Severe FVII deficiency presenting with Heterotopic gastric mucosa: Hoofbeats may be a zebra

Abstract

Gastrointestinal (GI) tract bleeding can present as symptomatic anemia in children. This presentation warrants evaluation for GI tract pathology and consideration of coagulation assays to rule out an underlying bleeding diathesis. A male child was diagnosed with a rare bleeding disorder, severe (<1%) factor VII (FVII) deficiency during evaluation of severe iron deficiency anemia (IDA) secondary to recurrent GI bleeding. Despite adequate prophylaxis for the underlying bleeding disorder, continued GI bleeding and anemia led to further evaluation and identification of a rare small intestinal tract lesion, heterotopic gastric mucosa (HGM). Surgical treatment of the lesion resulted in complete symptom resolution and abated the need for prophylactic factor replacement therapy.