Early Surgical Intervention of Congenital Syngnathia.

Congenital syngnathia is a rarely reported malformation when there is a fusion between the maxilla and the mandible. It is necessary to modify it in childhood because congenital syngnathia causes incongruity in pronunciation, diet, and esthetics during the growth process. In this case report, 1 case of syngnathia, a rare craniofacial anomaly, is presented with a review of reports. Prompt diagnosis and surgery were performed right after birth for the present case. A partial limitation point was resolved for further growth. Herein, the authors present the case of a female infant (7 d after birth) diagnosed with congenital syngnathia and treated by early surgical intervention.

Cleft palate-lateral synechiae syndrome: a case report.

Cleft palate-lateral synechiae syndrome (CPLSS) is an extremely rare congenital malformation syndrome with undetermined etiology, characterized by a cleft palate and lateral intraoral synechiae linking the free borders of the palate to the mouth floor. We report a case of a female neonate, admitted for suckling difficulties with a cleft lip and palate associated to multiple lateral intraoral synechiae. Resection of the synechiae allowed oral feeding. Cleft palate-lateral synechiae syndrome is an exceptional syndrome as only seventeen cases have been reported in the literature. Synechiae can be isolated or more frequently in association with other congenital anomalies such as cleft lip and/or palate. These synechiae can cause functional deficits, especially in the respiratory and feeding tracts, language disorders or recurrent otitis. Although it is exceptional, this malformative entity must be known by medical practitioners in order to set up a well-adapted therapeutic protocol.

Anesthetic Implications of Common Congenital Anomalies.

The most common congenital anomalies are congenital heart defects, cleft lip and palate, Down syndrome, and neural tube defects. Anesthetic considerations for Down syndrome include cervical spine instability, history of congenital heart disease, risk of bradycardia, hematologic, endocrine, and behavioral considerations. Patients with cleft lip and palate can have associated syndromes, and the potential for underlying abnormalities should be investigated prior to their anesthetic. A major anesthetic consideration for neural tube defect surgery is positioning for intubation. Fetal surgery for myelomeningocele has been shown to reduce the need for ventriculoperitoneal shunting and improved motor outcomes.

Vertical Distraction for the Treatment of Congenital Syngnathia.

Congenital syngnathia is a very rare condition that presents with several unique challenges. There is no consensus on the best operative approach to manage this condition. Readhesion of surgically separated tissues is a relatively common occurrence. The authors propose that the use of a vertical distraction technique helps maintain adequate separation to prevent relapse of the bony fusion. Long-term follow-up will be required to follow mandibular growth and implications of this treatment option.

Duplication of the oral cavity and mandible: a rare congenital craniofacial anomaly.

A newborn girl was referred to the otolaryngology service after prenatal imaging showed a right mandibular mass. Physical examination revealed a 1-2 cm mass along the right mandible with the appearance of a vestigial oral cavity. Tissue resembling the vermillion and primitive tongue appeared innervated and moved in conjunction with oral movements. MRI and CT of the mandible after birth confirmed a partially ossified soft tissue mass of the right mandibular body, containing unerupted teeth. She was taken to the operating room at 6 months of age for mass excision and reconstruction. Postoperatively, she healed well and was feeding without difficulty. Craniofacial duplication, including duplication of stomatodeal structures or diprosopus, is a rare condition with a variety of phenotypes. In the case of suspected craniofacial duplication, associated syndromes should be ruled out and appropriate imaging employed to determine the extent of involvement of adjacent structures, which will ultimately guide surgical planning.

Syngnathia in Spectrum of Oromandibular Limb Hypogenesis Syndrome.

Syngnathia is a rare facial anomaly associated with neonatal problems, including a compromised airway, and the inability to take in solids and/or liquids. Syngnathia is included within the spectrum of oromandibular limb hypogenesis syndrome, an extremely rare condition characterized by varying degrees of congenital malformation involving the tongue, mandible, and limbs. In this report, we describe the case of a 41-day-old Caucasian female infant who was unable to open her mouth beginning at birth. The authors performed osteotomies to separate fused bone, and placed a bite block at the osteotomy sites to prevent bone fusion recurrence. At 2 years of follow-up patient remained with 14 mm of mouth opening.

Congenital Unilateral Zygomatico-Maxillo-Mandibular Fusion.

Congenital fusion of the jaws (syngnathia) is a rare and severe disorder. The authors report a case of bony fusion of the left mandible with the maxilla and zygomatic complex in a 5-day-old male who was not able to feed and open his mouth normally. Early surgery was performed to release the bony fusion on the tenth day of life. The authors present a new technique based on the intraoperative use of 3-dimensional surgical guides.

Difference in Degrees of Satisfaction with Orthognathic Surgery and Orthodontic Treatment between Skeletal Class III and Cleft Patients.

OBJECTIVE: The aim of this study was to compare the degrees of satisfaction with orthognathic surgery and orthodontic treatment between skeletal Class III and cleft patients. MATERIALS AND METHODS: The samples consisted of Class III group (N = 25) and Cleft group (N = 16). The Modified Orthognathic Quality of Life Questionnaires, which had 5 domains (oral function [OF], awareness of dentofacial deformity [ADD], social relationship [SR], facial esthetics [FE], and nose/lip esthetics [NLE]), were evaluated with 5 rates (0 [very satisfactory] to 4 [very unsatisfactory]) at initial visit (T1), just before surgery (T2), 3 to 6 months after surgery (T3), and at debonding or 1 year after surgery (T4). The scores at each stage, amount of change between stages, and effect size (ES) in the 5 domains were investigated. RESULTS: Compared to Class III group, Cleft group exhibited lower satisfaction scores of NLE domain during all stages (all P < 0.001) and of SR domain and total domains at T4 stage (P < 0.05, P < 0.01). Cleft group showed significant improvement of satisfaction scores in FE domain during T1-T2 (P < 0.01), in SR, FE, NLE, and total domains during T2-T3 (all P < 0.01), in OF, SR, and total domains during T3-T4 (P < 0.05, P < 0.01, P < 0.01), and in all domains during T1-T4 (ADD, P < 0.05; OF, SR, and NLE, P < 0.01; FE and total, P < 0.001). Cleft group exhibited large improvement of ES only at SR and FE domains during T2-T3 (-0.81 and -1.09, respectively). CONCLUSIONS: Owing to lower satisfaction of NLE domain at all stages in cleft patients, clinicians should recommend adjunctive cosmetic surgery for nose and lip after completion of treatment.

Toxina botulínica para tratamento de síndrome do Choro Assimétrico: relato de caso / Botulinum toxin in the treatment of asymmetric crying face syndrome: a case report

Síndrome do choro assimétrico é uma condição congênita secundária à hipoplasia ou ausência do músculo depressor do ângulo da boca. Trata-se de uma condição não tão incomum que pode cursar com assimetria facial ao chorar e sorrir, além de poder estar associadas a outras malformações congênitas. Crianças com essa deformidade podem sofrer dificuldades psicossociais e introversão. O arsenal terapêutico dessa condição já foi estudado e discutido na literatura com ênfase em abordagens cirúrgicas e invasivas. Relatamos aqui um caso de uma criança de 9 anos com essa síndrome, tratada, de forma menos invasiva, com toxina botulínica, com um bom resultado e satisfação.

Asymmetric crying face syndrome is a congenital condition secondary to hypoplasia or absence of the depressor muscle at the mouth angle. It is a common condition that presents with facial asymmetry while crying and smiling and may be associated with other congenital malformations. Children with this deformity may experience psychosocial difficulties and introversion. The therapeutic arsenal of this condition has already been studied and discussed in the literature with an emphasis on surgical and invasive approaches. We report here a case of a 9-year-old child with this syndrome, treated less invasively with botulinum toxin, with good result and satisfaction.

Congenital Synechia and Syngnathia: Two Case Reports.

This case report presents two cases of rare congenital malformation, i.e syngnathia. First case is of 2-day infant with bilateral fusion of maxilla and mandible, leaving a small anterior portion. After consultation with other concerned specialties, early intervention was planned and fusion was released to facilitate feeding. The infant suffered from frequent respiratory tract infections and subsequently died at the age of ten months. The second case is of 8-month baby girl with unilateral congenital maxillomandibular bony fusion without any other anomaly. She underwent general anesthesia for thorough examination and release of soft tissue union. Second surgery was performed after few months for removal of bony fusion. Good mouth opening was seen on 1 month follow-up.

Procedimento de bichectomia: uma discussão sobre os aspectos éticos e legais em odontologia / Bichectomy procedure: a discussion on the ethical and legal aspects in odontology

Introdução: O procedimento denominado de bichectomia consiste na remoção parcial de uma estrutura adiposa na região das bochechas e ganhou recente popularidade entre a classe odontológica, que passou a executá-lo amplamente, com demandas predominantemente estéticas e, com isso, dúvidas e incertezas surgiram a respeito dos seus aspectos éticos e legais. Objetivo: O objetivo deste trabalho foi buscar legislações nacionais, bem como normativas e resoluções emitidas por conselhos profissionais, visando abordar tais controvérsias, alumiando os profissionais quanto à legitimidade do procedimento. Resultados: Verificou-se que desde 1978, os Conselhos Federais de Medicina e de Odontologia vêm emitindo resoluções para determinar limiares de atuação profissional, em especial, com enfoque na especialidade Cirurgia e Traumatologia Bucomaxilofacial, visto que é a especialidade odontológica clínica que mais se aproxima de campos de atuação médica. Com o tempo, estes Conselhos foram atualizando estas Resoluções, tendo em vista os avanços técnicos e científicos da área, porém todas as resoluções analisadas são unânimes em afirmar que a realização de bichectomia com finalidade estritamente estética é atribuição médica. Conclusão: Desta forma, com base nos documentos atualmente vigentes, verifica-se que o cirurgião-dentista que estiver disposto a realizar a cirurgia de bichectomia com finalidade exclusivamente estética estará incorrendo em transgressões administrativas e, consequentemente, tais interpretações podem ser vislumbradas em outras esferas (cíveis e criminais).

Introduction: Bichectomy consists of removing part of a fat structure in the region of the cheeks, and it recently gained popularity in the field of odontology, which began to widely perform the procedure, with predominantly aesthetic demands and, with that, doubts and uncertainties arose with respect to its ethical and legal aspects. Objective: The objective of this work was to seek national laws, as well as normative and resolutions issued by professional councils, aiming to address such controversies, enlightening professionals to the legitimacy of the procedure. Results: Since 1978, the Federal Councils of Medicine and Dentistry have issued resolutions to determine thresholds for professional performance, which particularly focused on Oral and Maxillofacial Surgery and Traumatology because it is the closest clinical dental specialty of medical practice. Over time, these Councils have been updating these resolutions, considering the technical and scientific advances of the area, but all the resolutions analyzed were unanimous in affirming that the accomplishment of bichectomy with a strictly aesthetic purpose is a medical attribution. Conclusion: Subsequently, based on the documents currently in force, it is verified that the dental surgeon who is willing to perform bichectomy surgery for aesthetic purposes will be incur administrative infractions and, consequently, such interpretations can be seen in other legal areas (civil and criminal).

Spearman-Brown prophecy formula and Cronbach's alpha: different faces of reliability and opportunities for new applications.

OBJECTIVES: There are similarities between the different forms of reliability, such as internal consistency (internal reliability) and interrater and intrarater reliability. Reliability coefficients that are based on classical test theory can be expressed as intraclass correlation coefficients (ICCs), such as Cronbach's alpha. The Spearman-Brown prophecy formula (SB formula) is used to calculate the reliability when the number of items in a questionnaire is changed. This paper aims to increase insight into reliability studies by pointing to the assumptions of reliability coefficients, similarities between various coefficients, and the subsequent new applications of reliability coefficients. DESIGN, SETTINGS AND RESULTS: The origin and assumptions of Cronbach's alpha and the SB formula are discussed. Cronbach's alpha is written as an ICC formula, using the well-known property that taking the average value of a number of ratings increases the reliability of a measurement. We illustrate with an example that the ICC formulas for average measurements of multiple raters and the SB formula give similar results. This implies that the SB formula can be used to decide on the number of measurements to be averaged and thus on the number of raters required, for obtaining measurements with acceptable reliability, even if the variance components of the ICC formula are not known. Using the same example, we illustrate the principle of "Cronbach's alpha if item deleted" to decide on the poorest performing raters in a set of raters. CONCLUSION: These applications have different assumptions: the principle of "Cronbach's alpha if item deleted" is based on the assumption of a fixed set of items/raters and the SB formula is based on the assumption of random raters. The example also emphasizes the need for more raters in the design of the reliability study to obtain a robust estimation of reliability.

A veil in the oral cavity: report of two cases of oral synechiae.

An oral synechia is an adhesion between the maxilla and the mandible; these adhesions can be found at various locations in the oral cavity. The presence of oral synechiae associated with cleft palate represents a rare congenital deformity. Oral synechiae with cleft palate can have serious implications for airway management and feeding in babies, and hence requires early intervention to reduce morbidity. Two cases of congenital oral synechiae accompanied by cleft palate, in which the synechiae veiled the oral cavity restricting a detailed intraoral examination, are reported here.

Tratamento primário da deformidade labial e nasal nas fissuras labiais e labiopalatinas unilaterais / Primary treatment of lip and nasal deformity in unilateral cleft lip or cleft lip and palate

Introdução: A fissura labiopalatina é a deformidade congênita mais frequente dentre as malformações craniofaciais, afetando principalmente o lábio superior, nariz e palato. A realização da queiloplastia associada à rinoplastia primária em tempo único é uma tendência. Avaliamos a correlação entre a gravidade da fissura, a idade cirúrgica e a qualidade estética do resultado pós-operatório. Métodos: Foram avaliados 26 pacientes com fissuras labiais ou labiopalatinas unilaterais, com idades entre 3 e 12 meses, operados pela técnica de queilorrinoplastia de Göteborg/McComb. Foi feita uma avaliação fotográfica do pré e pós-operatório por cinco cirurgiões plásticos separadamente e os dados analisados. Resultados: A idade média de realização do procedimento foi de 6,5 ± 3,15 meses, sendo que somente sete pacientes (26,9%) foram operados na idade de 3 meses preconizada pelo protocolo. Foi encontrada correlação entre a gravidade da fissura e a qualidade dos resultados, ao mesmo tempo em que não foi encontrada associação entre a idade da cirurgia e os resultados. Todos os casos do estudo foram considerados ótimos ou satisfatórios. Conclusões: A utilização de uma técnica de queiloplastia que seja de fácil reprodutibilidade, com bons resultados estéticos, e que possa ser utilizada em conjunto com outras técnicas de tratamento primário do nariz é uma boa opção a ser adotada por centros de tratamentos deste tipo de paciente. A gravidade da fissura é um fator importante na qualidade dos resultados. Quanto mais grave a fissura os resultados tendem ser piores. A idade da cirurgia, neste estudo, não teve correlação com a qualidade nos resultados.

Introduction: Cleft lip and palate, the most frequent congenital craniofacial deformity, mainly affects the upper lip, nose, and palate. One possible treatment is single-stage lip repair with primary rhinoplasty. Here we assessed the correlations among cleft severity, surgical age, and aesthetic results. Methods: A total of 26 patients with unilateral cleft lip or cleft lip and palate aged 3-12 months underwent surgical lip repair associated with the Göteborg/McComb rhinoplasty technique. Steps before and after surgery were separately evaluated by five plastic surgeons using pictures and the data were analyzed. Results: The average patient age at surgery was 6.5 ± 3.15 months, and only seven patients (26.9%) underwent surgery at 3 months of age as recommended by the protocol. Cleft severity and results quality were associated, whereas surgical age and aesthetic results were not correlated. Results in all cases were considered optimal or satisfactory. Conclusions: The lip repair technique, which presents good reproducibility and aesthetic results and can be used with other primary treatment techniques for the nose, should be adopted by treatment centers managing cleft lip and palate. Cleft severity is an important factor in results quality; the greater the severity, the worse the results. In the present study, surgical age was not correlated with results quality.

Congenital oral synechia and ankyloblepharon filiforme adnatum: Case report and literature review.

INTRODUCTION: Congenital intraoral synechias are adhesions of intraoral structures involving soft tissue. These conditions and another anomaly called ankyloblepharon filiforme adnatum, which is partial fusion of the eyelid margins by bands of tissue, are rarely reported in the literature. The association of both anomalies of the craniomaxillofacial region is an even more unusual finding. PURPOSE: This article adds more information to the literature by reporting a case of a neonate with a rare association of bilateral synechia between the jaws, fusion of buccal mucosa to the ridges and between the tongue and palate, and ankyloblepharon filiforme adnatum in both eyes, without any other facial or systemic malformation. METHODS: A literature review of similar cases published in PUBMED/MEDLINE was conducted followed by a discussion and comparison to the new case. CONCLUSION: The rare association of these congenital defects, without the presence of syndromic symptoms, could be diagnosed and treated easily with good prognostic evolution.

Multidisciplinary management of ankyloglossia in childhood. Treatment of 101 cases. A protocol.

BACKGROUND: Partial ankyloglossia is a limitation which restricts the possibility of protrusion and elevation of the tip of the tongue due to the shortness of either the lingual frenulum or the genioglossus muscles or both. The principal objective of this paper is to present our protocol of action for the treatment of ankyloglossia. The specific objectives are to study patients with ankyloglossia treated by the Service of Maxillofacial Surgery and the Service of Speech Therapy of our pediatric Hospital, describe the diagnostic procedures, the pre-surgical intervention, the surgical technique undertaken and the post-surgical rehabilitation taking into account the level of collaboration of the patients, and finally, describe the surgical complications and the referral of patients. MATERIAL AND METHODS: This is a descriptive study of healthy patients, without any diagnosis of syndrome, ranging between 4 and 14 years that have been surgically treated and rehabilitated post-surgery within a period of 2 years. RESULTS: 101 frenectomies and lingual plasties have been performed and patients have been treated following the protocol of action that we hereby present. After the surgical intervention, the degree of ankyloglossia has been improved, considering correction in 29 (28%) of the patients (95% CI: 20%, 38%), reaching, with the post-surgical orofacial rehabilitation, a correction of 97 (96%) of the participants (95% CI: 90%, 98%). CONCLUSIONS: The chosen surgical technique for moderate-severe ankyloglossia in our centre is the frenectomy and lingual plasty. The myofunctional training begins one week before the surgical intervention so that the patients learn the exercises without pain.

Developing a nurse-delivered frenulotomy service.

OBJECTIVES: Tongue tie (ankyloglossia) describes a short lingual frenulum that can lead to breastfeeding difficulties. It affects between 4% and 10% of infants and can be treated by frenulotomy. We developed a nurse-delivered frenulotomy service at a tertiary pediatric hospital and audited our results. STUDY DESIGN: Observational study. SETTING: Tertiary pediatric hospital. SUBJECTS AND METHODS: An outpatient tongue tie clinic was set up by an ear, nose, and throat consultant. Tongue tie division was undertaken using a standard technique without the need for anesthesia or analgesia, as per National Institute for Health and Care Excellence guidelines. Subsequently, a senior nurse was trained to undertake the clinic independently and saw most referrals. Patient satisfaction data were collected via questionnaires. RESULTS: Referrals to the service increased from 57 (2009) to 296 (2012). Outcome data from outpatient frenulotomy are discussed. Parent satisfaction measures were similar for both nurse- and doctor-delivered treatment. If all frenulotomies were undertaken in nurse-delivered clinics, a total of £3830 could have been saved in 2012, compared with the cost of doctor-delivered care. CONCLUSION: A nurse-delivered outpatient frenulotomy service is a safe and cost-effective method of delivering such care. We have demonstrated reduced costs and reduced waiting times without compromising patient satisfaction or the quality of care provided.