Von Willebrand disease (VWD) is a haemorrhagic diathesis resulting from an abnormality of the Von Willebrand factor (VWF) found in plasma. It is characterised by an increased tendency to mucosal bleeding, caused by impaired platelet adhesion and decreased activity of plasma factor VIII (FVIII). Adequate VWF levels ensure the proper functioning of the above processes (1). The disorder is inherited in an autosomal dominant or recessive fashion. Both men and women may be affected. There are three types of the disease. Type 1 VWD is the most common and also the mildest subtype (accounting for 70-80% of patients with VWD). Type 2 is seen in about 20% of patients, whereas type 3 is rare, occurring in < 5% of cases (2). Recent guidelines strongly recommend that patients with a history of bleeding and VWF levels < 50 IU/dL be diagnosed for VWD (3).

Although mucocutaneous bleeding is the main symptom, patients may additionally experience gastrointestinal or joint bleeding. Symptoms and their severity vary depending on the type of VWD, VWF activity, age and gender (2). The aim of this paper was to present two cases of anorectal patients with co-existing VWD and to summarise and review current scientific data on the perioperative management of VWD patients.

A 20-year-old woman with recurrent pilonidal cyst abscesses was scheduled for elective pilonidal cyst excision. The diagnosis was based on the characteristic clinical picture and confirmed by ultrasound examination. Transrectal ultrasound performed as part of the diagnosis ruled out perianal fistula. Initially, the patient presented with an elevated temperature, an abscess in the intergluteal cleft and local pain. The abscess perforated spontaneously. Empirical antibiotic therapy and topical treatment were started. However, the symptoms recurred in the months that followed and she underwent elective surgery one year after symptom onset. The patient had a coexisting history of type 1 VWD (VWF 29%, VWFAg 65%). She reported prolonged menstrual bleeding and bleeding after tooth extraction. Her VWD family history was negative. The patient had no history of major surgery. Due to her haematological status, the patient required appropriate perioperative management. She was consulted by an attending physician from an outpatient haematological clinic before the surgery. The consultation included recommendations for individually tailored perioperative management. Following the haematological recommendations, 1 x 2000 IU of intravenous VWF/FVIII concentrate was administered one hour before surgery. Subsequently, Bascom II procedure was performed for the pilonidal cyst. During the procedure, special attention was paid to thorough haemostasis, a Redon drain was placed in the wound and an additional pressure dressing was applied. The patient received intravenous VWF/FVIII concentrate at 1 x 2000 IU for two consecutive days; tranexamic acid 3 x 1 g and etamsylate 3 x 0.5 g were postoperatively started initially intravenously, then at home orally at the same dose, for a total of 7 days. The surgical drain was removed on the second postoperative day. No increased intra- or postoperative bleeding was observed compared to non-VWD patients. Histopathological examination confirmed the diagnosis of a pilonidal cyst excised with a healthy tissue margin. The patient was discharged home in good general and local condition on day 3 postoperatively. After 3 weeks, the patient developed surgical site infection associated with the formed hematoma. The wound was surgically debrided, a swab was taken, wound healing by granulation was continued, and empirical antibiotic therapy (amoxicillin with clavulanic acid) was included for 7 days. The patient admitted to non-compliance with the doctor’s recommendations on wound hygiene and infrequent wound disinfection. Again, no significant bleeding was observed, and the patient did not require VWF/FVIII concentrate. After discharge, the patient remained under the care of a surgical outpatient clinic at her place of residence. The wound healed 4 months postoperatively.

The second patient was a 36-year-old woman with grade III bleeding haemorrhoids. Her first symptoms of haemorrhoidal disease in the form of bleeding and hypertrophied anodermal folds appeared during her first pregnancy. The diagnosis of type 1 VWD (VWF 30%, VWF:Ag 50%, FVIII 60%) was also made at that time. The patient developed postpartum haemorrhage after vaginal delivery. Prior to the pregnancy, no increased bleeding was observed. This patient also had no prior history of surgery. However, she had a family history of VWD. In years that followed, the haemorrhoidal disease progressed with recurrent bleeding. The patient was qualified for surgery. She required appropriate perioperative management and was also under the care of a haematology outpatient clinic. Intravenous VWF/FVIII concentrate at 1 x 2000 IU was administered one hour before the procedure. Milligan-Morgan haemorrhoidectomy was performed. During the procedure, care was taken to ensure particularly thorough haemostasis, and the haemorrhoidal arteries were double-punctured. The patient received another dose of intravenous VWF/FVIII concentrate at 1 x 2000 IU only on the first day after the procedure. Tranexamic acid 3 x 1 g and etamsylate 3 x 0.5 g were started initially intravenously, then continued orally at home, at the same doses, for a total of 7 days, as in the previous patient. No bleeding occurred during treatment or follow-up. At the follow-up visit 8 weeks postoperatively, the anal canal was found to be healed, with no postoperative complications.