Mark Hamilton, John Peter Gruen, and Mark G. Luciano
Intraoperative magnetic resonance imaging
Mark M. Souweidane
Kelly J. Bullivant, Alim P. Mitha, and Mark G. Hamilton
The PS Medical Strata valve is a programmable shunt valve used in the treatment of hydrocephalus that allows for noninvasive changes in the pressure setting using a magnet. The Strata valve is sensitive to magnetic fields, and reprogramming is frequently necessary after MR imaging. A known but rare complication of the Strata valve is that the rotor can become locked, causing shunt malfunction. This complication can only occur in a first generation Strata valve.
Mark G. Hamilton and S. Terence Myles
✓ Injury to the spinal column and spinal cord occurs relatively infrequently in the pediatric population. The authors present a unique review of 61 pediatric deaths associated with spinal injury. This group represented 28% of the total pediatric spine-injured population and 45% of the total pediatric spinal cord-injured group studied. The ratio of pediatric to adult spinal injury mortality was 2.5:1. Of the 61 children, 54 (89%) died at the accident scene. Thirty patients underwent a complete autopsy, 19 of whom had an Abbreviated Injury Scale Grade 6 injury (maximum score, untreatable). Spinal cord injury was found to be the cause of death in only eight children and was associated with injury to the high cervical cord and cardiorespiratory arrest. These children typically sustained severe multiple trauma. In this population, there appears to be little room for improved outcome through changes in treatment strategy.
Mark G. Hamilton and S. Terence Mylks
✓ Injury to the spinal column and spinal cord occurs relatively infrequently in the pediatric population. A review of 174 pediatric patients is presented, representing 5.4% of all patients admitted with spinal injury, Spinal cord injury was present in 45% of patients. A distinct injury profile, explained by anatomical and biomechanical features, distinguishes the young patient with an immature spine from older adolescents with a more mature, adult-like spine. The younger patients, while less likely to have spinal injury, had a higher incidence of neurological injury, in addition to a higher frequency of both spinal cord injury without radiological abnormality and upper cervical cord injury. In addition, younger patients with spinal cord injury and no radiological abnormality were more likely to have complete or severe cord injury. Prognosis was determined by the severity of spinal cord injury. Patients with complete cord injuries showed little improvement, while patients with incomplete injuries generally fared much better, with 74% showing significant improvement and 59% experiencing a complete recovery of neurological functions. There were six deaths, but none was attributed solely to spinal injury. The authors conclude that outcome is quite good after pediatric spinal cord injury that does not produce a physiologically complete cord deficit.
Michael B. Keough, Albert M. Isaacs, Geberth Urbaneja, Jarred Dronyk, Andrew P. Lapointe, and Mark G. Hamilton
Acute low-pressure hydrocephalus (ALPH) is characterized by clinical manifestations of an apparent raised intracranial pressure (ICP) and ventriculomegaly despite measured ICP that is below the expected range (i.e., typically ≤ 5 cm H2O). ALPH is often refractory to standard hydrocephalus intervention protocols and the ICP paradox commonly leads to delayed diagnosis. The aim of this study was to characterize ALPH and develop an algorithm to facilitate diagnosis and management for patients with ALPH.
EMBASE, MEDLINE, and Google Scholar databases were searched for ALPH cases from its first description in 1994 until 2019. Cases that met inclusion criteria were pooled with cases managed at the authors’ institution. Patient characteristics, presenting signs/symptoms, precipitating factors, temporizing interventions, definitive treatment, and patient outcomes were recorded.
There were 195 patients identified, with 42 local and 153 from the literature review (53 pediatric patients and 142 adults). Decreased level of consciousness was the predominant clinical sign. The most common etiologies of hydrocephalus were neoplasm and hemorrhage. While the majority of ALPH occurred spontaneously, 39% of pediatric patients had previously undergone a lumbar puncture. Prior to ALPH diagnosis, 92% of pediatric and 39% of adult patients had a ventricular shunt in situ. The most common temporizing intervention was subatmospheric CSF drainage. The majority of patients underwent a shunt insertion/revision or endoscopic third ventriculostomy as definitive ALPH treatment. Although the mortality rate was 11%, 83% of pediatric and 49% of adult patients returned to their pre-ALPH neurological functional status after definitive treatment. Outcomes were related to both the severity of the underlying neurosurgical disease causing the hydrocephalus and the efficacy of ALPH treatment.
ALPH is an underrecognized variant phenotype of hydrocephalus that is associated with multiple etiologies and can be challenging to treat as it frequently does not initially respond to standard strategies of CSF shunting. With early recognition, ALPH can be effectively managed. A management algorithm is provided as a guide for this purpose.
Albert M. Isaacs, Yarema B. Bezchlibnyk, Heather Yong, Dilip Koshy, Geberth Urbaneja, Walter J. Hader, and Mark G. Hamilton
The efficacy of endoscopic third ventriculostomy (ETV) for the treatment of pediatric hydrocephalus has been extensively reported in the literature. However, ETV-related long-term outcome data are lacking for the adult hydrocephalus population. The objective of the present study was to assess the role of ETV as a primary or secondary treatment for hydrocephalus in adults.
The authors performed a retrospective chart review of all adult patients (age ≥ 18 years) with symptomatic hydrocephalus treated with ETV in Calgary, Canada, over a span of 20 years (1994–2014). Patients were dichotomized into a primary or secondary ETV cohort based on whether ETV was the initial treatment modality for the hydrocephalus or if other CSF diversion procedures had been previously attempted respectively. Primary outcomes were subjective patient-reported clinical improvement within 12 weeks of surgery and the need for any CSF diversion procedures after the initial ETV during the span of the study. Categorical and actuarial data analysis was done to compare the outcomes of the primary versus secondary ETV cohorts.
A total of 163 adult patients with symptomatic hydrocephalus treated with ETV were identified and followed over an average of 98.6 months (range 0.1–230.4 months). All patients presented with signs of intracranial hypertension or other neurological symptoms. The primary ETV group consisted of 112 patients, and the secondary ETV consisted of 51 patients who presented with failed ventriculoperitoneal (VP) shunts. After the initial ETV procedure, clinical improvement was reported more frequently by patients in the primary cohort (87%) relative to those in the secondary ETV cohort (65%, p = 0.001). Additionally, patients in the primary ETV group required fewer reoperations (p < 0.001), with cumulative ETV survival time favoring this primary ETV cohort over the course of the follow-up period (p < 0.001). Fifteen patients required repeat ETV, with all but one experiencing successful relief of symptoms. Patients in the secondary ETV cohort also had a higher incidence of complications, with one occurring in 8 patients (16%) compared with 2 in the primary ETV group (2%; p = 0.010), although most complications were minor.
ETV is an effective long-term treatment for selected adult patients with hydrocephalus. The overall ETV success rate when it was the primary treatment modality for adult hydrocephalus was approximately 87%, and 99% of patients experience symptomatic improvement after 2 ETVs. Patients in whom VP shunt surgery fails prior to an ETV have a 22% relative risk of ETV failure and an almost eightfold complication rate, although mostly minor, when compared with patients who undergo a primary ETV. Most ETV failures occur within the first 7 months of surgery in patients treated with primary ETV, but the time to failure is more prolonged in patients who present with failed previous shunts.
Mark G. Hamilton, Ian Parney, Odette A. Harris, Eric A. Schmidt, and Howard A. Riina
Michael A. Williams, Tessa van der Willigen, Patience H. White, Cathy C. Cartwright, David L. Wood, and Mark G. Hamilton
The health care needs of children with hydrocephalus continue beyond childhood and adolescence; however, pediatric hospitals and pediatric neurosurgeons are often unable to provide them care after they become adults. Each year in the US, an estimated 5000–6000 adolescents and young adults (collectively, youth) with hydrocephalus must move to the adult health care system, a process known as health care transition (HCT), for which many are not prepared. Many discover that they cannot find neurosurgeons to care for them. A significant gap in health care services exists for young adults with hydrocephalus. To address these issues, the Hydrocephalus Association convened a Transition Summit in Seattle, Washington, February 17–18, 2017.
The Hydrocephalus Association surveyed youth and families in focus groups to identify common concerns with HCT that were used to identify topics for the summit. Seven plenary sessions consisted of formal presentations. Four breakout groups identified key priorities and recommended actions regarding HCT models and practices, to prepare and engage patients, educate health care professionals, and address payment issues. The breakout group results were discussed by all participants to generate consensus recommendations.
Barriers to effective HCT included difficulty finding adult neurosurgeons to accept young adults with hydrocephalus into their practices; unfamiliarity of neurologists, primary care providers, and other health care professionals with the principles of care for patients with hydrocephalus; insufficient infrastructure and processes to provide effective HCT for youth, and longitudinal care for adults with hydrocephalus; and inadequate compensation for health care services.
Best practices were identified, including the National Center for Health Care Transition Improvement’s “Six Core Elements of Health Care Transition 2.0”; development of hydrocephalus-specific transition programs or incorporation of hydrocephalus into existing general HCT programs; and development of specialty centers for longitudinal care of adults with hydrocephalus.
The lack of formal HCT and longitudinal care for young adults with hydrocephalus is a significant health care services problem in the US and Canada that professional societies in neurosurgery and neurology must address. Consensus recommendations of the Hydrocephalus Association Transition Summit address 1) actions by hospitals, health systems, and practices to meet local community needs to improve processes and infrastructure for HCT services and longitudinal care; and 2) actions by professional societies in adult and pediatric neurosurgery and neurology to meet national needs to improve processes and infrastructure for HCT services; to improve training in medical and surgical management of hydrocephalus and in HCT and longitudinal care; and to demonstrate the outcomes and effectiveness of HCT and longitudinal care by promoting research funding.