We describe two rare cases of symptomatic cystic lung disease in premature babies that required lobectomy by thoracotomy. Histology revealed Persistant Interstitial Pulmonary Empysema (PIPE).
Retrospective case note review of two neonates within our centre diagnosed with PIPE following lung resection.
Our first patient was born at 32/40 at 2.4kg and required 24hours of BiPAP followed by 24hours CPAP before being maintained on high flow oxygen. He developed recurrent right sided pneumothoraces from day 5 requiring multiple chest drains with persistent air leak. Chest XRay on day 22 should multiple cystic lesions on right mid and lower hemithorax (Figure 1). He underwent a thoracotomy and middle lobectomy without complication and was discharged home in air.
Our second patient was born at 29+6/40 at 1.34kg. She required BiPAP until day 6, followed by CPAP for 7 days, then high flow oxygen for 18 days before being weaned into 0.1L/min O2. At 4 months old she underwent a thoracotomy and left lower lobectomy without complication. Post-operatively she was weaned off her oxygen.
Histology for both patients showed pulmonary cysts related to histiocytes and emphysematous appearance in keeping with PIPE.
Figure 1: Chest XRay of patient 1.
Surgeons have varying opinions of the management of congenital cystic lung lesions. PIPE is a rare cause of cystic lung disease which involves a triad of prematurity, respiratory distress and previous mechanical ventilation. It is due to rupture of terminal airways allowing gas into interstitial space – persistant interstitial empysema (PIE). PIE typically resolves but can persist to form an expanding radiolucent mass that can cause mass effect and progressive respiratory distress = PIPE. We present two cases of this rare condition whose respiratory condition improved significantly after resection of the affected lobe.