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\begin{document}

\title{Persistent headaches sometimes concern incidental findings of rare
internal jugular vein agenesis rare case in Bangladesh: A case report}



\author[1]{Mohammad Ashraful Amin}%
\author[2]{Sabrina Nahin}%
\author[1]{Mohammad Delwer Hossain Hawlader}%
\affil[1]{North South University}%
\affil[2]{Green Life Medical College and Hospital}%


\vspace{-1em}



  
  \date{May 24, 2024}


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\selectlanguage{english}
\begin{abstract}
Absence of an internal jugular vein at birth is infrequent.A dull
headache troubled a 32-year-old man for more than 20 years, which
persisted, a diagnosis of left internal jugular vein agenesis was made,
which was most likely the cause of the headache.%
\end{abstract}%



\sloppy


\textbf{Introduction:}

The internal jugular vein (IJV) is a significant factor leading to
venous drainage from intracranial components, draining blood from the
head and neck region. The absence of an internal jugular vein (IJV) is
an asymptomatic, relatively unusual vascular abnormality (1). In the
general community, 0.05\%-0.25\% of people have developmental venous
abnormalities (2).

Vascular tumors (hemangioma, hemangioendotelioma, and angiosarcoma) and
vascular malformations (hemangioma, hemangioendotelioma, and
angiosarcoma) are two types of vascular malformations (3). Even though
developmental venous anomalies (DVAs) are generally thought to be
innocuous vascular malformations (4), We have met patients with symptoms
of headache as DVAs features.

Internal jugular vein absence is a unique congenital condition. Only a
few cases (in the low double digits) of a missing internal jugular vein
have been described in English literature to our awareness. Most cases
were discovered by chance during a routine check before central venous
cannulation in patients who received major surgery. We report a
32-year-old male with left internal jugular vein agenesis who has
experienced a dull headache for more than 20 years and who has no family
history of the condition. There was not yet a remarkable intervention
for this situation.

\textbf{Case report}

On November 25th, 2021, a 32-year-old man with a 20-year history of
chronic headaches was referred to the National Institute of
Neurosciences \& Hospital. Apart from that, the physical checkup was
normal. Since 2002, he has had constant and recurring headaches,
occurring 2-3 times each week. The headaches were reported as forehead
pain that did not extend to his eye or any other body part. The attacks
lasted 30 to 45 minutes on average. These headaches frequently occurred
in the afternoon and were not characterized by eye redness or weeping.
The headaches were so bad that he could not stand it any longer. Except
for NSAIDs, which only eased the pain to a minor degree, no therapies
were helpful before consulting with a physician. He was previously
diagnosed with tension headaches and also treated as migraine for dull
on-off headaches. He had previously been prescribed paracetamol, NSAIDs,
Propranolol, methysergide, antihistamines anti-anxiety and sedative
medication and naproxen sodium were among the earlier ineffectual
treatments. A clinical interview, the Minnesota Multiphasic Personality
Inventory-2 (MMPI-2), the State-Trait Anxiety Inventory (STAI), and the
Beck Depression Inventory (BDI) were all used in a comprehensive
psychiatric assessment. The psychological perception was the situational
anxiety resulting from her disease, with no underlying psycho-emotional
dysfunction. On numerous occasions, physicians have recommended that he
undergo contrast-enhanced computed tomography (CT scan) and magnetic
resonance imaging (MRI), but he has always planned to do it later. He
experienced acute watery diarrhoea recently on 10 November 2021, and lab
tests revealed Hb-15.90 g/dL, WBC-28.93 x 109/L, Platelets-342 x 109/L,
ESR-16 mm in the first hour. Sodium 133 mmol/L, potassium 5.4 mmol/L,
creatinine 2.3 mg/dl. The electrocardiogram, liver/renal function,
clotting profile, chest X-ray, and liver/renal function were normal. But
this acute diarrhea period he had associated severe headache so he
underwent contrast-enhanced computed tomography (CT scan) (Figure:01)
and Magnetic Resonance Venography (MRV) (Figure:02); which revealed the
jugular venous system of the left side is not visualized and the right
side jugular venous system is prominent and deep veins and dural venous
sinuses appear normal which suggestive of congenital agenesis of jugular
venous system of left side. Due to financial difficulties, he has not
yet undergone an intervention or any other procedure. He still had a
persistent headache, but the patient remained constant during the
follow-up visit the following week.

\textbf{Discussion:}

Embryological developmental abnormalities cause vascular malformations.
Hemangiomas fade away over time. However, vascular abnormalities can
grow and last a lifetime. Even though vascular abnormalities are present
at birth, they might not have been discovered until adolescence or the
elderly (5). Venous abnormalities are generally asymptomatic and
typically occur in the head and neck region (6).

Congenital agenesis of the IJV is a very unusual occurrence (3). The CT
imaging of an instance of IJV agenesis is described in this article.
Early detection can help limit damages during medical therapy of
neurological conditions, including headaches, and avoid misdiagnosis and
other prevalent diagnoses. The IJVs are the prominent venous outflow
veins in the brain. In a patient with congenital agenesis of the IJV,
impairment of the alternate pathways of vascular supply from the cranial
cavity can have catastrophic repercussions. Embryological developmental
abnormalities cause vascular anomalies in the head and neck region,
which can persist asymptomatically (7).

IJV passes through the neck's carotid sheath and behind the clavicle's
sternal end. It then joins the subclavian vein to form the
brachiocephalic vein, which travels through the thorax to reach the
superior vena cava. Clinically, knowledge of IJV anatomical differences
is critical for venous applications. Central catheters are often used in
intensive care units. IJV anomalies may prevent the practitioner from
performing the procedure, causing significant consequences. To avoid
difficulties, the surgeon should conversate with the anatomy and
variations of IJV (3).

Different IJV anomalies have been revealed in the scientific literature:
partial or total duplication (8, 9), stenosis, complete occlusion,
distortions, and intraluminal structures, such as membranes, webs, and
inverted valves (10). These are frequently discovered by chance or
during neck diagnostic techniques due to a suspicious tumour and are
sometimes the consequence of compensatory contralateral IJV enlargement
(3). Our case was slightly different because he identified IJV agenesis
based on the headache evaluation. Our patient had no prior history of
any other illnesses or thrombosis history. There was no thrombosis on
ultrasonography, CT, or neuroimaging, and the IJV was congenitally
agenesis.

Patients who report to the emergency department frequently complain of
headaches. Most of these headaches are harmless, but others have a more
organic severe cause. Patients may present with a chronic headache issue
that they cannot manage. The history of headache diagnosis is thoroughly
addressed, followed by a discussion of headache patients' emergency
presentations. Subarachnoid haemorrhage, meningitis, sinusitis,
glaucoma, internal carotid artery dissection, and cerebrovascular
illness are the causes for worry and a full explanation of the
differential diagnosis. Analgesics, NSAIDs, opioids, and end ergotamine
formulations are among the drugs discussed for symptomatic headache
treatment. In the emergency service, strategies for treating frequent,
chronic headaches keep in mind that agenesis of the jugular venous
system can play a role---one of the uncommon causes of headaches that we
have seen in our instances. Limitations, more advanced intervention can
be used to rule out any linkage between internal jugular vein agenesis
and headaches, as well as surgical treatment for this result.

\textbf{Conclusion:}

The internal jugular vein is crucial in intensive care units and head
and neck surgeries. Handling the internal jugular vein without
considering anatomical variances is fatal. Early detection of these
anomalies allows for necessary measures to avoid patient injury (e.g.,
discussions with the patient and surgeons about ideal solution
anesthetics plans, cannulation sites, monitoring tactics, and thus
further inquiry prior to operation). The most common headache symptom is
usually caused by that benign, and it can generally be detected after a
comprehensive history and targeted neurologic and overall physical
tests. Headache caused by cerebral venous abnormalities is a new issue
that all health care workers should be aware of this issue.

\textbf{Funding}

This research did not receive any specific grant from funding agencies
in the public, commercial, or not-for-profit sectors.

\textbf{Declaration of competing interest}

The authors declare that they have no competing financial interests or
personal relationships that could have appeared to influence the work
reported in this paper.

\textbf{Consent~}

The patient's written informed consent for publishing of this case
report, as well as images, was acquired.

\textbf{Ethical approval}

The article is about a case study. As a result, our Ethics Committee's
consent was not required.

\textbf{Author contributions}

Conceptualization: Mohammad Ashraful Amin.

Supervision: Mohammad Delwer Hossain Hawlader.

Writing -- original draft: Mohammad Ashraful Amin, Sabrina Nahin

Writing -- review \& editing: Mohammad Ashraful Amin, Sabrina Nahin,
Mohammad Delwer Hossain Hawlader.

\textbf{Data availability statement}

Data can be shared based on the reader's reasonable re-quest and
priority base and some restrictions will apply.

\textbf{Reference:}

1. Filograna L, Calcagni A, Rossi G, Di Stefano C, Beninati E, Collura
A, et al. Internal jugular vein agenesis: a rare vascular abnormality
and incidental finding. A case of internal jugular vein agenesis in a
52-years old male. Radiology case reports. 2019;14(4):452-5.

2. Essafti M, Belhadj A, Qamouss Y, Rokhsi R, Aissaoui Y. Agenesis of
the left internal jugular vein: An unusual finding during an ultra-sound
guided central venous catheterization. Journal of clinical anesthesia.
2018;44:87-8.

3. Kayiran O, Calli C, Emre A, Soy FK. Congenital agenesis of the
internal jugular vein: an extremely rare anomaly. Case Reports in
Surgery. 2015;2015.

4. Pereira VM, Geibprasert S, Krings T, Aurboonyawat T, Ozanne A,
Toulgoat F, et al. Pathomechanisms of symptomatic developmental venous
anomalies. Stroke. 2008;39(12):3201-15.

5. Paltiel HJ, Burrows PE, Kozakewich HP, Zurakowski D, Mulliken JB.
Soft-tissue vascular anomalies: utility of US for diagnosis. Radiology.
2000;214(3):747-54.

6. Loose DA. Combined Treatment of Vascular Malformations: Indications,
Methods, and Techniques. Textbook of angiology: Springer; 2000. p.
1278-83.

7. Kong K, Jian C, Ji R, Irwin MG. Management of an incidental finding
of right internal jugular vein agenesis. Hong Kong Medical Journal.
2017.

8. Nayak SP, Ashraf M, Dam A, Biswas J. Internal jugular vein
duplication: review and classification. Indian Journal of Surgical
Oncology. 2017;8(2):222-6.

9. Prades J, Timoshenko A, Dumollard J, Durand M, Merzougui N, Martin C.
High duplication of the internal jugular vein: clinical incidence in the
adult and surgical consequences, a report of three clinical cases.
Surgical and radiologic anatomy. 2002;24(2):129-32.

10. Zamboni P, Consorti G, Galeotti R, Gianesini S, Menegatti E, Tacconi
G, et al. Venous collateral circulation of the extracranial
cerebrospinal outflow routes. Current neurovascular research.
2009;6(3):204-12.

Figures:\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image1/image1}

\end{center}
\end{figure}

\textbf{Figure 01:} Computed Tomography (CT) scans of the case: CT
demonstrated absence of Left IJV.\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image2/image2}

\end{center}
\end{figure}

\textbf{Figure 02:} Magnetic Resonance Venography (MRV): Sagittal view
of collateral system (dynamic magnetic resonance image) of the case:
Revealed the jugular venous system of the left side is not visualized
and the right side jugular venous system is prominent and deep veins and
dural venous sinuses appear normal which suggestive of congenital
agenesis of jugular venous system of left side\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/Figure-01/Figure-01}

\end{center}
\end{figure}\selectlanguage{english}
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\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/Figure-02/Figure-02}

\end{center}
\end{figure}

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