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Hirschsprungs disease Dr. Jie Chen Children hospital of ZJU


Congenital megacolon HD is characterized by the absence of myenteric and submucosal ganglion cells in the distal alimentary

tract; resulting in decreased motility in the affected bowel segment Pathophysiology

Hirschsprung disease results from the absence of parasympathetic ganglion cells in the myenteric and submucosal plexus of the rectum and/or colon. Ganglion cells, which are derived from the neural crest, migrate caudally with the vagal

nerve fibers along the intestine. These ganglion cells arrive in the proximal colon by 8 weeks of gestational age and in the rectum by 12 weeks of gestational age. Arrest in migration leads to an aganglionic segment

Frequency One in 5000 live births Male-to-female ratio of 4:1 In 90 % are mature newborns In 1-6 % are

familial In 10%-30% are associated anomalies Classification

Classical HD (75% of cases): Rectosegmoid Long segment HD (20% of cases)

Total colonic aganglionosis (3-12% of cases)

rare variants include the following: Total intestinal aganglionosis

Ultra-short-segment HD (involving the distal rectum below the pelvic floor and the anus Embriology

Migration of neuroblasts on the 5-7 g.week On the 12. g.week they reach the rectum Myenteric plexus Submucous plexus Ganglions The process of maturation continues after

the birth Histopathology

Absence of ganglionic cells Hypertrophic cholinergic nerve trunks

Limited number of adrenergic fiber Clinical symptoms The disease can considered to be

incomplete intestinal obstruction The lenght of the aganglionic segment is variable The symptoms are variable too The symtoms appears in different ages

Clinical symptoms Symptoms in newborn age Fail to pass meconium (in 24 hours of life) Abdominal distension, but the abdomen is

palpable Vomiting The rectal tube cant be put easily After irrigation the signs and symptoms return again in a few days

Clinical symptoms Symptoms in newborn age(enterocolitis)

Life-threatening condition Diarrhea: it can be an early sign

Toxic megacolon Abdominal distension Bile-stained vomiting Fiver and signs of dehydration Rectal tube:explosive expulsion of gas and foulsmelling stools

Clinical symptoms Symptoms in infants Constipation Meteorism Palpable faecaloma Sometimes putrescent diarrhea

Ulceration, bleeding Hypoproteinaemia, anaemia Electrolyt disorders Clinical symptoms Symptoms in childhood

Gracile limbs Dilated drumlike belly Long history of constipation Defecation in 7-10 days Multiple fecal masses The stimulus of defecation is missing

Rectum is empty and narrow Examinations Plain abdominal radiographs Contrast enema

Anorectal manometry Rectal biopsy,histopathology Plain X ray: air/fluid levels,free air Contrast enema: -Narrow distal segment

-Funnel-shaped transition zone -Dilation of proximal colon -After 24-48 hours the contrast material is in the bowel -Mucosal irregularity (enterocolitis)

Anorectal manometry Anorectal pressure is elevated No relaxation of the internal sphincter It can be put through safely in newborn

age as well Hirschsprungs disease Biopsy and Histology Full-thickness strip-biopsy

Suction biopsy Suction biopsy Nitrergic innervation pattern (light microscopy, x100)

Normal colon Hirschsprungs disease Nitrergic innervation pattern

(light microscopy, x100) Hirschsprung's disease Total intestinal aganglionosis

Treatment: Decompression: introduce a rectal tube and irrigation Colostomy Definitive procedures

Closing of the stoma Treatment Transanal Endorectal Pull-Trough It can be performed safely in infant as well Generally one-stage surgery

No abdominal phase The anastomosis is happening in a safe place at the pectinate line Hirschsprungs disease

Hirschsprungs disease Hirschsprungs disease Enterocolitis

Enterocolitis accounts for significant morbidity and mortality in patients with Hirschsprung disease.

Patients typically present with explosive diarrhea, abdominal distention, fever, vomiting, and lethargy. Approximately 10-30% of patients with Hirschsprung

disease develop enterocolitis. Long-segment disease is associated with an increased incidence of enterocolitis. Treatment consists of rehydration, intravenous antibiotics and colonic irrigations. Post operative complications

Anastomotic leak Anastomotic stricture Intestinal obstruction Pelvic abscess Wound infection

Thank you for your attention !

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