Nutrition for Disorders of The GI Tract
Upper digestive tract problems
There are many problems that may affect the upper digestive tract that require clinical care by a physician or other healthcare professional. We have provided a brief overview of some in these pages.
Gastroesophageal reflux disease (GERD)
Gastroesophageal reflux disease (GERD) is a digestive disorder that is caused by gastric acid flowing from the stomach into the esophagus. Gastroesophageal refers to the stomach and esophagus, and reflux means to flow back or return.
Gastroesophageal reflux (GER) is the return of acidic stomach juices, or food and fluids, back up into the esophagus. GER is very common in infants, though it can occur at any age. It is the most common cause of vomiting during infancy.
What causes GERD?
GERD is often the result of conditions that affect the lower esophageal sphincter (LES). The LES, a muscle located at the bottom of the esophagus, opens to let food in and closes to keep food in the stomach. When this muscle relaxes too often or for too long, acid refluxes back into the esophagus, causing vomiting or heartburn.
Everyone has gastroesophageal reflux from time to time. If you have ever burped and had an acid taste in your mouth, you have had reflux. The lower esophageal sphincter occasionally relaxes at inopportune times, and usually, all your child will experience is a bad taste in the mouth, or a mild, momentary feeling of heartburn.
Infants are more likely to have the lower esophageal sphincter (LES) relax when it should remain shut. As food or milk is digesting, the LES opens and allows the stomach contents to go back up the esophagus. Sometimes, the stomach contents go all the way up the esophagus and the infant or child vomits. Other times, the stomach contents only go part of the way up the esophagus, causing heartburn, breathing problems, or, possibly, no problems at all.
Some foods seem to affect the muscle tone of the lower esophageal sphincter, allowing it to stay open longer thaormal. These include, but are not limited to, the following:
Chocolate
Peppermint
High-fat foods
Other foods increase acid production in the stomach, including:
Citrus foods
Tomatoes and tomato sauces
Why is gastroesophageal reflux disease (GERD) a concern?
Some infants and children who have gastroesophageal reflux may not vomit, but may still have stomach contents move up the esophagus and spill over into the windpipe. This can cause asthma, pneumonia, and possibly even SIDS (sudden infant death syndrome).
Infants and children with GERD who vomit frequently may not gain weight and grow normally. Inflammation (esophagitis) or ulcers (sores) can form in the esophagus due to contact with stomach acid. These can be painful and also may bleed, leading to anemia (too few red blood cells in the bloodstream). Esophageal narrowing (stricture) and Barrett’s esophagus (abnormal cells in the esophageal lining) are long-term complications from inflammation.
Symptoms of GERD
Heartburn, also called acid indigestion, is the most common symptom of GERD. Heartburn is described as a burning chest pain that begins behind the breastbone and moves upward to the neck and throat. It can last as long as two hours and is often worse after eating. Lying down or bending over can also result in heartburn. The following are other common symptoms of GERD. However, each child may experience symptoms differently. Symptoms may include:
Belching
Refusal to eat
Stomachache
Fussiness around mealtimes
Frequent vomiting
Hiccups
Gagging
Choking
Frequent cough
Coughing fits at night
Wheezing
Frequent upper respiratory infections (colds)
Rattling in the chest
Frequent sore throats in the morning
Sour taste in the mouth
The symptoms of GERD may resemble other conditions or medical problems. Consult your child’s physician for a diagnosis.
Diagnosing GERD
Your child’s physician will perform a physical examination and obtain a medical history. Diagnostic procedures that may be done to help evaluate GERD include:
Chest x-ray a diagnostic test to look for evidence of aspiration.
Upper GI (gastrointestinal) series a diagnostic test that examines the organs of the upper part of the digestive system: the esophagus, stomach, and duodenum (the first section of the small intestine). A fluid called barium (a metallic, chemical, chalky, liquid used to coat the inside of organs so that they will show up on an x-ray) is swallowed. X-rays are then taken to evaluate the digestive organs.
Endoscopy a test that uses a small, flexible tube with a light and a camera lens at the end (endoscope) to examine the inside of part of the digestive tract. Tissue samples from inside the digestive tract may also be taken for examination and testing.
esophageal manometric studies
pH testing
gastric emptying studies
Treatment for GERD
Specific treatment will be determined by your child’s physician based on the following:
Your child’s age, overall health, and medical history
The extent of the disease
Your child’s tolerance for specific medications, procedures, or therapies
The expectations for the course of the disease
Your opinion or preference
In many cases, GERD can be relieved through diet and lifestyle changes, under the direction of your child’s physician. Some ways to better manage GERD symptoms include the following:
Ask your child’s physician to profile any of the medications he/she is taking – some may irritate the lining of the stomach or esophagus.
Watch your child’s food intake – limit fried and fatty foods, peppermint, chocolate, drinks with caffeine (such as colas, Mountain Dew™, and tea), citrus fruit and juices, and tomato products.
Offer your child smaller portions at mealtimes, and include small snacks in-between meals if your child is hungry. Avoid letting your child overeat. Allow him/her to let you know when he/she is hungry or full.
If your child is overweight, consult his/her physician to set weight loss goals.
Do not allow your child to lie down or go to bed right after a meal. Serve the evening meal early – at least two hours before bedtime.
After feedings, place your infant on his/her stomach with the upper body elevated at least 30° F, or hold him/her in a sitting position in your lap for 30 minutes.
If bottle-feeding, keep the nipple filled with milk so your infant does not swallow too much air while eating. Try different nipples to find one that allows your baby’s mouth to make a good seal with the nipple during feeding.
Adding rice cereal to feeding may be beneficial for some infants.
Burp your baby several times during bottle or breast feeding. Your child may reflux more often when burping with a full stomach.
Treatment may include:
§ Medications
§ If needed, your child’s physician may prescribe medications to help with reflux. There are medications which help decrease the amount of acid the stomach makes, which, in turn, will cut down on the heartburn associated with reflux.
One group of this type of medication is called “H2-blockers”. Medications in this category include cimetidine (Tagamet ®) and ranitidine (Zantac ®). Another group of medications is called “proton-pump inhibitors.” Medications in this category include omeprazole (Prilosec ®) and lansoprazole (Prevacid ®). These medications are taken daily to prevent excess acid secretion in the stomach.
Another type of medicine your child’s physician may prescribe helps the stomach empty faster. If food does not remain in the stomach as long as usual, there may be less chance of reflux occurring. A medicine in this category that is often prescribed is metoclopramide (Reglan ®). This medicine is usually taken three to four times a day, before meals or feedings and at bedtime.
Calorie supplements Some infants with reflux will not be able to gain weight due to frequent vomiting. Your child’s physician may recommend the following:
o Adding rice cereal to baby formula
o Providing your infant with more calories by adding a prescribed supplement (such as Polycose™ or Moducal™) to formula or breast milk to make the milk higher in calories thaormal
Tube feedings Some babies with reflux have other conditions that make them tired, such as congenital heart disease or prematurity. In addition to having reflux, these babies may not be able to drink very much without becoming sleepy.
Other babies are not able to tolerate a normal amount of formula in the stomach without vomiting, and would do better if a small amount of milk was given continuously. In both of these cases, tube feedings may be recommended. Formula or breast milk is given through a tube that is placed in the nose, guided through the esophagus, and into the stomach (nasogastric tube). Nasogastric tube feedings can be given in addition to or instead of what a baby takes from a bottle.
Surgery In severe cases of reflux, a surgical procedure called fundoplication may be performed. Your physician may recommend this operation if your child is not gaining weight due to vomiting, has frequent respiratory problems, or has severe irritation in the esophagus.
This procedure is usually done laparoscopically, which means that pain in minimized and the recovery time is faster after surgery. Small incisions are made in the abdomen, and a small tube with a camera on the end is placed into one of the incisions to look inside. The surgical instruments are placed through the other incisions while the surgeon looks at a video monitor to see the stomach and other organs. The top portion of the stomach is wrapped around the esophagus, creating a tight band that greatly decreases reflux.
The long-term outlook for a child with GERD
Many infants who vomit will “outgrow it” by the time they are about a year old, as the lower esophageal sphincter becomes stronger. For others, medications, lifestyle, and diet changes can minimize reflux, vomiting, and heartburn.
Crohn’s Disease an inflammatory bowel disease that can affect part of the gastrointestinal tract. Crohn’s disease Symptoms is characterized by abdominal pain, constipation, diarrhea , vomiting, weight loss or gain.
Crohn’s disease is genetically linked and right treatments includes specific food, diet and medicines like SEROVERA® that has been used with great success by many individuals with gastrointestinal disorders. Many people with Crohn’s Disaese and gastrointestinal disorders have also tried Carbohydrate Diet with good results.
The New Recipes of A Mediterranean Diet
Recipes of the Mediterranean diet: The Mediterranean diet is a kind of moderutritional model that got its inspiration from the traditional dietary patterns of some of the countries in the Mediteranean basin, particularly
Mediterranean-style diets are close to the dietary recommendations of the American Heart Association, but are not followed exactly. In general, the traditional Mediterranean diet consumed by the people of the region contains a high percentage of calories from fat. There are several websites on the Internet where you can find the recipes of the Mediterranean diet. Besides the recipes, you can also get a lot of information about the Mediterranean diet and its popularity.
Food in the Mediterranean diet: The main factor in the appeal of the Mediterranian diet is the full flavored and rich food. Hydrogenated oils and margarine are considered to be bland and lack the flavor that olive oil imparts to food items. It is not easy to understand and define the Mediterraneautrition and diet. The traditional diets in southern
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Irritable bowel syndrome
Irritable bowel syndrome is one of the manifestations of functional abdominal pain. Functional means that there is no organic disease. Functional abdominal pain describes a constellation of symptoms as a result of intestinal motility disorders. Symptoms may include:
Flatulence
Bloating
Diarrhea
Constipation
Urgency with defecation
Incomplete sensation of defecation
Passage of mucus in the stool
What causes irritable bowel syndrome?
The digestion and propulsion of nutrients and fluids through the gastrointestinal system (GI) is a very complicated and very well organized process. The GI tract has its own intrinsic muscles and nerves that connect, like an electrical circuit, to the spinal cord and brain.
Neuromuscular events occurring in the GI tract is relayed to the brain through neural connections, and the response of the brain is also relayed back to the gastrointestinal tract. As a result of this activity, motility and sensation in the bowel is generated.
An abnormality in this process results in a disordered propulsion of the intestinal contents and generates the sensation of pain. The nerves that control the digestive tract may also be more sensitive to the activity associated to the process of digestion. Children with irritable bowel syndrome may be more aware of gas and motion and rumbles of the intestines. They are more aware of these discomforts and hence more irritated when they occur. The child who experiences the symptoms of irritable bowel syndrome is thought to be as a result of the following and their interaction:
Motility abnormalities
Visceral hypersensitivity – The viscera are the organs inside the abdominal cavity. The GI tract is the largest in the abdomen and it has been seen that in patients with functional GI disorders there is heightened sensitivity to changes within the intestine, whether to the presence of food or as a result of distension of the viscera.
Psychosocial problems – It is well known that the emotional state of the person can directly influence the activity in the gastrointestinal tract. High levels of anxiety, stress, or anger can induce diarrhea.
A stressful event is not circumscribed to emotional issues but also to physical stresses such as a viral illness or any other particular disease state.
All of the above factors can trigger the occurrence of symptoms. It is important to stress to the child with a functional bowel disorder that his/her abdominal pain is real and not imaginary.
Who is affected by irritable bowel syndrome?
Irritable bowel syndrome occurs in both children and adults.
Ten to 15 percent of school-aged children and adolescents have symptoms of functional abdominal pain to the point of interrupting their daily normal life.
Thirty-three percent of adults who have irritable bowel syndrome can trace their symptoms back to childhood.
Girls are affected by the disorder slightly more often than boys.
There is no known gene that causes irritable bowel syndrome, but the disorder does seem to occur more often in families where either a child or a parent has the disorder.
Why is irritable bowel syndrome a concern?
Children with irritable bowel syndrome often do not feel well. Those who have diarrhea may have little warning of their need to go to the bathroom, and, therefore, may be embarrassed and avoid going to school or socializing with their schoolmates. Children can become depressed or anxious because of the disorder.
Most children with irritable bowel syndrome continue to grow and develop normally. However, some children may eat less to avoid the pain that can accompany digestion, and therefore, lose weight.
Its greatest morbidity resides in the fact that it affects normal daily activity of the child, affecting school and peer relations.
Symptoms of irritable bowel syndrome
According to the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), irritable bowel syndrome in children tends to produce two types of symptoms: diarrhea or pain, depending on the age of the child. Symptoms may include:
Recurrent abdominal pain. The pain becomes chronic when it has been present for a period greater than three months.
An altered bowel pattern with diarrhea and constipation, all of which suggests intestinal motility dysfunction.
Headaches
Pallor
Nausea
Dizziness
Anorexia
Limb pain
In children, symptoms of functional bowel disorders are variable and are age dependent. For example:
Infantile colic (younger than 4 months of age)
Gastroesophageal reflux (younger than 2 years of age and then reappears in adolescence and adulthood)
Chronic non-specific diarrhea (younger than 4 years of age)
Constipation (any age)
Irritable bowel syndrome (adolescents and adults)
The symptoms of irritable bowel syndrome are not unique for the condition. Altered bowel pattern and abdominal pain could be symptoms of organic disease — one reason why you should always consult your child’s physician for a diagnostic work-up.
Diagnosing irritable bowel syndrome
Your child’s physician will obtain a thorough medical history, perform a full physical examination, and obtain screening laboratories to assess for infection and inflammation. The laboratory tests, imaging studies, and procedures to be performed will be dictated by the history and physical examination. Tests and procedures that your child’s physician may order may include the following:
Blood tests – to evaluate whether your child is anemic, has an infection, or has an illness caused by inflammation or irritation.
Urine analysis and culture – to help assess for urinary tract infections.
Stool sample – for culture to check for bacteria and parasites that may cause diarrhea.
Stool samples for occult blood – occult blood cannot be seen and is only detected by a special solution that turns blue when coming into contact with blood. It suggests an inflammatory source in the gastrointestinal tract.
Lactose breath hydrogen test – to determine if your child is intolerant to lactose, a sugar present in milk and milk products.
Abdominal x-ray – a simple study that will give the physician an idea of how the internal organs look.
Abdominal ultrasound – a diagnostic imaging technique which creates images from the rebound of high frequency sound waves in the internal organs.
Endoscopy – a test that uses a small, flexible tube with a light and a camera lens at the end (endoscope) to examine the inside of part of the digestive tract. Tissue samples from inside the digestive tract may also be taken for examination and testing. Rarely, the physician will require ultrasound and/or an endoscopy.
Colonoscopy – a test that uses a long, flexible tube with a light and camera lens at the end (colonoscope) to examine inside the large intestine.
Treatment for irritable bowel syndrome
Specific treatment of irritable bowel syndrome will be determined by your child’s physician based on:
Your child’s age, overall health, and medical history
Extent of the problem
Your child’s tolerance for specific medications, procedures, or therapies
Expectations for the course of the condition
Your opinion and preference
The main objective of treatment for irritable bowel syndrome to restore normal daily function. Management begins with the positive diagnosis of irritable bowel syndrome. This will give your child reassurance that he/she does not have any life-threatening condition.
Environmental modification is important to identify the stresses surrounding your child and reverse them. Parents and school teachers must support the child rather than concentrating on the pain. Try to help the child focus on something fun or pleasant during a painful episode. In lactose intolerant patients, restriction of lactose or supplementing the enzyme that digests the sugar (lactase/Lactaid®) is recommended since this sugar can be a trigger for symptoms of irritable bowel syndrome.
A controversial issue is the use of high fiber in children since it could promote flatulence and abdominal distension. It is recommended in the adult population, and it may be beneficial in children in which the symptom of constipation predominates.
Depending on the severity of the symptom, medication may be indicated. In rare cases, pain control needs to be administered by specialists in the field of pain management. Biofeedback has become part of the treatment strategy, as well as acupuncture.
How much fiber is enough?
For children two years of age and older, use the following guideline: The child’s age plus five equals the number of grams of fiber that should be eaten daily.
For example, a 4 year old needs four + five grams per day = nine grams of fiber per day.
What foods are high in fiber?
Fiber is found in foods such as:
Fruits
Vegetables
Whole wheat breads and cereals
Beans
Peas
Suggestions for increasing fiber intake:
Use raw vegetables and fruits with dip.
Use unpeeled potatoes for potato salad or french fries.
Add high-fiber cereal or fruit to ice cream, frozen yogurt, or yogurt.
Add beans to soups, stews, and salads.
Use whole wheat bread for sandwiches.
What are good fiber sources?
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FOODS |
MODERATE FIBER |
HIGH FIBER |
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BREAD |
Whole wheat bread, granola bread, wheat bran muffins, Nutri-Grain™ waffles, popcorn |
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CEREAL |
Bran Flakes™, Raisin Bran™, Shredded Wheat™, Frosted Mini Wheats™, oatmeal, Muslix™, granola, oat bran |
All-Bran™, Bran Buds™, Corn Bran™, Fiber One™, 100% Bran™ |
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VEGETABLES |
Beets, broccoli, brussel sprouts, cabbage, carrots, corn, green beans, green peas, acorn and butternut squash, spinach, potato with skin, avocado |
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FRUITS |
Apples with peel, dates, papayas, mangos, nectarines, oranges, pears, kiwis, strawberries, applesauce, raspberries, blackberries, raisins |
Cooked prunes, dried figs |
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MEAT SUBSTITUTES |
Peanut butter, nuts Baked beans, black-eyed peas, garbanzo beans, lima beans, pinto beans, kidney beans, chili with beans, trail mix |
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High-fiber meal vs. a typical meal
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Typical Meal |
High-fiber Meal |
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Breakfast |
Breakfast |
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Lunch Beef patty |
Lunch Beef patty |
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Dinner |
Dinner |
Esophageal Atresia/Tracheoesophageal Fistula
EA is a condition in which the proximal and distal portions of the esophagus do not communicate. The upper segment of the esophagus is a dilated blind-ending pouch with a hypertrophied muscular wall. This pouch typically extends to the level of the second to fourth thoracic vertebra. In contrast, the distal esophageal portion is an atretic pouch with a small diameter and a thin muscular wall; it extends a variable distance above the diaphragm.
TEF is an abnormal communication between the trachea and esophagus. When associated with EA, the fistula most commonly occurs between the distal esophageal segment and the trachea. The distal esophageal segment communicates with the trachea just above the carina. An H-type TEF represents a TEF without EA. It can occur at any level from the cricoid cartilage to the carina, although it usually courses obliquely (with the tracheal end proximal) at or above the level of the second thoracic vertebra.
Background, types of EA and TEF
Five types of EA and TEF have been described. The most common abnormality is EA with a distal TEF (84%). Isolated atresia with no fistula is the next most common finding (8%), followed by TEF with no atresia (so-called H type) (4%). EA with proximal and distal fistulas (3%) and EA with a proximal fistula (1%) are less common.
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Etiologies and factors.
The etiologies of these anomalies are still largely unknown, but many theories concerning their origins have been proposed. The trachea and esophagus are foregut derivatives. Lateral mesodermal ridges form in the proximal esophagus during the fourth gestational week, and the fusion of these grooves in the midline separates the esophagus from the trachea around the 26th day of gestation.
Notochord abnormalities, desynchronous esophageal mesenchymal and epithelial growth rates, neural crest cell involvement, and incomplete tracheoesophageal separation resulting from a lack of apoptosis are mentioned in some of the theories that have been proposed for EA embryogenesis. Similarly, incomplete tracheoesophageal septation, lateral ridge fusion failure, and tracheal and esophageal proximity have been suggested in explanations of the origin of TEF. In addition, vascular insufficiencies; genetic factors; vitamin deficiencies; drug and alcohol exposures; and viral, chemical, and physical external events may contribute to the development of EA and/or TEF.
Pathophysiology.
Because of the discontinuous esophagus, infants with EA cannot clear their secretions. This defect leads to persistent drooling and aspiration or regurgitation of food after attempted feedings. TEF causes additional complications because of the tracheoesophageal communication. When infants with this anomaly strain, cough, or cry, air enters the stomach through the fistula. As a result, the stomach and small intestine become dilated, elevating the diaphragm and making respiration more difficult. The reflux of food and gastric secretions may also occur up the esophagus and through the fistula into the tracheobronchial tree; this reflux can contribute to pneumonia and atelectasis. Therefore, pneumonia and respiratory distress are common complications.
Abnormal esophageal motility has been observed in children with EA and/or TEF. Controversy often exists as to whether the abnormality was inherently present in the child’s esophagus or if the dysfunction was a result of the surgical treatment. Manometric studies have shown that the motility disorder is present before surgical treatment. Animal studies have also shown that esophageal transection followed by repair does not precipitate disturbances in motility. Discoordinated peristalsis has been reported from the level of the fistula to the stomach in patients with isolated TEF.
Frequency. The international occurrence varies, with estimates ranging from 0.4 to 3.6 cases per 10,000 live births in different regions of the world.
Mortality/Morbidity. Despite an increased number of patients with severe anomalies, survival rates as high as 95% have been reported. In uncomplicated cases, survival rates approach 100%.
Clinical signs.
The first clinical sign of an infant with EA is maternal polyhydramnios resulting from the infant’s inability to swallow and absorb amniotic fluid through the gut. Polyhydramnios is seen in infants with many diagnoses; only
Most infants with EA become symptomatic within the first few hours of life, unlike children with an isolated fistula, who have more subtle symptoms that may not be recognized initially. Excess salivation and fine, frothy bubbles in the mouth and sometimes nose result from an inability to swallow. Any attempts at feeding result in choking, coughing, cyanotic episodes, and food regurgitation. The presence of a fistula increases respiratory complications due to aspiration of food and secretions in the trachea and lungs. Pneumonitis and atelectasis develop quickly in the affected neonate, and rattles heard during respirations are common. Fistulas also allow air to enter the stomach and intestines, leading to abdominal distension. With atresia alone, the abdomen appears scaphoid.
Many anomalies are associated with EA, and 50-70% of children with EA have some other defect.
Diagnosis. Once the EA is considered, appropriate diagnostic procedures are necessary. The simplest and quickest diagnostic procedure is the passage of a 10 to 12 French oral tube into the esophagus. If an obstruction encountered (usually in 9 to
Ultrasound. Although ultrasonography has no role in the routine postnatal evaluation of EA and/or TEF, prenatal sonography is a valuable screening tool for EA and/or TEF. The diagnostic accuracy is increased if an anechoic area is present in the middle of the fetal neck; this sign differentiates EA from diseases with possible swallowing impairments.
Plain radiographs provide much information, including findings for EA confirmation and depiction of the side of the aortic arch side, presence of any vertebral or other associated anomalies, and others. Barium studies performed after the surgical placement of a gastrostomy may be used to evaluate the gap length and associated GI abnormalities such as duodenal atresia or malrotation. However, radiographs may not always demonstrate the presence of a fistula.
Findings on posteroanterior and lateral chest images confirm a diagnosis of EA by displaying a coiled nasogastric tube (placed for determination of EA) in the proximal esophageal pouch of a child with EA.
Any vertebral anomalies may be visualized, and some cardiac anomalies may be suggested. Aspiration pneumonia, especially in the right upper lobe, and patchy atelectasis are frequently present.
Aside from these general findings, the radiographic observations in children with EA and/or TEF vary depending on the type of anomaly present.
The anatomy should be clarified bronchoscopically at the time of the first anesthetic.
Treatment.
Preoperative management.
· Confirming the diagnosis and type of anomaly;
· Evaluating the pulmonary status, treating existing pulmonary problems, and preventing further tracheal contamination;
· Searching for, and treating other major associated problems.
To prevent further aspiration, the pharynx is frequently suctioned. The patient is cared far in a head-elevated position, with a catheter insert into the upper esophageal pouch and connected to continuous sump suction.
All patients are given antibiotic.
If there is clinical or radiological evidence of significant atelectasis or pneumonia, a decompression Stamm gastrostomy is performed using general anesthesia. Patients usually respond well within 24 to 72 hours, at which time the anomaly is repaired.
Gastrostomy decompression, upper pouch suction, and central venous nutritional support allow for the stabilization of the patient, the search for other disease, and the treatment of both medical or surgical problems.
Operative management.
Repair is performed using general anesthesia. The operative approach is through posterior lateral thoracotomy incision on the side of the chest opposite the aortic arch.
The fistula is divided and dissected superiory to its junction with trachea. The tracheal end is closed by several nonabsorbable sutures. The tip of the proximal pouch is excised. And the two ends are approximated. For this anastomosis most surgeons use a single layer of full thickness interrupted sutures. A catheter is placed in the retropleural space near the anastomosis to drain the area in case an anastomosis leak should occur.
A long gap between the ends of the esophageal segments presents a difficult problem. When recognized preoperatively stretching of the upper pouch by the twice-daily insertion of a large catheter or dilator and applying gentle pressure usually result in elongation of the proximal pouch over a period of 3 to 6 weeks. Wheo distal TEF is present, the distal pouch can also be stretched by retrograde dilatation via gastrostomy. At operation, he surgeon has several options that are helpful: a circumferential esophagomyotomy. On rare occasions, if the segments cannot be approximated, the lower segment is sewn over and fixed to the perivertebral fascia to prevent retraction. The upper pouch is brought out trough a supraclavicular incision and sutured to the skin. Esophageal reconstruction is carried out 6 to 12 month later.
Postoperative care.
Antibiotics are continued for several days.
The chest tube is left in place for 10 days.
In the large, healthy baby, the endotracheal tube can be safety removed soon after the procedure. In the small, ill infant, ventilatory support is usually required for several days. Small amounts of clear liquid oral feeding are started when the infant can handle the saliva.
When the patient is taking adequate oral feedings, swallowing without difficultly, and gaining weight, the parents are instructed in home care, and the infant is discharged. A gastrostomy tube, if present, is usually removed before the infant goes home.
Complications. The most common complications include anastomotic leak, recurrent fistula, stricture, and gastroesophageal reflux (GER).
Infantile hypertrophic pyloric stenosis
Pathophysiology.
Diffuse hypertrophy and hyperplasia of the smooth muscle of the antrum of the stomach and pylorus proper narrow the channel, which then can become easily obstructed. The antral region is elongated and thickened to as much as twice its normal size. In response to outflow obstruction and vigorous peristalsis, stomach musculature becomes uniformly hypertrophied and dilated. Gastritis may occur after prolonged stasis. Hematemesis is occasionally noted. The patient may become dehydrated as a result of vomiting and develop marked hypochloremic alkalosis.
Frequency. Pyloric stenosis is a common cause of gastric outlet obstruction in infants. The prevalence of HPS ranges from 1.5-4/1000 live births among whites, although it is less prevalent among African and Asian Americans.
Clinical features. The peak incidence for the onset of symptoms is between the 3rd and 6th weeks of life. However, the condition can commence before that time, and as late as the 7th week. The pillars upon with the diagnosis rests are:
Vomiting is the presenting symptom in all cases and withing 2 or 3 days it becomes forcible and progectove. Bile is not present in the vomiting. Immediately after vomiting the baby is often very hungry.
Visible peristalsis. After the child has been fed, peristaltic waves may be seen passing from the left to right across the upper abdomen. It is the so-cold symptom of “sand clock”. A ggod light is essential for this. The abdomen should be examined throughout a feed until vomiting occurs.
The presence of a lump. The palpation of the hypertrophyed pylorus is the most essential step in reaching a diagnosis. The surgeon should palpate under the liver with a warm hand. It may be helpful to examine the child more than once. The lump is most easily felt when the child is given a feed.
Constipation is usually present, and when a stool is passed it is small and dry. It is important to ask the mother about napkins. If the child is dehydrated, they are not wet and the case is correspondingly more urgent.
One of the most striking signs of infants sufferinf from IHPS is loss of weight. Moreover, it is not long before the infant begins to look emaciated and dehydrated.
Often a change from one type of feeding to another brings about a remission. Consequently a series of changes in diet are sometimes made before the diagnosis is established, by with time the infant`s condition may be pitiable.
In prematures infants, in whom the condition is not uncommon, the symptomatology is often paradoxical. There is anorexia instead of voracious appetite; the vomiting is regurgitant rather than projectily, and so frequently is peristalsis normally visible. None the less, amids this sea of bewilderment one diagnostic rock remains – a hypertrophied pylorus can be felt through the poorly-developed abdominal wall with comparative ease.
Ultrasonography has become the criterion standard imaging technique for diagnosing HPS. It is reliable and easily performed. An experienced ultrasonographer increases the test’s predictive value. Necessary measurements include pyloric muscle thickness and pyloric channel length. Muscle wall thickness
Barium upper gastrointestinal (UGI) study is an effective means of diagnosing HPS. It should demonstrate an elongated pylorus with antral indentation from the hypertrophied muscle. The classic “railroad track” sign of two thin parallel streams of barium traversing the pylorus is pathognomonic. Confirming the diagnosis of HPS is impossible if barium does not leave the stomach. Sufficient patience, however, usually demonstrates the above findings. An advantage of a barium UGI contrast study is the ability to identify gastroesophageal reflux, a frequent differential diagnosis of HPS. After UGI, irrigating and removing any residual barium from the stomach is advisable to avoid aspiration.
Although UGI endoscopy would demonstrate pyloric obstruction, physicians would find it difficult to differentiate accurately between HPS and pylorospasm. Endoscopic dilatation has rarely been employed as a method of treatment. This treatment is not standard for HPS; endoscopy should be used rarely, if ever.
Preoperative resuscitation: If necessary, administer an initial fluid bolus of 10 mL/kg with lactated Ringer solution or 0.45 isotonic sodium chloride solution. Continue intravenous (IV) therapy at an initial rate of 1.25-2 times the normal maintenance rate until adequate fluid status is achieved.
Adequate amounts of both chloride and potassium are necessary to correct metabolic acidosis. Unless renal insufficiency is a concern, initially add 2-4 mEq of KCL per 100 mL of IV fluid. Adequate chloride for resuscitation can usually be provided by 5% dextrose in 0.4% sodium chloride solution. Avoid adding hypertonic chloride or ammonium chloride.
Urine output and serial electrolyte determinations are performed during resuscitation. Correction of serum chloride level to 90 mEq/L or greater usually is adequate to proceed with surgical intervention.
Surgical Care. Before induction of anesthesia, aspirate the infant’s stomach with a large-caliber suction tube to remove any residual gastric fluid or barium. Saline irrigation occasionally is necessary to remove a large quantity of barium. Ramstedt pyloromyotomy remains the standard procedure for HPS because it is easily performed and is associated with minimal complications. The usual approach is via a right upper quadrant transverse incision that splits the rectus muscle and fascia.
Once the abdomen is entered, the stomach is grasped gently and held with a moist sponge. Using a gentle back-and-forth rocking motion, the stomach is used to deliver the pylorus into the incision. Be careful to avoid direct traction on the pylorus or duodenum, which can result in injury. A serosal incision is made from the pyloric vein (just proximal to the duodenum) to the gastric antrum. This incision is then spread apart to complete the myotomy. Carefully avoid undue stretch on the pyloric mucosa or injudicious transgression of the duodenum.
Completion of the myotomy well onto the gastric antrum assures success. Search for mucosal tears or perforations and correct with fine absorbable suture if found. Avoid use of electrocautery because most bleeding from the myotomy is venous and remits when the pylorus is returned to its normal position. Perforation of the mucosa should be a rare event. Some surgeons repair the mucosal defect and cover the area with a small finger of omentum. Other surgeons opt to close the myotomy and create a secondary myotomy 180° distant from the previous site.
Postoperative management
Continue IV maintenance fluid until the infant is able to tolerate enteral feedings. In most instances, feedings can begin within 8 hours following surgery. Graded feedings can usually be initiated every 3 hours, starting with Pedialyte and progressing to full-strength formula.
Complications
· Undetected mucosal perforation: Perform a diligent search for mucosal transgressions at the time of operation and examine the infant again before initiating feedings. In those rare cases where a perforation was not detected, the infant develops fever, tenderness in the abdomen, and abdominal distention. Return to the operating theater if perforation is suspected.
· Bleeding: In most instances, venous oozing from the myotomy site is self-limited and is not a concern in the postoperative period.
· Persistent vomiting: Incomplete pyloromyotomy is rare in the hands of an experienced pediatric surgeon. Less experienced surgeons occasionally commit this error, but they are more likely to cause injury to the mucosa from injudicious spreading during the myotomy. This problem is confounded when repeat studies performed after surgery provide a confusing picture. Patient observation resolves the problem in most cases
Many authors define intestinal malrotation as intestinal nonrotation or incomplete rotation around the superior mesenteric artery (SMA). It involves anomalies of intestinal fixation as well. Interruption of typical intestinal rotation and fixation during fetal development can occur at a wide range of locations, and this leads to a variety of both acute and chronic presentations of disease. The most common type found in pediatric patients is incomplete rotation predisposing to midgut volvulus, which can result in short-bowel syndrome or even death.
Malrotation in itself may not cause any problems. However, it may be accompanied by additional complications:
Ø Bands of tissue called Ladd bands may form, obstructing the first part of the small intestine (the duodenum).
Ø After birth, volvulus may occur. This is when the intestine twists on itself, causing a lack of blood flow to the tissue and leading to tissue death.
Causes. The cause of intestinal malrotation is disruption in the normal embryological development of the bowel. Clinical features depend on the stage of disruption and are discussed as follows. A full understanding of normal development aids in understanding the etiology of malrotation.
Normal embryology. Normal rotation takes place around the SMA as the axis. It is described by referring to 2 ends of the alimentary canal, the proximal duodenojejunal loop and the distal cecocolic loop, and usually is divided into 3 stages. Both loops make a total of 270° in rotation during normal development. Both loops start in a vertical plane parallel to the SMA and end in a horizontal plane.
Stage I occurs between the 5th and 10th weeks of gestation. It is the period of physiologic herniation of the bowel into the base of the umbilical cord. The duodenojejunal loop begins superior to the SMA at a 90° position and rotates 180° in a counterclockwise direction. At 180°, the loop is to the anatomical right of the SMA, and by 270°, it is beneath the SMA. The cecocolic loop begins beneath the SMA at 270°. It rotates 90° in a counterclockwise manner and ends at the anatomical left of the SMA at a 0° position. Both loops maintain these positions until the bowel returns to the abdominal cavity. Also during this period, the mid gut lengthens along the SMA, and, as rotation continues, a very broad pedicle is formed at the base of the mesentery. This broad base protects against midgut volvulus.
Stage II occurs during the 10th week of gestation, the period when the bowel returns to the abdominal cavity. As it returns, the duodenojejunal loop rotates an additional 90° to end at the anatomical left of the SMA, the 0° position. The cecocolic loop turns 180° more as it reenters the abdominal cavity. This turn places it to the anatomical right of the SMA, a 180° position.
Stage III lasts from 11 weeks’ gestation until term. It involves the descent of the cecum to the right lower quadrant and fixation of the mesenteries.
Nonrotation. Arrest in development at stage I results ionrotation. Subsequently, the duodenojejunal junction does not lie inferior and to the left of the SMA, and the cecum does not lie in the right lower quadrant. The mesentery in turn forms a narrow base as the gut lengthens on the SMA without rotation, and this narrow base is prone to clockwise twisting leading to midgut volvulus. The width of the base of the mesentery is different in each patient, and not every patient develops midgut volvulus.
Incomplete rotation. Stage II arrest results in incomplete rotation and is most likely to result in duodenal obstruction. Typically, peritoneal bands running from the misplaced cecum to the mesentery compress the third portion of the duodenum. Depending on how much rotation was completed prior to arrest, the mesenteric base may be narrow and, again, midgut volvulus can occur. Internal herniations may also occur with incomplete rotation if the duodenojejunal loop does not rotate but the cecocolic loop does rotate. This may trap most of the small bowel in the mesentery of the large bowel, creating a right mesocolic (paraduodenal) hernia.
Incomplete fixation. Potential hernial pouches form when the mesentery of the right and left colon and the duodenum do not become fixed retroperitoneally. If the descending mesocolon between the inferior mesenteric vein and the posterior parietal attachment remains unfixed, the small intestine may push out through the unsupported area as it migrates to the left upper quadrant. This creates a left mesocolic hernia with possible entrapment and strangulation of the bowel. If the cecum remains unfixed, volvulus of the terminal ileum, cecum, and proximal ascending colon may occur.
Frequency.
Intestinal malrotation occurs at a rate of
Mortality/Morbidity.
Younger patients have higher rates of morbidity and mortality. In infants, the mortality rate ranges from 2-24%. The presence of necrotic bowel at surgery increases the mortality rate by 25 times for infants, and the presence
Clinical signs
Acute midgut volvulus. Most patients present in the first year of life. The primary presenting sign of acute midgut volvulus is sudden onset of bilious emesis. Abdominal distention is frequently present, and the infant appears in acute pain. As vascular compromise persists, intraluminal bleeding may occur, which leads to blood per rectum and sometimes hematemesis. Abdominal guarding is usually present and prevents palpation of intestinal loops. As symptoms persist, the infant may develop signs of shock, including poor perfusion, decreased urine output, and hypotension. Patients also have signs of peritonitis, including abdominal tenderness and discoloration of the skin.
Chronic midgut volvulus is due to intermittent or partial twisting that results in lymphatic and venous obstruction. Multiple case reports show that 2 of the main presenting features are recurrent abdominal pain and malabsorption syndrome. Several patients presented with acute midgut volvulus, but further history revealed they had had chronic symptoms with misdiagnoses. Physical examination results may be completely normal if the patient presents during a period when the obstruction is relieved. If partial twisting is present at the time of examination, the patient may have signs and symptoms equivalent to those of acute midgut volvulus.
Acute duodenal obstruction is recognized in infants and is due to compression or kinking of the duodenum by peritoneal bands (Ladd bands). Patients present with forceful vomiting, which may or may not be bile stained depending on location of the obstruction with respect to the entrance of the common bile duct (ampulla of Vater).
Internal herniation usually has a chronic picture. Patients have recurrent abdominal pain, which may progress from intermittent to constant. They experience vomiting as well as constipation at times. They are often diagnosed with psychosocial problems.
Lab Studies.
Complete blood cell count. An elevated or decreased WBC count may indicate sepsis as a reason for abdominal distention and bilious emesis. A decreased platelet count may indicate a platelet consumptive process, eg, necrotizing enterocolitis (NEC).
Arterial, capillary, or venous blood gas. Metabolic acidosis provides evidence for ongoing ischemia as observed with NEC or strangulated bowel (volvulus).
Blood chemistries. Ongoing sodium, chloride, and bicarbonate losses occur through suctioned GI secretions. Furthermore, patients may have increased potassium levels due to metabolic acidosis and hemolysis.
Urinalysis and urine culture. Complete these tests only if abdominal distention is suspected because of another ongoing infectious process and not because of GI rotational or obstructive malformations.
Prothrombin time (PT) and activated partial thromboplastin time (aPTT): Perform clotting studies in older infants and children in whom surgery is highly likely.
Imaging Studies.
Plain abdominal radiography. Plain radiography has limited use for defining obstruction because infants may have a gasless abdomen or one that is almost normal.
The classic pattern for duodenal obstruction, if present, is the double-bubble sign produced by an enlarged stomach and proximal duodenum with little gas in the remainder of the bowel.
Upper gastrointestinal series is the study of choice in patients who are stable.
Normal rotation is present if the duodenal C-loop crosses the midline and places the duodenojejunal junction to the left of the spine at a level greater than or equal to the pylorus.
If contrast ends abruptly or tapers in a corkscrew pattern, midgut volvulus or some other form of proximal obstruction may be present.
Barium is the contrast of choice in patients who are stable or have chronic symptoms. Contrast studies may not be possible in patients who are actively vomiting or are otherwise unstable and need immediate surgical exploration. Water-soluble agents should be used if the study must be performed prior to imminent surgery.
Lower gastrointestinal series (contrast enema.) Occasionally, upper GI series findings may be indeterminate for the location of the duodenojejunal junction. In these cases, lower GI series may be used to identify location of the cecum.
Lower GI series can also rule out colonic obstruction and ileal atresia. However, a normally placed cecum does not unequivocally rule out a malrotation, and clinical judgment must be exercised.
Ultrasonography. In the hands of experienced ultrasonographers, ultrasonography has been shown to be very sensitive (approximately 100%) in detecting neonatal malrotation.
Highest sensitivity is achieved when inversion of the SMA and the superior mesenteric vein (SMV) is shown. Other diagnostic findings are fixed midline bowel loops and duodenal dilation with distal tapering.
Also, volvulus is highly probable if the SMV is shown to be coiling around the SMA.
All features are enhanced if water is instilled first by nasogastric (NG) tube.
The presence of ascites and thickened bowel wall were not found to be statistically significant predictors of malrotation with midgut volvulus.
Computed tomography scanning. CT scanning is not well developed for diagnosing malrotation and midgut volvulus. Scattered case reports of its use exist, but it is not recommended as the principal diagnostic tool.
Treatment
Medical Care. Medical care is directed toward stabilizing the patient.
Maintain patients oothing by mouth (NPO) and adjust NG or orogastric tube to low intermittent suction.
Correct fluid and electrolyte deficits.
Administer broad-spectrum antibiotics prior to surgery, if possible.
If a patient has signs of shock, administer appropriate fluids, blood products, and vasopressor medications to improve hypotension. Dopamine is used as first-line therapy because of its possible effects to increase splanchnic blood flow.
If the patient is unstable, do not delay surgical intervention for upper GI and laboratory studies. Quick surgical intervention, not prolonged medical management, produces the best results if midgut volvulus is suspected.
Surgical Care. The Ladd procedure remains the cornerstone of surgical treatment for malrotation today. Prior to William Ladd’s publication in 1936, surgical treatment for malrotation with or without volvulus had a mortality rate higher than 90%. In fact, at Ladd’s own institution, the mortality rate was 100% before the development of his new technique. A classic Ladd procedure is described as reduction of volvulus (if present), division of mesenteric bands, placement of small bowel on the right and large bowel on the left of the abdomen, and appendectomy. Published reports for laparoscopic Ladd procedure are now appearing in the literature as well.
Laparoscopy has been used to repair malrotation with signs of duodenal obstruction but no midgut volvulus.
Nutrition.
Patients require central venous catheter access for total parenteral nutrition until full oral feedings can be reestablished.
Furthermore, most of these patients have some degree of protein calorie malnutrition prior to surgery.
Achieve positive nitrogen balance as soon as possible by use of intravenous amino acid solutions.
Studies have shown that more malnourished patients are more likely to have ischemic bowel at surgery and need longer recovery times.
Appropriate nutrition is also necessary for wound healing and for protection from bacterial overgrowth in the GI tract.
Maintain maximum total parenteral nutrition until the patient is able to consume at least 50% of daily caloric requirement. Parenteral nutrition can then be weaned slowly as full enteral intake is being achieved.
Meconium ileus
Meconium ileus (MI) is among the most common causes of intestinal obstruction in the newborn, accounting for 9-33% of neonatal intestinal obstructions. MI is the earliest clinical manifestation of cystic fibrosis (CF) and occurs in approximately 16% of patients with CF, although MI also occurs in patients who do not have CF.
Genetic causes of cystic fibrosis.
CF is an autosomal recessive disease; its estimated heterozygote frequency in white people is
Pathophysiology.
Meconium in patients with MI has higher protein and lower carbohydrate concentration than that in control populations. In 1958, Green, Clarke, and Schwachman found that albumin was the major protein present in the meconium of infants with MI. Addition of albumin to normal meconium makes it viscid; addition of pancreatic protease liquefies the viscid mass. This led to the belief that pancreatic insufficiency played a central role in MI pathogenesis.
Abnormal intestinal motility also may contribute to MI development. Some patients with CF have prolonged small intestinal transit times. Non-CF diseases associated with abnormal gut motility (eg, Hirschsprung disease, chronic intestinal pseudo-obstruction) have been associated with MI-like disease, suggesting that decreased peristalsis may allow increased resorption of water, thus favoring MI development.
Postnatally, intestinal disease is characterized by a glandular abnormality that produces hyperviscous mucus. How mutations of the CF gene generate abnormal mucins is not fully described, nor is the developmental sequence of mucin secretion in the fetal intestine, although the CFTR ion channel defect possibly leads to dehydration of intraluminal contents. The meconium of fetuses with CF and MI has increased viscosity and decreased water content compared to normal controls.
Clinical signs
Simple MI usually presents with abdominal distension at birth, eventually progressing to failure to pass meconium, bilious vomiting, and progressive abdominal distension. Often, examination reveals dilated loops of bowel with a doughy character that indent on palpation. The rectum and anus usually are narrow, a finding possibly misinterpreted as anal stenosis.
Complicated MI presents more dramatically at birth with severe abdominal distension, sometimes accompanied by abdominal wall erythema and edema. Abdominal distension may be severe enough to cause respiratory distress. Signs of peritonitis include tenderness, abdominal wall edema, distension, and clinical evidence of sepsis. A palpable mass may indicate pseudocyst formation. Often, the neonate is in extremis and needs urgent resuscitation and surgical exploration.
CF is characterized clinically by the following triad:
Chronic obstruction and infection of the respiratory tract
Exocrine pancreatic insufficiency
Elevated sweat chloride levels
Imaging Studies.
Ultrasound evaluation. Prenatal sonographic characteristics associated with MI include hyperechoic masses (ie, inspissated meconium in the terminal ileum), dilated bowel, and inability to visualize the gallbladder.
Abdominal x-ray.
In uncomplicated MI, abdominal radiography reveals a characteristic pattern of unevenly dilated loops of bowel with variable air-fluid levels. Air-fluid levels may be absent due to the viscid, non-liquid nature of the inspissated meconium. Bubbles of gas may become evident as air mixes with the tenacious meconium. While this soap-bubble appearance depends on the viscosity of the meconium and is not a constant feature, this radiographic feature is pathognomonic and distinguishes MI from other causes of newborn intestinal obstruction. Although none of these features alone is diagnostic for MI, they strongly suggest the diagnosis when combined with a family history of CF.
Radiologic findings in complicated MI vary, based upon the associated complication. Speckled calcification visible on abdominal plain films strongly suggests intrauterine intestinal perforation and meconium peritonitis. Visible obstruction and a large dense mass with a rim of calcification suggest a pseudocyst.
When MI is suspected based on clinical and radiographic evidence, a contrast barium enema may be performed for diagnosis. If MI is likely, follow the contrast enema with a therapeutic Gastrografin enema. Some physicians advocate water-
A diagnosis of CF should be confirmed or refuted by a sweat test. A sweat test may be performed any time after the first 48 hours of life if the neonate is not edematous. The minimum amount of sweat needed is either 75 mg, a quantity that may be difficult to obtain from young infants. Never pool sweat from multiple sites to obtain the required quantity because the rate of sweating determines electrolyte content. Value over 60 meq/L of sweat sodium or chloride are diagnostic.
Mutation analysis, performed on buccal or blood cells, helps confirm the diagnosis if it yields at least 1 known CF mutations.
Treatment
Medical therapy. Manage both simple and complicated MI iewborns as an intestinal obstruction. Perform resuscitative measures, including mechanical respiratory support, if necessary. Initiate intravenous hydration with gastric decompression, evaluate and correct any coagulation disorders, and begin empiric antibiotic coverage. Immediately obtain a surgical evaluation when MI is suspected or diagnosed.
Gastrografin enemas.
In 1969, Noblett introduced the use of Gastrografin enemas to treat 4 infants with MI. Variations on this approach are now the preferred initial method to treat uncomplicated MI.
Gastrografin is meglumine diatrizoate, a hyperosmolar, water-soluble, radiopaque solution containing 0.1% polysorbate 80 (Tween 80) and 37% organically bound iodine. The solution’s osmolarity is 1900 mOsm/L.
Noblett’s criteria for proceeding with this therapy require the following:
The initial diagnostic contrast enema must exclude other causes of neonatal distal intestinal obstruction.
The infant must show signs of uncomplicated MI and no clinical or radiologic evidence of complicating factors (eg, volvulus, gangrene, perforation, peritonitis, atresia of the small bowel).
The infant should be well prepared for the enema, with adequate fluid and electrolyte replacement and correction of hypothermia.
The enema must be performed under fluoroscopic control.
Intravenous antibiotics should be administered.
Procedure.
Upon instillation, fluid is drawn into the intestinal lumen to hydrate and soften the meconium mass. Both transient osmotic diarrhea and diuresis follow. Adequate resuscitation and hydration in anticipation of these fluid losses is paramount.
Under fluoroscopic control, infuse a 25-50% solution of Gastrografin slowly at low hydrostatic pressure through a catheter inserted into the rectum. Avoid balloon inflation to minimize the risk of rectal perforation.
To help deconcentrate the inspissated meconium, 1% N-acetylcysteine may be added to the enema solution. The procedure requires a slow infusion, carefully monitored under fluoroscopy.
Obtain radiographs in 8-12 hours, or as clinically indicated, to confirm evacuation of the obstruction and to exclude late perforation.
If necessary, serial Gastrografin enemas can be performed at 6- to 24-hour intervals.
Surgical exploration is indicated for patients with progressive distension, signs of peritonitis, or clinical deterioration.
Following successful evacuation and resuscitation, Noblett suggests administering a 10% N-acetylcysteine solution (5 mL q6h) through a nasogastric (NG) tube to liquefy upper GI secretions.
Feedings, including supplemental pancreatic enzymes for infants with confirmed CF, may be initiated when signs of obstruction have subsided, usually within 48 hours.
Potential complications.
Perforation.
Hypovolemic shock is a profound risk when delivering hypertonic enemas.
Ischemia caused by overdistension is worsened by hypoperfusion; this hypoperfusion is caused by the hypovolemia that results from poor fluid resuscitation.
Surgical therapy. A number of surgical approaches to treat uncomplicated MI have been proposed over the years; variable success rates have been achieved. Individualize the approach for each infant.
The goal of operative management in simple uncomplicated MI is to evacuate meconium from the intestine while preserving maximal intestinal length.
Surgery always is indicated for complicated MI. Complicated MI requires resection more often than simple MI and always requires temporary stomas.
Nutritional management. Infants with uncomplicated MI and CF may receive breast milk or routine infant formula, enzymes, and vitamins.
Patients who have a complicated surgical course require either continuous enteral feedings or total parenteral nutrition (TPN).
IRRITABLE BOWEL SYNDROME
EPIDEMIOLOGY
The symptoms associated with irritable bowel syndrome (IBS) are experienced by up to 20% of the population in the West. Although most sufferers will not consult a doctor, the condition still represents 50% of referrals to gastroenterologists. It is a transcultural condition and and is recognised in Africa, India and China. It is more common in urban populations, and is twice as prevalent in women. Symptoms tend to begin in the teens and twenties and decrease with age but the condition may be lifelong.
CLINICAL FEATURES
There is a host of symptoms that are associated with IBS but the following are the most important.
Fig. Clinical features of IBS.
Abdominal pain
This is the central feature and is usually described as colicky or constant, particularly in the lower abdomen or left iliac fossa. However, the pain may take on a variety of qualities and may be located anywhere within the abdomen. The intensity of the pain varies from intermittently, mildly annoying to extremely severe. It may be present at any time of day or night but it is unlikely to awaken sufferers from their sleep. It is frequently worsened by eating and relieved by defaecation.
Altered bowel habit.
It is worth remembering that the range of normality for defaecation is between once every 3 days and three times a day. The bowel habit in IBS is most often alternating in that sufferers describe periods of infrequent, hard often ‘pellet-like’ motions interspersed with increased frequency of looser stools. It is usually possible to determine a diarrhoea-or constipation- predominant IBS type, which has implications for treatment strategies. There is often urgency, a feeling of incomplete evacuation and passage of mucus associated with defaecation. Rectal bleeding, steatorrhoea and nocturnal defaecation are not features of IBS and warrant further investigation. Passage of mucus is often described as being increased by sufferers but a mechanism for this has not been found nor has it been reliably documented.
Bloating.
A sensation of abdominal distension is often described although it is quite difficult to demonstrate this consistently in IBS sufferers. Younger women report that they feel as if they are 9 months pregnant. This symptom may be the result of increased intestinal gas, which is probably swallowed air, but may also reflect altered intestinal motility.
Non-colonic gastrointestinal symptoms.
Frequent associated symptoms are of heartburn, nausea, postprandial fullness and pain which may be attributable to the gallbladder or biliary tree. This may be due to a generalized smooth muscle abnormality.
Extra-intestinal symptoms.
These include urinary frequency and dysuria. Dyspareunia may be present if specifically enquired about, and there may be features of fibromyalgia or chronic fatigue syndrome. Psychological factors may be relevant as there does appear to be an increased incidence of depressive illness and neuroticism amongst sufferers. In order to try to standardise the diagnosis, first Manning in 1978 described a series of symptoms which positively discriminated for IBS and subsequently in Rome these symptoms were refined. However, these symptoms commonly occur in other organic gut conditions.
PATHOPHYSIOLOGY
Table 1 Rome criteria for the diagnosis of IBS
Perhaps because of the heterogeneous nature of the condition and lack of a definitive diagnostic test, elucidating the cause or causes of symptoms has been unsuccessful. Although no single, consistent feature has been identified, abnormalities have been detected in:
• gastrointestinal motility – there are shorter transit times and hypomotility in diarrhoea-predominant IBS, and reduced, high amplitude, peristaltic contractions in constipation-predominant IBS. The observed motility changes, however, do not correlate well with clinical features.
• altered visceral sensation – increased sensitivity to inflated balloons in both small and large bowel has been demonstrated and increased rectal sensitivity is a common finding.
• psychological abnormalities – both sufferers and doctors recognise the effect of psychological stress on the symptoms, but quantifying this is difficult. Psychological symptoms are more prevalent in IBS sufferers, particularly in those referred to hospital and up to 60% may fulfil diagnostic criteria for mental disorders such as depression and anxiety. Disease phobia and bodily preoccupation are also more common. Some patients describe the onset of their symptoms following an episode of gastroenteritis and there does not appear to be a major psychological component to their condition.
• endocrine changes – many women recognise that the symptoms of IBS are more marked during menstruation. No obvious hormonal correlations have been made but there are increased levels of prostaglandin E2 and F2 around this time and this may be important. Symptoms often worsen following hysterectomy which is presumably not explained by hormonal changes but may be due to damage to pelvic nerves at the time of surgery. Unfortunately, some patients undergo hysterectomy when the pain is actually caused by IBS which persists after the operation – a problem that needs to be recognised by gynaecologists.
MANAGEMENT
A thorough history is of prime importance because of the lack of a diagnostic test and broad differential diagnosis that the symptoms of IBS create. It was formerly taught that the diagnosis should be made positively and not by excluding other conditions, but some diagnoses are excluded by the history and examination and others excluded by simple tests. During the history-taking, special attention should be given to ensure that sinister symptoms such as marked weight loss, rectal bleeding, steatorrhoea, nocturnal diarrhoea, and associated skin or joint symptoms are not present. In addition to a general examination, sigmoidoscopy should be carried out and a rectal biopsy taken, particularly in diarrhoea-predominant IBS. Blood investigations should include full blood count, biochemistry, liver function tests, and the inflammatory markers: erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). With a good history and a normal result from the above investigations, a positive diagnosis of IBS can be made, particularly in the younger age group (<40 years). It is prudent to include further colonic examination such as barium enema studies in the older age group to exclude colonic neoplasia. Over-investigation may simply serve to convince sufferers that the physician is not sureof the diagnosis and is best avoided. Occasionally, factors will confound the diagnosis such as a slightly raised CRP which will usually warrant further GI investigations but may be due to many non-GI conditions.
TREATMENT
Successful treatment of sufferers with IBS takes considerable skill on the part of the physician. The approach taken at the time of diagnosis will have long-term effects on how patients view their condition. Careful discussion of possible mechanisms of the causes of pain and relevant trigger factors such as diet and anxiety and the universal nature of the condition will often serve to reassure sufferers.
THERAPEUTIC OPTIONS
Dietary manipulation An increase in dietary fibre has been favoured advice for years but makes as many sufferers worse as it does better. It is most useful in constipation-predominant IBS but may worsen bloating. Exclusion diets whereby various food types are removed then subsequently reintroduced into the diet until triggers are found may be beneficial in some cases but are a protracted and rather arduous treatment. Lactose intolerance may affect 10% of the population and contribute to symptoms of diarrhoea and bloating. Exclusion of dairy products from the diet is probably the easiest way to confirm this although a lactose breath test can also be used. Patients will often experiment with their diet themselves and may try unsubstantiated protocols such as low yeast diets which will usually do no harm.
Drugs
Anticholinergics such as dicyclomine and hyoscine may help pain and diarrhoea but can have side-effects with urinary retention and effects on intraocular pressures. Antispasmodics such as mebeverine and peppermint-based products (particularly for constipation-dominant IBS) may help pain and bloating and are widely used as they do not have anticholinergic side-effects. Antidepressants have long been used in patients with severe IBS and it may be most appropriate to consider a tricyclic for diarrhoea-predominant IBS and a selective serotonin reuptake inhibitor for constipation-predominant IBS. Prokinetics may help post-prandial fullness, bloating and constipation but worsen diarrhoea-predominant IBS. If constipation does not respond to adequate bulking of the stool or an osmotic laxative then a stimulant laxative may be required. Likewise, only if diarrhoea is intractable and troublesome should constipating agents such as loperamide be used.
Complementary therapies
Hypnotherapy, stress management, psychotherapy and acupuncture have all been used and may help some sufferers.
INFLAMMATORY BOWEL DISEASES
Crohn’s disease (CD) and ulcerative colitis (UC) are characterized by chronic inflammation at various sites in the GI tract. Both cause diarrhea, which may be profuse and bloody. Certain differences in disease patterns justify a distinction between Crohn’s disease and ulcerative colitis, although groupings and subgroupings are somewhat artificial. Some cases are difficult, if not impossible, to classify.
The term colitis applies only to inflammatory disease of the colon (eg, ulcerative, granulomatous, ischemic, radiation, or infectious colitis). Spastic or mucous colitis is a misnomer often applied to a functional disorder that is more properly described as irritable bowel syndrome.
CROHN’S DISEASE
A nonspecific chronic transmural inflammatory disease that most commonly affects the distal ileum and colon but may occur in any part of the GI tract.
Etiology and Epidemiology
The fundamental cause of Crohn’s disease is unknown. Evidence suggests that, a genetic predisposition leads to an unregulated intestinal immune response to an environmental, dietary, or infectious agent. However, no inciting antigen has been identified. Cigarette smoking seems to contribute to the development or exacerbation of Crohn’s disease.
Over the past few decades, incidence of Crohn’s disease has increased in Western populations of Northern European and Anglo-Saxon ethnic derivation, third-world populations, blacks, and Latin Americans. The disease occurs about equally in both sexes and is more common among Jews. Approximately one of six patients has at least one first-degree relative with the same disease or, less frequently, with ulcerative colitis. Most cases begin in patients < 30 yr, with the peak incidence in those aged 14 to 24 years.
Pathology
The earliest mucosal lesion of Crohn’s disease is crypt injury in the form of inflammation (cryptitis) and crypt abscesses, which progress to tiny focal aphthoid ulcers, usually located over nodules of lymphoid tissue. In some cases, these lesions regress; in others, the inflammatory process evolves with influx and proliferation of macrophages and other inflammatory cells, occasionally forming noncaseating granulomas with multinucleated giant cells.
Transmural spread of inflammation leads to lymphedema and bowel wall thickening, which may eventually result in extensive fibrosis. Development of patchy mucosal ulcers and longitudinal and transverse ulcers with intervening mucosal edema frequently creates a characteristic cobblestoned appearance. The attached mesentery is thickened and lymphedematous; mesenteric fat typically extends onto the serosal surface of the bowel. Mesenteric lymph nodes often enlarge. Transmural inflammation, deep ulceration, edema, muscular proliferation, and fibrosis cause deep sinus tracts and fistulas, mesenteric abscesses, and obstruction, which are the major local complications. Granulomas can occur in lymph nodes, peritoneum, the liver, and all layers of the rowel wall and are occasionally seen at laparotomy or laparoscopy as miliary nodules. Although pathognomonic, granulomas are absent in up to 50 % of patients and are therefore not essential to diagnose Crohn’s disease. They appear to have no definitive bearing on the clinical course.
Segments of diseased bowel are characteristically sharply demarcated from adjacent normal bowel (“skip areas”)—thus the name regional enteritis. Of all cases of Crohn’s disease, about 33% involve the ileum ileitis); about 45% involve the ileum and colon (ileocolitis), with a predilection for the right side of the colon; and about 15 % involve the colon alone (granulomatous colitis). Occasionally, the entire small bowel is involved jejunoileitis), and rarely, the stomach, duodenum, or esophagus. The perianal region is also affected in 1/4 to 1/3 of cases.
The spectrum of Crohn disease presentations includes (a) gastroduodenitis (7% of patients), (b, c) jejunoileitis and ileitis (33% of patients), (d) ileocolitis (45% of patients), and (e) colitis (15% of patients).
Symptoms, Signs, and Complications
Chronic diarrhea with abdominal pain, fever, anorexia, weight loss, and a right lower quadrant mass or fullness are the most common presenting features. However, many patents are first seen with an acute abdomen that simulates acute appendicitis or intestinal obstruction. About 1/3 of patients have a history of perianal disease, especially fissures and fistulas, which are sometimes the most prominent or even initial complaint. In children, extraintestinal manifestations frequently predominate over GI symptoms. Arthritis, fever of unknown origin, anemia, or growth retardation may be a presenting symptom, and abdominal pain or diarrhea may be absent.
The most common patterns of Crohn’s disease pathology are (1) inflammation characterized by right lower quadrant abdominal pain and tenderness; (2) recurrent partial obstruction caused by intestinal stenosis and leading to severe colic, abdominal distention, constipation, and vomiting; (3) diffuse jejunoileitis, with inflammation and obstruction resulting in malnutrition and chronic debility; and (4) abdominal fistulas and abscesses, usually late developments, often causing fever, painful abdominal masses, and generalized wasting.
Enterocutaneous fistulae in Chrohn’s disease
Obstruction; development of enteroenteric, enterovesical, retroperitoneal, or enterocutaneous fistulas; and abscess formation are common complications of inflammation. Intestinal bleeding, perforation, and small-bowel cancer develop rarely. When the colon alone is affected, the clinical picture may be indistinguishable from that of ulcerative colitis.
Endoscopic spectrum of Crohn disease includes (a) aphthous ulcerations amid normal colonic mucosal vasculature; (b) deeper, punched-out ulcers in ileal mucosa; (c) a single colonic linear ulcer; and (d) deep colonic ulcerations forming a stricture.
Extraintestinal manifestations are categorized as:
Complications that usually parallel the activity of the intestinal disease and possibly represent acute immunologic or microbiologic concomitants of the bowel inflammation: peripheral arthritis, episcleritis, aphthous stomatitis, erythema nodosum, and pyoderma gangrenosum. These manifestations may be reported by > 1/3 of patients hospitalized with inflammatory bowel disease. They are twice as common when colitis is present as when disease is confined to the small bowel. When extraintestinal manifestations occur, they are multiple in about 1/3 of patients. Disorders associated with inflammatory bowel disease but running an independent course: ankylosing spondylitis, sacroiliitis, uveitis, and primary sclerosing cholangitis. The genetic association of these syndromes and of Crohn’s disease (and ulcerative colitis) with the HLA antigen B27 is discussed under the extracolonic complications of ulcerative colitis, below. Complications that relate directly to disrupted bowel physiology: kidney stones from disorders of uric acid metabolism, impaired urinary dilution and alkalinization, and excessive dietary oxalate absorption; urinary tract infections, especially with fistulization into the urinary tract; and hydroureter and hydronephrosis from ureteral compression by retroperitoneal extension of the intestinal inflammatory process. Other bowel-related complications include malabsorption, especially in the face of extensive ileal resection or bacterial overgrowth from chronic small-bowel obstruction or fistulization; gallstones, related to impaired ileal reabsorption of bile salts; and amyloidosis, secondary to long-standing inflammatory and suppurative disease. Thromboembolic complications may occur, usually with severe disease activity, as a result of hypercoagulability associated with altered levels of clotting factors and platelet abnormalities.
Diagnosis
Crohn’s disease should be suspected in a patient with the inflammatory or obstructive symptoms described above and in a patient without prominent GI symptoms but with perianal fistulas or abscesses or with otherwise unexplained arthritis, erythema nodosum, fever, anemia, or stunted growth (in a child).
Laboratory findings are nonspecific and may include anemia, leukocytosis, hypoalbuminemia, and increased levels of acute-phase reactants reflected in elevated ESR, C-reactive protein, or orosomucoids. Elevated alkaline phosphatase and γ-glutamyltranspeptidase accompanying colonic disease often reflect primary sclerosing cholangitis.
Definitive diagnosis is usually made by x-ray. Barium enema x-ray may show reflux of barium into the terminal ileum with irregularity, nodularity, stiffness, wall thickening, and a narrowed lumen.
X-ray showing abnormal terminal ileum in Crohn’s disease
A small-bowel series with spot x-rays of the terminal ileum usually most clearly shows the nature and extent of the lesion. An upper GI series without small-bowel follow-through usually misses the diagnosis.
In advanced cases, the string sign may be seen with marked ileal strictures and separation of bowel loops. In earlier cases, x-ray diagnosis may sometimes be difficult, but air double-contrast barium enema and enteroclysis may show superficial aphthous and linear ulcers. In questionable cases, colonoscopy and biopsy may help confirm the diagnosis of Crohn’s colitis and allow direct visualization and biopsy of the terminal ileum. Upper GI endoscopy may identify gastroduodenal involvement in Crohn’s disease patients with upper GI symptoms. Although CT can detect extramural complications (eg, fistulas, abscesses, masses), it is not routinely needed for initial diagnosis. Ultrasound may help delineate gynecologic pathology in women with lower abdominal and pelvic pain.
Differential Diagnosis
Differentiation from ulcerative colitis may be difficult in the 20% of cases in which Crohn’s disease is confined to the colon (Crohn’s colitis). The principal differential diagnoses are acute infectious (self-limited) colitis and ulcerative colitis. Acute infectious colitis is best established by stool culture, rectal biopsy, and watchful waiting. Although perinuclear antineutrophil cytoplasmic antibodies are present in 60 to 70% of ulcerative colitis patients and in only 5 to 20% of Crohn’s disease patients, and anli-Saccharomyces cerevisiae antibodies are relatively specific for Crohn’s disease, these tests are not sufficiently refined in routine clinical application as to reliably separate the two diseases.
Crohn’s disease of the small bowel (ileitis) requires differentiation from other inflammatory, infectious, and neoplastic disorders in the right lower quadrant. If in the acute presentation a prior history of chronic bowel symptoms has not been elicited, ileitis may be first diagnosed during surgical exploration for suspected acute appendicitis. Periappendiceal abscess may produce more chronic symptoms and thus be more difficult to diagnose clinically.
Pelvic inflammatory disease, ectopic pregnancy, and ovarian cysts and tumors also produce right lower quadrant inflammatory signs and must be ruled out when considering Crohn’s disease in women. Cancer of the cecum, ileal carcinoid, lymphosarcoma, systemic vasculitis, radiation enteritis, ileocecal TB, and ameboma may mimic the x-ray findings of Crohn’s disease. AIDS-related opportunistic infections (eg, Mycobacterium avium-intracellulare, cytomegalovirus) must also be considered as causes of localized ileitis. Yersinia enterocolitica enteritis must be excluded if an inflamed, edematous terminal ileum and associated mesenteric adenitis are seen during surgery for acute right lower quadrant pain. Although Yersinia enteritis is self-limited without chronic intestinal sequelae, the initial clinical picture may be indistinguishable from Crohn’s disease, so appropriate serologic and bacteriologic studies are necessary. In questionable cases, a 3-mo follow-up x-ray of the terminal ileum is valuable, because Yersinia enteritis will usually resolve completely by this time but Crohn’s disease will not.
Nongranulomatous ulcerative jejunoileitis has features of both Crohn’s disease and sprue, with malabsorption, small-bowel ulceration, and villous atrophy, but it lacks granulomatous pathology, fistulization, and extraintestinal manifestations of Crohn’s disease. Eosinophilic gastroenteritis generally has prominent gastric involvement (rare in Crohn’s disease) and is often associated with peripheral eosinophilia, which is the clue to diagnosis.
Prognosis
Although spontaneous remission or medical therapy may result in a prolonged asymptomatic interval, established Crohn’s disease is rarely cured but instead is characterized by intermittent exacerbations. In the absence of surgical intervention, the disease never extends into new areas of small bowel beyond its initial distribution at first diagnosis. With judicious medical and, where appropriate, surgical therapy, most patients with Crohn’s disease function well and adapt successfully. Disease-related mortality is very low and continues to decrease.
GI cancer, including cancer of the colon and small bowel, is the leading cause of Crohn’s disease-related death. Patients with long-standing Crohn’s disease of the small bowel are at increased risk of small-bowel cancer, with bowel in continuity as well as in bypassed loops. Furthermore, patients with Crohn’s disease of the colon have a long-term risk of colorectal cancer equal to that of ulcerative colitis, given the same extent and duration of disease.
Approximately 70% of Crohn’s disease patients ultimately require surgery. Furthermore, Crohn’s disease is likely to recur even after resection of all clinically apparent disease.
TABLE DIFFERENTIATING BETWEEN CROHN’S DISEASE AND ULCERATIVE COLITIS
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Crohn’s Disease
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Ulcerative Colitis |
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Small bowel is involved in 80% of cases. |
Disease is confined to the colon. |
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Rectosigmoid is often spared; colonic involvement is usually right-sided. |
Rectosigmoid is invariably involved; colonic involvement is usually left-sided. |
|
Gross rectal bleeding is absent in 15-25% of cases. |
Gross rectal bleeding is always present. |
|
Fistula, mass, and abscess development is common.
|
Fistulas do not occur. |
|
Perianal lesions are significant in 25-35%.
|
Significant perianal lesions never occur. |
|
On x-ray, bowel wall is affected asymmetrically and segmentally, with “skip areas” between diseased segments.
|
Bowel wall is affected symmetrically and uninterruptedly from rectum proximally (ahaustral |
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Endoscopic appearance is patchy, with discrete ulcerations separated by segments of normal-appearing mucosa. |
Inflammation is uniform and diffuse (continuous superficial inflammation with granular) |
|
Microscopic inflammation and fissuring extend transmurally; lesions are often highly focal in distribution. |
Inflammation is confined to mucosa (diffuse, continuous, superficial inflammation) except In severe cases. |
|
Epithelioid (sarcoid-like) granulomas detected in bowel wall or lymph nodes in 25-50% of cases (pathognomonic). |
Typical epithelial granulomas do not occur. |
Treatment
No cure is known. Cramps and diarrhea may be relieved by oral administration up to 4 times a day (ideally before meals) of anticholinergics, diphenoxylate 2.5 to 5 mg, loperamide 2 to 4 mg, deodorized opium tincture 0.5 to 0.75 mL (10 to 15 drops), or codeine 15 to 30 mg. Such symptomatic treatments are safe, except in cases of severe, acute Crohn’s colitis, which may progress to toxic megacolon as in ulcerative colitis. Hydrophilic mucilloids (eg, methylcellulose or psyllium preparations) sometimes help prevent anal irritation by increasing stool firmness.
Sulfasalazine primarily benefits patients with mild to moderate colitis and ileocolitis but has some efficacy in ileitis as well. It may also maintain remission, although it has not been proven to prevent recurrence after surgery.
Mesalamine (5-aminosalicylic acid), the active moiety of sulfasalazine, is available in several oral formulations designed to release in various segments of the small bowel and colon. It is especially useful in patients who are intolerant of sulfasalazine. In doses of up to 4 g/day, mesalamine is effective for inducing and maintaining remission and is showing considerable promise for inhibiting postoperative recurrence.
Corticosteroid therapy treats the acute stages of Crohn’s disease by dramatically reducing fever and diarrhea, relieving abdominal pain and tenderness, and improving the appetite and sense of well-being. Large doses of oral prednisone, 40 to 60 mg/day, should be given initially. The equivalent dose of hydrocortisone (200 to 300 mg) may be given by continuous IV drip to hospitalized patients with severe disease. The daily dose of prednisone is gradually reduced after a satisfactory response so that, after 1 or 2 mg, it is ≤ 10 mg.
Although as little as 5 or 10 mg/day of prednisone may help control symptoms in some patients, long-term therapy often does more harm than good. Corticosteroids should be avoided when obvious infections (eg, fistulas, abscesses) are present. In uncertain cases (eg, those with a tender, inflammatory mass), antibiotics should be given concurrently.
The new topically active corticosteroid budesonide can be given orally or as an enema and has low systemic bioavailability and thus reduced adrenal suppression. Controlled-release budesonide given orally induces remissions with somewhat fewer side effects than prednisolone, but it is not as effective as the conventional corticosteroid and seems no better than placebo in preventing relapses beyond 6 monthes.
Broad-spectrum antibiotics that are active against enteric gram-negative and anaerobic flora may help reduce disease activity in many patients but are most consistently effective for suppurative complications (eg, infected; fistula, abscess). Metronidazole 1 to 1.5 g/day is beneficial, especially in Crohn’s colitis, and is particularly useful for treating perianal lesions. Neuropathy manifested chiefly by paresthesias is a common, potentially serious side effect of long-term use; it is usually reversible when the drug is stopped. There is a high incidence of relapse after discontinuing the drug. Among other broad-spectrum antibiotics, ciprofloxacin has shown particular promise, but the results of multidrug antituberculous regimens have been mixed.
Immunomodulating drugs, particularly the antimetabolites azathioprine and 6-mercaptopurine, are effective as long-term therару. Azathioprine 2.0 to 3.5 mg/kg/day or 6- mercaptopurine 1.5 to 2.5 mg/kg/day orally significantly improves overall clinical status, decreases corticosteroid requirements, heals fistulas, and maintains remission for many years. However, these drugs often do not produce clinical benefits for 3 to 6 monthes and side effects of allergy, pancreatitis, and leukopenia must be watched for.
Methotrexate 25 mg IM or subcutaneously once/week benefits some patients with severe corticosteroid-refractory disease, even those who have failed to respond to azathioprine or 6-mercaptopurine. High-dose cyclosporine has demonstrated benefits in inflammatory and fistulous disease, but its long-term use is contraindicated by multiple toxicities. Infliximab, a monoclonal antibody that inhibits tumor necrosis factor, can be given I/V for moderate to severe Crohn’s disease (especially fistulous disease) refractory to other treatments; long-term efficacy and side effect remain to be determined. Other potential immunoregulatory treatments include inter-leukin-1 blockers, antibody to interleukin-12, anti-CD4 antibodies, adhesion molecule inhibitors, and down-regulatory cytokines These many experimental treatment approaches attest to the inadequacy of current: drug therapy for Crohn’s disease. Some patients with intestinal obstruction or fistulas have improved with elemental diets or hyperalimentation, at least over a short term, and children often achieve increased rates of growth. Thus, these measures may serve as preoperative or adjunc-tive therapy and may even be valuable as primary therapy.
Surgery.
Surgery is usually necessary for recurrent intestinal obstruction or intractable fistulas oг abscesses. Resection of the grossly involved bowel may ameliorate symptoms indefinitely but does not cure the disease. Sulfasalazine has not been shown to prevent postoperative recurrence, but mesalamine > 2 g/day may be effective. The recurrence rate, defined by endoscopic lesions at the anastomotic site, is > 70% at 1 year and > 85% at 3 years; defined by clinical symptoms, it is about 25 to 30% at 3 years and 40 to 50% at 5 years. Ultimately, further surgery is required in nearly 50% of cases. However, recurrence rates appear to be reduced by early postoperative prophylaxis with mesalamine, metronidazole, or possibly 6-mercaptopurine. Moreover, when surgery has been performed for specific complications or failure of medical therapy, most patients experience an improved quality of life.
ULCERATIVE COLITIS
A chronic, inflammatory, and ulcerative disease arising in the colonic mucosa, characterized most often by bloody diarrhea.
Etiology and Epidemiology.
The cause of ulcerative colitis is unknown. Evidence suggests that a genetic predisposition leads to an unregulated intestinal immune response to an environmental, dietary, or infectious agent. However, no inciting antigen has been identified. The evidence for a specific microbial etiology for ulcerative colitis is even less convincing than for Crohn’s disease, and the familial tendency is less pro-nounced. Unlike in Crohn’s disease, current cigarette smoking appears to decrease risk. Like Crohn’s disease, ulcerative colitis may afflict people at any age, but the age-onset curve shows a bimodal distribution, with a major peak at ages 15 to 30 and a second smaller peak at ages 50 to 70; however, this later peak may include some cases of ischemic colitis.
Pathology.
Pathologic changes begin with degeneration of the reticulin fibers beneath the mucosal epithelium, occlusion of the subepithelial capillaries, and progressive infiltration of the lamina propria with plasma cells, eosinophils, lymphocytes, mast cells, and polymorphonuclear leukocytes. Crypt abscesses, epithelial necrosis, and mucosal ulceration ultimately develop. The disease usually begins in the rectosigmoid and may extend proximally, eventually involving the entire colon, or it may involve most of the large bowel at once. Ulcerative proctitis, which is localized to the rectum, is a very common and more benign form of ulcerative colitis. It is often refractory to treatment and undergoes late proximal spread in about 20 to 30% of cases.
Symptoms and Signs
Bloody diarrhea of varied intensity and duration is interspersed with asymptomatic intervals. Usually an attack begins insidiously, with increased urgency to defecate, mild lower abdominal cramps, and blood and mucus in the stools. However, an attack may be acute and fulminant, with sudden violent diarrhea, high fever, signs of peritonitis, and profound toxemia. Some cases develop following a documented infection (eg, amebiasis, bacillary dysentery).
When ulceration is confined to the rectosigmoid, the stool may be normal or hard and dry, but rectal discharges of mucus loaded with RBCs and WBCs accompany or occur between bowel movements. Systemic symptoms are mild or absent. If ulceration extends proximally, stools become looser and the patient may have > 10 bowel movements/day, often with severe cramps and distressing rectal tenesmus, without respite at night. The stools may be watery, may contain mucus, and frequently consist almost entirely of blood and pus. Malaise, fever, anemia, anorexia, weight loss, leukocytosis, hypoalbuminemia, and elevated ESR may be present with extensive active ulcerative colitis.
Complications
Bleeding is the most common local complication. Another particularly severe complication, toxic colitis, occurs when transmural extension of ulceration results in localized ileus and peritonitis. As toxic colitis progresses, the colon loses muscular tone and begins to dilate within hours or days. Plain x-rays of the abdomen show intraluminal gas accumulated over a long, continuous, paralyzed segment of colon—a result of lost muscle tone.
Toxic megacolon (or toxic dilation) exists when the diameter of the transverse colon exceeds
Major perirectal complications, such as those in granulomatous colitis (eg, fistulas, abscesses), do not occur.
The incidence of colon cancer is increased when the entire colon is involved and the disease lasts for > 10 yr, independent of disease activity. After 10 yr, the cancer risk in extensive colitis appears to be about 0.5 to 1%/yr. Although cancer incidence is highest in cases of universal ulcerative colitis, the risk is significantly increased with any extent of ulcerative colitis above the sigmoid. There is probably no higher absolute cancer risk among patients with childhood-onset colitis, independent of the longer duration of disease. The long-term survival after diagnosis of colitis-related cancer is about 50%, a figure comparable to that for colorectal cancer in the general population.
Regular colonoscopic surveillance, preferably during remission, is advised for patients whose disease duration (as 8 to 10 yr) and extent (beyond rectosigmoidal) place them at high risk of developing colon cancer. Endoscopic biopsies should be taken throughout the colon and reviewed by an experienced pathologist. Any grade of definite, confirmed dysplasia is a strong indication for colectomy because the likelihood of concomitant or imminent colorectal cancer may be as high as 80 %. In such cases, corroboratory pathologic interpretation is important to distinguish between definite neoplas- tic dysplasia and reactive or regenerative atypia secondary to inflammation. However delaying colectomy in favor of repeated follow-up surveillance is unwise if dysplasia is unequivocal. Pseudopolyps have no prognostic significance but may be difficult to distinguish from neoplastic polyps; thus, any suspicious polyp should undergo excision biopsy.
Extracolonic problems.
Extracolonic problems include peripheral arthritis, ankylosing spondylitis, sacroiliitis, anterior uveitis, erythema nodosum, pyoderma gangrenosum, episcleritis, and in children, retarded growth and development. Peripheral arthritis, skin complications, and episcleritis often fluctuate with the colitis whereas spondylitis, sacroiliitis, and uveitis usually follow a course independent of the bowel disease. Most patients with spinal or sacroiliac involvement also have evidence of uveitis, and vice versa. These latter conditions may precede the colitis by many years and tend to occur more commonly in persons with the HLA-B27 antigen.
Erythema nodosum on the skin
Pyоderma gangrenosum seen in UC
Episcleritis in UC
Minor changes in liver function tests are common, but clinically apparent liver disease occurs in only 3 to 5% of patients. Liver disease may manifest as fatty liver or more seriously as autoimmune hepatitis, primary sclerosing cholangitis, or cirrhosis.
Primary sclerosing cholangitis (PSC) occurs in 5% of ulcerative colitis patients, most commonly in those who were young when the colitis began. PSC may precede symptomatic ulcerative colitis by many years and is mort reliably diagnosed by endoscopic retrograd-cholangiography than by liver biopsy. Some investigators believe that signs of subclinics ulcerative colitis, if systematically sought, could be found in all patients with PSC. A late complication of ulcerative colitis-associated PSC is cancer of the biliary tract which may appear even 20 yr after colectomy. More than 50% of PSC and cholangiocarcinoma cases in Western countries occur in patients with Crohn’s disease or ulcerative colitis.
Diagnosis
The history and stool examination permit a presumptive diagnosis of ulcerative colitis that should always be confirmed by sigmoidoscopy, which provides a direct, immediate indication of disease activity. Total colonoscopy is not usually necessary before treatment and may be hazardous in active stages because of the risk of perforation. In early cases, the mucous membrane is finely granular and friable, with loss of the normal vascular pattern and often with scattered hemorrhagic areas; minimal trauma (friability) causes bleeding in multiple pinpoint spots.
The endoscopic spectrum of ulcerative colitis includes (a) mucosal edema, erythema, loss of vasculature; (b) granular mucosa with pinpoint ulceration and friability; (c) regenerated (i.e., healed) mucosa with distorted mucosal vasculature; and (d) regenerated mucosa with typical postinflammatory pseudopolyps
The mucosa soon breaks down into a red, spongy surface dotted with many tiny blood- and pus-oozing ulcers. As the mucosa becomes progressively involved, the inflammation and hemorrhage extend into the bowel muscle. Large mucosal ulcers with copious purulent exudate characterize severe disease. Islands of relatively normal or hyperplastic inflammatory mucosa (pseudo-polyps) project above areas of ulcerated mucosa. Biopsies may be nonspecific and sometimes cannot exclude acute infectious (self-limited) colitis; however, features that suggest chronicity (eg, distorted crypt architecture, crypt atrophy, a chronic inflammatory infiltrate) support the diagnosis of ulcerative colitis. Even during asymptomatic intervals, the sigmoidoscopic appearance is rarely normal; some degree of friability or granularity almost always persists. There is loss of the normal vascular pattern, and biopsy shows evidence of chronic inflammation.
Plain x-rays of the abdomen sometimes help to judge the severity and proximal extent of the colitis by showing loss of haustration, mucosal edema, and absence of formed stool in the diseased bowel. Barium enema, like colonoscopy, is not usually necessary before treatment and may be hazardous in active stages because of risk of perforation. Later in the course of disease, however, the entire colon should be evaluated to determine the extent of involvement. Total colonoscopy is the most sensitive and widely used method, although barium enema can be informative. Barium studies show loss of haustration, mucosal edema, minute serrations, or gross ulcerations in severe cases. A shortened, rigid colon with an atrophic or pseudopolypoid mucosa is often seen after several years’ duration.
In this air-contrast radiograph of ulcerative colitis, the mucosal pattern is granular with loss of normal haustrations in a diffuse, continuous pattern.
Colonoscopy with biopsy is mandatory to evaluate the nature of a stricture. Biopsy may also help distinguish ulcerative colitis from Crohn’s disease if the inflammation is highly focal or if a granuloma is seen.
Differential Diagnosis
It is of utmost importance to exclude an infectious cause of acute colitis before treatment, especially during the first attack. Stool cultures for Salmonella, Shigella, and Campylobacter must be obtained, and Entamoeba histolytica should be excluded by examination of fresh, still warm stool specimens or of colonic exudate aspirated at sigmoidoscopy. Mucosal biopsies may yield additional etiologic information. When amebiasis is suspected because of epidemiologic or travel history, serologic titers should be obtained in addition to biopsies.
History of prior antibiotic use should prompt stool assay for Clostridium difficile toxin. A detailed sexual history should be obtained, particularly in male homosexuals, to rule out specific sexually transmitted diseases, such as gonorrhea, herpesvirus, and chlamydia. Opportunistic infections (eg, cytomegalovirus, Mycobacterium avium-intracettulare) must also be considered in immunosuppressed patients. In women using birth control pills, contraceptive-induced colitis is possible; it usually resolves spontaneously after hormone therapy is stopped. In elderly patients, especially those with a history of atherosclerotic heart disease, ischemic colitis should be considered. X-ray findings of thumbprinting and segmental distribution would further suggest this diagnosis.
Severe perianal disease, rectal sparing, absence of bleeding, and asymmetric or segmental involvement of the colon indicate Crohn’s rather than ulcerative colitis.
Prognosis
Usually, ulcerative colitis is chronic with repeated exacerbations and remissions. A rapidly progressive initial attack becomes fulminant iearly 10% of patients, with complications of massive hemorrhage, perforation, or sepsis and toxemia. Complete recovery after a single attack may occur in another 10%; however, there always remains the possibility of an undetected specific pathogen.
Nearly 1/3 of patients with extensive ulcerative colitis require surgery. Total proctocolectomy is curative: Life expectancy and quality of life are restored to normal, and the risk of colon cancer is eliminated.
Patients with localized ulcerative proctitis have the best prognosis. Severe systemic manifestations, toxic complications, and malignant degeneration are unlikely, and late extension of the disease occurs in only about 20 to 30%. Surgery is rarely required, and life expectancy is normal. The symptoms, however, may prove exceptionally stubborn and refractory. Moreover, because extensive ulcerative colitis may begin in the rectum and spread proximally, localized proctitis should not be definitively diagnosed until it has stayed localized for > 6 monthes. Localized disease that later extends is often more severe and more refractory to therapy.
Treatment
Avoiding raw fruits and vegetables limits mechanical trauma to the inflamed colonic mucosa and may lessen symptoms. A milk-free diet may help but need not be continued if no benefit is noted. An anticholinergic drug or loperamide 2.0 mg or diphenoxylate 2.5 mg orally 2 times a day to 4 times a day is indicated for relatively mild diarrhea; higher oral doses of loperamide (4 mg in the morning and 2 mg after each bowel movement) or diphenoxylate (б mg 3 times a day or 4 times a day), deodorized opium tincture 0.5 to 0.75 mL (10 to 15 drops) every 4 to 6 hours, or codeine 15 to 30 mg every 4 to 6 hours may be required for more intense diarrhea. These antidiarrheal drugs must be used with extreme caution in more severe cases because they may precipitate toxic dilation.
In mild or moderate disease that does not extend proximally beyond the splenic flexure, remission may sometimes be achieved with a hydrocortisone enema instead of with oral corticosteroid therapy. Initially, hydrocortisone 100 mg in 60 mL of isotonic solution is given rectally once or twice/day. It should be retained in the bowel as long as possible; instillation at night, with the patient’s hips elevated, may prolong retention and extend distribution. Treatment, if effective, should be continued daily for about 1 wk, then every other day for 1 to 2 wk, then discontinued gradually over 1 to 2 wk. Because systemic side effects may occur as with oral corticosteroids, enema preparations of new corticosteroid analogs such as budesonide, which is topically potent but less systemically active, are becoming more widely used outside the USA.
Mesalamine may also be given by enema and is beneficial in many cases of refractory proctosigmoiditis and left-sided colitis. The standard dose is mesalamine
More extensive mild or moderate disease as well as localized disease may respond to oral sulfasalazine. Because GI intolerance common, the drug should be given with food and, if necessary, in the enteric-coated form. To minimize common side effects (eg, nausea, dyspepsia, headache), dosage should initially be low (eg,
Once remission is achieved, long-tem maintenance therapy with sulfasalazine 1 to 3 g/day is indicated to prevent relapse. The sulfapyridine component of sulfasalazine; interferes with folic acid absorption, so folate supplementation of 1 to 2 mg /day is generally recommended. Patients with chronic fecal blood loss may also require iron to prevent anemia. New oral analogs of sulfasalazine have been developed to eliminate the sulfapyridine moiety, which is responsible most of the common side effects, while allowing delivery of mesalamine to diseased reas of the small intestine and colon. Clinical trials have shown that olsalazine, a mesalamine dimer, is effective in treating mild-to-moderate colitis and maintaining remission. Like sulfasalazine, olsalazine depends on an azo-bond to prevent рrоxymal absorption of the mesalamine and to keep іt in the intestinal lumen until the azo- bond hydrolyzed and active mesalamine геleased by the enzymatic action of bacterial flora in the lower ileum and colon. Bacterial cleavage of the compound releases twice quantity of mesalamine without any sulfonamide. Another mesalamine-based azo compound, balsalazide, has also proven effective and has been approved in many countries.
Other forms of mesalamine have various delayed-release coatings. Asacol is monomelic mesalamine coated with an acrylic polymer whose pH solubility delays release of the drug until entry into the distal ileum and colon. Claversal and Salofalk (not available in the
Moderately severe disease in ambulatory patients usually requires systemic corticosteroid therapy.
Relatively intensive therapy with oral prednisone 40 to 60 mg/day in either single or divided doses frequently iduces dramatic remission. After 1 to 2 wk, the daily dose may be gradually reduced by about 5 to 10 mg/wk. Sulfasalazine (2 to 4 g/ day in divided doses) may be added when prednisone 20 mg/day is controlling the colitis; very gradual tapering and ultimate withdrawal of the corticosteroid may then be possible.
Severe disease, manifested by > 10 bloody bowel movements per day, tachycardia, high fever, or severe abdominal pain, requires hospitalization. If the patient had been receiving corticosteroid treatment > 30 days at the time of admission, hydrocortisone 300 mg/day should be given by continuous I/V drip. In patients who have not received recent corticosteroids, ACTH 75 to 120 U/day by continuous i/v drip has been reported to be a slightly more effective initial therapy, but adrenal hemorrhage is occasionally a complication. In either event, treatment is given for 7 to 10 days while the response is monitored by recording the nature and frequency of bowel movements. An initial abdominal x-ray should be obtained to assess the extent and severity of colonic involvement, and the patient must be observed closely for the development of toxic megacolon.
Unless dehydration resulting from diarrheal losses is imminent, use of hydrocortisone or ACTH in I/V 0.9% sodium chloride solution is not usually advised because edema is a frequent complication. Potassium chloride 20 to 40 mEq/L added to the I/V fluids usually helps prevent hypokalemia. Anemia may require transfusions. Parenteral hyperalimentation is sometimes used for nutritional support but is of no value as primary therapy; in fact, patients who can tolerate food will do better if they eat.
Oral prednisone 60 mg/day may be substituted after remission has been achieved with the 7- to 10-day course of parenteral treatment. A patient who remains well on the oral regimen for 3 to 4 days may leave the hospital, and the corticosteroid dosage may be gradually reduced at home under close medical supervision.
Immunomodulatory drugs.
Immunomodulatory drugs are acceptable for some patients with refractory or corticosteroid-dependent ulcerative colitis. Azathioprine and 6-mercaptopurine inhibit T-cell function, and a decline in the activity of both natural killer cells and cytotoxic T cells is correlated with a clinical response. The full benefit of azathioprine 2 to 3.5 mg/ kg/day or 6-mercaptopurine 1.5 to 2.5 mg/ kg /day may not be seen for 3 to 6 monthes because these drugs are slow-acting. Complications include pancreatitis (an absolute contraindication to continued use) and reversible neutropenia, which simply requires a lower dose with regular monitoring of WBC counts.
Cyclosporine has a rapid onset and is primarily indicated for acute severe ulcerative colitis unresponsive to high-dose IV corticosteroids. Continuous W infusion of cyclosporine can induce remission and avert surgery in about 80% of such cases. An additional 6 mo of treatment with oral cyclosporine, ultimately shifting to azathioprine or 6-mercaptopurine, may sustain longer-term remissions in 50 to 60% of cases. Because serious and even fatal complications (eg, renal toxicity, seizures, opportunistic infections) may occur, patients generally are not offered cyclosporine unless the safer curative option of colectomy is infeasible or inappropriate. Candidates for cyclosporine therapy should be referred to centers experienced in its use.
Toxic colitis is a grave emergency. As soon as signs of toxic colitis or impending toxic megacolon are detected, the following should be taken immediately: (1) discontinue all antidiarrheal drugs; (2) give nothing by mouth and pass a long intestinal tube attached to intermittent suction; (3) give aggressive I/V fluid and electrolyte therapy, with 0.9% sodium chloride, potassium chloride, albumin, and blood as needed; (4) give ACTH (adrenocorticotropic hormone) 120 U/day or hydrocortisone 300 mg/day by continuous I/V drip; and (5) give antibiotics (eg, ampicillin 2g I/V every 4 to 6 hours cefazolin
Having the patient roll over in bed from the supine to prone position every 2 to 3 hours may help redistribute colonic gas and prevent progressive distention. Passage of a soft rectal tube may also be helpful, but it must be done with extreme caution to avoid bowel perforation.
The patient must be watched closely for signs of progressive peritonitis or perforation. Percussion over the liver is important because loss of hepatic dullness may be the first clinical sign of free perforation, especially when peritoneal signs are suppressed by massive corticosteroid dosage. Abdominal x-rays should be obtained every 1 or 2 days to follow the course of colonic distention and to detect free or intramural air. If intensive medical measures do not produce definite improvement within 24 to 48 h, immediate surgery is required or the patient may die of perforation and attendant sepsis.
Emergency colectomy is indicated for massive hemorrhage, fulminating toxic colitis, or perforation. Subtotal colectomy with ileostomy and rectosigmoid closure is usally the procedure of choice because most critically ill patients cannot tolerate total proctocolectomy with abdominoperineal resection. The rectosigmoid stump may be electively removed later or may be used for ileoanal anastomosis with an intrapelvic itestinal reservoir, or pouch, with or without a rectal mucosectomy. In any event, the intact rectal stump should not be allowed to remain indefinitely because of the risk of disease activation and malignant degeneration.
Elective surgery is indicated for mucosa dysplasia or clinically suspected cancer, for all symptomatic strictures, for growth retardation in children, or most commonly, for intractable chronic disease resulting in invalidism or corticosteroid dependence Rarely, severe colitis-related extraintestinal manifestations (eg, pyoderma gangrenosum) may also be indications for surgery. Total proctocolectomy permanently cures ulcerative colitis. Permanent ileostomy has been the traditional price of this cure, although various alternatives (eg, the continent ileostomy, pelvic reservoirs or pouches with ileoanal anastomosis) are now available. The physical and emotional burdens imposed by any form of colon resection must be recognized, and care should be taken to: see that the patient receives all the instructions and psychologic support that are necessary before and after surgery.
