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Ian S. Mutchnick, Rashid M. Janjua, Karen Moeller and Thomas M. Moriarty

Object

The optimal surgical management of Chiari malformation (CM) is evolving. Evidence continues to accrue that supports decompression without duraplasty as an effective treatment to achieve symptomatic relief and anatomical decompression. The risks and benefits of this less invasive operation need to be weighed against decompression with duraplasty.

Methods

The authors performed a retrospective review of all CM decompressions from 2003 to 2007. All operations were performed by a single surgeon at a single institution. Data were analyzed for outcome, postoperative morbidity, and recurrence.

Results

Of 121 unique patients, 56 underwent posterior fossa decompressions without duraplasty (PFD) and 64 patients underwent posterior fossa decompressions with duraplasty (PFDD). Of the 56 PFD patients, 7 (12.5%) needed a subsequent PFDD for symptomatic recurrence. Of the 64 patients who underwent a PFDD, 2 (3.1%) needed a repeated PFDD for symptomatic recurrence. Patients treated with PFDD had an average operative time of 201 minutes in contrast to 127 minutes for those who underwent PFD (p = 0.0001). Patients treated with PFDD had average hospital stays of 4.0 days, whereas that for patients treated with PFD was 2.7 days (p = 0.0001). While in the hospital, patients treated with PFDD used low-grade narcotics, intravenous narcotics, muscle relaxants, and antiemetic medications at statistically significant differing rates.

Conclusions

While PFD was associated with a higher rate of recurrent symptoms requiring repeated decompression, this may be justified by the significantly lower morbidity rate. Clearer delineation of the trade-off between morbidity and recurrence may be used to help patients and their families make decisions regarding care.

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Ian S. Mutchnick, Travis E. Clegg, Leah Y. Carreon and Rolando M. Puno

Object

The current standard of care for symptomatic chronic spondylolysis (SP) is a one-level posterior spinal fusion for defects at L-5 or direct pars repair (motion segment sparing) for more rostral SP in younger patients and if no disc degeneration or listhesis is present. Since many patients with SP undergoing operative repair are young, a procedure with the lowest biomechanical profile is desirable, and direct pars repair is recommended. The authors here explore the limits of direct pars repair.

Methods

A retrospective review of all patients who underwent direct repair of SP between 2002 and 2009 was performed. Data were analyzed for predictors of symptom relief and radiographic fusion failure.

Results

Of 49 patients, only 7 required a reoperation to treat clinical symptoms, and 6 of them were female (p = 0.049). In all cases of treatment failure, the patient had bilateral L-5 SP. Patients with a slip percentage as high as 30% experienced radiographic fusion and symptom relief. Disc degeneration (measured using the Modified Pfirrmann Scale) did not predict symptom persistence or radiographic fusion failure. Patients with high-grade disc disease experienced symptom relief. The authors found no predictors of treatment failure.

Conclusions

The number of patients undergoing motion segment–sparing fusions of symptomatic chronic SP can be safely increased to include patients with Grade I spondylolisthesis as well as high-grade disc disease. Female patients with bilateral L-5 SP and low lordotic angles may be better served by a posterior spinal fusion from L-5 to S-1.

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David F. Jimenez

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Ian S. Mutchnick and Todd A. Maugans

Object

Multiple surgical procedures have been described for the management of isolated nonsyndromic sagittal synostosis. Minimally invasive techniques have been recently emphasized, but these techniques necessitate the use of an endoscope and postoperative helmeting. The authors assert that a safe and effective, more “minimalistic” approach is possible, avoiding the use of endoscopic visualization and routine postoperative application of a cranial orthosis.

Methods

A single-institution cohort analysis was performed on 18 cases involving infants treated for isolated nonsyndromic sagittal synostosis between 2008 and 2010 using a nonendoscopic, minimally invasive calvarial vault remodeling (CVR) procedure without postoperative helmeting. The surgical technique is described. Variables analyzed were: age at time of surgery, sex, estimated blood loss (EBL), operative time, intraoperative complications, postoperative complications, length of stay, pre- and postoperative cephalic index (CI), clinical impressions, and results of a 5-question nonstandardized questionnaire administered to patient caregivers regarding outcome.

Results

Eleven male and 7 female infants (mean age 2.3 months) were included in the study. The mean duration of follow-up was 16.4 months (range 6–38 months). The mean procedural time was 111 minutes (range 44–161 minutes). The mean length of stay was 2.3 days (range 2–3 days). The mean EBL in all 18 patients was 101.4 ml (range 30–475 ml). One patient had significant bone bleeding resulting in an EBL of 475 ml. Excluding this patient, the mean EBL was 79.4 ml (range 30–150 ml). There were no deaths or intraoperative complications; one patient had a superficial wound infection. The mean CI was 69 preoperatively versus 79 postoperatively, a statistically significant difference (p < 0.0001). Two patients were offered helmeting for suboptimal surgical outcome; one family declined and the single helmeted patient showed improvement at 2 months. No patient has undergone further surgery for correction of primary deformity, secondary deformities, or bony irregularities. Complete questionnaire data were available for 14 (78%) of the 18 patients; 86% of the respondents were pleased with the cosmetic outcome, 92% were happy to have avoided helmeting, 72% were doubtful that helmeting would have provided more significant correction, and 86% were doubtful that further surgery would be necessary. Small, palpable, aesthetically insignificant skull irregularities were reported by family members in 6 cases (43%).

Conclusions

The authors present a nonendoscopic, minimally invasive CVR procedure without postoperative helmeting. Their small series demonstrates this to be a safe and efficacious procedure for isolated nonsyndromic sagittal synostosis, with improvements in CI at a mean follow-up of 16.1 months, commensurate with other techniques, and with overall high family satisfaction. Use of a CVR cranial orthosis in a delayed fashion can be effective for the infrequent patient in whom this approach results in suboptimal correction.

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William C. Gump, Ian S. Mutchnick and Thomas M. Moriarty

Molding helmet therapy is a widely accepted treatment for positional plagiocephaly that is generally considered to be low risk. Multiple large outcome studies have shown good results, but adverse events are rarely reported. The literature on helmet therapy was reviewed to clarify the clinical experience with associated complications. Although significant complications were extremely rare, there was a large degree of variability in detection of lesser problems such as minor skin irritation. Patients with a primarily brachycephalic morphology may be at higher risk for poorly fitting orthoses. Most reported complications are minor and self-limited. Maintenance of good helmet hygiene appears to be the most effective strategy for reducing or eliminating complications.

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William C. Gump, Ian S. Mutchnick and Thomas M. Moriarty

Children with spastic diplegia from cerebral palsy (CP) experience measurable improvement in their spasticity and motor function following selective dorsal rhizotomy (SDR). The role of this operation in the treatment of other spasticity causes is less well defined. A literature review was undertaken to survey outcomes from SDRs performed outside the CP population. Multiple sclerosis was the most common diagnosis found, accounting for 74 of 145 patients described. Selective dorsal rhizotomies have also been reported in patients with traumatic brain and spinal cord injuries, ischemic and hemorrhagic stroke, neurodegenerative disease, hypoxic encephalopathy, and other causes of spasticity. Outcomes from surgery are generally described as favorable, although postoperative assessments and follow-up times are not standardized across reports. Long-term outcomes are sparsely reported. Larger numbers of patients and more detailed outcomes data have the potential to form a basis for expanding the inclusion criteria for SDR.

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Meena Thatikunta, Ian Mutchnick, Jennifer Elster, Matthew P. Thompson, Michael A. Huang, Aaron C. Spalding and Thomas Moriarty

Atypical teratoid rhabdoid tumors (ATRTs) are a rare pediatric brain tumor with high mortality rate. Several large series have reported achieving gross-total resection (GTR) in less than 50% of patients due to the lesions' large size, vascularity, and limited blood volume in young patients. While neoadjuvant chemotherapy for choroid plexus carcinomas in pediatric patients has become widely accepted, it has not been used as widely for other pediatric brain tumors. To the best of the authors' knowledge, there are only 3 published cases of neoadjuvant chemotherapy for ATRTs. In the present report, the authors present a fourth case of neoadjuvant chemotherapy for ATRT and review the available literature on this strategy. A 17-month-old child presented with a left ventricular ATRT for which imaging raised concern for a highly vascularized tumor. The authors undertook neoadjuvant chemotherapy with 2 cycles of Head Start II therapy, which reduced the size of the ventricular tumor by 35% and decreased the vascularity of the lesion on imaging. The estimated blood loss during resection was 425 ml and GTR was achieved. The patient continued with postoperative chemotherapy but suffered an on-therapy recurrence. While higher-quality data are necessary, available evidence suggests that neoadjuvant chemotherapy can reduce the size and vascularity of ATRTs and facilitate a surgical avenue for large or “inoperable” tumors.

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Meena Thatikunta, Jeffrey Bumpous, Jarrod Little, Samir Karia, Nicole R. Herring, Mohammed Nuru, Nitin Engineer and Ian Mutchnick

Primary encephaloceles (PEs) present only rarely in the temporal region; in the rare instance that they project through the floor of the middle fossa they are secondary. In this case report the authors report on the management of a giant PE extending through the floor of the middle fossa.

An 8-month-old boy presented to the authors’ service with a large PE projecting into his neck through a missing left middle fossa floor; the lesion was causing significant meta-, dys-, and hypoplasia of the structures of the anterolateral neck on that side. Surgical goals for this patient included the following: 1) removal of potentially epileptogenic and dysfunctional tissue; 2) preservation of cranial nerves; 3) prevention of cognitive decline or iatrogenic deficit; 4) prevention of CSF leak; 5) reconstruction of skull base; 6) prevention of airway and swallowing compromise; and 7) cosmesis. After a multidisciplinary evaluation with ENT, plastic surgery, and neurology, an operation was performed using a preauricular infratemporal approach when the patient was 3 years old. Gliotic tissue was resected and amygdala, hippocampus, and middle cerebral artery were preserved.

The immediate results of the operation showed good immediate outcome. Seizure freedom and neurodevelopment outcomes remain to be seen at follow-up.