Cerebral revascularization surgery reduces cerebrovascular events in children with sickle cell disease and moyamoya syndrome: Results of the stroke in sickle cell revascularization surgery retrospective study.

BACKGROUND: Recent studies suggest that cerebral revascularization surgery may be a safe and effective therapy to reduce stroke risk in patients with sickle cell disease and moyamoya syndrome (SCD-MMS). METHODS: We performed a multicenter, retrospective study of children with SCD-MMS treated with conservative management alone (conservative group)-chronic blood transfusion and/or hydroxyurea-versus conservative management plus surgical revascularization (surgery group). We monitored cerebrovascular event (CVE) rates-a composite of strokes and transient ischemic attacks. Multivariable logistic regression was used to compare CVE occurrence and multivariable Poisson regression was used to compare incidence rates between groups. Covariates in multivariable models included age at treatment start, age at moyamoya diagnosis, antiplatelet use, CVE history, and the risk period length. RESULTS: We identified 141 patients with SCD-MMS, 78 (55.3%) in the surgery group and 63 (44.7%) in the conservative group. Compared with the conservative group, preoperatively the surgery group had a younger age at moyamoya diagnosis, worse baseline modified Rankin scale scores, and increased prevalence of CVEs. Despite more severe pretreatment disease, the surgery group had reduced odds of new CVEs after surgery (odds ratio = 0.27, 95% confidence interval [CI] = 0.08-0.94, p = .040). Furthermore, comparing surgery group patients during presurgical versus postsurgical periods, CVEs odds were significantly reduced after surgery (odds ratio = 0.22, 95% CI = 0.08-0.58, p = .002). CONCLUSIONS: When added to conservative management, cerebral revascularization surgery appears to reduce the risk of CVEs in patients with SCD-MMS. A prospective study will be needed to validate these findings.

Open Retroperitoneal Inferior Vena Cava Cannulation for Distal Ventriculoatrial Shunt Catheter Placement.

Multiple alternative sites for distal ventriculoperitoneal shunts have been described including pleural, atrial, ureteral, fallopian, and gallbladder placement. In medically complex patients the sites for cerebrospinal fluid (CSF) diversion can be exhausted. We present a case where open retroperitoneal inferior vena cava cannulation was used for successful atrial catheter placement in a 17-month-old female. The patient had a complex abdominal, pulmonary, and vascular history precluding placement of the distal catheter in other sites or atrial placement through more peripheral venous cannulation. The patient underwent uncomplicated open retroperitoneal exposure of her inferior vena cava (IVC) with cannulation and placement of atrial catheter under fluoroscopic guidance. At the follow-up one year after surgery, the patient did not require revision with appropriate placement of the distal atrial catheter.

Utility of ventriculogallbladder shunts in complex cases of hydrocephalus related to extreme prematurity.

OBJECTIVE: The management of hydrocephalus resulting from intraventricular hemorrhage related to extreme prematurity remains demanding. Given the complexities of controlling hydrocephalus in this population, less commonly used procedures may be required. The authors examined the utility of ventriculogallbladder (VGB) shunts in a series of such children. METHODS: The authors retrospectively reviewed the medical records of all children who underwent surgery for hydrocephalus in the period from 2011 through 2019 at Children's Healthcare of Atlanta. Six patients who underwent VGB shunt placement were identified among a larger cohort of 609 patients who had either a new shunt or a newly changed distal terminus site. The authors present an analysis of this series, including a case of laparoscopy-assisted distal VGB shunt revision. RESULTS: The mean age at initial shunt placement was 5.1 months (range 3.0-9.4 months), with patients undergoing a mean of 11.8 shunt procedures (range 5-17) prior to the initial VGB shunt placement at a mean age of 5.3 years (range 7.9 months-12.8 years). All 6 patients with VGB shunt placement had hydrocephalus related to extreme prematurity (gestational age < 28 weeks). At the time of VGB shunt placement, all had complex medical and surgical histories, including poor venous access due to congenital or iatrogenic thrombosis or thrombophlebitis and a peritoneum hostile to distal shunt placement related to severe necrotizing enterocolitis. VGB complications included 1 case of shunt infection, identified at postoperative day 6, and 2 cases of distal shunt failure due to retraction of the distal end of the VGB shunt. In all, there were 3 conversions back to ventriculoperitoneal or ventriculoatrial shunts due to the 2 previously mentioned complications, plus 1 patient who outgrew their initial VGB shunt. Three of 6 patients remain with a VGB shunt, including 1 who underwent laparoscopy-assisted distal shunt revision 110.5 months after initial VGB shunt insertion. CONCLUSIONS: Placement of VGB shunts should be considered in the armamentarium of procedures that may be used in the particularly difficult cohort of children with hydrocephalus related to extreme prematurity. VGB shunts show utility as both a definitive treatment and as a "bridge" procedure until the patient is larger and comorbid abdominal and/or vascular issues have resolved sufficiently to allow conversion back to ventriculoperitoneal or ventriculoatrial shunts, if needed.

Chronic encapsulated intraventricular hematoma in a pediatric patient: case report.

Chronic encapsulated intraventricular hematoma (CEIVH) is a rare, intraventricular, nonneoplastic mass lesion that can become symptomatic from mass effect or obstructive hydrocephalus. Only 5 cases have been reported in the literature, and only one of these occurred in a pediatric patient and dates back to the pre-modern neuroimaging and pre-microsurgical era of neurosurgery. Imaging features can mimic those of many more common intraventricular lesions, such as choroid plexus tumors or cavernous malformations. In all reported symptomatic cases, resection was safely performed and led to a cure and symptom resolution. Here, the authors present a case of CEIVH in a pediatric patient, describe the operative techniques of resection, review the available literature, and discuss current understanding of the pathophysiology, making this the most comprehensive report on this disease entity to date. The case is a 14-year-old boy who presented with headaches and emesis. Computed tomography showed a hyperdense mass in the trigone of the right lateral ventricle. Magnetic resonance imaging showed a contrast-enhancing well-circumscribed mass. Right temporal craniotomy utilizing a posterior middle temporal gyrus transcortical approach was performed, and gross-total resection was achieved. Pathology revealed a CEIVH. The boy's postoperative course was uncomplicated, and he was discharged 2 days after surgery.

Gram-Positive Rods on a Cerebrospinal Fluid Gram Stain.

Cerebrospinal fluid (CSF) access device placement in the pediatric population presents challenges due to the development of infections following placement, access or revision, and/or shunt malfunctions. Here we report an unusual pediatric case of L. monocytogenes ventriculitis/VP shunt (VPS) infection and associated pseudocyst with an emphasis on the importance of VPS removal in clearing the infection due to biofilm formation.

Successful treatment of a child with a prolactin secreting macroadenoma with temozolomide.

Prolactinomas are a rare subset of brain tumors in pediatrics. We report a child with a prolactin secreting macroadenoma which was refractory to initial treatment with a dopamine antagonist. Given the location of her tumor she was ineligible for surgical resection. Temozolomide (200 mg/m2×5 days each month) was administered with a dramatic and prolonged response in tumor size, prolactin level, and symptoms, with no side effects from treatment. We demonstrate the benefit of temozolomide in the treatment of a pediatric patient with prolactinoma.

Posterior cervical spinal fusion in a 3-week-old infant with a severe subaxial distraction injury.

Unstable spinal injuries in the neonate pose particular challenges in the clinical and radiographic assessment as well as the surgical stabilization of the spine. In this report, the authors present the unfortunate case of a 3-week-old infant who suffered a severe subaxial cervical fracture dislocation with spinal cord injury that occurred as a result of nonaccidental trauma. Imaging demonstrated severe distraction at C5-6 and near-complete spinal cord transection resulting in quadri-paresis. Open surgical reduction was performed with noninstrumented posterior fusion augmented with split rib autograft and recombinant human bone morphogenetic protein-2. Postoperative imaging demonstrated progressive bony fusion at 2 months, and clinical examination findings progressed to a motor examination classification of ASIA C. At 2 years, the fusion mass is stable and cervical alignment is maintained. The patient remains flaccid in the bilateral lower extremities, but has movement with some dexterity in both hands. Follow-up MRI shows severe spinal cord injury with evidence of bilateral C-5 nerve root avulsions. This case represents the first report of spinal fusion in an infant of less than 1 month of age. Given the extreme young age of the patient, the diagnostic challenges as well as the mechanical and technical considerations of surgical fusion are discussed.

Preventability of Pediatric 30-Day Readmissions following Ventricular Shunt Surgery.

OBJECTIVE: To compare the preventability of 30-day pediatric ventricular shunt readmissions using clinical and administrative data review. STUDY DESIGN: We performed a retrospective chart review of one hundred forty-seven 30-day ventricular shunt readmissions at a tertiary pediatric center from May 2009-April 2013 under 2 scenarios: scenario 1 considered all ventricular shunt failures preventable; and scenario 2 considered shunt failures with excellent/good catheter positioning and no contributing deficiencies in care not preventable. Three physician reviewers independently assessed readmissions to determine their preventability and whether deficiencies in care existed that contributed to the readmission. We also evaluated the degree of interrater agreement in adjudicating readmission preventability. RESULTS: Only 42% of 30-day readmissions following ventricular shunt procedures were preventable when considering all shunt failures as preventable. When classifying shunts with excellent/good proximal catheter position as not preventable, 21% of ventricular shunt readmissions were deemed preventable. Interrater agreement on readmission preventability was high (kappa 0.88). Deficiencies in care existed in 29 readmissions (20%), the largest category being physician related, but not all deficiencies contributed to a readmission. CONCLUSIONS: Significant discrepancy exists in the preventability adjudication of ventricular shunt readmissions between administrative and chart review. Although using administrative data has determined that a majority of readmissions following pediatric ventricular shunt procedures are preventable, our review suggests a significantly lower degree of preventability.

Opioid overdose in a child: case report and discussion with emphasis on neurosurgical implications.

In environments in which opioids are increasingly abused for recreation, children are becoming more at risk for both accidental and nonaccidental intoxication. In toxic doses, opioids can cause potentially lethal acute leukoencephalopathy, which has a predilection for the cerebellum in young children. The authors present the case of a 2-year-old girl who suffered an accidental opioid overdose, presenting with altered mental status requiring cardiorespiratory support. She required emergency posterior fossa decompression, partial cerebellectomy, and CSF drainage due to cerebellar edema compressing the fourth ventricle. To the authors' knowledge, this is the first report of surgical decompression used to treat cerebellar edema associated with opioid overdose in a child.

A Novel Technique for Distal Shunt Revision: Retrospective Analysis of Guidewire-Assisted Distal Catheter Replacement.

BACKGROUND: Ventriculoperitoneal shunt revision is a common procedure. Disconnection and fracture of the distal catheter remain a common cause of ventriculoperitoneal shunt malfunction. OBJECTIVE: To describe a novel procedure for peritoneal replacement of the distal catheter by using a guidewire and a modified Seldinger technique (guidewire-assisted distal catheter replacement) and retrospectively evaluate the results of the surgical procedure. METHODS: Between September 2005 and December 2013, 68 patients were treated by a single surgeon (DMW) with distal catheter replacement using our technique. In brief, the previously placed distal catheter was exposed at its entry site into the abdomen. A soft guidewire with hydrophilic coating was inserted down the distal catheter into the peritoneum. The distal catheter was then removed over the guidewire, leaving the guidewire in place. A peel-away sheath and dilator were then inserted over the guidewire, and the dilator and guidewire were removed. The new distal catheter was then passed from the valve to the abdomen and was then fed through the peel-away sheath into the peritoneum. Charts were retrospectively reviewed for preoperative presentation, operative technique, and postoperative outcome. Records were specifically examined for any early or late complications. RESULTS: The mean patient age at surgery was 13 years. No immediate acute complications were noted. Of the 68 total patients, 45 patients had more than 6 months of follow-up. Of the 68 patients, 7 patients required another distal revision after guidewire-assisted distal catheter replacement. CONCLUSION: Distal shunt malfunction due to a mechanical failure is a common reason for shunt revision. We describe a technique for guidewire-assisted distal catheter replacement.

Clinical outcome of children with suspected shunt malfunction evaluated in the emergency department.

BACKGROUND: Patients with cerebrospinal fluid shunts frequently present to the emergency department (ED) with suspected shunt malfunction. The outcome of those patients who were discharged from ED when shunt malfunction was deemed unlikely has not been previously documented. OBJECTIVE: To demonstrate there is no increase in severity or likelihood of harm for patients who are discharged directly from the ED after adequate evaluation, as compared to patients who were selected for inpatient hospitalization. METHODS: The report screens 3080 ED visits between 2010 and 2013 made by patients with shunted hydrocephalus. ED visits preceded by another ED visit or neurosurgical procedures within 60 days were excluded. ED visits for reasons unrelated to shunt function were excluded, and 1943 visits met the inclusion criteria. Final dispositions from the ED included home (n = 1176), admission to neurosurgery service (n = 550), and admission to other services (n = 217). Subsequent events within 30 days, including ED visits and elective and nonelective shunt-related surgery, were reviewed. RESULTS: The clinical characteristics of the 3 groups were similar. Of patients discharged home from the ED, 19.0% returned to ED, and 4.5% required shunt-related surgeries. Of the patients admitted for observation, 18.7% returned to ED and 14.2% required shunt-related surgery. Of the patients admitted to other hospital services, 19.6% patients returned to the ED, with 2.0% requiring surgical intervention. There were no shunt-related mortalities in any of the 3 groups. CONCLUSION: Children with cerebrospinal fluid shunts are often evaluated in the ED. Discharge from the ED, when suspicion for shunt malfunction is low, is an appropriate practice.

Utility of surveillance imaging after minor blunt head trauma.

OBJECT: Nonoperative blunt head trauma is a common reason for admission in a pediatric hospital. Adverse events, such as growing skull fracture, are rare, and the incidence of such morbidity is not known. As a result, optimal follow-up care is not clear. METHODS: Patients admitted after minor blunt head trauma between May 1, 2009, and April 30, 2013, were identified at a single institution. Demographic, socioeconomic, and clinical characteristics were retrieved from administrative and outpatient databases. Clinical events within the 180-day period following discharge were reviewed and analyzed. These events included emergency department (ED) visits, need for surgical procedures, clinic visits, and surveillance imaging utilization. Associations among these clinical events and potential contributing factors were analyzed using appropriate statistical methods. RESULTS: There were 937 admissions for minor blunt head trauma in the 4-year period. Patients who required surgical interventions during the index admission were excluded. The average age of the admitted patients was 5.53 years, and the average length of stay was 1.7 days; 15.7% of patients were admitted for concussion symptoms with negative imaging findings, and 26.4% of patients suffered a skull fracture without intracranial injury. Patients presented with subdural, subarachnoid, or intraventricular hemorrhage in 11.6%, 9.19%, and 0.53% of cases, respectively. After discharge, 672 patients returned for at least 1 follow-up clinic visit (71.7%), and surveillance imaging was obtained at the time of the visit in 343 instances. The number of adverse events was small and consisted of 34 ED visits and 3 surgeries. Some of the ED visits could have been prevented with better discharge instructions, but none of the surgery was preventable. Furthermore, the pattern of postinjury surveillance imaging utilization correlated with physician identity but not with injury severity. Because the number of adverse events was small, surveillance imaging could not be shown to positively influence outcomes. CONCLUSIONS: Adverse events after nonoperative mild traumatic injury are rare. The routine use of postinjury surveillance imaging remains controversial, but these data suggest that such imaging does not effectively identify those who require operative intervention.

Return to system within 30 days of pediatric neurosurgery.

OBJECT: Quality assessment measures have not been well developed for pediatric neurosurgical patients. This report documents the authors' experience in extracting information from an administrative database to establish the rate of return to system within 30 days of pediatric neurosurgical procedures. METHODS: Demographic, socioeconomic, and clinical characteristics were prospectively collected in administrative, business, and operating room databases. The primary end point was an unexpected return to the hospital system within 30 days from the date of a pediatric neurosurgical procedure. Statistical methods were used to identify clinical and demographic factors associated with the primary end point. RESULTS: There were 1358 pediatric neurosurgical procedures performed in the Children's Healthcare of Atlanta operating rooms in 2012, with 37.4% of these surgeries being preceded by admissions through the emergency department. Medicare or Medicaid was the payor for 54.9% of surgeries, and 37.6% of surgeries were shunt related. There were 148 unexpected returns to the system within 30 days after surgery, and in 109 of these cases, the patient had a presenting complaint that was attributable to the index surgery (related returns). The most common complaints were headache, nausea, vomiting, or seizure after shunt revision or cranial procedures (n = 62). The next most common reason for re-presentation was for wound concerns (n = 30). Thirty-seven of the 109 related returns resulted in a reoperation. The monthly rate of related returns was 8.1% ± 2.5% over the 12-month study period. When using related returns as the dependent variable, the authors found that patients who underwent a shunt-related surgery were both more likely to unexpectedly return to the system (OR 1.86, p = 0.008) and to require surgery upon readmission (OR 3.28, p = 0.004). Because an extended hospitalization shortened the window of time for readmission after surgery, extended length of stay was protective against return to system within 30 days of surgery. Importantly, if related and unrelated returns were analyzed together as the dependent variable (n = 148), no independent clinical and demographic risk factor could be identified. CONCLUSIONS: Quality assessment measures need to be clearly and carefully defined, as the definition itself will impact the analytical results. Clinicians must play a leading role in the development of these measures to ensure their clinical meaningfulness.

Epstein-Barr virus associated smooth muscle tumors in post transplant pediatric patients two cases of rare locations, and review of the literature.

Epstein-Barr virus (EBV) may present few or no symptoms in immunocompetent individuals; however, in immunocompromised patients as in the case of AIDS and post-transplant patients, the virus occasionally stimulates neoplastic transformations. Epstein-Barr virus may play a role in the development of smooth muscle tumors (SMT). In the case of Epstein-Barr associated smooth muscle tumors (EBV+SMT), the virus is thought to be the leading factor to the tumorigenic pathway. We report two pediatric patients (6 and 13 years old) who underwent liver transplantation and developed EBV+SMT in the colon and orbit. These two cases represent rare locations for this kind of lesion.

The use of inside-outside screws for occipitocervical fusion in pediatric patients.

OBJECT: The authors describe the use of inside-outside occipital screws in 21 children with occipitocervical instability requiring occipitocervical fusion. METHODS: The ages of the patients were from 2 to 15 years, and patients presented with a variety of causes of occipitocervical instability, including congenital disorders, posttraumatic instability, idiopathic degeneration, and postoperative instability. Surgeries frequently included foramen magnum decompression, duraplasty, and laminectomy, but all patients required occipitocervical instrumentation and arthrodesis. Postoperative orthosis included the use of either a cervical collar or halo device. In all but one case, patients were followed postoperatively for at least 12 months. RESULTS: The mean age of patients was 9.93 years. Inside-outside screws were used in all reported cases. Rib autograft was used in all patients. In addition, demineralized bone matrix was used in 2 cases, and bone morphogenetic protein was used in 2 patients. Two patients required halo placement, and the other 19 were placed in cervical collars. The average time postoperative orthotics were used was 2.82 months. Arthrodesis was determined radiographically and was noted in all patients. No operative complications were noted; however, postoperative complications included 1 wound infection, 2 cases of hardware loosening, and the need for tracheostomy in 2 patients. CONCLUSIONS: Inside-outside screws were found to be a useful component of occipitocervical instrumentation in pediatric patients ranging from 2 to 15 years of age. Arthrodesis was demonstrated in all cases.

Transient enlargement of craniopharyngioma after radiation therapy: pattern of magnetic resonance imaging response following radiation.

Clinical experience suggests that craniopharyngiomas may temporarily increase in size after radiation therapy (RT). The study goal is to determine the incidence and natural history of this response in a cohort of patients managed at Children's Healthcare of Atlanta (CHOA) or Emory Healthcare (EHC). Between 08/1998 and 06/2009, 41 children and young adults were diagnosed with craniopharyngioma at CHOA and/or EHC. Of these, 21 received external-beam radiation and were included in our analysis. Serial magnetic resonance imaging (MRI) studies were evaluated volumetrically to assess response to RT. Median age at diagnosis was 8.2 years (range 3.2-23.5 years). Median radiation dose was 54.0 Gy using standard fractionation (1.8-2.0 Gy/day). With median follow-up of 41.3 months (range 7.2-121.8 months), actuarial local control and overall survival rates at 5 years were 78.7 % and 100 %, respectively. Of subjects, 52.4 % of subjects (11 of 21) were noted on serial MRI evaluation to have tumor enlargement (mostly cystic component) after radiation before eventual shrinkage without further intervention. For tumors that expanded, the median volume increase was 33.9 % (range 15.6-224.4 %). Median time to maximal tumor/cyst expansion was 1.5 months (range 1.0-5.0 months). Finally, nearly all patients (20 of 21) showed a measurable objective response to therapy by MRI regardless of ultimate disease control. Median time to maximal response post-radiation, as defined by MRI, was 9.5 months (range 3.5-39.9 months). In summary, RT is effective for managing craniopharyngioma. However, despite good ultimate responses, approximately 50 % of the patients show tumor/cyst expansion on MRI over the first few months post-radiation. Caution should be taken not to subject these patients to "salvage surgery" or cyst aspiration during this early time unless there are other overriding surgical indications. Understanding the natural history of this phenomenon could potentially help guide the management of these craniopharyngioma patients.

Seventeen-year-old adolescent with pituitary abscess.

Pituitary abscess is a rare but potentially life-threatening infectious process. Diagnosis is challenging as symptoms are non-specific and signs of infection may be absent. We report the case of a previously healthy 17-year-old male who presented with worsening headaches, polyuria, polydipsia and no clinical signs of infection. On evaluation, he was found to have hypopituitarism with diabetes insipidus, hypothyroidism and adrenal insufficiency. An imaging study revealed a pituitary mass. He underwent transsphenoidal biopsy to rule out tumor. The abscess was drained transsphenoidally and he was treated with parental antibiotics. Magnetic resonance imaging one year later revealed a normal pituitary without any evidence of abscess or mass. He continues to require thyroid, adrenal and anti-diuretic hormone replacements. As with any pituitary lesion, prompt complete hypothalamic pituitary evaluation is essential to avoid potentially life-threatening consequences.

Developmental outcomes of Down syndrome and Dandy-Walker malformation.

Dandy-Walker syndrome (DWS), or Dandy-Walker complex, is a congenital brain malformation of the posterior fossa, typically resulting in developmental delay and cognitive disability. The co-occurrence of Down syndrome (DS) and DWS is relatively uncommon; thus, its impact on developmental outcomes has not been fully elucidated. Herein, we report a case of a 37-month-old child with DS and DWS, who is functioning at the following age-equivalent: gross motor at a 9-mo level, fine motor 6 mo, expressive language 14 mo, receptive language 9 mo. As such, it is important to determine how the DWS influences developmental outcomes, and appreciate the importance of early interventional therapy.

Postoperative nonconvulsive encephalopathic status: identification of a syndrome responsible for delayed progressive deterioration of neurological status after skull base surgery. Clinical article.

OBJECT: Over a 10-year period, the authors have observed a rare but recurring syndrome manifested by a delayed, postoperative, progressive decline in the level of consciousness to deep coma that is time-limited to several days with abrupt awakening. Extensive evaluation and workup demonstrated an abnormality on continuous electroencephalographic monitoring that implied nonconvulsive status epilepticus after the exclusion of structural, perfusion, infectious, or metabolic causes. This state has been very refractory to treatment with antiepileptic medication. In this article, the authors raise the awareness of this syndrome and its diagnosis, management, and outcome. METHODS: The authors reviewed the medical records of a cohort of 7 patients who exemplified this syndrome who were treated during the last 5 years. RESULTS: All 7 patients were women with a mean (+/- SD) age of 55 +/- 15 years. The mean duration of surgery was 8.9 +/- 1.8 hours. All patients had a stereotypical course of delayed progressive decline in their level of consciousness after surgery (average 3.3 +/- 4.3 days) leading to deep coma. The unconscious state was time-limited, lasting on average 17.3 +/- 13.7 days. Continuous electroencephalographic monitoring demonstrated a generalized abnormality with periodic discharges and abundant slow delta activity. A rather abrupt awakening occurred a few days after cessation of electrographic seizure activity. Structural, vascular, infectious, or metabolic causes were excluded based on an extensive workup. CONCLUSIONS: In this study, the authors delineate and raise the awareness of an unusual syndrome. Recognition of this syndrome is important as a cause for delayed coma after surgery. The authors stress the need for respiratory, hemodynamic, and nutritional support for these patients until recovery. The origin of this syndrome remains enigmatic and is likely to be multifactorial with a prominent pharmacological role related to anesthetic agent or medication in a setting of craniotomy that is associated with alteration of the blood-brain barrier.