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.
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 ± 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 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 7A Military Hospital 103 Military Hospital Choray Hospital Corresponding author: Tran Quoc Doanh (drtranquocdoanh@gmail.com) Date received: 11/10/2018 Date accepted: 03/12/2018 142 Journal of military pharmaco-medicine n09-2018 SUBJECTS AND METHODS Subjects 33 OI patients underwent surgical treatment at 7A Military Hospital, from January 2012 to December 2016 * Inclusion criteria: + Femur: There are 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 + > 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 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, 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 143 Journal of military pharmaco-medicine n09-2018 RESULTS AND DISCUSSION We operated on 33 patients (from 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 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 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 months After months After 12 months After 24 months After 36 months n = 49 n = 47 n = 45 n = 44 n = 39 n = 20 nail 10 10 nails 38 38 35 35 29 12 nail 0 0 4 nails (*) 0 1 nail 10 10 nails 38 38 35 35 31 13 nail 0 0 0 nails (*) 0 0 nail 0 0 4 nails 0 0 1 nail 0 0 0 nails 0 1 Result Aligned Axial of limb Nonaligned Normal Bowing Nail Extruding Failure 144 After month Journal of military pharmaco-medicine n09-2018 (*: 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 intramedullary nail, the other cases were from operation with non expanding intramedually nail Follow-up assessment after 12 months in all cases showed good alignment of bones and instruments, of which exceptional case had bent nail, which was also handled properly Follow-up assessment after 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 years in 20 cases, there were 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 month After months After months After 12 months After 24 months After 36 months n (%) n (%) n (%) n (%) n (%) n(%) Yes (0.0) 11 (30.6) 32 (91.4) 33 (89.2) 25 (86.2) 11 (84.6) No 37 (100.0) 25 (59.4) (8.6) (10.8) (13.8) (15.4) 37 36 35 37 29 13 X-ray nails expanding Sum In cases with intramedullary nails, we monitored the nails expanding according to the growth of bones After just 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 months Therefore, the using of 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 months Length of nail expanding Mean SD Maximum value Minimum value 11 0.38 0.13 0.50 0.20 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 intramedullary nail fixation continuously increased after months, which had statistical significance (p < 0.05) 145 Journal of military pharmaco-medicine n09-2018 Table 5: Postoperative functional outcome After months After months After 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) (17.9) (3.6) (4.2) (0.0) (0.0) Crawling/bottom shuffling 17 (51.5) (17.2) (32.1) (32.1) (16.7) (12.5) (23.5) Independent stand (3.0) (0.0) (3.6) (0.0) (0.0) (0.0) (0.0) Assisted sit (0.0) (6.9) (14.3) (3.6) (16.7) (4.2) (0.0) Independent walk (3.0) (0.0) (0.0) (32.1) 12 (50.0) 12 (50.0) (29.4) Assisted walk (3.0) (0.0) (32.1) (25.6) (20.8) (33.3) (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 - 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 - 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 month (n = 49) After months (n = 47) After months (n = 45) After 12 months (n = 45) After 24 months (n = 37) After 36 months (n = 20) 0 0 0 Grade 49 1 0 Grade 45 0 0 Grade 0 42 0 No callus 0 (**) (**) 0 X-ray imaging Fracture (*) Callus (*: Number of cases on each operated site; **: patients shown non-union after 12 months, re-operation indicated) 146 Journal of military pharmaco-medicine n09-2018 There was case associated with delayed union (grade callus) and case with no bone healing after months After 12 months, these 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 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 REFERENCES Bùi Thị Hồng Châu Xác định đột biến gen Col1a1, col1a2 gây bệnh xương bất toàn (osteogenesis imperfecta) Luận án Tiến sỹ Y khoa Hóa sinh Trường Đại học Y Hà Nội Hà Nội Anam E.A, Rauch F, Glorieux F.H et al Osteotomy healing in children with osteogenesis imperfecta receiving bisphosphonate treatment J Bone Miner Res 2015, 30 (8), pp.1362-1368 Sillence D.O, Senn A, Danks D.M Genetic heterogeneity in osteogenesis imperfecta J Med Genet 1979, 16 (2), pp.101-116 A.I Lang-Stevensonand, W.J Sharrard Intramedullary rodding with Bailey-Dubow extensible rods in osteogenesis imperfecta An interim report of results and complications J Bone Joint Surg Br 1984, 66 (2), pp.227-232 G Finidori V Topouchian, C.Glorion Intramedullary stabilization of the long bones in children with osteogenesis imperfecta 2006, pp.219-229 T.J Cho et al Interlocking telescopic rod for patients with osteogenesis imperfecta J Bone Joint Surg Am 2007, 89 (5), pp.1028-1035 Georgescu I, Vlad C, Gavriliu T.Ș, Dan D, Pârvan A.A Surgical treatment in osteogenesis imperfecta - 10 years experience Journal of Medicine and Life 2013, AprilJune, Vol 6, Issue 2, pp.205-213 D.L.Rosemberg, E.O.Goiano, M.Akkari, C Santili Effects of a telescopic intramedullary rod for treating patients with osteogenesis imperfecta of the femur J Child Orthop 2018, 12, pp.97-103 Jerosch J, Mazzotti I, Tomasevic M Complications after treatment of patients with osteogenesis imperfecta with a Bailey Dubow rod Arch Orthop Trauma Surg 1998, 117 (4 - 5), p.2405 10 Shashank D Chitgopkar Internal fixation of femoral shaft fractures in children by intramedullary Kirschner wires (a prospective study): its significance for developing countries BMC Surgery 2005, pp.1-6 147 ... intramedullary rod for treating patients with osteogenesis imperfecta of the femur J Child Orthop 2018, 12, pp.97-103 Jerosch J, Mazzotti I, Tomasevic M Complications after treatment of patients with osteogenesis. .. expanding Sum In cases with intramedullary nails, we monitored the nails expanding according to the growth of bones After just months, the relative expanding of nails was shown in 11 out of 36... 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]