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1. Bone Cyst

BONE CYSTS

Oleh Dr H Subagyo SpB - SpOT

 

I.        PENDAHULUAN

Tumor primer tulang merupakan kelainan yang agak jarang ditemukan. Penderita  dengan tumor tulang umumnya datang karena pembengkakan, nyeri, atau fracture patologis. Pada diagnosis awal sering disangka cedera olahraga karena sering didapat riwayat trauma lokal pada neoplasma muskuloskeletal. Pertumbuhan neoplasma yang jinak jarang menimbulkan gejala. Nyeri biasanya timbul karena tekanan pada periosteum dan endosteum yang sensitif. Nyeri hebat dan tiba – tiba biasanya disebabkan fracture patologis.

Sel neoplasma tidak selalu merusak tulang, tetapi keberadaannya menyebabkan resorpsi tulang atau osteoklastik lokal. Sebaliknya, sel neoplasma tertentu dapat bersifat osteoblastik lokal dan membentuk tulang normal yang disebut kelainan tulang reaktif. Sel neoplasma kelompok osteogenic adalah sel yang dapat menghasilkan osteoid dan tulang, sedangkan sel kelompok osteolytic adalah sel yang meresorpsi tulang yang tampak pada gambaran radiologis sebagai osteolisis atau osteosclerosis.

Klasifikasi tumor tulang :

- Neoplasm – like Lesions of Bone

  • Osteogenic
    • Osteoma (Ivory exostosis)
    • Single osteochondroma (Osteocartilaginous exostosis)
    • Multiple osteochondromata (multiple hereditary exostoses)
    • Osteoid osteoma
    • Benign osteoblastoma (Giant osteoid osteoma)
    • Chodrogenic
      • Enchondroma
      • Enchondromata multiple (Ollier’s dyschondroplasia)
    • Fibrogenic
      • Subperiosteal cortical defect (Metaphyseal Fibrous Defect)
      • Non-osteogenic fibroma (non-ossifying fibroma)
      • Monostotic fibrous dysplasia
      • Polyostotic fibrous dysplasia
      • Osteofibrous dysplasia (Campanacci syndrome)
      • “Brown tumor” (hyperparathyroidism)
    • Angiogenic
      • Angioma tulang (hemangioma dan limfangioma)
      • Aneurysmal bone cyst (ABC)
    • Uncertain Origin
      • Simple bone cyst (Unicameral Bone Cysts / UBC)

 

- True Primary Neoplasms of Bone

  • Osteogenic
    • Osteosarcoma (sarcoma osteogenic)
    • Surface osteosarcoma (parosteal sarcoma; periosteal sarcoma)
    • Chondrogenic
      • Benign chondroblastoma
      • Chondromyxoid fibroma
      • Chodrosarcoma
    • Fibrogenic
      • Fibrosarcoma tulang
      • Fibrous histiositoma maligna tulang
    • Angiogenic
      • Angiosarcoma tulang
    • Myelogenic
      • Mieloma tulang (Multiple myeloma)
      • Ewing’s sarcoma
      • Hodgkin’s lymphoma of bone
      • Non – Hodgkin’s lymphoma (reticulum cell sarcoma)
      • Retikulose skeletal (Langerhans’s cell histiositosis)
      • Leukemia
    • Uncertain Origin
      • Giant cell tumor (osteoclastoma)

 

Pembagian stadium pada neoplasma jinak muskuloskeletal dideskripsikan oleh Enneking tahun 1986, yang mengklasifikasikan lesi jinak sebagai laten, akitf, atau agresif. Bagian dari klasifikasi Enneking ini mencakup klasifikasi Lodwick radiographic grading system.

Lodwick radiographic grading with bone destructioN

  • Lodwick IA
    • Destruksi area sekitar
    • Sudut multisentrik, regular, terlobulasi.
    • Tidak ada atau parsial penetrasi korteks
    • batas sklerotik
    • Ekspansi kurang dari 1 cm.

 

  • Lodwick IB
    • Destruksi tulang sekitar
    • Batas yang sulit ditentukan, lobulasi, multisentrik.
    • Tidak ada atau penetrasi korteks parsial
    • Tepi sklerotik lebih dari 1 cm.
    • Lodwick IC
      • Destruksi area sekitar
      • Karakteristik tepi regular, lobulasi, multisentrik, batas tidak dapat ditentukan.
      • Penetrasi total korteks
      • Tepi sklerotik
      • Expanded shell
      • Lodwick II
        • Destruksi geofrafik atau tepi ”moth eaten” lebih besar dari 1 cm.
        • Penetrasi korteks total
        • Tepi sklerotik
        • Expanded shell”
        • Lodwick III
          • Permeated destruction
          • Penetrasi total korteks
          • Tepi sklerotik
          • Expanded shell

 

II.        ANATOMI – FISIOLOGI TULANG  : tidak dibibacakan disini

 

III.       LESI KISTIK TULANG

 

III.1. UNICAMERAL BONE CYST

III.1.1. Sejarah dan Definisi [36], [42]

Memiliki nama lain yaitu solitary bone cysts atau simple bone cysts. UBC bukan merupakan suatu keadaan patologis yang baru. Virchow menemukan lesi kistik tulang ini pada akhir 1870. Pada 1942, Henry Jaffe dan Louis Lichenstein mempublikasikan literatur mengenai unicameral bone cysts :

"Solitary unicameral bone cyst is a lesion sui generis. It bears no relation whatever to giant cell tumor of bone, and in particular it does not represent a cystic-healing phase of this tumor. Nor is it to be linked with enchondroma, fibroma or focus of fibrous dysplasia of bone that has undergone partial or extensive cystic degeneration. Further, it should not be regarded as representing cystic expression of osteitis fibrosa, since to throw it into this wastebasket category (one which to us is also meaningless) is to obliterate its distinctiveness. Correspondingly, solitary unicameral bone cyst ought no longer to be classed as an expression of localized fibrocystic disease of bone or localized fibrous osteodystrophy—likewise blanket designations dating from a more primitive era of bone pathology.”

Unicameral Bone Cyst merupakan lesi jinak, terisi cairan, bersifat radiolusen, dan hampir selalu ditemukan pada anak – anak. UBC dapat menyebabkan penipisan tulang di sekitarnya, sehingga mudah terjadi fracture atau mikrofaktur. Bila kista terdapat pada lempeng pertumbuhan, disebut sebagai active cyst, sedangkan bila memiliki jarak dengan lempeng pertumbuhan disebut sebagai latent cyst. Pembagian ini digunakan pada waktu lampau untuk membedakan prognosis. Unicameral bone cyst biasanya unifokal dan terjadi pada penderita dengan tulang imatur.

Jarangnya lesi ditemukan pada orang dewasa mendukung hipotesis mengenai resolusi spontan. Bila tidak terdapat fracture, UBC seringkali asimtomatik. Biasanya lesi ditemukan karena pemeriksaan radiologi yang dilakukan untuk alasan lain. Pada keadaan asimtomatik dan tidak adanya keadaaan medis yang perlu dipertimbangkan, tidak ada terapi yang perlu diberikan, hanya dilakukan observasi.

Terapi harus diberikan untuk lesi yang telah menyebabkan fracture atau menurunnya kekuatan tulang. Terdapat beberapa bukti yang menunjukkan penyembuhan UBC setelah fracture, meskipun kasus tersebut sangat jarang. Gangguan pertumbuhan sebagai akibat UBC juga perlu dipertimbangkan.

III.1.2. Frekuensi

UBC terjadi terutama pada usia 5 – 15 tahun, dengan usia rata – rata 9 tahun. Beberapa peneliti mengemukakan bahwa lesi yang muncul pada usia dekade pertama bersifat lebih agresif. Presentasi kejadian pada pria 2 kali pada wanita. Lesi ini menempati 3% dari seluruh tumor pada tulang. Pada tumor jinak tulang, UBC menempati urutan ke-3 atau ke-4 pada anak setelah osteochondroma dan fibroma. Lesi ini dapat terjadi bersamaan dengan tumor jinak lain, seperti fibroma non-osifikasi.

Predileksi tersering adalah humerus proksimal, diikuti femur proksimal. Gabungan keduanya telah mencakup 90% dari seluruh unicameral bone cyst. Meskipun begitu semua jenis tulang dapat terserang. Predileksi tergantung pada usia penderita. Pada pasien berusia kurang dari 20 tahun, simple bone cysts ditemukan 55 – 65% pada humerus, 25 – 30% pada femur, dan jarang ditemukan pada tibia, fibula, radius, dan ulna. Pada penderita berusia lebih dari 20 tahun, simple bone cysts banyak ditemukan pada tulang pipih seperti iliaka dan kalkaneus. Jenis kista ini jarang ditemukan pada lebih dari satu lokasi dari seorang penderita. Pasien dengan simple bone cysts multipel biasanya berada pada kelompok usia lebih tinggi dan lebih dominan pada laki – laki.

III.1.3. ETIOLOGI DAN PATOFISIOLOGI [42]

Etiologi spesifik dari UBC belum diketahui dengan pasti. Berbagai teori telah dikemukakan, salah satunya oleh Cohen pada tahun 1960. Ia mempelajari cairan kista dari 6 anak yang sedang menjalani terapi dan menemukan 4 dari kista tersebut berisi cairan plasma, dan 2 darah. Cohen mengemukakan bahwa prinsip utama terjadinya lesi adalah karena blokade dari drainase cairan interstisial pada wilayah tulang yang mengalami pertumbuhan dan remodelling cepat.

Chigira dan sekelompok peneliti Jepang mempelajari tekanan internal dari 7 pasien dengan UBC dan menemukan bahwa tekanan meningkat (2-7 mmHg) dibandingkan dengan tekanan normal sumsum tulang kontralateral. Tekanan parsial O2 (PaO2) pada cairan kista juga lebih rendah dari sampel arteri dan vena yang diambil pada saat yang sama. Peneliti menyimpulkan bahwa obstruksi vena dalam tulang merupakan penyebab terjadinya UBC.

Teori vaskular yang lain juga didukung oleh Mirra et al yang mengemukakan bahwa UBC merepresentasikan area dari sisa jaringan sinovial kongenital dan mendukung teori ini dengan mendemonstrasikan baik sinovial tipe A (macrophagelike) dan tipe B (fibroblastlike) pada perbatasan kista. Shindel et al menemukan peningkatan prostaglandin E2 dalam cairan kista dari 7 pasien dan  mengemukakan teori bahwa hal ini dapat menjelaskan efek positif penyuntikan steroid pada lesi.

Kadar tinggi dari oksigen sitotoksik radikal bebas juga ditemukan pada cairan kista pada UBC.

Patogenesis dari terjadinya simple bone cysts masih belum diketahui. Yang sudah terbukti adalah obstruksi pada aliran vena dan blokade dari drainase cairan interstisial pada area tulang yang cepat mengalami pertumbuhan dan remodelling. Disamping pembuktian ini, patogenesis lain dari simple bone cysts belum diketahui dengan pasti.

Pada pemeriksaan secara umum, kista mengekspansi korteks tulang. Periosteum yang intak terbungkus dengan lapisan kortikal tipis. Kista biasanya berisi cairan serous jernih. Pada beberapa kasus, produk darah dapat ditemukan dalam cairan bila sebelumnya telah didahului oleh fracture. Membran dengan ketebalan yang berbeda membatasi dinding dalam dari kista tersebut. Septum fibrosa dapat terbentuk bila sebelumnya terdapat fracture, sehingga menghasilkan gambaran kista yang multilokular.

Secara histologis, sel – sel mesotel membatasi simple bone cysts. Dinding dalam tulang melekat pada membran mesotel - yang mengandung tulang yang baru terbentuk dan kaya vaskularisasi - melalui periosteum. Sel – sel raksasa multinuklear terkadang dapat ditemukan dalam dinding kista.

III.1.4. GEJALA KLINIS

Kebanyakan pasien dengan unicameral bone cysts datang ke klinik setelah mengalami fracture patologis. Unicameral bone cysts sering tidak menunjukkan gejala kecuali bila terdapat fracture sebagai komplikasi. Fracture yang terjadi banyak melibatkan humerus proksimal atau femur proksimal. Peristiwa yang mengakibatkan fracture bervariasi dari hal – hal ringan seperti melempar bola hingga peristiwa besar seperti jatuh dengan keras. Seperti pasien dengan fracture lainnya, pemeriksaan fisik harus mengeksklusi kemungkinan terdapatnya open fracture dan kerusakan neurosirkulatoris.

10% penderita mengalami gangguan pertumbuhan. Dapat disebabkan oleh kuretase kista yang mengganggu epifisis, atau ekstensi langsung dari kista ke wilayah epifisis. Penyebab terakhir ini dapat ditemukan pada pemeriksaan penunjang. Kemungkinan terdapatnya lesi perlu dipikirkan bila terdapat nyeri atau hambatan pergerakan anggota gerak yang menyertai suatu trauma sederhana. UBC akan membesar seiring pertumbuhan tulang dan menjadi inaktif atau laten setelah tulang matur.

III.1.5. ANATOMI

UBC ditemukan pada tulang panjang pada 90 – 95% pasien. Kebanyakan berlokasi pada metafisis proksimal. Simple bone cysts yang berlokasi pada diafisis hanya terdapat pada 4 – 12% pasien. Jarang ditemukan keterlibatan dari epifisis. Pasien dengan keterlibatan pada epifisis ini biasanya berusia lebih tua dibandingkan penderita tanpa keterlibatan epifisis. Simple bone cysts epifiseal ini memiliki rasio kejadian pada pria – wanita yang lebih rendah. Usia rata – rata penderita adalah 21 tahun, dimana epiphyseal plate telah tertutup pada 50% pasien. Sifat lesi ini lebih tidak agresif dengan prognosis yang lebih baik bila dibandingkan dengan simple bone cysts yang terdapat pada regio metafisis.

III.1.6. PEMERIKSAAN PENUNJANG

Foto polos radiologi merupakan jenis pemeriksaan terpilih karena memiliki kapabilitas diagnostic yang tinggi untuk mendiagnosa simple bone cysts. CT dan MRI biasanya tidak diperlukan dan sebaiknya hanya dilakukan untuk melakukan evaluasi pada area anatomi yang kompleks seperti vertebra dan pelvis. Area ini kadang - kadang sulit untuk dievaluasi secara akurat dengan foto polos. Penggunaan CT dan MRI bertujuan untuk mengetahui ekstensi lesi dan komplikasi seperti fracture.

III.1.6.a. Gambaran Radiologis [37]

Gambaran radiologi akan menunjukkan simple bone cysts sebagai lesi yang berbatas tegas dengan zona peralihan yang sempit. Batas sklerotik tipis merupakan temuan yang tipikal. Aksis memanjang dari lesi akan berjalan paralel dengan aksis memanjang dari tulang panjang. Simple bone cysts dapat melakukan ekspansi yang menyebabkan penipisan korteks tulang. Pada beberapa kasus dapat ditemukan gambaran multilokular. Pada tulang panjang simple bone cysts secara tipikal terletak dalam rongga medulla.

 

                                                   

Anak laki – laki berusia 7 tahun dengan simple bone cysts pada regio metafisis superior humerus dextra. Kista memiliki gambaran multilokular   Anak laki – laki berusia 9 tahun dengan simple bone cyst pada tibia superior. Foto lateral memperlihatkan kista yang berlokasi di senter medulla.

 Fracture patologis merupakan kejadian yang lazim pada penderita unicameral bone cysts. Hal ini dapat memberikan gambaran ”fallen fragment”, yaitu migrasi dari fragmen tulang ke posisi tepi dari kista yang berisi cairan. Meskipun gambaran ini hanya ditemukan pada sebagian kecil pasien, namun bila ditemukan gambaran ini merupakan ciri penting yang membedakan simple bone cyst dan lesi tulang non – litik lainnya. Bila terlihat, fallen fragment ini merupakan tanda patognomonik dari simple bone cysts.

                  

 

 

 

 

 

 

 

 

 

Anak laki – laki berusia 11 tahun dengan simple bone cysts pada diametafisis pada humerus proksimal. Pada lesi terdapat fraktur patologis. Fragmen fraktur ditunjukkan oleh panah. Dikenal sebagai fallen fragment.

Meskipun pemeriksaan radiologis cukup untuk menegakkan diagnosis dari simple bone cysts, kadang – kadang terdapat kesulitan untuk menegakkan diagnosa bila terdapat enchondroma atau fibrous dysplasia pada regio metafisis dari tulang panjang pada pasien dalam usia 2 dekade pertama.

III.1.6.b. Gambaran CT Scan

CT – Scan bukan merupakan pemeriksaan yang penting pada simple bone cysts karena sudah cukup tingginya akurasi diagnosa dengan foto polos. Pada kasus tertentu CT Scan digunakan untuk mengevaluasi atau observasi lesi pada area yang sulit diakses dengan pemeriksaan foto polos, seperti vertebra dan pelvis. Selain untuk mengetahui ekspansi lesi, CT Scan juga dapat mendeteksi adanya komplikasi yang sulit dideteksi dengan pemeriksaan radiologis biasa. Gambaran simple bone cysts yang terdapat pada pemerisaan radiografis juga terdapat pada CT Scan. Dapat terlihat udara atau air – fluid level. CT Scan dapat membantu mendiferensiasi simple bone cysts yang berisi cairan dan avaskular dengan lesi tulang solid yang memperlihatkan gambaran berbagai derajat vaskularisasi.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Anak perempuan 8 tahun dengan simple bone cysts pada metafisis proksimal femur dextra.

Pasien yang sama dengan gambar sebelumnya gambaran CT simple bone cysts pada region metafisis proksimal femur dextra. Kista terletak pada rongga medulla femur dengan ekspansi dan penipisan korteks.

Anak perempuan berusia 7 tahun dengan simple bone cysts pada tibia dextra proksimal. CT menunjukkan simple bone cysts dalam rongga medulla. Terdapat penipisan korteks pada anterolateral tulang (panah). Penebalan dan sclerosis terdapat pada bagaian lain  dari korteks (kepala panah)..

Terdapatnya air – fluid level dalam lesi skeleteal tidak merupakan diagnosa pasti atau mencirikan jenis tumor tertentu. Tanda ini dapat terlihat juga pada fibrous dysplasia, simple bone cysts, histiositoma fibrosa maligna rekuren, osteosarcoma, dan aneurysmal bone cysts.

III.1.6.c. Gambaran MRI

MRI dapat menunjukkan keberadaan cairan dalam simple bone cysts. Simple bone cysts tanpa komplikasi memiliki intensitas T1 yang rendah dan intensitas T2 tinggi. Lesi dengan fracture patologis memiliki sinyal intensitas baik T1 maupun T2 yang heterogen, karena terdapatnya perdarahan dalam kista. Septasi dalam lesi dapat diobservasi dengan MRI dan dapat tidak terlihat dengan pemeriksaan radiologi biasa.

MRI juga digunakan untuk mengevaluasi efektivitas injeksi steroid intrakavital ke dalam kista. MRI akan memperlihatkan lapisan tipis jaringan reparatif yang mengelilingi dinding kista. Jaringan reparatif ini makin lama akan menebal dan terjadi pembentukan tulang baru. Rongga kista residual juga dapat terlihat.

Anak perempuan berusia 10 tahun dengan simple bone cysts pada femur sinistra proksimal. Sinyal T1 memiliki intensitas rendah dan terletak pada region metefisis.

 

 

 

 

Pasien yang sama dengan gambar sebelumnya Sinyal T2 pada tampilan koronal dengan saturasi lemak menunjukkan kista dengan intensitas tinggi.

 

 

 

Pasien yang sama dengan 2 gambar sebelumnya – post-gadolinium T1-weighted potongan koronal dengan saturasi lemak menunjukkan penegasan gambaran perifer dari kista.

 

 

 

 

III.1.7. TERAPI [38]

Tujuan terapi pada simple bone cysts adalah mencegah fracture patologis, mengusahakan penyembuhan lesi dan menghindari rekurensi lesi atau fracture. Terapi dapat dilakukan dengan kuretase dan bone graft, krioterapi, intramedullary nailing, injeksi metilprednisolon dibawah panduan image intensifier, injeksi sumsum tulang, atau kombinasi dari metode – metode di atas.

 

III.1.7.a. Terapi farmakologi

Terapi non-operatif dari unicameral bone cysts biasanya dilakukan pada perawatan fracture tertutup. Seperti telah disebutkan, pada 25% kasus penyembuhan spontan dapat terjadi setelah fracture. Meskipun begitu, hanya melakukan observasi ketat dan perawatan rutin terhadap fracture bukan merupakan pilihan terapi.

III.1.7.b. Terapi Operatif

Terapi operatif pada UBC dapat dibagi menjadi prosedur terbuka dan prosedur perkutaneus. Penelitian oleh Spence pada 144 kasus unicameral bone cysts yang diterapi dengan kuretase, alograf tulang, menunjukkan tingkat rekurensi yang lebih tinggi pada pasien kurang dari 10 tahun, pasien wanita dan pasien dengan kista aktif. Neer mengemukakan teori bahwa rekurensi pasca bedah UBC dapat samasekali tidak diduga atau terlewatkan bila indikator keberhasilan adalah obliterasi total kista. Menurut teori tersebut rekurensi merupakan rongga kista yang kembali muncul dan membesar. Neer rating system untuk evaluasi keberhasilan terapi :

  • Excellent – obliterasi lengkap kista.
  • Defek residual – satu atau dua area seperti kista dengan pengembalian kekuatan tulang yang baik.
  • Reoperasi – operasi ulang perlu dilakukan karena rekurensi.

Teknik operasi terbuka telah dilaporkan meliputi reseksi subtotal dengan atau tanpa bone grafting. Materi bone graft yang digunakan adalah autograf, alograf, matriks tulang demineralisasi, high-porosity hydroxyapatite bone grafting material, dan plaster-of-paris pellets.

Subtotal resection with and without grafting

Pada tahun 1962  Fahey dan O’Brien mengenalkan teknik terapi UBC yaitu reseksi subtotal dan graft. Teknik yang dideskripsikan adalah eksposur subperiosteal ke kista dan sebagian dari jaringan tulang normal, diikuti dengan pengangkatan 2/3 atau 4/5 bagian kista. Bone graft berasal dari krista iliaka atau tibia pasien yang kemudian digunakan untuk mengisi defek.

Setelah 4 tahun follow up, Fahey dan O’Brien melaporkan 95% tingkat keberhasilan dengan teknik ini. Dikemukakan bahwa teknik ini merupakan pilihan terapi pada individu dengan lesi primer laten atau telah mengalami rekurensi setelah operasi konvensional.

15 tahun kemudian McKay dan Nason melaporkan metode reseksi subtotal yang sama  untuk terapi UBC, tetapi tanpa bone graft. Teknik ini memiliki keberhasilan 90% ( didefinisikan sebagai obliterasi kista total ). Tidak ada komplikasi maupun neurosirkulatorik yang dilaporkan, tetapi 3 pasien mengalami gangguan pertumbuhan. Hingga kini reseksi subtotal dan bone graft merupakan pilihan terapi untuk unicameral bone cyst, dengan pertimbangan jarak lesi dari lempeng pertumbuhan.

Percutaneous treatment techniques

Pada tahun 1974 Scaglietti mengenalkan prosedur dimana steroid secara perkutaneus disuntikkan ke dalam lesi. Minimal 40 mg metilprednisolon asetat digunakan untuk kista kecil pada pasien yang lebih muda, dan dosis lebih dari 200 mg digunakan untuk kista yang lebih besar pada usia yang lebih tua. Injeksi diberikan 3 – 4x dalam 12 – 20 bulan. Didapatkan hasil 55% kasus yang sembuh dan 45% mengalami perbaikan seperti penebalan korteks, atau pembentukan tulang baru dalam area kista. 24% pasien hanya memerlukan 1x injeksi, sementara sisanya memerlukan injeksi steroid multipel.

Cairan kista yang diambil dari kista – tampilan tipikal cairan yang diaspirasi dari lesi.

 

 

 

Berbagai penelitian lain mengungkapkan hasil dan mengusulkan penggunaan teknik steroid perkutan. Rosenberg menekankan pentingnya mengeliminasi septum fibrosa atau oseus dalam UBC untuk memfasilitasi perataan injeksi steroid pada lesi. Selain injeksi dengan steroid, bahan lain seperti alcohol-based fibrosing agents, juga dapat digunakan sebagai agen terapi.

Jenis – jenis terapi lain :

  • demineralized bone matrix melalui jalur perkutaneus , tanpa steroid.
  • Injeksi autolog sumsum tulang
    • Punksi ke dalam kista dengan trokar atau jarum kecil.
    • Aspirasi cairan kista à pemeriksaan patologi klinik
    • Menghancurkan batas atau septum dalam kista dengan trokar  atau jarum.
    • Injeksi autolog sumsum tulang yang diambil yang diaspirasi dari krista iliaka sebanyak 25 cc.

III.1.7.c. Kontraindikasi

Kontraindikasi utama untuk terapi operatif unicameral bone cysts adalah pasien yang tidak dapat mentoleransi obat – obat anestesi. Kontraindikasi relatif adalah bila terdapat kista laten asimtomatik atau kecenderungan untuk terjadi fracture patologis.

III.1.7.d. Komplikasi

Cedera pada lempeng pertumbuhan dapat terjadi sekunder akibat ekspansi langsung, fracture patologis, atau cedera mekanis saat intervensi bedah dilakukan. Gangguan pertumbuhan masih tetap dilaporkan setelah terapi baik dengan injeksi steroid lokal, kuretase, dan bone graft. Gangguan pertumbuhan menyebabkan deformitas dan terganggunya pertumbuhan longitudinal, terjadi pada 14% kasus.

III.1.8. PROGNOSIS

Secara keseluruhan prognosis unicameral bone cysts baik. Kebanyakan lesi sembuh spontan dengan waktu yang cukup. Kasus yang terdapat di poli bedah merupakan pasien dimana sudah terjadi penipisan korteks, dan sudah terdapat keluhan seperti patah tulang hanya karena kecelakaan kecil. Secara umum, terapi untuk UBC dapat disimpulkan sebagai membiarkan proses penyembuhan secara natural berlangsung.

 

Anak laki – laki berusia 11 tahun dengan simple bone cysts pada tibia sinistra proksimal.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Pasien yang sama pada gambar sebelumnya setelah pemberian kontras dalam masa terapi dengan metilprednisolon. Terlihat lapisan kontras dalam lesi, memberikan gambaran fluid  contrast level.

 

 

 

 

Anak laki – laki berusia 10 tahun dengan simple bone cysts pada tibia dextra 1/3 tengah yang telah diterapi dengan bone graft  memperlihatkan potongan tulang multiple dalam rongga kista.

 

 

 

 

Keuntungan dari terapi dengan injeksi metilprednisolon adalah lebih singkatnya waktu yang diperlukan, sedikit perdarahan, dan waktu hospitalisasi minimal. Tetapi tingkat kesembuhan seringkali tidak dapat diduga dan  tidak sempurna setelah beberapa kali penyuntikan. Gambaran radiologis dari terapi injeksi steroid yang berespon baik adalah reduksi dari kavitas, meningkatnya radioopasifitas dari kista, penebalan korteks tulang, dan remodelling tulang. Lesi besar dan terbukti secara radiologis adanya septasi, merupakan faktor – faktor yang mengindikasikan respons buruk terhadap terapi.

Anak perempuan berusia 9 tahun dengan simple bone cysts pada diametafisis distal tibia. Radiografi lateral menunjukkan simple bone cysts dengan komplikasi fraktur patologis.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Pria dewasa muda dengan simple bone cysts  pada kalkaneus

 

 

 

 

 

 

 

 

 

 

 

 

 

Anak laki – laki berusia 8 tahun dengan simple bone cysts pada metafisis proksimal femur dextra proksimal.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Anak laki – laki 7 tahun dengan simple bone cysts pada tibia dextra. Lesi dengan komplikasi fraktur.

 

 

 

 

 

 

 

 

 

 

 

 

 

Anak laki – laki 10 tahun dengan simple bone cysts pada region metafisis proksimal humerus dextra. Terdapat fraktur patologis dan “fallen fragment”.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Pasien yang sama dengan gambar sebelumnya. Pemeriksaan follow-up setelah 18 bulan menunjukkan fraktur yang telah sembuh, tetapi ukuran kista menjadi lebih besar.

 

 

 

 

Pasien yang sama dengan 2 gambar di atas – follow up 10 bulan kemudian menunjukkan sclerosis dalam kista.

 

 

 

 

 

 

 

 

 

 

 

 

 

Pasien yang sama pada 3 gambar sebelumnya – pemeriksaan 6 tahun kemudian menunjukkan simple bone cysts telah sembuh. Sklerosis terlihat pada area yang sebelumnya ditempati oleh kista.

 

 

 

 

III.2. ANEURYSMAL BONE CYSTS

III.2.1. SEJARAH DAN DEFINISI  [30] , [31]

Jaffe dan Lichenstein pertama kali mendeskripsikan aneurysmal bone cyst (ABC) sebagai suatu lesi tersendiri pada tahun 1942 dalam catatan : “a peculiar blood-containing cyst of large size”. 2 kasus ditemukan dengan lesi seperti busa sabun pada pemeriksaan radiologi. Kasus pertama pada laki – laki berusia 17 tahun pada ramus pubik superior dan kasus kedua pada vertebra anak laki – laki 18 tahun. Lesi tersebut ekspansif dan memperlihatkan adanya erosi pada tulang dan jaringan di sekitarnya. Saat dilakukan operasi, sebuah dinding tulang yang tipis dan mengandung cairan darah ditemukan.

Menurut defines WHO, aneurysmal bone cyst merupakan lesi jinak, dan dideskripsikan sebagai an expanding osteolytic lesion consisting of blood-filled spaces of variable size separated by connective tissue septa containing trabeculae or osteoid tissue and osteoclast giant cells." Meskipun jinak, aneurysmal bone cyst dapat tumbuh cepat dan bersifat destruktif terhadap tulang. Karakter ekspansif ini menyebabkan nyeri, udem, deformitas, gangguan pada lempeng pertumbuhan, deficit neurologis, dan fracture patologis.

III.2.2. FREKUENSI

Aneurysmal bone cyst merupakan jenis kasus yang jarang ditemukan, menduduki 1 – 6% dari seluruh tumor primer tulang. Sebuah studi di Austria melaporkan insidens tahunan adalah 0,14 per 100.000 orang. Angka kejadian sebenarnya sulit untuk dikalkulasi karena adanya regresi spontan lesi dan kasus – kasus yang tidak terungkap dalam klinik.

Penelitian berdasarkan hasil biopsi di Belanda menunjukkan bahwa ABC merupakan tumor nomor dua terbanyak pada anak – anak. Mayoritas penelitian menunjukkan peningkatan insiden pada wanita. Meskipun ABC dapat terjadi pada segala usia, pada umumnya ini adalah penyakit usia muda (tetapi tidak sangat muda). Sekitar 50 – 70% kasus terjadi pada dekade kedua hidup, dengan 70 – 86% terjadi pada pasien lebih muda dari 20 tahun. Usia rata – rata onset adalah 13 – 17,7 tahun.

III.2.3. ANATOMI

Aneurysmal bone cyst dapat muncul pada seluruh tulang di seluruh tubuh. Paling sering terdapat pada tulang panjang, diikuti vertebra dan tulang pipih. Area – area ini mencakup 80% dari seluruh keseluruhan kasus aneurysmal bone cysts. Bila terdapat pada tulang panjang, ABC cenderung berlokasi pada metafisis. Meskipun begitu, ABC dapat terjadi di segala lokasi, termasuk diafisis dan epifisis.

Meskipun jarang, ABC juga diketahui dapat menyerang tulang yang bersebelahan. Tetapi pada kasus ABC verterbra, resiko lesi kontagius ini meningkat. Lesi pada pelvis merupakan 50% dari seluruh lesi pada tulang pipih. Pada ABC sekunder, memiliki predileksi pada area dimana lesi primer terdapat. Pada penelitian terhadap 897 kasus, didapat angka kejadian sebagai berikut :

  • Tibia – 17.5%
  • Femur – 15.9%
  • Vertebra – 11.2%
  • Pelvis – 11.6%
  • Humerus – 9.1%
  • Fibula – 7.3%
  • Foot – 6.3%
  • Hand – 4.7%
  • Ulna – 3.8%
  • Radius – 3.1%
  • Lain - lain – 9.2%

III.2.4. PATOGENESIS [30] , [31]

Patogenesis pasti pada terbentuknya aneurysmal bone cyst hingga kini belum diketahui. Beberapa teori mengatakan bahwa lesi disebabkan oleh trauma yang mendahului atau subperiosteal hematom. Teori lain menyebutkan bahwa aneurysmal bone cysts merupakan hasil dari suatu proses reparasi. Meskipun ada beberapa teori yang disebutkan, lesi kistik ini secara umum disebabkan oleh reaksi vaskuler sekunder akibat paparan dengan lesi pendahulu, yang diasumsikan menginisiasi proses malformasi arterio-venosus periosteal atau intraosseus. Resultan kekuatan hemodinamik akibat tingginya tekanan dari jalur – jalur vaskuler yang terbentuk, secara cepat akan mengerosi trabekula tulang menjadi rongga kistik. Reaksi asosiatif yang terjadi pada endosteum dan periosteum akan mengakselerasi aktivitas osteoblas dan osteoklas, sehingga proses remodelling akan berlangsung sangat cepat, bersaing dengan tekanan hemodinamik yang tinggi. Kedua proses ini akan memberi gambaran lesi seperti balon, berbatas tipis, dan multi-septum (soap-bubble apperance).

Berbagai teori yang berbeda mengenai malformasi vaskuler juga menyebutkan mengenai fistula arteriovenosus dan blokade vena. Lesi ini meningkatkan tekanan, mengekspansi, erosi, dan resorpsi tulang sekitarnya. Malformasi ini juga dipercaya menyebabkan perdarahan lokal yang menginisiasi pembentukan jaringan osteolytic reaktif. Temuan dari penelitian dimana dilakukan pengukuran tekanan manometrik dalam aneurysmal bone cyst mendukung teori peningkatan tekanan hemodinamik.

Konsep yang menyebutkan bahwa aneurysmal bone cysts terjadi setelah lesi-pendahulu adalah berdasarkan fakta bahwa pada sekitar 20 – 30% kasus, aneurysmal bone cysts terjadi setelah terdapat riwayat lesi skeletal.

Tetapi karena pada banyak kasus juga tidak terdapat lesi awal, hal ini juga membuktikan bahwa memang terdapat aneurysmal bone cysts primer, dengan sifat – sifat klinis, gambaran radiologis, dan prognostiknya sendiri. Diagnosa aneurysmal bone cysts primer dapat dibuat bila seluruh lesi awal telah dieksklusi.

Meskipun etiologi pasti dari ABC belum diketahui secara tepat, tiga teori yang telah ditemukan adalah :

  • Akibat dari reaksi sekunder terhadap lesi tulang lainnya. Teori ini diajukan karena adanya tumor penyerta pada 23 – 32% dari ABC. Yang tersering adalah giant cell tumors. Terdapat juga berbagai tumor benign dan maligna, termasuk fibrous dysplasia, osteoblastoma, chondromyxoid fibroma, nonossifying fibroma, chondroblastoma, osteosarcoma, chondrosarcoma, unicameral or solitary bone cyst, hemangioendothelioma, dan metastatic carcinoma. Aneurysmal bone cyst yang terdapat bersama tumor lain ini disebut sebagai Aneurysmal bone cyst sekunder. Terapi terhadap ABC sekunder ini tergantung pada lesi utamanya.
  • ABC dapat muncul secara de novo; muncul tanpa bukti adanya lesi lain. Disebut sebagai aneurysmal bone cyst sekunder.
  • Aneurysmal bone cyst yang muncul di tempat dengan riwayat trauma.

Studi dalam pencitraan neurologis dan studi histopatologis telah dibuat untuk mengidentifikasi 4 stadium dalam perjalanan penyakit aneurysmal bone cyst.

  1. Stadium inisiasi ditandai dengan berbagai derajat ekspansi dan penipisan korteks tulang yang akan menghasilkan perubahan kistik.
  2. Stadium pertumbuhan ditandai dengan gambaran busa sabun yang dikelilingi dengan penipisan korteks progresif.
  3. Stadium ketiga merupakan stadium paling berbahaya karena dapat terjadi pembesaran kista vaskuler ini dengan amat cepat, yang dapat menyebabkan kolaps tulang dan ekspansi ke jaringan sekiarnya. Stadium ini juga merupakan salah satu tanda dimulainya onset defisit neurologis.
  4. Stadium penyembuhan akhir, pada beberapa kasus akan terjadi osifikasi spontan dan penyusutan lesi.

III.2.5. Gejala Klinis

Pasien biasanya datang dengan keluhan :

- nyeri, massa, dan fracture patologis pada area lesi. Gejala ini biasa muncul selama beberapa minggu hingga beberapa bulan sebelum diagnosis dibuat. Durasi dari munculnya gejala hingga diagnosis dibuat berkisar antara 4 – 8 bulan. Nyeri lokal merupakan gejala yang umum dari penyakit ini. Nyeri akan memburuk pada malam hari. Dapat juga ditemukan udem atau massa dengan nyeri tekan. Bila teraba massa posterior biasanya terletak pada paravertebral, sedangkan massa anterior dapat menyerupai neoplasma tiroid. Penderita juga sering mengeluhkan terdapatnya massa yang cepat membesar. Fracture patologis terjadi pada sekitar 8% kasus, tetapi angka kejadian dapat meningkat lebih dari 21% bila lesi terdapat pada vertebra. Gejala klinis lainnya dapat berupa :

  • Deformitas
  • Pergerakan terbatas
  • Tortikolis
  • Bruit di sekitar lesi
  • Rasa panas di sekitar lesi

- Defisit neurologis - dapat terjadi akibat tekanan pada saraf di sekitar lesi, khususnya pada vertebra. Pada ABC pada vertebra, wilayah posterior dan pedikel biasanya terserang lebih dahulu, dan pada 60 – 70% kasus akan meluas ke korpus vertebra. Ekspansi yang berlanjut dan destruksi korpus vertebra dapat menyebabkan kolaps mendadak, deformitas dari kolum vertebra, dan kompresi epidural akut. Pada 25 – 35% kasus lesi akan menyeberang ke vertebra di sebelahnya melalui jaringan lunak yang mengelilingi, tetapi tidak pernah melalui diskus intervertebralis. Skoliosis dan kifosis juga ditemukan pada 10 – 15% pasien.

Gejala neurologis dapat merupakan gejala awal pada 60 – 70 % kasus. Kompresi radiks – radiks saraf spinal dapat menyebabkan parestesia dan nyeri radikular yang menyebar ke regio toraks, abdomen, atau ekstremitas. Kompresi nervus spinalis oleh kolapsnya korpus vertebra atau deformitas (kifosis) dapat menyebabkan mielopati atau paraparese akut. Kompresi nervus spinalis akut juga dapat terjadi tanpa adanya kolaps korpus vertebra, bila terdapat kerusakan pada korteks posterior sehingga terjadi ekstensi lesi ke rongga epidural. Kemunduran keadaan penderita atau paraplegi total dapat terjadi bila lesi dibiarkan tanpa terapi.

Meskipun aneurysmal bone cysts merupakan lesi jinak, non-neoplastik, dan tidak bermetastase, tetapi kecenderungannya untuk cepat membesar, bersifat destruktif terhadap tulang, dan potensial untuk menyebabkan defisit neurologis, telah membawa jenis lesi kistik ini untuk mendapatkan terapi agresif. Aneurysmal bone cysts juga dapat mengalami regresi spontan atau regresi setelah tindakan biopsi pada lesi. Regresi ini merupakan fenomena langka, yang diduga terjadi karena trombosis atau fibrosis dari lesi.

III.2.6. PROSEDUR DIAGNOSTIK

Sebagian klinisi meyakini bahwa temuan patognomonik pada pemeriksaan radiologi dapat digunakan untuk menegakkan diagnosa ABC. Tetapi bila masih terdapat keraguan, biopsi terbuka harus dilakukan. Diagnosis berdasarkan pemeriksaan histologi sebaiknya selalu dilakukan karena penting untuk memastikan diagnosis ABC dan meyakinkan bahwa lesi lain (misal keganasan) tidak hadir bersama – sama ABC. Bila biopsi dilakukan, sampel harus mencakup materi dari sekitar lesi, karena bila biopsi hanya terbatas dari lesi maka lesi lain yang hadir bersama dapat terlewatkan dan menyebabkan prognosis yang buruk bagi pasien.

Seperti kasus – kasus onkologi ortopedi lainnya, biopsi merupakan bagian dari terapi, bukan penunjang. Operator yang melakukan biopsi juga harus dapat menangani baik kasus benign maupun maligna.

III.2.7. Staging

a. Latent or inactive musculoskeletal neoplasms

  • Asimtomatik
  • Biasanya ditemukan tidak sengaja
  • Jarang terdapat fracture patologis atau disfungsi organ lainnya
  • Dapat tumbuh perlahan, tetapi hampir selalu mencapai stadium dimana neoplasma tidak bertumbuh lagi
  • Tetap berada di dalam kompartemen
  • Tidak menyebabkan deformitas
  • Pada palpasi massa berukuran kecil, dapat digerakkan dari dasar, dan tidak nyeri
  • Pada pemeriksaan radiologis :
    • Berbatas tegas, tanpa deformasi atau ekspansi
    • Lodwick IA.
    • Scan isotop – sedikit atau tidak ada peningkatan uptake
    • Angiogram : tidak ditemukan angiogene
    • CT Scan : densitas homogen, berbatas tegas, tanpa ekstensi lintas fasia.
    • Karkteristik histologik :
      • Nucleus : cytoplasmic ( N : C ) ratio rendah
      • Matriks sel matur, berdiferensiasi baik
      • Karakteristik sitologik benign
      • Berkapsul dengan jaringan fibrosa matur
      • Sedikit atau tidak ada prolifeasi mesenkimal, infiltrat inflamasi, atau neoangiogenesis pada lesi.

 

b. Active musculoskeletal neoplasms                     

 

  • Sedikit simtomatik
  • Ditemukan karena keluhan pasien atau terjadinya fracture patologis atau disfungsi mekanik.
  • Terus bertumbuh, terus membesar selama observasi.
  • Tampak responsif terhadap inhibisi kontak, tetapi tidak pada level normal
  • Dapat mengekspansi
  • Masih berkapsul
  • Zona reaktif antara lesi dan jaringan normal di sekitarnya hanya dipisahkan oleh jaringan tipis.
  • Pada palpasi teraba lesi kecil dengan nyeri ringan sampai sedang dan dapat digerakkan.
  • Radiologi :
    • Margin iregular
    • Batas cancellous ring matur, bukan lapisan kortikal.
    • Bagian dalam defek iregular, sehingga menghasilkan gambaran septasi.
    • Ekspansi, bulging, deformitas sering ditemukan pada observasi.
    • Lodwick IB.
    • Scan isotop : peningkatan uptake isotop hanya terbatas pada defek.
    • Angiogram : terkadang angiogenesis reaktif dapat ditemukan di sekitar lesi, hampir tidak pernah di dalam lesi.
    • CT Scan :
      • Densitas homogen
      • Reactive bone iregular tetapi intak, ekspansi ke korteks sekitar
      • Karakteristik histologik :
      • N : C ratio seimbang
      • Karakteristik sitologi benign
      • Kapsul intak yang tersusun atas jaringan fibrosa atau cancellous bone.
      • Zona sempit yang berisi jaringan reaktif vaskular, inflamasi, dan mesenkim.
      • Resorpsi tulang sehat oleh osteoklas daripada oleh sel neoplastik, sebagai mekanisme ekspansi.

c. Aggressive musculoskeletal neoplasms

  • Bersifat benign, dapat juga bersifat low-grade malignancy.
  • Simtomatik
  • Ditemukan karena keluhan pasien, massa, atau fracture patologis
  • Pada palpasi, biasanya besar dan nyeri, dapat membesar dengan cepat pada pemeriksaan fisik serial, dan lebih terfiksasi.
  • Dapat memiliki gambaran inflamasi
  • Inhibisi kontak minimal
  • Menembus barier natural dari pertumbuhan tumor, yaitu tulang, septum fasia, dan kartilgo artikular.
  • Menembus kapsul dengan proyeksi “finger-like
  • Merusak atau mereabsorbsi tulang atau fasia dan menembus jaringan atau kompartemen sekitarnya .
  • Ekspansi cepat pada area yang tidak dibatasi, dapat diawali dengan pseudokapsul.
  • Radiologi :
    • Expansi ke tulang sekitar
    • Destruksi kortikal
    • Endosteal buttresses
    • Periosteal Codman triangles.
    • Ekspansi cepat ke jaringan sekitar
    • Lodwick IC
    • Isotope scan : peningkatan uptake
    • Angiogram : zona reaktif neovaskularisasi pada fase arterial awal dan blush hipervaskular intralesi pada fase vena akhir.
    • CT Scan :
      • Area non-homogen, mottled, dengen defek pada daerah reaktif
      • Ekstensi ekstrakompartemen dini dari tulang
      • Tidak berbatas tegas pada jaringan
      • Kemungkinan keterlibatan neurovaskular bundle.
      • Karakteristik histologi :
        • N : C ratio tinggi
        • Diferensiasi matrix dengan maturitas yang berbeda – beda
        • Karakteristik sitologi benign predominan, tanpa anaplasia atau pleomorfik, tetapi dengan lebih banyak nukleus hiperkromatik.
        • Gambaran mitosis
        • Invasi vaskular
        • Ekstensi dari massa utama, dapat juga terdapat lesi satelit.
        • Zona jaringan reaktif yang tebal diantara kapsul dan jaringan normal yang lebih perifer. ( Zona pseudokapsul tidak menghambat pertumbuhan tumor agresif,  tetapi menghambat nodul tumor untuk menginvasi langsung jaringan normal ).
        • Destruksi tulang sekitar melalui osteoklas reaktif, bukan oleh sel tumor.
        • ”Jari – jari” tumor yang bertumbuh hingga tulang reaktif.

III.2.7. PEMERIKSAAN PENUNJANG

  • Laboratorium

Alkali fosfatase dapat meningkat, tetapi pemeriksaan laboratorium tidak selalu bermakna.

  • Pemeriksaan Histologis

Pada inspeksi secara kasar, aneurysmal bone cysts memiliki tampilan multilokular, gambaran spongiosa dengan rongga yang terisi darah dan terpisah – pisah oleh septum fibrosa tipis. Merupakan lesi yang ekspansif yang mengerosi dan menghancurkan tulang sekitarnya, meninggalkan tepi setipis kulit telur pada tulang yang baru terbentuk di periosteal dan korteks sekitarnya. Pusat tumor merupakan jaringan lunak dan vaskular. Perdarahan  dapat muncul dari jaringan lunak yang mengelilingi kista dan akan sulit dikontrol hingga semua batas – batas tumor diangkat.

Secara histologis septum fibrosa pada aneurysmal bone cysts terbentuk dari fibroblas, miofibroblas, osteoklas multinuklear, deposit hemosiderin, pembuluh darah, dan osteoid. Cavernous, atau kista yang terisi darah, tidak merupakan saluran vaskular yang sebenarnya karena pembuluh – pembuluh tersebut tidak memiliki endotel dan jaringan elastis, atau otot polos yang ditemukan pada pembuluh darah normal. Dapat ditemukan aktivitas mitosis, yang menandakan adanya aktivitas proliferasi.

Aneurysmal bone cysts dapat menginvasi jaringan sekitar melalui penambahan ukuran kavitas atau proliferasi dari jaringan dasarnya.

               

Fotomikrograf jaringan pada pemeriksaan patologi anatomi. Menunjukkan fibroblas dan beberapa sel – sel raksasa.

Tampilan umum dari ABC adalah spongiosa yang terisi darah. Lapisan subperiosteal tipis mengelilingi struktur dan isi kista. Pada jaringan terlihat septum – septum. Stroma mengandung fibroblas proliferatif, sel spindel, area pembentukan osteoid, dan sel – sel raksasa multinuklear. Jaringan di dalam septum mengandung kavernosa- kavernosa yang tidak memiliki struktur muskular atau jaringan elastin pada dindingnya. Gambaran mitosis juga dapat ditemukan, tetapi, tetapi tidak khas dan tidak dapat dijadikan bukti pendukung diagnosis.

Varian solid dari ABC juga telah ditemukan, dengan gambaran histologis yang sama dengan varian kistik, tetapi dengan struktur yang lebih solid.  

  • Pemeriksaan Radiologi [35]
    • Gambaran radiologis biasanya menunjukkan lesi eksentrik lesi sentral subperiosteal yang terlihat kistik atau litik. Dapat terlihat juga ekspansi ke tulang sekitar yang mem’balon’, dan berbentuk busa sabun. Beberapa foto menunjukkan tepi bertulang setipis kulit telur di sekeliling lesi.
    •  Deskripsi Capanna mengenai 5 tipe morfologi berdasarkan gambaran radiologis pada ABC :

 

  • Tipe I

Presentasi pada sentral metafisis, terletak dengan baik di tengah – tengah struktur tulang, dengan profil tulang intak atau sedikit sekali ekspansi.

  • Tipe II

ABC menyerang hampir seluruh segmen tulang, gambaran inflated dan penipisan korteks.

  • Tipe III

Lokasi eksentrik pada metafisis, tanpa atau sedikit ekspansi ke korteks.

  • Tipe IV

Ekstensi ke subperiosteal, tanpa atau dengan erosi kortikal minimal, jarang pada diafisis.

  • Tipe V

Lokasi pada meta-diafisis, inflasi periosteum dari jaringan lunak, penetrasi korteks, ekstensi ke tulang.

  • CT – Scan : menunjukkan karakteristik yang sama seperti yang ditunjukkan oleh foto polos, tetapi pada pemeriksaan CT – Scan dapat terlihat septum internal (batas yang mengalami kalsifikasi, gambaran cangkang telur, dan sebagainya), dapat lengkap atau parsial. Fluid – fluid level dapat ditemukan, meskipun sering ditemukan pada ABC tetapi tidak eksklusif untuk mendiagnosa. Fluid – fluid level ini disebabkan oleh pemisahan antara bagian selular dan serum dalam ruang kista. Untuk memperihatkannya pasien harus berada pada posisi yang tetap selama 10 menit untuk memperoleh pemisahan yang cukup antara materi selular dan serum.
  • MRI : menghasilkan temuan yang sama seperti pada CT Scan, tetapi MRI lebi spesifik memerlihatkan darah dalam lesi, juga ekspansi ke jaringan lunak.
  • Pada pencitraan radiologi dibagi menjadi beberapa fase perkembangan : inisial atau insipien, pertumbuhan, stabil, dan penyembuhan.
    • Inisial atau insipien
      • Lesi kecil
      • Tidak terdapat ekspansi
      • Sedikit erosi pada korteks
      • Pertumbuhan
        • Pola pertumbuhan destruktif
        • Lisis tulang masif dan destruksi kortikal
        • Tumbuh sangat cepat hingga periosteum tulang tidak dapat bertahan utuh.
        • Tampak menggembung keluar.
        •  
        • Stabil
          • Tampilan ABC klasik
          • Ekspansi tulang sekitarnya
          • Lapisan tulang mengelilingi sejumlah trabekula interna
          • Penyembuhan
            • Osifikasi trabekula progresif
            • Membentuk massa trabekula ireguler
      • Scanning tulang : uptake meningkat disekitar lesi. Dapat terlihat halo atau efek ”donat” dari meningkatnya uptake radionuklir di sekitar area dengan uptake rendah.
      • Angiografi : pada beberapa kasus, area hipervaskular di sekeliling ABC terlihat. Area difus intens dengan kontras persisten terakumulasi dan tervisualisasi tanpa pembuluh darah aferen atau eferen yang terlihat. Dapat membantu bila akan dilakukan embolisasi arterial selektif.

 

 

 

 

 

 

 

 

 

 

ABC pada lengan atas.

Courtesy of Johannes Stahl, The Virtual Radiological Case Collection

 

 

 

 

ABC pada lengan atas.

Courtesy of Johannes Stahl, The Virtual Radiological Case Collection

 

 

 

 

 

 

ABC pada lengan atas pada anak perempuan 13 tahun. Ki-ka : radiografi  yang dibuat dalam selang waktu 2 bulan.

 Courtesy of Armed Forces Institute of Pathology.

 

 

 

 

 

 

 

 

 

 

ABC pada vertebra - lumbal.

Courtesy of Nick Oldnall, Index: Spine

 

 

ABC pada lengan atas. Terdapat erosi kortikal dan ekspansi ringan.

Courtesy of Armed Forces Institute of Pathology.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ABC pada lengan atas.

 Used with permission from the American Registry of Pathology. Courtesy of Armed Forces Institute of Pathology.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ABC pada lengan atas.

Used with permission from the American Registry of Pathology. Courtesy of Armed Forces Institute of Pathology.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Pada gambaran histologis, ruang–ruang vascular bervariasi dalam bentuk dan ukuran, dengan septum yang bervariasi dalam ukuran dan bentuk. Terlihat tulang yang baru terbentuk.

Courtesy of Armed Forces Institute of Pathology.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Gambaran histologis, septum mengandung fibroblast, mononuclear, sel raksasa multinuklear, dan kapiler. Batas dari ruang vascular dapat tidak terlihat atau tersusuan atas lapis tunggal sel stroma, seperti yang terlihat.

Used with permission from the American Registry of Pathology. Courtesy of Armed Forces Institute of Pathology.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Jaringan dengan lesi, dimana korteks telah rusak total.

Used with permission from the American Registry of Pathology. Courtesy of Armed Forces Institute of Pathology.

 

 

 

 

 

 

Gambaran histologis yang menunjukkan septum fibrosa yang mengandung barisan osteoid yang menggerombol di tengah.

Used with permission from the American Registry of Pathology. Courtesy of Armed Forces Institute of Pathology.

 

 

 

 

 

 

III.2.8. Karakteristik Pencitraan Neurologi

Studi pencitraan neurologi dapat memberikan karekteristik lesi litik dan ekspansi tulang karena erosi dan destruksi dari korteks. Fracture patologis dengan kolaps parsial dari korpus vertebra sering terjadi, dan seringkali berkembang menjadi kolaps sempurna (vertebra plana).

CT Scan pre-operasi lebih baik untuk menggambarkan lokasi dan wilayah destruksi tulang, untuk melihat integritas tulang, dan melihat deposit kalsium.Terkadang hubungan antar kavitas dapat terlihat.

 

Gambaran pencitraan neurologi menunjukkan anak perempuan berusia 3 tahun dengan keluhan massa pada leher posterior dan nyeri. CT Scan aksial menunjukkan lesi besar, multikistik, dan ekspansif pada C2.

 

 

 

 

Foto polos yang diambil setelah laminektomi C2 dengan reseksi aneurysmal bone cysts, diikuti dengan fusi C1–C3 dengan alograf kosta.

 

                               

 

Pemeriksaan MRI akan memberikan evaluasi paling optimal mengenai isi lesi dan ekstensi ke tulang dan jaringan lunak. Derajat kompresi elemen neuron juga terlihat lebih baik. Pada kasus dimana aneurysmal bone cysts melakukan ekspansi melalui jaringan lunak paravertebral ke vertebra yang bersebelahan, diskus intervertebralis tidak pernah terlibat dalam lesi ini.

III.2.9. Diagnosis Banding

Aneurysmal bone cysts harus dibedakan dari giant cell tumor, hemangioma, fibrous dysplasia, osteosarcoma, dan lesi metastatik.

  • Giant cell tumor, dengan predileksi utamanya di daerah sakrum. Tidak seperti aneurysmal bone cysts, pada giant cell tumor tidak terdapat ruang – ruang kavernous vascular. Pada pemeriksaan patologi anatomi, sel – sel raksasa lebih besar pada giant cell tumor dan jumlahnya jauh lebih banyak.
  • Vertebral hemangioma dibedakan dengan ciri trabekula dan gambaran sarang lebah pada pemeriksaan CT Scan. Biasanya hanya terdapat pada satu vertebra dan tidak mengakspansi dan mem”balon” keluar seperti aneurysmal bone cysts.
  • Fibrous dysplasia pada vertebra menyerang kelompok usia yang sama seperti pada aneurysmal bone cysts, dapat menyerang beberapa vertebra, dan pertumbuhannya lambat. Ciri yang penting adalah tidak terdapat nyeri.
  • Osteosarcoma dan lesi metastase biasanya disertai nyeri, dan tidak menyebabkan ekspansi tulang dan “ballooning”.

 III.2.10. TERAPI [13]

Pada terapi dengan kuretase, terjadi lesi persisten pada 25% kasus, maka dianjurkan untuk dilakukan reseksi luas . Pada kebanyakan lesi vertebra, dapat diperoleh hasil memuaskan dengan terapi operatif. Penggunaan radioterapi tidak dianjurkan karena:

  • Resiko induksi sarcoma, kerusakan sel – sel gonad, dan gangguan lempeng pertumbuhan.
  • Adverse effect yang merusak. Meskipun begitu, radioterapi masih digunakan terutama untuk lesi – lesi yang sulit diakses untuk terapi operatif.

Pada aneurysmal bone cysts dapat juga dilakukan trans – kateter embolisasi arterial.

  1. TERAPI MEDIS [33]

Embolisasi arteri selektif merupakan salah satu terapi yang banyak menjanjikan menurut beberapa penelitian. Meskipun begitu, hanya beberapa kasus ABC yang diterapi dengan embolisasi arteri selektif ini, karena ABC merupakan kasus yang jarang dan terapi ini baru mulai digunakan sejak 1980.

Dengan bantuan angiografi, embolic agent ditempatkan ke feeding artery yang menuju ABC, sehingga memotong suplai nutrisi dan hemodinamik dari lesi. Beberapa jenis material, seperti spiral dan busa, digunakan untuk menciptakan emboli. Keuntungan metode ini adalah dapat mencapai lokasi sulit, dapat menjaga fungsi persendian bila terjadi destruksi tulang subkondral, dan meminimalkan komplikasi yang dapat terjadi pada tindakan bedah invasif. Embolisasi arteri selektif ini dapat dilakukan 48 jam sebelum operasi untuk mengurangi perdarahan.

Embolisasi arteri selektif dapat menjadi terapi utama jika kondisi – kondisi berikut terpenuhi :

  • Diagnosis pasti aneurysmal bone cysts
  • Persyaratan teknis memenuhi (kelengkapan fasilitas dan keamanan)
  • Stabilitas lesi, tidak terdapat fracture patologis
  • Tidak terdapat defisit neurologis

Kontraindikasi terapi embolisasi arteri selektif adalah :

  • Diagnosis yang belum pasti, diperlukan open biopsy
  • Instabilitas struktural, seperti fracture patologis
  • Gejala defisit neurologis
  • Disrupsi mekanik
  • Lokasi yang tidak aman untuk dilakukan embolisasi (misal arteri segmental, area pada servikal dan thorax yang dapat berakibat iskemia medula spinalis)

 

Injeksi intralesi dapat digunakan pada kasus dimana terapi operatif sulit dilakukan atau terdapat kontraindikasi. Tetapi metode ini tidak dapat dilakukan bila terdapat alergi terhadap komponen injeksi, dan apabila dapat dilakukan terapi lain yang diindikasikan).

Terdapat juga bukti – bukti penggunaan injeksi kalsitonin dan metilprednisolon untuk regresi aneurysmal bone cysts. Injeksi ini mengkombinasikan inhibisi angiostatik efek fibroblastik dari metilpredisolon dan efek inhibisi osteoklas dan trabecular bone stimulating stimulating dari kalsitonin. Injeksi dilakukan dengan anestesi dan bantuan CT. Pertumbuhan ABC harus dimonitor ketat, dan terapi dapat harus diberikan beberapa kali, dapat mencapai hingga beberapa tahun.

ETHIBLOC injeksi juga diberikan di bawah anestesi dan bantuan CT. Solusi injeksi merupakan campuran dari zein, oleum papaverin, dan propilen glikol, yang berfungsi sebagai fibrosing agent. Reaksi inflamasi dapat terjadi setelah penyuntikan. Penyembuhan tulang dapat memakan waktu beberapa bulan hingga beberapa tahun. Beberapa kasus menunjukkan perbaikan dalam proses penyembuhan bila diberikan kalsitonin sistemik sebagai ad juvant untuk terapi lainya.

  1. Terapi Operatif [33]

-  ABC stadium I dapat diterapi dengan kuretase intralesi.  Pada stadium II terapi dilakukan dengan eksisi intralesi. Perbedaannya adalah pada eksisi dilakukan wide unroofing lesi dengan abrasi seluruh permukaan lesi dengan bor kecepatan tinggi, dan lokal ad juvant seperti fenol, metik – metakrilat (MMA), atau nitrogen cair. Penggunaan ad juvant ini masih controversial karena resiko yang dapat ditimbulkan.

-  En bloc atau eksisi luas dilakukan pada ABC stadium III dimana tidak dapat lagi dilakukan eksisi intralesi (misal karena destruksi tulang). Rekonstruksi setelah eksisi luas dapat dilakukan dengan alograf struktural atau komposit prostetik alograf.

- Pada waktu lampau, eksisi intralesi merupakan terapi utama. Dibuat lubang pada dinding kista dan isi kista diambil. Pada dinding dapat dilakukan eksisi dengan kuret atau bor berkecepatan tinggi. Metode intralesi ini meninggalkan bony structure lebih banyak dibanding metode en bloc atau wide resection. Eksisi intralesi dapat digunakan pada daerah sekitar sendi atau area vital lainnya untuk mempertahankan fungsi. Defek tulang kemudian dapat diisi dengan bone chips, bone strut, atau materi pendukung lainnya untuk menambah kekuatan dan merangsang penyembuhan area tersebut.

- Ahli bedah juga dapat menggunakan terapi adjuvant yang memperluas area terapi. Penggunaan nitrogen cair, fenol, dan polimetilmetakrilat (PMMA) dapat mencapai area terapi yang lebih luas. Adjuvant ini meliputi penggunaan reaksi kimia, pembekuan, atau termal yang dimaksudkan untuk menyebabkan nekrosis tulang dan kerusakan mikrovaskular pada dinding kista yang dieksisi. Dibandingkan dengan en bloc dan reseksi regional, penggunaan adjuvant meninggalkan lebih banyak tulang intak sehingga area yang mendapatkan terapi lebih luas dibandingkan dengan bila hanya digunakan metode intralesi secara tunggal.

Nitrogen cari merupakan adjuvant paling populer dan sering dideskripsikan dalam literatur. Setelah ABC terekspos dan dibuat ”jendela”, nitrogen cair diaplikasikan ke dalam kista menggunakan alat yang menyemprotkan nitrogen cair tersebut ke seluruh dinding kista. Operator harus meninggalkan lubang  yang telah dibuat tetap terbuka untuk membiarkan gas menguap. Jumlah total sekitar 2 – 3 siklus pembekuan harus digunakan untuk mendapatkan nekrosis tulang maksimum.

Fenol merupakan jenis adjvant lain yang sering digunakan. Beberapa peneliti mempertanyakan efektivitas fenol karena penetrasinya yang sulit ke dalam jaringan tulang dibandingkan dengan nitrogen cair. Salah satu keuntungan penggunaan fenol adalah mudah untuk digunakan. Kemudian sisa – sisa fenol yang tertinggal dibersihkan dengan suction dan kavitas diisi dengan alkohol absolut. Terakhir, kavitas diirigasi dengan larutan isotonik NaCl.

PMMA juga dapat digunakan, meskipun kemampuan termalnya dalam menyebabkan nekrosis tulang masih banyak dipertanyakan. Keuntungan penggunaan PMMA adalah memudahkan mengenali rekurensi lokal. Bila PMMA digunakan pada lokasi subkondral, permukaan sendi harus dilindungi dengan cancellous graft atau Gelfoam yang ditempatkan sebelum sementasi.

Perhatian khusus perlu diberikan pada ABC yang terletak dekat dengan epifisis. Bila akan dilakukan pembedahan diperlukan pertimbangan luas, disebabkan terdapatnya jumlah signifikan kerusakan epifisis. Begitu pula dengan ABC pada vertebra yang dapat menimbulkan deficit neurologis. Pada ABC vertebra, terdapat bukti – bukti yang mendukung untuk dilakukannya 1 – 2 kali percobaan embolisasi selektif arterial sebelum dilakukan eksisi.

-  Sejumlah peneliti di Jepang mengembangkan metode endoskopi untuk terapi ABC. Mereka sukses merawat 4 pasien dengan ABC. Teknik ini menggunakan kuret, ball forceps, Kirschner wires (K-wire), artroskopi, dan bor. Metode ini meninggalkan struktur yang lebih stabil dan tingkat invasif minimal.

-  Jenis – jenis terapi lainnya :

  • Reseksi lokal à digunakan pada area expendable dan tidak mempengaruhi fungsi. (Contoh : pada processus spinosus atau tibia)
  • Eksisi en bloc – lesi dalam à terdapat kemungkinan menurunnya fungsi persendian.
  • Intralesional removal à dilakukan pada wilayah yang sulit dijangkau secara operatif
  • Terapi intralesi dengan adjuvant à substansi seperti nitrogen cair dan fenol dapat melakukan penetrasi ke jaringan dan merusak struktur sekitarnya. Karena alasan ini beberapa peneliti tidak menyetujui penggunaan terapi intralesi dengan adjuvant pada vertebra.

Persiapan Pra-Bedah

Bila telah direncanakan untuk melakukan pembedahan sebaiknya dilakukan pencitraan cross – sectional. Embolisasi sebagai terapi pra – bedah dapat dilakukan, dan digunakan tourniquet.


Intraoperatif

Tergantung pada ukuran dan asal lesi, keseimbangan cairan dan kehilangan darah perlu dimonitor ketat.

Post - operatif

Modifikasi aktivitas pasien disesuaikan dengan keadaan umum pasien, lokasi pembedahan, dan proses bedah dan rekonstruksi. Profilaksis untuk mencegah deep vein thrombosis perlu dilakukan.

Komplikasi

Komplikasi dapat bervariasi tergantung pada lokasi ABC. Komplikasi yang umum terjadi termasuk :

  • Perdarahan
  • Rekurensi
  • Infeksi luka operasi
  • Hematoma
  • Osteomyelitis
  • Kerusakan jaringan sekitar
  • Kerusakan epifisis
  • Emboli pulmoner

III.2.11. PROGNOSIS

Pada umumnya prognosis ABC biasanya baik. Beberapa pasien memerlukan terapi berulang karena rekurensi, yang merupakan masalah utama dalam terapi ABC. Tingkat kesembuhan berkisar 90 – 95%. Bila lesi muncul pada usia lebih muda, lempeng pertumbuhan terbuka, dan lokasi pada metafisis telah diasosiasikan dengan meningkatnya resiko rekurensi. Stadium ABC tidak menunjukkan perubahan tingkat rekurensi, namun penelitian menunjukkan bahwa lesi dengan tipe morfologi III menunjukkan tingkat rekurensi tertinggi. Sementara tipe I dan II lebih sering rekuren dibanding tipe III, IV, V.

Tingkat rekurensi juga berbeda – beda tergantung pada cara terapi yang diberikan. Pada kesimpulan terhadap berbagai metode terapi, tingkat rekurensi yang didapatkan adalah :

  • Iradiasi : 11.8%
  • Iradiasi dan kuretase : 14,3%
  • Kuretase dan bone graft : 30,8%
  • Kuretase dan kriobiopsi : 12,8%
  • Reseksi marginal : 7,4%
  • Reseksi luas : 0%

III.3. JUXTA ARTICULAR CYSTS / GANGLION CYSTS

          III.3.1. Gambaran Umum [21]

Kista ganglion merupkan tumor atau pembengkakan pada puncak sendi atau tendon. Kista ini terlihat seperti kantung berisi cairan. Di dalamnya terdapat material seperti gel, lengket, kental, tidak berwarna, dan jernih. Tergantung pada ukurannya, lesi dapat teraba halus atau seperti spons. Sebuah kista berukuran besar atau beberapa kista kecil dapat terbentuk. Kista kecil multipel dapat memberikan gambaran seperti lebih dari satu kista, tetapi biasanya kista multipel ini terhubung satu sama lain oleh tangkai yang berada pada jaringan yang lebih dalam.

Kista ganglion, yang disebut juga Bible cysts, lebih sering terdapat pada wanita, dan 70% terdapat pada usia 20 – 40 tahun. Pada kasus jarang, kista ini dapat ditemukan pada anak di bawah usia 10 tahun. Predileksi tersering adalah punggung pergelangan tangan (60 – 70%), sendi pergelangan tangan, dan sisi palmar pergelangan. Pada kista yang terdapat pada punggung pergelangan tangan, akan lebih jelas terlihat pada posisi punggung tangan fleksi. Tempat predileksi lainnya adalah :

  • Dasar jari pada telapak tangan, dimana kista muncul seukuran kacang.
  • Ujung jari, di bawah kutikula, disebut mucous cysts.
  • Sisi lateral lutut atau pergelangan kaki.

Etiologi pasti dari kista ganglion belum diketahui. Sebuah teori menyebutkan bahwa trauma menyebabkan jaringan pada sendi mengalami kerusakan dan membentuk kista – kista kecil, yang kemudian bergabung membentuk massa yang lebih besar. Teori lain menyebutkan bahwa kerusakan pada kapsul sendi atau tendon menyebabkan jaringan sendi mengalami bulging. [29]

Pergelangan tangan memiliki struktur anatomi yang kompleks, selain itu juga memiliki banyak tulang dan sendi. Tulang – tulang dan sendi – sendi ini memungkinkan tangan bergerak dan digunakan ke segala arah dan berbagai cara.

Pergelangan tangan disusun oleh 8 buah tulang – tulang kecil yang terpisah, disebut tulang – tulang karpal. Tulang – tulang karpal ini menghubungan 2 tulang lengan, yaitu radius dan ulna.

Ligamen menghubungkan dan menahan tulang – tulang pergelangan tangan bersama – sama. Ligamen ini memungkinkan tulang – tulang bergerak ke segala arah. Ligamen – ligamen bersatu membentuk kapsul sendi pergelangan. Kapsul sendi ini merupakan kantung berisi cairan yang mengelilingi tulang – tulang. Di dalam kapsul ini terdapat sendi – sendi. Kapsul sendi ini mengandung sedikitl lubrikan yang disebut cairan synovial.

Ganglion biasanya terhubung dengan tangkai jaringan ke kapsul sendi, tendon, atau tendon sheath. Ganglion pergelangan tangan melekat pada kapsul sendi.


 

III.3.2. Gejala Klinis [23]

Kista ganglion muncul sebagai massa dengan ukuran yang berubah – ubah. Biasanya memiliki konsistensi lunak dengan diameter 1–3 cm, tidak mobile. Tumor dapat terlihat sepanjang waktu, tampak tiba – tiba, atau menjadi kecil, menghilang, dan kemudian muncul kembali. Kebanyakan kista ganglion menyebabkan berbagai derajat nyeri, terutama yang terjadi setelah trauma akut atau berulang. Bila terdapat nyeri, biasanya nyeri bersifat kontinu dan memburuk bila sendi digerakkan. Bila kista melibatkan tendon, jari yang bersesuaian dapat terasa lemas.

 

 

 

 

 

 

 

Kista ganglion karena trauma. Pada kasus ini penderita datang setelah pergelangan tangan terhantam oleh pintu mobil.

 

 

 

III.3.3. Pemeriksaan Penunjang [24]

Pemeriksaan fisik seringkali cukup untuk membuat diagnosa kista ganglion. Bila diperlukan pemeriksaan lebih lanjut, dapat dilakukan aspirasi cairan dari kista dengan syringe, atau pemeriksaan USG. USG berguna untuk menentukan apakah tumor berisi  cairan atau solid. Selain itu juga mendeteksi apakah ada struktur arteri atau pembuluh darah yang menyebabkan tumor.

III.3.4. Terapi [25]

Kebanyakan kista ganglion (38 – 58%) dapat menghilang tanpa terapi samasekali. Bila tidak terdapat keluhan, dapat dilakukan aspirasi. Aspirasi dilakukan dengan menempatkan jarum ke dalam kista, menarik cairan keluar, dan menginjeksikan steroid. Kemudian pergelangan dibebat dengan elastic verband untuk mencegah sendi bergerak. Berbagai penelitian menunjukkan 74% penderita sembuh dengan 1 kali aspirasi.

Bila telah dilakukan 3 kali aspirasi, persentase kesembuhan meningkat menjadi 85%, khususnya pada kista yang terletak di punggung tangan.

Teknik aspirasi pada kista ganglion [19]:

  • Jarum 18 atau 22 gauge dan syringe 20 – 30 ml ditusukkan ke dalam kista. Cairan disedot perlahan.
  • Setelah cairan kista keluar, diinjeksikan kortikosteroid.
  • Daerah aspirasi dikompresi dengan elastic verband.

Terapi operatif diperlukan bila terdapat nyeri dan terdapat gangguan fungsi (terutama bila kista terdapat di tangan yang dominan), atau terdapat rasa kesemutan atau hipestesi pada tangan atau jari. Atau pada ganglion yang sangat besar, dilakukan eksisi. Untuk prosedur ini dilakukan anestesi lokal atau regional.

Terapi non – operatif [48]

Dorsal Wrist Ganglion

Dahulu terapi untuk jenis ganglion ini adalah dengan memecahkannya. Terapi dilakukan dengan menekan kista dengan benda berat, seperti buku yang sangat tebal. Tetapi tingkat kekambuhan sangat tinggi, sehingga terapi ini tidak digunakan lagi. Observasi seringkali sudah cukup pada ganglion pergelangan tangan karena karakteristiknya yang tidak nyeri dan tidak selalu semakin besar seiring waktu. 50% jenis ganglion ini dapat menghilang dengan sendirinya.

Selama observasi, dapat dilakukan ruptur tertutup dengan punksi sebagai terapi non – operatif untuk dorsal wrist ganglion ini. Pada prosedur ini, dinding kista dipunksi dengan jarin dan obat anti-inflamasi disuntikkan ke dalam kista. Tindakan ini dapat mengecilkan kista dan menghilangkan gejala. Tetapi ganglion masih dapat muncul kembali.

Volar Wrist Ganglion

Untuk jenis ini biasanya dilakukan observasi.

Terapi operatif

Dianjurkan bila terdapat keluhan nyeri atau terdapat gangguan aktivitas. Juga dianjurkan bila ganglion mengkompresi saraf dan menimbulkan gangguan motorik dan sensorik.

Dorsal Wrist Ganglion

Terdapat 2 pilihan terapi operatif :

-  Aspirasi

Menghisap cairan kista keluar dengan syringe. Prosedur ini memiliki tingkat keberhasilan kurang dari 50%.

-  Eksisi

Dilakukan pengeluaran kista seluruhnya. Prosedur ini efektif bila tangkai yang menghubungkan kista dengan kapsul sendi dan jaringan di sekeliling kapsul dikeluarkan. Untuk mengeluarkan dorsal wrist ganglion, dibuat insisi di punggung pergelangan tangan. Tendon yang melewati lapangan operasi dari punggung pergelangan tangan menuju jari di retraksi. Kemudian terlihat ganglion, ditelusuri hingga hingga mencapai tempat perlekatan ganglion dan kapsul sendi. Bila telah ditemukan, seluruh ganglion diangkat, termasuk area dimana ganglion terhubung dengan kapsul sendi. Bagian kapsul sendi ini dapat diperbaiki atau tidak dengan penjahitan. Kemudian luka operasi ditutup.

Volar Wrist Ganglion

Punksi dan aspirasi tidak dianjurkan pada volar wrist ganglion karena kemungkinan cedera saraf dan pembuluh darah. Metode operatif yang paling baik untuk jenis ganglion ini adalah eksisi. Pada dasarnya teknik operasi yang digunakan sama dengan dorsal wrist ganglion, tetapi pada volar ganglion lesi terletak sangat dekat dengan arteri radialis. Pada beberapa kasus ganglion bahkan mengelilingi arteri. Hal ini menyebabkan pengangkatan ganglion menjadi lebih sulit. Yang harus diperhatikan opelh operator adalah melindungi arteri radialis, sekaligus mengangkat seluruh kista hingga tempat perhubungannya dengan kapsul sendi.

 

Cairan seperti gel yang di aspirasi dari massa kistik pada pergelanga tangan, memastikan diagnosa kista ganglion.

 

 

 

 

 

 

 

 

 

 

 

 

Gambaran kista ganglion pada pemeriksaan USG.

 

III.4. Intraosseous Epidermoid Cysts

Pada tahun 1968, Lerner dan South wick melaporkan bahwa intraosseous epidermoid cysts terdapat dua kali lebih banyak pada pria dibanding wanita, dan dengan konsentrasi usia antara 25 – 50 tahun, khususnya ada orang dengan kegiatan – kegiatan aktif atau pekerjaan yang bersifat manual.[6]  Pada tahun 1970, Schajowicz menyatakan bahwa selain lebih banyak terdapat pada pria, predileksi utama lesi ini adalah ujung jari - jari tangan. Yang lebih sering terkena adalah tangan kiri, terutama jari tengah. [3],[4]. Predileksi lainnya adalah jari kaki, ulna, femur, tibia, sternum, dan ujung bagian amputasi. [6], [15], [7].  Tanda dan gejala yang tampak adalah membesarnya ukuran jari, dengan atau tanpa nyeri, kemerahan, dan melengkungnya kuku. Interval antara trauma dan munculnya gejala bervariasi dari beberapa minggu hingga beberapa tahun. [6], [3] ,[4].

Gambaran radiologis epidermoid cysts belum dapat dijadikan patokan diagnostik. Pada foto Roentgen terlihat lesi osteolytic yang dibatasi tepian tipis tulang sklerotik. Bila lesi telah mencapai ukuran tertentu akan terjadi penipisan korteks tulang sehingga dapat terjadi fracture. [6], [4]. Diagnosis banding yang mungkin adalah enchondroma, tumor glomus, aneurysmal bone cysts, metastasis, dan osteomielitis. Enchondroma merupakan tumor primer utama pada tangan yang predileksi utamanya adalah ruas phalanx proksimal atau medial. Pada glomus tumor terdapat nyeri hebat dan hipersensitivitas terhadap suhu dingin. Pada glomus tumor juga terdapat erosi akibat tekanan dan deformitas berbentuk konkaf dengan tepi rata. Pada aneurysmal bone cysts, gambaran seperti busa sabun terdapat pada metafisis tubular bone, menyebabkan nyeri dan limitasi pergerakan. Pada epidermoid, tepi yang tegas membedakan lesi ini dari lesi osteolytic pada osteomielitis dan metastase. [6], [4] ,[15]. Diagnosis pasti didapatkan melalui pemeriksaan histologis.

Secara makroskopik, kavitas unilokular kistik dari intraosseous epidermoid cyst mencapai diameter antara 1 – 20 mm. Membran luar setebal 1 – 5 mm dan mudah dipisahkan dari tulang sekitarnya. Pemeriksaan histologis menunjukkan dinding kista dengan epitel skuamosa berlapis dan lapisan granulosa berdiferensiasi baik yang dilapisi massa keratin yang mengalami laminasi. Isi kista terdiri dari keratin, sel – sel raksasa, kolesterol, dan sel – sel radang kronik. Tidak terdapat perbedaan histologik pada perbedaan lokasi anatomis kista. [6], [15].

Etiologi epidermoid cyst diperkirakan adalah :

  1. trauma dan proses sekunder akibat dari implantasi materi epidermis ke dalam tulang . [6],[7], [14],[9].
  2. formasi blastomatosa, berasal dari sisa sel – sel epithelial dari neural tube selama perkembangan embrionik [3],[5]
  3. Sebab iatrogenic berkaitan dengan prosedur bedah sebelumnya. [4]

Pada kista epidermoid intraoseus, kuretase dengan atau tanpa bone grafting sudah cukup adekuat. Amputasi sebaiknya tidak dijadikan sebagai terapi pilihan utama. [6],[3],[4],[7]. Pemeriksaan histologis sangat penting untuk menegakkan diagnosis kista epidermoid intraoseus dan harus dilakukan sesegera mungkin.

 

 

 

 

 

Lamina keratin pada kista yang dikelilingi epitel skuamous berlapis (Pewarnaan HE, pembesaran 20x)

 

 

 

Gambaran MRI menunjukkan nodul berbatas tegas dan erosi tulang di bawah kuku pada jari tengah kiri.

 

 

 

 

Foto Roentgen jari tengah tangan kiri menunjukkan lesi osteolitik berbatas tegas pada phalanx distal.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Foto Roentgen jempol kanan setelah kuretase dan pengisian defek dengan autogenous bone graft dari radius distal ipsilateral.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Foto Roentgen jempol kanan setelah kuretase dan pengisian defek dengan autogenous bone graft dari radius distal ipsilateral.

Gambaran jari jempol kanan menunjukkan lesi osteolitik dan fraktur pada phalanx distal.

Foto Roentgen jempol kanan setelah kuretase dan pengisian defek dengan autogenous bone graft dari radius distal ipsilateral.

 

IV. KESIMPULAN

Pada kasus tumor tulang, sebuah tim yang terdiri atas ahli bedah, radiologis diagnostik, onkologis, dan bila mungkin radioterapis, perlu bekerja sama dalam penanganan berbagai spektrum tumor. Diperlukan pengalaman yang baik khususnya dalam hal teknik operasi sebagai terapi tumor.

Langkah pertama dalam evaluasi pasien dengan tumor adalah riwayat penyakit. Keluhan utama biasanya berupa nyeri, massa, disabilitas, atau temuan tidak sengaja ketidaknormalan pada Roentgen. Usia dan jenis kelamin pasien dapat membantu menegakkan diagnosis. Pemeriksaan fisik harus mencakup evaluasi secara umum dan evaluasi lokal ekstremitas atau bagian tertentu yang dikeluhkan. Bila terdapat massa harus ditentukan lokasi, bentuk, konsistensi, mobilitas, nyeri, dan temperatur lokal.

Hal – hal yang perlu diperhatikan dalam kasus tumor adalah gambaran umum dari masing – masing jenis lesi, insidens, diagnosis, prognosis, dan metode terapi. Prinsip – prinsip terapi :

1. Evaluasi akhir pasien, termasuk biopsi dan penentuan stadium lesi harus dilakukan oleh ahli bedah ortopedi yang memegang tanggung jawab terhadap pasien.

2. Komunikasi yang baik dengan pasien dan pendamping pasien.

3. Terapi harus berdasarkan diagnosis akurat.

4. Prognosis untuk tiap pasien dengan atau tanpa terapi, dan pilihan terapi, harus disertai pertimbangan mengenai durasi atau kuantitas hidup pasien, dan quality of life pasien.

5. Keuntungan dan kerugian dari berbagai jenis terapi harus diinformasikan kepada pasien dan kerabatnya.

6. Secara ideal, diagnosis dan rencana terapi harus didiskusikan dengan seluruh anggota ahli onkologi interdisiplin.

7. Metode terapi operatif harus direncanakan berdasarkan seluruh data khususnya stadium penyakit.

 

DAFTAR PUSTAKA

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