Comprehensive risk assessments and anesthetic management for children with osteogenesis imperfecta: A retrospective review of 252 orthopedic procedures over 5 years

Abstract

Background: Major anesthetic risks arise in orthopedic surgeries for children with osteogenesis imperfecta, a rare genetically inherited condition presenting diverse skeletal issues. Aim:We aimed to investigate anesthetic risks, including difficult airway, hypo-and hyperthermia, blood loss, and pain, in connection with patient, anesthetic, and surgical factors.Methods: Both descriptive and inferential statistics were employed to study the anesthetic risks and their predictors. Data of 252 surgeries for 132 Chinese osteogenesis imperfecta patients aged 18 or below were retrieved from the authors' hospital between 2015 and 2019.Results: Two thirds of the cohort were Sillence type IV patients, with types I, III, and V accounting for 7.6%, 14.4%, and 11.4%, respectively. Video and direct laryngoscopy were used. No case of difficult airway was identified. Due to a careful management strategy, intraoperative temperature varied on average between −0.38°C and +0.89°C from the initial temperature. Fifty-two and 18 cases of hyper-and hypothermia were encountered, respectively. The use of sevoflurane for maintenance resulted in a mean increase of +0.24°C [95% CI 0.05 ~ 0.42] in the maximum temperature. Massive blood losses (>20% of estimated total blood volume) were observed in 18.3% of the cases.Neither intraoperative temperature changes nor blood loss was found to be related to Sillence classification. Regional anesthesia techniques were applied to 72.6% of the cases. Ultrasound guidance was used per the judgment of anesthesiologists or when in case of difficult landmarks. The incidence of difficult regional anesthesia was low (4 out of 252). For postoperative analgesia, 154 neuraxial blocks (including 77 caudal and 77 lumbar epidural) and 29 peripheral nerve blocks were performed. Conclusion:Anesthesia for children with osteogenesis imperfecta undergoing complex orthopedic procedures was challenging. Proper anesthesia planning was essential for both intraoperative management and postoperative analgesia. Age, surgical duration, and use of sevoflurane for maintenance impacted the intraoperative temperature most,

Endoprothetische Versorgung von Patienten mit Skelettdysplasien

“…Teilweise gibt es von den Fachgesellschaften oder auch den Patientenorganisationen dazu erkrankungsspezifische Leitfäden und Handlungsempfehlungen mit Erfahrungswerten, die bei der Vorbereitung hilfreich sein können . Im Einzelfall kann und sollte durch die konsiliarische Beteiligung weiterer Fachdisziplinen im Vorfeld die Sicherheit für alle Beteiligten verbessert werden [2,4,6].…”

Gelenkerhaltende Therapie bei Osteogenesis imperfecta

“…Ob hierbei die restriktive Beeinträchtigung oder die Einschränkung der pulmonalen Kapazität durch die Grunderkrankung den größeren Einfluss hat, wird derzeit intensiv erforscht 25 26 . Insgesamt werden für die Schwere der Grunderkrankung wenige anästhesiologische Probleme beschrieben 27 . Auch ein immer wieder diskutiertes vermehrtes Auftreten einer malignen Hyperthermie konnte nicht bestätigt werden 28 .…”

Scoliosis in osteogenesis imperfecta: identifying the genetic and non-genetic factors affecting severity and progression from longitudinal data of 290 patients

“…OI patients present with low bone density, high fracture rates, long bone deformity, scoliosis and a wide array of other symptoms [ 3 , 4 ]. Current treatments, including bone density enhancement and orthopedic corrections [ 5 ], do not fundamentally cure the condition, causing considerable burdens on affected individuals and society.…”