What is the appropriate management for a patient presenting with severe pelvic pain and vomiting, potentially due to ovarian torsion, especially in someone with a history of Von Willebrand disease?

Management of Severe Pelvic Pain with Vomiting in a Patient with Von Willebrand Disease

Immediate surgical consultation for suspected ovarian torsion is mandatory, with concurrent hematology involvement to optimize hemostasis using factor VIII/VWF concentrate before any intervention. 1, 2

Immediate Diagnostic Workup

Mandatory Laboratory Tests

• Obtain serum β-hCG immediately to exclude ectopic pregnancy or other pregnancy-related complications, as this fundamentally alters the diagnostic pathway and imaging choices 1, 3
• Measure ristocetin-cofactor activity (VWF:RCo) levels urgently, as levels between 7-14% indicate severe VWD requiring factor replacement 2
• Check complete blood count, coagulation studies, and factor VIII levels to assess bleeding risk 2

First-Line Imaging

• Perform transvaginal and transabdominal pelvic ultrasound with Doppler as the initial imaging modality for suspected ovarian torsion, as this provides 98-100% sensitivity for gynecologic pathology without radiation exposure 1, 3
• Ultrasound should specifically assess for ovarian enlargement, free fluid, and vascular flow patterns 4

Critical pitfall: Normal Doppler flow does NOT exclude ovarian torsion—patients with severe pain and clinical suspicion may still require exploratory surgery despite normal vascularity 4

Specific Considerations for Von Willebrand Disease

Hemorrhagic Complications

• Women with VWD have disproportionately increased risk of hemorrhagic ovarian cysts, which can present with severe pelvic pain mimicking torsion 5, 6
• VWD patients are also at higher risk for endometriosis, which can cause acute pain episodes 5, 7

Preoperative Hemostatic Management

• Administer factor VIII/VWF concentrate (such as Confact F) before any surgical intervention, as this has been proven effective for controlling bleeding during emergency ovarian surgery in VWD patients 2
• Coordinate with hematology to determine appropriate dosing based on VWF:RCo levels and type of VWD 2
• Continue factor replacement during and after surgery to prevent abnormal bleeding 2

Surgical Decision Algorithm

When to Proceed Urgently to Surgery

• Severe, unrelenting pain with vomiting suggests high-grade obstruction or torsion requiring urgent intervention 1, 4
• Clinical suspicion of ovarian torsion warrants exploratory laparoscopy even with equivocal imaging, as delayed intervention risks ovarian loss 4
• Presence of peritoneal signs, hemodynamic instability, or worsening pain mandates immediate surgical exploration 4

Surgical Approach

• Laparoscopic approach is preferred when feasible, as demonstrated safe in VWD patients with appropriate factor replacement 2
• Partial ovarian resection rather than oophorectomy should be attempted when possible to preserve fertility 2

Alternative Diagnoses to Consider

If ultrasound does not confirm torsion, consider:

• Hemorrhagic ovarian cyst rupture (accounts for one-third of gynecologic pain cases and is more common in VWD patients) 8, 5
• Pelvic inflammatory disease with tubo-ovarian abscess (93% sensitivity on ultrasound, look for thick tubal walls and "cogwheel" sign) 3
• Endometriosis with acute exacerbation (98% sensitivity with transvaginal ultrasound for deep infiltrating disease) 3

Second-Line Imaging

• MRI pelvis without and with IV contrast should be obtained if ultrasound is equivocal and patient is stable enough to delay surgery 3
• CT abdomen/pelvis is contraindicated as first-line in reproductive-age women but may be appropriate if non-gynecologic causes (appendicitis, bowel obstruction) are suspected 8, 1

Critical Pitfalls to Avoid

• Never delay surgical consultation while pursuing extensive imaging in a patient with severe pain and vomiting concerning for torsion, as ovarian viability decreases with time 4
• Do not proceed to surgery without hematology consultation and factor replacement in known VWD patients, as this significantly increases hemorrhagic complications 2
• Do not assume normal Doppler flow excludes torsion—clinical judgment supersedes imaging in high-risk scenarios 4
• Avoid starting with CT imaging in reproductive-age women when gynecologic pathology is suspected, as this provides unnecessary radiation exposure 1