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.

Three case reports of infants with ankyloglossia affecting breastfeeding.

Ankyloglossia is a congenital anomaly which may reduce or restrict the tongue tip mobility. The restricted mobility is caused by an unusual short, thick lingual frenum. This condition may cause various problems in infants including breastfeeding in the new-borns. This case report describes 3 cases of ankyloglossia affecting breastfeeding and highlights the experiences of the mothers and their difficulties in breastfeeding babies with it. Comprehensive feeding examination was accomplished, the primary cause of feeding issues was identified, and frenotomy intervention was provided. Post frenotomy, infants were able to breastfeed easily and this was beneficial in continuation of breastfeeding and pain reduction in mothers.

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.

Variación de la dimensión del espacio orofaríngeo después de la cirugía de retroceso mandibular en pacientes con deformidad dentofacial clase III / Variation in the oropharyngeal space after mandibular setback surgery in class III malocclusion patients

OBJETIVOS: Comparar la variación de la dimensión del espacio orofaríngeo (EO) en el prequirúrgico, postquirúrgico inmediato y postquirúrgico de 5 a 24 meses después de la cirugía de retroceso mandibular (CRM). MATERIALES Y MÉTODOS: Se evaluaron las radiografías cefalométricas prequirúrgicas, postquirúrgicas inmediatas y postquirúrgicas de 5 a 24 meses de 20 pacientes con CRM del Servicio de Cirugía Oral y Maxilofacial de dos instituciones. Se cuantificó la dimensión del EO en diferentes momentos. RESULTADOS: No se encontraron diferencias de significación entre la cantidad de retroceso mandibular y la variación de la dimensión del EO. CONCLUSIONES: Existe una leve disminución de la dimensión del EO después de la CRM. No son estadísticamente significantes entre los momentos de evaluación. No se encontró correlación entre el retroceso mandibular y la disminución del EO

OBJECTIVE: The aim of this study was to compare the variation in the size of the oropharyngeal space (OS) in the preoperative, immediate postoperative and post-operative 5 to 24 months according to the amount of mandibular setback. MATERIAL AND METHOD: Variation were evaluated on cephalometric radiographs taken few days before the surgery (Rx1), immediate postoperative (Rx2) and postoperative long time (5 to 24 months) (Rx3). The subjects were 20 patients (18 - 36 years) in whom dentofacial deformity class III was corrected by bilateral sagittal split ramus osteotomy setback. Surgery was done in two Oral and Maxillofacial specialized institutions, since 2003 to 2010. We examined the size of the oropharyngeal space at different times before and after mandibular setback surgery. RESULTS: These shows no significant difference between the amount of mandibular setback and decrease the size of the oropharyngeal space. It was determined that the oropharyngeal space immediately after surgery increases and decreases over time as a result of soft and hard tissue adaptation. CONCLUSIONS: We conclude that there is a slight decreased in the anteroposterior dimension of oropharynx after mandibular setback surgery, however the variations is no statistically significant difference between the moments of evaluation (the amount of mandibular recoil and the decrease in OS)

Ankyloglossia and Other Oral Ties.

Ankyloglossia and other oral ties have been recognized for centuries, but interest in and literature on these topics has recently increased. This article presents the latest evidence on the diagnosis and management of tongue-tie and outlines some of the controversies and gaps in the existing evidence. Anterior tongue-tie is accepted in most clinical practices as a potential risk for breastfeeding difficulty, and good evidence exists that division of an anterior tongue-tie leads to improved breastfeeding outcomes. Posterior tongue ties and upper lip-ties are being studied more intensively to provide sound, evidence-based recommendations on their diagnosis and treatment.

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.

Complete bilateral Tessier's facial cleft number 5: surgical strategy for a rare case report.

The oro-ocular cleft number 5 according to the Tessier classification is one of the rarest facial clefts and few cases have been reported in the literature. Although the detailed structure of rare craniofacial clefts is well established, the cause of these pathological conditions is not. There are no existing guidelines for the management of this particular kind of cleft. We describe the case of a 19-month-old girl with a complete bilateral facial cleft. We describe the surgical steps taken to achieve the primary correction of the soft tissue deformation. Embryologic development and radiological approach are discussed, as are also the psychological and social aspects of severe facial deformities.

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).

Inclusión de caninos superiores en paciente con osteogénesis imperfecta. A propósito de un caso clínico / Impacted canines in a patient with osteogenesis imperfecta: a clinical case study

La osteogénesis imperfecta (OI) es un trastorno hereditario que cursa con fragilidad ósea lo que da lugar a fracturas de repetición. Afecta por igual ambos sexos, razas y grupos étnicos y se estima su incidencia en 20 casos por cada 100.000 nuevos nacimientos. Se presenta un caso de una paciente mujer de 16 años de edad diagnosticada de OI que acude a la consulta remitida por su ortodoncista por la presencia de ambos caninos superiores incluidos con la indicación de realizar exodoncia de canino superior izquierdo y realizar tratamiento quirúrgico-ortodóncico en canino superior derecho. Se hace una revisión de la literatura para conocer los aspectos más importantes de esta enfermedad, sus manifestaciones orales, así como el manejo quirúrgico de los trastornos odontoestomatológicos teniendo en cuenta su tratamiento farmacológico con bifosfonatos (AU)

Osteogenesis imperfecta (OI) is a hereditary condition which involves bone fragility, originating recurrent fractures. It affects both sexes, races and ethnicities equally, and its occurrence is estimated in 20 cases every 100,000 new births. The case presented relates to a 16-year-old female patient with an OI diagnosis who comes with a referral from her orthodontist due to both maxillary canines being impacted, with the direction of performing an exodontia of the left maxillary canine and a surgical-orthodontic treatment of the right maxillary canine. The literature is reviewed in order to determine the most relevant aspects of this disease, its oral manifestations and the surgical management of odontostomatological conditions considering their pharmacological treatment with biphosphonates (AU)

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.

Aplicaciones del colgajo sural medial en cirugía reconstructiva de cavidad oral y orofaringe / Applications of medial sural artery perforator flap in oral cavity and oropharynx reconstruction

Objetivos. Presentar nuestra serie clínica de colgajos de perforantes basados en la arteria sural medial revisando las indicaciones, las características del colgajo y las complicaciones asociadas. Material y métodos. Análisis retrospectivo de 25 pacientes con defectos de cavidad oral y orofaringe reconstruidos con colgajo sural medial desde 2010 hasta 2013 en el Servicio de Cirugía Oral y Maxilofacial del Hospital Universitario 12 de Octubre de Madrid. Resultados. El rango de edad fue de los 18 a los 82 años, siendo la edad media de 56,6 años. En cuanto a la distribución por sexos 14 eran varones y 11 mujeres. De los 25 colgajos realizados 15 fueron del miembro inferior izquierdo y 10 del derecho. La longitud media del pedículo fue de 11,21cm (7-16cm). En 8 casos presentaban 2 perforantes. No se evidenció ninguna perforante a menos de 8cm del pliegue poplíteo. El tamaño de la isla cutánea varió según las necesidades del defecto, entre 4 y 12cm de largo y de 2,5 a 6cm de ancho, con una media de 9,63×4,94cm. El cierre de la zona donante fue directo, sin precisar en ningún caso injerto dermoepidérmico. Dos colgajos presentaron necrosis total del mismo. Dos pacientes presentaron celulitis y dehiscencia parcial de la herida quirúrgica en miembro inferior, que evolucionó favorablemente con tratamiento antibiótico y curas locales. Conclusiones. El colgajo de perforantes de la arteria sural medial es una excelente opción en la reconstrucción de defectos de la cavidad oral y la orofaringe, proporcionándonos una adecuada adaptabilidad a la zona receptora y una mínima morbilidad de la zona donante (AU)

Objective. To present a clinical series of medial sural artery perforator flaps, including a review of its applications, characteristics, and related complications. Material and methods. A retrospective study was conducted on 25 patients who underwent oral cavity and oropharynx reconstruction with medial sural artery perforator flap from 2010 to 2013 in the Oral and Maxillofacial Surgery Department of the 12 de Octubre University Hospital (Madrid, Spain). Results. The ages ranged from 18 to 82 years (mean 56.6), with 14 males and 11 females. Of the 25 harvested flaps, 15 were from the left lower limb and 10 from the right. The mean pedicle length was 11.21cm (7-16cm). There were 2 perforations in 8 cases, but with no evidence of perforations less than 8cm away from the popliteal crease. The size of the skin paddle varied according to the defect, from 4 to 12cm long and 2.5 to 6cm wide, with a mean of 9.63×4.94. Direct closure of the donor site was performed in all cases with no skin graft needed. Two flap failures were reported. Two patients had inflammation and partial dehiscence of the surgical wound in the lower limb, with good results after local and antibiotic treatment. Conclusions. The medial sural artery perforator flap is an excellent alternative in oral cavity and oropharynx reconstruction, providing great adaptability to the defect and minimal donor site morbidity (AU)

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.