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Child's Nervous System : ChNS :... May 2022While the focus of craniosynostosis surgery is to improve head shape, neurocognitive sequelae are common and are incompletely understood. Neurodevelopmental problems... (Review)
Review
While the focus of craniosynostosis surgery is to improve head shape, neurocognitive sequelae are common and are incompletely understood. Neurodevelopmental problems that children with craniosynostosis face include cognitive and language impairments, motor delays or deficits, learning disabilities, executive dysfunction, and behavioral problems. Studies have shown that children with multiple suture craniosynostosis have more impairment than children with single-suture craniosynostosis. Children with isolated single-suture subtypes of craniosynostosis such as sagittal, metopic, and unicoronal craniosynostosis can have distinct neurocognitive profiles. In this review, we discuss the unique neurodevelopmental profiles of children with single-suture subtypes of craniosynostosis.
Topics: Child; Craniosynostoses; Facial Bones; Humans; Neurosurgical Procedures; Sutures
PubMed: 35192026
DOI: 10.1007/s00381-022-05448-0 -
Oral and Maxillofacial Surgery Clinics... Aug 2022The prevalence of sagittal and lambdoid suture craniosynostosis differs considerably, as they are notably the most and least prevalent sutures involved in isolated... (Review)
Review
The prevalence of sagittal and lambdoid suture craniosynostosis differs considerably, as they are notably the most and least prevalent sutures involved in isolated suture craniosynostosis, respectively. The goals of reconstructing the cranial vault in both entities is the same: to release the fused suture, expand cranial volume, restore normal head shape and morphology, and allow for normal growth of the cranial vault. With regards to sagittal suture synostosis, opinions vary on whether reconstruction should focus on either the anterior or poster cranial vault. In contrast, the poster cranial vault is always targeted in lambdoid suture craniosynostosis.
Topics: Cranial Sutures; Craniosynostoses; Humans; Infant; Skull; Tomography, X-Ray Computed
PubMed: 35752548
DOI: 10.1016/j.coms.2022.01.005 -
European Journal of Human Genetics :... Jun 2023
Topics: Humans; Craniosynostoses; Smad6 Protein
PubMed: 36797468
DOI: 10.1038/s41431-023-01317-9 -
Ophthalmic Plastic and Reconstructive...
Topics: Humans; Craniofacial Dysostosis; Abnormalities, Multiple
PubMed: 35657657
DOI: 10.1097/IOP.0000000000002207 -
The Indian Journal of Medical Research Mar 2022Ocular hypertelorism constitutes an important component of many clinical syndromes. It is typically recommended to use inter-pupillary distance (IPD) for objective...
BACKGROUND & OBJECTIVES
Ocular hypertelorism constitutes an important component of many clinical syndromes. It is typically recommended to use inter-pupillary distance (IPD) for objective evaluation of ocular hypo/hypertelorism. Barring infancy, there is a scarcity of data on this anthropometric parameter relating to the ocular apparatus. This study aims to study auxological dynamics of IPD in children of Indian origin.
METHODS
A total of 3622 ( 2239 males and, 1383 females) normal, healthy Indian children of North-western origin, aged one month to 14 yr comprised the sample for this study. Inner and outer-canthal distance were measured using standardized anthropometric techniques. None of the children who participated in this study had craniofacial dysmorphism or any body deformity. Mean (standard deviation SD) and percentiles were calculated for IPD in male and female subjects at different age levels.
RESULTS
IPD increased from 4.68±0.21 to 6.19±0.36 cm in males and from 4.59±0.26 to 6.08±0.25 cm in females between one month and 14 yr of age. Boys in general, possessed larger IPD than girls, however, the gender differences became significant (P≤0.05) at 10, 11, 16-18 and 22-24 months, respectively, and five and 10 yr of age, respectively.
INTERPRETATION & CONCLUSIONS
The results of this study suggest that the patients having IPD less than the 3 percentile should be treated as cases of hypotelorism while, those exceeding 97 percentile as cases of hypertelorism. The use of percentile grids presented for IPD may be used to detect ocular hypotelorism and hypertelorism in male and female children to corroborate diagnosis of different syndromes.
Topics: Child; Face; Female; Humans; Hypertelorism; India; Male; Pupil; Reference Values
PubMed: 36124511
DOI: 10.4103/ijmr.IJMR_758_20 -
Clinics in Plastic Surgery Jul 2021Monobloc and bipartition advancement by external distraction plays a major role in the treatment of syndromic craniosynostosis. They can reverse the associated facial... (Review)
Review
Monobloc and bipartition advancement by external distraction plays a major role in the treatment of syndromic craniosynostosis. They can reverse the associated facial deformity and play a role in the management of ocular exposure, intracranial hypertension, and upper airway obstruction. Facial bipartition distraction corrects the intrinsic facial deformities of Apert syndrome. Both procedures are associated with relatively high complication rates principally related to ascending infection and persistent cerebrospinal fluid leaks. Modern perioperative management has resulted in a significant decline in complications. External distractors allow fine tuning of distraction vectors and improve outcome but are less well tolerated than internal distractors.
Topics: Acrocephalosyndactylia; Craniofacial Dysostosis; Facial Bones; Female; Humans; Male; Osteogenesis, Distraction
PubMed: 34051902
DOI: 10.1016/j.cps.2021.03.004 -
Child's Nervous System : ChNS :... Nov 2021Hydrocephalus is variously associated to syndromic craniosynostosis (CS), while it is randomly encountered in monosutural CS. Pathogenesis is still debated and reliable...
INTRODUCTION
Hydrocephalus is variously associated to syndromic craniosynostosis (CS), while it is randomly encountered in monosutural CS. Pathogenesis is still debated and reliable criteria for the diagnosis of overt hydrocephalus are lacking. Additionally, optimal treatment is controversial since it should balance the need to relieve intracranial hypertension and the risk of recurrence favored by lowering intracranial pressure.
METHODS
A thorough review of the literature has been performed. Accordingly, pathogenic theories, diagnostic issues, and treatment options on hydrocephalus presenting in the context of CS are discussed.
RESULTS
The association of hydrocephalus to simple CS is considered a fortuitous event. Its treatment is usually driven by the etiology and clinical relevance of hydrocephalus, favoring treatment before surgical correction to reduce CSF-related complications. On the other side, pathogenesis of hydrocephalus in the context of syndromic CS has been mainly related to factors that are secondary to the synostostic process, such as craniocerebral disproportion and venous hypertension. Hydrocephalus complicates 12-15% of syndromic CS, though its incidence is more relevant in FGFR2-related CS and raises up to 88% in Pfeiffer syndrome. Overt hydrocephalus should be properly differentiated by non-tense ventriculomegaly that is more frequent in Apert syndrome. Since intracranial hypertension is constant in syndromic CS even in the absence of active hydrocephalus, radiological monitoring of ventricular size along with intracranial pressure monitoring is essential. Active hydrocephalus occurs more frequently in infants, though stable ventriculomegaly may evolve into overt hydrocephalus after cranial expansion. If hydrocephalus is not clinically prominent, cranial expansion should be favored as first surgical step. Although posterior cranial expansion may address posterior cranial fossa constriction and stabilize ventricular dilation, effectiveness in long-term control of hydrocephalus is not clear. ETV is an effective treatment option, though success rate is affected by the presence of brain malformations and patient age. Extrathecal CSF shunting should be used as last resource due to the increased risk of complications in this context.
CONCLUSIONS
The pathogenesis of hydrocephalus complicating syndromic CS should be further investigated. Concomitantly, the definition of reliable diagnostic criteria is advocated in order to promptly and properly identify active hydrocephalus. Finally, treatment algorithm should refine the best timing and treatment options aiming to relieve intracranial hypertension on one side and reduce the risk of restenosis on the other side.
Topics: Acrocephalosyndactylia; Craniosynostoses; Humans; Hydrocephalus; Infant; Neoplasm Recurrence, Local; Skull
PubMed: 33829280
DOI: 10.1007/s00381-021-05158-z -
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 -
The Pan African Medical Journal 2023
Topics: Humans; Acrocephalosyndactylia; Syndactyly; Fingers; Toes
PubMed: 37521759
DOI: 10.11604/pamj.2023.45.24.38946 -
Plastic and Reconstructive Surgery May 2022The purpose of this study was to detail perioperative ophthalmologic evaluations to characterize functional ocular outcomes after facial bipartition surgery.
BACKGROUND
The purpose of this study was to detail perioperative ophthalmologic evaluations to characterize functional ocular outcomes after facial bipartition surgery.
METHODS
Patients with hypertelorbitism who underwent facial bipartition surgery were studied specifically for eye motility disorders by separating patients into rare craniofacial clefts (midline and paramedian) (n = 34) and craniofacial dysostosis (Apert, Crouzon, and Pfeiffer) (n = 74). Preoperative and postoperative (12 months) ophthalmologic examinations (with depth perception tests), computed tomography scans, and magnetic resonance imaging scans were analyzed.
RESULTS
Among craniofacial cleft patients, mean interdacryon distance was reduced from 39 ± 4 mm to 17 ± 2 mm, with strabismus improved from 88 percent (exotropia 82 percent) preoperatively to only 29 percent postoperatively. Depth perception improved to a lesser degree, with abnormal tests at a rate of 79 percent preoperatively to 56 percent postoperatively. Wider hypertelorbitism had a higher degree of strabismus. Among craniofacial dysostotic patients, mean interdacryon distance was reduced from 37 ± 3 mm to 17 ± 2 mm, and strabismus improved from 55 percent to only 14 percent. Depth perception improved to a lesser degree, with 68 percent abnormal tests preoperatively and 46 percent postoperatively. Apert patients had more V-pattern strabismus and exotropia (79 percent) than did other craniofacial dysostosis patients (42 percent).
CONCLUSIONS
The authors' data indicate that facial bipartition for hypertelorbitism-known to improve periorbital aesthetics-also improves eye motility disturbances. Thus, vision problems related to exotropia should be considered a functional indication for facial bipartition surgery in patients with hypertelorbitism.
CLINICAL QUESTION/LEVEL OF EVIDENCE
Therapeutic, IV.
Topics: Acrocephalosyndactylia; Craniofacial Dysostosis; Exotropia; Face; Humans; Tomography, X-Ray Computed
PubMed: 35286295
DOI: 10.1097/PRS.0000000000009041