Von Willebrand’s disease mimicking postoperative bleeding after tibial nailing for tibial shaft fracture

Von Willebrand’s disease (VWD), or von Willebrand’s syndrome, is a bleeding syndrome characterized by low plasma levels of von Willebrand factor (VWF). VWD is the most common inherited human bleeding disorder. Partial quantitative deficiency of serum VWF is responsible for the majority of VWD cases. The effect of VWF deficiency on orthopedic operations is not well documented in the current literature. VWD may cause persistent blee­ding during the operative and postoperative periods. In the majority of cases, VWD occurs as a single episode, but frequent relapses with chronicity can be seen in a small number of cases. We reported a case of a 22-year-old man with VWD operated with intramedullary nailing due to tibial shaft fracture. The patient had no previous history of surgery, and was unaware of his VWD. The purpose of this study is to report a rare complication of an orthopedic surgical procedure with postsurgical bleeding mimicking tibialis anterior arterial perforation. Orthopedic surgeons must be alert to the possibility of VWD due to postsurgical difficulties and persistent bleeding.

Understanding the risks of total hip arthroplasty in patients with von Willebrand’s disease

Purpose: Patients with von Willebrand’s disease (VWD) have either a qualitative or quantitative deficiency in a key clotting protein called von Willebrand’s factor. Type Ⅰ disease is the most common variant, but its clinical implications in total hip arthroplasty (THA) are unclear. Our purpose is to describe the perioperative impact of VWD in THA. Methods: We retrospectively reviewed a total of 17 primary THAs in 14 patients with type Ⅰ VWD performed between 2008 and 2019. Almost all cases (88%) received tranexamic acid, and most (59%) received DDAVP. All patients had a direct anterior approach (DAA) THA. Results: None of these cases required a blood transfusion. Mean estimated blood loss was 229 mL, and the mean hemoglobin dropped from 13.9 g/dL to 10.2 g/dL. There were no major bleeding complications. After a mean follow-up of 4 years, the mean hip disability and osteoarthritis outcome score, junior (HOOS, JR) was 79, and there were no reoperations or revisions for any cause. Conclusion: Patients with type Ⅰ VWD do not experience severe bleeding with routine chemoprophylaxis combined with DAA THA.

Curative resection of a duodenal gastrointestinal stromal tumor in the setting of von Willebrand’s disease

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the alimentary tract and usually presents with gastrointestinal hemorrhage. The diagnosis of GIST is typically made with upper endoscopy after excluding other causes of bleeding. The surgical management of GIST can be challenging depending upon the location of the tumor. We present a unique case of duodenal GIST in the setting von Willebrand’s disease diagnosed after emergent laparotomy for massive gastrointestinal hemorrhage. Key strategies in curing our patient were treating the underlying bleeding disorder, collaborating with radiology and gastroenterology teams, and early exploratory laparotomy for refractory hemorrhage. This case demonstrates the challenges of diagnosing and managing GIST in patients with underlying coagulopathies.

Genetic disorders of coagulation

Haemophilia is a familial X-linked disorder due to deficiency of either factor VIII (haemophilia A) or factor IX (haemophilia B), components of the intrinsic enzymatic complex that activates factor X. Clinical features and diagnosis—the main manifestations are bleeding into joints and soft tissues, with haemophilic arthropathy and joint deformity being inevitable complications in untreated patients. Other features include pseudotumours, bleeding into the urinary system, and bleeding following clinical procedures. Laboratory diagnosis is based on a modification of the classic activated partial thromboplastin time (APTT) assay, with inhibitor screening used to exclude other causes of prolonged APTT. Treatment—involves the administration of the deficient factor VIII or factor IX, most commonly ‘on demand’ in response to bleeding, with prophylactic treatment given before surgery. Von Willebrand’s disease is a common autosomal dominant disorder of platelet function caused by a functional deficiency of von Willebrand factor (VWF). VWF, normally synthesized by megakaryocytes, prevents degradation of factor VIII; VWF, also made by endothelial cells, enhances platelet activation and recruitment at sites of tissue damage. Treatment—mild von Willebrand’s disease is treated with desmopressin 1-deamino-8-d-arginine vasopressin (DDAVP), which releases factor VIII and VWF from endothelial cells. Other treatments include ε‎-aminocaproic acid, oestrogens, and factor VIII concentrates. Other hereditary disorders of coagulation, including (1) hereditary deficiency of the plasma metalloproteinase ADAMTS13; (2) combined deficiency of coagulation factors V and VIII; (3) factor XI deficiency; (4) inherited deficiencies of factors II, V, VII, and X; and (5) deficiency of the contact activating factors, factor XIII, and fibrinogen, and hypercoagulable diseases due to deficiencies of anticoagulants or propensity to thrombosis are discussed in this chapter.