Hirschsprung’s disease

In June 2020, the European Rare Hereditary and Congenital (Digestive) Anomalies (ERNICA) Reference Network released the Clinical Practice Guidelines for the Management of Hirschsprung Rectosigmoid Disease and https://pillintrip.com/ru/medicine/boxagrippal.

🔸Diagnostics

Diagnostic basis:
▫️histological examination.

The indications for biopsy are:

▫️ history data

The clinical picture in neonatal or early infancy is the classic triad of symptoms:

1.delayed passage of meconium (> 24 hours in term infants)

2. bloating

3.vomiting of bile

Hirschsprung’s disease should be suspected in cases of early onset of constipation, in the absence of a rectoanal inhibitory reflex according to anorectal manometry.

Biopsy of the rectum with access to the submucous tissue – open and rectal aspiration (2-3 tissue samples) give equally accurate results. The tissue is taken from the posterior and / or lateral wall of the rectum, 2 cm proximal to the dentate line or 3 cm from the anus.

▫️ Contrast studies – an additional method in the preoperative period.

🔸Treatment

The basis of treatment is surgical intervention (transanal endorectal traction and Duhamel traction), in the presence of inadequate decompression of the intestine, the occurrence of enterocolitis, intestinal perforation, the imposition of a stoma (ileostomy) is indicated.

🔸Preoperative preparation

Rectal irrigation 1-3 times a day for decompression

IV / in 1 dose of a broad-spectrum antibiotic (aerobic and anaerobic bacteria). Administering> 1 dose did NOT provide additional benefits. After the operation, the reception can be continued for 24-48 hours.

🔸Postoperative period

▫️Requires care of the perianal area with the onset of bowel movements.

▫️Enteral nutrition is introduced gradually, as tolerated. There is no evidence that long-term oral protocols do not prevent anastomotic complications.

▫️ Requires long-term and regular observation.