Do Not Forget the Spine MRI in Children With Arthrogryposis Multiplex Congenita: High Prevalence of Tethered Spinal Cord and Preliminary Clinical Findings Following Detethering.

BACKGROUND: Arthrogryposis multiplex congenita (AMC) encompasses congenital conditions with joint contractures in 2 or more joints. Patients with AMC may have scoliosis and neural axis malformations. The study aimed to determine the prevalence of tethered spinal cord (TSC), characterized by a low-lying conus medullaris, and secondarily, present preliminary findings following surgical untethering in children with AMC. METHODS: Patients 18 years of age and younger with a diagnosis of AMC and a spine MRI were identified. The presence of a TSC was defined as a low-lying conus with termination at or below the lower third of the L2 vertebral body. A pediatric neurosurgeon and a pediatric orthopaedic surgeon independently reviewed MRIs. The medical records of patients with AMC who underwent untethering were examined to evaluate preoperative and postoperative clinical findings. The prevalence of TSC in our AMC patients was compared with published normative data using χ2 analysis. RESULTS: Forty-two of 105 AMC patients (40%) had TSCs. There was a greater percentage of patients with AMC and a TSC compared with the unaffected population (P<0.0001). Sixteen patients underwent detethering through filum terminale sectioning. Nine patients had preoperative neurological deficits in addition to their AMC. There were no postoperative complications. All patients had improvement in their bowel and bladder symptoms within 3 months after their detethering procedure. CONCLUSIONS: TSC is more prevalent in the pediatric population with AMC compared with those without AMC. MRI is recommended for all patients with AMC due to its high prevalence. Although not a benign procedure, surgical detethering may have potential benefits for children with AMC. LEVEL OF EVIDENCE: Level IV-therapeutic study.

Surgery for Spinal Stenosis in Achondroplasia: Causes of Reoperation and Reduction of Risks.

BACKGROUND: Individuals with achondroplasia are prone to symptomatic spinal stenosis requiring surgery. Revision rates are thought to be high; however, the precise causes and rates of reoperation are unknown. The primary aim of this study is to investigate the causes of reoperation after initial surgical intervention in individuals with achondroplasia and spinal stenosis. In addition, we report on surgical techniques aimed at reducing the risks of these reoperations. METHODS: A retrospective review was conducted over an 8-year period of all patients with achondroplasia at a single institution that serves as a large referral center for patients with skeletal dysplasias. Patients with achondroplasia who underwent spinal surgery for stenosis were identified and the need for revision surgery was studied. Data collected included demographic, surgical, and revision details. Fisher exact test was used to determine if an association existed between construct type and the need for revisions. RESULTS: Thirty-three of the 130 (22%) patients with achondroplasia required spinal stenosis surgery. Twenty-four individuals who met the criteria were selected for analysis. The initial spine surgery was at an average age of 18.7 years (SD: 10.1 y). Nine patients (38%) required revision surgeries, and 3 required multiple revisions. Five of 9 (56%) of the revisions had primary surgery at an outside institution. Revision surgeries were due to caudal pseudarthrosis (the distal instrumented segment) (8), proximal junctional kyphosis (PJK) (7), and new neurological symptoms (7). There was a significant association found between construct type and the need for revision ( P =0.0111). The pairwise comparison found that short fusions were significantly associated with the need for revision compared with the interbody group ( P =0.0180). PJK was associated with short fusions when compared with the long fusion group ( P =0.0294) and the interbody group ( P =0.0300). Caudal pseudarthrosis was associated with short fusions when compared with the interbody group ( P =0.0015). Multivariate logistic regression found long fusion with an interbody was predictive of and protective against the need for revision surgery ( P =0.0246). To date, none of the initial cases that had long fusions with caudal interbody required a revision for distal pseudarthrosis. CONCLUSIONS: In patients with achondroplasia, the rate of surgery for spinal stenosis is 22% and the risk of revision is 38% and is primarily due to pseudarthrosis, PJK, and recurrent neurological symptoms. Surgeons should consider discussing spinal surgery as part of the patient's life plan and should consider wide decompression of the stenotic levels and long fusion with the use of an interbody cage at the caudal level in all patients to reduce risks of revision. LEVEL OF EVIDENCE: Level IV-Retrospective case series.

Peroneal Nerve Decompression in Patients with Multiple Hereditary Exostoses: Indications, Complications, and Recurrence.

BACKGROUND: To our knowledge, there have been no studies examining peroneal nerve decompression and proximal fibular osteochondroma excision exclusively in patients with multiple hereditary exostoses (MHE). The purpose of this study was to evaluate the indications, complications, and recurrence associated with nerve decompression and proximal fibular osteochondroma excision in patients with MHE. METHODS: The records on patients with MHE undergoing peroneal nerve decompression from 2009 to 2023 were retrospectively reviewed. Indications, clinical status, surgical technique, recurrence, and complications were recorded and were analyzed using the Fisher exact test, logistic regression, and the Kaplan-Meier method. RESULTS: There were 126 limbs identified in patients with MHE who underwent peroneal nerve decompression. The most common indications were pain over the proximal fibula, tibialis anterior and/or extensor hallucis longus weakness, and dysesthesias and/or neuropathic pain. Seven cases experienced postoperative foot drop as a complication of the decompression and osteochondroma excision. Logistic regression found significant relationships between complications and excision of anterior osteochondromas (odds ratio [OR], 5.21; p = 0.0062), proximal fibular excision (OR, 14.73; p = 0.0051), and previous decompression (OR, 5.77; p = 0.0124). The recurrence rate was 13.8%, and all recurrences occurred in patients who were skeletally immature at the index procedure. The probability of skeletally immature patients not experiencing recurrence was 88% at 3 years postoperatively and 73% at 6 years postoperatively. CONCLUSIONS: Indications for peroneal nerve decompression included neurologic symptoms and pain. The odds of a complication increased with excision of anterior osteochondromas and previous decompression. Recurrence of symptoms following decompression and osteochondroma excision was found exclusively in skeletally immature patients. LEVEL OF EVIDENCE: Therapeutic Level III . See Instructions for Authors for a complete description of levels of evidence.

Transphyseal Humeral Separations: An Often-Missed Fracture.

BACKGROUND: Transphyseal humeral separations (TPHS) are rare injuries often associated with non-accidental trauma, necessitating accurate diagnosis. This study aims to assess the accuracy of diagnosis of TPHS. METHODS: A retrospective review was conducted at five academic pediatric institutions to identify all surgically treated TPHS in patients up to 4 years of age over a 25-year period. Demographics, misdiagnosis rates, and reported misdiagnoses were noted. Comparative analyses were performed to analyze the effects of patient age and injury mechanism on misdiagnosis rates. RESULTS: Seventy-nine patients (average age: 17.4 months) were identified, with injury mechanisms including accidental trauma (n = 49), non-accidental trauma (n = 21), Cesarean-section (n = 6), and vaginal delivery (n = 3). Neither age nor injury mechanism were significantly associated with diagnostic accuracy in the emergency department (ED)/consulting physician group. ED/consulting physicians achieved an accurate diagnosis 46.7% of the time, while radiologists achieved an accurate diagnosis 26.7% of the time. Diagnostic accuracy did not correlate with Child Protective Services (CPS) involvement or with a delay in surgery of more than 24 h. However, a significant correlation (p = 0.03) was observed between injury mechanism and misdiagnosis rates. CONCLUSION: This multicenter analysis is the largest study assessing TPHS misdiagnosis rates, highlighting the need for raising awareness and considering advanced imaging or orthopedic consultation for accurate diagnosis. This also reminds orthopedic surgeons to always have vigilant assessment in treating pediatric elbow injuries. LEVEL OF EVIDENCE: Level III-Retrospective Cohort Study.

Definitive fusions are better than growing rod procedures for juvenile patients with cerebral palsy and scoliosis: a prospective comparative cohort study.

PURPOSE: This study compared the outcomes of juvenile patients with cerebral palsy (CP) and scoliosis who underwent spinal fusion (SF) versus growing rod (GR) surgery. METHODS: Two prospective multicenter registries were queried for patients 8-10 years old with minimum 2-year follow-up who underwent SF or GR surgery (no MCGR). Demographics, radiographs, complications, and outcome scores were recorded. RESULTS: There were 35 patients in the SF and 15 in the GR group. The mean age at surgery was 10 and 9.3 years in the SF and GR groups, respectively (p = 0.004). In the SF group preoperatively, the major curve measured 86° and 80° in the GR group (p = 0.40). "Definitive" surgery in the GR group consisted of SF in 10, implant retention in three, and implant removal in two. The SF group had 60.8% and the GR group had 45.0% correction following "definitive" surgery (p = 0.03). In the SF group, 8 patients and in the GR group, 9 patients (SF = 22.9%, GR = 60.0%) had a complication (p = 0.01). In the SF group, two patients (5.7%) had reoperations for infection; eight patients (53.3%) in the GR group had reoperations for infection and implant complications (p < 0.001). In the SF group, 23/30 parents (76.6%) noted that the child's life "improved a lot." In the GR group, 3/6 parents (50.0%) noted they were "neutral" about their child's ability to do things, 2/6 (33.3%) were "very dissatisfied." CONCLUSIONS: SF treatment for juvenile patients with CP and scoliosis resulted in fewer complications and unplanned reoperations and better radiographic outcomes compared with GR. Quality of life improvements were also better in the SF group. LEVEL OF EVIDENCE: Level III.

Complications following surgical treatment of adolescent idiopathic scoliosis: a 10-year prospective follow-up study.

BACKGROUND: Accurate reporting of long-term complications of surgical treatment of adolescent idiopathic scoliosis (AIS) is critical, but incomplete. This study aimed to report on the rate of complications following surgical treatment of AIS among patients with at least 10 years of follow-up. METHODS: This was a retrospective review of prospectively collected data from a multicenter registry of patients who underwent surgical treatment for AIS with minimum 10-year follow-up. Previously published complications were defined as major if they resulted in reoperation, prolonged hospital stay/readmission, neurological deficits, or were considered life-threatening. Rates and causes of reoperations were also reviewed. RESULTS: Two hundred and eighty-two patients were identified with mean age at surgery of 14.6 ± 2.1 years. Mean follow-up was 10.6 (range 9.5-14) years. Eighty-seven patients had anterior spinal fusion (ASF); 195 had posterior spinal fusion (PSF). The overall major complication rate was 9.9% (n = 28) in 27 patients. Among PSF patients, the complication rate was 9.7% (n = 19) in 18 patients. The complications were surgical site infection (37%), adding-on (26%), pulmonary (16%), neurologic (11%), instrumentation (5%), and gastrointestinal (5%). In ASF patients, the complication rate was 10.3% (n = 9) among nine patients. The complications were pulmonary (44%), pseudoarthrosis (22%), neurologic (11%), adding-on (11%), and gastrointestinal (11%). The reoperation rate was 6.0% (n = 17) among 17 patients. Although most of the complications presented within the first 2 years (60.7%), surgical site infection and adding-on were also seen late into the 10-year period. CONCLUSION: This is the largest prospective study with at least a 10-year follow-up of complications following spinal fusion for AIS, the overall major complication rate was 9.9% with a reoperation rate of 6.0%. Complications presented throughout the 10-year period, making long-term follow-up very important for surveillance. LEVEL OF EVIDENCE: Therapeutic II.

Orthopaedic Manifestations of Transverse Myelitis in Children.

BACKGROUND: Transverse myelitis (TM) is a rare inflammatory disorder of the spinal cord. It can have a heterogeneous presentation with sensory, motor, and autonomic dysfunction. Neurological sequelae of TM include autonomic dysfunction, motor weakness, and/or spasticity. Studies describing orthopaedic deformities and treatments associated with TM are nonexistent. This purpose of this study was to describe the orthopaedic manifestations of TM in children. METHODS: A multicenter retrospective review was conducted of patients, 0 to 21 years of age, with TM presenting over a 15-year period at 4 academic children's hospitals. Those with confirmed diagnosis of TM and referred to an orthopaedic surgeon were included. Demographics, orthopaedic manifestations, operative/nonoperative treatments, and complications were recorded. Descriptive statistics were used for data reporting. RESULTS: Of 119 patients identified with TM, 37 saw an orthopaedic surgeon. By etiology, 23 were idiopathic (62%), 10 infectious (27%), 3 (8%) inflammatory/autoimmune, and 1 (3%) vascular. The mean age at diagnosis was 6.7 (SD: 5.5) years and at orthopaedic presentation was 8.4 (SD: 5.2) years. Orthopaedic manifestations included scoliosis in 13 (35%), gait abnormalities in 7 (19%), foot deformities in 7 (19%), upper extremity issues in 7 (19%), symptomatic spasticity in 6 (16%), lower extremity muscle contractures in 6 (16%), fractures in 6 (16%), hip displacement in 3 (8%), pain in 2 (5%), and limb length discrepancy in 2 (5%) patients. Seven children (19%) were seen for establishment of care. In all, 14 (38%) underwent operative intervention, mainly for soft-tissue and scoliosis management. Four patients had baclofen pump placement for spasticity management. Postoperative complications occurred in 36% of cases, most commonly because of infection. Neither topographic pattern nor location of lesion had a significant relationship with need for hip or spine surgery. CONCLUSIONS: This report describes the orthopaedic manifestations associated with TM in children, nearly 40% of whom required operative intervention(s). Understanding the breadth of musculoskeletal burden incurred in TM can help develop surveillance programs to identify and treat these deformities in a timely manner. LEVEL OF EVIDENCE: Level IV.

Transphyseal Humeral Separations: What Can We Learn? A Retrospective, Multicenter Review of Surgically Treated Patients Over a 25-Year Period.

PURPOSE: Transphyseal humeral separations (TPHS) are rare injuries with only case reports and small series reported in the literature. This multicenter study aimed to assess the various patient characteristics, injury patterns, treatments, outcomes, and complications in a large series of these injuries. METHODS: A retrospective review was conducted at 5 pediatric institutions to identify TPHS that were treated surgically in patients 0 to 3 years of age over a 25-year period. Patient demographics, mechanisms of injury, Child Protective Services involvement, diagnostic modalities, time to surgery, pin size and configuration, time to fracture union, and complications were recorded. RESULTS: A total of 79 patients aged 0 to 46 months, with a mean of 17.6 months, were identified and followed for a median of 57 days postoperatively. The most common mechanism of injury was accidental trauma (n=49), followed by nonaccidental trauma (n=21), cesarean section (n=6), and vaginal delivery (n=3). Child Protective Services were involved in 30 cases (38%). Additional injuries were reported in 19 of the patients; most commonly additional fractures including the humerus, rib, and skull fractures. All patients had elbow radiographs, whereas 4 patients had an elbow ultrasound and/or a magnetic resonance imaging. Time to surgery was greater than 24 hours in 62% of patients (n=49). Intraoperatively, 87% of patients underwent an arthrogram (n=69), 78% of patients had lateral pins only (n=62), averaging utilization of 2.2 pins, and 2 patients underwent an open reduction. In total, 11 complications (14%) were noted, including decreased range of motion (n=4), cubitus varus/valgus (n=6), and need for additional surgery (n=1). No cases of avascular necrosis or physeal arrest were found. No losses of reduction were noted. CONCLUSIONS: This multicenter review provides the largest known demographic and outcomes data on TPHS. TPHS have excellent outcomes in the vast majority of patients when treated surgically. Nonaccidental trauma accounted for 27% of these injuries so it needs to remain high on the differential diagnosis. LEVEL OF EVIDENCE: Level III-retrospective cohort study.

A Metaphyseal Corner Fracture that Wasn't: A Case Report of Osteitis from Congenital Syphilis.

CASE: Congenital syphilis (CS) is an infrequently seen condition in the United States; however, rates of CS have been on the rise. We present a case of an infant with a lesion of the radius that was initially diagnosed as a metaphyseal corner fracture and treated as such until maternal syphilis testing was noted to be positive. Ultimately, the child was diagnosed with CS. She is now undergoing treatment with penicillin and recovering well. CONCLUSION: Although CS is rare, the incidence is on the rise and should remain on the differential of lytic lesions of bone in young children.

Cost Analysis of Treating Pediatric Supracondylar Humerus Fractures in Community Hospitals Compared With a Tertiary Care Hospital.

OBJECTIVE: In the current healthcare environment, providing cost-efficient care is of paramount importance. One emerging strategy is to use community hospitals (CHs) rather than tertiary care hospitals (TCHs) for some procedures. This study assesses the costs of performing closed reduction percutaneous pinning (CRPP) of pediatric supracondylar humerus fractures (SCHFs) at a CH compared with a TCH. METHODS: A retrospective review of 133 consecutive SCHFs treated with CRPP at a CH versus a TCH over a 6-year period was performed. Total encounter and subcategorized costs were compared between the procedures done at a CH versus those done at a TCH. RESULTS: Performing CRPP for a SCHF at a CH compared with a TCH saved 44% in costs (P < 0.001). Cost reduction of 51% was attributable to operating room costs, 19% to anesthesia-related costs, 16% to imaging-related costs, and 7% to supplies. DISCUSSION: Performing CRPP for a SCHF at a CH compared with a TCH results in a 44% decrease in direct cost, driven largely by surgical, anesthesia, and radiology-related savings.

Branching patterns and localization of the common fibular (peroneal) nerve: an anatomical basis for planning safe surgical approaches.

PURPOSE: Given the severity and incidence of injury to the common fibular (peroneal) nerve (CFN), there is a need to further clarify its anatomical location and branching patterns. This project attempts to consolidate current anatomical understanding of this nerve and provide physicians with reproducible measurements regarding the CFN and its branches. METHODS: Dissections were performed on 50 specimens (28 cadavers), both fresh and preserved. The CFN was dissected from its emergence from the fibular tunnel to its anterior tibial recurrent nerve (ATRN), superficial fibular nerve (SFN), and deep fibular nerve (DFN) branches. The CFN branching patterns were assessed and all variations were categorized into four types. RESULTS: Several significant relationships were identified between observable traits and key anatomical characteristics of the CFN. A significant correlation was found between fibular length and distance from the tip of the fibula to the DFN/ATRN branch, as well as between fibular length and distance from the tibial tuberosity to the SFN/DFN and DFN/ATRN branches. An association was identified between length of exposed sub-cutaneous CFN and height. Thickness of the biceps femoris tendon correlated significantly with BMI. CONCLUSIONS: These findings allow physicians to better assess a patient's individual CFN anatomy based on correlations with measureable physical traits and will contribute to anatomic education and successful completion of various surgical, anesthetic, and physical therapy procedures.