The Journal of Craniofacial Surgery • Volume 26, Number 2, March 2015
Brief Clinical Studies
FIGURE 2. After 2 weeks of applying topical nystatin paste and peroral 200 mg/d itraconazole, areas of plaque regressed and disappeared.
FIGURE 3. After 1 year, the manifestation of the oral lesions seems to be similar to that of his first admission.
syndrome have a higher risk of systemic lupus erythematosus and other skin-related autoimmune diseases than do those without this syndrome. Humoral and cellular immunity are enhanced in patients with Klinefelter syndrome.8 But the oral lesions have rarely been reported in Klinefelter syndrome to our knowledge. Only 1 case report9 mentioned that a patient with Klinefelter syndrome had oral thrush. However, no particular oral clinical history or examination had been mentioned in the case. That patient also had multiple abnormal manifestations, such as anemia and defect of humoral immunity. The relationship between Klinefelter syndrome and oral thrush requires further investigation. On the basis of clinical diagnosis and the result of antifungal susceptibility testing, topical nystatin paste and peroral 200 mg/d itraconazole were performed. At review after 2 weeks, areas of plaque regressed and disappeared (Fig. 2). During subsequent visits, he performed recurrent symptoms of oral thrush for about 1 year. It seems that this kind of oral thrush is inveterate and persistent (Fig. 3). We do not have more effective therapeutic measures to restrain this stubborn disease because the real etiology is not clear yet.
DISCUSSION Klinefelter syndrome is a genetic nonhereditary disorder with genotype 47 XXY. Dermatologically, patients with Klinefelter syndrome have a higher risk of systemic lupus erythematosus and other skin-related autoimmune diseases than do those without this syndrome.8 Only 1 case report9 described that a patient with Klinefelter syndrome had oral thrush. However, no detailed message had been mentioned. Moreover, multiple abnormal manifestations were also noticed in that case, such as anemia and defect of humoral immunity, which is distinct from our case. In summary, we report a patient with pseudomembranous stomatitis with a persistent course and unknown cause. The relationship between Klinefelter syndrome and oral thrush requires further investigations.
REFERENCES 1. Williams DW, Kuriyama T, Silva S, et al. Candida biofilms and oral candidosis: treatment and prevention. Periodontol 2000 2011; 55:250–265 2. Kirkpatrick WR, Revankar SG, McAtee RK, et al. Detection of Candida dubliniensis in oropharyngeal samples from human immunodeficiency virus-infected patients in North America by primary CHROMagar Candida screening and susceptibility testing of isolates. J Clin Microbiol 1998;36:3007–3012
e108
3. Sullivan D, Coleman D. Candida dubliniensis: characteristics and identification. J Clin Microbiol 1998;36:329–334 4. Espinel-Ingroff A, Canton E, Gibbs D, et al. Correlation of Neo-Sensitabs tablet diffusion assay results on three different agar media with CLSI broth microdilution M27-A2 and disk diffusion M44-A results for testing susceptibilities of Candida spp. and Cryptococcus neoformans to amphotericin B, caspofungin, fluconazole, itraconazole, and voriconazole. J Clin Microbiol 2007;45:858–864 5. Williams D, Lewis M. Pathogenesis and treatment of oral candidosis. J Oral Microbiol 2011;3:5771–5781 6. Rugg EL, McLean WH, Allison WE, et al. A mutation in the mucosal keratin K4 is associated with oral white sponge nevus. Nat Genet 1995;11:450–452 7. Shah DN, Yau R, Weston J, et al. Evaluation of antifungal therapy in patients with candidaemia based on susceptibility testing results: implications for antimicrobial stewardship programmes. J Antimicrob Chemother 2011;66:2146–2151 8. Jimenez-Balderas FJ, Tapia-Serrano R, Fonseca ME, et al. High frequency of association of rheumatic/autoimmune diseases and untreated male hypogonadism with severe testicular dysfunction. Arthritis Res 2001;3:362–367 9. de Morentin HM, Dodiuk-Gad RP, Brenner S. Klinefelter's syndrome presenting with leg ulcers. Skinmed 2004;3:274–278
Acute Venous Sinus Thrombosis Induced by a Jugular Foramen Neurinoma Quanmin Nie, PhD, Pin Guo, PhD, Liemei Guo, PhD, Xiaoxiong Li, MD, Jianwei Ge, PhD, Yongming Qiu, MD, PhD
J
ugular foramen lesions are often associated with pathology of adjacent structures owing to either compression or direct invasion. The most common symptom of a jugular foramen tumor is involved in cranial nerve (mainly IX, X, and XI) damage. However, symptoms associated with the jugular vein at the first onset are rare,1 which we presumed is caused by a lower incidence of tumors originating from the jugular vein, chronic occlusion of the jugular vein with no specific symptoms, or slight clinical symptoms that were unnoticed by the patient. Up to date, there has been no previous report on acute cerebral venous sinus thrombosis induced by a jugular foramen tumor. A 45-year-old man was hospitalized, with complaints of unbearable headaches and vomiting for 1 hour after jogging for practice. There was no relevant medical history to note. On initial examination, ipsilateral pharyngeal reflex disappearance and papilledema were found. The results of routine blood examinations were normal, with a normal coagulation profile. Cranial computed tomography From the Department of Neurosurgery, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China. Received July 27, 2014. Accepted for publication September 13, 2014. Address correspondence and reprint requests to Dr. Yongming Qiu, Department of Neurosurgery, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, No.1630, Dongfang Rd, Shanghai 200127, China; E-mail: [email protected] The authors report no conflicts of interest. Copyright © 2015 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001332
© 2015 Mutaz B. Habal, MD
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery • Volume 26, Number 2, March 2015
FIGURE 1. The CT scans of the patient. The cranial CT scans show multiple hemorrhages with massive edema (A) and a jugular foramen mass (B) with bone destruction (C).
FIGURE 2. The magnetic resonance images of the patient. The cranial magnetic resonance images show that the jugular foramen mass was isointense on T1-weighted images (A) and hyperintense on T2-weighted images (B). The neoplasm demonstrated moderate enhancement after contrast administration (C), and its size was approximately 2.6 1.9 3.3 cm.
(CT) showed multiple hemorrhages with massive edema and a jugular foramen mass with bone destruction (Fig. 1). Magnetic resonance imaging (MRI) of the brain showed a jugular foramen mass with enhancement of approximately 2.6 1.9 3.3 cm (Fig. 2), whereas magnetic resonance venography (MRV) showed occlusion of the superior sagittal sinus, the right transverse sinus, and the right sigmoid sinus (Fig. 3). Subtotal tumor resection was performed via a far lateral approach. Intraoperatively, the tumor was found to originate from the jugular vein, which was occluded because of tumor compression. Histopathologic examination of the tumor confirmed the diagnosis of neurinoma (Fig. 4). Postoperatively, the venous sinus thrombosis was unresponsive to anticoagulant therapy, and the patient died 4 days later. Jugular foramen tumors rarely induce venous sinus thrombosis. To date, only 3 cases of jugular foramen tumors associated with venous sinus thrombosis are available in Pubmed searches,2–4 all with chronic onset. Their pathologic type involved glomus jugulare tumor, schwannoma, and the Ewing tumor metastasis. To the authors' knowledge, this is the first report on acute venous sinus thrombosis after the development of a jugular foramen neurinoma. The presence of a jugular foramen tumor obstructing venous outflow of the dominant sinus is the mechanism underlying cerebral venous sinus thrombosis, thus leading to increased intracranial pressure, cerebral infarction, or hemorrhage. In our case, we presumed that the neurinoma originating from the dominant jugular vein and rapid growth may be responsible for the acute venous sinus thrombosis and hemorrhagic infarction. Because glomus tumors arise from the jugular bulb, they may have a higher rate of
FIGURE 3. The magnetic resonance venograms of the patient. The cranial magnetic resonance venograms show occlusion of the right transverse sinus (A, C), the superior sagittal sinus (B), and the right sigmoid sinus (C).
Brief Clinical Studies
FIGURE 4. The histologic examination of the tumor. The pathologic picture shows the elongated spindle cells arranged in a palisading pattern and compact in nature (hematoxylin-eosin staining, original magnification 100).
jugulosigmoid complex occlusion.5 Surgery in the jugular foramen can lead to inadequate cerebrospinal fluid drainage,4 and sacrifice of dominant outflow risks for intracerebral venous infarction.5 Overcoagulation in this area during operation may also enhance the possibility of venous sinus thrombosis.6 Therefore, it is possible that the reported cases of postsurgical benign intracranial hypertension may, in fact, have represented undiagnosed, coexisting cerebral venous sinus thrombosis. Examinations of MRI and MRV can be used to detect intracranial tumors and venous sinus thrombosis, respectively, both of which ought to be considered as important diagnostic adjuncts. Treatment options for jugular foramen tumors include surgical excision and radiotherapy.4,7 Endovascular stents can be deployed to relieve the venous sinus stenosis and restore venous outflow.1,8 Cerebral hemorrhage and malignancy are important prognostic indicators of poor survival9; however, in our case, the acute onset with hemorrhagic infarction had a poor outcome. In conclusion, the unique presentation of a jugular foramen neurinoma in a 45-year-old man leading to hemorrhagic infarction highlights the need for investigation of potential etiologies for accurate diagnosis. Jugular foramen tumors may predispose patients to cerebral venous sinus thrombosis; therefore, particular attention must be paid to early signs and symptoms of raised intracranial pressure. Examinations of MRI and MRV are important techniques for identifying the cause of the disease and for diagnosing this potentially life-threatening condition. However, the effective treatment of this acute condition requires further research.
REFERENCES 1. Wilson M, Browne JD, Martin T, et al. Case report: atypical presentation of jugular foramen mass. Am J Otolaryngol 2012;33:370–374 2. Graus F, Slatkin NE. Papilledema in the metastatic jugular foramen syndrome. Arch Neurol 1983;40:816–818 3. Yamakami I, Ono J, Yamaura A. Sigmoid sinus dural arteriovenous malformation resulting from jugular foramen schwannoma—case report. Neurol Med Chir 1998;38:43–46 4. Izadi S, Karkos PD, Krishnan R, et al. Papilloedema secondary to venous sinus thrombosis following glomus jugulare tumour surgery. J Laryngol Otol 2009;123:1393–1395 5. Lustig LR, Jackler RK. The variable relationship between the lower cranial nerves and jugular foramen tumors: implications for neural preservation. Am J Otol 1996;17:658–668 6. Hoshi M, Yoshida K, Ogawa K, et al. Hypoglossal neurinoma—two case reports. Neurol Med Chir 2000;40:489–493 7. Samii M, Babu RP, Tatagiba M, et al. Surgical treatment of jugular foramen schwannomas. J Neurosurg 1995;82:924–932 8. Zilani G, Pereira EA, Baig F, et al. Venoplasty and stenting of a jugular foramen meningioma. Br J Neurosurg 2009;23:557–560 9. Ferro JM, Canhao P, Stam J, et al. Prognosis of cerebral vein and dural sinus thrombosis: results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Stroke 2004;35:664–670
© 2015 Mutaz B. Habal, MD
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
e109
Brief Clinical Studies
FIGURE 2. After 2 weeks of applying topical nystatin paste and peroral 200 mg/d itraconazole, areas of plaque regressed and disappeared.
FIGURE 3. After 1 year, the manifestation of the oral lesions seems to be similar to that of his first admission.
syndrome have a higher risk of systemic lupus erythematosus and other skin-related autoimmune diseases than do those without this syndrome. Humoral and cellular immunity are enhanced in patients with Klinefelter syndrome.8 But the oral lesions have rarely been reported in Klinefelter syndrome to our knowledge. Only 1 case report9 mentioned that a patient with Klinefelter syndrome had oral thrush. However, no particular oral clinical history or examination had been mentioned in the case. That patient also had multiple abnormal manifestations, such as anemia and defect of humoral immunity. The relationship between Klinefelter syndrome and oral thrush requires further investigation. On the basis of clinical diagnosis and the result of antifungal susceptibility testing, topical nystatin paste and peroral 200 mg/d itraconazole were performed. At review after 2 weeks, areas of plaque regressed and disappeared (Fig. 2). During subsequent visits, he performed recurrent symptoms of oral thrush for about 1 year. It seems that this kind of oral thrush is inveterate and persistent (Fig. 3). We do not have more effective therapeutic measures to restrain this stubborn disease because the real etiology is not clear yet.
DISCUSSION Klinefelter syndrome is a genetic nonhereditary disorder with genotype 47 XXY. Dermatologically, patients with Klinefelter syndrome have a higher risk of systemic lupus erythematosus and other skin-related autoimmune diseases than do those without this syndrome.8 Only 1 case report9 described that a patient with Klinefelter syndrome had oral thrush. However, no detailed message had been mentioned. Moreover, multiple abnormal manifestations were also noticed in that case, such as anemia and defect of humoral immunity, which is distinct from our case. In summary, we report a patient with pseudomembranous stomatitis with a persistent course and unknown cause. The relationship between Klinefelter syndrome and oral thrush requires further investigations.
REFERENCES 1. Williams DW, Kuriyama T, Silva S, et al. Candida biofilms and oral candidosis: treatment and prevention. Periodontol 2000 2011; 55:250–265 2. Kirkpatrick WR, Revankar SG, McAtee RK, et al. Detection of Candida dubliniensis in oropharyngeal samples from human immunodeficiency virus-infected patients in North America by primary CHROMagar Candida screening and susceptibility testing of isolates. J Clin Microbiol 1998;36:3007–3012
e108
3. Sullivan D, Coleman D. Candida dubliniensis: characteristics and identification. J Clin Microbiol 1998;36:329–334 4. Espinel-Ingroff A, Canton E, Gibbs D, et al. Correlation of Neo-Sensitabs tablet diffusion assay results on three different agar media with CLSI broth microdilution M27-A2 and disk diffusion M44-A results for testing susceptibilities of Candida spp. and Cryptococcus neoformans to amphotericin B, caspofungin, fluconazole, itraconazole, and voriconazole. J Clin Microbiol 2007;45:858–864 5. Williams D, Lewis M. Pathogenesis and treatment of oral candidosis. J Oral Microbiol 2011;3:5771–5781 6. Rugg EL, McLean WH, Allison WE, et al. A mutation in the mucosal keratin K4 is associated with oral white sponge nevus. Nat Genet 1995;11:450–452 7. Shah DN, Yau R, Weston J, et al. Evaluation of antifungal therapy in patients with candidaemia based on susceptibility testing results: implications for antimicrobial stewardship programmes. J Antimicrob Chemother 2011;66:2146–2151 8. Jimenez-Balderas FJ, Tapia-Serrano R, Fonseca ME, et al. High frequency of association of rheumatic/autoimmune diseases and untreated male hypogonadism with severe testicular dysfunction. Arthritis Res 2001;3:362–367 9. de Morentin HM, Dodiuk-Gad RP, Brenner S. Klinefelter's syndrome presenting with leg ulcers. Skinmed 2004;3:274–278
Acute Venous Sinus Thrombosis Induced by a Jugular Foramen Neurinoma Quanmin Nie, PhD, Pin Guo, PhD, Liemei Guo, PhD, Xiaoxiong Li, MD, Jianwei Ge, PhD, Yongming Qiu, MD, PhD
J
ugular foramen lesions are often associated with pathology of adjacent structures owing to either compression or direct invasion. The most common symptom of a jugular foramen tumor is involved in cranial nerve (mainly IX, X, and XI) damage. However, symptoms associated with the jugular vein at the first onset are rare,1 which we presumed is caused by a lower incidence of tumors originating from the jugular vein, chronic occlusion of the jugular vein with no specific symptoms, or slight clinical symptoms that were unnoticed by the patient. Up to date, there has been no previous report on acute cerebral venous sinus thrombosis induced by a jugular foramen tumor. A 45-year-old man was hospitalized, with complaints of unbearable headaches and vomiting for 1 hour after jogging for practice. There was no relevant medical history to note. On initial examination, ipsilateral pharyngeal reflex disappearance and papilledema were found. The results of routine blood examinations were normal, with a normal coagulation profile. Cranial computed tomography From the Department of Neurosurgery, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China. Received July 27, 2014. Accepted for publication September 13, 2014. Address correspondence and reprint requests to Dr. Yongming Qiu, Department of Neurosurgery, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, No.1630, Dongfang Rd, Shanghai 200127, China; E-mail: [email protected] The authors report no conflicts of interest. Copyright © 2015 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001332
© 2015 Mutaz B. Habal, MD
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery • Volume 26, Number 2, March 2015
FIGURE 1. The CT scans of the patient. The cranial CT scans show multiple hemorrhages with massive edema (A) and a jugular foramen mass (B) with bone destruction (C).
FIGURE 2. The magnetic resonance images of the patient. The cranial magnetic resonance images show that the jugular foramen mass was isointense on T1-weighted images (A) and hyperintense on T2-weighted images (B). The neoplasm demonstrated moderate enhancement after contrast administration (C), and its size was approximately 2.6 1.9 3.3 cm.
(CT) showed multiple hemorrhages with massive edema and a jugular foramen mass with bone destruction (Fig. 1). Magnetic resonance imaging (MRI) of the brain showed a jugular foramen mass with enhancement of approximately 2.6 1.9 3.3 cm (Fig. 2), whereas magnetic resonance venography (MRV) showed occlusion of the superior sagittal sinus, the right transverse sinus, and the right sigmoid sinus (Fig. 3). Subtotal tumor resection was performed via a far lateral approach. Intraoperatively, the tumor was found to originate from the jugular vein, which was occluded because of tumor compression. Histopathologic examination of the tumor confirmed the diagnosis of neurinoma (Fig. 4). Postoperatively, the venous sinus thrombosis was unresponsive to anticoagulant therapy, and the patient died 4 days later. Jugular foramen tumors rarely induce venous sinus thrombosis. To date, only 3 cases of jugular foramen tumors associated with venous sinus thrombosis are available in Pubmed searches,2–4 all with chronic onset. Their pathologic type involved glomus jugulare tumor, schwannoma, and the Ewing tumor metastasis. To the authors' knowledge, this is the first report on acute venous sinus thrombosis after the development of a jugular foramen neurinoma. The presence of a jugular foramen tumor obstructing venous outflow of the dominant sinus is the mechanism underlying cerebral venous sinus thrombosis, thus leading to increased intracranial pressure, cerebral infarction, or hemorrhage. In our case, we presumed that the neurinoma originating from the dominant jugular vein and rapid growth may be responsible for the acute venous sinus thrombosis and hemorrhagic infarction. Because glomus tumors arise from the jugular bulb, they may have a higher rate of
FIGURE 3. The magnetic resonance venograms of the patient. The cranial magnetic resonance venograms show occlusion of the right transverse sinus (A, C), the superior sagittal sinus (B), and the right sigmoid sinus (C).
Brief Clinical Studies
FIGURE 4. The histologic examination of the tumor. The pathologic picture shows the elongated spindle cells arranged in a palisading pattern and compact in nature (hematoxylin-eosin staining, original magnification 100).
jugulosigmoid complex occlusion.5 Surgery in the jugular foramen can lead to inadequate cerebrospinal fluid drainage,4 and sacrifice of dominant outflow risks for intracerebral venous infarction.5 Overcoagulation in this area during operation may also enhance the possibility of venous sinus thrombosis.6 Therefore, it is possible that the reported cases of postsurgical benign intracranial hypertension may, in fact, have represented undiagnosed, coexisting cerebral venous sinus thrombosis. Examinations of MRI and MRV can be used to detect intracranial tumors and venous sinus thrombosis, respectively, both of which ought to be considered as important diagnostic adjuncts. Treatment options for jugular foramen tumors include surgical excision and radiotherapy.4,7 Endovascular stents can be deployed to relieve the venous sinus stenosis and restore venous outflow.1,8 Cerebral hemorrhage and malignancy are important prognostic indicators of poor survival9; however, in our case, the acute onset with hemorrhagic infarction had a poor outcome. In conclusion, the unique presentation of a jugular foramen neurinoma in a 45-year-old man leading to hemorrhagic infarction highlights the need for investigation of potential etiologies for accurate diagnosis. Jugular foramen tumors may predispose patients to cerebral venous sinus thrombosis; therefore, particular attention must be paid to early signs and symptoms of raised intracranial pressure. Examinations of MRI and MRV are important techniques for identifying the cause of the disease and for diagnosing this potentially life-threatening condition. However, the effective treatment of this acute condition requires further research.
REFERENCES 1. Wilson M, Browne JD, Martin T, et al. Case report: atypical presentation of jugular foramen mass. Am J Otolaryngol 2012;33:370–374 2. Graus F, Slatkin NE. Papilledema in the metastatic jugular foramen syndrome. Arch Neurol 1983;40:816–818 3. Yamakami I, Ono J, Yamaura A. Sigmoid sinus dural arteriovenous malformation resulting from jugular foramen schwannoma—case report. Neurol Med Chir 1998;38:43–46 4. Izadi S, Karkos PD, Krishnan R, et al. Papilloedema secondary to venous sinus thrombosis following glomus jugulare tumour surgery. J Laryngol Otol 2009;123:1393–1395 5. Lustig LR, Jackler RK. The variable relationship between the lower cranial nerves and jugular foramen tumors: implications for neural preservation. Am J Otol 1996;17:658–668 6. Hoshi M, Yoshida K, Ogawa K, et al. Hypoglossal neurinoma—two case reports. Neurol Med Chir 2000;40:489–493 7. Samii M, Babu RP, Tatagiba M, et al. Surgical treatment of jugular foramen schwannomas. J Neurosurg 1995;82:924–932 8. Zilani G, Pereira EA, Baig F, et al. Venoplasty and stenting of a jugular foramen meningioma. Br J Neurosurg 2009;23:557–560 9. Ferro JM, Canhao P, Stam J, et al. Prognosis of cerebral vein and dural sinus thrombosis: results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Stroke 2004;35:664–670
© 2015 Mutaz B. Habal, MD
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
e109
