Ophthalmic features and management outcomes of 30 children having Goldenhar syndrome

Singh, Manpreet; Kaur, Manpreet; Mehta, Aditi; Yangzes, Sonam; Yadav, Deepti; Zadeng, Zoramthara; Gupta, Pankaj

doi:10.1007/s10792-019-01227-0

Cited by 9 publications

(7 citation statements)

References 31 publications

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“…In addition, the incidence of Goldenhar syndrome in our patients with limbal dermoids was quite low (10%) as compared to previous reports describing an incidence of 26% 2 to 30.3%. 13…”

Section: Discussionmentioning

confidence: 99%

Visual and surgical outcomes of limbal dermoid excision at a tertiary care eye hospital

Al-Ghadeer

Kirat

Vargas

et al. 2022

European Journal of Ophthalmology

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Purpose To evaluate the presenting complaints, surgical management, surgical outcomes, complications, and postoperative visual acuity following limbal dermoid excision. Design Retrospective cohort study. Methods Medical records of patients with limbal dermoid presenting between January 2012 and December 2020 were retrieved to extract data regarding demographics, presenting profiles including the best-corrected visual acuity (BCVA), symptoms, anterior segment examination, and refraction. The outcomes included cosmesis, complications, graft transparency, and BCVA at the last follow-up. Results Fifty-one eyes from 50 patients (27 males) were evaluated. The median age at the time of surgery was 11.5 years (interquartile range, IQR: 0.0–45.7). The median follow-up time was 5 years (IQR: 4–6). Goldenhar syndrome was noted in 5 patients (10%). The indications for surgery were cosmetic concerns (n = 20, 39%), anisometropia (n = 3, 6%), decreased vision (n = 4, 8%), and growth or Dellen formation (n = 2, 4%). Forty-eight were operated upon, opting for simple excision (n = 12, 23.5%), amniotic membrane transplantation (n = 16, 31.4%), lamellar keratoplasty (n = 15, 29.4%), and penetrating keratoplasty (n = 5, 9.8%). The most common complications were corneal scarring (n = 19, 37.2%), corneal vascularization (n = 2, 3.9%), and infection (n = 1, 2%). Astigmatism > 1 D was observed in 34 (66.7%) eyes after dermoid management ( p < 0.001). There were no complications in 14 eyes (27%), BCVA was > 20/60 in 43 eyes (84.3%), and only two eyes had BCVA < 20/400. Conclusion Surgical management of limbal dermoids offers promising functional and anatomic outcomes. However, postoperative astigmatism may require further follow-up and management.

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“…In addition, the incidence of Goldenhar syndrome in our patients with limbal dermoids was quite low (10%) as compared to previous reports describing an incidence of 26% 2 to 30.3%. 13…”

Section: Discussionmentioning

confidence: 99%

Visual and surgical outcomes of limbal dermoid excision at a tertiary care eye hospital

Al-Ghadeer

Kirat

Vargas

et al. 2022

European Journal of Ophthalmology

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“…Twenty-five articles were included in a 2020 systematic review on ocular and adnexal anomalies in CFM (33). Epibulbar dermoids and eyelid colobomata are common in patients with CFM, with a prevalence of 7%-69% and 4%-40%, respectively (33,38). Other CFM-associated anomalies include lacrimal duct or gland anomalies, microphthalmia, anophthalmia, iris colobomata, lipodermoids, optic nerve anomalies, blepharoptosis, strabismus, astigmatism, amblyopia.…”

Section: Eyementioning

confidence: 99%

A decade of clinical research on clinical characteristics, medical treatments, and surgical treatments for individuals with craniofacial microsomia: What have we learned?

Renkema

Caron

Heike

2022

Journal of Plastic, Reconstructive & Aesthetic Surgery

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“…1 También se ha reportado la presencia de colobomas, blefaroptosis, anoftalmia, o dermoides epibulbares ya sean uni o bilaterales lo cual genera restricción mecánica del globo ocular. 7,22 Los colobomas son defectos en el espesor de piel del párpado, con bordes libres y tiene una presentación entre el 6-24% de los pacientes con OAVS. 22 • Mandíbula y articulación temporo-mandibular (ATM).…”

Section: Manifestaciones Clínicasunclassified

“…7,22 Los colobomas son defectos en el espesor de piel del párpado, con bordes libres y tiene una presentación entre el 6-24% de los pacientes con OAVS. 22 • Mandíbula y articulación temporo-mandibular (ATM). Una de las principales características descritas en la literatura, es la hipoplasia mandibular.…”

Section: Manifestaciones Clínicasunclassified

“…Además de sinostosis, hemivertebras, cifoescoliosis vertebral fusionada y costillas faltantes. 9,22,25 Las anomalías cervicales son de primordial relevancia en aquellos pacientes sometidos a cirugía, por las complicaciones al momento de realizar laringoscopia directa y para intubación endotraqueal. 26 Por su parte, la escoliosis suele ser progresiva por lo que requiere de tratamiento oportuno.…”

Section: Manifestaciones Clínicasunclassified

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Manifestaciones bucales del espectro oculo-aurículo-vertebral (Síndrome de Goldenhar): Una revisión narrativa

Martínez-Chávez

Pacheco-Galván

2022

CyRS

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Introducción. El espectro oculo-auriculo-vertebral (OAVS, por sus siglas en inglés) también conocido como Síndrome de Goldenhar, es una condición congénita poco frecuente en la que se presentan alteraciones que deben ser tratadas por el odontólogo con un enfoque multidisciplinario. Objetivo. Presentar el estado del arte del conocimiento sobre las principales manifestaciones bucales y el abordaje estomatológico en pacientes pediátricos con espectro oculo-aurículo-vertebral. Desarrollo. El OAVS se caracteriza por alteraciones en las estructuras que se originan del primer y segundo arcos branquiales. Las principales alteraciones bucodentales que se presentan en el OAVS son (i) la hipoplasia mandibular, (ii) agenesia de la rama y cóndilo, cóndilo bifido (iii) desfase de la salivación debido a una aplasia de las glándulas salivales, (iv) defectos en la capacidad de deglución, (v) alteraciones del habla, (vi) taurodontismo, (vii) macrostomia. En el caso de los pacientes con presencia de cóndilo bífido se observa una limitación para la apertura bucal, lo cual genera una deficiente higiene bucal que desencadena caries y gingivitis. Por tal motivo, el odontólogo debe tener el conocimiento de dicho síndrome para proporcional un tratamiento integral con un enfoque multidisciplinario. Conclusión. El papel que juega la estomatología dentro del tratamiento multidisciplinario de los pacientes con OAVS es fundamenta, para que el diagnóstico preciso y oportuno permita brindar un tratamiento integral y multidisciplinario. Es importante la realizar un tratamiento individualizado en conjunto con un equipo multidisciplinario, donde se destaque la relevancia del papel del estomatólogo pediatra.

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Ophthalmic features and management outcomes of 30 children having Goldenhar syndrome

Cited by 9 publications

References 31 publications

Visual and surgical outcomes of limbal dermoid excision at a tertiary care eye hospital

Visual and surgical outcomes of limbal dermoid excision at a tertiary care eye hospital

A decade of clinical research on clinical characteristics, medical treatments, and surgical treatments for individuals with craniofacial microsomia: What have we learned?

Manifestaciones bucales del espectro oculo-aurículo-vertebral (Síndrome de Goldenhar): Una revisión narrativa

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