Mortality after spinal fusion in children with cerebral palsy and cerebral-palsy-like conditions: A 30-year follow-up study.

AIM: To report survival probability of a large cohort of children with cerebral palsy (CP) after spinal fusion. METHOD: All children with CP who had spinal fusion between 1988 and 2018 at the reporting facility were reviewed for survival. Death records of the institutional CP database, institutional electronic medical records, publicly available obituaries, and the National Death Index through the US Centers for Disease Control were searched. Survival probabilities with different surgical eras, comorbidities, ages, and curve severities were compared using Kaplan-Meier curves. RESULTS: A total of 787 children (402 females, 385 males) had spinal fusion at a mean age of 14 years 1 month (standard deviation 3 years 2 months). The 30-year estimated survival was approximately 30%. Survival decreased for children who had spinal fusion at younger ages, longer postoperative hospital stays, longer postoperative intensive care unit stays, gastrostomy tubes, and pulmonary comorbidities. INTERPRETATION: Children with CP who required spinal fusions had reduced long-term survival compared with an age-matched typically developing cohort; however, a substantial number survived 20 to 30 years after the surgery. This study had no comparison group of children with CP scoliosis; therefore, we do not know whether correction of scoliosis affected their survival.

Minimally Displaced Lateral Humeral Condyle Fractures: Optimizing Follow-up and Minimizing Cost.

BACKGROUND: Minimally displaced fractures of the lateral humeral condyle (LHC) may be treated nonoperatively in a long arm cast, but there is not a standardized evidence-based protocol. The aim of this study was to evaluate our nonoperatively treated LHC fractures, determine the risk of subsequent displacement, analyze our practice variability, and develop an evidence-based protocol to safely manage children with LHC fractures and to evaluate potential savings related to this analysis. METHODS: We retrospectively reviewed clinical and radiographic parameters of all patients with LHC fractures at our institution from 2009 to 2015. All patients treated nonoperatively initially were included. We recorded demographic data and calculated the number of visits, casts applied, and radiographs within the first 4 weeks. We also analyzed practice variation among 27 treating providers. The number of children with subsequent displacement needing operative fixation was determined. We also looked at the average duration for each follow-up visit and the charges/costs associated with casting and radiographs. RESULTS: There were 271 children with LHC fractures treated nonoperatively. Twenty-one were excluded because of the lack of adequate radiographs. There were 157 boys and 93 girls (average age 6.7 y). According to the Jakob classification, fracture types were as follows: 1 (230), 2 (20), and 3 (0). Within the first 4 weeks, the average number of visits was 2.6 (range: 1 to 5), average number of casts was 2.4 (range: 1 to 4), and the average number of radiographs was 9.4 (range: 2 to 31). Only 3 patients with LHC fracture type Jakob 1 were taken to the operating room post injury (9, 12, and 15 d, respectively) for subsequent displacement. CONCLUSION: Displacement in appropriately selected LHC fractures treated nonoperatively was rare (3/250, 1.2%) in this cohort, and the data question the need for multiple visits and radiographs in the first 4 weeks. Optimal follow-up (proposed follow-up at 10 to 15 d after injury and then 4 to 6 wk with radiographs, including an internal oblique view) would be safe, minimize waste, and result in better value-based care. LEVEL OF EVIDENCE: Level IV.

Tobramycin Blood Levels after Local Antibiotic Treatment of Bone and Soft Tissue Infection.

Local antibiotic delivery using different carriers plays an important role in both infection prophylaxis and treatment. Besides dead space management, these carriers have the advantage of providing a high concentration of local antibiotics with a lower risk of systemic toxicity. Few studies have reported on systemic toxicity associated with antibiotic-impregnated carriers. The present study investigates the systemic tobramycin concentration at 24, 48 and 72 h postoperatively after using tobramycin-loaded polymethyl methacrylate (PMMA) and calcium sulfate (CS) as local antibiotic carriers. Additionally, this work assesses the renal function postoperatively for indications of acute kidney injury (AKI). Fifty-two patients were treated in 58 procedures with tobramycin and vancomycin-loaded PMMA, CS, or both. All systemic tobramycin levels were <2 mcg/mL at 72 h, and the resulting rate of AKI was 12% (7/58). In conclusion, local tobramycin antibiotic delivery using PMMA, CS, or both remains a safe and effective modality in the treatment of osteomyelitis as long as the surgeon is aware of its possible nephrotoxic effect.

Neuromonitoring for Proximal Fibular Osteochondroma Excision.

BACKGROUND: The peroneal nerve is at risk when excising tumors in the proximal fibula. The rate of nerve injuries during proximal fibular tumor resection varies from 3% to 20%. Our goal was to report our experience with resection of osteochondromas in the proximal fibula and describe the technique and utility of neuromonitoring during excision of proximal fibular osteochondromas (PFO). METHODS: Patients with a diagnosis of symptomatic PFO who had undergone excision at one institution from 1994 to 2018 were included. An institutional review board-approved retrospective review was performed. Intraoperative neuromonitoring was provided from 2006 on by a single group utilizing a multimodality protocol. RESULTS: This study contains 29 patients who had excision of osteochondromas in the proximal fibula. Of these 29 consecutively monitored patients, there were 34 involved extremities. Intraoperative neuromonitoring alerts occurred in 10/29 (34.5%) procedures, which included 3 electromyography (EMG) (30%), 2 motor-evoked potential (20%), 1 somatosensory-evoked potential (10%), and 4 alerts with a combination of EMG/motor-evoked potential/somatosensory-evoked potential changes (40%). The interventions that were taken resulted in resolution of the neuromonitoring changes in all procedures. Postoperatively, we noted 2 (6.9%) new mild sensory deficits, which resolved during follow up. There were 3 patients in whom pre-existing sensory-motor deficits improved but not completely after surgery, 1 motor weakness, and 2 with residual paresthesia. In those initially presenting with paresis, there was improvement in 8 of the 8 extremities by the last follow-up visit. Pain as a symptom was resolved in all cases. There were no iatrogenic foot drop injuries. The average follow up was 32.2 months. CONCLUSIONS: Neuromonitoring during PFO excision demonstrated a high number of alerts, all of which resolved following timely corrective action. The use of neuromonitoring may help decrease the risk of iatrogenic postoperative neurological deficits following fibular osteochondroma surgery. LEVEL OF EVIDENCE: Level IV.

The impact of asymmetry on the radiographical outcomes following hip reconstruction in patients with cerebral palsy.

PURPOSE: The purpose of this study was to evaluate the impact of asymmetric hip dysplasia on the outcome of hip reconstruction in patients with cerebral palsy according to preoperative migration percentage (MP). METHODS: This study was institutional review board-approved for retrospective cohort review. From 2008 to 2018, 65 patients met inclusion criteria: Gross Motor Function Scale Classification (GMFSC) III to V with spastic hips (MP > 30%) who underwent bilateral hip reconstruction, with a follow-up > 24 months. Main exclusion criteria: children with associated syndromes or chromosomal disorders. The cohort was subdivided into three groups according to preoperative MP difference between hips: Group A > 50%, group B 20% to 50% and Group C < 20%. Subsequently, the groups were analyzed individually and then compared. The asymmetry of extended abduction of the hip was also evaluated and separated into three groups: no asymmetry (< 20° difference), mild asymmetry (20° to 50° difference) and severe (> 50° difference). RESULTS: In total, 65 patients underwent bilateral bony reconstructive surgery (130 hips). Mean age at surgery was 10.1 years (sd 3.6; 3.6 to 18.4). Mean age at follow-up was 14.7 years (sd 3.8; 8 to 21). Preoperative GMFSC distribution was grade III (four, 6%), IV (15, 23%) and V (46, 71%). In all, 21 symmetric hips (< 20% MP difference) had a preoperative MP difference of 9% and a follow-up MP difference of 18% (p > 0.05); 32 had a preoperative MP difference of 34% and a follow-up MP difference of 16% (p < 0.0001); 12 had a preoperative MP difference of 80% and a follow-up difference of 6% (p < 0.0001). According to pre- and postoperative abduction values, the mean high hip abduction preoperatively was 34° (sd 17°), whereas low hip abduction was 23° (sd 17°). CONCLUSION: Hips with asymmetrical dysplasia and/or abduction undergoing bilateral reconstructive surgery focused on symmetric abduction, and corrected dysplasia in patients with cerebral palsy has improved symmetry in hip abduction and MP. Obtaining this goal immediately postoperatively is maintained to medium-term follow-up. LEVEL OF EVIDENCE: IV.

Cervical kyphosis: A predominant feature of patients with osteogenesis imperfecta type 5.

OBJECTIVES: Osteogenesis imperfecta (OI) is a heterogeneous group of genetic disorders of connective tissue that cause skeletal fragility and extra-skeletal manifestations. Classically, four different types of OI were distinguished. Type 5 OI was added due to its distinct clinical and radiographic features. In 2012, two independent groups identified a recurrent heterozygous c.-14C>T mutation in IFITM5 as the responsible genetic change for this type of OI. To our knowledge, cervical kyphosis has not been identified in the literature as a finding in type 5 OI patients. This is a retrospective review of a cohort of patients with type 5 OI and a description of associated cervical spine deformity. METHODS: After institutional review board approval, a retrospective review identified 13 patients with type 5 OI. Clinical, radiologic, and genetic data from 2002 to 2020 were reviewed. RESULTS: We identified 13 patients with clinical diagnosis of type 5 OI. Twelve had molecular confirmation and the classic IFITM5, c.14C>T gene mutation was identified. The remaining individual did not undergo genetic testing. Dentinogenesis imperfecta was observed in one patient, while blue sclerae or hearing loss were not present. All patients had at least one fracture and four underwent intramedullary rodding. Radiologic features included subphyseal metaphyseal radiodense line in 12/13 patients (92%), interosseous membrane calcification in seven of 13 patients (54%) (more commonly noted in the upper extremities), and hypertrophic callus in six of 13 patients (46%). Thoracolumbar spinal deformities were seen in six of 13 patients (46%) with two of these individuals requiring surgery. Cervical kyphosis was noted in nine of 13 individuals (69%) ranging in age from 3 months to 22 years. Anterior wedging of the cervical vertebral bodies was noted in the absence of any fractures. Six of nine individuals demonstrated listhesis of C2-C3 or C3-C4 segment. Magnetic resonance imaging studies were performed and reviewed in patients with cervical kyphosis and subluxation; three patients showed narrowing of spinal canal without cervical cord compression and one asymptomatic patient showed impingement of the spinal cord. CONCLUSIONS: Cervical kyphosis appears to be a common feature of type 5 OI. It can be a presenting and apparently life-long association and does not appear to be caused by vertebral body fractures. Evaluation for cervical kyphosis should be performed in patients with a suspected or confirmed diagnosis of type 5 OI. Furthermore, if cervical kyphosis is noted in an individual with OI, type 5 OI should be considered.Level of evidence: IV.

Sprengel Deformity in Biological Sisters.

Sprengel deformity is the main congenital shoulder deformity seen in children. First described by Otto Sprengel in 1891, it consists of a high-riding, dysplastic, and malrotated scapula leading to functional and cosmetic impairments. It has never been reported in siblings. Case Presentation: Two sisters, 8 and 9 years old, presented for an evaluation of atraumatic limitation in the shoulder range of motion and neck webbing with an unknown family history. Physical examination revealed a small high-riding scapula, webbed neck, and painless limitation in shoulder abduction (<70°) and flexion (<80°). The 9-year-old sibling had a bilateral shoulder involvement, and the younger had unilateral. Imaging revealed bony and fibrous omovertebral connections between the dysplastic scapulas and cervical spine along with Klippel-Feil deformities. Both sisters underwent scapula repositioning via a modified Woodward procedure. The omovertebral connection was resected followed by scapula derotation and inferior migration. Both had a dramatic improvement in cosmesis and near-complete restoration of shoulder function at follow-up. Conclusions: Although uncommon, Sprengel deformity results in notable derangement of shoulder function. If untreated, children experience difficulty with most overhead activities and often have cosmetic reports. Although no previous genetic link has been identified, its presence in biological sisters suggests that more research is needed.

Desarrollo de un material de fijación ósea inédito (Clavo Gap) para pacientes con talla baja y fragilidad ósea: osteogénesis imperfecta / Development of a bone fixation device (Gap Nail) for patients with short stature and fragile bones: osteogenesis imperfecta

La mayoría de los sistemas de fijación ósea no están diseñados para pacientes de baja estatura con huesos de pequeño diámetro y longitud, como los que se presentan en osteogénesis imperfecta, raquitismo y displasias óseas con afectación de las fisis. En niños con defectos del metabolismo óseo, los clavos telescópicos que se fijan en las fisis, suelen ser los más indicados. El Clavo de Fassier-Duval tiene excelentes reportes, pero no controla la rotación y funciona solo en personas con placas de crecimiento abiertas. Para adultos y adolescentes con huesos pequeños y débiles, no existía un clavo intramedular ajustado a sus tamaños. Esta carencia llevó al autor a involucrarse en el diseño del CLAVO GAP. Tiene diámetros desde 4,8 mm y longitudes hasta 320 mm, canulado, capacidad de bloqueo proximal y distal, se puede utilizar en fémur, tibia y húmero. Es común que estos pacientes presenten debilidad de la cortical lateral del tercio proximal del fémur, por lo que se diseñó para agregarle una fijación al cuello femoral y el trocánter. Presentamos 5 casos realizados por los autores con este sistema de fijación ósea en pacientes con osteogénesis imperfecta. En todos los casos se logró el objetivo de estabilizar los segmentos óseos extremadamente débiles, con diámetros por debajo de los parámetros convencionales, sin requerir uso de férulas post-quirúrgicas(AU)

Most of the systems for bone fixation are not designed for patients of short stature with small bones, like in the patients with osteogénesis imperfecta, rickets and bone dysplasia with affectation of the fisis. In children with defects of the bone metabolism, the nails that telescope and hold in the physes, usually are indicated. Fassier-Duval Nails has excellent reports, but it does not have control of the rotation and it works only in patients with physes open. For adults and adolescents with small and weak bones, did not exist an endomedular nail that fit its sizes. This deficiency was an incentive to the author to become involved in the design of the GAP NAIL. It has diameters from 4.8 mm and lengths to 320 mm. It is canulated. It has capacity of being locked proximal and distally. It can be used in femur, tibia and humerus. It is common that these patients present weakness of the lateral cortex of the proximal third of the femur, reason why was designed to have the possibility to fix the femoral neck and trochanter. We present 5 cases were this system was used in patients with OI. In all the cases the objective was reached, that was to stabilized bones extremely fragile, with diameters below the conventional parameters, without requiring use of braces in the postop(AU)