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Progressive external ophthalmoplegia

Progressive external ophthalmoplegia is a disorder of the mitochondria. It is characterized by multiple mitochondrial DNA deletions in skeletal muscle. The most common clinical features include adult onset of weakness of the external eye muscles (ophthalmoplegia) and exercise intolerance. more...

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Additional symptoms are variable, and may include cataracts, hearing loss, sensory axonal neuropathy, ataxia, depression, hypogonadism, and parkinsonism. Both autosomal dominant and autosomal recessive inheritance can occur; autosomal recessive inheritance is usually more severe.

It is usually diagnosed by neurologists. There is no proven treatment; experimental agents such as coenyzme Q10 may provide benefit.


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Hemangioma of the sphenoid and ethmoid sinuses: Two case reports - Original Article
From Ear, Nose & Throat Journal, 3/1/03 by John D. Kilde


Hemangiomas of the paranasal sinuses are rare, particularly those of the sphenoid and ethmoid sinuses. Although imaging of the sinuses is key to determining the extent of involvement, the diagnosis is based on the lesion's histologic appearance. Obtaining an adequate biopsy can be difficult in light of the risk of bleeding and the relative inaccessibility of lesions in this region. These obstacles can make the diagnosis and management of these lesions particularly challenging. We describe two new cases of sinonasal hemangioma--one in the ethmoid sinus and one in the ethmoid and sphenoid sinuses-and we discuss the diagnostic and therapeutic interventions that are needed to manage these lesions.


Hemangiomas of the paranasal sinuses are rare, particularly those of the sphenoid and ethmoid sinuses. Fewer than 15 cases of ethmoid hemangioma have been previously reported since 1952.1 Vascular tumors that arise from the sphenoid sinus are extremely uncommon; only one report each of a hemangioma (2) and a hemangiopericytoma (3) of the sphenoid sinus has been previously published in the literature. Maxillary sinus and septal hemangiomas are more common, and multiple reports of each have appeared in the literature. (4-6)

Hemangiomas are classified as either cavernous (large space) or capillary (small space), depending on the size of the vessels that are predominant in the tumor. Hemangiomas of the paranasal sinuses can be difficult to diagnose for many reasons. Obtaining a biopsy specimen can be dangerous in view of the potential for profuse bleeding. Hayden et al described two cases of sphenoid hemangioma in which both patients died as a result of hemorrhage secondary to biopsy. (2) Histologic diagnosis is complicated by the fact that the appearance of sinonasal hemangiomas can be similar to that of other lesions; such similarities can make it extremely difficult to establish a definitive diagnosis on the basis of small-sample biopsies. Finally, sinonasal hemangiomas can also be clinically similar to other histologically distinct lesions that arise in the same region.

We treated two patients with sinonasal hemangiomas--one in the ethmoid sinus and one in the ethmoid and sphenoid sinuses. We describe our diagnostic and treatment strategies, with emphasis on the lesions' clinical appearance, radiographic features, and histologic characteristics.

Case reports

Patient 1. We evaluated a 9-year-old girl who had a history of right nasal obstruction. She denied epistaxis. Rhinoscopy detected a blue-gray mass that occluded the right nasal passage and extended into the nasopharynx.

Contrast-enhanced computed tomography (CT) in the coronal plane demonstrated an enhancing, expansile mass in the right nasal cavity and ethmoid sinus, with extension into the right orbit (figure 1, A). Magnetic resonance imaging (MRI) further delineated the extent of soft-tissue involvement and the characteristics of the mass (figure 1, B). A coronal gadolinium-enhanced Ti-weighted MRI with fat saturation demonstrated intense enhancement in the mass and signal voids consistent with the presence of vessels. The extension into the orbit was noted, but no involvement of the globe was seen. No intracranial involvement was evident. The differential diagnosis at this point included tumors that are characterized by high vascularity (e.g., rhabdomyosarcoma) or a tumor of vascular origin.

The patient was taken to the operating room for biopsy of the anterior aspect of the lesion. The biopsy caused copious bleeding, which was controlled with direct pressure. However, the biopsy specimen was nondiagnostic, and rhabdomyosarcoma was entertained as the leading suspect. The patient was taken for angiography to identify the vascular supply and for embolization prior to further biopsy and possible excision (figure 2, A). Embolization of the internal maxillary artery was successful in reducing blood flow to the lesion (figure 2, B). Following embolization, the patient was brought to the operating room for another biopsy. The second specimen was also nondiagnostic, and the decision was made to remove the lesion while the embolization was still effective.

The following morning, the patient underwent a lateral rhinotomy, medial maxillectomy, and excision of the lesion. The lesion was found to be adjacent to, but not invading, the periorbita and the dura. The patient lost 600 ml of blood during the procedure. Once the lesion was removed, the bleeding was controlled under excellent visualization. Final pathology of the tumor revealed that it was a cavernous hemangioma. The patient tolerated the procedure well, and her postoperative course was unremarkable. After 4 years, she had had no evidence of recurrence.

Patient 2. A 22-year-old man was referred with a history of headache and diplopia on lateral gaze. A previous biopsy of a sphenoid mass had identified the lesion as a hemangioma. During the biopsy, the patient lost approximately 1 L of blood.

Findings on the physical examination were unremarkable, and no lesions were visible on anterior rhinoscopy. Coronal CT of the sinuses and skull base demonstrated soft-tissue opacification of the left sphenoid and posterior ethmoid sinuses (figure 3, A). Erosion of the lateral splienoid wall adjacent to the cavernous sinus was noted. Gadolinium-enhanced MRI demonstrated an intensely enhancing mass that extended from the sphenoid sinus into the left cavernous sinus (figure 3, B). The mass surrounded and partially compressed the carotid siphon and petrous segment of the internal carotid artery.

Angiography was performed to better identify the vascular supply to the lesion. The mass was found to be supplied by both the internal and external carotid arteries (figure 4). A balloon occlusion test of the internal carotid artery was performed prior to internal carotid embolization in order to determine the degree of functional deficit that would ensue if the vessel had to be sacrificed. The patient tolerated the occlusion test, and subsequent embolization was successful in reducing blood flow to the tumor.

The patient was taken to the operating room for excision of the lesion. An extended lateral rhinotomy incision was made, with division of the lip for better exposure. The lesion was removed with approximately 1 L of blood loss. The tumor was found to have involved the cavernous sinus, with extension into the cranial cavity. Because of the tumor's close proximity to vital structures, it was only partially excised; the intracranial portion of the tumor was left in place to reduce the morbidity of the procedure. Histopathologic analysis of the excised portion of the tumor identified dilated blood-filled vessels of variable size that were lined with flattened epithelium (figure 5). The final histologic diagnosis was a capillary hemangioma. Postoperatively, the patient's diplopia resolved. At the 14-month follow-up, there was evidence of a reduction in the size of the residual tumor.


In hemangiomas, the vessel walls are frequently thickened by fibrosis, and except for a few feeder vessels, no muscular layer is seen. The fibrosis probably represents the scarring of old infarcts. Some vessels are frankly engorged with blood cells. There is a diffuse and haphazard vascular proliferation without the lobular arrangement frequently seen in the cutaneous forms of capillary hemangioma. No sinusoidal pattern, inflammatory infiltrate, cellular atypia, increased mitotic activity, or endothelial proliferation is seen.

The differential diagnosis of hemangioma includes pyogenic granuloma, hemangioendothelioma, angiosarcoma, bacillary (epithelioid) angiomatosis, and Kaposi's sarcoma. In cases of pyogenic granuloma, however, the radiologic evidence of destruction and invasion seen with a hemangioma is not present; also, a hemangioma does not exhibit the secondary inflammatory changes that are frequently seen in pyogenic granuloma. A hemangioendothelioma is more cellular than is a hemangioma. A welldifferentiated angiosarcoma can be ruled out by the absence of nuclear pleomorphism and hyperchromatism, mitotic activity, or a freely anastomosing sinusoidal pattern. Bacillary angiomatosis and Kaposi's sarcoma are characterized by plump, atypical endothelial cells, which are not seen in a hemangioma.

Posterior sinonasal lesions can be difficult to diagnose because of the scarcity of vascular lesions in the sphenoid area and the difficulty involved in acquiring an adequate biopsy. The initial symptoms of sphenoid hemangioma are similar to those of other lesions. Symptoms are caused by the involvement of surrounding structures. Initial complaints are usually related to vision and can be attributed to the lesion's lateral extension and subsequent involvement of the VIth cranial nerve, which can lead to palsy of the lateral rectus muscle. Progressive involvement of the Und, IIIrd. and IVth cranial nerves and involvement of the blood supply can lead to complete ophthalmoplegia with ptosis or possibly orbital apex syndrome. (7)

Ethmoid masses generally cause nasal obstruction or epistaxis. They can also cause retro-orbital pain, proptosis, and ipsilateral optic atrophy as a result of erosion into the orbital cavity. (1) Other initial symptoms include pain and headache, which can occur in almost any region and are nonspecific. In addition to nasal obstruction orepistaxis, anterior extension can lead to rhinorrhea, hyposmia, and anosmia. Posterior extension can lead to central nervous system involvement. (8)

Imaging studies play a vital role in the work-up of a hemangioma. CT and MRI are both useful in evaluating the extent of the lesion. CT is better at defining the bony anatomy surrounding the tumor, and it detects focal areas of bony lysis. On CT, enhancement is seen throughout the lesion. Vascularity is better evaluated with MRI and/or conventional angiography. MRI is more sensitive than CT in detecting extension into adjacent compartments (e.g., the orbit and cranial fossa). MRI is also better than CT in identifying soft-tissue characteristics. Hemangiomas demonstrate an intermediate T1 signal and a high T2 signal prior to contrast administration. A high T1 signal prior to contrast suggests a hemorrhage. Hemangiomas enhance vividly with contrast; the presence of punctate or serpiginous signal voids that are consistent with blood flow in vessels is diagnostic of hypervascularity. Conventional angiography further defines the vascular supply to the lesion. Endovascular embolization prior to surgical resection decreases blood loss intraoperatively and prevents uncontrollable hemorrhage.

The diagnosis of hemangioma must be based on the lesion's histologic appearance. However, the histologic diagnosis of sinonasal vascular lesions is complicated by a number of factors. For example, the primary components ofahemangioma are vascular structures and fibrous stroma, but these components are seen in many other lesions found in this region. Also, the distinction between a cavernous and a capillary lesion is not always sharp because the size of the vessels can vary in different parts of the tumor. (9)

Management decisions can be complicated by the fact that the natural history and recurrence rates of paranasal sinus hemangiomas are unknown because so few cases have been reported in the literature. These tumors are considered to be resistant to radiation therapy, but evidence in the literature is anecdotal. Resection is the treatment of choice, but the involvement of surrounding structures can make excision a risky and morbid procedure.


(1.) Kim Y, Stearns G, Davidson TM. Hemangioma of the ethmoid sinus. Otolaryngol Head Neck Surg 2000;123:517-9.

(2.) Hayden RE, LunaM, Goepfert H. Hemangiomas of the sphenoid sinus. Otolaryngol Head Neck Surg 1980;88:136-8.

(3.) Kimmelman CP. Hemangiopericytoma of the sphenoid sinus. Otolaryngol Head Neck Surg 1981;89:713-6.

(4.) Most DS. Hemangioma of the maxillary sinus. Oral Surg Oral Med Oral Pathol 1985;60:485-6.

(5.) Ghosh LM, Samanta A, Nandy T, Das S. Haemangioma of the maxilla. J Laryngol Otol 1988;102:725-6.

(6.) Tsutsumiuchi K, Hasegawa M, Okuno H, et al. Haemangioma of the maxillary sinus. Some comments on pre-operative diagnosis. ORL J Otorhinolaryngol Relat Spec 1982;44:43-50.

(7.) Lapayowker MS, Ronis ML. Problems in tumors of the ethmoid and sphenoid sinuses. Adv Otorhinolaryngol 1974;21:19-31.

(8.) Alexander FW. Primary tumors of the sphenoid sinus. Laryngoscope 1963;73:537-46.

(9.) Heffner DK. Problems in pediatric otorhinolaryngic pathology. II. Vascular tumors and lesions of the sinonasal tract and nasopharynx. Int J Pediatr Otorhinolaryngol 1983;5:125-38.

From the Department of Otolaryngology and Communication Sciences (Dr. Kilde, Dr. Rhee, and Dr. T.L. Smith), the Department of Pathology (Dr. Balla), and the Department of Radiology (Dr. M.M. Smith), Medical College of Wisconsin, Milwaukee.

Reprint requests: Timothy L. Smith, MD, Department of Otolaryngology and Communication Sciences, MCW Clinic at Froedtert, Milwaukee, WI 53226. Phone: (414) 805.5581; fax: (414) 8057936: e-mail:

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