Assessing the Safety and Efficacy of Tranexamic Acid Usage in Osteogenesis Imperfecta Patients.

BACKGROUND: Osteogenesis Imperfecta (OI) usually causes an increased fracture burden and bone deformity, with subsequent operations common. In addition to skeletal manifestations, there is a potential increase in bleeding susceptibility due to the increased frequency of orthopedic procedures, warranting investigation into methods to mitigate this risk. This study aims to evaluate the safety and efficacy of tranexamic acid (TXA) usage to reduce intraoperative blood loss in children with OI. We want to assess the potential benefits, risks, and complications involved with TXA use in this patient population. METHODS: TXA-receiving patients (cases) were matched 1:1 with non-TXA-receiving controls on the following criteria: age within 2 years, bone category, and OI Type. Descriptive statistics were used to summarize the data. Fisher Exact Test was performed to compare transfusion status between groups. A Wilcoxon Rank Sum test was performed to assess differences between the groups in days of stay, length of surgery, and estimated blood loss (EBL). All analyses were conducted using SAS version 9.4. P <0.05 was considered statistically significant. RESULTS: Our TXA-receiving population of 30 patients consisted of 11 females and 19 males. One patient was OI type I, 13 were OI type III, 14 were OI type IV, and 2 were categorized as Other (not Type I through Type IV). We found a significant difference in transfusion status ( P =0.02), with zero TXA patients requiring a transfusion compared with 20% of the control cases. There is also a significant difference in median EBL ( P =0.0004) between groups, with TXA patients having decreased intraoperative EBL (20 vs. 62.5 mL). There was also a difference in median days of postoperative stay between TXA-receiving and non-TXA-receiving patients ( P =0.001; 2.6 vs. 4 d). CONCLUSIONS: Our study concluded that TXA use in OI patients is associated with lower perioperative transfusions and intraoperative blood loss rates. These results support the standard usage of TXA in these patients to reduce intraoperative blood loss. LEVEL OF EVIDENCE: Level III.

[Features and results of surgical rehabilitation of hearing loss in osteogenesis imperfecta]. / Osobennosti i rezul'taty khirurgicheskogo lecheniya tugoukhosti pri nesovershennom osteogeneze.

Osteogenesis imperfecta (OI) is a form of congenital osteoporosis. Depending on the type of OI, patients experience various types of hearing loss. Depending on the type and degree of hearing loss, various methods of hearing rehabilitation are used in this category of patients. OBJECTIVE: To evaluate the features and results of surgical rehabilitation of hearing loss in patients with osteogenesis imperfecta. MATERIAL AND METHODS: During the period from 2009 to 2022, 2221 primary stapedoplasty was performed in the department, of which 23 (1.04%) in 21 patients were performed in patients with OI. There were 14 women and 7 men. According to TPA, bilateral hearing loss was detected in 19 patients and unilateral in 2. Conductive hearing loss was observed in 9 cases and mixed - in 14. The average thresholds for bone conduction (BC) were 22.7±8.04 dB, and the bone-air interval (ABG) - 36.1±5.3 dB. According to CT of the temporal bones, all patients showed a bilateral and symmetrical decrease in the density of the auditory ossicles, and in 7 patients there were extensive areas of non-uniform decrease in the density of the bone labyrinth up to +500 - +1000 HU.21 patients underwent 23 operations: in 21 cases stapedoplasty with laser assistance and in 2 cases ossiculoplasty. RESULTS: BC thresholds 6 months after surgery averaged 24.6±8.2 dB, and ABG - 12.1±2.9 dB. Closing of ABG ≤10 dB at spoken frequencies was detected in 30.5%, ABG ≤20 dB - in 95%. After 12 months or more after the operation, no change in the audiological parameters was noted. CONCLUSIONS: Stapes surgery for conductive and mixed hearing loss in OI patients is functionally effective. The best results are achieved after therapy with bisphosphonates with preparations of sodium fluoride, calcium and vitamin D, performing the operation when the density of demineralization zones reaches 1000 HU and using laser assistance. Taking into account the demineralization of the bone structures of the temporal bone, it is recommended to use autocartilaginous stirrup prostheses to restore sound conduction or to cover the attachment area of other prostheses with autologous tissues to prevent necrosis of the long stalk of the incus and stabilize long-term functional results.

Growth Arrest in Type IV Osteogenesis Imperfecta After Fassier-Duval Rod Insertion Treated by a Lengthening Magnetic Intramedullary Nail: A Case Report.

CASE: A child with Type IV Osteogenesis Imperfecta (OI) sustained a growth arrest of the distal femur after fixation of a left femur fracture with a Fassier-Duval expanding rod at 3 years old. Despite bar resection with fat interposition, the discrepancy progressed to 7.5 cm at maturity. Because the femur had grown to a sufficient diameter, he underwent successful lengthening with a magnetic intramedullary rod. CONCLUSION: Although it is a potential complication, growth arrest has not been reported in association with placement of an expanding nail in a child with osteogenesis imperfecta. This case illustrates this rare complication and treatment using a magnetic intramedullary rod.

The patient-related factors in revision procedures on tibia of patients with osteogenesis imperfecta treated with the Peter-Williams nail.

OBJECTIVE: To investigate the patient-related factors that affect the revision rate for the tibia in patients with osteogenesis imperfecta treated with the Peter-Williams nail, and to explore the relationship between the risk factors and complications postsurgery. METHODS: We retrospectively analysed the data of 211 patients (93 females (44.08%) and 118 males (55.92%)) with osteogenesis imperfecta treated with Peter-Williams. The factors affecting surgical revision were analysed by performing binary logistic regression. Then, a total of 211 patients with type III, type I or type IV OI were divided into five groups according to the results of regression. Statistical comparison of these groups was performed to further investigate the relationship between patient-related factors and revision procedures. Statistical comparison was also performed to analyse the relationship between the classification and postoperative complications. RESULTS: Among the 211 patients who underwent surgery, 40 had type I OI, 109 had type IV OI, and 62 had type III OI. Binary logistic regression revealed that the classification (OR = 3.32, 95% CI 1.06-10.39, P = 0.039) and initial operation age (OR = 0.83, 95% CI 0.76-0.92, P < 0.001) were significantly correlated with revision procedures. In type III patients, the initial operation age was significantly correlated with revision procedures (P < 0.001), and the revision rate was lower in patients aged 9 to12 years (P = 0.001). In type I and IV patients, the initial operation age was not significantly correlated with revision procedures (P = 0.281). Classification had a significant effect on postoperative deformity (P = 0.003). CONCLUSIONS: The study reported that the age of initial surgery and classification were the influencing factors affecting the revision procedures of tibia in patients with osteogenesis imperfecta treated with the Peter-Williams nail. In patients with type III disease, the revision rate was lower individuals aged 9-12 years old, and a higher incidence of postoperative deformity was observed.

Fixation techniques in lower extremity correction osteotomies and fractures in mild-to-severe osteogenesis imperfecta patients: evaluation of the results and complications.

INTRODUCTION: Osteogenesis imperfecta is a genetic disorder leading to multiple fractures and deformities. Intramedullary rods have been used in the surgical treatment of osteogenesis imperfecta for decades. Complication rates reported by current techniques have been high. This study aimed to examine the results of intramedullary fixation combined with plate and screw technique in patients with osteogenesis imperfecta compared to isolated intramedullary fixation. METHODS: Between 2006 and 2020, forty patients who had surgical treatment for deformities or fractures of the femur, tibia or both with at least two years of follow-up after surgery were included in the study. Patients were divided into groups according to fixation methods. Group 1 was intramedullary fixation only (Titanium Elastic Nail [TEN], Rush Pin, and Fassier-Duval Rod), and Group 2 was intramedullary fixation combined with plate and screws. Medical records and follow-up radiographs were reviewed to evaluate healing and callus formation, types of complications and infection rates. RESULTS: The total number of operated lower extremities of these forty patients was 61 (45 femur and 16 tibia). The mean age of the patients was 9.3 ± 4.6 years. Mean follow-up duration of the patients was 4.4 ± 1.7 years. Thirty-seven (61%) were in Group 1, and 24 (39%) were in Group 2. There was no statistically significant difference in callus formation time between Group 1 and Group 2 (p = 0.67). Complications occurred in 21 of 61 surgeries. While 17 of these complications were in Group 1, 4 were in Group 2 (p = 0.01). CONCLUSION: Intramedullary fixation combined with the plate and screw technique in children with osteogenesis imperfecta is successful considering the complications and revision requirements.

Bone allografting: an original method for biological osteosynthesis and bone reinforcement in children with osteogenesis imperfecta.

PURPOSE: Osteogenesis imperfecta (OI) is a genetic disorder responsible for various symptoms including deformities and frequent fractures. Bone allografting is poorly documented in this condition. The objective of this study was to describe our experience and assessments in a consecutive series of OI patients. METHODS: Thirty-nine lower limb allograft procedures (28 femurs, 11 tibias) were performed in 26OI patients (mean age, 12.9 years). They were classified as type III of Sillence (17), type IV (6), and 3 recessive forms. The indications for surgery were correction of deformity (19), fracture (16), and non-union (4). In all cases, bone allografting was added to reinforce areas of fragility and in 28 cases for osteosynthesis to lock the rotations at the osteotomy site and to avoid screwed metallic plate. The duration of bone consolidation and allograft fusion was assessed. Complications and Gillette functional score were reported. RESULTS: The mean follow-up was 6.7years (range, 2 to 10 years). On average, bone consolidation was achieved after 3.3 months and graft fusion after 7.7 months. No bone allograft-related complications were observed and there was any secondary displacement. The Gillette functional score was improved in 23 patients and stable in three cases. Complications were reported in two cases: one partial allograft resorption and one delayed consolidation of a non-union. One refracture was observed but after a significant trauma in a child who had regained significant physical activity. CONCLUSIONS: Bone allografting in children with OI is a reliable method of biological fixation, allowing efficient fusion and contributing to increased bone capital and functional outcome.

The Fate of the Bent Rod in Children With Osteogenesis Imperfecta.

BACKGROUND: Osteogenesis imperfecta (OI) is a genetic disorder characterized by brittle bones and long bone deformity. Realignment and intramedullary rodding with telescopic rods are indicated for progressive deformity and can help prevent fractures. Rod bending is a reported complication of telescopic rods and a common indication for revision; however, the fate of bent lower extremity telescopic rods in the setting of OI has not been reported. METHODS: Patients with OI at a single institution who underwent lower extremity telescopic rod placement with at least 1-year follow-up were identified. Bent rods were identified, and for these bone segments, we collected the location and angle of bend, subsequent telescoping, refracture, increasing angulation of bend, and date of revision. RESULTS: One hundred sixty-eight telescopic rods in 43 patients were identified. Forty-six rods (27.4%) bent during follow-up, with an average angulation of 7.3 (range: 1 to 24) degrees. In patients with severe OI, 15.7% of rods bent compared with 35.7% in nonsevere OI ( P =0.003). The proportion of bent rods was different between independent and nonindependent ambulators (34.1% and 20.5%; P =0.035). Twenty-seven bent rods (58.7%) were revised, with 12 rods (26.0%) revised early (within 90 d). The angulation of rods that were revised early was significantly higher than rods not (14.6 and 4.3 degrees, P <0.001). Of the 34 bent rods not revised early, the average time to revision or final follow-up was 29.1 months. Twenty-five rods (73.5%) continued to telescope, 14 (41.2%) increased in angulation (average 3.2 degrees), and 10 bones (29.4%) refractured. None of the refractures required immediate rod revision. Two bones had multiple refractures. CONCLUSIONS: Bending is a common complication of telescopic rods in the lower extremities of patients with OI. It is more common in independent ambulators and patients with nonsevere OI, possibly because of the increased demand placed on the rods. Rods with a small bend and maintained fixation can telescope and need not be an indication for immediate revision. LEVEL OF EVIDENCE: Level III-Retrospective review.

[Limbal stem cell-sparing lamellar keratoplasty for advanced keratoglobus in a patient with osteogenesis imperfecta: a case report].

This 15-year-old male patient has been diagnosed with osteogenesis imperfecta through genetic testing after birth and has poor vision. His full corneas in both eyes are unevenly thinned and bulging in a spherical shape, with the right eye being more severe. He underwent a limbal stem cell-sparing lamellar keratoplasty in the right eye, resulting in improved vision with a corrected visual acuity of 0.5, a decrease in corneal curvature, and a significant increase in corneal thickness. The surgery had a satisfactory outcome. The condition of the left eye is progressing and will require further surgical treatment.

Outcomes of Growth-friendly Instrumentation in Osteogenesis Imperfecta: A Preliminary Report.

BACKGROUND: There is limited literature on the outcomes in patients with osteogenesis imperfecta (OI) undergoing growth-friendly instrumentation (GFI). The purpose of this study was to report the outcomes of GFI in patients with early-onset scoliosis (EOS) and OI. We hypothesized that similar trunk elongation could be obtained in OI patients, but with higher complication rates. METHODS: A multicenter database was studied for patients with EOS and OI etiology who had GFI from 2005 to 2020, with a minimum 2-year follow-up. Demographic, radiographic, clinical, and patient-reported outcomes data were collected and compared with an idiopathic EOS cohort matched 2:1 for age, follow-up duration, and curve magnitude. RESULTS: Fifteen OI patients underwent GFI at a mean age of 7.3±3.0 years, with an average follow-up of 7.3±3.9 years. OI patients had a mean preoperative coronal curve of 78.1±14.5 and achieved 35% correction after index surgery. There were no differences in major coronal curves and coronal percent correction between the OI and idiopathic groups at all time points. T1-S1 length (cm) was lower for the OI group at baseline (23.3±4.6 vs. 27.7±7.0; P =0.028) but both groups had similar growth (mm) per month (1.0±0.6 vs. 1.2±1.1; P =0.491). OI patients had a significantly increased risk of proximal anchor failure, which occurred in 8 OI patients (53%) versus 6 idiopathic patients (20%) ( P =0.039). OI patients who underwent preoperative halo-traction (N=4) had greater T1-S1 length gain (11.8±3.2 vs. 7.3±2.8; P =0.022) and greater percent major coronal curve correction (45±11 vs. 23±17; P =0.042) at final follow-up versus patients with no halo-traction (N=11). Staged foundation fusion was performed in 2 cases. CONCLUSION: Compared with matched idiopathic EOS patients, OI patients undergoing GFI achieved similar radiographic outcomes but sustained greater rates of anchor failures, likely due to weakened bone. Preoperative halo-traction was a useful adjunct and may improve final correction. Staged foundation fusion is an idea to consider for difficult cases. LEVEL OF EVIDENCE: Therapeutic-III.

Which is the best femoral implant in children with osteogenesis imperfecta? a retrospective cohort study of 783 procedures.

BACKGROUND: Osteogenesis imperfecta (OI) is a hereditary genetic disorder characterized by bone fragility and extremity deformities. The surgical management for long-bone fractures and deformities in OI remains a challenge. We aimed to compare clinical outcomes after femoral surgery splinted with the telescopic rod, the plate and screws, the elastic nail and the non-elongating rod in setting of OI. METHODS:  A retrospective cohort study included 783 femoral procedures (mean age 6.00 (interquartile range (IQR) 5.00) years, 335 (42.8%) females) was conducted, and individuals were categorized into four groups according to implants. After verifying comparability among the groups, revision rate and implant survival period were compared among the Sillence types and the same comparison were made among four groups within each Sillence type. The incidence of refractures, deformities, and implant-related complications were also compared among the four groups. RESULTS: There were no significant differences in demographic information among the four groups in terms of sex (p = 0.101), laterality (p = 0.587), Sillence type (p = 0.122), and postoperative follow-up period (p = 0.214). In total, children with Sillence type III had the highest revision rate and the shortest implant survival period; children with Sillence type I had the lowest revision rate and the longest implant survival period; and children with Sillence type IV had the revision rate and the implant survival period between those observed in Sillence types I and III. In Sillence types III and IV, the telescopic rod had lower revision rate (III 24.8%; IV 20.9%) compared to the plate (III 97.2%, p<0.001; IV 80.3%, p<0.001), the elastic nail (III 100.0%, p=0.019; IV 73.9%, p<0.001) and the non-elongating rod (III 65.0%, p<0.001; IV46.9%, p<0.001); the median implant survival period of the telescopic rod (III 48.00 (IQR 28.50) months; IV 43.00 (33.00) months) is longer than the plate (III 11.00 (9.00) months, p<0.001; IV 19.00 (20.00) months, p<0.001), the elastic nail (III 45.00 (37.75) months, p=1.000; IV 19.00 (35.00) months, p=0.028) and the non-elongating rod (III 39.00 (31.75) months, p=0.473; IV 38.50 (29.75) months, p=1.000).A similar trend was observed in Sillence type I (p = 0.063, p = 0.003; respectively). In addition, the incidence of refracture (15.5%), deformity (2.8%) and implant-related complications (23.1%) were also statistically lower in the telescopic rod group. CONCLUSION: In our cohort, lower revision rate and longer implant survival period were observed in telescopic rod group. This was mainly due to the significant lower incidence of refracture, deformity and implant-related complications with the use of telescopic rod.

Scoliosis in osteogenesis imperfecta: results of posterior spinal fusion in 39 patients.

PURPOSE: To evaluate the outcomes of scoliosis corrective surgery in Osteogenesis Imperfecta (OI) patients with primarily pedicles screw fixation in terms of correcting and maintaining the correction of the spinal deformity, and to assess for several peri-operative parameters and complications associated with this surgical treatment. METHODS: Retrospective case series of 39 consecutive patients with OI treated surgically for scoliosis. The surgeries were performed between 2002 and 2020 by three different surgeons. All patients' medical peri-operative and post-operative charts were evaluated. Radiological assessment was performed by evaluation of the pre-operative, immediate post-operative and last follow-up plain radiographs. RESULTS: There were 20 females and 19 males included in this review with a mean age of 14 years (range, 6-20 years) at the time of surgery. The median follow-up time was 7.9 years. The mean pre-operative cobb angle (CA) of the major curve was 76.5 degrees (SD ± 18.9), decreasing to 42.6 (SD ± 17.4) in the long-term post-operative follow-up (P < 0.001). A total of 21 adverse events in 16 patients were noted. Only 4 patients required subsequent invasive surgical treatment or prolonged hospital stay. All other patients were treated conservatively with no lasting complication. CONCLUSION: Scoliosis surgical correction in OI patients seems to yield acceptable results, with maintained coronal plane surgical correction in the long-term follow-up. Even though a high peri-operative complications rate is observed in this series, there were no long-term sequelae or lasting complications. LEVEL OF EVIDENCE: Level IV, Case series.

Retrosigmoid microvascular decompression as a treatment for trigeminal neuralgia in a patient with osteogenesis imperfecta.

Individuals with osteogenesis imperfecta develop pathologic bone due to genetic defects in collagen synthesis. These patients are prone to skull base abnormalities with resultant lower cranial nerve deficits, most common of which is trigeminal neuralgia. Typically, such patients are managed medically, and surgical options are not well explored for those patients, who become refractory to medication management. While microvascular decompression is often recommended for patients with classical trigeminal neuralgia, neurovascular compression by MRI, and normal skull base anatomy, ablative procedures have been described for patients with trigeminal neuralgia and osteogenesis imperfecta. MVD via a retrosigmoid approach has not been described in a patient with trigeminal neuralgia and skull base abnormalities secondary to osteogenesis imperfecta. A 23-year-old man with osteogenesis imperfecta was referred with right-sided classical trigeminal neuralgia. His trigeminal pain had become refractory to a number of medications. High-resolution MRI demonstrated compression of the trigeminal nerve by the superior cerebellar artery. Microvascular decompression of the trigeminal nerve via a retrosigmoid craniectomy was performed, and he remains pain-free 6 months after surgery. Microvascular decompression of the trigeminal nerve through a retrosigmoid approach can be an effective surgical treatment for young patients with trigeminal neuralgia secondary to osteogenesis imperfecta.

Surgical outcomes for spinal deformity in osteogenesis imperfecta.

STUDY DESIGN: IRB approved retrospective review of the the SHOnet (Shriners Health Outcomes Network). OBJECTIVES: Osteogenesis imperfecta (OI) is the most prevalent genetic disorder of bone and connective tissue in the pediatric population, with an incidence of 1/15,000-20,000. Scoliosis has been reported to be present in 39-100% of OI patients and may continue to progress into adulthood but there is little information on the perioperative outcomes, rates of revision surgery and complications in this fragile population. The purpose of this study is to examine the prevalence of scoliosis in the OI population across a tertiary care multihospital system. The rate at which these patients progress to surgery, the perioperative complication rate and if there is an association between complications and age at surgical intervention, pre-operative Cobb angle, number of fractures, and type of OI. Incidence of scoliosis in OI may be lower than previously reported, many may never require surgical intervention and those that do require surgical intervention have an 8% reoperation rate at an average of 3.3 years postop. METHODS: IRB approved retrospective review of the the SHOnet (Shriners Health Outcomes Network), electronic health record data warehouse from January 1, 2011 and December 31, 2017. Inclusion criteria included International Classification of Diseases (ICD) code for osteogenesis imperfecta and scoliosis. The data warehouse was queried for age, gender, presence of vertebral body fractures, proximal junctional kyphosis, basilar invagination, bisphosphonate use, and perioperative complications including postoperative infection. RESULTS: There were 2372 patients with osteogenesis imperfecta, 429 or 18.1% also had a diagnosis of scoliosis, while 81.9% did not (see Table 1). Only 74 patients (17.2%) of the patients that had scoliosis underwent spine surgery, 12 of which had staged surgery with an average preop thoracic Cobb 58.18 (range 7-115), and thoracolumbar Cobb 59.83 (range 5-145). Six patients (8%) required revision spine surgery. Average time to revision was 3.88 years (6.9-69mo). Bisphosphonate use was present in 35.5% of patients that did not require surgery and in 40.5% that did. CONCLUSION: With over 2300 patients, this is the largest study to date on scoliosis in patients with OI. We found that contrary to prior studies which had indicated the incidence of scoliosis ranges from 39 to 100% that it was only present in 18% of our patients. This indicates that the incidence of scoliosis in OI is potentially lower than previously reported, though due to the nature of this study it may be underreported. Furthermore, only 17% of those patients in our study with scoliosis eventually underwent surgery. It is important to note that many patients treated non-operatively had deformity within the operative range, which may indicate that they were not considered good candidates for surgical intervention. Lastly, we found that bisphosphonate use did not appear to affect the likelihood of progression to surgery in this group of patients, though this may be due to later initiation of the bisphosphonate use.

Pelvic Ring and Peritrochanteric Fractures in a Patient with Osteogenesis Imperfecta Treated Surgically: Case Report with a 5-Year Follow-Up.

BACKGROUND Osteogensis imperfecta (OI) is a rare disorder associated with brittle bones, skeletal deformities, short stature, and conductive hearing loss. It is caused by mutations in genes encoding collagen type I production and is associated with multiple fractures occurring during a patient's lifetime. Atypical fractures can occur without a history of previous injury or diagnosis. CASE REPORT A 52-year old man sustained a fall from his own height. He experienced pelvic pain but could bear weight. He was referred to the hospital by his primary care physician and was admitted to the Orthopedic Department with a pelvic ring fracture. We performed open reduction and internal fixation with a reconstruction plate via an intrapelvic approach. At 12 weeks after discharge, he sustained a peritrochanteric fracture. It was surgically treated with a dynamic hip screw. The patient commenced weight-bearing 20 weeks after the initial procedure. Bone union was achieved clinically and radiographically. He did not have any revision procedures. At the time of this report, 5 years after the described period, he felt no discomfort around the hips, sacroiliac joints, and pubic symphysis. CONCLUSIONS OI poses a difficult treatment challenge, but, achieving a good clinical and functional result is possible with a precise surgical technique and meticulous preoperative planning. Clinicians should always consider the possibility of a simultaneous fracture occurrence in different parts of the body. Some patients present without a previous diagnosis, and we should always have some suspicion of OI in cases of young patients presenting with low-energy fractures.

Challenges of total knee arthroplasty in osteogenesis imperfecta: case report and literature review.

The majority of adults with mild osteogenesis imperfecta report significant functional impairment due to musculoskeletal concerns. Knee osteoarthritis is common in these patients. Although total knee arthroplasty has become a highly efficient surgical technique for osteoarthritis, this procedure remains uncommon in patients with osteogenesis imperfecta. This current case report describes the important clinical aspects of osteogenesis imperfecta that must be considered during the planning and performance of a total knee replacement. A 62-year-old female patient with a history of osteogenesis imperfecta suffered from severe osteoarthritis of the knee with valgus deformity. Two years after posterior stabilized total knee arthroplasty, her Hospital for Special Surgery knee score had improved from preoperative 53 points to 85 points at the final follow-up. The current case report describes the crucial technical aspects of a successful total knee replacement in this uncommon scenario. Underlying deformities and concomitant pathologies constitute specific surgical challenges. Special care should be taken to protect the patient from potential complications.

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

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, and massive blood loss was not uncommon. The risks for airway or regional anesthesia difficulties were low. Pain scores could be controlled to be ≤3 via multiple techniques.