Combined Type 1 and 2 split cord malformations, kyphoscoliosis, tethered cord, and a lipoma

Background: Split cord malformations (SCMs) are uncommon congenital anomalies. They can be divided into Type 1 (bony septum and two separate dural sheaths) and Type 2 (fibrous septum and a single dural sheath).[1,2,4] Rarely,SCM can be associated with orthopedic anomalies (mostly seen in older children), such as scoliosis, followed by kyphosis, and/or both.[1-3] Conversely, patients with congenital scoliosis have 40% risk of congenital underlying neural deformities, with 16.3% attributed to SCM.[1,4] Those with thoracic or lumbosacral SCM may have congenital tethering lesions, predominantly consisting of intradural lipomas.[3,4] Surgery is optimally performed in two stages: first, removal of the spinal septum and untethering of the cord, and second, correction of the kyphosis, scoliosis, and/or both.[1-5] Case Description: In this video, a 44-year-old female initially presented after having progressively developed kyphoscoliosis since childhood. Three months before presentation, she had developed increased left lower extremity pain with hypoesthesia followed 1 month later by the onset of the left lower extremity monoplegia (0/5). The computed tomography and magnetic resonance imaging studies revealed a SCM at the L3 level with bony and membranous septum, accompanied by an intradural lipoma, and tethered cord. She underwent a two- stage procedure; first, removal of the bony and membranous septum, resection of the intradural lipoma, and untethering of the cord; second, she had correction of the kyphoscoliosis. Postoperatively, although the pain and sensory deficits improved, the left lower extremity monoplegia remained. Conclusion: SCM is a rare cause of spinal deformity. If left untreated, the associated neurological deficits may progress. Treatment should include a two-staged approach; first, the bony and membranous septum should be removed followed by lipoma resection and untethering the cord with adequate cord decompression, while second, a fusion may be performed to address attendant kyphoscoliosis. Keywords: Intradural lipoma, Kyphoscoliosis, Split cord malformation, Tethered cord

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Successful treatment of a patient with congenital kyphoscoliosis associated with tethered cord

Journal of Neurosurgery Spine ◽

10.3171/2014.9.spine13528 ◽

2015 ◽

Vol 22 (1) ◽

pp. 64-69 ◽

Cited By ~ 5

Author(s):

Hui-Ren Tao ◽

Tian-Li Yang ◽

Michael S. Chang ◽

Huan Li ◽

Da-Wei Zhang ◽

...

Keyword(s):

Spinal Cord ◽

Tethered Cord ◽

Congenital Scoliosis ◽

Conus Medullaris ◽

Neurological Deficits ◽

Common Finding ◽

Vertebral Column Resection ◽

Apical Vertebra ◽

Shortening Osteotomy ◽

Posterior Spine

Tethered cord is a common finding in congenital scoliosis. The most frequently advocated approach for this condition is to perform prophylactic detethering of the cord before scoliosis corrective surgery. The authors report on a 14-year-old patient with congenital thoracic kyphoscoliosis associated with a tethered cord, who developed progressive paraparesis and was successfully treated by posterior spine shortening osteotomy alone without prophylactic untethering. The patient had a 103° scoliotic curve together with a 93° kyphotic curve with an apical vertebra of T-7. Furthermore, he developed a significant progression of neurological deficits, including weakness of both legs and urinary and bowel incontinence. Preoperative MRI revealed that the spinal cord was entrapped by the apical vertebra and the low-placed conus medullaris was at approximately L-5. A posterior vertebral column resection of T-7 was performed for the purpose of simultaneously correcting the kyphoscoliosis and releasing tension on the tethered cord without a true detethering surgery. The patient's spinal cord function recovered completely from Frankel D to Frankel E by 6 months after the procedure. Evaluation at 31 months after surgery showed maintenance of good curve correction and normal neurological function.

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Surgical management of tethered spinal cord in adults: report of 54 cases

Journal of Neurosurgery Spine ◽

10.3171/spi.2001.95.2.0173 ◽

2001 ◽

Vol 95 (2) ◽

pp. 173-178 ◽

Cited By ~ 17

Author(s):

Sabine Hüttmann ◽

Juergen Krauss ◽

Hartmut Collmann ◽

Niels Sörensen ◽

Klaus Roosen

Keyword(s):

Spinal Cord ◽

Tethered Cord ◽

Tethered Cord Syndrome ◽

Neurological Deficits ◽

Split Cord Malformation ◽

Content Type ◽

Adult Age ◽

Pregnancy And Childbirth ◽

Pain Status ◽

Fine Print

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.

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The Treatment of Severe Congenital Scoliosis Associated With Type I Split Cord Malformation: Is a Preliminary Bony Septum Resection Always Necessary?

Neurosurgery ◽

10.1093/neuros/nyy237 ◽

2018 ◽

Vol 85 (2) ◽

pp. 211-222 ◽

Cited By ~ 2

Author(s):

Zifang Huang ◽

Xueshi Li ◽

Yaolong Deng ◽

Wenyuan Sui ◽

Hengwei Fan ◽

...

Keyword(s):

Spinal Cord ◽

Congenital Scoliosis ◽

Single Stage ◽

Neurological Deficits ◽

Split Cord Malformation ◽

Control Group ◽

Type I ◽

Shortening Osteotomy ◽

Group Control ◽

Group Control Group

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.

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A rare case of thoracic myelocystocele associated with type 1 split cord malformation with low lying tethered cord, dorsal syrinx and sacral agenesis: Pentad finding

Journal of Neurosciences in Rural Practice ◽

10.4103/0976-3147.143209 ◽

2015 ◽

Vol 6 (01) ◽

pp. 087-090 ◽

Cited By ~ 1

Author(s):

Dipanker Singh Mankotia ◽

Guru Dutta Satyarthee ◽

Bhawani Shankar Sharma

Keyword(s):

Spina Bifida ◽

Surgical Management ◽

Rare Case ◽

Clinical Presentation ◽

Spinal Dysraphism ◽

Tethered Cord ◽

Split Cord Malformation ◽

Rare Form ◽

Sacral Agenesis

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.

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Spinal Intramedullary Metastasis of Breast Cancer

Case Reports in Medicine ◽

10.1155/2014/583282 ◽

2014 ◽

Vol 2014 ◽

pp. 1-3 ◽

Cited By ~ 1

Author(s):

Recep Basaran ◽

Mehmet Tiryaki ◽

Dilek Yavuzer ◽

Mustafa Efendioglu ◽

Ece Balkuv ◽

...

Keyword(s):

Breast Cancer ◽

Lower Extremity ◽

Cancer Metastasis ◽

Breast Cancer Metastasis ◽

Neurological Deficits ◽

Histological Evaluation ◽

High Morbidity ◽

Intramedullary Metastasis ◽

Left Lower Extremity ◽

Thoracic Spinal

Objective.Breast cancer accounts for approximately one-third of all cancers in females. Approximately 8.5 % of all central nervous system metastases are located in the spinal cord. These patients have rapidly progressing neurological deficits and require immediate examination. The aim of surgery is decompression of neural tissue and histological evaluation of the tumor. In this paper, we present a case of breast cancer metastasis in thoracic spinal intramedullary area which had been partially excised and then given adjuvant radiotherapy.Case.A 43-year-old female patient with breast cancer for 8 years was admitted to our hospital with complaints of weakness in both legs. Eight years ago, she received chemotherapy and radiotherapy. On her neurological examination, she had paraparesis (left lower extremity: 2/5, right lower extremity: 3/5) and urinary incontinence. Spinal MRI revealed a gadolinium enhancing intramedullary lesion. Pathologic examination of the lesion was consistent with breast carcinoma metastasis. The patient has been taken into radiotherapy.Conclusion.Spinal intramedullary metastasis of breast cancer is an extremely rare situation, but it has a high morbidity and mortality rate. Microsurgical resection is necessary for preservation or amelioration of neurological state and also for increased life expectancy and quality.

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Herniation of the cauda equina into the facet joint through a pseudomeningocele: A case report and literature review

Surgical Neurology International ◽

10.25259/sni_893_2020 ◽

2021 ◽

Vol 12 ◽

pp. 30

Author(s):

Jumpei Iida ◽

Naohisa Miyakoshi ◽

Michio Hongo ◽

Hiroshi Sasaki ◽

Hiroki Ito ◽

...

Keyword(s):

Lower Extremity ◽

Facet Joint ◽

Cauda Equina ◽

Neurological Deficits ◽

Cerebrospinal Fluid Leakage ◽

Facet Joints ◽

Secondary Complications ◽

Left Lower Extremity ◽

One Year ◽

Incidental Durotomy

Background: Incidental durotomy is a well-known complication of spinal surgery. It can lead to persistent cerebrospinal fluid leakage resulting in significant secondary complications. Here, we present a case in which the cauda equina herniated into a pseudomeningocele that penetrated a facet joint, leading to lower extremity radiculopathy warranting surgical correction. Case Description: One year ago, a 67-year-old male underwent a partial left L4-L5 laminectomy. At surgery, a durotomy was repaired with a nylon suture and reinforced with a fat patch. He subsequently presented with severe left lower extremity radiculopathy and a partial cauda equina syndrome. On MR, the cauda equina had herniated into a pseudomeningocele that penetrated the left facet joint. Once the defect was repaired at surgery, the patient’s symptoms improved. Conclusion: It is critical to correctly repair an intraoperative durotomy to avoid further neurological deficits that may include cauda equina herniation into pseudomeningoceles penetrating facet joints.

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Outcome of surgical untethering of tethered cord syndrome in adults due to spina bifida occulta

AL-Kindy College Medical Journal ◽

10.47723/kcmj.v15i1.82 ◽

2019 ◽

Vol 15 (1) ◽

pp. 68-74

Author(s):

Bassam Mahmood Flamerz Arkawazi

Keyword(s):

Spina Bifida ◽

Lower Extremity ◽

Tethered Cord ◽

Adult Patients ◽

Spina Bifida Occulta ◽

Neurological Deficits ◽

Neurological Injury ◽

Early Surgery ◽

Lower Extremity Weakness ◽

Pain Status

Background:. Children with spina bifida occulta require early surgery to prevent neurological deficits. The treatment of patients with a congenitally tethered cord who present in adulthood remains controversial. Objective: The aim of this study is to describe the outcome obtained in 61 adult patients with congenital TCS and no prior surgical treatment who underwent surgical untethering. Methods: This prospective study was conducted on 61 adult patients who underwent surgical untethering for spina bifida occulta at four neurosurgical centers in Baghdad / Iraq between March 2000 and January 2018. Patients who had undergone prior myelomeningocele repair or tethered cord release surgery were excluded. The most common intraoperative findings were lipomyelomeningocele (41%) and a tight terminal filum (36%). The follow-up duration ranged from 10.8 to 149.5 months (mean 20.9 months). Of the 34 patients with back pain, status improved in 65%, worsened in 3%, remained unchanged in 18%, and improved and later recurred in 15%. Lower-extremity pain improved in 16 patients (53%), remained unchanged in 23%, improved and then recurred in 17%, and worsened in 7%. Lower-extremity weakness improved in 47%, remained unchanged in 47%, and improved and then recurred in 5%. Finally, of the 17 patients with lower-extremity sensory changes, status improved in 35%, remained unchanged in 35%, and the information on five patients was unavailable. Surgical complications included three wound infections, one cerebrospinal fluid leak, and two pseudomeningoceles requiring surgical revision. One patient developed acute respiratory distress syndrome and sepsis postoperatively and died several days later. Conclusions: Adult-age presentation of a congenital tethered cord is unusual. Despite a slight increase in postoperative neurological injury in adults, surgery has relatively low risk and offers good potential for neurological improvement or stabilization. As in children, we recommend early surgery in adults with this disorder. The decision to undertake surgery, however, should be modulated by other factors such as a patient’s general medical condition and risk posed by anesthesia.

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Tethered cord due to spina bifida occulta presenting in adulthood: a tricenter review of 61 patients

Journal of Neurosurgery Spine ◽

10.3171/spi.2007.6.3.210 ◽

2007 ◽

Vol 6 (3) ◽

pp. 210-215 ◽

Cited By ~ 32

Author(s):

Sharad Rajpal ◽

R. Shane Tubbs ◽

Timothy George ◽

W. Jerry Oakes ◽

Herbert E. Fuchs ◽

...

Keyword(s):

Spina Bifida ◽

Lower Extremity ◽

Medical Condition ◽

Tethered Cord ◽

Spina Bifida Occulta ◽

Neurological Deficits ◽

Neurological Injury ◽

Early Surgery ◽

Lower Extremity Weakness ◽

Pain Status

Object Children with spina bifida occulta require early surgery to prevent neurological deficits. The treatment of patients with a congenitally tethered cord who present in adulthood remains controversial. Methods The authors studied the medical records of 61 adult patients who underwent surgical untethering for spina bifida occulta at three institutions between 1994 and 2003. Patients who had undergone prior myelomeningocele repair or tethered cord release surgery were excluded. The most common intraoperative findings were lipomyelomeningocele (41%) and a tight terminal filum (36%). The follow-up duration ranged from 10.8 to 149.5 months. Of the 34 patients with back pain, status improved in 65%, worsened in 3%, remained unchanged in 18%, and improved and later recurred in 15%. Lower-extremity pain improved in 16 patients (53%), remained unchanged in 23%, improved and then recurred in 17%, and worsened in 7%. Lower-extremity weakness improved in 47%, remained unchanged in 47%, and improved and then recurred in 5%. Finally, of the 17 patients with lower-extremity sensory changes, status improved in 35%, remained unchanged in 35%, and the information on five patients was unavailable. Surgical complications included three wound infections, one cerebrospinal fluid leak, and two pseudomeningoceles requiring surgical revision. One patient developed acute respiratory distress syndrome and sepsis postoperatively and died several days later. Conclusions Adult-age presentation of a congenital tethered cord is unusual. Despite a slight increase in postoperative neurological injury in adults, surgery has relatively low risk and offers good potential for neurological improvement or stabilization. As they do in children, the authors recommend early surgery in adults with this disorder. The decision to undertake surgery, however, should be modulated by other factors such as a patient's general medical condition and risk posed by anesthesia.

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