Double
Duodenal ByM.D.
Atresia/Stenosis:
A Report
of Four Cases
Stringer, R.J. Brereton, D.P. Drake, and V.M. Wright London, England
0 Four neonates with double duodenal atresia/stenosis are described. Preoperative plain radiographs in two patients demonstrated atypical appearances suggestive of complex pathology. Cystic dilatation of the second part of the duodenum was observed at laparotomy in two patients and in one of these the “cyst” was palpable preoperatively, causing diagnostic confusion. All four patients underwent successful surgery but one infant with Down’s syndrome subsequently died of congenital heart disease. Copyright o 1992 by W-B. Saunders Company INDEX WORDS: Duodenal atresia; multiple intestinal atresias.
D
URING THE past 10 years, 61 infants with duodenal atresia have been treated at Queen Elizabeth Hospital. Three patients had two atresias of the duodenum and a fourth, with duodenal atresia and stenosis treated at another institution, has been included in this report. Multiple congenital intrinsic duodenal obstructions are rare but must be recognized if potentially serious consequences are to be avoided.
of the left tunica vaginalis that required drainage. He went home at the age of 7 weeks but required readmission for investigation of persistent cholestatic jaundice. Liver biopsy specimen demonstrated nonspecific hepatitis and the jaundice, a complication of parenteral nutrition, resolved. At the age of 14 months he was making excellent progress but required dietary adjustment for cow’s milk protein intolerance. Case
2
A 2-day-old girl with Down’s syndrome, weighing 2.2 kg at birth, was referred because of bilious vomiting. She had features of a complete atrioventricular canal defect, confirmed by echocardiography. Plain radiographs suggested duodenal atresia with air in the distal duodenum and gallbladder, indicative of a bifid common bile duct (Fig 2).2 At laparotomy, two duodenal diaphragms were discovered (Fig l), a proximal preampullary one containing a central perforation and an imperforate distal one just beyond the ampulla of Vater. The former was treated by an anterolateral radial septotomy and a duodenoduodenostomy was constructed to bypass the latter. A gastrostomy and a transanastomotic tube were inserted. Four weeks postoperatively, her recovery was compli-
CASE REPORTS Case 1 A l-day-old boy, born at 33 weeks’ gestation and weighing 1.9 kg, was referred after an episode of vomiting complicated by right lower lobe aspiration pneumonia. Plain abdominal radiographs indicated duodenal atresia but the presence of flecks of calcification in the right upper quadrant suggested more complex pathology. Laparotomy showed a preampullary duodenal atresia (type 3),’ a membranous web in the fourth part of the duodenum and an intervening cystic segment containing bile (Fig 1). There was an ischemic perforation of the mesenteric border of the duodenal “cyst” just proximal to the imperforate distal web. A similar intrauterine perforation probably explained the calcification noted on the radiographs. In addition, the patient had intestinal malrotation and several type 2 midsmall bowel atresias. A duodenoduodenostomy was performed for the proximal duodenal atresia and, after resection of the duodenal web, an end-toend duodenojejunal anastomosis was fashioned. The midsmall bowel atresias were excised and continuity restored by one end-toend anastomosis. Ladd’s procedure and an appendicectomy were performed. Postoperative parenteral nutrition was gradually replaced by transanastomotic tube feeding but was interrupted by the development of necrotizing enterocolitis 3 weeks later. This settled with intensive medical treatment but was complicated by a pyocele
From the Queen Elizabeth Hospitalfor Children, London, England. Date accepted: November 12, 1990. Address reprint requests to M.D. Stringer, MS, FRCS, Department of I’aediattic Surgev, Institute of Child Health, Guilford St, London WCIN IEH, England. Copyright o 1992 by W. B. Saunders Company 0022-3468192/2705-0010$03.00/0 576
n3tiene
‘I
e
Patieno
Paeisnr
4
Fig 1. Diagrammatic representation of the four cases of double duodenal atresia/stenosis. The termination of the common bile duct is indicated by interrupted lines.
JournalofPediatric
Surgery, Vol27, No 5 (May), 1992: pp 576-580
DOUBLE DUODENAL
ATRESIA/STENOSIS
Fig 2. (A) Plain supine and (B) erect radiographs of infant (case 2) with double membranous gallbladder and the second part of the duodenum.
cated by necrotizing enterocolitis that resolved with medical treatment. She returned to her referring hospital but died at the age of 10 months due to congenital heart disease.
Case 3 A 31-week gestation girl, delivered by cesarean section and weighing 1.6 kg, developed copious bilious aspirates following nasogastric feeding. A mass was palpable in the right hypochondrium. An ultrasound scan indicated a 4 X 5 cm “cyst” anterior to the right kidney. Needle aspiration of the lesion produced pale green fluid. Plain abdominal radiographs indicated duodenal atresia (Fig 3A) and so a surgical opinion was sought. At laparotomy she was found to have two duodenal atresias (type 3) with cystic dilatation of the intervening part of the duodenum (Fig 1). Saline irrigation via the gallbladder filled the cyst and the gastric antrum, indicating dual biliary drainage. In addition, the third and fourth parts of the duodenum and the jejunum were absent, there were multiple type 2 atresias in the proximal ileum, and she had an “apple-peel” deformity of the distal ileum. Gastrointestinal continuity was restored by duodenoduodenostomy, resection of multiple proximal ileal atresias, and a duodenoileostomy (Fig 3B), which left an estimated 48 cm of small bowel. Gastrostomy and transanastomotic feeding tubes were inserted. Subsequent histological examination confirmed that the “cyst” wall was duodenal in origin. Postoperatively, she received total parenteral nutrition for 5 weeks until enteral feeding was fully established. Contrast studies demonstrated gastroesophageal reflux and poor peristalsis in the ileum but no mechanical obstruction (Fig 3B). Chromosome studies were normal. She went home after 2 months but required readmission for one episode of functional intestinal obstruction.
duodenal atresia/stenosis.
Note the air in the
Case 4 A l-day-old boy, born weighing 2.35 kg at 34 weeks’ gestation, was referred following an antenatal sonographic diagnosis of polyhydramnios and duodenal atresia. Plain abdominal radiographs confirmed a “double bubble” appearance. At laparotomy, two membranous duodenal atresias were identified. The first obstruction was at the junction of the second and third parts of the duodenum and the second was at the duodenojejunal flexure, situated in the midline. The common bile duct drained into both the dilated proximal duodenum and the isolated distal duodenal segment (Fig 1). There was no identifiable gallbladder. After tapering, the proximal duodenum was anastomosed end-toend to the upper jejunum. The third and fourth parts of the duodenum were excised, leaving a cuff of mucosa around the distal limb of the bifurcated terminal common bile duct and this was reimplanted into the proximal jejunum. Ladd’s procedure and an inversion appendicectomy were performed. The patient made an uncomplicated postoperative recovery and chromosome studies were normal. He remains well 5 years later. DISCUSSION
A review of the literature (Table 1) showed only 13 reported infants with double duodenal atresia/ stenosis 3-11 including the 5month-old infant reported by Bill and Pope with two perforate duodenal diaphragms.4 Double atresia of the duodenum occurred in 5% of neonates with duodenal atresia treated at this hospital during the past decade but has not been reported in many of the larger series of infants with duodenal atresia.‘*-l5 However, in his
578
Fig 3. (A) Preoperative plain radiograph of a neonate (case 3) with double duodenal atrasia and cystic dilatation of the second part of the duodenum. Gas was absent from the cyst. (6) Postoperative upper gastrointestinal contrast study of the same infant demonstrating the position of the cystic dilatation (C) of the second part of the duodenum after restoration of intestinal continuity.
review of 140 patients with duodenal atresia, Reid identified four infants with double intrinsic obstruction, giving an incidence of 3%.s All four infants died. Two of these patients had double membranous
STRINGER ET AL
atresias, similar to the present case 4 and to those recorded by other authors,4-7*9including one of the earliest descriptions of duodenal atresia.3 In case 4, the operation was complicated by the unusual anatomy of the terminal common bile duct and, in retrospect, membranoplasty may have been an alternative procedure. In contrast to double membranous atresias (type l), double atretic segments (types 2 and 3) appear to have been reported in only three patients,sJ1 none of whom survived. One of them had cystic dilatation of the duodenum similar to cases 1 and 3 in the present series. The common bile duct drained directly into this in each patient, underlining the importance of preserving this “cyst” when restoring gastrointestinal continuity if problems with biliary drainage are to be avoided.” This report of four patients emphasizes the fact that the classic double-bubble appearance on plain abdominal radiographs may signify a variety of duodenal anomalies, including both extrinsic and intrinsic complete or incomplete duodenal obstructions, which in turn may be single or multiple. In two of our patients there were radiographic features to suggest complex pathology-in the first patient there was evidence of calcification suggesting associated intrauterine perforation and in the second patient the pathology was evident in retrospect after close scrutiny of the atypical gas pattern. The importance of anomalous gas shadows indicating a bifid common bile duct has been stressed by other authors.2,r4 However, even in patients with “standard” radiographic appearances, the rare possibility of a double duodenal obstruction must be borne in mind and distal saline irrigation through a soft catheter passed into the distal duodenum is recommended. Present hypotheses suggest that duodenal atresia has a different etiology from atresias and stenoses in other parts of the small intestine. According to Tandler’s theory, I6 as modified by Boyden et al,” duodenal atresia results from a failure of complete recanalization of the embryonic duodenum after the phase of epithelial proliferation and subsequent vacuolization, which occurs between the 30th and 60th days of gestation. It is suggested that this developmental failure is most likely to occur in the vicinity of the ampulla of Vater and, thus, be associated with variations in the anatomy of the distal common bile duct. However, this simplistic theory has been challenged by Gourevitch,14 who argued that the atretic duodenal segment is derived from an abnormally situated bile duct, and by surgeons at the Alder Hey Hospital,*s who considered that the embryonic duodenum retains a lumen throughout its development.
579
DOUBLE DUODENAL ATRESIA/STENOSIS
Table 1. Collected Reports of Double Duodenal Atresia/Stenosis Reference
Year
Pathology
Associated
Outcome
Anomalies
-
Stillbirth
Boyd3
1845
2 duodenal diaphragms
Bill and Pope4
1954
2 duodenal diaphragms
Annular pancreas
Successful operation
Elliott et al5
1968
2 duodenal diaphragms
Annular pancreas
Successful operation
Intestinal malrotation Rowe et aI6 Richardson and Martin’
1968 1969
2 duodenal diaphragms
Intestinal malrotation
Successful operation
2 duodenal diaphragms
Polysplenia/situs inversus -
Successful operation Successful reoperation for
2 duodenal diaphragms
second diaphragm 1973
2 duodenal diaphragms
Esophageal atresia and TEF
Died postoperatively
2 duodenal diaphragms
Bilateral cleft lip and palate
Died preoperatively
2 duodenal atresias
Intestinal malrotation and
Died postoperatively
midgut volvulus Cystic fibrosis
Multiple diaphragms and
Died postoperatively
atresias Molenaar and Looyen
1974
3 duodenal diaphragms
RosselIot
1978
duodenal atresia + dia-
Elrereton et aI”
1980
-
Reoperation for 3rd diaphragm; survived
Trisomy 21lCHD
Successful reoperation for
2 duodenal atresias
Trisomy Zl/CHD
Died postoperatively
2 duodenal atresias
Small bowel atresias
second atresia
phragm Intestinal malrotation Present report
Successful operation
Intestinal malrotation 2 duodenal diaphragms
Trisomy 21/CHD
Successful operation
2 duodenal atresias
Small bowel atresias and
Successful operation
“apple peel” ileum 2 duodenal diaphragms
Intestinal malrotation
Successful operation
NOTE. Duodenal diaphragm includes perforate (duodenal stenosis) and imperforate (type I atresia) membranes and duodenal atresia refers to types 2 and 3 atresias.’ Abbreviations: TEF, tracheoesophageal
fistula; CHD, congenital heart disease.
In contrast to duodenal atresias, jejunoileal atresias are considered to result from an intrauterine vascular accident at a later stage in fetal life. Therefore, the unusual association between duodenal and jejunoileal atresias in two of the present patients is of particular interest, although this rare combination of anomalies has been previously reported.8J2Jg*20 On current hypotheses, the existence of two separate etiological processes has to be postulated to explain such a
combination and this would seem unlikely. It is of interest that duodenal atresia has been attributed in one neonate to prenatal midgut strangulation by an omphalocele, which suggests that atresia of the duodenum may have a vascular etiology in some patients.*l In both of the cases with combined multiple duodenal and intestinal atresias, there was no clinical or histological evidence to suggest a genetically determined malformation.22
REFERENCES 1. Schnaufer L: Duodenal atresia, stenosis and annular pancreas, in Welch KJ, Randolph JG, Ravitch MM, et al (eds): Pediatric Surgery (ed 4). Chicago, IL, Year Book, 1986, pp 829-837 2. Raine PAM, Noblett HR: Duodenal atresia with biliary anomalies and unusual gas pattern. J Pediatr Surg 12:763-765,1977 3. Boyd R: Description of a malformation of the duodenum. Lancet 1:648, 1845 4. Bill AH, Pope WM: Congenital duodenal diaphragm. Surgery 35:482-486,1954 5. Elliott GB, Kiiman MR, Elliott KA: Pancreatic annulus: A sign or a cause of duodenal obstruction? Can J Surg 11:357-364, 1968 6. Rowe MI, Buckner D, Clatworthy HW: Wind sock web of the duodenum. Am J Surg 116:444-449,1968 7. Richardson WR, Martin LW: Pitfalls in the surgical management of the incomplete duodenal diaphragm. J Pediatr Surg 4:303-312, 1969
8. Reid IS: The pattern of intrinsic duodenal obstructions. Aust N Z J Surg 42:349-352, 1973 9. Molenaar JC, Looyen SG: Wind sock web of the duodenum. Z Kinderchir 14:164-171,1974 10. Rossello PJ: Congenital duodenal atresia associated with a separate duodenal diaphragm. J Pediatr Surg 13:441-442, 1978 11. Brereton RJ, Cudmore RE, Bouton JM: Double atresia of the duodenum. Z Kinderchir 31:60-65, 1980 12. Young DG, Wilkinson AW: Abnormalities associated with neonatal duodenal obstruction. Surgery 63:832-836,1968 13. Nixon HH, Tawes R: Etiology and treatment of small intestinal atresia: Analysis of a series of 127jejunoileal atresias and comparison with 62 duodenal atresias. Surgery 69:41-51, 1971 14. Gourevitch A: Duodenal atresia in the newborn. Ann R Co11 Surg Engl48:141-158, 1969 15. Wesley JR, Mahour GH: Congenital intrinsic duodenal obstruction: A twenty-five year review. Surgery 82:716-720,1977
STRINGER ET AL
16. Tandler J: Zur Entwicklungsgeschichte des menschlichen Duodenum in fruhen Embryonal stadien. Morphologische Jahrbuch 29:187-216,1902 17. Boyden EA, Cope JG, Bill AH: Anatomy and embryology of congenital intrinsic obstruction of the duodenum. Am J Surg 114:190-201,1967 18. Irving IM: Duodenal atresia and stenosis: Annular pancreas, in Lister J, Irving IM (eds): Neonatal Surgery (ed 3). London, England, Butterworths, 1990, pp 424-429 19. Aitken J: Congenital intrinsic duodenal obstruction in in-
fancy. A series of 30 cases treated over a 6 year period. J Pediatr Surg 1:546-558,1966 20. Dickson JAS: Apple peel small bowel: An uncommon variant of duodenal and jejunal atresia. J Pediatr Surg 5:595-600, 1970 21. Shigemoto H, Horiya Y, Isomoto T, et al: Duodenal atresia secondary to intrauterine midgut strangulation by an omphalocele. J Pediatr Surg 17:420-421, 1982 22. Puri P, Fujimoto T: New observations on the pathogenesis of multiple intestinal atresias. J Pediatr Surg 23:221-225.1988
Duodenal ByM.D.
Atresia/Stenosis:
A Report
of Four Cases
Stringer, R.J. Brereton, D.P. Drake, and V.M. Wright London, England
0 Four neonates with double duodenal atresia/stenosis are described. Preoperative plain radiographs in two patients demonstrated atypical appearances suggestive of complex pathology. Cystic dilatation of the second part of the duodenum was observed at laparotomy in two patients and in one of these the “cyst” was palpable preoperatively, causing diagnostic confusion. All four patients underwent successful surgery but one infant with Down’s syndrome subsequently died of congenital heart disease. Copyright o 1992 by W-B. Saunders Company INDEX WORDS: Duodenal atresia; multiple intestinal atresias.
D
URING THE past 10 years, 61 infants with duodenal atresia have been treated at Queen Elizabeth Hospital. Three patients had two atresias of the duodenum and a fourth, with duodenal atresia and stenosis treated at another institution, has been included in this report. Multiple congenital intrinsic duodenal obstructions are rare but must be recognized if potentially serious consequences are to be avoided.
of the left tunica vaginalis that required drainage. He went home at the age of 7 weeks but required readmission for investigation of persistent cholestatic jaundice. Liver biopsy specimen demonstrated nonspecific hepatitis and the jaundice, a complication of parenteral nutrition, resolved. At the age of 14 months he was making excellent progress but required dietary adjustment for cow’s milk protein intolerance. Case
2
A 2-day-old girl with Down’s syndrome, weighing 2.2 kg at birth, was referred because of bilious vomiting. She had features of a complete atrioventricular canal defect, confirmed by echocardiography. Plain radiographs suggested duodenal atresia with air in the distal duodenum and gallbladder, indicative of a bifid common bile duct (Fig 2).2 At laparotomy, two duodenal diaphragms were discovered (Fig l), a proximal preampullary one containing a central perforation and an imperforate distal one just beyond the ampulla of Vater. The former was treated by an anterolateral radial septotomy and a duodenoduodenostomy was constructed to bypass the latter. A gastrostomy and a transanastomotic tube were inserted. Four weeks postoperatively, her recovery was compli-
CASE REPORTS Case 1 A l-day-old boy, born at 33 weeks’ gestation and weighing 1.9 kg, was referred after an episode of vomiting complicated by right lower lobe aspiration pneumonia. Plain abdominal radiographs indicated duodenal atresia but the presence of flecks of calcification in the right upper quadrant suggested more complex pathology. Laparotomy showed a preampullary duodenal atresia (type 3),’ a membranous web in the fourth part of the duodenum and an intervening cystic segment containing bile (Fig 1). There was an ischemic perforation of the mesenteric border of the duodenal “cyst” just proximal to the imperforate distal web. A similar intrauterine perforation probably explained the calcification noted on the radiographs. In addition, the patient had intestinal malrotation and several type 2 midsmall bowel atresias. A duodenoduodenostomy was performed for the proximal duodenal atresia and, after resection of the duodenal web, an end-toend duodenojejunal anastomosis was fashioned. The midsmall bowel atresias were excised and continuity restored by one end-toend anastomosis. Ladd’s procedure and an appendicectomy were performed. Postoperative parenteral nutrition was gradually replaced by transanastomotic tube feeding but was interrupted by the development of necrotizing enterocolitis 3 weeks later. This settled with intensive medical treatment but was complicated by a pyocele
From the Queen Elizabeth Hospitalfor Children, London, England. Date accepted: November 12, 1990. Address reprint requests to M.D. Stringer, MS, FRCS, Department of I’aediattic Surgev, Institute of Child Health, Guilford St, London WCIN IEH, England. Copyright o 1992 by W. B. Saunders Company 0022-3468192/2705-0010$03.00/0 576
n3tiene
‘I
e
Patieno
Paeisnr
4
Fig 1. Diagrammatic representation of the four cases of double duodenal atresia/stenosis. The termination of the common bile duct is indicated by interrupted lines.
JournalofPediatric
Surgery, Vol27, No 5 (May), 1992: pp 576-580
DOUBLE DUODENAL
ATRESIA/STENOSIS
Fig 2. (A) Plain supine and (B) erect radiographs of infant (case 2) with double membranous gallbladder and the second part of the duodenum.
cated by necrotizing enterocolitis that resolved with medical treatment. She returned to her referring hospital but died at the age of 10 months due to congenital heart disease.
Case 3 A 31-week gestation girl, delivered by cesarean section and weighing 1.6 kg, developed copious bilious aspirates following nasogastric feeding. A mass was palpable in the right hypochondrium. An ultrasound scan indicated a 4 X 5 cm “cyst” anterior to the right kidney. Needle aspiration of the lesion produced pale green fluid. Plain abdominal radiographs indicated duodenal atresia (Fig 3A) and so a surgical opinion was sought. At laparotomy she was found to have two duodenal atresias (type 3) with cystic dilatation of the intervening part of the duodenum (Fig 1). Saline irrigation via the gallbladder filled the cyst and the gastric antrum, indicating dual biliary drainage. In addition, the third and fourth parts of the duodenum and the jejunum were absent, there were multiple type 2 atresias in the proximal ileum, and she had an “apple-peel” deformity of the distal ileum. Gastrointestinal continuity was restored by duodenoduodenostomy, resection of multiple proximal ileal atresias, and a duodenoileostomy (Fig 3B), which left an estimated 48 cm of small bowel. Gastrostomy and transanastomotic feeding tubes were inserted. Subsequent histological examination confirmed that the “cyst” wall was duodenal in origin. Postoperatively, she received total parenteral nutrition for 5 weeks until enteral feeding was fully established. Contrast studies demonstrated gastroesophageal reflux and poor peristalsis in the ileum but no mechanical obstruction (Fig 3B). Chromosome studies were normal. She went home after 2 months but required readmission for one episode of functional intestinal obstruction.
duodenal atresia/stenosis.
Note the air in the
Case 4 A l-day-old boy, born weighing 2.35 kg at 34 weeks’ gestation, was referred following an antenatal sonographic diagnosis of polyhydramnios and duodenal atresia. Plain abdominal radiographs confirmed a “double bubble” appearance. At laparotomy, two membranous duodenal atresias were identified. The first obstruction was at the junction of the second and third parts of the duodenum and the second was at the duodenojejunal flexure, situated in the midline. The common bile duct drained into both the dilated proximal duodenum and the isolated distal duodenal segment (Fig 1). There was no identifiable gallbladder. After tapering, the proximal duodenum was anastomosed end-toend to the upper jejunum. The third and fourth parts of the duodenum were excised, leaving a cuff of mucosa around the distal limb of the bifurcated terminal common bile duct and this was reimplanted into the proximal jejunum. Ladd’s procedure and an inversion appendicectomy were performed. The patient made an uncomplicated postoperative recovery and chromosome studies were normal. He remains well 5 years later. DISCUSSION
A review of the literature (Table 1) showed only 13 reported infants with double duodenal atresia/ stenosis 3-11 including the 5month-old infant reported by Bill and Pope with two perforate duodenal diaphragms.4 Double atresia of the duodenum occurred in 5% of neonates with duodenal atresia treated at this hospital during the past decade but has not been reported in many of the larger series of infants with duodenal atresia.‘*-l5 However, in his
578
Fig 3. (A) Preoperative plain radiograph of a neonate (case 3) with double duodenal atrasia and cystic dilatation of the second part of the duodenum. Gas was absent from the cyst. (6) Postoperative upper gastrointestinal contrast study of the same infant demonstrating the position of the cystic dilatation (C) of the second part of the duodenum after restoration of intestinal continuity.
review of 140 patients with duodenal atresia, Reid identified four infants with double intrinsic obstruction, giving an incidence of 3%.s All four infants died. Two of these patients had double membranous
STRINGER ET AL
atresias, similar to the present case 4 and to those recorded by other authors,4-7*9including one of the earliest descriptions of duodenal atresia.3 In case 4, the operation was complicated by the unusual anatomy of the terminal common bile duct and, in retrospect, membranoplasty may have been an alternative procedure. In contrast to double membranous atresias (type l), double atretic segments (types 2 and 3) appear to have been reported in only three patients,sJ1 none of whom survived. One of them had cystic dilatation of the duodenum similar to cases 1 and 3 in the present series. The common bile duct drained directly into this in each patient, underlining the importance of preserving this “cyst” when restoring gastrointestinal continuity if problems with biliary drainage are to be avoided.” This report of four patients emphasizes the fact that the classic double-bubble appearance on plain abdominal radiographs may signify a variety of duodenal anomalies, including both extrinsic and intrinsic complete or incomplete duodenal obstructions, which in turn may be single or multiple. In two of our patients there were radiographic features to suggest complex pathology-in the first patient there was evidence of calcification suggesting associated intrauterine perforation and in the second patient the pathology was evident in retrospect after close scrutiny of the atypical gas pattern. The importance of anomalous gas shadows indicating a bifid common bile duct has been stressed by other authors.2,r4 However, even in patients with “standard” radiographic appearances, the rare possibility of a double duodenal obstruction must be borne in mind and distal saline irrigation through a soft catheter passed into the distal duodenum is recommended. Present hypotheses suggest that duodenal atresia has a different etiology from atresias and stenoses in other parts of the small intestine. According to Tandler’s theory, I6 as modified by Boyden et al,” duodenal atresia results from a failure of complete recanalization of the embryonic duodenum after the phase of epithelial proliferation and subsequent vacuolization, which occurs between the 30th and 60th days of gestation. It is suggested that this developmental failure is most likely to occur in the vicinity of the ampulla of Vater and, thus, be associated with variations in the anatomy of the distal common bile duct. However, this simplistic theory has been challenged by Gourevitch,14 who argued that the atretic duodenal segment is derived from an abnormally situated bile duct, and by surgeons at the Alder Hey Hospital,*s who considered that the embryonic duodenum retains a lumen throughout its development.
579
DOUBLE DUODENAL ATRESIA/STENOSIS
Table 1. Collected Reports of Double Duodenal Atresia/Stenosis Reference
Year
Pathology
Associated
Outcome
Anomalies
-
Stillbirth
Boyd3
1845
2 duodenal diaphragms
Bill and Pope4
1954
2 duodenal diaphragms
Annular pancreas
Successful operation
Elliott et al5
1968
2 duodenal diaphragms
Annular pancreas
Successful operation
Intestinal malrotation Rowe et aI6 Richardson and Martin’
1968 1969
2 duodenal diaphragms
Intestinal malrotation
Successful operation
2 duodenal diaphragms
Polysplenia/situs inversus -
Successful operation Successful reoperation for
2 duodenal diaphragms
second diaphragm 1973
2 duodenal diaphragms
Esophageal atresia and TEF
Died postoperatively
2 duodenal diaphragms
Bilateral cleft lip and palate
Died preoperatively
2 duodenal atresias
Intestinal malrotation and
Died postoperatively
midgut volvulus Cystic fibrosis
Multiple diaphragms and
Died postoperatively
atresias Molenaar and Looyen
1974
3 duodenal diaphragms
RosselIot
1978
duodenal atresia + dia-
Elrereton et aI”
1980
-
Reoperation for 3rd diaphragm; survived
Trisomy 21lCHD
Successful reoperation for
2 duodenal atresias
Trisomy Zl/CHD
Died postoperatively
2 duodenal atresias
Small bowel atresias
second atresia
phragm Intestinal malrotation Present report
Successful operation
Intestinal malrotation 2 duodenal diaphragms
Trisomy 21/CHD
Successful operation
2 duodenal atresias
Small bowel atresias and
Successful operation
“apple peel” ileum 2 duodenal diaphragms
Intestinal malrotation
Successful operation
NOTE. Duodenal diaphragm includes perforate (duodenal stenosis) and imperforate (type I atresia) membranes and duodenal atresia refers to types 2 and 3 atresias.’ Abbreviations: TEF, tracheoesophageal
fistula; CHD, congenital heart disease.
In contrast to duodenal atresias, jejunoileal atresias are considered to result from an intrauterine vascular accident at a later stage in fetal life. Therefore, the unusual association between duodenal and jejunoileal atresias in two of the present patients is of particular interest, although this rare combination of anomalies has been previously reported.8J2Jg*20 On current hypotheses, the existence of two separate etiological processes has to be postulated to explain such a
combination and this would seem unlikely. It is of interest that duodenal atresia has been attributed in one neonate to prenatal midgut strangulation by an omphalocele, which suggests that atresia of the duodenum may have a vascular etiology in some patients.*l In both of the cases with combined multiple duodenal and intestinal atresias, there was no clinical or histological evidence to suggest a genetically determined malformation.22
REFERENCES 1. Schnaufer L: Duodenal atresia, stenosis and annular pancreas, in Welch KJ, Randolph JG, Ravitch MM, et al (eds): Pediatric Surgery (ed 4). Chicago, IL, Year Book, 1986, pp 829-837 2. Raine PAM, Noblett HR: Duodenal atresia with biliary anomalies and unusual gas pattern. J Pediatr Surg 12:763-765,1977 3. Boyd R: Description of a malformation of the duodenum. Lancet 1:648, 1845 4. Bill AH, Pope WM: Congenital duodenal diaphragm. Surgery 35:482-486,1954 5. Elliott GB, Kiiman MR, Elliott KA: Pancreatic annulus: A sign or a cause of duodenal obstruction? Can J Surg 11:357-364, 1968 6. Rowe MI, Buckner D, Clatworthy HW: Wind sock web of the duodenum. Am J Surg 116:444-449,1968 7. Richardson WR, Martin LW: Pitfalls in the surgical management of the incomplete duodenal diaphragm. J Pediatr Surg 4:303-312, 1969
8. Reid IS: The pattern of intrinsic duodenal obstructions. Aust N Z J Surg 42:349-352, 1973 9. Molenaar JC, Looyen SG: Wind sock web of the duodenum. Z Kinderchir 14:164-171,1974 10. Rossello PJ: Congenital duodenal atresia associated with a separate duodenal diaphragm. J Pediatr Surg 13:441-442, 1978 11. Brereton RJ, Cudmore RE, Bouton JM: Double atresia of the duodenum. Z Kinderchir 31:60-65, 1980 12. Young DG, Wilkinson AW: Abnormalities associated with neonatal duodenal obstruction. Surgery 63:832-836,1968 13. Nixon HH, Tawes R: Etiology and treatment of small intestinal atresia: Analysis of a series of 127jejunoileal atresias and comparison with 62 duodenal atresias. Surgery 69:41-51, 1971 14. Gourevitch A: Duodenal atresia in the newborn. Ann R Co11 Surg Engl48:141-158, 1969 15. Wesley JR, Mahour GH: Congenital intrinsic duodenal obstruction: A twenty-five year review. Surgery 82:716-720,1977
STRINGER ET AL
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