Surface Cranial Ultrasound: The Natural Heir to X-Ray for the Screening of Skull Deformities in Infants.

PURPOSE: Volumetric tomography (3D-CT) is currently considered the gold standard for the diagnosis of craniosynostosis, but its use as the first-line examination for cranial deformities is a topic of debate, because of skull X-ray radiation and low sensitivity and specificity. Cranial ultrasound is an emerging noninvasive radiation-free alternative, but its diagnostic accuracy still needs confirmation. MATERIALS AND METHODS: The present prospective study included 350 infants with skull deformities, who underwent cranial ultrasound as the first-line examination, followed by 3D-CT if the echography results was positive or unclear. If the results were negative, infants underwent physical treatment and follow-up. To evaluate ultrasound reliability, we focused on cases that underwent both the index test and the gold standard and performed a double-blind comparison of the echography and 3D-CT results. RESULTS: Ultrasound documented patent sutures in 293 infants and 9 had inconclusive results. The 293 ultrasound-negative infants were followed clinically: all improved, except 28 that underwent 3D-CT. In all of these cases, 3D-CT confirmed the ultrasonography results (no false negatives). 48 infants showed premature suture closure and underwent 3D-CT: 47 were confirmed (true positive), 1 was false positive. The sensitivity was 100%, the specificity was 99.7%, the positive and negative predictive values were 97.9% and 100%, respectively, the accuracy was 99.7%, and the diagnostic test evaluation was conclusive. CONCLUSION: The study documented the high sensitivity and specificity of echography for the diagnosis of craniosynostosis in a referral center, with better results being achieved before 6 months of age. Major limitations are the loss of diagnostic significance as the child grows and the learning curve needed. The advantages are avoidance of radiation and chance to evaluate the brain at the same time.

Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document.

BACKGROUND: Chiari malformation type 1 (CM1) is a rare condition where agreed classification and treatment are still missing. The goal of this study is to achieve a consensus on the diagnosis and treatment of CM1 in children. METHODS: A multidisciplinary panel formulated 57 provisional statements based on a review of the literature. Thirty-four international experts (IE) participated in a Delphi study by independently rating each statement on a 4-point Likert scale ("strongly disagree," "disagree," "agree," "strongly agree"). Statements that were endorsed ("agree" or "strongly agree") by < 75% of raters were re-formulated, or new statements were added, and another Delphi round followed (up to a maximum of three). RESULTS: Thirty-five IE were contacted and 34 agreed to participate. A consensus was reached on 30/57 statements (52.6%) after round 1. Three statements were added, and one removed. After round 2, agreement was reached on 56/59 statements (94.9%). Finally, after round 3, which took place during the 2019 Chiari Consensus Conference (Milan, Italy), agreement was reached on 58/59 statements (98.3%) about four main sections (Definition and Classification, Planning, Surgery, Isolated Syringomyelia). Only one statement did not gain a consensus, which is the "definition of radiological failure 24 month post-surgery." CONCLUSIONS: The consensus document consists of 58 statements (24 on diagnosis, 34 on treatment), serving clinicians and researchers following children with CM1. There is a clear need for establishing an international network and registry and to promote collaborative studies to increase the evidence base and optimize the long-term care of this patient population.

Diagnosis and treatment of Chiari malformation and syringomyelia in adults: international consensus document.

BACKGROUND: Syringomyelia and Chiari malformation are classified as rare diseases on Orphanet, but international guidelines on diagnostic criteria and case definition are missing. AIM OF THE STUDY: to reach a consensus among international experts on controversial issues in diagnosis and treatment of Chiari 1 malformation and syringomyelia in adults. METHODS: A multidisciplinary panel of the Chiari and Syringomyelia Consortium (4 neurosurgeons, 2 neurologists, 1 neuroradiologist, 1 pediatric neurologist) appointed an international Jury of experts to elaborate a consensus document. After an evidence-based review and further discussions, 63 draft statements grouped in 4 domains (definition and classification/planning/surgery/isolated syringomyelia) were formulated. A Jury of 32 experts in the field of diagnosis and treatment of Chiari and syringomyelia and patient representatives were invited to take part in a three-round Delphi process. The Jury received a structured questionnaire containing the 63 statements, each to be voted on a 4-point Likert-type scale and commented. Statements with agreement <75% were revised and entered round 2. Round 3 was face-to-face, during the Chiari Consensus Conference (Milan, November 2019). RESULTS: Thirty-one out of 32 Jury members (6 neurologists, 4 neuroradiologists, 19 neurosurgeons, and 2 patient association representatives) participated in the consensus. After round 2, a consensus was reached on 57/63 statements (90.5%). The six difficult statements were revised and voted in round 3, and the whole set of statements was further discussed and approved. CONCLUSIONS: The consensus document consists of 63 statements which benefited from expert discussion and fine-tuning, serving clinicians and researchers following adults with Chiari and syringomyelia.

Chiari 1 malformation and untreated sagittal synostosis: a new subset of complex Chiari?

PURPOSE: Chiari 1 malformation (CM1) is a well-known association with complex craniosynostosis (CC), while it has been rarely reported in association with monosynostosis. The aim of the present study is to investigate on the association between CM1 and untreated sagittal synostosis (USS). METHOD: The study included 48 cases of sagittal synostosis (SS), untreated for misdiagnosis and associated with CM1. The children were firstly diagnosed for CM1 by MRI (mean age 9) than for SS (mean age 10.5) by three-dimensional computerized tomography (3D-CT), which documented the absence of the sagittal suture, in the presence of residual indentation of all the other sutures. Syndromic cases were diagnosed by clinical evaluation and molecular studies. RESULTS: Of the 48 children harboring CM1 plus USS, 21 were asymptomatic for CM1 and are still on follow-up, while 27 children were operated for syringomyelia and scoliosis and/or occurrence of symptoms, three of them had an acute presentation (two papilledema and one sleep apneas) and 11 children had a documented increase of preoperative ICP. Craniovertebral decompression (CVD) was the first-line surgery in 24 children, 16 with duroplasty and five without and eight had also cerebellar (CBL) tonsil coagulation. A cranial vault remodelling was firstly performed in three children. Fifteen percent of children submitted to CVD needed a revision for cerebrospinal fluid (CSF) collection, while two needed both the supra- and infratentorial decompressive procedure and another two needed a treatment for the associated hydrocephalus. CONCLUSIONS: The present study identified an USS in 27 (15.5%) of 174 CM1 children operated for a symptomatic CM1. We suggest to define this association CM1 plus USS, a new subtype of complex CM1. For the high percentage of complications and multiple procedures needed to solve the CM1, we advise to identify by 3D-CT scan these children before performing CVD. Our finding suggests also that, if left untreated, SS may lead to the delayed occurrence of a challenging subset of CM1.

Chiari malformation-related headache: outcome after surgical treatment.

The outcome of headache in a series of 135 operated CM1 is presented. Favorable results were obtained in 85% of atypical and 93% typical headache with the support of a multidisciplinary approach that restricted the indications for surgery.

Occult spinal dysraphism: lessons learned by retrospective analysis of 149 surgical cases about natural history, surgical indications, urodynamic testing, and intraoperative neurophysiological monitoring.

PURPOSE: Prophylactic surgery is indicated for lipoma of the filum, while it is still debated for the conus lipomas and more complex tethering malformations of the cord. METHODS: We retrospectively reviewed the preoperative and postoperative clinical histories and long-term outcomes of 149 operated patients (33 adults, 116 children). Intraoperative neurophysiological monitoring (NPM) was utilized since 1998. Their malformative lesions were reclassified following recent Pang's embryological criteria for surgical complexity. In nine cases, the spinal tethering malformation was associated with an anorectal malformation (ARM) and in nine with a Chiari I malformation (CM1). RESULTS: One hundred nineteen (80 %) patients were symptomatic at the time of surgery, 66 (44 %) having presented with progressive preoperative deterioration. Postoperative surgery-related deterioration was observed in 6 % of the cases operated on under the intraoperative NPM control. Surgery did not improve any deficit, especially of sphincter functions, independently from the type of associated malformation (ARM, CM1). Urodynamic testing was a reliable predictor both in the preoperative and in the follow-up period of subsequent neurological deterioration. In the long-term follow-up, an increasing percentage of retethering was observed, especially concerning complex cases submitted to partial excision. The surgical risk increased with repeated operations. CONCLUSIONS: This study demonstrates that the rate of the natural deterioration associated with a conservative approach is higher than in patients operated on prophylactically, if the operation is performed by a team with a special expertise. However, the first surgical procedure should be aimed at detethering the conus completely, with the aid of intraoperative NPM; even in expert hands, it is associated with a high risk of clinical deterioration. Based on these results, we are increasing the percentage of children to whom surgery is offered when still asymptomatic as well as the degree of the lipoma excision to prevent retethering. However, in cases of rethetering and subjects presenting in adult age, we suggest to consider for surgery only those symptomatic. Urodynamic testing and magnetic resonance imaging in prone position were, in our experience, the best tools for screening those patients at risk of symptomatic retethering.

Incidence and risk factors of neurosurgical site infections: results of a prospective multicenter cohort study on 6359 surgeries.

BACKGROUND: Neurosurgical surgical site infections (SSI) are life-threatening complications, requiring medical treatment and additional surgeries and remain a substantial cause of morbidity. In order to identify the incidence and the main risk factors for SSI, we developed the Prophylaxis with Antibiotic Protocol for Neurosurgical Site Infections Study (PASSIS), a prospective observational multicenter cohort study for examining a large number of neurosurgical procedures. METHODS: The study PASSIS involved four Italian departments of neurosurgery applying the same antibiotic prophylaxis (ABP) protocol on 6359 consecutive neurosurgical procedures. In high-risk conditions (intra-operative contamination and/or postoperative cerebro-spinal fluid [CSF], and/or subcutaneous drainage and/or postoperative hyperpyrexia) and in presence of wound complication (CSF leak and/or CSF collection and/or wound diastasis), a prolongation protocol was prescribed. RESULTS: The crude rate of SSI in the whole series was and 1.7% for patient and 1.5% for procedure. Patient related SSI risk factors: Younger patients (≤14 years) had a significantly higher SSI risk compared with older patients (RR: 2.17; 95% CI: 1.13-4.14). Patients underwent two surgeries were at increased SSI risk (RR: 3.80; 95% CI: 2.33-6.18), and the risk increased with the number of surgeries. Surgeries lasting longer than 3 hours (RR: 2.27; 95% CI: 1.15-4.50), undergoing two or more surgeries and the presence of prosthetic implants (RR: 2.40; 95% CI: 1.53-3.77) were procedure related SSI risk factors positively associated with SSI. In high-risk conditions and in wound complication as defined previously, ABP prolongation showed limited efficacy (RR:1.97; 95% CI: 1.21-3.22 and 9.31; 95% CI: 5.90-14.68 respectively). CONCLUSIONS: The subjects submitted to complicated, repeated, long lasting craniotomies, especially if experiencing postoperative deterioration, display the higher risk of SSIs, as a final life-threatening complication. In order to reduce the SSI rate, further studies should address to design tailored prophylaxis protocols for each high risk situation as hereby defined; the wound complications deserve an increased microbiological surveillance, focusing the attention on the timing and source of infections.