Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

September 2003

The Radiologic Manifestations of Necrotizing Enterocolitis

Lynn Ramirez-Avila Harvard Medical School Year IV Gillian Lieberman, MD

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Baby R
Former 28.5 weeker with episodes of respiratory distress in the first days of life On day of life 8 Baby R started full feeds On day of life 8 Baby R developed marked abdominal distension & guaiac positive stools
2

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Objectives
Overview of necrotizing enterocolitis Overview of common radiologic findings in nectrotizing enterocolitis Review the future role of imaging modalities in diagnosing necrotizing enterocolitis
3

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Necrotizing Enterocolitis (NEC)

Is the necrosis of the mucosa or submucosa of any portion of the GI tract Affects predominantly preterm & low birth weight infants Other risk factors include:
Compromise of mucosal integrity & bowel integrity Compromised mesenteric blood supply Changes in bowel lumen
4

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Pathophysiology
Multifactorial process that usually affects terminal ileum and right colon Exact pathophysiologic mechanism is not known, but it is thought that:
Bacterial colonization Intestinal hypoxia Formula feeding Activation of proinflammatory mediators & subsequently bowel necrosis
5

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Arterial Supply of the Colon

Superior Mesenteric Artery Ileocolic, Right colic, Superior Mesenteric Arteries

Terminal Ileum, Cecum, Right Colon Venous Tributaries

Superior Mesenteric Vein Hepatic Portal Vein

6
Norman W, http://mywebpages.comcast.net/wnor/smlintestinebloodsupply.jpg

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Clinical Presentation
Usually occurs in days 3-10 of life Systemic symptoms include:
Apnea, bradycardia, temperature instability, lethargy, poor feeding

Gastrointestinal symptoms include:

Diarrhea, abdominal distention, gastric retention, gasterointestinal bleeding
7

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

BELL Criteria
Stage
I (Suspected)

Clinical Signs
Abdominal distension, poor feeding, vomiting Abdominal distension, poor feeding, vomiting, GI bleeding Abdominal distension, poor feeding, vomiting, GI bleeding & septic shock

II (Definite)

III (Advanced)

8
Summarized from Rencken et al, 1997

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Imaging Modalities
Radiologic imaging is key to diagnosis and monitoring If NEC is suspected, abdominal films are obtained every 12-24 hours Supine abdominal, cross table lateral view, or left-side-down decubitus are standard
9

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Plain films and Bell Criteria

Stage
I (Suspected)

Clinical Signs
Abdominal distension, poor feeding, vomiting Abdominal distension, poor feeding, vomiting, GI bleeding Abdominal distension, poor feeding, vomiting, GI bleeding & septic shock

Radiologic Findings
Ileus

II (Definite)

Intestinal pneumatosis & portal venous air

III (Advanced)

Ileus, intestinal pneumatosis, portal venous air, pneumoperitoneum

10
Summarized from Rencken et al, 1997

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Normal Neonatal Abdominal Radiograph

Liver is prominent in pediatric abdominal films Difficult to discern the small from large intestine Bowel gas pattern bordering the liver is likely to be the transverse colon Bowel gas pattern in the lower pelvic region likely to be the rectum

11
Courtesy Dr. W. Durgin, BIDMC

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Radiography & Stage I NEC

Nonspecific radiographic findings:
Diffuse gaseous distension of intestine Loss of normal bowel gas pattern symmetry

12

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Bowel Distension
Occurs in the small intestine, colon, or both Distension of the small intestine often occurs 4-48 hours before the onset of clinical signs Distension of large colon occurs in 30% of NEC patients This is a relatively non-specific sign

From Buonomo, C. 1999. Imaging of neonatal gastrointestinal obstruction. Rad Clin North America, 37(6): 1187-98.

13

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Bowel Distension Radiographic Differential

Meconium Ileus Total Colonic Anganlionosis Mid-gut volvulus Gastroenteritis, peritonitis, sepsis

14

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Abnormal Gas Distribution

From Buonomo, C. 1999. Imaging of neonatal gastrointestinal obstruction. Rad Clin North America, 37(6): 1187-98.

15

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Radiology & Stage II NEC

Pneumatosis intestinalis is essentially pathognomonic for NEC Portal venous gas is correlated with worse prognosis

16

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Pneumatosis Intestinalis
Intramural

Air

Focal versus diffuse Air can be located in the - Submucosa Bubbly/cystic - Subserosa Linear/curvilinear
17
Courtesy of Dr. Makris, Childrens Hospital Boston

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Pneumatosis Intestinalis (continued)

Radiographic Differential
In combination with dilated bowel indicative of NEC Feces Milk impaction secondary to onset of feeding Benign pneumatosis from extension from air in the mediastinum Congenital obstruction (atresias, imperforate anus, meconium plug, etc. Hirschsprungs
18
Courtesy of Dr. Makris, Childrens Hospital Boston

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Portal Venous Gas (PVG)

Associated with severe NEC and babies with PVG have worst outcomes Visualized better on cross table lateral view On ultrasound PVG is seen as moving echogenicity in portal vein
Courtesy Dr. W. Durgin, BIDMC

19

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

PVG (continued)
Radiographic Differential Iatrogenic via umbilical vein catheters Air in biliary tree secondary to duodenal atresia with incompetent Sphincter of Oddi

http://www.hawaii.edu/medicine/pediatrics/neoxray/neoxray.html

20

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Radiology & Stage III NEC

Persistent (sentinel) loop sign Asymmetric bowel dilatation Ascites Pneumoperitoneum
21

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Persistent Loop Sign (Sentinel Loop)

Is the persistence of a dilated loop of bowel on subsequent radiographs for 24 to 36 hours
Radiographic Differential Appendicitis Paralytic Ileus Pancreatitis Drug-induced
From Buonomo, C. 1999. Imaging of neonatal gastrointestinal obstruction. Rad Clin North America, 37(6): 1187-98

22

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Pneumoperitoneum
Radiographic Differential Idiopathic perforation Focal intestinal perforation Intestinal obstruction Iatrogenic (puncture with nasogastric tube)
http://bms.brown.edu/pedisurg/Brown/Image%20bank%20pages/NEC.html

23

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Baby R
Baby gram radiographic findings:

- Distended bowel loops

-Pneumatosis intestinalis

- Free Air under the left diaphragm

24
Courtesy of Dr. Makris, Childrens Hospital Boston

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Treatment of NEC
Clinical Management
Discontinue feeds IV fluids Gastric decompression via NG tube Total parenteral nutrition Broad-spectrum antibiotics

Surgical Management
Indications include pneumoperitoneum, sentinel loops, ascites, or worsening clinical picture Resection of the necrotic bowel, proximal enterostomy, with subsequent reanastomosis at a later time

25

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Outcome of Baby R
Underwent exploratory laparotomy

Subsequently had right hemicolectomy with ileocecal valve resection

Follow-up radiograph is shown

26
Courtesy of Dr. Makris, Childrens Hospital Boston

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Chronic Complications of NEC

Usually occurs a few weeks after acute disease Radiographic follow-up conducted for 2 years Course can be complicated by NEC strictures, bowel obstruction, enterenterofistulae, enterocysts
27

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Post-NEC Stricture
Single or multiple strictures occur Commonly occur in the left colon Spontaneous resolution of NEC strictures occurs Routine barium enemas are performed in children who undergo surgery, but not in children who have had medical management of NEC
28

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

NEC Strictures

From Buonomo, C. 1999. Imaging of neonatal gastrointestinal obstruction. Rad Clin North America, 37(6): 1187-98

From Rabinowitz, JG. Radiographic Manifestations in Neonatal Necrotizing Enterocolitis, Brown EG, Sweet AY eds. 1980

29

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

NEC & other Imaging Modalities

Abdominal plain radiographs are nonspecific in the early and late stages of NEC The use of computed tomography in NEC diagnosis has been explored Use of MRI in NEC diagnosis is under study
30

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

NEC & CT
The permeation of contrast administered into ischemic bowel has been reported in animal models of NEC The contrast is resorbed from the peritoneum & is excreted into the urinary system
31

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

NEC & CT (continued)

Study included babies with NEC and controls (n=22) who were orally given non-ionic contrast After contrast administration, they collected the urine of babies with NEC and controls and CT the urine They found that after contrast, the urine of babies with suspected and definite NEC have higher Hounsfield Units than controls
Control Urine 5.6 HU +/- 3.9 Urine of babies who Urine of babies with Urine of babies with suspected NEC definite NEC underwent other GI study 6.7 HU +/- 3.2 26.0 HU +/- 3.4 71.0 HU +/- 18.8
32
summarized from Rencken et al, 1997

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

NEC & MRI

Maalouf et al report MRI findings of NEC amongst a group of low birth weight and preterm infants and their controls They conclude that the following characteristics were associated with severe forms of NEC:
Fluid levels within lumen Intramural gas Bubble-like appearance in bowel wall

Bowel areas with a bubble-like appearance corresponded to areas of bowel that were surgically resected
33

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

NEC & MRI

Intramural Air

Bubble-like Appearance

Air-fluid levels

From Maalouf: Pediatrics, Volume 105(3).March 2000.510-514

34

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

NEC & Ultrasound

Pseudo-kidney sign Non-specific sign seen in any process where blood, pus, fluid, tumor invades bowel wall

Necrotic bowel cannot be distinguished from inflammatory bowel disease

Given, demographics of preterm population, this sign is most consistent with NEC

From Kodroff et al, 1984

35

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Portal Venous Gas & Ultrasound

Right Upper Quadrant U/S Liver Ultrasound of Neonate with NEC

Normal Ultrasound

Echogenicities in liver parenchyma

Microbubble in portal vein 36

From Merrit et al, 1984

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Conclusion
The incidence of NEC is increasing secondary to increased survival of low-birth weight and preterm infants Diagnostic imaging, specifically plain films are important in the diagnosis, progression, and follow-up of NEC But, secondary to the sensitivity of current abdominal plain films, the use of CT, MRI, and U/S could provide a more sensitive and specific imaging alternative
37

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

References
Brown University Department of Pediatric Surgery http://bms.brown.edu/pedisurg/Brown/Image%20bank%20pages/NEC.html Buonomo C (1999). The Radiology of Necrotizing Enterocolitis in Neonatal Imaging Rad Clin North America 37: 1999. Caplan MS, Jilling T (2001). New Concepts in necrotizing enterocolitis. Curr Opin Ped 13: 111. Kodroff MB, Hartenberg, MA, Goldschmidt RA (1984). Ultrasonographic diagnosis of gangrenous bowel in neonatal necrotizing enterocolitis. Ped Rad 14: 168. Fotter R, Sorantin (1994). Diagnostic imaging in necrotizing enterocolitis. Acta Paed Supp 398: 41. Kodroff MB, Hartenberg, MA, Goldschmidt RA (1984). Ultrasonographic diagnosis of gangrenous bowel in neonatal necrotizing enterocolitis. Pediatr Radiol 14: 168. Merritt CRB, Goldsmith JP, Sharp MJ. (1984) Sonographic Detection of Portal Venous Gas in Infants with Necrotizing Enterocolitis. AJR 143: 1059. Maalouf EF, Fagbemi A, Duggan PJ, Jayanthi S, Counsell SJ, Lewis HJ, Fletcher AM, Lakhoo K, Edwards AD. (2000) Magnetic Resonance Imaging of Intestinal Necrosis in Preterm Infants. Pediatrics 105: 510. Norman, W. Superior Mesenteric Artery Ilustration available [Online] http://mywebpages.comcast.net/wnor/smlintestinebloodsupply.jpg. September 10, 2003. Rabinowitz, JG. (1980). Radiographic Manifestations in Monographs in Neonatology: Neonatal Necrotizing Enterocolitis Brown EG, Sweet AY (eds). New York: Grune and Stratton. Reeder MM, WG Bradley (2001). Reeder and Felsons Gamuts in Radiology: Comprehensive List of Roentgen Differential Diagnosis. New York: Springer Verlag Publishing. Renken IO, Sola A, Al-Ali F, Solano JP, Goldbergt HI, Cohen PA, Gooding CA. (1997). Necrotizing Enterocolitis: Diagnosis with CT Examination of Urine after Enteral Administration of Iodinated Water-soluble Contrast Material. Ped Radiology 205: 87. Schanler RJ. (2003). Up to Date: Clinical features and treatment of necrotizing enterocolitis in newborns available [Online] www.uptodate.com September 10, 2003. Wood BP. (2002). E medicine: Necrotizing Enterocolitis available [Online]: http://www.emedicine.com/radio/topic469.htm.

38

Lynn Ramirez-Avila, HMS IV Gillian Lieberman, MD

Acknowledgements
Dr. J. Makris, Childrens Hospital Boston Dr. W. Durgin, BIDMC Dr. G. Lieberman, BIDMC Pamela Lepkowski Larry Barbaras, BIDMC Webmaster

39