Can acute correction with simultaneous hemiepiphysiodesis of lateral proximal tibia physis prevent recurrence in neglected infantile Blount's disease?

PURPOSE: The treatment of infantile Blount's disease usually includes surgical correction, but high recurrence is still a problem regardless of the procedure. We conducted a cross-sectional study of severely neglected infantile Blount's disease treated with acute correction and simultaneous hemiepiphysiodesis of lateral proximal tibia physis. In this study, we aimed to observe the complication and recurrence. METHODS: This research is an analytical study with a cross-sectional design using retrospective data collection and total sampling. The subjects were patients with neglected infantile Blount's disease treated from 2018 to 2023 in our institution. Follow-up was conducted in 6, 12, 24, and 36 months. RESULTS: A total of 25 legs from twenty patients were recorded. We observed three legs (12.0%) had recurrence. No neurovascular complications and infections were observed. All subjects had significant postoperative improvement of TFA (mean 6.8 ± 0.730 valgus), Drennan angle, MPTA, MTPD, JLCA, and ligamentous laxity grading (p < 0.001). Lower than 5° postoperative valgus overcorrections and preoperative physeal bar were significant factors in patients with recurrence (p = 0.020 and p = 0.010). There was no significant increase in leg-length discrepancy during follow-up (p = 0.052). There were no significant differences between age, BMI, preoperative TFA, pre- and postoperative Drennan angle, MPTA, MTPD, JLCA, Langenskiöld stages, and length of follow-up in patients with recurrence and not. CONCLUSION: Acute correction with simultaneous hemiepiphysiodesis of lateral proximal tibia physis is an effective technique to prevent deformity recurrence in neglected infantile Blount's disease, provided that the postoperative TFA is more than 5° of valgus and no evidence of physeal bar in the preoperative radiograph.

Lessons learned from "the great mimicker disease": A retrospective study of 18 patients with scurvy.

Purpose: Scurvy is an uncommon medical condition that affects children and is caused by an inadequate intake of vitamin C. This study presents the characteristics of patients with scurvy to raise awareness of the diagnostic process in developing countries where laboratory testing for vitamin C levels is often not available. Methods: A retrospective study was performed from period of 2018 to 2023. Data extraction includes patient age, sex, body mass index, constitutional symptoms, musculoskeletal, mucosal, cutaneous symptoms, other accompanying disorders, anemia, erythrocyte sedimentation rate, C-reactive protein, radiographic examination, vitamin C dose, and duration of treatment. Descriptive statistical analysis was performed in this study. Results: Eighteen cases (17 males, 1 female) of scurvy were referred to our institution. Thirteen of 18 patients were misdiagnosed before referral. The median age at presentation was 4.5 (range, 2-11) years. The average body mass index was 13.93 ± 0.63 kg/m2. Half of patients had healthy weight. All patients presented with lower limb pain and 17 of 18 with refusal to walk. The median onset of diagnosis was 11 (range 4-48) weeks. White line of Frankel was described in all patients. Seven had anemia and 6 of 18 had increase in erythrocyte sedimentation rate and/or C-reactive protein levels. Only one patient had ascorbic acid levels evaluation before treatment since it was not readily available in our country. Treatment length varied from 2 weeks to 6 months. Conclusion: The diagnosis of scurvy is frequently delayed due to its extreme rarity in modern society and its ability to mimic numerous other conditions. In children presenting with limb pain and/or reluctance to walk and pathognomonic radiological findings, physicians must prioritize scurvy as a differential diagnosis. In scurvy, vitamin C supplementation is curative.

Caudal regression syndrome from radiology and clinical perspective: A case series and a proposed new integrated diagnostic algorithm.

Caudal regression syndrome (CRS) is a rare inherited disorder associated with orthopedic deformities, as well as urological, anorectal, and spine malformations. We present 3 cases of CRS found in our hospital, along with the respective radiologic and clinical findings of the disease. With different problems and chief complaints from each case, we propose a diagnostic algorithm that can be used as a helpful tool in managing CRS. CRS is a complex and rare congenital disorder that affects multiple systems and can result in a range of malformations. The diagnostic algorithm proposed from our findings from 3 CRS cases is important to help healthcare providers identify the types of CRS and apply a more individualized approach to improve the quality of life for the patient.

The efficacy of ilizarov method for management of long tibial bone and soft tissue defect.

INTRODUCTION: Patients with open fracture Gustillo-Anderson grade 3 had undergone several surgical procedures, but still ended up with expose long dead bone or infected. Illizarov method was used to address long bone and soft tissue defect after re-debridement with radical resection of long dead bone or infected segment. METHODS: We included 14 patients (mean age: 30.86 ± 11.49) with non-union tibial fracture with long dead and infected bone segment who had undergone several debridement, bone grafting or spacer and soft tissue closure procedure due to open fracture of tibia grade 3. The subjects underwent re-debridement with radical resection of dead or infected bone segment followed by Illizarov method to perform bone transport procedure for bone defect filling and simultaneously restore severe soft tissue loss and bone lengthening procedure. RESULTS: All subjects had achieved satisfactory results with mean docking period of bone transport 3.78 ± 0.54 months, union time at the docking side 7 (5.5-9) months. Soft tissue was covered and no recurrence of infection. Three subjects had Leg Length Discrepancy (LLD) of 1 cm, whereas the remaining had zero discrepancy. No significant pain was observed at final follow-up and 4 patients had ankle joint stiffness. CONCLUSION: The Illizarov method can effectively address long bone and soft tissue defects by distraction osteogenesis through bone transport procedure that filling the defect gradually without bone graft and simultaneously enhancing soft tissue closure without tertiary soft tissue procedure subsequently followed with bone lengthening procedure to correct the limb length discrepancy.

Diagnostic and treatment challenge in adult presentation of congenital pseudoarthrosis of the tibia: A case report.

INTRODUCTION: Congenital pseudoarthrosis of the tibia is a rare congenital disease. Late presentation in adult, makes the diagnostic far more challenging and often misdiagnosed as a common non-union fracture with high re-operation rate. In long courses of repeated surgery, non-union persisted along with severe leg length discrepancy. CASE PRESENTATION: A 19-year-old male presented with history of left tibia fracture with repeated surgery. Current problems were progressed bowing of the left lower leg and length discrepancy without recent injury. There was a sign of neurofibroma and pseudoarthrosis at distal third of the tibia shaft with fibula involvement. A radical resection was performed followed by staged deformity correction with Ilizarov's method which was consisted of bone transport procedure in 4 months and lengthening procedure in another 4 months, without grafts. DISCUSSION: After one-year, patient achieved union at docking site, equal lower limb length, good alignment and consolidation in bone transport and lengthening site. Three months after frame removal patient has achieved functional bipedal gait with no sign of recurrence. CONCLUSION: Diagnostic and therapeutic challenge in the late onset of congenital pseudoarthrosis of tibia is in differentiating it with another cause of non-union and in dealing with deformities. Although none of surgical methods have proven their superiority, reconstruction using Ilizarov method is proved to be safe, practical, and effective to solve both problems. However, the patient still needs to be closely observed and protected weight bearing due to the refractory nature of the disease.

Scurvy: Forgotten diagnosis, but still exist.

INTRODUCTION: Scurvy is a rare condition in pediatric patients, resulted from a vitamin C deficiency. Musculoskeletal symptoms occur in 80% of patient. The rarity and the polymorphisms of the clinical signs and symptoms often make scurvy an unknown or forgotten diagnosis. METHODS: A 3-years-old boy with limping gait, progressive pain and swelling on his left lower limb since 3 months prior was diagnosed as osteomyelitis based on MRI. When there was no improvement after 2 weeks of IV antibiotic administration, we did surgical debridement procedure. Intraoperative, we found extensive blood clot inside and in the entire periosteum instead of infection. One week after surgery, there was still no improvement clinically and radiographically. Histopathology result suggested a fibro-osseous tumor. After reviewing clinical sign including skin presentation and plain radiology, we suspected scurvy disease in this patient and started a vitamin C therapy course immediately. RESULTS: Two weeks after we started vitamin C treatment, the patient's condition was significantly improved clinically and radiographically. Serial radiograph was taken after 2 and 12 months of surgery, and it showed significant improvement on the site where the previous pathological process appeared. DISCUSSIONS: Because of the extremely rare occurrence of scurvy in modern society at present, it is difficult to differentiate it from other diseases such as infection. The diagnosis of scurvy is made by careful studies from history taking, physical examination, laboratory and especially radiological finding that show pathognomonic appearance. Examination of vitamin C level serum is a gold standard, but unfortunately not available in our country. Improvement after vitamin C administration also established that condition. CONCLUSION: Scurvy should have established by clinical and radiological findings that show pathognomonic signs around joint, even without supported by vitamin C serum.

Step cut "V" osteotomy for acute correction in Blount's disease treatment: A case series.

INTRODUCTION: Severe Blount's disease results in progressive multiplanar deformity of the lower limb. There are varus, procurvatum, and internal tibia rotational deformity. Treatment varies for each patient, depending on age, magnitude of deformity, discrepancy, psychosocial factor, and surgeon's experience. The aim of treatment is to acutely correct deformity and realign the mechanical axis. We proposed a technique of step cut "V" Osteotomy which could achieve the aim of treatment safely and effectively. METHODS: Twenty-seven legs from eighteen patients were underwent Step Cut "V" Osteotomy technique in our institution from 2015 to 2017 to produce overvalgus correction. Eleven patients had bilateral deformities, and seven patients had unilateral deformity. All patients had mechanical axis deviation (MAD) away medially to the center of the knee, increase of tibiofemoral angle, and Drennan angle. Outcomes were recorded postoperatively such as infection, compartment syndrome, nerve palsy, range of motion, stability of knee ligaments, union time, early weight bearing and recurrence. RESULTS: From this Step Cut "V" Osteotomy, we can accurately measure the angle of correction we want to achieve preoperatively. Drennan angle and Tibiofemoral angle (TFA) of the patients improved with subsequent correction of interbal tibia rotation and procurvatum. No complication of compartment syndrome and nerve palsy were found. Patient could perform early mobilization, and weight bearing on second month after surgery because of strong fixation and good ligament stability. No significant postoperative infection occurred. Union time achieved in two to three months, but four legs from patients over 4 years old who underwent surgery experienced a recurrence. CONCLUSION: Step Cut "V" Osteotomy is a simple, safe and effective technique for acute correction of severe Blount's disease. And can produce an accurate correction, high union rate and early weight bearing with no complication as a result that would be achieved at the end of treatment.