0071643192.pdf

Hirshprung Disease

Disease characterized by functional obstruction of the colon due to failure of

cephalocaudal migration of parasympathetic myenteric nerve cells into the distal

bowel. The bowel fails to relax in response to distention →constipation. The agan-

glionic segment begins at the anus and extends varying distances proximally. There

is a greater incidence in males, those with a family history, and Down syndrome.

SYMPTOMS

Severity is variable but almost always presents shortly after birth. Infants pass little

or no meconium in the first 24 hours after birth. Constipation is subsequently

chronic or intermittent. Other symptoms include abdominal distention, bilious

emesis, diarrhea, listlessness, reluctance to feed, and developmental delay.

EXAM

■ Normal or contracted rectum on digital rectal exam with absence of feces

■ Distended abdomen, abdominal mass on palpation due to impacted stool

in the sigmoid colon

DIFFERENTIAL

Rectal or colonic atresia, meconium ileus, meconium plug syndrome, hypo-

thyroidism, electrolyte imbalances

DIAGNOSIS

■ Contrast enema demonstrates narrowed (aganglionic) colon, and proximal

bowel dilatation.

■ Biopsy showing lack of ganglion cells is definitive.

TREATMENT

Surgical resection of aganglionic bowel segment

COMPLICATIONS

Enterocolitis, postoperative fecal incontinence, postoperative episodic abdom-

inal distention, constipation.

Irritable Bowel Disease (IBD)

A chronic functional bowel disorder of unknown etiology that is characterized

by abdominal pain or discomfort and alterations in stool frequency and form.

There is also a lack of structural GI abnormality and underlying medical dis-

ease. The disorder is more common in women and the average age of onset is

20 years. Onset can follow a bout of gastroenteritis.

SYMPTOMS

■ Chronic or intermittent diffuse colicky or crampy abdominal pain

■ Abdominal distention and bloating

■ Temporary relief with passage of flatus or bowel movement

■ Association with emotional upset or stress

EXAM

Abdominal exam is generally benign but may reveal a cordlike mass in the LLQ.

ABDOMINAL AND GASTROINTESTINAL

EMERGENCIES