Subject(s)
Blepharoptosis , Eyelid Neoplasms , Humans , Eyelid Neoplasms/complications , Eyelid Neoplasms/diagnosis , EyelidsABSTRACT
BACKGROUND: High-risk (HR) Human papillomavirus (HPV) has been implicated in pathogenesis of squamous cell carcinomas (SCC) at several sites with mucocutaneous junctions, including the head and neck. SCC is the second most common eyelid malignancy. However, its association with transcriptionally active HR-HPV has not been adequately studied. METHODS: Two index cases of eyelid HPV-associated SCC are described in detail. A retrospective cohort of eyelid SCC was examined for p16 immunoexpression. Cases demonstrating p16 positivity or equivocal staining were subjected to high-risk HPV mRNA in situ hybridization (ISH). Quantitative real-time PCR (qPCR) was performed in mRNA ISH-positive cases for HPV genotyping. RESULTS: The two index patients were older adult females, with upper eyelid tumours. On histology, both tumours were non-keratinizing SCC with trabecular and nested architecture reminiscent of oropharyngeal HPV-associated non-keratinizing SCC, prompting p16 immunohistochemistry, which was positive. HR-HPV mRNA ISH was positive, and qPCR detected HPV16 in both cases. Three of 20 (15%) archival cases showed p16 immunopositivity and two (10%) showed equivocal staining. However, mRNA ISH was negative. All cases showing p16 immunostaining and lacking HR-HPV were keratinizing SCCs. Thus, 9% of all eyelid SCC examined demonstrated HR-HPV. CONCLUSION: The prevalence of HR-HPV in eyelid SCC is low in Indian patients. HPV-associated SCC may mimic commoner eyelid carcinomas as it lacks overt keratinization. In basaloid-appearing eyelid carcinomas, p16 immunopositivity should be followed by reflex HR-HPV mRNA ISH, as p16 immunohistochemistry alone has low specificity. The prognostic role, if any, of HPV association needs further evaluation.
Subject(s)
Carcinoma, Squamous Cell , Eyelid Neoplasms , Papillomavirus Infections , Female , Humans , Aged , Immunohistochemistry , Eyelid Neoplasms/diagnosis , Eyelid Neoplasms/complications , RNA, Messenger , Papillomavirus Infections/diagnosis , Retrospective Studies , Carcinoma, Squamous Cell/diagnosis , Carcinoma, Squamous Cell/pathology , In Situ Hybridization , Eyelids/chemistry , Eyelids/pathology , Cyclin-Dependent Kinase Inhibitor p16/analysis , Papillomaviridae/genetics , Biomarkers, Tumor/analysisABSTRACT
PURPOSE: To investigate the risk of second primary neoplasms (SPNs) after primary sebaceous carcinoma of the eyelid (SCE). METHODS: Data on patients diagnosed with primary SCE as their first malignancy were extracted from the Surveillance, Epidemiology, and End Results database from 2000 to 2016. Observed-to-expected ratios of SPNs were calculated to estimate standardized incidence ratios (SIRs). Patients were compared with a reference population (RP) matched for age, gender, and race. RESULTS: Five hundred fifty-nine patients with primary SCE were identified, 16% of whom developed SPNs. SCE patients displayed a 61% increased risk of developing SPNs compared with the RP (p < 0.001). Overall, the risk of SPNs of the lungs (SIR = 1.82; p < 0.05), pancreas (SIR = 2.94; p < 0.05), salivary glands (SIR = 41.65; p < 0.001), and skin (SIR = 8.33; p < 0.05) was elevated. Only non-Hispanic Whites were at an increased risk (SIR = 1.51; p < 0.05). Patients 40-54 years old at the time of diagnosis were at the highest risk of developing SPNs compared with the RP (SIR = 3.15; p < 0.05). Women with SCE experienced an increased risk of breast cancer (SIR = 3.6; p < 0.05) and chronic lymphocytic leukemia (SIR = 8.8; p < 0.01). CONCLUSION: SCE patients are more likely to develop SPNs of the lungs, pancreas, salivary gland, and skin than the RP. Forty to fifty-four years old Caucasian patients are at the highest risk. Women are at an increased risk of developing breast malignancies and chronic lymphocytic leukemia. Clinicians should be cognizant of these risks when managing SCE patients.
Subject(s)
Adenocarcinoma, Sebaceous , Bone Neoplasms , Breast Neoplasms , Eyelid Neoplasms , Leukemia, Lymphocytic, Chronic, B-Cell , Neoplasms, Second Primary , Sebaceous Gland Neoplasms , Skin Neoplasms , Adenocarcinoma, Sebaceous/epidemiology , Adult , Eyelid Neoplasms/complications , Eyelid Neoplasms/epidemiology , Eyelids , Female , Humans , Incidence , Middle Aged , Neoplasms, Second Primary/epidemiology , Neoplasms, Second Primary/etiology , Risk Factors , Sebaceous Gland Neoplasms/epidemiology , Skin Neoplasms/complicationsABSTRACT
Oncocytoma of the eyelid is a rare neoplasm. Oncocytoma associated with an ocular surface squamous neoplasm, namely conjunctival intraepithelial neoplasia, is very hard to find in the literature. Herein we report a case of a 53-year-old male who presented with a swelling in the right lower lid over the last 6 years, along with a growth in the conjunctiva of the same eye for the last 2 years and encroaching upon the cornea for the last 4 months. Excision biopsy of the lower lid mass showed histopathological features consistent with oncocytoma. The conjunctival tissue revealed conjunctival intraepithelial neoplasia 3 (severe dysplasia). This case documents a rare synchronous dual ocular neoplasia, a very unlikely coexistence of oncocytoma with conjunctival intraepithelial neoplasia.
Subject(s)
Humans , Male , Middle Aged , Papilloma/pathology , Pterygium , Carcinoma in Situ , Adenoma, Oxyphilic/complications , Eyelid Neoplasms/complicationsABSTRACT
Chronic eyelid edema may be a symptom of different disease. The most common are autoimmune diseases such as orbital pseudotumor, vasculitis, sarcoidosis, or impaired vascular or lymphatic drainage. Rarely has it been reported as the sole manifestation of the lymphoma. Eyelid lymphoma is a special clinical entity in the spectrum of hematological malignancies. Here we present our clinical experience with eyelids lymphomas. First case is a 76-year-old female patient with bilateral edema of upper eyelid non-responding to anti-inflammatory therapy. Histological examination diagnosed mantle cells lymphoma. In the second case, 58-year-old patient was diagnosed with solitary unilateral tumor of the lower eyelid, where primary biopsy was ordered and diagnosis of MALT lymphoma was established after histological examination. In both cases, it was not solitary eyelid tumor, but systemic disease with multiple lymphadenopathy and bone marrow infiltration were found in follow-up examinations. Subsequently, patients care was given to the hemato-oncologist.
Subject(s)
Eyelid Neoplasms , Lymphoma, B-Cell, Marginal Zone , Adult , Aged , Biopsy , Edema/diagnosis , Edema/etiology , Eyelid Neoplasms/complications , Eyelid Neoplasms/diagnosis , Eyelids , Female , Humans , Lymphoma, B-Cell, Marginal Zone/complications , Lymphoma, B-Cell, Marginal Zone/diagnosis , Middle AgedSubject(s)
Edema/etiology , Eyelid Neoplasms/diagnosis , Hemangiosarcoma/diagnosis , Scalp , Skin Neoplasms/diagnosis , Aged, 80 and over , Eyelid Neoplasms/complications , Eyelids , Fatal Outcome , Hemangiosarcoma/complications , Hemangiosarcoma/pathology , Humans , Male , Skin Neoplasms/complications , Skin Neoplasms/pathologySubject(s)
Eyelid Neoplasms/diagnosis , Nevus of Ota/diagnosis , Skin Neoplasms/diagnosis , Child, Preschool , Eyelid Neoplasms/complications , Eyelid Neoplasms/pathology , Humans , Male , Mouth/pathology , Nevus of Ota/complications , Nevus of Ota/pathology , Pigmentation Disorders/complications , Pigmentation Disorders/diagnosis , Pigmentation Disorders/pathology , Scleral Diseases/complications , Scleral Diseases/diagnosis , Scleral Diseases/pathology , Skin Neoplasms/complications , Skin Neoplasms/pathologyABSTRACT
ABSTRACT Purpose: To determine the long-term functional and cosmetic outcomes in patients who underwent modified Hughes procedure with different types of anterior lamellar reconstruction for lower eyelid defects. Methods: This study included 58 patients who had undergone a modified Hughes flap for reconstruction of lower eyelids after tumor excision within a 10-year period. Data regarding patient demographics, size of eyelid defect, tumor pathology, surgical techniques, functional and cosmetic outcomes, and complications were recorded. Postoperative complications were evaluated according to the type of anterior lamella reconstruction (i.e., advancement flap or free skin graft). Multivariate logistic regression analysis was performed to identify risk factors affecting the success of the procedure. Results: The average size of the lower eyelid defect was 22 ± 6.3 mm (range: 11-30 mm). The anterior lamella was reconstructed with advancement flaps and full-thickness skin grafts in 36 (58.6%) and 24 (41.4%) patients, respectively. Mean follow-up time was 23.6 ± 11.9 months. Postoperative complications included trichiasis (three patients; 5.2%), ectropion (two patients; 3.0%), flap necrosis (one patient; 1.7%), flap dehiscence (one patient; 1.7%), infection (one patient; 1.7%), and eyelid margin erythema (one patient; 1.7%). The rates of complication and secondary surgery were similar among the different types of anterior lamellar reconstruction (p=768 and p=0.139, respectively). Success of the modified Hughes procedure was not significantly affected by any of the identified risk factors (p>0.05). Functional and cosmetic outcomes were 96.6% and 94.8%, respectively. Conclusion: Modified Hughes procedure is a safe and effective option for the reconstruction of small and large defects of the lower eyelid, regardless of the type of anterior lamella reconstruction (i.e., advancement flap or skin graft).
RESUMO Objetivo: Determinar os resultados funcionais e cosméticos a longo prazo de pacientes submetidos ao procedimento de Hughes modificado com diferentes tipos de reconstrução lamelar anterior para defeitos palpebrais inferiores. Métodos: Este estudo incluiu 58 pacientes que foram submetidos a um retalho de Hughes modificado para reconstrução das pálpebras inferiores após excisão do tumor durante um intervalo de 10 anos. Dados referentes à demografia dos pacientes, tamanho do defeito palpebral, patologia tumoral, técnicas cirúrgicas, resultados funcionais e cosméticos e complicações foram registrados. As complicações pós-operatórias foram avaliadas de acordo com o tipo de reconstrução da lamela anterior (ou seja, retalho de avanço ou enxerto de pele livre). A análise de regressão logística multivariada foi realizada para identificar os fatores de risco que afetam o sucesso do procedimento. Resultados: O tamanho médio do defeito da pálpebra inferior foi de 22 ± 6,3 mm (11-30 mm). A lamela anterior foi reconstruída com retalhos de avanço e enxertos de pele de espessura total em 36 (58,6%) e 24 (41,4%) pacientes, respectivamente. O tempo médio de acompanhamento foi de 23,6 ± 11,9 meses. Complicações pós-operatórias incluíram triquíase (três pacientes: 5,2%), ectrópio (dois pacientes: 3%), necrose de retalho (um paciente: 1,7%), deiscência de retalho (um paciente: 1,7%), infecção (um paciente: 1,7%) e eritema na margem palpebral (um paciente: 1,7%). As taxas de complicação e de cirurgia secundária foram semelhantes entre os diferentes tipos de reconstrução lamelar anterior (p=768 e p=0,139, respetivamente). O sucesso do procedimento de Hughes modificado não foi significativamente afetado por nenhum dos fatores de risco identificados (p>0,05). Resultados funcionais e cosméticos foram de 96,6% e 94,8%, respetivamente. Conclusão: O procedimento de Hughes modificado é uma opção segura e eficaz para a reconstrução de pequenos e grandes defeitos da pálpebra inferior, independentemente do tipo de reconstrução da lamela anterior (ou seja, retalho de avanço ou enxerto de pele).
Subject(s)
Humans , Male , Female , Middle Aged , Aged , Aged, 80 and over , Carcinoma, Basal Cell/surgery , Skin Transplantation/methods , Eyelid Neoplasms/surgery , Postoperative Complications , Surgical Flaps , Carcinoma, Basal Cell/complications , Retrospective Studies , Blepharoplasty/methods , Ectropion/surgery , Eyelid Neoplasms/complications , Eyelids/surgeryABSTRACT
PURPOSE: To determine the long-term functional and cosmetic outcomes in patients who underwent modified Hughes procedure with different types of anterior lamellar reconstruction for lower eyelid defects. METHODS: This study included 58 patients who had undergone a modified Hughes flap for reconstruction of lower eyelids after tumor excision within a 10-year period. Data regarding patient demographics, size of eyelid defect, tumor pathology, surgical techniques, functional and cosmetic outcomes, and complications were recorded. Postoperative complications were evaluated according to the type of anterior lamella reconstruction (i.e., advancement flap or free skin graft). Multivariate logistic regression analysis was performed to identify risk factors affecting the success of the procedure. RESULTS: The average size of the lower eyelid defect was 22 ± 6.3 mm (range: 11-30 mm). The anterior lamella was reconstructed with advancement flaps and full-thickness skin grafts in 36 (58.6%) and 24 (41.4%) patients, respectively. Mean follow-up time was 23.6 ± 11.9 months. Postoperative complications included trichiasis (three patients; 5.2%), ectropion (two patients; 3.0%), flap necrosis (one patient; 1.7%), flap dehiscence (one patient; 1.7%), infection (one patient; 1.7%), and eyelid margin erythema (one patient; 1.7%). The rates of complication and secondary surgery were similar among the different types of anterior lamellar reconstruction (p=768 and p=0.139, respectively). Success of the modified Hughes procedure was not significantly affected by any of the identified risk factors (p>0.05). Functional and cosmetic outcomes were 96.6% and 94.8%, respectively. CONCLUSION: Modified Hughes procedure is a safe and effective option for the reconstruction of small and large defects of the lower eyelid, regardless of the type of anterior lamella reconstruction (i.e., advancement flap or skin graft).
Subject(s)
Carcinoma, Basal Cell/surgery , Eyelid Neoplasms/surgery , Skin Transplantation/methods , Aged , Aged, 80 and over , Blepharoplasty/methods , Carcinoma, Basal Cell/complications , Ectropion/surgery , Eyelid Neoplasms/complications , Eyelids/surgery , Female , Humans , Male , Middle Aged , Postoperative Complications , Retrospective Studies , Surgical FlapsSubject(s)
Eyelid Neoplasms/diagnosis , Eyelids/pathology , Sarcoma, Kaposi/diagnosis , Coinfection/diagnosis , Coinfection/virology , Diagnosis, Differential , Eyelid Neoplasms/complications , Eyelid Neoplasms/virology , Eyelids/diagnostic imaging , Eyelids/virology , Haiti , Heart Transplantation , Herpesvirus 6, Human/isolation & purification , Herpesvirus 6, Human/physiology , Herpesvirus 8, Human/isolation & purification , Herpesvirus 8, Human/physiology , Humans , Immunocompromised Host , Male , Middle Aged , Roseolovirus Infections/complications , Roseolovirus Infections/diagnosis , Roseolovirus Infections/pathology , Roseolovirus Infections/virology , Sarcoma, Kaposi/complications , Sarcoma, Kaposi/pathology , Sarcoma, Kaposi/virology , Transplant RecipientsABSTRACT
Purpose: To examine the patency or secondary obstruction of the lacrimal drainage system in patients with a peripunctal tumour.Methods: This retrospective, observational, and comparative study included 10 patients with a peripunctal tumour. Lacrimal probing and syringing in all patients and dacryoendoscopic examinations in 5 patients were performed to check for patency of the lacrimal drainage system. Tear meniscus height (TMH) was measured bilaterally in the upper and lower eyelids using anterior segment optical coherence tomography and compared in relation to the affected side using one-way ANOVA.Results: All patients did not complain of epiphora. Probing gave a hard stop and irrigation fluid passed into the nose. A patent punctum/canaliculus was also confirmed by dacryoendoscopy in all of the 5 patients. TMH was not significantly different among the sides (P = .900).Conclusions: This study shows patency of the lacrimal drainage system in patients with a peripunctal tumour and no significant difference in TMH among the sides, resulting in absence of epiphora in all patients.
Subject(s)
Eyelid Neoplasms/complications , Lacrimal Duct Obstruction/etiology , Eyelid Neoplasms/diagnostic imaging , Eyelid Neoplasms/physiopathology , Female , Humans , Lacrimal Duct Obstruction/diagnostic imaging , Lacrimal Duct Obstruction/physiopathology , Male , Middle Aged , Retrospective Studies , Tears , Therapeutic Irrigation , Tomography, Optical CoherenceABSTRACT
OBJECTIVES: To identify clinical factors associated with complications of periocular infantile hemangioma (IH) and monitor improvement in complication rates post-treatment. METHODS: Retrospective cohort study. Eighty-nine patients diagnosed with periocular IH at a pediatric dermatology clinic of a tertiary care center between 2001 and 2013 were included with parental approval. Parents were interviewed by telephone between July and September of 2015, then again in January 2018 to inquire about ophthalmologic follow-up. Electronic medical records were reviewed from January 2001 through January 2018. RESULTS: Sixty percent of patients demonstrated ocular sequelae, including astigmatism (33%), visual axis obstruction (29%), nasolacrimal duct obstruction (7%), ptosis (4%), amblyopia (3%), and strabismus (1%). Compared with superficial IH, deep and mixed IH had higher odds, 3.4 (P = 0.025) and 3.8 (P = 0.034), respectively, of developing ocular sequelae. All patients with astigmatism prior to involution of IH received systemic therapy, with a significant post-treatment decrease in the proportion of patients with astigmatism (40% to 18%, P = 0.027). Three-quarters of patients experienced complete IH involution by time of enrollment in kindergarten. Fifty-one (57.3%) patients received formal ophthalmologic evaluation confirmed through chart review or phone interview, with average follow-up duration of 51.2 months (range: 1.9, 99.3). CONCLUSION: Deep and mixed IH were more likely to demonstrate ocular complications than superficial IH. Rate of astigmatism decreased with systemic therapy. Our study suggests that patients with periocular IH have a lower rate of amblyopia now compared with the prepropranolol era and emphasizes the importance of early treatment of periocular IH to prevent permanent visual sequelae.
Subject(s)
Eye Diseases/etiology , Eyelid Neoplasms/complications , Hemangioma/complications , Orbital Neoplasms/complications , Eye Diseases/therapy , Eyelid Neoplasms/therapy , Female , Hemangioma/therapy , Humans , Infant , Male , Orbital Neoplasms/therapy , Retrospective StudiesSubject(s)
Anodontia/pathology , Eccrine Glands/abnormalities , Eccrine Porocarcinoma/pathology , Eyelid Neoplasms/pathology , Hypotrichosis/pathology , Keratoderma, Palmoplantar/pathology , Sweat Gland Neoplasms/pathology , Aged , Anodontia/complications , Biopsy , Eccrine Glands/pathology , Eccrine Porocarcinoma/surgery , Eyelid Neoplasms/complications , Female , Humans , Hypotrichosis/complications , Keratoderma, Palmoplantar/complications , Keratoderma, Palmoplantar/etiology , Margins of Excision , Surgical Flaps/surgery , Sweat Gland Neoplasms/surgery , Treatment Outcome , Wound Healing/physiologyABSTRACT
Basal cell carcinoma (BCC) is the most common type of cancer located in the periocular area. We will present the clinical case of a 63-year-old male patient who was admitted to the 2nd Clinic of Neurosurgery, "Prof. Dr. Nicolae Oblu" Emergency Clinical Hospital, Iasi, Romania, for an ulcerated tumor of about 0.8×0.7 cm in diameter with rolled edges and central necrosis in the upper eyelid with orbital invasion. According to the patient's personal history, he also underwent Cortisone treatment for dermatomyositis. The magnetic resonance imaging (MRI) scan revealed behind the cutaneous flap, a lesion with 15∕38∕19 mm anteroposterior (AP)∕transverse (T)∕craniocaudal (CC) diameters. The surgeons made the excision of the tumor together with the eyelid remnants, and the left orbit exenteration defect. The histopathological exam of the surgical samples revealed an ulcerated epithelial tumor having its origin in the eyelid epidermis and invading all the thickness of the eyelid toward the palpebral conjunctiva, but also the orbital tissue. Immunohistochemical studies showed positive staining for cytokeratin (CK) AE1∕AE3, CK5∕6, and CK17, but not for CK7. The Ki-67 labeling index was 12%, suggesting a moderate proliferative activity. The final pathological diagnosis was mixed (nodular and morpheic) eyelid BCC infiltrative into the orbital tissue. Although BCC of the upper eyelid is a rare cancer and generally has a low recurrence risk, in the case of a patient undergoing Cortisone treatment for an autoimmune disease, the tumor may grow more rapidly by invading the neighboring tissues including orbit.
Subject(s)
Carcinoma, Basal Cell/complications , Eyelid Neoplasms/complications , Orbit Evisceration/methods , Carcinoma, Basal Cell/pathology , Eyelid Neoplasms/pathology , Humans , Male , Middle Aged , Orbit/pathologyABSTRACT
Ocular myiasis is a rare disease with invasion of the lids, conjunctiva, cornea and, rarely, orbit or globe of the mammalian eye by dipteral larvae.We report a case of a 74-year-old woman with chronic, infiltrating, ulcerative lesion of the left lower eyelid, which was confirmed as basal cell carcinoma subsequently, having ophthalmomyiasis.She was a destitute, living in unhygienic environmental conditions and having poor general health. She developed permanent blindness as a result of ophthalmomyiasis of the left eye. Proper wound care, hygiene and adequate nutrition helped her recover from the illness.
Subject(s)
Carcinoma, Basal Cell/complications , Eyelid Neoplasms/complications , Myiasis/complications , Skin Neoplasms/complications , Aged , Ampicillin/therapeutic use , Anti-Bacterial Agents/therapeutic use , Carcinoma, Basal Cell/diagnosis , Carcinoma, Basal Cell/pathology , Carcinoma, Basal Cell/surgery , Debridement , Eye Enucleation , Eyelid Neoplasms/diagnosis , Eyelid Neoplasms/pathology , Eyelid Neoplasms/surgery , Female , Humans , Insecticides/therapeutic use , Ivermectin/therapeutic use , Myiasis/therapy , Skin Neoplasms/diagnosis , Skin Neoplasms/pathology , Skin Neoplasms/surgery , Sulbactam/therapeutic use , Therapeutic Irrigation , Tomography, X-Ray ComputedABSTRACT
Over a 2-year period, swellings of all 4 eyelid margins developed in a 32-year-old woman and was accompanied by complete loss of eyelashes. An inflammatory dermatologic condition was considered the most likely cause. A full-thickness right lower eyelid biopsy revealed a multinodular lymphoid tumor at the eyelid margin which immunophenotypically and genetically was diagnosed as an extranodal marginal zone lymphoma. The mode of presentation of the disease was considered to be most unusual, as was its B cell lineage, since the majority of primary cutaneous lymphomas are of T-cell origin. Systemic workup demonstrated bilateral involvement of the external auditory canals.
Subject(s)
Eyelashes/pathology , Eyelid Neoplasms/complications , Hair Diseases/etiology , Lymphoma, B-Cell, Marginal Zone/complications , Adult , Female , HumansABSTRACT
An upper lid eccrine hidrocystoma presenting as early childhood progressive ptosis is very rare. We present a 9-year-old female child with droopy right upper lid since birth and progressive increase in symptoms. She had right upper lid ptosis (marginal reflex distance 1 of -1 mm) with fair levator function (8 mm) and abnormal cystic change on the conjunctival side. Computerized tomography imaging delineated the well-defined cystic lesion with homogeneous cavity with no contrast enhancement. Following the cyst excision, a giant eccrine hidrocystoma measuring 25 mm × 15 mm was removed, the largest reported in pediatric eyes. The case demonstrates the possibility of giant lid eccrine hidrocystomas presenting as progressive ptosis at a pediatric age and the need for early surgical intervention to prevent amblyopia.
Subject(s)
Blepharoptosis/etiology , Eyelid Neoplasms/complications , Eyelids/pathology , Hidrocystoma/complications , Neoplasm Staging , Sweat Gland Neoplasms/complications , Blepharoptosis/diagnosis , Blepharoptosis/surgery , Child , Diagnosis, Differential , Eyelid Neoplasms/diagnosis , Eyelid Neoplasms/surgery , Eyelids/surgery , Female , Hidrocystoma/diagnosis , Hidrocystoma/surgery , Humans , Ophthalmologic Surgical Procedures/methods , Sweat Gland Neoplasms/diagnosis , Sweat Gland Neoplasms/surgeryABSTRACT
Schöpf-Schulz-Passarge syndrome (SSPS) is thought to be a rare autosomal recessive condition similar to many other ectodermal dysplasias. Diagnosis is difficult, with many possible differential diagnoses; however, eyelid cysts are a commonly seen feature. This clinical report aims to highlight this and describe the dental features and management of this syndrome, which existing literature has not previously described.