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Kathryn Wagner, Francisco Vaz-Guimaraes, Kevin Camstra, and Sandi Lam

OBJECTIVE

Appropriately chosen candidates with medically refractory epilepsy may benefit from hemispheric disconnection. Traditionally, this involves a large surgical exposure with significant associated morbidity. Minimally invasive approaches using endoscopic assistance have been described by only a few centers. Here, the authors report on the feasibility of endoscope-assisted functional hemispherotomy in a cadaver model and its first translation into clinical practice in appropriately selected patients.

METHODS

Three silicone-injected, formalin-fixed cadaver heads were used to establish the steps of the procedure in the laboratory. The steps of disconnection were performed using standard surgical instruments and a straight endoscope. The technique was then applied in two patients who had been referred for hemispherectomy and had favorable anatomy for an endoscope-assisted approach.

RESULTS

All disconnections were performed in the cadaver model via a 4 × 2–cm paramedian keyhole craniotomy using endoscopic assistance. An additional temporal burr hole approach was marked in case the authors were unable to completely visualize the frontobasal and insular cuts from the paramedian vertical view. Their protocol was subsequently used successfully in two pediatric patients. Full disconnection was verified with postoperative tractography.

CONCLUSIONS

Full hemispheric disconnection can be accomplished with minimally invasive endoscope-assisted functional hemispherotomy. The procedure is technically feasible and can be safely applied in patients with favorable anatomy and pathology; it may lead to less surgical morbidity and faster recovery.

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Nisha Gadgil, Sandi Lam, Monika Pyarali, Michael Paldino, I-Wen Pan, and Robert C. Dauser

OBJECTIVE

Numerous surgical procedures facilitate revascularization of the ischemic brain in patients with moyamoya disease. Dural inversion is a technique in which flaps of dura mater centered around the middle meningeal artery are inverted, encouraging the formation of a rich collateral blood supply. This procedure has been used in combination with encephaloduroarteriosynangiosis for more than 20 years at the authors’ institution for the treatment of pediatric moyamoya disease. The objective of this study was to describe the clinical and radiographic outcomes for a cohort of consecutive pediatric moyamoya patients undergoing dural inversion.

METHODS

Clinical and radiographic data on patients who had undergone dural inversion in the period from 1997 to 2016 were reviewed. Univariate and multivariate logistic regression and Kaplan-Meier analyses were performed to assess the risk of postoperative stroke, functional outcome, and the angiographic degree of revascularization.

RESULTS

Dural inversion was performed on 169 hemispheres in 102 patients. Median follow-up was 4.3 years. Six patients (3.6% of hemispheres) suffered postoperative ischemic or hemorrhagic stroke. Overall mortality was 1.0%. Good postoperative neurological status (modified Rankin Scale [mRS] score ≤ 2) was observed in 90 patients (88%); preoperative and postoperative mRS scores showed significant improvement (p < 0.001). Eighty-six percent of hemispheres had Matsushima grade A or B revascularization. Younger age was associated with postoperative stroke and poor functional outcome. Patients with secondary moyamoya syndrome had a significantly worse radiographic outcome. The cumulative 5-year Kaplan-Meier risk for stroke was 6.4%.

CONCLUSIONS

Dural inversion is a useful technique of cerebral revascularization in pediatric moyamoya disease. A 20-year experience demonstrates the safety and efficacy of this technique with a relatively low rate of postoperative stroke, good functional outcomes, and favorable angiographic results.

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Virendra R. Desai, Jeffrey S. Raskin, Arvind Mohan, JoWinsyl Montojo, Valentina Briceño, Daniel J. Curry, and Sandi Lam

OBJECTIVE

Intrathecal baclofen pumps are generally placed in children for the treatment of spasticity and dystonia. Use of implants in this pediatric population with comorbidities is reported to have a high risk of complications and infections. With the aim of reducing baclofen pump–related infections, a quality improvement project was instituted at the authors’ institution.

METHODS

A workflow paradigm unique to baclofen pump implantation aimed at decreasing implant-related infections was implemented. All baclofen pump operations performed at the authors’ institution between August 2012 and June 2016 were reviewed. An infection prevention protocol was created and implemented in August 2014 based on a literature review and the consensus opinion of the pediatric neurosurgeons in the group. Compliance with the prevention bundle was tracked. Case outcomes before and after implementation of the protocol with a minimum of 3 months of follow-up were retrospectively reviewed. Univariate and multivariate analyses were performed to assess the association of the steps in the prevention bundle with infection and complication outcomes.

RESULTS

A total of 128 baclofen pump surgeries were performed (64 preprotocol and 64 postprotocol). The patient age range was 3 to 27 years. The overall compliance rate with the infection prevention bundle was 82%. The pre- and postimplementation infection rates were 12.5% and 6.3%, respectively (p = 0.225). The total pre- and postimplementation complication rates were 23.4% and 9.4%, respectively (p = 0.032). The absolute and relative risk reductions for infections were 6.3% (95% CI 3.8%–16.3%) and 50%, respectively; for complications, the absolute and relative risk reductions were 14.1% (95% CI 1.5%–26.7%) and 60%, respectively.

CONCLUSIONS

The total complication rate following intrathecal baclofen pump surgery was significantly lower after implementation of the quality improvement protocol. This study is an example of using checklist standardization to diminish special cause variability.

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Melissa LoPresti, Bradley Daniels, Edward P. Buchanan, Laura Monson, and Sandi Lam

Repeat surgery for restenosis after initial nonsyndromic craniosynostosis intervention is sometimes needed. Calvarial vault reconstruction through a healed surgical bed adds a level of intraoperative complexity and may benefit from preoperative and intraoperative definitions of biometric and aesthetic norms. Computer-assisted design and manufacturing using 3D imaging allows the precise formulation of operative plans in anticipation of surgical intervention. 3D printing turns virtual plans into anatomical replicas, templates, or customized implants by using a variety of materials. The authors present a technical note illustrating the use of this technology: a repeat calvarial vault reconstruction that was planned and executed using computer-assisted design and 3D printed intraoperative guides.

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Sandi Lam, I-Wen Pan, Ben A. Strickland, Caroline Hadley, Bradley Daniels, Jim Brookshier, and Thomas G. Luerssen

OBJECTIVE

Following institution of the Back to Sleep Campaign, the incidence of sudden infant death syndrome decreased while the prevalence of positional skull deformation increased dramatically. The management of positional deformity is controversial, and treatment recommendations and outcomes reporting are variable. The authors reviewed their institutional experience (2008–2014) with the treatment of positional plagiocephaly to explore factors associated with measured improvement.

METHODS

A retrospective chart review was conducted with risk factors and treatment for positional head shape deformity recorded. Univariate and multivariate analyses were used to assess the impact of these variables on the change in measured oblique diagonal difference (ODD) on head shape surface scanning pre- and posttreatment.

RESULTS

A total of 991 infants aged less than 1 year were evaluated for cranial positional deformity in a dedicated clinical program. The most common deformity was occipital plagiocephaly (69.5%), followed by occipital brachycephaly (18.4%) or a combination of both deformities (12.1%). Recommended treatment included repositioning (RP), physical therapy (PT) if indicated, or orthotic treatment with a customized cranial orthosis (CO) according to an age- and risk factor–dependent algorithm that the authors developed for this clinic. Of the 991 eligible patients, 884 returned for at least 1 follow-up appointment. A total of 552 patients were followed to completion of their treatment and had a full set of records for analysis: these patients had pre- and posttreatment 2D surface scanner evaluations. The average presenting age was 6.2 months (corrected for prematurity for treatment considerations). Of the 991 patients, 543 (54.8%) had RP or PT as first recommended treatment. Of these 543 patients, 137 (25.2%) transitioned to helmet therapy after the condition did not improve over 4–8 weeks. In the remaining cases, RP/PT had already failed before the patients were seen in this program, and the starting treatment recommendation was CO. At the end of treatment, the measured improvements in ODD were 36.7%, 33.5%, and 15.1% for patients receiving CO, RP/PT/CO, and RP/PT, respectively. Univariate analysis showed that sex, race, insurance, diagnosis, sleep position preference, torticollis history, and multiple gestation were not significantly associated with magnitude of ODD change during treatment. On multivariate analysis, corrected age at presentation and type of treatment received were significantly associated with magnitude of ODD change. Orthotic treatment corresponded with the largest ODD change, while the RP/PT group had the least change in ODD. Earlier age at presentation corresponded with larger ODD change.

CONCLUSIONS

Earlier age at presentation and type of treatment impact the degree of measured deformational head shape correction in positional plagiocephaly. This retrospective study suggests that treatment with a custom CO can result in more improvement in objective measurements of head shape.

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Sandi Lam, Thomas G. Luerssen, Caroline Hadley, Bradley Daniels, Ben A. Strickland, Jim Brookshier, and I-Wen Pan

OBJECTIVE

This study aimed to examine factors associated with adherence to recommended treatment among pediatric patients with positional skull deformity by reviewing a single-institution experience (2007–2014) with the treatment of positional plagiocephaly.

METHODS

A retrospective chart review was conducted. Risk factors, treatment for positional head shape deformity, and parent-reported adherence were recorded. Univariate and multivariate analyses were used to assess the impact of patient clinical and demographic characteristics on adherence.

RESULTS

A total of 991 patients under age 12 months were evaluated for positional skull deformity at the Texas Children's Hospital Cranial Deformity Clinic between 2007 and 2014. According to an age- and risk factor–based treatment algorithm, patients were recommended for repositioning, physical therapy, or cranial orthosis therapy or crossover from repositioning/physical therapy into cranial orthosis therapy. The patients' average chronological age at presentation was 6.2 months; 69.3% were male. The majority were white (40.7%) or Hispanic (32.6%); 38.7% had commercial insurance and 37.9% had Medicaid. The most common initial recommended treatment was repositioning or physical therapy; 85.7% of patients were adherent to the initial recommended treatment.

Univariate analysis showed differences in adherence rates among subgroups. Children's families with Medicaid were less likely to be adherent to treatment recommendations (adherence rate, 80.2%). Families with commercial insurance were more likely to be adherent to the recommended treatment (89.6%). Multivariate logistic regression confirmed that factors associated with parent-reported adherence to recommended treatment included primary insurance payer, diagnosis (plagiocephaly vs brachycephaly), and the nature of the recommended treatment. Families were less likely to be adherent if they had Medicaid, a child with a diagnosis of brachycephaly, or were initially recommended for cranial orthosis therapy than families with commercial insurance, a child with a diagnosis of plagiocephaly, or an initial recommendation for repositioning or physical therapy.

Factors associated with treatment completion included corrected age, insurance, diagnosis, recommended treatment, and distance to provider from patient's residence. Patients with commercial insurance (OR 1.49, 95% CI 1.10–2.02, p = 0.009), those diagnosed with both brachycephaly and plagiocephaly (OR 2.26, 95% CI 1.31–3.90, p = 0.003), those recommended for treatment with cranial orthosis (OR 4.55, 95% CI = 3.24–6.38, p < 0.001), and those living in proximity to the provider (OR 1.40, 95% CI 1.00–1.96, p = 0.047) were more likely to complete treatment.

CONCLUSIONS

Insurance type, degree of head shape deformity, and types of recommended treatment appear to affect rates of adherence to recommended treatments for positional skull deformation.

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Aditya Vedantam, Daniel Hansen, Bradley Daniels, and Sandi Lam

The authors report an unusual case of acute, rapidly progressive, unilateral neck swelling following extubation after elective left anterior temporal lobectomy with amygdalohippocampectomy. Due to severe neck swelling, the patient developed critical airway compromise, brachial plexopathy, and Horner's syndrome. After critical airway management and appropriate rehabilitation, the patient recovered completely and remains seizure free at 1.5 years of follow-up.

This case highlights the importance of early recognition of acute postoperative sialadenitis and the steps needed to prevent serious morbidity and possible mortality from this rare complication.

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Jacob Cherian, Kristen A. Staggers, I-Wen Pan, Melissa Lopresti, Andrew Jea, and Sandi Lam

OBJECTIVE

Due to improved nutrition and early detection, myelomeningocele repair is a relatively uncommon procedure. Although previous studies have reviewed surgical trends and predictors of outcomes, they have relied largely on single-hospital experiences or on databases centered on hospital admission data. Here, the authors report 30-day outcomes of pediatric patients undergoing postnatal myelomeningocele repair from a national prospective surgical outcomes database. They sought to investigate the association between preoperative and intraoperative factors on the occurrence of 30-day complications, readmissions, and unplanned return to operating room events.

METHODS

The 2013 American College of Surgeons National Surgical Quality Improvement Program Pediatric database (NSQIP-P) was queried for all patients undergoing postnatal myelomeningocele repair. Patients were subdivided on the basis of the size of the repair (< 5 cm vs > 5 cm). Preoperative variables, intraoperative characteristics, and postoperative 30-day events were tabulated from prospectively collected data. Three separate outcomes for complication, unplanned readmission, and return to the operating room were analyzed using univariate and multivariate logistic regression. Rates of associated CSF diversion operations and their timing were also analyzed.

RESULTS

A total of 114 patients were included; 54 had myelomeningocele repair for a defect size smaller than 5 cm, and 60 had repair for a defect size larger than 5 cm. CSF shunts were placed concurrently in 8% of the cases. There were 42 NSQIP-defined complications in 31 patients (27%); these included wound complications and infections, in addition to others. Postoperative wound complications were the most common and occurred in 27 patients (24%). Forty patients (35%) had at least one subsequent surgery within 30 days. Twenty-four patients (21%) returned to the operating room for initial shunt placement. Unplanned readmission occurred in 11% of cases. Both complication and return to operating room outcomes were statistically associated with age at repair.

CONCLUSIONS

The NSQIP-P allows examination of 30-day perioperative outcomes from a national prospectively collected database. In this cohort, over one-quarter of patients undergoing postnatal myelomeningocele repair experienced a complication within 30 days. The complication rate was significantly higher in patients who had surgical repair within the first 24 hours of birth than in patients who had surgery after the 1st day of life. The authors also highlight limitations of investigating myelomeningocele repair using NSQIP-P and advocate the importance of disease-specific data collection.

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Sandi Lam, I-Wen Pan, Andrew Jea, and Thomas G. Luerssen