The secondary complications of cerebral palsy (CP) present unique management challenges for the individual, the family, and the medical team.1 These complications are typically progressive in nature and may present with atypical tone, muscle contractures or over lengthening, muscle imbalances across joints, bony torsions (tibial, femoral, calcaneal), dysplasias (femoral, acetabular), and ligamentous laxity. The natural progression of a child with CP-spastic diplegia commonly includes gradual loss of the ability to extend the hip and knee in stance and during gait, also known as crouched posture or crouched gait, respectively.1–5 Severe crouch gait has been described by Rodda et al2 as knee flexion greater than 30°, dorsiflexion greater than 15° and hip extension less than 3° at late stance. Progression of this pathological gait pattern may trigger a cascade of additional impairments of body structures, particularly at the knee. As knee flexion and patella alta increase, the effectiveness of the knee extensor mechanism declines, which often leads to increasing knee pain, muscle fatigue, and reliance upon assistive devices for mobility.2 Hamstring and posterior capsule contractures, ligamentous laxity of the cruciate ligaments, and overlengthening of the patellar tendon contribute to passive insufficiency of the quadriceps.1,3,6 Hip flexion and internal rotation may serve the role of countering knee flexion or providing greater stability at the hip joint in the case of hip dysplasia.3,7 Walking in a plantarflexed position may provide improved ankle and knee stability during stance and gait.3,7 Over time, the crouched position may contribute to the development of muscle contractures, bony torsions, pain, and limitations in activity participation.1,7
A continuum of management strategies is available to address these secondary complications.8 Conservative management may include bracing,6–8 serial casting,6,8,9 and physical therapy (PT) interventions such as muscle stretching6–8 and strengthening.10,11 Medical management may include the use of oral or intrathecal medications,8,12 botox injections,8 and alcohol blocks.8,13 Once the benefits of these conservative management techniques have been exhausted, more invasive intervention is often considered. Soft tissue and bony modifications are common surgical interventions.3,7–8,14
OVERVIEW OF SURGICAL PROCEDURES
Soft tissue modifications may include muscle and tendon lengthening, shortening, or transfer. Lengthening procedures commonly involve a series of cuts through the muscle or tendon, passive movement of the joint to achieve functional length of the muscle-tendon unit, and securing the soft tissues at the new length with stitches. Casting during the healing process is common and weight-bearing restrictions vary.7,15,16 A gastrocnemius lengthening, for example, may require 4 to 6 weeks of immobilization in a cast. A minimally invasive method of lengthening muscle and fascial fibers may be used by some surgeons. This method, “selective percutaneous myofascial lengthening” (PERCs),17 may include a short (3 days for knees; 3-4 weeks for ankle) period of immobilization of the joint crossed by the target muscle. On the same day as the procedure, the individual is instructed to ambulate with full weight bearing on the operative lower extremity (LE).
Patella alta, one of the components of crouched gait, may be managed surgically with extensor mechanism shortening, also known as patellar tendon advancement. In this procedure, the tendon is detached from the distal pole of the patella, shortened to the proper length and reconstructed using transosseous sutures to the patella. A wire loop passed through the patella and the proximal tibia can be used to protect the repair, while healing (Figure 1). The goal is to position the distal pole of the patella at the tibiofemoral joint line with the knee fully extended. Some authors report that a period of immobilization (typically 4-8 weeks) of the joint crossed by the modified soft tissue is required following the procedure,6,7,15,16 while others advocate early mobility.2 Bony modifications such as osteotomy and fusion may be necessary to correct malpositioning and to provide a stable and functional joint from a biomechanical and arthrokinematic perspective. Poor femoroacetabular congruency may be modified with a number of bony surgical procedures. Recent literature reports excellent results following periacetabular osteotomy (PAO).18 This procedure involves a series of cuts through the pelvis around the periphery of the acetabulum. Once detached, the acetabulum is reoriented and then fixated with screws in a position that provides optimal surface contact between the acetabulum and the femur (Figure 2). Bony torsions of the femur are typically managed with a femoral derotation osteotomy (FDO).3,19–20 Three of the most common osteotomy sites in FDO are at the intertrochanteric line,14,19 distal (D) to the greater trochanter (subtrochanteric),20 or immediately proximal (P) to the femoral condyles (supracondylar).14,19 Following these procedures, the lower limb is externally rotated to neutral19 and fixated with pins19 plates14,20 or screws20 in this position (Figure 1). Immobilization ranging from 6 to 12 weeks, and in many cases a period of nonweight bearing, is required while the bone heals in the new position.14,19–20 Recent trends suggest better functional outcomes if a combination of soft tissue and bony surgical procedures are performed simultaneously, that is, single-event multiple-level surgery (SEMLS) rather than in isolation.2,3,21 SEMLS have become increasingly common for the management of secondary complications in individuals with CP.2,3,21
The outcome of SEMLS has been reported by several authors. Strength at 6 months following SEMLS has been reported to be significantly less than prior to surgery, although gait kinematics were improved,22 and still less than preoperative levels at 12 months.23 Rehabilitation of longer duration may be necessary to achieve optimal strength.23 A minimum of 1 year and more likely 18 to 24 months may be needed to return to preoperative functional levels.24 Long-term management following SEMLS has received little attention within the PT literature. The purpose of this case report is to describe the surgical interventions and the long-term PT management following SEMLS in a 13-year-old adolescent (S.R.) with CP diplegia. It was hypothesized that S.R. would exceed his preoperative levels of strength, mobility, and function 18 months following SEMLS with rehabilitation provided by PT.
DESCRIPTION OF THE CASE
S.R. was a 13-year-old adolescent, diagnosed with CP diplegia, “mixed motor” type (spasticity of the left LE and hyptonia of the trunk and right LE),25 and functioning at Gross Motor Function Classification System (GMFCS) level II prior to the SEMLS. He was an honor student and attended his neighborhood middle school (eighth grade) prior to the surgery. He lived with his biological parents and 2 younger sisters in a midwestern middle-class suburban neighborhood. Both parents worked outside the home; the mother in health care, and the father in government service.
The abilities that are expected for a 12-year-old at GMFCS level II include walking without an assistive device with limitations in walking outdoors and in the community.26 S.R. was independent ambulating with bilateral ankle foot orthoses, although distance was steadily declining. He was independent walking with a quad cane but preferred walking with one hand on a wall for stability when changing classes at school. A wheelchair was used in community situations that required walking long distances or long periods of time (eg, large shopping malls and amusement parks). See Table 1 for preoperative GMFM-66 scores. S.R.'s ability to participate in leisure activities was severely limited. Because of a significant decline in gait endurance, speed and efficiency, and finally, a patellar dislocation while running to a base, he stopped playing a modified version of baseball with other children with physical impairments. He enjoyed playing catch or basketball with family and friends at home.
Impairments in Body Structures
S.R. was experiencing frequent falls with multiple knee injuries, and knee pain requiring pain medication (acetaminophen or ibuprofen) daily. His standing posture was “crouched,” including excessive hip flexion, adduction and internal rotation, knee flexion, and ankle dorsiflexion bilaterally (left greater than right). The left midfoot was excessively mobile and pronated in weight bearing. Both patellae were resting above the proximal end of the trochlear groove of the femur (patella alta). The right patella was unstable, dislocated/relocated once, and subluxed on several occasions. Ligamentous integrity testing revealed multiplanar instability in both knees; the left knee was more lax than the right. Lower extremity strength prior to surgery was as follows: hip muscles ranged from 2+ to 3+, quadriceps 2+, hamstrings 3+, and ankles 1+ to 2+. Range of motion (ROM) was limited actively (−45°) and passively (−10°) in extension in both knees and within normal limits passively (but limited actively by 50%) in both ankles in all planes of motion. Although muscle tone was generally low, the left hip adductors were 2+ and gastrocsoleus 1+ on the Modified Ashworth Scale.27 Upper extremity (UE) strength was 4 to 4+ and the trunk was 3+ in both flexors and extensors.
The interventions used to manage secondary conditions prior to SEMLS are presented in Table 2. One bony modification, a left proximal (LP) FDO, was done at 9 years of age to correct internal torsion of the femur and to reduce the hip adduction/internal rotation component of the crouched posture. In spite of conservative care interventions following the LPFDO, the left hip returned to a position of internal rotation during gait and the crouched posture returned within 12 months. A second pediatric orthopedic surgeon was consulted and his recommendation was SEMLS that included a left PAO, bilateral DFDOs, bilateral patellar tendon advancements, removal of hardware from the previous proximal LPFDO, and fusion of the left calcaneocuboid joint. After extensive discussion and planning, S.R. and his family decided to proceed with the surgery. His maternal grandparents planned to live with the family for the first year following surgery to assist as needed. An extended network of family and friends living in the same community would also be available to provide support throughout the rehabilitation process. The family goals for the surgery were to decrease knee pain and medication use, walk without devices, decrease and/or eliminate falls, and increase pain-free participation in family and school activities.
Five outcome measures were selected for this case: the Gross Motor Function Measure-66 (GMFM-66), goniometry for ROM measurements, manual muscle testing (MMT), the Activity Scale for Kidsperformance38 (ASKp38), and the Numerical Pain Rating Scale (NPRS). The first 3 outcome measures were administered prior to surgery and postoperatively (postop) at 1-month intervals for 12 months, and at 18, 24, and 30 months by the same physical therapist. The ASKp38 was completed by SR once monthly for 12 months postop. The NPRS was used prior to surgery and at every treatment postop for the first 2 months. After the 2-month point, the NPRS was used each time SR indicated discomfort verbally, by facial expression and/or through body language. NPRS scores guided the therapist in the selection and intensity of interventions, and the parents in the administration of medication.
The GMFM-66 and the ASKp38 assess activity level. The GMFM-66, a functional outcome measure with item scores based on intervals, was used to evaluate gross motor function in 5 dimensions (Lying and Rolling; Sitting; Crawling and Kneeling; Standing; and Walking, Running and Jumping). The GMFM-66 has been reported to have excellent test-retest reliability, good intrarater reliability,28,29 interrater reliability,29,30 and validity.28,30–32 The minimum clinically important difference (MCID) for the GMFM-66 was determined by Oeffinger et al33 for GMFCS level II as 1.0 for a medium effect size and 1.5 for a large effect size.
The ASKp38, a self-report tool for children developed by Young et al,34 includes 38 questions regarding the child's perception of his or her ability to perform activities. The questions are divided into 9 domains, which include both self-care activities and gross motor abilities. The ASKp38 has been reported to have excellent interrater and intrarater reliability,34,35 test-retest reliability,36 and good criterion and construct validity. A clinically significant change is reported to be 1.73 SD units.34
The NPRS, goniometry, and muscle strength testing quantify impairments of body structures. The NPRS is a 0- to 10-point scale with the anchor of “no pain” for zero and “greatest pain imaginable” for 10. This scale has been reported to be valid and reliable.37 Childs et al38 found that a 2-point change in the NPRS demonstrates an MCID in patients with LBP.
Goniometry has been reported to be a valid measurement method.39–41 Reliability in subjects with hypertonicity has been studied by Ashton et al42 and Stuberg et al.43 Ashton et al found unacceptable intrarater reliability, and 10 years later, Stuberg et al found good intrarater reliability. They also found that interrater reliability varied significantly. To maximize reliability of the measurement process in this case report, a single rater used a consistent measurement protocol for all measures. Isokinetic strength testing has been found to have good intrarater reliability when used to assess children with CP.44,45 Overall, intrarater and interrater reliability for MMT in a population of children with Duchene Muscular Dystrophy was determined to be excellent in a study by Florence et al.46 Although hand-held dynamometers are more reliable for quantifying muscle strength, one was not available for use in this case report.
Surgery and Hospital Stay
The SEMLS, a 7-hour procedure, was performed on May 9, 2006. The left foot was placed in a cast immediately following the surgery. Adult hardware was used for the bilateral DFDOs (Figure 1) and PAO (Figure 2). Wires were anchored to the patella and the tibial tuberosity to limit cranial movement of the patella, therefore protecting the patellar tendon repair while healing (Figure 1).
S.R. received inpatient PT services days 1 through 5 postop with a primary focus on sitting, transfer training, and bed mobility. He sat at the edge of the bed and dangled his legs with maximum assistance of the physical therapist and his mother on the first postoperative day and transferred to a wheelchair with maximum assistance of 2 on the 2nd postoperative day. S.R. spent all 5 days of his hospital stay in the pediatric intensive care unit to allow management of tachycardia, which was believed to be a response to pain. S.R. was discharged to the care of his family once his heart rate was stabilized on the 5th postoperative day. Discharge instructions included (1) nonweight bearing on the left foot until cast removal at 6 weeks postop and (2) positioning and mobility as tolerated on the right LE and left hip and knee. The equipment needed for home was determined by a hospital discharge coordinator and the parents and included a hospital bed, wheelchair, and standard walker.
PT EXAMINATION AND INTERVENTION
PT Practice Patterns
Two practice patterns from the “Guide to Physical Therapy Practice” apply in this case.47 On the basis of his medical diagnosis of CP, S.R. fits Neuromuscular Practice Pattern C-Impaired Motor Function and Sensory Integrity Associated with Nonprogressive Disorders of the Central Nervous System-Congenital Origin. He presents with impairments in muscle strength and tone, ROM, gait distance and endurance, pain, and secondary changes in bone and ligament. Following SEMLS, he also fits “Musculoskeletal Pattern I–Impaired Joint Mobility: Motor Function, Muscle Performance and Range of Motion Associated with Bony or Soft Tissue Surgery,” following the SEMLS. As a result of the surgery, S.R. experienced further decreases in ROM, strength and endurance, impaired joint mobility, limitations in activities of daily living, and pain.
PT Plan of Care: Prognosis
Based upon physician input, knowledge of S.R.'s response to treatment in previous courses of PT, and family support, the treating physical therapist believed that S.R. had good potential to achieve preoperative GMFM-66 levels by 18 months after SEMLS. It was also expected that given improved biomechanical alignment, S.R. had good potential to exceed his preoperative GMFM-66 levels after 18 months. Long-term goals to be achieved beyond 18 months for impairments of body structures included the following: achieve a minimum of 3+/5 strength of both quadriceps and hip abductors, 4/5 in all other hip and knee muscles, and 5/5 in all UE muscle groups. Participation goals included the following: ambulate independently without assistive devices within the home; ambulate independently using a cane or Lofstrand crutch(es) within the community; perform all transfers independently; and transition to a community fitness facility for strength training.
Frequency of PT Services
The frequency of PT intervention can be described using the “frequency mode” descriptions reported by Bailes et al.48 Home-based PT began on the seventh postoperative day and continued through the third postoperative month at the “Intensive Frequency Mode,” in this case, 3 times per week. During this time period, modifications in his plan of care were necessary at each PT visit. When S.R. returned to school in the fourth postoperative month, PT services were transferred to the outpatient setting and the PT frequency decreased to 2 times per week, that is, the “Weekly/Biweekly Frequency Mode.” The duration of each visit varied between 45 and 60 minutes, based upon S.R.'s tolerance during each visit. In addition, he received “Consultative Frequency Mode” PT services in his school. The school physical therapist provided episodic consultation as needed to facilitate S.R.'s independence within school activities. The outpatient and school physical therapists consulted by phone as needed. PT intervention was interrupted by several 1 to 2 week breaks for vacation or other family events but was otherwise continuous through the 30 months of this case report. From months 3 to 30, S.R. demonstrated continuous measureable progress in achieving established goals. From months 31 to 34 the “Consultative Frequency Mode” was used in the outpatient setting, as the transition to home/community use of fitness equipment and community leisure activities was established. The ICD-9 codes used during this period varied on the basis of the primary focus of the intervention: knee, hip, lumbar, and gait diagnoses were used.
PT Goals and Intervention
For the purposes of this case report, the PT episode of care following SEMLS has been divided into sections based upon changes in weight bearing, mobility restrictions, impairments, and functional activities. During the “Intensive Frequency Mode” and the “Weekly/biweekly Frequency Modes” of care, each intervention included both strength training and task-specific training activities. Current evidence for strength training in individuals with CP supports the necessity for including both components in rehabilitation process49 and specifically following SEMLS.23,50 Each intervention was structured using the “circuit training” concept. The “circuit” included a predetermined sequence of exercises, alternating between UE, LE, trunk, and task-specific exercises with minimal rest periods (generally less than 1 minute) between each station.51 One region of the body rests while another region is working, which in theory minimizes individual muscle fatigue. The initial number of repetitions was defined by the maximum number of repetitions that could be completed without compromising technique without resistance. Generally, SR started with 2 sets of 10 repetitions and progressed to 3 sets of 30 repetitions, before adding/increasing resistance. Resistance was initially provided by gravity and then progressed to resistance bands, free weights, and finally machines. A summary of key impairments in body structures, interventions used, and outcomes for activity performance used during each time period is presented in Table 3.
TABLE 3-a Summary of...Image Tools
Month 1: Goals included the following: minimize harm to the healing tissues during PT intervention; minimize pain produced during PT intervention; preserve muscle length and joint integrity; maximize strength of trunk, UEs, and LEs; assist SR and family to establish safe and effective bed mobility and transfers, and independence with the use of all equipment; increase endurance and tolerance to specific exercise and activity, and patient/family will be independent in performing/assisting with home exercises. PT intervention: The primary focus was on active ROM (AROM), strength training, and bed mobility. Pain medication was timed carefully with each intervention.
TABLE 3-b Summary of...Image Tools
Months 2 to 4: Goals were modified to achieve the strength, ROM, and endurance needed for functional activities: independent bed to/from wheelchair and walker transfers at home, and power scooter to/from the classroom desk and toilet in school; independent ambulation within the home and classroom. PT intervention: Preparation for return to school at the end of month 4 was the primary focus during this time period.
Months 5 to 9: Goals were primarily participation directed: increase gait distance and endurance, and independent transfers. PT intervention: Strength training, gait training, and transfers were the primary focus. Knee pain and limitations in left knee extension during this time period necessitated modification of intervention strategies. Endurance changes were measured on the basis of time and/or repetitions completed prior to a change in quality. School and outpatient PT services were coordinated by phone.
Months 10 to 12: Goals of increasing gait distance and endurance and advancing transfer training and strength training continued. PT intervention required modifications due to a new onset of low back pain with referral into the left shin. Evaluation revealed hypermobility and provocation of symptoms at lumbar levels 4 and 5. For approximately 2 weeks, the focus of intervention shifted to management of back symptoms. Goals during this period included the following: (1) eliminate low back pain; (2) achieve independence in symptom management strategies and safe body mechanics and movement patterns; and (3) maximize strength of lumbar stabilizing musculature.
Months 13 to 18: Goals: Strength and endurance training continued in preparation for transition from walker to crutches, and ascending stairs on his feet (rather than his buttocks). PT interventions were structured with the intent of minimizing patellofemoral compression and overstretching of the patellar tendon continued to be the goal in all therapeutic exercises chosen.52
Months 19 to 24: Goals included the following: maintain full passive/active left knee extension; maximize strength of left hip abductors and extensors and left quadriceps; advance high-level gait-and-balance activities; and transition to the use of traditional weight training equipment within the home and/or a fitness facility. PT intervention was briefly interrupted in month 21 when PERCs on the left distal hamstrings and left short adductors, and a left nerve obturator alcohol block (outpatient procedures) were performed by the same surgeon who performed the SEMLS. Walking began within hours of the procedure, a soft knee immobilizer was used for 3 days (24 h/d), followed by night use for several days. The soft knee immobilizer was then replaced with an Ultraflex knee brace while sleeping. Immediately after PERCs and alcohol block, adductor spasms were eliminated and full passive left knee extension was achieved. PT intervention resumed without restrictions within 1 week and included circuit training with strength and task-specific training.
Months 25 to 34: Goals: During this time period, goals shifted to a new focus: achieving new motor skills (10 seconds of single leg stance, ascend/descend stairs alternating steps without the use of a device or hand railings, jump upward and forward, and walk safely without devices); preventing the return of the preoperative crouch position; and education regarding future management of impairments. PT intervention: The whole team discussed gait device use and knee joint protection during S.R.'s life span. S.R. was able to walk without a device and sincerely enjoyed the freedom of doing so. Yet, he recognized that gait quality declined with fatigue. Long-term knee joint protection was a concern of the physical therapist and surgeon. After much discussion, S.R.'s decision was to walk without devices in his home and use a standard cane outside of his home. The possibility of using the Lofstrand crutches for outings requiring longer walks such as a trip to the zoo or theme parks, and in the event of knee pain, was also discussed as an option.
Month 35: S.R. was discharged from PT for the first time in his life (Table 1). Extensive discharge planning was completed including (1) discussions regarding thoughtful selection of gait devices, (2) written guidelines for management of episodes of back and/or knee pain, and (3) a comprehensive written home exercise program based on the exercise equipment in S.R.'s home and typical equipment that is available in community fitness centers. The home exercise program included general strengthening that focused primarily on antigravity muscle groups and select muscle stretches.
DESCRIPTION OF OUTCOMES
GMFM-66 scores are presented in Table 1. All GMFM scores fall within the 95% confidence intervals, indicating that S.R.'s true score is highly likely to fall within the upper and lower bounds of the confidence interval. Based upon the MCID as determined by Oeffinger et al,33 the change in GMFM-66 scores from preoperation to 18 months postop of 0.88 points did not reach significance. The change from 18 to 24 months was 2.29 points, exceeding the MCID of 1.5 for a large effect size. The change from 24 to 30 months was 18.89 points, also achieving a large effect size. At month 1 following the SEMLS, S.R. was able to perform only 13 of the items of the test because of pain, nonweight bearing status on his left foot and ankle, and decreased strength and ROM in the trunk and bilaterally in the UEs and LEs. The inability to bear weight on his knees affected GMFM-66 scores (dimensions C and D) until month 10, when he was able to attempt a few criteria, and month 12 when he could perform all criteria. S.R.'s score increased from 59.86 to 71.69 (11.83 point change—exceeding the MCID for a large effect size) between months 10 and 18 when strength and endurance training were the key foci. Although strength and endurance training continued between months 18 and 30, the primary focus was new skill acquisition. Between months 24 and 30, GMFM-66 scores changed from 71.69 to 89.70 (18.01 points) with changes primarily occurring in dimensions D and E.
Participant Perception of Abilities
The 9 domains of the ASKp38 were grouped into 2 categories: (1) self-care (dressing, eating/drinking, and personal care) and (2) gross motor abilities (transfers, standing, stairs, and locomotion) (Table 1). S.R.'s self-care scores had reached the ceiling by 4 months after surgery. The gross motor abilities score displayed a gradual upward trend in the first year with 2 declines. The first correlated with the transition from a walker to Lofstrand crutches for home use and the second correlated with progression from a walker to Lofstrand crutches for community use. S.R. did not achieve all of the gross motor abilities included in the ASKp38 because some questions relate to participation in team sports, which S.R. did not choose to do.
ROM and Strength
AROM reached preoperative levels in all joints and movement planes except left hip flexion and extension and right hip extension within 6 weeks. Preoperative strength was achieved in the right LE within 7 months (see Table 4) and in the left LE within 11 months (see Table 5). All muscle groups exceeded preoperative strength within 1 year (see Tables 4 and 5). Hip abductors and extensors and quadriceps were the muscle groups of primary concern throughout the rehabilitation process because of their contribution to the preoperative crouched position.
Prior to surgery, S.R. experienced pain in both knees, primarily in the evenings, and pain reached the highest levels when he walked long distances. He used medication almost daily. Following surgery, the most common locations of pain were as follows: (1) both knees until the restraining wires were removed in postoperative month 9, and (2) the left hip adductors until the “PERCs” procedure in postoperative month 21. From month 10 to 36, knee pain requiring pain medication (acetaminophen or ibuprofen) was experienced 1 to 3 times per week but was not severe enough to limit activity participation. Low back pain limited activity on several occasions.
The gait pattern commonly known as “crouch gait” is a frequent sequelae in individuals with CP-diplegia. Crouch gait is characterized by knee flexion; hip flexion, adduction, and internal rotation; and either excessive ankle dorsiflexion or plantar flexion during the stance phase. Failure of the hip, knee, and/or ankle extensors to maintain the fully upright position against gravity may occur for a variety of reasons. Although weakness (quadriceps more than gluteals, and gluteals more than plantar flexors) was a primary contributor, multiple other factors also played a role. Bilateral internal femoral torsion, combined with an unstable left hip joint and medial longitudinal arch of the foot further, altered the function of antigravity muscles. For S.R. knee pain and patella alta further compromised the ability of the knee extensor mechanism to maintain knee extension during standing and gait. Despite a comprehensive strength training program and bilateral ankle bracing, the crouch gait progressed rapidly. The failure of a single PFDO to provide sustained correction of the functional position of the left LE prior to SEMLS contributed to the belief that additional factors must also be addressed. In the opinion of the orthopedic surgeon, an SEMLS that addressed bilateral internal femoral torsion and patella alta, left hip dysplasia, and instability of the left midfoot in a single surgery had the potential to provide the best functional outcome.
The SEMLS modified the biomechanical alignment of the left hip, both femurs, both knees, and the left foot, correcting most of the factors contributing to crouch gait for this adolescent. The surgical modification of bone and soft tissues established the potential for normal extensor mechanism function in the knee and successful strength training. It is noteworthy that the gastrocsoleus and quadriceps reached preoperative strength levels by the second postoperative month. The knee joint region experienced the most significant surgical intervention, including bilateral patellar tendon advancements and DFDOs, and yet preoperative strength returned within the normal time frame for healing of tendon and bone using only active assistive ROM (AAROM) and AROM during this time period. It is the author's belief that improved biomechanical alignment contributed to this achievement. These strength changes exceed those reported by other authors, who found that preoperative strength levels were not achieved by 1 year post-SEMLS. 23,50
Two additional surgical interventions were performed by the same surgeon within the time period covered by this case report. First, patellar restraining wires were removed at 9 months. Following this procedure, bilateral knee pain resolved rapidly and within 4 to 6 weeks S.R. tolerated prone and kneeling activities. Second, PERCs to the left adductors and a left obturator nerve alcohol block were done at 21 months. Full passive left hip abduction and knee extension were achieved immediately, and an improvement in strength followed within weeks. PERCs and the phenol block essentially provided “fine tuning”; reduced tone in the left hip adductors, paved the way for further hip abductor/extensor strengthening.
The functional outcomes of SEMLS may be further examined by considering the GMFCS and GMFM-66 scores. S.R.'s functional abilities were GMFCS level II prior to SEMLS as well as after rehabilitation following SEMLS.26 While GMFCS levels are generally considered to be stable over time,2 S.R.'s functional level was declining rapidly. It was the belief of the treating physical therapist that at this rate of decline, S.R. was close to reliance upon a wheelchair for primary mobility, therefore functioning at GMFCS level III. The timing of SEMLS for S.R. prevented this from occurring. Hanna et al53 determined percentile rankings for each GMFCS level for 2- to 12-year-old children. If the percentiles for 12-year-old children at GMFCS level II are used for comparison, S.R. was ranked at the 50th percentile prior to surgery and at the 95th percentile at 30 months postop, that is, moving closer to level I functionally. Changes in GMFCS levels in response to surgical intervention have been documented by Rodda et al,2 who reported that 2 of 10 participants in their study changed from level III to II after SEMLS.
GMFM-66 scores are reported to be sensitive to change over time,54 although changes are expected to be larger in younger children.32 Russell et al32 calculated the mean estimated abilities of 537 participants whose mean ages (±SD) ranged between 5.89 ± 2.77 and 6.67 ± 2.52 years. Children who functioned at GMFCS level II had mean (±SD) scores of 60.92 ± 11.16. Children with CP commonly achieve their best GMFM scores by 7 years of age. S.R.'s preoperative GMFM-66 score was 70.81, which falls within 1 SD above the mean reported by Russell et al.32 Considering all of these factors, S.R. exceeded predicted GMFM values by 24 months postop. This comparison must be interpreted with caution because the Russell study assessed children who were younger than S.R.
The outcomes in this case report may not be the representative of SEMLS for all children with CP-diplegia, GMFCS level II. S.R.'s preoperative strength was regained by 11 months postop, which exceeds the strength returns documented in other studies.23,50 There are several factors that may account for the success of SEMLS for SR. The most important factor is that S.R. was a very motivated, hard-working, and intelligent young man. His family was an exceptional team, working together to make tough decisions, managing all aspects of care, and providing physical and emotional support for one another. As a team, S.R. and his family were open-minded and realistic throughout the rehabilitation process. The surgeon must be given credit for his skill in selecting and performing the surgical procedures. In addition, the surgeon was realistic with his projections about the surgery, the postoperative period, and the long-term outcomes of the surgery; receptive to team input; and prompt in addressing all issues that arose during the rehabilitation process. And finally, the importance of appropriate PT intervention must be considered.
When choosing appropriate postoperative interventions, the physical therapist took into account the importance of pain control and immediate postoperative mobility. S.R.'s tolerance was always a primary consideration; he was never pushed through pain or to ultimate fatigue. Within the boundaries of pain, circuit training was used for the UE, LE, and trunk with the addition of task-specific functional activities that were appropriate for the stage of the rehabilitation program. Although most of the PT interventions used in this case could be considered common rehabilitation practices, there are 2 interventions that may be unique to this case. First, high repetition active exercise in gravity eliminated positions for the target muscle groups was used heavily in months 2 to 12. The rationale for this choice was to (1) enhance motor learning by providing a high number of repetitions in nonsynergistic movement patterns and (2) maximize endurance with high repetition and low load.51 Second, manual therapy interventions, such as dorsal gliding of the left femur to stretch the posterior capsular, and manual lumbar traction to reduce low back pain were incorporated as needed.55
The limitations of this study and recommendations for future research include the following. First, as a case report the results cannot be generalized to the larger population of adolescents with CP. Studying the rehabilitation process with a larger sample is needed. It is recommended that a hand-held dynamometer be used in place of MMT for improved reliability in strength assessment in future studies. The use of cardiopulmonary measures to quantify tolerance to activity within sessions and to calculate energy cost prior to surgery and at key points within the rehabilitation process would provide valuable quantification of the effects of PT, as well as surgical outcomes of SEMLS.
In summary, PT following SEMLS required extensive resources to achieve the optimal outcome. S.R. had the personal reserves to participate in the long rehabilitation process. The family had adequate resources in several areas: insurance, finances, time, emotional strength, and a strong support system of extended family and friends. A well-coordinated team (S.R., family, physician, surgeon, and physical therapist) was necessary to manage the multitude of issues that surfaced during the immediate postoperative period and the long-term rehabilitation process. PT intervention simultaneously focused on (1) S.R.'s immediate needs (physical, social, and emotional); (2) the factors, such as pain, fatigue levels, available strength, and current functional abilities, that affected each intervention; and (3) establishing the foundations for future function.
For S.R., SEMLS, along with a well-coordinated rehabilitation team, effectively addressed the impairments in body structures that contributed to crouch posture and gait, chronic knee pain, and limitations in participation. Following SEMLS and an extensive rehabilitation process, S.R.'s strength and participation levels exceeded his preoperative participation levels.
Two months before his untimely death, S.R. posted a letter to his friends on Facebook, including the following advice: “Give your greatest effort to overcome all obstacles and never give up on your goals. Nothing is impossible unless you say it is (even Calculus).” His words reflect the way he lived his life. This amazing young man was an inspiration to every person he met. This manuscript is dedicated to the loving memory of Robby Spellman (August 30, 1992, to June 17, 2010).
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activities of daily living; adolescent; case report; cerebral palsy/rehabilitation/surgery; disability evaluation; motor skills; orthopedic procedures/methods; physical therapy; treatment outcome
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