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Neonatal posthemorrhagic hydrocephalus from prematurity: pathophysiology and current treatment concepts

A review

Shenandoah Robinson

Object

Preterm infants are at risk for perinatal complications, including germinal matrix–intraventricular hemorrhage (IVH) and subsequent posthemorrhagic hydrocephalus (PHH). This review summarizes the current understanding of the epidemiology, pathophysiology, management, and outcomes of IVH and PHH in preterm infants.

Methods

The MEDLINE database was systematically searched using terms related to IVH, PHH, and relevant neurosurgical procedures to identify publications in the English medical literature. To complement information from the systematic search, pertinent articles were selected from the references of articles identified in the initial search.

Results

This review summarizes the current knowledge regarding the epidemiology and pathophysiology of IVH and PHH, primarily using evidence-based studies. Advances in obstetrics and neonatology over the past few decades have contributed to a marked improvement in the survival of preterm infants, and neurological morbidity is also starting to decrease. The incidence of IVH is declining, and the incidence of PHH will likely follow. Currently, approximately 15% of preterm infants who suffer severe IVH will require permanent CSF diversion. The clinical presentation and surgical management of symptomatic PHH with temporary ventricular reservoirs (ventricular access devices) and ventriculosubgaleal shunts and permanent ventriculoperitoneal shunts are discussed. Preterm infants who develop PHH that requires surgical treatment remain at high risk for other related neurological problems, including cerebral palsy, epilepsy, and cognitive and behavioral delay. This review highlights numerous opportunities for further study to improve the care of these children.

Conclusions

A better grasp of the pathophysiology of IVH is beginning to impact the incidence of IVH and PHH. Neonatologists conduct rigorous Class I and II studies to advance the outcomes of preterm infants. The need for well-designed multicenter trials is essential because of the declining incidence of IVH and PHH, variations in referral patterns, and neonatal ICU and neurosurgical management. Well-designed multicenter trials will eventually produce evidence to enable neurosurgeons to provide their smallest, most vulnerable patients with the best practices to minimize perioperative complications and permanent shunt dependence, and most importantly, optimize long-term neurodevelopmental outcomes.

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Diagnosis and management of deformational plagiocephaly

A review

Shenandoah Robinson and Mark Proctor

Object

The increase in the prevalence of nonsynostotic occipital deformational plagiocephaly in infants, which resulted from the American Academy of Pediatrics' 1992 recommendation to have healthy infants sleep supine, has been accompanied by significant controversy in diagnosis and management. The controversy was exacerbated by the 1998 FDA classification of cranial orthotic devices as Class II devices requiring premarket notification, and the subsequent increase in treatment-associated costs.

Methods

Two independent reviews of the literature were conducted to clarify the objective evidence available within the context of pediatric craniofacial knowledge.

Results

. Although deformational plagiocephaly is not a life-threatening problem, it is a source of disfigurement for children that may be detrimental to their well-being. Current methods for quantifying the degree of disfigurement have limited interrater reliability, and no prospective randomized controlled trials comparing the efficacy of cranial orthoses to repositioning and physical therapy protocols have been published. Despite this lack of Class I evidence, cranial orthoses are routinely and effectively used to treat persistent severe deformational plagiocephaly. The need for the current FDA regulations has not been supported by clinical experience and reported complications.

Conclusions

This review resulted in the following recommendations: 1) more parental education is needed to minimize the development and progression of deformational plagiocephaly; 2) mild deformity can be treated with repositioning and physical therapy protocols; and 3) severe deformity is likely to be corrected more quickly and effectively with cranial orthosis (when used during the appropriate period of infancy) than with repositioning and physical therapy. The available data do not support the need for FDA classification for cranial orthoses as Class II devices requiring premarket notification. Removal of the regulations, which centralized production of the orthoses to larger companies and markedly increased charges, will probably eliminate much of the controversy and parental anxiety generated by marketing strategies.

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Editorial: The best and the brightest

Shenandoah Robinson

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Exogenous Neonatal Erythropoietin Mitigates Brain Damage After a Combination of Prenatal Hypoxic-Ischemia and Lipopolysaccharide Inflammation in Rats

Shenandoah Robinson and Qing Li

Introduction

Many infants born very preterm who suffer brain damage most likely experienced a combined insult from intrauterine infection and placental insufficiency. Damage is thought to be synergistic rather than additive but the mechanisms of combined injury remain elusive. A combination of lipopolysaccharide-induced inflammation and hypoxia-ischemia has been used in rats to model the dual insult that occurs in human infants prenatally. Erythropoietin, a pleiotrophic cytokine that is essential for central nervous system development, ameliorates brain injury after isolated hypoxic-ischemic or inflammatory insults through different intracellular signaling pathways. We hypothesized that exogenous neonatal EPO administration would lessen the damage of a combined prenatal insult in rats.

Methods

On embryonic Day 18 fetal rats experienced 60 minutes of transient uterine artery occlusion with or without intracervical LPS administration with sham controls receiving surgery but no occlusion and saline for LPS. Survival was recorded and histological biochemical and functional assays were performed. Means were compared with ANOVA with Tukey HSD post hoc analysis.

Results

After a combined insult of HI and 0.15-mg/kg LPS on E18 the survival of pups by postnatal Day 1 (P1) decreased from 77% with HI alone to 22% for LPS plus HI. When exogenous systemic EPO was administered P1–P3 survival to P9 improved markedly from 40% (2 of 5) for saline-treated insult pups to 100% (6 of 6) for EPO-treated. Initial histological analyses show EPO decreases the number of brain activated caspase 3 and activated microglia by P9. Additional analyses will be presented.

Conclusion

As at least 60% of placentas from infants born pre-term show evidence of chorioamnionitis, assessment of the impact of exogenous EPO on a model of a combination injury is essential prior to proceeding with a clinical trial. Initial results indicate neonatal exogenous EPO mitigates damage from the combined insult.

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Editorial: Intraventricular hemorrhage and posthemorrhagic hydrocephalus

John R. W. Kestle

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Expanding the role of surgery in intractable extratemporal pediatric epilepsy

Monisha Goyal and Shenandoah Robinson

The aim of epilepsy surgery in children is not only to control seizures but also to curtail future adverse neurological sequelae and improve quality of life. If presurgical evaluation demonstrates discordant or multifocal disease, intracranial surgery may be denied as a viable treatment option. When surgical therapy is offered not just as curative but also as palliative therapy, many children not considered optimal surgical candidates may benefit. From a consecutive series of 57 cases involving children who underwent intracranial epilepsy surgery at Rainbow Babies and Children's Hospital during 2001–2005, the authors present in detail 3 cases involving children who benefited significantly from such an aggressive approach. Marked improvement occurred not only in seizure control, but also in developmental and social functioning.

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Cowden disease and Lhermitte–Duclos disease: an update

Case report and review of the literature

Shenandoah Robinson and Alan R. Cohen

Object

Cowden disease is a rare autosomal-dominant phacomatosis and cancer syndrome that is associated with Lhermitte–Duclos disease (LDD), also called dysplastic cerebellar gangliocytoma.

Methods

In this review the authors summarize the additions to the literature during the past 5 years, with emphasis on new case reports and advances in imaging and molecular biology. Adult-onset LDD is now considered pathognomonic for Cowden disease.

Approximately 220 cases of LDD have been reported. Magnetic resonance imaging in patients with LDD is often diagnostic, and imaging studies have facilitated accurate diagnosis and contributed to the improved outcome in affected patients. Cowden disease and other rare, related disorders, such as Bannayan-Riley-Ruvalcaba, Proteus, and Proteus-like syndromes, are often caused by mutations of the PTEN gene.

Conclusions

Because of the high incidence of systemic cancer in patients with Cowden disease, it is important for neurosurgeons to recognize the association between this disease and LDD and to refer affected patients for appropriate cancer screenings and interventions.

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Postnatal erythropoietin treatment mitigates neural cell loss after systemic prenatal hypoxic-ischemic injury

Laboratory investigation

Marcus Mazur, Robert H. Miller, and Shenandoah Robinson

Object

Brain injury from preterm birth predisposes children to cerebral palsy, epilepsy, cognitive delay, and behavioral abnormalities. The CNS injury often begins before the early birth, which hinders diagnosis and concurrent treatment. Safe, effective postnatal interventions are urgently needed to minimize these chronic neurological deficits. Erythropoietin (EPO) is a pleiotropic neuroprotective cytokine, but the biological basis of its efficacy in the damaged developing brain remains unclear. Coordinated expression of EPO ligand and receptor expression occurs during CNS development to promote neural cell survival. The authors propose that prenatal third trimester global hypoxiaischemia disrupts the developmentally regulated expression of neural cell EPO signaling, and predisposes neural cells to death. Furthermore, the authors suggest that neonatal exogenous recombinant human EPO (rhEPO) administration can restore the mismatch of EPO ligand and receptor levels, and enhance neural cell survival.

Methods

Transient systemic hypoxia-ischemia (TSHI) on embryonic Day 18 in rats mimics human early-thirdtrimester placental insufficiency. This model was used to test the authors' hypothesis using a novel clinically relevant paradigm of prenatal injury on embryonic Day 18, neonatal systemic rhEPO administration initiated 4 days after injury on postnatal Day 1, and histological, biochemical, and functional analyses in neonatal, juvenile, and adult rats.

Results

The results showed that prenatal TSHI upregulates brain EPO receptors, but not EPO ligand. Sustained EPO receptor upregulation was pronounced on oligodendroglial lineage cells and neurons, neural cell populations particularly prone to loss from CNS injury due to preterm birth. Postnatal rhEPO administration after prenatal TSHI minimized histological damage and rescued oligodendrocytes and γ-aminobutyric acidergic interneurons. Myelin basic protein expression in adult rats after insult was reduced compared with sham controls, but could be restored to near normal levels by neonatal rhEPO treatment. Erythropoietin-treated TSHI rats performed significantly better than their saline-treated peers as adults in motor skills tests, and showed significant seizure threshold restoration using a pentylenetetrazole increasing-dose paradigm.

Conclusions

These data demonstrate that neonatal rhEPO administration in a novel clinically relevant paradigm initiated 4 days after a global prenatal hypoxic-ischemic insult in rats rescues neural cells, and induces lasting histological and functional improvement in adult rats.

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Neurological injuries from car surfing

Clinical article

Arthur Wang, Alan R. Cohen, and Shenandoah Robinson

Object

Trauma secondary to car surfing is a unique mechanism of head and spinal injury in children and adolescents. In this study, the authors present their experience with neurological injuries resulting from car surfing and describe the growing national trend of car-surfing injuries and the increasing portrayal of this activity in the media.

Methods

A retrospective study of the Rainbow Babies and Children's Hospital trauma database was conducted to identify all cases of neurological injuries secondary to car surfing. Between January 1995 and December 2008, 7 patients ≤ 16 years of age, including 6 boys and 1 girl, were identified. The charts of these patients were reviewed. National data on car-surfing fatalities over the same time period in children and adolescents 10–20 years of age were obtained from the National Highway Traffic Safety Administration, and these data were analyzed for national trends in car-surfing fatalities.

Results

All 7 children with injuries due to car surfing suffered traumatic head injuries. Three patients fell from the back of the moving vehicle, 2 fell from the hood, 1 fell from the side of the vehicle, and 1 patient sustained head injuries after intentionally jumping off the moving vehicle. All 7 children suffered intracranial bleeding, and 4 had associated skull fractures. One patient underwent craniotomy to evacuate an acute subdural hematoma. The other 6 patients required nonoperative treatment. Four patients had permanent neurological problems. National statistics have shown a steady rise in car-surfing fatality rates since 2000, especially in California, Florida, and Texas.

Conclusions

Car surfing is an unusual but serious mechanism of neurological injury in children and adolescents. Despite its dangers, car surfing is becoming a more common pastime in the pediatric population. National statistics have shown a steady rise in car-surfing fatalities since 2000. This national rise in fatalities chronologically overlaps with the introduction of media depictions of the activity since 1995. Increased awareness of the dangers of car surfing may help prevent future head injuries.

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The “swing-ding”: a golf-related head injury in children

Clinical article

Arthur Wang, Alan R. Cohen, and Shenandoah Robinson

Object

In recent years there has been an increased incidence of golf-associated head injuries in children and adolescents. At the authors' institution, they have identified a unique pattern of head injury associated with a swinging golf club. In this study, the authors highlight the mechanism of this injury and report their experience treating it.

Methods

The authors reviewed the database of Rainbow Babies and Children's Hospital Trauma Center and performed a retrospective analysis of golf injuries recorded over a 10-year period (January 2000–April 2010). They identified 13 children (9 boys and 4 girls) who sustained head injuries in golfing accidents. All patients were 10 years of age or younger. The medical charts were reviewed and follow-up interviews were conducted to better delineate the details of the injuries.

Results

Injuries included 13 depressed skull fractures, 7 epidural hematomas, and 1 cerebral contusion. All 13 patients sustained their injuries after being struck in the head by a golf club. Seven sustained injuries on the follow-through of the initial swing and 3 sustained injuries on the backswing. All but one patient required neurosurgical intervention. Five patients developed neurological sequelae. None of the children had prior experience with golf equipment. All but one injury occurred in the child's own backyard. There was no direct supervision by an adult in any of the cases.

Conclusions

Golfing can lead to serious head injuries in children. The authors noticed a unique pattern of golf-related head injuries, previously not described, that they have termed the “swing-ding.” This golf club–inflicted injury occurs when a child stands too close to a swinging golfer and is struck in the head, subsequently sustaining a comminuted depressed skull fracture in the frontal or temporal region, with or without further intracranial injury. The study suggests that a lack of adult supervision, minimal previous golf experience, and proximity of the child to the swinging golfer are all implicated in this head injury pattern.