[One stage three-column osteotomy for the treatment of scoliosis with split spinal cord malformation].

OBJECTIVE: To analyze the effectiveness and safety of one stage three column osteotomy in treatment of scoliosis with split spinal cord malformation. METHODS: The clinical data of 41 patients with scoliosis and split spinal cord malformation underwent one-stage three-column osteotomy from January 2015 to December 2017 were retrospectively analyzed. There were 17 males and 24 females with average age of (25.14±4.51) years old and the average weight of (65.14±9.11) kg. According to the classification of longitudinal spina bifida, 15 cases of Pang typeⅠwere group A and 26 cases of Pang typeⅡwere group B. The general situations of two groups were recorded ; preoperative and postoperative Cobb angle were observed and the correction rate of Cobb angle of coronal plane was calculated ; the coronal and sagittal torso offset distances were compared between two groups and the trunk balance was evaluated ; the complication of two groups was recorded. RESULTS: All 41 patients were followed up for more than 12 months. The operation time, intraoperative blood loss, and perioperative blood transfusion volume in group A were (610.14±115.02) min, (4 001.12±1 014.33) ml, (3 951.14±1 021.55) ml, respectively, and group B were (520.12±101.14) min, (2 701.57±1 021.45) ml, (2 565.77±880.47) ml, the difference between the two groups was statistically significant (P<0.05). The postoperative hospital stays in the group A and B were (9.45±4.21) days and (9.14±3.01) days, respectively, and there was no significant difference (P>0.05). There was no significant difference in postoperative coronary Cobb angle and correction rate between two groups (P>0.05). Immediately after surgery and 12 months after surgery, there was no significant difference in the trunk displacement distance of coronal view and sagittal view between two groups (P>0.05). Six patients in group A had complications, which was higher than that in group B of 1 case (χ2=4.885, P< 0.05). CONCLUSION: One-stage three-column osteotomy in treatment of scoliosis with split spinal cord malformation has high correction rate and good balance of the trunk. However, for patients with typeⅠsplit spinal cord malformation, they will face longer operation time, more intraoperative bleeding volume, large amount of perioperative blood transfusion and higher risk of complications, and the safety is not as good as that of typeⅡpatients. Therefore, in the actual treatment of scoliosis, especially for those with typeⅠsplit spinal cord malformation, a more reasonable surgical plan should be developed in combination with the actual situations of the patients, so as to improve the safety of the operation.

One stage three-column osteotomy for the treatment of scoliosis with split spinal cord malformation / 中国骨伤

OBJECTIVE@#To analyze the effectiveness and safety of one stage three column osteotomy in treatment of scoliosis with split spinal cord malformation.@*METHODS@#The clinical data of 41 patients with scoliosis and split spinal cord malformation underwent one-stage three-column osteotomy from January 2015 to December 2017 were retrospectively analyzed. There were 17 males and 24 females with average age of (25.14±4.51) years old and the average weight of (65.14±9.11) kg. According to the classification of longitudinal spina bifida, 15 cases of Pang typeⅠwere group A and 26 cases of Pang typeⅡwere group B. The general situations of two groups were recorded ; preoperative and postoperative Cobb angle were observed and the correction rate of Cobb angle of coronal plane was calculated ; the coronal and sagittal torso offset distances were compared between two groups and the trunk balance was evaluated ; the complication of two groups was recorded.@*RESULTS@#All 41 patients were followed up for more than 12 months. The operation time, intraoperative blood loss, and perioperative blood transfusion volume in group A were (610.14±115.02) min, (4 001.12±1 014.33) ml, (3 951.14±1 021.55) ml, respectively, and group B were (520.12±101.14) min, (2 701.57±1 021.45) ml, (2 565.77±880.47) ml, the difference between the two groups was statistically significant (0.05). There was no significant difference in postoperative coronary Cobb angle and correction rate between two groups (>0.05). Immediately after surgery and 12 months after surgery, there was no significant difference in the trunk displacement distance of coronal view and sagittal view between two groups (>0.05). Six patients in group A had complications, which was higher than that in group B of 1 case (=4.885, < 0.05).@*CONCLUSION@#One-stage three-column osteotomy in treatment of scoliosis with split spinal cord malformation has high correction rate and good balance of the trunk. However, for patients with typeⅠsplit spinal cord malformation, they will face longer operation time, more intraoperative bleeding volume, large amount of perioperative blood transfusion and higher risk of complications, and the safety is not as good as that of typeⅡpatients. Therefore, in the actual treatment of scoliosis, especially for those with typeⅠsplit spinal cord malformation, a more reasonable surgical plan should be developed in combination with the actual situations of the patients, so as to improve the safety of the operation.

Split spinal cord malformations in 4 Holstein Friesian calves.

BACKGROUND: The split spinal cord malformation (SSCM) is an uncommon congenital malformation of the vertebral canal in which parts of the spinal cord are longitudinally duplicated. In SSCM Type I, each spinal cord has its own dura tube. In the SSCM Type II, both parts of the spinal cord are surrounded by a common dura tube. CASES PRESENTATION: During the clinical examination one calf showed ambulatory paresis and 3 calves non-ambulatory paraparesis. Calf 4 additionally had a congenital tremor. The examination of calf 4 using magnetic resonance imaging (MRI) showed a median hydrosyringomyelia at the level of the 4th lumbar vertebra. The caudal part of this liquid-filled cavity was split longitudinally through a thin septum. From there, the spinal cord structures duplicated with an incomplete division, so that the transverse section of the spinal cord appeared peanut-shaped and in each half a central canal could be observed. The pathological-anatomical examination after euthanasia showed a duplication of the spinal cord in the area of the lumbar vertebral column in all calves. The histopathological examination revealed two central lumbar vertebral column channels. The two spinal cord duplicates were each surrounded by two separate meninges in calf 2 (SSCM type I); in the other calves (1, 3, 4, and) the two central canals and the spinal cord were covered by a common meninx (SSCM type II). A pedigree analysis of calves 2, 3 and 4 showed a degree of relationship suggestive of a hereditary component. This supports the hypothesis of a possible recessive inheritance due to common ancestors, leading to partial genetic homozygosity. CONCLUSIONS: The clinical appearance of SSCM can vary widely. In calves with congenital paralysis SSCM should always be considered as a differential diagnosis. A reliable diagnosis intra vitam is possible only with laborious imaging procedures such as MRI. Further studies on the heritability of this malformation are necessary to confirm a genetic cause of this disease.

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

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.

Diagnosis and treatment of split spinal cord malformations combined with congenital spinal deformity / 中华实用儿科临床杂志

Split spinal cord malformation(SSCM) is a rare neurological abnormality that spinal cord was separated in the sagittal plane.Based on the tissue element and type of thecal shvac,SSCM is classified into Ⅰ,Ⅱ and mixed type.Congenital spinal deformity(CSD) is caused by the vertebral body formed or segmented obstacles.CSD can be divided into 3 types:Ⅰ,Ⅱ and mixed type.Both of SSCM and CSD are diagnosed depending on clinical symptoms,X rays,computed tomography (CT),magnetic resonance imaging(MRI) and other examine.Genitourinary and cardiac ultrasound should be performed at the same time.Progression of spinal deformity and nerve injury are accompanied with the growth process of children with CSD and SSCM.Early surgery can avoid further injury of nervous system,prevent spinal deformity progress and obtain good correction.Segmental tissue removal,tethered cord release and spinal deformity correction are generally consisted into an individual operative plan at present.Traditional surgical option included 2 steps:tissue removal and tethered cord release are conducted at Ⅰ phase surgery;then conducted the spinal deformity correction at Ⅱ phase.However,some reports suggested that completed the process of tissue removal,tethered cord release and spinal deformity correction at one surgery also obtain a satisfied effictiveness.The aim of this research is to review and discuss the diagnosis of the SSCM with CSD and evaluate the treatment effectiveness of Ⅰ phase surgery instead of the traditional surgical option.

Malformación del cordón espinal hendido con expresión clínica en el período neonatal / Split spinal cord malformation seen at the neonatal stage

La malformación del cordón espinal hendido es una forma rara de disrafia espinal oculta, se reporta que representa el 3 por ciento de los disrafismos ocultos. El mayor porcentaje de ellos es de localización lumbar. En la literatura cubana solo tenemos referencia de 2 pacientes publicados, una adulta y otro caso pediátrico. Se reporta un recién nacido remitido a nuestro centro por presentar meningocele occipital. Se detecta la presencia de una fosita en región sacro-coccígea, hipotonía muscular en miembro inferior derecho, pie varo con poca movilización del miembro, e incontinencia de esfínter vesical y anal. Los estudios de imagen demuestran la presencia de 2 hemimédulas a nivel lumbar, por lo que se confirma que el recién nacido presenta un síndrome de malformación del cordón espinal hendido, una entidad poco común. Presenta, además, la particularidad de haber sido diagnosticada en el período neonatal por exhibir desde su nacimiento manifestaciones clínicas, como consecuencia de esta entidad, aspecto también relevante, pues, habitualmente, la afección expresa la sintomatología más tarde en la vida, o se mantiene asintomática(AU)

Split spinal cord malformation is a rare form of occult spinal dysraphia and is reported in 3 percent of occult dysraphisms which are mainly located in the lumbar region. The Cuban literature made reference to two patients with this condition, an adult and a child. This is the case of a male newborn referred to our center because he presented with occipital meningocele. The examination revealed a small fossa in the sacrococcygeal region, muscular hypotonic in his right lower limb, varus foot and little movement as well as anal and vessical sphincter incontinence. Imaging studies show the presence of two hemicords at the lumbar region, so it is confirmed that this newborn infant had split spinal cord malformation syndrome, which is an uncommon entity. It is interesting to note that this malformation was diagnosed at the neonatal phase because of its clinical manifestations, a relevant aspect since this illness generally shows its symptoms in later life or remains asymptomatic(AU)

Malformación del cordón espinal hendido con expresión clínica en el período neonatal / Split spinal cord malformation seen at the neonatal stage

La malformación del cordón espinal hendido es una forma rara de disrafia espinal oculta, se reporta que representa el 3 % de los disrafismos ocultos. El mayor porcentaje de ellos es de localización lumbar. En la literatura cubana solo tenemos referencia de 2 pacientes publicados, una adulta y otro caso pediátrico. Se reporta un recién nacido remitido a nuestro centro por presentar meningocele occipital. Se detecta la presencia de una fosita en región sacro-coccígea, hipotonía muscular en miembro inferior derecho, pie varo con poca movilización del miembro, e incontinencia de esfínter vesical y anal. Los estudios de imagen demuestran la presencia de 2 hemimédulas a nivel lumbar, por lo que se confirma que el recién nacido presenta un síndrome de malformación del cordón espinal hendido, una entidad poco común. Presenta, además, la particularidad de haber sido diagnosticada en el período neonatal por exhibir desde su nacimiento manifestaciones clínicas, como consecuencia de esta entidad, aspecto también relevante, pues, habitualmente, la afección expresa la sintomatología más tarde en la vida, o se mantiene asintomática.

Split spinal cord malformation is a rare form of occult spinal dysraphia and is reported in 3 % of occult dysraphisms which are mainly located in the lumbar region. The Cuban literature made reference to two patients with this condition, an adult and a child. This is the case of a male newborn referred to our center because he presented with occipital meningocele. The examination revealed a small fossa in the sacrococcygeal region, muscular hypotonic in his right lower limb, varus foot and little movement as well as anal and vessical sphincter incontinence. Imaging studies show the presence of two hemicords at the lumbar region, so it is confirmed that this newborn infant had split spinal cord malformation syndrome, which is an uncommon entity. It is interesting to note that this malformation was diagnosed at the neonatal phase because of its clinical manifestations, a relevant aspect since this illness generally shows its symptoms in later life or remains asymptomatic.

Sacrococcygeal Teratoma with Split Spinal Cord Malformation

The incidence of diastematomyelia associated with teratoma is extremely rare. We present a case of sacrococcygeal teratoma in a neonate with split spinal cord malformation(SSCM). Magnetic resonance imaging(MRI) showed a heterogenous mass lesion with cyst in the sacrococcygeal region and multiple spinal anormalies (diastematomyelia, tethered cord, hydromyelia, and hemivertebrae). The mature teratoma was confirmed on histopathological examination. In SSCMs, the potential for coexisting congenital anomalies at separate levels of the spinal cord must be considered in radiological investigations.