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Thoracic spinal iophendylate-induced arachnoiditis mimicking an intramedullary spinal cord neoplasm

Case report

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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Insertion of magnetically controlled growing rods in a patient with a diaphragmatic pacemaker: case report

Andrew C. Vivas, Steven W. Hwang, and Joshua M. Pahys

Phrenic stimulators offer an alternative to standard mechanical ventilation as well as the potential for ventilator independence in select patients with chronic respiratory failure. Young patients (< 10 years old) with high cervical spinal cord injuries often develop paralytic scoliosis due to loss of muscle tone caudal to their spinal cord lesion. Growing rod systems allow for stabilization of spinal deformity while permitting continued growth of the spine and thoracic cavity. Magnetically controlled growing rods (MCGRs) offer the advantage of noninvasive expansion, as opposed to the operative expansion required in traditional growing rod systems. To the authors’ knowledge, this is the first reported case of MCGRs in a patient with a diaphragmatic pacemaker (DP). A 7-year-old boy with ventilator dependence after a high cervical spinal cord injury presented to the authors’ institution with paralytic scoliosis that progressed to > 120°. The patient had previously undergone insertion of phrenic nerve stimulators for diaphragmatic pacing. The decision was made to insert MCGRs bilaterally to stabilize his deformity, because the planned lengthening surgeries that are necessary with traditional growing rods would be poorly tolerated in this patient. The patient’s surgery and postoperative course were uneventful. The DP remained functional after insertion and lengthening of the MCGRs by using the external magnet. The DP had no effect on the expansion capability of the MCGRs. In conclusion, the MCGRs appear to be compatible with the DP. Further studies are needed to validate the long-term safety and compatibility of these 2 devices.

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Neuromonitoring changes in pediatric spinal deformity surgery: a single-institution experience

Clinical article

Joseph Ferguson, Steven W. Hwang, Zachary Tataryn, and Amer F. Samdani

Object

Intraoperative monitoring of the spinal cord has become the standard of care during surgery for pediatric spinal deformity correction. The use of both somatosensory and motor evoked potentials has dramatically increased the sensitivity and specificity of detecting intraoperative neurophysiological changes to the spinal cord, which assists in the intraoperative decision-making process. The authors report on a large, single-center experience with neuromonitoring changes and outline the surgical management of patients who experience significant neuromonitoring changes during spinal deformity correction surgery.

Methods

The authors conducted a retrospective review of all cases involving pediatric patients who underwent spinal deformity correction surgery at Shriners Hospital for Children, Philadelphia, between January 2007 and March 2010. Five hundred nineteen consecutive cases were reviewed in which neuromonitoring was used, with 47 cases being identified as having significant changes in somatosensory evoked potentials, motor evoked potentials, or both. These cases were reviewed for patient demographic data and surgical characteristics.

Results

The incidence of significant neuromonitoring changes was 9.1% (47 of 519 cases), including 6 cases of abnormal Stagnara wake-up tests, of which 4 had corroborated postoperative neurological deficits (8.5% of 47 cases, 0.8% of 519). In response to neuromonitoring changes, wake-up tests were performed in 37 (79%) of 47 cases, hardware was adjusted in 15 (32%), anesthesiology interventions were reported in 5 (11%), hardware was removed in 5 (11%), the patient was successfully repositioned in 3 (6%), and the procedure was aborted in 13 (28%). In 1 of the 4 patients with new postoperative deficits, the deficit had fully resolved by the last follow-up; the other 3 patients had persistent neurological impairment as of the most recent follow-up examination. The authors observed a sensitivity of 100% for intraoperative neuromonitoring.

Conclusions

Due to the profound risks associated with spinal deformity surgery, intraoperative neurophysiological monitoring is an integral tool to warn of impending spinal cord injury. Intraoperative neuromonitoring appears to provide a safe and useful warning mechanism to minimize spinal cord injury that may arise during scoliosis correction surgery in pediatric patients.

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Spontaneous atraumatic vertebral artery occlusion due to physiological cervical extension

Case report

Mina G. Safain, Jordan Talan, Adel M. Malek, and Steven W. Hwang

Vertebral artery (VA) occlusion is a serious and potentially life-threatening occurrence. Bow hunter's syndrome, a mechanical occlusion of the VA due to physiological head rotation, has been well described in the medical literature. However, mechanical VA compression due to routine flexion or extension of the neck has not been previously reported. The authors present the unique case of a woman without any history of trauma who had multiple posterior fossa strokes and was found to have dynamic occlusion of her right VA visualized via cerebral angiogram upon extension of her neck. This occlusion was attributed to instability at the occipitocervical junction in a patient with a previously unknown congenital fusion of both the occiput to C-1 and C-2 to C-3. An occiput to C-3 fusion was performed to stabilize her cervical spine and minimize the dynamic vascular compression. A postoperative angiogram showed no evidence of restricted flow with flexion or extension of the neck. This case emphasizes the importance of considering symptoms of vertebrobasilar insufficiency as a result of physiological head movement. The authors also review the literature on VA compression resulting from physiological head movement as well as strategies for clinical diagnosis and treatment.

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The impact of segmental and en bloc derotation maneuvers on scoliosis correction and rib prominence in adolescent idiopathic scoliosis

Clinical article

Steven W. Hwang, Amer F. Samdani, and Patrick J. Cahill

Object

Idiopathic scoliosis is a pathological process influencing the spinal column in 3 dimensions. Initial surgical treatment focused primarily on correction in the coronal plane, and with improved instrumentation, increasing attention has targeted balancing the sagittal profile. Newer surgical techniques now permit operative corrective forces to also directly address axial rotation. Although several technical variations of direct vertebral body derotation (DVBD) have been devised, no studies have compared outcomes from the differing techniques. The purpose of this study was to describe and compare the differences between segmental and en bloc DVBD.

Methods

A large prospectively collected database was queried for patients with adolescent idiopathic scoliosis (AIS) who underwent posterior spinal fusion and for whom there was a minimum of 2 years of follow-up. In all patients some type of DVBD maneuver was performed (segmental, en bloc, or both). Any patients with concurrent thoracoplasties were excluded.

Results

The authors identified 188 patients, of whom 120 underwent segmental derotation, 17 en bloc derotation, and 51 both. No significant radiographic or clinical differences existed among the groups preoperatively. The mean preoperative thoracic curve in the entire cohort was 53.1° ± 14.1° and the mean thoracic rib prominence was 14.0° ± 5.5°, whereas the respective postoperative values were 19.3° ± 8.3° and 7.2° ± 4.0°. No significant difference was identified between the various techniques postoperatively, either. However, when comparing intraoperative variables, significant differences were found for operative duration (p = 0.0001), estimated blood loss (p = 0.0081), and volume of blood transfusions (p = 0.041).

Conclusions

Although each surgical technique of DBVD may have theoretical benefits and risks, no apparent difference in outcomes was observed between techniques. The concurrent use of both techniques was associated with increased blood loss and operative duration without any appreciable benefit. The surgeon should adopt the derotation technique with which he or she is most comfortable, but concurrent use of both does not appear to improve results.

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Open reduction and internal fixation for angulated, unstable odontoid synchondrosis fractures in children: a safe alternative to halo fixation?

Report of 2 cases

Daniel H. Fulkerson, Steven W. Hwang, Akash J. Patel, and Andrew Jea

External orthosis is the accepted and historical management of odontoid synchondrosis fractures; however, this conservative therapy carries a significant complication and fracture nonunion rate among young children. The purpose of this study was to evaluate the authors' own experience in the context of the literature, to explore surgical fixation as a primary treatment for unstable fractures. The authors retrospectively reviewed 2 cases of unstable odontoid synchondrosis fractures treated at their institution; both showed radiographic progression of deformity and subsequently underwent an open surgical reduction and fusion. A literature review was conducted to compare the authors' management strategy with those in published data. External orthosis for treatment of odontoid synchondrosis fractures has a strong history of success. However, in the literature, patients treated with a halo orthosis had a 43.3% rate of complications and an 11.4% risk of nonunion. There are radiographic findings that suggest instability, such as severe angulation and displacement of the odontoid process. Both patients in the present report underwent successful fusion without complication, as documented on CT scans obtained 3 months after surgery. Given the high rate of fusion attained with conservative therapy, it is recommended for most synchondrosis fractures. However, there is a recognized subgroup of synchondrosis fractures with severe angulation (> 30°) and displacement suggestive of significant ligamentous injury. In these patients, surgical fixation may be a safe and efficacious alternative to halo orthosis as the primary treatment.

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Spinal column shortening for tethered cord syndrome associated with myelomeningocele, lumbosacral lipoma, and lipomyelomeningocele in children and young adults

Guillermo Aldave, Daniel Hansen, Steven W. Hwang, Amee Moreno, Valentina Briceño, and Andrew Jea

OBJECTIVE

Tethered cord syndrome is the clinical manifestation of an abnormal stretch on the spinal cord, presumably causing mechanical injury, a compromised blood supply, and altered spinal cord metabolism. Tethered cord release is the standard treatment for tethered cord syndrome. However, direct untethering of the spinal cord carries potential risks, such as new neurological deficits from spinal cord injury, a CSF leak from opening the dura, and retethering of the spinal cord from normal scar formation after surgery. To avoid these risks, the authors applied spinal column shortening to children and transitional adults with primary and secondary tethered cord syndrome and report treatment outcomes. The authors' aim with this study was to determine the safety and efficacy of spinal column shortening for tethered cord syndrome by analyzing their experience with this surgical technique.

METHODS

The authors retrospectively reviewed the demographic and procedural data of children and young adults who had undergone spinal column shortening for primary or secondary tethered cord syndrome.

RESULTS

Seven patients with tethered cord syndrome caused by myelomeningocele, lipomyelomeningocele, and transitional spinal lipoma were treated with spinal column shortening. One patient with less than 24 months of follow-up was excluded from further analysis. There were 3 males and 4 females; the average age at the time was surgery was 16 years (range 8–30 years). Clinical presentations for our patients included pain (in 5 patients), weakness (in 4 patients), and bowel/bladder dysfunction (in 4 patients). Spinal column osteotomy was most commonly performed at the L-1 level, with fusion between T-12 and L-2 using a pedicle screw-rod construct. Pedicle subtraction osteotomy was performed in 6 patients, and vertebral column resection was performed in 1 patient. The average follow-up period was 31 months (range 26–37 months). Computed tomography–based radiographic outcomes showed solid fusion and no instrumentation failure in all cases by the most recent follow-up. Five of 7 patients (71%) reported improvement in preoperative symptoms during the follow-up period. The mean differences in initial and most recent Scoliosis Research Society Outcomes Questionnaire and Oswestry Disability Index scores were 0.26 and –13%, respectively; minimum clinically important difference in SRS-22 and ODI were assumed to be 0.4% and –12.8%, respectively.

CONCLUSIONS

Spinal column shortening seems to represent a safe and efficacious alternative to traditional untethering of the spinal cord for tethered cord syndrome.

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Obliteration of a metameric spinal arteriovenous malformation (Cobb syndrome) using combined endovascular embolization and surgical excision

Case report

Clemens M. Schirmer, Steven W. Hwang, Ron I. Riesenburger, In Sup Choi, and Carlos A. David

Cobb syndrome represents the concurrent findings of a metameric spinal vascular malformation and a cutaneous vascular malformation within several dermatomes of each other. This rare entity engenders many difficult decisions with respect to appropriate therapeutic management. Historically, surgical excision carried a high morbidity, and conservative management without intervention was preferred. More recently, several cases of endovascular embolization have been reported with good success.

The authors describe the case of a 17-year-old boy who presented with a right gluteal angioma and was found to have a spinal arteriovenous malformation. Multiple embolizations failed to prevent neurological deterioration, and the patient eventually became wheelchair dependent. Surgical excision of the malformation led to partial recovery of neurological function, and at the latest follow-up, 52 months postoperatively, the patient was able to ambulate independently. This case demonstrates the successful treatment of a patient with Cobb syndrome with surgical excision after multiple refractory embolizations. A multidisciplinary approach, which balances the patient's current neurological function against the risks and potential gains from any interventional and surgical procedure, is recommended.

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Perioperative complications and risk factors in neuromuscular scoliosis surgery

Brandon J. Toll, Amer F. Samdani, M. Burhan Janjua, Shashank Gandhi, Joshua M. Pahys, and Steven W. Hwang

OBJECTIVE

High rates of perioperative complications are associated with deformity correction in neuromuscular scoliosis. The current study aimed to evaluate complications associated with surgical correction of neuromuscular scoliosis and to characterize potential risk factors.

METHODS

Data were retrospectively collected from a single-center cohort of 102 consecutive patients who underwent spinal fusions for neuromuscular scoliosis between January 2008 and December 2016 and who had a minimum of 6 months of follow-up. A subgroup analysis was performed on data from patients who had at least 2 years of follow-up. Univariate and multivariate regression analyses, as well as binary correlational models and Student t-tests, were employed for further statistical analysis.

RESULTS

The present cohort had 53 boys and 49 girls with a mean age at surgery of 14.0 years (± 2.7 SD, range 7.5–19.5 years). The most prevalent diagnoses were cerebral palsy (26.5%), spinal cord injury (24.5%), and neurofibromatosis (10.8%). Analysis reflected an overall perioperative complication rate of 27% (37 complications in 27 patients), 81.1% of which constituted major complications (n = 30) compared to a rate of 18.9% for minor complications (n = 7). Complications were predicted by nonambulatory status (p = 0.037), increased intraoperative blood loss (p = 0.012), increased intraoperative time (p = 0.046), greater pelvic obliquity at follow-up (p = 0.028), and greater magnitude of sagittal profile at follow-up (p = 0.048). Pulmonary comorbidity (p = 0.001), previous operations (p = 0.013), history of seizures (p = 0.046), diagnosis of myelomeningocele (p = 0.046), increase in weight postoperatively (p < 0.005), and increased lumbar lordosis at follow-up (p = 0.015) were identified as risk factors for perioperative infection.

CONCLUSIONS

These results suggest that in neuromuscular scoliosis, patients with preexisting pulmonary compromise and greater intraoperative blood loss have the greatest risk of experiencing a major perioperative complication following surgical deformity correction.

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The periinsular functional hemispherotomy

Leonardo Rangel-Castilla, Steven W. Hwang, George Al-Shamy, Andrew Jea, and Daniel J. Curry

The surgical treatment of refractory epilepsy has evolved as new innovations have been created. Disconnective procedures such as hemispherectomy have evolved. Presently, hemispherotomy has replaced hemispherectomy to reduce complication rates while maintaining good seizure control. Several disconnective techniques have been described including the Rasmussen, vertical, and lateral approaches. The lateral approach, or periinsular hemispherectomy, was derived from modifications on the functional hemispherectomy and involves removal of the temporal lobe mesial structures, exposure of the atrium via the circular sulcus, internal capsule transection under the central sulcus, intraventricular callosotomy, and frontobasal disconnection. The purpose of this article is to describe and illustrate in detail the anatomy and operative technique for periinsular hemispherotomy, as well as to discuss the nuances and issues involved with this procedure.