scholarly journals RARE CONGENITAL ANOMALIES AT THE CRANIOVERTEBRAL JUNCTION; OS ODENTOIDEUM

Author(s):  
Murat Guntel ◽  
Hanifi Bayarogullari

Aim: We aimed to identify dislocation, myelomalacia, and other congenital pathologies that may cause neurological complications arising from the odontoid process in the craniocervical junction (CVJ) of patients who were performed cervical tomography due to various complaints. Materials and Methods: Patients who were performed cervical CT with various complaints between 2010 and 2018 were retrospectively screened and evaluated, from the hospital registry system. Segmentation and fusion anomalies arising from the odontoid process in the craniovertebral junction area, as well as other accompanying pathologies such as subluxation, dislocation, compression to the spinal cord, and myelomalacia were also recorded. An additional 1.5 Tesla cervical MRI was performed for the 12 patients with os odontoideum. Radiological evaluation was performed on the workstation by an experienced radiologist. Results: 2525 patients with cervical CT were included in the study. 67 of these patients had congenital anomalies at the CVJ. 13 of 67 patients had os odontoideum, 38 had Klippel-Feil syndrome (KFS). Atlanto-occipital assimilation (AOA) and KFS often saw together. Only AOA was observed in 16 cases. Neck pain and limitation of movement were the most common findings in patients with congenital anomalies in the CVJ region. Conclusion: Congenital anomalies at the CVJ region, although rare, can cause mortality or morbidity. Cervical CT and MRI studies on the CVJ region should be evaluated carefully by the clinicians due to the risk of various complications.

2009 ◽  
Vol 64 (suppl_5) ◽  
pp. ons437-ons444 ◽  
Author(s):  
Promod Pillai ◽  
Mirza N. Baig ◽  
Chris S. Karas ◽  
Mario Ammirati

Abstract OBJECTIVE The transoral approach is the most direct and commonly used method to access the ventral craniocervical junction. Recently, an endonasal, endoscopic approach to the craniovertebral junction was proposed. We reasoned that the coupling of the endoscope with the direct transoral approach and image guidance could result in a minimally invasive, simple approach to the ventral craniovertebral junction. We investigated the potential usefulness of such an approach in a cadaver model. METHODS A direct transoral approach to the craniovertebral junction was performed using computed tomography-based image guidance in 9 fresh adult head specimens. Endoscopic odontoidectomy was performed in 5 specimens. In the remaining 4 specimens, the surgical working area and surgical freedom associated with an endoscopic and a microscopic approach to the ventral craniovertebral junction were evaluated and compared. In these 4 specimens, we also measured and compared the exposure of the clivus provided by the endoscope and by the operating microscope without splitting the soft palate. RESULTS With variously angled endoscopic assistance and image guidance, it was possible to tailor the excision of the anterior arch of the atlas and to precisely identify the odontoid process and its related ligaments intraoperatively, resulting in a complete and controlled odontoidectomy. The surgical area exposed over the posterior pharyngeal wall was significantly improved using the endoscope (606.5 ± 127.4 mm3) compared with the operating microscope (425.7 ± 100.8 mm3), without any compromise of surgical freedom (P < 0.05). The extent of the clivus exposed with the endoscope (9.5 ± 0.7 mm) without splitting the soft palate was significantly improved compared with that associated with microscopic approach (2.0 ± 0.4 mm) (P < 0.05). CONCLUSION With the aid of the endoscope and image guidance, it is possible to approach the ventral craniovertebral junction transorally with minimal tissue dissection, no palatal splitting, and no compromise of surgical freedom. In addition, the use of an angled-lens endoscope can significantly improve the exposure of the clivus without splitting the soft palate. An endoscope-assisted transoral approach is a direct and powerful tool for the treatment of surgical pathology at the craniovertebral junction.


2016 ◽  
Vol 15 (4) ◽  
pp. 330-333 ◽  
Author(s):  
Luis Miguel Sousa Marques ◽  
Clara Romero ◽  
José Gabriel Monteiro de Barros Cabral

ABSTRACT Surgical treatment of craniocervical junction pathology has evolved considerably in recent years with the implementation of short fixation techniques rather than long occipito-cervical fixation (sub-axial). It is often difficult and sometimes misleading to determine the particular bone and vascular features (high riding vertebral artery, for instance) using only the conventional images in three orthogonal planes (axial, sagittal and coronal). The authors describe a rare clinical case of congenital malformation of the craniovertebral junction consisting of hypoplasia/agenesis of the odontoid process and bipartite atlas associated with atlantoaxial instability which was diagnosed late in life in a patient with a previous history of rheumatologic disease. The authors refer to the diagnostic process, including new imaging techniques, and three-dimensional multiplanar reconstruction. The authors also discuss the surgical technique and possible alternatives.


2016 ◽  
Vol 12 (3) ◽  
pp. 222-230 ◽  
Author(s):  
Maurizio Iacoangeli ◽  
Alessandro Di Rienzo ◽  
Roberto Colasanti ◽  
Massimo Re ◽  
Davide Nasi ◽  
...  

Abstract BACKGROUND During the past decades, the transoral transpharyngeal approach has been advocated as the standard route for the removal of odontoid causing an irreducible symptomatic neural compression. However, it may be potentially associated with a significant built-in morbidity because of the splitting of the soft palate for an adequate working angle, tracheostomy, and incision of the oral mucosa, causing exposure to a higher risk of infection by oral flora. OBJECTIVE To describe our experience with the minimally invasive pure endoscopic transnasal odontoidectomy in patients with bulbomedullary compression affected by complex anterior craniovertebral junction abnormalities. METHODS Five patients underwent a pure endoscopic neuronavigation-assisted transnasal odontoidectomy with anterior C1 arch preservation. Moreover, the anterior cervical spine column was reconstructed by filling the gap between the C1 arch and the residual C2 body with autologous/artificial bone. Neither tracheostomy nor enteral tube feeding were needed in any case. RESULTS A postoperative neurological improvement was observed in all patients. Postoperative imaging confirmed a satisfactory spinal cord decompression with cervical anterior column arthrodesis, and without evidence of instability at follow-up, so far. CONCLUSION The endoscopic transnasal approach seems to represent an efficient and safe alternative to the transoral route for the resection of odontoid process causing irreducible bulbomedullary compression. It provides a straightforward and minimally invasive natural surgical corridor to the anterior craniocervical junction, allowing a better working angle with preservation of spine biomechanics, while minimizing potential comorbidities.


1988 ◽  
Vol 69 (6) ◽  
pp. 895-903 ◽  
Author(s):  
Arnold H. Menezes ◽  
John C. VanGilder

✓ The anterior transoral-transpharyngeal operation to correct ventral irreducible compression of the cervicomedullary junction was utilized in 72 individuals. The patients' ages ranged from 6 to 82 years, and 29 were children. The pathology encountered was primary basilar invagination, rheumatoid irreducible cranial settling, secondary basilar invagination due to migration of odontoid fracture fragments, dystopic os odontoideum, granulation masses, clivus chordoma, osteoblastoma, and chondroma of the atlas. Fifteen patients had associated Chiari malformation with basilar invagination. Fifty-two patients required subsequent atlantoaxial or occipitocervical fusion. Neurological improvement was the rule. There were two deaths within 30 days of surgery: one from myocardial infarction 4 weeks after surgery and one from Gram-negative septicemia of urinary tract origin. There was one pharyngeal wound infection. The ventral transoral approach provides a safe, rapid, and effective means for decompression of the abnormal craniovertebral junction.


2021 ◽  
Vol 8 (1) ◽  
Author(s):  
Cunha Júnior AL ◽  
◽  
Silva Champs AP ◽  
Meirelles Mello C ◽  
Barduco Carvalho CM ◽  
...  

Background: Craniocervical Junction (CCJ) imaging interpretation in patients with Genetic Skeletal Disorders (GSDs) is challenging due to bone tissue disorganization. CCJ abnormalities and spinal cord compression present potential risks. Purpose: To describe and compare CCJ measurements in patients with GSDs using XR, CT and MRI. Materials and Methods: This cross-sectional observational and analytical study prospectively included 287 participants. Clinical evaluation, spine XR, CCJ dynamic CT, and brain and spinal cord MRI data were recorded. The participants were separated into groups with and without cervical Spinal Cord Injury (cSCI). Three craniometry measurements were performed with each imaging method, and the reliability and reproducibility were analyzed. Results: cSCI was identified in 4.5%. Spinal canal stenosis at C2 (78.8%), a narrowed foramen magnum (12,5%), os odontoideum (5.9%), ventral cervicomedullary encroachment by the odontoid (20.2%), and basilar impression/invagination (12.9%) were associated with an increased chance of cSCI. CT showed the highest accuracy for bone abnormality diagnoses. The cutoff points for the spinal canal to diagnose cSCI were 17.3 mm with XR, 12.9 mm with CT and 10.4 mm with MRI. Conclusion: CT showed good reliability and reproducibility in evaluating the CCJ in GSDs. XR presented more limitations but provided complementary data to MRI.


2015 ◽  
Vol 16 (2) ◽  
pp. 138-145 ◽  
Author(s):  
Travis R. Ladner ◽  
Michael C. Dewan ◽  
Matthew A. Day ◽  
Chevis N. Shannon ◽  
Luke Tomycz ◽  
...  

OBJECT Osseous anomalies of the craniocervical junction are hypothesized to precipitate the hindbrain herniation observed in Chiari I malformation (CM-I). Previous work by Tubbs et al. showed that posterior angulation of the odontoid process is more prevalent in children with CM-I than in healthy controls. The present study is an external validation of that report. The goals of our study were 3-fold: 1) to externally validate the results of Tubbs et al. in a different patient population; 2) to compare how morphometric parameters vary with age, sex, and symptomatology; and 3) to develop a correlative model for tonsillar ectopia in CM-I based on these measurements. METHODS The authors performed a retrospective review of 119 patients who underwent posterior fossa decompression with duraplasty at the Monroe Carell Jr. Children’s Hospital at Vanderbilt University; 78 of these patients had imaging available for review. Demographic and clinical variables were collected. A neuroradiologist retrospectively evaluated preoperative MRI examinations in these 78 patients and recorded the following measurements: McRae line length; obex displacement length; odontoid process parameters (height, angle of retroflexion, and angle of retroversion); perpendicular distance to the basion-C2 line (pB–C2 line); length of cerebellar tonsillar ectopia; caudal extent of the cerebellar tonsils; and presence, location, and size of syringomyelia. Odontoid retroflexion grade was classified as Grade 0, > 90°; Grade I,85°–89°; Grade II, 80°–84°; and Grade III, < 80°. Age groups were defined as 0–6 years, 7–12 years, and 13–17 years at the time of surgery. Univariate and multivariate linear regression analyses, Kruskal-Wallis 1-way ANOVA, and Fisher’s exact test were performed to assess the relationship between age, sex, and symptomatology with these craniometric variables. RESULTS The prevalence of posterior odontoid angulation was 81%, which is almost identical to that in the previous report (84%). With increasing age, the odontoid height (p < 0.001) and pB–C2 length (p < 0.001) increased, while the odontoid process became more posteriorly inclined (p = 0.010). The pB–C2 line was significantly longer in girls (p = 0.006). These measurements did not significantly correlate with symptomatology. Length of tonsillar ectopia in pediatric CM-I correlated with an enlarged foramen magnum (p = 0.023), increasing obex displacement (p = 0.020), and increasing odontoid retroflexion (p < 0.001). CONCLUSIONS Anomalous bony development of the craniocervical junction is a consistent feature of CM-I in children. The authors found that the population at their center was characterized by posterior angulation of the odontoid process in 81% of cases, similar to findings by Tubbs et al. (84%). The odontoid process appeared to lengthen and become more posteriorly inclined with age. Increased tonsillar ectopia was associated with more posterior odontoid angulation, a widened foramen magnum, and an inferiorly displaced obex.


Author(s):  
İlyas Dündar ◽  
Cemil Göya ◽  
Salih Hattapoğlu ◽  
Sercan Özkaçmaz ◽  
Mesut Özgökçe ◽  
...  

Background: : Diverticula are commonly observed in the duodenum. Duodenal diverticulum (DD) usually does not give symptoms throughout life and is diagnosed by coincidence. However, it may present with different symptoms in patients. Objective:: This study aims to evaluate the prevalence of DD and juxtapapillary duodenal diverticulum (JDD) and its association with other possible pathologies and to determine its clinical impact by using Computed Tomography (CT). Methods: This retrospective observational study, which was taken consecutively between the years of 2013-2020, was evaluated in the Radiology Department. The total number of cases was 4850 (male-2440; female-2410). CT images were evaluated by two experienced radiologists at the workstation. DD and JDD prevalence and clinical findings in the hospital registry system were examined. Results: The age of the patients included in the study ranged from 17 to 92 years (mean age 46.94±16.42). In patients with DD (female-130; male-101), mean age was 62.24 ± 12.69 (21-92). The prevalence of DD was 4.76% (n=231). The prevalence of JDD was 4.02% (n=195) and increased with age (p<0.01). The average diameter of the JDD was measured as 23.29±8.22(9.5-55.3) mm. A significant positive correlation was found between age and DD diameter (p=0.039). DDs were found most commonly 84.42% (n=195) in the second segment of the duodenum as JDD. In patients with JDD, the mean diameter of choledochus and wirsung canal were 6.7 ± 2.4 (3-15.3) mm and 0.31 ± 0.1 (0.1-6.5) mm respectively. The choledochal diameter was correlated with the JDD size (p = 0.004). Cholelithiasis (n=56), choledocholithiasis (n=20), cholecystitis (n=52), diverticulitis (n=15), duodenitis (n=37), pancreatitis (n=5) and hiatal hernia (n=60) with JDD were observed. Periampullary carcinoma was detected in one patient.


Author(s):  
Jean-Louis Dietemann ◽  
H. Zhu ◽  
I. Bricout ◽  
S. Athale ◽  
C. Romero ◽  
...  

2003 ◽  
Vol 98 (1) ◽  
pp. 43-49 ◽  
Author(s):  
R. Shane Tubbs ◽  
John C. Wellons ◽  
Jeffrey P. Blount ◽  
Paul A. Grabb ◽  
W. Jerry Oakes

Object. The quantitative analysis of odontoid process angulation has had scant attention in the Chiari I malformation population. In this study the authors sought to elucidate the correlation between posterior angulation of the odontoid process and patients with Chiari I malformation. Methods. Magnetic resonance images of the craniocervical junction obtained in 100 children with Chiari I malformation and in 50 children with normal intracranial anatomy (controls) were analyzed. Specific attention was focused on measuring the degree of angulation of the odontoid process and assigning a score to the various degrees. Postoperative outcome following posterior cranial fossa decompression was then correlated to grades of angulation. Other measurements included midsagittal lengths of the foramen magnum and basiocciput, the authors' institutions' previously documented pB—C2 line (a line drawn perpendicular to one drawn between the basion and the posterior aspect of the C-2 body), level of the obex from a midpoint of the McRae line, and the extent of tonsillar herniation. Higher grades of odontoid angulation (retroflexion) were found to be more frequently associated with syringomyelia and particularly holocord syringes. Higher grades of angulation were more common in female patients and were often found to have obices that were caudally displaced greater than three standard deviations below normal. Conclusions. These results not only confirm prior reports of an increased incidence of a retroflexed odontoid process in Chiari I malformation but quantitatively define grades of inclination. Grades of angulation were not found to correlate with postoperative outcome. It is the authors' hopes that these data add to our current limited understanding of the mechanisms involved in hindbrain herniation.


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