Conservative treatment of congenital radial head subluxation and non-traumatic elbow locking: A case report.

Congenital radial head subluxation is relatively rare and may be overlooked due to mild symptoms. The diagnosis mainly relies on imaging and history. Observation is an option for those with insignificant symptoms, while surgical intervention, such as ulnar osteotomy or arthroscopy, is often required when dysfunction exists. A 30-year-old man was admitted with congenital radial head dislocation, which was treated with manipulative repositioning. During follow-up, the patient regained the original mobility of the elbow joint and had no recurrence of dislocation. In conclusion, in adults with congenital dislocation of the radial head, we recommend conservative treatment as a first step.

Congenital Knee Dislocation at Birth - An Extraordinary Case of Spontaneous Reduction / Luxação congênita do joelho no nascimento - Um caso extraordinário de redução espontânea

Abstract Congenital knee dislocation (CKD) is a rare malformation characterized by hyperextension deformity of the knee with anterior tibia displacement, present at birth. Rarely reported, CKD might occur as an isolated deformity or commonly associated with musculoskeletal abnormalities, with the most common ones being developmental dysplasia of the hip (DDH) and clubfoot. The etiology is unknown, but CKD has been associated with certain intrinsic and extrinsic factors. Treatment with conservative methods at an early stage is most likely to yield successful results. We report here a rare case of successful spontaneous reduction of CKD in an infant within 24 hours of life.

Resumo A luxação congênita do joelho (LCJ) é uma malformação rara caracterizada por deformidade de hiperextensão do joelho com deslocamento anterior da tíbia, presente ao nascimento. Raramente relatada, a LCJ pode ocorrer como uma deformidade isolada ou comumente associada a anormalidades musculoesqueléticas, sendo as mais comuns a displasia do desenvolvimento do quadril (DDQ) e o pé torto congênito (PTC). A etiologia é desconhecida, mas a LCJ foi associada a certos fatores intrínsecos e extrínsecos. O tratamento com métodos conservadores em um estágio inicial tem maior probabilidade de produzir resultados bem-sucedidos. Relatamos aqui um caso raro de redução espontânea bem-sucedida de LCJ em um bebê nas suas primeiras 24 horas de vida.

Anesthetic management for cesarean delivery in a woman with congenital atlantoaxial dislocation and Chiari type I anomaly: a case report and literature review.

BACKGROUND: The preferable choice of anesthesia for the patients with congenital atlantoaxial dislocation (CAAD) and type I Arnold Chiari malformations (ACM-I) has been a very confusing issue in clinical practice. We describe the successful administration of combined spinal-epidural anesthesia for a woman with CAAD and ACM-1 accompanied by syringomyelia. CASE PRESENTATION: Our case report presents the successful management of a challenging obstetric patient with CAAD and ACM-1 accompanied by syringomyelia. She had high risks of difficult airway and aspiration. The injection of bolus drugs through the spinal or epidural needle may worsen the previous neurological complications. The patient was well evaluated with a multidisciplinary technique before surgery and the anesthesia was provided by a skilled anesthesiologist with slow spinal injection. CONCLUSIONS: An interdisciplinary team approach is needed to weigh risks and benefits for patients with CAAD and ACM-1 undergoing cesarean delivery. Therefore, an individual anesthetic plan should be made basing on the available anesthetic equipments and physicians' clinical experience on anesthetic techniques.

Single oblique osteotomy for correction of congenital radial head luxation with concurrent complex angular limb deformity in a dog: a case report.

A 5-month old Shih Tzu was diagnosed with congenital elbow luxation and uniapical complex angular deformity of the radius. Single radial oblique and dynamic ulnar osteotomies were performed, using patient-specific 3D-printed osteotomy guide. External skeletal fixation was maintained for three weeks to prevent re-luxation of elbow joint. Three months after the surgery, objective gait analysis indicated markedly improved limb function. In addition, radiograph showed improved congruity of elbow joint and appropriate bone healing. In dogs with congenital radial head luxation and concurrent complex angular deformity, a single oblique osteotomy might be a viable option to preserve bone length and correct the luxation of elbow joint.

Double ulnar osteomy for the treatment of congenital radial head dislocation.

OBJECTIVE: The aim of this study was to retrospectively evaluate the effects of our double osteotomy technique in the treatment of congenital radial head dislocation (CRHD). METHODS: A total 14 children (14 elbows; 71.42% male; mean age: 9.31 ± 3.06 years) with CRHD who underwent double osteotomy of the proximal ulna between April 2010 and June 2015 were included in the study. The patients with CRHD were identified according to medical history, plain radiographs or magnetic resonance imagings. The outcomes were evaluated through comparison of the preoperative and postoperative motion range of elbow and Mayo Elbow Performance Score (MEPS). RESULTS: After a follow-up of 13-35 months (22.29 ± 5.80), compared with pre-operation, the flexion (132.14 ± 3.23° vs 123.21 ± 7.75°, P = 0.003), extension (8.21 ± 4.21° vs 1.07 ± 3.50°, P = 0.003), and pronation of elbow (83.21 ± 4.21° vs 80.36 ± 4.14°, P = 0.011) improved significantly in all patients. Furthermore, the carrying angle was recovered to the normal level (5-15°) in all of these patients (18.57 ± 5.69° vs 8.21 ± 2.49°, P = 0.001). MEPS score was significantly increased postoperatively (96.79 ± 2.49 vs. 90.71 ± 1.82, P = 0.000), with the good outcome in CRHD patients. CONCLUSION: The results of our study suggested that this double osteotomy on the proximal ulna might be an effective method for the treatment of CRHD. LEVEL OF EVIDENCE: Level IV, Therapeutic Study.

[Congenital radial head dislocation: report of two cases]. / Luxación congénita de cabeza radial: reporte de dos casos.

INTRODUCTION: Congenital dislocation of the Radial head is a condition that has been described in a few cases in the international literature, the anterior and lateral dislocation were the less frequent forms of presentation with 15% of cases, each, and the posterior dislocation the most frequent with 70% of the total cases reported. However, this pathology is considered the most frequent congenital pathology of the elbow in children. The present study describes the case of a patient with congenital dislocation of elbow anterior variety and another case with dislocation lateral variety, both diagnosed at an early age, in which it has been decided for conservative management and an annual follow-up. A bibliographic review of the subject is also carried out. DISCUSSION: The congenital elbow dislocation usually has a benign evolution, being painless and not very limiting for the patient, so it can be managed conservatively. Pain and limited movement are indicative of surgical treatment. There are multiple surgical treatments for this entity, however they are not widely accepted due to the complications and poor results presented by them.

INTRODUCCIÓN: La luxación congénita de cabeza radial es un padecimiento que se ha descrito en contados casos en la literatura internacional, siendo la luxación anterior y lateral las formas de presentación menos frecuentes con 15% de los casos cada una y la luxación posterior la más frecuente con 70% del total de los casos reportados. Sin embargo, esta patología es considerada la patología congénita más frecuente del codo en el niño. Se presenta el caso de un paciente con luxación congénita de codo variedad anterior y otro caso con luxación variedad lateral, ambos diagnosticados a temprana edad, en los que se decidió realizar un manejo conservador y un seguimiento anual. Asimismo, se hizo una revisión bibliográfica del tema. DISCUSIÓN: La luxación congénita de codo tiene por lo general una evolución benigna, siendo indolora y poco limitante para el paciente, por lo que puede ser manejada de manera conservadora. El dolor y la limitación de movimientos son indicativos de un tratamiento quirúrgico; sin embargo, no son ampliamente aceptados debido a las complicaciones y resultados deficientes que presentan.

[Elbow Luxation at Birth? - Differential Diagnosis of Elbow Disorders presenting at Birth]. / Ellbogenluxation bei Geburt? ­ Differenzialdiagnose von Ellbogenpathologien bei Geburt.

This is a case report of an isolated congenital radial head dislocation (CRHD) presenting after a breech delivery. The implications of this delivery mode led to the misdiagnosis of an elbow luxation. We found that elbow luxation is a common misdiagnosis of CRHD, although it has not been reported in children younger than one year. For the experienced clinician, repeated examinations and appropriate imaging usually lead to the exclusion of such misdiagnoses. CRHD itself usually remains asymptomatic until adolescence. Without pain or functional impairment of the joint, no specific therapy is needed. This case prompted us to provide an overview of elbow pathologies presenting at birth.

Luxación congénita de cabeza radial: reporte de dos casos / Congenital radial Head dislocation: report of two cases

Resumen: Introducción: La luxación congénita de cabeza radial es un padecimiento que se ha descrito en contados casos en la literatura internacional, siendo la luxación anterior y lateral las formas de presentación menos frecuentes con 15% de los casos cada una y la luxación posterior la más frecuente con 70% del total de los casos reportados. Sin embargo, esta patología es considerada la patología congénita más frecuente del codo en el niño. Se presenta el caso de un paciente con luxación congénita de codo variedad anterior y otro caso con luxación variedad lateral, ambos diagnosticados a temprana edad, en los que se decidió realizar un manejo conservador y un seguimiento anual. Asimismo, se hizo una revisión bibliográfica del tema. Discusión: La luxación congénita de codo tiene por lo general una evolución benigna, siendo indolora y poco limitante para el paciente, por lo que puede ser manejada de manera conservadora. El dolor y la limitación de movimientos son indicativos de un tratamiento quirúrgico; sin embargo, no son ampliamente aceptados debido a las complicaciones y resultados deficientes que presentan.

Abstract: Introduction: Congenital dislocation of the Radial head is a condition that has been described in a few cases in the international literature, the anterior and lateral dislocation were the less frequent forms of presentation with 15% of cases, each, and the posterior dislocation the most frequent with 70% of the total cases reported. However, this pathology is considered the most frequent congenital pathology of the elbow in children. The present study describes the case of a patient with congenital dislocation of elbow anterior variety and another case with dislocation lateral variety, both diagnosed at an early age, in which it has been decided for conservative management and an annual follow-up. A bibliographic review of the subject is also carried out. Discussion: The congenital elbow dislocation usually has a benign evolution, being painless and not very limiting for the patient, so it can be managed conservatively. Pain and limited movement are indicative of surgical treatment. There are multiple surgical treatments for this entity, however they are not widely accepted due to the complications and poor results presented by them.

Elongated Clivus with Deficient Anterior Atlantal Arch and Congenital Posterior Atlantooccipital Dislocation: Pathoembryology and Management Nuances of a Rare Form of Proatlas Segmentation Anomaly.

BACKGROUND: Proatlas segmentation anomalies represent a rare subset of congenital craniovertebral junction anomalies. In this condition, the structures originating from the proatlas, such as the clivus, occipital condyles, foramen magnum rim, and odontoid tip, may demonstrate congenital anomalies, usually without any spinal instability. Elongated clivus, as a result of nonsegmentation of the odontoid tip from the rest of the proatlas, has been reported before to cause ventral spinal cord compression. We report such a case with certain unreported other associations and explore the pathoembryology and management options of such complex anomalies. CASE DESCRIPTION: An 8-year old girl presented with a 2-year history of progressive spastic quadriparesis. On neuroimaging, the anterior arch of the atlas was deficient, the odontoid process was foreshortened, and the clivus was elongated, encroaching into the spinal canal leading to ventral spinal cord compression. Additionally, there was rotatory posterior dislocation of the occipital condyles onto the posterior atlantal arch and vertebral artery anomaly. This patient underwent transoral decompression followed by occipitocervical fusion using rods and screws with satisfactory results. CONCLUSIONS: Proatlas anomalies are rare, varied, and often subtle enough to go unrecognized. Knowledge of the embryology and its aberrations is necessary to understand these anomalies. Our case describes a rare form of bony anomalies pertaining to the fate of the proatlas with accompanying atlanto-occipital dislocation.

A modified microsurgical interfacet release and direct distraction technique for management of congenital atlantoaxial dislocation: technical note.

Various techniques have been used for management of congenital atlantoaxial dislocation. Recently, the reduction of atlantoaxial dislocation through a single posterior approach has attracted more and more attention. Here, we present a modified technique including direct interfacet release and distraction between C1 and C2 by a specially designed distractor, posterior internal fixation and bone graft fusion. The illustrated technique was performed in 15 consecutive patients, and the outcomes were recorded and analyzed. Follow-up ranged from 12 to 26 months. Clinical symptoms improved in 14 patients (93.3%) and were stable in 1 patient (6.7%). Radiologically, 60-100% reduction was achieved in 13 patients (86.6%). Bone fusion was obtained in all patients at 12 months after the operation. The two-tailed Wilcoxon signed-rank test was used to analyze the preoperative and postoperative Japanese Orthopedic Association scores (JOA), atlas-dens interval (ADI), and cervicomedullary angle (CMA) (P < 0.001). Our results suggested that this direct interfacet release and distraction technique with a specially designed C1-2 distractor can provide a definite effective C1-2 facet distraction and odontoid process restoration through a single posterior approach.

Anomalous V3 Segment Aneurysm Associated with Congenital Atlantoaxial Dislocation: Case Report and Discussing the Challenges in Management.

BACKGROUND: An anomalous vertebral artery is not a deterrent for posterior C1-C2 joint manipulation and reduction of atlantoaxial dislocation (AAD). However, presence of an incidental aneurysm in the aberrant segment of artery with concurrent AAD adds to the surgical challenge. CASE DESCRIPTION: A 30-year-old woman presented with neck pain and spastic quadriparesis. Her imaging revealed atlantoaxial dislocation and bony segmentation defects. Three-dimensional computed tomography angiography showed bilateral anomalous vertebral arteries (V3 segment) and an incidental aneurysm on the arterial segment that crossed the right C1-C2 joint posteriorly. Because the artery bearing the aneurysm was nondominant, it was ligated and successful C1-C2 posterior reduction and fusion could be performed. CONCLUSION: The association of an incidental aneurysm with an anomalous vertebral artery in congenital AAD is unusual. The etiology could be an underlying collagen defect or repeated shearing trauma to the vessel wall due to C1-C2 instability. It would be less risky to proceed with endovascular embolization followed by occipitocervical fusion without opening the joints in case the aneurysm is present on the dominant aberrant V3 segment. Ventral decompression can be supplemented for irreducible AAD. On the contrary, if the aneurysm is present on the nondominant aberrant V3 segment, the C1-2 joint can be opened and manipulated following an initial endovascular treatment of the aneurysm. If the circumstances demand, the nondominant artery can be ligated and sacrificed, although there is a small risk of formation of stump aneurysm.

Posterior C2 Fixation Using Trans-C2 Inferior Articular Process Screws: A Case Series and Technical Note.

OBJECTIVE: Upper cervical fixation with C2 pedicle screw insertion may predispose patients to vertebral artery injury, in particular, patients with craniovertebral junction anomalies. The aim of this study was to describe an alternative technique with trans-C2 inferior articular process screw (C2IAPS) insertion for rigid C2 fixation, which can be used to anchor the C2 vertebra for upper cervical fixation. METHODS: Records of 19 patients who underwent posterior atlantoaxial fixation using C2IAPS combined with C1 lateral mass screw were retrospectively reviewed. Efficacy was assessed by postoperative imaging and Japanese Orthopaedic Association scores. RESULTS: There were 22 C2IAPSs successfully implanted (3 on both sides and 16 on 1 side). With the exception of 2 screws that had intruded into the outlet of the intervertebral foramen, all screws were safely implanted. Average Japanese Orthopaedic Association scores improved from 11.8 ± 1.9 preoperatively to 15.3 ± 1.3 postoperatively. Bony fusion rate was 100%. CONCLUSIONS: For patients who are not eligible for C2 pedicle screw fixation, C2IAPS fixation can be considered as an alternative technique for upper cervical fixation of C2.

Bilateral persistent 'second' intersegmental vertebral arteries: illustrated with a case.

Congenital craniovertebral junction deformities can be associated with an anomalous vertebral artery (VA). At times, the artery crosses the joint posteriorly (i.e., persistent first intersegmental artery) and is at risk during posterior approach. We report a new variant, wherein the bilateral VA coursed medially after exiting the C3 transverse foramina to lie beneath C2 pars interarticularis and enter the foramen magnum (without passing through C2 transverse foramen anywhere along its course). This is possibly a result of bilateral persistent second intersegmental arteries. It is pertinent to recognize this unusual variant to avoid VA injury, especially while inserting C2 pars/transarticular screw.

Congenital Unilateral Atlanto-Occipital Rotatory Subluxation: Rare Cause of C1 Neuralgia.

STUDY DESIGN: Case report. OBJECTIVE: We report a rare case of congenital unilateral rotatory atlanto-occipital subluxation that presented with left C1 neuralgia. SUMMARY OF BACKGROUND DATA: Secondary occipital neuralgia is commonly attributed to pathologies of the atlanto-axial joint and C2/C3 nerve involvement. Our case depicts a model of slow creeping atlanto-occipital subluxation due to a rare left C1 superior articular facet dysplasia with C1 foraminal stenosis presenting as C1 neuralgia. We discuss the eitology and patho-anatomy of this rare undescribed presentation. METHODS: A 42-year-old gentleman presented with deteriorating and intractable left occipital headache of 6 months duration. The neck disability index (NDI) was 64%. Cervical MR/computed tomography scan showed a unilateral C1 facet dysmorphism with a left sided C1 foramen bony compression. There was no central canal stenosis. RESULTS: Posterior left C1 arch excision and decompression of C1 foramina with occipital-cervical fusion relieved C1 neuralgia. CONCLUSION: Our case depicts a model of slow creeping deformation due to left C1 superior articular facet dysplasia. An abnormal facet slope allowed the occipital condyle to migrate posteriorly and medially leading to crowding of the left C1 foramen. Although the etiology was congenital, the neck spasm was delayed till fourth decade. A secondary C1 foramen stenosis led to C1 occipital neuralgia that presented as an intractable headache. LEVEL OF EVIDENCE: 5.

'Congenital anomalies of craniovertebral junction presenting after 50 years of age': An oxymoron or An unusual variation?

OBJECTIVES: Manifestation of congenital anomalies of cranio-vertebral junction (CVJ) in the later half of life is unusual and intriguing. Coexisting cervical spondylotic changes with multilevel compression, poorer bone quality as well as less smooth post-surgical recuperation make management of elderly Congenital Atlantoaxial Dislocation/ Basilar Invagination (CAAD/BI) challenging. The clinico-radiological presentation, pathogenesis and outcome are analysed here. PATIENTS AND METHODS: Clinico-radiological data of 20 patients of CAAD/BI (with markers of congenital anomalies) presenting after 50 years of age, the challenges faced and outcomes after C1-C2 fusion have been analysed. RESULTS: Three distinct groups were identified. Seven patients with Os-odontoideum had reducible AAD (Type I). Seven patients had assimilated C1, C2-3 fusion and deformed C1-2 joints with irreducible AAD/BI (Type II). In type III, 4 patients had similar segmentation defects but with compression at both cervico-medullary junction and subaxial spine, although clinical localisation pointed to the CVJ. Spastic quadriparesis was the commonest presentation. All underwent C1-2 fusion alone. There was significant improvement in 18, including those with compression at additional level. Bony fusion was documented in all patients followed up beyond one year. CONCLUSION: Congenital CVJ anomalies may present in later half of life, though attempts at reasoning remain speculative. These patients improve after multiplanar realignment and C1-2 fusion. Careful clinico-radiological evaluation is required in those with additional subaxial compression. Bone quality in elderly is not a deterrent for instrumentation. Fusion eventually occurs in most.

Complex congenital atlantoaxial dislocation in an infant: case report.

The authors report the case of congenital atlantoaxial dislocation in a 9-month-old female infant, who presented with progressive quadriparesis and respiratory failure. The problems in management due to such an early age of presentation, including atypical clinical presentation, unique radiological findings, limited management options, and variable clinical outcomes discussed. This is the youngest case of non-syndromic congenital atlantoaxial dislocation, reported to date, and is a unique combination of lateral, rotatory, and antero-posterior atlantoaxial dislocation.

A device for three-dimensional quantitative assessment and alignment of C1-2 vertebrae during posterior distraction and fusion technique for atlantoaxial dislocation and/or basilar invagination.

The most common type of congenital C1-2 dislocation is a combined type in which atlanto-axial dislocation (AAD) and basilar invagination (BI) are often associated with a rotational dislocation and coronal tilt. An optimal surgical treatment involves reduction of AAD and BI with simultaneous correction of the rotation and coronal tilt to achieve an optimal cervical canal decompression, sagittal and coronal realignment and bony fusion. The most acceptable technique to facilitate this correction is the C1-C2 distraction technique, which is accomplished by the manual joint manipulation. In this study, the authors describe an instrument that accomplishes distraction of the C12 joint space along with its quantitative assessment, permits the easy installation of a joint spacer without damage to the articular surfaces, brings about reduction of AAD and BI, while simultaneously also helping in the correction of the coexisting coronal tilt and rotational dislocation. This distractor not only achieves a multi-planar three-dimensional correction of the displacements at the C1-2 vertebral level, but may be used for the quantitative assessment of the correction and is compatible with the related surgical instruments of all standard companies utilized in this operative procedure.

Atlantoaxial dislocation and os odontoideum in two identical twins: perspectives on etiology.

PURPOSE: There are two theories about the origin of os odontoideum: traumatic or congenital. However, most studies favor the hypothesis of traumatic theory. To emphasize the congenital theory, we report a pair of identical twins both with atlantoaxial dislocation and os odontoideum, which is believed to be a congenital defect. METHODS: We present two 14-year-old identical twins with atlantoaxial dislocation and os odontoideum. Neither of the twins had history of trauma in head nor cervical spine. We reviewed and compared the cervical radiographs of the identical twins. Posterior atlantoaxial reduction, pedicle screw fixation and atlantoaxial fusion were performed for the two twins. RESULTS: Radiological examination showed the identical twins had typical atlantoaxial dislocation and os odontoideum. The twins had high similarity in the appearance of atlantoaxial dislocation and os odontoideum. The etiology of the os odontoideum in the twins is believed to be congenital. Both the twins had improvement in neurological function after surgery. CONCLUSION: Although a great number of cases with os odontoideum have been reported to be traumatic, there are some cases believed to be congenital.

Occipitocervical Fusion for Severe Atlantoaxial Dislocation in an Underdeveloped Child with Chondrodysplasia Punctata: A Case Report.

CASE: We present a case of brachytelephalangic chondrodysplasia punctata with a severe atlantoaxial dislocation in an underdeveloped child. The patient underwent halo jacket application using 10 halo pins with <1 lb/in of torque, followed by posterior occipitocervical fusion with onlay rib and iliac autografts. After bone grafts and replacement of the halo ring multiple times, successful osseous fusion had been achieved by the 2-year follow-up. CONCLUSION: Although simple bone-grafting with a halo jacket is useful in underdeveloped patients with skeletal dysplasia, the complications related to halo fixation, including cranial bone perforation, and the patient's neurological status must be carefully monitored.

Surgical Intervention for Unstable Craniovertebral Junction Anomalies with Narrow C2 Pedicle.

OBJECTIVE: We sought to investigate and report a novel surgical technique of screws insertion and posterior surgical reduction, as well as explore its clinical results. METHODS: From September 2008 to September 2012, we treated 41 cases of unstable craniovertebral junction anomalies with a narrow C2 pedicle at our department. All patients underwent "posterior reduction and internal fixation of the occipital bone with superior or inferior articular process of C2 and lateral mass of C3 on the narrowed C2 pedicle side-for non-narrowed C2 pedicle side, the screw was only inserted into C2 pedicle without extending the fixation to C3 vertebrae-using a titanium screw-rod (plate) fixation system." The preoperative and postoperative atlantodens interval, Chamberlain line, McRae line, and cervicomedullary angle were all measured. In addition, the preoperative and postoperative Japanese Orthopedic Association score was used to evaluate the cervical myelopathy. RESULTS: A total of 134 screws were inserted into the C2 pedicle (30 screws), superior (35 screws) or inferior (17 screws) articular process of C2, and lateral mass of C3 (52 screws). There was a significant statistical difference between the preoperative and postoperative results in the reduction of the odontoid process, decompression of the upper cervical spinal cord and medulla, as well as the improvement of neurologic functions (P < 0.05). All patients have exhibited a major neurologic improvement and solid bony fusion. CONCLUSION: This novel surgical technique is safe, feasible, and effective for the treatment of unstable craniovertebral junction anomalies with a narrow C2 pedicle.