Management and outcomes for long-segment Hirschsprung disease: A systematic review from the APSA Outcomes and Evidence Based Practice Committee.
J Pediatr Surg
; 56(9): 1513-1523, 2021 Sep.
Article
en En
| MEDLINE
| ID: mdl-33993978
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.Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Enterocolitis
/
Enfermedad de Hirschsprung
Tipo de estudio:
Observational_studies
/
Qualitative_research
/
Risk_factors_studies
/
Systematic_reviews
Aspecto:
Patient_preference
Límite:
Humans
Idioma:
En
Revista:
J Pediatr Surg
Año:
2021
Tipo del documento:
Article