Agenesis
of the Gallbladder
in Duodenal
Atresia: Two Case Reports
By John P. Coughlin, Fredrick E. Rector, and Michael D. Klein Detroit, Michigan 0 Agenesis of the gallbladder not associated with biliary atresia is quite rare. We wish to report two patients with gallbladder agenesis in association with duodenal atresia without biliary atresia. Liver biopsy in one patient was notable for an absence of both inflammation and ductal pathology. The unremarkable postoperative course included normal direct bilirubins and a HIDA scan that demonstrated very good bile excretion into the duodenum. Both children are doing quite well in follow-up. Copyright o 7992 by W. B. Saunders Company INDEX WORDS: biliary atresia.
Gallbladder
agenesis;
duodenal
atresia;
A
LTHOUGH associated with one in six cases of biliary atresia,l gallbladder agenesis is otherwise quite rare, having an estimated incidence of 1 case per 6,000 births.2 We wish to report two patients with agenesis of the gallbladder in association with duode_ nal atresia without biliary atresia. CASE REPORTS Case 1
A 33-week, 2,120-g boy was born to a 19-year-old gravida 1 nulliparous woman without significant family history whose pregnancy was complicated by substance abuse, diabetes mellitus, hydramnios, and the antenatal diagnosis of duodenal atresia. At birth, imperforate anus and hypospadias were noted. Sonography documented a ventricular septal defect, patent ductus arteriosus, and left hydronephrosis. Plain films were confirmatory of duodenal atresia. At operation a short (1.5cm) dilated proximal duodenum and a 1.5-cm atretic midduodenum were found and a duodenoduodenostomy was fashioned. A grossly normal common bile duct was traced from the hilus to the proximal duodenum and a normal liver was noted. A thorough search of the right upper quadrant failed to show a gallbladder, cystic duct, or remnant of either structure. A colostomy concluded the case. The postoperative total bilirubin of 4.7 mg/dL increased to 14.7 mg/dL by the 7th postoperative day before falling to 1.0 mg/dL at discharge (postop-
From the Depatiment of Pediatric Surgery, Children S Hospital of Michigan, Wayne State University School of Medicine, Detroit, MI. Presented at the 23rd Annual Meeting of the Canadian Association of Paediattic Surgeons, Quebec City, Quebec, September 19-22, 1991. Address reprint requests to Michael D. Klein, MD, Children’s Hospital of Michigan, 3901 Beaubien Blvd, Detroit, MI 48201. Copyright o 1992 by W.B. Saunders Company 0022-3468/92/2710-0012$03.00/O
1304
erative day 15). Conjugated bilirubin remained normal throughout. The infant is now 6 months old and in good health. Case
2
A 1,620-g, 31-week gestation boy was born to a 24-year-old gravida 1 nulliparous woman without significant medical history with an antenatal diagnosis of hydramnios and duodenal atresia. After confirmatory plain films, the infant was taken to the operating room where a l-cm atretic segment of duodenum was found immediately distal to the insertion of the common bile duct. A duodenoduodenostomy was fashioned. Again a thorough exploration failed to show a gallbladder, cystic duct, or remnants of either structure. An open liver biopsy was notable for the absence of both inflammation and ductal pathology. The total bilirubin peaked at 12.3 mg/dL on the 5th postoperative day before returning to normal on the 16th postoperative day. The direct bilirubin was never elevated. Two weeks postoperatively, a HIDA scan showed rapid liver uptake with prompt excretion into a dilated duodenum. The gallbladder was not visualized and there was no evidence of biliary atresia. The infant is now 5 months old and in good health. DISCUSSION
Defective formation of the caudal bud of the embryonic hepatic diverticulum or failure of its vacuolization remain as theories of gallbladder agenesis. Indeed, failure of vacuolization, also invoked as etiology of duodenal atresia, is attractive as a single explanation of the duodenal atresia and gallbladder agenesis seen in our two patients. Our failure to find even rudimentary gallbladder or cystic duct remnants, however, temper our enthusiasm of this potential etiology. It does seem reasonable to include these patients into the category of gallbladder atresia associated with anomalies at the duodenal region where and when the liver, biliary ducts and pancreas develop, as proposed by Heij.3 We conclude with the recommendation of a thorough right upper quadrant exploration, liver biopsy, and remote HIDA scan as complete components in the care of the newborn found with incidental agenesis of the gall bladder and duodenal atresia. REFERENCES 1. Gross RE: Congenital anomalies of the gallbladder. Arch Surg 32:131-162,1936 2. Bennion RS, Thompson JE Jr, Tompkins RK: Agenesis of the gallbladder without biliary atresia. Arch Surg 123:1257-1260, 1988 3. Heij HA: Agenesis of the gallbladder. Arch Surg 124:1469, 1989 (letter)
JournalofPediatric Surgery, Vol27, No 10 (October), 1992: p 1304
of the Gallbladder
in Duodenal
Atresia: Two Case Reports
By John P. Coughlin, Fredrick E. Rector, and Michael D. Klein Detroit, Michigan 0 Agenesis of the gallbladder not associated with biliary atresia is quite rare. We wish to report two patients with gallbladder agenesis in association with duodenal atresia without biliary atresia. Liver biopsy in one patient was notable for an absence of both inflammation and ductal pathology. The unremarkable postoperative course included normal direct bilirubins and a HIDA scan that demonstrated very good bile excretion into the duodenum. Both children are doing quite well in follow-up. Copyright o 7992 by W. B. Saunders Company INDEX WORDS: biliary atresia.
Gallbladder
agenesis;
duodenal
atresia;
A
LTHOUGH associated with one in six cases of biliary atresia,l gallbladder agenesis is otherwise quite rare, having an estimated incidence of 1 case per 6,000 births.2 We wish to report two patients with agenesis of the gallbladder in association with duode_ nal atresia without biliary atresia. CASE REPORTS Case 1
A 33-week, 2,120-g boy was born to a 19-year-old gravida 1 nulliparous woman without significant family history whose pregnancy was complicated by substance abuse, diabetes mellitus, hydramnios, and the antenatal diagnosis of duodenal atresia. At birth, imperforate anus and hypospadias were noted. Sonography documented a ventricular septal defect, patent ductus arteriosus, and left hydronephrosis. Plain films were confirmatory of duodenal atresia. At operation a short (1.5cm) dilated proximal duodenum and a 1.5-cm atretic midduodenum were found and a duodenoduodenostomy was fashioned. A grossly normal common bile duct was traced from the hilus to the proximal duodenum and a normal liver was noted. A thorough search of the right upper quadrant failed to show a gallbladder, cystic duct, or remnant of either structure. A colostomy concluded the case. The postoperative total bilirubin of 4.7 mg/dL increased to 14.7 mg/dL by the 7th postoperative day before falling to 1.0 mg/dL at discharge (postop-
From the Depatiment of Pediatric Surgery, Children S Hospital of Michigan, Wayne State University School of Medicine, Detroit, MI. Presented at the 23rd Annual Meeting of the Canadian Association of Paediattic Surgeons, Quebec City, Quebec, September 19-22, 1991. Address reprint requests to Michael D. Klein, MD, Children’s Hospital of Michigan, 3901 Beaubien Blvd, Detroit, MI 48201. Copyright o 1992 by W.B. Saunders Company 0022-3468/92/2710-0012$03.00/O
1304
erative day 15). Conjugated bilirubin remained normal throughout. The infant is now 6 months old and in good health. Case
2
A 1,620-g, 31-week gestation boy was born to a 24-year-old gravida 1 nulliparous woman without significant medical history with an antenatal diagnosis of hydramnios and duodenal atresia. After confirmatory plain films, the infant was taken to the operating room where a l-cm atretic segment of duodenum was found immediately distal to the insertion of the common bile duct. A duodenoduodenostomy was fashioned. Again a thorough exploration failed to show a gallbladder, cystic duct, or remnants of either structure. An open liver biopsy was notable for the absence of both inflammation and ductal pathology. The total bilirubin peaked at 12.3 mg/dL on the 5th postoperative day before returning to normal on the 16th postoperative day. The direct bilirubin was never elevated. Two weeks postoperatively, a HIDA scan showed rapid liver uptake with prompt excretion into a dilated duodenum. The gallbladder was not visualized and there was no evidence of biliary atresia. The infant is now 5 months old and in good health. DISCUSSION
Defective formation of the caudal bud of the embryonic hepatic diverticulum or failure of its vacuolization remain as theories of gallbladder agenesis. Indeed, failure of vacuolization, also invoked as etiology of duodenal atresia, is attractive as a single explanation of the duodenal atresia and gallbladder agenesis seen in our two patients. Our failure to find even rudimentary gallbladder or cystic duct remnants, however, temper our enthusiasm of this potential etiology. It does seem reasonable to include these patients into the category of gallbladder atresia associated with anomalies at the duodenal region where and when the liver, biliary ducts and pancreas develop, as proposed by Heij.3 We conclude with the recommendation of a thorough right upper quadrant exploration, liver biopsy, and remote HIDA scan as complete components in the care of the newborn found with incidental agenesis of the gall bladder and duodenal atresia. REFERENCES 1. Gross RE: Congenital anomalies of the gallbladder. Arch Surg 32:131-162,1936 2. Bennion RS, Thompson JE Jr, Tompkins RK: Agenesis of the gallbladder without biliary atresia. Arch Surg 123:1257-1260, 1988 3. Heij HA: Agenesis of the gallbladder. Arch Surg 124:1469, 1989 (letter)
JournalofPediatric Surgery, Vol27, No 10 (October), 1992: p 1304
