儿童肠梗阻影像学表现

Matthew Zerden, HMS III Gillian Lieberman, MD Pediatric Bowel Obstruction Matt Zerden, Harvard Medical School III Gillian Lieberman, MD Matthew Zerden, HMS III Gillian Lieberman, MD Patient 1 • 16 year old presents with severe, episodic abdominal pain, nausea and vomiting. • Questionable abdominal mass in RLQ • Previous images from his record include the following CXR. What is his underlying condition? Matthew Zerden, HMS III Gillian Lieberman, MD Patient 1: Chest X-ray Image courtesy of Dr. Andrew Hines-Peralta • Markedly abnormal • Coarse, reticular interstitial diffuse infiltrates • Significant scarring, most notably along the minor fissure of the right lung • Consistent with bronchiectasis from long-standing respiratory infections in the setting of cystic fibrosis Matthew Zerden, HMS III Gillian Lieberman, MD Patient 1: Initial KUB Image courtesy of Dr. Andrew Hines-Peralta • Plain abdominal film shows mildly dilated loops of SB with multiple air fluid levels. • The transverse and descending color are relatively decompressed. • Concerning for an early obstructive process, but more imaging is required to narrow the differential diagnosis Matthew Zerden, HMS III Gillian Lieberman, MD Patient 1: Differential Dx • Given his age, symptoms of severe pain, and KUB, concerned about: – Appendectomy – Severe gastroenteritis • In the setting of CF, the differential is expanded to include the following: – Distal intestinal obstruction syndrome (previously known as “Meconium Ileus Equivalent”) – Intussusception – Adhesions (from prior abdominal surgeries) • Obtain a CT Matthew Zerden, HMS III Gillian Lieberman, MD Patient 1: Intussusception on Coronal CT Images courtesy of Dr. Andrew Hines-Peralta • Coronal CT slice with IV contrast showing a classic image of bowel within bowel, diagnosing an intussusception. • Other coronal CT slices confirmed that the intussusception occurred at the ileocecal junction, the most common site of intussuscpetion. Matthew Zerden, HMS III Gillian Lieberman, MD Patient 1: Intussusception on US Image from Timothy J. Carroll, MD, PhD and Janice M. Gallant, MD http://www.appliedradiology.com/articles/article.asp?ID=977&AdKeyword=Null&Search=intussusception • This slide is not from this patient, but demonstrates the classic “target” lesion of intussusception on ultrasound. • You can see the outer echoic layer of bowel surrounding an anichoic layer of fat surrounding the inner echoic layer of bowel. Matthew Zerden, HMS III Gillian Lieberman, MD Patient 1: Intussuscpetion on Contrast Enema Image courtesy of Dr. Andrew Hines-Peralta • Enemas can be used both to diagnose and treat intussuscption, with successful reduction occurring in 75-90% of cases • Here gastrograffin was used to opacify the large bowel. The terminal ileum is no longer telescoping into the cecum, demonstrating effective treatment of the intussusception. Matthew Zerden, HMS III Gillian Lieberman, MD Patient 1 Discussion: Intussusception • Occurs in 1 % of patients with CF • Typical presentation: Non-CF, previously healthy infant with sudden onset colicky abdominal pain, vomiting and red currant jelly stool • Mean age at presentation is 6-8 months • Can be fatal if untreated for 2-5 days • Males > females. This difference increases as children become older • Conditions that predispose children: – Recent URI or GI illness; Henoch-Schönlein purpura; CF; Chronic GI tubes; Meckel diverticulum; GI polyp; blunt trauma leading to intestinal hematoma; foreign body; or any other process creating a “lead point” • Radiological work-up: begin with plain film to look for obstruction; proceed with US, CT or barium enema depending on clinical suspicion Matthew Zerden, HMS III Gillian Lieberman, MD Patient 1: returns 1 year later with severe abdominal pain Image courtesy of Dr. Andrew Hines-Peralta • Dilated loops of air filled small bowel. • Distal large bowel is fairly decompressed. • Bubbly, stool filled lower right quadrant • These findings consistent with a mechanical obstruction of the small bowel • Differential is the same as in previous presentation of this patient, proceed to CT. Matthew Zerden, HMS III Gillian Lieberman, MD Patient 1: Coronal CT with IV contrast Image courtesy of Dr. Andrew Hines-Peralta • Dilated, fecalized SB loops filled with semi-solid fecal matter. • Note that this is abnormal - the SB should be filled with only fluid and gas. • This image is diagnostic for Distal Intestinal Obstruction Syndrome (DIOS) • DIOS is complete or partial obstruction of the bowel lumen by intestinal contents Matthew Zerden, HMS III Gillian Lieberman, MD Patient 1: Contrast Enema Images courtesy of Dr. Andrew Hines-Peralta Gastrograffin enema showing fecalization of the ileum, with a filling defect shown as a faint lucency. Abdominal X-ray s/p gastrograffin enema, with residual contrast lining the colonal haustra. SB loops are no longer distended. Matthew Zerden, HMS III Gillian Lieberman, MD Patient 1 Discussion: DIOS • Formerly known as “Meconium ileus equivalent”, DIOS is condition that occurs almost exclusively in CF patients, mostly adolescents and adults. • Prevalence is 5% - 40% of older CF patients • Viscid bowel contents become impacted in the distal small bowel • Radiograph: Distal SBO with bubbly stool filled lower right quadrant • Contrast enema can be therapeutic and avoid surgery Matthew Zerden, HMS III Gillian Lieberman, MD Patient 1 Discussion: Meconium Ileus • Meconium ileus: A condition that typically occurs in newborns with CF where abnormal meconium is thickened and obstructs the distal ileum • 10 % of CF patients present with meconium ileus, but it can also occur with congenital pancreatic diseases • Typical clinical presentation in the newborn: bilious vomiting, abdominal distention and failure to pass meconium • Radiographs will show a mechanical obstruction, most commonly at the ileo-cecal junction, with an absence of air fluid levels Matthew Zerden, HMS III Gillian Lieberman, MD Can’t Miss Cases of Pediatric Bowel Obstruction • There are a few pediatric conditions with characteristic radiological findings • Most are present at birth or in the first few months of life • Many of these conditions are known to be associated with other specific congenital abnormalities or occur as part of syndromes Matthew Zerden, HMS III Gillian Lieberman, MD Patient 2: Abdominal X-ray • Previously healthy 27 day old, first-born male presents with “projectile”, non-bilious vomiting • Palpable mass in RUQ • Abdominal film shows large, distended stomach • Gas pattern in small and large bowel are non- specific. Image courtesy of Dr. Andrew Hines-Peralta Matthew Zerden, HMS III Gillian Lieberman, MD Patient 2: Pylorus Stenosis • This is the classic presentation of infantile hypertrophic pyloric stenosis, resulting in gastric outlet obstruction • One of the most common forms of bowel obstruction with an incidence of 2-4 per 1000 live births • Male-to-female predominance of 4:1, with 30% of patients being first-born males. • The usual age of presentation is approximately 3 weeks of life, with 95% of cases diagnosed between 3-12 weeks • Corrected by pyloromyotomy Image courtesy of Dr. Andrew Hines-Peralta Matthew Zerden, HMS III Gillian Lieberman, MD Patient 2: Pylorus Stenosis Image courtesy of Dr. Andrew Hines-Peralta • Radiograph with barium contrast in the stomach • Barium is unable to move past the pylorus, resulting in “shouldering” of the barium Matthew Zerden, HMS III Gillian Lieberman, MD Patient 2: Pylorus Stenosis on US Image courtesy of Dr. Andrew Hines-Peralta • US with calipers measuring the size of the pylorus. • US is the modality of choice for imaging pylorus stenosis because it allows a quantitative measurement, with a pylorus of > 4mm being diagnostic. Matthew Zerden, HMS III Gillian Lieberman, MD Patient 3: Abdominal X-ray Image courtesy of Dr. Andrew Hines-Peralta • 10 month old presents with intermittent, abdominal pain. • Abdominal distention is noted on physical exam. • No significant past medical history. Matthew Zerden, HMS III Gillian Lieberman, MD Patient 3: Inguinal hernia Image courtesy of Dr. Andrew Hines-Peralta • Multiple loops of dilated small bowel • No apparent transition point • Upon close inspection of the scrotum, areas of lucencies are seen • Diagnosis: Incarcerated inguinal hernia Matthew Zerden, HMS III Gillian Lieberman, MD Patient 4: Contrast Enema • A 3 month old male child with a history of colicky abdominal pain and constipation • The patient had delayed (greater than 24 hours) passage of meconium at birth • One of the parents has suffered with constipation all their life Image courtesy of Dr. Andrew Hines-Peralta Matthew Zerden, HMS III Gillian Lieberman, MD Patient 4: Hirschsprung Disease (HD) • Supine x-ray with contrast in the large bowel. • Markedly abnormal, narrowed segment in the sigmoid colon. • Just proximal to the narrowed segment is enlarged colon Image courtesy of Dr. Andrew Hines-Peralta Matthew Zerden, HMS III Gillian Lieberman, MD Patient 4 Discussion: HD • HD is caused by failure of neural crest cell migration to both the myenteric and submucal ganglion • This patient had short segment Hirshprung, the most common form of the condition that affects only the rectosigmoid region vs. long-segment which can affect the entire colon • Occurs in 1/5000 live births, with an overall male:female ratio of 4:1 • Associated with other congenital abnormalities and syndromes, most notably Down’s syndrome in 10% of cases • Diagnosis requires a biopsy even if highly suggested HD via imaging • Presentation can be variable from complete obstruction to undiagnosed disease that produces constipation through adulthood Matthew Zerden, HMS III Gillian Lieberman, MD Patient 5: Abdominal X-ray www.e-radiography.net/radpath/d/duodenal_atresia2.jpg •Newborn with a prenatal history of polyhydramnios •Born with Down’s Syndrome • Within 24 hours has multiple episodes of bilious vomiting Matthew Zerden, HMS III Gillian Lieberman, MD Patient 5: Duodenal Atresia www.e-radiography.net/radpath/d/duodenal_atresia2.jpg •Characteristic “double-bubble” showing air in the stomach and duodenum •Failure of duodenum to completely form Matthew Zerden, HMS III Gillian Lieberman, MD Patient 5 Discussion: Duodenal Atresia • Atresias can occur anywhere along the GI tract, with ileum being the most common site in the intestines • Most atresias in the jejunum or ileum occur in isolation, while duodenal atresias co-occur with other congenital abnormalities in 50% of cases • Additionally, duodenal atresia is associated with Down’s Syndrome - 22-30% of patients with duodenal obstruction have trisomy 21. • Polyhydramnios occurs in 33% to 50% of cases • 45% of those with duodenal atresia are born premature Matthew Zerden, HMS III Gillian Lieberman, MD Patient 6: Abdominal X-ray • Premature infant born weighing 1300 grams • On the 10th day in the NICU, he begins to have decreased feedings, abdominal distention, and decreased bowel sounds • Later he develops bloody stools, respiratory distress and lethargy http://www.adhb.govt.nz/newborn/TeachingResources/Radiology/NEC.htm Matthew Zerden, HMS III Gillian Lieberman, MD Patient 6: Necrotizing Enterocolitis (NEC) • This supine abdominal radiograph shows dilated loops of bowel • Pneumatosis coli appears as linear lucencies along the bowel wall • There is distension of the stomach. • There are several lucencies in the liver, representing portal venous gas. http://www.adhb.govt.nz/newborn/TeachingResources/Radiology/NEC.htm Matthew Zerden, HMS III Gillian Lieberman, MD Patient 6 Discussion : NEC • Necrotizing Enterocolitis is a disorder commonly effecting low birth weight infants, with a mortality rate between 15-30% • Approximately 5-10% of infants with a birth weight less than 1500 grams will develop NEC with the incidence increasing with decreasing gestational age • The etiology is uncertain, but leading research suggests a combination of infection combined with ischemia and reperfusion injury which sets off an inflammatory cascade • Patients can rapidly evolve into sepsis. • If it is not caught early, permanent bowel necrosis can occur resulting in surgery with significant bowel resection. • Following the acute event, the patient can have long term consequences of damaged bowel wall, such as strictures, which occur in approximately 25% of those who survive NEC. Matthew Zerden, HMS III Gillian Lieberman, MD References • Agrons GA; Corse WR; Markowitz RI; Suarez ES; Perry DR. Gastrointestinal manifestations of cystic fibrosis: radiologic-pathologic correlation. Radiographics. 1996 Jul;16(4):871-93. • DiFiore JW. Intussusception. Semin Pediatr Surg. 1999 Nov;8(4):214-20. • Escobar MA; Ladd AP; Grosfeld JL; West KW; Rescorla FJ; Scherer LR, Engum SA; Rouse TM; Bilmire DF. Duodenal atresia and stenosis: long- term follow-up over 30 years. J Pediatr Surg. 2004 Jun;39(6):867-71. • Kessmann J. Hirschsprung's Disease: Diagnosis and Management. American Family Physician. 2006 Oct; 74 (8): 1319-22 • Khoshoo V; Udall JN Jr. Meconium ileus equivalent in children and adults. Am J Gastroenterol. 1994 Feb;89(2):153-7. • Lin PW; Stoll BJ. Necrotising Enterocolitis. Lancet. 2006 Oct 7;368(9543):1271-83. • Schechter R; Torfs CP; Bateson TF; The epidemiology of infantile hypertrophic pyloric stenosis. Paediatr Perinat Epidemiol. 1997 Oct; 11(4):407-27. Matthew Zerden, HMS III Gillian Lieberman, MD Acknowledgments • Dr. Andrew Hines-Perlata for his time, teaching and image contributions • Gillian Lieberman, MD for her efforts in developing this course and creating the opportunity for this presentation • Pamela Lepkowski for her assistance throughout the course • Larry Barbaras for his efforts as webmaster Pediatric Bowel Obstruction Patient 1 Patient 1: Chest X-ray Patient 1: Initial KUB Patient 1: Differential Dx Patient 1: Intussusception on Coronal CT Patient 1: Intussusception on US Patient 1: Intussuscpetion on Contrast Enema Patient 1 Discussion: Intussusception Patient 1: returns 1 year later with severe abdominal pain Patient 1: Coronal CT with IV contrast Patient 1: Contrast Enema Patient 1 Discussion: DIOS Patient 1 Discussion: Meconium Ileus Can’t Miss Cases of Pediatric Bowel Obstruction Patient 2: Abdominal X-ray Patient 2: Pylorus Stenosis Patient 2: Pylorus Stenosis Patient 2: Pylorus Stenosis on US Patient 3: Abdominal X-ray Patient 3: Inguinal hernia Patient 4: Contrast Enema Patient 4: Hirschsprung Disease (HD) Patient 4 Discussion: HD Patient 5: Abdominal X-ray Patient 5: Duodenal Atresia Patient 5 Discussion:� Duodenal Atresia Patient 6: Abdominal X-ray Patient 6: Necrotizing Enterocolitis (NEC) Patient 6 Discussion : NEC References Acknowledgments