusual presentation of PPB associated with DICER1 syndrome and to describe the difficulty in differentiating it from CPAM. CLINICAL CASE: Male patient with prenatal diagnosis of hypervascular left lung lesion, with mediastinal shift and progressive growth, initially interpreted as CPAM...

Cystic fibrosis (CF) is a multisystem disorder that occurs as a result of autosomal recessive congenital transmission of CF transmembrane conductance regulator (CFTR) gene mutation on chromosome 7. Because it is considered a disease of the Caucasian pediatric population or due to lack of awareness, it is rarely considered in developing countries like ours. This case report presents the first case of cystic fibrosis ever reported in Ethiopia and possibly East Africa, that of a 17-year-old female diagnosed with the disease following a CT scan of her abdomen and chest...

Congenital pulmonary airway malformations (CPAM) compose the major part of congenital lung malformations (CLM) and have traditionally been treated by pulmonary lobectomy. In terms of surgical strategy, lobectomy has conventionally been the preferred treatment for CPAM localized to a single lobe. More recently, alternative approaches including lung-sparing resections (LSR), such as wedge or non-anatomic resections and segmentectomy, have been suggested. In asymptomatic CPAM early surgical resection is often shown to reduce infection and malignancy development...

Objective: To investigate the clinicopathological features and genetic characteristics of congenital cystic adenomatoid malformation (CCAM) of lung and CCAM associated lung cancer in adults. Methods: A total of 13 cases of CCAM of lung in adults, diagnosed from June 2015 to May 2023, were collected from the Department of Pathology, Shanghai Pulmonary Hospital, Tongji University School of Medicine, China. Their histopathological features were correlated with probable development into lung cancer. Next-generation sequencing was performed on the benign and malignant areas of all cases...

BACKGROUND: Congenital pulmonary airway malformation (CPAM) is the most common type of congenital lung malformation, some children were born with serious respiratory and circulatory disorders, these children tend to have larger cystic lesions and emergency surgery should be taken after birth. This article aimed to explore the feasibility of video-assisted thoracic surgery (VATS) for CPAM in infants less than 3 months of age and analyze the early and mid-term postoperative outcomes. METHODS: A retrospective analysis was performed on 12 children with CPAM who were admitted to Shanghai Children's Hospital from January 2019 to December 2022...

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...

BACKGOUND: It is rarely seen that neonates with congenital macrocystic lung malformation (CMLM) presenting with respiratory distress require emergency intervention. No consensus has been achieved concerning the best policy facing such condition. This study aims to evaluate the efficacy and safety of our strategies in treating neonates with CMLM presenting with respiratory distress. METHODS: We retrospectively reviewed the data of six neonates with CMLM presenting with respiratory distress from April 2020 to October 2022 for whom drainage-prior-to-surgery strategy were adopted and favorable outcomes were obtained...

Lymphangioleiomyomatosis (LAM) is a rare systemic disease that typically presents like cystic lung disease. High-resolution computed tomography (CT) is the recommended imaging technique, with cysts being the hallmark: typically multiple, well-circumscribed, thin-walled, with a variable diameter (usually <2 cm) and widespread in distribution. The gold standard for diagnosis is a biopsy. LAM should be considered in the differential diagnosis of cystic lung diseases. The authors report a case of LAM presenting with a pneumothorax, which due to its atypical imaging characteristics, mimicked another uncommon cystic disease...

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: 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...

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...