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 ◽  
...  

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.

2013 ◽  
Vol 11 (3) ◽  
pp. 307-312 ◽  
Author(s):  
Charles G. Kulwin ◽  
Neal B. Patel ◽  
Laurie L. Ackerman ◽  
Jodi L. Smith ◽  
Joel C. Boaz ◽  
...  

Object The surgical management of patients with symptoms of tethered cord syndrome (TCS) who lack significant radiographic abnormalities is controversial. One potential MRI marker for TCS is a spinal cord syrinx or syringomyelia. Alternatively, a syrinx may be a benign and incidental finding. In this report the authors evaluated a highly selected cohort of patients with symptoms of TCS with minimal radiographic abnormalities other than syringomyelia. They analyzed clinical and radiographic outcomes after tethered cord release (TCR). Methods A retrospective review of data from 16 children who met the study inclusion criteria was performed. All patients had been surgically treated at Riley Hospital for Children in Indianapolis, Indiana, between 2006 and 2011. All children had clinical symptoms of TCS as well as available pre- and postoperative MRI data. Results The most common presentation (12 [75%] of 16 patients) was urinary dysfunction, defined as symptoms of urgency or incontinence with abnormal urodynamic studies. Clinical follow-up data were available in 11 of these 12 patients. All 11 had improvement in symptoms at an average follow-up of 17 months. Seven (87.5%) of 8 patients presenting with back or leg pain had improvement. Three patients had progressive scoliosis; 2 had stabilization of the curve or mild improvement, and 1 patient had worsening deformity. Radiographic follow-up data were obtained an average of 14.5 months after surgery. Twelve patients (75%) had stable syringomyelia after TCR. Four patients showed improvement, with 2 having complete radiographic resolution. Conclusions Highly selected patients with symptoms of TCS did very well clinically. Patients with abnormal urodynamic studies, pain, and gait disturbances showed a high rate of symptomatic improvement. However, a smaller percentage of patients had radiographic improvement of the syrinx. Therefore, the authors suggest that the decision to perform TCR should be based on clinical symptoms in this population. Symptomatic improvement was not necessarily related to radiographic resolution of the syrinx.


1998 ◽  
Vol 88 (6) ◽  
pp. 958-961 ◽  
Author(s):  
Bermans J. Iskandar ◽  
Benjamin B. Fulmer ◽  
Mark N. Hadley ◽  
W. Jerry Oakes

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.


Author(s):  
Zhao J ◽  

Ischemic Stroke (IS) is one of the leading diseases of mortality and disability worldwide. The rational administration of anticoagulant or antiplatelet drugs is of great importance to prevent stroke recurrence. Here we report an IS patient with a medical history of Catheter Ablation (CA) of Atrial Fibrillation (AF), who had a single vessel scattered lesions in the head Magnetic Resonance Imaging (MRI) scan, which indicated a remarkable correlation with morphology of atherosclerotic artery and artery embolism, while no AF recurrence was detected. At last, we focused on the possible criminal artery and found vulnerable plaques from apparent positive remodeling demonstrated by High-Resolution Vessel Wall MRI (HRVW-MRI). We provided a secondary prevention with the statin and antiplatelet therapy to reduce the risk of bleeding caused by anticoagulation and asked him to follow up Holter every three months. Along with this case report, we describe the imageology, review of literature and treatment outcomes in regard to CA of AF and discuss the prevention of IS.


2001 ◽  
Vol 10 (1) ◽  
pp. 1-5 ◽  
Author(s):  
Bermans J. Iskandar ◽  
Benjamin B. Fulmer ◽  
Mark N. Hadley ◽  
W. Jerry Oakes

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.


Neurosurgery ◽  
2020 ◽  
Author(s):  
Nicholas Theodore ◽  
Ethan Cottrill ◽  
Samuel Kalb ◽  
Corinna Zygourakis ◽  
Bowen Jiang ◽  
...  

Abstract BACKGROUND Few have explored the safety and efficacy of posterior vertebral column subtraction osteotomy (PVCSO) to treat tethered cord syndrome (TCS). OBJECTIVE To evaluate surgical outcomes after PVCSO in adults with TCS caused by lipomyelomeningocele, who had undergone a previous detethering procedure(s) that ultimately failed. METHODS This is a multicenter, retrospective analysis of a prospectively collected cohort. Patients were prospectively enrolled and treated with PVCSO at 2 institutions between January 1, 2011 and December 31, 2018. Inclusion criteria were age ≥18 yr, TCS caused by lipomyelomeningocele, previous detethering surgery, and recurrent symptom progression of less than 2-yr duration. All patients undergoing surgery with a 1-yr minimum follow-up were evaluated. RESULTS A total of 20 patients (mean age: 36 yr; sex: 15F/5M) met inclusion criteria and were evaluated. At follow-up (mean: 23.3 ± 7.4 mo), symptomatic improvement/resolution was seen in 93% of patients with leg pain, 84% in back pain, 80% in sensory abnormalities, 80% in motor deficits, 55% in bowel incontinence, and 50% in urinary incontinence. Oswestry Disability Index improved from a preoperative mean of 57.7 to 36.6 at last follow-up (P < .01). Mean spinal column height reduction was 23.4 ± 2.7 mm. Four complications occurred: intraoperative durotomy (no reoperation), wound infection, instrumentation failure requiring revision, and new sensory abnormality. CONCLUSION This is the largest study to date assessing the safety and efficacy of PVCSO in adults with TCS caused by lipomyelomeningocele and prior failed detethering. We found PVCSO to be an excellent extradural approach that may afford definitive treatment in this particularly challenging population.


2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Humphrey Okechi ◽  
A. Leland Albright ◽  
Ancent Nzioka

We describe a seminal case report of a child with a tethered cord syndrome secondary to the unusual constellation of a split cord malformation, lumbar myelomeningocele, and coexisting neurenteric cyst. A 17-year-old adolescent girl with a several-month history of myelopathy and urinary incontinence was examined whose spinal MRI scan demonstrated a type II split cord malformation with a large bone spur and an intradural neurenteric cyst in addition to lumbar myelomeningocele. Untethering of the spinal cord was achieved via a lumbar laminectomy. Pathological examination confirmed the intradural cyst to be a neurenteric cyst. Postoperatively there was stabilization of the neurological symptoms. Prophylactic surgery with total resection of the neurenteric cyst when feasible and spinal cord un-tethering appears to be associated with excellent outcomes.


2021 ◽  
Vol 55 (5) ◽  
Author(s):  
Val Constantine S. Cua ◽  
Eileen Liesl A. Cubillan

A 37-year-old Filipino man presented with a 9-month history of sagging skin progressing cephalocaudally from the chin and neck to the axillae, side of the trunk, and pelvic area. This was followed by a 2-month history of increasing serum creatinine levels associated with periorbital and bipedal edema, generalized weakness, decreased appetite, vomiting, and headache. Subsequently, skin-colored, non-tender sac-like plaques appeared on the abdomen, inguinal, and intergluteal areas. Histopathology of the latter lesions showed increased spaces between collagen bundles in the dermis. Staining with Verhoeff-van Gieson revealed focal sparse elastic fibers in the papillary dermis compared to that of the reticular dermis consistent with anetoderma. Further work-up revealed normal ANA titer and low serum C3. Kidney biopsy showed IgG deposition in the tubular basement membrane and trace C3 deposition in the glomerular mesangium, giving a diagnosis of rapid progressive glomerulonephritis. On subsequent follow-up, the sac-like plaques became lax and presented as generalized wrinkling of the skin, raising the question whether cutis laxa and anetoderma are occurring in a spectrum instead as distinct entities. Based on the current review of literature, this is the first reported case of primary anetoderma co-occurring with cutis laxa in a patient with glomerulonephritis. Deposition of immunoglobulins along the elastic fibers could have activated the complement system, mediating the destruction of the elastic fibers, resulting to cutis laxa and anetoderma. This case also considers the possibility of anetoderma and type I acquired cutis laxa occurring either in a spectrum or as distinct diseases in a single patient. Further investigations may identify an ultrastructural pattern that can help differentiate the two entities.


Neurosurgery ◽  
2002 ◽  
Vol 50 (5) ◽  
pp. 989-995 ◽  
Author(s):  
Loi K. Phuong ◽  
Kimberly A. Schoeberl ◽  
Corey Raffel

Abstract OBJECTIVE: The purpose of this study was to describe the natural history of tethered cord in patients who have undergone meningomyelocele repair. METHODS: We performed a retrospective review of 45 patients with a history of neonatal meningomyelocele repair who subsequently developed symptoms of tethered cord. Symptoms of tethered cord in this cohort consisted of the development of bladder spasticity or orthopedic foot deformity. None of these patients were treated with cord untethering; instead, they were treated symptomatically. RESULTS: On follow-up, 40 (88.9%) of these patients subsequently required additional orthopedic or urological procedures because of further symptoms of tethered cord. The incidence of progression of tethered cord syndrome is 27.5, 40, and 60% at 1, 2, and 5 years, respectively. CONCLUSION: Although this study does not address the question whether cord untethering will prevent further symptom development, these results do provide a strong rationale for consideration of an untethering procedure in patients with repaired meningomyelocele at the time of the onset of symptoms of tethered cord.


2006 ◽  
Vol 4 (2) ◽  
pp. 123-131 ◽  
Author(s):  
Gabriel Y. F. Lee ◽  
Guillermo Paradiso ◽  
Charles H. Tator ◽  
Fred Gentili ◽  
Eric M. Massicotte ◽  
...  

Object The adult presentation of tethered cord syndrome (TCS) is well recognized but continues to pose significant diagnostic and management challenges. The authors performed a retrospective study of clinical outcomes after neurosurgical intervention in 60 adults with TCS. Methods All patients who underwent detethering surgery for caudal cord tethering at Toronto Western Hospital between August 1993 and 2004 were identified. Their clinical charts, operative records, and follow-up data were reviewed. Detethering procedures were performed in 62 patients (age range 17–72 years) for TCS of various origins. Long-term (mean 41.5 months) follow-up data were obtained in 60 patients. The tethering lesions were tight terminal filum in 29 patients, postrepair myelomeningocele in 15, lipomyelomeningocele/lipoma in nine, split cord malformation in four, and arachnoidal adhesions in three. Fifty-nine patients presented with progressive pain and/or neurological dysfunction. One patient underwent prophylactic sectioning of the terminal filum. Most patients (71%) had bladder dysfunction at presentation. Microsurgical release of the tethered cord was performed in each case while using multimodality intraoperative neurophysiological monitoring. The most common complication was cerebrospinal fluid leakage, which occurred in nine patients and was managed by reinforcement sutures in four patients, temporary external drainage in three, and the placement of a lumboperitoneal shunt in two. Infective complications included superficial wound infection in three patients, meningitis in one, and urinary tract infection in one. One patient who had undergone multiple previous intradural procedures experienced worsened foot weakness postoperatively. Another patient experienced temporary unilateral lower-limb numbness. At follow up, improvement was noted in the majority of patients presenting with back (78%) and leg (83%) pain. Improvement was more likely in patients with preoperative motor weakness than in those with sensory deficits. Overall, neurological status was improved or stabilized in 90% of patients. Subjective improvement in bladder function was noted in 50% of patients with bladder dysfunction at presentation. Conclusions Surgery in adult patients with TCS is safe and effective for improving pain and neurological status in the majority of patients; however, patients who have undergone previous intradural detethering procedures in general fare less well, and considerable judgment is required in their management.


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