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Deep Orbital Dermoid with Draining Sinus
D E E P ORBITAL DERMOID W I T H DRAINING SINUS ZANE F. POLLARD, M.D.,
AND JOSEPH CALHOUN,
M.D.
Philadelphia, Pennsylvania
Dermoid cysts located deep within the orbit are rare. They more frequently appear in the upper-outer quadrant anteriorly. This report documents a case of a dermoid ex tending in an anterior direction from the apex of the orbit and exiting through the lateral wall. It formed a draining sinus in the skin 3 cm from the anterolateral orbital mar gin. Dermoids connected with draining sinuses in other parts of the body have been reported, but only rarely in the orbital area. Our patient had proptosis and lateral rectus palsy produced by the dermoid.
tion of abduction of the left eye (Fig. 1) and the patient had a small face turn to the left. The ophthalmoscopic examination in each eye was normal. Pressure exerted over the left orbit produced a mucopurulent drainage from the sinus tract (Fig. 2 ) . No mass was palpable ; and x-ray films of the orbits, skull, and paranasal sinuses were normal. B-scan ultrasound was normal. Results of complete blood count and physical examination were normal. The proptosis had been present for three years, and all studies were normal. Therefore it was decided to observe her periodically as an outpatient. Three months later the sinus tract was injected with contrast material, and x-ray films were taken. The study was nondiagnostic, since the dye went in only a few millimeters from the skin. Because of persistent drainage, the patient was admitted to the hospital in March 1974, for an exploration of this draining sinus. A t this time the proptosis was iden tical as it had been six months preivously. The R.E. was 16 mm, and L.E., 20 mm with the base of 88 on the exophthalmometer. The lateral rectus palsy was essentially unchanged. The patient could abduct her left eye 10 to IS degrees past the mid-line. This was unchanged from previous examinations. The complete blood count and physical examination were normal. At this time the patient underwent an exploration of the sinus tract. The tract extended from the skin, 3 cm poserior to the lateral canthus and 1 cm above it, down to the zygomatic bone. A Krönlein orbitotomy was performed by using the Stryker saw to remove a piece of bone 1.5 X 2 cm rectangularly. The fibrous tract, after going through the bone, was continuous with a cystic structure. This was dis sected free from the orbital surface of the bone of the lateral wall. The cyst was traced to the apex of the orbit and measured 1 X 2 cm. It was dissected free from the periorbita lining the inner surface of
CASE REPORT A S-year-old black girl, first seen in the eye clinic at St. Christopher's Children's Hospital in August 1973, had proptosis of the left eye of three years' duration, according to the child's mother. She also had a draining sinus 3 cm posterior to the lateral canthus and 1 cm above it. The sinus was 3 mm wide, but there was an area of excoriated skin around it, encompassing an oval-shaped area with the longest dimension of 2.5 cm. Three years previously, the patient had been observed at another eye clinic for proptosis and a left lateral rectus palsy. It did not progress over a year's period, so a conservative ap proach of watching was recommended. The small draining area had been present since she was 2 years old. H e r mother thought it was a nonhealing sore. In June 1973, the surgery department of St. Chris topher's Hospital resected a small sinus tract in this area. They diagnosed it as a branchial cleft sinus. The pathology report showed only fibrous tissue, and no pathologic diagnosis was made. In July 1973, a possible pre-auricular cyst was excised from this area. It became infected and had to be incised and drained. The area was still draining when the child was examined in our clinic in August 1973. At that time her visual acuity was 20/25 in each eye. The pupils were equal and reactive, and there was no Marcus-Gunn pupil. With the Hertel exoph thalmometer the reading was R.E., 16 mm, and L.E., 20 mm at the base of 88. There was marked limitaFrom the Department of Ophthalmology, St. Christopher's Children's Hospital, Philadelphia, Pennsylvania. Dr. Pollard is a Heed Ophthalmic Fellow in Pediatrie Ophthalmology at the Wills Eye Hospital. Reprint requests to Zane F. Pollard, M.D., De partment of Pediatrie Ophthalmology, Wills Eye Hospital, 1601 Spring Garden St., Philadelphia, P A 19130.
310
Fig. 1 (Pollard and Calhoun). Proptosis of the left eye and lateral rectus palsy with bandage cover ing draining sinus.
VOL. 79, NO. 2
ORBITAL DERMOID
311
the zygomatic bone. (The cyst actually was located between the bone and the periorbita.) The periorbita was not incised. After the cyst was removed, the bone was replaced. Then the sinus tract and the excoriated skin around it were excised. The histopathology of the specimen was characteristic of a dermoid cyst. It showed a cystic lesion lined by strat ified squamous epithelium with hair follicles and sebaceous glands (Fig. 3). The sinus tract contained fibrous tissue with hair follicles and sebaceous glands. A part of the tract was lined with stratified squamous epithelium, and part contained no epi thelium at all. Two months postoperatively, visual acuity was L.E. : 20/25, but there was still a palsy of the left lateral rectus muscle. DISCUSSION
T h e most common locations for dermoids are the ovaries, sacral area, and testicles. An analysis of 1,495 dermoid cysts at the Mayo Clinic from 1910 to 1935 showed 4 2 % in the genital area, 4 5 % in the sacral area, and 7 % in the head and neck. 1 Of the dermoids in the head and neck, one half were in the orbital region; of these, 6 0 % were located in the outer third of the eyebrow. Only 10% of the dermoids of the orbital region were within the orbit, and all but one were lo cated in the upper outer quadrant. Mortada, 3 Mehra and Bannerji, 3 Pfeiffer and Nicholl 4 reported proptosis due to dermoids. T h e connection of a dermoid cyst to a draining sinus has been reported before. But this is more common in areas other than the orbit. Szaley and Bledsoe 5 reported a case of congenital dermoid cyst and fistula of the
Fig. 2 (Pollard and Calhoun). Pressure with fingers on left eye produces mucopurulent discharge from area of sinus tract.
Fig. 3 (Pollard and Calhoun). Dermoid cyst lined by stratified squamous epithelium. A hair shaft (arrow) and other adnexal appendages are present in the cyst wall ( X64). nose. This is a frequent place for the occur rence of a draining dermoid. I n 1967, Dayal and Hameed e reported a case of a dermoid 'n the upper inner angle of the orbit. It was anteriorly located and could be seen easily. It drained via a fistula on the tip of the nose. Borley 7 reported a case of a dermoid in the posterolateral part of the orbit. It drained via a fistula into the lower cul-de-sac. Pressure on the lower eyelid resulted in an oily secretion exuding from the opening of the fistula. Samuels 8 reported a case of a dermoid cyst of the orbit that formed a nar row duct and extended through a tiny chan nel in the lateral wall of the orbit. H e r e it formed a knapsack-shaped diverticulum under the temporalis muscle.
312
AMERICAN JOURNAL OF OPHTHALMOLOGY
Dermoids may appear between 2 and 50 years of age. However, the peak ages are from 3 to 10 years of age and again in the third and fourth decades.9 During the development of the bones of the cranium, the embryonic ectoderm and the periostium are in apposition at the definitive suture lines. Some feel that pieces of ecto derm are pinched off as the suture lines close. Then the ectoderm develops as the skin develops but in an ectopie location forming a dermoid cyst. This theory accounts for the choristomatous tissue in both bone and the orbit. It may extend from the orbit to the cranium, paranasal sinuses, or the temporal fossa. A dermoid cyst of the cranium or paranasal sinuses may also in volve the orbit. Lesions in the bones of the cranium or orbit can often be seen with x-ray films,10'11 but we did not detect any defect. Maybe preoperative tomograms would have shown us the defect in the lat eral wall of the orbit through which the sinus exited. Also, the release of pressure via a draining sinus probably prevented ob vious x-ray film changes. The diagnosis of a branchial cleft sinus at the time of the first surgery was incorrect. Arey12 showed that a lesion this far anterior to the ear cannot represent a branchial cleft cyst or fistula. The indications for dermoid surgery are often for diagnosis of a mass lesion. Often inflammatory signs or evidence of pressure on contiguous structures present reasons for exploration. The dermoid cyst can produce lesions of a lytic nature in bone. Pressure from the dermoid can result in fossa forma tion in the bone. (Fossa formation is the same as indentation of bone.) Dermoids often form sharply circumscribed lesions in bone with an increased density of the sur rounding bone. They usually have smooth margins. Jones 13 had a patient with a der moid cyst of the orbit with carcinomatous degeneration of a squamous cell type. The Krönlein lateral orbitotomy has long been performed for tumors located deep in
FEBRUARY, 1975
the orbit, especially on the lateral side.14'15 Care should be taken to excise the entire dermoid without perforating the sac. The sebaceous, oily material that occupies the lumen of the cyst is capable of inciting an inflammatory reaction in the soft tissues sur rounding the cyst. The surgeon should check for diverticula at the time of surgery. They can be a source of recurrence if not re moved.16 Moffatt17 reported a dermoid that had an hourglass constriction with a deeper portion connected to the more superficial part. This was similar to our patient who had a super ficial draining sinus tract that had been pre viously excised. The deeper dermoid cyst that was connected to this sinus tract, and located on the inner side of the zygomatic bone, had been missed until our surgical exploration. SUMMARY
A dermoid cyst in a 6-year-old girl, deep Avithin the orbit, extended from the apex, through the lateral wall, via a fistulous tract to the skin, forming a draining sinus. Both the deep location and the cutaneous drain age of the cyst are rare presentations of this tumor. We used a Krönlein orbitotomy to successfully remove the tumor without loss of vision. ACKNOWLEDGMENT
We thank Merlyn Rodriguez, M.D., for the photo graph of the pathologic specimen. REFERENCES
1. New, G. B., and Erich, J. B. : Dermoid cyst of head and neck. Surg. Gynecol. Obst. 65:48, 1937. 2. Mortada, A.: Dermoid cyst of great wing of sphenoid bone. Br. J. Ophthalmol. 54:131, 1970. 3. Mehra, M. S., and Bannerji, C : Enlargement of optic foramen due to dermoid cyst of orbit. Am. J. Ophthalmol. 60:931, 1965. 4. Pfeiffer, R., and Nicholl, R.: Dermoids and epidermoids of the orbit. Trans. Am. Ophthalmol. Soc. 46:218, 1948. 5. Szaley, G., and Bledsoe, R. : Congenital dermoid cyst and fistula of the nose. Am. T. Dis. Child. 3:392, 1972. 6. Dayal, Y., and Hameed, S. : Periorbital der moid. Am. J. Ophthalmol. 53:1013, 1962.
ORBITAL DERMOID
VOL. 79, NO. 2
7. Borley, W. E. : Dermoid cyst of the orbit. Am. J. Ophthalmol. 22:1355,1939. 8. Samuels, B. : Dermoid cysts of the orbit. Trans. Am. Ophthalmol. Soc. 34:226, 1936. 9. Henderson, T. W.: Orbital Tumors. Philadel phia, W. B. Saunders, 1973, pp. 86-96. 10. Pfeiffer, R.: Roentgenography of exophthalmos with notes on the roentgen ray in ophthalmol ogy pt. 1. Am. J. Ophthalmol. 26:724, 1943. 11. : Roentgenography of exophthalmos with notes on the roentgen ray in ophthalmology pt. 2. Am. J. Ophthalmol. 26:816, 1943. 12. Arey, L. : Developmental Anatomy, 6th ed.
313
Philadelphia, W. B. Saunders, 1960, p. 196. 13. Jones, A.: Oil cyst of the orbit with carcinomatosis. Am. J. Ophthalmol. 18:532, 1935. 14. Knapp, A. : Oil cyst of orbit. Arch. Ophthal mol. 52:163, 1923. 15. MacDonald, R., and Byers. J. : Dermoid tumor simulating a neoplasm. Am. J. Ophthalmol. 47:863, 1959. 16. Gifford, H.: Recurrent dermoid cyst. Arch. Ophthalmol. 2:305, 1929. 17. Moffatt, P.: An orbital dermoid (lined by skin and conjunctiva). Br. J. Ophthalmol. 25:428, 1941.
OPHTHALMIC MINIATURE
Beard (1905)
Schmidt-Rimpler (1889)
Schmidt-Rimpler
A. E. Ewing: Eye specula. Three new designs. Am. J. Ophthalmol., series 2, 33:33, 1916
AND JOSEPH CALHOUN,
M.D.
Philadelphia, Pennsylvania
Dermoid cysts located deep within the orbit are rare. They more frequently appear in the upper-outer quadrant anteriorly. This report documents a case of a dermoid ex tending in an anterior direction from the apex of the orbit and exiting through the lateral wall. It formed a draining sinus in the skin 3 cm from the anterolateral orbital mar gin. Dermoids connected with draining sinuses in other parts of the body have been reported, but only rarely in the orbital area. Our patient had proptosis and lateral rectus palsy produced by the dermoid.
tion of abduction of the left eye (Fig. 1) and the patient had a small face turn to the left. The ophthalmoscopic examination in each eye was normal. Pressure exerted over the left orbit produced a mucopurulent drainage from the sinus tract (Fig. 2 ) . No mass was palpable ; and x-ray films of the orbits, skull, and paranasal sinuses were normal. B-scan ultrasound was normal. Results of complete blood count and physical examination were normal. The proptosis had been present for three years, and all studies were normal. Therefore it was decided to observe her periodically as an outpatient. Three months later the sinus tract was injected with contrast material, and x-ray films were taken. The study was nondiagnostic, since the dye went in only a few millimeters from the skin. Because of persistent drainage, the patient was admitted to the hospital in March 1974, for an exploration of this draining sinus. A t this time the proptosis was iden tical as it had been six months preivously. The R.E. was 16 mm, and L.E., 20 mm with the base of 88 on the exophthalmometer. The lateral rectus palsy was essentially unchanged. The patient could abduct her left eye 10 to IS degrees past the mid-line. This was unchanged from previous examinations. The complete blood count and physical examination were normal. At this time the patient underwent an exploration of the sinus tract. The tract extended from the skin, 3 cm poserior to the lateral canthus and 1 cm above it, down to the zygomatic bone. A Krönlein orbitotomy was performed by using the Stryker saw to remove a piece of bone 1.5 X 2 cm rectangularly. The fibrous tract, after going through the bone, was continuous with a cystic structure. This was dis sected free from the orbital surface of the bone of the lateral wall. The cyst was traced to the apex of the orbit and measured 1 X 2 cm. It was dissected free from the periorbita lining the inner surface of
CASE REPORT A S-year-old black girl, first seen in the eye clinic at St. Christopher's Children's Hospital in August 1973, had proptosis of the left eye of three years' duration, according to the child's mother. She also had a draining sinus 3 cm posterior to the lateral canthus and 1 cm above it. The sinus was 3 mm wide, but there was an area of excoriated skin around it, encompassing an oval-shaped area with the longest dimension of 2.5 cm. Three years previously, the patient had been observed at another eye clinic for proptosis and a left lateral rectus palsy. It did not progress over a year's period, so a conservative ap proach of watching was recommended. The small draining area had been present since she was 2 years old. H e r mother thought it was a nonhealing sore. In June 1973, the surgery department of St. Chris topher's Hospital resected a small sinus tract in this area. They diagnosed it as a branchial cleft sinus. The pathology report showed only fibrous tissue, and no pathologic diagnosis was made. In July 1973, a possible pre-auricular cyst was excised from this area. It became infected and had to be incised and drained. The area was still draining when the child was examined in our clinic in August 1973. At that time her visual acuity was 20/25 in each eye. The pupils were equal and reactive, and there was no Marcus-Gunn pupil. With the Hertel exoph thalmometer the reading was R.E., 16 mm, and L.E., 20 mm at the base of 88. There was marked limitaFrom the Department of Ophthalmology, St. Christopher's Children's Hospital, Philadelphia, Pennsylvania. Dr. Pollard is a Heed Ophthalmic Fellow in Pediatrie Ophthalmology at the Wills Eye Hospital. Reprint requests to Zane F. Pollard, M.D., De partment of Pediatrie Ophthalmology, Wills Eye Hospital, 1601 Spring Garden St., Philadelphia, P A 19130.
310
Fig. 1 (Pollard and Calhoun). Proptosis of the left eye and lateral rectus palsy with bandage cover ing draining sinus.
VOL. 79, NO. 2
ORBITAL DERMOID
311
the zygomatic bone. (The cyst actually was located between the bone and the periorbita.) The periorbita was not incised. After the cyst was removed, the bone was replaced. Then the sinus tract and the excoriated skin around it were excised. The histopathology of the specimen was characteristic of a dermoid cyst. It showed a cystic lesion lined by strat ified squamous epithelium with hair follicles and sebaceous glands (Fig. 3). The sinus tract contained fibrous tissue with hair follicles and sebaceous glands. A part of the tract was lined with stratified squamous epithelium, and part contained no epi thelium at all. Two months postoperatively, visual acuity was L.E. : 20/25, but there was still a palsy of the left lateral rectus muscle. DISCUSSION
T h e most common locations for dermoids are the ovaries, sacral area, and testicles. An analysis of 1,495 dermoid cysts at the Mayo Clinic from 1910 to 1935 showed 4 2 % in the genital area, 4 5 % in the sacral area, and 7 % in the head and neck. 1 Of the dermoids in the head and neck, one half were in the orbital region; of these, 6 0 % were located in the outer third of the eyebrow. Only 10% of the dermoids of the orbital region were within the orbit, and all but one were lo cated in the upper outer quadrant. Mortada, 3 Mehra and Bannerji, 3 Pfeiffer and Nicholl 4 reported proptosis due to dermoids. T h e connection of a dermoid cyst to a draining sinus has been reported before. But this is more common in areas other than the orbit. Szaley and Bledsoe 5 reported a case of congenital dermoid cyst and fistula of the
Fig. 2 (Pollard and Calhoun). Pressure with fingers on left eye produces mucopurulent discharge from area of sinus tract.
Fig. 3 (Pollard and Calhoun). Dermoid cyst lined by stratified squamous epithelium. A hair shaft (arrow) and other adnexal appendages are present in the cyst wall ( X64). nose. This is a frequent place for the occur rence of a draining dermoid. I n 1967, Dayal and Hameed e reported a case of a dermoid 'n the upper inner angle of the orbit. It was anteriorly located and could be seen easily. It drained via a fistula on the tip of the nose. Borley 7 reported a case of a dermoid in the posterolateral part of the orbit. It drained via a fistula into the lower cul-de-sac. Pressure on the lower eyelid resulted in an oily secretion exuding from the opening of the fistula. Samuels 8 reported a case of a dermoid cyst of the orbit that formed a nar row duct and extended through a tiny chan nel in the lateral wall of the orbit. H e r e it formed a knapsack-shaped diverticulum under the temporalis muscle.
312
AMERICAN JOURNAL OF OPHTHALMOLOGY
Dermoids may appear between 2 and 50 years of age. However, the peak ages are from 3 to 10 years of age and again in the third and fourth decades.9 During the development of the bones of the cranium, the embryonic ectoderm and the periostium are in apposition at the definitive suture lines. Some feel that pieces of ecto derm are pinched off as the suture lines close. Then the ectoderm develops as the skin develops but in an ectopie location forming a dermoid cyst. This theory accounts for the choristomatous tissue in both bone and the orbit. It may extend from the orbit to the cranium, paranasal sinuses, or the temporal fossa. A dermoid cyst of the cranium or paranasal sinuses may also in volve the orbit. Lesions in the bones of the cranium or orbit can often be seen with x-ray films,10'11 but we did not detect any defect. Maybe preoperative tomograms would have shown us the defect in the lat eral wall of the orbit through which the sinus exited. Also, the release of pressure via a draining sinus probably prevented ob vious x-ray film changes. The diagnosis of a branchial cleft sinus at the time of the first surgery was incorrect. Arey12 showed that a lesion this far anterior to the ear cannot represent a branchial cleft cyst or fistula. The indications for dermoid surgery are often for diagnosis of a mass lesion. Often inflammatory signs or evidence of pressure on contiguous structures present reasons for exploration. The dermoid cyst can produce lesions of a lytic nature in bone. Pressure from the dermoid can result in fossa forma tion in the bone. (Fossa formation is the same as indentation of bone.) Dermoids often form sharply circumscribed lesions in bone with an increased density of the sur rounding bone. They usually have smooth margins. Jones 13 had a patient with a der moid cyst of the orbit with carcinomatous degeneration of a squamous cell type. The Krönlein lateral orbitotomy has long been performed for tumors located deep in
FEBRUARY, 1975
the orbit, especially on the lateral side.14'15 Care should be taken to excise the entire dermoid without perforating the sac. The sebaceous, oily material that occupies the lumen of the cyst is capable of inciting an inflammatory reaction in the soft tissues sur rounding the cyst. The surgeon should check for diverticula at the time of surgery. They can be a source of recurrence if not re moved.16 Moffatt17 reported a dermoid that had an hourglass constriction with a deeper portion connected to the more superficial part. This was similar to our patient who had a super ficial draining sinus tract that had been pre viously excised. The deeper dermoid cyst that was connected to this sinus tract, and located on the inner side of the zygomatic bone, had been missed until our surgical exploration. SUMMARY
A dermoid cyst in a 6-year-old girl, deep Avithin the orbit, extended from the apex, through the lateral wall, via a fistulous tract to the skin, forming a draining sinus. Both the deep location and the cutaneous drain age of the cyst are rare presentations of this tumor. We used a Krönlein orbitotomy to successfully remove the tumor without loss of vision. ACKNOWLEDGMENT
We thank Merlyn Rodriguez, M.D., for the photo graph of the pathologic specimen. REFERENCES
1. New, G. B., and Erich, J. B. : Dermoid cyst of head and neck. Surg. Gynecol. Obst. 65:48, 1937. 2. Mortada, A.: Dermoid cyst of great wing of sphenoid bone. Br. J. Ophthalmol. 54:131, 1970. 3. Mehra, M. S., and Bannerji, C : Enlargement of optic foramen due to dermoid cyst of orbit. Am. J. Ophthalmol. 60:931, 1965. 4. Pfeiffer, R., and Nicholl, R.: Dermoids and epidermoids of the orbit. Trans. Am. Ophthalmol. Soc. 46:218, 1948. 5. Szaley, G., and Bledsoe, R. : Congenital dermoid cyst and fistula of the nose. Am. T. Dis. Child. 3:392, 1972. 6. Dayal, Y., and Hameed, S. : Periorbital der moid. Am. J. Ophthalmol. 53:1013, 1962.
ORBITAL DERMOID
VOL. 79, NO. 2
7. Borley, W. E. : Dermoid cyst of the orbit. Am. J. Ophthalmol. 22:1355,1939. 8. Samuels, B. : Dermoid cysts of the orbit. Trans. Am. Ophthalmol. Soc. 34:226, 1936. 9. Henderson, T. W.: Orbital Tumors. Philadel phia, W. B. Saunders, 1973, pp. 86-96. 10. Pfeiffer, R.: Roentgenography of exophthalmos with notes on the roentgen ray in ophthalmol ogy pt. 1. Am. J. Ophthalmol. 26:724, 1943. 11. : Roentgenography of exophthalmos with notes on the roentgen ray in ophthalmology pt. 2. Am. J. Ophthalmol. 26:816, 1943. 12. Arey, L. : Developmental Anatomy, 6th ed.
313
Philadelphia, W. B. Saunders, 1960, p. 196. 13. Jones, A.: Oil cyst of the orbit with carcinomatosis. Am. J. Ophthalmol. 18:532, 1935. 14. Knapp, A. : Oil cyst of orbit. Arch. Ophthal mol. 52:163, 1923. 15. MacDonald, R., and Byers. J. : Dermoid tumor simulating a neoplasm. Am. J. Ophthalmol. 47:863, 1959. 16. Gifford, H.: Recurrent dermoid cyst. Arch. Ophthalmol. 2:305, 1929. 17. Moffatt, P.: An orbital dermoid (lined by skin and conjunctiva). Br. J. Ophthalmol. 25:428, 1941.
OPHTHALMIC MINIATURE
Beard (1905)
Schmidt-Rimpler (1889)
Schmidt-Rimpler
A. E. Ewing: Eye specula. Three new designs. Am. J. Ophthalmol., series 2, 33:33, 1916