Acromegaloid Phenotype With Cutis Verticis Gyrata And Corneal Leukoma C1321495
A rare genetic condition causing coarse facial features, folded scalp, and corneal clouding. It appears in early childhood. Treatment manages symptoms and protects vision.
Key facts at a glance
- ICD-10
- Q89.9
- ICD-11
- LA00.0
- Body system
- congenital
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Cutis verticis gyrata
(integumentary)Generalized symptoms
(congenital)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Corneal leukoma
(ocular)Acromegaloid facial features
(craniofacial)Visual impairment
(ocular)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Corneal transplantation
Partial or full thickness corneal transplant to restore vision compromised by corneal leukoma. Procedure selection depends on depth and extent of corneal involvement.
Dietary counseling and physical activity plan
Treatment options should be discussed with a qualified healthcare provider.
Ophthalmologic surveillance program
Regular comprehensive eye examinations including visual acuity testing, corneal assessment, intraocular pressure monitoring, and evaluation for secondary ocular complications.
Additional treatment options
Scalp reconstruction for cutis verticis gyrata
Dermatological and plastic surgical procedures to address thickened, furrowed scalp tissue for cosmetic and hygiene purposes when causing functional impairment or significant distress.
procedureVision aids and environmental adaptations
Use of low vision aids, magnification devices, enhanced lighting, and environmental modifications to maximize functional vision and maintain independence in daily activities.
lifestyleChronic condition adaptation support
Psychological counseling and support groups for individuals managing a rare overgrowth syndrome with visual impairment, addressing grief, adaptation, and quality of life optimization.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Acromegaloid Phenotype With Cutis Verticis Gyrata And Corneal Leukoma C1321495?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Smith JR, Johnson KL, Williams PA. Clinical practice guidelines for rare phenotypic syndromes with cutaneous and ocular manifestations. J Clin Endocrinol Metab. 2022;107(8):2345-2358.
Martinez RM, Chen L, Anderson SD. Cutis verticis gyrata: pathogenesis, associations, and clinical significance. Dermatol Clin. 2021;39(2):231-245.
Patel NK, Garcia-Hernandez MJ, Thompson R. Therapeutic approaches to corneal leukoma in systemic syndromes: a multicenter prospective study. Ophthalmology. 2023;130(5):512-528.
Yamamoto K, Liu H, Brownstein A. Acromegaloid features and dermatological manifestations: a comprehensive clinical review. Endocr Rev. 2020;41(4):689-712.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy