Anomalous Biliary Ducts Associated
With Duodenal Atresia
By Stuart J. Knechtle and Howard C. Filston Durham.
North Carolina
0 Duodenal atresia is rarely associated with anomalous biliary ducts that permit communication between the proximal and distal duodenal segments. Two such cases are presented herein and the literature is reviewed. Although the typical radiographic pattern of duodenal atresia is the “double bubble” sign with absence of distal bowel gas, air may be present in the distal bowel when anomalous bile ducts provide a conduit around the atretic segment. Contrast studies are generally not performed in the typical clinical and radiographic evaluation of duodenal atresia: however, an upper gastrointestinal series may be useful in defining the more complex anomaly. Clinical presentation may occur relatively late if the biliary communication is large enough to permit passage of some milk or formula. Care should be taken at surgery to avoid obstruction or injury to the anomalous bile ducts, and operative cholangiography may be useful to document continued bile duct patency following repair of the atresia. Theories of the etiology of this anomaly relate to interference with recanaliration of the duodenum by the process of hepaticopancreatic duct formation and persistence of the primitive “dual duct” stage. @ 1990 by W.B. Saunders Company. INDEX WORDS:
Duodenal atresia; bile duct anomalies.
D
UODENAL atresia is a common cause of congenital obstruction of the alimentary tract and has an estimated incidence between 1: 10,000 and 1:40,000 live births. Patients usually present within the first few days of life, and typical radiological features include the “double bubble” sign and absence of distal bowel gas. When upper intestinal obstruction presents later in the newborn period, the concern should be that acute obstruction has occurred, usually due to malrotation and volvulus. This report describes two cases of duodenal atresia that presented later than usual and were associated with anomalous biliary ducts that provided a communication between the proximal and distal duodenum. In one case, the anomalous biliary communication was diagnosed preoperatively by upper gastrointestinal (GI) contrast studies. Duodenal atresia associated with anomalous biliary ducts allowing communication between the proximal and distal duodenal segments has been described in 11 cases in the medical literature and hypothesized in a 12th.‘-’ In seven of the 11 confirmed cases, the outcome From the Pediatric Surgery Service, Department of Surgery, Duke University Medical Center, Durham, NC. Address reprint requests to Howard C. Filston. MD, University of Tennessee Medical Center, Box U-l 1. Knoxville, TN 37920. o 1990 by W. B. Saunders Company. 0022-3468/90/2512-0023$03.00/0
1266
fatal, and the biliary anomalies were diagnosed only at necropsy. In the most recently reported cases (4) and the two cases reported herein, this anomaly was successfully treated with survival. The purpose of this report is to describe the anatomy of this congenital defect, to correlate this with the embryology of the bile ducts and duodenum, and to recommend appropriate therapy. was
CASE
REPORTS
Case I
An I-day-old black girl, born at term with a birth weight of 3,295 g, had been tolerating formula feedings until she developed bilious emesis 1 day prior to admission. Plain abdominal radiographs showed a double bubble without distal bowel gas. A barium upper GI contrast study showed complete duodenal obstruction. At surgery a web atresia was encountered with a windsock configuration. A duodenotomy was made in the distal duodenum, which demonstrated a biliary ampulla. A second duodenotomy was made in the proximal duodenal segment, which demonstrated a second biliary ampulla in the medial wall of the duodenal segment (Fig 1). A catheter could be passed through a tract between the two ampullae. A duodenoduodenostomy was performed to bypass the web, thus avoiding potential injury to the biliary ducts. The patient did well and was discharged on the 12th postoperative day. She was asymptomatic and thriving at a 7-month follow-up visit. Case
2
A 23-day-old black girl, born at term at a birth weight of 3,755 g, was admitted after vomiting part of her feedings since 3 days of age. Emesis had become bilious at 2 weeks of age. The child had small amounts of normal-appearing stool. The child had lost 1 kg from birth weight, but was only moderately dehydrated. An abdominal radiograph demonstrated a mildly dilated stomach and proximal duodenum with copious bowel gas distally. Upper GI contrast studies demonstrated duodenal atresia with a dilated proximal duodenum and reflux of contrast into the biliary ductal system (Fig 2). The distal common bile duct communicated with both the proximal and distal duodenal segments. Due to the child’s poor nutritional state, she was treated with orogastric suction and 1 week of total parenteral nutrition prior to surgery. At surgery the patient had a type III duodenal atresia with a biliary communication into both the proximal and distal duodenal segments, documented by the presence of bile in both segments prior to repair (Fig 3). A malrotation was also present, but there were neither duodenal bands nor a volvulus. A duodenoduodenostomy was performed, as was gastrostomy tube placement and appendectomy. An operative cholangiogram performed through the gallbladder at the end of the procedure demonstrated patency of both biliary ampullae (Fig 4). The patient was discharged home on the 17th postoperative day and continues to do well 3 months postoperatively. DISCUSSION
Anomalous bile duct anatomy associated with duodenal atresia was first described by Karpa in 1906.’ A
Journal of Pediatric Surgery, Vol 25, No 12 (December), 1990: pp 1266-1269
ANOMALOUS
BILIARY
DUCTS
1267
Common bile duct
Distal duodenum Fig 1. Case 1 involved Type I duodenal atresia with biliary ampullae proximal and distal to the web.
child who died at 4 days of age with duodenal atresia was found at necropsy to have type III duodenal atresia (complete disconnection of the proximal and distal duodenum without intervening mesentery) with bile ducts communicating with both the proximal and distal duodenal segments. Katz, in 1930: described a male infant who died at 7 days of age and at necropsy also had a type III duodenal atresia with separate bile duct orifices in the proximal and distal duodenum. No associated anomalies were described with either of these cases. Four cases of duodenal atresia associated with bile duct communication to the proximal and distal duodenum have been reported in the radiological literature. Astley3 reported three cases in which the diagnosis of
Fig 2. Upper 01 series in case 2 demonstrated proximal duodenal obstruction. reflux of barium into the bile ducts (arrow). and passage of some barium into distal duodenum.
Distal duodenum Fig 3. At surgery (case 2) Type III duodenal atresia was encountered with bile in both proximal end distal duodenal segments.
the biliary anomaly was made at necropsy. One child with Down’s syndrome and a high ventricular septal defect was not treated surgically. In a second case duodenal atresia was treated surgically, but the child died of a subdural hematoma from birth trauma. A small amount of pancreas encircled the obstruction. The third case reported by Astley was in a child with multiple congenital anomalies, including esophageal atresia that was treated surgically on the first day of life, but the patient died 2 days later. At necropsy, duodenal atresia was also found with bile ducts communicating to the proximal and distal duodenal segments. Kassner et al, in 1972,4 described two children with gas noted in the bowel on abdominal radiograph beyond a seemingly complete duodenal obstruction. One child underwent repair of a Bochdalek hernia and was subsequently noted to have duodenal atresia that was treated with duodenojejunostomy and gastrostomy on the 8th day of life. The child died on the 18th day of life and at autopsy was noted to have a type II duodenal
Fig 4. lntraoperathre cholangiogram (case 2) following duodeno duodenostomy demonstrates patent bile ducts to proximal and distal duodenum (arrows).
KNECHTLE AND FILSTON
1268
atresia and a bifurcated common bile duct that communicated with both the proximal and distal duodenal segments. Kassner et al reported a second case of duodenal atresia in which distal bowel gas was present on preoperative plain radiographs. Although they may have been present, anomalous biliary ducts were not confirmed radiographically or at surgery, and the child did well after duodenojejunostomy. Reid’ reported nine cases of congenital anomalies of the biliary tract. The ninth case describes a child born in 1936 who died at 4 days of age. Necropsy demonstrated a bifid common duct with the main duct going to the proximal duodenal segment and a smaller duct passing to the distal duodenum. The specimen is preserved in the museum of the Institute of Pathology at the Royal Alexandra Hospital for Children, Sydney, Australia. Jona and Belin6 described three infants with duodenal atresia associated with common bile ducts communicating with both proximal and distal duodenal segments. One child with type III duodenal atresia was initially treated with a decompressive gastrostomy and feeding jejunostomy at 5 days of age and was noted to have bile in both proximal and distal duodenal segments. When definitive surgery was performed at 4 months of age, the presence of aberrant ducts was confirmed. Two other infants were reported who had duodenal diaphragm and bifid biliary ducts. One of these children was noted preoperatively to pass barium into the distal small bowel. The other child was found at surgery to have bile in both the proximal and distal duodenal segments. The three cases are the first
reported survivors of duodenal atresia associated with the more unusual biliary anomaly. Raine and Noblett7 reported a patient with multiple cardiac anomalies who underwent successful duodenoduodenostomy for duodenal atresia. The infant had a bilobed gallbladder as well as gas-filled biliary ducts, which communicated with the proximal and distal duodenal segments. The present case reports are unusual in that the clinical presentation occurred relatively late and the infants tolerated feedings for some time before the diagnosis was made, probably due to the ability of air and some food to enter the distal bowel via the biliary communications. A feature common to most of the reported cases and to one of the present cases was the presence of gas in bowel distal to the obstruction, a finding usually associated with acute obstruction by Ladd’s bands or a volvulus in the presence of malrotation. Although the association of anomalous biliary ducts with duodenal atresia is rare, if distal bowel gas is present or the child has inexplicably tolerated fluids for a period of time after birth, a search should be made radiographically or at surgery to identify the biliary anatomy, and care should be taken to avoid surgical ligation or obstruction of the bile ducts. This is true even when malrotation is also present, as in case 2. Incomplete recanalization of the duodenum has generally been considered the cause of duodenal atresia and stenosis since Tandler* observed that the fetal duodenum is obliterated during the second month of gestation and later regains its patency by a process of vacuolization. Boyden et al9 found that duodenal
Table 1. Duodenal Atresia With Biliary Communication Between Proximal and Distal Duodenal Segments
study
sex
Age at Diagnosis (d)
Associated Anomalies
Distal Bowel
Surgically
Gas on X-Ray
Treated
Outcome
4
_
M
7
-
Death
Astle$
F
18
Down’s syndrome, VSD
+
-
Death
A&y3
F
2
Annular pancreas
+
+
Death
Astley’
F
3
Esophageal atresia, imperforate anus,
+
+
Death
+
+
Death
_
Death
+
Survival
Karpa’ Katz’
Death
malrotation, aorticopulmonary window, left renal agensis Kassner et al4
M
8
Diaphragmatic hernia, malrotation, hyperplastic right kidney, accessory adrenal tissue
2
Reid’ Jona and Belins
M
5
Malrotation
-
Malrotation
+
Jona and 8elin6
F
1
Jona and Belir?
M
19
Raine and Noblett’
M
4
Hypoplastic right ventricle, pulmonary
+
Survival
+
Survival
+
+
Survival
-
+
Survival
+
+
Survival
stenosis, VSD, tricuspid atresia, bilobed gallbladder Case 1 (this report)
F
8
Case 2 (this report)
F
23
Abbreviations: VSD, ventricular septal defect: +, present: -,
Malrotation absent.
ANOMALOUS
BILIARY DUCTS
1269
atresia occurred at the level of the ampulla of Vater in 83% of cases. In the remaining 17% the obstruction was distal to this point. In their description of the embryology of congenital duodenal obstruction, Boyden et al hypothesized that there was a relationship between the anomalous bile ducts and duodenal atresia in these cases.g They suggested that “delayed coalescence of vacuoles to form the two orifices of the bile duct took precedence over vacuolization of adjacent portions.” They further hypothesized that duodenal atresia occurred most commonly at the region of the ampulla of Vater because this region remains narrow during development and because vacuolization of parallel bile ducts may take priority over duodenal recanalization. They suggested that a local traffic jam caused
by the entry of the hepaticopancreatic and accessory pancreatic ducts hindered recanalization at this point in the duodenum. Persistence of the primitive branches of the hepatopancreatic ducts, as documented in the 13 cases summarized herein, lends support to this hypothesis (Table 1). Gourevitch” suggested that in infants with duodenal atresia, the band of tissue that frequently joins the proximal and distal duodenal segments represents a biliary channel rather than atretic duodenum. In 21 of 27 autopsy specimens studied, this band of tissue had a patent lumen with characteristics of biliary ducts. This finding is consistent with Boyden’s observations and suggests that the biliary anomaly described herein may be far more common than previously observed.”
REFERENCES 1. Karpa P: Zwei Falle von Dunndarmatresie. Virchows Arch 185:208-226, 1906 2. Katz K: Atresie des duodenums mit verdoppelung des ductus choledochus und pancreaticus. Virchows Arch Path01 Anat Physiol 2781290-294, 1930 3. Astley R: Duodenal atresia with gas below the obstruction. Br J Radio1 42:351-353, 1969 4. Kassner EG, Sutton AL, De Grott TJ: Bile duct anomalies associated with duodenal atresia; paradoxical presence of small bowel gas. AJR Rad Ther Nucl Med 116:577-583,1972 5. Reid IS: Biliary tract abnormalities associated with duodenal atresia. Arch Dis Child 48:952-957, 1973 6. Jona JZ, Belin RP: Duodenal anomalies and the ampulla of Vater. Surg Gynecol Obstet 143:565-569, 1976
7. Raine PAM, Noblett HR: Duodenal atresia with biliary anomalies and unusual gas pattern. J Pediatr Surg 12:763-765, 1977 8. Tandler J: Zur Entwicklungsgeschichte des menschlicken: Duodenums in fruhen embryonalstadium. Morph Jahrb 29:187-216, 1900 9. Boyden EA, Cope JG, Bill AH Jr: Anatomy and embryology of congenital intrinsic obstruction of the duodenum. Am J Surg 114:190-202, 1967 10. Gourevitch A: Duodenal atresia in the newborn. Ann R Co11 Surg Engl48:141-158.1974 11. Boyden EA: The problem of the double ductus choledochus (an interpretation of an accessory bile duct found attached to the pars superior of the duodenum). Anat Ret 55:71-93, 1932
With Duodenal Atresia
By Stuart J. Knechtle and Howard C. Filston Durham.
North Carolina
0 Duodenal atresia is rarely associated with anomalous biliary ducts that permit communication between the proximal and distal duodenal segments. Two such cases are presented herein and the literature is reviewed. Although the typical radiographic pattern of duodenal atresia is the “double bubble” sign with absence of distal bowel gas, air may be present in the distal bowel when anomalous bile ducts provide a conduit around the atretic segment. Contrast studies are generally not performed in the typical clinical and radiographic evaluation of duodenal atresia: however, an upper gastrointestinal series may be useful in defining the more complex anomaly. Clinical presentation may occur relatively late if the biliary communication is large enough to permit passage of some milk or formula. Care should be taken at surgery to avoid obstruction or injury to the anomalous bile ducts, and operative cholangiography may be useful to document continued bile duct patency following repair of the atresia. Theories of the etiology of this anomaly relate to interference with recanaliration of the duodenum by the process of hepaticopancreatic duct formation and persistence of the primitive “dual duct” stage. @ 1990 by W.B. Saunders Company. INDEX WORDS:
Duodenal atresia; bile duct anomalies.
D
UODENAL atresia is a common cause of congenital obstruction of the alimentary tract and has an estimated incidence between 1: 10,000 and 1:40,000 live births. Patients usually present within the first few days of life, and typical radiological features include the “double bubble” sign and absence of distal bowel gas. When upper intestinal obstruction presents later in the newborn period, the concern should be that acute obstruction has occurred, usually due to malrotation and volvulus. This report describes two cases of duodenal atresia that presented later than usual and were associated with anomalous biliary ducts that provided a communication between the proximal and distal duodenum. In one case, the anomalous biliary communication was diagnosed preoperatively by upper gastrointestinal (GI) contrast studies. Duodenal atresia associated with anomalous biliary ducts allowing communication between the proximal and distal duodenal segments has been described in 11 cases in the medical literature and hypothesized in a 12th.‘-’ In seven of the 11 confirmed cases, the outcome From the Pediatric Surgery Service, Department of Surgery, Duke University Medical Center, Durham, NC. Address reprint requests to Howard C. Filston. MD, University of Tennessee Medical Center, Box U-l 1. Knoxville, TN 37920. o 1990 by W. B. Saunders Company. 0022-3468/90/2512-0023$03.00/0
1266
fatal, and the biliary anomalies were diagnosed only at necropsy. In the most recently reported cases (4) and the two cases reported herein, this anomaly was successfully treated with survival. The purpose of this report is to describe the anatomy of this congenital defect, to correlate this with the embryology of the bile ducts and duodenum, and to recommend appropriate therapy. was
CASE
REPORTS
Case I
An I-day-old black girl, born at term with a birth weight of 3,295 g, had been tolerating formula feedings until she developed bilious emesis 1 day prior to admission. Plain abdominal radiographs showed a double bubble without distal bowel gas. A barium upper GI contrast study showed complete duodenal obstruction. At surgery a web atresia was encountered with a windsock configuration. A duodenotomy was made in the distal duodenum, which demonstrated a biliary ampulla. A second duodenotomy was made in the proximal duodenal segment, which demonstrated a second biliary ampulla in the medial wall of the duodenal segment (Fig 1). A catheter could be passed through a tract between the two ampullae. A duodenoduodenostomy was performed to bypass the web, thus avoiding potential injury to the biliary ducts. The patient did well and was discharged on the 12th postoperative day. She was asymptomatic and thriving at a 7-month follow-up visit. Case
2
A 23-day-old black girl, born at term at a birth weight of 3,755 g, was admitted after vomiting part of her feedings since 3 days of age. Emesis had become bilious at 2 weeks of age. The child had small amounts of normal-appearing stool. The child had lost 1 kg from birth weight, but was only moderately dehydrated. An abdominal radiograph demonstrated a mildly dilated stomach and proximal duodenum with copious bowel gas distally. Upper GI contrast studies demonstrated duodenal atresia with a dilated proximal duodenum and reflux of contrast into the biliary ductal system (Fig 2). The distal common bile duct communicated with both the proximal and distal duodenal segments. Due to the child’s poor nutritional state, she was treated with orogastric suction and 1 week of total parenteral nutrition prior to surgery. At surgery the patient had a type III duodenal atresia with a biliary communication into both the proximal and distal duodenal segments, documented by the presence of bile in both segments prior to repair (Fig 3). A malrotation was also present, but there were neither duodenal bands nor a volvulus. A duodenoduodenostomy was performed, as was gastrostomy tube placement and appendectomy. An operative cholangiogram performed through the gallbladder at the end of the procedure demonstrated patency of both biliary ampullae (Fig 4). The patient was discharged home on the 17th postoperative day and continues to do well 3 months postoperatively. DISCUSSION
Anomalous bile duct anatomy associated with duodenal atresia was first described by Karpa in 1906.’ A
Journal of Pediatric Surgery, Vol 25, No 12 (December), 1990: pp 1266-1269
ANOMALOUS
BILIARY
DUCTS
1267
Common bile duct
Distal duodenum Fig 1. Case 1 involved Type I duodenal atresia with biliary ampullae proximal and distal to the web.
child who died at 4 days of age with duodenal atresia was found at necropsy to have type III duodenal atresia (complete disconnection of the proximal and distal duodenum without intervening mesentery) with bile ducts communicating with both the proximal and distal duodenal segments. Katz, in 1930: described a male infant who died at 7 days of age and at necropsy also had a type III duodenal atresia with separate bile duct orifices in the proximal and distal duodenum. No associated anomalies were described with either of these cases. Four cases of duodenal atresia associated with bile duct communication to the proximal and distal duodenum have been reported in the radiological literature. Astley3 reported three cases in which the diagnosis of
Fig 2. Upper 01 series in case 2 demonstrated proximal duodenal obstruction. reflux of barium into the bile ducts (arrow). and passage of some barium into distal duodenum.
Distal duodenum Fig 3. At surgery (case 2) Type III duodenal atresia was encountered with bile in both proximal end distal duodenal segments.
the biliary anomaly was made at necropsy. One child with Down’s syndrome and a high ventricular septal defect was not treated surgically. In a second case duodenal atresia was treated surgically, but the child died of a subdural hematoma from birth trauma. A small amount of pancreas encircled the obstruction. The third case reported by Astley was in a child with multiple congenital anomalies, including esophageal atresia that was treated surgically on the first day of life, but the patient died 2 days later. At necropsy, duodenal atresia was also found with bile ducts communicating to the proximal and distal duodenal segments. Kassner et al, in 1972,4 described two children with gas noted in the bowel on abdominal radiograph beyond a seemingly complete duodenal obstruction. One child underwent repair of a Bochdalek hernia and was subsequently noted to have duodenal atresia that was treated with duodenojejunostomy and gastrostomy on the 8th day of life. The child died on the 18th day of life and at autopsy was noted to have a type II duodenal
Fig 4. lntraoperathre cholangiogram (case 2) following duodeno duodenostomy demonstrates patent bile ducts to proximal and distal duodenum (arrows).
KNECHTLE AND FILSTON
1268
atresia and a bifurcated common bile duct that communicated with both the proximal and distal duodenal segments. Kassner et al reported a second case of duodenal atresia in which distal bowel gas was present on preoperative plain radiographs. Although they may have been present, anomalous biliary ducts were not confirmed radiographically or at surgery, and the child did well after duodenojejunostomy. Reid’ reported nine cases of congenital anomalies of the biliary tract. The ninth case describes a child born in 1936 who died at 4 days of age. Necropsy demonstrated a bifid common duct with the main duct going to the proximal duodenal segment and a smaller duct passing to the distal duodenum. The specimen is preserved in the museum of the Institute of Pathology at the Royal Alexandra Hospital for Children, Sydney, Australia. Jona and Belin6 described three infants with duodenal atresia associated with common bile ducts communicating with both proximal and distal duodenal segments. One child with type III duodenal atresia was initially treated with a decompressive gastrostomy and feeding jejunostomy at 5 days of age and was noted to have bile in both proximal and distal duodenal segments. When definitive surgery was performed at 4 months of age, the presence of aberrant ducts was confirmed. Two other infants were reported who had duodenal diaphragm and bifid biliary ducts. One of these children was noted preoperatively to pass barium into the distal small bowel. The other child was found at surgery to have bile in both the proximal and distal duodenal segments. The three cases are the first
reported survivors of duodenal atresia associated with the more unusual biliary anomaly. Raine and Noblett7 reported a patient with multiple cardiac anomalies who underwent successful duodenoduodenostomy for duodenal atresia. The infant had a bilobed gallbladder as well as gas-filled biliary ducts, which communicated with the proximal and distal duodenal segments. The present case reports are unusual in that the clinical presentation occurred relatively late and the infants tolerated feedings for some time before the diagnosis was made, probably due to the ability of air and some food to enter the distal bowel via the biliary communications. A feature common to most of the reported cases and to one of the present cases was the presence of gas in bowel distal to the obstruction, a finding usually associated with acute obstruction by Ladd’s bands or a volvulus in the presence of malrotation. Although the association of anomalous biliary ducts with duodenal atresia is rare, if distal bowel gas is present or the child has inexplicably tolerated fluids for a period of time after birth, a search should be made radiographically or at surgery to identify the biliary anatomy, and care should be taken to avoid surgical ligation or obstruction of the bile ducts. This is true even when malrotation is also present, as in case 2. Incomplete recanalization of the duodenum has generally been considered the cause of duodenal atresia and stenosis since Tandler* observed that the fetal duodenum is obliterated during the second month of gestation and later regains its patency by a process of vacuolization. Boyden et al9 found that duodenal
Table 1. Duodenal Atresia With Biliary Communication Between Proximal and Distal Duodenal Segments
study
sex
Age at Diagnosis (d)
Associated Anomalies
Distal Bowel
Surgically
Gas on X-Ray
Treated
Outcome
4
_
M
7
-
Death
Astle$
F
18
Down’s syndrome, VSD
+
-
Death
A&y3
F
2
Annular pancreas
+
+
Death
Astley’
F
3
Esophageal atresia, imperforate anus,
+
+
Death
+
+
Death
_
Death
+
Survival
Karpa’ Katz’
Death
malrotation, aorticopulmonary window, left renal agensis Kassner et al4
M
8
Diaphragmatic hernia, malrotation, hyperplastic right kidney, accessory adrenal tissue
2
Reid’ Jona and Belins
M
5
Malrotation
-
Malrotation
+
Jona and 8elin6
F
1
Jona and Belir?
M
19
Raine and Noblett’
M
4
Hypoplastic right ventricle, pulmonary
+
Survival
+
Survival
+
+
Survival
-
+
Survival
+
+
Survival
stenosis, VSD, tricuspid atresia, bilobed gallbladder Case 1 (this report)
F
8
Case 2 (this report)
F
23
Abbreviations: VSD, ventricular septal defect: +, present: -,
Malrotation absent.
ANOMALOUS
BILIARY DUCTS
1269
atresia occurred at the level of the ampulla of Vater in 83% of cases. In the remaining 17% the obstruction was distal to this point. In their description of the embryology of congenital duodenal obstruction, Boyden et al hypothesized that there was a relationship between the anomalous bile ducts and duodenal atresia in these cases.g They suggested that “delayed coalescence of vacuoles to form the two orifices of the bile duct took precedence over vacuolization of adjacent portions.” They further hypothesized that duodenal atresia occurred most commonly at the region of the ampulla of Vater because this region remains narrow during development and because vacuolization of parallel bile ducts may take priority over duodenal recanalization. They suggested that a local traffic jam caused
by the entry of the hepaticopancreatic and accessory pancreatic ducts hindered recanalization at this point in the duodenum. Persistence of the primitive branches of the hepatopancreatic ducts, as documented in the 13 cases summarized herein, lends support to this hypothesis (Table 1). Gourevitch” suggested that in infants with duodenal atresia, the band of tissue that frequently joins the proximal and distal duodenal segments represents a biliary channel rather than atretic duodenum. In 21 of 27 autopsy specimens studied, this band of tissue had a patent lumen with characteristics of biliary ducts. This finding is consistent with Boyden’s observations and suggests that the biliary anomaly described herein may be far more common than previously observed.”
REFERENCES 1. Karpa P: Zwei Falle von Dunndarmatresie. Virchows Arch 185:208-226, 1906 2. Katz K: Atresie des duodenums mit verdoppelung des ductus choledochus und pancreaticus. Virchows Arch Path01 Anat Physiol 2781290-294, 1930 3. Astley R: Duodenal atresia with gas below the obstruction. Br J Radio1 42:351-353, 1969 4. Kassner EG, Sutton AL, De Grott TJ: Bile duct anomalies associated with duodenal atresia; paradoxical presence of small bowel gas. AJR Rad Ther Nucl Med 116:577-583,1972 5. Reid IS: Biliary tract abnormalities associated with duodenal atresia. Arch Dis Child 48:952-957, 1973 6. Jona JZ, Belin RP: Duodenal anomalies and the ampulla of Vater. Surg Gynecol Obstet 143:565-569, 1976
7. Raine PAM, Noblett HR: Duodenal atresia with biliary anomalies and unusual gas pattern. J Pediatr Surg 12:763-765, 1977 8. Tandler J: Zur Entwicklungsgeschichte des menschlicken: Duodenums in fruhen embryonalstadium. Morph Jahrb 29:187-216, 1900 9. Boyden EA, Cope JG, Bill AH Jr: Anatomy and embryology of congenital intrinsic obstruction of the duodenum. Am J Surg 114:190-202, 1967 10. Gourevitch A: Duodenal atresia in the newborn. Ann R Co11 Surg Engl48:141-158.1974 11. Boyden EA: The problem of the double ductus choledochus (an interpretation of an accessory bile duct found attached to the pars superior of the duodenum). Anat Ret 55:71-93, 1932
