- Email: [email protected]
Treatment of the windblown hand
Treatment of the windblown hand The windblown hand deformity, also known as congenital ulnar drift of thejngers and congenital contractures of the digits is reported infrequently in the literature. Although the syndrome is well described, authors’ use of different terms is confusing. In more than 20 years of practice, we have observed 11 patients with the windblown hand and have operated on 7 hands in 4 patients. The anomaly appears to be inherited as an autosomal dominant trait and may represent a variation of some type of arthrogryposis. Ulnar drift of the digits is often present at birth and becomes more pronounced as the child grows. The one factor common to all case reports is a flexion contracture of the metacarpophalangeal joints, with ulnar deviation of the fingers. Also, the thumb is webbed to the palm by a soft tissue bridge. Most cases of the windblown hand should be treated within the first 2 years of life. Because the windblown hand has many variations, each patient must be treated individually. We report a series on the windblown hand and propose a treatment plan for this rare anomaly. (J HAND SURC 1990;15A:431-8.)
Virchel
E. Wood,
MD,
and John Biondi,
C
ongenital ulnar drift is a little known clinical entity. Ulnar deviation of the fingers at birth, however, was reported by Emile Boix’ in 1897 who used Brissaud’s descriptive phrase deviation en coup de vent, or windblown fingers. After a review of our 11 cases over a 20-year period, we prefer to label this entity the windblown hand.* Each case is distinct from any other and may represent a spectrum of different problems, all of which may be a forme frust of some type of arthrogryposis.
MD, Loma Linda,
flexion contractures of the interphalangeal (IP) joints. The deformity is usually bilateral. Although ulnar deviation of the hands and fingers is the most common feature, webbing of the thumb to the palm is the most limiting disability (Fig. 2). Often the muscles in the shoulder girdle and entire upper extremity may be underdeveloped and their excursion restricted, limiting shoulder motion and pronation and supination of the forearm. Materials
Clinical
picture
Ulnar drift of the digits is often present at birth and becomes more pronounced as the child grows. The one factor common to all cases is a flexion contracture of the metacarpophalangeal (MP) joints, with ulnar deviation of the fingers (Fig. 1). The thumb is webbed to the palm by a soft tissue bridge. There may also be
From the Department Medical Center.
of Orthopaedic
Received for publication July 27, 1989.
March
Surgery, Loma Linda University
16, 1989; accepted
in revised form
No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Virchel E. Wood, MD, Department of Orthopaedic Surgery, Loma Linda University Medical Center, 11234 Anderson St., Loma Linda, CA 92350. 3/l/15982
Cal$
and methods
This article is a retrospective review of 11 patients with the windblown hand that we have seen in our clinic since 1968. There were nine males and two females. Seven patients preferred not to have surgery. We operated on seven hands in four patients; they all returned for follow-up evaluation. It is extremely difficult to evaluate the results of a problem with many facets. An excellent result would be a hand that has normal function and cosmesis. No patient could fit this criteria. A good result would be a hand that had almost normal function and had a satisfactory appearance. A fair hand would represent one with some remaining contractures that interfered with normal function. A poor result would be a contracted hand that could not function in the activities of daily living. Any evaluation of a windblown hand must be very subjective, and a hard set of criteria for evaluation is unrealistic. A summary of the surgical procedures with results is shown in Table I.
THE JOURNAL OF HAND SURGERY
431
432
Wood and Biondi
Fig. 1. Congenital ulnar drift of the digits and flexion con-
tractures of the MP and PIP joints.
Surgical treatment Because there are several variations of the windblown hand each case must be evaluated individually. Treatment should begin during the first 2 years of life, preferably within the first 6 months. There are few articles in the literature 3. 4 that outline treatment of this congenital problem. Splinting should be attempted first even though it may not be successful. All of our patients wore splints without any successful results. A cartilagenous and bony deformity may develop on the surfaces of the MP joints that could become fixed if not aligned correctly.’ The most important aspect of the windblown hand to be treated is the thumb contracture. The tight skin contracture of the thumb into the palm by a soft tissue bridge is associated with a great deficiency of skin (Fig. 3, A and B). We suggest two methods for release of the skin contracture. If the contracture is not severe, the thumb web may be opened by a four-flap Z-plasty or, by simply opening the skin and replacing it with a skin graft. Often, a dorsal rotation flap with a Y-V advancement will be required. We use a large dorsal rotation flap from the thumb (Fig. 4) or from the dorsum of the index finger, as described by Brown6 and Sandzen.’ To fully release the thumb, the adductor pollicis* may have to be released from the third metacarpal, and the
The Journal of HAND SURGERY
first dorsal interosseous released from the first metacarpal. Often the flexor pollicis longus must be lengthened in the wrist. If flexor tightness persists, the extensor pollicis brevis must be reefed up and shortened. When the distal thumb tip cannot extend effectively, the extensor indicis proprius should be transferred to the extensor pollicis longus to extend the distal joint. The thumb should then be held straight and abducted from the index finger with Kirschner wires for 3 to 6 weeks. The next problem to be solved is deficiency of skin in the fingers. The skin is not only shortened but is also pulled distally in the form of a web (Fig. 5) adding greatly to the flexion contractures and ulnar deviation of the digits. Although some surgeons divide the skin at the level of the MP joint, open the contracted finger, and replace the large defect with grafted skin, we have found this approach to be unsatisfactory. We do a syndactyly type release and insert full-thickness skin grafts from the groin into the area of skin deficiency (Fig. 6). In our experience, we have found this method to give the best results. Although contracture of the skin is a prime concern, all the bands of the palmar fascia, and other tight fascial structures must also be released, as advised by Zancolli.’ Correction of ulnar deviation of the digits is the next step. The intrinsic muscles may be shortened or tight, or they may be atrophic or absent. If the intrinsic muscles are tight the ulnar structures, such as the third and fourth dorsal interossei and the abductor digiti minimi, should be released. If the ulnar portions of the joint capsule and ulnar collateral ligament are tight when the joint is held in full correction, it may be necessary to excise some of the capsule and then lengthen the ligament. The intrinsic muscles may have to be transferred to the radial intrinsics of the adjacent finger. If the superticialis tendon is short and tight and the deviation is great, the flexor superficialis may be transferred to the radial intrinsics to prevent ulnar drift. The extensor tendons are often subluxed into the valleys between the metacarpal heads (Fig. 7). To correct subluxation the tendon must be centralized over the dorsum of the MP joint,5. ‘. ‘. ” and some type of reconstruction done to hold it in position. A slip of the extensor tendon can be passed around the collateral ligament and sutured back on itself to hold it in the relocated position. The extensor tendon may be held on top of the phalanx by suturing a slip of the junctura tendinum to the adjacent extensor hood. The desired goal with all of these procedures is to decrease capsular laxity on the radial side with growth over time. We recentralize the extensor tendon and hold it on
Vol. HA, No. 3 May 1990
Treatment of windblown hand
Fig. 2. A thick band of tissue holds the thumb to the palm.
Fig. 3. A, Preoperative picture of the thumb in palm deformity. leaves an enormous deficit of skin in the thumb web.
B, Release of the contracture
433
434
The Journal of HAND SURGERY
Wood and Biondi
Fig. 6. A syndactyly-type release is done and full-thickness skin graft from the groin covers the large areas of skin deficiency.
Fig. 4. This thumb web tissue loss is best closed with a large dorsal rotation flap from the dorsum of the index finger or thumb as shown.
Fig. 5. The skin between the fingers is pulled distally and
appears much like a syndactyly deformity.
top of the MP joint by suturing
it to the capsule of the dorsum of the joint if it is strong; or we create a true bony insertion by suturing the extensor tendon to the proximal phalanx, drilling two holes into the bone. When a bony insertion is used, holes are drilled into the bone and a nonabsorbable No. 2-O suture is passed through them to secure the tendon to the roughened
Fig. 7. The extensor metacarpal heads.
tendons
sublux ulnarly between
the
bone. The epiphysis must be avoided so as to prevent growth deformities. After the fingers are straightened, they may be drawn severely into the palm again because of tight shortened flexor tendons during the growth spurts of the child. The tight flexor tendons are released best by a fractional lengthening in the forearm. These procedures work well
Vol. 15A, No. 3 May 1990
Treatment of windblown hand
Fig. 8. The windblown
hand is commonly
associated
435
with a vertical talus.
Fig. 10. Even though the flexion deformities of this hand were fully corrected during a growth spurt the flexor tendons shortened as the bone lengthened. A fractional lengthening of the flexor tendons had to be done to improve function. This child is attempting full extension.
Fig. 9. Metacarpal windblown hand.
synostosis
commonly
occurs with the
in the young child, but results are not good in patients more than 5 or 6 years of age. A review of the literature indicates that the most enduring and satisfactory results in the neglected case or in an adult are obtained by corrective osteotomies.“’ I2 A corrective subcapital osteotomy of the metacarpal bones works well, although osteotomy of the proximal phalanges is also successful. In this pro-
cedure, a dorsal longitudinal incision is made between the index and long metacarpals, and the small and ring metacarpals. The extensor tendons are retracted carefully, and a dome-shaped osteotomy made in each metacarpal with an oscillating saw. It may be necessary to shorten each metacarpal to straighten the digits effectively. The metacarpals can be easily shifted on the rounded osteotomy.* Two Kirschner wires are inserted into each metacarpal to hold the bone in its proper position. A short arm cast is applied for 6 weeks. We have no experience with this operation in the adult patient.
436
The Journal of HAND SURGERY
Wood and Biondi
Table I. Surgical operations for treatment of windblown hand
I Patient
I
Birthdate
I
I
Dorsal rotation Z-plasty J?ap thumb ) thumb
Skin graft 1 thumb
N. P. (male) One hand
l/l3187
K. C. (male) Bilateral R. M. (male) Bilateral S. M. (male) Bilateral
719171
1
2111169
2
1
6
1
1014177
1
2
1
I Intrinsic release 1 thumb 2
2
I Lengthen
/ FPL 1
2
/
iT EIP to EPL or EPB 1
3
Syna’actyly release cut IM LIG.
1
3 1
6
6
1
8
Fii. 11. A, Twelve years after surgical treatment the fingers can extend well and B, The thumb webs spread open widely. Preoperative
pictures are shown in Figs. 3 and 10.
Skin graft only
/ jingersipalm
Vol. 15A, No. 3 May 1990
3
1
Treatment of windblown hand
4
4
6
8
3
4
6
8
6
4
437
4
2
6 mo
GOOd
1
3
12 yr
6
5
12.7 yr
6
10
10.4 yr
Declined further surgery; fair Declined further surgery; fair Good
Fig. 12. This 16year old child shows the results of no treatment. The fingers cannot extend, nor can the thumbs oppose.
Discussion Congenital ulnar drift, or the windblown hand, was vaguely identified in several isolated articles as the windmill-vane hand and similar terms until Powers and Ledbetter13 described 44 cases from the literature and one case of their own in 1976. Two popular names are congenital ulnar drift of the $ngers’ and congenital contructures of the digits. Zancolli and Zancolli’v lo call the deformity arthrogrypoid hand I and II. The syndrome may be recognized by defects other than the windblown hand. Descriptions of multiple defects added more confusion about terms. The syndrome was described as craniocarpotarsal dysplasia by Freeman and SheldonI in 1938, who emphasized the association of cranial, hand, and foot malformations. In 1963, Burian, I5 unaware of Freeman and Sheldon’s re-
port, described four cases with the same malformations, which he labeled the whistling face syndrome. Thus the abnormality can be found in the literature as craniocarpotarsal dysplasia, congenital windmill-vane fingers (as described by Lundblom16), the whistling face syndrome, ” and the Freeman-Sheldon syndrome. To date, at least 28 cases of the Freeman-Sheldon syndrome have been documened in the English-language literature.’ Undoubtedly, many more cases remain hidden under different descriptions. In 1972, Sallis and Brighton” described a syndrome they had observed in a large South African tribe. The common defects included: ulnar deviation of the phalanges, flexion contractures of the interphalangeal joints, soft tissue webbing between the thumb and palm, a vertical talus with rocker-bottom feet, and short stat-
438
The Journal of HAND SURGERY
Wood and Biondi
ure. Almost
all cases of the windblown
hand reported by an autosomal dominant gene, as was the case in this South African tribe. It is likely that many more cases remain unreported and untreated. Many other congenital defects have been recognized. Foot deformities, such as clubfoot, vertical talus, (Fig. 8) rocker-bottom feet, and tightly contracted toes, have also been described as a part of the syndrome. Characteristic craniofacial malformations that have been reported include a high skull compressed frontodorsally. The skull is broadened in the frontal plane, and the frontal bone arches in the midline. The mouth is narrowed (microstomia), sometimes to the point that it will not admit a teaspoon. I5 The upper lip is long and protrudes, as in whistling. The face looks stiff and masklike; hence, the term whistling-face syndrome. Asymmetry of the chest and shoulders with scoliosis is often present. Metacarpal synostosis of the hands can occur (Fig. 9). In addition to the ulnar deviation of the MP joints and the contracture of the thumb web, the entire muscle mass in the upper extremity may be hypoplastic. The cause of decreased muscle mass may be disuse or an inherited defect. Zancolli and coworkers9. lo contend that the deformity results from malformation of the retaining ligaments or retinacular cutis of the skin of the hand. After observing our patients, we believe that they all may represent a variation of some form of arthrogryposis. Zancolli and Zancolli’ classify the abnormality as segmentary arthrogryposis. The treatment of these cases has been extremely frustrating and is not always satisfactory (Table I). We have observed a hand that was corrected perfectly become severely flexed and deviated again during a growth spurt, because of shortened and tight extrinsic flexor tendons (Fig. IO). Two of seven hands developed recurrent flexion contractures to date. The digits can be straightened again with surgery. Although these children do not have perfect results from our treatment (Fig. 1 I), both the parents and patients believe that the treated hands are better than the hands not treated (Fig. 12).
3. Aldmger G, Eulert J. Das Freeman-Sheldon-Syndrom
in the literature’9-22 have been transmitted
REFERENCES 1. Boix E. Deviation des doigts en cop de vent et insuffis-
ante de L’opone’ vrose paliciare d’origine congenitale. Nouv Inconogr Salpet 1897;lO: 180-94. 2. Wood VE. Windblown hand. In: Green DP, ed. Operative hand surgery. 2nd ed. New York: Churchill Livingstone, 1988.
4.
5. 6.
7.
8.
9. 10.
11. 12. 13.
14.
15.
16.
17.
(The Freeman-Sheldon Syndrome). 2 Orthop 1983;121: 630-3. Martini AK, Bazan UB. Die Handdeformitaten beim Freeman-Sheldon-Syndrom und ihre operative Behandlung. Z Orthop 1983;121:623-9. Flatt AE. The care of congenital hand anomalies. St. Louis: The C.V. Mosby Co, 1977:164-6. Brown PW. Adduction flexion contracture of the thumb correction with dorsal rotation flap and release of contracture. Clin Orthop 1972;88:161-8. Sandzen SC. Dorsal pedicle flap for resurfacing a moderate thumb-index web contracture release. J HAND SURG 1982;7:21-4. Call WH, Strickland JW. Functional hand reconstruction in the whistling face syndrome. J HAND SURG 1981; 6:148-51. Zancolli E, Zancolli E, Jr. Congenital ulnar drift of the fingers. Hand Clinics 1985;1:443-56. Zancolli EA, Zancolli EA, Jr. Congenital ulnar drift and camptodactyly produced by malformation of the retaining ligaments of the skin. Bull Hosp Jt Disease 1984;44: 558-75. Boyes JH. Bunnell’s surgery of the hand. 4th ed. Philadelphia: JB Lippincott, 1964;72-5. Kelikian H. Congenital deformities of the hand and forearm. Philadelphia: WB Saunders, 1974;577-83. Powers RC, Ledbetter RH, Jr. Congenital flexion and ulnar deviation of the metacatpophalangeal joints of the hand. A case report. Clin Orthop 1976;116:173-5. Freeman EA, Sheldon JH. Craniocarpotarsal dystrophy. Undescribed congenital malformations. Arch Dis Child 1938;13:277. Burian F. The whistling face characteristic in a compound crania-facie-corpora1 syndrome. Br J Plast Surg 1963; 16:140. Lundblom A. On congenital ulnar deviation of the fingers of familiar occurrence. Acta Orthop Stand 1932;3:393-4. Malkawi H, Tarawneh M. The whistling face syndrome, or craniocarpotarsal dysplasia. Report of two cases in a father and son and review of the literature. J Pediat Orthop 1983;3:370-5.
18. Sallis JG, Beighton P. Dominantly inherited digitotalar dysmorphism. J Bone Joint Surg 1972;54B:509-15. 19. Fisk JR, House JH. Congenital ulnar deviation of the fingers with clubfoot deformities. Clin Orthop 1974;104: 200-5. 20. Jacquemain B. Die angeborene Windmuhlen flugelstelling 01s erbliche kombinationsnissbildung. Z Orthop 1966;102:146-54. 21. Stevenson RE, Scott CI, Epstein M. Dominantly inherited ulnar drift. Birth Defects 1975;11:75-7. 22. Temtamy SA, McKusick VA. Contracture deformities as a part of syndromes. Birth Defects 1978; 14447-51.
Virchel
E. Wood,
MD,
and John Biondi,
C
ongenital ulnar drift is a little known clinical entity. Ulnar deviation of the fingers at birth, however, was reported by Emile Boix’ in 1897 who used Brissaud’s descriptive phrase deviation en coup de vent, or windblown fingers. After a review of our 11 cases over a 20-year period, we prefer to label this entity the windblown hand.* Each case is distinct from any other and may represent a spectrum of different problems, all of which may be a forme frust of some type of arthrogryposis.
MD, Loma Linda,
flexion contractures of the interphalangeal (IP) joints. The deformity is usually bilateral. Although ulnar deviation of the hands and fingers is the most common feature, webbing of the thumb to the palm is the most limiting disability (Fig. 2). Often the muscles in the shoulder girdle and entire upper extremity may be underdeveloped and their excursion restricted, limiting shoulder motion and pronation and supination of the forearm. Materials
Clinical
picture
Ulnar drift of the digits is often present at birth and becomes more pronounced as the child grows. The one factor common to all cases is a flexion contracture of the metacarpophalangeal (MP) joints, with ulnar deviation of the fingers (Fig. 1). The thumb is webbed to the palm by a soft tissue bridge. There may also be
From the Department Medical Center.
of Orthopaedic
Received for publication July 27, 1989.
March
Surgery, Loma Linda University
16, 1989; accepted
in revised form
No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Virchel E. Wood, MD, Department of Orthopaedic Surgery, Loma Linda University Medical Center, 11234 Anderson St., Loma Linda, CA 92350. 3/l/15982
Cal$
and methods
This article is a retrospective review of 11 patients with the windblown hand that we have seen in our clinic since 1968. There were nine males and two females. Seven patients preferred not to have surgery. We operated on seven hands in four patients; they all returned for follow-up evaluation. It is extremely difficult to evaluate the results of a problem with many facets. An excellent result would be a hand that has normal function and cosmesis. No patient could fit this criteria. A good result would be a hand that had almost normal function and had a satisfactory appearance. A fair hand would represent one with some remaining contractures that interfered with normal function. A poor result would be a contracted hand that could not function in the activities of daily living. Any evaluation of a windblown hand must be very subjective, and a hard set of criteria for evaluation is unrealistic. A summary of the surgical procedures with results is shown in Table I.
THE JOURNAL OF HAND SURGERY
431
432
Wood and Biondi
Fig. 1. Congenital ulnar drift of the digits and flexion con-
tractures of the MP and PIP joints.
Surgical treatment Because there are several variations of the windblown hand each case must be evaluated individually. Treatment should begin during the first 2 years of life, preferably within the first 6 months. There are few articles in the literature 3. 4 that outline treatment of this congenital problem. Splinting should be attempted first even though it may not be successful. All of our patients wore splints without any successful results. A cartilagenous and bony deformity may develop on the surfaces of the MP joints that could become fixed if not aligned correctly.’ The most important aspect of the windblown hand to be treated is the thumb contracture. The tight skin contracture of the thumb into the palm by a soft tissue bridge is associated with a great deficiency of skin (Fig. 3, A and B). We suggest two methods for release of the skin contracture. If the contracture is not severe, the thumb web may be opened by a four-flap Z-plasty or, by simply opening the skin and replacing it with a skin graft. Often, a dorsal rotation flap with a Y-V advancement will be required. We use a large dorsal rotation flap from the thumb (Fig. 4) or from the dorsum of the index finger, as described by Brown6 and Sandzen.’ To fully release the thumb, the adductor pollicis* may have to be released from the third metacarpal, and the
The Journal of HAND SURGERY
first dorsal interosseous released from the first metacarpal. Often the flexor pollicis longus must be lengthened in the wrist. If flexor tightness persists, the extensor pollicis brevis must be reefed up and shortened. When the distal thumb tip cannot extend effectively, the extensor indicis proprius should be transferred to the extensor pollicis longus to extend the distal joint. The thumb should then be held straight and abducted from the index finger with Kirschner wires for 3 to 6 weeks. The next problem to be solved is deficiency of skin in the fingers. The skin is not only shortened but is also pulled distally in the form of a web (Fig. 5) adding greatly to the flexion contractures and ulnar deviation of the digits. Although some surgeons divide the skin at the level of the MP joint, open the contracted finger, and replace the large defect with grafted skin, we have found this approach to be unsatisfactory. We do a syndactyly type release and insert full-thickness skin grafts from the groin into the area of skin deficiency (Fig. 6). In our experience, we have found this method to give the best results. Although contracture of the skin is a prime concern, all the bands of the palmar fascia, and other tight fascial structures must also be released, as advised by Zancolli.’ Correction of ulnar deviation of the digits is the next step. The intrinsic muscles may be shortened or tight, or they may be atrophic or absent. If the intrinsic muscles are tight the ulnar structures, such as the third and fourth dorsal interossei and the abductor digiti minimi, should be released. If the ulnar portions of the joint capsule and ulnar collateral ligament are tight when the joint is held in full correction, it may be necessary to excise some of the capsule and then lengthen the ligament. The intrinsic muscles may have to be transferred to the radial intrinsics of the adjacent finger. If the superticialis tendon is short and tight and the deviation is great, the flexor superficialis may be transferred to the radial intrinsics to prevent ulnar drift. The extensor tendons are often subluxed into the valleys between the metacarpal heads (Fig. 7). To correct subluxation the tendon must be centralized over the dorsum of the MP joint,5. ‘. ‘. ” and some type of reconstruction done to hold it in position. A slip of the extensor tendon can be passed around the collateral ligament and sutured back on itself to hold it in the relocated position. The extensor tendon may be held on top of the phalanx by suturing a slip of the junctura tendinum to the adjacent extensor hood. The desired goal with all of these procedures is to decrease capsular laxity on the radial side with growth over time. We recentralize the extensor tendon and hold it on
Vol. HA, No. 3 May 1990
Treatment of windblown hand
Fig. 2. A thick band of tissue holds the thumb to the palm.
Fig. 3. A, Preoperative picture of the thumb in palm deformity. leaves an enormous deficit of skin in the thumb web.
B, Release of the contracture
433
434
The Journal of HAND SURGERY
Wood and Biondi
Fig. 6. A syndactyly-type release is done and full-thickness skin graft from the groin covers the large areas of skin deficiency.
Fig. 4. This thumb web tissue loss is best closed with a large dorsal rotation flap from the dorsum of the index finger or thumb as shown.
Fig. 5. The skin between the fingers is pulled distally and
appears much like a syndactyly deformity.
top of the MP joint by suturing
it to the capsule of the dorsum of the joint if it is strong; or we create a true bony insertion by suturing the extensor tendon to the proximal phalanx, drilling two holes into the bone. When a bony insertion is used, holes are drilled into the bone and a nonabsorbable No. 2-O suture is passed through them to secure the tendon to the roughened
Fig. 7. The extensor metacarpal heads.
tendons
sublux ulnarly between
the
bone. The epiphysis must be avoided so as to prevent growth deformities. After the fingers are straightened, they may be drawn severely into the palm again because of tight shortened flexor tendons during the growth spurts of the child. The tight flexor tendons are released best by a fractional lengthening in the forearm. These procedures work well
Vol. 15A, No. 3 May 1990
Treatment of windblown hand
Fig. 8. The windblown
hand is commonly
associated
435
with a vertical talus.
Fig. 10. Even though the flexion deformities of this hand were fully corrected during a growth spurt the flexor tendons shortened as the bone lengthened. A fractional lengthening of the flexor tendons had to be done to improve function. This child is attempting full extension.
Fig. 9. Metacarpal windblown hand.
synostosis
commonly
occurs with the
in the young child, but results are not good in patients more than 5 or 6 years of age. A review of the literature indicates that the most enduring and satisfactory results in the neglected case or in an adult are obtained by corrective osteotomies.“’ I2 A corrective subcapital osteotomy of the metacarpal bones works well, although osteotomy of the proximal phalanges is also successful. In this pro-
cedure, a dorsal longitudinal incision is made between the index and long metacarpals, and the small and ring metacarpals. The extensor tendons are retracted carefully, and a dome-shaped osteotomy made in each metacarpal with an oscillating saw. It may be necessary to shorten each metacarpal to straighten the digits effectively. The metacarpals can be easily shifted on the rounded osteotomy.* Two Kirschner wires are inserted into each metacarpal to hold the bone in its proper position. A short arm cast is applied for 6 weeks. We have no experience with this operation in the adult patient.
436
The Journal of HAND SURGERY
Wood and Biondi
Table I. Surgical operations for treatment of windblown hand
I Patient
I
Birthdate
I
I
Dorsal rotation Z-plasty J?ap thumb ) thumb
Skin graft 1 thumb
N. P. (male) One hand
l/l3187
K. C. (male) Bilateral R. M. (male) Bilateral S. M. (male) Bilateral
719171
1
2111169
2
1
6
1
1014177
1
2
1
I Intrinsic release 1 thumb 2
2
I Lengthen
/ FPL 1
2
/
iT EIP to EPL or EPB 1
3
Syna’actyly release cut IM LIG.
1
3 1
6
6
1
8
Fii. 11. A, Twelve years after surgical treatment the fingers can extend well and B, The thumb webs spread open widely. Preoperative
pictures are shown in Figs. 3 and 10.
Skin graft only
/ jingersipalm
Vol. 15A, No. 3 May 1990
3
1
Treatment of windblown hand
4
4
6
8
3
4
6
8
6
4
437
4
2
6 mo
GOOd
1
3
12 yr
6
5
12.7 yr
6
10
10.4 yr
Declined further surgery; fair Declined further surgery; fair Good
Fig. 12. This 16year old child shows the results of no treatment. The fingers cannot extend, nor can the thumbs oppose.
Discussion Congenital ulnar drift, or the windblown hand, was vaguely identified in several isolated articles as the windmill-vane hand and similar terms until Powers and Ledbetter13 described 44 cases from the literature and one case of their own in 1976. Two popular names are congenital ulnar drift of the $ngers’ and congenital contructures of the digits. Zancolli and Zancolli’v lo call the deformity arthrogrypoid hand I and II. The syndrome may be recognized by defects other than the windblown hand. Descriptions of multiple defects added more confusion about terms. The syndrome was described as craniocarpotarsal dysplasia by Freeman and SheldonI in 1938, who emphasized the association of cranial, hand, and foot malformations. In 1963, Burian, I5 unaware of Freeman and Sheldon’s re-
port, described four cases with the same malformations, which he labeled the whistling face syndrome. Thus the abnormality can be found in the literature as craniocarpotarsal dysplasia, congenital windmill-vane fingers (as described by Lundblom16), the whistling face syndrome, ” and the Freeman-Sheldon syndrome. To date, at least 28 cases of the Freeman-Sheldon syndrome have been documened in the English-language literature.’ Undoubtedly, many more cases remain hidden under different descriptions. In 1972, Sallis and Brighton” described a syndrome they had observed in a large South African tribe. The common defects included: ulnar deviation of the phalanges, flexion contractures of the interphalangeal joints, soft tissue webbing between the thumb and palm, a vertical talus with rocker-bottom feet, and short stat-
438
The Journal of HAND SURGERY
Wood and Biondi
ure. Almost
all cases of the windblown
hand reported by an autosomal dominant gene, as was the case in this South African tribe. It is likely that many more cases remain unreported and untreated. Many other congenital defects have been recognized. Foot deformities, such as clubfoot, vertical talus, (Fig. 8) rocker-bottom feet, and tightly contracted toes, have also been described as a part of the syndrome. Characteristic craniofacial malformations that have been reported include a high skull compressed frontodorsally. The skull is broadened in the frontal plane, and the frontal bone arches in the midline. The mouth is narrowed (microstomia), sometimes to the point that it will not admit a teaspoon. I5 The upper lip is long and protrudes, as in whistling. The face looks stiff and masklike; hence, the term whistling-face syndrome. Asymmetry of the chest and shoulders with scoliosis is often present. Metacarpal synostosis of the hands can occur (Fig. 9). In addition to the ulnar deviation of the MP joints and the contracture of the thumb web, the entire muscle mass in the upper extremity may be hypoplastic. The cause of decreased muscle mass may be disuse or an inherited defect. Zancolli and coworkers9. lo contend that the deformity results from malformation of the retaining ligaments or retinacular cutis of the skin of the hand. After observing our patients, we believe that they all may represent a variation of some form of arthrogryposis. Zancolli and Zancolli’ classify the abnormality as segmentary arthrogryposis. The treatment of these cases has been extremely frustrating and is not always satisfactory (Table I). We have observed a hand that was corrected perfectly become severely flexed and deviated again during a growth spurt, because of shortened and tight extrinsic flexor tendons (Fig. IO). Two of seven hands developed recurrent flexion contractures to date. The digits can be straightened again with surgery. Although these children do not have perfect results from our treatment (Fig. 1 I), both the parents and patients believe that the treated hands are better than the hands not treated (Fig. 12).
3. Aldmger G, Eulert J. Das Freeman-Sheldon-Syndrom
in the literature’9-22 have been transmitted
REFERENCES 1. Boix E. Deviation des doigts en cop de vent et insuffis-
ante de L’opone’ vrose paliciare d’origine congenitale. Nouv Inconogr Salpet 1897;lO: 180-94. 2. Wood VE. Windblown hand. In: Green DP, ed. Operative hand surgery. 2nd ed. New York: Churchill Livingstone, 1988.
4.
5. 6.
7.
8.
9. 10.
11. 12. 13.
14.
15.
16.
17.
(The Freeman-Sheldon Syndrome). 2 Orthop 1983;121: 630-3. Martini AK, Bazan UB. Die Handdeformitaten beim Freeman-Sheldon-Syndrom und ihre operative Behandlung. Z Orthop 1983;121:623-9. Flatt AE. The care of congenital hand anomalies. St. Louis: The C.V. Mosby Co, 1977:164-6. Brown PW. Adduction flexion contracture of the thumb correction with dorsal rotation flap and release of contracture. Clin Orthop 1972;88:161-8. Sandzen SC. Dorsal pedicle flap for resurfacing a moderate thumb-index web contracture release. J HAND SURG 1982;7:21-4. Call WH, Strickland JW. Functional hand reconstruction in the whistling face syndrome. J HAND SURG 1981; 6:148-51. Zancolli E, Zancolli E, Jr. Congenital ulnar drift of the fingers. Hand Clinics 1985;1:443-56. Zancolli EA, Zancolli EA, Jr. Congenital ulnar drift and camptodactyly produced by malformation of the retaining ligaments of the skin. Bull Hosp Jt Disease 1984;44: 558-75. Boyes JH. Bunnell’s surgery of the hand. 4th ed. Philadelphia: JB Lippincott, 1964;72-5. Kelikian H. Congenital deformities of the hand and forearm. Philadelphia: WB Saunders, 1974;577-83. Powers RC, Ledbetter RH, Jr. Congenital flexion and ulnar deviation of the metacatpophalangeal joints of the hand. A case report. Clin Orthop 1976;116:173-5. Freeman EA, Sheldon JH. Craniocarpotarsal dystrophy. Undescribed congenital malformations. Arch Dis Child 1938;13:277. Burian F. The whistling face characteristic in a compound crania-facie-corpora1 syndrome. Br J Plast Surg 1963; 16:140. Lundblom A. On congenital ulnar deviation of the fingers of familiar occurrence. Acta Orthop Stand 1932;3:393-4. Malkawi H, Tarawneh M. The whistling face syndrome, or craniocarpotarsal dysplasia. Report of two cases in a father and son and review of the literature. J Pediat Orthop 1983;3:370-5.
18. Sallis JG, Beighton P. Dominantly inherited digitotalar dysmorphism. J Bone Joint Surg 1972;54B:509-15. 19. Fisk JR, House JH. Congenital ulnar deviation of the fingers with clubfoot deformities. Clin Orthop 1974;104: 200-5. 20. Jacquemain B. Die angeborene Windmuhlen flugelstelling 01s erbliche kombinationsnissbildung. Z Orthop 1966;102:146-54. 21. Stevenson RE, Scott CI, Epstein M. Dominantly inherited ulnar drift. Birth Defects 1975;11:75-7. 22. Temtamy SA, McKusick VA. Contracture deformities as a part of syndromes. Birth Defects 1978; 14447-51.