CASE 18270 Published on 21.08.2023

Spaghetti junctions in the body: Rare cause of venous thrombosis

Section

Head & neck imaging

Case Type

Clinical Cases

Authors

Ibrahim Al-Mousllie¹, Clarissa Gevargez Lyngby², Rebeca Mirón Mombiela²

1. Hannover Medical School, Hanover, Germany

2. Herlev og Gentofte Hospital, Denmark

Patient

71 years, female

Categories

Area of Interest Anatomy, Head and neck, Oncology, Vascular, Veins / Vena cava ; Imaging Technique CT

Procedure Catheters ; Special Focus Aneurysms, Cancer, Drugs / Reactions, Embolism / Thrombosis ;

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Clinical History

A 71-year-old woman with a history of mamma cancer treated with mastectomy comes to the emergency department because of pain and swelling in the right neck. She received chemotherapy for the last 15 months through a Port-a-cath, which was removed 5 days ago from the right subclavian vein (Fig. 1).

Imaging Findings

The contrast-enhanced CT of neck and thorax in the venous phase revealed an expanded right internal jugular vein (IJV) with a contrast defect, and to a lesser extent right external jugular vein (EJV), compatible with two thrombi (Fig. 2).

Incidentally, four venous malformations were found. The first is the bilateral termination of the facial vein (FV) into the respective EJV (Fig. 3-4). The second is the duplication of the left EJV originating from the confluence of the left FV and EJV and terminating into the left brachiocephalic vein (BV) (Fig. 4). The third is the missing left anterior jugular vein (AJV) (Fig. 4). The fourth is the bilateral fenestration of the EJV, though the left EJV is double fenestrated (Fig. 3-4). The jugular venous arch is formed by branches of the right AJV and the duplication (Fig. 5).

Discussion

Background

Venous malformations are congenital malformations of the venous system [1] with an estimated prevalence of 1% [2]. They are classified as extratruncular and truncular based on the developmental stage [3]. The first describes disorganised changes of multiple vessels. The latter is more organised and affects fewer vessels, as it occurs in more advanced developmental stages. Miscommunication of endothelial and soft muscle cells or different signalling cascades [4-5] during vasculogenesis or angiogenesis [6] are assumed to cause the malformation.

To our knowledge, this is the first reported case of a bilateral fenestrated EJV, since only unilateral fenestrated EJVs are described in the literature. Fourteen cases of unilateral fenestrated EJVs [7-20] and 12 cases of duplicated EJVs were reported [21-29]. The fenestration of a vessel is defined as the separation into two branches that rejoin [30-31], while a duplication does not rejoin [30]. Fenestrations and duplications are truncular malformations.

Another variation, prevalent in 5% of the population, is the connection of the FV to the EJV. It is an embryological remnant as the connection usually retrogresses [32].

Although thrombosis is a common complication of a Port-a-cath (PAC) [33-34], it can be hypothesised that the increased turbulence in and decreased velocity of the bloodstream caused by the fenestration and the duplication increase the risk of thrombosis. Dinh et al. (2021) published a case report of a deep venous thrombosis (DVT) in a fenestrated common iliac vein (CIV) that was previously accessed for a supraventricular tachycardia ablation. They also suspect a connection between the fenestration of the CIV and the DVT [35]. The venous malformation combined with irritation of the intima for example by a central venous catheter (CVC) is expected to play a role in thrombosis.

Clinical Perspective

The detection and radiological evaluation of venous malformation such of the EJV requires special attention due to its clinical importance in planning different procedures (measurement of the central venous pressure [36-37], cannulation in diagnostics [39], CVCs [39-41]) and surgeries (reconstructive surgery, free flap surgeries [42]), and avoiding complications.

Outcome

In our case, if chemotherapy is contemplated in the future, an alternative access point for cannulation should be considered instead of the SV or a jugular vein. Two possible alternatives are the PICC-line using peripheral veins, such as the basilic or cephalic vein [43-44], and a CVC through the femoral vein. However, the latter is associated with a higher rate of infection and thrombosis [45].

Written informed patient consent for publication has been obtained.

Differential Diagnosis List

Bilateral fenestration with left-sided duplication of the EJV

Phlebectasia

Venous aneurysm

Final Diagnosis

Bilateral fenestration with left-sided duplication of the EJV

References

[1] [1] Colletti G, Ierardi AM. (2017) Understanding venous malformations of the head and neck: a comprehensive insight. Med Oncol. 34(3):42 (PMID: 28181207)

[2] [2] Legiehn GM, Heran MKS. (2008) Venous malformations: classification, development, diagnosis, and interventional radiologic manage- ment. Radiol Clin N Am. 46(3):545–97 (PMID: 18707962)

[3] [3] Belov S. (1989) Classification, terminology and nosology of congenital vascular defects. Reinbek: Einhorn-Presse:25–8

[4] [4] Gallione CJ, Pasyk KA, Boon LM, Lennon F, Johnson DW, Helmbold EA, Markel DS, Vikkula M, Mulliken JB, Warman ML, et al. (1995) A gene for familial venous malformations maps to chromosome 9p in a second large kindred. J Med Genet. 32(3):197-9 (PMID: 7783168)

[5] [5] Boon LM, Mulliken JB, Enjolras O, Vikkula M. (2004) Glomuvenous malformation (glomangioma) and venous malformation: distinct clinicopathologic and genetic entities. Arch Dermatol. 140(8):971-6 (PMID: 15313813)

[6] [6] Cohen MM Jr. (2006) Vascular update: morphogenesis, tumors, malformations, and molecular dimensions. Am J Med Genet A. 140(19):2013-38 (PMID: 16958055)

[7] [7] Comert E, Comert A. (2009) External jugular vein duplication. J Craniofac Surg. 20(6):2173-4 (PMID: 19884835)

[8] [8] Pillay P., Ishwarkumar S., Satyapal K. (2018) Variations of the external and internal jugular veins: A fetal study. Int. J. Morphol. 36(1):145–148

[9] [9] Gerard Iii NO, Tubbs RS, Iwanaga J. (2021) Duplicated transverse cervical nerve and external jugular vein. Anat Cell Biol. 54(3):404-406 (PMID: 34031273)

[10] [10] Cvetko E. (2015) A case of left-sided absence and right-sided fenestration of the external jugular vein and a review of the literature. Surg Radiol Anat. 37(7):883-6 (PMID: 25432662)

[11] [11] Cvetko E. (2013) A case of unilateral fenestration of the external jugular vein, through which the cervical branch of the facial nerve passes. Anat Sci Int. 88(3):151-2 (PMID: 23354878)

[12] [12] Ponnambalam SB, Karuppiah DS. (2020) Unilateral external jugular vein fenestration with variant anatomy of the retromandibular and facial vein. Anat Cell Biol. 53(1):117-120 (PMID: 32274258)

[13] [13] Sugiyama S., Iwai T., Tohnai I. (2017) Empty fenestration of the external jugular vein: A rare variant. J. Anat. Soc. India. 66(2):S1–S3

[14] [14] Eskell M.J., Bickerton R.D., Guy I.A., Jefferies B.J. (2019) A case of unilateral fenestration of the external jugular vein in a male cadaver with a review of existing literature. Int. J. Case Rep. Images. 2019;10:101048Z101001ME102019

[15] [15] Vani PC, Rajasekhar SSSN, Gladwin V. (2019) Unusual and multiple variations of head and neck veins: a case report. Surg Radiol Anat. 41(5):535-538 (PMID: 30778672)

[16] [16] Téllez-Hernández L.V., Tibaduiza-Rodriguez I.A., Ferreira-Arquez H. (2019) Unilateral anatomical variation in the venous drainage of face and neck. Int. J. Pharm. Res. 11:1152–1155

[17] [17] Rao S, Pandey S, Kumar Y, Rao S. (2018) Bifurcation of external jugular vein: an anatomical variation during neck dissection. Oral Maxillofac Surg. 22(4):475-476 (PMID: 30167901)

[18] [18] Snoj, Ziga, & Cvetko, Erika. (2013). A Case of Unilateral Fenestration and Duplication of the External Jugular Vein. International Journal of Morphology 31(1):107-109

[19] [19] Paraskevas, G.K., Asouhidou, I., Anastasopoulos, N. et al. (2023) External Jugular Vein Perforation by the Transverse Cutaneous Nerve of The Neck: A Case Report and Review of The Literature. Indian J Otolaryngol Head Neck Surg

[20] [20] Shenoy V, Saraswathi P, Raghunath G, Karthik JS. (2012) Double external jugular vein and other rare venous variations of the head and neck. Singapore Med J. 53(12):e251-3 (PMID: 23268166)

[21] [21] Pillay P., Ishwarkumar S., Satyapal K. (2018) Variations of the external and internal jugular veins: A fetal study. International Journal of Morphology 36:145–148

[22] [22] Suresh RR and T Ramesh R. (2020). A study of anatomical variation of external jugular vein. World Journal of Advanced Research and Reviews, 7(1):197-201

[23] [23] Snoj, Ziga, & Cvetko, Erika. (2013). A Case of Unilateral Fenestration and Duplication of the External Jugular Vein. International Journal of Morphology 31(1):107-109

[24] [24] Nayak SB, Vasudeva SK. (2022) Terminal bifurcation of the external jugular vein: a rare variation. Anat Cell Biol. 55(4):501-503 (PMID: 35989354)

[25] [25] Gerard Iii NO, Tubbs RS, Iwanaga J. (2021) Duplicated transverse cervical nerve and external jugular vein. Anat Cell Biol. 54(3):404-406 (PMID: 34031273)

[26] [26] Shenoy V, Saraswathi P, Raghunath G, Karthik JS. (2012) Double external jugular vein and other rare venous variations of the head and neck. Singapore Med J. 53(12):e251-3 (PMID: 23268166)

[27] [27] Olabu B.O., Loyal P.K., Matiko B.W., Nderitu J.M., Misiani M.K., Ogeng’o J.A. (2015) Variant anatomy of the external jugular vein. Anat. J. Afr. 2015;4:518–527

[28] [28] Singh R. (2020) Abnormal Formation of External Jugular Vein and its Repercussions. J Craniofac Surg. 31(4):e354-e355 (PMID: 32149966)

[29] [29] Singla R.K., Singla S., Sachdeva K. (2011) Partial duplication of external jugular vein forming a venous ring around supraclavicular nerve: A case report. J. Clin. Diagn. Res. JCDR 5:859–861

[30] [30] Rusu, M.C.; Tudose, R.C.; Vrapciu, A.D.; Toader, C.; Popescu, S.A. (2023) Anatomical Variations of the External Jugular Vein: A Pictorial and Critical Review. Medicina 59:622 (PMID: 36984623)

[31] [31] Ponnambalam, S. B., & Karuppiah, D. S. (2020). Unilateral external jugular vein fenestration with variant anatomy of the retromandibular and facial vein. Anatomy & cell biology, 53(1):117–120 (PMID: 32274258)

[32] [32] Choudhry, R.; Tuli, A.; Choudhry, S. (1997) Facial vein terminating in the external jugular vein. An embryologic interpretation. Surg. Radiol. Anat. 19:73–77 (PMID: 9210239)

[33] [33] Li, Y., Guo, J., Zhang, Y., & Kong, J. (2021). Complications from port-a-cath system implantation in adults with malignant tumors: A 10-year single-center retrospective study. Journal of interventional medicine, 5(1):15–22. (PMID: 35586285)

[34] [34] Machat, S., Eisenhuber, E., Pfarl, G., Stübler, J., Koelblinger, C., Zacherl, J., & Schima, W. (2019). Complications of central venous port systems: a pictorial review. Insights into imaging, 10(1):86 (PMID: 31463643)

[35] [35] Dinh, K., Manuel, L., McGill, A., & Daly, T. (2021). Fenestration of the common iliac vein: an unusual cause of deep venous thrombus. Journal of surgical case reports, 2021(4), rjab045. (PMID: 33859812)

[36] [36] Vinayak AG, Levitt J, Gehlbach B, Pohlman AS, Hall JB, Kress JP. (2006) Usefulness of the external jugular vein examination in detecting abnormal central venous pressure in critically ill patients. Arch Intern Med. 166(19):2132-7 (PMID: 17060544)

[37] [37] Nagata T, Masumoto K, Watanabe Y, Katou F. (2012) End-to-side anastomosis to the external jugular vein: preservation of external jugular vein blood flow. Br J Oral Maxillofac Surg 50:e31-2 (PMID: 21871698)

[38] [38] Peterson KA, Burnette CD, Fargen KM, Brown PA, West JL, Tatter SB, Wolfe SQ. (2020) External jugular venous sampling for Cushing's disease in a patient with hypoplastic inferior petrosal sinuses. J Neurosurg. 2020 Jan 17:1-4 (PMID: 31952032)

[39] [39] Ishizuka M, Nagata H, Takagi K, Kubota K. (2008) External jugular venous catheterization with a Groshong catheter for central venous access. J Surg Oncol. 98(1):67-9 (PMID: 18452215)

[40] [40] Cho SK, Shin SW, Do YS, Park KB, Choo SW, Choo IW. (2006) Use of the right external jugular vein as the preferred access site when the right internal jugular vein is not usable. J Vasc Interv Radiol. 17(5):823-9 (PMID: 16687748)

[41] [41] Teichgräber UK, Pfitzmann R, Hofmann HA. (2011) Central venous port systems as an integral part of chemotherapy. Dtsch Arztebl Int. 108(9):147-53 (PMID: 21442071)

[42] [42] Ibrahim AE, Adelman DM, Parham C, Hong Z, Villa M, Chahine FM, Ghieh FM. (2019) The external jugular vein used as recipient vessel in head and neck free flap reconstruction: out- comes compared to the internal jugular vein. J Craniofac Surg 30:178-83 (PMID: 30394970)

[43] [43] Li, G., Zhang, Y., Ma, H., & Zheng, J. (2019). Arm port vs chest port: a systematic review and meta-analysis. Cancer management and research, 11:6099–6112 (PMID: 31308748)

[44] [44] D'Angelo FA, Ramacciato G, Aurello P, Lauro S, Caramitti A, Lalle M, Magri M. (1997) Alternative insertion sites for permanent central venous access devices. Eur J Surg Oncol. 23(6):547-9 (PMID: 9484928)

[45] [45] Timsit JF. (2003) What is the best site for central venous catheter insertion in critically ill patients? Crit Care. 2003 7(6):397-9 (PMID: 14624670)

Case information

URL:	https://www.eurorad.org/case/18270
DOI:	10.35100/eurorad/case.18270
ISSN:	1563-4086

License

This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

Figure 1

Photograph of Port-a-caths previous insertion site with visible superficial veins in the neck area

Figure 2

Coronal contrast-enhanced CT image of the thorax and neck. It shows the two thrombi. The first thrombus (a) extends approximately 8 cm from the end of the right BV up to the middle of the IJV and proximal part of the right SV. The second thrombus (b) is located at the confluence of the fenestration of the right EJV. BV = brachiocephalic vein; IJV = internal jugular vein; SV = subclavian vein; EJV = external jugular vein

Figure 3

Image of volume rendered 3D post-processing reconstruction of the neck. The right lateral view shows the fenestration (b) of the right EJV (a), as well as the drainage of the FV (c) into the EJV (a). The AJV (d) drains as usual into the EJV (a). EJV = external jugular vein; FV = facial vein; AJV = anterior jugular vein

Figure 4

Image of volume rendered 3D post-processing reconstruction of the neck. The left lateral view shows the first (b) and second (c) fenestration of the left EJV (a), as well as the drainage of the FV (d) into the EJV (a). It also shows the duplication (e) of the EJV (a) draining into the BV (f). The left AJV is missing. EJV = external jugular vein; FV = facial vein; AJV = anterior jugular vein; BV = brachiocephalic vein

Figure 5

Play Video

Video of a volume rendered 3D reconstruction of the neck. It shows the bilateral fenestration of the EJV and the drainage of the FV into the respective EJV. It also shows the unilateral duplication of left EJV and the jugular venous arch that forms between the duplication and the right AJV. EJV = external jugular vein; FV = facial vein; AJV = anterior jugular vein

Figure 6

Play Video

Coronal (a) and sagittal (b) contrast-enhanced CT series of the thorax and neck. They show the bilateral fenestration and unilateral duplication of the EJV, as well as the two thrombi, in the IJV and EJV, during the venous phase.