Abstract
Aim of the study
To present a case series of four pediatric patients with PDPV, each with a different clinical presentation and surgical management.
Methods
We retrospectively reviewed four cases of PDPV managed at our institution. Two cases were associated with extrahepatic biliary atresia (EHBA) and discovered incidentally during surgery. The other two cases presented with duodenal obstruction but had differing etiologies and management approaches.
Results
Two patients with EHBA underwent successful Kasai portoenterostomy; PDPV was discovered intraoperatively and required no intervention. One patient had duodenal obstruction due to PDPV compressing the duodenum and underwent laparoscopic duodenoduodenostomy. Another patient had duodenal obstruction due to malrotation; a Ladd’s procedure was performed. PDPV was noted intraoperatively but was not the obstructing factor.
Conclusion
PDPV can present variably, ranging from an incidental finding to a causative factor in duodenal obstruction. Its recognition is crucial during abdominal surgery to avoid inadvertent injury and to tailor the surgical approach based on associated anomalies.
Keywords: Preduodenal portal vein, Kasai, Duodenoduodenostomy
Introduction
Preduodenal portal vein (PDPV) is a rare embryologic vascular anomaly characterized by an anomalous anterior course of the portal vein across the duodenum. It is frequently associated with other congenital abnormalities, including duodenal atresia, malrotation, biliary atresia, polysplenia, and heterotaxia syndrome [1]. PDPV may be discovered incidentally or present with obstructive symptoms, depending on its anatomical relationship to surrounding structures. Early recognition is vital for appropriate surgical management, particularly during procedures such as the Kasai operation or duodenal bypass surgery.
Methods
Case presentations
Cases 1 & 2: PDPV with Extrahepatic Biliary Atresia (Fig. 1)
Fig. 1.
PDPV associated with EHBA
Both patients presented with neonatal jaundice and pale stools. Liver biopsy confirmed EHBA. During laparotomy for Kasai portoenterostomy, PDPV was discovered coursing anterior to the duodenum. The vein was not causing obstruction and was left intact. Both patients tolerated the procedure well with no complications related to PDPV.
Case 3: PDPV Causing Duodenal Obstruction
A newborn presented with bilious vomiting. Imaging suggested duodenal obstruction. During diagnostic laparoscopy, a PDPV was found compressing the second part of the duodenum. A laparoscopic duodenoduodenostomy was performed anterior to the PDPV (Figs. 2, 3). The patient recovered well with resolution of symptoms.
Fig. 2.
Arrow showing PDPV
Fig. 3.
Laparoscopic duodenoduodenostomy
Case 4: Duodenal Obstruction Due to Malrotation with Incidental PDPV (Fig. 4)
Fig. 4.
PDPV associated with malrotation
Another neonate presented with features of duodenal obstruction. Laparoscopic exploration revealed malrotation; the procedure was converted to open surgery. Ladd’s bands compressing the duodenum were found and divided. A PDPV was also noted but was not the cause of obstruction. Ladd’s procedure was completed successfully, and the PDPV was preserved. Table 1.
Table 1.
Different presentations of PDPV
| Case | Presentation | Diagnosis | Type of operation | Age at operation | Birth weight | Operative time | Complication | Hospital stay |
|---|---|---|---|---|---|---|---|---|
| 1 | Clay-colored stool, jaundice | EHBA | Kasai | 2 months | 5 kg | 3 h | No | 1 week |
| 2 | Clay-colored stool, jaundice | EHBA | Kasai | 2 months | 6 kg | 3 h | No | 1 week |
| 3 | Bilious vomiting | Duodenal obstruction due to PDPV | Duodenoduodenostomy | 5 days | 4 kg | 2 h | No | 4 days |
| 4 | Bilious vomiting | Duodenal obstruction due to malrotation | Ladd’s Procedure | 6 days | 3.5 kg | 2 h | No | 5 days |
Results
Discussion
Preduodenal portal vein (PDPV) was first described by Knight in 1921 [1]. It is a rare anomaly, with only around 100 cases reported across different age groups; 29 cases have been documented in adults [2, 3, 8]. PDPV is characterized by anterior positioning of the portal vein relative to the duodenum, due to abnormal embryologic development—specifically, persistence of the ventral anastomosis of the vitelline veins and regression of the dorsal one.
Our case series highlights the variability in clinical presentations and surgical implications associated with PDPV. Ishizaki et al. [2] and Ooshima et al. [3] noted that approximately half of PDPV cases are asymptomatic and found incidentally, which aligns with our experience. In our series, two cases were incidentally discovered during surgery for EHBA.
The use of laparoscopic approaches in our cases mirrors current trends in neonatal surgery, with benefits including faster recovery and improved cosmetic outcomes.
The association between PDPV and EHBA, though uncommon, has been reported. Yamagiwa et al. [5] described a similar case during a Kasai operation, emphasizing the importance of careful dissection. In our two EHBA cases, PDPV was noted intraoperatively but did not interfere with portoenterostomy.
In Case 3, PDPV was the primary cause of duodenal obstruction. A laparoscopic duodenoduodenostomy was successfully performed, consistent with reports by Georgacopulo et al. [6]. Again, laparoscopy proved effective for both diagnosis and treatment.
In Case 4, PDPV was incidentally discovered during a Ladd’s procedure for malrotation. As seen in studies by Mordehai et al. [8] and Rusu et al. [9], PDPV in such settings may not be the obstructing element, and the primary surgical correction should address the actual cause of obstruction.
Yoon et al. [10] reported that preoperative imaging such as Doppler ultrasound or contrast-enhanced CT may help identify PDPV, although this is not always feasible in neonates. In our cases, PDPV was diagnosed intraoperatively, reinforcing the importance of surgical awareness and careful dissection.
Preoperative diagnosis remains a challenge. Routine imaging may miss PDPV, and contrast studies can be nonspecific. However, high suspicion and meticulous evaluation of imaging studies may lead to early recognition, especially when associated anomalies are present.
From a surgical perspective, early identification of PDPV is essential to avoid injury. Surgeons should remain vigilant for anomalous vascular courses, particularly in the context of duodenal obstruction or EHBA, to ensure safe and effective management.
Conclusion
PDPV is a rare but clinically relevant anomaly, especially when associated with duodenal obstruction. Early intraoperative identification is vital for avoiding complications. Surgeons should maintain a high index of suspicion, particularly when other congenital anomalies are present.
Acknowledgements
We express our deepest gratitude to our colleagues in pediatrics in Cairo University Hospitals.
Clinical trial number
Not applicable.
Disclosure
The authors report no conflict of interest in the materials or methods used in this study or the findings specified in this manuscript.
Abbreviations
- EHBA
Extrahepatic biliary atresia
- PDPV
Preduodenal portal vein
Authors’ contributions
All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by [Ahmed Arafa], [Ahmed S. Ragab] and [Abdelhalem Showkat Mohamed]. The first draft of the manuscript was written by [Abdelhafeez Mohamed Abdelhafeez] and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.
Funding
Open access funding provided by The Science, Technology & Innovation Funding Authority (STDF) in cooperation with The Egyptian Knowledge Bank (EKB). The authors received no financial support for the research, authorship, and/or publication of this paper.
Data availability
The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.
Declarations
Ethics approval and consent to participate
This research was performed at the Pediatric Surgery Department, Faculty of Medicine, Cairo University, Cairo, Egypt. Ethical committee approval and written informed consent were obtained. IRB Number: N347-2023. For children under 16 years, consent was obtained from parents.
Consent for publication
Written informed consent was obtained from the parents of all patients for publication of clinical details and images.
Competing interests
The authors declare no competing interests.
Footnotes
Publisher’s Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
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Associated Data
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Data Availability Statement
The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.