PROCEDURE 96
Camptodactyly Correction Sirichai Kamnerdnakta, Matthew Brown, and Kevin C. Chung Indications • A patient who has functional impairment and a severe flexion deformity with extension lag more than 60 degrees or a flexion contracture more than 30 degrees • A deformity that persists after 6 to 12 months of stretching and splinting • Camptodactyly with a flexion contracture less than 30 degrees causes minimal function limitation for most patients. A patient may be more concerned about the appearance of the flexed finger. Surgery should be cautioned in these individuals because surgery may lead to a decrease in function due to recurrent contracture.
Clinical Examination • Camptodactyly refers to a flexion deformity of the proximal interphalangeal (PIP) joint that most often manifests in the small finger. • Camptodactyly has been classified into three different types (Table 96.1). • The etiology of camptodactyly has been attributed to anomalous joint architecture, laxity of the extensor mechanism at the PIP level, anomalous fascial bands from the A1 pulley, abnormal flexor tendons, abnormal lumbrical muscles, and/or anomalous intrinsic muscles. The most commonly identified anomalies include an anomalous lumbrical muscle or an absence or anomaly of the flexor digitorum superficialis (FDS) tendon. • The clinical examination should assess the possible causes of the flexion deformity and document the severity of the deformity. • The PIP joint is checked for extension lag and flexion contracture with the wrist in neutral position. Extension lag is the maximum extension measurement when performing active motion testing. Flexion contracture is the maximum extension measurement when performing passive motion testing. A perfectly straight PIP joint is considered to have 0 degrees of lag or contracture. Extension lag and flexion contracture measurements are not mutually exclusive. A joint may have an extension lag of 60 degrees, but passive testing may reveal a joint correctable to a 30-degree flexion contracture. • Assessing the finger with the metacarpophalangeal (MCP) in flexion and extension is performed next. For patients with camptodactyly, when the MCP joint is in extension the finger assumes a flexed posture at the PIP. Passive extension of the PIP may produce blanching of the skin, which implies a skin deficiency (Fig. 96.1). Additionally, if passive flexion of the MCP improves PIP extension, the etiology of the contracture is outside the PIP joint. This can mean skin deficiency, subcutane-
TABLE 96.1 Classification of Camptodactyly
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Type
Description
1
A newborn presents with a flexion deformity of the fifth and/or fourth finger. This is the most common type and affects males and females equally.
2
Physical changes similar to type 1, but it develops between 7 and 11 years of age. Females are affected more often than males. The flexion deformity will not improve over time and may develop into a severe flexion contracture.
3
Present from the time of birth. It affects several fingers, is bilateral and often has an accentuated flexion deformity. It is associated with a variety of syndromes and other malformations.
PROCEDURE 96 Camptodactyly Correction
Degree of skin contracture
FIG. 96.1
ous fibrous bands, or tightness of the extrinsic finger flexors, principally the FDS. If passive extension is not improved with MCP flexion there is some component of primary joint contracture that will need to be surgically addressed. • A compensatory hyperextension deformity of the MCP is frequently found with a PIP flexion deformity. With the Bouvier maneuver, the examiner corrects the hyperextension by passively placing the MCP in neutral or slight flexion. If this restores full PIP active extension, this implies the MCP hyperextension as the cause of the PIP flexion deformity and may be secondary to an intrinsic muscle abnormality. An FDS transfer to the lateral band is the procedure of choice. This increases MCP flexion and PIP extension forces. • For patients with passively correctable flexion deformities, the extensor tenodesis effect is checked to assess the extrinsic extensor integrity. The wrist is placed in full flexion along with full flexion of the MCP joints. In a normal finger, this maneuver should produce full PIP extension through passive stretch on extensor digitorum communis. If it does not, this implies a laxity or hypoplasia of the central slip. • The FDS of the little and ring finger may have a tendinous interconnection, which prohibits independent PIP joint flexion of the little finger. Classically, if a patient cannot flex the little finger while holding the ring finger in full extension this is thought to mean an absence of an FDS to the little finger. However, the test should be repeated with liberation of the ring finger and similar assessment of active PIP joint flexion. If the patient is able to isolate PIP flexion of the ring finger and the small finger simultaneously flexes at the PIP this indicates an interconnected FDS. The small finger FDS must be separated from the ring finger at the time of surgery to be a suitable donor for transfer.
Imaging • Three-view standard hand x-rays are obtained. The configuration of the PIP joint and the adjacent bones are observed. The lateral film will be the most formative view. The head of the proximal phalanx loses its rounded articular convex contour, and there is flattening at the base of the middle phalanx articulation. The base of middle phalanx is volarly subluxated and the flexed middle phalanx creates an indentation in the palmar neck of the proximal phalanx (Fig. 96.2).
Surgical Anatomy • All anatomic structures surrounding the PIP joint can contribute to the flexion defomity. The FDS tendon and lumbrical muscles most often are involved. • The FDS tendon has been described as contracted, underdeveloped, or devoid of a functional muscle. The tendon may originate from palmar fascia or the transverse carpal ligament instead of a muscle belly. This abnormal musculotendinous architecture cannot elongate during periods of rapid growth, which creates a PIP joint flexion deformity. • The lumbrical may have an abnormal origin or insertion. An abnormal origin from the transverse carpal ligament or the ring flexor tendons has been described. Abnormal insertions are more common and include an attachment directly into the MCP joint
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The head of the proximal phalanx loses its rounded articular convex contour
Volar subluxation of the middle phalanx
Indentation at neck of proximal phalanx
FIG. 96.2
Dorsolateral transposition flap
FIG. 96.3
capsule, onto the FDS, into the ring finger extensor apparatus, or within the lumbrical canal. The lack of intrinsic contribution to PIP extension creates an intrinsic minus deformity.
Positioning • The operation can be performed under axillary/brachial plexus block or general anesthesia. • The patient is placed in a supine position with the arm extended and hand pronated on a hand table. A tourniquet is employed.
Exposures EXPOSURES PEARLS
In flexible camptodactyly without a fixed PIP joint flexion contracture and with mild-to-moderate contracture of skin, a palmar longitudinal approach with Z-plasty lengthening or Bruner incision may be used. When there is a significant degree of skin contracture and a Z-plasty or dorsolateral flap are not adequate, closure of the wound will require skin grafts.
• A transverse proximal interphalangeal volar incision is designed on the PIP joint crease. This incision is used to release contracted structures on the volar side of PIP joint. A dorsolateral transposition flap is designed, so it can rotate and cover the volar defect after releasing the contracture. The posterior border of the flap is on the midlateral line and the anterior border of the flap is dependent on the estimated size of the defect after releasing. The length of the flap is designed to reach beyond the DIP joint crease (Fig. 96.3). We prefer to use the transposition flap procedure to correct skin deficiency over the volar PIP joint and to provide wide exposure of all structures.
PROCEDURE 96 Camptodactyly Correction
Digital neurovascular bundle
A Digital neurovascular bundle
B Digital neurovascular bundle
C FIG. 96.4 A-C
Procedure Step 1: Skin and Facial Contracture Releasing and Dorsolateral Flap Elevation • The incisions are made. The dorsolateral flap is raised along the deep fascial plane, leaving only areolar tissue over the extensor expansion. A volar digital neurovascular bundle is identified and protected. The flap is raised entirely to the base of the flap at the level of the head of the proximal phalanx (Fig. 96.4A–C). • Skin, fascia, and soft tissue volar to the flexor tendon are released. Any abnormal fascia and linear fibrous bands are released during exposure of deeper structures. Both neurovascular bundles are identified and protected. • The flexor tendon sheath is exposed at the joint, and the sheath is opened at the level of the A3 pulley. The flexor digitorum profundus (FDP) tendon is retracted to expose the Camper chiasm and insertion of the FDS tendon.
Step 2: Release FDS Tendon • The Camper chiasm is divided longitudinally. Both slips of FDS are divided transversely and cut at a level distal to the chiasm (Fig. 96.5A and B). • PIP joint passive range of motion is tested. If there is no residual flexion contracture, the releasing process is complete. If a flexion contracture remains, then the PIP joint capsule and ligaments are released.
Step 3: Volar Plate and Collateral Ligament Releasing • With the FDP tendon retracted away, a transverse incision is made at the level of the membranous volar plate. The location is just distal to the checkrein ligament attachment. The incision is curved distally on each side separating the volar plate from the accessory collateral ligaments.
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Cut end of FDS
Pulleys
A5C3 A4 C2 A3 C1
A2
A1
PA
Cut end of FDS
Retracted FDP
A
Camper chiasm
B FIG. 96.5 A-B FDP, flexor digitorum profundus; FDS, flexor digitorum superficialis.
A3
Check rein ligaments
Volar plate
C1
Accessory collateral ligament
A2
MCPJ
Divide the volar plate as proximal as possible
Proper collateral ligament
A
Divide the volar plate as proximal as possible
B FIG. 96.6 A-B MCPJ, metacarpophalangeal joint.
STEP 3 PITFALLS
Do not divide the entire collateral ligaments. The middle and dorsal aspects of the ligaments should be preserved to retain the stability of the PIP joint.
• A Freer elevator is used to gently lift the volar plate. The plate should slide distally as the joint is released (Fig. 96.6A and B). • If additional restrictions remain, the accessory collaterals and collateral ligaments are partially divided. • After release of all restrictive structures, full passive PIP extension is confirmed.
Step 4: FDS Transfer to Ulnar Lateral Band When There Is Extension Lag • FDS transfer is used to augment the active extension force on the PIP joint. • The cut end of one slip of the FDS is transferred from the volar side to the dorsal lateral side of the finger through the lumbrical canal. The end of the FDS slip is woven into the ipsilateral lateral band with 4-0 braided nonabsorbable suture (Fig. 96.7).
PROCEDURE 96 Camptodactyly Correction
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Weaving FDS slip with ipsilateral lateral band
A Weaving FDS slip with ipsilateral lateral band
FIG. 96.7 A-B FDS, flexor digitorum superficialis.
• Tension is set with the MCP joint positioned in 30 degrees of flexion and the PIP joint held in full extension.
Step 5: Dorsolateral Flap Inset and Incision Closure • The dorsolateral flap is transposed 90 degrees to cover the volar skin defect. • A template is made of the donor site defect after the flap is transposed. A full-thickness skin graft is harvested from the hypothenar area, because the skin texture will be similar to the finger. • The tourniquet is deflated and hemostasis is performed. • The flap is inset, and all of the incisions are closed and sutured with 4-0 absorbable suture. The donor site of the full-thickness skin graft is closed primarily. • The skin graft is inset in the donor site defect and secured with 4-0 absorbable suture (Fig. 96.8A and B).
Postoperative Care and Expected Outcomes • The hand and wrist are immobilized in a short-arm splint for 3 weeks; rangeof-motion exercise is encouraged at 4 weeks after the operation. Therapy is initiated for scar management, tendon transfer training, and joint stiffness exercise. Six weeks after surgery, light resistive strengthening is started. The splint is removed during the day except for strenuous activity. It is discontinued for all activity 12 weeks after surgery, and unrestricted activity is allowed.
STEP 4 PEARLS
• FDS transfer is used for camptodactyly patients who are unable to actively extend the PIP joint when the MCP joint is positioned in flexion. Preoperatively, the risk for swanneck posturing and loss of flexion must be discussed with the patient and family. • The key step in this surgery is to maintain the FDS volar to the central axis of the MCP joint by passing the tendon through the lumbrical canal. This keeps the tendon palmar to the deep transverse metacarpal ligament and ensures MCP flexion after the tendon is attached to the lateral band. • An interconnection between FDS of the little finger and ring finger is occasionally encountered and is released to facilitate individual movement of each finger.
STEP 5 PEARLS
The flap inset should not have tension. If there is an insufficient amount of flap to cover the full defect, a full-thickness skin graft can be incorporated with the flap to cover any noncritical structures.
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A Hypothenar full thickness skin graft
B FIG. 96.8 A-B
A
B FIG. 96.9
• A variety of operations have been proposed and, surgical results have been variable. Patient/parent compliance with splinting and therapy are equally important as surgery. • Complications can include infection, incomplete PIP extension, decreased active flexion, recurrence of contracture, and stiffness and pain. • The functional status for severe flexion deformity patients is improved with the improved posture of the finger (Fig. 96.9A and B). See Video 96.1, Camptodactyly Correction, on ExpertConsult.com.
PROCEDURE 96 Camptodactyly Correction
EVIDENCE Foucher G, Lorea P, Khouri RK, Medina J, Pivato G. Camptodactyly as a spectrum of congenital deficiencies: a treatment algorithm based on clinical examination. Plast Reconstr Surg 2006;117: 1897–905. This is retrospective review of 68 patients treated for camptodactyly. The senior author created an algorithm for who was an acceptable surgical candidate after several years of clinical experience. The study compares the results of patients before and after establishment of the algorithm. Patients with stiff joints were first treated with splints. In patients who were compliant with preoperative splinting, but no satisfactory improvement in extension was achieved, surgery was performed resulting in improvement rates from 68% to 88%. The authors detail their preferred surgical techniques. Fewer operative failures were experienced after implementing the algorithm, which provided direction for surgical patient selection (Level V evidence). Hamilton KL, Netscher DT. Evaluation of a stepwise surgical approach to camptodactyly. Plast Reconstr Surg 2015;135(3):568e–76e. This retrospective study reviewed 18 digits operated on in 12 consecutive patients with camptodactyly. All operated digits did not respond to splinting and had greater than 30 degrees of flexion contracture. The patients were treated with a stepwise surgical approach to release tethering structures around the joint. Fifteen digits achieved full active extension with a range of 0 to 25 degrees. Mean PIP flexion was 88 degrees with a range of 50 to 100. The authors believe surgery is indicated to prevent a long-term, irreversible articular deformity. The authors also recommend that the patient’s caregivers postoperatively must be motivated to regularly stretch the operated digit after surgery or risk a recurrence of the flexion contracture secondary to scar tissue formation (Level V evidence). Siegert JJ, Cooney WP, Dobyns JH. Management of simple camptodactyly. J Hand Surg Br 1990;15:181–9. The authors reviewed 57 patients with camptodactyly. Involvement of multiple digits was common. Thirty-eight fingers were treated by surgery and 41 by therapy. Fifty patients were available for follow-up examination 6 years after treatment. Surgery consisted of release of the contracted structures with or without transfer of the FDS. The results were classified according to the ultimate improvement in PIP joint extension without a simultaneous loss in flexion. In the operative group, there were 25 poor, 6 fair, 7 good, and no excellent results. The overall improvement in extension was just 10 degrees, and 10 patients lost considerable flexion. Ankylosis of the PIP joint developed in an additional 6 patients. In the conservative group, there were 6 poor, 8 fair, 27 good, and no excellent results (Level IV evidence).
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