Syndactyly Workup

  • Author: E Gene Deune, MD, MBA; Chief Editor: Harris Gellman, MD   more...
 
Updated: Feb 15, 2012
 

Imaging Studies

  • Obtain radiographs of the hands to evaluate for any other bony anomalies, such as synostosis, delta phalanx, or symphalangism.
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Diagnostic Procedures

If the infant has no associated medical conditions, a formal preoperative evaluation by the anesthesia team is usually not necessary. However, should there be any congenital syndromes or associated medical conditions, the patients should be scheduled for operative clearance with the preoperative evaluation service of the hospital where the surgery will be done. Generally, if there is no issue with postoperative monitoring, many of these cases can be handled as outpatient procedures.

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Contributor Information and Disclosures
Author

E Gene Deune, MD, MBA  Associate Professor of Orthopedic Surgery, Associate Professor of Plastic Surgery, Co-Director, Division of Hand Surgery, Director, Section of Hand Surgery and Pediatric Hand Surgery, Johns Hopkins University School of Medicine

E Gene Deune, MD, MBA is a member of the following medical societies: Alpha Omega Alpha, American Association for Hand Surgery, American Association of Plastic Surgeons, American Society for Reconstructive Microsurgery, American Society for Surgery of the Hand, American Society of Plastic Surgeons, and Plastic Surgery Research Council

Disclosure: Nothing to disclose.

Specialty Editor Board

A Lee Osterman, MD  Director of Hand Surgery Fellowship, Director, Philadelphia Hand Center; Director, Professor, Department of Orthopedic Surgery, Division of Hand Surgery, University Hospital, Thomas Jefferson University

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Michael Yaszemski, MD, PhD  Associate Professor, Departments of Orthopedic Surgery and Bioengineering, Mayo Foundation, Mayo Medical School

Disclosure: Nothing to disclose.

Dinesh Patel, MD, FACS  Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital

Dinesh Patel, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Harris Gellman, MD  Consulting Surgeon, Broward Hand Center; Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami, Leonard M Miller School of Medicine

Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, and Arkansas Medical Society

Disclosure: Nothing to disclose.

Additional Contributors

I would like to thank Dr. William Dahl, MD, who helped compile new references for this revision.

References
  1. Schwabe GC, Mundlos S. Genetics of congenital hand anomalies. Handchir Mikrochir Plast Chir. 2004;36:85-97. [Medline].

  2. Mandal K, Phadke SR, Kalita J. Congenital swan neck deformity of fingers with syndactyly. Clin Dysmorphol. Apr 2008;17(2):109-11. [Medline].

  3. Malik S, Afzal M, Gul S, Wahab A, Ahmad M. Autosomal dominant syndrome of camptodactyly, clinodactyly, syndactyly, and bifid toes. Am J Med Genet A. Sep 2010;152A(9):2313-7. [Medline].

  4. Flatt AE. The Care of Congenital Hand Anomalies. St Louis, Mo: Quality Medical Publishing; 1977.

  5. Poland, Alfred. Deficiency of the pectoral muscles. Guy's Hospital Reports. 1841;VI:191-193.

  6. Upton J. Apert syndrome. Classification and pathologic anatomy of limb anomalies. Clin Plast Surg. Apr 1991;18(2):321-55. [Medline].

  7. De D, Narang T, Kanwar AJ, Dogra S. Brachycephaly and syndactyly: Apert's syndrome. Indian J Dermatol Venereol Leprol. Jul-Aug 2008;74(4):395-6. [Medline].

  8. Patterson TJ. Congenital ring constriction. Br J Plast Surg. 1961;14:1-31. [Medline].

  9. Percival NJ, Sykes PJ. Syndactyly: a review of the factors which influence surgical treatment. J Hand Surg [Br]. May 1989;14(2):196-200. [Medline].

  10. Eaton CJ, Lister GD. Syndactyly. Hand Clin. Nov 1990;6(4):555-75. [Medline].

  11. Lamb DW, Wynne-Davies R, Soto L. An estimate of the population frequency of congenital malformations of the upper limb. J Hand Surg [Am]. Nov 1982;7(6):557-62. [Medline].

  12. Light TR. Congenital anomalies: syndactyly, polydactyly, and cleft hand. In: Peimer CA, ed. Surgery of the Hand and Upper Extremity. New York, NY: McGraw-Hill: 1996: 2111-44.

  13. Man LX, Chang B. Maternal cigarette smoking during pregnancy increases the risk of having a child with a congenital digital anomaly. Plast Reconstr Surg. Jan 2006;117(1):301-8. [Medline].

  14. Vekris MD, Lykissas MG, Soucacos PN, Korompilias AV, Beris AE. Congenital syndactyly: outcome of surgical treatment in 131 webs. Tech Hand Up Extrem Surg. Mar 2010;14(1):2-7. [Medline].

  15. Agarwal A. Comment on Shevtsov and Danilkin: Application of external fixation for management of hand syndactyly. Int Orthop. Jul 16 2008;[Medline].

  16. Frick L, Fraisse B, Wavreille G, Fron D, Martinot V. [Results of surgical treatment in simple syndactily using a commissural dorsal flap. About 54 procedures]. Chir Main. Apr-Jun 2008;27(2-3):76-82. [Medline].

  17. Jose RM, Timoney N, Vidyadharan R, Lester R. Syndactyly correction: an aesthetic reconstruction. J Hand Surg Eur Vol. Jul 2010;35(6):446-50. [Medline].

  18. Upton J. Congenital anomalies of the hand and forearm: syndactyly. In: McCarthy JG, ed. Plastic Surgery. Philadelphia, Pa: WB Saunders Co: 1990: 5279.

  19. Sharma RK, Tuli P, Makkar SS, Parashar A. End-of-Skin Grafts in Syndactyly Release: Description of a New Flap for Web Space Resurfacing and Primary Closure of Finger Defects. Hand (N Y). Sep 19 2008;[Medline].

  20. Dillon CK, Iwuagwu F. Cavi-care dressings following syndactyly correction. J Plast Reconstr Aesthet Surg. Sep 24 2008;[Medline].

  21. Lumenta DB, Kitzinger HB, Beck H, Frey M. Long-term outcomes of web creep, scar quality, and function after simple syndactyly surgical treatment. J Hand Surg Am. Aug 2010;35(8):1323-9. [Medline].

  22. Barot LR, Caplan HS. Early surgical intervention in Apert's syndactyly. Plast Reconstr Surg. Feb 1986;77(2):282-7. [Medline].

  23. Colville J. Syndactyly correction. Br J Plast Surg. Jan 1989;42(1):12-6. [Medline].

  24. Wu JC, Cunningham BB. Ectopic acanthosis nigricans occurring in a child after syndactyly repair. Cutis. Jan 2008;81(1):22-4. [Medline].

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Dorsal view of a hand demonstrating simple incomplete syndactyly between the left long finger and ring finger. Note the incidental café-au-lait spot.
Palmar view of hand with syndactyly. The level of the syndactyly, just proximal to the proximal interphalangeal (PIP) joint, can be clearly seen on the palmar view.
Dorsal view of the hand of a 1-year-old child with a complete simple syndactyly. Note that both the long finger and the ring finger have distinct nail plates with a trough separating them.
Palmar view of the hand of a 1-year-old child with a complete simple syndactyly.
Dorsal view of hand of a 6-month-old patient with type I Apert syndrome. Note that the thumb is separate.
Radial view of hand of a 6-month-old patient with type I Apert syndrome. The index fingernail is separate, and the syndactyly is complete. The small fingernail is also separate from the rest of the hand. A simple syndactyly between the small finger and the rest of the hand is present. Note that the broad thumb and the abnormal curvature of the thumb is due to the presence of a delta phalanx seen in the x-ray below.
Radiograph of hand of the 6-month-old patient with type I Apert syndrome demonstrates the distinctive characteristics of Apert syndrome. In the small finger, the distal interphalangeal (DIP) is formed. Although a rudimentary proximal interphalangeal (PIP) joint is present, clinically this joint is stiff. In the ring, long, and index fingers, no PIP joint is present, and symphalangism is present between the middle and the proximal phalanges. The distal phalanges of the ring and long finger are fused. In the thumb, the proximal phalanx is shaped abnormally and is referred to as the delta phalanx. Synostosis of the ring and the small finger metacarpals is present.
Apert type II hand. Note the complete syndactyly between the ring and the small fingers. The patient's hand was complicated by a chronic paronychia and skin maceration preoperatively.
Apert syndrome (type III), dorsal view.
Apert syndrome (type III), volar view.
Radiograph of the left hand of a patient with Apert syndrome (type III). Note the complicated syndactyly with osseous union in the distal phalanges of all the fingers. Symphalangism is present between the proximal and middle phalanges, without the formation of a proximal interphalangeal (PIP) joint in the ring, long, and index fingers.
Poland Syndrome: Dorsal view of a left hand in a patient with Poland Syndrome with brachydactyly (short fingers) and adactyly (missing fingers) with associated simple incomplete syndactyly between the ring and the small finger
Volar view of the preceding hand in a patient affected with Poland syndrome.
Right hand of a patient with Poland syndrome. Note the incomplete syndactyly between the hypoplastic right index and long fingers.
Volar view of the index and long finger in incomplete syndactyly in a patient with Poland syndrome.
Dorsal view of the right hand of a 1.5-year-old patient with constriction band syndrome. The fingers can still be identified individually. Note the presence of a fistula tract between the affected fingers, particularly between the long finger and ring finger.
Palmar view of the right hand of a 1.5-year-old patient with constriction band syndrome. The fingers can still be identified individually. Note the presence of a fistula tract between the affected fingers, particularly between the long finger and ring finger.
Right hand of a 1.5-year-old patient with constriction band syndrome. Radiograph demonstrates that the level of the amputation occurred at the proximal interphalangeal (PIP) joints.
Dorsal view of left hand of a 1.5-year-old patient with constriction band syndrome. The left hand is more severely involved than the right, with all of the fingers being nearly indistinguishable from one another. Note the presence of pits between the fingers where normal webs would be. The most prominent one is between the ring and the small fingers. They often represent fistulas between the dorsal and the volar surface of the hand and are often the only thing remaining of the previous normal web space.
Volar view of left hand of a 1.5-year-old patient with constriction band syndrome. The left hand is more severely involved than the right, with all of the fingers being nearly indistinguishable from one another. Note the presence of a prominet fistula between the small finger and ring finger.
Radiograph of left hand of a 1.5-year-old patient with constriction band syndrome. The level of amputation is through the midportion of the proximal phalanges of the involved fingers.
Image of the left hand of a 1.5-year-old patient with constriction band syndrome after a second reconstructive procedure following release of the second and fourth web spaces. The syndactyly partially recurred because of a skin-graft loss. During the second-stage operation to separate the long finger and ring finger, the recurrence in the second and fourth web spaces were re-revised. Thus, sutures are present on both sides of the ring finger and long finger.
Palmar view of the right hand of a 1.5-year-old patient with constriction band syndrome after a previously staged syndactyly release. No skin graft was needed for the release between the long and ring fingers. The patient also underwent revision of the web space between the index and long fingers.
Complete simple syndactyly of the ring and small fingers. Note the ring finger proximal interphalangeal (PIP) joint flexion deformity due to the complete syndactyly between the border digits.
Complete simple ring and small finger syndactyly. Note the ulnar deviation of the ring finger due to the syndactyly of the small finger.
Paronychia in a patient with Apert syndrome. Despite the use of oral antibiotics and topical antibiotic solution, the paronychia did not fully resolve until the fingers were divided.
Left hand of a patient with Apert syndrome type II. The hand has already undergone a previous first-stage division. Bilateral releases were simultaneously performed to release the border digits along with deepening of the 1st webspaces using a 4-flap z-plasty.
Volar view of the left hand of a patient with Apert syndrome type II. The hand has already undergone first-stage division. Bilateral releases were simultaneously performed to release the border digits along with deepening of the 1st webspaces using a 4-flap z-plasty.
Left hand of a patient with Apert syndrome type II. This is a 6-month postoperative picture after second-stage release was performed for the central digits between the long finger and ring finger. Surgical release was performed with the standard zigzag incisions. Soft-tissue coverage for the phalanges was sufficient after the release; therefore, a pedicle groin flap was not needed. The long finger and ring finger shared a conjoint nail, which was also released successfully. This picture was taken during subsequent surgical revision of a tracheostomy.
Dorsal view of the left hand of a patient with Apert syndrome type II. This is a 6-month postoperative picture obtained after a second-stage release was performed for the central digits between the long finger and ring finger. Surgical release was performed with the standard zigzag incisions. Soft-tissue coverage for the phalanges was sufficient after the release; therefore, a pedicle groin flap was not needed. The long finger and ring finger shared a conjoint nail, which was also released successfully. This picture was taken during subsequent surgical revision of a tracheostomy.
Dorsal view of the hand of a 1-year-old patient with complete simple syndactyly between his long finger and ring finger. The typical zigzag incisions are marked on both the dorsal and volar surfaces. Note the length of the dorsal trapezoidal flap design. Because it needs to be inset at a 45° angle with the distal edge of the web at the midlevel of the proximal phalanges, the length of the flap needs to be long enough to achieve this goal.
Volar view of the hand of a 1-year-old patient with complete simple syndactyly between his long finger and ring finger. The typical zigzag incisions are marked on both the dorsal and volar surfaces. Note the length of the dorsal trapezoidal flap design. Because it needs to be inset at a 45° angle with the distal edge of the web at the midlevel of the proximal phalanges, the length of the flap needs to be long enough to achieve this goal. Note the incidental simian crease.
Hand of a 1-year-old patient with complete simple syndactyly between his long finger and ring finger. This picture was takenimmediately after the procedure. On the dorsal view, note the proximal skin graft on the ulnar and radial sides of the dorsal trapezoidal flap.
Hand of a 1-year-old patient with complete simple syndactyly between his long finger and ring finger. This picture was taken immediately after the procedure.
Dorsal view of left hand demonstrating a simple incomplete syndactyly between the long finger and ring finger. This incomplete simple syndactyly was released by using a V-to-M flap without the need for a skin graft. Note the markings.
Volar view of left hand demonstrates a simple incomplete syndactyly between the long finger and ring finger. Immediate postoperative results are shown. Note the markings.
Dorsal view of a left hand demonstrates a simple incomplete syndactyly between the long finger and ring finger. Immediate postoperative results are shown.
Immediate postoperative results are shown.
Immediate postoperative results are shown.
One month later, the hand has a sufficiently deepened web. However, hypertrophic and hyperpigmented scars are still present along the incision line.
One month later, the hand has a sufficiently deepened web. However, hypertrophic and hyperpigmented scars are still present along the incision line.
Skin-graft donor site. The donor site in the right inguinal region has healed well 10 months after the patient's syndactyly surgery. The graft harvested was able to resurface 2 web spaces during bilateral web-space releases. The incision is inconspicuous.
Hand of a 23-year-old patient who underwent releases of simple complete syndactyly of both the left third and fourth web spaces as an infant. The patient noticed progressive shortening of the web space and found it hard to wear protective gloves, necessary for employment in the fishing industry. This resulted in a poor fit of the glove and chronic irritation of the web. Note the radial deviation of the small finger due to the contracture from the patient's first surgery. The web space was deepened with local flaps and full-thickness skin grafts from the left groin.
Hand of a 26-month-old patient who underwent release of a complete simple syndactyly between the left ring and long fingers with flaps and full-thickness skin grafts at 7 months of age. The patient developed hypertrophic scarring along the incisions. This persisted despite conservative treatment with topical steroid cream and scar massage. Contracture was present mostly along the ring finger, causing tethering of the distal ring finger with a mild radial deviation. The contracture was released with multiple Z-plasties and the web deepened with a small full-thickness skin graft.
Hand of a 26-month-old patient with syndactyly who underwent release of a complete simple syndactyly of the left ring finger and long finger with flaps and full-thickness skin graft at 7 months of age. The patient developed hypertrophic scarring along the incisions, which persisted despite conservative treatment with topical steroid cream and scar massage. This contracture was present mostly along the ring finger, causing tethering of the distal ring finger with a mild radial deviation.
Hand of a 26-month-old patient who underwent release of a complete simple syndactyly between the left ring and long fingers with flaps and full-thickness skin grafts at 7 months of age. The patient developed hypertrophic scarring along the incisions. This persisted despite conservative treatment with topical steroid cream and scar massage. Contracture was present mostly along the ring finger, causing tethering of the distal ring finger with a mild radial deviation. The contracture was released with multiple Z-plasties and the web deepened with a small full-thickness skin graft.
Hand of a 26-month-old patient who underwent release of a complete simple syndactyly between the left ring and long fingers with flaps and full-thickness skin grafts at 7 months of age. The patient developed hypertrophic scarring along the incisions. This persisted despite conservative treatment with topical steroid cream and scar massage. Contracture was present mostly along the ring finger, causing tethering of the distal ring finger with a mild radial deviation. The contracture was released with multiple Z-plasties and the web deepened with a small full-thickness skin graft.
Hand of a 26-month-old patient who underwent release of a complete simple syndactyly between the left ring and long fingers with flaps and full-thickness skin grafts at 7 months of age. The patient developed hypertrophic scarring along the incisions. This persisted despite conservative treatment with topical steroid cream and scar massage. Contracture was present mostly along the ring finger, causing tethering of the distal ring finger with a mild radial deviation. The contracture was released with multiple Z-plasties and the web deepened with a small full-thickness skin graft. At 3 months after surgery, he was doing well and had no evidence of recurrence.
Hand of a 26-month-old patient who underwent release of a complete simple syndactyly between the left ring and long fingers with flaps and full-thickness skin grafts at 7 months of age. The patient developed hypertrophic scarring along the incisions. This persisted despite conservative treatment with topical steroid cream and scar massage. Contracture was present mostly along the ring finger, causing tethering of the distal ring finger with a mild radial deviation. The contracture was released with multiple Z-plasties and the web deepened with a small full-thickness skin graft. At 3 months after surgery, he was doing well and had no evidence of recurrence.
Hand of an 18-year-old patient of Mediterranean heritage who had undergone multiple syndactyly releases with a full-thickness skin graft harvested from the groin as an infant. The skin graft can be observed as hyperpigmented patches within the web space. Some parts of the full-thickness skin graft also were noted to be hair bearing. Parents should be informed of the potential sequelae of the full-thickness skin grafts as the infant matures.
Hand of an 18-year-old woman of Middle Eastern heritage who had undergone multiple syndactyly releases with a full-thickness skin graft harvested from her groin when she was an infant. The skin graft can be observed as hyperpigmented patches within the web space. Some parts of the full-thickness skin graft were also noted to be hair bearing. Parents should be informed of the potential sequelae of the full-thickness skin grafts as the infant matures.
 
 
 
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