Osteogenezis İmperfektalı Çocuk Hastada Anestezi Yönetimi
Özet
Altı yaşında, 13 kg ağırlığında, 90 cm boyunda, Osteogenezis imperfekta (Oİ) tanılı kadın hastaya sağ femur malunion nedeniyle cerrahi planlandı. Daha önce humerus ve femur fraktür öyküsü olan hastanın preoperatif muayenesinde Mallampati II, kısa boy, gelişme geriliği alt ve üst ekstremitelerde şekil bozukluğu ve kısalık, diş çürükleri, mavi sklera ve skolyozu mevcuttu. Yapılan rutin laboratuvar tetkikleri normal sınırlarda olan hasta Amerikan Anestezistler Derneği (ASA) ASA III olarak değerlendirilip operasyona alındı. Malign hipertermi (MH) oluşma riskine karşı gerekli hazırlıklar yapıldı ve yeni kırık oluşmaması için dikkatli bir şekilde operasyon masasına alındı. Genel anestezide kullanılan elektrokardiyogram, pulsoksimetre, noninvaziv kan basıncı (KB) ve özofagial ısı monitörizasyonu yapıldı. Preoksijenizasyonu takiben midazolam, remifentanil, propofol, roküronyum bromür ile indüksiyonu sağlandı ve sonrasında dikkatli bir şekilde videolaringoskop ile entübasyonu gerçekleştirildi. Anestezi idamesi için propofol ve remifentanil ile total intravenöz anestezi (TİVA) kullanıldı. Yakın hemodinamik takip için invaziv arteriyal monitörizasyon yapıldı. Yaklaşık 3 saat 10 dk süren operasyonda vücut ısısında da herhangi bir değişiklik saptanmadı ve vital bulguları stabil seyreden hasta dikkatli bir şekilde ekstübe edilerek postanestezik yoğun bakım ünitesine (PABÜ) alındı.
Surgery was planned due to right femur malunion for a six-year-old female patient, weighing 13 kg and 90 cm in height, diagnosed with Osteogenesis Imperfecta (OI). In the preoperative examination of the patient, who had a history of previous humerus and femur fractures, Mallampati II, short stature, developmental delay, deformity and shortness in the upper and lower extremities, dental caries, blue sclera, and scoliosis were present. The patient, whose routine laboratory tests were within normal limits, was evaluated as American Society of Anesthesiologists (ASA) class III and taken into operation. Necessary preparations were made against the risk of malignant hyperthermia (MH) development, and the patient was carefully placed on the operating table to prevent the occurrence of new fractures. Electrocardiogram, pulse oximetry, noninvasive blood pressure (BP), and esophageal temperature monitoring, which are used in general anesthesia, were performed. Following preoxygenation, induction was achieved with midazolam, remifentanil, propofol, and rocuronium bromide, and subsequent intubation was carefully performed using a videolaryngoscope. Total intravenous anesthesia (TIVA) with propofol and remifentanil was used for the maintenance of anesthesia. Invasive arterial monitoring was performed for close hemodynamic follow-up. No changes in body temperature were detected during the operation, which lasted approximately 3 hours and 10 minutes, and the patient, whose vital signs remained stable, was carefully extubated and transferred to the post-anesthesia care unit (PACU).
Referanslar
Reece MKJ, Quillin K, Homewood TJ, Bunevich J. Surgical Treatment of a Bilateral Mandibular Fracture in a Patient with Osteogenesis Imperfecta Type III. Plast Reconstr Surg Glob Open. 2021 Jul 19;9(7):e3702. doi: 10.1097/GOX.0000000000003702.
To M, Kamizato K, Shinzato H, Kakinohana M. Anesthetic management of ventricular-peritoneal shunt implantation in osteogenesis imperfecta type IIB. A case report Medicine (Baltimore). 2022 Jan 7; 101(1): e28483. doi: 10.1097/MD.0000000000028483
Marom R, Rabenhorst BM, Morello R. Management of endocrine disease: Osteogenesis imperfecta: An update on clinical features and therapies. Eur. J. Endocrinol. 2020;183:R95–R106. doi: 10.1530/EJE-20-0299.
Wang H, Huang X, Wu A, Li Q. Management of anesthesia in a patient with osteogenesis imperfecta and multiple fractures: a case report and review of the literatüre. J Int Med Res. 2021 Haz;49(6):3000605211028420. doi: 10.1177/03000605211028420.
Sillence DO, Senn A, Danks DM. Genetic heterogeneity in osteogenesi imperfecta. J Med Genet 1979;16:101-16.
Van Dijk FS, Cobben JM, Kariminejad A, Maugeri A, Nikkels PGJ, Rijn RRV, et al. Osteogenesis Imperfecta: A Review with Clinical Examples. Mol Syndromol. 2011 Dec;2(1):1-20. doi: 10.1159/000332228
Gornik-Wlaszczuk E, Majewski J, Szczygiel R, Dusiel A. Anaesthesia in children with osteogenesis imperfecta— report of 14 general anaesthetics in three children. Anaesthesiol Intensive Ther. 2013; 45 :85–8. doi: 10.5603/AIT.2013.0019.
Van Dijk FS, Sillence DO. Osteogenesis imperfecta: Clinical diagnosis, nomenclature and severity assessment. Am J Med Genet A. 2014 Jun; 164(6): 1470–1481. doi: 10.1002/ajmg.a.36545.
Sakkers JR, Montpetit K, Tsimicalis A, Wirth T, Verhoef M, Hamdy R. A roadmap to surgery in osteogenesis imperfecta: results of an international collaboration of patient organizations and interdisciplinary care teams. Acta Orthop. 2021 Oct;92(5):608-614. doi: 10.1080/17453674.2021.1941628.
Ross KE, Gibian JT, Christy J Crockett CJ, Martus JE. Perioperative Considerations in Osteogenesis Imperfecta: A 20-Year Experience with the Use of Blood Pressure Cuffs, Arterial Lines, and Tourniquets. Children (Basel). 2020 Nov 6;7(11):214. doi: 10.3390/children7110214.
Singh J, Sharma P, Mitra S. A novel approach to the anaesthetic management of a case of osteogenesis imperfecta. Indian J Anaesth. 2017 Jun; 61(6): 517–519. doi: 10.4103/ija.IJA_77_17
Rosenberg H, Davis M, James D, Pollock N, Stowell K. Malignant hyperthermia. Orphanet J Rare Dis. 2007;2:21. doi: 10.1186/1750-1172-2-21
Baranovic S, Lubina IZ, Vlahovic T, Bakota B, Maldini B. Unilateral spinal anaesthesia in a patient with Osteogenesis Imperfecta with a lower leg fracture: a case report. Injury. 2013 Sep;44 Suppl 3:S49-51. doi: 10.1016/S0020-1383(13)70198-0.
Santos ML, Anez C, Fuentes A, Mendez B, Perinan R, Rull M: Airway management with ProSeal LMA in a patient with osteogenesis imperfecta. Anesth Analg. 2006, 103:794. 10.1213/01.ANE.0000227155.44286.28
Sutton CD, Carvalho B. Supraglottic airway rescue after failed fiberoptic intubation in a patient with osteogenesis imperfecta: a case report. A A Pract. 2019;13:7–9. doi: 10.1213/XAA.0000000000000968
Sullivan BT, Margalit A, Garg VS, Njoku DB, Sponseller PD: Incidence of fractures from perioperative blood pressure cuff use, tourniquet use, and patient positioning in osteogenesis imperfecta. J Pediatr Orthop. 2019, 39:68-70. 10.1097/BPO.0000000000001105
Topçu İ, Özer M, Örgüç Ş, Sakarya M. The anesthetic management in a patient with osteogenesis imperfecta. Türk Anest Rean Journal. 2008;36(2):120-3.
Rampton AJ, Kelly DA, Shanahan EC, Ingram GS: Occurrence of hyperpyrexia in a patient with osteogenesis imperfecta. Br J Anaesth 1984;56: 1443–1446
Bojanić K, Kivela JE, Gurrieri C, Deutsch E, Flick R, Sprung J, et al. Perioperative course and intraoperative temperatures in patients with osteogenesis imperfecta. Eur J Anaesthesiol. 2011;28:370–5. doi: 10.1097/EJA.0b013e3283459616
Rodrigo C. Anesthesia for maxillary and mandibular osteotomies in osteogenesis imperfecta. Anesth Prog. 1995;42(1):17-20.
Ogawa S, Okutani R, Suehiro K. Anesthetic management using total intravenous anesthesia with remifentanil in a child with osteogenesis imperfecta. J Anesth. 2009;23(1):123-5. doi: 10.1007/s00540-008-0698-z.
Porsborg P, Astrup G, Bendixen D, Lund AM, Ording H. Osteogenesis imperfecta and malignant hyperthermia. Is there a relationship? Anaesthesia. 1996 Sep;51(9):863-5. doi: 10.1111/j.1365-2044.1996.tb12619.x.
Rüffert H, Bastian B, Bendixen D, Girard T, Heiderich S, Anna Hellblom A. Consensus guidelines on perioperative management of malignant hyperthermia suspected or susceptible patients from the European Malignant Hyperthermia Group. Br J Anaesth. 2021 Jan;126(1):120-130. doi: 10.1016/j.bja.2020.09.029.
Bozdoğan N, Eker HE, Çok OY, Akın Ş, Çınar M, Özkoç G, et al. Dexmedetomıdıne use ın total ıntravenous anesthesıa ın a case wıth osteogenesıs ımperfecta. Journal of Anesthesia. 2012;20(3):186-8.
Karabıyık L, Parpucu M, Kurtipek O. Total intravenous anesthesia and the use of an intubating laryngeal mask in a patient with osteogenesis imperfecta. Acta Anaesthesiol Scand 2002;46(5): 618-9. doi: 10.1034/j.1399-6576.2002.460525.x.
Hopkins PM. Malignant hyperthermia: pharmacology of triggering. Br J Anaesth. 2011; 107 :48-56. doi: 10.1093/bja/aer132.
Cho E, Dayan SS, Marx GF. Anaesthesia in parturient with osteogenesis imperfecta. Br J Anaesth. 1992 Apr;68(4):422-3. doi: 10.1093/bja/68.4.422
Ozyurt G, Basagan-Mogol E, Bilgin H, Tokat O. Spinal anesthesia in a patient with severe thoracolumbar kyphoscoliosis. Tohoku J Exp Med. 2005 Nov;207(3):239-42. doi: 10.1620/tjem.207.239.
Sezer DS, Küçük M, Yüksel H, Odabaşı AR, Şen S, Oğurlu M. A Pregnant Woman with Osteogenesis Imperfecta: Case Report. J Clin Obstet Gynecol.2011;21(1):62-65.
Onal,O, Zora ME, Aslanlar E, Ozdemirkan A, Celik JB. Spinal Anesthesia in an Infant with Osteogenesis Imperfecta, Anesth Pain Med. 2018 Feb; 8(1): e65917.doi: 10.5812/aapm.65917
Yeo ST, Paech MJ: Regional anaesthesia for multiple caesarean sections in a parturient with osteogenesis imperfecta. Int J Obstet Anesth. 1999, 8:284-287. 10.1016/S0959-289X(99)80111-3
Murray S, Shamsuddin W, Russell R. Sequential combined spinal-epidural for caesarean delivery in osteogenesis imperfecta. Int J Obstet Anesth. 2010 Jan;19(1):127-8. doi: 10.1016/j.ijoa.2009.07.009.
Kahraman C, Kahraman A. Infraclavicular Block and Anesthesia Management in Two Cases with Osteogenesis Imperfecta. Van Medical Journal. 2017, 24(2):116-118.
Storoni S, Treurniet S, Micha D, Celli M, Bugiani M,van den Aardweg JG et al. Pathophysiology of respiratory failure in patients with osteogenesis imperfecta: a systematic review, Ann Med. 2021; 53(1): 1676–1687. doi: 10.1080/07853890.2021.1980819.
Yan M, Knowland NP, Donna Lien D. The Anesthetic Management of a Parturient With Osteogenesis Imperfecta Type I Undergoing Cesarean Delivery. Cureus. 2021 Mar; 13(3): e13849. doi: 10.7759/cureus.13849.
