Craniofacial Syndromes Part I: Craniofacial Growth and Development, Craniosynostosis Syndromes, Craniofacial Microsomia, and Craniofacial Dysostoses

2020 ◽  
Author(s):  
Francesca Saldanha ◽  
Cory M. Resnick ◽  
Carolyn R. Rogers-Vizena
Keyword(s):  
Treatment Plan ◽  
Treacher Collins Syndrome ◽  
Apert Syndrome ◽  
Craniofacial Growth ◽  
Hemifacial Microsomia ◽  
Crouzon Syndrome ◽  
Treatment Needs ◽  
Craniofacial Microsomia ◽  
Nager Syndrome ◽  
Craniofacial Syndromes

Craniofacial syndromes are a diverse group of congenital disorders primarily affecting structures of the head and face.  Recent genetic advances have improved our ability to diagnosis specific syndromes, understand the molecular basis for abnormal embryogenesis, and anticipate future treatment needs. This is the first of a two-part series exploring the most common craniofacial disorders. This article will provide the embryologic and developmental foundation necessary to understand congenital craniofacial pathology. Clinical characteristics and molecular genetics needed to make an accurate diagnosis and formulate a treatment plan will be detailed for craniosynostosis syndromes, craniofacial microsomia, and craniofacial dysostoses. This review contains 13 figures, 4 tables, and 42 references. Keywords: craniofacial embryology, craniofacial growth, craniosynostosis, Apert syndrome, Crouzon syndrome, Pfieffer syndrome, craniofacial microsomia, hemifacial microsomia, Treacher Collins syndrome, Nager syndrome

The aetiology and pathogenesis of craniofacial deformity

Development ◽  
1988 ◽  
Vol 103 (Supplement) ◽  
pp. 207-212
Author(s):  
David Poswillo
Keyword(s):  
Neural Crest ◽  
Animal Studies ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Treacher Collins Syndrome ◽  
Apert Syndrome ◽  
Craniofacial Malformations ◽  
Craniofacial Microsomia ◽  
Animal System ◽  
Isolated Cleft Palate

Craniofacial malformations have been recorded since time immemorial. While observational studies have assisted in the recognition of syndromes, little light has been shed on the causal mechanisms which interfere with craniofacial development. Animal studies in which malformations occur spontaneously or have been induced by teratogenic agents have permitted step-by-step investigation of such common deformities as cleft lip and palate. The role of the ectomesenchymal cells of the neural crest and the possible phenomenon of disorganized spontaneous cell death are described in relation to lip clefts. The factors associated with isolated cleft palate, Pierre Robin syndrome and submucous clefts are described by reference to animal models. The haemorrhagic accident preceding the onset of craniofacial microsomia is discussed as is the distinctly different phenomenon of disturbance to the migration or differentiation of neural crest cells in the pathogenesis of Treacher Collins syndrome. The more severe anomalies of the calvarium such as plagiocephaly, Crouzon and Apert syndrome still defy explanation, in the absence of an appropriate animal system to study; some thoughts on the likely mechanism of abnormal sutural fusions are discussed.

Difficult Laryngoscopy/Intubation: The Child with Mandibular Hypoplasia

1983 ◽  
Vol 92 (4) ◽  
pp. 401-404 ◽  
Author(s):  
Steven D. Handler ◽  
Thomas P. Keon
Keyword(s):  
Temporomandibular Joint ◽  
Direct Laryngoscopy ◽  
Anterior Commissure ◽  
Difficult Laryngoscopy ◽  
Treacher Collins Syndrome ◽  
Pierre Robin Sequence ◽  
Hemifacial Microsomia ◽  
Mandibular Hypoplasia ◽  
Pierre Robin ◽  
Infants And Young Children

The child with mandibular hypoplasia (Treacher Collins syndrome, Pierre Robin sequence, hemifacial microsomia, etc) presents the otolaryngologist and anesthesiologist with considerable problems when direct laryngoscopy and/or endotracheal intubation is attempted. In addition to the small mandible, several other features of these patients contribute to the difficult laryngoscopy: macroglossia, glossoptosis, trismus related to temporomandibular joint abnormalities, and prominent maxilla or maxillary incisors. Most of the techniques that have been described for laryngoscopy/intubation in problem cases are difficult or impossible to use in infants and young children with mandibular hypoplasia. We present a modification of the standard direct laryngoscopic procedure, utilizing the 9-cm anterior commissure laryngoscope and an optical stylet in the task of exposing and intubating the larynx of a child with mandibular hypoplasia.

Craniofacial Syndromes Part II: Atypical Facial Clefts, Romberg Syndrome, Moebius Syndrome, Fibrous Dysplasia, and Neurofibromatosis

2020 ◽  
Author(s):  
Francesca Saldanha ◽  
Cory M. Resnick ◽  
Carolyn R. Rogers-Vizena
Keyword(s):  
Fibrous Dysplasia ◽  
Treatment Plan ◽  
Moebius Syndrome ◽  
Hemifacial Atrophy ◽  
Mccune Albright Syndrome ◽  
Facial Clefts ◽  
Progressive Hemifacial Atrophy ◽  
Parry Romberg Syndrome ◽  
Albright Syndrome ◽  
Craniofacial Syndromes

This final article of the two-part craniofacial series continues to provide the embryologic and developmental foundations necessary to understand congenital craniofacial pathology. Clinical characteristics and molecular genetics needed to make an accurate diagnosis and formulate a treatment plan will be detailed for atypical craniofacial clefts, Moebius Syndrome, Fibrous Dysplasia, Progressive Hemifacial Atrophy (Parry-Romberg syndrome) and Neurofibromatosis. Details of the operations applied in treatment of these disorders are discussed in separate Scientific American: Plastic Surgery (SAPS) articles as referenced. This review contains 3 tables, and 10 figures, and 42 references. Keywords: craniofacial, Tessier cleft, atypical facial cleft, Romberg syndrome, Parry-Romberg syndrome, Moebius syndrome, fibrous dysplasia, McCune Albright syndrome, neurofibromatosis

Facial Esthetics Classification and Gingival Pigmentation Index of Treatment Needs

2019 ◽  
Vol 11 (1) ◽  
pp. 77-88
Author(s):  
Puzhankara Lakshmi ◽  
Menon Sai Megha ◽  
Suresh Reshma
Keyword(s):  
Indian Population ◽  
Treatment Plan ◽  
Specific Treatment ◽  
Treatment Modalities ◽  
Treatment Needs ◽  
Complete Coverage ◽  
Facial Esthetics ◽  
Comprehensive Classification ◽  
Precise Assessment ◽  
Classification And Treatment

Background : The Indian population shows an extensive range of gingival pigmentation. A detailed description of the gingival pigmentation pattern would provide data for the formulation of an efficient plan for depigmentation. This article gives a novel comprehensive classification to aid in the precise assessment of the pattern and distribution of gingival pigmentation and decision regarding the methods for depigmentation. Methods : Previously existing, established indices and classifications for degree of pigmentation, facial complexion, smile line, lip colour and their modifications were used to differentiate various categories of Esthetics: Esthetic, Unaesthetic-mild, Unaesthetic-severe. Treatment needs were formulated based on the category of Esthetics. Results : The various categories of esthetics include E (Esthetic), M (Unaesthetic-Mild), N (UnaestheticSevere) and specific treatment modalities have been assigned based on the treatment needs. Discussion : The treatment for gingival pigmentation should be based on overall facial esthetics. The classification given in this article takes into consideration the components of facial esthetics: Facial Pigmentation, Degree and distribution of gingival pigmentation, smile line and lip colour. The treatment plan proposed considers the need for the treatment and the possible treatment for each category of esthetics. Conclusion : The classification proposed in this article attempts a complete coverage of combinations of facial esthetic components and the Treatment Index encompasses all the possible treatment modalities for each combination. Although a learning curve might exist, once mastered, the classification and treatment index would serve as a baseline for determining treatment needs in patients with gingival pigmentation.

scholarly journals Lack of association of cranial lacunae with intracranial hypertension in children with Crouzon syndrome and Apert syndrome: a 3D morphometric quantitative analysis

2019 ◽  
Vol 35 (3) ◽  
pp. 501-507
Author(s):  
Ankita Mondal ◽  
Naiara Rodriguez-Florez ◽  
Justine O’Hara ◽  
Juling Ong ◽  
N. u. Owase Jeelani ◽  
...  
Keyword(s):  
Quantitative Analysis ◽  
Intracranial Hypertension ◽  
Apert Syndrome ◽  
Crouzon Syndrome ◽  
Hypertension In Children

scholarly journals A review of documents assessing capacity and treatment needs to safeguard adults with incapacity

BJPsych Open ◽  
2021 ◽  
Vol 7 (S1) ◽  
pp. S82-S82
Author(s):  
Tereza Hoggard ◽  
Robin Holliday ◽  
Everett Julyan
Keyword(s):  
Treatment Plan ◽  
Healthcare Team ◽  
Treatment Needs ◽  
Legal Documents ◽  
Legal Standards ◽  
Vulnerable Patients ◽  
Legal Competence ◽  
Treatment Plans ◽  
Old Age Psychiatry ◽  
Clear Information

AimsTo audit the completion of Adults with Incapacity (AWI) documents (Assessment of Capacity, Section 47 Certificate of Incapacity and Treatment Plan) to ensure they met the legal standards required. We hypothesised that the forms were not all completed comprehensively, particularly with regards to the Treatment Plans.MethodIn addition to being legal documents, AWI documents provide an important framework to guide clinicians when giving treatment and balancing patient safety with patient autonomy. Correctly completed documents help provide vulnerable patients with ethical and lawful treatment that allows them to be treated with respect and dignity.An audit was conducted across two Old Age Psychiatry wards at Ayrshire Central Hospital during October 2020. We assessed all AWI documents available on the wards (n = 20) using criteria based on the standards set by the Mental Welfare Commission for Scotland to ensure legal competence.Result95% of the forms were signed and dated, and the nature of the incapacity was given in 100% of the documents. On the other hand, 35% of the forms gave no indication of the presence or absence of a guardian. Only one of those identified as having a guardian was consulted with regards to the treatment plan. Another member of staff was consulted on the Treatment Plan in 45% of cases. 30% of the Treatment Plans were not precisely worded enough to be considered justifiable for treatment. In the Certificate of Incapacity, two out-of-date certificates were found, and staff were notified immediately. 45% of certificates were considered over-generalised with regards to the description under medical treatment.ConclusionOverall, the forms were mostly signed and dated, with the nature of incapacity given. The two areas that appeared to be the most problematic were the issue of identifying and discussing plans with a guardian, and the specification of treatment covered by both the Certificate of Incapacity and the Treatment Plan.Discussion with members of the healthcare team found some confusion over how to complete the forms and many cited a lack of formal training as the main reason for their uncertainty. In addition, accessing clear information online or on the wards on how to complete the forms was challenging. We intend to improve the completion of these documents by implementing teaching and a guidance poster, based on the areas that we identified as being problematic, and completing the audit cycle.

“ABCD” Implant Classification: A Comprehensive Philosophy for Treatment Planning in Completely Edentulous Arches

2020 ◽  
Vol 46 (2) ◽  
pp. 93-99 ◽  
Author(s):  
Ali Tunkiwala ◽  
Udatta Kher ◽  
Nupur H. Vaidya
Keyword(s):  
Decision Making ◽  
Treatment Planning ◽  
Present Article ◽  
Treatment Plan ◽  
Bone Volume ◽  
Classification Systems ◽  
Decision Making Process ◽  
Careful Attention ◽  
Treatment Needs ◽  
Vital Parameters

A thorough and precise treatment plan that considers various factors such as age, availability of bone, interarch space for prosthesis design, smile line, lip support, patient desires, and economics is a necessity before implant surgery. Many previous classification systems for treatment planning in edentulous situations tend to focus on only a certain parameter such as esthetics, or available bone volume, or are specifically designed for the maxilla or mandible. The authors have proposed a simplified and universal ABCD classification that uses the 4 vital parameters of age, bone volume, cosmetic display, and degree of resorption to create an algorithm that satisfies the treatment needs of every patient. Various permutations of the 4 parameters can be used to arrive at a solution that streamlines the further phases of the rehabilitative process. The aim of the present article is to provide a science-driven approach to understand a patient's individual needs with careful attention to the interplay of all the aforementioned factors in the decision-making process.

scholarly journals Endodontic Treatment and Esthetic Management of a Geminated Central Incisor Bearing a Talon Cusp

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Elif Tarım Ertaş ◽  
Meral Yırcalı Atıcı ◽  
Hakan Arslan ◽  
Bilal Yaşa ◽  
Hüseyin Ertaş
Keyword(s):  
Case Report ◽  
Multidisciplinary Approach ◽  
Treatment Plan ◽  
Specific Treatment ◽  
Central Incisor ◽  
Dental Anomalies ◽  
Treatment Needs ◽  
Radiologic Diagnosis ◽  
Radiographic Findings ◽  
Clinical Problems

Gemination with talon cusps is an uncommon morphologic dental anomaly, characterized by the formation of clinically wide tooth that can cause significant aesthetic and clinical problems including esthetic impairment, pain, caries susceptibility, and tooth crowding. These morphological dental anomalies have specific treatment needs due to the abnormal morphology and need virtuous radiologic diagnosis. Multidisciplinary approach can supply success of the treatment plan that can provide esthetic and occlusal requirements. In this case report, the multidisciplinary approach for the treatment of geminated tooth with talon cusp is presented with the clinical and radiographic findings.

Facial Growth in Patients with Apert and Crouzon Syndromes Compared to Normal Children

2012 ◽  
Vol 49 (2) ◽  
pp. 185-193 ◽  
Author(s):  
Jacobus H. Reitsma ◽  
Edwin M. Ongkosuwito ◽  
Peter H. Buschang ◽  
Birte Prahl-Andersen
Keyword(s):  
Growth Patterns ◽  
Apert Syndrome ◽  
Control Group ◽  
Crouzon Syndrome ◽  
Facial Growth ◽  
Normal Children ◽  
Control Subjects ◽  
Facial Height ◽  
Anterior Cranial Base ◽  
Lateral Cephalograms

Objective To evaluate vertical and sagittal facial growth in children with Apert and Crouzon syndromes and compare it to the growth patterns of a nonsyndromic control group. Design Case-control study. Setting Department of Orthodontics, Children's Hospital Erasmus Medical Centre, Sophia, Rotterdam, The Netherlands. Patients, Participants Sixty-two patients (37 patients with Crouzon syndrome and 25 patients with Apert syndrome) born between 1971 and 2001 (age range 3.9 to 32 years) and 482 nonsyndromic children as a control group. Interventions Lateral cephalograms performed prior to any midfacial surgery of 62 patients and 482 nonsyndromic children were traced and horizontal and vertical measurements were digitized. Main Outcome Measures Cephalometric measurements of SNA, SNB, ANB, NSMe, and SN/palatal plane angles and lower facial height ratio. Results Horizontal measurements for the syndromic groups showed no change in SNA angle during growth. SNA angles were lower in patients with Apert syndrome compared to patients with Crouzon syndrome. The syndromic groups showed smaller values for ANB angles compared to the nonsyndromic group. Vertical measurements showed increased lower facial height ratios for the syndromic groups compared to control subjects. There was an increasing counterclockwise rotation of the palatal plane in relation to the anterior cranial base in syndromic patients. NSMe angles among the three groups were not significantly different. Conclusions Based on the growth differences identified, the sagittal and vertical jaw relationships differ in patients with Crouzon syndrome, patients with Apert syndrome, and control subjects. Syndromic patients show aggravation of midfacial underdevelopment and anterior rotation of the mandible.

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