Prosthetic femoral interposition arthroplasty with adult humeral implant, for children with cerebral palsy and symptomatic hips.

BACKGROUND: Immobile patients with cerebral palsy can suffer with painful dislocated hips. Decision-making and surgical management can prove challenging in this cohort of patients, with hips that cannot be reconstructed. METHODS: We conduced a retrospective chart review of all patients who underwent prosthetic femoral interposition arthroplasty (PFIA) by two surgeons from 2013 to 2021, for unreconstructable hips. We compared pain and range of motion in preoperative period to the postoperative period. Caregiver reported outcomes were used to assess satisfaction post operatively. During the follow up, radiographs of the PFIA were obtained to assess for proximal migration, heterotopic ossification and loosening of implants. RESULTS: Eleven index surgeries, which met the inclusion criteria, were included in this study. These were performed in eleven patients with an average follow up of 45 months. Regarding pain and range of motion post-operatively an excellent or good result was seen in nine cases. Two patients were classified as having a fair result with none having a poor result. Most caregivers reported being satisfied or very satisfied with the post-operative outcomes. CONCLUSION: A prescriptive operative solution to the painful dislocated hip in children with spastic cerebral palsy remains elusive. In this study, we have demonstrated both clinically and radiologically satisfactory results post proximal femoral interposition arthroplasty, for those patients with unreconstructable hips. Patient caregiver reported outcomes, show that the majority of caregivers were satisfied or very satisfied with the outcome of the surgery.

Management of bone disease in cystinosis: Statement from an international conference.

Cystinosis is an autosomal recessive storage disease due to impaired transport of cystine out of lysosomes. Since the accumulation of intracellular cystine affects all organs and tissues, the management of cystinosis requires a specialized multidisciplinary team consisting of pediatricians, nephrologists, nutritionists, ophthalmologists, endocrinologists, neurologists' geneticists, and orthopedic surgeons. Treatment with cysteamine can delay or prevent most clinical manifestations of cystinosis, except the renal Fanconi syndrome. Virtually all individuals with classical, nephropathic cystinosis suffer from cystinosis metabolic bone disease (CMBD), related to the renal Fanconi syndrome in infancy and progressive chronic kidney disease (CKD) later in life. Manifestations of CMBD include hypophosphatemic rickets in infancy, and renal osteodystrophy associated with CKD resulting in bone deformities, osteomalacia, osteoporosis, fractures, and short stature. Assessment of CMBD involves monitoring growth, leg deformities, blood levels of phosphate, electrolytes, bicarbonate, calcium, and alkaline phosphatase, periodically obtaining bone radiographs, determining levels of critical hormones and vitamins, such as thyroid hormone, parathyroid hormone, 25(OH) vitamin D, and testosterone in males, and surveillance for nonrenal complications of cystinosis such as myopathy. Treatment includes replacement of urinary losses, cystine depletion with oral cysteamine, vitamin D, hormone replacement, physical therapy, and corrective orthopedic surgery. The recommendations in this article came from an expert meeting on CMBD that took place in Salzburg, Austria, in December 2016.

The impact of the SARS-CoV-2 pandemic on referral characteristics in a national tertiary spinal injuries unit.

BACKGROUND: The SARS-CoV-2 pandemic has had profound implications on healthcare institutions. AIMS: This study aims to assess and compare referral patterns during COVID-19 to corresponding dates for the preceding 3 years (2017-2019), in order to preemptively coordinate the logistics of the surgical unit for similar future experiences. METHODS: Retrospective review for our institution, a national tertiary referral centre for spine pathology. Two distinct time-points were chosen to represent the varied levels of social restriction during the current pandemic: (i) study period 1 (SP1) from 11 November 2020 to 08 June 2020 represents a national lockdown, and (ii) study period 2 (SP2) from 09 June 2020 to 09 September 2020 indicates an easing of restrictions. Both periods were compared to corresponding dates (CP1: 11 March-08 June and CP2 09 June-09 September) for the preceding 3 years (2017-2019). Data collected included age, gender, and mechanism of injury (MOI) for descriptive analyses. MOIs were categorised into disc disease, cyclist, road-traffic-accident (RTA), falls < 2 m, falls > 2 m, malignancy, sporting injuries, and miscellaneous. RESULTS: All MOI categories witnessed a reduction in referral numbers during SP1: disc disease (-29%), cyclist (-5%), RTAs (-66%), falls < 2 m (-39%), falls > 2 m (-17%), malignancy (-33%), sporting injuries (-100%), and miscellaneous (-58%). Four of 8 categories (RTAs, falls < 2 m, malignancy, miscellaneous) showed a trend towards return of pre-lockdown values during SP2. Two categories (disc disease, falls > 2 m) showed a further reduction (-34%, -27%) during SP2. One category (sporting injuries) portrayed a complete return to normal values during SP2 while a notable increase in cyclist-related referrals was witnessed (+ 63%) when compared with corresponding dates of previous years. CONCLUSION: Spinal injury continues to occur across almost all categories, albeit at considerably reduced numbers. RTAs and falls remained the most common MOI. Awareness needs to be drawn to the reduction of malignancy-related referrals to dissuade people with such symptoms from avoiding presentation to hospital over periods of social restrictions.

Use of a novel corrective device for correction of deformities in adolescent idiopathic scoliosis.

BACKGROUND: Adolescent idiopathic scoliosis (AIS), with an incidence of 3%, is a common deformity. Correction of severe curvature of the deformity has attracted much investigation to achieve safe, reproducible results. We present our experience with a novel device for the correction of deformities across a spectrum of curve types, the rod link reducer. This system allows direct visualization of a mass derotation to achieve deformity correction. METHODS: Prospective cohort study of patients with severe AIS treated in our institution during 2017 with major TL/L curves. Pre- and postoperative Cobb angles and coronal balance; operative time; and estimated blood loss, fusion levels, and screw density were recorded. Our results were split between those with a Lenke A/B classification and Lenke C, with a goal of correction of curve in the former and achieving good coronal balance with a preservation of distal motion segments in the latter. RESULTS: There were 31 patients enrolled in our series. Within the Lenke A/B group, there were 18 patients, achieving a mean correction of 56% (SD 10%) and a correction of a mean coronal balance of 14.5 mm (SD 12.5 mm) of C7 from the CSVL to 10.9 mm (SD 10.6 mm). Our screw density was 1.3 screws (SD 0.1) per vertebrae fused. Our operative time was 185 min (SD 38 min). Average recorded blood loss was 721 ml (SD 289). In our Lenke C cohort, preoperative and postoperative mean Cobb angles (SD) were 73.3 (13.4) and 33.8 (11.9), respectively, with an average correction of 54% (SD 11%). The mean (SD) operative time was 03:35 (SD 00:35). Fusion levels were over a mean of 12.1 (SD 1.7) vertebrae, with a screw density of 1.3 (SD 0.1) per level. Mean intraoperative blood loss was 829 ml (SD 355). No patient received an allogenic blood transfusion. There were no adverse neurological events in this patient cohort. CONCLUSIONS: The link rod system allows for excellent correction of spinal deformity and a short operative time.

The role of real-time PCR testing in the investigation of paediatric patients with community-onset osteomyelitis and septic arthritis.

BACKGROUND: Culture yield in osteomyelitis and septic arthritis is low, emphasising the role for molecular techniques. AIMS: The purpose of this study was to review the laboratory investigation of childhood osteomyelitis and septic arthritis. METHODS: A retrospective review was undertaken in an acute tertiary referral paediatric hospital from January 2010 to December 2016. Cases were only included if they had a positive culture or bacterial PCR result from a bone/joint specimen or blood culture, or had radiographic evidence of osteomyelitis. RESULTS: Seventy-eight patients met the case definition; 52 (66%) were male. The median age was 4.8 years. Blood cultures were positive in 16 of 56 cases (29%), with 11 deemed clinically significant (Staphylococcus aureus = 8, group A Streptococcus = 3). Thirty-seven of 78 (47%) bone/joint samples were positive by culture with S. aureus (n = 16), coagulase-negative Staphylococcus (n = 9) and group A Streptococcus (n = 4), being the most common organisms. Sixteen culture-negative samples were sent for bacterial PCR, and four were positive (Kingella kingae = 2, Streptococcus pneumoniae = 1, group A Streptococcus = 1). CONCLUSIONS: Sequential culture and PCR testing can improve the detection rate of causative organisms in paediatric bone and joint infections, particularly for fastidious microorganisms such as K. kingae. PCR testing can be reserved for cases where culture is negative after 48 h. These results have been used to develop a standardised diagnostic test panel for bone and joint infections at our institution.

Traumatic spinal injuries on farms: Patients treated in the national spinal unit of Ireland 2005-2015.

INTRODUCTION: Farming is a major industry in Ireland, employing 6% of the workforce but accounts for 40% of workplace fatalities (Meredith, 2008).1 The use of machinery and proximity to large livestock poses significant risk for spinal injuries. This study reviews mechanism of injury, injuries sustained, and the treatment undertaken. RESULTS: Of 31 patients, 26 sustained high-energy injuries with machinery and livestock implicated in the majority of accidents. 77% sustained poly-trauma and 52% had permanent neurological injury. CONCLUSIONS: The proximity to heavy machinery and livestock pose significant risk for spinal injuries. This study should encourage increased efforts to improve farm safety.

Aggressive plasmablastic lymphoma of the thoracic spine presenting as acute spinal cord compression in a case of asymptomatic undiagnosed human immunodeficiency virus infection.

BACKGROUND CONTEXT: Plasmablastic lymphoma (PBL) is a rare aggressive variant of diffuse large B-cell lymphoma. PURPOSE: We describe a rare case of an aggressive PBL presenting as acute spinal cord compression requiring thoracic decompression and fusion, in a case of previously undiagnosed human immunodeficiency virus (HIV) infection. STUDY DESIGN: A case report. PATIENT SAMPLE: A patient with PBL of the thoracic spine. OUTCOME MEASURES: Preoperative magnetic resonance imaging, pathologic findings from the operative specimen, and serum HIV testing confirmed the diagnosis. METHODS: We present the case of a 33-year-old Caucasian woman with a 10-day history of thoracic back pain and a 1-day history of sudden-onset bilateral lower limb weakness and paresthesia from below the level of the umbilicus (American Spinal Injury Association [ASIA] Grade C). Magnetic resonance imaging demonstrated an extradural mass extending from T3 to T6 within the left posterior canal, resulting in significant cord compression. A complete debulking of the tumor mass and an instrumented posterior thoracic fusion was performed. RESULTS: Histopathologic examination of the specimen revealed tumor cells of PBL, and subsequent HIV testing was positive. She was treated with intravenous and intrathecal chemotherapy to prevent recurrence. Her lower limb neurologic status improved to ASIA Grade D over the subsequent 2 weeks. CONCLUSIONS: We report the case of an aggressive PBL presenting as acute spinal cord compression requiring urgent surgical intervention, on a background of undiagnosed HIV infection.

Birth-associated femoral fractures: incidence and outcome.

Femoral fractures resulting from birth injuries are rare. The authors undertook a study to assess their incidence and outcome. Seven patients from a total of 55,296 live births suffered 8 femoral fractures (incidence 0.13 per 1,000 live births). Twin pregnancies, breech presentations, prematurity, and disuse osteoporosis were associated with the occurrence of a fracture. The typical injury pattern was a spiral fracture of the proximal half of the femur, which was held in an extended position. A variety of treatment modalities were used, including gallows traction, spica cast, and Pavlik harness. All patients in this study, regardless of treatment, had a satisfactory clinical outcome, with no evidence of limb length discrepancy or angular deformity on follow-up.