Richard Hayward and Sonia Gonsalez
Object. Elevated intracranial pressure (ICP) is a well-recognized complication affecting children suffering from complex forms of craniosynostosis. The effects of ICP, including those on vision, and the underlying mechanisms involved remain uncertain. The aim of this study was to examine the relationships among ICP, cerebral perfusion pressure (CPP), and the episodic alterations in respiratory obstruction that are common in children with craniosynostosis.
Methods. Eleven children with complex craniosynostosis underwent monitoring overnight, including simultaneous recording of subdural ICP, arterial blood pressure (ABP), and a variety of respiratory parameters. Sleep status was also analyzed. Mean values were calculated for all variables, including ICP, CPP, and ABP, during both quiet and active sleep.
Mean ICP during quiet sleep was elevated in five patients, borderline in three, and normal in three children. During active sleep, plateaus of high mean ICP were observed in all patients. Marked decreases in CPP were demonstrated during active sleep with absolute values as low as 14 mm Hg. During quiet sleep, the mean baseline CPP was 53.3 mm Hg (range 34–70 mm Hg). During active sleep, CPP fell to a mean of 32.6 mm Hg (range 23–52 mm Hg). All patients experienced obstructive breathing problems, including 10 with obstructive sleep apnea, the effects of which demonstrated a temporal relationship to the alterations in sleep status, ICP, and CPP. Elevations of ABP appeared modest and remained within the normal limits for age.
Conclusions. The findings of this study indicate that ICP, CPP, and respiratory obstruction interact in a vicious cycle, an observation that helps explain the pattern of plateau waves of elevated ICP characteristic among children with complex forms of craniosynostosis. The data gathered in this series revealed levels of CPP considerably lower than those described previously in clinical reports. Such reductions in CPP most likely contribute to the neurological, cognitive, and ophthalmological morbidity from which these children suffer frequently; therefore, the results of this study have important implications for the management of children with complex forms of craniosynostosis as well as for our understanding of the control of cerebral blood flow in general.
Martin M. Tisdall, Richard D. Hayward and Dominic N. P. Thompson
A dermal sinus tract is a common form of occult spinal dysraphism. The presumed etiology relates to a focal failure of disjunction resulting in a persistent adhesion between the neural and cutaneous ectoderm. Clinical and radiological features can appear innocuous, leading to delayed diagnosis and failure to appreciate the implications or extent of the abnormality. If it is left untreated, complications can include meningitis, spinal abscess, and inclusion cyst formation. The authors present their experience in 74 pediatric cases of spinal dermal tract in an attempt to identify which clinical and radiological factors are associated with an infective presentation and to assess the reliability of MRI in evaluating this entity.
Consecutive cases of spinal dermal tract treated with resection between 1998 and 2010 were identified from the departmental surgical database. Demographics, clinical history, and radiological and operative findings were collected from the patient records. The presence or absence of active infection (abscess, meningitis) at the time of neurosurgical presentation and any history of local sinus discharge or infection was assessed. Magnetic resonance images were reviewed to evaluate the extent of the sinus tract and determine the presence of an inclusion cyst. Radiological and operative findings were compared.
The surgical course was uncomplicated in 90% of 74 cases eligible for analysis. Magnetic resonance imaging underreported the presence of both an intradural tract (MRI 46%, operative finding 86%) and an intraspinal inclusion cyst (MRI 15%, operative finding 24%). A history of sinus discharge (OR 12.8, p = 0.0003) and the intraoperative identification of intraspinal inclusion cysts (OR 5.6, p = 0.023) were associated with an infective presentation. There was no significant association between the presence of an intradural tract discovered at surgery and an infective presentation.
Surgery for the treatment of spinal dermal tract carries a low morbidity. While it seems intuitive that tracts without intradural extension carry a low risk of spinal cord tethering, it is not possible to reliably detect these cases using MRI. Similarly, intraspinal dermoid cannot be reliably excluded using MRI and carries an increased risk of infection. These points justify excision together with intradural exploration of all spinal dermal sinus tracts.
Matthew A. Kirkman, Richard Hayward, Kim Phipps and Kristian Aquilina
It is relatively unusual for pediatric CNS tumors to be disseminated at presentation, and the literature on the clinical features, management, and outcomes of this specific group is scarce. Surgical management in this population is often challenging, particularly in the presence of hydrocephalus. The authors present their recent experience of treating pediatric CNS tumors that were disseminated at presentation, and they compare these lesions with focal tumors.
The authors performed a retrospective review of prospectively collected data on children presenting to a tertiary center between 2003 and 2016 inclusive.
Of 361 children with CNS tumors, the authors identified 53 patients with disease dissemination at presentation (male/female ratio 34:19, median age 3.8 years, age range 7 days to 15.6 years) and 308 without dissemination at presentation (male/female ratio 161:147, median age 5.8 years, age range 1 day to 16.9 years). Five tumor groups were studied: medulloblastoma (disseminated n = 29, focal n = 74), other primitive neuroectodermal tumor (n = 8, n = 17), atypical teratoid rhabdoid tumor (n = 8, n = 22), pilocytic astrocytoma (n = 6, n = 138), and ependymoma (n = 2, n = 57). The median follow-up duration in survivors was not significantly different between those with disease dissemination at presentation (64.0 months, range 5.2–152.0 months) and those without it (74.5 months, range 4.7–170.1 months) (p > 0.05). When combining data from all 5 tumor groups, dissemination status at presentation was significantly associated with a higher risk of requiring CSF diversion, a higher surgical complication rate, and a reduced likelihood of achieving gross-total resection of the targeted lesion (all variables p < 0.05). Differences between the 5 tumor groups were evident. No factors that predicted the need for permanent CSF diversion following temporary external ventricular drainage were identified on multivariate analysis, and there was no clear superiority of either ventriculoperitoneal shunt surgery or endoscopic third ventriculostomy as a permanent CSF diversion procedure.
Tumor type and dissemination status at initial presentation significantly affect outcomes across a range of measures. The management of hydrocephalus in patients with CNS tumors is challenging, and further prospective studies are required to identify the optimal CSF diversion strategy in this population.
Ian K. Pople, Roger Bayston and Richard D. Hayward
✓ The aim of this study was to find reasons for the high incidence of cerebrospinal fluid shunt infections seen in neonates. Four-hundred sixty-six consecutive shunt operations were analyzed retrospectively in 294 children, and 60 children were studied prospectively by quantitative sampling of skin bacteria before surgery and by sampling open wounds, shunt catheters, surgical gloves, and airborne bacteria. In total, 110 strains of coagulase-negative Staphylococcus isolated from the skin of 53 children before surgery were then tested for bacterial adherence.
Retrospectively, the infection rate for infants younger than 6 months old was 15.7% (28 of 178 procedures), compared with 5.6% (16 of 288 procedures) for older children (p = 0.0005). Of all infections, 67% were due to coagulase-negative Staphylococcus. Age was the only major factor influencing the infection rate. Three of the 60 children studied prospectively developed postoperative shunt infections. All were younger than 6 months and all had high skin bacterial densities before surgery. Contamination during surgery was generally low, but correlated with the preoperative skin bacterial density. Strains of coagulase-negative Staphylococcus with high bacterial adherence were more commonly found in neonates than in older children.
High skin bacterial density in neonates before surgery was a risk factor for infection in this study. These results also suggest that there is selection of more virulent strains of coagulase-negative Staphylococcus on the skin of neonates. Prevention of shunt infections in this high-risk group could be facilitated by the reduction of skin bacterial density before surgery using chlorhexidine shampoos and by the elimination of contamination by skin bacteria during surgery using packs soaked in an antiseptic agent to isolate wound edges and glove-changing before handling the shunt.
Juan F. Martínez-Lage
Richard Hayward, Jonathan Britto, David Dunaway and Owase Jeelani
Ghassan S. Abu-Sittah, Owase Jeelani, David Dunaway and Richard Hayward
Patients with Crouzon syndrome (CS) are at risk for developing raised intracranial pressure (ICP), which has the potential to impair both vision and neurocognitive development. For this reason, some experts recommend early prophylactic cranial vault expansion on the basis that if ICP is not currently raised, it is likely to become so. The aim of this study was to examine the justification for such a policy. This was done by analyzing the incidence, causes, and subsequent risk of recurrence in a series of patients with CS, in whom raised ICP was treated only after it had been diagnosed.
This study was a retrospective review of the medical records and imaging data of patients with a clinical diagnosis of CS.
There were 49 patients in the study, of whom 30 (61.2%) developed at least 1 episode of raised ICP. First episodes occurred at an average age of 1.42 years and were attributable to craniocerebral disproportion/venous hypertension (19 patients), hydrocephalus (8 patients), and airway obstruction (3 patients). They were managed, respectively, by vault expansion, ventriculoperitoneal shunt insertion, and airway improvement. Fourteen of the 30 patients developed a second episode of raised ICP an average of 1.42 years after treatment for their initial episode, and 3 patients developed a third episode an average of 3.15 years after that. Causes of subsequent episodes of raised ICP often differed from previous episodes and required different management. Patients who were < 1 year old when the first episode was diagnosed were at increased risk of recurrence.
Although the incidence of raised ICP in CS is high, it did not occur in nearly 40% of children during the course of this study. The several possible causes of CS require different management and may vary from episode to episode. The authors recommend an expectant policy toward these children with careful clinical, ophthalmological, respiratory, and radiological monitoring for raised ICP, reserving intervention for when it has been detected and the appropriate treatment can be initiated.
Dawn E. Saunders, Kim P. Phipps, Angela M. Wade and Richard D. Hayward
Object. The authors sought to evaluate surveillance strategies for the detection and monitoring of residual and recurrent disease in children with cerebellar low-grade astrocytomas (CLGAs) treated surgically or with radiotherapy. Patients were divided into three groups: 1) those in whom a “complete” resection was achieved; 2) those with residual disease with no immediate adjuvant therapy; and 3) those who received radiotherapy for residual/recurrent disease.
Methods. Magnetic resonance (MR) imaging studies and clinical data obtained in children with CLGA who presented between January 1988 and September 1998 were reviewed. Eighty-four children were followed for a mean period of 73 months (range 2–159 months). One child died. Of the 70 children in whom a complete resection was achieved, nine (13%) developed a recurrence detected by surveillance imaging at 6, 8, 9, 9, 13, 27, 39, 44, and 47 months, respectively. Following an incomplete resection, radiologically detected tumor progression leading to further treatment was detected at 7, 9, 12, 13, and 20 months, respectively, and an additional six tumors regressed or stablized. In 11 of 12 children treated with radiotherapy, stabilization/regression occurred radiologically at a mean of 14.9 months.
Conclusions. The authors recommend surveillance MR imaging in children treated for CLGA at 6 months and 1, 2, 3.5, and 5 years following a complete resection and after radiotherapy performed either initially or following recurrence. For follow up of residual tumor, 6-month interval imaging for at least 3 years, yearly images for another 2 years, and subsequent 2-year imaging is recommended.