Adult Morquio syndrome requiring occipito-thoracic fusion

Morquio syndrome is a relatively rare entity that is often associated with atlantoaxial instability from early childhood due to odontoid dysplasia based on a mucopolysaccharoidal disorder. Here, we present the case of a 55-year-old male patient with Morquio syndrome who developed cervical myelopathy, which is an extremely rare condition in the older population. Myelopathy developed gradually with upper-limb paresthesia and clumsiness of both hands. The patient had a characteristic “gargoyle-like” coarse face with a trunk shortening-type short stature. Imaging of the cervical spine demonstrated several problems, including diminutive structures called platyspondyly with small pedicles and fragile bone quality, hypoplasia of the C1 posterior arch that migrated into the spinal canal, and os odontoideum with atlantoaxial instability. With intraoperative navigation guidance, posterior decompression of C1 followed by occipito-cervico-thoracic spinal fusion was successfully performed in this complicated case. Clinical and radiographic outcomes were both excellent and have been maintained for 2 years postoperatively.

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Use of a titanium mesh cage for posterior atlantoaxial arthrodesis

Journal of Neurosurgery Spine ◽

10.3171/spi.2002.96.1.0127 ◽

2002 ◽

Vol 96 (1) ◽

pp. 127-130 ◽

Cited By ~ 4

Author(s):

Morio Matsumoto ◽

Kazuhiro Chiba ◽

Takashi Tsuji ◽

Hirofumi Maruiwa ◽

Yoshiaki Toyama ◽

...

Keyword(s):

Donor Site ◽

Atlantoaxial Instability ◽

Os Odontoideum ◽

Posterior Arch ◽

Rigid Fixation ◽

Titanium Mesh ◽

Content Type ◽

Atlantoaxial Fixation ◽

Mesh Cage ◽

Atlantoaxial Arthrodesis

✓ The authors placed titanium mesh cages to achieve posterior atlantoaxial fixation in five patients with atlantoaxial instability caused by rheumatoid arthritis or os odontoideum. A mesh cage packed with autologous cancellous bone was placed between the C-1 posterior arch and the C-2 lamina and was tightly connected with titanium wires. Combined with the use of transarticular screws, this procedure provided very rigid fixation. Solid fusion was achieved in all patients without major complications. The advantages of this method include more stable fixation, better control of the atlantoaxial fixation angle, and reduced donor-site morbidity compared with a conventional atlantoaxial arthrodesis in which an autologous iliac crest graft is used.

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Agenesis of the posterior arch of the atlas

Revista do Hospital das Clínicas ◽

10.1590/s0041-87812002000200005 ◽

2002 ◽

Vol 57 (2) ◽

pp. 73-76 ◽

Cited By ~ 28

Author(s):

Martin Torriani ◽

José Leonardo Goes Lourenço

Keyword(s):

Computerized Tomography ◽

Congenital Abnormality ◽

Structural Instability ◽

Neurological Deficits ◽

Atlantoaxial Instability ◽

Posterior Arch ◽

Rare Entity ◽

Radiological Findings ◽

Plain Films ◽

Rare Congenital Abnormality

PURPOSE: To illustrate the radiological findings and review the current literature concerning a rare congenital abnormality of the posterior arch of the atlas. CASE REPORT: An adult female without neurological symptoms presented with an absent posterior arch of the atlas, examined with plain films and helical computerized tomography. Complete agenesis of the posterior arch of the atlas is a rare entity that can be easily identified by means of plain films. Although it is generally asymptomatic, atlantoaxial instability and neurological deficits may occur because of structural instability. Computerized tomography provides a means of assessing the extent of this abnormality and can help evaluate the integrity of neural structures. Although considered to be rare entities, defects of the posterior arch of the atlas may be discovered as incidental asymptomatic findings in routine cervical radiographs. Familiarity with this abnormality may aid medical professionals in the correct management of these cases.

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Cervical myelopathy caused by atlantoaxial instability in a patient with an os odontoideum and total aplasia of the posterior arch of the atlas: a case report

Journal of Medical Case Reports ◽

10.1186/1752-1947-6-171 ◽

2012 ◽

Vol 6 (1) ◽

Cited By ~ 2

Author(s):

Tadanori Ogata ◽

Tadao Morino ◽

Masayuki Hino ◽

Hiromasa Miura

Keyword(s):

Case Report ◽

Cervical Myelopathy ◽

Atlantoaxial Instability ◽

Os Odontoideum ◽

Posterior Arch

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Bifid Anterior and Posterior Arches of Atlas

Neurosurgery ◽

10.1227/neu.0000000000000761 ◽

2015 ◽

Vol 77 (2) ◽

pp. 296-306 ◽

Cited By ~ 22

Author(s):

Atul Goel ◽

Trimurti Nadkarni ◽

Abhidha Shah ◽

Raghvendra Ramdasi ◽

Neeraj Patni

Keyword(s):

Craniovertebral Junction ◽

Basilar Invagination ◽

Atlantoaxial Instability ◽

Os Odontoideum ◽

Occipital Condyle ◽

Posterior Arch ◽

Discrete Movements ◽

General Observation ◽

Fixation Techniques ◽

Group 1

Abstract BACKGROUND: On reviewing the database of patients with craniovertebral junction anomalies, the authors identified 70 patients with a bifid posterior arch of atlas. OBJECTIVE: To speculate on the pathogenesis of spondyloschisis of both the anterior and posterior arches of atlas, particularly as it relates to atlantoaxial instability. METHODS: Seventy patients with bifid anterior and posterior arches were identified by a retrospective review of the database from 2007 to 2013. RESULTS: The ages of the patients ranged from 14 months to 50 years. The patients were divided into 3 groups. Group 1 (3 patients) had multiple additional spinal bony and neural abnormalities. Group 2 (34 patients) had mobile and partially (5) or completely (29) reducible atlantoaxial dislocation. Group 3 (33 patients) had atlantoaxial instability and related basilar invagination. The os odontoideum was identified in 21 patients, and C2-3 fusion was seen in 24 patients. Two of 3 patients in group 1 were treated conservatively and without any surgery. All patients in groups 2 and 3 were surgically treated. Surgery was done using lateral mass plate/rod and screw fixation techniques. The general observation during surgery included identification of discrete movements of both halves of the atlas, lateral positioning of the facets of atlas in relation to the facets of the axis and occipital condyle and closer approximation of the occipital bone, atlas, and axis resulting in “crumpling” of bone and neural elements. CONCLUSION: Understanding of the pathogenesis and mechanical alterations in cases with a bifid arch of atlas can assist in evaluating the clinical implications and in conduct of surgery.

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Factors for a Good Surgical Outcome in Posterior Decompression and Dekyphotic Corrective Fusion with Instrumentation for Thoracic Ossification of the Posterior Longitudinal Ligament: Prospective Single-Center Study

Operative Neurosurgery ◽

10.1093/ons/opx043 ◽

2017 ◽

Vol 13 (6) ◽

pp. 661-669 ◽

Cited By ~ 10

Author(s):

Shiro Imagama ◽

Kei Ando ◽

Kazuyoshi Kobayashi ◽

Tetsuro Hida ◽

Kenyu Ito ◽

...

Keyword(s):

Spinal Cord ◽

Surgical Outcomes ◽

Ligamentum Flavum ◽

Posterior Longitudinal Ligament ◽

Neurophysiological Monitoring ◽

Posterior Decompression ◽

Thoracic Spinal Cord ◽

Thoracic Spinal ◽

Estimated Blood Loss ◽

Significant Factors

Abstract BACKGROUND Surgery for thoracic ossification of the posterior longitudinal ligament (T-OPLL) is still challenging, and factors for good surgical outcomes are unknown. OBJECTIVE To identify factors for good surgical outcomes with prospective and comparative study. METHODS Seventy-one consecutive patients who underwent posterior decompression and instrumented fusion were divided into good or poor outcome groups based on ≥50% and <50% recovery rates for the Japanese Orthopaedic Association score. Preoperative, intraoperative, and postoperative findings were compared in the 2 groups, and significant factors for a good outcome were analyzed. RESULTS Patients with a good outcome (76%) had significantly lower nonambulatory rate and positive prone and supine position tests preoperatively; lower rates of T-OPLL, ossification of the ligamentum flavum, high-intensity area at the same level, thoracic spinal cord alignment difference, and spinal canal stenosis on preoperative magnetic resonance imaging; lower estimated blood loss; higher rates of intraoperative spinal cord floating and absence of deterioration of intraoperative neurophysiological monitoring; and lower rates of postoperative complications (P < .0005). In multivariate logistic regression analysis, negative prone and supine position test (odds ratio [OR]: 17.00), preoperative ambulatory status (OR: 6.05), absence of T-OPLL, ossification of the ligamentum flavum, high-intensity area at the same level (OR: 5.84), intraoperative spinal cord floating (OR: 4.98), and lower estimated blood loss (OR: 1.01) were significant factors for a good surgical outcome. CONCLUSION This study demonstrated that early surgery is recommended during these positive factors. Appropriate surgical planning based on preoperative thoracic spinal cord alignment difference, as well as sufficient spinal cord decompression and reduction of complications using intraoperative ultrasonography and intraoperative neurophysiological monitoring, may improve surgical outcomes.

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Traumatic transverse atlantal ligamentous injury with posterior atlantoaxial instability in os odontoideum: Magnetic resonance features

Emergency Radiology ◽

10.1007/bf02749192 ◽

1998 ◽

Vol 5 (6) ◽

pp. 442-445

Author(s):

Peter Dross ◽

Azam Rizvi

Keyword(s):

Magnetic Resonance ◽

Atlantoaxial Instability ◽

Os Odontoideum ◽

Ligamentous Injury

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Os Odontoideum with Posterior Atlantoaxial Instability

Spine ◽

10.1097/00007632-199107000-00003 ◽

1991 ◽

Vol 16 (7) ◽

pp. 706-715 ◽

Cited By ~ 41

Author(s):

NOBUKI SHIRASAKI ◽

KOZO OKADA ◽

SHIRO OKA ◽

NOBORU HOSONO ◽

KAZUO YONENOBU ◽

...

Keyword(s):

Atlantoaxial Instability ◽

Os Odontoideum

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The application of C2 transpedicular screw, C1 laminar hook fixation and bone graft in the atlantoaxial instability

Journal of Experimental and Clinical Medicine ◽

10.52142/omujecm.38.2.7 ◽

2021 ◽

Vol 38 (2) ◽

pp. 94-98

Author(s):

Şükrü ORAL

Keyword(s):

Motor Vehicle ◽

Motor Vehicle Accident ◽

Surgical Techniques ◽

Atlantoaxial Instability ◽

Posterior Arch ◽

X Rays ◽

Advantages And Disadvantages ◽

Transpedicular Screw ◽

Flexion Extension ◽

Laminar Hooks

Traumatic atlantoaxial instability usually results from a motor vehicle accident, falls and motorcycle accidents. Atlantoaxial instability can lead to spinal cord compression and neck pain, but, spasticity and radicular symptoms as well. The purpose of surgery is to remove the compression and stabilize the joint permanently. To date, several surgical techniques have been described to remedy C1-C2 instability. In this study, the clinical and radiological outcomes of patients who operated with the C1 (Atlas bone) laminar hooks fixation and bilateral C2 (Axis bone) trans-pedicular screw technique were shown. Also, the advantages and disadvantages of this technique are discussed. From March 2010 to December 2017, 12 patients who have atlantoaxial instability were surgically treated by modified fixation technique which consists C1 laminar hooks fixation and bilateral C2 transpedicular screw. Twelve patients were operated with this procedure from March 2010 to December 2017. All the patients were checked with flexion-extension x-rays at the end of the twelfth week. The posterior bony fusion formation was observed on imaging in all patients. C2 bilateral pedicle screw combined with C1 laminar hook system is a good method for atlantoaxial instability in the conditions which is not convenient for insertion of C1 lateral mass and C2 trans-articular screw. However, this method may not be available in some cases such as traumatic, infection, neoplastic or degenerative pathologies in which the posterior arch of the atlas is damaged.

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Spontaneous ear nose throat bleed: hematohidrosis an unknown entity series of eight cases

International Journal of Otorhinolaryngology and Head and Neck Surgery ◽

10.18203/issn.2454-5929.ijohns20163140 ◽

2016 ◽

Vol 2 (4) ◽

pp. 164

Author(s):

Vikram Venkatsubramaniam Jagannathan ◽

Rajasekar M. K. ◽

Shailaja Mathivanan ◽

Meenaxi Praveen Mehta

Keyword(s):

Emotional Stress ◽

Biochemical Analysis ◽

Rare Condition ◽

The Body ◽

Bleeding Diathesis ◽

Rare Entity ◽

Blood Components ◽

Severe Stress ◽

Intact Skin ◽

Bleeding Episodes

Background: Spontaneous bleed from ear, nose, throat is a rare condition called as hematohidrosis, which occurs under condition of extreme physical or emotional stress. This condition manifests as spontaneous painless bleeding through unbroken skin in any part of the body. It is self-limiting in nature. This condition is relatively unaware among otorhinolaryngologists, the diagnosis of which is made by presence of bloody discharge without any obvious cause through intact skin, witnessed and confirmed by a health professional and the presence of blood components on biochemical analysis.Methods: Retrospective analysis of series of eight cases. Results: All eight cases had normal haematological parameters and were under emotional stress. Two cases manifested with oral bleed.Conclusions: Hematohidrosis is relatively unknown condition to the otorhinolaryngologists and the cases are occurring sporadically which goes unnoticed. This condition occurs in severe stress which may also lead to ear, nose, oral, gastrointestinal bleeds or in combination from any of the sites with a relation of severity of stress level with quantity and site of the bleeding episodes. The condition can be managed effectively if the condition is recognized after ruling out bleeding diathesis. If awareness is created about this clinical condition which is relatively unknown entity, it will lead to further insight on evaluation and management of this rare entity probably an exocrine disorder.

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