Congenital cystic adenoid malformation

Introduction to congenital cystic adenoid malformation Congenital cystic adenoid malformation (congenital cystic adenoid malformation) is congenital pulmonary local hypoplasia, lung tissue structure disorder, terminal bronchioles overgrowth, forming a polycystic mass. basic knowledge The proportion of illness: 0.001% Susceptible people: infants and young children Mode of infection: non-infectious Complications: edema

Congenital cystic adenoid malformation

(1) Causes of the disease

The disease is a local hypoplasia of the lung, disordered lung tissue, excessive growth of the terminal bronchioles, formation of polycystic immature alveolar tissue, a malformation of intrapulmonary cysts and adenomatous changes, once called For diffuse hamartoma, adenoid malformation, etc., the first case was Cai Guangqi's 1949 report. In 1955, Graham and Singleton's first lobectomy for cystic adenoid malformation was successful.

(two) pathogenesis

The pathogenesis is unknown. The lesion should occur in the embryos 10 weeks before, and can be seen in the leaves of both lungs. The middle and upper lungs are more common, and there are rarely more than one leaf.

Congenital cystic adenoid malformation occurs in the embryo development for about 10 weeks. After the lung leaves are separated and the bronchial cartilage, pulmonary blood vessels, alveoli, mucous glands, etc. have not yet differentiated, they are classified into cystic, solid and intermediate types according to clinical manifestations and pathological features. Type III, type I (cystic): 1 or more large cysts with a small number of small pockets occupying the lungs, the most common type, accounting for 52.7%; type II (intermediate), by Many smaller cysts are composed, the size of the cyst is 1~10mm, accounting for 38.2%; type III (solid): the least, about 9.1%, showing solid mass, no general capsule, cystic More common in full-term newborns and younger children, rarely with other malformations, the lesions are mainly cystic components, mature alveolar cells can be seen, the prognosis is good, the real is seen in the newborn and mature pre-baby, often combined with other malformations Mainly composed of solid components, immature alveolar cells, common mucous epithelium and cartilage, poor prognosis, intermediate histopathology between the two, the prognosis is good.

In general, cystic congenital cystic adenoids, for example, have a significant increase in lung volume and weight, and are cystic. The capsule contains gas and interstitial traffic, but there is no traffic with the bronchus. Microscopic features: 1 respiratory tract The end of the bronchoid-like structure is adenomatous hyperplasia, surrounded by ciliated columnar epithelium, scattered vesicles like immature alveolar, connective tissue matrix with chaotic and disordered elastic fibers and smooth muscle; 2 types of bronchial epithelial capsule The mucosa may have polypoid hyperplasia that protrudes into the cyst; 3 there is a lack of bronchial mucosa gland and cartilage plate in the interstitial space of the cyst; 4 occasional alveolar population may be arranged with mucus secreting cells like intestinal mucosa, but not normal bronchial cells. The distinguishing feature from the hamartoma is that the disease does not contain cartilage and the lesion is pulmonary circulation.

Congenital cystic adenoid malformation prevention

There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.

Congenital cystic adenoid malformation complications Complications edema

The lung tissue structure of this disease is disordered, and the small bronchial tissue overgrows to form a polycystic mass, which can be complicated by mediastinal shift.

Congenital cystic adenoid malformation symptoms Common symptoms Cyst wall is thin and contains... Cystic necrosis, edema, lung infection, dead fetus, excessive water

Neonatal respiratory distress is the main manifestation. A small number of children over one month can present with cough, fever, and repeated lung infections. Half of the fetal period combined with fetal general edema, and amniotic fluid accounts for about 25%, often combined with preterm birth. Or perinatal stillbirth, considering that it may be due to the swelling of the hard and hard lung, causing poor venous return and affecting heart function, which is found in 62% within 1 month after birth, and diagnosed within 5 years after birth. It accounts for 24% and is rarely diagnosed in adults.

Examination of congenital cystic adenoids

There is no co-infection, and the blood is mostly normal.

X-ray showed a large difference between the various cases, generally manifested as intrapulmonary mass with unequal light transmission area, extending to the ipsilateral thoracic cavity, and can compress the mediastinum shift, and even into the contralateral chest cavity, similar to the baby lung lobe Sexual emphysema, but the disease is slightly higher than the cystic density can be identified, the disease is occasionally found in adults, more asymptomatic, often misdiagnosed as hamartoma, but this disease has no cartilage.

Diagnosis and diagnosis of congenital cystic adenoid malformation

Diagnostic criteria:

1. Most of the patients are infants and young children.

2. Clinical manifestations: The main respiratory disorders are accompanied by symptoms such as cyanosis.

3. X-ray performance: The intrapulmonary mass can be diagnosed by spreading the light-transmitting area of different sizes to the ipsilateral thoracic cavity.

It should be differentiated from congenital lung diseases such as pulmonary isolation, cystic fibrosis, and transparent lung. In addition, gas-containing pulmonary cysts should be differentiated from bullous bullae, tuberculosis, and lung abscess.

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