True blue: a puzzling case of persistent cyanosis in a young child

Abstract

A 2-year-old twin boy was referred to a cardiology clinic for long-standing cyanosis. He had been born to a healthy 36-year-old mother. The diamnionic, dichorionic twin pregnancy had been complicated by maternal cholestasis, which had been treated with ursodiol. Amniocentesis had been performed, and the findings were normal.The twins had been delivered by cesarean section at 34+6 weeks' gestation. Our patient's Apgar scores were 8 at 5 minutes and 9 at 10 minutes. His birth weight was 2225 g. He required tracheal intubation shortly after birth because of low oxygen saturation levels. An initial radiograph of his chest showed an increased cardiothoracic ratio, which raised suspicion of congenital heart disease. An echocardiogram appeared normal except for a restrictive patent arterial duct. The tracheal tube was removed shortly thereafter.He remained on low-flow supplemental oxygen for 4 weeks. His oxygen saturation levels were in the low 90% range, falling to the mid-80% range without oxygen. The differential diagnosis for his dependence on supplemental oxygen included patent ductus arteriosus, neonatal chronic lung disease and mild pulmonary hypertension (although this last condition was not confirmed on an echocardiogram). The boy was given nebulized budesonide therapy and weaned off oxygen. He was discharged home with no oxygen therapy. His twin sibling had an unremarkable postpartum course and had no need for oxygen therapy.Over time, the patient showed normal growth, development and energy levels. His growth consistently measured in the 5th percentile for weight and the 10th percentile for height, and matched that of his twin sister.During his second year, his parents observed a persistently blue tinge around his lips and nail beds. He showed no associated shortness of breath, loss of consciousness or distress. A general pediatrician who was following him at the time ruled out methemoglobinemia and arranged a follow-up radiograph of the boy's chest. The radiograph was reported as normal. The boy's oxygen saturation levels had not been documented since his discharge from the neonatal intensive care unit.On examination in the cardiology clinic, the boy looked well and was in no distress. His heart rate was 114 beats/min and his blood pressure 90/43 mm Hg in the left arm. His oxygen saturation level was 73% in room air. His height was in the fifth percentile at 81 cm, and he weighed 10 kg, also in the fifth percentile. He had visible cyanosis of his mucous membranes and lips (Figure 1) and clubbing of his fingers and toes (Figure 2). He had a very short grade 1/6 systolic ejection murmur that was best heard at the upper left sternal border. The rest of the findings on examination were normal.A radiograph of the chest showed increased opacity in the left hilum (Figure 3). An electrocardiogram showed normal sinus rhythm and no evidence of chamber enlargement or ischemic changes. In a hyperoxia test using 100% oxygen and

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“…HIV, debilitated general conditions, and malignancy places patients at a higher risk (yatha preta) for developing BHT. 58 Cyanosis (shyaava) may be intermittent or persistent. Central cyanosis typically involves the lips, tongue (jihwa), mucous membranes and nail beds (nakha), whereas peripheral cyanosis involves the fingers and toes.…”