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Analiz Rodriguez, Elizabeth N. Kuhn, Aravind Somasundaram and Daniel E. Couture


Syringohydromyelia is frequently identified on spinal imaging. The literature provides little guidance to decision making regarding the need for follow-up or treatment. The purpose of this study was to review the authors' experience in managing pediatric syringohydromyelia of unknown cause.


A single-institution retrospective review of all cases involving pediatric patients who underwent spinal MRI from 2002 to 2012 was conducted. Patients with idiopathic syringohydromyelia (IS) were identified and categorized into 2 subgroups: uncomplicated idiopathic syrinx and IS associated with scoliosis. Clinical and radiological course were analyzed.


Ninety-eight patients (50 female, 48 male) met the inclusion criteria. Median age at diagnosis of syrinx was 11.9 years. Median maximum syrinx size was 2 mm (range 0.5–17 mm) and spanned 5 vertebral levels (range 1–20 vertebral levels). Thirty-seven patients had scoliosis. The most common presenting complaint was back pain (26%). Clinical follow-up was available for 78 patients (80%), with a median follow-up of 20.5 months (range 1–143 months). A neurological deficit existed at presentation in 36% of the patients; this was either stable or improved at last follow-up in 64% of cases. Radiological follow-up was available for 38 patients (39%), with a median duration of 13 months (range 2–83 months). There was no change in syrinx size in 76% of patients, while 16% had a decrease and 8% had an increase in syrinx size. Thirty-six patients had both clinical and radiological follow-up. There was concordance between clinical and radiological course in 14 patients (39%), with 11 patients (31%) showing no change and 3 patients (8%) showing clinical and radiological improvement.

No patients had concurrent deterioration in clinical and radiological course. One patient with scoliosis and muscular dystrophy underwent direct surgical treatment of the syrinx and subsequently had a deteriorated clinical course and decreased syrinx size.


There remains a paucity of data regarding the management of pediatric IS. IS in association with scoliosis can complicate neurosurgical decision making. There was no concordance between radiological syrinx size increase and clinical deterioration in this cohort, indicating that surgical decision making should reflect clinical course as opposed to radiological course.

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Aravind Somasundaram, Glenn J. Lesser, Ryan T. Mott and Wesley Hsu

Malignant transformation of epidermoid cysts (ECs) to squamous cell carcinomas (SCCs) in the CNS is exceedingly rare and has only been described in intracranial ECs. In this article, the authors describe a 53-year-old man with a history of a previously resected T3–4 EC, who presented with a 2-month history of progressively worsening weakness in the left side of his body. Magnetic resonance imaging revealed an enhancing mass in the T3–4 region, the exact location of the previous cyst. The mass was resected in gross-total fashion, and pathological analysis revealed an SCC. Postoperatively, the patient regained full strength in his lower extremities. After the resection, he received radiotherapy administered at an isodose of 50 Gy. To the authors' knowledge, this is the first reported case of malignant transformation of an intramedullary spinal EC in the literature.

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Daniel E. Couture, John C. Crantford, Aravind Somasundaram, Claire Sanger, Anne E. Argenta and Lisa R. David


There has been a tremendous increase in the incidence of deformational plagiocephaly in children throughout the world. Therapeutic options include observation, active counterpositioning, external orthotics, and surgery. The current treatment in the US is highly debated, but it typically includes external orthotic helmets in patients with moderate to severe plagiocephaly presenting between 4 and 10 months of age or in children with significant comorbidities limiting passive (no-pressure) therapy. The present study was designed to evaluate 3 key issues: 1) the accuracy of the Argenta classification in defining a progressive degree of severity, 2) identification of an upper age limit when treatment is no longer effective, and 3) the effectiveness of an off-the-shelf prefabricated helmet in correcting deformational plagiocephaly.


An institutional review board–approved retrospective study was conducted of all patients at the authors' clinic in whom deformational plagiocephaly was assessed using the Argenta classification system over a 6-year period; the patients underwent helmet therapy, and a minimum of 3 clinic visits were recorded. Inclusion criteria consisted of an Argenta Type II–V plagiocephalic deformity. Patients' conditions were categorized both by severity of the deformity and by patients' age at presentation. Statistical analysis was conducted using survival analysis.


There were 1050 patients included in the study. Patients with Type III, IV, and V plagiocephaly required progressively longer for deformity correction to be achieved than patients with Type II plagiocephaly (53%, 75%, and 81% longer, respectively [p < 0.0001]). This finding verified that the Argenta stratification indicated a progressive severity of deformity. No statistically significant difference in the time to correction was noted among the different age categories, which suggests that the previously held upper time limit for correction may be inaccurate. An overall correction rate to Type I plagiocephaly of 81.6% was achieved irrespective of severity and degree of the original deformity. This suggests that an inexpensive off-the-shelf molding helmet is highly effective and that expensive custom-fitted orthoses may not be necessary. The patients in the older age group (> 12 months) did not have a statistically significant longer interval to correction than the patients in the youngest age group (< 3 months). The mean length of follow-up was 6.3 months.


Patients treated with passive helmet therapy in the older age group (> 12 months) had an improvement in skull shape within the same treatment interval as the patients in the younger age group (< 3 months). This study supports the use of passive helmet therapy for improvement in deformational plagiocephaly in infants from birth to 18 months of age and verifies the stratification of degree of deformity used in the Argenta classification system.

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Aravind Somasundaram, Robert T. Wicks, Adrian L. Lata, Shadi A. Qasem and Wesley Hsu

In this article, the authors describe a 48-year-old man who initially presented with progressively worsening back pain. Magnetic resonance imaging revealed a soft-tissue mass involving the T10–11 vertebral bodies with extension anteriorly into the aorta as well as epidural extension without spinal cord compression. A biopsy of the mass showed findings consistent with a malignant fibrous histiocytoma (MFH). A total en bloc spondylectomy with resection and reconstruction of the involved aorta using a vascular graft was performed. The patient received postoperative radiation therapy and is neurologically intact at 18 months postoperatively. To the authors' knowledge, this is the first reported case of a spinal MFH resection with aortic reconstruction.

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Analiz Rodriguez, Matthew T. Neal, Ann Liu, Aravind Somasundaram, Wesley Hsu and Charles L. Branch Jr


Symptomatic adjacent-segment lumbar disease (ASLD) after lumbar fusion often requires subsequent surgical intervention. The authors report utilizing cortical bone trajectory (CBT) pedicle screw fixation with intraoperative CT (O-arm) image-guided navigation to stabilize spinal levels in patients with symptomatic ASLD. This unique technique results in the placement of 2 screws in the same pedicle (1 traditional pedicle trajectory and 1 CBT) and obviates the need to remove preexisting instrumentation.


The records of 5 consecutive patients who underwent lumbar spinal fusion with CBT and posterior interbody grafting for ASLD were retrospectively reviewed. All patients underwent screw trajectory planning with the O-arm in conjunction with the StealthStation navigation system. Basic demographics, operative details, and radiographic and clinical outcomes were obtained.


The average patient age was 69.4 years (range 58–82 years). Four of the 5 surgeries were performed with the Minimal Access Spinal Technologies (MAST) Midline Lumbar Fusion (MIDLF) system. The average operative duration was 218 minutes (range 175–315 minutes). In the entire cohort, 5.5-mm cortical screws were placed in previously instrumented pedicles. The average hospital stay was 2.8 days (range 2–3 days) and there were no surgical complications. All patients had more than 6 months of radiographic and clinical follow-up (range 10–15 months). At last follow-up, all patients reported improved symptoms from their preoperative state. Radiographic follow-up showed Lenke fusion grades of A or B.


The authors present a novel fusion technique that uses CBT pedicle screw fixation in a previously instrumented pedicle with intraoperative O-arm guided navigation. This method obviates the need for hardware removal. This cohort of patients experienced good clinical results. Computed tomography navigation was critical for accurate CBT screw placement at levels where previous traditional pedicle screws were already placed for symptomatic ASLD.

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Aravind Somasundaram, Nathan Ardanowski, Charles F. Opalak, Helen L. Fillmore, Archana Chidambaram and William C. Broaddus

Glioblastoma multiforme (GBM) is the most common type of primary brain tumor, and current treatment regimens are only marginally effective. One of the most vexing and malignant aspects of GBM is its pervasive infiltration into surrounding brain tissue. This review describes the role of the Wilms tumor 1 gene (WT1) and its relationship to GBM. WT1 has several alternative splicing products, one of which, the KTS+ variant, has been demonstrated to be involved in the transcriptional activation of a variety of oncogenes as well as the inhibition of tumor suppressor genes. Further, this paper will examine the relationship of WT1 with CD97, a gene that codes for an epidermal growth factor receptor family member, an adhesion G-protein–coupled receptor, thought to promote tumor invasiveness and migration. The authors suggest that further research into WT1 and CD97 will allow clinicians to begin to deal more effectively with the infiltrative behavior displayed by GBM and design new therapies that target this deadly disease.