Diastematomyelia – A 40-Year Experience

Gower, David J.; Curling, O. Del; Kelly, David L.; Alexander, Eben

doi:10.1159/000120369

Cited by 55 publications

(33 citation statements)

References 22 publications

(27 reference statements)

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“…Sensory improvement was noted in 4/12 of patients with preoperative sensory disturbances. The chances of autonomic improvement are still lower, reportedly in the range of 15–40% [10,11,12,13,14]. In the present series, autonomic disturbances improved in 2/12 patients.…”

Section: Discussionmentioning

confidence: 42%

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Proposal for a New Clinicoradiological Classification of Type I Split-Cord Malformations: A Prospective Study of 25 Cases

Gupta¹,

Mahapatra²

2006

Pediatr Neurosurg

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Background and Methods: Split-cord malformations (SCMs) are rare malformations of the spinal cord. A new classification is proposed based on intraoperative findings in 25 cases of type I SCMs (with bony spur). Patients’ demographic profile, radiological and surgical details, complications and outcome were noted. Findings: All patients had type I SCMs. The mean age of the patients was 6.2 years (female:male ratio 1.2:1). Asymmetric lower-limb and sphincter weakness were present in 17/25 (68%) and 5/25 (20%) cases, respectively. Of the symptomatic cases, 29.4% (5/17) showed improvement in motor power and sensory improvement was recorded in 33.3% (4/12) of patients while 40% (2/5) regained continence. No patient in the asymptomatic group (0/8) showed postoperative neurological deterioration. A new subclassification of type I SCM is proposed based on the location of the bony spur responsible for the split, which can affect surgical dissection and outcome. Conclusions: In SCM patients, the risk of developing neurological deficits increases with age, hence all SCM patients should undergo prophylactic surgery, even if they are asymptomatic. Our new classification is easy to use and takes into account intraoperative findings that may affect surgical outcome.

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Section: Discussionmentioning

confidence: 42%

“…Deficits also depend on the associated anomalies like syringomyelia, lipomeningomyelocele and congenital tumors such as dermoids and epidermoids. Overall, the outcome also depends on anomalies of other systems [2, 4, 10, 11]. …”

Section: Discussionmentioning

confidence: 99%

Proposal for a New Clinicoradiological Classification of Type I Split-Cord Malformations: A Prospective Study of 25 Cases

Gupta¹,

Mahapatra²

2006

Pediatr Neurosurg

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“…The frequency of composite-type SCM was less than 1% in the series reported by Harwood-Nash and McHugh [16]. Gower et al [17] had only 1 patient with composite-type SCM with a bone spur at the midcleft and another at the caudal end of the cleft. Erşahin et al [18] also reported on 3 unusual cases of SCM, 2 of which were composite type.…”

Section: Discussionmentioning

confidence: 99%

Long-Segment Type 1 Split Cord Malformation with Two-Level Split Cord Malformation and a Single Dural Sac at the Lower Split

Singh

Khandelwal²,

Singh³

et al. 2011

Pediatr Neurosurg

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Objective and Importance: Two-level bony spurs are rare and also a very long segment of bony spur is very rare. Bony spur with a single dural covering is not reported in the literature. Here, we report a case of prophylactic surgery performed on a patient with a bony spur with a single dural covering who showed a good postoperative outcome. This case highlights the importance of prophylactic surgery in such patients to avoid cord injuries. Clinical Presentation: Since birth a 1-year-old child had had a tuft of hair at the dorsal (D₆ and D₇) region. The patient was examined and found to have scoliosis with convexity towards the right. There was no neurological deficit. MRI and CT spine scans showed 2-level type 1 split cord malformation (SCM) and there was a low-lying conus at the L_3–4 level. Intervention: The patient was operated on at the age of 1 year and 2 separate bony septae were observed. The upper one extended from D₅ to D₉ and the lower one was at the D₁₁ level. The bony spurs were excised. The filum was sectioned at the L_4–5 level by a separate incision. The patient had a good postoperative recovery. Conclusion: With meticulous planning and careful surgery, prophylactic surgery can have a very good outcome in long-segment and two-level type 1 SCM.

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“…The clinical manifestations include cutaneous abnormality overlying the spine, neurologic deficits and orthopedic abnormalities. [1][2][3][4] Clinically, the symptomatology is not specific and does not differ from that seen in other forms of spinal dysraphism. [5][6][7][8] Despite its low incidence, diastematomyelia results in severe neurological dysfunction.…”

Section: Introductionmentioning

confidence: 99%

CT and MRI features of patients with diastematomyelia

Huang

et al. 2014

Spinal Cord

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Objective: To evaluate computed tomography (CT) and magnetic resonance imaging (MRI) features in patients with diastematomyelia and to investigate clinical characteristics of this lesion. Study design: A retrospectively study. Setting: The Second Affiliated Hospital, School of Medicine, Xi'an Jiaotong University. Methods: A total of 82 diastematomyelia cases were retrospectively studied. All the patients underwent neurological examinations as well as MRI and CT of the spine. A self-established neurological functional grading system was used, and posterior tibial nerve somatosensory cortical-evoked potential (PTNSCEP) was measured to assess the neurological status of the patients. Imaging features of symmetry of splitting, presence of septum, location of lesion and number of split segments were studied. The neurological functional grading, PTNSCEP, and imaging findings were then analyzed and compared, and the difference was considered to be significant if P-value was lower than 0.05. Results: Neurological functional grading and latency of PTNSCEP were significantly different but related in terms of symmetry of splitting, presence of septum and location of lesion. Although no significant differences were present in the number of split segments, the severity of the neurological functional grading and PTNSCEP impairment were not related to the number of split segments. INTRODUCTIONDiastematomyelia is an uncommon congenital malformation of the vertebral axis where the spinal cord is split longitudinally into two. Each hemicord contains a central canal and a set of dorsal and ventral horns and nerve roots. The precise etiology is not known. The clinical manifestations include cutaneous abnormality overlying the spine, neurologic deficits and orthopedic abnormalities. [1][2][3][4] Clinically, the symptomatology is not specific and does not differ from that seen in other forms of spinal dysraphism. [5][6][7][8] Despite its low incidence, diastematomyelia results in severe neurological dysfunction. The clinical diagnosis and treatment of the lesion are therefore important. Delayed or improper treatment due to incorrect diagnosis may lead to the worsening of neurological symptoms. Conventionally, diagnosis of diastematomyelia is based on its clinical manifestations and radiological examination. Plain radiography and myelography have traditionally been used. With the improvement in imaging technology, computed tomography (CT) and magnetic resonance imaging (MRI) are established as the primary diagnostic strategies for evaluating the symptoms of diastematomyelia. Although CT and MRI findings of diastematomyelia have been discussed in the literature, 9-11 there are few reports concerning the characteristic imaging and the clinical manifestations. 12,13 The relationship between the clinical severity of diastematomyelia and the morphological changes reflect in imaging findings has never been thoroughly investigated.

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Diastematomyelia – A 40-Year Experience

Cited by 55 publications

References 22 publications

Proposal for a New Clinicoradiological Classification of Type I Split-Cord Malformations: A Prospective Study of 25 Cases

Proposal for a New Clinicoradiological Classification of Type I Split-Cord Malformations: A Prospective Study of 25 Cases

Long-Segment Type 1 Split Cord Malformation with Two-Level Split Cord Malformation and a Single Dural Sac at the Lower Split

CT and MRI features of patients with diastematomyelia

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