Short bowel syndrome overview

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Epidemiology and Demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]

Overview

Short bowel syndrome is a malabsorption disorder caused by the surgical removal of the small intestine, or rarely due to the complete dysfunction of a large segment of bowel. Most cases are acquired, although some children are born with a congenital short bowel. It does usually not develop unless a person has lost more than two-thirds of their small intestine.

Historical Perspective

The first successful intestinal resection was performed in 1880. In 1935, Haymond following research on many patients with bowel resection, reported that 30 to 50% loss of bowel was well tolerated. Total parenteral nutrition (TPN) was introduced during 1960s which helped patients to survive following bowel resection.

Classification

Based on the length of the remaining bowel, short bowel syndrome may be divided into three types such as end-jejunostomy, jejunocolonic anastomosis, ileocolonic anastomosis. They have different progress from mild to severe dysfunction. All of them require home parenteral nutrition except ileocolonic anastomosis which has excellent prognosis and rarely needs parenteral nutrition. Based on the etiology, short bowel syndrome may be divided into three categories such as vascular abnormalities, mucosal disease of intestine and causes without preexisting intestinal disease.

Pathophysiology

Short bowel syndrome is the result of bowel resection following various causes including Crohn's disease, malignancies, ischemia, and trauma. The small intestine less than 2 meters is considered as short bowel syndrome and requires nutritional therapy to prevent malnutrition. Post bowel resection adaptation might occur which includes structural, motility and functional changes in the remaining intestine. Changes usually start in the first 24 hours following bowel resection and last for about two years. Adaptation depends on multiple factors including individual, intestinal and therapeutic measurements. Following the bowel resectionadaptation occurs in three phases including acute, adaptive, and maintenance phases. Successful adaptation depends on the length of remaining intestine, portion of the resected intestine, and early introduction of nutrition therapy. Total intestinal adaptation defines as when patient is weaned from parenteral nutrition. The main reason for malabsorption following bowel resection isreduced absorption capacity of the small intestine due to loss of surface area. On gross and microscopic pathology, the resected bowel may show the underlying causes including Crohn's diseasemalignancies or ischemia.

Causes

Short bowel syndrome in adults is usually caused by surgical removal of the intestine due to different diseases including Crohn's diseasemesenteric ischemiamalignancies or radiation enteritis. Less common causes include traumavolvulusadhesion and iatrogenic surgery on gastrointestinal system.

Differentiating short bowel syndrome from Other Diseases

Epidemiology and Demographics

The incidence and prevalence of short bowel syndrome is difficult to estimate. All the data is derived from patients receiving home parenteral nutrition. Therefore, there are different distributions around the world. The incidence of short bowel syndrome was estimated to be 1-2 cases per 100,000 individuals worldwide per year. The prevalence of short bowel syndrome is approximately 0.3-0.4 per 100,000 individuals in the USA to 0.1-4 per 100,000 individuals in Europe. It affects all age groups. There is no racial predilection to short bowel syndrome and is reported worldwide. Short bowel syndrome affects men and women equally.

Risk Factors

Common risk factor in the development of short bowel syndrome may be iatrogenic including any operation on the gastrointestinal system.

Screening

There is insufficient evidence to recommend routine screening for short bowel syndrome.

Natural History, Complications, and Prognosis

The symptoms of short bowel syndrome usually develop immediately following bowel resectionDiarrhea may cause massive fluid and electrolyte loss. Immediately after surgery, intestinal adaptation develops in three phases, including acute, adaptive and maintenance phase. During the adaptation, structural, motility and functional changes happen. Patients need hydration and nutritional support via parenteralenteral and oral routes. Length of remaining small bowel is the most important prognostic factor. patients with more than 200 cm length of small bowel, usually does not need parenteral nutrition. Patients with shorter small bowel may not weaned off from parenteral nutrition support. Complications might happen due to malnutritionsurgery and parenteral nutrition. Malnutrition presents with vitaminmineral and essential fatty acids deficiencies. Complications related to surgery including thrombosisinfectionhemorrhageatelectasis and anastomosis disruption might occur. Small intestinal bacterial overgrowth due to stasis and obstruction might happen. Chronic liver disease following parenteral nutrition is a common complication in short bowel syndrome. There is no definite cure for short bowel syndrome. However, medications and nutritional therapy significantly improve the quality of life and survival of the patients. Prognosis of short bowel syndrome depends on the location and size of the bowel resection, underlying pathologynutrition support, pharmacotherapy, and extent of intestinal adaptation. The 2 and 5-year survival rate of patients with short bowel syndrome are approximately 80% and 70%, respectively.

Diagnosis

Diagnostic Criteria

Short bowel syndrome is mainly diagnosed based on clinical presentation. There are no established criteria for the diagnosis of Short bowel syndrome. History of bowel resectionand clinical manifestation including diarrhea and malnutrition would confirm the diagnosis.

History and Symptoms

The hallmark of short bowel syndrome is diarrhea. A positive history of operation on gastrointestinal system and symptoms of malabsorption is suggestive of short bowel syndrome. The most common symptoms of short bowel syndrome include dehydrationabdominal pain, and fatigue.

Physical Examination

Patients with short bowel syndrome usually appear weak and tired. Physical examination of patients with short bowel syndrome is usually remarkable for signs of malabsorptiondehydration and abdominal tenderness.

Laboratory Findings

Laboratory findings consistent with the diagnosis of short bowel syndrome include anemiahypoalbuminemia, low level of vitaminsminerals, and micronutrients. Level of acute phase reactants is high. Abnormal liver function tests including elevated liver enzymes and bilirubin might be seen. Fluid and electrolyte imbalance might be present. Fecal fattest is usually positive.

Electrocardiogram

There are no ECG findings associated with short bowel syndrome. In case of malnutrition and electrolyte imbalance, an ECG may be helpful. Hypokalemia might present with arrhythmiaST segment depression, low T wave, prominent U waves and QRS prolongation. Hypocalcemia might present with QT interval prolongation. Hypomagnesemia might present with QT interval prolongation and ventricular and supraventricular arrhythmia.

X-ray

An abdominal x-ray may be helpful in the diagnosis of short bowel syndrome. Abdominal x-ray helps to rule out ileus or intestinal obstructionUpper gastrointestinal series might demonstrate strictures and dilation of the bowel.

Ultrasound

There are no echocardiography findings associated with short bowel syndrome. Ultrasound may be helpful in the diagnosis of complications of short bowel syndrome including gallstones and liver diseasesDoppler ultrasound might be used to diagnose venous thrombosis.

CT scan

Abdominal CT scan with contrast may be helpful in the diagnosis of short bowel syndrome complications including dilationobstruction of the bowel and signs of liver diseases.

MRI

There are no MRI findings associated with short bowel syndrome.

Other Imaging Findings

There are no other imaging findings associated with short bowel syndrome.

Other Diagnostic Studies

There are no other diagnostic studies associated with short bowel syndrome.

Treatment

Medical Therapy

Management of short bowel syndrome is complicated and requires close collaboration of all medical team members including the physician, nutritionist, and nurse with the patient and their families. Medical therapy consists of nutritional therapy and pharmacotherapyNutritional therapy is essential for short bowel syndrome and to restore the intestinal adaptation. It could be provided through oralenteral and parenteral routes. The ultimate goal is to provide necessary nutrients via oral route other than parenteral or enteral routes. All patients require enough fluidelectrolytessupplements and calories. Medications are used to control symptoms of short bowel syndrome include antimotility agents, antisecretory agents, and trophic agents. Lifelong follow-up is usually needed.

Surgery

Surgery is not the first-line treatment option for patients with short bowel syndrome. Surgery is usually reserved for patients with the goal to wean them off parenteral nutrition. Patients who have severe or worsened malabsorption might require surgery including intestinal transplantTransplant is contraindicated in patients with active infection or malignancies. Approximately half of the patients with short bowel syndrome will require surgery. Bianchi procedure and serial transverse enteroplasty (STEP) are performed to lengthen dilated bowel. Stricturoplasty might be necessary. Intestinal transplant is reserved for patients who have life-threatening complications of intestinal failure, irreversible permanent total parenteral nutrition requirement and episodes of sepsis or loss of venous access.

Primary Prevention

There are no established measures for the primary prevention of short bowel syndrome. However, treating underlying causes including Crohn's disease and malignancies might prevent short bowel syndrome.

Secondary Prevention

There are several ways to prevent complications of short bowel syndrome. Management strategies and regular follow-up are needed to find and treat complications accordingly. Effective measures must be done for secondary prevention of complications following total parenteral nutrition including liver disease, cholelithiasiskidney stonesmall bowel bacterial overgrowthlactic acidosislactic acidosisHydration, consuming supplementsantibiotic therapy, and regular monitoring with blood testsultrasound and scans are recommended.

References


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