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Mucocele of the ethmoid sinus
International Journal of Pediatric Otorhinolaryngology, 10 (1985) 81-86
81
Elsevier POR 00296
Case Reports
M u c o c e l e of the e t h m o i d sinus * Stuart G. Selkin Dioision of Otorhinolarvngology, Massapequa General Hospital, Seaford, N Y (U.S.A.)
(Received October 29th, 1984) (Accepted January 30th, 1985)
Key words: mucocele - ethmoid - pseudotumor - orbit
Summary A 15-year-old female was evaluated for pain in the region of the right medial canthus and increasing exophthalmos. She had been diagnosed as having pseudot u m o r of the right orbit when she was 8 years old. The diagnosis was sustained until a second ophtalmologist requested a C T scan which demonstrated a mucocele of the right ethmoid sinus. Intranasal endoscopy revealed medial bulging of the right middle turbinate and meatus. Following a right external ethmoidectomy the eye returned to its normal position and pain disappeared. She has been asymptomatic for the past 4 years.
Introduction A 15-year-old Caucasian female presented to her ophthalmologist with a 6-month history of pain in the region of the right medial canthus. N o visual difficulties were apparent. Seven years earlier the same ophthalmologist had made a diagnosis of pseudotumor of the right orbit based on a clinical history of right retro-orbital pain, and clinical findings of a hyperemic disk and a borderline degree of exophthalmos on the same side, a normal neurological evaluation, and normal X-rays of the orbits and paranasal sinuses. A minor degree of exophthalmos of the right eye was documented at the currently described examination (when she was 15). The disk was hyperemic; visual acuity was normal. The diagnosis of pseudotumor of the orbit was unchanged. N o further * Presented at: Society of Ear, Nose, and Throat Advances in Children Annual Meeting, November 2, 1984, New Orleans, LA, U.S.A. Correspondence: S.G. Selkin, 100 Manetto Hill Rd., Plainview, NY 11803, U.S.A. 0165-5876/85/$03.30 © 1985 Elsevier Science Publishers B.V. (Biomedical Division)
82
Fig. 1. CT scan showing expanding mass (mucocele) of right ethmoid sinus. Fig. 2. Patient showing exophthalmos of right eye.
investigations or treatments were d o n e over the next 6 m o n t h s despite the observation by both patient and physician of an increasing a m o u n t of e x o p h t h a l m o s on the right and despite increasing subjective complaints of pain on the same side. A second ophthalmologist became concerned over the increasing pain and exophthalmos and referred her for a C T scan. A mucocele o f the right e t h m o i d sinus was clearly demonstrated. (Fig. 1) The optic nerve on that side was intact. She was referred to the author for otolaryngologic evaluation.
Fig. 3. lntranasal endoscopic view (pre-operative) showing medial bulging of middle turbinate and middle meatus. Fig. 4. Intranasal endoscopic view (pre-operative) showing medial bulging of middle turbinate and middle meatus. Different angle from Fig. 3. Fig. 5. Intranasal endoscopic view (intra-operative) showing mucocele and early stage of creation of ethmoidectomy. Fig. 6. Intranasal endoscopic view (3 months post-operative) showing healed intranasal ethmoidectomy cavity with inspissated mucus. Fig. 7. Intranasal endoscopic view (3 months post-operative) showing healed intranasal ethmoidectomy cavity.
83
Case Report Examination of the face showed a mild degree of exophthalmos on the right side (Fig. 2).
3
4
5
6
7
84 Intranasal endoscopy with a flexible fiberoptic nasopharyngolaryngoscope revealed medial bulging of the right middle turbinate and right middle meatus. (Figs. 3, 4) There was no discharge of mucus or of pus into any of the sinus ostia. A small amount of adenoid tissue was noted in the nasopharynx. There was no evidence of neoplasm in the upper aerodigestive tract. She underwent exploration of the right ethmoidal sinus via an external (Lynch) approach. A mucocele was found which had destroyed the trabeculations of the ethmoid sinus and which had eroded into the floor of the frontal sinus. The remaining walls of the frontal sinus were intact. A right external ethmoidectomy was completed. A posteriorly based mucosal flap was created and a partial resection of the ipsilateral middle turbinate was done in to permit wide drainage into the nose. Cultures of the fluid within the mucocele were negative for aerobic and anerobic bacteria and fungi; histologic examination of the fluid showed it to be filled with blood and eosinophilic mucoid material. She was discharged from the hospital on the second post-operative day. All eye pain disappeared within the next 7 days and the right eye returned to its normal position within the next 2 months. She has been asymptomatic for the past 4 years.
Discussion
Orbital pseudotumor is a heterogeneous diagnostic category of lymphoid infiltrations of the orbit with a wide spectrum of pathologic conditions and locations within the orbit [1]. Initially it mimics an orbital neoplasm. Involvement of the other orbit is uncommon and extension into the paranasal sinuses is rare. Only 1 case of intracranial extension has been reported [4]. It is rare, but not unknown in children [5]. It develops over weeks or months and is manifested by orbital pain, ophthalmoplegia, lid swelling, ptosis, diplopia, uveitis, exophthalmos, a mass in or near the orbit, and optic neuropathy [2]. Management with steroids is usually effective, but a significant number will require radiation therapy [6,7]. Although this was the working diagnosis in the present case for almost 8 years, the intra-operative findings suggest that the diagnosis was in error. Mucoceles of the paranasal sinuses result from the accumulation of secreted mucus and desquamated epithelium enclosed within the secretory respiratory membrane of an obstructed sinus. Enlargement of a mucocele will erode and destroy adjacent bone, allowing the mucocele to extend into adjacent sinuses [8]. The frontal sinus is the most common location for a mucocele (65%); the ethmoid sinus is next most common (30%); the maxillary and sphenoid sinuses contain the remainder [9]. Signs and symptoms of mucoceles of the frontal and ethmoid sinuses are often identical to those of pseudotumor of the orbit. Extension into the nose will result in rhinorrhea and nasal obstruction on the involved side. Sphenoid sinus involvement may result in a superior orbital fissure syndrome [2]. A mucocele may become infected. The resulting pyocele is likely to be associated with fever and increasing pain.
85 R a d i o l o g i c findings consist of o p a c i f i c a t i o n of the sinus cavity by a h o m o g e n e o u s soft tissue m a s s with e x p a n s i o n , thinning, or erosion of the b o n y wall. F l u i d a c c u m u l a t i o n in the mucocele m a y result in increased o p a c i f i c a t i o n of the sinus cavity. If b o n e d e s t r u c t i o n exceeds the increase in fluid, the c a v i t y will a p p e a r to be more radiolucent. T'he c o n t e n t s o f a m u c o c e l e v a r y from thick m u c o i d secretions to a t e n a c i o u s g e l a t i n o u s type. C o l o r m a y be yellow, b r o w n , gray or red. D e s q u a m a t e d epithelial cells a n d lipid c o n t a i n i n g m a c r o p h a g e s m a y be f o u n d in the fluid. T h e c o n t e n t s of the m u c o c e l e are u s u a l l y sterile; p u r u l e n t e x u d a t e is seen in the pyocele. T h e sac consists of a fibrous s t r o m a with a lining of low c u b o i d a l or stratified c o l u m n a r r e s p i r a t o r y e p i t h e l i u m . C h r o n i c i n f l a m m a t i o n m a y i n d u c e s q u a m o u s m e t a p l a s i a [2]. T r e a t m e n t is always surgical a n d requires c o m p l e t e e x e n t e r a t i o n of d i s e a s e d m u c o s a plus c r e a t i o n of a d e q u a t e d r a i n a g e into the nose.
References 1 Abramovitz, J.N., Kasdon, D.L., Sutula, F., Post, K.D. and Chong, F.K., Sclerosing orbital pseudotumor, Neurosurgery, 12 (1983) 463-468. 2 Batsakis, J.G., Tumors of the Head and Neck, Williams and Wilkins, Baltimore, 1979, p. 523. 3 Eshaghian, ,i. and Anderson, R.L., Sinus involvement in sinus pseudotumor, Arch. Ophthalmol.. 99 (1981) 627-630. 4 Kaye, A.H., Hahn, J.F., Cracium, A., Hanson, M., Berlin, A.,I. and Tubbs, R.R., Intracranial extension of inflammatory pseudotumor of the orbit. Case Report, J. Neurosurg., 60 (1984) 625-629. 5 Mottow-Lippa, L., Jakobiec F.A. and Smith, M., Idiopathic inflammatory orbital pseudotumor in childhood, Ophthalmology, 88 (1981) 565-574. 6 Kennerdel, ,I.S., Johnson, B.L. and Deutsch, M., Radiation treatment of orbital lymphoid hyperplasia, Ophthalmology, 86 (1979) 942-947. 7 Sergott, R.C., Glaser, .I.S. and Charylu, K., Radiotherapy for idiopathic inflammatory pseudotumor. Indications and results, Arch. Ophthalmol,, 99 (1981) 853-856. 8 Wilson, W.R. and Nadol, J.B., Quick Reference to Ear, Nose, and Throat Disorders, Lippincott. Philadelphia, 1983, pp. 268-269. 9 Zizmor, J. and Noyek, A.M.. Cysts and benign tumors of the paranasal sinuses, Semin. Roentgenol., 3 (1968) 172-174.
81
Elsevier POR 00296
Case Reports
M u c o c e l e of the e t h m o i d sinus * Stuart G. Selkin Dioision of Otorhinolarvngology, Massapequa General Hospital, Seaford, N Y (U.S.A.)
(Received October 29th, 1984) (Accepted January 30th, 1985)
Key words: mucocele - ethmoid - pseudotumor - orbit
Summary A 15-year-old female was evaluated for pain in the region of the right medial canthus and increasing exophthalmos. She had been diagnosed as having pseudot u m o r of the right orbit when she was 8 years old. The diagnosis was sustained until a second ophtalmologist requested a C T scan which demonstrated a mucocele of the right ethmoid sinus. Intranasal endoscopy revealed medial bulging of the right middle turbinate and meatus. Following a right external ethmoidectomy the eye returned to its normal position and pain disappeared. She has been asymptomatic for the past 4 years.
Introduction A 15-year-old Caucasian female presented to her ophthalmologist with a 6-month history of pain in the region of the right medial canthus. N o visual difficulties were apparent. Seven years earlier the same ophthalmologist had made a diagnosis of pseudotumor of the right orbit based on a clinical history of right retro-orbital pain, and clinical findings of a hyperemic disk and a borderline degree of exophthalmos on the same side, a normal neurological evaluation, and normal X-rays of the orbits and paranasal sinuses. A minor degree of exophthalmos of the right eye was documented at the currently described examination (when she was 15). The disk was hyperemic; visual acuity was normal. The diagnosis of pseudotumor of the orbit was unchanged. N o further * Presented at: Society of Ear, Nose, and Throat Advances in Children Annual Meeting, November 2, 1984, New Orleans, LA, U.S.A. Correspondence: S.G. Selkin, 100 Manetto Hill Rd., Plainview, NY 11803, U.S.A. 0165-5876/85/$03.30 © 1985 Elsevier Science Publishers B.V. (Biomedical Division)
82
Fig. 1. CT scan showing expanding mass (mucocele) of right ethmoid sinus. Fig. 2. Patient showing exophthalmos of right eye.
investigations or treatments were d o n e over the next 6 m o n t h s despite the observation by both patient and physician of an increasing a m o u n t of e x o p h t h a l m o s on the right and despite increasing subjective complaints of pain on the same side. A second ophthalmologist became concerned over the increasing pain and exophthalmos and referred her for a C T scan. A mucocele o f the right e t h m o i d sinus was clearly demonstrated. (Fig. 1) The optic nerve on that side was intact. She was referred to the author for otolaryngologic evaluation.
Fig. 3. lntranasal endoscopic view (pre-operative) showing medial bulging of middle turbinate and middle meatus. Fig. 4. Intranasal endoscopic view (pre-operative) showing medial bulging of middle turbinate and middle meatus. Different angle from Fig. 3. Fig. 5. Intranasal endoscopic view (intra-operative) showing mucocele and early stage of creation of ethmoidectomy. Fig. 6. Intranasal endoscopic view (3 months post-operative) showing healed intranasal ethmoidectomy cavity with inspissated mucus. Fig. 7. Intranasal endoscopic view (3 months post-operative) showing healed intranasal ethmoidectomy cavity.
83
Case Report Examination of the face showed a mild degree of exophthalmos on the right side (Fig. 2).
3
4
5
6
7
84 Intranasal endoscopy with a flexible fiberoptic nasopharyngolaryngoscope revealed medial bulging of the right middle turbinate and right middle meatus. (Figs. 3, 4) There was no discharge of mucus or of pus into any of the sinus ostia. A small amount of adenoid tissue was noted in the nasopharynx. There was no evidence of neoplasm in the upper aerodigestive tract. She underwent exploration of the right ethmoidal sinus via an external (Lynch) approach. A mucocele was found which had destroyed the trabeculations of the ethmoid sinus and which had eroded into the floor of the frontal sinus. The remaining walls of the frontal sinus were intact. A right external ethmoidectomy was completed. A posteriorly based mucosal flap was created and a partial resection of the ipsilateral middle turbinate was done in to permit wide drainage into the nose. Cultures of the fluid within the mucocele were negative for aerobic and anerobic bacteria and fungi; histologic examination of the fluid showed it to be filled with blood and eosinophilic mucoid material. She was discharged from the hospital on the second post-operative day. All eye pain disappeared within the next 7 days and the right eye returned to its normal position within the next 2 months. She has been asymptomatic for the past 4 years.
Discussion
Orbital pseudotumor is a heterogeneous diagnostic category of lymphoid infiltrations of the orbit with a wide spectrum of pathologic conditions and locations within the orbit [1]. Initially it mimics an orbital neoplasm. Involvement of the other orbit is uncommon and extension into the paranasal sinuses is rare. Only 1 case of intracranial extension has been reported [4]. It is rare, but not unknown in children [5]. It develops over weeks or months and is manifested by orbital pain, ophthalmoplegia, lid swelling, ptosis, diplopia, uveitis, exophthalmos, a mass in or near the orbit, and optic neuropathy [2]. Management with steroids is usually effective, but a significant number will require radiation therapy [6,7]. Although this was the working diagnosis in the present case for almost 8 years, the intra-operative findings suggest that the diagnosis was in error. Mucoceles of the paranasal sinuses result from the accumulation of secreted mucus and desquamated epithelium enclosed within the secretory respiratory membrane of an obstructed sinus. Enlargement of a mucocele will erode and destroy adjacent bone, allowing the mucocele to extend into adjacent sinuses [8]. The frontal sinus is the most common location for a mucocele (65%); the ethmoid sinus is next most common (30%); the maxillary and sphenoid sinuses contain the remainder [9]. Signs and symptoms of mucoceles of the frontal and ethmoid sinuses are often identical to those of pseudotumor of the orbit. Extension into the nose will result in rhinorrhea and nasal obstruction on the involved side. Sphenoid sinus involvement may result in a superior orbital fissure syndrome [2]. A mucocele may become infected. The resulting pyocele is likely to be associated with fever and increasing pain.
85 R a d i o l o g i c findings consist of o p a c i f i c a t i o n of the sinus cavity by a h o m o g e n e o u s soft tissue m a s s with e x p a n s i o n , thinning, or erosion of the b o n y wall. F l u i d a c c u m u l a t i o n in the mucocele m a y result in increased o p a c i f i c a t i o n of the sinus cavity. If b o n e d e s t r u c t i o n exceeds the increase in fluid, the c a v i t y will a p p e a r to be more radiolucent. T'he c o n t e n t s o f a m u c o c e l e v a r y from thick m u c o i d secretions to a t e n a c i o u s g e l a t i n o u s type. C o l o r m a y be yellow, b r o w n , gray or red. D e s q u a m a t e d epithelial cells a n d lipid c o n t a i n i n g m a c r o p h a g e s m a y be f o u n d in the fluid. T h e c o n t e n t s of the m u c o c e l e are u s u a l l y sterile; p u r u l e n t e x u d a t e is seen in the pyocele. T h e sac consists of a fibrous s t r o m a with a lining of low c u b o i d a l or stratified c o l u m n a r r e s p i r a t o r y e p i t h e l i u m . C h r o n i c i n f l a m m a t i o n m a y i n d u c e s q u a m o u s m e t a p l a s i a [2]. T r e a t m e n t is always surgical a n d requires c o m p l e t e e x e n t e r a t i o n of d i s e a s e d m u c o s a plus c r e a t i o n of a d e q u a t e d r a i n a g e into the nose.
References 1 Abramovitz, J.N., Kasdon, D.L., Sutula, F., Post, K.D. and Chong, F.K., Sclerosing orbital pseudotumor, Neurosurgery, 12 (1983) 463-468. 2 Batsakis, J.G., Tumors of the Head and Neck, Williams and Wilkins, Baltimore, 1979, p. 523. 3 Eshaghian, ,i. and Anderson, R.L., Sinus involvement in sinus pseudotumor, Arch. Ophthalmol.. 99 (1981) 627-630. 4 Kaye, A.H., Hahn, J.F., Cracium, A., Hanson, M., Berlin, A.,I. and Tubbs, R.R., Intracranial extension of inflammatory pseudotumor of the orbit. Case Report, J. Neurosurg., 60 (1984) 625-629. 5 Mottow-Lippa, L., Jakobiec F.A. and Smith, M., Idiopathic inflammatory orbital pseudotumor in childhood, Ophthalmology, 88 (1981) 565-574. 6 Kennerdel, ,I.S., Johnson, B.L. and Deutsch, M., Radiation treatment of orbital lymphoid hyperplasia, Ophthalmology, 86 (1979) 942-947. 7 Sergott, R.C., Glaser, .I.S. and Charylu, K., Radiotherapy for idiopathic inflammatory pseudotumor. Indications and results, Arch. Ophthalmol,, 99 (1981) 853-856. 8 Wilson, W.R. and Nadol, J.B., Quick Reference to Ear, Nose, and Throat Disorders, Lippincott. Philadelphia, 1983, pp. 268-269. 9 Zizmor, J. and Noyek, A.M.. Cysts and benign tumors of the paranasal sinuses, Semin. Roentgenol., 3 (1968) 172-174.