Hirschsprung Disease Questions & Answers

Updated: Aug 02, 2021
  • Author: Justin P Wagner, MD; Chief Editor: BS Anand, MD  more...
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Questions & Answers

Overview

What is Hirschsprung disease?

When was Hirschsprung disease first identified?

What is the pathophysiology of Hirschsprung disease?

How prevalent is Hirschsprung disease?

What are the racial predilections for Hirschsprung disease?

How does the prevalence of Hirschsprung disease vary by sex?

At what age is Hirschsprung disease diagnosed?

What is the prognosis of Hirschsprung disease?

What is the mortality and morbidity for Hirschsprung disease?

What are possible complications of Hirschsprung disease?

Presentation

What are the signs and symptoms of Hirschsprung disease?

Which physical findings suggest Hirschsprung disease in newborns?

DDX

What are the differential diagnoses for Hirschsprung Disease?

Workup

What is the role of a chemistry panel in the diagnosis of Hirschsprung disease?

What is the role of CBC count in the diagnosis of Hirschsprung disease?

What is the role of coagulation studies in the diagnosis of Hirschsprung disease?

What is the role of imaging studies in the diagnosis of Hirschsprung disease?

What is the role of anorectal manometry in the diagnosis of Hirschsprung disease?

What is the role of rectal biopsy in the diagnosis of Hirschsprung disease?

What is the role of suction rectal biopsy in the diagnosis of Hirschsprung disease?

Which histologic findings suggest Hirschsprung disease?

Treatment

Which specialists should be consulted if Hirschsprung disease is suspected?

What are the goals of medical care for Hirschsprung disease?

How should Hirschsprung disease be managed postoperatively?

What dietary restrictions should be implemented following surgery for Hirschsprung disease?

What activity restrictions should be implemented following surgery for Hirschsprung disease?

What is included in surgical management of Hirschsprung disease?

What are the American Pediatric Surgical Association (APSA) guidelines for managing postoperative obstructive symptoms in Hirschsprung disease?

What is the Swenson procedure for surgical treatment of Hirschsprung disease?

What is the Duhamel procedure for surgical treatment of Hirschsprung disease?

What is the Soave procedure for surgical treatment of Hirschsprung disease?

What is the role of anorectal myomectomy in the treatment of Hirschsprung disease?

Which surgical procedures are used for treatment of long-segment Hirschsprung disease?

What is the role of laparoscopy in the treatment of Hirschsprung disease?

How is a transanal pull-through procedure performed in the treatment of Hirschsprung disease?

Which treatments for Hirschsprung disease are under investigation?

What is included in postoperative monitoring for Hirschsprung disease?

Guidelines

What are the ERNICA guidelines for the management of rectosigmoid Hirschsprung disease?

Medications

Which medications are used in the treatment of Hirschsprung disease?

Which medications in the drug class Toxins are used in the treatment of Hirschsprung Disease?

Which medications in the drug class Antibiotics are used in the treatment of Hirschsprung Disease?