Severe Hemophilia A with Comorbidities Presenting with Appendicular Perforation Peritonitis: Critical Care Management

A BSTRACT A 9-year-old boy with hemophilia A presented with appendicular lump with localized peritonitis. He was positive for dengue non-structural protein 1 (NS1) antigen. The patient had a history of seizure disorder (last 3 years) and had a decompressive craniectomy for subdural hemorrhage. The patient was preoperatively optimized. Intraoperatively, appendicectomy with aspiration of pus with adhesiolysis was conducted. The patient was managed perioperatively in an intensive care unit (ICU) and received a total of 10,000 units of recombinant coagulation factor VIII. He was doing well on follow-up. We conclude that patients with hemophilia A particularly severe sub-type may present with abdominal pain due to either complications of the underlying disease or abdominal conditions especially appendicitis. Timely referral to pediatric center with pediatric surgery facility is important to prevent undue morbidity. A comprehensive critical management involving monitoring of the clotting factor FVIII levels and replacement therapy with factor VIII for hemophilia A patient presenting with surgical emergency is important for successful outcomes.