GAMBARAN KARAKTERISTIK PENYAKIT HIRSCHSPRUNG’S DI RSUD GUNUNG JATI CIREBON PERIODE 2020–2022

Maryzka Andini; Dimas Aryo Kusumo; Iwan Hermawan; Ineu Nopita; Zulkifli Ahmad; Triono Adi Suroso

Authors

Maryzka Andini Fakultas Kedokteran UGJ Cirebon, Indonesia
Dimas Aryo Kusumo Fakultas Kedokteran UGJ Cirebon, Indonesia
Iwan Hermawan Fakultas Kedokteran UGJ Cirebon, Indonesia
Ineu Nopita Fakultas Kedokteran UGJ Cirebon, Indonesia
Zulkifli Ahmad Fakultas Kedokteran UGJ Cirebon, Indonesia
Triono Adi Suroso Fakultas Kedokteran UGJ Cirebon, Indonesia

Keywords:

Hirschsprung's disease, surgery, total sampling

Abstract

ABSTRAK

Latar Belakang: Penyakit Hirschsprung’s adalah terjadinya pembesaran usus yang tipikal sepanjang bagian proksimal yang mengalami aganglionosis coli. Insiden penyakit Hirschsprung’s di dunia adalah 1:5000 kelahiran hidup dengan angka kematian berkisar antara 1–10%. Tujuan: Mengetahui gambaran jenis kelamin, usia, usia gestasi, usia saat dilakukan Tindakan, gambaran gejala klinis, Tindakan operasi dan komplikasi pasien penderita Hirschsprung’s di RSUD Gunung Jati Kota Cirebon Metode: Desain penelitian ini deskriptif retrospektif. Data yang digunakan adalah data sekunder berupa rekam medis pasien Hirschsprung’s di RSUD Gunung Jati periode 2020-2022. Teknik pengambilan sampel yang digunakan adalah total sampling. Hasil: Berdasarkan uji univariat didapatkan kejadian penyakit Hirschsprung’s terbanyak pada kategori usia 0-6 bulan (37,1%) dengan jenis kelamin laki-laki (60,0%), pasien berdasarkan usia gestasi preterm (8,6%), usia saat operasi 0-6 bulan (37,1%), gambaran klinis yang terbanyak konstipasi (55,7%), Tindakan operasi yang paling sering dilakukan yaitu operasi Swenson (77,1%) dan pasien dengan komplikasi infeksi luka operasi (7,1%). Simpulan: Gambaran penyakit Hirschsprung’s yang didominasi paling banyak berdasarkan karakteristik penyakit yaitu pada kategori usi 0-6 bulan (37,1%) dengan jenis kelamin laki-laki (60,0%)

ABSTRACT

Background: Hirschsprung's disease is the typical occurrence of intestinal enlargement along the proximal part which has aganglionosis coli. The incidence of Hirschsprung's disease in the world is 1:5000 live births with a mortality rate ranging from 1–10%. Aim: Knowing the description of gender, age, gestational age, age at the time of action, description of clinical symptoms, surgical procedures and complications of patients with Hirschsprung's at Gunung Jati Hospital, Cirebon City. Methods: This research design is descriptive retrospective. The data used is secondary data in the form of medical records of Hirschsprung's patients at Gunung Jati Regional Hospital for the 2020-2022 period. The sampling technique used is total sampling. Results: Based on the univariate test, the highest incidence of Hirschsprung's disease was found in the age category 0-6 months (37.1%) with male sex (60.0%), patients based on preterm gestational age (8.6%), age at surgery 0-6 months (37.1%), the most clinical picture is constipation (55.7%), the most frequently performed surgery is Swenson surgery (77.1%) and patients with surgical wound infection complications (7.1%) %). Conclusion: The most dominant description of Hirschsprung's disease is based on disease characteristics, namely in the 0-6 month age category (37.1%) with male sex (60.0%)

References

Daftar Pustaka

Langer JC. Hirschsprung disease. Curr Opin Pediatr. 2013;25(3):368– 74.

Tjaden NE, Trainor PA. The Developmental Etiology and Pathogenesis of Hirschsprung disease. Natl institutes Heal. 2014;162(1):1–15.

Ali A, Haider F, Alhindi S. The Prevalence and Clinical Profile of Hirschsprung’s Disease at a Tertiary Hospital in Bahrain. Cureus. 2021;13(1):1–11.

Wilanda IBAK, Dewi IGASM, Winarti NW, Sriwidyani NP. KarakteristikKlinikopatologi Penyakit Hirschsprung di RSUP Sanglah Denpasar Tahun 2019-2020. J Med Udayana. 2022;11(11):2020–3.

Palissei AS, Ahmadwirawan A, Faruk M. Hirschsprung’s disease: epidemiology, diagnosis, and treatment in a retrospective hospital-based study. J thee Med Sci (Berkala Ilmu Kedokteran). 2021;53(3):127–34.

Torroglosa A, Alves MM, Fernández RM, Antiñolo G, Hofstra RM, Borrego

S. Epigenetics in ENS development and Hirschsprung disease. Dev Biol [Internet]. 2016;417(2):209–16.

Maidah SA. Gambaran Karakteristik Penyakit Hirschprung di RSUD Al- Ihsan Bandung Periode 1 Januari 2016 - 30 September 2019 [Internet]. Universitas Islam Bandung; 2020.

Peyvasteh M, Askarpour S, Ostadian N, Moghimi MR, Javaherizadeh

H. Diagnostic Accuracy of Barium Enema Findings in Hirschsprung’S Disease. Arq Bras Cir Dig. 2016;29(3):155–8.

George W. Holcomb, J., Patrick Murphy SDSP. Holcomb and Aschcrafts pediatrics surgery. Pediatrics Surgery. 2020. 621–627 p.

Townsend CM, Beauchamp RD, Evers BM, Mattox KL. Sabiston: Textbookof Surgery: the Biological Basis of Modern Surgical Practice, Twenty FirstEdition. 2022. 1–2100 p.

Henderson D, Zimmer J, Nakamura H, Puri P. Hirschsprung’s disease

in twins: a systematic review and meta-analysis. Pediatr Surg Int. 2017;33(8):855–9.

Huang WK, Li XL, Zhang J, Zhang SC. Prevalence, Risk Factors, and Prognosis of Postoperative Complications after Surgery for Hirschsprung Disease. J Gastrointest Surg. 2018;22(2):335–43.

Suryandari AE. Analisis Faktor Yang Mempengaruhi Hirschsprung Di Rumah Sakit Prof. Dr. Margono Soekarjo Purwokerto. J Publ Kebidanan AKBID YLPP Purwekoerto. 2017;1(1):8–18.

Corputty ED, Lampus HF, Monoarfa A. Gambaran Pasien Hirschsprung Di Rsup Prof. Dr. R. D. Kandou Manado Periode Januari 2010 – September 2014. e-CliniC. 2015;3(1).

Ritana A. Maternal Risk Factors For Hirschsprung-Associated Enterocolitis(HAEC) Post Soave Procedure in Dr. Sardjito Hospital Yogyakarta AZMI RITANA, r. Gunadi, Sp.BA, Ph.D ; Dr. dr. Akhmad Makhmudi, Sp.B, Sp.BA [Internet]. Gadjah Mada University; 2017.

Cinel L, Ceyran B, Güçlüer B. Calretinin immunohistochemistry for the diagnosis of Hirschprung disease in rectal biopsies. Pathol Res Pract. 2015;211(1):50–4.

Octavia PD, Darmajaya IM. Teknik Operasi Dua Tahap Pada Kasus Penyakit Hirschsprung Diagnosis Terlambat Di Rsup Sanglah: 1 Studi Deskriptif Tahun 2010-2012. E-Jurnal Med Udayana [Internet]. 2014;

Gao T, Xu W, Sheng Q, Xu T, Wu W, Lv Z. Clinical outcomes and risk factors for postoperative complications in children with Hirschsprung ’ s disease. Am J Transl Res [Internet]. 2022;14(7):48307.

Askarpour S, Peyvasteh M, Imanipour MH, Javaherizadeh H, Hesam S. Complications After Transabdominal Soave’s Procedure In Children With Hirschsprung’s Disease. 2019;32(1):10–1.

Elsherbeny M, Abdelhay S. Obstructive complications after pullthrough for Hirschsprung ’ s disease : different causes and tailored management. Elsherbeny Abdelhay Ann Pediatr Surg. 2019;15(2).

Wang H, Li Q, Zhang Z, Xiao P, Li L and Jiang Q (2019) Functional Studies on Novel RET Mutations and Their Implications for Genetic Counseling for Hirschsprung Disease. Front. Genet

Steele SR, Hull TL, Hyman N, Maykel JA, Read TE, Whitlow CB, editors. The ASCRS Textbook of Colon and Rectal Surgery. 4th ed. 2022. Cham: Springer International Publishing; 2022.

CLAVIEN PA, Sarr MG, Fong Y, Miyazaki M, editors. Atlas of Upper Gastrointestinal and Hepato-Pancreato-Biliary Surgery. 2nd ed. 2016. Berlin, Heidelberg: Springer Berlin Heidelberg; 2016.