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Clinical Imaging Nov 2023Osteochondromas account for 20%-50% of all benign bone lesions. These tumors may present as solitary non-hereditary lesions, which are the most common presentation, or... (Review)
Review
Osteochondromas account for 20%-50% of all benign bone lesions. These tumors may present as solitary non-hereditary lesions, which are the most common presentation, or as multiple tumors associated with hereditary conditions. Plain radiography is the imaging method of choice and demonstrates the typical cortical and medullary continuity of the tumor with the underlying bone. Magnetic resonance imaging is often performed to evaluate cartilage cap thickness, which correlates with malignant transformation. Other local complications include compression of adjacent neurovascular bundles, muscles, and tendons, bursitis, tendon tears, stalk fracture, and angular or rotational long bone deformities. Although the imaging features of osteochondromas are largely known, only a few papers in the literature have focused on their main complications and image-based follow-up. This paper aimed to illustrate the main complications of osteochondromas, suggest an image-based algorithm for management and follow-up and discuss differential diagnosis.
Topics: Humans; Exostoses, Multiple Hereditary; Bone Neoplasms; Osteochondroma; Magnetic Resonance Imaging; Toes
PubMed: 37778187
DOI: 10.1016/j.clinimag.2023.109989 -
The Journal of Bone and Joint Surgery.... Apr 2024To our knowledge, there have been no studies examining peroneal nerve decompression and proximal fibular osteochondroma excision exclusively in patients with multiple...
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.
PubMed: 38662808
DOI: 10.2106/JBJS.23.01398 -
International Journal of Surgery Case... May 2024Multiple Hereditary Exostoses is a rare autosomal dominant bone disorder that predominantly affects males at an incidence of (1:50,000 to 1:100,000) in Western...
INTRODUCTION AND IMPORTANCE
Multiple Hereditary Exostoses is a rare autosomal dominant bone disorder that predominantly affects males at an incidence of (1:50,000 to 1:100,000) in Western populations. The etiology is owed to mutations in the EXT gene group, specifically EXT1 and EXT2 which cause the formation of Osteochondromas. Diagnosis is typically established in childhood. Nevertheless, vascular complications are extremely rare while being potentially fatal. Therefore, timely diagnosis and treatment are vital for such patients.
CASE PRESENTATION
We present the case of a 37-year-old Middle Eastern male with Multiple Hereditary Exostoses who experienced sudden-onset left lower limb pain persisting for a month prior to admission. It was associated with coldness and paresthesia of the ipsilateral lower limb. The presurgical radiological workup uncovered a popliteal pseudoaneurysm subsequent to Multiple Hereditary Exostoses.
CLINICAL DISCUSSION
Through open surgery, the vascular perfusion was successfully restored, and a subsequent supra- to infra-geniculate popliteal artery anastomosis via saphenous vein grafting was done. Furthermore, the Osteochondroma was utterly resected to limit recurrence of another vascular injury. The following histopathological analysis confirmed the diagnosis of an Osteochondroma as a result of MHE.
CONCLUSION
Multiple Hereditary Exostoses is a rare occurrence leading to pseudoaneurysms. This event underscores the need for further documentation to aid in establishing a prompt diagnosis and carrying out suitable interventions. Considering this pathology in a multidisciplinary approach ensures proper treatment. Following a comprehensive literature review, our case stands as the first case in the published literature from our country which emphasizes its value and rarity.
PubMed: 38626641
DOI: 10.1016/j.ijscr.2024.109633 -
Clinical Oral Investigations Dec 2023To investigate factors influencing postoperative bleeding occurrence after dental extraction in older patients receiving anticoagulation therapy.
OBJECTIVES
To investigate factors influencing postoperative bleeding occurrence after dental extraction in older patients receiving anticoagulation therapy.
MATERIALS AND METHODS
This retrospective study included patients aged ≥ 65 years receiving one of the following anticoagulants: apixaban, edoxaban, rivaroxaban, and warfarin. Patients who underwent one to multiple tooth extractions in the geriatric dentistry clinic at Tokyo Medical and Dental University Hospital between August 1, 2016, and November 30, 2020, were included. The outcome variable was postoperative bleeding occurrence. Logistic regression analysis was performed with the following ten factors as explanatory variables: age, sex, maximum systolic blood pressure during the extraction, type of local anesthesia, vertical incision, osteotomy, usage of surgical splints, the mesiodistal width of the extracted tooth on a radiograph, use of antiplatelet agents, and history of diabetes requiring medication.
RESULTS
Among 395 participants (mean age, 82.3 ± 6.5 years) included in this study, 75 patients experienced postoperative bleeding after tooth extraction. Logistic regression analysis revealed that the odds ratios for the vertical incision (18.400, p < 0.001), osteotomy (3.630, p = 0.00558), usage of surgical splints (1.860, p = 0.0395), and the mesiodistal width of the extracted tooth on a radiograph (1.060, p = 0.0261) were statistically significant.
CONCLUSIONS
For dental extraction in older patients receiving anticoagulants, postoperative bleeding is more likely to occur in patients with vertical incision, osteotomy, and posterior or multiple tooth extractions.
CLINICAL RELEVANCE
Dentists should consider suturing and adjunctive hemostatic procedures for patients undergoing vertical incision, osteotomy, and multiple tooth extractions while receiving anticoagulation therapy to minimize the risk of postoperative bleeding.
Topics: Humans; Aged; Aged, 80 and over; Retrospective Studies; Postoperative Hemorrhage; Dental Care; Warfarin; Anticoagulants; Exostoses
PubMed: 38147161
DOI: 10.1007/s00784-023-05424-1 -
Orphanet Journal of Rare Diseases Feb 2024Multiple osteochondromas is genetic disorder characterized by the formation of multiple benign cartilage-capped bone tumors, named osteochondromas, during skeletal...
BACKGROUND
Multiple osteochondromas is genetic disorder characterized by the formation of multiple benign cartilage-capped bone tumors, named osteochondromas, during skeletal development. The most feared complication is the secondary peripheral chondrosarcoma, a malignant cartilaginous neoplasm that arises from the chondroid cap of pre-existent osteochondromas. We conducted a retrospective cohort study on patients diagnosed and followed up from 1960 to 2019 to describe the clinical and pathological features of individuals affected by peripheral chondrosarcoma in multiple osteochondromas, to evaluate follow up information and individual outcome and to compare the results with literature. Data, including age, gender, site, histological grade, cartilage cap thickness, surgical treatments, surgical margins, genotype mutational status as well as treatment details were captured from the hospital electronic health records and from Registry of Multiple Osteochondromas. In addition, a complete histological review of all hematoxylin and eosin (H&E)-stained sections has been performed by expert pathologists.
RESULTS
One hundred five of the screened cases were included in the present study. The age at diagnosis of SPC ranges from 13 to 63, with median age at diagnosis of 34 years. The site most frequently affected by malignant degeneration was the pelvis (46 patients, 44%) with higher incidence in male patients (32 males vs.14 females). The second one was lower limbs (including femur, fibula, or tibia), identified in 35 patients. Histological information - available for 103 patients - showed: 59 patients with grade 1; 40 patients had a grade 2 and 4 patients had a grade 3. The most common surgical treatment was the complete resection, followed by debulking, amputation and partial resection. Most of cases did not have recurrence of the disease. Outcome in disease-free survival highlights that a worse course of the disease was associated with histological grade 2 or 3, and partial resection surgery. In most of analyzed cases (94%) a pathogenic variant was identified.
CONCLUSIONS
In conclusion, the present study gives an overview of the secondary peripheral chondrosarcomas, confirming that this disease represents an impacting complication for multiple osteochondromas patients and suggests that malignant transformation can occur also in younger patient, in a not irrelevant number of cases.
Topics: Female; Humans; Male; Adult; Exostoses, Multiple Hereditary; Retrospective Studies; Chondrosarcoma; Osteochondroma; Disease-Free Survival; Bone Neoplasms
PubMed: 38351015
DOI: 10.1186/s13023-023-03006-8 -
Indian Journal of Orthopaedics Nov 2023Hereditary multiple exostoses (HME) Masada IIB has traditionally been treated by gradual ulnar lengthening with questionable efficacy in reducing the dislocated head....
INTRODUCTION
Hereditary multiple exostoses (HME) Masada IIB has traditionally been treated by gradual ulnar lengthening with questionable efficacy in reducing the dislocated head. One-bone forearm (OBF) has been used as a reconstructive procedure in forearm deformities with very scarce literature for HME. The study aims to report short-term results of OBF as a definitive procedure for severe forearm deformities in Masada IIB patients with respect to clinical and radiological parameters.
MATERIALS AND METHODS
Four patients with HME Masada IIb were included in this retrospective study. All patients complained of forearm and wrist deformity with an abnormal bony protrusion restricting elbow motion. Indications for OBF were ulnar shortening > 3 cm, dysplastic proximal radius with convex radial head and restricted prono-supination. All patients were examined pre-operatively and post-operatively clinically and radiographically using the Peterson's outcome score.
RESULTS
The average age was 13 years (12-14 years). Pre-operative ulnar shortening, carpal slip percentage, and radial articular angle was 3.4 cm, 79.5%, and 47.5°, respectively. All radial heads were dislocated with convex articular surface restricting elbow extension and forearm prono-supination. At the latest follow-up, the mean elbow flexion was 110° with forearm in 10° supination. The mean carpal slip percentage, radial articular angle, and Peterson functional grade was 15%, 22.5°, and 8 points, respectively. The mean follow-up period was 30.25 months with no recurrence.
CONCLUSION
We recommend one bone forearm as a definitive procedure in HME Masada IIB patients with severe forearm deformities with ulnar shortening > 3 cm and dysplastic proximal radius with a dislocated radial head, for faster return to function.
PubMed: 37881291
DOI: 10.1007/s43465-023-00968-6 -
Orthopaedics & Traumatology, Surgery &... Sep 2023Radial head dislocation in patients with multiple hereditary exostosis (MHE) can lead to functional deficit. We investigated whether the location of the exostosis and...
INTRODUCTION
Radial head dislocation in patients with multiple hereditary exostosis (MHE) can lead to functional deficit. We investigated whether the location of the exostosis and certain radiological criteria predict risk of radial head dislocation/subluxation.
HYPOTHESIS
We hypothesized that the radiological criteria differentiate between patients who need closer follow-up of the forearm and others for whom multiple radiographs are superfluous.
PATIENTS AND METHODS
We retrospectively reviewed the demographics of patients with MHE in our hospital, and radiographic measurements were made on forearm radiographs: radial length, ulnar length, ulnar variance, radial articular angle, and radial bowing.
RESULTS
Forty-nine forearms were analyzed in 30 patients. Mean age was 9.5 years at first evaluation and 11.8 years at last evaluation. Radial head dislocation or subluxation was found in 6 forearms (12%). Risk factors comprised isolated exostosis in the distal portion of the ulna or exostosis in the distal part of both the ulna and radius, radial or ulnar shortening>4.6cm, radial bowing>8.1%, radial articular angle>35°, and≥3 exostoses in the forearm.
DISCUSSION
In patients with MHE with risk factors for radial head dislocation, close follow- up with regular radiography is indicated and early surgery should be performed before the radial head dislocates.
LEVEL OF EVIDENCE
IV; retrospective study.
Topics: Humans; Child; Forearm; Retrospective Studies; Ulna; Radius; Joint Dislocations; Exostoses, Multiple Hereditary; Osteochondroma; Bone Neoplasms
PubMed: 36270444
DOI: 10.1016/j.otsr.2022.103445 -
Journal of Pediatric OrthopedicsGenu valgum is a well-known feature of multiple hereditary exostoses (MHE). Though prior reports have demonstrated successful treatment with hemiepiphysiodesis, details...
BACKGROUND
Genu valgum is a well-known feature of multiple hereditary exostoses (MHE). Though prior reports have demonstrated successful treatment with hemiepiphysiodesis, details regarding the correction rate and comparison to an idiopathic population are lacking. This study aimed to detail our institution's experience with guided growth of the knee in patients with MHE and compare this to an idiopathic population.
METHODS
All pediatric patients (age 18 and younger) with MHE who underwent lower extremity hemiepiphysiodesis at a tertiary care medical center between January 2016 and December 2022 were retrospectively reviewed. Preoperative and postoperative mechanical lateral distal femoral angle (mLDFA) and medial proximal tibial angle (MPTA, the primary outcomes) were measured in addition to mechanical axis deviation (MAD) and hip-knee-ankle angle (HKA). Patients were 1:2 matched based on age, sex, and physes instrumented to a cohort with idiopathic genu valgum.
RESULTS
A total of 21 extremities in 16 patients with MHE underwent hemiepiphysiodesis of the distal femur, proximal tibia, and/or distal tibia. The mean age at surgery was 11.7±2.2 years. Mean MAD corrected from zone 1.9±0.7 to -0.3±1.5, while mLDFA corrected from 83.4±2.9 to 91.7±5.2 degrees and MPTA corrected from 95.3±3.6 to 90.5±4.0 degrees in distal femurs and proximal tibias undergoing guided growth, respectively. Three extremities (14.3%) experienced overcorrection ≥5 degrees managed with observation. There were no differences in correction rates per month for mLDFA (0.54±0.34 vs. 0.51±0.29 degrees, P =0.738) or MPTA (0.31±0.26 vs. 0.50±0.59 degrees, P =0.453) between MHE and idiopathic groups. For 11 extremities in the MHE group with open physes at hardware removal, they experienced a mean recurrence of HKA of 4.0±3.4 degrees at 19-month follow-up.
CONCLUSION
Hemiepiphysiodesis corrects lower extremity malalignment in patients with MHE at a similar rate compared with an idiopathic coronal plane deformity population. Rebound deformity of 4 degrees at 19 months after hardware removal in patients with remaining open growth plates should make surgeons conscious of the remaining growth potential when planning deformity correction.
LEVEL OF EVIDENCE
Level III.
Topics: Humans; Child; Adolescent; Genu Valgum; Exostoses, Multiple Hereditary; Retrospective Studies; Tibia; Knee Joint; Femur
PubMed: 38450657
DOI: 10.1097/BPO.0000000000002654 -
Cureus Oct 2023Multiple hereditary exostosis syndrome is a rare diagnosis with approximately 1:50000 incidence prevailing in males. The exostoses or osteochondromas are benign but have...
Multiple hereditary exostosis syndrome is a rare diagnosis with approximately 1:50000 incidence prevailing in males. The exostoses or osteochondromas are benign but have the potential for malignant transformation in 1-5%. There is a strong genetic component, with exostosis (EXT) signaling pathways being an underlying cause. They can be symptomatic, with pain and functional deficit as the main complaints. We present a case of a 17-year-old male who presented with pain and anatomical deformity in his left lower femur. Magnetic resonance imaging revealed multiple osteochondromas compressing the popliteal neurovascular bundle. Excision of the osteochondromas was performed to decompress the neurovascular bundle in a multidisciplinary approach. Histological examination demonstrated no evidence of malignancy. Currently, there is no consensus for patients diagnosed with multiple osteochondromas regarding further investigation and/or screening for malignant transformation.
PubMed: 37927696
DOI: 10.7759/cureus.46396 -
Orthopaedics & Traumatology, Surgery &... Sep 2023In patients who have hereditary multiple osteochondroma (HMO), progressive deformity of the forearm skeleton may lead to radial head dislocation. The latter is...
INTRODUCTION
In patients who have hereditary multiple osteochondroma (HMO), progressive deformity of the forearm skeleton may lead to radial head dislocation. The latter is permanent, painful and causes weakness.
HYPOTHESIS
There is a relationship between the amount of ulnar deformity and the presence of radial head dislocation in patients with HMO.
MATERIALS AND METHODS
This was a cross-sectional radiographic study comprising an analysis of anterior-posterior (AP) and lateral x-rays of 110 forearms in children having a mean age of 8 years and 4 months who were followed for HMO between 1961 and 2014. Four factors reflecting on the ulnar deformity in the coronal plane were investigated on the AP view and three factors in the sagittal plane were investigated on the lateral view to identify any relationship between ulnar deformity and radial head dislocation. The forearms were separated into two groups: with radial head dislocation (26 cases) and without radial head dislocation (84 cases).
RESULTS
Ulnar bowing, intramedullary angle of ulnar bowing, tangent ulnar angle and overall ulnar angle were significantly higher in the group of children who had a radial head dislocation (0.05 vs 0.03, p<.001; 161 vs 167, p<001; 156 vs 162, p<001; 50 vs 30, p<.001) in univariate and multivariate analyses.
DISCUSSION
Ulnar deformity, evaluated using the method described here, is more often associated with radial head dislocation than other previously published radiological parameters. This provides new insight on this phenomenon and may help to determine which factors are associated with radial head dislocation and how to prevent it.
CONCLUSION
Ulnar bowing in the context of HMO, especially when evaluated on AP radiographs, is significantly associated with radial head dislocation.
LEVEL OF EVIDENCE
III; case-control study.
Topics: Child; Humans; Radius; Exostoses, Multiple Hereditary; Case-Control Studies; Cross-Sectional Studies; Retrospective Studies; Ulna; Joint Dislocations
PubMed: 36905956
DOI: 10.1016/j.otsr.2023.103591