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  • By Author: Luerssen, Thomas G. x
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Jonathan G. Thomas, Steven W. Hwang, Todd J. Blumberg, William E. Whitehead, Daniel J. Curry, Thomas G. Luerssen and Andrew Jea

Object

Over 85% of patients with myelomeningoceles require placement of a ventriculoperitoneal shunt for hydrocephalus, and between 25% and 85% of these patients develop scoliosis. Although most patients undergo repeated shunt series radiography to evaluate for device malfunction, scoliosis radiographs are less consistently obtained. The authors sought to determine if a correlation exists between these 2 radiographic techniques for a given patient, as shunt series are obtained with the patient supine, whereas scoliosis radiographs are acquired with the patient standing upright. The authors also endeavored to study if shunt series radiographs can reliably detect significant scoliosis.

Methods

The authors retrospectively reviewed a single institution's series of 593 patients with myelomeningoceles and identified all patients in whom a shunt series and scoliosis radiographs were obtained within a 6-month period. They reviewed the medical records and radiographs of these patients for demographic and radiographic parameters. They then applied a linear regression model and determined shunt series curve cutoffs to detect scoliotic curves greater than 20° and 50°.

Results

Of the 593 patients identified, 116 did not have radiographs available for interpretation. Of the remaining 477 patients, 201 had radiographic evidence of scoliosis (42%), and 66 had both a shunt series and a scoliosis radiographs acquired within a 6-month interval. In 4 patients, both end vertebrae of the scoliotic curve could not be visualized on a single radiograph. The mean age of the remaining cohort was 10.6 ± 5.2 years and the mean curve magnitude was 58° ± 37°. Using identical end vertebrae, the mean shunt series curve magnitude was 49° ± 35°. The mean interval between both radiographs was 2.3 ± 3.3 months. The regression model showed a strong linear association between shunt series and scoliosis series curves. A curve greater than 19° on shunt series radiographs would detect significant curves of greater than 20° on scoliosis series with 91% sensitivity and 78% specificity. A shunt series curve greater than 37° had 100% sensitivity and 93% specificity in identifying significant scoliotic curves greater than 50°.

Conclusions

Although shunt series radiographs may not precisely depict scoliotic curve magnitude because the impact of gravity is negated, they may be useful in helping to confirm clinical suspicion of scoliosis. The authors' results suggest a strong correlation between both types of radiographs.

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Steven W. Hwang, Jonathan G. Thomas, William E. Whitehead, Daniel J. Curry, Robert C. Dauser, Eugene S. Kim, Thomas G. Luerssen and Andrew Jea

Congenital long QT syndrome (LQTS) is a rare genetic ion transmembrane disorder that has been associated with multiple various genetic mutations including life-threatening cardiac arrhythmias and sudden death. Left thorascopic sympathectomy is an effective treatment for patients who are refractory to medical therapy or who need frequent epicardial internal cardiodefibrillator intervention. Although there is substantial literature about this therapy in adults, few reports detail the outcomes in children who undergo left thorascopic sympathectomies to treat LQTS.

The authors report the successful use of a left thoracic sympathectomy for the treatment of an 11-year-old girl who had persistently symptomatic LQTS, even after implantation of an automatic cardioverter-defibrillator. The patient remained clinically stable without arrhythmias through 6 months' of follow-up.

The authors also reviewed the relevant literature and found that it suggests that 77% of patients will have immediate resolution of their symptoms/arrhythmias after the procedure. When the outcome definition was broadened to include patients who had only 1 or 2 cardiac episodes in the follow-up period, 88% of cases were considered successful. The results of this case study and literature review suggest that left thorascopic sympathectomy is a safe and effective approach for treating pediatric patients with LQTS.

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Steven W. Hwang, Jonathan G. Thomas, Todd J. Blumberg, William E. Whitehead, Daniel J. Curry, Robert C. Dauser, Thomas G. Luerssen and Andrew Jea

Object

Significant lumbar kyphosis is frequently observed in patients with myelomeningocele and has been associated with increasing functional impairment, decreased abdominal volume, respiratory impairment, discomfort, and skin ulcerations overlying the prominent gibbus. Treatment of severe kyphotic deformities can include kyphectomy, with or without ligation of the thecal sac, with posterior spinal fixation. However, most series have reported a high rate of morbidity and complications associated with surgical intervention for correction of kyphosis in patients with myelomeningocele. The authors describe a technique in which pedicle screw (PS)–only constructs are used without transection of the thecal sac to treat severe kyphosis successfully, with minimal morbidity.

Methods

The authors retrospectively reviewed medical records and radiographic images in 2 patients with myelomeningoceles in whom kyphectomies had been performed at the authors' institution between January 2007 and July 2010. They also reviewed the existing literature for case reports or published series of patients with myelomeningocele treated with kyphectomies, to evaluate the outcomes.

Results

Both patients were male and had thoracic-level myelomeningoceles that had been repaired at birth, with associated paraplegia. Neither patient had any significant scoliotic deformity associated with the kyphosis, and both had fixation from T-9 to the ilium, which was performed using PS constructs, along with L1–2 kyphectomies. The patient in Case 1 was 20 years old and was treated for progressive kyphosis and an ulcerated nonhealing wound over the gibbus. The patient in Case 2 was 10 years old and was treated for progressive pain and functional impairment. The 2 patients had a mean correction of 63%, with a mean correction of kyphotic deformity from 136° to 51°. Neither patient developed any complication in the short term postoperatively, whereas published series have reported high complication rates, including wound infection, poor wound healing, CSF leakage, pseudarthrosis, and shunt malfunction.

Conclusions

Severe kyphotic deformities in patients with myelomeningocele can be safely treated using PS-only constructs without ligation of the thecal sac. Further evaluation with a larger sample and longer follow-up are needed to detect any associated complications, such as proximal junctional kyphosis. Further evaluation may also validate whether PS-only constructs permit successful outcomes with a shorter construct and fewer instrumented levels.

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Jonathan G. Thomas, Steven W. Hwang, William E. Whitehead, Daniel J. Curry, Thomas G. Luerssen and Andrew Jea

Object

Lumbar disc herniation is rare in the pediatric age group, but may still cause a significant amount of pain and disability. Whereas minimally invasive surgery (MIS) for lumbar disc herniation is routinely performed in adults, it has not yet been described in the pediatric population. The purpose of this study was to describe the surgical results of pediatric MIS-treated lumbar disc disease.

Methods

The authors retrospectively reviewed a series of 6 consecutive cases of lumbar microdiscectomy performed using MIS techniques between April 2008 and July 2010. Presenting symptoms, physical examination findings, and preoperative MR imaging results were obtained from medical records. Perioperative results, including blood loss, length of hospital stay, and complications were assessed. Findings at latest follow-up evaluation were also recorded.

Results

This report represents the first surgical series regarding pediatric lumbar microdiscectomies performed using MIS. The mean patient age was 16 years (range 14–17 years); there were 4 girls and 2 boys. Preoperative signs and symptoms of radiculopathic pain were notable in 100% of patients, and myotomal weakness was noted in 33% of patients. The first line of treatment in all patients was a period of conservative management lasting an average of 11.5 months (range 6–12 months). The mean intraoperative blood loss was 10.8 ml, and the mean postoperative length of stay was 1.3 days. There were no complications in this small series.

Conclusions

The treatment of pediatric lumbar disc herniation by using MIS techniques can be safe and efficacious. However, further study with a larger number of patients and longer follow-up is needed to compare outcomes between MIS and open microdiscectomies.