malformaciones pulmonares

EMBRIOLOGÍA Y ANOMALÍAS CONGÉNITAS

PULMONARES.Dr. Héctor Alejandro Domínguez Hernández

residente imagenología, diagnóstica y terapéutica

Hector Dominguez Hernandez

Agujero Laringeo. El pulmón se origina como un diverticulo desde el extremo caudal del surco laringotraqueal del intestino anterior al final de la cuarta semana.

Septum traqueo-esofágicoTabique


1. Diverticulo Laringotraqueal.2.Yema respiratoria. Tabique traque-oesofágico.3.Bronquios principales. El bronquio derecho es mas vertical.4.Bronquios lobulares.5.Bronquios segmentarios.6. Van jalando tejido mesodérmico.

64

16 2828 36

36

166


Pulmonary development involves a series of events closely regulated by genetic and environmental factors.A close interaction between the development of the airways and the blood vessels is present from the embryonic stage.The process of alveolization is not yet completed at birth and continues for the first two years of life.Due to a common embryological origin, anomalies of pulmonary development may be associated with anomalies of the digestive tract. These anomalies have thus been grouped under the term bronchopulmonary-foregut malformations.Certain malformations of the respiratory system are asymptomatic and may be discovered late.

ETAPA PSEUDO GLANDULAR(6-16 SEMANAS)


Poca vascularición.No hay intercambio gaseoso.

ETAPA CANALICULAR(16-28 SEMANAS)


SACOS TERMINALES.VASOS SE ACERCAN


AUMENTA LA LUZ BRONQUIALMAS VASOS SANGUINEOS.24-26 POSIBLE SUPERVIVENCIA24 LOS BRONQUIOS DARAN BRONQUIOLOS RESPIRATORIOS.

ETAPA SACULAR(28 AL NACIMIENTO)


SEGMENTACION MUCHO MAYOR.EPITELIO ADELGAZA MAS VASOS Y MAS CERCANOS A LOS SACOS.BARRRERA SANGRE-AIRE.PROLIFERAN LOS NEUMOCITOS TIPO I Y TIPO II.CELULAS PLANAS PARA DIFUSIÓN OXIGENO.

ETAPA ALVEOLAR(NACIMIENTO A 8 AÑOS)


MAS LUZ.ADHERENCIA DE CAPILARES.NEUMOCITOS TIPO I Y TIPO II

Movimientos Respiratorios Fetales.Cavidad Torácica.Líquido amniótico.

El corazón ocupa del 25 al 30% del volumen torácico.


Transverse US image of a normal fetal thorax demonstrates homogeneous and symmetric intermediate echogenicity of the lungs.The heart occupies 25%–30% of the thoracic volume. The interventricular septum (dashed line) is at a 45° angle with the midline (solid line). Only the right atrium (RA) and a small portion of the right ventricle (RV) are to the right of the midline.


On a coronal T2-weighted MR image of the fetal lungs at 28 weeks gestation, the lungs (L) demonstrate uniform symmetric high signal intensity relative to the chest wall muscles.The signal intensity of the trachea (arrow) and bronchi (arrowheads) is slightly higher than that of the lungs. (See also Fig E4 [online].)


Axial T2-weighted MR image obtained in a fetus at 28 weeks gestation shows normal fetal lungs (L) with uniform symmetric high signal intensity relative to the chest wall muscles. The heart (H) is dark due to flowing blood.

Falta del desarrollo Pulmonar

Agenesia Aplasia Hipoplasia

Bronquio rudimentario.No hay parénquima.

Bronquio y parénquima rudimentario.Alveolos y vasos pulmonares disminuidos en número y tamaño.


Pulmonary aplasia. Unenhanced CT scan shows the main pulmonary artery (MPA) and right pulmonary artery (RPA), but the left pulmonary artery is not seen.

Más del 50% tienen otras anomalías, cardiovasculares (DAP, Persistencia del foramen oval), gastrointestinales (fístula traqueoesofáfica, ano imperforado) genitourinarias y esqueléticas.


Pulmonary aplasia. (a) Frontal chest radiograph depicts the trachea (white arrow) and the right main bronchus (arrowhead); however, the left main bronchus is not seen. There is leftward mediastinal shift. Compensatory hyperinflation of the right middle lobe extending into the left hemithorax is also noted (black arrow). (b) Coronal CT scan shows a blind-ending left main bronchus (arrowhead) with absence of the left lung parenchyma. (See also Fig E5 [online].)

Hipoplasia

Primaria

SecundariaIntratorácica

Extratorácica

Hernia Diafragmática75-90% Izquierda.


Left-sided congenital diaphragmatic hernia.Transverse US image of the fetal chest shows the stomach (arrow) in an intrathoracic position.The heart is shifted to the right due to com- pression by the hernia.


Right-sided congenital diaphragmatic hernia. (a) Transverse US image of the fetal chest shows the liver (Li) within the right hemithorax. The heart (H) is displaced to the left, and there is associated moderate right pleural effusion (arrow) in the anterior chest. (b) Sagittal color Doppler image shows the right hepatic vein (RHV) coursing up through the solid tissue in the thorax, a finding that confirms liver herniation. Arrow indicates the diaphragm. LHV = left hepatic vein, MHV = middle hepatic vein. (c) T2-weighted MR image through the fetal chest and abdomen demonstrates herniation of the liver (Li) into the thorax. Note that there is herniation of the hepatic flexure as well (arrows), a finding that was not identified at US.


hypoplasia include CPAM, bronchopulmonary sequestration, a cardiac or mediastinal mass, lymphatic malformation, and agenesis of the diaphragm.

Hipoplasia

Primaria

SecundariaIntratorácica

Extratorácica Oligohidramnios Severo

Malformaciones genitourinarias.

Ruptura de membranas prolongada.


Pulmonary hypoplasia due to bilateral renal agenesis. (a) Transverse fetal US image shows a small chest with severe oligohydramnios. A small pericardial effusion (calipers) is also seen. (b) Frontal chest radiograph demonstrates bilateral pulmonary hypoplasia due to severe oligohydramnios secondary to bilateral renal agenesis. Note the low lung volumes and the bell-shaped configuration of the thorax.


Pulmonary hypoplasia due to bilateral renal agenesis. (a) Transverse fetal US image shows a small chest with severe oligohydramnios. A small pericardial effusion (calipers) is also seen. (b) Frontal chest radiograph demonstrates bilateral pulmonary hypoplasia due to severe oligohydramnios secondary to bilateral renal agenesis. Note the low lung volumes and the bell-shaped configuration of the thorax.

Síndrome de JeuneDisplasia tanatofórica


El síndrome de Jeune, también llamado distrofia torácica asfixiante, es una displasia con costillas cortas caracterizada por tórax estrecho, extremidades cortas y anomalías esqueléticas radiológicas que incluyen acetábulo en forma de tridente y cambios metafisarios.


Pulmonary hypoplasia secondary to skeletal dysplasia. (a) Transverse US image obtained in a fetus with thanatophoric dysplasia shows a small chest and short ribs (arrows) that cover only 50% of the chest circumference. Normally, the ribs should extend to two-thirds of the chest circumference


Pertenece al grupo de las osteocondrodiplasias de curso siempre letal.

Radio menor de 0.32 es indicativo de hipoplasia pulmonar

Malformaciones Congénitas de la Vía AéreaEl término ha sido recomendado en lugar de

malformación adenomatoidea quística congénita.


ince the lesions are cystic in only three of the five types of these lesions and adenomatoid in only one type


Large cyst CPAM. (a, b) Transverse (a) and coronal (b) fetal US images demonstrate two contigu- ous cystic lesions (calipers) in the left hemithorax, findings that are compatible with a macrocystic CPAM. The heart (H) is shifted to the right. Note that the diaphragm (arrows in b) is intact and the stomach (S) is in the normal position. (c) Postnatal chest radiograph shows a hyperinflated left lung with rightward mediastinal shift. Two large air-filled thin-walled cysts (arrows) are clearly seen in the left lung. (d) CT scan shows multiple cysts in the left lung (arrow), at least two of which are larger than 2 cm, findings that are compatible with a large cyst CPAM. (e) Photograph of a resected specimen from a different patient shows a large multiloculated cyst (large cyst CPAM) (arrow). (f) Photomicrograph (original magnification, ×20; hematoxylin-eosin [H-E] stain) shows the cysts lined by ciliated respiratory epithelium with focal clusters of mucigenic epithelium (arrow).


Small cyst CPAM. (a) CT scan shows a complex cystic mass (arrow) in the left lung. All the cysts are small (<2 cm), a finding that is compatible with a small cyst CPAM. (b) Photograph of the gross specimen shows multiple small cysts and solid areas of abnormal lung parenchyma. (c) Photomicrograph (original magnification, ×10; H-E stain) shows closely apposed bronchiole-like structures adjacent to normal lung tiss


Solid-appearing CPAM. (a) Coronal prenatal US image shows a large hyperechoic lesion (calipers) in the right hemithorax. No cystic areas are identified within the mass. Note that the diaphragm is intact (arrows). (b) Postnatal CT scan shows an ill-defined mass with intermediate attenuation in the right middle lobe (arrow), with an adjacent area of air trapping.The lesion was pathologically proved to represent a CPAM

LESIONES HIBRIDAS


CPAM-sequestration hybrid lung lesion. (a) Transverse US image through the fetal chest shows a hyperechoic mass (calipers) with a cystic area (arrow), findings that represent a CPAM. (b) Color Doppler image reveals a feeding artery (arrowhead) arising from the aorta, a finding that is compatible with sequestration.


CLO. (a) Transverse US image through the fetal chest shows a large hyperechoic mass (arrow) in the right lung. (b) Postnatal CT scan shows organized pulmonary vessels coursing through an overdistended portion of the lung (arrowhead), findings that represent congenital lobar-segmental overinflation. (c) Frontal chest radiograph obtained in a different patient shows a hyperlucent left hemithorax causing rightward mediastinal shift. (d) CT scan shows an overdistended left upper lobe. (e) Photograph of the gross specimen shows focal enlargement of the lung parenchyma. Arrow indicates the interface between the lesion and adjacent normal compressed parenchyma. (f) Photomicrograph (original magnification, ×10; H-E stain) shows dilatation and an increased number of alveoli without maldevelopment (compare with the normal fetal lung in Fig E2 [online]).


Photomicrograph (original magnification, ×10; H-E stain) shows dilatation and an increased number of alveoli without maldevelopment (compare with the normal fetal lung in Fig E2 [online]).


Photomicrograph (original magnification, ×10; H-E stain) of normal fetal lung tissue shows thin-walled alveoli composed of a single layer of squamous epithelium.


Bronchial atresia. CT scan shows mucoid impaction (arrow) just distal to bronchial atresia in the right upper lobe. Distal air trapping is also noted. (Reprinted, with permission, from reference 31.)


Bronchogenic cyst. (a, b) Transverse B-mode (a) and color Doppler (b) US images through the fetal chest show an avascular cystic lesion (arrow) in the left lung adjacent to the ductus arteriosus (arrowhead in a).The lesion was thought to represent either a congenital cystic adenomatoid malformation or a bronchogenic cyst. (c) Contrast material–enhanced postnatal CT scan shows a well-circumscribed unilocular water-attenuation cyst in the middle mediastinum. The cyst has smooth, imperceptible walls with no enhancement and was pathologically confirmed to be a bronchogenic cyst. (d) Photograph of cut sections of a resected bronchogenic cyst specimen shows a translucent cyst. (Courtesy of Ellen M. Chung, LTC, MC, USA, Department of Radiologic Pathology, Armed Forces Institute of Pathology, Washington, DC.) (e) Photomicro- graph (original magnification, ×20; H-E stain) shows a bronchogenic cyst lined by respiratory epithelium and containing cartilage plates, bronchial glands, and smooth muscle within its wall.


Congenital high airway obstruction syndrome. (a, b) Transverse (a) and coronal (b) US images through the fetal chest show enlarged hyperechoic lungs compressing the heart. Note the inversion of the diaphragm (arrow in b) and the dilated trachea and bronchi (arrowheads in b). These findings suggest the presence of congenital high airway obstruction syndrome, likely from tracheal atresia. (c) Photograph of the gross autopsy specimen demonstrates enlarged and edema- tous lungs secondary to tracheal atresia. (d) Photomicrograph (original magnification, ×4; H-E stain) shows an increase in the number and size of airspaces, which are poorly subdivided (compare with the normal fetal lung in Fig E2 [online]).


Lung sequestration. (a) Transverse color Doppler image of the fetal thorax shows a homogeneous echogenic mass (single arrow) in the left lung. A feeding vessel (double arrows) is seen arising from the aorta and supplying the mass. (b) Coronal postnatal CT scan shows a homogeneous mass in the posterior segment of the left lower lobe. A feeding artery (arrow) is seen arising from the aorta, a finding that is diagnostic for sequestration. (c) Intra- operative photograph obtained in a different patient with sequestration shows variable-sized ectopic masses inferior to the lung, the larger of which (*) is seen with a vascular supply (arrow) from the descending aorta.


Scimitar syndrome. (a) Postnatal frontal chest radiograph shows volume loss in the right hemithorax with rightward mediastinal shift. The right heart border is not well seen. An anomalous vessel (arrowheads) is seen in the right cardiophrenic angle. This vessel increases in caliber in the caudal direction (“scimitar sign,” so called because of its resemblance to a Turkish sword). (b) Coronal contrast-enhanced CT scan shows the lower lobe pulmonary vein (scimitar vein) draining into the intrahepatic inferior vena cava (arrows). (c) Volume-rendered CT image clearly depicts the anomalous vein (arrow). (Antenatal US images obtained in the same patient are shown in Fig E9 [online].)

malformaciones pulmonares

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