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ADC Online First, published on February 10, 2015 as 10.1136/archdischild-2014-306874
Review

Selective dorsal rhizotomy: an old treatment


re-emerging
Kristian Aquilina,1 David Graham,2 Neil Wimalasundera3
1
Department of Neurosurgery, ABSTRACT require multilevel orthopaedic surgery, which often
Great Ormond Street Hospital Selective dorsal rhizotomy (SDR) is a neurosurgical includes soft tissue release in combination with
for Children NHS Trust,
London, UK
technique developed to reduce spasticity and improve femoral osteotomies and hip reconstruction. Watt
2
Faculty of Medicine, University mobility in children with cerebral palsy (CP) and lower et al3 prospectively studied 74 children with spastic
of Sydney, Sydney, Australia extremity spasticity. It involves the selective division of CP and reported that 61% had already undergone
3
The Wolfson Neurodisability lumbosacral afferent (sensory) rootlets at the conus or at orthopaedic procedures by 8 years of age.
Service, Great Ormond Street the intervertebral foramina under intraoperative Moreover, spasticity can interfere with the ability
Hospital for Children NHS
Trust, London, UK neurophysiological guidance. First described in 1908, of carers to look after children with CP.
early procedures were effective at reducing spasticity but Multidisciplinary goal-oriented early intervention
Correspondence to were associated with significant morbidity. Technical promotes disease modification rather than symptom
Kristian Aquilina, Department advancements over the last two decades have reduced management and is preferable for positive long-term
of Neurosurgery, Great Ormond
Street Hospital for Children the invasiveness of the procedure, typically from a five- functional outcome. Occupational therapy, physio-
NHS Trust, Great Ormond level laminoplasty to a single-level laminotomy at the therapy and speech therapy are first-line interven-
Street, London WC1N 3JH, UK; conus. As practised today, SDR is an effective treatment tions and aim to improve activities of daily living
Kristian.aquilina@gosh.nhs.uk for young patients with bilateral spastic CP who are (ADL). Adjunct pharmacological interventions for
Received 6 October 2014
rigorously selected for surgery and for whom realistic bilateral spastic CP can be either oral (baclofen,
Revised 13 January 2015 objectives are set. SDR has therefore re-emerged as a diazepam or clonazepam) or intramuscular (botu-
Accepted 17 January 2015 valuable management option for spastic CP. In this linum toxin A or phenol). Surgical treatment
article, the authors review the single-level SDR technique options include intrathecal baclofen (ITB) or select-
and its role in the management of bilateral spastic CP, ive dorsal rhizotomy (SDR) for tone management,
with particular emphasis on patient selection and and orthopaedic surgery to improve lower limb
outcomes. alignment.

EVOLUTION OF SELECTIVE DORSAL


NATURAL HISTORY AND MANAGEMENT OF RHIZOTOMY
CEREBRAL PALSY Lumbosacral dorsal rhizotomy for spasticity was
Cerebral palsy (CP) affects approximately 2–3/1000 first advocated by Foerster in 1908. He observed
people in Europe.1 Bilateral spastic CP, with pre- that patients with tabes dorsalis who had hemiple-
dominant lower limb involvement, is the most gia did not develop spasticity, so he hypothesised
common subtype of CP in Europe, accounting for that division of the dorsal (sensory) roots could
over half of the patients.1 The severity of spastic relieve spasticity.4 Working with Tietze, his tech-
CP is classified according to the Gross Motor nique involved complete division of the dorsal
Function Classification System (GMFCS).2 The roots of L2, L3, L5 and S1, sparing the ventral
Gross Motor Function Measure (GMFM) has been (motor) roots, leading to marked improvement in
used along with GMFCS to describe the natural spasticity but also significant muscle weakness as
history of spastic CP in two landmark studies.2 In well as loss of sensation and proprioception.4
particular, the Adolescent Study of Quality of Life, Forty-five operations were conducted from 1908
Morbidity and Exercise found a decline in GMFM using Foerster’s technique; surgical complications
during the middle to late teenage years for patients were frequent, and eight patients died as a result of
at GMFCS III–V.2 Even though CP is considered a meningitis.4
non-progressive condition, a decline in motor The complications of deafferentation led to the
ability with age is evident. Interventions are direc- disuse of SDR until the 1960s when division of
ted at minimising or even halting this natural only a fraction of the dorsal rootlets, maintaining
decline. sufficient afferent input to preserve sensation and
Spasticity and associated spasms cause muscle proprioception, was considered.5 Partial sectioning
stiffness, pain and discomfort, which interfere with of the dorsal nerve roots on the basis of intraopera-
function. Muscles in children with CP are different tive electrophysiological stimulation was introduced
compared with children developing normally; they by Fasano in 1978 and is still used today. Nerve
are smaller, weaker and demonstrate histological rootlets producing sustained muscle activation with
architectural changes. Spasticity contributes to abnormal widespread involvement of unrelated
To cite: Aquilina K, muscle shortening, contracture development, muscles in the trunk and upper limbs on stimula-
Graham D,
Wimalasundera N. Arch Dis
torsion of long bones and joint degeneration. tion were assumed to be contributing to spasticity
Child Published Online First: Children with severe spasticity accumulate progres- and were divided. This led to good long-term
[please include Day Month sive lower limb muscular, skeletal and joint deform- results. The technique was adopted and popularised
Year] doi:10.1136/ ities before reaching skeletal maturity. Without by Peacock and Arens in the 1980s.5 Surgery
archdischild-2014-306874 early intervention to reduce tone, many children involved a multilevel laminectomy or laminoplasty,
Aquilina K, et al. Arch Dis Child 2015;0:1–5. doi:10.1136/archdischild-2014-306874 1
Copyright Article author (or their employer) 2015. Produced by BMJ Publishing Group Ltd (& RCPCH) under licence.
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Review

typically from L1 to L5. The nerve roots were identified at their amplitude (figure 2). Electromyographic (EMG) responses to
individual foramina. The sensory nerve roots were dissected 50 Hz tetanic stimulation at threshold amplitude are graded for
from the motor ones at the foramen; the afferent roots were each nerve rootlet (figure 3). Rootlets with diffuse responses
then further dissected into rootlets, evaluated electrophysiologi- beyond their segment (grade 3 and 4) are divided; in total, up to
cally and divided. However, concerns about the potential 60–70% of the sensory roots are divided. Fifty per cent of the L1
impact of extensive spinal surgery in ambulant children with CP sensory nerve root is then divided as it exits its foramen.
led to the development of a single-level technique involving div- Recovery following the single-level technique is rapid, and chil-
ision of dorsal rootlets at the level of the conus.6 dren typically resume physiotherapy after 2–3 days of bed rest.
This single-level technique, as it is often practised today,
involves identification of the conus on intraoperative ultrasonog- PATIENT SELECTION FOR SELECTIVE DORSAL RHIZOTOMY
raphy through a small T12–L1 midline fenestration (figure 1). The decision as to whether SDR is the optimal procedure for a
The conus is then exposed by removing the spinous process and particular child at that stage of motor development is not easy
central component of the appropriate lamina. At this level, and needs to be made within a multidisciplinary context.
removal of a single lamina allows exposure of the dorsal Agreement by all involved, including carers, on the goals of
(sensory) and ventral (motor) nerve roots, from L1 to the sacral treatment for the individual child is crucial. The principal goals
roots, as they enter and leave the conus. The dorsal (sensory) of SDR depend on a child’s motor abilities and include
nerve roots are located superficially and the ventral (motor) ones improved motor function, increased mobility and independence,
lie deeper ; there is a clear identifiable plane between the two. improvement in ease of care and reduction in pain.
The motor roots are protected throughout the procedure. The The general selection criteria defined by Peacock in 1987 still
L2 to S1 dorsal nerve roots are identified, divided into rootlets apply.5 Our current criteria are shown in table 1. Currently, most
and systematically stimulated to determine their threshold children selected for SDR are between 3 and 14 years of age.
As illustrated in a recent review, selection criteria for SDR
vary between centres and have not been generally validated.7
They are primarily based on clinical rationale rather than clinical
evidence. In many centres, several inclusion and exclusion cri-
teria are not based on standardised or reproducible measure-
ments. Most centres, however, use a multidisciplinary approach
that evaluates some or all of the International Classification of
Function domains, particularly the body structure and function,
activity and personal and environmental factors. Very strict
selection criteria may influence outcome. In the Oswestry series,
only 35% of referred children satisfied the selection criteria and
underwent SDR.8 In this series, children consistently showed
improvement in the GMFCS level after SDR; this was not
reproduced in other large series, where children tended to
improve only within the same GMFCS level. It is important to
remember that as children with CP get older their mobility diffi-
culties include more orthopaedic-type musculoskeletal difficul-
ties rather than just spasticity, which can explain differences in
outcome post-SDR. Although a trial of oral baclofen is not
necessary prior to making a decision on SDR, targeted botu-
linum toxin injections to reduce spasticity in some muscle
groups are useful in evaluating the potential functional impact
of SDR.

OUTCOMES OF SELECTIVE DORSAL RHIZOTOMY


SDR is a permanent and effective treatment for spasticity in
children with bilateral spastic CP.9 Several studies show there is
no loss of gross motor control in patients with bilateral spastic
CP, with most patients showing sustained improvement in gait
and spasticity.5 8 10–14 There is high-level evidence that SDR
combined with physiotherapy has better results than physiother-
apy alone, although patients are unlikely to decrease GMFCS
grade.9 14–17 This conclusion is largely based on the results of
three well-designed randomised controlled trials (RCTs) con-
ducted in North America (Toronto, Vancouver and Seattle) in
Figure 1 Intraoperative ultrasound is essential to locate the conus 1997 and 1998.15–17 In the Toronto study,15 evaluation at
intraoperatively in the single-level procedure. These ultrasound images 12 months showed significant improvements in GMFM scores,
demonstrate (A) sagittal view defining the triangular shape of the
knee and ankle tone, passive ankle range of motion, soleus
conus (white arrow); thin parallel lines around the conus represent the
nerve roots that constitute the cauda equina within the hypoechoic EMG reflex activity on forced dorsiflexion and foot-floor
cerebrospinal fluid. (B) An axial view showing the circular hypoechoic contact pattern. In the Vancouver trial,16 significant improve-
conus; the white arrow defines the boundary between the dorsal ments were observed at 1 year in GMFM, spasticity and range
sensory nerve roots (single asterisk) and the ventral motor roots of movement in the group undergoing SDR combined with
(double asterisks). physiotherapy. At 24 months in the Seattle study,17 the
2 Aquilina K, et al. Arch Dis Child 2015;0:1–5. doi:10.1136/archdischild-2014-306874
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Review

Figure 2 Intraoperative photomicrographs demonstrating intradural exposure at the level of the conus, typically through an L1 laminotomy in the
single-level procedure. (A) Following a midline longitudinal durotomy, the afferent nerve roots on the right (white arrow) and the conus (asterisk)
are evident. (B) The afferent nerve roots, from L2 to S1, are mobilised medially to expose the ventral motor roots (asterisk). (C) A small protective
cottonoid is placed over the motor roots (asterisk). (D) A sling is passed deep to the sensory nerve roots, and Peacock probes are used to stimulate
the roots systematically (white arrow) from lateral, corresponding to L2, to medial, corresponding to S1.

Figure 3 Intraoperative electromyography showing responses to tetanic stimulation of individual nerve roots to 50 Hz stimulated at their own
threshold. In (A), the response is restricted to a single muscle group on stimulation of S1 (hamstrings) (grade I). In (B), a response from an adjacent
muscle group on stimulation of L3 is recorded (vastus lateralis; adductor longus stimulated also) (grade II). In (C), there is diffuse activation of
several muscle groups on the same side on stimulation of S1 (hamstrings) (grade III). In (D), there is a diffuse and bilateral muscle group response
on stimulation of L5 on the left ( peroneus longus) (grade IV). The objective of the procedure is to divide 60–70% of the sensory nerve roots
between L2 and S1, including as many grade III and grade IV roots as possible. Also, 50% of the L1 sensory root is subsequently divided at its
intervertebral foramen.
Aquilina K, et al. Arch Dis Child 2015;0:1–5. doi:10.1136/archdischild-2014-306874 3
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Review

Dudley et al10 reviewed long-term follow-up data of children


Table 1 General selection criteria for selective dorsal rhizotomy
who were evaluated by a multidisciplinary team preoperatively
Type of CP Spastic without significant ataxia or dystonia and at 1, 5, 10 and 15 years after SDR. After SDR, through
Classically spastic diplegic CP adolescence and early adulthood, statistically significant and
Severity of CP A degree of ambulation with or without assistive devices; durable improvements in lower limb muscle tone, gross motor
currently most patients are GMFCS grade II or III
function and performance of ADL were identified. Only 28% of
MRI No injury to basal ganglia, brainstem or cerebellum on
MRI; typical periventricular leucomalacia
children required further lower extremity orthopaedic surgery
Musculoskeletal Good trunk control, good lower extremity antigravity
after SDR during the follow-up period, a significant improve-
strength on clinical examination ment compared with the expected 61% intervention rate for
No significant femoral head subluxation on pelvic similar children by the age of 8 years.3 The multidimensional
radiograph (Riemer’s index up to, or less than, 40%) benefits of SDR, reflected in the PEDI score, were also evident
No significant scoliosis
in this study, where significant gains in self-care and mobility
Previous Preferably at least 6 months after the last botulinum toxin
persisted to early adulthood. However, a recent systematic
interventions A injection
Preferably at least 1 year after orthopaedic surgery review of interventions for CP found no clear evidence that
Child and family Ability to cope with rehabilitation process (cognitive and SDR improves general activities and participation, which argu-
factors emotional) ably should be the primary goal of the procedure.9 Although
Supporting environment with access to rehabilitation there are enduring improvements in GMFM after SDR, most
facilities children will remain within their GMFCS grade following
CP, cerebral palsy; GMFCS, Gross Motor Function Classification System. surgery, with the exception of the Oswestry experience.
Nevertheless, the majority of former patients with SDR report
improvements in their ADL and that they would recommend
combined SDR/physiotherapy group showed a significant reduc- SDR, with very few reporting negative impressions of the pro-
tion in spasticity compared with the physiotherapy-only group. cedure.21 Indeed, the Cape Town experience found that none of
However, improvements in GMFM were not significant. This Peacock’s original cohort of patients required help with ADL.5
result could be attributed to the fact that only a mean of 25% Some studies have reported less satisfactory long-term out-
of dorsal roots were divided in the Seattle study, which is in comes, thus contributing to decisions on patient selection.
contrast to the currently accepted SDR technique, in which Children with spastic quadriplegia had poorer outcomes com-
between 50% and 70% of the sensory nerve roots are divided. pared with those with diplegia.23 Children over 10 years of age
Other studies have shown SDR to be superior to other inter- were demonstrated to have better long-term outcomes with
ventions in the management of spastic CP. In their RCT compar- multilevel orthopaedic surgery than with SDR.24 This underlines
ing SDR with botulinum toxin A injections, Wong et al18 found the particular challenges related to patient selection in this age
that the effectiveness of SDR was of a longer duration than group, where the primary difficulties with mobility often arise
botulinum toxin A. Moreover, patients with SDR have reduced from weakness and structural lower limb deformities rather than
requirements for orthopaedic interventions and intramuscular pure spasticity. It is now generally agreed that good long-term
botulinum toxin A injections.10 18–21 In their non-randomised results are achieved in young children, who are diplegic rather
patient series, Kan et al compared two groups of age and than quadriplegic, and those whose GMFCS grade is II–III. A
GMFCS score-matched children; an SDR group underwent recent study of 54 children followed up for 2 years confirmed
surgery prior to 1997, and an ITB group underwent surgery that children between 4 and 7 years old with preoperative
after 1997. The SDR group showed significantly better improve- GMFM scores between 65% and 85% benefit most from SDR.13
ments in Ashworth scale, lower extremity passive range of Permanent complications are now rare after SDR. In their
motion and GMFM scores.22 The SDR group had a significantly review of long-term adverse effects of SDR, Grunt et al25
reduced need for orthopaedic surgery compared with the ITB reported that back pain and spinal abnormalities were common,
group. A study evaluating the rate of orthopaedic surgery after including kyphosis, scoliosis, lumbar lordosis, spondylosis and
SDR showed that in all age groups 25% of independent walkers spondylolisthesis. But they found insufficient evidence to con-
and 44% of assisted walkers required orthopaedic surgery over clude such abnormalities are the direct result of SDR rather
a 9-year follow-up.19 Those undergoing SDR at a young age than related to the natural history of spastic CP. A large patient
demonstrated the lowest requirement for orthopaedic surgery series has shown that limited laminectomies at the level of the
after SDR.21 conus are not associated with long-term spinal deformity.6
Evidence on the long-term effects of SDR is emerging and is Transient dysaesthesiae are common, but permanent hypoesthe-
generally positive.5 10–12 Nordmark et al11 reported on a group sia is rare. Transient urinary retention was frequent during the
of 35 children with spastic diplegia over a 5-year postoperative earlier days of the resurgence of SDR, while permanent urinary
period. SDR resulted in immediate reduction of tone in adduc- incontinence was rare. Most centres now advocate pudendal
tors, hamstrings and dorsiflexors, with no recurrence of spasti- monitoring and limitation of the division of the S2 nerve root
city over 5 years. Similarly, there was significant improvement in in order to limit adverse effects related to detrusor function. As
passive range of movement in hip, knee and ankle joints, as well a result, the current risk of incontinence is very low.
as significant improvements in GMFM. In a 10-year follow-up In practice, the importance of setting appropriate specific
study of 24 children, undertaken by the same group, additional objectives with families, within the context of the surgical risks
improvement was evident in the functional skills, mobility and and the need for intensive postoperative rehabilitation, cannot
caregiver assistance (both self-care and mobility) domains of the be overemphasised. In our experience, in ambulant children,
Paediatric Evaluation of Disability Inventory (PEDI) scores SDR leads to improved mobility, increased stamina, better
between 5 and 10 years after SDR.12 This was particularly balance and fewer falls. Children who walk with assistance
evident in children in the GMFCS I to III subgroups. Children become more independent. Sitting and standing posture
in the GMFCS IV and V subgroups demonstrated only small improves. In addition, the pain associated with spasticity
changes between 5 and 10 years after SDR. responds well to SDR.
4 Aquilina K, et al. Arch Dis Child 2015;0:1–5. doi:10.1136/archdischild-2014-306874
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Review

CONCLUSIONS 9 Novak I, McIntyre S, Morgan C, et al. A systematic review of interventions for


SDR is an effective treatment for young patients who are rigor- cerebral palsy: state of the evidence. Dev Med Child Neurol 2013;5:885–910.
10 Dudley RW, Parolin M, Gagnon B, et al. Long-term functional benefits of selective
ously assessed for suitability. The evidence supports the use of dorsal rhizotomy for spastic cerebral palsy. J Neurosurg Pediatr 2013;12:142–50.
SDR in the management of spastic CP by significantly reducing 11 Nordmark E, Josenby AL, Lagergren J, et al. Long-term outcomes five years after
spasticity. This reduces need for further surgical interventions selective dorsal rhizotomy. BMC Pediatr 2008;8:54.
and improves independence in ADL and quality of life. The 12 Josenby AL, Wagner P, Jarnlo G, et al. Functional performance in self-care and
mobility after selective dorsal rhizotomy: a 10-year practice-based follow-up study.
current single-level technique, involving midline laminotomy at
Dev Med Child Neurol 2014; Published Online First: 30 Oct 2014. doi:10.1111/
the conus, represents a significant improvement on older techni- dmcn.12610
ques. Nevertheless, realistic goals must be set as GMFCS levels 13 Funk JF, Panethen A, Sinan Bakir M, et al. Predictors for the benefit of selective
are unlikely to advance, which is a limitation that must be dorsal rhizotomy. Res Dev Disabilities 2015;37:127–34.
clearly understood by patients, their parents and practitioners. 14 McLaughlin J, Bjornson K, Temkin N, et al. Selective dorsal rhizotomy: meta-analysis
of three randomized controlled trials. Dev Med Child Neurol 2002;44:17–25.
Acknowledgements The authors are indebted to Ms Ivana Jankovic and Dr 15 Wright FV, Shell EM, Drake JM, et al. Evaluation of selective dorsal rhizotomy for
Matthew Pitt for assistance with provision of figures related to intraoperative the reduction of spasticity in cerebral palsy: a randomized controlled trial. Dev Med
electromyography. Child Neurol 1998;40:239–47.
16 Steinbok P, Reiner AM, Beauchamp R, et al. A randomized clinical trial to compare
Contributors All authors contributed equally to the drafting and revision of the selective posterior rhizotomy plus physiotherapy with physiotherapy alone in children
manuscript. with spastic diplegic cerebral palsy. Dev Med Child Neurol 1997;39:178–84.
Competing interests None. 17 McLaughlin JF, Bjornson KF, Astley SJ, et al. Selective dorsal rhizotomy: efficacy and
safety in an investigator-masked randomized clinical trial. Dev Med Child Neurol
Provenance and peer review Commissioned; externally peer reviewed. 1998;40:220–32.
Data sharing statement This is a commissioned review article. 18 Wong AM, Pei YC, Lui TN, et al. Comparison between botulinum toxin type A
injection and selective posterior rhizotomy in improving gait performance in children
with cerebral palsy. J Neurosurg 2005;102:385–9.
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Aquilina K, et al. Arch Dis Child 2015;0:1–5. doi:10.1136/archdischild-2014-306874 5


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Selective dorsal rhizotomy: an old treatment


re-emerging
Kristian Aquilina, David Graham and Neil Wimalasundera

Arch Dis Child published online February 10, 2015

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