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A Case of Vernet Syndrome Associated With Internal Jugular Phlebectasia
Case Presentation
A Case of Vernet Syndrome Associated With Internal Jugular Phlebectasia Nicholas C. Daley, CSCS, Ethan B. Colliver, DO A 36-year-old woman presented with right shoulder weakness after a left parotid tumor resection. The overall clinical presentation included severe paralysis and atrophy of the right sternocleidomastoid and upper trapezius, an absent right gag reflex, and diminished right posterior tongue pinprick sensation. A diagnosis of right-sided Vernet syndrome (cranial nerve IX, X, XI lesions) was made, presumably from compression of cranial nerves by internal jugular vein phlebectasia. To our knowledge, this is the first case report of spontaneous Vernet syndrome associated with internal jugular vein phlebectasia in the absence of other lesions of the jugular foramen. PM R 2014;6:1163-1165
INTRODUCTION Vernet syndrome, often referred to as jugular foramen syndrome, is characterized by an intracranial abnormality affecting cranial nerves (CNs) IX, X, and XI, often causing loss of taste in the posterior one third of the tongue, vocal paralysis, and anesthesia of both the larynx and pharynx and sternocleidomastoid (SCM) and trapezius weakness, respectively [1]. Jugular foramen syndrome can be caused by multiple etiologies, with various lesions affecting the foramen (Table 1) [1]. The jugular foramen is located posterior to the carotid canal and is formed anteriorly by the petrous portion of the temporal bone and posteriorly by the occipital bone [2]. The foramen has been characterized as being generally greater in diameter on the right side of the skull versus the left (averaging 1.5 cm in length and 1.0 cm in width) [3]. It has been described to include a larger posterolateral portion (pars vascularis) containing the internal jugular vein (IJV), CN X, and CN XI and the smaller anteromedial portion (pars nervosa) containing CN IX and the inferior petrosal sinus (Figure 1) [2]. The right IJV normally has a greater diameter and cross-sectional area than the left, with a median area of 160 mm2 (range 108-235 mm2) [4]; however, internal jugular venous phlebectasia (IJVP) is described as a rare vascular entity resulting in an anomalously dilated IJV, primarily of the right side, and can be diagnosed by physical examination findings and imaging [2,4-7]. The finding is usually incidental, and although a cause for IJVP is not known, intrathoracic causes have been suggested, such as anomalous reduplication of the jugular vein, pressure over the brachiocephalic vein, and compression by the scalene muscles [5]. The etiology of Vernet syndrome often is attributed to malignancy, embolism, infection, thrombosis, aneurysm, injury, or fracture [2,8]. We are unaware of any reported cases of Vernet syndrome arising from compression of CNs by the IJVP. We report a case with Vernet syndrome associated with IJVP in which imaging shows a large dilated right IJV from the brachiocephalic vein to the jugular bulb at the entrance of the jugular foramen.
CASE REPORT A 36-year-old woman, traveling on holiday, presented to the author with a 6-year history of right shoulder weakness after a left parotid tumor resection. She noticed the weakness most during upper body strengthening exercise. She described chronic, constant weakness, PM&R 1934-1482/14/$36.00 Printed in U.S.A.
N.C.D. Edward Via College of Osteopathic Medicine, Virginia Campus, Blacksburg, VA Disclosure: nothing to disclose E.B.C. Edward Via College of Osteopathic Medicine, Virginia Campus, Blacksburg, VA. Address correspondence to: E.B.C., Edward Via College of Osteopathic Medicine - Virginia Campus, 1711 Emerald Street, Valley Sports & Spine Clinic, Blacksburg, VA 24060; e-mail: [email protected] Disclosure: nothing to disclose Submitted for publication September 7, 2013; accepted May 13, 2014.
ª 2014 by the American Academy of Physical Medicine and Rehabilitation Vol. 6, 1163-1165, December 2014 http://dx.doi.org/10.1016/j.pmrj.2014.05.025
1163
1164
Daley and Colliver
VERNET SYNDROME ASSOCIATED WITH IJVP
Table 1. Lesions of the jugular foramen Congenital Vascular
Infectious
Neoplastic
Trauma
Primary cholesteatoma Asymmetrically enlarged jugular foramen High jugular bulb Protruding jugular bulb Jugular thrombosis Aneurysm Embolism Jugular diverticulum Abscess Cholesteatoma Pyocele Paraganglioma Metastasis Squamous cell carcinoma Schwannoma (affecting cranial nerves IX-XII) Meningioma Multiple myeloma Chondrosarcoma Neurofibroma Lymphoma Histiocytosis Chordoma Fibrosarcoma Hemangiopericytoma Penetrating trauma Fracture
Adapted from Caldemeyer et al [2].
with nothing making it better or worse. She denied left shoulder weakness, chest pain, shortness of breath, right arm swelling, dysphagia, and dysphonia. On review of her records, magnetic resonance imaging (MRI) of the cervical spine showed C6 C7 degenerative disk disease and mild spinal stenosis, and computed tomography (CT) of the neck revealed “stable marked atrophy of the SCM and trapezius muscles on the right side suggestive of spinal accessory nerve denervation/injury (and) no evidence of mass lesions or bony destruction at the skull base.” No further investigation was expected. Upon examination by the author, atrophy of the right SCM and upper trapezius was present, an absent right gag reflex, and diminished right posterior tongue pinprick sensation. The remaining right upper limb was 5/5 with muscle strength testing. A dilated right IJV was visualized, and increased jugular venous distention was seen with Valsalva maneuver. Pulsation of the vein was seen in supine position. Intact and equal pulses were palpated in all 4 limbs. The authors independently reviewed the previous diagnostic studies to evaluate for congenital, vascular, neoplastic, or trauma-related causes for the apparent neurologic and vascular findings. MRI of the patient’s brain and neck from before her surgery revealed minimal atrophy of her right SCM and a dilated right jugular vein (288 mm2 compared with the average of 160 mm2) distal to the jugular foramen with no visible lesions or thrombi. CT imaging of the patient’s head, neck, and brain with and without intravenous contrast, from 6 years after her surgery,
Figure 1. A cranial computed tomography of the patient showing contents of skull base: internal carotid artery (IC) in carotid canal, sigmoid sinus (SS), pars vascularis (PV) portion of jugular foramen containing internal jugular vein, cranial nerves X, XI, and Arnold’s nerve; the outlined pars nervosa (asterisk) portion of jugular foramen containing cranial nerves IX and receiving the venous return from inferior petrosal sinus.
demonstrated a severely atrophied right SCM and dilated right IJV down to the origin of the right brachiocephalic vein (Figure 2). The diagnosis of right-sided Vernet syndrome associated with a dilated right IJV from the right jugular foramen down to the brachiocephalic vein was made on the basis of her neurological and musculoskeletal examination as well as imaging studies. The patient was referred to a vascular surgeon and referred for electrodiagnostic studies. A CT venogram from 7 years after her surgery revealed a dilated right IJV with no visible lesions of the jugular foramen. Electrodiagnostic studies, performed by an outside neurologist, demonstrated normal motor and sensory studies of the right upper limb, except for reduced amplitude of the right spinal accessory nerve. Needle electromyography demonstrated 20%-30% recruitment response from the right trapezius, with no mention of spontaneous activity. Laboratory studies were negative for Ehrlichia chaffeensis infection, babesiosis, Lyme disease, and Sjögren syndrome. The complete blood cell count with differential, sedimentation rate, thyroidstimulating hormone, angiotensin-converting enzyme, and serum protein electrophoresis were all within normal limits as well. Because extensive work-up had failed to elicit an infectious, auto-immune, vasculitic, or rheumatologic cause for the patient’s symptoms, and because the patient did not present with any acute neurologic deficit or instability
PM&R
Vol. 6, Iss. 12, 2014
1165
foramen from IJVP at the jugular bulb during a parotid tumor resection. Potential explanations for the increased pressure may have occurred during mechanical ventilation, from a transient thrombosis of the jugular vein or cerebral venous system, or from neck manipulation perioperatively. Treatment for Vernet syndrome is based on the etiology of eliciting factors. For instance, surgical intervention has been used for acute and chronic palsies in the spinal accessory nerve but has been shown less likely to succeed in patients with a spontaneous nerve palsy [9]. The goal of rehabilitation is to maintain function of swallowing and use therapies to stimulate and strengthen intact motor units of the affected muscle thereby retaining, at best, the limited function caused by the palsy [10].
CONCLUSIONS
Figure 2. A cervical computed tomography venogram of the patient showing dilated right internal jugular vein (IJV) and atrophied right sternocleidomastoid (SCM).
requiring intervention, conservative management was chosen. The patient continues to have stable right shoulder weakness without dysphagia or dysphonia. She is currently an exercise instructor and an active mother of 2 girls.
DISCUSSION Idiopathic IJVP is rare and is generally present on the right. In our review of the literature, we did not find an association of jugular phlebectasia with Vernet syndrome. A case report of jugular phlebectasia, hoarseness, and SCM weakness in an otherwise-healthy 10 year-old South American girl does suggest this association [6]; however, the authors did not define it as Vernet syndrome [6]. We believe ours is the first published case of Vernet syndrome occurring spontaneously as the result of IJVP. Although the Valsalva maneuver is the most useful clinical sign used to diagnose the dilation of IJV, CT, Doppler sonogram, and MRI are the most useful imaging studies to further investigate IJVP [5-7]. In our case, a head and neck MRI (with and without contrast) was supplemented with CT and CT venogram to obtain a more thorough evaluation of the vascular anomaly. Complete blood work was performed to evaluate for an infectious or autoimmune process. We theorize that the Vernet syndrome found in our patient was caused by compression of CNs at the jugular
Both Vernet syndrome and jugular phlebectasia are rare occurrences. In this case, no clear active neoplastic, infectious, thrombotic, embolismic, traumatic, or autoimmune process was uncovered. We theorize that compression of the CNs at the jugular foramen by IJVP caused this Vernet syndrome. Treatment was conservative, and the patient remains stable.
REFERENCES 1. Robbins KT, Fenton RS. Jugular foramen syndrome. J Otolaryngol 1980;9:505-516. 2. Caldemeyer KS, Mathews VP, Azzarelli B, Smith RR. The jugular foramen: A review of anatomy, masses, and imaging characteristics. Radiographics 1997;17:1123-1139. 3. Hussain Saheb S, Mavishetter GF, Thomas ST, Prasanna LC, Muralidhar P. A morphometric study of the jugular foramen in human adult skulls of south India. J Biomed Sci Res 2010;2:240-243. 4. Tartière D, Seguin P, Juhel C, Laviolle B, Mallédant Y. Estimation of the diameter and cross-sectional area of the internal jugular veins in adult patients. Crit Care 2009;13:R197. 5. Thulasiraman V, Ramesh Pandian T, Cheralathan S, Ashok S. Internal jugular phlebectasia as an incidental finding in cervical spine surgery. Indian J Orthop 2010;44:471-473. 6. Mv Souza A, Guerra AFM, Assis Lima TM, de Campos Duprat A. Jugular phlebectasia: A cause of neck swelling—Case report. Int J Pediatr Otorhinolaryngol Extra 2008;3:158-160. 7. Mickelson SA, Spickler E, Roberts K. Management of internal jugular vein Phlebectasia. Otolaryngol Head Neck Surg 1995;112:473-475. 8. Legros B, Fournier P, Chiaroni P, Ritz O, Fusciardi J. Basal Fracture of the Skull and Lower (IX, X, XI, XII) cranial nerves palsy: four case reports including two fractures of the occipital condyle—a literature review. J Trauma 2000;48(2):342-348. 9. Teboul F, Bizot P, Kakkar R, Sedel L. Surgical management of trapezius palsy. J Bone Joint Surg Am 2005;87(Suppl 1):285-291. 10. Cheesman AD, Kelly AM. Rehabilitation after treatment for jugular foramen lesions. Skull Base 2009;19:99-108.
A Case of Vernet Syndrome Associated With Internal Jugular Phlebectasia Nicholas C. Daley, CSCS, Ethan B. Colliver, DO A 36-year-old woman presented with right shoulder weakness after a left parotid tumor resection. The overall clinical presentation included severe paralysis and atrophy of the right sternocleidomastoid and upper trapezius, an absent right gag reflex, and diminished right posterior tongue pinprick sensation. A diagnosis of right-sided Vernet syndrome (cranial nerve IX, X, XI lesions) was made, presumably from compression of cranial nerves by internal jugular vein phlebectasia. To our knowledge, this is the first case report of spontaneous Vernet syndrome associated with internal jugular vein phlebectasia in the absence of other lesions of the jugular foramen. PM R 2014;6:1163-1165
INTRODUCTION Vernet syndrome, often referred to as jugular foramen syndrome, is characterized by an intracranial abnormality affecting cranial nerves (CNs) IX, X, and XI, often causing loss of taste in the posterior one third of the tongue, vocal paralysis, and anesthesia of both the larynx and pharynx and sternocleidomastoid (SCM) and trapezius weakness, respectively [1]. Jugular foramen syndrome can be caused by multiple etiologies, with various lesions affecting the foramen (Table 1) [1]. The jugular foramen is located posterior to the carotid canal and is formed anteriorly by the petrous portion of the temporal bone and posteriorly by the occipital bone [2]. The foramen has been characterized as being generally greater in diameter on the right side of the skull versus the left (averaging 1.5 cm in length and 1.0 cm in width) [3]. It has been described to include a larger posterolateral portion (pars vascularis) containing the internal jugular vein (IJV), CN X, and CN XI and the smaller anteromedial portion (pars nervosa) containing CN IX and the inferior petrosal sinus (Figure 1) [2]. The right IJV normally has a greater diameter and cross-sectional area than the left, with a median area of 160 mm2 (range 108-235 mm2) [4]; however, internal jugular venous phlebectasia (IJVP) is described as a rare vascular entity resulting in an anomalously dilated IJV, primarily of the right side, and can be diagnosed by physical examination findings and imaging [2,4-7]. The finding is usually incidental, and although a cause for IJVP is not known, intrathoracic causes have been suggested, such as anomalous reduplication of the jugular vein, pressure over the brachiocephalic vein, and compression by the scalene muscles [5]. The etiology of Vernet syndrome often is attributed to malignancy, embolism, infection, thrombosis, aneurysm, injury, or fracture [2,8]. We are unaware of any reported cases of Vernet syndrome arising from compression of CNs by the IJVP. We report a case with Vernet syndrome associated with IJVP in which imaging shows a large dilated right IJV from the brachiocephalic vein to the jugular bulb at the entrance of the jugular foramen.
CASE REPORT A 36-year-old woman, traveling on holiday, presented to the author with a 6-year history of right shoulder weakness after a left parotid tumor resection. She noticed the weakness most during upper body strengthening exercise. She described chronic, constant weakness, PM&R 1934-1482/14/$36.00 Printed in U.S.A.
N.C.D. Edward Via College of Osteopathic Medicine, Virginia Campus, Blacksburg, VA Disclosure: nothing to disclose E.B.C. Edward Via College of Osteopathic Medicine, Virginia Campus, Blacksburg, VA. Address correspondence to: E.B.C., Edward Via College of Osteopathic Medicine - Virginia Campus, 1711 Emerald Street, Valley Sports & Spine Clinic, Blacksburg, VA 24060; e-mail: [email protected] Disclosure: nothing to disclose Submitted for publication September 7, 2013; accepted May 13, 2014.
ª 2014 by the American Academy of Physical Medicine and Rehabilitation Vol. 6, 1163-1165, December 2014 http://dx.doi.org/10.1016/j.pmrj.2014.05.025
1163
1164
Daley and Colliver
VERNET SYNDROME ASSOCIATED WITH IJVP
Table 1. Lesions of the jugular foramen Congenital Vascular
Infectious
Neoplastic
Trauma
Primary cholesteatoma Asymmetrically enlarged jugular foramen High jugular bulb Protruding jugular bulb Jugular thrombosis Aneurysm Embolism Jugular diverticulum Abscess Cholesteatoma Pyocele Paraganglioma Metastasis Squamous cell carcinoma Schwannoma (affecting cranial nerves IX-XII) Meningioma Multiple myeloma Chondrosarcoma Neurofibroma Lymphoma Histiocytosis Chordoma Fibrosarcoma Hemangiopericytoma Penetrating trauma Fracture
Adapted from Caldemeyer et al [2].
with nothing making it better or worse. She denied left shoulder weakness, chest pain, shortness of breath, right arm swelling, dysphagia, and dysphonia. On review of her records, magnetic resonance imaging (MRI) of the cervical spine showed C6 C7 degenerative disk disease and mild spinal stenosis, and computed tomography (CT) of the neck revealed “stable marked atrophy of the SCM and trapezius muscles on the right side suggestive of spinal accessory nerve denervation/injury (and) no evidence of mass lesions or bony destruction at the skull base.” No further investigation was expected. Upon examination by the author, atrophy of the right SCM and upper trapezius was present, an absent right gag reflex, and diminished right posterior tongue pinprick sensation. The remaining right upper limb was 5/5 with muscle strength testing. A dilated right IJV was visualized, and increased jugular venous distention was seen with Valsalva maneuver. Pulsation of the vein was seen in supine position. Intact and equal pulses were palpated in all 4 limbs. The authors independently reviewed the previous diagnostic studies to evaluate for congenital, vascular, neoplastic, or trauma-related causes for the apparent neurologic and vascular findings. MRI of the patient’s brain and neck from before her surgery revealed minimal atrophy of her right SCM and a dilated right jugular vein (288 mm2 compared with the average of 160 mm2) distal to the jugular foramen with no visible lesions or thrombi. CT imaging of the patient’s head, neck, and brain with and without intravenous contrast, from 6 years after her surgery,
Figure 1. A cranial computed tomography of the patient showing contents of skull base: internal carotid artery (IC) in carotid canal, sigmoid sinus (SS), pars vascularis (PV) portion of jugular foramen containing internal jugular vein, cranial nerves X, XI, and Arnold’s nerve; the outlined pars nervosa (asterisk) portion of jugular foramen containing cranial nerves IX and receiving the venous return from inferior petrosal sinus.
demonstrated a severely atrophied right SCM and dilated right IJV down to the origin of the right brachiocephalic vein (Figure 2). The diagnosis of right-sided Vernet syndrome associated with a dilated right IJV from the right jugular foramen down to the brachiocephalic vein was made on the basis of her neurological and musculoskeletal examination as well as imaging studies. The patient was referred to a vascular surgeon and referred for electrodiagnostic studies. A CT venogram from 7 years after her surgery revealed a dilated right IJV with no visible lesions of the jugular foramen. Electrodiagnostic studies, performed by an outside neurologist, demonstrated normal motor and sensory studies of the right upper limb, except for reduced amplitude of the right spinal accessory nerve. Needle electromyography demonstrated 20%-30% recruitment response from the right trapezius, with no mention of spontaneous activity. Laboratory studies were negative for Ehrlichia chaffeensis infection, babesiosis, Lyme disease, and Sjögren syndrome. The complete blood cell count with differential, sedimentation rate, thyroidstimulating hormone, angiotensin-converting enzyme, and serum protein electrophoresis were all within normal limits as well. Because extensive work-up had failed to elicit an infectious, auto-immune, vasculitic, or rheumatologic cause for the patient’s symptoms, and because the patient did not present with any acute neurologic deficit or instability
PM&R
Vol. 6, Iss. 12, 2014
1165
foramen from IJVP at the jugular bulb during a parotid tumor resection. Potential explanations for the increased pressure may have occurred during mechanical ventilation, from a transient thrombosis of the jugular vein or cerebral venous system, or from neck manipulation perioperatively. Treatment for Vernet syndrome is based on the etiology of eliciting factors. For instance, surgical intervention has been used for acute and chronic palsies in the spinal accessory nerve but has been shown less likely to succeed in patients with a spontaneous nerve palsy [9]. The goal of rehabilitation is to maintain function of swallowing and use therapies to stimulate and strengthen intact motor units of the affected muscle thereby retaining, at best, the limited function caused by the palsy [10].
CONCLUSIONS
Figure 2. A cervical computed tomography venogram of the patient showing dilated right internal jugular vein (IJV) and atrophied right sternocleidomastoid (SCM).
requiring intervention, conservative management was chosen. The patient continues to have stable right shoulder weakness without dysphagia or dysphonia. She is currently an exercise instructor and an active mother of 2 girls.
DISCUSSION Idiopathic IJVP is rare and is generally present on the right. In our review of the literature, we did not find an association of jugular phlebectasia with Vernet syndrome. A case report of jugular phlebectasia, hoarseness, and SCM weakness in an otherwise-healthy 10 year-old South American girl does suggest this association [6]; however, the authors did not define it as Vernet syndrome [6]. We believe ours is the first published case of Vernet syndrome occurring spontaneously as the result of IJVP. Although the Valsalva maneuver is the most useful clinical sign used to diagnose the dilation of IJV, CT, Doppler sonogram, and MRI are the most useful imaging studies to further investigate IJVP [5-7]. In our case, a head and neck MRI (with and without contrast) was supplemented with CT and CT venogram to obtain a more thorough evaluation of the vascular anomaly. Complete blood work was performed to evaluate for an infectious or autoimmune process. We theorize that the Vernet syndrome found in our patient was caused by compression of CNs at the jugular
Both Vernet syndrome and jugular phlebectasia are rare occurrences. In this case, no clear active neoplastic, infectious, thrombotic, embolismic, traumatic, or autoimmune process was uncovered. We theorize that compression of the CNs at the jugular foramen by IJVP caused this Vernet syndrome. Treatment was conservative, and the patient remains stable.
REFERENCES 1. Robbins KT, Fenton RS. Jugular foramen syndrome. J Otolaryngol 1980;9:505-516. 2. Caldemeyer KS, Mathews VP, Azzarelli B, Smith RR. The jugular foramen: A review of anatomy, masses, and imaging characteristics. Radiographics 1997;17:1123-1139. 3. Hussain Saheb S, Mavishetter GF, Thomas ST, Prasanna LC, Muralidhar P. A morphometric study of the jugular foramen in human adult skulls of south India. J Biomed Sci Res 2010;2:240-243. 4. Tartière D, Seguin P, Juhel C, Laviolle B, Mallédant Y. Estimation of the diameter and cross-sectional area of the internal jugular veins in adult patients. Crit Care 2009;13:R197. 5. Thulasiraman V, Ramesh Pandian T, Cheralathan S, Ashok S. Internal jugular phlebectasia as an incidental finding in cervical spine surgery. Indian J Orthop 2010;44:471-473. 6. Mv Souza A, Guerra AFM, Assis Lima TM, de Campos Duprat A. Jugular phlebectasia: A cause of neck swelling—Case report. Int J Pediatr Otorhinolaryngol Extra 2008;3:158-160. 7. Mickelson SA, Spickler E, Roberts K. Management of internal jugular vein Phlebectasia. Otolaryngol Head Neck Surg 1995;112:473-475. 8. Legros B, Fournier P, Chiaroni P, Ritz O, Fusciardi J. Basal Fracture of the Skull and Lower (IX, X, XI, XII) cranial nerves palsy: four case reports including two fractures of the occipital condyle—a literature review. J Trauma 2000;48(2):342-348. 9. Teboul F, Bizot P, Kakkar R, Sedel L. Surgical management of trapezius palsy. J Bone Joint Surg Am 2005;87(Suppl 1):285-291. 10. Cheesman AD, Kelly AM. Rehabilitation after treatment for jugular foramen lesions. Skull Base 2009;19:99-108.