Case Report - (2022) Volume 13, Issue 6
We herein report a case of bilateral Cavernous Sinus Thrombosis in a 40-yearold male in the setting of nasopharyngeal adenocarcinoma of unknown primary origin. Cavernous Sinus.Thrombosis presents with an abrupt onset of unilateral periorbital edema, headache, photophobia and bulging of the eye. Other common presenting symptoms include ptosis, chemosis and cranial nerve palsies. The patient was initially treated with anticoagulation before further evaluation revealed the extensive metastatic disease. The clinical presentation, examination findings, radiological evidence and management have been discussed here.
Cavernous Sinus Thrombosis (CST) is a very rare condition with an incidence of 0.2-1.6 cases per 100,000 per annum [1]. Out of all cases of cerebral and dural venous sinus thrombosis, malignancy as a cause accounts for 7.4% which shows that the risk of CST in malignancy is increased by 5-fold [2].
It was associated with high mortality in the pre-antibiotic era [3]. Even with the use of antibiotics and other modern methods of medical management, the mortality rate is still high at 30% with up to 50% having residual cranial neuropathies [4]. Hence the correct identification of the cause of CST is paramount for appropriate management.
Our patient had aggressive occult malignancy with disseminated metastasis and he presented primarily with symptoms of CST. Diagnosis of Cavernous Sinus Thrombosis is quite challenging and requires a deeper understanding of the disease process and a high index of clinical suspicion, as these patients do not always present with classical symptoms, making it more challenging [5]. Malignancy is an extremely rare cause of CST and here we present a unique case of left-sided CST that eventually progressed to the right side in a 40-year-old male with extensive metastasis of adenocarcinoma of unknown primary origin.
A 40-year-old male presented, before hospitalization, with symptoms of double vision for 10 days,left-sided headache for 7 days, and ptosis of the left eye (Figure1) for 7 days.
Figure 1: Image of the patient showing proptosis and drooping of the left eyelid
His double vision was acute in onset, both the images one higher than the other and with the closing of either eye. It was associated with blurring of vision and pain with movement of the left eye but was not associated with head tilt. He developed a headache 7 days before hospitalization which was sudden in onset, gradually progressive, unilateral, and pulsatile, involving the temple region. It was associated with blurring of vision, not associated with photophobia, phonophobia, nausea, or vomiting. He also developed drooping of the left eyelid 7 days before hospitalization. Initially, the drooping was partial but it progressed to the stage where the eyelid completely closed the eyeball and the patient used his hands to lift the eyelid.
At admission, the patient was afebrile, blood pressure was 130 mmHg /80 mmHg, respiratory rate was 14/min, pulse rate was 88 bpm, and BMI-17.9 kg/m2. The patient had a history of significant weight loss of 7 kilograms in the preceding 6 months. He had no history of trauma, seizures, fever, cough, cold, ear discharge, dental infections or procedures, or skin infections. He was not an alcoholic or smoker.
On physical examination, the patient had complete ptosis and absent pupil reflex of the left eye. Two days after admission, right eye also developed ptosis and absent pupil reflexes. He had non-tender, palpable left cervical lymph nodes, two in number,2cm in size, firm and matted in consistency. He had decreased sensations in the distribution of ophthalmic nerves on the left side (Table 1).
Table 1. lists the Routine Blood Investigations done at admission
| Parameter | Value | Normal |
|---|---|---|
| Haemoglobin | 12.4 g/dl | 13.5 g/dl to 17.5 g/dl |
| Leukocyte count | 11400/µL | 4500/µL to 11500/µL |
| Platelet count | 128000/mm3 | 150000/mm3 to 450000/mm3 |
| Serum creatinine | 0.64 mg/dl | 0.6 mg/dl to 1.2 mg/dl |
| Blood urea | 56.4 mg/dl | 7 mg/dl to 18 mg/dl |
| Total bilirubin | 1.9 mg/dl | 0.30 mg/dl to 1.00 mg/dl |
| ALP | 458 µ/l | 40 µ/l to 150 µ/l |
| Total protein | 6.6 g/dl | 6 g/dl to 8 g/dl |
| Albumin | 2.83 g/dl | 5.50 g/dl |
Blood tests and Chest X-Ray were done. Ultrasound-guided fine-needle aspiration was used for the evaluation of palpable neck lymph nodes. CT Brain was done to evaluate and confirm the diagnosis of CST.
Serum Electrolytes are normal
Chest X-Ray findings were normal: CT Brain at the time of admission showed swollen left cavernous sinus, convexity of the lateral wall, asymmetry, and exophthalmos. FNAC revealed atypical epithelial cells arranged in papillary and acinar patterns. The report concluded with the impression of metastatic lesions of epithelial malignancy - possibility of adenocarcinomatous deposits. CT chest and abdomen along with PET scan were done to know the extent of metastatic disease (Figures 2-4).
Figure 2: Axial CT section of head showing evidence of mild hyper density noted in the region of the left cavernous sinus and orbital apex.
Figure 3: Coronal CT section of head showing fluid attenuation in sphenoid sinus adjacent to cavernous sinus suggestive of sphenoid sinusitis.
Figure 4: Axial CT section of head showing Sphenoid sinusitis.
CT Findings of the patient: Initially, non-contrast CT was done on the patient and the findings revealed evidence of hyperdense left cavernous sinus. Evidence of soft tissue mucosal thickening was noted in the sphenoid sinus and mild atrophy of the parasagittal, parietal and occipital lobes of the left cerebral hemisphere, likely due to cavernous sinus thrombosis.
PET scan revealed the presence of metastases in the nasopharynx infiltrating the skull base, floor of the mouth, cervical, mediastinal, abdominal lymph node metastasis, liver and splenic metastasis, and axial and appendicular skeletal metastasis with soft tissue involvement (Figures 5-8).
Figure 5: PET CT scan showing evidence of abnormal metabolic activity suggesting a tumor, in the skull base
Figure 6: PET CT scan showing abnormal FDG (Fluorodeoxyglucose) uptake in the floor of the mouth, cervical lymph nodes, mediastinal lymph nodes, cervical and thoracic spine, and sternum.
Figure 7: PET CT scan showing metastasis in the skull, abdomen, appendicular and axial skeleton.
Figure 8: Axial section of PET CT scan showing abnormal FDG uptake in liver and spleen.
The patient received empiric antibiotics( Nafcillin 1g every 6 hours and Ceftriaxone 1 g every 12 hours) and anticoagulants immediately after CT confirmation of CST. Chemotherapy with Cisplatin and Fluorouracil regimen was also started [6]. The patient had an Intracranial bleed on day 7 and he passed away. Hence further investigations could not be performed and the primary origin of the tumor remained unknown.
The cavernous sinuses are dural venous sinuses that communicate with one another. Each cavernous sinus is flanked laterally by the temporal bone of the skull and inferiorly by the sphenoid bone, with proximity to the sphenoid sinuses. The pituitary gland sits within the sella turcica which exists medial to the cavernous sinus, and the optic chiasm lies superior to the cavernous sinus on the midline close to the pituitary gland. Each cavernous sinus receives venous drainage from several structures within the face and eye. The superior and inferior ophthalmic veins drain anteriorly into the sinus. The superficial middle cerebral veins, deep cerebral veins (via the sphenoparietal sinus), and inferior cerebral veins drain into the cavernous sinus as well. Inferiorly, the cavernous sinus drains to the pterygoid plexus and posteriorly, it communicates with the superior and inferior petrosal sinuses. Both drainage systems ultimately converge at the internal jugular vein. The cavernous sinus holds many important structures like Cranial nerves - Oculomotor III, Trochlear IV, Trigeminal V1, V2 in its lateral wall while Abducens VI and Internal Carotid Artery, Carotid plexus within the substance. Cavernous Sinus Syndrome (CSS) is a condition caused by any pathology involving the cavernous sinus which may present as a combination of unilateral ophthalmoplegia (cranial nerves (CN) III, IV, VI), autonomic dysfunction (Horner syndrome) or sensory trigeminal (V1-V2) loss (Figure 9-10).
Figure 9: Anatomy of the cavernous sinus [7].
Figure 10: Drainage of cerebral venous sinuses [8].
Cavernous Sinus Thrombosis in and itself is a very rare condition and malignancy as a cause of CST is indeed very rare. To date, only a few such cases have been reported in the scientific literature. The most common causes of CST are infectious etiology like cellulitis, sinusitis, otitis media, mastoiditis, dental infections, and skin infections in the danger triangle of the face (the line joining the corner of the mouth to the bridge of the nose). Septic CST is treated with appropriate antibiotics and surgical drainage of the source of infection [6]. Aseptic causes of CST include trauma, surgery, pregnancy, oral contraceptive pill usage, and malignancy. Coagulopathy in cancer is speculated to be an adverse effect of radiation causing vessel injury or chemotherapy itself, compression, or infiltrating the vasculature [9]. Aseptic cases of CST are treated with anticoagulants and thrombolytic surgeries. Thrombocytopenia due to the infiltrating malignant disease and treatment greatly increase the risk of bleeding in patients on anticoagulant therapy [10]. Long Term anticoagulation with Vitamin K agonists or Dabigatran is proved to be efficient in a random control trial [11].LMWH caused fewer bleeding complications than warfarin in a study[12]. However, anticoagulants showed a narrow therapeutic index in cancer patients and have to be carefully monitored [12].
CST in the setting of malignancy is very rare and there are very few cases reported in the literature. Risks and benefits should be carefully assessed individually while considering anticoagulation as a treatment avenue in patients with malignancy.
Citation: Sravan Kumar Reddy E, Greeshma E, Sai Kumar Reddy P, et al. A Case Report of Cavernous Sinus Thrombosis in a 40- year-old patient with Adenocarcinoma of unknown primary origin. J Neurol Neurophysiol, 2022, 13(6), 001-004.
Received: 20-May-2022, Manuscript No. JNN-22-66827; Editor assigned: 24-May-2022, Pre QC No. JNN-22-66827 (PQ); Reviewed: 24-May-2022, QC No. JNN-22-66827 (PQ); Revised: 31-May-2022, Manuscript No. JNN-22-66827 (R); Published: 22-Jun-2022, DOI: 10.35248/2471-268X.22.13.6.583
Copyright: ©2022 Popescu C, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
