Faktor Prognosis Terhadap Gejala Obstruksi Persisten Pasca Operasi Duhamel, Transanal Endorectal Dan Swenson Like Pull-through Pada Pasien Dengan Penyakit Hirschsprung
Naisya Balela, dr.Gunadi, Ph.D, Sp.BA; dr.Andi Dwihantoro, Sp.B, Sp.BA(K)
2023 | Tesis-Spesialis | SP ILMU BEDAH ANAK
Latar
Belakang:
Penyakit
Hirschsprung
adalah
suatu penyakit
genetik yang
kompleks,
ditandai dengan tidak adanya ganglion enterik pada submukosa dan pleksus
myenterikus yang
bervariasi
sepanjang
usus bagian distal. Kebanyakan pasien mengalami perbaikan pasca pull-through, namun beberapa diantaranya mengalami gejala obstruksi persisten. Kami bertujuan mengidentifikasi faktor prognosis terhadap
gejala obstruksi persisten pasca operasi Duhamel, transanal endorectal dan Swenson
like pull-through pada pasien dengan penyakit Hirschsprung di institusi kami. Metode: Penelitian ini menggunakan desain retrospektif. Kami menganalisis rekam
medis pasien penyakit Hirschsprung dengan atau tanpa gejala obstruksi persisten pasca
Duhamel, transanal endorectal, dan Swenson like pull-through yang datang ke rumah
sakit dari Januari 2017 hingga Januari 2022.
Hasil: Seratus empat belas pasien masuk dalam penelitian: 79 laki-laki dan 35 perempuan. Kebanyakan pasien (90,4%) memiliki short aganglionosis. Pasien yang menjalani Duhamel (n = 38), transanal endorectal (n=63), and transanal Swenson like pull-through (n = 13). Sebanyak (21.9%) mengalami obstruksi persisten pasca operasi Duhamel, transanal endorectal dan Swenson like pull-through. Teknik pull-through dan usia saat operasi definitif berhubungan kuat dengan gejala obstruksi persisten pasca pull-through (masing-masing, p=0,032 dan 0,019), sedangkan jenis kelamin, tipe aganglionosis, global developmental delay dan sindrom Down, tidak (masing-masing, p=0,837; 0,525; 0,647 dan 0,301). Analisis multivariat menunjukkan bahwa usia saat operasi definitif merupakan faktor independent yang kuat terhadap kejadian gejala obstruksi persisten pasca pull-through (OR = 3.41 [95% CI 1.18-9.91]; p = 0.02).
Kesimpulan: Frekuensi gejala obstruksi persisten pasca pull-through di institusi kami relatif rendah. Selain
itu, pasien yang
menjalani
pull-through
diusia muda
lebih
mungkin mengalami gejala obstruksi persisten.
Background: Hirschsprung disease is a complex genetic disorder characterized by the
lack of enteric ganglia in the submucosal
and myenteric plexuses, along variable
portions
of
the distal gut. Most children had a good result after pull-through, but some
of
them had a persistent obstructive symptoms. We aimed to identify prognostic factors for persistent obstructive symptoms after Duhamel,
transanal endorectal, and Swenson like pull-through in patients with Hirschsprungs
disease in our institution.
Methods: This study was a retrospective design. We analyzed the medical records of
Hirschsprungs disease patients with
or without persistent obstructive symptoms after
Duhamel, transanal endorectal, and Swenson like pull-through that admitted to our hospital from January 2017 to January 2022.
Results: One hundred and fourteen patients were included in this study: 79 males
and
35
females. Most of them (90,4%) had short aganglionosis. They underwent the Duhamel (n = 38), transanal endorectal (n=63), and Swenson like pull-through (n =13). Twenty one point nine (21,9%) patient showed persistent obstructive symptoms
after Duhamel, transanal endorectal, and Swenson like pull-through. Pull-through
approach and age at definitive surgery
were strongly associated with persistent obstructive symptom
after pull-through (p=0,032 and 0,019, respectively), while sex,
aganglionosis type, presence of global developmental delay and Down syndrome were not (p=0,837; 0,525; 0,647 and 0,301,
respectively). Multivariate analysis showed that age at definitive surgery was a strong independent factor for persistent obstructive symptoms after pull-through. (OR = 3.41 [95% CI 1.18-9.91]; p = 0.02).
Conclusions: The frequency of persistent obstructive symptoms after pull-through in
our
institution is considered low. Moreover, patients who underwent pull-through at a younger age might more likely suffer from
persistent obstructive symptoms.
Kata Kunci : usia saat operasi definitif, penyakit Hirschsprung, gejala obstruksi persisten, pull-through, faktor prognosis