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Pediatric Neurosurgery 2022Craniosynostosis is a condition characterized by the premature fusion of 2 or more skull bones. Craniosynostosis of the lambdoid suture is one of the rarest forms,...
BACKGROUND
Craniosynostosis is a condition characterized by the premature fusion of 2 or more skull bones. Craniosynostosis of the lambdoid suture is one of the rarest forms, accounting for 1-4% of all craniosynostoses. Documented cases are separated into simple (single suture), complex (bilateral), and associated with adjacent synostoses ("Mercedes Benz" Pattern) or syndromes (i.e., Crouzon, Sathre-Chotzen, Antley-Bixler). This condition can manifest phenotypic deformities and neurological sequelae that can lead to impaired cognitive function if improperly treated or left undiagnosed. Preferred surgical techniques have varied over time but all maintain the common goals of establishing proper head shape and preventing of complications that could contribute to aforementioned sequelae.
SUMMARY
This comprehensive review highlights demographic distributions, embryological development, pathogenesis, clinical presentation, neurological sequelae, radiologic findings, surgical techniques, surgical outcomes, and postoperative considerations of patients with lambdoid craniosynostosis presentation. In addition, a systematic review was conducted to explore the operative management of lambdoid craniosynostosis using PubMed, Embase, and Scopus databases, with 38 articles included after screening. Key Messages: Due to a low volume of published cases, diagnosis and treatment can vary. Large overlap in presentation can occur in patients that display lambdoid craniosynostosis and posterior plagiocephaly, furthering the need for comprehensive analysis. Possessing the knowledge and tools to properly assess patients with lambdoid craniosynostosis will allow for more precise care and improved outcomes.
Topics: Cranial Sutures; Craniosynostoses; Humans; Infant; Neurosurgical Procedures; Plagiocephaly; Skull
PubMed: 34864743
DOI: 10.1159/000519175 -
Journal of Oral and Maxillofacial... Apr 2022The purpose of this systematic review and meta-analysis was to estimate and compare rates of unplanned reoperation and complications after undergoing either... (Meta-Analysis)
Meta-Analysis
PURPOSE
The purpose of this systematic review and meta-analysis was to estimate and compare rates of unplanned reoperation and complications after undergoing either fronto-orbital advancement (anterior cranial vault expansion) or posterior cranial vault expansion as an early surgery in the management of syndromic craniosynostosis.
MATERIALS AND METHODS
A literature search was performed following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Relevant articles were identified in 2 electronic databases (PubMed and EMBASE) from the time of electronic publication to November 2020. Quality assessment and risk of bias were appraised using the Grading of Recommendations Assessment, Development and Evaluation system. A meta-analysis was performed comparing rates of reoperation and complications between participants who underwent anterior or posterior cranial vault expansion as an early surgery.
RESULTS
Of 1,373 screened records, 7 met inclusion criteria. Six were included in the meta-analysis. The studies that met inclusion criteria reported on 103 patients treated with anterior techniques and 72 patients treated with a posterior approach. Anterior cranial vault expansion was associated with significantly higher rates of reoperation (Peto odds ratio = 2.83; 95% confidence interval = 1.19, 6.74, P = .02) and complications (Peto odds ratio = 2.61; 95% confidence interval = 1.12, 6.12, P = .03) than posterior cranial vault expansion.
CONCLUSIONS
Both anterior and posterior approaches are suitable options in the treatment of syndromic craniosynostosis depending on patient-specific factors. Anterior cranial vault expansion was associated with higher rates of unplanned reoperation and complications than posterior techniques in this analysis. Because of the paucity of literature which met inclusion criteria, this study was not able to assess critical outcome variables such as distance distracted/volumetric expansion, estimated blood loss, and cost. Larger studies evaluating both techniques under multiple institutions with long-term follow-up are indicated.
Topics: Craniosynostoses; Humans; Infant; Morbidity; Osteogenesis, Distraction; Reoperation; Skull
PubMed: 34863645
DOI: 10.1016/j.joms.2021.10.022 -
Hallermann Streiff syndrome: Cranio-facial manifestations systematic review and report of two cases.Journal of Stomatology, Oral and... Sep 2022Hallermann Streiff syndrome (HSS) is a rare congenital abnormality with about 200 case reports in the literature. Its etiology is unknown although it may be due to a...
Hallermann Streiff syndrome (HSS) is a rare congenital abnormality with about 200 case reports in the literature. Its etiology is unknown although it may be due to a sporadic mutation. Diagnosis is based on the association of craniofacial malformation, dental abnormalities, hypotrichosis, atrophy of the skin, proportionate nanism, congenital cataract and bilateral microphtalmos. Cranio-facial deformities are the main signs detected and the most easily recognizable. We report cranio-facial and oral signs from a systemic literature review, and illustrate our findings with two of our patients diagnosed with HSS. Common cranio-facial manifestations are craniofacial malformation with a « parrot beak » nose, micrognathia, aprominent skull, sutures closing anomaly, malocclusion, dental anomalies, eyebrows and eyelash lack and atrophy of the nose skin. Knowledge of these signs should allow for early diagnosis and adequate treatment and follow up.
Topics: Atrophy; Face; Hallermann's Syndrome; Humans; Malocclusion; Skull
PubMed: 34800747
DOI: 10.1016/j.jormas.2021.11.002 -
Neurogastroenterology and Motility Jun 2022Nocebo effects occurring in patients receiving placebo frequently impact on adverse events reported in randomized controlled trials (RCTs) in irritable bowel syndrome... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Nocebo effects occurring in patients receiving placebo frequently impact on adverse events reported in randomized controlled trials (RCTs) in irritable bowel syndrome (IBS). Therefore, we conducted a systematic review and meta-analysis to assess the proportion of patients randomized to placebo or active drug experiencing any adverse event in trials of licensed drugs for IBS with constipation (IBS-C) or diarrhea (IBS-D), and to estimate the risk of developing adverse events among patients randomized to placebo.
METHODS
We searched MEDLINE, EMBASE CLASSIC and EMBASE, and the Cochrane central register of controlled trials (through June 2021) to identify RCTs comparing licensed drugs with placebo in adults with IBS-C or IBS-D. We generated Forest plots of pooled adverse event rates in both active drug and placebo arms and pooled risk differences (RDs) with 95% confidence intervals (CIs).
KEY RESULTS
There were 21 RCTs of licensed drugs versus placebo in IBS-C (5953 patients placebo) and 17 in IBS-D (3854 patients placebo). Overall, 34.9% and 46.9% of placebo patients in IBS-C and IBS-D trials, respectively, developed at least one adverse event, with a statistically significantly higher risk of any adverse event and withdrawal due to an adverse event with active drug. In IBS-C and IBS-D trials, rates of each individual adverse event were generally higher with active drug. However, in IBS-C trials, only diarrhea or headache was significantly more common with active drug (RD 0.066 (95% CI 0.043-0.088) and RD 0.011 (95% CI 0.002-0.021), respectively), and in IBS-D trials only constipation, nausea, or abdominal pain (RD 0.096 (95% CI 0.054-0.138), 0.014 (95% CI 0.002-0.027), and 0.018 (95% CI 0.002-0.034), respectively).
CONCLUSIONS & INFERENCES
Patients with IBS randomized to placebo have a high risk of reporting adverse events, which might relate to both nocebo and non-nocebo factors. Although patients' expectations and psychosocial factors may be involved, further understanding of the mechanisms are important to control or optimize these effects in RCTs, as well as in clinical practice.
Topics: Abdominal Pain; Adult; Constipation; Craniosynostoses; Diarrhea; Humans; Intellectual Disability; Irritable Bowel Syndrome; Nocebo Effect; Randomized Controlled Trials as Topic
PubMed: 34672052
DOI: 10.1111/nmo.14279 -
World Neurosurgery May 2022The use of three-dimensional (3D) printing in neurosurgery has become more prominent in recent years for surgical training, preoperative planning, and patient education.... (Review)
Review
BACKGROUND
The use of three-dimensional (3D) printing in neurosurgery has become more prominent in recent years for surgical training, preoperative planning, and patient education. Several smaller studies are available using 3D printing; however, there is a lack of a concise review. This article provides a systematic review of 3D models in use by neurosurgical residents, with emphasis on training, learning, and simulation.
METHODS
A structured literature search of PubMed and Embase was conducted using PRISMA guidelines to identify publications specific to 3D models trialed on neurosurgical residents. Criteria for eligibility included articles discussing only neurosurgery, 3D models in neurosurgery, and models specifically tested or trialed on residents.
RESULTS
Overall, 40 articles were identified that met inclusion criteria. These studies encompassed different neurosurgical areas including aneurysm, spine, craniosynostosis, transsphenoidal, craniotomy, skull base, and tumor. Most articles were related to brain surgery. Of these studies, vascular surgery had the highest overall, with 13 of 40 articles, which include aneurysm clipping and other neurovascular surgeries. Twenty-two articles discussed cranial plus tumor surgeries, which included skull base, craniotomy, craniosynostosis, and transsphenoidal. Five studies were specific to spine surgery. Subjective outcome measures of neurosurgical residents were most commonly implemented, of which results were almost unanimously positive.
CONCLUSIONS
3D printing technology is rapidly expanding in health care and neurosurgery in particular. The technology is quickly improving, and several studies have shown the effectiveness of 3D printing for neurosurgical residency education and training.
Topics: Craniosynostoses; Humans; Internship and Residency; Models, Anatomic; Neurosurgery; Neurosurgical Procedures; Printing, Three-Dimensional
PubMed: 34648984
DOI: 10.1016/j.wneu.2021.10.069 -
Child's Nervous System : ChNS :... Dec 2021The optimal protocol for diagnostic workup of craniosynostosis and the role of specific imaging modalities remain controversial. Skull X-rays and 3-dimensional head CTs... (Review)
Review
INTRODUCTION
The optimal protocol for diagnostic workup of craniosynostosis and the role of specific imaging modalities remain controversial. Skull X-rays and 3-dimensional head CTs are options when physical exam is equivocal but involve ionizing radiation. Ultrasound has emerged as an alternative modality for visualization of cranial sutures, but its use is not widespread.
METHODS
The authors performed a systematic review of the literature on the use of ultrasound for the diagnosis of craniosynostosis.
RESULTS
A total of 12 studies involving 1062 patients were included. Overall, 300 patients (28.2%) were diagnosed with craniosynostosis. A total of 369 (34.7%) patients had their diagnosis (craniosynostosis vs. patent sutures) confirmed with another imaging modality in addition to ultrasound. Among studies, the specificity of ultrasound ranged from 86 to 100%, and the sensitivity from 71 to 100%.
CONCLUSIONS
Ultrasonography of cranial sutures is a feasible and accurate tool for the diagnosis of single-suture craniosynostosis when physical exam findings are insufficient. Although technical aspects of ultrasonography and its interpretation have an associated learning curve, ultrasound can achieve high sensitivity and specificity among patients with suspected craniosynostosis.
Topics: Cranial Sutures; Craniosynostoses; Humans; Infant; Radiography; Sutures; Ultrasonography
PubMed: 34611761
DOI: 10.1007/s00381-021-05301-w -
Foot (Edinburgh, Scotland) Dec 2021The standard of care in the treatment of symptomatic tarsal coalitions is open surgery. However, certain limitations exist with open surgery, which include limited... (Review)
Review
INTRODUCTION
The standard of care in the treatment of symptomatic tarsal coalitions is open surgery. However, certain limitations exist with open surgery, which include limited visualization leading to an incomplete resection and possible recurrence of the tarsal coalition. Arthroscopic tarsal coalition resection (TCR) is an alternative that is gaining traction, primarily as the safety profile of posterior ankle and subtalar arthroscopy is more well understood. This study provides a systematic review of the outcomes of arthroscopic TCR.
METHODS
PubMed and Embase were searched independently by 2 reviewers for relevant articles based on predetermined criteria. The subject heading "tarsal coalition" and its related key terms were used.
RESULTS
A total of 416 studies were revealed by the initial search, out of which only 6 met our predetermined inclusion criteria. A total of 42 patients (average age: 17.6 years) were treated with arthroscopic TCR. Thirty-three (78.6%) and 9 (21.4%) patients had talocalcaneal and calcaneonavicular coalitions, respectively. The follow-up period ranged from 6 to 60 months (mean: 26 months), and no recurrence of the tarsal coalition was detected (0.0%). Complications occurred in two (4.8%) patients only, with one developing complex regional pain syndrome (CRPS), and another patient developing hyperesthesia on the medial aspect of the calcaneus.
CONCLUSION
Arthroscopic TCR is a feasible and effective surgery for both CNC and TCC with minimal complications and no disease recurrence at an average of 26 months follow-up. Future high-level of evidence studies are needed to compare the outcomes of open versus arthroscopic TCR.
Topics: Adolescent; Arthroscopy; Calcaneus; Humans; Synostosis; Tarsal Bones; Tarsal Coalition
PubMed: 34597922
DOI: 10.1016/j.foot.2021.101864 -
The Journal of Craniofacial SurgeryBlood loss is a potential cause of morbidity and mortality in craniosynostosis surgery. Recent reports have suggested that the use of tranexamic acid (TXA), an... (Meta-Analysis)
Meta-Analysis
Blood loss is a potential cause of morbidity and mortality in craniosynostosis surgery. Recent reports have suggested that the use of tranexamic acid (TXA), an antifibrinolytic agent, mitigates this blood loss. A comprehensive systematic review and subsequent meta-analysis was undertaken, with the view to clarify the effectiveness of TXA in reducing blood loss and transfusion requirements in craniosynostosis surgery. Medline and PubMed databases were searched using the preferred reporting items for systematic reviews and meta-analyses technique, and 7003 articles were assessed based on predefined selection criteria. Seven trials were identified, of which 2 were randomized controlled trials and the remainder retrospective cohort studies. All trials were assessed using the Jadad and strengthening the reporting of observational studies in epidemiology scores. The meta-analysis found a clear statistical reduction in blood loss in those patients who received TXA perioperatively, with a combined blood loss reduction of 7.06 ml/kg (95% confidence interval -8.97 to -5.15, P < 0.00001). The blood loss reduction was found to extrapolate to a reduction in perioperative transfusion requirements by 8.47 ml/kg in this cohort (95% confidence interval -10.9 to -6.04, P < 00001). There were no TXA-related adverse outcomes recorded in the 258 patients who received TXA across all trials. The trials included in this meta-analysis were limited by underpowered population sizes and significant heterogeneity in blood loss recording techniques. Further, there was significant heterogeneity amongst operations performed. The current literature appears to support the use of TXA in craniosynostosis surgery, but further high quality randomized controlled trials are indicated, ideally including a subgroup analysis between the operations performed.
Topics: Antifibrinolytic Agents; Blood Loss, Surgical; Craniosynostoses; Humans; Retrospective Studies; Tranexamic Acid
PubMed: 34593743
DOI: 10.1097/SCS.0000000000008123 -
Acta Neurochirurgica Nov 2021Management of ventriculomegaly in pediatric patients with syndromic craniosynostosis (SC) requires understanding the underlying mechanisms that cause increased...
INTRODUCTION
Management of ventriculomegaly in pediatric patients with syndromic craniosynostosis (SC) requires understanding the underlying mechanisms that cause increased intracranial pressure (ICP) and the role of cerebrospinal fluid (CSF) in cranial vault expansion in order to select the best treatment option for each individual patient.
METHODS
A total of 33 pediatric patients with SC requiring craniofacial surgery were retrospectively evaluated. Cases of nonsyndromic craniosynostosis and shunt-induced craniosynostosis were excluded. Six syndrome-based categories were distinguished: Crouzon syndrome, Pfeiffer syndrome, Apert syndrome, cloverleaf skull syndrome, and others (Muenke syndrome, Sensenbrenner syndrome, unclassified). All of the patients were treated surgically for their cranial deformity between 2010 and 2016. The presence of ventriculomegaly and ventriculoperitoneal (VP) shunt requirement with its impact in cranial vault expansion were analyzed. Clinical and neuroimaging studies covering the time from presentation through the follow-up period were revised. The mean postoperative follow-up was 6 years and 3 months. A systematic review of the literature was conducted through a PubMed search.
RESULTS
Of the total of 33 patients with SC, 18 (54.5%) developed ventriculomegaly and 13 (39.4%) required ventriculoperitoneal (VP) shunt placement. Six patients (18.2%) required shunt placement previous to craniofacial surgery. Seven patients (21.2%) required a shunt after craniofacial surgery. Seven fixed pressure ventriculoperitoneal shunts and six programmable valves were placed as first choice. All patients improved their clinical symptoms after shunt placement. Aesthetic results seemed to be better in patients with programmable shunts.
CONCLUSIONS
Unless clear criteria for overt hydrocephalus are present, it is recommended to perform craniofacial surgery as a first step in the management of patients with SC in order to preserve the expansive effect of CSF for cranial vault expansion. In our experience, the use of externally programmable valves allows for the treatment of hydrocephalus while maintaining the expansive effect of CSF for the remodeling of the cranial vault. Prospective evaluations are needed to determine causality.
Topics: Child; Craniosynostoses; Humans; Hydrocephalus; Retrospective Studies; Skull; Ventriculoperitoneal Shunt
PubMed: 34570275
DOI: 10.1007/s00701-021-04980-3 -
Child's Nervous System : ChNS :... Dec 2021Posterior plagiocephaly (PP) is a common clinical condition in pediatric age. There are two main causes of PP: postural plagiocephaly and craniosynostosis. Early... (Review)
Review
PURPOSE
Posterior plagiocephaly (PP) is a common clinical condition in pediatric age. There are two main causes of PP: postural plagiocephaly and craniosynostosis. Early diagnosis is important, as it prevents neurological complications and emergencies. Diagnosis in the past was often made late and with imaging tests that subjected the infant to a high radiation load. Suture ultrasound does not use ionizing radiation; it is easy to perform, allows an early diagnosis, and directs toward the execution of the cranial 3D-CT scan, neurosurgical consultation, and possible intervention. The aim of the study is to describe the high sensitivity and specificity of suture ultrasound for the differential diagnosis between plagiocephaly and craniosynostosis.
METHODS
We reported our prospective experience and compared it with the data in the literature through a systematic review. The systematic review was conducted on electronic medical databases (PubMed, Embase, Cochrane Library, Scopus, and Web of Science) evaluating the published literature up to November 2020. According to Preferred Reporting Items for Systematic Reviews and Meta-ANALYSES (PRISMA statement), we identified 2 eligible studies. Additionally, according to AMSTAR 2, all included reviews have been critically rated as high quality. A total of 120 infants with abnormal skull shape were examined in NICU. All underwent clinical and ultrasound examination.
RESULTS
Of the total, 105 (87.5%) had plagiocephaly and 15 dolichocephaly/scaphocephaly (12.5%). None of these had associated other types of malformations and/or neurological disorders. The synostotic suture was identified ultrasonographically in 1 infant and subsequently confirmed by 3D CT scan (100%).
CONCLUSION
Cranial sutures ultrasonography can be considered in infants a selective, excellent screening method for the evaluation of skull shape deformities as first technique before the 3D CT scan exam and subsequent neurosurgical evaluation. Cranial suture ultrasonography should be considered part of clinical practice especially for pediatricians.
Topics: Child; Cranial Sutures; Craniosynostoses; Humans; Infant; Plagiocephaly; Prospective Studies; Skull; Sutures; Ultrasonography
PubMed: 34453581
DOI: 10.1007/s00381-021-05324-3