Coexistence of Meconium Ileus with Duodenal Atresia and Trisomy 21 in a Newborn: a Case Report

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Coexistence of Meconium Ileus with Duodenal Atresia and Trisomy 21 in a Newborn: a Case Report Journal of Perinatology (2014) 34, 875–876 © 2014 Nature America, Inc. All rights reserved 0743-8346/14 www.nature.com/jp PERINATAL/NEONATAL CASE PRESENTATION Coexistence of meconium ileus with duodenal atresia and trisomy 21 in a newborn: a case report OW Akinloye1, W Truong2, M Giacomantonio2, D Mateos3 and W El-Naggar1 The coexistence of duodenal atresia (DA) may mask the antenatal ultrasound findings of meconium ileus (MI) and delay its postnatal diagnosis. We report a rare case of MI in a newborn infant diagnosed antenatally to have trisomy 21 and DA. The diagnosis of MI was only established intraoperatively after the patient showed persistent signs of intestinal obstruction following the surgical repair of the DA. Journal of Perinatology (2014) 34, 875–876; doi:10.1038/jp.2014.124 INTRODUCTION of the contrast beyond the small intestine. A suction rectal biopsy Meconium ileus (MI) is characterized by obstruction of the bowel ruled out Hirchsprung disease. At exploratory laparotomy, the with inspissated meconium which, in most cases, represents the ileum was found to be impacted with copious amount of thick earliest manifestation of underlying cystic fibrosis.1 The diagnosis meconium. There was evidence of ischemic small bowel and of MI is suspected when echogenic bowel and dilated loops are decompressed microcolon. About 25 cm of small bowel was detected in ultrasound screening antenatally and/or when signs resected, and an ileostomy and mucous fistula were created. The of intestinal obstruction and characteristic X-ray findings are diagnosis of MI was confirmed by the finding of deep inspissated observed postnatally. The presence of a family history of cystic mucin, and luminal obstruction with meconium on histological fibrosis is suggestive.2–4 Duodenal atresia (DA) results from failure examination of the resected bowel. Sweat chloride tests on two of recanalization of the solid core of duodenum or from an occasions were insufficient but a molecular genetic analysis interruption of its blood supply early in intrauterine develop- confirmed the diagnosis of cystic fibrosis with the commonest ment.5,6 It accounts for 25 to 40% of all cases of intestinal atresia gene mutation of homozygous delta F508del. Fecal elastase test with an incidence of 1 in 10 000 live births. The double-bubble was consistent with pancreatic insufficiency. The infant later sign in prenatal ultrasound scans and postnatal abdominal X-rays succumbed to multiple intra-abdominal surgical complications characterizes the condition.7,8 As the potential for, and the and intractable infection at 4 months of age. chances of the occurrence of MI with DA are very unlikely, diagnosis may be unexpected or missed and proper management may be delayed. DISCUSSION MI occurs in up to 20% of patients with cystic fibrosis, accounting for an incidence of 0.8 in 10 000 live births.4 On the other hand, CASE the incidence of DA is 1 in 10 000 live births.7 The theoretical A full-term male infant was born to a 31-year-old gravida 2, para1 coexistence of both conditions is thus estimated to be as rare as 1 Caucasian mother whose first pregnancy resulted in a healthy full- in 125 million live births. From the pathophysiological point of term boy. Her antenatal ultrasound identified the double-bubble view, the occurrence of MI in the presence of DA may not be sign of DA. Subsequent amniocentesis confirmed the diagnosis of expected but is not impossible. Meconium is composed of trisomy 21. She developed severe polyhydraminos requiring mucous, bile and debris that is shed from the intestinal mucosa amnio-reduction at 38 weeks gestation. There was no echogenic in addition to the swallowed amniotic fluid during intrauterine bowel or dilated loops on repeat fetal ultrasounds. Family history life.9 As the presence of DA causes proximal obstruction for the was negative for cystic fibrosis. The baby was born by sponta- flow of amniotic fluid to the rest of the gut early in fetal life, the neous vaginal delivery at gestational age of 39 weeks and birth formation of meconium in the distal part of the bowel is expected weight of 2.93 kg. After birth, an abdominal X-ray confirmed to be reduced but not completely stopped. Our patient had the characteristic double-bubble sign of DA with no evidence of underlying cystic fibrosis, which, together with the low-fluid dilated bowel, ground glass opacification or calcifications bowel-formed meconium and the low gut motility resulting from (Figure 1). On his second day of age, the patient underwent the proximal DA obstruction, could have facilitated the develop- duodeno-duodenostomy. For the days following the repair, he ment of the viscid sticky meconium causing MI. failed to pass meconium and developed progressive abdominal The diagnosis of MI in our patient was delayed for different distension. An upper gastrointestinal contrast study on day 6 of reasons: antenatally, the proximal bowel obstruction by DA life did not reveal any anastomotic leak, but there was no passage prevented the development of more distal dilated loops, 1IWK Health Centre, Department of Pediatrics, Dalhousie University, Halifax, NS, Canada; 2Department of Surgery, Dalhousie University, Halifax, NS, Canada and 3Department of Respirology, Dalhousie University, Halifax, NS, Canada. Correspondence: Dr W El-Naggar, IWK Health Centre, Department of Pediatrics, Dalhousie University, 5850/5980 University Avenue, PO Box 9700, Halifax, NS, Canada B3K 6R8. E-mail: [email protected] Received 28 February 2014; revised 12 May 2014; accepted 14 May 2014 Coexistence of duodenal atresia OW Akinloye et al 876 with trisomy 21. Although the potential of having these com- binations may be rare, it is important to be considered in order to optimize the management and avoid the adverse consequences of delaying or missing the proper diagnosis. CONFLICT OF INTEREST The authors declare no conflict of interest. ACKNOWLEDGEMENTS Dr Olusegun Akinloye wrote the first draft of the manuscript. This project was conducted with no specific financial support. No honorarium, grant or other form of payment was given to anyone to produce this manuscript. REFERENCES 1 Park RW, Grand RJ. Gastrointestinal manifestation of cystic fibrosis: a review. Gastroenterology 1961; 81: 1143–1161. 2 Scotet V, Dugueperoux I, Audrezet MP, Audebert-Bellanger S, Muller M, Blayau M et al. Focus on cystic fibrosis and other disorders evidenced in fetuses with Figure 1. The preoperative abdominal X-ray of the patient showing sonographic finding of echogenic bowel: 16-year report from Brittany, France. Am the double-bubble sign of duodenal atresia. J Obstet Gynecol 2010; 203: 592.e1–6. 3 Bahado-Singh R, Morotti R, Copel JA, Mahoney MJ. Hyperechoic fetal bowel: the commonly detected by ultrasound in MI. The coexistence of cystic perinatal consequences. Prenat Diagn 1994; 14: 981–987. fibrosis with trisomy 21 was not expected as it is very rare with 4 Carlyle BE, Borowitz DS, Glick PL. A review of pathophysiology and management 10–13 of fetuses and neonates with meconium ileus for the pediatric surgeon. J Pediatr only a few cases reported in the literature. There has not been 47 – any previous report of the coexistence of MI with trisomy 21 or Surg 2012; (4): 772 781. 5O’Rahilly R, Muller F. Human Embryology and Teratology. Wiley-Liss: New York, with DA. Following DA repair, it is not unexpected to have delayed 1996 p 229. signs of intestinal obstruction for a few days. As our patient was a 6 Louw JH, Barnard CN. Congenital intestinal atresia; observations on its origin. boy with trisomy 21, the possibility of having Hirchsprung disease Lancet 1955; 269(6899): 1065–1067. was reasonably entertained and lead to more diagnostic delay. 7 Fonkalsrud EW, de Lorimer AA, Hays DM. Congenital atresia and stenosis of the In addition, there was no family history of cystic fibrosis and duodenum: a review compiled from the members of the surgical section. Am the postnatal abdominal X-rays did not show intra-abdominal Acad Pediatr 1969; 43:79–83. opacifications/calcification. Consequently, the diagnosis of MI was 8 Choudhry MS, Rahman N, Boyd P, Lakhoo K. Duodenal atresia: associated only possible when exploratory laparotomy was done after signs anomalies, prenatal diagnosis and outcome. Pediatr Surg Int 2009; 25(8): 727–730. 9 Rapoport S, Buchanan DJ. The composition of meconium; isolation of blood- of intestinal obstruction progressed and rectal biopsy revealed no fi evidence of Hirchsprung disease. When surgery was performed, group-speci c polysaccharides; abnormal compositions of meconium in meconium ileus. Science 1950; 112(2901): 150–153. there was already evidence of intestinal compromise and part of 10 Guy EL, Peckham DG, Brownlee KG, Conway SP, Lee TW. Cystic fibrosis co-existing the bowel had to be resected. with trisomy 21. J Cyst Fibros. 2010; 9(5): 330–331. Recent studies indicated that the long-term health outcomes of 11 Milunsky A. Cystic fibrosis and Down's syndrome. Pediatrics 1968; 42:501–504. patients with cystic fibrosis and MI are no worse than those of 12 Vetrella M, BartheImai W, Matsuda H. Down's syndrome and cystic fibrosis. 14–16 patients with cystic fibrosis only. However, the outcome of Pediatrics 1969; 43:905–906. infants with trisomy 21 and cystic fibrosis was described as being 13 Kruger C, Barmeier H, Sailer R, Harms D. Cystic fibrosis in Down's syndrome- poor.11–13 Unfortunately, our patient succumbed to postoperative diagnostic pitfalls and implications for the clinician. Arch Dis Child 1998; 78: 194. complications in the form of repeated intra-abdominal abscesses 14 Munck A, Gerardin M, Alberti C, Ajzenman C, Lebourgeois M, Aigrain Y et al. Clinical outcome of cystic fibrosis presenting with or without meconium ileus: a and intractable infection. It is not known whether the additional 41 – presence of MI in our patient with trisomy 21 and cystic fibrosis matched cohort study.
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