Latar Belakang Kelainan bawaan bertanggung jawab atas 11,3% kematian bayi di dunia. Di negara-negara berpenghasilan rendah dan menengah, neural tube defect merupakan jenis kelainan bawaan yang berperan penting dalam kecacatan dan kematian neonatus, yaitu sekitar 17% hingga 70% dari seluruh kematian akibat kelainan bawaan. Penelitian ini bertujuan untuk mengetahui profil karakteristik klinis dan tata laksana anak dengan neural tube defect di Departemen Bedah Saraf FKUI-RSCM pada tahun 2018–2022. Metode Desain penelitian ini adalah deskriptif observasional dengan metode penelitian potong lintang. Hasil Jenis kelamin yang paling banyak ditemukan adalah perempuan (51,7%). Jaminan kesehatan yang paling banyak digunakan adalah BPJS (94,8%) dengan rujukan paling banyak berasal dari fasilitas kesehatan di wilayah Jabodetabek (65,5%). Jenis NTD yang paling banyak ditemukan adalah myelomeningocele (25,9%), lipomyelomeningocele (24,1%), dan encephalocele occipital (20,7%). Faktor risiko maternal yang teridentifikasi adalah usia maternal > 35 tahun (12,1%). Proporsi ibu yang rutin mengonsumsi vitamin hamil sejak prekonsepsi sangat rendah (1,7%). Riwayat infeksi saat hamil ditemukan pada sebagian kecil kasus (3,4%) sedangkan riwayat kontak ibu dengan unggas atau hewan peliharaan cukup banyak ditemukan (22,4%). Waktu tahu hamil paling banyak saat usia kehamilan < 5 minggu (55,2%). Riwayat keluarga dengan NTD, riwayat diabetes pregestasional, dan riwayat konsumsi obat antiepilepsi maternal tidak ditemukan. Jenis operasi yang paling banyak dilakukan adalah rekonstruksi tutup defek. Sebagian besar operasi untuk NTD bersifat elektif (89,7%). Kesimpulan Jenis NTD yang paling banyak ditemukan adalah myelomeningocele, lipomyelomeningocele, dan encephalocele occipital dengan jenis operasi tersering berupa rekonstruksi tutup defek.

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Introduction Birth defects are responsible for 11.3% of infant deaths worldwide. In low- and middle-income countries, neural tube defect are a type of birth defect that plays an important role in neonatal disability and death, accounting for around 17% to 70% of all deaths due to birth defect. This study aims to determine the clinical and therapeutic profile of children with neural tube defects in the Department of Neurosurgery at Cipto Mangunkusumo Hospital, Indonesia, period 2018–2022. Method The design used in this research is descriptive observational with cross-sectional research method. Results The most common gender found was female (51.7%). The most widely used health insurance was BPJS (94.8%) with most referrals coming from health facilities in the Jabodetabek area (65.5%). The most common types of NTD were myelomeningocele (25.9%), lipomyelomeningocele (24.1%), and occipital encephalocele (20.7%). The maternal risk factor identified was maternal age > 35 years (12.1%). The proportion of mothers who regularly take pregnancy vitamins since preconception was very low (1.7%). A history of infection during pregnancy was found in a small number of cases (3.4%) while a history of maternal contact with poultry or pets was quite common (22.4%). The most common time the mothers knew that they’re pregnant was when the gestational age was < 5 weeks (55.2%). Family history of NTDs, history of pregestational diabetes, and history of maternal antiepileptic drug consumption were not found. The most common type of surgery performed was repair of the defect. Most operations for NTDs were elective (89.7%). Conclusion The most common types of NTD found were myelomeningocele, lipomyelomeningocele, and occipital encephalocele while the type of surgery most frequently performed was repair of the defect.