Minimally invasive surgical techniques for hemostasis in Osler–Weber–Rendu disease
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University of Medicine and Pharmacy ‘‘Carol Davila’’, Bucharest, Romania
Institute of Phonoaudiology and Functional ENT Surgery ‘‘Prof Dr D. Hociota’’, Bucharest, Romania
Andreea Sorică   

University of Medicine and Pharmacy ‘‘Carol Davila’’, 1 Mai Bld. 22, District 6, Bucharest 062631, Romania. Tel.: +40 723 003 513.
Submission date: 2014-07-09
Acceptance date: 2015-03-17
Online publication date: 2015-04-20
Publication date: 2020-03-24
Pol. Ann. Med. 2015;22(1):50–54
Osler–Weber–Rendu disease, also called ‘‘hereditary hemorrhagic telangiectasia’’ (HHT), is an autosomal dominant genetic disorder characterized by the appearance of small disseminated vascular malformations in the skin, mucous membranes and organs (lungs, brain, liver) with repeated bleeding tendency. Most common symptom is epistaxis. Argon plasma coagulation (APC) is an on-contact electrosurgical monopolar thermal procedure used for achieving hemostasis. The principle of this method consists in transferring energy to a target tissue via plasma, the fourth fundamental state of matter. To achieve coagulation, argon (an inert, non-toxic gas) is delivered through a probe and ionized by a discharge current elective high voltage. Electrical energy is transferred to the tissue through the argon plasma.

The authors present a clinical case of a HHT where a minimally invasive surgical technique based on APC proved to be effective for controlling repeated bleeding of nasal origin.

Case study:
We present the case of P.L. patient, aged 71, from rural environment, presented to the emergency room of our clinic for active bilateral anterior epistaxis. The patient reports a long history of the pathology, starting approximately 23 years ago and characterized by the existence of frequent episodes of recurrent epistaxis.

Results and discussion:
Immediate and late postoperative results are presented and compared. The APC proved to be effective both in the short and long term, in increasing bleeding-free interval, and in decreasing the frequency and intensity of episodes of nasal bleeding origin.

The presented clinical case with favorable outcome highlights the role of a modern surgical technology – APC – effective in achieving nose hemostasis. In the presented case, APC has brought many advantages in achieving hemostasis: non-contact coagulation with minimal tissue penetration and destruction, a safe method with minimal complications, decreased risk of perforation.

None declared.
Grover S, Grewal RS, Verma R, Sahni H, Muralidhar R, Sinha P. Osler–Weber–Rendu syndrome: a case report with familial clustering. Indian J Dermatol Venereol Leprol. 2009;75(1):100–101.
Adler DG, Leighton JA, Davila RE, et al. ASGE guideline: the role of endoscopy in acute non-variceal upper-GI hemorrhage. Gastrointest Endosc. 2004;60(4):497–504.
Zucco L, Zhang Q, Kuliszewski MA, et al. Circulating angiogenic cell dysfunction in patients with hereditary hemorrhagic telangiectasia. PLOS ONE. 2014;9(2):e89927.
Kjeldsen AD, Oxhøj H, Andersen PE, Elle B, Jacobsen JP, Vase P. Pulmonary arteriovenous malformations: screening procedures and pulmonary angiography in patients with hereditary hemorrhagic telangiectasia. Chest. 1999;116(2):432–439.
Dakeishi M, Shioya T, Wada Y, et al. Genetic epidemiology of hereditary hemorrhagic telangiectasia in a local community in the northern part of Japan. Hum Mutat. 2002;19(2):140–148.
Shovlin CL, Letarte M. Hereditary haemorrhagic telangiectasia and pulmonary arteriovenous malformations: issues in clinical management and review of pathogenic mechanisms. Thorax. 1999;54(8):714–729.
Choi EJ, Chen W, Jun K, Arthur HM, Young WL, Su H. Novel brain arteriovenous malformation mouse models for type 1 hereditary hemorrhagic telangiectasia. PLOS ONE. 2014;9(2):e88511.
McDonald J, Bayrak-Toydemir P, Pyeritz RE. Hereditary hemorrhagic telangiectasia: an overview of diagnosis, management, and pathogenesis. Genet Med. 2011;13(7):607–616.
Lessnau KD, Lanza J, Thirumala RRD, Izaguirre DE, Lopez Rowe V. Osler–Weber–Rendu Disease. 2014 Accessed 03.03.15.
Shovlin CL, Guttmacher AE, Buscarini E, et al. Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu–Osler–Weber syndrome). Am J Med Genet. 2000;91(1):66–67.
Urushihara M, Furukawa S, Ota A, Iwai A, Matsumura K, Hamada Y. Hemorrhagic telangiectasia with thrombocytopenia in a newborn infant. Pediatr Int. 2000;42(6):693–695.
Rebeiz EE, Bryan DJ, Ehrlichman RJ, Shapshay SM. Surgical management of life-threatening epistaxis in Osler–Weber–Rendu disease. Ann Plast Surg. 1995;35(2):208–213.
Govani FS, Shovlin CL. Hereditary haemorrhagic telangiectasia: a clinical and scientific review. Eur J Hum Genet. 2009;17(7):860–871.
Dupuis-Girod S, Bailly S, Plauchu H. Hereditary hemorrhagic telangiectasia (HHT): from molecular biology to patient care. J Thromb Haemost. 2010;8(7):1447–1456.
Schoen FJ, Cotran RS, Vinay K, Collins T. Robbins Pathologic Basis of Disease. 5th ed. Philadelphia: WB Saunders; 1994:509.
Berg J, Porteous M, Reinhardt D, et al. Hereditary haemorrhagic telangiectasia: a questionnaire based study to delineate the different phenotypes caused by endoglin and ALK1 mutations. J Med Genet. 2003;40(8):585–590.
Lacombe P, Lagrange C, Beauchet A, El Hajjam M, Chinet T, Pelage JP. Diffuse pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: long-term results of embolization according to the extent of lung involvement. Chest. 2009;135(4):1031–1037.
Jakobi P, Weiner Z, Best L, Itskovitz-Eldor J. Hereditary hemorrhagic telangiectasia with pulmonary arteriovenous malformations. Obstet Gynecol. 2001;97(5 Pt 2):813–814.
McDonald MJ, Brophy BP, Kneebone C. Rendu–Osler–Weber syndrome: a current perspective on cerebral manifestations. J Clin Neurosci. 1998;5(3):345–350.
Khalid SK, Pershbacher J, Makan M, Barzilai B, Goodenberger D. Worsening of nose bleeding heralds high cardiac output state in hereditary hemorrhagic telangiectasia. Am J Med. 2009;122(8):779.e1–779.e9.
Yaniv E, Preis M, Hadar T, Shvero J, Haddad M. Antiestrogen therapy for hereditary hemorrhagic telangiectasia: a doubleblind placebo-controlled clinical trial. Laryngoscope. 2009;119(2):284–288.
Isaacs E. Aminocaproic acid. In: Pediatric Drug Dosage Handbook. 8th ed. Ottawa, Canada: Winnipeg Health Sciences Center and CSHP; 1998:161.
Franchini M, Frattini F, Crestani S, Bonfanti C. Novel treatments for epistaxis in hereditary hemorrhagic telangiectasia: a systematic review of the clinical experience with thalidomide. J Thromb Thrombolysis. 2013;36(3):355–357.
de Gussem EM, Snijder RJ, Disch FJ, Zanen P, Westermann CJ, Mager JJ. The effect of N-acetylcysteine on epistaxis and quality of life in patients with HHT: a pilot study. Rhinology. 2009;47(1):85–88.
Massoud OI, Youssef WI, Mullen KD. Resolution of hereditary hemorrhagic telangiectasia and anemia with prolonged alpha-interferon therapy for chronic hepatitis C. J Clin Gastroenterol. 2004;38(4):377–379.
Wheatley-Price P, Shovlin C, Chao D. Interferon for metastatic renal cell cancer causing regression of hereditary hemorrhagic telangiectasia. J Clin Gastroenterol. 2005;39(4):344–345.
Harvey RJ, Kanagalingam J, Lund VJ. The impact of septodermoplasty and potassium-titanyl-phosphate (KTP) laser therapy in the treatment of hereditary hemorrhagic telangiectasia-related epistaxis. Am J Rhinol. 2008;22(2):182–187.
Layton KF, Kallmes DF, Gray LA, Cloft HJ. Endovascular treatment of epistaxis in patients with hereditary hemorrhagic telangiectasia. AJNR Am J Neuroradiol. 2007;28(5):885–888.
Lesnik GT, Ross DA, Henderson KJ, Joe JK, Leder SB, White Jr RI. Septectomy and septal dermoplasty for the treatment of severe transfusion-dependent epistaxis in patients with hereditary hemorrhagic telangiectasia and septal perforation. Am J Rhinol. 2007;21(3):312–315.
Grund KE, Storek D, Farin G. Endoscopic argon plasma coagulation (APC) first clinical experiences in flexible endoscopy. Endosc Surg Allied Technol. 1994;2(1):42–46.