A rare combination: a single common pulmonary vein with polysplenia syndrome.

A single common pulmonary vein is a rare anomaly, mostly occurring with asplenia. We wanted to report our case because it co-exists with polysplenia and ciliary dyskinesia.A baby girl about 2 months old with respiratory arrest was admitted to the emergency room of our hospital. She responded to pulmonary resuscitation. We ascertained from her medical history that she had been hospitalised twice because of recurrent lung infections. After the initial check and treatment, we applied imaging modalities to detect underlying problems of recurrent lung infection. Echocardiographic evaluation was suboptimal because of the widespread pulmonary infiltration. Still, we noticed a single common pulmonary vein without obstruction at the junction of the left atrium. Subsequently, a chest CT scan with contrast was performed. It revealed a single common pulmonary vein entering the left atrium with a single orifice, atrial septal defect, two lobes in both lungs, hepatic veins draining directly into the right atrium, and polysplenia. When the flow of a single common pulmonary vein goes directly to the right atrium through the defect, it may cause right heart dilatation as it did in our case. Therefore, patients may need to undergo surgery at an earlier age.

Tetralogy of Fallot with double aortic arch and aortopulmonary window: a very rare trifecta.

Tetralogy of Fallot with an aortopulmonary window and double aortic arch is very rare. This complex coexistence may be over a wide clinical spectrum. Herein, we present an asymptomatic 8-day-old infant who was diagnosed as having tetralogy of Fallot, double aortic arch, and an aortopulmonary window using transthoracic echocardiography while being examined for microcephaly.

A rare cause of fatal cardiac arrhythmia: Inhalation of butane gas.

Pamuk U, Gürsu HA, Emeksiz S, Özdemir-Sahan Y, Çetin I. A rare cause of fatal cardiac arrhythmia: Inhalation of butane gas. Turk J Pediatr 2018; 60: 755-756. Butane gas, especially available in lighters, is commonly misused among adolescents and its side effects are rarely observed but serious. A 14-yearold male was brought to our emergency department. The electrocardiographic (ECG) examination showed biphasic T waves in leads V4-V6, and long QTc at 481ms. Echocardiographic study showed left ventricular systolic dysfunction. Troponin I level was found to be high at 9.1 ng/ml. Taking into consideration the patient`s history, clinical and laboratory findings, ventricular fibrillation and myocardial injury resulting from butane gas inhalation was diagnosed.

A rare complication of chronic granulomatous disease in a child: constrictive aspergillus pericarditis.

A 3-year-old girl with the diagnosis of chronic granulomatous disease (CGD) was hospitalized for bronchopneumonia and congestive heart failure. Her medical history included methylprednisolone medication for autoimmune gastric outlet obstruction. Computed tomography revealed pneumonic infiltrations and pericardial thickening. A pulsed-wave Doppler recording revealed E/A >1. During a pericardiectomy, multiple islands of thick, firm-walled, fibrinous exudate-containing, small abscess formations were observed. Histopathological evaluation of pericardial tissue revealed granulomatous inflammation. Aspergillus fumigatus was cultured from the abscess. In conclusion, development of constrictive aspergillus pericarditis should be considered in patients with CGD because immediate initiation of antifungal management with aggressive surgical treatment is life-saving.

Necrotizing Liver Granuloma/Abscess and Constrictive Aspergillosis Pericarditis with Central Nervous System Involvement: Different Remarkable Phenotypes in Different Chronic Granulomatous Disease Genotypes.

Chronic granulomatous disease (CGD) is a primary immune deficiency causing predisposition to infections with specific microorganisms, Aspergillus species and Staphylococcus aureus being the most common ones. A 16-year-old boy with a mutation in CYBB gene coding gp91phox protein (X-linked disease) developed a liver abscess due to Staphylococcus aureus. In addition to medical therapy, surgical treatment was necessary for the management of the disease. A 30-month-old girl with an autosomal recessive form of chronic granulomatous disease (CYBA gene mutation affecting p22phox protein) had invasive aspergillosis causing pericarditis, pulmonary abscess, and central nervous system involvement. The devastating course of disease regardless of the mutation emphasizes the importance of early diagnosis and intervention of hematopoietic stem cell transplantation as soon as possible in children with CGD.