scholarly journals Chinese expert consensus on diagnosis and management of split cord malformation

2020 ◽  
Vol 8 (2) ◽  
pp. 83-92
Author(s):  
Bo Xiu
Keyword(s):  
Spinal Cord ◽  
Neural Tube ◽  
Neural Tube Defect ◽  
Working Group ◽  
Surgical Techniques ◽  
Tethered Cord ◽  
Split Cord Malformation ◽  
Clinical Presentations ◽  
Sensorimotor Deficit

Split cord malformation (SCM) is a neural tube defect that the spinal cord is longitudinally separated into two hemicords with individual functions, which causes severe spinal cord impairment and sensorimotor deficit. As a kind of myelodysplasia and a special type of tethered cord syndrome, SCM is not widely understood, and common issues in the diagnosis and treatment of SCM should be clarified. In this paper, the Chinese Split Cord Malformation Working Group made a consensus for SCM on embryopathogenesis and types, clinical presentations, neuroimaging assessment, indications and principle of the surgery, surgical techniques and nuances, and prognosis and follow up.

Congenital tethered spinal cord syndrome in adults

1998 ◽  
Vol 88 (6) ◽  
pp. 958-961 ◽  
Author(s):  
Bermans J. Iskandar ◽  
Benjamin B. Fulmer ◽  
Mark N. Hadley ◽  
W. Jerry Oakes
Keyword(s):  
Spinal Cord ◽  
Tethered Cord ◽  
Tethered Cord Syndrome ◽  
Early Surgery ◽  
Split Cord Malformation ◽  
Tethered Spinal Cord ◽  
Content Type ◽  
Fine Print

Object. The management of tethered spinal cord syndrome with onset of symptomatology occurring in adulthood remains controversial, although the necessity of early surgery in the pediatric tethered cord syndrome population is well established. To ascertain the results of surgery in adult patients with this anomaly, the authors undertook a retrospective review of 34 cases. Methods. The authors studied the hospital records of 34 consecutive patients who presented in adulthood with tethered cord syndrome and conducted follow-up phone interviews with 28 of them. The population consisted of 12 men and 22 women, ranging in age from 18 to 70 years (mean 34 years). The most common presenting feature was pain, followed by weakness and incontinence. All patients underwent surgery. The most common operative findings were tight filum terminale, split cord malformation, and lipomyelomeningocele, paralleling those observed in pediatric studies. Long-term surgical results and patient outcome ratings were encouraging. After a mean clinical follow-up period of 4 years, significant improvement occurred in 22 of 27 patients presenting with pain, 13 of 27 patients with motor or sensory dysfunction, and 11 of 18 patients with bowel and bladder disturbance. In addition, telephone interviews were obtained after a period of 8.6 years. Twenty-two (79%) of 28 patients called the operation a long-term success; 21 (75%) of 28 patients believed that they had significant postoperative improvement (and not just stabilization) in pain and/or neurological function. Surgical complications were generally minor. Nineteen (86%) of 22 employed patients returned to work after surgery. Two (33%) of six patients who were not employed before surgery worked full time postoperatively. Only two of the 28 patients interviewed had received Workers' Compensation benefits; both of these had good outcomes and returned to work. Conclusions. Tethered spinal cord syndrome in adults is an uncommon entity that can become symptomatic. Although surgery in adults involves greater risk of neurological injury than in children, it is a low-risk procedure with encouraging results. Because neurological deficits are generally irreversible, early surgery is recommended.

scholarly journals Congenital tethered spinal cord syndrome in adults

2001 ◽  
Vol 10 (1) ◽  
pp. 1-5 ◽  
Author(s):  
Bermans J. Iskandar ◽  
Benjamin B. Fulmer ◽  
Mark N. Hadley ◽  
W. Jerry Oakes
Keyword(s):  
Spinal Cord ◽  
Tethered Cord ◽  
Tethered Cord Syndrome ◽  
Early Surgery ◽  
Split Cord Malformation ◽  
Full Time ◽  
Tethered Spinal Cord ◽  
Sensory Dysfunction

Object The management of tethered cord syndrome with onset of symptomatology occurring in adulthood remains controversial, although the necessity of early surgery in the pediatric tethered cord syndrome population is well established. To ascertain the results of surgery in adult patients with this anomaly, the authors undertook a retrospective review of 34 cases. Methods The authors studied the hospital records of 34 consecutive patients who presented in adulthood with tethered cord syndrome and conducted follow-up phone interviews with 28 of them. The population consisted of 12 men and 22 women, ranging in age from 18 to 70 years (mean 34 years). The most common presenting feature was pain, followed by weakness and incontinence. All patients underwent surgery. The most common operative findings were tight filum terminale, split cord malformation, and lipomyelomeningocele, paralleling those observed in pediatric studies. Long-term surgical results and patient outcome ratings were encouraging. After a mean clinical follow-up period of 4 years, significant improvement occurred in 22 of 27 patients presenting with pain, 13 of 27 patients with motor or sensory dysfunction, and 11 of 18 patients with bowel and bladder disturbance. In addition, telephone interviews were obtained after a period of 8.6 years. Twenty-two (79%) of 28 patients called the operation a long-term success; 21 (75%) of 28 patients believed that they had significant postoperative improvement (and not just stabilization) in pain and/or neurological function. Surgical complications were generally minor. Surgery-related complications included one cerebrospinal fluid leak and five pseudomeningoceles. Two patients complained of worsening motor or sensory dysfunction and one of worsening bladder dysfunction, whereas four patients experienced persistent pain and one complained of worsening pain postoperatively. According to the patients, the vast majority of these complications were minor. Nineteen (86%) of 22 employed patients returned to work after surgery. Two (33%) of six patients who were not employed before surgery worked full time postoperatively. Only two of the 28 patients interviewed had received Workers' Compensation benefits; both of these had good outcomes and returned to work. Conclusions Tethered spinal cord syndrome in adults is an uncommon entity that can become symptomatic. Although surgery in adults involves greater risk of neurological injury than in children, it is a low-risk procedure with encouraging results. Because neurological deficits are generally irreversible, early surgery is recommended.

scholarly journals Limited dorsal myeloschisis in three cats: a distinctive form of neural tube defect

2020 ◽  
Vol 6 (1) ◽  
pp. 205511692092430
Author(s):  
Sarah Butterfield ◽  
Beatriz Garcia-Gonzalez ◽  
Colin J Driver ◽  
Clare Rusbridge
Keyword(s):  
Spinal Cord ◽  
Neural Tube ◽  
Neural Tube Defect ◽  
Case Series ◽  
Tethered Cord ◽  
Neurological Deficits ◽  
Skin Defect ◽  
Mri Features ◽  
Spinal Cord Segment ◽  
Mri Sequences

Case series summary The aim of this case series was to describe the clinical presentation, imaging findings and histopathology of three cats with limited dorsal myeloschisis (LDM). The history, examination and MRI sequences were reviewed in three cases presented to a single referral hospital. The surgery report and histopathology were described in two cases. All cats were young (10 weeks old, 5 months old, 4 years old), presenting with varying degrees of progressive paraparesis. All had a midline skin defect overlying the spinal column that was either sunken or saccular, containing fluid thought to be cerebrospinal fluid. MRI sequences demonstrated tissue extending from the dura through an overlying bifid spinous process and attached to the dermis, with associated spinal cord tethering, atrophy and syringomyelia. Lesions were located at L2–L3, T8–T9 and L4. Histopathology described a fibroneural stalk with a glio-ependymal lining, surrounded by glial nests and nerve fibres. The youngest and most severely affected was euthanased, while the other two underwent surgery. Both regained independent ambulation with persistent paraparesis; however, one required ongoing management of urinary incontinence. Relevance and novel information LDM is a primary neural tube defect that may result in neurological deficits, including bladder dysfunction, and is characterised by a fibroneural stalk between the dermis and the spinal cord. Distinct MRI features, such as a visible intrathecal tract, dorsally tethered cord and syringomyelia, help distinguish this condition from the clinically similar dermoid sinus. The presence of progressive neurological signs, with a palpable midline defect overlying the affected spinal cord segment, may raise suspicion for this clinical entity in veterinary patients.

scholarly journals Limited Dorsal Myeloschisis with and without Type I Split Cord Malformation: Report of 3 Cases and Surgical Nuances

Medicina ◽  
2019 ◽  
Vol 55 (2) ◽  
pp. 28
Author(s):  
Yusuf Izci ◽  
Cahit Kural
Keyword(s):  
Spinal Cord ◽  
Tethered Cord ◽  
Intraoperative Neuromonitoring ◽  
The Other ◽  
Split Cord Malformation ◽  
Type I ◽  
Cystic Lesions ◽  
Rare Form ◽  
Urological Complications ◽  
Spinal Malformations

Limited dorsal myeloschisis (LDM) is a rare form of spina bifida which is characterized by a fibroneural stalk between the inner part of the skin and the spinal cord. It may be associated with split cord malformation (SCM). Diagnosis and management of this complex malformation is challenging. We presented 3 different cases of LDM. Two of them were associated with Type I SCM and the other had no associated malformation. All of them were evaluated radiologically just after the birth and underwent surgical treatment under intraoperative neuromonitoring. They discharged without any complication. Newborns with spinal cystic lesions should be carefully evaluated for spinal malformations after the birth and treated surgically as soon as possible in order to prevent neurological and urological complications secondary to tethered cord syndrome. Surgical technique in LDM-SCM patients is quite different than the patients with solitary LDM.

Primary polymorphous hemangioendothelioma of the spinal cord

2001 ◽  
Vol 95 (1) ◽  
pp. 93-95 ◽  
Author(s):  
Federico Roncaroli ◽  
Bernd W. Scheithauer ◽  
Savvas Papazoglou
Keyword(s):  
Spinal Cord ◽  
Vascular Tumor ◽  
Nerve Roots ◽  
Content Type ◽  
Sensorimotor Deficit ◽  
History Of ◽  
Intradural Extramedullary ◽  
Borderline Malignancy ◽  
Current Experience

✓ A case of polymorphous hemangioendothelioma of the spinal cord is described. This 55-year-old woman presented with an 18-month history of lower-extremity sensorimotor deficit. A magnetic resonance image revealed an enhancing, intradural, extramedullary nodule at the T1–2 level. On gross inspection, the lesion measured 3.5 cm and was firmly attached to spinal cord parenchyma and adjacent nerve roots. It was completely removed. Fourteen months after surgery the patient's neurological deficit had resolved. Polymorphous hemangioendothelioma is a rare vascular tumor of borderline malignancy. Most occur in lymph nodes. None has been reported to occur in the central and peripheral nervous system. Based on current experience, resection and close follow up seems the best therapeutic approach.

Tethered cord syndrome in adults

2011 ◽  
Vol 15 (3) ◽  
pp. 258-270 ◽  
Author(s):  
Jörg Klekamp
Keyword(s):  
Spinal Cord ◽  
Tethered Cord ◽  
Tethered Cord Syndrome ◽  
Neurological Deficits ◽  
Short Term ◽  
Group A ◽  
The Mean ◽  
Group B

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 23–74 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.

The outcome of tethered cord release in secondary and multiple repeat tethered cord syndrome

2009 ◽  
Vol 4 (1) ◽  
pp. 28-36 ◽  
Author(s):  
Wajd N. Al-Holou ◽  
Karin M. Muraszko ◽  
Hugh J. Garton ◽  
Steven R. Buchman ◽  
Cormac O. Maher
Keyword(s):  
Spinal Cord ◽  
Primary Repair ◽  
Tethered Cord ◽  
Tethered Cord Syndrome ◽  
Neurological Outcomes ◽  
Younger Age ◽  
Long Term Follow Up ◽  
Multiple Repeat

Object After primary repair of a myelomeningocele or a lipomyelomeningocele, patients can present with symptoms of secondary tethered cord syndrome (TCS). After surgical untethering, a small percentage of these patients can present with multiple repeat TCS. In patients presenting with secondary or multiple repeat TCS, the role as well the expected outcomes of surgical untethering are not well defined. Methods Eighty-four patients who underwent spinal cord untethering after at least 1 primary repair were retrospectively evaluated using scaled and subjective outcome measures at short-term and long-term follow-up visits. Outcomes were analyzed for predictive measures using multivariate logistic regression. Results Surgical untethering was performed in 66 patients with myelomeningoceles and 18 patients with lipomyelomeningoceles. Fourteen patients underwent multiple repeat spinal cord untethering. Patients were followed up for an average of 6.2 years. Most patients had stability of function postoperatively. Motor function and weakness improved in 7 and 16% of patients at 6 months, respectively, and 6 and 19% of patients at long-term follow-up evaluation, respectively. Of the patients who presented with back pain, 75% had improvement in symptoms at 6 months postoperatively. Younger age at untethering was significantly associated with worse long-term neurological outcomes. The number of previous untethering procedures, original diagnosis, sex, anatomical level, and degree of untethering had no effect on surgical outcomes. Conclusions Patients presenting with secondary or multiple repeat TCS may benefit from surgical untethering.

scholarly journals Spinal column shortening for tethered cord syndrome associated with myelomeningocele, lumbosacral lipoma, and lipomyelomeningocele in children and young adults

2017 ◽  
Vol 19 (6) ◽  
pp. 703-710 ◽  
Author(s):  
Guillermo Aldave ◽  
Daniel Hansen ◽  
Steven W. Hwang ◽  
Amee Moreno ◽  
Valentina Briceño ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Young Adults ◽  
Spinal Column ◽  
Tethered Cord ◽  
Tethered Cord Syndrome ◽  
Neurological Deficits ◽  
Csf Leak ◽  
Cord Injury ◽  
Children And Young Adults

OBJECTIVETethered cord syndrome is the clinical manifestation of an abnormal stretch on the spinal cord, presumably causing mechanical injury, a compromised blood supply, and altered spinal cord metabolism. Tethered cord release is the standard treatment for tethered cord syndrome. However, direct untethering of the spinal cord carries potential risks, such as new neurological deficits from spinal cord injury, a CSF leak from opening the dura, and retethering of the spinal cord from normal scar formation after surgery. To avoid these risks, the authors applied spinal column shortening to children and transitional adults with primary and secondary tethered cord syndrome and report treatment outcomes. The authors' aim with this study was to determine the safety and efficacy of spinal column shortening for tethered cord syndrome by analyzing their experience with this surgical technique.METHODSThe authors retrospectively reviewed the demographic and procedural data of children and young adults who had undergone spinal column shortening for primary or secondary tethered cord syndrome.RESULTSSeven patients with tethered cord syndrome caused by myelomeningocele, lipomyelomeningocele, and transitional spinal lipoma were treated with spinal column shortening. One patient with less than 24 months of follow-up was excluded from further analysis. There were 3 males and 4 females; the average age at the time was surgery was 16 years (range 8–30 years). Clinical presentations for our patients included pain (in 5 patients), weakness (in 4 patients), and bowel/bladder dysfunction (in 4 patients). Spinal column osteotomy was most commonly performed at the L-1 level, with fusion between T-12 and L-2 using a pedicle screw-rod construct. Pedicle subtraction osteotomy was performed in 6 patients, and vertebral column resection was performed in 1 patient. The average follow-up period was 31 months (range 26–37 months). Computed tomography–based radiographic outcomes showed solid fusion and no instrumentation failure in all cases by the most recent follow-up. Five of 7 patients (71%) reported improvement in preoperative symptoms during the follow-up period. The mean differences in initial and most recent Scoliosis Research Society Outcomes Questionnaire and Oswestry Disability Index scores were 0.26 and –13%, respectively; minimum clinically important difference in SRS-22 and ODI were assumed to be 0.4% and –12.8%, respectively.CONCLUSIONSSpinal column shortening seems to represent a safe and efficacious alternative to traditional untethering of the spinal cord for tethered cord syndrome.

scholarly journals Type I split cord malformation and tethered cord syndrome in an adult patient: A case report and literature review

2019 ◽  
Vol 10 ◽  
pp. 90
Author(s):  
Erin N. D’Agostino ◽  
Daniel R. Calnan ◽  
Vyacheslav I. Makler ◽  
Imad Khan ◽  
John H. Kanter ◽  
...  
Keyword(s):  
Conservative Therapy ◽  
Tethered Cord ◽  
Tethered Cord Syndrome ◽  
Leg Pain ◽  
Split Cord Malformation ◽  
Type I ◽  
Review Of Literature ◽  
History Of ◽  
Magnetic Resonance Imaging Mri

Background: In a split cord malformation (SCM), the spinal cord is divided longitudinally into two distinct hemicords that later rejoin. This can result in a tethered cord syndrome (TCS). Rarely, TCS secondary to SCM presents in adulthood. Here, we present an adult female with Type I SCM resulting in TCS and a review of literature. Case Description: A 57-year-old female with a history of spina bifida occulta presented with a 2-year history of worsening back and left leg pain, difficulty with ambulation, and intermittent urinary incontinence; she had not responded to conservative therapy. Magnetic resonance imaging (MRI) revealed a tethered cord secondary to lumbar type I SCM. The patient underwent an L1–S1 laminectomy for resection of the bony septum with cord detethering. At 2-month follow-up, the patient had improvement in her motor symptoms and less pain. In literature, 25 cases of adult-onset surgically managed SCM with TCS were identified (between 1936 and 2018). Patients averaged 37 years of age at the time of diagnosis, and 56% were female. Conclusion: TCS can present secondary to SCM in adulthood and is characterized predominantly by back and leg pain.

scholarly journals Neural Tube Defects: A case report

2018 ◽  
Vol 14 (4) ◽  
pp. 228-230
Author(s):  
Bishnu Gautam
Keyword(s):  
Folic Acid ◽  
Case Report ◽  
Neural Tube ◽  
Neural Tube Defects ◽  
Neural Tube Defect ◽  
Prenatal Counseling ◽  
Folic Acid Supplement ◽  
Folic Acid Supplements ◽  
Period Of Gestation

Neural tube defect is rare congenital defect of fetus. I have a case of 13 weeks period of gestation with meningomeyelocele came for termination of pregnancy. The diagnosis was confirmed by ultrasonography. History suggests that patient had previous neural tube defect baby, non-compliance to folic acid supplements. It is concluded that prenatal counseling, adherence to folic acid supplement and regular follow-up can prevent further complication.

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