Congenital abnormalities may be defined as defects in the developments of body form on function that are present at the time

of birth. They may be : Localized, as in club foot Generalized, as in Osteogenesis imperfecta. Etiological factors may be caused by genetic defects, enviromental in fluences or combination of the two.

Congenital overriding of the fifth toe Dorsalsublux, of the MP joint, is associated with a shortened extensor tendon and tightness of the over lying skin. Treatment by Z plasty of the skin, tenotomy of the extensor tendon and dorsal capsulotomy of the joint.

Congenital varus of the small toes (curly toes) Is common, particularly in the third toe, the end of the curved toe tends to lie under its medial neighbor but almost never causes discomfort Strapping is ineffectual, and operative treatment is seldom necessary because the deformity usually corrects it self spontaneously.

Metatarsus Primus Varus A varus or adduction deformity of the first Mt in relation to the other four Mts. Medial border of the forefoot is curve inward, and there is a wide space between the first and second toes. Treatment : early by the application of a series of corrective plaster casts to prevent the development of adolescent hallux valgus.

Metatarsus Varus Deviation of all five metatarsals in relation to the hindfoot couses the foot to have a concave innerborder and convex auter border, especially when in a weightbearing position. In most children the deformity is mild (flexible) Avoidance of sleeping face down with the feet curled in when the deformity is rigid or resistant treatment should be started at least within the first few week of life by application of a series of plaster casts for 6 to 12 weeks.

Then a denis browne boot splints is applied nigthly for a few months. When intreated older then 2 years of age : Soft tissue release The child has reached the age 4 years : Perform an osteotomy at the base of each metatarsal

Club foot (Talipes Equinovarus)

Deformity is easy to diagnose but difficult to correct completely The deformities including : Fore foot adduction and supination Heel varus Ankle joint equinus Foot in relation the knee, medial deviation

The degree of deformity : mild, moderate, severe, assessed by its feel of flexibility. Or conversely of rigidity (resistance) Bilateral in half of the afflicted children, boys twice as often as girl. The cause of congenital club foot remains of unsolved puzzles Calf muscles and tibialis posterior are unduly short, fibrous capsules of all the deformed joints are thick and contracted on the concave side of the deformity. Severe clubfoot must be differentiated with either spina bifida or arthrogryposis.

The general principles of treatment : Plaster cast are applied weekly about 6 weeks Then be continued to a Denis Browne shoe splint for 3 months Out flare boots for daily use until the child is 3 years of age

60% of congenital clubfoot are resistent. In resistent clubfoot is better to perform soft tissue correction operation at 4 to 6 months. Soft tissue operations (capsulotomies, tendon lengthening and tendon transfer) are effective in the first 5 years of life, In the child 5 years to 10 year need bony operation (bony reshapping), more than10 years : arthrodesis are usually necessary.

Talipes calcaneovalgus

At the time of birth, have one or both feet in dorsiflexed. This result of intra uterine position, daily passive stretching of the soft tissues by a parent produces excellent and permanent correction of the deformity Only the more resistant deformities require the application of plaster cast

Congenital plantar flexed (Vertical) talus

The talus is rigidly fixed in a position of extreme equinus, a sole of the foot being convex. The diagnosis is confirmed by a lateral X-ray of the foot. Non operative treatment are seldom adequate and soft tissue releasing operation is indicated.

Tarsal coagulation

Any two of the tarsal bones may be joined together by a bridge or bar (Coalition). As a result, movement in the involved tarsal joints is restricted.

Trigger thumb

A constantly flexed I.P joint of the thumb in children Usually caused by a stenosis of fibrous sheath of the flexor pollicis longus tendon. And a secondary nodular enlargement in the tendon at the proximal edge of the contriction Always prevents active extention of IP joint and prevents passive extention (trigger phenomenon), snapping flexion is seldom seen in the congenital type. Under the proximal skin crease, the enlargement in the tendon is readily felt as a nodule that moves with the tendon during passive movement of IP joint Treatment : Surgical

Webbing of fingers (syndactyly)

The most common congenital abnormality of the hand When two adjacent and otherwise normal fingers are joined by a skin web proximally, or throughout their length, a recontruction operation may be expected Incomplete webbing may be corrected during infancy, but in the complete web the recontruction is about 4 years old.

Hypoplasia of Radius (Clubhand)

Relatively uncommon but serious, abnormality. Consists of varying degrees of hypoplasia, or even aplasia of the radial ray. Including radius, scaphoid, trapezium, first metacarpal, thumb and associated muscles, nerves, and blood vessels.

The principles of treatment : early correction, maintenance during growth, improvement of hand function. Passive stretching of the deformity is of limited and temporary value. Early soft tissue operation consisting of Z plasty of the skin, division of the fibrous band, and maintenance of correction in cast (for several month) followed by removable splints. Permanent correction : implantation of the distal end of ulna into a slot fashioned in the carpus (bony operation).

Muscular Torticolis (Way Neck)

The deformity is minimal at birth, within first few weeks of life, a swelling develops in one sternocleido mastoid muscle, this swelling, called a sternocleido mastoid tumor, gradually disappears but leaves a contracture of in volved muscle. As a result, the head becomes tilted, or laterally flexed, toward the affected side and rotated toward the opposite side.

The contracture of the muscle prevents its normal growth in length and, therefore, as the cervical spine grows, the muscle fail to keep pace and becomes relatively shorter. Shortness of muscle on one side not only causes an increase in the tilting and rotation of the head but also results in progressive facial asymetry during the growing years. x-ray is helpful in differentiating congenital muscular torticolis from the uncorrectable of torticolis seen in cervical synostosis (Klippel Feil Syndrome) Treatment : Daily stretching by an experienced physiotherapist

Klippel Feil Syndrome

Failure of vertebral segmentation in cervical spine results in congenital fusion (synostosis) between varying numbers of cervical vertebral. Clinically the childs neck is unduly short relatively stiff, and posterior hairline is low and transverse The head is usually straight, occasionally tilted to one side, resembling muscular toticolis. Surgical treatment is limited to improving the appearance

Developmental Dysplasia of the Hip (DDH) dahulu lebih dikenal sebagai Dislocation of the Hip (CDH) atau cerai panggul bawaan. Perjalanan penyakit, diagnosa dan penatalaksanaan DDH bervariasi, tergantung pada usia pasien. Developmental dysplasia of the hip adalah suatu kondisi dimana struktur dari panggul tidak berkembang secara sempurna. Hal ini dapat disebabkan oleh

1. Lengkung asetabulum yang tidak normal 2. Elastisitas yang berlebihan dari kapsul sendi panggul sehingga kaput femur dapat meluncur ke sisi atas dan lateral dari asetabulum 3. Pemutaran abnormal dari bagian atas tulang femur yang dapat menyebabkan gangguan hubungan antara asetabulum dan kaput femur.

Dengan terjadinya kelainan-kelainan seperti yang disebutkan di atas maka dapat terjadi patologi di bawah ini : 1. Kaput femur tidak sepenuhnya dilindungi oleh socket asetabulum 2. Kaput femur sedikit berpindah ke sisi lateral terhadap socket asetabulum 3. Kaput femur tergelincir keluar dari socket asetabulum

Satu dari 1000 kelahiran hidup ditemukan dengan sendi panggul yang terdislokasi. Sebagian besar para ahli setuju bahwa terjadinya DDH ditentukan oleh factor-faktor genetic yang dimiliki si bayi. Factor intra uterin dan ekstra uterin juga memiliki peranan dalam menyebabkan terjadinya DDH.

Untuk kepentingan deteksi dini, kemungkinan terjadinya DDH harus selalu dipikirkan apabila terjadinya DDH harus selalu dipikirkan apabila pada bayi ditemukan : Letak sungsang Kelamin wanita Adanya riwayat keluarga dengan menderita DDH Tortikolis Metatarsus adduktus Gangguan musculoskeletal

Terjadinya kontraktur pada jaringan lunak dan perubahan pertumbuhan pada kaput femur dan mangkuk asetabulum. Perubahan jaringan lunak terjadi pada neolimbus, labrum dan ligamentus teres. Perubahan pertumbuhan tulang dapat berupa malorientasi pada lempeng pertumbuhan epiisis, gangguan pembentukan tulang baru dari periosteum, gangguan ossifikasi asetabulum, perubahan pada kandungan hiskomia tulang rawan panggul.

Pemeriksaan fisik dilakukan pada ruangan yang tenang dan hangat, bayi diperiksa tanpa memakai baju atau popok. Dimulai dengan observasi untuk mencari adanya asimetri pada lipatan kulit di inguinal atau gluteal mencari tanda dari galleazi. Apabila terdapat keterbatasan dalam kisaran sendi panggul harus selalu dipikirkan kemungkinan DDH.

Tes Ortolani dan Barlow dilakukan untuk mengevaluasi stabilitas sendi panggul. Pada test Ortolani bayi berbaring telentang pada alas yang keras. Untuk memeriksa panggul kiri bayi, tangan kanan pemeriksa menggenggam tungkai atas kiri bayi, dengan jari tengah atau jari manis pemeriksa pada trochanter mayor, sedangkan ibu jari pemeriksa pada trochanter minor. Tangan kiri pemeriksa digunakan untuk memfiksasi pelvis bayi dengan panggul kanan bayi dalam abduksi.

Tangan kanan pemeriksa mulai melakukan abduksi panggul bayi sekaligus melakukan tekanan pada trochanter mayor ke atas sehingga panggul bayi terangkat. Sensasi klik menandakan panggul yang terdislokasi tereposisi ke dalam asetabulum. Pada tes Barlow, bayi diletakan dalam posisi yang sama. Ibu jari pemeriksa berada pada bagian distal yang sama. Ibu jari pemeriksa berada pada bagian distal sisi medial tungkai atas dan digunakan untuk menekan tungkai atas ke bawah dan ke lateral. Apabila kaput femur keluar dari mangkuk asetabulum, dikatakan panggul dislocatable. Apabila kaput femur bergeser tetapi tidak keluar dari mangkuk asetabulum dikatakan panggul subluxatable

Pada anak berusia 2 tahun akan ditemukan cara berjalan yang pincang (trendelenburg)bila satu sisi panggul menderita DDH, atau bahkan berjalan seperti bebek (waddling gait) bila kedua sisi panggul terkena. Juga akan ditemukan lipatan kulit sekitar panggul yang multiple, perineumnya melebar dan trochanter terlihat menonjol.

Foto Polos Garis Hilgenreiner adalah garis yang ditarik horizontal melewati kedua kartilago triradiata. Garis perkins adalah garis yang ditarik tegak lurus terhadap garis Hilgenreiner pada sisi lateral asetabulum. Pergeseran kaput femur atau eksternal rotasi berlebihan pada panggul akan mengakibatkan terputusnya garis shenton.

Pavlik Harness ditemukan pada tahun 1958 oleh Arnold Pavlik (Chekoslovakia). Indikasi pemakaian alat ini adalah DDH yang masih dapat direduksi pada anak yang masih belum belajar berjalan. Pada usia 5-6 minggu dilakukan pemeriksaan klinis dan ultrasonografi. Apabila pemeriksaan pada usia 5-6 bulan hasilnya normal, maka observasi dilanjutkan dan pada usia 6 bulan dilakukan pemeriksaan radiologis. Pada bayi dengan panggul yang tidak stabil dan Tes Barlow (+) maka dilakukan pemasangan Pavlik Harness, pemakaian Pavlik Harness dihentikan setelah 2-3 bulan. Pasien di follow up sampai remaja.

Aplikasi pavlik Harness

Ultrasanografi untuk merekam masuknya panggul ke asetabulum

Follow up klinis dan ultrasonografi tiap minggu

Evaluasi klinis dan ultrasonografi dengan cermat pada minggu ke-3

Tereduksi teruskan harness sampai USG dan Radiologis normal

Meragukan Tidak stabil namun dapat tereduksi fixed abduction brace

Tidak tereduksi Lakukan traksi kemudian adductor tenotomy konfirmasi dengan arthrogram kemudian gips

1. Ruang tenang dan hangat 2. Bayi tanpa pakai baju (popok) 3. Observasi asimetri lipatan kulit di inguinal atau gluteal 4. Cari tanda galeazi

5. Bila ada keterbatasan kisaran sendi panggul pikirkan akan DDH 6. Test Ortholani dan Barlow Evaluasi stabilitas sendi panggul Anak 2 tahun : Jalan pincang (trendelenburg) Bila 2 sisi panggul : waddling gait.

TERIMA KASIH