Abdominal pain is one of the most common complaints for which children present to the emergency department, making up perhaps 10% of such visits. In addition, pain is only one of the many signs and symptoms with which abdominal disorders may present. The problem the clinician confronts lies in differentiating those complaints that represent acute, serious illness from those that do not. It would be helpful, for example, if every case of appendicitis was just like every other. Unfortunately, particularly when dealing with young children, this is simply not the case. Appendicitis commonly presents in an obtuse manner and, even in this era of advanced diagnostic techniques, is still frequently missed, as are intussusception and testicular torsion. A systematic approach is essential to minimize misdiagnosis and morbidity. This article focuses on the surgical abdomen in children - its differential diagnosis, evaluation, and management - from the perspective of the primary provider or the emergency department physician who first encounters the patient.
Pediatric abdominal surgical emergencies may present in a variety of ways - abdominal pain, vomiting, gastrointestinal (GI) bleeding, diarrhea, distemion, abdominal mass, or with systemic complaints such as fever, lethargy, weight loss, or malaise. In addition, signs and symptoms are age-dependent. The young infant is not able to complain of pain and instead presents with crying or with such nonspecific signs as poor feeding, irritability, or lethargy. The toddler may be able to complain of pain but will not provide an accurate history or description of pain character and location. Progression of symptoms can be helpful in decision'tnaking, but may not be easily or accurately elicited from a young child or poorly observant parent. All of these factors frequently make the diagnosis of abdominal surgical emergencies in young children difficult.
The causes of the acute surgical abdomen vary with age (Table 1 ). In the neonate, the most common diagnoses include necrotizing enterocolitis and congenital anomalies such as malrotation with midgut volvulus, duplications, gastroschisis, and omphalocele. Pyloric stenosis typically presents between 2 and 6 weeks of age, while intussusception is most common after 2 months of age. Incarcerated inguinal hernias and intussusception account for the majority of abdominal surgical emergencies in the 2-month-old to 1-year-old age group; the former decreases in incidence after 1 year. Intussusception remains the most important abdominal surgical problem until around 2 years of age, when appendicitis becomes more common. After 2 years of age, appendicitis remains the most important cause of the acute surgical abdomen until young adulthood. With sexual maturity comes an additional set of considerations in the differential diagnosis of the surgical abdomen. Testicular torsion, most common in puberty, may present as abdominal pain. Consideration and exclusion of this entity early in the evaluation is critical. Gynecological disorders including pregnancy, ectopie pregnancy, ovarian cyst, and pelvic inflammatory disease must all be considered in the pubertal girl.
In all age groups, abdominal trauma - unintentional and that related to abuse - must always be considered as a possible etiology for abdominal complaints. Severe gastroenteritis can mimic an acute surgical abdomen and present with ileus and dehydration. In addition to abdominal causes for abdominal complaints, a number of systemic, nonsurgical illnesses may mimic an acute abdomen. These include streptococcal pharyngitis, diabetic ketoacidosis, sickle cell vaso-occlusive pain crisis of the abdomen, lower lobe pneumonia, certain poisonings and evenomations, and urinary tract infections.
APPROACH TO THE PATIENT WITH A SUSPECTED ACUTE SURGICAL ABDOMEN
The age of the patient and a knowledge of the most likely diagnoses in that age group are important factors influencing the direction an evaluation should follow. Regardless of age, however, a thorough history and complete physical examination are essential elements of the work-up. Often, the diagnosis can be made on the basis of the history alone, supported by physical findings, without extensive laboratory testing or imaging studies.
Careful thought should be given to which ancillary studies are likely to have a high yield and will actualIy be helpful in decision making. For example, the presence of a right lower quadrant fecalith on an abdominal radiograph is suggestive of appendicitis in the appropriate clinical setting. On the other hand, a plain film of the abdomen in a patient with a classic presentation for appendicitis adds little information that the history and physical examination has not already offered; the presence or absence of fecaliths in this setting will not influence management, and the radiograph is an unnecessary added expense.
Most Common Etiologies of Pediatrie Abdominal Surgical Emergencies by Age Group
When a surgical diagnosis is suspected, consultants should be involved early in the evaluation. For instance, the surgeon should be made aware of the patient with suspected intussusception prior to obtaining a barium enema in case there are complications - such as bowel perforation - from the imaging procedure itself that necessitate surgical intervention. Working together in a cooperative manner, the primary physician and surgeon should be able to move quickly through the evaluation of a child with a suspected surgical abdomen Figure 1).
Malrotation With Midgut Volvulus
Between the 5th and I2th weeks of gestation, fetal intestinal development normally involves extrusion of the elongating gut from the abdominal cavity, a counterclockwise rotation and subsequent broad retroperitoneal attachment of the intestine, and eventual reduction of the extra-abdominal intestine back into the abdominal cavity. Failure of normal rotation and attachment of the developing gut around the superior mesenterio artery results in a narrow mesenteric base through which the artery passes. Twisting around this narrow base (midgut volvulus) or constriction of the duodenum by abnormal peritoneal bands may occur, resulting in intestinal obstruction, vascular compromise, and eventual infarction of the involved gut segment. Although duodenal obstruction in the newborn is the most common presentation of malrotation, some patients have a less prominent duodenal component to the condition and are asymptomatic into later infancy or childhood, remaining undiagnosed until recurring attacks of midgut volvulus become manifest as recurrent abdominal pain.1 Partial or incomplete malrotation may result in intermittent symptoms as well (Figure 2). Recurrent midgut volvulus has the potential for becoming lethal rapidly and should be considered early in the evaluation of recurrent abdominal pain, particularly if there is evidence of bilious vomiting.
Initial evaluation and treatment of malrotation with midgut volvulus include supportive therapy (establishing intravenous access, volume resuscitation, and gastric decompression) and radiographie evaluation. Plain abdominal radiographs may suggest malrotation by the presence of colonie gas in the right upper abdomen or distension of the stomach and duodenum with absence of distal intestinal gas. Contrasted upper gastrointestinal films often show the malrotated duodenum coiled to the right of the midline in a "corkscrew" appearance (Figure 2). Failure of the duodenum to cross to the midline constitutes the radiographie criteria for making the diagnosis of malrotation. It must be kept in miind that volvulus may occur without complete obstruction. Immediate surgical intervention should follow radiographie diagnosis. Occasionally, a newborn may present with obstruction and signs of intestinal ischemia already present, in which case surgery should not be delayed for radiographie imaging.2
Infants with pyloric stenosis usually present between 2 and 6 weeks of life with a history of gradually increasing, forceful (projectile) vomiting and failure to thrive. These infants are generally alert and eager to take the breast or bottle, latching on quickly and sucking vigorously, only to vomit within a few minutes of feeding. While these babies are feeding, it is often possible to visualize a peristaltic wave cross the epigastrium as the stomach attempts to empty through the narrowed pylorus. Additionally, the experienced practitioner, with a light touch, often can palpate the thickened pyloric muscle (the "olive") in the right upper abdomen when the infant's stomach is empty.
Although the diagnosis of pyloric stenosis generally can be made by clinical evaluation, ultrasound is commonly used as a noninvasive, simple, and accurate method of confirming the diagnosis prior to surgery. On ultrasound, the pylorus is located between the antrum of the stomach and the liver and is recognized as a ring with low echogenic density in the high echogenic density of surrounding hypertrophie muscle and mucosa (Figure 3). A pyloric muscle width of 2.5 to 3 mm generally is accepted as normal. However, in a child with symptoms consistent with pyloric stenosis, a pyloric muscle width less than 2.53 mm does not exclude pyloric stenosis and a contrast study should be performed if there is still concern about the diagnosis.3
The initial evaluation and management of a child with suspected pyloric stenosis consists of a thorough history and physical examination as well as supportive care. Intravascular access should be established and blood obtained for electrolytes. These infants may present with a metabolic alkalosis with low serum potassium and chloride. Volume resuscitation and correction of metabolic abnormalities should occur prior to surgical repair.
Intussusception is the most common abdominal surgical emergency in children up to 2 years of age, after which age the incidence decreases. In some series, however, up to 37% of intussusceptions occurred in children older than 2 years.4
Intussusception is typically (>80%) ileocolic - the terminal portion of the ileum prolapses through the ileocecal valve into the colon. The prolapsed portion, the tntussusceptum, then travels through the colon, now termed the intussusceptiens, a variable distance. On occasion, the intussusceptum may progress as far as the rectum where it can be palpated on digital exam. Ileoileal, colocolic, jejunojejunal, and other types of intussusception occur but are rare. In 90% of children younger than 2 years, there is no obvious lead point for the intussusception other than hyperplastic lymphoid tissue.5 Adenovirus and rotavirus infections have been implicated as preceding illnesses. In children older than 2 years, a pathological lead point may be found in as many as one third of patients with intussusception (lymphoma, polyp, and Meckel's diverticulum). Children with Henoch-Schonlein purpura have an increased risk of developing intussusception as well, as these lesions may be lead points.
Intussusception results in compression of the invaginated bowel and bowel obstruction. Early in the course, the typical symptom is crampy abdominal pain that may be manifest by episodic crying, irritability, and pulling the legs up to the abdomen. In between episodes, the child may appear perfectly well. Alternatively, the only finding may be that of unexplained lethargy or prostration in which the child appears pale and motionless. On examination, a sausage-shaped mass may be palpated (most often) in the right upper quadrant, and there may be blood on the examining finger after rectal examination even if there is no history of bloody stool. The classic description of vomiting, intermittent crying, and bloody stools is fully present in only 10% of cases. Eventually, if undetected, the intussusception will progress to bowel obstruction with vomiting, abdominal distention, and shock. It must be remembered that the "currant jelly" stool so often referred to is a late finding that indicates bowel ischemia, which results from compression and strangulation of the mesenteric vessels supplying the intussusceptum. The goal should be to detect the intussusception and correct it before progression to that point.
Figure 2. Plain and contrast films showing malrotation with intermittent volvulus. Figure 2A. Grossly abnormal plain film of the abdomen with distended bowel loops. Figure 2B. Follow-up plain film taken approximately 12 hours later is normal. Figure 2C. A UGI shows incomplete malrotation with jejunal loops on the right side. Figure 2D. A barium enema showing cecum in the right upper quadrant.
if intussusception is suspected, the child should receive nothing by mouth, and a nasogastric tube should be passed if there is evidence of obstruction. If the child appears volume depleted, intravenous fluid resuscitation should be started. Plain radiographs often provide some diagnostic clues; the most reliable of these is a crescent of gas that caps the intussusceptum and outlines its leading edge (Figure 4), but this is present only in a minority of patients.6 The soft-tissue mass of the intussusception may be observed in 50% to 60% of cases as a relatively gasless area. If concentric lines can be identified within this gasless area to the right of the spine (the so-called target sign), this becomes much more specific for intussusception.6
Figure 3. Uftrasound diagnosis of pyloric stenosis. Figure 3A. Longitudinal view of the elongated and narrowed gastric outlet. Figure 3B. Transverse view of the same narrowed lumen with thickened musculature.
Imaging and nonsurgical reduction techniques for intussusception are a popular topic in pediatrie radìology literature, and a number of different methods and combinations of modalities have been proposed. The barium enema is still frequently used, but pneumatic reduction and ultrasound -guided hydrostatic reduction are preferred in some centers. Pneumatic reduction has been shown in some studies to have a greater efficacy in reduction, lower radiation exposure (shorter fluoroscopic times), and increased safety when compared to barium. Ultrasound recently has emerged as a reliable (sensitivity 100% and negative predictive value 100%), noninvasive screening tool for intussusception.7 Those patients with positive ultrasound examinations then undergo a reduction procedure using barium, air, or ultrasound-guided hydrostatics.8 An added benefit of ultrasound is its ability to detect underlying abnormalities (Meckel's, lymphomas, polyps, etc.)5 The rate of recurrent intussusception after nonsurgical reduction is around 10%. 5
The risk of perforation must be weighed prior to reduction attempts. If the child has had a prolonged course, appears toxic, or has signs of shock, barium should not be used. Ultrasound can safely and rapidly confirm the diagnosis prior to emergency surgery. Alternatively, a water-soluble contrast medium such as gastrograffin can be used to visualize the lesion with no attempt at reduction (Figure 5).
A recurrence rate of 1% to 4% has been reported after surgical reduction.5 Nonsurgical reduction can be attempted in these cases, but a second surgery may be required. Older children have a higher recurrence rate because of the higher incidence of pathological lead points, and probably should undergo surgical exploration on the first occurrence.
Incarcerated Inguinal Hernia
Repair of inguinal hernias is the most common surgical procedure performed in younger children. Fortunately, most of these repairs are done as elective procedures in an otherwise well child. However, incarceration with subsequent obstruction may occur, necessitating emergency intervention.
Pediatrie inguinal hernias occur as a result of incomplete or abnormal obliteration of the processus vaginalis. The processus is a diverticulum of the peritoneal cavity that accompanies the testicle as it moves into the scrotum. Normal fusion of the layers of the processus occurs after the testicle is in place, but the timing of this closure varies widely among individuals. Autopsy reports show that the processus is patent in 80% to 90% of newboms. Most closures apparently occur by age 2 years, but up to 30% of adults may have a patent processus vaginalis. There must be a patent processus for symptoms to develop, but not all patencies result in the formation of a hydrocele or hernia.
Inguinal hernias occur in approximately 10 to 20 per 1000 live births. Prematurity and low birthweight increase the risk. Although most inguinal hernias are unilateral, bilateral hernias are present in 50% of lowbirthweight or premature infants. The right side is involved more often than the left because of the later descent of the right testis. There is a 4:1 male-tofemale predominance, but girls have a higher rate of incarceration (in which the ovary may be involved). Other congenital abnormalities that may be associated with inguinal hernias include cryptorchidism, hypospadias, and ambiguous genitalia. Increased intra-abdominal pressure from a variety of causes (ventriculoperitoneal shunts, peritoneal dialysis, and ascites) may result in the development of clinical hernias in children with patent processus.9·10
Figure 4. Soft-tissue mass of intussusception on plain film "capped" with a crescent of bowel gas. Also note small bowel distortion and relatively gasless right colon.
Figure 5. Gastrograffin study showing intussusception with "coiled spring" appearance near the hepatic flexure. Attempts at reduction were unsuccessful, and the patient underwent surgery.
Incarceration with or without strangulation is the primary complication of inguinal hernia. Incarceration is defined as the inability to reduce the contents of the hernia back into the abdominal cavity. The goal of management is to repair hernias before such complications occur. Elective hernia repair has a 1% to 2% complication rate, but that rate is higher than 20% in patients with incarceration.10 A child with a previously reducible inguinal hernia who presents with irritability, poor feeding, or vomiting and an irreducible hernia represents a surgical emergency. In addition, incarceration may be the presenting finding in previously undiagnosed inguinal hernias (Figure 6).
As long as the child appears relatively well without evidence of toxicity or bowel ischemia, attempts should be made to reduce the hernia manually. Maneuvers that may help in this are calming a crying child (this may require sedation) or placing an older child in a Trendelenburg position for a period of time, allowing gravity to assist the reduction. Once the child is calm, one hand should be used to apply gentle pressure at the inguinal ring while attempting to squeeze the herniated contents back into the abdominal cavity with the other hand. If manual reduction is successful, elective surgery is scheduled within 48 hours.
When the child is extremely ill or toxic appearing, manual reduction should not be attempted, and stabilization for surgery should be accomplished immediately. Intravascular access should be established, a nasogastric tube placed, and volume resuscitation performed in preparation for surgery. There is some disagreement about whether all patients should undergo exploration of the contralateral groin at the time of surgery, but this is generally indicated in patients younger than 1 year and otherwise healthy, in all female patients, and in those with chronically increased intra-abdominal pressure.9
Even though it is the most common nontraumatic pediatrie surgical emergency, appendicitis continues to be a frequently missed diagnosis, primarily because there is no "typical" presentation in young children. The majority of cases present between 10 and 19 years of age (incidence of 23 cases per 10,000 per year),11 but it is not uncommon in younger children, and cases in early infancy have been reported. The classic history of progression from periumbilical pain (where did it hurt?) to pain localizing in the right lower quadrant (where does it hurt?) is less common in young children. Progression is also much more rapid in young children, who may present with evidence of perforation within 12 hours of the apparent onset; children under 8 are twice as likely to perforate prior to surgery than are older children.
Typical PaIn Localization Based on Anatomic Lie of Appendix In Acute Appendicitis
Initial symptoms usually include anorexia, which may progress to vomiting and fever. The absence of any of these findings, however, is not proof of the absence of appendicitis, and great care must be taken not to be misled. Fever as an initial symptom points away from appendicitis, in which fever generally develops at some time after the onset of other symptoms. Again, the absence of fever does not exclude appendicitis. At times, diarrhea is a prominent feature of appendicitis and is thought to result from an inflamed, low-lying appendix causing direct irritation of the sigmoid colon. The location of the appendix in children is variable and influences the location of pain (Table 2). In particular, a retrocecal acute appendicitis may have no anterior abdominal pain; the pain in these patients may be located entirely in the flank and suggest a ureteral calculus. Dysuria may be present as well and does not always signify urinary tract infection.
The evaluation of possible appendicitis includes a careful physical examination to exclude other causes of abdominal pain. If the general examination is normal, attention should then be focused on the abdomen and pelvis. A gentle approach to the child with abdominal pain cannot be overemphasized. Specifically, demonstration of direct rebound tenderness in children should be avoided. Simple observation provides a great deal of information. In general, the child with appendicitis walks slowly, splinting toward the painful area and lies quietly on the examination table, frequently with the right hip flexed.
Irritation of the psoas muscle from an inflamed appendix (psoas sign) may be elicited by active flexion or passive extension of the hip. The obturator sign (pain on passive internal rotation of the flexed thigh) may occur when the appendix lies on the obturator internus muscle. On rectal examination, a sudden involuntary wincing to pain may be elicited by palpating high on the right rectal wall. If there has been progression to appendiceal abscess, a boggy mass may be felt in the same location.11·12 Repeated rectal examinations are unwarranted in the young chiJd, and the primary/emergency department physician should have an understanding with the consulting surgeon about who will routinely do the procedure.
Ideally, the diagnosis of acute appendicitis can be made on the basis of history and physical examination, în the very young child especialty, circumstances are not always so clear. A number of diagnostic tests have been suggested as aiding the diagnosis of appendicitis.13·14 While these tests may be helpful, they do not replace a thorough history and meticulous physical examination. The white blood cell count may be elevated with an increase in neutrophils. If there is ureteral or bladder irritation from a nearby inflamed appendix, there may be a few white cells in the urine. A serum pregnancy test should be obtained in all pubertal girls. When obtained early in the course, Creactive protein may be helpful in sorting out those patients who do not have disease (highly specific),14 but this assay is not available quickly in most centers. The plain radiograph may show a number of findings, but more often than not is normal or at most "nonspecific." The presence of an appendicolith is helpful but occurs in fewer than 10% of cases (Figure 7). Barium enema, abdominal computed tomography, and ultrasound have all been suggested as methods of confirming the diagnosis of appendicitis. Ultrasound may be particularly helpful in differentiating gynecological conditions from acute appendicitis in the pubertal girl.
Laboratory studies, including blood urea nitrogen and electrolyte levels, should be obtained for patients thought to have appendicitis and an intravenous line started in preparation for surgery. If the patient has been vomiting or has had poor intake, a fluid bolus should be given. A surgeon should be consulted early in the course of the evaluation.
Thorek describes MeckeFs diverticulum as the disease of "2s". A remnant of the omphalomesenteric duct, Meckel's diverticulum occurs in 2% of the population with a 2:1 male-to-female ratio, may contain two types of tissue (gastric and pancreatic), is typically located within 2 feet of" the iíeocecaí junction, is 2 inches long, and becomes symptomatic in 2% of people in whom it occurs.15 Complications are primarily related to the presence of gastric mucosa, with hemorrhage and perforation being the most common.16 While massive, painless rectal bleeding is the typical initial symptom in Meckel's, other modes of presentation include Meckel's diverticulitis and intussusception in which the Meckel's is the lead point. Additionally, foreign bodies may become impacted within a Meckel's diverticulum, necessitating surgical removal.15
Figure 6. Plain radiograph showing bowel obstruction secondary to incarcerated inguinal hernia. There is dilatation of the proximal small bowel and an absence of colonie gas. Note soft-tissue mass in right groin and bowel gas in scrotum.
Figure 7. A string of fecaltths found at surgery to be obstructing the lumen of a long vermiform appendix.
Initial evaluation and management of Meckel's diverticulum includes supportive care; volume resuscitation is often necessary, and blood replacement may be required. Barium studies are generally ineffective in outlining the diverticulum, and die imaging modality of choice is nuclear scintigraphy. In diverticula that contain gastric mucosa and appear at about the same time as activity in the stomach, 99m-technetium pertechnetate will accumulate. Nuclear scans are accurate in detecting MeckePs approximately 95% of the time. However, false negatives may occur if the diverticulum is bleeding briskly or if there is only pancreatic tissue present.
Trauma is the leading cause of death in children older than 1 year, with approximately 22,000 deaths per year in the United States. Motor vehicle accidents account for most trauma deaths overall, but homicide is now the most common cause of death in urban adolescent males. Because of their smaller, more compact bodies, multisystem trauma is the rule in children. It follows that the abdomen, with its relatively large and poorly protected solid organs, is frequently a site of significant involvement.
Solid organ (liver and spleen) injury predomi' nates, and most abdominal injury in children results from blunt force impact, although penetrating injuries are becoming more common. Penetrating injuries in children are treated similarly to the same injuries in adults. Blunt abdominal trauma, however, is managed in a different manner. While there is always the exception that must be recognized, most solid organ injuries in children can be managed nonoperati vely.17 In the face of splenic trauma in which nonoperative management fails, because of the high mortality rate associated with postsplenectomy sepsis, every effort is made to repair rather than to remove the damaged spleen.
Incorrect use of seat belts in young children increases the risk of a specific pattern of injury, including abdominal wall bruising, intestinal bruising or perforation, and lumbar spine injury. This combination of injuries is seen primarily in young children who are placed in adult restraints.18
The possibility of abuse as a source of abdominal trauma should always be considered. Blows to the abdomen with a fist can produce lethal liver and spleen lacerations, pancreatic injuries, and hollow viscus rupture.
Management of abdominal trauma should follow the principles outlined in the Advanced Trauma Life Support program sponsored by the American College of Surgeons.17 Initial management should focus on the basics of life support - airway, breathing, and circulation (the primary survey). In the trauma victim, particularly if there is a concomitant head injury, special attention should be paid to protecting the cervical spine. After the primary survey, the physician should next perform a systematic secondary survey searching for injury to specific organ systems.
In addition to a carefully thorough physical examination, diagnostic techniques used to evaluate abdominal trauma include computed tomography, ultrasound, and diagnostic peritoneal lavage. While all of these have their proponents, they should be considered complementary studies, each with its own advantages and disadvantages.19'21
Pediatric abdominal surgical emergencies may present the primary physician with a diagnostic challenge. A systematic approach will help to minimize missed diagnoses and resultant complications. It always must be kept in mind that children often have atypical presentations of common entities. Prudent and directed use of laboratory and imaging studies will minimize misdiagnosis. The early involvement of surgical consultants in the care of pediatrie patients who have significant abdominal symptoms or findings is always appropriate.
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Most Common Etiologies of Pediatrie Abdominal Surgical Emergencies by Age Group
Typical PaIn Localization Based on Anatomic Lie of Appendix In Acute Appendicitis