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Protocol for the multicentre prospective paediatric craniectomy and cranioplasty registry (pedCCR) under the auspices of the European Society for Paediatric Neurosurgery (ESPN)

Authors:
  • Fondazione Policlinico Universitario A. Gemelli IRCCS - Rome (Italy)

Abstract and Figures

Purpose In the paediatric age group, the overall degree of evidence regarding decompressive craniectomy (DC) and cranioplasty is low, whereas in adults, randomised controlled trials and prospective multicentre registries are available. To improve the evidence-based treatment of children, a consensus was reached to establish a prospective registry under the auspices of the European Society for Pediatric Neurosurgery (ESPN). Methods This international multicentre prospective registry is aimed at collecting information on the indication, timing, technique and outcome of DC and cranioplasty in children. The registry will enrol patients ≤ 16 years of age at the time of surgery, irrespective of the underlying medical condition. The study design comprises four obligatory entry points as a core dataset, with an unlimited number of further follow-up entry points to allow documentation until adolescence or adulthood. Study centres should commit to complete data entry and long-term follow-up. Results Data collection will be performed via a web-based portal (homepage: www.pedccr.com ) in a central anonymised database after local ethics board approval. An ESPN steering committee will monitor the project’s progress, coordinate analyses of data and presentation of results at conferences and in publications on behalf of the study group. Conclusion The registry aims to define predictors for optimal medical care and patient-centred treatment outcomes. The ultimate goal of the registry is to generate results that are so relevant to be directly transferred into clinical practice to enhance treatment protocols.
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https://doi.org/10.1007/s00381-022-05540-5
ORIGINAL ARTICLE
Protocol forthemulticentre prospective paediatric craniectomy
andcranioplasty registry (pedCCR) undertheauspices oftheEuropean
Society forPaediatric Neurosurgery (ESPN)
ThomasBeez1 · MartinU.Schuhmann2· PaoloFrassanito3· FedericoDiRocco4· UlrichW.Thomale5·
HansChristophBock6
Received: 1 March 2022 / Accepted: 21 April 2022
© The Author(s) 2022
Abstract
Purpose In the paediatric age group, the overall degree of evidence regarding decompressive craniectomy (DC) and cranio-
plasty is low, whereas in adults, randomised controlled trials and prospective multicentre registries are available. To improve
the evidence-based treatment of children, a consensus was reached to establish a prospective registry under the auspices of
the European Society for Pediatric Neurosurgery (ESPN).
Methods This international multicentre prospective registry is aimed at collecting information on the indication, timing,
technique and outcome of DC and cranioplasty in children. The registry will enrol patients 16years of age at the time of
surgery, irrespective of the underlying medical condition. The study design comprises four obligatory entry points as a core
dataset, with an unlimited number of further follow-up entry points to allow documentation until adolescence or adulthood.
Study centres should commit to complete data entry and long-term follow-up.
Results Data collection will be performed via a web-based portal (homepage: www. pedccr. com) in a central anonymised
database after local ethics board approval. An ESPN steering committee will monitor the project’s progress, coordinate
analyses of data and presentation of results at conferences and in publications on behalf of the study group.
Conclusion The registry aims to define predictors for optimal medical care and patient-centred treatment outcomes. The
ultimate goal of the registry is to generate results that are so relevant to be directly transferred into clinical practice to enhance
treatment protocols.
Keywords Decompressive craniectomy· Autologous cranioplasty· Allogeneic cranioplasty· Intracranial pressure· Bone
flap resorption· Functional outcome
Introduction
Decompressive craniectomy (DC) is part of the armamen-
tarium to control critically raised intracranial pressure (ICP)
occurring at different stages after severe cerebral insults [1]
: Primary DC is used to treat patients with significant space-
occupying lesions, in whom the risk of evolving brain edema
is high. Secondary or delayed DC is usually considered as
a final step if intracranial hypertension becomes refractory
to conservative measures. In addition to the implications of
primary and secondary brain injury itself, the limitations,
inherent risks and complications of DC and also of subse-
quent cranioplasty have to be taken into account.
Looking at the adult age group, several studies with high-
quality methodology have been or are being conducted: A
randomised controlled trial (RCT) investigating the role of
* Thomas Beez
thomas.beez@med.uni-duesseldorf.de
1 Department ofNeurosurgery, Medical Faculty, Heinrich-
Heine-Universität, Düsseldorf, Moorenstrasse 5,
40225Düsseldorf, Germany
2 Pediatric Neurosurgery, Universitätsklinikum Tübingen,
Tübingen, Germany
3 Pediatric Neurosurgery, Fondazione Policlinico Universitario
A. Gemelli IRCCS, Rome, Italy
4 Service de Neurochirurgie Pédiatrique, Hôpital Femme Mère
Enfant, Lyon, France
5 Pediatric Neurosurgery, Charité Universitätsmedizin Berlin,
Berlin, Germany
6 Department ofNeurosurgery, Universitätsmedizin Göttingen,
Göttingen, Germany
/ Published online: 9 May 2022
Child's Nervous System (2022) 38:1461–1467
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1 3
primary DC (RESCUE-ASDH) has finished enrolment and
the final results are not yet published(ISRCTN87370545).
Concerning secondary DC for refractory intracranial hyper-
tension (> 25mmHg) due to severe traumatic brain injury
(TBI), the RESCUEicp trial indicated a lower mortality com-
pared to conservative management [2]. The similar DECRA
trial had a lower ICP threshold (> 20mmHg) and demon-
strated reduced mortality but more unfavourable outcomes
after DC [3]. For malignant ischemic stroke, several RCTs
(including HAMLET, DECIMAL, DESTINY I and II) proved
a significant reduction of mortality, although DC renders a
relevant subgroup with moderately severe disability [47].
In contrast, such high-level evidence is not available in
the paediatric age group, where the large majority of previ-
ous publications are retrospective and monocentric [818].
For a recent review on DC in paediatric TBI, Ardissino etal.
screened 212 studies, but only 12 ultimately qualified for
systematic comparison [19]. The authors concluded that DC
reduces mortality and may improve functional outcome, but
they also highlighted significant knowledge gaps. Results for
DC in paediatric ischemic stroke are limited to case series
and anecdotal case reports [20].
Regarding cranioplasty, the level of evidence is low for
all age groups. Klieverik etal. recently screened 393 publi-
cations on paediatric cranioplasty and ultimately included
24 articles in their systematic review [21]. They concluded
that both autologous and alloplastic cranioplasty appeared
to be associated with relevant complication rates, with the
problem of aseptic bone flap resorption having a pronounced
impact on the paediatric cohort. Beyond this, no reliable
conclusions were possible and the authors emphasised the
relevance of large prospective cohort studies. To improve
the evidence base in adults, two prospective multicentre reg-
istries are actively recruiting patients
18years of age in
Europe (UKCRR in the UK and GCRR in Germany, Austria
and Switzerland) [22, 23].
The recent efforts of both Ardissino etal. and Klieverik
etal. highlight the problems encountered in paediatric DC
and cranioplasty [19, 21] : The pooling of published results
is hindered by heterogeneous data elements and by miss-
ing information. Few studies provide long-term information
spanning craniectomy and cranioplasty, although both opera-
tions are closely related and relevant to the overall morbidity
and outcome of individual patients. If we attempt to fill these
evidence gaps with extrapolation of study results obtained in
the adult age group, there is a significant caveat: Highly rel-
evant differences in anatomy and physiology are described
between adults and children and even within the paediatric
age spectrum [24, 25]. Additionally, a fixed point of outcome
assessment as used in adults (in virtually all RCTs after 6
and/or 12months) does not adequately reflect the impact
of injuries and treatments on the developing child’s brain.
Children require longitudinal observation over many years
with age-adjusted outcome measures.
To this point, it should have become clear that the field of
paediatric DC and cranioplasty requires significant research
activity. However, to further justify such efforts, the rele-
vance of the field has to be taken into account as well. In
the paediatric age group, the main cause of severe acute
primary and secondary brain injury with consecutive intrac-
ranial hypertension and need for decompressive craniectomy
(and thus later cranioplasty) is TBI. Therefore, the best epi-
demiological and health-economic data is available for this
condition: 30% of all TBI cases occur in patients under the
age of 16, of which approximately 10% suffer moderate or
severe TBI [26]. The financial burden of TBI is significant,
with estimated annual costs for TBI-related hospitalisation
of children in the USA of more than $ 1 billion [27]. With
regard to medical outcomes, it is assumed that 30% of chil-
dren do not survive severe TBI despite DC [14, 28]. Among
survivors a good outcome can be expected in 60–90%
depending on the type of initial cerebral insult [28, 29]. In
addition to the sequelae of the insult itself, the risks of DC
and cranioplasty (especially CSF disorders, infections and
resorption of autologous bone flaps with the need for revi-
sion surgery) need to be taken into account [13, 3034]. The
field is therefore highly relevant for the individual child and,
not least due to associated health-care costs, also to society.
The initial proposal for this study was presented at the
ESPN Consensus Conference 2019 in Paris. At this confer-
ence, a consensus was reached to establish a multicentric
registry under the auspices of ESPN. Achieving optimal out-
comes after severe insults to the child’s brain is of utmost
importance and the ESPN pedCCR will significantly con-
tribute towards this aim.
Aims andobjectives
After reaching consensus to establish a multicentre, prospec-
tive, registry under the auspices of ESPN, an initial interna-
tional steering committee was formed to formulate the study
goals and generate a proposal for a study protocol, which
was subsequently ratified by the ESPN board.
The primary objective of this study is a detailed system-
atic assessment of DC and cranioplasty in children (defined
as patients 16years of age at the time of surgery) with
regard to indication, timing, technique and outcomes, irre-
spective of underlying disease and with a minimum follow-
up of 24months after cranioplasty. The secondary objective
is the comparison of treatment strategies and identification
of predictors for optimal outcomes of DC and cranioplasty.
This study will generate an international multicentric, pro-
spectively collected data set to achieve these objectives.
1462 Child's Nervous System (2022) 38:1461–1467
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Such systematic and high-quality data collection and
analysis will improve the evidence base and thus medi-
cal care in several specific aspects. The concept of the
study is characterised by patient orientation: Based on a
large, prospective patient series, we aim for identifying
risk factors as well as optimal and suboptimal approaches.
This effort may ultimately reduce the complication rate,
thereby increasing patient safety and optimizing outcome.
The study protocol explicitly includes the long-term course
and health-related quality of life (using KIDSCREEN-10)
in age-dependent self or external assessment, in addition
to the King’s Outcome Scale for Childhood Head Injury
(KOSCHI) [35, 36]. The latter scale is validated for TBI,
but can be applied to other conditions similar to the Glas-
gow Outcome Scale.
A systematic analysis and comparison of different cranio-
plasty materials and techniques will deliver further knowledge
in order to optimise quality of care and cost-effectiveness in
this critical phase after TBI, as cranioplasty carries significant
short- and long-term risks in children [34]. Ultimately, based
on the data collected, revision surgery could be avoided and
implants with the best cost–benefit ratio could be identified.
Additionally, the optimal timing of cranioplasty will be ana-
lysed, as there is currently conflicting data on early versus
delayed cranial reconstruction [3740]. A further strength of
the registry is its focus on specific technical details from a
paediatric neurosurgical perspective, which are often impos-
sible to be reconstructed retrospectively from operation notes.
Study design
This is a multicentre, prospective, registry. Patients 16
years of age at the time of surgery can be included after
informed consent as detailed below, irrespective of under-
lying disease (i.e. indication for DC). In surviving patients,
the study centres are committed to contributing data on
subsequent cranioplasty as well as a minimum follow-up of
24months after cranioplasty (Fig.1). Further follow-up until
adolescence or adulthood is encouraged.
The criteria applying for patient enrolment into the regis-
try were kept simple as a result of the low incidence of DC
in children and to actively encourage recruitment (Table1).
Based on retrospective data and an exploratory review of
the literature, we estimate an annual recruitment of 2 to 5
patients per centre. The experience of the German Cranio-
plasty Registry for adult patients (GCRR) has shown that
approximately 10 national centres can be expected to partici-
pate, depending on the size of the country [22]. Sample size
justification is based on a minimal assumption of 20 contrib-
uting centres for the ESPN pedCCR, with mean enrolment
of 3.5 patients per year. The registry could therefore have an
annual recruitment of 70 patients.
Fig. 1 Illustration of the study
design, with 4 obligatory data
entry points for a complete core
data set
Table 1 Overview of inclusion
and exclusion criteria Inclusion criteria Exclusion criteria
- Age
16years at the time of surgery - Cranioplasty for conditions other
than DC (e.g. congenital skull
defects)
- Any type of underlying cerebral insult
- Informed consent obtained
- Ability to provide follow-up of at least 24months after cranio-
plasty
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1 3
Data entry will be web-based and the protocol is opti-
mised towards efficiency and low resource requirements for
the contributing centres in order to encourage active and
lasting enrolment, complete data entries and sufficient fol-
low-up. A complete minimal data set for a patient surviving
after DC would include four data entry points, i.e. forms
for DC Module, Cranioplasty Module and two Routine
Follow-up Modules at 12 and 24months after cranioplasty,
respectively (Fig.1). The data elements for each module
are partially based on the National Institute of Neurologi-
cal Disorders and Stroke (NINDS) Common Data Elements
(https:// commo ndata eleme nts. ninds. gov). Additionally, sev-
eral validated scores and measures are explicitly or implic-
itly contained within the forms. The forms for each module
are outlined below:
Decompressive craniectomy module
48 items
Paediatric GCS prior to DC and at discharge or day 30
after DC [41]
Paediatric Risk of Mortality Score (PRISM) within 4h
after admission [42]
Rotterdam CT Score [43]
KOSCHI at discharge or day 30 after DC [35]
Cranioplasty module
26 items
Paediatric GCS prior to cranioplasty and at discharge or
day 30 after cranioplasty
KOSCHI prior to cranioplasty and at discharge or day 30
after cranioplasty
KIDSCREEN-10 prior to cranioplasty [36]
Routine follow‑up module
10 items
Paediatric GCS
– KOSCHI
– KIDSCREEN-10
Oulo Resorption Score (if bone flap resorption observed
on imaging) [44]
Incident reporting module
13 items
Paediatric GCS
– KOSCHI
– KIDSCREEN-10
Oulo Resorption Score (if bone flap resorption observed
on imaging)
Data management andstatistical analysis
A web-based database using Filemaker® software has been
designed to allow password-protected data entry (Fig.2),
similar to the system successfully used for the TROPHY
registry [45, 46]. The central server is physically located
and professionally hosted in Europe. Data transfer between
the user and the study server is encrypted (SSL coding) to
assure data privacy. Access to the online registry applica-
tion is provided via the study homepage: www. pedccr. com.
Patients will be pseudonymised (consecutive numbers)
locally by the respective centre. The central data collection
will then be done anonymously, i.e. the central database
itself does not contain any identifying patient information
and the pseudonymisation key will be securely kept at the
local centre.
As this is a prospective registry without a limited study
period, data collection will be ongoing and no endpoints
were predefined. Regular audits will be performed to
ensure data quality and integrity. Data analysis will be
performed with descriptive statistics. Based on results
from the previous literature, the following statistical
assumptions regarding relevant clinical variables were
made: 30-day-complication rate after DC — 40%; good
outcome after DC — 50%; mortality after DC — 30% and
autologous bone flap resorption rate — 80%. With the aim
of a confidence level of 90% and an error margin of less
than
±
10%, an analysis will be carried out for N = 100
included cases. To compare the complication rate between
autologous versus allogeneic cranioplasty, an evaluation
of N = 150 cases per cranioplasty modality will be carried
out in view of the complication rates from the literature
of 33% versus 14%.
Ethics andinformed consent
The study will be carried out in accordance with the prin-
ciples of the Declaration of Helsinki in the revised version
of 2013. The study protocol has been approved by the ethi-
cal review board at Heinrich-Heine-University, Düsseldorf,
Germany (study number 2021–1653). Each centre will need
to have obtained a positive local ethics vote before begin-
ning enrolment. The study protocol and consent forms in
German and English will be available for download on the
study homepage upon user registration. Since we are includ-
ing underage subjects, the legal representatives or the carer
must provide written consent. If the minor is able to under-
stand the nature of the study, his/her written consent is also
required. An age-appropriate adapted patient information
leaflet and consent form will be provided. The individual
patient data can be deleted completely and irretrievably at
any time upon the patient’s request, without giving reasons.
1464 Child's Nervous System (2022) 38:1461–1467
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Conclusions
The lack of high-quality data and thus the low degree
of evidence on which treatment decisions can be based
with regard to DC and cranioplasty in children became
evident during the ESPN Consensus Conference 2019
in Paris. Consensus was reached to create this registry
as an important way to systematically collect real-world
experiences in the field and analyse and compare treat-
ment approaches across paediatric neurosurgical centres.
We believe that this “science of practice” approach will
achieve a high degree of internal and external validity and
answer important questions and stimulate further research.
Contribution and collaboration at all levels, including
Fig. 2 Representative screen shots of the pedCCR database — A homepage (homepage: www. pedccr. com) and B data entry form for DC
1465Child's Nervous System (2022) 38:1461–1467
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1 3
optimizing the study design, is highly appreciated to
advance this project together.
Funding Open Access funding enabled and organized by Projekt
DEAL. The study receives financial support from the European Soci-
ety for Pediatric Neurosurgery (ESPN), Geneva, Switzerland, and the
independent not-for-profit organisation ZNS – Hannelore Kohl Stiftung
für Unfallverletzte mit Schäden des Zentralen Nervensystems, Bonn,
Germany.
Declarations
Conflict of interest The authors declare that they have no conflict of
interest.
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need to obtain permission directly from the copyright holder. To view a
copy of this licence, visit http:// creat iveco mmons. org/ licen ses/ by/4. 0/.
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Article
Cranial repair in children deserves particular attention since many issues are still controversial. Furthermore, literature data offer a confused picture of outcome of cranioplasty, in terms of results and complication rates, with studies showing inadequate follow-up and including populations that are not homogeneous by age of the patients, etiology, and size of the bone defect. Indeed, age has merged in the last years as a risk factor for resorption of autologous bone flap that is still the most frequent complication in cranial repair after decompressive craniectomy. Age-related factors play a role also when alloplastic materials are used. In fact, the implantation of alloplastic materials is limited by skull growth under 7 years of age and is contraindicated in the first years if life. Thus, the absence of an ideal material for cranioplasty is even more evident in children with a steady risk of complications through the entire life of the patient that is usually much longer than surgical follow-up. As a result, specific techniques should be adopted according to the age of the patient and etiology of the defect, aiming to repair the skull and respect its residual growth. Thus, autologous bone still represents the best option for cranial repair, though limitations exist. As an alternative, biomimetic materials should ideally warrant the possibility to overcome the limits of other inert alloplastic materials by favoring osteointegration or osteoinduction or both. On these grounds, this paper aims to offer a thorough overview of techniques, materials, and peculiar issues of cranial repair in children.
Article
Full-text available
Introduction Traumatic brain injury (TBI) remains the commonest neurological and neurosurgical cause of death and survivor disability among children and young adults. This review summarizes some of the important recent publications that have added to our understanding of the condition and advanced clinical practice. Methods Targeted review of the literature on various aspects of paediatric TBI over the last 5 years. Results Recent literature has provided new insights into the burden of paediatric TBI and patient outcome across geographical divides and the severity spectrum. Although CT scans remain a standard, rapid sequence MRI without sedation has been increasingly used in the frontline. Advanced MRI sequences are also being used to better understand pathology and to improve prognostication. Various initiatives in paediatric and adult TBI have contributed regionally and internationally to harmonising research efforts in mild and severe TBI. Emerging data on advanced brain monitoring from paediatric studies and extrapolated from adult studies continues to slowly advance our understanding of its role. There has been growing interest in non-invasive monitoring, although the clinical applications remain somewhat unclear. Contributions of the first large scale comparative effectiveness trial have advanced knowledge, especially for the use of hyperosmolar therapies and cerebrospinal fluid drainage in severe paediatric TBI. Finally, the growth of large and even global networks is a welcome development that addresses the limitations of small sample size and generalizability typical of single-centre studies. Conclusion Publications in recent years have contributed iteratively to progress in understanding paediatric TBI and how best to manage patients.
Article
Full-text available
Introduction The TROPHY registry has been established to conduct an international multicenter prospective data collection on the surgical management of neonatal intraventricular hemorrhage (IVH)-related hydrocephalus to possibly contribute to future guidelines. The registry allows comparing the techniques established to treat hydrocephalus, such as external ventricular drainage (EVD), ventricular access device (VAD), ventricular subgaleal shunt (VSGS), and neuroendoscopic lavage (NEL). This first status report of the registry presents the results of the standard of care survey of participating centers assessed upon online registration. Methods On the standard of treatment forms, each center indicated the institutional protocol of interventions performed for neonatal post-hemorrhagic hydrocephalus (nPHH) for a time period of 2 years (Y1 and Y2) before starting the active participation in the registry. In addition, the amount of patients enrolled so far and allocated to a treatment approach are reported. Results According to the standard of treatment forms completed by 56 registered centers, fewer EVDs (Y1 55% Y2 46%) were used while more centers have implemented NEL (Y1 39%; Y2 52%) to treat nPHH. VAD (Y1 66%; Y2 66%) and VSGS (Y1 42%; Y2 41%) were used at a consistent rate during the 2 years. The majority of the centers used at least two different techniques to treat nPHH (43%), while 27% used only one technique, 21% used three, and 7% used even four different techniques. Patient data of 110 infants treated surgically between 9/2018 and 2/2021 (13% EVD, 15% VAD, 30% VSGS, and 43% NEL) were contributed by 29 centers. Conclusions Our results emphasize the varying strategies used for the treatment of nPHH. The international TROPHY registry has entered into a phase of growing patient recruitment. Further evaluation will be performed and published according to the registry protocol.
Article
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Purpose Decompressive craniectomy (DC) is an established neurosurgical emergency technique. Patient selection, optimal timing, and technical aspects related to DC and subsequent cranioplasty remain subjects of debate. For children, the overall degree of evidence is low, compared with randomized controlled trials (RCTs) in adults. Methods Here, we present a detailed retrospective analysis of pediatric DC, covering the primary procedure and cranioplasty. Results are analyzed and discussed in the light of modern scientific evidence, and conclusions are drawn to stimulate future research. Results The main indication for DC in children is traumatic brain injury (TBI). Primary and secondary DC is performed with similar frequency. Outcome appears to be better than that in adults, although long-term complications (especially bone flap resorption after autologous cranioplasty) are more common in children. Overt clinical signs of cerebral herniation prior to DC are predictors of poor outcome. Conclusions We conclude that DC is an important option in the armamentarium to treat life-threatening intracranial hypertension, but further research is warranted, preferentially in a multicenter prospective registry.
Article
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Malignant stroke occurs in a subgroup of patients suffering from ischemic cerebral infarction and is characterized by neurological deterioration due to progressive edema, raised intracranial pressure, and cerebral herniation. Decompressive craniectomy (DC) is a surgical technique aiming to open the “closed box” represented by the non-expandable skull in cases of refractory intracranial hypertension. It is a valuable modality in the armamentarium to treat patients with malignant stroke: the life-saving effect has been proven for both supratentorial and infratentorial DC in virtually all age groups. This leaves physicians with the difficult task to decide who will require early or preemptive surgery and who might benefit from postponing surgery until clear evidence of deterioration evolves. Together with the patient’s relatives, physicians also have to ascertain whether the patient will have acceptable disability and quality of life in his or her presumed perception, based on preoperative predictions. This complex decision-making process can only be managed with interdisciplinary efforts and should be supported by continued research in the age of personalized medicine.
Article
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Introduction Among children with hydrocephalus, neonates with intraventricular hemorrhage (IVH) and posthemorrhagic hydrocephalus (PH) are considered a group with one of the highest complication rates of treatment. Despite continued progress in neonatal care, a standardized and reliable guideline for surgical management is missing for this challenging condition. Thus, further research is warranted to compare common methods of surgical treatment. The introduction of neuroendoscopic lavage has precipitated the establishment of an international registry aimed at elaborating key elements of a standardized surgical treatment. Methods The registry is designed as a multicenter, international, prospective data collection for neonates aged 41 weeks gestation, with an indication for surgical treatment for IVH with ventricular dilatation and progressive hydrocephalus. The following initial temporizing surgical interventions, each used as standard treatment at participating centers, will be compared: external ventricular drainage (EVD), ventricular access device (VAD), ventricular subgaleal shunt (VSGS), and neuroendoscopic lavage (NEL). Type of surgery, perioperative data including complications and mortality, subsequent shunt surgeries, ventricular size, and neurological outcome will be recorded at 6, 12, 36, and 60 months. Results An online, password-protected website will be used to collect the prospective data in a synchronized manner. As a prospective registry, data collection will be ongoing, with no prespecified endpoint. A prespecified analysis will take place after a total of 100 patients in the NEL group have been entered. Analyses will be performed for safety (6 months), shunt dependency (12, 24 months), and neurological outcome (60 months). Conclusion The design and online platform of the TROPHY registry will enable the collection of prospective data on different surgical procedures for investigation of safety, efficacy, and neurodevelopmental outcome of neonates with IVH and hydrocephalus. The long-term goal is to provide valid data on NEL that is prospective, international, and multicenter. With the comparison of different surgical treatment modalities, we hope to develop better therapy guidelines for this complex neurosurgical condition.
Article
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Background Bone flap resorption (BFR) is the most prevalent complication resulting in autologous cranioplasty failure, but no consensus on the definition of BFR or between the radiological signs and relevance of BFR has been established. We set out to develop an easy-to-use scoring system intended to standardize the interpretation of radiological BFR findings. Methods All 45 autologous cranioplasty patients operated on at Oulu University Hospital from 2004 to 2014 were identified, and the bone flap status of all the available patients was evaluated using the new scoring system. Derived from previous literature, a three-variable score for the detection of BFR changes is proposed. The variables “Extent” (estimated remaining bone volume), “Severity” (possible perforations and their measured diameter), and “Focus” (the number of BFR foci within the flap) are scored from 0 to 3 individually. Using the sum of these scores, a score of 0–9 is assigned to describe the degree of BFR. Additionally, independent neurosurgeons assessed the presence and relevance of BFR from the same data set. These assessments were compared to the BFR scores in order to find a score limit for relevant BFR. Results BFR was considered relevant by the neurosurgeons in 11 (26.8%) cases. The agreement on the relevance of BFR demonstrated substantial strength (κ 0.64, 95%CI 0.36 to 0.91). The minimum resorption score in cases of relevant BFR was 5. Thus, BFR with a resorption score ≥ 5 was defined relevant (grades II and III). With this definition, grade II or III BFR was found in 15 (36.6%) of our patients. No risk factors were found to predict relevant BFR. Conclusions The score was proven to be easy to use and we recommend that only cases with grades II and III BFR undergo neurosurgical consultation. However, general applicability can only be claimed after validation in independent cohorts.
Article
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Introduction Complications following cranioplasty with either autografts or cranial implants are commonly reported in pediatric patients. However, data regarding cranioplasty strategies, complications and long-term outcomes are not well described. This study systematically reviews the literature for an overview of current cranioplasty practice in children. Methods A systematic review of articles published from inception to July 2018 was performed. Studies were included if they reported the specific use of cranioplasty materials following craniectomy in patients younger than 18 years of age, and had a minimum follow-up of at least 1 year. Results Twenty-four manuscripts, describing a total of 864 cranioplasty procedures, met the inclusion criteria. The age of patients in this aggregate ranged from 1 month to 20 years and the weighted average was 8.0 years. The follow-up ranged from 0.4 months to 18 years and had a weighted average of 40.4 months. Autologous bone grafts were used in 484 cases (56.0%). Resorption, infection and/or hydrocephalus were the most frequently mentioned complications. In this aggregate group, 61 patients needed a revision cranioplasty. However, in 6/13 (46%) papers studying autologous cranioplasties, no data was provided on resorption, infection and revision cranioplasty rates. Cranial implants were used in 380 cases (44.0%), with custom-made porous hydroxyapatite being the most commonly used material (100/380, 26.3%). Infection and migration/fracturing/loosening were the most frequently documented complications. Eleven revision cranioplasties were reported. Again, no data was reported on infection and revision cranioplasty rates, in 7/16 (44%) and 9/16 (56%) of papers, respectively. Conclusion Our systematic review illuminates that whether autografts or cranial implants are used, postcranioplasty complications are quite common. Beyond this, the existing literature does not contain well documented and comparable outcome parameters, suggesting that prospective, long-term multicenter cohort studies are needed to be able to optimize cranioplasty strategies in children who will undergo cranioplasty following craniectomy.
Article
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Introduction Paediatric traumatic brain injury (pTBI) is one of the most frequent neurological presentations encountered in emergency departments worldwide. Every year, more than 200,000 American children suffer pTBIs, many of which lead to long-term damage. Objectives We aim to review the existing evidence on the efficacy of the decompressive craniectomy (DC) in controlling intracranial pressure (ICP) and improving long-term outcomes in children with pTBI. Methods A comprehensive search of the MEDLINE and EMBASE databases led to the screening of 212 studies, 12 of which satisfied inclusion criteria. Data extracted included the number and ages of patients, Glasgow Coma Scale scores at presentation, treatment protocols and short- and long-term outcomes. Results Each of the nine studies including ICP as an outcome reported that it was successfully controlled by DC. The 6–12 month outcome scores of patients undergoing DC were positive, or superior to those of medically treated groups in nine of 11 studies. Mortality was compared in only two studies, and was lower in the DC group in both.Very few studies are currently available investigating short- and long-term outcomes in children with TBI undergoing DC. Conclusion The currently available evidence may support a beneficial role of DC in controlling ICP and improving long-term outcomes.
Article
Full-text available
OBJECTIVE In children, the repair of skull defects arising from decompressive craniectomy presents a unique set of challenges. Single-center studies have identified different risk factors for the common complications of cranioplasty resorption and infection. The goal of the present study was to determine the risk factors for bone resorption and infection after pediatric cranioplasty. METHODS The authors conducted a multicenter retrospective case study that included all patients who underwent cranioplasty to correct a skull defect arising from a decompressive craniectomy at 13 centers between 2000 and 2011 and were less than 19 years old at the time of cranioplasty. Prior systematic review of the literature along with expert opinion guided the selection of variables to be collected. These included: indication for craniectomy; history of abusive head trauma; method of bone storage; method of bone fixation; use of drains; size of bone graft; presence of other implants, including ventriculoperitoneal (VP) shunt; presence of fluid collections; age at craniectomy; and time between craniectomy and cranioplasty. RESULTS A total of 359 patients met the inclusion criteria. The patients’ mean age was 8.4 years, and 51.5% were female. Thirty-eight cases (10.5%) were complicated by infection. In multivariate analysis, presence of a cranial implant (primarily VP shunt) (OR 2.41, 95% CI 1.17–4.98), presence of gastrostomy (OR 2.44, 95% CI 1.03–5.79), and ventilator dependence (OR 8.45, 95% CI 1.10–65.08) were significant risk factors for cranioplasty infection. No other variable was associated with infection. Of the 240 patients who underwent a cranioplasty with bone graft, 21.7% showed bone resorption significant enough to warrant repeat surgical intervention. The most important predictor of cranioplasty bone resorption was age at the time of cranioplasty. For every month of increased age the risk of bone flap resorption decreased by 1% (OR 0.99, 95% CI 0.98–0.99, p < 0.001). Other risk factors for resorption in multivariate models were the use of external ventricular drains and lumbar shunts. CONCLUSIONS This is the largest study of pediatric cranioplasty outcomes performed to date. Analysis included variables found to be significant in previous retrospective reports. Presence of a cranial implant such as VP shunt is the most significant risk factor for cranioplasty infection, whereas younger age at cranioplasty is the dominant risk factor for bone resorption.
Article
Full-text available
General and central nervous system anatomy and physiology in children is different to that of adults and this is relevant to traumatic brain injury (TBI) and spinal cord injury. The controversies and uncertainties in adult neurotrauma are magnified by these differences, the lack of normative data for children, the scarcity of pediatric studies, and inappropriate generalization from adult studies. Cerebral metabolism develops rapidly in the early years, driven by cortical development, synaptogenesis, and rapid myelination, followed by equally dramatic changes in baseline and stimulated cerebral blood flow. Therefore, adult values for cerebral hemodynamics do not apply to children, and children cannot be easily approached as a homogenous group, especially given the marked changes between birth and age 8. Their cranial and spinal anatomy undergoes many changes, from the presence and disappearance of the fontanels, the presence and closure of cranial sutures, the thickness and pliability of the cranium, anatomy of the vertebra, and the maturity of the cervical ligaments and muscles. Moreover, their systemic anatomy changes over time. The head is relatively large in young children, the airway is easily compromised, the chest is poorly protected, the abdominal organs are large. Physiology changes—blood volume is small by comparison, hypothermia develops easily, intracranial pressure (ICP) is lower, and blood pressure normograms are considerably different at different ages, with potentially important implications for cerebral perfusion pressure (CPP) thresholds. Mechanisms and pathologies also differ—diffuse injuries are common in accidental injury, and growing fractures, non-accidental injury and spinal cord injury without radiographic abnormality are unique to the pediatric population. Despite these clear differences and the vulnerability of children, the amount of pediatric-specific data in TBI is surprisingly weak. There are no robust guidelines for even basics aspects of care in children, such as ICP and CPP management. This is particularly alarming given that TBI is a leading cause of death in children. To address this, there is an urgent need for pediatric-specific clinical research. If this goal is to be achieved, any clinician or researcher interested in pediatric neurotrauma must be familiar with its unique pathophysiological characteristics.
Article
Background Postnatal growth of neurocranium is prevalently completed in the first years of life, thus deeply affecting the clinical presentation and surgical management of pediatric neurosurgical conditions involving the skull. This paper aims to review the pertinent literature on the normal growth of neurocranium and critically discuss the surgical implications of this factor in cranial repair. Methods A search of the electronic database of Pubmed was performed, using the key word “neurocranium growth”, thus obtaining 217 results. Forty-six papers dealing with this topic in humans, limited to the English language, were selected. After excluding a few papers dealing with viscerocranium growth or pathological conditions not related to normal neurocranium growth 18 papers were finally included into the present review. Results and conclusions The skull growth is very rapid in the first 2 years of life and approximates the adult volume by 7 years of age, with minimal further growth later on, which is warranted by the remodeling of the cranial bones. This factor affects the outcome of cranioplasty. Thus, it is essential to consider age in the planning phase of cranial repair, choice of the material, and critical comparison of results of different cranioplasty solutions.