Piezosurgery in endoscopic-assisted trigonocephaly correction: a technical note.

PURPOSE: This study aims to evaluate the effectiveness of the Piezosurgery® device in endoscopic-assisted correction of trigonocephaly. Trigonocephaly is a type of craniosynostosis characterized by a triangular-shaped forehead due to the premature fusion of the metopic suture. Traditional open cranial vault reconstruction, although common, is invasive and poses risks. The study explores a less invasive alternative using ultrasonic microvibrations for bone cutting, potentially reducing soft tissue damage and improving surgical outcomes. METHODS: The Piezosurgery® device was employed in endoscopic trigonocephaly correction surgeries performed on patients under 4 months old at the French Referral Center for Craniosynostosis in Lyon. The technique involves making a small skin incision and performing osteotomies from the anterior fontanel to the glabella. A rigid 0° endoscope provides visibility, and the Piezosurgery® device enables precise bone cutting while preserving the dura mater. Post-surgery, patients were discharged within 3 days and required to wear a remodeling helmet for 6-8 months. RESULTS: The use of Piezosurgery® device allowed precise osteotomies with minimal soft tissue damage. No dura mater injuries occurred in the patient series. The procedure was efficient, with an average duration of 80 min, and blood loss was minimal, reducing the need for blood transfusions. The endoscopic approach facilitated shorter surgical times and reduced postoperative infection risks. Enhanced visibility during surgery, due to cavitation effects, improved the accuracy of bone cuts. The technique demonstrated promising safety and esthetic outcomes, although it incurred higher costs compared to traditional methods. CONCLUSION: Piezosurgery® device provides a safe and effective method for minimally invasive endoscopic correction of trigonocephaly. The device's ability to selectively cut bone while preserving soft tissues offers significant advantages, despite longer surgical times and higher costs. This technique represents a viable alternative to traditional open surgery, promoting better clinical outcomes and reduced recovery times.

Role of sodium fluorescein in pediatric low-grade glioma surgery: an update.

PURPOSE: Complete surgical resection is still the mainstay in the treatment of central nervous system low-grade tumors, eventually resulting curative. The complete surgical removal of these lesions, however, may be difficult in some cases because of their infiltrative nature. Intraoperative adjuncts may be a game changer. Sodium fluorescein (SF) is among the ideal candidates as intraoperative tools to favor the actual recognition of the tumor extension, since it accumulates in areas of altered blood-brain barrier, a typical characteristic of pediatric gliomas, and has a low rate of adverse events. This work proposes an update of previous works about the evaluation of the feasibility and usefulness of a systematic use of SF in a low-grade lesion group of pediatric patients. METHODS: Pediatric patients operated on for a resection or a biopsy of a low-grade glial or glioneuronal lesion (WHO grade I and II) at our Institution between September 2021 and December 2023, with the intraoperative use of sodium fluorescein (SF), were enrolled in the study. We collected pre-operative and postoperative clinical and radiological data, intraoperative findings, and post-operative pathological diagnoses. RESULTS: No adverse events were registered related to the intraoperative use of SF. SF appeared useful for the localization of boundaries of tumors, especially when characterized by a high degree of infiltration or by a deep-seated location, and for the checking of possible tumor remnants at the end of surgery. A good tumor-to-healthy tissue contrast was registered when tumor visualization was in a range between 1 to 2 h and 30 min after SF injection. Possible "false positives" due to intraoperative vascular wall injury and clearance of SF from both tumor and healthy tissue were observed in some cases and still remain open issues. CONCLUSIONS: SF is a feasible and safe intraoperative adjunct tool in the surgical removal of pediatric low-grade tumors. SF may show its usefulness especially in selected cases, such as deep-seated lesions and infiltrating tumors. Its safety profile, user-friendly management, and potential utility in both tumor resections and neuronavigated biopsies favor its wider use in the surgical treatment of pediatric low-grade tumors.

Sodium fluorescein in pediatric neurosurgery: a systematic review with technical considerations and future perspectives.

BACKGROUND: Sodium fluorescein (SF) is routinely used in several centers as a valid intraoperative adjunct in adult oncological neurosurgery. Its use in pediatric neurosurgery is increasing, although its role is not yet well-defined in children. We reviewed the current literature in order to evaluate the use of SF in children with CNS and PNS lesions. METHODS: For this systematic review, we searched PubMed, Scopus, and Embase databases, and forward and backward citations for studies published between database inception and July 31st, 2022. We included any article type or congress abstract adding at least a new case, without restrictions of language or publication status, concerning the use of SF in neurosurgical procedures in patients under 18 years of age. We excluded studies concerning purely vascular cases and cerebrospinal fluid leaks. RESULTS: Of 4094 records identified, 19 articles were eligible and included for further analysis. As per July 31st, 2022, at least 119 patients aged from 11 months to 17.9 years underwent surgery with SF. No serious adverse events were reported. A large variety of tumor types was operated, in most cases resected under the specific YELLOW 560 nm filter after a low-dose SF injection (2-5 mg/kg) at the end of anesthesia induction. SF was reported particularly useful in gangliogliomas and pilocytic astrocytomas. DISCUSSION/CONCLUSION: Given its easy-to-use profile, low cost, and safety, SF seems to be a feasible and valid adjunct in the pediatric population when aiming at individuating a biopsy target or maximizing extent of resection, particularly in some tumor types. Further studies are required to strengthen the evidence on its impact on outcomes.

Sodium fluorescein in pediatric oncological neurosurgery: a pilot study on 50 children.

BACKGROUND: Sodium fluorescein (SF) is currently considered a valid intraoperative adjunct in the resection of high-grade brain lesions in adults. Experiences in pediatric groups and in low-grade gliomas and other low-grade lesions are still limited in literature, and subjective evaluation of fluorescence is still a limitation. MATERIAL AND METHODS: This study retrospectively reviewed all patients with brain or spine lesions operated on from September 2021 to July 2022 in the Pediatric Neurosurgery Unit of Hôpital Femme Mère Enfant, Lyon, who had received 5 mg/kg of 10%. Surgery was performed using a YELLOW560 filter at crucial times. At the end of surgery, the first operator completed a questionnaire, including his opinion on whether SF had been useful in tumor resection, recorded as a binary variable. Post hoc, surgical images were reviewed using ImageJ, an open-source Java image processing platform. In order to compare independent discrete variables, we applied the Student's t test, and we applied the Chi-square or Fisher exact test for binary variables. A threshold of p < 0.05 was set for statistical significance. RESULTS: We included 50 pediatric patients (0.2-17.6 years old). Forty/50 lesions showed SF uptake (80%). The differentiation between healthy and affected tissue, thanks to SF, subjectively evaluated by the surgeon, had as objective counterpart the statistically significant higher brightness of green in lesions, registered by the software (p < 0.001). SF overall allowed a good differentiation in 33/50 lesions, and overall utility of SF has been noted in 67% of them. When specifically considering gliomas, overall utility reached 75%. CONCLUSION: SF is a feasible, safe, and useful intraoperative adjunct in pediatric neurosurgery. In particular, it seems to have a promising role in some low-grade infiltrating glial tumors. The subjective evaluation of fluorescence seems to be reliable with respect to image analyses software.

Auricles Anomalies in Patients With a TCF12 Gene Mutation.

Craniostenosis is a morphological anomaly affecting about 0.5 of 1000 births and one third of the cases are of genetic origin. Among the syndromes responsible for craniostenosis, there is the Saethre-Chotzen syndrome due to a mutation of the TWIST 1 gene located on chromosome 7. This polymalformative syndrome classically includes a particular morphology of the auricles. The penetrance is variable and results in a phenotypic variability at the origin of "Saethre-Chotzen like" clinical pictures for which the TWIST 1 gene mutation is sometimes not found. Recently, the TCF 12 gene has been implicated in some of these cases. Among the multiple facial malformations, we have carefully examined the particular morphology of the auricle of these patients. The authors found several abnormalities in patients with a TCF 12 gene mutation, namely a thickened and hammered upper pole of the helix, a narrow concha without crux cymbae and a thickened lobe. These morphological features may guide the diagnosis and allow an earlier search for a TCF 12 gene mutation.

Answer to Squier et al.

The author responds to critics formulated by W Squier regarding our paper "The legal challenges to the diagnosis of shaken baby syndrome Or: how to counter 12 common fake news.

Abusive head injuries in infants: from founders to denialism and beyond.

INTRODUCTION: Abusive head injuries is a major cause of severe morbidity and the main cause of mortality by head trauma in infants. MATERIAL AND METHODS: Based on published data and their own clinical and medicolegal practice, the authors review briefly the historical roots and emergence of the concept of abusive head injuries (AHI), until the present scientific understanding of shaken baby syndrome (SBS) and Silverman syndrome. They then discuss the present epidemic of denialism and how this challenge to science should be seen as a stimulus to increase research and improve the accuracy of diagnosis and medical practice. RESULTS: The denial of SBS is especially damaging because it undermines the possibilities of prevention and reparation for victims. The authors expand on AHI being part of a wider context of domestic violence and the prevention of child abuse being part of a broad and long-term endeavor to defend civilization values. CONCLUSIONS: Prevention of AHI is a major challenge for the future. In the fields of science and prevention of child abuse, the input of pediatric neurosurgeons should not be underestimated.

The role of the pediatric neurosurgeon in abusive head injuries: a survey of members of the International Society for Pediatric Neurosurgery.

BACKGROUND AND PURPOSE: Pediatric neurosurgeons are at the forefront of the clinical management of abusive head injuries (AHI) all over the world. However, the discrepancies regarding medical practice and legal requirements in different centers have not been assessed before. MATERIAL AND METHODS: We decided to perform an online survey among members of the International Society for Pediatric Neurosurgery (ISPN) regarding their activity, their usual clinical practice regarding the medical and legal management of AHI, and their involvement in judiciary proceedings, research, and prevention campaigns. RESULTS: Ninety members of the ISPN participated in the survey, representing 26% of the registered members. Most responders were senior pediatric neurosurgeons practicing in a university hospital. Their responses show great homogeneity regarding surgical, intensive care, and legal management. We also noted the widespread use of invasive intracranial pressure monitoring and decompressive craniotomy. By contrast, the responses show great disparity regarding the circuit of patients. This disparity may be an obstacle to the collection of data and clinical research, as well as for the involvement of neurosurgeons in multidisciplinary evaluation. A minority of neurosurgeons were engaged in research and actions of prevention. CONCLUSION: Pediatric neurosurgeons play a pivotal role in the diagnosis and medical management of AHI. Because of their proficiency in head trauma and cerebrospinal fluid disorders, it is desirable that they become more implicated in multidisciplinary meetings, medical expertise, and scientific research, as well as actions of prevention.

Cerebellar mutism syndrome in pediatric head trauma with cerebellar injury.

PURPOSE: Cerebellar mutism syndrome (CMS) after cerebellar injury in pediatric head trauma is a poorly recognized condition that is not properly diagnosed or treated in our daily practice. We aimed to clinically identify this syndrome after isolated posttraumatic cerebellar injury and to propose pathophysiological explanation. METHODS: We retrospectively analyzed 8 consecutive children presenting with isolated cerebellar injury over 16 years. Clinical presentation, radiological type and localization of injury, clinical initial CMS symptoms, and long-term neurocognitive outcome were reviewed. RESULTS: Out of 8 patients presenting with isolated traumatic cerebellar injury, we diagnosed 2 cases with initial clinical symptoms of CMS. Both patients had an injury damaging median structures of the posterior fossa, especially the fourth ventricle and dentate nuclei. Initial symptoms lasted more than 1 month for one patient, who still presented concentration difficulties almost 1 year after the head injury. CONCLUSION: CMS after traumatic cerebellar injury does exist, even if it seems to be a very rare entity. It has to be better detected and studied in order to enrich pathophysiological knowledge about CMS of all etiologies and to bring our concerned patients the suitable follow-up and rehabilitative care that they could benefit from.

The legal challenges to the diagnosis of shaken baby syndrome or how to counter 12 common fake news.

BACKGROUND: The shaken baby syndrome (SBS) is a common cause of severe traumatic lesions in infants. Although well established for almost five decades, SBS and its diagnosis are becoming more and more aggressively challenged in courts. These challenges feed on the scientific debate and controversies regarding the pathophysiology and the differential diagnoses, scientific uncertainty being readily exploited by specialized barristers. MATERIAL AND METHODS: In the present review, we analyze the most common challenges to the concept of SBS and its diagnosis, as well as the scientific evidence available to counter these challenges, the differential diagnoses, and how SBS can be diagnosed with confidence. RESULTS: We found that the pathophysiology of SBS is well documented, with stereotyped descriptions by perpetrators, in good correlation with experimental studies and computer models. SBS is a well-defined clinico-pathological entity with a characteristic constellation of lesions; with a rigorous evaluation protocol, its diagnosis can be made rapidly and with excellent accuracy beyond a reasonable doubt. CONCLUSION: It is important that medical experts master an extensive knowledge regarding the pathophysiology of the lesions of SBS, in particular infantile subdural hematomas, as well as other CSF-related conditions. This emphasizes the role that pediatric neurosurgeons should play in the clinical and medicolegal management of these patients.

Spinal lesions caused by abusive trauma in young children.

BACKGROUND AND PURPOSE: Spinal lesions are increasingly recognized as an integral part of the child abuse spectrum; however, the description of lesions, their biomechanics, true incidence, clinical impact, and medicolegal implications are poorly understood. MATERIAL AND METHODS: We report from the literature and our personal experience on abusive spinal lesions (ASL) in children under 3 years, compared with cases of abusive head injuries (AHI) without spinal lesions on the one hand and with accidental spinal lesions on the other. RESULTS: Between 2002 and 2021, we collected 12 observations of ASL, 4 male and 8 female. These were compared with 338 cases of infants having AHI without ASL and 18 cases of accidental spinal trauma in the same age group. Fractures were found in 10 cases of ASL: wedge fracture in 9, and complete disruption with paraplegia in one, which required emergency reduction and stabilization with a good motor recovery. Two patients had intraspinal hemorrhagic lesions without fracture, associated in one case with tetraplegia which contributed to the fatal outcome. ASL affected girls more often and had a more severe clinical presentation; more than half of ASL involved the lumbar levels, which were unaffected in accidental traumas. CONCLUSIONS: ASL are not exceptional, and their presence corroborates cranial lesions indicating child abuse. Two etiologies emerge from this study: wedge fractures and cervical spinal cord lesions caused by shaking and the rare thoraco-lumbar dislocation indicating a particularly violent assault. Systematic MRI study of the spine is warranted in cases of child abuse.

Answer to Lynøe: Interesting data about confessions and abusive head trauma, but suboptimal analysis.

The authors of the cited paper respond to the critics formulated by a Swedish leading expert regarding methodology shortcomings of our study "Confessed versus denied inflicted head injuries in infants: similarities and differences." They admit some methodological limitations but maintain their conclusions that the diagnosis was correct in the confession and denial groups and that the denial was more difficult in the more severe cases.

Confessed versus denied inflicted head injuries in infants: similarities and differences.

BACKGROUND AND PURPOSE: Abusive head injuries (AHI), and in particular shaken baby syndrome (SBS), are common causes of mortality and morbidity in infants. Although SBS is a well-established entity, based on clinical experience and experimental data, and confirmed by the perpetrators' confessions, a growing number of publications challenge the diagnostic criteria, and even the validity of the perpetrators' confession. We decided to study AHI in infants and compare cases with and without confession. MATERIAL AND METHODS: We collected prospectively all cases of infantile traumatic head injuries hospitalized in our institution between 2001 and 2021. From this database, we selected victims of AHI, comparing cases for which the perpetrator confessed during police inquiry ("confession" group) versus cases without confession ("denial" group). RESULTS: We studied 350 cases of AHI in infants; 137 of these (39.1%) were confessed. We found no statistically significant difference between the two groups regarding the child's previous history, as well as the personality and previous history of the caretakers. However, the "confession" group showed significantly more severe clinical presentation, cerebral lesions, retinal hemorrhages, and a more pejorative outcome. CONCLUSIONS: We conclude that the diagnosis of AHI was confirmed by the confession in a large number of cases, indicating that the diagnostic criteria of AHI are robust. We also found that denial, although possibly sincere, was likely ill-founded, and that the perpetrators' decision to confess or deny was markedly influenced by the severity of the inflicted lesions.

Non-accidental head injury of infants: legal implications for the pediatric neurosurgeon.

BACKGROUND: Though the neurosurgeon's role in non-accidental head injury (NAHT) is the prompt recognition and care of the inflicted injuries, he/she should be aware of the possible legal implications related to this particular neurosurgical condition. MATERIAL AND METHODS: Based on published data and their own clinical and medico-legal practice, the authors review the role of the neurosurgeon in NHAT. RESULTS: Besides the contribution that can be given by the neurosurgeon to a correct differential diagnosis, he/she is likely to be involved in the interpretations of the pathological findings in the case where the judge may request for a medical opinion concerning etiology, circumstances, severity, and consequences. As a member of a multidisciplinary team, usually the neurosurgeon is only requested for information regarding the lesions he was called to recognize and treat. Nevertheless, such information may have a pivotal part in the evaluation process. Consequently, the neurosurgeon should be able to reach a correct differential diagnosis of NAHT among all the events that may share similar clinical and anatomo-pathological characteristics and be aware of the ongoing scientific controversies related to the diagnosis and pathophysiology. CONCLUSIONS: In practical terms, the pediatric neurosurgeon is called to individuate and record all the precise details of the condition to be eventually offered to the judge in case of controversy. Whereas the diagnosis of NAHT should be evoked at the initial phase in order not to leave the child unprotected, all possible alternative hypotheses should be ruled out "beyond reasonable doubt" for the court. The medical file should be maintained in the record carefully and be accessible even after a long time.

Decompressive surgery in abusive head injury: Experience from a Singapore children's hospital and a review of literature.

PURPOSE: Abusive head trauma (AHT) is a major cause of morbidity and mortality in children. Studies on pediatric head injury observe that AHT patients often have a higher incidence of malignant cerebral oedema and, overall, worse prognosis. There are limited studies with a focus on the outcome of decompressive surgery in children with AHT. This is a study undertaken to review our institutional experience on the role of decompressive surgery in AHT patients and objectively assess its outcomes, in corroboration with current literature. METHODS: This is an ethics-approved, retrospective study. Inclusion criteria consist of all children with a diagnosis of AHT managed by the Neurosurgical Service, KK Women's and Children's Hospital. Demographical and clinical variables are incorporated in the statistical analyses. RESULTS: From 2011 to 2021, a total of 7 patients required decompressive surgery for AHT. Mean age of the cohort was 17.1 months (with the majority of patients being male (n = 5, 71.4%). During the follow-up period, there was 1 mortality (14.3%), 3 patients developed cerebral palsy (42.9%), and 3 patients had post-traumatic epilepsy (42.9%). With regards to functional outcome, 4 patients (57.1%) had a favorable KOSCHI score at 6 months follow-up. CONCLUSION: Decompressive surgery in children with AHT presents with its own unique challenges. We therein present our neurosurgical experience in decompressive surgery for this extremely vulnerable group of patients. Given the potential role of decompressive surgery in AHT, the development of an objective marker to select such patients who may benefit most from intervention should be the way forward.

Crouzon Syndrome Anatomy, Usefulness of Vestibular Orientation.

ABSTRACT: Spatial resolution of computerized tomographic scanner has reached a level to which accurate anatomic measurements could be done in. Three-dimensional accurate measurements require a reliable referential system. In craniology landmarks are usually selected in the skull base. For craniofacial malformation the classical landmarks are of no help so the authors have used the vestibular orientation to study a series of 50 Crouzon syndrome computerized tomographic scan and compare the results (shape, position, linear mensuration, volumes…) with 122 controls in unaffected patients. The authors have confirmed Crouzon description of a high level of polymorphism in phenotypes, the authors propose an organigram to understand the abnormal growth patterns in crouzon syndrome, which lead to such consequences. This polymorphism implies that the planning of surgical treatment should be tailored for each case.

Crouzon syndrome: posterior fossa volume studies in vestibular orientation.

INTRODUCTION: With the increasing possibilities of surgical treatment addressing the complex spectrum of defects in Crouzon syndrome (CS), it is of utmost importance to obtain accurate assessment of the malformation. Evaluating the volume of the posterior fossa is of great importance since many authors, considering that its volume is always decreased, favor posterior fossa enlargement as a first step in the treatment of CS. MATERIAL AND METHODS: We studied CT scans of children with CS, which were vestibular-oriented (VO) in order to conform with physiological landmarks, and we measured intracranial volume by manual segmentation; these abnormal CT scans were superposed with VO 3D-CT scanners of age-matched controls. We studied the volume index of the posterior fossa for each CS patient defined as the ratio of its volume in CS patients with the normal for age calculated from normal controls. RESULTS: We studied the imaging of 41 children with CS and 70 control. Among CS patients, the volume of the posterior fossa was increased in 10, compared with control. We found closure of the sphenoidal synchondrosis was correlated with age and with the width of the posterior fossa, but not with its length nor with the posterior fossa volume index. CONCLUSION: Segmentation on VO-CT scanner and superposition with CT scanners of normal controls is a powerful tool for the study of the impact of CS or other synostoses on volume and shape. We found that CS is more heterogeneous than previously thought, and surgical strategies should be adapted accordingly.

Morphological and surgical results in sagittal synostosis: early craniectomy versus later cranioplasty.

BACKGROUND AND PURPOSE: Morphological correction is one of the main aims of surgery for sagittal synostosis (SSO). Different surgical techniques have been developed; however, few studies have compared the different surgical protocols. The morphological outcome is poorly documented, because a consensual evaluation tool is lacking. MATERIAL AND METHODS: We performed a prospective study of children operated for SSO in our institution. Children were operated whenever possible at 4 months for craniectomy; by default, children underwent cranioplasty at or after 9 months. The morphological outcome of all children was evaluated using traditional craniometry with head circumference (HC) and the cephalic index (CI), and with the Rotterdam scaphocephaly morphology score (RSMS), a total of semi-quantitative assessments of morphological hallmarks. RESULTS: Craniectomy was significantly associated with a shorter operation time and hospital stay, and a better impact on HC and CI measurements, compared with cranioplasty. The RSMS was markedly improved after surgery in both groups; however, we found no significant difference in improvement between the two groups. Although the transfusion rate and the prevalence of developmental delay were lower in the craniectomy group, and reoperations for calvarial lacunae or complex craniosynostosis occurred only this group, these differences were not significant. CONCLUSIONS: Our results support early surgery with craniectomy whenever possible; however, cranioplasty at a later age is a very acceptable by-default indication. In addition to classical craniometry, morphological evaluation using the RSMS or a similar quantitative scale appears highly desirable for future studies.

Linear craniectomy for early posterior decompression in craniosynostoses: technique and results.

INTRODUCTION: Early decompressive posterior linear craniectomy (PLC) can be indicated in very young infants with complex multisuture synostosis, which often involve the lambdoid suture (LS). The literature data on the surgical technique and its results are scarce. MATERIAL AND METHODS: Based on our experience with PLC during the last 10 years, we detail our surgical technique for PLC, the possible pitfalls, and complication avoidance. RESULTS: We review seven observations, 5 girls and 2 boys, 6 of these with identified mutations, operated for PLC at a mean age of 3.19 months (6 days to 6.1 months). One patient died of unexplained cardiac arrest on postoperative day two, the others had a favorable outcome with good development and no visual loss. Three of these required additional cranioplasty at a later age, one of these with craniofacial distraction. DISCUSSION: PLC can be a salvage operation in very young patients with complex synostosis involving the LS, and with proper preparation and careful technique, allows favorable outcome. The approach must be versatile in order to anticipate further surgeries in these complex, most often syndromic cases.

Synostosis of the lambdoid suture: a spectrum.

PURPOSE: Lambdoid synostosis (LS) is a rare condition, which is either isolated; associated with sagittal synostosis, the "Mercedes-Benz" syndrome (MBS); or with synostosis of the coronal sutures (oxycephalic form). In addition, LS is part of the phenotype of a growing number of genetic diseases. The nosology, pathophysiology, and management are controversial. We decided to review our experience with LS. METHODS: We reviewed retrospectively pediatric cases of LS proved on CT-scanner, isolated or associated with other conditions, followed in our craniofacial center during the last 15 years, regarding clinical presentation, anatomical lesions, syndromic associations, surgical management, and outcome. RESULTS: We reviewed 48 cases: 6 isolated LS, 22 MBS, and 20 oxycephalic. A syndromic context was present in 72% (up to 80% of oxycephalic cases), and faciostenosis was present in 23%, mostly oxycephalic cases (40%). Transverse sinus agenesis was found in 61% of documented patients. A total of 31% of children had a dystocic birth, up to 45% of MBS. Decompressive craniectomy or cranioplasty was needed in a majority of patients, often young infants, while posterior fossa decompression was mostly performed in older children. CONCLUSION: LS is rarely isolated and non syndromic; most cases are found in a wide spectrum of diseases, and LS is often associated with sagittal or coronal synostosis. Genetic evaluation is mandatory for LS; conversely, geneticists may require neurosurgical advice for LS in an increasing number of very rare diseases. The surgical management of LS should be tailored according to clinical presentation, age, and syndromic context.