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SURGICAL TECHNIQUE

The Apert Hand—Angiographic Planning of

a Single-Stage, 5-Digit Release for All Classes

of Deformity

Isaac Harvey, MBBS, Scott Brown, Oliver Ayres, MD, Timothy Proudman, MD

Purpose To demonstrate the utility of computed tomography angiographic planning of asingle-stage, complete release of syndactyly in Apert syndrome.

Methods Computed tomography angiograms were performed as a preoperative planning toolin 6 patients. Five came to surgery. All had a single-stage operation for complete release oftheir syndactyly.

Results Five patients, ranging from Upton type 1 to type 3 Apert hand deformities, have hadpreoperative computed tomography angiography that delineated the vascular anatomy. Thisallowed planning and execution of a single-stage syndactyly release in all patients. Thepreoperative imaging identified noteworthy abnormalities in vascular anatomy that wereincorporated into surgical planning.

Conclusions The protocol presented allows preoperative planning and single-stage operation forcomplete release of syndactyly in patients with Apert syndrome. (J Hand Surg 2012;37A:152–158. Copyright © 2012 by the American Society for Surgery of the Hand. All rights reserved.)

Key words Angiography, Apert, single stage, syndactyly, vascular.

APERT SYNDROME WAS originally described1 in1906. The hand in Apert syndrome is per-haps one of the most challenging problems

acing the pediatric hand surgeon. The patholo-ic anatomy has been described by several au-hors.2–10 It involves all components of the hand,

including skin, bone, nerves, tendons, and vessels.Various classifications have been used to assistsurgeons in planning surgery.5,11,12 The Upton11

From the Department of Medical Imaging and Department of Plastic and Reconstructive Surgery,Women’s and Children’s Hospital, North Adelaide, South Australia.

The authors would like to acknowledge the support of the Australian Craniofacial Unit and Profes-sor David David for patient referrals and the Department of Medical Imaging and Dr. Rebecca Linkefor their collaboration with this work.

Received for publication November 30, 2010; accepted in revised form October 12, 2011.

No benefits in any form have been received or will be received related directly or indirectly to thesubject of this article.

Corresponding author: Isaac Harvey, MBBS, Women’s and Children’s Hospital, Department ofPlastic and Reconstructive Surgery, 57 King William Rd, North Adelaide, South Australia 5006;e-mail: harveyi@live.com.

0363-5023/12/37A01-0031$36.00/0

doi:10.1016/j.jhsa.2011.10.017

152 � © ASSH � Published by Elsevier, Inc. All rights reserved.

classification is most commonly used; it dividesthe hand deformity into 3 types that correspondwith increasing severity. Type 1 consists of a com-plex (osseous and soft-tissue) syndactyly of digits2 through 5, with the thumb free. This is describedas the spade hand. A type 2 deformity involves acomplex syndactyly of digits 2 through 5, with anassociated simple syndactyly of the thumb. Thishas been described as the spoon or mitten hand. Atype 3 deformity consists of complex syndactyly ofdigits 1 through 5, with complex syndactyly ofthe thumb; it has been described as the rosebudhand.

Many variations of surgical release of the first webspace and of the remaining syndactyly have been de-scribed.7–10,12–14 Various approaches to the bony de-formity of the thumb have also been described.4,8,12,15

All previously described techniques advocate releasinga single side of a digit at any given surgery to maintainthe vascularity of that digit. This is due to the unreli-

ability of the vascular branching pattern to the digits.11

ANGIOGRAPHY PLANNING SINGLE-STAGE APERT HAND 153

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Based on work in imaging of perforators in free flapbreast reconstruction,17,18 we have developed a proto-col for preoperative imaging of the Apert hand thatenables visualization of the arterial anatomy to a leveldistal to the distal interphalangeal joints (Fig. 1). Thisinformation is used by the surgeon to plan and executea single-stage syndactyly release of the entire hand,which reduces the total number of operations a child

FIGURE 1: Representative images of the 3-dimensional angiogtype 2, and D type 3 apert hands.

must have.

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PATIENTS AND METHODSAll patients who presented to our institution withApert hand deformity were assessed concurrentlyby both craniofacial and plastic surgery depart-ments. As part of the work-up for craniofacialsurgery, these patients all had planning computedtomography (CT) scans of the calvaria and facialbones, under general anesthesia. Patients who had

s of the Apert hand deformity. Angiograms of A, B type 1, C

ram

not had previous surgery on the hand had preop-

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erative, bilateral CT angiography of the upper limbduring the same scan. This reduces the total num-ber of anesthetics and can reduce the overall radi-ation exposure of these patients because, wherepossible, patients were positioned in the proneposition with arms above the head. Of the 6 pa-tients, only 1 was scanned in the prone positionwith the hands above the head. The remaining 5were scanned with the hands by the side due totechnical considerations. With the short limbs andturricephalic skulls found in the Apert patient, thefully extended hand might not be above the vertexof the skull. Age range of patients was 9 months to6 years. This large age range exists because mostpatients were overseas patients presenting for thefirst time to our institution.

The scanner used was a General Electric Dis-

TABLE 1. Representative Age-Adjusted and Weight

Route Patient Age (Weight) Agent

Intravenous 9 mo (7 kg) Omnipaque 350

Saline

Intravenous 2 y (13 kg) Omnipaque 350

Saline

Intravenous 6 y (20 kg) Omnipaque 350

Saline

TABLE 2. Scan Protocol for Acquisition of Images

Indications:Apert syndromePreoperative separation of digitsPostoperative separation of digits

Scan range:Start: 1 cm proximalEnd: Clear distal pha

ThenStart: Clear distal phaEnd: 1 cm proximal t

Acquisition:Routine helical

Landmark:Wrist joint

Patient Age(Weight) Scan Mode

Detector/SliceConfiguration

Pitch/TSpee

9 mo (7 kg) High-resolutionhelical

32 � 0.625 (20 mm) 0.531:1,

2 y (13 kg) High-resolutionhelical

32 � 0.625 (20 mm) 0.531:1,

6 y (20 kg) High-resolutionhelical

32 � 0.625 (20 mm) 0.531:1,

n/a, noise index (denotes “not applicable”).

covery CT 750HD CT scanner (Waukesha, WI).

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Radio contrast material lohexol 350 mg/mL (Om-nipaque 350; Winthrop, Princeton, NJ) was in-jected intravenously at a dose adjusted accordingto body weight (Table 1).

Refinements in the timing of contrast adminis-tration and acquisition of films were made over thefirst few patients. A protocol was developed thatenabled optimal image capture with minimal radi-ation dosage (Table 2). Images were acquired ofboth arterial and venous phases. Imaging of ves-sels to beyond the distal interphalangeal joint waspossible in 5 of 6 patients.

With our protocol, the total radiation exposure,or dose length product, for a CT angiogram of thehands averaged 450 mGycm during both arterialand venous phases. The venous images did notaffect the planning of surgery. Potentially, arterial-

justed Dosage of Contrast

Volume Delay

4 mL at 2.0 mL/s (maximum 2 mL/kg) Use timing bolus

4 mL at 2.0 mL/s (Same as contrast)

0 mL at 2.0 mL/s (maximum 2 mL/kg) Use timing bolus

0 mL at 2.0 mL/s (same as contrast)

6 mL at 3.0 mL/s (maximum 2 mL/kg) Use timing bolus

0 mL at 3.0 mL/s

ist joints

esst joint

Patient positioning:Supine, head firstCentral to tableHands by sides, palms upWhen patient can be positioned arms up with the

wrist clear of the head, prone, palms down

Bismuth shielding:Nil applicable

Scan Field ofView kV mA

NoiseIndex

RotationTime

AdaptiveStatisticalIterative

Reconstruction

Pediatric body 100 140 n/a 0.5 VS30

Pediatric body 100 150 n/a 0.5 VS30

Pediatric body 100 120 n/a 0.5 VS30

-Ad

1

1

2

2

3

3

to wrlange

lango wri

abled

10.62

10.62

10.62

phase scans only would be sufficient to plan sur-

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secti

ANGIOGRAPHY PLANNING SINGLE-STAGE APERT HAND 155

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gery, thus halving the radiation dose. This resultsin an exposure, or dose length product, that is lessthan that for a standard head CT performed in thetrauma setting.

We were able to manipulate 3-dimensional recon-structions of the angiograms to allow operative plan-ning (Fig. 1). The images were then analyzed toidentify at least one artery to each digit, demon-strating continuity of contrast material. The posi-tion of these arteries was recorded, and surgicaldissection was planned to ensure that each arterywas included in the correct digit. Abnormalities inthe anatomy were identified and incorporated intothe surgical plan. Syndactyly releases were per-formed using dorsal rectangular flaps for the webspace, straight-line incisions, and full-thicknessskin grafts from the lateral groin crease, to avoidthe hair-bearing skin. Graft inset was performedusing 5-0 absorbable sutures and a soft dressingconsisting of chloromycetin ointment, paraffingauze, gauze, and a crepe bandage with elastic tapein a boxing-glove style.

The first dressing check was performed at 1 weekafter surgery under ward sedation and, thereafter, asrequired.

RESULTSSix patients had CT angiography of both upper limbsfor Apert hand deformity. Two patients with type 1

FIGURE 2: A Distal branching of the common digital vesseldigital vessel in the second web into the index finger during dis

deformity, 2 patients with type 2 deformity, and 2

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patients with type 3 deformity were imaged. Of the 6patients, 1 who was an overseas patient did not receivehand surgery due to visa issues.

The remaining 5 patients all had bilateral surgi-cal release of syndactyly of all digits in a singleoperation (total of 10 hands). All patients that hadsurgery had successful complete release of theirmulti-ray syndactyly in a single operation. Noischemia, venous congestion, or need for amputa-tion was encountered. One patient had partial lossof grafts to the proximal aspect of a single digitthat healed with dressing changes.

Several anatomical abnormalities were identified be-fore surgery and were incorporated into the surgicalplan.

One patient demonstrated an extremely distalbranching of the common vessel of the second web(Fig. 2). The preoperative imaging suggested thatthis was the major blood supply to the index finger.Planning allowed this vessel to be identified andincorporated into the index finger, thereby ensur-ing the vascularity of this finger. The CT angio-gram demonstrated a sufficient ulnar digital vesselto the middle finger that was incorporated with thisdigit to also ensure its vascularity.

One patient demonstrated an extremely proxi-mal and large branch of the radial artery traversingthe wrist obliquely on the dorsum (Fig. 3). Thisvessel appeared to be the dominant blood supply to

second web. B Incorporating the distally branching commonon ensured vascularity of both digits.

in the

the ring and little fingers, with a minor contribu-

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tion from the palmar arch. From this vessel,branches were identified to both the ring and littlefingers. Therefore, extra care was taken in raisingdorsal skin flaps and the separation of these digitsto incorporate the dorsal vessel with each finger. Inthe early patients in the series, possibly due totiming of image acquisition, vessels were not ap-parent in the fingertips. However, in all patients,visualization and localization of the vessels to themid-level of the symphalangeal proximal phalanxwas possible.

DISCUSSIONSeveral variations on the surgical management of thehand deformity exist. In 1970, Hoover7 published areview of 200 cases in the literature and added 20 newcases. He reviewed the outcomes of surgical manage-ment and recommended amputation of a digit to sim-plify the procedure. Since that time, there has beendebate as to the merits of creation of a 5-digit versus a4-digit hand. Zuker10 describes one of the goals oftreatment as the creation of a 5-digit hand, whereasothers accept a 4-digit hand as necessary to allow cre-

FIGURE 3: Abnormal dorsal branch of radial artery as thedominant blood supply to the ring and small fingers.

ation of a sufficient first web space.12

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Emphasis has been placed on the importance ofreleasing border digits first, to limit the growth distur-bance created by differential growth of the border andcentral digits. Upton10 espoused release of the borderdigits at a first operation to allow for differentialgrowth, and Zuker10 similarly argues that border digitsshould be released first to avoid worsening of angulargrowth deformity. This results in a series of opera-tions—at least 3 operations for a type 3 hand. Patientswith Apert syndrome will have multiple operations intheir lifetime for their hands, possibly their feet, andtheir craniofacial deformities. Several authors havecommented on the need to minimize the number ofinterventions that these children will require. Fearon16

was able to decrease the number of operations on thehand by ignoring the principle of releasing border digitsfirst and releasing one side of the central digit at the firstoperation.

The dilemma surrounding the order of opera-tions arises as a result of uncertainty about thevascularity of a digit at the time of syndactylyrelease and the need to maintain a broad base alongone side of the digit to ensure this vascularity. Byusing preoperative CT angiography, we have beenable to relieve this uncertainty and perform releaseof the entire hand in one stage (Fig. 4).

At our institution, the scanning is done in con-junction with the craniofacial planning CT of thehead and face. Positioning the patient in the proneposition with the arms above the head providesseveral benefits. It keeps the radiation dose awayfrom the solid organs of the abdomen, improvesthe quality of the images due to reduced scatter,and enables a lower radiation dose to be used.However in 5 of our 6 cases, due to the short lengthof the limbs and turricephalic skulls of the Apertsyndrome, it was not possible to elevate the handsabove the head. In these cases, scans were per-formed with hands at the patients’ sides. Arterial-phase scans alone should be sufficient to plansurgery, thus halving the radiation dose.

The ability to manipulate the reconstructed im-ages in 3 dimensions allows unsurpassed visual-ization of the vascular anatomy. This allows forconfident planning of a single-stage syndactylyrelease operation. In our small series, there was nodiscrepancy between the preoperative vascularanatomy and that observed at surgery.

A single-stage release of the syndactyly provides thebenefit to the patient of a single operation and a singlerecovery period. For many of our patients who travel

from overseas and remain in our center during the

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ANGIOGRAPHY PLANNING SINGLE-STAGE APERT HAND 157

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recovery period, this means noteworthy savings interms of hospital bills and accommodation expenses, inaddition to the obvious benefit of fewer operations.

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1906;23:1310–1330.2. Blank CE. Apert’s syndrome (a type of acrocephalosyndactyly):

observations on a British series of thirty nine cases. Ann Hum Genet1960;24:151–164.

3. Buchanen RC. Acrocephalosyndactyly, or Apert’s syndrome. Br JPlast Surg 1968;21:406–418.

FIGURE 4: A 5-digit hand created in a single-stage opera

4. Fereshetian S, Upton J. The anatomy and management of the thumbin Apert syndrome. Clin Plast Surg 1991;18:365–380.

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5. Flatt AE. The care of congenital hand anomalies. 2nd ed. St. Louis:Quality Medical Publishing, 1994:262–270.

6. Green SM. Pathological anatomy of the hands in Apert’s syndrome.J Hand Surg 1982;7:450–453.

7. Hoover GH, Flatt AE, Weiss MW. The hand and Apert’s syndrome.J Bone Joint Surg 1970;52A:878–895.

8. Kaplan LC. Clinical assessment and multispecialty management ofApert syndrome. Clin Plast Surg 1991;18:217–225.

9. Smith RJ, Lipke RW. Treatment of congenital deformities of thehand and forearm. N Engl J Med 1979;300:402–407.

10. Zuker RM, Cleland HJ, Haswell T. Syndactyly correction of thehand in Apert syndrome. Clin Plast Surg 1991;18:357–364.

11. Upton J. Classification and pathologic anatomy of limb anomalies.

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12. Van Heest AE, House JH., Recklin WC. Two-stage reconstruction ofApert acrosyndactyly. J. Hand Surg 1997;22A:315.

13. Barot LR, Caplan HS. Early surgical intervention in Apert’s syndac-tyly. Plast Reconstr Surg 1986;77:282.

14. Coombs CJ, Mutimer K. Tissue expansion for the treatment ofcomplete syndactyly of the first web. J Hand Surg 1994;19A:968.

15. Gupta A, Kay SPJ, Scheker L, eds. The growing hand: diagnosis andmanagement of the upper extremity in children. New York: Mosby,2000:345–362.

16. Fearon JA. Treatment of the hands and feet in Apert syndrome: an

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evolution in management. Plast Reconstr Surg 2003;112:1–12;discussion 13–19.

17. Rozen WM, Phillips TJ, Ashton MW, Stella DL, Taylor GI. A newimaging modality for free flaps in breast reconstruction: CT angiog-raphy. Plast Reconstr Surg 2008;121:367–373.

18. Rozen WM, Palmer KP, Suami H, Pan WR, Ashton MW, CorlettRJ, et al. The DIEA branching pattern and its relationship toperforators: the importance of preoperative computed tomo-graphic angiography for DIEA perforator flaps. Plast Reconstr

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