Massive Post-Traumatic Gluteal and Thigh Hematoma Unveiling Factor XIII Deficiency in an 8-Year-Old Girl: A Diagnostic Odyssey and Multidisciplinary Management
This case report describes the successful multidisciplinary management of an 8-year-old girl with a massive, refractory post-traumatic gluteal and thigh hematoma caused by previously undiagnosed Factor XIII deficiency, highlighting the disorder's tendency to evade routine screening and the critical need for targeted hemostatic therapy combined with surgical and interventional procedures.
Original paper licensed under CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). This is an AI-generated explanation of the paper below. It is not written or endorsed by the authors. For technical accuracy, refer to the original paper. Read full disclaimer
The Invisible Glue and the Great Bleeding Mystery
Imagine your body is a bustling construction site, constantly repairing tiny cracks and sealing up small leaks. When you get a cut or a bruise, your body sends out a team of workers called platelets to plug the hole, followed by a crew of proteins that weave a net to hold everything together. This net is made of a material called fibrin. But here's the secret: just weaving the net isn't enough. To make the net strong enough to stop a flood, you need a special "super-glue" that cross-links the strands, turning a flimsy web into a tough, waterproof shield. In the world of biology, this super-glue is a protein called Factor XIII.
Usually, when doctors check if your blood is clotting correctly, they run standard tests that look at the early stages of the process—like checking if the workers arrived or if the net was woven. These tests are great at finding most problems, but they have a blind spot: they don't check if the super-glue was applied. If the net is woven but the glue is missing, the clot looks fine on paper but falls apart the moment it faces pressure. This is why a person with a Factor XIII deficiency can have "normal" blood test results while still suffering from dangerous, unexplained bleeding. Understanding this hidden mechanism is crucial because without it, doctors might treat the wrong problem, leaving a patient vulnerable to life-threatening hemorrhages that seem to have no cause.
The Case of the 8-Year-Old and the "Egg Drop Soup"
This paper tells the dramatic story of an 8-year-old girl who went on a medical detective journey to solve a mystery that stumped three different hospitals. It all started with a minor accident: she landed hard on her buttocks while doing a long jump at school. While most kids would just have a bruise, she developed a massive, growing lump in her right thigh and buttock that kept getting worse, even after doctors tried to fix it.
The Diagnostic Odyssey
The girl was first treated at a local hospital where doctors found a large blood collection (hematoma) and performed surgery to drain it. But the bleeding didn't stop; the wound just kept oozing, and the lump grew back. She was then sent to a bigger university hospital, where doctors tried a different approach: they used a catheter to block the bleeding arteries from the inside (a procedure called arterial embolization). Even with this high-tech intervention, the bleeding continued, and her condition became critical. She developed a high fever, her heart raced, and her blood pressure dropped dangerously low.
When she finally arrived at the authors' hospital (Hunan Children's Hospital), the medical team realized that standard blood tests were lying to them. Her routine clotting numbers looked almost normal, which is a classic "diagnostic blind spot" for Factor XIII deficiency. The team used a special tool called Thromboelastography (TEG), which acts like a stress test for blood clots, watching how strong they get in real-time. The results were alarming: her clots were incredibly weak and falling apart, with a "maximum amplitude" (a measure of clot strength) dropping to just 25.9 mm (normal is usually 50–70 mm). This pointed them toward the missing super-glue. A specific test confirmed it: her Factor XIII antigen level was only 20.3% (normal is >70%).
The "Egg Drop Soup" Discovery
Armed with this new diagnosis, the team launched a coordinated attack. They performed another round of super-selective artery blocking, followed immediately by open surgery to drain the massive hematoma. When they opened the wound, they found something bizarre: the blood inside wasn't forming solid clots at all. Instead, it looked like "egg-drop soup"—a thin, non-coagulating liquid. This visual clue perfectly confirmed that without Factor XIII, the fibrin net couldn't hold together.
The Rescue Mission
Because specific Factor XIII medicine wasn't available in their region at the time, the doctors had to get creative. They used a "kitchen sink" approach, flooding her system with cryoprecipitate (a blood product rich in clotting factors), fresh frozen plasma, and recombinant activated factor VIIa (a powerful clotting booster). They also gave her antibiotics and pain medication.
The Outcome
The strategy worked. The bleeding finally stopped, and her blood levels stabilized. Over the next month, the massive 7.6 × 11.7 × 11.4 cm lump slowly shrank. By the time she was discharged 31 days after admission, she could walk, and at a two-month follow-up, she had returned to school with full movement in her leg.
What This Means
The authors suggest that this case highlights a critical lesson: when a patient has unexplained, stubborn bleeding after an injury but their standard blood tests look normal, doctors should suspect Factor XIII deficiency. They argue that using tools like TEG can act as a "salvage" diagnostic method to catch these hidden defects. While they couldn't prove if the girl was born with a mild version of the condition or if the trauma simply used up her Factor XIII faster than she could replace it, they conclude that a multidisciplinary team—combining interventional radiology, surgery, and creative blood product management—can save limbs and lives even when the perfect medicine isn't on the shelf. The paper explicitly rules out the idea that this was a simple surgical failure or a common clotting disorder, pointing instead to this rare, "invisible" glue deficiency as the true culprit.
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