Short bowel syndrome K91.2

The first successful major small bowel resection was performed by Koeberle in 1880. The first clinical description of short bowel syndrome dates back to 1935 by Haymond. Dudrick and Wilmore developed total parenteral nutrition in the 1960s.

Short bowel syndrome (KDS) is defined as a small bowel length of less than 200 cm in adults from the Treitz ligament onwards (Dabsch 2025 / Hilberath 2025 / Pironi 2023).

KDS is one of the malabsorption disorders (Massironi 2020).

A distinction is made between 3 anatomical subtypes of KDS:

• Type 1: terminal jejuno-ileostomy
• Type 2: jejuno-ileocolic anastomosis
• Type 3: jejuno-ileal anastomosis with preserved ileocecal valve and completely preserved colon (Dabsch 2025)

In KDS itself, a distinction is made between:

• Intestinal insufficiency

Patients affected by this can compensate for the losses with optimal pharmacological and dietary therapy (Dabsch 2025)

• Intestinal failure

These patients can only survive with parenteral support (Dabsch 2025)

Short bowel syndrome (KDS) can (rarely) be congenital (e.g. microvillous inclusion disease) or acquired through resection of sections of the small intestine (Kasper 2015).

KDS is a rare clinical picture. The prevalence in Europe is estimated at 1.4 cases per 1 million inhabitants (Dabsch 2025). Women tend to be affected more frequently than men (Hilberath 2025).

However, KDS is the most common cause of chronic intestinal failure in both children and adults (Hilberath 2025).

In most cases, the causes of KDS are acquired (Dabsch 2025).

Congenital KDS can occur due to e.g.:

• microvillous inclusion disease (Kasper 2015)
• small bowel atresia
• malrotations (Dabsch 2025)
• Gastroschisis with or without atresia
• Abdominal wall defects
• Congenital short bowel syndrome (Hilberath 2025)

Acquired KDS occurs:

• in children due to:
  • necrotizing enterocolitis in approx. 30 % of those affected (Dabsch 2025)
• in adults due to:
  • venous or arterial mesenteric ischemia in approx. 1/3 of those affected
  • Small bowel resection in e.g. Crohn's disease, chronic inflammatory bowel disease, intestinal fistulas
  • Radiation enteritis
  • Volvulus (Dabsch 2025)
  • Intestinal intussusception
  • Trauma (Hilberath 2025)

The reduction in the absorptive surface of the intestine primarily results in malabsorption. This malabsorption leads to restrictions in both macro- and micronutrient and/or fluid intake (Dabsch 2025).

The postoperative adaptation process is divided pathophysiologically into 3 phases:

• Acute phase (hypersecretory phase): It usually lasts a few weeks (up to months) and is characterized by massive enteral losses of electrolytes, nutrients and fluids.
• Adaptation phase (physiological adaptation process): This follows in the subsequent 1-2 years after the acute phase. During this time, the remaining intestine increasingly takes over the absorptive functions of the missing section of the intestine. This leads to structural adaptations of the small intestinal mucosa with hypertrophy of the villi and a deepening of the crypts.
• Stable phase (chronically adapted phase): In this phase, the maximum of adaptation is reached (Dabsch 2025).

Around 1/3 of those affected are children. Only around 20% of KDS patients fall ill outside the newborn population (Dabsch 2025).

Overall, the clinical presentation sometimes varies due to the different underlying diseases and anatomical differences (Dabsch 2025)

The following symptoms can occur due to short bowel syndrome:

• Fatigue
• Concentration disorders
• Metabolic derailments
• Night blindness
• Diarrhea (Dabsch 2025)
• Megaloblastic anemia
• Chologenic steatorrhea / diarrhea due to loss of bile acids with
  • Increased risk of gallstones and calcium oxalate kidney stones
• Malnutrition with
  • osteoporosis
  • Cachexia
  • Vitamin A, D, E and K deficiency
  • Lack of potassium, magnesium (Herold 2025 / Dabsch 2025)

The diagnosis can be made postoperatively by measuring the remaining intestinal length using entero-MRI / CT. It has been shown that the intestinal length measured postoperatively correlates well with the intraoperative measurement. For this reason, every surgical report after bowel resection should include the residual small bowel length (Dabsch 2025).

Regular laboratory checks are strongly recommended in order to detect deficiency symptoms and complications as early as possible (Dabsch 2025).

As complications contribute significantly to the mortality and morbidity of patients, they should be recognized and treated as early as possible (Dabsch 2025).

Possible complications include:

• Catheter infections in patients who are fed intravenously
• Catheter-associated thromboses (Dabsch 2025)
• Vitamin D deficiency (Kasper 2015)
• Zinc deficiency (Herold 2025)
• Renal insufficiency up to and including kidney failure
• Nephrolithiasis: Magnesium citrate can be added to drinks here
• Hepatopathy up to liver cirrhosis: This is found in approx. 15 - 40 % of all patients with long-term parenteral nutrition and chronic intestinal failure
• Cholecystolithiasis
• Small intestinal bacterial overgrowth (SIBO): The disturbed intestinal motility can lead to adhesions, stenoses, dilatation or the appearance of blind intestinal loops. An attempt at therapy with Rifaximin 1200-1600 mg /d for 14 days often helps here
• Osteoporosis: Annual screening of bone density measurement is recommended
• Psychological consequences: These should not be underestimated in this disease, therefore early consultation of psychiatrists and psychologists, but also support from self-help groups (Dabsch 2025)

The treatment of patients with KDS is complex and requires interdisciplinary collaboration, including consultation with a specialist diabetologist (Dabsch 2025).

In children whose growth process is not yet complete, regular monitoring of growth and cognitive development is therefore essential in order to be able to intervene at an early stage (Jannasch 2025).

In the postoperative acute phase, also known as the hypersecretory phase, the main therapeutic focus is usually on the large loss of fluid and electrolytes. Sometimes considerable amounts of parenteral fluid of 4-8 liters must be administered. In this phase, proton pump inhibitors or H2 antagonists help to reduce gastric hypersecretion. However, both drugs should be reduced in the further course and ultimately discontinued (Dabsch 2025).

Motility-inhibiting drugs such as loperamide can be administered in significantly higher doses than usual (up to 32 mg / d). In addition, the administration of pancreatic enzymes (even with normal pancreatic function) can optimize fat digestion (Dabsch 2025).

Parenteral support for food intake of almost 100 % is available:

• in type 1 (terminal jejuno-ileostomy) with a residual small intestine length of < 90 cm
• in type 2 (jejuno-ileocolic anastomosis) with a residual small intestine length of < 60 cm
• in type 3 (jejuno-ileal anastomosis with preserved ileocecal valve and completely preserved colon) with a residual small bowel length of < 30 cm (Dabsch 2025)

The influence of the KDS on the absorption of drugs is drug-specific, but also depends on the location and extent of the resection (Bok-Thoe Hong 2021).

Teduglutide:

Teduglutide, which has been approved for short bowel syndrome on the German market since 2014, significantly reduces the amount of parenteral nutrition or fluid substitution required through the following modes of action:

- Hyperproliferation of the small intestinal mucosa

- Increasing the absorption of fluids

- Slowing down the transit (Rosien 2021)

However, the medication must be administered for life and therefore causes very high therapy costs (Rosien 2021).

In carefully selected patients, intestinal lengthening was attempted in order to surgically reduce parenteral nutrition (Lauro 2018).

Another group of patients with total or irreversible intestinal failure underwent intestinal transplantation. However, this procedure is currently still associated with poor long-term results (Lauro 2018).

Clinical examinations regarding general condition, stool and urine intake and output, body weight and vital signs should initially be performed daily after bowel resection (Dabsch 2025).

In the further course it is recommended

• Checks of the:
  • Laboratory values (blood count, CRP, electrolytes, albumin, kidney and liver values, vitamin D, parathyroid hormone and determination of urine sodium)
• Every 6 months Checks of:
  • pH, bicarbonate determination in venous blood
  • Vitamin B 12 and folic acid
  • Iron status
• Every 6-12 months Checks of:
  • Coagulation (Viatmin K)
  • Vitamin A and E (Dabsch 2025)

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