Congenital cystic adenomatoid malformation encompasses a series of cystic malformative lesions characterized by aberrant bronchiolar formations of varying size and dispersion. Most cases of this illness are detected in the first few years of life, usually affecting infants. We report a case of CCAM presenting as pleural effusion in a 15-month-old boy who presented with acute respiratory distress. Chest CT revealed a cystic image in the right lower lobe of the lung. The patient had surgical excision, and a pathological examination validated the diagnosis of CCAM type 1 with no malignant material. Following surgery, the patient\'s general condition improved, and no new respiratory symptoms were observed during an 8-month follow-up period. Increased awareness of this rare condition among pediatricians and radiologists is crucial for facilitating early diagnosis and appropriate treatment.

BACKGROUND: Congenital pulmonary airway malformations (CPAM) are a spectrum of cystic and non-cystic anomalies arising from abnormal airway development in utero, with an incidence of 1 in 25,000 to 35,000 births. CPAM can present prenatally or postnatally with respiratory distress, recurrent infections, or occasionally as an incidental finding. This case series aims to highlight the clinical, radiological, and histopathological characteristics of CPAM through three pediatric cases, which include types 1, 2, and 3 CPAM.
METHODS: Case 1: A four-month-old male presented with cough, cold, and respiratory distress. Imaging revealed hypoattenuation and overinflation of the left upper lobe with mediastinal shift. Left upper lobectomy confirmed CPAM type 2. Case 2: A one-month-old female presented with recurrent respiratory distress and infections. Imaging showed a large cystic lesion in the right middle lobe. Right middle lobectomy confirmed CPAM type 3. Case 3: A two-month-old male presented with dyspnea. Imaging showed a large bullae in the right chest with mediastinal shift. Right upper lobectomy confirmed CPAM type 1.
UNASSIGNED: CPAM is a rare congenital lung malformation characterized by abnormal bronchial development and localized glandular overgrowth. Management involves surgical resection, with the timing of surgery dependent on symptomatology. Early identification and intervention are crucial for preventing complications such as pulmonary hypoplasia and recurrent infections. Histopathological examination post-resection is essential for accurate classification and management of CPAM.
CONCLUSIONS: This case series provides valuable insights into the clinical, radiological, and histopathological features of CPAM, including the types encountered in each case. It underscores the importance of early diagnosis and timely surgical intervention, advocating for routine prenatal and postnatal screenings to effectively identify and manage CPAM.

Anomalies of the fetal chest require advanced imaging with ultrasound and MR imaging as well as expertise on the part of the interpreting pediatric radiologist. Congenital diaphragmatic hernia and congenital lung malformation are the most frequently seen, and in both conditions, the radiologist should provide both detailed anatomic description and measurement data for prognostication. This article provides a detailed approach to imaging the anatomy, in-depth explanation of available measurements and prognostic value, and keys to identifying candidates for fetal intervention. Less common congenital lung tumors and mediastinal and chest wall masses are also reviewed.

UNASSIGNED: Congenital pulmonary airway malformation (CPAM) is a rare benign deformity of the lungs in adults. Our study aimed to evaluate the clinical features and compare the effect of thoracoscopic lobectomy and wedge resection for adult CPAMs.
UNASSIGNED: This was a retrospective study including eighteen adults with CPAMs recruited between 2013 and 2023. Radiological scans and pulmonary function test (PFT) were performed before operation. All the patients were treated with thoracoscopic approach, which were categorized into groups of lobectomy and wedge resection. The baseline, preoperative, and operative data were evaluated and analyzed.
UNASSIGNED: Four males and fourteen females were diagnosed with CPAMs at a median age of 57.5 years. Cough was the main symptom, reported by 55.6% of the patients. CPAMs were always initially misdiagnosed as other conditions due to heterogeneous computed tomography (CT) characteristics. The mean of PFT results showed normal (>80% predicted) in forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), and FEV1 to FVC ratio (FEV1/FVC), but less than 70% predicted in forced expiratory flow (FEF) at 25-75%, 50% and 75% of FVC. All patients underwent video-assisted thoracic surgery (VATS) with a total of nine wedge resections and nine lobectomies. Age at surgery varied statistically between the two groups. Whereas, duration of surgery, blood loss, postoperative drainage, days of drainage, days of hospitalization and postoperative complications showed no statistical difference between the two groups. There were 27.8% of the lesions showing CPAMs mixed with other diseases during histological evaluations.
UNASSIGNED: CPAM in adults showed a complex presentation in terms of clinical symptoms, imaging performance and pathological findings. Half of the patients were detected with small airway dysfunction preoperatively. Thoracoscopic lobectomy and wedge resection for the treatment can achieve satisfactory short-term outcomes.

In this article, we describe a newborn with Prune belly syndrome who presented with left ventricular dilation due to an extensive intralobar sequestration of the left lung. Pulmonary sequestration was combined with congenital cystic adenomatoid malformation and also had coarctation of the aorta. Percutaneous closure of the anomalous aberrant artery feeding the sequestrated lung and balloon angioplasty for coarctation resulted in prompt regression of the left ventricular enlargement in the catheterization lab.

BACKGROUND: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.

Congenital cystic lesions of the lung are rare. The most common congenital anomaly among them, involving the lower respiratory tract, is congenital cystic adenomatoid malformation (CCAM), currently referred to as congenital pulmonary airway malformation (CPAM). They may be incidentally detected on prenatal or postnatal imaging. They usually present within two years of life, rarely in adults. CPAMs may be asymptomatic at birth. Some may opt for observation alone due to lack of evidence on the incidence of long-term complications. However, rarely has a CPAM remained asymptomatic throughout life and complications eventually develop. Pneumonia is most common, which is not amenable to medical treatment alone. CPAMs are notorious for their known malignant potential and they may also lead to pneumothorax, hemoptysis and hemothorax. Computed Tomography Thorax is the investigation of choice. Surgical resection is known to be safe and is the mainstay of treatment. For patients who are diagnosed prenatally, surgery is recommended at 3 to 6 months, so that compensatory lung growth can occur. A five-month old boy from Bhutan, with cough, intermittent fever, respiratory distress and a history of recurrent lower respiratory tract infections, was diagnosed with CPAM, complicated by pneumonia and underwent surgery at our centre, following optimal medical management viz. antibiotics and supplemental oxygen. The post-operative course was relatively uneventful and he was discharged from hospital in 7 days.

Thoracic trauma is commonly encountered in daily clinical practice; however, blunt thoracic trauma in patients with congenital cystic adenomatoid malformation (CCAM) is rare. CCAM rupture denotes a broad spectrum of manifestations on imaging and may be misdiagnosed as many other conditions. Consequently, this leads to inaccurate treatment and poor patient outcomes. We report the case of a girl with the initial diagnosis of a cavitary lung lesion that was likely a traumatic pulmonary pseudocyst or CCAM. The patient received medical therapy for 20 days; however, her condition did not improve. Subsequently, she underwent right lower lobectomy. Ruptured CCAM was confirmed during surgery and with histopathology. No complications occurred postoperatively, and the patient made a good recovery.

OBJECTIVE: Insertion of a thoraco amniotic shunt (TAS) during fetal life is a therapeutic option where there is a high risk of death secondary to large congenital lung malformations (CLM). The aim of this study is to present our center\'s long-term experience.
METHODS: Retrospective single center review of the period (Jan 2000-Dec 2020). We included all fetuses that underwent TAS insertion for CLM with detailed analysis of those live newborns managed in our center. Data are quoted as median (range).
RESULTS: Thirty one fetuses underwent 37 TAS insertions at a 25 (20-30) weeks gestational age. This was successful on 1st attempt in 30 (97%) fetuses. In 6 cases a 2nd shunt was required at 6.5 (2-10) weeks following the 1st insertion. Twenty-eight survived to be born. Sixteen (9 male) infants were delivered in our center at 39 (36-41) weeks gestational age and birth weight of 3.1 (2.6-4.2) kg. All infants underwent surgery at 2 (0-535) days (emergency surgery, n = 9; expedited n = 4; elective surgery, n = 3). Final histopathology findings were CPAM Type 1 (n = 14, n.b. associated with mucinous adenocarcinoma, n = 1), CPAM Type 2 (n = 1) and an extralobar sequestration (n = 1). Postoperative stay was 16 (1-70) days with survival in 15/16 (94%). One infant died at 1 day of life secondary to a combination of pulmonary hypoplasia and hypertension. Median follow up period was 10.7 (0.4-20.4) years. Nine (60%) children developed a degree of chest wall deformity though none have required surgical intervention. Clinically, 14/15 (93%) have otherwise normal lung function without limitations of activity, sporting or otherwise. One child has a modest exercise limitation (FVC - 70% predicted).
CONCLUSIONS: TAS insertion is associated with high perinatal survival and should be considered in fetuses at risk of hydrops secondary to large cystic lung malformation. Their long term outcome is excellent although most have a mild degree of chest wall deformity.

Pectus excavatum, carinatum, and arcuatum are 3 developmental chest wall deformities that may evolve during childhood and cause cardiac and/or pulmonary compression. Evaluation may include nonsurgical subspecialty consultations and imaging studies. Treatment may be nonoperative or surgical. Long-term follow-up studies have identified rare complications of traditional open repair. Routine in utero ultrasonography has led to increasing identification of congenital lung anomalies, including congenital cystic adenomatoid malformations, pulmonary sequestrations, and bronchogenic cysts. Short-term follow-up studies have suggested that some lesions may regress spontaneously. Minimally invasive techniques, including thoracoscopy, may allow for early surgical resection with less morbidity than traditional open surgery.