The Journal of Craniofacial Surgery • Volume 25, Number 6, November 2014
Delayed Cerebrospinal Fluid Rhinorrhea Four Years After Gamma Knife Surgery for Juvenile Angiofibroma Hyun Jin Min, MD, PhD,* Hyo Jin Chung, MD,* Chang-Hoon Kim, MD, PhD*† Abstract: Juvenile angiofibroma (JA) is a highly vascularized tumor that often recurs or regrows. Recently, gamma knife surgery (GKS) was attempted on unresectable or remnant JA. We experienced a JA case that developed cerebrospinal fluid (CSF) rhinorrhea 4 years after GKS. Two surgical excisions using midfacial degloving approach were performed before GKS. After radiosurgery, the tumor was controlled, and no early complications were observed. However, 4 years after, intractable CSF leakage developed as a late complication, and we thus performed 4 subsequent endoscopic surgical repairs. The CSF leakage is very rare but can occur as a late complication of GKS and should be treated aggressively. Key Words: Juvenile angiofibroma, CSF leakage, endoscopic repair, topical tissue adhesives
J
uvenile angiofibroma (JA) is a rare benign vascular tumor with a prevalence of approximately 0.05% of all head and neck tumors.1 The JA is showing highly vascularized nature, mostly found in the nasopharynx and pterygopalatine fossa of male adolescents be2 tween 9 and 19 years old. Patients typically complain unilateral nasal obstruction, rhinorrhea, and frequent unilateral epistaxis, but also cheek swelling, proptosis, headache, anosmia, and acute sinusitis can appear less often.2–4 This tumor initially arises from the nasopharynx, expands intranasally, and then progresses into the pterygomaxillary space slowly. Unlike its slowly growing pattern, it would eventually erode the surrounding bones and invade the infratemporal fossa, middle cranial fossa,0 or orbit.3 Surgical removal is the treatment of choice in most patients, and because of its extremely vascular characteristic, preoperative embolization of feeding vessels is highly recommended as a standard procedure by majorities.2 According to staging system4 (Table 1), stages I and II are resected via endoscopic approach or midfacial degloving approach (MFDA) nowadays, taking advantage of no external scars.3 Stages IIIA and IIIB, which means extensive invasions, need resection via open approach; stage IIIA can be removed by combined transpalatal and transmaxillary approach, and stage IIIB can From the *Department of Otorhinolaryngology, Severance Hospital, Yonsei University College of Medicine; and †Research Center for Human Natural Defense System, Yonsei University College of Medicine, Seoul, Korea. Received April 24, 2014. Accepted for publication June 19, 2014. Address correspondence and reprint requests to Dr. Chang-Hoon Kim, Department of Otorhinolaryngology, Severance Hospital, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemoon-gu, Seoul 120-752, South Korea; E-mail: [email protected] Hyun Jin Min and Hyo Jin Chung contributed equally and are co-first authors. The authors report no conflicts of interest. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001164
Brief Clinical Studies
be removed by lateral infratemporal approach, which is mainly conservative.5 Nonsurgical treatments also have been used in unresectable or recurrent tumors and in failure of complete tumor removal.2 Radiation therapy is still debated for many possible complications such as sarcomatoid change or growth retardation.6,7 Chemotherapy is not commonly used; only few reports mentioned about reasonable results.2 Recently, stereotactic gamma knife surgery (GKS) was reported to be used for the adjuvant treatment for JA patients, because of its insignificant morbidity and optimized radiation treatment of the tumor.7,8 The GKS is mainly used in residual tumor after incomplete surgical resection, which is located in the infratemporal fossa and cavernous sinus.8,9 This modality is believed to be potentially safe and effective,9 but not much late complications as radiotherapy have been reported yet.7 Here, we report a case of delayed-onset CSF rhinorrhea developed after GKS, which needed surgical repairs in a patient with recurrent JA.
CLINICAL REPORT A 21-year-old male patient visited the outpatient clinic for persistent watery rhinorrhea. Previously, he was diagnosed with JA and had 2 surgeries using MFDA. After these 2 surgical excisions, GKS was chosen as the treatment of the regrown angiofibroma. The primary tumor was located in the nasopharynx and choana with partial involvement of the right pterygopalatine fossa (Fig. 1A). Preoperative angioembolization was performed on both branches of the internal maxillary artery (IMA) (Fig.1B), and we attempted a first tumor excision via MFDA. Six months after the initial operation, the tumor was shown to have regrown on magnetic resonance imaging, and it was approximately 4 cm, invading the base of the skull (Fig. 2A). Again, preoperative angioembolization was performed on the proximal and distal branches of the right IMA (Fig. 2B), and we reexcised the tumor via MFDA. The GKS was planned for the treatment of the unresectable tumor area, with a maximal dose of 40 Gy. Immediately after GKS, there was no evidence of complications or morbidities. Since then, we have regularly assessed the endoscopic findings. In addition, computed tomography and magnetic resonance imaging scans were obtained regularly to check for evidence of a recurrent tumor, and the patient has had a no-evidence-of-disease status for 4 years (Fig. 2C). Four years after GKS, the patient visited the outpatient clinic with continuous watery rhinorrhea and a recent history of meningitis. He had no history of trauma, upper respiratory tract infection, or other surgeries. We evaluated the rhinorrhea and diagnosed it as CSF leakage. A defect was observed in the right anterior wall of the sphenoid sinus (Fig. 3A, left) and was endoscopically repaired using a mucosal flap harvested from the inferior turbinate under general anesthesia. We overlayed the harvested mucosal graft and sequentially applied Surgicel (Ethicon, Inc [a Johnson & Johnson company], Somerville, NJ), Tisseel (Baxter, Deerfield, IL), and Gelfoam (Baxter, Deerfield, IL). Lumbar drainage was performed, and third-generation cephalosporin antibiotics were used. The patient was confined to absolute bed rest. Fourteen days after surgery, when the patient stood up, we found recurrent CSF leakage in the same area. We again endoscopically repaired the leakage using a mucosal flap harvested from the inferior turbinate. Five days after the second repair, CSF leakage recurred (Fig. 3A, center). We endoscopically reinforced the leakage site with abdominal fat using the underlay method (Fig. 3A, right). However, 14 days after the third operation, CSF leakage was still noted (Fig. 3B, left). Finally, we resected the other side of the middle turbinate, harvested a large mucosal flap, and applied it to the leakage site. We additionally applied topical tissue adhesive, Histoacryl (B. Braun Aesculap AG & Co, Ann Arbor, MI) to attach the mucosal flap, and then series of
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
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The Journal of Craniofacial Surgery • Volume 25, Number 6, November 2014
Brief Clinical Studies
TABLE 1. Radkowski et al’s4 Staging System of JA Staging System by Radkowski et al I
A B
II
A B C
III
A B
Limited to posterior nares and/or nasopharyngeal vault Involving the posterior nares and/or nasopharyngeal vault with involvement of at least 1 paranasal sinus Minimal lateral extension into the pterygopalatine fossa Full occupation of pterygopalatine fossa with or without superior erosion orbital bones Extension into the infratemporal fossa or extension posterior to the pterygoid plates Erosion of skull base (middle cranial fossa/base of pterygoids), minimal intracranial extension Erosion of skull base, extensive with or without extension into the cavernous sinus
BioGlue (Cryolife, Atlanta, GA), Surgicel, and Gelfoam were applied (Fig. 3B, center). During these procedures, the lumbar region was fully drained so as to decrease intracranial pressure and dry the leakage site. For 2 months after the fourth operation, we did not find evidence of CSF leakage (Fig. 3B, right) or tumor recurrence in this patient.
DISCUSSION In this clinical report, we describe CSF leakage that occurred 4 years after GKS. Usual indications of GKS are brain pathologies, including intracranical vascular tumors, pituitary tumors, and vestibular schwannomas.10–12 In the nasal cavity, olfactory neuroblastomas and anterior skull base malignancies are treated with GKS.13,14 The JA is a highly vascularized tumor that primarily affects young men and easily extends into adjacent areas. Therefore, various treatment options have been suggested, such as external surgical approaches, endoscopic resection, external radiation, chemotherapy, and GKS.7,15–17 In our group, several unresectable angiofibromas have been controlled using GKS (data not shown). Therefore, we suggest that welldesigned GKS is a suitable option for inoperable or residual JA. Neurotoxicity can occur as an early complication of external radiation therapy; late complications such as radiation necrosis, radiation leukoencephalopathy, and radiation myopathy have been reported.18 However, late complications of GKS have not been reported much. Only 3 patients with delayed CSF rhinorrhea with recurrent meningitis were reported until now; durations from operation to CSF rhinorrhea ranged from 4 months19 to 11 years,20 and none of them were reported as JA. In our case, the JA was abutting the dura posteriorly, so there is a possibility that GKS targeting the JA might have damaged the dura of the anterior skull base and caused CSF leakage, an unusual complication. Ogawa and Tominaga21 reported a case with 10-year delayed CSF rhinorrhea after pituitary adenoma transsphenoidal removal and after GKS. They presumed that delayed CSF rhinorrhea might be due to damage of the mucosal membranes of the sphenoid sinus, arachnoid membrane, and dura. Hongmei et al20 also reported a
FIGURE 1. A, Magnetic resonance imaging scan. Angiofibroma is noted in the nasopharynx, choana, and right pterygopalatine fossa. B, Preoperative angiography. Tumor staining is noted in the distal branches of the bilateral IMA.
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FIGURE 2. A, Magnetic resonance imaging scan. A regrown angiofibroma is noted at the central skull base. B, Tumor staining is noted in the proximal and distal branches of the right IMA. C, CT scan. The tumor was not noted 4 years after GKS.
growth hormone-secreting pituitary adenoma case with 11-year delayed CSF rhinorrhea after GKS. In this case, authors presumed that empty sella might have been the cause of delayed CSF rhinorrhea. We believe that this is the first report of the occurrence of CSF rhinorrhea long after GKS in the JA case. Usually, JA patients previously treated with GKS have a disruption of the normal anatomy of the nasal cavity. In our case, the patient had 2 previous operations, and the normal anatomy of the nasal cavity and paranasal area were destroyed. Furthermore, bony structures around the sphenoid sinus were destroyed, and the dura was only consisted of a thin mucosal layer. Therefore, it was difficult to use the underlay technique with a mucosal flap and sustain the graft materials successfully. We think that the destruction of the normal anatomy of the nasal cavity and paranasal area and postirradiated mucosa were reasons for the failures in repairing the CSF leakage. It is noteworthy that we used Histoacryl and BioGlue on our fourth repair operation. Histoacryl is suitable for the repair of CSF leakage, because it is strong, works fast, has no toxicity, and has an antibacterial effect.22 BioGlue has also been used for the prevention of CSF leakage after transsphenoidal sellar surgery.23 This is our first trial using Histoacryl and BioGlue with a conventional nasal mucosal flap for CSF leakage repair. In summary, we experienced delayed-onset CSF leakage as a complication of GKS in a JA patient. In patients undergoing GKS for the treatment of unresectable angiofibroma, delayed complications such as CSF leakage could be observed, especially if the
FIGURE 3. Operative findings. A, A leakage of CSF was noted on the anterior wall of the right sphenoid sinus 4 years after GKS (left). A mucosal flap was applied to seal the leakage during a second surgery (center). Abdominal fat was harvested and applied to repair the leakage area during the third surgery (right). B, The leakage of CSF was still noted after 3 endoscopic repairs (left). A mucosal flap, Histoacryl, and BioGlue were sequentially applied to repair the leakage site (center). After the fourth endoscopic repair, no additional CSF leakage was noted (right).
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery • Volume 25, Number 6, November 2014
angiofibroma is located close to the dura. In such cases, aggressive treatment options should be evaluated, and the use of new biomaterials could be a treatment of choice.
Brief Clinical Studies
23. Cho JM, Ahn JY, Chang JH, et al. Prevention of cerebrospinal fluid rhinorrhea after transsphenoidal surgery by collagen fleece coated with fibrin sealant without autologous tissue graft or postoperative lumbar drainage. Neurosurgery 2011;68(1):130–136
REFERENCES 1. Lund VJ, Stammberger H, Nicolai P, et al. European position paper on endoscopic management of tumours of the nose, paranasal sinuses and skull base. Rhinol Suppl 2010;1–143 2. Nicolai P, Schreiber A, Bolzoni Villaret A. Juvenile angiofibroma: evolution of management. Int J Pediatr 2012;2012:412545 3. Enepekides DJ. Recent advances in the treatment of juvenile angiofibroma. Curr Opin Otolaryngol Head Neck Surg 2004;12:495–499 4. Radkowski D, McGill T, Healy GB, et al. Angiofibroma: changes in staging and treatment. Arch Otolaryngol Head Neck Surg 1996;122:122–129 5. Tyagi I, Syal R, Goyal A. Staging and surgical approaches in large juvenile angiofibroma—study of 95 cases. Int J Pediatr Otorhinolaryngol 2006;70:1619–1627 6. Makek MS, Andrews JC, Fisch U. Malignant transformation of a nasopharyngeal angiofibroma. Laryngoscope 1989;99(10 pt 1):1088–1092 7. Park CK, Kim DG, Paek SH, et al. Recurrent juvenile nasopharyngeal angiofibroma treated with gamma knife surgery. J Korean Med Sci 2006;21:773–777 8. Dare AO, Gibbons KJ, Proulx GM, et al. Resection followed by radiosurgery for advanced juvenile nasopharyngeal angiofibroma: report of two cases. Neurosurgery 2003;52:1207–1211 9. Alvarez FL, Suarez V, Suarez C, et al. Multimodality approach for advanced-stage juvenile nasopharyngeal angiofibromas. Head Neck 2013;35:209–213 10. Zeiler FA, Bigder M, Kaufmann A, et al. Gamma knife in the treatment of pituitary adenomas: results of a single center. Can J Neurol Sci 2013;40:546–552 11. Zeiler FA, Bigder M, Kaufmann A, et al. Gamma knife radiosurgery for large vestibular schwannomas: a canadian experience. Can J Neurol Sci 2013;40:342–347 12. Nanney AD III, El Tecle NE, El Ahmadieh TY, et al. Intracranial aneurysms in previously irradiated fields: literature review and case illustration. World Neurosurg 2013 13. Papacharalampous GX, Vlastarakos PV, Chrysovergis A, et al. Olfactory neuroblastoma (esthesioneuroblastoma): towards minimally invasive surgery and multi-modality treatment strategies—an updated critical review of the current literature. J BUON 2013;18:557–563 14. Clarke MJ, Foy AB, Garces YI, et al. Prone positioning for successful gamma knife radiosurgical treatment of far anterior skull base lesions: a technical note. J Neurol Surg 2012;73:243–248 15. Carrau RL, Snyderman CH, Kassam AB, et al. Endoscopic and endoscopic-assisted surgery for juvenile angiofibroma. Laryngoscope 2001;111:483–487 16. Goepfert H, Cangir A, Ayala AG, et al. Chemotherapy of locally aggressive head and neck tumors in the pediatric age group. Desmoid fibromatosis and nasopharyngeal angiofibroma. Am J Surg 1982;144:437–444 17. Gates GA, Rice DH, Koopmann CF Jr, et al. Flutamide-induced regression of angiofibroma. Laryngoscope 1992;102:641–644 18. Rahmathulla G, Marko NF, Weil RJ. Cerebral radiation necrosis: a review of the pathobiology, diagnosis and management considerations. J Clin Neurosci 2013;20:485–502 19. Kim CH, Chung SK, Dhong HJ, et al. Cerebrospinal fluid leakage after gamma knife radiosurgery for skull base metastasis from renal cell carcinoma: a case report. Laryngoscope 2008;118:1925–1927 20. Hongmei Y, Zhe W, Jing W, et al. Delayed cerebrospinal fluid rhinorrhea after gamma knife surgery in a patient with a growth hormone-secreting adenoma. J Clin Neurosci 2012;19:900–902 21. Ogawa Y, Tominaga T. Delayed cerebrospinal fluid leakage 10 years after transsphenoidal surgery and gamma knife surgery—case report. Neurol Med Chir 2007;47:483–485 22. Eiferman RA, Snyder JW. Antibacterial effect of cyanoacrylate glue. Arch Ophthalmol 1983;101:958–960
Primary Malignant Mucosal Melanoma of the Nasopharynx: An Unusual Cause of Unilateral Hearing Loss Tümay Bekci, MD, Kerim Aslan, MD, Hediye Pinar Günbey, MD, Lütfi Incesu, MD Abstract: There are many causes of unilateral hearing loss, and making the correct differential diagnosis, especially in elderly patients, is difficult. A primary malignant mucosal melanoma of the nasopharynx is extremely rare and can cause a variety of symptoms. Hearing loss, as a presenting symptom of mucosal malignant melanoma of the nasopharynx, has not yet been defined in the literature. Herein, we report a case of primary mucosal malignant melanoma of the nasopharynx presented with unilateral hearing loss in a 70-year-old man. Key Words: Hearing loss, malignant mucosal melanoma, MRI
M
any causes of unilateral hearing loss have been described in the literature, such as noise, illness, chemicals, and physical trauma.1 Nasopharyngeal tumors are a rare but are a significant cause of unilateral hearing loss, with related Eustachian tube obstruction and otitis media with effusion.2 Melanomas usually occur in sunlight-exposed cutaneous areas, such as the neck, extremities, and face.3 Mucosal melanomas arising from the nasopharynx are extremely rare, with an incidence of 0.6% of all mucosal melanomas occurring in the head and neck.4 Sinonasal melanomas have nonspecific symptoms, such as nasal obstruction, epistaxis, and headache, so diagnosis is often delayed.5 In addition, making a differential diagnosis, especially in elderly patients, is difficult. Hearing loss, as a first presenting symptom of nasopharyngeal mucosal melanoma, has not yet been defined in the literature. Mucosal melanomas have more aggressive behavior than cutaneous lesions, and because of the rarity, aggressiveness, and nonspecific symptoms, the mean 5-year survival rate is only approximately 10%.5 This highlights the importance of early diagnosis and timely treatment. Here, we report a unique presentation of nasopharyngeal mucosal malignant melanoma and its imaging and clinical findings in a 70-year-old man. From the Faculty of Medicine, Department of Radiology, Ondokuz Mayis University, Samsun, Turkey. Received April 24, 2014. Accepted for publication June 19, 2014. Address correspondence and reprint requests to Tumay Bekci, MD, Faculty of Medicine, Department of Radiology, Ondokuz Mayis University, 55139 Kurupelit, Samsun, Turkey; E-mail: [email protected] The authors report no conflicts of interest. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001165
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
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Delayed Cerebrospinal Fluid Rhinorrhea Four Years After Gamma Knife Surgery for Juvenile Angiofibroma Hyun Jin Min, MD, PhD,* Hyo Jin Chung, MD,* Chang-Hoon Kim, MD, PhD*† Abstract: Juvenile angiofibroma (JA) is a highly vascularized tumor that often recurs or regrows. Recently, gamma knife surgery (GKS) was attempted on unresectable or remnant JA. We experienced a JA case that developed cerebrospinal fluid (CSF) rhinorrhea 4 years after GKS. Two surgical excisions using midfacial degloving approach were performed before GKS. After radiosurgery, the tumor was controlled, and no early complications were observed. However, 4 years after, intractable CSF leakage developed as a late complication, and we thus performed 4 subsequent endoscopic surgical repairs. The CSF leakage is very rare but can occur as a late complication of GKS and should be treated aggressively. Key Words: Juvenile angiofibroma, CSF leakage, endoscopic repair, topical tissue adhesives
J
uvenile angiofibroma (JA) is a rare benign vascular tumor with a prevalence of approximately 0.05% of all head and neck tumors.1 The JA is showing highly vascularized nature, mostly found in the nasopharynx and pterygopalatine fossa of male adolescents be2 tween 9 and 19 years old. Patients typically complain unilateral nasal obstruction, rhinorrhea, and frequent unilateral epistaxis, but also cheek swelling, proptosis, headache, anosmia, and acute sinusitis can appear less often.2–4 This tumor initially arises from the nasopharynx, expands intranasally, and then progresses into the pterygomaxillary space slowly. Unlike its slowly growing pattern, it would eventually erode the surrounding bones and invade the infratemporal fossa, middle cranial fossa,0 or orbit.3 Surgical removal is the treatment of choice in most patients, and because of its extremely vascular characteristic, preoperative embolization of feeding vessels is highly recommended as a standard procedure by majorities.2 According to staging system4 (Table 1), stages I and II are resected via endoscopic approach or midfacial degloving approach (MFDA) nowadays, taking advantage of no external scars.3 Stages IIIA and IIIB, which means extensive invasions, need resection via open approach; stage IIIA can be removed by combined transpalatal and transmaxillary approach, and stage IIIB can From the *Department of Otorhinolaryngology, Severance Hospital, Yonsei University College of Medicine; and †Research Center for Human Natural Defense System, Yonsei University College of Medicine, Seoul, Korea. Received April 24, 2014. Accepted for publication June 19, 2014. Address correspondence and reprint requests to Dr. Chang-Hoon Kim, Department of Otorhinolaryngology, Severance Hospital, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemoon-gu, Seoul 120-752, South Korea; E-mail: [email protected] Hyun Jin Min and Hyo Jin Chung contributed equally and are co-first authors. The authors report no conflicts of interest. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001164
Brief Clinical Studies
be removed by lateral infratemporal approach, which is mainly conservative.5 Nonsurgical treatments also have been used in unresectable or recurrent tumors and in failure of complete tumor removal.2 Radiation therapy is still debated for many possible complications such as sarcomatoid change or growth retardation.6,7 Chemotherapy is not commonly used; only few reports mentioned about reasonable results.2 Recently, stereotactic gamma knife surgery (GKS) was reported to be used for the adjuvant treatment for JA patients, because of its insignificant morbidity and optimized radiation treatment of the tumor.7,8 The GKS is mainly used in residual tumor after incomplete surgical resection, which is located in the infratemporal fossa and cavernous sinus.8,9 This modality is believed to be potentially safe and effective,9 but not much late complications as radiotherapy have been reported yet.7 Here, we report a case of delayed-onset CSF rhinorrhea developed after GKS, which needed surgical repairs in a patient with recurrent JA.
CLINICAL REPORT A 21-year-old male patient visited the outpatient clinic for persistent watery rhinorrhea. Previously, he was diagnosed with JA and had 2 surgeries using MFDA. After these 2 surgical excisions, GKS was chosen as the treatment of the regrown angiofibroma. The primary tumor was located in the nasopharynx and choana with partial involvement of the right pterygopalatine fossa (Fig. 1A). Preoperative angioembolization was performed on both branches of the internal maxillary artery (IMA) (Fig.1B), and we attempted a first tumor excision via MFDA. Six months after the initial operation, the tumor was shown to have regrown on magnetic resonance imaging, and it was approximately 4 cm, invading the base of the skull (Fig. 2A). Again, preoperative angioembolization was performed on the proximal and distal branches of the right IMA (Fig. 2B), and we reexcised the tumor via MFDA. The GKS was planned for the treatment of the unresectable tumor area, with a maximal dose of 40 Gy. Immediately after GKS, there was no evidence of complications or morbidities. Since then, we have regularly assessed the endoscopic findings. In addition, computed tomography and magnetic resonance imaging scans were obtained regularly to check for evidence of a recurrent tumor, and the patient has had a no-evidence-of-disease status for 4 years (Fig. 2C). Four years after GKS, the patient visited the outpatient clinic with continuous watery rhinorrhea and a recent history of meningitis. He had no history of trauma, upper respiratory tract infection, or other surgeries. We evaluated the rhinorrhea and diagnosed it as CSF leakage. A defect was observed in the right anterior wall of the sphenoid sinus (Fig. 3A, left) and was endoscopically repaired using a mucosal flap harvested from the inferior turbinate under general anesthesia. We overlayed the harvested mucosal graft and sequentially applied Surgicel (Ethicon, Inc [a Johnson & Johnson company], Somerville, NJ), Tisseel (Baxter, Deerfield, IL), and Gelfoam (Baxter, Deerfield, IL). Lumbar drainage was performed, and third-generation cephalosporin antibiotics were used. The patient was confined to absolute bed rest. Fourteen days after surgery, when the patient stood up, we found recurrent CSF leakage in the same area. We again endoscopically repaired the leakage using a mucosal flap harvested from the inferior turbinate. Five days after the second repair, CSF leakage recurred (Fig. 3A, center). We endoscopically reinforced the leakage site with abdominal fat using the underlay method (Fig. 3A, right). However, 14 days after the third operation, CSF leakage was still noted (Fig. 3B, left). Finally, we resected the other side of the middle turbinate, harvested a large mucosal flap, and applied it to the leakage site. We additionally applied topical tissue adhesive, Histoacryl (B. Braun Aesculap AG & Co, Ann Arbor, MI) to attach the mucosal flap, and then series of
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
e565
The Journal of Craniofacial Surgery • Volume 25, Number 6, November 2014
Brief Clinical Studies
TABLE 1. Radkowski et al’s4 Staging System of JA Staging System by Radkowski et al I
A B
II
A B C
III
A B
Limited to posterior nares and/or nasopharyngeal vault Involving the posterior nares and/or nasopharyngeal vault with involvement of at least 1 paranasal sinus Minimal lateral extension into the pterygopalatine fossa Full occupation of pterygopalatine fossa with or without superior erosion orbital bones Extension into the infratemporal fossa or extension posterior to the pterygoid plates Erosion of skull base (middle cranial fossa/base of pterygoids), minimal intracranial extension Erosion of skull base, extensive with or without extension into the cavernous sinus
BioGlue (Cryolife, Atlanta, GA), Surgicel, and Gelfoam were applied (Fig. 3B, center). During these procedures, the lumbar region was fully drained so as to decrease intracranial pressure and dry the leakage site. For 2 months after the fourth operation, we did not find evidence of CSF leakage (Fig. 3B, right) or tumor recurrence in this patient.
DISCUSSION In this clinical report, we describe CSF leakage that occurred 4 years after GKS. Usual indications of GKS are brain pathologies, including intracranical vascular tumors, pituitary tumors, and vestibular schwannomas.10–12 In the nasal cavity, olfactory neuroblastomas and anterior skull base malignancies are treated with GKS.13,14 The JA is a highly vascularized tumor that primarily affects young men and easily extends into adjacent areas. Therefore, various treatment options have been suggested, such as external surgical approaches, endoscopic resection, external radiation, chemotherapy, and GKS.7,15–17 In our group, several unresectable angiofibromas have been controlled using GKS (data not shown). Therefore, we suggest that welldesigned GKS is a suitable option for inoperable or residual JA. Neurotoxicity can occur as an early complication of external radiation therapy; late complications such as radiation necrosis, radiation leukoencephalopathy, and radiation myopathy have been reported.18 However, late complications of GKS have not been reported much. Only 3 patients with delayed CSF rhinorrhea with recurrent meningitis were reported until now; durations from operation to CSF rhinorrhea ranged from 4 months19 to 11 years,20 and none of them were reported as JA. In our case, the JA was abutting the dura posteriorly, so there is a possibility that GKS targeting the JA might have damaged the dura of the anterior skull base and caused CSF leakage, an unusual complication. Ogawa and Tominaga21 reported a case with 10-year delayed CSF rhinorrhea after pituitary adenoma transsphenoidal removal and after GKS. They presumed that delayed CSF rhinorrhea might be due to damage of the mucosal membranes of the sphenoid sinus, arachnoid membrane, and dura. Hongmei et al20 also reported a
FIGURE 1. A, Magnetic resonance imaging scan. Angiofibroma is noted in the nasopharynx, choana, and right pterygopalatine fossa. B, Preoperative angiography. Tumor staining is noted in the distal branches of the bilateral IMA.
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FIGURE 2. A, Magnetic resonance imaging scan. A regrown angiofibroma is noted at the central skull base. B, Tumor staining is noted in the proximal and distal branches of the right IMA. C, CT scan. The tumor was not noted 4 years after GKS.
growth hormone-secreting pituitary adenoma case with 11-year delayed CSF rhinorrhea after GKS. In this case, authors presumed that empty sella might have been the cause of delayed CSF rhinorrhea. We believe that this is the first report of the occurrence of CSF rhinorrhea long after GKS in the JA case. Usually, JA patients previously treated with GKS have a disruption of the normal anatomy of the nasal cavity. In our case, the patient had 2 previous operations, and the normal anatomy of the nasal cavity and paranasal area were destroyed. Furthermore, bony structures around the sphenoid sinus were destroyed, and the dura was only consisted of a thin mucosal layer. Therefore, it was difficult to use the underlay technique with a mucosal flap and sustain the graft materials successfully. We think that the destruction of the normal anatomy of the nasal cavity and paranasal area and postirradiated mucosa were reasons for the failures in repairing the CSF leakage. It is noteworthy that we used Histoacryl and BioGlue on our fourth repair operation. Histoacryl is suitable for the repair of CSF leakage, because it is strong, works fast, has no toxicity, and has an antibacterial effect.22 BioGlue has also been used for the prevention of CSF leakage after transsphenoidal sellar surgery.23 This is our first trial using Histoacryl and BioGlue with a conventional nasal mucosal flap for CSF leakage repair. In summary, we experienced delayed-onset CSF leakage as a complication of GKS in a JA patient. In patients undergoing GKS for the treatment of unresectable angiofibroma, delayed complications such as CSF leakage could be observed, especially if the
FIGURE 3. Operative findings. A, A leakage of CSF was noted on the anterior wall of the right sphenoid sinus 4 years after GKS (left). A mucosal flap was applied to seal the leakage during a second surgery (center). Abdominal fat was harvested and applied to repair the leakage area during the third surgery (right). B, The leakage of CSF was still noted after 3 endoscopic repairs (left). A mucosal flap, Histoacryl, and BioGlue were sequentially applied to repair the leakage site (center). After the fourth endoscopic repair, no additional CSF leakage was noted (right).
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery • Volume 25, Number 6, November 2014
angiofibroma is located close to the dura. In such cases, aggressive treatment options should be evaluated, and the use of new biomaterials could be a treatment of choice.
Brief Clinical Studies
23. Cho JM, Ahn JY, Chang JH, et al. Prevention of cerebrospinal fluid rhinorrhea after transsphenoidal surgery by collagen fleece coated with fibrin sealant without autologous tissue graft or postoperative lumbar drainage. Neurosurgery 2011;68(1):130–136
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Primary Malignant Mucosal Melanoma of the Nasopharynx: An Unusual Cause of Unilateral Hearing Loss Tümay Bekci, MD, Kerim Aslan, MD, Hediye Pinar Günbey, MD, Lütfi Incesu, MD Abstract: There are many causes of unilateral hearing loss, and making the correct differential diagnosis, especially in elderly patients, is difficult. A primary malignant mucosal melanoma of the nasopharynx is extremely rare and can cause a variety of symptoms. Hearing loss, as a presenting symptom of mucosal malignant melanoma of the nasopharynx, has not yet been defined in the literature. Herein, we report a case of primary mucosal malignant melanoma of the nasopharynx presented with unilateral hearing loss in a 70-year-old man. Key Words: Hearing loss, malignant mucosal melanoma, MRI
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any causes of unilateral hearing loss have been described in the literature, such as noise, illness, chemicals, and physical trauma.1 Nasopharyngeal tumors are a rare but are a significant cause of unilateral hearing loss, with related Eustachian tube obstruction and otitis media with effusion.2 Melanomas usually occur in sunlight-exposed cutaneous areas, such as the neck, extremities, and face.3 Mucosal melanomas arising from the nasopharynx are extremely rare, with an incidence of 0.6% of all mucosal melanomas occurring in the head and neck.4 Sinonasal melanomas have nonspecific symptoms, such as nasal obstruction, epistaxis, and headache, so diagnosis is often delayed.5 In addition, making a differential diagnosis, especially in elderly patients, is difficult. Hearing loss, as a first presenting symptom of nasopharyngeal mucosal melanoma, has not yet been defined in the literature. Mucosal melanomas have more aggressive behavior than cutaneous lesions, and because of the rarity, aggressiveness, and nonspecific symptoms, the mean 5-year survival rate is only approximately 10%.5 This highlights the importance of early diagnosis and timely treatment. Here, we report a unique presentation of nasopharyngeal mucosal malignant melanoma and its imaging and clinical findings in a 70-year-old man. From the Faculty of Medicine, Department of Radiology, Ondokuz Mayis University, Samsun, Turkey. Received April 24, 2014. Accepted for publication June 19, 2014. Address correspondence and reprint requests to Tumay Bekci, MD, Faculty of Medicine, Department of Radiology, Ondokuz Mayis University, 55139 Kurupelit, Samsun, Turkey; E-mail: [email protected] The authors report no conflicts of interest. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001165
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
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