Management and outcomes for long-segment Hirschsprung disease: A systematic review from the APSA Outcomes and Evidence Based Practice Committee

Abstract

Objective: Long-Segment Hirschsprung Disease (LSHD) differs clinically from short-segment disease. This review article critically appraises current literature on the definition, management, outcomes, and novel therapies for patients with LSHD.

Methods: Four questions regarding the definition, management, and outcomes of patients with LSHD were generated. English-language articles published between 1990 and 2018 were compiled by searching PubMed, Scopus, Cochrane Central Register of Controlled Trials, Web of Science, and Google Scholar. A qualitative synthesis was performed.

Results: 66 manuscripts were included in this systematic review. Standardized nomenclature and preoperative evaluation for LSHD are recommended. Insufficient evidence exists to recommend a single method for the surgical repair of LSHD. Patients with LSHD may have increased long-term gastrointestinal symptoms, including Hirschsprung-associated enterocolitis (HAEC), but have a quality of life similar to matched controls. There are few surgical technical innovations focused on this disorder.

Conclusions: A standardized definition of LSHD is recommended that emphasizes the precise anatomic location of aganglionosis. Prospective studies comparing operative options and long-term outcomes are needed. Translational approaches, such as stem cell therapy, may be promising in the future for the treatment of long-segment Hirschsprung disease.

Keywords: Aganglionosis; Hirschsprung; Level 3; Level 4; Level of Evidence; Long-segment; Outcome; Surgery.

Figure 1.. Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) methodology.

779 records were identified through database searches and 730 records remained after duplicates were removed. 150 full-text articles were assessed for inclusion and 66 articles were included in the qualitative synthesis. Some articles addressed more than one of the pre-identified questions

Figure 2.. Anatomic definitions of Long-segment Hirschsprung disease.

(A) Of 33 manuscripts, 3 defined LSHD as proximal to the splenic flexure (dotted line), 22 defined LSHD as proximal to the sigmoid/rectosigmoid (blue line) and 8 preferred to define the anatomic location of the most proximal level of aganglionosis (e.g. green arrows). (S = sigmoid, DC= descending colon, SF= splenic flexure, TC = transverse colon, HF= hepatic flexure, AC = ascending colon) (B) N=22 studies provided detailed information on length of aganglionosis for a total of 2450 patients. From this group of studies, the median length of aganglionosis was Short-Segment in 72% (range 64–83%), Long-Segment in 15% (range 5–24%), Total Colonic in 8% (range 1–20%), and Small Intestinal in 0% (0–11%). Data are shown as violin plots, with heavy dotted lines for the median, and light dotted lines for the 25% and 75% quartiles.