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AI-Generated response:

What are the anesthetic implications of Osteogenesis Imperfecta?

Answer generated on June 4, 2024

The AI-generated responses are for illustration purposes only and should not be used for medical purposes.

Anesthetic management in patients with Osteogenesis Imperfecta (OI) must carefully consider risks of bone fractures, difficult airway management, hypo- and hyperthermia, and significant blood loss.[1-2]

  • Patients with OI, especially severe types like Type IIB, are at high risk for bone fractures during anesthetic procedures, necessitating gentle handling and careful positioning.[2]

  • Difficult airway management is a concern due to potential skeletal abnormalities; however, careful planning and the use of appropriate techniques can mitigate this risk.[1-2]

  • Intraoperative temperature management is crucial as both hypo- and hyperthermia can occur; maintaining a stable temperature is aided by the careful selection of anesthetic agents like sevoflurane.[1]

  • Significant blood loss (>20% of estimated total blood volume) can occur, particularly in complex orthopedic procedures, requiring vigilant monitoring and management.[1]

  • Regional anesthesia techniques are often used and can be safely applied with the aid of ultrasound guidance to minimize complications and manage postoperative pain effectively.[1]

Additional info

In the context of Osteogenesis Imperfecta, anesthetic management requires a multidisciplinary approach to address the unique challenges posed by the condition. The use of gentle techniques during airway management and patient positioning is crucial to avoid fractures. Monitoring and managing intraoperative temperature and blood loss are also vital due to the increased risks associated with these factors in OI patients. The application of regional anesthesia, guided by ultrasound, can help in reducing the need for systemic analgesics and in providing effective pain control postoperatively. This comprehensive approach helps in minimizing complications and improving outcomes for patients with Osteogenesis Imperfecta undergoing anesthesia for surgical procedures.

References

Reference 1

1.

Liang X, Chen P, Chen C, et al. Paediatric Anaesthesia. 2022;32(7):851-861. doi:10.1111/pan.14454.

Publish date: July 5, 2022.

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.

Reference 2

2.

To M, Kamizato K, Shinzato H, Kakinohana M. Medicine. 2022;101(1):e28483. doi:10.1097/MD.0000000000028483. Copyright License: CC BY

Publish date: January 5, 2022.

INTRODUCTION: Osteogenesis imperfecta (OI), a rare congenital disorder, has a risk of bone fracture and progressive bone deformity. OI type II is the most serious subtype, and very few reports on its anesthetic management exist. Patients face several anesthetic difficulties, of which easy fracturing of OI-affected bones is critical. Herein, we report our experience with the anesthetic management of a patient with OI type II. PATIENT CONCERNS AND DIAGNOSES: Through genetic testing, a 14-month-old girl (height: 45 cm, weight: 3.9 kg) was diagnosed with OI type IIB due to COL1A gene abnormality. The clinical manifestations included hydrocephalus, blue sclera, dental dysplasia, short stature, limb deformity and shortening, thoracic hypoplasia, rabbit eye, left inguinal hernia, and tricuspid valve regurgitation. Physical examination revealed an enlarged head due to skull dysplasia and hydrocephalus. The pediatrician confirmed that mask ventilation was possible even under spontaneous breathing, and that there was no history of bone fracture during mask holding. INTERVENTIONS AND OUTCOMES: The patient was scheduled for gastrostomy and ventriculo-peritoneal shunt implantation. An arterial pressure line was inserted at the neonatal intensive care unit. Propofol and remifentanil were selected for general anesthesia. Confirming that mask-assisted ventilation was possible under sleep, rocuronium was administered. Attentive mask ventilation was performed via the 2-person method to avoid fractures. We were able to intubate successfully using a Macintosh laryngoscope.

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