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1.  The efficacy and complications of posterior hemivertebra resection 
European Spine Journal  2011;20(10):1692-1702.
There have been several reports on hemivertebra resection via a posterior-only procedure. However, the number of reported cases is small, and various types of instrumentation have been used. In our study, we retrospectively investigated 56 consecutive cases of congenital scoliosis that were treated by posterior hemivertebra resection with transpedicular instrumentation. Radiographs were reviewed to determine the type and location of the hemivertebra, the coronal curve magnitude and the sagittal alignment pre-operatively, post-operatively and at the latest follow-up. Radiographs were also used to assess implant failure and inter-body fusion. Surgical reports and patient charts were reviewed to record any peri-operative complications. Fifty-eight posterior hemivertebrae resections from 56 patients aged 1.5–17 years with fully segmented non-incarcerated hemivertebra were evaluated. The average age at surgery was 9.9 years (1.5–17 years). The average follow-up was 32.9 months (24–58 months). The mean fusion level was 5.0 segments (2–11 segments). There was a mean improvement of 72.9% in the segmental scoliosis, from 42.4° before surgery to 12.3° at the time of the latest follow-up, and there was a mean improvement of 70% in segmental kyphosis from 42.0° to 14.5° over the same time period. The thoracic kyphosis (T5–T12) averaged 10.8° before surgery and 23.9° at the latest follow-up. The lumbar lordosis (L1–S1) averaged −52.8° before surgery and −51.6° at the latest follow-up. Two cases with neurological claudications had complete recovery immediately after the surgery. There was one case of delayed wound healing, two fractures of the pedicle at the instrumented level, two rod breakages and one proximal junction kyphosis that required revision. There were no neurological complications. Radiolucent gaps were found in the residual space after resection on the lateral view in five cases, without any sign of implant failure or correction loss. Our results show that one-stage posterior hemivertebra resection with transpedicular instrumentation can achieve excellent correction, 360° decompression and short fusion without neurological complications. Pedicle cutting still remains a challenge in younger children when using bisegmental instrumentation. In addition, the radiolucent gaps in the residual space require further investigation.
PMCID: PMC3175869  PMID: 21318279
Congenital scoliosis; Hemivertebra; Resection; Posterior surgery
2.  Severe kyphoscoliosis after primary Echinococcus granulosus infection of the spine 
European Spine Journal  2010;19(9):1415-1422.
A primary Echinococcus granulosus infection of the spine involving the vertebrae T8 and T9 of a 6-year-old child was treated elsewhere by thoracotomy, partial corporectomy, multiple laminectomies and uninstrumented fusion. Owing to inappropriate stabilization, severe deformity developed secondary to these surgeries. X-rays, CT and MRI scans of the spine revealed a severe thoracic kyphoscoliosis of more than 100° (Fig. 1) and recurrence of Echinococcus granulosus infection. The intraspinal cyst formation was located between the stretched dural sac and the vertebral bodies of the kyphotic apex causing significant compression of the cord (Figs. 2, 3, 4). A progressive neurologic deficit was reported by the patient. At the time of referral, the patient was wheelchair bound and unable to walk by herself (Frankel Grade C). Standard antiinfectious therapy of Echinococcus granulosus requires a minimum treatment period of 3 months. This should be done before any surgical intervention because in case of a rupture of an active cyst, the delivered lipoprotein antigens of the parasite may cause a potentially lethal anaphylactic shock. Owing to the critical neurological status, we decided to perform surgery without full length preoperative antiinfectious therapy. Surgical treatment consisted in posterior vertebral column resection technique with an extensive bilateral costotransversectomy over three levels, re-decompression with cyst excision around the apex and multilevel corporectomy of the apex of the deformity. Stabilisation and correction of the spinal deformity were done by insertion of a vertebral body replacement cage anteriorly and posterior shortening by compression and by a multisegmental pedicle screw construct. After the surgery, antihelminthic therapy was continued. The patients neurological deficits resolved quickly: 4 weeks after surgery, the patient had Frankel Grade D and was ambulatory without any assistance. After an 18-month follow-up, the patient is free of recurrence of infection and free of neurologically deficits (Frankel E). This case demonstrates that inappropriate treatment—partial resection of the cyst, inappropriate anterior stabilization and posterior multilevel laminectomies without posterior stabilization—may lead to severe progressive kyphoscoliotic deformity and recurrence of infection, both leading to significant neurological injury presenting as a very difficult to treat pathology.Fig. 1X-rays of the patient showing a kyhoscoliotic deformity. a ap view, b lateral viewFig. 2CT reconstruction of the whole spine showing the apex of the deformity is located in the area of the previous surgeriesFig. 3Sagittal CT-cut showing the bone bloc at the apex with a translation deformityFig. 4Sagittal T2-weighted MRI image showing the cystic formation at the apex
PMCID: PMC2989289  PMID: 20514501
Echinococcus granulosus; Infection; Thoracic spine; Kyphotic deformity; Neurologic deficit
3.  Posterior hemivertebra resection with bisegmental fusion for congenital scoliosis: more than 3 year outcomes and analysis of unanticipated surgeries 
European Spine Journal  2012;22(2):387-393.
Until now there have been many reports on hemivertebra resection. But there were no large series on the posterior hemivertebra resection with bisegmental fusion. This is a retrospective study to evaluate the surgical outcomes of posterior hemivertebra resection only with bisegmental fusion for congenital scoliosis caused by fully segmented non-incarcerated hemivertebra.
In our study, 36 consecutive cases (19 males, 17 females) diagnosed with congenital scoliosis, resulting from fully segmented non-incarcerated hemivertebra, treated by posterior hemivertebra resection with bisegmental fusion were investigated retrospectively, with at least a 3 year follow-up period (36–106 months).
The total number of resected hemivertebra was 36. Mean operation time was 188.6 min with average blood loss of 364.2 ml. The segmental scoliosis was corrected from 36.6° to 5.1° with a correction rate of 86.1 %, and segmental kyphosis(difference to normal segmental alignment) from 21.2° to 5.8° at the latest follow-up. The correction rate of the compensatory cranial and caudal curve is 76.4 and 75.1 %. Unanticipated surgeries were performed on eight patients, including one delayed wound healing, two pedicle fractures, one progressive deformity and four implants removals.
Posterior hemivertebra resection with bisegmental fusion allows for early intervention in very young children. Excellent correction can be obtained while the growth potential of the unaffected spine could be preserved well. However, it is not indicated for the hemivertebra between L5 and S1. The most common complication of this procedure is implant failure. Furthermore, in the very young children we noted that although solid fusion could be observed in the fusion level, implants migration may still happen during the time of adolescence, when the height of the body developed rapidly. So a close follow-up is necessary.
PMCID: PMC3555607  PMID: 23179979
Posterior instrumentation; Bisegmetal fusion; Hemivertebra
4.  Late onset Pott’s paraplegia in patients with upper thoracic sharp kyphosis 
International Orthopaedics  2011;36(2):381-385.
The purpose of this study was to determine the clinical results of patients with late onset upper thoracic sharp Pott’s kyphosis and to predict the prognosis for Pott's paraplegics.
The study included five patients who developed late onset upper thoracic (T1–T4) sharp Pott’s kyphosis /kyphoscoliosis within a period from 19 to 37 years after the active disease was healed. The kyphosis angle of the patients ranged from 95° to 105°. Among them, three patients suffered onset of paraplegia ranging from 26 to 31 years after spinal tuberculosis was healed. The duration of neurological deterioration before surgery ranged from four to five years. All patients underwent decompressive surgery with an attempt to correct the curve. Neurological status was evaluated using the ASIA impairment classification and the motor score.
Postoperatively, kyphosis correction ranged from 20° to 30° for five patients. No neurological deficit occurred in two patients with normal neurological status. Two ASIA D paraplegics remained unchanged after surgery and no further improvement was found at one year follow-up. One ASIA C paralysis deteriorated neurologically to ASIA B after surgery and persisted to a deterioration of neurological status at one year follow–up.
Upper thoracic sharp Pott's kyphosis and neurological deficits occur progressively. The neurological recovery or improvement of Pott's paraplegics with upper thoracic severe sharp kyphosis results in poor prognosis after decompressive surgery.
PMCID: PMC3282853  PMID: 21656306
5.  SAPHO syndrome with rapidly progressing destructive spondylitis: two cases treated surgically 
European Spine Journal  2008;17(Suppl 2):331-337.
The authors present two cases of synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome with rapidly progressing destructive spondylitis treated surgically. The spinal lesions in SAPHO syndrome generally have a good prognosis and rarely cause the structural destruction or neurological deterioration. Case 1: a 63-year-old female had palmoplantar pustulosis for 2 years. At first, she only felt a pain in the nape with no inducing factor. Two months later, she had incomplete quadriplegia (ASIA scale C). Magnetic resonance imaging showed destruction of C4–C7, kyphotic deformity, and severe compression of the spinal cord. Decompression and reconstruction surgery using anterior and posterior approach improved her paralysis. Case 2: a 69-year-old female complained of persistent back pain. Magnetic resonance imaging revealed spondylitis of T7–T9. Although there were no typical skin lesions, we diagnosed SAPHO syndrome by hyperostosis of the sternocostoclavicular joint and sacral joint. Destruction with kyphotic deformity of the spine progressed gradually for 3 months. Curettage and reconstruction surgery using thoracic endoscope relieved her pain and prevented the destruction of the spine. The histopathology of the specimen obtained surgically showed non-specific inflammation in both cases. Spondylitis in SAPHO syndrome may cause severe destruction and kyphotic deformity followed by paralysis.
PMCID: PMC2525915  PMID: 18389286
SAPHO syndrome; Spondylitis; Paralysis
6.  Posterior approach lumbar and thoracolumbar hemivertebra resection in congenital scoliosis in children under 10 years of age: results with 3 years mean follow up 
European Spine Journal  2013;23(1):209-215.
The authors present 15 cases of congenital scoliosis with lumbar or thoracolumbar hemivertebra in children under 10 years of age (mean age at the time of surgery was 5.5 years). Patients were treated by posterior hemivertebra resection and pedicle screws two levels stabilization or three or more levels stabilization in the case of deformity above or under hemivertebra or for severe curve deformities.
Materials and methods
All operated patients had worsening curves; mean follow up was 40 months. The mean scoliosis curve value was 44° Cobb, and reduced to a mean 11° Cobb after surgery. The mean segmental kyphosis value was 19.7° Cobb, and reduced to a mean −1.8° Cobb after surgery. We did not consider total dorsal kyphosis value as all hemivertebras treated were at lumbar or thoracic lumbar level. No major complications emerged (infections, instrumentation mobilization or failure, neurological or vascular impairment) and only one pedicle fracture occurred.
Our findings show that the hemivertebra resection with posterior approach instrumentation is an effective procedure, which has led to significant advances in congenital deformity control, which include excellent frontal and sagittal correction, excellent stability, short segment arthrodesis, low neurological impairment risk, and no necessity for further anterior surgery.
Surgery should be considered as soon as possible in order to avoid severe deformity and the use of long segment arthrodesis. The youngest patient we treated, with a completed dossier at the end the follow up was 24 months old at the time of surgery; the youngest patient treated by this procedure was 18 months old at the time of surgery.
PMCID: PMC3897828  PMID: 23934348
Hemivertebra; Posterior approach lumbar hemivertebra resection; Congenital scoliosis
7.  Thoracic pedicle subtraction osteotomy in the treatment of severe pediatric deformities 
European Spine Journal  2011;20(Suppl 1):95-104.
The traditional surgical treatment of severe spinal deformities, both in adult and pediatric patients, consisted of a 360° approach. Posterior-based spinal osteotomy has recently been reported as a useful and safe technique in maximizing kyphosis and/or kyphoscoliosis correction. It obviates the deleterious effects of an anterior approach and can increase the magnitude of correction both in the coronal and sagittal plane. There are few reports in the literature focusing on the surgical treatment of severe spinal deformities in large pediatric-only series (age <16 years old) by means of a posterior-based spinal osteotomy, with no consistent results on the use of a single posterior-based thoracic pedicle subtraction osteotomy in the treatment of such challenging group of patients. The purpose of the present study was to review our operative experience with pediatric patients undergoing a single level PSO for the correction of thoracic kyphosis/kyphoscoliosis in the region of the spinal cord (T12 and cephalad), and determine the safety and efficacy of posterior thoracic pedicle subtraction osteotomy (PSO) in the treatment of severe pediatric deformities. A retrospective review was performed on 12 consecutive pediatric patients (6 F, 6 M) treated by means of a posterior thoracic PSO between 2002 and 2006 in a single Institution. Average age at surgery was 12.6 years (range, 9–16), whereas the deformity was due to a severe juvenile idiopathic scoliosis in seven cases (average preoperative main thoracic 113°; 90–135); an infantile idiopathic scoliosis in two cases (preoperative main thoracic of 95° and 105°, respectively); a post-laminectomy kypho-scoliosis of 95° (for a intra-medullar ependimoma); an angular kypho-scoliosis due to a spondylo-epiphisary dysplasia (already operated on four times); and a sharp congenital kypho-scoliosis (already operated on by means of a anterior–posterior in situ fusion). In all patients a pedicle screws instrumentation was used, under continuous intra-operative neuromonitoring (SSEP, NMEP, EMG). At an average follow-up of 2.4 years (range, 2–6) the main thoracic curve showed a mean correction of 61°, or a 62.3% (range, 55–70%), with an average thoracic kyphosis of 38.5° (range, 30°–45°), for an overall correction of 65% (range, 60–72%). Mean estimated intra-operative blood loss accounted 19.3 cc/kg (range, 7.7–27.27). In a single case (a post-laminectomy kypho-scoliosis) a complete loss of NMEP occurred, promptly assessed by loosening of the initial correction, with a final negative wake-up test. No permanent neurologic damage, or instrumentation related complications, were observed. According to our experience, posterior-based thoracic pedicle subtraction osteotomies represent a valuable tool in the surgical treatment of severe pediatric spinal deformities, even in revision cases. A dramatic correction of both the coronal and sagittal profile may be achieved. Mandatory the use of a pedicle screws-only instrumentation and a continuous intra-operative neuromonitoring to obviate catastrophic neurologic complications.
PMCID: PMC3087044  PMID: 21468647
Thoracic pedicle subtraction osteotomy; Severe pediatric spinal deformities; Surgical treatment; Posterior-instrumented fusion only; Thoracic pedicle screws
8.  Results of complete hemivertebra excision followed by circumferential fusion and anterior or posterior instrumentation in patients with type-IA formation defect 
European Spine Journal  2006;15(8):1219-1229.
To evaluate the results of surgical treatment in patients with unlocked full-segmented hemivertebra treated by excision. Twenty-six patients with a mean age of 12.4±1.7 years were included in the study. The mean duration of follow-up was 47.8±21.9 months. Diagnosis of type-IA hemivertebra was established by clinical, radiological, CT, and MRI evaluation. Preoperatively, patients were randomly allocated into two groups. In the first group, patients underwent anterior hemivertebrectomy initially; this was followed by posterior excision of the hemivertebra, posterior instrumentation, and fusion. In the second group, posterior components of the hemivertebra were excised at first, then the hemivertebra body was excised anteriorly, and this was followed by anterior instrumentation and fusion. For both groups, compression was applied to the convex side while distraction was applied to the concave side. Frontal and sagittal plane analysis of radiograms obtained preoperatively, postoperatively, and after a minimum period of 2 years was performed. The balance was analyzed clinically and radiologically by the measurement of the lateral trunk shift (LT) and shift of head (SH). The mean preoperative and postoperative Cobb angles were 45.5°∓11.4° and 16.8°∓7.9°, respectively, and postoperatively, a mean correction rate of 64.4±13.9% was obtained (P=0.00). The mean correction rate was 61.2±13.3% (19.2°∓7.6°) for the last follow-up visit. Sagittal plane analysis demonstrated either conservation of physiological sagittal contours or a normalizing effect following excision of hemivertebra combined with anterior or posterior instrumentation. When postoperative balance values were compared, a statistically significant correction was found in terms of LT and SH values. Although none of the patients had complete balance (SH: 0 mm) or balanced curves (0 mm
PMCID: PMC3233956  PMID: 16395617
Congenital scoliosis; Surgical treatment; Hemivertebra excision
European Spine Journal  2011;20(7):1106-1113.
Priority of neurological decompression was regarded as necessary for scoliosis patients associated with Chiari I malformation in order to decrease the risk of spinal cord injury from scoliosis surgery. We report a retrospective series of scoliosis associated with Chiari I malformation in 13 adolescent patients and explore the effectiveness and safety of posterior scoliosis correction without suboccipital decompression. One-stage posterior approach total vertebral column resection was performed in seven patients with scoliosis or kyphosis curve >90° (average 100.1° scoliotic and 97.1° kyphotic curves) or presented with apparent neurological deficits, whereas the other six patients underwent posterior pedicle screw instrumentation for correction of spinal deformity alone (average 77.3° scoliotic and 44.0° kyphotic curves). The apex of the scoliosis curve was located at T7–T12. Mean operating time and intraoperative hemorrhage was 463 min and 5,190 ml in patients undergoing total vertebral column resection, with average correction rate of scoliosis and kyphosis being 63.3 and 71.1%, respectively. Mean operating time and intraoperative hemorrhage in patients undergoing instrumentation alone was 246 min and 1,450 ml, with the average correction rate of scoliosis and kyphosis being 60.8 and 53.4%, respectively. The mean follow-up duration was 32.2 months. No iatrogenic neurological deterioration had been encountered during the operation procedure and follow-up. After vertebral column resection, neurological dysfunctions such as relaxation of anal sphincter or hypermyotonia that occurred in three patients preoperatively improved gradually. In summary, suboccipital decompression prior to correction of spine deformity may not always be necessary for adolescent patients with scoliosis associated with Chiari I malformation. Particularly in patients with a severe and rigid curve or with significant neurological deficits, posterior approach total vertebral column resection is likely a good option, which could not only result in satisfactory correction of deformity, but also decrease the risk of neurological injury secondary to surgical intervention by shortening spine and reducing the tension of spinal cord.
PMCID: PMC3176690  PMID: 21399931
Scoliosis; Kyphosis; Chiari malformation; Adolescent; Corrective surgery
European Spine Journal  2010;19(10):1657-1676.
The second, internet-based multicenter study (MCSII) of the Spine Study Group of the German Association of Trauma Surgery (Deutsche Gesellschaft für Unfallchirurgie) is a representative patient collection of acute traumatic thoracolumbar (T1–L5) injuries. The MCSII results are an update of those obtained with the first multicenter study (MCSI) more than a decade ago. The aim of the study was to assess and bring into focus: the (1) epidemiologic data, (2) surgical and radiological outcome, and (3) 2-year follow-up (FU) results of these injuries. According to the Magerl/AO classification, there were 424 (57.8%) compression fractures (A type), 178 (24.3%) distractions injuries (B type), and 131 (17.9%) rotational injuries (C type). B and C type injuries carried a higher risk for neurological deficits, concomitant injuries, and multiple vertebral fractures. The level of injury was located at the thoracolumbar junction (T11–L2) in 67.0% of the case. 380 (51.8%) patients were operated on by posterior stabilization and instrumentation alone (POSTERIOR), 34 (4.6%) had an anterior procedure (ANTERIOR), and 319 (43.5%) patients were treated with combined posteroanterior surgery (COMBINED). 65% of patients with thoracic (T1–T10) and 57% with lumbar spinal (L3–L5) injuries were treated with a single posterior approach (POSTERIOR). 47% of the patients with thoracolumbar junction (T11–L2) injuries were either operated from posterior or with a combined posterior–anterior surgery (COMBINED) each. Short angular stable implant systems have replaced conventional non-angular stable instrumentation systems to a large extent. The posttraumatic deformity was restored best with COMBINED surgery. T-spine injuries were accompanied by a higher number and more severe neurologic deficits than TL junction or L-spine injuries. At the same time T-spine injuries showed less potential for neurologic recovery especially in paraplegic (Frankel/AISA A) patients. 5% of all patients required revision surgery for perioperative complications. Follow-up data of 558 (76.1%) patients were available and collected during a 30-month period from 1 January 2004 until 31 May 2006. On average, a posterior implant removal was carried out in a total of 382 COMBINED and POSTERIOR patients 12 months after the initial surgery. On average, the rehabilitation process required 3–4 weeks of inpatient treatment, followed by another 4 months of outpatient therapy and was significantly shorter when compared with MCSI in the mid-1990s. From the time of injury until FU, 80 (60.6%) of 132 patients with initial neurological deficits improved at least one grade on the Frankel/ASIA Scale; 8 (1.3%) patients deteriorated. A higher recovery rate was observed for incomplete neurological injuries (73%) than complete neurological injuries (44%). Different surgical approaches did not have a significant influence on the neurologic recovery until FU. Nevertheless, neurological deficits are the most important factors for the functional outcome and prognosis of TL spinal injuries. POSTERIOR patients had a better functional and subjective outcome at FU than COMBINED patients. However, the posttraumatic radiological deformity was best corrected in COMBINED patients and showed significantly less residual kyphotic deformity (biseg GDW −3.8° COMBINED vs. −6.1° POSTERIOR) at FU (p = 0.005). The sagittal spinal alignment was better maintained when using vertebral body replacement implants (cages) in comparison to iliac strut grafts. Additional anterior plate systems did not have a significant influence on the radiological FU results. In conclusion, comprehensive data of a large patient population with acute thoracolumbar spinal injuries has been obtained and analyzed with this prospective internet-based multicenter study. Thus, updated results and the clinical outcome of the current operative treatment strategies in participating German and Austrian trauma centers have been presented. Nevertheless, it was not possible to answer all remaining questions to contradictory findings of the subjective, clinical outcome and corresponding radiological findings between different surgical subgroups. Randomized-controlled long-term investigations seem mandatory and the next step in future clinical research of Spine Study Group of the German Trauma Society.
PMCID: PMC2989217  PMID: 20499114
Spinal injuries; Fracture; Treatment; Spine; Prospective; Multicenter study; Online database; Epidemiology; Complications; Spine Study Group (SSG) of the German Association of Trauma Surgery (DGU); Radiological findings; Follow-up; Rehabilitation; Activities of daily living; Outcome
Childhood laminectomy can lead to spinal deformity. This is a report of a case of paraplegia caused by rotokyphoscoliosis, a late complication of laminectomy.
A 55-year-old woman developed paraplegia due to post-laminectomy kyphoscoliosis. She had surgery for a spinal tumor at age 13 years. She developed kyphosis 2 years after the laminectomy, which has been gradually progressing over the years. She experienced weakness of lower limbs that progressed to paraplegia. There was no evidence for tumor recurrence. To our knowledge, this is the first reported case of post-laminectomy kyphoscoliosis causing late-onset paraplegia.
Conclusions/clinical relevance
This case highlights a possible long-term complication of laminectomy without stabilization or untreated kyphoscoliosis. Children should be followed closely after laminectomy because development of spinal deformity is very common. Without intervention, the kyphosis might progress and in the long term, serious neurological complications may result, including paraplegia.
PMCID: PMC3324835  PMID: 22507027
Laminectomy; Spinal tumor; Astrocytoma; Intramedullary; Spinal deformity; Kyphosis; Scoliosis; Myelopathy; Paraplegia
European Spine Journal  2011;20(10):1703-1710.
The management goal of sharp angular spinal deformity is to realign the spinal deformity and safely decompress the neurological elements. However, some shortcomings related to current osteotomy treatment for these deformities are still evident. We have developed a new spinal osteotomy technique—vertebral column decancellation (VCD), including multilevel vertebral decancellation, removal of residual disc, osteoclasis of the concave cortex, compression of the convex cortex accompanied by posterior instrumentation with pedicle screws, with the expectation to decrease surgical-related complications. From January 2004 to March 2007, 45 patients (27 males/18 females) with severe sharp angular spinal deformities at our institution underwent VCD. The diagnoses included 29 congenital kyphoscoliosis and 16 Pott’s deformity. Preoperative and postoperative radiographic evaluation was performed. Intraoperative, postoperative and general complications were noted. For a kyphosis deformity, an average of 2.2 vertebrae was decancellated (range, 2–4 vertebrae). The mean preoperative kyphosis was +98.6° (range, 82°–138°), and the mean kyphosis in the immediate postoperative period was +16.4° (range, 4°–30°) with an average postoperative correction of +82.2° (range, 61°–124°). For a kyphoscoliosis deformity, the correction rate was 64% in the coronal plane (from 83.4°–30.0°) postoperatively and 32.5° (61% correction) at 2 years’ follow-up. In the sagittal plane, the average preoperative curve of 88.5° was corrected to 28.6° immediately after surgery and to 31.0° at 2 years’ follow-up. All patients had solid fusion at latest follow-up. Complications were encountered in eight patients (17.8%), including CSF leak (n = 1), deep wound infection (n = 1), epidural hematoma (n = 1), transient neurological deficit (n = 4), and complete paralysis (n = 1). The results of this study show that single-stage posterior VCD is an effective option to manage severe sharp angular spinal deformities.
PMCID: PMC3175886  PMID: 21424339
Posterior approach; Vertebral column decancellation (VCD); Kyphosis; Kyphoscoliosis; Osteotomy
European Spine Journal  2008;17(3):361-372.
We report a multilevel modified vertebral column resection (MVCR) through a single posterior approach and clinical outcomes for treatment of severe congenital rigid kyphoscoliosis in adults. Transpedicular eggshell osteotomies and vertebral column resection are two techniques for the surgical treatment of rigid severe spine deformities. The authors developed a new technique combining the two surgical methods as a MVCR, through a single posterior approach, for surgical treatment of severe congenital rigid kyphoscoliosis in adults. Thirteen adult patients with severe rigid congenital kyphoscoliosis deformity were treated by a single posterior approach using a MVCR technique. The surgery processes included a one-stage posterior transpedicular eggshell technique first, and then expanded the eggshell technique to adjacent intervertebra space through abrasive reduction of the vertebral cortices from inside out. All posterior vertebral elements were removed including the cortical vertebral bone around the neural canal. Range of resection of the vertebral column at the apex of the deformity included apical vertebra and both cephalic and/or caudal adjacent wedged vertebrae. Totally, 32 vertebrae had been removed in 13 patients, with 2.42 vertebrae being removed on average in each case. The average fusion extent was 7.69 vertebrae. Mean operation time was 266 min with average blood loss of 2,411.54 ml during operation. Patients were followed up for an average duration of 2.54 years. Deformity correction was 59% in the coronal plane (from 79.7° to 32.4°) postoperatively and 33.7° (57% correction) at 2 years follow-up. In the sagittal plane, correction was from preoperative 85.9° to 27.5° immediately after operation, and 32.0° at 2 years follow-up. Postoperative pain was reduced from preoperative 1.77 to 0.54 at 2 years follow-up in visual analog scale. SRS-24 scale was from 38.2 preoperatively to 76.9 at 2 years follow-up postoperative. Complications were encountered in four patients (30.7%) with transient neurology that spontaneously improved without further treatment within 3 months. MVCR technique through a single posterior approach is an effective procedure for the surgical treatment of severe congenital rigid kyphoscoliosis in adults.
PMCID: PMC2270391  PMID: 18172699
Kyphoscoliosis; Eggshell technique; Vertebral column resection; Modified vertebral column resection
European Spine Journal  2011;21(3):514-529.
The treatment of rigid and severe scoliosis and kyphoscoliosis is a surgical challenge. Presurgical halo-gravity traction (HGT) achieves an increase in curve flexibility, a reduction in neurologic risks through gradual traction on a chronically tethered cord and an improvement in preoperative pulmonary function. However, little is known with respect to the ideal indications for HGT, its appropriate duration, or its efficacy in the treatment of rigid deformities.
Materials and methods
To investigate the use of HGT in severe deformities, we performed a retrospective review of 45 patients who had severe and rigid scoliosis or kyphoscoliosis. The analysis focused on the impact of HGT on curve flexibility, pulmonary function tests (PFTs), complications and surgical outcomes in a single spine centre.
PFTs were used to assess the predicted forced vital capacity (FVC%). The mean age of the sample was 24 ± 14 years. 39 patients had rigid kyphoscoliosis, and 6 had scoliosis. The mean apical rotation was 3.6° ± 1.4°, according to the Nash and Moe grading system. The curve apices were mainly in the thoracic spine. HGT was used preoperatively in all the patients. The mean preoperative scoliosis was 106.1° ± 34.5°, and the mean kyphosis was 90.7° ± 29.7°. The instrumentation used included hybrids and pedicle screw-based constructs. In 18 patients (40%), a posterior concave thoracoplasty was performed. Preoperative PFT data were obtained for all the patients, and 24 patients had ≥3 assessments during the HGT. The difference between the first and the final PFTs during the HGT averaged 7.0 ± 8.2% (p < .001). Concerning the evolution of pulmonary function, 30 patients had complete data sets, with the final PFT performed, on average, 24 months after the index surgery. The mean preoperative FVC% in these patients was 47.2 ± 18%, and the FVC% at follow-up was 44.5 ± 17% (a difference that did not reach statistical significance). The preoperative FVC% was highly predictive of the follow-up FVC% and the response during HGT. The mean flexibility of the scoliosis curve during HGT was only 14.8 ± 11.4%, which was not significantly different from the flexibility measures achieved on bending radiographs or Cotrel traction radiographs. In rigid curves, the Cobb angle difference between the first and final radiographs during HGT was only 8° ± 9° for scoliosis and 7° ± 12° for kyphosis. Concerning surgical outcomes, 13 patients (28.9%) experienced minor and 15 (33.3%) experienced major complications. No permanent neurologic deficits or deaths occurred. Additional surgery was indicated in 12 patients (26.7%), including 7 rib-hump resections. At the final evaluation, 69% of the patients had improved coronal balance, and at a mean follow-up of 33 ± 23.3 months, 39 patients (86.7%) were either satisfied or very satisfied with the overall outcome.
The improvement of pulmonary function and the restoration of sagittal and coronal balance are the main goals in the treatment of severe and rigid scoliosis and kyphoscoliosis. A review of the literature showed that HGT is a useful tool for selected patients. Preoperative HGT is indicated in severe curves with moderate to severe pulmonary compromise. HGT should not be expected to significantly improve severe curves without a prior anterior and/or posterior release. The data presented in this study can be used in future studies to compare the surgical and pulmonary outcomes of severe and rigid deformities.
PMCID: PMC3296862  PMID: 22042044
Halo-gravity traction; Severe scoliosis; Kyphoscoliosis; Pulmonary function; Surgical treatment
European Spine Journal  2012;21(8):1471-1476.
We present the case of a 2-year-old patient with congenital scoliosis due to a lumbar hemivertebra. The current gold standard treatment of such an abnormality would be hemivertebra resection and short level posterior spinal fusion. However, due to the young age of the patient, we considered that application of a fusionless solution might offer advantages in terms of retaining normal segmental motion and the potential for growth.
The incarcerated hemivertebra was resected and the facet joints of the neighbouring vertebrae were joined to create a new functional motion segment and correct the kyphoscoliotic deformity. Transpedicular screws were inserted on the convex side in L2 and L3 and a tension band was applied.
16 years after the surgery, the patient was completely pain-free, motion of the lumbar spine was preserved and the physiological curvatures were maintained.
To our knowledge a fusionless surgical solution for the treatment of a hemivertebra has never been described before. Although this is only a single case, the good result with a long follow-up suggests the technique is worthwhile considering when planning the treatment of a lumbar hemivertebra in very young children.
PMCID: PMC3535243  PMID: 22349966
Early onset scoliosis; Congenital deformity; Motion preservation; Non-fusion procedure
European Spine Journal  2009;18(9):1249-1254.
Congenital scoliosis associated with split cord malformation raises the issue on how to best manage these patients to avoid neurologic injury while achieving satisfactory correction. We present the case of a 12-year-old girl who first presented when she was 11-year old with such combination but without much physical handicap or neurological deficit. The corrective surgery offered at that time was refused by the family. She again presented after 1 year with documented severe aggravation of the curve resulting in unstable walking and psychological upset. Her imaging studies showed multiple malformations in lower cervical and thoracic spine and a split cord malformation type 2 (fibrous septum with diplomyelia) at the apex of the deformity. A one-stage correction was deemed neurologically too risky. We therefore performed during a first stage a thoracotomy with anterior release. This was followed by skeletal traction with skull tongs and bilateral femoral pins. After gradual increase in traction weights a reasonable correction was achieved without any neurological deficit, over the next 10 days. A second-stage operation was done on the 11th day and a posterior instrumented fusion was performed. Post-operative recovery was uneventful and there were no complications. She was discharged with a Boston Brace to be worn for 3 months. At 2-year follow-up the patient outcome is excellent with excellent balance and correction of the deformity. In this grand round case, we discuss all the different option of treatment of congenital scoliosis associated with split cord malformation. In a medical environment where spinal cord monitoring is lacking, we recommend an initial release followed by skull and bifemoral traction over several days to monitor the neurologic status of the patient. Once optimal correction is achieved with the traction, a posterior instrumentation can be safely done.
PMCID: PMC2899544  PMID: 19626347
Scoliosis; Congenital abnormalities; Split cord malformation; Corrective surgery; Staged surgery; Spinal instrumentation
Treacher Collins syndrome is an autosomal dominant disorder resulting in congenital craniofacial deformities. Scoliosis has not been previously reported as one of the extracranial manifestations of this syndromic condition.
Case presentation
We present a 15-year-old British Caucasian girl with Treacher Collins syndrome who developed a severe double thoracic scoliosis measuring 102° and 63° respectively. The deformity was noted at age 14 years by the local general practitioner and gradually progressed until she was referred to our service and subsequently was scheduled for surgical correction. There were no congenital vertebral anomalies. As part of the condition, she had bilateral conductive hearing impairment. She also had reduced respiratory reserves and a restrictive lung disease. Both curves were rigid on supine maximum traction radiographs. She underwent a single-stage anterior and posterior spinal arthrodesis with pedicle hook/sublaminar wire/screw and rod instrumentation and autologous rib graft, supplemented by allograft bone and made a good postoperative recovery. Her scoliosis was corrected to 25° and 24° and a balanced spine in the coronal and sagittal planes was achieved. At latest follow-up beyond skeletal maturity (3 years post-surgery) she had an excellent cosmetic outcome with no loss of deformity correction, no detected pseudarthrosis and a normal level of activities.
Scoliosis can occur in patients with Treacher Collins syndrome with the deformity demonstrating significant deterioration around the adolescent growth spurt. A high index of awareness will allow for an early diagnosis and scoliosis correction at a stage when this can be safer and performed through a single-stage posterior procedure. If the deformity is detected at a later age and stage of growth as occurred in our patient, more complex surgery is required and this increases the risk for major morbidity and potential mortality. Surgical treatment can correct the deformity, balance the spine and restore cosmesis, as well as prevent mechanical back pain and respiratory complications if the scoliosis progressed to cause severe thoracic distortion. A thorough preoperative assessment can diagnose associated comorbidities and reduce the risk for postoperative complications.
PMCID: PMC4320598  PMID: 25524572
Posterior spinal fusion; Scoliosis; Surgical treatment; Treacher Collins syndrome
Pyogenic spondylodiscitis represents a potentially life-threatening condition. Due to the low incidence, evidence-based surgical recommendations in the literature are equivocal, and the treatment modalities remain controversial.
Case presentation
A 59 year-old patient presented with a history of thoracic spondylodiscitis resistant to antibiotic treatment for 6 weeks, progressive severe back pain, and a new onset of bilateral lower extremity weakness. Clinically, the patient showed a deteriorating spastic paraparesis of her lower extremities. An emergent MRI revealed a kyphotic wedge compression fracture at T7/T8 with significant spinal cord compression, paravertebral and epidural abscess, and signs of myelopathy. The patient underwent surgical debridement with stabilization of the anterior column from T6–T9 using an expandable titanium cage, autologous bone graft, and an anterolateral locking plate. The patient recovered well under adjunctive antibiotic treatment. She presented again to the emergency department 6 months later, secondary to a repeat fall, with acute paraplegia of the lower extremities and radiographic evidence of failure of fixation of the anterior T-spine. She underwent antero-posterior revision fixation with hardware removal, correction of kyphotic malunion, evacuation of a recurrent epidural abscess, decompression of the spinal canal, and 360° fusion from T2–T11. Despite the successful salvage procedure, the patient deteriorated in the postoperative phase, when she developed multiple complications including pneumonia, acute respiratory distress syndrome, bacterial meningitis, abdominal compartment syndrome, followed by septic shock with multiple organ failure and a lethal outcome within two weeks after revision surgery.
This catastrophic example of a lethal outcome secondary to failure of anterior column fixation for pyogenic thoracic spondylodiscitis underlines the notion that surgical strategies for the infected spine must be aimed at achieving absolute stability by a 360° fusion. This aggressive – albeit controversial – concept allows for an adequate infection control by adjunctive antibiotics and reduces the imminent risk of a secondary loss of fixation due to compromises in initial fixation techniques.
PMCID: PMC2654872  PMID: 19243602
Rubinstein-Taybi syndrome is an autosomal dominant disorder resulting in congenital craniofacial deformities, and divided into types 1 and 2. Scoliosis has not been reported as one of the extra-cranial manifestations of Rubinstein-Taybi syndrome type 2.
Case presentation
We present a 14-year-old British Caucasian girl with Rubinstein-Taybi type 2 syndrome who developed a severe double thoracic scoliosis measuring 39° and 68° respectively. Her scoliosis was associated with thoracic hypokyphosis, causing a marked reduction in the anteroposterior diameter of her chest and consequent severe restrictive lung disease. The deformity was noted by her local pediatrician as part of a chest infection assessment when she was aged 13 years, and gradually progressed as the result of spinal growth. Our patient underwent a posterior spinal arthrodesis using a single concave pedicle hook and screw rod construct and locally harvested autologous graft supplemented by allograft bone. This spinal fixation technique was selected because of our patient’s low body weight to avoid prominence of the instrumentation causing skin healing problems and pain. Her scoliosis was corrected to 18° and 30° and we achieved a balanced spine in the coronal and sagittal planes. An underarm spinal jacket was provided for six months after surgery. During her latest follow-up at skeletal maturity, our patient had an excellent cosmetic outcome with no loss of deformity correction or detected pseudoarthrosis and a normal level of activities.
Scoliosis can develop in young children with Rubinstein-Taybi syndrome type 2, with the deformity deteriorating around the pubertal growth spurt. Surgical treatment can correct the deformity, balance the spine and prevent mechanical back pain. It can also stabilize the chest area and avoid respiratory complications developing as the scoliosis progresses, which can result in severe restrictive pulmonary disease. The use of single concave instrumentation is indicated in very slim patients with poor muscle bulk; in our patient, this produced satisfactory deformity correction and a favorable outcome at completion of growth. Peri-operative care in this group of patients can be very challenging because of associated co-morbidities as well as the presence of severe behavioral issues that result in poor patient compliance.
PMCID: PMC4334754  PMID: 25596810
Posterior spinal fusion; Rubinstein-Taybi syndrome; Scoliosis; Surgical treatment
Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a rare inflammatory disease characterized by hypertrophic inflammation of the dura mater and various clinical courses that are from myelopathy. Although many associated diseases have been suggested, the etiology of IHSP is not well understood. The ideal treatment is controversial. In the first case, a 55-year-old woman presented back pain, progressive paraparesis, both leg numbness, and voiding difficulty. Initial magnetic resonance imaging (MRI) demonstrated an anterior epidural mass lesion involving from C6 to mid-thoracic spine area with low signal intensity on T1 and T2 weighted images. We performed decompressive laminectomy and lesional biopsy. After operation, she was subsequently treated with steroid and could walk unaided. In the second case, a 45-year-old woman presented with fever and quadriplegia after a spine fusion operation due to lumbar spinal stenosis and degenerative herniated lumbar disc. Initial MRI showed anterior and posterior epidural mass lesion from foramen magnum to C4 level. She underwent decompressive laminectomy and durotomy followed by steroid therapy. However, her conditions deteriorated gradually and medical complications occurred. In our cases, etiology was not found despite through investigations. Initial MRI showed dural thickening with mixed signal intensity on T1- and T2-weighted images. Pathologic examination revealed chronic nonspecific inflammation in both patients. Although one patient developed several complications, the other showed slow improvement of neurological symptoms with decompressive surgery and steroid therapy. In case of chronic compressive myelopathy due to the dural hypertrophic change, decompressive surgery such as laminectomy or laminoplasty may be helpful as well as postoperative steroid therapy.
PMCID: PMC3243847  PMID: 22200026
Idiopathic hypertrophic spinal pachymeningitis; Spinal cord compression; Chronic nonspecific inflammation; Dural thickening
European Spine Journal  2012;22(2):305-309.
Adolescent scar contracture kyphoscoliosis is a very rare disease.
Methods and Results
Here, we present the case of a 21-year-old man who was scalded due to ebullient water when he was 10 years old. Moreover, kyphoscoliosis was found when he was 12 years old and developed rapidly. Thereafter, no management was proposed before his consultation at our center. On examination, kyphoscoliosis was detected in thoracolumbar, the trunk deviated to the right on standing view, extensive contractured scar presented on the right side of the back, abdomen, chest wall, hip, right thighs and armpit anterior, especially in the right flank. A one-stage correction was deemed too risky, we therefore released contractured scar during the first stage with the defect of soft tissue protected by vaccum sealing drainage and then performed skeletal traction with halo and bilateral femoral pins. A reasonable correction was achieved without any neurological deficits 1 month after traction. Next, a second-stage operation was taken to translate a free anterolateral thigh myocutaneous flap to overlay the extensive defect of soft tissue. 1.5 months later, a third posterior segmental pedicle screw instrumented fusion with Smith-Peterson osteotomy between T9 and L2 was performed. Postoperative recovery was uneventful and as there were no complications, he was discharged 10 days after the third surgery. At 2-year follow-up the patient’s outcome is excellent with balance and correction of the deformity.
Based this grand round case and relevant literature, we discuss the different options for the treatment of adolescent scar contracture scoliosis.
PMCID: PMC3555617  PMID: 23053756
Adolescent scar contracture kyphoscoliosis; Corrective surgery; Staged surgery; Spinal instrumentation; Free flap
Asian Spine Journal  2014;8(6):711-719.
Study Design
Retrospective clinical series.
To study the clinical, functional and radiological results of patients with tuberculous spondylitis with and without paraplegia, treated surgically using the "Extended Posterior Circumferential Decompression (EPCD)" technique.
Overview of Literature
With the increasing possibility of addressing all three columns by a single approach, posterior and posterolateral approaches are gaining acceptance. A single exposure for cases with neurological deficit and kyphotic deformity requiring circumferential decompression, anterior column reconstruction and posterior instrumentation is helpful.
Forty-one patients with dorsal/dorsolumbar/lumbar tubercular spondylitis who were operated using the EPCD approach between 2006 to 2009 were included. Postoperatively, patients were started on nine-month anti-tuberculous treatment. They were serially followed up to thirty-six months and both clinical measures (including pain, neurological status and ambulatory status) and radiological measures (including kyphotic angle correction, loss of correction and healing status) were used for assessment.
Disease-healing with bony fusion (interbody fusion) was seen in 97.5% of cases. Average deformity (kyphosis) correction was 54.6% in dorsal spine and 207.3% in lumbar spine. Corresponding loss of correction was 3.6 degrees in dorsal spine and 1.9 degrees in the lumbar spine. Neurological recovery in Frankel B and C paraplegia was 85.7% and 62.5%, respectively.
The EPCD approach permits all the advantages of a single or dual session anterior and posterior surgery, with significant benefits in terms of decreased operative time, reduced hospital stay and better kyphotic angle correction.
PMCID: PMC4278975  PMID: 25558312
Extended posterior approach; Circumferential spinal canal decompression; Kyphosis correction; Interbody fusion; Neurological recovery
Indian Journal of Orthopaedics  2008;42(4):454-459.
Paraplegia of late onset in adolescents with caries of dorsal spine is considered to be due to the reactivation of infection. Internal salient at the level of acute kyphotic deformity of the dorsal spine is formed by posterior cartilaginous remains of grossly destroyed vertebral bodies. The author presents a study of eight adolescent patients with paraplegia of late onset associated with severe kyphotic deformity of dorsal spine with observations on the cause of paraplegia, the final neurological outcome following anterior decompression and its prevention.
Materials and Methods:
Eight adolescent patients mean age 14.4 yrs 6 males and 2 females with healed childhood caries of dorsal spine, having a mean kyphotic angle of 80° (range 60°–140°) presented with paraplegia of late onset. Of these patients, two had medical research council grade 0 muscle power; four had grade 2 muscle power, and two others had grade 3 muscle power in the lower limbs and were unable to walk unaided. One patient with 140° kyphoscoliotic deformity with grade 3 muscle power had post-polio residual paralysis (PPRP) in addition. All patients were subjected to thorough anterior spinal decompression through transthoracic, transpleural thoracotomy from the left side.
In six of the eight patients, the spine at the site of deformity being very rigid, the deformity could not be corrected and the intervertebral gap was bridged with appropriate autogenous tricortical cortico cancelluous bone graft. In one patient (case 4), the kyphotic deformity could be corrected by 50%. In one patient with 140° kyphosis and PPRP, the gap after the decompression of cord, could not be bridged with bone graft and was given a custom made, well molded plastic black shell to wear while walking and, in particular, while traveling in a vehicle. In all seven patients, bone grafts took six months for bridging the intervertebral gaps. All patients recovered to grade 4 muscle power 6–12 months after surgery.
In adolescents with healed caries of dorsal spine with acute kyphosis and paraplegia, the treatment of choice is anterior surgical decompression of the cord and bridging the gap thus created with bone graft.
PMCID: PMC2740360  PMID: 19753235
Late onset of paraplegia in adolescents; management; anterior decompression
European Spine Journal  2009;19(4):583-592.
The correction of severe post-tubercular kyphosis (PTK) is complex and has the disadvantage of being multiple staged with a high morbidity. Here, we describe the procedure and results of closing–opening osteotomy for correction of PTK which shortens the posterior column and opens the anterior column appropriately to correct the deformity without altering the length of the spinal cord. Seventeen patients with PTK (10 males; 7 females) with an average age of 18.3 ± 10.6 years (range 4–40 years) formed the study group. There were ten thoracolumbar, one lumbar and six thoracic deformities. The number of vertebrae involved ranged from 2 to 5 (average 2.8). Preoperative kyphosis averaged 69.2° ± 25.1° (range 42°–104°) which included ten patients with deformity greater than 60°. The average vertebral body loss was 2.01 ± 0.79 (range 1.1–4.1). The neurological status was normal in 13 patients, Frankel’s grade D in three patients and grade C in one. Posterior stabilization with pedicle screw instrumentation was followed by a preoperatively calculated wedge resection. Anterior column reconstruction was performed using rib grafts in four, tricortical iliac bone graft in five, cages in six, and bone chips alone and fibular graft in one patient each. Average operating time was 280 min (200–340 min) with an average blood loss of 820 ml (range 500–1,600 ml). The postoperative kyphosis averaged 32.4° ± 19.5° (range 8°–62°). The percentage correction of kyphosis achieved was 56.8 ± 14.6% (range 32–83%). No patient with normal preoperative neurological status showed deterioration in neurology after surgery. The last follow-up was at an average of 43 ± 4 months (range 32–64 months). The average loss of correction at the last follow-up was 5.4° (range 3°–9°). At the last follow-up, the mean preoperative pain visual analogue scale score decreased significantly from 9.2 (range 8–10 points) to 1.5 (range 1–2 points). There was also a significant decrease in mean preoperative Oswestry’s Disability Index from 56.4 (range 46–68) to 10.6 (range 6–15). Complications were superficial wound infections in two, neurological deterioration in one, temporary jaundice in one and implant failure requiring revision in one. Single-stage closing–opening wedge osteotomy is an effective method to correct severe PTK. The procedure has the advantage of being a posterior only, single-stage correction, which allows for significant correction with minimal complications.
PMCID: PMC2899834  PMID: 20013004
Tuberculosis; Kyphosis; Closing–opening wedge; Osteotomy
Case Study 
Sarah is a 58-year-old breast cancer survivor, social worker, and health-care administrator at a long-term care facility. She lives with her husband and enjoys gardening and reading. She has two grown children and three grandchildren who live approximately 180 miles away.
One morning while showering, Sarah detected a painless quarter-sized lump on her inner thigh. While she thought it was unusual, she felt it would probably go away. One month later, she felt the lump again; she thought that it had grown, so she scheduled a visit with her primary care physician. A CT scan revealed a 6.2-cm soft-tissue mass in the left groin. She was referred to an oncologic surgeon and underwent an excision of the groin mass. Pathology revealed a grade 3 malignant melanoma. She was later tested and found to have BRAF-negative status. Following her recovery from surgery, Sarah was further evaluated with an MRI scan of the brain, which was negative, and a PET scan, which revealed two nodules in the left lung.
As Sarah had attended a cancer support group during her breast cancer treatment in the past, she decided to go back to the group when she learned of her melanoma diagnosis. While the treatment options for her lung lesions included interleukin-2, ipilimumab (Yervoy), temozolomide, dacarbazine, a clinical trial, or radiosurgery, Sarah's oncologist felt that ipilimumab or radiosurgery would be the best course of action. She shared with her support group that she was ambivalent about this decision, as she had experienced profound fatigue and nausea with chemotherapy during her past treatment for breast cancer. She eventually opted to undergo stereotactic radiosurgery.
After the radiosurgery, Sarah was followed every 2 months. She complained of shortness of breath about 2 weeks prior to each follow-up visit. Each time her chest x-ray was normal, and she eventually believed that her breathlessness was anxiety-related. Unfortunately, Sarah’s 1-year follow-up exam revealed a 2 cm × 3 cm mass in her left lung, for which she had a surgical wedge resection. Her complaints of shortness of breath increased following the surgery and occurred most often with anxiety, heat, and gardening activities, especially when she needed to bend over. Sarah also complained of a burning "pins and needles" sensation at the surgical chest wall site that was bothersome and would wake her up at night.
Sarah met with the nurse practitioner in the symptom management clinic to discuss her concerns. Upon physical examination, observable signs of breathlessness were lacking, and oxygen saturation remained stable at 94%, but Sarah rated her breathlessness as 7 on the 0 to 10 Borg scale. The nurse practitioner prescribed duloxetine to help manage the surgical site neuropathic pain and to assist with anxiety, which in turn could possibly improve Sarah’s breathlessness. Several nonpharmacologic modalities for breathlessness were also recommended: using a fan directed toward her face, working in the garden in the early morning when the weather is cooler, gardening in containers that are at eye level to avoid the need to bend down, and performing relaxation exercises with pursed lip breathing to relieve anxiety-provoked breathlessness. One month later, Sarah reported relief of her anxiety; she stated that the fan directed toward her face helped most when she started to feel "air hungry." She rated her breathlessness at 4/10 on the Borg scale.
Sarah’s chest x-rays remained clear for 6 months, but she developed a chronic cough shortly before the 9-month exam. An x-ray revealed several bilateral lung lesions and growth in the area of the previously resected lung nodule. Systemic therapy was recommended, and she underwent two cycles of ipilimumab. Sarah’s cough and breathlessness worsened, she developed colitis, and she decided to stop therapy after the third cycle. In addition, her coughing spells triggered bronchospasms that resulted in severe anxiety, panic attacks, and air hunger. She rated her breathlessness at 10/10 on the Borg scale during these episodes. She found communication difficult due to the cough and began to isolate herself. She continued to attend the support group weekly but had difficulty participating in conversation due to her cough.
Sarah was seen in the symptom management clinic every 2 weeks or more often as needed. No acute distress was present at the beginning of each visit, but when Sarah began to talk about her symptoms and fear of dying, her shortness of breath and anxiety increased. The symptom management nurse practitioner treated the suspected underlying cause of the breathlessness and prescribed oral lorazepam (0.5 to 1 mg every 6 hours) for anxiety and codeine cough syrup for the cough. Opioids were initiated for chest wall pain and to control the breathlessness. Controlled-release oxycodone was started at 10 mg every 12 hours with a breakthrough pain (BTP) dose of 5 mg every 2 hours as needed for breathlessness or pain. Sarah noted improvement in her symptoms and reported a Borg scale rating of 5/10. Oxygen therapy was attempted, but subjective improvement in Sarah’s breathlessness was lacking.
Sarah’s disease progressed to the liver, and she began experiencing more notable signs of breathlessness: nasal flaring, tachycardia, and restlessness. Opioid doses were titrated over the course of 3 months to oxycodone (40 mg every 12 hours) with a BTP dose of 10 to 15 mg every 2 hours as needed, but her breathlessness caused significant distress, which she rated 8/10. The oxycodone was rotated to IV morphine continuous infusion with patient-controlled analgesia (PCA) that was delivered through her implantable port. This combination allowed Sarah to depress the PCA as needed and achieve immediate control of her dyspneic episodes. Oral lorazepam was also continued as needed.
Sarah’s daughter moved home to take care of her mother, and hospice became involved for end-of-life care. As Sarah became less responsive, nurses maintained doses of morphine for control of pain and breathlessness and used a respiratory distress observation scale to assess for breathlessness since Sarah could no longer self-report. A bolus PCA dose of morphine was administered by Sarah’s daughter if her mother appeared to be in distress. Sarah died peacefully in her home without signs of distress.
PMCID: PMC4093448  PMID: 25032021

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