Preoperative inspiratory muscle training for patients with severe scoliosis and high-risk pulmonary dysfunction in duchenne muscular dystrophy

Severe spinal collapse and pulmonary function deterioration are so common in patients with Duchenne muscular dystrophy (DMD). The limit for scoliosis surgery has been a minimum forced vital capacity (FVC) of about 30% of predicted value. DMD patients with low %FVC who might benefit from scoliosis surgery have not been being offered surgery. Between 2005 and 2007, a total of 17 DMD patients with FVC of <30% at admission underwent scoliosis surgery. %FVC at admission was 22% (range 16–27%). After admission, they were trained with inspiratory muscle training, using a pulmonary trainer (threshold IMT) for 6 weeks prior to surgery and encouraged to continue the training even after surgery and discharge. %FVC increased in all patients and noted 26% (range 22–31%) the day before surgery. The preoperative scoliosis was 97° (range 81°–130°). All patients had posterior spinal fusion and were extubated on the operative day. No patients developed any respiratory complications. No ventilatory support was needed. The mean ICU stay was 0.5 days (range 0–1 day). The postoperative scoliosis was 31° (range 18°–40°). DMD patients with severe scoliosis and FVC considered too low to permit reasonable surgical risk could undergo surgery after inspiratory muscle training, with no major complications.     

Severe restrictive lung disease and vertebral surgery in a pediatric population

The aim of this study is to describe the outcome of surgical treatment for pediatric patients with forced vital capacity (FVC) <40% and severe vertebral deformity. Few studies have examined surgical treatment in these patients, who are considered to be at a high risk because of their pulmonary disease, and in whom preoperative tracheostomy is sometimes recommended. Inclusion criteria include FVC <40%, age <19 years and diagnosis of scoliosis. The retrospective study of 24 patients with severe restrictive lung disease, who underwent spinal surgery. Variables studied were age and gender, pre- and postoperative spirometry (FVC, FEV1, FEV1/FVC), preoperative, postoperative and late use of non-invasive ventilation (BiPAP) or mechanical ventilation, associated multidisciplinary treatment, type and location of the curve, pre- and postoperative curve values, type of vertebral fusion, intra- and postoperative complications, duration of intensive care unit (ICU) stay and length of postoperative hospitalization. Mean age was 13 years (9–19) of which 13 were males and 11 females. Mean follow-up was 32 months (24–45). The etiology was neuromuscular in 17 patients and other etiologies in 7 patients. Mean preoperative FVC was 26% (13–39%). Eight patients had preoperative home BiPAP, 15 preoperative in-hospital BiPAP, and 2 preoperative mechanical ventilation. Nine patients had preoperative nutritional support. Preoperative curve value of the deformity was 88° (40°–129°). Nineteen patients with posterior fusion alone and 5 with anterior and posterior fusion were found. Mean duration of ICU stay was 5 days (1–21). Total postoperative hospital stay was 17 days (7–33). Ventilatory support in the immediate postoperative includes 16 patients requiring BiPAP and 2 volumetric ventilation. None of the patients required a tracheostomy. The intraoperative complications include one death due to acute heart failure; immediate postoperative, four respiratory failures (2 required ICU readmission) and one respiratory infection; and other minor complications occurred in six patients. Overall, 58% of patients had complications. Percentage of angle correction was 56%. After a follow-up of 30 months, FVC was 29% (13–50%). In conclusion, corrective scoliosis surgery in pediatric patients with severe restrictive lung disease is well tolerated, but the management of this population requires extensive experience with the vertebral surgery involved, and a multidisciplinary approach that includes pulmonologists, nutritionists and anesthesiologists. Currently, there is no indication for routine preoperative tracheostomy.     

Morbidity and radiographic outcomes of severe scoliosis of 90° or more: a comparison of hybrid with total pedicle screw instrumentation

Objectives

Untreated severe scoliosis is associated with increased mortality and remains a significant surgical challenge. Few studies have reported mortality after the surgical treatment of severe scoliosis beyond a 2-year follow-up. The objectives of this study were to evaluate mortality beyond standard 2-year follow-up and compare radiographic outcomes using hybrid or pedicle screw instrumentation for severe scoliosis.

Methods

We evaluated 32 consecutive patients [11 males, mean age at surgery 15.3 (range 10.7–20.7) years] operated for a scoliosis of 90° or more using either hybrid (n = 15) or pedicle screw (n = 17) instrumentation. The follow-up time averaged 2.9 (2.0–6.6) years for radiographic and quality of life measurements and 5.5 years (2.0–9.0) years for mortality data. Of these patients, one had adolescent idiopathic scoliosis, three secondary scoliosis, and 28 neuromuscular scoliosis. Twelve patients in the hybrid and two patients in the pedicle screw groups underwent anteroposterior surgery (p < 0.001), and three patients in both groups had an apical vertebral column resection.

Results

One (3.1 %) patient died during follow-up for severe pneumonia. Preoperatively, the mean magnitude of the major curve was 109° (90°–127°) in the hybrid and 100° (90°–116°) in the pedicle screw groups (p = 0.015), and was corrected to 45° (19°–69°) in the hybrid and 27° (18°–40°) in the pedicle screw groups at the 2-year follow-up (p < 0.001), with a mean correction of the major curve of 59 % (37–81 %) in the hybrid versus 73 % (60–81 %) in the pedicle screw groups, respectively (p = 0.0023). There were six postoperative complications, including one transient spinal cord deficit necessitating reoperation in the hybrid group as compared with five complications in the pedicle screw group (p = 0.53).

Conclusions

The mid-term mortality rate after the surgical treatment of severe scoliosis was low. Severe scoliosis can be treated safely with significantly better correction of the spinal deformity using pedicle screws than hybrid instrumentation.     

Two-year results for scoliosis secondary to Duchenne muscular dystrophy fused to lumbar 5 with segmental pedicle screw instrumentation

Background

Instrumentation and fusion to the sacrum/pelvis has been a mainstay in the surgical treatment of scoliosis in patients with Duchenne muscular dystrophy since the development of the intrailiac post. It is recommended for correcting pelvic obliquity. However, caudal extent of instrumentation and fusion has remained a matter of considerable debate. This study was performed to determine the efficacy and safety of stopping segmental pedicle screw constructs at L5 during surgical treatment of scoliosis associated with Duchenne muscular dystrophy (DMD).

Methods

From May 2005 to June 2007, a total of 20 consecutive patients underwent posterior spinal fusion and segmental pedicle screw instrumentation only to L5 for scoliosis secondary to DMD. All patients had progressive scoliosis, difficulty sitting, and back pain before surgery. A minimum 2-year follow-up was required for inclusion in this study. Assessment was performed clinically and with radiological measurements. The Cobb angles of the curves and spinal pelvic obliquity were measured on the coronal plane. Thoracic kyphosis and lumbar lordosis were measured on the sagittal plane. These radiographic assessments were performed before surgery, immediately after surgery, and at a 3-month interval thereafter. The operating time, blood loss, and complications were evaluated. Patients were questioned about whether they had difficulty sitting and felt back pain before surgery and at 6 weeks, 1 year, and 2 years after surgery.

Results

A total of 20 patients, aged 11–17 years, were enrolled. The average follow-up period was 37 months. Preoperative coronal curves averaged 70° (range 51°–85°), with a postoperative mean of 15° (range 8°–25°) and a mean of 17° (range 9°–27°) at the last follow-up. Pelvic obliquity improved from 13° (range 7°–15°) preoperatively to 5° degrees (range 3°–8°) postoperatively and 6° (range 3°–9°) at the last follow-up. Good sagittal plane alignment was recreated and maintained. Only a small loss of correction of scoliosis and pelvic obliquity was noted. The mean operating time was 271 min (range 232–308 min). The mean intraoperative blood loss was 890 ml (range 660–1260 ml). The mean total blood loss was 2100 ml (range 1250–2880 ml). There was no major complication. All patients reported that difficulty sitting and back pain were alleviated after surgery.

Conclusion

Segmental pedicle screw instrumentation and fusion only to L5 is safe and effective in patients with DMD scoliosis of <85° and pelvic obliquity of <15°. Good sagittal plane alignment was achieved and maintained. All patients benefited from surgery in terms of improved quality of life. There was no major complication.     

Segmental pedicle screws instrumentation and fusion to L5 for spinal deformity secondary to Duchenne muscular dystrophy: results with a minimum of 2 years follow-up

Background

Traditional treatment recommendations in the surgical treatment of scoliosis in Duchenne muscular dystrophy have included instrumentation and fusion to the sacrum/pelvis to correct pelvic obliquity and to restore the sitting balance of the trunk. However, caudal extent of instrumentation and fusion has remained a matter of considerable debate. This study was performed to determine the efficacy and safety of stopping segmental pedicle screw constructs at L5 in the surgical treatment of scoliosis in Duchenne muscular dystrophy (DMD), with mild pelvic obliquity (<15°).

Materials and methods

From May 2005 to June 2007, a total of 22 consecutive patients underwent posterior spinal fusion and segmental pedicle screw instrumentation only to L5 for scoliosis secondary to DMD. A minimum 2-year follow-up was required for inclusion in this study. Assessment was performed clinically and with radiologic measurements. Radiologic measurements included the Cobb angles of the curves in the coronal plane, thoracic kyphosis and lumbar lordosis in the sagittal plane, and pelvic obliquity. The operating time, blood loss, and complications were evaluated.

Results

Twenty patients, aged 11–17, were enrolled. The average follow-up period was 35 months. Preoperative coronal curves averaged 70° (range: 51–85°), with a postoperative mean of 15° (range: 8–25°) and 17° (range: 9–27°) at the last follow-up. Pelvic obliquity improved from 13° (range: 7–15°) preoperatively to 5° (range: 3–8°) postoperatively and 6° (range: 3–9°) at the last follow-up. Good sagittal plane alignment was recreated and maintained. No loss of correction of scoliosis and pelvic obliquity was noted. The mean operating time was 271 min (range: 232–308 min). The mean intraoperative blood loss was 890 ml (range: 660–1260 ml). The mean total blood loss was 2100 ml (range: 1250–2880 ml).There was no major complication.

Conclusion

Segmental pedicle screw instrumentation and fusion to L5 is effective and safe in patients with scoliosis secondary to DMD without significant pelvic obliquity initially and long term, obviating the need for fixation to the sacrum/pelvis. There was no major complication.     

Autogenous iliac crest bone graft versus banked allograft bone in scoliosis surgery in patients with Duchenne muscular dystrophy

A total of 36 consecutive nonambulatory DMD patients underwent scoliosis surgery. Patients were divided into two groups: the autogenous iliac crest bone graft group (the ICBG group; 20 patients) and the allogenous bone graft group (the ALBG group; 16 patients). The mean preoperative curves measured 87° and 31° at the last follow-up in the ICBG group and 83° and 28° in the ALBG group. In the ICBG group, three (15%) patients had intraoperative sacroiliac joint penetration, five (25%) had iliac crest inner cortex penetration and three (15%) had postoperative prolonged wound drainage at the donor site. At three months after surgery, donor site pain caused by bone harvest was found in 50% with severe pain limiting their physical function and causing difficulties in sitting in a wheelchair in 40% of the patients, whereas patients in the ALBG group returned to their preoperative level of function soon after surgery.     

Comparison of survival analysis between surgical and non-surgical treatments in Duchenne muscular dystrophy scoliosis

BACKGROUND CONTEXTThere are still controversies about the effects of spinal surgeries for Duchenne muscular dystrophy (DMD) scoliosis on functional outcome, respiratory function, and the survival rate.PURPOSEThe purpose of this retrospective investigation was to compare the clinical course over time between the patients who were treated surgically and those who were treated nonsurgically. Through this comparison, we tried to determine how surgical treatment could affect the functional status, pulmonary function, and survival rate in patients with DMD scoliosis.STUDY DESIGN/SETTINGSingle-center retrospective cohort study.PATIENT SAMPLEWe reviewed the clinical data of 199 male patients with DMD scoliosis who were followed up at our center for an average of 6.4 years between 2003 and 2017.OUTCOME MEASURESThe basic radiologic parameters evaluated include the Cobb angle and pelvic obliquity on a whole spine X-ray. Further, the Swinyard scale for functional status, forced vital capacity (FVC) for respiratory function, and mortality were compared between the surgical group and nonsurgical group.METHODSThe radiologic parameters and Swinyard scale stage were compared between the surgical group and nonsurgical group at baseline and 2, 5, and 10 years. For the FVC, serial changes every year were investigated in both groups. Mortality was surveyed between the surgical group and nonsurgical group.RESULTSOf the 199 patients, 99 patients underwent the instrumented spinal fusion surgery and 100 patients in the nonsurgical group opted for conservative management. Radiologic results of the two groups were not different at baseline, but during the follow-up periods, the surgical group demonstrated better Cobb angles and pelvic obliquities. The surgical group showed a better functional status than did the nonsurgical group (6.7±0.9 versus [vs.] 7.2±0.7, p<.001). These functional differences between the groups were continuously observed during the follow-up period. Similarly, the FVC at baseline was higher in the surgical group than in the nonsurgical group (1005.7±421.4 mL vs. 787.3±574.1 mL, p=.005). Although FVC in the nonsurgical group consistently decreased during the follow-up (4.8% decrease/year), FVC in the surgical group increased up to the 2-year follow-up period compared with the baseline value and decreased during the follow-up period (2.8% decrease/year). Mortality was higher in the nonsurgical group than in the surgical group (n=22/100, 22.0% vs. n=8/99, 8.1%; p<.001) during an average follow-up duration of 6.4 years. Mean survival was longer in the surgical group than in the nonsurgical group (12.2 years vs. 8.3 years, hazard ratio=2.43, p=.02).CONCLUSIONSSpinal surgery for DMD scoliosis improved the FVC for approximately 2 years postoperatively compared to non-surgical treatment. The surgical group had a better functional status and FVC at baseline than the non-surgical group. The positive effect of surgical treatment on the FVC is owing to scoliosis correction, which delayed the decrease of FVC and consequently extended the survival rate of the patients with DMD scoliosis.     

Comparison of two techniques in hemivertebra resection: transpedicular eggshell osteotomy versus standard anterior–posterior approach

Hemivertebrae are the most frequent cause of congenital scoliosis. They have growth potential similar to normal vertebra, creating wedge-shaped deformity that progresses during further spinal growth. This study aims to compare the interventions for hemivertebrae resections in congenital scoliosis by posterior transpedicular eggshell osteotomy approach only and with combined anterior and posterior approach. Ten patients who underwent hemivertebra resection between 1995 and 2002 were evaluated by retrospective charts and radiographic views. Mean follow-up time was 32 months (range 12–48). Except one patient, all were female and mean age at surgery was 7 years (range 3–13). Transpedicular eggshell osteotomy was performed in five patients (group I) and by combined anterior and posterior approach in five patients (group II). All patients had a single non-incarcerated hemivertebra and the locations of the hemivertebra were Th7, Th8, Th11, Th12, L2 in group I and Th7, Th10, L1, L4, L5 in group II. The average operation time was 3 h in group I and 6 h in group II (P < 0.05). The number of instrumented vertebrae was 4 for group I and 6 for group II. The mean blood loss during the operation for groups I and II was 354 and 500 cc, respectively (P < 0.05). The mean Cobb angle was measured as 37° before surgery, 18° after surgery and 21° at the latest follow-up for group I; 32°, 14° and 17°for group II. The correction ratio was 51% in group I and 56% (P > 0.05). The loss of correction was 8% in group I and 9% in group II (P > 0.05). No intra-operative complications were noted and no implant failure was verified at the final radiographic evaluations. Transpedicular eggshell osteotomy is a technique that should be considered for older patients who have congenital scoliosis with multiplanar spinal abnormalities. It is a technically demanding procedure that provides an effective correction in selected patients.     

The effect of posterior spinal fusion on respiratory function in Duchenne muscular dystrophy

Purpose

Posterior instrumented spinal fusion is indicated for progressive scoliosis that develops in Duchenne muscular dystrophy (DMD) patients. Whilst spinal fusion is known to improve quality of life, there is inconsistency amongst the literature regarding its specific effect on respiratory function. Our objective was to determine the effect of scoliosis correction by posterior spinal fusion on respiratory function in a large cohort of patients with DMD. Patients with DMD undergoing posterior spinal fusion were compared to patients with DMD not undergoing surgical intervention.

Methods

An observational study of 65 patients with DMD associated scoliosis, born between 1961 and 2001: 28 of which underwent correction of scoliosis via posterior spinal fusion (Surgical Group) and 37 of which did not undergo surgical intervention (Non-Surgical Group). Pulmonary function was assessed using traditional spirometry. Comparisons were made between groups at set times, and by way of rates of change over time.

Results

There was no correlation between the level of respiratory dysfunction and the severity of scoliosis (as measured by Cobb angle) for the whole cohort. The Surgical Group had significantly worse respiratory function at a comparable age pre-operatively compared to the Non-Surgical Group, as measured by per cent predicted forced vital capacity (p = 0.02) on spirometry. The rate of decline of forced vital capacity and per cent predicted forced vital capacity was not slowed following surgery compared to the non-operated cases. There was no significant difference in survival between the two groups.

Conclusions

Severity of scoliosis was not a key determinant of respiratory dysfunction. Posterior spinal fusion did not reduce the rate of respiratory function decline. These two points suggest that intrinsic respiratory muscle weakness is the main determinant of decline in respiratory function in DMD.     

Non-invasive positive pressure ventilation to facilitate the post-operative respiratory outcome of spine surgery in neuromuscular children

Purpose

Scoliosis surgery may be associated with a high morbidity and even mortality in children with non-idiopathic scoliosis. The aim of the study was to report our experience with a pre-operative training to non-invasive positive pressure ventilation (NPPV) and a mechanical insufflator–exsufflator (MI–E) device to improve the post-operative respiratory outcome of children scheduled for scoliosis surgery.

Methods

Consecutive patients with non-idiopathic scoliosis undergoing posterior arthrodesis were trained to NPPV and MI–E before intervention. NPPV and MI–E were performed immediately after extubation. Length of intubation and intensive care unit (ICU) stay, duration of NPPV, and respiratory complications were assessed.

Results

Thirteen patients participated in the training (mean age 13.9 ± 2.6, mean vital capacity 52.3 ± 15.4 % predicted). The patients had severe respiratory muscle weakness with a mean sniff oesophageal pressure of 35.8 ± 14.2 cmH₂O (50 % predicted) and a mean gastric pressure during a cough of 31.9 ± 7.8 cmH₂O (30 % predicted). The mean length of intubation was 19.9 ± 12.3 h with a mean length of ICU stay of 2.5 ± 2.5 days. NPPV was used during a mean of 2.7 ± 1.9 days after surgery. No respiratory complication was observed. One patient died 3 months after surgery from multi-organ failure of non-respiratory origin.

Conclusions

No respiratory complications were observed after scoliosis correction surgery in children with non-idiopathic scoliosis after pre-operative training and post-operative use of NPPV and MI–E, underlying the interest of this management in these high-risk patients.

     

Minimally invasive decompression surgery for lumbar spinal stenosis with degenerative scoliosis: Predictive factors of radiographic and clinical outcomes

There is ongoing controversy regarding the most appropriate surgical treatment for lumbar spinal stenosis (LSS) with concurrent degenerative lumbar scoliosis (DLS): decompression alone, decompression with limited spinal fusion, or long spinal fusion for deformity correction. The coexistence of degenerative stenosis and deformity is a common scenario; Nonetheless, selecting the appropriate surgical intervention requires thorough understanding of the patients clinical symptomatology as well as radiographic parameters. Minimally invasive (MIS) decompression surgery was performed for LSS patients with DLS. The aims of this study were (1) to investigate the clinical outcomes of MIS decompression surgery in LSS patients with DLS, and (2) to identify the predictive factors for both radiographic and clinical outcomes after MIS surgery. 438 consecutive patients were enrolled in this study. Inclusion criteria was evidence of LSS and DLS with coronal curvature measuring greater than 10°. The Japanese Orthopaedic Association (JOA) score, JOA recovery rate, low back pain (LBP), and radiographic features were evaluated preoperatively and at over 2 years postoperatively. Of the 438 patients, 122 were included in final analysis, with a mean follow-up of 2.4 years. The JOA recovery rate was 47.6%. LBP was significantly improved at final follow-up. Cobb angle was maintained for 2 years postoperatively (p = 0.159). Clinical outcomes in foraminal stenosis patients were significantly related to sex, preoperative high Cobb angle and progression of scoliosis (p = 0.008). In the severe scoliosis patients, the JOA recovery was 44%, and was significantly depended on progression of scoliosis (Cobb angle: preoperation 29.6°, 2-years follow-up 36.9°) and mismatch between the pelvic incidence (PI) and the lumbar lordosis (LL) (preoperative PI–LL 35.5 ± 21.2°) (p = 0.028). This study investigated clinical outcomes of MIS decompression surgery in LSS patients with DLS. The predictive risk factors of clinical outcomes were severe scoliosis, foramina stenosis, progressive scoliosis and large mismatch of PI–LL.     

Spinal fusion in patients with Duchenne's muscular dystrophy and a low forced vital capacity

Traditionally, spinal fusion has been denied to patients with scoliosis secondary to Duchenne's muscular dystrophy (DMD) when their forced vital capacity (FVC) is less than 30-40% of predicted values (PFVC). The reasons for this decision are a theoretically increased risk of adverse events from a prolonged anaesthetic and extensive surgery. This paper presents a retrospective analysis of 30 patients with DMD scoliosis who underwent posterior spinal fusion at the Royal National Orthopaedic Hospital. Two subgroups of patients were compared: those with more than 30% PFVC (17 patients) and those with less than 30% PVFC (13 patients). One patient in each group required a temporary tracheotomy and there were nine complications in total. The post-operative stay for patients in each group was similar (24 days in the >30% group, 20 days in the <30% group) and the complication rate was comparable with other published series. We conclude that spinal fusion can be offered to patients with DMD even in the presence of a low FVC.     

The clinical outcomes of early internal fixation for undisplaced femoral neck fractures and early full weight-bearing in elderly patients

Objective

The purpose of the present study was to evaluate the clinical outcomes of early internal fixation for undisplaced femoral neck fractures and early full weight-bearing in patients aged 65 years and older.

Patients and methods

The records of consecutive patients who underwent surgery for undisplaced femoral neck fractures between 1999 and 2011 were retrospectively reviewed. The patients underwent the surgery as early as possible, and allowed early full weight-bearing. The interval between initial injury and surgery, time to admission and operation, operation time, decrease in hemoglobin, the postoperative day starting to walk, postoperative walking status, and the incidence of any secondary procedures were evaluated. The average patient age was 77.5 years and the average duration of postoperative follow-up was 46.8 months. The patients were divided to two groups to determine the effect of early operation: the early operation group within 24 h on admission, and the late operation group done 24 h after admission.

Results

Eighty-six percent of surgeries were performed within 48 h of admission. The average operation time was 46 min (range 20–95 min). Transfusions were performed in 6.9 % (4/58) of patients. The mortality rate was 6.9 %, and the rate of complications was 9.3 % (5/54): four cases of avascular necrosis (AVN) and one case of fixation failure. The rate of secondary procedures was 7.4 % (4/54). Seventy-two percent (39/54) of patients recovered their postoperative walking ability to pre-injury levels.

Conclusion

This study demonstrated that early internal fixation of undisplaced femoral neck fractures in elderly patients produced satisfactory clinical outcomes.     

Halo-gravity traction combined with assisted ventilation: an effective pre-operative management for severe adult scoliosis complicated with respiratory dysfunction

Purpose

To investigate the change of pulmonary function in adult scoliosis patients with respiratory dysfunction undergoing HGT combined with assisted ventilation.

Methods

21 adult patients were retrospectively reviewed with a mean age of 26.2 years. Inclusion criteria were as follows: age over 18 years old; coronal Cobb angle greater than 100°; with respiratory failure; and duration of HGT more than 1 month. All patients underwent respiratory training.

Results

The Cobb angle averaged 131.21° and was reduced to 107.68° after HGT. Significantly increased mean forced vital capacity (FVC) was found after HGT (P = 0.003) with significantly improved percent-predicted values for FVC (P < 0.001). Meanwhile, significantly increased forced expiratory volume in 1 s (FEV1) was also observed (P < 0.001) with significantly improved percent-predicted values for FEV1 (P = 0.003) after HGT.

Conclusion

The results of our study revealed that combined HGT and assisted ventilation would be beneficial to pulmonary function improvement in severe adult scoliosis cases, most of which were young adults.

     

Severe idiopathic scoliosis with respiratory insufficiency treated with preoperative traction and staged anteroposterior spinal fusion with a 2-level apical vertebrectomy

Background contextSevere adolescent idiopathic scoliosis with respiratory insufficiency is infrequently seen in North America currently.PurposeTo present the case of a teenager from Moscow, Russia who was referred to our center with a severe scoliosis and respiratory compromise.Study design/settingA case report on the evaluation and surgical treatment of a severely deformed teenager.MethodsA 14+10-year-old was referred to our center for treatment of a 149° thoracic scoliosis. Preoperative pulmonary function tests (PFTs) revealed severe restrictive disease with a forced vital capacity (FVC) of 1.3 L (34% predicted) and a forced expiratory volume in 1 second (FEV₁) of 0.99 L (31% predicted). She underwent a 2-stage anterior and posterior 2-level vertebral column resection (VCR) with preoperative and in between anterior and posterior stage perioperative halo-gravity traction.ResultsHer thoracic scoliosis was corrected to 48° over 3 years postoperative. Her 3-year follow-up PFT revealed an FVC of 1.85 L (52% predicted) and an FEV₁ of 1.6 L (50% predicted).ConclusionsA staged anterior and posterior VCR with intervening halo-gravity traction is a viable option to treat severe scoliosis in patients with restrictive pulmonary function.     

Evaluation of thoracic factors after scoliosis surgery in patients with both scoliosis and pectus excavatum

Purpose

Pectus excavatum can negatively impact cardiac function during scoliosis surgery. Several authors reported severe hypotension associated with the prone position during scoliosis surgery in children that had both scoliosis and pectus excavatum. However, we could find no studies that evaluated the change in the thoracic factors, such as sternal tilt angle and Haller index after scoliosis surgery in patients with both scoliosis and pectus excavatum. The purpose of this study is to evaluate the change in thoracic factors after surgical treatment for scoliosis associated with pectus excavatum.

Methods

We performed a retrospective review on 20 patients (10 males and 10 females) who underwent surgical treatment for scoliosis associated with pectus excavatum from August 2004 to April 2014 in our hospital. We investigated the scoliosis diagnosis, preoperative and postoperative Cobb and thoracic kyphosis (TK) angles, the change in TK after surgery and thoracic factors, including the AP and transverse diameters of the chest, the sternal tilt angle, and Haller index.

Results

Patient mean age was 13.2 years old (4–27 years old) at surgery. Types of scoliosis were idiopathic in 8 patients, syndromic in 10, and neuromuscular in 2. The mean Cobb angles were 72.1° preoperatively and 19.0° postoperatively. Curve locations were thoracic in 13 patients, thoracolumbar in 4, and lumbar in 3. Surgical treatment of pectus excavatum was performed in 9 patients (45 %) before scoliosis treatment. Mean sternal tilt angles were 11.5° preoperatively and 11.1° postoperatively. Mean Haller indices were 4.8 preoperatively and 5.3 postoperatively. This was especially true for syndromic or neuromuscular scoliosis and thoracolumbar/lumbar curve type patients in which scoliosis surgery tended to worsen the Haller index.

Conclusion

The Haller index increased postoperatively in 11 of 20 patients, which means sternal depression deteriorated after scoliosis surgery in about 50 % of patients. We suggest that surgeons fully assess the thoracic factors in patients with scoliosis and pectus excavatum prior to performing scoliosis surgery and carefully monitor their patient’s general condition during surgery.

     

Family history and its association to curve size and treatment in 1,463 patients with idiopathic scoliosis

Purpose

To study family history in relation to curve severity, gender, age at diagnosis and treatment in idiopathic scoliosis.

Methods

A self-assessment questionnaire on family history of scoliosis was administered to 1,463 untreated, brace or surgically treated idiopathic scoliosis patients.

Results

Out of the 1,463 patients, 51 % had one or more relatives with scoliosis. There was no significant difference between females and males, nor between juvenile and adolescent study participants in this respect (p = 0.939 and 0.110, respectively). There was a significant difference in maximum curve size between patients with one or more relatives with scoliosis (median 35°, interquartile range 25) and patients without any relative with scoliosis (median 32°, interquartile range 23) (p = 0.022). When stratifying patients according to treatment (observation, brace treatment or surgery), we found that it was more common to have a relative with scoliosis among the treated patients (p = 0.011). The OR for being treated was 1.32 (95 % CI 1.06–1.64) when the patient had a relative with scoliosis, compared to not having.

Conclusions

Larger curve sizes were found in patients with a family history of scoliosis than in the ones without. No relation between family history and gender or between family history and age at onset of idiopathic scoliosis was found. Although the presence of a family history of scoliosis may not be a strong prognostic risk factor, it indicates that these patients are at higher risk of developing a more severe curve.     

The use of posterior vertebral column resection in the management of severe posttuberculous kyphosis: a retrospective study and literature review

Purpose

We present a retrospective study of 15 cases with severe posttuberculous kyphosis of thoracolumbar region that underwent posterior vertebral column resection.

Methods

From 2004 to 2009, 15 consecutive patients with posttubercular kyphotic deformity underwent posterior vertebral resection osteotomy. Six subjects were females and nine were males with an average age of 35.8 years (range 20–60 years) at the time of surgery. None of the patients had neurological deficits. The mean preoperative visual analogue scale was 8.7 (range 3–9), and the average preoperative Oswestry Disability Index was 46.5 (range 40–56).

Results

The average duration of postoperative follow-up was 36.1 ± 10.7 months (range 24–62 months). The number of vertebra resected was 1.3 (range 1–2) on average. There were ten patients with one-level osteotomy and five patients with two-level osteotomy. The average operation time was 446.0 ± 92.5 min (range 300–640 min) with an average blood loss of 1,653.3 ± 777.9 ml (range 800–3000 ml). The focal kyphosis before surgery averaged 92.3 ± 8.9° (range 74–105°), and the kyphotic angle decreased to 34.5 ± 8.7° on average after the surgical correction. The average kyphotic angle at the last follow-up was 36.9 ± 8.5°, loss of correction was 2.4 ± 1.4° on average. All patients postoperatively received bony fusion within 6–9 months.

Conclusions

Our results showed that although posterior vertebral resection is a highly technical procedure, it can be used safely and effectively in the management of severe posttuberculous kyphosis. It is imperative that operations be performed by an experienced surgical team to prevent operation-related complications.     

Anterior surgery for adolescent idiopathic scoliosis

Anterior open scoliosis surgery using the dual rod system is a safe and rather effective procedure for the correction of scoliosis (50–60 %). Thoracic hypokyphosis and rib hump correction with open anterior rather than posterior instrumentation appear to be the better approaches, although the latter is somewhat controversial with current posterior vertebral column derotation devices. In patients with Risser grade 0, hyperkyphosis and adding-on may occur with anterior thoracic spine instrumentation. Anterior thoracoscopic instrumentation provides a similar correction (65 %) with good cosmetic outcomes, but it is associated with a rather high risk of instrumentation (pull-out, pseudoarthrosis) and pulmonary complications. Approximately 80 % of patients with adolescent idiopathic scoliosis (AIS) curves of >70° have restrictive lung disease or smaller than normal lung volumes. AIS patients undergoing anterior thoracotomy or anteroposterior surgery will demonstrate a significant decrease in percentage of predicted lung volumes during follow-up. The thoracoabdominal approach and thoracoscopic approach without thoracoplasty do not produce similar changes in detrimental lung volume. In patients with severe AIS (>90°), posterior-only surgery with TPS provides similar radiographic correction of the deformity (44 %) with better pulmonary function outcomes than anteroposterior surgery. Vascular spinal cord malfunction after segmental vessel ligation during anterior scoliosis surgery has been reported. Based on the current literature, the main indication for open anterior scoliosis instrumentation is Lenke 5C thoracolumbar or lumbar AIS curve with anterior instrumentation typically between T11 and L3.