Osteogenesis imperfecta (OI) is an inherited disorder of the connective tissue the primary manifestation of which is an increased
susceptibility to fractures. Patients often require orthopaedic treatment of fractures and resultant deformities. Commonly encountered
intraoperative complications include diffi cult airway with easily fractured teeth, patient positioning problems, intraoperative
bleeding, hypothermia, respiratory compromise due to skeletal deformity, cardiac problems or basilar invagination caused by
congenital anomalies, and very rarely development of perioperative hyperthermia. Therefore, multidisciplinary approach to preoperative
assessment of patients with OI, including identifi cation of risk factors and optimization of health before surgery is needed.Osteogenesis imperfecta (OI) je nasljedna bolest vezivnog tkiva, a njezina glavna manifestacija je povećana sklonost prijelomima.
Ovi bolesnici često zahtijevaju ortopedsko liječenje prijeloma i posljedičnih deformiteta. Česte intraoperacijske komplikacije su
otežani dišni put s lako lomljivim zubima, problemi pozicioniranja bolesnika, intraoperacijsko krvarenje, hipotermija, respiracijski
poremećaji zbog koštanih deformacija, srčani problemi ili bazilarna invaginacija uzrokovana prirođenim anomalijama te vrlo
rijetko razvoj perioperacijske hipertermije. Stoga je neophodan multidisciplinski pristup prijeoperacijskoj procjeni bolesnika s OI,
uključujući utvrđivanje rizičnih čimbenika i postizanje optimalnog zdravstvenog stanja prije operacije

Ana Blagaić

Goran Pavelić

Slobodan Mićić

Tomislav Čutura

English

Osteogenesis imperfecta (OI) is an inherited disorder of the connective tissue the primary manifestation of which is an increased
 susceptibility to fractures. Patients often require orthopaedic treatment of fractures and resultant deformities. Commonly encountered
 intraoperative complications include diffi cult airway with easily fractured teeth, patient positioning problems, intraoperative
 bleeding, hypothermia, respiratory compromise due to skeletal deformity, cardiac problems or basilar invagination caused by
 congenital anomalies, and very rarely development of perioperative hyperthermia. Therefore, multidisciplinary approach to preoperative
 assessment of patients with OI, including identifi cation of risk factors and optimization of health before surgery is needed.Osteogenesis imperfecta (OI) je nasljedna bolest vezivnog tkiva, a njezina glavna manifestacija je povećana sklonost prijelomima.
 Ovi bolesnici često zahtijevaju ortopedsko liječenje prijeloma i posljedičnih deformiteta. Česte intraoperacijske komplikacije su
 otežani dišni put s lako lomljivim zubima, problemi pozicioniranja bolesnika, intraoperacijsko krvarenje, hipotermija, respiracijski
 poremećaji zbog koštanih deformacija, srčani problemi ili bazilarna invaginacija uzrokovana prirođenim anomalijama te vrlo
 rijetko razvoj perioperacijske hipertermije. Stoga je neophodan multidisciplinski pristup prijeoperacijskoj procjeni bolesnika s OI,
 uključujući utvrđivanje rizičnih čimbenika i postizanje optimalnog zdravstvenog stanja prije operacije

Mićić, Slobodan

Pavelić, Goran

Čutura, Tomislav

Blagaić, Ana

Repository of the University of Rijeka, Faculty of Medicine

Preoperative assessment of patients with osteogenesis imperfecta

Hrčak - Portal of scientific journals of Croatia

134 Paediatr Croat. 2017;61:134-6PREGLED / REVIEWwww.paedcro.comhttp://dx.doi.org/10.13112/PC.2017.19Preoperative assessment of patients with osteogenesis imperfectaSlobodan Mićić1, Goran Pavelić1, Tomislav Čutura2, Ana Blagaić1Osteogenesis imperfecta (OI) is an inherited disorder of the connective tissue the primary manifestation of which is an increased susceptibility to fractures. Patients often require orthopaedic treatment of fractures and resultant deformities. Commonly encoun-tered intraoperative complications include diffi  cult airway with easily fractured teeth, patient positioning problems, intraoperative bleeding, hypothermia, respiratory compromise due to skeletal deformity, cardiac problems or basilar invagination caused by  congenital anomalies, and very rarely development of perioperative hyperthermia. Therefore, multidisciplinary approach to preop-erative assessment of patients with OI, including identifi cation of risk factors and optimization of health before surgery is needed.Key words: intraoperative complications; osteogenesis imperfecta; preoperative assessment 1  Clinical Department of Anaesthesia and Intensive Medicine, Rijeka University Hospital Centre, Rijeka, Croatia2 Sv. Katarina Special Hospital, Zabok, CroatiaCorrespondence to:Slobodan Mićić, MD, Clinical Department of Anesthesia and Intensive Medicine, Rijeka University Hospital Centre, Istarska 43, HR-51000 Rijeka, CroatiaPrimljeno/Received: 11. 9. 2017., Prihvaćeno/Accepted: 12. 10. 2017.INTRODUCTIONOsteogenesis Imperfecta (OI) is an inherited disorder of the connective tissue the primary manifestation of which is an increased susceptibility to fractures. Commonly encoun-tered intraoperative complications include diffi  cult airway with easily fractured teeth, patient positioning problems, intraoperative bleeding (coagulation or platelet dysfunc-tion and technical aspect of the surgery), increased tenden-cy to hypothermia due to the duration of surgery, respira-tory compromise due to skeletal deformity, and the last but not the least, cardiac problems caused by congenital anom-alies, basilar invagination, and very rarely development of perioperative hyperthermia.Proper preoperative assessment, correct identifi cation of risk factors, and optimization of health before surgery is im-portant, as is the choice of anaesthetic technique.PROCEDURE RISK LEVELSurgical placement of simple intramedullary rods to pre-vent or correct long-bone deformities are usually low to moderate risk procedures, but placement of telescoping rods, which may be appropriate for patients older than two years who are actively growing, is usually long-lasting and characterized by a signifi cant loss of blood. These are high-risk procedures. PATIENT RELATED RISK FACTORS In OI patients, most of the interventions are usually per-formed at the age of two to ten years and they are usually classifi ed as ASA score 1-2. The main and most common clinical manifestations of OI important for anaesthesia are short stature, scoliosis, megalocephaly, short neck, limited range of motion of cervical spine, basilar skull deformities that may cause nerve compression or other neurologic symptoms, hearing loss, opalescent fragile teeth, easy bruis-ing, bone fragility, and joint laxity.Good physical examination includes examination of the oral cavity and the teeth, Mallampati score, hearing check, examination of tongue size, neck and facial deformity, neck mobility, thyromental distance, central venous access, aus-cultation of the lungs and the heart, and fi nally, access to peripheral blood vessels. It is mandatory to check the lum-bar region due epidural catheter setting. However, spinal deformity associated with OI may make neuraxial anaesthe-135PAEDIATR CROAT. 2017;61:134-6 MIĆIĆ S., PAVELIĆ G., ČUTURA T., BLAGAIĆ A. PREOPERATIVE ASSESSMENT OF PATIENTS WITH OSTEOGENESIS IMPERFECTA.sia (i.e. epidural, spinal, or combined spinal epidural) techni-cally challenging and may make the spread of neuraxial lo-cal anaesthetic unpredictable.Patients with OI are at risk of facial bone fractures, cervical spine injury, and dental damage during endotracheal intu-bation, placement of supraglottic airways, or even mask ventilation. They are at risk of positioning-related fracture and joint dislocation. Fracture or bruising may also occur with excessive infl ation of the blood pressure cuff .The cardiopulmonary abnormalities that are associated with OI (i.e. kyphoscoliosis, restrictive lung disease, valvular heart disease, aortic root dilatation) may require invasive monitoring and/or modifi cation of the choice of anaesthet-ic induction agents. Patients with type III deforming OI or other moderate to severe types of OI should have yearly spi-rometry (to evaluate for restrictive lung disease due to ky-phoscoliosis and chest deformities) and an electrocardio-gram (ECG) and echocardiogram (cardiac echo) every two years to detect aortic root dilation and valvular dysfunction. Chest and spine radiography performed every one to two years are usually helpful, but it is recommended to perform cardiac echo for up to six months, along with follow-up spi-rometry. ECG and cardiac echo of the heart and spirometry also include the opinion of competent paediatricians.Basilar skull deformity may lead to basilar invagination. Thus, neurologic examination and magnetic resonance imaging/multi-slice computed tomography should be performed as indicated by symptoms or behavioural changes, particularly in patients with type III OI.Bleeding diathesis related to capillary fragility and platelet function abnormalities is associated with OI and can result in signifi cant haemorrhage during surgery or slow oozing after the procedure. Long-lasting treatments are character-ized by bleeding from the bone canal and suppressing the extremities, which also increases blood loss. Bleeding time and standard coagulation tests including prothrombin time, INR, activated partial thromboplastin time, fi brinogen level, d-dimer, and platelet number with platelet aggrega-tion should be obtained prior to any surgical procedure. If there is a history suggestive of abnormal bleeding, haema-tologist and/or transfusiologist consultation is advised. Nonsteroidal anti-infl ammatory drugs should be avoided because of their antiplatelet eff ects.Hyperthermia may occur in patients with OI for unclear rea-sons and the mechanism has been linked to either malig-nant hyperthermia or increased thyroid hormones, but most of these patients are not at an increased risk of malig-nant hyperthermia.Typical laboratory fi ndings in patients with OI are elevated levels of serum alkaline phosphatase, hypercalciuria, and hypercalcemia after bisphosphonate therapy. A common but not typical fi nding is iron defi ciency anaemia due to so-cial reasons and eating habits. Reduced levels of protein and fi brinogen are not uncommon. A routine preoperative laboratory examination should include complete blood count, electrolyte levels (especially calcium), liver and kid-ney function tests, and complete coagulogram. In case of hyperthyroidism, the concentrations of triiodothyronine, thyroxine and thyroid-stimulating hormone should be ana-lysed, but these tests should not be older than six months. Endocrinologist opinion should also be obtained.To reduce the risk of osteomyelitis occurrence, it is neces-sary to obtain swab of the nose, throat and skin. CONCLUSIONMany anaesthetic problems may be encountered in ortho-paedic treatment of deformity in patients with OI, caused by positioning the patient on the operating table, malig-nant hyperthermia, basilar invagination, or cardiac prob-lems due to congenital anomalies. However, the most com-mon are diffi  cult airway, fragile teeth, central venous or epi-dural catheter placing problems, intraoperative bleeding with fl uid shift and consequent coagulation disorder, hypo-thermia, and fi nally possible postoperative respiratory func-tion problems. Proper preparation, optimization of health before surgery, and preoperative assessment are important. This implies a multidisciplinary approach to patients with OI that includes diff erent physicians such as orthopaedist, pae-diatrician, transfusiologist, radiologist, operating room staff , psychologist, etc., which should lead to an uneventful an-aesthetic course.NOVČANA POTPORA/FUNDINGNema/NoneETIČKO ODOBRENJE/ETHICAL APPROVALNije potrebno/NoneSUKOB INTERESA/CONFLICT OF INTERESTAutori su popunili the Unifi ed Competing Interest form na www.icmje.org/coi_disclosure.pdf (dostupno na zahtjev) obrazac i izjavljuju: nemaju potporu niti jedne organizacije za objavljeni rad; nemaju fi nancijsku potporu niti jedne organizacije koja bi mogla imati interes za objavu ovog rada u po-sljednje 3 godine; nemaju drugih veza ili aktivnosti koje bi mogle utjecati na objavljeni rad./All authors have completed the Unifi ed Competing Interest form at www.icmje.org/coi_disclosure.pdf (available on request from the cor-responding author) and declare: no support from any organization for the sub-mitted work; no fi nancial relationships with any organizations that might have an interest in the submitted work in the previous 3 years; no other relationships or activities that could appear to have infl uenced the submitted work. REFERENCES 1. Stynowick GA, Tobias JD. Perioperative care of the patient with osteogenesis imperfecta. Orthopedics. 2007;30:1043-9.136MIĆIĆ S., PAVELIĆ G., ČUTURA T., BLAGAIĆ A. PREOPERATIVE ASSESSMENT OF PATIENTS WITH OSTEOGENESIS IMPERFECTA. PAEDIATR CROAT. 2017;61:134-6 2. Oakley I, Reece LP. Anesthetic implications for the patient with osteogenesis imperfecta. AANA J. 2010;78:47-53. 3. Vetter U, Maierhofer B, Müller M, et al. Osteogenesis imperfecta in childhood: cardiac and renal manifestations. Eur J Pediatr. 1989;149:184-7. 4. Hortop J, Tsipouras P, Hanley JA, et al. Cardiovascular involvement in osteogenesis imperfecta. Circulation. 1986;73:54-61. 5. Rauch F, Glorieux FH. Osteogenesis imperfecta. Lancet. 2004;363:1377-85. 6. Osteogenesis imperfecta. In: Wilson GN, Cooley WC, editors. Preventive Management of Children with Congenital Anomalies and Syndromes. Cambridge University Press, Cambridge, UK, 2000; p. 256. 7. Cremin B, Goodman H, Spranger J, Beighton P. Wormian bones in osteogenesis imperfecta and other disorders. Skeletal Radiol. 1982;8:35-8. 8. Greeley CS, Donaruma-Kwoh M, Vettimattam M, et al. Fractures at diagnosis in infants and children with osteogenesis imperfecta. J Pediatr Orthop. 2013;33:32-6. 9. Huang J, Dinh M, Kuchle N, Zhou J. Anesthetic management for combined mitral valve replacement and aortic valve repair in a patient with osteogenesis imperfecta. Ann Card Anaesth. 2011;14:115-8.10. Kristensen SD, Knuuti J, Saraste A, et al. 2014 ESC/ESA Guidelines on non-cardiac surgery: cardiovascular assessment and management: The Joint Task Force on non-cardiac surgery: cardiovascular assessment and management of the European Society of Cardiology (ESC) and the European Society of Anaesthesiology (ESA). Eur Heart J. 2014;35:2383-431.S A Ž E T A K Prijeoperacijska procjena bolesnika s osteogenesis imperfectaSlobodan Mićić, Goran Pavelić, Tomislav Čutura, Ana BlagaićOsteogenesis imperfecta (OI) je nasljedna bolest vezivnog tkiva, a njezina glavna manifestacija je povećana sklonost prijelomima. Ovi bolesnici često zahtijevaju ortopedsko liječenje prijeloma i posljedičnih deformiteta. Česte intraoperacijske komplikacije su otežani dišni put s lako lomljivim zubima, problemi pozicioniranja bolesnika, intraoperacijsko krvarenje, hipotermija, respiracijski poremećaji zbog koštanih deformacija, srčani problemi ili bazilarna invaginacija uzrokovana prirođenim anomalijama te vrlo  rijetko razvoj perioperacijske hipertermije. Stoga je neophodan multidisciplinski pristup prijeoperacijskoj procjeni bolesnika s OI, uključujući utvrđivanje rizičnih čimbenika i postizanje optimalnog zdravstvenog stanja prije operacije. Ključne riječi: intraoperacijske komplikacije; osteogenesis imperfecta; prijeoperacijska procjena

Prijeoperacijska procjena bolesnika s osteogenesis imperfecta

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Prijeoperacijska procjena bolesnika s osteogenesis imperfecta

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