Dr.Shobika,Dr.Mohan abraham,Dr.Aswin Prabhakaran(Paed surgery)DrCJayakumar
AIMS KOCHI
Term,AGA,Male ba,whose antenatal scan showed Enlarged echogenic left lung causing mediastinal shift to right-suggestive of Congenital pulmonary airway malformationType 3(microcytic)(CPAM volume-12.6cc,CVR ratio-0.4cm2).
After birth,ba had no distress.Air entry bilaterally equal,,hemodynamically stable,not required any support.
Contrast enhanced CT chest which showed CPAM 3 or hybrid lesion with no significant change in size, started on oral steroids.
Repeat CECT done after 2 months which showed no significant changes and small nodular air space opacity noted in apical segment of right upper lobe,
Child was taken up for open left lower lobe lobectomy under Anasthesia.
Labs:
TC-17.23ku/ml,N-42.8,L-44.1%,plt-441lakh,pre op serology-negative.
Intraoperative and postoperative period were uneventful. ICD was removed on POD 5.
Histhopathology reported of no malignancy.
Child was discharged with oral antibiotics for a week and on a regular follow up.
CPAM:
Most common congenital lung lesion.
It is the developmental malformation of the lower respiratory tract.
A large cyst called macrocytic lesion, small cyst or solid appearing lesion called microcytic.
Incidence: 1 per 8300 to 35000 live births.
Causes:The malformation results from abnormal lung tissue that grows,usually in one lobe of the lung.
Evaluation: usually discovered during a routine prenatal ultrasound
Severity : CVR or CCAM volume ratio is more than 1.6 of higher risk for developing heart failure.
Investigations:CT Chest
Management during pregnancy: If CPAM is small and don’t cause any stress on ba’s heart before birth or any problems with breathing after birth require only close monitoring
In rare circumstances,CPAM grow to be large require treatment before birth.
When the lung mass grows,it can take up valuable space in the chest.This can restrict normal lung growth and can lead to underdeveloped lungs which will not function adequately at birth.
Large lesions can also shift the heart and impair blood flow.
This leads to fetal heart failure.
Fetal intervention: steroids to prevent the growth of lesion
Draining the fluid from the cyst
Open Fetal surgery to remove CPAM
EXIT procedure(Ex utero intrapartum treatment) procedure.
Potential Pulmonary Causes for Respiratory Distress in Neonates
Parenchymal conditions
• Transient tachypnea of the newborn
• Meconium aspiration syndrome and other aspirations
• Respiratory distress syndrome
• Pneumonia
• Pulmonary edema
• Pulmonary hemorrhage
• Pulmonary lymphangiectasia
Developmental abnormalities
• Lobar emphysema
• Pulmonary sequestration
• Congenital pulmonary airway malformation
• Congenital diaphragmatic hernia
• Tracheoesophageal fistula
• Pulmonary hypoplasia
Airway abnormalities
• Choanal atresia/stenosis
• Laryngeal web
• Laryngotracheomalacia or bronchomalacia
• Subglottic stenosis
Mechanical abnormalities
• Rib cage anomalies (eg, Jeune syndrome)Pneumothorax
• Pneumomediastinum
• Pleural effusion
• Chylothorax