Congenital hand anomalies :management

Dr.Khaldon abdulrhamn alaghbari ZoReKh

Principles of surgical management

• When considering surgery the following question must always be asked: “Is reconstructive surgery likely to improve function and/or appearance with minimum risk?” .

• Hand function itself depends on the ability to use a mobile thumb ray against other parts of the hand, ulnar digits for grasp and radial digits for precision pinch.

• The hands are expressive and communicative. Deformity of a part is often more obvious than its absence and its correction may clearly result in an improved appearance for all eyes.However, for many severe deformities it is simply not possible to create a normal-looking hand. Surgery to improve appearance in these cases must be undertaken realistically.

Timing

• Surgical results may be compromised by the technical difficulties of procedures on small structures in early life.

• It is a reasonable approach to allow the child to adapt to the new hand as early as possible. However, cortical plasticity allows significant adaptation at older ages and, if the surgeon is doubtful that the surgery

is indicated or the parents demand delay, it is preferable to wait.

• Child decision Sholud be considered when functional improvement is unlikely and the operation will be done only for cosmetic resons.

• Hand surgery for congenital anomyles will be delayed to treat more life-threatening conditions.

Disorders to formation(transverse and longitudinal arrest)

• Most transverse deformities will not require surgery.They may benefit from good quality prostheses for which they should bereferred early.

• Occasionally, after full assessment by the rehabilitation team, the plastic surgeon is asked to remove tissue or make surgical adjustment to the stump to make prosthesis fitting easier. Sometimes, the parents themselves request to have the apparently useless tissue on the terminal part of the limb removed.

Congenital transverse arrest

Congenital transverse arrest

At level of forarm :Any treatment is usually restricted to functional and

cosmetic prosthetics.The Krukenberg procedurer has been used in this

condition,particularly when bilateral, to provide a pincer grip between the two

forearm bones.

At Carpal level: prosthetics are the mainstay of

management. From a surgical point of view, where

there is sufficient carpus to be able to provide some

intercarpal movement, a double free toe transfer

may provide the ability to grip broader objects but

is unlikely to be able provide a fine pincer movement

Metacarpal level (symbrachydactyly)

• Where all metacarpals are present, option is to deepen the first web to create some form of crude

pinch(phalangization). However, the pinch is a crude lateral pinch, opposition is not possible and

grasp is limited due to the poor span.

Other options include

1. Free phalangealtransfer (FPT)±distraction augmentation manoplasty (DAM).

2. Single or double free vascularized toe transfer.

• FPT This procedure is only appropriate where there is a sufficiently large soft tissue envelope.

• A pocket created by dividing the longitudinal fibrous bands that extend to the tip of the soft

tissue from the flexor-extensor tendon confluence over the metacarpal head. It is only then

that the true extent of the soft tissue envelope becomes apparent.

• Hole phalanx must be harvested.

• phalanx is sutured onto the flexor-extensor hood which covers the metacarpal head.with k-

wire fixation.

• Distraction manoplasty starts at 8 years with or without bone graft.

• Free toe transfer

Congenital longitudinal arrest

• Longitudinal deficiencies usually benefit from surgery both for

functional and aesthetic reasons.

• Surgery is ideally completed by school age,within period of rapid growth

• Early surgery may prevent progression of deformity and allow early

skeletal realignment.

• Any surgery has the potential of impairing growth.

• Surgical tissue augmentation,tendon or muscle transfer, will not improve

over all strength but will redistribute it to allow more normal function.

Phocomelia• The main stay of treatment is with therapist ,orthotist and

prosthetist,surgeon may be asked to help fitting prothesis to patient.

Radial hypoplasia or aplasia

• Treatment may range from simple tendon transfers to complex soft tissue and bony distractions.

• Soft tissue distraction makes wrist centralizati or stabilization technically easier.

• Stabilization retains motion at the“wrist”but is unstable.

• Centralization is stable but relatively immobile.

• Tendon transfers are always required in all but a small group of type I radial club hand.

• Wrist stabilization should precede thumb reconstruction.

• Treatment begin to learn parents of stretching exercise then splenting at night when child is

older to mentain morning gain.

• When sutable length gained A distraction fixator is applied between the ulna and the

second metacarpal on the radial side to begin the process of soft tissue distraction.

• Stablization is then proceed by either centralization or radialization,and both need tendon

transfer of ECRL and ECRB to ECU.

Central ray deficiency

• The strongest indication for treatment in cleft hands is the presence of

transverse lying bones. Care needs to be taken here to avoid

destabilization of involved joints.

• Try to avoid longitudinal scar in web spaces to prevent V shape deformity.

• The Snow–Littler procedure involves transposition of a palmar based flap

from the cleft to supplement the first web, release of the first dorsal

interosseous and adductor pollicis and simultaneous osteotomy and transfer

of the index finger into the position of the first ray. This may compromise

the vascularity of the index finger and the long palmar flap moves poorly

and may necrose distally. Modifications of this technique have been

developed, by Miura and Komada technique involves a dorsally based flap

which therefore moves with greater ease and is technically easier.

Thumb hypoplasia management

• In type I : patient not always in need for surgical intervention but some time deepning of

1st web is needed.

• In type II : five specific problems must be addressed individually:

(1) Narrowed first web space Zplasty or Jumping man flap.

(2) Instability of the MP joint Tightening of the existing ligament and capsule,

arthrodesis or opposition .

(3) Poor palmar abduction (opposition)Transfer of abductor digiti minimi muscle ,or

FDS tendon of ring finger after looping around FCU tendon.

(4) Lack of IP joint flexionDorsal capsulotomy and tendon recentralization will

improve the minimal deformities.

(5) Abduction posturing of the thumb (pollex abductus) Look for FPL deformetis.

Z-plasty

Ring FDS after looping around FCU tendon

Arthrodesis

• Type III :Management vary from as type II to pollicization.

• Type IV and Type V : Pollicization is the best choose for management.

Disorders of differentiation and duplication

Syndactyly

Least number of operation to decrease complication,Relase of skin and subcutaneous

tissue with neurovascular bundels protection,for sefaty if both sides of finger are

included operation has to be done at one side every time.

The main problems to be addressed are :

• Creation of a web palf lasrod

• Treating the lateral soft tissue defects giz-GSTF+ spalf gaz

• Separation of the fingertips palf eplup tnecajda ro, noitarapes elpmis rehtiE

• Bone separation as necessary

• In complicated syndactylies, ligament reconstructions, osteotomies, chondrodesis

and arthrodeses can be necessary.

Poland syndrome

Symbrachydactyly and syndactyly can also present with other Anomiles as absent

pectorales major ,breast ,..etc

Pulp flap for nail reconstraction

Acrocephalosyndactyly syndrome is characterized by craniosynostosis combined with

acrosyndactyly (distal part of the fingers fused) and symphalangism (congenital

ankylosis of the proximal phalangeal joints) of the index, long and ring fingers of

both hands and a radial clinodactyly of the thumb in a symmetrical way.

Treattment will include separation of fingers,Deepinig of 1st web space treatment of

clinodactaly of thumb if present by wedge Osteotomy, every procedure had it own time to be

done Starting from 3 mothes till 12 years .

Apert syndrome

Synostosis

Treatment of symphalangism is mostly conservative. In well-segmented joints only,

exploration and release of the collateral ligaments and dorsal capsule may improve

function, Arthrodesis in a more functional position of the involved joints at skeletal

maturity will improve function and grip.

Polydactyly

Treatment policy will include :

• Make one thumb from two.

• Do not just excise the most hypoplastic thumb but preserve

tendons, ligaments and skin to align, balance and augment the

residual thumb.

• At the first operation as much correction as possible is performed

on both soft tissues and bones.

• Align articular surfaces by transverse and longitudinal osteotomies.

• Perform ligament reconstructions or releases.

• Balance tendon insertions.

• Adjust skin cover as accurately as possible.

• Postoperative dressings should be meticulously applied and resistant

to removal by the child.

• Same rules to central or ulnar polydactaly

Camptodactyly

Contracture in PIP joint Treatment mainly by splient from 3 to 12 months , if

no response gained, Operations range from skin plasties, arthrolysis,

tenotomies, tendon transfers, and osteotomies to arthrodesis.

Clinodactyly

The angle of deviation defining clinodactyly varies among authors from >8° to

>15°.Clinodactyly is a symptom and not a disease. It may be isolated or may

be associated with other congenital malformations.

Treatment for clinodactyly can be divided into the following procedures:

• Opening wedge osteotomy with or without bone graft.

• Closing wedge osteotomy.

• Reversed wedge osteotomy.

• Release of soft tissues on the concave side.

• Tightening of soft tissues on the convex side.

Disorders of overgrowth and undergrowth

Macrodactyly

Over growth of limbe which is a rare condation ,Surgical correction is complicated and

difficult because of the extensive involvement, treatment consists of radical

excision of the soft tissues around the digital nerves,Excision of the entire involved

nerve has also been performed. When the bones and joints have undergone

secondary hyperplasia, shortening with or without excision of one growth plate or

fusion of the joint or wedging may have to be done. In the worst case, sacrifice

of digit or some digital segment might be necessary.

Brachydactyly

Hypoplasia of limb which often with other congenital deformities which make it

difficult to treatment , treatment may vary from conservative to osteotomy and bone

lengthening and treating other deformites.

Constriction band syndrome

If the constriction is very mild, it is not necessary to release the constriction. If

the constriction is serious and is complicated with edema distally, it should be

released as soon as possible. Urgent release may be performed under local

anaesthesia with a direct perpendicular incision over the constriction site. This

would be left dressed openly for 2 days to allow the distal edema to subside

before properly fashioning a local Zplasty to close the defect ,web space deepning

may also considered.

Thank you