Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by high fever, maculopapular rash, neurological symptoms, abnormal liver functions and coagulopathy. Primary HLH is due to an underlying genetic abnormality. Secondary HLH are due to an underlying infection, autoimmune disease or malignancy. Secondary HLH due to viral infections are commonly due to the herpes group commonest of which is the Ebstein Barr virus (EBV). We describe two children with virus associated hemophagocytic lymphohistiocytosis (VAHLH) secondary to hepatitis A infection.
A one and half month old infant was brought to the paediatric emergency department in June 2014 with complaints of fever and fast breathing of one week duration. He was evaluated in a local hospital and was suspected to have pneumothorax on chest radiograph and hence referred to our institution for further management of pneumothorax.He was the first child to non-consanguineous parents born by normal vaginal delivery at term. His neonatal period was uneventful and he was feeding well and active till he developed the above symptoms. At admission to emergency department, he had tachypnoea and severe respiratory distress. In spite of severe respiratory distress he was alert. His respiratory system examination revealed decreased air entry on the left side. No other dysmorphic features were noticed. In the emergency department chest radiograph was done. As the clinicians initially suspected pneumothorax, an emergency needle thoracostomy was done by inserting the needle in the left second intercostal space; minimal air bubbling was noticed in the underwater seal. He was started on high flow oxygen and admitted to paediatric ICU.However, his symptoms were not improving and an intercostal drain was placed as an emergency measure. The chest radiograph was reviewed and a differential diagnosis of congenital lobar emphysema was also considered as there was hyperinflation of the left lung with paucity of vessels and the mediastinum was shifted to the right [Table/ Fig-1]. CT thorax was done to confirm the diagnosis. Limited MRI sections were also done for better characterisation of the mediastinum. Imaging revealed a well-defined oval shaped lesion located just posterior to the left main bronchus, left lateral to thoracic oesophagus and just inferomedial to distal aortic arch [Table /Fig-2a,b &3a-c]. The lesion was compressing the left main bronchus and was causing obstructive emphysema in the form of hyperinflated left upper and lower lobes with normal vascular divisions which are widely separated within the overinflated lung [Table /Fig-2b,3c]. Small pneumothorax was also noted. There was herniation of left lung into the right hemithorax with mediastinal shift to the right and associated compressive atelectasis of right
ABSTRACTBronchogenic cysts are the most common cystic mediastinal lesion in children. Bronchogenic cyst causing unilateral obstructive emphysema is a rare presentation. We report the case of a one and half month old infant who presented with respiratory distress which was initially suspected as left pneumothorax on frontal chest radiograph but was later found to be due to hyperinflated left lung and hence the possibility of congenital lobar emphysema was considered. CT thorax and limited MRI sections revealed a cystic lesion in mediastinum causing obstructive emphysema and mediastinal displacement. He underwent an emergency thoracotomy and excision of the cyst via an extrapleural approach. Post operatively, rapid improvement of the infant was noticed both clinically as well as radiologically. Cross section...
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