scholarly journals A Case of Achondroplasia Associated with Cervicomedullary-Junction Compression

2003 ◽  
Vol 12 (1) ◽  
pp. 39-42
Author(s):  
Toshiro Takezaki ◽  
Shinobu Nakai ◽  
Toshihiro Tajima ◽  
Koji Okuhara ◽  
Kouhei Satoh ◽  
...  
Keyword(s):  
Cervicomedullary Junction

Pediatric ganglioglioma of the brainstem and cervicomedullary junction: a retrospective cohort study

2020 ◽  
Vol 25 (1) ◽  
pp. 30-36
Author(s):  
Soliman Oushy ◽  
Avital Perry ◽  
Christopher S. Graffeo ◽  
Aditya Raghunathan ◽  
Lucas P. Carlstrom ◽  
...  
Keyword(s):  
Tertiary Care ◽  
Significant Risk ◽  
Foot Drop ◽  
Cranial Neuropathy ◽  
Primary Study ◽  
Tumor Control ◽  
Subtotal Resection ◽  
Low Grade ◽  
Surgical Morbidity ◽  
Cervicomedullary Junction

OBJECTIVEGanglioglioma is a low-grade central nervous system neoplasm with a pediatric predominance, accounting for 10% of all brain tumors in children. Gangliogliomas of the cervicomedullary junction (GGCMJs) and brainstem (GGBSs) present a host of management challenges, including a significant risk of surgical morbidity. At present, understanding of the prognostic factors—including BRAF V600E status—is incomplete. Here, the authors report a single-institution GGCMJ and GGBS experience and review the pertinent literature.METHODSA prospectively maintained neurosurgical database at a large tertiary care academic referral center was retrospectively queried for cases of GGCMJ pathologically confirmed in the period from 1995 to 2015; appropriate cases were defined by diagnosis codes and keywords. Secondary supplemental chart review was conducted to confirm or capture relevant data. The primary study outcome was treatment failure as defined by evidence of radiographic recurrence or progression and/or clinical or functional decline. A review of the literature was conducted as well.RESULTSFive neurosurgically managed GGBS patients were identified, and the neoplasms in 4 were classified as GGCMJ. All 5 patients were younger than 18 years old (median 15 years, range 4–16 years) and 3 (60%) were female. One patient underwent gross-total resection, 2 underwent aggressive subtotal resection (STR), and 2 underwent stereotactic biopsy only. All patients who had undergone STR or biopsy required repeat resection for tumor control or progression. Progressive disease was treated with radiotherapy in 2 patients, chemotherapy in 2, and chemoradiotherapy alone in 1. Immunostaining for BRAF V600E was positive in 3 patients (60%). All 5 patients experienced at least one major complication, including wound infection, foot drop, hemiparesis, quadriparesis, cranial neuropathy, C2–3 subluxation, syringomyelia, hydrocephalus, aspiration, and coma. Overall mortality was 20%, with 1 death observed over 11 years of follow-up.CONCLUSIONSGGBS and GGCMJ are rare, benign posterior fossa tumors that carry significant perioperative morbidity. Contemporary management strategies are heterogeneous and include combinations of resection, radiotherapy, and chemotherapy. The BRAF V600E mutation is frequently observed in GGBS and GGCMJ and appears to have both prognostic and therapeutic significance with targeted biological agents.

A case of cervicomedullary junction compression

2017 ◽  
Vol 4 (4) ◽  
pp. 100
Author(s):  
Priyanshu Mathur ◽  
Ashok Gupta ◽  
Manish Sharma ◽  
Vimesh Parmar
Keyword(s):  
Cervicomedullary Junction

Arterial Brown‐Séquard's syndrome after a penetrating injury of the spinal cord at the cervicomedullary junction

1985 ◽  
Vol 143 (2) ◽  
pp. 84-85 ◽  
Author(s):  
John M. F. Grant ◽  
John D. Yeo ◽  
William R. Sears ◽  
Michael C. Copeman
Keyword(s):  
Spinal Cord ◽  
Penetrating Injury ◽  
Cervicomedullary Junction

scholarly journals Foramen Magnum Stenosis in Achondroplasia

2021 ◽  
Vol 8 (4) ◽  
Author(s):  
Choayb S ◽  
◽  
Adil H ◽  
El Haddad S ◽  
◽  
...  
Keyword(s):  
Sudden Death ◽  
Skull Base ◽  
Short Stature ◽  
Skeletal Dysplasia ◽  
Endochondral Ossification ◽  
Foramen Magnum ◽  
Severe Complication ◽  
Occipital Bone ◽  
Severe Morbidity ◽  
Cervicomedullary Junction

Achondroplasia is the most common hereditary skeletal dysplasia and is characterized by disproportionately short stature with rhizomelic short extremities [1]. The skull features include a narrowed foramen magnum, short skull base, and clivus [2]. Foramen magnum stenosis is a characteristic funding, secondary to an abnormal placement and premature fusion of the posterior synchondroses [1]. The second factor responsible for stenosis is a defect in endochondral ossification in the basiocciput that may result in an extension of the squamous occipital bone [2]. It can cause hydrocephalus and prominent emissary and meningeal veins (Figure 1). Figure 1: Sagittal T1WI revealing a narrowed stenosis of the foramen magnum and compression of the cervicomedullary junction. The most severe complication is the compression of the cervicomedullary junction, associated with severe morbidity and sudden death in younger children [1].

Intra-axial tumors of the cervicomedullary junction

1987 ◽  
Vol 67 (4) ◽  
pp. 483-487 ◽  
Author(s):  
Fred Epstein ◽  
Jeffrey Wisoff
Keyword(s):  
Operative Management ◽  
Neurological Recovery ◽  
Radical Excision ◽  
Term Survival ◽  
Lower Cranial Nerve ◽  
Content Type ◽  
Cervicomedullary Junction ◽  
Cranial Nerve Dysfunction ◽  
Aggressive Surgical Approach ◽  
Spinal Cord Dysfunction

The authors present their experience with the operative management of 20 intra-axial tumors of the cervicomedullary junction. There were two distinct modes of clinical presentation: lower cranial nerve dysfunction and spinal cord dysfunction. Both groups of patients had indolent courses: in 75% of the patients the symptoms had been present for 6 months to 2 years. Radical excision was carried out in all patients. There was no surgical mortality. Postoperative neurological recovery was directly related to the preoperative status; one patient had a significant new deficit. The authors conclude that intrinsic gliomas of the cervicomedullary junction are amenable to radical excision and that an aggressive surgical approach offers the potential for both neurological recovery and long-term survival. The neuroradiological evaluation and operative technique are discussed.

Paraplegia related to solitary lesion of the cervicomedullary junction

2016 ◽  
Vol 117 (2) ◽  
pp. 545-546 ◽  
Author(s):  
Guillaume Taieb ◽  
Xavier Ayrignac ◽  
Clarisse Carra-Dalliere ◽  
Pierre Labauge
Keyword(s):  
Solitary Lesion ◽  
Cervicomedullary Junction

Intractable facial pain associated with a ganglioglioma of the cervicomedullary junction

Neurosurgery ◽  
1982 ◽  
Vol 10 (5) ◽  
pp. 612???6
Author(s):  
N Epstein ◽  
F Epstein ◽  
J C Allen ◽  
S Aleksic
Keyword(s):  
Facial Pain ◽  
Cervicomedullary Junction
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