ORIGINAL ARTICLE
The Effectiveness of the Ponseti Method for Treating Clubfoot Associated With Amniotic Band Syndrome Aaron M. Carpiaux, MD,* Pooya Hosseinzadeh, MD,w Ryan D. Muchow, MD,z Henry J. Iwinski, MD,z Janet L. Walker, MD,z and Todd A. Milbrandt, MDz
Background: Amniotic band syndrome (ABS) is a congenital disorder with an associated incidence of clubfoot deformity in over 50% of patients. Although early reports in the literature demonstrated a poor response to casting treatments, recent application of the Ponseti technique in ABS patients have been more promising. Methods: A retrospective review of all patients with clubfoot and a concurrent diagnosis of ABS were reviewed at a single institution. Patients not managed initially with the Ponseti method were excluded. Data collected included patient age at presentation, sex, unilateral or bilateral, amniotic band location and associated findings, and response to treatment—number of casts and requirement of Achilles tenotomy, tibialis anterior tendon transfer, or other surgical procedures. Duration of treatment at latest follow-up visit was noted and outcome was based on clinical foot appearance and plan for any further procedures. Results: Twelve patients (7 female and 5 male) with a total of 21 feet (9 bilateral and 3 unilateral) were identified. The average age at presentation was 3 weeks (range, 1 to 9 wk). The average number of casts was 6 (range, 3 to 11). Seventeen of 21 feet (81.0%) underwent percutaneous Achilles tenotomy. The initial correction rate for all patients with the Ponseti technique was 20/21 feet (95.2%) and recurrence was noted in 7/21 feet (33.3%). One patient underwent primary posteromedial release and 2 patients had associated neurological deficits. The average follow-up was 3.9 years (range, 9 mo to 10 y) and all but one patient had supple, plantigrade feet. Conclusion: The Ponseti technique is an effective first-line treatment in patients who have clubfeet associated with ABS, including those with a neurological deficit. Level of Evidence: Level IV.
From the *Department of Orthopaedic Surgery and Sports Medicine, University of Kentucky; zShriners Hospital for Children, Lexington, KY; and wBaptist Pediatric Orthopaedics Center, Miami, FL. None of the above authors have received financial support for this study. The authors declare no conflicts of interest. Reprints: Aaron M. Carpiaux, MD, Department of Orthopaedic Surgery and Sports Medicine, University of Kentucky, 740 South Limestone Street, K401, Lexington, KY 40536. E-mail: aaron. [email protected]. Supplemental Digital Content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s Website, www. pedorthopaedics.com. Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.
284 | www.pedorthopaedics.com
Key Words: amniotic band syndrome, clubfoot, Ponseti method, casting, congenital annular band syndrome, talipes equinovarus (J Pediatr Orthop 2016;36:284–288)
A
mniotic band syndrome (ABS) is a congenital disorder believed to be caused by separation of the amnion from the chorion resulting in fibrous bands which can encircle limbs and lead to anomalies and amputation during fetal development.1 The resultant oligohydramnios from fetal membrane disruption seen in ABS patients is thought to be the link to the high incidence of concurrent clubfoot. Although anomalies of the hand and fingers are most common, there has been an associated incidence with clubfoot deformity in 12% to 56%.1–8 ABS clubfoot patients have often been grouped with other nonidiopathic clubfoot disorders, such as arthrogryposis and spina bifida, based on their poor response to conservative treatment with casting, requiring many to undergo comprehensive soft-tissue releases.2–4,6,9–12 The success of the Ponseti method at treating idiopathic clubfoot has recently been extended to the management of nonidiopathic clubfoot including ABS.13–18 Zionts and Habell published a report of the Ponseti technique in ABS patients successfully treating 6 clubfeet in 5 ABS patients with casting plus Achilles tenotomy. Four of the 6 clubfeet had ipsilateral bands and no neurological deficits were noted. One patient required a tibialis anterior tendon transfer (TATT) and none required extensive soft-tissue releases.14 Because of the rarity of ABS and clubfoot, as well as the relatively recent popularization of the Ponseti method as the standard of care in the treatment of clubfoot, these case series have remained small and the outcomes in these patients are still being elucidated. In addition, no recent series have analyzed band location or included clubfeet in ABS patients with an ipsilateral neurological deficit from banding, which were hypothesized by early authors to be even more rigid and resistant to nonoperative treatment.2,3,6,9,10 The purpose of this study, therefore, was to examine our cohort of ABS patients with clubfoot treated initially with the Ponseti method and compare treatment effectiveness to recent literature reports. Our hypothesis was that the Ponseti method would be an effective initial treatment with few patients requiring comprehensive softtissue releases. J Pediatr Orthop
Volume 36, Number 3, April/May 2016
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J Pediatr Orthop
Volume 36, Number 3, April/May 2016
METHODS An IRB-approved, retrospective review of all patients between January 2000 and December 2012 with concurrent diagnoses of clubfoot and ABS was performed. Patients were excluded if they had received any clubfoot treatment before presentation at our institution or if their primary treatment was not Ponseti casting. Data collected included patient age at presentation, sex, unilateral or bilateral, amniotic band location and associated findings, and response to treatment—number of casts and requirement of Achilles tenotomy, TATT, or other surgical procedures. Neurological deficits were defined as lack of active foot and/or ankle motion with response to stimulus at the initial visit or subsequent follow-up. Duration of treatment at latest follow-up visit was noted and outcome was based on clinical foot appearance and plan for any further procedures. Our institution’s clubfoot treatment algorithm initiates Ponseti casting at the initial patient visit with weekly cast changes, thereafter to sequentially correct the cavus, adductus, varus, and equinus deformities as previously described by Ponseti.19 Percutaneous Achilles tenotomy was performed in the operating room under sedation for patients with residual equinus deformity. Three months of full-time (23 h) foot abduction orthosis wear was then instituted to maintain deformity correction followed by part-time wear (night-time and naps, approximately 12 h) until age 3 or when the family decided to discontinue wear. Recurrence was defined by initiating any treatment—bracing, casting, or surgery—once a corrected, plantigrade foot had been achieved.
RESULTS Twelve patients (7 female and 5 male) with a total of 21 feet (9 bilateral, 3 unilateral) were identified and summarized in Table 1. The average age at presentation was 3 weeks (range, 1 to 9 wk). Lower extremity ABS involvement was found in 10 patients and upper extremity involvement was found in 10 patients (Fig. 1). Seven patients had ipsilateral bands of the lower extremity with clubfoot deformity and 2 of these (patients 3 and 7) had an associated neurological deficit, both with deep thigh bands and one with a calf band. Both patients had absent active ankle dorsiflexion and 1 patient also lacked ankle eversion on the affected side. The other patient underwent band release at birth for vascular compromise (Fig. 2). Other than the presence of an amniotic banding, the most common distal extremity finding was acrosyndactyly. The Appendix Table (Supplemental Digital Content 1, http://links.lww.com/BPO/A37) documents these additional findings and their non–clubfoot-related surgical interventions.
Ponseti Treatment The average number of casts was 6 (range, 3 to 11). Seventeen of 21 feet (81.0%) underwent percutaneous Achilles tenotomy. The initial correction rate for all patients with the Ponseti technique was 20/21 feet (95.2%) (Table 2). Copyright
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Ponseti Method and Amniotic Band Syndrome
Three patients had significantly different courses and they are described here: patient 7 had 5 serial casts placed bilaterally, without any improvement to the left foot. The left foot underwent an additional 4 rounds of casting before proceeding with posteromedial soft-tissue release because of a lack of correction. Casting was continued during this time on the right to maintain correction before Achilles tenotomy was performed concurrently with the left-sided procedure. Patient 11 achieved correction of bilateral clubfoot deformity after 5 casts with residual equinus but had 6 more casts to maintain position while waiting to overcome a respiratory syncytial virus infection before Achilles tenotomy. Bilateral calcaneal pins were placed into the calcaneal tuberosity in patient 8 at the same time as Achilles tenotomy to help achieve and hold plantigrade position during final casting. They were removed in clinic 3 weeks later with good correction.
Recurrence Seven feet were defined as recurrent at an average time of 3.7 years (recurrence rate = 7/21, 33.3%). All required surgical intervention. TATT was performed for those with evidence of dynamic muscle imbalance by gait examination and/or pedobaric foot pressure analysis, and further soft-tissue releases were performed for stiff, recurrent clubfoot deformity. Three patients underwent TATT, 1 patient was scheduled to have TATT but was lost to follow-up, and 1 patient was scheduled for revision posteromedial release. The average follow-up was 3.9 years (range, 9 mo to 10 y). Four patients were still in part-time foot abduction orthosis wear and 1 patient was lost to follow-up. The remaining 10 patients had supple, plantigrade feet (Table 2).
Amniotic Bands and Clubfoot Treatment course and recurrence were stratified by band location because of previous authors’ findings that proximal band location was associated with a more recalcitrant clubfoot.2 There were 18 feet with ipsilateral lower extremity amniotic bands and clubfeet. The remaining 3 feet were included as an internal control for comparison. The average number of casts did not differ between those with bands located proximal to the ankle, distal to the ankle, and those without banding (7.0 vs. 6.4 vs. 4.6 casts). The recurrence rate was similar between the 3 groups as well, 3/8 feet (38%) versus 3/14 feet (21%) versus 1/3 feet (33%). Ipsilateral banding did not affect the effectiveness of the Ponseti technique in the treatment of clubfeet associated with ABS (Table 3). There were 2 lower extremities with deep thigh bands resulting in a neurological deficit and associated with clubfoot. Patient 3 was found to have vascular compromise at birth from a left deep thigh band and underwent emergent release. No active ankle dorsiflexion was found on the left side at presentation but the patient responded well to casting and Achilles tenotomy, requiring no further clubfoot procedures and having a plantigrade foot at 7-year follow-up. The other patient www.pedorthopaedics.com |
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TABLE 1. Patient Data Patient Number
Sex
Age (wk)
1 2 3
Male Male Male
1 9 8
4 5 6 7
Female Female Male Female
1 1 3 1
8
Male
4
Bilateral No Bilateral No Left Yes, no ankle dorsiflexion on left Bilateral No Bilateral No Right No Bilateral Yes, no ankle dorsiflexion or eversion on left Bilateral No
9 10 11 12
Female Female Female Female
1 4 1 2
Left Bilateral Bilateral Bilateral
Side
Neurological Deficit
No No No No
failed Ponseti treatment and has had recurrence after the initial posteromedial release. Non–clubfoot-related procedures for ABS anomalies were quite common. Nine of 12 patients underwent or had plans for soft-tissue procedures to release bands and correct digital anomalies. Most of these procedures were performed in a staged manner under separate anesthetic events (Appendix Table, Supplemental Digital Content 1, http://links.lww.com/BPO/A37).
DISCUSSION Historically, clubfoot treatment associated with ABS has been difficult and often resulted in correction by extensive soft-tissue releases.2–4,6,9–12 This study supports the effectiveness of the Ponseti method for clubfoot correction by demonstrating its success in the largest series of ABS patients to date and the first to include patients with neurological deficits. Initial correction was able to be obtained in 20 of 21 patients (95.2%) treated by the
FIGURE 1. Patient 8—bilateral lower extremities. Baseline photograph at presentation demonstrating bilateral clubfoot deformity with left calf band. Note acrosyndactyly and partial toe amputations on both feet.
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Lower Extremity Findings Left deep calf band. Right midfoot band with acrosyndactyly. Acrosyndactyly of bilateral feet Bilateral multiple bands. Left calf band. Left deep thigh band released at birth for vascular compromise Left thigh band. Right foot acrosyndactyly with partial toe amputations Acrosyndactyly of bilateral feet with partial amputations of right toes None Left thigh deep band. Right foot band Left calf band. Acrosyndactyly of bilateral feet with partial toe amputations. Right thigh band. Bilateral leg bands. Partial amputations of left toes Acrosyndactyly of bilateral feet Bilateral calf partial bands. Partial amputations of bilateral toes None
Ponseti technique with 1 patient requiring extensive softtissue release. Further, 7 of the 21 feet (33.3%) required additional surgery for recurrence of the clubfoot deformity. Supple, plantigrade feet can be achieved with serial casting and surgical intervention is necessary only in resistant or recurrent cases. Serial casting before the popularization of the Ponseti technique had poor results at gaining correction without posteromedial release. Gomez9 was able to successfully treat 2 patients of 35 with ABS and clubfoot using serial casting; however, no casting technique was specified. Chang and Huang10 reported similar results in a series of 10 patients with ABS and clubfoot with only 1 achieving correction with casting and Achilles tenotomy alone. A retrospective review by Allington et al11 of 21 clubfeet with ipsilateral bands found that all but 2 patients required one or more surgical procedures for clubfoot correction. The Ponseti technique has demonstrated effectiveness in treating other difficult, nonidiopathic diagnoses including arthrogryposis, trisomy 21, and spina bifida prompting trial in ABS as well.14–18 Janicki and colleagues studied a cohort of 23 patients (40 feet) with nonidiopathic
FIGURE 2. Patient 3—left lower extremity. Postoperative photographs after Achilles tenotomy showing correction of equinus deformity. The patient also underwent concurrent Z-plasty of ipsilateral deep thigh and calf bands. Copyright
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J Pediatr Orthop
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Ponseti Method and Amniotic Band Syndrome
TABLE 2. Treatment and Outcomes Patient Number
No. Casts
Achilles Tenotomy
Followup
1 2 3
6 6 5
Yes Yes Yes
4 5 6 7
5—left 6—right 6 4 9
8
6
Yes—left No—right Yes Yes Yes—right NA—left Yes
9
3
No
10
4
Yes
None
2y
11
11
Yes
None
1y
12
5
No
None
9 mo
Other Clubfoot Surgeries Bilateral tibialis anterior tendon transfer Bilateral tibialis anterior tendon transfer None None
10 y 7y 7y 2y
None Right tibialis anterior tendon transfer Left posteromedial release
5y 4y 3y
Bilateral calcaneal pin insertion to assist casting None
3y
clubfeet, 3 of which had ABS and bilateral clubfeet treated with Ponseti. All 3 were successfully treated with casting and tenotomy with 1 patient’s recurrence managed with repeat casting.16 Ozkan and colleagues reviewed 2 patients with ABS and clubfoot with ipsilateral band and found successful deformity correction with 7 casts each and Achilles tenotomy. Follow-up averaged 1 year and both patients were still being managed with foot abduction orthosis wear without recurrence.18 Zionts and Habell14 have published the only other series of solely ABS patients treated successfully with the Ponseti technique. They described the treatment of 5 patients, 6 feet, who had achieved initial correction in all feet with an average of 6 casts. All received Achilles tenotomy and early recurrence was managed with repeat casting in 5 feet. TATT was performed in 1 foot (17%). The recurrence rate in our study was 33% (7/21 feet) with the most common procedure being TATT (5/7). All feet (6/6) in the Zionts and Habell’s study were plantigrade at most recent follow-up compared with 90% (19/21) in our series. The difference in average follow-up, 2.7 versus 3.9 years may explain differences in recurrence rate and the increased incidence of TATT in the current study. No patient under 3 years old underwent TATT in the current study. One patient in our study required extensive posterior release primarily, compared with zero in the Zionts and Habell’s study. There were no patients with neurological deficits in the Zionts and Habell’s series compared with 2 in the current.
2y
Outcomes No pain, plantigrade feet, active in sports No pain, plantigrade feet Plantigrade foot. Plan for epiphysiodesis in future for 4.5 cm leg length discrepancy, right > left Planned for tibialis anterior transfer on left, but lost to follow-up Plantigrade foot Plantigrade foot Left foot recurrence, plan for repeat soft-tissue release in future Plantigrade feet Plantigrade orthosis Plantigrade orthosis Plantigrade orthosis Plantigrade orthosis
foot. Night-time foot abduction feet. Night-time foot abduction feet. Night-time foot abduction feet. Part-time foot abduction
Multiple prior reports of clubfeet associated with ABS have emphasized the poor prognostic finding of an ipsilateral band. In the current series, ipsilateral banding location (proximal to ankle vs. distal to ankle vs. no ipsilateral banding) did not have a significant effect on the treatment of the clubfoot by the Ponseti technique. The number of casts (7.0 vs. 6.4 vs. 4.6), initial correction rate (88% vs. 100% vs. 100%), and recurrence rate (38% vs. 21% vs. 33%) were similar. Our study demonstrates that the presence of ipsilateral bands should not deter physicians from using the Ponseti technique in patients with clubfoot associated with ABS. Further, a clubfoot with a neurological deficit secondary to an ipsilateral band has been reported as a significant hindrance in the ability to treat an ABS clubfoot. Henningan and Kuo2 documented an average of 3.7 surgeries to achieve correction in these feet that were associated with neurological deficits. Tada et al3 theorized that bands causing neurological deficits resulted in a more rigid and cast-resistant, “paralytic” clubfoot. They had 10 “paralytic” clubfeet in their series and all that required soft-tissue releases and tendon transfers.3 There were 2 patients in our series who had an ipsilateral neurological deficit associated with a band, one of which was successfully treated with casting. Although the sample size for the patients with neurological deficits is small, the successful treatment of this patient demonstrates the possibility of correcting clubfeet with a neurological deficit from an ipsilateral band with the Ponseti technique.
TABLE 3. Effect of Ipsilateral Clubfoot Banding Band Location Proximal to ankle Distal to ankle No ipsilateral banding
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No. Feet [n/N (%)]
Average Casts
Achilles Tenotomy [n/N (%)]
Recurrence [n/N (%)]
8/21 (38) 14/21 (67) 3/21 (14)
7.0 6.4 4.6
6/8 (75) 13/14 (93) 1/3 (33)
3/8 (38) 3/14 (21) 1/3 (33)
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The limitations of this study are reflective of the retrospective design. Standardized clubfoot severity scores are not available for ABS and exact band location and depth were not recorded in all charts. Follow-up is still ongoing and recurrence rates may change as time increases, especially in those patients that are still in bracing treatment. There are no objective tests, functional measures, or patient-reported outcome measures to assess the success of treatment, solely chart documentation of a corrected foot. Neurological deficits were based on documented physical examination as in previous studies,2 but more objective measures of specific muscle function with electromyogram or nerve conduction testing may provide insight as to why one foot may respond to casting while another does not. In addition, while this study represents the largest series of ABS clubfeet thus far in the literature, it still suffers from being an overall small sample size. In conclusion, this study is the largest series of ABS clubfoot patients to date treated by the Ponseti technique and demonstrates its effectiveness at achieving initial correction without surgery. The recurrence rate of 33% is an improvement over historical literature, and serial casting avoided extensive soft-tissue releases in all but one patient. The presence of an ipsilateral band did not affect the success rate of Ponseti casting and the technique has success even in a clubfoot with a neurological deficit. The Ponseti technique is an effective first-line treatment in patients who have clubfeet associated with ABS. REFERENCES 1. Kino Y. Clinical and experimental studies of the congenital constriction band syndrome, with an emphasis on its etiology. J Bone Joint Surg Am. 1975;57:636–643. 2. Hennigan SP, Kuo KN. Resistant talipes equinovarus associated with congenital constriction band syndrome. J Pediatr Orthop. 2000; 20:240–245.
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3. Tada K, Yonenobu K, Swanson AB. Congenital constriction band syndrome. J Pediatr Orthop. 1984;4:726–730. 4. Cowell HR, Hensinger RN. The relationship of clubfoot to congenital annular bands. In: Bateman J, ed. Foot Science. Philadelphia: WB Saunders; 1976:41–46. 5. Patterson TJ. Congenital ring-constrictions. Br J Plast Surg. 1961; 14:1–31. 6. Askins G, Ger E. Congenital constriction band syndrome. J Pediatr Orthop. 1988;8:461–466. 7. Foulkes GD, Reinker K. Congenital constriction band syndrome: a seventy-year experience. J Pediatr Orthop. 1994;14:242–248. 8. Walter JH Jr, Goss LR, Lazzara AT. Amniotic band syndrome. J Foot Ankle Surg. 1998;37:325–333. 9. Gomez VR. Clubfeet in congenital annular constricting bands. Clin Orthop Relat Res. 1996;323:155–162. 10. Chang CH, Huang SC. Clubfoot deformity in congenital constriction band syndrome: manifestations and treatment. J Formos Med Assoc. 1998;97:328–334. 11. Allington NJ, Kumar SJ, Guille JT. Clubfeet associated with congenital constriction bands of the ipsilateral lower extremity. J Pediatr Orthop. 1995;15:599–603. 12. Greene WB. One-stage release of congenital circumferential constriction bands. J Bone Joint Surg Am. 1993;75:650–655. 13. Morcuende JA, Dolan LA, Dietz FR, et al. Radical reduction in the rate of extensive corrective surgery for clubfoot using the Ponseti method. Pediatrics. 2004;113:376–380. 14. Zionts LE, Habell B. The use of the Ponseti method to treat clubfeet associated with congenital annular band syndrome. J Pediatr Orthop. 2013;33:563–568. 15. Moroney PJ, Noe¨l J, Fogarty EE, et al. A single-center prospective evaluation of the Ponseti method in nonidiopathic congenital talipes equinovarus. J Pediatr Orthop. 2012;32:636–640. 16. Janicki JA, Narayanan UG, Harvey B, et al. Treatment of neuromuscular and syndrome-associated (nonidiopathic) clubfeet using the Ponseti method. J Pediatr Orthop. 2009;29:393–397. 17. Gurnett CA, Boehm S, Connolly A, et al. Impact of congenital talipes equinovarus etiology on treatment outcomes. Dev Med Child Neurol. 2008;50:498–502. 18. Ozkan K, Unay K, Goksan B, et al. Congenital constriction ring syndrome with foot deformity: two case reports. Cases J. 2009;2: 6696. 19. Ponseti IV. Congenital Clubfoot Fundamentals of Treatment. Oxford: Oxford University Press; 1996.
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The Effectiveness of the Ponseti Method for Treating Clubfoot Associated With Amniotic Band Syndrome Aaron M. Carpiaux, MD,* Pooya Hosseinzadeh, MD,w Ryan D. Muchow, MD,z Henry J. Iwinski, MD,z Janet L. Walker, MD,z and Todd A. Milbrandt, MDz
Background: Amniotic band syndrome (ABS) is a congenital disorder with an associated incidence of clubfoot deformity in over 50% of patients. Although early reports in the literature demonstrated a poor response to casting treatments, recent application of the Ponseti technique in ABS patients have been more promising. Methods: A retrospective review of all patients with clubfoot and a concurrent diagnosis of ABS were reviewed at a single institution. Patients not managed initially with the Ponseti method were excluded. Data collected included patient age at presentation, sex, unilateral or bilateral, amniotic band location and associated findings, and response to treatment—number of casts and requirement of Achilles tenotomy, tibialis anterior tendon transfer, or other surgical procedures. Duration of treatment at latest follow-up visit was noted and outcome was based on clinical foot appearance and plan for any further procedures. Results: Twelve patients (7 female and 5 male) with a total of 21 feet (9 bilateral and 3 unilateral) were identified. The average age at presentation was 3 weeks (range, 1 to 9 wk). The average number of casts was 6 (range, 3 to 11). Seventeen of 21 feet (81.0%) underwent percutaneous Achilles tenotomy. The initial correction rate for all patients with the Ponseti technique was 20/21 feet (95.2%) and recurrence was noted in 7/21 feet (33.3%). One patient underwent primary posteromedial release and 2 patients had associated neurological deficits. The average follow-up was 3.9 years (range, 9 mo to 10 y) and all but one patient had supple, plantigrade feet. Conclusion: The Ponseti technique is an effective first-line treatment in patients who have clubfeet associated with ABS, including those with a neurological deficit. Level of Evidence: Level IV.
From the *Department of Orthopaedic Surgery and Sports Medicine, University of Kentucky; zShriners Hospital for Children, Lexington, KY; and wBaptist Pediatric Orthopaedics Center, Miami, FL. None of the above authors have received financial support for this study. The authors declare no conflicts of interest. Reprints: Aaron M. Carpiaux, MD, Department of Orthopaedic Surgery and Sports Medicine, University of Kentucky, 740 South Limestone Street, K401, Lexington, KY 40536. E-mail: aaron. [email protected]. Supplemental Digital Content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s Website, www. pedorthopaedics.com. Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.
284 | www.pedorthopaedics.com
Key Words: amniotic band syndrome, clubfoot, Ponseti method, casting, congenital annular band syndrome, talipes equinovarus (J Pediatr Orthop 2016;36:284–288)
A
mniotic band syndrome (ABS) is a congenital disorder believed to be caused by separation of the amnion from the chorion resulting in fibrous bands which can encircle limbs and lead to anomalies and amputation during fetal development.1 The resultant oligohydramnios from fetal membrane disruption seen in ABS patients is thought to be the link to the high incidence of concurrent clubfoot. Although anomalies of the hand and fingers are most common, there has been an associated incidence with clubfoot deformity in 12% to 56%.1–8 ABS clubfoot patients have often been grouped with other nonidiopathic clubfoot disorders, such as arthrogryposis and spina bifida, based on their poor response to conservative treatment with casting, requiring many to undergo comprehensive soft-tissue releases.2–4,6,9–12 The success of the Ponseti method at treating idiopathic clubfoot has recently been extended to the management of nonidiopathic clubfoot including ABS.13–18 Zionts and Habell published a report of the Ponseti technique in ABS patients successfully treating 6 clubfeet in 5 ABS patients with casting plus Achilles tenotomy. Four of the 6 clubfeet had ipsilateral bands and no neurological deficits were noted. One patient required a tibialis anterior tendon transfer (TATT) and none required extensive soft-tissue releases.14 Because of the rarity of ABS and clubfoot, as well as the relatively recent popularization of the Ponseti method as the standard of care in the treatment of clubfoot, these case series have remained small and the outcomes in these patients are still being elucidated. In addition, no recent series have analyzed band location or included clubfeet in ABS patients with an ipsilateral neurological deficit from banding, which were hypothesized by early authors to be even more rigid and resistant to nonoperative treatment.2,3,6,9,10 The purpose of this study, therefore, was to examine our cohort of ABS patients with clubfoot treated initially with the Ponseti method and compare treatment effectiveness to recent literature reports. Our hypothesis was that the Ponseti method would be an effective initial treatment with few patients requiring comprehensive softtissue releases. J Pediatr Orthop
Volume 36, Number 3, April/May 2016
Copyright r 2016 Wolters Kluwer Health, Inc. All rights reserved.
J Pediatr Orthop
Volume 36, Number 3, April/May 2016
METHODS An IRB-approved, retrospective review of all patients between January 2000 and December 2012 with concurrent diagnoses of clubfoot and ABS was performed. Patients were excluded if they had received any clubfoot treatment before presentation at our institution or if their primary treatment was not Ponseti casting. Data collected included patient age at presentation, sex, unilateral or bilateral, amniotic band location and associated findings, and response to treatment—number of casts and requirement of Achilles tenotomy, TATT, or other surgical procedures. Neurological deficits were defined as lack of active foot and/or ankle motion with response to stimulus at the initial visit or subsequent follow-up. Duration of treatment at latest follow-up visit was noted and outcome was based on clinical foot appearance and plan for any further procedures. Our institution’s clubfoot treatment algorithm initiates Ponseti casting at the initial patient visit with weekly cast changes, thereafter to sequentially correct the cavus, adductus, varus, and equinus deformities as previously described by Ponseti.19 Percutaneous Achilles tenotomy was performed in the operating room under sedation for patients with residual equinus deformity. Three months of full-time (23 h) foot abduction orthosis wear was then instituted to maintain deformity correction followed by part-time wear (night-time and naps, approximately 12 h) until age 3 or when the family decided to discontinue wear. Recurrence was defined by initiating any treatment—bracing, casting, or surgery—once a corrected, plantigrade foot had been achieved.
RESULTS Twelve patients (7 female and 5 male) with a total of 21 feet (9 bilateral, 3 unilateral) were identified and summarized in Table 1. The average age at presentation was 3 weeks (range, 1 to 9 wk). Lower extremity ABS involvement was found in 10 patients and upper extremity involvement was found in 10 patients (Fig. 1). Seven patients had ipsilateral bands of the lower extremity with clubfoot deformity and 2 of these (patients 3 and 7) had an associated neurological deficit, both with deep thigh bands and one with a calf band. Both patients had absent active ankle dorsiflexion and 1 patient also lacked ankle eversion on the affected side. The other patient underwent band release at birth for vascular compromise (Fig. 2). Other than the presence of an amniotic banding, the most common distal extremity finding was acrosyndactyly. The Appendix Table (Supplemental Digital Content 1, http://links.lww.com/BPO/A37) documents these additional findings and their non–clubfoot-related surgical interventions.
Ponseti Treatment The average number of casts was 6 (range, 3 to 11). Seventeen of 21 feet (81.0%) underwent percutaneous Achilles tenotomy. The initial correction rate for all patients with the Ponseti technique was 20/21 feet (95.2%) (Table 2). Copyright
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Ponseti Method and Amniotic Band Syndrome
Three patients had significantly different courses and they are described here: patient 7 had 5 serial casts placed bilaterally, without any improvement to the left foot. The left foot underwent an additional 4 rounds of casting before proceeding with posteromedial soft-tissue release because of a lack of correction. Casting was continued during this time on the right to maintain correction before Achilles tenotomy was performed concurrently with the left-sided procedure. Patient 11 achieved correction of bilateral clubfoot deformity after 5 casts with residual equinus but had 6 more casts to maintain position while waiting to overcome a respiratory syncytial virus infection before Achilles tenotomy. Bilateral calcaneal pins were placed into the calcaneal tuberosity in patient 8 at the same time as Achilles tenotomy to help achieve and hold plantigrade position during final casting. They were removed in clinic 3 weeks later with good correction.
Recurrence Seven feet were defined as recurrent at an average time of 3.7 years (recurrence rate = 7/21, 33.3%). All required surgical intervention. TATT was performed for those with evidence of dynamic muscle imbalance by gait examination and/or pedobaric foot pressure analysis, and further soft-tissue releases were performed for stiff, recurrent clubfoot deformity. Three patients underwent TATT, 1 patient was scheduled to have TATT but was lost to follow-up, and 1 patient was scheduled for revision posteromedial release. The average follow-up was 3.9 years (range, 9 mo to 10 y). Four patients were still in part-time foot abduction orthosis wear and 1 patient was lost to follow-up. The remaining 10 patients had supple, plantigrade feet (Table 2).
Amniotic Bands and Clubfoot Treatment course and recurrence were stratified by band location because of previous authors’ findings that proximal band location was associated with a more recalcitrant clubfoot.2 There were 18 feet with ipsilateral lower extremity amniotic bands and clubfeet. The remaining 3 feet were included as an internal control for comparison. The average number of casts did not differ between those with bands located proximal to the ankle, distal to the ankle, and those without banding (7.0 vs. 6.4 vs. 4.6 casts). The recurrence rate was similar between the 3 groups as well, 3/8 feet (38%) versus 3/14 feet (21%) versus 1/3 feet (33%). Ipsilateral banding did not affect the effectiveness of the Ponseti technique in the treatment of clubfeet associated with ABS (Table 3). There were 2 lower extremities with deep thigh bands resulting in a neurological deficit and associated with clubfoot. Patient 3 was found to have vascular compromise at birth from a left deep thigh band and underwent emergent release. No active ankle dorsiflexion was found on the left side at presentation but the patient responded well to casting and Achilles tenotomy, requiring no further clubfoot procedures and having a plantigrade foot at 7-year follow-up. The other patient www.pedorthopaedics.com |
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TABLE 1. Patient Data Patient Number
Sex
Age (wk)
1 2 3
Male Male Male
1 9 8
4 5 6 7
Female Female Male Female
1 1 3 1
8
Male
4
Bilateral No Bilateral No Left Yes, no ankle dorsiflexion on left Bilateral No Bilateral No Right No Bilateral Yes, no ankle dorsiflexion or eversion on left Bilateral No
9 10 11 12
Female Female Female Female
1 4 1 2
Left Bilateral Bilateral Bilateral
Side
Neurological Deficit
No No No No
failed Ponseti treatment and has had recurrence after the initial posteromedial release. Non–clubfoot-related procedures for ABS anomalies were quite common. Nine of 12 patients underwent or had plans for soft-tissue procedures to release bands and correct digital anomalies. Most of these procedures were performed in a staged manner under separate anesthetic events (Appendix Table, Supplemental Digital Content 1, http://links.lww.com/BPO/A37).
DISCUSSION Historically, clubfoot treatment associated with ABS has been difficult and often resulted in correction by extensive soft-tissue releases.2–4,6,9–12 This study supports the effectiveness of the Ponseti method for clubfoot correction by demonstrating its success in the largest series of ABS patients to date and the first to include patients with neurological deficits. Initial correction was able to be obtained in 20 of 21 patients (95.2%) treated by the
FIGURE 1. Patient 8—bilateral lower extremities. Baseline photograph at presentation demonstrating bilateral clubfoot deformity with left calf band. Note acrosyndactyly and partial toe amputations on both feet.
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Lower Extremity Findings Left deep calf band. Right midfoot band with acrosyndactyly. Acrosyndactyly of bilateral feet Bilateral multiple bands. Left calf band. Left deep thigh band released at birth for vascular compromise Left thigh band. Right foot acrosyndactyly with partial toe amputations Acrosyndactyly of bilateral feet with partial amputations of right toes None Left thigh deep band. Right foot band Left calf band. Acrosyndactyly of bilateral feet with partial toe amputations. Right thigh band. Bilateral leg bands. Partial amputations of left toes Acrosyndactyly of bilateral feet Bilateral calf partial bands. Partial amputations of bilateral toes None
Ponseti technique with 1 patient requiring extensive softtissue release. Further, 7 of the 21 feet (33.3%) required additional surgery for recurrence of the clubfoot deformity. Supple, plantigrade feet can be achieved with serial casting and surgical intervention is necessary only in resistant or recurrent cases. Serial casting before the popularization of the Ponseti technique had poor results at gaining correction without posteromedial release. Gomez9 was able to successfully treat 2 patients of 35 with ABS and clubfoot using serial casting; however, no casting technique was specified. Chang and Huang10 reported similar results in a series of 10 patients with ABS and clubfoot with only 1 achieving correction with casting and Achilles tenotomy alone. A retrospective review by Allington et al11 of 21 clubfeet with ipsilateral bands found that all but 2 patients required one or more surgical procedures for clubfoot correction. The Ponseti technique has demonstrated effectiveness in treating other difficult, nonidiopathic diagnoses including arthrogryposis, trisomy 21, and spina bifida prompting trial in ABS as well.14–18 Janicki and colleagues studied a cohort of 23 patients (40 feet) with nonidiopathic
FIGURE 2. Patient 3—left lower extremity. Postoperative photographs after Achilles tenotomy showing correction of equinus deformity. The patient also underwent concurrent Z-plasty of ipsilateral deep thigh and calf bands. Copyright
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J Pediatr Orthop
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Ponseti Method and Amniotic Band Syndrome
TABLE 2. Treatment and Outcomes Patient Number
No. Casts
Achilles Tenotomy
Followup
1 2 3
6 6 5
Yes Yes Yes
4 5 6 7
5—left 6—right 6 4 9
8
6
Yes—left No—right Yes Yes Yes—right NA—left Yes
9
3
No
10
4
Yes
None
2y
11
11
Yes
None
1y
12
5
No
None
9 mo
Other Clubfoot Surgeries Bilateral tibialis anterior tendon transfer Bilateral tibialis anterior tendon transfer None None
10 y 7y 7y 2y
None Right tibialis anterior tendon transfer Left posteromedial release
5y 4y 3y
Bilateral calcaneal pin insertion to assist casting None
3y
clubfeet, 3 of which had ABS and bilateral clubfeet treated with Ponseti. All 3 were successfully treated with casting and tenotomy with 1 patient’s recurrence managed with repeat casting.16 Ozkan and colleagues reviewed 2 patients with ABS and clubfoot with ipsilateral band and found successful deformity correction with 7 casts each and Achilles tenotomy. Follow-up averaged 1 year and both patients were still being managed with foot abduction orthosis wear without recurrence.18 Zionts and Habell14 have published the only other series of solely ABS patients treated successfully with the Ponseti technique. They described the treatment of 5 patients, 6 feet, who had achieved initial correction in all feet with an average of 6 casts. All received Achilles tenotomy and early recurrence was managed with repeat casting in 5 feet. TATT was performed in 1 foot (17%). The recurrence rate in our study was 33% (7/21 feet) with the most common procedure being TATT (5/7). All feet (6/6) in the Zionts and Habell’s study were plantigrade at most recent follow-up compared with 90% (19/21) in our series. The difference in average follow-up, 2.7 versus 3.9 years may explain differences in recurrence rate and the increased incidence of TATT in the current study. No patient under 3 years old underwent TATT in the current study. One patient in our study required extensive posterior release primarily, compared with zero in the Zionts and Habell’s study. There were no patients with neurological deficits in the Zionts and Habell’s series compared with 2 in the current.
2y
Outcomes No pain, plantigrade feet, active in sports No pain, plantigrade feet Plantigrade foot. Plan for epiphysiodesis in future for 4.5 cm leg length discrepancy, right > left Planned for tibialis anterior transfer on left, but lost to follow-up Plantigrade foot Plantigrade foot Left foot recurrence, plan for repeat soft-tissue release in future Plantigrade feet Plantigrade orthosis Plantigrade orthosis Plantigrade orthosis Plantigrade orthosis
foot. Night-time foot abduction feet. Night-time foot abduction feet. Night-time foot abduction feet. Part-time foot abduction
Multiple prior reports of clubfeet associated with ABS have emphasized the poor prognostic finding of an ipsilateral band. In the current series, ipsilateral banding location (proximal to ankle vs. distal to ankle vs. no ipsilateral banding) did not have a significant effect on the treatment of the clubfoot by the Ponseti technique. The number of casts (7.0 vs. 6.4 vs. 4.6), initial correction rate (88% vs. 100% vs. 100%), and recurrence rate (38% vs. 21% vs. 33%) were similar. Our study demonstrates that the presence of ipsilateral bands should not deter physicians from using the Ponseti technique in patients with clubfoot associated with ABS. Further, a clubfoot with a neurological deficit secondary to an ipsilateral band has been reported as a significant hindrance in the ability to treat an ABS clubfoot. Henningan and Kuo2 documented an average of 3.7 surgeries to achieve correction in these feet that were associated with neurological deficits. Tada et al3 theorized that bands causing neurological deficits resulted in a more rigid and cast-resistant, “paralytic” clubfoot. They had 10 “paralytic” clubfeet in their series and all that required soft-tissue releases and tendon transfers.3 There were 2 patients in our series who had an ipsilateral neurological deficit associated with a band, one of which was successfully treated with casting. Although the sample size for the patients with neurological deficits is small, the successful treatment of this patient demonstrates the possibility of correcting clubfeet with a neurological deficit from an ipsilateral band with the Ponseti technique.
TABLE 3. Effect of Ipsilateral Clubfoot Banding Band Location Proximal to ankle Distal to ankle No ipsilateral banding
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No. Feet [n/N (%)]
Average Casts
Achilles Tenotomy [n/N (%)]
Recurrence [n/N (%)]
8/21 (38) 14/21 (67) 3/21 (14)
7.0 6.4 4.6
6/8 (75) 13/14 (93) 1/3 (33)
3/8 (38) 3/14 (21) 1/3 (33)
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The limitations of this study are reflective of the retrospective design. Standardized clubfoot severity scores are not available for ABS and exact band location and depth were not recorded in all charts. Follow-up is still ongoing and recurrence rates may change as time increases, especially in those patients that are still in bracing treatment. There are no objective tests, functional measures, or patient-reported outcome measures to assess the success of treatment, solely chart documentation of a corrected foot. Neurological deficits were based on documented physical examination as in previous studies,2 but more objective measures of specific muscle function with electromyogram or nerve conduction testing may provide insight as to why one foot may respond to casting while another does not. In addition, while this study represents the largest series of ABS clubfeet thus far in the literature, it still suffers from being an overall small sample size. In conclusion, this study is the largest series of ABS clubfoot patients to date treated by the Ponseti technique and demonstrates its effectiveness at achieving initial correction without surgery. The recurrence rate of 33% is an improvement over historical literature, and serial casting avoided extensive soft-tissue releases in all but one patient. The presence of an ipsilateral band did not affect the success rate of Ponseti casting and the technique has success even in a clubfoot with a neurological deficit. The Ponseti technique is an effective first-line treatment in patients who have clubfeet associated with ABS. REFERENCES 1. Kino Y. Clinical and experimental studies of the congenital constriction band syndrome, with an emphasis on its etiology. J Bone Joint Surg Am. 1975;57:636–643. 2. Hennigan SP, Kuo KN. Resistant talipes equinovarus associated with congenital constriction band syndrome. J Pediatr Orthop. 2000; 20:240–245.
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3. Tada K, Yonenobu K, Swanson AB. Congenital constriction band syndrome. J Pediatr Orthop. 1984;4:726–730. 4. Cowell HR, Hensinger RN. The relationship of clubfoot to congenital annular bands. In: Bateman J, ed. Foot Science. Philadelphia: WB Saunders; 1976:41–46. 5. Patterson TJ. Congenital ring-constrictions. Br J Plast Surg. 1961; 14:1–31. 6. Askins G, Ger E. Congenital constriction band syndrome. J Pediatr Orthop. 1988;8:461–466. 7. Foulkes GD, Reinker K. Congenital constriction band syndrome: a seventy-year experience. J Pediatr Orthop. 1994;14:242–248. 8. Walter JH Jr, Goss LR, Lazzara AT. Amniotic band syndrome. J Foot Ankle Surg. 1998;37:325–333. 9. Gomez VR. Clubfeet in congenital annular constricting bands. Clin Orthop Relat Res. 1996;323:155–162. 10. Chang CH, Huang SC. Clubfoot deformity in congenital constriction band syndrome: manifestations and treatment. J Formos Med Assoc. 1998;97:328–334. 11. Allington NJ, Kumar SJ, Guille JT. Clubfeet associated with congenital constriction bands of the ipsilateral lower extremity. J Pediatr Orthop. 1995;15:599–603. 12. Greene WB. One-stage release of congenital circumferential constriction bands. J Bone Joint Surg Am. 1993;75:650–655. 13. Morcuende JA, Dolan LA, Dietz FR, et al. Radical reduction in the rate of extensive corrective surgery for clubfoot using the Ponseti method. Pediatrics. 2004;113:376–380. 14. Zionts LE, Habell B. The use of the Ponseti method to treat clubfeet associated with congenital annular band syndrome. J Pediatr Orthop. 2013;33:563–568. 15. Moroney PJ, Noe¨l J, Fogarty EE, et al. A single-center prospective evaluation of the Ponseti method in nonidiopathic congenital talipes equinovarus. J Pediatr Orthop. 2012;32:636–640. 16. Janicki JA, Narayanan UG, Harvey B, et al. Treatment of neuromuscular and syndrome-associated (nonidiopathic) clubfeet using the Ponseti method. J Pediatr Orthop. 2009;29:393–397. 17. Gurnett CA, Boehm S, Connolly A, et al. Impact of congenital talipes equinovarus etiology on treatment outcomes. Dev Med Child Neurol. 2008;50:498–502. 18. Ozkan K, Unay K, Goksan B, et al. Congenital constriction ring syndrome with foot deformity: two case reports. Cases J. 2009;2: 6696. 19. Ponseti IV. Congenital Clubfoot Fundamentals of Treatment. Oxford: Oxford University Press; 1996.
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