pathology 5 - Hirschsprung disease Other intestinal disorders

Question 1

In a patient with Hirschsprung disease, where is the dilated segment of the colon relative to the aganglionic segment?

Answer 1

Proximal

Question 2

What would the biopsy of a patient with Hirschsprung disease likely demonstrate?

Answer 2

Lack of ganglionic cells (Auerbach and Meissner plexuses) that allow relaxation of the affected bowel

Question 3

Hirschsprung disease results from the failure of what process?

Answer 3

Neural crest cell migration

Question 4

A woman who heard about a condition called Hirschsprung disease wonders if her baby may have it. What symptoms would you ask about?

Answer 4

Inability to pass meconium after birth or chronic constipation in a child, bilious emesis, abdominal distention

Question 5

A newborn has bilious emesis and fails to pass meconium after 48 hours. What is the treatment for this disease? How is it diagnosed?

Answer 5

Treated with resection; diagnosed by rectal suction biopsy (this is Hirschsprung disease)

Question 6

Colonoscopy is performed on a newborn with Hirschsprung disease. What will be found proximal to the diseased segment of bowel?

Answer 6

A transition zone (dilated portion of bowel) proximal to the diseased (aganglionic) segment

Question 7

Does Hirschsprung disease involve the rectum?

Answer 7

Yes

Question 8

A newborn with bilious vomiting since birth has a distended abdomen. What is the likely diagnosis?

Answer 8

Duodenal atresia

Question 9

A baby w/bilious vomiting has an X-ray to look for failure of recanalization in development. What finding do you look for to confirm this?

Answer 9

“Double bubble” sign (the bubbles are the stomach and the duodenum proximal to the atresia) (this is duodenal atresia)

Question 10

A newborn presents with early bilious vomiting and a “double bubble” on X-ray. What chromosomal abnormality is associated with his disease?

Answer 10

Trisomy 21 (Down syndrome) (this newborn likely has duodenal atresia, which is associated with this chromosomal abnormality)

Question 11

Which patients are most at risk for necrotizing enterocolitis?

Answer 11

Premature neonates because of their decreased immunity, & formula feeding also increases risk

Question 12

A premature infant develops feeding intolerance; he has a distended abdomen and pneumatosis on x-ray. He has no bilious vomiting. Diagnosis?

Answer 12

Necrotizing enterocolitis

Question 13

Where does ischemic colitis commonly occur?

Answer 13

The splenic flexure and distal colon (watershed areas between arterial territories that therefore have tenuous blood flow)

Question 14

An 80-year-old man with CAD presents has 6 months of weight loss due to pain after eating. Where do you suspect the pain is occurring?

Answer 14

The splenic flexure (LUQ) or distal colon (LLQ) (this is ischemic colitis, which affects watershed areas)

Question 15

What is a common complaint of patients with ischemic colitis?

Answer 15

Pain after eating (increased metabolic demand in intestines and inability to appropriately increase blood flow leads to an ischemic state)

Question 16

An elderly patient has painless hematochezia. After colonoscopy fails to detect any neoplasms, what test can confirm the most likely cause?

Answer 16

Angiography, as the most likely cause of the painless bleeding is angiodysplasia

Question 17

A 70-year-old man complains of bright-red blood per rectum. Angiography shows tortuous dilation of vessels at the bleeding site. Diagnosis?

Answer 17

Angiodysplasia

Question 18

A patient with angiodysplasia will typically complain of what symptoms?

Answer 18

Hematochezia, due to bleeding from tortuous dilation of vessels

Question 19

Is angiodysplasia more common in children, young adults, or the elderly?

Answer 19

The elderly

Question 20

A 48-y/o man with many past abdominal surgeries has a distended abdomen with decreased bowel sounds. What does this patient likely have?

Answer 20

Ileus, or intestinal hypomotility without obstruction

Question 21

An opiate abuser complains of increased constipation and decreased flatus. On CT, no obstructions are noted. What does he have?

Answer 21

Ileus (intestinal hypomotility without obstruction)

Question 22

causes of ileus

Answer 22

Abdominal surgeries, opiates, hypokalemia, sepsis

Question 23

A recent abdominal surgery patient has stomach pain and vomiting. Laparotomy is performed and adhesions are seen. What do you see grossly?

Answer 23

Adhesions that are symptomatic may demonstrate well-demarcated necrotic zones

Question 24

A man with a history of heart disease has abdominal pain and red “currant jelly” stools. Do you limit your differential to intussusception?

Answer 24

No, as acute mesenteric ischemia may also present with red “currant jelly” stools

Question 25

A patient with sepsis is diagnosed with intestinal hypomotility without obstruction. What treatment options may be of use for this patient?

Answer 25

Treat with bowel rest, electrolyte correction, and cholinergic drugs to stimulate intestinal motility (this is ileus)

Question 26

Signs of perforation

Answer 26

including pneumatosis intestinalis, free air in the abdomen, portal venous gas