Pediatrics

Intussusception Deception: An Atypical Presentation

THE CASE

A previously healthy 10 year-old male presents with one day of RLQ pain and vomiting.  He awoke earlier that morning with mild to moderate pain, ate oatmeal for breakfast, and then vomited twice. About one hour later, he was sitting at his desk at school when he suddenly developed more severe abdominal pain. He initially presented to his pediatrician’s office, and was subsequently referred to Hasbro Children’s Hospital Emergency Department. No known sick contacts and no recent travel outside Rhode Island. No prior surgeries. He denies fever, chills, respiratory symptoms, melena or hematochezia, diarrhea, or urinary symptoms.

On exam, BP 115/71, HR 80, Temp 98.5F, RR 20, SpO2 99%. He is ill-appearing and acutely distressed. He has RLQ tenderness to palpation and involuntary guarding. He has normal testicular lie without tenderness, edema or erythema.  

DIAGNOSTIC STUDIES

Lab studies notable for WBC 7.9, blood glucose 114.

Abdominal/appendiceal ultrasound was ordered and showed an enteroenteric intussusception in the RLQ with adjacent inflammation and free fluid concerning for possible focal perforation (Figure 1).

 Figure 1. “Crescent in a donut” sign. Transverse view of intestinal intussusception. The hyperechoic crescent is formed by mesentery that has been dragged into the intussusception.

Figure 1. “Crescent in a donut” sign. Transverse view of intestinal intussusception. The hyperechoic crescent is formed by mesentery that has been dragged into the intussusception.

DISCUSSION

Intussusception occurs when a part of the bowel invaginates into itself, causing venous and lymphatic congestion. Untreated, intussusception may lead to ischemia and perforation.

Classic Presentation

Intussusception most commonly occurs in infants and toddlers ages 6 to 36 months-old, and approximately 80 percent of cases occur in children younger than 2 years-old [1]. Classically, parents report 15-20 minute episodes, during which their child seems acutely distressed, characterized by vomiting, inconsolable crying, and curling the legs close to the abdomen in apparent pain. They may also describe a “normal period” between episodes or offer a history that includes grossly bloody stools.

75 percent of cases of intussusception in young children have no clear trigger. Some evidence suggests that viral illness plays a role, particularly enteric adenovirus, which is thought to stimulate GI tract lymphatic tissue, in turn causing Peyer’s patches in the terminal ileum to hypertrophy and act as lead points for intussusception [2].

Atypical Presentation

Approximately 10 percent of intussusceptions occur in children older than 5 years [3]. Unlike their younger counterparts, these patients tend to present atypically, with pathologic lead points that triggered the event [4]. The patient described above illustrates this well. At 10 years-old, he presented with peritonitis after his intussusception caused focal perforation, and had no prior history of colicky abdominal pain or bloody stools. Ultimately, he was found to have Meckel’s diverticulum. This is the most common lead point among children, but other causes include polyps, small bowel lymphoma, and vascular malformations [5].

 Figure 2. Elongated soft tissue mass. Case courtesy of A.Prof Frank Gaillard,  radiopaedia.org

Figure 2. Elongated soft tissue mass. Case courtesy of A.Prof Frank Gaillard, radiopaedia.org

Diagnostic Testing

Plain abdominal radiographs are not sufficient to rule out intussusception, but they can be useful to exclude perforation and ensure that non-operative reduction by enema is safe.  Some signs of intussusception on abdominal x-ray include an elongated soft tissue mass (classically in the right upper quadrant as in Figure 2) and/or an absence of gas is the distal collapsed bowel, consistent with bowel obstruction.

The optimal diagnostic test for intussusception depends on the patient’s presentation. When infants or toddlers present classically with intermittent severe abdominal pain and no signs of peritonitis, air or contrast enema is the study of choice because it is both diagnostic and therapeutic (Figure 3).

 Figure 3. Intussusception treat with air enema. Case courtesy of Dr Andrew Dixon,  radiopaedia.org

Figure 3. Intussusception treat with air enema. Case courtesy of Dr Andrew Dixon, radiopaedia.org

When the diagnosis is unclear, however, abdominal ultrasound is preferred. Ultrasound has been shown to be 97.9% sensitive and 97.8% specific for diagnosing ileocolic intussusception, and is increasingly becoming the initial diagnostic study of choice at some institutions [6,7]. In addition to the ultrasound finding of “crescent in a donut” shown above, other sonographic signs of intussusception include the “target sign” (Figure 4) and the “pseudokidney sign” (Figure 5).

 Figure 4. Target Sign. Transverse view of the intestinal intussusception. The hyperechoic rings are formed by the mucosa and muscularis, and the hypoechoic bands are formed by the submucosa. Case courtesy of A.Prof Frank Gaillard,  radiopaedia.org

Figure 4. Target Sign. Transverse view of the intestinal intussusception. The hyperechoic rings are formed by the mucosa and muscularis, and the hypoechoic bands are formed by the submucosa. Case courtesy of A.Prof Frank Gaillard, radiopaedia.org

 Figure 5. Pseudokidney sign. Longitudinal view of intestinal intussusception. This view of the intussuscepted bowel mimics a kidney. Case courtesy of A.Prof Frank Gaillard,  radiopaedia.org

Figure 5. Pseudokidney sign. Longitudinal view of intestinal intussusception. This view of the intussuscepted bowel mimics a kidney. Case courtesy of A.Prof Frank Gaillard, radiopaedia.org

Treatment

Without clinical or radiographic signs of perforation, non-operative reduction is first-line treatment. Operative intervention is indicated when the patient is acutely ill, has a lead point needing resection, or the intussusception is in a location unlikely to respond to non-surgical management. For example, small bowel intussusceptions are less likely than ileocolic intussusceptions to respond to non-operative techniques [8].  


CASE CONCLUSION

The patient was taken emergently to the OR, where he underwent exploratory laparoscopy with laparoscopic appendectomy and resection of a Meckel’s diverticulum. No intussusception was noted intraoperatively.  He recovered well, and was discharged home two days later.


A BIT MORE ABOUT MECKEL’S DIVERTICULUM

Meckel’s diverticulum is the most common congenital anomaly of the GI tract. It is a true diverticulum (meaning it contains all layers of the abdominal wall) that is a persistent remnant of the omphalomesenteric duct, which connects the midgut to the yolk sac of the fetus. The “rule of twos” is the classic mnemonic to recall some other important features: it occurs in approximately 2% of the population; the male-to-female ratio is 2:1; it most often occurs within 2 feet the ileocecal valve; it is approximately 2 inches in size; and 2-4% of patients will develop complications related to Meckel’s diverticulum (such as intussusception), usually before age 2 [9].


TAKEAWAY POINTS

  • Consider intussusception in older patients. While it is less likely, approximately 10% of cases occur in patients over 5 years old.

  • In older patients, suspect pathological lead points, such as Meckel’s diverticulum, as potential etiologies of intussusception.

  • Obtain an abdominal x-ray before performing diagnostic/therapeutic enema to rule out perforation.

  • Ultrasound is the preferred test when the diagnosis is uncertain.

  • Patients with small bowel intussusceptions or known lead points are less likely to respond to non-operative reduction.

  • Patients who are acutely ill-appearing require surgery as first-line treatment.


Faculty Reviewer: Dr. Jane Preotle


SOURCES

  1. Intussusception: clinical presentations and imaging characteristics.. Retrieved June 22, 2018, from https://www.ncbi.nlm.nih.gov/pubmed/22929138

  2. Adenovirus infection and childhood intussusception. - NCBI. Retrieved June 22, 2018, from https://www.ncbi.nlm.nih.gov/pubmed/1415074

  3. Surgical approach to intussusception in older children: influence of .... Retrieved June 22, 2018, from https://www.ncbi.nlm.nih.gov/pubmed/25840080

  4. The clinical implications of non-idiopathic intussusception. - NCBI. Retrieved June 22, 2018, from https://www.ncbi.nlm.nih.gov/pubmed/9880737

  5. The leadpoint in intussusception. - NCBI. Retrieved June 22, 2018, from https://www.ncbi.nlm.nih.gov/pubmed/2359000

  6. Pediatric Emergency Medicine-Performed Point-of-Care Ultrasound. Retrieved June 22, 2018, from http://www.annemergmed.com/article/S0196-0644(17)31265-9/fulltext

  7. Comparative Effectiveness of Imaging Modalities for the Diagnosis .... Retrieved June 22, 2018, from https://www.ncbi.nlm.nih.gov/pubmed/28268146

  8. Small bowel intussusception in symptomatic pediatric patients - NCBI. Retrieved June 22, 2018, from https://www.ncbi.nlm.nih.gov/pubmed/11910476

  9. Sagar, Jayesh, Vikas Kumar, and D. K. Shah. "Meckel's diverticulum: a systematic review." Journal of the Royal Society of Medicine 99, no. 10 (2006): 501-505.








An Interview with Dr. Steven Selbst

Welcome to the Brown University EM Podcast and our Visiting Professor Series, featuring resident interviews with guest lecturers on their careers, their expertise, and their advice for emergency medicine residents.

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In this episode of the Brown Emergency Medicine Podcast Series we speak with Dr. Steven Selbst, a pediatric emergency medicine physician in Wilmington, DE. Dr. Selbst discusses his path in medicine – having first completed a residency in pediatrics and then becoming one of the first pediatric emergency medicine physicians.

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Dr. Steven Selbst, MD

Pediatric Emergency Medicine 

Nemours/Alfred I duPont Hospital for Children

Dr. Selbst offers tips that he has learned along the way and words of wisdom regarding medical-legal issues. A topic which he has significant experience speaking and writing about. Thank you to Dr. Selbst for your visit and taking the time to share your experience and your career journey.

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Tough on Stains... and on Bodies

The Case

 Figure 1: :Laundry Detergent Pod  By  Soulbust  - Own work, CC BY-SA 4.0,

Figure 1: :Laundry Detergent Pod

By Soulbust - Own work, CC BY-SA 4.0,

A previously healthy 12-month-old male was brought to the Emergency Department by his parents 20 minutes after ingesting a laundry detergent pod. The patient’s mother reported finding the child with pieces of the lining of an ALL Mighty Pacs detergent pod in his mouth. She removed the pieces and noted the packet was empty of liquid. At that point, the child started gagging and vomiting “almost immediately.” En route to the ED the patient had 2-3 more episodes of clear emesis. On arrival, he continued to have non-bloody, non-bilious emesis and dry heaves. Vitals were within normal limits with oxygen saturations in the mid 90s. On exam, the child was noted to have a hoarse voice and was mildly somnolent but easily arousable. He was drooling and crying in pain with swallowing, but his oropharynx was otherwise clear. Stridor was noted as well as suprasternal, substernal and supraclavicular retractions. The child was given Zofran, a 20cc/kg fluid bolus and decadron. ENT was consulted for increasing stridor and upper airway symptoms. The patient underwent nasopharyngeal scope at beside and was found to have mild vocal cord edema. He was taken emergently to the OR for definitive airway and bronchoscope. GI was also consulted for endoscopy. 

Findings

In the OR the child was intubated and underwent formal bronchoscopy and endoscopy. Significant findings included:

  1. Watery edema of the supraglottic structures
  2. Mild mucosal changes in the proximal esophagus
  3. Somewhat nodular proximal esophagus with patchy edema and mild sloughing of the mucosa (Fig 1. a, b, c)
  4. Mild patchy sloughing and nodularity distally
  5. One small erosion in the stomach
  6. Normal duodenum
  7. Congenital laryngomalacia and elliptical cricoid consistent with congenital subglottic stenosis

Detergent Pods

Laundry detergent “pods” or “packets” are small, often colorful, dissolvable packs containing concentrated laundry detergent. These laundry capsules have been in Europe since 2001, but were introduced to United States markets in 2010. [1] Laundry pods have been identified as a threat to pediatric patients who are often attracted to the candy-like appearance of the pods. The most common route of toxicity is via ingestion in patients younger than 5 years of age.[2] Recently, however, teenagers have become a significant percentage of the patient population via the “Tide Pod Challenge,” a viral, social-media campaign that dares teens to eat the pods. Detergent pods are often packaged in soft linings that consist of a water-soluble polyvinyl alcohol membrane that easily dissolves when exposed to saliva or moist skin.[3] The liquid mixture inside is usually composed of an anionic and a nonionic detergent as well as a cationic surfactant. All contain irritants and some brands also contain alkaline substances.[4] The alkaline nature of detergent pods can cause inflammation and mucosal destruction in the oropharynx, larynx and esophagus.[5]

Ingestion of detergent pods is associated with more severe symptoms than traditional laundry detergent.[6] One explanation for this is the concentrated nature of the detergent pack and the ingredients, which may include propylene glycol and ethoxylated alcohols.[7] Propylene glycol is found in great proportion in detergent packets than in typical detergent formulations.[8] It is not clear exactly how detergent pods cause injury, but there are several explanations.[9] When ingested, propylene glycol is metabolized by the liver to form lactate, acetate and pyruvate. The increased lactate results in a metabolic acidosis. The drug is excreted in the urine, but at higher doses of propylene glycol the renal tubules ability to secrete the drug is impaired. In children, propylene glycol remains in the blood longer than in adults, which results in more toxic effects, such as renal failure and CNS depression. Another important ingredient in laundry pods is ethoxylated alcohols, which can cause sedative effects. Lethargy is a unique feature of pod ingestion that is not seen with less concentrated detergent formulations.[10]

Ingredient Proposed Effect Clinical Manifestation
Alkalinity Inflammation and damage to oral, laryngeal and esophageal mucosa Hoarse Voice, Dysphagia, Drooling, Stridor, Respiratory Distress
Multiple Noxious response Nausea, vomiting, diarrhea
Propylene glycol Conversion to lactic acid and impaired renal clearance CNS Depression, Metabolic acidosis, Renal insufficiency
Phosphates Caustic Rash, Burns

Management

In the case of any suspected ingestion local poison control should be contacted for advice. Management efforts should initially focus on stabilizing airway, breathing and circulation. If eyes are involved, copious irrigation should begin as soon as possible, as delayed irrigation may be associated with increased morbidity, including burns.[11] Any contaminated clothing should be removed. Activated charcoal, whole bowel irrigation, or gastric lavage is not indicated in the treatment of alkaline ingestions such as detergents.[12] Charcoal and whole bowel irrigation has not been shown to have an effect. Gastric lavage is contraindicated due to risk of perforation and aspiration.[13]

The most important aspects of management are supportive care and symptom control. It is necessary to monitor for respiratory failure and depressed mental status, which may lead to the need for mechanical ventilation. Steroids have been used to mitigate airway edema, but studies have not confirmed their utility.[14] Zofran and other anti-emetics are useful for nausea and vomiting. Fluids should be administered for metabolic derangements or losses secondary to emesis. Endoscopy is important for injury staging and can help to risk stratify patients, however, many complications are delayed. Esophageal stricture is a rare, but possible, long-term sequela.[15]

Case Conclusion

The patient was admitted to the pediatric ICU for further care and management. On hospital day 1 frothy secretions were noted to be draining from his endotracheal tube. He was treated with Lasix for pulmonary edema and had improvement. Decadron was continued for a total of 4 doses of 0.5mg/kg. Feeds were given via NG tube. On hospital day 2 the child underwent repeat endoscopy to monitor for possible progression of mucosal damage. On hospital day 3 he was successfully extubated. Prior to discharge the patient was tolerating a regular pediatric diet with instructions to avoid acidic foods and juices. On hospital day 4 the child was discharged with ENT and GI follow-up. He was instructed to take omeprazole daily for 4-6 weeks

Faculty Reviewer: Dr. Jane Preotle

References

[1] Celentano A, Sesana F, Settimi L, Milanesi G, Assisi F, Bissoli M, Borghini R, Della Puppa T, Dimasi V. Accidental exposures to liquid detergent capsules. SKIN. 2012 May 25;5:0-9.

[2] Stromberg PE, Burt MH, Rose SR, Cumpston KL, Emswiler MP, Wills BK. Airway compromise in children exposed to single-use laundry detergent pods: a poison center observational case series. The American journal of emergency medicine. 2015 Mar 1;33(3):349-51.

[3] Bonney AG, Mazor S, Goldman RD. Laundry detergent capsules and pediatric poisoning. Canadian family physician. 2013 Dec 1;59(12):1295-6.

[4] Fraser L, Wynne D, Clement WA, Davidson M, Kubba H. Liquid detergent capsule ingestion in children: an increasing trend. Archives of disease in childhood. 2012 Aug 1:archdischild-2012.

[5] Zargar SA, Kochhar R, Nagi B, Mehta S, Mehta SK. Ingestion of strong corrosive alkalis: spectrum of injury to upper gastrointestinal tract and natural history. American Journal of Gastroenterology. 1992 Mar 1;87(3).

[6] Valdez AL, Casavant MJ, Spiller HA, Chounthirath T, Xiang H, Smith GA. Pediatric exposure to laundry detergent pods. Pediatrics. 2014 Nov 10:peds-2014.

[7] Beuhler MC, Gala PK, Wolfe HA, Meaney PA, Henretig FM. Laundry detergent “pod” ingestions: a case series and discussion of recent literature. Pediatric emergency care. 2013 Jun 1;29(6):743-7.

[8] Shah LW. Ingestion of Laundry Detergent Packets in Children. Critical care nurse. 2016 Aug 1;36(4):70-5.

[9] Huntington S, Heppner J, Vohra R, Mallios R, Geller RJ. Serious adverse effects from single-use detergent sacs: Report from a US statewide poison control system. Clinical toxicology. 2014 Mar 1;52(3):220-5.

[10] Shah LW. Ingestion of Laundry Detergent Packets in Children. Critical care nurse. 2016 Aug 1;36(4):70-5.

[11] Haring RS, Sheffield ID, Frattaroli S. Detergent Pod–Related Eye Injuries Among Preschool-Aged Children. JAMA ophthalmology. 2017 Mar 1;135(3):283-4.

[12] Riordan M, Rylance G, Berry K. Poisoning in children 4: household products, plants, and mushrooms. Archives of disease in childhood. 2002 Nov 1;87(5):403-6.

[13] McGregor T, Parkar M, Rao S. Evaluation and management of common childhood poisonings. American family physician. 2009 Mar 1;79(5).

[14] Anderson KD, Rouse TM, Randolph JG. A controlled trial of corticosteroids in children with corrosive injury of the esophagus. New England Journal of Medicine. 1990 Sep 6;323(10):637-40.

[15] Smith E, Liebelt E, Nogueira J. Laundry detergent pod ingestions: is there a need for endoscopy?. Journal of medical toxicology. 2014 Sep 1;10(3):286-91.