Rhombencephalosynapsis (RES) is a rare congenital malformation of the cerebellum characterized by hypogenesis or agenesis of the vermis and fusion of the cerebellar hemispheres with or without fusion of the dentate nuclei and superior cerebellar peduncles. No genetic or chromosomal abnormalities have been identified for RES. Although the occurrence of RES is presumed to be sporadic, no clear pattern of inheritance has been identified. The authors report on a 17-year-old girl with autosomal dominant polycystic kidney disease Type 1 as well as RES.
Robert Elliott and David H. Harter
Vishwajeet Singh, Tarush Rustagi, and Robert Hart
The sacrum forms the distal end of the spine and communicates with the pelvis. Fractures involving the sacrum are complex and may disrupt this vital communication. Neglecting these fractures may result in malunion, which often causes significant alteration in the pelvic parameters and sagittal balance. Management of ensuing deformities is complex and poorly described. The authors present a case of sacral malunion with sagittal imbalance treated with a low lumbar osteotomy.
Natalie Zusman, Jacqueline L. Munch, Alexander Ching, Robert Hart, and Jung Yoo
A lack of information exists on the relationship between preoperative epidural spinal injections and outcomes after spine surgery. There is concern that injections might cause local changes, increasing the infection risk and surgical difficulty. Therefore, the authors explored the relationship between preoperative spinal injections and postoperative outcome.
The cohort was comprised of patients who underwent thoracic and/or lumbar arthrodesis during the years 2007–2010 and had complete (preoperatively and 3 months postoperatively) outcome scores. Patients’ clinical courses were reviewed to determine the occurrence of major complications within a 30-day postoperative period. Patient-perceived outcomes were evaluated using the Oswestry Disability Index (ODI) and the SF-12 (12-Item Short Form Health Survey): mental component summary (MCS) and physical component summary (PCS) scores. Analyses were based on exposure to injections and were performed using chi-square exact tests and paired and unpaired t-tests.
Two hundred eighty patients met the inclusion criteria: 117 patients (41.8%) received and 163 patients (58.2%) did not receive preoperative epidural spinal injections. Overall, the likelihood of complication did not differ with respect to exposure (13.7% injection vs 11.7% noninjection); however, injected patients observed a 7.4-fold risk of developing surgical wound complications over noninjected patients (5.1% vs 0.6%, p = 0.02).
Patient-perceived outcomes measures demonstrated no differences between groups. Three months postoperatively, the MCS and ODI scores were similar (MCS: 49.6 ± 11.6 injection vs 47.4 ± 12.8 noninjection; ODI: 35.8 ± 18.0 vs 34.4 ± 19.1). MCS or ODI score improvement (preoperatively compared with 3 months postoperatively) did not vary between groups. Injected patients maintained a 2-point lower PCS score at entry and 3 months postoperatively as compared with noninjected peers (entry: 27.6 ± 8.2 injection vs 29.5 ± 9.3 noninjection, p = 0.09; 3 months: 33.3 ± 8.6 vs 35.7 ± 9.0, p = 0.03); the PCS score improvements between injected and noninjected groups were similar (5.7 ± 9.9 vs 6.2 ± 9.7).
Patients exposed to preoperative epidural injections had similar complication rates to those who never received a spinal injection. However, they had a greater risk of developing wound complications. These complications had no effect on short-term improvements in outcome measures.
Michael L. Levy, Robert C. Granville, David Hart, and Hal Meltzer
Object. The objective of this retrospective review was to identify patients on the pediatric neurosurgical ward with deep venous thrombosis (DVT) to develop diagnostic and treatment-related guidelines.
Methods. The authors performed a retrospective chart review of all cases of DVT presenting to the Children's Hospital between March 1986 and February 1997. Of 32 patients identified, 14 were followed by the neurosurgical service. Current records were additionally evaluated to assess follow-up condition in the patients as well as outcome. Variables included diagnosis, race, age, follow-up duration, outcome, presenting signs/symptoms, involved vessel(s), concurrent disease, diagnostic modalities, and treatment.
Patient age ranged from 1 to 16 years (mean 12.6 years, median 15 years). There were five girls and nine boys. Eight DVTs were right sided and six were left sided. Presenting symptoms included swelling of the affected extremity in 11 patients, pain in five, erythema in one, and cardiopulmonary arrest in two. Comorbidities included previous orthopedic procedures in three, brain tumors in two, and sepsis, fracture, pulmonary disease, preexisting coagulation disorders, and brain abscess in one patient each. Eight patients presented with a history of trauma. Two patients had undergone chemotherapy. Diagnostic studies included ultrasonography and venography in one, venography alone in two, computerized tomography (CT) scanning and venography in one, tagged red blood cell studies in one, ultrasonography and CT scanning in one, and ultrasonography alone in eight. In one patient an inferior vena cava filter was placed and one patient was treated with oral warfarin alone. One patient with a brain tumor died while hospitalized. In four patients there was evidence of rheumatological disease in the group of patients not treated neurosurgically. Two patients suffered recurrences during the follow-up period (mean 20 months)In the entire series of 32 patients there were five total deaths. Of these, two patients experienced six recurrences each, and one other patient suffered four recurrences. Of note, none of the recurrences was observed in patients with underlying coagulation disorders.
Conclusions. Children with DVT can experience serious complications in the form of both morbidity and mortality. Although most thrombotic complications have been found in patients with femoral lines, prolonged treatment involving a central line has been found to be a significant predictor of DVT. Multiple treatment modalities currently exist for children with DVT. Low-molecular weight heparin therapy has many benefits over unfractionated heparin agents and may be more appropriate for the prophylaxis or treatment of children and adolescents with DVT because of its acceptable safety and efficacy. Clinical data for neonates and young children remain incomplete.
Robert Elliott, Stephen Kalhorn, Donato Pacione, Howard Weiner, Jeffrey Wisoff, and David Harter
Patients with symptomatic Chiari malformation Type I (CM-I) typically exhibit a chronic, slowly progressive disease course with evolution of symptoms. However, some authors have reported acute neurological deterioration in the setting of CM-I and acquired Chiari malformations. Although brainstem dysfunction has been documented in patients with CM-II and hydrocephalus or shunt malfunction, to the authors' knowledge only 1 report describing ventriculoperitoneal (VP) shunt malfunction causing neurological deterioration in a patient with CM-I exists.
The authors report on their experience with the treatment of previously asymptomatic CM-I in 2 children who experienced quite different manifestations of acute neurological deterioration secondary to VP shunt malfunction. Presumably, VP shunt malfunction created a positive rostral pressure gradient across a stenotic foramen magnum, resulting in tetraparesis from foramen magnum syndrome in 1 patient and acute ataxia and cranial nerve deficits from syringobulbia in the other. Although urgent shunt revisions yielded partial recovery of neurological function in both patients, marked improvement occurred only after posterior fossa decompression.
Alan H. Daniels, Satoshi Kawaguchi, Alec G. Contag, Farbod Rastegar, Garrett Waagmeester, Paul A. Anderson, Melanie Arthur, and Robert A. Hart
Beginning in 2008, the Centers for Medicare and Medicaid Service (CMS) determined that certain hospital-acquired adverse events such as surgical site infection (SSI) following spine surgery should never occur. The following year, they expanded the ruling to include deep vein thrombosis (DVT) and pulmonary embolism (PE) following total joint arthroplasty. Due to their ruling that “never events” are not the payers' responsibility, CMS insists that the costs of managing these complications be borne by hospitals and health care providers, rather than billings to health care payers for additional care required in their management. Data comparing the expected costs of such adverse events in patients undergoing spine and orthopedic surgery have not previously been reported.
The California State Inpatient Database (CA-SID) from 2008 to 2009 was used for the analysis. All patients with primary procedure codes indicating anterior cervical discectomy and fusion (ACDF), posterior lumbar interbody fusion (PLIF), lumbar laminectomy (LL), total knee replacement (TKR), and total hip replacement (THR) were analyzed. Patients with diagnostic and/or treatment codes for DVT, PE, and SSI were separated from patients without these complication codes. Patients with more than 1 primary procedure code or more than 1 complication code were excluded. Median charges for treatment from primary surgery through 3 months postoperatively were calculated.
The incidence of the examined adverse events was lowest for ACDF (0.6% DVT, 0.1% PE, and 0.03% SSI) and highest for TKA (1.3% DVT, 0.3% PE, 0.6% SSI). Median inpatient charges for uncomplicated LL was $51,817, compared with $73,432 for ACDF, $143,601 for PLIF, $74,459 for THR, and $70,116 for TKR. Charges for patients with DVT ranged from $108,387 for TKR (1.5 times greater than index) to $313,536 for ACDF (4.3 times greater than index). Charges for patients with PE ranged from $127,958 for TKR (1.8 times greater than index) to $246,637 for PLIF (1.7 times greater than index). Charges for patients with SSI ranged from $168,964 for TKR (2.4 times greater than index) to $385,753 for PLIF (2.7 times greater than index).
Although incidence rates are low, adverse events of spinal procedures substantially increase the cost of care. Charges for patients experiencing DVT, PE, and SSI increased in this study by factors ranging from 1.8 to 4.3 times those for patients without such complications across 5 common spinal and orthopedic procedures. Cost projections by health care providers will need to incorporate expected costs of added care for patients experiencing such complications, assuming that the cost burden of such events continues to shift from payers to providers.
Ahmed S. Mohamed, Jung Yoo, Robert Hart, Brian T. Ragel, Jayme Hiratzka, D. Kojo Hamilton, Penelope D. Barnes, and Alexander C. Ching
The authors evaluated the efficacy of posterior instrumentation for the management of spontaneous spinal infections. Standard surgical management of spontaneous spinal infection is based on debridement of the infected tissue. However, this can be very challenging as most of these patients are medically debilitated and the surgical debridement requires a more aggressive approach to the spine either anteriorly or via an expanded posterior approach. The authors present their results using an alternative treatment method of posterior-only neuro-decompression and stabilization without formal debridement of anterior tissue for treating spontaneous spinal infection.
Fifteen consecutive patients were treated surgically by 2 of the authors. All patients had osteomyelitis and discitis and were treated postoperatively with intravenous antibiotics for at least 6 weeks. The indications for surgery were failed medical management, progressive deformity with ongoing persistent spinal infection, or neurological deficit. Patients with simple epidural abscess without bony instability were treated with laminectomy and were not included in this series. Fourteen patients were treated with posterior-only decompression and long-segment rigid fixation, without formal debridement of the infected area. One patient was treated with staged anterior and posterior surgery due to delay in treatment related to medical comorbidities. The authors examined as their outcome the ambulatory status and recurrence of deep infection requiring additional surgery or medical treatment.
Of the initial 15 patients, 10 (66%) had a minimum 2-year follow-up and 14 patients had at least 1 year of followup. There were no recurrent spinal infections. There were 3 unplanned reoperations (1 for loss of fixation, 1 for early superficial wound infection, and 1 for epidural hematoma). Nine (60%) of 15 patients were nonambulatory at presentation. At final followup, 8 of 15 patients were independently ambulatory, 6 required an assistive device, and 1 remained nonambulatory.
Long-segment fixation, without formal debridement, resulted in resolution of spinal infection in all cases and in significant neurological recovery in almost all cases. This surgical technique, when combined with aggressive antibiotic therapy and a multidisciplinary team approach, is an effective way of managing serious spinal infections in a challenging patient population.
Frank J. Schwab, Ashish Patel, Christopher I. Shaffrey, Justin S. Smith, Jean-Pierre Farcy, Oheneba Boachie-Adjei, Richard A. Hostin, Robert A. Hart, Behrooz A. Akbarnia, Douglas C. Burton, Shay Bess, and Virginie Lafage
Pedicle subtraction osteotomy (PSO) is a surgical procedure that is frequently performed on patients with sagittal spinopelvic malalignment. Although it allows for substantial spinopelvic realignment, suboptimal realignment outcomes have been reported in up to 33% of patients. The authors' objective in the present study was to identify differences in radiographic profiles and surgical procedures between patients achieving successful versus failed spinopelvic realignment following PSO.
This study is a multicenter retrospective consecutive PSO case series. The authors evaluated 99 cases involving patients who underwent PSO for sagittal spinopelvic malalignment. Because precise cutoffs of acceptable residual postoperative sagittal vertical axis (SVA) values have not been well defined, comparisons were focused between patient groups with a postoperative SVA that could be clearly considered either a success or a failure. Only cases in which the patients had a postoperative SVA of less than 50 mm (successful PSO realignment) or more than 100 mm (failed PSO realignment) were included in the analysis. Radiographic measures and PSO parameters were compared between successful and failed PSO realignments.
Seventy-nine patients met the inclusion criteria. Successful realignment was achieved in 61 patients (77%), while realignment failed in 18 (23%). Patients with failed realignment had larger preoperative SVA (mean 217.9 vs 106.7 mm, p < 0.01), larger pelvic tilt (mean 36.9° vs 30.7°, p < 0.01), larger pelvic incidence (mean 64.2° vs 53.7°, p < 0.01), and greater lumbar lordosis–pelvic incidence mismatch (−47.1° vs −30.9°, p < 0.01) compared with those in whom realignment was successful. Failed and successful realignments were similar regarding the vertebral level of the PSO, the median size of wedge resection 22.0° (interquartile range 16.5°−28.5°), and the numerical changes in pre- and postoperative spinopelvic parameters (p > 0.05).
Patients with failed PSO realignments had significantly larger preoperative spinopelvic deformity than patients in whom realignment was successful. Despite their apparent need for greater correction, the patients in the failed realignment group only received the same amount of correction as those in the successfully realigned patients. A single-level standard PSO may not achieve optimal outcome in patients with high preoperative spinopelvic sagittal malalignment. Patients with large spinopelvic deformities should receive larger osteotomies or additional corrective procedures beyond PSOs to avoid undercorrection.
Meredith V. Olds, Robert W. Griebel, Harold J. Hoffman, Marilyn Craven, Sylvester Chuang, and Hart Schutz
✓Moyamoya disease is a progressive disorder, predominantly seen in childhood, that can cause severe permanent disability. The search for effective treatment has largely been unsuccessful in the past, but recent efforts at surgical intervention have shown promising results.
The natural history of moyamoya disease, the options for treatment, and a series of patients from the Hospital for Sick Children in Toronto are reviewed. The results of surgical treatment are encouraging and the authors believe that it should be offered to all pediatric patients in the progressive stage of the disease.
Robert A. Hart, Christopher M. Domes, Brady Goodwin, Charles R. D'Amato, Jung U. Yoo, Ronald J. Turker, and Matthew F. Halsey
The ideal surgical management of high-grade spondylolisthesis remains unclear. Concerns regarding the original Bohlman transsacral interbody fusion technique with stand-alone autologous fibular strut include late graft fracture and incomplete reduction of lumbosacral kyphosis. The authors' goal was to evaluate the radiographic and surgical outcomes of patients treated for high-grade spondylolisthesis with either transsacral S-1 screws or standard pedicle screw fixation augmenting the Bohlman posterior transsacral interbody fusion technique.
A retrospective review of patients who underwent fusion for high-grade spondylolisthesis in which a Bohlman oblique posterior interbody fusion augmented with either transsacral or standard pedicle screw fixation was performed by 4 spine surgeons was completed. Estimated blood loss, operating time, perioperative complications, and need for revision surgery were evaluated. Upright pre- and postsurgical lumbar spine radiographs were compared for slip percent and slip angle.
Sixteen patients (12 female and 4 male) with an average age of 29 years (range 9–66 years) were evaluated. The average clinical follow-up was 78 months (range 5–137 months) and the average radiographic follow-up was 48 months (range 5–108 months). Ten L4–S1 and 6 L5–S1 fusions were performed. Five fibular struts and 11 titanium mesh cages were used for interbody fusion. Six patients had isolated transsacral screws placed, with 2 (33%) of the 6 requiring revision surgery for nonunion. No nonunions were observed in patients undergoing spanning pedicle screw fixation augmenting the interbody graft. Six patients experienced perioperative complications including 3 iliac crest site infections, 1 L-5 radiculopathy without motor involvement, 1 deep vein thrombosis, and 1 epidural hematoma requiring irrigation and debridement. The average estimated blood loss and operating times were 763 ml and 360 minutes, respectively. Slip percent improved from an average of 62% to 37% (n = 16; p < 0.01) and slip angle improved from an average of 18° to 8° (n = 16; p < 0.01). No patient experienced L-5 or other motor deficit postoperatively.
The modified Bohlman technique for treatment of high-grade spondylolisthesis has reproducible outcomes among multiple surgeons and results in significant improvements in slip percent and slip angle. Fusion rates were high (14 of 16; 88%), especially with spanning instrumentation augmenting the oblique interbody fusion. Rates of L-5 motor deficit were low in comparison with techniques involving reduction of the anterolisthesis.