Abstract
A wide range of congenital anomalies may affect the whole gastrointestinal tract, from the esophagus to the rectum. High intestinal obstructions are those occurring proximal to the ileum, whereas low obstructions involve the distal ileum and colon and typically result in dilation of several bowel loops. These abnormalities are a significant cause of morbidity in children and, less frequently, in adults. Most of these congenital anomalies, especially those involving the small intestine, present with obstructive symptoms, while some of them with abdominal pain, vomiting, and gastrointestinal bleeding. Definitive therapy is surgical, and in some cases, it has to be performed urgently. Their diagnosis may require several imaging modalities in order to tailor the most appropriate treatment. Some of them, such as gastric or high intestinal obstruction, do not usually require further investigation; on the other hand, low obstructions require a barium enema study. US has become important for the evaluation of some of these abnormalities; it can help to differentiate a small bowel obstruction from a colonic obstruction. In addition, it may help to suggest possible anorectal malformations through dynamic procedures. A detailed anatomical investigation is crucial in tailoring the most appropriate surgical approach for each patient. For this reason MRI is essential because it well depicts the pelvic and perineal anatomy in order to evaluate the location and morphology of the anorectal malformation and to study the grade of development of the puborectalis and sphincteric musculature before the surgery.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Al Shahwani N, Mandhan P, Elkadhi A, Ali MJ, Latif A (2013) Congenital duodenal obstruction associated with Down’s syndrome presenting with hematemesis. J Surg Case Rep 2013(12). pii: rjt108. doi:10.1093/jscr/rjt108
Alabbad SI, Ryckman J, Puligandla PS, Shaw K, Nguyen LT, Laberge JM (2009) Use of transanastomotic feeding tubes during esophageal atresia repair. J Pediatr Surg 44:902–905
Alamo L, Laswad T, Schnyder P et al (2010) Fetal MRI as complement to US in the diagnosis and characterization of anomalies of the genito-urinary tract. Eur J Radiol 76(2):258–264
Alamo L, Meyrat BF, Meuwly JY, Meuli RA, Gudinchet F (2013) Anorectal malformations: finding the pathway out of the labyrinth. Radiographic 33:491–512
Ali YA, Rahman S, Bhat V, Al Thani S, Ismail A, Bassiouny I (2011) Hereditary multiple intestinal atresia (HMIA) with severe combined immunodeficiency (SCID): a case report of two siblings and review of the literature on MIA, HMIA, and HMIA with immunodeficiency over the last 50 years. BMJ Case Rep. doi:10.1136/bcr.05.2010.3031. pii: bcr0520103031
Anderson N, Malpas T, Robertson R (1993) Prenatal diagnosis of colon atresia. Pediatr Radiol 23:63–64
Applegate KE, Goske MJ, Pierce G, Murphy D (1999) Situs revisited: imaging of the heterotaxy syndrome. RadioGraphics 19:837–852; discussion, 853–854
Azzie G, Craw S, Beasley SW (2002) Colonic atresia: from suspicion to confirmation on pre-operative radiology. J Paediatr Child Health 38:518–520
Baglaj M, Carachi R, Lawther S (2008) Multiple atresia of the small intestine: a 20-year review. Eur J Pediatr Surg 18:13–18
Benawra R, Puppala BL, Mangurten HH, Booth C, Bassuk A (1981) Familial occurrence of congenital colonic atresia. J Pediatr 99:435–436
Berrocal T, Torres I, Gutierrez J, Prieto C, del Hoyo ML, Lamas M (1999a) Congenital anomalies of the upper gastro-intestinal tract. Radiographics 19:855–872
Berrocal T, Lamas M, Gutieerrez J, Torres I, Prieto C, del Hoyo ML (1999b) Congenital anomalies of the small intestine, colon, and rectum. Radiographics 19(5):1219–1236
Best KE, Tennant PW, Addor MC, Bianchi F, Boyd P, Calzolari E et al (2012) Epidemiology of small intestinal atresia in Europe: a register based study. Arch Dis Child Fetal Neonatal Ed 97(5):F353–F358
Bischoff A, Levitt MA, Lim FY, Guimarães C, Peña A (2010) Prenatal diagnosis of cloacal malformations. Pediatr Surg Int 26(11):1071–1075
Blair GK, Jamieson DH (2001) Colon atresia-type III. J Pediatr Surg 36:530–531
Boemers TM, Beek FJ, Bax NM (1999) Guidelines for the urological screening and initial management of lower urinary tract dysfunction in children with anorectal malformations: the ARGUS protocol. BJU Int 83(6):662–671
Brantberg A, Blaas HG, Salvesen KA, Haugen SE, Mollerlokken G, Eik-Nes SH (2002) Fetal duodenal obstructions: increased risk of prenatal sudden death. Ultrasound Obstet Gynecol 20(5):439–446
Calisti A, Olivieri C, Coletta R, Briganti V, Oriolo L, Giannino G (2012) Jejunoileal atresia: factors affecting the outcome and long-term sequelae. J Clin Neonatol 1(1):38–41
Calvo-Garcia MA, Kline-Fath BM, Levitt MA et al (2011) Fetal MRI clues to diagnose cloacal malformations. Pediatr Radiol 41(9):1117–1128
Chew AL, Friedwald JP, Donovan C (1992) Diagnosis of congenital astral web by ultrasound. Pediatr Radiol 22:342–343
Clements JL, Jinkins JR, Torres WE et al (1979) Antral mucosal diaphragm in adults. AJR Am J Roentgenol 133:1105–1109
Coran AG, Eraklis AJ (1969) Atresia of the colon. Surgery 65:828–831
Correia-Pinto J, Ribeiro A (2014) Congenital duodenal obstruction and double-bubble sign. N Engl J Med 371(11):e16
Cox SG, Numaouglu A, Millar AJW, Rode H (2005) Colonic atresia: spectrum of presentations and pitfalls in management. A review of 14 cases. Pediatr Surg Int 21:813–818
Cumming WA (1975) Esophageal atresia and tracheoesophageal fistula. Radiol Clin N Am 13:277–285
Currarino G, Coln D, Votteler T (1981) Triad of anorectal, sacral, and presacral anomalies. AJR Am J Roentgenol 137(2):395–398
de Jong EM, Felix JF, de Klein A, Tibboel D (2010) Etiology of esophageal atresia and tracheoesophageal fistula: “mind the gap”. Curr Gastroenterol Rep 12:215–222
deVries PA, Peña A (1982) Posterior sagittal anorectoplasty. J Pediatr Surg 17(5):638–643
Di Giacomo V, Trinci M, Van der Byl G, Catania VD, Calisti A, Miele V (2015) Ultrasound in newborns and children suffering from nontraumatic acute abdominal pain: imaging with clinical and surgical correlation. J Ultrasound 18:385–393. doi:10.1007/s40477-014-0087-4. Epub 2014 Apr 9
Di Gianantonio E, Schaefer C, Matroiacovo PP et al (2001) Adverse effects of prenatal methimazole exposure. Teratology 64:262–266
El-Gohary Y, Gittes GK, Tovar JA (2010) Congenital anomalies of the esophagus. Semin Pediatr Surg 19:186–193
Erskine JM (1970) Colonic stenosis in the newborn: the possible thromboembolic etiology of intestinal stenosis and atresia. J Pediatr Surg 5:321–333
Escobar MA, Ladd AP, Grosfeld JL, West KW, Rescorla FJ, Scherer LR 3rd et al (2004) Duodenal atresia and stenosis: long-term follow-up over 30 years. J Pediatr Surg 39(6):867–871
Etensel B, Temir G, Karkiner A et al (2005) Atresia of the colon. J Pediatr Surg 40:1258–1268
Felix JF, Steegers-Theunissen RP, de Walle HE, de Klein A, Torfs CP, Tibboel D (2007) Esophageal atresia and tracheo-esophageal fistula in children of women exposed to diethylstilbestrol in utero. Am J Obstet Gynecol 197:e31–e35
Free EA, Gerald B (1968) Duodenal obstruction in the newborn due to annular pancreas. Am J Roentgenol 103(2):321–325
Frischer JS, Azizkhan RG (2012) Jejunoileal atresia and stenosis. In: Coran AG (ed) Pediatric surgery, 7th edn. Elsevier Saunders, Philadelphia
Gans SL (1970) Classification of anorectal anomalies: a critical analysis. J Pediatr Surg 5:511–513
Genevieve D, de Pontual L, Amiel J, Sarnacki S, Lyonnet S (2007) An overview of isolated and syndromic esophageal atresia. Clin Genet 71:392–399
Georgeson KE, Inge TH, Albanese CT (2000) Laparoscopically assisted anorectal pull-through for high imperforate anus: a new technique. J Pediatr Surg 35(6):927–930, discussion 930–931
Grosfeld JL, Ballantine TVN, Shoemaker R (1968) Operative management of intestinal atresia and stenosis based on pathologic findings. J Pediatr Surg 14:368–375
Grosfeld JL, Ballantine TV, Shoemaker R (1979) Operative management of intestinal atresia and stenosis based on pathologic findings. J Pediatr Surg 14(3):368–375
Gross GW, Wolfson PJ, Pena (1991) An augmented-pressure colostogram in imperforate anus with fistula. Pediatr Radiol 21(8):560–562
Gupta AK, Guglani B (2005) Imaging of congenital anomalies of the gastrointestinal tract. Indian J Pediatr 72:403–414
Haber HP (2009) Ultrasonography of imperforate anus in neonate: an approach correlated with current surgical concepts. Ultraschall Med 30(2):189–195
Haber HP, Seitz G, Warmann SW, Fuchs J (2007) Transperineal sonography for determination of the type of imperforate anus. AJR Am J Roentgenol 189(6):1525–1529
Hertzberg BS, Bowie JD (1990) Fetal gastrointestinal abnormalities. Radiol Clin N Am 28:101–114
Holland AJ, Fitzgerald DA (2010) Oesophageal atresia and trachea-esophageal fistula: current management strategies and complications. Pediatr Respir Rev 11:100–106, quiz 106–107
Holschneider A, Hutson J, Peña A et al (2005) Preliminary report on the international conference for the development of standards for the treatment of anorectal malformations. J Pediatr Surg 40(10):1521–1526
Houben CH, Curry JI (2008) Current status of prenatal diagnosis, operative management and outcome of esophageal atresia/tracheo-esophageal fistula. Prenat Diagn 28:667–675
Ioannides AS, Copp AJ (2009) Embryology of esophageal atresia. Semin Pediatr Surg 18:2–11
Juang D, Snyder CL (2012) Neonatal bowel obstruction. Surg Clin North Am 92(3):685–711
Karnak I, Ciftci AO, Senocak ME, Tanyel FC, Buyukpamukcu N (2001) Colonic atresia: surgical management and outcome. Pediatr Surg Int 17:631–635
Kuo MF, Tsai Y, Hsu WM, Chen RS, Tu YK, Wang HS (2007) Tethered spinal cord and VACTERL association. J Neurosurg 106(3 Suppl):201–204
Lahdes-Vasama TT, Sihvonen R, Iber T (2009) Perforation of the upper and lower segments of atretic esophagus (type C) secondary to nasogastric tube insertion. Pediatr Surg Int 25:537–538
Lai ECS, Tompkins RK (1986) Heterotopic pancreas: review of 26-year experience. Am J Surg 151:697–700
Landes A, Shuckett B, Skarsgard E (1994) Non-fixation of the colon in colonic atresia: a new finding. Pediatr Radiol 24:167–169
Laya BF, Andres MM, Conception NDP, Dizon RH (2015) Patterns of microcolon: imaging strategies for diagnosis of lower intestinal obstruction in neonates. J Am Osteopat Coll Radiol 4(1):1–11
Levitt MA, Peña A (2007) Anorectal malformations. Orphanet J Rare Dis 2:33
Louw JH (1967) Resection and end-to-end anastomosis in the management of atresia and stenosis of the small bowel. Surgery 62:940–950
Martin LW, Zerella JT (1976) Jejunoileal atresia: a proposed classification. J Pediatr Surg 11:399–403
Materne R (2001) The duodenal wind sock sign. Radiology 218(3):749–750
Miele V, Di Giampietro I (2014) Diagnostic imaging in emergency. Salute Soc (2EN): 127–138. doi:10.3280/SES2014-002010EN
Miele V, Andreoli C, Grassi R (2006) The management of emergency radiology: key facts. Eur J Radiol 59:311–314. Epub 2006 Jun 27
Moore CCM (1989) Congenital gastric outlet obstruction. J Pediatr Surg 24:1241–1246
Morris G, Kennedy A Jr, Cochran W (2016) Small bowel congenital anomalies: a review and update. Curr Gastroenterol Rep 18:16. doi:10.1007/s11894-016-0490-4
Mortell AE, Azizkhan RG (2009) Esophageal atresia repair with thoracotomy: the Cincinnati contemporary experience. Semin Pediatr Surg 18:12–19
Nagpal R, Schnaufer L, Altschuler SM (1993) Duodenal web presenting with gastrointestinal bleeding in a seven-month-old infant. J Pediatr Gastroenterol Nutr 16(1):90–92
Narasimha Rao KL, Prasad GR, Katariya S, Yadav K, Mitra SK, Patak IC (1983) Prone cross table lateral view: an alternative to the invertogram in imperforate anus. AJR Am J Roentgenol 140:227–229
Niedzielski JK (2005) Invertography versus ultrasonography and distal colostography for the determination of bowel skin distance in children with anorectal malformations. Eur J Pediatr Surg 15(4):262–267
Nievelstein RA, Hartwig NG, Vermeij-Keers C, Valk J (1993) Embryonic development of the mammalian caudal neural tube. Teratology 48(1):21–31
Nievelstein RA, van der Werff JF, Verbeek FJ, Valk J, Vermeij-Keers C (1998) Normal and abnormal embryonic development of the anorectum in human embryos. Teratology 57(2):70–78
Nievelstein RA, Vos A, Valk J, Vermeij-Keers C (2002) Magnetic resonance imaging in children with anorectal malformations: embryologic implications. J Pediatr Surg 37(8):1138–1145
Nora JJ, Nora AH, Pervinche AG, Ingram JV, Fountain AK, Peterson MJ (1976) Congenital abnormalities and first trimester exposure to progestagen/oestrogen (letter). Lancet 1:313–314
Norton KI, Tenreiro R, Rabinowitz JG (1992) Sonographic demonstration of annular pancreas and a distal duodenal diaphragm in a newborn. Pediatr Radiol 22:66–67
Oppenheimer DA, Carroll BA, Shochat SJ (1983) Sonography of imperforate anus. Radiology 148:127–128
Pasto ME, Deiling JM, O’Hara AE, Rifkin MD, Goldberg BB (1984) Neonatal colonic atresia: ultrasound findings. Pediatr Radiol 14:346–348
Peña A, Hong A (2000) Advances in the management of anorectal malformations. Am J Surg 180(5):370–376
Pinheiro PFM, Simoes AC, Pereira RM (2012) Current knowledge on esophageal atresia. World J Gastroenterol 18(28):3662–3672
Pohlson EC, Hatch EI Jr, Glick PL, Tapper D (1988) Individualized management of colonic atresia. Am J Surg 155:690–692
Puri P, Fujimoto T (1988) New observations on the pathogenesis of multiple intestinal atresias. J Pediatr Surg 23:221–225
Ratan SK, Rattan KN, Pandey RM, Mittal A, Magu S, Sodhi PK (2004) Associated congenital anomalies in patients with anorectal malformations: a need for developing a uniform practical approach. J Pediatr Surg 39(11):1706–1711
Sarin YK (2000) Pyloric atresia associated with intestinal atresias. Indian Pediatr 37:205–207
Sarin YK, Sharma A, Sinha S, Deshpande VP (2012) Duodenal webs: an experience with 18 patients. J Neonatal Surg 1(2):20
Sauve RS, Leung AK (2003) Congenital varicella syndrome with colonic atresias. Clin Pediatr (Phila) 42:451
Schiller M, Aviad I, Freund H (1979) Congenital colonic atresia and stenosis. Am J Surg 138:721–724
Seitz G, Warmann SW, Schaefer J, Poets CF, Fuchs J (2006) Primary repair of esophageal atresia in extremely low birth weight infants: a single-center experience and review of the literature. Biol Neonate 90:247–251
Seo J, do Kim Y, Kim AR, Kim DY, Kim SC, Kim IK et al (2010) An 18-year experience of tracheoesophageal fistula and esophageal atresia. Korean J Pediatr 53:705–710
Sharma AK, Shekhawat NS, Agrawal LD, Chaturvedi V, Kothari SK, Goel D (2000) Esophageal atresia and tracheoesophageal fistula: a review of 25 years’ experience. Pediatr Surg Int 16:478–482
Shaw-Smith C (2010) Genetic factors in esophageal atresia, trachea-esophageal fistula and the VACTERL association: roles for FOXF1 and the 16q24.1 FOX transcription factor gene cluster, and review of the literature. Eur J Med Genet 53:6–13
Solomon BD (2011) VACTERL/VATER association. Orphanet J Rare Dis 6:56–67
Solomon BD, Raam MS, Pineda-Alvarez DE (2011) Analysis of genitourinary anomalies in patients with VACTERL (vertebral anomalies, anal atresia, cardiac malformations, tracheo-esophageal fistula, renal anomalies, limb abnormalities) association. Congenit Anom (Kyoto) 51(2):87–91
Son TN, Liem NT, Kien HH (2015) Laparoscopic simple oblique duodenoduodenostomy in management of congenital duodenal obstruction in children. J Laparoendosc Adv Surg Tech A 25(2):163–166
Spitz L (2007) Oesophageal atresia. Orphanet J Rare Dis 2:24
Stephens FD, Smith ED, Paoul NW (1988) Anorectal malformations in children: update 1988. Liss, New York
Strouse PJ (2008) Malrotation. Semin Roentgenol 43(1):7–14
Tao HA, Lin CC, Huang FY, Yeh ML, Shih SL (1987) Congenital atresia of the colon: a case report. Acta Pediatr Sin 28:120–122
van der Zee DC (2011) Laparoscopic repair of duodenal atresia: revisited. World J Surg 35(8):1781–1784
Van Winckel MAJM, Afschrift MB, Vande Walle JGJ (1994) Ultrasound diagnosis of a prepyloric diaphragm. J Clin Ultrasound 22:141–143
Wang J, Du L, Cai W, Pan W, Yan W (2014) Prolonged feeding difficulties after surgical correction on intestinal atresia: a 13-year experience. J Pediatr Surg 49(11):1593–1597
Watts AC, Sabharwal AJ, MacKinlay GA, Munro FD (2003) Congenital colonic atresia: should primary anastomosis always be the goal? Pediatr Surg Int 19:14–17
Winters WD, Weinberger E, Hatch EI (1992) Atresia of the colon in neonates: radiographic findings. AJR 159:1273–1276
Wong-Gibbons DL, Romitti PA, Sun L, Moore CA, Reefhuis J, Bell EM, Olshan AF (2008) Maternal periconceptional exposure to cigarette smoking and alcohol and esophageal atresia +/− trachea-esophageal fistula. Birth Defects Res A Clin Mol Teratol 82:776–784
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2016 Springer International Publishing Switzerland
About this chapter
Cite this chapter
Miele, V., Piccolo, C.L., Saracco, V., Napoletano, M., Trinci, M., Brunese, L. (2016). Intestinal Stenosis and Atresia. In: Miele, V., Trinci, M. (eds) Imaging Non-traumatic Abdominal Emergencies in Pediatric Patients. Springer, Cham. https://doi.org/10.1007/978-3-319-41866-7_1
Download citation
DOI: https://doi.org/10.1007/978-3-319-41866-7_1
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-41865-0
Online ISBN: 978-3-319-41866-7
eBook Packages: MedicineMedicine (R0)