An original preoperative orthopaedic management of a complex spinal deformity in a 16-year-old patient with spondyloepiphyseal dysplasia congenita.

PURPOSE: Spinal deformities in patients with Spondyloepiphyseal Dysplasia congenita are particularly challenging to treat. Addressing these deformities requires a holistic surgical strategy. The aim of this case report is to highlight an original preoperative protocol combining atlantoaxial instability stabilization by halo jacket with progressive correction of a thoracic kyphoscoliosis using continuous elongation by tension plaster cast as described by Stagnara. METHOD: A 16-year-old patient with spondyloepiphyseal dysplasia congenita presenting a severe thoracic kyphoscoliosis associated with atlantoaxial instability causing cervical myelopathy was managed through a preoperative protocol combining a halo jacket with a Stagnara elongation cast allowing progressive correction of the kyphoscoliosis while stabilizing the cervical instability. RESULTS: The preoperative protocol allowed a safer and a more effective surgical spine fusion, reducing neurological risks, improving the final correction, and offering better postoperative recovery. Clinical and radiological solid fusion was observed at 3-year-follow-up with significant improvement of the patient's quality of life. CONCLUSION: This case report highlights the effectiveness of the preoperative preparation combining a halo jacket with a Stagnara elongation cast to safely stabilize the cervical spine while correcting the thoracic kyphoscoliosis. This strategy can serve as a valuable tool for spine teams when addressing complex spinal deformities particularly in this patient with SEDc. LEVEL OF EVIDENCE: Level IV.

Structural scoliosis secondary to thoracic osteoid osteoma: a case report of delayed diagnosis.

PURPOSE: The aim of this case report is to show that late diagnosis of vertebral osteoid osteoma gives rise to structural scoliosis which sometimes requires long-term management. METHODS: We report a case of an osteoid osteoma in the thoracic spine associated with structural scoliosis. We describe a 14-year-old boy who complained chronic nightly left back pain and scoliosis. Spine's X-ray was reported thoraco-lumber scoliosis without bone lesion. RESULTS: MRI as well as technetium-99 m total body bone scan and a computed tomography scan revealed a bony lesion in the upper left joint of T11 vertebra consistent with the diagnosis of OO. Anatomopathological study of the resection piece confirmed the diagnosis of OO. Surgical excision of the tumor resolved pains, but scoliosis needed an orthopedic treatment for 1 year. CONCLUSION: Through this case, it has been demonstrated that late diagnosed vertebral OO can be the cause of structural scoliosis. Clinical and radiological results indicate that OO resection is an effective and safe method of treatment. LEVELS OF EVIDENCE: IV.

Growing rods for early-onset scoliosis in Ehlers-Danlos disease.

PURPOSE: To study the results and complications of Traditional Growing Rods (TGR) for the treatment of Early-Onset Scoliosis (EOS) in patients with Ehlers-Danlos syndrome (EDS). METHODS: This is a retrospective study of patients with EDS treated for EOS between 2012 and 2018 by TGR. For each patient, we evaluated the evolution of the Cobb angle of the main coronal curve before the first surgery, postoperatively and at the last follow-up. We also noted every complication. RESULTS: Four patients have been evaluated. Age at the index surgery ranged from 3- to 6-year-old. All the patients had a Kyphoscoliosis. The preoperative Cobb angle of the main coronal curve ranged from 70° to 104°. Prior to surgery, a progressive correction of the spinal deformity by Halo-gravity traction was performed for all patients. One patient had several complications with a poor result after final fusion. Three patients did not sustain any complication and presented good results. Follow-up ranged from 3 to 8 years. For two of our patients, further lengthening procedures are still needed. CONCLUSIONS: TGR is a good surgical option for the treatment of EOS in EDS. A progressive preoperative correction is recommended. Anchor-related complications must be minimized in these patients with an important kyphosis. Level of evidence Level IV.

Reconstruction of isolated hypoplasia of the patella by a modified Galeazzi procedure: a case report.

BACKGROUND: Isolated Patellar Aplasia Hypoplasia is a very rare autosomal dominant disorder. Its treatment depends on the clinical manifestations that can vary widely. The lack of active extension, which can be responsible for frequent falls due to a knee instability, is the most frequent and disabling manifestation. We report an original technique that is a modification of the Galeazzi technique for recurrent dislocation of the patella to gain active extension in case of PTLAH. CASE REPORT: A 7-year-old Caucasian boy with isolated Patellar Aplasia Hypoplasia and an extension lag of the right knee has been treated by a modified Galeazzi technique. The tendons of the semi-tendinous and gracilis muscles have been harvested and their distal insertion was kept intact. Both tendons were fixed over the top of the patella to restore knee active extension. After 6 years of follow up the patient is symptom free with a strong active extension of the operated knee. CONCLUSION: Reconstruction of isolated hypoplasia of the patella by a modified Galeazzi procedure is a safe and reliable technique for skeletally immature patients offering satisfying long-term outcomes.

Case report: Exome sequencing revealed disease-causing variants in a patient with spondylospinal thoracic dysostosis.

Background: Spondylocostal dysostosis is a rare genetic disorder caused by mutations in DLL3, MESP2, LFNG, HES7, TBX6, and RIPPLY2. A particular form of this disorder characterized by the association of spondylocostal dysostosis with multiple pterygia has been reported and called spondylospinal thoracic dysostosis. Both disorders affect the spine and ribs, leading to abnormal development of the spine. Spondylospinal thoracic dysostosis is a rare syndrome characterized by the association of multiple vertebral segmentation defects, thoracic cage deformity, and multiple pterygia. This syndrome can be considered a different form of the described spondylocostal dysostosis. However, no genetic testing has been conducted for this rare disorder so far. Methods: We report here the case of an 18-month-old female patient presenting the clinical and radiological features of spondylospinal thoracic dysostosis. To determine the underlying genetic etiology, whole exome sequencing (WES) and Sanger sequencing were performed. Results: Using WES, we identified a variant in the TPM2 gene c. 628C>T, already reported in the non-lethal form of multiple pterygium syndrome. In addition, following the analysis of WES data, using bioinformatic tools, for oligogenic diseases, we identified candidate modifier genes, CAP2 and ADCY6, that could impact the clinical manifestations. Conclusion: We showed a potential association between TPM2 and the uncommon spondylocostal dysostosis phenotype that would require further validation on larger cohort.

Cranio-cervical decompression associated with non-instrumented occipito-C2 fusion in children with mucopolysaccharidoses: Report of twenty-one cases.

Background: Mucopolysaccharidosis (MPS) is a multisystemic storage disorder of glycosaminoglycan deposits. Infiltration of the dura mater and supporting ligaments caused spinal cord compression and consecutive myelopathy, especially at the cranio-cervical junction (CCJ). Craniocervical instability and posterior decompression often raise the problem of fixation in children. The main purpose of this paper was to report the result of an original technique of occipito-cervical arthrodesis using a cranial halo-cast system in pediatric population. Methods: We recorded 21 patients with cervical myelopathy. All of them had spinal cord decompression by enlargement of the foramen magnum, C1 laminectomy, and occipito-C2 fusion using corticocancellous bone graft. Only one child has an extended laminectomy from C1 to C3. The occiput-C2 arthrodesis was stabilized by the cranial halo-cast system. This immobilization was performed preoperatively and kept for three months then switched to rigid cervical collar. Clinical assessment, including the Goel grade and mJOA, radiographs and magnetic resonance imaging were performed before surgery. The occipito-cervical arthrodesis was controlled by standard X-rays and CT scan. Results: According to the type of mucopolysaccharidosis, the patients were divided into MPS type I: n= 3, II: n=7, IV: n=11. The mean age of patients at surgery was 6.76 years. All mucopolysaccharidoses cases required a foramen magnum decompression by craniectomy, C1 laminectomy and occipito-C2 arthrodesis. As major complications, a child had immediate post-operative paraplegia due to spinal cord ischemia. The postoperative follow-up ranged from 1.5 to 4 years, with an average of 3.3 years. The average preoperative mJOA score was 8.9, and it improved to 14 points at the last follow-up. Conclusions: Satisfactory fusion and good clinical results were obtained with the 2-stage approach to CCJ anomalies.

Ankle sprain injury in child and adolescent: Diagnostic pitfalls.

Introduction and importance: Sprained ankles in children and adolescents are rare. Certain diagnostic pitfalls must arise in the mind of the clinician because many differential diagnoses must be confirmed or refuted urgently. Among them, the infectious osteoarticular pathology including acute hematogenous osteomyelitis of the tibia or distal fibula and septic arthritis of the subtalar joint.The main purpose of this work, through four clinical cases, we will discuss diagnostic pitfalls in the course of ankle trauma in children and adolescents. Case presentation clinical: In this work, we have demonstrated through four cases some diagnostic errors in the context of ankle trauma in children and adolescents. Infectious and malignant tumor pathologies are real diagnostic and therapeutic emergencies. For all these cases, the initial diagnosis was a sprained ankle following a gambling or sports accident. Discussion: Ankle trauma in children and adolescents is a frequent reason for consultation in emergencies. The fear of the orthopedic surgeon is to miss a serious pathology and wrongly make the simple diagnosis of a sprained ankle. Temperature measurement must be systematic. The clinical examination must be meticulous. The interpretation of the radiograph must be cautious. Acute hematogenous osteomyelitis and malignant tumor pathology should be on the surgeon's mind. Conclusion: We have shown through these four examples that a serious pathology can simulate a sprain. Hence, a careful analysis of clinical, biological and radiological elements is the only guarantee of adequate care.

Acute hematogenous osteomyelitis in children: Management of pandiaphysitis with extensive bone destruction: A case series of thirteen child.

Introduction: and Importance: Acute hematogenous osteomyelitis (AHO) poses a public health problem in severe forms from the outset or with delayed diagnosis. The aim of this work is to describe the management of pandiaphysitis with extensive bone destruction by the Ilizarov external fixator and antibiotics and to evaluate the results. Methods: This is a retrospective, descriptive, cross-sectional and single-center study. It was performed over a seven-year period. We included children with acute hematogenous osteomyelitis complicated by pandiaphysitis of the long bones with extensive bone necrosis. All patients underwent stabilization with an Ilizarov external fixator and antibiotic therapy adapted to the antibiogram in ten cases. One patient had the induced membrane technique. All the patients underwent dynamization of the Ilizarov external fixator followed by immobilization with a cast or a splint for the lower limb. Outcomes: 13 medical files were collected during the study period. The sex ratio was 1.6, the average age was 7.56 years. Three localization sites: humerus for 3 cases, femur for 3 cases and tibia for 7 cases. The germ has been identified in ten cases. Methicillin-resistant staphylococcus aureus was the most common. Bone consolidation is obtained in all cases with an average delay of ten months. Conclusion: The Ilizarov external fixator associated with antibiotics has proven its effectiveness in the management of pandiaphysitis with extensive bone destruction. This method is an alternative in the therapeutic arsenal available to pediatric orthopedic surgeons. Level of evidence: IV, Case series.

Calcaneal lengthening osteotomy in the management of idiopathic flatfoot in children: cCase series of twenty-one feet.

INTRODUCTION: Flatfoot is a frequent reason for consultation in pediatric orthopedics. The calcaneal lengthening osteotomy according to the EVANS technique is a therapeutic alternative. The objective of this work was to evaluate the short and medium term clinical and radiological results of calcaneal lengthening osteotomy in children with idiopathic flat foot valgus. METHODS: This study concerned 12 children and 15 ft treated surgically by calcaneal lengthening osteotomy by an orthopedic surgeon in a pediatric orthopedic surgery center. The evaluation of the results was clinical according AOFAS score and radiological. RESULTS: The deformity was reducible in all of our patients. The mean preoperative AOFAS score was 61, postoperatively 90. The overall result was excellent in 11 cases (11 ft) and good in 4 cases. The postoperative radiological result was close to normal values. CONCLUSION: Calcaneal lengthening osteotomy is a reliable and recommended technique for the correction of symptomatic idiopathic flatfoot. LEVEL OF EVIDENCE: IV, Case series.

Subacute osteomyelitis of the tibial diaphysis associated with Brodie's abscess: A rare case report of a four-year-old child.

INTRODUCTION: Septic osteomyelitis is a hematogenous bacterial bone infection. The acute presentation is the most common; the subacute one is less frequent. The aim of our case report is to put forward the features of this uncommon presentation and to propose a therapeutic management. PRESENTATION OF CASE: We report a rare case of subacute osteomyelitis associated with Brodie's abscess of the tibial diaphysis in a four-year-old child. The chief complaint was a pain in the left tibia evolving for five weeks. The radiological findings and the unusual location of this pathology suggested a malignant bone tumor. Thanks to imaging assessment and bone biopsy the diagnosis of subacute osteomyelitis associated with Brodie's abscess was made. Therefore, the child had antibiotic therapy and plaster immobilization to avoid pathological fracture. Pain relief and radiological improvement was obtained after 3 months of antibiotic treatment. At a two-year follow-up, the X-ray was normal. DISCUSSION: We report an uncommon case of subacute osteomyelitis which evolved to Brodie's abscess with diaphyseal location. The mainstream treatment is surgery combined with antibiotics. However, medical treatment alone can lead to recovery without sequelae. CONCLUSION: Because of the atypical location and presentation of subacute osteomyelitis, the other differential diagnoses, especially malignant bone tumors have to be eliminated. In some cases, treatment may be based on antibiotics therapy alone.

Primary subacute hematogenous osteomyelitis of navicular bone: A rare case report in 7-year-old child.

INTRODUCTION AND IMPORTANCE: Navicular bone location of primary subacute hematogenous osteomyelitis uncommon. There are few cases reported in literature. We aim to describe the clinico-radiological features of primary subacute hematogenous osteomyelitis of the navicular bone in a 7-year-old child, to explain our management of this rare disease and demonstrate that medical treatment without surgery is enough. CASE PRESENTATION: A 7-year-old child presented to emergency department. His chief complaint was fever, left limping and foot pain. The positive examination features of were painful palpation of the dorsal side of the foot and a swelling of the homolateral ankle. The erythrocyte sedimentation rate was high. The x-ray revealed a lytic lesion of the left navicular bone. The MRI findings led to the diagnosis of subacute osteomyelitis. Pain relief and normalization of inflammatory markers were obtained after 8 weeks of antibiotic therapy. Complete radiological healing was obtained after 9 months. One year after treatment, the patient was able to practice sports as previously. CLINICAL DISCUSSION: Subacute osteomyelitis of the navicular bone in pediatric population is a rare condition. This case shows the importance of early diagnosis thanks to MRI findings and appropriate antibiotic therapy based on the endemic bacteriological profile. CONCLUSION: The navicular bone may develop primary subacute osteomyelitis in immunocompetent child. Early diagnosis is important for prescribing effective conservative treatment.

Infected primary hydatid cyst of the right lumbar paraspinal muscles: A rare case in a ten-year-old child.

INTRODUCTION AND IMPORTANCE: Most of the time, intramuscular hydatid cysts are secondary. The unusual topographies are a source of diagnostic delay and treatment difficulties. The aim of our case report is to put forward the features of this uncommon presentation and to propose a therapeutic management. CASE PRESENTATION: We report the case of a primary hydatid cyst located in the right lumbar paraspinal muscles in a ten-year-old girl, in good health and without a pathological medical history. The patient presented with a discreet right lumbar swelling of firm consistency, painless and without signs of inflammation. Ultrasound and magnetic resonance imaging suggested the diagnosis of an intramuscular paravertebral hydatid cyst. The hydatid serology was positive. The patient had en-bloc resection of the cyst. He was infected with pus. The histopathological examination confirmed the diagnosis of muscular hydatidosis. Medical treatment was started. Three years after the operation, the MRI confirmed that was no recurrence. CLINICAL DISCUSSION: Echinococcus is a parasitic cestode that can infect dogs and other pets and farm animals, with humans as incidental hosts. Muscular hydatid localization, secondary to hematogenous dissemination, is rare, even in endemic countries. The combination of medical treatment with surgery ensures the best condition to avoid recurrence. CONCLUSION: In regions where hydatidosis is endemic, a tumor in any part of the body should be considered a hydatid cyst until proven otherwise.

The "Patient management problem" as a formative evaluation during the internship.

INTRODUCTION: The evaluation of the internship in pediatric orthopedics is based on a clinical examination in the form of ECOS, after the internship period involving all surgical specialties. The validation of the clinical examination test does not necessarily mean that the student has achieved his internship objectives and vice versa. This discrepancy between the evaluation test and the field of the internship prompted us to question the need to establish a formative evaluation method during the clinical internship. METHODS: Our study is a prospective, evaluative, longitudinal study, from October 2019 until September 2020, in the infantile orthopedics and traumatology department of the "Béchir HAMZA" children's hospital in Tunis. We carried out a formative evaluation using the "Patient Management Problem" (PMP), with two different subjects, one on acute hematogenous osteomyelitis, the other on malignant bone tumors, with a Pre-test and a Post-test for each PMP. RESULTS: We had 18 interns, during the period between October 2019 and September 2020, including 13 who had medical training in Tunisian faculties. The score for the acute osteomyelitis pretest was 14.56 on average, the PMP score was 16.33 on average, with one interne scoring zero. The posttest mean was 18.11 [13.75-20], with a statistically significant difference from the pretest. The pre-test for the bone tumors' PMP had an average of 9.7 [3.75-15], that of PMP was 12.6 [0-20]. There was an improvement in post test scores with an average of 16.45. Comparing the two tests we noticed that the scores for the PMP osteomyelitis were higher than the scores for the PMP bone tumors with a significant difference between the two groups (p = 0.04). CONCLUSIONS: The results of this pilot project are encouraging for the establishment of a continuous evaluation by the PMP method, intended for interne during the pediatric orthopedic surgery internship. This method allows learning and formative evaluation of learners by evaluating clinical and therapeutic reasoning.

Isolated tuberculosis of the navicular bone in a child.

Tuberculosis is a topical issue in endemic countries. Foot involvement is rare and accounts for 5-10% of all osteoarticular tuberculosis. Pediatric cases of osteoarticular tuberculosis are uncommon especially in well vaccinated children. The clinical presentation is not specific and can be confused with common germ like staphylococcus. Biopsy is mandatory in endemic zone, and if the evolution is not good with antibiotherapy. Debridement can be indicated but must be gentle, because of the risk of navicular necrosis.

Management of septic non union and discrepancy of humerus in a child: A case report.

INTRODUCTION: The management of large bone defect in the upper limb is fraught with difficulties and problems. It's is a long course treatment which include many components: infection, osseous loosening and shortening. CASE PRESENTATION: We present our experience of an 12-years-old boy with septic non union of the proximal left humerus with length discrepancy of 6 cm treated with Ilizarov fixator(IL) followed by vascularized fibula graft (VFG) with a good clinical result without complications. DISCUSSION: The bone loss may be treated with allografts, bone transfer and bone substitute. Consideration of the quality of bed tissue and the size of the bone defect are condition to ensure a good result. CONCLUSION: Ilizarov fixator is an attractive and the most used technique for solving the problem of discrepancy and stabilizing in the upper limb.

Congenital pseudarthrosis of the forearm treated with induced membrane technique: A case report.

Congenital pseudarthrosis of the forearm (CPF) is an uncommon entity frequently described in association with neurofibromatosis type 1. Surgical treatment Is required. This is a challenging pathology in pediatric orthopedic surgery due to the difficulty of achieving a satisfying result in terms of bone defect healing. Many techniques are described such us free vascularized fibula, illizarov and induced membrane. We describe a first case of the forearm non union associated with neurofibromatosis type 1 successfully treated with 2 steps of induced membrane. Whatever the technique selected it's remain challenging problem in pediatric orthopaedic.

[The proximal humeral osteotomy associated with the transfert of Latissimus Dorsi and Teres major in treatment of sequelae of the obstetrical brachial plexus]. / L'ostéotomie humérale proximale associée au transfert du latissimus dorsi et teres major dans le traitement de certaines séquelles tardives de la paralysie obstétricale du plexus brachial.

BACKGROUND: Shoulder sequelae of obstétrical brachial plexus palsy put a different problem of coverage according to the age of the patients and the presence or not of ostéo-articular deformations. At an advanced age and in the presence of ostéo-articular deformations, the muscles liberation and transfers tendineux only are insufficient for the restoring of a satisfactory function to the paralytic shoulder. AIM: The purpose of this study is to report an original technique by the association of libertation of retracted muscles and a humeral osteotomy to improve the abduction and to acquire an active external rotation in internal rotation retraction of the shoulder sequelae of obstétrical brachial plexus palsy. METHODS: This retrospective study concerned twelve patients admitted in the service of Childish Orthopaedics of Children's hospital of Tunis between 1997 and 2003. The average age of the patients are 11 years. All the patients have a proximal humeral osteotomy above the deltoïdien V with a desinsertion of the Subscapularis and to the transfer of the Latissimus Dorsi and Teres Major, realized by a single posterior approch. RESULTS: After a mean follow up of 48 month a frank aesthetic and functional improvement was noted in every case. CONCLUSION: The importance of retraction for an advanced age, made that an humeral osteotomy of external derotation is necessary. Have a practice above the deltoïdien V it allows to improve at the same time the external rotation and the abduction due to the lateral translation of the deltoid. This last one is strengthen by the désinsertion of a retracted Subscapularis and levying of the co-contractions of the Latissimus Dorsi and Teres major with the deltoid almost constant.