SÉRIE DE CASOS DE 19 PACIENTES OPERADOS PARA ESCOLIOSE SEVERA E DIASTEMATOMIELIA

Mikhaylovskiy, Mikhail Vitalievich; Dubousset, Jean; Novikov, Vjacheslav Victorovtch; Vasyura, Alexander Sergeevich; Udalova, Inga Gennadievna; Sadovoi, Mikhail Anatolievich

doi:10.1590/S1808-185120191801201849

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Review Article, Deformities • Coluna/Columna 18 (1) • Jan-Mar 2019 • https://doi.org/10.1590/S1808-185120191801201849 copiar

SÉRIE DE CASOS DE 19 PACIENTES OPERADOS PARA ESCOLIOSE SEVERA E DIASTEMATOMIELIA

Autoria SCIMAGO INSTITUTIONS RANKINGS

RESUMO

Objetivo:

A diastematomielia é uma má formação rara da espinha dorsal e medula congênita, quando a medula espinhal é dividida em duas partes pelo septo ósseo ou fibroso. A incidência de diastematomielia em pacientes com formas mais graves de escoliose congênita é muito maior do que a incidência geral em uma população. Ao realizar cirurgias para corrigir deformidades escolióticas, surge a questão sobre a escolha de uma estratégia para o manuseio do septo. Uma resposta inequívoca à essa questão não existe, pois a doença é muito rara e heterogênea. O objetivo foi resumir os dados sobre diferentes estratégias cirúrgicas para detecção da diastematomielia.

Métodos:

Revisão de literatura e análise retrospectiva de nossos próprios dados clínicos.

Resultados:

Apresentamos nossa própria experiência no tratamento de 19 pacientes com diastematomielia e escoliose congênita grave. Transtorno de postura foi corrigido em todos os casos; em nenhum dos casos o septo foi removido. Correção significativa foi alcançada para todos os pacientes e nenhuma complicação neurológica foi observada a curto e longo prazo de acompanhamento.

Conclusões:

A não remoção cirúrgica do esporão permite obter uma compensação e ter a falta de complicações neurológicas, tanto no período pós-operatório imediato quanto a longo prazo (mais de 2 anos) de acompanhamento. Nível de Evidência IV; Série de casos^h.

Descritores:
Diastematomielia; Escoliose; Cirurgia

Case number	Age (years)	Type of deformity	Side of deformity	Vertebral anomalies	Spinal cord anomalies	Length of the diastematomyelic bony septum
1	12.9	Scoliosis	D	Complete fusion of the bodies and arches of the T8, T9, T10, and T11 vertebrae. Spina bifida posterior at the 8, T11, T12, L1, L2, and L3 level	No	Complete diastematomyelic bony septum in the spinal canal at the T9-T10-T11 level
2	11.3	Scoliosis	D	Butterfly deformity of the D11 vertebral body; wedge-shaped deformity of the D10 and D12 vertebral bodies; concrescence of the D10-D12 articular processes on the left side, D11-D12 articular processes on the right side, and D10-D12 ribs on the left side; bilateral defect of the interarticular portion of the L4 vertebral arch; Spina bifida posterior at the L2-L5 level	No	Diastematomyelia at the level of lumbar enlargement and cauda equina
3	12.1	Kyphoscoliosis	d	Left wedge-shaped lateral T1 and T3 hemivertebrae; between the T5 and T6, T6 and T7 bodies: extra lateral hemivertebrae, hypoplastic intervertebral discs at the T1-2 - T7-8 levels. Spina bifida posterior of the T7 vertebra	No	A bony septum divides the canal into the right and left halves at the T5-T6 level
4	11.6	Scoliosis	D	Concrescence of the D10-L1 vertebral bodies combined with failure of segmentation. Spina bifida posterior at the D10-S3 level	No	Complete sagittal bony septum (19.4 mm thick in the ventral portion and 4.4 mm thick in the dorsal portion of the spinal cord)
5	13.1	Scoliosis	D	Butterfly deformity of the T7, T10, T12 vertebrae; T9 – right-sided wedge-shaped hemivertebra, spina bifida of T8, L2-S1	No	Widening of the spinal canal at the T12-L2 level; splitting of the lumbar enlargement and the medullary cone
6	9.2	Lordoscoliosis	d	Symmetric butterfly defect of the T3 and T5 vertebrae; nonsymmetric butterfly defect of the T7 and T8 vertebrae; spina bifida of the T1, T6-T10, and L5 vertebrae; spina bifida sacralis totalis	No	Osseous diastematic crest at the T3-T11 level
7	19.6	Lordoscoliosis	D	Butterfly defect of the T3, T4, T6, and T10 vertebrae; spina bifida posterior of the T1, T2, T6, T7, and T9 – T12 vertebrae	No	Sagittal bony septum in the spinal canal at the T6, T7 level
8	11.8	Scoliosis	S	Left-sided posterolateral wedge-shaped defect of the T5 vertebra; butterfly defect of the T7, T9-T11 vertebrae; concrescence of the arches of the T4-T6, T7-T10 vertebrae; multiple synostosis of the ribs on the right and left sides; spina bifida posterior of the C6-T3, T11-L1 vertebrae	No	Bony septum of the spinal canal at the apex of kyphosis (T8-T10) lying in the sagittal plane and separating the spinal canal into the right and left lateral portions
9	14.4	Kyphoscoliosis	d	Hypoplastic T4-5,10-11, L3-4 vertebral discs; concrescence of spinous processes of the L2- L3 vertebrae, the 2^nd and 3^rd ribs on the left side; spina bifida at the T10-T12, L3-L4 level	No	Bony septum at the T9-T 10 level
10	18	Kyphoscoliosis	d	Hypoplastic T12-L1, L1-2 vertebral discs; concrescence of the posterior portions of vertebrae at the same level	No	Bony septum at the T10-T12 level
11	9.3	Kyphosis		Wedge-shaped posterior defect of the L2 hemivertebra, hypoplastic T12-L2 vertebral discs	No	Splitting of the medullary cone into two halves at the level of the L2 vertebral body
12	5	Scoliosis	D	Butterfly defect of the T3-T8 vertebrae; wedge-shaped hemivertebra between the T2 and T3 vertebrae on the left side; concrescence of the T2-T4, T7-T10 vertebrae at the level of their arches; hypoplastic T3-4, T5-6, and T6-7 intervertebral discs; spina bifida posterior of the T5, T6, T10-T12 vertebrae	No	Bony septum at the T7-T8 level dividing the spinal canal into the right and left halves
13	9	Kyphoscoliosis	d	Butterfly defect of the T11 vertebra with the right half of the vertebral body predominating; the left half of the body is fused with the L1 vertebral body; L2 is the right-sided lateral wedge-shaped hemivertebra; concrescence of the bodies of the T12 and L3 vertebrae. Hypoplastic T12-L1 – L2-3 discs. Spina bifida posterior of the T12 - S2 vertebrae. Left-sided concrescence of the ribs	A CSF-filled cyst (10.mm in diameter and 36.0 mm long) is detected at the level of lumbar enlargement of the spinal cord. Two terminal filaments linked by a commissure at the L4 level are detected caudally.	Partial bony septum at the level of L2 and L3 vertebral bodies (after subtotal resection of the septum) protruding into the lumen by 9 mm, 23 mm long, 12 mm thick
14	8	Kyphoscoliosis	d	Butterfly T8 and T10 vertebra; concrescence of the bodies of the T7-T10 vertebrae; aplasia of left hemiarches of the T8-T10 vertebrae; L2-L4 vertebral bodies are fused, with their arches separated and a wide diastasis present; concrescence of right hemiarches at the T12, L1 level; multiple rib abnormalities	Syringomyelic cavity (16 mm × 6.7 mm) with smooth walls at the T9-T10 level; diastematomyelia combined with syringomyelia of every vertebral column starting from the T11-T12 intervertebral disc to the S1 spinal unit.	There is a wide continuous sagittal bony septum in the spinal canal at the L2, L3 level, which separates the dural sac into two unequal halves
15	7	Kyphoscoliosis	s	Left-sided lateral T10 hemivertebra fused with the T11 vertebral body; multiple rib abnormalities on the right side	Syringomyelia at the cervical, thoracic, and lumbar levels. Condition after drainage of the syringomyelic cavity at the C3-5 level. Condition after emptying and draining the cystic tumor in the thoracolumbar spine; trophic lesions of the postoperative cicatrix. Syringomyelic cavities are also visualized in both vertebral columns below the T11 level.	There is a 6 mm thick bony septum in the sagittal plane in the spinal canal at the T11-T12 level
16	12	Scoliosis	D	Concrescence of the T1-2, L5-S1 vertebrae; spina bifida posterior at the T4, L5 level; synostosis of the 1^st–3^rd ribs on the right side	Fixed spinal cord, syringomyelia at the T5-7 level.	Diastematomyelia at the L1, L3 level

Case number	Cobb angle		Type of surgery	Neurological symptoms prior to surgery	Neurological symptoms after surgery
Case number	preoperative	postoperative	Type of surgery	Neurological symptoms prior to surgery	Neurological symptoms after surgery
1	85°	46°	Skeletal traction, correction by segmental spinal instrumentation	No abnormalities	No changes
2	75°	38°	Antares	No abnormalities	No changes
3	100°	76°	Skeletal traction, correction by segmental spinal instrumentation	No abnormalities	No changes
4	68°	65°	Skeletal traction, correction by segmental spinal instrumentation	No abnormalities	No changes
5	70°	46°	Skeletal traction, correction by segmental spinal instrumentation	Mild peripheral paraparesis of the lower limbs without pelvic organ dysfunction	No changes
6	73°	46°	Skeletal traction, correction by segmental spinal instrumentation	No abnormalities	No changes
7	117°	94°	Skeletal traction, correction by segmental spinal instrumentation	No abnormalities	No pathology
8	114°	101°	Correction by segmental spinal instrumentation	Pyramidal insufficiency syndrome	No changes
9	85°	51°	Skeletal traction, correction by segmental spinal instrumentation	No abnormalities	No changes
10	75°	27°	Skeletal traction, correction by segmental spinal instrumentation	No abnormalities	No changes
11	10°	63°	Skeletal traction, correction by segmental spinal instrumentation	No abnormalities	No changes
12	51°	36°	Multi-stage correction using VEPTR instrumentation (5 stages)	No abnormalities	No changes
13	103°	92°	Multi-stage correction using VEPTR instrumentation (4 stages)	No abnormalities	No changes
14	146°	102°	Multi-stage correction using VEPTR instrumentation (the first stage)	Mild combined paraparesis without pelvic organ dysfunction	No changes
15	105°	77°	Multi-stage correction using VEPTR instrumentation (the first stage)	Lumbar myelopathy presenting as flaccid paraparesis and pelvic organ dysfunction	No changes
16	60°	9°	Skeletal traction, correction by segmental spinal instrumentation	Thoracolumbar myelopathy presenting as mild left-sided monoparesis of a lower limb	No changes

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[1] Correspondence: Mikhail V. Mikhaylovskiy. Frunze str., 17, Novosibirsk, 630091, Russia. MMihailovsky@niito.ru