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Hand Congenital Deformities (Reconstruction of Deformed Hand) Hand

What Are the Criteria of Normal Hand?hand

Hands are the chief organs for physically manipulating the environment, used for both gross motor skills (such as grasping a large object) and fine motor skills (such as picking up a small pebble). The fingertips contain some of the densest areas of nerve endings on the body, are the richest source of tactile feedback, and have the greatest positioning capability of the body; thus the sense of touch is intimately associated with hands.

Like other paired organs (eyes, ears, legs), each hand is dominantly controlled by the opposing brain hemisphere, and thus handedness, or preferred hand choice for single-handed activities such as writing with a pen, reflects a significant individual trait.
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Anatomy of the Hand:

The human hand consists of a broad palm (metacarpus) with 5 digits, attached to the forearm by a joint called the wrist (carpus). The back of the hand is formally called the dorsum of the hand.

Fingers and Digits:
The four fingers on the hand are used for the outermost performance; these four digits can be folded over the palm which allows the grasping of objects. Each finger, starting with the one closest to the thumb, has a colloquial name to distinguish it from the others: index, middle, ring and little fingers.

The thumb is located on one of the sides, parallel to the arm. The thumb can be easily rotated 90°, on a level perpendicular to the palm, unlike the other fingers which can only be rotated approximately 45°. A reliable way of identifying true hands is from the presence of opposable thumbs. (the ability of thumb to be brought opposite to the fingers, a muscle action known as opposition).

Bones:
The human hand has 27 bones:

anatomy of hand

 
Articulations and Joints:
The articulation of the human hand is more complex and delicate than that of comparable organs in any other animals. Without this extra articulation, we would not be able to operate a wide variety of tools and devices. The articulations are:
- Interphalangeal joints (PIP, DIP).
- Metacarpophalangeal joints (MP).
- Intercarpal articulations.
- Wrist Joint.

Muscles and tendons:
The movements of the human hand are accomplished by two sets of each of these tissues. They can be subdivided into two groups:
- The Extrinsic Muscle Groups: are the long flexors and extensors. They are called extrinsic because the muscle belly is located on the forearm.
- The Intrinsic Muscle Groups: are the thenar and hypothenar muscles (thenar referring to the thumb, hypothenar to the small finger), the interossei muscles (between the metacarpal bones, four dorsally and three volarly) and the lumbrical muscles.

hand-anatomy



Timing of Hand Reconstruction:

Advantages of Early reconstruction within the first 2 years of life include:
- The full potential for growth, development, and patterns of use.
- Minimize scarring.
- Early integration of the reconstructed hand.
- Avoid psychological problems.

Disadvantages of early surgery are the technical difficulty and anesthetic risk.


Development of the Hand:

Limb development takes place during the 3rd to 8th weeks of gestation. The period of hand differentiation is short, taking place during the 4th to 7th weeks.

The limb buds appear as swellings on the frontal aspect of the embryo (mesenchyme covered with a thick layer of ectoderm - termed the apical ectodermal ridge (AER). The mesoderm is organized into a posteriorly located zone of polarizing activity (ZPA) and a progress zone (PZ) more anteriorly. Vessels and then nerves subsequently grow into the limb.

Mesoderm differentiates into cartilage and muscle. The limb begins to pronate, the elbow flexes, and the hand is flexed and medially deviated. Then, ossification of the phalanges takes place, while the carpal bones ossify after birth. Ossification of the congenitally deformed hand is delayed.

Muscle formation is derived from extensor and flexor muscle mass. Tendons develop independently of their muscle bellies, only linking later in development. If the adjacent bone is not developed, the tendon attaches to the nearest adjacent structure.


What Are the Causes of Deformed Hand?

The causes of congenital anomalies can be divided conveniently into genetic, environmental, and unknown causes. Multiple problems in morphogenesis may be explained in terms of a sequence, which is a simple problem leading to a cascade of subsequent problems.

There are three types of sequences:
- Malformation sequence, in which an intrinsic malformation exists in the embryo
- Deformation sequence, in which no intrinsic defect is found in the embryo; rather, an abnormal external mechanical or structural force results in secondary distortion or deformation.
- Disruption sequence, in which the healthy embryo is subjected to tissue breakdown or injury.


Types of Hand Deformities and Treatment options:

The classification proposed by Swanson has been adopted by the American Society for Surgery of the Hand (ASSH) and the International Federation of Societies for Surgery of the Hand (IFSSH).

Type I - Failure of formation:
- Transverse arrest: Can be at any level, shoulder to phalanx. Treatment is by prosthesis.
- Longitudinal arrest:
-- Preaxial - Varying degrees of hypoplasia of the thumb or radius:
              Type I - Short radius, no treatment
              Type II - Hypoplastic radius, no treatment
              Type III - Partial absence with fibrous band; requires centralization
              Type IV - Total absence; soft tissue release and centralization

Syndromes associated with preaxial deficiency include the following:
a- Holt-Oram syndrome = Heart defects
b- Thrombocytopenia-absent radius (TAR) syndrome
c- VACTERL = Vertebral abnormalities, anal atresia, cardiac abnormalities, tracheoesophageal fistula, esophageal atresia, renal defects, radial dysplasia, lower limb abnormalities
d- Fanconi anemia = Aplastic anemia at 6 years of age.

-- Central - This deficiency manifests as the absence of digits or metacarpals within the central portion of the hand. Divided into typical and atypical types of cleft hand
-- Postaxial - Varying degrees of ulnar hypoplasia to hypothenar hypoplasia: Cole and Manske developed a classification based on the development of the hand, as follows:
Type A - Normal first web space and normal thumb
Type B - Mild deficiency of first web space and mild thumb hypoplasia with intact opposition and extrinsic tendon function
Type C - Moderate-to-severe deficiency of first web space and similar thumb hypoplasia with loss of opposition and dysfunction of extrinsic tendons
Type D - Absence of thumb
Physiotherapy and splinting are used to improve passive range of motion.

- Intercalated longitudinal arrest - Various types of phocomelia:
Type 1 - those in which the hand attaches to the shoulder (forearm and arm deficient)
Type 2 - those in which the forearm attaches to the shoulder (arm deficient)
Type 3 - those in which the hand attaches to the arm (forearm deficient)
Treatment is by prosthesis. However, deviation of the hand may require centralization.

Type II - Failure of differentiation:
- Soft tissue: Syndactyly, trigger thumb, Poland syndrome, camptodactyly:

a- Syndactyly: is a condition where two or more digits are fused together. In early human fetal development, webbing (syndactyly) of the toes and fingers is normal. At about 16 weeks of gestation, the hands and feet have selective cells that die, called selective cell death or apoptosis, causing separation of the digits, and the webbing disappears. In some fetuses, this process does not occur completely between all fingers or toes and some residual webbing remains.

Syndactyly Can be classified into:

A- Syndactyly can be simple or complex:
i- In simple Syndactyly, adjacent fingers or toes are joined by soft tissue.
ii- In complex Syndactyly, the bones of adjacent digits are fused.

B- Syndactyly can be complete or incomplete.
i- In complete Syndactyly, the skin is joined all the way to the tip of the finger
ii- In incomplete Syndactyly, the skin is only joined part of the distance to the fingertip.
iii- Complex Syndactyly occurs as part of a syndrome (such as Apert's syndrome) and typically involves more digits and with complex Syndactyly.
iv- Fenestrated Syndactyly means the skin is joined for most of the digit but in a proximal area there is gap in the Syndactyly with normal skin. This type of Syndactyly is found in amniotic band syndrome.

Syndactyly


Syndromes related to Syndactyly:
- Apert syndrome
- Poland syndrome

Syndromes related to Syndactyly



Treatment: The aims of surgery are separation of the digits and avoidance of scars. Many techniques exist with the following principles: Either a dorsal or volar flap, Zigzag incisions to lessen the contracture, Operating on one side of the digit at a time, Correction of skeletal abnormality, Creation of a normal nail, Full-thickness skin graft (FTSG) for defects to avoid contracture.

b- Symphalangism: the failure of segmentation that results in stiff fingers. There are three types:
i- true symphalangism, the digits are of normal length.
ii- brachysymphalangism, the digits are short and stiff.
iii- Syndromic symphalangism, particularly Poland and Apert syndromes.

c- "Thumb in palm" deformities: lack of extension (clasped thumb) may be due to trigger thumb, congenital absence of the thumb extensors, camptodactyly, or arthrogryposis.

d- Camptodactyly: may be in one of three forms:
Type I - Congenital, isolated, unilateral.
Type II - appear at 7-11 years, affects females much more often than males (A progressive flexion deformity that does not resolve spontaneously).
Type III - Severe, congenital, effecting multiple digits bilaterally. This type is associated with a variety of generalized conditions, as follows:
i- Arthrogryposis
ii- Craniofacial disorders: Orofacial digital syndrome, Freeman-Sheldon syndrome, Oculodentodigital dysplasia, Oculopalatodigital syndrome
iii- Chromosomal disorders: Trisomy 13-15
iv- Short stature: Camptomelic dysplasia, Mucopolysaccharidosis, Aaskorg-Scott syndrome
v- Others: Turner-Kieser syndrome, Zellweger syndrome

Treatment: Primarily, conservative by splinting and stretching. Surgically, the flexor tendon may need to be released or may require tendon transfer.

e- Clinodactyly: a deviation of a finger as a result of an abnormally shaped middle phalanx.
Three treatment options are available:
- Opening wedge osteotomy with nonvascularized bone graft (NVBG)
- Closing wedge osteotomy
- Reversed wedge osteotomy.

f- Delta phalanx

- Skeletal: Various synostoses (Radioulnar synostosis, Humeroradial synostosis) and carpal coalitions

- Tumorous conditions: Include all vascular and neurologic malformations

Type III - Duplication: whole limb, mirror hand, polydactyly:

a- Polydactyly: or polydactylism, also known as hyperdactyly, is a congenital physical anomaly in humans having supernumerary fingers or toes. When each hand or foot has six digits, it is sometimes called sexdactyly, hexadactyly, or hexadactylism. It could be false finger (soft tissue) or true finger. It could be complex where there is a fusion between extra finger and the adjacent normal one.

Treatment: Surgical correction of complex duplications is timed according to most other anomalies, as a compromise between delay on technical grounds and early operation for functional reasons. For example, treatment of thumb polydactyly must take into consideration that development of pinch occurs in the first year of life.

Polydactyly



b- Mirror hand (ulnar dimelia): The classic presentation of ulnar dimelia is duplication of the ulna, with no radius, and 7 or 8 fingers with no thumbs. It may occur in association with fibular dimelia and absence of the tibia.

Treatment: the accessory preaxial middle and small fingers are resected, and the accessory ring digit is used as a thumb by a modified pollicization procedure.

Type IV - Overgrowth: hemihypertrophy and macrodactyly

Macrodactyly: an enlargement may occur due to vascular malformations, enchondromatoses, osteoid osteoma, fibrous dysplasia, lipomatoses. Macrodactyly can be presented as:
i- Macrodactyly associated with nerve-orientated lipofibromatosis:
ii- Macrodactyly associated with neurofibromatosis: Gigantism of the upper or lower limbs + 6 café au lait spots greater than 2.5 cm + Multiple neurofibromas along peripheral nerves + skin masses termed molluscum fibrosum
iii- Macrodactyly associated with hyperostosis:
iv- Macrodactyly /gigantism and hemihypertrophy: This is rare.

Treatment options are as follows:
- Epiphysiodesis or total resection
- Staged debulking
- Partial amputation or ray amputation

Type V - Undergrowth: radial hypoplasia, brachysyndactyly, or brachydactyly
Treatment options are as follows:
1- (pollicization of the index) The index finger is pronated in order to oppose the other digits. The metacarpal head is rotated into flexion in order to prevent hyperextension.
2- Vascularized toe-to-thumb transfers

Type VI - Constriction band syndromes may be presented as:
a- Simple constriction ring
b- Constriction ring with distal deformity (lymphedema)
c- Constriction ring with distal soft tissue fusion
d- Intrauterine amputations

Treatment options are as follows:
1- Excision of the groove with multiple Z-plasty or W-plasty
2- Excision of functionless nubbins of tissue
3- Pollicization for patients in whom the thumb is absent

Type VII - Generalized anomalies and syndromes:

a- Arthrogryposis multiplex congenital: This is a syndrome of unknown etiology that presents at birth and manifests with multiple joint contractures.

b- Vascular malformations.


How Much Do Hand Reconstruction Typically Cost?

Although price is a factor when deciding on surgery, the most important factor is that you feel you are getting a good quality, natural-looking result from the procedure.

Reconstructive Procedures costs are variable according to the deformity and the used technique. Usually the cost is ranging from 1000 to 3500 U.S. dollars. When dealing with costs, you also need to take into consideration the fact that the procedure may need to be repeated. Most medical insurance providers cover the procedure.


Summary of Hand Reconstruction Procedures:

The time of the procedure:
from half to four hours.
Type of anesthesia:
General anesthesia.
Location:
hospital with or without postoperative hospital stay.
Expected Side effects:
Temporary swelling and pain.
Recovery time:
return to normal life after 2 weeks and the final outcome within 3 weeks.
Cost:
according to procedure ranging from 1000 to 3500 U.S. dollars.
Results: Permanent results.