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Kyphosis in myelomeningocele is a rare and difficult problem. Many strategies have been used with no single procedure universally agreed on. Techniques involving anterior and posterior fixation may provide better fusion.
We describe a novel procedure for anteroposterior kyphectomy in patients with myelomeningocele. Apical posterior kyphectomy is fo...
Context in source publication
Context 1
... are few conditions in the pediatric spine more chal- lenging than kyphosis in patients with myelomeningocele (MMC) (Fig. 1). Although techniques for treatment of this condition have evolved, a definitive ''gold standard'' pro- cedure, with uniformly predictable outcomes, remains elusive. The incidence of MMC is between 0.005% and 0.2% of all live births [6,7]. Deformities in MMC occur in 50% of patients [7]. Congenital kyphosis and kyphoscoliosis are less ...
Similar publications
Patients with myelomeningocele and rigid lumbar and thoracolumbar kyphosis face substantial functional difficulties with sitting and lying supine and are prone to skin breakdown over the gibbus and risk of infection. Kyphectomy, along with cordotomy and segmental spinal instrumentation down to the pelvis, is one alternative that can provide reliabl...
Citations
... Accepted articles were divided considering the number of patients in studies with less than 5 patients (case reports and case series) ( Table 1) [8,[11][12][13][14][15][16][17][18][19][20][21][22][23][24], studies with a number of patients between 5 and 20 (Table 2) [5,7,9,[25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41][42][43][44], and all the studies with more than 20 patients were showed in Table 3 [6,[45][46][47][48][49][50][51][52]. ...
Objectives
To describe surgical treatment of 3 cases of severe and progressive thoracolumbar kyphosis in myelomeningocele and provide a systematic review of the available literature on the topic.
Methods
Medical records and pre- and post-operative imaging of 3 patients with thoracolumbar kyphosis and myelomeningocele were reviewed. A database search was performed for all manuscripts published on kyphectomy and/or surgical treatment of kyphosis in myelomeningocele. Patients’ information, preoperative kyphosis angle, type of surgery, levels of surgery degrees of correction after surgery and at follow-up, and complications were reviewed for the included studies.
Results
Three cases underwent posterior vertebral column resection (pVCR) of 2–4 segments at the apex of the kyphosis (kyphectomy). Long instrumentation was performed with all pedicle screws constructed from the thoracic spine to the pelvis using iliac screws. According to literature review, a total of 586 children were treated for vertebral kyphosis related to myelomeningocele. At least one vertebra was excised to gain some degree of correction of the deformity. Different types of instrumentation were used over time and none of them demonstrated to be superior over the other.
Conclusion
Surgical treatment of progressive kyphosis in myelomeningocele has evolved over the years incorporating all major advances in spinal instrumentation techniques. Certainly, the best results in terms of preservation of correction after surgery and less revision rates were obtained with long construct and screws. However, complication rate remains high with skin problems being the most common complication. The use of low-profile instrumentation remains critical for treatment of these patients.
... Some authors suggested the use of instrumentation which provided fixation from anterior in order to prevent skin problems [15]. However, this is a rather invasive procedure, and it does not allow pelvic fixation in rigid kyphosis surgeries which makes it biomechanically less reliable [16]. ...
... However, we observed a kyphosis corrective rate of 99.1% in our patients. This rate highlights the superiority of our technique (sliding growing rod) compared to short-segment fusion surgery [16,23,28]. ...
... Despite the many defined techniques, complication rates are often high [5, [29][30][31]. Average complication rates of the surgeries applied to patients with kyphosis associated with MMC are 50% regardless of the technique being either long-segment fusion or growing rod technique [16,23,28]. The most common complications were wound site problems and implant failures [30,32]. ...
Objective
Neural tube defects are the most common congenital disorders after cardiac anomalies. Lumbar kyphosis deformity is observed in 8–15% of these patients. This deformity severely limits the daily lives of these patients. In our study, we aimed to correct the kyphosis angle of the patients with lumbar kyphosis associated with myelomeningocele (MMC) and allow them to continue their growth without limiting their lung capacity by applying kyphectomy and sliding growing rod technique.
Patients and methods
In this study, we retrospectively evaluated 24 patients with congenital lumbar kyphosis deformity associated with MMC, aged between 4 and 9 years, and who applied to Umraniye Training and Research Hospital between the dates of 2018 and 2021. We evaluated preoperative and postoperative kyphosis angles, correction rates, bleeding during operations, operation time, level of instrumentation, number of the resected vertebrae, initial levels of the posterior defects, duration of hospital stays, annual lengthening, and weight of the patients.
Results
Mean age was 5.04 (between 4 and 9). Mean preoperative and early postoperative kyphosis angles were 129.8° (87–175°) and 0.79° (− 20–24°), respectively. The kyphotic deformity correction rate was 99.1%. A difference was found regarding kyphosis measurements between preoperative and early period values (p < 0.05). The annual height lengthening of patients was calculated as 0.74 cm/year and 0.77 cm/year between T1–T12 and T1–S1, respectively. Mean preoperative level of hemoglobin (Hgb) was 11.95, postoperative Hgb value was 10.02, and the decrease was significant (p < 0.05). In terms of complications, 50% (12) had broken/loosen screws, 50% (12) had undergone debridement surgery, 37.5% (9) had vacuum-assisted closure therapy, and 33.3% (8) had to get all of their implants removed.
Conclusion
We believe that our sliding growing rod technique is a new and updated surgical method that can be applied in these patient groups, facilitating the life, rehabilitation process, and daily care of MMC patients with lumbar kyphosis. This technique seems to be a safe and reliable method which preserves lung capacity and allows lengthening.
... Kyphectomy aligns the spine sagittally, enhancing sitting balance, urinary drainage, and reducing abdominal crowding, intra-thoracic pressure, and ulcers over the kyphosis apex [3]. Instrumentation extending distally to the pelvis improves correction and stabilization, yet complications and re-interventions remain high [9,10], prompting questions about surgery's real benefits and impact on quality of life [11,12]. ...
... Sacral and iliac screws have the same suboptimal anchorage in pelvic bone of poor quality, in addition to the large implant size, making its dislodgment and subcutaneous prominence along with loss of lumbosacral lordosis a serious complication [4,[28][29][30][31][32]. Ilio-sacral screws insure a low profile and stable fixation to the pelvis as shown in our cases. Distal intravertebral rod fixation as suggested by Comstock et al. [12] predisposes to suboptimal bone-to-bone contact at the kyphectomy site despite an acceptable correction of the sacral inclination angle. A further association of this technique to growing rods was reported by Alshaalan et al. [33], but kyphosis correction in their patients was incomplete and the sacrum remained retroverted, predisposing to further deterioration of the result. ...
Purpose
Lumbar kyphosis occurs in approximately 8–20% of patients with myelomeningocele (MMC). The purpose of this article is to analyze the risks and benefits of vertebrectomy and spinal stabilization in MMC children with severe lumbar kyphosis and to establish treatment guidelines.
Methods
This is an IRB-approved retrospective analysis of 59 patients with MMC who underwent kyphectomy and posterior instrumentation in three centers. Average age at surgery was 7.9 years (2 weeks–17 years). Sitting trunk position, skin status, kyphosis angle, and thoracic lordosis were analyzed preoperatively, postoperatively, and at an average follow-up of 8.2 years (range 2.5–16). The correction was maintained by applying a short posterior instrumentation in 6 patients, and extending to the pelvis in 53 cases. Pelvic fixation was achieved using the Warner and Fackler technique in 24 patients, the Dunn–McCarthy in 8, Luque–Galveston in 8, sacral screws in 2, and ilio-sacral screws in 11.
Results
Sitting position improved postoperatively in 47 of the 53 patients who underwent pelvic fixation and only in one patient with short instrumentation. All 6 patients with long instrumentation and poor postoperative sitting balance were in the Dunn–McCarthy fixation group. Skin sores at the apex of the deformity disappeared postoperatively in all patients but recurred in two patients with short instrumentations. Kyphosis angle improved from 109° (45°–170°) preoperatively to 10° (0°–45°) postoperatively and 21° (0°–55°) at last follow-up. The best results were seen in cases where a cross-k-wire fixation of the kyphectomy site was used, augmented with a long thoraco-pelvic instrumentation consisting of Luque sublaminar wires in the thoracic region and a Warner–Fackler type of pelvic fixation. Good results were also found with the bipolar technique and ilio-sacral screw fixation. Six over 24 patients with the Warner and Fackler technique showed gradual dislodgment or hardware failure, with subsequent nonunion of the kyphectomy site in four. Infection, with or without wound dehiscence and/or hardware exposure, occurred in 17 cases, necessitating hardware removal in 9 patients.
Conclusion
Lumbar kyphosis in MMC children is best managed by resection of enough vertebrae from the apex to produce a flat lumbar spine, with perfect bone-to-bone contact and long thoraco-pelvic instrumentation using the Warner and Fackler technique through the S1 foramina or the bipolar technique with ilio-sacral screw fixation. Additional local fixation of the osteotomy site using cross-wires with or without cerclage increases the stability of the construct. The majority of complications occurred in patients with short instrumentations or where residual kyphosis persisted postoperatively regardless of the type of pelvic fixation or hardware density. The Dunn–McCarthy technique for pelvic fixation following kyphectomy in MMC was less successful in producing stable pelvic fixation and should not be considered in this patient category.
... At the end of the follow-up period of this study, no single case had a loss of postoperative correction. No revision surgeries were done in our series; on the contrary, Garg et al. [3] reported 7 revisions out of 18 cases, Comstock et al. [30] reported 8 implant removals in their study. Two of the largest series in literature each reporting 24 cases had a high rate of complications were Niall et al. [2] reported 20 complications and Akbar et al. [1] reported 12 complicated cases including a perioperative death. ...
Study design : Prospective case series. Purpose : To describe a new technique for anterior column reconstruction after kyphectomy in myelomeningocele patients using titanium mesh cage and to evaluate outcomes and complications. Methods : Sixteen patients with severe dorsolumbar kyphosis 2 ry to myelomeningocele were enrolled with a mean age of 10.1 years. Kyphectomy procedure and long spinopelvic fixation were done, titanium mesh cage was used to reconstruct the anterior column. Operative time and intraoperative blood loss were calculated. Using the Cobb method, pre and postoperative measurements of local/regional kyphosis were done. Degree and mean percentage of correction were calculated. Anterior intervertebral height of the kyphotic area was also measured. The mean follow-up period was 27 months. Results : Operative time was 271.3 min ± 25, and estimated intraoperative blood loss was 781.3 mL ± 92.3. On average, 2.5 vertebrae were resected. All 16 patients were able to lie supine immediately postoperatively. The mean preoperative local/regional kyphosis was 107.5°, and 106.9° respectively, corrected to 22.5° and 28.8° postoperatively, with a mean degree of correction of 85° and 78.1° respectively. Mean preoperative anterior intervertebral height was 3.54 cm, improved to 4.64 cm postoperatively. Only 2 cases had a superficial wound infection managed conservatively. At the latest follow-up, no loss of correction pseudoarthrosis occurred, and all patients showed solid fusion. Conclusion : Titanium mesh cage is an efficient, easy method for anterior reconstruction following kyphectomy in myelomeningocele patients, to maintain postoperative correction.
Level of evidence: Therapeutic studies, Level IV study
... In the literature, complication rates in MMC patients have been reported as very high after kyphectomy. Whether a long segment fusion is performed or the growing rod lengthened, comparison of these techniques also results in 50% complications [5,22,23]. The most common complications were wound infections and implant failures. ...
Background
The aim of this study was to present clinical and radiological results of myelomeningocele (MMC) patients treated with the sliding growing rod (SGR) technique after kyphectomy.
Methods
Between 2016 and 2019, 30 patients (21 males and nine females) who underwent the SGR technique with kyphectomy and posterior instrumentation due to MMC were retrospectively reviewed. Patients’ pre- and postoperative kyphosis, scoliosis, correction rates, bleeding during surgery, blood supply during and after surgery, operation time, instrumentation levels, number of vertebrae removed, MMC onset levels, hospital stay, annual lengthening amounts, and complications were evaluated.
Results
The mean patient age was 6.9 (4–10) years. Mean preoperative kyphosis was 115° (87–166°), mean early postoperative kyphosis was 3.9° (20–10°), and final follow-up postoperative kyphosis was 5.1° (22–8°). In nine patients presenting with scoliosis, scoliosis was evaluated as 60.2° (115–35°) preoperative, as 12.9° (32–0°) early postoperative, and 15.7° (34–0°) in the final measurement. The kyphotic deformity correction rate was 96.5%, and the scoliotic deformity correction rate was 74.9%. A statistically significant difference was seen between pre- and early postoperative values in kyphosis and scoliosis measurements (p < 0.05). The annual prolongation of the patients was calculated as averages of 0.72 and 0.77 cm/year between T1–T12 and T1–S1, respectively.
Conclusion
Kyphectomy performed during the early MMC period patients appears to be an excellent method for facilitating rehabilitation and daily care of these patients. It appears that the SGR technique, which provides lung volume protection and lengthening with kyphectomy, is a safe and reliable method in patients.
Level of evidence
Level 4
... The current literature describes multiple techniques. [8,9,27,[30][31][32][33] 9. Teach children and families about fractures and related precautions. Clinical consensus 13-17 years 11 months 1. Monitor for the development or progression of scoliosis clinically, with radiographs as necessary, if indicated by physical exam. ...
Orthopedic or musculoskeletal problems are common in individuals with spina bifida. They can affect function and mobility and, in the case of spinal deformity, affect pulmonary function. We discuss the current treatment guidelines developed through collaboration with the Spina Bifida Association and the Orthopedics and Mobility working group using a specific methodology previously reported [1,2]. General considerations are discussed followed by evaluation and treatment guidelines for specific age ranges. References are provided where applicable, but where data is lacking treatment guidelines fall under the umbrella of clinical consensus. This leaves “research gaps” where areas of possible future study could be considered.
... However, no unique surgical approach has been described in the literature. Since the first description of vertebra body resection by Sharrard in 1968, numerous techniques has been described such as posterior fusion, anterior fusion, sublaminar wires, pedicle hooks, plates, cables and pedicle screws (6,13,32,33,35). However, due to rarity of the disease and the high risk of the surgery, published clinical series are scarce and no consensus has been made yet. ...
... Both short and long segment fixation has been described in the literature (6)(7)(8)12,13,33). Short segment fixation is usually done by wires and sutures around the posterior dysplastic elements especially in younger children. ...
Aim:
To define the natural course of kyphosis and to evaluate the efficiency of a new technique in surgical correction of kyphosis seen in myelomeningocele(MM) patients Material and Methods: We retrospectively reviewed our patients with MM. The rate of kyphosis, mean angle of progression and mean angle of surgical correction were evaluated. Surgical correction was achieved with the same technique in all patients; kyphectomy, short segment instrumentation with plate system and long segment instrumentation with screw-rod system.
Results:
14 patients were treated surgically and the mean age at the surgery was 39 months. The incidence of kyphosis rate was %21 in this study. The mean angle of kyphosis was 85.8°. Average angle of progression was 15.7° whereas it was 6.3° degree in patients whose kyphosis angle ≤90 and 90 degree, respectively, at birth. 14 patients were treated surgically and the mean age at the surgery was 39 months. The mean angle of correction of kyphosis was 86 degree. The most common complications were wound dehiscence and cerebro-spinal fluid leak. 1 patient died 3 months after surgery and one patient was reoperated due to pull-out of screws.
Conclusion:
Effective surgical correction of kyphosis in MM patients can be achieved with the described surgical technique even in younger ages. Prospective studies in larger study population are necessary for more accurate definition of natural history of kyphosis in MM patients.
... In the literature, serious wound problems are commonly reported in this context with 30 day hospital stays being common. [13][14][15] Some patients have required rotational flaps as secondary surgery 9,10 after midline incisions due thin scarred skin and lack of adipose tissue leading to wound breakdown and even osteomyelitis. Our biggest advantage with Y incision is muscle anc fat coverage of screw heads preventing skin irritation and pressure points. ...
... In our study, wound healing delay was observed in 3 patients but these all resolved and were failry minor for this context, with shorter recovery periods than other reports. [13][14][15] There is a high probability of dural sac injury with midline incisions which leads to the patient's hospitalization process being prolonged and increacsed costs with dura repair. Minimising this risk is another advantage of the reverse Y incision. ...
We report a new surgical incision for spinal deformity in patients who had undergone meningomyelocele closure surgery. Six patients underwent kifectomy using an inverse Y incision. They all had multiple prior operations in the lumbar region due to dural meningomyeloceles. Four of the patients were girls. The primary indication for the reverse Y incision was a T12 and above posterior fusion defect and a large lumbar posterior fusion defect. These patients included four with kyphoscoliosis, one with lordoscoliosis, and one with lumbar kyphosis. The median age of the patients was 5.52 at the time of operation. The median follow up time was 17.3 months. Under appropriate indication, the reverse Y approach offers a reliable and successful solution for surgery.
... [8][9][10][11][12][13][14] Most kyphectomy techniques require distal dissection of the bifid posterior spinal elements for the placement of implants in the thoracolumbar/pelvic regions, traversing the scarred tissue associated with the previous MMC closure, and theoretically increasing the risk of postoperative infection. To avoid this compromised area, similar techniques have been reported by centers in Canada (the so-called Halifax kyphectomy) 15 and Australia, 4 which avoid the MMC closure scar. These techniques require dissection just distal to the apex of the gibbus; in the aforementioned case series, favorable complication rates over those of other techniques were reported. ...
... These techniques require dissection just distal to the apex of the gibbus; in the aforementioned case series, favorable complication rates over those of other techniques were reported. 4,15 As symptomatic gibbus in MMC often has an early onset, procedures that maintain thoracic growth must be considered to avoid TIS. To our knowledge, a technique that combines the benefits of the Halifax kyphectomy and spine-based growing rods has not been previously described. ...
... Despite this theoretical concern, we feel that the stable anterior multilevel intravertebral fixation achieved with the Halifax kyphectomy technique allows for a cantilever to reduce both sagittal and coronal plane deformity in the lumbosacral spine, particularly when the distal extent of fixation extends to S1 or below, as is technically feasible. 15,24 According to this report, the combination of distal anterior multilevel vertebral body fixation with spinebased thoracic growing rods can successfully achieve kyphosis correction in MMC and it has the potential to reduce complication rates by minimizing blood loss, wound dehiscence, and deep wound infection while facilitating thoracic growth. Further investigation is necessary to prove whether the outcomes and the complication rates are superior to other established techniques. ...
Most kyphectomy techniques require distal dissection of the bifid posterior spinal elements for implants placement in the thoracolumbar/pelvic regions, traversing the scarred tissue associated with previous MMC closure, thereby theoretically increasing the risk of wound complications. The Halifax kyphectomy technique avoids the MMC scar but does not reliably facilitate thoracic growth for early-onset kyphosis. This study aims to report the technique and outcomes of a combined Halifax kyphectomy (resection of the apical vertebrae with distal anterior multilevel vertebral body fixation) and thoracic growing rod construct used to treat early-onset symptomatic gibbus in a patient with myelomeningocele (MMC).
Methods:
A 3-year-old girl with a thoracic MMC presented with symptomatic gibbus requiring surgical intervention. Correction by the Halifax kyphectomy technique combined with spine-based growing rods was performed.
Results:
After the correction, the skin was closed primarily without the need for any flap for coverage. No wound complications or infection occurred post-operatively. The intraoperative blood loss was 200 mL, and the surgical time was 419 minutes. No pulmonary complications occurred postoperatively. At the final follow-up at 3 years 11 months postoperatively, the child had no recurrence of the deformity.
Conclusions:
The combination of distal anterior multilevel vertebral body fixation with spine-based thoracic growing rods can successfully achieve kyphosis correction in MMC, with the potential to reduce complication rates and facilitate thoracic growth. Further investigation is necessary to prove whether the outcomes and the complication rates are superior to other established techniques.
... Lumbar kyphosis is a complex spinal deformity occurring in approximately 8% to 20% of patients with myelomeningocele [8,14,21]. Kyphosis can progress from 6°to 12°per year after birth [2,3,6,24]. Worsening kyphosis is the result of incomplete formation of the posterior spinal elements, an imbalance in the paraspinal musculature anterior to the vertebral axis, unopposed action of the psoas muscle, and neurologic deficits caused by dysraphism [5,8,15]. ...
Background:
Lumbar kyphosis is a complex spinal deformity occurring in approximately 8% to 20% of patients with myelomeningocele. The resulting gibbosity may cause pressure ulcers, difficulty lying down in the supine position and sitting on the ischia without support, decreasing quality of life (QOL). Surgery is generally performed to correct kyphosis and maintain vertebral alignment, but high complication rates have been reported. Despite satisfactory radiological results, the impact of surgery and its complications on health-related QOL (HRQOL) has not yet been established.
Questions/purposes:
Among children with myelomeningocele undergoing corrective surgery for lumbar kyphosis: (1) What is the risk of complications and reoperation after this procedure? (2) Does this procedure improve HRQOL scores in these patients?
Methods:
Between 2012 and 2013, five surgeons at three centers treated 32 patients for myelomeningocele-related kyphosis with kyphectomy and posterior instrumentation. During that period, all surgeons used the same indications for the procedure, which were progressive postural decompensation and chronic ulceration at the apex of the deformity. Data were prospectively collected, and all patients who underwent surgery were considered in this retrospective study. The legal guardians of one patient declined to sign the informed consent form, resulting in 31 patients included. A total of 9.7% (3 of 31) were lost to follow-up before the 2-year period, and the remaining 90.3% (28 of 31) were seen at a mean of 3 years (± 9 months) after surgery. The average age was 10 years, 7 months (± 21 months) at the time of surgery. The patients had a mean kyphosis angle of 130° ± 36° before surgery. This technique involved posterior fixation using S-shaped rods inserted through the foramina of S1 and pedicle screws inserted in the thoracic spine. The patients' caregivers answered both the generic and specific (neuromuscular module) Pediatric Quality of Life Inventory questionnaires preoperatively and 2 years postoperatively. The minimum clinically important difference (MCID) considered for the instruments used was 5.
Results:
Reoperation was performed in 68% of patients (19 of 28), mostly to treat deep infection. In all, 18% of patients (five of 28) underwent implant removal to control infection. Eleven percent (three of 28) had a loss of reduction and pseudarthrosis. The HRQOL increased from 71 ± 11 preoperatively to 76 ± 10 postoperatively (p < 0.001), resulting in a 5-point increase (95% CI 3 to 7) in the generic questionnaire score and from 71 ± 13 to 79 ± 11 (p < 0.001), resulting in an 8-point increase (95% CI 5 to 10) in the neuromuscular Paediatric Quality of Life Inventory questionnaire score, mainly in the physical health domain on both questionnaires.
Conclusions:
Kyphectomy was associated with a high risk of complications and reoperations and did not seem to deliver a substantial clinical benefit for patients who underwent the procedure. Most of our HRQOL score improvements were below the minimum clinically important difference for the Pediatric Quality of Life Inventory questionnaires. Although it seems that surgeons lack a better surgical alternative when facing the challenging health impairments these patients suffer, efforts should be made to improve the technique and reduce surgical complications. Additionally, patients and caregivers should be advised of the high reoperation rate and notified that the procedure may not result in a better QOL and should thus be avoided when possible. Future studies should verify whether decreasing the complication rate could imply improvement in the HRQOL of these patients after surgery.
Level of evidence:
Level IV, therapeutic study.
