Is Using a Ring External Fixator in the Treatment of Congenital Pseudarthrosis of the Tibia Associated With Better Results or Using a Locking Plate?

BACKGROUND: Congenital pseudarthrosis of the tibia (CPT) is a rare disease. CPT is often unilateral and occurs between the middle and distal third of the tibia. Concurrent involvement of the fibula is present in more than half of cases. histologic studies indicate the presence of fibrous hamartoma tissue and a sick periosteum, which leads to recalcitrant bone fracture and, eventually, pseudoarthrosis. Although there are various surgical techniques, we intend to compare the 2 methods of external fixation versus internal plating. METHODS: Demographic data were collected from 26 patients with frank pseudoarthrosis. After exclusion criteria, patients were compared in groups A (12 patients) and B (11 patients). Resection of hamartoma and sclerotic bone, intramedullary rodding and autologous bone, and periosteal grafting were performed for all patients. In group A, we used a ring external fixator for compression and rotational stability, but in group B, a locking plate was used for these purposes. RESULTS: Plating takes less time to use during surgery. In group A, the primary bony union was obtained in 67% of patients, while in group B, 82% of patients had a primary union. Meanwhile, the average time till the final union in group A was 6 months, while in group B, this time was 3.5 months. Positive union mass was obtained in 58% of the patients in group A and 82% of group B. In addition, plating prevented ankle valgus deformity in group B. CONCLUSIONS: Permanent intramedullary rodding is a surgical requirement for correction of deformity and refracture prevention, but additional stability can be achieved with the use of a ring external fixator or internal plate. Cross union and positive union mass are 2 important factors in the treatment of pseudoarthrosis; these results are achieved to a greater extent and in a shorter period of time using the plate. LEVEL OF EVIDENCE: level IV - case series.

A Method of Time Prediction in Epiphysiodesis for Limb Length Discrepancy Based On Multiplier.

Objectives: The most critical step in the calculation of final limb length discrepancy (LLD) is estimating the length of the short limb after skeletal maturity(Sm). Paley's multiplier method is a fast, convenient method for calculating Sm and LLD after skeletal maturity; nonetheless, the calculation of the process of Sm and LLD in acquired type cases is complex in contrast to congenital type in this method. Notwithstanding, the multiplier method uses a variable called "growth inhibition" for the calculation process in acquired type LLD; however, its mathematical proof has not been published yet. The present study aims to find out whether there is an alternative way to estimate the length of Sm and LLD in skeletal maturity without using growth inhibition (GI) and its complex calculation process in acquired type LLD. Methods: We used trigonometric equations to prove the GI concept and conducted proportionality analysis to calculate the length of short limbs and LLD in skeletal maturity without using GI. Results: Based on the results, the following proportionality can estimate the length of the short limb in skeletal maturity. (ΔLm/ΔL = ΔSm/ΔS). Conclusion: The GI concept can be proved trigonometrically; nonetheless, its numerical value is not necessary for estimating the length of the short limb in skeletal maturity. Instead, a simple proportionality analysis serves the purpose of calculation.

A new case of Melnick-Needles syndrome with skeletal manifestations: A case report.

INTRODUCTION AND IMPORTANCE: Melnick-Needles syndrome (MNS) is a rare skeletal dysplasia that affects skeletal and connective tissue. Less than 70 cases of MNS reported in the literature. MNS had various clinical manifestations such as skeletal deformity, cortical bony sclerosis, facial abnormality, and urogenital symptoms. CASE PRESENTATION: We presented a 5-year-old girl who referred to our orthopedic clinic with knee valgus deformity, spinal kyphoscoliosis, bilateral coxa valga, and humerus cortical irregularity. Based on some facial and skeletal feature, MNS was confirmed with genetic evaluation (heterozygote Filamin A genome). CLINICAL DISCUSSION: The diagnosis of MNS requires a thorough medical and family history, physical examination, and radiographic evaluation. Differential diagnoses for patients with skeletal and facial deformities like MNS include Camurati-Engelmann disease, cystinuria, Galloway-Mowat syndrome, Joubert syndrome, and mucopolysaccharidosis. Treatment for MNS patients with bony deformities without lethal conditions can be conservative, but corrective surgery may be necessary in some cases. CONCLUSIONS: MNS was a rare syndrome with common clinical manifestations such as limb and spine deformity. It is important to conduct a careful examination of any patient who presents with limb and skeletal deformity to the orthopedic clinic, as the disease may have some lethal clinical implications.

Refracture, a Potential Complication of the Periosteal Flap Technique Without Bone Graft for Isolated Congenital Pseudarthrosis of the Fibula: A Report of Two Cases and Literature Review.

BACKGROUND: It is a rare condition defined by congenital pseudarthrosis of the fibula (CPF), which may be associated with neurofibromatosis (NF) and congenital pseudarthrosis of the tibia. A condition such as this may be symptomatic or asymptomatic, but it requires treatment because of the progression of signs such as ankle valgus and pseudarthrosis of the tibia. There are a number of surgical techniques that can be used to treat this condition, such as tibiofibular fusion, internal fixation, the Ilizarov method, and periosteal flap. AIM: The goal of this study was to describe the treatment results of two patients with CPF using vascularized fibular periosteal flaps. CASE REPORT: We described the case of a 5-year-old patient and a 19-month-old patient with isolated CPF. Both patients underwent a distal-based vascularized fibular periosteal flap, and intramedullary fixation was used to treat the patients. CONCLUSION: The patients had full union in the pseudarthrosis site, but in the end, both had asymptomatic refracture in the union site. Our experiences showed that it is necessary to use strong intramedullary fixation and bone graft.

Congenital hypoplastic thumbs treated by staged nonvascularized MTP joint transfer for absent MCP joints and abductor digiti minimi tendon transfer for opposition: a case series study.

BACKGROUND: We developed a 2-stage, MTP (metatarsophalangeal) joint- plus ADM (abductor digiti minimi) tendon-transfer, procedure for treatment of hypoplastic thumb. This method is intended to achieve both structural and functional goals of reconstruction. Structurally, it preserves a five-digit hand with minimal donor site complications. Functionally, it provides a functioning opposable thumb. CASE PRESENTATION: The case series included 7 patients with type IV hypoplastic thumb. At the first stage non-vascularized joint (not bone) was transplanted. In the second stage abductor digiti minimi tendon was transferred. Patients were followed for a median 5-yr period (range: 37-79 months). Functional outcome was assessed using a modified Percival assessment tool. Participants aged 17 to 36 months at the time of surgery with (2 male, 4 female). All patients were able to grasp large and small objects after the procedure. The thumb tip could actively move to touch the tips of index (2 patients) middle, ring, and little fingers (all patients) in an ulnar ward sequence and vice versa. All patients attained the ability to do lateral, palmar, and tripod pinch. As for donor site complications, none of the patients were found to have difficulty walking or keeping their balance. CONCLUSIONS: An alternative surgical procedure was developed to reconstruct a hypoplastic thumb. We obtained a good functional and cosmetic outcome with few donor site complications. Future studies will be needed to determine the long-term outcomes, to refine the selection criteria and to examine the necessity of additional procedure at the older ages.

Extensive Deep Tissue Involvement in Nicolau Syndrome and Below-Knee Amputation: A Case Report and Literature Review.

Nicolau syndrome (NS) is a rare cutaneous drug reaction in response to injections administered via any route. Based on the available studies in the medical literature, NS presents as skin and subcutaneous fat necrosis, and typically, it does not cause severe complications such as acute limb ischemia or death. In this study, we report the case of a 6-year-old boy who received an intramuscular injection of benzathine penicillin G for the treatment of bacterial pharyngitis, and subsequently developed a severe case of NS, which eventually led to below-knee amputation of the right lower limb. Although a few approaches have been suggested for the management of NS, they might not be effective under certain circumstances. Early detection, close monitoring, and consistent interventions, such as surgical fasciotomy and debridement procedures, might be necessary in severe cases of NS.

Glenoid osteotomy with various tendon transfers for brachial plexus birth palsy: clinical outcomes.

BACKGROUND: Posterior shoulder dislocation is one of the disabling complications of brachial plexus birth injury (BPBI), and various treatment options including capsule and surrounding muscles release for open reduction, humeral derotational osteotomy, and tendon transfers have been recommended to manage it. In the present study, we aimed to determine the clinical outcome of open reduction with soft tissue release, tendon transfer, and glenoid osteotomy in patients with BPBI and posterior shoulder dislocation or subluxation. METHODS: From 2018 to 2020, 33 patients who underwent open reduction, glenoid osteotomy, and tendon transfer were included. The glenohumeral deformity was classified according to the Waters radiographic classification. Functional assessment was performed using the Mallet grading system before and at least 2 years after the surgery. RESULTS: The patients were monitored for 26.88 ± 5.47 months. Their average age was 27.5 ± 14 months. Significant improvement was seen in the overall Mallet score (from 13.5 to 18.91 points) and its segments including hand-to-mouth, hand-to-neck, global abduction, global external rotation, abduction range of motion (ROM), and external rotation ROM. Hand-to-back score and the presence of a Trumpet sign were significantly decreased in the postoperation phase (all P values < .001). The above-mentioned variables significantly changed for both infantile and noninfantile dislocations. CONCLUSION: Our study demonstrated that open reduction along with glenoid osteotomy improves retroversion, and muscle strengthening with different muscle transfers is an effective technique for BPBI.

Clinical, radiological, pedobarographic, and quality of life outcomes of the Ponseti treatment for clubfoot: a prospective study.

BACKGROUND: Talipes equinovarus, also known as clubfoot, is a congenital anomaly that affects one newborn per 1000 live births. Its standard treatment strategy is Ponseti casting management. This study aims to report the long-term outcomes of the Ponseti treatment in Iran. METHODS: A prospective cohort study was enrolled to evaluate clinical outcomes, radiological results, pedobarographic measurements, and quality of life after the Ponseti treatment in patients with clubfoot who were followed for at least five years. RESULTS: In this study, 25 clubfeet of 18 patients were included. Significant reductions in Pirani, Dimeglio, and CAP scores, improved ankle dorsiflexion, and acceptable pedobarographic indices were observed in this study. From the radiological evaluation indices, the calcaneal pitch and lateral talus-first metatarsal angles were significantly reduced. After five years of treatment, patients' quality of life was favorable, which was better in females. This study showed that the results of the Ponseti treatment remained acceptable after five years. CONCLUSIONS: The Ponseti management for clubfoot in the long term appears to maintain significant improvements. However, the recurrence rate - albeit without disruption to daily activities - cannot be ignored.

Periscapular Fibromatosis With Intrathoracic Invasion in a 3-Year-Old Boy.

Fibromatosis is an extremely rare tumor that arises from the myofascial tissue and has high rates of infiltration and recurrence. Fibromatosis of the chest wall is even less common, and wide surgical resection is the preferred treatment alternative to radiation therapy, chemotherapy, and systemic treatments. We report the case of a 3-year-old boy with radiologically and pathologically confirmed fibromatosis of the periscapular region who underwent wide resection. We discuss diagnosis, treatment options, and technical pearls for a desmoid tumor of the chest wall. The resection should go through healthy tissue because of the infiltrative nature of the tumor. Although visceral involvement did not occur in this case, surgeons should be aware of its possibility, and surgery should be performed by a multidisciplinary team, including a pediatric orthopedic surgeon, a thoracic surgeon, and an anesthesiologist. [Orthopedics. 2022;45(5):e276-e279.].

Langenskiöld classification of tibia vara: a multicenter study on interrater reliability.

Since 1952, when Langenskiöld introduced his six-stage classification on roentgenographic morphologies of tibia vara, this method has been used extensively in Orthopaedic studies. Different studies have tried to determine the reliability of this method. Although there are scarce reports of major variability in using Langenskiöld classification, but the question still remains, as to whether using expert subspecialists and MRI would improve its reliability. The aim of this study is to evaluate inter-rater reliability of Langenskiöld classification using simple radiograph and MRI, by expert raters. This is a cross-sectional multicenter study, involving patients from 4 tertiary pediatric orthopedic centers in France and Iran. Radiograph and MRI (T1, T2, fat saturation) of the affected knees were independently classified by 12 academy members from France and Iran: six pediatric fellowship orthopedic surgeons and six musculoskeletal radiologists. All data were analyzed afterwards by an independent researcher. Mean weighted kappa for agreement based on radiograph and MRI was 0.47 and 0.45, respectively. Mean percent of total agreement was 46.6 and 40.8% in the same order. Kappa statistics for the new grouping were 0.53 and 0.46 for roentgenograms and MRIs, respectively. The new regrouping was proposed so that each group would need a different treatment strategy. Fleiss kappa statistics for group B (stages 2 and 3), rose from (0.33 and 0.09) to 0.44. These values for group C (stages 4 and 5) rose from (0.42 and 0.11) to 0.54. This study concludes that Langenskiöld's classification when used by experts, still has a moderate inter-rater reliability at best. This variability is most at stages 2, 3, and 4; and can cause different treatment approaches. Use of MRI does not have a significant effect on its reliability. Regrouping the stages improved the inter-rater reliability. Level of evidence: III.

Chronic excruciating forearm pain in a child with intra-neural hemangioma: A challenging case report.

INTRODUCTION AND IMPORTANCE: Cavernous hemangioma is a rare form of hemangioma. It usually arises in the central nervous system, but the tumor has also been reported in the liver, retina and skin with a lower prevalence. Its occurrence into the peripheral nerves has only been reported a few times. Herein, we report an extremely rare case of intra-neural hemangioma in the ulnar nerve and discuss the complications we faced following surgery. CASE PRESENTATION: We present a 6-year-old boy with history of severe progressive left forearm pain in the last two years. Imaging studies revealed a soft tissue mass and histopathological exam was in favor of a cavernous hemangioma. Patient underwent surgery to excise the tumor. Despite temporary response, he began to experience excruciating pain shortly after surgery which caused him to adopt bizarre postures. CLINICAL DISCUSSION: In the more common form of nerve involvement in a hemangioma, the nerve is displaced and surrounded by the tumor. However, in cases with intra-neural involvement, the nerve would have to be sacrificed. This case report brings some rare but important characteristics of a hemangioma in to attention, such as the intra-neural location, possibility of recurrence and aggravating pain with bizarre positions as a result. CONCLUSION: In cases of intra-neural hemangioma, there is a chance that the patient experiences recurrence and/or excruciating pain following surgery. The orthopedic surgeon should be prepared for the possibility of nerve transfer, repeat surgeries and the need for prolonged palliative pain suppression modalities in the approach to intra-neural hemangioma.

Prognosis of Congenital Pseudarthrosis of the Tibia: Effect of Site of Tibial Pseudarthrososis and Fibular Involvement.

BACKGROUND: Congenital pseudarthrosis of the tibia is a rare condition that has long been one of the most challenging concerns in pediatric orthopedic surgery. When the fracture occurs, a recalcitrant nonunion is expected. This is why successful treatment means maintaining a long-term union. In this study, we aimed to assess the therapeutic outcomes for the middle and distal third fractures of the tibia and to explore whether the treatment of concurrent fibular pseudoarthrosis affects the outcome. METHODS: We studied 12 patients with congenital pseudarthrosis of the tibia (Crawford type 4) from 2014 to 2019. A combination approach including intramedullary rod, Ilizarov apparatus, corticocancellous bone graft, and periosteal graft was used. In the initial surgery, we did not fix the ankle and subtalar joints. RESULTS: As a result, the union was achieved in 67% of the cases after the index surgery. All of the cases with primary nonunion were related to the concurrent fibular and distal third tibial pseudarthrosis. In addition, ignoring the treatment of fibular pseudarthrosis in the index surgery led to ankle valgus deformity both in the middle and in the distal third tibial pseudarthrosis. We finally achieved a 100% union rate in all cases, with no subsequent refracture. CONCLUSIONS: Surgery at an early age was associated with favorable results and minimized deformity. In concurrent fibular and distal third tibial pseudarthrosis, it is recommended to transfix the ankle and subtalar joints to create a cross-union with fibula so that the intense union can be confidently obtained with more cross-section to prevent ankle valgus deformity. LEVEL OF EVIDENCE: Level IV-case series.

The path to minimizing instability in developmental dysplasia of the hip: is Capsulorrhaphy a necessity or a futile habit?

BACKGROUND: To evaluate and quantify the intraoperative effect of capsulorrhaphy on the deep seating of femoral head within the acetabulum as measured by medial joint space, a surrogate measure of acetabular-head contact. METHODS: In order to determine the exact effect of capsulorrhaphy, we prospectively scrutinized a consecutive sample of 18 patients with unilateral dysplastic hips aging > 18 months and followed them for a period of at least 12 months. The procedure of open reduction is described in detail. Two pediatric orthopedists carried out the operations from August 2014 to January 2019 at a tertiary pediatric hospital. Intraoperatively, AP radiographs of the pelvis were obtained before and after capsulorrhaphy. The distance between the inferomedial edge of the proximal femoral metaphysis and the lateral edge of the obturator foramen was recorded. To determine if there were differences in medial joint space due to capsulorrhaphy, a generalized linear model was run on the study sample. All patients were followed for at least 12 months to determine the rate of re-dislocation. RESULTS: Mean age (±standard deviation) of the participants was 37.5 (±24.7) months. All cases underwent Salter osteotomy, 5 cases needed femoral shortening (27.8%) and none needed derotational osteotomy. Capsulorrhaphy lead to a statistically significant decrease in the mean medial joint space from 1.59 cm before (95% CI: 1.12-2.05) to 0.76 cm after (95% CI: 0.50-1.02) the capsulorrhaphy (P < 0.001). When we took the effect of age into account the corresponding figures were 1.47 (95% CI: 1.22-1.75) and 0.67 (95% CI: 0.39-0.94), respectively (P < 0.001). After follow up periods of 1 to 5.5 years, none of the patients experienced instability or re-dislocation. CONCLUSIONS: Capsulorrhaphy, independently, of age was associated with a 1-cm decrease in the mean medial hip joint space and a more deeply seated femoral head. Furthermore, this study presents a successful experience with capsulorrhaphy to prevent hip instability.

Orofaciodigital syndrome type II (Mohr syndrome): a case report.

BACKGROUND: Orofacial digital syndrome is a rare genetic disorder with oral cavity, facial and digits anomalies. Orofacial digital syndrome type II, also called the "Mohr syndrome" is a very rare subtype that has been reported scarcely in Asia especially in Japanese patients. CASE PRESENTATION: The case is an Iranian 5-year old girl who had been admitted for orthopedic surgery. She surprisingly had pre and postaxial polydactyly of all the four limbs concurrent with syndromic face and most of the features of Orofaciodigital syndrome type II. CONCLUSION: Mohr syndrome, anesthesia and surgical considerations are discussed in this case report. It is recommended to consider these considerations and the possibility of OFDS in every child with pre and postaxial polydactyly of the four limbs and to try to distinguish type II from other types of ODFS.

Anterior Distal Hemiepiphysiodesis of Tibia for Treatment of Recurrent Equinus Deformity Due to Flat-Top Talus in Surgically Treated Clubfoot.

Treatment of persistent or recurrent equinus after repeated surgical releases can be challenging in patients with clubfoot. Anterior distal tibial epiphysiodesis has recently been used in patients with recurrent progressive equinus deformity, with inconsistent outcomes. Herein, we used this technique in a carefully selected subgroup (8 children, 9 feet) of patients with a severe equinus deformity and a flat-top talus. The patients were followed up with radiological and clinical measures for 12 to 18 months. The mean angle of the ankle improved significantly (25.5°, p < .0001). The mean anterior distal tibial angle decreased from 86.3° to 69° (p < .0001). Plantigrade foot was obtained in all patients, except 1 with arthrogryposis. When applied to carefully selected patients, anterior distal hemiepiphysiodesis of the tibia is an effective method for management of recurrent equinus deformity.

The Utility of Leukocyte Esterase Strip Test in the Diagnosis of Pediatric Septic Arthritis.

BACKGROUND: Most tests used to diagnose pediatric septic arthritis are either not accurate or do not produce rapid results. A leukocyte esterase (LE) strip test has previously been validated for the diagnosis of adult native and periprosthetic joint infections. The purpose of this prospective study was to: (1) evaluate the performance characteristics of the LE strip test in the diagnosis of pediatric septic arthritis and (2) determine the false positive rate of LE strip test on the aseptic synovial fluid (SF). METHODS: Between May 2016 and November 2018, SF was obtained from children who were hospitalized at our tertiary referral center on the basis of suspicion of septic arthritis. All patients underwent arthrocentesis, and the aspirate was tested with LE strip test, leukocyte count, and culture. Twenty-five patients satisfied the inclusion criteria. For the second part of the study, SF from 25 children undergoing surgery for developmental dysplasia of the hip was collected and tested with LE strip test, leukocyte count, and culture. RESULTS: In the first part of this study, 19 joints were classified as septic and 6 as aseptic. Considering a positive LE strip test ("++" and "+++" readings) indicative of septic arthritis yielded a sensitivity of 100%, specificity of 83%, positive predictive value of 95%, and negative predictive value of 100%. In the second part, all 25 patients with an aseptic SF had a negative test result ("-" and "+" readings). CONCLUSIONS: The LE strip test seems to be a valuable additional tool in the diagnosis of pediatric septic arthritis. The LE strip test has the advantages of being inexpensive and simple, providing real-time results and having a perfect negative predictive value to rule out the diagnosis of septic arthritis. LEVEL OF EVIDENCE: Level II-diagnostic.

Is bipolar latissimus dorsi transfer a reliable option to restore elbow flexion in children with arthrogryposis? A review of 13 tendon transfers.

BACKGROUND: Scant data are published about different available therapeutic options for restoration of active elbow flexion in arthrogryposis with varying degrees of improvement. In this retrospective study we evaluated medium-term effects of bipolar latissimus dorsi transfer in patients with arthrogryposis. METHODS: Pedicled latissimus dorsi bipolar muscle transfer was used to restore elbow flexion in 11 patients (13 limbs) with arthrogryposis. Elbow and shoulder range of motions and strength of elbow flexion were measured preoperatively and at the last follow-up. The functional use of the upper limb was examined by observation of activities of daily living and presence of adaptive mechanisms. General satisfaction was assessed at the final follow-up. RESULTS: The patients were a mean age of 5.69 ± 2.49 years. The follow-up period was 27.31 ± 17.8 months. At the last examination, according to transferred muscle function and elbow range of motion, function of 12 limbs was graded as excellent and good, and 1 was graded poor. The active postoperative elbow range of motion was 97.7° ± 34.5°. The general satisfaction of the patients with the surgical results was 92.3%. Activities of daily living were improved in 10 of 13 limbs, but adaptive mechanisms were detected in all patients except 2 at the last follow-up. CONCLUSIONS: We suggest pedicled bipolar latissimus dorsi transfer as a reliable therapeutic option to restore active elbow flexion in arthrogryposis with acceptable results regarding regaining range of motion and patient satisfaction. Functional passive elbow range of motion and good quality of latissimus dorsi muscle preoperatively is mandatory.

Comparison of Pediatric and General Orthopedic Surgeons' Approaches in Management of Developmental Dysplasia of the Hip and Flexible Flatfoot: the Road to Clinical Consensus.

Developmental dysplasia of the hip (DDH) and flatfoot are common pediatric orthopedic disorders, being referred to and managed by both general and pediatric orthopedic surgeons, through various modalities. Our study aimed to evaluate their consensus and perspective disagreements in terms of diagnostic and therapeutic approaches of the mentioned deformities. Forty participants in two groups of general orthopedic surgeons (GOS) (n=20) and pediatric orthopedic surgeons (POS) (n=20), were asked to answer an 8-item questionnaire on DDH and flexible flatfoot. The questions were provided with two- or multiple choices and a single choice was accepted for each one. Chi-square and Fisher's exact tests was performed to compare the responses. For a neonate with limited hip abduction, hip ultrasonography was the agreed-upon approach in both groups (100% POS vs 71% GOS), and for its interpretation 79% of POS relied on their own whereas 73% of GOS relied on radiologist's report (P=0.002). In failure of a 3-week application of the Pavlik harness, ending it and closed reduction (57% POS vs. 41% GOS) followed by surgery quality assessment with CT scan (64% POS vs. 47% GOS) and without the necessity for avascular necrosis evaluation (79% POS vs. 73% GOS) were the choice measures. In case of closed reduction failure, open reduction via medial approach was the favorite next step in both groups (62% POS and 80% GOS). For the patient with flexible flat foot, reassurance was the choice plan of 79% of pediatric orthopedists. Our findings demonstrated significant disagreements among the orthopedic surgeons. This proposes insufficiency of high-level evidence.