Severe Agnathia-Otocephaly Complex: Surgical Management and Longitudinal Follow-up From Birth Through Adulthood.

Agnathia-otocephaly complex (AOC) is characterized by mandibular hypo- or aplasia, ear abnormalities, microstomia, and microglossia. Though rare and often fatal, this is the first report detailing various reconstructive strategies beyond infancy as well as longitudinal follow-up into adulthood. All patients with AOC treated at our institution over a 30 year period were reviewed. Four patients were identified, one with agnathia, one with micrognathia. Two males with nanognathia (defined as a symphyseal remnant without body nor ramus) were also included. The mean follow-up was 17 years. All four underwent perinatal tracheostomy and gastrostomy-tube placement. Commissuroplasties were typically performed before 3 years of age and repeated as necessary to allow for oral hygiene. Mandibular reconstruction was most successful with rib between ages 3 and 8, after which time, free fibula transfer was utilized. Due to some resoprtion or extrusion, all patients underwent repeated bone grafting procedures. Tissue expansion of the neck was used to restore the lower third of the face, but was most successful in the teenage years. At last follow-up of the eldest patients, one was in college while another was pursuing graduate education. AOC need not be a fatal nor untreatable condition; a reasonable quality of life can be achieved. Although the lower-facial contour may be improved, and a stoma created, the lack of musculature make deglutition virtually impossible with current therapies. Just as transplantation has emerged as a modality for facial restoration following severe trauma, so too may it be a future option for congenital deformities.

Effects of orthognathic surgery on psychological status of patients with jaw deformities.

The purpose of this study was to determine the effect of orthognathic surgery on psychological status. The subjects were 119 patients (38 males and 81 females, mean age 25.5±9.4 years) who underwent orthognathic surgery. They were divided into class III (84 patients), class II (20 patients), and class I (15 patients) groups according to the anteroposterior skeletal pattern, and they were also divided into an asymmetry group (51 patients) and a symmetry group (68 patients). We assessed psychological status using the Minnesota Multiphasic Personality Inventory (MMPI) before surgery and at more than 6 months after surgery. The MMPI scores for the depression, hysteria, psychasthenia, and social introversion scales were significantly higher than standard values before surgery, and the hypomania scale significantly lower. The cannot say scale, depression scale, and hysteria scale decreased significantly after surgery. A comparison of MMPI scores among the groups showed the depression scale in the class III group to be higher than those in the class I and II groups; there was no significant difference between the asymmetry and symmetry groups. In conclusion, orthognathic surgery has a positive influence on the psychological status of patients with jaw deformities, especially patients with skeletal class III malocclusion.

The clinical relevance of orthognathic surgery on quality of life.

The aim of orthognathic surgery is to produce a more aesthetic facial skeletal appearance, and improve jaw function. This prospective study, aimed to evaluate the impact of orthognathic surgery on quality of life for patients with dentofacial deformity, and whether it was clinically meaningful. 62 consecutive patients were recruited (27 male, 35 female) aged 18-38 years. Baseline data were collected using a validated health status measure (Orthognathic Quality of Life Questionnaire (OQLQ)) and a visual analogue scale (VAS). Postoperative questionnaires (OQLQ, VAS) and a Global Transition Scale (GTS) were completed at 6 months after completion of treatment and compared with pre-treatment scores. Following surgery, there was a significant (p<0.05, paired t test) improvement in OQLQ scores for each domain. The proportion of patients reporting a moderate or large improvement was: facial appearance (93%), chewing function (64%), comfort (60%) and speech (32%). Clinical relevance of change scores was reported in terms of effect sizes, and the largest effect was on facial aesthetics. The clinical impact was moderate on social aspects of deformity and oral function and a small effect on awareness of facial deformity. This research reaffirms that orthognathic surgery has positive effects on quality of life.

Qualidade de vida em portadores de disfunção temporomandibular: um estudo transversal / Health-related quality of life in patients with temporomandibular: a cross sectional study

Introdução - A hiperatividade muscular pode desencadear dor e/ou cansaço muscular (fadiga) e estar associado à portadores de disfunção temporomandibular. O objetivo deste estudo foi classificar os portadores de disfunção temporomandibular (DTM) e correlacionar com o impacto na qualidade de vida (QV). Material e Métodos - Foi realizado um estudo transversal em uma amostragem de 302 participantes, com faixa etária entre 17 a 50 anos, que foram divididos em dois grupos (80 pacientes do sexo masculino e 80 do sexo feminino) e randomizados através de programa de geração de números aleatórios. O diagnóstico e classificação da DTM, em ambos os grupos, foi obtido pelo índice anamnético de Fonseca e a qualidade de vida através dos questionários da versão brasileira do SF36. Resultados - Os resultados mostraram que 38.75% dos participantes não apresentaram DTM sendo que a grande parte dos participantes analisados possuíram disfunção leve (41.87%) e moderada (14.37%) com prejuízo principalmente das características mentais como, Vitalidade e Aspectos Emocionais, sendo que a Capacidade Funcional foi o domínio que se demonstrou mais preservado dentro da amostra. Conclusão - Existe uma influência direta do grau de DTM com a qualidade de vida dos participantes sintomáticos.

Introduction - The muscle hyperactivity can be associated to temporomandibular disorders(TMDs) and cause pain and/or muscle fatigue. The aim of this study was to classify TMD patients and provide a correlation with quality of life (QL) impact. Material and Methods - A cross-section study was conducted on a 302-participant sample with age level from 17 to 50 years old. The participants were randomly divided into two groups (80 male subjects and 80 female subjects). TMD diagnosis and classification were obtained for both groups by means of the Fonseca anamnesic index while the quality of life was obtained by means of the Brazilian version of the SF-36. Results -The data collected demonstrated that 38.75% of the participants did not present TMD. Most of the participants assessed presented mild (41.87%) and moderate TMD (14.37%) with deficits observed mainly on the mental characteristics, such as Vitality and Emotional Aspects. Functional Capacity was the most preserved domain within the sample. Conclusion - There is a direct influence of the TMD level on the quality of life of symptomatic subjects.

The psychosocial impact of orthognathic surgery: a systematic review.

The aim of this study was to review the reported psychosocial benefits of orthognathic surgery. A systematic review of the literature was conducted using MEDLINE (1966 to December 2000), Web of Science (1981 to December 2000), and reference sections of identified articles. We also hand searched key orthodontic, oral surgery, and psychology journals. No language limitations were imposed. Randomized controlled trials, other controlled clinical trials, prospective studies (with or without controls), and retrospective studies (with or without controls) were considered for inclusion. Two reviewers extracted the data and independently assessed the quality of the studies. In all, 29 studies, including a number of prospective and retrospective studies, were identified as relevant. The results of the review indicated that orthognathic patients experience psychosocial benefits as a result of orthognathic surgery, including improved self-confidence, body and facial image, and social adjustment. However, there were wide variations in the study designs and a lack of uniformity in measuring the psychosocial constructs. This made it difficult to quantify the extent and the duration of the psychosocial benefits.

La adaptación psicosocial en niños y adolescentes con malformaciones cranofaciles / Psychosocial adjustment in children and adolescents with craneofacial malformations

El objetivo de este trabajo es determinar la percepción que los niños y los adolescentes con malformaciones craneofaciales tienen de su adaptación psicosocial y compararla con la de su padres. Determinar cuáles son los síndromes psicopatológicos más frecuentes en ellos, el funcionamiento de sus familias y tratar de correlacionar estos datos. El estudio se realizó en 19 menores con malformaciónrs cranofacisles y su padres. Contestaron los cuestionarios de Columbia, sobre el comportamiento del niño y de McMaster. Se pudo observar que el funcionamiento psicosocial de estos menores tiende a estar alterado en opinión de ellos y de sus padres. Hubo correlación entre la autovaloración del paciente, la psicopatología global y los síndromes de esternalización y de agresividad. Una correlación menor se observó con los síndromes de internalización, de somatización y de atención deficiente y con el funcionamiento de la familia. Se concluye que los menores con malformaciones craneofaciales enfrentan riesgos especiales para su adapatación psicosocial, derivados tanto de la enfermedad como de los factores familiares y ambientales

Orthognathic surgery: patient expectations; psychological profile and satisfaction with outcome.

61 orthognathic surgery patients were studied (39% male, 61% female). It confirms that the group of patients we see in the West of Scotland are well adjusted psychologically and that the majority seek orthognathic surgery for aesthetic reasons. The degree of satisfaction is high (87%). Dissatisfaction is not related to sex, age or procedure. Patients who were dissatisfied tend to have higher neuroticism scores on the Eysenck Personality Inventory and those patients who had had unreal expectations of post surgical pain, numbness and swelling, were likely to express dissatisfaction with the outcome in the early stages. Several patients initially expressed some dissatisfaction with the outcome after 3 months then went on to increase their score and by 12 months became satisfied with the procedure. The authors outline the importance of adequate patient preparation prior to surgery and the use of printed literature is recommended.

Psychological aspects of orthognathic surgery: a review of the literature.

A review of the literature clearly shows that dental and facial disfigurements have significant effects and can be an important social disadvantage. The motivational patterns of patients requesting orthognathic surgery are many and varied, but a desire for improvement in esthetics and alleviation of functional problems are the two most commonly cited reasons. The careful assessment of patients requesting orthognathic surgery is imperative, because the success of surgery may well depend on careful patient selection. Care must be taken with those patients suspected of exhibiting dysmorphophobic tendencies and, if there is any doubt, psychiatric referral should be undertaken. Patient satisfaction following orthognathic surgery has been reported as high overall, as has been the number of patients who have said they would re-elect to have surgery. Many of the studies found that patients had improved self-confidence and social skills after treatment.

Scope of oral and maxillofacial surgery: the psychosocial dimensions of orthognathic surgery.

A group of 20 patients (12 females and 8 males) who underwent orthognathic surgery during the previous three years were surveyed. The reasons which determined the decision to undergo surgery are discussed. Patient satisfaction and the effect of surgery on psychological and social attitudes are explored.

Psychological factors in orthognathic surgery.

27 patients subjected to various orthognathic surgery procedures were studied preoperatively and during a period of 18 months postoperatively by a team of psychologists to evaluate the patients' own opinions concerning the indication for surgery, information about the surgical procedure, the postoperative period and the long-term aesthetic and functional results. The patients were interviewed 5 times: 2 days before surgery, 2 days after surgery and 2, 6 and 18 months after surgery. The decision to undergo surgery was taken after a long period of time, 4 years on average, and half of the patients were influenced by their family or dentist before they made their decision. 60% gave three or more reasons for surgery, 85% mentioned functional problems, 74% facial appearance and 59% craniomandibular symptoms. 63% indicated that the facial appearance problems had negatively influenced their personal life and 44% their social life. Women experienced this problem more often than men. The results showed that the overall majority of patients were relieved of their presurgical problems. The aesthetic improvement was better than expected. It was found that improvements in facial features had a beneficial influence on the patients as individuals and also on their social life situation.

[The psychological rehabilitation of patients following reconstructive operations in congenital deformities of the jaw bones]. / Psikhologicheskaia reabilitatsiia bol'nykh posle rekonstruktivnykh operatsii pri vrozhdennykh deformatsiiakh cheliustnykh kostei.

Various methods of psychotherapeutic correction, used in patients with congenital deformations of the jaw bones, are described. Individual and group psychotherapy before, immediately and in late periods after surgery was used. Good results were achieved, confirmed by objective psychologic tests.

The use of psychometric tests in the evaluation of patients presenting for orthognathic surgery--a preliminary report.

Psychological evaluation in some orthognathic patients may be necessary as the psychosocial stress of the deformity may lead to psychiatric morbidity such as depression or paranoid disorders. It is also possible for patients with underlying psychosis to present first to the dental surgeon for jaw correction. The use of several psychological tests appropriate for patients presenting with dentofacial deformities is discussed. Nine patients with skeletal malocclusions who were referred for psychiatric assessment are reported here. Three case vignettes are included to give an impression of the type of clinical situations where such an assessment may be helpful.

The psychological and behavioral considerations of orthognathic surgery on identical (monozygotic) twins.

The psychologic and behavioral changes that may occur with the impact of orthognathic surgery on the physical appearance of an identical twin set have been addressed. This unique situation has not been discussed in the literature with respect to reconstructive or cosmetic facial surgery. A report on elective orthognathic surgery performed on identical twins and the subsequent impact on the twin relationship due to alterations of "self-image phenomenon" is given. Such an impact was a consideration in this case. Further collective study in this area is needed but is difficult to obtain because of the rarity of occurrence of this type of case.