Surgical treatment of pycnodysostosis associated with pathological tibial fracture : Case report and review of the literature.

BACKGROUND: Pycnodysostosis is a rare autosomal recessive lysosomal disorder of bone characterized by diffuse skeletal condensation with thickening of the cortex and narrowing of the medullary canal. CASE PRESENTATION: We present the case of a 4-year-old girl diagnosed with pycnodysostosis and associated pathological tibial fracture. The tibia had an absence of medullary canal. Surgery included reduction and reaming of the canal with placement of a 5 mm diameter telescopic growing nail. CONCLUSION: The presentation of pycnodysostosis as tibial fracture is rare and there is limited literature on its management. We showed its approach focusing mainly on the management of the absent medullary canal.

Treatment of hallux valgus in children and adolescents.

Juvenile hallux valgus is one of the most common pathologies of the forefoot in children and adolescents. Nevertheless, its treatment is still controversial. This deformity has some distinct anatomical features, particularly a lateral tilt of the articular facet of the head of the first metatarsal and congenital metatarsus adductus, which often occur in combination. Some mediocre surgical treatment results can be explained by the lack of correction of all these factors when we approach the problem as we would in adults. A double osteotomy to correct the DMAA and varus of the first metatarsal is a good solution in most cases with satisfactory functional outcomes. A percutaneous approach seems efficient in the pediatric population, given that the periosteum and growth plates, which are very active in younger children, help the osteotomies to heal and remodel. Guided growth surgery - using this approach is a viable alternative in this age bracket. Finally, minimally invasive surgery for juvenile hallux valgus allows another surgery to be done on minimally or undamaged tissues if needed later on.

Arthroscopic Calcaneonavicular Coalition and Too-long Anterior Process of the Calcaneus Resection in Children.

Persistent symptomatic calcaneonavicular coalition (CNC) and too-long anterior process of the calcaneus (TLAP) are congenital disorders that can benefit from surgical treatment. The arthroscopic technique for CNC and TLAP resection has previously been described. The aim of this prospective study was to describe outcomes following arthroscopic resection of 12 (38.71%) CNC and 19 (61.29%) TLAP cases in 30 consecutive pediatric patients treated between July 2009 and March 2013. There mean age was 12.4 (range 10 to 15) years, and the mean follow-up was 55.2 (range 24 to 79) months. Radiographs, American Orthopaedic Foot and Ankle Society (AOFAS) Ankle-Hindfoot Scores, AOFAS pain scores, and patient satisfaction were assessed. All final postoperative imaging scans revealed complete synostosis resection without recurrence. The mean overall AOFAS Ankle-Hindfoot Score increased from 78.87 (95% confidence interval [CI] 76.74 to 81.01) to 93.06 (95% CI 91.10 to 95.03) (p < .001). All patients showed pain reduction after surgery; even 1 patient (3.23%) who initially developed complex regional pain syndrome eventually had a successful outcome. The mean AOFAS pain score increased from 23.87 (95% CI 22.05 to 25.69) to 34.84 (95% CI 32.97 to 36.70) (p < .001). All patients were either satisfied (n = 9 [30%]) or very satisfied (n = 21 [70%]) with the intervention at the final follow-up. Although both arthroscopic CNC and TLAP resection are demanding techniques, they allow for precise coalition resection through a less invasive approach, which may ultimately lead to faster recovery and improved outcomes.

Bone nonunion management in children with a vascularized tibial periosteal graft.

BACKGROUND: Vascularized periosteal graft have demonstrated a tremendous bone healing capacity in children. The objective is to report outcomes on the use of vascularized tibial periosteal graft (VTPG) during bone reconstruction in a series of children with complex bone healing problems. PATIENTS AND METHODS: Cases were collected retrospectively since May 2013 to May 2019, excluding cases of congenital pseudarthrosis of the tibia. Mean age at surgery was 12.8 (range 11-18) years. Indications included treatment of recalcitrant bone nonunion and the prevention of bone allograft-host junction nonunion in seven and three patients, respectively. The periosteal flap, based on the anterior tibial vessels, was harvested as a free flap in six instances and as a pedicled flap in four. RESULTS: Mean follow-up was 25.2 months (range 8-36). The flap showed a 13.6 cm (range 9-16) and mean width 3.4 cm (range 2.7-3.9). Early bone union was achieved, initially through periosteal callus, followed by cortical union at mean times of 2 and 4 months, respectively, in nine cases. The flap was not successful in a patient with severe comorbidities. No donor site complications were registered. CONCLUSIONS: VTPG was fast and high effective for the treatment complex bone nonunion or the prevention of allograft nonunion in children.

Growth hormone effects on childrens height with achondroplasia and hypochondroplasia: a systematic review and metanalysis

Introduction: Achondroplasia (Ach) is the most frequent cause of dwarfism. The first therapeutic strategy offered to patients with Ach was. However, GH has played un important role in Ach and Hypochondroplasia (Hch), despite short-term and long-term effects. Purpose: The aim of this systematic review and meta-analysis was to assess the efficacy of GH in the height of patients with Ach and Hch in the short and long term. Methods: 12 studies were included selected from the Pubmed database (3 Randomized Clinical trials (RCTs) and 9 prospective studies) from 1993 to 2014. Comparing high and low doses of GH. The systematic review included 9 prospective studies and the high-dose GH arm of the 3 RCTs. Inclusion criteria was focused on paediatric patients with Ach and Hch treated with GH. Demographic variables were collected including age, gender, dose, height and follow-up. The height variables included height increase and height velocity. Finally, 363 patients with Ach and 41 patients with Hcb were included. A was performed with a follow-up from one to 3 years. Results: In patients with Ach the average height velocity at one, two and three years were 2.65, 1.07 and -0.87 cm/years respectively (p<0.05). The RCTs showed a significant increase in height velocity in patients treated with high dose of GH (MD= 1.38, 95% CI: 0.68-2.07, p=0.0001, I2=0%) . Height at one year increased 0.61 cm. The RCTs did not show significant differences (MD 0.11, 95% CI: 0.17-0.39, p=0.44, I2 = 0%). Finally, patients with Hch increased height velocity 4 cm/year at the first year (p<0.05). Conclusion: GH treatment is beneficial in the shor-term height of children with Ach and Hch. GH effect on different ages and subgroups is unknown, as well as its possible long--term consequences

Non-invasive expandable prosthesis in musculoskeletal oncology paediatric patients for the distal and proximal femur. First results.

PURPOSE: The non-invasive expandable prosthesis for skeletally immature patients is used after limb salvage surgery following tumor resection. The aim of the study was to assess the effectiveness of this treatment. METHODS: Seven paediatric patients with femoral tumors had resection and limb salvage with an uncemented non-invasive growing prosthesis. Mean age at the time of surgery was 9.8 (range 8-12) years. There were six distal femur osteosarcomas and one proximal femur Ewing sarcoma. Six total knee prosthesis were implanted at the time of primary tumor resection and one bipolar hip prosthesis was a revision from a failed osteoarticular hip allograft. Functional outcomes and emotional acceptance were assessed using the MSTS score. RESULTS: The mean follow-up was 65.3 months (range 29-91) months. Two patients died of pulmonary metastasis and there was no local recurrence. The mean femoral resection was 18 cm (range 17-19) on the knee, and 24 cm on the hip. Mean total expansion was 36.4 mm (range 12.3-63.5). The mean MSTS score after rehabilitation was 26.3 (range 21-29). There was one lengthening device failure, one late infection and one patient who required iliofemoral bypass grafting surgery for a pelvic metastasis. No local recurrence occurred. CONCLUSIONS: The non-invasive expandable prosthesis reduces the final limb-length discrepancy in growing patients with an acceptable function and appears to have an advantage as compared to invasive expandable prostheses which require multiple surgical procedures, but the complications rate is still high.

Arthroscopic Talocalcaneal Coalition Resection in Children.

PURPOSE: To present the technique and outcomes of arthroscopic talocalcaneal coalition (TCC) resection in pediatric patients. METHODS: We performed a prospective study of 16 consecutive feet with persistent symptomatic TCCs in 15 children. The mean age was 11.8 years (range, 8 to 15 years), and the mean follow-up period was 28 months (range, 12 to 44 months). A posterior arthroscopic TCC resection was performed. The plantar footprint, subtalar motion, pain, and the American Orthopaedic Foot & Ankle Society Ankle-Hindfoot scale score were evaluated preoperatively and postoperatively. Preoperative computed tomography (CT) scans were used to classify the coalition according to the Rozansky classification, to measure the percentage of involvement of the surface area, and to determine the degree of hindfoot valgus. Postoperative CT scans at 1 year (n = 15) and 3 years (n = 5) were used to assess recurrences. Patient satisfaction was also evaluated. RESULTS: The TCC distribution according to the Rozansky classification was type I in 7 cases, type II in 3, type III in 3, and type IV in 3. In all cases the arthroscopic approach enabled complete coalition resection. All patients increased by at least 1 stage in the footprint classification and showed clinical subtalar mobility after surgery. All patients showed a statistically significant improvement in pain after surgery except for 1 patient in whom complex regional pain syndrome developed (P < .001). The mean American Orthopaedic Foot & Ankle Society score was 56.8 (range, 45 to 62) preoperatively versus 90.9 (range, 36 to 100) postoperatively, showing a statistically significant increase (P < .001). Preoperative CT scans showed that all TCCs involved the medial subtalar joint facet, with mean involvement of 40.8% of the articular surface. All postoperative CT scans showed complete synostosis resections with no recurrences at final follow-up. At final follow-up, all patients were either satisfied (n = 4 [27%]) or extremely satisfied (n = 10 [67%]) with the outcome, except the 1 patient (7%) in whom complex regional pain syndrome developed. CONCLUSIONS: Arthroscopic TCC resection provides good outcomes (symptom relief and restoration of subtalar motion), with no recurrence of the coalition. LEVEL OF EVIDENCE: Level IV, therapeutic case series.

Resultado a largo plazo de las fusiones largas espinopelvicas en escoliosis: importancia de la edad, la capacidad de marcha y el tipo de fijación ilíaca / Resultado a longo prazo das grandes artrodeses espinopélvicas em escoliose: importância da idade, da capacidade de marcha e do tipo de fixação ilíaca / Long-term result of major spinopelvic arthrodeses in scoliosis: importance of age, walking ability and type of iliac fixation

OBJETIVO: Conocer los factores que influyen en los fracasos instrumentales lumbosacros después de fusiones espinopélvicas largas. MÉTODO: Estudio retrospectivo de pacientes diagnosticados con escoliosis, tratados quirúrgicamente por vía posterior, realizándo se fusión espinopélvica de T2 o T3 a ilíaco, utilizando tornillos pediculares e ilíacos. Se analizaron los fracasos instrumentales y su asociación con diferentes parámetros clínicos y radiológicos. RESULTADOS: Se presentan 44 pacientes con edad promedio de 24 años, con diferentes etiologías. El valor promedio preoperatorio de la curva mayor era de 74,2º, y en la revisión final la corrección promedio fue 67%. El desequilibrio anteroposterior y la inclinación pélvica, la cifosis torácica, la lordosis lumbar y el desequilibrio lateral mejoraron significativamente en la revisión final. Hubo 41% de fracasos de instrumentación, todos a nivel lumbosacro. Se encontró asociación significativa con más fracasos instrumentales en mayores de 17 años y en los que tenían deambulación autónoma. En 24 pacientes, se utilizó un solo tornillo ilíaco bilateralmente y en 20 pacientes, dos o más. Los dos grupos tuvieron una incidencia similar de fracasos. En el grupo de dos o más tornillos solo existieron roturas de barras sin desanclajes, ni lisis. El uso de implantes intersomáticos de L3 a S1 o cerclajes sublaminares con una tercera barra disminuyó la incidencia de fracasos. CONCLUSIONES: En esta serie se presentaron un 41% de fracasos instrumentales, todos localizados a nivel lumbosacro. Los pacientes con capacidad de deambulación autónoma y mayores de 17 años presentaron significativamente más fallas instrumentales. En los que se realizó fusión intersomática L3-S1 o una tercera barra con cerclajes sublaminares, disminuyó la incidencia.

OBJETIVO: Compreender os fatores que influenciam as falhas instrumentais lombossacrais depois de fusões espinopélvicas grandes. MÉTODOS: Estudo retrospectivo de pacientes com diagnóstico de escoliose, tratados cirurgicamente com fusão espinopélvica por via posterior, realizada de T2 ou T3 até o ilíaco, com parafusos pediculares e ilíacos. As falhas instrumentais foram analisadas, além de sua associação com diferentes parâmetros clínicos e radiológicos. RESULTADOS: Apresentamos 44 pacientes com média de idade de 24 anos, com diferentes etiologias. O valor médio da curva pré-operatória maior foi 74,2 graus, e na revisão final foi de 67%. O desequilíbrio anteroposterior e a inclinação pélvica, a cifose torácica, a lordose lombar e o desequilíbrio lateral melhoraram significantemente na revisão final. Houve falhas de instrumentação de 41%, todas na região lombossacral. Verificou-se associação significante com mais falhas instrumentais em pacientes com mais de 17 anos e nos que tinham deambulação independente. Em 24 pacientes, utilizou-se um único parafuso ilíaco bilateral e em 20 pacientes, foram usados dois ou mais. Os dois grupos tiveram incidência de falhas semelhante. No grupo de dois ou mais parafusos ocorreram apenas quebra de hastes, sem perda da ancoragem ou lise. Os implantes intersomáticos de L3 a S1 ou amarrias sublaminares com uma terceira haste diminuíram a incidência de fracassos. CONCLUSÕES: Nesta série ocorreram 41% de falhas instrumentais, todas localizadas na região lombossacral. Os pacientes com capacidade de marcha independente e com mais de 17 anos tiveram significantemente mais falhas instrumentais. Nos pacientes submetidos à fusão intersomática de L3-S1 ou com uma terceira haste com amarrias sublaminares, a incidência foi reduzida.

OBJECTIVE: To determine the factors that influence lumbosacral instrumentation failures following spino-pelvic fusions. METHODS: A retrospective study of patients diagnosed with scoliosis who underwent spinopelvic fusion via posterior, from T2 or T3 proximally to iliac crest, using pedicle and iliac screws. Instrumentation failures were analyzed, and the association of this complication with different parameters was studied. RESULTS: We present 44 patients, with an average age of 24 years, with different etiologies. The mean value of the largest preoperative major curve was 74.2°, and in the final review, it was 67%. The anterior-posterior imbalance, pelvic tilt, thoracic kyphosis, lumbar lordosis and lateral imbalance were significantly improved in the final review. There were instrumentation failures in 41% cases, all at the lumbosacral level. A significant association was found between increased instrumentation failures in patients over 17 years and in patients with independent walking ability. In 24 patients, a bilateral single iliac screw was used and in 20 patients, two or more screws were used. Both groups had a similar incidence of failures. In the group with two or more screws, only rod breakages occurred, without detachment or screw lysis. There was lower instrumentation failure incidence in the patients who underwent L3-S1 interbody fusion or a third rod attached with sublaminar compression hooks. CONCLUSIONS: This series had 41% instrumentation failures, all located at lumbosacral level. There were significant more instrumentation failures in patients with independent walking ability and those aged over 17 years. There was lower instrumentation failure incidence in the patients who underwent L3-S1 interbody fusion or a third rod attached with sublaminar compression hooks.

Anthropometry and body composition profile of girls with nonsurgically treated adolescent idiopathic scoliosis.

STUDY DESIGN: Cross-sectional study with level III of evidence. OBJECTIVE: To describe different anthropometric and body composition parameters of adolescent girls with adolescent idiopathic scoliosis (AIS), comparing them with the standards of a healthy age-matched population. SUMMARY OF BACKGROUND DATA: Body growth and development of adolescent girls with AIS seems to differ from the healthy subjects, especially at perpubertal stages. Young scoliotic adults have been found to be taller, lighter, and thinner than age-matched healthy population. Body composition profile taken from measurements of skin-fold thickness, bony diameters at different levels, and arm and legs perimeters has not been previously reported in AIS patients. METHODS: A nonconsecutive series of 52 AIS girls (mean age: 13.9 years) with an average scoliotic curve of 27° Cobb (range: 20-58) underwent an anthropometric study. None of the AIS girls had been treated previously with spinal surgery. The control group consisted of 92 girls without spine deformity, matched in age (mean: 13.8 years). Weight, height, and skin-fold thickness in millimeters at six levels were measured. Body mass index (BMI), Ponderal index, percentage of body fat, percentage of muscular tissue, fat mass, lean body mass, muscular weight, bony weight, and residual weight were calculated using standard rules to estimate body composition. The somatotype components (endomorphy, mesomorphy, and ectomorphy) were calculated according to the Carter equations. RESULTS.: Compared with the control population, scoliotic girls had a significantly lower mean weight (51.4 ± 10.2 kg vs. 54.7 ± 8.1 kg; P < 0,05), a lower BMI (20.1 ± 3.4 vs. 21.4 ± 2.4; P < 0.001), and a higher Ponderal index (43.2 ± 2.4 vs. 42.2 ± 1.6; P < 0.01). Girls with AIS showed a progressive decrease of the BMI as the age increased. The percentage of body fat was also lower in scoliotic girls, without significant statistical differences (14.1 ± 3.8 vs. 15 ± 3.6). Out of the 52 AIS girls, 11 (21.2%) showed a BMI below 17.5, which has been considered the limit for anorexia. In the control group, only 3 of 92 girls (3.3%) had BMI below that level. The somatotype differed also between scoliotics and controls: higher in the ectomorphic component (3.29 ± 1.68 vs. 2.40 ± 1.11; P < 0.001), and lower in the mesomorphic component (2.86 ± 0.82 vs. 3.70 ± 1.11; P < 0.01) in AIS patients. CONCLUSION: The differences in some anthropometric parameters (weight, IMC, IP) and in the somatotype suggest that the idiopathic scoliosis not only disturbs normal spine growth but also seems to have implications on the whole corporal development. Whether these changes could be related to abnormal spinal growth or subsequent to nutritional changes in AIS still remains uncertain. Presumably, some endocrine factors affecting body composition and growth might be involved in the etiology of idiopathic scoliosis.