A 48-year-old Hispanic female patient presented to the Emergency Department (ED) for hematemesis for the past 2 days associated with generalized weakness, abdominal pain, nausea, and subjective fevers. The patient’s initial vital signs were significant for a heart rate of 127 beats per minute, blood pressure of 92/62 mmHg, temporal temperature of 36.6 °C, respiration rate of 20 breaths per minute, and an oxygen saturation of 100% on room air. Past medical history was significant for childhood seizures, and a cerebral vascular accident more than 15 years ago with mild residual left side weakness. Her physical examination on arrival at the ED was notable for significant skin pallor and diffuse abdominal tenderness that was most pronounced in the epigastric area and left lower quadrant. No costovertebral angle tenderness, rebound tenderness, or guarding were noted. The remainder of her physical examination was unremarkable. Given the reported history of hematemesis with tachycardia, a complete blood count, comprehensive metabolic panel, lipase, abdominal X-ray, and urinalysis were ordered. Laboratory studies were significant for a venous lactate of 3.53 mmol/L, hemoglobin of 9.2 g/dL, hematocrit of 26%, leukocytosis at white cell count of 23.7 × 109/L, creatinine level at 4.23 mg/dL, and estimated glomerular filtration rate (eGFR) of 12.3 mL/min. An abdominal portable kidney, ureter, and bladder x-ray showed a nonobstructive bowel gas pattern (Fig. 1).
Abdominal X-ray demonstrating nonobstructive gas pattern
Her electrocardiogram revealed sinus tachycardia with no acute ST segment changes. The patient was unable to urinate during this time to provide a sample for analysis. Owing to concern for acute kidney injury with creatinine of 4.23 mg/dL and an eGFR of 12.3 mL/min, the decision was made to perform a CT of the abdomen and pelvis without contrast. The CT of the abdomen indicated probable large bilateral angiomyolipomas in the kidneys with hemorrhage, left worse than right (Fig. 2). Normal renal tissue could not be visualized on the CT owing to angiomyolipomas. The lack of intravenous (IV) contrast limited the evaluation of active hemorrhage in the retroperitoneal area.
Computed tomography scan demonstrating large bilateral angiomyolipomas of the kidneys with hemorrhage
Subsequently, the patient was started on IV fluids resulting in improved tachycardia. Following the results of CT abdomen and pelvis demonstrating signs of hemorrhage, interventional radiologist was consulted regarding possible selective arterial embolization. Interventional radiologist did not recommend any acute intervention at that time. The recommendation was to continue to trend hemoglobin and hematocrit every 4 h, repeat creatinine after adequate fluid resuscitation, and admit the patient for observation. If the patient demonstrated clinical evidence of persistent bleeding, reconsultation for possible embolization was recommended. General surgery and nephrology were also consulted, with no acute surgical intervention and no emergent indication for hemodialysis recommended at that time.
The patient was admitted to the internal medicine for close monitoring owing to concern for active hemorrhage of angiomyolipomas with concern of impending hemorrhagic shock. Repeat laboratory studies performed 3 hours later showed that the patient’s hemoglobin and hematocrit had trended down to levels of 6.1 g/dL and 18%, respectively. The patient was transfused with two units of packed red blood cells and subsequently underwent bilateral arterial embolization of left lower pole renal artery, left upper pole renal artery, left pole segmental renal artery, and right upper pole renal artery by interventional radiology team.
Serial serum studies after the embolization demonstrated that the patient’s hemoglobin and hematocrit had stabilized. Her kidney function showed minimal improvement during the course of her hospital stay. The patient did not have any further hematemesis and was discharged with close nephrology follow-up for likely necessary hemodialysis in the future along with consideration of kidney transplant. She was advised to seek further genetic counseling for potential undiagnosed tuberous sclerosis owing to her history of childhood seizures and bilateral AMLs.
