Skeletal Manifestations of Gaucher's Disease: A Case Report and Literature Review.

Gaucher's disease (GD) is a lysosomal storage disorder characterized by the storage of glucosylceramide in macrophages ("Gaucher cells"), mainly in the reticuloendothelial system. GD type 1 (GD1) is the most common phenotype that usually manifests with hepatosplenomegaly, cytopenias, and bone involvement. Skeletal manifestations are the most debilitating characteristic and result in significant morbidities. We describe a case of GD1, first presented by a nontraumatic bone fracture. The case presentation highlights the importance of considering GD among the differential diagnosis of nontraumatic fractures, avascular necrosis, and infarcts of the bones. Early diagnosis and treatment improve the course of disease and avoid irreversible sequelae.

Birmingham Hip Resurfacing at 25 years.

Aims: The Birmingham Hip Resurfacing (BHR) was introduced in 1997 to address the needs of young active patients using a historically proven large-diameter metal-on-metal (MoM) bearing. A single designer surgeon's consecutive series of 130 patients (144 hips) was previously reported at five and ten years, reporting three and ten failures, respectively. The aim of this study was to extend the follow-up of this original cohort at 25 years. Methods: The study extends the reporting on the first consecutive 144 resurfacing procedures in 130 patients for all indications. All operations were undertaken between August 1997 and May 1998. The mean age at operation was 52.1 years (SD 9.93; 17 to 76), and included 37 female patients (28.5%). Failure was defined as revision of either component for any reason. Kaplan-Meier survival analysis was performed. Routine follow-up with serum metal ion levels, radiographs, and Oxford Hip Scores (OHSs) was undertaken. Results: Overall implant survival was 83.50% (95% confidence interval (CI) 0.79 to 0.90) at 25 years, and the number at risk was 79. Survival in male patients at 25 years was 89.5% (95% CI 0.83 to 0.96) compared to 66.9% for female patients (95% CI 0.51 to 0.83). Ten additional failures occurred in the period of ten to 25 years. These involved an adverse reaction to metal debris in four patients, a periprosthetic femoral neck fracture affecting five patients, and aseptic loosening in one patient. The median chromium levels were 49.50 nmol/l (interquartile range (IQR) 34 to 70), and the median cobalt serum levels were 42 nmol/l (IQR 24.50 to 71.25). The median OHS at last follow-up was 35 (IQR 10 to 48). During the 25-year study period, 29 patients died. Patient survival at 25 years was 75.10% (95% CI 0.67 to 0.83). Conclusion: This study demonstrates that MoM hip resurfacing using the BHR provides a durable alternative to total hip arthroplasty (THA), particularly in younger male patients with osteoarthritis wishing to maintain a high level of function. These results compare favourably to the best results for THAs.

Can Non-Contrast 3T MRI scans predict the type of labral procedure in hip arthroscopy?

PURPOSE: The study proposed a simple classification system that aimed at predicting the labral procedure during hip arthroscopy using images from unenhanced 3T MRI scans. PATENTS AND METHODS: Forty patients undergoing hip arthroscopy had their MRI scans reviewed pre-operatively by one of two senior radiologists and classified into: type 1: stable labrum with intra-substance degeneration (no labral repair required), type 2: unstable labrum, no intra-substance degeneration (labral repair required), or type 3: unstable labrum with intra-substance degeneration (Labral repair not feasible). Hip arthroscopy is carried out by one of two surgeons and classified accordingly while blinded to the radiologists' assessment. RESULTS: The pre-operative radiological classification managed to predict the labral procedure in 29 cases out of 40 (72.5%). The gamma value was 0.83, and the kappa value was 0.46 denoting moderate agreement. The inter-class correlation coefficient was 0.52 indicating moderate reliability. CONCLUSION: The proposed classification showed a moderate agreement between the radiological findings and arthroscopic findings. As we cannot accurately correlate the MRI findings with the type of surgical management, the surgeon should be prepared for all scenarios of labral procedures. LEVEL OF EVIDENCE: Level IV (case series).

Minimally invasive sacroiliac fusion, a case series, and a literature review.

INTRODUCTION: Non-autoimmune sacroiliac joint pain contributes to nearly a quarter of low back pain patients. Non-surgical management fails to satisfy patients. A new minimally invasive technique for sacroiliac stabilization has been introduced, defying the traditional rules of fusion. The results outside explanatory trials and in day-to-day practice have not been reported. MATERIALS AND METHODS: This case series includes 20 patients diagnosed with chronic sacroiliac pain resistant to conservative management for at least 6 months. The diagnosis was confirmed with a positive sacroiliac injection. Patients underwent stabilization using the iFuse® implant. Patients were followed up for a minimum of one year. The primary outcome was the functional outcomes, assessed using VAS, ODI, and SF36. Secondary procedure rates, complication rates, and radiological assessments of fusion were collected as secondary outcomes. RESULTS: At one year, the mean VAS score improved from 81.25 ± 10.7 SD preoperatively to 52.5 ± 26.8, p-value 0.0013. The mean ODI improved from 54.8 ± 11.21 SD preoperatively to 41.315 ± 15.34, P value = 0.0079. The mean PCS and MCS of SF36 improved by 17 and 20 points, respectively. Only 55% of patients achieved the MCID for the VAS score. 35% of the cohort had secondary procedures. DISCUSSION: Minimally invasive sacroiliac fusion resulted in an improvement in mean functional scores with a wide dispersion. Patients not achieving MCID are patients with either a malpositioned implant, an associated lumbar pathology, or an inaccurate diagnosis. Our results are underwhelming compared to similar work but are still better than conservative cohorts in comparative studies. CONCLUSION: Minimally invasive sacroiliac fusion can be used successfully in select patients. Attention to diagnosis and surgical technique can improve the reproducibility of results.

Incidence of DVT and PE after surgical reconstruction for pelvic and acetabular fractures: Does routine duplex scanning affect management?

BACKGROUND: Prevention and detection of thromboembolism with pelvic and acetabular fractures remains controversial. The aim of this study was to evaluate a protocol using LMWH prophylaxis and duplex screening both pre-operatively (if there is a delay > 72 h to surgery) and post-operatively at day 5-7. We assessed the incidence of thromboembolism and associated risk factors. METHODS: A total of 255 consecutive patients who underwent pelvic and/or acetabular reconstruction in a major trauma and tertiary referral centre between January 2013 and December 2015 were studied. Forty-three patients were excluded due to non-adherence to protocol leaving 212 patients included in the study. RESULTS: Patients were of mean age 49 years (15-94) and mean ISS 24.5 (4-66). Pre-operative screening detected two patients with asymptomatic above-knee DVT who then underwent pre-operative IVC filter insertion. Post-operative screening detected seven patients (3%) with lower limb DVTs (3 proximal and 4 below knee). The three patients with proximal DVTs were fully anticoagulated and did not develop symptomatic PE. Six patients (2.8%) developed post-operative PE, four of which were symptomatic and confirmed by CT angiography. Seven patients (3%) died in the post-operative period due to non-VTE-related causes. The overall rate of VTE was 6%, including DVT 4% and PE 2.8%. PE was associated with administration of tranexamic acid in ED (p > 0.03) and total amount of blood transfused during admission (p > 0.001). VTE was not associated with age, injury type, ISS, delay to surgery or associated injuries. CONCLUSION: A protocol-based approach to VTE prophylaxis and screening in trauma patients with pelvic and/or acetabular reconstruction resulted in no VTE-related mortality. Pre- and post-operative screening for DVT changed the management in five patients, with none developing PE. Patients requiring more aggressive resuscitation had a higher rate of PE. The VTE rate was lower than previously reported.

Strategies of management of traumatic periprosthetic acetabular fractures around a pre-existing total hip arthroplasty.

INTRODUCTION: and aim: Traumatic peri-prosthetic fractures are relatively rare fractures that pose a significant surgical challenge. They have a fracture pattern that is different from its iatrogenic counterpart. This study aimed at reviewing the modalities of treatment of such injuries, proposing a treatment algorithm and reporting the outcomes of these injuries. PATIENTS AND METHODS: We propose an algorithm for management of traumatic peri-prosthetic acetabular fractures which depends on fracture displacement, implant stability and associated bone loss.Twelve patients with traumatic peri-prosthetic acetabular fractures were treated between January 2012 and December 2018. All patients were treated surgically. Patients were allowed immediate weight bearing as pain allowed. Assessment was carried out using the Oxford Hip Score (OHS) and the Merle D'aubigné score (MDP). RESULTS: Mean patient age 71 (range: 59-80). 8 patients underwent implant removal, fracture fixation and re-implantation of revision acetabular cup (displaced fracture, unstable implant, adequate bone stock). Three patients required acetabular reconstruction (inadequate bone stock). One patient underwent revision acetabular component. One patient suffered from recurrent dislocation that required surgical intervention. Mean follow up was 27 Months (range 12-48). Mean OHS was 36 (range 10-47). Mean MDP was 12 (range 9-18) with 80% excellent and good results. CONCLUSION: Surgical management of peri-prosthetic acetabular fractures can produce good to excellent results provided adequate assessment and surgical planning have been carried out. Fracture displacement, implant stability and bone stock should be carefully assessed.

Does Routine Postoperative Computerized Tomography After Acetabular Fracture Fixation Affect Management?

INTRODUCTION AND AIMS: The use of routine postoperative computerized tomography (CT) scan after acetabular fracture reconstruction remains controversial. CT scan may provide more accurate detail regarding metalwork position, retained intra-articular fragments, and quality of reduction but does expose the patient to additional radiation dosage and incurs increased cost. The aim of this study was to evaluate a protocol of routine postoperative CT scan for all acetabular fractures after surgical fixation and assess the effect this has on patient management. PATIENTS AND METHODS: The perioperative fluoroscopic images and postoperative plain radiographs of 122 patients who underwent surgical stabilization of a displaced acetabular fracture were reviewed and categorized into 3 groups: (1) safe, when there was no suspicion of metalwork malposition or intra-articular fragments; (2) inconclusive, when it was not possible to exclude malposition; or (3) definite malposition or intra-articular penetration of implants. The findings were compared with postoperative CT scans. The quality of reduction of the acetabular fracture was graded on plain radiographs using the Matta criteria and compared with the CT scan using a standardized technique. RESULTS: Fractures that were categorized as safe on plain radiographs were confirmed to have no metalwork malposition on CT scan in 94% of the cases, with the other 6% having insignificant findings that did not require revision surgery. When plain radiographs were inconclusive (n = 17), 4 patients had metalwork malposition documented on CT scan and 2 of these required revision surgery. There was an increased risk of implant malposition with use of spring plates for posterior wall stabilization. There was significant variation between the quality of reduction when assessed with plain radiographs as compared with CT scans (P < 0.001). In 42% of the patients who were thought to have anatomic reduction on plain radiographic assessment, the reduction was either imperfect or poor based on CT assessment. CONCLUSIONS: CT scans were more accurate than plain radiographs in detecting metalwork malposition and in assessing quality of reduction of the acetabular fracture. The use of postoperative CT scans may be restricted to a group of fractures that have inconclusive or definite malpositioning of implants on perioperative or postoperative radiographs, especially with use of spring pates or to those patients in whom quality of reduction needs more accurate assessment for quality assurance or prognostic reasons. LEVEL OF EVIDENCE: Level IV; Diagnostic -Investigating a diagnostic test.

Single versus double column fixation in transverse fractures of the acetabulum: A randomised controlled trial.

INTRODUCTION: Biomechanical studies have compared fixation methods in transverse acetabular fractures, yet there is not enough clinical data to suggest an optimal fixation method. The aim of this randomized controlled trail was to compare fracture stability in posterior plating alone versus posterior plating and anterior column lag-screw fixation in treatment of transverse and transverse with posterior wall acetabular fractures. METHODS: Thirty patients were randomized to one of two groups, either posterior fixation alone (single column group), or posterior plating and anterior fixation with percutaneous anterior column screw (double column group). Patients were followed up with serial radiographic assessments documenting any loss of reduction, utilizing Matta's radiological criteria, measuring the roof arc angles and by measuring any change in the femoral head offset. RESULTS: Fifteen patients were randomized to each group. Mean patient age was 31 years, mean follow up period was 19 months (range 12-24). There was no significant differences between the two groups with regards the quality of post-operative reduction, blood loss, hospital stay and functional score using the modified Merle D'Aubinge and Postel score. The operative time was significantly longer in the double column fixation group (130 min versus 104 min). There was no loss of reduction observed in either of the two groups. CONCLUSION: Single poster column fixation in transverse and transverse posterior wall fractures showed similar result to double column fixation, in terms of fractures stability in the follow up period, quality of reduction and early functional outcome.

Routine Postoperative Computed Tomography Scans After Pelvic Fracture Fixation: A Necessity or a Luxury?

INTRODUCTION: There is no consensus regarding the postoperative radiology imaging protocol after pelvic fracture surgery. Some institutes routinely scan all patients after their surgery, others do not. The aim of this study was to assess the value of routine use of computed tomography (CT) scans after pelvic fracture surgery and to determine the sensitivity of conventional plain radiographs and intraoperative fluoroscopy in detecting metalwork malposition. PATIENTS AND METHODS: The radiographs and clinical notes of patients undergoing pelvic fracture surgery in the period between January 2010 and December 2015 were reviewed. Patients were categorized into 2 main groups: group A-patients whose fixation entailed the use of a sacroiliac (SI) screws and group B-patients whose fixation did not require an SI screw. Furthermore, the patients were classified according to the position of metalwork in their postoperative plain radiographs and perioperative fluoroscopy into 3 groups: (1) Safe: When there was no suspicion of metalwork malposition. (2) Suspicious: When there was some suspicion of malposition but radiographs were inconclusive. (3) Definite: When plain imaging showed a definite malposition. RESULTS: One hundred ninety-eight patients were included in this study (161 in group A and 37 in group B). In group A, 148 (92%) were classified as safe, 10 were suspicious (6%), and 3 (2%) showed definite malposition. Of the fractures that were believed to be safe on plain radiographs, 78% were confirmed to be safe on CT scans, whereas 22% showed malpositioned metalwork, and 7 patients (4%) required a revision surgery. Plain radiographs showed a sensitivity of 27% in detecting metalwork malposition and a specificity of 99%. Increasing the number of screws significantly increased the risk of malposition and reoperation (P = 0.006 and 0.002 respectively). The plain images of group B were all classified as safe. The CT scans detected 2 cases with long metalwork protruding into the soft tissues, none of which required a revision surgery. CONCLUSION: Perioperative fluoroscopy and plain postoperative radiographs have a low sensitivity in detecting the metalwork malposition after pelvic fracture surgery. We recommend the use of routine postoperative CT scans in patients whose fixation entails the use of SI screws. In this series, routine scanning of patients who did not have SI screws added no significant clinical value. LEVEL OF EVIDENCE: Level IV Diagnostic. See Instructions for Authors for a complete description of levels of evidence.

Open complete anterior dislocation of the sacro-iliac joint in a 4-year-old boy: a case report of a rare injury with 5-year follow-up.

Pelvic fractures are uncommon in children and account for between 0.3 and 7.5% of all pediatric injuries. Open pelvic fractures only account for up to 12.9% of all pediatric pelvic fractures. An unusual case of open complete anterior sacro-iliac joint dislocation in a 4-year-old boy is presented with a long-term follow-up. The multidisciplinary approach is reported with review of the current literature. A 4-year-old male presented to our institution in January 2012 after having been run over by a tractor. He presented with gross hemodynamical instability, MISS of 25, and an unstable lateral compression type III pelvic fracture with complete anterior dislocation of the left hemipelvis and a groin wound extending into the left thigh. The patient was managed in accordance with the ATLS and open fracture guidelines. Reduction in the dislocated SI joint was achieved via a posterior approach to the SI joint, followed by fixation with 2K wires in S1 and S2 sacral segments, with an anterior external fixator. Pelvic asymmetry post-reduction was 0.9 cm, compared to 16 cm post-injury, and asymmetry persisted till final follow-up at 5 years. At 5 years, patient regained full function, including recreational sport activities. Patients scored a 96/96 on the Majeed score (after excluding 4 points for sexual function). We believe that posterior reduction in an anteriorly dislocated SI joint in the pediatric population is a viable option. A coordinated, multidisciplinary approach and restoration of pelvic ring stability can lead to optimal outcome.

Quadrilateral plate fractures of the acetabulum: Proposition for a novel classification system.

BACKGROUND: Currently, there is no definition or classification system for quadrilateral plate (QLP) fractures; the aim was to anatomically and radiologically characterise the QLP, propose a definition and classify QLP fractures. METHODS: This study included an anatomical component and a radiological component. The anatomical study aimed at defining the characteristics of the QLP; the QLP was identified using four articulating bony pelvis specimens. A titanium mesh was placed on the specimens' quadrilateral surface; standard anteroposterior and oblique views were obtained, and axial CT images, to determine the radiological landmarks. The radiological study included the review of images of patients with QLP fractures; fractures involving the QLP were identified in a series of 609 consecutive patients with acetabular fractures. RESULTS: We considered QLP fractures where the QLP is separated from both columns of the acetabulum; this was found in 16% (98 cases). They were mostly encountered with associated both columns fractures (60 cases; 61%), Separation of the QLP could be complete or incomplete, or simple or comminuted, so QLP fractures were divided into three types: QLP1, simple with incomplete separation; QLP2, comminuted with incomplete separation; QLP3, comminuted with complete separation (QLP4), simple with complete separation. CONCLUSION: The QLP was characterised, and a definition and classification system; Cairo University Hospitals (CUH) Classification was proposed for these fractures. We believe that this classification may prove useful in the future for the identification and management of these fractures.