Treatment of congenital syndactyly

Treatment of congenital syndactyly

F Fitoussi, M Lehanneur

Hôpital Trousseau, Paris

Plan• Generality

• Classification• Epidemiology

• Indication and timing of surgery

• Principle of surgery• Patient’s setup• Commissure reconstruction• Digital separation• Resurfacing• Paronychial reconstruction• Post-op care

• Specific situations

• Complications

• Conclusion

→ Exclude from this presentation:• Symbrachydactyly• Amniotic band syndrome

Swanson / International Federation of Societies for Hand Surgery Classification - 1983

Swanson AB, deGroot Swanson G, Tada K. A classification for congenital limb malformation. J Hand Surg. 1983;8(5):693–702

Oberg-Manske-Tonkin classification - 2013

Tonkin MA, Tolerton SK, Quick TJ, Harvey I, Lawson RD, Smith NC, et al. Classification of congenital anomalies of the hand and upper limb: development and assessment of a new system. J Hand Surg. 2013 Sep;38(9):1845–53

Epidemiology

• Incidence = 1 in 2000 to 3000 live births

• One of the most common congenital hand deformities

• 2/1 ratios in favor of males and of the Caucasian population

• 10% to 40% of patients have a family history of previous similar malformations

Jordan D, Hindocha S, Dhital M, Saleh M, Khan W. The epidemiology, genetics and future management of syndactyly. Open Orthop J 2012;6:14-27

Epidemiology

• In isolated syndactyly:• both hands are involved in 50% of

cases

• The 3rd (50%) and 4th interdigital (30%) spaces are the most commonly affected

• In syndromic syndactyly : the 1st

and 2nd web spaces are relatively more frequently involved

Waters PM, Bae DS. Syndactyly. In: Waters PM, Bae DS, editors. Pediatric Hand and Upper Limb Surgery: a practical guide. Philadelphia: Lippincott Williams & Wilkins; 2012, p. 12-25.

General examination

Assess:

• The whole affected limb and:

• Other limbs

• Chest wall

• Craniofacial areas → syndromic concomitant malformations

(Poland, Apert’s syndrome…)

→ genetic counseling

Classification

• Complete or incomplete

• Simple, complex and complicated

Ger E. Syndactyly. In: Buck-Gramcko D, editor. Congenital Malformation of the Hand and Forearm. London: Churchill Livingstone; 1998, p. 131-40Tonkin MA. Failure of differentiation part I: Syndactyly. Hand Clin 2009;25:171-93

Indications of surgery • Surgical management is indicated in most cases

• Surgery is not indicated in complex cases without sufficient osteo-articular tissues to provide stable and mobile independent digits, since separation may worsen hand function

Ger E. Syndactyly. In: Buck-Gramcko D, editor. Congenital Malformation of the Hand and Forearm. London: Churchill Livingstone; 1998, p. 131-40

Timing of surgery• Remains controversial

• Surgical program should end before three years of age

• Syndactyly between rays of different lengths induces angular deformities of the longer digit • Result in fixed camptodactyly

and/or clinodactyly• 1st , 2nd and 4th interdigital spaces =

before one year• 3rd interdigital space = between 12

and 18 months

Buck-Gramcko D. Syndactyly Between the Thumb and Index Finger. In: Buck-Gramcko D, editor. Congenital Malformation of the Hand and Forearm. London: Churchill Livingstone; 1998, p. 141-7

Flatt AE. Practical factors in the treatment of syndactyly. In: Littler JW, Cramer LM, Smith JW, editors. Symposium on reconstructive hand surgery. St. Louis: The CV Mosby Company; 1974, p. 144-56

Timing of surgery

• Most authors advocate staged separation surgeries when a finger is webbed on both sides

• Syndactyly of all interdigital spaces will need two procedures:• first release of the first and third

webs

• and 3 to 6 months later the release of the second and fourth webs

Kozin SH, Zlotolow DA. Common Pediatric Congenital Conditions of the Hand. Plast Reconstr Surg 2015;136:241e-57e

Timing of surgery• Some authors recently reported on single-staged release of all fingers• Using dorsal gull-wing flaps• Preoperative CT angiography to demonstrated at least one digital artery in

all fingers →All were simple syndactyly

Tian X, Xiao J, Li T, Chen W, Lin Q, Chim H. Single-Stage Separation of 3- and 4-Finger Incomplete Simple Syndactyly With Contiguous Gull Wing Flaps: A Technique to Minimize or Avoid Skin Grafting. J Hand Surg Am 2017;42:257-64

Hynes SL, Harvey I, Thomas K, Copeland J, Borschel GH. CT angiography-guided single-stage release of adjacent webspaces in non-Apert syndactyly. J Hand Surg Eur Vol 2015;40:625-32

Principles of surgery

1. Patient’s setup

2. Commissure reconstruction

3. Digital separation

4. Resurfacing

5. Paronychial reconstruction

6. Post-op care





4. Resurfacing


6. Post-op care

Patient’s setup

• Ambulatory surgery

• General anesthesia

• Sterile tourniquet for access to the arm and elbow for skin graft harvest if necessary

• Optical magnification

• Anatomical landmarks using a skin-marker pencil:• metacarpal heads• longitudinal midlines of each digit• relation between dorsal and palmar flaps

• Measurements of proximal thickness of the conjoined digit





4. Resurfacing


6. Post-op care

Commissure reconstruction

Use of skin flaps is widely accepted

Second, third and fourth normal web spaces present with a 45° dorso-palmar slope

Chouairi F, Mercier MR, Persing JS, Gabrick KS, Clune J, Alperovich M. National Patterns in Surgical Management of Syndactyly: A Review of 956 Cases. Hand (N Y) 2019

Commissure reconstruction: Incomplete syndactyly• Three-flaps web plasties : Several designs have proposed over the years

(MOSS 1990; OSTROWSKI 1991 BANDOH 1997…).

• If webbing is proximal to the proximal phalanx distal epiphysis: island flap and translate it proximally as in “V-Y”-plasty

1 2 3 4

5 6 7 8

1: eaton, 2: ostrowski, 3: flatt, 4: bandoh, 5: yao, 6: yamashita, 7: tadiparti, 8: brennen

Island flaps

Three-flaps

web plasties

Ostrowski DM, Feagin CA, Gould JS. A three-flap web-plasty for release of short congenital syndactyly and dorsal adduction contracture. J Hand Surg Am 1991;16:634-41

Ostrowski’s plasty

Dorsal flaps

1 2 3 4

Palmar flaps

5 6

Combined dorsal and

palmar flaps

7 8 9 10 11

1: flatt, 2: vekris, 3: hourglass, 4: omega, 5: delord, 6: jose, 7: karacoglan, 8: killian, 9: karamese, 10: savaci, 11: mericli

Commissure reconstruction: Complete syndactyly


• Proximally-based dorsal flap is the most commonly used technique• Starting proximally at the level of

the metacarpal heads

• flap length is equal to the antero-posterior thickness of conjoined digits

• About 1/2 ratio

• Anastomosis digital artery and dorsal intermetacarpal artery

Island flaps

Proximally-based flaps

1 2 3 4

5 6 7

8 9 10 11

1: gao, 2: wang, 3: ni, 4: dong, 5: sahin, 6: niranjan, 7: tian, 8: hsu, 9: liu, 10: sherif, 11: yildrim

Dorsal flaps: Numerous shapes (pentagonal, hexagonal, bilobed, double wing-shaped, seagull wing–shaped…)


• Proximally-based dorsal flap : Numerous variations have been proposed:• hourglass flaps (GLICENSTEIN 1998,

MALLET 2013)

• trapezoid flaps (VEKRIS 2010)

Col. P. JehannoGlicenstein J. [What's new in syndactyly?]. Ann Chir Plast Esthet. 1998

Dec;43(6):611-6


• Proximally-based dorsal flap : Numerous variations have been proposed:• Dorsal omega flaps

(DARCANGELO 1996) with palmar ancor

D’Arcangelo M, Gilbert A, Pirrello R. Correction of syndactyly using a dorsal omega flap and two lateral and volar flaps. A long-term review. J Hand Surg Br 1996;21:320-4





4. Resurfacing


6. Post-op care

Digital separation

• Matched or non mached broken-line incisions to elevate palmar and dorsal laterally-based interdigitating flaps• Avoid retractile scar formation

• Avoid web creep

• Anticipate coverage of zone of interest (i.e., exposed bone or joint)

Cronin TD. Syndactylism: results of zig-zag incision to prevent postoperative contracture. Plast Reconstr Surg 1956;18:460-8.

Digital separation

• Precautious dissection • from distal to proximal

• from dorsal to palmar

• Release all fibrous connections

• Identify the neurovascular structures• If the division of the common

interdigital artery is too distal, one of the two branches may be ligated

Digital separation

• Identify the neurovascular structures• provided that the contralateral digital

artery is known to be intact• clamping test ++

• Nerve: intraneural dissection

• Once all flaps are positioned tourniquet is released for hemostasis before grafting





4. Resurfacing


6. Post-op care

Resurfacing - Controversy : with or without skingraft?

Most of the recent studies described satisfactory results with graftless procedures:• Reduce operating time• No donor site morbidityResurfacing is achieved through flap positioning and defatting, as well as leaving open the residual defects

Ni F, Mao H, Yang X, Zhou S, Jiang Y, Wang B. The Use of an Hourglass Dorsal Advancement Flap Without Skin Graft for Congenital Syndactyly. J Hand Surg Am 2015;40:1748-54

Tian X, Xiao J, Li T, Chen W, Lin Q, Chim H. Single-Stage Separation of 3- and 4-Finger Incomplete Simple Syndactyly With Contiguous Gull Wing Flaps: A Technique to Minimize or Avoid Skin Grafting. J Hand Surg Am 2017

Resurfacing: Controversy with or without skingraft?

• Recent prospective comparative study or retrospective comparative study = advantages in appearance and web creep in the group with skin graft

Author Date Journal Number of syndactylies Skin graft Mean follow-

up Years)

Level of Evidence

Landi 2014 JHS Eur 26 Hyalomatrix 2 Therapeutic IV

Ni 2015 JHS Am 116 No 4.2 Therapeutic IV

Duteille 2016 JHS Eur 40 Matriderm Therapeutic IV

Widerberg 2016 JHS Eur 29 No 16 Therapeutic IV

Sullivan 2017 JHS Am Systematic review : 34

articles

Comparative

graft/no graft

N/A Therapeutic IV

Tian 2017 JHS Am 74 No graft in 21 out

of 31 patients

1 Therapeutic IV

Dong 2017 Medicine

(Baltimore)

35 No 4.6 Therapeutic IV

Yuan 2018 PRS 45 Comparative

graft/no graft

Therapeutic III

Ferrari 2019 JHS Eur 49 Comparative

graft/no graft

7.4 Therapeutic IV

Wang 2019 JHS Am 22 No 1 to 2 Therapeutic IV

Wang 2019 JHS Eur 28 Comparative

graft/no graft

4.3 Therapeutic II

Wang AA, Hutchinson DT. Syndactyly release: a comparison of skin graft versus graftless techniques in the same patient. J Hand Surg Eur Vol 2019Yuan F, Zhong L, Chung KC. Aesthetic Comparison of Two Different Types of Web-Space Reconstruction for Finger Syndactyly. Plast Reconstr Surg 2018;142:963-71

Failed graftless with Hyalomatrix

Donor site

• Full-thickness grafts: • Anterior Elbow crease

• Wrist flexor crease

• Medial part of the arm

• Groin area

• Retroauricular area

• Abdomen…

→Should match skin color

Sulser PS, Kalisch M, Weber DM. Retroauricular full-thickness skin grafts in syndactyly repair: outcome and comparison with inguinal full-thickness skin grafts: retrospective (cross-sectional) study. J Plast Surg Hand Surg 2016





4. Resurfacing


6. Post-op care

12 3

Paronychial reconstruction

• Complex syndactyly• fused phalangeal tufts

• bony elements are exposed at the end of the release

• Pulp flaps:• 1: buck gramcko• 2: sugihara• 3: lundkvist

Paronychial reconstruction

Two laterally-based long and narrow triangular flaps





4. Resurfacing


6. Post-op care

Post op care

• Surgical wounds are closed without tension using 5.0 absorbable sutures

• The newly separated web space is maintained as opened as possible without compromising flap and/or digit vascularization • Most authors recommend to use

only gauze to open to web space

• First dressing change is made 7 to 21 days after surgery

Patel NK, Hoehn JG. "Sandwich" dressing for pediatric hand surgery. Plast Reconstr Surg 2009;123:213e-4e

Post op care

• In complicated syndactyly, the newly separated web space can be maintained opened with an external fixator when needed

Kamath JB, Vardhan H, Naik DM, Bansal A, Rai M, Kumar A. A novel method of using mini external fixator for maintaining web space after the release of contracture and syndactyly. Tech Hand Up Extrem Surg 2013;17:37-40

Artuso M, Mas V, Ilharreborde B, Mazda K, Jehanno P. External fixation: Role in decreasing postoperative complications of complex syndactyly release - A reviewof 18 patients. Orthop Traumatol Surg Res. 2019 Jul 25

Specific situations

Specific situations:First web space syndactyly

• Mild syndactyly:• “Z”-plasty• Four-flaps “Z”-plasty ++

• Dorsal advancement rotation flap • Preliminary tissue expansion on

the dorsum of the hand• Transposition flap from the radial

border of the index• Severe contracture = free groin,

forarm or lateral arm flap

Gülgönen A, Güdemez E. Reconstruction of the first web space in symbrachydactyly using the reverse radial forearm flap. J Hand Surg Am 2007;32:162-7

First web space syndactyly : large advancement-rotation flap raised from the dorsum of the hand

Buck-Gramcko D. Syndactyly Between the Thumb and Index Finger. In: Buck-Gramcko D, editor. Congenital Malformation of the Hand and Forearm. London: Churchill Livingstone; 1998, p. 141-7

Ghani HA. Modified dorsal rotation advancement flap for release of the thumb web space. J Hand Surg Br 2006;31:226-9

Complicated syndactylyPolysyndactyly

• Can be associated with:• Clinodactyly• Brachydactyly• Symphalangism• Synostosis

• Ultrasound or magnetic resonance imaging may precise the tendon and neurovascular anatomies

• Problem with IP stiffness, joint instability, flexors/extensors tendon insertions….

Kay SP, McCombe DB, Kozin SH. Deformities of the hand and fingers. In: Wolfe SW, editor. Green’s Operative Hand Surgery Vol 2, 6th ed. Philadelphia: Elsevier Churchill Livingstone; 2011, p. 1303-69

Syndromes

Apert’s syndrome• Autosomic dominant and mutations

• Mutation of the fibroblast growth factor receptor type 2 gene (FGFR2)

• Severe complex syndactyly of the hands and feet• Complex syndactyly of the index, long,

and ring fingers• Simple syndactyly between the ring and

small finger• Incomplete first web space syndactyly• Radial clinodactyly of the thumb

• Abnormalities of the shoulder and elbow

• Craniostenosis, Mid facial hypoplasia, Hypertelorism

Apert’s Syndrome

• The goal of surgery is to complete separation of the digits and correct the thumb deformity before 2 years of age • preop soft tissue expansion (Lohmeyer)

• Reconstruction of an adequate first web space = first priority

• Thumb clinodactyly = corrective osteotomy (opening wedge)

• Release of the fourth and fifth metacarpal synostosis between the ages of 4 and 6 years

Guero SJ. Algorithm for treatment of Apert hand. Tech Hand Up Extrem Surg. 2005 Sep;9(3):126-33

Poland’s Syndrome

• Attributed to disruption of the blood flow in the subclavian artery in the embryo

• Sternocostal head of pectoralis major agenesis

• Brachymésophalangy or une symbrachydactyly• Always unilateral• Smaller hand• Stiff fingers• Clinodactyly

Poland’s Syndrome

• Associated anomalies: • Hypoplasia of breast• Costal hypoplasia• Lung hernia• Scapula hypoplasia• Short axillary fold• Radioulnar synostosis

Complications

Complications

• Digital ischemia

• Flap necrosis

• Graft loss

• Wound dehiscence

• Delayed healing

• Superficial surgical site infections (most common)

→ about 2% of cases

McQuillan TJ, Hawkins JE, Ladd AL. Incidence of Acute Complications Following Surgery for Syndactyly and Polydactyly: An Analysis of the National Surgical Quality Improvement Program Database from 2012 to 2014. J Hand Surg Am 2017;42:74-9

Complications

• Web creep = distal translation of the commissure

• Palmar scar contractures with growth-related frontal and/or sagittal angulations

• Joint motion limitation

• Nail dystrophy

• Keloid formation

→ complexity of the malformation++

Comer GC, Ladd AL. Management of complications of congenital hand disorders. Hand Clin 2015;31:361-75Lumenta 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 2010;35:1323-9

Complications: Web-creepFlexion-extension deformity 0 Normal digit

1 Finger can not be hyperextended

2 Reductible flexion deformity

3 Fixed finger deformity

Web-creep 0 Soft web. Abduction mirrors the adjacent web or equivalent controlateral web

1 No web advancement but thickening of the web with reduced span

2 Creep of web to 1/3 of the distance betwwen base of the web and PIPJ crease

3 Creep of web to 2/3 of the distance betwwen base of the web and PIPJ crease

4 Creep of web to the PIPJ crease

Withey SJ, Kangesu T, Carver N et al. The open finger technique for the release of syndactyly. J Hand Surg Br. 2001;26B:1:4-7

Palmar scar contracture and web creep

Results

• Dorsal Omega flap• Palmar ancor• Skin graft

Mallet C, Ilharreborde B, Jehanno P, Litzelmann E, Valenti P, Mazda K, et al. study of 2 commissural dorsal flap techniques for the treatment of congenitalsyndactyly. J Pediatr Orthop 2013;33:197-204

Conclusion

• One of the most common deformity of the hand congenital deformity

• Initial examination must focus on the determining the syndromic or isolated

• Numerous techniques have been described • very little comparative data exists

• The physician has the choice regarding the technique to use

• Outcomes are most commonly satisfactory, provided that the syndactyly is simple and that basic operating rules are followed

Thank You

Thanks to Adeline Cambon-Binder for the drawings

Documents

Treatment of congenital syndactyly