ABSTRACT
Performing a hemispherotomy or hemispherectomy is known to treat medically intractable epilepsy successfully, yet contralateral hemiparesis and increased muscle tone follow the epilepsy surgery. Spasticity and coexisting dystonia presumably cause the increased muscle tone in the lower extremity on the opposite side of epilepsy surgery. However, the extent of the role of spasticity and dystonia in high muscle tone is unknown. A selective dorsal rhizotomy is performed to reduce spasticity. If a selective dorsal rhizotomy is performed in the affected patient and muscle tone is reduced, the high muscle tone is not due to dystonia. Two children, who previously underwent a hemispherectomy or hemispherotomy, had a selective dorsal rhizotomy (SDR) performed in our clinic. Both children underwent orthopedic surgery to treat heel cord contractures. To study the extent of the role of spasticity and dystonia in high muscle tone, the mobility of the two children was examined pre- and post-SDR. The children had follow-ups 12 months and 56 months after SDR to study long-term effects. Before SDR, both children showed signs of spasticity. The SDR procedure removed spasticity, and muscle tone in the lower extremity became normal. Importantly, dystonia did not surface after SDR. Patients started independent walking less than two weeks after SDR. Sitting, standing, walking, and balance improved. They could walk longer distances while experiencing less fatigue. Running, jumping, and other more vigorous physical activities became possible. Notably, one child showed voluntary foot dorsiflexion that was absent before SDR. The other child showed improvement in voluntary foot dorsiflexion that was present before SDR. Both children maintained the progress at the 12 and 56-month follow-up visits. The SDR procedure normalized muscle tone and improved ambulation by removing spasticity. The high muscle tone following the epilepsy surgery was not due to dystonia.
ABSTRACT
Background A limited number of publications have described a reduction of spasticity associated with hereditary spastic paraplegia (HSP) after selective dorsal rhizotomy (SDR). Typically, the SDR procedure is performed on patients with spastic cerebral palsy to remove spasticity and to help these patients with ambulatory function. Whether SDR has similar effects on HSP patients, requires further investigation. Thus, we are providing a personal experience of the effects of SDR on this specific cohort of patients. Objectives To examine the safety of SDR, changes in spasticity, and ambulatory function after SDR on patients with HSP. Methods The Institutional Review Board of Washington University School of Medicine approved this study (#201704003). A total of 37 children and adults received SDR for the treatment of HSP-associated spasticity between 1988 and 2021. SDR was performed through an L1 laminectomy, as we previously described in an earlier publication. The patients took part in the follow-up examination either in-person or by email. The follow-up focused on the patients' motor functions (primarily ambulation), adverse effects of SDR, and orthopedic treatments after SDR. Results Of the total 37 patients who participated in this study, 46% were female and 54% were male. The age range of when HSP was diagnosed was one month to 34 years. Six of the patients' diagnoses were made, based on the family history of HSP in six patients and the remaining 31 patients' diagnoses were confirmed by genetic tests. The most common genetic mutations were SPG4 and SPG3A. Of the patients with positive genetic tests, 40% had no family history of HSP. SDR was performed at the age of 2 to 45 years (mean: 14.7 years). The follow-up period ranged from 0 to 33 years (mean: 3.8 years). One patient developed a spinal fluid leak requiring surgical repair. Two patients reported mild numbness in parts of the lower limbs. Spasticity was removed in 33 patients (89%). Four patients (11%) experienced a return in spasticity. Regarding ambulatory function, 11% of patients reported a decline in function. Two patients walked independently before surgery but declined, requiring a wheelchair eight years and seven years, respectively, after surgery for each patient. In contrast, 16% saw an improvement in ambulatory function, improving from walking with a walker to walking independently. The remaining 73% of patients maintained their level of ambulation. These two groups of patients showed improvement in other motor functions and independence. Conclusions The present analysis suggests the potential role of SDR in the management of spasticity in HSP patients. We found no sign of SDR being a direct cause of deleterious effects on patients with HSP.
ABSTRACT
Background A selective dorsal rhizotomy (SDR) is employed to treat spastic cerebral palsy. The surgical techniques and patient care protocols vary among hospitals. One of the variations is the age cut-off for SDR. We have been advocating SDR to be performed early - especially at ages 2 and 3. With this study, we are reporting the feasibility and parent-reported surgical outcomes of receiving SDR at an early age for the treatment of spastic diplegia. Objectives Our aim is to examine the safety and benefits of receiving SDR at the ages of 2 and 3 for the treatment of spastic diplegia. Methods The Institutional Review Board (IRB) of Washington University School of Medicine approved this retrospective quality of life survey and chart review (approval #202009056). The subjects of this study were children and teens (ages: 3.9-18.1) with spastic diplegic cerebral palsy who underwent SDR at ages 2 or 3 between years 2005 and 2019 at St. Louis Children's Hospital. Only domestic patients that were minors at the time of the study were selected to be participants in compliance with IRB regulations to protect patient health information that could potentially be breached by sending information to an incorrect or dated email. Thus, all contact was made through postal mail. The study included 141 patients from a total of 362 eligible patients. Parents of eligible patients were sent the research survey via postal mail. Only patients who responded to the survey were included in this study. The survey included questions on demographic information, quality of life, health perception, motor and ambulatory functions, braces and orthotics, pain issues, side effects of SDR, and post-SDR treatment. Results The study included 141 diplegic patients. Of all patients at the time of the study, 91% reported an improvement in walking, 92% in standing, and 89% in sitting. In daily life activities, 87% of patients became more independent after SDR. 65% of patients were able to walk without a walking aid and about 4% were not able to walk. 11% of all patients relied mostly on a wheelchair. Moreover, 43% of patients were able to run independently. Regarding post-SDR orthopedic surgery, 48% of patients received at least one type of orthopedic surgery, with Achilles tendon lengthening, hamstring lengthening, and calf muscle release being the most common types. Conclusions SDR performed at an early age through a single-level laminectomy was proved feasible and safe. A follow-up until the adult age (18 years) showed improvements in walking and other motor functions. The results support the implementation of early-age SDR for the treatment of spastic diplegia.
ABSTRACT
Background Selective dorsal rhizotomy (SDR) can remove spasticity in cerebral palsy (CP). Spastic hemiplegia is associated with spasticity in the upper and lower limbs on one side. Only a single report described the outcome of SDR specifically in patients with spastic hemiplegic CP. The effect of SDR on spastic hemiplegia requires further investigation. Objectives To analyze the outcomes of motor functions, the quality of life, and satisfaction of patients who received SDR for the treatment of spastic hemiplegia. Methods A total of 29 children and 1 adult who received SDR were surveyed. The survey questionnaire asked about demographic information, patient's perception of SDR, functional outcomes, SDR surgical outcomes, pain, braces/orthotics, and post-SDR treatment. Results Our study included 30 patients. The age at the time of surgery was 2 to 36 years. The follow-up period ranged from one to six years. Of all parents, 90% of parents reported that SDR benefited their children, and 93% stated that they would recommend the SDR procedure to other families of children with hemiplegic CP. Of all patients, 90% reported improved walking, 63% reported improved sitting, and 87% reported improved balance and posture. In daily life functioning after the SDR, 67% were more independent and confident. Moreover, 33% of patients were pain-free and 43% had reduced pain in their legs and back. In activities of daily living, 93% transferred independently from one position to another. A majority of the patients reported regular strengthening and stretching of the lower limb, and 50% of the patients played sports. A majority (73%) of patients underwent post-SDR orthopedic surgery for heel cord, hamstring, and adductor contractures. Five patients experienced numbness in the small part of the lower limb after SDR. None reported that the numbness affected their daily activities. One child required surgical repair of the cerebrospinal fluid leak. Conclusions In our 29 children and 1 adult with spastic hemiplegia, SDR improved motor function and daily life function. Nearly all parents of children and the one adult felt that SDR was beneficial and that they would recommend surgery to other children with spastic hemiplegia.
ABSTRACT
Background Spastic triplegia is a recognized subtype of cerebral palsy (CP). In the course of treating spastic triplegic children with selective dorsal rhizotomy (SDR), we found that some children who had "minimal or mild involvement" in the stronger arm improved strikingly after undergoing SDR. Some of them became independent ambulators, which was an outcome that is not usually achieved in spastic quadriplegic children. However, the literature currently contains no data on the natural clinical course and the effects of CP interventions on spastic triplegia. Objectives Our aim was to elucidate the clinical characteristics of spastic triplegia and the effects of SDR on functional outcomes and the quality of life after childhood SDR. Methods The Institutional Review Board of the Washington University School of Medicine approved this quality of life survey (number: 201908177). The subjects of this study were children and adults (ages: 3.9-23.8 years at the time of the survey completion; mean: 12.1 ± 5.1 years) with spastic triplegic CP who had undergone SDR (ages: 2.2-15.9 years; mean: 6.1 ± 3.2 years) between 2003 and 2018 at the St. Louis Children's Hospital. The follow-up period ranged from 1-16 years (mean: 6.0 ± 4.3 years). The study included a 76-patient cohort selected from a total of 253 spastic triplegic CP patients who had undergone SDR. All 253 patients were contacted via email or postal mail soliciting their participation in the study including the survey. The cohort included all patients who responded. The survey included questions on demographic information, quality of life, perceptions of health and the SDR procedure, motor and ambulatory functions, braces and orthotics, pain issues, side effects of SDR, and post-SDR treatment. Results Thirteen patients had presented with scissored gait, and these patients had undergone partial ventral rhizotomy (PVR) of L1-3 ventral roots immediately after the completion of SDR. Of note, 91% of 76 patients reported that SDR improved their quality of life, and 93% would recommend the procedure to other patients. After SDR, 21 more patients were able to run, 14 more played recreational sports, and 18 more could walk without using walking aids. Sixteen fewer patients used a wheelchair for long-distance walking and in crowds; 37 and 32 patients reported an improvement in the more affected arm and hand, respectively. Sixty-eight patients were able to regularly strengthened their muscles at least once a week, and 60 patients regularly stretched their legs. However, 53 patients required assistance with bathing or showering, 50 with getting dressed, and 56 with grooming or hygiene. Forty-eight patients had orthopedic surgery after SDR. Percutaneous hamstring-lengthening was the most common type of orthopedic surgery performed. Three of 13 patients who received PVR and SDR required adductor release. Six patients used medications for spasticity or dystonia. No late side effects of SDR were observed. Conclusions Our report elucidates the clinical features of spastic triplegia before and after SDR. A distinct clinical feature was the wide variation in ambulatory functions, ranging from total independent walking to wheelchair mobility. The vast majority of patients felt that SDR improved their motor functions and quality of life. PVR also resulted in favorable outcomes, with only three of 13 patients requiring additional adductor release surgery. There were no late complications related to SDR surgery.
