Visual Diagnosis: An 11-Month-Old With Nausea, Vomiting, and an Abdominal Mass Laura A. Whittington, David C. Stevens, Sarah A. Jones and Julie M. Mayo Pediatrics in Review 2013;34;e47 DOI: 10.1542/pir.34-12-e47
The online version of this article, along with updated information and services, is located on the World Wide Web at: http://pedsinreview.aappublications.org/content/34/12/e47
Pediatrics in Review is the official journal of the American Academy of Pediatrics. A monthly publication, it has been published continuously since 1979. Pediatrics in Review is owned, published, and trademarked by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois, 60007. Copyright © 2013 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0191-9601.
Downloaded from http://pedsinreview.aappublications.org/ at Dahlgren Medical Library on October 29, 2014
visual diagnosis
An 11-Month-Old With Nausea, Vomiting, and an Abdominal Mass Laura A. Whittington, DO,* David C. Stevens, MD,* Sarah A. Jones, MD,* Julie M. Mayo, DO*†
Presentation
Figure 1. Abdominal bulge due to underlying mass.
Author Disclosure Drs Whittington, Stevens, Jones, and Mayo have disclosed no financial relationships relevant to this article. This commentary does not contain discussion of unapproved/ investigative use of a commercial product/device.
An 11-month-old girl presents to the emergency department (ED) with 12 episodes of nonbilious, nonbloody vomiting and decreased activity for the past 24 hours. Her medical history and family history are notable for the following. She was born at term via an uncomplicated, induced vaginal delivery. There is a paternal history of diabetes. The patient’s half-sister, who has phenylketonuria, was ill with nausea without vomiting 2 days before the patient’s presentation. Yesterday the patient was seen in the ED for nausea and 5 bouts of emesis. She was discharged after receiving one dose of ondansetron and could tolerate clear liquids by mouth. She now returns to the ED for continued vomiting. On physical examination the patient’s temperature is 37.9°C, heart rate is 114 beats per minute, respiratory rate is 30 breaths per minute, blood pressure is 105/65 mm Hg, and oxygen saturation is 98% in room air. She is comfortable and sleeping but awakens when physically examined. Mucous membranes are slightly dry, and her posterior oropharynx is erythematous. Cardiac auscultation reveals normal S1 and S2 heart sounds without a murmur. She has good peripheral perfusion and normal capillary refill. Her lungs are clear to auscultation. She has no abdominal scars, and bowel sounds are normal. Her abdomen is soft and nontender, and no masses are palpable. The spleen and liver are not enlarged. Abdominal radiography reveals a normal bowel gas pattern. The patient is admitted for intravenous hydration. Despite treatment with ondansetron, frequent vomiting persists. During her second hospital night, she has several large bowel movements that contain blood and mucus. Her father notices a visible mass in her abdomen (Fig 1). Laboratory evaluation reveals that her stool sample is negative for Shiga-like toxin and rotavirus. An abdominal radiograph is suggestive of underlying disease (Fig 2). On the basis of the radiographic findings, a water-soluble contrast enema is performed (Fig 3) that confirms the suspected underlying diagnosis.
*Department of Pediatrics, University of South Dakota Sanford School of Medicine, Sioux Falls, SD. † Pediatric Hospital Service/Sanford Children’s Hospital, Sioux Falls, SD.
Pediatrics in Review Vol.34 No.12 December 2013 e47
Downloaded from http://pedsinreview.aappublications.org/ at Dahlgren Medical Library on October 29, 2014
visual diagnosis
Figure 3. Water-soluble contrast enema reveals an intra-
intestinal mass.
Figure 2. Abdominal radiograph showing paucity of air in bowel and a curvilinear mass within the course of the colon.
Diagnosis Intussusception The patient’s history of bloody stools, a palpable abdominal mass, and the soft tissue opacification on abdominal radiography are highly suggestive of ileocecal intussusception. Subsequent contrast enema revealed a large intussusception that involves the ileum, cecum, and ascending and transverse colon.
Discussion Intussusception occurs when a portion of the proximal segment of the bowel (the intussusceptum) invaginates into a distal bowel segment (the intussuscipiens). Without treatment, bowel obstruction, necrosis, perforation, and death can ensue. Fortunately, most intussusceptions can be reduced by contrast enema. However, some patients require surgical intervention with manual reduction or excision of the affected portion of the bowel. Longer duration of intussusception is associated with increased morbidity and mortality. Thus, prompt recognition and treatment are imperative. Diagnosis is contingent on clinical suspicion and confirmation by abdominal ultrasonography or contrast enema. Intussusception is the most common cause of bowel obstruction in children between age 6 and 36 months. Infants
between age 4 and 7 months are most commonly affected. Intussusception rarely occurs in children younger than 2 months, and 70% of cases occur in the first year of life. Most cases are idiopathic, but some, particularly those occurring in children outside the normal age range, form around pathologic lead points, such as a Meckel diverticulum, intestinal polyp, or edema caused by Henoch-Schönlein purpura. Males develop intussusception at roughly twice the rate of females. The incidence is estimated to be 18 to 56 per 100,000 live births, and there is evidence in the United States and abroad that the incidence is decreasing overall. Infants and children with intussusception present with a variety of signs and symptoms. Emesis, abdominal pain, bloody stool, and lethargy are among the most common. Unfortunately, these symptoms are nonspecific and carry a vast differential diagnosis. The classic currant jelly stool is generally a late finding that occurs when the bowel wall undergoes significant vascular injury and blood mixes with mucoid material to produce this characteristic appearance. Either gross or occult blood in the stool is present in more than half of children with confirmed intussusception. The classic triad of abdominal pain, vomiting, and bloody stool is observed in less than 50% of affected individuals and should not be relied on for diagnosis. However, this triad is highly predictive of intussusception. To date, no reliable individual discriminating factors have been identified, and the diagnosis of intussusception continues to rely on a high degree of clinical suspicion and confirmatory imaging studies.
e48 Pediatrics in Review Vol.34 No.12 December 2013
Downloaded from http://pedsinreview.aappublications.org/ at Dahlgren Medical Library on October 29, 2014
visual diagnosis
Abdominal radiography, ultrasonography, and contrast enema are the 3 most common imaging modalities used in the evaluation of a child with possible intussusception. Abdominal radiography is occasionally indicative of intussusception, but the sensitivity of radiography is too low (29%-50%) to safely exclude the diagnosis. When intussusception is suspected, it must be definitely ruled out with either ultrasonography or contrast enema. The sensitivity and specificity of both studies approach 100%. However, ultrasonography is the preferred initial test because it is not invasive and does not expose the patient to ionizing radiation. If intussusception is found on ultrasonography, stable patients should proceed to contrast enema under ultrasonic or fluoroscopic guidance for initial therapy. Unstable patients and those with evidence of bowel rupture should have emergent surgical consultation.
Differential Diagnosis The differential diagnosis depends on the presenting symptoms. Gastrointestinal bleeding and abdominal pain in children can be caused by Henoch-Schönlein purpura, hemolytic uremic syndrome, and allergic colitis. Vomiting and abdominal pain have an equally broad differential diagnosis, including infectious gastroenteritis and malrotation with volvulus. From a surgical standpoint, the presence of hematochezia, a palpable abdominal mass, and a curvilinear mass within the course of the colon with a paucity of air in the bowel on abdominal radiography suggests segmental bowel obstruction from either volvulus or a persistent omphalomesenteric duct besides an intussusception.
Management Contrast enema continues to be the diagnostic gold standard and first-line therapy for an intussusception. A total of 46% to 80% of intussusceptions can be reduced with enema. Traditionally, a liquid enema was performed under fluoroscopic guidance; however, the current trend is toward air enemas under ultrasonic guidance, which do not use ionizing radiation. The efficacy of the 2 treatments is similar, and the choice of technique primarily depends on the experience of the attending radiologist. Before any planned contrast enema, intravenous access should be obtained, the child should be fully fluid resuscitated, and a pediatric surgeon should be notified. Many physicians will also administer a dose of intravenous antibiotics. When contrast enema reduces intussusception, the child should be closely monitored because 10% of patients will experience intussusception again. Primary surgical treatment is indicated for unstable patients and those with signs of bowel perforation. Surgical treatment is also necessary when contrast enema fails to reduce the intussusception.
Figure 4. Necrotic ileum within the intussuscepted colon.
Most pediatric surgeons approach uncomplicated, irreducible intussusception with laparoscopic reduction. If necessary, manual reduction of the intussusception is often successful; however, bowel excision may also be necessary. A delay in definitive diagnosis may lead to greater bowel ischemia and greater likelihood of resection.
Patient Course Unfortunately, the patient’s intussusception could not be reduced by contrast enema. The patient was taken to the operating room for an exploratory laparotomy and manual reduction of the intussusception. During the exploratory laparotomy, the patient was found to have necrotic ileum within the intussuscipiens (Fig 4). A right hemicolectomy with primary anastomosis was performed. Two days later the patient had abdominal distention with free air seen on abdominal radiography. She returned to the operating room, where an anastomotic leak was repaired. After 3 weeks in the pediatric intensive care unit, she was discharged home in good condition.
Summary • Children with intussusception can present with a wide variety of symptoms, including vomiting, fever, lethargy, and abdominal pain. The classic triad of abdominal pain, hematochezia, and palpable abdominal mass is seen in a few patients. • Early diagnosis of intussusception depends on a high level of clinical suspicion in any child with nonspecific abdominal findings followed by appropriate radiographic or ultrasonographic evaluation and confirmation with a contrast enema. • Abdominal radiography, although an appropriate component of the initial workup for gastrointestinal
Pediatrics in Review Vol.34 No.12 December 2013 e49
Downloaded from http://pedsinreview.aappublications.org/ at Dahlgren Medical Library on October 29, 2014
visual diagnosis
symptoms, lacks the sensitivity to reliably exclude the presence of intussusception. • Because ultrasonography is a safe, sensitive, and specific test for the diagnosis of intussusceptions, it should be performed early whenever there is clinical suspicion of intussusception. • Contrast enema is the gold standard for diagnosis and first-line treatment of intussusception. There is an increasing trend for pneumatic reduction of intussusception compared with hydrostatic reduction. Intravenous placement, fluid resuscitation, and notification of the pediatric surgeon should be completed before contrast enema.
Suggested Reading Fischer TK, Bihrmann K, Perch M, et al. Intussusception in early childhood: a cohort study of 1.7 million children. Pediatrics. 2004;114(3):782–785 Kaiser AD, Applegate KE, Ladd AP. Current success in the treatment of intussusception in children. Surgery. 2007;142 (4):469–475. Mandeville K, Chien M, Willyerd FA, Mandell G, Hostetler MA, Bulloch B. Intussusception: clinical presentations and imaging characteristics. Pediatr Emerg Care. 2012;28(9):842– 844 Samad L, Marven S, El Bashir H, et al. Prospective surveillance study of the management of intussusception in UK and Irish infants. Br J Surg. 2012;99(3):411–415
e50 Pediatrics in Review Vol.34 No.12 December 2013
Downloaded from http://pedsinreview.aappublications.org/ at Dahlgren Medical Library on October 29, 2014
Visual Diagnosis: An 11-Month-Old With Nausea, Vomiting, and an Abdominal Mass Laura A. Whittington, David C. Stevens, Sarah A. Jones and Julie M. Mayo Pediatrics in Review 2013;34;e47 DOI: 10.1542/pir.34-12-e47
Updated Information & Services
including high resolution figures, can be found at: http://pedsinreview.aappublications.org/content/34/12/e47
References
This article cites 4 articles, 1 of which you can access for free at: http://pedsinreview.aappublications.org/content/34/12/e47#BIBL
Subspecialty Collections
This article, along with others on similar topics, appears in the following collection(s): Gastroenterology http://pedsinreview.aappublications.org/cgi/collection/gastroenterolo gy_sub Abdominal Pain http://pedsinreview.aappublications.org/cgi/collection/abdominal_pai n_sub Surgery http://pedsinreview.aappublications.org/cgi/collection/surgery_sub
Permissions & Licensing
Information about reproducing this article in parts (figures, tables) or in its entirety can be found online at: http://pedsinreview.aappublications.org/site/misc/Permissions.xhtml
Reprints
Information about ordering reprints can be found online: http://pedsinreview.aappublications.org/site/misc/reprints.xhtml
Downloaded from http://pedsinreview.aappublications.org/ at Dahlgren Medical Library on October 29, 2014
The online version of this article, along with updated information and services, is located on the World Wide Web at: http://pedsinreview.aappublications.org/content/34/12/e47
Pediatrics in Review is the official journal of the American Academy of Pediatrics. A monthly publication, it has been published continuously since 1979. Pediatrics in Review is owned, published, and trademarked by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois, 60007. Copyright © 2013 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0191-9601.
Downloaded from http://pedsinreview.aappublications.org/ at Dahlgren Medical Library on October 29, 2014
visual diagnosis
An 11-Month-Old With Nausea, Vomiting, and an Abdominal Mass Laura A. Whittington, DO,* David C. Stevens, MD,* Sarah A. Jones, MD,* Julie M. Mayo, DO*†
Presentation
Figure 1. Abdominal bulge due to underlying mass.
Author Disclosure Drs Whittington, Stevens, Jones, and Mayo have disclosed no financial relationships relevant to this article. This commentary does not contain discussion of unapproved/ investigative use of a commercial product/device.
An 11-month-old girl presents to the emergency department (ED) with 12 episodes of nonbilious, nonbloody vomiting and decreased activity for the past 24 hours. Her medical history and family history are notable for the following. She was born at term via an uncomplicated, induced vaginal delivery. There is a paternal history of diabetes. The patient’s half-sister, who has phenylketonuria, was ill with nausea without vomiting 2 days before the patient’s presentation. Yesterday the patient was seen in the ED for nausea and 5 bouts of emesis. She was discharged after receiving one dose of ondansetron and could tolerate clear liquids by mouth. She now returns to the ED for continued vomiting. On physical examination the patient’s temperature is 37.9°C, heart rate is 114 beats per minute, respiratory rate is 30 breaths per minute, blood pressure is 105/65 mm Hg, and oxygen saturation is 98% in room air. She is comfortable and sleeping but awakens when physically examined. Mucous membranes are slightly dry, and her posterior oropharynx is erythematous. Cardiac auscultation reveals normal S1 and S2 heart sounds without a murmur. She has good peripheral perfusion and normal capillary refill. Her lungs are clear to auscultation. She has no abdominal scars, and bowel sounds are normal. Her abdomen is soft and nontender, and no masses are palpable. The spleen and liver are not enlarged. Abdominal radiography reveals a normal bowel gas pattern. The patient is admitted for intravenous hydration. Despite treatment with ondansetron, frequent vomiting persists. During her second hospital night, she has several large bowel movements that contain blood and mucus. Her father notices a visible mass in her abdomen (Fig 1). Laboratory evaluation reveals that her stool sample is negative for Shiga-like toxin and rotavirus. An abdominal radiograph is suggestive of underlying disease (Fig 2). On the basis of the radiographic findings, a water-soluble contrast enema is performed (Fig 3) that confirms the suspected underlying diagnosis.
*Department of Pediatrics, University of South Dakota Sanford School of Medicine, Sioux Falls, SD. † Pediatric Hospital Service/Sanford Children’s Hospital, Sioux Falls, SD.
Pediatrics in Review Vol.34 No.12 December 2013 e47
Downloaded from http://pedsinreview.aappublications.org/ at Dahlgren Medical Library on October 29, 2014
visual diagnosis
Figure 3. Water-soluble contrast enema reveals an intra-
intestinal mass.
Figure 2. Abdominal radiograph showing paucity of air in bowel and a curvilinear mass within the course of the colon.
Diagnosis Intussusception The patient’s history of bloody stools, a palpable abdominal mass, and the soft tissue opacification on abdominal radiography are highly suggestive of ileocecal intussusception. Subsequent contrast enema revealed a large intussusception that involves the ileum, cecum, and ascending and transverse colon.
Discussion Intussusception occurs when a portion of the proximal segment of the bowel (the intussusceptum) invaginates into a distal bowel segment (the intussuscipiens). Without treatment, bowel obstruction, necrosis, perforation, and death can ensue. Fortunately, most intussusceptions can be reduced by contrast enema. However, some patients require surgical intervention with manual reduction or excision of the affected portion of the bowel. Longer duration of intussusception is associated with increased morbidity and mortality. Thus, prompt recognition and treatment are imperative. Diagnosis is contingent on clinical suspicion and confirmation by abdominal ultrasonography or contrast enema. Intussusception is the most common cause of bowel obstruction in children between age 6 and 36 months. Infants
between age 4 and 7 months are most commonly affected. Intussusception rarely occurs in children younger than 2 months, and 70% of cases occur in the first year of life. Most cases are idiopathic, but some, particularly those occurring in children outside the normal age range, form around pathologic lead points, such as a Meckel diverticulum, intestinal polyp, or edema caused by Henoch-Schönlein purpura. Males develop intussusception at roughly twice the rate of females. The incidence is estimated to be 18 to 56 per 100,000 live births, and there is evidence in the United States and abroad that the incidence is decreasing overall. Infants and children with intussusception present with a variety of signs and symptoms. Emesis, abdominal pain, bloody stool, and lethargy are among the most common. Unfortunately, these symptoms are nonspecific and carry a vast differential diagnosis. The classic currant jelly stool is generally a late finding that occurs when the bowel wall undergoes significant vascular injury and blood mixes with mucoid material to produce this characteristic appearance. Either gross or occult blood in the stool is present in more than half of children with confirmed intussusception. The classic triad of abdominal pain, vomiting, and bloody stool is observed in less than 50% of affected individuals and should not be relied on for diagnosis. However, this triad is highly predictive of intussusception. To date, no reliable individual discriminating factors have been identified, and the diagnosis of intussusception continues to rely on a high degree of clinical suspicion and confirmatory imaging studies.
e48 Pediatrics in Review Vol.34 No.12 December 2013
Downloaded from http://pedsinreview.aappublications.org/ at Dahlgren Medical Library on October 29, 2014
visual diagnosis
Abdominal radiography, ultrasonography, and contrast enema are the 3 most common imaging modalities used in the evaluation of a child with possible intussusception. Abdominal radiography is occasionally indicative of intussusception, but the sensitivity of radiography is too low (29%-50%) to safely exclude the diagnosis. When intussusception is suspected, it must be definitely ruled out with either ultrasonography or contrast enema. The sensitivity and specificity of both studies approach 100%. However, ultrasonography is the preferred initial test because it is not invasive and does not expose the patient to ionizing radiation. If intussusception is found on ultrasonography, stable patients should proceed to contrast enema under ultrasonic or fluoroscopic guidance for initial therapy. Unstable patients and those with evidence of bowel rupture should have emergent surgical consultation.
Differential Diagnosis The differential diagnosis depends on the presenting symptoms. Gastrointestinal bleeding and abdominal pain in children can be caused by Henoch-Schönlein purpura, hemolytic uremic syndrome, and allergic colitis. Vomiting and abdominal pain have an equally broad differential diagnosis, including infectious gastroenteritis and malrotation with volvulus. From a surgical standpoint, the presence of hematochezia, a palpable abdominal mass, and a curvilinear mass within the course of the colon with a paucity of air in the bowel on abdominal radiography suggests segmental bowel obstruction from either volvulus or a persistent omphalomesenteric duct besides an intussusception.
Management Contrast enema continues to be the diagnostic gold standard and first-line therapy for an intussusception. A total of 46% to 80% of intussusceptions can be reduced with enema. Traditionally, a liquid enema was performed under fluoroscopic guidance; however, the current trend is toward air enemas under ultrasonic guidance, which do not use ionizing radiation. The efficacy of the 2 treatments is similar, and the choice of technique primarily depends on the experience of the attending radiologist. Before any planned contrast enema, intravenous access should be obtained, the child should be fully fluid resuscitated, and a pediatric surgeon should be notified. Many physicians will also administer a dose of intravenous antibiotics. When contrast enema reduces intussusception, the child should be closely monitored because 10% of patients will experience intussusception again. Primary surgical treatment is indicated for unstable patients and those with signs of bowel perforation. Surgical treatment is also necessary when contrast enema fails to reduce the intussusception.
Figure 4. Necrotic ileum within the intussuscepted colon.
Most pediatric surgeons approach uncomplicated, irreducible intussusception with laparoscopic reduction. If necessary, manual reduction of the intussusception is often successful; however, bowel excision may also be necessary. A delay in definitive diagnosis may lead to greater bowel ischemia and greater likelihood of resection.
Patient Course Unfortunately, the patient’s intussusception could not be reduced by contrast enema. The patient was taken to the operating room for an exploratory laparotomy and manual reduction of the intussusception. During the exploratory laparotomy, the patient was found to have necrotic ileum within the intussuscipiens (Fig 4). A right hemicolectomy with primary anastomosis was performed. Two days later the patient had abdominal distention with free air seen on abdominal radiography. She returned to the operating room, where an anastomotic leak was repaired. After 3 weeks in the pediatric intensive care unit, she was discharged home in good condition.
Summary • Children with intussusception can present with a wide variety of symptoms, including vomiting, fever, lethargy, and abdominal pain. The classic triad of abdominal pain, hematochezia, and palpable abdominal mass is seen in a few patients. • Early diagnosis of intussusception depends on a high level of clinical suspicion in any child with nonspecific abdominal findings followed by appropriate radiographic or ultrasonographic evaluation and confirmation with a contrast enema. • Abdominal radiography, although an appropriate component of the initial workup for gastrointestinal
Pediatrics in Review Vol.34 No.12 December 2013 e49
Downloaded from http://pedsinreview.aappublications.org/ at Dahlgren Medical Library on October 29, 2014
visual diagnosis
symptoms, lacks the sensitivity to reliably exclude the presence of intussusception. • Because ultrasonography is a safe, sensitive, and specific test for the diagnosis of intussusceptions, it should be performed early whenever there is clinical suspicion of intussusception. • Contrast enema is the gold standard for diagnosis and first-line treatment of intussusception. There is an increasing trend for pneumatic reduction of intussusception compared with hydrostatic reduction. Intravenous placement, fluid resuscitation, and notification of the pediatric surgeon should be completed before contrast enema.
Suggested Reading Fischer TK, Bihrmann K, Perch M, et al. Intussusception in early childhood: a cohort study of 1.7 million children. Pediatrics. 2004;114(3):782–785 Kaiser AD, Applegate KE, Ladd AP. Current success in the treatment of intussusception in children. Surgery. 2007;142 (4):469–475. Mandeville K, Chien M, Willyerd FA, Mandell G, Hostetler MA, Bulloch B. Intussusception: clinical presentations and imaging characteristics. Pediatr Emerg Care. 2012;28(9):842– 844 Samad L, Marven S, El Bashir H, et al. Prospective surveillance study of the management of intussusception in UK and Irish infants. Br J Surg. 2012;99(3):411–415
e50 Pediatrics in Review Vol.34 No.12 December 2013
Downloaded from http://pedsinreview.aappublications.org/ at Dahlgren Medical Library on October 29, 2014
Visual Diagnosis: An 11-Month-Old With Nausea, Vomiting, and an Abdominal Mass Laura A. Whittington, David C. Stevens, Sarah A. Jones and Julie M. Mayo Pediatrics in Review 2013;34;e47 DOI: 10.1542/pir.34-12-e47
Updated Information & Services
including high resolution figures, can be found at: http://pedsinreview.aappublications.org/content/34/12/e47
References
This article cites 4 articles, 1 of which you can access for free at: http://pedsinreview.aappublications.org/content/34/12/e47#BIBL
Subspecialty Collections
This article, along with others on similar topics, appears in the following collection(s): Gastroenterology http://pedsinreview.aappublications.org/cgi/collection/gastroenterolo gy_sub Abdominal Pain http://pedsinreview.aappublications.org/cgi/collection/abdominal_pai n_sub Surgery http://pedsinreview.aappublications.org/cgi/collection/surgery_sub
Permissions & Licensing
Information about reproducing this article in parts (figures, tables) or in its entirety can be found online at: http://pedsinreview.aappublications.org/site/misc/Permissions.xhtml
Reprints
Information about ordering reprints can be found online: http://pedsinreview.aappublications.org/site/misc/reprints.xhtml
Downloaded from http://pedsinreview.aappublications.org/ at Dahlgren Medical Library on October 29, 2014
