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1999-07-09-05 Answer of case of the week #4 © Imses www.TheFetus.net

Case #4:

July 9-23, 1999

Submitted by Hytham M. Imseis, MD,  MAHEC, Asheville, NC

Discussion Philippe Jeanty, MD, PhD and Hytham M Imseis, MD

Observations

These are 3 images of a 31-week fetus.

There was also a 2 minutes video clip (Download the video ( about 2 MB) that contained other findings not seen on these images.

I also mentioned that this was not a duodenal atresia and asked for differential diagnoses.

Some other observations that could be made from the video included:

  1. Distended esophagus
  2. A sandal gap
  3.  An amniotic fold

So what looks like a duodenal atresia but is not a duodenal atresia ?

Actually there are several differential diagnoses including:

Intrinsic duodenal obstruction

Extrinsic duodenal obstruction

  1. duodenal web9/diaphragms[1],[2],[3],[4],[5],[6],[7] (mucosal diaphragms, that can have some microperforation, complicating the presentation[8])
  2. duodenal stenosis[9],[10],[11] (the obstruction site is usually more distal in the duodenum)
  3. duodenal duplication2
  1. pyloric atresia[12],2
  2. Ladd’s band[13],[14] (obstruction is typically in the third portion of the duodenum)
  3. annular pancreas

 A double bubble is noted on transverse section of the fetal abdomen when there is a duodenal obstruction.  This appearance is the result of a distended stomach in the left abdomen and a distended duodenal bulb in the center of the abdomen, just to the right of the midline.  It is important to demonstrate continuity between the stomach and duodenum.

If the two fluid-filled cavities do not appear to communicate, then non-obstructive causes of the “double bubble” must be entertained.   Cystic structures in the left upper abdomen that can cause a “double bubble” appearance include

1.        Gastric duplication cyst

2.        Splenic cyst

3.        Renal cyst 

Cystic structures in the right upper abdomen that can cause a “double bubble” appearance include

1.        Choledochal cyst41 (most common cause of non-obstructive “double bubble”)

2.        Hepatic cyst

3.        Omental cyst

4.        Renal cyst

5.        Omental cyst

6.        Ovarian cyst

Prenatal findings

The first prenatal diagnosis I found was in 1982[15]. Same incidence in boys and girls.

  1. Polyhydramnios11,[16],[17]
  2. Low birth weight
  3. Preterm delivery[18],11.

Embryology

The pancreas normally develops during the 5th to 8th weeks of embryonic life.  It forms from two components that bud off the gut 180 degrees apart:  the larger dorsal bud (ultimately forms the body and tail of the pancreas) and the smaller ventral bud (forms the head of the pancreas).  As the duodenum grows and rotates, the ventral bud rotates toward the dorsal bud and fuses with it to form the pancreas. An annular pancreas most likely results from the rotation and growth of a bifid ventral bud that encircles the second part of the duodenum as it fuses with the dorsal bud.42

Postnatal presentation

Bilious vomiting, abdominal distension, dehydration and weight loss5,[19], but may remain silent until adulthood[20].  In fact, a recent case presented a patient first diagnosed at 79 years of age.43 

This baby had a 5-month history of epigastric pain after meals associated with early satiety and weight loss.  Diagnosis in adults can be made by endoscopic retrograde cholangiopancreatography (ERCP).  When radioopaque dye enters the pancreatic duct, it appears to encircle the second portion of the duodenum instead of entering directly.

Frequency

Although some authors report that annular pancreas are responsible for 1/3 of duodenal obstruction[21] (a surgical series), only a few hundred cases have been reported in the literature (280 by 1980-again a surgical series20).

Associated anomalies

  1. Trisomy 21[22],[23],10,[24]. In fact in a recent very large study[25] of almost 2900 Trisomy 21, annular pancreas ran second as a risk factor (risk ration of 430) for trisomy 21 (atrioventricular canal had a risk ratio = 1,009, and duodenal atresia a risk ratio = 265) (By the way, papers written by Torfs are usually very interesting, check them out!).
  2. Situs inversus1,5,[26],[27],[28], and aplenia-polysplenia syndrome27
  3. Communicating bronchopulmonary foregut malformation[29]
  4. Lipomyelomeningocele[30]
  5. Dorsal mesodermal sinus[31]
  6. Myofibromatosis[32]
  7. CHARGE association[33]
  8. Cornelia de Lange syndrome[34],[35]
  9. Congenital absence of the gallbladder[36] 

Etiology

Probably autosomal dominant in certain cases[37],[38],[39],[40],[41].

 Prognosis

Without associated anomalies, the prognosis tends to be good. Long term complications include: steatorrhea, diarrhea after fatty diet, malnutrition, failure to thrive, and lower concentration of stool trypsin19.

References

[1] Akel S, Halabi J, Shawis R Abdominal situs inversus with congenital duodenal stenosis: rare association. Eur J Pediatr Surg 1998 Feb;8(1):55-7

2 Bailey PV, Tracy TF Jr, Connors RH, Mooney DP, Lewis JE, Weber TR Congenital duodenal obstruction: a 32-year review. J Pediatr Surg 1993 Jan;28(1):92-5

3 Spigland N, Yazbeck S Complications associated with surgical treatment of congenital intrinsic duodenal obstruction. J Pediatr Surg 1990 Nov;25(11):1127-30

4 Aubrespy P, Derlon S, Seriat-Gautier B Congenital duodenal obstruction: a review of 82 cases. Prog Pediatr Surg 1978;11:109-24

5 Iuchtman M, Golan Y, Heldenberg D, Kessler FB Situs inversus abdominis in association with duodenal obstruction and internal hernia. Am J Perinatol 1993 May;10(3):255-7

6 Norton KI, Tenreiro R, Rabinowitz JG Sonographic demonstration of annular pancreas and a distal duodenal diaphragm in a newborn. Pediatr Radiol 1992;22(1):66-7

7 Spigland N, Yazbeck S Complications associated with surgical treatment of congenital intrinsic duodenal obstruction. J Pediatr Surg 1990 Nov;25(11):1127-30

8 Bickler SW, Harrison MW, Blank E, Campbell JR Microperforation of a duodenal diaphragm as a cause of paradoxical gas in congenital duodenal obstruction. J Pediatr Surg 1992 Jun;27(6):747-9

9 Grosfeld JL, Rescorla FJ Duodenal atresia and stenosis: reassessment of treatment and outcome based on antenatal diagnosis, pathologic variance, and long-term follow-up. World J Surg 1993 May-Jun;17(3):301-9

10 Bajaj RT Down syndrome with congenital duodenal stenosis and annular pancreas presenting as hematemesis: a case report. Indian Pediatr 1975 Apr;12(4):361-3

11 Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg 1998 May;133(5):490-6

12 Falade AG, Shonubi AM Pattern of congenital alimentary tract malformation in Ibadan, Nigeria. East Afr Med J 1997 Jun;74(6):385-8

13 Barrack SM, Kyambi JM, Ndungu J, Wachira N, Anangwe G, Safwat S Intestinal atresia and stenosis as seen and treated at Kenyatta National Hospital, Nairobi. East Afr Med J 1993 Sep;70(9):558-64

14 Tovar JA, Benavent M, Bachiller C, Diez-Pardo JA  Preduodenal portal vein . An Esp Pediatr 1978 Aug-Sep;11(8-9):565-74

15 Boomsma JH, Weemhoff RA, Polman HA Sonographic appearance of annular pancreas in utero. A case report. Diagn Imaging 1982;51(6):288-90

16 Jotz GP, Carnevale FC, Onofrio PL, Chaib E, Mello JB:  Annular pancreas. Report of a case and review of the literature . Arq Gastroenterol 1996 Jan-Mar;33(1):29-31

17 Merrill JR, Raffensperger JG Pediatric annular pancreas: twenty years" experience. J Pediatr Surg 1976 Dec;11(6):921-5

18 Soroka AA, Bairov AG Annular pancreas in children. Vestn Khir 1976 Aug;117(8):68-73

19Lin YT, Chang MH, Hsu HY, Lai HS, Chen CC A follow-up study of annular pancreas in infants and children. Chung Hua Min Kuo Hsiao Erh Ko I Hsueh Hui Tsa Chih 1998 Mar-Apr;39(2):89-93

20 Gernigon Y, Lozach P, Blain F, Bellet M, Griffe J  Annular pancreas in the adult . Nouv Presse Med 1980 Mar 12;9(17):1229-31

21 Hecker WC, Kraeft H, Stroh M   Pediatric problems arising from surgery of the pancreas in childhood . Monatsschr Kinderheilkd 1980 Dec;128(12):758-63

22 Aynaci FM, Orhan F, Celep F, Karaguzel Frequency of cardiovascular and gastrointestinal malformations, leukemia and hypothyroidism in children with Down syndrome in Trabzon, Turkey. Turk J Pediatr 1998 Jan-Mar;40(1):103-9

23 Clark JF, Hales E, Ma P, Rosser SB Duodenal atresia in utero in association with Down"s syndrome and annular pancreas. J Natl Med Assoc 1984 Feb;76(2):190-2

24 Milunsky A, Fisher JH Annular pancreas in Down"s syndrome. Lancet 1968 Sep 7;2(7567):575

25 Torfs CP, Christianson RE Anomalies in Down syndrome individuals in a large population-based registry. Am J Med Genet 1998 Jun 5;77(5):431-8

26 Adeyemi SD Combination of annular pancreas and partial situs inversus: a multiple organ malrotation syndrome associated with duodenal obstruction. J Pediatr Surg 1988 Feb;23(2):188-91

27 Toriello HV, Kokx N, Higgins JV, Hofman R, Waterman DF Sibs with the polyasplenia developmental field defect. Am J Med Genet Suppl 1986;2:31-6

28 Ruben GD, Templeton JM Jr, Ziegler MM Situs inversus: the complex inducing neonatal intestinal obstruction. J Pediatr Surg 1983 Dec;18(6):751-6

29 Sumner TE, Auringer ST, Cox TD A complex communicating bronchopulmonary foregut malformation: diagnostic imaging and pathogenesis. Pediatr Radiol 1997 Oct;27(10):799-801

30 Shian WJ, Chi CS, Wong TT: Lipomyelomeningocele: a 9-year review Chung Hua Min Kuo Hsiao Erh Ko I Hsueh Hui Tsa Chih 1994 Jan-Feb;35(1):57-62

31 Okten A, Demirci A, Saruhan H, Mocan H, Karaguzel G Dorsal mesodermal sinus associated with annular pancreas and meconium peritonitis. Pediatr Radiol 1994;24(4):302-3

32 Michel M, Ninane J, Claus D, Gosseye S, Wese FX, Moulin D Major malformations in a case of infantile myofibromatosis. Eur J Pediatr 1990 Jan;149(4):251-2

33 Lim SY Charge association. A case report and short annotation. J Singapore Paediatr Soc 1990;32(1-2):46-9

34 Maruiwa M, Nakamura Y, Motomura K, Murakami T, Kojiro M, Kato M, Morimatsu M, Fukuda S, Hashimoto T Cornelia de Lange syndrome associated with Wilms" tumour and infantile haemangioendothelioma of the liver: report of two autopsy cases. Virchows Arch A Pathol Anat Histopathol 1988;413(5):463-8

35 Wick MR, Simmons PS, Ludwig J, Kleinberg F Duodenal obstruction, annular pancreas, and horseshoe kidney in an infant with Cornelia de Lange syndrome. Minn Med 1982 Sep;65(9):539-41

36 Heij HA, Niessen GJ Annular pancreas associated with congenital absence of the gallbladder. J Pediatr Surg 1987 Nov;22(11):1033

37 Mitchell CE, Marshall DG, Reid WD Preampullary congenital duodenal obstruction in a father and son. J Pediatr Surg 1993 Dec;28(12):1582-3

38 Rogers JC, Harris DJ, Holder T Annular pancreas in a mother and daughter. Am J Med Genet 1993 Jan 1;45(1):116

39 Hendricks SK, Sybert VP Association of annular pancreas and duodenal obstruction--evidence for Mendelian inheritance? Clin Genet 1991 May;39(5):383-5

40 MacFadyen UM, Young ID Annular pancreas in mother and son. Am J Med Genet 1987 Aug;27(4):987-9

41 Montgomery RC, Poindexter MH, Hall GH, Leigh JE Report of a case of annular pancreas of the newborn in two consecutive siblings. Pediatrics 1971 Jul;48(1):148-50

42 Dewbury KC, Aluwihare MC, Birch SJ, et al.  Prenatal ultrsound demonstration of a choledochal cyst.   Br J Radiol 1980; 53:906

43 Moore KL.  The digestive system.  The developing human. Third edition. WB Saunders, 1982.  pp. 234-7.

44 Godil A, McCracken JD.  Annular pancreas:  Images in clinical medicine.  N Engl J Med 1997 Jun 19;336(25): 1794.


[1] Akel S, Halabi J, Shawis R Abdominal situs inversus with congenital duodenal stenosis: rare association. Eur J Pediatr Surg 1998 Feb;8(1):55-7

[2] Bailey PV, Tracy TF Jr, Connors RH, Mooney DP, Lewis JE, Weber TR Congenital duodenal obstruction: a 32-year review. J Pediatr Surg 1993 Jan;28(1):92-5

[3] Spigland N, Yazbeck S Complications associated with surgical treatment of congenital intrinsic duodenal obstruction. J Pediatr Surg 1990 Nov;25(11):1127-30

[4] Aubrespy P, Derlon S, Seriat-Gautier B Congenital duodenal obstruction: a review of 82 cases. Prog Pediatr Surg 1978;11:109-24

[5] Iuchtman M, Golan Y, Heldenberg D, Kessler FB Situs inversus abdominis in association with duodenal obstruction and internal hernia. Am J Perinatol 1993 May;10(3):255-7

[6] Norton KI, Tenreiro R, Rabinowitz JG Sonographic demonstration of annular pancreas and a distal duodenal diaphragm in a newborn. Pediatr Radiol 1992;22(1):66-7

[7] Spigland N, Yazbeck S Complications associated with surgical treatment of congenital intrinsic duodenal obstruction. J Pediatr Surg 1990 Nov;25(11):1127-30

[8] Bickler SW, Harrison MW, Blank E, Campbell JR Microperforation of a duodenal diaphragm as a cause of paradoxical gas in congenital duodenal obstruction. J Pediatr Surg 1992 Jun;27(6):747-9

[9] Grosfeld JL, Rescorla FJ Duodenal atresia and stenosis: reassessment of treatment and outcome based on antenatal diagnosis, pathologic variance, and long-term follow-up. World J Surg 1993 May-Jun;17(3):301-9

[10] Bajaj RT Down syndrome with congenital duodenal stenosis and annular pancreas presenting as hematemesis: a case report. Indian Pediatr 1975 Apr;12(4):361-3

[11] Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg 1998 May;133(5):490-6

[12] Falade AG, Shonubi AM Pattern of congenital alimentary tract malformation in Ibadan, Nigeria. East Afr Med J 1997 Jun;74(6):385-8

[13] Barrack SM, Kyambi JM, Ndungu J, Wachira N, Anangwe G, Safwat S Intestinal atresia and stenosis as seen and treated at Kenyatta National Hospital, Nairobi. East Afr Med J 1993 Sep;70(9):558-64

[14] Tovar JA, Benavent M, Bachiller C, Diez-Pardo JA  Preduodenal portal vein . An Esp Pediatr 1978 Aug-Sep;11(8-9):565-74

[15] Boomsma JH, Weemhoff RA, Polman HA Sonographic appearance of annular pancreas in utero. A case report. Diagn Imaging 1982;51(6):288-90

[16] Jotz GP, Carnevale FC, Onofrio PL, Chaib E, Mello JB:  Annular pancreas. Report of a case and review of the literature . Arq Gastroenterol 1996 Jan-Mar;33(1):29-31

[17] Merrill JR, Raffensperger JG Pediatric annular pancreas: twenty years" experience. J Pediatr Surg 1976 Dec;11(6):921-5

[18] Soroka AA, Bairov AG Annular pancreas in children. Vestn Khir 1976 Aug;117(8):68-73

[19] Lin YT, Chang MH, Hsu HY, Lai HS, Chen CC A follow-up study of annular pancreas in infants and children. Chung Hua Min Kuo Hsiao Erh Ko I Hsueh Hui Tsa Chih 1998 Mar-Apr;39(2):89-93

[20] Gernigon Y, Lozach P, Blain F, Bellet M, Griffe J  Annular pancreas in the adult . Nouv Presse Med 1980 Mar 12;9(17):1229-31

[21] Hecker WC, Kraeft H, Stroh M   Pediatric problems arising from surgery of the pancreas in childhood . Monatsschr Kinderheilkd 1980 Dec;128(12):758-63

[22] Aynaci FM, Orhan F, Celep F, Karaguzel Frequency of cardiovascular and gastrointestinal malformations, leukemia and hypothyroidism in children with Down syndrome in Trabzon, Turkey. Turk J Pediatr 1998 Jan-Mar;40(1):103-9

[23] Clark JF, Hales E, Ma P, Rosser SB Duodenal atresia in utero in association with Down"s syndrome and annular pancreas. J Natl Med Assoc 1984 Feb;76(2):190-2

[24] Milunsky A, Fisher JH Annular pancreas in Down"s syndrome. Lancet 1968 Sep 7;2(7567):575

[25] Torfs CP, Christianson RE Anomalies in Down syndrome individuals in a large population-based registry. Am J Med Genet 1998 Jun 5;77(5):431-8

[26] Adeyemi SD Combination of annular pancreas and partial situs inversus: a multiple organ malrotation syndrome associated with duodenal obstruction. J Pediatr Surg 1988 Feb;23(2):188-91

[27] Toriello HV, Kokx N, Higgins JV, Hofman R, Waterman DF Sibs with the polyasplenia developmental field defect. Am J Med Genet Suppl 1986;2:31-6

[28] Ruben GD, Templeton JM Jr, Ziegler MM Situs inversus: the complex inducing neonatal intestinal obstruction. J Pediatr Surg 1983 Dec;18(6):751-6

[29] Sumner TE, Auringer ST, Cox TD A complex communicating bronchopulmonary foregut malformation: diagnostic imaging and pathogenesis. Pediatr Radiol 1997 Oct;27(10):799-801

[30] Shian WJ, Chi CS, Wong TT: Lipomyelomeningocele: a 9-year review Chung Hua Min Kuo Hsiao Erh Ko I Hsueh Hui Tsa Chih 1994 Jan-Feb;35(1):57-62

[31] Okten A, Demirci A, Saruhan H, Mocan H, Karaguzel G Dorsal mesodermal sinus associated with annular pancreas and meconium peritonitis. Pediatr Radiol 1994;24(4):302-3

[32] Michel M, Ninane J, Claus D, Gosseye S, Wese FX, Moulin D Major malformations in a case of infantile myofibromatosis. Eur J Pediatr 1990 Jan;149(4):251-2

[33] Lim SY Charge association. A case report and short annotation. J Singapore Paediatr Soc 1990;32(1-2):46-9

[34] Maruiwa M, Nakamura Y, Motomura K, Murakami T, Kojiro M, Kato M, Morimatsu M, Fukuda S, Hashimoto T Cornelia de Lange syndrome associated with Wilms" tumour and infantile haemangioendothelioma of the liver: report of two autopsy cases. Virchows Arch A Pathol Anat Histopathol 1988;413(5):463-8

[35] Wick MR, Simmons PS, Ludwig J, Kleinberg F Duodenal obstruction, annular pancreas, and horseshoe kidney in an infant with Cornelia de Lange syndrome. Minn Med 1982 Sep;65(9):539-41

[36] Heij HA, Niessen GJ Annular pancreas associated with congenital absence of the gallbladder. J Pediatr Surg 1987 Nov;22(11):1033

[37] Mitchell CE, Marshall DG, Reid WD Preampullary congenital duodenal obstruction in a father and son. J Pediatr Surg 1993 Dec;28(12):1582-3

[38] Rogers JC, Harris DJ, Holder T Annular pancreas in a mother and daughter. Am J Med Genet 1993 Jan 1;45(1):116

[39] Hendricks SK, Sybert VP Association of annular pancreas and duodenal obstruction--evidence for Mendelian inheritance? Clin Genet 1991 May;39(5):383-5

[40] MacFadyen UM, Young ID Annular pancreas in mother and son. Am J Med Genet 1987 Aug;27(4):987-9

[41] Montgomery RC, Poindexter MH, Hall GH, Leigh JE Report of a case of annular pancreas of the newborn in two consecutive siblings. Pediatrics 1971 Jul;48(1):148-50

42 Moore, KL.  The digestive system.  The developing human, WB Saunders, 1982.  pp 235-7.

43Godil A, McCracken JD Annular pancreas: Images in clinical medicine.  N Engl J Med 1997 June 19;336(25):1794.

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