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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].
Common Tips on Communication
Published in Justin C Konje, Complete Revision Guide for MRCOG Part 3, 2020
Your baby has an abnormality known as congenital pulmonary airway malformation (CPAM). Everyone has five parts to their lungs. These are known as lobes, with two on the left and three on the right. Occasionally a problem occurs during development, resulting in one of the lobes containing fluid space(s) (cysts) rather than lung tissue. These cysts are usually confined to one lobe but may occasionally affect more than one lobe. The cysts can be detected on ultrasound before birth and have various appearances ranging from a small number of large cysts to a large number of small cysts. When this happens, the baby is said to have CPAM, which is what your baby has.
Paediatrics
Published in Vincent Helyar, Aidan Shaw, The Final FRCR, 2017
This is the second most common pulmonary anomaly diagnosed antenatally. It is composed of a non-functioning lung segment with no communication to the bronchial tree and a systemic arterial supply. More common in males. Hybrid lesions composed partly of a sequestration and congenital pulmonary airway malformation (CPAM) can occur (Table 5.7).
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
As reports published before, patients only usually seek treatment after tumors were already large and cause complications. Core needle biopsy should have been performed sooner. Due to various pathology characteristics of PPB, it is required that the samples were taken from different locations on the tumor which can be performed only by core needle biopsy.3 We reviewed the previous pathology and the new pathology to give definite diagnosis for this case. There are two indicators that clinicians should evaluate to ensure that there is not a misdiagnosis in the future. First, patients with recurrent pneumothorax should be referred to experienced radiologists to carefully analyze characteristic of lesions, location, size in lung CT scan. Second, clinicians have to check pathology results when patient are discharged even if they were diagnosing benign disease and to consult experienced pathologists to find any malignant lesions. We now are in contact with some free central pathology review as the KK Children and Woman Hospital in Singapore, and St Jude Children’s Research Hospital in the USA. Moreover, the International PPB/DICER1 Registry provides free central pathology review for any suspected case of PPB or other DICER1-related cancer. We, clinicians and pathologists in our hospital, will send samples of any cases of suspected PPB which include some cases of CCAM, congenital pulmonary airway malformation (CPAM) to review. This resource will hopefully avert early misdiagnosis and reduce risk for progression of disease.
Fetal hydrops – a review and a clinical approach to identifying the cause
Published in Expert Opinion on Orphan Drugs, 2020
Esther Dempsey, Tessa Homfray, John M Simpson, Steve Jeffery, Sahar Mansour, Pia Ostergaard
A congenital pulmonary airway malformation (CPAM) of the lung is a developmental abnormality caused by the failure of maturation of the bronchiolar structures during lung development. This results in overgrowth of terminal bronchioles without alveoli [61]. These are usually identified at the 20-week anomaly scan. CPAMs can be microcystic, macrocystic or a mixed type. In up to 65% of cases, the CPAM will spontaneously regress, possibly due to outgrowing of its blood supply [62]. In a large retrospective study of CPAM affected pregnancies, those cases without fetal hydrops had a 95% chance of survival. Those cases which developed hydrops, and were managed expectantly, had a 95% risk of perinatal death [63].