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).

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.

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.

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.

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.

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.

Intradiploic pseudomeningocele and ossified occipitocervical pseudomeningocele after decompressive surgery for Chiari I malformation: report of two cases and literature review.

Intradiploic cerebrospinal fluid (CSF) collections are rare findings. The authors describe two pediatric patients with iatrogenically induced occipital CSF collections after decompressive surgery for Chiari I malformation. The first patient presents a large occipital intradiploic pseudomeningocele and the second patient an intradiploic pseudomeningocele merging with an ossified occipitocervical pseudomeningocele. Though being rarities after decompression for Chiari I malformation, intradiploic fluid collection and ossified pseudomeningocele should be considered if patients represent with aggravating presurgical or new symptoms.

Constant dripping wears away the bone: intraosseous C2 and suboccipital intradiploic cerebrospinal fluid collection after Chiari I decompressive surgery.

The objective of decompressive surgery for Chiari I malformation is to create additional space to accommodate the ectopic cerebellar tonsils and to improve the flow of cerebrospinal fluid. To the best of our knowledge, we report the first case of a patient with previous Chiari I decompression presenting 5 years after surgery at the same time as an intraosseous C2 and a suboccipital intradiploic cerebrospinal fluid accumulation. Because of the progressive nature of the C2 cerebrospinal fluid collection causing bone destruction, surgical management was indicated. To avoid the erosion of the bone, we propose that the iatrogenic exposure of the diploe and the vertebral spongiosa that may come into contact with the cerebrospinal fluid should be sealed off with bone wax or a similar material.

Routine radiographs one day after anterior cervical discectomy and fusion are neither necessary nor cost-effective.

OBJECTIVES: Anterior cervical discectomy and fusion (ACDF) is a common operative treatment of compressive pathology of the cervical spinal cord, when caused by one or more degenerated intervertebral discs or related osteophytes. In addition to intra-operative radiographs to confirm spinal level before discectomy and implant position after insertion, traditional practice is to obtain post-operative antero-posterior and lateral plain radiographs (XR) before hospital discharge, despite a paucity of evidence supporting their benefit to patient care. Minimising unnecessary radiation to radiosensitive neck structures is desirable, and furthermore, with increasing financial pressure on healthcare resources, routine investigations should be clinically justified and evidence-based. We aim to compare the utility of routine post-operative cervical spine X-rays following ACDF. METHODS: We compare two groups of consecutive patients undergoing ACDF in a single UK neurosurgical centre. The first group (n = 109) received routine post-operative XR imaging, and the second group (n = 113) received radiographs only when clinically indicated. RESULTS: There were no differences in post-operative complication rates (4.6% vs. 5.3%), or requirement for further imaging or of further operative intervention (1.8% vs. 0.9%). The group that did not have routine post-operative radiographs had a significantly shorter stay in hospital (median two days vs. three days). There were no patients in either group where post-operative XR changed clinical management and mandated revision surgery or further imaging. All cases requiring surgery or further imaging were identified by clinical deterioration. CONCLUSIONS: We suggest that the practice of obtaining routine radiographs of the cervical spine following ACDF should be abandoned, unless there is a clear clinical indication.

Long-term morphological outcomes in nonsyndromic sagittal craniosynostosis: a comparison of 2 techniques.

Correction of scaphocephaly is one of the principle goals of surgery in sagittal craniosynostosis. Reported relapse in head shape after surgery and continued head growth into late adolescence underscores the need for long-term outcomes to be considered when comparing between different surgical approaches in this condition; yet there are relatively few reports of results to 5 years and beyond in the literature. Therefore, a retrospective review was performed of the anthropometric data of 224 patients with sagittal craniosynostosis who underwent primary surgery between 1994 and 2012. During this period, patients underwent either a modified strip craniectomy (MSC) or calvarial remodeling (CR) procedure. Sixty-two patients were treated by MSC and followed up for a mean of 44 months. One hundred sixty-two patients had CR, with follow-up for a mean of 45 months. Overall, 90 patients were seen up to 5 years, and 47 patients to 9 years or more after surgery. The cephalic index (CI) of MSC-treated patients improved from a mean of 67.0 to 72.7, with 31% achieving a CI greater than 75 at one year. Calvarial remodeling was significantly more effective at correcting the scaphocephalic deformity. Patients treated with CR improved from a mean CI of 66.7 to 76.1. Sixty-two percent of the patients achieved a CI greater than 75. In both groups, outcomes were stable throughout follow-up with no significant relapse up to 14 years after surgery.

Recurrent subdural hygromas after foramen magnum decompression for Chiari Type I malformation.

A paediatric case of foramen magnum decompression for Chiari Type I malformation complicated by recurrent subdural hygromas (SH) and raised intracranial pressure without ventriculomegaly is described. SH pathogenesis is discussed, with consideration given to arachnoid fenestration. We summarise possibilities for treatment and avoidance of this unusual consequence of foramen magnum decompression.

Split mesencephalon: diplomyelia of the basicranium.

We report a novel case of congenitally split mesencephalon, in a 3-year old with hydrocephalus. We speculate that the ontogenetic mechanism is shared with split cord malformations (SCM). Our case adds to the two other cases of basicranial SCM which involved more caudal brainstem.

Lessons learned in posterior cranial vault distraction.

INTRODUCTION: Posterior distraction (PD) is rapidly emerging as an important technique to increase the intracranial volume and correct calvarial morphology in patients with severe brachycephaly or turribrachycephaly. METHODS: A retrospective review was performed of all 31 patients who underwent PD at the Oxford Craniofacial Unit between 2007 and 2012. RESULTS: Twenty-three patients (74.2%) underwent PD as a primary procedure at a median age of 8 months. Eight patients (25.8%) had PD as a secondary transcranial procedure at a median age of 48 months. Full distraction to 20 mm was achieved in 28 patients (90.3%). Of these, all but 1 demonstrated a significant improvement in morphology, with a resolution of the symptoms and signs of raised intracranial pressure in all proven to have it preoperatively. Unanticipated events occurred in 61.3% of patients, with 19.4% undergoing one or more unplanned procedures. Wound infection (29.0%) and tissue necrosis (22.6%) were the commonest. Cerebrospinal fluid leaks were rarer (6.5%) but prevented full distraction. Nine patients (29.0%) had a consolidation period of less than 30 days without experiencing relapse. In 11 patients who had a later fronto-orbital advancement and remodeling, wound closure was tight, resulting in dehiscence in 3 cases (27.3%). CONCLUSIONS: Posterior distraction is an effective procedure in the management of severe brachycephaly or turribrachycephaly but has associated risks. Our protocol has evolved with experience to favor a reduced latency period and consolidation phase and the use of 2 distractor devices.

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.