Preduodenal ByEduardo
Portal Vein: Surgery and Radiographic T. Fernandes,
Edward
Appearance
M. Burton, S. Douglas Hixson, and Robert S. Holtabaugh
Memphis, Tennessee 6 Preduodenal portal vein is rare, with 63 cases reported in the literature. In general, this anomaly occurs in children with associated small bowel obstruction. We report a newborn infant who presented with duodenal stenosis, mongolism, and preduodenal portal vein. Treatment consisted of a duodenoduodenal anastomosis without mobilizing the portal vein. The correlation between imaging techniques and the operative findings is discussed. Because identification of preduodenal portal vein at surgery is important, preoperative sonography may be useful in selected cases to define the position of the vein. 6 1990 by W.B. Saunders Company. INDEX WORDS:
Duodenal obstruction;
portal vein anomaly.
A
BNORMALITIES in the position of the portal vein are rare. We report the second case of preduodenal portal vein (PDPV) in combination with duodenal stenosis and Down’s syndrome. CASE
REPORT
An &hour-old black girl was transferred to LeBonheur Children’s Medical Center with the prenatal sonographic diagnosis of duodenal atresia. The mother was a 24-year-old woman (gravida 6, para 5) who had used marijuana, intravenous cocaine, and alcohol during the pregnancy. The child was born at 39 weeks’ gestation by spontaneous vaginal delivery. Her weight and length were 2.485 kg and 47 cm. Typical stigmata of Down’s syndrome (47,Xx,+21) were noted on physical examination. On the first day of life, a barium study of the upper gastrointestinal tract (UGI) showed a partially obstructing web in the second portion of the duodenum (Fig 1). At surgery, a nonobstructing PDPV was found proximal to theduodenal web (Fig 2). With transverse proximal and longitudinal distal incisions, a duodenoduodenal anastomosis was performed without mobilizing or compressing the vein (Fig 3). Recovery was uneventful. Two months later, abdominal ultrasound demonstrated the unusual position of the portal vein (Fig 4). DISCUSSION
Esscher,’ in 1980, reviewed 52 cases of PDPV; 11 additional patients with this anomaly have been reported from 1979 to 1988. The embryogenesis of this anomaly, described by Gray and Skandalakis,* consists
From the Departments of Surgery and Radiology, LeBonheur Children’s Medical Center and University of Tennessee School of Medicine, Memphis, TN. Address reprint requests to Edward M. Burton, MD, Radiology Associates, 1920 Huntington Rd. Birmingham, AL 35209. 0 1990 by W.B. Saunders Company. 0022-3468/90/2512-0024%03.00/0
1270
Fig 1. UGI. Web involving proximal second portion of duodenum. Vascular impression of PDPV is not visible.
of the persistence of a preduodenal vitelline communicating vein. Although PDPV has been reported in adults, 64% of patients are children.3 Most patients with PDPV present with intestinal obstruction. Two thirds of children with PDPV are detected in the first week of life due to associated intrinsic duodenal anomaly, malrotation, or Ladd’s bands. Other anomalies associated with PDPV include annular pancreas, biliary atresia, preduodenal common bile duct, and cardiovascular malformations.‘~4-6 The role of PDPV as the etiology of intestinal obstruction is controversial. In 80% of patients with PDPV, an intrinsic lesion of the duodenum or malrotation is responsible for obstruction.’ In this patient, PDPV was clearly proximal to the area of obstruction. Despite the well-known association of duodenal atresia or stenosis and Down’s syndrome, this case is the second of PDPV in combination with duodenal obstruction and mongolism.5 Identification of PDPV is rarely made preoperatively, but has been reported in an adult with the use of computed tomography and angiography.4 This report is the second to describe the sonography of PDPV.3 The presence of PDPV complicates surgery for duodenal obstruction. The integrity of the vessel must
Journal of Pediatric Surgery, Vol 25, No 12 (December), 1990: pp 1270-1272
PREDIJODENAL PORTAL VEfN
1271
Fig 2. (A) Operative anterior to duodenum. portion of duodenum Umbilical tape is around c, caudad; curved arrow,
be preserved to avoid portal vein thrombosis. On the other hand, the duodenum cannot be completely divided and anastomosed anterior to the vein, due to the proximity of the pancreas and common bile duct. Gastrectomy, gastrojejunostomy, gastroduodenostomy, duodenojejunostomy, and duodenoplasty were previously advocated to bypass duodenal stenosis in patients with PDPV.’ Currently, duodenoduodenal anastomosis is the procedure of choice to treat this anomaly.6 The anastomosis is created anterior to the portal vein, between the segments of the duodenum immediately proximal and distal to the obstruction (Fig 3). The portal vein will then lie between the
Fig 3. Diagram of anterior duodenoduodenal anastomosis performed to bypass the duodenal web. The PDPV (arrow) is loosely surrounded by the anastomosis.
photograph and (B) diagram showing PDPV The duodenal bulb (arrow) and second (arrowhead) are posterior to the PDPV. the portal vein. L, left; R. right: C, cephalad: region of duodenal web.
second portion of the duodenum and the newly created anastomosis. The demonstration of vascular anatomy by real-time ultrasound may be useful in patients with malrotation or congenital duodenal obstruction. The correct preoperative diagnosis of PDPV may prevent accidental injury to this vessel. Although a vascular impression on the duodenum by the PDPV was not visible by UGI in this case, the superior orientation of the obstructed duodenum and the abnormal course of the distal duodenum resembled images of PDPV and duodenal
Fig 4. Postoperative transverse ultrasound (curved arrow) anterior to the pancreas (p).
showing
PDPV
FERNANDES ET AL
1272
obstruction reported by Johnson’ and by Georgacopulo and Vigi.s In patients such as this one, sonography can be confusing to the radiologist unfamiliar with the anomalous position of the vein and surgical technique to treat duodenal obstruction. The anterior location of the
portal vein in relation to the pancreas, shown by sonography, is diagnostic of PDPV (Fig 4). ACKNOWLEDGMENT We thank Laura Burton for editing and Patty Felix for accurately preparing this manuscript.
REFERENCES 1. Esscher T: Preduodenal portal vein-A cause of intestinal obstruction? J Pediatr Surg 15:609-612, 1980 2. Gray SW, Skandalakis JH: Embryology for Surgeons. The Embryological Basis for the Treatment of Congenital Defects. Philadelphia, PA, Saunders, 1972, pp 177-178 3. McCarten KM, Teele RL: Preduodenal portal vein: Venography, ultrasonography, and review of the literature. Ann Radio1 21:155-160, 1978 4. Sasai K, Sano A, Nishizawa S, et al: Preduodenal portal vein in an adult-Angiography and CT. Radiat Med 3:87-89, 1985
5. Braun P, Cuendet A: Preduodenal portal vein. Progr Pediatr Surg 3:121-140, 1972 6. Patti G, Marrocco G, Mazzoni G, et al: Esophageal and duodenal atresia with preduodenal common bile duct and portal vein in a newborn. J Pediatr Surg 20:167-168, 1985 7. Johnson GF: Congenital preduodenal portal vein. AJR 112:9399.1971 8. Georgacopulo P, Vigi V: Duodenal obstruction due to a preduodenal portal vein in a newborn. J Pediatr Surg 15:339-340, 1980
Portal Vein: Surgery and Radiographic T. Fernandes,
Edward
Appearance
M. Burton, S. Douglas Hixson, and Robert S. Holtabaugh
Memphis, Tennessee 6 Preduodenal portal vein is rare, with 63 cases reported in the literature. In general, this anomaly occurs in children with associated small bowel obstruction. We report a newborn infant who presented with duodenal stenosis, mongolism, and preduodenal portal vein. Treatment consisted of a duodenoduodenal anastomosis without mobilizing the portal vein. The correlation between imaging techniques and the operative findings is discussed. Because identification of preduodenal portal vein at surgery is important, preoperative sonography may be useful in selected cases to define the position of the vein. 6 1990 by W.B. Saunders Company. INDEX WORDS:
Duodenal obstruction;
portal vein anomaly.
A
BNORMALITIES in the position of the portal vein are rare. We report the second case of preduodenal portal vein (PDPV) in combination with duodenal stenosis and Down’s syndrome. CASE
REPORT
An &hour-old black girl was transferred to LeBonheur Children’s Medical Center with the prenatal sonographic diagnosis of duodenal atresia. The mother was a 24-year-old woman (gravida 6, para 5) who had used marijuana, intravenous cocaine, and alcohol during the pregnancy. The child was born at 39 weeks’ gestation by spontaneous vaginal delivery. Her weight and length were 2.485 kg and 47 cm. Typical stigmata of Down’s syndrome (47,Xx,+21) were noted on physical examination. On the first day of life, a barium study of the upper gastrointestinal tract (UGI) showed a partially obstructing web in the second portion of the duodenum (Fig 1). At surgery, a nonobstructing PDPV was found proximal to theduodenal web (Fig 2). With transverse proximal and longitudinal distal incisions, a duodenoduodenal anastomosis was performed without mobilizing or compressing the vein (Fig 3). Recovery was uneventful. Two months later, abdominal ultrasound demonstrated the unusual position of the portal vein (Fig 4). DISCUSSION
Esscher,’ in 1980, reviewed 52 cases of PDPV; 11 additional patients with this anomaly have been reported from 1979 to 1988. The embryogenesis of this anomaly, described by Gray and Skandalakis,* consists
From the Departments of Surgery and Radiology, LeBonheur Children’s Medical Center and University of Tennessee School of Medicine, Memphis, TN. Address reprint requests to Edward M. Burton, MD, Radiology Associates, 1920 Huntington Rd. Birmingham, AL 35209. 0 1990 by W.B. Saunders Company. 0022-3468/90/2512-0024%03.00/0
1270
Fig 1. UGI. Web involving proximal second portion of duodenum. Vascular impression of PDPV is not visible.
of the persistence of a preduodenal vitelline communicating vein. Although PDPV has been reported in adults, 64% of patients are children.3 Most patients with PDPV present with intestinal obstruction. Two thirds of children with PDPV are detected in the first week of life due to associated intrinsic duodenal anomaly, malrotation, or Ladd’s bands. Other anomalies associated with PDPV include annular pancreas, biliary atresia, preduodenal common bile duct, and cardiovascular malformations.‘~4-6 The role of PDPV as the etiology of intestinal obstruction is controversial. In 80% of patients with PDPV, an intrinsic lesion of the duodenum or malrotation is responsible for obstruction.’ In this patient, PDPV was clearly proximal to the area of obstruction. Despite the well-known association of duodenal atresia or stenosis and Down’s syndrome, this case is the second of PDPV in combination with duodenal obstruction and mongolism.5 Identification of PDPV is rarely made preoperatively, but has been reported in an adult with the use of computed tomography and angiography.4 This report is the second to describe the sonography of PDPV.3 The presence of PDPV complicates surgery for duodenal obstruction. The integrity of the vessel must
Journal of Pediatric Surgery, Vol 25, No 12 (December), 1990: pp 1270-1272
PREDIJODENAL PORTAL VEfN
1271
Fig 2. (A) Operative anterior to duodenum. portion of duodenum Umbilical tape is around c, caudad; curved arrow,
be preserved to avoid portal vein thrombosis. On the other hand, the duodenum cannot be completely divided and anastomosed anterior to the vein, due to the proximity of the pancreas and common bile duct. Gastrectomy, gastrojejunostomy, gastroduodenostomy, duodenojejunostomy, and duodenoplasty were previously advocated to bypass duodenal stenosis in patients with PDPV.’ Currently, duodenoduodenal anastomosis is the procedure of choice to treat this anomaly.6 The anastomosis is created anterior to the portal vein, between the segments of the duodenum immediately proximal and distal to the obstruction (Fig 3). The portal vein will then lie between the
Fig 3. Diagram of anterior duodenoduodenal anastomosis performed to bypass the duodenal web. The PDPV (arrow) is loosely surrounded by the anastomosis.
photograph and (B) diagram showing PDPV The duodenal bulb (arrow) and second (arrowhead) are posterior to the PDPV. the portal vein. L, left; R. right: C, cephalad: region of duodenal web.
second portion of the duodenum and the newly created anastomosis. The demonstration of vascular anatomy by real-time ultrasound may be useful in patients with malrotation or congenital duodenal obstruction. The correct preoperative diagnosis of PDPV may prevent accidental injury to this vessel. Although a vascular impression on the duodenum by the PDPV was not visible by UGI in this case, the superior orientation of the obstructed duodenum and the abnormal course of the distal duodenum resembled images of PDPV and duodenal
Fig 4. Postoperative transverse ultrasound (curved arrow) anterior to the pancreas (p).
showing
PDPV
FERNANDES ET AL
1272
obstruction reported by Johnson’ and by Georgacopulo and Vigi.s In patients such as this one, sonography can be confusing to the radiologist unfamiliar with the anomalous position of the vein and surgical technique to treat duodenal obstruction. The anterior location of the
portal vein in relation to the pancreas, shown by sonography, is diagnostic of PDPV (Fig 4). ACKNOWLEDGMENT We thank Laura Burton for editing and Patty Felix for accurately preparing this manuscript.
REFERENCES 1. Esscher T: Preduodenal portal vein-A cause of intestinal obstruction? J Pediatr Surg 15:609-612, 1980 2. Gray SW, Skandalakis JH: Embryology for Surgeons. The Embryological Basis for the Treatment of Congenital Defects. Philadelphia, PA, Saunders, 1972, pp 177-178 3. McCarten KM, Teele RL: Preduodenal portal vein: Venography, ultrasonography, and review of the literature. Ann Radio1 21:155-160, 1978 4. Sasai K, Sano A, Nishizawa S, et al: Preduodenal portal vein in an adult-Angiography and CT. Radiat Med 3:87-89, 1985
5. Braun P, Cuendet A: Preduodenal portal vein. Progr Pediatr Surg 3:121-140, 1972 6. Patti G, Marrocco G, Mazzoni G, et al: Esophageal and duodenal atresia with preduodenal common bile duct and portal vein in a newborn. J Pediatr Surg 20:167-168, 1985 7. Johnson GF: Congenital preduodenal portal vein. AJR 112:9399.1971 8. Georgacopulo P, Vigi V: Duodenal obstruction due to a preduodenal portal vein in a newborn. J Pediatr Surg 15:339-340, 1980
