Title MS 3_3.4_ PEDIATRIC LIMB DEFICIENCIES.pdf ✅

PT10121 | MEDICAL & SURGICAL FOUNDATIONS 3 LEC 3.4 PEDIATRIC LIMB DEFICIENCIES 3PTA | Third Shift, First Semester A.Y. 2023-2024 BACKGROUND ON BONE GROWTH AND DEVELOPMENT Bone formation or ossification occurs in 2 different ways ● Bones can be formed from chondrocytes or directly from osteoblast ● Endochondral ossification ○ Mesenchymal cells underdog chondrogenesis to form cartilage that matures to become bone ○ Exile and appendicular skeleton ● Intramembranous ossification ○ Osteoblast are formed by direct differentiation of mesenchymal cells into bone ○ Examples flat bones (skull) and clavicle Centers of ossification ● Primary Centers: Chondrocytes of long bone form to primary centers of growth from which bone lengthens ○ Found at the center of the bone ○ Limb development occurs between 4 to 8 weeks after fertilization thus most limb defects occur during 4 - 6 weeks ○ Found in the mid shaft of long bones ● Secondary Centers: appear in chondroepiphysis and appear postnatally ○ Direct formation of bone throughout growth ○ Epiphyseal plates, at the end of bones ○ If a child has trauma after birth it can affect the length of the bone ○ Can cause the shortening of the limb ○ Ossification centers appear at birth ■ Distal femur ■ Proximal tibia ■ Calcaneus ■ Talus BACKGROUND ANATOMY & PHYSIOLOGY Long bones have the following parts: ● Epiphysis - secondary ossification center (has different times of growth or closure) ○ Physis - growth plate at end of bones (between the epiphysis and metaphysis) ● Metaphysis ● Diaphysis ● Perichondrial ring - contributes to appositional growth ● Important growth and developmental milestones ● Ages of onset of secondary epiphyseal and epiphyseal ossification of major bones of the upper (A) and lower extremities (B) SKELETAL GROWTH CONSIDERATIONS ● Normally arm span is almost equal to standing height ● Head is disproportionately large at birth; with a ratio of head height to total body height 1:4 ; at skeletal maturity it changes to 1:7.5 ○ Arm span = Height ● Lower extremity accounts for 15% of length at birth and 30% at skeletal maturity ● Rate of height and growth increase is not constant and varies with growth spurts ● By 5 years old, birth height usually doubles and the child is approximately 60% of adult height. The child is approximately 80% of final height at 9 years old. During puberty, the standing height increases by approximately 1 cm/month LIMB DEFICIENCY DEFINITION ● A limb efficiency is when a child is missing part of or all of a leg, arm, hand or foot. INCIDENCE AND ETIOLOGY Congenital Amputation ● Acquired = not a limb deficiency ● Occurs inside the womb while developing ● Absence of part or all of an extremity at birth ● More approximately referred to as congenital skeletal deficiency or limb deficiency CONGENITAL DEFICIENCIES INCIDENCE ● 5 to 9.7 per 10,000 live births ● 3:1 upper to lower extremity (For UE 1.58/10,000 and for LE 83/10,000 ○ Most common congenital amputation in the lower extremity is fibular longitudinal deficiency or fibular hemimelia ○ Most common upper extremity amputation terminal transradial limb deficiency left ● 6% of all types of birth defects are limb deficiencies ETIOLOGY ● The first trimester is crucial for the genesis of limb production ○ The results of failure of formation of part or all of the limb bud ○ Development of limb happens during the 1st trimester, if anything happens to the mother it may cause the failure of formation of the limbs ○ Mesodermal formation of the limbs occurs at 26 days gestation and continues with differentiation until 8 weeks gestation ○ Risk factors: ■ Maternal diabetes ■ All maternal ingestions during 1st trimester must be documented ■ Uterine abnormality CONGENITAL LIMB DEFICIENCY Caused by ● Vascular disruption (eg amniotic band syndrome) ● Vascular malformations (eg Poland syndrome) ● Genetic factors (spontaneous point mutation) ● Most common cause is unknown Amniotic band syndrome is a rare condition caused by strands of the amniotic sac that separate and entangle digits, limbs, or other parts of the fetus ● In the placenta, there is band which bring the 1 A.D. | D.B. | M.G. | A.M. | C.Z. | F.M.

LONGITUDINAL LIMB ● Involve specific maldevelopments (eg, complete or partial absence of the radius, fibula, or tibia). Radial ray deficiency is the most common upper-limb deficiency, and hypoplasia of the fibula is the most common lower-limb deficiency ● For longitudinal deficiency, the residual limb is named by the bones that are affected ● E.g. radial ray deficiency, the radius is absent, as in this patient. The hand is angulated and deformed. LONGITUDINAL LIMB CLASSIFICATION LONGITUDINAL NAMING CONGENITAL LIMB DEFICIENCY NAMING CONGENITAL LIMB DEFICIENCY ISO/IPSO FRANTZ-O'RAHILLY CLASSIC LONGITUDINAL RADIUS DEFICIENCY INTERCALARY RADIAL DEFICIENCY RADIAL HEMIMELIA/R ADIAL CLUB HAND TERMINAL TRANSVERSE HUMERUS DEFICIENCY TERMINAL HORIZONTAL HUMERUS DEFICIENCY ABOVE ELBOW AMPUTATION ESSENTIALS OF ASSESSMENT History ● includes obstetrical and genetic history ● maternal exposures/ingestions ● syndromic features, an expanded history regarding feedings difficulties, respiratory distress, bowel dysfunction, and cardiac abnormalities are indicated. 1. cause of limb loss 2. medical problems not related to limb loss (vision, hearing, learning disability) 3. family support system, living condition or situation 4. hobbies or recreations 5. patient’s and family’s goals for rehabilitation Physical Examination ● A complete initial examination identifies other anomalies ● Assessment includes growth and development; pectoral structures; residual limb length; HEENT exam; cranial nerves; heart, spine, skin, and anal structures. ● Muscle strength and range of motion are examined with and without the prosthesis ● ISO/ISPO radiographic classification ● ACQUIRED AMPUTATION ● Loss of limb or part of limb, the etiology of which may be secondary to trauma, part of treatment etc. ● Accounts to 96 to 99% of all limb losses ● Lower limb amputation is more common the upper extremity amputation Epidemiology ● Average age is 6.18 years ● More distal sites (toe/finger comprising 93% of traumatic amputation) are common than proximal ● Males more common than female ● Single limb 3 A.D. | D.B. | M.G. | A.M. | C.Z. | F.M.
Causes ● Trauma ○ More common ○ Single limb involved (90%) ○ Common in males (3:1) ○ Lower limb more affected ○ Examples: ■ Automobile and motorcycle collisions ■ Burns ■ Gunshot wounds ■ Power tools (lawn mower etc) ● Tumors ○ Highest incidence of malignancy is 12-21 years old ○ Osteogenic sarcoma ○ Ewing’s sarcoma ○ Rhabdomyosarcoma ● Neurologic disorder ● infection ○ Infectious emboli from meningococcemia ○ Pneumococcal septicemia ○ ETIOLOGY ● In children, trauma is the most common cause of amputation ● In adults’ UE: ○ Trauma (80%) ○ Tumors ● In adults’ LE ○ Vascular disease (80%) ■ Diabetic, vascular disease, atherosclerosis, immunologic, idiopathic) ○ Trauma (6-10%) ○ Benign or malignant tumors CLASSIFICATIONS SURGICAL APPROACH AND GENERAL PRINCIPLES ● Surgery guides to optimal function: ○ Preserve length ○ Preserve growth plates ○ Perform disarticulation rather than transosseous amputation ○ Preserve knee joint whenever possible ○ Stabilize and normalize proximal portions of limb ● UE amputations include: ○ Shoulder disarticulation ■ Remove shoulder down to the distal ○ Transhumeral (above-elbow amputation) ○ Elbow disarticulation ○ Transradial (below-elbow amputation) ○ Wrist disarticulation ○ Partial hand amputations LEVELS OF AMPUTATION FOR UE Level of Amputation Procedure Phalangeal disarticulation Excision of any part of one or more finger Metacarpophalangeal Disarticulation at the metatarsophalangeal joint Wrist disarticulation Amputation through metacarpal and distal radius-ulna Below elbow Long below elbow 55-90% of the radius/ulna Medium below elbow 35-55% of the radius/ulna Short below elbow 0-35% of the radius/ulna Elbow disarticulation At the proximal radius/ulna and humerus Above elbow Long above elbow 90-100% short above elbow Standard above elbow 50-90% Short above elbow 30-50% of humerus Very short above elbow 0-30% of humerus Shoulder disarticulation At the shoulder joint Forequarter amputation Removal of part of scapula, clavicle and glenohumeral LE amputations include: ● Translumbar (hemicorporectomy) ● Transpelvic (hemipelvectomy) ● Hip disarticulation ● Transfemoral (above-knee amputation) ● Knee disarticulation (through-knee) ● Transtibial (below-knee amputation) ● Ankle disarticulations (ie, Syme, Boyd, and Pirogoff) ● Partial foot (ie, Chopart and Lisfranc) 4 A.D. | D.B. | M.G. | A.M. | C.Z. | F.M.
LEVELS OF AMPUTATION FOR LE Level of Amputation Procedure Partial toe Excision of any part of one or more toes Toe disarticulation Disarticulation at the metatarsophalangeal joint Transmetatarsal Amputation through midsection of all metatarsals Ankle disarticulation (Syme’s) Ankle disarticulation with attachment of heel pad to distal end of tibia Long transtibial (below knee) More than 50% of tibial length Transtibial (below knee) Between 20-50% of tibial length Short transtibial Less than 20% of tibial length Knee disarticulation Amputation through knee joint Long transfemoral (above knee) More than 60% of femoral length Transfemoral (above knee) Between 35% to 60% of femoral length Short transfemoral (above knee) Less than 35% of femoral length Hip disarticulation Amputation through hip joint Hemipelvectomy Resection of lower half of pelvis Hemicorporectomy Amputation both lower limbs and pelvis below L4-L5 KINDS OF ANKLE DISARTICULATIONS Symes ● ankle disarticulation Boyd’s (Chopart’s) ● preservation of the calcaneus and heel pad and consequent fixation of the calcaneus to the tibia; partial foot amputation through the talonavicular and calcaneocuboid joints Pirogoff ● amputation forefoot and talus are removed and calcaneotibial arthrodesis is performed KINDS OF PARTIAL FOOT Chopart’s ● transtarsal; through talonavicular and calcaneocuboid disarticulation Lisfranc’s ● transmetatarsal COMPLICATIONS ● Terminal growth or spiking at translated end of long bone ○ Most common complication ○ Diaphyseal; overgrowth common in humerus, fibula, tibia and then femur ○ Treatment: distal resection and stump capping ● Bone Spurs ○ Stump scarring and interfere with weight bearing ○ Prosthetic modification ● Phantom Sensation ○ Awareness of missing limb ○ Usually no pain ● Phantom limb pain ○ Non pathologic ○ Rare below 10 years old ○ Not common in congenital ● Pain from overuse syndromes from compensatory techniques, low back pain from altered gait, neuroma ○ Pain can be from overuse 5 A.D. | D.B. | M.G. | A.M. | C.Z. | F.M.