A New Classification Based on the Kaban's Modification for Surgical Management of Craniofacial Microsomia.
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ABSTRACT: In medicine, classifications are designed to describe accurately and reliably all anatomic and structural components, establish a prognosis, and guide a given treatment. Classifications should be useful in a universal way to facilitate communication between health professionals and to formulate management protocols. In many situations and particularly with craniofacial microsomia, there have been many different classifications that do not achieve this goal. In fact, when there are so many classifications, one can conclude that there is not a clear one that accomplishes all these ends and defines a treatment protocol. It is our intent to present a new classification based on the Pruzansky's classification, later modified by Kaban, to determine treatment protocols based on the degree of osseous deficiency present in the body, ramus, and temporomandibular joint. Different mandibular defects are presented in two patients with craniofacial microsomia type III and IV according to our classification with the corresponding management proposed for each type and adequate functional results.
Project description:Craniofacial anomalies are some of the most variable and common defects affecting the population. Herein, we examine a group of craniofacial disorders that are the result of defects in primary cilia; ubiquitous, microtubule-based organelles that transduce molecular signals and facilitate the interactions between the cell and its environment. Based on the frequent appearance of craniofacial phenotypes in diseases born from defective primary cilia (ciliopathies) we propose a new class of craniofacial disorders referred to as craniofacial ciliopathies. We explore the most frequent phenotypes associated with ciliopathic conditions and the ciliary gene mutations responsible for craniofacial defects. Finally, we propose that some non-classified disorders may now be classified as craniofacial ciliopathies.
Project description:Cleidocranial dysplasia (CCD, MIM 119600) is a rare autosomal dominant disorder affecting bone, cartilage, craniofacial growth, and tooth formation leading to supernumerary teeth. Few reports delineate the genotype-phenotype correlations related to the variations in craniofacial morphology and patterning of the dentition and the complexity of treating patient's malocclusion. Successful management of the craniofacial deformities in patients with CCD requires a multidisciplinary team of healthcare specialists. Approximately 70% of patients are due to point mutations in RUNX2 and <20% due to copy number variations with the remainder unidentified. There is no literature to date, describing the orthognathic management of CCD patients with deletion in one of the RUNX2 alleles. The purpose of this study was to evaluate the craniofacial morphology and dental patterning in a 14-year-old Caucasian female with CCD resulting from a novel microdeletion of RUNX2 in 1 allele. The CCD patient with RUNX2 haploinsufficiency due to microdeletion had decreased craniofacial bone and ankyloses in the permanent dentition. An altered extraction protocol of supernumerary teeth was followed in this patient. Craniofacial growth and morphologic analysis demonstrated atypical skull shape, persistent metopic suture, and decreased mandibular size.
Project description:The complex three-dimensional anatomy of the craniofacial skeleton creates a formidable challenge for surgical reconstruction. Advances in computer-aided design and computer-aided manufacturing technology have created increasing applications for virtual surgical planning in craniofacial surgery, such as preoperative planning, fabrication of cutting guides, and stereolithographic models and fabrication of custom implants. In this review, the authors describe current and evolving uses of virtual surgical planning in craniofacial surgery.
Project description:A new classification is proposed for cerebral aneurysms treated with any endovascular technique, for example, coiling with or without adjunctive devices, flow diversion, intrasaccular flow modifiers, or any combination of the above. Raymond-Roy Occlusion Classification is expanded with novel subgroups such as class 1 represents complete occlusion and is subdivided if a branch is integrated to, or originated from, the aneurysm sac; class 2 represents neck filling; class 3 represents incomplete occlusion with aneurysm filling as in the previous classification; and class 4 describes the immediate postoperative status after extra- or intrasaccular flow modification treatment. A new concept, "stable remodeling," is included as class 5, which represents filling in the neck region that stays unchanged or reduced, as shown with at least 2 consecutive control angiographies, at least 6 months apart, for not <1 year, or the remodeled appearance of a dilated and/or tortuous vessel in continuation with the parent artery without sac filling.
Project description:PurposeTo report surgical repair of a rare case of Tessier number 9 craniofacial cleft.MethodsCase report.ResultsTessier number 9 craniofacial cleft is the rarest cleft anomaly. This article reports a congenital eyelid coloboma in a 21-year-old woman that involved the lateral third of the left upper eyelid and extended to the lateral canthus, consistent with number 9 craniofacial cleft Tessier classification. The additional findings included a fibrotic band between the globe and the remnant of the upper lid, which caused a small-angle exotropia. There were also skin appendages in the preauricular area and the inner surface of the nasal columella consistent with Goldenhar syndrome. The eyelid coloboma was repaired by releasing the adhesions and using a composite graft of the hard palate to repair the posterior lamella. The anterior lamella was repaired by creating a skin advancement flap. The esthetic and functional outcomes were acceptable in the 2-year postoperative follow-up period.ConclusionThe composite hard palate graft can be used to repair posterior lamella defect in the case of Tessier number 9 craniofacial cleft.
Project description:ImportanceQuantifying severity of complications in rhegmatogenous retinal detachment (RRD) surgical trials is needed. A consensus classification system will help surgeons to more effectively compare harms of different surgical techniques.ObjectiveTo develop a new consensus-based classification to quantify severity of complications of RRD surgery.Design, setting, and participantsA comprehensive list of complications was developed followed by a Delphi consensus survey of international vitreoretinal surgeons. The survey was conducted in 17 countries in mainland Europe, the United Kingdom, the United States, Asia, South Africa, and Australia. Seventy vitreoretinal surgeons were invited to take part in the Delphi survey; 45 agreed to participate. Participants were selected through boards/members lists of retinal societies. Data were analyzed between April 2019 and August 2019.Main outcomes and measuresConsensus-derived classification of complications of RRD surgery, according to their severity.ResultsForty-three of 45 vitreoretinal surgeons who agreed to participate in the Delphi survey completed round 1 (96%); all but 1 (98%) completed round 2. Consensus was reached for 96% of the 84 complications assessed. Examples of complications classified as least severe (graded 1) included subconjunctival hemorrhage and chemosis while those classified as most severe included endophthalmitis and sympathetic ophthalmia (graded 9) and phthisis (graded 10).Conclusions and relevanceWe propose a new classification for quantifying severity of surgical complications based on an international consensus of vitreoretinal surgeons to quantify harm and improve the reporting of complications of RRD surgery.
Project description:The goals of this retrospective case review were to analyze the long-term results of surgery for petrous temporal bone cholesteatomas and to propose a new classification system for these lesions. Patients with a surgically confirmed petrous temporal bone cholesteatoma were treated at Addenbrooke's Hospital, a tertiary referral center. Postoperative facial function, hearing, residual/recurrent cholesteatoma, and other complications were assessed in relation to preoperative signs, intraoperative findings, and surgical approach. Between 1983 and 2004, 43 patients were treated. There were no perioperative deaths. There was no long-term recurrence in 95.4% of the patients, possibly because of meticulous surgical technique, bipolar diathermy, and use of the laser to denature the cholesteatoma matrix that was adherent to the dura. At presentation, 95% of the patients had no socially useful hearing in the affected ear. Facial nerve function, however, was usually preserved. Both direct anastomosis and nerve grafting can improve facial nerve function from House-Brackmann grade VI to grade III if the palsy is not longstanding. Four patients had cerebrospinal fluid leakage; other complications were rare. The proposed classification facilitates surgical planning and predicts the postoperative outcome with regards to hearing.
Project description:The aim of the present study was to propose a new pCLE classification of gastric pit patterns and vessel architecture, and to assess the accuracy and interobserver agreement of this new pCLE classification system in the stomach.
Project description:Development of the human skull and face is a highly orchestrated and complex three-dimensional morphogenetic process, involving hundreds of genes controlling the coordinated patterning, proliferation and differentiation of tissues having multiple embryological origins. Craniofacial malformations that occur because of abnormal development (including cleft lip and/or palate, craniosynostosis and facial dysostoses), comprise over one-third of all congenital birth defects. High-throughput sequencing has recently led to the identification of many new causative disease genes and functional studies have clarified their mechanisms of action. We present recent findings in craniofacial genetics and discuss how this information together with developmental studies in animal models is helping to increase understanding of normal craniofacial development.
Project description:AimTo compare three periodontitis clusters (A, B and C) for alveolar bone loss (ABL) patterns, antibiotic prescriptions and surgeries and to relate them to the new classification of periodontitis.Materials and methodsABL patterns, prescription of systemic antibiotics and the number of surgeries were retrieved for all patients (n = 353) in the clusters. Comparisons and possible predictors for antibiotics were assessed, and results also evaluated in relation to the new classification.ResultsCluster A is characterized by angular defects often affecting the first molars and localized stage III/IV grade C periodontitis. Cluster B contains mainly localized or generalized stage III/IV, grade C patients. Cluster C contains mainly patients with generalized stage III/IV grade C periodontitis. Patients in cluster A received significantly more antibiotics compared to B and C (78% vs. 23% and 17%); the predictors for antibiotic prescription were young age and localized ABL. No differences in numbers of periodontal surgeries were observed between clusters (A = 1.0 ± 1.4, B = 1.3 ± 1.4 and C = 1.3 ± 1.5).ConclusionsWithin stage III/IV grade C periodontitis, we could detect three clusters of patients. The distinct localized ABL pattern and younger age in cluster A presumably prompted clinicians to prescribe antibiotics.