Database : MEDLINE
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[PMID]: 29498494
[Au] Autor:Musani V; Ozretic P; Trnski D; Sabol M; Poduje S; Tosic M; Situm M; Levanat S
[Ad] Address:Vesna Musani, Division of Molecular Medicine, Ruder Boskovic Institute, Bijenicka 54, 10000 Zagreb, Croatia, vmusani@irb.hr.
[Ti] Title:Potential hot spot for de novo mutations in PTCH1 gene in Gorlin syndrome patients: a case report of twins from Croatia.
[So] Source:Croat Med J;59(1):20-24, 2018 Feb 28.
[Is] ISSN:1332-8166
[Cp] Country of publication:Croatia
[La] Language:eng
[Ab] Abstract:We describe a case of twins with sporadic Gorlin syndrome. Both twins had common Gorlin syndrome features including calcification of the falx cerebri, multiple jaw keratocysts, and multiple basal cell carcinomas, but with different expressivity. One brother also had benign testicular mesothelioma. We propose this tumor type as a possible new feature of Gorlin syndrome. Gorlin syndrome is a rare autosomal dominant disorder characterized by both developmental abnormalities and cancer predisposition, with variable expression of various developmental abnormalities and different types of tumors. The syndrome is primarily caused by mutations in the Patched 1 (PTCH1) gene, although rare mutations of Patched 2 (PTCH2) or Suppressor of Fused (SUFU) genes have also been found. Neither founder mutations nor hot spot locations have been described for PTCH1 in Gorlin syndrome patients. Although de novo mutations of the PTCH1 gene occur in almost 50% of Gorlin syndrome cases, there are a few recurrent mutations. Our twin patients were carriers of a de novo mutation in the PTCH1 gene, c.3364_3365delAT (p.Met1122ValfsX22). This is, to our knowledge, the first Gorlin syndrome-causing mutation that has been reported four independent times in distant geographical locations. Therefore, we propose the location of the described mutation as a potential hot spot for mutations in PTCH1.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180307
[Lr] Last revision date:180307
[St] Status:In-Process

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[PMID]: 29390315
[Au] Autor:Wang HW; Ma CY; Qin XJ; Zhang CP
[Ad] Address:Department of Oral and Maxillofacial-Head and Neck Oncology, Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai Key Laboratory of Stomatology, Shanghai, China.
[Ti] Title:Management strategy in patient with familial gigantiform cementoma: A case report and analysis of the literature.
[So] Source:Medicine (Baltimore);96(50):e9138, 2017 Dec.
[Is] ISSN:1536-5964
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:RATIONALE: Familial gigantiform cementoma (FGC) is a rare benign autosomal dominant fibrocemento-osseous lesion generally limited to the facial bones, typically in the anterior portion of the mandible; it is often associated with abnormalities of the long bones and prepubertal pathologic fractures. Owing to the small number of such patients, a uniform treatment criterion has not been established. This paper presents a patient with FGC who was treated in our department, and offers a systematic review of the patients reported in the literature. Our aim was to explore the treatment strategy for patients with FGC. PATIENT CONCERNS: Our patient, a 13-year-old boy, presented with a painless enlargement of the mandible first noted 2 years earlier. It had grown rapidly over the preceding 8 months, affecting both his appearance and ability to chew. DIAGNOSIS: Based on the pathologic, clinical, and radiographic features, FGC was diagnosed. INTERVENTIONS: Mandibuloectomy was performed. The mandibular defect was immediately reconstructed with his right vascularized iliac crest flap. At the same time, a PubMed search was conducted to identify studies reporting on other patients with FGC. OUTCOMES: A 3-dimensional computed tomography (3D-CT) scan demonstrated appropriate height of the new alveolar bone. Follow-up results showed recovery of the patient's appearance and mandibular function. He was free of recurrence at 4-year follow-up. LESSONS: FGC is a rare benign fibrocemento-osseous lesion of the jaws that can cause severe facial deformity. Incomplete removal leads to more rapid growth of the residual lesion. Therefore, extensive resection is a suitable strategy to avoid recurrence. Defects of the facial bones found intraoperatively should be repaired with resort to an appropriate donor site. However, it is important to be aware that patients with FGC always have concomitant abnormalities of skeletal metabolism and structure, as well as a vulnerability to fractures of the long bones of the lower extremity. Therefore, the optimal management strategy should include a review of treatment options for other patients as reported in the literature. An optimal protocol can not only provide sufficient high-quality bone suitable for the reconstruction of bone defects, but also minimize complications and maximize quality of life.
[Mh] MeSH terms primary: Cementoma/diagnosis
Cementoma/surgery
Jaw Neoplasms/diagnosis
Jaw Neoplasms/surgery
Mandibular Neoplasms/diagnosis
Mandibular Neoplasms/surgery
[Mh] MeSH terms secundary: Adolescent
Bone Transplantation
Cementoma/diagnostic imaging
Diagnosis, Differential
Humans
Ilium/transplantation
Imaging, Three-Dimensional
Jaw Neoplasms/diagnostic imaging
Male
Mandibular Neoplasms/diagnostic imaging
Mandibular Reconstruction
Surgical Flaps
Tomography, X-Ray Computed
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Em] Entry month:1802
[Cu] Class update date: 180301
[Lr] Last revision date:180301
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:180203
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000009138

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[PMID]: 29480247
[Au] Autor:Surve A; Meel R; Pushker N; Bajaj MS
[Ad] Address:Dr. R. P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India.
[Ti] Title:Ultrasound biomicroscopy image patterns in normal upper eyelid and congenital ptosis in the Indian population.
[So] Source:Indian J Ophthalmol;66(3):383-388, 2018 Mar.
[Is] ISSN:1998-3689
[Cp] Country of publication:India
[La] Language:eng
[Ab] Abstract:Purpose: To study the features of upper eyelid in healthy individual and different types of congenital ptosis in the Indian population using ultrasound biomicroscopy (UBM). Methods: This was a prospective observational study at a tertiary care center. Eyelid structure of healthy individuals with no eyelid abnormalities (n = 19); simple congenital ptosis (n = 33) cases; Marcus Gunn jaw-winking ptosis (MGJWP, n = 7) cases, and blepharophimosis-ptosis-epicanthus inversus syndrome (BPES, n = 20) cases were studied on a vertical UBM scan using 50-MHz probe. Lid-thickness, tarsal-thickness, orbicularis oculi and levator-Muller-orbital septum-conjunctival (LMSC) complex were measured in primary gaze. Comparison was made between four groups and results were statistically analyzed using ANOVA test. In normal individuals, LMSC measurements were repeated in down-gaze imaging. Results: Skin with subcutaneous tissue, LMSC complex and pre-aponeurotic fat-pad appeared echodense while orbicularis oculi and tarsus appeared echolucent. In primary gaze, mean thickness (± standard deviation) of the eyelid, tarsus, orbicularis oculi and LMSC, respectively, were: 1.612 ± 0.205, 0.907 ± 0.098, 0.336 ± 0.083, and 0.785 ± 0.135 mm in normal individual. LMSC showed 46.64% increase in thickness on down-gaze. The mean eyelid thickness and LMSC were thicker in MGJWP and BPES as compared to normal. In different types of congenital ptosis cases, various patterns of UBM imaging were observed. Conclusion: UBM allows noninvasive imaging of eyelid structures with good anatomical correspondence in normal eyelids and study the structural alterations of eyelids in different types of congenital ptosis. UBM can be used to highlight the anatomical difference in normal eyelids that may help modify the surgery for better cosmetic outcomes. Furthermore, it has the potential to be used in preoperative evaluation and operative planning in certain types of acquired ptosis, which needs to be evaluated.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180226
[Lr] Last revision date:180226
[St] Status:In-Data-Review
[do] DOI:10.4103/ijo.IJO_915_17

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[PMID]: 29390531
[Au] Autor:Tu Y; Gao F
[Ad] Address:Department of Anesthesiology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, PR China.
[Ti] Title:Dexmedetomidine-based monitored conscious sedation combined local anesthesia for levator resection in a 10-year-old child with Marcus Gunn jaw-winking synkinesis: A case report.
[So] Source:Medicine (Baltimore);96(51):e9369, 2017 Dec.
[Is] ISSN:1536-5964
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:RATIONALE: Levator resection has become a routine procedure for patients with severe Marcus Gunn jaw-winking synkinesis (MGJWS). To optimize the surgical outcome, adult patients need to be kept awake, or easily aroused and responsive to verbal commands during the operation. However, levator resection is commonly performed under general anesthesia in pediatric patients. In the present case, we described a successful anesthetic protocol of conscious sedation with local anesthesia for levator resection in a child. PATIENT CONCERNS: A 10-year-old boy with MGJWS was admitted to our hospital and scheduled for levator resection. The patient was born through a normal delivery and had no previous history of allergy, no comorbidity, and no history of receiving anesthesia or operation. The laboratory tests of the patient were unremarkable. DIAGNOSES: The diagnosis of MGJWS was made by two experienced ophthalmologists. INTERVENTIONS: A 10-year-old boy with MGJWS was admitted to our hospital and scheduled for levator resection. The levator resection was performed under monitored conscious sedation with dexmedetomidine and local anesthesia. OUTCOMES: Patient with spontaneous breathing responded normally to verbal commands throughout the operation, and no adverse events occurred. The patient and ophthalmologist reported high satisfaction with anesthesia management. LESSONS: Dexmedetomidine-based monitored conscious sedation with local anesthesia is a feasible alternative to general anesthesia for levator resection in collaborative patients.
[Mh] MeSH terms primary: Blepharoptosis/diagnosis
Blepharoptosis/therapy
Dexmedetomidine/administration & dosage
Heart Defects, Congenital/diagnosis
Heart Defects, Congenital/therapy
Jaw Abnormalities/diagnosis
Jaw Abnormalities/therapy
Monitoring, Physiologic/methods
Nervous System Diseases/diagnosis
Nervous System Diseases/therapy
Oculomotor Muscles/surgery
[Mh] MeSH terms secundary: Anesthesia, Local
Child
Conscious Sedation/methods
Follow-Up Studies
Humans
Male
Rare Diseases
Reflex, Abnormal
Treatment Outcome
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Nm] Name of substance:67VB76HONO (Dexmedetomidine)
[Em] Entry month:1802
[Cu] Class update date: 180219
[Lr] Last revision date:180219
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:180203
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000009369

  5 / 4131 MEDLINE  
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[PMID]: 29447804
[Au] Autor:Akakpo K; Iobst C; Old M; Grischkan J
[Ad] Address:The Ohio State University College of Medicine, Columbus, OH 43201, USA.
[Ti] Title:Long-term follow-up with mention of complications in pediatric microvascular mandibular reconstruction.
[So] Source:Int J Pediatr Otorhinolaryngol;105:154-157, 2018 Feb.
[Is] ISSN:1872-8464
[Cp] Country of publication:Ireland
[La] Language:eng
[Ab] Abstract:The fibular free flap has become the gold standard for mandibular reconstruction. Despite its vast array of benefits, this procedure has the potential for several complications. In the pediatric setting, the immature skeleton sets the stage for an increased risk of future skeletal abnormalities, both in the jaw, and in the donor site. Herein we describe the case of a toddler who underwent mandibular reconstruction using a fibula free flap and, years later, subsequently developed ankle instability as a result of residual fibula migration. This indicates the need for careful consideration of long-term issues in the growing pediatric skeleton prior to surgery.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180215
[Lr] Last revision date:180215
[St] Status:In-Process

  6 / 4131 MEDLINE  
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[PMID]: 29407505
[Au] Autor:Nardi C; De Falco L; Selvi V; Lorini C; Calistri L; Colagrande S
[Ad] Address:Department of Experimental and Clinical Biomedical Sciences, Radiodiagnostic Unit number 2, University of Florence, Azienda Ospedaliero, Universitaria Careggi, Florence, Italy. Electronic address: cosimo.nardi@unifi.it.
[Ti] Title:Role of cone-beam computed tomography with a large field of view in Goldenhar syndrome.
[So] Source:Am J Orthod Dentofacial Orthop;153(2):269-277, 2018 Feb.
[Is] ISSN:1097-6752
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:INTRODUCTION: Goldenhar syndrome is a rare disease with hemifacial microsomia and craniofacial disorders originating from the first and second branchial arches, such as ocular, auricular, and vertebral anomalies. The complexity and variety of the ways in which the disease presents itself usually need several examinations. In this study, we aimed to evaluate both craniofacial and vertebral skeletal anomalies and asymmetries between the nonaffected and affected sides in patients with Goldenhar syndrome by using cone-beam computed tomography. METHODS: Ten patients (7-14 years old; 6 boys, 4 girls) were evaluated via NewTom 5G cone-beam computed tomography (QR srl, Verona, Italy) with a large field of view (18 × 16 cm). Ten anatomic facial landmarks were identified to measure the following distances bilaterally: sella turcica (ST)-mandibular angle, ST-condyle, ST-mastoid, ST-mental foramen, ST-fronto zygomatic suture, ST-zygomatic temporal suture, ST-zygomatic facial foramen, ST-sphenopalatine fossa, mandibular angle-mandibular symphysis, and mandibular angle-condyle. The following 6 volumes were calculated bilaterally: orbit, maxillary sinus, condyle, external ear canal, middle ear, and internal auditory canal. These measurements were performed to assess skeletal asymmetries to compare the nonaffected side with the affected side by the Wilcoxon test. Cervical spine anomalies were classified into fusion anomalies and posterior arch deficiencies. RESULTS: All patients showed a deficit of skeletal development on the affected side. Statistically significant differences (0.001 ≤ P value ≤ 0.043) between the nonaffected and affected sides were recorded for all measurements, except for ST-frontozygomatic suture, mandibular angle-mandibular symphysis, and maxillary sinus volume. Vertebral fusion anomalies and posterior arch deficiencies were found in 7 and 4 patients, respectively. CONCLUSIONS: Cone-beam computed tomography with a large field of view was able to accurately identify craniofacial and vertebral skeletal anomalies, and to quantify asymmetries between the nonaffected and affected sides for an efficient maxillofacial treatment planning.
[Mh] MeSH terms primary: Cone-Beam Computed Tomography
Goldenhar Syndrome/diagnostic imaging
[Mh] MeSH terms secundary: Adolescent
Child
Cone-Beam Computed Tomography/methods
Craniofacial Abnormalities/diagnostic imaging
Female
Foramen Magnum/diagnostic imaging
Humans
Male
Mandible/diagnostic imaging
Mandibular Condyle/diagnostic imaging
Mastoid/diagnostic imaging
Sella Turcica/diagnostic imaging
Spine/diagnostic imaging
Zygoma/diagnostic imaging
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180213
[Lr] Last revision date:180213
[Js] Journal subset:D; IM
[Da] Date of entry for processing:180207
[St] Status:MEDLINE

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[PMID]: 29178655
[Au] Autor:Laughlin RS; Niu Z; Wieben E; Milone M
[Ad] Address:Department of Neurology, Mayo Clinic, Rochester, Minnesota.
[Ti] Title:RYR1 causing distal myopathy.
[So] Source:Mol Genet Genomic Med;5(6):800-804, 2017 11.
[Is] ISSN:2324-9269
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: Congenital myopathies due to ryanodine receptor (RYR1) mutations are increasingly identified and correlate with a wide range of phenotypes, most commonly that of malignant hyperthermia susceptibility and central cores on muscle biopsy with rare reports of distal muscle weakness, but in the setting of early onset global weakness. METHODS: We report a case of a patient presenting with childhood onset hand stiffness and adult onset progressive hand weakness and jaw contractures discovered to have two variants in the RYR1 gene. RESULTS: The patient manifested with distal upper limb weakness which progressed to involve the distal lower limb, proximal upper limb, as well as the face in addition to limited jaw opening. Creatine kinase was mildly elevated with EMG findings supporting a myopathy. Muscle biopsy showed features consistent with centronuclear myopathy. Whole exome sequencing revealed a novel heterozygous pathogenic variant in RYR1 (c.12315_12328delAGAAATCCAGTTCC, p.Glu4106Alafs*8), and a heterozygous missense variant (c.10648C>T, p.Arg3550Trp) of unknown significance in compound heterozygous state. CONCLUSION: We expand the spectrum of RYR1-related myopathy with the description of a novel phenotype in an adult patient presenting with hand weakness and suggest considering RYR1 analysis in the diagnosis of distal myopathies.
[Mh] MeSH terms primary: Distal Myopathies/genetics
Ryanodine Receptor Calcium Release Channel/genetics
[Mh] MeSH terms secundary: Adult
Creatine Kinase/metabolism
DNA Mutational Analysis
Distal Myopathies/diagnosis
Electromyography
Heterozygote
Humans
Jaw Abnormalities/physiopathology
Male
Muscle, Skeletal/pathology
Pedigree
Phenotype
Polymorphism, Single Nucleotide
Upper Extremity/physiopathology
Whole Exome Sequencing
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Name of substance:0 (Ryanodine Receptor Calcium Release Channel); EC 2.7.3.2 (Creatine Kinase)
[Em] Entry month:1801
[Cu] Class update date: 180207
[Lr] Last revision date:180207
[Js] Journal subset:IM
[Da] Date of entry for processing:171128
[St] Status:MEDLINE
[do] DOI:10.1002/mgg3.338

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[PMID]: 28468208
[Au] Autor:Sisti A; Freda N; Giacomina A; Gatti GL
[Ad] Address:Plastic and Reconstructive Surgery, Santa Chiara Hospital, Pisa, Italy.
[Ti] Title:Popliteal Pterygium Syndrome With Syngnathia.
[So] Source:J Craniofac Surg;28(3):e250-e251, 2017 May.
[Is] ISSN:1536-3732
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Popliteal pterygium syndrome is a condition characterized by skin webs on the popliteal fossa, which may impair mobility unless surgically repaired. Affected individuals may also have syndactyly on the fingers and/or toes. Most people with this disorder present cleft lip and cleft palate and they can have syngnathia, that is a congenital adhesion between maxilla and mandible by fibrous bands, which affects the opening of the mouth. The case that we report is about a 2-month-old male, who presented skin webs bilaterally on the popliteal fossa, syndactyly between the IV and the V toe of the right foot and between the III and the IV toe of the left foot, and genital malformations. He was born with complete bilateral cleft lip and complete cleft palate on the left side and incomplete cleft palate on the right side and syngnathia with 4 fibrous bands between the mandibular arch and the maxilla arch on the right side, which affected the opening of the mouth. The case of our patient is very interesting because there have been few reported patients affected by popliteal pterygium syndrome with syngnathia.
[Mh] MeSH terms primary: Abnormalities, Multiple/diagnosis
Cleft Lip/diagnosis
Cleft Palate/diagnosis
Eye Abnormalities/diagnosis
Fingers/abnormalities
Knee Joint/abnormalities
Lower Extremity Deformities, Congenital/diagnosis
Mandible/abnormalities
Maxilla/abnormalities
Syndactyly/diagnosis
Urogenital Abnormalities/diagnosis
[Mh] MeSH terms secundary: Humans
Infant
Male
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180202
[Lr] Last revision date:180202
[Js] Journal subset:D
[Da] Date of entry for processing:170505
[St] Status:MEDLINE
[do] DOI:10.1097/SCS.0000000000003473

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[PMID]: 29336428
[Au] Autor:Manohar B; Baidya D; Bhuvaneswari S; Rai AB
[Ad] Address:Department of Periodontics, Pacific Dental College and Hospital, Debari. Udaipur, Rajasthan, Pin. - 313024.
[Ti] Title:Keratocystic Odontogenic Tumour Mimicking Lateral Periodontal Cyst: A Diagnostic Dilemma.
[So] Source:Kathmandu Univ Med J (KUMJ);14(56):370-372, 2016 Oct.-Dec..
[Is] ISSN:1812-2078
[Cp] Country of publication:Nepal
[La] Language:eng
[Ab] Abstract:The Keratocystic Odontogenic Tumor is a developmental cyst derived from the enamel organ or from the dental lamina. It is a benign, multicystic, intraosseous tumor of odontogenic origin, with a characteristic lining of parakeratinized stratified squamous epithelium and has a potential for aggressive, infiltrative behavior and recurrence. Keratocystic Odontogenic Tumors have a predilection for males and occurs mainly in the second and third decade of life, most commonly in the mandible, mostly in the posterior body, the angle and the ascending ramus. It extends in the intramedullary space making it difficult to diagnose at an early stage. It is regarded as a distinctive entity because of its characteristic histology, proliferation kinetics and behavior. Main in 1970 described, collateral variant of Keratocystic Odontogenic Tumor, which presents adjacent to the roots of the teeth usually in the mandibular premolar region and radiologically is indistinguishable from the lateral periodontal cyst and gingival cyst.
[Mh] MeSH terms primary: Jaw Abnormalities/diagnosis
Jaw Abnormalities/pathology
Odontogenic Cysts/diagnosis
Odontogenic Cysts/pathology
[Mh] MeSH terms secundary: Aged
Diagnosis, Differential
Humans
Male
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180129
[Lr] Last revision date:180129
[Js] Journal subset:IM
[Da] Date of entry for processing:180117
[St] Status:MEDLINE

  10 / 4131 MEDLINE  
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[PMID]: 28468148
[Au] Autor:Ligh CA; Swanson J; Yu JW; Samra F; Bartlett SP; Taylor JA
[Ad] Address:*Division of Plastic and Reconstructive Surgery, Department of Surgery, The Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA †Division of Plastic and Reconstructive Surgery, Department of Surgery, University of Southern California, Los Angeles, CA.
[Ti] Title:A Morphological Classification Scheme for the Mandibular Hypoplasia in Treacher Collins Syndrome.
[So] Source:J Craniofac Surg;28(3):683-687, 2017 May.
[Is] ISSN:1536-3732
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: Mandibular hypoplasia is a hallmark of Treacher Collins syndrome (TCS), and its severity accounts for significant functional morbidity. The purpose of this study is to develop a mandibular classification scheme. METHODS: A classification scheme was designed based on three-dimensional computed tomography (3D-CT) scans to assess 3 characteristic features: degree of condylar hypoplasia, mandibular plane angle (condylion-gonion-menton), and degree of retrognathia (sella-nasion-B point angle). Each category was graded from I to IV and a composite mandible classification was determined by the median value among the 3 component grades. RESULTS: Twenty patients with TCS, aged 1 month to 20 years, with at least one 3D-CT prior to mandibular surgery were studied. Overall, 33 3D-CTs were evaluated and ordered from least to most severe phenotype with 10 (30%) Grade 1 (least severe), 14 (42%) Grade 2, 7 (21%) Grade 3, and 2 (7%) Grade 4 (most severe). Seven patients had at least 2 longitudinal scans encompassing an average 5.7 (range 5-11) years of growth. Despite increasing age, mandibular classification (both components and composite) remained stable in those patients over time (P = 0.2182). CONCLUSION: The authors present a classification scheme for the TCS mandible based on degree of condylar hypoplasia, mandibular plane angle (Co-Go-Me angle), and retrognathia (SNB angle). While there is a natural progression of the mandibular morphology with age, patients followed longitudinally demonstrate consistency in their classification. Further work is needed to determine the classification scheme's validity, generalizability, and overall utility.
[Mh] MeSH terms primary: Malocclusion/surgery
Mandibulofacial Dysostosis/classification
Mandibulofacial Dysostosis/surgery
[Mh] MeSH terms secundary: Adolescent
Cephalometry/methods
Child
Child, Preschool
Female
Humans
Imaging, Three-Dimensional
Infant
Male
Malocclusion/classification
Malocclusion/diagnosis
Mandible/abnormalities
Mandibulofacial Dysostosis/diagnosis
Retrognathia/classification
Retrognathia/diagnosis
Retrognathia/surgery
Retrospective Studies
Tomography, X-Ray Computed/methods
Tooth Abnormalities/classification
Tooth Abnormalities/diagnosis
Tooth Abnormalities/surgery
Young Adult
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180126
[Lr] Last revision date:180126
[Js] Journal subset:D
[Da] Date of entry for processing:170505
[St] Status:MEDLINE
[do] DOI:10.1097/SCS.0000000000003470


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