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Jacob K. Greenberg, Donna B. Jeffe, Christopher R. Carpenter, Yan Yan, Jose A. Pineda, Angela Lumba-Brown, Martin S. Keller, Daniel Berger, Robert J. Bollo, Vijay M. Ravindra, Robert P. Naftel, Michael C. Dewan, Manish N. Shah, Erin C. Burns, Brent R. O’Neill, Todd C. Hankinson, William E. Whitehead, P. David Adelson, Mandeep S. Tamber, Patrick J. McDonald, Edward S. Ahn, William Titsworth, Alina N. West, Ross C. Brownson, and David D. Limbrick Jr.

OBJECTIVE

There remains uncertainty regarding the appropriate level of care and need for repeating neuroimaging among children with mild traumatic brain injury (mTBI) complicated by intracranial injury (ICI). This study’s objective was to investigate physician practice patterns and decision-making processes for these patients in order to identify knowledge gaps and highlight avenues for future investigation.

METHODS

The authors surveyed residents, fellows, and attending physicians from the following pediatric specialties: emergency medicine; general surgery; neurosurgery; and critical care. Participants came from 10 institutions in the United States and an email list maintained by the Canadian Neurosurgical Society. The survey asked respondents to indicate management preferences for and experiences with children with mTBI complicated by ICI, focusing on an exemplar clinical vignette of a 7-year-old girl with a Glasgow Coma Scale score of 15 and a 5-mm subdural hematoma without midline shift after a fall down stairs.

RESULTS

The response rate was 52% (n = 536). Overall, 326 (61%) respondents indicated they would recommend ICU admission for the child in the vignette. However, only 62 (12%) agreed/strongly agreed that this child was at high risk of neurological decline. Half of respondents (45%; n = 243) indicated they would order a planned follow-up CT (29%; n = 155) or MRI scan (19%; n = 102), though only 64 (12%) agreed/strongly agreed that repeat neuroimaging would influence their management. Common factors that increased the likelihood of ICU admission included presence of a focal neurological deficit (95%; n = 508 endorsed), midline shift (90%; n = 480) or an epidural hematoma (88%; n = 471). However, 42% (n = 225) indicated they would admit all children with mTBI and ICI to the ICU. Notably, 27% (n = 143) of respondents indicated they had seen one or more children with mTBI and intracranial hemorrhage demonstrate a rapid neurological decline when admitted to a general ward in the last year, and 13% (n = 71) had witnessed this outcome at least twice in the past year.

CONCLUSIONS

Many physicians endorse ICU admission and repeat neuroimaging for pediatric mTBI with ICI, despite uncertainty regarding the clinical utility of those decisions. These results, combined with evidence that existing practice may provide insufficient monitoring to some high-risk children, emphasize the need for validated decision tools to aid the management of these patients.

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Nikita G. Alexiades, Edward S. Ahn, Jeffrey P. Blount, Douglas L. Brockmeyer, Samuel R. Browd, Gerald A. Grant, Gregory G. Heuer, Todd C. Hankinson, Bermans J. Iskandar, Andrew Jea, Mark D. Krieger, Jeffrey R. Leonard, David D. Limbrick Jr., Cormac O. Maher, Mark R. Proctor, David I. Sandberg, John C. Wellons III, Belinda Shao, Neil A. Feldstein, and Richard C. E. Anderson

OBJECTIVE

Complications after complex tethered spinal cord (cTSC) surgery include infections and cerebrospinal fluid (CSF) leaks. With little empirical evidence to guide management, there is variability in the interventions undertaken to limit complications. Expert-based best practices may improve the care of patients undergoing cTSC surgery. Here, authors conducted a study to identify consensus-driven best practices.

METHODS

The Delphi method was employed to identify consensual best practices. A literature review regarding cTSC surgery together with a survey of current practices was distributed to 17 board-certified pediatric neurosurgeons. Thirty statements were then formulated and distributed to the group. Results of the second survey were discussed during an in-person meeting leading to further consensus, which was defined as ≥ 80% agreement on a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree).

RESULTS

Seventeen consensus-driven best practices were identified, with all participants willing to incorporate them into their practice. There were four preoperative interventions: (1, 2) asymptomatic AND symptomatic patients should be referred to urology preoperatively, (3, 4) routine preoperative urine cultures are not necessary for asymptomatic AND symptomatic patients. There were nine intraoperative interventions: (5) patients should receive perioperative cefazolin or an equivalent alternative in the event of allergy, (6) chlorhexidine-based skin preparation is the preferred regimen, (7) saline irrigation should be used intermittently throughout the case, (8) antibiotic-containing irrigation should be used following dural closure, (9) a nonlocking running suture technique should be used for dural closure, (10) dural graft overlay should be used when unable to obtain primary dural closure, (11) an expansile dural graft should be incorporated in cases of lipomyelomeningocele in which primary dural closure does not permit free flow of CSF, (12) paraxial muscles should be closed as a layer separate from the fascia, (13) routine placement of postoperative drains is not necessary. There were three postoperative interventions: (14) postoperative antibiotics are an option and, if given, should be discontinued within 24 hours; (15) patients should remain flat for at least 24 hours postoperatively; (16) routine use of abdominal binders or other compressive devices postoperatively is not necessary. One intervention was prioritized for additional study: (17) further study of additional gram-negative perioperative coverage is needed.

CONCLUSIONS

A modified Delphi technique was used to develop consensus-driven best practices for decreasing wound complications after cTSC surgery. Further study is required to determine if implementation of these practices will lead to reduced complications. Discussion through the course of this study resulted in the initiation of a multicenter study of gram-negative surgical site infections in cTSC surgery.

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Rajiv R. Iyer, Xiaobu Ye, Qiuyu Jin, Yao Lu, Luckmini Liyanage, and Edward S. Ahn

OBJECTIVE

Many infants with sagittal craniosynostosis undergo effective surgical correction with endoscopic strip craniectomy (ESC) and postoperative helmet therapy (PHT). While PHT is essential to achieving optimal cosmesis following ESC, there has been little comprehensive analysis of the ideal PHT duration needed to attain this goal.

METHODS

The authors retrospectively reviewed the charts of infants undergoing ESC and PHT for sagittal synostosis at our institution between 2008 and 2015. Data collected included age at surgery, follow-up duration, and PHT duration. Cephalic index (CI) was evaluated preoperatively (CIpre), at its peak level (CImax), at termination of helmet therapy (CIoff), and at last follow-up (CIfinal). A multivariate regression analysis was performed to determine factors influencing CIfinal.

RESULTS

Thirty-one patients (27 male, 4 female) were treated in the studied time period. The median age at surgery was 2.7 months (range 1.6 to 3.2) and the median duration of PHT was 10.4 months (range 8.4 to 14.4). The mean CImax was 0.83 (SD 0.01), which was attained an average of 8.4 months (SD 1.2) following PHT initiation. At last follow-up, there was an average retraction of CIfinal among all patients to 0.78 (SD 0.01). Longer helmet duration after achieving CImax did not correlate with higher CIfinal values. While CImax was a significant predictor of CIfinal, neither age at surgery nor CIpre were found to be predictive of final outcome.

CONCLUSIONS

Patients undergoing ESC and PHT for sagittal synostosis reach a peak CI around 7 to 9 months after surgery. PHT beyond CImax does not improve final anthropometric outcomes. CIfinal is significantly dependent on CImax, but not on age, nor CIpre. These results imply that helmet removal at CImax may be appropriate for ESC patients, while helmeting beyond the peak does not change final outcome.

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David S. Hersh, Nir Shimony, Mari L. Groves, Gerald F. Tuite, George I. Jallo, Ann Liu, Tomas Garzon-Muvdi, Thierry A. G. M. Huisman, Ryan J. Felling, Joseph A. Kufera, and Edward S. Ahn

OBJECTIVE

Pediatric cerebral venous sinus thrombosis has been previously described in the setting of blunt head trauma; however, the population demographics, risk factors for thrombosis, and the risks and benefits of detection and treatment in this patient population are poorly defined. Furthermore, few reports differentiate between different forms of sinus pathology. A series of pediatric patients with skull fractures who underwent venous imaging and were diagnosed with intrinsic cerebral venous sinus thrombosis or extrinsic sinus compression is presented.

METHODS

The medical records of patients at 2 pediatric trauma centers were retrospectively reviewed. Patients who were evaluated for blunt head trauma from January 2003 to December 2013, diagnosed with a skull fracture, and underwent venous imaging were included.

RESULTS

Of 2224 pediatric patients with skull fractures following blunt trauma, 41 patients (2%) underwent venous imaging. Of these, 8 patients (20%) had intrinsic sinus thrombosis and 14 patients (34%) displayed extrinsic compression of a venous sinus. Three patients with intrinsic sinus thrombosis developed venous infarcts, and 2 of these patients were treated with anticoagulation. One patient with extrinsic sinus compression by a depressed skull fracture underwent surgical elevation of the fracture. All patients with sinus pathology were discharged to home or inpatient rehabilitation. Among patients who underwent follow-up imaging, the sinus pathology had resolved by 6 months postinjury in 80% of patients with intrinsic thrombosis as well as 80% of patients with extrinsic compression. All patients with intrinsic thrombosis or extrinsic compression had a Glasgow Outcome Scale score of 4 or 5 at their last follow-up.

CONCLUSIONS

In this series of pediatric trauma patients who underwent venous imaging for suspected thrombosis, the yield of detecting intrinsic thrombosis and/or extrinsic compression of a venous sinus was high. However, few patients developed venous hypertension or infarction and were subsequently treated with anticoagulation or surgical decompression of the sinus. Most had spontaneous resolution and good neurological outcomes without treatment. Therefore, in the setting of pediatric skull fractures after blunt injury, venous imaging is recommended when venous hypertension or infarction is suspected and anticoagulation is being considered. However, there is little indication for pervasive venous imaging after pediatric skull fractures, especially in light of the potential risks of CT venography or MR venography in the pediatric population and the unclear benefits of anticoagulation.

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Wuyang Yang, Risheng Xu, Jose L. Porras, Clifford M. Takemoto, Syed Khalid, Tomas Garzon-Muvdi, Justin M. Caplan, Geoffrey P. Colby, Alexander L. Coon, Rafael J. Tamargo, Judy Huang, and Edward S. Ahn

OBJECTIVE

Sickle cell disease (SCD) in combination with moyamoya syndrome (MMS) represents a rare complication of SCD, with potentially devastating neurological outcomes. The effectiveness of surgical revascularization in this patient population is currently unclear. The authors’ aim was to determine the effectiveness of surgical intervention in their series of SCD-MMS patients by comparing stroke recurrence in those undergoing revascularization and those undergoing conservative transfusion therapy.

METHODS

The authors performed a retrospective chart review of patients with MMS who were seen at the Johns Hopkins Medical Institution between 1990 and 2013. Pediatric patients (age < 18 years) with confirmed diagnoses of SCD and MMS were included. Intracranial stroke occurrence during the follow-up period was compared between surgically and conservatively managed patients.

RESULTS

A total of 15 pediatric SCD-MMS patients (28 affected hemispheres) were included in this study, and all were African American. Seven patients (12 hemispheres) were treated with indirect surgical revascularization. The average age at MMS diagnosis was 9.0 ± 4.0 years, and 9 patients (60.0%) were female. Fourteen patients (93.3%) had strokes before diagnosis of MMS, with an average age at first stroke of 6.6 ± 3.9 years. During an average follow-up period of 11.6 years, 4 patients in the conservative treatment group experienced strokes in 5 hemispheres, whereas no patient undergoing the revascularization procedure had any strokes at follow-up (p = 0.029). Three patients experienced immediate postoperative transient ischemic attacks, but all recovered without subsequent strokes.

CONCLUSIONS

Indirect revascularization is suggested as a safe and effective alternative to the best medical therapy alone in patients with SCD-MMS. High-risk patients managed on a regimen of chronic transfusion should be considered for indirect revascularization to maximize the effect of stroke prevention.

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Miguel Gelabert-González, Eduardo Arán-Echabe, and José María Santín-Amo

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Jonathan Pindrik, Joseph Molenda, Rafael Uribe-Cardenas, Amir H. Dorafshar, and Edward S. Ahn

OBJECTIVE

Subjective evaluations typically guide craniosynostosis repair. This study provides normative values of anthropometric cranial indices that are clinically useful for the evaluation of multiple types of craniosynostosis and introduces 2 new indices that are useful in the evaluation and management of metopic and bicoronal synostosis. The authors hypothesize that normative values of the new indices as well as for established measures like the cephalic index can be drawn from the evaluation of CT scans of normal individuals.

METHODS

High-resolution 3D CT scans obtained in normal infants (age 0–24 months) were retrospectively reviewed. Calvarial measurements obtained from advanced imaging visualization software were used to compute cranial indices. Additionally, metopic sutures were evaluated for patency or closure.

RESULTS

A total of 312 participants were included in the study. Each monthly age group (total 24) included 12–18 patients, yielding 324 head CT scans studied. The mean cephalic index decreased from 0.85 at age 0–3 months to 0.81 at 19–24 months, the mean frontoparietal index decreased from 0.68 to 0.65, the metopic index from 0.59 to 0.55, and the towering index remained comparatively uniform at 0.64 and 0.65. Trends were statistically significant for all measured indices. There were no significant differences found in mean cranial indices between sexes in any age group. Metopic suture closure frequency for ages 3, 6, and 9 months were 38.5%, 69.2%, and 100.0%, respectively.

CONCLUSIONS

Radiographically acquired normative values for anthropometric cranial indices during infancy can be used as standards for guiding preoperative decision making, surgical correction, and postoperative helmeting in various forms of craniosynostosis. Metopic and towering indices represent new cranial indices that are potentially useful for the clinical evaluation of metopic and bicoronal synostoses, respectively. The present study additionally shows that metopic suture closure appears ubiquitous after 9 months of age.

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Alan F. Utria, Joseph Lopez, Regina S. Cho, Gerhard S. Mundinger, George I. Jallo, Edward S. Ahn, Craig Vander Kolk, and Amir H. Dorafshar

OBJECTIVE

Due to the changing properties of the infant skull, there is still no clear consensus on the ideal time to surgically intervene in cases of nonsyndromic craniosynostosis (NSC). This study aims to shed light on how patient age at the time of surgery may affect surgical outcomes and the subsequent need for reoperation.

METHODS

A retrospective cohort review was conducted for patients with NSC who underwent primary cranial vault remodeling between 1990 and 2013. Patients' demographic and clinical characteristics and surgical interventions were recorded. Postoperative outcomes were assessed by assigning each procedure to a Whitaker category. Multivariate logistic regression analysis was performed to determine the relationship between age at surgery and need for minor (Whitaker I or II) versus major (Whitaker III or IV) reoperation. Odds ratios (ORs) for Whitaker category by age at surgery were assigned.

RESULTS

A total of 413 unique patients underwent cranial vault remodeling procedures for NSC during the study period. Multivariate logistic regression demonstrated increased odds of requiring major surgical revisions (Whitaker III or IV) in patients younger than 6 months of age (OR 2.49, 95% CI 1.05–5.93), and increased odds of requiring minimal surgical revisions (Whitaker I or II) in patients older than 6 months of age (OR 2.72, 95% CI 1.16–6.41).

CONCLUSIONS

Timing, as a proxy for the changing properties of the infant skull, is an important factor to consider when planning vault reconstruction in NSC. The data presented in this study demonstrate that patients operated on before 6 months of age had increased odds of requiring major surgical revisions.

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Wuyang Yang, Heather Anderson-Keightly, Erick M. Westbroek, Justin M. Caplan, Xiaoming Rong, Alice L. Hung, Geoffrey P. Colby, Alexander L. Coon, Rafael J. Tamargo, Judy Huang, and Edward S. Ahn

OBJECTIVE

Compared with the general population, the specific natural history of arteriovenous malformations (AVMs) in pediatric patients is less well understood. Furthermore, few pediatric studies have compared posttreatment hemorrhagic risk and functional outcome across different treatment modalities. The objective of this study was to elucidate these points.

METHODS

The authors retrospectively reviewed all pediatric patients with AVMs evaluated at their institution between 1990 and 2013. The AVM natural history was represented by hemorrhagic risk during the observation period. For treated patients, the observation period was defined as the interval between diagnosis and treatment. Posttreatment hemorrhagic risk and functional outcomes were also assessed.

RESULTS

A total of 124 pediatric patients with AVMs were evaluated, and 90 patients (72.6%) were retained through follow-up. The average patient age was 13.3 ± 3.8 years, with a mean follow-up period of 9.95 years. The overall AVM obliteration rate was 59.7%. Radiosurgery had an obliteration rate of 49.0%. Thirteen patients were managed conservatively. Four patients under observation hemorrhaged during a total interval of 429.4 patient-years, translating to an annual risk of 0.9%. Posttreatment hemorrhagic risk by treatment modalities were categorized as follows: surgery ± embolization (0.0%), radiosurgery ± embolization (0.8%), embolization alone (2.8%), surgery + radiosurgery ± embolization (3.5%), and observation (0.8%). A significantly higher risk of posttreatment hemorrhage was observed for patients with hemorrhagic presentation (p = 0.043) in multivariate analysis. Seizure presentation, frontal lobe location, nonheadache presentation, and treatment modality were significantly associated with increased risk of poor functional outcomes.

CONCLUSIONS

In this study of pediatric patients with AVMs, the natural history of hemorrhage was relatively low at 0.9%. Resection remained the optimal management for hemorrhage control and functional outcome perseverance in these pediatric patients with AVMs. AVM obliteration is a valid treatment goal, especially for patients with ruptured presentation, to prevent further hemorrhages later in life.

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Joanna Y. Wang, Amir H. Dorafshar, Ann Liu, Mari L. Groves, and Edward S. Ahn

OBJECTIVE

Because the metopic suture normally fuses during infancy, there are varying degrees of severity in head shape abnormalities associated with premature fusion. A method for the objective and reproducible assessment of metopic synostosis is needed to guide management, as current methods are limited by their reliance on aesthetic markers. The object of this study was to describe the metopic index (MI), a simple anthropometric cranial measurement. The measurements can be obtained from CT scans and, more importantly, from palpable cranial landmarks, and the index provides a rapid tool for evaluating patients in both pre- and postoperative settings.

METHODS

High-resolution head CT scans obtained in 69 patients (age range 0–24 months) diagnosed with metopic craniosynostosis were retrospectively reviewed. Preoperative 3D reconstructions were available in 15 cases, and these were compared with 3D reconstructions of 324 CT scans obtained in a control group of 316 infants (age range 0–24 months) who did not have any condition that might affect head size or shape and also in a subset of this group, comprising 112 patients precisely matched to the craniosynostosis patients with respect to age and sex. Postoperative scans were available and reviewed in 9 of the craniosynostosis patients at a mean time of 7.1 months after surgical repair. 3D reconstructions of these scans were matched with controls based upon age and sex.

RESULTS

The mean preoperative MI for patients with trigonocephaly was 0.48 (SD 0.05), significantly lower than the mean values of 0.57 (SD 0.04) calculated on the basis of all 324 scans obtained in controls (p < 0.001) and 0.58 (SD 0.04) for the subset of 112 age- and sex-matched controls (p < 0.001). For 7 patients with both pre- and postoperative CT scans available for evaluation, the mean postoperative MI was 0.55 (SD 0.03), significantly greater than their preoperative MIs (mean 0.48 [SD 0.04], p = 0.001) and comparable to the mean MI of the controls (p = 0.30). In 4 patients, clinically obtained postoperative MIs by caliper measurement were comparable to measurements derived from CT (p = 0.141).

CONCLUSIONS

The MI is a useful measurement of the severity of trigonocephaly in patients with metopic synostosis. This simple quantitative assessment can potentially be used in the clinical setting to guide preoperative evaluation, surgical repair, and postoperative degree of correction.