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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. Mosof them (90,4%) had shoraganglionosis. 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

  1. SPESIALIS-2023-435568-abstract.pdf  
  2. SPESIALIS-2023-435568-bibliography.pdf  
  3. SPESIALIS-2023-435568-tableofcontent.pdf  
  4. SPESIALIS-2023-435568-title.pdf