Cervical Diastematomyelia Associated with an Intradural Epidermoid Cyst between the Hemicords and Multiple Vertebral Body Anomalies

Korinth, Marcus C.; Kapser, Alexandra; Nolte, Kay; Gilsbach, Joachim M.

doi:10.1159/000082303

Cited by 6 publications

(2 citation statements)

References 14 publications

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“…13,16,20 A review of the literature shows that pediatric presentation of thoracal diastematomyelia with a syrinx concurrent with an intradural intramedullary epidermoid cyst and scoliosis is unique. [22][23][24] In only 1 case, diastematomyelia, epidermoid cyst, and scoliosis have been reported together. Only 2 previous reports have noted syringomyelia with an accompanying intradural intramedullary epidermoid cyst.…”

Section: Discussionmentioning

confidence: 99%

Scoliosis With Concurrent Thoracic Diastematomyelia, Syrinx, and Intradural Intramedullary Epidermoid Cyst

Ketencı¹,

Bezer²,

Saygı

et al. 2012

Neurosurgery Quarterly

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The aim of this paper is to report the first presentation of type 2 thoracic diastematomyelia with concurrent syrinx, intradural intramedullary epidermoid cyst, and scoliosis, in which scoliosis correction was performed. The incidence of intraspinal anomalies in patients with congenital spinal deformity varies from 4.9% to 58% depending on the reports by the authors and diagnostic techniques. Most of these are tethered cord, diastematomyelia, and syringomyelia. Intramedullary epidermoid cysts of the spinal cord are very rare tumors, especially those associated with spinal dysraphism. Clinical manifestations of these anomalies may be absent at first; therefore, MRI is important at initial evaluation. Intervention for intraspinal anomaly and scoliosis correction can be performed separately or together as 1 procedure. A 12-year-old girl was brought to us because of progressive spinal deformity. The patient had a right thoracic curve at T5-T12 of 31 degrees. The curve was rigid and partial fusion at T6-T8 was noted. MRI of the spinal cord was taken to detect any neural axis abnormalities, and it revealed diastematomyelia at T5-T8 levels and adjacent syringomyelia superiorly. Neurological examination was normal. The patient underwent syrinx drainage and diastematomyelia resection together with scoliosis correction in 1 surgical procedure. A whitegray-colored tissue of 1 cm diameter and well-defined borders was detected in the syrinx cavity, and it was excised. There were no intraoperative or postoperative complications. The patient began ambulating on postoperative day 2. Postoperative Cobb angle was 5 degrees and the pathologic diagnosis of the lesion was an epidermoid cyst.

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Section: Discussionmentioning

confidence: 99%

Scoliosis With Concurrent Thoracic Diastematomyelia, Syrinx, and Intradural Intramedullary Epidermoid Cyst

Ketencı¹,

Bezer²,

Saygı

et al. 2012

Neurosurgery Quarterly

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show abstract

“…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][10][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.…”

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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Cervical split cord malformation (diastematomyelia) with associated Klippel–Feil deformity presenting in adulthood with bimanual synkinesis

Goacher,

Lee

2024

Eur Spine J

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Cervical Diastematomyelia Associated with an Intradural Epidermoid Cyst between the Hemicords and Multiple Vertebral Body Anomalies

Cited by 6 publications

References 14 publications

Scoliosis With Concurrent Thoracic Diastematomyelia, Syrinx, and Intradural Intramedullary Epidermoid Cyst

Scoliosis With Concurrent Thoracic Diastematomyelia, Syrinx, and Intradural Intramedullary Epidermoid Cyst

CT and MRI features of patients with diastematomyelia

Cervical split cord malformation (diastematomyelia) with associated Klippel–Feil deformity presenting in adulthood with bimanual synkinesis

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