Volume 18 Number 2 / April 2014
Ilhan et al
189
Orbital compression syndrome complicated by epidural hematoma and wide cephalohematoma in a patient with sickle cell disease Nilufer Ilhan, MD,a Can Acipayam, MD,b Fusun Aydogan, MD,c Nesrin Atci, MD,d Ozgur Ilhan, MD,a Mesut Coskun, MD,a Mutlu Cihan Daglioglu, MD,a and Esra Ayhan Tuzcu, MDa Orbital wall infarctions resulting in orbital and epidural hematomas are rare manifestations of sickle cell disease (SCD). We report orbital compression syndrome associated with an epidural hematoma and wide cephalohematoma in a 15-year-old boy with SCD. An infarcted orbital bone was observed on magnetic resonance imaging and three-phase bone scintigraphy with Technetium-99m methylene diphosphonate. The patient recovered completely without surgical intervention at the end of the fourth week. Prompt diagnosis and proper management are critical for complete recovery.
Case Report
A
15-year-old boy with homozygous sickle cell (HbSS) disease presented to the Department of Ophthalmology of the medical faculty at Mustafa Kemal University, Antakya, Turkey. The patient was febrile, complained of frontal headache, back pain, bilateral lower limb pain and swelling of both eyelids. He denied a history of trauma, insect bite, or other disease. On the ophthalmological examination, bilateral periorbital swelling, worse on the right side, and moderate limitation of eye movements in the right eye were observed. The patient had conjunctival icterus and edema. Visual acuity was 20/20 bilaterally. Pupillary reflexes and intraocular pressure were normal. Dilated fundus examination revealed retinal venous tortuosity without proliferative retinopathy. Blood, urine, and throat cultures were negative. Noncontrast computerized tomography (CT) showed a subperiosteal hematoma in the superolateral region of the right eye
Author affiliations: aDepartment of Ophthalmology, Mustafa Kemal University Faculty of Medicine, Antakya, Turkey; bDepartment of Pediatric Hematology, Antakya State Hospital, Antakya, Turkey; cDepartment of Nuclear Medicine, Mustafa Kemal University Faculty of Medicine, Antakya; dDepartment of Radiology, Mustafa Kemal University Faculty of Medicine, Antakya Submitted June 21, 2013. Revision accepted November 4, 2013. Correspondence: Nilufer Ilhan, MD, Department of Ophthalmology, Mustafa Kemal University Faculty of Medicine, Antakya, Hatay, Turkey (email: [email protected]). J AAPOS 2014;18:189-191. Copyright Ó 2014 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2013.11.011
Journal of AAPOS
FIG 1. Computed tomography of the cranium. A, Sagittal view showing subperiosteal hematoma in the superolateral region of the right orbit and right frontal epidural hematoma (arrows). B, Coronal view showing an extensive cephalohematoma in the frontoparietal region (between stars); a hyperdense lesion adjacent to the right parietal bone due to active hemorrhage was detected (arrow).
and a right frontal epidural hematoma (Figure 1A). The patient was hospitalized in the intensive care unit. Intravenous fluids, broad spectrum antibiotics, and analgesics were administered. Two days later, his headache returned, and a large scalp swelling appeared. There was no marked change in the size of the orbital and epidural hematomas; however, a wide cephalohematoma covering the frontoparietal region had occurred (Figure 1B). Intravenous dexamethasone under antibiotic cover was added to the treatment. On day
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FIG 2. T2-weighted magnetic resonance imaging of the brain showing abnormal hyperintense signals due to the frontal bone infarct (large arrow) and marrow hyperplasia (small arrow).
5, the patient was stabilized, and the periorbital and scalp swelling decreased. Eye movements were normal. Magnetic resonance imaging (MRI) of the brain showed diffuse calvarial thickening due to the expansion of the diploic space. Marrow hyperplasia and bone infarction with increased signal intensities were detected in the frontal region of the calvarium with T2-weighted scans (Figure 2). Threephase bone scintigraphy was performed the following day with Technetium-99m methylene diphosphonate. In addition to the planar images, blood pool single-photon emission computed tomography (SPECT) and late-phase SPECT images were obtained and revealed a defective hypoactive area at the frontal region (Figure 3). The orbital and epidural hematomas resolved completely within 4 weeks. There was no recurrence of bleeding during 9 months of follow-up.
Discussion Vasoocclusive crises (VOC), also referred to as sickle crises, are caused by local vasoocclusion within the bone marrow, resulting in bone infarction and the release of inflammatory mediators.1 Recurrent painful episodes due to VOC are the hallmark of sickle cell disease (SCD) and constitute the majority of urgent hospital admissions.2 Pain is generated by the enhanced intramedullary pressure, caused by the inflammatory response to avascular bone marrow.3 VOC normally involves long bones and vertebrae but may also affect bones with active marrow. Involvement of the calvarium and orbita has rarely been reported.1,4,5 Orbital wall infarct is common in younger patients due to relatively large spongy bone tissue between the hard outer- and inner-bone layers in orbital bones. The inflammatory response created by infarcted bone includes vessel wall necrosis and extravasation of blood.4 Subperiosteal hematoma and periorbital swelling may form proptosis, limited ocular motility, corneal hypoesthesia,
FIG 3. Blood pool (A) and single-photon emission computed tomography image (B) showing a defective, hypoactive area at the frontal region of the cranium (arrows).
and/or decreased visual impairment (orbital compression syndrome).6 This condition can mimic infections, such as preseptal cellulitis, subperiosteal abscess, and osteomyelitis. MRI and radionuclide scanning play an important role in the differential diagnosis. MRI has the added advantage of demonstrating ischemic changes in bone marrow.7 The MRI findings of acute bone infarcts include abnormal bone marrow signal, subperiosteal fluid collections, and heterogeneous enhancement within the bone marrow. Acute bone marrow infarct generally appears as an increased signal on T2-weighted images. Bone scintigraphy shows the absence of or decrease in tracer uptake in the affected bone, which points to the impaired blood supply to the area in the acute phase.8 Bone SPECT is more beneficial in the separation of bone structures because the bones are superimposed in planar cranial imaging.9 Planar bone scintigraphy may not give sufficiently accurate information that detects the anatomic location of lesions in the assessment of complex bony structures such as the skull base.10 In the present case, the patient’s orbital compression syndrome did not include optic nerve dysfunction and was accompanied by epidural and large scalp hematoma. The absence of radionuclide uptake confirmed frontal bone infarction, which was seen through the scintigraphy scans. To our knowledge, this is the first reported case of blood pool SPECT and a late-phase SPECT scan of orbital bone infarction in a case with SCD.
Journal of AAPOS
Volume 18 Number 2 / April 2014
Literature Search The authors searched PubMed (MEDLINE) for Englishlanguage articles for the period 1946-2013 using combinations of the following search terms: orbital compression syndrome, sickle cell disease, epidural hematoma, and cephalohematoma. References 1. van Beers EJ, van Tuijn CF, Nieuwkerk PT, Friederich PW, Vranken JH, Biemond BJ. Patient-controlled analgesia versus continuous infusion of morphine during vaso-occlusive crisis in sickle cell disease, a randomized controlled trial. Am J Hematol 2007;82:955-60. 2. Ganesh A, Al-Zuhaibi S, Pathare A, et al. Orbital infarction in sickle cell disease. Am J Ophthalmol 2008;146:595-601. 3. Ganesh A, William RR, Mitra S, et al. Orbital involvement in sickle cell disease: a report of five cases and review literature. Eye (Lond) 2001;15:774-80. 4. Karacostas D, Artemis N, Papadopoulou M, Christakis J. Case report: epidural and bilateral retroorbital hematomas complicating sickle cell anemia. Am J Med Sci 1991;302:107-9. 5. Ozkavukcu E, Fitoz S, Yagmurlu B, Ciftci E, Erden I, Ertem M. Orbital wall infarction mimicking periorbital cellulitis in a patient with sickle cell disease. Pediatr Radiol 2007;37:388-90. 6. Curran EL, Fleming JC, Rice K, Wang WC. Orbital compression syndrome in sickle cell disease. Ophthalmology 1997;104:1610-15. 7. Mankad VN, Williams JP, Harpen MD, et al. Magnetic resonance imaging of bone marrow in sickle cell disease: clinical, hematologic, and pathologic correlations. Blood 1990;75:274-83. 8. Kim SK, Miller JH. Natural history and distribution of bone and bone marrow infarction in sickle hemoglobinopathies. J Nucl Med 2002;43: 896-900. 9. Buyukdereli G, Guney IB, Ozerdem G, Kesiktas E. Evaluation of vascularized graft reconstruction of the mandible with Tc-99m MDP bone scintigraphy. Ann Nucl Med 2006;20:89-93. 10. Fukumoto M, Yoshida S, Yoshida D, Kishimoto S. Dual-isotope SPECT of skull-base invasion of head and neck tumors. J Nucl Med 1995;36:1741-6.
Anterior lentiplane associated with posterior lenticonus and microcornea Michelle D. Lingao, MD,a Grace T. Liu, MD,a,b,c Kammi Gunton, MD,a,d and Alex V. Levin, MD, MHSca,d
Author affiliations: aPediatric Ophthalmology and Ocular Genetics, Wills Eye Institute, Philadelphia, Pennsylvania; bPediatric Ophthalmic Consultants, New York, New York; c Department of Ophthalmology, New York University, Langone Medical Center New York, New York; dThomas Jefferson University, Philadelphia, Pennsylvania Funded in part by the Foerderer Fund (AVL), the Alfiero and Lucia Palestroni Foundation (GTL), and an Alcon Fellowship Grant (MDL, GTL). Submitted June 7, 2013. Revision accepted November 1, 2013. Correspondence: Alex V. Levin, MD, MHSc, Chief, Pediatric Ophthalmology and Ocular Genetics, Wills Eye Institute, 840 Walnut St., Philadelphia, Pennsylvania 19107-5109 (email: [email protected]). J AAPOS 2014;18:191-192. Copyright Ó 2014 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2013.11.009
Journal of AAPOS
Lingao et al
191
We report a 12-year-old boy with a rare condition consisting of familial bilateral anterior lentiplane (a flat anterior lens capsule) posterior lenticonus, and microcornea.
Case Report
A
12-year-old boy was referred to the Wills Eye Institute, Philadelphia, for evaluation for possible cataract surgery. He had been followed since infancy for bilateral congenital cataracts and high hyperopia. The cataracts were initially visually insignificant but eventually caused symptomatic decreased visual acuity in the left eye. The patient was the product of an uncomplicated full-term pregnancy and delivery. Physical examination was normal. He was otherwise healthy. A multigenerational pedigree revealed no consanguinity. His 13-year-old sister had congenital cataracts, microcephaly, microphthalmia (axial length, 19.91 mm in the right eye and 20.31 mm in the left eye), and learning disability; she underwent lens extraction at 2 weeks of age and successful rehabilitation with contact lenses. On his ophthalmological examination, the best-corrected visual acuity (17.00 10.75 100 right eye, 19.75 12.00 95 left eye) was 20/30 and 20/600 respectively. Pupillary responses were normal; there was no afferent pupillary defect. Slit-lamp examination showed a right posterior subcapsular cataract within a posterior lenticonus with spokelike subcapsular opacities radiating from the center of the posterior capsule (Figure 1) and a more diffuse posterior subcaspular cataract within a large posterior lenticonus in the left eye. There was also a left anterior subcapsular cataract. Corneal diameters were 9.5 mm in each eye. Intraocular pressure was 14 mm Hg in each eye by Goldmann applanation tonometry. Dilated fundus examination was normal, but the view of the left retina was obscured by the cataract. Immersion A-scan biometry (Eyecubed V4; Ellex, Minneapolis, MN) revealed axial length of 22.45 mm in the right eye and 22.52 mm in the left eye. Keratometry (Eyecubed V4 ) showed 37.79 38.84 D in the right eye and 37.09 38.93 D in the left eye. Anterior chamber depth was 2.90 mm on the right and 3.18 mm on the left left. Lens thickness was 3.62 mm on the right and 3.13 mm on the left. Ultrasound biomicroscopy (SKU 24-6300; Accutome, Malvern, PA) of both eyes showed a flat anterior surface of the lens (Figure 2). The patient underwent left cataract extraction with anterior vitrectomy. Intraoperatively, the flattened lens surface was not discernible. The anterior capsule seemed friable, and a tag broke off during manual capsulorhexis. Capsulorhexis was completed despite a rent that remained confined to the anterior lens surface. Because of his anterior segment dimensions, capsule abnormalities, and positive family experience with contact lenses, he was left
Ilhan et al
189
Orbital compression syndrome complicated by epidural hematoma and wide cephalohematoma in a patient with sickle cell disease Nilufer Ilhan, MD,a Can Acipayam, MD,b Fusun Aydogan, MD,c Nesrin Atci, MD,d Ozgur Ilhan, MD,a Mesut Coskun, MD,a Mutlu Cihan Daglioglu, MD,a and Esra Ayhan Tuzcu, MDa Orbital wall infarctions resulting in orbital and epidural hematomas are rare manifestations of sickle cell disease (SCD). We report orbital compression syndrome associated with an epidural hematoma and wide cephalohematoma in a 15-year-old boy with SCD. An infarcted orbital bone was observed on magnetic resonance imaging and three-phase bone scintigraphy with Technetium-99m methylene diphosphonate. The patient recovered completely without surgical intervention at the end of the fourth week. Prompt diagnosis and proper management are critical for complete recovery.
Case Report
A
15-year-old boy with homozygous sickle cell (HbSS) disease presented to the Department of Ophthalmology of the medical faculty at Mustafa Kemal University, Antakya, Turkey. The patient was febrile, complained of frontal headache, back pain, bilateral lower limb pain and swelling of both eyelids. He denied a history of trauma, insect bite, or other disease. On the ophthalmological examination, bilateral periorbital swelling, worse on the right side, and moderate limitation of eye movements in the right eye were observed. The patient had conjunctival icterus and edema. Visual acuity was 20/20 bilaterally. Pupillary reflexes and intraocular pressure were normal. Dilated fundus examination revealed retinal venous tortuosity without proliferative retinopathy. Blood, urine, and throat cultures were negative. Noncontrast computerized tomography (CT) showed a subperiosteal hematoma in the superolateral region of the right eye
Author affiliations: aDepartment of Ophthalmology, Mustafa Kemal University Faculty of Medicine, Antakya, Turkey; bDepartment of Pediatric Hematology, Antakya State Hospital, Antakya, Turkey; cDepartment of Nuclear Medicine, Mustafa Kemal University Faculty of Medicine, Antakya; dDepartment of Radiology, Mustafa Kemal University Faculty of Medicine, Antakya Submitted June 21, 2013. Revision accepted November 4, 2013. Correspondence: Nilufer Ilhan, MD, Department of Ophthalmology, Mustafa Kemal University Faculty of Medicine, Antakya, Hatay, Turkey (email: [email protected]). J AAPOS 2014;18:189-191. Copyright Ó 2014 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2013.11.011
Journal of AAPOS
FIG 1. Computed tomography of the cranium. A, Sagittal view showing subperiosteal hematoma in the superolateral region of the right orbit and right frontal epidural hematoma (arrows). B, Coronal view showing an extensive cephalohematoma in the frontoparietal region (between stars); a hyperdense lesion adjacent to the right parietal bone due to active hemorrhage was detected (arrow).
and a right frontal epidural hematoma (Figure 1A). The patient was hospitalized in the intensive care unit. Intravenous fluids, broad spectrum antibiotics, and analgesics were administered. Two days later, his headache returned, and a large scalp swelling appeared. There was no marked change in the size of the orbital and epidural hematomas; however, a wide cephalohematoma covering the frontoparietal region had occurred (Figure 1B). Intravenous dexamethasone under antibiotic cover was added to the treatment. On day
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FIG 2. T2-weighted magnetic resonance imaging of the brain showing abnormal hyperintense signals due to the frontal bone infarct (large arrow) and marrow hyperplasia (small arrow).
5, the patient was stabilized, and the periorbital and scalp swelling decreased. Eye movements were normal. Magnetic resonance imaging (MRI) of the brain showed diffuse calvarial thickening due to the expansion of the diploic space. Marrow hyperplasia and bone infarction with increased signal intensities were detected in the frontal region of the calvarium with T2-weighted scans (Figure 2). Threephase bone scintigraphy was performed the following day with Technetium-99m methylene diphosphonate. In addition to the planar images, blood pool single-photon emission computed tomography (SPECT) and late-phase SPECT images were obtained and revealed a defective hypoactive area at the frontal region (Figure 3). The orbital and epidural hematomas resolved completely within 4 weeks. There was no recurrence of bleeding during 9 months of follow-up.
Discussion Vasoocclusive crises (VOC), also referred to as sickle crises, are caused by local vasoocclusion within the bone marrow, resulting in bone infarction and the release of inflammatory mediators.1 Recurrent painful episodes due to VOC are the hallmark of sickle cell disease (SCD) and constitute the majority of urgent hospital admissions.2 Pain is generated by the enhanced intramedullary pressure, caused by the inflammatory response to avascular bone marrow.3 VOC normally involves long bones and vertebrae but may also affect bones with active marrow. Involvement of the calvarium and orbita has rarely been reported.1,4,5 Orbital wall infarct is common in younger patients due to relatively large spongy bone tissue between the hard outer- and inner-bone layers in orbital bones. The inflammatory response created by infarcted bone includes vessel wall necrosis and extravasation of blood.4 Subperiosteal hematoma and periorbital swelling may form proptosis, limited ocular motility, corneal hypoesthesia,
FIG 3. Blood pool (A) and single-photon emission computed tomography image (B) showing a defective, hypoactive area at the frontal region of the cranium (arrows).
and/or decreased visual impairment (orbital compression syndrome).6 This condition can mimic infections, such as preseptal cellulitis, subperiosteal abscess, and osteomyelitis. MRI and radionuclide scanning play an important role in the differential diagnosis. MRI has the added advantage of demonstrating ischemic changes in bone marrow.7 The MRI findings of acute bone infarcts include abnormal bone marrow signal, subperiosteal fluid collections, and heterogeneous enhancement within the bone marrow. Acute bone marrow infarct generally appears as an increased signal on T2-weighted images. Bone scintigraphy shows the absence of or decrease in tracer uptake in the affected bone, which points to the impaired blood supply to the area in the acute phase.8 Bone SPECT is more beneficial in the separation of bone structures because the bones are superimposed in planar cranial imaging.9 Planar bone scintigraphy may not give sufficiently accurate information that detects the anatomic location of lesions in the assessment of complex bony structures such as the skull base.10 In the present case, the patient’s orbital compression syndrome did not include optic nerve dysfunction and was accompanied by epidural and large scalp hematoma. The absence of radionuclide uptake confirmed frontal bone infarction, which was seen through the scintigraphy scans. To our knowledge, this is the first reported case of blood pool SPECT and a late-phase SPECT scan of orbital bone infarction in a case with SCD.
Journal of AAPOS
Volume 18 Number 2 / April 2014
Literature Search The authors searched PubMed (MEDLINE) for Englishlanguage articles for the period 1946-2013 using combinations of the following search terms: orbital compression syndrome, sickle cell disease, epidural hematoma, and cephalohematoma. References 1. van Beers EJ, van Tuijn CF, Nieuwkerk PT, Friederich PW, Vranken JH, Biemond BJ. Patient-controlled analgesia versus continuous infusion of morphine during vaso-occlusive crisis in sickle cell disease, a randomized controlled trial. Am J Hematol 2007;82:955-60. 2. Ganesh A, Al-Zuhaibi S, Pathare A, et al. Orbital infarction in sickle cell disease. Am J Ophthalmol 2008;146:595-601. 3. Ganesh A, William RR, Mitra S, et al. Orbital involvement in sickle cell disease: a report of five cases and review literature. Eye (Lond) 2001;15:774-80. 4. Karacostas D, Artemis N, Papadopoulou M, Christakis J. Case report: epidural and bilateral retroorbital hematomas complicating sickle cell anemia. Am J Med Sci 1991;302:107-9. 5. Ozkavukcu E, Fitoz S, Yagmurlu B, Ciftci E, Erden I, Ertem M. Orbital wall infarction mimicking periorbital cellulitis in a patient with sickle cell disease. Pediatr Radiol 2007;37:388-90. 6. Curran EL, Fleming JC, Rice K, Wang WC. Orbital compression syndrome in sickle cell disease. Ophthalmology 1997;104:1610-15. 7. Mankad VN, Williams JP, Harpen MD, et al. Magnetic resonance imaging of bone marrow in sickle cell disease: clinical, hematologic, and pathologic correlations. Blood 1990;75:274-83. 8. Kim SK, Miller JH. Natural history and distribution of bone and bone marrow infarction in sickle hemoglobinopathies. J Nucl Med 2002;43: 896-900. 9. Buyukdereli G, Guney IB, Ozerdem G, Kesiktas E. Evaluation of vascularized graft reconstruction of the mandible with Tc-99m MDP bone scintigraphy. Ann Nucl Med 2006;20:89-93. 10. Fukumoto M, Yoshida S, Yoshida D, Kishimoto S. Dual-isotope SPECT of skull-base invasion of head and neck tumors. J Nucl Med 1995;36:1741-6.
Anterior lentiplane associated with posterior lenticonus and microcornea Michelle D. Lingao, MD,a Grace T. Liu, MD,a,b,c Kammi Gunton, MD,a,d and Alex V. Levin, MD, MHSca,d
Author affiliations: aPediatric Ophthalmology and Ocular Genetics, Wills Eye Institute, Philadelphia, Pennsylvania; bPediatric Ophthalmic Consultants, New York, New York; c Department of Ophthalmology, New York University, Langone Medical Center New York, New York; dThomas Jefferson University, Philadelphia, Pennsylvania Funded in part by the Foerderer Fund (AVL), the Alfiero and Lucia Palestroni Foundation (GTL), and an Alcon Fellowship Grant (MDL, GTL). Submitted June 7, 2013. Revision accepted November 1, 2013. Correspondence: Alex V. Levin, MD, MHSc, Chief, Pediatric Ophthalmology and Ocular Genetics, Wills Eye Institute, 840 Walnut St., Philadelphia, Pennsylvania 19107-5109 (email: [email protected]). J AAPOS 2014;18:191-192. Copyright Ó 2014 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2013.11.009
Journal of AAPOS
Lingao et al
191
We report a 12-year-old boy with a rare condition consisting of familial bilateral anterior lentiplane (a flat anterior lens capsule) posterior lenticonus, and microcornea.
Case Report
A
12-year-old boy was referred to the Wills Eye Institute, Philadelphia, for evaluation for possible cataract surgery. He had been followed since infancy for bilateral congenital cataracts and high hyperopia. The cataracts were initially visually insignificant but eventually caused symptomatic decreased visual acuity in the left eye. The patient was the product of an uncomplicated full-term pregnancy and delivery. Physical examination was normal. He was otherwise healthy. A multigenerational pedigree revealed no consanguinity. His 13-year-old sister had congenital cataracts, microcephaly, microphthalmia (axial length, 19.91 mm in the right eye and 20.31 mm in the left eye), and learning disability; she underwent lens extraction at 2 weeks of age and successful rehabilitation with contact lenses. On his ophthalmological examination, the best-corrected visual acuity (17.00 10.75 100 right eye, 19.75 12.00 95 left eye) was 20/30 and 20/600 respectively. Pupillary responses were normal; there was no afferent pupillary defect. Slit-lamp examination showed a right posterior subcapsular cataract within a posterior lenticonus with spokelike subcapsular opacities radiating from the center of the posterior capsule (Figure 1) and a more diffuse posterior subcaspular cataract within a large posterior lenticonus in the left eye. There was also a left anterior subcapsular cataract. Corneal diameters were 9.5 mm in each eye. Intraocular pressure was 14 mm Hg in each eye by Goldmann applanation tonometry. Dilated fundus examination was normal, but the view of the left retina was obscured by the cataract. Immersion A-scan biometry (Eyecubed V4; Ellex, Minneapolis, MN) revealed axial length of 22.45 mm in the right eye and 22.52 mm in the left eye. Keratometry (Eyecubed V4 ) showed 37.79 38.84 D in the right eye and 37.09 38.93 D in the left eye. Anterior chamber depth was 2.90 mm on the right and 3.18 mm on the left left. Lens thickness was 3.62 mm on the right and 3.13 mm on the left. Ultrasound biomicroscopy (SKU 24-6300; Accutome, Malvern, PA) of both eyes showed a flat anterior surface of the lens (Figure 2). The patient underwent left cataract extraction with anterior vitrectomy. Intraoperatively, the flattened lens surface was not discernible. The anterior capsule seemed friable, and a tag broke off during manual capsulorhexis. Capsulorhexis was completed despite a rent that remained confined to the anterior lens surface. Because of his anterior segment dimensions, capsule abnormalities, and positive family experience with contact lenses, he was left
