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Gastroduodenal duplication
Gastroduodenal By
T.V.M. Mum/, R.K. Bhargava, and Fawzi Salem Rakas Benghazi,
l A newborn baby presented with a lump in the right hypochondrium. She had a bout of upper gastrointestinal bleeding. At operation, a pyloroduedenal duplication cyst was successfully removed. The relevant literature is reviewed. Copyright o 1992 by W.B. Saunders Company INDEX WORDS:
Duplication,
gastroduodenal.
D
UPLICATIONS of the alimentary tract are uncommon congenital malformations of which only 3.8% are of gastric origin.’ Only a third of them present in the neonatal period’ and the most common presentation is a mass in the abdomen3 Symptoms due to gastric outlet obstruction by the duplication occur frequently in newborns.4-6 Gastrointestinal (GI) hemorrhage is a rare occurence in this disease, and it too may occur more often at a later age? Recently, we have encountered a pyloric duplication that presented as a lump detected in the newborn period; the patient did not have any obstructive symptoms but had an attack of GI bleeding. CASE REPORT A full-term baby boy born after a normal delivery to a 32-yearold mother (para 6) was diagnosed at birth to have an intraabdominal lump. The pregnancy was uneventful. At birth, the baby had mild respiratory distress that responded to resuscitation. The baby weighed 3.860 kg, abdomen was mildly distended, and the intraabdominal lump was mainly in the right hypochendrium extending to midline and right lumbar region. It was about 3 cm in diameter, cystic, nontender, relatively mobile in both axes, separate from the liver, and dull on percussion. There was no organomegaly and the systemic examination was normal. On the second day of birth, the patient had an episode of bleeding in the form of melena and hemorrhagic gastric asporate associated with mild fever. PT and AP’IT were prolonged and other investigations were normal. The patient responded to non operative treatment and the bleeding quickly subsided. Later the patient started to tolerate oral breast feeding and remained symtomless. There was no evidence of any pressure effects due to the lump on the GI tract or bihary system and the size remained unchanged. The hemoglobin level remained stable intially but on the 20th day of birth, it was found to be 9.7 g/dL, which was corrected by packed cell transfusion. On the 22nd day after birth, the patient had an attack of high fever with acute tenderness in the upper abdomen but it quickly responded to broad-spectrum antibiotic treatment. However, the lump did not show any significant alteration in its size. Routine investigations including liver and kidney function tests were normal. Ultrasound showed hepatomegaly and the lump was detected as well encapsulated fluid 5.5 x 8 cm situated in the right lobe of the liver, very superficially. Gallbladder and kidneys were normal. Intravenous pyelography and intravenous cavagraphy were also normal, Barium series showed normal bowel except that the upper jejunal loops were pushed to the left. The stomach emptied JournalofPediatric Surgery, Vol27, No
Duplication
4 (April). 1992: pp 515-517
Libya normally but there was narrowing of the pyloric canal with widening of the “C” loop of the duodenum (Fig 1). The patient was operated on and a duplication cyst measuring 5 cm X 8 cm was found occupying the posteroinferior surface of the distal third of the stomach and extending on to the second part of the duodenum. The adjoining stomach and dudenum were stretched over it. The portal vein and the bile duct entered the duodenal area anterior to the superior part of the cyst. Part of the cyst adjacent to the greater curvature was firm with increased vascularity. On aspiration, the cyst yielded about 40 mL of turbid fluid, which on culture grew Kfeibseilln organisms. The cyst opened and at one area there was evidence of inflammation with ulceration, which has corresponded with the firm area found on the surface. However, there was no communication with the GI tract. A near-total excision of the cyst was done, leaving an area overlying the pyloroduodenal region as the portal vein and the common bile duct were closely entangled with the cyst wall there. Mucosa of this residual cyst was cored out, margins were sutured by a continuous catgut suture and hemostasis was secured. A continuous patch of omentum was sutured over the denuded area. The abdomen was closed with a drain. The postoperative period was uneventful. DISCUSSION
In 1984, Bommen and Singh reported that 86 cases of this anomaly were recorded in the literature. We have noted subsequently another three case reports,s-‘Obringing the total number of cases reported in the English la rguage literature to 90. Pruksapong et al3 noted that 3.8% of GI duplications are formed by duplications of the stomach, whereas Wrenn,” while analyzing the alimentary tract duplications reported by various authors, found 13 cases of gastric duplication in a total of 246 cases, thereby giving an incidence of 5.28%. However, only about a third of these gastric duplications were actually diagnosed in the newborn period.2 Of these 90 cases of gastric duplications, 59 cases (65.5%) had a palpable mass. Vomiting was the other common symptom, having been observed in 55 (61%) cases (Table 1). In fact, many of the gastric duplications were mistaken for hypertrophic pyloric stenosis.h6 However, our patient did not vomit, although the cyst was at the pylorus and was fairly large. GI bleeding was found in only 25 (27.7%) reported cases. Unlike in the present case, it was found in older infants and children. It is commonly presumed that
From the Pediatric Surgery Unit, Department of Surgery, Faculty of Medicine, Arab Medical lJniver@, Benghazi, Libya. Address reprint requests to T. V.M. Mu?, MD, Professor of Pediatric Surgery, Arab Medical University, Post Box 6537, Benghazi, Libya. Copyright o 1992 by WB. Saunders Company 0022-346819212704~0025$03.OOlO 515
MURTY, BHARGAVA, AND RAKAS
516
Table 2. Location of the Cyst Location
No. of Cases
Percent
56
65.5
Lesser curvature
7
7.7
Anterior wall
5
5.5
Posterior wall
9
10.0
Pylorus
2
2.2
Others
9
10.0
Greater curvature
NOTE. Total cases = 90.
Fig 1. Barium meal study of stomach and small Intesthw showing nanowing of pyloric canal with widening of “C” loop of duodenum.
bleeding in a gastric duplication requires communication of the duplication with the gastric lumen.3 However, in this case, there was no communication found at surgery. Sometimes, intraperitoneal bleeding may Table 1. Symptoms in Gastric Duplication Symptom
No. of Cases
Percent
Palpable mass
59
65.5
Vomiting
55
61.0
Weight loss
30
33.3
Abdominal pain
27
30.0
Anemia
26
28.8
GI bleeding
25
27.7
8
8.8
Fever
occur due to perforation into the peritoneal cavity,* which may also cause peritonitis,12 pneumoperitoneum,13 or sometimes result in a pseudocyst of pancreas. The location of the duplication cyst is mostly in relation to the greater curvature,3 whereas pyloric cysts are rarely found (Table 2). Usually the cysts are confined to the stomach, although Tehansky et al9 have reported a pyloroduodenal duplication cyst, as was found in the present case. Sometimes they may extend into the thorax, presenting as a double esophagus or, occasionally, they may present at ectopic sites.13 Conversely, pancreatic tissue may be found in the cyst.8 To find other tissues is rare, although Horie et al” reported a carcinoid in a lZyear-old boy and Mayo has reported carcinoma arising in one case. Ideally gastric duplications are treated by total excision with restoration of continuity of the lumen. However, in some cases, due to their extensive size or location, alternate procedures like subtotal excision with removal of mucosa or cystogastrostomy have been done. In our case, in view of its close relation to the common bile duct and the pancreatic duct, we did subtotal excision with stripping of the mucosa as described by Wrenn,” covering the raw area with omentum to prevent subsequent adhesions. Any procedure should ideally ensure removal of mucosa to prevent subsequent malignant transformation. ACKNOWLEDGMENT We thank the Director, Jala Hospital, for permitting us to use the hospital records to report this case and Vivian B. Florido, medical secretary, for typing the manuscript.
NOTE. Total cases = 90.
REFERENCES 1. Silverman A, Roy CC, Cozetto FJ: Pediatric Clinical Gastroenterology. St Louis, MO, Mosby, 1971, p 71 2. Lister J, Rickham PP: Neonatal surgery (ed 2). London, England, Buttetworths, 1978, pp 401404 3. Pruksapong C, Donovan RJ, Pinit A, et al: Gastric duplication. J Pediatr Surg 14183~86,1979 4. Grosfeld J, Boles T, Reiner C: Duplication of pylorus in new born. A rare cause of gastric outlet obstruction. J Pediatr Surg 5:365-366,197O 5. Kammerer GJ: Duplication of stomach resembling hypertrophic pyloric stenosis. JAMA 207:2101-2102, 1969
6. Al Schibaja T, Putnam TC, Yablin BA: Duplication of the stomach simulating hypertrophic pyloric stenosis. Am J Dis Child 127:120-122,1974 7. Bommen M, Singh MP: Pyloric stenosis in a preterm neonate. J Pediatr Surg 19:158-159, 1984 8. Spencer RK, Schaufer L, Mahboubi S: Existant gastric duplication and accessory pancreas: Clinical manifestations embryogenesis and treatment. J Pediatr Surg 21:68-70, 1986 9. Tehansky DP, Sukarochana K, Hanrahan JB: Pyloroduodenal duplication cyst. Am J Gastroenterol81:189-191, 1986
GASTRODUODENAL
DUPLICATION
10. Horie H, Iwasaki I, Takahashi H: Carcinoid in a G.I. duplication. J Pedtiatr Surg 21:902-904, 1986 11. Wrenn EL Jr: Alimentary tract duplication, in Holder TM, Ashcraft KW (eds): Pediatric Surgery. Philadelphia, PA, Saunders, 1980, pp 445-456
517
12. Kleinhaus S, Boley SJ, Winslow P: Occult bleeding from a perforated gastric duplication in an infant. Arch Surg 116:122-123,198l 13. Schwartz DL, So HB, Becker JM, et al: An extopic gastric duplication arising from the pancreas and presenting with pneumoperitoneum. J Pediatr Surg 14:187-188,1979
T.V.M. Mum/, R.K. Bhargava, and Fawzi Salem Rakas Benghazi,
l A newborn baby presented with a lump in the right hypochondrium. She had a bout of upper gastrointestinal bleeding. At operation, a pyloroduedenal duplication cyst was successfully removed. The relevant literature is reviewed. Copyright o 1992 by W.B. Saunders Company INDEX WORDS:
Duplication,
gastroduodenal.
D
UPLICATIONS of the alimentary tract are uncommon congenital malformations of which only 3.8% are of gastric origin.’ Only a third of them present in the neonatal period’ and the most common presentation is a mass in the abdomen3 Symptoms due to gastric outlet obstruction by the duplication occur frequently in newborns.4-6 Gastrointestinal (GI) hemorrhage is a rare occurence in this disease, and it too may occur more often at a later age? Recently, we have encountered a pyloric duplication that presented as a lump detected in the newborn period; the patient did not have any obstructive symptoms but had an attack of GI bleeding. CASE REPORT A full-term baby boy born after a normal delivery to a 32-yearold mother (para 6) was diagnosed at birth to have an intraabdominal lump. The pregnancy was uneventful. At birth, the baby had mild respiratory distress that responded to resuscitation. The baby weighed 3.860 kg, abdomen was mildly distended, and the intraabdominal lump was mainly in the right hypochendrium extending to midline and right lumbar region. It was about 3 cm in diameter, cystic, nontender, relatively mobile in both axes, separate from the liver, and dull on percussion. There was no organomegaly and the systemic examination was normal. On the second day of birth, the patient had an episode of bleeding in the form of melena and hemorrhagic gastric asporate associated with mild fever. PT and AP’IT were prolonged and other investigations were normal. The patient responded to non operative treatment and the bleeding quickly subsided. Later the patient started to tolerate oral breast feeding and remained symtomless. There was no evidence of any pressure effects due to the lump on the GI tract or bihary system and the size remained unchanged. The hemoglobin level remained stable intially but on the 20th day of birth, it was found to be 9.7 g/dL, which was corrected by packed cell transfusion. On the 22nd day after birth, the patient had an attack of high fever with acute tenderness in the upper abdomen but it quickly responded to broad-spectrum antibiotic treatment. However, the lump did not show any significant alteration in its size. Routine investigations including liver and kidney function tests were normal. Ultrasound showed hepatomegaly and the lump was detected as well encapsulated fluid 5.5 x 8 cm situated in the right lobe of the liver, very superficially. Gallbladder and kidneys were normal. Intravenous pyelography and intravenous cavagraphy were also normal, Barium series showed normal bowel except that the upper jejunal loops were pushed to the left. The stomach emptied JournalofPediatric Surgery, Vol27, No
Duplication
4 (April). 1992: pp 515-517
Libya normally but there was narrowing of the pyloric canal with widening of the “C” loop of the duodenum (Fig 1). The patient was operated on and a duplication cyst measuring 5 cm X 8 cm was found occupying the posteroinferior surface of the distal third of the stomach and extending on to the second part of the duodenum. The adjoining stomach and dudenum were stretched over it. The portal vein and the bile duct entered the duodenal area anterior to the superior part of the cyst. Part of the cyst adjacent to the greater curvature was firm with increased vascularity. On aspiration, the cyst yielded about 40 mL of turbid fluid, which on culture grew Kfeibseilln organisms. The cyst opened and at one area there was evidence of inflammation with ulceration, which has corresponded with the firm area found on the surface. However, there was no communication with the GI tract. A near-total excision of the cyst was done, leaving an area overlying the pyloroduodenal region as the portal vein and the common bile duct were closely entangled with the cyst wall there. Mucosa of this residual cyst was cored out, margins were sutured by a continuous catgut suture and hemostasis was secured. A continuous patch of omentum was sutured over the denuded area. The abdomen was closed with a drain. The postoperative period was uneventful. DISCUSSION
In 1984, Bommen and Singh reported that 86 cases of this anomaly were recorded in the literature. We have noted subsequently another three case reports,s-‘Obringing the total number of cases reported in the English la rguage literature to 90. Pruksapong et al3 noted that 3.8% of GI duplications are formed by duplications of the stomach, whereas Wrenn,” while analyzing the alimentary tract duplications reported by various authors, found 13 cases of gastric duplication in a total of 246 cases, thereby giving an incidence of 5.28%. However, only about a third of these gastric duplications were actually diagnosed in the newborn period.2 Of these 90 cases of gastric duplications, 59 cases (65.5%) had a palpable mass. Vomiting was the other common symptom, having been observed in 55 (61%) cases (Table 1). In fact, many of the gastric duplications were mistaken for hypertrophic pyloric stenosis.h6 However, our patient did not vomit, although the cyst was at the pylorus and was fairly large. GI bleeding was found in only 25 (27.7%) reported cases. Unlike in the present case, it was found in older infants and children. It is commonly presumed that
From the Pediatric Surgery Unit, Department of Surgery, Faculty of Medicine, Arab Medical lJniver@, Benghazi, Libya. Address reprint requests to T. V.M. Mu?, MD, Professor of Pediatric Surgery, Arab Medical University, Post Box 6537, Benghazi, Libya. Copyright o 1992 by WB. Saunders Company 0022-346819212704~0025$03.OOlO 515
MURTY, BHARGAVA, AND RAKAS
516
Table 2. Location of the Cyst Location
No. of Cases
Percent
56
65.5
Lesser curvature
7
7.7
Anterior wall
5
5.5
Posterior wall
9
10.0
Pylorus
2
2.2
Others
9
10.0
Greater curvature
NOTE. Total cases = 90.
Fig 1. Barium meal study of stomach and small Intesthw showing nanowing of pyloric canal with widening of “C” loop of duodenum.
bleeding in a gastric duplication requires communication of the duplication with the gastric lumen.3 However, in this case, there was no communication found at surgery. Sometimes, intraperitoneal bleeding may Table 1. Symptoms in Gastric Duplication Symptom
No. of Cases
Percent
Palpable mass
59
65.5
Vomiting
55
61.0
Weight loss
30
33.3
Abdominal pain
27
30.0
Anemia
26
28.8
GI bleeding
25
27.7
8
8.8
Fever
occur due to perforation into the peritoneal cavity,* which may also cause peritonitis,12 pneumoperitoneum,13 or sometimes result in a pseudocyst of pancreas. The location of the duplication cyst is mostly in relation to the greater curvature,3 whereas pyloric cysts are rarely found (Table 2). Usually the cysts are confined to the stomach, although Tehansky et al9 have reported a pyloroduodenal duplication cyst, as was found in the present case. Sometimes they may extend into the thorax, presenting as a double esophagus or, occasionally, they may present at ectopic sites.13 Conversely, pancreatic tissue may be found in the cyst.8 To find other tissues is rare, although Horie et al” reported a carcinoid in a lZyear-old boy and Mayo has reported carcinoma arising in one case. Ideally gastric duplications are treated by total excision with restoration of continuity of the lumen. However, in some cases, due to their extensive size or location, alternate procedures like subtotal excision with removal of mucosa or cystogastrostomy have been done. In our case, in view of its close relation to the common bile duct and the pancreatic duct, we did subtotal excision with stripping of the mucosa as described by Wrenn,” covering the raw area with omentum to prevent subsequent adhesions. Any procedure should ideally ensure removal of mucosa to prevent subsequent malignant transformation. ACKNOWLEDGMENT We thank the Director, Jala Hospital, for permitting us to use the hospital records to report this case and Vivian B. Florido, medical secretary, for typing the manuscript.
NOTE. Total cases = 90.
REFERENCES 1. Silverman A, Roy CC, Cozetto FJ: Pediatric Clinical Gastroenterology. St Louis, MO, Mosby, 1971, p 71 2. Lister J, Rickham PP: Neonatal surgery (ed 2). London, England, Buttetworths, 1978, pp 401404 3. Pruksapong C, Donovan RJ, Pinit A, et al: Gastric duplication. J Pediatr Surg 14183~86,1979 4. Grosfeld J, Boles T, Reiner C: Duplication of pylorus in new born. A rare cause of gastric outlet obstruction. J Pediatr Surg 5:365-366,197O 5. Kammerer GJ: Duplication of stomach resembling hypertrophic pyloric stenosis. JAMA 207:2101-2102, 1969
6. Al Schibaja T, Putnam TC, Yablin BA: Duplication of the stomach simulating hypertrophic pyloric stenosis. Am J Dis Child 127:120-122,1974 7. Bommen M, Singh MP: Pyloric stenosis in a preterm neonate. J Pediatr Surg 19:158-159, 1984 8. Spencer RK, Schaufer L, Mahboubi S: Existant gastric duplication and accessory pancreas: Clinical manifestations embryogenesis and treatment. J Pediatr Surg 21:68-70, 1986 9. Tehansky DP, Sukarochana K, Hanrahan JB: Pyloroduodenal duplication cyst. Am J Gastroenterol81:189-191, 1986
GASTRODUODENAL
DUPLICATION
10. Horie H, Iwasaki I, Takahashi H: Carcinoid in a G.I. duplication. J Pedtiatr Surg 21:902-904, 1986 11. Wrenn EL Jr: Alimentary tract duplication, in Holder TM, Ashcraft KW (eds): Pediatric Surgery. Philadelphia, PA, Saunders, 1980, pp 445-456
517
12. Kleinhaus S, Boley SJ, Winslow P: Occult bleeding from a perforated gastric duplication in an infant. Arch Surg 116:122-123,198l 13. Schwartz DL, So HB, Becker JM, et al: An extopic gastric duplication arising from the pancreas and presenting with pneumoperitoneum. J Pediatr Surg 14:187-188,1979