Aldo Mariottini

The importance of surgery in supratentorial ependymomas : Long-term survival in a series of 23 cases

Abstract In view of the widely recognized correlation between extent of surgical resection and length of survival of children with intracranial ependymoma and the statement that total resection is more likely to be achieved in supratentorial than infratentorial primaries, we decided to review our experience with supratentorial ependymomas and the pertinent literature to verify the importance of surgery in treating this subgroup of pediatric ependymal neoplasms. Of 23 patients operated on, 12 are still alive without evidence of disease 72–357 months after surgery (mean 227, median 237 months). One girl treated by surgery alone was lost to follow-up after 234 months when she, and 7 other patients in the series, had already passed the end of the period of risk for recurrence according to Collins’ law. Six surviving patients (2 with subependymoma and 4 with ependymoma) were treated by surgery alone and only 1, the oldest in the series, had to undergo a second operation for recurrence after 10 years. The idea of treating intracranial ependymoma by surgery alone was favored by eminent neurosurgeons in the past and has recently received renewed attention. This was in part the consequence of recognizing that unlike diffuse astrocytoma, in which neoplastic cells can be found up to several centimeters away from the apparent tumor borders, ependymoma has more or less well-defined margins and grows mainly by expansion. Early experience with the policy of electively deferring adjuvant therapy after radiologically controlled total resection of ependymoma seems encouraging, although postoperative MRI does not yet indicate absolute certainty. Close surveillance is recommended. The majority of ependymomas so far treated by surgery alone, with relatively good success, have been supratentorial. In conclusion, on the basis of our experience and a review of the literature we favor a change in attitude to the management of intracranial ependymomas, especially of the cerebrum, with radiologically controlled radical surgery alone followed by close surveillance with periodic MR imaging until the child passes the period of risk for recurrence according to Collins’ law as the initial option. In children less than 3 years old the period of surveillance should be doubled. In case of recurrence, reoperation should be considered first, particularly for supratentorial primaries. Radiotherapy continues to be a major option in malignant ependymoma and unresectable primary or recurrent benign ependymoma.

Preliminary results of a soft novel lumbar intervertebral prothesis (DIAM) in the degenerative spinal pathology.

The authors report a series of 43 patients suffering from lower limb pain, almost constantly associated with chronic or acute backpain, treated by microsurgical nerve root decompression and by implantation of a soft intervertebral prothesis (DIAM). Satisfying results were obtained in 97% of cases, inducing the authors to consider the device a reliable tool for curing low-back pain and sciatica. Selection criteria are exposed and discussed.

Vagus nerve stimulation for drug-resistant epilepsy in children and young adults

We present our experience with the use of intermittent vagal nerve stimulation in 13 patients with medically intractable epilepsy. A surgical approach, with the exception of callosotomy, was impossible. The age range was 6-28 years (median 17 years). In all patients the epilepsy was severe and in six of them was symptomatic. Seven patients had Lennox-Gastaut syndrome, one epilepsy with myoclonic-astatic seizures, four localization-related and one symptomatic generalized epilepsy. The length of the follow-up averaged 22 months (range 8 months-3 years). Of the 13 patients, five (38.4%) had a 50% or more reduction in the number of seizures compared with preimplantation. Of these patients, one with a localization-related epilepsy had a 90% reduction as well as a significant improvement in alertness. Three patients showed no improvement with regard to the number of seizures but there was an improvement in alertness and, in one case in hyperactivity. Some seizure types responded better than others did: complex partial seizures with secondary generalization and atonic seizures. All our responsive patients improved in the first 2 months of VNS activation and only one case with further improvement was observed after this period. Considering the severity of the epilepsy the results can be considered satisfactory. We think that this treatment appears to be a safe adjunctive therapy for children and adults with medically and surgically intractable epilepsy.

Long-term follow-up of childhood cerebellar astrocytomas after incomplete resection with particular reference to arrested growth or spontaneous tumour regression.

SummaryBackground. The long-term prognosis for incompletely excised benign cerebellar astrocytomas in children remains largely unpredictable as the incidence of symptomatic recurrence ranges from 18 to 100%. Arrested growth and spontaneous tumour regression are a recognized but neglected phenomenon, the incidence and objective demonstration of which have received relatively little attention in the literature. With the aim of providing more information on this question, we reviewed our experience with incompletely resected benign cerebellar astrocytomas in children. Clinical material and methods. Our series consisted of 31 children with long-term follow-up. None had signs of type 1 neurofibromatosis (NF1). Histology included 22 classic pilocytic and nine diffuse astrocytomas. Seventeen children had symptomatic recurrence after 25 to 450 months and fourteen children remained in good health for 84 to 516 months.In looking for evident clinical differences that could help elucidate the different courses, we also examined recent radiographic images to assess tumour status. The literature was reviewed to obtain further information on nonrecurring and on regressing cerebellar astrocytomas after partial resection in the setting of modern surveillance imaging resources. Findings. No differences in factors that could elucidate the different clinical courses were found between the two groups of children. Recent negative CT scans or NMR images were reported in seven cases. In two cases arrested growth or regression of a previously visible neoplastic remnant was noted. Review of the literature showed that radiographic follow-up has revealed arrested growth or spontaneous regression of benign cerebellar astrocytoma remnants in 32.5% and 14% of cases, respectively. Conclusions. As hypothesized by Cushing, our study shows that a large proportion of cerebellar astrocytomas do not progress or even regress after partial excision. This phenomen is not understood and unfortunately cannot be anticipated. However, it suggests that a policy of “wait and see” with serial MR imaging can reasonably be recommended in children with cerebellar astrocytoma after partial resection, if the remnant is too risky to be removed by second-look surgery.

Growing skull fractures: progressive evolution of brain damage and effectiveness of surgical treatment

The growing skull fracture of childhood is a well-known but variously interpreted syndrome. Attempts have been made to find different pathogeneses for clinical and pathological patterns that are really successive phases of a single process, arising from the interaction of three basic conditions: (1) head injury with a large gaping fracture; (2) corresponding dural tear; (3) occurrence nearly always in infancy (the first year of life or period of maximum brain growth). This combination of factors alters the normal distribution of the intracranial pressure vectors and the fracture behaves like a “neosuture” with abnormal growth of the skull on the injured side. Simultaneously, the ventricular system tends to deform, dilating and shifting towards the side of the fracture. Three cases, successfully treated at a very late stage, are described. The good surgical results confirm the validity of the surgical method and its underlying theoretical basis.

Recruitment curve of the soleus H-reflex in chronic back pain and lumbosacral radiculopathy

BackgroundNeedle EMG may be negative in mild or predominantly sensory lumbosacral radiculopathies. In such cases, an increase in the latency of the soleus H-reflex is a useful diagnostic criterion for establishing sensory fiber compromise at the S1 root level. However, if clinical signs of radicular involvement are lacking, the latency of the H-reflex is normal. We therefore studied the recruitment curve of the soleus H-reflex to investigate whether a change in the electrical threshold for eliciting the H-reflex might be a more sensitive criterion for detecting subclinical S1 root dysfunction.MethodsClinical and electrophysiological findings from 26 patients with chronic back pain and radiculopathy were compared with data obtained from 40 healthy subjects.ResultsAn increase in the mean H-reflex threshold was the only abnormal electrophysiological finding in patients with no clinical sign of root injury (58%). A decrease in the mean H-reflex amplitude and a prolongation of H-reflex latency was observed in patients with radicular signs (42%). In both patients groups, F-wave and needle EMG studies were normal. No radiological evidence of S1 root compression was found.ConclusionsThe study of the recruitment curve of the soleus H-reflex may be usefully associated to F-wave and needle EMG studies to detect possible S1 root dysfunction in mild lumbosacral radiculopathies. An increase in H-threshold may be the earliest abnormality in the absence of focal neurological signs.

Microsurgery for recurrent lumbar disk herniation at the same level and side: do patients fare worse? Experience with 95 consecutive cases

BACKGROUND In reviewing our experience with reoperation of RLDH, our aim was mainly to determine whether patients fared worse than after primary surgery. We found no uniform answers to this question in the literature. METHODS The data of 95 patients (29 women and 66 men) who underwent reoperation for RLDH at the same level and side were analyzed retrospectively. Forty-two patients underwent the first operation in our clinic (recurrence rate, 2.6% of 1586 cases). Gadolinium-enhanced MRI was performed in all patients. Main clinical data of patients, pain-free interval, operation time, surgical complications, duration of hospital stay, and clinical improvement rate were recorded. RESULTS The mean pain-free interval was 55 months (range, 3-120 months). Levels of recurrent herniation were L4 through L5 and L5 through S1 (65% and 35% of cases, respectively). Revision surgery lasted longer on average than the previous diskectomy (P < .01) and was complicated by dural tear in 4 cases (4.2% vs 0.9% during primary diskectomy, P < .05). There were no significant differences between revision and previous surgery in terms of hospital stay. However, rates of excellent/good outcomes were significantly less for RLDH (89% vs 95%, P < .05); and the percentage of poor results was higher (2% vs 0.5%, P < .05). Age, sex, smoking, profession, trauma, level and degree of herniation, and pain-free interval were not correlated with clinical outcome. CONCLUSION Conventional microsurgery for RLDH showed lightly but significantly worse results than those of primary microdiskectomy. Patients contemplating reoperation should be informed of this fact and of the risk of dural tear and prolonged operation time.

Posterior retroextramarginal disc hernia (PREMDH): Definition, diagnosis, and treatment

BACKGROUND Detachment of the posterior part of the lumbar vertebral ring apophysis has been reported by many authors, associated or not with disc prolapse, and has been ascribed to various mechanisms, although the relationship between the two pathologies remains unclear. METHODS We studied 26 patients (17 males and 9 females; mean age, 34.3 years) suffering from a lumbar disc herniation with nontraumatic detachment of the ring apophysis. Investigations included standard X ray, computed tomography (CT), tridimensional CT, and magnetic resonance imaging. Nineteen patients were operated on by microsurgical discectomy and removal of bone fragments. RESULTS Clinical and neuroradiologic features of herniated disc associated with detachment of the ring apophysis have been recognized and have led to the definition of posterior retroextramarginal disc herniations. A further classification is suggested, considering two morphological types that imply clinically distinctive features and a different surgical approach. In all operated cases, removal of the bone fragments was necessary and the results were good. CONCLUSIONS Our observations led us to postulate a common mechanism in the pathogenesis of disc herniation with nontraumatic detachment of the ring apophysis. They should be distinguished from other calcifications of the disc because a proper surgical technique, including removal of apophyseal fragments, is required.

Homocystinuria With Transverse Sinus Thrombosis

A case of cerebral venous thrombosis caused by undiagnosed homocystinuria is reported. The pitfalls regarding the diagnosis of a potentially medically treatable condition are discussed. Cerebral venous thrombosis in children has a variable type of onset and a multiplicity of causes. This type of pathology, although not frequent, is more common than previously thought. Among the different etiologies, undiagnosed homocystinuria is not routinely considered. We report a case of venous thrombosis of the left transverse cerebral sinus in a girl with drug-resistant partial epilepsy and homocystinuria. This diagnosis was considered and confirmed after the appearance of acute cerebral symptoms caused by venous thrombosis. (J Child Neurol 2001;16:688-690).

Abnormalities of the soleus H-reflex in lumbar spondylolisthesis: a possible early sign of bilateral S1 root dysfunction.

Using routine electrodiagnostic procedures, the authors searched for physiologic evidence of nerve root compromise in patients with chronic mechanical perturbation to the lumbar spine. They examined 37 patients with spondylolisthesis and various degrees of degenerative changes in the lumbar canal. Clinical and neurophysiologic findings were compared with data obtained from 36 healthy persons. The soleus H-reflex appeared to be a sensitive indicator of sensory fiber compromise at the S1 root level, because changes correlated well with the focal sensory signs and preceded clinical and electromyographic signs of motor root involvement. When these occurred, the clinical findings were consistent with a more severe nerve root deficit and with radiographic evidence of neural compression. The greater sensitivity of the soleus H-reflex may be related to the pathophysiologic events that occur at the lesion site.