Major orthopedic surgery in a patient with fibromuscular dysplasia in the right renal artery and mild hemophilia A: First case reported

Fernández Caballero, Mariana; Collado, Fernando; Navarro, José-Tomás

doi:10.1016/j.medcli.2020.09.015

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José-Tomás Navarro" "autores" => array:3 [ 0 => array:4 [ "nombre" => "Mariana" "apellidos" => "Fernández Caballero" "email" => array:1 [ 0 => "marifc87@gmail.com" ] "referencia" => array:3 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:2 [ "etiqueta" => "*" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Fernando" "apellidos" => "Collado" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "c" "identificador" => "aff0015" ] ] ] 2 => array:3 [ "nombre" => "José-Tomás" "apellidos" => "Navarro" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "b" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Hematology Laboratory, ICO-Badalona, Hospital Germans Trias i Pujol, Barcelona, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Josep Carreras Leukaemia Research Institute, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Department of Traumatology, Hospital Germans Trias i Pujol, Badalona, Barcelona, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Paciente con displasia fibromuscular en arteria renal derecha y hemofilia A leve intervenido de cirugía ortopédica mayor: primer caso reportado" ] ] "textoCompleto" => "Fibromuscular dysplasia (FMD) is a rare, idiopathic, nonatherosclerotic, noninflammatory arterial disease that results in stenosis, aneurysm, dissection, occlusion, or arterial tortuosity, and as a consequence, thrombosis or bleeding complications.<a class="elsevierStyleCrossRef" href="#bib0030">1</a>On the other hand, mild hemophilia A is an X-linked congenital bleeding disorder caused by mutations in F8 gene, that results in levels of FVIII:C>5IUdL−1 and <40IUdL−1 (5–40%).<a class="elsevierStyleCrossRef" href="#bib0035">2</a>Herein we describe the case of a 61 year-old patient with FMD in the right renal artery and mild hemophilia A who underwent major orthopedic surgery.He was diagnosed with mild hemophilia A, after a traumatic bleeding, when he was 19-year-old, (baseline FVIII levels of 30–40%). Later on, he presented minor post-traumatic hemorrhages, hemarthroses in the right knee, and various surgical interventions in this joint, for which he received either desmopressin or FVIII concentrates.In July 2017, he was diagnosed with FMD in the right renal artery, with renal and splenic infarction. The renal involvement was a result of dissection of the right renal artery, that required stent placement, and double antiplatelet therapy for 1 year (clopidogrel 75mg per day and acetylsalicylic acid 100mg per day).<a class="elsevierStyleCrossRef" href="#bib0040">3</a> In March 2018, he presented gastrointestinal hemorrhage of unknown origin, and clopidogrel was discontinued.Due to osteoarthritis in the right knee, in January 2019, the patient underwent surgery for replacement with a total prosthesis in this joint. In the immediate postoperative period, despite maintaining levels of FVIII:C>100%, the patient developed a large intramuscular hematoma in the right quadriceps, approximately 34cm of diameter, secondary to the presence of an 18mm diameter pseudoaneurysm dependent on a distal branch of the superficial femoral artery. This vessel was embolized with resolution of the bleeding, but resulting in a torpid postoperative course, with infection of the prosthesis by Staphylococcus epidermidis, sensitive to vancomycin and rifampin.He was referred to our center in May 2020, where it was decided to remove the prosthetic elements from the right knee. At that time, baseline FVIII:C levels were of 39%.Regarding the hemostasis, he discontinued acetylsalicylic acid 3 days before surgery, and as a major surgery, recombinant FVIII was administered to ensure levels of 80–100% during the first 3–5 days.<a class="elsevierStyleCrossRef" href="#bib0035">2</a>Prior the surgery, 3000IU of recombinant FVIII (50 UI/kg) was administered, reaching levels of FVIII:C greater of 113%, as well as 500mg of tranexamic acid intravenously. Ischemia (100mmHg higher than systolic blood pressure) was performed for 111min without incidents, later bleeding was checked, and hemostasis was performed prior to surgery closure. Immediately to closure, 2500mg of tranexamic acid in 10mL SF was infiltrated locally, as protocol. Post-surgery FVIII:C levels were 90%, so we administered 1000IU of FVIII more, and every 12h for the first 5 days, as well as tranexamic acid every 8h. As prophylactic antithrombotic measure, the patient wore mechanical compression stockings. In the 48h after surgery, the round could be removed, and rehabilitation could be started. On post-surgery day +7, the 24h residual levels of FVIII:C were 64%, hence, on day +8 the patient was discharged with the prescription of intranasal desmopressin, 300 mcg every 24h, but only for 3 days due to tachyphylaxis effects. In order to reduce the thrombotic risk, tranexamic acid was stopped at discharge while acetylsalicylic acid (100mg every 24h) and low molecular weight heparin (LMWH), enoxaparin 40mg per day, restarted.<a class="elsevierStyleCrossRef" href="#bib0045">4</a>On day +14 the level of FVIII:C was 49.9%, after that, the patient had not present bleeding complications, had not require administration of recombinant FVIII or desmopressin, and no inhibitor was developed.To our knowledge, this is the first reported case of FDM and mild hemophilia A. The conjunction of these two pathologies makes our patient a case with difficult hemostatic management during the course of a major surgery. Treatment with tranexamic acid and recombinant FVIII seems to be feasible, as it reinforces clot stability, and compensate for the poor quality of the blood vessels, with no thrombotic complications during or after the procedure.<a class="elsevierStyleCrossRef" href="#bib0050">5</a> This case illustrates the complexity of patients with mild coagulopathies who have other associated disorders in the blood vessel wall.Ethics approvalThis article does not contain any studies with human participants or animals performed by any of the authors.FundingNot applicable.Conflicts of interestThe authors have no conflicts of interest." "textoCompletoSecciones" => array:1 [ "secciones" => array:5 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Ethics approval" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Funding" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Conflicts of interest" ] 3 => array:2 [ "identificador" => "xack561453" "titulo" => "Acknowledgments" ] 4 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0030" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Fibromuscular dysplasia: contemporary concepts and future directions" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "N. Narula" 1 => "D. Kadian-Dodov" 2 => "J.W. Olin" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:4 [ "tituloSerie" => "Prog Cardiovasc Dis" "fecha" => "2018" "paginaInicial" => "580" "paginaFinal" => "585" ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0035" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "The definition, diagnosis and management of mild hemophilia A: communication from the SSC of the ISTH" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "M. Makris" 1 => "J. Oldenburg" 2 => "E.P. Mauser-Bunschoten" 3 => "K. Peerlinck" 4 => "G. Castaman" 5 => "K. Fijnvandraat" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/jth.14315" "Revista" => array:6 [ "tituloSerie" => "J Thromb Haemost" "fecha" => "2018" "volumen" => "16" "paginaInicial" => "2530" "paginaFinal" => "2533" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/30430726" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0040" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "First International Consensus on the diagnosis and management of fibromuscular dysplasia" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "H.L. Gornik" 1 => "A. Persu" 2 => "D. Adlam" 3 => "L.S. Aparicio" 4 => "M. Azizi" 5 => "M. Boulanger" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Vasc Med (United Kingdom)" "fecha" => "2019" "volumen" => "24" "paginaInicial" => "164" "paginaFinal" => "189" ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0045" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Acute ST-elevation myocardial infarction due to in-stent thrombosis after administering tranexamic acid in a high cardiac risk patient" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "Y.E. Kaptein" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:4 [ "tituloSerie" => "BMJ Case Rep" "fecha" => "2019" "volumen" => "12" "paginaInicial" => "227957" ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0050" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:1 [ "referenciaCompleta" => "Franchini M, Favaloro EJ, Lippi G. Mild hemophilia A. J Thromb Haemost. 2010; 421–32." ] ] ] ] ] ] ] "agradecimientos" => array:1 [ 0 => array:4 [ "identificador" => "xack561453" "titulo" => "Acknowledgments" "texto" => "We thank Dr. Jose Mateo and Dr. Marina Carrasco (Hemostasis and Thrombosis Unit, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain) for their collaboration in this case report." "vista" => "all" ] ] ] "idiomaDefecto" => "en" "url" => "/00257753/0000015700000008/v1_202110170505/S0025775320307533/v1_202110170505/en/main.assets" "Apartado" => array:4 [ "identificador" => "66430" "tipo" => "SECCION" "es" => array:2 [ "titulo" => "Cartas al Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "es" ] "PDF" => "https://static.elsevier.es/multimedia/00257753/0000015700000008/v1_202110170505/S0025775320307533/v1_202110170505/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0025775320307533?idApp=UINPBA00004N"]

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