CONGENITAL HIP DISLOCATIONAndLEGG

CALV

PERTHES DISEASEI.

Meaning / Other Names / History of DiseaseCHD LCPDMEANING: Congenital dislocation of hip is a conditionpresent since birth in which the head of the femur isdetached from the acetabulum or can be moved in and outof the acetabulum easily. Over time the ability to move thehead back into the acetabulum is lost and the headremains permanently dislocated (outside the acetabulum). OTHER NAME/s: Developmental dislocation of the hip joint; Developmental hip dysplasia; DDH; Congenitaldysplasia of the hip; Congenital dislocation of the hip. HISTORY: The term

Congenital dislocation of the hip datesback to the time of Hippocrates. This condition, alsoknown as DDH, has been diagnosed and trested for severalhundred years. Most notably, Ortolani, an Italianpediatrician in the early 1900s, evaluated, diagnosed, andbegan treating hip dysplasia. Galeazzi later reviewed morethan 12,000 cases of DDH and reported the associationbetween apparent shortening of the flexed femur and hipdislocation. Since then, significant progress has been madein the evaluation and treatment of DDH. MEANING: LCPD is a disorder of the epiphysis (growingpart of bone) of the upper end of femur. It is characterisedby the loss of blood supply in the upper femoral epiphysis.The epiphysis is necrosed (becomes dead). It then getsabsorbed and replaced. This leads to deformation of thefemoral head. OTHER NAME/s: Perthes disease, ischemic necrosis of thehip, coxa plana, osteochondritis and avascular necrosis of the femoral head, Legg Perthes Disease or Legg Calve-Perthes Disease (LCPD). HISTORY: It is named for Arthur Legg, Jacques Calv andGeorg Perthes. II.

INCIDENCE AND PREVALENCECHD LCPDGlobal: The annual incidence of congenital hip dislocation isapproximately 2-4 cases per 1000 births, andapproximately 80-85% of the affected individuals are girls. Philippines:Global: LCPD usually occurs in children aged 4-10 years, witha mean age of 7 years. It occurs more commonly inboys than in girls, with a male-to-female ratio of 4:1.The condition is rare, occurring in approximately 4 of 100,000 children. Philippines:III.

SIGNS AND SYMPTOMS

Hip Dysplasia and Hip Anatomy

To understand how hip dysplasia occurs and how doctors treat it, you need to know a little bit about the hip jointitself. The hip is a ball-in-socket joint, or an enarthrosis. The rounded head of the femur forms the ball, which fits into the socket of the acetabulum. The hip forms the primary connection between the bones of the lower limbs and the axial skeleton of the trunk and pelvis. Three pelvic bones -- the ilium, the ischium and the pubis -- come together to form the acetabulum. The joint itself may not be fully ossified, or hardened into bone, until a person reaches the age of 25 years, which is one reason why early detection is important in hip dysplasia treatment. A strong, lubricated layer of articular hyaline cartilagecovers both surfaces of the joint completely, helping it move more smoothly. A rim called the labrum grips the head of the femur and secures it in the joint. This increases the depth of the acetabulum. While in the uterus, a baby's hip should develop with the femoral head sitting perfectly centered in the acetabulum. The acetabulum should cover the head of the femur as if it were a ball sitting inside of a cup. In congenital hip dysplasia, the development of the acetabulum allows the femoral head to ride upward out of the socket, especially when the baby begins to walk. This results in a shallow socket -- it's shaped less like a cup and more like a bowl. It can also lead to femoral misplacement because the acetabulum doesn't cover the femoral head sufficiently. If this isn't corrected, the hip joint will be unstable. In some cases, dislocation may also occur. Over the years, as the femoral head and acetabulum move without correct alignment, the cartilage in the joint wears down prematurely and unevenly. The result is differing degrees of osteoarthritis, depending on the severity of the misalignment. The osteoarthritis can occur on the joint head or in the socket itself. An atypically shaped thighbone can also be part of hip dysplasia. This can occur in addition to or in place of a misshaped acetabulum. The ball and shaft of the femur ideally form a 120- to 135-degree angle where they meet. But in hip dysplasia, that angle can change in one of two ways:

Coxa valga: The angle between the ball and the shaft of the femur is increased, usually above 135 degrees. Coxa vara: The angle is reduced to less than 120 degrees. In either case, the length of the femur is affected -- it's shorted with coxa vara or lengthened with coxa valga. This creates additional imbalance within the hip joint and results in difficulty walking, pain and joint stiffness. Doctors don't know exactly what causes hip dysplasia. However, a possible cause could be hormonal changes within the mother. During pregnancy, hormones loosen a woman's joints to allow them to expand and make room for the baby during delivery. These hormones are thought to cross over the placenta and cause the baby to have increased ligament looseness. The baby's position in the womb may play a role as well -- breech birth positioning and firstborn children have increased rates of developmental dysplasia of the hip (DDH). As many as one in 15 females born in breech position has DDH [source: Ramsey]. The left leg is more often involved due to the baby's positioning in the uterus. There is also a 10-fold increase in the frequency of hip dysplasia in children whose parents had DDH compared with those whose parents did not [source: Bjerkreim].

Diagnosing Infant Hip Dysplasia

Ortolani and Barlow maneuvers have been the standard techniques for detecting hip problems in babies for the last 70 years. The Ortolani maneuver starts with the baby in a supine position -- on his or her back -- without a diaper. The physician bends the infant's hip and knees to 90 degrees. Once in this position, the doctor abducts, or moves the infant's legs apart, into the frog-leg position. If the hip is dislocated, the femoral head slides into the hip socket during this movement. There is a palpable and audible clunk as the head slips back into the socket. Each hip should be tested individually. Hearing or feeling the clunk is called a positive Ortolani maneuver. In the Barlow maneuver, the physician will examine each leg individually beginning with the infant lying in the supine position. If testing the left leg, the physician will support the pelvis with the right hand. Using the left hand, the doctor will flex and adduct the left leg, or draw it across the body. The hip will gently be pushed posteriorly -- toward the back -- along the line of the femur shaft. In this case, a positive test occurs if the physician feels the femoral head slip out of the acetabulum as the hip dislocates out of the socket. Ultrasonograms, or ultrasounds, can also be useful for diagnosis, particularly for babies under 9 months of age. Prior to 9 months, babies have little bone, and X-rays show only bone. Ultrasound also allows the doctor to see multiple angles of the joint rather than the single, two-dimensional view an X-ray provides. An ultrasound exam can accurately show the location of the ball within the socket, and the doctor can stress the hip during the examination to determine the stability of the joint. Ultrasound can also show the cartilage in infants who do not yet have a lot of bone. Once children develop sufficient bone, X-rays tend to be used because they can more clearly show the bones of the whole hip joint in one image. This makes them more useful than ultrasound after the age of 9 months. The process of diagnosing an adult with hip dysplasia is a little different from diagnosing an infant -- we'll look at how on the next page.