Surgical Treatment of Abdominal Aortoiliac Aneurysms in Children: A Case Series and Systematic Literature Review

Abstract

Purpose

Abdominal aortoiliac aneurysms (AAIAs) in pediatric patients are very rare, but rupture is often fatal. This study aimed to analyze causes, surgical management strategies, and clinical outcomes in pediatric patients with AAIAs who underwent surgical treatment. We also performed a systematic literature review of previously reported cases to inform diagnostic and therapeutic approaches.

Materials and Methods

A retrospective case series analysis was conducted of consecutive pediatric patients (age <20 years) diagnosed with abdominal aortic or iliac artery aneurysms who underwent surgical treatment at Seoul National University Hospital from 1997 through 2025. A systematic literature review was performed according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. PubMed, Embase, and Web of Science were searched for publications from 1913 through 2025 using search terms including “child”, “pediatric”, “abdominal aortic aneurysm” and “iliac artery aneurysm”.

Results

Surgical treatment was successfully performed in five patients, with no perioperative mortality. During a median follow-up of 5 years, four patients were alive with stable outcomes and no aneurysm recurrence or ischemic complications. One patient with tuberous sclerosis complex died 5 years after surgery from seizure-related complications. One patient developed early graft thrombosis but remained asymptomatic due to good collateral circulation. The systematic literature review summarized 96 reported cases of pediatric AAIAs, showing diverse etiologies and surgical approaches and generally favorable long-term outcomes when appropriately managed.

Conclusion

Although rare, pediatric AAIAs carry a significant risk of rupture and death, requiring prompt diagnosis and surgical treatment. Experience from pediatric vascular reconstruction suggests that appropriate graft selection and anticipation of somatic growth are key for durable outcomes. Preservation of collateral circulation is crucial, as children demonstrate remarkable tolerance to graft occlusion. Further multicenter studies are needed to establish standardized management protocols for this rare condition.

INTRODUCTION

Abdominal aortoiliac aneurysms (AAIAs) in the pediatric population are extremely rare. The true incidence remains unknown because of the paucity of reports; only a limited number of cases have been reported in the medical literature [1]. The etiology and clinical manifestations differ from those in adults. These aneurysms typically present in infancy or early childhood and are often associated with underlying genetic conditions, particularly connective tissue disorders and tuberous sclerosis complex (TSC). The cause of pediatric AAIA remains poorly understood; proposed mechanisms include congenital weakness of the arterial wall, abnormal collagen synthesis, and developmental abnormalities of the vascular system [2]. Vasculitis, vascular malformation, trauma, and infection are additional causes. Unlike the degenerative aneurysms that predominate in adults, pediatric aneurysms represent a distinct entity linked to intrinsic arterial wall defects, with clinically relevant risks of progression and rupture during growth [3].

Management of pediatric AAIA often requires prompt surgical treatment because of the high risk of rupture, while the operative planning must account for the challenges posed by somatic growth [1,3]. Current surgical approaches include open repair with prosthetic grafting, patch angioplasty, and, in selected cases, endovascular techniques [4]. Strategies must account for pediatric anatomical and physiological factors, including vessel size, growth potential, and long-term graft function. The choice of graft material, whether synthetic or biological, requires careful consideration of durability, biocompatibility, and the potential for graft–vessel size mismatch as the child matures. Early surgical treatment has been associated with favorable clinical outcomes, including excellent long-term survival and low rates of graft-related complications, when appropriate surgical principles are followed [3].

Despite the clinical importance of pediatric AAIA, evidence for optimal management remains limited. The existing literature consists mainly of isolated case reports and small case series, which restricts the ability to establish evidence-based treatment protocols and predict long-term outcomes. The lack of standardized diagnostic criteria, treatment guidelines, and long-term follow-up data remains a major barrier to improving care. Furthermore, heterogeneity in underlying causes, anatomical presentation, and operative strategy across institutions complicates the development of unified management pathways. This study integrates institutional experience with a systematic literature review to generate practical guidance for the diagnosis and surgical management of pediatric AAIA.

MATERIALS AND METHODS

1) Patient selection and data collection

A retrospective analysis was performed of pediatric patients (aged <20 years) diagnosed with abdominal aortic or iliac artery aneurysms and treated surgically at Seoul National University Hospital from January 1997 through December 2025. Patients treated for ascending or thoracic aortic disease due to connective tissue disorder or vasculitis were excluded.

Clinical data were extracted from electronic medical records, including patient demographics, clinical presentation, imaging studies, underlying conditions, surgical interventions, perioperative outcomes, and long-term follow-up data. This study was approved by the Institutional Review Board of Seoul National University Hospital (H-2507-060-1656), with a waiver of informed consent because of the retrospective design.

2) Systematic literature review

A systematic review of the literature was conducted using PubMed, Embase, and Web of Science for publications from 1913 through December 2025. The Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) flow diagram is shown in Fig. 1. Of 717 records identified, 70 studies met the inclusion criteria after duplicate removal (n=203), title and abstract screening (n=376, excluded), retrieval assessment (n=9, not retrieved), and full-text eligibility assessment (n=59, excluded). The search strategy used the following key terms: “abdominal aortic aneurysm”, “iliac artery aneurysm”, and “pediatric” or “child”. Searches were limited to English-language publications. Additional articles were identified through manual review of reference lists from retrieved articles and relevant review papers. Case reports and case series of abdominal aortic or iliac artery aneurysms in patients aged 20 years or younger were included in the analysis. Patients with thoracic aortic aneurysms were excluded. Among abdominal aortic aneurysm (AAA) cases, those secondary to umbilical artery catheterization were also excluded.

Fig. 1.

PRISMA 2020 flow diagram depicting the study selection process of this systematic review evaluating pediatric abdominal aortoiliac aneurysms, with the inclusion of a total of 70 studies. PRISMA, Preferred Reporting Items for Systematic reviews and Meta-Analyses; AAA, abdominal aortic aneurysm; UAC, umbilical artery catheter

Descriptive statistics were used to summarize patient characteristics and outcomes.

RESULTS

1) Patient demographics and clinical presentation

During the study period, five children with AAIAs were treated surgically. The cohort included one male and four female patients, with a median age of 4 years. Patient demographic and clinical characteristics are summarized in Table 1. All patients presented with a pulsatile abdominal mass or had an aneurysm detected incidentally on examination; none had pain or clinical signs of rupture at presentation. Physical and developmental milestones were appropriate for age in all children, with no documented history of birth trauma, umbilical catheterization, or newborn infections.

Table 1.

Patient demographics

No.	Age	Sex	Location	Size (cm)	Etiology	Treatment	Outcome	Follow-up (y)
1a	8 months	F	Infrarenal AAA	3.1	TSC	PTFE 10 mm	Seizure, death	5
2	1 year	F	Infrarenal AAA	8.0	Congenital	Dacron 12 mm	Graft thrombosisb	5
3	11 years	F	Infrarenal AAA	8.5	TSC	Dacron 12 mm	Good	27
4a	4 years	F	Rt. CIA	4.6	Idiopathic	Aneurysmorrhaphy	Rt. EIA occlusionb	6
5	7 years	M	Lt. CIA	2.5	Idiopathic	Dacron 12-6 mm	Good	12

F, female; M, male; Rt., right; Lt., left; AAA, abdominal aortic aneurysm; TSC, tuberous sclerosis complex; PTFE, polytetrafluoroethylene; CIA, common iliac artery; EIA, external iliac artery.

^aPreviously reported in reference 6 and 22. ^bBoth patients remain asymptomatic with good collaterals.

Two patients had TSC, two were classified as idiopathic (no prenatal detection), and one had a congenital aneurysm detected during prenatal duplex ultrasonography (DUS). The two idiopathic cases may have been congenital AAAs; however, they were classified as idiopathic because there was no evidence of a perinatal aneurysm. In two patients, TSC was diagnosed during evaluation of infantile seizures, and AAAs were subsequently detected. Two patients with iliac aneurysms presented with a pulsatile abdominal mass.

2) Diagnostic evaluation and decision to operate

At our institution, pediatric AAIA is initially screened by DUS, which is preferred for its safety and absence of ionizing radiation. Computed tomography angiography (CTA) is subsequently performed for definitive anatomical assessment, including aneurysm size, location, extent, branch-vessel involvement, and the presence of mural thrombus or contained rupture. Three-dimensional reconstruction is routinely used to facilitate surgical planning. Magnetic resonance angiography is reserved for patients with contraindications to iodinated contrast or when repeated long-term surveillance is anticipated. Accordingly, all five patients in this series were evaluated using DUS and CTA for definitive diagnosis.

The timing of surgery is individualized based on aneurysm size relative to the adjacent normal aorta, growth rate on serial imaging, symptoms, underlying etiology, and patient age and body size. Indications for prompt surgical repair include symptomatic aneurysms, signs of impending rupture, rapid growth on serial imaging, or aneurysm diameter exceeding twice the adjacent normal aortic diameter. Asymptomatic small aneurysms are followed by DUS every 3 to 6 months with interval CTA. In the present series, the interval between diagnosis and surgery was less than 1 month in three patients. In Patient 2, prenatal DUS detected a 4.3 cm AAA; after 1 year of surveillance, the aneurysm enlarged to 8×8×7 cm, and surgical repair was performed (Fig. 2). In Patient 3, an AAA was detected at age 1 year, but surgical intervention was delayed for 11 years owing to initial family refusal.

Fig. 2.

Computed tomography angiography showed an infrarenal abdominal aortic aneurysm (8×8×7 cm) in a 1-year-old female (Patient 2 in Table 1).

Open surgical repair with prosthetic graft reconstruction is the preferred treatment, as endovascular options are limited by device sizing constraints and the inability of stent-grafts to accommodate somatic growth. Graft material and caliber are selected to match anticipated adult vessel size, and the graft length is configured with redundancy to permit longitudinal growth. Postoperatively, all patients undergo lifelong surveillance with DUS or CTA.

3) Surgical management

All five patients underwent open surgery under general anesthesia. Three patients with AAA received endoaneurysmal interposition using expanded polytetrafluoroethylene (ePTFE) or Dacron grafts (10 to 16 mm), selected to match pediatric vessel caliber and anticipated growth. One common iliac artery aneurysm (CIAA) was treated with interposition using a bifurcated Dacron graft (12×6 mm). The other CIAA was treated with aneurysm resection and aneurysmorrhaphy. This patient had external iliac artery (EIA) occlusion due to aneurysm compression. EIA revascularization was not performed because of severe adhesions and good collateral circulation. No endovascular procedures were performed.

4) Clinical outcomes and follow-up

All patients experienced an uneventful immediate postoperative recovery and were discharged in stable condition. Early graft thrombosis was detected in Patient 2, but the patient remained asymptomatic because of good collateral circulation (Fig. 3). Subsequent surveillance showed stable collateral flow without ischemic sequelae, and no reintervention was required.

Fig. 3.

Follow-up computed tomography angiography of the same patient (Patient 2 in Table 1) one year after surgery showed graft thrombosis with maintained collateral flow.

The median follow-up period was 5 years. Patient 1 died 5 years after surgery from seizure-related complications of TSC, while the graft remained patent without aneurysm recurrence. The four surviving patients showed excellent long-term outcomes with normal growth and development. No ischemic limb events, aneurysm recurrence, graft infection, or reintervention were observed during follow-up. Except for one patient with early graft thrombosis, regular surveillance with DUS or CTA showed stable graft function in all surviving patients.

5) Systematic review of the literature

① Iliac artery aneurysms

From 1913 to 2025, 34 pediatric patients (≤20 years) with isolated iliac artery aneurysms were identified, including the patients presented herein (Supplementary Table 1). Two were diagnosed prenatally, one at birth, seven in infancy, and 24 (71%) after 1 year of age. The disease was more common in females (n=19, 56%). The lesion was right-sided in 17 patients, left-sided in 14, bilateral in two, and unspecified in one. The common iliac artery (CIA) was the most frequently involved segment (n=28, 82%), followed by the internal iliac artery (IIA) (n=9) and EIA (n=4). Aneurysm morphology was saccular in 16 patients and fusiform in 14, and pseudoaneurysm was present in two patients; rupture was reported in three patients. Maximum diameter ranged from 2.5 cm to 15 cm. Among the three ruptured aneurysms, maximum diameter was unspecified in one patient and measured 5 cm and 9.3 cm in the other two. Maximum diameter was >3.5 cm in 20 patients (59%).

Regarding etiology, an idiopathic cause was most common (n=17, 50%), followed by infectious (n=6), congenital (n=4), inflammatory (n=3), and genetic (n=2) causes; etiology was not addressed in two cases. Aneurysms with prior umbilical artery catheterization or bacteremia were categorized as infectious. Inflammatory aneurysms included Takayasu arteritis, Kawasaki disease, and rheumatism.

The majority of patients underwent open surgical repair (n=28), most commonly aneurysm excision with or without primary reconstruction (n=19) or interposition or bypass grafting (n=9). Endovascular or hybrid approaches were reported in five recent cases, including three covered stents and two embolization procedures. Early postoperative outcomes were generally favorable; however, long-term follow-up data were inconsistently reported.

Two deaths were reported in ruptured cases. One patient with a ruptured aneurysm survived after an extensive operation (left CIA ligation, aorto-right common femoral artery interposition, and crossover femoro-femoral bypass with a 5 mm ePTFE graft).

Target lesion reoperation was performed in three cases: bleeding control, IIA ligation due to type 2 endoleak, and coil embolization after incomplete thrombin injection. Two late operations were performed for aneurysms at other sites (popliteal artery and contralateral IIA).

In two patients, EIAs were occluded preoperatively; however, they were not revascularized because of good collateral circulation to the affected limb. In one case of excision and CIA–EIA reanastomosis, the EIA occluded postoperatively; revascularization was not performed because of good collateral circulation. All three patients remained in good condition.

② Abdominal aortic aneurysms

From 1967 to 2025, 62 pediatric patients (≤20 years) with AAAs were identified from the literature (Supplementary Table 2). Patient age distribution showed that 21 patients (34%) were diagnosed in infancy (<1 year), including prenatal and neonatal cases. The disease was more common in males (n=33, 53%).

The infrarenal aorta was most commonly involved (n=45, 73%), followed by suprarenal (n=8, 13%), juxtarenal (n=4, 6%), pararenal (n=2, 3%), and supraceliac (n=1, 2%). Two cases were described as “abdominal” without further specification. Maximum diameters ranged from 1.3 to 11.0 cm; five patients presented with rupture at diagnosis.

The etiology was diverse. Idiopathic aneurysms were most common (n=26, 42%), followed by TSC (n=19, 31%), congenital (n=11, 18%), Takayasu arteritis (n=4, 6%), infectious (n=1, 1.5%), and other genetic conditions (n=1, 1.5%).

Most patients underwent surgical repair. Prosthetic graft interposition was the most common technique (n=34, 55%), using Dacron (n=19) or ePTFE (n=15). Primary repair (aneurysmorrhaphy or primary closure) was performed for 9 patients (15%). Cryopreserved arterial allografts were used for five patients (8%). Conservative management (observation or medical treatment alone) was used for 8 patients (13%), primarily in patients with severe comorbidities, spontaneous thrombosis, or prenatal or neonatal death.

Overall mortality was 19% (n=12). Causes of death included rupture-related complications (n=5), comorbidities (n=6; heart failure, pulmonary hypertension, sepsis, renal failure, bleeding, or aortic dissection), and unspecified causes (n=1). Among survivors, 36 (73%) had favorable long-term outcomes, with follow-up periods ranging from 1 month to 13 years. Late complications occurred in seven patients (11%), including graft thrombosis (n=3), arterial stenosis or pseudoaneurysm formation (n=1), ischemic colitis from arterial thrombosis (n=1), reoperation for a suture-site aneurysm (n=1), and a new thoracoabdominal aortic aneurysm requiring surgery (n=1). Notably, patients with graft thrombosis remained asymptomatic due to adequate collateral circulation.

DISCUSSION

Pediatric AAIAs represent a varied group of vascular abnormalities with different causes. In our series, AAIAs were associated with TSC in two patients, idiopathic aneurysms in two patients, and a congenital aneurysm in one. This distribution is consistent with the literature, where TSC represents one of the most common identifiable causes of pediatric AAIA. The association between TSC and aortic aneurysms was first described by Hagood et al. [5] in 1976. Subsequent reports have highlighted the aggressive nature of these aneurysms, with rapid progression and high rupture rates [6].

The pathogenesis of congenital AAIA formation remains poorly understood. Proposed mechanisms include intrinsic arterial wall weakness, abnormal collagen synthesis, and developmental vascular anomalies. Eliason et al. [3] classified pediatric AAA into four groups: idiopathic-congenital, genetic, infectious-inflammatory, and traumatic. Sarkar et al. [7] reported 165 arterial aneurysms in children and proposed a clinicopathological classification of nine etiologies: infection, giant cell aortoarteritis, autoimmune connective disease, Kawasaki disease, medial degeneration in Marfan and Ehlers–Danlos syndrome, other forms of noninflammatory medial degeneration, arterial dysplasia, congenital-idiopathic, and traumatic false aneurysm.

Herein, we propose classifying the etiology more precisely into six categories: congenital, idiopathic, genetic, infectious, inflammatory, and traumatic. Congenital refers to AAIAs that are detected prenatally or present in the neonatal period without known causes [1]. Idiopathic aneurysm has no known cause and are usually detected in infancy or childhood [8]. Genetic AAIAs include those associated TSC, Marfan syndrome, Ehlers–Danlos syndrome, and Menkes disease. Infectious AAIAs in children are often associated with umbilical artery catheterization [9] or cardiac catheterization [10]. Inflammatory AAIA is associated with polyarteritis nodosa, Takayasu arteritis, giant cell arteritis or Kawasaki syndrome [11]. Traumatic AAIAs include iatrogenic catheterization injury or injury during birth [9].

Given the rarity of pediatric AAAs, their natural history is poorly characterized, and growth rates and rupture risk factors remain unclear. However, the aggressive nature of these aneurysms is particularly concerning, with unpredictable growth patterns and a high risk of rupture; this supports prompt surgical intervention after diagnosis [3,12,13]. This is exemplified by Patient 2, in whom the aneurysm nearly doubled from 4.3 cm to 8 cm within 1 year, underscoring the importance of early surgical intervention. Rupture has been reported in untreated AAAs and in stillborn infants [14,15].

The most common clinical presentation was a pulsatile abdominal or inguinal mass, which was present in all patients in our series. None had symptoms of rupture at initial presentation, contrasting with reported cases in which rupture was the first manifestation [8,16,17]. This emphasizes the importance of early detection and elective repair before life-threatening complications develop.

Our surgical approach was individualized based on patient age, aneurysm size, and anatomical considerations. Prosthetic grafts (Dacron or ePTFE) were used and showed excellent durability, except for one early thrombosis. We did not use autogenous or biologic grafts, which may be valuable in infected fields [18]. Eliason et al. [3] reported that they favored ePTFE over Dacron grafts because of frequent post-implantation dilatation of Dacron grafts. In some cases, allografts have been used; however, long-term results are unknown, with possible risks of graft deterioration and pseudoaneurysm formation [8,19]. Kaye et al. [20] reported that children younger than 6 years rarely have adequate autologous options for graft construction and benefit from the use of cryopreserved arterial allografts.

The graft-sizing strategy (10 to 16 mm for aortic segments) was designed to accommodate future growth in diameter while maintaining adequate blood flow. In addition, graft length should be redundant to allow future longitudinal growth, as described elsewhere [21]. Barral et al. [12] recommended technical measures to compensate for the traction force applied to the graft due to normal vertebral growth. The first involves reinforcing the anastomoses externally with a Dacron band. The second measure is to oversize the length of the grafts, taking care not to kink the graft or cause stenosis. The angulation of the graft decreases as the patient grows. Kaye et al. [20] recommended that artificial grafts be placed in a redundant C-shape to allow growth, with interrupted sutures for circumferential growth.

Graft thrombosis in Patient 2 provides insight into physiological factors that may benefit pediatric patients. Despite complete graft occlusion, the patient remained asymptomatic, with normal lower extremity function and growth. This tolerance to vascular occlusion has been documented previously. Barral et al. [12] reported better outcomes in children than in adults after graft complications. Smaller vessel caliber, shorter limb length, and lower metabolic demands of peripheral tissues in children reduce the hemodynamic burden on collateral circulation. At 1-year follow-up, the patient participated in normal activities without claudication. This suggests that graft thrombosis in pediatric patients may have different clinical implications than in adults, where such complications typically necessitate urgent reintervention.

Preoperative CTA of Patient 4 showed EIA occlusion due to compression from a thrombosed aneurysm [22]. Miyamoto et al. [23] reported EIA occlusion due to a chronic contained rupture of a CIA aneurysm, similar to our case. EIA reconstruction was not performed because of severe adhesions and well-developed collateral circulation. As in Patient 2 and Patient 4, major arterial occlusion is well compensated in children because of robust collateral development. Therefore, high-risk revascularization is unnecessary, and preservation of collateral circulation is crucial during the initial operation.

Patient 3 represents one of the longest reported follow-up periods after pediatric aortic aneurysm repair (27 years). It demonstrates the potential for excellent long-term outcomes with appropriate surgical management and durable synthetic graft reconstruction in children. The patient has successfully transitioned through adolescence into adulthood without graft revision or growth-related complications.

The role of endovascular aneurysm repair (EVAR) in pediatric AAA is very limited. Due to the fixed geometry of the stent-grafts, EVAR is generally not appropriate for growing children. It may be considered only in older adolescents in whom further growth is unlikely or as a bridging procedure before definitive open surgery in critically ill children [24]. Moreover, the long-term durability of endografts is unknown, and lifelong imaging surveillance is mandatory after EVAR. Therefore, endovascular treatment of pediatric AAIAs should be limited to selected patients.

Proprotein Convertase Subtilisin/Kexin Type 9 (PCSK9) has been implicated in AAA pathogenesis through medial degeneration and smooth muscle cell apoptosis [25]. Given the clinical availability of PCSK9 inhibitors, their potential application in preventing pediatric AAIA progression merits future study [26].

The primary limitation of our study is the small sample size, inherent to the condition’s rarity. Additionally, the lack of standardized diagnostic criteria and treatment protocols limits direct comparisons. Postrevascularization medication was not standardized and depended on surgeon discretion. Larger multi-institutional prospective studies with comprehensive long-term follow-up are needed to better characterize the natural history of pediatric AAIAs, optimize surgical techniques, establish standardized protocols, and improve patient outcomes.

CONCLUSION

AAIAs in children, while rare, require prompt diagnosis and individualized surgical management. Our experience shows that excellent long-term outcomes can be achieved with early identification, appropriate surgical technique, and careful graft selection. The association with TSC warrants comprehensive screening in affected patients. Continued research and multicenter collaboration are essential to further refine management strategies and improve outcomes for these challenging cases.

SUPPLEMENTARY MATERIALS

Supplementary data can be found via https://doi.org/10.5758/vsi.260024

Funding Statement

FUNDING None.