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Intranasal Visualization for Nasolacrimal Duct Intubation
N i l ! MI U l i Fig. 1 (Santora, Biglan, and Johnson). Gross photograph of bean-shaped nodule composed of equal parts bone, left, and cartilage, right (scale in millimeters).
The specimen removed at the initial operation was a flat ovoid disk of firm tissue measuring 7 X 4.5 mm and less than 1 mm thick (Fig. 1). One half of the disk had a gray translucent appearance and the other half was pink, hard, and vascularized. Histopathologic examination disclosed well-formed lamellar bone with fat-filled medullary spaces adjacent to hyaline cartilage, which contained uniform chondrocytes (Fig. 2). Because of the combination of mesenchymal elements, the diagnosis of episcleral osteocartilaginous choristoma was made. The tissue removed at the subsequent operation consisted of fibroadipose and glandular tissue and was considered to be a dermolipoma containing lacrimal tissue. Bone formation occurs through the condensation of mesenchyme in one of two ways. Membranous bones form from mesenchymal condensation into a tough membrane from which bone develops.5 This process is characteristic of many bones of the skull. Elsewhere, bone originates from mesenchymal forma tion of a cartilage template.5 This template is sub sequently invaded by blood vessels and osteoblasts, and is replaced by bone.5 In 1962 Boniuk and Zimmerman2 concluded that episcleral osseous choristomas were composed of mature compact bone that was formed in a membranous fashion like orbital bone. Their conclusion was based on observations that there had been "no cartilage associated with any of the lesions with bone present."2
VOL 119, NO. 5
Fig. 2 (Santora, Biglan, and Johnson). Photomicrograph shows thick osseous trabeculae with medullary fat, left, adjacent to hyaline cartilage rich in mature chondro cytes, right (hematoxylin and eosin, X100).
Our study demonstrates two interesting features. First, our patient had a right hypertropia caused by an episcleral choristoma. Second, the choristoma was a combination of bone and cartilage. The combination of these mesenchymal elements implies lesion forma tion from a cartilage template instead of the previous ly suggested membranous fashion. REFERENCES 1. Beckman H, Sugar SH. Episcleral osseous choristoma: report of two cases. Arch Ophthalmol 1964;71:377-8. 2. Boniuk M, Zimmerman LE. Epibulbar osteoma (episcleral osseous choristoma). Am J Ophthalmol 1962;53:290-6. 3. Pittke EC, Marquardt R, Mohr W. Cartilage choristoma of the eye. Arch Ophthalmol 1983;101:1569-71. 4. Ferry AP, Hein HF. Epibulbar osseous choristoma within an epibulbar dermoid. Am J Ophthalmol 1970;70:764-6. 5. Hollinshead WH, Rosse C. Textbook of anatomy: the connec tive tissues, 4th ed. Philadelphia: Harper & Row, 1985:27.
Intranasal Visualization for Nasolacrimal Duct Intubation Allan M. Eisenbaum, M.D., Scott K. McClatchey, M.D., and Nigel R. T. Pashley, F.R.C.S.(C.) PURPOSE/METHODS: To improve intranasal vis ualization of nasolacrimal procedures we use a self-retaining nasal speculum with or without a sinuscope.
BRIEF REPORTS
655
RESULTS/CONCLUSIONS: Placement and retrie val of silicone stents can be accomplished with less trauma than with a blind sweep technique. Addi tionally, the valve of Hasner can be seen and the creation of false passages avoided.
N
ASOLACRIMAL DUCT INTUBATION FOR PEDIATRIC nasolacrimal duct obstruction is commonly done with silicone tubes attached to a metal rod as described by Quickert and Dryden 1 and by Crawford.2 These probes often are retrieved from beneath the inferior turbinate with a grooved director or hook by using a blind sweep technique. This approach to retrieval of the probes can result in excessive bleeding and trauma to the nasal mucosa and in the creation of false passages through the wall of the nose. We have found that the use of a self-retaining nasal speculum to move the inferior turbinate away from the lateral wall of the nose permits direct viewing and remarkably easy retrieval of the probes. Additionally, a sinuscope can be used with this speculum to give outstanding visualization of the valve of Hasner (Figure).3 We place cotton-tipped swabs soaked in 0.25% phenylephrine hydrochloride under the inferior turbi nate for vasoconstriction and use a headlamp for illumination. We have also used a zero-degree pédiat rie sinuscope, which permits monitoring or recording of the procedure. The speculum (a Cottle nasal speculum with a thumbscrew, V. Mueller, McGaw Park, Illinois) is inserted under the inferior turbinate with the blades closed. The speculum enters along the floor of the nose with the handle angled toward the midline, keeping the tapered blades between the lateral wall of the nose and inferior turbinate. The blades are opened slowly, displacing the inferior turbinate medially. Careful insertion of the speculum with a twisting motion prevents slippage of the blades.
Department of Ophthalmology, University of Colorado Health Scienc es Center and Children's Hospital (A.M.E.); Department of Ophthal mology, Children's National Medical Center (S.K.M.); and Presbyteri an/St. Luke's Medical Center (N.R.T.P.). The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the United States Navy or Department of Defense. Inquiries to Allan M. Eisenbaum, M.D., 14991 E. Hampden Ave., Suite 300, Aurora, CO 80014; fax: (303) 369-2031.
656
The blades are occasionally inserted too far posterior ly and must be pulled out slightly. W h e n proper exposure has been achieved the blades are locked open with the thumbscrew. The handle is pointed inferiorly away from the eye and rested on folded gauze pads. If there is any bleeding at this point a thin suction tip is used to remove the blood. The probes are then inserted into the nasolacrimal duct and removed with a hook or grooved director under direct visualization. We have used our technique for the past three years, and in every case we easily visualized and retrieved the probes in less time and with less bleeding than with the blind technique. In one instance we saw the probe poke through a membrane over the valve of Hasner while viewing the probe with the pédiatrie sinuscope. We occasionally saw the probe directed laterally into the mucosa of the wall of the nose and we were able to grasp and remove it. Had we been using a blind technique we would have been unable to feel the metal-on-metal tap with the probe retriever or would have caused excess bleeding if a Crawford hook (Jedmed Instrument Company, St. Louis, Missouri) were inserted. Additionally, because
Figure (Eisenbaum, McClatchey, and Pashley). Bowman probe beneath inferior turbinate through the valve of Hasner (arrow). Probe visualized with sinuscope.
AMERICAN JOURNAL OF OPHTHALMOLOGY
MAY
1995
we were able to see the probe intranasally, we never inserted the probe too far inferiorly. Our technique frees one hand of the surgeon and allows the surgeon to visualize directly Bowman's probes or remove a previously placed stent. Our technique also could be helpful in teaching the surgical anatomy of nasolacrimal duct intubation and removal, particularly when viewed by a sinuscope and seen on a video monitor. REFERENCES 1. Quickert MH, Dryden RM. Probes for intubation in lacrimal drainage. Trans A m Acad Ophthalmol Otolaryngol 1970; 74:431-3. 2. Crawford JS. Intubation of obstructions in the lacrimal system. C a n ] Ophthalmol 1977;12:289-92. 3. Bolger WE, Kennedy DW. Nasal endoscopy in the outpatient clinic. Otolaryngol Clin North A m 1992;25:791-802.
Ocular Findings in a Family With Sotos Syndrome (Cerebral Gigantism) Robert K. Koenekoop, M.D., Kenneth N. Rosenbaum, M.D., and Elias I. Traboulsi, M.D. PURPOSE/METHODS: We examined the ocular features in a two-generation family with Sotos syndrome (cerebral gigantism). Sotos syndrome is characterized by excessive growth in prenatal and early life, advanced bone age, and typical facial features. RESULTS/CONCLUSION: One patient had presenile nuclear sclerotic cataracts, megalophthalmos, hypoorbitism, and exotropia. One of her daughters had megalocornea, exophoria, and iris hypoplasia. Her other daughter had megalocornea. The oph thalmologist can play an important role in the diagnosis and treatment of Sotos syndrome.
S
OTOS
SYNDROME,
OR
CEREBRAL GIGANTISM,
IS
characterized by a typical craniofacial appearance, increased birth weight and length, excessive growth
Johns Hopkins Center for Hereditary Eye Diseases, Wilmer Eye Insti tute (R.K.K., E.I.T.); and Department of Medical Genetics, Children's National Medical Center, and Department of Pediatrics, George Wash ington University (K.N.R.). Inquiries to Elias I. Traboulsi, M.D., Maumenee Building, Suite 517, Wilmer Eye Institute, 600 N. Wolfe St., Baltimore, MD 21287-9237; fax: (410) 614-4363.
VOL.119, No. 5
in the first few years of life, advanced bone age, and a nonprogressive developmental delay in some patients. We examined a family with features that reflect the overgrowth and involvement of the ocular and orbital structures in Sotos syndrome. • CASE l: A 30-year-old woman had a persistent exotropia. Visual acuity was R.E.: 20/25 and LE.: 20/20. Corneal diameters measured 14 mm horizon tally. Refractions measured —4.50 +0.75 X 70 in the right eye and —5.75 in the left eye and her crystalline lenses were clear. At age 44 years, bilateral, nuclear sclerotic cataracts developed for which she underwent successful cataract surgery with intraocular lens im plantation. Keratometric measurements were 40 diop ters in both eyes and axial lengths measured 27 mm in each eye. In the vertical plane one of her orbits was lower than the other; we refer to this condition as hypoorbitism. Her medical history and results of physical examination have been previously reported.1 • CASE 2: The oldest daughter of the patient de scribed in Case 1 had an ocular examination at age 13 months when she was referred with macrocephaly to rule out a metabolic storage disorder. At 18 years of age her visual acuity was 20/20 in both eyes. Horizon tal corneal diameters measured 12.5 mm. She had clear lenses, normal irides, and mild myopia. • CASE 3: The youngest daughter of the patient described in Case 1 was examined at 14 years of age (Figure). Visual acuity was R.E.: 20/25 with a refrac tion of -1.25 +0.75 x 75 and LE.: 20/20 with a refraction of —1.00 +0.75 x 85. She had an exopho ria and horizontal corneal diameters of 14 mm. The iris was hypoplastic, without crypts or a collarette, and with peripheral iris transillumination defects. As a child she underwent craniosynostectomy (surgical opening of cranial sutures) for premature fusion of the lambdoid, sagittal, and coronal sutures. We found that cataracts, megalocornea, megaloph thalmos, hypoorbitism, exotropia, iris hypoplasia, and their intrafamilial phenotypic variability in patients can be signs of Sotos syndrome. This small pedigree probably represents autosomal dominant transmission with complete penetrance and variable expressivity. The daughters may develop cataracts before the age of
BRIEF REPORTS
657
The specimen removed at the initial operation was a flat ovoid disk of firm tissue measuring 7 X 4.5 mm and less than 1 mm thick (Fig. 1). One half of the disk had a gray translucent appearance and the other half was pink, hard, and vascularized. Histopathologic examination disclosed well-formed lamellar bone with fat-filled medullary spaces adjacent to hyaline cartilage, which contained uniform chondrocytes (Fig. 2). Because of the combination of mesenchymal elements, the diagnosis of episcleral osteocartilaginous choristoma was made. The tissue removed at the subsequent operation consisted of fibroadipose and glandular tissue and was considered to be a dermolipoma containing lacrimal tissue. Bone formation occurs through the condensation of mesenchyme in one of two ways. Membranous bones form from mesenchymal condensation into a tough membrane from which bone develops.5 This process is characteristic of many bones of the skull. Elsewhere, bone originates from mesenchymal forma tion of a cartilage template.5 This template is sub sequently invaded by blood vessels and osteoblasts, and is replaced by bone.5 In 1962 Boniuk and Zimmerman2 concluded that episcleral osseous choristomas were composed of mature compact bone that was formed in a membranous fashion like orbital bone. Their conclusion was based on observations that there had been "no cartilage associated with any of the lesions with bone present."2
VOL 119, NO. 5
Fig. 2 (Santora, Biglan, and Johnson). Photomicrograph shows thick osseous trabeculae with medullary fat, left, adjacent to hyaline cartilage rich in mature chondro cytes, right (hematoxylin and eosin, X100).
Our study demonstrates two interesting features. First, our patient had a right hypertropia caused by an episcleral choristoma. Second, the choristoma was a combination of bone and cartilage. The combination of these mesenchymal elements implies lesion forma tion from a cartilage template instead of the previous ly suggested membranous fashion. REFERENCES 1. Beckman H, Sugar SH. Episcleral osseous choristoma: report of two cases. Arch Ophthalmol 1964;71:377-8. 2. Boniuk M, Zimmerman LE. Epibulbar osteoma (episcleral osseous choristoma). Am J Ophthalmol 1962;53:290-6. 3. Pittke EC, Marquardt R, Mohr W. Cartilage choristoma of the eye. Arch Ophthalmol 1983;101:1569-71. 4. Ferry AP, Hein HF. Epibulbar osseous choristoma within an epibulbar dermoid. Am J Ophthalmol 1970;70:764-6. 5. Hollinshead WH, Rosse C. Textbook of anatomy: the connec tive tissues, 4th ed. Philadelphia: Harper & Row, 1985:27.
Intranasal Visualization for Nasolacrimal Duct Intubation Allan M. Eisenbaum, M.D., Scott K. McClatchey, M.D., and Nigel R. T. Pashley, F.R.C.S.(C.) PURPOSE/METHODS: To improve intranasal vis ualization of nasolacrimal procedures we use a self-retaining nasal speculum with or without a sinuscope.
BRIEF REPORTS
655
RESULTS/CONCLUSIONS: Placement and retrie val of silicone stents can be accomplished with less trauma than with a blind sweep technique. Addi tionally, the valve of Hasner can be seen and the creation of false passages avoided.
N
ASOLACRIMAL DUCT INTUBATION FOR PEDIATRIC nasolacrimal duct obstruction is commonly done with silicone tubes attached to a metal rod as described by Quickert and Dryden 1 and by Crawford.2 These probes often are retrieved from beneath the inferior turbinate with a grooved director or hook by using a blind sweep technique. This approach to retrieval of the probes can result in excessive bleeding and trauma to the nasal mucosa and in the creation of false passages through the wall of the nose. We have found that the use of a self-retaining nasal speculum to move the inferior turbinate away from the lateral wall of the nose permits direct viewing and remarkably easy retrieval of the probes. Additionally, a sinuscope can be used with this speculum to give outstanding visualization of the valve of Hasner (Figure).3 We place cotton-tipped swabs soaked in 0.25% phenylephrine hydrochloride under the inferior turbi nate for vasoconstriction and use a headlamp for illumination. We have also used a zero-degree pédiat rie sinuscope, which permits monitoring or recording of the procedure. The speculum (a Cottle nasal speculum with a thumbscrew, V. Mueller, McGaw Park, Illinois) is inserted under the inferior turbinate with the blades closed. The speculum enters along the floor of the nose with the handle angled toward the midline, keeping the tapered blades between the lateral wall of the nose and inferior turbinate. The blades are opened slowly, displacing the inferior turbinate medially. Careful insertion of the speculum with a twisting motion prevents slippage of the blades.
Department of Ophthalmology, University of Colorado Health Scienc es Center and Children's Hospital (A.M.E.); Department of Ophthal mology, Children's National Medical Center (S.K.M.); and Presbyteri an/St. Luke's Medical Center (N.R.T.P.). The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the United States Navy or Department of Defense. Inquiries to Allan M. Eisenbaum, M.D., 14991 E. Hampden Ave., Suite 300, Aurora, CO 80014; fax: (303) 369-2031.
656
The blades are occasionally inserted too far posterior ly and must be pulled out slightly. W h e n proper exposure has been achieved the blades are locked open with the thumbscrew. The handle is pointed inferiorly away from the eye and rested on folded gauze pads. If there is any bleeding at this point a thin suction tip is used to remove the blood. The probes are then inserted into the nasolacrimal duct and removed with a hook or grooved director under direct visualization. We have used our technique for the past three years, and in every case we easily visualized and retrieved the probes in less time and with less bleeding than with the blind technique. In one instance we saw the probe poke through a membrane over the valve of Hasner while viewing the probe with the pédiatrie sinuscope. We occasionally saw the probe directed laterally into the mucosa of the wall of the nose and we were able to grasp and remove it. Had we been using a blind technique we would have been unable to feel the metal-on-metal tap with the probe retriever or would have caused excess bleeding if a Crawford hook (Jedmed Instrument Company, St. Louis, Missouri) were inserted. Additionally, because
Figure (Eisenbaum, McClatchey, and Pashley). Bowman probe beneath inferior turbinate through the valve of Hasner (arrow). Probe visualized with sinuscope.
AMERICAN JOURNAL OF OPHTHALMOLOGY
MAY
1995
we were able to see the probe intranasally, we never inserted the probe too far inferiorly. Our technique frees one hand of the surgeon and allows the surgeon to visualize directly Bowman's probes or remove a previously placed stent. Our technique also could be helpful in teaching the surgical anatomy of nasolacrimal duct intubation and removal, particularly when viewed by a sinuscope and seen on a video monitor. REFERENCES 1. Quickert MH, Dryden RM. Probes for intubation in lacrimal drainage. Trans A m Acad Ophthalmol Otolaryngol 1970; 74:431-3. 2. Crawford JS. Intubation of obstructions in the lacrimal system. C a n ] Ophthalmol 1977;12:289-92. 3. Bolger WE, Kennedy DW. Nasal endoscopy in the outpatient clinic. Otolaryngol Clin North A m 1992;25:791-802.
Ocular Findings in a Family With Sotos Syndrome (Cerebral Gigantism) Robert K. Koenekoop, M.D., Kenneth N. Rosenbaum, M.D., and Elias I. Traboulsi, M.D. PURPOSE/METHODS: We examined the ocular features in a two-generation family with Sotos syndrome (cerebral gigantism). Sotos syndrome is characterized by excessive growth in prenatal and early life, advanced bone age, and typical facial features. RESULTS/CONCLUSION: One patient had presenile nuclear sclerotic cataracts, megalophthalmos, hypoorbitism, and exotropia. One of her daughters had megalocornea, exophoria, and iris hypoplasia. Her other daughter had megalocornea. The oph thalmologist can play an important role in the diagnosis and treatment of Sotos syndrome.
S
OTOS
SYNDROME,
OR
CEREBRAL GIGANTISM,
IS
characterized by a typical craniofacial appearance, increased birth weight and length, excessive growth
Johns Hopkins Center for Hereditary Eye Diseases, Wilmer Eye Insti tute (R.K.K., E.I.T.); and Department of Medical Genetics, Children's National Medical Center, and Department of Pediatrics, George Wash ington University (K.N.R.). Inquiries to Elias I. Traboulsi, M.D., Maumenee Building, Suite 517, Wilmer Eye Institute, 600 N. Wolfe St., Baltimore, MD 21287-9237; fax: (410) 614-4363.
VOL.119, No. 5
in the first few years of life, advanced bone age, and a nonprogressive developmental delay in some patients. We examined a family with features that reflect the overgrowth and involvement of the ocular and orbital structures in Sotos syndrome. • CASE l: A 30-year-old woman had a persistent exotropia. Visual acuity was R.E.: 20/25 and LE.: 20/20. Corneal diameters measured 14 mm horizon tally. Refractions measured —4.50 +0.75 X 70 in the right eye and —5.75 in the left eye and her crystalline lenses were clear. At age 44 years, bilateral, nuclear sclerotic cataracts developed for which she underwent successful cataract surgery with intraocular lens im plantation. Keratometric measurements were 40 diop ters in both eyes and axial lengths measured 27 mm in each eye. In the vertical plane one of her orbits was lower than the other; we refer to this condition as hypoorbitism. Her medical history and results of physical examination have been previously reported.1 • CASE 2: The oldest daughter of the patient de scribed in Case 1 had an ocular examination at age 13 months when she was referred with macrocephaly to rule out a metabolic storage disorder. At 18 years of age her visual acuity was 20/20 in both eyes. Horizon tal corneal diameters measured 12.5 mm. She had clear lenses, normal irides, and mild myopia. • CASE 3: The youngest daughter of the patient described in Case 1 was examined at 14 years of age (Figure). Visual acuity was R.E.: 20/25 with a refrac tion of -1.25 +0.75 x 75 and LE.: 20/20 with a refraction of —1.00 +0.75 x 85. She had an exopho ria and horizontal corneal diameters of 14 mm. The iris was hypoplastic, without crypts or a collarette, and with peripheral iris transillumination defects. As a child she underwent craniosynostectomy (surgical opening of cranial sutures) for premature fusion of the lambdoid, sagittal, and coronal sutures. We found that cataracts, megalocornea, megaloph thalmos, hypoorbitism, exotropia, iris hypoplasia, and their intrafamilial phenotypic variability in patients can be signs of Sotos syndrome. This small pedigree probably represents autosomal dominant transmission with complete penetrance and variable expressivity. The daughters may develop cataracts before the age of
BRIEF REPORTS
657