History and exam
Key diagnostic factors
common
weight loss
Patients often struggle to maintain weight despite intentional hyperphagia and nutritional supplementation.
The degree of weight loss is directly proportional to the reduction in absorptive capability of the bowel.
diarrhea
Results from increased osmolarity of the intestinal contents (mostly due to fat malabsorption), bile salt irritation of the colon, decreased intestinal transit time, increased gastric acid production, and decreased gut surface area for water reabsorption.
volume depletion
Due to excessive fluid loss from the bowel. Signs include dry mucous membranes, low skin turgor, tachycardia, and hypotension.
peripheral or presacral edema
Due to protein malabsorption, or decreased albumin synthesis due to intestinal failure-associated liver failure.
Other diagnostic factors
common
fatigue
A direct consequence of vitamin deficiency, weight loss, and volume depletion.
postprandial epigastric or right upper quadrant abdominal pain
Can occur as a result of cholecystitis.
Interruption of the enterohepatic circulation of bile salts causes an increase in biliary lipid concentrations, which can precipitate gallstone formation.
dysuria or renal colic
Due to the formation of calcium oxalate crystals in the kidney.
Oxalate normally binds to calcium and is passed in the stool. Fat malabsorption competes for calcium binding, leading to free oxalate absorption in the colon, which can then form calcium oxalate crystals in the kidney.
In one study of patients with SBS who had enterectomies and intact colons, symptomatic kidney stones developed in 24% of patients within 2 years.[19]
abnormal neurologic examination
Vitamin E or B12 deficiency can cause neuropathy. Examination may reveal orthostatic hypotension, asterixis, altered reflexes, or sensory deficits.
jaundice and pruritus
Key sign/symptom of liver failure secondary to long-term intestinal failure.
uncommon
dermatologic signs
Vitamin, zinc, and essential fatty acid deficiency are associated with a range of dermatologic signs.
Vitamin B2 deficiency: swelling and fissuring of the lips.
Vitamin C deficiency: bruising/ecchymoses at nontraumatic sites, spontaneous bleeds (also results from vitamin K deficiency).
Zinc deficiency: skin rash.
Essential fatty acid deficiency: "chicken skin" on the backs of the arms.
night blindness
Refers to an inability to see in dim light.
Due to vitamin A deficiency.
motor weakness or altered gait
Due to vitamin E or B12 deficiency.
proximal muscle weakness
Features of vitamin D deficiency.
excessive bleeding
Due to vitamin C or K deficiency. Vitamin K deficiency is more common.
confusion
Confusion can be a symptom of D-lactate acidosis, or can occur as a result of severe parenteral nutrition-related liver failure.
Risk factors
strong
bowel resection
The majority of patients with SBS have undergone bowel resection to treat Crohn disease, abdominal trauma, or ischemic bowel. Gastric and intestinal volvulus as a complication of bariatric surgery is becoming a more frequent finding. Children have usually undergone bowel resection to treat intestinal atresia, midgut volvulus, intussusception, or necrotizing enterocolitis.[9]
Morbidity is directly related to the functional surface area of the small bowel available for absorption, the location of remaining bowel, the level of intestinal motility, and the presence of a colonic anastomosis to aid in water and nutrient absorption. The highest-risk patients have a duodenostomy or jejunoileal anastomosis with less than 35 cm of residual small bowel, jejunocolic or ileocolic anastomosis with less than 60 cm of residual small bowel, or an end jejunostomy with less than 115 cm of residual small bowel.[11][17][18][19]
The colon has a critical role for water absorption and eventually nutrient absorption. In addition, the ileocecal valve provides a crucial means of slowing intestinal transit and maximizing the time available for the reabsorption of fluid, electrolytes, and nutrients. SBS occurring in patients who have had a colectomy, or lack a colonic anastomosis, is usually severe because these crucial abilities of the colon are lost.[14]
extensive abdominal radiation injury
Radiation can cause severe damage to both dividing and nondividing cells of the small intestine, resulting in a loss of function. Morbidity is directly related to the functional surface area of the small bowel available for absorption and the level of intestinal motility.
gastroschisis
Gastroschisis is a congenital defect of the abdominal wall that can produce bowel ischemia, leading to loss of functioning surface area for absorption. Ischemia is usually the result of constriction of the mesenteric artery by the defect. Morbidity is directly related to the functional surface area of the small bowel available for absorption and the level of intestinal motility.
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