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

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.

2018 ◽  
Vol 46 (05) ◽  
pp. 323-329 ◽  
Author(s):  
Nele Ondreka ◽  
Sara Malberg ◽  
Emma Laws ◽  
Martin Schmidt ◽  
Sabine Schulze

SummaryA 2-year-old male neutered mixed breed dog with a body weight of 30 kg was presented for evaluation of a soft subcutaneous mass on the dorsal midline at the level of the caudal thoracic spine. A further clinical sign was intermittent pain on palpation of the area of the subcutaneous mass. The owner also described a prolonged phase of urination with repeated interruption and re-initiation of voiding. The findings of the neurological examination were consistent with a lesion localization between the 3rd thoracic and 3rd lumbar spinal cord segments. Magnetic resonance imaging revealed a spina bifida with a lipomeningocele and diplomyelia (split cord malformation type I) at the level of thoracic vertebra 11 and 12 and secondary syringomyelia above the aforementioned defects in the caudal thoracic spinal cord. Surgical resection of the lipomeningocele via a hemilaminectomy was performed. After initial deterioration of the neurological status postsurgery with paraplegia and absent deep pain sensation the dog improved within 2 weeks to non-ambulatory paraparesis with voluntary urination. Six weeks postoperatively the dog was ambulatory, according to the owner. Two years after surgery the owner recorded that the dog showed a normal gait, a normal urination and no pain. Histopathological diagnosis of the biopsied material revealed a lipomeningocele which confirmed the radiological diagnosis.


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.


2016 ◽  
Vol 5 (1) ◽  
pp. 42-45
Author(s):  
Md Abu Taher ◽  
Jafreen Sultana ◽  
Md Mofazzal Sharif ◽  
Md Mohit Ul Alam ◽  
Md Towhidur Rahman ◽  
...  

A 8 months old male child reported to paediatric OPD of Shaheed Suhrawardy Medical College Hospital with the complaints of small swelling in the back and tilting of body on one side during sitting and standing posture. His antenatal period was unremarkable, post natal period revealed delayed crying and immunization was not completed. Local examination showed a small hairy patch in lower dorsal region. General and systemic examination revealed no significant abnormality. Consulting physician advised to do X- ray of dorso- lumbar spine (both views), Ultrasonography (USG) of the swelling and FNAC from the swelling. X- ray report concluded as spinal dysraphism with widening of spinal canal. Ultrasonography showed dural ectasia. No malignant or granulomatous cells were found in FNAC. For confirmation of plain x- ray and USG findings, the patient’s parent was advised to do MRI of dorsolumbar spine in Radiology and Imaging Department of BIRDEM hospital. MRI findings were compatible with Diastematomyelia with tethered cord as evidenced by two unequal hemicord separated by signal void cleft and low insertion of spinal cord at L4-L5 level. As MRI is less sensitive regarding bone , CT scan was done and CT revealed grossly abnormal vertebral bodies with an osseous spur traversing the entire canal dividing the canal into two halves. Spinal cord was also divided into two unequal cord. Considering X-ray, USG, CT and MRI, final Diagnosis was made as Type I Diastematomyelia with Tethered cord.Birdem Med J 2015; 5(1): 42-45


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.


2001 ◽  
Vol 95 (2) ◽  
pp. 173-178 ◽  
Author(s):  
Sabine Hüttmann ◽  
Juergen Krauss ◽  
Hartmut Collmann ◽  
Niels Sörensen ◽  
Klaus Roosen

Object. The clinical features specific to tethered cord syndrome (TCS) in adults as well as factors determining outcome and prognosis have rarely been addressed systematically. The authors studied 56 patients, 54 of whom were treated surgically over the last 16 years. Methods. In 17 patients who had been asymptomatic during childhood, TCS was diagnosed 8 years after onset of symptoms. Tethered cord syndrome was diagnosed 4 years after worsening in 39 patients with neurological signs or symptoms since childhood. The patients were followed for an average of 8 years. Features specific to adult-age presentation included nondermatomal pain aggravated by movement in 34 patients and conditions such as pregnancy and childbirth (in five of 11 pregnant patients). The most frequent tethering lesions were lipoma in 32, tight terminal filum in 28, and split cord malformation and secondary adhesions in 12 patients, respectively. Improvement or stabilization of symptoms at 6 months after surgery was noted in 46 (85%) of 54 patients. Improvement in pain status was most frequent (86%) followed by improvements in spasticity (71%), bladder dysfunction (44%), and sensorimotor deficits (35%). Factors associated with adverse outcome included preoperative duration of neurological deficits more than 5 years and incomplete untethering. On average, 8 (80%) of 10 patients with incomplete untethering developed recurrent symptoms 5 years after surgery compared with only seven (16%) of 44 patients in whom complete untethering was achieved. Seven patients underwent reoperation and in five of them stabilization of symptoms was attained. At a mean follow up of 8 years, 46 (85%) of the 54 surgically treated patients were in stable neurological condition, including those in whom reoperation was performed. Conclusions. Surgery for TCS is as beneficial in adults as it is in children. Its success depends on early diagnosis and complete untethering of the spinal cord.


Neurosurgery ◽  
2018 ◽  
Vol 85 (2) ◽  
pp. 211-222 ◽  
Author(s):  
Zifang Huang ◽  
Xueshi Li ◽  
Yaolong Deng ◽  
Wenyuan Sui ◽  
Hengwei Fan ◽  
...  

Abstract BACKGROUND Single-stage spine-shortening osteotomy without treating spinal cord malformations may have potential advantages for the treatment of severe congenital scoliosis (CS) with type I split spinal cord malformation (SSCM); however, the study of this technique was limited. OBJECTIVE To evaluate the safety and efficacy of a single-stage spine-shortening osteotomy in the treatment of severe CS associated with type I SSCM. METHODS A retrospective study was designed to compare 2 case series including 12 severe CS patients with type I SSCM and 26 patients with type A cord function (without spinal cord malformations, evoked potential abnormalities, and neurological dysfunctions preoperatively) treated with a single-stage spine-shortening posterior vertebral column resection (PVCR). Patient demographic, clinical, operative, and radiographic data were obtained and compared between groups. RESULTS The surgical procedure was successfully performed in both groups, and the patients were observed for an average of 44.9 mo (range 25-78 mo) after the initial surgery. The radiographic parameters, intraoperative data, and new neurological deficits showed no difference, while deformity angular ratio (SSCM group: control group = 16.6 ± 3.6: 20.1 ± 3.9, P = .01) and corrective rate (SSCM group: control group = 50%: 58%, P = .046) of the main curve were statistically different between groups. All of the new neurological deficits were recovered within 1 yr. CONCLUSION The single-stage spine-shortening PVCR with moderate correction could be applied to the treatment of CS associated with type I SSCM. This strategy can achieve safe spinal deformity correction while obviate the neurological complications brought by the detethering procedures, which merits further clinical investigation.


2015 ◽  
Vol 6 (01) ◽  
pp. 087-090 ◽  
Author(s):  
Dipanker Singh Mankotia ◽  
Guru Dutta Satyarthee ◽  
Bhawani Shankar Sharma

ABSTRACTMyelocystocele is a rare form of spinal dysraphism. Thoracic myelocystocele is still rarer. The occurrence of thoracic myelocystocele associated with type-1 split cord malformation, low lying tethered cord, dorsal syrinx and spina bifida is extremely rare. Clinical presentation of such a rare case and an early surgical management is discussed briefly.


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.


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.


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