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Allied disorders of Hirschsprung's disease

Definition
Allied disorders of Hirschsprung's disease are a group of conditions characterized by functional obstruction of the intestine despite the presence of ganglion cells in the terminal rectum.
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Pathophysiology
The pathophysiology of allied disorders of Hirschsprung's disease involves functional obstruction of the intestine, which can occur despite the presence of ganglion cells in the terminal rectum. This can be due to abnormalities in the ganglia or normal ganglia with other underlying issues.
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Epidemiology
A nationwide survey in Japan collected 355 cases of these disorders over a 10-year period.
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Disease course
Clinical manifestations can vary widely depending on the specific disorder, but they generally involve symptoms of intestinal obstruction, such as abdominal distension, vomiting, and constipation.
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Prognosis and risk of recurrence
The prognosis of these disorders can vary. Congenital hypoganglionosis, megacystis microcolon intestinal hypoperistalsis syndrome, and chronic idiopathic intestinal pseudo-obstruction have been associated with poor prognosis.
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Key sources
The following summarized guidelines for the evaluation and management of allied disorders ofHirschsprung's disease are prepared by our editorial team based on guidelines from the Ministry of Health, Labour and Welfare of Japan (MHLW 2018).
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Guidelines

1.Screening and diagnosis

Diagnosis: suspect the diagnosis of megacystis microcolon intestinal hypoperistalsis syndrome in patients with observed microcolon and megalocystis confirmed on cystography, CT, or ultrasound.
B
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  • Differential diagnosis

2.Classification and risk stratification

Prognosis: expect long-term survival with appropriate treatment in patients with isolated hypoganglionosis and megacystis microcolon intestinal hypoperistalsis syndrome with appropriate treatment.
B
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3.Diagnostic investigations

History and physical examination: elicit clinical history and perform physical examination to determine the duration of symptoms and bowel obstruction conditions in patients with suspected chronic idiopathic intestinal pseudo-obstruction.
B

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  • Diagnostic imaging

4.Diagnostic procedures

Biopsy: perform a full-thickness biopsy of the gastrointestinal tract, especially the small intestine and sigmoid colon, for a definitive diagnosis of isolated hypoganglionosis.
B
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5.Medical management

Pharmacologic therapy
Insufficient evidence to support the use of any medication for the management of patients with isolated hypoganglionosis.
I
Insufficient evidence to support the use of any of the following medications for the management of patients with chronic idiopathic intestinal pseudo-obstruction:
prucalopride
cisapride
antibiotics (erythromycin)
I

6.Nonpharmacologic interventions

Nutrition
Offer enteral and parenteral nutrition for nutritional management of patients with isolated hypoganglionosis, megacystis microcolon intestinal hypoperistalsis syndrome, and chronic idiopathic intestinal pseudo-obstruction.
B
Recognize that adequate nutritional support is required for a long period of time in many patients with isolated hypoganglionosis, megacystis microcolon intestinal hypoperistalsis syndrome, and chronic idiopathic intestinal pseudo-obstruction.
B

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  • Probiotics

  • Herbal products

7.Therapeutic procedures

Antegrade continence enema: consider performing antegrade continence enema and antidromic enema to improve defecation in patients with chronic idiopathic intestinal pseudo-obstruction.
C

8.Surgical interventions

Intestinal decompression: consider performing upper jejunostomy in patients with isolated hypoganglionosis.
C
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  • Radical surgery

  • Small bowel transplantation