OBJECTIVE: Achondroplasia is the most common of the skeletal dysplasias that cause fatal and disabling growth and developmental disorders in children, and is caused by a mutation in the fibroblast growth factor receptor, type 3 gene(FGFR3). This study aims to analyse the clinical characteristics and gene mutations of ACH to accurately determine whether a patient has ACH and to raise public awareness of the disease.
METHODS: The database of Pubmed, Cochrane Library, Wanfang and CNKI were searched with terms of \"Achondroplasias\" or \"Skeleton-Skin-Brain Syndrome\" or \"Skeleton Skin Brain Syndrome\" or \"ACH\" and \"Receptor, Fibroblast Growth Factor, Type 3\" or \"FGFR3\".
RESULTS: Finally, four hundred and sixty-seven patients with different FGFR3 mutations were enrolled. Of the 138 patients with available gender information, 55(55/138, 40%) were female and 83(83/138, 60%) were male. Among the patients with available family history, 47(47/385, 12%) had a family history and 338(338/385, 88%) patients were sporadic. The age of the patients ranged from newborn babies to 36 years old. The mean age of their fathers was 37 ± 7 years (range 31-53 years). Patients came from 12 countries and 2 continents, with the majority being Asian (383/432, 89%), followed by European (49/432, 11%). Short stature with shortened arms and legs was found in 112(112/112) patients, the abnormalities of macrocephaly in 94(94/112) patients, frontal bossing in 89(89/112) patients, genu valgum in 64(64/112) patients and trident hand were found in 51(51/112) patients. The most common mutation was p.Gly380Arg of the FGFR3 gene, which contained two different base changes, c.1138G > A and c.1138G > C. Ten rare pathogenic mutations were found, including c.831A > C, c.1031C > G, c.1043C > G, c.375G > T, c.1133A > G, c.1130T > G, c.833A > G, c.649A > T, c.1180A > T and c.970_971insTCTCCT.
CONCLUSIONS: ACH was caused by FGFR3 gene mutation, and c.1138G > A was the most common mutation type. This study demonstrates the feasibility of molecular genetic testing for the early detection of ACH in adolescents with short stature, trident hand, frontal bossing, macrocephaly and genu valgum.

Nonossifying fibroma is common in children and adolescents, and nonossifying fibroma with genu valgum is rare in the clinic. This article evaluated the effectiveness of treatment in a case of nonossifying fibroma of the lower femur with genu valgum.
A 16-year-old girl complained of pain in the lower part of her right thigh for one year. She was diagnosed as non ossifying fibroma of the right femur with secondary valgus deformity of the right knee, and was treated in our hospital. We performed curettage, bone grafting and internal fixation,and corrected the valgum deformity at the same time. The patient\'s incision healed well, the pain was disappeared, and the mechanical axis of lower limbs was corrected. No tumor recurrence was found on X- ray examination one year after operation, and the fracture end was healed. The patient could walk normally, and she was satisfied with her limb function.
Nonossifying fibroma with genu valgum is rare in the clinic. The patient was satisfied with our treatment, which achieved a good curative effect.

Genu valgus is one of the most common limb deformities in hereditary multiple exostoses (HME). However, it is easily concealed and may account for subsequent osteoarthritis of the knee. The knees of 56 patients (33 men and 23 women) with HME were investigated bilaterally. Knee valgus was described by the mechanical axis deviation (MAD), mechanical lateral distal femoral angle (LDFA), and medial proximal tibial angle (MPTA). We investigated sex, age, BMI, total number of palpable osteochondromas, number of radiographic osteochondromas around the knee, forearm deformities, morphology and distribution of lesions, and correlations between these factors and genu valgus. The measurement of LDFA and MPTA was performed to identify the sources of genu valgus deformity. Based on the measurement of the mechanical axis, limbs were classified as genu valgus (n = 22) or normal mechanical axis groups (n = 90). The different severities of the genu valgus patients were classified by MAD. By bivariate logistic regression, genu valgus was significantly associated with more sessile and flared metaphyseal lesions. However, only the number of flared metaphyseal lesions had a significant influence on the severity of genu valgus. By analyzing the LDFA and MPTA, it was found that abnormalities of both proximal tibia and distal femur play important roles in genu valgus. Early detection of sessile and flared metaphyseal knee lesions in patients with HME can contribute to early intervention of genu valgus. Level of relevance: Level 2.

BACKGROUND: The purpose of this study was to evaluate the clinical and radiographic outcomes after medial patellofemoral ligament (MPFL) reconstruction combined with supracondylar biplanar femoral derotation osteotomy (FDO) in recurrent patellar dislocation (RPD) with increased femoral anteversion angle (FAA) and genu valgum.
METHODS: Between January 2017 to December 2020, a total of 13 consecutive patients (13 knees, 4 males and 9 females, mean age 18.7 (range, 15-29 years) with RPD with increased FAA (FAA > 25°) and genu valgum (mechanical axis deformity of ≥5°) who underwent supracondylar biplanar FDO using a Tomofix-locking plate combined with MPFL reconstruction in our institution were included. Preoperative full-leg standing radiographs, lateral views, and hip-knee-ankle computed tomography (CT) scans were used to evaluate the mechanical lateral distal femoral angle (mLDFA), anatomical femorotibial angle (aFTA), mechanical axis, patellar height, tibial tubercle-trochlear groove (TT-TG) distance, and torsional angle of the tibial and femoral in the axial plane. Patient reported outcomes were evaluated using the International Knee Documentation Committee (IKDC) score, Kujala score, Lysholm score, visual analog scale (VAS), and Tegner score preoperatively and postoperatively. Postoperative CT scans were used to evaluate the changes of FAA and TT-TG, and full-leg standing radiographs was used to evaluate the changes of mLDFA, aFTA, and mechanical axis.
RESULTS: A total of 13 patients (13 knees) were included with an average follow-up period of 26.7 months (range 24-33). No cases developed wound infection, soft tissue irritation, and recurrent patellar dislocation during the follow-up period after surgery. Bone healing at the osteotomy site was achieved in all cases, and all patients regained full extension and flexion. Clinical outcomes (VAS, Kujala, IKDC, Lysholom, and Tegner scores) improved significantly at the final follow-up after surgery (p < 0.05). The mean mLDFA, aFTA, mechanical axis, and TT-TG distance showed statistically significant improvement following the combined surgery (p < 0.05), while the CDI did not change significantly after surgery (p>0.05).
CONCLUSIONS: MPFL reconstruction combined with supracondylar biplanar FDO showed satisfactory clinical outcomes and radiographic results in the short-term follow-up period.

BACKGROUND: Medial patellofemoral ligament reconstruction (MPFLR) is a well-established procedure for addressing recurrent patellar dislocation (RPD) in young patients. However, despite being a promising procedure for RPD with genu valgum, there is a scarcity of reports on simultaneous MPFLR and closing-wedge distal femoral osteotomy (CWDFO). The purpose of the present study was to observe and analyse the clinical and imaging findings of CWDFO combined with MPFLR for RPD with genu valgum.
METHODS: From May 2015 to April 2018, 25 patients with RPD and genu valgum were surgically treated in our department. Anteroposterior long-leg, weight-bearing, lower-extremity radiographs, lateral radiographs and computed tomography (CT) scans of the patellofemoral joint were obtained, and the anatomical femorotibial angle (aFTA), mechanical lateral distal femoral angle (mLDFA), weight-bearing line rate (WBLR), patellar height, patellar lateral shift (PLS) and tibial tubercle-trochlear groove (TT-TG) distance were analysed. Validated knee scores, such as the Kujala, Lysholm, visual analogue scale (VAS) scores and Tegner socres, were evaluated preoperatively and 2 years postoperatively.
RESULTS: 25 patients, with an average age of 19.8 years (14-27), were evaluated. During the 2-year follow-up period, all patients were able to achieve a better sports level without any problems, with no recurrence of patellar instability. Compared with preoperation, the aFTA, mLDFA, WBLR and PLS showed statistically significant improvement following the procedure (p < 0.001). Meanwhile, no significant differences in the Insall index and TT-TG distance were found. The mean Kujala score, average Lysholm score, VAS score and Tegner socres showed significant postoperative improvement.
CONCLUSIONS: CWDFO combined with MPFLR is a suitable treatment for RPD with genu valgum, and can lead to significant improvement in the clinical and imaging findings of the knee in the short term.

The rate of angular correction (ROAC) is very unpredictable and may be affected by various factors in the treatment of genu valgum and varum by means of guided growth. The purpose of this study was to assess the ROAC in cases from our institution and to identify risk factors associated with the occurrence of lower ROAC.We retrospectively reviewed the chart records of 68 patients undergoing guided growth with figure-eight plate for the correction of genu valgum and varum. Based on the data from these patients, the annual increment of physeal growth was calculated and compared with data from the Anderson chart. The associations between patient characteristics and ROAC were evaluated with the use of univariate logistic regression.The mean rate of femoral angular correction was 10.29 degrees/year, while the mean rate of tibial angular correction was 7.92 degrees/year. In a univariate logistic regression analysis, the variables associated with a higher risk of lower ROAC included non-idiopathic coronal deformity of the knee (odds ratio = 13.58, p < 0.001) and body weight at or above the 95th percentile for children (odds ratio = 2.69, p = 0.020).Obesity and non-idiopathic coronal deformity of the knee are risk factors for lower ROAC. It is still uncertain whether severity of deformity, race, and operative procedure have a substantial effect on the rate of correction.Level III evidence.

BACKGROUND: Temporary hemiepiphysiodesis (TH) using an eight-Plate implant is one of the most common surgeries used for the correction of coronal angular deformities around the knee in adolescents. However, few studies have focused on children aged less than 10 years treated with TH using an eight-Plate implant. The purpose of this study was to investigate the efficacy, correction velocity, and complications of TH with an eight-Plate implant as well as the occurrence of rebound and risk factors in this population.
METHODS: This retrospective study included a total of 135 physes (101 knees) from 66 children (mean age of 4.69 years old, range from 1 to 10 years old) who underwent TH with an eight-Plate implant to correct coronal genu angular deformities in our hospital. Related clinical factors were recorded and analysed by multivariable linear and logistic regression models.
RESULTS: The mean deformity correction period was 13.26 months, and the mean follow-up after eight-Plate removal was 12.71 months. In all, 94.06% (95/101 knees) of the genu angular deformities were completely corrected. Non-idiopathic genu angular deformity was found to be an independent risk factor for deformity correction failure (odds ratio (OR) = 2.47). The femoral correction velocity was significantly higher than the tibial correction velocity (1.28° vs. 0.83° per month, p < 0.001). After adjustment for other factors, younger children had higher correction velocities in the distal femur; however, genu valgum and idiopathic deformities were associated with higher correction velocities in the proximal tibia. In addition, we found three (3/101, 2.97%) knees with genu valgum that experienced rebound after removal of the eight-Plate, while five (5/101, 4.95%) knees with non-idiopathic genu angular deformity experienced screw loosening. No other complications were found, and non-idiopathic deformity was the only risk factor for complications (OR = 3.96). No risk factor was found for rebound in our study.
CONCLUSIONS: TH using an eight-Plate implant is an effective procedure for coronal genu angular deformities with a low incidence of complications and rebound in patients younger than 10 years old. For this population, TH using an eight-Plate should be considered as soon as the deformity stops responding to conservative treatments. The parents of children younger than 10 years of age with non-idiopathic deformities should be informed preoperatively that the deformity may be prone to correction failure or screw loosening after eight-Plate implantation.

BACKGROUND: Common peroneal nerve (CPN) palsy is a devastating complication that follows total knee arthroplasty (TKA). However, there are only a few studies on concrete measures for protecting the CPN in patients. This study aimed to put forward the CPN \'pre-release\' method to protect the nerve.
METHODS: A prospective study was conducted on 30 patients (34 knees) with severe valgus knees who underwent CPN pre-release. This was a two-incision approach, and required a separate dissection of about three centimeters from the TKA operation. Clinical measurements including pre- and postoperative motor and sensory nerve function of CPN, radiological evaluation, complications, and the revised data were analyzed and compared.
RESULTS: The average preoperative femorotibial angle was 31.3 ± 8.0°. All patients had completely normal motor (grade 5) and sensory nerve function of CPN postoperatively, and there was no transient or late-onset CPN palsy. Patients had a routine rehabilitation with full weight bearing after recovery from anesthesia, including the knees with unconstrained extension/flexion motion. During the last follow-up visit, the visual analog scale, Knee Society Score, Hospital for Special Surgery knee-rating scale, and range of motion were 2.06 ± 1.13, 92.18 ± 5.57, 90.18 ± 3.70, and 115.59 ± 7.76°, respectively. There were no revisions for instability and recurrent valgus deformities during follow-up. Also, the femorotibial angle, hip-knee-ankle angle, condylar-hip angle, and plateau-ankle angle were 4.9 ± 2.0°, 179.09 ± 3.21°, 89.97 ± 2.41°, and 90.53 ± 1.26°, respectively.
CONCLUSIONS: The CPN pre-release for severe valgus knees is an effective method for nerve protection, achieving an adequate and safe release of lateral soft tissue, and providing immediate and early functional rehabilitation with decreasing constrained implant.

BACKGROUND: The purpose of this study was to determine the relationship between the Fujisawa point and postoperative knee valgus angle and the anatomical factors influencing this relationship.
METHODS: An experimental study was conducted including 116 patients with medial compartment knee osteoarthritis undergoing treatment with open-wedge high tibial osteotomy (OWHTO). Each patient received simulated HTO through the Fujisawa point in the picture archiving and communication system (PACS). The preoperative hip-knee-ankle (HKA) angle and lower extremity anatomical parameters were recorded before the computerized HTO simulation. The postoperative knee valgus angle was measured after this procedure. A second simulation HTO was performed to adjust the mechanical axis to the optimal valgus angle (4.5°) and calculate the percentage of the tibial plateau width where the Mikulicz line crossed the knee. The Spearman correlation test and multivariate regression were used for analysis.
RESULTS: The median preoperative HKA varus angle of this study cohort was 174.1° (170.8, 176.2°). The median knee valgus angle after simulated osteotomy through the Fujisawa point was 2.4° (2.1, 2.7°). The valgus angle was positively correlated with the tibial plateau width (r = 0.23, p = .013) and preoperative HKA angle (r = 0.32, p < .001). Multivariate regression analysis showed that the preoperative HKA angle was a significant contributor to the postoperative valgus angle. When conducting the osteotomy with the optimal valgus angle (4.5°), the percentage of the Mikulicz line passing through the tibial plateau was 71.93% (67-78%).
CONCLUSIONS: The preoperative HKA angle affects the postoperative valgus angle after HTO. If the optimal valgus angle of 4.5° is desired, a more lateral position of the Fujisawa point should be targeted during OWHTO, which accounts for approximately 71.9% of the tibial plateau.

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