Treatment of Down syndrome—associated craniovertebral junction abnormalities

2000 ◽  
Vol 93 (2) ◽  
pp. 205-213 ◽  
Author(s):  
Derek A. Taggard ◽  
Arnold H. Menezes ◽  
Timothy C. Ryken
Keyword(s):  
Down Syndrome ◽  
Craniovertebral Junction ◽  
Neurological Dysfunction ◽  
Atlantoaxial Instability ◽  
Os Odontoideum ◽  
Upper Respiratory Tract ◽  
General Anesthetic ◽  
Surgical Morbidity ◽  
Content Type ◽  
Fine Print

Object. Operative intervention for craniovertebral junction (CVJ) instability in patients with Down syndrome has become controversial, with reports of a low incidence of associated neurological dysfunction and high surgical morbidity rates. The authors analyzed their experience in light of these poor results and attempted to evaluate differences in management. Methods. Medical and radiographic records of 36 consecutive patients with Down syndrome and CVJ abnormalities were reviewed. The most common clinical complaints included neck pain (15 patients) and torticollis (12 patients). Cervicomedullary compression was associated with ataxia and progressive weakness. Hyperreflexia was documented in a majority of patients (24 cases), and 13 patients suffered from varying degrees of quadriparesis. Upper respiratory tract infection precipitated the presentation in five patients. Four patients suffered acute neurological insults after a minor fall and two after receiving a general anesthetic agent. Atlantoaxial instability was the most common radiographically observed abnormality (23 patients), with a rotary component present in 14 patients. Occipitoatlantal instability was also frequently observed (16 patients) and was coexistent with atlantoaxial dislocation in 15 patients. Twenty individuals had bone anomalies, the most frequent of which was os odontoideum (12 patients) followed by atlantal arch hypoplasia and bifid anterior or posterior arches (eight patients). Twenty-seven patients underwent surgical procedures without subsequent neurological deterioration, and a 96% fusion rate was observed. In five of 11 patients basilar invagination was irreducible and required transoral decompression. Overall, 24 patients enjoyed good or excellent outcomes. Conclusions. The results of this series highlight the clinicopathological characteristics of CVJ instability in patients with Down syndrome and suggest that satisfactory outcomes can be achieved with low surgical morbidity rates.

Anatomical suitability of C1–2 transarticular screw placement in pediatric patients

2000 ◽  
Vol 92 (1) ◽  
pp. 7-11 ◽  
Author(s):  
Douglas L. Brockmeyer ◽  
Julie E. York ◽  
Ronald I. Apfelbaum
Keyword(s):  
Pediatric Patients ◽  
Screw Fixation ◽  
Vascular Complications ◽  
Screw Placement ◽  
Atlantoaxial Instability ◽  
Os Odontoideum ◽  
Transarticular Screw ◽  
Content Type ◽  
Transarticular Screw Fixation ◽  
Fine Print

Object. Craniovertebral instability is a challenging problem in pediatric spinal surgery. Recently, C1–2 transarticular screw fixation has been used to assist in craniovertebral joint stabilization in pediatric patients. Currently there are no available data that define the anatomical suitability of this technique in the pediatric population. The authors report their experience in treating 31 pediatric patients with craniovertebral instability by using C1–2 transarticular screws. Methods. From March 1992 to October 1998, 31 patients who were 16 years of age or younger with atlantooccipital or atlantoaxial instability, or both, were evaluated at our institution. There were 21 boys and 10 girls. Their ages ranged from 4 to 16 years (mean age 10.2 years). The most common causes of instability were os odontoideum (12 patients) and ligamentous laxity (eight patients). Six patients had undergone a total of nine previous attempts at posterior fusion while at outside institutions. All patients underwent extensive preoperative radiological evaluation including fine-slice (1-mm) computerized tomography scanning with multiplanar reconstruction to evaluate the anatomy of the C1–2 joint space. Preoperatively, of the 62 possible C1–2 joint spaces in 31 patients, 55 sides (89%) were considered suitable for transarticular screw placement. In three patients the anatomy was considered unsuitable for bilateral screw placement. In three patients the anatomy was considered inadequate on one side. Fifty-five C1–2 transarticular screws were subsequently placed, and there were no neurological or vascular complications. Conclusions. The authors conclude that C1–2 transarticular screw fixation is technically possible in a large proportion of pediatric patients with craniovertebral instability.

Rotational vertebral artery occlusion from occipital bone anomaly: a rare cause of embolic stroke

2002 ◽  
Vol 97 (6) ◽  
pp. 1456-1459 ◽  
Author(s):  
Teiji Tominaga ◽  
Toshiyuki Takahashi ◽  
Hiroaki Shimizu ◽  
Takashi Yoshimoto
Keyword(s):  
Vertebral Artery ◽  
Three Dimensional ◽  
Craniovertebral Junction ◽  
Artery Occlusion ◽  
Occipital Bone ◽  
Atlantoaxial Instability ◽  
Posterior Arch ◽  
Content Type ◽  
The Right ◽  
Fine Print

✓ Vertebral artery (VA) occlusion by rotation of the head is uncommon, but can result from mechanical compression of the artery, trauma, or atlantoaxial instability. Occipital bone anomalies rarely cause rotational VA occlusion, and patients with nontraumatic intermittent occlusion of the VA usually present with compromised vertebrobasilar flow. A 34-year-old man suffered three embolic strokes in the vertebrobasilar system within 2 months. Magnetic resonance imaging demonstrated multiple infarcts in the vertebrobasilar territory. Angiography performed immediately after the third attack displayed an embolus in the right posterior cerebral artery. Radiographic and three-dimensional computerized tomography bone images exhibited an anomalous osseous process of the occipital bone projecting to the posterior arch of the atlas. Dynamic angiography indicated complete occlusion of the left VA between the osseous process and the posterior arch while the patient's head was turned to the right. Surgical decompression of the VA resulted in complete resolution of rotational occlusion of the artery. An occipital bone anomaly can cause rotational VA occlusion at the craniovertebral junction in patients who present with repeated embolic strokes resulting from injury to the arterial wall.

Surgical management of atlantoaxial nonunions

1995 ◽  
Vol 83 (2) ◽  
pp. 248-253 ◽  
Author(s):  
Curtis A. Dickman ◽  
Volker K. H. Sonntag
Keyword(s):  
Bone Grafts ◽  
Radicular Pain ◽  
Atlantoaxial Instability ◽  
Os Odontoideum ◽  
Autogenous Bone ◽  
Osteoporotic Bone ◽  
Content Type ◽  
Cervical Orthosis ◽  
Fracture Nonunion ◽  
Fine Print

✓ Sixteen patients referred for atlantoaxial fixation failures were treated surgically with revision procedures during the past decade. Of these 16 patients, atlantoaxial instability occurred because of rheumatoid arthritis in five, os odontoideum in seven, transverse ligament disruption in two, and odontoid fracture nonunion in two. The 16 individuals (10 men, six women; mean age 43.7 years; age range 20–77 years) had undergone a total of 20 C1–2 internal fixation procedures that failed. Surgical strategies for definitive revision of the nonunions in these 16 subjects included 10 rigid internal fixations with transarticular screws, three revised C1–2 fixations with autogenous bone struts and wire or cables, and three extended fixations with occipitocervical instrumentation. Autogenous grafts were used in all revisions. A postoperative halo brace was used in five individuals with osteoporotic bone; all patients wore a restrictive postoperative cervical orthosis. Postoperatively, 15 patients (94%) had a stable construct (mean follow up 35 months; range 12–79 months), which included 13 osseous unions and two stable fibrous unions. One patient had nonunion; he fractured his anterior C1–2 transarticular screws 2 years postoperatively. He had occipital radicular pain without myelopathy but refused further surgery. Atlantoaxial pseudarthroses were effectively treated by addressing the pathological, biomechanical, and technical reasons for failed fusion. Successful fusion after reoperation was improved by using autologous bone grafts, adequately controlling atlantoaxial motion (with rigid transarticular screws internally or externally with a halo vest), compressing the bone grafts between the arches of C-1 and C-2 with wire cables, meticulously preparing the fusion bed, and by optimizing the pharmacological and clinical parameters to promote bone healing.

Posterior atlantoaxial facet screw fixation

1993 ◽  
Vol 79 (2) ◽  
pp. 234-237 ◽  
Author(s):  
Paul Marcotte ◽  
Curtis A. Dickman ◽  
Volker K. H. Sonntag ◽  
Dean G. Karahalios ◽  
Janine Drabier
Keyword(s):  
Screw Fixation ◽  
Metastatic Tumor ◽  
Atlantoaxial Instability ◽  
Os Odontoideum ◽  
Rigid Fixation ◽  
Spinal Fixation ◽  
Strut Graft ◽  
Content Type ◽  
Fine Print

✓ Eighteen patients with atlantoaxial instability were treated with posterior atlantoaxial facet screws to obtain immediate rigid fixation of C1–2. Of these 18 patients, instability occurred due to trauma in nine, rheumatoid arthritis in six, neoplasms in two, and os odontoideum in one. Four patients presented with nonunion after failed C1–2 wire and graft procedures. In all cases in this series the screw fixations were augmented with an interspinous C1–2 strut graft which was wired in place to provide three-point stabilization and to facilitate bone fusion. In every case fixation was satisfactory, and C1–2 alignment and stability were restored without complications due to instrumentation. One patient died 3 months postoperatively from metastatic tumor; the spinal fixation was intact. All 17 surviving patients have developed osseous unions (mean follow-up period 12 months, range 6 to 16 months). Posterior atlantoaxial facet screw fixation provides immediate multidirectional rigid fixation of C1–2 that is mechanically superior to wiring or clamp fixation. This technique maximizes success without the need for a supplemental rigid external orthosis, and is particularly useful for pseudoarthrosis.

Instability of the craniovertebral junction and treatment outcomes in patients with Down's syndrome

1999 ◽  
Vol 6 (6) ◽  
pp. E5 ◽  
Author(s):  
Derek A. Taggard ◽  
Arnold H. Menezes ◽  
Timothy C. Ryken
Keyword(s):  
Down's Syndrome ◽  
Down’S Syndrome ◽  
Craniovertebral Junction ◽  
Operative Intervention ◽  
Atlantoaxial Instability ◽  
Os Odontoideum ◽  
Complication Rates ◽  
General Anesthetics ◽  
Surgical Morbidity ◽  
Radiographic Findings

Operative intervention for the treatment of instability at the craniovertebral junction in patients with Down's syndrome has become somewhat controversial because some authors have reported high surgery-related complication rates and suggested that the incidence of neurological abnormality associated with this abnormal motion may be low. In this report, the authors describe the clinical and radiographic findings in 33 patients treated at their institution. Common presenting symptoms included neck pain (14 patients), torticollis (12 patients), and myelopathy manifested as hyperreflexia (21 patients), or varying degrees of quadriparesis (11 patients). Four patients suffered acute neurological insults, two after receiving routine general anesthetics for minor surgical procedures and two other patients following minor falls. Atlantoaxial instability was the most common abnormality documented on radiography (22 patients). Atlantooccipital instability (15 patients) was also frequently observed and was coexistent with the presence of atlantoaxial luxations in 14 patients. A rotary component of the atlantoaxial luxation was present in 13 cases. In 17 patients bony anomalies were present, the most frequent of which was os odontoideum (10 patients). Twenty-four patients underwent operative intervention, and successful fusion was achieved in 23. In six of nine patients with basilar invagination, reduction was achieved with preoperative traction and thus avoided the need for ventral decompressive procedures. There were no cases of postoperative deterioration, and 22 patients made excellent or good recoveries. The results of this series highlight the clinicopathological phenomena of craniovertebral instability in patients with Down's syndrome and suggest that satisfactory outcomes can be achieved with a low rate of surgical morbidity.

Acute subdural hematoma

1982 ◽  
Vol 57 (2) ◽  
pp. 254-257 ◽  
Author(s):  
Henry A. Shenkin
Keyword(s):  
Computerized Tomography ◽  
Subdural Hematoma ◽  
Neurological Status ◽  
Neurological Dysfunction ◽  
Acute Subdural Hematoma ◽  
Consecutive Series ◽  
Content Type ◽  
Subdural Hematomas ◽  
Fine Print

✓ In a consecutive series of 39 cases of acute subdural hematoma (SDH), encountered since computerized tomography diagnosis became available, 61.5% were found to be the result of bleeding from a small cortical artery, 25.6% were of venous origin, 7.7% resulted from cerebral contusions, and 5% were acute bleeds into chronic subdural hematomas. Craniotomy was performed promptly on admission, but there was no difference in survival (overall 51.3%) between patients with arterial and venous bleeds. The only apparent factor affecting survival in this series was the preoperative neurological status: 67% of patients who were decerebrate and had fixed pupils prior to operation died. Of patients with less severe neurological dysfunction, only 20% failed to survive.

Use of a titanium mesh cage for posterior atlantoaxial arthrodesis

2002 ◽  
Vol 96 (1) ◽  
pp. 127-130 ◽  
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.

Progressive neurological dysfunction secondary to postoperative cervical pseudomeningocele in a C-4 quadriplegic

1978 ◽  
Vol 48 (2) ◽  
pp. 289-291 ◽  
Author(s):  
Kenneth P. Burres ◽  
Frances K. Conley
Keyword(s):  
Neurological Deficit ◽  
Neurological Symptoms ◽  
Diagnostic Evaluation ◽  
Neurological Dysfunction ◽  
Previous Surgery ◽  
Content Type ◽  
Progressive Neurological Deficit ◽  
Operative Closure ◽  
Fine Print

✓ A case is detailed of a patient who developed progressive neurological deficit above a fixed quadriplegic level at C-4 18 years after posterior cervical decompression for trauma. Diagnostic evaluation revealed a pseudomeningocele at the site of his previous surgery. Subsequent operative closure resulted in reversal of his neurological symptoms.

Surgical management of spinal cord hemangioblastomas in patients with von Hippel—Lindau disease

2003 ◽  
Vol 98 (1) ◽  
pp. 106-116 ◽  
Author(s):  
Russell R. Lonser ◽  
Robert J. Weil ◽  
John E. Wanebo ◽  
Hetty L. Devroom ◽  
Edward H. Oldfield
Keyword(s):  
Spinal Cord ◽  
Surgical Management ◽  
Postoperative Outcome ◽  
Tumor Location ◽  
Neurological Dysfunction ◽  
Autosomal Dominant Disorder ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Vhl Disease ◽  
Fine Print

Object. Von Hippel—Lindau (VHL) disease is an autosomal-dominant disorder frequently associated with hemangioblastomas of the spinal cord. Because of the slow progression, protean nature, and high frequency of multiple spinal hemangioblastomas associated with VHL disease, the surgical management of these lesions is complex. Because prior reports have not identified the factors that predict which patients with spinal cord hemangioblastomas need surgery or what outcomes of this procedure should be expected, the authors have reviewed a series of patients with VHL disease who underwent resection of spinal hemangioblastomas at a single institution to identify features that might guide surgical management of these patients. Methods. Forty-four consecutive patients with VHL disease (26 men and 18 women) who underwent 55 operations with resection of 86 spinal cord hemangioblastomas (mean age at surgery 34 years; range 20–58 years) at the National Institutes of Health were included in this study (mean clinical follow up 44 months). Patient examination, review of hospital charts, operative findings, and magnetic resonance imaging studies were used to analyze surgical management and its outcome. To evaluate the clinical course, clinical grades were assigned to patients before and after surgery. Preoperative neurological status, tumor size, and tumor location were predictive of postoperative outcome. Patients with no or minimal preoperative neurological dysfunction, with lesions smaller than 500 mm3, and with dorsal lesions were more likely to have no or minimal neurological impairment. Syrinx resolution was the result of tumor removal and was not influenced by whether the syrinx cavity was entered. Conclusions. Spinal cord hemangioblastomas can be safely removed in the majority of patients with VHL disease. Generally in these patients, hemangioblastomas of the spinal cord should be removed when they produce symptoms or signs.

The natural history of intracranial venous angiomas

1991 ◽  
Vol 75 (5) ◽  
pp. 715-722 ◽  
Author(s):  
Timothy B. Garner ◽  
O. Del Curling ◽  
David L. Kelly ◽  
D. Wayne Laster
Keyword(s):  
Natural History ◽  
Imaging Techniques ◽  
Venous Drainage ◽  
Vascular Lesion ◽  
Neurological Dysfunction ◽  
Content Type ◽  
History Of ◽  
Clinical Records ◽  
Venous Angiomas ◽  
Fine Print

✓ Cerebral venous angiomas are congenital anomalies of the intracranial venous drainage. Many believe that they are associated with a high risk of hemorrhage and neurological dysfunction, but newer neurodiagnostic imaging techniques are showing not only that they are more common than previously known but also that many have no associated symptoms. In this retrospective study, the natural history of venous angiomas was examined in 100 patients (48 males and 52 females) with radiographically identifiable lesions treated over a 14-year period. Information on the natural history of the lesion was obtained from clinical records and follow-up data. Imaging studies included angiography, computerized tomography, and magnetic resonance imaging. Angioma locations were classified as frontal (42 cases), parietal (24 cases), occipital (4 cases), temporal (2 cases), basal or ventricular (11 cases), cerebellar (14 cases), or brain stem (3 cases); 47 lesions were on the left side. Headache as a presenting symptom was common (36 patients) and often led to other radiographic studies, but this appeared to be related to the vascular lesion in only four patients. Other possibly related complications were hemorrhage in one patient, seizures in five, and transient focal deficits in eight. Fifteen patients had no neurological signs or symptoms. The mean patient age at last contact was 45.3 years (range 3 to 94 years). All patients have been managed without surgery. It is concluded that significant complications secondary to venous angiomas are infrequent and that surgical resection of these lesions and of surrounding brain is rarely indicated.

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