Symptomatic cerebral vasospasm after posterior fossa surgery in pediatric patients: single-center study and systematic literature review.

PURPOSE: The most common cause of cerebral vasospasm is subarachnoid hemorrhage, less frequently it occurs after trauma, infection, and tumor resection. Vasospasm in children is rare and has not been systematically investigated in posterior fossa surgery. METHODS: The authors undertook a single-center retrospective study of all the pediatric patients who underwent surgery on the posterior fossa and presented with postoperative symptomatic vasospasm in the period from January 2018 to February 2024. Subsequently, a systematic literature review in accordance with the PRISMA guidelines was performed in the PubMed and Scopus databases to identify the published papers on symptomatic vasospasm after posterior fossa surgery in children. RESULTS: Of the 178 patients who underwent surgery on the posterior fossa, only one patient was diagnosed with symptomatic diffuse vasospasm on postoperative day 21. The systematic literature review provided further 9 children. The underlying pathology comprised 8 intra-axial lesions with 4 medulloblastomas, 1 schwannoma in the medulla oblongata, 1 pilocytic astrocytoma, 1 primitive neuroectodermal tumor, and 1 arteriovenous malformation. The extra-axial lesions were 1 hypoglossal schwannoma and 1 oculomotor nerve schwannoma. CONCLUSION: Iatrogenic symptomatic vasospasm after posterior fossa surgery in children is a rare complication with an outcome ranging from complete recovery to the death of the patient. It is important for all staff involved in the care of patients undergoing surgery on the posterior fossa to be aware of this rare postoperative complication. The small number of patients affected does not allow a substantiated conclusion to be drawn about predictive risk factors.

Safety and effectiveness of Evicel® fibrin sealant as an adjunct to sutured dural repair in children undergoing cranial neurosurgery.

PURPOSE: Cerebrospinal fluid (CSF) leakage is a challenging complication of intradural cranial surgery, and children are particularly at risk. The use of dural sealants confers protection in adults, but pediatric studies are scarce. We evaluated the safety and efficacy of Evicel® fibrin sealant as an adjunct to primary dural suturing in children undergoing cranial surgery. METHODS: A multicenter trial prospectively enrolled pediatric subjects (< 18 years) undergoing cranial neurosurgery who, upon completion of primary sutured dural repair, experienced CSF leakage. As agreed by the EMA Evicel® Pediatric Investigation Plan, 40 subjects were intra-operatively randomized 2:1 to Evicel® or additional sutures ('Sutures'). Data analysis was descriptive. The efficacy endpoint was treatment success rate, with success defined as intra-operative watertight closure after provocative Valsalva maneuver (primary endpoint). Safety endpoints were postoperative CSF leakage (incisional CSF leakage, pseudomeningocele or both) and surgical site complications (secondary endpoints). RESULTS: Forty subjects (0.6-17 years) were randomized to Evicel® (N = 25) or Sutures (N = 15) (intention-to-treat). Intracranial tumor was the most common indication and procedures were mostly supratentorial craniotomies. Success rates were 92.0% for Evicel® and 33.3% for Sutures, with a 2.76 estimated ratio of success rates (Farrington-Manning 95% CI [1.53, 6.16]). Sensitivity analyses in per-protocol and safety sets showed similar results. Despite a higher rescue treatment rate, the frequencies of postoperative CSF leakage and wound complications were higher for Sutures than for Evicel®. CONCLUSION: This small-scale prospective study shows Evicel® treatment to be safe and effective as an adjunct to primary sutured dura mater closure in a pediatric population. Compared to additional sutures, Evicel® was associated with reduced postoperative CSF leakage and surgical site complications. (Trial registration: The trial was registered as NCT02309645 and EudraCT 2013-003558-26).

Imaging in Craniofacial Disorders With Special Emphasis on Gradient Echo Black-Bone and Zero Time Echo MRI Sequences.

Context: The well-known effects of ionizing radiation on brain cells have been a major driving force toward the use of non-ionizing methods of imaging in both elective and emergency settings. Pediatric neurosurgery has certainly leveraged on this shift in clinical practice, however patients with craniofacial disorders could not fully benefit from the adoption of magnetic resonance imaging (MRI) because computed tomography (CT) scans still retain superior imaging power on bone tissue. Aims: To explore the knowledge available on the use of MRI as surrogate for CT scan in the assessment of craniosynostosis. Settings and Design: A scoping review was designed to identify landmark studies and ongoing clinical trials exploring the accuracy of MRI-based bone imaging in the preoperative planning of pediatric patients with craniosynostosis. Materials and Methods: A total of 492 records were screened from Pubmed, Ovid Medline, Scopus, and Cochrane Library databases; while 55 records were retrieved from ClinicalTrials.gov register. Only clinical studies revolving around the use of Gradient Echo Black-Bone (BB) and Zero Time Echo (ZTE) MRI sequences for the preoperative planning of pediatric craniosynostosis were retained for inclusion. Results and Conclusions: This review identified only five clinical studies reporting a high accuracy of MRI-based 3D bone reconstruction in 47 pediatric candidates to surgical correction of craniosynostosis. Although promising, limited evidence (Level IV) exist that BB and ZTE MRI could help in the surgical planning for craniosynostosis management. The results of two ongoing randomized clinical trials, which are actively enrolling patients, will hopefully help answering this research question.

Neuroendoscopic lavage for the management of neonatal post-haemorrhagic hydrocephalus: a retrospective series.

INTRODUCTION: Intraventricular haemorrhage (IVH) is a common complication of preterm birth, and optimal treatment remains uncertain. Neuroendoscopic lavage (NEL) has gained interest as a method for removal of intraventricular haematoma, with outcomes suggesting it to be safe and potentially effective. METHODS: A retrospective review was carried identifying infants who underwent NEL for post-IVH hydrocephalus at our institution. Data was extracted on patient baseline demographics, comorbidities, complications, re-operation requirement, and neurodevelopmental outcomes. RESULTS: Twenty-six patients (17 male) were identified, who underwent NEL at a mean age of 39 weeks and 4 days. Eighteen patients underwent simultaneous endoscopic third ventriculostomy (ETV). Mean patient follow-up was 57.7 months ± 11.8 months. A total of 17/26 patients went on to require a ventriculoperitoneal shunt (VPS). Nine patients did not require further surgical management of hydrocephalus; all had been managed with NEL + ETV. The relative risk of requiring VPS with NEL + ETV compared with NEL alone was 0.500 (CI: 0.315-0.794; p = 0.0033). The 24-month survival rate of VPS inserted following NEL was 64.7%. Exactly 5/26 (19.2%) had post-procedure complications: 2 CSF leaks (7.7%), 2 infections (7.7%), and 1 rebleed within 72 h of NEL (3.8%). On long-term follow-up, 22/25 patients achieved good motor outcome, either walking independently or with mobility aids. A total of 8/15 children attended mainstream schooling with adaption. DISCUSSION: NEL is safe and potentially efficacious treatment for neonatal IVH. The procedure may reduce shunt dependence and, for those who require CSF diversion, improve shunt survival. Neurodevelopmentally, good motor and cognitive outcome can be achieved.

Iatrogenic syringomyelia postcranial surgery in pediatric patients: systematic review and illustrative case.

INTRODUCTION: Iatrogenic syringomyelia has previously been described preoperatively due to space occupying lesions and postoperatively predominantly postspinal CSF drainage. Iatrogenic syringomyelia after cranial surgery is a rare entity. The purpose of this study is to review the current literature for iatrogenic syringomyelia following cranial surgery in children. MATERIAL AND METHODS: We performed a systematic review in accordance with the PRISMA (Preferred Reporting Items for Systematic Reviews and meta-analyses) guidelines. Using keywords "de novo," "acquired," "iatrogenic," "postoperative development," and "syringomyelia," we searched PubMed and Scopus databases. We paid particular attention to type of surgery, clinical presentation, management, and outcome. We also present a relevant case report, operated on at John Radcliffe Hospital, Oxford. RESULTS: The systematic literature review provided 19 cases of iatrogenic syringomyelia postcranial surgery in pediatric patients, in addition to our case report, resulting in a total of 20 cases reported. The patients' mean age at surgery preceding syringomyelia was 8.1 years (range 0.1-17) with a female sex predilection (2.7:1). The mean time of manifestation of the iatrogenic syringomyelia after surgery was 5.7 years (range 0.1-51), and the median time to presentation was 1.7 years. Nine children had surgery for Chiari I malformation before formation of the syrinx, 8 patients underwent shunt surgery, 2 children had intracranial tumor surgery, and 1 patient had lambdoid and sagittal suturectomy for scaphocephaly before developing a syrinx. Surgery after the manifestation of the iatrogenic syrinx resolved the symptoms in 9 patients; in 8 patients, the symptoms partially resolved; and in 1 patient, the symptoms persisted. After surgery addressing the syrinx, it resolved in 5, decreased in size in 10, and remained unchanged in 2 of the patients. CONCLUSION: Iatrogenic syringomyelia after cranial surgery in pediatric patients occurs mainly after decompressive surgery for Chiari I malformation, shunt surgery, and less frequently following intracranial tumor surgery. Risk factors are postoperative intracranial scarring, shunt malfunction, and mass effect on the foramen magnum. Surgery addressing the iatrogenic syrinx seems to be effective in the majority of the patients in terms of clinical and radiological outcome.

Pre-operative imaging and post-operative appearance of standard paediatric neurosurgical approaches: a training guide for neuroradiologists.

A short-cut narrative review was conducted according to the SANRA guidelines to identify studies describing normal and abnormal postoperative radiological features of the most common paediatric neurosurgical procedures. Rather than focusing on the original pathology addressed by neurosurgical means, this review explored three main areas of operative neurosurgery: ventricular access, supratentorial & infratentorial craniotomies, and posterior fossa/craniocervical junction decompression. A total of twenty-three landmark papers were included for review based on their relevance to address the research question and serve as a practical guide for paediatric neuroradiology trainees and fellows. Accurate in text referencing of the ClinicalTrials.gov identifier, and weblink, has also been provided for all trials discussed in the results section. All the above is complemented by relevant iconography meant to describe a wide range of postoperative changes and early complications. Finally, the review is enriched by a discussion touching upon haemostatic agents, intentionally retained foreign bodies and the future of machine learning for neuroradiology reporting. Overall, the information presented in a systematic fashion will not only help trainees and fellows to deepen these topics and expand their knowledge in preparation for written and oral boards, but will also represent a useful resource for everyone including trained neuroradiologists and neurosurgeons themselves.

An Integrated Approach to the Management of Meningoencephalocoeles.

ABSTRACT: Meningoencephalocoeles are congenital herniations of meningeal and cerebral tissues through a cranial defect. They occur most commonly in South-East Asia, and are relatively rare amongst European ancestry populations, with an estimated prevalence of 1/40,000 live births. The treatment of congenital meningoencephalocoeles is primarily surgical and are best managed by dedicated multi-disciplinary craniofacial teams. The authors performed a retrospective case review of all primary meningoencephalocoeles managed in the Oxford University Hospitals NHS Foundation Trust between 1986 and 2012. Twenty-nine cases (13 frontal, 9 occipital, 2 parietal, and 5 basal) were included in this study. The median age at presentation was 11 months (range 0-60 years). Twenty-five cases presented with an external mass; 3 with recurrent meningitis and 1 with otorrhoea. Twenty-six cases underwent surgery, and 17 of these were managed by an integrated approach between 2 or more surgical specialties. Twenty out of 26 operations were performed via a transcranial approach. The authors describe a particularly complex case in order to highlight the challenges associated with management of meningoencephalocoeles, the surgical technique employed, and the importance of a multidisciplinary surgical approach. This is the largest reported case series of meningoencephalocoeles managed in a single hospital in the United Kingdom. Designated craniofacial units with access to multidisciplinary surgical specialties provide a safe and optimal setting for the management of meningoencephalocoeles.

Feeding, Communication, Hydrocephalus, and Intracranial Hypertension in Patients With Severe FGFR2-Associated Pfeiffer Syndrome.

BACKGROUND: Pfeiffer syndrome is associated with a genetic mutation of the FGFR2 (or more rarely, FGFR1) gene, and features the combination of craniosynostosis, midface hypoplasia, broad thumbs and broad great toes. Previous research has identified a wide spectrum of clinical phenotypes in patients with Pfeiffer syndrome. This study aimed to investigate the multifactorial considerations for speech, language, hearing and feeding development in patients with severe genetically-confirmed Pfeiffer syndrome. METHODS: A 23-year retrospective case-note review of patients attending the Oxford Craniofacial Unit was undertaken. Patients were categorized according to genotype. Patients with mutations located in FGFR1, or outside the FGFR2 IgIII domain-hotspot, or representing known Crouzon/Pfeiffer overlap substitutions were excluded. Twelve patients with severe FGFR2-associated Pfeiffer syndrome were identified. RESULTS: Patients most commonly had pansynostosis (n = 8) followed by bicoronal (n = 3), and bicoronal and sagittal synostosis (n = 1). Seven patients had a Chiari I malformation. Four patients had a diagnosis of epilepsy. Ten patients had with hydrocephalus necessitating ventriculoperitoneal shunt insertion.Feeding difficulties were common (n = 10/12) and multifactorial. In 5/12 cases, they were associated with pansynostosis, hydrocephalus, tracheostomy and tube feeding in infancy.Hearing data were available for 10 patients, of whom 9 had conductive hearing loss, and 8 required hearing aids. Results indicated that 3/4 patients had expressive language difficulties, 3/4 had appropriate receptive language skills. 6/12 patients had a speech sound disorder and abnormal resonance. CONCLUSION: This study has identified important speech, language, hearing and feeding issues in patients with severe FGFR2-associated Pfeiffer syndrome. Results indicate that a high rate of motor-based oral stage feeding difficulties, and pharyngeal stage swallowing difficulties necessitating regular review by specialist craniofacial speech and language therapists.

Central Retinal Artery Occlusion Following Prone Transcranial Surgery for Craniosynostosis and Discussion of Risk Factors.

Vision loss is a known rare complication of prone positioning during surgery. Vision loss following prone surgery is most commonly attributed to direct pressure on the eye but can also be caused by central retinal artery occlusion (CRAO) in the absence of pressure on the eye. Central retinal artery occlusion has not been previously described following prone transcranial surgery for craniosynostosis. We present two cases of monocular CRAO following prone calvarial expansion. A multidisciplinary root cause analysis suggested that raised intracranial pressure and intraoperative tranexamic acid may have been risk factors for the development of CRAO in these cases as no conventional risk factors for CRAO following prone surgery were present. Because of this, we retrospectively reviewed all prone transcranial procedures performed at the Oxford Craniofacial Unit for the presence of raised intracranial pressure and intraoperative tranexamic acid use. A total of 662 prone procedures have been performed between 1994 and March, 2019. Tranexamic acid has been used routinely in all transcranial procedures since 2012 and in the last 311 consecutive prone cases. Fifty-one (7.7%) prone procedures were performed for raised intracranial pressure, and tranexamic acid was used in the 33 most recent of these. Since the implementation of standard intraoperative administration of tranexamic acid there have been 2 cases of CRAO following prone surgery. The overall incidence of CRAO was 0.3% but was 6% in the context of raised intracranial pressure and tranexamic acid use. Prone positioning raised intracranial pressure and tranexamic acid use together may represent a potent combination of risk factors for CRAO.

CSF goes astray no matter what is in the way: occipital intradiploic pseudomeningocele and de novo syringomyelia following foramen magnum decompression for Chiari I malformation in a pediatric patient.

Nontraumatic intradiploic pseudomeningoceles and de novo syringomyelia formation are very rare entities. The authors have previously reported the case of a 4-year-old girl who underwent foramen magnum decompression without dural closure for Chiari I malformation. Three years after the operation an intradiploic pseudomeningocele was documented, but the patient was lost to follow-up without undergoing revision surgery. Four years later, at the age of 11 years, the patient returned for treatment of intensifying symptoms. Radiological imaging then showed an increase in the size of the intradiploic pseudomeningocele and a new cervical syrinx. The patient underwent a first revision surgery in which a part of the internal layer of the occipital bone was removed and arachnoid scar lysis was performed. Two months later the syrinx had worsened, and in a second revision surgery a pseudomeningocele-peritoneal shunt was placed. Here, the authors describe what is to their knowledge the first case of an intradiploic pseudomeningocele and de novo syringomyelia formation following Chiari I decompressive surgery.

Language Development, Hearing Loss, and Intracranial Hypertension in Children With TWIST1-Confirmed Saethre-Chotzen Syndrome.

Saethre-Chotzen syndrome (SCS) is an autosomal dominant condition defined by mutations affecting the TWIST1 gene on chromosome 7p21.1. Previous research has identified an elevated prevalence of intracranial hypertension and hearing impairment associated with this syndrome. This study aimed to investigate the influence of hearing history and presence of intracranial hypertension on language development in children with SCS.A retrospective study note analysis was performed for all patients with a confirmed TWIST1 gene abnormality who attended the Oxford Craniofacial Unit and underwent a language assessment over a 22-year period. Intracranial pressure monitoring, hearing status, and language outcomes were examined in detail.Thirty patients with genetically confirmed SCS and language assessment data were identified. Twenty-eight patients underwent surgical intervention; 10 presented with intracranial hypertension (5 prior to, and 5 after primary surgical intervention). Language data coinciding with the presentation of intracranial hypertension were available for 8 children. About 44% of children with intracranial hypertension presented with concurrent receptive and expressive language delay (n = 4/8). For both children (n = 2) with longitudinal language data available, the onset of intracranial hypertension reflected a concurrent decline in language skills. Audiometric data were available for 25 children, 80% (n = 20/25) had a history of hearing loss. About 50% of these had confirmed conductive hearing loss with middle ear effusion and the other 50% had presumed conductive hearing loss with middle ear effusion. About 100% of the children with available hearing data in our study had evidence of middle ear effusion in at least 1 ear. Results also indicated that 43% (n = 13/30) of the children presented with receptive and/or expressive language delay during childhood.Given the importance of hearing for language development and the preliminary findings of a potential decline in language skills in children during periods of intracranial hypertension, regular follow-up of hearing, language, and intracranial hypertension are indicated in children with SCS.

Decompressive surgery for Chiari I malformation in children without dural repair: a still effective and safe procedure?

PURPOSE: There are numerous publications about the technical aspects of decompressive surgery for Chiari I malformation highlighting many variations of this procedure. Each approach has its followers. Bony decompression of the foramen magnum alone or with the removal of a portion of the posterior arch of C1, dural splitting with keeping arachnoid intact, and durotomy are described. Dural closure is done with various materials. We retrospectively reviewed foramen magnum decompression without dural repair (FMDWDR) following the technique used by Gardener and Williams as an option in pediatric patients with Chiari I malformation in terms of complication rate and clinical outcome. METHODS: The surgical database of our unit identified 65 consecutive children who underwent FMDWDR surgery for Chiari I malformation between 2009 and 2016. The retrospective assessment included patient demographics, clinical data, surgical technique, revision rate, complications, and clinical outcome. RESULTS: Durotomy without repair was performed in 65 patients. Complications included aseptic meningitis and subdural hematoma respectively in three cases, intradiploic CSF collections in three patients, and CSF leaks in six children. The CSF leak rate has reduced dramatically after introducing a technical modification. Revision surgery was performed in seven cases. None of the patients was identified with postoperative hydrocephalus or infection. There was no mortality and no long-term surgical morbidity. In terms of clinical outcome, 52 patients reported postoperative improvement, 10 were clinically unchanged, and three noticed worsening of symptoms. CONCLUSION: Applying a "T"-shaped fascial incision which allows a watertight closure of the fascia FMDWDR is still a safe and effective treatment option for Chiari I malformation in children.

Chiari 1 malformation and raised intracranial pressure.

BACKGROUND: The pathophysiology of Chiari 1 malformation (CM1) is inextricably related to intracranial pressure (ICP). The characteristic cerebellar tonsil herniation at the foramen magnum may either cause raised ICP by disturbing CSF flow (as observed in idiopathic CM1) or may itself be the effect of raised ICP (as observed in acquired CM1). Distinguishing between these two phenomena, therefore, is of paramount importance in successfully alleviating the symptoms of the condition and preventing serious complications. OBJECTIVES: In this article, we discuss the pathophysiology of raised ICP in CM1 and review the current evidence for its investigation and treatment. We also share our own clinical experience which investigates the utility of ICP monitoring in a series of 26 children with CM1.

Multisuture Craniosynostosis and Papilledema in Pycnodysostosis: A Paradox?

Pycnodysostosis (PYCD) is a rare autosomal-recessive skeletal disorder that typically presents with osteosclerosis of the majority of the postcranial skeleton and osteolysis of the calvarium, manifesting as persistent open cranial fontanelles and widely spaced cranial sutures. Craniosynsostosis in PYCD is a somewhat paradoxical feature, and has only been rarely reported. The authors present a unique case of a 6-year-old girl with PYCD, multisuture craniosynostosis involving the coronal and sagittal sutures, severe obstructive sleep apnoea, and raised intracranial pressure presenting as papilledema. She underwent a frontofacial monobloc distraction advancement which successfully corrected her papilledema and obstructive sleep apnoea.Pycnodysostosis is caused by a loss of function mutation in the CTSK gene that codes for the lysosomal cysteine protease, cathepsin K (CTSK). Loss of CTSK impairs the ability of osteoclasts to degrade bone extracellular matrix. Differences in osteoclast phenotype and extracellular matrix composition between membranous and cartilaginous bone may explain the clinical features of PYCD. Animal model studies suggest that craniosynostosis may arise due to variations in patient genetic background.

Long-Term Results in Isolated Metopic Synostosis: The Oxford Experience over 22 Years.

BACKGROUND: Metopic synostosis causing trigonocephaly is treated by fronto-orbital advancement and remodeling to correct the deformity and cerebral distortion and to treat intracranial hypertension in a small number of cases. The aim of this study was to evaluate complications, revisions, and long-term outcomes. METHODS: A retrospective chart review was performed on consecutive metopic craniosynostosis patients treated between February of 1995 and February of 2017 at the Oxford Craniofacial Unit. RESULTS: Two hundred forty-five patients with isolated metopic synostosis were seen. Two hundred two patients underwent fronto-orbital advancement and remodeling. Fifty patients were girls and 152 patients were boys. Mean age at surgery was 16.8 months. Mean weight preoperatively was 12 kg. All patients received blood transfusion. Mean postoperative stay was 6 days. Average follow-up time was 8 years (range, 0.5 to 22 years). There were eight major complications (4 percent). Six patients (2.9 percent) required secondary calvarial expansion for late raised intracranial pressure. Thirty-one (15 percent) had other subsequent procedures, including wire removal and forehead shape contouring with alloplastic onlay. Raised intracranial pressure before surgery was confirmed in two cases by intracranial pressure monitoring. CONCLUSIONS: Trigonocephaly is caused by metopic synostosis and is treated by fronto-orbital advancement and remodeling to restore both internal and external skull configuration. After surgery, the authors identified a 2.9 percent risk of late raised intracranial pressure requiring a secondary calvarial expansion, necessitating prolonged follow-up in all cases. Temporal hollowing and forehead contour defects were not uncommon. This is the largest reported series of metopic synostosis. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Should all paediatric patients with presumed idiopathic scoliosis undergo MRI screening for neuro-axial disease?

BACKGROUND: Idiopathic scoliosis is a relatively common childhood condition affecting 0.47-5.2% of the population. Traditional interventions focus on orthopaedic correction of the curve angle. There is a spectrum of patients with scoliosis who are found to have neuro-axial abnormality on full MRI of the spine, but not all surgeons request imaging in the absence of neurological symptoms. There is evidence to suggest that treatment of neuro-axial disease may improve scoliosis curve outcome. We therefore sought to estimate what proportion of patients with normal neurology and scoliosis are found to have neuro-axial abnormality on full MRI imaging of the spine, in particular Chiari malformation and syringomyelia. RESULTS: Out of 11 identified studies consisting of 3372 paediatric patients (age < 18 years), mean weighted proportion demonstrates that 14.7% of patients with scoliosis (Cobb angle > 20°) and normal neurological examination will demonstrate a neuro-axial abnormality on full MRI imaging of the spine. Of patients, 8.3 and 8.4% were found to have Chiari malformation and syringomyelia, respectively. CONCLUSIONS: Up to one in seven paediatric patients with scoliosis and normal neurological examination will demonstrate neuro-axial disease on MRI imaging of the spine. Given that younger age and earlier age of decompression is associated with improvement in curve angle, it seems important that MRI screening be considered in all patients regardless of neurological examination findings. There is a potentially long-term benefit in these patients. Multi-cross institutional prospective studies are encouraged to further investigate effect on curve angle.