What Is Congenital Cystic Adenomatoid Malformation?

Congenital cystic adenoid malformation refers to partial hypoplasia of the lung, disordered lung tissue structure, and excessive growth of terminal bronchioles to form polycystic immature alveolar tissue. It is a mixture of intrapulmonary cysts and adenoma-like changes This kind of malformation was once called diffuse hamartoma, adenoid deformity, etc. The clinical manifestations are mainly respiratory disorders accompanied by cyanosis. Graham and Singleton successfully performed lobectomy for the first time in 1955 to treat cystadenoid deformities.

Basic Information

English name: congenital cystic adenomatoid malformation
Visiting department: surgical
Multiple groups: Newborns and children

Common causes: Local hypoplasia of lungs, structural disorder of lung tissue, terminal bronchioles overgrowth, polycystic immature formation
Common symptoms: Fever, chest pain, cough, and paroxysmal lung infections

Causes of Congenital Cystic Adenoid Deformity

The disease is characterized by partial hypoplasia of the lung, disordered lung tissue structure, and excessive bronchiole growth, resulting in polycystic immature alveolar tissue. It is a deformity in which pulmonary cysts and adenoma-like changes are mixed.

Clinical manifestations of congenital cystic adenoid malformations

The disease often accumulates all lung lobes and is more common in the right lower lobe. There are three types of clinical manifestations;

1. Stillbirth or perinatal death. About half of the fetuses have general edema, and 1/4 of pregnant women have too much amniotic fluid, often prematurely or stillbirth.

2. There are progressive respiratory distress and cyanosis in the neonatal period, which are mostly caused by the development of pulmonary disease to emphysema, and most patients die during the neonatal period.

3. A few children do not show symptoms until childhood, and they often have fever, chest pain, cough, and paroxysmal lung infections. Occasionally, a few patients have no clinical symptoms and are only found during X-ray examination or autopsy.

Examination of congenital cystic adenoids

Laboratory inspection

There were no co-infection and most of the blood was normal.

2. Other auxiliary inspections

X-rays behave differently. Usually manifested as intrapulmonary masses with light transmitting areas of varying sizes, which expand to the ipsilateral thorax and compress the mediastinal displacement, and even hernias enter the contralateral thorax, like infantile lobar emphysema.

Differential diagnosis of congenital cystic adenoids

This disease should be distinguished from congenital lung diseases, such as pulmonary isolation, pulmonary cystic fibrosis, and clear lungs. In addition, air-containing pulmonary cysts should be distinguished from bullae, tuberculosis, and lung abscesses.

Treatment of congenital cystic adenoids

After diagnosis, surgery should be performed. Local resection is feasible for patients with substantial disease, and is limited to one lobe for lobectomy, and multilobar lesions for one side of the lung. During the operation, attention should be paid to the treatment of abnormal blood vessels from the systemic circulation. Extensive bilateral lesions are prohibited from surgery.