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Nonimmune Hydrops Fetalis
Published in Vincenzo Berghella, Maternal-Fetal Evidence Based Guidelines, 2022
Chelsea DeBolt, Katherine Connolly, Mary E. Norton, Joanne Stone
Congenital pulmonary airway malformation (CPAM) (previously also called congenital cystic adenomatoid malformation, CCAM), pulmonary sequestration, and congenital diaphragmatic hernia (CDH) are the most common causes of NIH in this category. Other less common causes in this category are lymphangiectasia, bronchogenic cyst, and other thoracic tumors. Congenital high airway obstruction sequence (CHAOS) has also been linked to the development of NIH, as a result of aplasia or intrinsic obstruction of the larynx and/or trachea that occurs during development [21].
Paper 3
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
CPAM, previously known as congenital cystic adenomatoid malformation, is a hamartomatous lesion of the lung most commonly causing a mass composed of multiple large cysts (type I). Detected either antenatally or in neonates with respiratory distress, these lesions initially are fluid filled and then, due to their connection with the bronchial tree, fill with air. Their appearance therefore changes to contain air-fluid levels. types II and III are less common, type II are multiple smaller cysts and type III has microscopic cysts and therefore the lesions appear solid.
Common Tips on Communication
Published in Justin C Konje, Complete Revision Guide for MRCOG Part 3, 2020
Exomphalos may be inherited, especially if it is not associated with chromosome problems. If the chromosomes are normal but there are associated abnormalities, the baby may have a syndrome (a combination of problems). If abnormalities (chromosome or syndrome) are present, then the recurrence risk will depend on these. You will be asked to see a clinical geneticist who will provide more information on recurrence in the next pregnancy. Congenital pulmonary (cystic) airway malformation (CPAM) formerly congenital cystic adenomatoid malformation (CCAM) of the lung
The prenatal ultrasonic character and postnatal follow-up of 227 microcystic and macrocystic congenital cystic adenomatoid malformations
Published in Journal of Obstetrics and Gynaecology, 2021
Yuan Chen, Baihui Zhao, Fangfang Xi, Yayun Wang, Mengmeng Yang, Qiong Luo
Congenital cystic adenomatoid malformation (CCAM), also called congenital pulmonary airway malformation (CPAM), is a well-known developmental abnormality of the lung. Prenatal diagnosis of CCAMs has increased with recent improvements in foetal prenatal imaging and routine foetal anomaly scanning. The prevalence of CCAM in China is 4.01/10000 (1.14/10000–16.09/10000) foetuses (Fan et al. 2017). In Developed countries, such as Canada, the prevalence of CCAM has been reported as 1/25000 to 1/35000 (Laberge et al. 2001). Instead of Stocker’s classification (type 0–4), Adzick’s classification, which simply differentiates antenatally CCAMs into two types: microcystic and macrocystic, is thought to be more appropriate (Cruz-Martinez et al. 2017). Whilst the outcome for most cases of CCAM is good, and the management of foetuses with large lesions, severe polyhydramnios or hydrops in utero remains controversial. Moreover, for asymptomatic lesions, whether the preventive surgical resection is necessary is debating. This study is one of the largest case cohorts identifying the prenatal ultrasound difference of microcystic and macrocystic CCAMs, comparing perinatal outcome with normal foetuses and following up the postnatal results after a comprehensive consultation by a prenatal diagnosis centre. Our study could provide important reference for the clinical management of foetal CCAMs.
Pleuropulmonary blastoma: Difficulty in diagnosis and treatment of a case in Vietnam
Published in Pediatric Hematology and Oncology, 2021
Bui Ngoc Lan, Le Thi Kim Ngoc, Hoang Ngoc Thach, Phan Canh Duy
Pleuropulmonary blastoma (PPB) is a very rare malignant disease in childhood cancer. The tumor is derived from the pleura or lungs. This primary tumor occurs mainly in children under 6 years old.1 Diagnosis of this tumor is difficult both by imaging and by pathology because of its similarity to other diseases. PPB is classified into three groups. Type I (cystic form) is similar to congenital cystic adenomatoid malformation (CCAM).2 This type is commonly seen in infants and has a more favorable prognosis than type II and III. Type I can be treated successfully with surgical resection, however it can relapse and require neoadjuvant chemotherapy. In contrast, type II (mixed form) or type III (solid form) are aggressive diseases with poor prognosis. Type II and III PPB have metastatic potential to the brain, the bone, and in some cases, to the liver. Both these types need multidisciplinary therapy including surgical resection, intensive chemotherapy and radiation if it is available.3 Effective treatment plans are aggressive and require hospitals with the capability to perform all the above. The international PPB protocol was developed in developed countries and application in developing countries is challenged by lack access to required resources.
Invasive mucinous adenocarcinoma of the lung arising in a type 1 congenital pulmonary airway malformation in a 68-year-old patient: a case report
Published in Acta Chirurgica Belgica, 2021
A. E. Frick, H. Decaluwé, B. Weynand, M. Proesmans, D. Van Raemdonck
The development of a congenital parenchymatous lung disorder, known as congenital pulmonary airway malformation (CPAM), has previously been described as congenital cystic adenomatoid malformation (CCAM) with an estimated incidence at 1:25,000–1:35,000 births. The classification by Stocker et al. in 1977 originally described 3 different subtypes and was later expanded and renamed into 5 subtypes on the basis of clinical and pathological features [1–3]. Most congenital CPAMs are uncommon and primarily identified in infants and in adults. Depending on the type of CPAM, clinical presentation and prognosis are different. Typical symptoms are recurrent pulmonary infection, productive cough, and hemoptysis. Some patients remain asymptomatic [4]. A number of reports have been published describing the association of adenocarcinoma with type 1 CPAM. The occurrence of rhabdomyosarcoma or pleuropulmonary blastoma arising in a CPAM has also been rarely documented [5,6].