A rare case of nasopharyngeal angiofibroma in a pregnant woman

A rare case of nasopharyngeal angiofibroma in a pregnant woman

OtolaryngologyHead and Neck Surgery Volume 117 Number 6 3. Fullmer HM, Lille RD. The oxytalan fibres, a previously undescribed connective tissue fibr...

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OtolaryngologyHead and Neck Surgery Volume 117 Number 6

3. Fullmer HM, Lille RD. The oxytalan fibres, a previously undescribed connective tissue fibre. J Histochem Cytochem 1958;6:425-30. 4. Hamner JE HI, Fullmer HM. Oxytalan fibres in benign fibroosseous jaw lesions. Arch Pathol 1966;82:35-9. 5. Damjanov I, Maenga RM, Snyder GG, Ruiz JW, Toomey JM. Juvenile ossifying fibroma: an ultrastructural study. Cancer 1978;42:2668-74.

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6. Margo CE, Ragsdale BD, Eerman KI, Zimmerman LE, Sweet DE. Psammomatoid (juvenile) ossifying fibroma of the orbit. Ophthahnology 1985;92:150-9. 7. Burgess J, Greiner FG. Computed tomography of the skull bone. In: Delbalso AM, editor. Maxillofacial imaging. Philadelphia: W. B. Saunders Company; 1990. p. 263-89, 8. Jaffe HE. Tumours and tumourous conditions of the bones and joints. Philadelphia: Lea and Febiger; 1974. p. 117-41.

A rare case of nasopharyngeal angiofibroma in a

pregnant w o m a n LOUIS Pf:LOQUIN, MD, FRCSC,JEAN-MICHEL KLOSSEK,MD, FRf:Df:RICBASSO-BRUSA,MD, JEAN-MICHEL GOUGEON, MD, PAULH. TOFFEL,MD, FACS,and JEAN-PIERREFONTANEL,MD, Poitiers, France, and Glendale, California

N a s o p h a r y n g e a l angiofibromas account for less than 0.5% of all head and neck tumors. 1,2 Their cause remains unknown. A striking male distribution during or after adolescence has led to conclusions of a possible hormonal cause. Much research has been performed on hormonal receptors confined to the tumor. Johns et al. 3 observed in six patients a reduction of the tumoral mass with estrogenal therapy. Testosterone therapy was also associated with the enlargement of tumors. 4 The literature reports only a few female cases of angiofibroma. 2,5,6 Women are thought to be protected by their high estrogen levels. To our knowledge we present the first reported case of nasopharyngeal angiofibroma in a pregnant woman.

CASE REPORT A 31-year-old woman of Caribbean origin consulted in her twenty-sixlih week of gestation at our outpatient clinic in May 1994. She had right recurrent epistaxis. She had had two previous voluntary abortions before the twelfth week of gestation in 1989 and 1992. She had a well-controlled ankytosing spondylarthritis and had undergone surgery for a benign thyroid nodule in 1989. Initial examination of the nasal fossae revealed diffuse bilateral inflammation that was attributed to a recent episode of upper respiratory tract infection. Regular recommendations were given, and an emollient ointment was prescribed. Two weeks later she consulted at the emergency depart-

From the Depm'tment of Otolaryngology-Head and Neck Surgery (Drs. P~loquin, Klossek, Basso-Brusa, Gougeon, and Fontanel), Centre Hospitalier Universitaire Jean-Bernard, University of Poitiers, and the Department of Otolaryngology-Head and Neck Surgery (Dr. Toffel), University of Southern California School of Medicine, Glendale. Reprint requests: Louis P61oquin, MD, FRCSC, 161 Chester Ave., Town of Mount-Royal, Quebec, H3R 1W1 Canada. Ototaryngol Head Neck Surg 1997; 117:S 111-S 114. Copyright © 1997 by the American Academy of OtolaryngologyHead and Neck Surgery Foundation, Inc. 0194-5998/97/$5.00 + 0 23/4/'74552

ment for persistent Needing on the right side. Arterial pressure was 145/80 on admission. Gynecologic examination was normal. A simple nasal packing with Vaseline-impregnated gauze controlled the bleeding. The patient reported no such bleeding before the current pregnancy. Anterior rhinoscopy showed persisting bilateral hypertrophic rhinitis with a severe serous rhinorrhea. A hormonal rhinitis associated with the pregnancy was the most credible cause for the chronic swelling. A topical beclomethasone regimen was begun. A few weeks later the patient consulted again at the emergency department for recurrent nasal epistaxis on the fight side associated with intense right facial pain. Endoscopic examination with the patient under local anesthesia showed a lobulated grayish-reddish mass in the posterior part of the right nasal fossa. A malignancy was first suspected because of the painful presentation and the evolution of the disease. A C T scan was obtained without contrast agent because the patient was pregnant. It showed an expanding mass in the posterior part of the right nasal fossa (Fig. 1). The mass bulged into the right maxillary sinus and seemed to expand from the right to the left nasal fossa. Osteolysis was suspected at the medial wall of the maxillary sinus, the right inferior turbinate, and the nasal septum. Biopsy specimens were taken with the patient under local anesthesia. A diagnosis of possible rhabdomyosarcoma was suspected from small fragments taken superficially in the tumoral bed. A decision was made by a multidisciplinary committee to clarify the diagnosis with more biopsies performed with the patient under general anesthesia. New biopsy specimens now showed an angiofibroma. Specimens were also sent to the Gustave-Roussy Institute in Paris for a second opinion. A fasciculated fibroblastic proliferation was identified with primitive capillaries and frequent interstitial hemorrhages (Fig. 2). A positive reaction was found at immunohistochemistry with the actine marker for smooth muscle fibers and the vimentine marker for fibroblastic growth. The presence of a nasopharyngeal angiofibroma in a pregnant woman was confirmed. At 33 weeks her baby was delivered by a cesarean section. We followed the tumoral evolution in the postpartum peri-

$112 PI~LOQUINetal.

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Fig. 1. Axial cut on CT showing mass in posterior part of right nasal cavity.

Fig. 2. Paraffin section showing fasciculated fibroblastic proliferations with individualized primitive capillaries and frequent interstitial hemorrhages, (Hematoxilin-eosin-Safran; original magnification x 125,)

OtolaryngologyHead and Neck Surgery Volume 117 Number 6

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Fig. 3. Tvweighted paramidline lateral section with gadolinium showing high signal intensity of tumor in inferior part of ethmoid bulging down to floor of nasal fossa.

Fig. 4. Preoperative angiography of right primitive carotid showing endonasal hypervascularization with nourishing branches originating from internal carotid artery.

od. Six weeks after birth the patient still reported right nasal obstruction associated with painful recurrent headaches. No sign of regression of the tumor was found at endoscopy. MRI showed a large tumor enhancing on the Tl-weighted sections with gadolinium. It protruded from the right ethmoid to the right maxillary sinus and compressed the nasal septum (Fig. 3). We therefore decided to operate on the patient. Angiography 1 week before surgery showed a pathognomonic hypervascularization associated with an angiofibroma (Fig. 4). Nourishing vessels from the internal maxillary artery were easily embolized. The degloving approach was performed with a sublabial incision of the superior lip. Enbloc excision of the medial wall of the right maxillary sinus was undertaken on September 29 with an inferior turbinectomy and a partial ethmoidectomy. Frozen sections taken during surgery showed no microscopic infiltration of the maxillary sinus mucosa. A moderate amount of inflammatory adhesions was found at the inferior turbinate and in the mucosa that covered the lateral wall of the nasal fossa. The tumor had a pedicle on the inferoposterior portion of the right middle turbinate near the sphenopalatine foramen. The nasal septum was left intact because it was not invaded by the tumor. Endoscopic and radiologic follow-up examinations showed no trace of tumor after 12 months.

150 and 38 cases, respectively, reported no female patient. Gruber et al. 9 reviewed the literature in 1985 and found only 28 female cases. Juvenile nasopharyngeal angiofibroma was mostly diagnosed in male teenagers with a mean age of l 5 years. 1,6,1°'I1 The minimal recorded age was 7 years. 7 The small number of patients older than 30 years confirms that a presentation after this age is exceptional. 6' 12-14 Our 31-yearold pregnant woman is thus a very exceptional case. History and clinical examination are usually the keys for the diagnosis of a juvenile angiofibroma. We met an unusual presentation. In such cases histopathologic examination of the tumor is often a difficult task. Diagnosis seems even more challenging during pregnancy. Tissues are then diffusely infiltrated with an edematous stroma, and much vasodilation is found throughout the specimen. 15 We faced a tumor that had little chance by its presentation to be an angiofibroma. The CT suggested the possibility of a malignant expanding tumor with associated bony lysis. Iodine contrast agents could not be used in our pregnant patient. The first biopsy specimens strongly suggested the presence of a rhabdomyosarcoma. This diagnosis in a pregnant woman had to be confirmed with larger biopsy specimens a second time before any cesarean section was performed. The cause of angiofibroma remains unknown. A hormonal theory suggests that high estrogen levels protect females. 1,3,4 This could explain the small number of reported female cases in the literature. Our patient presented with an m~giofibroma during pregnancy at a period in her life when estrogen and progesterone are at their highest levels. The tumor did not regress after delivery. This presentation favors the possibility of other contributing factors of c o n -

DISCUSSION To our knowledge the diagnosis of nasopharyngeal angiofibroma in a pregnant woman has never been reported in the literature. Neel et al. 7 and Conley et al. 8 with series of

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genital or inflammatory origins in the pathogenesis of the nasopharyngeal angiofibroma. 9.

REFERENCES 1. Bremer JW, Neel HB, De Santo LW, Jones GC. Angiofibroma: treatment trends in 150 patients during 40 years. Laryngoscope I986;96:1321-9. 2. Ewing JA, Shively EH. Angiofibroma: a rare case in an elderly female. Otolaryngol Head Neck Surg 1981 ;89:602-3. 3. Johns ME, Mac Leod RM, CantreI1 RW. Estrogen receptors in nasopharyngeal angiofibromas. Laryngoscope 1980;90:628-34. 4. Johnson S, Kloster JH, Schiff M. The action of hormones on juvenile nasopharyngeal angiofibroma. Acta Otolaryngol (Stockh) 1966;61:153-60. 5. Osborn DA, Sokolovski A. Juvenile angiofibroma in a female. Arch Otolaryngol 1965;82:626-32. 6. Parchet V. L'angiofibrome naso-pharyngien chez Ia femme. Ann Otol Laryngol (Paris) 1951;68(1):60-9. 7. Neel HB, Whicker JH, Devine KD, Weiland LH. Juvenile angiofibroma: review of 120 cases. Surgery 1973; 126:547-56. 8. Conley J, Healy WV, Blaugrund SM, Perzin KH. Nase-

10. 11. 12. 13. 14. 15.

pharyngeal angiofibroma in the juvenile. Surg Gynecol Obstet 1968;126:825-37. Gruber B, Kron TK, Goldman ME, Matz GJ. Nasopharyngeal angiofibroma in two young children. Otolaryngol Head Neck Surg 1985;93(6):803-6. Briant TDR, Fitzpatrick PJ, Berman J. Nasopharyngeal angiofibroma: a twenty year study. Laryngoscope 1978;88:1247-51. Bourguet J, Bourdini6re J, Carsin J, Le Clech G, Cornec J, Coudray C. Apropos de 30 fibromes naso-pharyngiens. Ann OtoI Laryngol (Paris) 1980;97(4):295-303. Economou TS, Abemayor E, Ward PH. Juvenile nasopharyngeal angiofibroma: an update of the UCLA experience, 1960-1985. Laryngoscope 1988;98:170-5. Gullane PJ, Davidson J, O'Dwyer T, Forte V. Juvenile angiofibroma: a review of the literature and a case series report. Laryngoscope 1992;102:928-33. Jacobsson M, Petruson B, Svendsen E Berthelsen B. Juvenile nasopharyngeal angiofibroma: a report of eighteen cases. Arch Otolaryngol 1988;105:132-9. Chomette G, Gompel A, Malet A, Tranbaloc P, Kuttenn F, Mauvais-Jarvis E Les cellules humaines du sein normal en culture. Arch Anat Cytol Pathol 1985;33(1):32-8.

Complete facial paralysis as a result of parotid abscess DARRELL R. SMITH, MD, and GREGORY K. HARTIG, MD, Madison, Wisconsin

F a c i a l paralysis associated with parotid disease is usually caused by a malignant neoplasm. Benign parotid neoplasms and inflammatory processes resulting in facial paralysis are rare. Facial nerve dysfunction as a result of parotid infection is exceedingly rare, with 14 cases reported previously in the world literature. We present a case of acute suppurative parotitis with early abscess formation resulting in complete facial nerve paralysis. The diagnosis, bacteriology, management, and prognosis for recovery of facial nerve function will be reviewed.

CASE REPORT A 68-year-old woman was seen with a 3-day history of progressive left-sided facial pain, swelling, and dysphagia to solid foods. She reported chills but no fever. She denied any recent upper respiratory infection, dehydration, history of parotitis, parotid mass, facial nerve dysfunction, hemifacial

From the Division of Otolaryngology-Head and Neck Surgery, the University of Wisconsin School of Medicine. Presented at the Annual Meeting of the American Academy of Otolaryngology-Head and Neck Surgery Foundation, New Orleans, La., Sept. 17-20, 1995. Reprint requests: Darrell R. Smith, MD, Division of Otolaryngology-Head and Neck Surgery, H4/318, University of Wisconsin Hospital and Clinics, 600 Highland Ave., Madison, WI 53792-3236. Otolaryngol Head Neck Surg 1997;117:S 114-S 117. Copyright © 1997 by the American Academy of OtolaryngologyHead and Neck Surgery Foundation, Inc. 0194-5998/97/$5.00 + 0 23/4/74531

spasm, or pain. The physical examination was significant for a temperature of 37.7 ° C, severe cellulitis, swelling, and induration of the left side of the face and neck, purulent drainage from the left Stensen's duct, a left external auditory canal that was mildly edematous with a normal tympanic membrane, no lymphadenopathy, and no focal neurologic findings. The laboratory data were remarkable for a white blood cell count of 19,200 cells/gl, blood urea nitrogen/creatinine level of 11 mg/dl, 1.0 mg/dl, a urine specific gravity of 1.020, and urine osmolality of 675 mOsm/L. She was admitted to the hospital and given intravenous nafcillin and oral metronidazole. During the next 8 hours, she noted a complete left-sided facial paralysis (Fig. 1). A C T scan confirmed the presence of a left parotid phlegmon with early parotid abscess formation (Fig. 2). The patient was taken to the operating room for incision and drainage of the parotid abscess. At the time of surgery, multiple loculated collections of purulent discharge and necrotic parotid tissue were identified and drained with multiple radial incisions into the gland along the direction of the facial nerve fibers (Fig. 3). Intraoperative cultures were obtained that grew Staphylococcus aureus. After surgery, intravenous antibiotics were continued with gradual resolution of the facial swelling and induration. A repeat CT scan on postoperative day 2 showed no residual abscess. She was discharged on postoperative day 9 with a completely resolved infection and clean wound. Her facial nerve remained weak with only minimal platysmal branch function returning. Discharge medications included amoxicillin/clavulanic acid, a Medrol dose pack (methylprednisolone), and Lacri-Lube for corneal protection.