Diagnosis and surgical approach of adult Hirschsprung's disease: About two observations and review of the literature. Case series

Abstract

IntroductionHirschsprung's disease (HD) is uncommon in adulthood. In this study, we describe the management of two cases of adult Hirschsprung's disease treated with transanal colonic pull-through procedure followed by a delayed coloanal anastomosis.Patients and methodsThis was a retrospective (December 2016 to Jun 2019) study included two cases of adult HD with confirmed Hirschsprung disease who underwent surgery at Zinder National hospital, Niger. The registration number is researchregistry 5174.ResultsThese were two patients aged 21 years (male) and 22 years (female) admitted to the emergency department with an acute bowel obstruction. The history finds a delayed passage of meconium at birth with a history of long-standing recurrent constipation since early childhood for the 2 patients. A lateral colostomy was performed urgently in both patients and the barium enema revealed a disparity of the sigmoid colon with corn shaped transition zone. Histologic examination of the biopsy specimen confirmed the diagnosis of HD. Surgery was done according to transanal endorectal pull-through procedure followed by delayed coloanal anastomosis. Patients were regularly followed over a period of 16 months. Constipation was gone, no continence problem was reported and quality of life was rated satisfactory.ConclusionThe discovery of Hirschsprung's disease is rare in adulthood. Transanal endorectal pull-through procedure followed by delayed coloanal anastomosis with conventional surgery is a suitable option for the treatment of HD and gives a good result.

Adult Hirschsprung’s disease presenting as chronic constipation: a case report

“…Diagnosis and treatment are conducted at an early age, in the first 5 years of life in more than 90% of cases. Consequently, the diagnosis of Hirschsprung’s disease in adulthood is rare [ 9 ]. The classic clinical symptoms including abdominal distension (in more than 90% cases), vomiting (more than 85% cases), which may be bilious, and failure to pass meconium during the first 24 h of life (more than 60% cases) [ 10 ].…”

Adult Hirschsprung’s disease: A case report and literature review

“…Chen et al, for example, mentions 300 reported cases up until 2006 [ 5 ], while Zhang and Ding [ 9 ], report 300 reported cases up until 2016 [ 9 ]. Adamou (2019) in his turn described a growth from 490 to 600 cases from 2009 till 2019 [ 10 ]. Part of this is due to the lack of medical access in certain parts of the world.…”

“…Fecal matter is palpable in over 50% due to fecal impaction being present in around 30% of the cases. In the study of Prato et al [ 13 ], he describes that 100% of his pediatric population with Hirschsprung’s disease presented with delayed meconium passage [ 5 , 8 , 10 ]. This is also noted in this literature review where Bakari et al [ 14 ], Ahmad et al [ 15 ], and Shitta et al [ 11 ] noted a history of delayed meconium passage in childhood.…”

Hirschsprung’s Disease in Adults Revealed by an Occlusive Syndrome

“…According to some authors, surgery can have good and satisfying outcomes by considering a less invasive technique using laparoscopic sigmoidorectal resection followed by a transanal lowering of the healthy colon without a stoma. This approach provides a better aesthetic and functional result than the techniques described above [ 17 ].…”