119 C OPYRIGHT Ó 2015
BY
T HE J OURNAL
OF
B ONE
AND J OINT
S URGERY, I NCORPORATED
Two-Stage Surgical Treatment of Complex Femoral Deformities with Severe Coxa Vara in Polyostotic Fibrous Dysplasia Ernesto Ippolito, MD, Pasquale Farsetti, MD, Matteo Benedetti Valentini, MD, and Vito Potenza, MD Investigation performed at the Department of Orthopaedic Surgery, University of Rome “Tor Vergata,” Rome, Italy
Background: In patients with polyostotic fibrous dysplasia with extensive femoral involvement, severe coxa vara may cause complex femoral deformities that are difficult to treat with a single-stage surgical procedure. We evaluated the results of treatment of such patients with a two-stage procedure. Methods: Eleven patients with polyostotic fibrous dysplasia and severe coxa vara (including one who required bilateral treatment and one who required repeat treatment) were treated surgically at a mean age of fourteen years and four months. A two-stage surgical procedure was planned to correct the deformity. The first stage involved correction of the coxa vara and fixation with a hip plate. The second stage involved correction of a shepherd’s crook deformity if present and definitive fixation with an interlocking cervicodiaphyseal nail. All patients were evaluated clinically and radiographically at a mean of four years and seven months after the second-stage procedure. Results: The femoral neck-shaft angle averaged 83° before surgery and was corrected to a mean of 130° after the firststage procedure. In two patients, cutout of the hip screw-plate caused acute postoperative loss of correction (by 40° in one patient and 20° in the other). After the second-stage procedure, the neck-shaft angle was either fully corrected or improved and the shepherd’s crook deformity was fully corrected in all patients. At the time of the latest follow-up, the mean neckshaft angle was 124°, with a mean loss of correction of 5° relative to the angle measured at the end of the second-stage procedure. The mean estimated blood loss was 625 mL in the first stage and 979 mL in the second. Conclusions: The two-stage surgical procedure designed to treat patients with polyostotic fibrous dysplasia with complex femoral deformity and severe coxa vara restored a nearly normal femoral alignment that was maintained at a mean of four years and seven months of follow-up. The clinical benefits were pain relief in all of the patients and gait normalization or improvement in most. The estimated blood loss was substantial in both stages. Level of Evidence: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.
Peer Review: This article was reviewed by the Editor-in-Chief and one Deputy Editor, and it underwent blinded review by two or more outside experts. The Deputy Editor reviewed each revision of the article, and it underwent a final review by the Editor-in-Chief prior to publication. Final corrections and clarifications occurred during one or more exchanges between the author(s) and copyeditors.
C
oxa vara, either isolated or associated with a shepherd’s crook deformity, may be present in patients with polyostotic fibrous dysplasia1-10. Deformities are more severe in phosphaturic patients with McCune-Albright syndrome11,12. Coxa vara appears to be caused by body weight and by gluteal muscle traction on both the femoral neck and the trochanteric area, which are weaker than normal1,2,4,5.
The clinical consequences of coxa vara are a Trendelenburg gait (in patients with unilateral involvement) or a waddling gait (in bilateral cases), pain, and limitation of hip motion1,4,5,13. An association between the severity of coxa vara and the level of patient disability has been reported14,15. Surgical correction of coxa vara may be achieved by means of an intertrochanteric femoral osteotomy stabilized with a hip
Disclosure: None of the authors received payments or services, either directly or indirectly (i.e., via his or her institution), from a third party in support of any aspect of this work. None of the authors, or their institution(s), have had any financial relationship, in the thirty-six months prior to submission of this work, with any entity in the biomedical arena that could be perceived to influence or have the potential to influence what is written in this work. Also, no author has had any other relationships, or has engaged in any other activities, that could be perceived to influence or have the potential to influence what is written in this work. The complete Disclosures of Potential Conflicts of Interest submitted by authors are always provided with the online version of the article.
J Bone Joint Surg Am. 2015;97:119-25
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http://dx.doi.org/10.2106/JBJS.N.00230
120 TH E JO U R NA L O F B O N E & JO I N T SU RG E RY J B J S . O RG V O L U M E 97-A N U M B E R 2 J A N UA R Y 21, 2 015
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TABLE I Patient Demographics, Deformities, and Surgical Treatment* At Surgery
Patient (Sex)
Age (yr, mo)
Side
First-Stage Procedure
NeckShaft Angle (deg)
Shepherd’s Crook Deformity
Intertrochanteric Valgus Osteotomy
EBL (mL)
Hip Plate Applied
Neck-Shaft Angle After Surgery (deg)
Hip Spica Cast or Abduction Brace
Complications
1 (M)
5, 10
R
50
N
Closing-wedge
340
90° blade-plate
120
Y
2 (M)
5, 11
L
90
Y
Closing-wedge
405
130° blade-plate
125
Y
None
3 (M)†
7
L
85
N
Linear
280
Variable-angle screw-plate
140
N
Cutout cervical screw through the femoral neck with 40° loss of correction to 100°
12 4 (M)
None
L
100
Y
Linear
290
130° blade-plate
130
Y
None
8, 11
L
70
Y
Linear
730
130° blade-plate
135
N
Screws loosening with plate lateralization
130° blade-plate
130
Y
None
Variable-angle screw-plate
140
N
Fracture below the plate None
9, 2
R
90
Y
Linear
1090
5 (M)
9, 3
L
95
Y
Linear
360
6 (F)
10, 10
R
95
Y
Linear
130° blade-plate
120
N
7 (F)
10, 11
L
100
Y
Linear
130° blade-plate
135
N
None
8 (F)
16, 6
L
65
Y
Closing-wedge
Variable-angle screw-plate
120
N
None None
520
9 (F)
20, 8
R
85
Y
Linear
1870
130° blade-plate
135
N
10 (M)
33, 2
R
75
Y
Closing-wedge
515
130° blade-plate
135
N
None
11 (M)
35, 9
L
80
Y
Linear
480
Variable-angle screw-plate
130
N
Cutout cervical screw through the femoral neck with 20° loss of correction to 110°
*EBL = estimated blood loss, PHN = proximal humeral nail (Synthes), and PFN = proximal femoral nail (Synthes). †This patient had acute loss of correction after the first valgus osteotomy; the screw-plate was removed and a hip spica cast was applied. The patient underwent a second valgus osteotomy five years after the first because of recurrence of varus deformity.
plate2-5. However, in patients with polyostotic femoral dysplasia, cutout of the cervical screw, diaphyseal plate screw loosening and pullout, and fracture or deformity below the plate have been described in the intermediate term6,9,10,16,17. Consequently, stabilization with an interlocking cervicodiaphyseal nail after correction of the femoral deformity is the ideal treatment6,9,10,16,18,19. However, correction of severe coxa vara and simultaneous stabilization of the osteotomy with a cervicodiaphyseal nail to provide sufficient valgus correction of the proximal aspect of the femur (resulting in a neck-shaft angle of at least 120° to 130°) is not feasible for technical reasons9. Therefore, since 2005 we have attempted to correct severe coxa vara in such patients with a two-stage procedure. In the present study, we review both the clinical and the radiographic results in our patients at a mean of four years and four months after the second-stage procedure. Materials and Methods
F
rom 2005 to 2012, we treated eleven consecutive patients (seven male and four female) with polyostotic fibrous dysplasia and severe coxa vara that was either isolated or associated with a shepherd’s crook deformity of the femoral shaft. All patients and/or their parents provided informed consent prior to
inclusion in the study, which was approved by the ethics committee of our hospital. The adult patients and the parents of the children were also informed of the custom modification of the nails to provide better correction of the femoral deformity. Seven of the patients had McCune-Albright syndrome. The deformity in one patient was bilateral and another patient required repeat surgery, resulting in a total of thirteen sets of procedures. Four patients were skeletally mature and the remaining seven had open physes. The mean age of the patients at the time of the first-stage surgery was fourteen years and four months. Four patients had been treated with bisphosphonates. Physical examination revealed that nine of the eleven patients limped and had limited hip motion. Eight patients with thigh pain had a combination of a Trendelenburg limp and an antalgic gait. Three patients walked with either a crutch or a cane, and two who had several previous fractures and a complex femoral deformity used a wheelchair for traveling long distances. Nine patients had a lower limb length discrepancy of 1.5 to 4 cm and wore an insole or a builtup heel. A valgus osteotomy was performed at the intertrochanteric level. The osteotomy was linear in nine of the thirteen procedures and closing-wedge in four. One osteotomy was fixed with a Synthes (Switzerland) 90° blade-plate; one, with a Synthes 130° blade-plate; four, with a Martin (Germany) variable-angle hip screw-plate; and seven, with a Treu (Germany) 130° blade-plate. All patients were advised to undergo the second-stage procedure as soon as the valgus osteotomy had healed to avoid possible complications caused by the hip plate. After healing of the osteotomy and removal of the hip plate, one or two
121 TH E JO U R NA L O F B O N E & JO I N T SU RG E RY J B J S . O RG V O L U M E 97-A N U M B E R 2 J A N UA R Y 21, 2 015 d
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T WO - S TA G E S U R G I C A L T R E AT M E N T O F C O M P L E X F E M O R A L D E F O R M I T I E S W I T H S E V E R E C O X A VA R A I N P F D
TABLE I (continued) Second-Stage Procedure
Age (yr, mo)
No. of Femoral Shaft Osteotomies
6, 2
1
400
PHN
120
None
8, 3
115
6, 2
2
480
PHN
125
None
8, 5
125
EBL (mL)
Nail
Neck-Shaft Angle After Surgery (deg)
Complications
Age at Follow-up (yr, mo)
Neck-Shaft Angle at Follow-up (deg)
—
12, 10
2
480
PFN
126
None
14, 2
122
9, 7
2
1380
PHN
130
None
11, 11
125
2
1200
PHN
130
None
11, 11
130
10, 1
9, 10
1 (utilizing fracture)
1050
PFN
140
None
16, 11
130
11, 2
2
1150
11, 4
2
17
2
980
21, 1
1
1580
PFN
135
None
23, 5
130
33, 7
1
670
PFN
135
None
40
125
36, 3
2
1400
PFN
110
None
44, 5
110
PFN
130
None
19, 4
130
PFN
135
None
13, 5
130
PFN
120
None
22, 10
120
osteotomies were performed below the lesser trochanter to correct a shepherd’s crook deformity, if present, and to allow the introduction of the cervicodiaphyseal nail. In two adult patients in whom fibrous dysplasia had caused overlengthening of the femur by 2 and 3 cm, a shortening osteotomy was also performed at the same level. A medullary canal was created in the osteotomy fragments with use of a cannulated rigid reamer, and a cervicodiaphyseal nail with a spiral blade (PFN [proximal femoral nail] or PHN [proximal humeral nail]; Synthes) was used to stabilize the femoral neck. The standard PFN is straight, manufactured to be inserted into the femoral shaft through the piriformis fossa, and the maximum angulation of the spiral blade on the nail is 120°. In several cases, particularly in patients with open physes, we used a custom-modified PFN in which the proximal part of the nail has a lateral bend of 7°. This modification allowed insertion of the nail from the greater trochanter and at the same time provided 7° of additional valgus angulation to the standard 120° of the spiral blade, thereby increasing valgus correction of the femoral neck. In children less than eight years of age with a small femoral shaft diameter, a 7.5-mm-thick PHN was used to achieve an appropriate diaphyseal fit. In the PHN, the angulation of the spiral blade on the nail is 90°, and the proximal part of the nail (which contains the spiral blade) has a lateral bend of 10° to allow insertion of the nail into the femoral shaft from the tip of the greater trochanter. Since the maximum valgus angulation of the spiral blade in the PHN was 100°, we positioned the spiral blade within the lowest part of the femoral neck to hold the femoral neck in the corrected valgus position. Estimated blood loss was calculated for each of the two procedures. Statistical analysis was performed with use of SPSS software (version 15.0; IBM). Histograms confirmed that all parameters conformed to a Gaussian distribution, and descriptive statistics are therefore reported as the mean
and the standard deviation. Comparisons were made with use of ANOVA (analysis of variance) for repeated measures. A p value of
BY
T HE J OURNAL
OF
B ONE
AND J OINT
S URGERY, I NCORPORATED
Two-Stage Surgical Treatment of Complex Femoral Deformities with Severe Coxa Vara in Polyostotic Fibrous Dysplasia Ernesto Ippolito, MD, Pasquale Farsetti, MD, Matteo Benedetti Valentini, MD, and Vito Potenza, MD Investigation performed at the Department of Orthopaedic Surgery, University of Rome “Tor Vergata,” Rome, Italy
Background: In patients with polyostotic fibrous dysplasia with extensive femoral involvement, severe coxa vara may cause complex femoral deformities that are difficult to treat with a single-stage surgical procedure. We evaluated the results of treatment of such patients with a two-stage procedure. Methods: Eleven patients with polyostotic fibrous dysplasia and severe coxa vara (including one who required bilateral treatment and one who required repeat treatment) were treated surgically at a mean age of fourteen years and four months. A two-stage surgical procedure was planned to correct the deformity. The first stage involved correction of the coxa vara and fixation with a hip plate. The second stage involved correction of a shepherd’s crook deformity if present and definitive fixation with an interlocking cervicodiaphyseal nail. All patients were evaluated clinically and radiographically at a mean of four years and seven months after the second-stage procedure. Results: The femoral neck-shaft angle averaged 83° before surgery and was corrected to a mean of 130° after the firststage procedure. In two patients, cutout of the hip screw-plate caused acute postoperative loss of correction (by 40° in one patient and 20° in the other). After the second-stage procedure, the neck-shaft angle was either fully corrected or improved and the shepherd’s crook deformity was fully corrected in all patients. At the time of the latest follow-up, the mean neckshaft angle was 124°, with a mean loss of correction of 5° relative to the angle measured at the end of the second-stage procedure. The mean estimated blood loss was 625 mL in the first stage and 979 mL in the second. Conclusions: The two-stage surgical procedure designed to treat patients with polyostotic fibrous dysplasia with complex femoral deformity and severe coxa vara restored a nearly normal femoral alignment that was maintained at a mean of four years and seven months of follow-up. The clinical benefits were pain relief in all of the patients and gait normalization or improvement in most. The estimated blood loss was substantial in both stages. Level of Evidence: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.
Peer Review: This article was reviewed by the Editor-in-Chief and one Deputy Editor, and it underwent blinded review by two or more outside experts. The Deputy Editor reviewed each revision of the article, and it underwent a final review by the Editor-in-Chief prior to publication. Final corrections and clarifications occurred during one or more exchanges between the author(s) and copyeditors.
C
oxa vara, either isolated or associated with a shepherd’s crook deformity, may be present in patients with polyostotic fibrous dysplasia1-10. Deformities are more severe in phosphaturic patients with McCune-Albright syndrome11,12. Coxa vara appears to be caused by body weight and by gluteal muscle traction on both the femoral neck and the trochanteric area, which are weaker than normal1,2,4,5.
The clinical consequences of coxa vara are a Trendelenburg gait (in patients with unilateral involvement) or a waddling gait (in bilateral cases), pain, and limitation of hip motion1,4,5,13. An association between the severity of coxa vara and the level of patient disability has been reported14,15. Surgical correction of coxa vara may be achieved by means of an intertrochanteric femoral osteotomy stabilized with a hip
Disclosure: None of the authors received payments or services, either directly or indirectly (i.e., via his or her institution), from a third party in support of any aspect of this work. None of the authors, or their institution(s), have had any financial relationship, in the thirty-six months prior to submission of this work, with any entity in the biomedical arena that could be perceived to influence or have the potential to influence what is written in this work. Also, no author has had any other relationships, or has engaged in any other activities, that could be perceived to influence or have the potential to influence what is written in this work. The complete Disclosures of Potential Conflicts of Interest submitted by authors are always provided with the online version of the article.
J Bone Joint Surg Am. 2015;97:119-25
d
http://dx.doi.org/10.2106/JBJS.N.00230
120 TH E JO U R NA L O F B O N E & JO I N T SU RG E RY J B J S . O RG V O L U M E 97-A N U M B E R 2 J A N UA R Y 21, 2 015
T WO - S TA G E S U R G I C A L T R E AT M E N T O F C O M P L E X F E M O R A L D E F O R M I T I E S W I T H S E V E R E C O X A VA R A I N P F D
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TABLE I Patient Demographics, Deformities, and Surgical Treatment* At Surgery
Patient (Sex)
Age (yr, mo)
Side
First-Stage Procedure
NeckShaft Angle (deg)
Shepherd’s Crook Deformity
Intertrochanteric Valgus Osteotomy
EBL (mL)
Hip Plate Applied
Neck-Shaft Angle After Surgery (deg)
Hip Spica Cast or Abduction Brace
Complications
1 (M)
5, 10
R
50
N
Closing-wedge
340
90° blade-plate
120
Y
2 (M)
5, 11
L
90
Y
Closing-wedge
405
130° blade-plate
125
Y
None
3 (M)†
7
L
85
N
Linear
280
Variable-angle screw-plate
140
N
Cutout cervical screw through the femoral neck with 40° loss of correction to 100°
12 4 (M)
None
L
100
Y
Linear
290
130° blade-plate
130
Y
None
8, 11
L
70
Y
Linear
730
130° blade-plate
135
N
Screws loosening with plate lateralization
130° blade-plate
130
Y
None
Variable-angle screw-plate
140
N
Fracture below the plate None
9, 2
R
90
Y
Linear
1090
5 (M)
9, 3
L
95
Y
Linear
360
6 (F)
10, 10
R
95
Y
Linear
130° blade-plate
120
N
7 (F)
10, 11
L
100
Y
Linear
130° blade-plate
135
N
None
8 (F)
16, 6
L
65
Y
Closing-wedge
Variable-angle screw-plate
120
N
None None
520
9 (F)
20, 8
R
85
Y
Linear
1870
130° blade-plate
135
N
10 (M)
33, 2
R
75
Y
Closing-wedge
515
130° blade-plate
135
N
None
11 (M)
35, 9
L
80
Y
Linear
480
Variable-angle screw-plate
130
N
Cutout cervical screw through the femoral neck with 20° loss of correction to 110°
*EBL = estimated blood loss, PHN = proximal humeral nail (Synthes), and PFN = proximal femoral nail (Synthes). †This patient had acute loss of correction after the first valgus osteotomy; the screw-plate was removed and a hip spica cast was applied. The patient underwent a second valgus osteotomy five years after the first because of recurrence of varus deformity.
plate2-5. However, in patients with polyostotic femoral dysplasia, cutout of the cervical screw, diaphyseal plate screw loosening and pullout, and fracture or deformity below the plate have been described in the intermediate term6,9,10,16,17. Consequently, stabilization with an interlocking cervicodiaphyseal nail after correction of the femoral deformity is the ideal treatment6,9,10,16,18,19. However, correction of severe coxa vara and simultaneous stabilization of the osteotomy with a cervicodiaphyseal nail to provide sufficient valgus correction of the proximal aspect of the femur (resulting in a neck-shaft angle of at least 120° to 130°) is not feasible for technical reasons9. Therefore, since 2005 we have attempted to correct severe coxa vara in such patients with a two-stage procedure. In the present study, we review both the clinical and the radiographic results in our patients at a mean of four years and four months after the second-stage procedure. Materials and Methods
F
rom 2005 to 2012, we treated eleven consecutive patients (seven male and four female) with polyostotic fibrous dysplasia and severe coxa vara that was either isolated or associated with a shepherd’s crook deformity of the femoral shaft. All patients and/or their parents provided informed consent prior to
inclusion in the study, which was approved by the ethics committee of our hospital. The adult patients and the parents of the children were also informed of the custom modification of the nails to provide better correction of the femoral deformity. Seven of the patients had McCune-Albright syndrome. The deformity in one patient was bilateral and another patient required repeat surgery, resulting in a total of thirteen sets of procedures. Four patients were skeletally mature and the remaining seven had open physes. The mean age of the patients at the time of the first-stage surgery was fourteen years and four months. Four patients had been treated with bisphosphonates. Physical examination revealed that nine of the eleven patients limped and had limited hip motion. Eight patients with thigh pain had a combination of a Trendelenburg limp and an antalgic gait. Three patients walked with either a crutch or a cane, and two who had several previous fractures and a complex femoral deformity used a wheelchair for traveling long distances. Nine patients had a lower limb length discrepancy of 1.5 to 4 cm and wore an insole or a builtup heel. A valgus osteotomy was performed at the intertrochanteric level. The osteotomy was linear in nine of the thirteen procedures and closing-wedge in four. One osteotomy was fixed with a Synthes (Switzerland) 90° blade-plate; one, with a Synthes 130° blade-plate; four, with a Martin (Germany) variable-angle hip screw-plate; and seven, with a Treu (Germany) 130° blade-plate. All patients were advised to undergo the second-stage procedure as soon as the valgus osteotomy had healed to avoid possible complications caused by the hip plate. After healing of the osteotomy and removal of the hip plate, one or two
121 TH E JO U R NA L O F B O N E & JO I N T SU RG E RY J B J S . O RG V O L U M E 97-A N U M B E R 2 J A N UA R Y 21, 2 015 d
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T WO - S TA G E S U R G I C A L T R E AT M E N T O F C O M P L E X F E M O R A L D E F O R M I T I E S W I T H S E V E R E C O X A VA R A I N P F D
TABLE I (continued) Second-Stage Procedure
Age (yr, mo)
No. of Femoral Shaft Osteotomies
6, 2
1
400
PHN
120
None
8, 3
115
6, 2
2
480
PHN
125
None
8, 5
125
EBL (mL)
Nail
Neck-Shaft Angle After Surgery (deg)
Complications
Age at Follow-up (yr, mo)
Neck-Shaft Angle at Follow-up (deg)
—
12, 10
2
480
PFN
126
None
14, 2
122
9, 7
2
1380
PHN
130
None
11, 11
125
2
1200
PHN
130
None
11, 11
130
10, 1
9, 10
1 (utilizing fracture)
1050
PFN
140
None
16, 11
130
11, 2
2
1150
11, 4
2
17
2
980
21, 1
1
1580
PFN
135
None
23, 5
130
33, 7
1
670
PFN
135
None
40
125
36, 3
2
1400
PFN
110
None
44, 5
110
PFN
130
None
19, 4
130
PFN
135
None
13, 5
130
PFN
120
None
22, 10
120
osteotomies were performed below the lesser trochanter to correct a shepherd’s crook deformity, if present, and to allow the introduction of the cervicodiaphyseal nail. In two adult patients in whom fibrous dysplasia had caused overlengthening of the femur by 2 and 3 cm, a shortening osteotomy was also performed at the same level. A medullary canal was created in the osteotomy fragments with use of a cannulated rigid reamer, and a cervicodiaphyseal nail with a spiral blade (PFN [proximal femoral nail] or PHN [proximal humeral nail]; Synthes) was used to stabilize the femoral neck. The standard PFN is straight, manufactured to be inserted into the femoral shaft through the piriformis fossa, and the maximum angulation of the spiral blade on the nail is 120°. In several cases, particularly in patients with open physes, we used a custom-modified PFN in which the proximal part of the nail has a lateral bend of 7°. This modification allowed insertion of the nail from the greater trochanter and at the same time provided 7° of additional valgus angulation to the standard 120° of the spiral blade, thereby increasing valgus correction of the femoral neck. In children less than eight years of age with a small femoral shaft diameter, a 7.5-mm-thick PHN was used to achieve an appropriate diaphyseal fit. In the PHN, the angulation of the spiral blade on the nail is 90°, and the proximal part of the nail (which contains the spiral blade) has a lateral bend of 10° to allow insertion of the nail into the femoral shaft from the tip of the greater trochanter. Since the maximum valgus angulation of the spiral blade in the PHN was 100°, we positioned the spiral blade within the lowest part of the femoral neck to hold the femoral neck in the corrected valgus position. Estimated blood loss was calculated for each of the two procedures. Statistical analysis was performed with use of SPSS software (version 15.0; IBM). Histograms confirmed that all parameters conformed to a Gaussian distribution, and descriptive statistics are therefore reported as the mean
and the standard deviation. Comparisons were made with use of ANOVA (analysis of variance) for repeated measures. A p value of
