Deformidad congénita en dedos de manos y pies asociados a camptodactilia. Presentación de una familia afectada / Congenital deformity in fingers and toes associated with camptodactyly. Presentation of an affected family

Las deformidades de los dedos son alteraciones frecuentes y en ocasiones se asocian a camptodactilia. Estas pueden ser congénitas o adquiridas, estar asociadas a teratógenos durante la etapa prenatal o determinado por un gen heredado que provoque este defecto y manifestarse de forma aislada o en varios miembros de una misma familia, segregándose con un patrón de herencia autosómico dominante. Se presenta una familia con tres miembros afectados a partir de una progenitora con el defecto en los dedos de las manos y los pies, que tiene dos descendientes con las mismas deformidades, sin otras alteraciones. Se considera un defecto congénito con implicación estética, en la movilidad de todos los dedos en alguna medida. Resulta necesario el seguimiento multidisciplinario, en conjunto ortopédicos, psicólogos y genetista clínico, para brindar un adecuado asesoramiento genético a la familia, informar el posible tratamiento quirúrgico y la rehabilitación oportuna para elevar la calidad de vida de estos afectados.

Finger deformities are frequent alterations and are sometimes associated with camptodactyly. These can be congenital or acquired, and may be associated with teratogens during prenatal stage or determined by an inherited gene that causes this defect, as well as appear in isolation or in several members of the same family, segregating with an autosomal dominant inheritance pattern. We present a family with three affected members from a mother with a defect in fingers and toes, who has two descendants with the same deformities, without other alterations. It is considered a congenital defect with aesthetic implications, and repercussions, to some extent, on the mobility of all fingers. Multidisciplinary follow-up is necessary, together with orthopedics, psychologists, and clinical geneticists, to provide adequate genetic counseling to the family, inform possible surgical treatment, and timely rehabilitation to improve the quality of life of those affected subjects.

Reconstrução da ponta nasal: relatos de casos e revisão histórica / Nasal tip reconstruction: case reports and historical review

As amputações traumáticas da ponta nasal são lesões desfigurantes, que determinam importante rejeição social, impondo ao paciente um sofrimento adicional, além daqueles já causados pela ferida e suas limitações funcionais decorrentes do traumatismo. Tais defeitos representam um desafio para os cirurgiões plásticos, e existem diversas técnicas que poderão ser adotadas para tratar este mesmo tipo de defeito. Neste trabalho são mostrados inicialmente três casos de amputação traumática exclusivamente da ponta nasal com seus respectivos tratamentos, para em seguida apresentar uma revisão histórica e discussão das diversas técnicas utilizadas para reconstrução da ponta nasal, avaliando comparativamente e enfatizando a evolução técnica no armamentário da cirurgia plástica.

Traumatic amputations of the nasal tip are disfiguring injuries, which determine important social rejection, imposing additional suffering on the patient and those already caused by the wound and its functional limitations resulting from the trauma. Such defects represent a challenge for plastic surgeons, and several techniques can be adopted to treat this type of defect. This work shows three cases of traumatic amputation exclusively of the nasal tip with their respective treatments. They present a historical review and discussion of the different techniques used for nasal tip reconstruction, comparatively evaluating and emphasizing the technical evolution in plastic surgery armament.

Plication of the Suprahyoid Muscles Concomitant with Genioplasty to Improve the Aesthetics of the Submandibular Region / Plicatura de los Músculos Suprahioideos Concomitante con Genioplastia para Mejorar la Estética de la Región Submandibular

In addition to functionally correcting skeletal facial deformities, orthognathic surgery also has a great impact on aesthetics and facial proportions. However, patients who have soft tissue changes, such as tissue flaccidity, may not achieve an ideal result, and require complementary surgical techniques that promote aesthetic refinement. The literature on less invasive techniques is limited, and therefore, this work aims to report a surgical technique performed concomitantly with genioplasty to refine the harmony of the submandibular region, in a simple and effective way. Patient with skeletal class II deformity, with lack of mandibular definition and submandibular flaccidity, underwent bimaxillary advancement orthognathic surgery with advancement genioplasty to correct chin retrusion. Due to limited results with bone repositioning alone, plication of the suprahyoid musculature was performed in association with the same approach used in the genioplasty. As a result, there was traction in the cervical region, with a consequent decrease in the submental-cervical angle and improvement in submandibular aesthetics. Furthermore, there was no need for extensive surgical intervention and no impairment of the functional results of the orthognathic surgery itself. Therefore, it can be concluded that plication of the suprahyoid musculat ure concomitantly with genioplasty is a technique that promotes aesthetic gains in the cervical region.

Además de corregir funcionalmente las deformidades faciales esqueléticas, la cirugía ortognática también tiene un gran impacto en la estética y las proporciones faciales. Sin embargo, los pacientes que tienen cambios en los tejidos blandos, como la flacidez, pueden no lograr un resultado ideal y requieren técnicas quirúrgicas complementarias que promuevan el refinamiento estético. La literatura sobre técnicas menos invasivas es limitada, por lo que este trabajo tiene como objetivo reportar una técnica quirúrgica realizada concomitantemente con la genioplastia para refinar la armonía de la región submandibular, de manera simple y efectiva. Se trató a un paciente con deformidad esquelética clase II, con falta de definición mandibular y flacidez submandibular, intervenida de cirugía ortognática de avance bimaxilar con genioplastia de avance para corregir la retrusión del mentón. Debido a los resultados limitados solo con el reposicionamiento óseo, se realizó la plicatura de la musculatura suprahioidea en asociación con el mismo abordaje utilizado en la genioplastia. Como resultado, hubo tracción en la región cervical, con la consiguiente disminución del ángulo submentoniano-cervical y mejoría en la estética submandibular. Además, no hubo necesidad de una intervención quirúrgica extensa ni deterioro de los resultados funcionales de la cirugía ortognática en sí. Por lo tanto, se puede concluir que la plicatura de la musculatura suprahioidea concomitantemente con la genioplastia es una técnica que promueve ganancias estéticas en la región cervical.

Reconstrucción por técnica de Chevron reverso y transferencia tendinosa con técnica no convencional en Hallux Varus Iatrogénico Severo / Reconstruction by reverse Chevron technique and tendon transfer with unconventional technique in severe iatrogenic Hallux Varus

El Hallux Varus Iatrogénico es una complicación de la cirugía correctiva del Hallux Valgus muy poco frecuente. El tratamiento incluye la intervención de los tejidos blandos, y óseos. El propósito de este trabajo es mostrar los resultados clínico-radiológicos del tratamiento quirúrgico del Hallux Varus Iatrogénico Flexible Severo empleando la combinación de técnicas de reconstrucción alternativas en una paciente femenina de 59 años de edad. Se realizó liberación medial de la capsula articular MTF y abductor, osteotomía de Chevron reverso, artrodesis interfalángica izquierda, transferencia tendinosa del extensor largo de Hallux hacia plantar con botón artesanal. De acuerdo a la Escala AOFAS aumentó de 30 a 90 puntos. Se logró la corrección estable de la deformidad en varo y la consolidación de la osteotomía. El Hallux Varus Iatrogénico es una complicación poco frecuente considerada una deformidad mal tolerada, con pocos casos reportados y pocos reportes. Se debe hacer un enfoque de tratamiento paso a paso para abordar todos los elementos involucrados en el Hallux Varus iatrogénico. La corrección quirúrgica del Hallux Varus iatrogénico severo con técnicas de reconstrucción no convencionales tipo Chevron reverso y transferencia tendinosa con botón plantar, es una excelente opción terapéutica de acuerdo a los resultados obtenidos en este caso(AU)

Iatrogenic Hallux Varus is a very rare complication of Hallux Valgus corrective surgery. The treatment includes the intervention of soft tissues and bones. The purpose of this paper is to show the clinical-radiological results of the surgical treatment of Severe Flexible Iatrogenic Hallux Varus using the combination of alternative reconstruction techniques in a 59-year-old female patient. Medial release of the MTF and abductor joint capsule, reverse Chevron osteotomy, left interphalangeal arthrodesis, tendon transfer of the long extensor of Hallux to plantar with artisanal button was performed. According to the AOFAS Scale, it increased from 30 to 90 points. Stable correction of the varus deformity and consolidation of the osteotomy were achieved. Iatrogenic Hallux Varus is a rare complication considered a poorly tolerated deformity, with few reported cases and few reports. A step-by-step treatment approach must be taken to address all the elements involved in iatrogenic Hallux Varus. Surgical correction of severe iatrogenic Hallux Varus with unconventional reconstruction techniques such as reverse Chevron and plantar button tendon transfer is an excellent therapeutic option according to the results obtained in this case(AU)

Flexible flatfoot in the childhood: negative influence of inappropriate footwear

An analysis was carried out in relation to the adverse effects produced by the use of inadequately made footwear in patients with flexible flat feet in pediatric ages; we carried out an anatomical-functional reminder of the foot as a basic structure for standing and walking. The functional adaptability of the feet was valued in those populations with the habit of walking barefoot, also making a description of the basic characteristics that a properly made footwear must have. We address the different therapeutic approaches and their behavior over time. We highlight the widespread use in our society of inappropriate footwear, mainly of the flip-flop type, and its negative influence on the feet of those children with flexible flat feet and valgus of the ankle.

Se llevó a cabo un análisis en relación a los efectos adversos producidos por el uso de calzados de confección inadecuada en los pacientes portadores de pies planos flexibles en edades pediátricas. Se realizó un recordatorio anatomo-funcional del pie como estructura básica para la bipedestación y la marcha. Se valoró la adaptabilidad funcional de los pies en aquellas poblaciones con hábito de andar descalzos, haciéndose, además, una descripción de las características básica que debe reunir un calzado adecuadamente confeccionado. Se abordó los diferentes enfoques terapéuticos y su comportamiento en el tiempo, destacando el uso generalizado en la sociedad cubana de calzados inapropiados, principalmente del tipo chancletas, y su influencia negativa sobre los pies de aquellos niños con pies planos flexibles y tobillo valgo.

Correction of tibial multiplanar deformities using single Taylor external fixator combined with biplanar osteotomy / 中国修复重建外科杂志

OBJECTIVE@#To investigate the effectiveness of single Taylor external fixator combined with biplanar osteotomy on correction of tibial multiplanar deformities.@*METHODS@#Between October 2016 and December 2021, 11 patients with tibial multiplanar deformities (20 sides) were treated with single Taylor external fixator and biplanar osteotomy. Of them, 4 were male and 7 were female; the average age ranged from 13 to 33 years (mean, 21.9 years). Diagnosis included rickets severe genu varum deformity (7 cases, 14 sides), rickets severe genu valgum deformity (2 cases, 4 sides), multiple osteochondromatosis calf deformity (1 case, 1 side), neurofibromatosis medial lower leg anterior arch deformity with short of leg (1 case, 1 side). After fibular osteotomy and tibial multiplanar osteotomy, a Taylor external fixator was installed. After operation, the deformities were corrected successively and fixed completely. The osteotomy healed, then the external fixator was removed. Before operation and at 12 months after operation, the full-length X-ray films were taken. The leg-length discrepancy, medial proximal tibial angle (MPTA), lateral distal tibial angle (LDTA), posterior proximal tibial angle (PPTA), anterior distal tibial angle (ADTA), and tibial rotation angle were measured. The degree of lower limb deformity was scored with reference to a customized tibial mechanical axis scoring table.@*RESULTS@#Osteotomy was successfully completed without neurovascular injury and other complications. The external fixator was adjusted for 28-46 days, with an average of 37 days, and the external fixator was worn for 136-292 days, with an average of 169 days. Mild needle infection during the fixation period occurred in 3 sides, refracture at the distal tibial osteotomy in 1 side after removing the external fixator, and nonunion of the distal fibular osteotomy in 1 side. All patients were followed up 369-397 days (mean, 375 days). At 12 months after operation, the lower limb discrepancy decreased, but there was no significant difference ( P>0.05). MPTA, LDTA, PPTA, ADTA, and tibial rotation angle improved, and the differences in LDTA, ADTA, and tibial rotation angle were significant ( P<0.05). The score of lower limb deformity was significantly higher than that before operation ( P<0.05), and the results were excellent in 9 sides, good in 8 sides, fair in 3 sides, with the excellent and good rate of 85%.@*CONCLUSION@#Single Taylor external fixator combined with biplanar osteotomy is effective in the correction of tibial multiplanar deformities.

Prevalence of Absence of Palmaris Longus Tendon in a Population Sample from a Multiethnic Brazilian City / Prevalência de ausência de tendão palmaris longus em uma amostra populacional de uma cidade brasileira multiétnica

Abstract Objective The objective of the study was to determine the prevalence of absence of palmaris longus tendon in a population sample from a multiethnic Brazilian city. Methods A cross-sectional observational study was carried out between October 2017 and April 2018. We included male and female volunteers aged 18 years or older. The absence of palmaris longus tendon was determined by asking the volunteers to perform the Schaeffer test bilaterally. Results We prospectively collected data on 1,008 volunteers, 531 male and 477 female, with age between 18 and 74 years (mean 38.4 years). The absence of palmaris longus tendon was observed in 264 (26.2%) volunteers. Bilateral absence was detected in 123 volunteers (12.2%), 60 female (48.8%) and 63 male (51.2%). Unilateral absence was found in 141 patients (14.0%), 54 female (38.2%) and 87 male (61.8%) (p < 0.05). Conclusion The prevalence of absence of palmaris longus tendon in our study was 26.2%. There was no statistically significant difference between gender and bilaterally. The absence of palmaris longus tendon was predominant on unilateral right side in males.

Resumo Objetivo O objetivo do presente estudo foi determinar a prevalência de ausência de tendão palmaris longus em uma amostra populacional de uma cidade multiétnica brasileira. Métodos Um estudo observacional transversal foi realizado entre outubro de 2017 e abril de 2018. Incluímos voluntários dos sexos masculino e feminino com 18 anos ou mais. A ausência do tendão palmaris longus foi determinada pedindo aos voluntários que realizassem o teste de Schaeffer bilateralmente. Resultados Foram coletados prospectivamente dados de 1.008 voluntários, 531 homens e 477 mulheres, com idade entre 18 e 74 anos (média de 38,4 anos). A ausência do tendão palmaris longus foi observada em 264 (26,2%) voluntários. A ausência bilateral foi detectada em 123 voluntários (12,2%), 60 mulheres (48,8%) e 63 homens (51,2%). A ausência unilateral foi encontrada em 141 pacientes (14,0%), 54 mulheres (38,2%) e 87 homens (61,8%) (p<0,05). Conclusão A prevalência de ausência do tendão palmaris longus em nosso estudo foi de 26,2%. Não houve diferença estatisticamente significativa bilateralmente e entre gêneros. A ausência do tendão palmaris longus foi predominante no lado direito unilateral no sexo masculino.

Treatment of osteogenesis imperfecta using the fassier-duval telescopic rod / Tratamento da osteogênese imperfeita com a haste telescópica fassier-duval

ABSTRACT Objectives: This study aimed to assess the treatment of patients with Osteogenesis Imperfecta (OI) operated on with a telescopic Fassier-Duval (FD) rod in a querterenario hospital from 2010 to 2020. Methods: We analyzed indication for surgical treatment, causes of reoperation, complications and the effectiveness of telescoping rod. Results: The results were compared with the literature and with the same parameters from a previous study which a different telescopic rod developed by the same authors. This was a retrospective study based on the analysis of digital and radiographic clinical records. Fifteen patients with 21 FD rods were evaluated, most were used on the femur (18 rods or 85.7%), eight patients were female (53.3%), with a mean age of 10.47 (3.92 to 16.44) years, most of whom had type III Sillence (46.7%), with a mean follow-up of 5.22 (1.43 to 7.02) years. Seven rods (33.3%) had complications. The main indication was for fracture (57.1%). Regarding the ability to telescope, we observed that 15 rods (71.4%) followed the child's growth. Conclusion: We had good results using FD rods, similar to the data found in the literature and the data obtained with our rod. Level of Evidence III,Retrospective comparative study .

RESUMO Objetivos: O objetivo deste estudo foi avaliar o tratamento de pacientes com Osteogênese Imperfeita (OI) operados com a haste telescopada de Fassier-Duval (FD) num hospital quaternário no período de 2010 a 2020. Métodos: Analisamos a indicação cirúrgica do tratamento, as causas de revisão, suas complicações e a eficácia na telescopagem da haste. Resultados: Os resultados foram comparados com a literatura e com os mesmos parâmetros de um artigo anterior no qual foi utilizada uma haste telescopada desenvolvida pelo nosso grupo. O estudo foi retrospectivo baseado na análise dos prontuários clínicos digitais e radiográficos dos pacientes. Quinze pacientes com 21 hastes de FD foram avaliados, sendo a maioria no fêmur (85,7%), oito pacientes eram do sexo feminino (53,3%), com média de 10,47 (3,92 a 16,44) anos, a maioria do tipo III de Sillence (46,7%), com tempo de seguimento médio de 5,22 (1,43 a 7,02) anos. Deste total, sete hastes (33,3%) apresentaram complicações. A principal indicação cirúrgica foram fraturas (57,1%). Em relação à telescopagem, observamos que 15 hastes (71,4%) acompanharam o crescimento da criança. Conclusão: No presente estudo verificamos bons resultados com as hastes de FD, à semelhança dos dados encontrados na literatura e dos dados encontrados com a haste do nosso serviço. Nível de Evidência III; Estudo retrospectivo comparativo .

Establishing Cephalometric Norms in Primary Dentition Using Comprehensive Craniofacial Growth Analysis - A Digital Cephalometric Study

ABSTRACT Objective: To establish cephalometric norms in primary dentition among males and females using novel customized Comprehensive Cephalometric Growth (CCG) Analysis. Material and Methods: The study was conducted on 67 subjects with a mean age of 5.5 yrs. Digital lateral cephalometric radiographs were obtained using Planmeca Pro One. The digital images were then transferred to Nemoceph software. Craniofacial Growth (CCG) Analysis was configured in the software with five sub-groups. This sub-grouping was done such that related components were grouped together and comprehensively; it would provide an assessment of every component of the craniofacial region that could be affected either by treatment maneuver or growth process. The same was used for the cephalometric analysis and to determine the cephalometric norms in the primary dentition. Results: Certain linear measurements were higher among males when compared to females. However, most measurements remained similar among males and females during this age group. The CCG analysis provided a comprehensive knowledge of the craniofacial parameters during the growth process. Conclusion: The cephalometric norms during primary dentition thus established using Comprehensive Craniofacial Growth analysis would provide the data for early diagnosis and treatment planning in interceptive orthodontic treatment procedures.

Body mass index and weight loss in patients submitted to orthognathic surgery: a prospective study

ABSTRACT Objective: To compare the body mass index (BMI) and the weight loss (WL) in patients with dentofacial deformities who underwent monomaxillary versus bimaxillary orthognathic surgery. Materials and Methods: This prospective longitudinal study included 69 patients with dentofacial deformities who underwent surgical orthodontic treatment. Patients were divided into two groups according to the type of orthognathic surgery: monomaxillary or bimaxillary. A preoperative nutritional assessment based on BMI was performed; the percentage of involuntary WL between the preoperative and postoperative periods was also calculated. Data were collected at preoperative and 10, 40, and 90 days postoperative (PO). Statistical analysis was performed using SPSS 17.0 (IBM Corp., Armonk, NY, USA), and data are reported with 95% confidence interval. Results: According to BMI, patients who underwent monomaxillary surgery presented: underweight = 2.6%, normal weight = 51.3%, overweight = 35.9%, and obese = 10.3%. The subjects who underwent bimaxillary surgery presented: normal weight = 43.3%, overweight = 36.7%, and obese = 20%. BMI was similar between the groups at all time points (preoperative, p= 0.237; 10 days PO, p= 0.325; 40 days PO, p= 0.430; and 90 days PO, p= 0.609). All patients lost weight postoperatively, and WL was similar among the PO measurements (p= 0.163). Conclusions: Although both monomaxillary and bimaxillary orthognathic surgeries resulted in WL and lower BMI, there was no statistically significant difference in these metrics between the two types of surgery.

RESUMO Objetivo: Comparar o Índice de Massa Corporal (IMC) e a perda de peso (PP) de pacientes com deformidades dentofaciais após cirurgia ortognática monomaxilar e bimaxilar. Material e Métodos: Foi realizado um estudo longitudinal prospectivo em 69 pacientes com deformidade dentofacial submetidos a tratamento cirúrgico-ortodôntico. Os pacientes foram divididos em dois grupos: cirurgia ortognática monomaxilar e bimaxilar. Foi realizada avaliação nutricional pré-operatória de acordo com o IMC. Foi calculada a porcentagem de PP involuntária entre o pré e o pós-operatório (PO). A coleta de dados foi realizada no pré-operatório e aos 10, 40 e 90 dias PO. A análise estatística foi realizada no software SPSS v. 17.0, com intervalo de confiança de 95%. Resultados: De acordo com o IMC, os indivíduos submetidos à cirurgia monomaxilar apresentaram: baixo peso = 2,6%, peso normal = 51,3%, sobrepeso = 35,9% e obesidade = 10,3%. Entre os indivíduos submetidos à cirurgia bimaxilar, 43,3% estavam com peso normal, 36,7% estavam com sobrepeso e 20% eram obesos. O IMC foi semelhante em todos os períodos (pré-operatório, p= 0,237; 10 dias PO, p= 0,325; 40 dias PO, p= 0,430; e 90 dias PO, p= 0,609). Todos os pacientes perderam peso no pós-operatório. A PP foi semelhante entre os tempos de PO (p= 0,163). Conclusões: A cirurgia ortognática monomaxilar ou bimaxilar pode causar redução do peso corporal e diminuição no IMC, mas não há diferença estatisticamente significativa entre esses dois tipos de cirurgia em relação à PP e ao IMC.

Incidence of nasal pressure injury in preterm infants on nasal mask noninvasive ventilation / Incidência de lesão por pressão nasal em prematuros em ventilação não invasiva com máscara nasal

ABSTRACT Objective The aim of this study was to evaluate the incidence of nasal injury in preterm newborns (NB) using the Neonatal Skin Condition Score within 7 days of noninvasive ventilation (NIV) and to compare the incidence of injury in NB weighing ≥1,000 g and those weighing <1,000 g at the time of initiation of NIV support. Methods This is a prospective, observational study carried out in a neonatal intensive care unit of a public hospital in Rio Grande do Sul from July 2016 to January 2021. Patients were stratified into two groups at the time of NIV initiation: group 1 (weight ≥1,000 g) and group 2 (weight <1,000 g). To assess the condition of nasal injury, a rating scale called the Neonatal Skin Condition Score was applied during the first seven consecutive days on NIV. Kaplan-Meier, log-rank test, and Cox proportional hazards regression were used to estimate the hazard ratio (HR) and 95% confidence interval (CI). Results In total, 184 NB were evaluated. Nasal injury was reported in 55 (30%) NB. The risk of nasal injury was 74% higher in group 2 (19/45) than in group 1 (36/139) (HR: 1.74; 95%CI 0.99-3.03, p=0.048). Conclusion The incidence of nasal injury in infants submitted to NIV by nasal mask was high, and the risk of this injury was greater in preterm infants weighing <1,000 g.

RESUMO Objetivo: Avaliar a incidência de lesão por pressão nasal em recém-nascidos (RN) pré-termos usando a Escala de Condição da Pele do Recém-Nascido durante sete dias de ventilação não invasiva (VNI) e comparar a incidência em RN ≥N.000 g e aqueles <1.000 g ao início da VNI. Métodos: Estudo observacional prospectivo realizado em uma Unidade Neonatal de Terapia Intensiva de um hospital público do Rio Grande do Sul, no período de julho de 2016 a janeiro de 2021. Os RN prematuros foram estratificados em dois grupos no momento do início da VNI: Grupo 1 (1u.000 g) e Grupo 2 (<1.000 g). O Neonatal Skin Condition Score foi aplicado durante os primeiros sete dias consecutivos de VNI. Curvas de Kaplan-Meier e teste Log-Rank e regressão de riscos proporcionais de Cox foram utilizados para estimar a razão de risco (HR) e intervalo de confiança (IC) de 95%. Resultados: Foram avaliados 184 RN. A lesão nasal foi relatada em 55 (30%) deles. O risco de lesão nasal foi 74% maior no Grupo 2 (n=19 em 45) do que no Grupo 1 (n=36 em 139) (HR=1,74; IC95% 0,99-3,03; p=0,048). Conclusão: A incidência de lesão nasal em neonatos submetidos à VNI por máscara nasal foi alta, e o risco dessa lesão foi maior em RN com peso <1.000 g.

Evaluation of early treatment of idiopathic clubfoot using the ponseti method / Avaliação do tratamento precoce do pé torto congênito idiopático pelo método de ponseti

ABSTRACT Congenital clubfoot is one of the most common congenital deformities of the lower limbs, with an estimated incidence of 1 for every 1,000 live births. Its treatment is controversial, and currently the Ponseti method has been widespread, showing promising results and decreasing the need for extensive surgical releases, as was usually done until the introduction of the technique. Currently used in approximately 55 countries, the method has changes and scopes that vary according to the society in which it is applied, with the results and peculiarities of the method molded according to the sample studied. Objective: To evaluate the clinical outcome of clubfoot treatment using the Ponseti method under local conditions. Methods: The clinical evaluation will include a descriptive analysis of the sample, as well as radiographic evaluation and family satisfaction with the treatment. Results: In total, 46% of the patients had good results and no family was dissatisfied with the treatment results. No statistically relevant relationships were found between the studied variables. Conclusion: The results are good and generally similar to those in the literature. Our epidemiological data generally agree with those reported by other authors. Level of Evidence IV, Case Series.

RESUMO O pé torto congênito (PTC) é uma das deformidades congênitas mais comuns dos membros inferiores, com incidência estimada de um para cada 1.000 nascidos vivos. Seu tratamento é motivo de controvérsia, mas, atualmente, o método de Ponseti tem sido difundido, mostrando resultados promissores e diminuindo a necessidade das liberações cirúrgicas extensas, como geralmente se fazia até a introdução da técnica. Utilizado em aproximadamente 55 países, o método apresenta alterações e alcances que variam de acordo com a comunidade em que é aplicado, sendo o resultado e as peculiaridades do método moldados de acordo com a amostra estudada. Objetivo: Avaliar o resultado clínico do tratamento do PTC através do método de Ponseti em condições locais. Métodos: A avaliação clínica incluiu uma análise descritiva da amostra, além de avaliação radiográfica e satisfação da família com o tratamento. Resultados: 46% dos pacientes apresentaram bons resultados e nenhuma família se mostrou insatisfeita. Não foram encontradas relações estatisticamente relevantes entre as variáveis estudadas. Conclusão: Os resultados são bons e, de maneira geral, semelhantes àqueles da literatura. Há, também, concordância geral dos dados epidemiológicos deste estudo com os relatados por outros autores. Nível de Evidência IV, Série de Casos.

Exercises and neuromuscular electric stimulation for medial longitudinal arch: clinical trial / Exercícios e estimulação elétrica neuromuscular para arco longitudinal medial: ensaio clínico

ABSTRACT Objective The extrinsic muscles, such as the posterior tibialis and long flexor of the hallux and the intrinsic of the foot, are part of the active subsystem of the central system of the foot and play an essential role in the control of the medial longitudinal arch resulting from difficulty in contracting the muscle, neuromuscular electrostimulation (NMES) becomes a resource combined with strengthening and recommended for rehabilitation. T this work aims to evaluate the effectiveness of NMES associated with exercise in deforming the medial longitudinal arch. Methods This is a randomized blind clinical trial. 60 asymptomatic participants were divided into three groups: NMES, exercise and control. The NMES and exercise group performed seven exercises for the intrinsic and extrinsic muscles twice a week for 6 weeks, and the NMES group used an NMES associated with five exercises. Navicular height and medial longitudinal arch angle were taken before and after the intervention period. Results No statistically significant differences existed between groups for navicular height and medial longitudinal arch angle. Conclusion NMES associated with exercise does not change the characteristics of the medial longitudinal arch in association with asymptomatic. Level of Evidence I; Randomized clinical trial.

RESUMO Objetivo Os músculos extrínsecos, como o tibial posterior e flexor longo do hálux e os intrínsecos do pé fazem parte do subsistema ativo do foot core system e exercem papel essencial no controle do arco longitudinal medial. Devido à dificuldade na contração desses músculos, a eletroestimulação neuromuscular (EENM) torna-se um recurso aliado ao fortalecimento e é recomendada para reabilitação. O objetivo desse trabalho é avaliar a eficácia da EENM associada ao exercício na deformação do arco longitudinal medial. Métodos Este é um ensaio clínico randomizado cego. 60 participantes assintomáticos foram divididos em três grupos: EENM, exercício e controle. O grupo EENM e exercício realizaram sete exercícios para os músculos intrínsecos e extrínsecos duas vezes por semana por seis semanas, sendo o grupo EENM utilizou a EENM associada a cinco exercícios. A altura do navicular e o ângulo do arco longitudinal medial foram medidos antes e após o período de intervenção. Resultados Não houve diferenças estatisticamente significativas entre os grupos para a altura do navicular e ângulo do arco longitudinal medial. Conclusão A EENM associada ao exercício não altera as características do arco longitudinal medial em indivíduos assintomáticos. Nível de Evidência I; Estudo Clínico Randomizado.

Outcomes of Management of Severe Clubfoot among Children by Ilizarov External Fixator

@#Introduction: Clubfoot remains the most common birth defect involving the musculoskeletal system. There are various surgical and non-surgical treatment options available for the management of clubfoot. Using the minimally invasive Ilizarov external fixator method has been reported to have good success rates and fewer complications. Materials and methods: This study aimed at analysing the morphological and functional outcomes of treating severe clubfoot by Ilizarov external fixator among children from July 2017 to March 2020. Thirty-two children who had either failed Ponseti / surgery or neglected with 44 clubfeet of Dieglio type III and type IV were included in the study. A short-leg walking cast was applied for an additional six weeks after removing of Ilizarov frame and additionally followed by an orthosis for another six weeks. Outcomes were measured by the functional rating system by Laaveg and Ponseti and interpretation done at 1 month and 12 months after the ankle-foot arthrosis. Results: About 86.4% of the patients had good or excellent outcome scores. Pre and post-Demeglio scores and functional rating scores were statistically significant (p<0.001) by using Paired t-test. Complications included superficial pin site infections in 13 feet (29.54%), 5 feet (11.36%) had claw toes, 3 feet (6.81%) had linear skin necrosis and 2 feet (4.54%) had calcaneal fractures which were manageable with minor interventions. Conclusion: The study findings highlighted that the Ilizarov external fixator method can correct complex foot deformities of severe clubfoot with minimum morbidity. Further larger and long-term studies are needed to investigate the effects of the stiff hindfoot and possible degenerative changes on the function and symptoms of these patients as adults.

The short-term outcomes of congenital radioulnar synostosis patients treated with modified reverse Sauvé-Kapandji technique / 中华骨科杂志

Objective:To evaluate the short-term outcomes of modified reverse Sauvé-Kapandji technique in treating the congenital radioulnar synostosis.Methods:A retrospective analysis was performed on the data of 46 congenital radioulnar synostosis patients were treated with modified reverse Sauvé-Kapandji technique in Beijing Jishuitan Hospital from December 2018 to January 2020, including 38 males (45 sides), 8 females (9 sides), average age 6.6 (3.2, 8.1) years old. All the patients were classified as type III according to Cleary-Omer classification and were followed up for at least 1 year. All the patients were treated with same operation, in which 1.5 cm shaft was resected at the proximal radius, allogeneic graft tendon was used as interposition, and rotational osteotomy was performed in the middle of the ulnar shaft, with intramedullary needle or Kirschner wire fixation, depending on the intramedullary width of ulnar shaft. The radiological features were collected and recorded preoperatively and at the latest follow-up, together with the following evaluation indexes: modified Morrey tasks score, subjective function score, active forearm rotation range without compensation, active forearm rotation range with wrist joint compensation, and active forearm rotation range with wrist and shoulder joint compensation.Results:All patients were followed up for 14.6±3.4 months (range, 11.2-19.5 months). The uncompensated forearm rotation Angle was 0.0°±0.0° before surgery and 62.3°±23.7° after surgery. The forearm rotation angles before and after surgery with wrist compensatory surgery were 86.9°±29.4° and 133.2°±27.9°, respectively. The forearm rotation angles before and after surgery with wrist and shoulder joint compensatory surgery were 205.2°±42.7° and 245.2°±35.8°, respectively. There were statistically significant differences in the above indexes before and after surgery ( t=8.71, P<0.001; t=2.54, P=0.030; t=5.05, P<0.001). Ulnar union was observed in 31 patients (37 sides) after the operation, and the union duration was 6.1±2.3 months. There were 15 patients (17 sides) ulnar shafts faced with postoperative delayed union, the union duration was 8.4±1.6 months and were recovered after prolonging brace fixation and orthopedic shock wave treatment. The scores of subjective function and improved Morrey tasks of the 43 sides with good pseudo-joint were 12.1 (0.0, 20.8) and 0.7 (0.0, 1.0) points, respectively, which were improved compared with 33.9 (25.0, 41.6) and 3.2 (2.0, 4.0) points before surgery. The differences were statistically significant ( Z=-2.44, P=0.015; Z=-2.83, P=0.005). There were 11 forearms with postoperative pseudo-joint re-ankylosis, the average forearm rotation ranges without compensation was 11.4°±10.5°(range, 0°-30°), the average forearm rotation ranges with wrist compensation was 98.6°±15.9° (range, 80°-120°), the average forearm rotation ranges with wrist and shoulder compensation was 231.7°±16.9° (range, 210°-255°). The average subjective function scores was 26.7 (8.3, 39.6). The average modified Morrey tasks scores was 1.2 (0, 2), and there were no other postoperative complications. Conclusion:The reverse Sauvé-Kapandji technique showed a satisfying short-term outcome, and can be a new choice of treatment for type III congenital radioulnar synostosis.

Síndrome de Adams-Oliver y complicaciones asociadas: reporte de una familia en Colombia y revisión de la literatura / Adams-Oliver syndrome and associated complications: Report of a family in Colombia and review of the literature

El síndrome de Adams-Oliver es un trastorno congénito raro, caracterizado por aplasia cutis congénita en el cuero cabelludo, defectos terminales transversales de las extremidades y piel marmorata telangiectásica congénita. Este puede presentarse debido a diferentes patrones de herencia de tipo autosómico dominante o autosómico recesivo, o por mutaciones dominantes de novo. Aunque el síndrome de Adams-Oliver es una enfermedad poco frecuente, es importante conocer sus características clínicas y patrones de herencia, para así establecer un correcto diagnóstico y sus posibles complicaciones durante el seguimiento. En el presente estudio, se describe el caso de una adolescente con síndrome de Adams-Oliver con patrón de herencia autosómica dominante, hipertensión pulmonar y bronquitis plástica. Había varios miembros de su familia con el mismo compromiso

The Adams-Oliver syndrome is a rare congenital disorder characterized by aplasia cutis congenita of the scalp, terminal transverse limb defects, and congenital telangiectatic cutis marmorata. It can occur through different inheritance patterns: autosomal dominant, autosomal recessive, or de novo dominant mutations. Although the Adams-Oliver syndrome is a rare disease, it is essential to know its clinical characteristics and inheritance patterns, to establish a correct diagnosis and its possible complications during follow-up. In the present study, we describe the case of an adolescent with Adams-Oliver syndrome with an autosomal dominant inheritance pattern, pulmonary hypertension and plastic bronchitis, and several compromised family members.

Classificação e tratamento da mão em fenda: revisão da literatura / Cleft hand classification and treatment: literature review

A mão em fenda é uma deformidade congênita rara caracterizada por uma deficiência longitudinal dos raios centrais da mão, podendo estar associada a outras malformações. Devido ao amplo espectro de manifestações, o tratamento de mãos em fenda é desafiador. Este estudo objetiva apresentar as classificações, técnicas cirúrgicas mais indicadas e seguimentos adotados conforme a manifestação clínica. Foi realizada uma pesquisa nos bancos de dados Web of Science, PubMed, Scopus, Cochrane e Embase, descritores e termos relacionados à anomalia mão em fenda típica. Trinta e dois artigos foram incluídos, sendo analisados quanto a classificação da anomalia, classificação da gravidade de expressão, técnicas cirúrgicas e estudos com informações da intervenção cirúrgica adotada para uma coorte de pacientes. Considerando que estudos sobre mão em fenda são diretamente afetados pelas descobertas embriológicas, genéticas e de biologia molecular, diferentes classificações foram descritas e diversos estudos de complementação de técnicas cirúrgicas já existentes foram encontrados. Estudos inovadores são escassos. A padronização na descrição das técnicas e resultados, além de pesquisas de melhor qualidade, poderiam elucidar lacunas ainda existentes em torno das opções de tratamento.

Cleft hand is a rare congenital deformity characterized by a longitudinal deficiency of the central rays of the hand, which may be associated with other malformations. Due to the wide spectrum of manifestations, the treatment is challenging. This study aims to present the most suitable classifications, surgical techniques and follow-up adopted according to the clinical manifestation. A search was performed in the databases Web of Science, PubMed, Scopus, Cochrane and Embase, descriptors and terms related to the hand anomaly in a typical cleft. Thirty-two articles were included and analyzed regarding the classification of the anomaly, classification of the severity of expression, surgical techniques and studies with information on the surgical intervention adopted for a cohort of patients. Considering that studies about cleft hand could be directly affected by embryological, genetic and molecular biology discoveries, different classifications have been described and several studies to complement existing surgical techniques have been found. Innovative studies are scarce. Standardization in the description of techniques and results, in addition to better quality research, could elucidate gaps that still exist around treatment options.

Análise das vias aéreas superiores pós cirurgia ortognática bimaxilar: estudo retrospectivo / Upper airway analysis after bimaxillary orthognathic surgery: retrospective study / Análisis de la vía aérea superior después de la cirugía ortognática bimaxilar: estudio retrospectivo

Objetivo: O objetivo do presente estudo foi avaliar retrospectivamente as mudanças ocorridas nas vias aéreas superiores (VAS) pós cirurgia ortognática bimaxilar. Metodologia: A amostra compreendeu 14 pacientes, que foram divididos em dois grupos, conforme o tipo de movimentação realizada na cirurgia: grupo 1 (n = 6), avanço bimaxilar; grupo 2 (n = 8) cirurgia de avanço de maxila e recuo de mandíbula. Foram realizadas tomografias computadorizadas no pré-operatório (T0) e pós-operatório de 1 ano (T1). Através do software Dolphin Imaging procedeu-se a análise das VAS em três parâmetros: área total (AT), volume total (VT) e área axial mínima (AAM), que foram comparadas entre T0 e T1 em um mesmo grupos pelo Teste de Wilcoxon e entre grupos pelos Teste de Mann-Whitney (p < 0.05). Resultados: Ambos os grupos apresentaram aumento significativo de AT, VT e AAM entre T0 e T1. Contudo, essas variações foram estatisticamente maiores no grupo 1 quando comparadas ao grupo 2. Conclusão: As cirurgias bimaxilares promoveram o aumento da AT, VT e AAM das VAS e essas mudanças foram significativamente superiores nos pacientes submetidos ao avanço bimaxilar... (AU)

Objective: The objective of the present study was to retrospectively evaluate the changes that occurred in the upper airways (UAS) after bimaxillary orthognathic surgery. Methodology: The sample comprised 14 patients, who were divided into two groups, according to the type of movement performed in the surgery: group 1 (n = 6), bimaxillary advancement; group 2 (n = 8) maxillary advancement and mandibular setback surgery. Computed tomography scans were performed preoperatively (T0) and 1 year postoperatively (T1). Through the Dolphin Imaging software, the analysis of the UAS was carried out in three parameters: total area (TA), total volume (TV) and minimum axial area (MAA), which were compared between T0 and T1 in the same groups by the Wilcoxon Test and between groups by the Mann-Whitney test (p < 0.05). Results: Both groups showed a significant increase in TA, TV and MAA between T0 and T1. However, these variations were statistically higher in group 1 when compared to group 2. Conclusion: Bimaxillary surgeries promoted an increase in the TA, TV and MAA of the UAS and these changes were significantly higher in patients undergoing bimaxillary advancement... (AU)

Objetivo: El objetivo del presente estudio fue evaluar retrospectivamente los cambios ocurridos en las vías aéreas superiores (VAS) después de la cirugía ortognática bimaxilar. Metodología: La muestra estuvo compuesta por 14 pacientes, quienes fueron divididos en dos grupos, según el tipo de movimiento realizado en la cirugía: grupo 1 (n = 6), avance bimaxilar; grupo 2 (n = 8) cirugía de avance maxilar y retroceso mandibular. Las tomografías computarizadas se realizaron antes de la operación (T0) y 1 año después de la operación (T1). A través del software Dolphin Imaging se realizó el análisis de la VAS en tres parámetros: área total (AT), volumen total (VT) y área axial mínima (AAM), los cuales fueron comparados entre T0 y T1 en los mismos grupos por el Prueba de Wilcoxon y entre grupos por la prueba de Mann Whitney (p < 0,05). Resultados: Ambos grupos mostraron un aumento significativo de AT, VT y AAM entre T0 y T1. Sin embargo, estas variaciones fueron estadísticamente mayores en el grupo 1 en comparación con el grupo 2. Conclusión: Las cirugías bimaxilares promovieron un aumento de la AT, VT y AAM de las VAS y estos cambios fueron significativamente mayores en los pacientes sometidos a avance bimaxilar... (AU)

O papel da estereorradiografia na avaliação das deformidades dos membros inferiores / The role of stereoradiography in the evaluation of lower limb deformities

Resumo As deformidades dos membros inferiores são uma condição comum e podem levar a alterações da marcha e afetar a função e longevidade das articulações do quadril, do joelho e da coluna. Uma abordagem diagnóstica sistemática é essencial para definir o tratamento e alcançar o resultado terapêutico desejado com a menor taxa de complicações. A radiografia panorâmica é frequentemente utilizada para caracterizar as deformidades dos membros inferiores por meio de medidas de comprimento e desvios angulares dos eixos, além de se tratar de um método de baixo custo e alta disponibilidade. No entanto, como é frequente a combinação de deformidades em dois ou três planos ortogonais dos membros, a avaliação radiográfica perde acurácia por se tratar de um método de imagem bidimensional. Nesse sentido, deformidades em valgo/varo avaliadas radiograficamente no plano coronal apresentarão variações crescentes nas medidas dependendo do grau de flexão/recurvatum, torções ósseas anômalas ou, não menos importante, um posicionamento inadequado. A estereorradiografia biplanar de baixa dose, por meio de modelos tridimensionais, permite obter medidas mais acuradas de vários parâmetros usados na avaliação das deformidades dos membros inferiores, incluindo comprimentos, eixos e as torções tibial e femoral, antes disponíveis apenas pela tomografia computadorizada, com a vantagem de ser realizada em posição funcional com carga. Além disso, por permitir uma avaliação global da cabeça aos pés, abre uma nova perspectiva de compreender a inter-relação das deformidades dos membros com o posicionamento da bacia e com as deformidades da coluna.

Abstract Deformities of the lower limbs are a common condition and can lead to changes in gait, as well as affecting the function and longevity of the hips, knees, and spine. A systematic approach is essential to achieve the desired therapeutic result with the lowest rate of complications. Panoramic radiography is a widely available, low-cost method that is commonly used in order to assess the length and angular deformities of the lower limbs, by measuring the length and angular deviations of the axes. However, because the combination of lower limb deformities in two or three orthogonal planes is common, conventional radiography lacks accuracy because it is a two-dimensional imaging method. Therefore, the measurements of valgus/varus deformities on X-rays restricted to the coronal plane will present increasing variations in measurements depending on the degree of flexion/recurvatum alignment, anomalous bone torsions, or, last but not least, inappropriate patient positioning. Low-dose biplanar stereoradiography using three-dimensional models increases the accuracy of the measurement of several parameters used in the evaluation of lower limb alignment, including lengths, axes, and tibial/femoral torsions, parameters that could previously be evaluated only by computed tomography. Stereoradiography also makes it possible to perform a head-to-toe evaluation, as well as to evaluate the interactions among the lower limbs, pelvis, and spine.

Artrogriposis múltiple congénita. Caso clínico

RESUMEN La artrogriposis múltiple congénita es una enfermedad de baja frecuencia, esporádica, no progresiva, que aparece en el período prenatal y se caracteriza por varias contracturas articulares presentes al nacimiento en los cuatro miembros. Se estima una incidencia de 1/10 000 nacidos vivos. El diagnóstico es posible al realizar los ultrasonidos en un feto que tiene posiciones viciosas y que no se mueve. La prevalencia de la artrogriposis múltiple congénita es variable, resultando la más frecuente la artrogriposis múltiple clásica (amioplasia), presente entre el 40 y el 50 % de los afectados. La búsqueda ultrasonográfica en el tercer trimestre del embarazo es fundamental con fines diagnósticos, para brindar asesoramiento genético y preparar un equipo para el nacimiento. Es importante tener sospecha diagnóstica para sugerir la vía alta por cesárea, para bienestar fetal. Un grupo multidisciplinario debe llevar a cabo el manejo y tratamiento de estos enfermos. Se presenta el caso de un neonato nacido a las 39 semanas por parto eutócico prolongado por presentación de cara, con sufrimiento fetal agudo, meconio ++++, apgar 5-7, con peso de 3 300 g, que presentó luxación y contractura generalizada de hombros, codos, así como de caderas, rodillas y tobillos, con dedos de manos y pies en flexión.

ABSTRACT Congenital multiple arthrogryposis is a low-frequency, sporadic, non-progressive disease that appears in the prenatal period, and is characterized by several contractures present at birth in the four limbs. The estimated incidence is 1/10 000 born alive. The diagnosis is possible performing ultrasounds on a fetus that has vicious positions and does not move. The prevalence of congenital multiple arthrogryposis is variable, being classical multiple arthrogryposis (amyoplasia) the most frequent one, present in between 40 and 50 % of the affected persons. Ultrasonographic search in the third semester of pregnancy is essential for diagnostic purposes to provide genetic counseling and to prepare a ream for birth. It is important to have diagnostic suspicion to suggest the high cesarean way for fetal well-being. A multidisciplinary group should carry out the management and treatment of these patients. The case of a newborn is presented, who was born at 39 weeks by prolonged eutocic delivery due to presenting face, with acute fetal suffering, meconium ++++, apgar 5-7, weighing 3 300 g, that presented luxation and general contracture of shoulders, elbows, and also hips, knees and ankles, with fingers and toes in flexion.