Multidisciplinary Approach for Management of Dentofacial Deformity of a Patient with Goldenhar Syndrome

2018 ◽  
Vol 52 (4_suppl1) ◽  
pp. 34-39
Author(s):  
Usha Shenoy ◽  
Gauri Vichare ◽  
Deepak Kulkarni ◽  
Himija Karia
Keyword(s):  
Multidisciplinary Approach ◽  
Goldenhar Syndrome ◽  
Dentofacial Deformity

scholarly journals Multidisciplinary Approach for Management of Dentofacial Deformity of a Patient with Goldenhar Syndrome

2018 ◽  
Vol 52 (4_suppl1) ◽  
pp. 34-39
Author(s):  
Usha Shenoy ◽  
Gauri Vichare ◽  
Deepak Kulkarni ◽  
Himija Karia
Keyword(s):  
Multidisciplinary Approach ◽  
Goldenhar Syndrome ◽  
Dentofacial Deformity

scholarly journals Do you know this syndrome?

2012 ◽  
Vol 87 (3) ◽  
pp. 495-497
Author(s):  
Luiz Maurício Costa Almeida ◽  
Michelle dos Santos Diniz ◽  
Lorena dos Santos Diniz
Keyword(s):  
Multidisciplinary Approach ◽  
Goldenhar Syndrome ◽  
Clinical Aspects ◽  
Genetic Syndrome ◽  
Live Births ◽  
Mandibular Hypoplasia ◽  
Radiologic Findings ◽  
Branchial Arches ◽  
Skin Tags ◽  
Systemic Manifestations

Goldenhar syndrome is a sporadic or inherited genetic syndrome characterized by limbal dermoids, preauricular skin tags and mandibular hypoplasia. Vertebral abnormalities may occur. The incidence of this syndrome ranges from 1 in 5,600 to 1 in >20,000 live births. It consists of abnormalities involving the first and second branchial arches. The etiology of the syndrome is heterogeneous. Diagnosis should be based principally on clinical aspects, which should be associated with the patient's systemic conditions and radiologic findings. Treatment depends on the patient's age and systemic manifestations, with a multidisciplinary approach often being required.

scholarly journals Goldenhar syndrome: a rare entity

2017 ◽  
Vol 4 (5) ◽  
pp. 1897 ◽  
Author(s):  
Tariq Harris ◽  
Mohammed Abdul Bashith ◽  
Manas M. Shanbhag ◽  
Moideen Faheem
Keyword(s):  
Multidisciplinary Approach ◽  
Systemic Treatment ◽  
Obstructive Hydrocephalus ◽  
Severe Form ◽  
Goldenhar Syndrome ◽  
Branchial Arches ◽  
Hydrocephalus Treatment ◽  
Ophthalmic Treatment ◽  
Cns Malformations ◽  
Limbal Dermoid

Goldenhar syndrome was first described by Maurice Goldenhar in 1952 it involves abnormalities in the first and second branchial arches. This syndrome appears to have varying degree of presentation from mild to severe form. Different pathophysiological mechanisms have been proposed .Oculo auriculo vertebral syndrome which includes reduction in blood flow resulting in focal haemorrhage in the first and second branchial arches. In addition there may be cardiac vertebral and CNS defects. A male baby 30-32 wks, preterm SGA was admitted in NICU for evaluation of Congenital Malformations which includes swelling of the anterior border of both eyes with b/l preauricular ear tags, malformed pinna, high arched palate and micrognatia ophthalmology examination revealed b/l limbal dermoid inferotemporal aspect. ECHO done revealed ASD 4mm.CT scan showed obstructive hydrocephalus. Treatment of the condition varies according to the severity. Ophthalmic treatment aimed first because of amblyogenic risk. Craniofacial surgery in view of severe micrognathia systemic treatment may be related to cardiac, renal and CNS malformations. Goldenhar Syndrome is a rare congenital anomaly, with cosmetic defects whose treatment may pose numerous challenges with multidisciplinary approach for optimal management.

scholarly journals Craniofacial Microsomia: Goldenhar Syndrome in Association with Bilateral Congenital Cataract

2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
U. D. Shrestha ◽  
S. Adhikari
Keyword(s):  
Congenital Cataract ◽  
Multidisciplinary Approach ◽  
Goldenhar Syndrome ◽  
Hemifacial Microsomia ◽  
Pediatric Ophthalmology ◽  
Pupillary Reflex ◽  
Craniofacial Microsomia ◽  
Branchial Arches ◽  
Vertebral Anomalies ◽  
Bilateral Congenital Cataract

Craniofacial microsomia (CFM) includes a spectrum of malformations primarily involving structures derived from the first and second branchial arches. Patients with hemifacial microsomia and epibulbar dermoids are said to have Goldenhar syndrome (GHS). Four-month-old boy with whitish pupillary reflex presented with the features of GHS in pediatric ophthalmology clinic. The child had ocular and auricular manifestations. There were no vertebral anomalies, but he had bilateral congenital cataract. The peculiarity of this case is the presence of the bilateral total congenital cataract, in association with CFM. There is absence of epibulbar dermoid or lipodermoid in the eyes, although the child had features of GHS. In addition to it, anesthetic intubation was smooth in this case. Any case diagnosed with CFM and/or GHS needs treatment through multidisciplinary approach, consultation in ophthalmology department is one of them.

scholarly journals Оcular manifestation in Goldenhar syndrome- case report

2020 ◽  
Vol 12 (2) ◽  
pp. 64-70
Author(s):  
Bisera Velkovska ◽  
Natasha Trpevska Shekerinov ◽  
Emilija Gjoshevska Dashtevska ◽  
Maja Ivanova
Keyword(s):  
Case Report ◽  
Multidisciplinary Approach ◽  
Treatment Protocol ◽  
Facial Asymmetry ◽  
Goldenhar Syndrome ◽  
Female Ratio ◽  
Mandibular Hypoplasia ◽  
Optimal Functioning ◽  
Clinical Presentations ◽  
Male To Female

Goldenhar's syndrome (GS) is a complex syndrome characterized by a combination of abnormalities: hemifacial macrosomia, mandibular hypoplasia, ocular dermoids, ear abnormalities, and vertebral disorders. The incidence of GS is about 1 in 5000 to 25,000 live births. The male to female ratio is 3: 2.The etiology of this rare disease is not fully understood. The treatment protocol depends on the age of the patient and systemic clinical presentations. This paper presents a case of a 46-year-old woman with Goldenhar syndrome, which manifested with facial asymmetry, epibulbar dermoid, hearing loss, and scoliosis. Because it is a congenital syndrome, these people's lives are complex from an early age. There is a need for timely recognition of this syndrome and a multidisciplinary approach in treating these patients, with a team of specialist physicians, to enable them as much optimal functioning as possible in the environment. By presenting this case report, we would like to attract the attention of GS, which would be of educational significance for all physicians, primarily ophthalmologists, as well as otolaryngologists and orthopedists.

AAC Supports for Individuals With Rett Syndrome Across the Lifespan

2015 ◽  
Vol 24 (3) ◽  
pp. 74-85
Author(s):  
Sandra M. Grether
Keyword(s):  
Rett Syndrome ◽  
Communication Skills ◽  
Preliminary Analysis ◽  
Multidisciplinary Approach ◽  
Motor Deficits ◽  
Complex Profile ◽  
Communication Behaviors ◽  
The Individual

Individuals with Rett syndrome (RS) present with a complex profile. They benefit from a multidisciplinary approach for diagnosis, treatment, and follow-up. In our clinic, the Communication Matrix © (Rowland, 1990/1996/2004) is used to collect data about the communication skills and modalities used by those with RS across the lifespan. Preliminary analysis of this data supports the expected changes in communication behaviors as the individual with RS ages and motor deficits have a greater impact.

Goldenhar Syndrome: A Cause of Hearing Loss and Other Defects in Children

2003 ◽  
Vol 13 (2) ◽  
pp. 10-17
Author(s):  
R. Steven Ackley ◽  
Kerri McDill ◽  
Elizabeth Hellmuth
Keyword(s):  
Hearing Loss ◽  
Goldenhar Syndrome

Traumatic Brain Injury: A Trauma Surgeon's Perspective

2012 ◽  
Vol 22 (3) ◽  
pp. 82-89
Author(s):  
Oscar D. Guillamondegui
Keyword(s):  
Traumatic Brain Injury ◽  
Brain Injury ◽  
Multidisciplinary Approach ◽  
Trauma Team ◽  
The United States ◽  
Long Term Outcomes ◽  
Term Care ◽  
Injured Brain ◽  
The Moment

Traumatic brain injury (TBI) is a serious epidemic in the United States. It affects patients of all ages, race, and socioeconomic status (SES). The current care of these patients typically manifests after sequelae have been identified after discharge from the hospital, long after the inciting event. The purpose of this article is to introduce the concept of identification and management of the TBI patient from the moment of injury through long-term care as a multidisciplinary approach. By promoting an awareness of the issues that develop around the acutely injured brain and linking them to long-term outcomes, the trauma team can initiate care early to alter the effect on the patient, family, and community. Hopefully, by describing the care afforded at a trauma center and by a multidisciplinary team, we can bring a better understanding to the armamentarium of methods utilized to treat the difficult population of TBI patients.

Percutaneous Tracheostomy: A Multidisciplinary Approach

2008 ◽  
Vol 18 (2) ◽  
pp. 87-98 ◽  
Author(s):  
Vinciya Pandian ◽  
Thai Tran Nguyen ◽  
Marek Mirski ◽  
Nasir Islam Bhatti
Keyword(s):  
Health Care ◽  
Quality Assurance ◽  
Data Collection ◽  
Health Care Professionals ◽  
Multidisciplinary Approach ◽  
Percutaneous Tracheostomy ◽  
Johns Hopkins Hospital ◽  
Prospective Data ◽  
Comprehensive Service ◽  
Over Time

Abstract The techniques of performing a tracheostomy has transformed over time. Percutaneous tracheostomy is gaining popularity over open tracheostomy given its advantages and as a result the number of bedside tracheostomies has increased necessitating the need for a Percutaneous Tracheostomy Program. The Percutaneous Tracheostomy Program at the Johns Hopkins Hospital is a comprehensive service that provides care to patients before, during, and after a tracheostomy with a multidisciplinary approach aimed at decreasing complications. Education is provided to patients, families, and health-care professionals who are involved in the management of a tracheostomy. Ongoing prospective data collection serves as a tool for Quality Assurance.

Trauma: A Novel, Multidisciplinary Approach

1995 ◽  
Vol 40 (1) ◽  
pp. 7-8
Author(s):  
Heather Cecil ◽  
Melinda A. Stanley
Keyword(s):  
Multidisciplinary Approach
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