[5] Discussion
FXIII (also known as fibrin stabilizing factor) deficiency is one of the rarest factor deficiencies, occurring in approximately 1 in every 2 million people worldwide. Its function includes stabilizing blood clot formation, aiding in tissue repair, depositing extracellular matrix, and contributing to the differentiation of osteoblasts [2]. The deficiency typically presents as excessive and prolonged bleeding from the umbilical stump, poor wound healing, and can lead to complications such as intracranial hemorrhage, either spontaneously or post-traumatically [10]. Additionally, superficial and deep hematomas are associated with the deficiency, although they are more commonly observed in older age groups [11].
Diagnosing FXIII deficiency can be challenging, as standard clotting tests such as PT, aPTT, and INR are usually normal. Key laboratory investigations for diagnosis include the clot solubility test, FXIII activity assay, FXIII antigen assay, inhibitor assay, and molecular diagnostics [2]. This case is unique because we believe it is the first reported instance of a duodenal hematoma due to FXIII deficiency. Duodenal hematomas are typically caused by crushing blunt force that ruptures intramural blood vessels or as a complication of anticoagulant therapy, endoscopic biopsy, vasculitis, pancreatitis, tumors, or bleeding disorders [6-9].
Early diagnosis of FXIII deficiency allows for conservative treatment, avoiding surgery [6, 8, 9, 12, 13]. Treatment options for FXIII deficiency include fresh frozen plasma (FFP) or cryoprecipitate. However, with advancements, FXIII concentrate and recombinant FXIII (rFXIII) are available for severe deficiencies to reduce bleeding events.
For duodenal hematomas, treatment can be conservative or invasive. Conservative management involves keeping the patient NPO for bowel rest and nasogastric (NG) decompression, placing a peripherally inserted central catheter (PICC), and initiating parenteral nutrition [14-17]. The duration of conservative management varies, and enteral nutrition can begin once gastric aspirates are no longer bilious.
Surgical treatment options include hematoma removal or bypass surgery [18]. In the past, when radiological advancements were not available, an exploratory laparotomy was performed after blunt abdominal trauma for evacuation, but this is now an option primarily in cases of jaundice and delayed bowel obstruction [19]. Invasive surgical procedures may still be necessary for complications such as uncontrolled bleeding or panperitonitis [20]. Recent advances include endoscopic drainage with fistula formation or mucosal puncture, which has shown success without major complications [21]. In cases of intramural duodenal hematomas (IMDH), percutaneous catheter insertion can be used for decompression [22].