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11110 Gastrointestinal Tract
typically reveals benign tissue though complica­tions may arise from cyst growth over time [26].
Necrotizing Enterocolitis
Necrotizing enterocolitis (NEC) is a multifactori­al disease that leads to disruption of intestinal in­tegrity followed ultimately by bowel necrosis and bacterial translocation. Birth weight is inversely associated with incidence (1:1000) and mortality
%). Younger gestational age is also a pre-
(15–30 dictor of poor outcome. of NEC includes feeding intolerance, abdominal distention, and often bloody stool [27].
Currently, abdominal radiograph remains the preferred modality of choice for diagnosis and classification of NEC. However, abdominal ul­trasound is increasingly being applied in NEC diagnosis and management for the advantages it offers over standard radiographs. Ultrasound permits visualization of bowel wall thickness and echogenicity, peristalsis, free fluid, and bowel wall perfusion [28]; this may allow for earlier diagnosis of bowel compromise and expedition of needed surgical intervention when bowel is threatened, but before actual perforation or frank necrosis occurs.
Pneumatosis intestinalis (PI) has been used as a marker for the condition. Most often observed in the ileum and colon, the finding is almost pathognomonic for NEC, though its presence ranges between 13 and 100 ing on the study examined. Ultrasound has prov­en superior
in the early diagnosis of PI. In a study of 40 neonates with Bell stage I NEC, ultrasound located PI in all 40, despite the lack of such find­ings on abdominal radiographs, demonstrating the value of ultrasound in making an early, de­finitive diagnosis [29]. However, for patients in
Bell stage II NEC, abdominal
been shown, in some studies, to be superior in identifying pneumatosis. Given this variation, more studies are needed to clarify the role of ul­trasound in diagnosing PI in neonates with NEC.
The protocol for ultrasound of the abdomen
for NEC has been well described by Faingold
al. [30] and includes evaluation of the intestine
et
The clinical presentation
% of cases, depend-
radiographs have
Fig. 10.13 Necrotizing enterocolitis—intramural gas
for increased ing (greater than 2.7 wall thinning (less than
wall echogenicity, wall thicken-
mm) suggestive of edema,
mm) suggestive of
1.0 ischemia, intramural gas (PI; Fig. 10.13), bowel wall perfusion on color Doppler, and bowel peristalsis. The peritoneal
cavity of the abdo­men and pelvis is then evaluated for free-fluid or discrete-fluid collections, and the liver and portal venous system are evaluated for portal venous gas (Fig.
10.14). The splanchnic
and hepatic cir­culation should then be assessed via Doppler for flow and perfusion. The sensitivity, specificity,
Fig. 10.14 Necrotizing enterocolitis—portal venous gas
112 C. M. Leeper et al.
positive predictive value, and negative predictive values of sonography for the detection of bowel necrosis were reported to be 100, 95, 80, and 100 %, respectively [31].
Appendicitis
Ultrasound is a reliable tool in the workup of pediatric abdominal pain and diagnosis of acute appendicitis (sensitivity 72.5 % (95 % CI = 58.8–
86.3 %) and specificity 97.0 % (95 % CI = 96.2–
97.9 %)) [32, 33]. It is now the standard of care in pediatric centers to obtain ultrasound imaging as the first-line modality with selective use of CT scan in some cases. This demonstrates a change in practice that has decreased the burden of ion­izing radiation in pediatric patients without an increase in the negative appendectomy or missed appendectomy rates [34, 35].
The sonographic criteria to diagnose acute ap­pendicitis in children include visualizing a blind­ending tubular structure that is noncompressible (Fig. 10.15) and lacks peristalsis, with an appen­dicular diameter greater than 6 mm and/or a wall thickness greater than 2.0 mm.
Other signs to support the diagnosis of appen­dicitis include the lack of air in the appendiceal lumen, periappendiceal fat stranding, presence of appendicolith, complex right lower quadrant mass, enlarged mesenteric lymph nodes, and presence of free fluid [36] (Fig. 10.16). Recent
Fig. 10.16 Appendicitis—enlarged tubular blind-ending structure in right lower quadrant with fecalith visible at the base, longitudinal view
evidence suggests that using a cutoff point of
7.0 mm appendicular diameter and 1.7 mm wall thickness [37] may be more predictive of acute appendicitis in pediatric patients.
Ultrasound is operator dependent and is limited by the lie of the appendix. In up to one third of the cases, the appendix may be nonvi­sualized on ultrasound imaging with the pa­tient in the traditional supine position. Some protocols recommend turning the patient to left posterior oblique position in an attempt to iden­tify a potentially retrocecal appendix [38]. In the absence of a leukocytosis, patients with a nonvi­sualized appendix can be safely observed without the immediate need for additional imaging [39].
Fig. 10.15 Appendicitis—sagittal view of noncompressible enlarged appendix
11310 Gastrointestinal Tract
Anorectal Malformations
Anorectal malformations are a complex group of congenital anomalies with an incidence of approximately 1:5000 births. The most com­mon presentation in males is imperforate anus (anal atresia) with a rectourethral fistula, and in females imperforate anus with a rectovestibular fistula [39]. Some patients are diagnosed ante­natally after visualizing dilated distal bowel or rectum on obstetric ultrasound [40, 41], though the technical difficulty in making that diagnosis means that most patients are identified by physi­cal examination on the first day of life. Ultra­sound plays many roles in the care of patients with anorectal malformations, including diag­nosis and preoperative planning, intraoperative guidance, and postoperative assessment.
For children with imperforate anus, early diagnosis and clarification of the patient’s anato­my is critical in planning the appropriate surgical intervention. The distance from bowel to skin is a determining factor in the type of procedure that is indicated: for a low defect (< 1 cm bowel-skin dis­tance), an anoplasty typically can be done without a colostomy; but for an intermediate or high defect (> 1 cm bowel-skin distance), a decompressive colostomy may be created before posterior sagit­tal anorectoplasty described by Pena [42]. Both radiographs (invertogram and prone cross-table lateral views) and ultrasound can be utilized to ob­tain this information. Transperineal ultrasound is feasible and valid for determining bowel-skin dis­tance with sensitivity 100 % and specificity 86 % with an error in distance measurement of 0.12 cm (± 0.33) [43, 44] (Fig. 10.17).
Another important consideration in patients with imperforate anus is the location of fistulae to adjacent anatomic structures. Transperineal ul­trasound can be utilized to identify the presence of multiple types of fistulae including rectoure­thral, rectovaginal, rectovestibular, rectovesical, and rectocloacal [45, 46]. Finally, preoperative evaluation is not complete without identification of any associated anomalies that frequently ac­company imperforate anus—a thorough evalu­ation typically includes abdominal, pelvic, and spine ultrasound [46].
Fig. 10.17 a and b Imperforate anus—a Transperineal ultrasound shows blind-ending rectum of a low imper­forate anus without fistula (note the round shape of the rectal tip), the ruler measures the bowel-skin distance ( A = skin); b Transperineal ultrasound of a different patient: the position of the rectum, the rectourethral fistula and the urethra can be clearly differentiated. To aid identification of the urethra, placement of a Foley catheter is helpful
114 C. M. Leeper et al.
Laparoscopy-assisted sagittal anorectoplasty with intraoperative ultrasound is an alternative to the traditional open procedure in some centers. A transperineal ultrasound probe in addition to electrical stimulation of the anal sphincter com­plex is applied to aid in identification of anatomic structures and creation of the pull through canal [47, 48].
Endoanal ultrasound may also be useful fol­lowing repair of anorectal malformations. An evaluation of the presence and function of the internal and external anal sphincters can guide therapy such as feedback training for symptoms like constipation or incontinence with the goal of improving quality of life [49].
Hirschsprung’s Disease
Hirschsprung’s disease (HD; incidence 1:5000) is the congenital absence of ganglion cells in the myenteric and submucosal plexuses of the intestine. In over 80 % of the cases, the affected location is the rectum or rectosigmoid. The diag­nostic pathway at present includes either a con­trast enema or anorectal manometry followed by confirmation with tissue biopsy [50]. Abdomi- nal ultrasound is not a first-line test in HD but, if utilized, will show massively dilated colon, possibly filled with meconium, and a contracted and empty rectum [51]. Orno and colleagues validated the use of ultrasound in visualizing the rectoanal inhibitory reflex, the reflex relaxation of the internal anal sphincter caused by distention of the normally innervated rectum that is absent in HD. The internal anal sphincter is viewed as a hypoechoic structure that decreases in diam­eter and allows passage of injected contents in a reactive test, but does not contract or result in movement of rectal contents in an inconclusive or nonreactive test [52].
Fig. 10.18 Ascites—anechoic, mobile fluid overlying the liver
sonographic characteristics of the fluid, such as wall structure, contour, and echogenicity, can aid in determining the underlying etiology [53]. A fluid collection may be anechoic (acute hem­orrhage, ascites; Fig. 10.18), hypoechoic (old hematoma, bile, and pus; Fig. 10.19), or hyper­echoic (air). A collection may be fixed (mass) or mobile (fluid), well circumscribed or poorly defined [54] (Fig. 10.20). Abdominal ultrasound is capable of providing an accurate depiction of peritoneal fluid which can greatly impact the management of the pediatric patient.
Peritoneal Fluid
Ultrasound can detect as little as 5–10 mL of fluid within the abdominal cavity. The differ­ential diagnosis for intraperitoneal fluid is vast;
Fig. 10.19 Organized hematoma—large, irregular, com- plex fluid collection in the pelvis displacing loops of in­testine
11510 Gastrointestinal Tract
Fig. 10.20 Free fluid—anechoic, mobile, ill-defined pelvic fluid adjacent to bladder, associated with acute ap­pendicitis
Abscess
Ultrasound is useful in both the diagnosis and management of abdominal and pelvic abscesses. The etiology is attributed to the introduction of enteric microorganisms into the peritoneal com­partment. This may occur from perforated ap­pendicitis, inflammatory bowel disease (IBD), and NEC, or ischemic enteritis; it may also occur after direct contamination from surgery or trau­ma. Mixed aerobic and anaerobic flora are typi­cally found within the abscess [55].
Walled-off fluid collections can be localized by scanning the subhepatic, subdiaphragmatic, pelvic, and interloop locations. Diagnosis is made through the identification of a hypoechoic
mass with irregular contour or an extraluminal air fluid level, with or without a localized ileus (Fig. 10.21). Ultrasound in the diagnosis of ab­scess is sensitive but not very specific, meaning that it is difficult to exclude abscess with ultra­sound alone.
Traditional ultrasound can be used to guide transabdominal percutaneous drainage of these abscesses with published success rates of any­where from 33 to 100 % depending on abscess location and etiology [55]. Transrectal ultrasound can also be used for localization and drainage of deep pelvic abscesses. Transrectal aspiration and/or drain placement can be accomplished with high success rates and low incidence of compli­cation [56].
Inflammatory Bowel Disease
Crohn’s disease (annual incidence 1–8.5:100,000) and ulcerative colitis (annual incidence 1–4.3:100,000) are common GI pathologies in pediatric patients. Diagnosis can be delayed due to the nonspecific nature of patient complaints and often relies on a variety of imaging studies as well as endoscopy, tissue biopsy, and laboratory testing [57]. The low cost, lack of radiation, and high-negative predictive value make abdominal ultrasound an important initial imaging modality in the workup of suspected IBD. It can also be
Fig. 10.21 Abscess—17-year-old female patient who presented with ruptured appendicitis, ultrasound demonstrated an irregular, localized, hypoechoic fluid collections in the pelvis and left lower quadrant (pictured)
116 C. M. Leeper et al.
Fig. 10.22 Perianal abscess—Transrectal ultrasound demonstrates a perianal, heterogeneous, localized fluid collection measuring 6.3 × 3.3 × 5.9 cm that is surrounded by hyperemia
used to surveil patients who carry a diagnosis of IBD for active inflammation and complications like abscess or stricture [58] (Fig. 10.22). Accu­racy for diagnosing the number and site of small bowel lesions is enhanced when oral contrast is given [59]. Importantly, limitations to the use of ultrasound in IBD include the fact that many findings are nonspecific, the intestine cannot be assessed over its entire length, and the quality of the images and interpretation are operator depen­dent [57].
Other Diseases
Ultrasound may also demonstrate nonspecific findings including dilated, fluid-filled loops of intestine, wall thickening or hyperemia (Fig. 10.23). These findings are seen across a range of pathology and may indicate infectious or inflammatory enteritis, partial obstruction, or other issue. Imaging in this case is not diagnostic but can help to guide the clinician in conjunction with the clinical history, laboratory testing, and physical examination.
Abdominal ultrasound is a common first-line test for pediatric patients presenting with general abdominal pain, and consequently may identify unusual pathology that is more commonly associ­ated with other imaging modalities. For instance, a patient with Henoch–Schönlein purpura who
Fig. 10.23 Nonspecific finding of mildly dilated, thick- ened loops of small bowel
presented with hematochezia was found to have severe bowel-wall thickening throughout the ab­domen, consistent with intramural hemorrhage (Fig. 10.24a and b). A bezoar can be detected as an intraluminal mass with a hyperechoic curved surface and an acoustic shadow [60]. In devel­oping regions where certain infectious etiologies are more common and in patients who offer a history of travel to such locations, the differen­tial must include parasitic and tuberculin disease. Intestinal ascariasis may be diagnosed after vi­sualizing echogenic structures within the lumen at multiple locations, arranged in a thin line or coil [61]. Intestinal tuberculosis may appear as bowel-wall thickening with intramural abscesses, with or without fistula; there may also be evi­dence of mesenteric thickening accompanied by enlarged mesenteric lymph nodes [62]. Hydatid cysts, commonly located in the abdomen or the liver, show a typical ultrasound appearance.
Summary
Abdominal ultrasound is a popular modality for the screening and diagnosis of GI pathology in pediatric patients. There is a steep learning curve for the operator given the challenges of imaging the dynamic and variable intestine; however, pro­ficiency can be achieved with training and expe­rience. Ultrasound can be utilized to identify a broad spectrum of pathology.
11710 Gastrointestinal Tract
Fig. 10.24 a and b Henoch–Schoenlein purpura—extreme wall thickening consistent with intramural hemorrhage
118 C. M. Leeper et al.
Hernanz-Schulman M. Infantile hypertrophic py-
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