PORTOFOLIOKASUS MEDISRADIAL CLUBHAND

Disusun oleh:dr. Aditya Agung Prasetyo

DOKTER INTERNSHIP PERIODE MEI 2013 MEI 2014RSUD BENDAN KOTA PEKALONGANBERITA ACARA PRESENTASI PORTOFOLIO

Pada hari ini tanggal ........................................................... telah dipresentasikan oleh:Nama Peserta: dr. Aditya Agung PrasetyoDengan Judul/Topik: Radial ClubHandNama Pendamping I: dr. Yusuf Khairul, Sp.OTNama Pendamping II: dr. Rini Handayani Lokasi Wahana: RSUD Bendan Kota PekalonganNo.Nama Peserta PresentasiTanda Tangan

Berita acara ini ditulis dan disampaikan sesuai dengan yang sesungguhnya.

Pendamping I Pendamping II

(dr. Yusuf Khairul, Sp.OT) (dr. Rini Handayani)

Borang PortofolioNama 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 PenyegaranTinjauan 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. GNo. Registrasi : 091xxx

Nama Klinik : RSUD BendanTelp : 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 : disangkal Riwayat penggunaan jamu-jamuan: disangkal Riwayat sakit parah: disangkal2. Riwayat persalinanPasien 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 : 6,7kgc) Keadaan Tubuh Kepala : mesosefal Kulit : turgor baik, pucat (-), sianosis (-), ikterik (-) Mata : 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 Paru-paruInspeksi : statis dan dinamis simetris, retraksi (-)Palpasi : fremitus taktil kanan = kiriPerkusi : sonor/sonorAuskultasi : suara dasar vesikuler (+/+) normal, suara tambahan (-/-) JantungInspeksi : ictus cordis tidak tampakPalpasi : ictus cordis tidak terabaPerkusi : 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 sinistraAuskultasi : bunyi jantung I-II reguler, frekuensi 120 x/menit, bising (-), gallop (-) AbdomenInspeksi : permukaan cembung, dinding perut sejajar dinding dadaAuskultasi : bising usus (+) 11x/menitPerkusi : timpaniPalpasi : nyeri tekan (-), defans muskular (-), hepar & lien sulit teraba

Sistema Genitalia : ulkus (-), sekret (-), tanda-tanda radang (-). EkstremitasAkral dingin - - Oedem - - - - - Status LokalisRegio Manus Dekstra Inspeksi : tampak angulasi ke arah radial, tampak ada crease antara digiti I dengan pergelangan tangan, tampak atrofi muskulus thenarPalpasi : teraba adanya jaringan fibrosis di daerah radius distal, muskulus thenar mengecil, manipulasi ke arah ulna side bisa namun sedikit lebih susah dibandingkan dengan yang kiriROM 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 thumbPalpasi : 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 LaboratoriumPemeriksaan Laboratorium tanggal 9 Februari 2014Hemoglobin :10,1Golongan Darah : O+Hematokrit : 30PT : 11,6AL : 7,483.103APTT : 36,8AT : 228.103INR : 0,91AE : 3,72.106

Daftar Pustaka :

Albee, 1928. AlbeeFH: 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 Hftgelenkes ausgefhrt worden war, Berich ber d Verhandl d Deutsche Gesellschaft f Chir, Leipzig, 23 Kongr, 105, 1894. Bartlett et al., 2001. BartlettGR,CoombsCJ,JohnstoneBR: Primary shortening of the pollicized long flexor tendon in congenital pollicization. J Hand Surg 2001;26A:595. Bayne and Klug, 1987. BayneLG,KlugMS: Long-term review of the surgical treatment of radial deficiencies. J Hand Surg 1987;12A:169. Behrens and Sabharwal, 2000. BehrensF,SabharwalS: Deformity correction and reconstructive procedures using percutaneous techniques. Clin Orthop Relat Res 2000;375:113. Bora et al., 1970. BoraJrFW,NicholsonJT,CheenaHM: Radial meromelia. J Bone Joint Surg 1970;52A:966. Bora et al., 1981. BoraFW,OstermanAL,KanedaRR,et al: Radial clubhand deformity: long-term follow-up. J Bone Joint Surg 1981;63A:741. Buck-Gramcko, 1971. Buck-GramckoD: 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-GramckoD: Radialization as a new treatment for radial club hand. J Hand Surg 1985;10A:964. Catagni et al., 1993. CatagniM,SzaboRM,CattaneoR: Preliminary experience with Ilizarov method in late reconstruction of radial hemimelia. J Hand Surg 1993;18A:316. Damore et al., 2000. DamoreE,KozinSH,ThoderJJ,et al: The recurrence of deformity after surgical centralization for radial clubhand. J Hand Surg 2000;25A:745. DeLorme, 1969. DeLormeTL: Treatment of congenital absence of radius by transepiphyseal fixation. J Bone Joint Surg 1969;51A:117. Goldfarb et al., 2002. GoldfarbCA,KleppsSJ,DaileyLA,et al: Functional outcome after centralization for radius dysplasia. J Hand Surg 2002;27A:118. Gossett, 1949. GossettJ: La pollicisation de l'index. J Chir 1949;65:403. Heikel, 1959. HeikelHVA: Aplasia and hypoplasia of the radius. Acta Orthop Scand Suppl 1959;39:1. James et al., 2004. JamesMA,GreenHD,McCarrollJrHR,et al: The association of radial deficiency with thumb hypoplasia. J Bone Joint Surg 2004;86A:2196. James et al., 1999. JamesMA,McCarrollR,ManskePR: The spectrum of radial longitudinal deficiency: a modified classification. J Hand Surg 1999;24A:1145. Kato, 1924. KatoK: 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. KawabataH,MasatomiT,YasuiN: 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. KesslerI: Centralization of the radial club hand by gradual distraction. J Hand Surg 1989;14B:37. Kummel, 1895. KummelW: Die Missbildungen der Extremitten durch Defekt, Verwachsung und Ueberzahl, Kassel, Germany,Bibliotheca Medica,1895. heft 3 Lamb, 1972. LambDW: 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. LambDW: 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. LambDW,Wynne-DaviesR,SotoL: An estimate of the population frequency of congenital malformations of the upper limb. J Hand Surg 1982;7A:557. Lidge, 1969. LidgeRT: Congenital radial deficient club hand. J Bone Joint Surg 1969;51A:1041. Manske and McCarroll, 1978. ManskePR,McCarrollJrHR: Abductor digiti minimi opponensplasty in congenital radial aplasia. J Hand Surg 1978;3A:552. Manske et al., 1981. ManskePR,McCarrollJrHR,SwansonK: Centralization of the radial club hand: ulnar surgical approach. J Hand Surg 1981;6A:423. Menelaus, 1976. MenelausMB: 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. MilfordL: The hand: congenital anomalies. In:CrenshawAH,ed. Campbell's operative orthopaedics, 7th ed.St Louis:Mosby;1987. Mo and Manske, 2004. MoJH,ManskePR: Surgical treatment of type 0 radial longitudinal deficiency. J Hand Surg 2004;29A:1002. Nanchahal and Tonkin, 1996. NanchahalJ,TonkinA: Preoperative distraction lengthening for radial longitudinal deficiency. J Hand Surg 1996;21A:103. Niall et al., 1998. NiallDM,MurphyPG,FogartyEE,et al: Correction of soft tissue contractures of the wrist using the Ilizarov technique. J Hand Surg 1998;23A:442. Prokopovich, 1980. ProkopovichVS: Aligning of length of the forearm bones in the congenital club-hand in children. Ortop Travmatol Protez 1980;1:51. Riordan, 1955. RiordanDC: Congenital absence of the radius. J Bone Joint Surg 1955;37A:1129. Riordan, 1963. RiordanDC: 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. SabharwalS: Treatment of acquired radial club hand deformity with Ilizarov apparatus. Clin Orthop Relat Res 2004;424:143. Sabharwal et al., 2005. SabharwalS,FinuoliAL,GhobadiF: Pre-centralization soft tissue distraction of Bayne type IV congenital radial deficiency in children. J Pediatr Orthop 2005;25:377. Sayre, 1893. SayreRH: A contribution to the study of club-hand. Trans Am Orthop Assoc 1893;6:208. Smith and Greene, 1995. SmithAA,GreeneTL: Preliminary soft tissue distraction in congenital forearm deficiency. J Hand Surg 1995;20A:420. Starr, 1945. StarrDE: Congenital absence of the radius: a method of surgical correction. J Bone Joint Surg 1945;27:572. Tsuyuguchi et al., 1987. TsuyuguchiY,YukiokaM,KawabataY,et al: Radial ray deficiency. J Pediatr Orthop 1987;7:699. Watson et al., 1984. WatsonHK,BeebeRD,CruzNI: Centralization procedure for radial clubhand. J Hand Surg 1984;9A:541. Wynne-Davies and Lamb, 1985. Wynne-DaviesR,LambDW: 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. EtiologiPenyebab 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. KlasifikasiMenurut 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 Ishort distal radius. B, Type IIhypoplastic radius. C, Type IIIpartial absence of radius. D, Type IVtotal 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 tipeTipeIbu jariPergelangan tanganBagian distal os radiusBagian proximal os radius

NHipoplasia atau tidak adaNormalNormalNormal

0Hipoplasia atau tidak adaTidak ada, hipoplasia, atau menyatuNormalNormal, os radius dan ulna menyatu, atau dislokasi kongenital proximal os radius

1Hipoplasia atau tidak adaTidak ada, hipoplasia, atau menyatuLebih pendek > 2mm dari os ulnaNormal, os radius dan ulna menyatu, atau dislokasi kongenital proximal os radius

2Hipoplasia atau tidak adaTidak ada, hipoplasia, atau menyatuHipoplasiaHipoplasia

3Hipoplasia atau tidak adaTidak ada, hipoplasia, atau menyatuBagian fisis hilangHipoplasia berbagai jenis

4Hipoplasia atau tidak adaTidak ada, hipoplasia, atau menyatuTidak adaTidak ada

IV. PenatalaksanaanPenanganan radial clubhand dibagi menjadi 2, yaitua. NonoperatifDilakukan 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. OperatifOperasi 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 tanganMacam-macam prosedur sentralisasi: Prosedur Manske, McCarroll, and SwansonManske, 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.

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.)

Prosedur Watson, Beebe, and CruzWatson, 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).

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.)

Tendon transferBora 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 PolisisasiBuck-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.)

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 tanganMetode 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 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

S O A P

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 klinis1) Kedua tangan bengkok sejak lahir Tanda Vital1) Nadi : 120 x/menit2) RR : 30 x/menit3) Suhu : 36,2 C4) BB : 6,7 kg Pemeriksaan Fisik1) Status LokalisRegio Manus DekstraInspeksi : tampak angulasi ke arah radial, tampak ada crease antara digiti I dengan pergelangan tangan, tampak atrofi muskulus thenarPalpasi : teraba adanya jaringan fibrosis di daerah radius distal, muskulus thenar mengecil, manipulasi ke arah ulna side bisa namun sedikit lebih susah dibandingkan dengan yang kiriROM pasif : susah dievaluasi karena pasien tidak kooperatifRegio Manus SinistraInspeksi : tampak angulasi ke arah radial, tampak ada crease antara digiti I dengan pergelangan tangan, tampak atrofi muskulus thenar, hipoplasia thumbPalpasi : 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 thenarPalpasi : teraba adanya jaringan fibrosis di daerah radius distal, muskulus thenar mengecil, manipulasi ke arah ulna side bisa namun sedikit lebih susah dibandingkan dengan yang kiri3. 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 thumbPalpasi : 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, 2014

Pembimbing Dokter Internship

(dr. Yusuf Khairul, Sp.OT) (dr. Aditya Agung Prasetyo)

Pendamping

(dr. Rini Handayani)