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Painless intussusception and altered mental status
Painless Intussusception and Altered Mental Status ROBERT BIRKHAHN, MD, MELISSA FIORINI, MD, THEODORE J. GAETA, DO A 7-month-old child presented to the emergency department (ED) with 2 hours of painless, nonprojectile emesis and a normal mental status. Over a 3-hour period in the ED, the child remained pain-free, but developed hematemesis, hematochezia, and lethargy, progressing to unresponsiveness. The patient was evaluated for toxic ingestion, intracranial bleed, sepsis/meningitis, and intraabdominal pathology. The diagnosis was made by an abdominal ultrasound, which demonstrated an ileal-cecal intussusception that ultimately required surgical reduction. This case illustrates an insidious and poorly understood presentation of a common childhood affliction, as well as the utility of abdominal ultrasound in evaluating a hemodynamically stable patient with intussusception. (Am J Emerg Meal 1999;17:345-347. Copyright © 1999 by W.B. Saunders Company) Intussusception is a common pediatric condition that occurs when one segment of bowel telescopes into the lumen of an adjacent segment of bowel. The peak incidence is in the third to ninth month of life, with a male to female ratio of 2:1, and classically presents with the triad of colicky abdominal pain, vomiting, and current j e l l y stools. Although this "classic triad" is seen in only 30% to 65% of the cases, 86% to 94% of all cases have colicky abdominal pain on presentation. ~,2,3 Profound lethargy without a preceding history o f abdominal pain is an infrequent presentation, and can pose a diagnostic challenge to the emergency physician.
CASE REPORT A 7-month-old male infant was brought to the ED for vomiting. The mother stated that the child had 8 episodes of effortless, nonprojectile emesis over the last 2 hours. The vomitus was described as being 2 to 3 tablespoons of clear liquid without blood. The child was previously healthy, active, and playful. There was no history of fever or diarrhea in the last week, and the last meal consisted of formula and commercial baby food 2 hours prior to arrival. Inquiries to possible trauma revealed that the child had "bumped his head against crib while crawling" 5 hours prior to arrival. Past medical history was significant for full term vaginal delivery without antepartum or postpartum complications. The infant had never been hospitalized, had no surgical history, no known food or drug allergies, and took no medications. On physical examination the child was initially alert, interactive, and playful with the examiner. The patient's temperature was 99.30°F rectally, pulse 125, respiration 35, and blood pressure 100/80 mmHg. His neck was supple, anterior fontanel was open, From the Department of Emergency Medicine, New York Methodist Hospital, Brooklyn, NY. Manuscript received November 21, 1997, returned January 12, 1998; revision received February 12, 1998, accepted February 25, 1998. Address reprint requests to Theodore J. Gaeta, DO, New York Methodist Hospital, Brooklyn, NY 11215. Key Words: Intussusception, altered mental status. Copyright © 1999 by W.B. Saunders Company 0735-6757/99/1704-000651 O.00/0
soft and flat, and mucous membranes were moist with no oral/ pharyngeal lesions. Pupils were 4 ram, equal round and reactive to light. Lung fields were clear with good air movement. The cardiovascular exam was normal with a brisk capillary refill and symmetric pulses. His abdomen was rounded, nondistended, nontender, bowel sounds were active, and there were no palpable masses. His testicles were descended bilaterally and nontender. The rectal exam showed hemoccult positive stool. There were no visible fissures externally or with the anoscope. The skin and extremities were warm and dry without ecchymosis or rashes. The neurologic exam was initially normal, but the patient's mental status rapidly went from playful to somnolescent. The patient became lethargic and finally unresponsive with hypotonia over a course of 2½ hours. The laboratory studies were normal, including a white blood cell (WBC) count of 8,400 and normal serum electrolytes. Radiography of the chest and abdomen were without abnormalities. The patient was observed to have 6 more episodes of emesis over a 3-hour period in the ED, each time effortless and without signs of abdominal discomfort. The earlier episodes of emesis were gastrocult positive for blood, and the last episode consisting of 4 mL of bright red blood. The patient was given two fluid challenges of 20cc/kg each, which resulted in a blood pressure of 125/80, but unchanged mental status or physical exam. A toxicology screen was sent and the patient was scheduled for an immediate computed tomography (CT) of the cranium. Although the abdominal exam was benign, the triad of lethargy, vomiting and hemoccult positive stools was suggestive enough to warrant the use of an ED abdominal ultrasound as a diagnostic screening tool while awaiting the CT scan. The transabdominal ultrasound demonstrated a 2 × 3 cm midabdominal target-shaped mass (Figure 1) consistent with intussusception. Barium enema confirmed the diagnosis of intussusception, which was not reducible in the radiology suite. Manual reduction of the ileal-cecal intussusceptum was successful in the operating room, with no lead point identified. The patient spent three days in the intensive care unit (ICU) and was discharged to home on postoperative day number seven.
DISCUSSION The presentation of intussusception in the previously healthy child who develops intermittent abdominal pain and hematochezia has been well described, and few physicians would overlook the diagnosis when these symptoms are present in a child of 7 to 10 months of age. While this stereotyping of symptoms has led to faster diagnosis and treatment, the disease may be overlooked in both older children and in children without abdominal pain. Recent epidemiologic data suggests that intussusception is occurring more frequently in older children, with only 67% of the cases in one series occurring in patients below 2 years of age. 1 The same study by Luks et al found that 86% of all cases presented with colicky abdominal pain, 64% had vomiting, 39% had hematochezia, 26% had a palpable 345
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AMERICAN JOURNAL OF EMERGENCY MEDICINE • Volume 17, Number 4 • July 1999
FIGURE 1. (A) Longitudinal cut demonstrating "pseudokidney sign," composed of the same hypo/hyper echoic layers viewed on long axis. (B) Transverse cut through lesion demonstrating the classic "target sign" with a hypoechoic rim of edematous bowel wall, and a hyperechoic center of compressed mucosa. abdominal mass, and 19% had some degree of lethargy or somnolescence. The etiology of the intussusception remains unknown in 80% to 90% of all cases, although the postulated "lead point" is thought to be inflamed Peyer's patches secondary to viral infection. The gold standard for diagnosing and treating intussusception has been barium enema and, more recently, air contrast enema. Successful reduction rates have been suggested to be as high as 90% for air and 65% to 85% for barium or water soluble contrast agents. 4 Barium and air contrast enemas require technical skill and equipment not available in the emergency department, and each carry with them the risks of bowel perforation and radiation exposure. Plain radiographs of the abdomen are notoriously nonspecific in the diagnosis of intussusception, but have a role in the identification of free air in the abdomen. The presence of lethargy and vomiting in a previously well patient raises the specter of a closed head injury with an evolving mass lesion or an uncommon presentation of meningitis/sepsis in a child less than a year of age. But
profound lethargy and hypotonia can also be seen in cases of intussusception. The etiology of this alteration in mental status is uncertain. It is possible that the lethargy is similar to the well-categorized, but equally enigmatic, phenomenon of aseptic fever sometimes seen after the successful intussusception reduction. Lethargy is felt to be caused by the release of uncharacterized cytokines by the entrapped bowel wall. Other cases of lethargy or somnolescence can be attributed to the dehydration and shock that can occur rapidly in the pediatric population. The order in which to obtain diagnostic tests is guided not only by the pretest probability and consideration for lifethreatening disease, but also the expediency and availability of a given test. In a patient who is hemodynamically stable with altered mental status, the possibility of serious intracranial pathology must take first priority. Unforttmately, the procedure necessary, CT scans, requires that the patient be transferred out of the ED. Similarly, a clinician may be wary of sending a patient out of the department for a barium or air contrast enema when there is some doubt of the diagnosis of intussusception based on history and physical examination. In our case, the presence of ED ultrasonography helped establish a prompt diagnosis without the risks of leaving the department. Abdominal ultrasonography (see Figure 1) has been well documented as a sensitive and specific noninvasive test for the presence of intussusceptionS,6,7; however, the utility of ultrasound in cases of suspected intussusception remains controversial. The use of ultrasound is limited by the therapeutic implications of using barium and air contrast enemas for diagnosis. In spite of this fact, one recent study found that 67% of patients undergoing contrast enemas for possible intussusception did not have disease. 7 This same study found that the initial use of a screening ultrasound correlated well with the negative findings of the contrast enemas, and could have been used to reliably rule out the presence of intussusception. The increasing availability of ED sonography could enhance the utility of real-time ultrasound in accurately identifying patients with intussusception, thereby preventing the use of the more invasive barium and air contrast enemas in a number of patients suspected of having intussusception. Abdominal ultrasonography may have a role in older children who have a lower incidence of intussusception, as well as in patients less than a year of age in whom the diagnosis in not assured. The use of abdominal ultrasound has also been advocated as a screening tool for all patients with suspected intussusception, not only for training, but to reduce the use of the more invasive barium/air contrast enema. 8,9,1° Additionally, abdominal ultrasonography may replace fluoroscopy in guiding water and air contrast enema reduction in cases of intussusception. 6,11,12 In summary, intussusception is a common disease that can present with nonspecific symptoms, and may mimic a spectrum of serious illnesses. The findings of profound lethargy and hypotonia may lead the clinician towards a diagnosis of intracranial pathology, particularly if a good history of crampy and intermittent abdominal pain cannot be elicited. Fortunately, ED ultrasonography provides quick and accurate assessment of intraabdominal pathology that may be confounded by another process, or masked by vague presenting symptoms.
BIRKHAHN ET AL • PAINLESS INTUSSUSCEPTION
CONCLUSION Our case reminds us that intussusception can present with no abdominal findings, by history or on physical examination. One must maintain a high index of suspicion in order to direct appropriate diagnostic studies and therapeutic maneuvers for correction. The growing availability of ED ultrasonography, coupled with the changing prevalence of intussusception, leads us to advocate the use of screening ultrasound in all cases of suspected intussusception, for both expedient management, as well as a reduction in the number of negative barium enemas performed.
REFERENCES 1. Luks FI, Yazbeck S, Perreautt G, et al: Changes in the presentation of intussusception. Am J Emerg Med 1992;10:574-576 2. Wayne ER, Campbell JB, Burrington JD: Management of 344 children with intussusception. Pediatr Radiol 1973; 107:597-601 3. Losek JD: Intussusception: Don't miss the diagnosis! Pediatr Emerg Care 1993;9:46-51
347
4. Bisset GS, Kirks DR: Intussusception in infants and children: Diagnosis and treatment. Radiology 1998;168:141-145 5. StanleyA, Logan H, Bate TW, et al: Ultrasound in the diagnosis and exclusion of intussusception. Isr J Med Sci 1997;90:64-65 6. Lim HK, Bae SH, Lee HK, et al: Assessment of reducibility of ileocolic intussusception in children: Usefulness of color doppler sonography. Radiology 1994; 191:781-785 7. Bhistkul DM, Listernick R, Shkolnik A, et al: Clinical application of ultrasonography in the diagnosis of intussusception. J Pediatr 1992;121:182-186 8. Pracros JP, Tran-Minth VA, Morin De Finfe CH, et al: Acute intestinal intussusception in children: Contribution of ultrasonography. Ann Radiol (Paris) 1987;30:525-530 9. Swischuk LE, Hayden CK, Boulden T: Intussusception: Indications for ultrasonography and an explanation of the doughnut and pseudokidney signs. Pediatr Radiol 1985;15:388-391 10. Bowerman RA, Silver TM, Jaffe MH: Real-time ultrasound diagnosis of intussusception in children. Radiology 1982;143:527529 11. Peh WC, Khong PL, Chan KL, et al: Sonographic guided hydrostatic reduction of childhood intussusception using Hartmann's solution. Am Journ Roentgen 1996;167:1237-1241 12. Choi SO, Park WH, Woo SK: Ultrasound guided water enema: An alternative method of non-operative treatment for childhood intussusception. J Pediatr Surg 1994;29:498-500
CASE REPORT A 7-month-old male infant was brought to the ED for vomiting. The mother stated that the child had 8 episodes of effortless, nonprojectile emesis over the last 2 hours. The vomitus was described as being 2 to 3 tablespoons of clear liquid without blood. The child was previously healthy, active, and playful. There was no history of fever or diarrhea in the last week, and the last meal consisted of formula and commercial baby food 2 hours prior to arrival. Inquiries to possible trauma revealed that the child had "bumped his head against crib while crawling" 5 hours prior to arrival. Past medical history was significant for full term vaginal delivery without antepartum or postpartum complications. The infant had never been hospitalized, had no surgical history, no known food or drug allergies, and took no medications. On physical examination the child was initially alert, interactive, and playful with the examiner. The patient's temperature was 99.30°F rectally, pulse 125, respiration 35, and blood pressure 100/80 mmHg. His neck was supple, anterior fontanel was open, From the Department of Emergency Medicine, New York Methodist Hospital, Brooklyn, NY. Manuscript received November 21, 1997, returned January 12, 1998; revision received February 12, 1998, accepted February 25, 1998. Address reprint requests to Theodore J. Gaeta, DO, New York Methodist Hospital, Brooklyn, NY 11215. Key Words: Intussusception, altered mental status. Copyright © 1999 by W.B. Saunders Company 0735-6757/99/1704-000651 O.00/0
soft and flat, and mucous membranes were moist with no oral/ pharyngeal lesions. Pupils were 4 ram, equal round and reactive to light. Lung fields were clear with good air movement. The cardiovascular exam was normal with a brisk capillary refill and symmetric pulses. His abdomen was rounded, nondistended, nontender, bowel sounds were active, and there were no palpable masses. His testicles were descended bilaterally and nontender. The rectal exam showed hemoccult positive stool. There were no visible fissures externally or with the anoscope. The skin and extremities were warm and dry without ecchymosis or rashes. The neurologic exam was initially normal, but the patient's mental status rapidly went from playful to somnolescent. The patient became lethargic and finally unresponsive with hypotonia over a course of 2½ hours. The laboratory studies were normal, including a white blood cell (WBC) count of 8,400 and normal serum electrolytes. Radiography of the chest and abdomen were without abnormalities. The patient was observed to have 6 more episodes of emesis over a 3-hour period in the ED, each time effortless and without signs of abdominal discomfort. The earlier episodes of emesis were gastrocult positive for blood, and the last episode consisting of 4 mL of bright red blood. The patient was given two fluid challenges of 20cc/kg each, which resulted in a blood pressure of 125/80, but unchanged mental status or physical exam. A toxicology screen was sent and the patient was scheduled for an immediate computed tomography (CT) of the cranium. Although the abdominal exam was benign, the triad of lethargy, vomiting and hemoccult positive stools was suggestive enough to warrant the use of an ED abdominal ultrasound as a diagnostic screening tool while awaiting the CT scan. The transabdominal ultrasound demonstrated a 2 × 3 cm midabdominal target-shaped mass (Figure 1) consistent with intussusception. Barium enema confirmed the diagnosis of intussusception, which was not reducible in the radiology suite. Manual reduction of the ileal-cecal intussusceptum was successful in the operating room, with no lead point identified. The patient spent three days in the intensive care unit (ICU) and was discharged to home on postoperative day number seven.
DISCUSSION The presentation of intussusception in the previously healthy child who develops intermittent abdominal pain and hematochezia has been well described, and few physicians would overlook the diagnosis when these symptoms are present in a child of 7 to 10 months of age. While this stereotyping of symptoms has led to faster diagnosis and treatment, the disease may be overlooked in both older children and in children without abdominal pain. Recent epidemiologic data suggests that intussusception is occurring more frequently in older children, with only 67% of the cases in one series occurring in patients below 2 years of age. 1 The same study by Luks et al found that 86% of all cases presented with colicky abdominal pain, 64% had vomiting, 39% had hematochezia, 26% had a palpable 345
346
AMERICAN JOURNAL OF EMERGENCY MEDICINE • Volume 17, Number 4 • July 1999
FIGURE 1. (A) Longitudinal cut demonstrating "pseudokidney sign," composed of the same hypo/hyper echoic layers viewed on long axis. (B) Transverse cut through lesion demonstrating the classic "target sign" with a hypoechoic rim of edematous bowel wall, and a hyperechoic center of compressed mucosa. abdominal mass, and 19% had some degree of lethargy or somnolescence. The etiology of the intussusception remains unknown in 80% to 90% of all cases, although the postulated "lead point" is thought to be inflamed Peyer's patches secondary to viral infection. The gold standard for diagnosing and treating intussusception has been barium enema and, more recently, air contrast enema. Successful reduction rates have been suggested to be as high as 90% for air and 65% to 85% for barium or water soluble contrast agents. 4 Barium and air contrast enemas require technical skill and equipment not available in the emergency department, and each carry with them the risks of bowel perforation and radiation exposure. Plain radiographs of the abdomen are notoriously nonspecific in the diagnosis of intussusception, but have a role in the identification of free air in the abdomen. The presence of lethargy and vomiting in a previously well patient raises the specter of a closed head injury with an evolving mass lesion or an uncommon presentation of meningitis/sepsis in a child less than a year of age. But
profound lethargy and hypotonia can also be seen in cases of intussusception. The etiology of this alteration in mental status is uncertain. It is possible that the lethargy is similar to the well-categorized, but equally enigmatic, phenomenon of aseptic fever sometimes seen after the successful intussusception reduction. Lethargy is felt to be caused by the release of uncharacterized cytokines by the entrapped bowel wall. Other cases of lethargy or somnolescence can be attributed to the dehydration and shock that can occur rapidly in the pediatric population. The order in which to obtain diagnostic tests is guided not only by the pretest probability and consideration for lifethreatening disease, but also the expediency and availability of a given test. In a patient who is hemodynamically stable with altered mental status, the possibility of serious intracranial pathology must take first priority. Unforttmately, the procedure necessary, CT scans, requires that the patient be transferred out of the ED. Similarly, a clinician may be wary of sending a patient out of the department for a barium or air contrast enema when there is some doubt of the diagnosis of intussusception based on history and physical examination. In our case, the presence of ED ultrasonography helped establish a prompt diagnosis without the risks of leaving the department. Abdominal ultrasonography (see Figure 1) has been well documented as a sensitive and specific noninvasive test for the presence of intussusceptionS,6,7; however, the utility of ultrasound in cases of suspected intussusception remains controversial. The use of ultrasound is limited by the therapeutic implications of using barium and air contrast enemas for diagnosis. In spite of this fact, one recent study found that 67% of patients undergoing contrast enemas for possible intussusception did not have disease. 7 This same study found that the initial use of a screening ultrasound correlated well with the negative findings of the contrast enemas, and could have been used to reliably rule out the presence of intussusception. The increasing availability of ED sonography could enhance the utility of real-time ultrasound in accurately identifying patients with intussusception, thereby preventing the use of the more invasive barium and air contrast enemas in a number of patients suspected of having intussusception. Abdominal ultrasonography may have a role in older children who have a lower incidence of intussusception, as well as in patients less than a year of age in whom the diagnosis in not assured. The use of abdominal ultrasound has also been advocated as a screening tool for all patients with suspected intussusception, not only for training, but to reduce the use of the more invasive barium/air contrast enema. 8,9,1° Additionally, abdominal ultrasonography may replace fluoroscopy in guiding water and air contrast enema reduction in cases of intussusception. 6,11,12 In summary, intussusception is a common disease that can present with nonspecific symptoms, and may mimic a spectrum of serious illnesses. The findings of profound lethargy and hypotonia may lead the clinician towards a diagnosis of intracranial pathology, particularly if a good history of crampy and intermittent abdominal pain cannot be elicited. Fortunately, ED ultrasonography provides quick and accurate assessment of intraabdominal pathology that may be confounded by another process, or masked by vague presenting symptoms.
BIRKHAHN ET AL • PAINLESS INTUSSUSCEPTION
CONCLUSION Our case reminds us that intussusception can present with no abdominal findings, by history or on physical examination. One must maintain a high index of suspicion in order to direct appropriate diagnostic studies and therapeutic maneuvers for correction. The growing availability of ED ultrasonography, coupled with the changing prevalence of intussusception, leads us to advocate the use of screening ultrasound in all cases of suspected intussusception, for both expedient management, as well as a reduction in the number of negative barium enemas performed.
REFERENCES 1. Luks FI, Yazbeck S, Perreautt G, et al: Changes in the presentation of intussusception. Am J Emerg Med 1992;10:574-576 2. Wayne ER, Campbell JB, Burrington JD: Management of 344 children with intussusception. Pediatr Radiol 1973; 107:597-601 3. Losek JD: Intussusception: Don't miss the diagnosis! Pediatr Emerg Care 1993;9:46-51
347
4. Bisset GS, Kirks DR: Intussusception in infants and children: Diagnosis and treatment. Radiology 1998;168:141-145 5. StanleyA, Logan H, Bate TW, et al: Ultrasound in the diagnosis and exclusion of intussusception. Isr J Med Sci 1997;90:64-65 6. Lim HK, Bae SH, Lee HK, et al: Assessment of reducibility of ileocolic intussusception in children: Usefulness of color doppler sonography. Radiology 1994; 191:781-785 7. Bhistkul DM, Listernick R, Shkolnik A, et al: Clinical application of ultrasonography in the diagnosis of intussusception. J Pediatr 1992;121:182-186 8. Pracros JP, Tran-Minth VA, Morin De Finfe CH, et al: Acute intestinal intussusception in children: Contribution of ultrasonography. Ann Radiol (Paris) 1987;30:525-530 9. Swischuk LE, Hayden CK, Boulden T: Intussusception: Indications for ultrasonography and an explanation of the doughnut and pseudokidney signs. Pediatr Radiol 1985;15:388-391 10. Bowerman RA, Silver TM, Jaffe MH: Real-time ultrasound diagnosis of intussusception in children. Radiology 1982;143:527529 11. Peh WC, Khong PL, Chan KL, et al: Sonographic guided hydrostatic reduction of childhood intussusception using Hartmann's solution. Am Journ Roentgen 1996;167:1237-1241 12. Choi SO, Park WH, Woo SK: Ultrasound guided water enema: An alternative method of non-operative treatment for childhood intussusception. J Pediatr Surg 1994;29:498-500