Hipoplasia pulmonar en el adulto: embriología, presentación clínica y métodos diagnósticos. Experiencia propia y revisión de la literatura

Comet, R.; Marín, A.; Sans, J.; Domingo, C.

doi:10.1016/S0300-2896(15)30505-6

Archivos de Bronconeumología

ISSN: 0300-2896

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Vol. 34. Issue 1.

Pages 48-51 (January 1998)

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Pages 48-51 (January 1998)

DOI: 10.1016/S0300-2896(15)30505-6

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Hipoplasia pulmonar en el adulto: embriología, presentación clínica y métodos diagnósticos. Experiencia propia y revisión de la literatura

Pulmonary hypoplasia in adults: embryology, clinical presentation and diagnostic methods. Our experience and review of the literature

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R. Comet, A. Marín, J. Sans, C. Domingo*

Servicio de Neumología. Consorci Hospitalari del Pare Taulí. Sabadell

R.M. Mirapeixa, E. Castañerb

a Departament de Ciéncies Morfológiques. UAB. Bellaterra. Barcelona

b Servicio de Radiología. Consorci Hospitalari del Pare Taulí. Sabadell

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Presentamos 5 pacientes diagnosticados de hipoplasia pulmonar (HP) en la edad adulta (edad > 18 años); el rango de edad en el momento del diagnóstico fue de 45-67 años. La HP fue izquierda en 4 casos y las malformaciones asociadas fueron: 2 agenesias renales, 2 hernias diafragmáticas y una hernia hiatal. El funcionalismo respiratorio mostró: FEV1 0,88±0,32 1; FVC 1,30±0,52 1; FEV1/FVC 68,5±5%. La gasometría arterial (con Fi02 0,21) fue de: Pa02 63,6±24; PaC02 47,1±11,2mmHg. Tres casos fueron remitidos para control de su insuficiencia respiratoria (dos precisaron oxigenoterapia domiciliaria) y 2 casos para estudio de radiografía de tórax patológica. El diagnóstico se realizó mediante tomografía computarizada (TC) torácica. Las enfermedades congénitas debidas a la detención del desarrollo pulmonar en la fase seudoglandular son raramente diagnosticadas en el adulto (hemos recogido 48 casos -incluyendo los actualespublicados en la literatura), siendo la HP muy infrecuentemente diagnosticada (posiblemente por atribuirse los cambios observados en la radiografía a procesos infecciosos antiguos). La clínica es muy variable, y depende en gran medida del hábito tabáquico y de las infecciones respiratorias de repetición. La TC torácica es hoy día la técnica diagnóstica de elección.

Palabras clave:

Hipoplasia pulmonar en adultos

Malformaciones asociadas

Tomografía computarizada torácica

We report five patients diagnosed of pulmonary hypoplasia (PH) in adulthood (age > 18 years, range 45 to 67 years). PH was left-sided in four cases. The malformations found were 2 cases of renal agenesis, 2 diaphragmatic hernias and 1 hiatal hernia. Lung function data were FEV1 0.88±0.32 L; FVC 1.30±0.52 L; FEV1/FVC 68.5±5%. Arterial gases (with Fi02 0.21) were PaO2 63.6±24 and PaC02 47.1±11.2mmHg. Three cases were referred for monitoring of respiratory insufficiency (2 requiring home oxygen therapy) and two for radiographic study of chest disease. The diagnoses were made by computerized tomography (CT) of the chest. Congenital diseases due to underdeveloped lungs in the pseudoglandular phase are rarely diagnosed in adults (48 cases, including ours, have been found in the literature), and PH is rarely diagnosed, possibly because the anomalies observed are attributed to old infections. Clinical presentation is highly variable, depending in large measure on a history of smoking and repeated respiratory infections. Chest CT is at present the diagnostic tool of choice.

Key words:

Adult pulmonary hypoplasia

Associated malformations

Computed tomography, chest

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