1,378 results match your criteria Congenital Cystic Adenomatoid Malformation


Clinical characteristics and surgical treatment of congenital cystic adenomatoid malformation in adults: the largest cohort of 46 patients.

Ann Transl Med 2022 May;10(10):596

Department of Thoracic Surgery, West China Hospital, Sichuan University, Chengdu, China.

Background: Congenital cystic adenomatoid malformation (CCAM) is a rare congenital malformation of the lungs, however it lacks a summary of pathognomonic clinical and imaging features in adults. Our study aims to evaluate clinical characteristics and surgical treatment in the largest case series of adult CCAM.

Methods: The records of 46 adult patients with CCAM admitted to West China Hospital between February 2009 and March 2019 were reviewed. Read More

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Diagnostic value of congenital pulmonary airway malformation volume ratio for fetal hydrops due to congenital lung malformations: a systematic review and meta-analysis.

Orphanet J Rare Dis 2022 06 3;17(1):213. Epub 2022 Jun 3.

Department of Epidemiology and Health Statistics, West China School of Public Health and West China Fourth Hospital, Sichuan University, Sichuan, China.

Objective: Meta-analysis was used to evaluate the diagnostic value of a CVR cut-off value of 1.6 for fetal hydrops due to congenital lung malformation (CLM).

Methods: A systematic search of PubMed, Embase, Web of Science, CNKI, VIP, and Wanfang published before 7/30/2021 for the value of a congenital pulmonary airway malformation volume ratio (CVR) cut-off value of 1. Read More

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[Congenital Pulmonary Airway Malformation Diagnosed in Adulthood].

Kyobu Geka 2022 Jun;75(6):432-435

Department of Thoracic Surgery, Aomori Prefectural Central Hospital, Aomori, Japan.

Congenital pulmonary airway malformation diagnosed in adulthood is very rare. A 54-year-old woman was admitted to our hospital with complaints of abnormal chest shadow. Computed tomography( CT) demonstrated multiple cysts, mass lesions, and consolidation in left lower lobe. Read More

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Congenital cystic adenomatoid malformation of the lung: a case report with embryological review.

Anat Cell Biol 2022 May 26. Epub 2022 May 26.

Department of Anatomy, All India Institute of Medical Sciences, Bibinagar, Hyderabad (Telangana), India.

Congenital cystic adenomatoid malformation (CCAM) is a rare developmental dysplastic lesion that affects the fetal bronchial tree. Etiopathogenesis is still poorly understood. Most accepted view is that of abnormal branching of bronchioles during the period of morphogenesis. Read More

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A case of juvenile systemic sclerosis and congenital pulmonary airway malformation related mucinous adenocarcinoma of the lung: paraneoplastic syndrome or just a coincidence?

Turk J Pediatr 2022 ;64(2):394-399

Departments of Pediatric Rheumatology, İstanbul Cerrahpaşa University Faculty of Medicine, İstanbul, Turkey.

Background: Juvenile systemic sclerosis (JSS) is an extremely rarely seen auto-immune disease characterized by the increased fibrosis of skin and internal organs. Congenital pulmonary airway malformation (CPAM) is a developmental disorder of the lung, characterized by atypical cell hyperplasia which creates the ground for lung adenocarcinoma. In general, CPAM is diagnosed in early childhood, due to recurrent respiratory symptoms including cough, hemoptysis and respiratory infections. Read More

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Congenital Cystic Adenomatoid Malformation in an Adult.

Am J Med Sci 2022 Apr 29. Epub 2022 Apr 29.

Department of Radiology, Yantai Affiliated Hospital of Binzhou Medical Universtiy, Yantai, Shandong, China. Electronic address:

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Persistent Pulmonary Interstitial Emphysema With Respiratory Infection: A Clinicopathological Analysis of Six Cases and Detection of Infectious Pathogens by Metagenomic Next-Generation Sequencing (mNGS).

Front Pediatr 2022 7;10:836276. Epub 2022 Apr 7.

Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.

Background: Persistent pulmonary interstitial emphysema (PPIE) is known to be related to mechanical ventilation and preterm. However, PPIE is also reported rarely in non-ventilated and full-term infants. Its relationship with respiratory infection is rarely reported in the literature. Read More

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Acutely presenting congenital chest lesions: a primer for the radiologist.

Emerg Radiol 2022 Jun 7;29(3):557-570. Epub 2022 Mar 7.

Department of Imaging Sciences, Division of Pediatric Radiology, University of Rochester Medical Center, Rochester, NY, 14642, USA.

Congenital chest lesions encompass several important entities. Without prompt intervention, many of these can culminate in serious complications. Timely and accurate radiologic interpretation of these entities is integral to patient management. Read More

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Lambdoid Craniosynostosis in an Infant with Congenital Cystic Adenomatoid Malformation (CCAM).

J Craniofac Surg 2022 Mar 3. Epub 2022 Mar 3.

Division of Plastic Surgery, Children's Hospital of Philadelphia Department of Surgery, Children's Hospital of Philadelphia, Philadelphia, PA.

Abstract: Both congenital cystic adenomatoid malformation (CCAM) and lambdoid craniosynostosis are exceedingly rare, with estimated incidences of around 1 in 30,000 live births for each. Herein, we present a case of a patient with prenatally diagnosed CCAM requiring 4 doses of prenatal steroids and ultimately thoracotomy with right lower lobectomy. The operation was uncomplicated, and genome-wide analysis of SNPs revealed no pathologic abnormalities. Read More

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Multimodality Imaging of Pleuropulmonary Blastoma: Pearls, Pitfalls, and Differential Diagnosis.

Semin Ultrasound CT MR 2022 Feb 15;43(1):61-72. Epub 2021 May 15.

Department of Medical Imaging, A. I. DuPont Hospital for Children, Wilmington, DE.

Pleuropulmonary blastomas are rare, potentially aggressive embryonal cancers of the lung parenchyma and pleural surfaces that account for 0.25%-0.5% of primary pulmonary malignancies in children. Read More

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February 2022

Congenital lung malformation patients experience respiratory infections after resection: A population-based cohort study.

J Pediatr Surg 2022 May 13;57(5):829-832. Epub 2022 Jan 13.

University of Manitoba, Department of Surgery, Division of Pediatric Surgery and Children's Hospital Research Institute of Manitoba, Winnipeg, MB, Canada. Electronic address:

Purpose: The benefit of elective resection of congenital lung malformations continues to be debated. Proponents of resection endorse a decreased risk of respiratory complications as one indication for surgery. Our study aimed to compare the prevalence of respiratory infections in cases, before and after resection of congenital lung malformations, to controls without a history of congenital lung malformation. Read More

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Use of Corticosteroids in Prenatal Treatment of Congenital Pulmonary Adenomatoid Malformation: Integrative Review.

Rev Bras Ginecol Obstet 2022 Mar 2;44(3):304-310. Epub 2022 Feb 2.

Fetal Medicine Program, Department of Gynecology and Obstetrics, Women's Hospital, University of Campinas, Campinas, SP, Brazil.

Objective:  To review data on the use of corticosteroids for the treatment of fetuses with high-risk congenital pulmonary adenomatoid malformation (CPAM).

Methods:  Integrative review based on the literature available on MEDLINE and LILACS, including articles published until November, 2020.

Results:  The initial search resulted in 87 articles, 4 of which were selected for analysis, with all of them being retrospective descriptive observational studies. Read More

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Global interpretation of novel alternative splicing events in human congenital pulmonary airway malformations: A pilot study.

J Cell Biochem 2022 04 22;123(4):736-745. Epub 2022 Jan 22.

Department of Pediatric Surgery, The Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, China.

Little is known about differentially expressed genes (DEGs) and alternative splicing (AS) landscapes in congenital lung malformations (CLMs). We applied reference-based assembly of sequencing reads from RNA sequencing (RNA-seq) libraries to identify DEGs and AS landscapes in the lesions and normal lung tissue from the most common types of CLMs, including congenital pulmonary airway malformation-Ⅰ (CPAM-Ⅰ), CPAM-Ⅱ, intralobar sequestration (ILS), and ILS with CPAM (ILS-CPAM). We analyzed the expression profiles and related biological functions of AS events (ASEs). Read More

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[Congenital Malformations of the Lung - an Overview].

Zentralbl Chir 2022 Feb 19;147(1):90-97. Epub 2022 Jan 19.

Klinik für Thoraxchirurgie, Universitätsklinikum Freiburg, Medizinische Fakultät, Albert-Ludwigs-Universität Freiburg, Freiburg, Deutschland.

Congenital pulmonary malformations comprise a heterogenous group of rare developmental diseases. The most common malformations are the tracheal bronchus, bronchial atresia, bronchogenic cyst, pulmonary sequestration, congenital lobar emphysema, and congenital pulmonary airway malformation. Due to their space-consuming effect, patients suffer early postnatal respiratory distress which generally requires immediate surgical resection. Read More

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February 2022

18FDG PET-Positive Congenital Pulmonary Airway Malformation Mimicking Lung Cancer.

Clin Nucl Med 2022 Apr;47(4):357-358

Division of Pathology, National Hospital Organization Kochi Hospital, Kochi, Japan.

Abstract: Adult cases of type 2 congenital pulmonary airway malformation (CPAM) are extremely rare, and no PET/CT findings have been reported for CPAM. We encountered a case of 18FDG PET-positive CPAM mimicking lung cancer in a 45-year-old asymptomatic man. CT revealed a large cavitary mass in the left lower lobe. Read More

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Neonatal congenital pulmonary airway malformation associated with mucinous adenocarcinoma and KRAS mutations.

J Pediatr Surg 2021 Dec 25. Epub 2021 Dec 25.

Departments of Paediatric Surgery, Kings College Hospital, London, UK SE5 9RS. Electronic address:

Aim Of The Study: Congenital pulmonary airway malformation (CPAM) has an estimated prevalence in Europe of 1.06/10,000 live births with most being detected using maternal ultrasound screening. Malignant transformation is a possible complication though its prevalence is unknown and previous reports have usually been in older children. Read More

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December 2021

A national descriptive epidemiologic analysis on congenital pulmonary airway malformation in China, 2010-2019.

Pediatr Pulmonol 2022 03 11;57(3):674-681. Epub 2022 Jan 11.

National Center for Birth Defects Monitoring, West China Second University Hospital, Sichuan University, Chengdu, Sichuan, China.

Objective: To depict the epidemiological features of congenital pulmonary airway malformation (CPAM) in Chinese population.

Methods: Using 2010-2019 data from the Chinese Birth Defects Monitoring Network, we calculated the prevalence rates (PR) and 95% confidence intervals of isolated and nonisolated CPAM according to birth year, infant sex, maternal age and residence area, and examined the secular trends by using Poisson regression models. The perinatal outcomes of affected infants and associated malformations with CPAM were also analyzed. Read More

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Quantitative CT imaging analysis to predict pathology features in patients with a Congenital Pulmonary Airway Malformation.

J Pediatr Surg 2021 Oct 14. Epub 2021 Oct 14.

Department of Pediatric Surgery, Erasmus Medical Center, Sophia Children's Hospital, Molewaterplein 40. 3015 GD Rotterdam, Mailing address: Postbus 2060, Rotterdam 3000 CB, the Netherlands. Electronic address:

Background: Risk for infection and potential malignant degeneration are the most common arguments for resecting asymptomatic Congenital Pulmonary Airway Malformations (CPAM). We aimed to investigate if CT- imaging characteristics can be used to predict histopathological features, by using an objective quantitative CT scoring method.

Methods: Archival CPAM tissue samples were histologically re-assessed and patients who had a pre-operative volumetric CT-scan were included. Read More

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October 2021

Difficulties in the differential diagnosis of large solitary pulmonary cysts.

Interact Cardiovasc Thorac Surg 2022 06;34(6):1157-1159

Division of Thoracic Surgery, Department of Surgery, Medical University of Vienna, Vienna, Austria.

Large solitary cystic lesions are a rare finding, and their differential diagnosis includes cystic airspaces associated with lung cancer, congenital pulmonary airway malformations and pneumatoceles. Here, we report 3 consecutive patients who presented with a large solitary pulmonary cyst on chest computed tomography. All underwent surgical resection, and the histopathological findings were different in all 3 cases. Read More

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Congenital lung lesions: a radiographic pattern approach.

Pediatr Radiol 2022 Apr 30;52(4):622-636. Epub 2021 Oct 30.

Division of Pediatric Radiology, Department of Radiology, NYU Grossman School of Medicine, 550 First Ave., New York, NY, 10016, USA.

Congenital lung malformations represent a spectrum of abnormalities that can overlap in imaging appearance and frequently coexist in the same child. Imaging diagnosis in the neonatal period can be challenging; however, the recognition of several archetypal radiographic patterns can aid in narrowing the differential diagnosis. Major radiographic archetypes include (1) hyperlucent lung, (2) pulmonary cysts, (3) focal opacity and (4) normal radiograph. Read More

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Congenital Pulmonary Airway Malformation (CPAM): A Case Report, Jimma University Medical Center, Southwest Ethiopia.

Ethiop J Health Sci 2021 Jul;31(4):907-910

Department of Pediatrics and Child Health, Jimma University, Ethiopia.

Background: Congenital pulmonary airway malformation is a very rare congenital cystic lung disease that presents in 0.004% of all pregnancies and constitutes <25% of all congenital pulmonary anomalies in children. Respiratory distress is a major concern in these patients. Read More

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Thoracoscopic Lobectomy in Infants and Children.

J Laparoendosc Adv Surg Tech A 2021 Oct 4;31(10):1157-1161. Epub 2021 Oct 4.

Department of Pediatric Surgery, Rocky Mountain Hospital for Children, Denver, Colorado, USA.

Indications for pulmonary lobectomy in infants and children include cystic pulmonary adenomatoid malformation, congenital lobar emphysema, chronic infection, and malignancy. These procedures can now all be done thoracoscopically avoiding the short- and long-term morbidity of an open thoracotomy. In this article we describe the technique of thoracoscopic lobectomy as well as the preoperative and postoperative care. Read More

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October 2021

Congenital pulmonary airway malformation (cpam) mimicking an spontaneous pneumothorax in a newborn.

Cir Pediatr 2021 Oct 1;34(4):207-210. Epub 2021 Oct 1.

Pediatric Surgery Department. La Paz Children's University Hospital. Madrid.

Introduction: Patients with congenital pulmonary airway malformation (CPAM) are usually asymptomatic, but some may present with respiratory distress. We report a rare presentation of a CPAM as an image compatible with persistent and localized spontaneous pneumothorax.

Case Report: A 2-month-old male infant without prenatal diagnosis, postnatal distress or barotrauma, was admitted with acute respiratory symptoms and a right tension pneumothorax on chest X-ray. Read More

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October 2021

In-utero treatment of prenatal thoracic abnormalities by thoraco-amniotic shunts, short and long term neuro developmental outcome: A single center experience.

J Pediatr Surg 2022 Mar 4;57(3):364-368. Epub 2021 Sep 4.

The institute of obstetrical and gynaecological imaging, Department Obstetrics and Gynecology, The Chaim Sheba Medical Center at Tel Hashomer, Tel-Aviv University, Ramat Gan, 5262000, Israel; Sackler School of Medicine, Tel Aviv University, Israel. Electronic address:

Background: Severe primary fetal hydrothorax (PFH) and fetal lung lesions (FLL) such as congenital pulmonary airway malformation (CPAM) and Bronchopulmonary sequestration (BPS) are often treated by thoraco-amniotic shunt (TAS).

Objectives: To compare short and long-term outcome of fetuses treated by TAS due to FLL to those treated due to PFH.

Method: A retrospective analysis was performed for all fetuses treated by TAS, between the years 2004-2015, evaluating the short and long term neurodevelopmental outcome. Read More

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Lung Isolation in an Infant With Congenital Cystic Adenomatoid Malformation: Novel Indication for Microcuffed Tube.

AANA J 2021 Oct;89(5):429-433

is employed by the Department of Pediatric Surgery, All India Institute of Medical Sciences.

Congenital cystic adenomatoid malformation is a rare malformation of the lungs, which can pose complex anesthetic challenges because of the pathology and the frequent need for one-lung ventilation. Large lesions can rapidly enlarge, leading to a mediastinal shift, cardiovascular compromise, and pneumothorax. The most common choice for one-lung ventilation in young infants is endobronchial intubation of the non-operative lung using a conventional uncuffed endotracheal tube. Read More

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October 2021

Routine betamethasone therapy of non-hydropic fetuses with CPAM - the way to improve perinatal outcome?

Bratisl Lek Listy 2021 ;122(9):657-662

Authors discuss novel approach to the management of fetal congenital pulmonary cystic malformation (CPAM) and possible benefit of routine administration of betamethasone, which is currently recommended only for severe cases. The article presents authors' own experience with antenatally diagnosed CPAM and describes 4 cases of prenatally diagnosed CPAM without hydrops treated by two doses of betamethasone at 21-31 weeks of gestation with the aim of improving the perinatal prognosis by effect on not only mortality but also postnatal morbidity. Article also summarizes current knowledge on all aspects of the prenatal CPAM focusing on its treatment options. Read More

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September 2021

A rare case of congenital pulmonary airway malformation in a 14-year-old male presenting with spontaneous pneumothorax.

Ann Med Surg (Lond) 2021 Aug 5;68:102692. Epub 2021 Aug 5.

Damascus University, Damascus, Syrian Arab Republic.

Introduction: and importance: Congenital pulmonary airway malformation (CPAM), formerly known as congenital cystic adenomatoid malformation (CCAM), is a rare developmental dysplatic lesion of the fetal tracheobronchial tree. It accounts for approximately 25 % of all congenital lung malformations. It is usually unilateral and involves one lobe with no significant gender or racial predilection. Read More

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Obstructive shock in pediatric patient with congenital cystic adenomatoid malformation: A case report.

Ann Med Surg (Lond) 2021 Aug 28;68:102614. Epub 2021 Jul 28.

Department of Child Health, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr. Sardjito Hospital, Yogyakarta, 55281, Indonesia.

Introduction: and importance: Congenital cystic adenomatoid malformation (CCAM) is a rare cystic lesion in the lungs. CCAM might present in the early neonatal period with symptoms of respiratory distress.

Case Presentation: A 2-year-old girl was admitted to our Pediatric Intensive Care Unit with signs of severe respiratory distress. Read More

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Anesthetic considerations in patients with cystic pulmonary adenomatoid malformations.

J Anaesthesiol Clin Pharmacol 2021 Apr-Jun;37(2):146-152. Epub 2021 Jul 15.

Department of Paediatric Surgery, Maulana Azad Medical College, New Delhi, India.

Congenital pulmonary adenomatoid malformation (CPAM) is a rare entity. The authors searched the US National Library of Medicine Database, EMBASE, Google Scholar, PubMed Central for anesthetic management in CPAM. The search was performed using the terms: congenital cystic adenomatoid malformation, congenital pulmonary adenomatoid malformation, CCAM, CPAM, anesthetic management. Read More

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