Spina Bifida – Penyebab, Gejala, Diagnosis, Pengobatan, Pencegahan
DokterSehat.com – Spina bifida adalah kondisi tulang belakang dan sumsum tulang belakang pada bayi tidak berkembang baik di dalam rahim, yang menyebabkan adanya celah di tulang belakang. Spina bifida merupakan jenis cacat tabung saraf. Tabung saraf adalah struktur yang berkembang menjadi otak dan sumsum tulang belakang bayi. Dalam spina bifida, bagian tabung saraf tidak menutup dengan benar, yang menyebabkan kerusakan sumsum tulang belakang dan tulang tulang belakang (vertebra).
Jenis Spina Bifida
Spina bifida terbagi menjadi empat jenis, dari yang paling parah hingga paling ringan. Berikut di antaranya:
1. Myelomeningocele
Adalah jenis spina bifida yang paling parah, karena kondisi kanal tulang belakang bayi tetap terbuka sepanjang beberapa tulang belakang di bagian belakang, kemungkinan berisiko sumsum tulang belakang dan selaput pelindung di sekitarnya mendorong keluar dan membentuk kantung di punggung bayi.
2. Meningocele
Jenis spina bifida yang serius lainnya di mana selaput pelindung di sekitar sumsum tulang belakang (meninges) mendorong keluar melalui tulang belakang – sumsum tulang belakang biasanya berkembang secara normal sehingga pembedahan biasanya dapat digunakan untuk mengangkat membran tanpa merusak saraf.
3. Spina bifida occulta
Selanjutnya adalah jenis spina bifida yang paling sering terjadi dan paling ringan. Sekitar 1 atau lebih tulang belakang tidak terbentuk dengan benar, tetapi celah di tulang belakang berukuran sangat kecil – spina bifida occulta biasanya tidak menyebabkan masalah dan kebanyakan orang tidak menyadari telah memilikinya.
4. Cacat tabung saraf tertutup
Jenis spina bifida yang satu ini, mungkin ada berbagai cacat potensial pada lemak, tulang, sumsum tulang belakang, atau meninges (sistem membran yang melapisi sistem saraf pusat). Dalam banyak kasus, jenis spina bifida ini tidak menunjukan gejala, namun dalam beberapa, terdapat kelumpuhan parsial dan inkontinensia usus dan urine.
Penyebab Spina Bifida
Belum jelas apa yang menjadi penyebab spina bifida hingga saat ini. Namun, para ilmuwan mengatakan bahwa spina bifida kemungkinan besar disebabkan oleh gabungan faktor genetik, lingkungan, dan gizi. Berikut di antaranya:
1. Asam folat
Spina bifida lebih mungkin terjadi jika ibu tidak memiliki asam folat yang cukup selama kehamilan, walaupun alasannya tidak jelas. Semua wanita dengan usia reproduksi harus memastikan asupan asam folat tercukupi.
2. Protein nabati, magnesium, zat besi, dan niasin
Asupan nutrisi yang rendah sebelum program kehamilan berhubungan dengan peningkatan risiko cacat tabung saraf dua hingga lima kali lipat.
3. Riwayat keluarga
Jika satu bayi dilahirkan menderita spina bifida, ada kemungkinan 4 persen saudara kandung di masa depan mungkin memiliki kondisi yang sama.
4. Obat-obatan
Salah satu obat-obatan seperti valproate, digunakan untuk mengobati epilepsi atau gangguan bipolar, kemungkinan berisiko lebih tinggi melahirkan bayi dengan cacat bawaan, seperti spina bifida.
5. Diabetes
Wanita yang menderita diabetes lebih cenderung melahirkan bayi menderita spina bifida, daripada yang tidak memiliki penyakit diabetes.
6. Obesitas
Penyebab spina bifida selanjutnya adalah berat badan berlebih atau obesitas. Wanita dengan indeks massa tubuh (BMI) 30 atau lebih, memiliki berisiko lebih tinggi melahirkan bayi dengan spina bifida.
Gejala Spina Bifida
Kedua jenis spina bifida, yaitu meningocele dan myelomeningocele yang tampak jelas saat lahir (lapisan otak meninges dan sumsum tulang belakang yang keluar dari tulang belakang). Selain adanya deformitas tulang belakang, gejala spina bifida juga dapat disebabkan oleh komplikasi dari penyakit ini sendiri. Komplikasi yang paling umum adalah sebagai berikut:
1. Gangguan mobilitas
Setelah satu kali operasi atau lebih untuk memperbaiki cacat, beberapa cacat hampir selalu menetap atau bertahan. Berbagai derajat kelumpuhan kaki, kelengkungan tulang belakang (skoliosis), pinggul, kaki, kelainan bentuk kaki, masalah dengan usus dan kontrol kandung kemih adalah cacat sisa (residual) yang paling umum.
2. Kelainan tulang punggung
Kelainan pada tulang punggung bagian bawah selalu disertai kelainan tulang belakang bagian atas (malformasi Arnold-Chiari), menyebabkan masalah pada koordinasi halus tubuh yang biasanya dapat diperbaiki dengan terapi fisik.
Kelainan tulang panggung, pinggul, kaki, dan kelainan bentuk kaki sering terjadi karena ketidakseimbangan kekuatan otot dan fungsi yang dihasilkan dari sebagian besar kelumpuhan residuan.
Sementara banyak anak-anak dengan myelomeningocele memiliki masalah pada sumsum tulang belakang menyebabkan cacat pada kaki, dislokasi pinggul, atau skoliosis. Masalah dapat memburuk seiring tubuh bertambah panjang sehingga terjadi penarikan sumsum tulang belakang.
3. Gangguan pencernaan
Masalah kandung kemih dan usus yang paling umum adalah ketidakmampuan untuk secara otomatis mengendurkan otot-otot (sfingter) yang menahan air kencing di kandung kemih dan tinja dalam rektum.
4. Hidrosefalus
Hidrosefalus (akumulasi cairan di otak) adalah masalah lain dari masalah residual yang sering terjadi, memengaruhi kebanyakan pasien dengan spina bifida. Tanpa pengobatan, cairan ekstra ini dapat menyebabkan masalah neurologis atau keterbelakangan mental. Namun, orang-orang ini memiliki kecerdasan yang normal jika hidrosefalus ditangani secara agresif. Hidrosefalus sering berulang secara bertahap setelah pengobatan.
5. Obesitas
Obesitas (karena tidak aktif) dan gangguan saluran kemih (karena drainase yang buruk) adalah komplikasi umum dari spina bifida.
6. Tulang rapuh
Patah tulang (fraktur) patologis (patah tulang yang terjadi secara tidak wajar karena kerapuhan tulang) terjadi dengan data persentase yang signifikan dari orang-orang dengan spina bifida. Cedera yang sangat kecil dapat memperburuk fraktur patologis, menyebabkan rasa sakit dan membawa fraktur untuk perhatian medis.
7. Kekurangan hormon pertumbuhan
Kekurangan hormon pertumbuhan (Somatotropin) yang mengakibatkan perawakan pendek sering terjadi pada orang dengan spina bifida. Rata-rata, spina bifida pada orang dewasa lebih pendek beberapa sentimeter daripada saudara atau teman sebayanya.
8. Gangguan belajar
Meskipun kebanyakan orang yang menderita penyakit spina bifida memiliki kecerdasan normal, namun mereka memiliki gangguan belajar. Perawatan yang memadai untuk hidrosefalus dan terapi fisik adaptif diperlukan untuk memungkinkan penderita mendapatkan kesempatan pendidikan.
9. Gangguan psikologis, sosial, dan seksual
Spina bifida pada orang dewasa, masalah psikologis, sosial, dan masalah seksual lebih sering terjadi dibandingkan pada populasi umum.
10. Alergi lateks
Alergi terhadap lateks (karet alami yang digunakan dalam sarung tangan medis, beberapa jenis elastis, balon, dan banyak item umum lainnya) sangat biasa pada penderita spina bifida. Hal ini dianggap sebagai akibat dari paparan intens untuk lateks di tahun-tahun awal kehidupan karena sering operasi dan prosedur medis lainnya. Reaksi alergi terhadap lateks dapat mengancam kehidupan.
Bahkan individu tanpa gejala yang jelas atau cacat dari spina bifida mungkin memiliki tanda-tanda halus atau ringan atau masalah neurologis. Beberapa orang memiliki bulu halus namun banyak pada kulit yang melapisi dasar tulang belakang. Lainnya memiliki pertumbuhan lemak disebut lipoma epidural yang terbentuk dalam kanal tulang belakang; ini biasanya tidak berbahaya tetapi dapat mengakibatkan penarikan dari sumsum tulang belakang.
Diagnosis Spina Bifida
Penyakit spina bifida dapat terdeteksi selama pemindaian ultrasound selama kehamilan kehamilan secara rutin. Pemindaian ini bisa dilakukan antara 18 dan 21 minggu kehamilan. Jika tes mengkonfirmasi bahwa janin menderita spina bifida, implikasinya akan didiskusikan dengan calon ibu.
Ini akan mencakup diskusi tentang kemungkinan masalah yang terkait dengan kondisi ibu dan anak, perawatan dan dukungan yang mungkin dibutuhkan anak jika Anda memutuskan untuk melanjutkan kehamilan, dan apa pilihan Anda untuk mengakhiri kehamilan, jika itu pilihan Anda.
Pengobatan Spina Bifida
Pengobatan untuk penyakit spina bifida tergantung pada keparahan kondisi. Berikut penaganan yang bisa dilakukan untuk mengobati spina bifida:
- Biasanya orang dengan spina bifida occulta tidak memerlukan pengobatan sama sekali.
- Anak-anak dengan meningocele biasanya memerlukan operasi pengangkatan kista dan biasanya sedikit yang bertahan, kalaupun ada biasanya dengan cacat.
- Anak-anak dengan myelomeningocele memerlukan pengobatan dan bantuan yang kompleks dan biasanya bantuan tersebut seumur hidup. Hampir semua dari mereka bertahan hidup dengan perawatan yang tepat dimulai segera setelah lahir. Kualitas hidup tergantung pada kecepatan, efisiensi, dan kelengkapan dengan yang perawatan yang disediakan.
Seorang anak yang lahir dengan myelomeningocele membutuhkan perawatan khusus berikut ini:
- Anak harus dipindahkan segera ke pusat di mana operasi yang baru lahir dapat dilakukan.
- Pengobatan dengan antibiotik dimulai sesegera mungkin ketika diagnosis myelomeningocele ditegakan; ini mencegah infeksi sumsum tulang belakang, yang bisa berakibat fatal.
- Operasi melibatkan penutupan pembukaan di sumsum tulang belakang dan menutupi saraf yang terlihat dengan otot dan kulit yang diambil dari kedua sisi punggung. Komplikasi yang paling sering adalah penambatan sumsum tulang belakang dan hidrosefalus, yang dapat memiliki konsekuensi yang sangat parah.
Setiap orang dengan spina bifida yang parah memerlukan perawatan intensif dan kompleks oleh tim terlatih dan terkoordinasi. Tim perawatan mencakup satu atau lebih dokter anak, ahli saraf, ahli bedah saraf, ahli bedah ortopedi, spesialis kedokteran rehab medik, ahli endokrin, urolog, terapis fisik, terapis okupasi, psikolog, perawat, ahli gizi, pekerja sosial, dan profesional lainnya.
Jika memungkinkan, individu dengan spina bifida harus menerima perawatan di sebuah klinik multidisiplin spina bifida khusus, di mana semua layanan yang diperlukan dapat disediakan secara terkoordinasi dan nyaman.
Tidak ada obat untuk spina bifida. Tujuan dari pengobatan untuk spina bifida adalah untuk memungkinkan individu untuk mencapai tingkat tertinggi dari fungsi dan kemandirian hidup. Pengobatan harus mengatasi kecacatan apapun, fisik, emosional, atau pendidikan, yang mengganggu potensi orang itu
Setelah operasi yang dijalankan ketika baru lahir, anak-anak dengan spina bifida berat menjalani penilaian rutin untuk mendeteksi cacat apapun di tubuhnya, masalah perkembangan, atau komplikasi lain yang mungkin memerlukan intervensi.
Penderita harus diawasi untuk tanda-tanda hidrosefalus, penambatan sumsum tulang belakang, kejang, obesitas, masalah kontrol usus atau kandung kemih, infeksi saluran kemih yang sering terjadi, gangguan belajar, masalah emosional dan psikososial, dan komplikasi lain dari spina bifida.
Perawatan multidisiplin akan memungkinkan deteksi sedini mungkin untuk komplikasi, dan pengobatan yang paling mungkin dapat menjadi efektif untuk mencegah kerusakan lebih lanjut.
Fokus pengobatan adalah pada mengembangkan kekuatan, mobilitas, dan kemandirian. Orang tua harus bekerja dengan ahli terapi fisik untuk belajar memaksimalkan kekuatan dan gerakan. Mereka harus mulai latihan ini segera setelah operasi pertama. Tidak hanya mempersiapan anak untuk berjalan, tetapi juga mencegah osteoporosis karena tulang yang jarang digunakan.
Pencegahan Spina Bifida
Kendati penyebab spina bifida tidak diketahui, sulit untuk mencegahnya, namun asam folat dan tes selama kehamilan dapat membantu. Berikut kiat-kiat untuk mencegah penyakit spina bifida:
1. Asam folat
Wanita usia reproduksi harus mengonsumsi 400 mikrogram (mcg) asam folat dalam sehari, nutrisi yang merupakan kunci untuk perkembangan janin yang sehat. Sumber asam folat termasuk sayuran hijau tua, sayuran berdaun, kuning telur, buah-buahan, dan produk sereal yang diperkaya. Bunda yang sedang hamil atau menjalani program hamil harus mengonsumsi suplemen asam folat 400 mcg setiap hari.
2. Tes
Tes yang sedang berlangsung untuk cacat tabung saraf dan masalah lain selama kehamilan juga dapat mengurangi risiko spina bifida dan memungkinkan tindakan pencegahan untuk diambil.
Spina bifida pada orang dewasa atau yang memiliki anak dengan kondisi ini berisiko lebih tinggi memiliki anak yang menderita spina bifida di kehamilan berikutnya. Penderita mungkin disarankan mengonsumsi asam folat ekstra sebelum hamil kembali.
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