Scoliosis and hydrocephalus in myelocele patients

1979 ◽  
Vol 50 (2) ◽  
pp. 174-178 ◽  
Author(s):  
Peter Hall ◽  
Richard Lindseth ◽  
Robert Campbell ◽  
John E. Kalsbeck ◽  
Alonso Desousa

✓ Developmental scoliosis is a common cause of increasing disability and deformity in long-term myelocele survivors, and is believed to result from a paralytic collapsing spine. The possible etiological role of compensated hydrocephalus and hydromyelia was assessed by determining the effect of ventricular shunting on 11 myelocele patients with developmental scoliosis. After successful shunting, one patient with a 47° curve continued to deteriorate. Three cases with curves greater than 60° were stabilized for short periods, but eventually required spinal fusion. Seven cases with curves less than 55° were improved from a mean scoliosis of 29° to 13° during a 20-month follow-up period. Several patients had pre-existing shunts that were found to be non-functional on shuntogram. These findings suggest that the spinal complications of hydrocephalus may be more common than previously recognized in myelocele patients and that advanced developmental scoliosis may be avoided by early recognition and ventricular shunting.

2005 ◽  
Vol 102 (1) ◽  
pp. 6-9 ◽  
Author(s):  
Simone A. Betchen ◽  
Jane Walsh ◽  
Kalmon D. Post

Object. Vestibular schwannomas (VSs) are now amenable to resection with excellent hearing preservation rates. It remains unclear whether immediately postoperative hearing is a durable result and will not diminish over time. The aim of this study was to determine the rate of long-term preservation of functional hearing following surgery for a VS and to examine factors influencing hearing preservation. Methods. All patients eligible for hearing preservation (Gardner—Robertson Class I or II) who had undergone resection of a VS by a single surgeon were reviewed retrospectively. Follow-up audiograms and magnetic resonance images were obtained. Of 142 patients deemed eligible for hearing preservation surgery, 38 had immediate postoperative hearing confirmed by an audiogram. In these patients with preserved hearing, the audiographic results demonstrated functional hearing in 30 (85.7%) of 35 patients who underwent repeated testing at a mean follow-up time of 7 years. Delayed hearing loss occurred in five (14.3%) of the 35 patients and did not correlate significantly with the size of the tumor. Hearing improved one Gardner—Robertson class postoperatively in three (7.9%) of the 38 patients. Conclusions. Long-term functional hearing was maintained in 85.7% of patients when it was preserved immediately postoperatively and the result was independent of tumor size. The results of this study emphasize that long-term preservation of functional hearing is a realistic goal following VS surgery and should be attempted in all patients in whom preoperative hearing is determined to be Gardner—Robertson Class I or II.


1995 ◽  
Vol 82 (5) ◽  
pp. 745-751 ◽  
Author(s):  
Michael J. Ebersold ◽  
Michel C. Pare ◽  
Lynn M. Quast

✓ The long-term outcome of cervical spondylitic myelopathy after surgical treatment was retrospectively reviewed and critically evaluated in 100 patients with documented cervical myelopathy treated between 1978 and 1988 at our institution. Eighty-four patients were available for long-term study. The median duration of follow up was 7.35 years (range 3 to 9.5 years). There were 67 men and 17 women; their ages ranged from 27 to 86 years. The duration of preoperative symptoms ranged from 1 month to 10 years. Preoperative functional grade as evaluated with the Nurick Scale for the group was 2.1. Thirty-three patients with primarily anterior cord compression, one- or two-level disease, or a kyphotic neck deformity were treated by anterior decompression and fusion. Fifty-one patients with primarily posterior cord compression and multiple-level disease were treated by posterior laminectomy. There was no difference in the preoperative functional grade in these two groups. The patients in the posterior treatment group were older (59 vs 55 years). There was no surgical mortality from the operative procedures; morbidity was 3.6%. Of the 33 patients undergoing anterior decompression and fusion, 24 showed immediate functional improvement and nine were unchanged. Of the 51 patients who underwent posterior laminectomy, 35 demonstrated improvement, 11 were unchanged, and five were worse. Six patients, one in the anterior group and five in the posterior group, demonstrated early deterioration. Late deterioration occurred from 2 to 68 months postoperatively. Four (12%) patients who had undergone anterior procedures had additional posterior procedures, and seven (13.7%) patients who had undergone posterior procedures had additional decompressive surgery. The final functional status at last follow-up examination for the 33 patients in the anterior group was improved in 18, unchanged in nine, and deteriorated in six. Of the 51 patients who underwent posterior decompression, 19 benefited from the surgery, 13 were unchanged, and 19 were worse at last follow up than before their initial surgical procedure. Age, severity of disease, number of levels operated, and preoperative grade were not predictive of outcome. The only factor related to potential deterioration was the duration of symptoms preoperatively. The results indicate that with anterior or posterior decompression, long-term outcome is variable, and a subgroup of patients, even after adequate decompression and initial improvement, will have late functional deterioration.


1981 ◽  
Vol 55 (3) ◽  
pp. 414-419 ◽  
Author(s):  
Blaine S. Nashold ◽  
Elizabeth Bullitt

✓ Thirteen patients with intractable long-term pain following spinal cord injury and paraplegia were treated with dorsal root entry zone lesions placed at the level just above the transection. Pain relief of 50% or more was achieved in 11 of the 13 patients, with follow-up periods ranging from 5 to 38 months. A previous report showed that central pain from brachial plexus avulsion could be relieved by dorsal root entry zone lesions, and this technique has been extended to the central pain phenomena associated with spinal trauma and paraplegia.


2001 ◽  
Vol 94 (5) ◽  
pp. 757-764 ◽  
Author(s):  
José Guimarães-Ferreira ◽  
Fredrik Gewalli ◽  
Pelle Sahlin ◽  
Hans Friede ◽  
Py Owman-Moll ◽  
...  

Object. Brachycephaly is a characteristic feature of Apert syndrome. Traditional techniques of cranioplasty often fail to produce an acceptable morphological outcome in patients with this condition. In 1996 a new surgical procedure called “dynamic cranioplasty for brachycephaly” (DCB) was reported. The purpose of the present study was to analyze perioperative data and morphological long-term results in patients with the cranial vault deformity of Apert syndrome who were treated with DCB. Methods. Twelve patients have undergone surgery performed using this technique since its introduction in 1991 (mean duration of follow-up review 60.2 months). Eleven patients had bicoronal synostosis and one had a combined bicoronal—bilambdoid synostosis. Perioperative data and long-term evolution of skull shape visualized on serial cephalometric radiographs were analyzed and compared with normative data. Changes in mean skull proportions were evaluated using a two-tailed paired-samples t-test, with differences being considered significant for probability values less than 0.01. The mean operative blood transfusion was 136% of estimated red cell mass (ERCM) and the mean postoperative transfusion was 48% of ERCM. The mean operative time was 218 minutes. The duration of stay in the intensive care unit averaged 1.7 days and the mean hospital stay was 11.8 days. There were no incidences of mortality and few complications. An improvement in skull shape was achieved in all cases, with a change in the mean cephalic index from a preoperative value of 90 to a postoperative value of 78 (p = 0.000254). Conclusions. Dynamic cranioplasty for brachycephaly is a safe procedure, yielding high-quality morphological results in the treatment of brachycephaly in patients with Apert syndrome.


2003 ◽  
Vol 99 (3) ◽  
pp. 480-483 ◽  
Author(s):  
Goro Otsuka ◽  
Kiyoshi Saito ◽  
Tetsuya Nagatani ◽  
Jun Yoshida

Object. Neurofibromatosis Type 2 (NF2) is an intractable disorder predisposing to multiple, recurrent tumors of the central nervous system (CNS). To clarify the survival rate and characteristics that predict poor survival, we retrospectively reviewed clinical data in cases of NF2. Methods. From among 283 patients with neurofibromatosis who had been registered in a nationwide study in Japan between 1986 and 1987, 74 patients with bilateral vestibular schwannomas were analyzed. The mean duration of follow up after diagnosis was 121 months (range 2–287 months). Results of a Kaplan—Meier product-limit analysis indicated that overall 5-, 10-, and 20-year patient survival rates following diagnosis of NF2 were 85, 67, and 38%, respectively. Early onset of the initial symptom significantly compromised survival; 5-, 10-, and 20-year survival rates in patients with symptom onset at an age younger than 25 years were 80, 60, and 28%, respectively, whereas in patients with symptom onset at an age of 25 years or older the rates were 100, 87, and 62%, respectively. Patients with small vestibular schwannomas at diagnosis (< 2 cm in diameter) had better rates of survival. Other variables such as sex, additional tumors in the CNS, or dermal abnormalities did not significantly affect survival. Conclusions. This first report of long-term follow-up results concerning the survival of patients with NF2 indicates an adverse effect of early symptom onset.


1996 ◽  
Vol 84 (4) ◽  
pp. 696-701 ◽  
Author(s):  
Philippe Decq ◽  
Pierre Brugières ◽  
Caroline Le Guerinel ◽  
Michel Djindjian ◽  
Yves Kéravel ◽  
...  

✓ The use of an endoscope in the treatment of suprasellar arachnoid cysts provides an opening of the upper and lower cyst walls, thereby allowing the surgeon to perform a ventriculocystostomy (VC) or a ventriculocystocysternostomy (VCC). To discover which procedure is appropriate, magnetic resonance (MR)—imaged cerebrospinal fluid (CSF) flow dynamics in two patients were analyzed, one having undergone a VC and the other a VCC using a rigid endoscope. Magnetic resonance imaging studies were performed before and after treatment, with long-term follow-up periods (18 months and 2 years). The two patients were reoperated on during the follow-up period because of slight headache recurrence in one case and MR—imaged CSF flow dynamics modifications in the other. In each case surgery confirmed the CSF flow dynamics modifications appearing on MR imaging. In both cases, long-term MR imaging follow-up studies showed a secondary closing of the upper wall orifice. After VCC, however, the lower communication between the cyst and the cisterns remained functional. The secondary closure of the upper orifice may be explained as follows: when opened, the upper wall becomes unnecessary and tends to return to a normal shape, leading to a secondary closure. The patent sylvian aqueduct aids this phenomenon, as observed after ventriculostomy when the aqueduct is secondarily functional. The simplicity of the VCC performed using endoscopic control, which is the only procedure to allow the opening in the cyst's lower wall to remain patent, leads the authors to advocate this technique in the treatment of suprasellar arachnoid cysts.


2003 ◽  
Vol 98 (4) ◽  
pp. 751-763 ◽  
Author(s):  
H. Gregor Wieser ◽  
Marcos Ortega ◽  
Alon Friedman ◽  
Yasuhiro Yonekawa

Object. Analyses of the results of surgery for epilepsy are hindered by inconsistent classifications of seizure outcome, small numbers of patients, and short postoperative follow-up periods. The authors conducted a retrospective study with a reassessment of the long-term seizure outcomes in patients who underwent selective amygdalohippocampectomy (SelAH) for pharmacotherapy—resistant mesial temporal lobe epilepsy (MTLE) at the Zurich University Hospital from 1975 to 1999. Methods. Year-by-year data and the last available data on seizure outcomes were retrospectively assessed for 369 consecutively surgically treated patients who had participated in a follow-up period longer than 1 year as of 1999 and whose outcomes were classified according to the Engel scale and the proposed new International League Against Epilepsy (ILAE) scale. Patients were grouped into nonlesional and lesional MTLE groups depending on whether they harbored a gross anatomical lesion that caused the MTLE. Differentiation was made between curative and palliative operations. Complications related to surgery are reported for 453 patients who underwent SelAH and participated in more than 3 months of follow-up review. The last available outcome data according to the Engel scale were found to be generally similar to those of the new ILAE classification, with 66.9% of patients free from disabling seizures (Engel Class I) compared with 57.1% who were completely seizure and aura free (ILAE Class 1). The last available data on seizure outcome were not significantly different between patients in the lesional and nonlesional MTLE groups. In the lesional group, seizure outcomes were significantly better when patients underwent surgery early in the course of the disease. Overall, 70% of the patients received reductions in their antiepileptic drug treatment at the time of the last available follow-up review. Complications related to the surgical procedures were rare. Conclusions. The authors conclude that SelAH is a safe and effective surgical procedure for MTLE.


2005 ◽  
Vol 102 ◽  
pp. 225-229 ◽  
Author(s):  
En-Min Wang ◽  
Li Pan ◽  
Bing-Jiang Wang ◽  
Nan Zhang ◽  
Liang-Fu Zhou ◽  
...  

Object. The authors assessed the long-term result of gamma knife surgery (GKS) for hemangioblastomas of the brain (HABs) and show histopathological findings after GKS. Methods. Thirty-five patients, 28 men and seven women, with a mean age of 36 years underwent GKS. Eighteen patients presented with multiple tumors and 17 with a solitary tumor. Twenty-one patients had von Hippel—Lindau (VHL) disease. The mean tumor diameter was 13 mm (range 5–55 mm). The mean follow up after GKS was 66 months (range 24–114 months). The mean prescription dose was 17.2 Gy (range 12–24 Gy) at the tumor margin. For tumors close to or within the brainstem a prescription dose of 12 to 13 Gy was used. At the most recent follow up, 29 patients were alive, six were dead, and satisfactory tumor control had been achieved in 29. A stable or improved neurological status was obtained in 21 patients. Eight patients underwent open surgery because of tumor-associated cyst enlargement or the development of new tumors after GKS. Seven patients developed new tumors and five of them required a second GKS. The 1-year tumor control rate was 94%; 2 years, 85%; 3 years, 82%; 4 years, 79%; and 5 years, 71%. Histopathology showed that no tumor cells were found and there was degeneration and necrosis in a tumor nodule 48 months after GKS with a prescription dose of 18 Gy. Conclusions. Gamma knife surgery was a useful choice for small- or medium-sized, solid HAB in the long term, especially when the tumor margin dose was 18 Gy. Although GKS can treat multiple tumors in a single session, for HABs associated with VHL disease, GKS faces the dual problems of tumor recurrence or development of a new tumor.


1991 ◽  
Vol 75 (4) ◽  
pp. 575-582 ◽  
Author(s):  
Mark G. Belza ◽  
Sarah S. Donaldson ◽  
Gary K. Steinberg ◽  
Richard S. Cox ◽  
Philip H. Cogen

✓ Seventy-seven patients presenting with medulloblastoma between 1958 and 1986 were treated at Stanford University Medical Center and studied retrospectively. Multimodality therapy utilized surgical extirpation followed by megavoltage irradiation. In 15 cases chemotherapy was used as adjunctive treatment. The 10- and 15-year actuarial survival rates were both 41% with an 18-year maximum follow-up period (median 4.75 years). There were no treatment failures after 8 years of tumor-free survival. Gross total removal of tumor was achieved in 22 patients (32%); the surgical mortality rate was 3.9%. No significant difference was noted in the incidence of metastatic disease between shunted and nonshunted patients. The classical form of medulloblastoma was present in 67% of cases while the desmoplastic subtype was found in 16%. Survival rates were best for patients presenting after 1970, for those with desmoplastic tumors, and for patients receiving high-dose irradiation (≥ 5000 cGy) to the posterior fossa. Although early data on freedom from relapse suggested a possible beneficial effect from chemotherapy, long-term follow-up results showed no advantage from this modality of treatment. The patterns of relapse and survival were examined; 64% of relapses occurred within the central nervous system, and Collins' rule was applicable in 83% of cases beyond the period of risk. Although patients treated for recurrent disease could be palliated, none were long-term survivors. The study data indicate that freedom from relapse beyond 8 years from diagnosis can be considered as a cure in this disease. Long-term follow-up monitoring is essential to determine efficacy of treatment and to assess survival patterns accurately.


1999 ◽  
Vol 90 (3) ◽  
pp. 448-454 ◽  
Author(s):  
Giuseppe Cinalli ◽  
Christian Sainte-Rose ◽  
Paul Chumas ◽  
Michel Zerah ◽  
Francis Brunelle ◽  
...  

Object. The goal of this study was to analyze the types of failure and long-term efficacy of third ventriculostomy in children.Methods. The authors retrospectively analyzed clinical data obtained in 213 children affected by obstructive triventricular hydrocephalus who were treated by third ventriculostomy between 1973 and 1997. There were 120 boys and 93 girls. The causes of the hydrocephalus included: aqueductal stenosis in 126 cases; toxoplasmosis in 23 cases, pineal, mesencephalic, or tectal tumor in 42 cases; and other causes in 22 cases. In 94 cases, the procedure was performed using ventriculographic guidance (Group I) and in 119 cases by using endoscopic guidance (Group II). In 19 cases (12 in Group I and seven in Group II) failure was related to the surgical technique. Three deaths related to the technique were observed in Group I. For the remaining patients, Kaplan—Meier survival analysis showed a functioning third ventriculostomy rate of 72% at 6 years with a mean follow-up period of 45.5 months (range 4 days–17 years). No significant differences were found during long-term follow up between the two groups. In Group I, a significantly higher failure rate was seen in children younger than 6 months of age, but this difference was not observed in Group II. Thirty-eight patients required reoperation (21 in Group I and 17 in Group II) because of persistent or recurrent intracranial hypertension. In 29 patients shunt placement was necessary. In nine patients in whom there was radiologically confirmed obstruction of the stoma, the third ventriculostomy was repeated; this was successful in seven cases. Cine phase-contrast (PC) magnetic resonance (MR) imaging studies were performed in 15 patients in Group I at least 10 years after they had undergone third ventriculostomy (range 10–17 years, median 14.3 years); this confirmed long-term patency of the stoma in all cases.Conclusions. Third ventriculostomy effectively controls obstructive triventricular hydrocephalus in more than 70% of children and should be preferred to placement of extracranial cerebrospinal shunts in this group of patients. When performed using ventriculographic guidance, the technique has a higher mortality rate and a higher failure rate in children younger than 6 months of age and is, therefore, no longer preferred. When third ventriculostomy is performed using endoscopic guidance, the same long-term results are achieved in children younger than 6 months of age as in older children and, thus, patient age should no longer be considered as a contraindication to using the technique. Delayed failures are usually secondary to obstruction of the stoma and often can be managed by repeating the procedure. Midline sagittal T2-weighted MR imaging sequences combined with cine PC MR imaging flow measurements provide a reliable tool for diagnosis of aqueductal stenosis and for ascertaining the patency of the stoma during follow-up evaluation.


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