Pediatric Hand and Wrist Fractures in Osteogenesis Imperfecta: An Analysis of Incidence, Patient-specific Risk Factors, and Fracture-specific Characteristics.

BACKGROUND: Children with osteogenesis imperfecta (OI) frequently present with fractures; however, hand and wrist fractures (HWFs), those distal to the radial and ulnar diaphysis, are seldom observed. Yet, HWFs remain among the most common fractures in children with non-OI. The objective of this study was to identify the incidence of OI HWFs. Secondary objectives aimed at identifying patient-specific risk factors for HWFs in OI and comparing clinical courses to non-OI HWFs. METHODS: A retrospective cohort study was conducted. Database query by ICD-10 codes identified 18 patients with OI HWF, 451 patients with OI without HWFs, and 26,183 patients with non-OI HWF. Power analysis estimated appropriate sample sizes and random sampling was utilized to collect patients. Patient demographics, OI-specific variables, fracture morphology, and fracture clinical courses were recorded. Data were analyzed for patient-specific and fracture-specific factors affecting OI HWF incidence. RESULTS: Of patients with OI, 3.8% (18/469) sustained HWFs. Patients with OI HWF were significantly older than patients with OI without HWFs ( P = 0.002) with no differences in height, weight, ethnicity, sex, or ambulatory status. Compared with non-OI HWFs, patients with OI HWF were significantly shorter ( P < 0.001), weighed less ( P = 0.002), and were less likely to be ambulatory ( P < 0.001). OI HWFs were more commonly on the side of hand dominance ( P < 0.001) with transverse patterns ( P = 0.001). OI HWFs were less frequent in the thumb ( P = 0.048) and trended towards significance in the metacarpals ( P = 0.054). All OI HWFs were treated nonoperatively with similar union rates and refracture rates to non-OI HWFs. Multivariate regression showed that older patient age (odds ratio: 1.079, 95% CI: 1.005,1.159, P = 0.037) and OI type I (odds ratio: 5.535, 95% CI: 1.069, 26.795, P = 0.041) were significant prognosticators for HWFs in patients with OI. CONCLUSION: OI HWFs are uncommon (3.8%, 18/469) but specific HWF morphologies and locations are more common in patients with OI; however, these are not pathognomonic. Older patients with mild penetrance of type I OI are at the highest risk for HWFs. OI HWFs do well when managed nonoperatively with noninferior clinical courses compared with non-OI HWFs. LEVEL OF EVIDENCE: Level III.

The Incidence of Vertebral Exostoses in Multiple Hereditary Exostoses and Recommendations for Spinal Screening.

BACKGROUND: Multiple hereditary exostoses (MHE) lead to the development of pedunculated or sessile osteocartilaginous lesions. Vertebral involvement occurs in MHE and encroaching intracanal exostoses can result in devastating consequences. Magnetic resonance imaging (MRI) of the entire spine has been used to screen for vertebral exostoses to detect high-risk patients. The primary purpose of this investigation is to determine the incidence of vertebral and encroaching intracanal exostoses in patients with MHE. A secondary purpose is to determine if pelvis and rib exostoses serve as "harbinger" lesions of vertebral involvement in MHE. METHODS: A retrospective chart review was performed on 39 patients (21 male and 18 female individuals) with MHE who underwent routine spinal screening with noncontrast entire spine MRI. The average age at screening was 12.3 years (range, 3 to 17 y). Screening was ordered consecutively on patients seen during the study period who were between ages 8 and 18 years or had complaints that could be related to encroaching intracanal exostoses. RESULTS: The incidence of vertebral exostoses in this cohort of 39 patients with MHE was 28% (11 total). An encroaching intracanal exostosis was seen in 3 patients (2 cervical, 1 thoracic). Nonencroaching vertebral exostoses were discovered in 8 patients. Sufficient pelvis and rib imaging to determine the presence of pelvis and rib exostoses was available in 8 of those with vertebral exostoses and 19 of those with no vertebral exostoses on screening MRI. In this cohort, the sensitivity and specificity of the presence of both pelvis and rib exostoses for determining the presence of spinal involvement in MHE are 88% and 5%, respectively. CONCLUSIONS: Based on the results of this cohort, vertebral exostoses are common in MHE, and screening MRI of the entire spine can be used to determine which patients need close observation. If a more selective screening protocol is utilized, an entire spine MRI could be obtained for patients who desire increased physical activity levels or for patients with both pelvis and rib exostoses. At a minimum, treating physicians should monitor patients with MHE closely for neurological symptoms and have a low threshold to obtain advanced spinal imaging. LEVEL OF EVIDENCE: Level III-diagnostic.

Comparison of baseline characteristics and postoperative complications in neuromuscular, syndromic and congenital scoliosis.

Nonidiopathic scoliosis encompasses a group of diagnoses, including neuromuscular scoliosis, syndromic scoliosis and congenital scoliosis. The objective of this study was to compare the preoperative and postoperative clinical differences in pediatric nonidiopathic scoliosis patients with neuromuscular scoliosis vs. syndromic scoliosis/congenital scoliosis. This is a single-center retrospective review of all pediatric patients undergoing spinal instrumentation for nonidiopathic scoliosis during a 5-year period. Neuromuscular scoliosis patients ( n = 144), syndromic scoliosis patients ( n = 44) and congenital scoliosis patients ( n = 52) were compared. Demographics, patient characteristics and outcomes were compared. Neuromuscular scoliosis patients had lower BMI z-scores and were more likely to have pulmonary disease, technology dependence and seizure disorder. Additionally, neuromuscular scoliosis patients underwent bigger procedures with more levels fused and a higher rate of pelvis fixation. By direct comparison, neuromuscular scoliosis patients tended to have more complications including deep surgical site infections, readmission in 30 days, return to operating room in 90 days and emergency care visits in 90 days. When controlling for the differences in their preexisting conditions and surgical procedure, we found that pelvic fixation was a major confounding factor, whereas the others had no effect. We further subanalyzed cerebral palsy patients and found this group to exhibit no difference in complications compared to other neuromuscular scoliosis subtypes. Neuromuscular scoliosis patients have different characteristics and subsequent postoperative complications than those with syndromic scoliosis and congenital scoliosis. The difference in complication profile is mainly due to differences in surgical procedure and a higher rate of pelvic fixation. This should be considered when planning nonidiopathic scoliosis surgery among multidisciplinary teams.