Periventricular nodular heterotopia in patients with a prenatal diagnosis of myelomeningocele/myeloschisis: associations with seizures and neurodevelopmental outcomes during early childhood.

PURPOSE: Historically, the presence of gray matter heterotopia was a concern for adverse postnatal neurocognitive status in patients undergoing fetal closure of open spinal dysraphism. The purpose of this study was to evaluate neurodevelopmental outcomes and the onset of seizures during early childhood in patients with a prenatal diagnosis of myelomeningocele/myeloschisis (MMC) and periventricular nodular heterotopia (PVNH). METHODS: All patients evaluated at the Center for Fetal Diagnosis and Treatment with a diagnosis of MMC between June 2016 to March 2023 were identified. PVNH was determined from prenatal and/or postnatal MRI. The Bayley Scales of Infant and Toddler Development (edition III or IV) were used for neurodevelopmental assessments. Patients were screened for seizures/epilepsy. RESULTS: Of 497 patients evaluated with a prenatal diagnosis of MMC, 99 were found to have PVNH on prenatal MRI, of which 35 had confirmed PVNH on postnatal imaging. From the 497 patients, 398 initially did not exhibit heterotopia on prenatal MRI, but 47 of these then had confirmed postnatal PVNH. The presence of PVNH was not a significant risk factor for postnatal seizures in early childhood. The average neurodevelopmental scores were not significantly different among heterotopia groups for cognitive, language, and motor domains. CONCLUSION: The presence of PVNH in patients with a prenatal diagnosis of MMC does not indicate an increased risk for neurodevelopmental delay at 1 year of age. We did not demonstrate an association with seizures/epilepsy. These findings can aid clinicians in prenatal consultation regarding fetal repair of open spinal dysraphism. Long-term follow-up is required to discern the true association between PVNH seen on prenatal imaging and postnatal seizures/epilepsy and neurodevelopmental outcomes.

Vaulting further: cranial vault expansion for craniocerebral disproportion without primary craniosynostosis.

PURPOSE: Treatment of subjects with refractory idiopathic intracranial hypertension (IIH) or shunted hydrocephalus with chronic shunt complications is challenging. What is the role for cranial vault expansion, particularly utilizing posterior vault distraction osteogenesis (PVDO), in these cases? This study assesses medium-term efficacy of cranial vault expansion in this unique patient population. METHODS: A retrospective review was conducted of patients who underwent cranial vault expansion from 2008 to 2023 at the Children's Hospital of Philadelphia. Subjects who did not have a diagnosis of primary craniosynostosis were included in the study. Demographic information, medical history, and perioperative details were collected from medical records. Primary outcomes were the rate of CSF diversion procedures and resolution of presenting signs and symptoms. Secondary outcomes were perioperative and 90-day complications and reoperation requirement. RESULTS: Among 13 included subjects, nine (69.2%) patients had a primary diagnosis of shunted hydrocephalus and 4 (30.8%) patients had IIH. Twelve (92.3%) subjects underwent posterior vault distraction osteogenesis (PVDO) and one (7.7%) underwent posterior vault remodeling (PVR). All 4 patients with IIH demonstrated symptomatic improvement following PVDO, including resolution of headaches, vomiting, and/or papilledema. Among 9 patients with shunted hydrocephalus, CSF diversion requirement decreased from 2.7 ± 1.6 procedures per year preoperatively to 1.2 ± 1.8 per year following cranial vault expansion (p = 0.030). The mean postoperative follow-up was 4.1 ± 2.1 years and four (30.8%) patients experienced complications within 90 days of surgery, including infection (n = 2), CSF leak (n = 1), and elevated ICP requiring lumbar puncture (n = 1). Four (30.8%) patients underwent repeat cranial vault expansion for recurrence of ICP-related symptoms. At most recent follow-up, 7 of 9 patients with shunted hydrocephalus demonstrated symptomatic improvement. CONCLUSION: Cranial vault expansion reduced intracranial hypertension-related symptomology as well as the rate of CSF diversion-related procedures in patients with refractory IIH and shunted hydrocephalus without craniosynostosis, and should be considered in those who have significant shunt morbidity.

Long-term aesthetic and photogrammetric outcomes in non-syndromic unicoronal synostosis: comparison of fronto-orbital distraction osteogenesis and fronto-orbital advancement and remodeling.

BACKGROUND: Fronto-orbital advancement and remodeling (FOAR) is among the most common surgical approaches for unicoronal craniosynostosis (UCS), although some data demonstrate failure to achieve long-term aesthetic normalcy, leading some to seek alternative treatment paradigms such as fronto-orbital distraction osteogenesis (FODO). This study compares long-term aesthetic outcomes of patients with UCS treated with FOAR and FODO. METHODS: Twenty patients (four males) with non-syndromic UCS presenting to our institution and undergoing distraction were compared to a matched cohort of 20 patients (six males) undergoing FOAR. Clinical photographs and ImageJ were used to quantify periorbital anatomy including palpebral fissures, pupil-to-brow distance (PTB), and margin-reflex distance (MRD1) in pixels. Whitaker classification was blindly assigned by craniofacial surgeons. RESULTS: Photogrammetric analysis and Mann-Whitney U tests demonstrated significantly improved postoperative symmetry in distraction patients for palpebral width (p = 0.020), MRD1 (p = 0.045), and canthal tilt (p = 0.010). Average Whitaker classification scores between FOAR (1.94) and distraction (1.79) cohorts were similar (p = 0.374). CONCLUSIONS: UCS patients demonstrated significant postoperative improvements in periorbital symmetry, with distraction patients demonstrating superior results in palpebral width and canthal tilt. FOAR and FODO patients achieved similar Whitaker classification scores. These cohorts will be followed until craniofacial maturity prior to making any definitive conclusions.

Tethered cord release in patients after open fetal myelomeningocele closure: intraoperative neuromonitoring data and patient outcomes.

PURPOSE: The purpose of the study was to better understand the clinical course and impact of tethered cord release surgery on patients who have previously undergone open spinal dysraphism closure in utero. METHODS: This is a single-center retrospective observational study on patients undergoing tethered cord release after having previously had open fetal myelomeningocele (MMC) closure. All patients underwent tethered cord release surgery with a single neurosurgeon. A detailed analysis of the patients' preoperative presentation, intraoperative neuromonitoring (IONM) data, and postoperative course was performed. RESULTS: From 2009 to 2021, 51 patients who had previously undergone fetal MMC closure had tethered cord release surgery performed. On both preoperative and postoperative manual motor testing, patients were found to have on average 2 levels better than would be expected from the determined anatomic level from fetal imaging. The electrophysiologic functional level was found on average to be 2.5 levels better than the anatomical fetal level. Postoperative motor levels when tested on average at 4 months were largely unchanged when compared to preoperative levels. Unlike the motor signals, 46 (90%) of patients had unreliable or undetectable lower extremity somatosensory evoked potentials (SSEPs) prior to the tethered cord release. CONCLUSION: Tethered cord surgery can be safely performed in patients after open fetal MMC closure without clinical decline in manual motor testing. Patients often have functional nerve roots below the anatomic level. Sensory function appears to be more severely affected in patients leading to a consistent motor-sensory imbalance.

Craniosynostosis in primary metabolic bone disorders: a single-institution experience.

PURPOSE: The incidence of metabolic bone diseases in pediatric neurosurgical patients is rare. We examined our institutional experience of metabolic bone diseases along with a review of the literature in an effort to understand management for this rare entity. METHODS: Retrospective review of the electronic medical record database was performed to identify patients with primary metabolic bone disorders who underwent craniosynostosis surgery between 2011 and 2022 at a quaternary referral pediatric hospital. Literature review was conducted for primary metabolic bone disorders associated with craniosynostosis. RESULTS: Ten patients were identified, 6 of whom were male. The most common bone disorders were hypophosphatemic rickets (n = 2) and pseudohypoparathyroidism (n = 2). The median age at diagnosis of metabolic bone disorder was 2.02 years (IQR: 0.11-4.26), 2.52 years (IQR: 1.24-3.14) at craniosynostosis diagnosis, and 2.65 years (IQR: 0.91-3.58) at the time of surgery. Sagittal suture was most commonly fused (n = 4), followed by multi-suture craniosynostosis (n = 3). Other imaging findings included Chiari (n = 1), hydrocephalus (n = 1), and concurrent Chiari and hydrocephalus (n = 1). All patients underwent surgery for craniosynostosis, with the most common operation being bifronto-orbital advancement (n = 4). A total of 5 patients underwent reoperation, 3 of which were planned second-stage surgeries and 2 of whom had craniosynostosis recurrence. CONCLUSIONS: We advocate screening for suture abnormalities in children with primary metabolic bone disorders. While cranial vault remodeling is not associated with a high rate of postoperative complications in this patient cohort, craniosynostosis recurrences may occur, and parental counseling is recommended.

Spinal pseudomeningocele closure: capsulofascial interposition technique.

INTRODUCTION: The development of a spinal pseudomeningocele is a complication of dural repair or reconstruction that carries significant morbidity for pediatric patients. In addition to cerebrospinal fluid (CSF) hypotension and positional headaches, CSF leaking into the extradural space increases the risk for incisional breakdown, meningitis, and cosmetic deformity. Spinal pseudomeningocele management is challenging, and reported techniques range from local wound revisions/exploration to shunt placement for permanent CSF diversion. METHODS: At our institution, we have recently implemented a multidisciplinary approach of capsulofascial interposition for the surgical repair of the symptomatic spinal pseudomeningocele with a combined neurosurgery-plastic surgery team. To our knowledge, this technique has not previously been characterized, and we describe the technical aspects of this surgery here. RESULTS: Among 10 patients treated with this technique, none required reoperation or developed infections following pseudomeningocele closure; 2 patients received postoperative transfusions; and 2 patients underwent CSF diversion procedures. CONCLUSION: We propose that this capsulofascial interposition technique should be employed in the surgical treatment of symptomatic spinal pseudomeningoceles.

Survey of Quaternary Neonatal Management of Posthemorrhagic Hydrocephalus.

OBJECTIVE: This study aimed to determine clinical care practices for infants at risk for posthemorrhagic hydrocephalus (PHH) across level IV neonatal intensive care units (NICUs). STUDY DESIGN: Cross-sectional survey that addressed center-specific surveillance, neurosurgical intervention, and follow-up practices within the Children's Hospitals Neonatal Consortium. RESULTS: We had a 59% (20/34 sites) response rate, with 10 sites having at least two participants. Respondents included neonatologists (53%) and neurosurgeons (35%). Most participants stated having a standard guideline for PHH (79%). Despite this, 42% of respondents perceive inconsistencies in management. Eight same-center pairs of neonatologists and neurosurgeons were used to determine response agreement. Half of these pairs disagreed on nearly all aspects of care. The greatest agreement pertained to a willingness to adopt a consensus-based protocol. CONCLUSION: Practice variation in the management of infants at risk of PHH in level IV NICUs exists despite the perception that a common practice is available and used. KEY POINTS: · Practice variation exists despite the perception that common practices are available/used for PHH.. · Our survey had same-center pairs of neonatologist and neurosurgeons to determine response agreement.. · The greatest agreement pertained to a willingness to adopt a consensus-based protocol..

Separation of Craniopagus Twins by a Multidisciplinary Team.

Conjoined twins who are classified as craniopagus (joined at the cranium) have a rare congenital anomaly. Despite advances in surgical techniques and critical care, the rate of complications and death is still high among twins with total fusion in which the superior sagittal sinus is shared. Here, we describe total-fusion craniopagus twins who at 10 months of age underwent successful surgical separation performed by a multidisciplinary team. Computer-aided design and modeling with a three-dimensional printer, custom-designed cranial distraction and constriction devices, and intraoperative navigation techniques were used. These techniques allowed for separation of the twins at an early age and harnessed the regenerative capacity of their young brains.

Neurocognitive outcomes of children with non-syndromic single-suture craniosynostosis.

While the focus of craniosynostosis surgery is to improve head shape, neurocognitive sequelae are common and are incompletely understood. Neurodevelopmental problems that children with craniosynostosis face include cognitive and language impairments, motor delays or deficits, learning disabilities, executive dysfunction, and behavioral problems. Studies have shown that children with multiple suture craniosynostosis have more impairment than children with single-suture craniosynostosis. Children with isolated single-suture subtypes of craniosynostosis such as sagittal, metopic, and unicoronal craniosynostosis can have distinct neurocognitive profiles. In this review, we discuss the unique neurodevelopmental profiles of children with single-suture subtypes of craniosynostosis.

Validation of Sonographic Fronto-Occipital Ratio Based on Anatomical Landmarks Compared to MR/CT-Derived Indexes in Children with Chiari II and Ventriculomegaly.

INTRODUCTION: Ultrasound (US)-based indexes such as fronto-occipital ratio (FOR) can be used to obtain an acceptable estimation of ventricular volume. Patients with colpocephaly present a unique challenge due to the shape of their ventricles. In the present study, we aimed to evaluate the validity and reproducibility of the modified US-FOR index in children with Chiari II-related ventriculomegaly. METHODS: In this retrospective study, we evaluated Chiari II patients younger than 1 year who underwent head US and MR or CT scans for ventriculomegaly evaluation. MR/CT-based FOR was measured in the axial plane by identifying the widest diameter of frontal horns, occipital horns, and the interparietal diameter (IPD). US-based FOR (US-FOR) was measured using the largest diameter based on the following landmarks: frontal horn and IPD in the coronal plane at the level of the foramen of Monro, IPD just superior to the Sylvian fissures, and occipital horn posterior to the thalami and inferior to the superior margins of the thalami. Intraclass correlation coefficients (ICCs) were used to evaluate inter-rater reliability, and Pearson correlation coefficients and Bland-Altman plots were applied to assess agreement between US and other two modalities. RESULTS: Sixty-three paired US and MR/CT exams were assessed for agreement between US-FOR and MR/CT-FOR measurements. ICC showed an excellent inter-rater reliability for US-FOR (ICC = 0.99, p < 0.001) and MR/CT-FOR (ICC = 0.99, p < 0.001) measurements. The mean (range) values based on US-FOR showed a slight overestimation in comparison with MR/CT-FOR (0.51 [0.36-0.68] vs. 0.46 [0.34-0.64]). The Pearson correlation coefficient showed high cross-modality agreement for the FOR index (r = 0.83, p < 0.001). The Bland-Altman plot showed excellent concordance between US-FOR and MR/CT-FOR with a bias of 0.05 (95% CI: -0.03 to 0.13). CONCLUSION: US-FOR in the coronal plane is a comparable tool for evaluating ventriculomegaly in Chiari II patients when compared with MR/CT-FOR, even in the context of colpocephaly.

Endoscopic-Assisted Fronto-Orbital Distraction Osteogenesis: Initial Patient Report.

BACKGROUND: The purpose of this manuscript is to introduce a novel endoscopic-assisted approach for creation of osteotomies and distractor hardware placement for unicoronal craniosynostosis. METHODS: Only three small incisions are performed, one at the anterior fontanelle, pterional region, and upper lateral blepharoplasty location. Unicoronal strip craniectomy and osteotomies in temporal bone, nasofrontal junction, lateral orbital rim, sphenoid wing, orbital roof, and contralateral frontal bone are performed through these incisions with the assistance of a 30° 5-mm endoscope. One linear cranial distractor is inserted through the pterional incision and fixated across the unicoronal suturectomy. RESULTS: Endoscopic-assisted fronto-orbital distraction osteogenesis has been performed on 3 patients with unicoronal craniosynostosis. Median age at surgery was 5.4 months (95%CI 5.0-6.1). Median operative duration was 98 minutes (95%CI 91-112), and estimated blood loss was 25 mL (95%CI 15-150). Median length of distraction achieved was 25.7 mm (95%CI 21.9-28.0), based on lateral skull x-rays obtained on the last day of activation. Distractors were removed 2.3 months postoperatively (95%CI 2.1-3.1), and operative duration of distractor removal was 20 minutes (95%CI 19-29). There was 1 complication, a transient cerebrospinal fluid leak thought to have been caused by a pinpoint injury to the dura at the nasofrontal junction, which was managed expectantly and resolved spontaneously by the fourth postoperative day. CONCLUSIONS: Endoscopic-assisted fronto-orbital distraction osteogenesis is a promising treatment modality for children with unicoronal craniosynostosis combining the benefits of distraction osteogenesis with a minimally invasive approach. Additional experience and follow-up are needed to determine its utility, safety, and longevity.

Preprocedural Electrophysiological Monitoring in Craniofacial Surgery for a Patient with Chiari Malformation.

Head and neck positioning is a key element of craniofacial reconstructive surgery and can become challenging when intervention necessitates broad exposure of the calvarium. We present a case of craniosynostosis secondary to Apert's syndrome requiring anterior and posterior cranial vault access during surgical correction. A modified sphinx position was used that required significant neck extension. The patient had concurrent Chiari I malformation with brain stem compression so intraoperative neuromonitoring (IONM) was used to ensure that there were no negative effects on the neural elements with positioning. This highlights benefits of IONM in a setting not typically associated with its use.

Optical Detection of Intracranial Pressure and Perfusion Changes in Neonates With Hydrocephalus.

OBJECTIVE: To demonstrate that a novel noninvasive index of intracranial pressure (ICP) derived from diffuse optics-based techniques is associated with intracranial hypertension. STUDY DESIGN: We compared noninvasive and invasive ICP measurements in infants with hydrocephalus. Infants born term and preterm were eligible for inclusion if clinically determined to require cerebrospinal fluid (CSF) diversion. Ventricular size was assessed preoperatively via ultrasound measurement of the fronto-occipital (FOR) and frontotemporal (FTHR) horn ratios. Invasive ICP was obtained at the time of surgical intervention with a manometer. Intracranial hypertension was defined as invasive ICP ≥15 mmHg. Diffuse optical measurements of cerebral perfusion, oxygen extraction, and noninvasive ICP were performed preoperatively, intraoperatively, and postoperatively. Optical and ultrasound measures were compared with invasive ICP measurements, and their change in values after CSF diversion were obtained. RESULTS: We included 39 infants, 23 with intracranial hypertension. No group difference in ventricular size was found by FOR (P = .93) or FTHR (P = .76). Infants with intracranial hypertension had significantly higher noninvasive ICP (P = .02) and oxygen extraction fraction (OEF) (P = .01) compared with infants without intracranial hypertension. Increased cerebral blood flow (P = .005) and improved OEF (P < .001) after CSF diversion were observed only in infants with intracranial hypertension. CONCLUSIONS: Noninvasive diffuse optical measures (including a noninvasive ICP index) were associated with intracranial hypertension. The findings suggest that impaired perfusion from intracranial hypertension was independent of ventricular size. Hemodynamic evidence of the benefits of CSF diversion was seen in infants with intracranial hypertension. Noninvasive optical techniques hold promise for aiding the assessment of CSF diversion timing.

The Expanding Role of Posterior Vault Distraction Osteogenesis in Idiopathic Intracranial Hypertension and Slit Ventricle Syndrome.

ABSTRACT: The purpose of this study was to compare perioperative safety and efficacy of posterior vault distraction osteogenesis (PVDO) in patients with primary nonsynostotic cephalo-cranial disproportion, namely slit ventricle syndrome and idiopathic intracranial hypertension (IIH), to a cohort of patients with craniosynostosis (CS). A retrospective review of patients undergoing PVDO from 2009 to 2019 at our institution was performed. Craniosynostosis patients were matched by sex and age at PVDO to the nonsynostotic cohort. Operative details, perioperative outcomes, and distraction patterns were analyzed with appropriate statistics. Nine patients met inclusion criteria for the non-CS cohort. Six patients (67%) underwent PVDO for slit ventricle and the remaining 3 patients (33%) underwent PVDO for IIH. The majority of CS patients were syndromic (n = 6, 67%) and had multisuture synostosis (n = 7, 78%). The non-CS cohort underwent PVDO at a median 56.1 months old [Q1 41.1, Q3 86.6] versus the CS cohort at 55.7 months [Q1 39.6, Q3 76.0] (P = 0.931). Total hospital length of stay was longer in the non-CS patients (median days 5 [Q1 4, Q3 6] versus 3 [Q1 3, Q3 4], P = 0.021). Non-CS patients with ventriculoperitoneal shunts had significantly less shunt operations for ICP concerns post-PVDO (median rate: 1.74/year [Q1 1.30, Q3 3.00] versus median: 0.18/year [Q1 0.0, Q3 0.7]; P = 0.046). In this pilot study using PVDO to treat slit ventricle syndrome and IIH, safety appears similar to PVDO in the synostotic setting. The cohort lacks adequate follow-up to assess long term efficacy, although short-midterm follow-up demonstrates promising results with less need for shunt revision and symptomatic relief. Future studies are warranted to identify the preferred surgical approach in these complex patients.

What is the Role of Force in Correcting Scaphocephaly Through Spring-Mediated Cranial Vault Expansion for Sagittal Craniosynostosis?

OBJECTIVES: Spring-mediated cranial vault expansion (SMC) may enable less invasive treatment of sagittal craniosynostosis than conventional methods. The influence of spring characteristics such as force, length, and quantity on cranial vault outcomes are not well understood. Using in vivo and ex vivo models, we evaluate the interaction between spring force, length, and quantity on correction of scaphocephalic deformity in patients undergoing SMC. METHODS: The authors retrospectively studied subjects with isolated sagittal craniosynostosis who underwent SMC between 2011 and 2019. The primary outcome measure of in vivo analysis was head shape determined by cephalic index (CI). Ex vivo experimentation analyzed the impact of spring length, bend, and thickness on resultant force. RESULTS: Eighty-nine subjects underwent SMC at median 3.4 months with median preoperative CI 69% (interquartile range: 66, 71%). Twenty-six and 63 subjects underwent SMC with 2 and 3 springs, with mean total force 20.1 and 27.6 N, respectively (P < 0.001).Postoperative CI increased from 71% to 74% and 68% to 77% in subjects undergoing 2- and 3-spring cranioplasty at the 6-month timepoint, respectively (P < 0.001). Total spring force correlated to increased change in CI (P < 0.002). Spring length was inversely related to transverse cranial expansion at Postoperative day 1, however, directly related at 1 and 3 months (P < 0.001). Ex vivo modeling of spring length was inversely related to spring force regardless of spring number (P < 0.0001). Ex vivo analysis demonstrated greater resultant force when utilizing wider, thicker springs independent of spring arm length and degree of compression. CONCLUSIONS: A dynamic relationship among spring characteristics including length, bend, thickness, and quantity appear to influence SMC outcomes.

Decadron, Diamox, and Zantac: A Novel Combination for Ventricular Shunt Failure in Pediatric Neurosurgical Patients.

OBJECTIVE: Cerebral ventricular shunt failure is common and presents with symptoms that range from headaches to death. The combination of Diamox (acetazolamide), Decadron (dexamethasone), and Zantac (ranitidine) (DDZ) is used at our institution to medically stabilize pediatric patients presenting with symptomatic shunt failure before shunt revision. We describe our experience of this drug combination as a temporizing measure to decrease symptoms associated with shunt failure. METHODS: We performed a single-center retrospective chart review of patients younger than 18 years with ventricular shunt failure who underwent a shunt revision between January 2015 to October 2017 and received DDZ before surgery. The outcome variables evaluated included pre-DDZ and post-DDZ clinical symptoms, pain scores, and vital signs. RESULTS: There were 112 cases that received DDZ before shunt revision. The 4 most commonly reported symptoms were analyzed. Headache was observed in 42 cases pre-DDZ, and post-DDZ there was a 71% reduction in headache (P < 0.0001); emesis was reported pre-DDZ in 76 cases, and post-DDZ there was an 83% reduction (P < 0.0001); irritability was noted pre-DDZ in 30 cases, and post-DDZ there was a 77% reduction (P = 0.0003); lethargy pre-DDZ was observed in 60 cases, and post-DDZ 73% demonstrated improvement (P < 0.0001). Maximum pain scores significantly decreased post-DDZ (P < 0.0001). Heart rate, systolic, and diastolic blood pressures significantly decreased post-DDZ (P < 0.0001, P < 0.0001, P = 0.0002, respectively). CONCLUSIONS: The combination of Decadron, Diamox, and Zantac is a novel treatment for ventricular shunt failure that may temporarily improve symptoms in patients awaiting shunt revision. Future studies could compare efficacy with other medical treatments.

Neonatal intraoperative neuromonitoring in thoracic myelocystocele: a case report.

Thoracic myelocystocele are extremely rare, non-terminal, closed neural tube defects. Intraoperative neuromonitoring (IONM) is a mainstay of pediatric spinal surgery. However, in neonates and infants, incomplete myelination of the corticospinal tract presents unique challenges to successful use of IONM in this vulnerable patient population. Surgery can often be delayed until patients are older, but there are circumstances in which early intervention is necessary. We report a case of T6 myelocystocele resection and wound closure in an infant on day of life 15 with the use of IONM. To our knowledge, this is the youngest reported patient to undergo successful IONM in the spinal cord. Given that the majority of thoracic myelocystoceles present without any neurological deficits, early intervention for this rare closed spinal dysraphism is sometimes necessary. This case study reports the possibility of IONM use in neonatal patients and also highlights the techniques that make its use more possible.

Intracranial pressure patterns in children with craniosynostosis utilizing optical coherence tomography.

BACKGROUND: Better understanding the incidence and patterns of elevated intracranial pressure (ICP) in patients with craniosynostosis may facilitate more timely intervention to alter neurocognitive outcomes. Spectral-domain optical coherence tomography (OCT) of the retina can non-invasively diagnose elevated ICP, and has demonstrated high sensitivity and specificity among patients with craniosynostosis. This study sought to characterize patterns of elevated ICP among patients with craniosynostosis. METHODS: Quantitative retinal parameters were prospectively assessed in both eyes of patients with craniosynostosis using spectral-domain OCT. Based on retinal OCT thresholds associated with elevated ICP (> 15 mmHg), subjects were assigned an OCT diagnosis of elevated or non-elevated ICP which was analyzed relative to clinical characteristics and craniosynostosis patterns. RESULTS: Eighty subjects (aged 0.2-18 years) with craniosynostosis were enrolled; among these, 67 (84%) were nonsyndromic. OCT evaluation was performed at initial vault expansion in 56 (70%) patients. Among this subset, 27 (48%) patients had peri-papillary changes suggestive of elevated ICP, reflecting a 44% incidence in nonsyndromic and 83% in syndromic patients. The median age at initial vault expansion was higher among those with elevated ICP (11.1 months) than those without (7.8 months; p = 0.04.) Multi-suture synostosis was associated with changes consistent with elevated ICP in 9 (75%) patients compared with 18 (41%) with single suture synostosis (p = 0.05). CONCLUSIONS: OCT of the retina produces a potentially sensitive indicator of ICP in craniosynostosis patients. Elevated ICP may be associated with number of involved sutures and older patient presentation, and refining appropriate "cutoffs" will be important as the technology becomes more widespread.

Standardizing treatment of preterm infants with post-hemorrhagic hydrocephalus at a single institution with a multidisciplinary team.

BACKGROUND: Preterm infants with post-hemorrhagic hydrocephalus (PHH) are often treated with temporizing measures such as ventricular access devices (VADs) in order to drain cerebrospinal fluid (CSF) prior to permanent diversion with ventriculoperitoneal shunt (VPS) placement. LOCAL PROBLEM: There is little consensus on the timing and management of VADs and VPSs. This leads to marked practice variations among treating services that can adversely affect patient outcomes. METHODS: This is a quality improvement study evaluating practices from February 2011 to September 2017 including infants with PHH in a single level IV NICU. INTERVENTIONS: A multidisciplinary team created a local clinical pathway modified from the Hydrocephalus Clinical Research Network's Shunting Outcomes in Post-Hemorrhagic Hydrocephalus protocol to manage infants with PHH. Methods of CSF diversion and shunt timing were based on weight. Neonatal care providers performed VAD aspiration; timing was guided by imaging and clinical exam criteria. Surgical procedures were performed in the NICU. RESULTS: There were 78 patients eligible for the study. Prior to pathway implementation, infections occurred in 4% of VAD and 3% of VPS patients. There have been no infections since inception of the pathway. With pathway implementation, treatment compliance improved from 55 to 86% while conversion compliance rate improved from 89 to 100%. CONCLUSIONS: Standardization of care for PHH infants leads to improvement in patient outcomes such as a decrease in time to VAD placement. Reservoir aspirations by the neonatology team did not result in an increase in infection rate.

Perioperative Outcomes in the Treatment of Isolated Sagittal Synostosis: Cranial Vault Remodeling Versus Spring Mediated Cranioplasty.

BACKGROUND: Patients undergoing cranial expansion including spring-mediated cranioplasty (SMC) and cranial vault remodeling (CVR) receive costly and high acuity post-operative intensive care (ICU) given concerns over neurologic and hemodynamic vulnerability. The authors analyzed perioperative and post-operative events for patients presenting with sagittal craniosynostosis (CS) undergoing SMC and CVR in order to compare complication profiles. METHODS: The authors performed a single center retrospective cohort study of patients undergoing SMC and CVR for the treatment of nonsyndromic, isolated sagittal CS from 2011 to 2018. Perioperative and post-operative factors were collected, focusing on hemodynamic instability and events necessitating ICU care. Mann-Whitney U and Fisher exact tests were used to compare data with significance defined as P < 0.05. RESULTS: Among 106 patients, 65 (61%) underwent SMC and 41 (39%) CVR. All CVR patients received prophylactic whole blood transfusion at time of scalp incision. Acute blood loss anemia was the most common post-operative complication, prompting n = 6 (9.2%) and n = 7 (17.1%) blood transfusions in the SMC and CVR cohorts, respectively (P < 0.24). Hemodynamic instability requiring blood transfusion was rare, occurring post-operatively in n = 2 (3.1%) and n = 2 (4.9%) patients in the SMC and CVR cohorts, respectively (P < 0.64). Two patients in the CVR cohort exhibited new neurologic symptoms that self-resolved, compared to no patients in the SMC cohort (P < 0.15). CONCLUSION: Despite differing degrees of operative invasiveness, post-operative hemodynamic and neurologic decompensation following CVR and SMC for isolated sagittal CS repair remains similarly rare. Indications necessitating post-operative intensive care are infrequent. Post-operative hemoglobin monitoring may enable early prediction for hemodynamic instability.