PORTOFOLIO
KASUS MEDIS
RADIAL CLUBHAND
Disusun oleh:
dr. Aditya Agung Prasetyo
DOKTER INTERNSHIP PERIODE MEI 2013 – MEI
2014
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 : RSUD Bendan – Kota Pekalongan
No .
Nama Peserta Presentasi Tanda Tangan
Berita acara ini ditulis dan disampaikan sesuai dengan yang sesungguhnya.
Pendamping I Pendamping II
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 □ Bumil □ Deskripsi :
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 No. Registrasi : 091xxx
Nama Klinik : RSUD Bendan Telp : 0285- Terdaftar: 09/02/2014
Data utama untuk bahan diskusi :
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 : disangkal - Riwayat penggunaan jamu-jamuan : disangkal
- Riwayat sakit parah : 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:
Tenggorokan : T1-T1, faring hiperemis (-)
Thoraks
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
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 :
Albee, 1928. Albee FH: Formation of radius congenitally absent: condition seven 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, 1894.
Bartlett et al., 2001. Bartlett GR, Coombs CJ, Johnstone BR: Primary shortening of the pollicized long flexor tendon in congenital pollicization. J Hand Surg 2001; 26A:595.
Bayne and Klug, 1987. Bayne LG, Klug MS: Long-term review of the surgical treatment of radial deficiencies. J Hand Surg 1987; 12A:169.
Bora et al., 1970. Bora Jr FW, Nicholson JT, Cheena HM: Radial meromelia. J Bone Joint Surg 1970; 52A:966.
Bora et al., 1981. Bora FW, Osterman AL, Kaneda RR, et al: Radial clubhand deformity: long-term follow-up. J Bone Joint Surg 1981; 63A:741. Buck-Gramcko, 1971. Buck-Gramcko D: Pollicization of the index finger:
method and results in aplasia and hypoplasia of the thumb. J Bone Joint Surg 1971; 53A:1605.
Buck-Gramcko, 1985. Buck-Gramcko D: Radialization as a new treatment for 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
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 follow-up. 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.
Sayre, 1893. Sayre RH: A contribution to the study of club-hand. Trans Am Orthop Assoc 1893; 6:208.
Smith and Greene, 1995. Smith AA, Greene TL: Preliminary soft tissue
distraction in congenital forearm deficiency. J Hand Surg 1995; 20A:420. Starr, 1945. Starr DE: Congenital absence of the radius: a method of surgical
correction. J Bone Joint Surg 1945; 27:572.
deficiency. J Pediatr Orthop 1987; 7:699.
Watson et al., 1984. Watson HK, Beebe RD, Cruz NI: Centralization procedure for radial clubhand. J Hand Surg 1984; 9A:541.
Wynne-Davies and Lamb, 1985. Wynne-Davies R, Lamb DW: Congenital upper limb anomalies. J Hand Surg 1985; 10A:958.
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 :
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.
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
tangan
Bagian distal os radius
Bagian
radius
Penanganan radial clubhand dibagi menjadi 2, yaitu a. Nonoperatif
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:
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. 76-10D). 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.
• 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, andsuture 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,
(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 Z-plasty 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).
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.
(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 itfrom 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 fingermetacarpal, 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.
• 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 tothemselves 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.
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 theulnar nerve, the extensor carpi ulnaris, and the flexor carpi ulnaris.
• Expose the distal ulna, avoiding damage to the epiphyseal bloodsupply.
• 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 anomaloussuperficial 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
• 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 thethird metacarpal, crossing the metacarpophalangeal joint.
• Place the proximal end of the wire in the pilot hole in the centralportion of the end of the ulna, and drive it retrograde proximally through the ulna (Fig. 76-14B).
• 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 proximallyand suture it in place.
• Advance the extensor carpi ulnaris as far distally as possible on thefifth 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.)
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 theindex 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 extensordigitorum 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 musclesfrom 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.
• Bony union is not essential, and fibrous fixation of the head issufficient 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.
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).
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
Abductor digiti minimi opponensplasty (see text). A, Incisions. B, Detachment of tendinous insertions. C, Abductor digiti minimi passed through subcutaneous tunnel.
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
• 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 andmobilize 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 120degrees 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 :
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
Pekalongan, 2014
Pembimbing Dokter Internship
(dr. Yusuf Khairul, Sp.OT) (dr. Aditya Agung Prasetyo)
Pendamping
