scholarly journals A 360-Degree Surgical Approach for Correction of Cervical Kyphosis and Atlantoaxial Dislocation in the Case of Larsen Syndrome

2020 ◽  
Vol 11 (01) ◽  
pp. 196-201
Author(s):  
Harsh Deora ◽  
Suyash Singh ◽  
Jayesh Sardhara ◽  
Sanjay Behari
Keyword(s):  
Cervical Spine ◽  
Early Intervention ◽  
Treatment Approach ◽  
Atlantoaxial Dislocation ◽  
Tissue Component ◽  
Cervical Kyphosis ◽  
Physical Damage ◽  
Larsen Syndrome ◽  
Joint Dislocations ◽  
Compressive Myelopathy

AbstractLarsen syndrome is chronic debilitating disease that presents with multiple joint dislocations and severely affects the cervical spine in the form of cervical kyphosis and atlantoaxial dislocation. Children usually present in early with a myriad of deficits, compressive myelopathy being the most common. In addition to a bony compression, there is sometimes a soft tissue component, which is seldom addressed. We present here a case of atlantoaxial dislocation with cervical kyphosis due to Larsen syndrome, and along with our previous experience on syndromic atlantoaxial dislocations, we try to define an algorithm for the treatment approach of these onerous challenges. The importance of early intervention is also emphasized with a literature review of similar cases. In addition to the obvious physical damage, early intervention can also avoid the more sinister socioeconomic face of this debilitating disease.

Management of severe cervical kyphosis in a patient with Larsen syndrome

2008 ◽  
Vol 1 (4) ◽  
pp. 320-324 ◽  
Author(s):  
Marci Madera ◽  
Alvin Crawford ◽  
Francesco T. Mangano
Keyword(s):  
Genetic Disorder ◽  
Treatment Options ◽  
Surgical Approaches ◽  
Facial Features ◽  
Review Of The Literature ◽  
Cervical Instability ◽  
Cervical Kyphosis ◽  
Larsen Syndrome ◽  
Joint Dislocations ◽  
Hands And Feet

✓ Larsen syndrome is a rare genetic disorder of the connective tissue that is characterized by multiple joint dislocations, distinctive deformities of the hands and feet, characteristic facial features, kyphoscoliosis, and segmentation anomalies of the vertebrae. Diverse treatment options, including conservative observation and surgical correction, have been reported for patients who present with cervical spine pathophysiology. Differences in surgical approaches, timing of the correction, and pre- or postoperative bracing have been reported. According to the authors, the present case is the first report of a pediatric patient with Larsen syndrome in whom an asymptomatic cervical instability was treated before neurological deterioration with synchronous anterior decompression and fixation, posterior fusion and fixation, and halo placement. A review of the literature on similar patients reveals the variety of practices associated with a diagnosis of Larsen syndrome.

scholarly journals An orthodontic perspective on Larsen syndrome

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Madoka Yasunaga ◽  
Hiroyuki Ishikawa ◽  
Kenichi Yanagita ◽  
Sachio Tamaoki
Keyword(s):  
Foot Deformities ◽  
Dental Arch ◽  
Cephalometric Analysis ◽  
Craniofacial Anomalies ◽  
Nasal Bridge ◽  
Skeletal Morphology ◽  
Larsen Syndrome ◽  
Craniofacial Dysmorphism ◽  
Joint Dislocations ◽  
Large Joint

Abstract Background Larsen syndrome (LS) is a rare disorder of osteochondrodysplasia. In addition to large-joint dislocations, craniofacial anomalies are typical characteristics. In this report, we performed orthodontic analyses, including skeletal and occlusal evaluations, to examine whether the craniofacial skeletal morphology leads to the craniofacial anomalies in LS. Case presentation A 5 year old Japanese girl who was clinically diagnosed with LS was referred to the orthodontic clinic in the Fukuoka Dental College Medical and Dental Hospital because of a malocclusion. Clinical findings at birth were knee-joint dislocations, equinovarus foot deformities, and cleft soft palate. The patient showed craniofacial anomalies with hypertelorism, prominent forehead, depressed nasal bridge, and flattened midface. To evaluate the craniofacial skeletal morphology, cephalometric analysis was performed. In the frontal cephalometric analysis, the larger widths between bilateral points of the orbitale were related to hypertelorism. The lateral cephalometric analysis revealed the midface hypoplasia and the retrognathic mandible. These findings were responsible for the flattened appearance of the patient’s face, even if the anteroposterior position of the nasion was normal. Her forehead looked prominent in relation to the face probably because of the retrognathic maxilla and mandible. Both the study model and the frontal cephalometric analysis indicated constriction of the upper and lower dental arches. The posterior crossbite facilitated by the premature contacts had developed in association with the constriction of the upper dental arch. Conclusions This patient had some craniofacial anomalies with characteristic appearances in LS. It was evident that the underlying skeletal morphology led to the craniofacial dysmorphism.

Larsen Syndrome With C3–C4 Spondyloptosis and Atlantoaxial Dislocation in an Adult

Spine ◽  
2013 ◽  
Vol 38 (1) ◽  
pp. E43-E47 ◽  
Author(s):  
Roopesh Kumar V. R. ◽  
Venkatesh S. Madhguiri ◽  
Gopalakrishnan M. Sasidharan ◽  
Sudheer Kumar Gundamaneni ◽  
Awdhesh Kumar Yadav
Keyword(s):  
Atlantoaxial Dislocation ◽  
Larsen Syndrome

scholarly journals Trauma of the upper cervical spine: focus on vertical atlantoaxial dislocation

2013 ◽  
Vol 22 (10) ◽  
pp. 2167-2175 ◽  
Author(s):  
M. L. Pissonnier ◽  
J. Y. Lazennec ◽  
J. Renoux ◽  
M. A. Rousseau
Keyword(s):  
Cervical Spine ◽  
Atlantoaxial Dislocation ◽  
Upper Cervical Spine ◽  
Upper Cervical

Safeguarding the Anomalous Vertebral Artery While Dissecting, Drilling, and Instrumentation of C1-2 Joint for Congenital Atlantoaxial Dislocation: 2-Dimensional Operative Video

2018 ◽  
Vol 15 (5) ◽  
pp. E57-E57 ◽  
Author(s):  
Pravin Salunke ◽  
Sushanta K Sahoo
Keyword(s):  
Cervical Spine ◽  
Informed Consent ◽  
Vertebral Artery ◽  
Atlantoaxial Dislocation ◽  
Atlantoaxial Instability ◽  
The Third ◽  
Abnormal Anatomy

Abstract The pathology in congenital atlantoaxial instability is usually in C1-2 joints. Addressing the joints appears to be the most rationale approach. The joints are usually approached posteriorly, manipulated, and fused. Understanding the normal and abnormal anatomy is important. Normally, the third segment of the vertebral artery courses lateral to the C1-2 joint. However, in about 20% of the cases with complex congenital craniovertberal junction anomalies the artery crosses the joint posteriorly. The artery in such cases may be injured while joint manipulation and instrumentation with disastrous consequences. Alternatively, occipital squama can be fused to the cervical spine. However, this requires fusion of multiple segments affecting the neck movements significantly. An anomalous vertebral artery can be dissected, mobilized, and safeguarded while dissecting, manipulating, and fusing the C1-2 joint. In this operative video, authors have highlighted the technique to safeguard the anomalous vertebral artery during joint manipulation.  Proper informed consent was obtained from the patient.

scholarly journals Congenital Joint Dislocations Caused by Carbohydrate Sulfotransferase 3 Deficiency in Recessive Larsen Syndrome and Humero-Spinal Dysostosis

2008 ◽  
Vol 83 (2) ◽  
pp. 293
Author(s):  
Pia Hermanns ◽  
Sheila Unger ◽  
Antonio Rossi ◽  
Antonio Perez-Aytes ◽  
Hector Cortina ◽  
...  
Keyword(s):  
Larsen Syndrome ◽  
Joint Dislocations

scholarly journals Evaluation of clinical profile, radiological and functional outcome following anterior cervical discectomy and fusion for cervical compressive myelopathy

2020 ◽  
Vol 7 (8) ◽  
pp. 2679
Author(s):  
Ranjit Kumar ◽  
Brijesh Kumar Tiwari ◽  
Sanjeev Kumar Pandey
Keyword(s):  
Cervical Spine ◽  
Blood Loss ◽  
Surgical Outcome ◽  
Postoperative Outcome ◽  
Anterior Cervical Discectomy ◽  
Cervical Disc ◽  
Cervical Cord ◽  
Cervical Discectomy ◽  
Cervical Compressive Myelopathy ◽  
Compressive Myelopathy

Background: Symptomatic cervical disc prolapse is best managed with anterior cervical discectomy with or without fusion. We studied the clinical, radiological and surgical profile with postoperative outcome of the patients undergoing anterior cervical discectomy and fusion (ACDF) for cervical compressive myelopathy at one level.Methods: In this retrospective study, data was collected from patients who underwent ACDF for cervical compressive myelopathy in our hospital between 2016 and 2019. Clinical, radiological profile with surgical outcome were studied.Results: A total of 283 patients were recruited. They could be followed up for a period of at least 6 months and so were recruited for the study. Out of these 283 patients (201 males; 82 females; mean age, 48.4 years, range, 22 years to 83 years), magnetic resonance imaging (MRI) cervical spine without contrast and x-ray cervical spine was available for all the patients. Nape of neck pain was the most common presenting symptom followed by tingling sensation in all four limbs and trunk. Average time taken for surgery was 2 hours 10 minutes with an average blood loss of 50 ml. Mean follow up duration was 2.7 years (range 6 months to 4 years).Conclusions: ACDF is the treatment of choice for the patients with clinical and radiological evidence of cervical cord compression. The techniques is associated with minimal blood loss and exceptionally good surgical outcome. Following discectomy, putting an autologous graft or titanium cage improves the spine stability and maintains the cervical lordosis.

scholarly journals Unusual Painful Muscle Spasm in a Dialysis-Dependent Patient with Progressive Cervical Kyphosis

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Daisuke Tsunoda ◽  
Yoichi Iizuka ◽  
Tokue Mieda ◽  
Haku Iizuka ◽  
Hirotaka Chikuda
Keyword(s):  
Cervical Spine ◽  
Cervical Myelopathy ◽  
Muscle Spasm ◽  
Blood Analysis ◽  
Gait Disturbance ◽  
Intractable Pain ◽  
Cervical Kyphosis ◽  
Revision Procedure ◽  
Destructive Spondyloarthropathy ◽  
Dependent Patient

We herein report a case of cervical myelopathy due to destructive spondyloarthropathy (DSA) with an unusual painful muscle spasm. A 51-year-old man presented to our hospital due to numbness in the hands and progressive gait disturbance. The patient underwent laminoplasty for cervical myelopathy. One and a half years after surgery, the patient developed progressive kyphosis of the cervical spine and increasing spastisity. He also started to suffer from intractable pain associated with a muscle spasm. A blood analysis revealed marked CK elevation. The patient underwent 2-stage circumferential decompression and fusion. After the revision procedure, his symptoms improved, and his serum CK level normalized. Physicians should be alert to the possibility of pain associated with an excessive muscle spasm in cervical myelopathy patients.

scholarly journals Does atlantoaxial dislocation influence the subaxial cervical spine?

2013 ◽  
Vol 22 (7) ◽  
pp. 1603-1607 ◽  
Author(s):  
Shenglin Wang ◽  
Peter G. Passias ◽  
Libin Cui ◽  
Gang Li ◽  
Ming Yan ◽  
...  
Keyword(s):  
Cervical Spine ◽  
Atlantoaxial Dislocation ◽  
Subaxial Cervical Spine
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