Dr. Ammar Talib Al- Yassiri College of Medicine / Baghdad University

CLASSIFICATION:  failure of formation of parts;  failure of differentiation of parts;  duplication;  overgrowth;  undergrowth;  constriction bands;  generalized skeletal abnormalities.

 FAILURE OF FORMATION ◦ Transverse arrest:  The most common levels of absence are at the proximal forearm and midcarpus, then at the metacarpals and humerus.  Associated anomalies are unusual.  Treatment:  Proximal forearm:  Prosthetic fittings in young children  myoelectric prostheses in older children and adolescents  Transverse arrest of fingers  microvascular transfer of a toe  non-vascularized transfer of a toe phalanx

◦ Longitudinal arrest  radial (pre-axial),  ulnar (post-axial),  central (cleft hand) or  intersegmental (intercalated) structures

 rare condition (incidence 1: to 1: live births)  may involve any (or all) of the structures from the elbow to the thumb, it usually occurs as an isolated abnormality  occasionally associated with other skeletal, cardiac, haematological, renal or craniofacial anomalies  The infant is born with the wrist in marked radial deviation ‘radial club hand’  half the patients are affected bilaterally  There is absence of the whole or part of the radius; often the thumb, scaphoid and trapezium fail to develop normally.  Treatment: ◦ mild→gentle stretching and splintage, ◦ More serious cases →distraction prior to a tension-free soft-tissue correction ◦ older technique of ‘centralizing’ the carpus over the remaining forearm structures.

 less common than radial dysplasia  Most cases are sporadic, but the condition may be part of a larger syndrome,  The new-born infant presents with ulnar deviation of the wrist (or both wrists),  Due to partial or complete absence of the ulna;  some of the carpal bones may be absent  ulnar rays of the hand may be missing.  With growth the radius elongates and bowed;  ultimately the radial head may dislocate.  Treatment ◦ stretching and splinting ◦ excision of any tethering ulnar anlage and osteotomy of the radius ◦ the radial head can be excised ◦ (The Straub procedure).

 The true cleft hand presents with V-shaped cleft in the centre of the hand  may be associated with the absence of one or more digits, transverse bones, syndactyly of digits bordering the cleft, and a tight first web space.  Surgery is complex, having to deal with closure of the cleft, reconstruction of the first web space

 Very rarely an intercalary segment in the upper limb fails to develop  the forearm or hand may be attached directly to the trunk,  or the hand is attached to the humerus.  also known as phocomelia

Syndactyly:  Conjoined digits is the commonest congenital malformation  The anomaly may be ◦ simple (soft tissue only) or complex (skin and bone), ◦ complete (affecting the entire web) or incomplete (only part of the web).  Treatment: ◦ Mild incomplete, of central digits need no treatment ◦ complete syndactyly involves separation and skin grafting.

DUPLICATION Polydactyly (‘extra digits’) ◦ radial (pre-axial), ◦ the ulnar (post-axial) ◦ the central part of the hand. ◦ Duplication of the little finger:  is one of the most common  often inherited  If a phalanx or entire digitis duplicated, removal and soft-tissue reconstruction ◦ Duplications of the thumb or central digits:  extremely rare  require complex reconstructive surgery of the digit, its tendons and the overlying skin.

OVERGROWTH  DDX: other forms of enlarged digits ◦ Neurofibromatosis ◦ multiple enchondromatosis ◦ vascular malformations  TREATMENT: ◦ Surgical correction: extremely difficult and unrewarding. ◦ Amputation may be the best option. UNDERGROWTH  Undergrowth (brachydactyly) is common  May be part of a wider syndrome (e.g. Turner’s syndrome).  It can affect a single bone, a digit or an entire limb.

CONSTRICTION RING SYNDROME  The condition presents as localized ‘strangulation’, most commonly of the ring finger;  the distal part of the finger may be painful, swollen and cyanotic, or sometimes threatened with amputation  Treatment consists of excision of the constricting band and soft-tissue reconstruction using multiple Z-plasties

 congenital or post-traumatic,  the lower radius curves forwards (ventrally), carrying with it the carpus and hand but leaving the lower ulna sticking out as a lump on the back of the wrist.  The congenital ◦ isolated entity ◦ part of a generalized dysplasia;  although the abnormality is Present at birth but is rarely seen before the age of 10 years.  Function is usually excellent.  Treatment: If deformity is severe, the lower end of the ulna may be shortened; this is sometimes combined with osteotomy of the radius

 a form of ischaemic necrosis, probably due to chronic stress or injury  Pathology:the pathological changes proceed in four stages  Clinical features: ◦ young adult, ◦ Ache and stiffness. ◦ Tenderness ◦ grip strength is diminished. ◦ movements are limited and painful  Imaging: ◦ Radioscintigraphy may reveal increased activity ◦ X-rays ◦ MRI  Treatment ◦ NON-OPERATIVE TREATMENT  splintage of the wrist for 6–12 weeks ◦ OPERATIVE TREATMENT  pedicled bone graft  shortening the radius  replacement by a silicone prosthesis  intercarpal fusion or excision of the proximal row of the carpus  radio-carpal arthrodesis  Wrist replacement

 Pathology: reactive thickening of the sheath around the extensor pollicis brevis and abductor pollicis longus tendons  Clinical features ◦ woman aged 40–50 ◦ Pain on the radial side of the wrist ◦ history of unaccustomed activity ◦ Swelling ◦ Tenderness ◦ Finkelstein’s test ◦ (hitch-hiker’s sign)  The differential diagnosis: ◦ arthritis at the base of the thumb ◦ scaphoid non-union ◦ intersection syndrome  Treatment

 GANGLION CYSTS ◦ the most common swelling in the wrist ◦ Pathology ◦ Clinical features  young adult,  painless lump  occasionally there is slight ache and weakness ◦ Treatment  EXTENSOR TENOSYNOVITIS ◦ Localized swelling of a tendon sheath on the dorsum of the wrist  CARPO-METACARPAL BOSS ◦ firm round swelling over the back of the second and third carpo-metacarpal joint ◦ may be caused by some instability at the joint.  COMPOUND PALMAR GANGLION’ ◦ Chronic inflammation distends the common sheath of the flexor tendons both above and below the flexor retinaculum.

 Carpal tunnel is a fibro-osseous tunnel  there is barely room for all the tendons and the median nerve  compression and ischaemia of the nerve. ◦ (1)the soft tissues increase in bulk (pregnancy,menopause, myxoedema,rheumatoid arthritis,trauma or drugs) or ◦ (2) there is a local obstruction (e.g. a ganglion or osteophytic spur).  Clinical features: ◦ Pain and paraesthesia ◦ Night after night the patient is woken with burning pain, tingling and numbness. ◦ clumsiness and weakness ◦ more common in women (40–50 y) ; in younger patients it is not uncommon to find related factors such as pregnancy, rheumatoid disease, chronic renal failure or gout. ◦ Tinel’s sign ◦ Phalen’s test. ◦ In late cases there is wasting of the thenar muscles, weakness of thumb abduction and sensory dulling in the median nerve territory. ◦ Electrodiagnostic tests are reserved for those with atypical symptoms

 Differential diagnosis: Radicular symptoms of cervical spondylosis  Treatment ◦ Light splints ◦ Steroid injection into the carpal canal, provides temporary relief ◦ Open surgical division of the transverse carpal ligament usually provides a quick and simple cure.

 Cuts and burns of the palmar skin are liable to heal with contracture  Surgical incisions should never cross skin creases perpendicularly they should lie more or less parallel or oblique to them, or in the mid-axial line of the fingers  A useful alternative zig-zag incision  Treatment: ◦ excision of the scar, Z-plasty of the remaining skin, skin grafts, a pedicled flap and occasionally a free flap.

 Nodular hypertrophy and contracture of the superficial palmar fascia (palmar aponeurosis).  autosomal dominant trait  most common in people of European (especially Anglo-Saxon) descent  more common in males than females  prevalence increases with age  There is high incidence in epileptics receiving phenytoin therapy;  associations with diabetes, smoking, alcoholic cirrhosis, AIDS and pulmonary tuberculosis

 The essential problem in Dupuytren’s disease is proliferation of myofibroblasts  initial proliferative phase  fibrous tissue within the palmar fascia and fascial bands within the fingers contracts  flexion deformities of the MCP and PIP joints  Fibrous attachments to the skin lead to puckering  digital nerve is displaced or enveloped, but not invaded, by fibrous tissue  Occasionally the plantar aponeurosis also is affected

 middle-aged man –  nodular thickening in the palm.  Gradually this extends distally to involve the ring or little finger.  Pain  Often both hands are involved, one more than the other.  The palm is puckered, nodular and thick.  flexion deformities at the MCP and PIP joints.  (Garrod’s pads).  (Ledderhose’s disease).  (Peyronie’s disease).

 (1) skin contracture (where the previous laceration is usually obvious),  (2) tendon contracture (in which the finger deformity changes with wrist position) and  (3) PIP joint contracture (in which there may  be a history of clinodactyly or joint injury).

 Indication ◦ nuisance or rapidly progressing ◦ PIP joint contractures can become irreversible  The aim is reasonable, not complete, correction  recurrence or extension is common  Only the thickened part of the fascia is excised (complete fasciectomy is usually unnecessary)  Skin closure may be facilitated by multiple Z- plasties

 MALLET FINGER ◦ injury to the extensor tendon of the terminal phalanx ◦ Due to direct trauma but more often when the finger tip is forcibly bent during active extension ◦ terminal joint is held flexed and the patient cannot straighten it, but passive movement is normal. ◦ X-rays show or exclude a fracture ◦ Treatment:

 BOUTONNIÈRE DEFORMITY ◦ flexion deformity of the PIP joint and extension of the DIP joint ◦ interruption or stretching of the central slip of the extensor tendon ◦ The usual causes are direct trauma or rheumatoid disease. ◦ treatment:

 SWAN-NECK DEFORMITY ◦ reverse of the boutonnière deformity; the PIP joint is hyperextended and the DIP joint flexed. ◦ imbalance of extensor versus flexor action at the PIP joint and laxity of the palmar plate. ◦ Thus it may occur: (1) if the PIP extensors overact (e.g. due to intrinsic muscle spasm or contracture, after mallet finger, or following volar subluxation of the MCP joint); (2) if the PIP flexors are inadequate (inhibition or division of the flexor superficialis); or (3) if the palmar plate of the PIP joint fails (in rheumatoid arthritis, lax- jointed individuals or trauma). ◦ Treatment depends on the cause and whether or not the deformity has become fixed.

 TRIGGER FINGER (DIGITAL TENOVAGINOSIS) ◦ A flexor tendon may become trapped by thickening at the entrance to its sheath; on forced extension it passes the constriction with a snap (‘triggering’). ◦ common in patients with diabetes. ◦ rheumatoid disease may ◦ Occupational factors are unlikely to be causative ◦ Clinical features  Any digit may be affected, but the thumb, ring and middle fingers most commonly  click as the finger is flexed  tender nodule ◦ Treatment:  injection of corticosteroid carefully placed at the mouth of the tendon sheath  Refractory cases need operation

 most common sites:(MCP) joints, the wrists and distal (RA) joints  PATHOLOGY: ◦ Synovitis of the joints and tendon sheaths ◦ the disease joints become eroded ◦ attenuation of the ligaments and tendons ◦ instability and progressive deformity ◦ tendon rupture  Clinical features: ◦ Stiffness and swelling of the fingers ◦ carpal tunnel compression ◦ swelling of the MCP and PIP joints, giving the fingers a spindle shape; ◦ Bilateral symmetrically. ◦ The joints are tender and crepitus may be felt on moving the tendons. ◦ grip strength are diminished. ◦ Early deformity : slight radial deviation of the wrist and ulnar deviation of the fingers, correctable swanneck deformities of some fingers, an isolated boutonnière or the sudden appearance of a drop-finger or mallet thumb (from extensor tendon rupture). ◦ Late deformity: the carpus settles into radial tilt and volar subluxation; there is marked ulnar drift of the fingers and volar dislocation of the MCP joints, often associated with multiple swan-neck and boutonnière deformities.

 X-ray:  TREATMENT: ◦ EARLY STAGE DISEASE: ◦ Established disease ◦ Late disease

 80% of people over the age of 65 have radiological signs of osteoarthritis in one or more joints of the hand; fortunately, most of them are asymptomatic.  DISTAL INTERPHALANGEAL JOINTS: ◦ very common in postmenopausal women. ◦ pain in one or two fingers; ◦ swollen and tender, ◦ On examination: bony thickening around the joints (Heberden’s nodes) and some restriction of movement. ◦ Treatment: symptomatic, cortisone injection will give temporary relief. Joint fusion is a good solution.

 PROXIMAL INTERPHALANGEAL JOINTS: ◦ (Bouchard’s nodes). ◦ Strongly associated with osteoarthritis elsewhere in the body (polyarticular OA). ◦ The joints are swollen and tend to deviate ulnarwards due to mechanical pressure in daily activities. ◦ Treatment : is usually non-operative. If the joint is very painful or unstable then surgery is considered. Fusion restores reliable, pain-free pinch in the index and middle finger PIP joints; fusion of the ring and little fingers compromises grip and so joint replacement is usually preferable.

 Carpo-metacarpal joint of the thumb: ◦ Osteoarthritis of the trapezio-metacarpal joint is common in postmenopausal women. ◦ Heberden’s nodes of the finger joints, ◦ bilateral and part of a generalized osteoarthritis.

 Infection of the hand is frequently limited to one of several well-defined compartments: ◦ (1)under the nail-fold (paronychia); ◦ (2) the pulp space (felon) ◦ (3) the deep fascial spaces; ◦ (4) tendon sheaths; and joints.  Usually the cause is a staphylococcus which has been implanted during fairly trivial injury.

 Pathology:  infection an acute inflammatory reaction (oedema, suppuration) increased tissue tension pressures may rise to levels where the local blood supply is threatened tissue necrosis.  In neglected cases infection can spread from one compartment to another  lymphatic and haematogenous spread;

 Clinical features: ◦ history of trauma (a superficial abrasion, laceration or penetrating wound), ◦ painful and swollen. ◦ throbbing ◦ the patient feels ill and feverish. ◦ finger or hand is red and swollen, ◦ exquisitely tender over the site of tension. ◦ With deep infections active flexion is not possible. ◦ lymphangitis and swollen glands, ◦ signs of septicaemia.

 X-ray examination: ◦ foreign body but is ◦ features of osteomyelitis ◦ septic arthritis, ◦ bone necrosis  IX: If pus becomes available, this should be sent for bacteriological examination.  Differential diagnosis: ◦ (1)an insect bite or sting (which can closely mimic a subcutaneous infection), ◦ (2)a thorn prick (which, itself, can become secondarily infected), ◦ (3) acute tendon rupture (which may resemble a septic tenosynovitis) and ◦ (4)acute gout (which is easily mistaken for septic arthritis).

 Principles of treatment: ◦ Antibiotics ◦ Rest, splintage and elevation ◦ Drainage ◦ Rehabilitation

 Apley’s System of Orthopaedics and Fractures  Review of Orthopedics, Miller  Campbell’s operative orthopaedics