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Developmental Neuropsychology Jul 2023There is conflicting evidence whether single-suture craniosynostosis (SSC), is linked to adversities of cognitive development. To assess the evidence for a link between... (Review)
Review
There is conflicting evidence whether single-suture craniosynostosis (SSC), is linked to adversities of cognitive development. To assess the evidence for a link between SSC and cognition, a systematic literature search was conducted and eligible studies assessed for inclusion by two independent readers. Forty-eight studies met inclusion criteria. Small to medium but persistent effects on both general and some specific cognitive functions across age bands were found in higher quality studies for SSC overall. There was limited evidence for effects related to surgical correction. Methodologies varied substantially and there was a lack of longitudinal studies using broad assessment batteries.
Topics: Humans; Craniosynostoses; Cognition; Longitudinal Studies; Sutures
PubMed: 37341559
DOI: 10.1080/87565641.2023.2225662 -
European Journal of Orthopaedic Surgery... Dec 2023Reconstructive surgery of the clavicle using free vascularised fibula grafting (FVFG) is sometimes required for the management of severe bone loss or non-union. As the... (Review)
Review
Reconstructive surgery of the clavicle using free vascularised fibula grafting (FVFG) is sometimes required for the management of severe bone loss or non-union. As the procedure is relatively rare, there is no universal agreement on the management and outcome. This systematic review aimed to first, identify the conditions for which FVFG has been applied; second, to gain an understanding of the surgical techniques used; and third, to report outcomes related to bone union, infection eradication, function and complications. A PRISMA strategy was used. Medline, Cochrane Central Register of Controlled Trials, Scopus and EMBASE library databases were interrogated using pre-defined MeSH terms and Boolean operators. Quality of evidence was evaluated based on OCEBM and GRADE systems. Fourteen studies based on 37 patients were identified with a mean follow-up time of 33.3 months. The most common reasons for the procedure were: fracture non-union; tumours requiring resection; post-radiation treatment osteonecrosis and osteomyelitis. The operation approaches were similar, involving graft retrieval, insertion and fixation and vessels chosen for reattachment. The mean clavicular bone defect size was 6.6 cm (± 1.5), prior to FVFG. Bone union occurred in 94.6% with good functional outcomes. Complete infection eradication occurred in those with preceding osteomyelitis. The main complications were broken metalwork, delayed union/non-union and fibular leg paraesthesia (n = 20). The mean re-operation number was 1.6 (range 0-5.0). The study demonstrates that FVFG is well tolerated and has a high success rate. However, patients should be advised about complication development and re-intervention requirement. Interestingly, overall data is sparse with no large cohort groups or randomised trials.
Topics: Humans; Fibula; Treatment Outcome; Clavicle; Fractures, Bone; Osteomyelitis; Bone Transplantation; Synostosis
PubMed: 37289244
DOI: 10.1007/s00590-023-03598-8 -
Clinical Neurology and Neurosurgery Jul 2024Craniosynostosis, a developmental craniofacial anomaly, can impair brain development and cause abnormal skull shape due to premature closure of one or more cranial... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Craniosynostosis, a developmental craniofacial anomaly, can impair brain development and cause abnormal skull shape due to premature closure of one or more cranial sutures. Traditional surgical treatments have evolved from open operations to minimally invasive endoscopic techniques. This systematic review and meta-analysis aim to evaluate the effectiveness and safety of the endoscopic approach in craniosynostosis correction.
METHODS
Adhering to Cochrane Group standards and the PRISMA framework, this review utilized databases like PubMed, Embase, and Web of Science, focusing on clinical and surgical outcomes of endoscopic craniosynostosis operations up to December 2023. Inclusion criteria emphasized studies with at least five patients undergoing endoscopic procedures, while exclusion criteria involved non-English papers, incomplete texts, and overlapping data. Statistical analysis used R software with various packages, and methodological bias was assessed using the ROBINS-I framework.
RESULTS
The review included 30 studies (4 prospective, 26 retrospective) with 2561 patients. The median age at operation was 3.20 months. Findings showed a mean operative time of 68.06 min, median hospital stay of 1.28 days, and mean blood loss of 29.89 ml. Blood transfusion was required in 9.97% of cases. Helmet therapy post-operation was common, with a median duration of 9 months. The rate of postoperative complications was 1.86%, and the reoperation rate was 3.07%. No procedure-related mortality was observed. The study noted substantial variations in the handling of craniosynostosis and a lack of consensus on the optimal timing and surgical approach.
CONCLUSION
Endoscopic techniques for craniosynostosis repair demonstrate safety and effectiveness, characterized by low complication risks and favorable surgical outcomes. However, due to the limitations of observational studies and inherent heterogeneity, further comprehensive and controlled trials are needed to validate these findings and understand the long-term outcomes of the endoscopic approach.
Topics: Craniosynostoses; Humans; Neuroendoscopy; Treatment Outcome; Postoperative Complications; Endoscopy; Infant; Operative Time; Length of Stay
PubMed: 38749357
DOI: 10.1016/j.clineuro.2024.108296 -
Archives of Orthopaedic and Trauma... Dec 2023To analyze the outcome of surgical treatment of tarsal coalition, assess the role of the surgical technique, as well as of coalition size and type on outcomes. (Review)
Review
PURPOSE
To analyze the outcome of surgical treatment of tarsal coalition, assess the role of the surgical technique, as well as of coalition size and type on outcomes.
METHODS
The search followed the Preferred Reporting Items of Systematic Review and Meta-Analysis and was performed in four databases: MEDLINE, Central, Scopus and Web of Science. The protocol has been registered in the international prospective register of systematic reviews. Patient-reported outcomes (PROMs), complications, revisions and radiographic recurrence were collected. Risk of bias was assessed using MINORS criteria. A random-effects model for meta-analysis was applied for analysis of data heterogeneity.
RESULTS
Twenty-five studies including 760 tarsal coalitions were included and had a weighted average follow-up of 44 months. Studies scored fair to poor on the risk of bias assessment with a mean MINORS score of 67% (44-81%). In 77.8% (37.5-100%) of surgically treated tarsal coalitions, good/excellent/non-limiting or improved PROMs were reported. Calculated data heterogeneity was moderate (I = 57%). Open bar resection with material interposition had a clinical success rate of 78.8% (50-100%). Complications occurred in 4.96% of cases. Coalition size did not prove to be a determining factor in postoperative outcome. The influence of the coalition type was not investigated by any of the studies.
CONCLUSION
Data on outcomes of surgical management for tarsal coalitions is limited to retrospective case series with high risk of bias and moderate data heterogeneity. In about ¾ of cases, open resection and interposition of material results in improved PROMs. The arbitrary margin of ≥ 50% of TC coalition size in relation to the posterior facet has little importance in surgical decision-making. None of the studies reported on the influence of the coalition type on postoperative clinical success.
Topics: Humans; Retrospective Studies; Synostosis; Systematic Reviews as Topic; Tarsal Bones; Tarsal Coalition
PubMed: 37462747
DOI: 10.1007/s00402-023-04982-z -
Annals of Plastic Surgery Oct 2023Although craniosynostoses involving the major sutures have been well described, the frequency of isolated minor suture craniosynostoses is much lower. Squamosal...
BACKGROUND
Although craniosynostoses involving the major sutures have been well described, the frequency of isolated minor suture craniosynostoses is much lower. Squamosal craniosynostosis (SQS) is a rare form of cranial synostosis, and the paucity of literature has made the creation of a standardized treatment plan difficult. We present a systematic review of the literature on isolated SQS to identify disease characteristics that lead to a need for operative intervention and to delineate patterns in surgical management.
METHODS
A systematic literature review was performed using the electronic databases of PubMed, Scopus, and MEDLINE and the key words "squamosal AND craniosynostosis," "squamous AND craniosynostosis," "squamosal craniosynostosis, "squamosal suture craniosynostosis," and "isolated squamosal craniosynostosis." Only human studies that described presentation and management of SQS were included. A blinded, 2-reviewer analysis of the articles was performed. Data collected included patient and disease characteristics, imaging workup, and treatment specifics, which were analyzed by descriptive statistics.
RESULTS
A total of 19 studies examining 119 patients with SQS were reviewed, with 97 (82%) multisutural cases and 22 isolated cases (18%). Of the isolated cases, 6 (27%) required surgical craniosynostosis repair, of which 1 (17%) had unilateral sutural involvement and 5 (83%) had bilateral involvement. Of the patients with isolated SQS, 7 (32%) had a congenital syndrome and comprised 33% of patients who required surgical intervention. The nonsyndromic patients with isolated SQS who required surgery presented with a wide array of phenotypic findings; 3 patients underwent some form of cranial vault remodeling, whereas 1 patient underwent ventriculoperitoneal shunt only. Of the 4 nonsyndromic patients with isolated SQS who underwent surgical repair, half required operative intervention because of elevated intracranial pressure and the other half because of dysmorphic head shape.
CONCLUSION
The findings of this updated systematic review suggest a trend toward surgical management in bilateral SQS versus unilateral SQS, and that patients with isolated SQS, previously considered to be a nonsurgical finding, should be carefully monitored, as there remains risk of increased intracranial pressure. Pooled systematic review data suggest isolated SQS has a 27% operative intervention rate, with the presence of coexisting syndromic diagnoses increasing that risk.
Topics: Humans; Infant; Tomography, X-Ray Computed; Craniosynostoses; Skull; Cranial Sutures; Plastic Surgery Procedures; Retrospective Studies
PubMed: 37553899
DOI: 10.1097/SAP.0000000000003642 -
Child's Nervous System : ChNS :... Dec 2023This study provides a systematic review on cosmetic satisfaction and other patient-reported outcomes (PROMs) of patients who underwent surgical treatment of SSC. (Review)
Review
PURPOSE
This study provides a systematic review on cosmetic satisfaction and other patient-reported outcomes (PROMs) of patients who underwent surgical treatment of SSC.
METHODS
A systematic review of all articles published from inception to 1 June 2022 was performed. Articles were included if they reported on subjective assessment of cosmetic satisfaction or other PROMs by patients or their families using questionnaires or interviews.
RESULTS
Twelve articles, describing 724 surgical treatments of SSC, met the inclusion criteria. Cosmetic satisfaction was evaluated in the following ways: 1) use of the VAS score, binary questions or a 5-point scale to rate general, facial or skull appearance; 2) use of an aesthetic outcome staging in which personal opinion was added to the treating surgeon's opinion; and 3) use of an evaluation of anatomical proportions of the skull and face. A trend towards an overall improvement in cosmetic satisfaction following surgical treatment of SSC was observed. Reported PROMs included general health, socioeconomic status, patients' and their families' rating of the normalcy and noticeability of their appearance and how much this bothered them, and patients' answers to the Youth Quality of Life with Facial Differences (YQOL-FD) questionnaire. No clear overall trend of the reported PROMs was identified.
CONCLUSION
This systematic review illuminates that there is a wide variation in outcomes for evaluating cosmetic satisfaction and other PROMs of patients who underwent surgical treatment of SSC, suggesting that further research is needed to develop an inclusive and uniform approach to assess these outcomes.
Topics: Adolescent; Humans; Patient Satisfaction; Quality of Life; Craniosynostoses; Patient Reported Outcome Measures; Sutures; Personal Satisfaction
PubMed: 37477663
DOI: 10.1007/s00381-023-06063-3 -
The Cleft Palate-craniofacial Journal :... Mar 2024Lambdoid craniosynostosis affects approximately 1 in 33 000 live births per year, and surgical correction is often sought in order to achieve normocephaly and allow... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Lambdoid craniosynostosis affects approximately 1 in 33 000 live births per year, and surgical correction is often sought in order to achieve normocephaly and allow for adequate brain growth. However, the effects of lambdoid synostosis and its treatment on cognitive development are unknown.
DESIGN
Systematic review and meta-analysis.
PATIENTS, PARTICIPANTS
A systematic review of Ovid Medline, Embase, Web of Science, Scopus, Cochrane Central Register of Controlled Trials, and Clinicaltrials.gov was conducted in January 2022. Included studies assessed cognitive development of patients with nonsyndromic unilateral lambdoid craniosynostosis.
MAIN OUTCOME MEASURES
Results of developmental tests were compared to normative data or controls to generate Hedges' g for meta-analysis.
RESULTS
Nine studies met the inclusion criteria. Meta-analysis of 3 studies describing general cognition showed that cases scored significantly lower than their peers, but within 1 standard deviation ( = 0.37, 95% CI [-0.64, -0.10], = .01). Meta-analysis of verbal and psychomotor development showed no significant differences in children with lambdoid synostosis. Studies were of fair quality and had moderate-to-high heterogeneity.
CONCLUSIONS
Patients with lambdoid craniosynostosis may score slightly below average on tests of general cognition in comparison to normal controls, but results in other domains are variable. Analyses were limited by small sample sizes. Multidisciplinary care and involvement of a child psychologist may be helpful in identifying areas of concern and providing adequate scholastic support. Further research recruiting larger cohorts will be necessary to confirm these findings and extend them to other developmental domains such as attention and executive function.
Topics: Child; Humans; Craniosynostoses; Cognition; Head; Brain
PubMed: 36177519
DOI: 10.1177/10556656221129978 -
The British Journal of Oral &... Jan 2024This review provides a comprehensive overview of the literature on velopharyngeal insufficiency, associated anomalies, and speech/language impairment in patients with... (Review)
Review
This review provides a comprehensive overview of the literature on velopharyngeal insufficiency, associated anomalies, and speech/language impairment in patients with craniofacial microsomia (CFM). A systematic search of the literature was conducted to identify records on VPI and speech impairment in CFM from their inception until September 2022 within the databases Embase, PubMed, MEDLINE, Ovid, CINAHL EBSCO, Web of Science, Cochrane, and Google Scholar. Seventeen articles were included, analysing 1,253 patients. Velopharyngeal insufficiency results in hypernasality can lead to speech impairment. The reported prevalence of both velopharyngeal insufficiency and hypernasality ranged between 12.5% and 55%, while the reported prevalence of speech impairment in patients with CFM varied between 35.4% and 74%. Language problems were reported in 37% to 50% of patients. Speech therapy was documented in 45.5% to 59.6% of patients, while surgical treatment for velopharyngeal insufficiency consisted of pharyngeal flap surgery or pharyngoplasty and was reported in 31.6% to 100%. Cleft lip and/or palate was reported in 10% to 100% of patients with CFM; these patients were found to have worse speech results than those without cleft lip and/or palate. No consensus was found on patient characteristics associated with an increased risk of velopharyngeal insufficiency and speech/language impairment. Although velopharyngeal insufficiency is a less commonly reported characteristic of CFM than other malformations, it can cause speech impairment, which may contribute to delayed language development in patients with CFM. Therefore, timely recognition and treatment of speech impairment is essential.
Topics: Humans; Cleft Lip; Cleft Palate; Goldenhar Syndrome; Language Development Disorders; Retrospective Studies; Speech; Speech Disorders; Treatment Outcome; Velopharyngeal Insufficiency
PubMed: 38057178
DOI: 10.1016/j.bjoms.2023.09.008 -
Operative Neurosurgery (Hagerstown, Md.) Aug 2023Few studies have described a transmandibular approach for decompression in a patient with Klippel-Feil syndrome (KFS) for cervical myelopathy.
BACKGROUND
Few studies have described a transmandibular approach for decompression in a patient with Klippel-Feil syndrome (KFS) for cervical myelopathy.
OBJECTIVE
To describe the transmandibular approach in a KFS patient with cervical myelopathy and to perform a systematic review according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses.
METHODS
A systematic review was performed using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Embase and PubMed databases were searched from January 2002 to November 2022 for articles examining patients with KFS undergoing cervical decompression and/or fusion for cervical myelopathy and/or radiculopathy were included. Articles describing compression due to nonbony causes, lumbar/sacral surgery, nonhuman studies, or symptoms only from basilar invagination/impression were excluded. Data collected were sex, median age, Samartzis type, surgical approach, and postoperative complications.
RESULTS
A total of 27 studies were included, with 80 total patients. Thirty-three patients were female, and the median age ranged from 9 to 75 years. Forty-nine patients, 16 patients, and 13 patients were classified as Samartzis Types I, II, and III, respectively. Forty-five patients, 21 patients, and 6 patients underwent an anterior, posterior, and combined approach, respectively. Five postoperative complications were reported. One article reported a transmandibular approach for access to the cervical spine.
CONCLUSION
Patients with KFS are at risk of developing cervical myelopathy. Although KFS manifests heterogeneously and may be treated through a variety of approaches, some manifestations of KFS may preclude traditional approaches for decompression. Surgical exposure through the anterior mandible may prove an option for cervical decompression in patients with KFS.
Topics: Humans; Female; Child; Adolescent; Young Adult; Adult; Middle Aged; Aged; Male; Klippel-Feil Syndrome; Spinal Cord Compression; Cervical Vertebrae; Spinal Cord Diseases; Postoperative Complications
PubMed: 37219571
DOI: 10.1227/ons.0000000000000754 -
Developmental Biology Jan 2024Congenital craniofacial abnormalities are congenital anomalies of variable expressivity and severity with a recognizable set of abnormalities, which are derived from... (Review)
Review
Congenital craniofacial abnormalities are congenital anomalies of variable expressivity and severity with a recognizable set of abnormalities, which are derived from five identifiable primordial structures. They can occur unilaterally or bilaterally and include various malformations such as cleft lip with/without palate, craniosynostosis, and craniofacial microsomia. To date, the molecular etiology of craniofacial abnormalities is largely unknown. Noncoding RNAs (ncRNAs), including microRNAs, long ncRNAs, circular RNAs and PIWI-interacting RNAs, function as major regulators of cellular epigenetic hallmarks via regulation of various molecular and cellular processes. Recently, aberrant expression of ncRNAs has been implicated in many diseases, including craniofacial abnormalities. Consequently, this review focuses on the role and mechanism of ncRNAs in regulating craniofacial development in the hope of providing clues to identify potential therapeutic targets.
Topics: Humans; RNA, Untranslated; MicroRNAs; RNA, Long Noncoding; Craniofacial Abnormalities; Craniosynostoses
PubMed: 37923186
DOI: 10.1016/j.ydbio.2023.10.007