scholarly journals Hydrops fetalis secondary to supradiaphragmatic extrapulmonary sequestration with congenital cystic adenomatoid malformation

2017 ◽  
Vol 7 (1) ◽  
pp. 1130-1132 ◽  
Author(s):  
S Shukla ◽  
H Kini ◽  
ML Ilias ◽  
K Gautam
Keyword(s):  
Left Lung ◽  
Breech Presentation ◽  
Hydrops Fetalis ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Associated Anomalies ◽  
Male Baby ◽  
Pain Abdomen ◽  
Cystic Adenomatoid Malformation ◽  
Mediastinal Shift ◽  
Fetal Movements

Congenital cystic adenomatoid malformation is a hamartomatous lesion. A 30 year old woman, G2P1L1, in the 29th week of gestation presented with pain abdomen, chest pain, cough with expectoration, fever and inability to appreciate fetal movements of 2 days duration. Clinically, she had pneumonia. An ultrasound revealed a single fetus in breech presentation with features of hydrops fetalis, hypoplastic left lung, mediastinal shift to the left and poor diastolic and systolic flow in the umbilical artery.A still born male baby delivered subsequently was found at autopsy to have hydrops fetalis, supradiaphragmatic right sided extralobar sequestration with associated congenital cystic adenomatoid malformation.The right lung also showed congenital cystic adenomatoid malformation. There were no other associated anomalies. We present a rare case of extralobar sequestrationwith congenital cystic adenomatoid malformation.

scholarly journals Congenital cystic adenomatous malformation: a case report and a literature review

2018 ◽  
Vol 25 (2) ◽  
pp. 95-100
Author(s):  
Julita Račaitė ◽  
Alina Šumkovskaja ◽  
Audronė Arlauskienė ◽  
Ingrida Pilypienė ◽  
Elena Landsbergytė-Bukauskienė
Keyword(s):  
Case Report ◽  
Multiple Pregnancy ◽  
Left Lung ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Chromosomal Anomalies ◽  
X Ray ◽  
Cystic Adenomatoid Malformation ◽  
Mediastinal Shift ◽  
Breathing Therapy ◽  
Caffeine Citrate

Background. A congenital cystic adenomatoid malformation (CCAM) is a foetal pulmonary development abnormality caused by airway dysgenesis that is characterized by cystic or adenomatous lesions in the terminal bronchioles. The size of the mass, the degree of the mediastinal shift, and the presence of hydrops and polyhydramnios can all affect the severity of a case. Treatment can be initiated at early stages by applying prenatal and postnatal methods. Because CCAM is a rare pathology that is often only accidentally diagnosed during routine ultrasounds, we would like to share our case report to enrich the literature on this pathology and to present a case successfully treated at our hospital. Materials and methods. A patient with her first multiple pregnancy was seen for prenatal care and her first ultrasound at 17 weeks of gestation. One of the twins was diagnosed with a congenital cystic adenomatoid malformation of the left lung. At 20 weeks of gestation, an enlarged left lung with small cysts, a compressed right lung, a compressed and displaced heart, and oligohydramnios were observed. At 28 weeks of gestation, a fetoplacental circulation disorder appeared. At 32 weeks of gestation, due the unstable condition of the affected foetus, the twins were delivered via a C-section. The treatment of the newborn included antibiotics, caffeine citrate, and breathing therapy. Results and conclusions. CCAM are often diagnosed by accident when performing routine pregnancy ultrasound examinations. CT is the most reliable X-ray-based examination method for confirming a diagnosis. When CCAM is suspected in the foetus, amniocentesis and cariotype identification are performed, but chromosomal anomalies related to CCAM are often not identified. Currently, the best treatment results have been achieved by applying combined prenatal therapy and early surgical treatment.

Resolution of hydrops fetalis in congenital cystic adenomatoid malformation after prenatal steroid therapy

2003 ◽  
Vol 38 (3) ◽  
pp. 508-510 ◽  
Author(s):  
KuoJen Tsao ◽  
Samuel Hawgood ◽  
Lan Vu ◽  
Shinjiro Hirose ◽  
Roman Sydorak ◽  
...  
Keyword(s):  
Steroid Therapy ◽  
Hydrops Fetalis ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Cystic Adenomatoid Malformation

Congenital Cystic Adenomatoid Malformation Type 4 and Aneurysm of the Vein of Galen: A Rare Coincidence or Possibly Related Association

2004 ◽  
Vol 7 (3) ◽  
pp. 268-272 ◽  
Author(s):  
Erdener Ozer ◽  
Aydin Sanli ◽  
Serhat Erbayraktar ◽  
Cagnur Ulukus ◽  
Ahmat Onen
Keyword(s):  
Case Report ◽  
Later Life ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Vein Of Galen ◽  
Associated Anomalies ◽  
Cystic Adenomatoid Malformation ◽  
First Case ◽  
Embryonic Life ◽  
Associated Malformations ◽  
Related Association

As far as the prognosis is concerned, it is important to diagnose the exact type of congenital cystic adenomatoid malformation (CCAM) in order to exclude associated anomalies, as well as the risk of development of malignancies in later life. The frequency of associated malformations of CCAM type 4 is unknown. We report a 4-month-old boy with CCAM type 4 and aneurysmal malformation of the vein of Galen (AVG). Although AVG is the most frequent arteriovenous malformation during childhood, this is the first case report, to our knowledge, of CCAM type 4 in association with an anomaly. In addition, we speculate that the relation between CCAM type 4 and AVG appears to be more likely a possibly related association rather than a rare coincidence, as both malformations develop at the same stages of embryonic life, and pathogenetically, apoptosis play a significant role in both entities. However, further studies are needed to validate this speculation placing emphasis on the association of the two anomalies, otherwise a rare coincidence cannot be excluded.

scholarly journals An optimistic point in COVID-19 pandemic: a case report of large adult congenital cystic adenomatoid malformation

2021 ◽  
Vol 52 (1) ◽  
Author(s):  
Mohamed rafi Kathar Hussain ◽  
N. Kulasekeran ◽  
A. M. Anand
Keyword(s):  
Case Report ◽  
Cystic Lesion ◽  
Histopathological Examination ◽  
Left Lung ◽  
Lower Lobe ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Unexpected Finding ◽  
Rare Presentation ◽  
Cystic Adenomatoid Malformation ◽  
Large Adult

Abstract Background Covid-19 pandemic is a major health calamity causing global crisis involving every aspect of the society. CT chest has become an essential diagnostic investigation and as a prognostic tool for assessment for COVID-19 bronchopneumonia. This case report is about an incidental unexpected finding in a young female, who underwent CT chest screening with suspicion of COVID-19 bronchopneumonia. Case presentation A 29-year-old female came with the complaints of sore throat, myalgia, and fever for the past 3 days. She was referred to our department for plain screening CT chest to rule out COVID 19 infection. She was an active sports person since childhood. CT chest revealed a large well-defined bullous cystic lesion of size 16 × 9.5 × 9.5 cm in the left lung lower lobe with partial sparing of its superior, anterior, and posterior basal segments. Imaging diagnosis of large bullous cystic lesion with emphysematous changes was made. No features of COVID 19 bronchopneumonia. Thoracoscopy-guided lobectomy was done, and tissue was sent to histopathological examination. Final diagnosis was large type 1 congenital cystic adenomatoid malformation with mucinous metaplasia. Our case is unique in the sense that large adult CCAM with mucinous metaplasia of the epithelium is a rare presentation. Further it was diagnosed as a part of COVID 19 screening. Conclusion CCAM presentation in adult is rare. Asymptomatic CCAM lesion of this size diagnosed during COVID 19 chest CT screening was rarely described.

scholarly journals Congenital cystic adenomatoid malformation: a rare serial case report

2021 ◽  
Vol 9 (1) ◽  
pp. 190
Author(s):  
Dedy C. Haryono ◽  
Muhammad Kartika ◽  
Prima K. Hayuningrat ◽  
Darmawan Ismail
Keyword(s):  
Respiratory Distress ◽  
Lung Resection ◽  
Left Lung ◽  
Chest Ct ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Congenital Lung Malformation ◽  
Cystic Adenomatoid Malformation ◽  
The Right ◽  
Multiple Cysts

Congenital cystic adenomatoid malformation (CCAM) is a rare congenital lung malformation which a part of the lung becomes polycystic. CCAM accounts for 25% of congenital lung malformations and 95% of lung lesions. Case 1 was a 5-month-old female infant who was diagnosed with pneumothorax, with multiple cysts in the right lung, using chest computed tomography (CT). Thoracotomy lung resection was performed. Case 2 was one-day-old newborn infant who had respiratory distress with Downe score 3. Multiple cystic lesions with septations in left lung was observed on chest CT. Lobectomy inferior lobes of left lung was performed. Both patients were diagnosed as CCAM type 1 pathologically. CCAM can be detected in the gestation by ultrasonography or after delivery through the appearance of respiratory distress signs.

Fetal Bronchoscopy as a Useful Procedure in a Case with Prenatal Diagnosis of Congenital Microcystic Adenomatoid Malformation

2014 ◽  
Vol 37 (1) ◽  
pp. 75-80 ◽  
Author(s):  
Rogelio Cruz-Martinez ◽  
Antonio Méndez ◽  
Oscar Perez-Garcilita ◽  
Araceli Monroy ◽  
Karla Aguilar-Vidales ◽  
...  
Keyword(s):  
Prenatal Diagnosis ◽  
Left Lung ◽  
Fetal Surgery ◽  
Lung Hypoplasia ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Complete Disappearance ◽  
Therapeutic Tool ◽  
Mediastinal Shift ◽  
Bronchial Atresia

Massive microcystic congenital cystic adenomatoid malformation (CCAM) and bronchial atresia are associated with a high perinatal mortality secondary to lung hypoplasia and cardiac dysfunction, and fetal intervention should be considered to improve prognosis. Therapeutic options include open fetal surgery with pulmonary resection, fetal sclerotherapy and fetoscopy. We present a case with a severely enlarged left lung without ultrasound signs of dilated airways compatible with the diagnosis of microcystic CCAM, hydrops and severe contralateral lung hypoplasia that was treated successfully at 30 weeks of gestation by fetal bronchoscopy, through which bronchial atresia was identified at the end of the left mainstem bronchi and permeabilized by laser ablation. After fetal surgery, weekly follow-up showed a progressive decrease in the affected lung size and an increase in the contralateral hypoplastic lung size, demonstrating normal dimensions of both lungs at 34 weeks of gestation, reversal of the mediastinal shift, and complete disappearance of hydrops. A healthy neonate was delivered uneventfully at term with no need for respiratory support, and the boy is now doing well at 15 months of age. This report demonstrates that in cases with prenatal diagnosis of large microcystic CCAM, fetal bronchoscopy can be used to refine the diagnosis of bronchial atresia and as a therapeutic tool with good outcome.

scholarly journals The First of Congenital Cystic Adenomatoid Lung Malformation in Iraq: A Radiologic and Pathologic Study

2020 ◽  
Vol 1 (1) ◽  
pp. 01-03
Author(s):  
Aamir Mosawi
Keyword(s):  
Respiratory Distress ◽  
Left Lung ◽  
Rare Condition ◽  
Male Infant ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Lung Field ◽  
Microscopical Examination ◽  
Pulmonary Tissue ◽  
Cystic Adenomatoid Malformation ◽  
The Right

Background: Congenital cystic adenomatoid lung malformation is a very rare condition characterized by replacement of normal pulmonary tissue with cysts of variable size and distribution. The condition is usually unilateral. Very few cases of bilateral and congenital cystic adenomatoid malformation with good outcome after resections of the lesions have been reported, and most cases were stillborn or died early during life. Patients and methods: A forty-day male infant presented with progressive respiratory distress since the first week of life observed at the Children Teaching Hospital of Baghdad Medical City was studied. The literature was reviewed with aim of describing the early documentation of the condition in the literature. Results: Clinically, the infant had significant respiratory distress interfering with feeding and evidence of shift of the mediastinum to the right. Chest X ray showed increased translucency of left lung field and mediastinal shit to the right. CT-scan showed large air-filled cyst in the left lung and small air-filled cyst in the right lung. The child was treated successfully by left upper lobecomy. The gross examination of two gray pieces of lung tissue showed on cut section microcysts that were apparent grossly. Microscopical examination showed intercommunicating cysts lined by cuboidal epithelium with gland like appearance confirming the diagnosis of congenital cystic adenomatoid malformation of the lung. Conclusion: A very rare case of bilateral congenital cystic adenomatoid malformation of the lung is reported and the early documentation of the condition in the literature is described.

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