Chronic Intestinal Pseudo-Obstruction

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Chronic intestinal pseudo-obstruction (CIPO) is a rare digestive motility disorder that is characterized by frequent episodes resembling mechanical obstruction in the absence of organic, metabolic, or systemic issues and with no physical obstructions detected with X-rays as well as during surgery. CIPO is most often seen in infants and can be present from birth. There is muscle contraction inside the gastrointestinal tract, which is called motility. Read More

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Chronic Intestinal Pseudo-Obstruction

What is chronic intestinal pseudo-obstruction?

Chronic intestinal pseudo-obstruction (CIPO) is a rare digestive motility disorder that is characterized by frequent episodes resembling mechanical obstruction in the absence of organic, metabolic, or systemic issues and with no physical obstructions detected with X-rays as well as during surgery. CIPO is most often seen in infants and can be present from birth. There is muscle contraction inside the gastrointestinal tract, which is called motility.

Peristalsis is used to ensure normal functioning and nutrient absorption. This process involves using the alimentary canal to stimulate diet with the help of contractions. These contractions are usually due to hormones and muscles in the alimentary canal on which peristalsis relies. Chronic intestinal pseudo-obstruction generally results in nerve or muscle abnormalities, making peristalsis ineffective. Chronic intestinal pseudo-obstruction mainly breaks into two parts depending upon the involvement of the nerves or muscles of the alimentary canal. This problem is somewhat identical to mechanical obstruction. However, the prediction of chronic intestinal pseudo-obstruction differs from mechanical obstruction. 

Chronic Intestinal Pseudo-obstruction
Picture Courtesy: healing specialty

Causes

The causes of chronic intestinal pseudo-obstruction are many. They could be genetic in some instances or occur as an outcome of a different disease or disorder. Some severe problems of the digestive tract or esophagus may lead to pseudo-obstruction.

It occurs mainly in children. However, it is a very rare disease. Usually, chronic intestinal pseudo-obstruction is characterized by neuropathic chronic intestinal pseudo-obstruction and myopathic chronic intestinal pseudo-obstruction. To elaborate further, primarily, the causes are divided into three categories birth-related, genetic related and random occurrence. It is known that in children, chronic intestinal pseudo-obstruction is primary and random, whereas, in adults, it is secondary and random.

  • Abdominal or pelvic surgery.
  • Diseases that affect muscles and nerves, such as lupus erythematosus, scleroderma, and Parkinson’s disease.
  • Infections.
  • Medications, for example, antidepressants and opiates, which alter nerves and muscles.
  • Radiation to the abdomen.

Symptoms

Patients often have chronic, severe “obstructive” symptoms like abdominal pain, distension/fullness, vomiting, nausea or diarrhea and/or persistent constipation, malnutrition that cause weight loss, and/or inability to grow. Lab abnormalities typically reflect the extent of malabsorption and malnutrition. The radiological findings commonly include paralytic ileus or signs of apparent clinical obstruction with dilated loops of the bowel. The areas of the gut affected could be affected in isolation (small intestinal involvement is the most frequent) or dispersed, and occasionally other visceral muscles, like the bladder for urinary discharge, can be affected.

How is chronic intestinal pseudo-obstruction diagnosed?

Several tests are available to diagnose chronic intestinal pseudo-obstruction easily. For some tests, the patient needs to follow some guidelines and should be physically stable. Primary diagnosis includes 

  • Laboratory test: They include complete blood tests for blood count, certain infections, anemia, deficiency of nutrition, growth of bacteria which results in infection, SED rate to check inflammation of the body, serum electrolyte level, albumin and level of thyroid hormone in the body along with platelet count. These tests are necessary for an overview of the disease and treatment. 
  • Investigational scans: X-rays and CT scanning is employed for a precise, detailed analysis. Often MRI is also done for high-quality images of abdominal tissues.  
  • Manometry: To estimate the contraction in the intestinal tract, a manometry test (pressure measurement of the bowel) is performed. It is conducted to determine the hidden causes and to inspect the esophagus abnormalities. 
Manometry
Picture Courtesy: inflamed- diagnosis
  • Biopsy: The biopsy can be done according to the severity of pseudo-obstruction. In a biopsy, tissues of nerves or muscles are detached to scrutinize the problem microscopically.

How is chronic intestinal pseudo-obstruction treated?

Mild symptoms can be treated with light medications, but in case of severe chronic intestinal pseudo-obstruction, surgery can be employed.  

  • Nutritional support therapy- Nutritional support therapy is an essential plan for treating chronic intestinal pseudo-obstruction in children. Children who are diagnosed with CIP should have small portions of meals at regular intervals throughout the day. For children who cannot eat even a small amount of meals, in that case, the doctor will advise parenteral nutrition for the children, which supplies nutrients directly through the veins. 
  • Medications- Prokinetic drugs may improve motility (the ability to move food through the intestine). Other medications include antibiotics, painkillers, anti-nausea, and anti-diarrhea medications or laxatives for constipation.
  • Surgical decompression- This procedure is done to relieve pressure in the intestines. During this procedure, the doctor will insert a feeding tube directly into the stomach or create an opening in the abdomen through which the intestine can release gas and empty stool.
  • Bowel surgery- surgery used to treat pseudo-obstruction is called bowel resection. Firstly, the bowel comprises the rectum, large and small intestine. In this surgery, the doctor removes the affected part of the bowel to cure the disease. Usually, the blocked pathway is cured using this method. Bowel surgery can be done if the patient is diagnosed with certain intestinal cancer, infections in the abdomen, severe bleeding, and blocked pathways of the alimentary canal. Bowel surgery can be done in two ways: laparoscopic resection and open resection. The choice of surgery depends on the problem’s seriousness, the problem’s location, and the problem’s size. 

Preparation for bowel resection surgery

The patient should stop all the medications at least one week before the surgery, consume a liquid diet to vacate the bowels, and avoid unhealthy practices like smoking and drinking to reduce the chances of complications. The patient may need to fast overnight before the surgery in the hospital. He must take only liquids as a diet and should not eat or drink anything at least 12 hours before the surgery. Bring all the reports for reference and bring the necessities for one to two weeks as the patient will be under medical attention for some time. 

The procedure of bowel resection surgery

Initially, anesthesia is administered to numb the pain. For open surgery, a large cut is made in the abdomen area, whereas, for laparoscopic surgery, several small cuts are made in the abdomen. In open surgery, there is enough space for the tool to be inserted, while gas is filled in the area in laparoscopic surgery to provide space for tools. A tube is used to check the disease with a camera fixed on the head. The diseased area is cut from the organ. The remaining part is sutured (stitched) along with the main cut, and the incision area is rinsed to avoid infection.  

Recovery from surgery

Recovery can take a few weeks. Usually, two -or- four weeks of rest are required in the hospital for regular observation. The patient can drink or eat after some time. A fibrous diet is helpful. The patient can recover from laparoscopic surgery faster compared to open surgery. The patient can recover completely after 6 – 8 weeks. However, the patient can return to their everyday life in 2 weeks. Weightlifting, straining, and heavy exercises are prohibited for an extended period.

Benefits of surgery

Laparoscopic surgery has faster recovery, less or no pain, and the risks of scars are extremely low. The patient can eat anything after some time. This surgery provides better results and reduces the chances of future intestinal infections.  

Colectomy

Another surgery to treat chronic intestinal pseudo-obstruction is colectomy, which cures colon diseases such as diverticulitis, cancer, and inflammation. It is similar to laparoscopic bowel resection surgery. During this surgery, a part of the colon is detached from the intestine. The recovery rate is faster in colectomy, which can be either a laparoscopic or open surgery. This procedure is done under anesthesia. The diet for some time will be liquid to avoid pressure on the intestine. Yogurt, soup, energy drinks, fruit juices, and gelatine are recommended to provide energy. The food which must be avoided is meat, egg, bread, chewy food, and deep-fried and spicy food, which leads to gas accumulation (bloating) in the abdomen. In order to prevent the development of gas, one must avoid intake through a straw, avoid smoking and tobacco and avoid chewing gums. 

Can chronic intestinal pseudo-obstruction be prevented?

To avoid intestinal problems, one should eat fiber-rich foods like figs, apples, milk, almond, broccoli, bananas, cauliflower, pumpkin seeds, sunflower seeds, flax seeds, and sprouts.

FAQ

What is the cost of the treatment of chronic intestinal pseudo-obstruction?

Bowel resection is an expensive surgery. It costs between $24,000 and $35,000. It can cost less if a person has insurance coverage and depending on the hospital facilities.

What are the complications of chronic intestinal pseudo-obstruction surgery?

Shortness of breath, heart attack, strokes, blood clotting, damage to other parts, pneumonia, infections, bleeding from the incision, fluid leakage, hernia and scars.

How long does the treatment of chronic intestinal pseudo-obstruction take?

The treatment takes 3-4 hours of surgery. However, preparation takes a few more hours. Regular follow-up is needed for some time to look after the healing process. 

What is the life expectancy of patients with chronic intestinal pseudo-obstruction?

Pseudo-obstruction is a life-threatening disease. The life expectancy of people with this disease is usually less compared to others. The life of affected people is usually around 60-70 years. 

Can chronic intestinal pseudo-obstruction be cured?

Yes, it can be cured. In the initial stages, mild medications are given, but in intense situations, laparoscopic and open surgeries are available. 

What is the prognosis of children diagnosed with chronic intestinal pseudo-obstruction?

Early diagnosis and early treatment can lead to improvement of symptoms and quality of life in children with CIP.