Beruflich Dokumente
Kultur Dokumente
CASE REPORT
KEYWORDS
Acrocephalosyndactylia/surgery;
Chondroitin sulphates/
therapeutic use;
Hand deformities,
congenital/surgery;
Reconstructive surgical
procedures;
Skin, artificial;
Syndactyly/surgery
Summary The reconstruction of the third web space in Apert syndactyly often involves pedicled groin flaps to resurface exposed distal (and sometimes proximal) phalanges. We report
a case in which the right-hand third web space was reconstructed with traditional pedicled
groin flaps and the left hand with the Integra regenerative skin template. We report that both
left and right hands achieved similar outcomes, but the hand reconstructed with groin flaps
required debulking, whilst the hand reconstructed with Integra was easier to care for.
2011 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by
Elsevier Ltd. All rights reserved.
* Corresponding author. 555 University Ave. Rm 5547 Roy C. Hill Wing, Toronto, ON, Canada M5G 1X8. Tel.: 1 416 813 7654x28197; fax: 1
416 813 6637.
E-mail address: gregory.borschel@sickkids.ca (G.H. Borschel).
1748-6815/$ - see front matter 2011 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.
doi:10.1016/j.bjps.2011.06.033
119
Introduction
First described by Eugene Apert in 1906, Apert syndrome is
associated with craniosynostosis, midface hypoplasia and
complex symmetrical bilateral syndactylies of the hands
and feet. The deformity in Apert hands includes syndactyly
and symphalangism of the index, middle and ring fingers;
syndactyly of the ringelittle finger web; fourth and fifth
metacarpal synostosis; and short thumbs with radial clinodactyly. There are three different types of Apert hand,
which correlate to the severity of functional and anatomical impairment.1,2
Operations for Apert hands (for type 1 or 2 hands) may
start at age 6 months with bilateral release of the second
and fourth web spaces with reconstruction using a dorsal
flap and full-thickness skin grafts. Separation of the third
web takes place 3e6 months after release of the second
and fourth webs. This separation often leaves significant
area of bony exposure with inadequate soft-tissue
coverage. One popular method to cover the bony defect
is the use of pedicled groin flaps as described by Zuker
et al.2e4 However, groin flaps can be challenging for families to manage, sometimes require prolonged in-patient
management in small children, and sometimes require
revision for dehiscence or bulkiness.
Integra (Integra LifeSciences Corporation, NJ, USA) is
a bilayer skin regenerative template with a thin silicone
outer layer and a collagen matrix inner layer.5,6 The use of
Integra for treatment of thermal injuries and scar
contracture has been described previously.6,7 We present
a method of two-stage reconstruction technique of the Apert
third web using Integra template and full-thickness skin
graft. We present this method as an alternative for patients
in which a groin flap may be difficult for families to care for.
Figure 1
Case report
A 7-month-old boy presented for surgical treatment of type
2 Apert hands (Figures 1 and 2). He first underwent
syndactyly release of his bilateral second and fourth web
spaces followed by reconstruction using dorsal flaps and
full-thickness skin grafts. Seven months later, he underwent syndactyly release of the third web on his right hand
with a dorsal flap and full-thickness coverage of the web
space defects in the form of a pedicle groin flap. The family
had difficulty caring for the flap, citing difficulties with
hygiene, and the child being inconsolable during immobilisation. Eight days after the operation, the groin flap
became dehiscent and the patient was returned to the
operating room for re-insetting. Two weeks later, we
divided the groin flap and inset it to the ulnar side of the
middle finger and the radial side of the ring finger. The flaps
were treated postoperatively with light compressive
wrapping. Three months later, an operation to release the
syndactyly on the left hand was planned. This time, instead
of using a pedicle groin flap, we used the Integra template
to cover the area over exposed bone and joints. We inset
a piece of Integra on both sides of the third web space
using interrupted 5/0 chromic gut sutures. Three weeks
later, the patient was returned to the operating room, and
the outer silicone layer of Integra was removed and used
Figure 2
120
of the ring finger from the previous groin flap was undertaken 13 months after the right-hand operation. The
patient also developed web space creep on the left third
web space, which was released 8 months after the left hand
operation. The patient subsequently developed acceptable
appearance and function of his hands (Figures 3 and 4).
Images are used with permission from the patients family
in accordance with the Declaration of Helsinki and the
guidelines of our institutional review board.
Discussion
Several methods to cover the bony defect after release of
the third web space syndactyly in Apert hands have been
described. Chang et al.2 preferred using only local flaps and
full-thickness skin grafts from the groin. Some surgeons
have used double-opposing triangular pulp flaps to recreate
the nail folds or toe pulp grafts to cover the entire bony
defect.8 However, this method may result in pointed
fingertips with nail distortion. Zuker et al.3 advocated the
use of pedicle distant flaps as this method has less risk of
scar contracture over the distal joint and subsequent
deviation with growth. Interestingly, Stafansson and Stilwell9 interposed a 1-mm-thick silastic sheet over the
separated bones before applying a full-thickness skin graft
a month after. In this report, we demonstrated the use of
Integra template to cover the bony defect left by
osteotomy of the fused phalanges of the third web.
Our patient underwent reconstruction of the right third
web space using pedicle groin flaps. Postoperatively, his
parents had difficulty keeping the hand immobilised, and the
groin flaps dehisced from the hand despite the hand being
tethered to the torso with heavy temporary sutures and
binding the hand to the torso with elastic wraps. In light of
Conclusion
Funding
None.
Conflict of interest
None of the authors has any financial interest in any
product mentioned in this manuscript.
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