Sudden Death Caused by Plastic Bronchitis in a Child with Sickle Cell Disease

Abstract Presentation Date: 6/8/2024 Presentation Start Time: 6:00:00 PM Background Plastic bronchitis is a rare and poorly described complication of sickle cell disease and acute chest syndrome. It is defined as the formation of casts made of various materials, which obstruct lung bronchi. Plastic bronchitis has been diagnosed in several disease states including asthma, cystic fibrosis, and congenital heart disease following Fontan procedure. This case describes a case of a 5-year-old male with Sickle Cell SS Disease, admitted for pain crisis and Acute Chest Syndrome, which rapidly progressed to cardiovascular decompensation and death secondary to diffuse lung infiltration and obstruction due to plastic bronchitis. Methods Single case report Results A 5-year-old male with Sickle Cell SS Disease and moderate persistent asthma was admitted to the pediatric ward for generalized abdominal pain and extremity pain consistent with vaso-occlusive pain crisis. Subsequently, the patient developed a fever associated with respiratory distress and pain at his port-a-cath site. He was started on broad-spectrum antibiotics for Acute Chest Syndrome, given a new lung infiltrate in the right lower lobe on chest x-ray, and possible central line-associated bloodstream infection (CLABSI). He was initiated on high-flow nasal cannula (HFNC) and transferred to the pediatric intensive care unit. His respiratory status stabilized on HFNC with moderate supplemental oxygen and intermittent albuterol; however, he continued to have persistent tachycardia, tachypnea, and worsening lung infiltrates on repeat chest x-ray. Several hours later, he went into sudden cardiac arrest with asystole. Despite immediate CPR, intubation, and resuscitative measures, the patient expired. The autopsy revealed plastic bronchitis with fibrinous casts with 95% airway occlusion from the carina to the bronchioles in both lung fields. Conclusions Plastic bronchitis, a rare complication, can progress to rapid deterioration and death in pediatric patients with acute chest syndrome and sickle cell disease. The presentation of plastic bronchitis in sickle cell disease is unique compared to other underlying disease states. Clinicians should be on high alert for signs and symptoms indicating this potential complication, especially worsening chest radiographs in a patient with acute chest syndrome.