CRP7 CASE REPORT: A RARE CAUSE OF HEMOPTYSIS IN A 5 YEAR-OLD GIRL

Shemalatha Kejendaram1, Jaeson Chong1 , Mahfidz Abdul Jalil1 , Hasliza A Razak1, Liew Zheyi1 , Mohd Nizam Mat Bah1 , Hanafi Sapian1 , Norliza Othman2, Hazrini Abdullah2
1 Paediatrics Department, Hospital Sultanah Aminah, Johor Bahru
2 Radiology Department, Hospital Sultanah Aminah, Johor Bahru

Background 

Pulmonary vein stenosis (PVS) is a rare congenital anomaly characterised by the narrowing or complete obstruction of one or more pulmonary veins. PVS typically manifests with respiratory distress, chronic cough, and failure to thrive in children, often leading to misdiagnosis as tuberculosis. Effective management of PVS may require both medical therapy for symptom control and surgical interventions in severe cases.

Case Presentation 

We report a case of a 6-year-old Malay girl presenting with recurrent lower respiratory tract infections (LRTI) and persistent hemoptysis. Born prematurely at 31 weeks' gestation, she required brief ventilation. At 4 years old, she experienced her first episode of hemoptysis, accompanied by a chronic cough and failure to thrive. Initially treated for smear-negative pulmonary tuberculosis, she completed a nine-month course of anti-TB therapy and a six-week course of prednisolone, with no resolution of symptoms. Comprehensive evaluations, including bronchoscopy, echocardiogram, and computed tomography pulmonary angiography (CTPA), ultimately diagnosed PVS. A lung perfusion scan, conducted as part of the preoperative assessment, revealed solitary left lung perfusion with absent right lung perfusion, predicting a postoperative forced expiratory volume in one second (FEV1) of 68.6%. The patient is scheduled for right pneumonectomy if symptoms persist.

Conclusion  

This case underscores the diagnostic challenge of PVS in the context of persistent hemoptysis and recurrent pneumonia, particularly in children with previously failed anti-TB treatment. It highlights the necessity of considering rare etiologies for hemoptysis, employing comprehensive diagnostic modalities, and evaluating surgical interventions for managing severe PVS cases.