DEVELOPMENT OF LIMBS Aims and Objectives At the end of lecture, students should be able to: 1. Know and define the site and time of appearance of upper and lower limb bud 2. Define apical ectoderrmal ridge (AER) 3. Understand mesenchymal proliferation under the influence of AER and differentiation into cartilaginous models of future limb bones 4. Understand and define the source of mesoderm forming the limb muscles 5. Know the hand plate and formation of digital rays resulting into digits 6. Define and understand the rotation of upper limb with final position of flexor and extensor limb muscles 7. Define common congenital anomalies of limbs The parts of the vertebrate limb ► ► ► ► ► ► ► ► ► ► ► The resulting limb is divided into four basic elements; a root consisting of the scapula and clavicle in the forelimb and hip bones in the hind limb, a proximal part consisting of a single skeletal element called the Stylopod (in the forelimb this is the Humerus, in the hind limb the femur), a middle portion, the Zeugopod with two elements (ulna and radius in the forelimb or fibula and tibia in the hind limb) and a distal part, the Autopod composed of a carpus (hand) or tarsus (foot) with a variable number of digits. autopod stylopod zeugopod THE DEVELOPMENT OF LIMBS ► The limb bud appear from ventro-lateral body wall during 5th week of development at the level of lower cervical and first two thoracic segments ► The mesenchymal core was initially derived from somatoplrure of lateral plate mesoderm ► This mesenchyme give rise to bones & connective tissue covered by a layer of cuboidal ectoderm ► The ectoderm at tip of limb ,thickened to form Apical Ectodermal Ridges ► Ridges exerts the inductive influence on underlying mesoderm ► The mesenchyme adjacent to AER remains as undifferenciated , rapidly proliferating cells ► The cells far away from from inductive influence begins to differentiate into cartilage &muscles ► The development of limb proceeds to proximo-distal direction ► During sixth week the limb bud flattens to form the handplate & footplate ► The first circular constriction separate these plates from proximal segment ► Second constriction divide the proximal segment into two segments ► Fingers & toes are formed when cell death in AER separate ridges into five parts ► The formation of finger is dependent upon their continuing outgrowth under the inductive influence of five segments of ridge ectoderm ,condensation of mesenchyme to form cartilaginous digital rays & death of intervening tissue between rays ► The development of U/L & L/l is similar , morphogens of L/L appear two days later than U/L ► During 7th week the limbs rotate in opposite direction ► The U/L rotates 90 degrees laterally so that, the extensor muscle lie on lateral and posterior surface & thumb lie laterally ► The L/L rotates 90 degrees medially ,so that extensor muscles lie anteriorly & big toe faces medially ► When the external shape is being established , mesenchyme in buds begin to condense ,by the end of sixth week the first hyaline cartilage model of bones can be recognized ► Ossification of bones of extremities, the endochondral ossification begins by the end of embryonic period. ► From primary centres of ossification in shaft or diaphysis ,endochondral ossification progresses gradually towards the end of cartilaginous “model.” ► At birth diaphysis of bone is completely ossified but the epiphysis remains cartilaginous,shortly ossification centres appear in epiphysis ► Temporarily the cartilaginous plate remains between diaphysis & epiphysis ► This cartilaginous plate plays an important role in growth in length of long bones ► On both sides of plate , endochondral ossification proceeds, when the bone acquire a full length , the epiphyseal plate disappear & the epiphysis then join the shaft of long bone ► In long bones epiphyseal plate is found on both extremeties , whereas in smaller bones ,it is found only on one extremity CINICAL COORRELATES ► The bone age of child can be studied by the radiologist to sort out whether the child has reached his or her proper maturation age ► The ossification centers in hand &wrist of children are studied ► Prenatal analysis of fetal bones by U/S provide information about fetal growth & gestational age Anomalies ► Minor congenital limb anomalies are relatively more common than major anomalies and can be corrected surgically. ► Most of the major defects are caused by genetic factors ► Most critical period for limb development is from day 24 to 36 in which environmental factors can affect ► ► ► ► ► MEROMELIA : is partial absence of limbs AMELIA is total absence of limb PHOCOMELIA where long bones absent or rudimentary MICROMEDIA all segments of bones are present but are very short These abnormalities are may be due to teratogenic insults e.g. thalidomide (sleeping pills or antinauseant falum during 4-5th week of development may lead to absence or gross deformities of long bones, intestinal atresia & cardiac anomalies ► Other category of limb development is presence of extra fingers and toesPOLYDACTLY ► The extra digit lack proper muscular connection excessive number of bones are present and may be bilateral ► ECTODACTYLY- absence of digit, such as thumb-umblitical ► SYNDACTYLY- abnormal fusion of fingers or toes ► CLEFT HAND AND FOOT (lobster claw deformity, abnormal cleft between 2nd & 4th metacarpal bones & soft tissues) ► The 3rd metacarpal & phalangeal bones are about always absent, thumb & index fingers as well as 4-5th fingers may be fused ► The two parts of hands are some what opposed to each other and act like lobster claw ► CLUB FOOT: is usually present in combination with syndactyly ► The sole of foot is turned inwards, the foot is adducted and planter flexed ► ► ► ► ► Common in males, hereditary. Congenital absence of deficiency of radius Genetic Associated with craneisyntosis-radial aplasia syndrome Digital defects along with it are absent thumb and short corner ulna usually present ► AMNIOTIC BANDS: may cause rung constrictions of limbs or digits & amputation ► CONGENITAL HIP DISLOCATIONS: ► Consists of underdevelopment of Acetabulum & head of femur ► Common and occurs in female ► Abnormality of bones occur precaudally whereas dislocation may occur after birth ► Baby with breech deliveries are affected. CONGENITAL TALIPES EQUINAS VARUS