Luaran Pasien Penyakit Hirschsprung Tipe Long-Segment dan Total Colon Aganglionosis Pasca Pull-Through di RSUP Dr. Sardjito Yogyakarta

ASTRI KHAIRANA WARDHANI, dr. Gunadi, Ph.D., Sp.BA.; dr. Junaedy Yunus, M.Sc., Ph.D.

2021 | Skripsi | S1 KEDOKTERAN

Latar Belakang: Penyakit Hirschsprung (HSCR), disebut juga megacolon congenital, merupakan kelainan kongenital yang disebabkan oleh kurangnya pergerakan kolon bagian distal karena hilangnya inervasi inhibisi interinsik karena tidak adanya sel ganglion parasimpatik. HSCR dibedakan menjadi segmen pendek, segmen panjang, dan segmen total (TCA). Pengkajian luaran pascaoperasi pasien dengan tipe segmen panjang dan segmen total masih jarang. Luaran pascaoperasi dievaluasi berdasarkan luaran fungsional, hirschsprung associated enterocolitis (HAEC), serta komplikasi. Tujuan: Untuk mengetahui luaran fungsional, HAEC, dan komplikasi pascaoperasi pasien penyakit Hirschsprung dengan tipe segmen panjang dan TCA. Metode: Penelitian ini merupakan penelitian deskriptif analisis dengan pengambilan data melalui rekam medis. Subjek penelitian adalah pasien HSCR dengan tipe long-segment dan TCA yang melakukan operasi pull-through di RSUP Dr. Sardjito Yogyakarta dengan usia kurang dari 18 tahun dari Januari 2013 sampai Agustus 2020. Luaran fungsional pasien kemudian dievaluasi menggunakan klasifikasi Krickenbeck yang menilai tiga aspek yaitu voluntary bowel movement (VBM), soiling, dan konstipasi. HAEC dievaluasi dengan menggunakan sistem skor HAEC. Hasil: Terdapat 19 pasien HSCR dengan tipe long-segment dan TCA yang melakukan operasi pull-through. Seluruh pasien memiliki luaran fungsional yang baik. Terdapat 7 pasien yang mengalami HAEC dengan batas diagnosis minimal 4, serta terdapat 3 pasien yang mengalami HAEC dengan batas diagnosis minimal 10. Dalam 19 pasien, terdapat 12 pasien yang mengalami komplikasi. 10 di antaranya mengalami komplikasi berupa infeksi luka operasi dan fistula enterokutan dan 2 di antaranya mengalami komplikasi berupa ruam popok. Kesimpulan: Lebih dari 50% pasien HSCR dengan tipe long-segment dan TCA memiliki luaran fungsional yang baik, tidak mengalami HAEC, serta memiliki komplikasi yang buruk.

Background: Hirschsprung's disease (HSCR), also called congenital megacolon, is a congenital disorder caused by a lack of movement of the distal colon due to loss of intrinsic inhibitory innervation due to absence of parasympathetic ganglion cells. HSCR is divided into short segment, long segment, and total segment (TCA). Postoperative outcome assessment of patients with long segment and total segment types is rare. Postoperative outcomes were evaluated based on functional outcome, hirschsprung associated enterocolitis (HAEC), and complications. Objective: To determine the functional outcome, HAEC, and postoperative complications of patients with Hirschsprung's disease with long segment and TCA types. Methods: This research is a descriptive analysis research by collecting data through medical records. The research subjects were HSCR patients with long-segment and TCA types who performed a pull-through operation at Dr. Sardjito Yogyakarta, aged less than 18 years from January 2013 to August 2020. The patient's functional output was then evaluated using the Krickenbeck classification which assesses three aspects, namely voluntary bowel movement (VBM), soiling, and constipation. HAEC was evaluated using the HAEC score system. Results: There were 19 HSCR patients with long-segment and TCA types who underwent pull-through surgery. All patients had a good functional outcome. There were 7 patients who experienced HAEC with a diagnosis limit of min 4, and there were 3 patients who had HAEC with a diagnosis limit of min 10. In 19 patients, there were 12 patients who had complications. 10 of them had complications in the form of surgical wound infection and enterocutaneous fistula and 2 of them had complications in the form of diaper rash. Conclusion: More than 50% of HSCR patients with long-segment and TCA types have good functional outcome, do not experience HAEC, and have bad complications.

Kata Kunci : Hirschsprung, luaran fungsional, voluntary bowel movement, soiling, konstipasi, HAEC, komplikasi pascaoperasi, segmen panjang, segmen total