PORTOFOLIO KASUS MEDIS RADIAL CLUBHAND D

PORTOFOLIO
KASUS MEDIS
RADIAL CLUBHAND

Disusun oleh:
dr. Aditya Agung Prasetyo

DOKTER INTERNSHIP PERIODE MEI 2013 – MEI
2014
RSUD BENDAN
KOTA PEKALONGAN

BERITA ACARA PRESENTASI PORTOFOLIO

Pada hari ini tanggal ........................................................... telah dipresentasikan
oleh:
Nama Peserta

: dr. Aditya Agung Prasetyo

Dengan Judul/Topik : Radial ClubHand

Nama Pendamping I : dr. Yusuf Khairul, Sp.OT
Nama Pendamping II : dr. Rini Handayani
Lokasi Wahana
No
.

: RSUD Bendan – Kota Pekalongan
Nama Peserta Presentasi

Tanda Tangan

Berita acara ini ditulis dan disampaikan sesuai dengan yang sesungguhnya.

Pendamping I

(dr. Yusuf Khairul, Sp.OT)

Pendamping II

(dr. Rini Handayani)

Borang Portofolio
Nama Peserta: dr. Aditya Agung Prasetyo
Nama Wahana : RSUD Bendan Kota Pekalongan
Topik : Radial Clubhand
Tanggal (Kasus) : 10 Februari 2014
Nama Pasien : An. G (7 bulan)
No. RM : 091xxx
Tanggal Presentasi :
Pendamping : dr. Yusuf Khairul, Sp.OT dan
dr. Rini Handayani
Tempat Presentasi : RSUD Bendan Kota Pekalongan
Objektif Presentasi :
□ Keilmuan □ Ketrampilan □ Penyegaran □Tinjauan Pustaka
□ Diagnostik □ Manajemen □ Masalah
□ Istimewa
□ Neonatus □ Bayi □ Anak □ Remaja □ Dewasa □ Lansia
□ Deskripsi :

□ Bumil

Pasien anak laki-laki usia 7 bulan datang dengan keluhan kedua tangan
bengkok. Keluhan tersebut sudah ada sejak pasien lahir. Menangis kebiruan (-),
mual muntah (-), BAB dan BAK dalam batas normal. Pasien hanya minum ASI
saja.
Pasien sudah pernah berobat ke dokter spesialis anak dan dikatakan dalam
keadaan baik dan tidak ada kelainan yang lainnya.
□ Tujuan :
Mengetahui klinis dan penegakkan diagnosis radial clubhand
Bahan Bahasan : □ Tinjauan Pustaka □ Riset
□ Kasus
□ Audit
Cara Menbahas : □ Diskusi
□ Presentasi dan Diskusi □ Email □ Pos
Data Pasien :

Nama : An. G

Nama Klinik : RSUD Bendan

No. Registrasi : 091xxx
Telp : 0285-

Terdaftar: 09/02/2014

Data utama untuk bahan diskusi :
1. Diagnosis/gambaran klinis : Pasien anak laki-laki usia 7 bulan datang
dengan keluhan kedua tangan bengkok. Keluhan tersebut sudah ada sejak
pasien lahir. Menangis kebiruan (-), mual muntah (-), BAB dan BAK dalam

batas normal. Pasien hanya minum ASI saja.
Pasien sudah pernah berobat ke dokter spesialis anak dan dikatakan dalam
keadaan baik dan tidak ada kelainan yang lainnya.
2. Riwayat Kesehatan/Penyakit : Riwayat trauma disangkal. Riwayat sakit berat
disangkal. Riwayat imunisasi lengkap sesuai umur.
3. Riwayat Obstetri :
1. Riwayat kehamilan
-

Riwayat penggunaan obat-obatan

Riwayat penggunaan jamu-jamuan
Riwayat sakit parah

: disangkal
: disangkal
: disangkal

2. Riwayat persalinan
Pasien lahir cukup bulan, spontan, menangis spontan, ditolong oleh
dokter spesialis kandungan, BBL 3200 gram, PB 49cm.
4. Riwayat Keluarga : riwayat sakit serupa disangkal
5. Riwayat Asuhan dan Ekonomi : Pasien anak kelima dari 5 bersaudara. Pasien
sehari-hari diasuh oleh ayah dan ibunya. Ayah pasien bekerja di perusahaan
swasta. Pasien berobat dengan fasilitas Umum.
6. Kondisi Lingkungan Sosial dan Fisik : pasien merupakan anak kelima dan
diasuh di rumah bersama ayah dan ibunya di rumah yang sederhana.
7. Pemeriksaan Fisik:
a) Keadaan Umum : pasien tampak baik, keadaan gizi cukup, kesadaran
compos mentis.
b) Tanda-tanda vital : suhu 36,20 C; RR 30 x/ menit; nadi 120 x/menit, BB :

6,7kg
c) Keadaan Tubuh










Kepala
Kulit
Mata

: mesosefal
: turgor baik, pucat (-), sianosis (-), ikterik (-)
: konjungtiva anemis (-/-), pupil
isokor, reflek pupil (+/+), sklera ikterik (-/-)

Hidung
: sekret (-/-)
Telinga
: discharge (-/-)
Mulut
: kering (-), sianosis (-), lidah typhoid (-)
Leher
: simetris, pembesaran kelenjar limfe (-)
Tenggorokan : T1-T1, faring hiperemis (-)
Thoraks
o Paru-paru








Inspeksi : statis dan dinamis simetris, retraksi (-)

Palpasi
: fremitus taktil kanan = kiri
Perkusi : sonor/sonor
Auskultasi : suara dasar vesikuler (+/+) normal, suara tambahan
(-/-)
o Jantung
Inspeksi : ictus cordis tidak tampak
Palpasi
: ictus cordis tidak teraba
Perkusi : batas atas jantung : ICS II linea parasternalis sinistra
batas pinggang jantung: ICS II midclavicularis sinistra
batas kanan bawah jantung : ICS IV linea sternalis
dextra
batas kiri jantung
: ICS V 2 cm medial linea
midclavicularis sinistra
Auskultasi : bunyi jantung I-II reguler, frekuensi 120 x/menit,
bising (-), gallop (-)
Abdomen
Inspeksi : permukaan cembung, dinding perut sejajar dinding dada

Auskultasi : bising usus (+) 11x/menit
Perkusi : timpani
Palpasi
: nyeri tekan (-), defans muskular (-), hepar &
lien sulit teraba
Sistema Genitalia : ulkus (-), sekret (-), tanda-tanda radang (-).
Ekstremitas
Akral dingin
- Oedem - - - Status Lokalis

Regio Manus Dekstra

Inspeksi : tampak angulasi ke arah radial, tampak ada crease antara digiti I dengan
pergelangan tangan, tampak atrofi muskulus thenar
Palpasi : teraba adanya jaringan fibrosis di daerah radius distal, muskulus thenar
mengecil, manipulasi ke arah ulna side bisa namun sedikit lebih susah
dibandingkan dengan yang kiri

ROM pasif : susah dievaluasi karena pasien tidak kooperatif
Regio Manus Sinistra

Inspeksi : tampak angulasi ke arah radial, tampak ada crease antara digiti I dengan
pergelangan tangan, tampak atrofi muskulus thenar, hipoplasia thumb
Palpasi : teraba adanya jaringan fibrosis di daerah radius distal, muskulus thenar
mengecil, os digiti I tidak terbentuk, manipulasi ke arah ulna side bisa lebih
ringan dibandingkan yang kanan
ROM pasif : susah dievaluasi karena pasien tidak kooperatif
d) Pemeriksaan Laboratorium
Pemeriksaan Laboratorium tanggal 9 Februari 2014
Hemoglobin :10,1

Golongan Darah : O+

Hematokrit : 30

PT : 11,6

AL : 7,483.103

APTT : 36,8

AT : 228.103

INR : 0,91

AE : 3,72.106
Daftar Pustaka :
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years after implantation of bone graft. Ann Surg 1928; 87:105.
Bardenheuer, 1894. Bardenheuer B: Vorstellung von 4 Patienten, an welchen die
totale Resection des ganzen Hüftgelenkes ausgeführt worden war, Berich
über d Verhandl d Deutsche Gesellschaft f Chir, Leipzig, 23 Kongr, 105,
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Bartlett et al., 2001. Bartlett GR, Coombs CJ, Johnstone BR: Primary shortening
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Bayne and Klug, 1987. Bayne LG, Klug MS: Long-term review of the surgical
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Behrens and Sabharwal, 2000. Behrens F, Sabharwal S: Deformity correction and
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Relat Res 2000; 375:113.

Bora et al., 1970. Bora Jr FW, Nicholson JT, Cheena HM: Radial meromelia. J
Bone Joint Surg 1970; 52A:966.
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method and results in aplasia and hypoplasia of the thumb. J Bone Joint
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radial club hand. J Hand Surg 1985; 10A:964.
Catagni et al., 1993. Catagni M, Szabo RM, Cattaneo R: Preliminary experience
with Ilizarov method in late reconstruction of radial hemimelia. J Hand
Surg 1993; 18A:316.
Damore et al., 2000. Damore E, Kozin SH, Thoder JJ, et al: The recurrence of
deformity after surgical centralization for radial clubhand. J Hand Surg
2000; 25A:745.
DeLorme, 1969. DeLorme TL: Treatment of congenital absence of radius by
transepiphyseal fixation. J Bone Joint Surg 1969; 51A:117.
Goldfarb et al., 2002. Goldfarb CA, Klepps SJ, Dailey LA, et al: Functional
outcome after centralization for radius dysplasia. J Hand Surg
2002; 27A:118.
Gossett, 1949. Gossett J: La pollicisation de l'index. J Chir 1949; 65:403.
Heikel, 1959. Heikel HVA: Aplasia and hypoplasia of the radius. Acta Orthop
Scand Suppl 1959; 39:1.
James et al., 2004. James MA, Green HD, McCarroll Jr HR, et al: The
association of radial deficiency with thumb hypoplasia. J Bone Joint Surg
2004; 86A:2196.
James et al., 1999. James MA, McCarroll R, Manske PR: The spectrum of radial
longitudinal deficiency: a modified classification. J Hand Surg
1999; 24A:1145.
Kato, 1924. Kato K: Congenital absence of the radius, with review of the
literature and report of three cases. J Bone Joint Surg 1924; 6:589.
Kawabata et al., 1998. Kawabata H, Masatomi T, Yasui N: Residual deformity in
congenital radial club hands after previous centralization of the wrist: ulnar
lengthening and correction by the Ilizarov method. J Bone Joint Surg
1998; 18A:316.
Kessler, 1989. Kessler I: Centralization of the radial club hand by gradual
distraction. J Hand Surg 1989; 14B:37.
Kummel, 1895. Kummel W: Die Missbildungen der Extremitäten durch Defekt,
Verwachsung und Ueberzahl, Kassel, Germany, Bibliotheca Medica, 1895.
heft 3
Lamb, 1972. Lamb DW: The treatment of radial club hand: absent radius, aplasia
of the radius, hypoplasia of the radius, radial paraxial hemimelia. Hand
1972; 4:22.
Lamb, 1977. Lamb DW: Radial club hand, a continuing study of sixty-eight
patients with one hundred and seventeen club hands. J Bone Joint Surg
1977; 59A:1.

Lamb et al., 1982. Lamb DW, Wynne-Davies R, Soto L: An estimate of the
population frequency of congenital malformations of the upper limb. J
Hand Surg 1982; 7A:557.
Lidge, 1969. Lidge RT: Congenital radial deficient club hand. J Bone Joint Surg
1969; 51A:1041.
Manske and McCarroll, 1978. Manske PR, McCarroll Jr HR: Abductor digiti
minimi opponensplasty in congenital radial aplasia. J Hand Surg
1978; 3A:552.
Manske et al., 1981. Manske PR, McCarroll Jr HR, Swanson K: Centralization of
the radial club hand: ulnar surgical approach. J Hand Surg 1981; 6A:423.
Menelaus, 1976. Menelaus MB: Radial club hand with absence of the biceps
muscle treated by centralization of the ulna and triceps transfer: report of
two cases. J Bone Joint Surg 1976; 58B:488.
Milford, 1987. Milford L: The hand: congenital anomalies.
In: Crenshaw AH, ed. Campbell's operative orthopaedics, 7th ed. St
Louis: Mosby; 1987.
Mo and Manske, 2004. Mo JH, Manske PR: Surgical treatment of type 0 radial
longitudinal deficiency. J Hand Surg 2004; 29A:1002.
Nanchahal and Tonkin, 1996. Nanchahal J, Tonkin A: Preoperative distraction
lengthening for radial longitudinal deficiency. J Hand Surg
1996; 21A:103.
Niall et al., 1998. Niall DM, Murphy PG, Fogarty EE, et al: Correction of soft
tissue contractures of the wrist using the Ilizarov technique. J Hand Surg
1998; 23A:442.
Prokopovich, 1980. Prokopovich VS: Aligning of length of the forearm bones in
the congenital club-hand in children. Ortop Travmatol Protez 1980; 1:51.
Riordan, 1955. Riordan DC: Congenital absence of the radius. J Bone Joint
Surg 1955; 37A:1129.
Riordan, 1963. Riordan DC: Congenital absence of the radius, a 15-year followup. J Bone Joint Surg 1963; 45A:1783.
Riordan et al., 1975. Riordan DC, Powers RC, Hurd RA: The Huber procedure
for congenital absence of thenar muscle. Paper presented at the annual
meeting of the American Society for Surgery of the Hand, San Francisco,
Calif, Feb 27, 1975.
Sabharwal, 2004. Sabharwal S: Treatment of acquired radial club hand deformity
with Ilizarov apparatus. Clin Orthop Relat Res 2004; 424:143.
Sabharwal et al., 2005. Sabharwal S, Finuoli AL, Ghobadi F: Pre-centralization
soft tissue distraction of Bayne type IV congenital radial deficiency in
children. J Pediatr Orthop 2005; 25:377.
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Orthop Assoc 1893; 6:208.
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distraction in congenital forearm deficiency. J Hand Surg 1995; 20A:420.
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deficiency. J Pediatr Orthop 1987; 7:699.
Watson et al., 1984. Watson HK, Beebe RD, Cruz NI: Centralization procedure
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Hasil Pembelajaran
I. Definisi
Radial clubhand atau sering disebut radial defisiensi adalah semua bentuk
kelainan berupa kegagalan pembentukan tulang panjang sepanjang preaxial
atau garis os radius pada ektremitas atas, yang meliputi defisiensi atau
ketidakadaan otot thenar; ibu jari memendek, tidak stabil atau tidak terbentuk;
os radius memendek atau tidak terbentuk. Kejadian radial clubhand sekitar
satu banding 100.000 kelahiran hidup. Deformitas bilateral terjadi sekitar
50% dari keseluruhan pasien, sementara jika deformitasnya unilateral, sisi
kanan lebih sering terkena. Laki-laki dan perempuan sama.
II. Etiologi
Penyebab terjadinya radial clubhand belum diketahui. Dari 35 pasien dengan
radial clubhand, Lamb pada tahun 1977 menemukan bahwa semua pasien
dengan gangguan derajat 3 dengan defisiensi yang sama, tidak ada hubungan
darah. 12 dari 35 pasien diketahui ibunya mengkonsumsi thalidomide. Pada
studi lainnya, Wynne-Davies dan Lamb menemukan adanya hubungan yang
lebih besar antara gangguan derajat pertama dengan anomali kongenital
minor dibandingkan dengan survei secara random, yang dihubungkan dengan
faktor genetik. Mereka juga menemukan adanya faktor lingkungan pada
penyebab terjadinya radial clubhand. Defisiensi radial dengan anemia
Fanconi dan trombositopenia diturunkan secara autosom resesive, sedangkan
defisiensi radial dengan sindrom Holt-Oram diturunkan secara autosom
dominan.
III. Klasifikasi
Menurut Heikel, radial clubhand diklasifikasikan dalam 4 tipe :
a. Tipe I atau disebut “short distal radius”, yaitu di mana tulang radius
bagian distal ada, namun pertumbuhan terlambat, sedangkan bagian
proximal normal. Biasanya pada tipe ini, tulang radius hanya memendek
sedikit dan tidak ditemukan adanya tulang ulna yang melengkung.

b. Tipe II atau disebut “hypoplastic radius”, yaitu di mana tulang radius
bagian distal dan proximal ada, namun keduanya pertumbuhannya
terlambat. Biasanya pada tipe ini, tulang radius memendek sedang dan
tulang ulna menebal dan sedikit melengkung.
c. Tipe III atau disebut “partial absence of radius”, yaitu di mana tulang
radius bagian distal, medial, dan proximal menghilang, namun bagian
distal yang paling sering. Pergelangan tangan biasanya terdeviasi ke arah
radial dan tidak dapat menopang tangan. Tulang ulna menebal dan
melengkung.
d. Tipe IV atau disebut ”total absence of the radius”, merupakan jenis yang
paling sering, di mana pergelangan tangan terdeviasi ke arah radial,
telapak tangan dan jari-jari tangan bagian proksimal mengalami
subluksasi, paling sering terjadi pseudoartikulasi tulang ulna distal pada
garis radial. Tulang ulna memendek dan melengkung.

Heikel's classification of radial dysplasia. A, Type I—short distal radius. B, Type II—
hypoplastic radius. C, Type III—partial absence of radius. D, Type IV—total absence of
radius.
(Redrawn from Heikel HVA: Aplasia and hypoplasia of the radius, Acta Orthop Scand
39(suppl):1, 1959.)

Berdasarkan keparahan defisiensi tulang radius, dibagi menjadi beberapa tipe
Tipe

Ibu jari

Pergelangan

Bagian distal Bagian

tangan

os radius

proximal

os

Normal

radius
Normal

ada, Normal

Normal,

N

Hipoplasia

Normal

0

atau tidak ada
Hipoplasia

Tidak

atau tidak ada

hipoplasia, atau

radius dan ulna

menyatu

menyatu,

os
atau

dislokasi
kongenital
proximal
1

radius
ada, Lebih pendek Normal,

os

Hipoplasia

Tidak

atau tidak ada

hipoplasia, atau > 2mm dari os radius dan ulna
menyatu

ulna

menyatu,

os
atau

dislokasi
kongenital
proximal
2

3

4

ada, Hipoplasia

os

radius
Hipoplasia

Hipoplasia

Tidak

atau tidak ada

hipoplasia, atau

Hipoplasia

menyatu
Tidak

atau tidak ada

hipoplasia, atau hilang

berbagai jenis

Hipoplasia

menyatu
Tidak

Tidak ada

atau tidak ada

hipoplasia, atau

ada, Bagian

ada, Tidak ada

fisis Hipoplasia

menyatu

IV. Penatalaksanaan
Penanganan radial clubhand dibagi menjadi 2, yaitu
a. Nonoperatif
Dilakukan segera setelah lahir, dengan koreksi secara pasif. Metode yang
paling sering digunakan adalah penggunaan casting dan splinting. Splint
yang digunakan dari bahan yang ringan seperti plastik dan mudah

dibentuk. Splint dipasang sepanjang lengan tangan dan dilepas hanya saat
mandi; waktu tidur tetap dipakai. Cast dan splinting harus memenuhi tiga
daerah, yaitu telapak tangan, pergelangan tangan, dan lengan tangan.
Telapak tangan dan pergelangan tangan yang pertama kali dikoreksi,
sedangkan lengan tangan dikoreksi sebisa mungkin.

Plastic splint for congenital absence of radius. Note especially middle strap that is placed
over wrist at apex of angulation. Splint is useful for hands that can be properly aligned
passively and for maintaining proper position after surgery.

b. Operatif
Operasi dapat ditunda sampai anak usia 2-6 tahun dengan penggunaan
splint dan cast yang adekuat. Namun demikian, pada beberapa keadaan di
mana derajat defisiensi radial sangat tinggi, sehingga tidak mampu
menopang pergelangan tangan, dapat dilakukan operasi sedini mungkin
saat anak umur 3-6 bulan. Jika diperlukan dapat juga dilakukan polisisasi
saat anak berusia 9-12 bulan.
Ada beberapa kontraindikasi dilakukan operasi, yaitu anak dengan
gangguan yang berat sehingga tidak dapat bertahan hidup, fleksi genu
yang tidak adekuat, deformitas yang ringan (derajat 1 dan 2), dan pasien
yang usianya sudah tua.
Beberapa prosedur operasi yang dilakukan :
 Sentralisasi pergelangan tangan terhadap lengan tangan
Macam-macam prosedur sentralisasi:
o Prosedur Manske, McCarroll, and Swanson

Manske, McCarroll, and Swanson

•

Begin the incision just radial to the midline on the dorsum of the
wrist at the level of the distal ulna, and proceed ulnarward in a
transverse direction to a point radial to the pisiform at the volar
wrist crease. Pass the incision through the bulbous soft-tissue mass
on the ulnar side of the wrist, incising considerable fat and
subcutaneous tissue (Fig. 76-10A).

•

Identify and preserve the dorsal sensory branch of the ulnar nerve,
which is deep in the subcutaneous tissue and lies near the extensor
retinaculum.

•

Expose the extensor retinaculum and the base of the hypothenar
muscles. It is not necessary to identify the ulnar artery or nerve on
the volar aspect of the wrist (Fig. 76-10B).

•

Identify and dissect free the extensor carpi ulnaris tendon at its
insertion on the base of the fifth metacarpal, and detach and retract
it proximally.

•

Identify and retract radially the extensor digitorum communis
tendons. This exposes the dorsal and ulnar aspects of the wrist
capsule. Incise the capsule transversely, exposing the distal ulna
(Fig. 76-10C).

•

The carpal bones are a cartilaginous mass deep in the wound on the
radial side of the ulna. The carpoulnar junction is most easily
identified by dissecting from proximal to distal along the radial
side of the distal ulna. Do not mistake one of the intercarpal
articulations for the carpoulnar junction.

•

Define the cartilaginous mass of carpal bones, and excise a square
segment of its midportion (measuring approximately 1 cm) to
accommodate the distal ulna.

•

Dissect free the distal ulnar epiphysis from the adjacent soft tissue,
and square it off by shaving perpendicular to the shaft (Fig. 7610D). Avoid injury of the physis or the attached soft tissue.

•

Place the distal ulna in the carpal defect, and stabilize it with a
smooth Kirschner wire (Fig. 76-10E). In practice, this usually is
accomplished by passing the Kirschner wire proximally down the
shaft of the distal ulna to emerge at the olecranon (or at the
midshaft if the ulna is bowed). Pass the wire distally across the
carpal notch into the third metacarpal. Cut off the proximal end of
the wire beneath the skin.

•

Stabilize the ulnar side of the wrist by imbricating the capsule or by
suturing the distal capsule to the periosteum of the shaft of the
distal ulna. (If there is insufficient distal capsule, suture the
cartilaginous carpal bones to the periosteum.)

•

Obtain additional stabilization by advancing the extensor carpi
ulnaris tendon distally and reattaching it to the base of the fourth or
fifth metacarpal (Fig. 76-10F).

•

Advance the origin of the hypothenar musculature proximally, and
suture it to the ulnar shaft to provide additional stability to the
wrist.

•

Excise the bulbous excess of the skin and soft tissue, and suture the
skin. This results in a pleasing cosmetic closure and helps stabilize
the hand in the ulnar position (Fig. 76-11).

Centralization arthroplasty technique, transverse ulnar approach (see text). A,
Incision. B, Exposure of muscle, tendon, and nerve. C, Capsular incision. D,
Exposure of carpoulnar junction and excision of segment of carpal bones. E,
Insertion of Kirschner wire. F, Reattachment of extensor carpi ulnaris tendon.

(Redrawn from Manske PR, McCarroll HR Jr, Swanson K: Centralization of the
radial club hand: ulnar surgical approach, J Hand Surg 6A:423, 1981.)

o Prosedur Watson, Beebe, and Cruz
Watson, Beebe, and Cruz

•

Under pneumatic tourniquet control, make two skin incisions (Fig.
76-12A). On the radial aspect, perform a standard 60-degree Zplasty with a longitudinal central limb to obtain lengthening along
the longitudinal axis of the forearm. On the ulnar aspect, perform a
similar Z-plasty, but with a transverse central limb to take up skin
redundancy in this area, transposing the excess tissue to the
deficient radial wrist area (Fig. 76-12B).

•

When the skin incisions are completed, carry the dissection along
the radial side, identifying the median nerve (Fig. 76-12C). The
median nerve is more radially located than usual and may be the
most superficial structure encountered after the radial skin incision
is made. Identification and preservation of the “radial-median”
nerve are vital to the resulting functional capacity of the hand.

•

Continue the dissection ulnarward, resecting the fibrotic distal
radial anlage, which may act as a restricting band to maintain the
hand in radial deviation (Fig. 76-12D).

•

Identify and protect the ulnar nerve and artery through the ulnar
incision to allow complete dissection around the distal ulna without
damage to crucial structures (Fig. 76-12E).

•

Perform a complete capsular release of the ulnocarpal joint,
avoiding injury to the ulnar physis. At this point, the hand should
be fully movable, attached to the forearm only by the skin, the
dorsal and palmar tendons, and the preserved neurovascular
structures.

•

Remove all the fibrotic material in the “center” of the wrist and
forearm area. The ulna and ulnar incision should be clearly visible
through the radial incision, and the reverse should be true. It should
not be necessary to remove any carpal bones or to remodel the
distal ulna to maintain the hand in a centralized position.

•

Pass a 0.045-inch Kirschner wire through the lunate, capitate, and
long finger metacarpal, exiting through the metacarpophalangeal
joint (Fig. 76-12F).

•

Centralize the hand in the desired position, and pass the Kirschner
wire in a retrograde fashion into the ulna to maintain the position of
the hand (Fig. 76-12G).

•

Deflate the tourniquet, and obtain hemostasis before skin closure,

or deflate the tourniquet immediately after the application of the
dressing and splint.
•

Apply a bulky hand dressing with a dorsal plaster splint extending
above the elbow.

•

Before discontinuing anesthesia, ensure that circulation in the hand
is satisfactory.

Centralization of radial clubhand (see text). A, Z-plasties on radial and ulnar
sides of wrist. B, Incisions allow lengthening on radial side. Ulnar incision takes
up skin redundancy, transposing it to deficient radial side. C, Radial incision in
wrist for identification of median nerve. D, View from ulnar incision across wrist
to radial incision after resection of all nonessential central structures. E, Distal
ulna seen through radial incision at wrist. F, Kirschner wire passed through
lunate, capitate, and long finger metacarpal. G, After centralization, Kirschner
wire
passed
into
ulna
to
maintain
position.

(Redrawn from Watson HK, Beebe RD, Cruz NI: Centralization procedure for
radial clubhand, J Hand Surg 9A:541, 1984.)

o Tendon transfer
Bora et al. (Fig. 76-13)
STAGE I

•

Make a radial S-shaped incision, and excise the radiocarpal
ligament. Isolate and excise the lunate and capitate.

•

Make a longitudinal incision over the distal ulnar epiphysis, free it
from the surrounding tissue, and preserve the tendons of the
extensor carpi ulnaris and extensor digitorum quinti minimus.

•

Transpose the distal end of the ulna through the plane between the
flexor and extensor tendons and into a slot formed by the removal
of the lunate and capitate.

•

With the distal end of the ulna at the base of the long finger
metacarpal, transfix it with a smooth Kirschner wire.

•

Check the position of the ulna and carpus by radiographs in the
operating room to ensure that the ulna is aligned with the long axis
of the long finger metacarpal.

•

Suture the dorsal radiocarpal ligament over the neck of the ulna,
close the skin, and apply a long-arm cast with the elbow at 90
degrees.

•

If the deformity is unilateral, the wrist and hand should be placed in
neutral, and if it is bilateral, they should be placed in 45 degrees of
pronation on one side and 45 degrees of supination on the other.
The cast is removed at 6 weeks, and a splint is applied at night.

STAGE II

•

Three tendon transfers are performed 6 to 12 months after the
centralization procedure (see Fig. 76-13B).

•

Before attempting to transfer the flexor digitorum sublimis tendons,
test for function because in some instances the sublimis tendon is
nonfunctioning in one or more of the three ulnar digits.

•

Passively maintain the metacarpophalangeal joints and the wrist
joint in hyperextension and the interphalangeal joints in extension,
and release one finger at a time. An intact sublimis tendon flexes
the proximal interphalangeal joint of the released finger.

•

Make a midlateral incision on the ulnar side of the long finger at the
level of the proximal interphalangeal joint.

•

Divide the sublimis tendon at the level of the middle phalanx, and
divide the chiasm of the decussating fibers. Perform a similar
procedure on the ring finger.

•

Make a short transverse incision on the volar aspect of the forearm,
and pull the two tendons into it. At the site of the previous dorsal
incision, reenter the wrist, and transfer the sublimis tendons
subcutaneously around the ulnar side of the ulna to the dorsum of
the hand.

•

Loop the tendon from the long finger around the shaft of the index
finger metacarpal and the tendon from the ring finger around the
shaft of the long finger metacarpal (Fig. 76-13B).

•

Transpose the tendons extraperiosteally, and suture them back to
themselves with the wrist in 15 degrees of dorsiflexion and
maximal ulnar deviation.

•

Transfer the extensor carpi ulnaris tendon distally along the shaft of
the little finger metacarpal, and transfer the origin of the hypothenar
muscles proximally along the ulnar shaft. An effort is made to
maintain balance and prevent recurrence of the deformity.

Centralization of hand and tendon transfer (see text). A, Volar aspect of radial
clubhand deformity showing right-angle relationship of hand and forearm and
acute angulation of extrinsic flexor tendons. B, Volar aspect after centralization
and transfer of sublimis tendons of ring and long fingers.
(Redrawn from Bora FW Jr, Nicholson JT, Cheena HM: Radial meromelia, J
Bone Joint Surg 52A:966, 1970.)

Bayne and Klug

•

Make a transverse wedge incision over the end of the ulna to excise
the redundant skin and fibrofatty tissue (Fig. 76-14A). A Z-plasty
incision also may be necessary on the radial surface of the distal
forearm and wrist to give extra length to the tight skin on the radial
side and make the wrist flexors and tight capsular attachments more
accessible. If the radial contracture has been corrected before
surgery, a Z-plasty incision may not be necessary.

•

Through the ulnar incision, identify the dorsal sensory branch of the
ulnar nerve, the extensor carpi ulnaris, and the flexor carpi ulnaris.

•

Expose the distal ulna, avoiding damage to the epiphyseal blood
supply.

•

Develop a distally based ulnocarpal flap. Locate the interval
between the carpus and the radial aspect of the ulna. Using sharp
dissection, free the capsular attachments to the carpal structures,
flex the elbow, and reduce the carpus over the end of the ulna. If
this cannot be done easily, use the radial incision.

•

Elevate the skin flaps, and identify and protect the anomalous
superficial branch of the median nerve.

•

The flexor carpi radialis and frequently the brachioradialis are
attached to the radial carpal bones, producing a strong tethering
force; release these if necessary.

•

If reduction is still difficult, lightly shave the cartilage of the distal
ulna to flatten the surface, avoiding exposure of the epiphyseal
bone. Because carpal bone excision or excessive shaving often
leads to intercarpal fusion and a stiff wrist, Bayne and Klug
recommend ulnar osteotomy rather than carpal bone excision if
reduction cannot be obtained.

•

Select a Kirschner wire slightly smaller than the one to be used for
final fixation, and use it to make a pilot channel from distal to
proximal through the center of the ulna.

•

Introduce the larger Kirschner wire into the carpal bones and the
third metacarpal, crossing the metacarpophalangeal joint.

•

Place the proximal end of the wire in the pilot hole in the central
portion of the end of the ulna, and drive it retrograde proximally
through the ulna (Fig. 76-14B).

•

Withdraw the pin so that it does not block motion of the third
metacarpophalangeal joint.

•

Obtain radiographs to ensure that the carpus is perfectly centralized
on the distal ulna; failure to achieve perfect reduction is a common
cause of subsequent loss of centralization.

•

After fixation of the hand, advance the ulnocarpal flap proximally
and suture it in place.

•

Advance the extensor carpi ulnaris as far distally as possible on the
fifth metacarpal.

•

Suture the flexor carpi ulnaris into the extensor carpi ulnaris as far
distally and dorsally as possible (Fig. 76-14C). The force of the
transfer should be directed dorsally and ulnarward to counteract the
palmar- and radial-deviating structures and balance the hand
dynamically on the end of the ulna.

•

Close the incisions.

•

Place the hand in a neutral position, release the tourniquet and
evaluate circulation, and apply a bulky dressing and long arm
plaster splint.

•

If the ulna is severely bowed, a closing wedge osteotomy may be
necessary; bowing of more than 30 degrees should be corrected.
Make the osteotomy at the apex of angulation of the ulna.

Centralization of radial clubhand (see text). A, Radial release and resection of
redundant soft tissue. B, Centralization and pin fixation with ulnar osteotomy. C,
Radial
capsular
release
and
tendon
transfer.
(Redrawn from Bayne LG, Klug MS: Long-term review of the surgical treatment
of radial deficiencies, J Hand Surg 12A:169, 1987.)

Komplikasi dari sentralisasi adalah pertumbuhan os ulna bagian distal
terhenti, ankilosis pada pergelangan tangan, instabilitas berulang pada
pergelangan tangan, kerusakan saraf, insufisiensi vaskular pada

tangan, infeksi, fraktur, pergeseran pin, dan patah.
 Rekonstruksi ibu jari
o Polisisasi
Buck-Gramcko

•

Make an S-shaped incision down the radial side of the hand just onto
the palmar surface. Begin the incision near the base of the index
finger on the palmar aspect, and end it just proximal to the wrist.

•

Make a slightly curved transverse incision across the base of the
index finger on the palmar surface, connecting at right angles to the
distal end of the first incision (Fig. 76-17A).

•

Make a third incision on the dorsum of the proximal phalanx of the
index finger from the proximal interphalangeal joint, extending
proximally to end at the incision around the base of the index finger
(Fig. 76-17B).

•

Through the palmar incision, free the neurovascular bundle between
the index and long fingers by ligating the artery to the radial side of
the long finger.

•

Separate the common digital nerve carefully into its component parts
for the two adjacent fingers so that no tension would be present after
the index finger is rotated. Sometimes an anomalous neural ring is
found around the artery; split this ring carefully so that angulation of
the artery after transposition of the finger does not occur. When the
radial digital artery to the index finger is absent, it is possible to
perform the pollicization on a vascular pedicle of only one artery.

•

On the dorsal side, preserve at least one of the great veins.

•

On the dorsum of the hand, sever the tendon of the extensor
digitorum communis at the metacarpophalangeal level.

•

Detach the interosseous muscles of the index finger from the
proximal phalanx and the lateral bands of the dorsal aponeurosis.

•

Partially strip subperiosteally the origins of the interosseous muscles
from the second metacarpal, being careful to preserve the
neurovascular structures.

•

Perform an osteotomy, and resect the second metacarpal as follows.
If the phalanges of the index finger are of normal length, resect the
whole metacarpal with the exception of the base of the metacarpal,
which must be retained to obtain the proper length of the new thumb.
When the entire metacarpal is resected except for the head, rotate the
head as shown in Figure 76-17C, and attach it with sutures to the
joint capsule of the carpus and to the carpal bones, which in young
children can be pierced with a sharp needle.

•

Rotate the digit 160 degrees to allow apposition (Fig. 76-17D).

•

Bony union is not essential, and fibrous fixation of the head is
sufficient for good function.

•

When the base of the metacarpal is retained, fix the metacarpal head
to its base with one or two Kirschner wires, in the previously
described position. In attaching the metacarpal head, bring the
proximal phalanx into complete hyperextension in relation to the
metacarpal head for maximal stability of the joint. Unless this is
done, hyperextension is likely at the new “carpometacarpal” joint
(Fig. 76-17E).

•

Suture the proximal end of the detached extensor digitorum
communis tendon to the base of the former proximal phalanx (now
acting as the first metacarpal) to create the new “abductor pollicis
longus.”

•

Section the extensor indicis proprius tendon, shorten it appropriately,
and suture it by end-to-end anastomosis.

•

Suture the tendinous insertions of the two interosseous muscles to
the lateral bands of the dorsal aponeurosis by weaving the lateral
bands through the distal part of the interosseous muscle and turning
them back distally to form a loop that is sutured to itself. In this way,
the first palmar interosseous becomes an “adductor pollicis,” and the
first dorsal interosseous becomes an “abductor brevis” (Fig. 76-17F).

•

Close the wound by fashioning a dorsal skin flap to close the defect
over the proximal phalanx, and fashion the rest of the flaps as
necessary for skin closure as in Figure 76-17G and H.

Pollicization of index finger. A and B, Palmar and dorsal skin incisions. C,
Rotation of metacarpal head into flexion to prevent postoperative hyperextension.
D, Index finger rotated about 160 degrees along long axis to place finger pulp into
position of apposition. E, Final position of skeleton in about 40 degrees of palmar
abduction with metacarpal head secured to metacarpal base or carpus. F,
Reattachment of tendons to provide control of new thumb. First palmar
interosseous (PI) functions as adductor pollicis (AP); first dorsal interosseous (DI)
as abductor pollicis brevis (APB); extensor digitorum communis (EDC) as
abductor pollicis longus (APL); and extensor indicis proprius (EIP) as extensor
pollicis longus (EPL). G and H, Appearance after wound closure.
(Redrawn from Buck-Gramcko D: Pollicization of the index finger: method and
results in aplasia and hypoplasia of the thumb, J Bone Joint Surg 53A:1605,
1971.)

o Opponeplasty
Manske and McCarroll

•

Make an incision beginning over the ulnar border of the proximal
phalanx of the little finger and palm, curving radialward proximal to
the metacarpophalangeal joint, and crossing the wrist crease on the
radial side of the pisiform (Fig. 76-18A).

•

Detach the tendinous insertions into the extensor hood and the
proximal phalanx of the little finger, retaining as much tendon length

as possible (Fig. 76-18B).
•

Starting distally, dissect the abductor digiti minimi muscle out of its
fascial sheath to its origin at the pisiform, avoiding dissection on the
proximal and radial sides of the muscle where the neurovascular
structures enter.

•

Make a second incision over the dorsoradial aspect of the
metacarpophalangeal joint of the thumb, and pass the muscle
through a large subcutaneous tunnel between the thumb incision and
the proximal ulnar incision (Fig. 76-18C). Ensure that the muscle
glides freely in the tunnel and is not restricted by soft tissue.

•

The method of insertion of the transferred tendon at the
metacarpophalangeal joint (Fig. 76-19A) depends on the patient's
deformity. In patients with thenar aplasia with other radial
anomalies, suture one of the transferred slips to the soft tissue at the
radial aspect of the base of the proximal phalanx and the other to the
extensor pollicis longus muscle at the level of the
metacarpophalangeal joint as recommended by Riordan, Powers, and
Hurd (Fig. 76-19B).

•

In patients with isolated thenar aplasia, stabilize the
metacarpophalangeal joint by imbricating the ulnar capsule in a
pants-over-vest fashion (Fig. 76-19C). Suture one of the tendinous
insertions to the radial capsule and the other to the imbricated ulnar
capsule and to the extensor pollicis longus tendon (Fig. 76-19D).

•

If the opponensplasty is performed after pollicization, suture one slip
to the radial lateral band and the other to the central slip at the
proximal interphalangeal joint of the pollicized finger (Fig. 76-19E).

•

Close the incisions in routine fashion, and apply a bulky dressing
and splint, holding the thumb in opposition.

Abductor digiti minimi opponensplasty (see text). A, Incisions. B, Detachment of
tendinous insertions. C, Abductor digiti minimi passed through subcutaneous
tunnel.
(Redrawn from Manske PR, McCarroll HR Jr: Abductor digiti minimi
opponensplasty in congenital radial aplasia, J Hand Surg 3A:552, 1978.)

A, Tendon insertion at thumb metacarpophalangeal (MP) joint depends on
patient's deformity. B, Insertion in patients with thenar aplasia and other radial
anomalies. C and D, Insertion in patients with isolated thenar aplasia. E, Insertion
when
opponensplasty
follows
pollicization.
(Redrawn from Manske PR, McCarroll HR Jr: Abductor digiti minimi
opponensplasty in congenital radial aplasia, J Hand Surg 3A:552, 1978.)

 Transfer muskulus tricep untuk memperbaiki fleksi lengan tangan
Metode ini dilakukan jika anak tersebut tidak dapat melakukan aktif
fleksi lengan tangan. Hal ini dikarenakan tidak adanya muskulus
flexor elbow.
Menelaus

SOAP

•

Make a lateral incision to expose the lower end of the triceps muscle
and the anterior, lateral, and posterior aspects of the proximal end of
the ulna. Identify the triceps insertion, and dissect a tongue of
periosteum from the proximal end of the ulna in continuity with the
triceps tendon.

•

Dissect the triceps proximally to the midarm level. Identify and
mobilize the ulnar nerve; perform a posterior capsulotomy of the
elbow.

•

Roll the periosteal tongue and the triceps tendon, and pass this
through a tunnel created in the coronoid process of the ulna.

•

Secure the transfer with a nonabsorbable suture.

•

Close the wound, and apply a splint or cast with the elbow in 120
degrees of flexion

1. Subjektif : Pasien anak laki-laki usia 7 bulan datang dengan keluhan kedua
tangan bengkok. Keluhan tersebut sudah ada sejak pasien lahir. Menangis
kebiruan (-), mual muntah (-), BAB dan BAK dalam batas normal. Pasien hanya
minum ASI saja. Pasien sudah pernah berobat ke dokter spesialis anak dan
dikatakan dalam keadaan baik dan tidak ada kelainan yang lainnya.
2. Objektif : Hasil diagnosis pada kasus ini ditemukan berdasarkan





Gejala klinis
1) Kedua tangan bengkok sejak lahir
Tanda Vital
1) Nadi
: 120 x/menit
2) RR
: 30 x/menit
3) Suhu
: 36,2 ͦC
4) BB
: 6,7 kg
Pemeriksaan Fisik
1) Status Lokalis
Regio Manus Dekstra
Inspeksi : tampak angulasi ke arah radial, tampak ada crease antara
digiti I dengan pergelangan tangan, tampak atrofi muskulus thenar
Palpasi

: teraba adanya jaringan fibrosis di daerah radius distal,

muskulus thenar mengecil, manipulasi ke arah ulna side bisa namun
sedikit lebih susah dibandingkan dengan yang kiri
ROM pasif : susah dievaluasi karena pasien tidak kooperatif
Regio Manus Sinistra
Inspeksi : tampak angulasi ke arah radial, tampak ada crease antara
digiti I dengan pergelangan tangan, tampak atrofi muskulus thenar,
hipoplasia thumb
Palpasi

: teraba adanya jaringan fibrosis di daerah radius distal,

muskulus thenar mengecil, os digiti I tidak terbentuk, manipulasi ke
arah ulna side bisa lebih ringan dibandingkan yang kanan
ROM pasif : susah dievaluasi karena pasien tidak kooperatif
3. Assessment (Penalaran Klinis):
Berdasarkan penilaian sebagai berikut :
1.

Dari keluhan utama dan riwayat penyakit sekarang, yaitu
kedua tangan bengkok sejak pasien lahir.

2.

Dari pemeriksaan fisik regio manus dekstra didapatkan :
Inspeksi : tampak angulasi ke arah radial, tampak ada crease antara digiti I
dengan pergelangan tangan, tampak atrofi muskulus thenar
Palpasi

: teraba adanya jaringan fibrosis di daerah radius distal, muskulus

thenar mengecil, manipulasi ke arah ulna side bisa namun sedikit lebih
susah dibandingkan dengan yang kiri
3.

Dari pemeriksaan fisik regio manus sinistra didapatkan :
Inspeksi : tampak angulasi ke arah radial, tampak ada crease antara digiti I
dengan pergelangan tangan, tampak atrofi muskulus thenar, hipoplasia
thumb
Palpasi

: teraba adanya jaringan fibrosis di daerah radius distal, muskulus

thenar mengecil, os digiti I tidak terbentuk, manipulasi ke arah ulna side
bisa lebih ringan dibandingkan yang kanan
Dari penilaian di atas, maka dapat didiagnosis Bilateral Radial Club Hand tipe
I dengan atrofi thenar muscle dan hipoplasia thumb.
4. Plan


Diagnostik : Keluhan dan gejala klinis yang muncul serta temuan dari



pemeriksaan fisik mengarah ke radial clubhand.
Pengobatan : pada kasus ini, pengobatan dilakukan dengan tindakan
nonoperatif, yaitu dengan pemasangan cast pada kedua tangan dan
amputasi pada digiti I sinistra. Pemasangan cast bertujuan untuk



centralisasi tangan, sehingga tangan tidak bengkok ke arah radial.
Pendidikan : edukasi dilakukan pada keluarga pasien mengenai penyakit
yang diderita yaitu radial clubhand. Edukasi meliputi kemungkinan
penyebab, gejala yang timbul, komplikasi, dan yang terpenting bahwa
penyakit ini merupakan penyakit genetik. Pasien diminta untuk kontrol
rutin, untuk melihat perkembangan pertumbuhan tulang tangannya dan



tindakan selanjutnya yang harus diambil
Konsultasi : perlu dijelaskan secara rasional perlunya konsultasi dengan
spesialis bedah orthopedi, konsultasi ini merupakan upaya pemantauan
dan penanganan keadaan umum pasien selama perawatan pasien.

Pekalongan,

Pembimbing

2014

Dokter Internship

(dr. Yusuf Khairul, Sp.OT)

(dr. Aditya Agung Prasetyo)

Pendamping

(dr. Rini Handayani)