Abstract
Background:
A dorsal bunion may occur in nonambulatory adolescents with cerebral palsy (CP) and a Gross Motor Function Classification System (GMFCS) level of IV or V. The deformity can cause pain, skin breakdown, and difficulty wearing shoes and braces. A consensus on the biomechanics and surgical management of dorsal bunions in persons with severe CP has not been established.
Methods:
This retrospective cohort study included 23 nonambulatory adolescents with CP, GMFCS level IV or V, and symptomatic dorsal bunions requiring surgery. The median age at surgery was 17 years, and the median follow-up was 56 months. Reconstructive surgery included the excision of a 2 to 3-cm segment of the tibialis anterior tendon to correct the elevation of the first metatarsal. The fixed deformity of the first metatarsophalangeal joint was managed with use of corrective arthrodesis and dorsal plate fixation. Clinical and radiographic outcomes were assessed preoperatively and postoperatively at the transition to adult services.
Results:
There were significant improvements in the clinical and radiographic outcome measures (p < 0.001). Pain was relieved, and there were no further episodes of skin breakdown. The elevation of the first metatarsal was corrected from a mean of 3° of dorsiflexion to a mean of 19° of plantar flexion. The deformity of the first metatarsophalangeal joint was corrected from a mean of 55° of plantar flexion to a mean of 21° of dorsiflexion. Six patients had complications, all of which were grade I or II according to the modified Clavien-Dindo system.
Conclusions:
The surgical reconstruction of a dorsal bunion via soft-tissue rebalancing of the first ray and corrective arthrodesis of the first metatarsophalangeal joint resulted in favorable medium-term clinical and radiographic outcomes in nonambulatory adolescents with CP.
Level of Evidence:
Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.
Hip displacement management and neuromuscular scoliosis correction dominate the orthopaedic care of nonambulatory children with cerebral palsy (CP)1-4. However, once children with CP reach adolescence, painful deformities of the first metatarsophalangeal joint (MTPJ) may also develop5. Ambulatory adolescents with spastic diplegia and a Gross Motor Function Classification System (GMFCS) level of I to III mainly develop symptomatic hallux valgus, whereas nonambulatory adolescents with a GMFCS level of IV or V more commonly develop dorsal bunions5,6. The accepted treatment of symptomatic hallux valgus in most adolescents with CP is arthrodesis of the first MTPJ7,8. The optimal surgical treatment of dorsal bunions in adolescents with CP, however, remains unknown6.
To date, research on dorsal bunions and their treatment has consisted of small case series and case reports on heterogeneous patient groups with different functional levels, prior surgical treatment, and diagnoses, including hallux rigidus, congenital talipes equinovarus, poliomyelitis, and CP9-11. Various surgical combinations have been advocated to correct dorsiflexion at the metatarsocuneiform joint, elevation of the first metatarsal, and fixed plantar flexion contracture at the first MTPJ. Treatment algorithms usually comprise a combination of the following: plantar flexion osteotomy of the first ray; corrective arthrodesis of the metatarsocuneiform and/or naviculocuneiform joints, sometimes with a partial excision of the proximal phalanx; and tendon transfers to rebalance the first ray and to maintain correction6,9-11. The first MTPJ is typically preserved, except when it demonstrates severe degeneration6,9-11.
Lapidus and Goldner identified an overactive tibialis anterior as being associated with a dorsal bunion, and both advised a transfer of the tibialis anterior tendon (TAT) as a part of the surgical reconstruction6,9. In a previous population-based study of deformities of the first MTPJ in patients with CP5, Van de Velde et al. hypothesized that the emergence of dystonia or spastic dystonia in the tibialis anterior muscle during the adolescent growth spurt results in increased dorsiflexion at the ankle, progressive elevation of the first metatarsal, and flexion at the first MTPJ (Figs. 1 and 2). In the present study, we hypothesized that the elevation of the first metatarsal remains flexible in patients in whom dystonic hypertonia is intermittent. In contrast, the hyperflexed first MTPJ undergoes degeneration with severe cartilage loss, and the deformity becomes fixed. On the basis of this hypothesis, we developed a surgical algorithm for dorsal bunion correction in nonambulatory adolescents with CP, which consists of both the excision of a 2 to 3-cm segment of the TAT to correct the elevation of the first metatarsal and corrective arthrodesis of the first MTPJ, and does not require a plantar flexion osteotomy of the first ray or tendon transfers. The principal deforming force, the dystonic tibialis anterior muscle, is defunctioned, allowing the first metatarsal to flex. The intact flexor hallucis brevis (FHB) and flexor hallucis longus (FHL) are then able to act as depressors of the first ray (Figs. 3 and 4).
The purpose of this study was to review the clinical and radiographic outcomes of 23 nonambulatory adolescents with CP and symptomatic dorsal bunions who underwent excision of a segment of the TAT and arthrodesis of the first MTPJ.
Materials and Methods
Study Design and Setting
We performed a retrospective observational study using prospectively collected data on adolescents with CP who had undergone surgery for a dorsal bunion between 1996 and 2019 at a single tertiary-level children’s hospital. The Ethics in Human Research Committee of the investigating institution gave ethical approval for the study (QA/97547/RCHM-2023). The inclusion criteria were a diagnosis of CP with registration on the statewide CP register, the surgical correction of a dorsal bunion in 1 or both feet, and the availability of standardized radiographs made at baseline and at a minimum follow-up of 36 months postoperatively. Patients were classified according to their functional level, which was determined with use of the GMFCS12; the movement disorder (spastic, dystonic, or mixed tone); and the topographical distribution (hemiplegia, diplegia, triplegia, or quadriplegia)1,13. Out of 27 patients who were identified, 2 patients who had previously undergone surgery for pes valgus were excluded, as were 2 patients who underwent lengthening of the TAT rather than excision of a segment of the tendon.
A total of 23 patients with CP (12 male and 11 female) were included in the study and their data were evaluated. All except 1 patient underwent bilateral surgery for dorsal bunions (45 feet). The median age at the time of surgery was 17 years (range, 14 to 20 years). The functional level was classified as GMFCS level V in 14 patients and level IV in 9 patients. Spastic dystonia was present in 14 patients, and a dystonic movement disorder was present in the remaining 9 patients. All patients had quadriplegia.
Clinical and radiographic outcomes were obtained before and after surgery. Clinical and radiographic follow-up continued until the transition to adult services at age 20 to 25.
Surgical Procedure
The tibialis anterior muscle was injected with botulinum neurotoxin A (BoNT-A) to reduce hypertonia, postoperative pain, and the risk of a postoperative dystonic crisis. Using 100 units of onabotulinumtoxinA (BOTOX; Allergan) diluted in 4 mL of normal saline solution, a dose of 3 U per kg of body weight was administered per side, with use of muscle stimulation for improved accuracy14. A thigh tourniquet was utilized, and the operation was performed with the patient under general anesthesia, which was supplemented by a sciatic nerve block for postoperative analgesia.
A 2 to 3-cm segment of the TAT was excised through a short incision just proximal to the extensor retinaculum (Fig. 3). The dorsal bunion was approached through a 6-cm dorsomedial incision, with care taken to avoid atrophic skin that was damaged from previous inflammation or ulceration (Fig. 4). The extensor hallucis longus tendon was identified, mobilized, and retracted. Extensive division of the joint capsule and mobilization of the collateral ligaments were required to fully mobilize the fixed flexion deformity of the proximal phalanx11. A trial reduction was performed to confirm that the first metatarsal would have a satisfactory degree of plantar flexion and that the proximal phalanx could be reduced onto the head of the metatarsal. Preparation of the joint was performed with use of cup-and-cone reamers of the appropriate size8,15. Care was taken to avoid excessive shortening of osteoporotic bone. The subchondral bone was perforated on both sides of the joint to promote earlier fusion. A pre-bent, 4-hole Vitallium plate (Stryker) was utilized in the first 6 patients to provide stable internal fixation and to correct the deformity16. Later, in the remaining patients, a custom dorsal locking plate (Acumed) designed for fusion of the first MTPJ was utilized. This plate has a preset hallux valgus angle (HVA) and a preset lateral metatarsophalangeal angle (LMTPA)8,17. Following copious wound irrigation, the incision was closed routinely in layers with use of interrupted 3-0 nylon sutures in the skin. Two patients underwent percutaneous release of toe flexion contractures at the time of the index procedure, and no patients underwent a concomitant procedure for pes valgus during the index surgery (Figs. 5 and 6).
Several layers of gauze dressing were placed directly over the dorsal incision, and a well-padded posterior back-slab cast was applied with the foot and ankle in 10° to 20° of plantar flexion to stretch out the lengthened TAT. The cast was removed at 2 weeks postoperatively to inspect the wound and remove the skin sutures. A well-padded cast was reapplied for a further 4 weeks; alternatively, a CAM (controlled ankle motion) walker was utilized in adolescents who were capable of weight-bearing transfers. Radiographs were made prior to discharge and at 6 and 12 weeks to assess the integrity of the fixation and the state of fusion. An ankle-foot orthosis (AFO) was provided after cast removal to protect the fusion site and to reduce the risk of recurrent contractures.
Clinical Evaluations
To record patient-reported outcome measures (PROMs) in the present study, we devised a 10-point visual analog scale (VAS) for each of 3 domains: pain, skin inflammation and/or sores, and difficulty with shoe and brace wear18. Each scale ranged from “none” to “very severe” (see Appendix Fig. 1) and was completed by the patient or their parent or caregiver. PROMs were recorded preoperatively and at the time of final follow-up. The final evaluations were conducted during the clinic appointment for the formal transition to adult services. Postoperative complications were noted and graded with use of the modified Clavien-Dindo (MCD) system, which has good reliability8.
Radiographic Evaluations
Standardized simulated weight-bearing anteroposterior and lateral radiographs were made by having the patient seated in their wheelchair with the film cassette placed under the foot for the anteroposterior view or at the side for the lateral view (Figs. 7 through 10)5. Measurements included the HVA, the LMTPA (see Appendix Fig. 2), and the first metatarsal declination angle (MTDA; see Appendix Fig. 3)5,7,19,20. Radiographic measurements on digital radiographs were made by 1 attending orthopaedic surgeon and 1 fellow in orthopaedic surgery with use of PACS (picture archiving and communication system) tools. The mean of each pair of measurements was utilized for the subsequent analysis. The analysis was performed with use of the preoperative radiographs and radiographs from the clinic appointment for the transition to adult services.
Statistical Analysis
The statistical analysis was performed with use of IBM SPSS Statistics (version 29.0.0.0). The results are given as the mean, with the standard deviation or range, or as the median and range. Data were tested for normality with use of the Kolmogorov-Smirnov test. Significance was analyzed with use of the Student t test for unpaired data, with the level of significance set at p < 0.05.
Results
Two patients with a previous surgery for pes valgus were excluded. Two patients with deformities that had been managed with intramuscular lengthening of the tibialis anterior before the surgical approach was standardized, and who required revision surgery to excise a segment of the TAT, were also excluded. This left 23 patients (45 feet) for analysis. No patients were lost to follow-up in the period between the surgery and the evaluation at the appointment for the transition to adult services. The median follow-up duration was 56 months (range, 36 to 152 months).
Clinical Outcomes
Preoperatively, the mean VAS score in each of the 3 domains was 7 (“severe”; range, 5 to 10). In the 12 months before the surgery, 11 patients had skin ulceration and 12 had inflammation without frank skin breakdown (Fig. 2). One patient required admission to the hospital for the management of infected sores over the apex of the dorsal bunion, 1 patient had a sore that was managed at home with intravenous antibiotics and dressings, and another 3 patients received ≥1 course of oral antibiotics.
Intraoperatively, a fixed flexion deformity of the first MTPJ, with at least 50% of the area exhibiting full-thickness cartilage loss, was encountered in all patients. The first metatarsocuneiform joint was flexible, and the elevation of the first metatarsal was reduced with use of manual pressure after resecting the TAT.
There were no recurrences of dorsal bunion deformity, pain, skin irritation, or skin breakdown following surgical reconstruction. There were no instances of nonunion or acquired equinus deformity. At the final postoperative follow-up, the mean VAS scores for pain, skin irritation, and difficulties with shoes and braces were 1.0, 0.8, and 0.7, respectively (range for each domain, 0 to 3), which were significantly improved from baseline (p < 0.001). VAS scores were completed by parents or caregivers for 15 patients with communication difficulties. All patients were prescribed solid AFOs after cast removal. A total of 12 patients (8 with GMFCS level IV) continued to use AFOs in the longer-term follow-up period in order to facilitate standing transfers and limited ambulation postoperatively.
The surgical and medical complications are listed in Table I. There were 10 complications across 6 patients; all complications had an MCD grade of I or II. Adverse events were mild, self-limiting, and usually multiple.
TABLE I.
MCD Grade | Complication | No. of Patients | Treatment |
---|---|---|---|
Grade I | Acute increase in dystonia and painful spasms | 3 | Benzodiazepines, baclofen, and opiate analgesia |
Grade I | Temporary respiratory depression | 2 | Chest physiotherapy |
Grade II | Urinary tract infection | 1 | IV antibiotics |
Grade II | Superficial wound infection | 2 | Suture removal and dressings |
Grade II | Superficial cast sores | 2 | Dressings and change of cast |
IV = intravenous. Ten complications were recorded in 6 patients. When complications occurred, they were usually multiple: for example, increased dystonia and spasms required increased doses of benzodiazepines and opiates, and were followed by either rubbing against the cast, with associated skin sores, or respiratory depression requiring chest physiotherapy.
Radiographic Outcomes (Table II)
The mean HVA (and standard deviation) was 27° ± 6° preoperatively and 9° ± 3° postoperatively (Figs. 6 and 7). The deformity of the first MTPJ was corrected from a mean LMTPA of 55° ± 10° of plantar flexion preoperatively to a mean of 21° ± 5° of dorsiflexion at the final follow-up (p < 0.0001). The elevation of the first metatarsal was significantly (p < 0.0001) improved from a mean first MTDA of 3° ± 9° of dorsiflexion preoperatively to a mean of 19° ± 3° of plantar flexion postoperatively (Figs. 8 and 9; Table II).
TABLE II.
Radiographic Outcome | Preoperative† (deg) | Postoperative† (deg) | P Value‡ |
---|---|---|---|
HVA, right | 26.8 ± 6.2 (14 to 38) | 9.1 ± 3.3 (3 to 15) | <0.0001 |
HVA, left | 27.0 ± 7.1 (16 to 43) | 9.0 ± 3.5 (3 to 16) | <0.0001 |
LMTPA, right | −54.9 ± 9.3 (−77 to −37) | 21.0 ± 4.0 (15 to 28) | <0.0001 |
LMTPA, left | −55.2 ± 9.7 (−74 to −35) | 21.2 ± 5.8 (13 to 36) | <0.0001 |
First MTDA, right | −2.7 ± 8.8 (−24 to 13) | 18.9 ± 2.6 (14 to 23) | <0.0001 |
First MTDA, left | −2.8 ± 8.7 (−24 to 12) | 19.1 ± 3.5 (12 to 26) | <0.0001 |
HVA = hallux valgus angle, LMTPA = lateral metatarsophalangeal angle, first MTDA = first metatarsal declination angle. See Appendix Figs. 2 and 3 for the measurement techniques for the LMTPA and MTDA.
Values are given as the mean ± standard deviation, with the range in parentheses.
Determined with use of the Student t test for unpaired data. P < 0.05 was significant.
Discussion
In this study, we found that dorsal bunion correction by excision of a 2 to 3-cm segment of the TAT and corrective arthrodesis of the first MTPJ resulted in favorable medium-term clinical and radiographic outcomes in nonambulatory adolescents with CP. The lasting effect of this procedure confirmed our hypothesis regarding the underlying abnormal biomechanics of dorsal bunions in CP—that is, that dystonia in the tibialis anterior muscle leads to progressive but flexible elevation of the first metatarsal and fixed flexion at the first MTPJ.
We agree with the views of Lapidus, Goldner, and Bleck that the elevation of the first metatarsal, due to a combination of hypertonia and the contracture of the TAT, is the primary deformity in symptomatic dorsal bunions in CP6,9,10. In an analysis of a population-based CP register over a study period of 21 years, Van de Velde et al. reported that symptomatic dorsal bunions developed in nonambulatory adolescents who had a GMFCS level of IV or V (as seen in the present study) and either dystonic hypertonia or mixed-tone hypertonia (i.e., spastic dystonia) in the tibialis anterior muscle5. We considered that the TAT contracture required lengthening, and we judged that a tendon transfer was unlikely to succeed (Figs. 2 and 5).
Given the inherent risks of deformity reversal associated with dystonia, we cautiously approached the defunctioning of the TAT. Before performing any surgery in the first 4 patients, we injected the tibialis anterior muscle with BoNT-A to see if it would be beneficial and if there was a risk of precipitating an equinus deformity1,14. The clinical effects of injections of BoNT-A were marginal and short-lived. Prior to the standardization of the surgical approach to include the resection of a segment of the TAT, 2 patients underwent conservative intramuscular lengthening of the TAT that was performed proximal to the extensor retinaculum. Intramuscular lengthening was effective in the short term, but painful dystonic contractions were noted at 8 to 10 weeks after the index surgery. A revision surgery with excision of the tendon was required for both patients, who demonstrated excellent outcomes at the time of the final follow-up.
We believe that surgical reconstruction of the first ray is advisable as a matter of urgency once there is symptomatic inflammation over the apex of the dorsal bunion and should preferably be performed before there are multiple episodes of skin ulceration5,6. Theoretically, if the deformity were recognized in its earliest stage, then isolated defunctioning of the TAT might be sufficient to correct the subsequent abnormal biomechanics of the first ray5. However, we found that, once the presence of a dorsal bunion was established, the deformity progressed rapidly, with escalating pain and skin ulceration, subluxation, and a severe fixed flexion deformity of the first MTPJ that included full-thickness loss of articular cartilage at the head of the first metatarsal (Fig. 7). In contrast to the severe and fixed flexion contracture at the first MTPJ, the elevation of the first metatarsal remained flexible and was easily reduced with use of manual pressure after resecting the TAT. None of our patients required an osteotomy or arthrodesis at the proximal end of the first metatarsal to correct the elevation of the first metatarsal. The primary benefit of corrective arthrodesis of the first MTPJ was a lasting correction of the fixed deformity, with pain relief and improved skin care. A secondary benefit was that the intact FHB and FHL actively depressed the first ray instead of flexing the first MTPJ (Fig. 8).
The use of cup-and-cone reamers and dorsal plating allowed for optimal correction and stable fixation8,15,16. Surgery can trigger an increase in dystonia in patients with CP, which can occasionally lead to a dystonic crisis. We considered stable fixation essential for reducing the risks of delayed union or malunion in patients at a risk for low bone density21-23.
Major reconstructive surgery in nonambulatory patients with CP has been associated with significant morbidity and mortality1,24. Orthopaedic surgery in children with dystonia has had a reputation for unpredictable outcomes, but a recent study showed that good outcomes can be achieved25. In the present study, 3 patients had multiple medical adverse events related to an increase in dystonia, pain, and spasms that resulted in increased medication, which was followed by either skin irritation from the cast or respiratory depression (Table I). Multidisciplinary perioperative management and the involvement of a pain management service are advised25-27.
The present study has limitations. The study was retrospective and did not include a control group. No validated PROMs have been developed for foot and ankle surgery in this population18. Each VAS that we utilized was simple, feasible, and acceptable but has not been tested for validity or reliability18. Sixty-five percent of the study group (15 adolescent patients with severe communication difficulties) had VAS scores completed by parents or caregivers. The study population had moderate hallux valgus as well as dorsal bunions. The diagnosis of a dorsal bunion was based on the location of the symptoms rather than prespecified radiographic criteria5-8.
The strengths of the study were the standardization of the surgical approach and the prospective collection of data with use of standardized clinical and radiographic measures.
There were no cases of acquired equinus deformity at the time of the final follow-up. At the time of writing, none of the implants have been symptomatic and none have been removed.
In conclusion, the surgical reconstruction of a dorsal bunion via soft-tissue rebalancing of the first ray and corrective arthrodesis of the first MTPJ resulted in favorable medium-term clinical and radiographic outcomes in nonambulatory adolescents with CP.
Appendix
Supporting material provided by the authors is posted with the online version of this article as a data supplement at jbjs.org (http://links.lww.com/JBJS/I169).
Supplementary Material
Footnotes
Investigation performed at The Royal Children’s Hospital, Parkville, Victoria, Australia
Disclosure: No external funding was received for this work. The Article Processing Charge for open access publication was funded by the Murdoch Children’s Research Institute, Melbourne. The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJS/I168).
Contributor Information
Samuel K. Van de Velde, Email: samuelvdv@gmail.com.
Ken Ye, Email: mr.yekken@gmail.com.
Henry Chambers, Email: hankchambers@me.com.
Erich Rutz, Email: erich.rutx@rch.org.au.
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