Intussusception is when the proximal portion of the intestine invaginates into the distal portion, causing an obstruction. Early in the process, lymphatic return is impeded, then as the edema and pressure increase, venous and arterial flow is compromised. Infarction of the collapsed bowel segment may occur. Intussusception usually occurs at age 3 months to 3 years, with a peak in infants <1 year old. In children <3 years old, it is generally idiopathic and possibly due to prominent lymphoid tissue in the intestine serving as a lead point. The majority are ileocolic, although may present anywhere along the lower gastrointestinal tract. In older patients, lead points include Meckel diverticula, polyp, tumor (lymphoma or hemangioma) or intramural edema and hematoma from Henoch-Schönlein purpura. Sites other than ileocolic are also associated with underlying pathology.
Patients typically present with sudden-onset, severe, colicky abdominal pain at regular intervals and vomiting (initially nonbilious; may progress to bilious), bloody stool (“current jelly”—with mucus and gross blood or occult blood), and a “sausage-like” abdominal mass. Patients often curl up to guard the abdomen. Typically patients present without some, not all of the above, symptoms and they may be perfectly well between episodes. Lethargy and irritability may be prominent. The physical exam may show a “Dance sign” or an “empty right lower quadrant.”
The differential diagnosis includes gastroenteritis, constipation, volvulus, toxin ingestion, or encephalopathy and any other condition that produces GI obstruction.
Obtain abdominal radiographs (supine, prone, and left lateral decubitus) for screening and to exclude free air. Obtain ultrasound for definitive diagnosis. Order complete blood count (CBC), serum electrolytes, blood type and screen, and consult surgery. Provide IV hydration and keep patients NPO (nothing by mouth). Attempt reduction of intussusceptions involving the colon with hydrostatic enema (air, barium, water). If reduction is not achieved after 3 attempts, consider repeated delayed reduction, or refer the child for operative reduction. Ileoileal intussusceptions either spontaneously reduce or require operative reduction. Free air on the radiograph or peritonitis is indication for immediate operative reduction. Whether or not to admit patients after successful reduction is controversial because of the potential for early recurrence.
Figure 10.1 ▪ Intussusception.
Extreme lethargy was the presenting complaint of this 10-month-old infant (A). While in the ED, he passed this stool mixed with blood and mucus (B). Other neurologic signs of intussusception include coma or shock-like state (out of proportion to abdominal signs), seizures, hypotonia, or opisthotonic posturing. (Photo contributor: Binita R. Shah, MD.)
Figure 10.2 ▪ Currant-Jelly Stool in Intussusception.
(A) Commercially available currant jelly. (B, C) Diarrhea containing mucus and blood constitutes the classic currant-jelly stool (seen in about 60% of cases). This Hemoccult-positive stool was passed by an 8-month-old infant presenting with inconsolable crying episodes and bilious vomiting. (Photo contributor: Binita R. Shah, MD.)
Figure 10.3 ▪ Intussusception.
The apex of the intussusception may extend into the transverse, descending, or sigmoid colon—even to and through the anus mimicking rectal prolapse. This type of intussusception can be distinguished from rectal prolapse by the separation between the protruding intestine and the rectal wall, which does not exist in rectal prolapse. (Photo contributor: Binita R. Shah, MD.)
Figure 10.6 ▪ Intussusception.
(A) Fluoroscopic image taken in the prone position during air reduction procedure shows soft tissue mass, which represents intussusception. (B) Fluoroscopic image taken after successful reduction demonstrates air diffusely throughout colon and small bowel. (Photo contributor: John Amodio, MD.)
Figure 10.7 ▪ Intussusception in Henoch-Schönlein Purpura.
A magnified view of ileoileal colic intussusception after bowel resection in a patient with Henoch-Schönlein purpura in whom the intussusception could not be reduced by barium enema. The proximal portion of the ileum (intussusceptum) telescopes into the more distal ileum and cecum (intussuscipiens). This image also illustrates necrosis of both the intussusceptum and intussuscipiens. (Reproduced with permission from Shah BR, laude TL: Atlas of Pediatric Clinical Diagnosis. WB Saunders, Philadelphia, 2000, p. 342.)
Intussusception is often misdiagnosed as gastroenteritis.
Profound lethargy may be the only symptom of intussusception and patients often get misdiagnosed as septic or in a postictal state, delaying diagnosis.
Perform a rectal exam for occult blood in all suspected cases of intussusception (50%–75% positive).
Evaluate for a pathologic lead point in patients > 3 years old.
The authors acknowledge the special contributions of Binita R. Shah, MD, to prior edition.
Pyloric stenosis is the idiopathic hypertrophy of the pylorus muscles, which creates a gastric outlet obstruction. Generally occurring in infants between the second week and second month of life (or later in preterms), it occurs more frequently in first-born boys, with a male-female ratio of about 4:1.
Figure 10.8 ▪ Hypertrophic Pyloric Stenosis.
(A) Longitudinal image of pyloric stenosis. There is thickening of the pyloric wall, greater than 3 mm and elongation of the length, greater than 1.5 cm. In addition, there is hypertrophy of the mucosa, with prolapse into the antrum producing the “nipple sign” (arrow). (B) Transverse image through the pylorus producing the “doughnut sign.” (Photo contributor: John Amodio, MD.)
Infants present as otherwise well, but with increasing nonbilious vomiting (often described as projectile) soon after feeding. The patient is hungry right after vomiting. The history often includes “constipation” or a decrease in the number of stools. After several days of vomiting, dehydration and weight loss may occur. Vigorous vomiting may cause a Mallory-Weiss tear that presents with coffee-ground emesis.
Physical exam may reveal an “olive” (a firm, ballotable mass with size and shape of an olive) in the mid-epigastrum as well as a visible peristaltic wave. With delayed presentation, cachexia may be present. The differential diagnosis includes gastroesophageal reflux, overfeeding, gastroenteritis, inborn errors of metabolism, adrenal insufficiency and other high-grade obstructions.
Order serum electrolytes, which commonly reveals hypochloremic hypokalemic metabolic alkalosis. Other findings include acidic urine pH (paradoxical aciduria in the face of metabolic alkalosis) and mild hyperbilirubinemia. Order ultrasound to confirm diagnosis, and consider an upper gastrointestinal (GI) study if the ultrasound is inconclusive. Correct dehydration and electrolyte abnormalities, which must be done prior to pyloromyotomy. Place a nasogastric tube for continued vomiting. Consult surgery and admit patient for intravenous (IV) hydration and pyloromyotomy. Most patients will tolerate feeds and be discharged home within 48 hours of surgery.
Pyloric stenosis is the most common cause of hypochloremic metabolic alkalosis in infancy.
The best time to examine for an “olive” is soon after the infant has vomited.
Pyloric stenosis is not a surgical emergency but may be a medical emergency because of electrolyte disturbances and dehydration. Most patients will require rehydration for 24 to 48 hours prior to surgery.
Acute nonperforated appendicitis is most commonly seen in school-aged children and teens. It is extremely difficult to diagnose in these preschoolers, and is often perforated at presentation in these young children. Patients classically present with periumbilical pain migrating to the right lower quadrant, fever, anorexia, vomiting, localized peritonitis, and/or guarding. Localization of pain and accompanying complaints vary with the length and position of the appendix, which is highly variable. For example, a retrocecal appendix is likely to cause rectal irritation and diarrhea, whereas an appendix abutting the bladder may cause dysuria, and one that abuts the liver may cause pain in the right upper quadrant.
Various “signs” are used to describe the classic findings. These are also variable depending on the position of the appendix, including McBurney point tenderness: tenderness one-third the distance from the right anterior superior iliac spine to the umbilicus; Rovsing sign: pain felt in the right lower quadrant with palpation of the left lower quadrant; Psoas sign: pain with extension of the right hip due to an inflamed appendix adjacent to the psoas muscle; and Obturator sign: pain with passive internal rotation of the flexed right hip. Differential diagnoses include lower lobe pneumonia, genitourinary pathologies (eg, testicular torsion, pyelonephritis), ovarian pathologies (eg, ovarian torsion, mittelschmerz), ectopic pregnancy, pelvic inflammatory disease, and other surgical pathologies (eg, intussusception, midgut volvulus).
Appendicitis is a clinical diagnosis that does not require routine imaging. If diagnosis is uncertain, order ultrasound or computed tomographic (CT) scan, noting that neither is 100% sensitive or specific. Obtain laboratory tests including CBC with differential, electrolytes including glucose, C-reactive protein, pregnancy test (adolescent females), and urinalysis—all of which may facilitate diagnosis, though no laboratory value alone is diagnostic. Provide IV hydration as needed and keep patients NPO. Provide adequate analgesia, including morphine as needed (does not mask symptoms). Consult surgery and consider admission for serial abdominal exams if diagnosis is unclear. Provide antibiotics (coverage for gram-negative and anaerobic bacteria) after the diagnosis is made and in consultation with surgery.
Figure 10.9 ▪ Acute Non Perforated Appendicitis.
(A) Longitudinal sonographic image of an enlarged (diameter greater than 6 mm), noncompressible appendix. Note increased echogenicity of the surrounding periappendiceal fat compatible with surrounding inflammation (arrow). (B) Same image as above with color flow imaging demonstrating increased flow compatible with hyperemia. (C) Longitudinal image of an enlarged appendix with an echogenic focus with acoustical shadowing, compatible with an appendicolith. (Photo contributor: John Amodio, MD.)
Rectal exam may be useful in localizing the pain of a retrocecal appendix.
Ability to hop up and down on the right leg without pain is often helpful to determine if there is peritonitis.
Missed appendicitis is one of the most common diagnoses resulting in malpractice claims.
Diarrhea is one of the most confusing symptom of appendicitis and patients often get misdiagnosed as gastroenteritis.
Perforation of appendicitis generally occurs after 48 to 72 hours of symptoms. Clinical symptoms differentiating perforated appendicitis or appendicitis with abscess from simple acute appendicitis include higher fever, systemic toxicity, diffuse abdominal tenderness and guarding (potentially a rigid abdomen), tender right lower quadrant mass and elevated markers of inflammation, including white blood cell count, absolute neutrophil count, and C-reactive protein.
Patients with perforated appendicitis do not like to move and feel most comfortable lying on their sides with the hips and knees flexed. They walk very slowly in a bent-over position with a shuffling gait while holding the right side of the abdomen and will refuse to hop or jump because of extreme pain. When initial perforation occurs, there may be some initial relief of pain, but until abscess is fully walled off, generalized peritoneal irritation is typically present.
Place all patients with suspected perforation on NPO status. Obtain CBC with differential, serum electrolytes including glucose, urinalysis, pregnancy test (as appropriate), and a C-reactive protein. Provide IV fluid resuscitation with crystalloids as clinically indicated. Provide adequate analgesia with morphine or other opiates. Consult surgery emergently and order CT, which may be foregone if the patient is taken to surgery before CT is obtained. CT gives detailed information about abscess formation and location, and may be used to plan drainage by interventional radiology (various protocols using rectal, oral, and IV contrast are used based on consultation with the radiologist). Once the diagnosis is confirmed, start broad-spectrum antibiotics with gram-negative and anaerobic coverage (eg, ampicillin, gentamicin, metronidazole).
Definitive management of perforated appendicitis varies from emergent surgery for septic patients to antibiotics to “cool off” the abscess followed by “interval appendectomy” several weeks later. These decisions are made by surgery.
Preschool-age children are often difficult to evaluate for appendicitis and often present with perforated appendicitis.
Adequate fluid resuscitation and stabilization prior to surgical intervention is imperative.
Patients with intestinal obstruction are restless, and move around the bed, while patients with peritonitis are quiet, without movement.
Incarceration is the inability to reduce hernia sac contents into the abdominal cavity. Hernias containing intestines generally occur in boys, especially with a history of prematurity, and are caused by failure of the process vaginalis to fuse after testicular descent. In girls, hernia most often includes the ovary. Patients most often present with a history of an inguinal or scrotal bulge that becomes more prominent with increased abdominal pressure (crying, straining, coughing, laughing). Other symptoms include inguinal swelling alone or in conjunction with pain and vomiting (may become bilious from intestinal obstruction or strangulation), irritability, poor feeding, lack of bowel movements, and abdominal distension. Incarceration is more common in patients <2 years old. Clinical characteristics include a firm, immobile, and often tender and erythematous swelling. When the hernia is in the scrotum, the bowel is palpable distinct of the testicle.
If diagnosis is unclear, order an ultrasound to differentiate hernia from hydrocele, testicular torsion or other inguinal or testicular pathology. Incarcerated hernias need to be reduced emergently. Provide analgesia and sedation as needed and apply gentle constant manual pressure to decompress the sac and push it in. If the hernia is not reducible or if there is any concern for intestinal perforation or gangrenous bowel, consult surgery emergently for operative management. If there is concern for perforation, order upright or cross-table lateral plain radiographs to assess for free air. Admit patient for observation and surgical repair (usually after 24 hours to allow for resolution of edema) or discharge for delayed repair (after surgical consultation) with very specific instructions to return for recurrence.
Figure 10.12 ▪ Incarcerated Inguinal Hernia.
This infant presented with inconsolable crying associated with vomiting. His hernia could not be reduced into the abdominal cavity mandating a surgical repair. Other entities that cause inconsolable crying in infants include testicular torsion, paraphimosis, fracture [child abuse] and hair-tourniquet syndrome. (Photo contributor: Binita R. Shah, MD.)
Figure 10.13 ▪ Incarcerated Inguinal Hernia.
(A) A sonographic image of the right scrotum showing a normal-appearing testicle (T) with normal color flow. Adjacent to the testicle is a fluid filled loop of bowel, compatible with a hernia (H). There is a small reactive hydrocele (h). (B) CT image of the same patient through the right scrotum showing incarcerated loop of bowel containing air and fluid. (Photo contributor: John Amodio, MD.)
Figure 10.14 ▪ Incarcerated Inguinal Hernia; Differential Diagnosis of Testicular Torsion.
Inguinal hernia can be seen as a distinct bulge separate from the testicle in this image. Both incarcerated inguinal hernia and testicular torsion present with acute onset of painful and swollen scrotum. (Photo contributor: Jennifer Chao, MD.)
Always consider incarcerated hernia in inconsolable infants.
Do not try to reduce the hernia if there is evidence of peritonitis or toxicity.
The hernia may cause pressure on the spermatic cord, causing venous congestion and infarction of the testicle.
Placing the child in Trendelenburg and maximizing relaxation will aid in reduction.
Figure 10.15 ▪ Hydrocele; Nonpainful Scrotal Swelling; Differential Diagnosis of Acute Scrotum.
(A) A swelling of the right hemiscrotum due to a hydrocele is seen in this infant. A hydrocele may be confused with hernia, cannot be reduced, lacks a mass at the inguinal ring, and occurs more often bilaterally. (B) Bilateral hydroceles seen in a sonographic image of scrotum in a different infant. (Photo contributors: Binita R. Shah, MD [A] and Mark Silverberg, MD [B].)
Testicular torsion is most common in the newborn and the early adolescent. In adolescents, it occurs when there is a “bell-clapper” deformity: testis is not fixed within the tunica vaginalis, allowing twisting of the testicle on the spermatic cord, which obstructs blood flow. Patients present with acute onset of severe pain in the groin (sometimes with radiation to the abdomen), vomiting and a swollen, tender testicle often in a transverse lie and higher than usual. There may also be absence of the cremasteric reflex, erythema of the overlying skin, and lack of relief with elevation of the testicle (a negative Prehn sign). There may be a history of similar episodes as the testes can twist and untwist spontaneously. With delayed diagnosis, the pain may subside and there is thickening of the scrotal skin. Torsion occurs more often on the left side, likely from the increased length of the spermatic cord.