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.
Trang 1INITIAL EVALUATION OF THE RESULTS OF OSTEOTOMY WITH INTRAMEDULLARY FIXATION FOR BOTH LOWER LIMBS
IN OSTEOGENESIS IMPERFECTA PATIENTS
AT 7A MILITARY HOSPITAL
Tran Quoc Doanh 1 ; Pham Dang Ninh 2 ; Luong Dinh Lam 3
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
COL1A2genes 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 (drtranquocdoanh@gmail.com)
Date received: 11/10/2018
Date accepted: 03/12/2018
Trang 2SUBJECTS AND METHODS
1 Subjects
33 OI patients underwent surgical
treatment at 7A Military Hospital, from
January 2012 to December 2016
* Inclusion criteria:
- Patient was diagnosed with OI based
on Neish A.S Winalski (1995) [1], Pattekar
M.A (2003) [1] and Sillence (1979) [3]
- Indication of surgical intervention:
+ Deformity of extremity or fracture
+ > 2 years old, unable to ambulate
+ Intervention to reduce incidence of
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:
+ 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 under guiding of C-arm When the nail is stuck in angulated point, expose the bone and oteotomy then continute advancing the nail until it touches the distal femur
Retreat the nail to the last angulated point, cut the femur in this location, introduce the second nail retrogrately, 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
Trang 3RESULTS 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)
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)
After 1 month
After 3 months
After 6 months
After 12 months
After 24 months
After 36 months
Aligned
Axial of
limb
Nonaligned
Normal
Bowing
Extruding
Nail
Failure
Trang 4(*: 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)
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
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 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 X-ray
Yes 0 (0.0) 11 (30.6) 32 (91.4) 33 (89.2) 25 (86.2) 11 (84.6)
2 nails
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)
Length of nail expanding
< 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)
Trang 5Table 5: Postoperative functional outcome
Preoperative (n = 33)
After 1 months (n = 29)
After 3 months (n = 28)
After 6 months (n = 28)
After 12 months (n = 24)
After 24 months (n = 24)
After 36 months (n = 17) Function
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
Independent stand 1 (3.0) 0 (0.0) 1 (3.6) 0 (0.0) 0 (0.0) 0 (0.0) 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)
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
X-ray imaging
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)
Callus
(*: Number of cases on each operated site; **: 2 patients shown non-union after
12 months, re-operation indicated)
Trang 6There 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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