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Ippolito E, Farsetti P, Caterini R, Gorgolini G, Caterini A, De Maio F. Lower-limb intramedullary nailing in patients with polyostotic fibrous dysplasia who had a previous unsuccessful treatment. A report of 48 cases. J Orthop Traumatol 2023; 24:35. [PMID: 37402946 DOI: 10.1186/s10195-023-00705-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/28/2022] [Accepted: 05/01/2023] [Indexed: 07/06/2023] Open
Abstract
BACKGROUND Intramedullary nailing (IN) seems to be the best primary surgical treatment for patients with either polyostotic fibrous dysplasia or McCune-Albright syndrome (PFD/MAS) when the femur and tibia are totally affected by fibrous dysplasia (FD) and pain, fracture and deformity are likely to occur. However, other management protocols have been applied in these cases, often leading to disabling sequelae. This study sought to evaluate if IN could also have been effective as a salvage procedure to provide patients with satisfactory results, regardless of the poor results due to the improper treatment previously performed. MATERIALS AND METHODS Twenty-four retrospectively registered PFD/MAS patients with 34 femurs and 14 tibias totally affected by fibrous dysplasia had received various treatments with unsatisfactory results in other institutions. Before the IN performed in our hospital, 3 patients were wheelchair bound; 4 were fractured; 17 limped; and many used an aid for walking. Salvage IN was performed in our hospital at a mean patient age of 23.66 ± 6.06 years (range, 15-37 years). The patients were evaluated before-except for the four fractured ones-and after IN using the validated Jung scoring system, and the data were statistically analyzed. RESULTS The mean length of follow-up after IN was 9.12 ± 3.68 years (range, 4-17 years). The patients' mean Jung score significantly improved from 2.52 ± 1.74 points before IN to 6.78 ± 2.23 at follow-up (p < 0.05). Ambulation was improved in ambulatory patients and restored in wheelchair users. The complication rate was 21%. CONCLUSIONS Regardless of the high rate of complications, IN may be considered a reliable surgical procedure to salvage a failed treatment in PFD/MAS, with long-lasting satisfactory results achieved in most patients. Trial registration statement: Not applicable. LEVEL OF EVIDENCE IV.
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Affiliation(s)
- E Ippolito
- Department of Clinical Science and Traslational Medicine, Section of Orthopaedics and Traumatology, University of Rome "Tor Vergata", Viale Oxford 81, 00133, Rome, Italy.
| | - P Farsetti
- Department of Clinical Science and Traslational Medicine, Section of Orthopaedics and Traumatology, University of Rome "Tor Vergata", Viale Oxford 81, 00133, Rome, Italy
| | - R Caterini
- Department of Clinical Science and Traslational Medicine, Section of Orthopaedics and Traumatology, University of Rome "Tor Vergata", Viale Oxford 81, 00133, Rome, Italy
| | - G Gorgolini
- Department of Clinical Science and Traslational Medicine, Section of Orthopaedics and Traumatology, University of Rome "Tor Vergata", Viale Oxford 81, 00133, Rome, Italy
| | - A Caterini
- Department of Clinical Science and Traslational Medicine, Section of Orthopaedics and Traumatology, University of Rome "Tor Vergata", Viale Oxford 81, 00133, Rome, Italy
| | - F De Maio
- Department of Clinical Science and Traslational Medicine, Section of Orthopaedics and Traumatology, University of Rome "Tor Vergata", Viale Oxford 81, 00133, Rome, Italy
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Dozo MA, Abrego MO, Comba F, Zanotti G, Buttaro MA, Slullitel PA. Late Periprosthetic Femoral Stress Fracture Associated with Extra-articular Deformity in a Patient with Fibrous Dysplasia: A Case Report. JBJS Case Connect 2023; 13:01709767-202309000-00013. [PMID: 37478317 DOI: 10.2106/jbjs.cc.23.00017] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/23/2023]
Abstract
CASE In the setting of a total hip arthroplasty performed in a patient with a proximal femoral deformity, atraumatic periprosthetic femoral stress fracture may arise as a complication. We report a rare case of a late periprosthetic femoral stress fracture around a cemented stem in a patient with a history of fibrous dysplasia of the proximal femur. After a 10-year uneventful period, the patient complained about a subtle, subacute pain in his left thigh induced by exercise but not with daily axial load. Diagnosis of a nondisplaced, incomplete (i.e., only compromising the lateral femoral cortex) periprosthetic femoral stress fracture was made with plain radiographs, blood work, and bone scintigraphy. Surgical treatment consisted of a minimally invasive plate osteosynthesis bridging the femoral deformity plus percutaneous osteoperiosteal decortication. At 5-year follow-up, the patient was asymptomatic with full return to physical activity, with radiographs evidencing callus formation. CONCLUSION Stress fractures around well-fixed femoral stems, while infrequent, should be addressed in patients with a history of severe proximal femur deformity experiencing atraumatic thigh pain.
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Affiliation(s)
- Marcelo Adolfo Dozo
- Hip Surgery Unit, Institute of Orthopaedics "Carlos E. Ottolenghi", Italian Hospital of Buenos Aires, Buenos Aires, Argentina
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Gorgolini G, Caterini A, Efremov K, Marsiolo M, De Maio F, Farsetti P. Surgical correction of valgus deformities of the knee in Polyostotic Fibrous Dysplasia. Orthop Rev (Pavia) 2022; 14:38827. [PMID: 36540073 PMCID: PMC9760661 DOI: 10.52965/001c.38827] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/23/2022] Open
Abstract
Coxa vara and shepherd's crook deformity represent the most common femoral deformities in patients affected by polyostotic fibrous dysplasia (PFD) and McCune Albright syndrome (MAS). The tibia is also commonly affected, with antero-medial bowing and valgus deformity. Surgical treatment of these deformities are technically demanding and, in most cases, intramedullary nails are the preferred method to stabilize corrective osteotomies. Genu valgus may also be present, as a residual defect after the surgical correction of the aforementioned deformities. The aim of our study was to report the outcomes obtained in five patients with PFD or MAS operated on for complex deformities of the lower limbs with an associated residual genu valgum greater than 15°. It was surgically treated by distal femoral or proximal tibial osteotomy stabilized with a screw plate. The osteotomy was performed distal to the intramedullary nail in femurs, while it was performed after removing the intramedullary nail in the proximal metaphysis of the tibia. In latter cases, the intramedullary device was reinserted at least 6 months after surgery. At follow-up, the femoro-tibial angle was corrected in all cases and the patients were satisfied with the final result. Two patients showed mild limping, unrelated to the alignment of the knee. In our opinion, residual valgus deformities of the lower limb greater than 15 degrees should be corrected to improve function and cosmetic appearance of these patients. The osteotomy of the distal femur or of the proximal tibia stabilized by a screw-plate represent the treatment of choice for the correction of these deformities.
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Affiliation(s)
- Giulio Gorgolini
- Department of Clinical Science and Translational Medicine, Section of Orthopaedics and TraumatologyUniversity of Rome “Tor Vergata”, Italy
| | - Alessandro Caterini
- Department of Clinical Science and Translational Medicine, Section of Orthopaedics and TraumatologyUniversity of Rome “Tor Vergata”, Italy
| | - Kristian Efremov
- Department of Clinical Science and Translational Medicine, Section of Orthopaedics and TraumatologyUniversity of Rome “Tor Vergata”, Italy
| | - Martina Marsiolo
- Department of Clinical Science and Translational Medicine, Section of Orthopaedics and TraumatologyUniversity of Rome “Tor Vergata”, Italy
| | - Fernando De Maio
- Department of Clinical Science and Translational Medicine, Section of Orthopaedics and TraumatologyUniversity of Rome “Tor Vergata”, Italy
| | - Pasquale Farsetti
- Department of Clinical Science and Translational Medicine, Section of Orthopaedics and TraumatologyUniversity of Rome “Tor Vergata”, Italy
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Wang Y, Luo Y, Min L, Zhou Y, Wang J, Zhang Y, Lu M, Duan H, Tu C. The West China Hospital radiographic classification for fibrous dysplasia in femur and adjacent bones: A retrospective analysis of 205 patients. Orthop Surg 2022; 14:2096-2108. [PMID: 35924702 PMCID: PMC9483050 DOI: 10.1111/os.13404] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/19/2021] [Revised: 06/21/2022] [Accepted: 06/23/2022] [Indexed: 02/05/2023] Open
Abstract
Objective This study aims to investigate the reliability and clinical outcome of a newly developed classification system for patients with fibrous dysplasia (FD) of the femur and adjacent bones, optimizing its evaluation and management. Methods A total of 205 patients (121 female and 84 male) with FD in the femur and adjacent bones were included in this retrospective study. All affected femurs were measured and treated based on this classification at our institution between 2009 and 2019. Based on previous studies and extensive clinical follow‐up, we cautiously proposed the West China Hospital radiographic classification for FD in femur and adjacent bones following corresponding treatment options. There are five types with five radiographic features, including proximal femur bone loss, coxa vara, femoral shaft deformity, genu valgum, and hip arthritis. The intraobserver and interobserver reproducibility of this classification was assessed by four observers using the Cohen kappa statistic. The clinical outcome was evaluated using the criteria of Guille. Results At a median follow‐up of 60 months (range 6–120), 205 patients (median 34.8 years old, range 18–73 years old) were categorized into the following five types: Type I 31.7%, Type II 30.2%, Type III 20.5%, Type IV 10.2%, and Type V 7.3%. The mean interobserver and intraobserver kappa scores were 0.85 (range 0.77–0.89) and 0.85 (range 0.79–0.92), respectively. For clinical outcomes, there was no significant difference in the postoperative Guille score for Type I patients (mean 9.01 ± 1.22). There was a significant increase in the postoperative Guille score in Type II, III, IV, and V, compared to the preoperative values (P < 0.01). For complications, two Type III patients reported pain, and one Type III patient had mild‐to‐moderate Trendelenburg gait. One Type IV patient had a mild Trendelenburg gait. And two Type V patients still had mild limping. Conclusion This classification is reproducible and serves as a tool for evaluating and treating FD in the femur and adjacent bones. Therefore, we recommend this classification for the diagnosis and treatment of FD‐related deformities in the femur and adjacent bones.
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Affiliation(s)
- Yitian Wang
- Department of Orthopedics, Orthopedic Research Institute, West China Hospital, Sichuan University, Chengdu, P. R. China.,Bone and Joint 3D-Printing & Biomechanical Laboratory, Department of Orthopedics, West China Hospital, Sichuan University, Chengdu, Sichuan, P. R. China
| | - Yi Luo
- Department of Orthopedics, Orthopedic Research Institute, West China Hospital, Sichuan University, Chengdu, P. R. China.,Bone and Joint 3D-Printing & Biomechanical Laboratory, Department of Orthopedics, West China Hospital, Sichuan University, Chengdu, Sichuan, P. R. China
| | - Li Min
- Department of Orthopedics, Orthopedic Research Institute, West China Hospital, Sichuan University, Chengdu, P. R. China.,Bone and Joint 3D-Printing & Biomechanical Laboratory, Department of Orthopedics, West China Hospital, Sichuan University, Chengdu, Sichuan, P. R. China
| | - Yong Zhou
- Department of Orthopedics, Orthopedic Research Institute, West China Hospital, Sichuan University, Chengdu, P. R. China.,Bone and Joint 3D-Printing & Biomechanical Laboratory, Department of Orthopedics, West China Hospital, Sichuan University, Chengdu, Sichuan, P. R. China
| | - Jie Wang
- Department of Orthopedics, Orthopedic Research Institute, West China Hospital, Sichuan University, Chengdu, P. R. China.,Bone and Joint 3D-Printing & Biomechanical Laboratory, Department of Orthopedics, West China Hospital, Sichuan University, Chengdu, Sichuan, P. R. China
| | - Yuqi Zhang
- Department of Orthopedics, Orthopedic Research Institute, West China Hospital, Sichuan University, Chengdu, P. R. China.,Bone and Joint 3D-Printing & Biomechanical Laboratory, Department of Orthopedics, West China Hospital, Sichuan University, Chengdu, Sichuan, P. R. China
| | - Minxun Lu
- Department of Orthopedics, Orthopedic Research Institute, West China Hospital, Sichuan University, Chengdu, P. R. China.,Bone and Joint 3D-Printing & Biomechanical Laboratory, Department of Orthopedics, West China Hospital, Sichuan University, Chengdu, Sichuan, P. R. China
| | - Hong Duan
- Department of Orthopedics, Orthopedic Research Institute, West China Hospital, Sichuan University, Chengdu, P. R. China
| | - Chongqi Tu
- Department of Orthopedics, Orthopedic Research Institute, West China Hospital, Sichuan University, Chengdu, P. R. China.,Bone and Joint 3D-Printing & Biomechanical Laboratory, Department of Orthopedics, West China Hospital, Sichuan University, Chengdu, Sichuan, P. R. China
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Ippolito E, Farsetti P, Caterini R, Micciulli E, Gorgolini G, Ruzzini L. Intramedullary Nailing for Lower Limb Polyostotic Fibrous Dysplasia in Children: A Long-term Follow-up Study. J Pediatr Orthop 2022; 42:e492-e500. [PMID: 35200214 PMCID: PMC10810345 DOI: 10.1097/bpo.0000000000002097] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
BACKGROUND In children, intramedullary nailing (IN) has been proposed as the best treatment when the femur and tibia are totally affected by fibrous dysplasia (FD). However, in younger children IN must be repeated to maintain stabilization of the affected skeletal segment during growth. We report the long-term results in a cohort of patients in whom more than two-thirds of cases had IN repeated during growth. METHODS Twenty-nine femurs and 14 tibias totally affected by FD were treated by IN in 21 patients with polyostotic FD and McCune-Albright syndrome. Thirteen patients with 35 femoral and tibial deformities had a painful limp whereas 8 presented fractures. The patients had their first IN at a mean age of 9.26±2.68 years (range: 4 to 14 y). IN was repeated during growth in the younger patients, and all the patients underwent a mean of 2.13 femoral and 1.50 tibial IN per limb. The last IN was performed at a mean age of 16.42±1.95 years (range: 11 to 19 y). Titanium elastic nails and adult humeral nails were used in younger children, whereas adult femoral cervicodiaphyseal and interlocking tibial nails were used in older children and adolescents. At the latest follow-up, the patients were evaluated with a clinicoradiographic scale. All the data were statistically analyzed. RESULTS The mean length of follow-up from the last IN was 6.47±3.10 years (range: 3 to 14 y), and the mean age of the patients at follow-up was 22.85±3.53 years (range: 14 to 29 y) when lower limbs were fully grown in all but 1 patient. Satisfactory long-term results were obtained in about 81% of our patients, while complications occurred in 32.5% of the 43 cases. CONCLUSION Lower limb IN-that was repeated in younger children during growth-provided satisfactory long-term results in most of our patients, with fracture and deformity prevention and pain control, regardless of the high rate of complications that mainly affected the femoral cases. Missing scheduled follow-ups was the main predictor of a poor result. LEVEL OF EVIDENCE Level IV-case series.
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Affiliation(s)
- Ernesto Ippolito
- Department of Orthopaedic Surgery, University of Rome “Tor Vergata”, Viale Oxford
| | - Pasquale Farsetti
- Department of Orthopaedic Surgery, University of Rome “Tor Vergata”, Viale Oxford
| | - Roberto Caterini
- Department of Orthopaedic Surgery, University of Rome “Tor Vergata”, Viale Oxford
| | - Enrico Micciulli
- Department of Orthopaedic Surgery, Pediatric Hospital “Bambino Gesù”, Palidoro, Rome, Italy
| | - Giulio Gorgolini
- Department of Orthopaedic Surgery, University of Rome “Tor Vergata”, Viale Oxford
| | - Laura Ruzzini
- Department of Orthopaedic Surgery, Pediatric Hospital “Bambino Gesù”, Palidoro, Rome, Italy
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Gorgolini G, Caterini A, Nicotra L, De Maio F, Efremov K, Farsetti P. Surgical treatment of femoral deformities in polyostotic fibrous dysplasia and McCune-Albright syndrome: A literature review. World J Orthop 2022; 13:329-338. [PMID: 35317251 PMCID: PMC8935334 DOI: 10.5312/wjo.v13.i3.329] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/15/2021] [Revised: 10/25/2021] [Accepted: 02/16/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Surgical correction of femoral deformities in polyostotic fibrous dysplasia (PFD) or McCune-Albright syndrome (MAS), such as coxa vara or shepherd’s crook deformity, is a challenge.
AIM To evaluate the treatment of patients with femoral deformities caused by PDF or MAS treated by osteotomies and stabilized with different methods, by analyzing the most relevant studies on the topic.
METHODS A literature search was performed in Medline database (PubMed). Articles were screened for patients affected by PFD or MAS surgically managed by osteotomies and stabilized with different methods.
RESULTS The initial search produced 184 studies, with 15 fulfilling the eligibility criteria of our study. Selected articles (1987-2019) included 111 patients overall (136 femurs).
CONCLUSION Based on our results, the preferred method to stabilize corrective osteotomies is intramedullary nailing with neck cross pinning. When the deformity is limited to the proximal part of the femur, a screw or blade plate may be used, although there is a high risk of fracture below the plate. When the femur is entirely involved, a two-stage procedure may be considered.
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Affiliation(s)
- Giulio Gorgolini
- Department of Clinical Science and Translational Medicine, University of Rome Tor Vergata, Section of Orthopaedics and Traumatology, Tor Vergata Hospital, Rome 00133, Italy
| | - Alessandro Caterini
- Department of Clinical Science and Translational Medicine, University of Rome Tor Vergata, Section of Orthopaedics and Traumatology, Tor Vergata Hospital, Rome 00133, Italy
| | - Lorenzo Nicotra
- Department of Clinical Science and Translational Medicine, University of Rome Tor Vergata, Section of Orthopaedics and Traumatology, Tor Vergata Hospital, Rome 00133, Italy
| | - Fernando De Maio
- Department of Clinical Science and Translational Medicine, University of Rome Tor Vergata, Section of Orthopaedics and Traumatology, Tor Vergata Hospital, Rome 00133, Italy
| | - Kristian Efremov
- Department of Clinical Science and Translational Medicine, University of Rome Tor Vergata, Section of Orthopaedics and Traumatology, Tor Vergata Hospital, Rome 00133, Italy
| | - Pasquale Farsetti
- Department of Clinical Science and Translational Medicine, University of Rome Tor Vergata, Section of Orthopaedics and Traumatology, Tor Vergata Hospital, Rome 00133, Italy
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Jud L, Vlachopoulos L, Grob K. Correction of complex three-dimensional deformities at the proximal femur using indirect reduction with angle blade plate and patient-specific instruments: a technical note. J Orthop Surg Res 2021; 16:427. [PMID: 34217344 PMCID: PMC8254345 DOI: 10.1186/s13018-021-02579-z] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/27/2021] [Accepted: 06/24/2021] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Corrective osteotomies for complex proximal femoral deformities can be challenging; wherefore, subsidies in preoperative planning and during surgical procedures are considered helpful. Three-dimensional (3D) planning and patient-specific instruments (PSI) are already established in different orthopedic procedures. This study gives an overview on this technique at the proximal femur and proposes a new indirect reduction technique using an angle blade plate. METHODS Using computed tomography (CT) data, 3D models are generated serving for the preoperative 3D planning. Different guides are used for registration of the planning to the intraoperative situation and to perform the desired osteotomies with the following reduction task. A new valuable tool to perform the correction is the use of a combined osteotomy and implant-positioning guide, with indirect deformity reduction over an angle blade plate. RESULTS An overview of the advantages of 3D planning and the use of PSI in complex corrective osteotomies at the proximal femur is provided. Furthermore, a new technique with indirect deformity reduction over an angle blade plate is introduced. CONCLUSION Using 3D planning and PSI for complex corrective osteotomies at the proximal femur can be a useful tool in understanding the individual deformity and performing the aimed deformity reduction. The indirect reduction over the implant is a simple and valuable tool in achieving the desired correction, and concurrently, surgical exposure can be limited to a subvastus approach.
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Affiliation(s)
- Lukas Jud
- Department of Orthopaedic Surgery, Kantonsspital St. Gallen, Rorschacherstrasse 95, 9007 St. Gallen, Switzerland
- Department of Orthopedics, Balgrist University Hospital, University of Zurich, Forchstrasse 340, 8008 Zürich, Switzerland
| | - Lazaros Vlachopoulos
- Department of Orthopedics, Balgrist University Hospital, University of Zurich, Forchstrasse 340, 8008 Zürich, Switzerland
| | - Karl Grob
- Department of Orthopaedic Surgery, Kantonsspital St. Gallen, Rorschacherstrasse 95, 9007 St. Gallen, Switzerland
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Bocchi MB, Cianni L, Perna A, Vitiello R, Greco T, Maccauro G, Perisano C. A rare case of Bacillus megaterium soft tissues infection. ACTA BIO-MEDICA : ATENEI PARMENSIS 2020; 91:e2020013. [PMID: 33559642 PMCID: PMC7944702 DOI: 10.23750/abm.v91i14-s.10849] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Download PDF] [Subscribe] [Scholar Register] [Received: 10/24/2020] [Accepted: 11/23/2020] [Indexed: 12/20/2022]
Abstract
BACKGROUND AND AIM OF THE WORK To report the history and clinical presentation of a 60-year-old male who developed a rare soft tissue infection of the right leg caused by Bacillus megaterium and to perform a Literature review focusing on clinical manifestations and diagnostic difficulties of the aforementioned bacterium. METHODS Medical history and clinical presentation suggested the infectious etiology, which led to the surgical procedure of fistulectomy and to further histological and microbiological investigations with bacterial cultures. RESULTS The histological report tested negative for osteomyelitis. Bacterial cultures revealed Bacillus megaterium, which was sensitive to all antibiotics against which it was tested. The oral antibiotic therapy was set for 12 days with benefits. The patient has always been apyretic, inflammation indexes and white cells count have been within normal limits. Conclusions: This was a rare case of soft tissue infection caused by Bacillus megaterium known to be a "non-pathogenic" bacterium. The infection was likely acquired by the penetration through the injury wound. The combination of surgical and antibiotic therapy lead to complete skin healing and infection resolution at the 6-months follow up.
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Affiliation(s)
| | - Luigi Cianni
- Fondazione Policlinico Universitario Agostino Gemelli IRCSS, Rome.
| | - Andrea Perna
- Fondazione Policlinico Universitario Agostino Gemelli IRCSS, Rome.
| | | | - Tommaso Greco
- Fondazione Policlinico Universitario Agostino Gemelli IRCSS, Rome.
| | - Giulio Maccauro
- Fondazione Policlinico Universitario Agostino Gemelli IRCSS, Rome.
| | - Carlo Perisano
- Fondazione Policlinico Universitario Agostino Gemelli IRCSS, Rome.
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Surgical treatment for shepherd’s crook deformity in fibrous dysplasia: THERE IS NO BEST, ONLY BETTER. INTERNATIONAL ORTHOPAEDICS 2018; 43:719-726. [DOI: 10.1007/s00264-018-4074-9] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 12/21/2017] [Accepted: 07/24/2018] [Indexed: 02/07/2023]
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10
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Individualized approach to the surgical management of fibrous dysplasia of the proximal femur. Orphanet J Rare Dis 2018; 13:72. [PMID: 29720212 PMCID: PMC5932767 DOI: 10.1186/s13023-018-0805-7] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/09/2017] [Accepted: 04/10/2018] [Indexed: 02/07/2023] Open
Abstract
BACKGROUND Fibrous dysplasia of the proximal femur presents with heterogeneous clinical manifestations dictating different surgical approaches. However, to date there are no clear recommendations to guide the choice of surgical approach and no general guidelines for the optimal orthopedic management of these lesions. The objective of this study was to evaluate treatment outcomes of angled blade plates and intramedullary nails, using as outcome indicators revision-free survival, pain, function and femoral neck-shaft-angle. Based on a review of published literature and our study findings, we propose a treatment algorithm, taking into account different factors, which may play a role in the selection of one surgical approach over another. METHODS Data were evaluated in thirty-two patients (18 male) from a combined cohort from the Netherlands and Austria, who had a surgical intervention using an angled blade plate (n = 27) or an intramedullary nail (n = 5) between 1985 and 2015, and who had a minimal follow-up of one year. The primary outcome was success of the procedure according to the revised Henderson classification. Secondary outcomes, which were assessed at one year and at the end of follow-up included: function (as measured by walking ability), pain and change in femoral neck-shaft angle over time. RESULTS Analysis of data showed that revision-free survival was 72% after a median follow-up of 4.1 years. Revision was necessary in two patients for structural failure due to a fracture distal to an angled blade plate and in 7 patients due to angled blade plate-induced iliotibial tract pain. At the end of follow-up 91% of all patients had good walking ability and 91% were pain free. There was no significant postoperative change in femoral neck shaft angle. CONCLUSION Our data show that fibrous dysplasia of the proximal femur can be adequately and safely treated with angled blade plates or intramedullary nails, providing these are used according to specific characteristics of the individual patient. Based on published literature and our own experience, we propose an individualized, patient-tailored approach for the surgical management of fibrous dysplasia of the proximal femur.
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Hefti F, Donnan L, Krieg AH. Treatment of shepherd's crook deformity in patients with polyostotic fibrous dysplasia using a new type of custom made retrograde intramedullary nail: a technical note. J Child Orthop 2017; 11:64-70. [PMID: 28439311 PMCID: PMC5382339 DOI: 10.1302/1863-2548.11.170002] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
AIMS The severe form of coxa vara, the 'shepherd's crook deformity', is always a consequence of a locally extensive form of polyostotic fibrous dysplasia (or McCune-Albright syndrome). Treatment of this deformity is a challenge. The soft bone does not tolerate any implant that depends on the stability of the cortical bone (like plates or external fixators). Intramedullary nails are the most appropriate implants for stabilisation, but if they are inserted from the greater trochanter, they cannot correct the varus deformity enough. PATIENTS AND METHODS We have developed a special intramedullary nail that can be inserted from the osteotomy site and can be driven retrograde into the femoral neck in an appropriate valgus position. We have operated 15 legs in 13 patients. The average age at surgery was 14 years and 5 months (6 to 28.9). In all, 11 femora had been operated before (unsuccessfully) with various implants. RESULTS The average follow-up was 54.2 months (7 to 132). The average correction of the neck/(distal) shaft angle was 57.5° (10° to 80°) ( = 72.8%). While pre-operatively none of the patients was able to walk without aid, at follow-up only one patient was unable to walk, three used the aid of crutches because of tibial lesions and one patient had an increased external rotation of the leg. At follow-up, most patients were free of pain. One implant broke and had to be replaced. CONCLUSION This new operative method offers the possibility of efficient correction and stabilisation of this severe and difficult deformation.
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Affiliation(s)
- F. Hefti
- Children’s University Hospital Basel (UKBB), Spitalstrasse 33, 4056 Basel, Switzerland,Correspondence should be sent to: Professor F. Hefti, Children’s University Hospital Basel (UKBB), Spitalstrasse 33, 4056 Basel, Switzerland.
| | - L. Donnan
- Department of Paediatric Orthopaedics, The Royal Children’s Hospital, Victoria, Australia
| | - A. H. Krieg
- Department of Paediatric Orthopaedics, The Royal Children’s Hospital, Victoria, Australia
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Hamdy RC, Bernstein M, Fragomen AT, Rozbruch SR. What's New in Limb Lengthening and Deformity Correction. J Bone Joint Surg Am 2016; 98:1408-15. [PMID: 27535447 DOI: 10.2106/jbjs.16.00460] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/01/2023]
Affiliation(s)
- Reggie C Hamdy
- Shriners Hospitals for Children-Canada, Montréal, Québec, Canada
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Ippolito E, Farsetti P, Benedetti Valentini M, Fichera A. Two-Stage Surgical Treatment of Complex Femoral Deformities with Severe Coxa Vara in Polyostotic Fibrous Dysplasia. JBJS Essent Surg Tech 2016; 6:e14. [PMID: 30237924 DOI: 10.2106/jbjs.st.15.00050] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/14/2022] Open
Abstract
Introduction For complex femoral deformities in patients affected by polyostotic fibrous dysplasia and coxa vara associated with shepherd's crook deformity of the femoral shaft, the correct treatment is stabilization with an interlocking cervicodiaphyseal nail after correction of all of the femoral deformities. Indications & Contraindications Step 1 The First-Stage Procedure—Intertrochanteric Valgus Osteotomy and Fixation with a Hip Blade-Plate Preoperative Planning Evaluate the patient radiographically and select the hardware. Step 2 Patient Preparation Position the patient on the traction table. Step 3 Preparation of the Lateral Aspect of the Proximal Part of the Femur and Marking the Position for Both the Osteotomy and the Hip Blade-Plate Expose the lateral aspect of the proximal part of the femur and mark the position of both the blade-plate and the osteotomy. Step 4 Blade-Plate Positioning Osteotomy and Osteotomy Fixation Prepare the slot for the blade, perform the osteotomy, and correct the valgus alignment of the proximal part of the femur. Step 5 Postoperative Management Check postoperative bleeding, provide blood transfusions if needed, and have the patient resume walking. Step 6 The Second-Stage Procedure—Hip Plate Removal Osteotomy of the Femoral Shaft to Correct the Shepherd’s Crook Deformity and Stabilization with a Cervicodiaphyseal Nail Preoperative Planning Study the patient radiographically, simulate the osteotomies, perform another assessment of lower-limb length, and select the hardware. Step 7 Patient Preparation Plate Removal and Drilling of Kirschner Wires to Mark the Site of the Planned Osteotomies Position the patient on the traction table and expose the lateral aspect of the femur from the tip of the greater trochanter to the level of the most distal planned osteotomy. Step 8 Perform All of the Osteotomies and Drill a Medullary Canal in the Osteotomy Fragments All of the osteotomies are performed, and a medullary canal is drilled in each femoral fragment. Step 9 Nail and Spiral Blade Insertion Stabilization of Osteotomies and Closure Introduce the nail into the osteotomy fragments and insert the spiral blade and distal locking screws. Step 10 Postoperative Management Check postoperative bleeding, which is usually marked; administer blood transfusions as needed; and have the patient resume walking. Results We performed this surgery on 13 femora in 11 patients. Pitfalls & Challenges
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Affiliation(s)
- Ernesto Ippolito
- Department of Orthopaedic Surgery, University of Rome "Tor Vergata," Rome, Italy
| | - Pasquale Farsetti
- Department of Orthopaedic Surgery, University of Rome "Tor Vergata," Rome, Italy
| | | | - Alessandro Fichera
- Department of Orthopaedic Surgery, University of Rome "Tor Vergata," Rome, Italy
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Zhang X, Chen C, Duan H, Tu C. Radiographic classification and treatment of fibrous dysplasia of the proximal femur: 227 femurs with a mean follow-up of 6 years. J Orthop Surg Res 2015; 10:171. [PMID: 26567848 PMCID: PMC4645476 DOI: 10.1186/s13018-015-0313-6] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/08/2015] [Accepted: 10/30/2015] [Indexed: 02/07/2023] Open
Abstract
BACKGROUND Research into the optimal treatment of fibrous dysplasia has been limited by the lack of an established classification system for the disease. The purposes of this study were to develop a radiographic classification for fibrous dysplasia of the proximal femur and to test this classification's intra- and interobserver reliability as well as the effectiveness of our treatments. METHODS We retrospectively reviewed radiographs and computed tomography (CT) of 227 femurs from 206 patients with fibrous dysplasia. The radiographs were evaluated in the coronal plane for neck-shaft angle, varus deformity in the proximal femoral shaft, and distal juxtaarticular valgus deformity. CT was evaluated in the axial plane for destruction of cortex. Reduction of bone strength was defined as the thickness of the remaining cortex less than 50 % of the original on axial CT. Two senior orthopedists evaluated each radiograph and CT twice at 8-week intervals. Intra- and interobserver reliability testing was performed using the kappa statistic. Treatments were assessed through mid-term follow-up. RESULTS The 227 femurs were classified into five reproducible types: type 1 (33 %), normal bone strength without angular deformity; type 2 (30 %), decreased bone strength without angular deformity; type 3 (12 %), isolated coxa vara with neck-shaft angle <120°; type 4 (11 %), isolated varus deformity in the proximal femoral shaft; and type 5 (14 %), coxa vara with varus deformity in the proximal femoral shaft. Intra- and interobserver kappa values were excellent, ranging from 0.85 to 0.88. Good clinical outcomes were achieved. CONCLUSIONS This radiographic classification of fibrous dysplasia is reproducible and useful for describing and assessing this disease. The treatments based on this classification were effective.
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Affiliation(s)
- Xuelei Zhang
- Department of Orthopedics, West China Hospital, Sichuan University, No. 37 Guo Xue Lane, Wuhou District, 610041, Chengdu, Sichuan, China.
| | - Chunyu Chen
- Department of Orthopedics, West China Hospital, Sichuan University, No. 37 Guo Xue Lane, Wuhou District, 610041, Chengdu, Sichuan, China.
| | - Hong Duan
- Department of Orthopedics, West China Hospital, Sichuan University, No. 37 Guo Xue Lane, Wuhou District, 610041, Chengdu, Sichuan, China.
| | - Chongqi Tu
- Department of Orthopedics, West China Hospital, Sichuan University, No. 37 Guo Xue Lane, Wuhou District, 610041, Chengdu, Sichuan, China.
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Internal fixation after fracture or osteotomy of the femur in young children with polyostotic fibrous dysplasia. J Pediatr Orthop B 2015; 24:291-5. [PMID: 25932827 DOI: 10.1097/bpb.0000000000000192] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Children from 4 to 7 years of age with polyostotic fibrous dysplasia (PFD) may need internal fixation of the femur for either fracture or osteotomy. At that age, the small size of the femur allows only the use of small intramedullary nails. However, titanium elastic nails and rigid intramedullary pediatric interlocking femoral nails - good for fracture or osteotomy fixation in the normal femur - are not indicated in PFD. From 2009 to 2011, we treated eight cases of PFD femoral fracture and deformity by internal fixation with a custom-modified adult humeral nail to which a spiral blade was connected. The 7-mm thick nail fit properly into the small femoral shaft and the spiral blade conferred ideal mechanical support to the femoral neck. Three cases had replacement of the humeral nail with a more appropriate adult femoral nail 3 years after the index procedure when the femur had reached an adequate size. In the maximum follow-up period of 4 years, few complications were observed.
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