Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
Article copyright, the authors; Journal compilation copyright, J Med Cases and Elmer Press Inc
Journal website https://jmc.elmerpub.com

Case Report

Volume 17, Number 3, March 2026, pages 107-114

Perioperative Care of an Eleven-Year-Old Child With Osteogenesis Imperfecta Type II During Posterior Spinal Fusion

Figures

Figure 1.
Figure 1. Preoperative seated radiographs demonstrating severe scoliosis with 71° right thoracic curve (T1-T9), 85° left lumbar curve (T9-L3, major), and thoracolumbar kyphosis.
Figure 2.
Figure 2. Preoperative computed tomography reconstruction of the vertebral column showing severe scoliosis.
Figure 3.
Figure 3. Postoperative seated radiographs following scoliosis correction with T2 to pelvis posterior spinal instrumented fusion, posterior column osteotomies, and cement vertebral screw augmentation.

Tables

Table 1. Previous Reports of Anesthetic Care in Patients With Osteogenesis Imperfecta Undergoing Posterior Spinal Fusion
 
Author and referencePatient demographicAnesthetic techniqueComments and outcome
BP: blood pressure; OI: osteogenesis imperfecta; PSF: posterior spinal fusion.
Patino and Soliman [26]A 4-year-old, 11 kg girl with OI and severe scoliosis (Cobb angle 90°) for PSF (T2–L3). Prior history of multiple atraumatic fractures and restrictive lung disease.Induction and maintenance: Intravenous induction with propofol, oxygen/nitrous oxide, isoflurane, and remifentanil (0.1–0.3 µg/kg/min). Airway approach: Gentle mask ventilation and direct laryngoscopy with neutral head positioning.Attention to prone positioning with pressure points padded. Invasive BP monitoring and avoidance of noninvasive blood pressure monitoring due to fracture risk. Cell saver to limit need for allogeneic blood. Patient’s trachea was extubated uneventfully, transferred to the PICU with no new fractures or complications. Uncomplicated postoperative course.
Chin and Stuart [27]An 11-year-old, 21.6 kg boy with autosomal recessive OI type VIII (LEPRE1 mutation) for posterior spinal fusion (T3–L5) for progressive scoliosis (Cobb angle 54.5°). Prior history of extreme bone fragility, barrel chest, visual/hearing impairment, and restricted growth.Induction and maintenance: Total intravenous anesthesia (TIVA) using target-controlled propofol and remifentanil. Airway approach: Endotracheal intubation by direct laryngoscopy with minimal neck movement and head in neutral position.Invasive BP monitoring. Blood conservation strategies included intraoperative cell salvage and tranexamic acid. Temperature management included forced air warming and in-line fluid warmer. The patient’s trachea was extubated on postoperative day 1 but later sustained humeral and rib fractures (conservative management).

 

Table 2. Perioperative Concerns in Patients With Osteogenesis Imperfecta
 
IV: intravenous; NOBP: non-invasive blood pressure; OI: osteogenesis imperfecta.
1. Airway concerns
  • Atlanto-occipital subluxation (potential for spinal cord injury during airway manipulation)
  • Abnormal dentition – risk of dental trauma
  • Difficult bag-valve-mask ventilation and endotracheal intubation (short neck, dental problems, micrognathia, midface and mandibular deformities, limited neck extension)
  • Tracheostomy
2. Progressive kyphoscoliosis and thoracic wall deformities
  • Respiratory insufficiency (restrictive lung disease, decreased pulmonary compliance)
  • Pulmonary hypoplasia can cause chronic hypoxemia and postoperative respiratory failure
  • Postoperative ventilatory support
3. Cardiac involvement
  • Valvular insufficiency (aortic and mitral regurgitation)
  • Heart failure due to collagen defects affecting myocardium
  • Progressive aortic dilatation or rarely dissection
  • Associated congenital heart disease
4. Bleeding tending
  • Capillary fragility
  • Abnormal platelet aggregation with prolonged bleeding time
  • Pharmacologic manipulation of coagulation cascade and platelet function (antifibrinolytic therapy and DDAVP)
  • Availability of blood and blood products
5. Bone fragility
  • Fractures may occur during transfer, positioning, or NIBP cuff use
  • Attention to positioning and padding
  • Avoid excessive manipulation of limbs or neck
  • Limb deformities and previous fractures may make IV and arterial access difficult
  • NIBP cuffs, if used, should be placed over well-padded areas and cycled minimally
6. Difficult venous and arterial access
  • Tourniquet to be loosely placed for IV access
  • Consider invasive arterial monitoring
  • Ultrasound-guided techniques may be preferred
7. Positioning concerns
  • Use soft padding under pressure points
  • Neutral head positioning and minimal neck movement
  • Head rings or foam supports to prevent cervical injury
  • Transfers and repositioning should involve multiple staff members
8. Potential for intraoperative hyperthermia
  • Not malignant hyperthermia related
  • Continuous temperature monitoring
  • Maintain normothermia using warming/cooling devices as needed
9. Central nervous system involvement
  • Chiari malformation commonly occurs in OI type II (potential brainstem compression and cervical instability) with potential for cranial nerve involvement
  • Cranio-vertebral junction involvement with atlanto-axial subluxation and potential for cervical spinal cord injury
  • Hydrocephalus