Deformidad en equino del tobillo en edades pediátricas

La deformidad en equino del tobillo ocurre como consecuencia de múltiples entidades. Aunque la primera línea de tratamiento es la conservadora, las modalidades quirúrgicas son necesarias en la mayoría de los enfermos pediátricos. Estas últimas son las más empleadas por zonas del complejo músculo-tendinoso, en especial los alargamientos fraccionados y en forma de Z-plastia. El objetivo de este trabajo es actualizar y brindar información sobre los distintos procedimientos quirúrgicos en la corrección de la deformidad en equino del tobillo. En la búsqueda y análisis de la información se emplearon las siguientes palabras: equinus deformity, equino varus, equino valgus; drop foot deformity y Achilles tendon Z-lengthening. A partir de la información obtenida, se realizó unala revisión bibliográfica de un total de 187 artículos publicados en las bases de datos PubMed, Hinari, SciELO, EBSCO, Scopus, Medscape y Medline, mediante el gestor de búsqueda y administrador de referencias EndNote. De ellos se utilizaron 30, 28 de los últimos cinco años. Se hace referencia a la anatomía esencial de la zona, al igual que a la prueba de Silfverskiöld. Con relación a la imagenología, se describe la técnica para calcular la distancia del tendón a alargar. Se mencionan las técnicas quirúrgicas de alargamiento fraccionado, por Z-plastia, trasposición anterior del tendón de Aquiles y la hemiepifisiodesis.

Equinus deformity of the ankle occurs as a consequence of multiple entities. Although the first line of treatment is conservative, surgical modalities are necessary in most pediatric patients. The latter are the most used for areas of the muscle-tendinous complex, especially fractional and Z-plasty-shaped lengthening. The aim of this work is to update and provide information on the different surgical procedures in the correction of equinus deformity of the ankle. In the search and analysis of the information, the following words were used: equinus deformity, equinovarus, equinovalgus; drop foot deformity and Achilles tendon Z-lengthening. Based on the information obtained, a bibliographic review of a total of 187 articles published in PubMed, Hinari, SciELO, EBSCO, Scopus, Medscape and Medline databases was carried out using the search manager and reference administrator EndNote. Of these, 30 were used, 28 of the last five years. Reference is made to the essential anatomy of the area, as well as to the Silfverskiöld test. In relation to imaging, the technique to calculate the distance of the tendon to be lengthened is described. Fractional lengthening surgical techniques are mentioned, by Z-plasty, anterior transposition of the Achilles tendon and hemiepiphysiodesis.

Hyaluronic Acid Dermal Fillers for Treatment of Congenital and Acquired Lip Asymmetries and Deformities

Hyaluronic acid (HA) dermal fillers are widely used in aesthetic treatments for facial and lip modeling. Despite HA fillers has largely known to be use in procedures for augmentation the lip volume, their application to management lip abnormalities is not widespread. This study reviewed the use of HA fillers for reconstruction of congenital and acquired li p asymmetries and deformities, searching to expand knowledge about this treatment modality. To undertake this narrative review, the Medline-Pubmed, Web of Science, Scopus, Embase, Google Schoolar e Lillacs databases were searched. Several studies have reported positive results in the use of HA fillers for the treatment of lip deformities caused by different factors such as: Insatisfactory dermal fillers, permanent lip implants, excision of lip carcinoma, post-operative scars and electrical burns. HA fillers are also an alternative for the management of lip asymmetries and residual surgical scars in patients with cleft lip. Moreover, injection of HA fillers in individuals with facial paralysis and facioscapulohumeral muscular dystrophy can also improve lip incompetence. Additionally, HA fillers can be used as a complementary treatment in cases of severe malocclusion associated with skeletal changes, helping to maintain the seal and refine the lip appearance. Therefore, HA fillers can be used as alternative treatment for several types of congenital and acquired lip deformities and asymmetries. However, it is necessary to carry out randomized clinical trials with a greater number of patients and follow-up time, in order to investigate the benefits of the HA fillers for carriers patients of lip abnormalities.

Los rellenos dérmicos de ácido hialurónico (AH) son muy utilizados en tratamientos estéticos para el modelado facial y de labios. A pesar de que se sabe en gran medida que los rellenos de HA se utilizan en procedimientos para aumentar el volumen de los labios, su aplicación para el tratamiento de anomalías labiales no está muy extendida. Este estudio revisó el uso de rellenos de HA para la reconstrucción de asimetrías y deformidades labiales congénitas y adquiridas, buscando ampliar el conocimiento sobre esta modalidad de tratamiento. Para realizar esta revisión narrativa, se realizaron búsquedas en las bases de datos Medline-Pubmed, Web of Science, Scopus, Embase, Google Schoolar y Lillacs. Varios estudios han reportado resultados positivos en el uso de rellenos de HA para el tratamiento de deformidades labiales causadas por diferentes factores tales como: rellenos dérmicos insatisfactorios, implantes labiales permanentes, escisión de carcinoma labial, cicatrices postoperatorias y quemaduras eléctricas. Los rellenos de HA también son una alternativa para el manejo de asimetrías labiales y cicatrices quirúrgicas residuales en pacientes con labio hendido. Además, la inyección de rellenos de HA en personas con parálisis facial y distrofia muscular facioescapulohumeral también puede mejorar la incompetencia de los labios. Además, los rellenos de HA se pueden usar como un tratamiento complementario en casos de maloclusión severa asociada con cambios esqueléticos, lo que ayuda a mantener el sellado y refinar la apariencia de los labios. Por lo tanto, los rellenos de HA se pueden utilizar como tratamiento alternativo para varios tipos de deformidades y asimetrías labiales congénitas y adquiridas. Sin embargo, es necesario realizar ensayos clínicos aleatorizados con mayor número de pacientes y tiempo de seguimiento, para investigar los beneficios de los rellenos HA para pacientes portadores de anomalías labiales.

Customization of spectacle frames for individuals with facial deformities by altering conventional spectacle frames – Phase I

Purpose: Manufacturing a spectacle frame for a facially deformed individual is challenging because of facial asymmetry. One of the solutions is the customization of spectacle frames. Customization of spectacle frames for facially deformed individuals requires a better understanding of the facial anthropometry of deformed faces. This study aimed to analyze the facial anthropometry of deformed faces to understand the range of variability. The results of this study will be used to find customization methods in the future. Methods: We measured and analyzed the 12 facial parameters of individuals with facial deformities using the ImageJ software. Results: The data collected were normally distributed. Paired sample test revealed a statistically significant difference between innercanthus distances (right innercanthus distance [RICD] and left innercanthus distance [LICD]). Correlation analysis showed a positive difference between horizontal and vertical pupillary distance?innercanthus distance (PD?ICD) (0.68, 0.75, and 0.81) and pupillary distance?helix distance (PD?HD) (0.57, 0.68, and 0.59) relations. PD?ICD correlations are stronger compared to the PD?HD relation. Conclusion: Altering the frame center distance and the temple heights are the most important for asymmetric faces. Large population?based data are required to make concrete decisions to design a spectacle frame for asymmetric faces.

Analysis of the effectiveness of sequential plate internal fixation in correction of Madelung deformity after ulnar osteotomy and shortening / 中国修复重建外科杂志

OBJECTIVE@#To investigate the effectiveness of sequential plate internal fixation in the correction of Madelung deformity after ulnar osteotomy and shortening.@*METHODS@#The clinical data of 13 patients with Madelung deformity admitted between September 2015 and July 2021 were retrospectively analyzed. There were 5 males and 8 females with an average age of 18.3 years ranging from 17 to 23 years. The disease duration ranged from 12 to 24 months, with an average of 17 months. Three cases had a clear history of trauma. All patients had external radial deviation deformity and limited movement of the ulnar deviation, and the ulnar impact pain was significant during ulnar deviation movement; 9 patients had limited wrist joint supination movement, and the supination movement was normal. In the first stage, ulnar osteotomy and shortening combined with external fixator were used to correct wrist deformity in 13 patients. After operation, bone transfer was performed 6 times per day, with adjustments made every 4 hours, which was 1 mm per day. After the osteotomy was in place, the ulnar plate internal fixation was performed to reconstruct the ulnar stability in the second stage. The Cooney wrist joint score was used to assess the pain, function, range of motion, flexion and extension range of motion, and grip strength of the wrist joint before operation and before the removal of internal fixator. The subjective feeling and appearance satisfaction of patients were recorded.@*RESULTS@#After the second-stage operation, all the 13 patients were followed up 10-22 months, with an average of 15 months. The deformity of wrist joint disappeared after operation, and the flexion, extension, and ulnar deviation were basically normal. There was no complication such as ulnar impingement sign, nonunion or infection. Wrist function, pain, and range of motion were significantly improved after operation, except for 1 patient who had no significant improvement in rotation and pain. The ulnar internal fixator was removed at 10-18 months after the second-stage operation. The scores of pain, function, range of motion, flexion and extension range of motion, and grip strength in the Cooney wrist score before removal of internal fixator significantly improved when compared with those before operation ( P<0.05). Subjective and appearance satisfaction of patients were excellent in 9 cases, good in 3 cases, and fair in 1 case.@*CONCLUSION@#Ulnar osteotomy and shortening with sequential plate internal fixation for correction of Madelung deformity, with mild postoperative pain, can effectively avoid bone nonunion, improve wrist joint function, and have significant effectiveness.

Short-term effectiveness of INBONE TM Ⅱ total ankle prosthesis arthroplasty in the treatment of moderate to severe varus-type ankle arthritis / 中国修复重建外科杂志

OBJECTIVE@#To investigate the short-term effectiveness of INBONE TM Ⅱ total ankle prosthesis arthroplasty in the treatment of moderate to severe varus-type ankle arthritis.@*METHODS@#The clinical and radiographic data of patients with moderate to severe varus-type ankle arthritis, who were admitted between May 2017 and November 2021 and treated with total ankle arthroplasty (TAA) using INBONE TM Ⅱ prosthesis, was retrospectively analyzed. A total of 58 patients (58 ankles) met the selection criteria and were included in the study. Among them, there were 24 males and 34 females, with an average age of 62.6 years (range, 41-85 years). According to the preoperative tibiotalar angle (TTA), the patients were divided into a moderate varus group (group A, TTA 5°-15°, n=34) and a severe varus group (group B, TTA>15°, n=24). There was no significant difference in gender, side, etiology, preoperative American Orthopaedic Foot and Ankle Society (AOFAS) score, ankle dorsiflexion, plantarflexion, and total range of motion, and tibial lateral surface angle (TLS) between the two groups ( P>0.05). Yet the patients in group A were younger than group B, the degrees of oesteoarthritis (Takakura stage) and ankle pain [visual analogue scale (VAS) score] were milder, and the TTA, talar tilt angle (TT), hindfoot alignment angle (HAA) were smaller while the tibial articular surface angle (TAS) was larger, showing significant differences ( P<0.05). The pre- and post-operative VAS score, AOFAS score, the occurrence of early and late complications, the radiographic parameters of the ankle (TTA, TAS, TT, HAA, TLS), ankle dorsiflexion, plantarflexion, and total range of motion were recorded and compared.@*RESULTS@#All patients were followed up 19-72 months, with an average of 38.9 months. Compared with the preoperative data, the VAS score of all patients significantly decreased ( P<0.05); the AOFAS score, ankle dorsiflexion range of motion, and total range of motion significantly increased ( P<0.05); and the TTA, TAS, TT, HAA, and TLS significantly improved at last follow-up ( P<0.05); but there was no significant difference in plantarflexion range of motion ( P>0.05). Early complications occurred in 13 patients, and only 1 patient underwent revision surgery due to a larger size of the talar component. At last follow-up, there was no significant difference in the difference of clinical parameters before and after operation between the two groups ( P>0.05); there was a significant difference in the difference of other radiographic parameters ( P<0.05) except TLS. No significant difference in the incidence of complications between the two groups was found ( P>0.05).@*CONCLUSION@#TAA using the INBONE TM Ⅱtotal ankle prosthesis is an effective treatment for moderate or severe varus-type ankle arthritis, and good clinical and radiographic results can be obtained. Correcting bony deformities and balancing soft tissue are the keys to successful surgery.

Application of back-forward Bending CT localization image in the prediction of proximal junctional kyphosis after spinal deformity surgery in adults / 中国修复重建外科杂志

OBJECTIVE@#To investigate the feasibility of predicting proximal junctional kyphosis (PJK) in adults after spinal deformity surgery based on back-forward Bending CT localization images and related predictive indicators.@*METHODS@#A retrospective analysis was performed for 31 adult patients with spinal deformity who underwent posterior osteotomy and long-segment fusion fixation between March 2017 and March 2020. There were 5 males and 26 females with an average age of 62.5 years (range, 30-77 years). The upper instrumented vertebrae (UIV) located at T 5 in 1 case, T 6 in 1 case, T 9 in 13 cases, T 10 in 12 cases, and T 11 in 4 cases. The lowest instrumented vertebrae (LIV) located at L 1 in 3 cases, L 2 in 3 cases, L 3 in 10 cases, L 4 in 7 cases, L 5 in 5 cases, and S 1 in 3 cases. Based on the full-length lateral X-ray film of the spine in the standing position before and after operation and back-forward Bending CT localization images before operation, the sagittal sequence of the spine was obtained, and the relevant indexes were measured, including thoracic kyphosis (TK), lumbar lordosis (LL), local kyphosis Cobb angle (LKCA) [the difference between the different positions before operation (recovery value) was calculated], kyphosis flexibility, hyperextension sagittal vertical axis (hSVA), T 2-L 5 hyperextension C 7-vertebral sagittal offset (hC 7-VSO), and pre- and post-operative proximal junctional angle (PJA). At last follow-up, the patients were divided into PJK and non-PJK groups based on PJA to determine whether they had PJK. The gender, age, body mass index (BMI), number of fusion segments, number of cases with coronal plane deformity, bone mineral density (T value), UIV position, LIV position, operation time, intraoperative blood loss, osteotomy grading, and related imaging indicators were compared between the two groups. The hC 7-VSO of the vertebral body with significant differences between groups was taken, and the receiver operating characteristic curve (ROC) was used to evaluate its accuracy in predicting the occurrence of PJK.@*RESULTS@#All 31 patients were followed up 13-52 months, with an average of 30.0 months. The patient's PJA was 1.4°-29.0° at last follow-up, with an average of 10.4°; PJK occurred in 8 cases (25.8%). There was no significant difference in gender, age, BMI, number of fusion segments, number of cases with coronal plane deformity, bone mineral density (T value), UIV position, LIV position, operation time, intraoperative blood loss, and osteotomy grading between the two groups ( P>0.05). Imaging measurements showed that the LL recovery value and T 8-L 3 vertebral hC 7-VSO in the PJK group were significantly higher than those in the non-PJK group ( P>0.05). There was no significant difference in hyperextension TK, hyperextension LL, hyperextension LKCA, TK recovery value, LL recovery value, kyphosis flexibility, hSVA, and T 2-T 7, L 4, L 5 vertebral hC 7-VSO ( P>0.05). T 8-L 3 vertebral hC 7-VSO was analyzed for ROC curve, and combined with the area under curve and the comprehensive evaluation of sensitivity and specificity, the best predictive index was hC 7-L 2, the cut-off value was 2.54 cm, the sensitivity was 100%, and the specificity was 60.9%.@*CONCLUSION@#Preoperative back-forward Bending CT localization image can be used to predict the occurrence of PJK after posterior osteotomy and long-segment fusion fixation in adult spinal deformity. If the patient's T 8-L 2 vertebral hC 7-VSO is too large, it indicates a higher risk of postoperative PJK. The best predictive index is hC 7-L 2, and the cut-off value is 2.54 cm.

Characteristics of neuropsychological development in infants with different types of morphological cranial malformation / 中国康复理论与实践

ObjectiveTo compare the neuropsychological development of infants with different types of morphologic cranial deformities. MethodsA total of 954 children aged 0 to 18 months who came to Beijing Children's Hospital from January, 2020 to August, 2021 for cranial measurement and neuropsychological development measurement were selected. They were divided into brachycephaly group, plagiocephaly group, asymmetric brachycephaly group, scaphocephaly group and normal group according to the cranial measurement. The development quotient (DQ) was calculated from Children Neuropsychological Development Scale (0-6). ResultsThere were 449 cases in the normal group, 94 cases in the brachycephaly group, 201 cases in the plagiocephaly group, 82 cases in the asymmetric brachycephaly group and 128 cases in the scaphocephaly group. The detection rate of Developmental Edge and Delay (DQ < 85) for gross motor area was the most in brachycephaly group (60.6%), and it was the most for fine motor (64.6%), language (45.1%), adaption (51.2%) and social behavior areas (48.8%) in the asymmetrical brachycephaly group. The DQ was different among the five groups for all the areas except the language area (F > 14.835, P < 0.001); compared with the normal group, DQ decreased for all the four areas in all the groups except the scaphocephaly group; DQ of the areas of gross motor, fine motor and adaption was more in the plagiocephaly group than in the asymmetric brachycephaly group (P < 0.05), while DQ of the areas of gross motor and fine motor was more in the plagiocephaly group than in the brachycephaly group (P < 0.05). Linear regression analysis showed that, DQ negative linear correlated with the cephalic ratio and cranial vault asymmetry index (|B| > 0.967, P < 0.05). ConclusionAmong four kinds of cranial malformation in infants, the neuropsychological development of the scaphocephaly group is almost normal, and somehow delays for brachycephaly, plagiocephaly and asymmetric brachycephaly, especially in the aspects of gross motor, fine motor, adaption and social behavior. The more serious the cranial deformity, the greater the risk of developmental delay in each functional area.

Clubfoot Deformity Treatment with Ilizarov Apparatus in the Paediatric Population without Corrective Osteotomies and Soft Tissue Release: A Cross-Sectional Study

@#Introduction: Neglected Club Foot deformity is not an uncommon limb anomaly encountered by orthopaedic surgeons. Many treatment methods have been proposed. Ilizarov apparatus is one of the techniques used to correct this deformity. Materials and methods: In this cross-sectional study 47 patients (56 feet) between the ages of 5 and 10 years with clubfoot deformity were treated using the Ilizarov external fixator. Age, sex, type of deformity, and radiographic parameters were measured on foot radiographs. Also, the American Orthopaedic Foot and Ankle Society (AOFAS) score and the Dimeglio classification were recorded for each patient before and after treatment. Results: The treatment was unilateral in 38 patients and bilateral in 9 patients. 39 patients (69.6%) were male, and 17 patients (30.4%) were female with a mean age of 7.86 ± 1.4 years. Plantar angles of ankle flexion and ankle flexion curve increased from 20.12±6.52 and -16.51±8.36 to 25.89±6.44 and 6.19±6.42, respectively. There was also an improvement in the talocalcaneal and tibiocalcaneal angles. Also, the angle between the first metatarsus and the talus in the front and side views improved (P<0.00). Additionally, the mean AOFAS score and Dimeglio classification significantly improved. Three cases were complicated with distal tibial physeal separation that were treated with additional open surgeries. Conclusion: Ilizarov technique without osteotomies and soft tissue release could be considered a less invasive and successful method of treatment for neglected clubfoot deformity in patient five to ten years old that are not good candidate for Ponseti method.

The Clinical Outcomes and Perioperative Care of Neurofibromatosis Type 1 Thoracolumbar Kyphoscoliosis / 罕见病研究

  Objective  To evaluate the clinical outcomes and complications of posterior spinal fusion surgery in the treatment of neurofibromatosis type 1(NF1)thoracolumbar kyphoscoliosis, and to explore the mode of perioperative care for nurses provided to the patients.  Methods  We used the retrospective analysis on the 134 patients with NF1 thoracolumbar kyphoscoliosis admitted to our department from March 2012 to April 2022 and analyzed the clinical outcomes and perioperative complications by using the related statistics. We evaluated the Perioperative care by the nurses in the treatment of NF1 to explore the mode of nursing to the patients with the NF1, by using specific observation points and evaluation indicators.  Results  NF1 kyphoscoliosis patients had poor preoperative nutritional status and lung function. NF1 kyphoscoliosis underwent longer operation time, lost more blood in operation, had higher osteotomy grade and more postoperative complications. All the patients successfully completed the operation in our group. The correction rate of scoliosis was (52.8±22.7)%, and the correction rate of kyphosis was (57.3±34.6)%. 25 patients had complications but no such serious complications as nerve damage.  Conclusions  The practice of the perioperative nursing to NF1 type scoliosis patients facilitates the shortening of the recovery period, the prevention or timely detection of complications, and improvement of the therapeutic effect.

Progress in the Research of the Spinal Deformity Related to Marfan Syndrome / 罕见病研究

Marfan syndrome(MFS) is an autosomal dominant systemic connective tissue disease. The incidence rate of MFS is about 2-3 per 10 000. Main cause of MFS is FBN1 gene mutation. About 2/3 of MFS patients have spinal deformities, showing symptoms of scoliosis, thoracic lordosis and lumbar kyphosis, severe spondylolisthesis, dural dilatation and pedicle dystrophy. MFS scoliosis develops with age and may continue even after bone maturation. Conservative treatments such as brace are usually ineffective. Surgical treatment of main curve > 40°-45 °is recommended, but due to the special anatomical structure of MFS patients, such surgical complications as dural leakage, failure of internal fixation and revision surgery are not uncommon.

Perioperative allogenic blood transfusion for single disease in patients undergoing spinal deformity correction surgery:a longitudinal observational study / 中国输血杂志

【Objective】 To analyze the profile of perioperative allogenic blood transfusion for single disease in patients who underwent spinal deformity correction surgery and risk factors of the blood transfusion, in order to provide reference for clinical decision making. 【Methods】 Clinical data from medical record homepage of 292 patients who underwent elective spinal deformity correction surgery at Chengdu Third People′s Hospital from January 2015 to December 2017 were retrospectively analyzed. Statistical analysis of the transfusion profile of allogeneic blood based on the type of single disease in patients undergoing correction surgery was performed. Multiple factor linear regression analysis was used to identify the risk factors of perioperative allogenic blood transfusion in patients with spinal deformity correction surgery. Hospital length of stay and discharge status were compared between transfusion group and non-transfusion group using Mann Whitney U test and chi-square test respectively. 【Results】 The year prevalence of perioperative allogeneic blood transfusion from 2015 to 2017 were 90.38%(47/52), 93.62%(44/47) and 81.35%(157/193), respectively. The prevalence of perioperative allogeneic blood transfusion in patients with kyphotic deformity in ankylosing spondylitis, kyphosis, adolescent idiopathic scoliosis, scoliosis and spinal stenosis were 89.08%(106/119)、79.49%(62/78)、95.24%(40/42)、84.38%(27/32) and 61.90%(13/21), respectively. Multivariate linear regression analysis showed that the regression coefficients for age and osteotomy were -0.060 (P<0.05) and 2.060 (P<0.05), respectively. Compared with non-transfusion group, the transfusion group had longer hospital length of stay (P<0.05). 【Conclusion】 Perioperative allogeneic blood transfusion in patients with spinal deformity correction surgery is closely related to the type of single disease. Age is a protective factor for perioperative allogeneic blood transfusion, while osteotomy is a risk factor for perioperative allogeneic blood transfusion in patients undergoing the spinal deformity correction surgery. Perioperative blood transfusion can also prolong the hospital length of stay of the patients.

Supplementing rehabilitation training with botulinum neurotoxin improves outcomes for Parkinson′s disease patients with striatal foot deformity / 中华物理医学与康复杂志

Objective:To observe any therapeutic effect of combining botulinum toxin type A (BTX-A) with rehabilitation training in treating Parkinson′s disease (PD) patients with striatal foot deformity (SFD).Methods:A total of 68 PD patients with SFD were randomly divided into a control group and a treatment group. Both groups were given routine medication with pramipexole and dopamine receptor agonists and received lower limb rehabilitation training, including passive activity training, strength training and walking training. The treatment group was additionally injected with BTX-A. Sciatic pain was quantified using a visual analogue scale. The Unified Parkinson′s Disease Rating Scale-lower limb motor lower limb motor function (UPDRS-LLM) scale, the Berg balance scale and the modified Barthel index were applied to test all of the participants before the experiment and on the 7th, 14th and 30th day of the treatment.Results:The average scores of the control group on all of measures at were significantly better than those of the control group at the same time points, and by the 14th and 30th day had improved significantly compared with those before treatment.Conclusion:Supplementing rehabilitation training with BTX-A can significantly improve foot deformity and relieve the muscle tension and spastic pain of PD patients with SFD, promoting the motor functioning of their lower limbs, their balance and their performance in the activities of daily living.

Intrathecal delivery of nusinersen using the Ommaya reservoir in a spinal muscular atrophy type 2 patient: a case report / 中华神经科杂志

Ommaya reservoir implantation is generally used in the treatment of hydrocephalus and intraventricular drug administration. Ommaya reservoir implantation in the subarachnoid space of the spinal cord for the intrathecal drug administration has not been carried out in China, and only several reports can be retrieved from PubMed. About 60%-90% of untreated patients with spinal muscular atrophy type 2 (SMA2) who survive to adulthood often have complex scoliosis and joint deformities. Nusinersen is an effective drug for the treatment of SMA2. And the route of administration is intrathecal injection, which is difficult for patients with severe scoliosis. This article summarizes the process of Ommaya reservoir implantation and postoperative drug administration in a patient with complex scoliosis type SMA2, which provides a new method for clinical treatment of this disease.

Early curative effects of robot-assisted total knee arthroplasty for valgus knee / 中华创伤骨科杂志

Objective:To investigate the early curative effects of robot-assisted total knee arthroplasty (TKA) in the treatment of valgus knee.Methods:A retrospective study was conducted to analyze the data of 40 patients with valgus knee who had been treated by TKA at Department of Orthopaedics, The 920th Hospital of Joint Logistics Support Force of Chinese People's Liberation Army from January to December 2021. The patients were divided into 2 groups according to whether a robot had been used or not for TKA. In the observation group of 15 cases for which TKA was assisted by a robot, there were 4 males and 11 females with an age of (65.5±6.2) years, and the disease course was 42 (36, 54) months; in the control group of 25 cases for which conventional TKA was performed, there were 8 males and 17 females with an age of (65.8±7.5) years, and the disease course was 42 (36, 60) months. Surgical time, hemoglobin decrease, and knee joint range of motion, American Knee Society Score (KSS), hip-knee-ankle angle (HKA), lateral distal femoral angle (LDFA), and medial proximal tibial angle (MPTA) at 12 months after surgery were compared between the 2 groups.Results:There was no significant difference in the preoperative general data between the 2 groups, indicating comparability ( P>0.05). The surgical time in the observation group was (148.0±21.2) min, significantly longer than that in the control group [(115.2±7.1) min], and the hemoglobin decreased by (11.8±1.1) g/L in the observation group, significantly less than that in the control group [(18.1±1.8) g/L] ( P<0.05). The observation group and the control group were followed up for 13 (13, 14) and 13 (13, 14) months after surgery, respectively, showing no statistically significant difference ( P>0.05). At 12 months after surgery, the KSS knee score, KSS functional score, and knee range of motion in the observation group were (86.1±4.6) points, (86.9±3.1) points, and 115.7°±5.0°, significantly larger than those in the control group [(82.2±3.5) points, (82.8±0.9) points, and 108.2°±5.0°] ( P<0.05). Reexamination of full-length radiographs of both lower limbs in all patients showed good positions of the prostheses and no such adverse events as loosening or sinking at 12 months after surgery. The HKA (178.5°±1.2°) and LDFA (89.1°±0.7°) at 12 months after surgery in the observation group were significantly larger than those in the control group (176.6°±1.5°, 88.2°±8.2°) ( P<0.05); there was no statistically significant difference in MPTA between the 2 groups ( P>0.05). Conclusions:In the treatment of valgus knee, robot-assisted TKA can correct joint deformity, and achieve precise osteotomy and functional alignment of lower limbs, leading to better early curative effects than conventional TKA.

Esclerodermia localizada en escolar de 10 años / Localized scleroderma in a 10-year-old schoolgirl

Dentro del grupo de enfermedades reumáticas la esclerodermia es una de las de menor frecuencia de presentación, por lo que muchos autores la consideran una enfermedad rara. Aunque afecta predominantemente a pacientes adultos, en ocasiones se presenta en edades pediátricas y sus formas localizadas son las manifestaciones más frecuentes a estas edades. El objetivo del presente reporte es presentar el caso de una escolar de 10 años de edad, con un cuadro de lesión en la piel de 3 años de evolución a la cual se le diagnostica, mediante las características clínicas y los resultados de estudios anatomopatológicos una esclerodermia localizada profunda. En la actualidad la paciente se mantiene en régimen de seguimiento multidisciplinario. Este reporte de caso es importante para compartir con la comunidad médica los elementos básicos relacionados con el diagnóstico y tratamiento de esta enfermedad, como alternativa a la reducción de las complicaciones que genera(AU)

Within the group of rheumatic diseases, scleroderma is one of those with the lowest frequency of presentation; being considered a rare disease by many authors. Although it has a predominance of affectation in adult patients, it sometimes occurs in pediatric ages, its localized forms being the most frequent forms of presentation. The objective of this report is to present the case of a 10-year-old schoolgirl, with a 3-year history of skin lesions, which was diagnosed, through clinical characteristics and results of pathological studies, as deep localized scleroderma. The case report is considered important to share with the medical community the basic elements related to the diagnosis and treatment of this disease, as an alternative to reducing the complications it generates(AU)

Resultados de la técnica septum bisagra en desviación de la punta nasal

Introducción: La desviación de la punta nasal suele producir alteración estética y funcional nasal. Generalmente, asocia alteraciones morfológicas de los cartílagos alares, además de dismorfia del cartílago septal. Objetivo: Presentar una técnica quirúrgica alternativa para el tratamiento de la laterorrinia en tercio inferior nasal y sus resultados. Material y Método: Se trataron 27 pacientes con una laterorrinia en punta nasal con la técnica del septum bisagra, incluyendo la colocación de uno o dos injertos de expansión. Describimos el protocolo de anamnesis y exploración que seguimos en estos pacientes y la descripción detallada de la técnica quirúrgica. Resultados: En 19 pacientes se utilizó un injerto de expansión unilateral para completar la técnica y en ocho de forma bilateral. Se obtuvo una puntuación media en la escala visual analógica (EVA) de ventilación de 8,3, con una mejoría de seis puntos, y en la EVA de aspecto estético de 8,1, mejorando en 4,6 puntos. Conclusión: la utilización de la técnica del septum bisagra es beneficiosa para el tratamiento de la desviación de la punta nasal, tanto a nivel funcional como estético. Presenta ciertas diferencias frente a otras técnicas descritas. La indicación se debe adecuar a cada paciente de manera individualizada.

Introduction: Deviation of nasal tip usually produces aesthetic disturbance and nasal ventilation decrease. It is usually associated with alar cartilages morphologic disturbances and dysmorphia of the septal cartilage. Aim: To present an alternative surgical technique for treating laterorrhinia in the nasal inferior third and its outcomes. Material and Method: 27 patients with nasal tip laterorrhinia were treated with the hinge septum technique, including the placement of one or two spreader grafts. We describe the anamnesis and exploration protocol that we carried out in these patients and a detailed description of the surgical procedure. Results: Unilateral spreader graft was used in 19 patients to complete the technique, and bilateral spreader graft was used in eight. The average score in visual analogue scale (VAS) related to nasal flow was 8.3, which means an improvement of six points, and in VAS related to aesthetic appearance the score was 8.1, improving 4.6 points. Conclusion: The use of the hinge septum technique is useful to treat the deviation of the nasal tip in a functional and aesthetic way. There are several differences compared to other described techniques. The indication has to be suitable for each patient individually.

Hunchback with “S” Shape Spine: Cadaveric Case series

Introduction: Kyphosis is a sharp posterior angulation due to localized collapse or wedging of one or more vertebrae and scoliosis is the lateral curvature of the spine. These may occur due to a congenital defect, fracture, and sometimes pathological or spinal tuberculosis. Materials and Methods: During routine cadaver dissection, four cases of kyphoscoliosis were identified. In the first case, a protrusion on the posterior surface of the trunk of the cadaver at L1 level was observed in a 60- year-old male. The lungs were collapsed and adherent with the pleura and thoracic wall. The posterior wall of the stomach was adherent to the pancreas and a depression was noted on the anterior surface of abdominal aorta. The T12 and L1 vertebrae were fused, and the spine was deviated to the left side. The anterior aspect of the body of L1 vertebrae was marginally eroded. The second case of kyphoscoliosis was from 80-year-old female cadaver. The descending and abdominal aorta had an abnormal course along the vertebral column, third and fourth cases were observed with slight protrusion and scoliosis but not much changes in the vertebral column. Results: The cause of the deformity was confirmed by pathological examination of the tissues showed inter vertebral disc degeneration with calcification and ossification and osteopenia. The underlying etiology can be tuberculosis, injuries, or infections. Conclusion: Severe kyphoscoliosis can affect wide range of systems in the body. It will affect not only the respiratory system also pressure on great vessels as a consequence of bony deformity.

Hemifacial Hypertrophy – Report of 2 Cases

Hemifacial hyperplasia (HH) is a rare congenital condition involving enlargement of one or more tissues of the face. The treatment is surgically challenging and requires expertise. This manuscript aims to report two similar appearing HH but warranting different surgical treatment. A 19?year?old female and a 14?year?old boy presented with right facial asymmetry since birth and sought correction of the same. Surgical treatment was planned. Based on clinical history, diagnosis and imaging, HH was diagnosed. The first case was entirely a soft tissue abnormality that was treated with debulking while the second case had involvement of facial bones, necessitating surgical recontouring. The facial asymmetry was addressed. Healing was uneventful. Though the aesthetical concern and appearance of the two cases of HH were same, the treatment vastly differed. This was based on the source of asymmetry. Proper diagnosis and informed decision are a key for successful surgical outcome.

Effect of varying dietary crude protein and metabolizable energy levels on leg abnormalities and performance in broiler chickens

Background: Nutrient restriction is a common strategy to prevent metabolic disorders in fast growing broiler chickens, but limited information is available regarding the impact of diets with low protein and energy on leg abnormalities Objective: Two experiments were conducted to evaluate the effect of varying crude protein (CP) and metabolizable energy (ME) levels on gastrocnemius tendon (GTeBS) and tibia breaking strength (TiBS), gait score (GS) and valgus/varus angulation (VAng). Methods: In Experiment 1, eight-d-old Ross 308 broilers (n=90) were randomly assigned into three treatments: 13/2,900 (13% CP and 2,900 kcal ME kg-1 of diet), 17/3,000 (17% CP and 3,000 kcal ME kg-1 of diet), and 21/3,025 or control (21% CP and 3,025 kcal ME kg-1 of diet). In Experiment 2, six-d-old Ross 308 chickens (n=192) were randomly distributed into two treatments: 16/3,000; 16% CP and 3,000 kcal ME kg-1 of diet and 21/3,000 or control; 21% CP and 3,000 kcal ME kg-1 of diet. In both experiments data were analyzed as one-way ANOVA. Results: In Experiment 1, broilers in the 17/3,000 and control treatments had similar (p>0.05) GTeBS (202 and 224 N, respectively), and TiBS (338 and 332 N, respectively). Birds in the 13/2,900 treatment showed higher GS (lower walking ability; 80% of birds with score >3), and greater VAng (53% of birds with score >2) than broilers in the control treatment (0% of birds with GS score >3 and 0% of birds with VAng score >2). Experiment 2, broilers in the 16/3,000 showed similar VAng, GS and TiBS than birds in the control treatment. However, birds in the 16/3,000 treatment showed lower (-26%) GTeBS than birds in the control treatment (p<0.05). Conclusions: Feeding broilers with 16% CP and 3,000 kcal ME kg-1 of diet did not affect tibia breaking strength, gait score and valgus/varus angulation. A diet containing 16% CP or less and 2,900-3,000 kcal ME kg-1 reduces tendon and tibia breaking strength.

Antecedentes: La restricción de nutrientes es una estrategia común para prevenir trastornos metabólicos en pollos de engorde, sin embargo, se dispone de información limitada sobre el impacto de las dietas bajas en proteína y energía en las anomalías de piernas. Objetivo: Se llevaron a cabo dos experimentos para evaluar el efecto de niveles variables de proteína cruda (PC) y energía metabolizable (EM) en la fuerza de rotura del tendón del gastrocnemio (GTeBS) y tibia (TiBS), la evaluación de marcha (GS) y la angulación en valgus/varus (VAng). Métodos: En el Experimento 1, pollos de engorde Ross 308 de ocho días de edad (n = 90) se asignaron al azar en tres tratamientos: 13/2.900; 13% PC y 2.900 kcal ME kg-1 de dieta, 17/3.000; 17% PC y 3.000 kcal ME kg-1 de dieta y 21/3.025 o control; 21% PC y 3.025 kcal ME kg-1 de dieta. En el Experimento 2, pollos Ross 308 de seis días de edad (n = 192) se distribuyeron aleatoriamente en dos tratamientos: 16/3.000; 16% PC y 3.000 kcal ME kg-1 de dieta y 21/3.000 o control; 21% PC y 3.000 kcal ME kg-1 de dieta. En ambos experimentos los datos se analizaron como ANOVA de una vía. Resultados: En el Experimento 1, los pollos de los tratamientos 17/3.000 y control tuvieron GTeBS (202 y 224 N, respectivamente) y TiBS (338 y 332 N, respectivamente) similares (p>0,05). Las aves del tratamiento 13/2.900 mostraron mayor GS (menor capacidad para caminar; 80% de las aves con puntaje >3) y mayor VAng (53% de las aves con puntaje >2) que los pollos del tratamiento control (0% de aves con un puntaje GS >3 y 0% de aves con un puntaje VAng >2). En el Experimento 2, los pollos del 16/3.000 mostraron VAng, GS y TiBS similares a los de las aves del tratamiento control. Sin embargo, las aves del tratamiento 16/3.000 mostraron menor (-26%) GTeBS que las aves control (p<0,05). Conclusiones: La alimentación de pollos de engorde con 16% PC y 3.000 kcal EM kg-1 de dieta no afecta la resistencia a la ruptura de la tibia, la marcha y la angulación valgus/varus. La dieta formulada con 16% de PC o menos y 2.900-3.000 kcal de EM kg-1 reduce la resistencia a la ruptura del tendón y de la tibia.

Antecedentes: A restrição de nutrientes é uma estratégia comum para prevenir distúrbios metabólicos em frangos de corte, no entanto, há informações limitadas disponíveis sobre o impacto de dietas de baixa proteína e baixa energia nas anormalidades de pernas destas aves. Objetivo: Dois experimentos foram realizados para avaliar o efeito de diferentes níveis de proteína bruta (PB) e energia metabolizável (EM) em força de ruptura do tendão do músculo gastrocnêmio (GTeBS), forca de ruptura da tíbia (TiBS), gait score (GS) e angulação em valgus/varus (VAng). Métodos: Experimento 1, foram utilizados 308 frangos de corte machos da linhagem Ross com oito dias de idade (n = 90) distribuidos aleatoriamente em três tratamentos: dieta controle com 21% PB e 3.025 kcal EM kg-1 13/2.900; dieta con 13% PB e 2.900 kcal EM kg-1, y 17% PB e 3.000 kcal EM kg-1 . Experimento 2, foram utilizados 308 frangos de corte da linhagem Ross com seis dias de idade (n = 192) distribuídos aleatoriamente em dois tratamentos: dieta controle con 21% PB e 3.000 kcal EM kg-1 e dieta com 16% PB e 3.000 kcal EM kg-1 . Em ambos os experimentos, os dados foram submetidos a ANOVA. Resultados: Experimento 1, os frangos de corte submetidos ao dos tratamentos 17/3.000 e controle nao apresentaram diferencas significativas (p>0,05) para as variáveis GteBS (202 y 224 N, respectivamente) e TiBS (338 y 332 N, respectivamente). Os frangos submetidos ao 13/2.900 apresentaram maior frequência de GS (menor capacidade de locomocao; 80% das aves com pontuação>3) e maior VAng (53% das aves com pontuação>2) do que os frangos do tratamento controle (0% das aves com pontuação GS>3 e 0% das aves com pontuação VAng>2). Experimento 2, os frangos do 16/3.000 apresentaram VAng, GS e TiBS semelhantes aos das aves do tratamento controle. Entretanto, as aves do tratamento 16/3.000 apresentaram GTeBS mais baixo (-26%) em comparacao as aves do tratamento de controle (p<0,05). Conclusões: A dieta com 16% PB e 3.000 kcal EM kg-1 não afetou a forca de ruptura da tíbia, gaite score marcha e angulação em valgus/varus de frangos de corte. Uma dieta com níveis de 16% de PB ou menos e 2.900-3.000 kcal EM kg-1 reduziu a resistência à ruptura do tendão e a resistência de tibia de frangos de corte.

Hemimelia tibial: Presentación de un caso / Tibial Hemimelia

Introducción: La hemimelia tibial o hemimelia paraxial longitudinal tibial, es una deficiencia congénita de la tibia. Esta deficiencia de los miembros inferiores longitudinal tibial, es muy rara y su frecuencia está en el orden de 1: 1 000 000 de niños nacidos vivos. Objetivo: Presentar un caso de hemimelia tibial diagnosticado por medio del cuadro clínico y radiografías y tratado quirúrgicamente. Presentación del caso: Paciente masculino de dos horas de nacido, atendido por presentar malformación congénita a nivel de la pierna derecha que se presentaba acortada con una prominencia dura a nivel proximal y el pie con deformidad marcada en supinación, aducción y rotación interna. Se realizó examen físico exhaustivo de la extremidad afecta y se constató el acortamiento evidente de la misma. Se indicó radiografía anteroposterior y lateral de la pierna y se observó que el segmento proximal de la tibia y el peroné estaban bien, pero con implantación alta, por lo que se diagnosticó una hemimelia tibial tipo II de Jones. Luego del alta el niño recibió seguimiento por consulta de Genética y el servicio de Ortopedia donde se decidió someterlo a un primer tiempo quirúrgico a los 6 meses de edad, mediante tibialización del peroné. A los 10 meses se realizó un segundo tiempo quirúrgico para centrar el astrágalo al peroné. Conclusiones: La hemimelia tibial se considera un diagnóstico poco frecuente en nuestro medio. No existe prevención conocida. El tratamiento es complejo y altamente especializado, y en algunos casos requiere la amputación temprana del miembro afectado para adaptar al paciente al uso de prótesis(AU)

Introduction: Tibial hemimelia or tibial longitudinal paraxial hemimelia is a congenital deficiency of the tibia. This tibial longitudinal lower limb deficiency is very rare and its frequency is in the order of 1: 1,000,000 live births. Objective: To report a case of tibial hemimelia diagnosed through the clinical condition and radiographs and treated surgically. Case report: We report the case of a two-hour-old male patient, treated for a congenital malformation at the level of the right leg that was shortened with a hard prominence at the proximal level and the foot with marked deformity in supination, adduction and internal rotation. An exhaustive physical examination of the affected limb was carried out and its evident shortening was confirmed. Anteroposterior and lateral X-rays of the leg were indicated and it revealed that the proximal segment of the tibia and fibula were fine, but with high implantation, for which a Jones type II tibial hemimelia was diagnosed. After discharge, the child was followed up by the Genetics consultation and the Orthopedics service, where it was decided to undergo a first stage surgery at 6 months of age, by means of tibialization of the fibula. At 10 months, a second surgical time was performed to center the talus to the fibula. Conclusions: Tibial hemimelia is considered a rare diagnosis in our setting. There is no known prevention. Treatment is complex and highly specialized, and in some cases requires early amputation of the affected limb to adapt the patient to the use of a prosthesis(AU)