New bone formation accelerates during lower limb lengthening and deformity correction in children with Ollier's disease.

BACKGROUND: Ollier's disease can cause severe length discrepancy of the lower extremities and deformity in children. Osteotomy and limb lengthening with external fixation can correct the limb deformity. This study evaluated (1) whether the duration of external fixation was reduced in patients with Ollier's disease, and (2) the incidence of complications such as pin tract infection, external fixation loosening, and joint stiffness. METHODS: Two groups were compared with respect to age, angular correction (AC), lengthening gap (LG), distraction index (DI), lengthening length (LL), lengthening length percentage (L%), lengthening index (LI), bone healing index (BHI), and external fixation index (EFI). Group 1 (Ollier's disease) comprised nine patients undergoing 11 lower limb lengthening procedures using external fixators; group 2 (control, normal lengthened bone) comprised 28 patients undergoing 29 lengthening procedures with external fixators. RESULTS: In patients with Ollier's disease, full correction of the deformity and full restoration of length were achieved in all cases. In the femur, the mean AC (15.97° vs. 6.72°) and DI (1.11 mm/day vs. 0.78 mm/day) were significantly larger, while the LI (9.71 days/cm vs. 13.49 days/cm), BHI (27.00 days/cm vs. 42.09 days/cm), and EFI (37.86 days/cm vs. 56.97 days/cm) were all significantly shorter in group 1 than in group 2 (p < 0.05). In the tibia, the mean AC and L% were larger, while the LG, LI, BHI, and EFI were all shorter in group 1 than in group 2. There was no significant difference between the two groups in the incidence of complications. CONCLUSION: In children with Ollier's disease, new bone formation accelerated and the healing speed of the lengthened segments was faster throughout the whole lengthening period with external fixation, and full correction of the deformity and full restoration of length could be achieved.

Intra- and interobserver reliability analysis of pediatric lower limb parameters on digital long leg radiographs.

BACKGROUND: Malalignments of the lower extremity are common reasons for orthopedic consultation because it may lead to osteoarthritis in adulthood. An accurate and reliable radiological assessment of lower limb alignment in children and adolescents is essential for clinical decision-making on treatment of limb deformities and for regular control after a surgical intervention. OBJECTIVE: First, does the analysis of full-length standing anteroposterior radiographs show a good intra- and interobserver reliability? Second, which parameter is most susceptible to observer-dependent errors? Third, what is the Standard Error of Measurement (SEM95%) of the absolute femoral and tibial length? METHODS: Two observers evaluated digital radiographs of 144 legs from 36 children and adolescents with pathological valgus alignment before a temporary hemiepiphysiodesis and before implant removal. Parameters included Mechanical Femorotibial Angle (MFA), Mechanical Axis Deviation (MAD), mechanical Lateral Distal Femoral Angle (mLDFA), mechanical Medial Proximal Tibial Angle (mMPTA), mechanical Lateral Proximal Femoral Angle (mLPFA), mechanical Lateral Distal Tibial Angle (mLDTA), Joint Line Convergence Angle (JLCA), femur length, tibial length. Intra- and interobserver reliability (ICC2,1), SEM95% and proportional errors were calculated. RESULTS: The intra- and interobserver reliability for almost all measurements was found to be good to excellent (Intra-ICC2,1: 0.849-0.999; Inter-ICC2,1: 0.864-0.996). The SEM95% of both observers was found to be ± 1.39° (MFA), ± 3.31 mm (MAD), ± 1.06° (mLDFA) and ± 1.29° (mMPTA). The proportional error of MAD and MFA is comparable (47.29% vs. 46.33%). The relevant knee joint surface angles show a lower proportional error for mLDFA (42.40%) than for mMPTA (51.60%). JLCA has a proportional error of 138%. Furthermore, the SEM95% for the absolute values of the femoral and tibial length was 4.53 mm for the femur and 3.12 mm for the tibia. CONCLUSIONS: In conclusion, a precise malalignment measurement and the knowledge about SEM95% of the respective parameters are crucial for correct surgical or nonsurgical treatment. The susceptibility to error must be considered when interpreting malalignment analysis and must be considered when planning a surgical intervention. The results of the present study elucidate that MAD and MFA are equally susceptible to observer-dependent errors. This study shows good to excellent intra- and interobserver ICCs for all leg alignment parameters and joint surface angles, except for JLCA. TRIAL REGISTRATION: This study was registered with DRKS (German Clinical Trials Register) under the number DRKS00015053. LEVEL OF EVIDENCE: I, Diagnostic Study.

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)

An accessory muscle of the posterior compartment of leg of clinical significance: a cadaveric case report / Un músculo accesorio del compartimento posterior de la pierna de importancia clínica: reporte de un caso cadavérico

SUMMARY: Accessory and anomalous muscles are common in humans, although their unique morphologic characteristics can make accurate identification difficult. In this case report, we attempt to identify an anomalous accessory muscle of the posterior compartment of the leg [Compartimentum posterius cruris] detected during cadaveric dissection and discuss its clinical significance. The muscle was found on the right lower limb of an 81-year-old female cadaver and extended from the distal femur to attach to the gastrocnemius muscle at the point where the medial and lateral heads fuse. At its origin, the muscle was found lateral to the popliteal vessels and crossed posterior to these vessels and tibial nerve. It displayed characteristics similar to both an accessory plantaris muscle and gastrocnemius tertius, thus making its ultimate identification difficult. Though the muscle displayed a morphologically similar appearance to the plantaris, we suggest that its common insertion with the gastrocnemius best identifies it as a gastrocnemius tertius. In addition, due to its relationship with the popliteal neurovasculature, it is possible that this muscle could have resulted in neurovascular entrapment although it is unknown whether or not this cadaver exhibited symptoms.

RESUMEN: Los músculos accesorios y anómalos son comunes en los seres humanos, aunque sus características morfológicas pueden dificultar la identificación precisa. En este reporte de caso, intentamos identificar un músculo accesorio anómalo del compartimento posterior de la pierna [Compartimentum posterius cruris] detectado durante la disección cadavérica y discutir su importancia clínica. El músculo fue encontrado en el miembro inferior derecho de una mujer de 81 años de edad y se extendía desde la parte distal del fémur para unirse al músculo gastrocnemio en la fusión de sus cabezas medial y lateral. En su origen, el músculo se encontraba lateral a los vasos poplíteos y cruzaba posteriormente a estos vasos y al nervio tibial, presentando características similares tanto al músculo plantar accesorio como al gastrocnemio tercero, lo que dificultaba su identificación final. Similar al músculo plantar, sugerimos que debido a su inserción común con el gastrocnemio lo identifica mejor como un músculo gastrocnemio tercero. Además, debido a su relación con la neurovasculatura poplítea, es posible que este músculo haya dado lugar a un síndrome de compresión neurovascular aunque se desconoce si este individuó presentó síntomas o no en vivo.

Better outcomes and reduced failures for arthroplasty over osteotomy for advanced compartmental knee osteoarthritis in patients older than 50 years.

BACKGROUND: Both compartmental knee arthroplasty (CKA) and open-wedge high tibial osteotomy (HTO) may be used to treat patients older than 50 years of age with advanced compartmental osteoarthritis (OA) secondary to leg axis deformities. A meta-analysis was conducted to clarify the role of open-wedge HTO versus CKA for patients older than 50 years with advanced compartmental knee OA. The present investigation aimed to analyse the clinical outcomes, implant failure and survivorship. METHODS: This meta-analysis was performed in accordance with the PRISMA guidelines. In September 2020, the main online databases were accessed. All clinical trials comparing the outcomes of open-wedge HTO versus CKA for compartmental knee OA were considered. Data analysis was performed through the Review Manager Software 5.3 (the Nordic Cochrane Collaboration, Copenhagen). Implant survivorship was analysed with a Kaplan-Meier (KM) curve that was performed using the STATA/MP software (StataCorp, College Station, TX). RESULTS: Data from 618 (HTO, 307; CKA, 311) patients were collected. Good baseline comparability among patient age, BMI and gender was detected. The Tegner Activity Scale was higher in the CKA group (P = 0.04), as were the Lysholm score (P = 0.001), the International Knee Documentation Committee (P = 0.0001) and the Knee injury and Osteoarthritis Outcome Score (P = 0.05). At a mean follow-up of 5 years, revisions were less in the CKA cohort (OR, 2.27; P = 0.004). The Kaplan-Meier curve evidenced longer implant survivorship in favour of the CKA group (P = 0.01). CONCLUSION: In patients older than 50 years of age with compartmental knee OA secondary to frontal axis leg deformities, CKA performed better than open-wedge HTO.

One-Step Lower Leg Reconstruction with Vascularized Functional Vastus Lateralis Muscle Flap in the Treatment of Embryonal Rhabdomyosarcoma for a Six-Month-Old Boy: A Case Report.

Rhabdomyosarcoma (RMS) is a common soft tissue sarcoma in childhood, however, it is very rare in the neonatal period (0.4-2% of cases). This case depicts a boy, who presented with RMS at two weeks of age, but officially diagnosed at the age of three months. MRI and scintigraphy determined a soft tissue tumor in the soleus muscle, while biopsy confirmed embryonal RMS with high mitotic activity (Ki67 (monoclonal antibodies) ~80%). CWS (Cooperative Weichteilsarkom Studiengruppe)-2012 with I2VA (ifosfamide, vincristine, actinomycin) chemotherapy regimen was administered per protocol. Surgical treatment was performed at age of six months and 18 days. The operation consisted of radical tumor resection and total triceps surae with partial fibula resection. Immediate reconstruction of triceps muscle was accomplished using a vascularized functional musculocutaneous vastus lateralis flap. Functional outcome was measured using the Lower Extremity Functional Scale (LEFS) and the Foot and Ankle Outcome Score (FAOS) with the results of 92.5% and 99% respectively.

Rabbit growth plate morphology in temporary bilateral blocking / Morfología de la placa de crecimiento de conejos durante bloqueo bilateral temporario

Blocking of the growth plate (GP) using plates with screws (tension band plating) is a modern method used to correct deformities and moderate leg length discrepancy in growing children. Determining the duration of temporary bilateral blocking without the occurrence of irreversible changes of GP is of paramount importance important. Methods: Two-month-old Californian breed male rabbits (n=30) were exposed to bilateral blocking of the distal GP of the right femur locking plates with screws for 3, 5, and 7 weeks. The fixators were removed after 5 and 7 weeks in 18 rabbits and 3 weeks after that, animals were sacri!ced. The contralateral limb was used as a control. Histological, histomorphometric, and X-ray analyses were performed. Results: During GP blocking, its height gradually decreased. This decreased was more pronounced after 7 weeks. Destructive changes progressed with an increase in the blocking duration. Three weeks after discontinuation of the bilateral blocking that lasted 5 weeks, the height of the GP signi!cantly increased 1.2 times on the lateral side and 1.9 times on the medial side (p<0.001) compared to the control. When blocking was discontinued after 7 weeks, the structure of the GP was partially restored after 3 weeks, the height of GP signi!cantly increased 1.2 times on the lateral side, and 1.07 times on the medial side (p<0.01) compared to the control. Conclusion: Restoration of the structuralfunctional features of the GP after the removal of the plates depends on the duration of temporary bilateral blocking, which must be taken into account in the clinical setting. (AU)

El bloqueo de la placa de crecimiento (PC) utilizando placas con tornillos (banda de tensión) es un método moderno utilizado para corregir deformidades y alteraciones moderadas en la longitud de las piernas en niños en crecimiento. Es de suma importancia determinar cuál debe ser la duración del bloqueo bilateral temporal sin que ocurran cambios irreversibles en la PC. Métodos: Conejos machos de raza californiana de dos meses de edad (n = 30) fueron expuestos al bloqueo bilateral de la PC distal colocando placas del fémur derecho con tornillos durante 3, 5 y 7 semanas. Los fijadores fueron retirados después de 5 y 7 semanas en 18 de los conejos, y 3 semanas después los animales fueron sacrificados. La extremidad contralateral se utilizó como control. Se realizaron análisis histológicos, histomorfométricos y de rayos X. Resultados: Durante el bloqueo de la PC, su altura disminuyó gradualmente. Esta disminución fue más pronunciada después de 7 semanas. Los cambios destructivos se incrementaron a medida aumentaba la duración del bloqueo. Tres semanas después de la interrupción del bloqueo bilateral que duró 5 semanas, la altura de la PC aumentó significativamente 1.2 veces en el lado lateral y 1.9 veces en el lado medial (p <0.001) en comparación con el control. Conclusión: La restauración de las características funcionales estructurales de la PC después de la extracción de las placas depende de la duración del bloqueo bilateral temporal, lo que debería tenerse en cuenta en el tratamiento clínico de estas alteraciones. (AU)

Radiography, CT, and MRI of Hip and Lower Limb Disorders in Children and Adolescents.

Infants and children are vulnerable to congenital and developmental hip and lower extremity disorders. These disorders have diverse causes in pediatric patients, and owing to potential related complications that can lead to degenerative disease in adulthood, an accurate diagnosis is essential. A common disease is developmental dysplasia of the hip, which affects nearly 1% of newborns. This condition is best evaluated with US and conventional radiography. Slipped capital femoral epiphysis affects approximately 0.01% of young teenagers and is initially evaluated with radiography. Femoroacetabular impingement is a risk factor for early osteoarthritis and can be assessed with radiography, CT, or MRI. Limb length discrepancy is defined as a greater than 2-cm difference in length between paired bilateral lower extremities. There are several methods of measuring this difference, and the use of an accurate imaging modality is essential for treatment. Developmental bowing is a physiologic condition involving varus angulation of the knee and is best evaluated by using conventional radiography. Blount disease is a progressive pathologic genu varum centered at the tibia; the three subtypes are infantile, juvenile, and adolescent. In- and out-toeing disorders are caused by abnormal tibial and femoral torsion that usually self-corrects during lower limb growth. The ability to recognize these conditions is essential for differentiating those that will resolve spontaneously versus those that will require treatment. The imaging features of congenital and developmental hip and lower extremity disorders are reviewed, with emphasis on diagnosis, radiologic assessment, associated findings, and classification. ©RSNA, 2019.

A rare disease with a rare presentation: hemi-atrophy caused by fibromuscular dysplasia in a 27 month old girl.

We discuss the case of a 27-month-old girl afflicted with fibromuscular dysplasia. She presented with hemiatrophy of left upper and lower limbs, nail dystrophy, ulcers on the tips of her toes, cold and painful limbs, foot drop, and hypertension. The initial appearance started at 2 months of age and other diagnoses such as complex regional pain syndrome, reflex sympathetic syndrome, vasculitis and coagulation disorders had been considered. Angiography revealed that all the arterial branches of the left lower and upper limbs, from brachial to ulnar and radial, and from iliac and femoral to tibialis arteries were affected. Sural nerve biopsy confirmed the diagnosis. In the follow-up visits until 2 years after the patient's discharge she did not develop any new problem and her blood pressure was controlled by enalapril and amlodipine.

An attenuated rate of leg muscle protein depletion and leg free amino acid efflux over time is seen in ICU long-stayers.

BACKGROUND: There is extensive documentation on skeletal muscle protein depletion during the initial phase of critical illness. However, for intensive care unit (ICU) long-stayers, objective data are very limited. In this study, we examined skeletal muscle protein and amino acid turnover in patients with a prolonged ICU stay. METHODS: Patients (n = 20) were studied serially every 8-12 days between days 10 and 40 of their ICU stay as long as patients stayed in the ICU. Leg muscle protein turnover was assessed by measurements of phenylalanine kinetics, for which we employed a stable isotope-labeled phenylalanine together with two-pool and three-pool models for calculations, and results were expressed per 100 ml of leg volume. In addition, leg muscle amino acid flux was studied. RESULTS: The negative leg muscle protein net balance seen on days 10-20 of the ICU stay disappeared by days 30-40 (p = 0.012). This was attributable mainly to an increase in the de novo protein synthesis rate (p = 0.007). It was accompanied by an attenuated efflux of free amino acids from the leg. Leg muscle protein breakdown rates stayed unaltered (p = 0.48), as did the efflux of 3-methylhistidine. The arterial plasma concentrations of free amino acids did not change over the course of the study. CONCLUSIONS: In critically ill patients with sustained organ failure and in need of a prolonged ICU stay, the initial high rate of skeletal muscle protein depletion was attenuated over time. The distinction between the acute phase and a more prolonged and more stable phase concerning skeletal muscle protein turnover must be considered in study protocols as well as in clinical practice. TRIAL REGISTRATION: Australian New Zealand Trial Registry, ACTRN12616001012460 . Retrospectively registered on 1 August 2016.

[Functional results of open wedge osteotomies in lower limb angular deformities in teenagers]. / Resultados funcionales en pacientes adolescentes con corrección de deformidades angulares de los miembros inferiores tratados con osteotomía en cuña abierta.

OBJECTIVE: The aim of this study was to evaluate the functional and radiographic results of adolescent patients with angular deformities of the pelvic limbs treated with an open wedge osteotomy system. MATERIAL AND METHODS: Observational, prospective, analytical and cross-sectional study of patients with angular deformity of the pelvic limbs treated with an open wedge osteotomy. We made a radiographic evaluation of the femorotibial angle, MAD angle, lower limb discrepancy, and pre- and postoperative degrees of the rotational center of deformity (CORA), as well as a telephone survey of the postoperative functional evaluation IKDC 2000. RESULTS: It was a sample of 28 patients, 21 female and seven male, with a mean age of 15.3 years. Their diagnoses: idiopathic genu valgo, 10 cases; Blount disease, four; benign tumors (enchondromatosis and osteochondromatosis), three; hypophosphatemic rickets; three, among others, such as sequelae of septic arthritis of the knee, femoral hypoplasia, and fracture sequels. It was a total of 29 osteotomies, 17 of the tibia and 12 of the femur; 17 osteotomies presented deviation to lateral of the mechanical axis, and 11, medial deviation, with a mean femorotibial angle of 17 and 7.35 (p 0.03), respectively. The mean initial discrepancy had an improvement of 0.5 cm. Postoperative IKDC in the subjective evaluation had an average of 95.3 points. CONCLUSIONS: The fixation of osteotomies with open wedge plates is an option with good clinical and radiographic results.

OBJETIVO: Evaluar los resultados funcionales y radiográficos de los pacientes adolescentes con deformidades angulares de los miembros pélvicos tratados con sistema de osteotomía en cuña abierta. MATERIAL Y MÉTODOS: Estudio observacional, prospectivo, analítico y transversal de los pacientes con diagnóstico de deformidad angular de los miembros pélvicos tratados con sistema de osteotomía en cuña abierta. Se valoraron mediante radiografía el ángulo femorotibial, el ángulo de desviación del eje mecánico (MAD), la discrepancia de miembros inferiores y los grados de centro de rotación de la deformidad (CORA) pre- y post­quirúrgico. Se realizó una encuesta telefónica de la escala funcional postquirúrgica IKDC 2000. RESULTADOS: Se valoraron 28 pacientes, 21 femeninos y siete masculinos, con un promedio de edad de intervención quirúrgica de 15.3 años. Sus diagnósticos: genu valgo idiopático, 10 casos; enfermedad de Blount, cuatro; tumoraciones benignas, tres; raquitismo hipofosfatémico, tres y otros como secuelas de artritis séptica de rodilla, hipoplasia femoral y secuelas de fractura. Fueron un total de 29 osteotomías: 17 de tibia y 12 de fémur; 17 osteotomías presentaban desviación lateral del eje mecánico, 11 desviación medial, con una media de ángulo femorotibial de 17 y 7.35 (p 0.03), respectivamente; la discrepancia inicial promedio tuvo una mejoría de 0.5 cm. El IKDC postquirúrgico en la evaluación subjetiva tuvo una media de 95.3 puntos. CONCLUSIONES: La fijación de osteotomías con placas de cuña abierta es una opción con buenos resultados tanto clínicos como radiográficos.

Resultados funcionales en pacientes adolescentes con corrección de deformidades angulares de los miembros inferiores tratados con osteotomía en cuña abierta / Functional results of open wedge osteotomies in lower limb angular deformities in teenagers

Resumen: Objetivo: Evaluar los resultados funcionales y radiográficos de los pacientes adolescentes con deformidades angulares de los miembros pélvicos tratados con sistema de osteotomía en cuña abierta. Material y métodos: Estudio observacional, prospectivo, analítico y transversal de los pacientes con diagnóstico de deformidad angular de los miembros pélvicos tratados con sistema de osteotomía en cuña abierta. Se valoraron mediante radiografía el ángulo femorotibial, el ángulo de desviación del eje mecánico (MAD), la discrepancia de miembros inferiores y los grados de centro de rotación de la deformidad (CORA) pre- y post­quirúrgico. Se realizó una encuesta telefónica de la escala funcional postquirúrgica IKDC 2000. Resultados: Se valoraron 28 pacientes, 21 femeninos y siete masculinos, con un promedio de edad de intervención quirúrgica de 15.3 años. Sus diagnósticos: genu valgo idiopático, 10 casos; enfermedad de Blount, cuatro; tumoraciones benignas, tres; raquitismo hipofosfatémico, tres y otros como secuelas de artritis séptica de rodilla, hipoplasia femoral y secuelas de fractura. Fueron un total de 29 osteotomías: 17 de tibia y 12 de fémur; 17 osteotomías presentaban desviación lateral del eje mecánico, 11 desviación medial, con una media de ángulo femorotibial de 17 y 7.35 (p < 0.03), respectivamente; la discrepancia inicial promedio tuvo una mejoría de 0.5 cm. El IKDC postquirúrgico en la evaluación subjetiva tuvo una media de 95.3 puntos. Conclusiones: La fijación de osteotomías con placas de cuña abierta es una opción con buenos resultados tanto clínicos como radiográficos.

Abstract: Objective: The aim of this study was to evaluate the functional and radiographic results of adolescent patients with angular deformities of the pelvic limbs treated with an open wedge osteotomy system. Material and methods: Observational, prospective, analytical and cross-sectional study of patients with angular deformity of the pelvic limbs treated with an open wedge osteotomy. We made a radiographic evaluation of the femorotibial angle, MAD angle, lower limb discrepancy, and pre- and postoperative degrees of the rotational center of deformity (CORA), as well as a telephone survey of the postoperative functional evaluation IKDC 2000. Results: It was a sample of 28 patients, 21 female and seven male, with a mean age of 15.3 years. Their diagnoses: idiopathic genu valgo, 10 cases; Blount disease, four; benign tumors (enchondromatosis and osteochondromatosis), three; hypophosphatemic rickets; three, among others, such as sequelae of septic arthritis of the knee, femoral hypoplasia, and fracture sequels. It was a total of 29 osteotomies, 17 of the tibia and 12 of the femur; 17 osteotomies presented deviation to lateral of the mechanical axis, and 11, medial deviation, with a mean femorotibial angle of 17 and 7.35 (p < 0.03), respectively. The mean initial discrepancy had an improvement of 0.5 cm. Postoperative IKDC in the subjective evaluation had an average of 95.3 points. Conclusions: The fixation of osteotomies with open wedge plates is an option with good clinical and radiographic results.

Agenesis of the medial gastrocnemius and plantar muscle: case presentation / Agenesia del gastrocnemio medial y del músculo plantar: presentación de caso

We present the case of a left medial gastrocnemius and plantar muscle agenesis in a 58-years old male cadaveric specimen from the Anatomy Laboratory at the Universidad Industrial de Santander (Bucaramanga-Colombia). These unusual muscle absences and associated functional alterations, enrich anatomical knowledge and become important by the time the affected individual may need a reconstructive procedure.

Se presenta un caso de agenesia de los músculos gastrocnemio medial y plantar izquierdos en un espécimen cadavérico masculino de 58 años, del laboratorio de anatomía de la Universidad Industrial de Santander (Bucaramanga- Colombia). Estas raras ausencias musculares enriquecen el conocimiento anatómico con sus alteraciones funcionales concomitantes y permiten postular los posibles procedimientos reconstructivos requeridos en los individuos que las presentan.

Parasitic twin--a supernumerary limb associated with spinal malformations. A case report.

We describe a case of rachipagus parasitic twin with spinal cord malformations (lipomyelomeningocele and tethered cord) in a 7-month-old Ethiopian infant. The parasitic mass had a well-formed foot, ankle and lower leg and a small sinus that resembled an anus. Magnetic resonance imaging scans revealed spinal malformations including a distal syringohydromyelia. The mass was successfully resected and the dural attachment was closed. Histopathological examination confirmed the diagnosis. Postoperatively, the child had unchanged, intact neurological function in both lower limbs. Almost all rachipagus parasitic twins are associated with spinal malformations. They should, therefore, be operated on by surgeons experienced in myelomeningocele surgery.

Calf Augmentation and Reshaping with Autologous Fat Grafting.

BACKGROUND: Despite multiple advantages of fat grafting for calf augmentation and re-shaping over traditional silicone calf implants, few reports have been published. OBJECTIVES: To report our technique and results with autologous fat grafting for calf augmentation and reshaping. METHODS: A retrospective review of the senior author's (JEV) experience with autologous fat grafting for calf augmentation was performed. Medial and lateral calf augmentation was accomplished with injection of prepared autologous lipoaspirate intramuscularly and subcutaneously. RESULTS: Over a 5-year period, 13 patients underwent calf augmentation and reshaping with the described technique. Ten cases were bilateral (77%), and 3 cases (23%) were performed for congenital leg discrepancies. Mean 157 cc of prepared lipoaspirate was transferred per leg, with roughly 60% and 40% transferred into the medial and lateral calf, respectively. Four patients (31%) underwent a second round of autologous fat injection for further calf augmentation because they desired more volume. At mean 19.6 month follow-up, durable augmentation and improvement in calf contour was documented by comparison of standardized preoperative and postoperative photographs. CONCLUSIONS: Autologous calf fat grafting is a viable alternative to traditional implant-based calf augmentation for congenital calf discrepancies and the aesthetic pseudo-varus deformity. This technique provides results comparable to those obtainable with traditional methods. LEVEL OF EVIDENCE 4: Therapeutic.

Microdeletions on 6p22.3 are associated with mesomelic dysplasia Savarirayan type.

INTRODUCTION: Mesomelic dysplasias are a group of skeletal disorders characterised by shortness of the middle limb segments (mesomelia). They are divided into 11 different categories. Among those without known molecular basis is mesomelic dysplasia Savarirayan type, characterised by severe shortness of the middle segment of the lower limb. OBJECTIVE: To identify the molecular cause of mesomelic dysplasia Savarirayan type. METHODS AND RESULTS: We performed array comparative genomic hybridisation in three unrelated patients with mesomelic dysplasia Savarirayan type and identified 2 Mb overlapping de novo microdeletions on chromosome 6p22.3. The deletions encompass four known genes: MBOAT1, E2F3, CDKAL1 and SOX4. All patients showed mesomelia of the lower limbs with hypoplastic tibiae and fibulae. We identified a fourth patient with intellectual disability and an overlapping slightly larger do novo deletion also encompassing the flanking gene ID4. Given the fact that the fourth patient had no skeletal abnormalities and none of the genes in the deleted interval are known to be associated with abnormalities in skeletal development, other mutational mechanisms than loss of function of the deleted genes have to be considered. Analysis of the genomic region showed that the deletion removes two regulatory boundaries and brings several potential limb enhancers into close proximity of ID4. Thus, the deletion could result in the aberrant activation and misexpression of ID4 in the limb bud, thereby causing the mesomelic dysplasia. CONCLUSIONS: Our data indicate that the distinct deletion 6p22.3 is associated with mesomelic dysplasia Savarirayan type featuring hypoplastic, triangular-shaped tibiae and abnormally shaped or hypoplastic fibulae.