Initial evaluation of the results of osteotomy with intramedullary fixation for both lower limbs in osteogenesis imperfecta patients at 7A Military Hospital

Journal of military pharmaco-medicine n09-2018

INITIAL EVALUATION OF THE RESULTS OF OSTEOTOMY
WITH INTRAMEDULLARY FIXATION FOR BOTH LOWER LIMBS
IN OSTEOGENESIS IMPERFECTA PATIENTS
AT 7A MILITARY HOSPITAL
Tran Quoc Doanh1; Pham Dang Ninh2; Luong Dinh Lam3

SUMMARY
Objectives: To evaluate the effect of osteotomy with intramedullary fixation in osteogenesis
imperfecta. Subjects and methods: 33 patients were treated by osteotomy with intramedullary
fixation in lower limbs. Results: 47 operations, 53 sites of surgery, mean operation time 85 ± 8
minutes. Follow-up: In the first year, 44/44 axial of limbs were aligned, in the second year 6/39
patients developed nonaligned axial of limbs, but without indication of surgery, in the third year,
5/20 cases developed deformity of nail. Osteotomy with 2 intramedullary nails fixation is
effective in lengthening lower limbs. Good outcome was obtained postoperatively, recurrent
fracture was not recorded. Conclusions: Osteotomy with intramedullary fixation in 33 patients
obtained good outcome. This is a safe procedure.
* Keywords: Osteogensis imperfecta; Intramedullary nail.

INTRODUCTION
Osteogenesis imperfecta (OI) is a
disorder of bone fragility chiefly caused
by mutations in the COL1A1 and
COL1A2 genes

that

encode type I

procollagen. Because OI is a genetic

condition, it has no cure [4]. Cyclic
administration of intravenous pamidronate
reduces pain and increases bone mineral
density, however the incidence of fracture
is still high [2].

So surgical treatment is the main
option for OI. The aim of surgery is to
correct the deformity, increase the strength
of bones and reduce the incidence of
fracture. The technique of multiple
osteotomy with intramedullary fixation is
safe and effective. This technique was
introduced by Sofield - Millar.
Bailey-Dubow [4] technique has gained
significant improvement with intramedullary
nails in both proximal and distal long bone.
Recently, by the combination with C-arm,
this technique can be done minimal invasively.

1 7A Military Hospital
2 103 Military Hospital
3 Choray Hospital
Corresponding author: Tran Quoc Doanh ()
Date received: 11/10/2018
Date accepted: 03/12/2018

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Journal of military pharmaco-medicine n09-2018

SUBJECTS AND METHODS
1. Subjects.
33 OI patients underwent surgical
treatment at 7A Military Hospital, from
January 2012 to December 2016.
* Inclusion criteria:

+ Femur: There are 2 situations.
The first situation: With moderately
deformed femur or only angular distortion,
broad canal bone, almost normal human
size, not flat in the posterior direction. Drill
the intramedullary canal through top of
greater trochanter, introduce the first nail

- Patient was diagnosed with OI based

under guiding of C-arm. When the nail is

on Neish A.S Winalski (1995) [1], Pattekar

stuck in angulated point, expose the bone

M.A (2003) [1] and Sillence (1979) [3].
- Indication of surgical intervention:

and oteotomy then continute advancing
the nail until it touches the distal femur.

+ Deformity of extremity or fracture.

Retreat the nail to the last angulated

+ > 2 years old, unable to ambulate.

point, cut the femur in this location,

+ Intervention to reduce incidence of

introduce the second nail retrogrately,

fracture.
+ Bowing angle > 100, legs discrepancy
makes it difficult to walk.
+ The illness makes patient depressed
and hopeless and needs to be operated.
2. Methods.
- Research design: Clinical trial of
surgical intervention.
- Technique of procedure: Multiple
osteotomy and intramedullary fixation
(using Kirschner, Rush nails) according to
Topouchian [5].
- Data analysis: Using SPSS 22.0
software.
* Procedure technique:
- Anesthesia: General anesthesia (inhaled
anesthesia).

- Technique details:

advance the first nail to the distal part of
femur, then advace the second nail,
measure the length of femur to cut the
nails appropriately.
The second situation: The femur is
small, AP diameter is small, the canal is
not visible under C-arm guiding, nailing is
difficult:
Cut the femur in the location of being
stuck, do osteotomy to correct the axial,
create canal in this part, then introduce
the nail. With very narrow canal bone, we
use only one nail.
Tibia: Similar technique is used, the
entry point is just posterior the insertion of
pattela tendon.
* Data collection: Data was collected
perioperatively.

The

follow-up

was

36 months.
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Journal of military pharmaco-medicine n09-2018
RESULTS AND DISCUSSION
We operated on 33 patients (from 2 to 33 years old), with 47 operations and 53 sites
of surgery.
Table 1: The duration of operations (n = 33).
Location

n

Mean

SD

Min

Max

Right femur

14

83.93

7.39

70.0

90.0

Left femur

20

85.00

7.78

70.0

100.0

Right tibia

9

86.67

6.61

75.0

90.0

Left tibia

10

86.00

10.49

75.0

110.0

Total

53

85.19

7.90

70.0

110.0

Mean of sugery duration was 85.19 ± 7.9 minutes. There was no difference in the
surgery duration between femur and tibia. This amount of time is greater than
Chitgopkar’s (2005) in Egypt, whose average operative time was 40 minutes (range, 20
- 72 minutes) [10]. There were no severe complications. In 1 case, the femoral cortex
was broken during drilling that needed augmented wire, eventually had good result.
* Complication and iatrogenic:
There was no serious complication and iatrogenic. Bone cortex was broken in one
case, but we used steel wire to fix the problem and the outcome was good.
Table 2: Radiology results (after 1, 3, 6, 12, 24, 36 months).
Criteria

After 3

months

After 6
months

After 12
months

After 24
months

After 36
months

n = 49

n = 47

n = 45

n = 44

n = 39

n = 20

1 nail

10

9

10

9

4

3

2 nails

38

38

35

35

29

12

1 nail

0

0

0

0

4

4

2 nails

1 (*)

0

0

0

2

1

1 nail

10

9

10

9

8

7

2 nails

38

38

35

35

31

13

1 nail

0

0

0

0

0

0

2 nails

1 (*)

0

0

0

0

0

1 nail

0

0

0

0

4

4

2 nails

0

0

0

0

1

1

1 nail

0

0

0

0

0

0

2 nails

0

0

0

1

1

1

Result

Aligned
Axial of
limb
Nonaligned

Normal

Bowing
Nail
Extruding

Failure

144

After 1
month


Journal of military pharmaco-medicine n09-2018
(*: 1 case of postoperative bent
femoral intramedullary nail, later was
augmented with plaster cast. 36-month
outcome was good with bone healing and
good alignment)

intramedullary nail penetrated bone
cortex. 4/6 bone re-bending cases were
from operation with 1 intramedullary nail,
the other 2 cases were from operation
with 2 non expanding intramedually nail.

Follow-up assessment after 12 months
in all cases showed good alignment of bones
and instruments, of which 1 exceptional
case had bent nail, which was also
handled properly. Follow-up assessment
after 2 years showed 6/39 cases
associated with re-bending bone but
within acceptable degree and no required
re-operation, usually associated with

Follow-up assessment after 3 years in
20 cases, there were 5 cases represented

bending deformity, which such deformity
existed before operation, and the degree
did not change significantly throughout
the years. This figure was higher
compared with Bailey-Dubow’s study [4]
extensible rodsmethod of Jerosch (1998)
[9] and Rosemberg (2018) [8].

Table 3: Results of nails expanding (after 1, 3, 6, 12, 24, 36 months).
After 1
month

After 3
months

After 6
months

After 12
months

After 24
months

After 36
months

n (%)

n (%)

n (%)

n (%)

n (%)

n(%)

Yes

0 (0.0)

11 (30.6)

32 (91.4)

33 (89.2)

25 (86.2)

11 (84.6)

No

37 (100.0)

25 (59.4)

3 (8.6)

4 (10.8)

4 (13.8)

2 (15.4)

37

36

35

37

29

13

X-ray

2 nails
expanding
Sum

In cases with 2 intramedullary nails, we monitored the nails expanding according to
the growth of bones. After just 3 months, the relative expanding of nails was shown in
11 out of 36 cases (30.56%) and 32 out of 35 cases (91.43%) after 6 months.
Therefore, the using of 2 nails was not adverse to the growth of bones. This result was
better compared with Tae-Joon Cho et al (2007) [6] who enhanced Sheffield rod for no

articular exposure.
Table 4: Postoperative length of nail expanding (after 3, 6, 12, 24, 36 months).
After

n

3 months

Length of nail expanding
Mean

SD

Maximum value

Minimum value

11

0.38

0.13

0.50

0.20

6 months

32

0.85

0.42

2.10

0.30

12 months

33

1.79

0.80

4.00

0.50

24 months

25

2.70

1.00

4.10

1.50

36 months

11

3.01

1.09

5.00

1.50

p

< 0.05

The speed of nail expanding in cases with 2 intramedullary nail fixation continuously
increased after 3 months, which had statistical significance (p < 0.05).
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Journal of military pharmaco-medicine n09-2018
Table 5: Postoperative functional outcome.
After 1
months

After 3

months

After 6
months

After 12
months

After 24
months

After 36
months

(n = 29)

(n = 28)

(n = 28)

(n = 24)

(n = 24)

(n = 17)

n (%)

n (%)

n (%)

n (%)

n (%)

n (%)

n (%)

Independent sitting

13 (39.4)

22 (75.9)

5 (17.9)

1 (3.6)

1 (4.2)

0 (0.0)

0 (0.0)

Crawling/bottom
shuffling

17 (51.5)

5 (17.2)

9 (32.1)

9 (32.1)

4 (16.7)

3 (12.5)

4 (23.5)

Independent stand

1 (3.0)

0 (0.0)

1 (3.6)

0 (0.0)

0 (0.0)

0 (0.0)

0 (0.0)

Assisted sit

0 (0.0)

2 (6.9)

4 (14.3)

1 (3.6)

4 (16.7)

1 (4.2)

0 (0.0)

Independent walk

1 (3.0)

0 (0.0)

0 (0.0)

9 (32.1)

12 (50.0)

12 (50.0)

5 (29.4)

Assisted walk

1 (3.0)

0 (0.0)

9 (32.1)

8 (25.6)

5 (20.8)

8 (33.3)

8 (47.1)

Preoperative
Function

(n = 33)

There was a significant improvement in
functions in 3rd - 6thmonth postoperation.
Function improvement was also shown
after 12, 24, 36 months. Preoperatively,
most of the patients could only sit
independently and crawl. However, after
operation, these patients could walk
instead.

The result was similar to Chitgopkar’s
(2005) [10]. Bone healling achieved
after 6 - 14 weeks. The function also
improved after 12, 24, 36 months.
Preoperatively, the majority of patients
could only sit independently and crawl

comparing to walking posoperatively. The
result was consistent with Georgescu’s
(2013) [7].
There was a significant improvement in
mobility in the first 3 - 6 months after
surgery. Changes in mobility compared to
preoperation had statistically significant
differences with p < 0.05. Postoperative
evaluation of 12, 24, 36 months improved
significantly. Before the operation, the
patient was independent sitting and
crawling/bottom shuffling, then the patient
was able to walk. Our results were similar
to Georgescu’s findings (2013) [7].

Table 6: Posoperative bone fracture and callus formation.
After 1
month
(n = 49)

After 3
months
(n = 47)

After 6
months
(n = 45)

After 12
months
(n = 45)

After 24
months
(n = 37)

After 36
months
(n = 20)

0

0

0

0

0

0

Grade 1

49

2

1

1

0

0

Grade 2

0

45

0

0

0

0

Grade 3

0

0

42

0

0

0

No callus

0

0

1 (**)

1 (**)

0

0

X-ray imaging
Fracture (*)

Callus

(*: Number of cases on each operated site; **: 2 patients shown non-union after
12 months, re-operation indicated)
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Journal of military pharmaco-medicine n09-2018
There was 1 case associated with
delayed union (grade 1 callus) and 1 case
with no bone healing after 6 months. After
12 months, these 2 cases represented
non-union and therefore were indicated
for secondary operation. Accordingly, the
number of secondary operation was low
with only 2/49 cases (4.08%). This result
was higher compared with Jerosch’s (1998)
[9], Tae-Joon Cho’s findings (2007) [6].
CONCLUSION
Treating osteogenesis imperfecta with
operation has brought effective results.
Our trial on 33 patients suggest that it is
safe and effective to perform deformity
correction operation. Follow-up assessment
after 36 months shows good result in
bone alignment and re-fracture number.
Especially 2 intramedullary nail fixation
guarantees good alignment in both bone
and nail, and postoperative nail expanding
feature is advisable for the growth of
chidren’s bones.
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