41/868. scoliosis associated with typical Mayer-Rokitansky-Kuster-Hauser syndrome. Disorders that cause congenital scoliosis include Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. We present the case of a 46-year-old karyotypical (XX) woman with mullerian agenesis (MRKH type A, typical form), a rudimentary bicornate uterus, a blind vaginal pouch, and adenocarcinoma of both ovaries with subsequent bilateral salpingo-oophorectomy. She also had scoliosis of the thoracic and lumbar spine, an association thus far seen only among patients with type B (atypical) MRKH. We describe typical and atypical forms of MRKH and emphasize how these various anomalies associated with mullerian agenesis have affected the classification of the syndrome. We also outline possible embryologic etiologies of mullerian agenesis. ( info) |
42/868. Progression of idiopathic thoracolumbar scoliosis after breast reconstruction with a latissimus dorsi flap: a case report. STUDY DESIGN: A report of a patient in whom progressive symptomatic thoracolumbar scoliosis developed after breast reconstruction with a latissimus dorsi myocutaneous flap. OBJECTIVES: To present the first reported case of progressive symptomatic scoliosis after breast reconstruction with a latissimus dorsi myocutaneous flap and to suggest that latissimus flap harvest may be contraindicated in patients with preexisting scoliosis. SUMMARY OF BACKGROUND DATA: Latissimus dorsi myocutaneous flap harvest incorporated into several surgical operations including breast reconstruction has been presented as a relatively benign procedure without significant biomechanical consequence. Nevertheless, various anatomic and animal studies have suggested an important role for balanced latissimus function in terms of proper spinal alignment. Long-term follow-up evaluation of patients after latissimus flap harvest is insufficient and fails to address the specific issue of spinal deformity. methods: Postoperative radiographs demonstrated significant progression of the patient's thoracolumbar scoliosis as compared with radiographs taken before her latissimus harvest. Curve progression accompanied by development of severe and disabling back pain were considered indications for surgical curve correction and stabilization. RESULTS: At the time of 1-year follow-up assessment after posterolateral spinal fusion and instrumentation, the patient had experienced complete relief from her back pain and satisfactory spinal fusion. CONCLUSIONS: Although a cause and effect relation cannot be established, this case study suggests that latissimus harvest may have a destabilizing effect on the thoracolumbar spine in the long term, especially in patients with preexisting scoliosis. Alternative procedures should be considered in these patients. ( info) |
| Ocular complications after spinal surgery are rare, although ischemic optic neuropathy, occipital lobe infarcts, and central retinal vein thrombosis have been reported. Our purpose is to report a case of an acute, comitant, postoperative exotropia that rapidly and spontaneously resolved. This case is particularly interesting in that it may indirectly shed some light on mechanisms of vergence control. ( info) |
44/868. Prevention of positional brachial plexopathy during surgical correction of scoliosis. Continuous intraoperative monitoring of spinal cord function using somatosensory evoked potentials (SSEP) has gained nearly universal acceptance as a reliable and sensitive method for detecting and possibly preventing neurologic injury during surgical correction of spinal deformities. In several reports, spinal cord injury was identified successfully based on changes in SSEP response characteristics, specifically amplitude and latency. Less well documented and used, however, is monitoring of peripheral nerve function with SSEPs to identify and prevent the neurologic sequelae of prolonged prone positioning on a spinal frame. The authors describe a patient who underwent surgical removal of spinal instrumentation but was not monitored. A brachial plexopathy developed in this patient from pressure on the axilla exerted by a Relton-Hall positioning frame during spinal surgery. In addition, data are presented from 15 of 500 consecutive pediatric patients who underwent surgical correction of scoliosis between 1993 and 1997 with whom intermittent monitoring of ulnar nerve SSEPs was used successfully to identify impending brachial plexopathy, a complication of prone positioning. A statistically significant reduction in ulnar nerve SSEP amplitude was observed in 18 limbs of the 500 patients (3.6%) reviewed. Repositioning the arm(s) or shoulders resulted in nearly immediate improvement of SSEP amplitude, and all awoke without signs of brachial plexopathy. This complication can be avoided by monitoring SSEPs to ulnar nerve stimulation for patients placed in the prone position during spinal surgery. ( info) |
| Two patients with coexisting neurofibromatosis and hyperparathyroidism are described, bringing the total number of such cases in the world literature to seven. Other more classic examples of the association of tumorous conditions of neuroectodermal and entodermal origin are discussed to support the suggestion that the association of these two diseases may be another variant of multiple endocrine neoplasia type 2 (MEN2b). It may be clinically profitable to investigate all patients with either disease in order to uncover their coexistence. ( info) |
46/868. A child with neurofibromatosis-1 and a lumbar epidural arteriovenous malformation. A 10-year-old child with neurofibromatosis-1 was evaluated for progressive lumbar scoliosis, back pain, and foot numbness. magnetic resonance imaging showed several lumbar intraspinal and extraspinal masses consistent with neurofibromas. The mass at L3-L5 compressed the thecal sac and was thought to be the source of the symptoms. On operative exploration, a lumbar epidural arteriovenous malformation was found, which was removed in its entirety. The child's back pain and foot numbness resolved. Epidural arteriovenous malformations in patients with neurofibromatosis-1 are rare and have been reported only in the cervical spine. Our finding of a lumbar epidural arteriovenous malformation in a child with neurofibromatosis-1 demonstrates that vascular anomalies can be present throughout the spine of patients with neurofibromatosis-1 and should be considered in the differential diagnosis of any neurofibromatosis-1-related epidural mass. ( info) |
47/868. Osteoid osteoma of the spine treated with percutaneous computed tomography-guided thermocoagulation. STUDY DESIGN: Two cases are reported in which an osteoid osteoma of the lumbar spine was treated with CT-guided thermocoagulation. OBJECTIVES: To review an alternative and minimally invasive treatment for spinal osteoid osteomas. SUMMARY OF BACKGROUND DATA: Surgical resection of a spinal osteoid osteoma can, depending on the location, be a formidable undertaking. Bone scintigraphy can be helpful in intraoperative identification. More recently, resection through a computed tomography-guided drill hole was found to minimize exposure. Using a thermocoagulation probe, as has been used in osteoid osteoma of the extremities, may be technically easier and cause less morbidity. METHOD: With the patient under general anesthesia, a bone biopsy cannula was introduced into the center of the osteoid osteoma. Material was subjected to histologic examination. A thermocoagulation probe was then inserted and heated to 90 C for 4 minutes. The two patients were kept overnight for observation. RESULTS: Both patients had complete pain relief and no evidence of recurrence after 2 years' follow-up. There were no complications. scoliosis resolved in one patient and persisted in the other. CONCLUSION: Percutaneous computed tomography-guided thermocoagulation is a minimally invasive and technically straightforward method to achieve ablation of a spinal osteoid osteoma. No complications were encountered in these two patients. Future research should focus on the safety of thermocoagulation, especially cephalad to the level of the conus medullaris. ( info) |
48/868. An autopsy case of sudden death in a patient with idiopathic scoliosis. A 38-year-old woman with idiopathic scoliosis (right convex thoracic scoliosis, 78 degrees; left convex lumbar curvature, 75 degrees) died suddenly. Forensic autopsy and histopathologic examination revealed chronic congestive oedema, numerous cavities and atrophic changes of heart. These changes, including both respiratory changes and biventricular failure caused by hypoplastic cardiac changes, were due to a deformed thoracic cage. This case illustrates that not only abnormalities of respiratory function and cor pulmonare, but also hypoplastic cardiac changes, may cause sudden death in a patient with untreated scoliosis. ( info) |
49/868. Decompensation following scoliosis surgery: treatment by decreasing the correction of the main thoracic curve or "letting the spine go". Coronal decompensation following correction of adolescent idiopathic scoliosis (AIS) has been reported to be due to the Cotrel-Dubousset rod derotation maneuver, or to a hypercorrection of the main thoracic curve. The treatment of such decompensation consists classically in observation, bracing, or extension of the instrumentation in the lumbar spine for a King 2 curve, or in the upper thoracic spine for a King 5 curve. As the postoperative decompensation is related to a hypercorrection of the main thoracic curve (relative to the compensatory curve), we hypothesized that if we were to "let the spine go" to some of its initial deformity, the balance of the patient would be improved. The purpose of the study was therefore to report on two cases where a postoperative imbalance following scoliosis surgery was successfully treated by decreasing the correction of the main thoracic curve. Two patients with AIS were found to have significant imbalance after scoliosis surgery. Both patients had been treated for a right thoracic curve (82 degrees and 85 degrees respectively) with an anterior release and posterior instrumentation. The revision surgery consisted for both patients in removing all the hooks between the end vertebrae of the main thoracic curve. This was done before the 3rd postoperative month for both patients. After revision surgery, the balance of both patients improved dramatically within a few weeks. The shoulders became almost level, and the trunk shift improved concomitantly. The Cobb angle increased by 8 degrees and 10 degrees, and the apical vertebra shifted to the right by 15 and 10 mm for the respective patients. These results were stable at 1-year follow-up. In the event of a persisting imbalance, we recommend, in selected cases, letting the spine go by removing all the implants located between the end vertebrae of the main thoracic curve. This adjustment or fine-tuning of the instrumentation should be done before the fusion takes place, and is best achieved with an instrumentation in which the hooks can be easily removed from the rod. ( info) |
50/868. femoral artery ischemia during spinal scoliosis surgery detected by posterior tibial nerve somatosensory-evoked potential monitoring. STUDY DESIGN: A case report of unilateral leg ischemia caused by femoral artery compression detected using posterior tibial nerve somatosensory-evoked potentials during spinal scoliosis instrumentation surgery. OBJECTIVES: To report a rare cause of intraoperative unilateral loss of all posterior tibial nerve somatosensory-evoked potential waveforms. SUMMARY OF BACKGROUND DATA: Failure to obtain adequate popliteal fossa, spinal, subcortical, and cortical potentials during posterior tibial nerve somatosensory-evoked potential spinal cord monitoring usually results from technical factors or chronic conditions affecting the peripheral nerve. methods: A 16-year-old boy with thoracic scoliosis had normal posterior tibial nerve somatosensory-evoked potentials both before surgery and in the operating room immediately after anesthesia induction and prone positioning on a four-post spinal frame. RESULTS: One hour after the start of surgery, a minimal amplitude reduction of the right popliteal fossa potentials appeared. Fifteen minutes later, the amplitudes of the popliteal fossa, subcortical, and cortical potentials evoked by right posterior tibial nerve stimulation became substantially reduced. Subsequently, all waveforms were lost. Malfunction of the right posterior tibial nerve stimulator was initially suspected, but when proper function was verified, a search for other causes of this loss led to discovery of leg ischemia. The patient was repositioned on the spinal frame, and all posterior tibial nerve somatosensory-evoked potentials waveforms began to reappear 7 minutes later. There was no postoperative clinically detectable complication. CONCLUSIONS: Although technical malfunction should always be suspected when all intraoperative somatosensory-evoked potential waveforms are initially seen and subsequently lost, one should also consider the possibility that intraoperative ischemia due to limb positioning could be the etiology. ( info) |