RESUMEN
Acquired hemophilia A (AHA) is a rare disease that results from factor VIII inhibitors causing abnormal coagulation, and certain cases may develop after highly invasive surgery. The present case study reports on a 68-year-old male patient who developed AHA after undergoing a subtotal stomach-preserving pancreatoduodenectomy for distal cholangiocarcinoma. The patient experienced complications after surgery, requiring reoperation on postoperative day (PD) 5 due to rupture of the Braun's enterostomy. On PD 6, angiography was performed after bleeding was detected in the jejunal limb, but hemostasis occurred spontaneously during the examination. Bleeding was observed again on PD 8 and direct surgical ligation was performed. On PD 14, bleeding recurred in the jejunal limb and angiography was performed to embolize the periphery of the second jejunal artery. During the procedure, the prothrombin time was normal, but only the activated partial thromboplastin time was prolonged. A close examination of the coagulation system revealed a decrease in factor VIII levels and the presence of factor VIII inhibitors, resulting in the diagnosis of AHA. Administration of steroids was initiated on PD 15 and, in addition to daily blood transfusions, activated prothrombin complex concentrate was administered to achieve hemostasis. The patient was discharged from the intensive care unit on PD 36 but later developed an intractable labial fistula due to suture failure at the gastrojejunostomy site. As the use of factor VIII inhibitors continued despite the administration of steroids, cyclophosphamide (CPA) pulse therapy was added at PD 58. However, CPA was ineffective and the administration of rituximab was initiated on PD 98. After 12 courses of rituximab, the patient tested negative for factor VIII inhibitors on PD 219. On PD 289, labial fistula closure was performed with continuous replacement of factor VIII and the patient was discharged on PD 342.
RESUMEN
BACKGROUND: During restorative proctocolectomy with ileal pouch-anal anastomosis for ulcerative colitis-associated colorectal cancer or dysplasia, ileal pouch-anal handsewn anastomosis (IAA) is preferred to avoid the risk of cancer development in the remaining rectal mucosa. However, there is a risk of the ileal pouch not reaching the anus with this procedure. Here, we created deformable 3-dimensional (3D) models for simulation. METHOD: Six patients who underwent IAA without vessel ligation and 5 patients who underwent ileal pouch-anal canal double-stapled anastomosis (IACA) because the ileal pouch did not reach the anus were studied. A 3D printer was used to create deformable 3D models from the data obtained from computed tomography scans. The positional relationship among the mesenteric arteries, pubis, and coccyx were evaluated. RESULT: The distance between the superior mesenteric artery root and the tip of the ileal artery was longer in the IAA group than that in the IACA group (IAA vs IACA: 26.2â ±â 2.1 cm vs 20.9â ±â 1.6cm). The distance from the tip of the ileal artery to the coccyx (IAA vs IACA: 6.7â ±â 1.7 cm vs 12.1â ±â 2.1 cm) and the distance from the tip of the ileal artery to the lower edge of the pubis (IAA vs IACA; 8.1â ±â 1.3 cm vs 12.7â ±â 2.4 cm) were longer in the IACA group than those in the IAA group. CONCLUSIONS: We established a method for creating 3D deformable models of patients with ileal pouch-anal anastomosis. These 3D models may be useful for preoperative simulation.
We established the method for creating deformable 3-dimensional models of the patients who underwent restorative proctocolectomy with ileal pouchanal anastomosis, and the distance from the tip of the ileal artery to the coccyx was shorter in ileal pouchanal handsewn anastomosis group.