J Neurosurg Spine 15:258270, 2011

Tethered cord syndrome in adults

Clinical article
JRG KLEkAMP, M.D.
Department of Neurosurgery, Christliches Krankenhaus, Quakenbrck, Germany
Object. The treatment of tethered cord syndromes in adults is discussed regarding the natural history and surgical indications. The author analyzes data obtained in patients who were diagnosed with a tethered cord in adulthood and either underwent surgical or conservative therapy between 1991 and 2009. Methods. Since 1991, data obtained in 2515 patients with spinal cord pathologies were entered into the spinal cord database, and prospective follow-up was performed through outpatient visits and questionnaires. Of the 2515 patients, 85 adults with a tethered cord syndrome formed the basis of this study. The tethering effect was caused either by a split cord malformation, a thick filum terminale, a conus medullaris lipoma with extradural extension, or various combinations of these mechanisms. The mean age of the patients was 46 13 years (range 2374 years) and the mean follow-up duration was 61 62 months. Two groups were distinguished based on the absence (Group A, 43 patients) or presence (Group B, 42 patients) of an associated lipoma or dysraphic cyst (that is, dermoid, epidermoid, or neurenteric cyst). Surgery was recommended for patients with symptoms only. Short-term results were determined within 3 months of surgery, whereas long-term outcomes (clinical recurrences) were evaluated using Kaplan-Meier statistics. Results. For all patients, pain was the most common major complaint. Severe neurological deficits were rare. In Group A, 20 of 43 patients underwent surgery, whereas in Group B 23 of 42 patients underwent surgery. Among individuals who did not undergo surgery, 17 patients refused surgery and 25 patients underwent recommended conservative treatment. Short-term postoperative results indicated a significant improvement of pain and a stabilization of neurological symptoms. Long-term results showed a good prognosis in patients in whom first-time (that is, nonrevision) surgery achieved successful untethering, with a 10-year rate of neurological stabilization in 89% of Group A and a 10-year rate of neurological stabilization in 81% of Group B patients. The benefit of secondary operations in Group B was limited, with eventual clinical deterioration occurring in all patients within 10 years. For patients treated conservatively, follow-up information could be obtained in 33 of 42 patients. Twenty-eight patients remained in stable clinical condition. Only 5 of the conservatively treated patients experienced clinical deterioration over time; in 4 of these individuals with deterioration, surgery had been recommended but was refused by the patient. The clinical recurrence rate in all conservatively treated patients was 21% after 10 years. With a recommendation for surgery this figure rose to 47% within 5 years. Conclusions. Surgery in adult patients with a tethered cord syndrome should be reserved for those with symptoms. In surgically treated patients, pain relief can often be achieved, and long-term neurological stabilization tends to persist more often than it does in conservatively treated patients. A conservative approach is warranted, however, in adult patients without neurological deficits. Revision surgery in patients with complex dysraphic lesions should be performed in exceptional cases only. (DOI: 10.3171/2011.4.SPINE10504)

KEY WORdS tethered cord syringomyelia neurenteric cyst split cord malformation dermoid cyst spinal lipoma

PINAL dysraphic lesions are rare pathological entities. With folate acid substitution during pregnancy and prenatal diagnosis of dysraphic malformations often leading to termination of pregnancy, the number of children with spina bifida aperta has been reduced dramatically in Western countries. In contrast, patients with occult spinal dysraphism are often overlooked, even though their disease is often far from occult given the cutaneous manifestations in many of them. With early diagnosis of these lesions, however, there remains an ongoing debate of whether asymptomatic patients with a tethered cord should undergo prophylactic surgery. Since 1991, data obtained in all patients with spinal cord pathological entities such as tumors, syringomyelia,

Abbreviation used in this paper: KPS = Karnofsky Performance Scale.

or dysraphism have been entered into a spinal cord database at the authors institution and the patients have been monitored clinically in prospective fashion. In 1994, a retrospective study described spinal dysraphism in 23 adult patients who had been treated since 1977. The authors concluded that surgery should be reserved for symptomatic patients.39 The present study presents the results acquired in adult patients with tethered cord syndrome that was not diagnosed in childhood; the patients presented between 1991 and 2009 and were managed according to this policy. Do all adult patients with tethered cord syndromes eventually become symptomatic with progressive neurological deficits? Does untethering surgery provide a long-term benefit in adults? It was the purpose of this study to determine the short- and long-term results of surgery in these patients and to compare their outcome with those obtained in patients who underwent conservative treatment.
J Neurosurg: Spine / Volume 15 / September 2011

258

Adult tethered cord syndrome

A total of 2515 patients presented to the Nordstadt Hospital in Hannover, Germany, between 1991 and 2003, or the Christliche Krankenhaus in Quakenbrck, Germany, between 2004 and 2009, with spinal cord pathologies and relevant data were entered into the spinal cord database. Apart from general patient data and specific features of each spinal cord pathological entity, the neurological examinations before surgery, before discharge from the hospital, after 3 months postoperatively, and yearly thereafter were analyzed for individual symptoms according to a scoring system for individual symptoms (Table 1).40 Patients were also evaluated according to the KPS.36 For each patient, the most important symptom was noted as the main symptom. Among these 2515 patients, 126 adult patients presented with spinal dysraphic lesions. Of these, I excluded 13 patients with surgically treated myelomeningoceles and Chiari Type II malformations who presented with signs of a retethered cord and 28 patients with spinal hamartomas (lipoma or dysraphic cyst) without a tethered cord. Eighty-five patients with a tethered cord not diagnosed in childhood were entered in this study. All patients presented with MR images. Depending on the pathology, CT scanning and conventional radiography were also performed. Myelography and postmyelograhic CT scanning were performed only in exceptional cases, such as complex pathologies or after previous surgeries. Two categories of patients were defined: Group A: patients with either a low-positioned conus medullaris below L-2, a thick filum terminale, or a split cord malformation (that is, a tethered cord syndrome only [43 patients]) and Group B: patients with a combination of a tethered cord and a hamartoma (42 patients). In general, surgery was recommended as soon as neurological symptoms were present. In patients with complex dysraphic lesions in Group B, surgery was reserved for those with progressive neurological symptoms. Surgical strategies depended on the individual pathological entity. In patients with a tethered cord due to a thick filum terminale, the filum was transected. In patients with a split cord malformation, we resected the tethering components at the level of the split cord and transected the filum in cases with a low-lying conus.26,57,58
TABLE 1: Neurological scoring system Neurological Deficit Score 5 4 3 2 1 0 Pain none slight, no medication Sensory Disturbance, Dysesthesias normal present, not significant Motor Weakness full power movement against resistance movement against gravity movement w/o gravity contraction w/o movement paralysis Gait Ataxia Sphincter Function

Methods

The extent of untethering was evaluated intraoperatively and considered successful if all tethering components could be released. For all dysraphic cysts, a complete resection, including the capsule, was intended. Whenever possible a lipoma was left untouched to avoid arachnoid adhesions. A lipoma mass was reduced only if the spinal cord and lipoma would not fit comfortably in the spinal canal and dural sac or if the lipoma had extradural extension. In such cases, intra- and extradural components of the lipoma were separated from each other at the level of the dura, the extradural part in the subcutaneous space was left in place to avoid problems of wound healing, and the intradural part was reduced to accommodate the cord and the rest of the lipoma in the reconstructed dural sac. Whenever I partially resected a lipoma, I attempted closure of the capsule to avoid recurrent tethering. No additional measures such as shunt placement were taken in patients with an associated syrinx. In all Group B patients and in Group A patients with a split cord, duraplasty was performed to avoid retethering. Except for 3 patients treated early in the series with fascia lata grafts, artificial materials were used. The duraplasty graft was lifted off the spinal cord by means of tenting sutures to minimize the risk of retethering.41,42 After discharge from the hospital, all surgically treated patients were examined after 3 months, and yearly follow-up information was obtained by further outpatient visits or questionnaires. Likewise, conservatively treated patients were asked to complete follow-up questionnaires. For statistical tests of significance, Student t-tests and Fisher exact tests were employed. Long-term follow-up data were analyzed with Kaplan-Meier statistics34 to determine the percentages of patients with either a stable neurological status or progressive symptoms (that is, a clinical recurrence). For all statistical analyses, the software package PC-Stastik version 4.0 (Hoffmann-Software) was employed. Surgical morbidity was defined as a new, permanent postoperative deficit or a permanent aggravation of a preexisting deficit.
Preoperative Data

Results

Since 1991, 85 adult patients presented with either a

good control w/ medication significant, function not re stricted insufficient control w/ med- some restriction of function ication severe despite medication severe restriction of func tion incapacitating incapacitated function

normal normal unsteady, no aid slight disturbance, no catheter mobile w/ aid residual, no cath eter few steps w/ aid rarely incontinent standing w/ aid paralysis frequent catheter permanent catheter

J Neurosurg: Spine / Volume 15 / September 2011

259

J. Klekamp
tethered cord syndrome (Group A, 43 patients) or a combination of a tethered cord with a hamartoma (Group B, 42 patients). The mean age at clinical presentation for the entire group was 46 13 years (range 2374 years), with a mean clinical history of just over 8 years (99 141 months) before diagnosis and no significant differences between both groups (91 144 months in Group A and 106 139 months in Group B). Group A patients were significantly older than Group B patients (51 15 years vs 44 12 years, respectively; p = 0.0082 [t-test]). Overall, female patients predominated in this study. This dominance was more pronounced in Group A than in Group B (female/male ratio 35:8 and 26:16, respectively; p = 0.0288 [Fisher exact test]). Table 2 lists the cutaneous manifestations and radiological features for both groups. Cutaneous stigmata were more common in Group B (p = 0.02 [Fisher exact test]). Dermal sinuses, however, were observed in both groups in similar proportions. No such patient gave a history of meningitis. Syringomyelia and split cord malformations were significantly more common in Group A (p < 0.0001 and p = 0.0152, respectively [Fisher exact test]). All syrinx cavities started at the level of tethering, expanded in a rostral direction, and almost all were of small caliber. No patient had a history of increasing neurological symptoms related to an expanding syrinx. Twenty-four split cord malformations were encountered. Three patients harbored a Type 1 malformation, with both hemicords in separate dural sacs (Fig. 1), in contrast to the remaining 21 patients with both hemicords in a single dural sac (that is, Type 2) (Fig. 2).59 In Group A, 15 split cord malformations were located at or close to the conus medullaris, with one Type 2 malformation in the cervical spine. In Group B, 7 split cord malformations were found close to the conus (Fig. 1), with 1 patient harboring a cervical Type 2 malformation (Fig. 2). All 32 lipomas were attached to the conus (Figs. 35); 7 of these lipomas demonstrated extradural extension into the subcutaneous area (Fig. 5). One dermoid cyst and one neurenteric cyst (Fig. 2) were located in the cervical spine, whereas the remaining cystic hamartomas were located in the lumbosacral region (Fig. 1). Table 3 lists the percentages and Table 4 the clinical scores for individual neurological symptoms in both groups at presentation to provide an overview of the clinical spectrum. Most patients had minor neurological symptoms: just 1 patient in Group A and 3 patients in Group B were unable to walk, whereas 3 patients in Group A and 4 patients in Group B required a urinary
TABLE 2: Associated features in patients with a tethered cord Group A: tethered cord, thick filum 27 (63%), split cord 16 (37%), 1 Type 1, 15 Type 2 B: tethered cord & hamartoma, split cord 8 (19%), 2 Type 1, 6 Type 2 Cutaneous Stigmata total 6 (14%), dermal sinus 5 (12%), hypertrichosis 3 (7%), capillary hemangioma 1 (2%) total 19 (45%), subcutaneous lipoma 7 (17%), dermal sinus 7 (17%), hypertrichosis 6 (14%), capillary hemangioma 1 (2%) Syringomyelia 24 (56%) 6 (14%) 27 lipoma, 7 dermoid cyst, 5 li poma & dermoid cysts, 2 neur enteric cysts, 1 epidermoid cyst Hamartomas

catheter at presentation, even though the great majority of patients had pathological entities at or close to the conus. Group B patients tended to be more affected by neurological deficits than Group A patients (Table 4). Looking at the major presenting complaints revealed similar features and no significant differences between groups. Pain was considered the main problem for the majority of patients (60% and 69% for Groups A and B, respectively).
Management

In Group A, 20 patients underwent 22 operations to untether the cord. Twenty-three patients did not undergo surgery, either because surgery was not recommended (15 cases) or the individual refused it (8 cases). Reasons for recommending conservative therapy were either absence of neurological symptoms or other spinal diseases such as degenerative changes of the spine, which were considered more likely to explain the patients problems. In Group B, 23 patients underwent 29 operations (Figs. 2, 4, and 5), whereas 19 did not undergo surgery (surgery not recommended in 10, surgery refused in 9) (Figs. 1 and 3). Reasons for recommending a conservative approach were absence of recent clinical progression and/or previous operations making it less likely to achieve a benefit with another operation. Comparing clinical presentations of surgically and nonsurgically treated patients in both groups (Table 4), those who did not undergo surgery tended to be less neurologically affected, but this difference was not significant. Table 5 provides an overview about the operations that were performed. For Group A, all untethering operations were finally successful. Two patients with split cord malformations underwent transection of the filum terminale in separate operations representing the 2 patients with incomplete untethering in Table 5, whereas 3 patients had previously undergone surgery at other hospitals. In Group B, 9 lipomas were partially resected (Fig. 5), whereas 8 were decompressed only (Fig. 4). Of the 12 cystic hamartomas, 7 were removed completely (Fig. 2) and 5 incompletely. For cystic hamartomas, the rate of complete resections decreased in secondary operations. All but one cystic hamartoma was completely removed in a first operation, whereas a complete resection was achieved in only 1 of 5 secondary surgeries. Considering the aspect of untethering the cord, this was successful in 22 of 29 operations with no difference between primary and secondary operations. Reasons for unsuccessful untethering were shortened functional nerve roots or sig-

260

J Neurosurg: Spine / Volume 15 / September 2011

Adult tethered cord syndrome

FIG. 1. Left: Sagittal T1-weighted MR image demonstrating a large dermoid cyst from L-4 to L-5. Right: Corresponding axial image showing a split cord malformation Type 1 and attachment of the cyst to the left hemicord. This 50-year-old patient presented with an almost 30year history of back pain and a slight distal paraparesis but no sphincter problems. Surgery was not recommended and would have been refused by the patient. She has been in stable condition for 11 years.

FIG. 3. Sagittal T1-weighted image demonstrating a L24 conus lipoma with a tethered cord in a 54-year-old woman with a 35-year history of back pain, as well as moderate myelopathy with a distal paraparesis and minor sphincter problems. She refused surgery and her condition has remained unchanged for 9 years.

nificant arachnoid adhesions, which were either due to a previous operation or meningeal reactions related to ruptured dermoid cysts.
Outcome

FIG. 2. A and B: Sagittal T2-weighted MR images revealing a large cystic C6T1 lesion associated with a spina bifida occulta at these segments, a C7T1 Klippel-Feil deformity, and a large syrinx from C-2 to C-6. The 50-year-old patient presented with a 4-month history of a rapidly progressing tetraparesis rendering him unable to walk by the time of presentation. Intraoperatively, a dermal sinus was discovered underneath the skin, which led to a large neurenteric cyst as part of a split cord malformation Type 2 with both hemicords on either side. The dermal sinus and the neurenteric cyst were removed completely to untether the cord. C and D: Postoperative sagittal and axial T2weighted images demonstrating the complete resection of the cyst, a reduction of the syrinx, and the 2 hemicords in the reconstructed dural sac. The patient improved significantly and regained his ability to walk but required a walker.

Surgery-related complications were encountered in 8 cases (16% [8 of 51 operations]). We observed no differences between the patient groups. There were 3 CSF leaks, 2 postoperative hematomas, 2 urinary tract infections, and 1 impetigo contagiosa. Permanent surgical morbidity was observed only in Group B at a rate of 7% and this was restricted to revision surgeries. The mean follow-up period for all patients in the study was 60 63 months; for those who underwent surgery it was 60 65 months, and for those who did not undergo surgery it was 60 58 months. Looking at short-term results after 3 months, 31 (61%) of 51 operations resulted in clinical improvement, whereas 17 operations (33%) left the patients unchanged in that period. Three operations in Group B patients were followed by early clinical deterioration. The clinical scores for individual symptoms within the first 3 months
261

J Neurosurg: Spine / Volume 15 / September 2011

J. Klekamp

FIG. 4. A: Sagittal T2-weighted MR image demonstrating a L5S1 conus lipoma in 41-year-old woman with a 20-year history of slight urinary disturbances and no other clinical problems. B: Axial T2-weighted image showing the position of the rotated conus and the lipoma anterior to it. In this case, the lipoma was not resected. Just the thickened filum was transected and a duraplasty graft placed. C and D: Postoperative T2-weighted MR images obtained 1 year postoperatively demonstrating the conus and lipoma comfortably contained in the dural sac despite reinserted lamina. The urinary disturbances improved and the patient has been free of recurrence for 16 months.

are presented in Table 6. For both groups, significant pain relief was achieved (p < 0.01, Fisher exact test). Neurological deficits were not improved significantly with surgery. The preoperative KPS score improved slightly in Group A (not significant) but not in Group B. Comparing primary and secondary operations for both groups combined, a significant pain improvement was reserved for first-time operations (p < 0.01, Fisher exact test), although a trend for improvement was seen after revisions also. Likewise, patients with a split cord malformation did not experience significant improvement when I compared pre- and postoperative scores after 3 months. Except for patients in Group A, patients in all other subgroups tended to experience a slight worsening of bladder function postoperatively with recovery within 3 months. In Group A, catheter dependency in both patients requiring catheterization before surgery remained unchanged postoperatively, and

no other patient became catheter dependent after surgery. In Group B, status in all 4 patients requiring catheters remained unchanged after surgery, and an additional 4 patients became catheter dependent after the operation; in the latter 4 patients, catheter independence was achieved within 3 months. In 36% of the patients with associated syringomyelia, untethering and/or resection of the hamartoma led to a reduction of syrinx size (Fig. 2), whereas in 64% no postoperative change occurred. The results of long-term analyses of clinical recurrence rates are listed in Table 7. In Group A, 24% of patients experienced a clinical recurrence within 10 years of surgery (Fig. 6). In Group B, long-term results tended to be worse, with clinical recurrence rates of 42% after 5 years and 61% after 10 years (Fig. 6A). In both groups, better long-term results were associated with first-time

FIG. 5. Imaging studies obtained in a 34-year-old woman with a 5-year history of pain, myelopathy, and incontinence. A: Sagittal T1-weighted MR image demonstrating an S12 conus lipoma and a tethered cord. B: Corresponding axial image showing the extension of the lipoma into the subcutaneous tissue spreading apart the fascial layer. The patient underwent subtotal resection of the intradural part of the lipoma and transection of the filum, but the subcutaneous portion was not touched. After resection of the lipoma, the capsule was closed with sutures and a duraplasty graft placed. C: Postoperative sagittal T1weighted image revealing the small lipoma remnant attached to the conus. D: Axial T2-weighted image showing the conus and lipoma remnant in the reconstructed dural sac. During the 2-year follow-up period, the patient experienced improvement of her myelopathy and pain but no resolution of incontinence. She delivered a healthy child 18 months after the operation.

262

J Neurosurg: Spine / Volume 15 / September 2011

Adult tethered cord syndrome

TABLE 3: Clinical signs and symptoms at presentation* Group A: tethered cord surgery no surgery B: tethered cord & hamartoma surgery no surgery Hypesthesia 61 68 55 70 68 75 Dysesthesia 34 41 30 27 25 31 Pain 82 77 85 80 79 81 Motor Weakness 48 59 40 65 75 56 Gait Ataxia 50 45 55 63 68 56 Sphincter Disturbance 50 59 40 65 75 56 KPS Score 79 9 78 7 79 10 74 12 72 11 77 13

* Except for KPS scores, which are presented as the mean SD, each value is a percentage.

operations (recurrence rates of 11% after 10 years in Group A and 31% after 10 years in Group B) (Fig. 6B) compared with secondary surgeries (Fig. 6C). Whether untethering was fully achieved in Group B affected longterm results after first operations only (clinical recurrence rates of 19% after 10 years vs 60% after 5 years with incomplete tethering, respectively) (Fig. 6D), whereas all patients undergoing a secondary operation in Group B eventually experienced new neurological problems within 10 years irrespective of the degree of untethering (Fig. 6C) (Table 7). Long-term results of in nonsurgically treated patients were analyzed to determine how many patients had experienced a clinical worsening during follow-up. Of 23 conservatively treated patients in Group A, 7 were lost to follow-up, whereas of 19 nonsurgically treated patients in Group B 2 were lost to follow-up. In the remaining 33 patients (Figs. 1 and 3), no neurological deterioration was
TABLE 4: Clinical scores at presentation for surgically and nonsurgically treated patients* Group Deficit hypesthesia surgery no surgery dysesthesia surgery no surgery pain surgery no surgery motor weakness surgery no surgery gait ataxia surgery no surgery bladder dysfunction surgery no surgery A: Tethered Cord 3.8 0.8 4.1 1.0 4.1 1.0 4.6 0.8 3.4 1.0 3.5 0.9 4.3 0.7 4.5 0.8 4.3 0.8 4.2 0.9 4.0 1.1 4.2 1.2 B: Tethered Cord & Hamartoma 3.6 1.2 4.0 0.9 4.5 1.0 4.5 0.8 3.3 1.1 3.2 0.9 3.6 1.2 4.3 0.9 3.9 1.2 4.3 1.0 3.6 1.4 4.3 0.8

observed among those in whom surgery had not been advised, except in 1 such patient in whom surgery at another hospital resulted in permanent morbidity. Progressive neurological deficits were detected exclusively in symptomatic patients who had refused surgery despite receiving recommendation to undergo the procedure. Four such patients experienced neurological deterioration within 2, 8, 20, and 24 months of their initial presentation. According to Kaplan-Meier analysis, the rate of clinical recurrences among all nonsurgically treated patients was 21% after 10 years. For those patients who had refused an operation, the corresponding value rose to 47% within 5 years (Table 7) (Fig. 6C). Compared with all patients undergoing a first successful untethering procedure, with a clinical recurrence rate of 16% after 10 years, this difference almost reached statistical significance (p = 0.0519, log-rank test). Diagnosis and treatment of congenital malformations such as the tethered cord syndrome in adults is a challenging task, because these malformations are rare and present a wide spectrum of pathologies: some are very simple and easy to manage, such as a low-lying conus with a thick filum terminale, whereas others are very complex with combinations of a split cord malformation and a dysraphic cyst or a lipoma and vertebral dysplasias. In such instances, surgery can be associated with considerable morbidity. Unlike pediatric patients, adults may present with additional degenerative problems that may complicate treatment even further. Even though the term occult spinal dysraphism is used for the pathological entities described in this paper, the disease is anything but occult in many patients. Foot deformities, abnormal hair growth, capillary hemangiomas, or a dermal sinus can be found on inspection in a large number of these patients,21 and they were found more commonly in the more complex Group B patients in the present study (Table 2). Magnetic resonance imaging is most often used to make the neuroradiological diagnosis. The radiological diagnosis of a tethered cord is based on a low position of the conus below L-2,7,38 demonstration of a thickened filum terminale, the presence of a split cord malformation, or a lipoma with extradural extension. Furthermore,
263 Diagnosis and Clinical Presentation

Discussion

* Scores are based on the classification presented in Table 1 and are presented here as the mean SD.

J Neurosurg: Spine / Volume 15 / September 2011

J. Klekamp
TABLE 5: Operations in patients with a tethered cord No. of Patients Group A: tethered cord filum split cord 1st surgery revision surgery B: tethered cord & hamartoma lipoma dermoid cyst lipoma & dermoid neurenteric cyst epidermoid cyst 1st surgery cysts revision surgery cysts No. of Patients Untethering Untethered Incomplete 11 9 15 5 14 2 3 2 1 15 7 Complete Resection Partial Resection Decompression

17 5

2 2 3 2 1 1 5 2 9 2 2 1 1 4 8

20 7 9 5

2 2 2 1 6 1

TABLE 6: Early postoperative clinical results Mean Neurological Score Group hypesthesia preop postop 3 mos dysesthesia preop postop 3 mos pain preop postop 3 mos motor weakness preop postop 3 mos gait ataxia preop postop 3 mos bladder dysfunction preop postop 3 mos KPS score preop postop 3 mos Group A: Tethered Cord 3.8 0.8 3.8 0.8 3.8 0.8 4.1 1.0 4.3 0.8 4.4 0.8 3.4 1.0 3.9 0.7 3.9 0.7* 4.3 0.7 4.4 0.6 4.4 0.6 4.3 0.8 4.4 0.7 4.4 0.7 4.0 1.1 4.0 1.0 4.2 1.0 77 7 78 6 79 8 Group B: Tethered Cord & Hamartoma 3.6 1.2 3.6 1.3 3.8 1.2 4.5 1.0 4.6 0.8 4.5 0.9 3.3 1.1 3.8 1.0 4.2 0.9* 3.6 1.2 3.6 1.1 3.9 1.1 3.9 1.2 3.9 1.3 4.0 1.1 3.6 1.4 3.2 1.6 3.5 1.5 72 11 72 14 72 19 Groups A & B: 1st Op 4.0 0.8 4.0 0.8 4.2 0.7 4.4 0.9 4.6 0.7 4.6 0.7 3.3 1.0 3.9 0.8 4.2 0.7* 4.0 1.1 4.1 1.0 4.2 0.9 4.2 1.2 4.3 1.1 4.3 1.0 3.9 1.3 3.7 1.4 4.0 1.4 75 11 76 12 76 17 Groups A & B: Revision Surgery 2.9 1.2 2.8 1.4 2.8 1.3 4.4 1.2 4.3 1.0 4.1 1.2 3.4 1.2 3.6 1.1 3.8 1.1 3.4 0.9 3.4 0.9 3.8 1.1 3.7 0.8 3.6 1.1 3.8 1.0 3.3 1.2 3.0 1.5 3.3 1.1 72 4 69 12 71 9 Groups A & B: Split Cord 3.4 1.2 3.2 1.3 3.3 1.2 3.7 1.1 4.0 0.9 3.9 1.0 3.2 1.0 3.6 0.9 3.8 0.9 3.6 1.2 3.7 1.1 3.8 1.1 3.6 1.4 3.7 1.4 3.8 1.3 3.8 1.1 3.5 1.4 3.8 1.0 71 12 68 14 72 14

* Significant differences between pre- and postoperative scores after 3 months (p < 0.01, Fisher exact test). Values are shown as mean SD.

264

J Neurosurg: Spine / Volume 15 / September 2011

Adult tethered cord syndrome

FIG. 6. Long-term results (A) for all patients of Groups A and B (p = 0.1119, log-rank test), (B) for first-time operations in patients of Groups A and B (p = 0.179, log-rank test), for (C) all patients combined comparing first-time operations, revision surgeries, and patients who refused surgery (p = 0.0036, log-rank test), and (D) for first-time operations in patients with successful untethering in Groups A and B and in patients with partial untethering in Group B (p = 0.0025, log-rank test). Gr. = group; Part. Un. = partial untethering; Rec. = recommended; Rev. = revision; Unteth. = complete untethering.

shortened nerve roots and arachnoid adhesions between spinal cord and dura after previous operations or ruptures of dermoid cysts may contribute to the tethering problem. Remnants of cyst contents indicating arachnoid reactions may be found attached to spinal cord, cauda equina, 50,52,53 or inside the central canal.23,25,35,37 Various combinations of these tethering mechanisms may be found in an individual patient. For all pathological entities associated with changes of the bony anatomy, bone window CT scans are recommended.57 In such instances, bony landmarks are the best guides during surgery. In complex dysraphic malformations, a 3D reconstruction of CT images can be helpful. However, such examinations do expose the patient to high doses of radiation and should not be performed routinely. Overall, patients presented at a mean age of 46 13 years (range 2374 years). Similar clinical and dysraphic features were reported in other adult series30,31,49,65 as well as those of mixed adult and pediatric populations.69 The female predominance among adult patients with a tethered cord syndrome is a well-established observation.5,31,47,65 A comparison between adult and pediatric series, however, discloses a higher proportion of neurological deficits with
J Neurosurg: Spine / Volume 15 / September 2011

sphincter problems, in particular in children, in whom pain is a much more significant problem.21 In the present study, adults had a long clinical history before presentation. Symptoms were present on average for about 89 years. The pathophysiology of tethered cord syndrome is related most of all to chronic ischemia.74,75 Symptoms may be initiated by sudden efforts, exaggerated bending of the back, trauma, obesity, or pregnancy.5,30,63 In this study, the initial symptom in the long clinical history was often pain, which preceded the development of neurological deficits for quite some time.5,17,30,49 In most patients, neurological deficits were minor at the time of presentation despite the long delay before diagnosis. Sphincter disturbances affected half to two-thirds of the patients (Table 3). Most patients indicated they had only minor dysfunctions such as a feeling of residual urine after voiding or not being able to hold water for as long as they had been used to. Self-catheterization or incontinence was present in 8% of these patients only.50 As one would expect, neurological deficits were more likely and were severe in patients with more complex malformations (Tables 3 and 4). In this series, none of the patients with a dermal si265

J. Klekamp
TABLE 7: Clinical recurrence rates in patients with a tethered cord Recurrence Rate (%) Group A: tethered cord all 1st surgery B: tethered cord hamartoma all 1st surgery complete untethering partial untethering secondary surgery A & B: all patients 1st surgery secondary surgery no surgery all surgery recommended 5 Years 24 11 42 31 19 60 86 22 78 21 47 10 Years 24 11 61 31 19 100 22 100 21

nus had a history of meningitis. This may be related to a selection bias as infections are not uncommon in children with dermal sinus tracts.64 Finally, no patient with an associated syrinx demonstrated ascending neurological deficits correlating to an expanding syrinx. Therefore, syringomyelia was considered clinically irrelevant in these patients. Dysraphic cysts require a complete resection including their capsule to avoid a recurrence.41 Excellent outcomes have been reported after complete resections of neurenteric cysts1,4,6,12 despite recurrence rates reported as high as 37%13 and 38%.66 In the present series, 7 of 12 cystic hamartomas were removed completely. In first operations, 6 of 7 cysts could be totally resected, whereas only 1 of 5 was completely removed in a second operation. Recurrent dermoid cysts or arachnoid reactions after cyst rupture may cause severe problems to identify and dissect the cyst capsule without damage to spinal cord or nerve roots.5052 It has been estimated that no more than 40% of dermoid cysts are totally resectable.10 A complete resection was achieved in 4 of 8 patients with dermoid cysts in this study. Mathew and Todd52 removed 6 of 21 dermoid cysts in the cauda equina area completely, whereas Lunardi et al.50 achieved a complete resection of 3 of 8 dermoid cysts. Because lipomas do not have any proliferating potential18 and behave according to the rest of the body fat,19,63 neurological progression may be related to weight gain, pregnancy, or steroid therapy, with possible restitution after weight loss, child birth, or termination of steroids. The absence of proliferative potential raises the question of whether a lipoma should be resected at all. Some studies have emphasized the importance of a complete lipoma resection to achieve a favorable long-term outcome, 21,61 whereas others found no such correlation.63 After partial lipoma resections, severe arachnoid scarring may attach
266 Surgical Management: Hamartomas and Syringomyelia

the conus and lipoma to the dura.61,63 This is probably the main reason for the rather unsatisfactory outcome of surgical revisions in Group B patients. Therefore, Pang et al.60,61 changed their strategy for lipoma surgery to a more aggressive approach; they completely resected the lesion and closed the placode with pial sutures to lower the risk of postoperative retethering. In the present series, a complete lipoma resection was never attempted. If the lipoma was small and the spinal cord or cauda appeared not to be compressed, tethering was considered the exclusive cause of neurological symptoms. In such cases, the lipoma was left intact and the cord was untethered (Fig. 3). This policy avoids any formation of arachnoid adhesions associated with partial resections. If the lipoma appeared to be of considerable size sufficient to compress spinal cord and cauda or was involved in the tethering, its mass was reduced and the outer capsule was preserved and sutured at the end of the debulking. This was considered to limit the risk of postoperative tethering, analogous to the effect of pial sutures after removal of intramedullary tumors.41 In this series every syrinx was considered asymptomatic70 and of small size in most patients. Shunting of a syrinx associated with a tethered cord has been advocated by some authors.2,20,21,32 However, the course of a syrinx is related to CSF flow and cord tethering. If untethering is successful and CSF flow maintained, the syrinx will not become symptomatic and sizeable cavities will become smaller (Fig. 2).8,11,22,29,46,56,63,73 Therefore, the presence of syringomyelia does not require additional surgical measures.
Surgical Management: Untethering and Prevention of Retethering

In the present study, untethering was considered successful, if all tethering components could be released as judged by intraoperative assessment. This was achieved in 42 of 51 operations. In Group A, all untethering procedures were successful with 2 patients with split cord malformations treated early in the series undergoing transection of the filum in a second operation. Presently, both untethering the split cord and transection of the filum are performed in a single procedure.26 In Group B, untethering was successful in 22 (76%) of 29 operations. Htt mann et al.30 reported successful untethering procedures in 82% of their 54 adult patients; they did not provide separate analyses for subgroups. Several features may be involved in the tethering process in an individual patient. Not all of them can be identified on MR imaging and some have to be looked for during surgery. Chances for successful untethering correlate with the complexity of the malformation.44 Therefore, patients with complex malformations should be treated by surgeons with considerable experience in the management of these complex lesions. In the simplest type of tethering (that is, for a tight filum terminale), the cord can be released with transection of the filum. With split cord malformations, the spinal cord is tethered at the site where either intradural mesenchymal tissue and/or an epidural bony spur splits the cord.
J Neurosurg: Spine / Volume 15 / September 2011

Adult tethered cord syndrome

In either case, aberrant nerve roots on the medial surfaces of both hemicords, hamartomas, and other mesenchymal tissue may connect the hemicords to these separating structures and contribute to the tethering (Figs. 1 and 2). All of these structures need to be resected to untether the cord.57,58 At the end of surgery both hemicords should be completely free of any posterior or anterior attachments.58 In patients with lipomas that extend extradurally, the lipoma itself tethers the cord at the site of dural penetration.14,54 Therefore, the dura should be circumcised at this point and the lipoma transected, separating the intra- and extradural parts from each other.14 After that, the intradural portion can be managed as described. The epidural part should be left in place to avoid a large soft-tissue defect, which may predispose the patient to CSF fistulas21,60 and wound-healing problems (Fig. 5).60,63 The tethering effect of shortened nerve roots or arachnoid adhesions, however, is more difficult to manage. Arachnoid adhesions should be dissected using microscissors. Blunt dissection should not be used because it may tear blood vessels embedded in the thickened arachnoid.41,42 With shortened nerve roots, intraoperative dissection may disclose the nerves blind ending in the dura or the fibrous tissue of a split cord malformation. Otherwise, intraoperative stimulation should be used to identify roots that have to be preserved. In such situations, a compromise may be required (for example, a partial untethering to avoid neurological deficits). To limit the risk of retethering, meticulous hemostasis is required during the entire operation to avoid any contamination of the subarachnoid space with blood because this could provoke arachnoid adhesions. Furthermore, the use of duraplasty to enlarge the subarachnoid space is recommended for patients with a tethered cord in general.16,33,60,67,76 Samuels et al.67 observed a considerably lower rate of retethering in patients with complex malformations if a duraplasty graft was placed rather than performing primary dural closure. The type of material that should be used is a matter of debate. The prevention of postoperative adhesions between spinal cord and the duraplasty graft has been evaluated by examining data from the authors spinal cord database for patients with syringomyelia42 and spinal cord tumors.41 Artificial materials for dural grafting were less often associated with the formation of such adhesions than autologous grafts or primary dural closures63 and were used in all but 3 patients in the present study. Judging from revision surgeries, retethering is very common along the suture line of the dura mater. Therefore, the duraplasty and suture line should be raised away from the spinal cord by means of tenting sutures, which are placed in the suture line and anchored laterally to periosteum, muscle attachments, or facet joints.41,42 Almost all major series on tethered cord syndromes deal with children or describe patient groups in which children predominate. It appears that postoperative improvements are more common in children than adults,14,61,68 even though most pediatric studies report neurological stabilization as the most likely outcome.43,48,51,62,71 The
J Neurosurg: Spine / Volume 15 / September 2011 Postoperative Outcome: Short-Term Clinical Results

only major series on adult patients with tethered cord syndromes were published by Httmann et al.30 in 2001 (54 patients), Iskandar et al.31 in 2001 (36 patients), and Rajpal et al.65 in 2007 (61 patients). In the present series of 51 operations in 43 adult patients, 61% considered their postoperative result after 3 months to be improved, whereas, in the same period, 33% reported no change, bearing in mind that most patients did not have severe neurological deficits before surgery. Six percent considered their condition to be worsened. Httmann et al.30 reported postoperative improvements in 85% of their patients, stabilizations in 7%, and deterioration in 7%. Looking at individual symptoms within the first 3 months, pain improved significantly with no significant changes of preoperative neurological deficits in either group (Table 6).49,65 Rajpal et al.65 reported postoperative improvement of pain in 70% of their patients, whereas in 23% pain was left unchanged. In the present study, looking at the results of surgery, it became obvious that pain improvement was restricted to first operations and almost never achieved in revision surgeries. The results, however, suggest that one should advise revision surgery in Group B patients in selected cases only. It appears that further clinical deterioration can only be postponed by surgical intervention but not prevented in the long term. A separate analysis for patients with split cord malformations revealed no significant clinical changes postoperatively, with a trend toward improvement of pain that just failed to reach statistical significance (Table 6).24 Surgical morbidity in patients with spinal dysraphism is related to the complexity of the malformation and the presence of arachnoid adhesions.63 In the present study, surgical morbidity was 7% in Group B and 0% in Group A. Complications were encountered in 16%. Almost identical morbidity figures have been published in the literature.61,63,73 Looking at long-term results for surgically treated tethered cord syndromes, late deteriorations may develop due to regrowth of a cystic hamartoma, postoperative spinal instability, or symptomatic retethering of the cord,28,30 with symptomatic retethering being the most common cause. In the present study, retethering was considered to be the cause in every case in which a late deterioration developed after surgery. In their study on 54 adults, Httmann et al.30 observed a clinical recurrence rate of 16% after complete untethering, whereas with incomplete untethering the recurrence rate rose to 80%. The authors concluded that surgery provided a long-term benefit for the patients in whom untethering could be achieved. Iskandar et al.31 found long-term improvement in 21 (78%) of 27 patients, with no change in 4 (15%) and worsening in 2 (7%) patients without applying Kaplan-Meier analysis. Looking at the long-term results in the present series (Table 7), similar results are seen in cases of successful and incomplete untethering, respectively. In Group A, clinical stabilization was achieved for a minimum of 10 years in 76% of patients (Fig. 6C). If first-time surgery
267 Postoperative Outcome: Long-Term Clinical Results

J. Klekamp
was performed, the clinical stabilization rate rose to 89%. In Group B, the corresponding rate for complete untethering in a first operation was 81%. After incomplete untethering in Group B, the clinical recurrence rate rose to 60% within 5 years. Revision surgeries for this group failed to stabilize the clinical course in the long term (Table 7) (Figs. 6). The considerable worsening of the long-term prognosis in Group B, once a first surgical attempt was not successful, places an enormous responsibility on the surgeon conducting the initial surgery. Comparing these data with pediatric series, one finds similar results with rates for symptomatic retethering reported between 10% and 26% and a trend for worse results for more complex malformations.3,15,16,21,44,55,72 In their series of 238 patients, Pang et al.61 reported that 82.8% of their patients were free from neurological progression for 16 years, which they attributed to their aggressive approach to lipoma resection. In an earlier study with partial lipoma resections, this rate had been higher34.6% for 10.5 years. Leaving a partially resected lipoma open to the subarachnoid space was associated with a considerable risk for retethering and was probably the major cause of the poor results after secondary operations in Group B patients in the present study as well as in other reports.5,16,21,60 However, closing the lipoma capsule after partial resection, as described, may serve the same purpose, as suggested by the similar retethering rates of this study.
Comparison of Surgically and Nonsurgically Treated Patients

We still lack precise information on the natural history of tethered cord syndromes. Consequently, the controversy concerning prophylactic surgery of these lesions is still unresolved. This debate started as early as 1918, when Brickner9 suggested surgery to treat asymptomatic infants in the hope of preventing future deterioration. In a comparative study of operated and unoperated conus lipomas, Kulkarni et al.45 reported follow-up results in 2 groups comprising 53 asymptomatic patients: those obtained not surgically treated (data accrued since 1994) and those prophylactically surgically treated (data acquired between 1972 and 1994). According to their Kaplan-Meier analysis, the risk of deterioration within 9 years was 33% for conservatively treated patients and 46% for surgically treated patients. This difference was not statistically significant, and the authors concluded that there was no evidence for a prophylactic effect to justify surgery of asymptomatic conus lipomas. Pang et al.,61 however, observed a prophylactic effect in asymptomatic children who underwent surgery. After 16 years, 98.4% of the patients remained neurologically intact. In the present series, surgery was offered to 60 of 85 patients because of pain and/or neurological symptoms that were either progressing or considered improvable with surgery. The postoperative clinical recurrence rates in Groups A and B were 11% and 31%, respectively, after 10 years in cases in which this was the initial surgery (Figs. 6BD). For patients in whom surgery was not performed, 1 of 16 patients in Group A and 4 of 17 in Group B suffered neurological progression. Of these 5 patients, just 1 in Group B had not been advised to undergo surgery
268

because progressive symptoms were absent at presentation. In that case, the clinical deterioration was related to surgical morbidity incurred at another institution and not to the natural history. The other 4 patients had refused surgery despite neurological symptoms and a recommendation to undergo surgery; their neurological symptoms deteriorated further. Even the majority of those patients who had presented with neurological symptoms reported a stable clinical situation throughout follow-up. The corresponding clinical recurrence rates for all nonsurgically treated patients combined was 21% after 10 years and the rate increased to 47% in 5 years, if calculated separately for patients with a recommendation for surgery (Fig. 6C). This rate is more than twice that in patients who underwent surgery for the first time with successful untethering but just missed the level of statistical significance. Do these results have implications for the management of asymptomatic children? This study implies that not all asymptomatic children will become symptomatic. Some patients in this study had been asymptomatic even throughout their adult life when symptoms finally arose with advancing age. How many and which patients will develop neurological problems remain questions not known with certainty. Once patients have reached adulthood, the clinical course appears to be more benign than it does in symptomatic children, as indicated by the low number of adult patients with severe deficits and the significant proportion of them refusing surgery: 32% in this study and 12 of 20 patients in a report by Dz et al.17 Thus, prophylactic surgery in asymptomatic patients requires an extremely high standard of treatment for justification. The results of this study do not support a recommendation for prophylactic surgery in asymptomatic patients with tethered cord syndromes. The essential preconditions for optimal management are an early diagnosis and close clinical monitoring to be able to intervene as soon as neurological symptoms appear or progress.27,65

The present study demonstrates that complete untethering in symptomatic adult patients with tethered cord syndromes undergoing surgery for the first time improved pain and prevented further neurological progression in 81%89% of patients. In 47% of symptomatic patients who refused an operation, further progression of the deficit occurred. On the one hand, these rates indicate that untethering has a prophylactic effect in symptomatic adults with a tethered cord. On the other hand, this study also shows that not every adult patient requires surgery. No progressive clinical worsening was observed in patients without neurological symptoms or in those in whom other factors such as degenerative spinal diseases were considered responsible for the clinical condition. For an adult patient with a tethered cord only (those in Group A, for example), surgery should be recommended as soon as neurological symptoms are present or pain attributable to the tethered cord has appeared. For adult patients with complex malformations (those in Group B, for example), surgery should be advised as soon as symptoms progress or pain has become severe. Secondary opJ Neurosurg: Spine / Volume 15 / September 2011

Conclusions

Adult tethered cord syndrome

erations in patients with complex malformations should be undertaken in exceptional cases only because surgical morbidity is higher and a long-term benefit less likely. The long-term prognosis appears to be related to the rate of symptomatic retethering. All intraoperative steps should be planned and executed carefully to minimize this risk.
Disclosure The author reports no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper. Acknowledgment The author expresses his sincere gratitude to Madjid Samii, M.D., former chairman of the neurosurgical clinic at Nordstadt Hospital in Hannover, Germany, for his continuous support of this work. References 1. Agnoli AL, Laun A, Schnmayr R: Enterogenous intraspinal cysts. J Neurosurg 61:834840, 1984 2. Aoyama T, Ohtaki M, Koyanagi I, Kawamura M, Chiba M: Syringo-subarachnoidal shunting to correct unilateral leg deformity in a child with terminal syringomyelia: case report. Childs Nerv Syst 24:869873, 2008 3. Arai H, Sato K, Okuda O, Miyajima M, Hishii M, Nakanishi H, et al: Surgical experience of 120 patients with lumbosacral lipomas. Acta Neurochir (Wien) 143:857864, 2001 4. Arai Y, Yamauchi Y, Tsuji T, Fukasaku S, Yokota R, Kudo T: Spinal neurenteric cyst. Report of two cases and review of forty-one cases reported in Japan. Spine (Phila Pa 1976) 17: 14211424, 1992 5. Aufschnaiter K, Fellner F, Wurm G: Surgery in adult onset tethered cord syndrome (ATCS): review of literature on occasion of an exceptional case. Neurosurg Rev 31:371384, 2008 6.Bassiouni H, Hunold A, Asgari S, Hbschen U, Knig HJ, Stolke D: Spinal intradural juxtamedullary cysts in the adult: surgical management and outcome. Neurosurgery 55:1352 1360, 2004 7. Bauer DF, Shoja MM, Loukas M, Oakes WJ, Tubbs RS: Study of the effects of flexion on the position of the conus medullaris. Childs Nerv Syst 24:10431045, 2008 8. Beaumont A, Muszynski CA, Kaufman BA: Clinical significance of terminal syringomyelia in association with pediatric tethered cord syndrome. Pediatr Neurosurg 43:216221, 2007 9. Brickner WM: Spina bifida occulta. Am J Med Sci 155:473 502, 1918 10. Bristow RG, Laperriere NJ, Tator C, Milosevic M, Wong CS: Post-operative radiotherapy for recurrent dermoid cysts of the spine: a report of 3 cases. J Neurooncol 33:251256, 1997 11. Caird J, Flynn P, McConnell RS: Significant clinical and radiological resolution of a spinal cord syrinx following the release of a tethered cord in a patient with an anatomically normal conus medullaris. Case report. J Neurosurg Pediatr 1:396398, 2008 12. Chang IC: Surgical experience in symptomatic congenital intraspinal cysts. Pediatr Neurosurg 40:165170, 2004 13. Chavda SV, Davies AM, Cassar-Pullicino VN: Enterogenous cysts of the central nervous system: a report of eight cases. Clin Radiol 36:245251, 1985 14. Choux M, Lena G, Genitori L, Foroutan M: The surgery of occult spinal dysraphism. Adv Tech Stand Neurosurg 21:183 238, 1994 15. Chumas PD: The role of surgery in asymptomatic lumbosacral spinal lipomas. Br J Neurosurg 14:301304, 2000 16. Colak A, Pollack IF, Albright AL: Recurrent tethering: a common long-term problem after lipomyelomeningocele repair. Pediatr Neurosurg 29:184190, 1998 17. Dz B, Gocmen S, Secer HI, Basal S, Gnl E: Tethered cord syndrome in adulthood. J Spinal Cord Med 31:272278, 2008 18. Ehni G, Love JG: Intraspinal lipomas: report of cases, review of the literature, and clinical and pathologic study. Arch Neurol Psychiat 53:128, 1945 19. Endoh M, Iwasaki Y, Koyanagi I, Hida K, Abe H: Spontaneous shrinkage of lumbosacral lipoma in conjunction with a general decrease in body fat: case report. Neurosurgery 43: 150152, 1998 20. Erkan K, Unal F, Kiris T, Karalar T: Treatment of terminal syringomyelia in association with tethered cord syndrome: clinical outcomes with and without syrinx drainage. Neurosurg Focus 8(3):e9, 2000 21. Finn MA, Walker ML: Spinal lipomas: clinical spectrum, embryology, and treatment. Neurosurg Focus 23(2):E10, 2007 22. Fujimura Y, Kimura F, Ishida S, Shinoda K, Ohsawa N: [An adult case of tethered cord syndrome with lipoma and thoraco-lumbar syringomyelia presenting slow progressive muscular atrophy in the lower limbs.] Rinsho Shinkeigaku 34: 918921, 1994 (Jpn) 23. Garg A, Gupta V, Gaikwad S, Deol P, Mishra NK, Suri A, et al: Isolated central canal rupture of spinal dermoid: report of two cases. Australas Radiol 47:194197, 2003 24. Gower DJ, Del Curling O, Kelly DL Jr, Alexander E Jr: Diastematomyeliaa 40-year experience. Pediatr Neurosci 14: 9096, 1988 25. Goyal A, Singh D, Singh AK, Gupta V, Sinha S: Spontaneous rupture of spinal dermoid cyst with disseminated lipid droplets in central canal and ventricles. J Neurosurg Sci 48: 6365, 2004 26. Guthkelch AN, Hoffmann GT: Tethered spinal cord in association with diastematomyelia. Surg Neurol 15:352354, 1981 27. Hajnovic L, Trnka J: Tethered spinal cord syndromethe importance of time for outcomes. Eur J Pediatr Surg 17:190 193, 2007 28. Herman JM, McLone DG, Storrs BB, Dauser RC: Analysis of 153 patients with myelomeningocele or spinal lipoma reoperated upon for a tethered cord. Presentation, management and outcome. Pediatr Neurosurg 19:243249, 1993 29. Hsu AR, Hou LC, Veeravagu A, Barnes PD, Huhn SL: Resolution of syringomyelia after release of tethered cord. Surg Neurol 72:657661, 2009 30. Httmann S, Krauss J, Collmann H, Srensen N, Roosen K: Surgical management of tethered spinal cord in adults: report of 54 cases. J Neurosurg 95 (2 Suppl):173178, 2001 31. Iskandar BJ, Fulmer BB, Hadley MN, Oakes WJ: Congenital tethered spinal cord syndrome in adults. Neurosurg Focus 10(1):e7, 2001 32. Iskandar BJ, Oakes WJ, McLaughlin C, Osumi AK, Tien RD: Terminal syringohydromyelia and occult spinal dysraphism. J Neurosurg 81:513519, 1994 33. Kang JK, Lee KS, Jeun SS, Lee IW, Kim MC: Role of surgery for maintaining urological function and prevention of retethering in the treatment of lipomeningomyelocele: experience recorded in 75 lipomeningomyelocele patients. Childs Nerv Syst 19:2329, 2003 34. Kaplan EL, Meier P: Nonparametric estimation from incomplete observations. J Am Stat Assoc 53:457481, 1958 35. Karadag D, Karaglle AT, Erden A, Erden I: MR imaging of a ruptured intraspinal dermoid tumour with fat droplets in the central spinal canal. Australas Radiol 46:444446, 2002 36. Karnofsky DA, Burchenal JH: The clinical evaluation of chemotherapeutic agents in cancer, in MacLeod CM (ed): Evaluation of Chemotherapeutic Agents. New York: Columbia Uni versity Press, 1949, pp 191205 37. Kasliwal MK, Sinha S, Sharma BS, Garg A: Symptomatic

J Neurosurg: Spine / Volume 15 / September 2011

269

J. Klekamp
central canal rupture heralding the presence of an asymptomatic conus dermoid. J Neurooncol 84:3940, 2007 38. Kesler H, Dias MS, Kalapos P: Termination of the normal conus medullaris in children: a whole-spine magnetic resonance imaging study. Neurosurg Focus 23(2):E7, 2007 39. Klekamp J, Raimondi AJ, Samii M: Occult dysraphism in adult hood: clinical course and management. Childs Nerv Syst 10:312320, 1994 40. Klekamp J, Samii M: Introduction of a score system for the clinical evaluation of patients with spinal processes. Acta Neurochir (Wien) 123:221223, 1993 41. Klekamp J, Samii M: Surgery of Spinal Tumors. Heidelberg: Springer Verlag, 2007 42. Klekamp J, Samii M: SyringomyeliaDiagnosis and Treatment. New York: Springer Verlag, 2001 43. Koyanagi I, Iwasaki Y, Hida K, Abe H, Isu T, Akino M: Surgical treatment supposed natural history of the tethered cord with occult spinal dysraphism. Childs Nerv Syst 13:268274, 1997 44. Koyanagi I, Iwasaki Y, Hida K, Abe H, Isu T, Akino M, et al: Factors in neurological deterioration and role of surgical treatment in lumbosacral spinal lipoma. Childs Nerv Syst 16: 143149, 2000 45. Kulkarni AV, Pierre-Kahn A, Zerah M: Conservative management of asymptomatic spinal lipomas of the conus. Neurosurgery 54:868875, 2004 46. La Marca F, Grant JA, Tomita T, McLone DG: Spinal lipomas in children: outcome of 270 procedures. Pediatr Neurosurg 26:816, 1997 47. Lad SP, Patil CG, Ho C, Edwards MS, Boakye M: Tethered cord syndrome: nationwide inpatient complications and outcomes. Neurosurg Focus 23(2):E3, 2007 48. Lagae L, Verpoorten C, Casaer P, Vereecken R, Fabry G, Plets C: Conservative versus neurosurgical treatment of tethered cord patients. Z Kinderchir 45 (Suppl 1):1617, 1990 49. Lapsiwala SB, Iskandar BJ: The tethered cord syndrome in adults with spina bifida occulta. Neurol Res 26:735740, 2004 50. Lunardi P, Missori P, Gagliardi FM, Fortuna A: Long-term results of the surgical treatment of spinal dermoid and epidermoid tumors. Neurosurgery 25:860864, 1989 51. Maiuri F, Gangemi M, Cavallo LM, De Divitiis E: Dysembryogenetic spinal tumours in adults without dysraphism. Br J Neurosurg 17:234238, 2003 52. Mathew P, Todd NV: Intradural conus and cauda equina tumours: a retrospective review of presentation, diagnosis and early outcome. J Neurol Neurosurg Psychiatry 56:6974, 1993 53. Messori A, Polonara G, Serio A, Gambelli E, Salvolini U: Expanding experience with spontaneous dermoid rupture in the MRI era: diagnosis and follow-up. Eur J Radiol 43:1927, 2002 54. Muthukumar N: Congenital spinal lipomatous malformations: part IClassification. Acta Neurochir (Wien) 151:179188, 197, 2009 55. Muthukumar N: Congenital spinal lipomatous malformations: part IIClinical presentation, operative findings, and outcome. Acta Neurochir (Wien) 151:189197, 2009 56. Ng WH, Seow WT: Tethered cord syndrome preceding syrinx formationserial radiological documentation. Childs Nerv Syst 17:494496, 2001 57. Pang D: Split cord malformation: Part II: Clinical syndrome. Neurosurgery 31:481500, 1992 58. Pang D: Ventral tethering in split cord malformation. Neurosurg Focus 10(1):e6, 2001 59. Pang D, Dias MS, Ahab-Barmada M: Split cord malformation: Part I: A unified theory of embryogenesis for double spinal cord malformations. Neurosurgery 31:451480, 1992 60. Pang D, Zovickian J, Oviedo A: Long-term outcome of total and near-total resection of spinal cord lipomas and radical reconstruction of the neural placode: part Isurgical technique. Neurosurgery 65:511529, 2009 61. Pang D, Zovickian J, Oviedo A: Long-term outcome of total and near-total resection of spinal cord lipomas and radical reconstruction of the neural placode, part II: outcome analysis and preoperative profiling. Neurosurgery 66:253273, 2010 62. Peter JC: Occult dysraphism of the spine. A retrospective analysis of 88 operative cases, 1979-1989. S Afr Med J 81: 351354, 1992 63. Pierre-Kahn A, Zerah M, Renier D, Cinalli G, Sainte-Rose C, Lellouch-Tubiana A, et al: Congenital lumbosacral lipomas. Childs Nerv Syst 13:298335, 1997 64. Radmanesh F, Nejat F, El Khashab M: Dermal sinus tract of the spine. Childs Nerv Syst 26:349357, 2010 65. Rajpal S, Tubbs RS, George T, Oakes WJ, Fuchs HE, Hadley MN, et al: Tethered cord due to spina bifida occulta presenting in adulthood: a tricenter review of 61 patients. J Neurosurg Spine 6:210215, 2007 66. Rauzzino MJ, Tubbs RS, Alexander E III, Grabb PA, Oakes WJ: Spinal neurenteric cysts and their relation to more common aspects of occult spinal dysraphism. Neurosurg Focus 10(1):e2, 2001 67. Samuels R, McGirt MJ, Attenello FJ, Garcs Ambrossi GL, Singh N, Solakoglu C, et al: Incidence of symptomatic retethering after surgical management of pediatric tethered cord syndrome with or without duraplasty. Childs Nerv Syst 25:10851089, 2009 68. Satar N, Bauer SB, Shefner J, Kelly MD, Darbey MM: The effects of delayed diagnosis and treatment in patients with an occult spinal dysraphism. J Urol 154:754758, 1995 69. Sattar MT, Bannister CM, Turnbull IW: Occult spinal dysraphismthe common combination of lesions and the clinical manifestations in 50 patients. Eur J Pediatr Surg 6 (Suppl 1):1014, 1996 70. Scatliff JH, Kendall BE, Kingsley DP, Britton J, Grant DN, Hayward RD: Closed spinal dysraphism: analysis of clinical, radiological, and surgical findings in 104 consecutive patients. AJR Am J Roentgenol 152:10491057, 1989 71. Schmidt DM, Robinson B, Jones DA: The tethered spinal cord. Etiology and clinical manifestations. Orthop Rev 19: 870876, 1990 72. Van Calenbergh F, Vanvolsem S, Verpoorten C, Lagae L, Casaer P, Plets C: Results after surgery for lumbosacral lipoma: the significance of early and late worsening. Childs Nerv Syst 15:439443, 1999 73. Xenos C, Sgouros S, Walsh R, Hockley A: Spinal lipomas in children. Pediatr Neurosurg 32:295307, 2000 74. Yamada S, Iacono RP, Andrade T, Mandybur G, Yamada BS: Pathophysiology of tethered cord syndrome. Neurosurg Clin N Am 6:311323, 1995 75. Yamada S, Won DJ, Pezeshkpour G, Yamada BS, Yamada SM, Siddiqi J, et al: Pathophysiology of tethered cord syndrome and similar complex disorders. Neurosurg Focus 23(2):E6, 2007 76. Zide B, Constantini S, Epstein FJ: Prevention of recurrent tethered spinal cord. Pediatr Neurosurg 22:111114, 1995 Manuscript submitted July 12, 2010. Accepted April 18, 2011. Please include this information when citing this paper: published online May 20, 2011; DOI: 10.3171/2011.4.SPINE10504. Address correspondence to: Jrg Klekamp, M.D., Christliches Krankenhaus, Department of Neurosurgery, Danziger Strasse 2, 49610 Quakenbrck, Germany. email: j.klekamp@ckq-gmbh.de.

270

J Neurosurg: Spine / Volume 15 / September 2011