Delphi Consensus on Diagnostic Criteria for LUMBAR Syndrome.

OBJECTIVE: To develop consensus on diagnostic criteria for LUMBAR syndrome, the association of segmental infantile hemangiomas that affect the Lower body with Urogenital anomalies, Ulceration, spinal cord Malformations, Bony defects, Anorectal malformations, Arterial anomalies and/or Renal anomalies. STUDY DESIGN: These diagnostic criteria were developed by an expert multidisciplinary and multi-institutional team based on analysis of peer-reviewed data, followed by electronic-Delphi consensus of a panel of 61 international pediatric specialists. RESULTS: After 2 Delphi rounds, a 92% or higher level of agreement was reached for each Delphi statement. 98% of panelists agreed with the diagnostic criteria, and 100% agreed the criteria would be useful in clinical practice. The diagnosis of LUMBAR requires the presence of a segmental, or patterned, infantile hemangioma of the lumbosacral, sacrococcygeal, or pelvic cutaneous regions plus one additional criterion of the urogenital, spinal, bony, anorectal, arterial, or renal organ systems. CONCLUSIONS: These diagnostic criteria will enhance clinical care by improving screening, detection, and overall awareness of this poorly understood neurocutaneous disorder. The criteria can be utilized by a wide variety of pediatric subspecialists. In addition, formal criteria will improve phenotypic uniformity among LUMBAR syndrome cohorts and a patient registry, allowing investigators to assess clinical features, long-term outcomes, and results of genetic sequencing in a standardized manner. Finally, these criteria will serve as a starting point for prospective studies to establish formal screening and management guidelines.

First-time tethered cord release among adults with myelomeningocele: an analysis of people in the National Spina Bifida Patient Registry.

OBJECTIVE: The authors analyzed the National Spina Bifida Patient Registry (NSBPR), a national registry that tracks the outcomes for people with various forms of spinal dysraphism, to determine the ongoing longitudinal risk for tethered cord release (TCR) among adults with myelomeningocele who had not previously undergone TCR during childhood. The authors also sought to identify the impact of lesion level, ambulation status, and prior treatments for hydrocephalus or Chiari malformations on TCR rates. METHODS: Adults in the registry who had not previously undergone TCR during childhood were studied. This group was compared with the remaining adults in the registry. The frequency of first-time TCR and time to TCR (using Kaplan-Meier analysis) were determined independently for males and females. Cox proportional hazards analysis identified correlations between sex, best lesion level and ambulation status prior to TCR, and previous treatments for hydrocephalus and Chiari decompression. RESULTS: Among 967 adults in the NSBPR (422 [43.6%] males and 545 [56.4%] females) who had not undergone TCR during childhood, the authors identified 47 people (4.9%) who underwent their first TCR during adulthood. This study cohort had significantly better mean functional motor levels and ambulation compared with the remaining adult cohort (both p < 0.001). The study group included 35 females (74.5%) and 12 males (25.5%); this sex distribution was significantly different in comparison with the remaining adult cohort (p = 0.016). The Kaplan-Meier curves for first TCR for females and males were significantly different (p = 0.01, log-rank test). TCR rates were correlated with sex (males had decreased risk; OR 0.31, 95% CI 0.16-0.62, p < 0.001), prior treatment for hydrocephalus (those who underwent prior treatment had decreased risk; OR 0.21, 95% CI 0.20-0.42, p < 0.001), and prior treatment for Chiari malformation (those who underwent prior treatment had greater risk; OR 3.84, 95% CI 1.50-9.88, p = 0.005). CONCLUSIONS: Adults with myelomeningocele who escape childhood without undergoing TCR have an ongoing, albeit decreased, risk for spinal cord tethering requiring TCR. This risk is obviously not due to spinal column growth and therefore must reflect other factors such as dynamic changes in spinal cord health over time. Among people with MMC who underwent their first TCR as adults, females seemed to be overrepresented. Similar to the authors' prior childhood study, people who underwent previous Chiari decompression seemed to be overrepresented, whereas those who underwent previous treatment for hydrocephalus seem to be underrepresented. These novel findings deserve further study.

A Cluster Randomized Trial to Reduce Missed Abusive Head Trauma in Pediatric Intensive Care Settings.

OBJECTIVE: To estimate the impact of the PediBIRN (Pediatric Brain Injury Research Network) 4-variable clinical decision rule (CDR) on abuse evaluations and missed abusive head trauma in pediatric intensive care settings. STUDY DESIGN: This was a cluster randomized trial. Participants included 8 pediatric intensive care units (PICUs) in US academic medical centers; PICU and child abuse physicians; and consecutive patients with acute head injures <3 years (n = 183 and n = 237, intervention vs control). PICUs were stratified by patient volumes, pair-matched, and randomized equally to intervention or control conditions. Randomization was concealed from the biostatistician. Physician-directed, cluster-level interventions included initial and booster training, access to an abusive head trauma probability calculator, and information sessions. Outcomes included "higher risk" patients evaluated thoroughly for abuse (with skeletal survey and retinal examination), potential cases of missed abusive head trauma (patients lacking either evaluation), and estimates of missed abusive head trauma (among potential cases). Group comparisons were performed using generalized linear mixed-effects models. RESULTS: Intervention physicians evaluated a greater proportion of higher risk patients thoroughly (81% vs 73%, P = .11) and had fewer potential cases of missed abusive head trauma (21% vs 32%, P = .05), although estimated cases of missed abusive head trauma did not differ (7% vs 13%, P = .22). From baseline (in previous studies) to trial, the change in higher risk patients evaluated thoroughly (67%→81% vs 78%→73%, P = .01), and potential cases of missed abusive head trauma (40%→21% vs 29%→32%, P = .003), diverged significantly. We did not identify a significant divergence in the number of estimated cases of missed abusive head trauma (15%→7% vs 11%→13%, P = .22). CONCLUSIONS: PediBIRN-4 CDR application facilitated changes in abuse evaluations that reduced potential cases of missed abusive head trauma in PICU settings. TRIAL REGISTRATION: ClinicalTrials.gov: NCT03162354.

Assessment of population-based input functions for Patlak imaging of whole body dynamic 18F-FDG PET.

BACKGROUND: Arterial blood sampling is the gold standard method to obtain the arterial input function (AIF) for quantification of whole body (WB) dynamic 18F-FDG PET imaging. However, this procedure is invasive and not typically available in clinical environments. As an alternative, we compared AIFs to population-based input functions (PBIFs) using two normalization methods: area under the curve (AUC) and extrapolated initial plasma concentration (CP*(0)). To scale the PBIFs, we tested two methods: (1) the AUC of the image-derived input function (IDIF) and (2) the estimated CP*(0). The aim of this study was to validate IDIF and PBIF for FDG oncological WB PET studies by comparing to the gold standard arterial blood sampling. METHODS: The Feng 18F-FDG plasma concentration model was applied to estimate AIF parameters (n = 23). AIF normalization used either AUC(0-60 min) or CP*(0), estimated from an exponential fit. CP*(0) is also described as the ratio of the injected dose (ID) to initial distribution volume (iDV). iDV was modeled using the subject height and weight, with coefficients that were estimated in 23 subjects. In 12 oncological patients, we computed IDIF (from the aorta) and PBIFs with scaling by the AUC of the IDIF from 4 time windows (15-45, 30-60, 45-75, 60-90 min) (PBIFAUC) and estimated CP*(0) (PBIFiDV). The IDIF and PBIFs were compared with the gold standard AIF, using AUC values and Patlak Ki values. RESULTS: The IDIF underestimated the AIF at early times and overestimated it at later times. Thus, based on the AUC and Ki comparison, 30-60 min was the most accurate time window for PBIFAUC; later time windows for scaling underestimated Ki (- 6 ± 8 to - 13 ± 9%). Correlations of AUC between AIF and IDIF, PBIFAUC(30-60), and PBIFiDV were 0.91, 0.94, and 0.90, respectively. The bias of Ki was - 9 ± 10%, - 1 ± 8%, and 3 ± 9%, respectively. CONCLUSIONS: Both PBIF scaling methods provided good mean performance with moderate variation. Improved performance can be obtained by refining IDIF methods and by evaluating PBIFs with test-retest data.

Identifying the Misshapen Head: Craniosynostosis and Related Disorders.

Pediatric care providers, pediatricians, pediatric subspecialty physicians, and other health care providers should be able to recognize children with abnormal head shapes that occur as a result of both synostotic and deformational processes. The purpose of this clinical report is to review the characteristic head shape changes, as well as secondary craniofacial characteristics, that occur in the setting of the various primary craniosynostoses and deformations. As an introduction, the physiology and genetics of skull growth as well as the pathophysiology underlying craniosynostosis are reviewed. This is followed by a description of each type of primary craniosynostosis (metopic, unicoronal, bicoronal, sagittal, lambdoid, and frontosphenoidal) and their resultant head shape changes, with an emphasis on differentiating conditions that require surgical correction from those (bathrocephaly, deformational plagiocephaly/brachycephaly, and neonatal intensive care unit-associated skill deformation, known as NICUcephaly) that do not. The report ends with a brief discussion of microcephaly as it relates to craniosynostosis as well as fontanelle closure. The intent is to improve pediatric care providers' recognition and timely referral for craniosynostosis and their differentiation of synostotic from deformational and other nonoperative head shape changes.

Dermal Sinus Tract of Lumbosacral Spine in Children: Patterns on Magnetic Resonance Imaging and Scoring System.

Purpose Dermal sinus tract (DST) is a challenging clinical diagnosis in children. The purpose of our study was to analyze the added value of magnetic resonance imaging (MRI) in the diagnosis of DST involving the lumbosacral spine. We also sought to establish an MRI-based scoring system to simplify the diagnosis of DST. Methods MRI images of 20 patients with clinically suspected DST were retrospectively assessed by two neuroradiologists blinded to the surgical results. The MRI studies were performed from July 2003 to July 2013. Institutional Review Board (IRB) approval was obtained. All MRI studies were assessed with respect to five imaging signs: A) visualization on both sagittal and axial images, B) dural penetration, C) associated tethered cord, D) presence of tumor or inflammation, and E) attachment to conus medullaris. The frequency of each imaging sign in the study population was calculated. For the 20 patients who underwent surgery, sensitivity and specificity of each neuroradiologist's interpretation of the MRI data were calculated using operative findings as the gold standard. Results Twelve of the 20 had confirmed DSTs. The incidences of the five imaging signs were as follows: A) visualization on both sagittal and axial images (12/12, 100%), B) dural penetration (10/12, 83.3%), C) associated tethered cord (7/12, 58.3%), D) presence of tumor or inflammation (4/12, 33.3%), and E) attachment to the conus medullaris (4/12, 33.3%). The best combination of findings predictive of DST was simply the appearance of DST on both axial and sagittal imaging, which resulted in a sensitivity of 100% and a specificity of 75-100%. Conclusion Visualization of DST on both axial and sagittal imaging is the best marker for pathology proven DST. Using a higher threshold score on the five-point scoring system that we proposed did not increase sensitivity or specificity in the diagnosis of DST; however, it may still prove clinically helpful in standardizing reporting leading to a more accurate and detailed assessment.

Congenital Brain and Spinal Cord Malformations and Their Associated Cutaneous Markers.

The brain, spinal cord, and skin are all derived from the embryonic ectoderm; this common derivation leads to a high association between central nervous system dysraphic malformations and abnormalities of the overlying skin. A myelomeningocele is an obvious open malformation, the identification of which is not usually difficult. However, the relationship between congenital spinal cord malformations and other cutaneous malformations, such as dimples, vascular anomalies (including infantile hemangiomata and other vascular malformations), congenital pigmented nevi or other hamartomata, or midline hairy patches may be less obvious but no less important. Pediatricians should be aware of these associations, recognize the cutaneous markers associated with congenital central nervous system malformations, and refer children with such markers to the appropriate specialist in a timely fashion for further evaluation and treatment.

Pediatric tectal plate gliomas: clinical and radiological progression, MR imaging characteristics, and management of hydrocephalus.

OBJECT: Tectal plate gliomas are generally low-grade astrocytomas with favorable prognosis, and observation of the lesion and management of hydrocephalus remain the mainstay of treatment. METHODS: A cohort of patients with tectal plate gliomas at 2 academic institutions was retrospectively reviewed. RESULTS: Forty-four patients with a mean age of 10.2 years who harbored tectal plate gliomas were included in the study. The mean clinical and radiological follow-up was 7.6 ± 3.3 years (median 7.9 years, range 1.5-14.7 years) and 6.5 ± 3.1 years (median 6.5 years, range 1.1-14.7 years), respectively. The most frequent intervention was CSF diversion (81.8% of patients) followed by biopsy (11.4%), radiotherapy (4.5%), chemotherapy (4.5%), and resection (2.3%). On MR imaging tectal plate gliomas most commonly showed T1-weighted isointensity (71.4%), T2-weighted hyperintensity (88.1%), and rarely enhanced (19%). The initial mean volume was 1.6 ± 2.2 cm(3) and it increased to 2.0 ± 4.4 cm(3) (p = 0.628) at the last follow-up. Frontal and occipital horn ratio (FOHR) and third ventricular width statistically decreased over time (p < 0.001 and p < 0.05, respectively). CONCLUSIONS: The authors' results support existing evidence that tectal plate gliomas frequently follow a benign clinical and radiographic course and rarely require any intervention beyond management of associated hydrocephalus.

Multifocal lymphangioendotheliomatosis with devastating intracranial hemorrhage.

An in utero female was found to have a small hemorrhage at the foramen of Monro, hydrocephalus, and what was originally interpreted as a Dandy-Walker variant. At birth she had macrocephaly and numerous cutaneous, multifocal, red-pink blanchable macules. Postnatal MRI demonstrated a hemorrhagic soft-tissue mass involving the upper brainstem, thalamus, and basal ganglia most consistent with in utero complex multifocal intracranial hemorrhage. The skin lesions were thought to be consistent with multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT). The size and location of the hemorrhage precluded operative intervention, although the hydrocephalus was treated with a ventricular shunt. The child continues to have severe developmental delays. Multifocal lymphangioendotheliomatosis with thrombocytopenia is a multifocal vascular disorder most commonly involving the skin and gastrointestinal tract. Intracranial hemorrhages are rare in this context. This case is the third reported instance of MLT with associated intracranial hemorrhage and the only case described in the neurosurgical literature. The authors review the presenting features and pathophysiology of this condition.

Primary intramedullary spinal cord germinoma.

Primary intramedullary spinal cord germinoma (PISCG) is an exceedingly rare diagnosis, with fewer than 30 cases reported in the literature. It is even less common in the pediatric population. Usually, initial imaging at patient presentation reveals a mass. The authors describe the unique case of a child whose initial imaging showed only focal spinal cord atrophy, which was the earliest sign of a slowly growing intramedullary lesion that was eventually proven via biopsy to represent a PISCG. The authors outline this child's diagnostically challenging presentation, review the events leading up to a diagnosis, briefly discuss PISCG, and summarize their recommendations for other physicians who may encounter a similar case. They assert that PISCG should be considered as a rare entity in the differential diagnosis of progressive spinal cord dysfunction even in the absence of an MRI abnormality of an intrinsic spinal cord mass, especially if there is unexplained focal atrophy of the cord.

American College of Surgeons National Surgical Quality Improvement Program Pediatric: a beta phase report.

PURPOSE: The American College of Surgeons (ACS) National Surgical Quality Improvement Program Pediatric (NSQIP-P) expanded to beta phase testing with the enrollment of 29 institutions. Data collection and analysis were aimed at program refinement and development of risk-adjusted models for inter-institutional comparisons. METHODS: Data from the first full year of beta-phase NSQIP-P were analyzed. Patient accrual used ACS-NSQIP methodology tailored to pediatric specialties. Preliminary risk adjusted modeling for all pediatric and neonatal operations and pediatric (excluding neonatal) abdominal operations was performed for all cause morbidity (other than death) and surgical site infections (SSI) using hierarchical logistic regression methodology and eight predictor variables. Results were expressed as odds ratios with 95% confidence intervals. RESULTS: During calendar year 2010, 29 institutions enrolled 37,141 patients. 1644 total CPT codes were entered, of which 456 accounted for 90% of the cases. 450 codes were entered only once (1.2% of cases). For all cases, overall mortality was 0.25%, overall morbidity 7.9%, and the SSI rate 1.8%. For neonatal cases, mortality was 2.39%, morbidity 18.7%, and the SSI rate 3%. For the all operations model, risk-adjusted morbidity institutional odds ratios ranged 0.48-2.63, with 9/29 hospitals categorized as low outliers and 9/29 high outliers, while risk-adjusted SSI institutional odds ratios ranged 0.36-2.04, with 2/29 hospitals low outliers and 7/29 high outliers. CONCLUSION: This report represents the first risk-adjusted hospital-level comparison of surgical outcomes in infants and children using NSQIP-P data. Programmatic and analytic modifications will improve the impact of this program as it moves into full implementation. These results indicate that NSQIP-P has the potential to serve as a model for determining risk-adjusted outcomes in the neonatal and pediatric population with the goal of developing quality improvement initiatives for the surgical care of children.

Intracardiac migration of a distal shunt catheter: an unusual complication of ventricular shunts. Report of 2 cases.

Unusual complications of peritoneal shunts are a well-known occurrence. The authors present 2 cases of intracardiac migration of a distal shunt catheter, summarizing the diagnosis and management of each case. This complication seems to be a rare occurrence; the transgression of the jugular vein leading to intracardiac migration of a shunt catheter has been reported only 6 times previously. The authors highlight the importance of careful and proper placement of the distal peritoneal catheter during the tunneling process, in particular avoiding too deep a penetration of the shunt passer into the neck tissues and too medial a shunt passage near the sternal notch to avoid vascular structures.

Cranial auto graft expansion (CAGE) technique for cranial vault expansion.

We present a method for cranial vault expansion that provides a strong construct, leaves small skull defects, and provides bony coverage of the sagittal sinus. The resultant small defects optimize the possibility for spontaneous reossification while avoiding large calvarial defects. This method has been used in 2 children who developed clinical evidence of total cranial growth restriction.

Arachnoid cyst causing third cranial nerve palsy manifesting as isolated internal ophthalmoplegia and iris cholinergic supersensitivity.

An 8-month-old boy presented with anisocoria, a sluggishly reactive right pupil, and cholinergic supersensitivity as the only signs of what proved months later to be compressive third cranial nerve palsy due to an arachnoid cyst. Tonic constriction and dilation, segmental iris sphincter palsy, aberrant regeneration phenomena, ductional deficits, and ptosis were absent. The initial diagnosis was postganglionic internal ophthalmoplegia attributed to a viral ciliary ganglionopathy. Nineteen months later, he had developed an incomitant exodeviation and a supraduction deficit. Brain MRI revealed a mass consistent with an arachnoid cyst compressing the third cranial nerve in the right interpeduncular cistern. Resection of the cyst led to a persistent complete third cranial nerve palsy. This is the second reported case of prolonged internal ophthalmoplegia in a young child as a manifestation of a compressive third cranial nerve palsy. Our patient serves as a reminder that isolated internal ophthalmoplegia with cholinergic supersensitivity is compatible with a preganglionic compressive third nerve lesion, particularly in a young child.

Temporal factors and the incidence of physical abuse in young children: decreased nonaccidental trauma during Child Abuse Prevention Month.

BACKGROUND/PURPOSE: Educational efforts have been shown to decrease the incidence of abusive injuries. Information related to factors that associate with child abuse would further these educational efforts. We undertook this study to determine the influence of temporal factors and a national preventative program on the incidence of abusive injuries in young children. METHODS: The Pennsylvania Trauma Outcome Study database was queried for all children 36 months old or younger admitted with abusive injuries. Data collected included day, month, and season. Data were analyzed by Poisson regression. RESULTS: Six hundred seventy-two cases of child abuse were reported, with a linear increase in the number of cases per year (P < .001). Sunday had fewer abuse cases admitted than any other day of the week (risk ratio, 0.70; P = .008). Younger children were less likely to be injured during April, which is National Child Abuse Prevention Month, and more likely to be injured during August and October (all P < .05). CONCLUSIONS: We observed fewer inflicted injuries in young children on Sundays, and during April. The causes for these significant variations deserve further study.

Termination of the normal conus medullaris in children: a whole-spine magnetic resonance imaging study.

OBJECT: The mean level of the conus medullaris (CM) has been estimated to lie opposite the L1/2 disc space in several previous studies using ultrasound, CT myelography, and magnetic resonance (MR) imaging, but these studies have been limited in examining only the lumbar spine and including patients being evaluated for back pain and sciatica (creating a selection bias). Moreover, significant variability was found in the termination of the CM, with a small subset of subjects having a CM as low as the mid-body of L4. The authors sought to determine the normal level of the CM and its variability. METHODS: Children with brain or spinal cord tumors who underwent whole-spine surveillance MR imaging were identified retrospectively. The level of the CM was identified in each subject by counting down from C1. Vertebral anomalies, such as lumbarized S1, sacralized L5, or fewer rib-bearing segments, and the presence of fatty filum were noted. RESULTS: Findings regarding the level of termination of the CM were tightly grouped; the average was at the lower third of L1 and the mode of the distribution was at the L1/2 disc space, with very little variation. No CM ended below the mid-body of L2. The level of the CM was not significantly different among individuals with lumbarized or sacralized vertebrae or 11 rib-bearing segments. CONCLUSIONS: The CM terminates most commonly at the L1-2 disc space and in the absence of tethering, the CM virtually never ends below the mid-body of L2. A CM that appears more caudal on neuroimages should be considered tethered.

Incidence and severity of postoperative cerebellar mutism syndrome in children with medulloblastoma: a prospective study by the Children's Oncology Group.

OBJECT: Cerebellar mutism syndrome (CMS) is a unique postoperative syndrome typically arising 1 to 2 days after resection of a midline posterior fossa tumor; it consists of diminished speech progressing to mutism, emotional lability, hypotonia, and ataxia. Most descriptions have been limited to small institutional series using a retrospective chart review methodology. METHODS: The authors incorporated a CMS questionnaire in two large clinical trials (Children's Cancer Group [CCG] 9931, treatment for high-risk medulloblastoma/primitive neuroectodermal tumor; and CCG/Pediatric Oncology Group [POG] A9961, treatment for average-risk medulloblastoma) to prospectively survey for incidence, severity, and possible causes of CMS in children with newly diagnosed medulloblastoma. Information pertaining to 450 of the 463 patients enrolled in the studies was available for review (82 patients in CCG 9931, and 368 patients in CCG/POG A9961). Cerebellar mutism syndrome occurred in 107 (24%) of 450 children. Symptom intensity was judged to have been severe in 43%, moderate in 49%, and mild in 8% of these 107 patients. Mutism and ataxia were the features most frequently judged as severe. In both cohorts, preoperative brainstem invasion was the only feature that correlated with risk of CMS. One year after diagnosis, nonmotor speech/language deficits, neurocognitive deficits, and/or ataxia persisted in a significant fraction of patients. CONCLUSIONS: Nearly one quarter of patients who underwent resection of a medulloblastoma developed symptoms of CMS, of which 92% were judged to be of moderate or severe intensity. Brainstem invasion by tumor was the only risk factor that correlated positively with CMS occurrence; there was a negative correlation with cerebellar hemisphere tumor location. As more radical resections are attempted for medulloblastoma, the potential for increased morbidity must be carefully weighed against prognostic factors, especially in patients with brainstem invasion.

Neurosurgical causes of scoliosis in patients with myelomeningocele: an evidence-based literature review.

OBJECT: Scoliosis in children with myelomeningocele occurs frequently and is multifactorial in origin. The aim of this paper is to review the evidence regarding the causal association, if any, between isolated scoliosis and spinal cord tethering, Chiari malformation, or syringomyelia in this population. METHODS: A Medline search of the English-language literature from 1966 to 2002 was undertaken. All of the pertinent articles that were identified underwent careful review to determine the strength of the evidence supporting the claim that isolated scoliosis in children with myelomeningocele is related to spinal cord tethering, Chiari malformation, or syringomyelia. The strength of the evidence was graded using the Oxford Centre for Evidence-Based Medicine Levels of Evidence (May 2001). A survey was also sent to practicing pediatric neurosurgeons and the returned responses were evaluated. CONCLUSIONS: All available studies were classified as Level 4 studies (case series and flawed cohort and case-control studies). Based on the strength of the available data, there may be an association between spinal cord tethering and scoliosis, particularly in those patients with upper lumbar lesions and spinal curves less than 45 degrees. There is little evidence to support a causal relationship between scoliosis and Chiari malformation or syringomyelia in this population.

Idiopathic thrombocytopenic purpura complicated by an intracranial hemorrhage secondary to an arteriovenous malformation.

OBJECTIVE: To report a case of idiopathic thrombocytopenic purpura (ITP) complicated by an intracranial hemorrhage (ICH) in a child with a previously undiagnosed arteriovenous malformation. CASE: We describe a child with known ITP who developed a severe headache, was evaluated in an emergency department of a community hospital, and was found by computer tomography (CT) scan to have an ICH. Despite treatment with platelets, corticosteroids, and intravenous immunoglobulin, she subsequently developed an acute change in mental status. A second CT scan showed that the hemorrhage had significantly increased in size despite treatment. The patient underwent an emergent splenectomy prior to a craniotomy to remove the hemorrhage. At the time of surgery, it was discovered that she had an arteriovenous malformation at the sight of the hemorrhage. Her recovery was unremarkable and she was discharged to home with no neurologic sequelae. CONCLUSIONS: ICH is a rare but life-threatening complication of ITP. Neurologic symptoms in a child with ITP should be quickly evaluated by CT scan. Most experts suggest careful observation for most cases of ITP. However, when neurologic symptoms occur, more aggressive treatment options must be used. Care of this child included an emergency splenectomy prior to her craniotomy. Pediatric emergency medicine practitioners must be aware of these neurologic symptoms and must not hesitate to involve pediatric surgeons and neurosurgeons in the care of the child. Prompt recognition and early intervention are the keys to improving outcomes when ICH complicates ITP.