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.

Surgical treatment of osteogenesis imperfecta: a summary of the incidence of femoral implant-related complications in children with Sillence type I, III and IV.

PURPOSE: This study explored the incidence of IRCs used in the procedures of the femur in children with osteogenesis imperfecta (OI) and investigated the independent risk factors of IRCs. METHODS: Three hundred eight-eight cases of surgical data about children with OI were included, who were treated with plate, elastic nail, Kirschner wire and telescopic rod. The choice of different procedures depended on the age of children, the status of femur and the availability of devices. Patient demographics and major IRCs were recorded to compare the outcomes of the four procedures. Then, Cox proportional hazard regression was used to analyse the independent risk factors of IRC, and subgroup analysis was applied to further verify the above results. RESULTS: The total incidence of IRC in the four groups was 90.1% (191/212) for plate, 96.8% (30/31) for Kirschner wire, 87.7% (57/65) for elastic nail and 30.0% (24/80) for telescopic rod. The incidence of IRC in the telescopic rod was lower than that in plate, elastic nail and Kirschner wire (P < 0.001). Cox proportional hazard regression analysis confirmed that procedure was the independent risk factor of IRC (HR, 0.191; 95% CI, 0.126-0.288; P < 0.001), fracture (HR, 0.193; 95% CI, 0.109-0.344; P < 0.001) and deformity (HR, 0.086; 95% CI, 0.027-0.272; P < 0.001). In addition, age of surgery was the independent risk factor of fracture (HR, 0.916; 95% CI, 0.882-0.952; P < 0.001) and deformity (HR, 1.052; 95% CI, 1.008-1.098; P = 0.019). Subgroup analysis confirmed that age of surgery, gender, classification, preoperative state and angle did not affect the effect of telescopic rod on reducing the risk of IRCs. CONCLUSIONS: In our cohort, lower incidence of IRCs was observed in telescopic rod group compared with plate, Kirschner wire and elastic nail. Procedure and age of surgery were independent risk factors of fracture. Likewise, procedure and age of surgery were independent risk factors of deformity, and procedure was independent risk factors of IRC.

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.

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.

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.

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.

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.

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.

[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.

[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.

Severe Basilar impression in osteogenesis imperfecta treated with halo gravity traction, occipitocervicothoracic fusion, foramen magnum and upper cervical decompression and expansive duroplasty: a technical note.

Osteogenesis imperfecta (OI) is a rare bone disease due to an abnormal synthesis of 1-type collagen. OI is frequently associated with basilar impression (BI), defined by the elevation of the clivus and floor of the posterior fossa with subsequent migration of the upper cervical spine and the odontoid peg into the base of the skull. Bone intrinsic fragility leading to fractures and deformity, brainstem compression and impaired CSF circulation at cranio-vertebral junction (CVJ) makes the management of these conditions particularly challenging. Different surgical strategies, including posterior fossa decompression with or without instrumentation, transoral or endonasal decompression with posterior occipito-cervical fusion, or halo gravity traction with posterior instrumentation have been reported, but evidence about best modalities treatment is still debated. In this technical note, we present a case of a 16-years-old patient, diagnosed with OI and BI, treated with halo traction, occipito-cervico-thoracic fixation, foramen magnum and upper cervical decompression, and expansive duroplasty. We focus on technical aspects, preoperative work up and postoperative follow up. We also discuss advantages and limitations of this strategy compared to other surgical techniques.

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.

Multicenter Series of Deformity Correction Using Guided Growth in the Setting of Osteogenesis Imperfecta.

BACKGROUND: The mainstay of deformity correction and fracture prevention for patients with osteogenesis imperfecta (OI) includes osteotomies and intramedullary rodding. Guided growth, described in the setting of skeletal dysplasias, offers a less invasive means of deformity correction. We report a multicenter case series of guided growth procedures in the setting of OI. METHODS: We retrospectively reviewed patients with OI at three institutions from April 2012 to April 2019: 18 patients underwent guided growth for angular deformity correction with minimum 1-year follow-up or full deformity correction and removal of guided growth hardware. Clinical characteristics, deformity measurements, and complications were collected. Distal femoral and proximal tibial hemiepiphysiodesis was performed using figure-of-eight plates and screws, and distal tibial medial hemiepiphysiodesis with cannulated screws. Preoperative and postoperative lateral distal femoral angle, medial proximal tibial angle, and lateral distal tibial angle were measured. Frequency and descriptive statistics were completed. RESULTS: Eighteen patients with OI (five-I, four-III, six-IV, three-V) underwent 33 guided growth procedures with mean follow-up of 3.09 years; all received routine bisphosphonate treatment. Preoperative and postoperative mean joint angles were measured. The location for hemiepiphysiodesis included 8 distal femoral medial, 2 distal femoral lateral, 8 proximal tibial medial, 3 proximal tibial lateral, and 12 distal tibial medial. Twelve of the 33 procedures were in patients who had an intramedullary rod; 1 demonstrated backout of the epiphyseal and metaphyseal screws of a distal femoral medial figure-of-eight plate. It was revised to a larger plate with longer screws and removed upon completion of deformity correction. CONCLUSION: Guided growth may be used as an effective means of angular deformity correction with dysplastic OI bone. Having an intramedullary rod did not preclude the use of a guided growth technique. One procedure demonstrated screw backout. Given the short stature associated with OI, performing a guided growth procedure at an early enough age to allow time for correction should be considered. LEVEL OF EVIDENCE: Level IV-case series.

Complications of Elongating Intramedullary Rods in the Treatment of Lower Extremity Fractures for Osteogenesis Imperfecta: A Meta-Analysis of 594 Patients in 40 Years.

BACKGROUND: Osteogenesis imperfecta is a collagen mutation-related disease characterized by bone fragility and other extraskeletal manifestations. Intramedullary fixation for deformity correction or fracture is the standard care. Elongating rods are designed to accommodate growth, with the aim of preventing additional operations and/or complications associated with nonelongating rods. Although elongating rods have been in use for many years, estimates of the clinical outcomes vary. We conducted a systematic review and meta-analysis to synthesize the literature on outcomes of elongating rods and nonelongating rods. Meta-analysis was used to compare the complication rates and reoperation rates. METHODS: We conducted the literature search, systematic review, and meta-analysis in accordance with Preferred Reporting Items for Systematic reviews and Meta-analyses (PRISMA) guidelines. Comparative cohort studies and large case series detailing complication rates and reoperation rates of elongating and nonelongating rods were included. Random effect models were used to summarize the complication rates and reoperation rates of intramedullary rod procedures. RESULTS: A total of 397 studies were identified and 24 studies were included in the final cohort. Compared with rates from nonelongating rods, osteogenesis imperfecta Patients using elongating rods had a complication rate of 61% and a reoperation rate of 78%. Reoperation rates dropped with succeeding generations of elongating rods. Pooling data from 600 patients, we identified a 9% complication rate per rod per follow up year and 5% reoperation rate per rod and per follow up year in the cohort of elongating rod fixation. The Bailey-Dubow rod had the highest complication rate per rod per follow up year (12%), largely because of its T piece relate problems. The most popular fixator Fassier-Duval rod had a complication rate per rod per follow up year of 9%. About 68% of complications were mechanical-biological related. CONCLUSION: Pooling data from published literature demonstrates the advantage of elongating rods over nonelongating rods. However, as high as 9% complication rate per rod per follow up year was associated with elongating fixation. Notably, most complications are both mechanical and biological related. LEVEL OF EVIDENCE: Level III.

Retrograde Application of Humerus Fassier-Duval Rod in Osteogenesis Imperfecta: A New Surgical Technique.

BACKGROUND: The aim of this study is to describe the technique of retrograde application of Fassier-Duval (FD) rod for the humerus in patients with osteogenesis imperfecta (OI). This technique was developed to overcome the downsides of the previously used techniques of humerus rodding. METHODS: The study was done at a tertiary care pediatric orthopaedic hospital from April 2014 to August 2021. Skeletally immature patients with OI who underwent retrograde FD rodding were included. This surgery was performed for humeral shaft fractures/bowing limited to the distal half of the bone to ensure appropriate stability of the fixation. Surgical technique of the procedure is described in detail. RESULTS: Six patients with OI, of which 2 (33.3%) had FD rodding bilaterally, were included. The mean age at rodding was 7.6±3.5 (range: 3 to 14) years. The mean duration of postoperative follow-up was 45.5±18.0 (range: 24 to 75) months. All patients had full healing of the fracture/osteotomy, with functional alignment of their humeri. No surgical complications were observed; however, 1 (12.5%) segment only had a traumatic humerus fracture following a fall that was associated with rod migration, occurring 60 months postoperatively. This was treated with a retrograde FD rodding again, with fracture augmentation with plate and screws. CONCLUSIONS: The retrograde FD rodding technique of the humerus in OI patients is relatively simple and preserves the soft tissue surrounding the shoulder joint, with favorable outcomes. Studies with larger sample size and long-term follow-up duration are needed. LEVEL OF EVIDENCE: Level IV-case series.

Tibial Sliding Elastic Nailing Technique in Moderate-to-Severe Osteogenesis Imperfecta: Long-term Outcomes.

BACKGROUND: Osteosynthesis of leg fractures and deformities in children with osteogenesis imperfecta should align the skeleton and overcome its fragility during growth with a telescopic effect. A high rate of mechanical complications is associated with various surgical techniques described in the literature. PURPOSE: The objective of this work was to assess the long-term clinical and radiologic outcomes of tibial sliding elastic nailing technique. METHODS: A total of 22 children with an average age of 4.7 years were operated using the technique between 2004 and 2018 unilaterally (6) or bilaterally (16), that is, 38 operations. They were listed according to the Sillence classification into type I (3), III (17), or V (2). The nails were introduced percutaneously at the distal tibial epiphysis through the medial malleolus, and in the prespinal area for the proximal tibial epiphysis. The stainless-steel rods diameter was 1.5 to 2.5 mm, adapted to the size and weight of the patient. Realignment osteotomies were performed if necessary. Radiographic data including the correction of the deformation in the frontal and sagittal planes, as well as the width at mid-shaft of the tibia in the frontal and sagittal planes, were reviewed. Gillette Functional Score, assessment of pain, mechanical and infectious complications were collected. RESULTS: The average follow-up was 8.6 years. In the frontal plane, preoperative average varus was 8 degrees (maximum, 40 degrees), 5 degrees (maximum, 13 degrees) postoperatively, and 6 degrees (maximum, 12 degrees) at last follow-up. Preoperative valgus was 11 degrees (maximum, 22 degrees), 9 degrees (maximum, 15 degrees) postoperatively, and 9 degrees (maximum, 14 degrees) at the last follow-up. In the sagittal plane, the mean sagittal bowling of the tibia was 32 degrees (4 to 75 degrees) preoperatively, 9 degree (1 to 26 degrees) postoperatively, and 9 degrees (1 to 24 degrees) at last follow-up. The width at mid-shaft of the tibia in the frontal plane was 1.1 cm (0.6 to 1.8 cm) preoperatively and 1.3 cm at the last follow-up (0.7 to 2.0 cm). In the sagittal plane, it was 1.25 cm (0.7 to 2.7 cm) preoperatively and 1.27 cm (0.8 to 2.8 cm) at the last follow-up. Ten patients did not require revision surgery during their follow-up. Sixteen mechanical complications occurred in 12 patients (12 fractures or deformities following a lack of overlap of the 2 rods at an average time of 4.9 years after the initial surgery, 3 prominence of the nail, 1 pseudarthrosis). No infectious complication was reported. Gillette Functional Score was 20.54/65. Fifteen patients were able to walk at last follow-up, and 18 had no painful discomfort. CONCLUSIONS: The tibial sliding elastic nailing technique provides satisfactory clinical and radiologic results over time. Performed in case of fracture or as a preventive treatment, it allows a good correction of angular deformations. It is particularly suitable for young patients with a narrow medullary shaft. LEVEL OF EVIDENCE: Level IV-therapeutic study.

Osteogenesis imperfecta: treatment and surgical management.

PURPOSE OF REVIEW: Osteogenesis imperfecta is a disease with many different causes and clinical presentations. Surgery at a young age is the often required in order to improve the patients' growth development and quality of life. This manuscript highlights the current approach to treat children with osteogenesis imperfecta. The main purpose of this review is to compare and discuss the latest surgical techniques and procedures. RECENT FINDINGS: Recent studies have indicated that telescoping intramedullary Faisser-Duval rods are one of the most suitable surgical devices to correct long bone deformities. The design permits elongation with growth and helps reduce the number of revision surgeries compared to previous static devices. SUMMARY: Osteogenesis imperfecta patients require an interdisciplinary and tailored treatment that involves both medical and surgical components. On the basis of the most recent surgical and medical findings, the authors recommend treating osteogenesis imperfecta patients early with bisphosphonates prior to surgical intervention and then utilizing Faisser-Duval rods in a surgical setting to correct lower extremity deformities and fractures.

Osteogenesis Imperfecta-Who Needs Rodding Surgery?

PURPOSE OF REVIEW: The purpose of this review is to precise the indications for intramedullary rodding of long bones in osteogenesis imperfecta, the classic treatment for fractures and deformities in this condition. RECENT FINDINGS: The use of plates and screws alone is not recommended, but its use in conjunction with rodding is becoming more popular as demonstrated in recent literature. The different types of rods are reviewed and their advantages/disadvantages exposed. There is a clear advantage for telescopic rods in terms of incidence of revision surgery but complications are still to be expected. An interdisciplinary approach combining a medical treatment with a surgical correction of deformities as well as a rehabilitation program is the key for success in the treatment of osteogenesis imperfecta children.

Stapes surgery in osteogenesis imperfecta: retrospective analysis of 18 operated ears.

PURPOSE: To evaluate short and long term results of stapes surgery in patients with osteogenesis imperfecta (OI), METHODS: Retrospective case series of 18 primary stapes surgeries performed on 11 hearing-impaired OI patients with evidence of stapes fixation, in a Tertiary referral center. We analysed pre-operative and post-operative hearing results at 1 month and at least 1 year RESULTS: The main operative findings were stapes fixation, thickened footplate and fragile or fractured stapes crura. No revision surgery was necessary. Hearing improvement was achieved in 94% of the cases. We obtained an air-bone gap closure to within 10 dB in 46% of the cases and to within 15 dB in 92% of the cases at 1-year follow-up. The mean hearing gain in air conduction (at 0.5, 1, 2 and 4 kHz) was 18.4 dB at 1 month and 22.4 dB at 1 year. CONCLUSION: Stapes surgery in OI gives good results with few complications in our series. A hearing gain is often obtained in spite of the sensorineural hearing loss caused by the natural progression of the disease.

Mechanical Analysis of Explanted Telescopic Rods in the Management of Osteogenesis Imperfecta: A Multicenter Study.

BACKGROUND: Telescopic rods in the management of osteogenesis imperfecta fail frequently. This could be attributed to technical errors, rod design, and rod structure. We aimed to analyze the mechanical properties and tribology of explanted male and female components to identify effects of in vivo telescoping that may relate to observed patterns of successful telescoping or failure. METHODS: Recruitment took place at 3 of the 4 English centers for osteogenesis imperfecta. Twenty-five rods explanted for growth or failure during revision to a new rod were analyzed in terms of clinical indication and prerevision imaging to identify if there was a technical mode of failure. Laboratory analysis was performed using optical and scanning electrical microscopy, radiograph diffraction analysis, hardness test, bending test, and energy-dispersive x-ray spectroscopy. RESULTS: All implants tested were of high-grade stainless steel. Female components had inferior strength [mean Vickers hardness property (HV0.3) at 0.3 to 313 kg] in comparison to male components (HV0.3 406) due to different techniques of manufacture. Female rods also had a higher wear coefficient (7.89×10-12 m3/N/m3) than the male rods (6.46×10-12 m3/N/m3). Abrasive wear, shear deformation, scratches, and wear debris were identified in all rods. Male and female components displayed corrosion contributing to adhesive wear. Intraoperatively cut rods, particularly the female components, had irregular ends leading to more wear. CONCLUSIONS: Current manufacturing techniques result in inferior material strength in female components compared with males, which combined with wear patterns is likely to lead to implant failure. Intraoperative cutting of rods may increase risk of failure due to wear. Considering techniques to improve strength as well as design in new implants may lead to better outcomes. LEVELS OF EVIDENCE: Level IV-cross-sectional study.