P471 Hirschsprung's disease in new born (about 17 cases). (June 2019)

• Record Type: Journal Article
• Authors: Regaieg, Chiraz; Kamoun, Dhouha; Hmed, Amel Ben; Zitouni, Hayet; Bouraoui, Amira; Charfi, Manel; Hmida, Nedia; Mhiri, Riadh; thabet, Afef Ben; Gargouri, Abdellatif
• Is Part Of: Archives of disease in childhood. Volume 104:(2019)Supplement 3
• Page Start: A341
• Page End: A341
• Publication Date: 2019-06
• Subjects: Children -- Diseases -- Periodicals; Infants -- Diseases -- Periodicals; 618.920005
• DOI: 10.1136/archdischild-2019-epa.807
• Languages: English
• ISSNs: 0003-9888

Abstract

Abstract : Introduction: Hirschsprung's disease is a congenital intestinal paralysis due to absence of ganglion cells in enteric plexuses. We aim to describe the specificities of the neonatal form. Patients and methods: It is a retrospective study of 17 cases of Hirschsprung's disease hospitalized in our unit between 2006 and 2018. Diagnosis was based on radiological and/or pathological signs. Results: A male predominance was noted. Two newborns were premature. Two newborns had a congenital heart disease. One of them had Trisomy 21. Another newborn had hypothyroidism. An emission delay of méconium (average of 46 hours) was noted in all cases. The disease was revealed by a lower digestive occlusion in 7 cases, an acute enterocolitis in 2 cases and a bowel perforation in one case. The contrast enema practiced in 15 cases, was pathognomic in 13 cases. Rectal biopsy performed in 6 cases, confirmed histological diagnosis in all cases. Surgical treatment was performed in 9 cases with a median time between symptoms and surgery of 19 days. It was a colo-anal lowering in 7 cases, a resection of the right colon with double colostomy in one case and a right transverse colostomy in four cases. Outcome was favorable in 12 cases. Four newborns died consecutively to sever congenital heart disease in one case and sever sepsis in other cases. Conclusion: Hirschsprung's disease is the most common cause of digestive occlusion in the newborn. The main complications in the neonatal form are acute enterocolitis and intestinal perforation.