The purpose of the present study is to evaluate retrospectively the effects of several intra-operative manipulations on the results of foramen magnum decompression (FMD) in patients having syringomyelia associated with type I Chiari malformation. Seventy-five patients having syringomyelia associated with Chiari I malformation were operated on between 1975 and 1996. This population was grouped into 4 subgroups according to the surgical protocol: group I = 42 patients with FMD alone; group II = 16 patients with FMD and third ventricle shunting; group III = 9 patients with FMD and syringosubarachnoid shunting (SSS); group IV = 8 patients with FMD and cerebellar tonsils resection. Pre- and postsurgical magnetic resonance imaging (MRI) studies were analyzed (and compared). Nine patients were lost to follow-up. The results were evaluated in the 66 remaining patients (mean follow-up: 52 months), using the Bidzinski's outcome scale (ref). Two patients (3%) died postoperatively, 31 (47%) had very good results (after additional surgery in 7), 16 (24.2%) had good results (after additional surgery in 7) and 17 (25.7%) had poor results despite further surgery in 9. A total of 27 reoperations were undertaken after primary FMD in 23 patients (35%). Thirty-nine patients (59%) had both pre- and postsurgical MRI evaluation. In 28 (72%) the syrinx had markedly decreased whereas it had remained stable in 11 (28%). Clinical results were not significantly different between the patients of groups I, II and III. Very good or good results were obtained in 24 patients (64.8%) of group I (after additional surgery in 10), in 8 (61.5%) of group II (after additional surgery in 1) and in 7 (87.5%) of group III (after additional surgery in 3). Results in group IV were as follows: 7 patients (87%) had very good results and one had a good result. With a mean follow-up of 28 months, no patient required additional surgery. Postsurgical MRI syrinx reduction was observed in all 8 patients either in the early postoperative course or on delayed followup. It is suggested that tonsils resection might enhance the results of FMD in individuals having Chiari I-related syringomyelia.
Hypothalamic hamartoma are rare lesions. We report a new series of eight patients treated for precocious puberty (six cases) or gelastic seizures (two cases). Surgical resection was total in four cases (three pediculated and one sessile). Precocious puberty was controlled by surgical treatment in all cases. Gelastic seizures were controlled by medical treatment, but the patients did not become seizure free. We observed no mortality and no endocrinological or visual morbidity. The fact that a vascular "rete mirabilis" was observed on the surface of the lesion in our surgical material is an argument favoring a vascular mechanism in precocious puberty. Coagulation of this vascular structure can help control precocious puberty. Our series confirms that the hypothalamic hamartoma can be surgically treated when patients fail to respond to medical treatment, when the length of the treatment cannot be tolerated by the chidren and their families, and when there are uncontrolled gelastic seizures
The best surgical approach to TMs is still a controversial matter. The advantages and drawbacks of conventional versus transbasal approaches are reviewed. Our experience suggests that subtotal removal can be associated with long recurrence-free intervals and preserved quality of life. TMs located at the tentorial edge carried a definitely worse prognosis than peripheral forms.
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