Objective: This study aimed to describe the clinical and histopathologic findings in four patients with complex limbal choristomas associated with linear nevus sebaceous syndrome (LNSS), a rare disorder including nevus sebaceous, seizures, and mental retardation, and often accompanied by ocular anomalies.
Design: Small observational case series.
Methods: A retrospective review of the clinical and histopathologic records of four patients.
Results: Each of four patients had complex limbal choristomas in the setting of clinical and histopathologic LNSS. The limbal choristomas were multiple in three patients and bilateral in two patients. Most choristomas involved the superotemporal limbus (6 of 10), although nasal (3 of 10) and inferior (1 of 10) limbal tumors also were present. Three patients had significant corneal astigmatism or involvement of the central cornea requiring surgical removal of their choristomas, one accompanied by a lamellar keratoplasty and another accompanied by two consecutive penetrating keratoplasties. Each graft eventually vascularized, reducing vision. One patient's vision was limited by amblyopia and another by occipital cortical dysgenesis with visual impairment. Histopathologic examination of the excised choristomas showed foci of lacrimal gland (3 of 4 patients), adipose tissue (3 of 4), neural tissue (1 of 4), cartilage (1 of 4), lymphoid follicles (1 of 4), skin adnexal tissue (1 of 4), and smooth muscle (1 of 4). Other associated ocular findings included an eyelid mass (1 of 4), colobomas of the eyelid (3 of 4), colobomas of the choroid and retina (2 of 4), nonparalytic strabismus (2 of 4), scleral ectasia (1 of 4), partial oculomotor palsy with ptosis and anisocoria (1 of 4), microphthalmia (1 of 4), hypertelorism (1 of 4), and cortical visual impairment (1 of 4).
Conclusions: Complex limbal choristomas, although rare, can occur in the setting of LNSS and can be associated with multiple ocular and systemic abnormalities. Visual prognosis appears poor in most cases despite aggressive management.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/S0161-6420(98)98029-0 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Congenital limbal dermoid in a 10-year-old male.
Pan Afr Med J
April 2023
Department of Ophthalmology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher education and Research, Sawangi (Meghe), Wardha, Maharashtra, India.
Encephalocraniocutaneous Lipomatosis Associated with Orbital Cyst: A Variant or New Entity?
Turk J Ophthalmol
February 2021
University of Maiduguri Teaching Hospital, Department of Pediatrics, Maiduguri, Nigeria.
Article Synopsis
- Encephalocraniocutaneous lipomatosis (ECCL) is a rare congenital disorder affecting neurocutaneous tissues like the brain, eyes, and skin, with an unknown cause.
- A case is presented involving a 3-year-old boy from Sub-Saharan Africa who has bilateral eye issues, including cystic swelling and microphthalmia, along with atrophy of the right cerebral hemisphere and an orbital cyst.
- The findings align with diagnostic criteria for ECCL established in 2009, highlighting the importance of neuroimaging for accurate diagnosis and management of potential complications.
Acellular Porcine Corneal Stroma May Not Be Optimal for Peripheral Keratoplasty: Reports of 2 Cases.
Cornea
April 2021
State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangzhou, China.
Purpose: Although acellular porcine corneal stroma (APCS) is a promising alternative to the human donor cornea for lamellar keratoplasty, here, we report 2 patients who exhibited persistent epithelial defects and sterile keratolysis after APCS transplantation to treat peripheral corneal diseases.
Methods: Two patients with different peripheral corneal diseases underwent lamellar keratoplasty by using D-shaped lamellar APCS as graft materials. Standard keratoplasty postoperative treatments, including topical antibiotic-corticosteroid, tacrolimus, and artificial tears, were applied.
Geometric Profiling of Corneal Limbal Dermoids for the Prediction of Surgical Outcomes.
Cornea
October 2020
Laboratory of Ocular Regenerative Medicine and Immunology, Seoul Artificial Eye Center, Seoul National University Hospital Biomedical Research Institute, Seoul, Korea.
Purpose: To search for novel geometric parameters for corneal limbal dermoids that enable the prediction of clinical and surgical outcomes.
Methods: We reviewed the medical records and anterior segment photographs of 85 eyes of 85 patients with corneal limbal dermoids that had been surgically excised. Patients were assigned to the following 4 cohorts (multiple assignments allowed): postoperative visual acuity (cohort 1, n = 65), amblyopia (cohort 2, n = 67), postoperative scar formation (cohort 3, n = 51), and preoperative spontaneous progression of dermoids (cohort 4, n = 39).
Epibulbar complex choristoma with simultaneous involvement of eyelid: a case report.
BMC Ophthalmol
November 2019
Department of Ophthalmology, National Taiwan University Hospital, No 7, Chung-Shan S. Rd, Taipei 100, Taiwan.
Background: Epibulbar complex choristoma, a rare congenital epibulbar tumor, has many diverse forms. Reviewing the literature, it can present clinically as either a circumferential or isolated epibulbar mass, limbal tumor, lateral canthal mass, aggregate of ectopic cilia in the upper eyelid, eyelid mass mimicking chalazion, or lacrimal caruncle mass. The management depends on the extent of involvement, the risk of amblyopia, and cosmetic concerns.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!