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Steven W. Hwang, Rafeeque A. Bhadelia and Julian Wu

✓Iophendylate (Pantopaque or Myodil) was commonly used from the 1940s until the late 1980s for myelography, cisternography, and ventriculography. Although such instances are rare, several different long-term sequelae have been described in the literature and associated with intrathecal iophendylate. The authors describe an unusual case of arachnoiditis caused by residual thoracic iophendylate imitating an expansile intramedullary lesion on magnetic resonance images obtained 30 years after the initial myelographic injection.

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Ron I. Riesenburger, Steven W. Hwang, Clemens M. Schirmer, Vasilios Zerris, Julian K. Wu, Kerry Mahn, Paul Klimo Jr., John Mignano, Clinton J. Thompson and Kevin C. Yao

Object

Gamma Knife surgery (GKS) has been shown to be effective in treating trigeminal neuralgia (TN). Existing studies have demonstrated success rates of 69.1–85% with median follow-up intervals of 19–60 months. However, series with uniform long-term follow-up data for all patients have been lacking. In the present study the authors examined outcomes in a series of patients with TN who underwent a single GKS treatment followed by a minimum follow-up of 36 months. They used a clinical scale that simplifies the reporting of outcome data for patients with TN.

Methods

Fifty-three consecutive patients with typical, intractable TN received a median maximum radiation dose of 80 Gy applied with a single 4-mm isocenter to the affected trigeminal nerve. Follow-up data were obtained by clinical examination and questionnaire. Outcome results were categorized into the following classes (in order of decreasing success): Class 1A, complete pain relief without medications; 1B, complete pain relief with either a decrease or no change in medications; 1C, ≥ 50% pain relief without medications; 1D, ≥ 50% pain relief with either a decrease or no change in medications; and Class 2, < 50% pain relief and/or increase in medications. Patients with Class 1A–1D outcome (equivalent to Barrow Neurological Institute Grades I–IIIb) were considered to have a good treatment outcome, whereas in patients with Class 2 outcome (equivalent to Barrow Neurological Institute Grades IV and V) treatment was considered to have failed.

Results

A good treatment outcome from initial GKS was achieved in 31 (58.5%) patients for whom the mean follow-up period was 48 months (range 36–66 months). Outcomes at last follow-up were reflected by class status: Class 1A, 32.1% of patients; 1B, 1.9%; 1C, 3.8%; 1D, 20.8%; and Class 2, 41.5%. Statistical analysis showed no difference in outcomes between patients previously treated with microvascular decompression or rhizotomy compared with patients with no previous surgical treatments. Thirty-six percent of patients reported some degree of posttreatment facial numbness. Anesthesia dolorosa did not develop in any patient.

Conclusions

Despite a time-dependent deterioration in the success rate of GKS for medically intractable TN, the authors' study showed that > 50% of patients can be expected to have a good outcome based on their scoring system, with ~ 33% having an ideal outcome (pain free with no need for medications). Long-term data, as those presented here, are important when counseling patients on their treatment options.

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Oral Presentations

2010 AANS Annual Meeting Philadelphia, Pennsylvania May 1–5, 2010

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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.

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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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Steven W. Hwang, George Al-Shamy, William E. Whitehead, Daniel J. Curry, Robert Dauser, Thomas G. Luerssen and Andrew Jea

Endoscopic third ventriculostomy (ETV) is an accepted option in the treatment of obstructive hydrocephalus in children and is considered by many pediatric neurosurgeons to be the treatment of choice in this population. The procedure involves perforation of the floor of the third ventricle, specifically, the tuber cinereum, which is part of the hypothalamic-pituitary axis of cerebral endocrine regulation. Endocrine dysfunction, such as amenorrhea, weight gain, and precocious puberty, which are recognized only months to years after the procedure, may be underreported because patients and physicians may not relate the endocrine sequelae to the ETV. Few detailed reports of endocrinerelated complications following ETV exist to better understand these issues. In this study, the authors add to the literature with case descriptions of and correlative laboratory findings in 2 adolescent girls who underwent ETV for obstructive hydrocephalus and in whom amenorrhea subsequently developed.

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Steven W. Hwang, Amer F. Samdani, Mark Tantorski, Patrick Cahill, Jason Nydick, Anthony Fine, Randal R. Betz and M. Darryl Antonacci

Object

Several studies have characterized the relationship among postoperative thoracic, lumbar, and pelvic alignment in the sagittal plane. However, little is known of the relationship between postoperative thoracic kyphosis and sagittal cervical alignment in patients with adolescent idiopathic scoliosis (AIS) treated with all pedicle screw constructs. The authors examined this relationship and associated factors.

Methods

A prospective database of pediatric patients with AIS undergoing spinal fusion between 2003 and 2005 was reviewed for those who received predominantly pedicle screw constructs for Lenke Type 1 or Type 2 curves. Parameters analyzed on pre- and postoperative radiographs were the fusion levels; cervical, thoracic, and lumbar sagittal balance; and C-2 and C-7 plumb lines.

Results

Preoperatively, 6 (Group A) of the 22 patients included in the study had frank cervical kyphosis (mean angle 13.0°) with mean associated thoracic kyphosis of 27.2° (range 16°–37°). Postoperatively, cervical kyphosis (13.0°) remained in the patients in Group A along with mean thoracic kyphosis of 17.7° (range 4°–26°, p < 0.05). Preoperatively, the remaining 16 of 22 patients had neutral to lordotic cervical alignment (mean −13.8°) with thoracic kyphosis (mean 45°, range 30°–76°). Postoperatively, 8 (Group B) of these 16 patients demonstrated cervical sagittal decompensation (> 5° kyphosis), with 6 showing frank cervical kyphosis (10.5°, p < 0.05). In Group B, the mean postoperative thoracic kyphosis was 25.6° (range 7°–49°, p < 0.05). The other 8 patients (Group C) had mean postoperative thoracic kyphosis of 44.1° (range 32°–65°), and there was no cervical decompensation (p < 0.05).

Conclusions

The sagittal profile of the thoracic spine is related to that of the cervical spine. The surgical treatment of Lenke Type 1 and 2 curves by using all pedicle screw constructs has a significant hypokyphotic effect on thoracic sagittal plane alignment (19 [86%] of 22 patients). If postoperative thoracic kyphosis is excessively decreased (mean 25.6°, p < 0.05), the cervical spine may decompensate into significant kyphosis.

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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.