bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pure Ectodermal Dysplasia: Retrospective Study of 16 Cases and Literature Review
Authors: Ruhin B. , Martinot V. , Lafforgue P. , Catteau B. , Manouvrier-Hanu S. , Ferri J. 
Published: October 2018

Objective: To review the possible craniomaxillofacial deformative consequences associated with ectodermal dysplasias and embryonic malformations, which include dental ageneses. Setting: Oral and Maxillofacial Surgery Department, University Hospital, Lille, France. Patients: Sixteen patients (seven boys and nine girls, aged 4 to 34 years) with pure ectodermal dysplasia (no ectodermal dysplasia syndromes). Interventions: All patients had a clinical examination. Seven (two boys and five girls, aged 4 to 25 years) had undergone plaster casts and radiographic and Delaire's cephalometric studies before being treated. Main Outcome Measures: All patients had tooth ageneses (from hypodontia to anodontia), associated with cutaneous dyshidrosis and hair and nail dystrophy. Most of them had a short face, with an unusual facial concavity, a maxillary retrusion, and a relative mandibular protrusion. Management Results and Discussion: Depending on their ages and their orthopedic abnormalities, patients underwent either dental or prosthodontic, orthodontic, orthopedic, orthognathic, or implant treatment. So as not to interfere with the growth pattern, we preferred to reserve implant and orthognathic surgery for full-grown cases. Conclusions: Oral and maxillofacial surgeons must undertake a comprehensive approach to these patients to improve their dental, masticatory, growing, and orthognathic conditions.
The Tessier Number 5 Cleft with Associated Extremity Anomalies
Authors: Gökalan Kara İnci , Öçsel Hakan 
Published: October 2018

Objective: Oblique facial clefts are rare congenital deformities. They constitute 0.22% of all facial malformations. In this case report, an oblique facial cleft defined as Tessier number 5 is presented. The number 5 cleft of Tessier is one of the most rare congenital facial clefts; review of the literature revealed 20 patients. This case was also demonstrated with associated limb anomalies, suggesting that the patient may have been affected by the amnion rupture sequence. The teratology of these malformations is discussed, and attention is drawn to the amniotic rupture sequence as a possible cause.
The Faith of a Coronal Suture Grafted onto Midline Synostosis Inducing Dura and Deprived from Tensile Stress
Authors: Mommaerts Maurice Y. , Staels Patrick F. J. , Casselman Jan W. 
Published: October 2018

Objective: To discuss possible reasons for the synostosis of a coronal suture that was transplanted onto synostosis inducing dura in a scaphocephalic human cranium. Design: Case report. Setting: Supraregional teaching hospital, center for craniofacial anomalies. Patient: A bathmocephalic boy, followed from age 7½ to 26 months. Intervention: Radical synostosectomy, radial osteotomies in the parietal bone with outward fracturing of the barrel staves, and left-sided coronal suture transplantation onto the midline was undertaken at the age of 11 months. Methods: Computer tomography and clinical follow-up. Results: The sutural graft, initially deprived from tensile stress and quickly exposed to the anomalous dura, turned synostotic in one year. Conclusions: Both cell signaling and biomechanical theories on calvarial morphogenesis, sutural development, and synostosis can apply. An animal experiment is recommended to test which hypothesis prevails.
Vestibuloplasty after Secondary Alveolar Bone Grafting
Authors: Iino Mitsuyoshi , Fukuda Masayuki , Murakami Kaho , Horiuchi Toshikatsu , Niitsu Kouta , Seto Kanichi 
Published: October 2018

Objective: This paper introduces a surgical technique for vestibuloplasty after secondary alveolar bone grafting of patients with cleft lip and palate (CLP). This paper also reports on the patients who underwent this modified vestibuloplasty. Surgical Procedure: The vestibuloplasty technique described in this paper consists of: (1) reduction of submucosal scar tissue of the upper lip, (2) V-Y plasty of the superficial mucosa, (3) placement of horizontal mattress sutures between nostril floor skin and freed marginal mucosa, (4) application of artificial skin to cover the exposed periosteal surface, and (5) use of a removable retention splint. Conclusion: This surgical procedure appears to be very useful for patients with CLP. The technique enables the surgeon to obtain an adequate sulcus depth around the graft area. In addition, this technique releases the mucosal scar contraction and improves the shape and mobility of the upper lip.
Children of the Philippines: Attitudes toward Visible Physical Impairment
Authors: Harper Dennis C. , Peterson David B. 
Published: October 2018

Objective: This pilot study was designed to evaluate children's attitudes and understanding of physical disabilities with special reference to those with craniofacial anomalies in the Philippines. Children with and without craniofacial anomalies were studied. Design: This was a two-group correlational design with additional statistical assessment of subgroup differences. Each group was interviewed and information obtained on a standard disability preference task, attributions for playmate choice, and frequency of contact with disabilities. Parents completed a structured interview. Setting: Participants were 122 children recruited from Negros, Philippines. Participants: Fifty-four children with craniofacial anomalies (aged 7 to 12 years) were enrolled in the study, and 68 children without any disabilities were recruited from a local school in Bacolod City, Negros, Philippines. Main Outcome Measures: Participants completed a picture-ranking interview of specific physical disabilities and provided their reasons for their play choices and their contact with physical disabilities. Results: The Kendall W correlation was significant for the children with craniofacial anomalies and for those without physical disabilities. Both groups reported lower preferences for disabilities that interfere with play and social interactions. Children depicted with facial anomalies received lower preference, compared with other physical disabilities. Children with craniofacial anomalies who have experienced surgical repair reported more positive rankings for the child depicted with a facial cleft. Sex differences in disability preference were noted. Conclusions: Children in the Philippines with and without craniofacial differences revealed similarities in preferences to children in several Western (United States) and non-Western countries. Children depicted with facial anomalies received lower preference than other visible physical differences. Children reported both positive and negative explanations for their disability play preferences. Facial differences may result in illogical and negative explanations for social avoidance among children. Similar reactions are noted in other parts of the world.
Facial Anthropometry in Cleft Patients: A Historical Appraisal
Authors: Vegter Florine , Hage J. Joris 
Published: October 2018

Objective: To present a historical appraisal of the use of anthropological and cephalometrical facial soft tissue measurements in cleft patients. Design: The McDowell Indexes and a Medline search were used to trace references up to 1999. Also, references listed with chapters and articles on facial clefts were searched for anthropometrical studies. Twenty-six retrieved articles and book chapters on soft tissue anthropometry and 12 cephalometric publications on soft tissue measurements on radiographs and plaster casts of cleft patients were reviewed. Results and Conclusions: Since 1931, the facial soft tissue appearance of cleft patients has been evaluated by means of anthropometric and cephalometric techniques. Not all of the older studies were performed in a statistically correct fashion. Many of the conclusions of the studies overlap despite differences in technique of assessment. Most studies demonstrate the deficient growth of the maxilla and the deformities of the facial profile in cleft patients.
Cranial Base Growth and Morphology in Second-Trimester Normal Human Fetuses and Fetuses with Cleft Lip
Authors: Sherwood Todd F. , Mooney Mark P. , Sciote James J. , Smith Timothy D. , Cooper Gregory M. , Siegel Michael I. 
Published: October 2018

Objective: The present radiographic study describes the size and shape of the cranial base from the sagittal aspect for a sample of 77 second-trimester “normal” control fetuses (n = 61) and fetuses (n = 16) exhibiting isolated, unilateral clefts of the lip (CL), ranging in fertilization age from 10 to 22 weeks. Methods: Fetuses were placed in a cephalostat, and standardized, lateral head radiographs were taken. The radiographs were traced, and 15 cephalometric landmarks were identified and digitized for analysis. Growth curves for cranial base lengths, angles, and areas were compared between control and CL groups. Also, cranial base triangles were constructed and shape comparisons were made using tensor biometric analysis. Results: No significant differences (p > .05) in regression line slopes were noted for any comparisons between the control and CL samples. Tensor biometric analysis also revealed no significant differences in the shapes of various cranial base triangles between the control and CL samples. Conclusion: This report presents second-trimester baseline growth curves for various cranial base components in CL human fetal specimens, and these data suggest that CL fetuses may also be used as an appropriate control sample for prenatal growth comparison studies of cleft lip and palate and cleft palate.
Endocranial Vascular Patterns in a Familial Rabbit Model of Coronal Suture Synostosis
Authors: Burrows Annie M. , O'Loughlin Valerie D. , Mooney Mark P. , Smith Timothy D. , Wolfgang Losken H. , Siegel Michael I. 
Published: October 2018

Objective: The present study investigates the potential relationship between craniosynostosis and any changes in endocranial vasculature. The hypothesis that crania from rabbits with familial, nonsyndromic coronal suture synostosis and crania from rabbits with experimental immobilization of the coronal suture are associated with altered form of the middle meningeal vessels and dural venous sinuses is tested. Design: Silicone rubber endocasts from 14 adult New Zealand white rabbits (Oryctolagus cuniculus) with familial nonsyndromic coronal suture synostosis (five with bilateral coronal suture synostosis and nine with unilateral coronal suture synostosis) were made to assess middle meningeal vessel and dural venous sinus form. For comparative purposes, endocasts were made from 25 rabbits with normal, patent coronal sutures and 10 rabbits with experimental immobilization of the coronal suture. Impressions of the dural venous sinuses were assessed for depth and width. The area of the confluens of sinuses was also assessed. Impressions of the middle meningeal vessels were assessed for depth, width, and degree of convolution. For width of the dural venous sinuses and area of the confluens of sinuses, comparisons among groups were made with a one-way analysis of variance (ANOVA). For depth of the dural venous sinuses and impressions of the middle meningeal vessels, comparisons among groups were made using a Kruskal-Wallis one-way ANOVA. Results: Crania with familial coronal suture synostosis had significantly (p < .05) reduced posterior dural venous sinus dimensions when compared with both crania from rabbits with experimental immobilization of the coronal suture and rabbits with normal coronal sutures. Crania with both coronal suture synostosis and experimental immobilization had significant increases in dimensions of the middle meningeal vessels relative to normal crania. In addition, casts from rabbits with unicoronal suture synostosis showed marked asymmetry in the dural venous sinuses. Conclusions: These results support the hypothesis that craniosynostosis is associated with alterations in endocranial vasculature. These changes are most likely a secondary response to synostosis rather than a causal factor and may reflect increased intracranial pressure, decreased intracranial volume, and local accumulations and reductions of cerebrospinal fluid in the posterior region of the skull and immediately deep to the coronal suture.
The Dental Health and Caries-Related Microflora in Children with Craniosynostosis
Authors: Mustafa Duaa , Lucas Victoria S. , Junod Pablo , Evans R. , Mason Carol , Roberts Graham J. 
Published: October 2018

Objective: To compare levels of dental caries, bacterial dental plaque, gingivitis, enamel defects, and caries-related microflora in children with and without craniosynostosis. Study group: Fifty-seven children with craniosynostosis and their matched controls. Outcome measures: The decayed, missing, and filled teeth and surfaces in both the deciduous (dmfs and dmft) and the permanent dentition (DMFS and DMFT). The plaque and gingivitis scores and developmental enamel defects were also recorded. The caries-related microflora was sampled using an alginate swab and the prevalence of Streptococcus mutans and Lactobacillus and Candida species were recorded. Results: The dmfs (p < .02) and dmft (p < .01) were significantly greater in the control children. The plaque score for the deciduous dentition only (p < .02) and also the gingivitis score for the permanent teeth only (p < .008) in the craniosynostosis group were significantly greater. The total aerobic bacterial count (p < .004), anaerobic count (p < .002), and Candida count (p < .05) were significantly greater in the control group. The proportion of S. mutans both as a percentage of the total anaerobic count (p < .04) and the total streptococcal count (p < .05) was significantly greater in the control group.
Displacement of the Mandible in a Child with Congenital Unilateral Temporomandibular Joint Ankylosis Treated with Two-Stage Condylar Replacement: A Long-Term Study with the Aid of Roentgen Stereometric Analysis
Authors: Wittbjer Jan , Sarnäs Karl-Victor , Rune Bodil 
Published: October 2018

Objective: To monitor mandibular displacement in three dimensions with a high degree of accuracy before, during, and after treatment. Design: Prospective roentgen stereometric analysis from age 3 years 7 months to age 13 years 8 months. Setting: Center for Craniofacial Anomalies and Department of Maxillofacial Surgery, Malmö University Hospital, Malmö, Sweden. Patient: Girl with unilateral congenital temporomandibular joint (TMJ) ankylosis and marked facial asymmetry. Interventions: Epipharyngeal inspection at age 2 years 6 months and insertion of implants under general anesthesia. Resection of the affected right condyle at age 5 years 4 months, replacement of the ramus-condyle complex with a custom made titanium prosthesis at age 5 years 5 months, and replacement of the artificial condyle by a costochondral graft at age 9 years 7 months. Roentgen examinations were performed at intervals from age 3 years 7 months to age 13 years 8 months. Body length was measured annually. Main Outcome Measures: Stereo roentgenograms were digitized by the Department of Orthopedic Surgery, Malmö University Hospital. Results: Unexpected lowering of the mandible occurred before treatment. Each procedure had a distinct effect on the direction of mandibular displacement. There was little or no pathological involvement of the functional matrix. Growth of the costochondral graft exceeded growth of the unaffected TMJ. Anterior displacement as well as rotation and tilting of the mandible to the unaffected side accelerated after the age of body peak height velocity. Conclusions: Accurate monitoring of mandibular displacement may contribute to a rationale for clinicians’ decision on treatment protocol and reveal compensatory differential growth.
Three-Dimensional Analysis of Morphological Changes in the Maxilla of Patients with Cleft Lip and Palate
Authors: Braumann Bert , Keilig Ludger , Bourauel Christoph , Jäger Andreas 
Published: October 2018

Objective Three-dimensional (3-D) morphological changes in the maxilla of patients with cleft lip and palate (CLP) have been recorded, mainly using two-dimensional cast analyses. Although these seem to be insufficient, no standardized 3-D method has been developed until now. In this study, accuracy, precision, and validity of a newly developed 3-D digital computer-aided procedure to visualize and metrically analyze the growth of the edentulous maxilla of infants with CLP have been evaluated. Patients The method was applied to 10 infants with complete unilateral CLP. Interventions Consecutive casts of the maxilla (1 week and 3, 6, and 12 months) of each patient were optically measured with a 3-D laser scanner. Following digitizing, the casts were computer reconstructed, aligned, and superimposed using specialized computer software. The distances between the surfaces were measured. Additionally, the surfaces were segmented perpendicular to the alveolar crest, the reference points being C1, C1′, C2, C2′, and I. The volumes of the resulting segments were determined and compared with one another. Results The newly developed analysis enables a visualization of the extent and direction of morphological changes in the maxilla of infants with CLP. With this method it is possible to quantify these changes of the volume of defined alveolar segments. Conclusions The 3-D analysis developed is an ideal tool for the examination of 3-D morphological changes in the edentulous maxilla of patients with CLP. The results will serve as the starting point for a longitudinal study on the efficacy of different methods, not only of presurgical infant orthopedics but also of surgical procedures.
Alveolar Bone Grafting in Patients with Complete Clefts: A Comparative Study between Secondary and Tertiary Bone Grafting
Authors: Dempf Rupert , Teltzrow Thomas , Kramer Franz-Josef , Hausamen Jarg-Erich 
Published: October 2018

Objective This investigation evaluated the results of alveolar bone grafting in patients with complete clefts, comparing secondary alveolar bone grafting (during the mixed stage of dentition) versus tertiary bone grafting (after completion of the second stage of dentition). Design This was a retrospective study. Of 140 osteoplasties, which all were operated according to the same standardized surgical technique, a clinical and roentgenological follow-up investigation was carried out in a collective of 85 osteoplasties. Clinically we searched for oronasal fistulae, assessed the periodontal status, determined the extent of the gingival attachment in the area of the osteoplasty, and searched for vertical growth disturbances in the area of the osteoplasty. Roentgenologically the height of the alveolar bone in the former clefted area was ascertained. Setting Records were obtained from a clinical and radiological study of the Department of Oral and Maxillofacial Surgery of the Medical University of Hannover (Germany). Interventions All patients were operated with the same standardized surgical method. The osteoplastic bridging of the alveolar cleft was performed via a vestibular gingival marginal incision and exclusively by grafting of cancellous bone from the iliac crest. Results The best results of alveolar bone grafting in cases of secondary osteoplasty were obtained when the lateral incisor or canine had grown into the transplant and had led to a functional stress of the transplanted bone. Approximately good results were to be found in tertiary osteoplasty when the transplanted bone had been stressed functionally through a dental implant. Comparing the secondary with the tertiary osteoplasty, there was a trend of lower resorption in secondary osteoplasty. Conclusions Secondary osteoplasty should represent an integral component of any concept for the comprehensive treatment of patients with cleft lip and palate.
Ultrasound Diagnosis of Craniosynostosis
Authors: Miller Carolyn , Losken H. Wolfgang , Towbin Richard , Bowen A'delbert , Mooney Mark P. , Towbin Alex , Faix Richard S. 
Published: October 2018

Objective To retrospectively study prenatal ultrasound images of patients with craniosynostosis to determine the extent to which prenatal diagnosis is possible. Method Prenatal ultrasound images of 19 patients with postnatally diagnosed metopic or coronal suture craniosynostosis were retrospectively reviewed. The 26 ultrasound examinations obtained were compared with normal images and tables of gestation. Results It was not possible to diagnose craniosynostosis in the first trimester. In the second trimester, Kleeblattschädel was diagnosed at 20.5 weeks. A multilobular shape to the skull and diastasis of the frontotemporal suture was identified. In a second child with Kleeblattschädel, the cephalic index was above normal 86.4 (normal range 70 to 86), and the head circumference to abdominal circumference was increased. In the third trimester, the head shape deformation was more obvious. Brachycephaly diagnosis was made in the second trimester. In Crouzon syndrome the hypertelorism was identified at 19.9 weeks. Plagiocephaly was diagnosed at 21.4 weeks. In trigonocephaly the reduced cephalic index was noted at 18.8 weeks. In the third trimester, the deformity was diagnosed in all cases. Conclusion No diagnosis of craniosynostosis was made in the first trimester. In the second trimester, it was possible to diagnose Kleeblattschädel, tri-gonocephaly, brachycephaly (bilateral coronal suture craniosynostosis), and plagiocephaly (unilateral coronal suture craniosynostosis) in nine of the examinations. In the third trimester and at term, it was possible to diagnose previously listed conditions from six of the seven examinations obtained. Klee-blattschädel was suspected during original examination. A total of 15 examinations of 26 were correctly diagnosed during this investigation.
Validity of the HfmTransgenic Mouse as a Model for Hemifacial Microsomia
Authors: Cousley Richard , Naora Hiroyuki , Yokoyama Minesuke , Kimura Minoru , Otani Hiroki 
Published: October 2018

Objective Our comprehension of hemifacial microsomia (HFM) has been hindered by its diverse phenotype and unclear etiopathogenesis. The conventional view has been that HFM's facial defects result from embryonic hemorrhages in the region of the first and second branchial arches. A more recent model based on a transgenic mutation of a locus termed Hfm (B1 to B3 on chromosome 10) appears to provide an insight into HFM causation. This study investigated the validity of this model by examining the Hfm craniofacial phenotype and histological development of the embryonic head (E13.5 to 17.5). Results The results confirmed that although the loss-of-function mutation was transmitted in an autosomal dominant manner, the penetrance rate was significantly reduced and only Hfm heterozygotes were viable. The observations here extend the Hfm phenotype beyond microtia and jaw asymmetry to include structural and positional anomalies affecting the external auditory meatus, middle ear, cranial base, maxilla, and pharyngeal structures. Temporo-mandibular joint (TMJ) development and palatal shelf fusion were also affected in a small number of cases. In addition, some Hfmembryos displayed a novel finding: transposition of the developing inner ear between the tubotympanic recess and cranial base. Conclusions These craniofacial features, especially the ear anomalies and facial asymmetry indicate that the Hfm transgenic mouse represents a useful model for the HFM-microtia spectrum. In particular, it supports the hypothesis that at least a proportion of HFM anomalies has a genetic causation mediated via mesenchymal disruptions and possibly embryonic hemorrhages.