- Email: [email protected]
Iris mammillations. Three case reports
a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(7):279–281
ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA www.elsevier.es/oftalmologia
Short communication
Iris mammillations. Three case reports夽 B. Sánchez Marugán ∗ , M. Acebes García, J. García Hinojosa, ˜ M.J. León Cabello, M. Casal Valino Servicio de Oftalmología, Hospital Universitario Infanta Leonor, Madrid, Spain
a r t i c l e
i n f o
a b s t r a c t
Article history:
Clinical cases: We report the cases of 3 young patients who were seen in our hospital with
Received 20 September 2012
the diagnosis of iris mammillations. Two of them were bilateral without familial association.
Accepted 12 April 2013
The third child had iris mammillations as part of an ocular melanocytosis.
Available online 26 September 2014
None of them had abnormalities in the posterior segment or the pachymetry. Discussion: The discovery of iris mammillations during an examination requires a long-term
Keywords:
follow-up of these patients due to its association with uveal melanoma. This is required
Mammillations
whether it is a casual discovery or in the presence of ocular melanocytosis.
Melanocytosis
˜ © 2012 Sociedad Espanola de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.
Nevus de Ota Uveal melanoma Iris hyperpigmentation
Mamelones de iris. A propósito de tres casos r e s u m e n Palabras clave:
Casos clínicos: Se presentan tres pacientes jóvenes vistos en nuestro servicio con diagnóstico
Mamelones
de mamelones de iris. Dos de los casos eran bilaterales, sin asociación familiar. El tercero
Melanocitosis
presentó mamelones de iris en el contexto de una melanocitosis ocular. Ninguno mostró
Nevus de Ota
asociación con anomalías en el polo posterior o en la paquimetría.
Melanoma uveal
Discusión: La presencia de mamelones de iris en una exploración, tanto como hallazgo casual
Hiperpigmentación de iris
como en el contexto de una melanocitosis ocular, requiere del seguimiento a largo plazo de estos pacientes por su posible asociación con el melanoma de úvea. ˜ © 2012 Sociedad Espanola de Oftalmología. Publicado por Elsevier España, S.L.U. Todos los derechos reservados.
Introduction Mammillations are congenital anomalies of the iris characterized by dome-shaped elevations of the iris, regularly
distributed over a part or the entire surface of a characteristically velvety iris (without surface crypts). Iris mammillations appear isolated or associated with ocular or dermal-ocular (Nevus of Otta [sic: Ota]) melanocytosis, in such cases with increased risk of uveal melanoma.
夽
˜ M. Mamelones de iris. Please cite this article as: Sánchez Marugán B, Acebes García M, García Hinojosa J, León Cabello MJ, Casal Valino A propósito de tres casos. Arch Soc Esp Oftalmol. 2014;89:279–281. ∗ Corresponding author. E-mail address: [email protected] (B. Sánchez Marugán). ˜ 2173-5794/$ – see front matter © 2012 Sociedad Espanola de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.
280
a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(7):279–281
Fig. 1 – Details of the iris showing iris mammillations on a hyperpigmented iris of patient in case 1.
Fig. 3 – Details of the iris of right eye (left panel) and left eye (right panel) of patient in case 3.
Therefore, it is important to know these lesions for long-term patient follow-up, preventing uncontrolled development of this severe ocular complication. This is a report of three cases of children who were seen in our department and had iris mammillations during eye examination.
to 170◦ ): 1 and left eye (+1.75; −0.75 to 5◦ ): 1. The previous biomicroscopy has bilateral mammillations of the iris (Fig. 3). Fundus examination is normal. Pachymetry values for her right eye were 550, and 540 for the left one.
Case 1 5-year-old male patient, Caucasian, seen for pigmentation in the sclera of his left eye, from neonatal period. On examination the patient showed 20/25 uncorrected visual acuity in both eyes. Previous biomicroscopy shows scleral pigmentation and iris hyperpigmentation with elevated pigmented nodules and regularly distributed in the left eye (iris mamelons) (Fig. 1). Fundus examination showed no changes. Intraocular pressure could not be measured due to lack of cooperation of the patient; however, it was possible to measure corneal thickness; results were 564 in the right eye and 567 on the left.
Case 2 8-year-old Hispanic female patient who is seen for reduced sight. Examination reveals: AV: right eye (−2.00; 180◦ ): 1 and left eye (+1.00, −4.00 to 5◦ ): 0.3. Biomicroscopy shows bilateral iris mammillations diffusely distributed (Fig. 2). Fundus examination was within normal limits. Pachymetry was around 540–545 , in the right and left eye respectively.
Case 3 16-year-old female patient, white race, who comes in for headaches. Examination reveals: AV: right eye (+1.75; −0.50
Fig. 2 – Details of the iris of right eye (left panel) and left eye (right panel) of patient in case 2.
Discussion Iris mammillations are raised nipple-shaped protrusions that fully or partially cover the surface of the iris. The term was coined by Coats in 1912 to describe the iris of a subject with ocular melanosis. They are characterized by being raised lesions, soft on their surface and variously dome-shaped, conical or stellate at their base, regularly covering the surface of all or part of the iris. When the surface of the iris is fully covered with mammillations, these become larger as they approach the pupillary margin. The iris where they are located is usually more pigmented, appearing as heterochromia in subjects with unilateral mammillations.1 Congenital and sporadic lesions have been reported in family cases with autosomal dominant or recessive, polygenic and multifactorial heredity. Most of the cases reported in literature are unilateral, although there are also bilateral cases.2 Iris mammillations appear more frequently in subjects with ocular melanocytosis (hyperpigmentation of uveal tissue, iris, sclera, etc.) but can also occur without other coexisting ocular or dermal pigmentary conditions. It has been suggested that iris mammillations could be either a low expression of ocular melanocytosis or an isolated disorder.3 If we consider them as a variant of ocular melanocytosis, then we can also consider them as premalignant lesions; however, in most reported cases associated with an intraocular tumor, a preexisting melanocytosis is the most important etiological factor. Differential diagnosis includes Lisch nodules (neurofibromatosis type I), tapioca melanoma, granulomatous inflammation of the iris and the Cogan-Reese syndrome.4,5 Lisch nodules have a cushiony appearance, are unevenly distributed and have a color lighter than the iris surface. They tend to be larger than mammillations and are more variable in size and number. They look brown in blue or green iris, and paler than underlying iris in brown eyes. By contrast, iris mammillations are the same color of the underlying iris in brown eyes and are a medium brown on blue or green eyes. Tapioca melanoma is a pale or amelanotic tumor. It grows slowly and is typical in young patients.6 Cogan-Reese syndrome is characterized
a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(7):279–281
by diffuse nodules, peripheral anterior synechiae, iris atrophy and dyscoria. Granulomatous inflammatory nodules occur in uveitis as Koeppe and Busacca nodules. Prevalence of iris mammillations in the general population is unknown. We can ensure that these lesions are rare, probably underdiagnosed,2 to be considered as a sign of ocular melanocytosis and be followed by the long-term risk posed by developing uveal melanoma.
Conflict of interest Authors declare having no conflict of interest.
references
1. Ragge NK, Acheson J, Murphree AL. Iris mammillations significance and associations. Eye. 1996;10:86–91.
281
2. Ozdamar Y. A case of bilateral iris mammillations with unusual papillary appearance and pesudohypertension. Clin Exp Optom. 2007;90:471–3. 3. Gündünz K, Shields CL, Shields JA, Eagle Jr RC, Singh AD. Iris mammillations as the only sign of ocular melanocitosis in a child with choroidal melanoma. Arch Opthalmol. 2000;118:716–7. 4. Ragge NK, Acheson J, Murphree AL. Iris mammillations: significance and associations. Eye. 1996;10:86–91. 5. Swann PG. Iris mammillations in ocular melanocytosis. Clin Exp Optom. 2001;84:35–8. 6. Viestenz A, Conway RM, Kuchle M. Tapioca melanoma of the iris mimicking a vascular tumour: a clinicopathological correlation. Clin Exp Ophthalmol. 2004;32:327–30.
ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA www.elsevier.es/oftalmologia
Short communication
Iris mammillations. Three case reports夽 B. Sánchez Marugán ∗ , M. Acebes García, J. García Hinojosa, ˜ M.J. León Cabello, M. Casal Valino Servicio de Oftalmología, Hospital Universitario Infanta Leonor, Madrid, Spain
a r t i c l e
i n f o
a b s t r a c t
Article history:
Clinical cases: We report the cases of 3 young patients who were seen in our hospital with
Received 20 September 2012
the diagnosis of iris mammillations. Two of them were bilateral without familial association.
Accepted 12 April 2013
The third child had iris mammillations as part of an ocular melanocytosis.
Available online 26 September 2014
None of them had abnormalities in the posterior segment or the pachymetry. Discussion: The discovery of iris mammillations during an examination requires a long-term
Keywords:
follow-up of these patients due to its association with uveal melanoma. This is required
Mammillations
whether it is a casual discovery or in the presence of ocular melanocytosis.
Melanocytosis
˜ © 2012 Sociedad Espanola de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.
Nevus de Ota Uveal melanoma Iris hyperpigmentation
Mamelones de iris. A propósito de tres casos r e s u m e n Palabras clave:
Casos clínicos: Se presentan tres pacientes jóvenes vistos en nuestro servicio con diagnóstico
Mamelones
de mamelones de iris. Dos de los casos eran bilaterales, sin asociación familiar. El tercero
Melanocitosis
presentó mamelones de iris en el contexto de una melanocitosis ocular. Ninguno mostró
Nevus de Ota
asociación con anomalías en el polo posterior o en la paquimetría.
Melanoma uveal
Discusión: La presencia de mamelones de iris en una exploración, tanto como hallazgo casual
Hiperpigmentación de iris
como en el contexto de una melanocitosis ocular, requiere del seguimiento a largo plazo de estos pacientes por su posible asociación con el melanoma de úvea. ˜ © 2012 Sociedad Espanola de Oftalmología. Publicado por Elsevier España, S.L.U. Todos los derechos reservados.
Introduction Mammillations are congenital anomalies of the iris characterized by dome-shaped elevations of the iris, regularly
distributed over a part or the entire surface of a characteristically velvety iris (without surface crypts). Iris mammillations appear isolated or associated with ocular or dermal-ocular (Nevus of Otta [sic: Ota]) melanocytosis, in such cases with increased risk of uveal melanoma.
夽
˜ M. Mamelones de iris. Please cite this article as: Sánchez Marugán B, Acebes García M, García Hinojosa J, León Cabello MJ, Casal Valino A propósito de tres casos. Arch Soc Esp Oftalmol. 2014;89:279–281. ∗ Corresponding author. E-mail address: [email protected] (B. Sánchez Marugán). ˜ 2173-5794/$ – see front matter © 2012 Sociedad Espanola de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.
280
a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(7):279–281
Fig. 1 – Details of the iris showing iris mammillations on a hyperpigmented iris of patient in case 1.
Fig. 3 – Details of the iris of right eye (left panel) and left eye (right panel) of patient in case 3.
Therefore, it is important to know these lesions for long-term patient follow-up, preventing uncontrolled development of this severe ocular complication. This is a report of three cases of children who were seen in our department and had iris mammillations during eye examination.
to 170◦ ): 1 and left eye (+1.75; −0.75 to 5◦ ): 1. The previous biomicroscopy has bilateral mammillations of the iris (Fig. 3). Fundus examination is normal. Pachymetry values for her right eye were 550, and 540 for the left one.
Case 1 5-year-old male patient, Caucasian, seen for pigmentation in the sclera of his left eye, from neonatal period. On examination the patient showed 20/25 uncorrected visual acuity in both eyes. Previous biomicroscopy shows scleral pigmentation and iris hyperpigmentation with elevated pigmented nodules and regularly distributed in the left eye (iris mamelons) (Fig. 1). Fundus examination showed no changes. Intraocular pressure could not be measured due to lack of cooperation of the patient; however, it was possible to measure corneal thickness; results were 564 in the right eye and 567 on the left.
Case 2 8-year-old Hispanic female patient who is seen for reduced sight. Examination reveals: AV: right eye (−2.00; 180◦ ): 1 and left eye (+1.00, −4.00 to 5◦ ): 0.3. Biomicroscopy shows bilateral iris mammillations diffusely distributed (Fig. 2). Fundus examination was within normal limits. Pachymetry was around 540–545 , in the right and left eye respectively.
Case 3 16-year-old female patient, white race, who comes in for headaches. Examination reveals: AV: right eye (+1.75; −0.50
Fig. 2 – Details of the iris of right eye (left panel) and left eye (right panel) of patient in case 2.
Discussion Iris mammillations are raised nipple-shaped protrusions that fully or partially cover the surface of the iris. The term was coined by Coats in 1912 to describe the iris of a subject with ocular melanosis. They are characterized by being raised lesions, soft on their surface and variously dome-shaped, conical or stellate at their base, regularly covering the surface of all or part of the iris. When the surface of the iris is fully covered with mammillations, these become larger as they approach the pupillary margin. The iris where they are located is usually more pigmented, appearing as heterochromia in subjects with unilateral mammillations.1 Congenital and sporadic lesions have been reported in family cases with autosomal dominant or recessive, polygenic and multifactorial heredity. Most of the cases reported in literature are unilateral, although there are also bilateral cases.2 Iris mammillations appear more frequently in subjects with ocular melanocytosis (hyperpigmentation of uveal tissue, iris, sclera, etc.) but can also occur without other coexisting ocular or dermal pigmentary conditions. It has been suggested that iris mammillations could be either a low expression of ocular melanocytosis or an isolated disorder.3 If we consider them as a variant of ocular melanocytosis, then we can also consider them as premalignant lesions; however, in most reported cases associated with an intraocular tumor, a preexisting melanocytosis is the most important etiological factor. Differential diagnosis includes Lisch nodules (neurofibromatosis type I), tapioca melanoma, granulomatous inflammation of the iris and the Cogan-Reese syndrome.4,5 Lisch nodules have a cushiony appearance, are unevenly distributed and have a color lighter than the iris surface. They tend to be larger than mammillations and are more variable in size and number. They look brown in blue or green iris, and paler than underlying iris in brown eyes. By contrast, iris mammillations are the same color of the underlying iris in brown eyes and are a medium brown on blue or green eyes. Tapioca melanoma is a pale or amelanotic tumor. It grows slowly and is typical in young patients.6 Cogan-Reese syndrome is characterized
a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(7):279–281
by diffuse nodules, peripheral anterior synechiae, iris atrophy and dyscoria. Granulomatous inflammatory nodules occur in uveitis as Koeppe and Busacca nodules. Prevalence of iris mammillations in the general population is unknown. We can ensure that these lesions are rare, probably underdiagnosed,2 to be considered as a sign of ocular melanocytosis and be followed by the long-term risk posed by developing uveal melanoma.
Conflict of interest Authors declare having no conflict of interest.
references
1. Ragge NK, Acheson J, Murphree AL. Iris mammillations significance and associations. Eye. 1996;10:86–91.
281
2. Ozdamar Y. A case of bilateral iris mammillations with unusual papillary appearance and pesudohypertension. Clin Exp Optom. 2007;90:471–3. 3. Gündünz K, Shields CL, Shields JA, Eagle Jr RC, Singh AD. Iris mammillations as the only sign of ocular melanocitosis in a child with choroidal melanoma. Arch Opthalmol. 2000;118:716–7. 4. Ragge NK, Acheson J, Murphree AL. Iris mammillations: significance and associations. Eye. 1996;10:86–91. 5. Swann PG. Iris mammillations in ocular melanocytosis. Clin Exp Optom. 2001;84:35–8. 6. Viestenz A, Conway RM, Kuchle M. Tapioca melanoma of the iris mimicking a vascular tumour: a clinicopathological correlation. Clin Exp Ophthalmol. 2004;32:327–30.