What is Cullen sign?

Cullen Sign: Clinical Significance and Diagnosis

Cullen sign is a periumbilical ecchymosis (bruising around the umbilicus) that indicates intra-abdominal or retroperitoneal hemorrhage, most commonly associated with acute hemorrhagic pancreatitis but can occur in various other serious conditions. 1, 2

Definition and Appearance

• Cullen sign appears as superficial edema with bruising in the subcutaneous fatty tissue around the periumbilical region 3
• It represents blood tracking along the round ligament to the umbilicus 4
• The ecchymosis typically manifests as bluish discoloration around the umbilicus 2, 5

Clinical Significance

• Cullen sign is an important physical examination finding that may indicate serious underlying pathology 1, 3
• It is most frequently associated with acute hemorrhagic pancreatitis but is not specific to this condition 6
• The presence of Cullen sign should prompt immediate investigation for potential life-threatening conditions 3, 4

Common Etiologies

• Acute hemorrhagic pancreatitis (most commonly recognized cause) 1, 2
• Ruptured ectopic pregnancy 7
• Leaking or ruptured abdominal aortic aneurysm 3
• Ruptured iliac artery aneurysm 4
• Splenic rupture 6
• Intra-abdominal malignancy with hemorrhage 8
• Other sources of retroperitoneal or intraperitoneal hemorrhage 4

Diagnostic Considerations

• Cullen sign may be accompanied by Grey-Turner sign (flank ecchymosis), which also indicates retroperitoneal hemorrhage 1, 7
• The clinical diagnosis of acute pancreatitis is often unreliable if made on clinical findings alone, including Cullen sign 1
• When Cullen sign is observed, appropriate diagnostic testing should include:
  • Serum amylase and lipase measurements 1, 5
  • Abdominal imaging (ultrasound, CT scan) to identify the source of hemorrhage 5, 3
  • In women of childbearing age, pregnancy testing to rule out ruptured ectopic pregnancy 7

Prognostic Implications

• In cases of malignancy, Cullen sign may be considered a pre-terminal finding 8
• In acute pancreatitis, Cullen sign suggests hemorrhagic pancreatitis, which carries a higher mortality risk 1, 5
• When associated with ruptured aneurysms, it indicates significant blood loss requiring urgent intervention 4

Clinical Pearls

• Cullen sign may develop hours to days after the initial hemorrhagic event 3
• The presence of both Cullen sign and Grey-Turner sign increases the specificity for significant intra-abdominal or retroperitoneal hemorrhage 1, 7
• When evaluating a patient with Cullen sign, clinicians should maintain a broad differential diagnosis beyond just pancreatitis 6, 4

Remember that while Cullen sign is classically associated with acute hemorrhagic pancreatitis, its presence should trigger consideration of multiple potential life-threatening conditions requiring prompt diagnosis and management.