Our study yielded a total of 6 patients (3 female). Patient demographics are described in Table 1.
Table 1
Demographics and Baseline Clinical Information
Case No | Age, Sex | Race, Ethnicity | Type of Hypertonia | CP Etiology | GMFCS | G-Tube | Scoliosis |
1 | 22,M | Hispanic/Latino | Triplegic, Mixed | post-meningitis hydrocephalus | II | No | No |
2 | 14,M | NHW | Tetraplegic, Mixed | Schizencephaly | V | Yes | Yes |
3 | 9,F | NHW, Black | Tetraplegic, Mixed | nonaccidental trauma | V | Yes | Yes |
4 | 15,F | Hispanic/Latino | Tetraplegic, Mixed | Microlissencephaly | V | Yes | Yes |
5 | 7,M | Hispanic/Latino | Tetraplegic, Spastic | unknown | V | Yes | Yes |
6 | 34,F | NHW | Hemiplegic, Mixed | IVH | I | No | No |
No, number; GMFCS, gross motor function classification scale; M, male; F, female; NHW, non-Hispanic White; CP, cerebral palsy; IVH, intraventricular hemorrhage; G-tube, Gastrostomy tube
Mean age at surgery was 16.8 years (range 7–34). Three patients had quadriplegic mixed hypertonia, one patient had quadriplegic spasticity, one patient had triplegic mixed hypertonia, and one patient had mixed hemiplegic hypertonia. 50% of patients were delivered prematurely, mean gestational age was 34 weeks (range 26–40 weeks). CP etiologies included CNS injury, CNS infection, genetics and unknown. Four patients had a GMFCS of V, one had a score of II, and one had a score of I. Four patients had a gastrostomy tube and scoliosis, no patients had a tracheostomy tube, ITBP placement, or prior spinal fusion.
Four patients underwent a bilateral C6-T1 VDR, one patient underwent a left C7-T1 rhizotomy, and another underwent a left C6-T1 rhizotomy. Mean operative duration was 239 ± 25.2 minutes, mean estimated blood loss was 93.3 ± 83.2 mL, mean length of stay was 7 ± 3.9 days (Table 2).
Table 2
Surgical Characteristics and Perioperative Events
Case No | Surgery | Roots Cut, % | LOS (days) | Op time (min) | EBL (ml) | Follow-up (days) | Complications |
1 | Left C7-T1 | 80 | 7 | 252 | 200 | 392 | --- |
2 | Bilateral C6-T1 | 80–90 | 3 | 552* | 200 | 105 | -- |
3 | Bilateral C6-T1 | 80–90 | 14 | 457* | 50 | 118 | Transient BiPap Support |
4 | Bilateral C6-T1 | 80–90 | 8 | 491* | 50 | 206 | Transient BiPap Support |
5 | Bilateral C6-T1 | 80–90 | --^ | 210 | 30 | 136 | -- |
6 | Left C6-T1 | 50 dorsal 80 ventral | 4 | 239 | 30 | 42 | -- |
*pt underwent concurrent lumbosacral VDR |
^LOS is not reported because this surgery was performed during a prolonged hospitalization for various issues to help address subsequent tone control.
No, number; LOS, length of stay; Op, operative; EBL, estimated blood loss; min, minutes; BiPap = bilevel positive airway pressure
Average follow-up was 191.4 days (range 42–392 days). The mean difference of proximal upper extremity mAS was − 1.4 ± 0.55 (p = 0.002), and − 2.2 ± 0.45 (p < 0.001) for the distal upper extremity (Fig. 2).
There was not a significant difference between pre- and post-operative mAS in the lower extremities (p > 0.05). All patients demonstrated quality of life improvements, mainly in dressing, orthotics fit, and ease when transferring (Table 3).
Table 3
Case No | Quality of Life Improvement | Physical Exam Improvement |
1 | Dressing, orthotics | No significant dystonia, can flex/extend arm. Able to supinate with good control. Able to hold a tray with his left arm. Able to hold controller and play video games better. |
2 | Positioning, transfers | Able to extend arm straight |
3 | Positioning | Able to open hand- previously fisted |
4 | Passive dressing, changing, transfers, orthotics | Improved tone |
5 | Passive dressing, changing, positioning, transfers, orthotics | Improved tone, no clonus, full range of motion |
6 | Dressing, Orthotics | Improved grip Able to close and open hand around ball. Can stack rings |
No, number; G-tube, gastrostomy tube; GA, gestational age; C, cervical
Two patients with existing restrictive lung disease required transient increased BiPAP for 1- and 3-days following surgery. There was no incidence of infection, pneumonia, or wound dehiscence. Two patients had available follow-up at 6-months postoperatively and exhibited sustained tone control and quality of life improvements. Long-term functional outcomes were not available for the remaining four patients.
Case 1
A 22-year-old man presented to the movement disorders clinic with triplegic mixed hypertonia secondary to CP caused by post-meningitic hydrocephalus. This patient has trialed botulinum toxin injections, multiple antispasmodic medications, and baclofen which was discontinued due to lowered seizure threshold.
On examination he had a GMFCS of II and preoperative MACS of 5. He had dystonia in his left upper extremity with BADS of 8. His brachial hypertonia was interfering with his ability to complete job requirements and causing significant pain.
A left C7-T1 VDR was performed with 80% of both the ventral and dorsal roots sectioned. There was a significant reduction in tone, his BADS was 6 at 6-months postoperatively and 3 at 1-year post-operative. At 1-year post-operatively his MACS score was a 3 and he demonstrated improved function in his left arm. He met 100% of his goals which included holding a tray in his left hand while opening a door with his right hand furthermore he was able to supinate with good control. He noted improvement in dressing and wearing of orthotics and was able to perform his job successfully.
Case 6
A 34-year-old woman presented with hemiplegic mixed hypertonia secondary to CP caused by intraventricular hemorrhage. She had trialed multiple antispasmodics and botulinum toxin injections without adequate effect.
On examination she had a GMFCS of I and a preoperative MACS of 5. She had severe left brachial hypertonia with a mAS of 3 in her proximal and distal left upper extremity. Her hand was rigid in dystonic posture and she was unable to use it.
A left C6-T1 VDR was performed with 50% of dorsal and 80% of ventral roots sectioned. Postoperatively there was a significant reduction in tone. At 42 days postoperatively her she demonstrated a proximal mAS of 1 with complete improvement in distal hypertonicity (mAS = 0). She met 100% of her goals which included opening and closing her hand. At 3-months postoperatively she was able to open and close her hand around a ball and transfer rings on a post. She also demonstrated improved grip due to decreased interference by tone. She was extremely pleased with the result and noted improvement in dressing and orthotics wearing.