Goldenhar Syndrome and Orthodontics UK

What is Goldenhar syndrome?

Goldenhar syndrome is a rare congenital condition, present from birth, in which structures derived from the first and second branchial arches fail to develop fully — usually on one side of the face, though both sides can be affected to different degrees. It sits within a wider group of conditions known as oculo-auriculo-vertebral spectrum (OAVS), which also includes hemifacial microsomia; Goldenhar syndrome is generally used to describe presentations that also involve the eye (typically an epibulbar dermoid, a benign growth on the surface of the eye) and the spine (vertebral anomalies), in addition to the ear and jaw differences common across the spectrum. The exact cause is not fully understood, and most cases occur without a clear family history, though the underlying mechanism is thought to relate to disrupted blood supply or development of tissue in the affected region during early pregnancy.

How Goldenhar syndrome affects the jaw and bite

The jaw findings in Goldenhar syndrome centre on underdevelopment of the mandible on the affected side, most often involving the ramus (the vertical part of the jawbone) and the condyle — the rounded end that forms the jaw joint. Where the condyle is absent, rudimentary, or structurally abnormal, the temporomandibular joint on that side does not function normally, which can limit mouth opening and alters how the lower jaw grows relative to the upper jaw over childhood. As the unaffected side continues to grow normally, the difference between the two sides tends to become more pronounced with age rather than staying static, producing a chin and lower jaw that deviate towards the affected side, a tilted (canted) bite plane, and dental crowding or displacement, particularly on the affected side.

Macrostomia and its effect on orthodontic assessment

Macrostomia — a lateral facial cleft that widens the corner of the mouth on the affected side, sometimes described as an unusually wide mouth on one side — is a recognised feature in some children with Goldenhar syndrome, alongside soft tissue tags near the ear and cheek. Where present, macrostomia is usually corrected surgically by a craniofacial or plastic surgery team in early childhood, since it affects both appearance and oral competence (the ability to close the lips fully). For orthodontic assessment, macrostomia repair and the resulting scar tissue around the corner of the mouth are relevant background: they can affect lip and cheek muscle tone and tension on the affected side, which in turn can influence tooth position and the pattern of dental crowding as the permanent teeth erupt.

Ear involvement, hearing, and coordinating dental appointments

Ear differences — ranging from small skin tags to microtia (an underdeveloped outer ear) and aural atresia (absence or narrowing of the ear canal) — are common in Goldenhar syndrome and often accompany conductive hearing loss on the affected side. While ear reconstruction and audiology care sit with ENT and plastic surgery teams rather than the dental team, hearing loss is worth flagging at dental and orthodontic appointments so that instructions are given clearly, ideally facing the child and confirming understanding, particularly before any procedure involving unfamiliar sounds such as impression-taking or intraoral scanning.

Distraction osteogenesis and staged jaw correction

For children with significant mandibular underdevelopment, distraction osteogenesis — a technique in which the jawbone is surgically divided and then gradually lengthened using a small device that separates the bone by about a millimetre a day, allowing new bone to fill the gap — may be used in early to mid-childhood to reduce asymmetry and, in cases with airway involvement, to improve breathing. This is typically followed by a period of orthodontic monitoring and, in adolescence or early adulthood once facial growth is complete, definitive orthognathic (jaw) surgery to correct any residual asymmetry and establish a stable, functional bite. Our separate guide to hemifacial microsomia orthodontic management covers the staging of distraction osteogenesis and orthognathic surgery in more detail, since the surgical pathway is shared across the OAV spectrum.

Vertebral anomalies and positioning for dental treatment

Vertebral anomalies — commonly affecting the cervical (neck) spine, such as fused or malformed vertebrae — occur in a proportion of children with Goldenhar syndrome and are usually identified through spinal imaging as part of the wider diagnostic work-up. Where present, these can affect neck mobility and the range of head positioning that is comfortable or safe during dental treatment, particularly for procedures requiring the head to be tilted back. The dental or orthodontic team should be made aware of any diagnosed vertebral involvement so that the dental chair position and any general anaesthetic airway management, if ever required, can be planned accordingly by the anaesthetic team.

What NHS orthodontic and craniofacial care looks like for Goldenhar syndrome

In the UK, children with Goldenhar syndrome are typically cared for by one of the NHS specialist craniofacial units — including Great Ormond Street Hospital, Alder Hey Children's NHS Foundation Trust, Birmingham Children's Hospital, and the Oxford Craniofacial Unit — which coordinate input from maxillofacial surgery, orthodontics, ENT, ophthalmology, speech and language therapy, and clinical psychology across childhood and into early adulthood. Orthodontic assessment usually begins in the mixed dentition years to establish a baseline and monitor how the asymmetry is developing, with active treatment timed around any planned distraction osteogenesis or definitive jaw surgery rather than following a fixed generic schedule. Because the condition spans multiple specialties, sharing imaging, surgical reports, and audiology results with the orthodontic team at each stage helps keep the overall treatment plan properly coordinated.

Supporting a child with Goldenhar syndrome through orthodontic treatment

Children with visible facial differences, including those from Goldenhar syndrome, can experience self-consciousness or social anxiety related to their appearance, and orthodontic treatment — particularly any discussion of jaw surgery — can bring this to the surface at different ages. UK charities such as Goldenhar UK connect families for peer support and practical guidance, and craniofacial units typically include clinical psychology support as a routine part of the care pathway rather than something families need to specifically request. Involving the child in age-appropriate discussion about their treatment plan, and giving realistic timeframes for what orthodontics can and cannot achieve before any planned jaw surgery, helps manage expectations through what is often a many-year treatment journey.

Useful related pages

This guide is for general information only. It is not a diagnosis, treatment plan, or substitute for advice from a registered dentist or orthodontist.