Goldenhar Syndrome (Hemifacial Microsomia)

Mandibulofacial dysostosis with congenital eyelid dermoids.

Also Known As:

Hemifacial Microsomia; Craniofacial Microsomia; Facioauriculovertebral Dysplasia; Facioauriculovertebral Sequence; First and Second Branchial Arch Syndrome; First and Second Pharyngeal Arch Syndromes; Goldenhar Disease; Goldenhar Syndrome with Ipsilateral Radial Defect; Goldenhar-Gorlin Syndrome; Hemifacial Microsomia with Radial Defects; Lateral Facial Dysplasia; Microsomia Hemifacial Radial Defects; Moeschler Clarren Syndrome; OAVS with Radial Defect; Oculoauriculovertebral Dysplasia; Oculoauriculovertebral Spectrum; Oculoauriculovertebral Spectrum with Radial Defect; Oral-Mandibular-Auricular Syndrome; Otomandibular Dysostosis; Craniofacial Microsomias; Dysostosis, Otomandibular; Dysplasia, Facioauriculovertebral; Dysplasia, Lateral Facial; Dysplasia, Oculoauriculovertebral; Dysplasias, Facioauriculovertebral; Dysplasias, Lateral Facial; Dysplasias, Oculoauriculovertebral; Facial Dysplasia, Lateral; Facial Dysplasias, Lateral; Facioauriculovertebral Dysplasias; Facioauriculovertebral Sequences; Goldenhar Gorlin Syndrome; Goldenhar-Gorlin Syndromes; Lateral Facial Dysplasias; Microsomia, Craniofacial; Microsomias, Craniofacial; Oculoauriculovertebral Dysplasias; Oculoauriculovertebral Spectrums; Oral Mandibular Auricular Syndrome; Oral-Mandibular-Auricular Syndromes; Oculoauriculovertebral Syndrome

Networked: 317 relevant articles (13 outcomes, 36 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Pierre Robin Syndrome (Syndrome, Pierre Robin)
2.	Dermoid Cyst (Dermoid)
3.	Sialorrhea
4.	Facial Paralysis (Facial Palsy)
5.	Astigmatism

Experts

1.	Liu, Wei: 9 articles (03/2022 - 05/2011)
2.	Yin, Lin: 7 articles (03/2022 - 05/2013)
3.	Zhang, Zhi-Yong: 5 articles (03/2022 - 05/2013)
4.	Chai, Gang: 5 articles (11/2021 - 01/2015)
5.	Zhang, Yan: 5 articles (11/2021 - 01/2015)
6.	Shi, Lei: 5 articles (10/2018 - 05/2013)
7.	Feng, Shi: 4 articles (03/2022 - 03/2015)
8.	Tang, Xiao-Jun: 4 articles (03/2022 - 05/2013)
9.	Zhang, Zhiyong: 4 articles (01/2022 - 05/2011)
10.	Chen, Xiaojun: 4 articles (11/2021 - 02/2019)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Goldenhar Syndrome:

1.	cenegerminIBA 02/01/2021 - "Cenegermin was effective in treating NK in an infant with Goldenhar syndrome." 02/01/2021 - "This study aimed to present the efficacy and safety of cenegermin eye drop (Oxervate; Dompè Farmaceutici, Milan, Italy) treatment in a pediatric patient affected by neurotrophic keratopathy (NK) with Goldenhar syndrome. "
2.	Glycopyrrolate (Glycopyrronium Bromide)FDA LinkGeneric 09/06/2022 - "Long-term efficacy of glycopyrrolate on sialorrhea in Goldenhar syndrome: a case report."
3.	antineoplaston A10 (A 10)IBA 07/01/2014 - "Here we have described the case of a 10 years old girl had ocular, auricular & vertebral changes consistent with Goldenhar syndrome, she was managed with multidisciplinary approach and she was symptomatically improved but corrective surgery was planned as schedule of respective department. "
4.	ElementsIBA 11/01/2021 - "The Stress Concentration and Biomechanical Properties of the Intraoral Distractors and the Extraoral Distractors Used in Treatment of Hemifacial Microsomia Patients: A Simulation Finite Element Analysis Study." 11/01/2021 - "Finite element analysis of the stresses subjected to both the intraoral and the extraoral distractors devices used in the mandibular distraction of hemifacial microsomia patients. " 01/01/2011 - "Therefore, this article presents a finite element analysis of the mechanobiological behavior of a pediatric patient's mandible with hemifacial microsomia during the distraction process. " 10/01/2010 - "This paper presents a finite element analysis of the biomechanical behavior of a patient's mandible with hemifacial microsomia (HFM) before and after distraction. " 01/01/1999 - "The aim of this study was to undertake finite element modeling of craniofacial microsomia (CFM) patients who exhibited a unilateral mandibular deformity that was surgically corrected by an inverted L osteotomy and autogenous bone graft. "
5.	Thalidomide (Thalomid)FDA Link 06/01/1997 - "The clinical appearance was investigated of 29 patients with mandibulofacial dysostosis, 26 with hemifacial microsomia, and seven with thalidomide-induced malformations affecting derivatives of the first and second branchial arches. " 01/01/1997 - "The aim of the clinical investigation was to compare mandibulofacial dysostosis (MFD) with hemifacial microsomia (HFM) and thalidomide-induced malformations restricted to the first and second branchial arches. " 06/01/1997 - "Facial nerve and 6th cranial nerve paralysis as well as anophthalmia or microphthalmia were seen only in patients with hemifacial microsomia and in the thalidomide-induced syndrome. " 01/01/1975 - "Animal models of hemifacial microsomia and thalidomide-induced otomandibular dysostosis are described; the causal mechanism of malformation appeared to be closely related to the formation of a hematoma at the time of development of the stapedial arterial systems. " 01/01/2004 - "To review the association of autism spectrum disorder (ASD) in individuals manifesting thalidomide embryopathy and Möbius sequence and compare them with three new studies in which ASD was also associated with ocular and systemic malformations: (1) a Swedish study of individuals with CHARGE association (Coloboma, Heart, choanal Atresia, developmental or growth Retardation, Genital anomaly, and Ear involvement); (2) a Swedish study of Goldenhar syndrome; and (3) Brazilian Möbius syndrome (sequence) study. "
6.	Therapeutic UsesIBA 01/01/2022 - "Owing to the complex clinical manifestations of hemifacial microsomia (HFM), multidisciplinary cooperation is required to achieve better therapeutic effects in terms of function and aesthetics. " 11/01/2010 - "To investigate the therapeutic effect of combined treatment for hemifacial microsomia (HFM). " 06/01/2019 - "The purpose of this study was to evaluate the therapeutic effect of a hybrid treatment for hemifacial microsomia that combines distraction osteogenesis and a mandible-guided functional appliance to correct mandibular asymmetry. "
7.	Ophthalmic Solutions (Eye Drops)IBA 02/01/2021 - "This study aimed to present the efficacy and safety of cenegermin eye drop (Oxervate; Dompè Farmaceutici, Milan, Italy) treatment in a pediatric patient affected by neurotrophic keratopathy (NK) with Goldenhar syndrome. "
8.	MetalsIBA 05/01/2023 - "This study developed a customized asymmetric fixation plate for hemifacial microsomia, integrating topology optimization, metal 3D printing, and in vitro biomechanical testing to resist occlusal forces and differential muscle traction on both sides of the mandible to reduce relapse and improve fixation stability."
9.	PlasticsIBA 01/01/2019 - "Goldenhar syndrome is a rare entity that affects various structures, which is why an early diagnosis and multidisciplinary management headed by a team of plastic surgeons is necessary. " 01/01/2019 - "A descriptive, observational, retrospective study was carried out using the medical and photographic record of all patients diagnosed with Goldenhar syndrome treated by the craniofacial surgery unit of the plastic and reconstructive surgery department of the Dr. Manuel Gea González hospital between 2010 and 2018. " 04/01/2012 - "From the 3-dimensional files at the Department of Plastic and Reconstructive Surgery, Skane University Hospital in Malmö, Sweden, data of 11 subjects (3 girls, 8 boys) with hemifacial microsomia were retrieved. " 04/01/2011 - "We also attempted to score the severity of the facial anomalies in each MMF patient using a system created by plastic surgeons for patients with hemifacial microsomia. " 06/01/2006 - "Fifty-eight cases of hemifacial microsomia that have been treated by plastic and reconstructive surgery and followed-up in our department during last 20 years have been reviewed. "
10.	Anesthetics (Anesthetic Agents)IBA 12/01/1994 - "We report anesthetic management of two children with Goldenhar syndrome, 9 and 14-year-old. " 01/01/1980 - "Hemifacial microsomia: an anesthetic airway problem." 01/01/2022 - "Effects of mandibular distraction osteogenesis on anesthetic implications in children with hemifacial microsomia." 01/01/2013 - "Axillary block as the sole anesthetic for peripherally inserted central catheter placement in an infant with goldenhar syndrome." 05/04/2021 - "TMJ ankylosis impacts airway management, and anesthesia providers must be aware of this clinical phenotype when planning the anesthetic care of patients with Goldenhar syndrome."

Therapies and Procedures

1.	Distraction Osteogenesis 06/01/2019 - "The hybrid technique combining distraction osteogenesis and the mandible-guided functional appliance proved to be effective in correcting canting and deviation during mandibular elongation, which improved facial symmetry and occlusal balance in patients with hemifacial microsomia." 03/01/2007 - "The sequential planning procedure presented in this paper is considered to be helpful in performing effective preoperative simulation of distraction osteogenesis for hemifacial microsomia." 11/01/2023 - "The aim of this study was to investigate the characteristics of condylar resorption in craniofacial microsomia (CFM) patients following mandibular distraction osteogenesis (MDO). " 03/08/2023 - "The aim of this study was to evaluate the volumetric airway changes using three-dimensional images following unilateral vertical mandibular distraction osteogenesis (uVMD) among patients with hemifacial microsomia (HFM). " 01/01/2023 - "This study aimed to verify the accuracy and safety of distraction osteogenesis for hemifacial microsomia assisted by a robotic navigation system based on artificial intelligence. "
2.	Prostheses and Implants (Prosthesis) 01/01/2011 - "It is beneficial for the defects of a hemifacial microsomia patient to be managed as early as possible, consistent with the patient's ability to manage the prosthesis." 08/01/1999 - "New research initiatives, such as tissue engineering and fabrication of auricular prosthesis by CAD/CAM, offer the potential for improved treatment for the future treatment of hemifacial microsomia." 08/02/2023 - "Total temporomandibular joint reconstruction prosthesis in hemifacial microsomia: A systematic review." 05/01/2022 - "A combined digital technique to fabricate an implant-retained auricular prosthesis for rehabilitation of hemifacial microsomia." 01/01/2011 - "The management and fabrication of an auricular prosthesis replacement supported by endosseous craniofacial implants for a young patient with Goldenhar-Gorlin Syndrome has been reported. "
3.	Orthognathic Surgery 04/01/2012 - "The purpose of this study was to evaluate the final outcome of team care for patients with hemifacial microsomia after a protocol of growth management, orthodontic treatment, orthognathic surgery, and soft-tissue augmentation. " 01/01/2023 - "We report on a patient with Goldenhar syndrome who was found to have a unique course of the inferior alveolar nerve during orthognathic surgery work-up and treatment." 01/01/2023 - "Orthognathic Surgery in Goldenhar Syndrome With a Rare Course of the IAN." 10/07/2022 - "Clinical Outcomes in Orthognathic Surgery for Craniofacial Microsomia Following Mandibular Distraction Using CBCT Analysis: A Retrospective Study." 12/01/2015 - "Orthognathic surgery and alloplastic grafting are the main stay in management in hemifacial microsomia. "
4.	Mandibular Reconstruction 07/18/2023 - "Costochondral grafts (CCGs) can be used in mandibular reconstruction of Kaban-Pruzansky IIB/III hemifacial microsomia (HFM). " 01/01/2020 - "Mandibular reconstruction in patients with hemifacial microsomia is still challenging. " 09/01/2018 - "Mandibular reconstruction in craniofacial microsomia (CFM) has been described and reviewed at length although final results are not always (aesthetically) satisfactory due to maxillo-mandibular asymmetry, for which optimal correction techniques remain unclear. " 02/01/2015 - "An institutional review board-approved retrospective chart review was performed of all patients with craniofacial microsomia who underwent costochondral rib grafting for mandibular reconstruction performed by the senior author (S.P.B.) at The Children's Hospital of Philadelphia from January of 1998 to September of 2013. " 03/01/2014 - "Mandibular reconstruction in the growing patient with unilateral craniofacial microsomia: a systematic review."
5.	Free Tissue Flaps 01/01/2017 - "Facial augmentation, Free flaps, Free SCIA/SIEA flap, Hemifacial microsomia. " 05/01/2011 - "The cases of 4 patients who underwent free-flap reconstruction of a severely hypoplastic mandible due to Goldenhar syndrome are presented. " 01/01/2000 - "The groin free flap was used most frequently for patients with Rombergapos;s disease, whereas the scapular free flap was used for patients with hemifacial microsomia. " 01/01/2000 - "The experience with free flaps and conventional reconstructive procedures for 118 patients with Rombergapos;s disease and hemifacial microsomia over a 10-year period is presented. " 01/01/1992 - "Though the majority of patients with hemifacial microsomia do not require free flap grafts, the procedure does present as a viable approach to severe tissue deficiency with excellent results."