Congenital Cystic Adenomatoid Malformation (Congenital Pulmonary Airway Malformation)

2010 ◽  
pp. 86-88
Keyword(s):  
Congenital Cystic Adenomatoid Malformation ◽  
Congenital Pulmonary Airway Malformation ◽  
Cystic Adenomatoid Malformation ◽  
Pulmonary Airway

Congenital Pulmonary Airway Malformation (Congenital Cystic Adenomatoid Malformation) with Multiple Extrapulmonary Anomalies: Autopsy Report of a Fetus at 19 Weeks of Gestation

2004 ◽  
Vol 7 (6) ◽  
pp. 661-666 ◽  
Author(s):  
Truc T. Pham ◽  
Kurt Benirschke ◽  
Eliezer Masliah ◽  
J. Thomas Stocker ◽  
Eunhee S. Yi
Keyword(s):  
Surfactant Protein ◽  
Maternal Serum ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Normal Female ◽  
Cytokeratin 20 ◽  
Congenital Pulmonary Airway Malformation ◽  
Thyroid Transcription Factor 1 ◽  
Cystic Adenomatoid Malformation ◽  
Estimated Risk ◽  
Pulmonary Airway

Congenital pulmonary airway malformation, or congenital cystic adenomatoid malformation, is postulated to be a disorder of pulmonary airway morphogenesis and encompasses 5 different types with distinct levels or stages of tracheobronchial development. We present a unique case of type 2 congenital pulmonary airway malformation with a previously undocumented combination of multiple extrapulmonary anomalies, featuring ipsilateral multicystic renal dysgenesis, contralateral renal agenesis, and ovarian germ cell hypoplasia, diagnosed in a 19-week gestational age fetus by autopsy. Epithelial cells comprising the pulmonary lesions were positive for thyroid transcription factor-1, surfactant protein-B, and cytokeratin-7 but negative for cytokeratin-20 immunostainings, with the pattern seen in normal terminal bronchioles. Chromosomal analysis showed a normal female karyotype, despite a high estimated risk for Down syndrome suggested by the low maternal serum α-fetoprotein level.

scholarly journals It is not pneumothorax: Multicystic lung lesion in a six-year-old child

2016 ◽  
Vol 26 (2) ◽  
pp. 121-124
Author(s):  
Eman Sobh ◽  
Amira Bahour ◽  
Shady Elia Anis
Keyword(s):  
Surgical Resection ◽  
Lung Development ◽  
Lung Lesion ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Congenital Pulmonary Airway Malformation ◽  
Cystic Lung ◽  
Cystic Adenomatoid Malformation ◽  
Pulmonary Airway ◽  
Cystic Lung Lesion

Congenital cystic adenomatoid malformation (CCAM) – recently reported as congenital pulmonary airway malformation – is a rare abnormality of lung development. We present a case of a six-year-old child having a cystic lung lesion that proved to be CCAM after surgical resection. The child is performing well on follow-up.

Prenatal Diagnosis and Postnatal Management of Congenital Pulmonary Airway Malformation

2017 ◽  
Vol 8 (2) ◽  
pp. 89-92
Author(s):  
Rema Nambiar ◽  
Dinesh Nayak ◽  
Krupa H Shah ◽  
Shashikala K Bhat
Keyword(s):  
Prenatal Diagnosis ◽  
Lung Lesion ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Congenital Pulmonary Airway Malformation ◽  
Diagnostic Features ◽  
Postnatal Management ◽  
Mediastinal Shift ◽  
Exact Etiology ◽  
Threatened Preterm Labor ◽  
Pulmonary Airway

ABSTRACT Congenital pulmonary airway malformation (CPAM) is a rare developmental lung abnormality. It is also referred as a congenital cystic adenomatoid malformation. The exact etiology is not known yet. The majority of cases are detected during targeted prenatal scan or within 2 years of the birth. Here, we report a case of CPAM type II diagnosed at 23 weeks of gestation, the key diagnostic features of which were mediastinal shift and lung lesion. The fetus had transient polyhydramnios and pericardial effusion. Glucocorticoids were administered for threatened preterm labor and CPAM. Healthy neonate was delivered at term without any immediate neonatal complications. How to cite this article Bhat SK, Nambiar R, Nayak D, Shah KH. Prenatal Diagnosis and Postnatal Management of Congenital Pulmonary Airway Malformation. Int J Infertil Fetal Med 2017;8(2):89-92.

scholarly journals A Missed Late Presentation of a Congenital Pulmonary Airway Malformation as a Large Infected Bulla

2019 ◽  
Vol 18 (4) ◽  
pp. 541 ◽  
Author(s):  
Chung Y. Lee ◽  
Syazarina S. Osman ◽  
Helmee M. Noor ◽  
Nur S. A. Isa
Keyword(s):  
Histopathological Examination ◽  
Thoracoscopic Surgery ◽  
Late Presentation ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Kuala Lumpur ◽  
Congenital Pulmonary Airway Malformation ◽  
Video Assisted ◽  
Chest X Ray ◽  
Pulmonary Airway ◽  
Video Assisted Thoracoscopic Surgery

A congenital pulmonary airway malformation (CPAM) is a rare cystic anomaly that may occur during development of the fetal airways. The vast majority of CPAMs are detected in neonates; as such, it is unusual for diagnosis to occur in adulthood. We report a 21-year-old male patient who presented to the emergency department of the Hospital Ampang, Kuala Lumpur, Malaysia, in 2015 with chest pain, breathlessness and tachypnoea. Based on an initial chest X-ray, the patient was misdiagnosed with pneumothorax and underwent urgent chest tube insertion; however, his condition deteriorated over the course of the next three days. Further imaging was suggestive of infected bullae or an undiagnosed CPAM. The patient therefore underwent video-assisted thoracoscopic surgery, during which a large infected bulla was resected. A diagnosis of an infected CPAM was confirmed by histopathological examination. Following the surgery, the patient recovered quickly and no bullae remnants were found at a one-month follow-up.Keywords: Respiratory System Abnormalities; Congenital Cystic Adenomatoid Malformation of Lung; Adult; Respiratory Tract Infection; Pneumothorax; Video-Assisted Thoracic Surgery; Case Report; Malaysia.

Adenocarcinoma in situ in type I congenital pulmonary airway malformation

2019 ◽  
Vol 27 (7) ◽  
pp. 606-608
Author(s):  
Rajan Santosham ◽  
Rajiv Santosham ◽  
Vamshidhar Tirunagari ◽  
Sheba SK Jacob
Keyword(s):  
Lower Respiratory Tract ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Adenocarcinoma In Situ ◽  
Type I ◽  
Prenatal Period ◽  
Congenital Pulmonary Airway Malformation ◽  
Pulmonary Airway ◽  
Viral Oncogene Homolog ◽  
Adult Presentation

Congenital pulmonary airway malformation, previously known as congenital cystic adenomatoid malformation, is a rare developmental anomaly of the lower respiratory tract. Widespread use of ultrasonography in the prenatal period has increased the rate of early diagnosis of congenital pulmonary airway malformations, thus adult presentation of this condition is extremely unusual. Adenocarcinoma in situ is seen very rarely in adolescent and adult patients with long-standing congenital pulmonary airway malformations. We describe a 28-year-old man with type I congenital pulmonary airway malformation with pulmonary Kirsten rat sarcoma viral oncogene homolog mutation positive mucinous adenocarcinoma in situ.

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