- Email: [email protected]
Preduodenal portal vein in association with midgut malrotation and duodenal web—triple anomaly?
Journal of Pediatric Surgery (2009) 44, E5–E7
www.elsevier.com/locate/jpedsurg
Preduodenal portal vein in association with midgut malrotation and duodenal web—triple anomaly? Arbinder Kumar Singala,⁎, Chithra Ramub , Sarah Paulc , John Matthaic a
Department of Pediatric Surgery, MGM Medical College, Kamothe, Navi Mumbai, Maharashtra, India 400703 Department of General Surgery, PSG Institute of Medical Sciences, Coimbatore, Tamil Nadu, India 641004 c Department of Pediatrics, PSG Institute of Medical Sciences, Coimbatore, Tamil Nadu, India 641004 b
Received 7 September 2008; revised 20 October 2008; accepted 22 October 2008
Key words: Preduodenal portal vein; Duodenal web; Midgut malrotation; Duodenal obstruction; Neonate
Abstract Preduodenal portal vein (PDPV) is a rare anomaly in which the portal vein passes anterior to the duodenum rather than posteriorly. Generally asymptomatic, PDPV may rarely cause duodenal obstruction or may coexist with other anomalies. We report a neonate who presented with duodenal obstruction and was found out to have 3 coexisting anomalies, each of which can lead to duodenal obstruction independently—PDPV, midgut malrotation, and duodenal web. A duodenoduodenostomy and a Ladd procedure were done, and the child recovered uneventfully. The mechanism of obstruction, interesting metabolic aberrations observed, outcome, and relevant literature are presented. © 2009 Elsevier Inc. All rights reserved.
Preduodenal portal vein (PDPV) is a rare anomaly in which the portal vein passes anterior to the duodenum rather than posteriorly. The condition was first described by Knight [1] in 1921. The portal vein arises during fetal life by a schematic absorption of the interconnecting veins of the vitelline venous system. The 2 parallel vitelline veins are joined by 3 interconnecting veins—the cephalad branch within the liver; the middle branch posterior to duodenum, and the caudal branch anterior to duodenum. Later in development, the caudal and the cephalad anastomosis, with the caudal part of the right vitelline vein and the cephalad part of the left vitelline vein, disappear, leaving an S-shaped portal vein passing behind the duodenum. A variation in this ⁎ Corresponding author. MGM Hospital, Vashi, Navi Mumbai, Maharashtra 400703, India. Tel.: +91 9323269944; fax: +91 2227820520. E-mail address: [email protected] (A.K. Singal). 0022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2008.10.075
process, in which the middle and the cephalad anastomosis disappear together with the left vitelline vein, accounts for the PDPV [1-3]. Preduodenal portal vein may be found incidentally or may be a rare cause of duodenal obstruction. We report a neonate with partial duodenal obstruction who had a PDPV in addition to midgut malrotation and a duodenal web.
1. Case report A 7-day-old girl was referred with a history of repeated nonbilious vomiting since the first day of life. The child was the product of an uncomplicated pregnancy and a normal labor and delivery. There was no abdominal distension or features of sepsis. Plain abdominal radiograph showed a dilated stomach and the first part of duodenum with air-fluid levels. There was some gas seen in the distal intestines
E6 signifying a partial obstruction. An upper gastrointestinal contrast study showed dilated stomach and the first part of duodenum with an abrupt cutoff and some distal runoff of contrast confirming partial duodenal obstruction. The duodenum distal to the cutoff had a cork screw pattern suggestive of malrotation (Fig. 1). The serum electrolyte pattern showed severe metabolic alkalosis with hypokalemia and hypochloremia consistent with abnormalities often seen in gastric outlet obstruction. Intravenous normal saline and potassium replacement was started, and a laparotomy was planned after correction of electrolyte abnormalities. At laparotomy, there were features suggestive of midgut malrotation with a 360° volvulus but preserved vascularity. The stomach and first part of duodenum were dilated, and the portal vein was seen to cross the first part of duodenum anteriorly, about 3 cm from the pylorus (Fig. 2). A Fogarty catheter inserted through the stomach to check intrinsic obstruction could not be passed beyond the area where the PDPV was crossing. A transverse duodenotomy was made in the first part of duodenum proximal to PDPV, and a longitudinal opening was made in the duodenum distally. A thick duodenal web with a small central hole was observed to obstruct the lumen. The ampulla of Vater was located on the distal side of the web. A diamond-shaped duodenoduodenostomy was performed anterior to the portal vein with a single layer of interrupted polyglactin sutures. A Ladd procedure was completed by lysis of adherent bands, widening the mesentery, appendicectomy, and placing the duodenum in the right paracolic gutter and cecum in the left upper abdomen. Postoperatively, parenteral nutrition was administered, and small volume of oral feeds were started on day 7 and gradually advanced. The baby was discharged uneventfully
Fig. 1 Contrast study showing partial duodenal obstruction; some contrast has passed distally beyond the abrupt cutoff. Note the cork screw appearance of the duodenum distal to the cutoff. Also some gas is seen in bowel loops distally.
A.K. Singal et al.
Fig. 2 Operative picture showing PDPV (arrow), distended stomach, and the first part of duodenum (stay sutures).
on day 12 on full feeds. At 1 year of follow-up, the child has remained asymptomatic.
2. Discussion Preduodenal portal vein is a rare entity, and less than 90 cases have been thus far reported in the literature [4]. Duodenal obstruction has been noted to be the most common clinical presentation in reported cases; although in less than half of these, PDPV was thought to be directly responsible for the duodenal obstruction. Most cases had associated anomalies that led to duodenal obstruction—duodenal atresia or web, annular pancreas, or anomalies of rotation and fixation [2-9]. Thus, it is prudent to rule out an intrinsic duodenal obstruction in all cases before PDPV is labeled as a cause for duodenal obstruction. Esscher [3] reported a case of PDPV and malrotation, in which initial surgery was unsuccessful, and on reexploration, a duodenal diaphragm was found to be the cause of duodenal obstruction. Other associated anomalies include situs inversus, polysplenia, and asplenia [2,4,6]. Preduodenal portal vein, when unrecognized, is at risk to injury during surgery in the vicinity such as during cholecystectomy, gastrectomy, portoenterostomy, and pancreatectomy. A vigilant surgeon can prevent these complications by recognizing this anomaly early. In the present case, the duodenal obstruction resulted from a duodenal web, which was present at the junction of the first and second part of duodenum causing a preampullary type of duodenal obstruction. This preduodenal obstruction is further evidenced by the presence of nonbilious vomiting and metabolic alterations reminiscent of pyloric stenosis. In our assessment, PDPV was not directly responsible for the duodenal obstruction, but its location at the site was unusual and posed technical problems. The preferred treatment of duodenal obstruction caused by PDPV is duodenoduodenostomy, as was done in the present case [5,6,9]. Duodenoduodenostomy in this
PDPV in association with midgut malrotation and duodenal web circumstance bypassed both the abnormalities—duodenal web as well as the PDPV. In the present report, there were 3 anomalies present concomitantly, and each one has a potential to cause duodenal obstruction independently. Furthermore, the metabolic abnormalities observed in the present case were reminiscent of pyloric stenosis because the preampullary obstruction leads to nonbilious vomiting of gastric secretions. Preduodenal portal vein should alert the surgeon to look for other associated anomalies that can lead to duodenal obstruction. Labeling PDPV as the obvious and apparent cause of obstruction may be misleading.
References [1] Knight HO. An anomalous portal vein with its surgical dangers. Ann Surg 1921;74:697-9.
E7
[2] Ziv Y, Lombrozo R, Dintsman M. Preduodenal portal vein with situs inversus and duodenal atresia. Aust Paediatr J 1986;22:69-70. [3] Esscher T. Preduodenal portal vein—a cause of intestinal obstruction? J Pediatr Surg 1980;15:609-12. [4] Mordehai J, Cohen Z, Kurzbart E, et al. Preduodenal portal vein causing duodenal obstruction associated with situs inversus, intestinal malrotation, and polysplenia: a case report. J Pediatr Surg 2002;37 (4):E5. [5] Georgacopulo P, Vigi V. Duodenal obstruction due to a preduodenal portal vein in a newborn. J Pediatr Surg 1980;15:339-40. [6] Kataria R, Bhatnagar V, Wadhwa S, et al. Gastric pneumatosis associated with preduodenal portal vein, duodenal atresia, and asplenia. Pediatr Surg Int 1998;14:100-1. [7] Fernandes ET, Burton EM, Hixson SD, et al. Preduodenal portal vein: surgery and radiographic appearance. J Pediatr Surg 1990; 25:1270-2. [8] Golombek S, Bilgi J, Ukabiala O. Duodenal web with preduodenal portal vein. Iowa Med 1995;85:247-9. [9] Choi SO, Park WH. Preduodenal portal vein: a cause of prenatally diagnosed duodenal obstruction. J Pediatr Surg 1995;30:1521-2.
www.elsevier.com/locate/jpedsurg
Preduodenal portal vein in association with midgut malrotation and duodenal web—triple anomaly? Arbinder Kumar Singala,⁎, Chithra Ramub , Sarah Paulc , John Matthaic a
Department of Pediatric Surgery, MGM Medical College, Kamothe, Navi Mumbai, Maharashtra, India 400703 Department of General Surgery, PSG Institute of Medical Sciences, Coimbatore, Tamil Nadu, India 641004 c Department of Pediatrics, PSG Institute of Medical Sciences, Coimbatore, Tamil Nadu, India 641004 b
Received 7 September 2008; revised 20 October 2008; accepted 22 October 2008
Key words: Preduodenal portal vein; Duodenal web; Midgut malrotation; Duodenal obstruction; Neonate
Abstract Preduodenal portal vein (PDPV) is a rare anomaly in which the portal vein passes anterior to the duodenum rather than posteriorly. Generally asymptomatic, PDPV may rarely cause duodenal obstruction or may coexist with other anomalies. We report a neonate who presented with duodenal obstruction and was found out to have 3 coexisting anomalies, each of which can lead to duodenal obstruction independently—PDPV, midgut malrotation, and duodenal web. A duodenoduodenostomy and a Ladd procedure were done, and the child recovered uneventfully. The mechanism of obstruction, interesting metabolic aberrations observed, outcome, and relevant literature are presented. © 2009 Elsevier Inc. All rights reserved.
Preduodenal portal vein (PDPV) is a rare anomaly in which the portal vein passes anterior to the duodenum rather than posteriorly. The condition was first described by Knight [1] in 1921. The portal vein arises during fetal life by a schematic absorption of the interconnecting veins of the vitelline venous system. The 2 parallel vitelline veins are joined by 3 interconnecting veins—the cephalad branch within the liver; the middle branch posterior to duodenum, and the caudal branch anterior to duodenum. Later in development, the caudal and the cephalad anastomosis, with the caudal part of the right vitelline vein and the cephalad part of the left vitelline vein, disappear, leaving an S-shaped portal vein passing behind the duodenum. A variation in this ⁎ Corresponding author. MGM Hospital, Vashi, Navi Mumbai, Maharashtra 400703, India. Tel.: +91 9323269944; fax: +91 2227820520. E-mail address: [email protected] (A.K. Singal). 0022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2008.10.075
process, in which the middle and the cephalad anastomosis disappear together with the left vitelline vein, accounts for the PDPV [1-3]. Preduodenal portal vein may be found incidentally or may be a rare cause of duodenal obstruction. We report a neonate with partial duodenal obstruction who had a PDPV in addition to midgut malrotation and a duodenal web.
1. Case report A 7-day-old girl was referred with a history of repeated nonbilious vomiting since the first day of life. The child was the product of an uncomplicated pregnancy and a normal labor and delivery. There was no abdominal distension or features of sepsis. Plain abdominal radiograph showed a dilated stomach and the first part of duodenum with air-fluid levels. There was some gas seen in the distal intestines
E6 signifying a partial obstruction. An upper gastrointestinal contrast study showed dilated stomach and the first part of duodenum with an abrupt cutoff and some distal runoff of contrast confirming partial duodenal obstruction. The duodenum distal to the cutoff had a cork screw pattern suggestive of malrotation (Fig. 1). The serum electrolyte pattern showed severe metabolic alkalosis with hypokalemia and hypochloremia consistent with abnormalities often seen in gastric outlet obstruction. Intravenous normal saline and potassium replacement was started, and a laparotomy was planned after correction of electrolyte abnormalities. At laparotomy, there were features suggestive of midgut malrotation with a 360° volvulus but preserved vascularity. The stomach and first part of duodenum were dilated, and the portal vein was seen to cross the first part of duodenum anteriorly, about 3 cm from the pylorus (Fig. 2). A Fogarty catheter inserted through the stomach to check intrinsic obstruction could not be passed beyond the area where the PDPV was crossing. A transverse duodenotomy was made in the first part of duodenum proximal to PDPV, and a longitudinal opening was made in the duodenum distally. A thick duodenal web with a small central hole was observed to obstruct the lumen. The ampulla of Vater was located on the distal side of the web. A diamond-shaped duodenoduodenostomy was performed anterior to the portal vein with a single layer of interrupted polyglactin sutures. A Ladd procedure was completed by lysis of adherent bands, widening the mesentery, appendicectomy, and placing the duodenum in the right paracolic gutter and cecum in the left upper abdomen. Postoperatively, parenteral nutrition was administered, and small volume of oral feeds were started on day 7 and gradually advanced. The baby was discharged uneventfully
Fig. 1 Contrast study showing partial duodenal obstruction; some contrast has passed distally beyond the abrupt cutoff. Note the cork screw appearance of the duodenum distal to the cutoff. Also some gas is seen in bowel loops distally.
A.K. Singal et al.
Fig. 2 Operative picture showing PDPV (arrow), distended stomach, and the first part of duodenum (stay sutures).
on day 12 on full feeds. At 1 year of follow-up, the child has remained asymptomatic.
2. Discussion Preduodenal portal vein is a rare entity, and less than 90 cases have been thus far reported in the literature [4]. Duodenal obstruction has been noted to be the most common clinical presentation in reported cases; although in less than half of these, PDPV was thought to be directly responsible for the duodenal obstruction. Most cases had associated anomalies that led to duodenal obstruction—duodenal atresia or web, annular pancreas, or anomalies of rotation and fixation [2-9]. Thus, it is prudent to rule out an intrinsic duodenal obstruction in all cases before PDPV is labeled as a cause for duodenal obstruction. Esscher [3] reported a case of PDPV and malrotation, in which initial surgery was unsuccessful, and on reexploration, a duodenal diaphragm was found to be the cause of duodenal obstruction. Other associated anomalies include situs inversus, polysplenia, and asplenia [2,4,6]. Preduodenal portal vein, when unrecognized, is at risk to injury during surgery in the vicinity such as during cholecystectomy, gastrectomy, portoenterostomy, and pancreatectomy. A vigilant surgeon can prevent these complications by recognizing this anomaly early. In the present case, the duodenal obstruction resulted from a duodenal web, which was present at the junction of the first and second part of duodenum causing a preampullary type of duodenal obstruction. This preduodenal obstruction is further evidenced by the presence of nonbilious vomiting and metabolic alterations reminiscent of pyloric stenosis. In our assessment, PDPV was not directly responsible for the duodenal obstruction, but its location at the site was unusual and posed technical problems. The preferred treatment of duodenal obstruction caused by PDPV is duodenoduodenostomy, as was done in the present case [5,6,9]. Duodenoduodenostomy in this
PDPV in association with midgut malrotation and duodenal web circumstance bypassed both the abnormalities—duodenal web as well as the PDPV. In the present report, there were 3 anomalies present concomitantly, and each one has a potential to cause duodenal obstruction independently. Furthermore, the metabolic abnormalities observed in the present case were reminiscent of pyloric stenosis because the preampullary obstruction leads to nonbilious vomiting of gastric secretions. Preduodenal portal vein should alert the surgeon to look for other associated anomalies that can lead to duodenal obstruction. Labeling PDPV as the obvious and apparent cause of obstruction may be misleading.
References [1] Knight HO. An anomalous portal vein with its surgical dangers. Ann Surg 1921;74:697-9.
E7
[2] Ziv Y, Lombrozo R, Dintsman M. Preduodenal portal vein with situs inversus and duodenal atresia. Aust Paediatr J 1986;22:69-70. [3] Esscher T. Preduodenal portal vein—a cause of intestinal obstruction? J Pediatr Surg 1980;15:609-12. [4] Mordehai J, Cohen Z, Kurzbart E, et al. Preduodenal portal vein causing duodenal obstruction associated with situs inversus, intestinal malrotation, and polysplenia: a case report. J Pediatr Surg 2002;37 (4):E5. [5] Georgacopulo P, Vigi V. Duodenal obstruction due to a preduodenal portal vein in a newborn. J Pediatr Surg 1980;15:339-40. [6] Kataria R, Bhatnagar V, Wadhwa S, et al. Gastric pneumatosis associated with preduodenal portal vein, duodenal atresia, and asplenia. Pediatr Surg Int 1998;14:100-1. [7] Fernandes ET, Burton EM, Hixson SD, et al. Preduodenal portal vein: surgery and radiographic appearance. J Pediatr Surg 1990; 25:1270-2. [8] Golombek S, Bilgi J, Ukabiala O. Duodenal web with preduodenal portal vein. Iowa Med 1995;85:247-9. [9] Choi SO, Park WH. Preduodenal portal vein: a cause of prenatally diagnosed duodenal obstruction. J Pediatr Surg 1995;30:1521-2.