Background
Methods
Patient selection
Treatment protocol
Assessment of outcomes of surgical interventions
Statistical analysis
Results
A. Combined cohort study
Patient characteristics
LUMC | MUG | Total | |
---|---|---|---|
N
| 17 | 15 | 32 |
Male:Female | 9:8 | 9:6 | 18:14 |
Median age at diagnosis (years (range)) | 9 (3–42) | 23 (0–51) | 12 (0–51) |
Type of fibrous dysplasia | |||
Monostotic | 3 | 12 | 15 |
Polyostotic | 10 | 2 | 12 |
McCune-Albright | 4 | 1 | 5 |
Type of surgery | |||
Angled Blade Plate | 16 | 11 | 28 |
Intramedullary nail | 1 | 4 | 5 |
Median age at surgery (years (range)) | 19 (11–67) | 23 (6–51) | 20 (6–67) |
Preoperative fracture | 77% | 13% | 47% |
Characteristics of surgery | |||
Osteotomy | 35% | 13% | 25% |
Custom made | 53% | 0% | 28% |
Additional cancellous bone grafting | 18% | 53% | 34% |
Additional cortical strut grafting | 53% | 0% | 28% |
Median follow up after surgery (years) | 4.1 (1–31) | 4.7 (1–18) | 4.1 (1–31) |
Patient ID | Gender/Age at Surgery | Type of fibrous dysplasiaa | Prior surgery of the proximal femur | Indication for Surgery | Type of implantb | Osteotomy | Cancellous bone grafting | Cortical bone grafting | Follow-up in years | Failure |
---|---|---|---|---|---|---|---|---|---|---|
1 | M/16 | MAS | TEN Nails | Deformity | Custom made ABP | Yes | No | No | 1,4 | No |
2 | M/17 | MAS | TEN Nails | Fracture | Custom made ABP | No | No | Yes | 1,3 | No |
3 | F/67 | PFD | Fibular graft | Fracture | Custom made ABP | No | No | No | 1,8 | No |
4 | F/58 | MAS | – | Fracture | Custom made ABP | No | No | Yes | 2,0 | No |
5 | M/12 | PFD | Fibular graft, TEN Nails, external fixture | Fracture | Custom made ABP | No | No | No | 4,1 | No |
6 | M/29 | PFD | Fibular graft (2×) | Pain | Custom made ABP | No | No | Yes | 3,8 | No |
7 | M/20 | PFD | CBG, Plate osteosynthesis | Deformity | ABP | Yes | No | No | 6,7 | Yes |
8 | F/45 | PFD | Fibular graft | Pain | Custom made ABP | No | Yes | No | 4,1 | No |
9 | F/19 | MFD | Fibular graft | Fracture | ABP | No | No | No | 4,7 | No |
10 | F/18 | PFD | Fibular graft (3×) | Deformity | ABP | Yes | Yes | Yes | 9,4 | No |
11 | M/16 | MFD | – | Deformity | ABP | Yes | No | Yes | 14,8 | No |
12 | M/14 | PFD | – | Deformity | ABP | Yes | No | Yes | 17,3 | Yes |
13 | M/11 | PFD | – | Deformity | ABP | Yes | No | Yes | 31,1 | No |
14 | F/26 | MFD | – | Fracture | ABP | No | Yes | Yes | 1,8 | No |
15 | F/40 | MAS | Plate osteosynthesis, Fibular graft | Fracture | Custom made ABP | No | No | Yes | 4,3 | No |
16 | M/14 | PFD | – | Fracture | ABP | No | No | No | 1,0 | No |
17 | F/30 | PFD | Fibular graft | Pain | Custom made IMN | No | No | No | 1,1 | No |
18 | M/16 | MFD | TEN Nails | Impending fracture | IMN | No | No | No | 1,0 | No |
19 | F/15 | MFD | TEN Nails | Deformity | IMN | Yes | Yes | No | 1,1 | No |
20 | M/15 | MAS | – | Deformity | IMN | Yes | No | No | 1,4 | No |
21 | M/51 | MFD | – | Pain | ABP | No | No | No | 7,4 | No |
22 | F/21 | MFD | – | Pain | ABP | No | Yes | No | 7,8 | Removal (irritation) |
23 | M/43 | MFD | – | Impending fracture | ABP | No | Yes | No | 3,6 | Removal (irritation) |
24 | F/50 | MFD | – | Impending fracture | ABP | No | No | No | 4,0 | Removal (irritation) |
25 | M/33 | MFD | – | Pain | ABP | No | No | No | 2,2 | Removal (irritation) |
26 | F/29 | MFD | – | Impending fracture | ABP | No | No | No | 10,1 | No |
27 | M23 | PFD | – | Pain | ABP | No | Yes | No | 15,9 | No |
28 | M23 | MFD | – | Pain | ABP | No | Yes | No | 3,3 | No |
29 | M14 | MFD | – | Fracture | ABP | No | Yes | No | 9,3 | Removal (irritation) |
30 | F/46 | MFD | – | Impending fracture | ABP | No | Yes | No | 4,7 | Removal (irritation) |
31 | F/6 | MFD | – | Impending fracture | ABP | No | Yes | No | 17,7 | Removal (irritation) |
32 | M/13 | PFD | Nancy Nails | Impending fracture | IMN | No | No | No | 16,4 | No |
Revision-free survival
Pain symptoms and functional outcome
Femoral neck shaft angle
B. Literature review of surgical procedures used in fibrous dysplasia of the proximal femur
Curettage and bone grafting
Type of Surgery | Author/Year |
N
| Type of Surgery | Mean Follow-up | Failure | Outcome |
---|---|---|---|---|---|---|
Grafts | Harris et al. (1962) [10] | 10 | Cancellous Autograft | Unknown | 5/10 | Poor in 50% |
Nakashima et al. (1984) [21] | 8 | Autograft (unknown origin) | Unknown | 2/8 | Poor in 25% | |
Enneking er al. (1986) [24] | 15 | Cortical Autograft | 6 years | 2/15 | Poor in 2out of 15 (revision surgery) | |
Stephenson et al. (1987) [20] | 18 | Cancellous Autograft | 10.4 years | 25/31 | Poor in 81% | |
Guille et al. (1998) [19] | 22 | Cancellous Autograft | 15 years | 22/22 | Resorption of graft in 100% | |
Ippolito et al. (2003) [11] | 5 | Cancellous Autograft* | Unknown | 3/5 | Poor in 60% (revision surgery) | |
George et al. (2008) [25] | 8 | Cortical Autografts | 4.1 years | 1/8 | Poor in 12.5% (recurrence). | |
Tong et al. (2013) [27] | 13 | Cancellous Autograft with internal fixation | 12–32 months | 0/13 | No patients required revision surgery | |
Kushare et al. (2014) [13] | 8 | Various Grafts | 3 years | Unknown | Unclear | |
Nishida et al. (2015) [26] | 8 | Cortical Autograft with compression hip screw | 75 months | 0/8 | No patient had poor outcome | |
Leet et al. (2016) [23] | 46 | Various Grafts | 19.6 years | 39/52 | KM-survival: 50% survival at 14.5 years | |
Majoor et al. (2016) [17] | 28 | Cortical Allograft | 13 years | 13/28 | Good outcome in patients without a preoperative fracture and adequate proximal anchoring | |
Intramedullary Nail | Harris et al. (1962) [10] | 3 | Single intramedullary rod | Unknown | 1/3 | The only patient with fibrous dysplasia of the collum developed a severe varus deformity |
Freeman et al. (1987) [29] | 6 | Multiple osteotomies with a Zickel Nail | 34.5 months | 2/6 | Two patients needed revision surgery. | |
Keijser et al. (2001) [22] | 5 | Intramedullary nails, additional multiple osteotomies in one patient | 19.4 years | 3/5 | Three patients needed at least one revision surgery after the first IMN. | |
O’Sullivan et al. (2002) [39] | 10 | Bilateral osteotomies and Sheffield rods | 18 months | 3/10 | Three femurs needed revision surgery. 4/5 patients had a bad functional outcome due to severe coxa vara. | |
Ippolito et al. (2003) [11] | 19 | Interlocking cephalomedullary nails | Unknown | 0/19 | All patients had a good outcome with no worsening of deformities | |
Jung et al. (2006) [30] | 7 | Multiple osteotomies with intramedullary nails | 30 months | 0/7 | No patients needed a revision surgery and good functional outcome in all patients | |
Yang et al. (2010) [14] | 14 | Valgus osteotomy with intramedullary nails | 75.3 months | 0/14 | No patient needed revision surgery | |
Zhang et al. (2012) [32] | 28 | IMN, additional osteotomy in 8 patients | 50 months | 0/28 | No patients needed revision surgery. Good functional outcome in the majority | |
Kushare et al. (2014) [13] | 16 | Intramedullary nails | 3 years | 1/16 | One patient required further surgery and 5 had pain at last follow-up | |
Ippolito et al. (2015) [16] | 11 | Two stage coxa vara correction and definitive fixation with an interlocking nail | 4.7 years | 4/11 | Four patients had complications after the first surgery and another four needed further surgery after the second implant. | |
Benedetti Valentini et al. (2015) [15] | 8 | Customized adult humeral nail in children (4–7 years) | 2.9 years | 3/8 | Three patients required revision surgery as an adult. One patient required distal screw removal and acquired nail breakage | |
Present study | 5 | Intramedullary nails, one of which was customized | 4.1 years | 0/5 | All patients had a good outcome with no worsening of deformities | |
Angled Blade Plate | Ippolito et al. (2003) [11] | 2 | Angled blade plates after valgus osteotomy | 4.5 years | 1/2 | One failed due to cutting out of the plate. The other ABP had a good outcome. |
Leet et al. (2016) [23] | 2 | Angled Blade Plates | Unknown | Unknown | Outcome of ABP not described | |
Ippolito et al. (2015) [16] | 8 | Angled Blade Plates | Unknown | 1/8 | One patient had screw loosening with lateralization of the plate | |
Present study | 28 | Angled Blade Plates, 8 of which were customized | 4.1 years | 2/28 | Two failures, in 7 patients ABP removed due to complaints of the iliotibial tract | |
Dynamic/Compression Hip Screw | Li et al. (2013) [34] | 21 | Valgus osteotomy with DHS fixation | 19–128 months | 2/21 | One patient revision surgery with an intramedullary nail after a fracture and one had a loose lag screw. |
Tong et al. (2013) [27] | 2 | Valgus osteotomy with DHS fixation | 12–32 months | 0/2 | No patient needed revision surgery | |
Nishida et al. (2015) [26] | 8 | Cortical Autograft with compression hip screw | 75 months | 0/8 | No patient needed revision surgery |