What is the appropriate management for a 63-year-old patient with von Willebrand factor (VWF) deficiency, Barrett's esophagitis, and black solid stool, following a recent flu and empiric courses of Rocephin (Ceftriaxone) intramuscularly (IM) and Cefdinir orally (PO), with a chest X-ray showing a prominent stomach bubble and normal cardiac and mediastinal findings?

Black Stool in VWF Deficiency Patient: Urgent Endoscopy Required

This patient requires urgent upper endoscopy within 24 hours given the combination of black stool (melena), von Willebrand factor deficiency, Barrett's esophagitis, and concerning radiographic findings suggesting possible foreign body or mass. 1

Immediate Risk Stratification

This 63-year-old patient has multiple high-risk features for significant upper GI bleeding and mortality 1:

• Age >60 years - independently increases mortality risk 1
• Von Willebrand disease - creates dual hemostatic defect (prolonged bleeding time and factor VIII deficiency) requiring specialized management 1, 2
• Barrett's esophagitis - premalignant condition requiring surveillance and consideration of malignancy 1
• Abnormal chest X-ray - "bumpy" gastric margins with radio-opaque line suggests possible foreign body, mass, or bezoar requiring urgent evaluation 1

Using the Rockall scoring system, this patient scores at minimum 3-4 points (age 60-79 = 1 point, comorbidity = 2 points, potential diagnosis requiring endoscopy), placing them in a category requiring urgent intervention rather than outpatient management. 1

Differential Diagnosis

Most Likely Causes of Black Stool:

Upper GI bleeding sources (above ligament of Treitz):

• Peptic ulcer disease - most common cause of melena, especially with Barrett's esophagitis history 1
• Esophageal ulceration or erosion - related to Barrett's esophagitis or medication-induced 1
• Gastric varices - less likely without cirrhosis, but can occur with splenic vein thrombosis 1
• Malignancy - gastric or esophageal cancer must be excluded given Barrett's history and radiographic findings 1

Foreign body or bezoar:

• The radio-opaque line from esophagus to stomach bubble strongly suggests foreign body impaction or food bolus, which can cause pressure necrosis and bleeding 1
• "Bumpy" gastric margins could represent mass effect from bezoar or tumor 1

Medication-related:

• Recent ceftriaxone and cefdinir are unlikely to cause black stool directly
• Rule out concurrent NSAID or antiplatelet use

Critical Workup - Immediate (Emergency Department)

Laboratory studies (STAT): 1

• Complete blood count with hemoglobin/hematocrit
• Type and crossmatch (given VWF deficiency)
• Prothrombin time (PT), activated partial thromboplastin time (aPTT)
• VWF antigen (VWF:Ag), VWF activity (VWF:RCo or VWF:GPIbR), and factor VIII activity - essential for bleeding disorder assessment 1, 3, 4
• Blood urea nitrogen and creatinine
• Liver function tests

Resuscitation: 1

• Establish large-bore IV access (two sites given bleeding disorder)
• Fluid resuscitation if hemodynamically unstable
• Blood transfusion if hemoglobin <7-8 g/dL or symptomatic anemia 1

Hematology consultation: 1, 3

• Immediate consultation required for VWF deficiency management before endoscopy
• Determine VWD type and severity to guide hemostatic therapy
• Consider desmopressin (DDAVP) 0.3 mcg/kg IV if type 1 VWD and minor bleeding 5
• VWF/FVIII concentrate replacement if type 3 VWD, severe type 1 or 2, or if DDAVP contraindicated/ineffective 3, 6
• Target VWF activity ≥50 IU/dL before endoscopy 3, 7

Endoscopic Evaluation - Urgent (<24 hours)

Flexible upper endoscopy is indicated urgently for: 1

• Diagnosis of bleeding source
• Evaluation of Barrett's esophagitis and exclusion of dysplasia/malignancy
• Assessment and removal of suspected foreign body (radio-opaque line on X-ray)
• Therapeutic intervention if active bleeding identified

Endoscopic findings that predict high rebleeding risk: 1

• Active arterial bleeding (80% risk of continued bleeding/death if shocked)
• Non-bleeding visible vessel (50% rebleeding risk)
• Adherent clot
• Ulcer >2 cm
• Posterior duodenal or lesser gastric curvature location

If foreign body confirmed: 1

• Gentle pushing into stomach is first-line for food bolus impaction
• Retrieval with baskets, snares, or grasping forceps if resistant
• Biopsy underlying mucosa - up to 25% have underlying esophageal disorder (stricture, eosinophilic esophagitis, tumor) 1

Hemostatic Management for VWF Deficiency

Pre-procedure preparation: 3, 5, 7

For Type 1 VWD (most common - 75% of cases):

• Trial of desmopressin (DDAVP) 0.3 mcg/kg IV if previously responsive 5
• Monitor VWF:RCo and FVIII levels pre- and 1-hour post-treatment
• Fluid restriction to prevent hyponatremia, especially in elderly 5
• If inadequate response or type 2/3 VWD, use VWF/FVIII concentrate

For Type 2 or Type 3 VWD:

• VWF/FVIII concentrate replacement is required 3, 6
• Target VWF activity ≥50 IU/dL before and during procedure 3, 7
• Type 2B VWD: DDAVP is contraindicated (causes thrombocytopenia) 2
• Type 3 VWD: DDAVP ineffective (no endogenous VWF to release) 6

Adjunctive hemostatic measures: 5

• Tranexamic acid 1g IV TID as adjunct (not monotherapy for endoscopy) 5
• Proton pump inhibitor (omeprazole 40 mg IV BID) for peptic ulcer disease 8
• Avoid aspirin and NSAIDs 3

Surgical Consultation

Indications for surgical evaluation: 1

• Irretrievable foreign body
• Esophageal perforation (suggested by mediastinal air, pleural effusion)
• Foreign body near vital structures
• Severe bleeding uncontrolled by endoscopy
• Malignancy requiring resection

Why NOT Outpatient Management

This patient cannot be safely managed as outpatient because: 1

1. Rockall score ≥3 indicates need for intervention and hospitalization 1
2. VWF deficiency requires specialized hemostatic preparation before any procedure 1, 3
3. Suspected foreign body on imaging requires urgent endoscopic removal within 24 hours 1
4. Barrett's esophagitis with new symptoms mandates exclusion of malignancy 1
5. Melena indicates significant upper GI bleeding requiring monitoring and potential transfusion 1

Common Pitfalls to Avoid

• Never perform endoscopy without correcting VWF deficiency first - target VWF activity ≥50 IU/dL 3, 7
• Do not assume DDAVP will work - must document prior response or perform test dose 5
• Do not use DDAVP in type 2B VWD - causes dangerous thrombocytopenia 2
• Do not delay endoscopy beyond 24 hours with suspected foreign body - risk of perforation increases 1
• Do not rely on normal hemoglobin to exclude significant bleeding - may not drop acutely 1
• Do not forget fluid restriction with DDAVP - hyponatremia risk, especially age 63 5

Disposition

Admit to hospital with:

• Hematology co-management for VWF deficiency
• Gastroenterology for urgent endoscopy within 24 hours
• Surgical consultation available if complications arise
• Continuous monitoring until hemostasis confirmed and hemoglobin stable