A typical case of spinal cord intramedullary teratoma combined with congenital spinal developmental malformation - a case of scoliosis

Spinal cord intramedullary tumors account for about 16 % of spinal canal tumors and 2% to 4% of all central nervous system tumors .

16 2% to 4% of the common ones include ependymoma, astrocytoma, teratoma,

Case characteristics

case characteristics case characteristics

Adolescent female, 13 years old, junior high school student

13

admission examination

admission examination admission examination

His vital signs were stable, his voice was clear, his height was 150 cm, and his weight was 53 kg

▲Figure 1 The patient's right shoulder showed a razorback-like change, and the thoracic spine was deviated to the right

▲Figure 1 The patient’s right shoulder showed a razorback-like change, and the thoracic spine was deviated to the right ▲Figure 1 The patient’s right shoulder showed a razorback-like change, and the thoracic spine was deviated to the right

Auxiliary examination

Auxiliary examination

Hematuria and stool were routine, no obvious abnormality in coagulation function, and normal blood HCG and alpha-fetoprotein levels

▲Figure 2 Preoperative X-ray films, frontal and lateral, left and right oblique, full-length lower extremity films in standing, and sitting

▲Figure 2 Preoperative X-ray films, frontal and lateral, left and right oblique, full-length lower extremity films in standing, and sitting

▲Figure 3 The preoperative three-dimensional CT of the whole spine can further evaluate the bone development of each segment, indicating that the T2 vertebral body is a hemivertebra, and the T6-7 vertebral bodies are abnormally developed

▲Figure 3 The preoperative three-dimensional CT of the whole spine can further evaluate the bone development of each segment, indicating that the T2 vertebral body is a hemivertebra, and the T6-7 vertebral bodies are abnormally developed

▲Figure 4 Whole spine MRI showed intramedullary mass at T6 level, mixed signal at T2, uneven enhancement, combined with the developmental deformity of the vertebral body, considering the possibility of intramedullary teratoma

▲Fig.

preoperative assessment preoperative assessment

The patient was an adolescent child who was born at full term, and there was no obvious abnormality in the growth and development process of infants and young children

surgical procedure

surgical procedure surgical procedure

After successful general anesthesia, the motor somatosensory evoked potentials of the limbs were connected, and the patient was placed in the prone position

The T6 spinous process and the lamina on both sides were removed to expose the dural sac.

Use ultrasonic osteotome and lamina rongeur for osteotomy, bite out T6~7 bilateral facet joints and part of the lamina, and excise the vertebral body to the anterior bone cortex in a forward "V" shape, T2~3, T5~ 6, T7~8 facet joints and part of the lamina were removed

▲Fig.

▲Fig.

▲Figure 6 Pathology suggests mature cystic teratoma without other germ cell tumor components

▲Fig.
6 Pathology suggests mature cystic teratoma, but no other germ cell tumor components ▲Fig 6 Pathology suggests mature cystic teratoma, but no other germ cell tumor components

Postoperative situation

Postoperatively, the patient developed left lower extremity motor dysfunction, and decreased superficial sensation and proprioception
.
Giving hormones, dehydration, acid suppression, nerve nutrition, thrombosis prevention and other treatments
.
After the subcutaneous drainage tube was removed, early rehabilitation exercise was performed
.
The patient was discharged 10 days after the operation, and the motor function of the left lower extremity recovered somewhat at the time of discharge
.
After being discharged from the hospital, he continued his rehabilitation in a specialized hospital
.
At present, 2 months after the operation, he has been recovering with a brace
.

▲Fig.
7 Postoperative imaging blood test showed satisfactory resection of intramedullary lesions, no cerebrospinal fluid leakage and subcutaneous effusion in the operation area
.
Thoracic scoliosis was mostly corrected, with satisfactory internal fixation position and bone grafting

▲Fig.
7 Postoperative imaging blood test showed satisfactory resection of intramedullary lesions, no cerebrospinal fluid leakage and subcutaneous effusion in the operation area
.
The thoracic scoliosis was mostly corrected, and the internal fixation position and bone graft were satisfactory .
Thoracic scoliosis was mostly corrected, with satisfactory internal fixation position and bone grafting

case discussion

case discussion case discussion

01 Congenital spinal deformity

01 Congenital spinal deformity

Congenital spinal deformity can be divided into segmental malformation and vertebral malformation.
The incidence rate is about 1/10000.
If not treated by surgery, most cases will develop spinal deformity
.
Unilateral malsegmentation, where the bony bridge restricts the growth and development of the concave side, can lead to scoliosis
.
Poor spine formation, the formation of lateral hemivertebrae can also cause scoliosis, while the left and right symmetrical hemivertebrae are not fused at the front midline, called butterfly vertebrae, and generally do not cause scoliosis
.
This patient had a congenital spinal deformity with both malsegmentation and malformation of the spine, and the scoliosis gradually increased with puberty
.
Aggressive surgical treatment is required
.

▲Figure 8 The weekly "reading club" of our department, this case reviewed the congenital spinal deformity before the operation

▲Fig.
8 Our department's weekly "reading club", the case reviewed the congenital spinal deformity before the operation

02 Level 6 Osteotomy

02 Level 6 Osteotomy02 Level 6 Osteotomy

Osteotomy has a history of nearly 100 years in the field of spinal surgery.
The most widely used grading system is the Schwab grading system proposed by the famous Dr.
Frank Schwab in 2014.
The system is based on the anatomical surgical approach for spinal deformity correction.
A grading system designed to facilitate scholarly communication and standardize research results
.
The system classifies 6 types of osteotomy methods, reflecting the severity of spinal instability and the potential angle of deformity correction
.

In simple terms, a level 1 osteotomy of each vertebral segment can theoretically correct 10 degrees of deformity, a level 2 osteotomy of a segment can theoretically correct 20 degrees of deformity, and so on
.
In this case, a grade 3 osteotomy of the T6 vertebral body and a grade 1 osteotomy of multiple levels were performed
.
For the application of the osteotomy technique, neurosurgeons also need to fully exchange and learn with spine surgeons, and further reflect on this.
If this patient performs a level 3 osteotomy on one side at the level of the T2-3 hemivertebra to release the anteriorly fused vertebrae A better correction effect will be achieved, but this means that the operation time will be further prolonged and the blood loss will increase, and the benefit needs to be evaluated during the operation
.

▲Figure 9 Schematic diagram of the six-step osteotomy, the picture is selected from the article The comprehensive anatomical spinal osteotomy classification by Frank Schwab

▲Fig.
9 Schematic diagram of six-step osteotomy, the picture is from Frank Schwab's article The comprehensive anatomical spinal osteotomy classification ▲Fig.
9 Six-step osteotomy, the picture is taken from Frank Schwab's article The comprehensive anatomical spinal osteotomy classification

03 Surgical strategy for intramedullary tumors with scoliosis

03 Surgical strategies for intramedullary tumors with scoliosis 03 Surgical strategies for intramedullary tumors with scoliosis

The most common intramedullary tumor in children is astrocytoma.
As the intramedullary astrocytoma progresses, the patient's muscle function will be gradually impaired, affecting the stability of the spine
.
At the same time, adolescence is the most rapid period of skeletal development
.
In addition, the growth and development of the spine is an important factor that induces spinal deformities
.
Combining the above reasons, juvenile spinal cord intramedullary tumors are often complicated by spinal deformities
.
Its incidence can reach about 50%
.
This type of spinal deformity is mostly neuromuscular spinal deformity
.

Nonsurgical measures rarely fully control the progression of neuromuscular scoliosis
.
In addition, because the patient had a primary spinal cord tumor, a laminectomy was required to fully expose the tumor
.
This surgical maneuver further destabilizes the spinal structure
.
Although there have been reports that the use of laminar reduction techniques can reduce the incidence of postoperative spinal deformity
.
However, previous retrospective analysis showed that preoperative spinal deformity was an independent risk factor for the exacerbation of spinal deformity after tumor resection
.

The jury is still out on whether fractionated or single-stage surgery is used to manage intramedullary tumors and scoliosis
.
We believe that for juvenile benign tumors such as low-grade astrocytoma, teratoma, and severe scoliosis, the COBB angle is greater than 50 degrees
.
One-stage surgery can be attempted for intramedullary tumors and scoliosis
.
The surgical procedure and perioperative management require careful preoperative evaluation, skilled surgical skills and team effort to achieve satisfactory results
.

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