OBJECTIVE: To report the clinical characteristics and treatment analysis of 3 cases of congenital ulnar collateral flexor contracture of the forearm and take a reference for clinic. METHODS: A total of 3 patients with congenital ulnar collateral flexor contracture of the forearm were admitted between February 2019 and August 2021. Two patients were male and 1 was female, and their ages were 16, 20, and 16 years, respectively. The disease durations were 8, 20, and 15 years, respectively. They all presented with flexion deformity of the proximal and distal interphalangeal joints of the middle, ring, and little fingers in the neutral or extended wrist position, and the deformity worsened in the extended wrist position. The total action motion (TAM) scores of 3 patients were 1 and the gradings were poor. The Carroll's hand function evaluation scores were 48, 55, and 57, and the grip strength indexes were 72.8, 78.4, and 30.5. Preoperative CT of case 2 showed a bony protrusion of the flexor digitorum profundus tendon at the proximal end of the ulna; and MRI of case 3 showed that the ulnar flexor digitorum profundus presented as a uniform cord. After diagnosis, all patients were treated with operation to release the denatured tendon, and functional exercise was started early after operation. RESULTS: The incisions of 3 patients healed by first intention. Three patients were followed up for 12, 35, and 12 months, respectively. The hand function and the movement range of the joints significantly improved, but the grip strength did not significantly improve. At last follow-up, TAM scores were 3, 4, and 4, respectively, among which 2 cases were excellent and 1 case was good. Carroll's hand function evaluation scores were 95, 90, and 94, and the grip strength indexes were 73.5, 81.3, and 34.2, respectively. CONCLUSION Congenital ulnar collateral flexor contracture is a rare clinical disease that should be distinguished from ischemic muscle contracture. The location of the contracture should be identified and appropriate surgical timing should be selected for surgical release. Active postoperative rehabilitation and functional exercise can achieve good hand function.
Humans ; Male ; Female ; Forearm/surgery* ; Contracture/surgery* ; Muscle, Skeletal ; Tendons/surgery* ; Ulna/surgery* ; Range of Motion, Articular

BACKGROUND: Total knee arthroplasty (TKA) can reduce severe joint pain and improve functional disability in hemophilia. However, the long-term outcomes have rarely been reported in China. Therefore, this study aimed to evaluate the long-term outcomes and complications of TKA in Chinese patients with hemophilic arthropathy. METHODS: We retrospectively reviewed patients with hemophilia who underwent TKA between 2003 and 2020, with at least 10 years of follow-up. The clinical results, patellar scores, patients' overall satisfaction ratings, and radiological findings were evaluated. Revision surgery for implants during the follow-up period was recorded. RESULTS: Twenty-six patients with 36 TKAs were successfully followed up for an average of 12.4 years. Their Hospital for Special Surgery Knee Score improved from an average of 45.8 to 85.9. The average flexion contracture statistically significantly decreased from 18.1° to 4.2°. The range of motion (ROM) improved from 60.6° to 84.8°. All the patients accepted patelloplasty, and the patients' patellar score improved from 7.8 preoperatively to 24.9 at the last follow-up. There was no statistically significant difference in clinical outcomes between the unilateral and bilateral procedures, except for a better ROM at follow-up in the unilateral group. Mild and enduring anterior knee pain was reported in seven knees (19%). The annual bleeding event was 2.7 times/year at the last follow-up. A total of 25 patients with 35 TKAs were satisfied with the procedure (97%). Revision surgery was performed in seven knees, with 10- and 15-year prosthesis survival rates of 85.8% and 75.7%, respectively. CONCLUSIONS TKA is an effective procedure for patients with end-stage hemophilic arthropathy, which relieves pain, improves knee functions, decreases flexion contracture, and provides a high rate of satisfaction after more than ten years of follow-up.
Humans ; Arthroplasty, Replacement, Knee/methods* ; Hemophilia A/surgery* ; Follow-Up Studies ; Retrospective Studies ; Treatment Outcome ; Knee Joint/surgery* ; Range of Motion, Articular ; Arthritis/complications* ; Pain ; Contracture/surgery* ; Surgeons ; Knee Prosthesis

OBJECTIVE: To summarize the clinical characteristics, differential diagnosis, and treatment methods of finger flexion contracture caused by three kinds of forearm flexor diseases. METHODS: Between December 2008 and August 2021, 17 patients with finger flexion contracture were treated, including 8 males and 9 females, aged 5-42 years, with a median of 16 years. The disease duration ranged from 1.5 months to 30 years, with a median of 13 years. The etiology included 6 cases of Volkmann's contracture, all of which were flexion deformity of the 2nd to 5th fingers, accompanied by limitation of thumb dorsiflexion in 3 cases and limitation of wrist dorsiflexion in 3 cases; 3 cases of pseudo-Volkmann's contracture, including 2 cases of flexion deformity of middle, ring, and little fingers, and 1 case of flexion deformity of ring and little fingers; 8 cases of ulnar finger flexion contracture caused by forearm flexor disease or anatomical variations, all of which were flexion deformity of middle, ring, and little fingers. Operations such as slide of flexor and pronator teres origin, excision of abnormal fibrous cord and bony prominence, and release of entrapped muscle (tendon) were performed. Hand function was evaluated according to WANG Haihua's hand function rating standard or modified Buck-Gramcko classification standard, and muscle strength was evaluated according to British Medical Research Council (MRC) muscle strength rating standard. RESULTS: All patients were followed up 1-10 years (median, 1.5 years). At last follow-up, 8 patients with contracture caused by forearm flexor disease or anatomical variations and 3 patients with pseudo-Volkmann's contracture achieved excellent hand function, with muscle strength of grade M5 in 6 cases and grade M4 in 5 cases. One patient with mild Volkmann's contracture and 3 patients with moderate Volkmann's contracture without severe nerve damage had excellent hand function in 2 cases and good in 2 cases, with muscle strength of grade M5 in 1 case and grade M4 in 3 cases. Two patients with moderate or severe Volkmann's contracture had poor hand function, with 1 case of muscle strength of grade M3 and 1 case of grade M2, which improved when compared with those before operation. The overall excellent and good rate of hand function and the proportion of patients with muscle strength of grade M4 and above were 88.2% (15/17), respectively. CONCLUSION The finger flexion contracture caused by different etiology can be differentiated by analyzing the history, physical examination, radiographs, and intraoperative findings. After different surgical treatments, such as resection of contracture band, release of compressed muscle (tendon), and downward movement of flexor origin, most patients have a good outcome.
Male ; Female ; Humans ; Forearm/surgery* ; Contracture/surgery* ; Ischemic Contracture/surgery* ; Fingers/surgery* ; Muscle, Skeletal/surgery*

OBJECTIVE: To evaluate the effectiveness of neurovascular staghorn flap for repairing defects in fingertips. METHODS: Between August 2019 and October 2021, a total of 15 fingertips defects were repaired with neurovascular staghorn flap. There were 8 males and 7 females with an average age of 44 years (range, 28-65 years). The causes of injury included 8 cases of machine crush injury, 4 cases of heavy object crush injury, and 3 cases of cutting injury. There were 1 case of thumb, 5 cases of index finger, 6 cases of middle finger, 2 cases of ring finger, and 1 case of little finger. There were 12 cases in emergency, and 3 cases with finger tip necrosis after trauma suture. Bone and tendon exposed in all cases. The range of fingertip defect was 1.2 cm×0.8 cm to 1.8 cm×1.5 cm, and the range of skin flap was 2.0 cm×1.5 cm to 2.5 cm×2.0 cm. The donor site was sutured directly. RESULTS: All flaps survived without infection or necrosis, and the incisions healed by first intention. All patients were followed up 6-12 months, with an average of 10 months. At last follow-up, the appearance of the flap was satisfactory, the wear resistance was good, the color was similar to the skin of the finger pulp, and there was no swelling; the two-point discrimination of the flap was 3-5 mm. One patient had linear scar contracture on the palmar side with slight limitation of flexion and extension, which had little effect on the function; the other patients had no obvious scar contracture, good flexion and extension of the fingers, and no dysfunction. The finger function was evaluated according to the total range of motion (TAM) system of the Hand Surgery Society of Chinese Medical Association, and excellent results were obtained in 13 cases and good results in 2 cases. CONCLUSION The neurovascular staghorn flap is a simple and reliable method to repair fingertip defect. The flap has a good fit with the wound without wasting skin. The appearance and function of the finger are satisfactory after operation.
Adult ; Female ; Humans ; Male ; Cicatrix/surgery* ; Contracture/surgery* ; Crush Injuries/surgery* ; Finger Injuries/surgery* ; Plastic Surgery Procedures ; Skin Transplantation/methods* ; Soft Tissue Injuries/surgery* ; Treatment Outcome ; Middle Aged ; Aged

Post-burn contractures are common entities seen in developing countries. There are multiple reasons for the development of contractures, most are preventable. In extensive contractures, a strategic plan is necessary to release all contractures and yet not antagonize post-operative positions. It is also necessary to be cost-effective and minimize the number of surgeries needed. Conventionally the release sequence in extensive burn contractures is proximal to distal. In this case report, we discuss an unusual sequence where we released distal contractures before the proximal to achieve optimum results. A 3-year-old child with post-burn contracture of hand, wrist, elbow, and axilla was treated in 2 stages, with the release of wrist contracture and cover with pedicled abdominal flap in the first stage and division of pedicled flap with the release of axilla and elbow contracture in the second stage. Thus, the release of all contractures was achieved without antagonizing post-operative positions and minimized the number of surgeries. A case-based approach may be crucial in making a strategic surgical plan to minimize the rehabilitation phase, rather than following known dictums.
Humans ; Child, Preschool ; Surgical Flaps/surgery* ; Upper Extremity ; Plastic Surgery Procedures ; Skin Transplantation ; Contracture/surgery*

For many years, surgical treatment of buried penis in children has been researched by several scholars, and numerous methods exist. This study aimed to explore the clinical effect of a modified fixation technique in treating buried penis in children. Clinical data of 94 patients with buried penis who were treated using the modified penile fixation technique from March 2017 to February 2019 in Fujian Maternity and Child Health Hospital (Fuzhou, China) were retrospectively collected, compared, and analyzed. Clinical data of 107 patients with buried penis who were treated using traditional penile fixation technique from February 2014 to February 2017 were chosen for comparison. The results showed that at 6 months and 12 months after surgery, the penile lengths in the modified penile fixation group were longer than those in the traditional penile fixation group (both P < 0.05). The incidence of postoperative skin contracture and penile retraction in the modified penile fixation group was less than that in the traditional penile fixation group (P = 0.034 and P = 0.012, respectively). When the two groups were compared in terms of parents' satisfaction scores, the scores for penile size, penile morphology, and voiding status in the modified penile fixation group were higher than those in the traditional penile fixation group at 2-week, 6-month, and 12-month follow-ups after surgery (all P < 0.05). We concluded that the modified penile fixation technique could effectively reduce the incidence of skin contracture and penile retraction and improve the penile length and satisfaction of patients' parents.
Female ; Pregnancy ; Male ; Humans ; Child ; Retrospective Studies ; Urologic Surgical Procedures, Male/methods* ; Penis/surgery* ; China ; Contracture

Objective: To explore the family rehabilitation model for children with scar contracture after hand burns and observe its efficacy. Methods: A retrospective non-randomized controlled study was conducted. From March 2020 to March 2021, 30 children with scar contracture after deep partial-thickness to full-thickness burns of hands, who met the inclusion criteria, were hospitalized in the Burn Center of PLA of the First Affiliated Hospital of Air Force Medical University. According to the rehabilitation model adopted, 18 children (23 affected hands) were included in a group mainly treated by family rehabilitation (hereinafter referred to as family rehabilitation group), and 12 children (15 affected hands) were included in another group mainly treated by hospital rehabilitation (hereinafter referred to as hospital rehabilitation group). In the former group, there were 11 males and 7 females, aged (4.8±2.1) years, who began rehabilitation treatment (3.1±0.8) d after wound healing; in the latter group, there were 7 males and 5 females, aged (4.6±2.1) years, who began rehabilitation treatment (2.8±0.7) d after wound healing. The children in hospital rehabilitation group mainly received active and passive rehabilitation training in the hospital, supplemented by independent rehabilitation training after returning home; after 1-2 weeks of active and passive rehabilitation training in the hospital, the children in family rehabilitation group received active and passive rehabilitation training at home under the guidance of rehabilitation therapists through WeChat platform. Both groups of children were treated for 6 months. During the treatment, they wore pressure gloves and used hand flexion training belts and finger splitting braces. Before treatment and after 6 months of treatment, the modified Vancouver scar scale, the total active movement of the hand method, and Carroll quantitative test of upper extremity function were used to score/rate the scar of the affected hand (with the difference of scar score between before treatment and after treatment being calculated), the joint range of motion (with excellent and good ratio being calculated), and the function of the affected limb, respectively. Data were statistically analyzed with independent sample t test, equivalence test, Fisher's exact probability test, and Mann-Whitney U test. Results: The differences of scar scores of the affected hands of children in family rehabilitation group and hospital rehabilitation group between after 6 months of treatment and those before treatment were 3.0 (2.0, 7.0) and 3.0 (2.0, 8.0) respectively (with 95% confidence interval of 2.37-5.38 and 1.95-5.91). The 95% confidence interval of the difference between the differences of the two groups was -2.43-2.21, which was within the equivalent boundary value of -3-3 (P<0.05). The excellent and good ratios of joint range of motion of the affected hand of children in family rehabilitation group and hospital rehabilitation group were 3/23 and 2/15 respectively before treatment, and 15/23 and 12/15 respectively after 6 months of treatment. The ratings of joint range of motion of the affected hand of children in family rehabilitation group and hospital rehabilitation group after 6 months of treatment were significantly higher than those before treatment (with Z values of 3.58 and 2.30, respectively, P<0.05), but the ratings of joint range of motion of the affected hand between the two groups were similar before treatment and after 6 months of treatment (with Z values of 0.39 and 0.55, respectively, P>0.05). The functional ratings of the affected limbs of children in family rehabilitation group and hospital rehabilitation group after 6 months of treatment were significantly higher than those before treatment (with Z values of 3.98 and 3.51, respectively, P<0.05), but the functional ratings of the affected limbs between the two groups were similar before treatment and after 6 months of treatment (with Z values of 1.27 and 0.38, respectively, P>0.05). Conclusions: The WeChat platform assisted rehabilitation treatment with mainly family rehabilitation, combined with hand flexion and extension brace can effectively reduce the scarring after children's hand burns, improve the joint range of motion of the affected hands, and promote the recovery of affected limb function. The effect is similar to that of hospital-based rehabilitation providing an optional rehabilitation, treatment method for children who cannot continue to receive treatment in hospital.
Male ; Female ; Humans ; Child ; Cicatrix/therapy* ; Retrospective Studies ; Treatment Outcome ; Wound Healing ; Hand Injuries/rehabilitation* ; Wrist Injuries ; Contracture/etiology* ; Burns/complications*

OBJECTIVE: To evaluate the clinical features, laboratory and imaging results, treatment and outcomes of eosinophilic fasciitis (EF) and assess the value of ultrasound in the diagnosis of EF. METHODS: We retrospectively analyzed the clinical data of 45 patients with EF treated in our center from January 1, 2006 to February 28, 2022. The consistency between the diagnoses of EF based on ultrasound and MRI findings was assessed. RESULTS: In the 45 EF patients (male/female ratio 3.5:1), the age of onset ranged from 16 to 64 years with a mean disease course of 22.6 months. The average time from symptom onset to diagnosis was 16 months. The most common possible trigger of the disease was vigorous exercise (10/45), causing symmetrical lesions in the limbs, most commonly in the forearms (86.7%) and lower legs (80%). Clinical features of EF included subcutaneous swelling and induration (95.6%), arthralgia and arthritis (55.6%), groove sign (42.2%), hand joint contractures (42.2%), skin pigmentation (37.8%), and peau d'orange appearance (13.3%). Eosinophilia was found in 31 patients (68.9%). Hypergammaglobulinemia was seen in 23/44 (52.3%) and positive antinuclear antibodies in 9 (20%) of the patients. Twentyone of the patients were treated with high-dose methylprednisolone (≥200 mg daily for 3 to 5 consecutive days), and compared with the patients who did not receive this treatment, these patients more frequently experienced relapse before admission, had more extensive involvement, and had a higher rate of hypergammaglobulinemia without fever, but these differences were not statistically significant. Of the 31 patients (68.9%) with follow-up data (for a median of 3.2 years [range 0.2-15.9]), complete remission was achieved in 12 (38.7%) patients, and the accumulative complete remission rate was 44.1% at 5.5 years. No specific baseline characteristics or immunosuppressants were found to correlate with the treatment response. A total of 26 patients underwent both ultrasound and MRI examination, and the Kappa value of the diagnostic results between ultrasound and MRI was 0.91. CONCLUSION EF is characterized by symmetrical subcutaneous swelling and induration in the limbs, accompanied by eosinophilia and hypergammaglobulinemia. Glucocorticoid is effective for treating EF. Ultrasound examination can identify thickening of subcutaneous fascia for an early diagnosis of EF.
Humans ; Female ; Male ; Infant ; Child, Preschool ; Retrospective Studies ; Hypergammaglobulinemia ; Eosinophilia ; Ultrasonography ; Hand ; Contracture ; Treatment Outcome

Based on the development of conditions, the etiology and pathogenesis of jingjin (muscle region of meridian) diseases are summarized as 3 stages, i.e. stagnation due to over-exertion at early stage, manifested by tendon-muscle contracture and tenderness; cold condition due to stagnation, interaction of stasis and cold, resulting in clustered nodules at the middle stage; prolonged illness and missed/delayed treatment, leading to tendon-muscle contracture and impairment of joint function at the late stage. It is proposed that the treatment of jingjin diseases should be combined with the characteristic advantages of fire needling and bloodletting technique, on the base of "eliminating stagnation and bloodletting/fire needling". This combined therapy warming yang to resolve stasis and dispels cold to remove nodules, in which, eliminating the stagnation is conductive to the tissue regeneration, and the staging treatment is delivered in terms of the condition development at different phases.
Acupuncture Therapy/methods* ; Bloodletting ; Medicine, Chinese Traditional ; Muscular Diseases/therapy* ; Humans ; Hot Temperature/therapeutic use* ; Contracture/therapy*

Objective: To investigate the clinical features and gene variation characteristics of children with dynein cytoplasmic 1 heavy chain 1 (DYNC1H1) gene associated spinal muscular atrophy with lower extremity predominant (SMALED) 1. Methods: The clinical data of 4 SMALED1 children admitted to Peking University First Hospital from December 2018 to May 2021, who were found to have pathogenic variation of DYNC1H1 gene through genetic testing, except for other genes known to be related to motor retardation, were retrospectively summarized to analyze the phenotype and genotype characteristics. Results: There were 3 males and 1 female. The age of onset was 1 year, 1 day, 1 day and 4 months, respectively. The age of diagnosis was 4 years and 10 months, 9 months, 5 years and 9 months, and 3 years and 1 month, respectively. The clinical manifestations were muscle weakness and muscular atrophy of lower limbs, 2 cases with foot deformity, 1 case with early non progressive joint contracture, 1 case with hip dislocation and 1 case with mental retardation. De novo heterozygous missense variations in DYNC1H1 gene were found in all 4 children. According to the rating of American College of medical genetics and genomics, they were all possible pathogenic and pathogenic variations, with p.R598C, p.P776L, p.Y1109D variations had been reported, and p.I1086R variation had not been reported. Conclusions: For those with unexplained lower limb muscle weakness, muscle atrophy, joint contracture and foot deformity, upper limb motor ability related retention, with or without mental retardation, as well as the motor ability progresses slowly, it is necessary to consider the possibility of SMALED1 and the detection of DYNC1H1 gene when necessary.
Female ; Male ; Humans ; Intellectual Disability ; Retrospective Studies ; Muscular Atrophy, Spinal/genetics* ; Lower Extremity ; Muscle Weakness ; Muscular Atrophy ; Contracture ; Cytoplasmic Dyneins/genetics*