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Renal angiomyolipoma rupture in a young female with COVID-19

Published:February 11, 2021DOI:https://doi.org/10.1016/j.ajem.2021.02.012

      Abstract

      While primarily a respiratory illness, infection with the novel coronavirus (COVID-19) is associated with pathologic changes in normal coagulation, characterized by both thromboembolic and bleeding events. We present the case of a 22-year-old female diagnosed with renal angiomyolipoma (AML) rupture 2 weeks after COVID-19 infection, ultimately requiring admission for hemorrhage control via endovascular embolization. Emergency medicine physicians should maintain a high index of suspicion for renal AML rupture and other spontaneous bleeding events in patients with recent COVID-19 infection due to a possible correlation between the two.

      Keywords

      1. Background

      While primarily a respiratory illness, infection with the novel coronavirus (COVID-19) is associated with both thromboembolic and bleeding events [
      • Chen N.
      • Zhou M.
      • Dong X.
      • et al.
      Epidemiological and clinical characteristics of 99 cases of 2019 novel coronavirus pneumonia in Wuhan, China: a descriptive study.
      ,
      • Klok F.A.
      • Kruip M.
      • van der Meer N.J.M.
      • et al.
      Confirmation of the high cumulative incidence of thrombotic complications in critically ill ICU patients with COVID-19: an updated analysis.
      ,
      • Agarwal S.
      • Jain R.
      • Dogra S.
      • et al.
      Cerebral microbleeds and leukoencephalopathy in critically ill patients with COVID-19.
      ,
      • Long A.
      • Grimaldo F.
      Spontaneous hemopneumothorax in a patient with COVID-19: a case report.
      ,
      • Al-Samkari H.
      • Leaf R.S.
      • Dzik W.H.
      • et al.
      COVID-19 and coagulation: bleeding and thrombotic manifestations of SARS-COV-2 infection.
      ]. The pathologic clotting associated with COVID-19 has been established in the literature, however, there is emerging data suggesting a concomitant risk of hemorrhage. The association with bleeding is most prevalent in critically ill patients, though it has been described in non-critically ill patients, such as a case of spontaneous kidney hematoma [
      • Al-Samkari H.
      • Leaf R.S.
      • Dzik W.H.
      • et al.
      COVID-19 and coagulation: bleeding and thrombotic manifestations of SARS-COV-2 infection.
      ,
      • Scialpi M.
      • Russo P.
      • Piane E.
      • Gallo E.
      • Scalera G.B.
      First case of retroperitoneal hematoma in COVID-19.
      ]. Renal angiomyolipoma (AML) are typically benign neoplasms which rarely present with rupture and hemorrhagic shock. We describe a case of a 22-year-old female who was diagnosed with a ruptured renal AML two weeks after COVID-19 infection.

      2. Case report

      A 22-year-old female presented to the emergency department with acute-onset left flank pain. Her pain began while at rest, reached maximum intensity within minutes, radiated to her left shoulder, and was described as having been “kicked in the back.” The patient denied fevers, nausea, vomiting, dysuria, and hematuria. Two weeks prior, the patient had presented to the ED with five days of cough, shortness of breath, myalgias, and fevers, and was subsequently diagnosed with COVID-19 via nasopharyngeal PCR assay. Her medical history was notable for iron deficiency anemia and an appendectomy. Her only medication was an oral combined estrogen-progesterone contraceptive. She denied family history of renal disease or coagulopathic disorders.
      Her initial vital signs included: heart rate 109, blood pressure 135/84, oxygen saturation 99%, respiratory rate 18, and temperature 36.9C. An abdominal exam revealed a soft, nondistended abdomen with focal left upper quadrant tenderness. There was no palpable mass, rebound, or guarding. The patient also had severe tenderness upon percussion of the left costovertebral angle. A tachycardic rate with normal rhythm was auscultated on cardiopulmonary exam, otherwise the remainder of her physical exam was unremarkable. Laboratory studies revealed: white blood cell count 13.8 cells/mm3, hemoglobin 13.4 g/dl, PT 14 s, APTT 35.4 s, INR 1.1. Urine studies showed microhematuria and a negative pregnancy test. A chest x-ray was unremarkable. Non-contrast computed tomography of her abdomen and pelvis demonstrated left peri-renal moderate volume hemorrhage with a fat dense lesion in the left kidney measuring 2.5 × 2.3 × 2.1 cm, consistent with ruptured renal AML. After this discovery a type and screen was ordered, urology and interventional radiology were consulted, and the patient was admitted to the intensive care unit. Interventional radiology performed embolization 12 h after admission. The intraprocedural evaluation of the renal artery system found no aneurysmal dilatation.
      The patient's post-procedural course was complicated by continued hemorrhage and retroperitoneal hematoma, with a hemoglobin nadir of 6.8 g/dl requiring transfusion of 2 units packed red blood cells. She also developed diaphragmatic irritation and required supplemental oxygen by nasal canula. On hospital day 6 she developed respiratory COVID-19 symptoms and again tested positive by PCR assay for COVID-19. She was discharged on hospital day 8 with oral analgesics.

      3. Discussion

      Renal AML is a benign neoplasm caused by proliferation of epithelioid cells and composed of adipose tissue, blood vessels, and smooth muscle [
      • Bissler J.J.
      • Kingswood J.C.
      Renal angiomyolipomata.
      ]. The incidence is approximately 0.13% with a female predominance [
      • Flum A.S.
      • Hamoui N.
      • Said M.A.
      • et al.
      Update on the diagnosis and management of renal angiomyolipoma.
      ,
      • Fujii Y.
      • Ajima J.
      • Oka K.
      • Tosaka A.
      • Takehara Y.
      Benign renal tumors detected among healthy adults by abdominal ultrasonography.
      ]. 80% of renal AMLs develop sporadically while the remainder are associated with tuberous sclerosis. Renal AMLs larger than 4 cm have a significantly higher risk of rupture, however, rupture can occur at smaller sizes and larger AMLs may remain stable [
      • Flum A.S.
      • Hamoui N.
      • Said M.A.
      • et al.
      Update on the diagnosis and management of renal angiomyolipoma.
      ,
      • Xu X.F.
      • Hu X.H.
      • Zuo Q.M.
      • Zhang J.
      • Xu H.Y.
      • Zhang Y.
      A scoring system based on clinical features for the prediction of sporadic renal angiomyolipoma rupture and hemorrhage.
      ]. The median age at time of solitary renal AML rupture is 50 years old [
      • Xu X.F.
      • Hu X.H.
      • Zuo Q.M.
      • Zhang J.
      • Xu H.Y.
      • Zhang Y.
      A scoring system based on clinical features for the prediction of sporadic renal angiomyolipoma rupture and hemorrhage.
      ,
      • Nason G.J.
      • Morris J.
      Bhatt JR, et al.
      ]. Over 90% of renal AMLs are discovered incidentally on imaging, though some patients may present with Wunderlich syndrome, spontaneous, nontraumatic hemorrhage into the perinephric space characterized by the triad of flank pain, flank mass, and hypovolemic shock [
      • Nason G.J.
      • Morris J.
      Bhatt JR, et al.
      ,
      • Kim J.W.
      • Kim J.Y.
      • Ahn S.T.
      • et al.
      Spontaneous perirenal hemorrhage (Wunderlich syndrome): an analysis of 28 cases.
      ]. Spontaneous rupture has also been associated with coagulopathic states such as pregnancy [
      • Zhang T.
      • Xue S.
      • Wang Z.M.
      • Duan X.M.
      • Wang D.X.
      Diagnostic value of ultrasound in the spontaneous rupture of renal angiomyolipoma during pregnancy: a case report.
      ,
      • Snow A.
      • Halpenny D.
      • McNeill G.
      • Torreggiani W.C.
      Life-threatening rupture of a renal angiomyolipoma in a patient taking over-the-counter horse chestnut seed extract.
      ,
      • Heck A.M.
      • DeWitt B.A.
      • Lukes A.L.
      Potential interactions between alternative therapies and warfarin.
      ].
      A statement of causality cannot be made from a single incident of a ruptured renal AML rupture following a COVID-19 infection, though the timing is notable. The patient had none of the previously mentioned risk factors, including pregnancy, aneurysm, or AML size greater than 4 cm. Other spontaneous bleeding complications have been described after infection with COVID-19, such as retroperitoneal hematoma, gastrointestinal bleeding, hemopneumothorax, and cerebral hemorrhage [
      • Long A.
      • Grimaldo F.
      Spontaneous hemopneumothorax in a patient with COVID-19: a case report.
      ,
      • Al-Samkari H.
      • Leaf R.S.
      • Dzik W.H.
      • et al.
      COVID-19 and coagulation: bleeding and thrombotic manifestations of SARS-COV-2 infection.
      ,
      • Flum A.S.
      • Hamoui N.
      • Said M.A.
      • et al.
      Update on the diagnosis and management of renal angiomyolipoma.
      ,
      • Gadiparthi C.
      • Perisetti A.
      • Sayana H.
      • Tharian B.
      • Inamdar S.
      • Korman A.
      Gastrointestinal bleeding in patients with severe SARS-CoV-2.
      ,
      • Li J.
      • Long X.
      • Zhu C.
      • et al.
      A case of COVID-19 pneumonia with cerebral hemorrhage.
      ]. Proposed mechanisms of COVID-associated coagulopathy include direct infection of endothelial cells via ACE-2 receptors and delayed autoantibody development against endothelial cells [
      • Iba T.
      • Levy J.H.
      • Levi M.
      • Thachil J.
      Coagulopathy in COVID-19.
      ,
      • Yang Y.H.
      • Huang Y.H.
      • Chuang Y.H.
      • et al.
      Autoantibodies against human epithelial cells and endothelial cells after severe acute respiratory syndrome (SARS)-associated coronavirus infection.
      ]. Prolonged PT, APTT, and INR and elevated D-dimer are associated with poor COVID-19 outcomes and can be considered by EM physicians concerned for a COVID-related coagulopathy [
      • Lee A.Y.Y.
      • Connors J.M.
      • Kreuziger L.B.
      • et al.
      COVID-19 and Coagulopathy: Frequently Asked Questions.
      ].
      Rupture of a renal AML may mimic other diagnoses and can lead to life-threatening hemorrhagic shock. Understanding the possibility of increased bleeding risk in patients with recent COVID-19 infection, emergency medicine physicians should maintain a high index of suspicion for such spontaneous events. Emergency treatment considerations include blood product transfusion, reversal of anticoagulant medications, and expedient consultation to surgical, critical care, and interventional radiology services.
      Unlabelled Image
      Unlabelled Image
      Images: non-contrast CT of the abdomen and pelvis demonstrating left kidney with a cortically based, interpolar 2.5 × 2.3 cm mass with central fat density. Associated moderate volume perirenal hematoma and edema.

      Funding

      None

      Disclaimer

      The views expressed herein are those of the authors and do not necessarily reflect the official policy or position of the Department of the Navy, Department of Defense, or the United States Government.

      Uncited reference

      [
      • Degeneffe A.
      • Bruneau M.
      • Spitaels J.
      • Gilis N.
      • De Witte O.
      • Lubansu A.
      Acute hemorrhage after Intracerebral biopsy in COVID-19 patients: report of 3 cases.
      ]

      Declaration of Competing Interest

      None

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