Abstract Ref Number = APCP1216
Case report Pulmonary Hypoplasia in Children
Tika Ermawati,Diah Asri Wulandari
medical faculty of Pediatric Padjadjaran University
Background : Pulmonary hypoplasia characterized by the number and size of airways, vessels and alveoli are decreased. Clinical manifestations of pulmonary hypoplasia in children depend on the extent of the lung involved and other comorbidities. Pulmonary hypoplasia is one of the conditions that can cause respiratory distress and has a high mortality rate. Pulmonary hypoplasia may be primary (idiopathic) or secondary. Primary pulmonary hypoplasia is very rare and very lethal. The incidence is 9-11 of 10,000 live births. The mortality rate of pulmonary hypoplasia can reach 71-95%. The cause of primary hypoplastic disorders can be idiopathic or associated with the syndrome. Secondary pulmonary hypoplasia is associated with diaphragmatic abnormalities, central nervous system lesions or neuromuscular disorders, or congenital heart disease.
Case Presentation Summary : A boy, 4 months old came to the hospital with a chief complaint of shortness of breath since 2 months before entering the hospital. Complaints accompanied by a cough and fever since 4 days before entering the hospital. A history of contact with an adult TB patient does not exist. Patients born from the mother P1A0, term, spontaneous, immediately crying, birth weight 2300 grams. During pregnancy mothers routinely control patients to midwives to check their pregnancies, and never get sick. New patients get Hepatitis B immunization 1 time and BCG. Nutritional status of marasmus. He has microcephalal head circumference. On physical examination found takipneu, febris, oxygen desaturation. On examination of the pulmo there are suprasternal, intercostal and subcostal retractions, there is a decrease in vesicular breathing sound on the left and an additional pulse lung sound crackles. In the heart is the sound of systolic murmur sound. On supporting investigation, leukocytosis, increased CRP. On thorax examination is suggestive of a pulmonary aplasia. Echocardiography results obtained Truncus Arteriosus type I and Large subtruncal VSD. The result of thorax CT is left lung hypoplasia. The patient manage with symptomatic treatment.
Learning Points/Discussion : Pulmonary hypoplasia may be primary or secondary. Primary pulmonary hyperlasia is less common than secondary hypoplasia. Primary pulmonary hypoplasia is a condition that can cause respiratory distress and has a high mortality rate. Primary pulmonary hyperlasia may be caused by deficiency of transcription factors or growth factors such as TTF-1 deficiency, GATA factor, hepatocyte nuclear factor HNF310, epidermal growth factor and EGFR receptors, mitogen-activated protein (MAP) kinase, or other congenital disorders such as multiple pterygium syndrome , fetal akinesia-hypokinesia sequence, Scimitar syndrome, or Trisomy 21. The causes of secondary hypoplasia can be classified as: (1) abnormal thoracic cavities such as congenital diaphragm hernia and congenital adenomatoid malformation; (2) abnormal fetal breathing movements such as central nervous system injury or neuromuscular disorders; (3) lung fluid abnormalities or pulmonary fluid pressure as in the case of oligohydramnios; and (4) congenital heart disease. The diagnostic criteria have different manifestations depending on the extent of the lung involved and other comorbidities. Some infants may be asymptomatic tachypnea and others are acquired with respiratory distress within a few hours of life and require immediate mechanical ventilation. Other patients may come at the age of several months or years with infections recurrent lung and shortness of breath. Children with pulmonary hypoplasia will be more susceptible to respiratory infections due to abnormal angulation of the airway, ineffective mucocilliary clearance, and accumulated secretions. Pulmonary hypoplasia may be accidentally diagnosed as a result of suspicion recurrent respiratory infections so that the diagnosis of pulmonary hypoplasia requires clinical suspicion and good radiographic readings. For radiographic examinations are chest xray, computed tomography (CT) thoracic scan, fibre optic bronchoscopy, and pulmonary angiography and bronchography whenever possible. One study, however, suggests that chest CT scan is more useful in diagnosing pulmonary hypoplasia. The management of pulmonary hypoplasia are surgical or medical procedure. Surgical action that can be done is the correction of the diaphragm hernia, correction of cystic adenomatoid malformations. Medical management in the form of symptomatic supportive therapy aims to control the occurrence of infections and complications. The prognosis of patients with hypoplasia depends on the degree of abnormality. Complications that can occur are pulmonary hypertension that can be an irreversible blood vessel disease.
Keywords: pulmonary Hypoplasia congenital heart diseases CT scan Thorax