Spinal neurological complications of achondroplasia

1980 ◽  
Vol 52 (4) ◽  
pp. 463-472 ◽  
Author(s):  
David F. Morgan ◽  
Ronald F. Young

✓ Spinal neurological complications caused the admission of 17 patients with achondroplasia to the UCLA affiliated hospitals between 1955 and 1979. These patients constituted 41% of all achondroplastic patients admitted during that period. The spinal stenotic syndromes could be divided into three groups: Group I: thoracolumbar stenosis (10 patients); Group II: foramen magnum and upper cervical stenosis (five patients); and Group III: generalized spinal stenosis (two patients). Eleven patients underwent a total of 18 decompressive operative procedures for treatment of paraparesis, quadriparesis, sensory deficits, and sphincter dysfunction. Excellent results were obtained with patients in Group I and II, 77% of whom were ambulatory and continent postoperatively. Two patients in Group III fared less well, showing steady neurological deterioration despite multiple operative procedures. The spectrum of spinal neurological manifestations secondary to achondroplasia is reviewed. Problems with conventional radiological studies and the potential role of computerized tomographic analysis of such patients are discussed. Recommendations for surgical technique are made. Early recognition, prompt clinical evaluation, and safe and accurate radiological analysis of spinal neurological complications of achondroplasia will allow appropriate decompressive surgical procedures to be performed. Excellent results may be anticipated in the reversal and prevention of neurological deficit secondary to achondroplasia with such an approach.

2000 ◽  
Vol 93 (2) ◽  
pp. 305-314 ◽  
Author(s):  
Susan A. Stern ◽  
Brian J. Zink ◽  
Michelle Mertz ◽  
Xu Wang ◽  
Steven C. Dronen

Object. Studies of isolated uncontrolled hemorrhage have indicated that initial limited resuscitation improves survival. Limited resuscitation has not been studied in combined traumatic brain injury and uncontrolled hemorrhage. In this study the authors evaluated the effects of limited resuscitation on outcome in combined fluid-percussion injury (FPI) and uncontrolled hemorrhage.Methods. Twenty-four swine weighing 17 to 24 kg each underwent FPI (3 atm) and hemorrhage to a mean arterial pressure (MAP) of 30 mm Hg in the presence of a 4-mm aortic tear. Group I (nine animals) was initially resuscitated to a goal MAP of 60 mm Hg; Group II (nine animals) was resuscitated to a goal MAP of 80 mm Hg; and Group III (control; six animals) was not resuscitated. After 60 minutes, the aortic hemorrhage was controlled and the animals were resuscitated to baseline physiological parameters and observed for 150 minutes.Mortality rates were 11%, 50%, and 100% for Groups I, II, and III, respectively (Fisher's exact test; p = 0.002). The total hemorrhage volume was greater in Group II (69 ± 32 ml/kg), as compared with Group I (41 ± 18 ml/kg) and Group III (37 ± 3 ml/kg) according to analysis of variance (p < 0.05). In surviving animals, cerebral perfusion pressure, cerebral blood flow (CBF), cerebral venous O2 saturation (ScvO2), and cerebral metabolic rate of O2 did not differ among groups. Although CBF was approximately 50% of baseline during the period of limited resuscitation in Group I, ScvO2 remained greater than 60%, and arteriovenous O2 differences remained within normal limits.Conclusions. In this model of FPI and uncontrolled hemorrhage, early aggressive resuscitation, which is currently recommended, resulted in increased hemorrhage and failure to optimize cerebrovascular parameters. In addition, a 60-minute period of moderate hypotension (MAP = 60 mm Hg) was well tolerated and did not compromise cerebrovascular hemodynamics, as evidenced by physiological parameters that remained within the limits of cerebral autoregulation.


2001 ◽  
Vol 95 (5) ◽  
pp. 839-844 ◽  
Author(s):  
Ann-Christin Sandberg Nordqvist ◽  
Hubert Smurawa ◽  
Tiit Mathiesen

Object. Meningiomas display clinical characteristics that vary from very benign to clearly malignant with rapid invasive growth and metastasis. Benign meningiomas differ in their invasiveness and concomitant edema. This study was undertaken to analyze the expression of matrix metalloproteinases 2 and 9 (MMP-2 and MMP-9, respectively) in meningiomas associated with different degrees of brain invasion and edema. Methods. Tissue samples from 16 meningiomas were selected according to tumor invasiveness from a consecutive series of patients. Samples were analyzed for expression of both MMP-2 and MMP-9 by using in situ hybridization. The meningiomas consisted of three types: Group I, benign meningiomas that did not interfere with the arachnoid plane and exhibited no edema; Group II, benign meningiomas that invaded the arachnoid plane and caused edema; and Group III, aggressive and malignant meningiomas that caused edema and displayed brain invasion. In all 16 tumors analyzed, MMP-2 mRNA was identified. Levels of expression of MMP-2 mRNA were similar in all samples, and no correlation with increasing tumor invasiveness or associated edema could be detected. Expression of MMP-9 mRNA was identified in 14 of the 16 tumors, and a clear correlation with increasing tumor invasion into the brain was noted. Conclusions. Meningiomas express both MMP-2 and MMP-9. Tumor invasiveness, which ranged from minor with respect to the arachnoid membrane and progressed to frank brain invasion, correlated with the extent of MMP-9 expression. The findings indicate that MMP-9 expression and brain invasion are relevant mechanisms that must be interfered with in the treatment of aggressive and malignant meningiomas. No such correlation with MMP-2 was found.


1990 ◽  
Vol 73 (6) ◽  
pp. 840-849 ◽  
Author(s):  
Ossama Al-Mefty

✓ Anterior clinoidal meningiomas are frequently grouped with suprasellar or sphenoid ridge meningiomas, masking their notorious association with a high mortality and morbidity rate, failure of total removal, and recurrence. To avoid injury to encased cerebral vessels, most surgeons are content with subtotal removal. Without total removal, however, recurrence is expected. Recent advances in cranial-base exposure and cavernous sinus surgery have facilitated radical total removal. The author reports 24 cases operated on with vigorous attempts at total removal of the tumor with involved dura and bone. This experience has distinguished three groups (I, II, and III) which influence surgical difficulties, the success of total removal, and outcome. These subgroups relate to the presence of interfacing arachnoid membranes between the tumor and cerebral vessels. The presence or absence of arachnoid membranes depends on the origin of the tumor and its relation to the naked segment of carotid artery lying outside the carotid cistern. Total removal was impossible in the three patients in Group I, with postoperative death occurring in one patient and hemiplegia in another. Total removal was achieved in 18 of the 19 patients in Group II, with one death from pulmonary embolism. In the two patients in Group III, total removal without complications was easily achieved.


1998 ◽  
Vol 88 (6) ◽  
pp. 962-968 ◽  
Author(s):  
Atul Goel ◽  
Mohinish Bhatjiwale ◽  
Ketan Desai

Object. The authors analyzed the cases of 190 patients with basilar invagination that was diagnosed on the basis of criteria laid down in 1939 by Chamberlain to assess the appropriate surgical procedure. Methods. Depending on the association with Chiari malformation, the anomaly of basilar invagination was classified into two groups. Eighty-eight patients who had basilar invagination but no associated Chiari malformation were assigned to Group I; the remainder of the patients, who had both basilar invagination and Chiari malformation, were assigned to Group II. The principal pathological characteristic was observed to be direct brainstem compression due to odontoid process indentation in Group I and a reduction in posterior cranial fossa volume in Group II. Conclusions. Despite the anterior concavity of the brainstem in both groups, transoral surgery was the most suitable procedure for those patients in Group I and decompression of the foramen magnum was found to be appropriate for patients in Group II. After surgical decompression, a fixation procedure was found to be necessary in most Group I cases, but only in a small minority of Group II cases.


1997 ◽  
Vol 86 (1) ◽  
pp. 143-150 ◽  
Author(s):  
Luis Renato Mello ◽  
Leonir T. Feltrin ◽  
Paulo T. Fontes Neto ◽  
Fernando A. P. Ferraz

✓ In the search for a new synthetic substitute for the dura mater, the authors conducted a research study using 32 mongrel dogs divided into three groups. Group I animals (21 dogs) underwent a right-sided parietooccipital craniotomy and substitution of two 1-cm pieces of dura mater by two different grafts: one piece of biosynthetic cellulose (50 µ thick) and one fragment of temporal fascia. The animals were observed for 30, 90, or 180 days. Group II animals (five dogs) underwent a somewhat larger craniotomy, removal of a 2-cm piece of dura mater, and lesioning of the cortex made by a thin sharp forceps, which caused bleeding that was controlled by application of a thin film of cellulose (10 µ thick). Duraplasty was performed using a 50-µ-thick cellulose membrane to complete the procedure and the animals were observed over a period of 270 days. Group III animals (six dogs) underwent smaller (1-cm diameter) bilateral parietal craniectomy, which included additional covering of the dura on the left side with 50-µ-thick cellulose and a suture of temporalis muscle. This group was observed for 40, 60, 80, or 120 days. Transient mild clinical symptoms were observed during the early postoperative period. At autopsy, macroscopic examination demonstrated good acceptance of the grafts with few and moderate extradural fibrosis, which caused adherence of the implants to the bone fragment. No adherence to the cortex was observed. Microscopic examination demonstrated absence of graft adherence to the cortical surface even when the cortex was injured. The cellulose was enveloped by two layers of connective tissue, the external layer being thicker than the internal one. Cellulose fibers increased in thickness over time until 30 days and then decreased in thickness until 270 days. This decrease in thickness between 30 to 270 days was statistically significant (p < 0.05). The physical properties of biosynthetic cellulose and the low cellular reaction to its implantation qualify this material as a dural substitute. Additional long-term studies must be undertaken to complete this report.


2002 ◽  
Vol 96 (2) ◽  
pp. 280-286 ◽  
Author(s):  
Eiji Tachibana ◽  
Kiyoshi Saito ◽  
Keizo Fukuta ◽  
Jun Yoshida

Object. This study was undertaken to investigate the healing process and to delineate factors important for the survival of free fascial grafts used for dural repair. Methods. A dural defect was created in guinea pigs and then reconstructed using either a free fascial graft or an expanded polytetrafluoroethylene (ePTFE) sheet. The fascial graft was covered directly by subcutaneous tissue (Group I) or by a silicone sheet to prevent tissue ingrowth from the subcutaneous tissue (Group II). The ePTFE sheet was covered with a silicone sheet (Group III). One or 2 weeks postoperatively, the strength of the dural repair was evaluated by determining the pressure at which cerebrospinal fluid (CSF) leaked through the wound margins. The dural repair was also histologically examined. In addition, using a rat model, specimens obtained from similar reconstruction sites were immunohistochemically stained with antibodies against basic fibroblast growth factor (bFGF), epidermal growth factor, or transforming growth factor—β. The pressures at which CSF leaked after 1 and 2 weeks, respectively, were 50 ± 14 mm Hg and 126 ± 20 mm Hg in Group I, 70 ± 16 mm Hg and 101 ± 38 mm Hg in Group II, and 0 mm Hg and 8 ± 8 mm Hg in Group III. Failure of repairs made in Group III occurred at significantly lower pressures when compared with Groups I and II. In Groups I and II, a thick fibrous tissue formed around the fascial graft. This tissue tightly adhered to adjacent dura mater. The fibrous tissue displayed a positive reaction for the presence of bFGF. In Group III, only a thin fibrous membrane surrounded the ePTFE sheet. Conclusions. Fascial grafts tolerated extraordinary intracranial pressures at 1 week postoperatively. Free fascial grafts can heal with durable fibrous tissue without the presence of a blood supply from an overlying vascularized flap.


2005 ◽  
Vol 103 (6) ◽  
pp. 1046-1051 ◽  
Author(s):  
Mohammad A. Jamous ◽  
Shinji Nagahiro ◽  
Keiko T. Kitazato ◽  
Junichiro Satomi ◽  
Koichi Satoh

Object. Estrogen has been shown to play a central role in vascular biology. Although it may exert beneficial vascular effects, its role in the pathogenesis of cerebral aneurysms remains to be determined. To elucidate the role of hormones further, the authors examined the effects of bilateral oophorectomy on the formation and progression of cerebral aneurysms in rats. Methods. Forty-five female, 7-week-old Sprague—Dawley rats were divided into three equal groups. Group I consisted of intact rats (controls). To induce cerebral aneurysms, the animals in Groups II and III were subjected to ligation of the right common carotid and bilateral posterior renal arteries. One month later, the rats in Group II underwent bilateral oophorectomy. Three months after the experiment began all animals were killed and cerebral vascular corrosion casts were prepared and screened for cerebral aneurysms by using a scanning electron microscope. Plasma was used to determine the level of estradiol and the gelatinase activity. Hypertension developed in all rats except those in the control group. The estradiol level was significantly lower in Group II than in the other groups (p < 0.01). The incidence of cerebral aneurysm formation in Group II (60%) was three times higher than that in Group III (20%), and the mean size of aneurysms in Group II (76 ± 27 µm, mean ± standard deviation) was larger than that in Group III (28 ± 4.6 µm) (p < 0.05). No aneurysm developed in control animals (Group I), and there was no significant difference in plasma gelatinase activity among the three groups. Conclusions. The cerebral aneurysm model was highly reproducible in rats. Bilateral oophorectomy increased the susceptibility of rats to aneurysm formation, indicating that hormones play a role in the pathogenesis of cerebral aneurysms.


2000 ◽  
Vol 92 (1) ◽  
pp. 70-78 ◽  
Author(s):  
Prakash Sampath ◽  
David Rini ◽  
Donlin M. Long

Object. Great advances in neuroimaging, intraoperative cranial nerve monitoring, and microsurgical technique have shifted the focus of acoustic neuroma surgery from prolonging life to preserving cranial nerve function in patients. An appreciation of the vascular and cranial nerve microanatomy and the intimate relationship between neurovascular structures and the tumor is essential to achieve optimum results. In this paper the authors analyze the microanatomical variations in location of the facial and cochlear nerves in the cerebellopontine angle (CPA) associated with acoustic neuromas and, additionally, describe the frequency of involvement of surrounding neural and vascular structures with acoustic tumors of varying size. The authors base these findings on their experience with 1006 consecutive patients who underwent surgery via a retrosigmoid or translabyrinthine approach.Methods. Between July 1969 and January 1998, the senior author (D.M.L.) performed surgery in 1022 patients for acoustic neuroma: 705 (69%) via the retrosigmoid (suboccipital); 301 (29%) via the translabyrinthine; and 16 (2%) via the middle fossa approach. Patients undergoing the middle fossa approach were excluded from the study. The remaining 1006 patients were subdivided into three groups based on tumor size: Group I tumors (609 patients [61%]) were smaller than 2.5 cm; Group II tumors (244 patients [24%]) were between 2.5 and 4 cm; and Group III tumors (153 patients [15%]) were larger than 4 cm. The senior author's operative notes were analyzed for each patient. Relevant cranial nerve and vascular “involvement” as well as anatomical location with respect to the tumor in the CPA were noted. “Involvement” was defined as adherence between neurovascular structure and tumor (or capsule), for which surgical dissection was required to free the structure. Seventh and eighth cranial nerve involvement was divided into anterior, posterior, and polar (around the upper or lower pole) locations. Anterior and posterior locations were further subdivided into upper, middle, or lower thirds of the tumor.The most common location of the seventh cranial nerve (facial) was the anterior middle third of the tumor for all groups, although a significant number were found on the anterior superior portion. The posterior location was exceedingly rare (< 1%). Interestingly, patients with smaller tumors (Group I) had an incidence (3.4%) of the seventh cranial nerve passing through the tumor itself, equal to that of patients with larger tumors. The most common location of the eighth cranial nerve complex was the anterior inferior portion of the tumor. Not surprisingly, larger tumors (Group III) had a higher incidence of involvement of fourth cranial nerve (41%), fifth cranial nerve (100%), ninth—11th cranial nerve complex (99%), and 12th cranial nerve (31%), as well as superior cerebellar artery (79%), anterior inferior cerebellar artery (AICA) trunk (91.5%), AICA branches (100%), posterior inferior cerebellar artery (PICA) trunk (59.5%), PICA branches (79%), and the vertebral artery (VA) (93.5%). A small number of patients in Group III also had AICA (3.3%), PICA (3.3%), or VA (1.3%) vessels within the tumor itself.Conclusions. In this study, the authors show the great variation in anatomical location and involvement of neurovascular structures in the CPA. With this knowledge, they present certain technical lessons that may be useful in preserving nerve function during surgery and, in doing so, hope to provide neurosurgeons and neurootologists with valuable information that may help to achieve optimum outcomes in patients.


2000 ◽  
Vol 92 (1) ◽  
pp. 24-29 ◽  
Author(s):  
John R. Vender ◽  
Steven J. Harrison ◽  
Dennis E. McDonnell

Object. The high anterior cervical, retropharyngeal approach to the anterior foramen magnum and upper cervical spine is a favorable alternative to the transoral and posterolateral approaches, which both cause instability of the craniovertebral junction. Previously, such instability was corrected via an occipitocervical fusion during a separate surgical procedure. Methods. Seven patients requiring C-2 corpectomy (foramen magnum meningioma [two patients], critical stenosis secondary to rheumatoid arthritis [two patients], C-2 fracture [two patients], and stenosis secondary to Rickets [one patient]) are presented. All patients underwent C1–3 fusion followed by instrumentation with a Caspar plate. A solid fusion was achieved in six patients. One patient experienced erosion of the anterior arch of C-1 requiring posterior stabilization. Conclusions. Fusion and instrumentation at C1–3 can be performed safely and with minimal increase in surgical time. In selected patients, this may eliminate the need for an additional posterior procedure and maintain occipital—C1 mobility.


1986 ◽  
Vol 65 (4) ◽  
pp. 476-483 ◽  
Author(s):  
Robert F. Spetzler ◽  
Neil A. Martin

✓ An important factor in making a recommendation for treatment of a patient with arteriovenous malformation (AVM) is to estimate the risk of surgery for that patient. A simple, broadly applicable grading system that is designed to predict the risk of morbidity and mortality attending the operative treatment of specific AVM's is proposed. The lesion is graded on the basis of size, pattern of venous drainage, and neurological eloquence of adjacent brain. All AVM's fall into one of six grades. Grade I malformations are small, superficial, and located in non-eloquent cortex; Grade V lesions are large, deep, and situated in neurologically critical areas; and Grade VI lesions are essentially inoperable AVM's. Retrospective application of this grading scheme to a series of surgically excised AVM's has demonstrated its correlation with the incidence of postoperative neurological complications. The application of a standardized grading scheme will enable a comparison of results between various clinical series and between different treatment techniques, and will assist in the process of management decision-making.


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