Intestinal Obstruction and Malrotation

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109 Intestinal Obstruction and Malrotation

In its most simplistic conceptualization, the gastrointestinal (GI) tract is a long, flexible tube designed for active, unidirectional flow, with its only openings to the outside world being the mouth for input and the anus for output. Any impediment to the forward progress of ingested contents results in luminal distension proximal to the obstruction and eventual vomiting or perforation. A variety of congenital and acquired defects may cause throughput failure, with clinical presentations ranging from subtle findings that go undiagnosed for years to acute clinical deterioration. The clinician’s first responsibilities are to triage the severity of the obstruction and provide relief of the obstruction to prevent further complications, most notably intestinal perforation, peritonitis, sepsis, and death.

Etiology and Pathogenesis

The many etiologies that cause intestinal obstruction ultimately result in a final common pathophysiologic pathway (Figure 109-1). Anything that prevents forward progress of intestinal contents sets into motion this sequence of events, leading to worsening distension, vomiting, and systemic dysfunction. Not only do the resulting dehydration and electrolyte imbalances create significant complications, bacterial proliferation within the static intestinal contents and compromised mucosal integrity set the stage for bacterial translocation across the intestinal wall, with consequent bacteremia and sepsis.

Although many disorders may cause obstruction (Figure 109-2), they can be summarized by several pathophysiologic categories that aid in constructing a rational differential diagnosis.

Intrinsic Decrease in Lumen Caliber

Acquired Defects

Chronic bowel inflammation can, over time, lead to narrowing of the lumen. An important example is Crohn’s disease, in which there is discontinuous full-thickness bowel inflammation anywhere from the mouth to the anus (see Chapter 110). Over time, inflamed areas may stricture and obstruct. Another example of this is eosinophilic esophagitis, an entity that lies within the spectrum of food allergies and atopic disease. Because of persistent eosinophilic inflammation in the presence of the offending antigen, the esophagus can eventually stricture over time, leading to dysphagia and the need for esophageal dilatation to relieve the obstruction. Malignancy, although relatively uncommon in children compared with adults, is another important cause of decreased lumen caliber. For example, the GI tract is the most common extranodal site affected in non-Hodgkin’s lymphoma. Infiltration of the bowel wall by tumor leads to decreased effective luminal diameter and a higher risk of obstruction. Achalasia causes functional esophageal obstruction caused by aperistalsis and inadequate lower esophageal sphincter relaxation. An example of gastric outlet obstruction caused by decreased diameter is hypertrophic pyloric stenosis, a disease primarily affecting infants in the first few months of life in which smooth muscle hypertrophy leads to occlusion of the pylorus (Figure 109-3).

Clinical Presentation

Patients with obstruction may experience a wide range of symptoms depending on the nature and severity of the etiology. The physician’s primary responsibility at first contact with any patient is to triage the severity of illness and prevent the further progress of medical complications. Patients with obstruction may exhibit severe pain, abdominal distension (unless involvement is in the proximal GI tract), diaphoresis, stigmata of dehydration, and vomiting with inability to tolerate oral input. The patient may be tachycardic (both from pain and hypovolemia). The blood pressure can be difficult to interpret, with high values being most likely a response to pain; a normal blood pressure should not be taken as a necessarily reassuring sign without a measure of skepticism. Indeed, a normal value in the context of an ill-appearing, tachycardic patient may be indicative of compensated shock (see Chapter 2). Fever raises concerns for intestinal ischemia, perforation, and peritonitis.

There are many potential causes of GI obstruction (see Figure 109-2). However, because the clinical presentation of the patient is governed by the specific characteristics of the obstructive etiology, attention to the patient’s particular symptom complex can yield clues helpful to constructing a rational differential diagnosis. Several key questions help to organize the approach to the patient.

What Is the Character of the Vomitus?

Obstruction can occur anywhere from the esophagus to the anus, with the location being an important influence on the symptom pattern that the patient experiences. If the obstruction is in the esophagus, the patient may experience dysphagia (difficulty swallowing) or outright inability to intake orally. Low-grade esophageal obstruction can hinder the swallowing of solids, and high-grade obstruction can also interfere with the ingestion of liquids, which results in drooling and frequent spitting. Gastric outlet obstruction, as in pyloric stenosis, causes nonbilious vomiting because bile is secreted by the liver into the duodenum at the ampulla of Vater beyond the point of obstruction.

Small bowel obstruction leads to abdominal distension, cramping discomfort in the middle or upper abdomen, and repeated episodes of bilious vomiting. Symptoms often have a rapid onset after the obstructive process has begun. If there is total obstruction, patients eventually become obstipated (i.e., cease to pass stool). Fluid and electrolyte disturbances are relatively common. Abdominal pain raises concerns for bowel ischemia. Large bowel obstruction, on the other hand, tends to have symptoms that are milder and slower in onset compared with small bowel obstruction. Vomiting does not invariably occur, although the bowel does become distended. Patients may have lower abdominal cramping. On rectal examination, if the obstruction is high in the colon, the rectum will be devoid of stool, but hard stool in the rectum may be present if the patient has fecal impaction. Explosive stooling may be precipitated by rectal examination in a patient with Hirschsprung’s disease. Fluid and electrolyte disturbances do not tend to develop as precipitously as in small bowel obstruction.

Does the Child Have Other Historical or Physical Examination Abnormalities?

The presence of other abnormalities may raise suspicion for particular etiologies. Trisomy 21 is one of many congenital disorders that can be associated with intestinal atresia and other anomalies such as malrotation (see Chapter 117). Heterotaxy is associated with malrotation, which can lead to Ladd’s bands and volvulus. Patients who have recently had Henoch-Schönlein purpura, an acquired form of vasculitis, are at risk for intussusception, as are those with polyps and duplication cysts (see Chapter 28). Crohn’s disease is a common cause of intestinal stenosis (see Chapter 110).

Evaluation And Management

Initial Care in the Emergency Department

Patients reporting symptoms concerning for obstruction should be directed to emergency care. The goal of the initial evaluation in the emergency department is to determine the acuity and severity of the child’s illness. The clinician should rapidly obtain a general sense of the patient’s predicament through the general appearance, ascertaining clinical signs such as lethargy, diaphoresis, pallor, and restlessness. The first pass on physical examination should quickly acquire information regarding the patient’s critical features, beginning with the ABCs (airway, breathing, and circulation). Vital signs should be evaluated for fever (in the setting of obstruction, concerning for ischemia), tachycardia, hypotension (worrisome for decompensated shock), and low-normal blood pressure with widened pulse pressure (concerning for compensated shock). On the second pass, the clinician should perform a thorough but expedient head-to-toe examination that includes assessments of mental status, level of dehydration, signs of trauma, and stigmata of disease. The abdominal examination must assess for distension (and whether it is caused by gas or fluid), bowel sounds (early obstruction results in hyperactive sounds, but ischemic bowel will be silent), the presence of masses, and the presence and location of pain. If peritoneal signs are present (involuntary guarding, rebound tenderness to palpation, exquisite sensitivity to movement), there should be immediate concern for perforation. In addition, the assessment should include a rectal examination to check stool for the presence of blood via stool guaiac testing and to screen for low-lying pathologies such as distal intussusception, Hirschsprung’s disease, fecal impaction, and pelvic masses.

After the patient’s status has been determined, initial interventions should be initiated without delay while the workup continues. Patients with vomiting should receive nothing by mouth (NPO) and should have an intravenous (IV) line started for maintenance fluids. If there are signs of dehydration, isotonic fluid boluses are appropriate until the patient is hemodynamically stable. Patients with repeated emesis should also receive a nasogastric tube using a sump-type catheter (which is more rigid, larger in caliber, and less likely to collapse than a feeding tube), which can then be placed on low intermittent suction. If the sump aspirates large volumes, it is important to replace those losses via IV fluid administration. Indeed, without decompression, even when a patient is NPO, vomiting may continue because of the significant volume of fluid that the GI tract produces in the form of secretions. If the patient is ill appearing or has fever in the context of suspected obstruction, the physician should strongly consider initiating IV antibiotic therapy with adequate coverage for common gut flora (gram-negative and anaerobic organisms) after obtaining a blood culture. Early in this process, the team should contact a surgeon to evaluate the need for operative intervention. If patients are determined in the course of their workup to have partial small bowel obstruction, they may be admitted for observation and continued decompression before further evaluation and management. Those with complete obstruction or other complications, such as ischemia and perforation, require surgical management.

It is essential to keep in mind that many illnesses can initially present with a constellation of signs and symptoms resembling obstruction. This can make the assessment of the vomiting child challenging. On the more benign end of the spectrum, viral gastroenteritis is an example of a disorder that can cause repeated vomiting. What distinguishes this from obstruction is that there is typically a history of ill contact, the vomiting is nonbilious (or perhaps “highlighter” green after repeated vomiting episodes, as opposed to the “spinach green” color of truly bilious emesis in obstructed patients), diarrhea is present, and the patient’s abdomen is not distended. On the other end of the spectrum are several pitfalls that are important for the physician not to miss:

Intracranial lesions: Although malignancy is generally rare in children, it is critical not to miss brain tumors, which can present with recurrent emesis (see Chapter 57). Distinguishing characteristics may include a concurrent history of neurologic symptoms (e.g., headaches, vision changes, or loss of motor coordination) or changes in other psychological parameters (e.g., loss of milestones or changes in speech). The vomiting typically occurs without abdominal pain or even nausea, and other signs of obstruction such as distension should also be absent. The physical examination should include a funduscopic examination and neurologic assessment. If there is clinical concern, the team should obtain intracranial imaging (typically computed tomography [CT] in the emergency setting because of its short scan time, although magnetic resonance imaging may eventually be required because of its higher resolution and superiority in detecting posterior fossa lesions).

Using the clinical questions presented in the previous section, the clinician can begin to localize and characterize the nature of the symptoms to construct a differential diagnosis based on data that addresses those questions.

Radiologic Tests

All patients with signs consistent with obstruction should have initial flat and upright abdominal radiographs to assess the bowel gas pattern and to screen for other pathologies, such as intraabdominal free air, foreign bodies, volvulus, and masses. Air-fluid levels denote stasis of intraluminal contents, either from obstruction or ileus. Obstruction is generally associated with significant proximal intestinal distension, either with fluid or air, and the portions of bowel that are distended may help locate the obstruction. For example, an image that shows gas in the stomach but not in any distal regions suggests gastric outlet obstruction or, less likely, gastric volvulus. The “double-bubble” sign, in which air is seen both in the stomach and in the proximal duodenum but not distally, is suggestive of duodenal atresia.

Real-time fluoroscopic contrast studies greatly enhance the radiologic ability to discern anatomic features of the bowel and thus are of considerable value in determining the region of obstruction in stable patients. Furthermore, fluoroscopy provides dynamic information about the function of the bowel. Historically, the contrast agent has been barium, but in the setting of evaluating obstruction, it is more appropriate to use a water-soluble contrast agent. This is because barium that extravasates into the peritoneum through a perforation can create severe peritonitis, and stagnant barium that becomes trapped at the obstruction will solidify over time, potentially creating further complications. When a patient is medically unstable, fluoroscopic contrast studies are inadvisable given that they involve introduction of a foreign substance into the GI tract, and they do not provide enough information about extraintestinal pathology that may be contributing to the clinical picture.

If the individual has primarily esophageal symptoms, an esophagram can delineate the site and nature of defect, as well as evaluate esophageal function (see Chapter 107). In a stable patient with small bowel obstructive symptoms, the study of choice is the upper GI series, in which the contrast is followed to the ligament of Treitz. This study assesses for malrotation or volvulus in addition to a host of other anatomic defects. It is essential that any child younger than 1 year of age with bilious vomiting must be assessed for malrotation and volvulus. A variant on the upper GI series is the upper GI with small bowel follow-through, in which the radiologist assesses the bowel to the terminal ileum. This study is inappropriate for the acute setting given the length of time required to perform the study and the need to use barium, which is required because endogenous secretions dilute water-soluble contrast as it passes through the GI tract, making it ineffective for delineating the distal small bowel. However, this is a valuable tool when the suspicion is that a defect beyond the ligament of Treitz may be causing partial small bowel obstruction.

In limited settings, contrast enemas help to delineate causes of colonic obstruction. For example, the unprepped barium enema is an essential tool in the workup of Hirschsprung’s disease. In the rare case of colonic tumors (a far more common problem in adults), it is also helpful. It is unnecessary in the evaluation of fecal impaction, which can be diagnosed by clinical assessment. A special case is the air-contrast enema study, which is used in the setting of suspected intussusception. In this situation, air is the contrast agent used to diagnose the area involved. After the radiologist identifies the intussusception, this modality becomes useful as a therapeutic tool because the air then serves as a means to reduce the intussusception.

In patients who are medically unstable, who have a history of trauma, or who have a suspected perforation, the study of choice is the CT scan because of its rapid throughput and ability to provide the clinical team with large amounts of information about the intestinal anatomy as well as extraintestinal pathologies. The drawback to CT is its radiation burden, which is equal to many plain radiographs and is at least twice that of an upper GI series with small bowel follow-through. However, this consideration is outweighed by its benefits in the acute setting, and newer pediatric protocols have reduced the radiation load significantly.

In limited situations, ultrasound is a useful tool when specific etiologies are suspected. For example, it is widely used in the assessment of pyloric stenosis and intussusception. Because it does not create a radiation burden, it is also finding use at some centers in the form of small bowel evaluation of strictures that may cause partial small bowel obstruction, as can occur in Crohn’s disease.

Additional Evaluation

Although it is not indicated in the acute setting, GI endoscopy is a useful tool for diagnosing mucosal disorders that may not be obvious on radiologic imaging, and it offers the advantage of allowing therapeutic intervention in some cases. Endoscopy involves a gastroenterologist inserting an endoscope into either the mouth or anus, depending on the region of interest. The instrument allows direct visualization of the mucosa and provides the ability to sample the tissue for histologic diagnosis. For example, an older patient who has had a long-standing history of intermittent abdominal pain and poor weight gain and is found to have small bowel strictures causing partial small bowel obstruction could have Crohn’s disease, which requires mucosal biopsies for definitive diagnosis. In progressive esophageal strictures, eosinophilic esophagitis is an important consideration; this also requires histologic diagnosis from biopsies.

Surgical evaluation is critical at all stages of the assessment of children with obstruction. After enough clinical information is available to determine whether the child should go to the operating room, the surgical team will proceed with exploratory laparotomy or, at some centers, laparoscopy. Although the most invasive of all diagnostic modalities, it provides definitive diagnosis of the obstruction, and it is lifesaving and absolutely indicated in patients with complete obstruction and who have evidence of bowel ischemia or perforation.