Readmission and reoperation for hydrocephalus: a population-based analysis across the spectrum of age

J Neurosurg 134:1210–1217, 2021

Hydrocephalus is a common, chronic illness that generally requires lifelong, longitudinal, neurosurgical care. Except at select research centers, surgical outcomes in the United States have not been well documented. Comparative outcomes across the spectrum of age have not been studied.

METHODS Data were derived for the year 2015 from the Nationwide Readmissions Database, a product of the Healthcare Cost and Utilization Project of the Agency for Healthcare Research and Quality. In this data set patients are assigned state-specific codes that link repeated discharges through the calendar year. Discharges with diagnostic codes for hydrocephalus were extracted, and for each patient the first discharge defined the index admission. The study event was readmission. Observations were censored at the end of the year. In a similar fashion the first definitive surgical procedure for hydrocephalus was defined as the index operation, and the study event was reoperation for hydrocephalus or complications. Survival without readmission and survival without reoperation were analyzed using life tables and Kaplan- Meier plots.

RESULTS Readmission rates at 30 days ranged between 15.6% and 16.8% by age group without significant differences. After the index admission the first readmission alone generated estimated hospital charges of $2.25 billion nationwide. Reoperation rates at 30 days were 34.9% for infants, 39.2% for children, 47.4% for adults, and 32.4% for elders. These differences were highly significant. More than 3 times as many index operations were captured for adults and elders as for infants and children. Estimated 1-year reoperation rates were 74.2% for shunt insertion, 63.9% for shunt revision, but only 34.5% for endoscopic third ventriculostomy. Univariate associations with survival without readmission and survival without reoperation are presented.

CONCLUSIONS In the United States hydrocephalus is predominantly a disease of adults. Surgical outcomes in this population-based study were substantially worse than outcomes reported from research centers. High reoperation rates after CSF shunt surgery accounted for this discrepancy.

Pediatric Clival Chordoma: A Curable Disease that Conforms to Collins’ Law

Neurosurgery 82:652–660, 2018

Skull base chordomas in children are extremely rare. Their course, management, and outcome have not been defined.

OBJECTIVE: To describe the preeminent clinical and radiological features in a series of pediatric patients with skull base chordomas and analyze the outcome of a cohort who underwent uniform treatment. We emphasize predictors of overall survival and progression-free survival, which aligns with Collins’ law for embryonal tumors.

METHODS: Thirty-one patients with a mean age of 10.7 yr (range 0.8-22) harboring skull base chordomas were evaluated. We retrospectively analyzed the outcomes and prognostic factors for 18 patients treated by the senior author, with uniform management of surgery with the aim of gross total resection and adjuvant proton-beam radiotherapy. Mean follow-up was 119.2 mo (range 8-263).

RESULTS: Abducens nerve palsy was the most common presenting symptom. Imaging disclosed large tumors that often involve multiple anatomical compartments. Patients undergoing gross total resection had significantly increased progression-free survival (P = .02) and overall survival (P = .05) compared with those having subtotal resection. Those who lived through the period of risk for recurrence without disease progression had a higher probability of living entirely free of progression (P = .03; odds ratio = 16.0). Age, sex, and histopathological variant did not yield statistical significance in survival.

CONCLUSION: Long-term overall and progression-free survival in children harboring skull base chordomas can be achieved with gross surgical resection and proton-beam radiotherapy, despite an advanced stage at presentation. Collins’ law does apply to pediatric skull base chordomas, and children with this disease have a high hope for cure.

Lumbar Disk Herniation in Children and Adolescents

Lumbar Disk Herniation in Children and Adolescents

Neurosurgery 77:954–959, 2015

Lumbar disk herniation in adults is thought to be caused by repetitive overloading and age-rated degenerative changes. However, these causes are absent in children and adolescent patients. We assume that structural malformations in the lumbar spine could predispose intervertebral disks to early degeneration and hence need to be surgically fused. This issue has never been raised before.

OBJECTIVE: To investigate the assumption that structural malformations in the lumbar spine could predispose intervertebral disks to early degeneration and hence need to be surgically fused.

METHODS: Lumbar spine configurations, including the height of the intercrestal line, the length of L5 transverse processes, and the presence of transitional vertebrae, were recorded from anteroposterior radiographs taken from 63 consecutive pediatric patients with lumbar disk herniation admitted to our hospital over a period of 8 years. Each configuration was compared in relation to the level of disk herniation. Diskectomy alone was performed in 36 cases; arthrodesis was added in the remaining 27 cases. Patients’ back and leg pain visual analog scale scores and frequency and their Oswestry Disability Index scores were recorded before surgery and at follow-up. The results were compared for assessment of outcome.

RESULTS: Patients with high intercrestal lines and long L5 transverse processes had a significantly higher incidence of L4/5 disk herniation, whereas low intercrestal line and lumbarization were associated with L5/S1 disk herniation. Patients’ visual analog scale scores, pain frequency, and Oswestry Disability Index score all improved significantly after surgery, but there was no significant difference with or without arthrodesis.

CONCLUSION: Pediatric lumbar disk herniation is significantly associated with structural malformations of the lumbar spine, but arthrodesis does not improve the clinical outcome.

Pediatric Epilepsy Surgery: Long-term 5-Year Seizure Remission and Medication Use

Neurosurgery 71:985–993, 2012

It is unclear whether long-term seizure outcomes in children are similar to those in adult epilepsy surgery patients.

OBJECTIVE: To determine 5-year outcomes and antiepilepsy drug (AED) use in pediatric epilepsy surgery patients from a single institution.

METHODS: The cohort consisted of children younger than 18 years of age whose 5-year outcome data would have been available by 2010. Comparisons were made between patients with and without 5-year data (n = 338), patients with 5-year data for seizure outcome (n = 257), and seizure-free patients on and off AEDs (n = 137).

RESULTS: Five-year data were available from 76% of patients. More seizure-free patients with focal resections for hippocampal sclerosis and tumors lacked 5-year data compared with other cases. Of those with 5-year data, 53% were continuously seizure free, 18% had late seizure recurrence, 3% became seizure free after initial failure, and 25% were never seizure free. Patients were more likely to be continuously seizure free if their surgery was performed during the period 2001 to 2005 (68%) compared with surgery performed from 1996 to 2000 (61%), 1991 to 1995 (36%), and 1986 to 1990 (46%). More patients had 1 or fewer seizures per month in the late seizure recurrence (47%) compared with the not seizure-free group (20%). Four late deaths occurred in the not seizure-free group compared with 1 in the seizure-free group. Of patients who were continuously seizure free, 55% were not taking AEDs, and more cortical dysplasia patients (74%) had stopped taking AEDs compared with hemimegalencephaly patients (18%).

CONCLUSION: In children, 5-year outcomes improved over 20 years of clinical experience. Our results are similar to those of adult epilepsy surgery patients despite mostly extratemporal and hemispheric operations for diverse developmental etiologies.

Neuropathological and Neuroradiological Spectrum of Pediatric Malignant Gliomas: Correlation With Outcome

Neurosurgery 69:215–224, 2011 DOI: 10.1227/NEU.0b013e3182134340

The diagnostic accuracy and reproducibility for glioma histological diagnosis are suboptimal.

OBJECTIVE: To characterize radiological and histological features in pediatric malignant gliomas and to determine whether they had an impact on survival.

METHODS: We retrospectively reviewed a series of 96 pediatric malignant gliomas. All histological samples were blindly and independently reviewed and classified according to World Health Organization 2007 and Sainte-Anne classifications. Radiological features were reviewed independently. Statistical analyses were performed to investigate the relationship between clinical, radiological, and histological features and survival.

RESULTS: Cohort median age was 7.8 years; median follow-up was 4.8 years. Tumors involved cerebral hemispheres or basal ganglia in 82% of cases and brainstem in the remaining 18%. After histopathological review, low-grade gliomas and nonglial tumors were excluded (n = 27). The World Health Organization classification was not able to demonstrate differences between groups and patients survival. The Sainte-Anne classification identified a 3-year survival rate difference between the histological subgroups (oligodendroglioma A, oligodendroglioma B, malignant glioneuronal tumors, and glioblastomas; P = .02). The malignant glioneuronal tumor was the only glioma subtype with specific radiological features. Tumor location was significantly associated with 3-year survival rate (P = .005). Meningeal attachment was the only radiological criteria associated with longer survival (P = .02).

CONCLUSION: The Sainte-Anne classification was better able to distinguish pediatric malignant gliomas in terms of survival compared with the World Health Organization classification. In this series, neither of these 2 histological classifications provided a prognostic stratification of the patients.

Shunting for the Treatment of Arachnoid Cysts in Children

Neurosurgery 67:1632–1636, 2010 DOI: 10.1227/NEU.0b013e3181f94476

The optimal treatment of symptomatic arachnoid cysts remains controversial.

OBJECTIVE: We present a single-institution experience on surgical treatment of arachnoid cysts.

METHODS: Between January 1983 and January 2008, 92 patients with symptomatic arachnoid cysts underwent surgery at our institute. All patients were evaluated with CT and/or MRI and were operated upon and had regular follow-up examinations.

RESULTS: There were 60 males and 32 females. The mean age was 3.6 years (range, 2 days to 14 years). Forty-eight cysts (52%) were within the sylvian fissure, 16 (17%) were in the cerebral convexity, 15 (16%) were infratentorial, 10 (11%) were in the interhemispheric fissure, and 3 (4%) were parasellar. Cystoperitoneal shunt was placed in 67 (73%) patients; ventricular drainage alone was performed in 14 (15%) patients; and combined drainage of the ventricular system and cyst, using a 3-way connector, was performed in 8 (9%) patients. Craniotomy and fenestration of the cyst was performed in 2 (2%) patients, whereas total excision of the cyst was performed in 1 patient. Shunt revision for various reasons was performed in 34 patients (39%), whereas 13 (14%) patients required more than one revision. Complete alleviation of symptoms was achieved in all patients after treatment, regardless of cyst reduction. After a mean follow-up of 8.5 years no further enlargement of the arachnoid cysts was noted.

CONCLUSION: Shunt placement is a safe and effective surgical treatment of symptomatic arachnoid cysts in children. Cyst reduction is greater in children younger than 2 years of age.

Cavernous Malformations of the Brainstem Presenting in Childhood: Surgical Experience in 40 Patients

Neurosurgery 67:1589–1599, 2010 DOI: 10.1227/NEU.0b013e3181f8d1b2

Brainstem cavernous malformations (BSCMs) are believed to compose 9% to 35% of all cerebral cavernous malformations, but these lesions have been reported in children in very limited numbers.

OBJECTIVE: To review surgical outcomes of pediatric patients with BSCMs treated at 1 institution.

METHODS: We retrospectively analyzed the course of 40 pediatric patients (19 males, 21 females; age range, 10 months to 18.9 years; mean, 12.3 years) who underwent surgery between 1984 and 2009. Age, sex, presenting symptoms, location of lesion, surgical approach, new postoperative deficits, Glasgow Outcome Scale score, recurrences, and resolution of baseline symptoms were recorded.

RESULTS: Thirty-nine patients experienced hemorrhage before surgery. Lesion locations included the pons (n = 22), midbrain (n = 4), midbrain and thalamus (n = 4), pontomesencephalic junction (n = 3), medulla (n = 3), pontomedullary junction (n = 3), and cervicomedullary junction (n = 1). Mean lesion size was 2.3 cm. Mean length of hospital stay was 10.7 days. The average clinical follow-up was 31.9 months in 36 patients with follow-up after discharge. At last follow-up, 5 patients had experienced symptoms and/ or imaging consistent with rehemorrhage, either from a residual that enlarged or true recurrence (5.25% annual rebleed risk per patient after surgery); 2 required reoperation for further resection of cavernoma. Mean Glasgow Outcome Scale score was 4.2 on admission, 4.05 at discharge, and 4.5 at latest follow-up. Preoperative symptoms and deficits improved in 16 patients (40%). New neurological deficits developed in 19 patients (48%) and resolved in 9, leaving 10 patients (25%) with new permanent deficit.

CONCLUSION: Compared with adults, pediatric patients with BCSMs tend to have larger lesions and higher rates of recurrence (regrowth of residual lesion). Given the greater life expectancy of children, surgical treatment seems warranted in those with surgically accessible lesions that have bled. Outcomes were similar to those in our adult series of patients with BSCMs.

Experiences with a gravity-assisted valve in hydrocephalic children

J Neurosurg Pediatrics 4:020880–200930, 2009. (DOI: 10.3171/2009.4.PEDS08204)

Over the past decade, a gravity-assisted valve (GAV) has become a standard device in many European pediatric hydrocephalus centers. Because past comparative clinical outcome studies on valve design have not included any GAV, the authors in this trial evaluated the early results of GAV applications in a pediatric population.

Methods. For a minimum of 2 years the authors monitored 169 of 182 hydrocephalic children who received a pediatric GAV at their first CSF shunt insertion (61.5%) or as a substitute for any differential pressure valve (38.5%) at 1 of 7 European pediatric hydrocephalus centers. Outcomes were categorized as valve survival (primary outcome) or shunt survival (secondary outcome). The end point was defined as valve explantation.

Results. Within a follow-up period of 2 years, the valve remained functional in 130 (76.9%) of 169 patients. One hundred eight of these patients (63.9%) had an uncomplicated clinical course without any subsequent surgery, and 22 (13%) were submitted to a valve-preserving catheter revision without any further complications during the follow- up period. Thirty-nine patients (23.1%) reached an end point of valve explantation: 13 valve failures from infection (7.7%), 8 (4.7%) from overdrainage, and 18 (10.6%) from underdrainage.

Conclusions. Compared with nongravitational shunt designs, a GAV does not substantially affect the early complication rate. Valve-preserving shunt revisions do not increase the risk of subsequent valve failure and therefore should not be defined as an end point in studies on valve design. A significant impact of any well-established valve design on the early complication rate in shunt surgery is not supported by any current data; therefore, this correlation should be dismissed. As overdrainage-related complications have been shown to occur late, the presumed advantages of a pediatric GAV remain to be shown in a long-term study.

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