Pediatric congenital anomalies

DDH, CDH, CHD

DDH is a spectrum of hip development disorders that is present at different ages and in different forms. The common etiology is excessive laxity of the capsule that fails to maintain the femoral head inside the acetabulum. So the hip can be subluxatable, subluxated or dislocated. Which can be manifested later in childhood or adolescence as a poor acetabular coverage as ( acetabular dysplasia). CHD Guillaume Dupuytren 1832 versus DDH, Klisic 1989. Teratologic hip dislocation, not reducible, other anomalies, myelodysplasia and A.m.c.

Prof.Adolph Lorenz the father of AVN. Putti, 1927, early tx before 1y of age. Ortolani 1935, mother click 5mons . Incidence 1-3 per 1000. 1.7-1000 in Sweden, 75-1000 in yugos., 188.5-1000 Canada

etiology
Racial prediliction. Ligamentous laxity (maternal relaxin hormones) , Wynne-Davies criteria, collagen 3 >coll. 1 . Prenatal positioning, breech positions have a significant higher risk of DDH. First born children and pregnancies with oligohydramnios. Left hips in female (sacrum) Postnatal positioning (wrap babies in a hip extended position). Failure of acetabular development

Associated conditions
Torticollis 20%

Metatarsus adductus 5%

Normal hip development.

The hip begins to form at the 7th week of gestation. The acetabulum at 11th week. At birth the acetabulum is composed of cartilage with a thin rim of fibrocartilage called the labrum. The triradiate cartilage Large part of the proximal femur is cartilagenous, head. Great and lesser trochanters. Ossification of the head starts at 4-7 mons. Meyers dysplasia.

Hip development in DDH

DDH development is gradually progressive. At birth the affected hip will slide in and out of the acetabulum, to do so the posteriosuperior labral rim should be flattened and thickened (neolimbus). This movement will produce a clunk or sacatto sound (Ortolani) Some hips return to act. And becomes normal. Other hips remain out of the acet. and develop secondary barriers to reduction.

Secondary barriers to reduction

In depth of acet. Fat tissue thickens (pulvinar) and may impede reduction Ligamentum teres may elongate and thickens and occupy space to impede reduction. The transverse acetabular ligament may enlarge and impede reduction. The inferior capsule portion of the hip forms an hourglass shape with an opening smaller than the femoral head. The iliopsoas muscle contributes to the narrowing of the isthmus.

When the femoral head is reduced the head gradually deepens into the acetabulum (docking the head) . If head remains dislocated, other changes occure in the acetabulum, Acetabular roof becomes more oblique. The concavity flattens and becomes straight . The medial wall of acet. Thickens The acet. Becomes more anteverted. Medial twisting of the whole wing of the pelvis. All these changes are reversible until age of 4 (harris), because growth of the acetabulum continues until age of 8. If dislocation remains and becomes irreversible a pseudoscetabulum starts to form.

TERMS
Unstable hip, hip instability + ortolani or Barlow tests. Stable hips, radiologically unstable. Coleman criteria of unstable hip: Jerk on exit and entry sign (ortolani sign). Acetabular index more than 40 degree. Lateral displacement of femoral head to the vertical line of Perkins. Acetabular dysplasia Sublaxation, dislocation.

Clinical presentation the neonate

Femoral head ossification Clinical evaluation and USG

Physical examination Barlows test:

The examiner tend to subluxate or dislocate the head from the acetabulum by gentle adduction and posterior push off (Barlows sign).

Physical examination Ortolanis test

The reverse of barlows test , the examiner relocate a dislocated hip by abduction and medial push.

Radiological examination
X-ray USG can show the delicate soft tissue anatomy of the hip joint. static and dynamic teqn. Graf proposed a classification system based on angles formed by the sonographic structures of the hip.

The infant
By the second and third mons of life other signs appear. Previous tests become less sensetive Limitted abduction. Shortening of the thigh (the Galeazzi sign). Both hips in 90 degree of flexion.

Asymetrical guteal folds (because of dislocation) more thigh folds on the affected side, be careful of a bilateral dislocation.

THE Klisic test : Place the third finger over the great trochanter and the index finger over the SAIS, an imaginary line drown bwt tips of fingers should pass through the umbilicus, when hip is dislocated the line passes bwt the umbilicus and the pubis. Nelatons line: is drawn between the ischial tuberosity and anterior superior iliac spine. In the normal hip the tip of the greater trochanter lies at or below Nelatons line whereas in the dislocated hip it lies superior to the line. X-RAY

The walking child

Dislocated side shorter than other side, the baby will walk on toes (abductor lurch,Trendelenbergs gait) (trendelenbergs sign). Limited abduction Galeazzi sign Excessive lordosis X-ray

X-ray can show the dislocated hip easily at any age but in neonates unstable hips can be missed easily Femoral head ossification 4-6 mons

lines
Hilgenreiners line. Perkins- Ombredanne line. Shentons line.

Angles
The acetabular index In new borns averages 27.5 degrees At 6 mons of age mean is 23.5 deg. By 2 years decrease to 2o degr. 30 degree is consideredthe upper limit of normal.

Central edge angle of Wilberg; measures hip position. From 6-13 years old patients; angle of 19 deg. is considered normal. Children of 14 yrs and older; angle of 25 deg. And more is normal

The Von Rosen view: - Both hips are abducted , internally rotated,and extended. * In normal hips an imaginary line from the femoral shaft intersect the acetabulum.

TEAR DROP FIGURE

Seen on AP views of the pelvis. Formed by the acetabulum laterally,the wall of the lesser pelvis medially,and the acetabular notch. It appears bwt 6-24 mons in normal hips, and later in dislocated hips. Four types of tear drop bodies ( open, closed, reversed and crossed) . It has tow shapes U and V , V shaped tear drops associated with dysplastic hips and carry a poor prognosis.

treatment
Neonate: place in Pavlik Harness for 6 weeks. 1 to 6 mons: place in pavlik harness for 6 weeks after hip reduces. 6 to 18 mons: traction and reduction and placement in cast for 3 mons, if open reduction doesn't succeed open reduction is done. 18 to 24 mons: try close reduction. 24 to 6 years: open reduction is done,

In open reduction
To correct acetabular dysplasia; acetabular osteotomiies can be done. Innominate osteotomy. Salter osteotomy. Pembertons osteotomy Dega osteotomy Steel , chiari, ganz.etc.

THANX