| Dr Anakha V Ajay* Dr Sajitha Nair* Dr Mahesh K** Dr C Jayakumar* (Dept of Pediatrics* and Pediatric Cardiology**) Amrita Institute Of Medical Science, Kochi. |
Three year 11 month old female presented with recurrent episodes of fever, cough and breathing difficulty since birth. History is negative for choking of feeds, aspiration of foreign body or pasty foul smelling stools. This afebrile child was breathing at a rate of 30 per min. Clubbing and cyanosis were absent . Auxology -weight between 0 & 2 SD, height between 0 & 2 SD. Scattered crackles and rhonchi were heard in right mammary area. Signs cardiac disease like murmur , muffling ,hepatomegaly were conspicuously absent .But Xray Chest showed mesocardiac heart with infiltrates in the right middle and lower lobes.
Previous X-ray also showed persistent infiltrates in right middle and lower lobe. Mantoux and gastric aspirate for genexpert were negative.GE scintigraphy done to rule out reflux was negative . HRCT Chest with angio done to rule out sequestration of lung showed anomalous drainage of right upper and middle pulmonary veins into suprahepatic IVC. Right lower,left upper and lower pulmonary veins drain into LA, Right middle and lower lobes of the lung are hypoplastic , Right pulmonary artery was smaller than the Left. –Heart was Dextropositioned. ECHO done showed partial anomalous pulmonary veins(PAPVC) of right upper pulmonary veins into suprahepatic IVC .Diagnosis of Scimitar syndrome was considered. As PA pressures were normal and as there were no significant collaterals she was advised conservative management.
Chest xray showing mesocardiac heart with anomalous pulmonary vein*(resembling backsword/scimitar)
CT Pulmonary angiogram showing *anomalous venous anomaly
Conjugated pneumococcal vaccine yearly influenza and 2doses of varicella was advised
Child was advised the need for multidisciplinary team follow up and early treatment of infections.
Scimitar syndrome is a rare congenital heart defect, an unusual developmental anomaly causing partial anomalous pulmonary venous return that results in a left-to-right shunt. It has a characteristic anatomic feature that resembles a backsword or a saber with a curved blade known as the Middle Eastern or Turkish sword, “scimitar.”
It is a variant of a partial anomalous pulmonary venous return that results in a left to right shunt with characteristic features which include:
(a) Partial or entire anomalous curved venous drainage of the right lung to the inferior vena cava.
(b)Association with variable right lung and pulmonary artery hypoplasia.
(c) Dextroposition of heart.
(d)An anomalous systemic blood supply to the ipsilateral lung.
Other associated findings include atrial septal defects & aortopulmonary collaterals, patent ductus arteriosus, ventricular septal defect , pulmonary vein stenosis, tetralogy of Fallot, aortic arch hypoplasia ,coarctation or hypoplastic left heart syndrome. Accessory diaphragm, eventration or partial absence of the diaphragm; phrenic cyst; horseshoe lung; and pericardial absence, severe pulmonary hypertension causing congestive heart failure are associated features contributing to significant mortality.
Children may be asymptomatic with a benign outcome, or with signs of congestive heart failure and/or respiratory distress. Right lower lobe infections and bronchiectasis and interstitial pulmonary disorders are common
Scimitar syndrome is primarily an imaging diagnosis
For children with signs of heart failure, medical therapy should be started promptly. Lobectomy or pneumonectomy is only indicated in a patient with recurrent infections, diffuse bronchiectasis, persistent hemoptysis, or marked hypoplasia of the right lung.
Presentation in infancy has a poor prognosis.With early diagnosis and an established surgical strategy, the outcomes tend to be good with a low rate of morbidity and mortality after corrective surgery.
Carry Home Message:In a child with recurrent respiratory infections it is important to review with all previous chest xrays.When the same lobe of lung is recurrently affected from birth it is important to consider congenital malformation of lung or less commonly venous anomaly of heart like scimitar syndrome.