Robotic-assisted laparoscopic transposition of the left renal vein with three-dimensional image reconstruction for treatment of pediatric nutcracker syndrome: a case description and literature analysis
Introduction
Nutcracker syndrome, also known as “left renal vein compression syndrome”, is a clinical condition resulting from the compression of the left renal vein (LRV) between the abdominal aorta and the superior mesenteric artery (1,2). This syndrome manifests in both pediatric and adult populations, with a slightly higher incidence in females (2,3). Pediatric nutcracker syndrome (PNS) commonly presents with nonglomerular hematuria. According to the recent literature, the prevalence of hematuria in pediatric cases ranges from 69% to 100% (4,5), and in recent systematic reviews, abdominal pain has also been reported in approximately 14.3% to 19.1% of pediatric cases (4,5). Other symptoms may include fatigue orthostatic proteinuria or varicocele in males (6).
Recent advancements in diagnostic techniques and heightened clinical awareness have led to an increased recognition of PNS (7). Consequently, pediatric specialists worldwide are paying more attention to this condition, emphasizing the importance of accurate diagnosis and effective treatment strategies (8). In this context, we present a case study of PNS, followed by a discussion on clinical management approaches and prognostic considerations for children affected by this syndrome.
Case presentation
A 13-year-old female patient presented with a chief complaint of intermittent flank pain that had persisted for 6 years. The patient’s height was 165 cm, and her weight was 40 kg. She had previously sought medical attention multiple times for intermittent left flank pain and was diagnosed with nutcracker syndrome 2 years prior. The pain had worsened and was accompanied by hematuria in the previous month after a common cold. She underwent a series of investigations: hepatic, biliary, pancreatic, splenic, and urinary ultrasonography; right abdominal ultrasound; gastrointestinal endoscopy; and C13 breath testing. However, the findings from all of these tests were unremarkable. She was then referred to The Seventh Medical Center of Chinese PLA General Hospital, where abdominal computed tomography (CT) and pelvic ultrasonography again failed to reveal any abnormalities. It was only after renal vein ultrasonography was performed that compression of the LRV was detected, which was consistent with nutcracker syndrome. On initial examination, the patient had no urinary irritation, fever, night sweats, or other systemic symptoms. Urinalysis revealed the presence of hematuria, and further imaging studies were conducted to investigate the underlying cause. Duplex ultrasound examination demonstrated the presence of the nutcracker phenomenon, revealing compression of the LRV between the superior mesenteric artery and the abdominal aorta. The distal diameter of the LRV was found to be 9.1 mm, with significant narrowing of the inner LRV measuring 1.2 mm due to compression. Additionally, the angle between the abdominal aorta and the superior mesenteric artery was measured to be 18°, which is 53° less than the average angle of females, highlighting the significance of this anatomical narrowing (9). According to the most recent consensus on nutcracker syndrome, a diagnosis can be considered when the aortomesenteric angle is less than 30° (10). CT was also performed to obtain a more detailed visualization of the vasculature (Figure 1A,1B), with which we obtained a 3D reconstruction of the vasculature via VitaWerks software (San Francisco, CA, USA) (Figure 1C). The effect of a narrow angle between the superior mesenteric artery and aorta compressing the LRV was clearly depicted, and when considered in conjunction with the patient’s characteristic symptoms, this prompted the diagnosis of nutcracker syndrome.
Given the persistent flank pain that severely impaired the patient’s daily activities combined with the strong preference for surgery from by herself and her family, robotic-assisted laparoscopic transposition of the LRV was performed. Its success supports the effectiveness of minimally invasive surgical techniques in managing PNS. The surgeon was an experienced pediatric urologist with prior experience in complex pediatric surgeries involving vascular management, such as robotic-assisted pediatric renal transplantation (11) and robotic-assisted laparoscopic management of Wilms’ tumor with vena cava thrombi (12). The da Vinci Surgical System (Intuitive Surgical, Sunnyvale, CA, USA) was used for this procedure. The patient was placed in the lithotomy position with the right side elevated for optimal access (Figure 2A). Trocar placement was performed as follows: The camera port was established 3 cm below the umbilicus with an 8.5-mm trocar. The first and third robotic arms were inserted through 8-mm trocars placed 6 cm lateral to the left and right sides of the camera port, respectively. The fourth robotic arm was inserted with an 8-mm trocar placed at the intersection of the right midclavicular line and the bottom edge of the rib cage. Four assistant ports were positioned to facilitate the procedure. Two 12-mm trocars were placed between the camera port and the first robotic arm, as well as between the camera port and the third robotic arm. For the third assistant port, a 5-mm trocar was positioned at the intersection of the right anterior axillary line and the bottom edge of the rib cage.
The patient underwent robotic-assisted laparoscopic transposition of the LRV (Figure 2B). After the instrument arms were docked, the bowel was mobilized to expose the surgical field. The peritoneum and vascular sheath were opened to access the inferior vena cava (IVC), and a fan-shaped suspension of the mesenteric root peritoneum was applied to optimize exposure via sutured Hem-o-lok clips. The IVC was dissected proximally toward the subhepatic region. The lumbar vein and left adrenal central vein were ligated. Both renal veins and the left renal artery were clearly exposed. Temporary vascular occlusion was achieved via a combination of laparoscopic bulldog clamps and Hem-o-lok clips. Specifically, laparoscopic bulldog clamps were applied to the distal vessels, including the left renal artery, the distal IVC, the right renal vein, and the distal portion of the LRV. Meanwhile, Hem-o-lok clips were used to occlude the proximal IVC above the LRV, ensuring secure and stable control of vascular flow during the procedure. This allowed for a safe and bloodless field for the transection and reanastomosis of the LRV. The surgical approach also involved the ligation of lumbar vein and the left adrenal central vein. During this process, a side-biting clamp was applied to the IVC at the confluence with the LRV. The LRV was transected with a small cuff of IVC remaining and then reanastomosed to the left lateral aspect of the IVC in a more distal position. Meticulous care was taken to preserve the integrity of the vascular wall and prevent any inadvertent injury. This was achieved in a tension-free, end-to-side manner through use of continuous sutures of 4-0 polypropylene. The proximal opening in the IVC was oversewn with continuous sutures of the same material. The LRV was then transposed anterior to the aorta and secured in its new position with additional Hem-o-lok clips for stabilization. Finally, the vascular sheath and peritoneum were closed to complete the procedure, and the integrity of the vein’s new route was confirmed through intraoperative ultrasound.
The procedure was completed successfully with an estimated blood loss of 400 mL. The total operative time was 180 minutes, including 35 minutes of IVC occlusion. The patient was managed with a postoperative drain, which was removed on postoperative day 3. She was discharged on postoperative day 7 in stable condition.
At the 1-month follow-up, the patient reported complete resolution of hematuria and abdominal pain. Postoperative ultrasound showed that inner diameter of the LRV was approximately 3.1 and 6.0 mm at its distal end, confirming that the LRV was free of stenosis or compression (Figure 2C). The patient expressed high satisfaction with the surgical approach and noted significant improvement in her quality of life.
All procedures performed in this study were in accordance with the ethical standards of the institutional research committee, and with the Helsinki Declaration and its subsequent amendments. Written informed consent was obtained from the patient’s legal guardians for publication of this article and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.
Discussion
Management options for nutcracker syndrome range from conservative observation to endovascular stenting or open surgery, as demonstrated in Table 1, with the approach determined by the severity of symptoms and local expertise (25). Conservative observation is often considered the first-line treatment, as spontaneous remission may occur during growth due to the development of fat tissue around the LRV or the establishment of collateral circulation. Symptomatic relief with medications such as analgesics for abdominal pain or angiotensin-converting enzyme inhibitors for proteinuria is frequently applied during this observation period (26). Miró et al. examined 21 pediatric patients with nutcracker syndrome managed over 18 years and reported that 76.2% of patients showed resolution of symptoms with conservative measures alone (5). Similarly, Akdemir et al. observed complete symptom improvement in 123 out of 123 pediatric patients managed conservatively over a 10-year period, highlighting the potential efficacy of nonsurgical treatment in this population (8). The duration of conservative management typically ranges up to 24 months in pediatric patients before surgical intervention is considered, especially when symptoms persist or worsen (27). In our case, the patient also attempted to gain weight, which is often recommended to increase retroperitoneal fat and potentially relieve LRV compression. However, she remained thin. This may be explained by gastrointestinal malfunction, which is commonly observed in this population and may interfere with digestion and nutrient absorption (28). According to the cohort study of 21 pediatric patients with nutcracker syndrome, the average body mass index (BMI) was only 18.2 kg/m2, suggesting that this population tends to be underweight (5).
Table 1
| Study | Follow-up time |
Total patients | Patients with clinical improvement | Management |
|---|---|---|---|---|
| Akdemir I, et al. (8) | 10 years | 123 | 123 | Conservative management |
| Miró I, et al. (5) | 52.3 months | 21 | 16 | Conservative management |
| Reed N, et al. (13) | 10 years | 11 | 11 | Left renal vein transposition |
| Dieleman F, et al. (14) | 10 years | 11 | 10 | Left renal vein transposition |
| Chau A, et al. (15) | 3 months | 3 | 3 | Robotic-assisted left renal vein transposition |
| Wu Z, et al. (16) | 10 years | 75 | 70 | Endovascular stenting |
| Hartung O, et al. (17) | 2 years | 5 | 5 | Endovascular stenting |
| Wang SZ, et al. (18) | 4 years | 13 | 12 | Laparoscopic extravascular stent placement |
| Manzano JP, et al. (19) | 30 months | 1 | 1 | Robot-assisted extravascular stent placement |
| Sarikaya S, et al. (20) | 23 months | 19 | 15 | Left renal vein transposition [14] and stenting [5] |
| Philip JL, et al. (21) | 1 year | 101 | 94 | Kidney auto-transplant |
| Mejia A, et al. (22) | 5 years | 29 | 28 | Robotic-assisted kidney auto-transplant |
| Kawaguchi N, et al. (23) | 16 months | 1 | 1 | Balloon angioplasty |
| Wang H, et al. (24) | 42 months | 1 | 1 | Three-dimensional-printed extravascular titanium stent |
However, in severe cases presenting with persistent flank pain that is unresponsive to medication and recurrent hematuria impairing kidney function or leading to anemia, more invasive treatment options may be necessary (14). These include endovascular stenting or surgical interventions such as LRV transposition and renal autotransplant (19,20). Endovascular techniques, while effective in adults, present challenges in children due to complications such as postoperative thrombosis and stent migration during growth (27), with the latter being the most severe. Wu et al. reported a 6.7% incidence of stent migration in 5 of 75 patients over a mean follow-up of 55 months. Notably, the migration resulted in two stents reaching the right atrium and one stent becoming completely prolapsed into the IVC, necessitating open cardiac surgery for retrieval (16). Hartung et al. also documented perioperative stent migration in their early experience with five patients, with one case involving migration into the IVC that required additional endovascular intervention. They also noted late migrations leading to recompression of the LRV and recurrence of symptoms, which clearly illustrates the chronic challenge of stent stability and the necessity for vigilant postoperative monitoring (17).
Recent studies have suggested that renal autotransplant can provide durable pain relief and reduced chronic opioid use in cases of PNS. However, it is typically only considered for patients who are not candidates for endovascular interventions or LRV transposition or for those seeking a definitive cure (21). Renal vein transpositions can also lead to restenosis, which might be attributed to persistent external pressure on the distal superior mesenteric artery or tension from the aorta on the posterior wall of the transplanted vessel, as well as restenosis due to intimal hyperplasia or thrombosis (4). To complicate matters, a standard diagnostic and treatment algorithm are currently lacking for this condition, and scientific evidence for pediatric cases remains limited.
In our case, due to the patient’s persistent and worsening flank pain that was unresponsive to conservative measures including aspirin intake for 2 years, we opted for surgical treatment. To mitigate the risks of stent migration due to patient growth and to provide a minimally invasive procedure, robotic-assisted laparoscopic renal vein transposition was applied. The renal vein transposition method, which was first proposed by Grant in 1938 and clinically reported on by Mina and El-Sadr in 1950 (29,30), has seen significant advancements in its management through robotic-assisted laparoscopic approaches. For instance, in 2019, Yu et al. reported a series of three patients who underwent robotic-assisted laparoscopic LRV transposition; in all three cases, symptoms of hematuria and flank pain were resolved, with no major perioperative complications, supporting the safety and efficacy of this approach (31). Similarly, Chau et al. described the treatment of a 19-year-old woman using the same surgical method, which resulted in complete resolution of symptoms in follow-up (15). Another study conducted by Wang et al. applied this method in a 26-year-old male, which also showed successful resolution of symptoms pos-operatively (32). These cases collectively attest to the potential of robotic-assisted laparoscopic transposition in effectively managing nutcracker syndrome with minimal complications and rapid recovery times, indicating it as a viable option especially when traditional treatments pose higher risks or are ineffective (32).
These early outcomes, involving no perioperative complications and complete resolution of abdominal pain, are encouraging. Nevertheless, longer-term follow-up is necessary to assess the durability of symptom relief and the long-term patency of the transposed vein.
The integration of 3D imaging technologies into surgical practice for pediatric cases represents a transformative advance (33). These innovations allow for the detailed visualization and precise mapping of anatomical structures, which may assist with surgical planning and execution (34). For instance, in hepatic separation of conjoined twins, 3D models of the liver and surrounding vasculature can help surgeons plan the resection margins and vascular anastomoses more accurately, thus reducing operative time and improving patient outcomes (35). Furthermore, 3D models have also been used to monitor tumor volume during chemotherapy (36) and adjust the target irradiation volume (37) in the therapy of pediatric tumors. As augmented reality continues to evolve, it may have the potential to overlay digital information onto the physical real world, potentially facilitating surgical navigation (38).
Postoperative anticoagulation therapy for each patient is also needed, as prolonged compression of the LRV may cause permanent distortion of the vein even after transposition (39). In such cases, administration of dual-antiplatelet therapy for 1 to 3 months postoperatively is also recommend, followed by the lifetime intake of low-dose aspirin (81 mg) (2). For our patient, we prescribed 2 months of apixaban, after which anticoagulation was discontinued without any complications. However, the antiplatelet therapy with 100 mg of aspirin was continued for 6 months.
In addition, the transposed LRV may undergo elongation at its new site due to the aortic protrusion pushing it forward (40). Thus, complications such as bleeding and vessel thrombosis may occur. Although children are reported to have good results, careful attention to these complications is warranted (41). In light of these challenges, the medical community continues to seek improvements in both the surgical and postoperative management of patients. With a growing number of minimally invasive therapies being explored, such as balloon angioplasty of the LRV, additional clinical trials on larger cohorts of children and multicenter clinical trials will become increasingly necessary (23).
Conclusions
Robot-assisted surgery represents a promising solution for PNS, providing significant advantages over traditional surgical approaches. This method enhances surgical precision and control, reduces the invasiveness of procedures, and offers superior visualization, which are crucial in pediatric psychological care. Further data will assist in the development of optimal treatment algorithms.
Acknowledgments
None.
Footnote
Funding: This work was supported by
Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://qims.amegroups.com/article/view/10.21037/qims-2025-260/coif). The authors have no conflicts of interest to declare.
Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional research committee, and with the Helsinki Declaration and its subsequent amendments. Written informed consent was obtained from the patient’s legal guardians for publication of this article and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.
Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.
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