Facial nerve hemangioma: a case report.
Although hemangiomas are relatively common in the head and neck, those that originate in the facial nerve are extremely rare. These benign tumors have the potential to compress or invade the adjacent facial nerve and thereby produce facial paralysis and other associated symptoms. We present a case of facial nerve hemangioma in a 15-year-old girl that initially manifested as unilateral facial weakness. We also discuss the diagnostic imaging and management options.
Facial nerve hemangioma, first described in 1901 by Adam Politzer, is a rare benign vascular tumor that accounts for approximately 0.7% of all temporal bone neoplasms. (1) The diagnosis is based on key findings on radiologic imaging, and the management options depend on the severity of its clinical manifestations.
We report a case of a facial nerve hemangioma in an adolescent girl who presented with only subtle and gradual facial paralysis.
A 15-year-old girl presented with a 2- to 3-year history of progressive right facial weakness and a 1-year history of increasing difficulty closing her right eye. Examination of her yearly school photographs starting at the age of 7 years revealed a subtle asymmetry of her smile. She reported no ocular complaints, hearing changes, tinnitus, or vertigo. Her medical, surgical, and family histories were otherwise negative.
Examination was significant for right-sided VIIth cranial nerve findings that included facial symmetry at rest, mild forehead weakness, incomplete eye closure, positive Bell phenomenon, moderate midface weakness with asymmetry of the nasolabial fold, mild weakness of the marginal mandibular nerve with a nearly symmetric smile, normal platysma movement, and synkinetic twitching of the mentalis muscle. Findings on the remainder of the cranial nerve examination as well as on the neurologic and head and neck examinations were within normal limits and symmetric.
Computed tomography (CT) and magnetic resonance imaging (MRI) were performed to rule out an intracranial abnormality. High-resolution CT showed expansion of the right geniculate fossa to 5 mm along with an area of central calcification and no extension to the cochlea (figure 1). On MRI, intense enhancement of the right geniculate ganglion was noted with gadolinium contrast (figure 2). These findings are consistent with the presence of a facial nerve hemangioma.
Options for treatment were discussed with the patient. They included surgical removal of the tumor or continued observation. Since the patient presented with a relatively stable unilateral facial weakness and no tumor growth, observation was recommended. Surveillance of the tumor at 1 year after the initial visit demonstrated no growth of the tumor and no new or worsening symptoms.
In general, hemangiomas tend to occur early in life, and most of them involute as the individual ages. (2) Facial nerve hemangiomas, however, tend to grow over time and they sometimes compress or invade adjacent structures. (3) Manifestations include sudden, progressive, or recurrent unilateral facial paralysis or spasm. In cases where the hemangioma invades the internal auditory canal, unilateral sensorineural hearing loss or vertigo may occur. (4,5) One of the most common sites of origin of facial nerve hemangiomas is the perigeniculate region. (5)
The mainstay of diagnosis is imaging. On high-resolution CT, the tumor may contain calcified intratumoral spicules with a honeycomb appearance and cause irregular expansion of the surrounding bone. Facial nerve hemangiomas appear hypointense on T1-weighted MRI and hyperintense on T2 weighting, and they enhance with gadolinium contrast. (4,6)
Other neoplasms that occur in the same region (e.g., schwannomas and meningiomas) tend to have a similar appearance on CT and MRI. In general, hemangiomas tend to be smaller than schwannomas at the time of diagnosis. (4) In our patient, indications that the mass was a hemangioma included the early age of onset and the characteristic location in the perigeniculate region. The recommended management for all tumors in this area is surgical removal, although the timing of surgery remains controversial. (7)
Cases of both compressive and invasive facial nerve hemangiomas have been documented. (3,8) Perigeniculate tumors have been found to be more invasive in nature than facial nerve hemangiomas in other locations? The ability to resect the tumor and maintain facial nerve integrity is therefore less likely in this region. Typically, a middle cranial fossa approach is employed to access the tumor. If facial nerve infiltration is evident, the involved segment of the facial nerve is resected and a graft is placed. (9)
In terms of outcomes after grafting, the best postoperative results have been a House-Brackmann grade III function? Various authors have recommended excision of the tumor and facial nerve repair with nerve grafting within 1 year of the onset of a facial nerve deficit (10) or within 2 years after the onset of complete preoperative facial paralysis. (11)
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(2.) Hassmann-Poznanska E, Kurzyna A. Hemangiomas and vascular malformations of the head and neck [in Polish]. Otolaryngol Pol 2006;60(5):663-74.
(3.) Miyashita T, Hoshikawa H, Kagawa M, Mori N. A case report of facial nerve hemangioma. Auris Nasus Larynx 2007;34(4):519-22.
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(5.) Friedman O, Neff BA, Willcox TO, et al. Temporal bone hemangiomas involving the facial nerve. Otol Neurotol 2002;23(5):760-6.
(6.) Achilli V, Mignosi S. Facial nerve hemangioma. Otol Neurotol2002; 23(6):1003-4.
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(8.) Escada P, Capucho C, Silva JM, et al. Cavernous haemangioma of the facial nerve, l Laryngol Otol 1997;111 (9):858-61.
(9.) Shelton C, Brackmann DE, Lo WW, Carberry JN. Intratemporal facial nerve hemangiomas. Otolaryngol Head Neck Surg 1991;104(1): 116-21.
(10.) Falcioni M, Russo A, Taibah A, Sanna M. Facial nerve tumors. Otol Neurotol 2003;24(6):942-7.
(11.) Salib RJ, Tziambazis E, McDermott AL, et al. The crucial role of imaging in detection of facial nerve haemangiomas. J Laryngol Otol 2001;115(6):510-13.
Edward C. Wu, MD, MBA; Vanessa S. Rothholtz, MD; Shawn Zardouz, BA; Alice D. Lee, MD, MPH; Hamid R. Djalilian, MD
From the Department of Head and Neck Surgery, University of California Los Angeles Medical Center (Dr. Wu); the Department of Otolaryngology-Head and Neck Surgery, University of California Irvine Medical Center, Orange, Calif. (Dr. Rothholtz, Mr. Zardouz, and Dr. Djalilian); and the Michigan Ear Institute, Farmington Hills, Mich. (Dr. Lee). The case described in this article occurred at the UG Irvine Medical Center.
Corresponding author: Hamid R. Djalilian, MD, Department of Otolaryngology-Head and Neck Surgery, UC Irvine Medical Center, 101 The City Drive South, Pavilion II, Orange, CA 92868. Email: firstname.lastname@example.org
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|Title Annotation:||ORIGINAL ARTICLE|
|Author:||Wu, Edward C.; Rothholtz, Vanessa S.; Zardouz, Shawn; Lee, Alice D.; Djalilian, Hamid R.|
|Publication:||Ear, Nose and Throat Journal|
|Article Type:||Case study|
|Date:||Jun 1, 2013|
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