Congenital Pseudoarthrosis of the Tibia (CPT) is a rare condition with a reputation for recurrent fractures and failure to achieve union. A large variety of surgical procedures have been attempted for the treatment of fractured cases of CPT with an average rate of union without refracture of only 50%. Intentional cross-union between the tibia and fibula has been reported to improve these results to 100% union with no refractures. This is a retrospective study of 39 cases of CPT in 36 patients treated by the Paley cross-union protocol with internal fixation, bone grafting, zoledronic acid infusion and bone morphogenic protein 2 (BMP2) insertion. All 39 cases of CPT united at the tibia and developed a cross-union to the fibula. Two patients had a persistent fibular pseudarthrosis, one that was later treated at the time of planned rod exchange and one that has remained asymptomatic. There were few postoperative complications. There were no refractures during the up to 7-year follow-up period. The most common problem was the Fassier-Duval (FD) rod pulling through the proximal or distal physis into the metaphysis (66.7%). This did not negatively affect the results and was remedied at the time of the planned rod exchange. The Paley Cross-Union Protocol is very technically demanding, but the results have radically changed the prognosis of this once sinister disease.
Intramedullary limb lengthening (LL) is now achievable through motorized intramedullary devices. While this technology mitigates some complications of external-fixation-based lengthening, many complications common to all lengthening procedures persist. New challenges and complications exclusive to this newer technology are also presented. The LL surgeon should be aware of and ready to respond to complications involving device malfunctions, poor local bony and soft-tissue biology, patient compliance, neurovascular compromise, joint instability, regenerate problems, and others. While technology will continue to evolve, study of and adherence to foundational principles of LL will minimize risks and optimize patient outcomes.
Background: One of the most common pediatric fractures is a midshaft both bone forearm fracture. The preferred nonoperative treatment is cast immobilization for 6 to 8 weeks; however, 4% to 8% refracture within 6 months. There are no comparative studies evaluating the efficacy of bracing after cast immobilization. We hypothesized that children treated with prolonged functional bracing would have a lower rate of refracture than casting alone or shortterm bracing. Methods: This is a retrospective review of children younger than 15 years of age treated nonoperatively following radius and ulnar shaft fractures treated at 3 tertiary pediatric hospitals. We excluded distal radius/ulna fractures, isolated fractures of the radius/ulna, and fractures near the elbow. Logistic regression analysis on casting plus functional bracing was run to determine if age, translation, or the number of days in brace were associated with refracture. The incidence of refracture was compared between groups. Results: A total of 1549 patients were screened and 426 were included in the study [111 casting only (CO), 259 casting plus functional brace <8 wk (CFB <8 wk), 56 casting plus functional brace ≥ 8 wk (CFB ≥ 8 wk)]. In comparing the groups, CO was the youngest (4.4 y vs. 6.3 and 8.4 y). The initial translation and angulation of the radius and ulna were significantly greater in the CFB ≥ 8 weeks group. Regression analyses shows no association between refracture and initial fracture characteristics including age, translation, or the number of days in brace. The CO group had 3 refractures (2.7%), the CFB <8 weeks group had 13 (5%) and the CFB ≥ 8 weeks group had 1 (1.8%); demonstrating no statistical significance. Conclusion: Extended fracture bracing, following a period of cast immobilization, did not lead to a statistically significant difference in refracture rate. Contrary to previous cases series, the benefit of bracing seems nominal. Larger, prospective studies are needed to better understand targets for treatment. Level of Evidence: This is the first level III retrospective comparison study of its kind.
Background: Congenital synostosis of the knee is a rare condition with limited data on treatment options and outcomes. This study reports clinical findings, treatment approach, and surgical/clinical outcomes for congenital synostosis of the knee. Methods: An institutional review board-approved retrospective review of patients with congenital synostosis of the knee presenting to 2 institutions between 1997 and 2021 was performed. Results: Eight patients (13 knees) with a median follow-up of 11.3 years (3.3 to 17 y) were included. Seven patients had associated syndromes. Patients presented with an average knee flexion deformity of 100° (range 60 to 130°) and delayed walking ability. Seven patients had associated upper extremity hypoplasia/phocomelia. The average age at the index surgery was 4.3 years (range 1.2 to 9.2 y). Synostosis resection with gradual deformity correction was performed in most patients. An attempt was made at a mobile knee in some patients, but all went on to knee fusion. Mean flexion deformity at final follow-up was 11.6° (range: 0 to 40°) and 5 limbs were fused in full extension. Mean limb length discrepancy at final follow-up was 6.8 cm (range: 0 to 8 cm). All patients maintained their improved ambulation status at final follow-up. Twenty-two complications were identified. Conclusions: Reliable correction of the deformity associated with congenital knee synostosis was achieved at a median follow-up of 11 years. Importantly, all patients maintained their improved ambulation at final follow-up. This is the largest study on patients with congenital knee synostosis and outlines a reconstructive approach to improve ambulatory status. Level of Evidence: Level IV
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Background: Congenital femoral deficiency (CFD) is a rare condition that affects the morphology of the hip and surrounding soft tissues. Bony deformity and distorted muscular anatomy are well known, but no studies have described the relationship of the femoral neurovascular (NV) bundle to surgically relevant anatomic landmarks. The authors compared the location of the femoral NV bundle on the affected side in patients with CFD with the unaffected side. The authors hypothesized that the bundle on the pathologic side would be in an abnormal position relative to the unaffected side. Methods: Thirty-three patients diagnosed with unilateral CFD who had undergone preoperative magnetic resonance imaging of the pelvis were included in our study. The authors identified the femoral NV bundle on the axial cuts and measured its distance from the anterior superior iliac spine (ASIS), anterior inferior iliac spine (AIIS), and lesser trochanter (LT). Anatomic percent change and absolute measurements were then compared and correlated with associated boney deformities and the Paley classification. Results: The distance from the femoral NV bundle to the ASIS, AIIS, and LT was significantly different compared with the unaffected side. The AIIS absolute distance and AIIS percent change significantly correlated with the neck-shaft angle of the proximal femur. Conclusions: In patients with CFD, the femoral NV bundle seems to be further from the LT and closer to the AIIS on the affected side when compared with the unaffected side. magnetic resonance imaging may be helpful to understand the course of the femoral NV bundle before reconstruction in patients with CFD; however, the authors recommend identification of the femoral NV bundle before transection of the proximal rectus femoris tendon to provide safe surgical care. Level of Evidence: Level IV—case-control study of diagnostic studies.
There are multiple forms of enchondromatosis with Ollier’s and Maffucci’s being the most prevalent types. Limb length discrepancy is a common problem in patients with Ollier’s and Maffucci’s enchondromatosis. There are multiple reports about lengthening bones in patients with enchondromatosis using external fixators. However, there are no case series regarding the use of implantable lengthening technology. The purpose of this paper is to describe our experience with implantable nail lengthening in patients with enchondromatosis. A retrospective chart and radiographic review of patients with enchondromatosis who underwent implantable nail limb lengthening was performed. Seven patients with 14 bony segments were reviewed. A total of 11/14 lengthenings were completed without difficulty. There were no issues in terms of fixation location in patients with Ollier’s disease. One patient with Maffucci’s syndrome experienced migration of the nail during two lengthenings due to a combination of intralesional fixation and preconsolidation. One patient with Ollier’s disease developed a knee extension contracture requiring manipulation under anesthesia. No other complications were recorded. The use of implantable nail lengthening to resolve limb length discrepancies in patients with Ollier’s disease appears to be safe and effective.
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