HAND/PERIPHERAL NERVE Evaluation of a Stepwise Surgical Approach to Camptodactyly Kristy L. Hamilton, M.D. David T. Netscher, M.D. Houston, Texas
Background: Camptodactyly is common, but its treatment remains controversial. Multiple deforming forces have been implicated in its pathogenesis. This study evaluates a logical clinical assessment and corresponding stepwise surgical plan. Methods: Eighteen consecutive fingers (12 children) had surgery to treat camptodactyly of the proximal interphalangeal joint at a mean age of 8 years (range, 9 months to 15 years). The little (n = 13), ring (n = 2), and middle fingers (n = 3) were involved. Mean preoperative flexion contracture was 57 degrees (range, 35 to 75 degrees). All digits had moderate to severe contracture with functional impairment and were offered surgery. Preoperative and postoperative active range of motion was recorded. The sequential treatment steps correspond to the clinical examination and potentially involve volar skin release with flap, fascial release, flexor digitorum superficialis tenotomy, sliding volar plate release, extension lag correction, and Fowler extensor tenotomy. Results: Mean postoperative flexion contracture resolved to 3 degrees (range, 0 to 25 degrees) at a mean follow-up of 11 months (range, 3 to 32 months). Mean proximal interphalangeal joint flexion was 88 degrees (range, 50 to 100 degrees). Fifteen of 18 fingers achieved full active proximal interphalangeal joint extension. The remaining digits had residual contractures of 5, 20, and 25 degrees. All digits had soft-tissue release with flap and flexor digitorum superficialis tenotomy, 16 had volar plate release, two had intrinsic transfers, and three had Fowler tenotomy release performed. Conclusions: This stepwise surgical approach effectively treats severe camptodactyly and appears to confirm the authors’ suspected pathogenesis of the disorder. Lumbricals and interossei were not involved. (Plast. Reconstr. Surg. 135: 568e, 2015.) CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.
C
amptodactyly is a condition defined by a persistent flexion contracture of the interphalangeal joints of the hand, with a reported 1 percent incidence.1 The proximal interphalangeal joints of the bilateral little fingers are most commonly affected, although involvement of all other fingers, other joints of the finger, and even the toes has been described.2–4 Camptodactyly presents in a bimodal fashion, with a cohort of patients in infancy and another of those entering From the Department of Orthopedic Surgery and the Division of Plastic Surgery, Baylor College of Medicine. Received for publication May 24, 2014; accepted September 9, 2014. Presented at the 2014 Annual Meeting of the American Association for Hand Surgery, in Kauai, Hawaii, January 8 through 11, 2014. Copyright © 2015 by the American Society of Plastic Surgeons DOI: 10.1097/PRS.0000000000000958
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puberty.5 Both of these periods are times of rapid growth. Despite it being a fairly common congenital hand disorder,1 the pathogenesis and treatment of camptodactyly are controversial.6 Although it is commonly believed that the soft tissues tether the finger, creating the deformity as the bone grows, there is no consensus regarding which structures Disclosure: The authors have no financial interest to declare in relation to the content of this article. Supplemental digital content is available for this article. Direct URL citations appear in the text; simply type the URL address into any Web browser to access this content. Clickable links to the material are provided in the HTML text of this article on the Journal’s Web site (www.PRSJournal.com).
www.PRSJournal.com
Volume 135, Number 3 • A Stepwise Approach to Camptodactyly cause the contracture nor what the underlying cause of the tethering itself may be.2,7,8 The skin, subcutaneous fascia, flexor tendons, lumbricals, interosseous muscles, extensor apparatus, and transverse and oblique retinacular ligaments have all been implicated, in addition to deformities of the phalangeal bones and joints themselves.3,8–10 With this in mind, we devised a stepwise surgical technique to release the restraining structures as necessary to passively extend the affected digit. Although other unifying theories have been proposed, our surgical and postoperative algorithm emphasizes logical integration of the clinical examination at every step, from the preoperative assessment to development of the tailored surgical plan and subsequent postoperative therapy. We hypothesized that by the time of presentation—regardless of the initial underlying cause— multiple structures were involved in patients with moderate to severe contracture and thus needed to be addressed individually. Our study focused on patients with the most severe presentation of simple camptodactyly and aggressively worsening deformity that resulted in a lack of function. Our experience with this protocol has produced either long-term resolution or major improvement of the flexion contracture, restoring function to patients’ hands.
PATIENTS AND METHODS We performed a review of 18 consecutively operated digits in 12 surgically treated patients with nonsyndromic, simple camptodactyly of the proximal interphalangeal joint. All patients referred to our private office, which receives tertiary hand surgery referrals, had failed nonoperative treatment (including stretching and splinting regimens) or presented with aggressively and rapidly worsening contracture, seen in the youngest of patients. All patients had notable loss of function, preventing them from participating in their activities of daily living and extracurricular activities—namely, the ability to play musical instruments, participate in sports, or even hold a brush.
The study consisted of five girls and eight boys, with an average age of 8 years (range, 9 months to 15 years) at the time of surgery. The youngest patients presented with the worst contractures that were progressing rapidly. Seven digits fell within the infantile category of camptodactyly (type I). The remaining 11 were categorized as the adolescent type (type II).11 All operations occurred between February of 2009 and February of 2013. The involved fingers were the little (n = 13), ring (n = 2), and middle (n = 3). None of the patients or their family members had been diagnosed with a syndrome involving camptodactyly. None of the patients had evidence of distal arthrogryposis or other associated conditions after a thorough physical examination. One patient had a paternally linked family history of little finger camptodactyly. All of the patients were otherwise healthy and developmentally normal. Preoperative and Postoperative Assessment Each finger was assessed clinically before and after surgery. The active range of motion of each finger was examined and measured from full active flexion to extension at the proximal interphalangeal, distal interphalangeal, and metacarpophalangeal joints. Mean preoperative flexion contracture was 57 degrees (range, 35 to 75 degrees) at the proximal interphalangeal joint. All patients presented with a definite skin deficit. The senior author (D.T.N.) recorded all measurements and performed each clinical assessment. All of the children who presented to us had digits with a moderate to severe degree of contracture. We do not operate on children with less than 30 degrees of contracture but will closely monitor those with rapidly progressive deformity. The clinical examination focused on three elements, each of which corresponded to a step in the surgical treatment (Table 1). (See Video, Supplemental Digital Content 1, which displays a three-step preoperative assessment. This video is available in the “Related Videos” section of the full-text article on PRSJournal.com or at http:// links.lww.com/PRS/B235.)
Table 1. Preoperative Assessment Guides the Surgical Plan Preoperative Assessment Presence of skin pterygium Flexion deformity with the MP joint extended Flexion deformity with the MP joint flexed PIP extensor lag by Bouvier blocking DIP extension with PIP flexed (boutonnière)
Corresponding Treatment Step Volar skin and fascia release FDS tenotomy Sliding volar plate release If mild: postoperative extension block splinting If severe: intrinsic transfer Fowler extensor tenotomy
MP, metacarpophalangeal; FDS, flexor digitorum profundus; PIP, proximal interphalangeal joint; DIP, distal interphalangeal joint.
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Plastic and Reconstructive Surgery • March 2015 interphalangeal joint to fully extend while the metacarpophalangeal joint is held in flexion. 3. Distal interphalangeal extension with the proximal interphalangeal joint flexed, assessing the presence of a boutonnière deformity.
Video 1. Supplemental Digital Content 1, which displays a three-step preoperative assessment, is available in the “Related Videos” section of the full-text article on PRSJournal.com or at http://links.lww.com/PRS/B235.
1. The degree of flexion deformity with the metacarpophalangeal joint both flexed and extended. This step reveals the presence of a soft-tissue pterygium and relative extrinsic flexor versus volar plate tightness. 2. Proximal interphalangeal extensor lag by Bouvier blocking maneuver to assess extensor central slip attenuation. This maneuver assesses the ability of the proximal
These patients were treated in a single private physician office and used well-established treatment protocols. In this setting, our study was exempt from institutional review board oversight. Patient consent to use their information was obtained and protected in accordance with Health Insurance Portability and Accountability Act compliance standards and the Declaration of Helsinki. Surgical Planning The patients are treated with a stepwise surgical approach to release tethering structures around the joint, which cause progressively more flexion as the bones of the hand grow.5 Soft tissues are released in a sequential order until the digit can be passively extended to its normal position. A maximum of six treatment steps are performed, of which the first three were always performed (Figs. 1 and 2). (See Video, Supplemental Digital Content 2, which displays the six following operative steps. This video is available in the
Fig. 1. Stepwise treatment steps. A local transposition flap is designed (above, left). The skin and soft tissues are released (above, right) followed by an flexor digitorum superficialis tenotomy (below, left) and sliding volar plate release (below, right).
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Volume 135, Number 3 • A Stepwise Approach to Camptodactyly
Fig. 2. Stepwise treatment steps: part II. The neurovascular bundles remain intact (above). The flap is mobilized and full-thickness skin grafting is planned (center). The digit is fully passively extensible at the end of surgery (below).
“Related Videos” section of the full-text article on PRSJournal.com or at http://links.lww.com/PRS/ B236.) 1. Release of the skin pterygium using Z-plasties or a local transposition flap and fullthickness skin graft. 2. Release of the underlying fascia and subcutaneous tissues. 3. Transverse flexor digitorum superficialis tenotomy at the level of the Camper chiasm. 4. Sliding volar plate release.
5. Correction of a proximal interphalangeal extension lag, if identified by the Bouvier maneuver. a. If mild, postoperative relative motion splinting is sufficient. b. If severe, an intrinsic transfer using the released flexor digitorum superficialis tendon is required.7 6. Fowler distal extensor tenotomy in the presence of a hyperextended distal interphalangeal joint.12
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Video 2. Supplemental Digital Content 2, which displays the six following operative steps, is available in the “Related Videos” section of the full-text article on PRSJournal.com or at http:// links.lww.com/PRS/B236.
It is important to note that patients who underwent additional step 4, 5, or 6 did so based on their clinical examination findings. It would be possible, therefore, to have a patient undergo Fowler tenotomy but not an intrinsic transfer. The additional steps are not necessarily progressive. Surgical Technique Approach the affected digit through a transverse proximal interphalangeal volar incision, incorporating an axial pattern Joshi transposition flap into the design.13 Alternatively, a midvolar longitudinal incision is used that is subsequently broken up into Z-plasties. Release the pterygium of skin and underlying soft tissue. The soft-tissue void will ultimately be augmented through Z-platies or the transposition flap and full-thickness skin graft from the ulnar edge of the hypothenar eminence. Release the fascia and subcutaneous tissues while preserving the ulnar and radial digital neurovascular bundles. Dissect down to the flexor tendon sheath, and open the sheath at the level of the A3 pulley. Retract the flexor digitorum profundus tendon to encounter the flexor digitorum superficialis tendon. Divide both slips of the flexor digitorum superficialis tendon transversely at the level of the Camper chiasm. This step should improve the flexion deformity. If it does not totally correct the deformity, proceed to the next step. The next structure to address is the volar plate. Tightness of the proximal interphalangeal joint volar plate was assessed preoperatively by flexing the metacarpophalangeal joint and passively extending the proximal interphalangeal joint. Divide the volar plate transversely as proximal as possible at the checkrein ligaments, and allow the plate to slide forward distally. The flexion contracture on
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passive manipulation should improve. It may be necessary to minimally take down the collateral ligaments to accomplish full finger extension. Based on the preoperative assessment with the Bouvier maneuver, an additional step may be required if the extensor mechanism appears to be severely attenuated. Those with a minor extension lag can be treated postoperatively with proximal phalanx extension block splinting or relative motion splinting. An intrinsic transfer into the lateral bands addresses a more major extension deficit and augments extension of the proximal interphalangeal joint, using the already divided flexor digitorum superficialis tendon. One slip of the flexor digitorum superficialis is simply excised to taper the tendon end. The flexor digitorum superficialis is passed from palmar to dorsal through the lumbrical canal and retrieved through a middorsal incision over the proximal phalanx. Finally, a Fowler extensor tenotomy may be required to treat digits with a stiff, hyperextended distal interphalangeal joint as seen in the traumatic boutonnière deformity. Postoperative Management The initial splint immobilizes the digit with the metacarpophalangeal joints flexed and the interphalangeal joints in extension. Range-ofmotion exercises are initiated 4 weeks after surgery. Standard soft-tissue management is used, using tissue massage, encouragement of adequate skin moisturization, and compression dressings or scar molds to address hypertrophic scarring, if indicated. All of our patients participated in hand therapy following surgery until their best result was obtained, often for as long as 3 months.
Video 3. Supplemental Digital Content 3, which shows a postoperative examination of the patient with the use of a proximal phalanx extension block splint, is available in the “Related Videos” section of the full-text article on PRSJournal.com or at http://links.lww.com/PRS/B237.
0 0 0 0 0 0 0 0 25 0 0 20 0 5 0 0 0 0 2.78
8 4 10 3 13 22 22 32 18 18 18 4.5 4 6 4.5 4.5 6.5 8 11.44
11 11 10 10 11 12 12 12 4 4 4 10 15 12 1 1 0.75 1 7.88
100 80 100 100 95 50 75 55 65 85 85 100 95 100 100 100 100 100 88.06
Yes Yes Yes Yes Yes Yes Yes No Yes Yes Yes Yes Yes Yes Yes No Yes Yes 88.89%
No No No No No Yes Yes Yes No No No No No No No No No No 16.67%
No No No No No Yes Yes No No No No No No No No No No No 11.11%
As with operative management, postoperative management is guided by the clinical examination and is essential for producing excellent outcomes. If there is persistent deformity at the proximal interphalangeal joint at this point, it is necessary to distinguish between an extension lag versus a residual flexion contracture. In the case of the former, a mild extension lag can be identified during preoperative assessment and successfully treated postoperatively with proximal phalanx extension block splinting or relative motion splinting by limiting the extension of the proximal phalanx at the metacarpophalangeal joint relative to the unaffected digits. (See Video, Supplemental Digital Content 3, which shows a postoperative examination of the patient with the use of a proximal phalanx extension block splint. This video is available in the “Related Videos” section of the full-text article on PRSJournal.com or at http://links.lww.com/PRS/B237.) This splint allows the extensor mechanism to tighten. For the latter, static splinting of the digit is used. For all digits, close attention is paid to ensure that full flexion is ultimately achieved. Some digits present with compensatory distal interphalangeal joint hyperextension or, following extensor Fowler tenotomy, a distal interphalangeal joint extension lag. The former is addressed by promoting the use of the flexor digitorum profundus with graduated dowel isometric grip exercises and dowel finger walking. Simple mallet splinting resolves the latter.
RESULTS
3 1 1 1 6 7 8 9
2 1 1 18
3 5
10 11 12 Total
2 1 3 4
ROM, range of motion; R, right; L, left.
60 70 55 35 60 55 35 55 70 55 35 65 60 75 55 60 60 60 56.67 R little L little R little L little L middle L little L ring R middle L little L ring L middle R little R little R little L little R little L little R little Average 1 1
Postoperative Preoperative Patient
1 2
Intrinsic Transfer Volar Plate Release Fowler Tenotomy Age at First Postoperative Surgery (yr) ROM (degrees)
FollowUp (mo) Contracture (degrees)
Location of Digits Operated On No. of Digits Operated On
Table 2. Clinical Assessment of Digits Affected by Camptodactyly before and after Surgery
Additional Surgical Steps Performed
Volume 135, Number 3 • A Stepwise Approach to Camptodactyly
Mean postoperative flexion contracture resolved to 3 degrees (range, 0 to 25 degrees) at a mean follow-up of 11 months (range, 3 to 32 months) (Table 2). Mean proximal interphalangeal joint flexion active arc of motion was 88 degrees (range, 50 to 100 degrees) (Fig. 3). (See Video Supplemental Digital Content 4, which displays the radiographs and final clinical examination after 6 months. This video is available in the “Related Videos” section of the full-text article on PRSJournal.com or at http:// links.lww.com/PRS/B238.) Fifteen of 18 digits could achieve full active proximal interphalangeal joint extension. The other three had residual contractures of 5, 20, and 25 degrees, respectively. Fifteen of 18 digits achieved full active range of motion at the proximal interphalangeal joint. No digit developed a recurrence of the flexion contracture, and there were no reported neurosensory deficits. No patient developed instability or laxity at the joint. No patients were lost to follow-up.
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Fig. 3. Postoperative range of motion. The patient retains a mild extension lag on the operated little finger that was noted on preoperative examination (above, left). The patient demonstrates full flexion (above, right). The patient used pencil-blocking exercises to successfully help central slip pull-through (below, left). The little finger is fully passively extensible (below, right).
Video 4. Supplemental Digital Content 4, which displays the radiographs and final clinical examination after 6 months, is available in the “Related Videos” section of the full-text article on PRSJournal.com or at http://links.lww.com/PRS/B238.
There was no significant difference found between the flexion contracture resolution in the infantile (type I) group versus the adolescent (type II group). The infantile group had a
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preoperative contracture of 56.4 degrees, which resolved to 3.6 degrees, compared with the adolescent group, which improved from 56.8 degrees to 2.3 degrees. All had soft-tissue release and flap coverage or Z-plasties, all had flexor digitorum superficialis tenotomy, 16 had volar plate release, two had intrinsic transfers, and three had Fowler release. Lumbricals and interossei did not appear to be the tethering structure in any patient, whereas flexor digitorum superficialis contributed to the flexion contracture in all cases. (See Video, Supplemental Digital Content 5, which shows intrinsic transfer and aberrant flexor digitorum superficialis insertion. This video is available in the “Related Videos” section of the full-text article on PRSJournal.com or at http://links.lww. com/PRS/B239.) The involved flexor digitorum superficialis tendon was seen to have aberrant adhesions to adjacent flexor digitorum superficialis tendons in the palm.
Volume 135, Number 3 • A Stepwise Approach to Camptodactyly
Video 5. Supplemental Digital Content 5, which shows intrinsic transfer and aberrant flexor digitorum superficialis insertion, is available in the “Related Videos” section of the full-text article on PRSJournal.com or at http://links.lww.com/PRS/B239.
DISCUSSION Despite the controversy surrounding the treatment and pathogenesis of camptodactyly,7 this stepwise surgical approach effectively addresses severe camptodactyly. By approaching camptodactyly as a spectrum of disease—rather than targeting one of many implicated structures—we were able to develop a comprehensive treatment algorithm that addresses many contributing structures. Tailoring treatment to the clinical examination in camptodactyly has been found to be more effective in improving surgical outcomes.14 Moreover, the very success of the algorithm appears to confirm our suspected pathogenesis of the disorder—that regardless of whether or not a single structure is at the root of camptodactyly, by the time of presentation, multiple structures are involved in the contracture. Indeed, patient by patient, there may very well be different underlying causes. However, they all ultimately present with a flexion contracture, and it is therefore logical to uniformly address all affected structures rather than to try to identify and treat a single one. The stepwise release protocol is indicated for digits with moderate to severe camptodactyly greater than 30 degrees or in patients with rapidly progressive disease.15 These digits have not responded to splinting and are beginning to or already demonstrate radiographic changes in the involved joints. Surgery is indicated to prevent long-term, irreversible articular deformity. The patient—or the patient’s caregivers—must be motivated to regularly stretch the operated digit after surgery or risk a recurrence of the flexion contracture secondary to scar tissue formation. Relative motion extension blocking splinting of
the metacarpophalangeal joint may need to be encouraged in postoperative treatment to enhance pull-though at the central slip and dorsal tightening of the lateral bands of the proximal interphalangeal joint. Strengths of our study include the encompassing surgical technique resulting in successful improvement of the flexion contracture at an average follow-up of 11 months. Although other studies have looked at unifying theories, the strength of our study lies in the algorithm emphasizing the use of the clinical examination at each stage: preoperatively for surgical planning, execution of the surgical procedure itself, and postoperatively in the development of an appropriate hand therapy regimen. Although digits with camptodactyly certainly share anatomical features, the presentation of each digit is distinct and therefore merits its own tailored surgical plan and postoperative therapy. It is this guiding principle that we believe has led to our successful results. The study was limited by the smaller cohort size and mean follow-up time. Future directions include long-term follow-up of these patients and the ongoing recruitment of a larger study group. In addition, it will be important to follow the infantile group (type 1) when its members enter adolescence to further evaluate the longevity of the surgical correction during this period of rapid growth. In our series of patients, we have observed almost complete resolution of the flexion contractures from camptodactyly following surgical treatment. In patients with severe camptodactyly, we therefore recommend this stepwise surgery as a definitive treatment in patients who have failed splinting therapy. David T. Netscher, M.D. 6624 Fannin Street, Suite 2730 Houston, Texas 77030 [email protected]
references 1. Jones KG, Marmor L, Lankford LL. An overview on new procedures in surgery of the hand. Clin Orthop Relat Res. 1974;99:154–167. 2. Engber WB, Flatt AE. Camptodactyly: An analysis of sixtysix patients and twenty-four operations. J Hand Surg Am. 1997;2:216–224. 3. Courtemanche AD. Campylodactyly: Etiology and management. Plast Reconstr Surg. 1969;44:451–454. 4. Hamilton KL, Netscher DT. Multidigit camptodactyly of the hands and feet: A case study. Hand (N Y) 2013;8:324–329. 5. Barinka L. Campylodactylia (second communication). Acta Chir Plast. 1964;6:154–151.
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Plastic and Reconstructive Surgery • March 2015 6. Smith PJ, Grobbelaar AO. Camptodactyly: A unifying theory and approach to surgical treatment. J Hand Surg Am. 1998;23:14–19. 7. McFarlane RM, Curry GI, Evans HB. Anomalies of the intrinsic muscles in camptodactyly. J Hand Surg Am. 1983;8:531–544. 8. Smith RJ, Kaplan EB. Camptodactyly and similar atraumatic flexion deformities of the proximal interphalangeal joints of the fingers. J Bone Joint Surg Am. 1968:50:1187–203. 9. Stoddard EE. Nomenclature of hereditary crooked fingers. J Hered. 1939;30:511–512. 10. Millesi H. Camptodactyly. In: Symposium on Reconstructive Hand Surgery. Vol 9. St. Louis: Mosby; 1974:175–177.
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11. Benson LS. Camptodactyly: Classification and results of nonoperative treatment. J Pediatr Orthop. 1994;14:814–819. 12. Lucas GL. Fowler central slip tenotomy for old mallet deformity. Plast Reconstr Surg. 1987;80:92–94. 13. Joshi BB. Dorsolateral flap from same finger to relieve flexion contracture. Plast Reconstr Surg. 1972;49:186–189. 14. Foucher G, Lorea P, Khouri RK, et al. Camptodactyly as a spectrum of congenital deficiencies: A treatment algorithm based on clinical examination. Plast Reconstr Surg. 2006;117:1897–1905. 15. Siegert JJ, Cooney WP, Dobyns JH. Management of simple camptodactyly. J Hand Surg Br. 1990;15:181–189.
Background: Camptodactyly is common, but its treatment remains controversial. Multiple deforming forces have been implicated in its pathogenesis. This study evaluates a logical clinical assessment and corresponding stepwise surgical plan. Methods: Eighteen consecutive fingers (12 children) had surgery to treat camptodactyly of the proximal interphalangeal joint at a mean age of 8 years (range, 9 months to 15 years). The little (n = 13), ring (n = 2), and middle fingers (n = 3) were involved. Mean preoperative flexion contracture was 57 degrees (range, 35 to 75 degrees). All digits had moderate to severe contracture with functional impairment and were offered surgery. Preoperative and postoperative active range of motion was recorded. The sequential treatment steps correspond to the clinical examination and potentially involve volar skin release with flap, fascial release, flexor digitorum superficialis tenotomy, sliding volar plate release, extension lag correction, and Fowler extensor tenotomy. Results: Mean postoperative flexion contracture resolved to 3 degrees (range, 0 to 25 degrees) at a mean follow-up of 11 months (range, 3 to 32 months). Mean proximal interphalangeal joint flexion was 88 degrees (range, 50 to 100 degrees). Fifteen of 18 fingers achieved full active proximal interphalangeal joint extension. The remaining digits had residual contractures of 5, 20, and 25 degrees. All digits had soft-tissue release with flap and flexor digitorum superficialis tenotomy, 16 had volar plate release, two had intrinsic transfers, and three had Fowler tenotomy release performed. Conclusions: This stepwise surgical approach effectively treats severe camptodactyly and appears to confirm the authors’ suspected pathogenesis of the disorder. Lumbricals and interossei were not involved. (Plast. Reconstr. Surg. 135: 568e, 2015.) CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.
C
amptodactyly is a condition defined by a persistent flexion contracture of the interphalangeal joints of the hand, with a reported 1 percent incidence.1 The proximal interphalangeal joints of the bilateral little fingers are most commonly affected, although involvement of all other fingers, other joints of the finger, and even the toes has been described.2–4 Camptodactyly presents in a bimodal fashion, with a cohort of patients in infancy and another of those entering From the Department of Orthopedic Surgery and the Division of Plastic Surgery, Baylor College of Medicine. Received for publication May 24, 2014; accepted September 9, 2014. Presented at the 2014 Annual Meeting of the American Association for Hand Surgery, in Kauai, Hawaii, January 8 through 11, 2014. Copyright © 2015 by the American Society of Plastic Surgeons DOI: 10.1097/PRS.0000000000000958
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puberty.5 Both of these periods are times of rapid growth. Despite it being a fairly common congenital hand disorder,1 the pathogenesis and treatment of camptodactyly are controversial.6 Although it is commonly believed that the soft tissues tether the finger, creating the deformity as the bone grows, there is no consensus regarding which structures Disclosure: The authors have no financial interest to declare in relation to the content of this article. Supplemental digital content is available for this article. Direct URL citations appear in the text; simply type the URL address into any Web browser to access this content. Clickable links to the material are provided in the HTML text of this article on the Journal’s Web site (www.PRSJournal.com).
www.PRSJournal.com
Volume 135, Number 3 • A Stepwise Approach to Camptodactyly cause the contracture nor what the underlying cause of the tethering itself may be.2,7,8 The skin, subcutaneous fascia, flexor tendons, lumbricals, interosseous muscles, extensor apparatus, and transverse and oblique retinacular ligaments have all been implicated, in addition to deformities of the phalangeal bones and joints themselves.3,8–10 With this in mind, we devised a stepwise surgical technique to release the restraining structures as necessary to passively extend the affected digit. Although other unifying theories have been proposed, our surgical and postoperative algorithm emphasizes logical integration of the clinical examination at every step, from the preoperative assessment to development of the tailored surgical plan and subsequent postoperative therapy. We hypothesized that by the time of presentation—regardless of the initial underlying cause— multiple structures were involved in patients with moderate to severe contracture and thus needed to be addressed individually. Our study focused on patients with the most severe presentation of simple camptodactyly and aggressively worsening deformity that resulted in a lack of function. Our experience with this protocol has produced either long-term resolution or major improvement of the flexion contracture, restoring function to patients’ hands.
PATIENTS AND METHODS We performed a review of 18 consecutively operated digits in 12 surgically treated patients with nonsyndromic, simple camptodactyly of the proximal interphalangeal joint. All patients referred to our private office, which receives tertiary hand surgery referrals, had failed nonoperative treatment (including stretching and splinting regimens) or presented with aggressively and rapidly worsening contracture, seen in the youngest of patients. All patients had notable loss of function, preventing them from participating in their activities of daily living and extracurricular activities—namely, the ability to play musical instruments, participate in sports, or even hold a brush.
The study consisted of five girls and eight boys, with an average age of 8 years (range, 9 months to 15 years) at the time of surgery. The youngest patients presented with the worst contractures that were progressing rapidly. Seven digits fell within the infantile category of camptodactyly (type I). The remaining 11 were categorized as the adolescent type (type II).11 All operations occurred between February of 2009 and February of 2013. The involved fingers were the little (n = 13), ring (n = 2), and middle (n = 3). None of the patients or their family members had been diagnosed with a syndrome involving camptodactyly. None of the patients had evidence of distal arthrogryposis or other associated conditions after a thorough physical examination. One patient had a paternally linked family history of little finger camptodactyly. All of the patients were otherwise healthy and developmentally normal. Preoperative and Postoperative Assessment Each finger was assessed clinically before and after surgery. The active range of motion of each finger was examined and measured from full active flexion to extension at the proximal interphalangeal, distal interphalangeal, and metacarpophalangeal joints. Mean preoperative flexion contracture was 57 degrees (range, 35 to 75 degrees) at the proximal interphalangeal joint. All patients presented with a definite skin deficit. The senior author (D.T.N.) recorded all measurements and performed each clinical assessment. All of the children who presented to us had digits with a moderate to severe degree of contracture. We do not operate on children with less than 30 degrees of contracture but will closely monitor those with rapidly progressive deformity. The clinical examination focused on three elements, each of which corresponded to a step in the surgical treatment (Table 1). (See Video, Supplemental Digital Content 1, which displays a three-step preoperative assessment. This video is available in the “Related Videos” section of the full-text article on PRSJournal.com or at http:// links.lww.com/PRS/B235.)
Table 1. Preoperative Assessment Guides the Surgical Plan Preoperative Assessment Presence of skin pterygium Flexion deformity with the MP joint extended Flexion deformity with the MP joint flexed PIP extensor lag by Bouvier blocking DIP extension with PIP flexed (boutonnière)
Corresponding Treatment Step Volar skin and fascia release FDS tenotomy Sliding volar plate release If mild: postoperative extension block splinting If severe: intrinsic transfer Fowler extensor tenotomy
MP, metacarpophalangeal; FDS, flexor digitorum profundus; PIP, proximal interphalangeal joint; DIP, distal interphalangeal joint.
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Plastic and Reconstructive Surgery • March 2015 interphalangeal joint to fully extend while the metacarpophalangeal joint is held in flexion. 3. Distal interphalangeal extension with the proximal interphalangeal joint flexed, assessing the presence of a boutonnière deformity.
Video 1. Supplemental Digital Content 1, which displays a three-step preoperative assessment, is available in the “Related Videos” section of the full-text article on PRSJournal.com or at http://links.lww.com/PRS/B235.
1. The degree of flexion deformity with the metacarpophalangeal joint both flexed and extended. This step reveals the presence of a soft-tissue pterygium and relative extrinsic flexor versus volar plate tightness. 2. Proximal interphalangeal extensor lag by Bouvier blocking maneuver to assess extensor central slip attenuation. This maneuver assesses the ability of the proximal
These patients were treated in a single private physician office and used well-established treatment protocols. In this setting, our study was exempt from institutional review board oversight. Patient consent to use their information was obtained and protected in accordance with Health Insurance Portability and Accountability Act compliance standards and the Declaration of Helsinki. Surgical Planning The patients are treated with a stepwise surgical approach to release tethering structures around the joint, which cause progressively more flexion as the bones of the hand grow.5 Soft tissues are released in a sequential order until the digit can be passively extended to its normal position. A maximum of six treatment steps are performed, of which the first three were always performed (Figs. 1 and 2). (See Video, Supplemental Digital Content 2, which displays the six following operative steps. This video is available in the
Fig. 1. Stepwise treatment steps. A local transposition flap is designed (above, left). The skin and soft tissues are released (above, right) followed by an flexor digitorum superficialis tenotomy (below, left) and sliding volar plate release (below, right).
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Volume 135, Number 3 • A Stepwise Approach to Camptodactyly
Fig. 2. Stepwise treatment steps: part II. The neurovascular bundles remain intact (above). The flap is mobilized and full-thickness skin grafting is planned (center). The digit is fully passively extensible at the end of surgery (below).
“Related Videos” section of the full-text article on PRSJournal.com or at http://links.lww.com/PRS/ B236.) 1. Release of the skin pterygium using Z-plasties or a local transposition flap and fullthickness skin graft. 2. Release of the underlying fascia and subcutaneous tissues. 3. Transverse flexor digitorum superficialis tenotomy at the level of the Camper chiasm. 4. Sliding volar plate release.
5. Correction of a proximal interphalangeal extension lag, if identified by the Bouvier maneuver. a. If mild, postoperative relative motion splinting is sufficient. b. If severe, an intrinsic transfer using the released flexor digitorum superficialis tendon is required.7 6. Fowler distal extensor tenotomy in the presence of a hyperextended distal interphalangeal joint.12
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Video 2. Supplemental Digital Content 2, which displays the six following operative steps, is available in the “Related Videos” section of the full-text article on PRSJournal.com or at http:// links.lww.com/PRS/B236.
It is important to note that patients who underwent additional step 4, 5, or 6 did so based on their clinical examination findings. It would be possible, therefore, to have a patient undergo Fowler tenotomy but not an intrinsic transfer. The additional steps are not necessarily progressive. Surgical Technique Approach the affected digit through a transverse proximal interphalangeal volar incision, incorporating an axial pattern Joshi transposition flap into the design.13 Alternatively, a midvolar longitudinal incision is used that is subsequently broken up into Z-plasties. Release the pterygium of skin and underlying soft tissue. The soft-tissue void will ultimately be augmented through Z-platies or the transposition flap and full-thickness skin graft from the ulnar edge of the hypothenar eminence. Release the fascia and subcutaneous tissues while preserving the ulnar and radial digital neurovascular bundles. Dissect down to the flexor tendon sheath, and open the sheath at the level of the A3 pulley. Retract the flexor digitorum profundus tendon to encounter the flexor digitorum superficialis tendon. Divide both slips of the flexor digitorum superficialis tendon transversely at the level of the Camper chiasm. This step should improve the flexion deformity. If it does not totally correct the deformity, proceed to the next step. The next structure to address is the volar plate. Tightness of the proximal interphalangeal joint volar plate was assessed preoperatively by flexing the metacarpophalangeal joint and passively extending the proximal interphalangeal joint. Divide the volar plate transversely as proximal as possible at the checkrein ligaments, and allow the plate to slide forward distally. The flexion contracture on
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passive manipulation should improve. It may be necessary to minimally take down the collateral ligaments to accomplish full finger extension. Based on the preoperative assessment with the Bouvier maneuver, an additional step may be required if the extensor mechanism appears to be severely attenuated. Those with a minor extension lag can be treated postoperatively with proximal phalanx extension block splinting or relative motion splinting. An intrinsic transfer into the lateral bands addresses a more major extension deficit and augments extension of the proximal interphalangeal joint, using the already divided flexor digitorum superficialis tendon. One slip of the flexor digitorum superficialis is simply excised to taper the tendon end. The flexor digitorum superficialis is passed from palmar to dorsal through the lumbrical canal and retrieved through a middorsal incision over the proximal phalanx. Finally, a Fowler extensor tenotomy may be required to treat digits with a stiff, hyperextended distal interphalangeal joint as seen in the traumatic boutonnière deformity. Postoperative Management The initial splint immobilizes the digit with the metacarpophalangeal joints flexed and the interphalangeal joints in extension. Range-ofmotion exercises are initiated 4 weeks after surgery. Standard soft-tissue management is used, using tissue massage, encouragement of adequate skin moisturization, and compression dressings or scar molds to address hypertrophic scarring, if indicated. All of our patients participated in hand therapy following surgery until their best result was obtained, often for as long as 3 months.
Video 3. Supplemental Digital Content 3, which shows a postoperative examination of the patient with the use of a proximal phalanx extension block splint, is available in the “Related Videos” section of the full-text article on PRSJournal.com or at http://links.lww.com/PRS/B237.
0 0 0 0 0 0 0 0 25 0 0 20 0 5 0 0 0 0 2.78
8 4 10 3 13 22 22 32 18 18 18 4.5 4 6 4.5 4.5 6.5 8 11.44
11 11 10 10 11 12 12 12 4 4 4 10 15 12 1 1 0.75 1 7.88
100 80 100 100 95 50 75 55 65 85 85 100 95 100 100 100 100 100 88.06
Yes Yes Yes Yes Yes Yes Yes No Yes Yes Yes Yes Yes Yes Yes No Yes Yes 88.89%
No No No No No Yes Yes Yes No No No No No No No No No No 16.67%
No No No No No Yes Yes No No No No No No No No No No No 11.11%
As with operative management, postoperative management is guided by the clinical examination and is essential for producing excellent outcomes. If there is persistent deformity at the proximal interphalangeal joint at this point, it is necessary to distinguish between an extension lag versus a residual flexion contracture. In the case of the former, a mild extension lag can be identified during preoperative assessment and successfully treated postoperatively with proximal phalanx extension block splinting or relative motion splinting by limiting the extension of the proximal phalanx at the metacarpophalangeal joint relative to the unaffected digits. (See Video, Supplemental Digital Content 3, which shows a postoperative examination of the patient with the use of a proximal phalanx extension block splint. This video is available in the “Related Videos” section of the full-text article on PRSJournal.com or at http://links.lww.com/PRS/B237.) This splint allows the extensor mechanism to tighten. For the latter, static splinting of the digit is used. For all digits, close attention is paid to ensure that full flexion is ultimately achieved. Some digits present with compensatory distal interphalangeal joint hyperextension or, following extensor Fowler tenotomy, a distal interphalangeal joint extension lag. The former is addressed by promoting the use of the flexor digitorum profundus with graduated dowel isometric grip exercises and dowel finger walking. Simple mallet splinting resolves the latter.
RESULTS
3 1 1 1 6 7 8 9
2 1 1 18
3 5
10 11 12 Total
2 1 3 4
ROM, range of motion; R, right; L, left.
60 70 55 35 60 55 35 55 70 55 35 65 60 75 55 60 60 60 56.67 R little L little R little L little L middle L little L ring R middle L little L ring L middle R little R little R little L little R little L little R little Average 1 1
Postoperative Preoperative Patient
1 2
Intrinsic Transfer Volar Plate Release Fowler Tenotomy Age at First Postoperative Surgery (yr) ROM (degrees)
FollowUp (mo) Contracture (degrees)
Location of Digits Operated On No. of Digits Operated On
Table 2. Clinical Assessment of Digits Affected by Camptodactyly before and after Surgery
Additional Surgical Steps Performed
Volume 135, Number 3 • A Stepwise Approach to Camptodactyly
Mean postoperative flexion contracture resolved to 3 degrees (range, 0 to 25 degrees) at a mean follow-up of 11 months (range, 3 to 32 months) (Table 2). Mean proximal interphalangeal joint flexion active arc of motion was 88 degrees (range, 50 to 100 degrees) (Fig. 3). (See Video Supplemental Digital Content 4, which displays the radiographs and final clinical examination after 6 months. This video is available in the “Related Videos” section of the full-text article on PRSJournal.com or at http:// links.lww.com/PRS/B238.) Fifteen of 18 digits could achieve full active proximal interphalangeal joint extension. The other three had residual contractures of 5, 20, and 25 degrees, respectively. Fifteen of 18 digits achieved full active range of motion at the proximal interphalangeal joint. No digit developed a recurrence of the flexion contracture, and there were no reported neurosensory deficits. No patient developed instability or laxity at the joint. No patients were lost to follow-up.
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Fig. 3. Postoperative range of motion. The patient retains a mild extension lag on the operated little finger that was noted on preoperative examination (above, left). The patient demonstrates full flexion (above, right). The patient used pencil-blocking exercises to successfully help central slip pull-through (below, left). The little finger is fully passively extensible (below, right).
Video 4. Supplemental Digital Content 4, which displays the radiographs and final clinical examination after 6 months, is available in the “Related Videos” section of the full-text article on PRSJournal.com or at http://links.lww.com/PRS/B238.
There was no significant difference found between the flexion contracture resolution in the infantile (type I) group versus the adolescent (type II group). The infantile group had a
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preoperative contracture of 56.4 degrees, which resolved to 3.6 degrees, compared with the adolescent group, which improved from 56.8 degrees to 2.3 degrees. All had soft-tissue release and flap coverage or Z-plasties, all had flexor digitorum superficialis tenotomy, 16 had volar plate release, two had intrinsic transfers, and three had Fowler release. Lumbricals and interossei did not appear to be the tethering structure in any patient, whereas flexor digitorum superficialis contributed to the flexion contracture in all cases. (See Video, Supplemental Digital Content 5, which shows intrinsic transfer and aberrant flexor digitorum superficialis insertion. This video is available in the “Related Videos” section of the full-text article on PRSJournal.com or at http://links.lww. com/PRS/B239.) The involved flexor digitorum superficialis tendon was seen to have aberrant adhesions to adjacent flexor digitorum superficialis tendons in the palm.
Volume 135, Number 3 • A Stepwise Approach to Camptodactyly
Video 5. Supplemental Digital Content 5, which shows intrinsic transfer and aberrant flexor digitorum superficialis insertion, is available in the “Related Videos” section of the full-text article on PRSJournal.com or at http://links.lww.com/PRS/B239.
DISCUSSION Despite the controversy surrounding the treatment and pathogenesis of camptodactyly,7 this stepwise surgical approach effectively addresses severe camptodactyly. By approaching camptodactyly as a spectrum of disease—rather than targeting one of many implicated structures—we were able to develop a comprehensive treatment algorithm that addresses many contributing structures. Tailoring treatment to the clinical examination in camptodactyly has been found to be more effective in improving surgical outcomes.14 Moreover, the very success of the algorithm appears to confirm our suspected pathogenesis of the disorder—that regardless of whether or not a single structure is at the root of camptodactyly, by the time of presentation, multiple structures are involved in the contracture. Indeed, patient by patient, there may very well be different underlying causes. However, they all ultimately present with a flexion contracture, and it is therefore logical to uniformly address all affected structures rather than to try to identify and treat a single one. The stepwise release protocol is indicated for digits with moderate to severe camptodactyly greater than 30 degrees or in patients with rapidly progressive disease.15 These digits have not responded to splinting and are beginning to or already demonstrate radiographic changes in the involved joints. Surgery is indicated to prevent long-term, irreversible articular deformity. The patient—or the patient’s caregivers—must be motivated to regularly stretch the operated digit after surgery or risk a recurrence of the flexion contracture secondary to scar tissue formation. Relative motion extension blocking splinting of
the metacarpophalangeal joint may need to be encouraged in postoperative treatment to enhance pull-though at the central slip and dorsal tightening of the lateral bands of the proximal interphalangeal joint. Strengths of our study include the encompassing surgical technique resulting in successful improvement of the flexion contracture at an average follow-up of 11 months. Although other studies have looked at unifying theories, the strength of our study lies in the algorithm emphasizing the use of the clinical examination at each stage: preoperatively for surgical planning, execution of the surgical procedure itself, and postoperatively in the development of an appropriate hand therapy regimen. Although digits with camptodactyly certainly share anatomical features, the presentation of each digit is distinct and therefore merits its own tailored surgical plan and postoperative therapy. It is this guiding principle that we believe has led to our successful results. The study was limited by the smaller cohort size and mean follow-up time. Future directions include long-term follow-up of these patients and the ongoing recruitment of a larger study group. In addition, it will be important to follow the infantile group (type 1) when its members enter adolescence to further evaluate the longevity of the surgical correction during this period of rapid growth. In our series of patients, we have observed almost complete resolution of the flexion contractures from camptodactyly following surgical treatment. In patients with severe camptodactyly, we therefore recommend this stepwise surgery as a definitive treatment in patients who have failed splinting therapy. David T. Netscher, M.D. 6624 Fannin Street, Suite 2730 Houston, Texas 77030 [email protected]
references 1. Jones KG, Marmor L, Lankford LL. An overview on new procedures in surgery of the hand. Clin Orthop Relat Res. 1974;99:154–167. 2. Engber WB, Flatt AE. Camptodactyly: An analysis of sixtysix patients and twenty-four operations. J Hand Surg Am. 1997;2:216–224. 3. Courtemanche AD. Campylodactyly: Etiology and management. Plast Reconstr Surg. 1969;44:451–454. 4. Hamilton KL, Netscher DT. Multidigit camptodactyly of the hands and feet: A case study. Hand (N Y) 2013;8:324–329. 5. Barinka L. Campylodactylia (second communication). Acta Chir Plast. 1964;6:154–151.
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Plastic and Reconstructive Surgery • March 2015 6. Smith PJ, Grobbelaar AO. Camptodactyly: A unifying theory and approach to surgical treatment. J Hand Surg Am. 1998;23:14–19. 7. McFarlane RM, Curry GI, Evans HB. Anomalies of the intrinsic muscles in camptodactyly. J Hand Surg Am. 1983;8:531–544. 8. Smith RJ, Kaplan EB. Camptodactyly and similar atraumatic flexion deformities of the proximal interphalangeal joints of the fingers. J Bone Joint Surg Am. 1968:50:1187–203. 9. Stoddard EE. Nomenclature of hereditary crooked fingers. J Hered. 1939;30:511–512. 10. Millesi H. Camptodactyly. In: Symposium on Reconstructive Hand Surgery. Vol 9. St. Louis: Mosby; 1974:175–177.
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11. Benson LS. Camptodactyly: Classification and results of nonoperative treatment. J Pediatr Orthop. 1994;14:814–819. 12. Lucas GL. Fowler central slip tenotomy for old mallet deformity. Plast Reconstr Surg. 1987;80:92–94. 13. Joshi BB. Dorsolateral flap from same finger to relieve flexion contracture. Plast Reconstr Surg. 1972;49:186–189. 14. Foucher G, Lorea P, Khouri RK, et al. Camptodactyly as a spectrum of congenital deficiencies: A treatment algorithm based on clinical examination. Plast Reconstr Surg. 2006;117:1897–1905. 15. Siegert JJ, Cooney WP, Dobyns JH. Management of simple camptodactyly. J Hand Surg Br. 1990;15:181–189.
