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Congenital choledochal cyst: Reconstruction of the normal anatomy
Congenital Choledochal Cyst: Reconstruction of the Normal Anatomy By ALOISSCHXFU
AND
MARCELBETIEX
A
LTHOUGH CONGENITAL CHOLEDOCHAL CYST is generally considered to be a rare condition, the number of cases reported in the literature during the past two decades has almost reached 500.1-3 Growing awareness of the anomaly has led to earlier recognition than in the past and subsequently, the results following surgery have been better. DIAGNOSIS Diagnosis is suggested by the classic triad of pain, jaundice, and mass. In the newborn, however, vomiting because of partial intestinal obstruction and a palpable mass in the-right upper quadrant are more usual.4 Roentgenographic studies confirm the diagnosis. Plain films of the abdomen and contrast studies of the gastrointestinal tract reveal a radiolucent mass, displacing duodenum, colon, and liver. The intravenous pyelogram demonstrates a normal kidney through the mass and excludes a primary tumor originating from this organ. In spite of these findings the differential diagnosis between choledochal cyst on one hand and congenital cystic dilatation of the common duct, duplication of the duodenum, and mesenteric or primary hepatic cyst on the other is not always possible. Solid tumors of the liver, kidney, or retroperitoneum must also be considered. Laboratory examinations are not pathognomonic.
There is considerable controversy regarding the treatment of choledochal cysts as indicated by the multiplicity of surgical procedures described in the literature. Among these, three procedures are used most often: (1) Anastomosis of a portion of the intestinal tract to the choledochal cyst, (2) bypassing the choledochal cyst by cholecysto-enterostomy, and (3) removal of the choledochal cyst with anastomosis of the biliary tree to the intestinal tract. Anastomosis of the choledochal cyst or the gall bladder to the gastrointestinal tract can be hazardous and, although technically easier, they have a higher risk of complications and a higher m0rbidity.l From the Switzerland.
Department
ALOIS SCHXRLI, Switzerkznd. Surgery, 604
of Pediatric
M.D.: Department
Surgery, of
Children’s
Pediatric
MARCEL BETTEX, M.D.: Professor,
Hospital,
Surgery,
Surgeon-in-Chief,
University
Children’s
of (Berne,
Hospital,
Department
Berne,
of Pediatric
Children’s Hcxpital, Berne, Switzerland. JOURNAL OF PEDIATRICSURGERY,VOL. 3, No. 5
(OCTOBER),1968
605
COXGENITAL CHOLEDOCHAL CYST
Fig. 1.-A,
Dilated common bile duct at operation. B, Resected
In our cases anatomy
discussed
after removal
below
we have
therefore
choledochal
reconstructed
c!,st.
the normal
of the cyst. CASE REPORT
P.G., a 7-day-old girl was admitted to the Children’s Hospital of Berne with vomiting and progressive dehydration of 3 days’ duration. Delivery had been by cesarean section because of breech presentation. Birth weight 3880 Gm. Child vigorous, tolerated initial feedings. Normal meconium passed. Physical examination revealed a moderately dehydrated, but active newborn girl with 10 by 15 cm. elastic mass palpable in the right upper abdominal quadrant. Plain films of the abdomen showed a faint shadow below the liver. The right kidney was well visualized by intravenous pyelogram. Contrast studies of the gastrointestinal tract showed displacement of the pyloric area, duodenum and, ascending colon. Laboratory data: Hemoglobin 20 Gm. per cent, Hematocrit 63 per cent, WBC 9200 with normal distribution. Prothrombin time 58 per cent. Bleeding time: normal. Stool; color, green, massive amounts of neutral fats and saponifiable fractions. At laparotomy the liver was of normal size, color, and consistency. A large cystic mass, 15 I 12 x 12 cm., was attached to the under surface of the liver. The gallbladder was slightly filled, but very small. The entire ascending colon and a portion of the transverse colon covered the mass. An 8 cm. long distal portion of the common duct originated somewhat above the lower pole of the cyst, ending at the normal position in the duodenum. Cranially, the cystic dilatation began immediately distal to the fusion of the right and left hepatic duct (Fig. 1). The terminal portion of the common duct was catheterized transduodenally through the papilla of Vater with a Fr. 6 tube. The transitional zone between cyst and common duct was extremely stenotic. The choledochal cyst was dissected free from all covering tissues, and removed together with the gallbladder. A direct anastomosis was then achieved between the hepatic duct and the distal common duct, using interrupted 4-O chromic catgut suhrres. The Fr. 6 catheter was used as a splint and brought out through a stab
606
S-AND
wound io the retroperitoneum
BFTIEX
abdominal wall. Duodenum and ascending colon were sutured in their normal position which eased the tension on the anastomosis.
to
the
Histologic examination of the stenotic portion of the duct wall revealed partly hyalinized connective tissue with very few blood vessels, suggestiqg that the stenosis which led to the cystic dilatation of the common duct was caused by an angiectatic scar, probably the result of an early embryonic vascular accident. During the first 8 postoperative days drainage was 10 to 30 ml. and 60 to 100 ml. until the twelfth day when the drain was removed. The child tolerated feedings well and showed a good weight curve. Stools were dark. By the time of discharge liver enzymes were within normal limits. During a 3-year followup there has been no fever, vomiting or jaundice and liver function has remained normal. DISCUSSION
Uncorrected congenital cystic dilatation of the common duct leads to an almost certain fatal outcome. Twenty-nine out of 30 patients reviewed by Tsardakas and RobnetP died. Most of the cases reported in the literature have been treated with direct cystoduodenostomy. Ravitch and Snider5 consider the Roux-en-Y-anastomosis the procedure of choice for large cysts. Alonso-Lei et a1.l prefer removal of the cyst and anastomosis of the biliary tree to the second portion of the duodenum. Although mortality in patients with choledochal cyst has decreased from 50-80 percent before 1945 to 12 percent in recent years,a,F the morbidity remains high. Stones in the cyst have been reported 15 times, 4 times in the gallbladder, and malignant degeneration of the cyst has been recorded 4 times-l If the cyst remains large it may cause pressure upon the portal vein and lead to portal hypertension. The development of liver cirrhosis depends on the degree and duration of the obstruction.. In direct cystoenterostomies ascending cholangitis is common. It is generally believed that a Roux-en-Y-anastomosis of sufficient length prevents ascending infection better than other procedures.” In view of these complications it seems logical to perform a resection of the cyst and an anastomosis of the hepatic duct to the stump of the common duct, or if technically not feasible, with the duodenum. Gross,? in 1933, felt that this procedure was not advisable because of its higher mortality. The high incidence of complications induced us to undertake the reconstruction of a normal anatomy in our case. SUMMARY
A choledochal cyst was excised and the biliary tree reconstructed by an anastomosis between the common hepatic duct and the distal remnant of the common bile duct. When technically feasible this procedure seems preferable to other methods of surgical management. SUMMARIO Un
cyste
anastomose Quando
choledochal
esseva
inter le commun
illo es technicamente
excidite
ducto
IN INTERLINGUA e un arbore
hepatic
practicabile,
ibile a altere methodos de correction
chirurgic.
biliari
e le residuo
esseva
reconstruite
distal de1 commun
le procedimento
describite
pare
per
un
ducto biliari. esser prefer-
CONGENITAL
CHOLJZDOCHAL
607
CYST
REFERENCES 1. Alonso-Lei, F., Rever, W. B., and Pessagno, D. J.: Congenital choledochal cyst, with a report of 2, and an analysis of 94 cases. Surg. Gyn. Obstet. 10&l, 1959. 2. Tsardakeas, E., and Robnett, A. H.: Congenital cystic dilatation of the common bile duct. Arch. Surg. 72:311, 1957. 3. Attar, S., and Obeid, S.: Congenital cyst of the common bile duct. Ann. Surg. 142: 289, 1955. 4. Grob,
M., Stockmann,
M., and Bettex,
M.: Lehrbuch fur Kinderchirurgie. Stuttgart, Georg Thieme Verlag, 1957. 5. Ravitch, M. M., and Snider, G. B.: Congenital cystic dilatation of the common bile duct. Surgery 44:752, 1958. 6. Shields, A. B.: Congenital cystic dilatation of the common bile duct. Amer. J. Surg. 108:142,1964. 7. Gross, R. E.: Idiopathic dilatation of the common bile duct in children J. Pediat. 3: 730, 1933.
AND
MARCELBETIEX
A
LTHOUGH CONGENITAL CHOLEDOCHAL CYST is generally considered to be a rare condition, the number of cases reported in the literature during the past two decades has almost reached 500.1-3 Growing awareness of the anomaly has led to earlier recognition than in the past and subsequently, the results following surgery have been better. DIAGNOSIS Diagnosis is suggested by the classic triad of pain, jaundice, and mass. In the newborn, however, vomiting because of partial intestinal obstruction and a palpable mass in the-right upper quadrant are more usual.4 Roentgenographic studies confirm the diagnosis. Plain films of the abdomen and contrast studies of the gastrointestinal tract reveal a radiolucent mass, displacing duodenum, colon, and liver. The intravenous pyelogram demonstrates a normal kidney through the mass and excludes a primary tumor originating from this organ. In spite of these findings the differential diagnosis between choledochal cyst on one hand and congenital cystic dilatation of the common duct, duplication of the duodenum, and mesenteric or primary hepatic cyst on the other is not always possible. Solid tumors of the liver, kidney, or retroperitoneum must also be considered. Laboratory examinations are not pathognomonic.
There is considerable controversy regarding the treatment of choledochal cysts as indicated by the multiplicity of surgical procedures described in the literature. Among these, three procedures are used most often: (1) Anastomosis of a portion of the intestinal tract to the choledochal cyst, (2) bypassing the choledochal cyst by cholecysto-enterostomy, and (3) removal of the choledochal cyst with anastomosis of the biliary tree to the intestinal tract. Anastomosis of the choledochal cyst or the gall bladder to the gastrointestinal tract can be hazardous and, although technically easier, they have a higher risk of complications and a higher m0rbidity.l From the Switzerland.
Department
ALOIS SCHXRLI, Switzerkznd. Surgery, 604
of Pediatric
M.D.: Department
Surgery, of
Children’s
Pediatric
MARCEL BETTEX, M.D.: Professor,
Hospital,
Surgery,
Surgeon-in-Chief,
University
Children’s
of (Berne,
Hospital,
Department
Berne,
of Pediatric
Children’s Hcxpital, Berne, Switzerland. JOURNAL OF PEDIATRICSURGERY,VOL. 3, No. 5
(OCTOBER),1968
605
COXGENITAL CHOLEDOCHAL CYST
Fig. 1.-A,
Dilated common bile duct at operation. B, Resected
In our cases anatomy
discussed
after removal
below
we have
therefore
choledochal
reconstructed
c!,st.
the normal
of the cyst. CASE REPORT
P.G., a 7-day-old girl was admitted to the Children’s Hospital of Berne with vomiting and progressive dehydration of 3 days’ duration. Delivery had been by cesarean section because of breech presentation. Birth weight 3880 Gm. Child vigorous, tolerated initial feedings. Normal meconium passed. Physical examination revealed a moderately dehydrated, but active newborn girl with 10 by 15 cm. elastic mass palpable in the right upper abdominal quadrant. Plain films of the abdomen showed a faint shadow below the liver. The right kidney was well visualized by intravenous pyelogram. Contrast studies of the gastrointestinal tract showed displacement of the pyloric area, duodenum and, ascending colon. Laboratory data: Hemoglobin 20 Gm. per cent, Hematocrit 63 per cent, WBC 9200 with normal distribution. Prothrombin time 58 per cent. Bleeding time: normal. Stool; color, green, massive amounts of neutral fats and saponifiable fractions. At laparotomy the liver was of normal size, color, and consistency. A large cystic mass, 15 I 12 x 12 cm., was attached to the under surface of the liver. The gallbladder was slightly filled, but very small. The entire ascending colon and a portion of the transverse colon covered the mass. An 8 cm. long distal portion of the common duct originated somewhat above the lower pole of the cyst, ending at the normal position in the duodenum. Cranially, the cystic dilatation began immediately distal to the fusion of the right and left hepatic duct (Fig. 1). The terminal portion of the common duct was catheterized transduodenally through the papilla of Vater with a Fr. 6 tube. The transitional zone between cyst and common duct was extremely stenotic. The choledochal cyst was dissected free from all covering tissues, and removed together with the gallbladder. A direct anastomosis was then achieved between the hepatic duct and the distal common duct, using interrupted 4-O chromic catgut suhrres. The Fr. 6 catheter was used as a splint and brought out through a stab
606
S-AND
wound io the retroperitoneum
BFTIEX
abdominal wall. Duodenum and ascending colon were sutured in their normal position which eased the tension on the anastomosis.
to
the
Histologic examination of the stenotic portion of the duct wall revealed partly hyalinized connective tissue with very few blood vessels, suggestiqg that the stenosis which led to the cystic dilatation of the common duct was caused by an angiectatic scar, probably the result of an early embryonic vascular accident. During the first 8 postoperative days drainage was 10 to 30 ml. and 60 to 100 ml. until the twelfth day when the drain was removed. The child tolerated feedings well and showed a good weight curve. Stools were dark. By the time of discharge liver enzymes were within normal limits. During a 3-year followup there has been no fever, vomiting or jaundice and liver function has remained normal. DISCUSSION
Uncorrected congenital cystic dilatation of the common duct leads to an almost certain fatal outcome. Twenty-nine out of 30 patients reviewed by Tsardakas and RobnetP died. Most of the cases reported in the literature have been treated with direct cystoduodenostomy. Ravitch and Snider5 consider the Roux-en-Y-anastomosis the procedure of choice for large cysts. Alonso-Lei et a1.l prefer removal of the cyst and anastomosis of the biliary tree to the second portion of the duodenum. Although mortality in patients with choledochal cyst has decreased from 50-80 percent before 1945 to 12 percent in recent years,a,F the morbidity remains high. Stones in the cyst have been reported 15 times, 4 times in the gallbladder, and malignant degeneration of the cyst has been recorded 4 times-l If the cyst remains large it may cause pressure upon the portal vein and lead to portal hypertension. The development of liver cirrhosis depends on the degree and duration of the obstruction.. In direct cystoenterostomies ascending cholangitis is common. It is generally believed that a Roux-en-Y-anastomosis of sufficient length prevents ascending infection better than other procedures.” In view of these complications it seems logical to perform a resection of the cyst and an anastomosis of the hepatic duct to the stump of the common duct, or if technically not feasible, with the duodenum. Gross,? in 1933, felt that this procedure was not advisable because of its higher mortality. The high incidence of complications induced us to undertake the reconstruction of a normal anatomy in our case. SUMMARY
A choledochal cyst was excised and the biliary tree reconstructed by an anastomosis between the common hepatic duct and the distal remnant of the common bile duct. When technically feasible this procedure seems preferable to other methods of surgical management. SUMMARIO Un
cyste
anastomose Quando
choledochal
esseva
inter le commun
illo es technicamente
excidite
ducto
IN INTERLINGUA e un arbore
hepatic
practicabile,
ibile a altere methodos de correction
chirurgic.
biliari
e le residuo
esseva
reconstruite
distal de1 commun
le procedimento
describite
pare
per
un
ducto biliari. esser prefer-
CONGENITAL
CHOLJZDOCHAL
607
CYST
REFERENCES 1. Alonso-Lei, F., Rever, W. B., and Pessagno, D. J.: Congenital choledochal cyst, with a report of 2, and an analysis of 94 cases. Surg. Gyn. Obstet. 10&l, 1959. 2. Tsardakeas, E., and Robnett, A. H.: Congenital cystic dilatation of the common bile duct. Arch. Surg. 72:311, 1957. 3. Attar, S., and Obeid, S.: Congenital cyst of the common bile duct. Ann. Surg. 142: 289, 1955. 4. Grob,
M., Stockmann,
M., and Bettex,
M.: Lehrbuch fur Kinderchirurgie. Stuttgart, Georg Thieme Verlag, 1957. 5. Ravitch, M. M., and Snider, G. B.: Congenital cystic dilatation of the common bile duct. Surgery 44:752, 1958. 6. Shields, A. B.: Congenital cystic dilatation of the common bile duct. Amer. J. Surg. 108:142,1964. 7. Gross, R. E.: Idiopathic dilatation of the common bile duct in children J. Pediat. 3: 730, 1933.