Abstract
Background
The aberrant right subclavian artery (ARSA) is the most common anomaly of the arch of aorta and is significantly associated with a non-recurrent laryngeal nerve (NRLN). Nevertheless, the co-occurrence of ARSA with complex thoracic wall vascular remodeling is scarcely described in the current literature. This exploration describes an unusual group of neurovascular variations with important surgical significance.
Case presentation
A precise dissection was performed on a 43-year-old female donor of Thai descent embalmed with soft-embalming technique with vascular silicone-color injection. The cervical and upper thoracic regions were dissected with emphasis on neurovascular pattern, and morphometric measurements. A retroesophageal ARSA was found to be the fourth branch of the aortic arch after the left subclavian artery, without a Kommerell diverticulum. A right-sided NRLN was confirmed to arise directly from the vagus nerve to then travel upwards next to the larynx. Crucially, the right internal thoracic artery (ITA) was completely absent, and its usual vascular territory was replaced by the superior intercostal artery (SIA) arising from the inferior aspect of the ARSA. Moreover, a separate muscular branch of the proximal ARSA was observed to provide blood supply to the left longus colli muscle.
Conclusion
The current report describes a novel association of ARSA, NRLN, complete absence of the right ITA with a compensatory SIA and a muscular branch from the ARSA to the left longus colli muscle. This research emphasizes the embryological connection of the development of the cervical and thoracic vessels and indicates that thorough preoperative imaging is required to prevent any iatrogenic injury during surgery of the thyroid gland, cervical spine, esophagus and coronary bypass surgeries.
Keywords: Aberrant right subclavian artery, Non-recurrent laryngeal nerve, Absent internal thoracic artery, Superior intercostal artery, Surgical anatomy
Background
An aberrant right subclavian artery (ARSA), also referred to as arteria lusoria, represents the most common abnormality of the aortic arch, occurring in approximately 0.5-2% of the population, usually appearing as the fourth branch of the arch located after the left subclavian artery [1–3]. Based on the anatomical pathway, the ARSA is classified into three distinct variants. The most common route is retroesophageal, occurring in approximately 83% of cases [3]. Less frequently, the artery can pass between the trachea and the esophagus in approximately 16.7% of patients or take a pre-tracheal course anterior to the trachea in approximately 5% of cases [3, 4]. The retroesophageal course can lead to clinical symptoms such as dysphagia lusoria, dyspnea, cough, weight loss, and retrosternal pain. These symptomatic presentations primarily arise from the direct compression of adjacent structures, aneurysmal dilation, vessel stiffness, and age-related vascular alterations [5].
ARSA is also strongly linked to the presence of a non-recurrent laryngeal nerve (NRLN) [6], a rare but clinically significant nerve variant that is encountered in approximately 0.51% of thyroid and parathyroid surgeries. Finding this nerve before or during surgery reduces the chances of vocal cord palsy [7, 8]. Another variation of surgical relevance is the presence of arterial muscular branches supplying the deep prevertebral musculature, especially the left longus colli muscle. Surgeons must be aware of these differences to prevent accidentally damaging blood vessels, especially when cutting through the longus colli muscles and prevertebral fascia. Understanding the expected and variant anatomy surrounding the longus colli muscles is essential for minimizing complications [9, 10].
In addition to the ARSA, the different types of cervical branches of the subclavian artery also affect the safety of surgery. Unusual origins of the vertebral, inferior thyroid, and internal thoracic arteries (ITA) may alter the connections between blood vessels during neck dissections, anterior cervical spine surgery, and thoracic inlet approaches [11, 12]. An additional clinically important but underreported association is the complete absence of the right ITA, a conduit widely used in coronary artery bypass grafting. Its absence may complicate intraoperative graft selection and indicates altered subclavian branching patterns [13, 14]. A previous study indicated that ARSA is associated with an abnormal bilateral origin of the ITA from the third part of the subclavian artery, as well as the simultaneous presence of an atypical common origin of the deep brachial artery with the right subscapular artery [3]. A difference in the branching of the ITA is observed on the lateral thoracic wall, along with the presence of the third part of the subclavian artery [15, 16]. The variation of the ITA coursing along the posterior thoracic wall in this ARSA case has not been reported.
Although previous studies have individually described ARSA, NRLN, or ITA anomalies individually, reports documenting multiple anomalies co-occurring in the same donor remain rare. To our knowledge, there are no published cases that described a retroesophageal ARSA with a NRLN, an absent right ITA, and a muscular branch to the left longus colli muscle. The unique coexistence of these variations underscores the importance of careful preoperative imaging and anatomical awareness for clinicians working in the cervical and superior mediastinal regions.
Methodology
1) Donor preparation
The donor was a 43-year-old female of Thai descent who died of ovarian carcinoma with metastatic spread. No surgical history of previous thoracic and cervical operation was recorded. Soft embalming method was used to preserve the elasticity of vascular structures for high-resolution dissection. The present study was approved by the Human Research Ethics Committee, Faculty of Medicine Ramathibodi Hospital, Mahidol University (certificate of approval number: MURA2025/1031).
2) Vascular injection of the silicone mixture
To improve the visualization of the cervical and thoracic vessels, a vascular injection with a silicone color mixture was performed prior to the dissection. The materials, including silicone rubber (RA-125), silicone oil (RA-50), curing agent (RA-125), and red (P-206) and blue (P-506) silicone pigments, were purchased from RUNGART (Bangkok, Thailand). The silicone mixture was prepared using a standardized ratio of 150 mL silicone rubber, 100 mL silicone oil, 2.5 mL curing agent, and 0.5 mL silicone pigment, which were thoroughly mixed.
To achieve optimal perfusion, the right common carotid artery was cannulated for the arterial system, and the superior vena cava was cannulated for the venous system. Prior to the injection, the distal vessels, including the thoracic aorta, inferior vena cava, left common carotid artery, and pulmonary artery, were clamped to prevent leakage and ensure adequate regional vascular filling. The injection was performed using a 50-mL syringe to draw the silicone mixture and inject it directly into the intravascular catheters. A red silicone mixture was injected into the right common carotid artery for the arterial system, while a blue silicone mixture was injected into the superior vena cava for the venous system. The injection was administered under low and steady manual pressure. Afterward, the donor was stored at room temperature for 2 h to complete the polymerization process prior to the regional dissection.
3) Dissection and morphometric measurements
The cervical and upper thoracic regions were dissected with emphasis on neurovascular pattern and morphometric measurements. We traced the arterial branch of the aorta to investigate further arterial variations. Multiple neurovascular variations were found. To ensure precision and reproducibility, all morphometric measurements of the vascular structures were performed using a digital Vernier caliper (Mitutoyo, Japan). For the evaluation of the ARSA, the following specific parameters were measured: the origin diameter, mid-course diameter, distal end diameter, and total length. Additionally, the dimensions of the various branches arising from the ARSA were also recorded. To minimize measurement error, each parameter was measured at least three times, and the mean values were reported.
Case presentation
The ARSA originated as the fourth branch of the aortic arch, distal to the left subclavian artery, passing posterior to the esophagus without a Kommerell diverticulum. The vertebral, thyrocervical trunk, inferior thyroid, and transverse cervical arteries on the right side emerged in normal patterns (Fig. 1A-F). The ARSA morphometry is listed in Table 1. Moreover, a small muscular branch of the proximal part of the ARSA was observed to be giving rise to the left longus colli muscle. The branch passes posterior to the esophagus and ascends along the prevertebral fascia. The branch measured 1.1 mm in diameter and 35.18 mm in length, supplying the deep prevertebral musculature (Fig. 1D, F).
Fig. 1.
ARSA arising as the fourth branch of the aortic arch after the left subclavian artery, coursing posterior to the esophagus without a Kommerell diverticulum (A). Enlarged anterior view, clearly demonstrating that the ARSA is located laterally to the left subclavian artery (B). Posterior view, demonstrating the ARSA course passing posterior to the esophagus (C). The muscular branch to the left longus colli muscle arises from the proximal part of the ARSA, pointed by the red arrow (D). ARSA becomes clearly visible when the esophagus and trachea are retracted laterally (E). Branch of the ARSA, consisting of the right thyrocervical trunk, right vertebral artery, and a muscular branch to the longus colli muscle, with absence of the right internal thoracic artery (F). Abbreviations: AOA-arch of aorta, ARSA-aberrant right subclavian artery, ASM-anterior scalene muscle, DCA-descending aorta, EG-esophagus, LCCA-left common carotid artery, LSCA-left subclavian artery, RCCA-right common carotid artery, RTCA-right transverse cervical artery, RTCT-right thyrocervical trunk, RVA-right vertebral artery, RVN-right vagus nerve, TC-trachea
Table 1.
Morphometry of the ARSA
| Parameter | Value |
|---|---|
| Origin diameter | 8.07 mm |
| Mid-course diameter | 5.83 mm |
| Distal end diameter | 6.08 mm |
| Total length | 12.16 cm |
| Angle of origin (relative sagittal plane) | 42° |
| Kommerell diverticulum | Absent |
A right-sided NRLN arose directly from the right vagus nerve and coursed parallel with the inferior thyroid artery into the larynx, without looping around the right subclavian artery (Fig. 2A, B). On the left, the left recurrent laryngeal nerve showed a constant recurrent course, looping under the arch of the aorta and rising within the tracheoesophageal groove, situated in close lateral contact to the esophagus following the usual course to the larynx (Fig. 2C).
Fig. 2.
NRLN and left recurrent laryngeal nerves. Right NRLN (black arrows) arising directly from the right vagus nerve, coursing parallel to the inferior thyroid artery into the larynx (A–B). Normal pattern of left recurrent laryngeal nerve, pointed by the red arrow (C). Abbreviations: AOA-arch of aorta, ARSA-aberrant right subclavian artery, EG-esophagus, LCCA-left common carotid artery, LRLN-left recurrent laryngeal nerve, LSCA-left subclavian artery, LVN-left vagus nerve, NRLN-non-recurrent laryngeal nerve, RCCA-right common carotid artery, RITA-right inferior thyroid artery, RVN-right vagus nerve, TC-trachea
` The right ITA was absent from its typical position on the anterior thoracic wall, while the left ITA was found running along the internal aspect of the anterior thoracic wall (diameter 1.59 mm) (Fig. 3A). Instead, a prominent superior intercostal artery (SIA) (diameter 2.60 mm) arose from the first part of the inferior surface of the ARSA and descended posteriorly along the upper thoracic wall. This enlarged SIA provided predominant posterior intercostal branches to the first and second intercostal spaces (Fig. 3B) and exhibited clear anastomoses with the corresponding posterior intercostal arteries from the thoracic aorta (Fig. 3C), systematically taking over the vascular territory typically supplied by the ITA. The overall configuration reflects a rare compensatory arterial remodeling pattern that has not been previously described in association with an ARSA.
Fig. 3.
Variation of the right ITA: absent right ITA in the posterior view of the anterior thoracic wall where LITA is intact (A). A prominent SIA arose from the ARSA and descended posteriorly along the upper thoracic wall, indicated by the red arrow (B). Anastomosis between the SIA and the posterior intercostal artery is indicated by the blue arrow (C). Abbreviations: ARSA-aberrant right subclavian artery, ASM-anterior scalene muscle, LITA-left internal thoracic artery, PIA-posterior intercostal artery, SIA-superior intercostal artery
Discussion
ARSA is the most frequent congenital anomaly of the aortic arch, with a prevalence of approximately 0.5–2.0% [2, 3]. Although the present case shows the most common morphological type of ARSA [17], which includes a retroesophageal course of the ARSA, a right NRLN, the absence of Kommerell’s diverticulum; it also presents with the complete absence of the right ITA, and a muscular branch arising from the proximal part of the ARSA, supplying the left longus colli muscle (Fig. 4). These additional findings have not been previously characterized with the most common type of ARSA.
Fig. 4.
Diagram of the four anatomical variation combinations: a retroesophageal ARSA, a right-sided NRLN, SIA replacing the absent right ITA, and a muscular branch to the left longus colli muscle from the ARSA
Although the association between an ARSA and a right NRLN is well established, with ARSA present in approximately 86.7% of right NRLN cases [18], the concurrent occurrence of multiple major neurovascular anomalies remains exceptionally rare. One such example is the previously reported triad comprising ARSA, NRLN, and an anomalous right thoracic duct [19]. Moreover, the congenital absence or hypoplasia of the ITA is an extremely uncommon anatomical variation [20]. By comparing our findings with previously documented variations, we highlight the distinct clinical novelty of this case. Specifically, the unique tetrad observed in our donor, comprising a retroesophageal ARSA, a right NRLN, an absent right ITA replaced by a compensatory SIA, and a variant muscular branch to the left longus colli muscle, has not been reported in the existing literature.
Embryological basis of related anomalies
The concurrence of ARSA with NRLN is developmentally inextricably linked. In normal embryogenesis, the proximal part of the right subclavian artery (at the origin point of ITA) derives from the right 4th aortic arch and the most caudal portion of the dorsal aorta, while the distal part derives from the right 7th intersegmental artery [21, 22]. The right recurrent laryngeal nerve typically loops around the right 4th aortic arch (Fig. 5A, B). In the case of an ARSA, the right 4th aortic arch and the proximal right dorsal aorta are involuted abnormally. Consequently, the right subclavian artery forms by the persistence of the 7th intersegmental artery attached to the distal dorsal aorta. This regression of the 4th arch eliminates the anatomical fulcrum that pulls typically the right recurrent laryngeal nerve caudally during the descent of the heart; as a result, the nerve migrates directly from the right vagus nerve to the larynx, becoming an NRLN [6] (Fig. 5C). This mechanism explains the strong predictive value of ARSA for NRLN, with studies indicating that nearly all right-sided NRLNs are associated with an ARSA.
Fig. 5.
Diagram of embryological development of the aortic arch system and the origin of ARSA associated with NRLN. (A) Schematic illustration of the early embryonic aortic arch system. (B) Normal development pattern of the aortic arch derivative. The right 4th aortic arch contributes to the proximal segment of the right subclavian artery, allowing the right recurrent laryngeal nerve to loop beneath the subclavian artery before ascending into the larynx. (C) Aberrant development with regression of the right 4th aortic arch and persistence of the right dorsal aorta connected with the 7th intersegmental artery, resulting in an ARSA with NRLN
A novel finding in this case is the absence of the right ITA and its replacement by a prominent SIA. Embryologically, the vertical part of the ITA is developed from the ventral longitudinal anastomosis that links the thoracic intersegmental arteries along the anterior chest wall, and the origin of the right ITA connects proximally to the right 7th intersegmental artery [16, 20]. Previous research found that the right fourth dorsal segmental artery forms the proximal part of the right subclavian artery [23]. In normal anatomy, these ventral channels connect proximally to the segment of the subclavian artery derived from the fourth aortic arch [22]. As previously mentioned, when the right 4th aortic arch and the proximal right dorsal aorta are involuted abnormally, it will effectively eliminate the “normal” proximal landing zone for these ventral longitudinal channels. The failure of the ventral somatic anastomosis to establish a connection with the aberrant vessel likely led to the aplasia of the right ITA. In addition, due to the caudal shifting of the aorta, the proximal parts of the ARSA are exposed to longitudinal tension and bending, with resulting ischemia [24]. This may result in abnormal connections between the longitudinal anastomotic channels, such as the ITA.
Consequently, the hemodynamic demand of the anterior chest wall likely triggered a compensatory hypertrophy of the dorsal longitudinal connections, represented by the SIA. The typical pattern of SIA is the artery that branches from the costocervical trunk of the second part of the subclavian artery and runs postero-inferior to supply the first and second intercostal spaces. In this record, the origin of this prominent SIA from the inferior surface of ARSA and medial to the anterior scalene muscle of ARSA supports the angiographic evidence that the SIA retains embryonic connections to the last cervical and first thoracic intersegmental arteries [25]. This illustrates a mechanism of vascular plasticity where the dorsal somatic territory systematically re-vascularizes the territory typically supplied by the ventral somatic system. The variation of the ARSA branch was also found in the previous report. Ostrowski et al. reported that the ARSA has a costocervical trunk running posteriorly to supply the first intercostal space [26]. In contrast, this case report demonstrated that the SIA arising from the ARSA did not appear as a trunk, but rather emerged as a single dominant SIA descending directly along the posterior thoracic wall.
In the present case, there is also a muscular branch of ARSA that runs superiorly to supply the left longus colli muscle. This could be facilitated by vascular endothelial growth factor (VEGF)-induced angiogenesis. When the cervical somites that will become the cervical axial muscle differentiate into myoblasts [27]. They secreted VEGF to stimulate sprout angiogenesis from nearby retroesophageal ARSA [28], supplying the left longus colli muscle in addition to the typical muscular branch from the vertebral arteries, ascending cervical arteries, and ascending pharyngeal arteries [29].
Clinical and surgical implications
The presence of these variations poses distinct risks across multiple surgical domains. First, although often asymptomatic, the retroesophageal course of the ARSA can cause dysphagia lusoria through mechanical compression of the esophagus [2, 5, 30]. Although the retroesophageal course of the ARSA being in close proximity to the esophagus highlight a potential anatomical basis for compression, these findings alone cannot confirm the presence of clinical symptoms. Because a detailed clinical history specifically regarding compressive symptoms was unavailable for this donor, the clinical implications of this anatomical proximity should be interpreted with caution.
Second, The NRLN represents a major hazard during thyroidectomy and anterior cervical spine surgery. In this report, the NRLN observed in this case is classified as type 2a, as the nerve runs transversely superior to the trunk of the inferior thyroid artery [31, 32]. The nerve arises from the right vagus nerve at the level of the inferior border of the thyroid gland. It ascends superior to the inferior thyroid artery into the larynx, a pattern previously reported [8, 33]. Because the NRLN does not lie along the tracheoesophageal groove, standard surgical approaches that rely on this landmark for nerve identification may lead to iatrogenic injury, vocal cord paralysis, and dysphonia [6, 34, 35]. The identification of an ARSA on preoperative CT or MRI should automatically trigger a high index of suspicion for a NRLN.
Third, the lack of the appropriate ITA significantly affects cardiac surgery outcomes. The ITA is used as the most common conduit for coronary artery bypass grafting, attributed to its resistance to atherosclerosis and enhanced long-term patency rates [36, 37]. The unexpected absence of the right ITA, or its replacement by a vessel with different histological properties (such as the SIA), complicates the harvesting of the graft [38]. The compensatory SIA resulting from a retroesophageal ARSA presents technical challenges for harvesting and is likely inappropriate for grafting. This highlights the importance of preoperative angiography to confirm conduit availability in patients with aortic arch anomalies.
Finally, the presence of a muscular branch artery arising directly from the proximal ARSA that ascends to supply the left longus colli muscle and prevertebral area is a critical finding for spine surgeons. The longus colli muscle is a key landmark in anterior cervical discectomy and fusion. An unexpected arterial branch in the prevertebral fascia creates a risk of obscure bleeding that can retract into the retroesophageal space, complicating hemostasis and obscuring the surgical field [39].
Limitations of the study
Despite the novel anatomical insights provided by this report, several limitations must be acknowledged. First, as this study is based on a single donor case, it inherently lacks the statistical power to determine the population prevalence of this specific tetrad of neurovascular anomalies. Second, although the retroesophageal course of the ARSA and the presence of the right NRLN suggest a potential anatomical basis for surgical complications or compressive symptoms, the donor’s specific clinical history regarding dysphagia, dyspnea, or voice alterations prior to her death from metastatic ovarian carcinoma was unavailable. Consequently, a definitive clinical-anatomical correlation cannot be established. Finally, the nature of a donor dissection precludes any functional or in vivo hemodynamic assessments, such as evaluating the physiological impact of the absent right ITA on the chest wall perfusion or determining the exact hemodynamic compensatory mechanism of the enlarged SIA.
Conclusion
This report provides morphometric evidence of a complex four-point anomaly. The developmental regression of the 4th aortic arch caused a retroesophageal ARSA with NRLN, and it also interrupted the normal development of the circulation in the anterior portion of the chest wall, leading to the replacement of the right ITA by a compensatory SIA and a distinct muscular branch supplying the left longus colli muscle. These findings highlight the importance of comprehensive preoperative imaging to ensure patient safety during neck, thoracic, and coronary revascularization procedures.
Acknowledgements
The authors sincerely thank those who donated their bodies to the field of anatomical research. The results of such research can potentially increase humankind’s overall knowledge and improve patient care. Therefore, the donors and their families deserve our highest gratitude.
Abbreviations
- AOA
Arch of aorta
- ARSA
Aberrant right subclavian artery
- ASM
Anterior scalene muscle
- EG
Esophagus
- ITA
Internal thoracic artery
- LCCA
Left common carotid artery
- LITA
Left internal thoracic artery
- LRLN
Left recurrent laryngeal nerve
- LSCA
Left subclavian artery
- LVN
Left vagus nerve
- NRLN
Non-recurrent laryngeal nerve
- PIA
Posterior intercostal artery
- RCCA
Right common carotid artery
- RITA
Right inferior thyroid artery
- RTCA
Right transverse cervical artery
- RTCT
Right thyrocervical trunk
- RVA
Right vertebral artery
- RVN
Right vagus nerve
- SIA
Superior intercostal artery
- TC
Trachea
- VEGF
Vascular endothelial growth factor
Author contributions
A.P. conceptualized the study. A. P., N. S., N. M., P. T., and P. B. performed the methodology and investigation. A. P., N. S. and N. M. conducted the formal analysis. A. P., R. M. and D. J. prepared the original draft and visualizations. A. P. and R. M. critically revised the manuscript. All authors reviewed and approved the final manuscript before submission.
Funding
This study did not receive any funding support.
Data availability
No datasets were generated or analysed during the current study.
Declarations
Ethics approval and consent to participate
This study was approved by the Human Research Ethics Committee, Faculty of Medicine Ramathibodi Hospital, Mahidol University (certificate of approval number: MURA2025/1031).
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
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Data Availability Statement
No datasets were generated or analysed during the current study.