Nitial evaluation of the results of osteotomy with intramedullary fixation for both lower limbs in osteogenesis imperfecta patients at 7a military hospital – Tran Quoc Doanh

Journal of military pharmaco-medicine n 0 9-2018 142 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 (drtranquocdoanh@gmail.com) Date received: 11/10/2018 Date accepted: 03/12/2018 Journal of military pharmaco-medicine n 0 9-2018 143 SUBJECTS 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. Journal of military pharmaco-medicine n 0 9-2018 144 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). After 1 month After 3 months After 6 months After 12 months After 24 months After 36 months Criteria Result n = 49 n = 47 n = 45 n = 44 n = 39 n = 20 1 nail 10 9 10 9 4 3 Aligned 2 nails 38 38 35 35 29 12 1 nail 0 0 0 0 4 4 Axial of limb Nonaligned 2 nails 1 (*) 0 0 0 2 1 1 nail 10 9 10 9 8 7 Normal 2 nails 38 38 35 35 31 13 1 nail 0 0 0 0 0 0 Bowing 2 nails 1 (*) 0 0 0 0 0 1 nail 0 0 0 0 4 4 Extruding 2 nails 0 0 0 0 1 1 1 nail 0 0 0 0 0 0 Nail Failure 2 nails 0 0 0 1 1 1 Journal of military pharmaco-medicine n 0 9-2018 145 (*: 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 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) 2 nails expanding No 37 (100.0) 25 (59.4) 3 (8.6) 4 (10.8) 4 (13.8) 2 (15.4) Sum 37 36 35 37 29 13 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 After n Mean SD Maximum value Minimum value p 3 months 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 < 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). Journal of military pharmaco-medicine n 0 9-2018 146 Table 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 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) 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) Fracture (*) 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 Callus No callus 0 0 1 (**) 1 (**) 0 0 (*: Number of cases on each operated site; **: 2 patients shown non-union after 12 months, re-operation indicated) Journal of military pharmaco-medicine n 0 9-2018 147 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. REFERENCES 1. 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. 2. 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. 3. Sillence D.O, Senn A, Danks D.M. Genetic heterogeneity in osteogenesis imperfecta. J Med Genet. 1979, 16 (2), pp.101-116. 4. A.I. Lang-Stevensonand, W.J. Sharrard. 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