A Comparison of Intracranial Volume Growth in Normal Children and Patients With Metopic Craniosynostosis.

BACKGROUND: The impact of metopic craniosynostosis on intracranial volume (ICV) and ICV growth is unclear. In addition, the relationship between head circumference (HC) and ICV in these patients is not previously described. METHODS: A retrospective review of 72 patients with metopic craniosynostosis was performed. The ICVs were calculated from manually segmented preoperative computed tomography scans. Magnetic resonance imaging data for 270 healthy children were available. The ICVs were calculated in FreeSurfer.First, a growth curve for metopic patients was generated and a logarithmic best-fit curve was calculated. Second, the impact of metopic craniosynostosis on ICV relative to healthy controls was assessed using multivariate linear regression. Third, the growth curves for metopic patients and healthy children were compared.Pearson's correlation was used to measure the association between HC and ICV. RESULTS: Mean metopic ICV was significantly lower than normal ICV within the first 3 to 6 months (674.9 versus 813.2 cm; P = 0.002), 6 to 9 months (646.6 versus 903.9 cm; P = 0.005), and 9 to 12 months of life (848.0 versus 956.6 cm; P = 0.038). There was no difference in ICV after 12 months of age (P = 0.916).The ICV growth in patients with metopic craniosynostosis is defined by a significantly different growth curve than in normal children (P = 0.005).The ICV and HC were highly correlated across a broad range of ICVs and patient age (r = 0.98, P < 0.001). CONCLUSION: Patients with metopic craniosynostosis have significantly reduced ICVs compared to healthy children, yet greater than normal ICV growth, which allows them to achieve normal volumes by 1 year of age. The HC is a reliable metric for ICV in these patients.

A Study to Evaluate Change in Ventricular Volume Obtained by Cranial Distraction for Craniosynostosis.

BACKGROUND: The aims of the current analysis were to study the change in ventricular volume (VV) obtained with cranial distraction in patients with craniosynostosis and compare it with the change in total intracranial volume (ICV) and brain volume. METHODS: After institutional review board approval, a retrospective review was performed on patients undergoing cranial distraction over a 5-year period. GE Healthcare AdW 4.3 volume assessment software was utilized to calculate preoperative and postdistraction ICV, VV, and whole-brain volume. Data were also collected on patient demographics, age at the time of distraction, time spent in distraction and consolidation, and length of stay. t Tests were used for comparison. RESULTS: Twenty-three patients met our inclusion criteria. Forty-eight percent of patients (n = 11) had right-sided cranial distraction, 30% (n = 7) had bilateral distraction, and 22% of patients (n = 5) had left-sided distraction. At the preoperative stage, mean head circumference was 42.5 ± 4.7 cm, mean ICV was 810.1 ± 27 cm, mean non-VV (NVV) was 796.2 ± 268 cm, and mean VV was 13.9 ± 9 cm. After a mean of 27.4 mm of distraction, occurring over a mean of 26 days and consolidation period of 149 days, a second computed tomography scan was obtained. Mean postdistraction head circumference was 49.1 ± 3.9 cm, mean ICV was 1074.1 ± 203 cm, mean NVV was 1053.5 ± 197 cm, and VV was 20.6 ± 14 cm. Mean % increase in ICV at this stage was 47.4%; mean % NVV increase was 48.5% as opposed to 60.3% increase in VV. CONCLUSIONS: Cranial distraction is known to effectively increase ICV. Our study suggests that the effect of this volumetric increase is much more pronounced on the VV compared with the brain volume. Further studies are underway to investigate whether this short-term marked increase in VV is sustained over a long-term period.

Can Head Circumference Be Used as a Proxy for Intracranial Volume in Patients With Craniosynostosis?

BACKGROUND: Calculation of intracranial volume from neuroimaging can be complex and time consuming. In the adult population, there is evidence suggesting that owing to its strong correlation, head circumference (HC) may be used as a surrogate for intracranial volume (ICV). We were interested in studying the correlation between HC and ICV in patients with craniosynostosis. METHODS: After institutional review board approval, a retrospective review was performed on patients with craniosynostosis. GE Healthcare AdW 4.3 volume assessment software was used to calculate ICV and HC based on preoperative computed tomographic scans. Pearson correlation was used to estimate correlation coefficients between ICV and HC for this patient population, with 0 to 0.3 considered a weak correlation, 0.4 to 0.6 considered a moderate correlation, 0.7 to 1 considered a strong correlation, and P < 0.05 was considered statistically significant. RESULTS: A total of 196 craniosynostosis patients were included in this study. There were 121 male and 75 female patients. Seventy-nine patients had metopic, 45 had coronal, 64 had sagittal, and 8 had lambdoid synostosis. Mean age was 8.2 months. Mean HC and ICV were 42.9 cm and 829 cm, respectively. Overall, there was a strong correlation between HC and ICV (r = 0.81). Patients were further categorized by craniosynostosis type. Very strong correlation was obtained for patients with coronal (0.89), metopic (0.98), and lambdoid craniosynostosis (0.97). Strong correlation was obtained for patients with sagittal synostosis (0.73). When categorized by sex, a stronger correlation was obtained for female patients (0.84) compared with male patients (0.80). Statistical significance was reached for all reported correlations. CONCLUSION: Our preliminary data suggest that a very strong correlation exists between HC and ICV for male and female patients with all types of craniosynostosis, making HC a useful surrogate for ICV in this patient population.

Predictors of Postoperative Complications of Craniosynostosis Repair in the National Inpatient Sample.

INTRODUCTION: Timing of intervention and complication profiles in surgical repair of craniosynostosis have been widely debated. Early intervention is frequently promoted as a means of decreasing morbidity while maintaining favorable outcomes via minimally invasive techniques such as endoscopic strip craniectomy. Immediate postoperative morbidity due to complications of early vs late intervention remains a key element in comparing timing and technique for craniosynostosis repair. In addition, concurrent fronto-orbital advancement with open cranial vault remodeling may increase the risk of postoperative complications. We present an evaluation of surgical timing and the presence of fronto-orbital advancement as independent predictors of in-hospital complications after craniosynostosis repair. METHODS: Retrospective analysis was performed in the National Inpatient Sample database from 1998 to 2009. Patients younger than 3 years having undergone elective surgical repair for craniosynostosis were identified. Comorbidities, demographics, transfusion status, and syndromic diagnosis were included as covariates. A multivariate regression model was used to characterize the association between age at the time of surgery and in-hospital complications. A subgroup analysis using the variable of concurrent fronto-orbital advancement was restricted to patients 8 to 24 months of age to exclude endoscopic craniosynostosis repair, which is traditionally repaired less than 6 months of age. Multivariate logistic regression was used to assess the impact of concurrent fronto-orbital advancement on postoperative complications. RESULTS: A total of 6010 craniosynostosis surgery cases (42.9%, age 0-7 months; 29.5%, age 8-12 months; and 27.6%, age 13-36 months) were included. Patients in the 7-to 12-month age group were more likely to experience complications when compared with the 0- to 6-month age group (odds ratio [OR],1.32; P < 0.05) and 13-to 36-month age group (OR, 1.32; P = 0.056). Syndromic patients (OR, 1.92; P < 0.001) and patients receiving an intraoperative blood transfusion (OR, 1.60; P < 0.05) demonstrated an increased risk for complications. In the subanalysis of 2936 patients aged 8 to 24 months, 15.1% of patients received frontoorbital advancement, which was associated with a significantly increased risk of complications (OR, 1.43; P < 0.05). CONCLUSIONS: Intermediate age (7-12 months) and concurrent fronto-orbital repair were independent risk factors for immediate postoperative complications. These findings may better inform the decision-making process for craniosynostosis repair in terms of timing and need for concurrent fronto-orbital reconstruction.

A Novel Method for Quantifying Intracranial Volume Change by Distraction Osteogenesis for Craniosynostosis.

INTRODUCTION: Methods of reporting quantitative results for distraction osteogenesis (DO) of craniosynostosis have been inconsistent. Therefore, the efficacy of differing techniques and timing in regard to volume change is not well established, with no uniform metric for comparisons. Given that cranial vault remodeling with DO may be completed with different approaches, analysis was made to determine (1) the relative efficiency of different approaches in expanding intracranial volume (ICV) and (2) the impact of adjusting for ICV growth on measured DO efficiency. METHODS: Patients with craniosynostosis were treated with open cranial vault reconstruction combined with internal distraction. Preoperative and postoperative computed tomography scans were used to quantify ICV change. The metric was determined by dividing percent ICV change by total distraction length. The metric was used as a proxy for efficiency to compare posterior and anterior distraction between groups using the Mann-Whitney U test and within a subgroup of patients who underwent 2-stage distraction using the Wilcoxon matched-pairs signed rank test. RESULTS: Twenty patients underwent cranial vault remodeling with DO: 14 unicoronal, 3 bicoronal, 2 multisutural, and 1 lambdoid. Results are reported in medians. Distraction efficiency was 0.99%/mm for primary anterior, unilateral distraction for unicoronal patients (n = 13, aged 9.1 months) and 4.28%/mm for posterior distraction using multiple distractors (n = 4, aged 6.3 months). In terms of the metric, primary posterior distraction was significantly more efficient than primary anterior distraction (P = 0.007). Three patients who had undergone primary posterior distraction later underwent secondary anterior distraction. Again, posterior distraction was shown to be significantly more efficient (5.16 vs 0.62, P = 0.050). For the unicoronal patients who received anterior unilateral distraction, an adjusted metric was calculated to account for normal intracranial growth. This was found to be 0.39%/mm, which was significantly different from the unadjusted metric (P = 0.0001). CONCLUSIONS: Posterior distraction is more efficient for ICV expansion than anterior distraction, which may have implications for the choice of approach for craniosynostosis repair. In addition, this is the first report of a novel standardized metric for analyzing ICV change achieved by DO. This tool allows for adjusting the efficiency metric for expected ICV growth, which significantly impacts its value.

Intracranial Hypotension and Hypertension Associated With Reconstructive Cranioplasty After Decompressive Craniectomy: Report of a Lethal Complication With Recommended Strategies for Future Avoidance.

Reconstructive cranioplasty can be associated with many complications and add to the not insignificant potential risks associated with decompressive craniectomy. In the setting of post-traumatic hydrocephalus, treatment with a ventriculoperitoneal (VP) shunt prior to reconstructive cranioplasty likely increases these risks even further. The authors report a case of a 17-year-old male with a history of a severe closed head injury who initially suffered a life-threatening complication associated with intracranial hypotension after cranioplasty only to succumb to malignant intracranial hypertension following a second cranioplasty attempt. To our knowledge, this is the first description of a single patient developing both these disparate complications after reconstructive cranioplasty and emphasizes the likely synergistic hazards involved with decompressive craniectomy in the setting of a VP shunt in particular and the overall myriad potential complications that may be associated with reconstructive cranioplasty in general.

Subgaleal Drain Placement Improves Surgical Outcomes After Primary Cranioplasty in Craniosynostosis Patients.

BACKGROUND: There is no published data addressing the use of postoperative subgaleal drains in patients undergoing primary cranioplasty for craniosynostosis. We conducted a retrospective chart review in this population of patients, comparing outcomes of those who received postoperative drains with those who did not. We hypothesize that the subgaleal drains can significantly diminish postoperative facial edema and reduce the length of hospital stay. METHODS: We conducted a retrospective chart review of all patients undergoing primary cranioplasty for craniosynostosis with subgaleal drain placement (May 2010-March 2012). A comparison group without drain placement was matched appropriately to establish a comparison of outcomes. We determined whether subgaleal drainage led to improvement in postoperative facial edema, reduced length of hospital stay, postoperative changes in hematocrit (Hct), and complication rates. RESULTS: Of the 50 patients in this cohort, 25 patients had received subgaleal drains. The mean length of stay was 2.4 versus 3.5 days for the respective drained and undrained cohorts (P = 0.03). There was no significant difference in the mean decline in Hct between drained and undrained patients, with the mean Hct drop of 4.8% versus 5.0%, respectively (P = 0.83). Postoperative seroma formation developed in 3 undrained patients (17%) versus none in the drained cohort (0%). Although subjective, drained patients were observed to achieve quicker resolution of facial swelling and earlier recovery of eye opening. CONCLUSIONS: There is clinical benefit in subgaleal drain placement as earlier resolution of postoperative facial edema and a significantly shortened length of hospital stay was found among the drained cohort. Future studies warrant prospective clinical trials to establish the safety and efficacy of using subgaleal drains in cranial remodeling procedures of craniosynostosis.

Risk of meningomyelocele mediated by the common 22q11.2 deletion.

Meningomyelocele is one of the most severe forms of neural tube defects (NTDs) and the most frequent structural birth defect of the central nervous system. We assembled the Spina Bifida Sequencing Consortium to identify causes. Exome and genome sequencing of 715 parent-offspring trios identified six patients with chromosomal 22q11.2 deletions, suggesting a 23-fold increased risk compared with the general population. Furthermore, analysis of a separate 22q11.2 deletion cohort suggested a 12- to 15-fold increased NTD risk of meningomyelocele. The loss of Crkl, one of several neural tube-expressed genes within the minimal deletion interval, was sufficient to replicate NTDs in mice, where both penetrance and expressivity were exacerbated by maternal folate deficiency. Thus, the common 22q11.2 deletion confers substantial meningomyelocele risk, which is partially alleviated by folate supplementation.

Complications associated with prolonged hypertonic saline therapy in children with elevated intracranial pressure.

OBJECTIVES: Safe upper limits for therapeutic hypernatremia in the treatment of intracranial hypertension have not been well established. We investigated complications associated with hypernatremia in children who were treated with prolonged infusions of hypertonic saline. DESIGN: Retrospective chart analysis. SETTING: PICU in university-affiliated children's hospital. PATIENTS: All children from 2004 to 2009 requiring intracranial pressure monitoring (external ventricular drain or fiberoptic intraparenchymal monitor) for at least 4 days who were treated with hypertonic saline infusion for elevated intracranial pressure and did not meet exclusion criteria. INTERVENTION: Continuous hypertonic saline infusion on a sliding scale was used to achieve target sodium levels that would keep intracranial pressure less than 20 mm Hg once the conventional therapies failed. MEASUREMENTS AND MAIN RESULTS: Eighty-eight children met inclusion criteria. Etiologies of elevated intracranial pressure included trauma (n = 48), ischemic or hemorrhagic stroke (n = 20), infection (n = 8), acute disseminated encephalomyelitis (n = 5), neoplasm (n = 2), and others (n = 5). The mean peak serum sodium was 171.3 mEq/L (range, 150-202). The mean Glasgow Outcome Score was 2.8 (± 1.1) at time of discharge from the hospital. Overall mortality was 15.9%. Children with sustained (> 72 hr) serum sodium levels above 170 mEq/L had a significantly higher occurrence of thrombocytopenia (p < 0.001), renal failure (p < 0.001), neutropenia (p = 0.006), and acute respiratory distress syndrome (p = 0.029) after controlling for variables of age, gender, Pediatric Risk of Mortality score, duration of barbiturate-induced coma, duration of intracranial pressure monitoring, vasopressor requirements, and underlying pathology. Children with sustained serum sodium levels greater than 165 mEq/L had a significantly higher prevalence of anemia (p < 0.001). CONCLUSIONS: Children treated by continuous hypertonic saline infusion for intracranial hypertension whose serum sodium levels exceeded certain thresholds experienced significantly more events of acute renal failure, thrombocytopenia, neutropenia, anemia, and acute respiratory distress syndrome than those whose sodium level was maintained below these thresholds.

Craniofacial reconstruction as a treatment for elevated intracranial pressure.

INTRODUCTION: Craniofacial procedures may be needed to address symptomatic intracranial hypertension. The authors review their institutional experience in the treatment of children with symptomatic increased intracranial pressure (ICP) utilizing craniofacial reconstructive procedures. METHODS: The senior authors' (HSM, SRC) craniofacial experience of 222 patients over a 7-year period from 2000 to 2007 at a single institution (Rady Children's Hospital, San Diego) is reviewed. Seventeen patients were identified who were felt to be candidates for craniofacial surgery with symptomatic increased ICP. RESULTS: Patient diagnoses included single-suture craniosynostosis, craniofacial dysostoses, shunt-induced craniostenosis, and shunt-associated intracranial hypertension (slit-ventricle syndrome). Seventeen patients underwent 21 craniofacial procedures. Age at surgery ranged from 3 months to 13 years with a mean of 5 years. Preoperative symptoms and signs included headaches, unexplained irritability, seizures, papilledema, and visual loss. All patients had diagnostic neuroimaging. Seven patients had preoperative invasive ICP measurements. Surgery was deferred on three of these patients based on these measurements. The mean total operative (including anesthetic preparation) and surgical times were 3 h 12 min and 2 h 20 min, respectively. Percentage operative blood loss averaged 11.3%. In six procedures, no transfusions were required. Average hospital stay was 4 days. There was no perioperative mortality or significant surgery associated morbidity. All patients have had postoperative clinical improvement in signs and symptoms of increased ICP. CONCLUSIONS: Using modern diagnostic and surgical techniques, including invasive ICP monitoring, increased intracranial pressure can be successfully managed by an experienced, multidisciplinary, craniofacial team. Our treatment paradigm and operative management scheme is discussed.

Nonsyndromic craniosynostosis: the Rady Children's Hospital approach.

ABSTRACT: Surgical correction for nonsyndromic craniosynostosis has continued to evolve over the last century. The criterion standard has remained open correction of the cranial deformities, and many techniques have been described that yield satisfactory results. However, technology has allowed for minimally invasive techniques to be developed with the aid of endoscopic visualization. With proper patient selection and the aid of postoperative helmet therapy, there is increasing evidence that supports these techniques' safety and efficacy. In this article, our purpose was to describe our algorithm for treating nonsyndromic craniosynostosis at Rady Children's Hospital.

In situ fronto-orbital advancement with medial orbital osteotomies for trigonocephaly-associated hypotelorism.

In treating trigonocephaly, the value of direct surgical correction of orbital hypotelorism is controversial. In many cases of hypotelorism, the distance between the orbits increases over time after traditional fronto-orbital advancement. Still, more severe hypotelorism is not fully corrected and may benefit from a more definitive surgical intervention. We describe an in situ fronto-orbital advancement that improves severe hypotelorism and simplifies the surgical treatment of trigonocephaly. The key modification to traditional fronto-orbital advancement is an in situ medial orbital osteotomy that extends along the medial orbit, posterior to the medial canthus, and then across the inferior orbital rim into the piriform aperture. The procedure is indicated only in patients with more severe hypotelorism on physical examination. Rather than creating a freely removable bandeau during the operation, the bone segment composed of the supraorbital bar and superior orbits remains attached at the medial canthi. A midline osteotomy allows the respective orbital segments to be independently mobilized with the medial canthi left attached, and the space between them widened with gentle lateral traction and placement of an interpositional bone graft. Concomitantly, the lateral orbits and lateral supraorbital bar are contoured, advanced, and fixed with resorbable plates and screws. Representative results are shown. In situ fronto-orbital advancement with medial orbital osteotomies is a safe, efficient, and relatively simple technique that results in immediate improvement of hypotelorism and may be a worthwhile maneuver to consider in selected cases.

The "SHIFT" technique: Suprahepatic IntraFalciform tubing for placement of ventriculoperitoneal shunts.

Ventriculoperitoneal (VP) shunts in pediatric patients are an important aspect of management for patients with hydrocephalus and are fraught with complications. Surgical revision rates for VP shunts in the pediatric population are currently high, which necessitates innovation in operative techniques for placing VP shunts in attempt to decrease complication risks. Here we describe a novel approach for placement of VP shunts that we hypothesize can reduce potential morbidity among pediatric patients. By utilizing the falciform ligament of the liver and the suprahepatic recess to suspend and maintain the shunt, outcomes may portend fewer iatrogenic intra-abdominal injuries, enhanced ease of shunt removal, provide a large surface area for absorption of drained cerebrospinal fluid, and result in fewer adhesions secondary to device placement. We are referring to the operative technique as the "SupraHepatic IntraFalciform Tubing" (SHIFT) technique. In summary, the SHIFT shunt is fashioned by creating a window through the falciform ligament, inserting the shunt, and placing tubing in the suprahepatic recess.

Variations of endoscopic and open repair of metopic craniosynostosis.

In contrast to sagittal craniosynostosis, the role of endoscopic, minimally invasive approaches in the treatment of metopic craniosynostosis with resulting trigonocephaly is not as well defined. We reviewed the senior authors' (H.M. and S.C.) clinical experience in the treatment of children with metopic craniosynostosis using a variety of endoscopic and open techniques. Thirty-three patients were treated at a single institution during a 5-year period with between 3 and 8 years of follow-up. Sixteen patients underwent 3 variations of endoscopic approaches, and 17 patients had open fronto-orbital advancement. Clinical parameters of the 2 groups were examined including age at surgery, blood loss, operative time, transfusion volume, hospital stay, complications, use of postoperative cranial banding, and the need for reoperation for persistent deformity. The various endoscopic and open techniques used by the authors in the treatment of metopic craniosynostosis are discussed in detail, including rational for individual technique selection and preliminary impressions regarding clinical outcome.

Surgical relevance of pediatric skull base maturation for the far-lateral and extreme-lateral infrajugular transcondylar-transtubercular exposure approaches.

OBJECTIVE: Lesions of the foramen magnum, inferolateral-to-midclival areas, and ventral pons and medulla are often treated using a far-lateral or extreme-lateral infrajugular transcondylar-transtubercular exposure (ELITE) approach. The development and surgical relevance of critical posterior skull base bony structures encountered during these approaches, including the occipital condyle (OC), hypoglossal canal (HGC), and jugular tubercle (JT), are nonetheless poorly defined in the pediatric population. METHODS: Measurements from high-resolution CT scans were made of the relevant posterior skull base anatomy (HGC depth from posterior edge of the OC, OC and JT dimensions) from 60 patients (evenly distributed among ages 0-3, 4-7, 8-11, 12-15, 16-18, and > 18 years), and compared between laterality, sex, and age groups by using t-tests and linear regression. RESULTS: There were no significant differences in posterior skull base parameters by laterality, and HGC depth and JT size did not differ by sex. The OC area was significantly larger in males versus females (174.3 vs 152.2 mm2; p = 0.01). From ages 0-3 years to adult, the mean HGC depth increased 27% (from 9.0 to 11.4 mm) and the OC area increased 52% (from 121.4 to 184.0 mm2). The majority of growth for these parameters occurred between the 0-3 year and 4-7 year age groups. Conversely, JT volume increased nearly 3-fold (281%) from 97.4 to 370.9 mm3 from ages 0-3 years to adult, with two periods of substantial growth seen between the 0-3 to 4-7 year and the 12-15 to 16-18 year age groups. Overall, JT growth during pediatric development was significantly greater than increases in HGC depth and OC area (p < 0.05). JT volume remained < 65% of adult size up to age 16. CONCLUSIONS: When considering a far-lateral or ELITE approach in pediatric patients, standard OC drilling is likely to be needed due to the relative stability of OC and HGC anatomy during development. The JT significantly increases in size with development, yet is only likely to need to be drilled in older children (> 16 years) and adults.

Endoscopic surgery for nonsyndromic craniosynostosis: a 16-year single-center experience.

OBJECTIVE: In this paper the authors review their 16-year single-institution consecutive patient experience in the endoscopic treatment of nonsyndromic craniosynostosis with an emphasis on careful review of any associated treatment-related complications and methods of complication avoidance, including preoperative planning, intraoperative management, and postoperative care and follow-up. METHODS: A retrospective chart review was conducted on all patients undergoing endoscopic, minimally invasive surgery for nonsyndromic craniosynostosis at Rady Children's Hospital from 2000 to 2015. All patients were operated on by a single neurosurgeon in collaboration with two plastic and reconstructive surgeons as part of the institution's craniofacial team. RESULTS: Two hundred thirty-five patients underwent minimally invasive endoscopic surgery for nonsyndromic craniosynostosis from 2000 to 2015. The median age at surgery was 3.8 months. The median operative and anesthesia times were 55 and 105 minutes, respectively. The median estimated blood loss (EBL) was 25 ml (median percentage EBL 4.2%). There were no identified episodes of air embolism or operative deaths. One patient suffered an intraoperative sagittal sinus injury, 2 patients underwent intraoperative conversion of planned endoscopic to open procedures, 1 patient experienced a dural tear, and 1 patient had an immediate reexploration for a developing subgaleal hematoma. Two hundred twenty-five patients (96%) were admitted directly to the standard surgical ward where the median length of stay was 1 day. Eight patients were admitted to the intensive care unit (ICU) postoperatively, 7 of whom had preexisting medical conditions that the team had identified preoperatively as necessitating a planned ICU admission. The 30-day readmission rate was 1.7% (4 patients), only 1 of whom had a diagnosis (surgical site infection) related to their initial admission. Average length of follow-up was 2.8 years (range < 1 year to 13.4 years). Six children (< 3%) had subsequent open procedures for perceived suboptimal aesthetic results, 4 of whom (> 66%) had either coronal or metopic craniosynostosis. No patient in this series either presented with or subsequently developed signs or symptoms of intracranial hypertension. CONCLUSIONS: In this large single-center consecutive patient series in the endoscopic treatment of nonsyndromic craniosynostosis, significant complications were avoided, allowing for postoperative care for the vast majority of infants on a standard surgical ward. No deaths, catastrophic postoperative morbidity, or evidence of the development of symptomatic intracranial hypertension was observed.

The identification of a subgroup of children with traumatic subarachnoid hemorrhage at low risk of neuroworsening.

OBJECTIVEPediatric traumatic subarachnoid hemorrhage (tSAH) often results in intensive care unit (ICU) admission, the performance of additional diagnostic studies, and ICU-level therapeutic interventions to identify and prevent episodes of neuroworsening.METHODSData prospectively collected in an institutionally specific trauma registry between 2006 and 2015 were supplemented with a retrospective chart review of children admitted with isolated traumatic subarachnoid hemorrhage (tSAH) and an admission Glasgow Coma Scale (GCS) score of 13-15. Risk of blunt cerebrovascular injury (BCVI) was calculated using the BCVI clinical prediction score.RESULTSThree hundred seventeen of 10,395 pediatric trauma patients were admitted with tSAH. Of the 317 patients with tSAH, 51 children (16%, 23 female, 28 male) were identified with isolated tSAH without midline shift on neuroimaging and a GCS score of 13-15 at presentation. The median patient age was 4 years (range 18 days to 15 years). Seven had modified Fisher grade 3 tSAH; the remainder had grade 1 tSAH. Twenty-six patients (51%) had associated skull fractures; 4 involved the petrous temporal bone and 1 the carotid canal. Thirty-nine (76.5%) were admitted to the ICU and 12 (23.5%) to the surgical ward. Four had an elevated BCVI score. Eight underwent CT angiography; no vascular injuries were identified. Nine patients received an imaging-associated general anesthetic. Five received hypertonic saline in the ICU. Patients with a modified Fisher grade 1 tSAH had a significantly shorter ICU stay as compared to modified Fisher grade 3 tSAH (1.1 vs 2.5 days, p = 0.029). Neuroworsening was not observed in any child.CONCLUSIONSChildren with isolated tSAH without midline shift and a GCS score of 13-15 at presentation appear to have minimal risk of neuroworsening despite the findings in some children of skull fractures, elevated modified Fisher grade, and elevated BCVI score. In this subgroup of children with tSAH, routine ICU-level care and additional diagnostic imaging may not be necessary for all patients. Children with modified Fisher grade 1 tSAH may be particularly unlikely to require ICU-level admission. Benefits to identifying a subgroup of children at low risk of neuroworsening include improvement in healthcare efficiency as well as decreased utilization of unnecessary and potentially morbid interventions, including exposure to ionizing radiation and general anesthesia.

Extended middle fossa approach to lateralized pontine cavernomas in children.

OBJECTIVE Treatment of hemorrhagic cavernous malformations within the lateral pontine region demands meticulous surgical planning and execution to maximize resection while minimizing morbidity. The authors report a single institution's experience using the extended middle fossa rhomboid approach for the safe resection of hemorrhagic cavernomas involving the lateral pons. METHODS A retrospective chart review was performed to identify and review the surgical outcomes of patients who underwent an extended middle fossa rhomboid approach for the resection of hemorrhagic cavernomas involving the lateral pons during a 10-year period at Rady Children's Hospital of San Diego. Surgical landmarks for this extradural approach were based on the Fukushima dual-fan model, which defines the rhomboid based on the following anatomical structures: 1) the junction of the greater superficial petrosal nerve (GSPN) and mandibular branch of the trigeminal nerve; 2) the lateral edge of the porus trigeminus; 3) the intersection of the petrous ridge and arcuate eminence; and 4) the intersection of the GSPN, geniculate ganglion, and arcuate eminence. The boundaries of maximal bony removal for this approach are the clivus inferiorly below the inferior petrosal sinus; unroofing of the internal auditory canal posteriorly; skeletonizing the geniculate ganglion, GSPN, and internal carotid artery laterally; and drilling under the Gasserian ganglion anteriorly. This extradural petrosectomy allowed for an approach to all lesions from an area posterolateral to the basilar artery near its junction with cranial nerve (CN) VI, superior to the anterior inferior cerebellar artery and lateral to the origin of CN V. Retraction of the mandibular branch of the trigeminal nerve during this approach allowed avoidance of the region involving CN IV and the superior cerebellar artery. RESULTS Eight pediatric patients (4 girls and 4 boys, mean age of 13.2 ± 4.6 years) with hemorrhagic cavernomas involving the lateral pons and extension to the pial surface were treated using the surgical approach described above. Seven cavernomas were completely resected. In the eighth patient, a second peripheral lesion was not resected with the primary lesion. One patient had a transient CN VI palsy, and 2 patients had transient trigeminal hypesthesia/dysesthesia. One patient experienced a CSF leak that was successfully treated by oversewing the wound. CONCLUSIONS The extended middle fossa approach can be used for resection of lateral pontine hemorrhagic cavernomas with minimal morbidity in the pediatric population.

The pathophysiologic mechanism of cerebellar mutism.

OBJECTIVE: Cerebellar mutism (CM) is a postoperative complication of mainly pediatric posterior fossa surgery. Multiple theories exist for explaining this phenomenon. We have made an attempt to further understand this entity given a particularly interesting case as it relates to multiple pathophysiologic pathways. METHODS: We have reviewed the details surrounding a particularly interesting case of CM. A retrospective analysis of this patient's clinical history and recovery is described. An extensive literature review has been performed in conjunction with an attempt to help elucidate details and a better understanding of CM. RESULTS: A thorough analysis of existing theories as to the pathophysiologic mechanism of CM has been performed as it relates to the details of this particular case. A case is described in which a child exhibiting CM abruptly improved and made a relatively quick recovery after the triggering of the melodic speech pathway by way of watching and beginning to sing along with a video. It appears that this incident involving a familiar song catalyzed various speech pathways, which apparently were in some state of shock. This phenomenon seems to be a temporary entity involving not only the mechanical coordination of speech production, but also the initiation of speech itself. CONCLUSIONS: Evidence exists for a pathophysiologic pathway for speech by way of coordinating phonation and articulation. In addition, there seems to exist a pathway by which the initiation of speech may be altered or halted by posterior fossa pathology, namely, vermian or dentate nuclear injury. In particular to this case, we found that the incidental appreciation of other forms of speech, melodic in this instance, may be the key to help stimulate and accelerate the recovery from CM.