Juvenile angiofibroma

Juvenile Angiofibroma Review of 120 Cases H. Bryan Neel,.lll, MD, Rochester, Minnesota James H. Whicker, MD{Rochester, Minnesota Kenneth D. Devine, M D, Rochester, Minnesota Louis H. Weiland, MD, Rochester, Minnesota

In juvenile angiofibroma, the typical patient is a young boy who has had an occasional nosebleed and whose nose has become somewhat obstructed; he may have some bulging of the face or eye, and examination shows a pale, bluish; smooth mass in the nasopharynx. The purpose of this paper is to present the Mayo Clinic experience involving 120 patients with microscopically confirmed juvenile angiofibroma who were seen from January 1, 1945 through December 31, 1971. The last series reported from this clinic involved 51 patients seen from January 1, 1940 through December 31, 1949 [I]. The two series overlap by five years. The overlap has allowed us to evaluate the two forms of treatment most commonly utilized during this period. From 1945 to 1955 the treatment consisted primarily of interstitial irradiation with radon seeds. The main approach was a transantral one through a Caldwell-Luc incision. From 1955 to 1971 avulsion of the tumor was the primary method of removal, although early in this era, electrocoagulation and implantation of radon seeds were employed frequently. Lateral rhinotomy was used to expose the tumor and its extensions.

Material and Methods

patients had carotid arteriography. The lateral rhinotomy approach was employed in fifty-six patients. Follow-up information on 113 patients was obtained two months to three years after treatment; seven patients were lost to follow-up study.

Findings

Age and Sex. All 120 patients were white males who, when first seen at the Mayo Clinic, ranged in age from seven to twenty-nine years, with a mean of fifteen years. (Figure 1.) Symptoms and Signs. The triad of nasal obstruction, epistaxis, and nasal drainage was the most common combination of symptoms and signs. (Table i.) The mean duration of symptoms and signs was 19.9 4-. 1.9 (SE) months. Forty patients had these symptoms less than one year, thirty-five from one to two years, fifteen from two to three years, and nineteen from three to eight years. ~, 30 .....

% \

20-

From 1945 through 1971, 120 patients with histologicaUy confirmed juvenile angiofibroma were seen at the Mayo Clinic. All patients had general otorhinolaryngologic examinations, laboratory studies consisting of a hemogram and urinalysis, and roentgenograms of the thorax and paranasal sinuses. Several patients underwent tomography of the paranasal sinuses, and a few From the ~.4ayoClinic and Mayo Foundation, Rochester,.Minnesota. Reprint requests should be addressed to Section of Publications, Mayo Clinic, Rochester, Minnesota 55901. Presented at the First Joint Meeting of the Society of Head and Neck Surgeons and the American Society for Head and Neck Surgery, Hot SDrings, Virginia, April 30 to May 2, 1973.

Volume 126, October 1973

5

15

25

35

Age Figure 1. Age distribution of 120 patients with juvenile angiofibroma.

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TABLE

I

Symptoms and Signs in 120 Cases of Juvenile Angiofibroma Patients Symptom and/or Sign

Nasal obstruction Epistaxis Nasal drainage Facial deformity Deafness or otitis (or both) Bulging palate Proptosis Other

Number Per cent 110 84 25 23 15 12 10 24

92 70 21 19 13 10 8 20

logic disturbances, and four of these had evidence of extensive involvement of the middle cranial fossa at the time of death. Treatment. Prior to evaluation at our clinic, 91 of the 120 patients had had one or more procedures: biopsy, excision, irradiation, electrocoagulation, or hormone therapy. The mean duration between diagnosis and registration at our clinic was 41.8 months. Of the fifty-nine patients in whom surgical approaches were used, fifty.six underwent lateral rhinotomy. (Table IV.) This approach in the opinion of the surgeon resulted in the complete removal of tumor in forty cases and the incomplete removal in seven; in nine cases the outcome was not stated. Fifteen patients required two or more secondary procedures after lateral rhinotomy. Twelve patients had electrocoagulation or implantation of radon seeds in residual tumor or both, one patient had external irradiation, one patient had secondary ligation of the external carotid artery, and three patients had repeated lateral rhinotomies for excision of residual tumor.

Tumor Location. The t u m o r was located on one side or the other of the nasopharynx in eighty-two patients and it filled the nasopharynx in twentyfour. (Table II.) Ulceration was unusual unless the patient had had previous biopsy or therapy. Roentgenographic bindings. Seventy per cent of the patients had definite roentgenographic abnormalities. (Table III.) There was obvious opacity involving one or more sinuses in fifty-two patients, bone displacement or erosion (or both) in fifteen patients, and both opacity of sinus(es) and bone involvement in twenty patients. T h e maxillary sinus alone was most commonly involved; next in frequency were the maxillary, ethmoid, and sphenoid sinuses in combinatio.~s. In 1965 l ~ l m a n and Miller [2] described the roentgenographic manifestations of forty-six of our patients. They found bony erosion of the body of the sphenoid in thirty, anterior bowing of the posterior wall of the maxillary a n t r u m in forty, erosion of the hard palate in eighteen, and enlargement of the superior orbital fissure in sixteen. We found tumor invasion of adjacent structures in approximately 80 per cent of our patients. Of the six patients in our series who died, five had evidence of extensive disease such as facial deformity, bulging palate, exophthalmos, and neuro-

without disease. (Table V.) Nine patients for whom the surgeon did not state whether all of the tumor was removed are alive and free of tumor. Of the seven patients with known incomplete removal, two are alive without disease, three have small symptomless masses in the nasopharynx, and two have died as a result of the tumor. Three others have died, and one was lost to follow-up study. Deaths. The five patients with lateral rhinotomy who died (age ten to seventeen years) had had symptoms from two to forty-eight months. All five had roentgenographic evidence of extensive involvement of maxillary ethmoid and sphenoid sinuses. O n e patient had a tumor of the upper part Of the neck t h a t extended beneath the mandible, bulging the upper part of the neck. Three

TABLE II

TABLE I I I

Sites of Tumor in 120 Cases of Juvenile Angiofibroma

Site Nasopharynx and nose Right side Left side Filling entire nasopharynx Midline tntranasal only Not stated Total

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Number of Patients

48 34 24 2 2 10 120

Outcome and Regression after Late'ral Rhino. tomy. Of the fifty-six patients, forty-five are alive

Roentgenographic Findings in 120 Cases of Juvenile Angiofibroma Number of Patients

Sinus(es) Involved Maxillary onl~' Maxillary, etl3moid, and sphenoid Maxillary and ethmoid Sphenoid only Maxillary and sphenoid Ethmoid and sphenoid Ethmoid Total

28 20 18 10 6 3 2 87

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Juvenile Angiofibroma

TABLE IV

Primary Treatment in 120 Cases of Juvenile Angiofibroma

TABLE V

Status after Lateral Rhlnotomy for Juvenile Angiofibroma Patients

Patients Treatment Excision Radon seed implantation and electrocoagulation External irradiation Biopsy only Tot al

Number

Per cent

59

49

50 6 5 120

42 5 4 100

patients had been operated on previously, and one patient had been treated with radon seeds. Four patients had facial deformity, exophthalmos, bulging palate, a mass in the neck, or a combination of these. One patient had had partial removal of the tumor before we saw him. He had facial deformity, exophthalmos, serous otitis media, and involvement of the ipsilateral maxillary, ethmoid, and sphenoid sinuses. Seven months after lateral rhinotomy, he had recurrence, and interstitial radon seeds were employed on four occasions. Three months after his last treatment, he died of exsanguination at home. A second patient had been operated on three times and had been treated with radon seeds twice before his referral. Six years after lateral rhinotomy, tumor filled the operated region and ipsilateral submaxillary triangle. Suprahyoid dissection and lateral rhinotomy were performed. He died at home eight years after his first lateral rhinotomy; the immediate cause of death was bronchopneumonia. There was intracranial extension and direct extension of tumor into the neck, around the trachea, and into the lungs. The third patient had undergone two lateral rhinotomies. At the second rhinotomy, radon seeds were implanted in the t u m o r involving the cavernous sinus, Exsang'uination occurred at home two years later. Autopsy revealed an angiofibroma that was 5 cm i n diameter and located within the middle cranial fossa; the lesion had elevated the temporal lobe, displaced the pituitary gland, e x t e n d e d into the cavernous sinus, and infiltrated around the carotid artery. The fourth patient died during a fifth rhinotomy four years after the first rhinotomy. At the first rhinotomy, the tumor had involved the sphenoid and cavernous sinus, ithe nasopharynx, and the pterygomaxillary and infratemporal fossae. The tumor was extremely vascular, and after the fourth rhinotomy, he had massive hemorrhage during removal of the pack. Right frontotemporal craniotomy was per-

Volume 126, October 1973

Status Alive Without disease With disease Dead Lost to follow-up study Total

Number

Per cent

50 (45) (5) 5 1 56

89 (80) (g) 9 2 100

formed so t h a t intracranial ligation of the internal carotid artery could be carried out. A tumor, approximately 3 cm in diameter, was found in the middle cranial fossa. The patient survived the procedure and returned two years later with extensive regrowth of the tumor. The fifth rhinotomy was performed with cryosurgery, but the patient died of cardiac arrest. The fifth patient had been operated on before referral. Lateral rhinotomy was performed, and all of the tumor was removed. Cardiac arrest and death, presumably from hypovolemia, occurred during wound closure. Complications. Postoperative and late complications after lateral rhinotomy consisted mainly (twelve patients) of nasal crusting or sequestrum (or both). (Table VI.) Five patients had neurologic changes consisting of either diplopia or conductive deafness, and two complained of an unsightly scar. Thirteen patients had other complications consisting of serous otitis media, nasal septal perforation, or nasal synechia, or a combination of these. One patient had an oral-antral fistula, and one patient had a cerebrospinal fluid leak. Blood Replacement. The mean volume of blood replacement required by forty-nine of the fifty-six patients in the rhinotomy group was 1,279 ± 157 ml; seven patients did not require transfusion. TABLE Vl

Postoperative and Late Complications in 120 Cases of Juvenile Angiofibroma Patients

Complication Nasal crusting or sequestrum (or both) Hemorrhage requiring transfusion Eye symptoms or deafness (or both) Unsightly scar Meningitis Other

Lateral Rhinotomy

Interstitial Irradiation

12

39

6 5 2 0 13

11 9 0 2 26

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The volume of blood replacement during the opera t i o n or in the immediate postoperative period ranged from 100 to 5,500 ml at the first operation. Most (70 per cent} of the patients who had blood replacement required infusions of 500 to 2,000 ml. Rt!.don Seeds. In our experience, irradiation delivered'l~y radon seeds approximately doubles all complications, compared with complications from excision. (Table VI.) Fifteen of the fifty-six patients treated by lateral rhinotomy required two or more procedures, whereas thirty-five of the fifty treated with radon seeds required two or more seedings. One of our patients who had had external irradiation prior to lateral rhinotomy had hemifacial atrophy, and several patients had obvious skin changes. Ten patients had had external irradiation before coming to our clinic. Other Adjuncts. We commonly used electrocoagulation until 1955. Since then,, we have used it less and now hardly ever use it. We have used cryotherapy in two patients, once in the present series. Both patients had recurrence. The tumors were so large t h a t we could freeze only a small portion, and when the frozen part was removed, bleeding in the r e m a i n i n g part was a problem. Butler, Nahum, and Hanafee [3] reported t h a t they could not remove any portion of the tumor while it was frozen. We have not used hyi~othermia but have used hypotensive anesthesia in ten operations and have found no improvement in the m a n a g e m e n t of the bleeding problem. We have not administered hormones although some of our patients had had this t r e a t m e n t before coming to our clinic. Six patients had ligation of the external carotid artery just prior to excision of the tumor, without any convincing evidence that the procedure reduced the loss of blood.

Point of Origin. The lesion is lateralized and always obstructs or fills to some degree one nasal cavity; this has led to the belief t h a t the origin is somewhere in the posterior part of the nasal cavity and not in the nasopharynx. The specific point of origin is on the posterior lateral wall of the roof of the nose, where the sphenoidal process of the palatine bone meets the horizontal ala of the vomer bone and the root of the pterygoid process of the sphenoid bone. This junction forms the superior margin of the sphenopalatine foramen (Figure 2), and the ethmoid crest (or a t t a c h m e n t of the posterior end of the middle turbinate) lies on or above the foramen. This site represents the approximate location of the a t t a c h m e n t of the buccopharyngeal membrane in the embryo [4], which is the boundary between the stomatodeal ectoderm and the foregut endoderm: Rathke's pouch marks tile line on the roof. The site is also the junction of the membranous viscerocranium, represented by the palatine, vomer, and pterygoid lamina and the sphenoid bone formed in the cartilaginous neurocranium. The point of origin used to be of more interest to surgeons than it is now, as witnessed by the numerous articles about this is the early literature. The belief was that the tumor originated in the vault of the nasopharynx (fibrocartilage of Tillaux [5] or fascia basilaris of Brunner [6]) or at the spheno-occipital synchondrosis. Hohnan and Miller [2] noted roentgenographic evidence of destruction of the body of the sphenoid in nineteen of thirty patients, but in all patients the destruction occurred in front of the spheno-occipital synchondrosis. Sebileau [7], Jacques [8], and Figi and Davis [1], among others, believed that the primary

Figure 2. Left nasal cavity showing point of origin, which lies near superior margin of sphenopalatine foramen at attachment of posterior end of middle turbinate.

Figure 3. Right side of skufl showing infratemporal, temporal, and pterygomaxillary fossae, and their relation to sphenopalatine foramen, pterygomaxillary fissure, and posterior waft of maxillary antrum.

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Comments

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Juvenile A n g i o f i b r o m a

site of origin was the back of the nose at the margin of the choana. The question of origin still remains. S a m y and Girgis [9] stated that the ability of a benign tumor with such big lobulations to move through the narrow pterygomaxillary fissure is difficult to accept. G u p t a [10] also believed that the tumor exited behind the pterygoid plates (not in front of them) and through the pharyngobasilar fascia, between the upper border of the superior constructor muscles and the base of the skull, and destroyed the plates from behind forward. We believe that the tumor reaches the infratemporal and temporal fossae through the pterygomaxillary fissure in front of the pterygoid plate. If the posterior wall of the maxillary sinus is removed, one can often find the tumor in the pterygomaxillary fossa when the pterygoid plates are intact. Natural Course. The tumor first grows under the mucosa just inside the posterior choanal margin on the roof laterally. As the tumor enlarges, it extends under the mucosa along the roof, reaching the posterior border of the septum and extending downward along this margin (the tumor almost always has to be separated from the septum along its posterior margin at surgery), forming a projecting mass in the roof of the posterior nasal cavity. The lesion grows forward to fill the nasal cavity, crowding the septum into the opposite nasal cavity and flattening the turbinates. The lesion does not enter the maxillary sinus through the lateral wall of the nose, b u t comes out of the posterior choana, where it may fill the nasopharynx, displace the soft palate, and be visible below the free edge. As the lesion grows, it pushes laterally through the sphenopalatine foramen and expands the posterior end of the middle turbinate which becomes continuous with the mass (a constant finding at operation). Having entered the pterygomaxillary fossa [II, I2] through t h e s p h e n o p a l a t i n e foramen, the lesion exerts pressure on th~..surrounding bony walls, pushing itself into the anterior wall of the pterygomaxillary fossa (Figure 3); =iwhich is the posterior wall of the maxillary sinus, and destroying the root of the pterygoid process 0 f t h e sphenoid bone posteriorly. The lesion continue~s.lt0 . enlarge into the infratemporal fossa t h r o u g h the pterygomaxillary fissure which it has expanded to form the classic bulging (Figure 4) in the cheek; if the lesion becomes large enough, it can bulge into the lower part of the temporal fossa and form a swelling above the zygoma. When this happens, the lesion usually moves into the inferior orbital fissure, which opens into the upper anterior part

Volume 126, October 1973

Figure 4. Patient with juvenile angiofibroma extending into infratemporal fossa causing bulging in right

cheek.

of the pterygomaxillary fossa, and enters the lower end of the superior orbital fissure, which meets the inferior orbital fissure here in the posterosuperior wall of the pterygomaxillary fossa. When it reaches this site, the lesion destroys the great wing of the sphenoid bone, forming the characteristic widening along the lower lateral margin of the superior orbital fissure. (Figure 5.) When this happens, proptosis becomes apparent. The lesion enlarges in the infratemporal and pterygomaxillary fossae and destroys the bone that forms the base of the pterygoid processwhere the body and great wing of the sphenoid bone meet. When this bone is destroyed, the lesion lies against the dura of the middle fossa, anterior to the foramen lacerum and lateral to the cavernous sinus. Meanwhile, the lesion grows straight up from its point of origin through the floor of the sphenoid sinus and, if it continues to expand, fills the sinus and comes out through the top, pushing the pituitary gland to one side and appearing i n the sella turc~ca, where it can cause blindness by pressure on the optic chiasma. Thus, the lesion enters [13] the cranial cavity in the middle fossa either anterior to the foramen lacerum and lateral to the cavernous sinus and carotid artery or through the sella medial to the carotid artery and lateral to the pituitary gland or by both paths. The speed with which the angiofibroma can "accomplish" all this is unknown. Some tumors are more aggressive, b u t attempts to identify these by microscopic examination, gross appearance, age of patient, or duration o f s y m p t o m s are not satisfactory. Regression. Clinicians h a v e been intrigued by the question of spontaneous regression at or about the age of twenty-five years; the tumor is extremely rare after the age of twenty-five years. Regression after incomplete surgical removal or radiation therapy is not uncommon, b u t we have not seen

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Figure 5. Sinuses showing enlargement of superior orbital fissure. (From Holman CB, Miller WE [2J. By permission of the American Roentgen Ray Society, inc.) Figure 6. Sinuses showing the a;tterior bowing of the posterior waft of the maxillary sinus. (From Holman CB, Miller WE [2]. By permission of the American Roentgen Ray Society, Inc.)

a n y patient who had spontaneous regression of the tumor. In a patient with symptoms, one should not wait for regression. Se x . Although the disease is almost exclusively limited to the male, it can occur in females. Svobode and Kirchner [14J stated that an angiofibroma removed from a female was indistinguishable from those removed from males. Fitzpatrick [15] had three females in his series, two of whom were evaluated to determine genetic makeup and endocrine states. Laboratory studies, the details of which were not stated, confirmed the sex identity of the two girls. Hunter, Smyth, and Mac qfee [16] studied the twenty-four hour excretion of ketosteroids in seven boys who had angiofibroma. The ~tudies were performed because of the belief that boys with this tumor may have an endocrine abnormality; however, no endocrine abnormality was found in any of the seven patients. Diagnosis. Not every boy with nasal obstruction, nosebleeds, and a polypoid tumor in the nasopharynx has juvenile angiofibroma, and diagno. sis may be difficult. Conventional roentgenograms of the sinuses, submentovertical views, tomography, physical examination, and knowledge of the natural history and point of origin do help in diag: nosing and determining the extent of the tumor. Holman and Miller [2], reporting on the roentgenologic characteristics of forty-six cases of angiofibrema, en~phasized the anterior bowing (Figure 6) of the posterior walt of the maxillary sinus, the enlargement of the superior orbital fissure (Figure 5), and the characteristic bone erosion of the adjacent structures. Angiography may aid in diagnosis; if there is rapid dye uptake (staining), then the lesion is an angiofibroma (and vice versa). In determining the extent of the tumor and in detecting [17] residual tumor, the technic of subtraction [18] angiography is of considerable assistance. These studies are not without risk, as shown by the report of a death

552

ten hours after angiographic studies for angiofibroma [I5], permanent transverse myelit:is [19] in another patient in a series of sixteen patients, the development of transient hemiplegia [20] in one patient in a series of two, and the mention [3] of another patient in whom hemiplegia developed b u t who recovered most of his function in ninety days. We agree with Holman and Miller [2], who emphasized that the roentgenographic features are so characteristic that only in unusual circumstances would angiography be necessary. If, after physical examination and roentgenographic studies, doubt remains about the nature of the lesion in a patient with a symptomatic tumor, we examine the patient under anesthesia, perform biopsy if there is still doubt, and then remove the tumor transnasally, transantrally, or by rhinotomy, depending on the pathologic diagnosis. A limited rhinotomy incision, which does not cut through the lip, gives excellent: exposure for removal of sphenochoanal polyps or similar fibromyxomatous polyps involving the sphenoid or nasopharynx, without causing any harm to the patient and eliminating the tumor and the distressing symptoms, and at the same time confirming the diagnosis. T r e a t m e n t Blood supply and hemorrhage: Bleeding a t surgery and when the biopsy specimen is taken, as well as the t r o u b l e s o m e s y m p t o m of spontaneous epistaxis, has interested the medical profession since the time of Hippocrates and Celsus [2I]. Studies with angiography [18] and subtraction technic revealed that the main blood suppl~ comes from an enlarged internal maxillary artery. The venous plexus draining the tumor consists of a tangle of numerous small irregular vessels. The blood supply can come from both sides of the neck, particularly when the external carotid artery has been ligated. Arterial supply has been identified from dural, sphenoidal, and ophthalmic branches of the internal carotid and the vertebral artery and thyrocervical trunk. The tumor itself

The American Journal of Surgery

Juvenile Angiofibroma

m a y be a pulsating, compressible spongelike mass, b u t when it is removed, its marked vascularity is not apparent without the use of the microscope. Microscopically, the tumor has two striking features: a fibrous stroma and a rich vascular network. (Figure 7.) The fibrous stroma varies. In some areas, the cells resemble'young fibroblasts and are spindled, plump, and stellate. In other areas, the stroma is less cellular, the cells being replaced by collagen. The vascular channels vary from capillary-size to venous-size and are lined by endothelial cells; this lesion is properly called "hemangiofibroma." The endothelial cells lie directly against the stromal cells, without any intervening smooth muscle, and this feature undoubtedly contributes to the capacity for the massive bleeding t h a t sometimes occurs. The tumor inside the nose, sphenoid, and nasopharynx is covered with mucosa; the lobulations t h a t extend laterally through the pterygomaxillary fossa have a firm fibrous pseudocapsule, and manipulation of these lobule=s~is:less likely to cause troublesome bleeding than would m a m p u l a t m n of those within the nasopharyr~x or nasal cavity. Attempts to manage the bleeding have been made by ligating the carotid or internal maxillary artery or by occluding the terminal arterioles and spaces within the tumor by embolization of Silastic ® spheres [22]. Angiographic studies by Wilson and Hanafee [19] have shown t h a t if recurrence follows ligation of the external carotid artery, the new blood supply is less likely to originate from a vessel t h a t is easy to control at the second operation. The problem has been attacked more directly by using cocaine packs, injection of eplnephrine-saline solution into the t u m o r base [11], electrocoagulation, and freezing [23]: Hormone therapy [24,25] and radiation therapy [15,26,27] are used to change the endothelial lining of the vascular spaces and hopefully to decrease the vascularity. Hypotension (artificially produced) and hypothermia [28] have been recommended. Whether a particular angiofibroma will be extremely vascular at surgery cannot be predicted /

•

.

.

rhinotomy t h a t was practiced 100 years previously. There are eight approaches: 1. The natural orifice approach has been described by Furstenberg and Boles [32], who reco m m e n d e d avulsion of the main mass in the nasopharynx, with a special clamp being introduced through the mouth. By manipulation through the nose, the obstructing part of the tumor can be pulled out. T h e y Condemned as being too traumatic and not •practical any operations t h a t involved external incisions and bone removal. 2. The transpalatal approach originally was a midline approach in an anteroposterior direction, but Wilson [29] popularized the transverse incision. Some of the advocates of the transpalatal approach recommend rhinotomies or alveolobuccal and transantral incisions [33-37] to expose the cheek extension, and Cocke [38] believed t h a t the transpalatal approach was not practical for any tumor larger t h a n 5 cm. Pressman [28] believed t h a t the approach through the palate was difficult if the lesion was large, because the inferior face of the tumor is reached whereas the a t t a c h m e n t is ordinarily superior. In the transpalatal approach, the operator can get closer to the roots, although not necessarily near the various extensions. This should not present any problem if you believe as Thomsen [34] does: " . . . when the tumor has invaded neighboring regions, it has not only paved the way for its invasion, but also created a pathway for its surgical withdrawal." Unfortunately, the tumor has a predilection to form large lobules once it gets through the various fissures ahd foramina. 3 and 4. The transmandibular [39] and transzygomatic [9] approaches are tailored to expose the extension of the t u m o r into the infratemporal and

[29]. Surgical exposure: In 1834 Chelius [30] wrote t h a t the danger of hemorrhage was always less if the tumor could be extirpated with the roots, and this is as true today as it was then. The problem remains the same: How to get at the roots. In 1911 Hellat [31] surveyed fifty-five different methods with the aim of determining a technic for good exposure. In 1957 Wilson [29] told of a method of approach to the n a s o p h a r y n x by lateral

Volume 126, October 1973

Figure 7. Typical juvenile angiofibroma (hematoxylin and eosin stain; original magnification × 60).

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Neel et al

IIIIII \

Figure 8. Skin incision that is most commonly used in the t r e a t m e n t of juvenile angiofibroma.

temporal fossae but either could lead to much difficulty in getting at the other extensions of the tumor. 5. The transhyoidal approach gives reasonable exposure of the nasopharynx but is inadequate if there are other extensions. 6. The transantral approach [40] is often used in combination with the transpalatal approach. Figi and Davis [1] used the Caldwell-Luc incision and the transantrat operation almost exclusively. The resort to electrocoagulation and radon seeds furnishes testimony to the inadequacy of this approach. 7. The craniotomy-rhinotomy approach is reco m m e n d e d by Krekorian and Kempe [41J for any patient with a benign t u m o r of the nasal cavity t h a t has evidence of definite or suspected intracranial extension. Even this approach m a y not be

Figure 9. Patient with i n t r a c r a n i a l extension, progressive proptosis, and loss of vision after excision of angiofibroma. There is no t u m o r in the nose o r nasopharynx. Patient was treated with 6°Co, causing arrest of tumor.

554

satisfactory if the extension has impacted itself extensively in the" middle cranial fossa or pituitary fossa. 8. The lateral rhinotomy approach is excellent for getting at the extensions upward into the sphenoid sinus and the pterygomaxillary fossa and its roots in the lateral wall of the nose. Lateral rhinotomy: The skin incision (Figure 8) begins or ends just under the medial aspect of the brow and curves downward and forward to .within 5 mm of the inner canthus. It then curves forward and downward on the side of the nose to the base of the ala where it may terminate if the rhinotomy is a limited one. The extent of the incision usually can be determined before operation, by physical and roentgenographic examinations. Most angiofibromas extend beyond the nose and nasopharynx, and the incision must be continued around the base of the nose to the base of the eolumella, where it turns and runs down the center of the upper lip. The incision on the mucosal surface of the upper lip has a " Z " incorporated into it. The incision reaches the depth of the alveolobuccal sulcus and continues in the sulcus to the maxillary tuherosity. The only important structures encountered are the angular vein and the facial artery. The underlying bone must be adequately removed so as to get good exposu'.'e, and this should be completed before the tumor is touched. The nasal bone is removed to the midline and to its junction with the frontal bone. The frontal process of the maxilla and the facial surface of the maxilla are removed. A small margin of bone around the infraorbital nerve at the level of the foramen is preserved. By this time, t h e a n t r u m is opened and the posterior wall is inspected. If the a n t r u m is bulging forward excessively, more exposure is needed. The infratemporal surface of the maxilla should be removed, along with the eggshell of bone on the posterior wall of the antrum. The terminal branches of the internal maxillary artery usually can be identified and either ligated or electrocoagulated. The tumor can be worked out of the pterygomaxillary, infratemporal, or temporal fossa and excised or pushed medially with packing. The fatpad of the cheek is usually in the way and often has to be removed. As the frontal process of the maxilla is removed, the nasolacrimal sac and duct are exposed. The bone surrounding these should be carefully removed so that they are standing free. The nasoantral wall below the inferior turbinate is removed from front to back. The nasal

The American Journal e! Surgery

Juvenile Angiofibroma

mucosa is entered in front of the anterior end of the inferior turbinate, and the incision is continued backward along the floor of the nose. In front, the incision is continued upward to the roof~of the nose in front of the anterior end of the middle turbinate. The inferior turbinate is severed posteriorly. The posterior end of the middle turbinate is incorporated into the tumor, and must be severed anterior to this. If the bone has been removed thoroughly around the nasolacrimal sac and duct, the remnants of the two turbinates and the middle m e a t u s can be lifted from the wound or pushed out of the line of sight. The periosteum of the medial wall of the orbit is elevated carefully to avoid tearing and releasing the periorbital fat. As the periosteum is elevated posteriorly, the anterior ethmoid artery is identified and coagulated or clipped. The a t t a c h m e n t of the superior oblique muscle may have to be stripped off superiorly if more exposure is needed. The periosteal elevator usually lifts off below the attachment of the inferior oblique muscle. The lacrimal bone and the lamina papryacea of the ethmoid bone are removed, and at the same time, the ethmoid cells are removed, going along the cribriform plate. As one proceeds backward, the tumor may be encountered in the posterior ethmoid cells, b u t most often it is not encountered until the sphenoid sinus is reached. The tumor usually can be pushed downward out of the sinus with packing. The a t t a c h m e n t of the tumor to the posterior margin of the septum is then broken through, and the tumor is pushed laterally with packing;'there never is any attachment along the floor of the nose. All of the lobulations now should be free and be seen to communicate with each other and with the body of the tumor attached in the roof and lateral wall of the posterior nasal cavity. All intervening bone that would interfere with removal of the tumor should have been excised. Most large angiofibr0inas extend u n d e r the adenoid pad and lift it posteriorly in the vault and posterior base on the nasopharynx on the side of the lesion. Once the tumor is firmly grasped with a strong forceps, it is necessary to work with an elevator or index finger around the base of the tumor to aid in avulSion of the tumor. Upon removal o'f the tumor, a search is made for any residual fingers, lobules, or pseudopods remaining in the body of the sphenoid bone. T h e cavity is p a c k e d with Vaseline®-iodoform gauze, which is removed one week later with the patient under general anesthesia. Tracheotomy t u b e s a n d feeding tubes are unnecessary.

Volume 126, October 1973

Summary All young males with nasal obstruction or nosebleed (or both) should be suspected of having juvenile angiofibroma. This awareness, as welt as a good physical examination and history taking, is essential. Plain roentgenograms of the sinus, with submentovertical views, and tomograms should be sufficient adjuncts for cliagnosis. Knowledge of the point of origin in the posterior nasal cavity and the natural history of extension of the tumor into the nasal cavity, nasopharynx, sphenoid sinus, pterygomaxillary and infratemporal fossae, and cranial cavity is essential in planning treatment. Angiography for diagnostic purposes should be used only in unusual circumstances. The use of hormones, cryotherapy, carotid ligation, or hypotensive anesthesia probably will not be of any help in controlling hemorrhage at operation. Our experience with patients who have received irradiation and who still had the angiofibroma-with the ever present risk of irradiation [15] in the preadult p a t i e n t - - h a s caused us to be skeptical of this modality as a primary form of treatment. However, the radiotherapist is a member of the team managing the t r e a t m e n t of patients with this most difficult tumor. (Figure 9.) All operations presently used to obtain surgical exposure of the angiofibroma and its extension may be inadequate when the tumor bleeds excessively on manipulation. We believe.lateral rhinotomy, in one procedure, allows the surgeon to expose the extensions of the t u m o r and to remove the tumor from its point of origin in the posterior nasal cavity without disiurbing facial growth or causing facial deformity. Intracranial extension indicates advanced disease and is not unusual [13,16]. We have successfully removed angiofibromas involving the middle fossa, but four of our patients who died had evidence of intracranial angiofibroma.

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The American Journal of Surgery