pathology 5 - Hirschsprung disease Other intestinal disorders Flashcards
In a patient with Hirschsprung disease, where is the dilated segment of the colon relative to the aganglionic segment?
Proximal
What would the biopsy of a patient with Hirschsprung disease likely demonstrate?
Lack of ganglionic cells (Auerbach and Meissner plexuses) that allow relaxation of the affected bowel
Hirschsprung disease results from the failure of what process?
Neural crest cell migration
A woman who heard about a condition called Hirschsprung disease wonders if her baby may have it. What symptoms would you ask about?
Inability to pass meconium after birth or chronic constipation in a child, bilious emesis, abdominal distention
A newborn has bilious emesis and fails to pass meconium after 48 hours. What is the treatment for this disease? How is it diagnosed?
Treated with resection; diagnosed by rectal suction biopsy (this is Hirschsprung disease)
Colonoscopy is performed on a newborn with Hirschsprung disease. What will be found proximal to the diseased segment of bowel?
A transition zone (dilated portion of bowel) proximal to the diseased (aganglionic) segment
Does Hirschsprung disease involve the rectum?
Yes
A newborn with bilious vomiting since birth has a distended abdomen. What is the likely diagnosis?
Duodenal atresia
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?
“Double bubble” sign (the bubbles are the stomach and the duodenum proximal to the atresia) (this is duodenal atresia)
A newborn presents with early bilious vomiting and a “double bubble” on X-ray. What chromosomal abnormality is associated with his disease?
Trisomy 21 (Down syndrome) (this newborn likely has duodenal atresia, which is associated with this chromosomal abnormality)
Which patients are most at risk for necrotizing enterocolitis?
Premature neonates because of their decreased immunity, & formula feeding also increases risk
A premature infant develops feeding intolerance; he has a distended abdomen and pneumatosis on x-ray. He has no bilious vomiting. Diagnosis?
Necrotizing enterocolitis
Where does ischemic colitis commonly occur?
The splenic flexure and distal colon (watershed areas between arterial territories that therefore have tenuous blood flow)
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?
The splenic flexure (LUQ) or distal colon (LLQ) (this is ischemic colitis, which affects watershed areas)
What is a common complaint of patients with ischemic colitis?
Pain after eating (increased metabolic demand in intestines and inability to appropriately increase blood flow leads to an ischemic state)
An elderly patient has painless hematochezia. After colonoscopy fails to detect any neoplasms, what test can confirm the most likely cause?
Angiography, as the most likely cause of the painless bleeding is angiodysplasia
A 70-year-old man complains of bright-red blood per rectum. Angiography shows tortuous dilation of vessels at the bleeding site. Diagnosis?
Angiodysplasia
A patient with angiodysplasia will typically complain of what symptoms?
Hematochezia, due to bleeding from tortuous dilation of vessels
Is angiodysplasia more common in children, young adults, or the elderly?
The elderly
A 48-y/o man with many past abdominal surgeries has a distended abdomen with decreased bowel sounds. What does this patient likely have?
Ileus, or intestinal hypomotility without obstruction
An opiate abuser complains of increased constipation and decreased flatus. On CT, no obstructions are noted. What does he have?
Ileus (intestinal hypomotility without obstruction)
causes of ileus
Abdominal surgeries, opiates, hypokalemia, sepsis
A recent abdominal surgery patient has stomach pain and vomiting. Laparotomy is performed and adhesions are seen. What do you see grossly?
Adhesions that are symptomatic may demonstrate well-demarcated necrotic zones
A man with a history of heart disease has abdominal pain and red “currant jelly” stools. Do you limit your differential to intussusception?
No, as acute mesenteric ischemia may also present with red “currant jelly” stools
