ASSOCIATIONS OF PERIPHERAL MUSCLE STRENGTH, PULMONARY FUNCTION AND FUNCTIONAL CAPACITY IN CHILDREN AND ADOLESCENTS WITH CYSTIC FIBROSIS

Background and Aims: Congenital pulmonary airway malformation (CPAM) is a rare congenital anomaly affecting the lower respiratory tract. Most CPAMs occur in isolation, although other abnormalities like bronchopulmonary sequestration and congenital diaphragmatic hernia, have been reported to occur in association with CPAM. Affected patients may present with respiratory distress in the newborn period or may remain asymptomatic until later in life. Fortunately, many cases are now detected by routine prenatal ultrasound examination. Definitive management for CPAM remains to be surgery. This paper presents a case of a prenatally diagnosed congenital pulmonary airway malformation; subsequently, underwent lobectomy. Methods: This is a case report. Results: A 7-month old infant was antenatally diagnosed with congenital pulmonary airway malformation at 24 months of gestation. Monthly antenatal ultrasound was done to monitor the size of the cystic mass, and to detect other possible congenital anomalies. Antenatally, the mother was subsequently referred to a perinatologist, a thoracovascular surgeon and a paediatric pulmonologist for consult and confer with the delivery plan of the foetus. Upon delivery, patient had good APGAR score and not in respiratory distress. A baseline CT scan was done which showed CPAM, bilateral. Initial plan was for observation. At 7 months old, patient was readmitted and repeat CT scan was done which showed the persistence of the cystic masses. Patient subsequently lobectomy of the left lower lobe. Conclusion: In general, the prognosis for a foetus with CPAM is good with only a small number developing to hydrops. Prenatal diagnosis of CPAM warrants early consultation with neonatal and paediatric surgical staff. Postnatally, patient’s presentation is varied ranging from asymptomatic to respiratory distress. Management for asymptomatic CPAM remains controversial. However, definitive treatment is still surgical. Currently, there are no guidelines as to the optimal timing of surgery.