Extracorporeal membrane oxygenation to rescue profound pulmonary hemorrhage due to idiopathic pulmonary hemosiderosis in a child

Abstract

Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage (DAH) in pediatric patients. During the acute phase, death due to massive alveolar hemorrhage and subsequent severe respiratory failure with associated multiple organ failure often occurs. We report the case of an 11-year-old girl who developed severe hypoxemic respiratory failure and pulmonary hemorrhage. Extracorporeal membrane oxygenation (ECMO) was instituted on the next day because medical treatment and mechanical ventilation failed to maintain oxygenation. She was successfully decannulated 5 days later without ECMO-related complications. Four months later, she was intubated again and the diagnosis of IPH was made by open lung biopsy. She was treated with systemic steroid therapy and discharged smoothly. We suggest that ECMO provides a chance of recovery and survival for patients with IPH, even if accompanied by severe pulmonary hemorrhage.

Fatal idiopathic pulmonary haemosiderosis in association with pregnancy – Medico-legal evaluation

“…15 Clinically, IPH has a triad manifestation of haemoptysis, diffuse parenchyma infiltration on chest radiography and iron-deficiency anaemia. 9 In IPH patients death may occure suddenly and unexpectedly. 16 Laboratory and radiologic findings that have been found to be helpful to diagnose the disease are anaemia, chest X-ray showing minimal transient infiltrates to massive parenchymal involvement with secondary atelectasis, emphysema and hilar lymphadenopathy.…”

“…IPH is a rare condition, and to date, about 500 cases have been reported in the literature. 9 Although IPH may be associated with an immunological, 1,10 genetic 11 or toxic 12 mechanism, the exact aetiological mechanisms remain unknown. 7,13 Pathologically, IPH is characterised by heavy lungs with aggregates of haemosiderin-laden macrophages due to recurrent diffuse alveolar haemorrhage in the absence of vasculitis or capillaritis and by eventual interstitial fibrosis.…”

“…6e8 Death may occur suddenly from acute pulmonary haemorrhage or progressive respiratory failure. 9 Post-partum complications have a great public interest and require medico-legal evaluation.…”

Successful application of extracorporeal membrane oxygenation due to pulmonary hemorrhage secondary to granulomatosis with polyangiitis

“…Reviewing the ELSO database they reported 72% and 67% survival among 18 neonates and three children, respectively, who received ECMO for the treatment of pulmonary hemorrhage. Subsequently, ECMO has been successfully applied to children with severe respiratory failure due to pulmonary hemorrhage secondary to idiopathic pulmonary hemosiderosis,27 fulminant Wilson disease,28 nodose polyarteritis,29 Goodpasture’s syndrome,6 and ANCA-related vasculitis, including severe microscopic polyangiitis,5,8 and GPA 7,9…”

Extracorporeal Membrane Oxygenation for Diffuse Alveolar Hemorrhage Caused by Idiopathic Pulmonary Hemosiderosis: A Case Report and a Review of the Literature

“…5 However, only a few instances of DAH in children caused by IPH and treated with ECMO have been reported. 6,7 We report, herein, a case of an 8-year-old girl with severe respiratory failure caused by DAH who underwent ECMO.…”