Recurrent apnea episodes as an onset of a primary pulmonary hypertension, a case report

A 15-year-old teenager boy presented to emergency room, because of dyspnoea and prolonged dry cough, worsening at night time. He gained 15kg in the last 6mo, when began complaining of physical load intolerance. He was suspected having asthma in case the positive family history and allergy to house dust mites was confirmed. No specific treatment was given, no other investigations were done. He became worse in the last 3mo: night sweats and wheezing increased, face, eyes swelling episodes in the morning appeared. He was healthy by then. Findings: weight 89 kg (97‰), height 179 cm (90‰). His face seemed as if swollen, flushed, dilated capillaries on the chest were observed. No other pathological findings on clinical evaluation. Blood, urine tests and CRP were normal. Spirometry: severely decreased peak expiratory flow (PEF), no other changes. Increased mediastinal size on chest X-ray was suspected. The patient was hospitalised for further investigations. Not enough data supporting allergic asthma, atypical infections, TB, GERD, cardiac pathology were found. Due to weight gain in a short time he was consulted by endocrinologist and further tests recommended. But increased ESR (24mm/h), b2 microglobulin (1.83mg/l), constantly decreased PEF-metry results (max volume of 270ml) and primary changes on X-ray resembled more severe condition. Repeated chest X-ray showed persistent mediastinal enlargement, and the limited homogenic mass on lateral view. Chest CT demonstrated anterior mediastinal enlargement due to non homogenic masses; lymphoid-like masses surrounding the aortic arch, spreading to all the superior mediastinum and tracheal bifurcation, circularly surrounding all the aortic branches, pressing superior v. cava, distal postclavicular venous part, merging with postclavicular lymph node conglomerates; infiltrating pericardium. Mediastinal tumor was suspected and biopsy was planned. The procedure and ventilation were complicated due to tracheal obstruction and significant bleeding. Afterwards the patient was treated at the PICU where he was on continues ventilation without possibility for extubation due to expressed pressure to trachea. The swelling of upper part of the body was significant as well. Methylprednisolone till the answer of the biopsy was started, but without any success. Biopsy results revealed sarcoma of the primitive formed elements of the blood. The patient was transferred to leukemia center. Conclusion: Not every child with asthmatic complaints could have asthma. If complaints persist or progress, the more extent evaluation, especially chest X-ray should be done. Every child with mediastinal enlargement on chest X-ray should undergo screening for oncological conditions.