Treatment of a Goldenhar Syndrome Patient With Zygomatic and Pterygoid Implants Using the PATZi Protocol: A Case Report

Goldenhar syndrome is a rare craniofacial condition that impacts tissues developing from the first and second branchial arches.¹ Common features of this disorder include asymmetric mandibular and condylar hypoplasia with compensatory underdevelopment of the maxilla, zygomas, and temporal bones.^2,3 Additional systemic maladies of Goldenhar syndrome are microtia, ear tags, hearing impairment, ocular epidermoid tumors, coloboma of the upper eyelid, ocular motility abnormalities, vision disturbances, pulmonary anomalies, cervical vertebral fusions, renal afflictions such as hydronephrosis or kidney agenesis, and cardiac problems, including ventricular septal defects or tetralogy of Fallot.^2-6 Goldenhar syndrome has numerous alternative names, including oculoauriculovertebral dysplasia, hemifacial microsomia, and otomandibular dysostosis, among others.^6-8

Although the condition was first reported as far back as 1861, its etiology remains poorly understood.⁹ Consensus, however, is that Goldenhar syndrome is genetic rather than hereditary.¹⁰ Consanguineous (related by blood or descended from the same common ancestor) familial relations as well as environmental factors such as the use of vasoactive medications, retinoic acid, thalidomide, and cocaine and hormonal issues have also been associated with Goldenhar syndrome patients’ parents prior to pregnancy or during early gestation stages.^11-13 The condition has a predilection for males at a 3:2 ratio to females and is estimated to affect one in 3,500 to one in 5,600 live births, making it the second most common craniofacial anomaly next to cleft lip and cleft palate. ^1,14-17

While a few articles have examined dental treatment of Goldenhar syndrome patients, these publications tend to deal with multidisciplinary treatment of children and adolescents.^5,18-20 The articles have focused on procedures that address the correction of craniofacial skeletal deficiencies, such as orthognathic surgery or distraction osteogenesis,^21-24 in combination with medical procedures like auricular reconstruction²⁰ and correction of cardiac defects.⁶ Children with Goldenhar syndrome often have oral hygiene issues, partly due to small mouth opening and significant malocclusion, which has been associated with higher risks of periodontal disease and dental caries.^25,26 Accordingly, it is fairly common for many of these patients to have progressive dental maladies as they age. Despite Goldenhar syndrome having multiple known risk factors negatively affecting dental health, no publications have documented treatment of adults afflicted with this condition. The purpose of this case report is to document dental implant treatment of an adult suffering from Goldenhar syndrome.

Methods

A 61-year female patient with Goldenhar syndrome presented with a chief complaint of wanting to resolve her edentulism. The patient had worn dentures for more than 20 years and had severe maxillary atrophy as well as moderate mandibular atrophy. Her medical history was consistent with many of the hallmarks of Goldenhar syndrome. Oculosensory issues included partial blindness of the left eye, epibulbar dermoids, and colobomas, while auditory deficits were reconstructed microtia, preauricular tags, and conductive hearing loss. Systemic maladies included a history of tetralogy of Fallot with persistent pulmonary regurgitation, chronic hypertension secondary to reduced nephron mass, and managed diabetes mellitus. Skeletal abnormalities included vertebral fusion, scoliosis, and mandibular hypoplasia with concomitant underdevelopment of the maxilla and zygoma. 

Concerning the patient’s dental health, she presented with a complete maxillary denture that was ill-fitting due to significant maxillary atrophy and a mandibular overdenture that was secured by a pair of interforaminal dental implants (brand unknown). The patient had an overclosed vertical dimension of occlusion (VDO), which resulted in angular cheilitis bilaterally and poor esthetics with a lack of maxillary tooth display (Figure 1). Intraorally, she screened negative for oral cancer but did have a few traumatic ulcers and epulis fissuratum due to poorly fitting dentures (Figure 2). 

Consultation with the patient’s medical providers was achieved prior to surgery. Furthermore, a dental anesthesiologist completed a careful evaluation to determine the safety of performing general endotracheal intubation anesthesia (GETA). Patients with Goldenhar syndrome are often difficult GETA candidates due to compromised airway anatomy, including limited mouth opening, hemifacial asymmetry, retrognathia, and limited neck movement. After receiving clearance from all medical and anesthetic providers, a final cone-beam computer tomography (CBCT) analysis was performed to determine potential treatment plans involving dental implants. While the pterygomaxillary osseous structures seemed adequate for dental implants, the zygomas were significantly lacking in volume with only 10.8 mm and 6.5 mm of vertical height and 3.7 mm and 4.2 mm in thickness for the right and left zygomas, respectively (Figure 3 through Figure 5). Although both malar processes were of minimal height, they had exceptional density making placement of a single zygomatic fixture possible in each side. Anteriorly, the patient had minimal alveolar ridge thickness, but the bones surrounding the nasopalatine canal had adequate height and thickness to accommodate a conventional dental implant. In the opposing arch, the anterior mandible had sufficient bone to accommodate conventional fixtures while bone posterior to the mental foramina was acceptable for the placement of shorter-length implants to avoid the inferior alveolar nerve. 

After providing informed consent, the patient received GETA from the dental anesthesiologist, and a latex-free lip/cheek retractor (OptraGate®, Ivoclar, Ivoclar.com) was installed to protect soft tissues. Local anesthesia was administered with posterior superior alveolar, middle superior alveolar, infraorbital, V2, and maxillary incisive nerve blocks. A full-thickness incision was achieved with a 15-blade scalpel from the right to left hamular notch with vertical releases at the midline and zygomaticomaxillary buttresses bilaterally. A full-thickness flap was elevated to expose bilateral hamular notches, masseteric tendons, infraorbital nerves, malar processes, and the nasal spine. 

Following the PATZi (posterior, anterior, tilted, zygomatic implants) protocol, a treatment algorithm specific for maxillary atrophy,^27-30 dental implants (4.2 mm x 18 mm PteryFit™, Noris Medical, norismedical.com) were first placed in the pterygomaxillary complexes to achieve posterior support. After dissection of the maxillary incisive neurovascular bundle, anterior support was then achieved via the placement of a conventional dental implant (4.2 mm x 10 mm Tuff®, Noris Medical), which transected the nasopalatine canal and engaged the dense bone of the nasal spine. Because the use of tilted implants was not possible in this case due to the amount of severe maxillary atrophy, the PATZi algorithm next dictated that zygomatic implants be placed. Although bone volume in the malar processes was limited, single zygomatic implants (4.2 mm x 42.5 mm and 4.2 x 45 mm, Noris Medical) were successfully placed into each zygoma. 

Composite torque value for the five implants placed in the maxilla exceeded 300 Ncm. Angled multi-unit abutments were secured to each dental implant fixture (Figure 6) and quick conversion caps (Smart Denture Conversions, LLC, smartonx.com) were installed. After copious irrigation with sterile saline, buccal fat pads were accessed and advanced to cover the extrasinus portions of the zygomatic implants. Primary mucoperiosteal flap closure was achieved with 3-0 chromic gut suture using the “Texas 2-Step” technique, a specialized running stitch primarily used in dentistry for full-arch or quadrant wound closure.²⁷ A conventional denture was relined and seated to pick up the quick conversion caps and later converted into a transitional screw-retained fixture.

Upon completion of the maxillary surgery, local anesthetic was applied to the mandible via bilateral inferior alveolar and mental nerve blocks. A full-thickness incision was made from the right to left retromolar pads with lateral releasing incisions, and a full-thickness flap was elevated. After positive identification of the mental nerves bilaterally, the pre-existing dental implants were removed with retrieval tools (Figure 7), as they were in inadequate positions to accommodate the planned fixed prosthesis. Minor alveoloplasty was performed with a surgical high-speed handpiece under copious irrigation. Three conventional dental implants (3.75 mm x 11.5 mm Tuff) were placed in the mandibular interforaminal bone, while two additional implants were placed in the mandible posterior to the mental foramina (4.2 mm x 8.5 mm Tuff). The remainder of the surgery was completed in a fashion similar to the maxilla. 

After conversion of the maxillary and mandibular conventional dentures to transitional screw-retained bridges immediately upon completion of the surgery (Figure 8), the prostheses were delivered and the patient’s bite was adjusted to achieve even single-point contact throughout the arch with light contact in the mandibular cantilevers and no protrusive or excursive interferences. Because the patient was diagnosed presurgically with an overclosed VDO, the prosthetic bite was opened vertically 6 mm. Post-surgical CBCT scans were satisfactory and revealed ideal placement of the dental implant fixtures (Figure 9). 

Results

Post-surgically, the patient healed uneventfully and without complication. Opening the VDO alleviated the patient’s angular cheilitis, and she noted improved chewing capacity with reduced temporomandibular joint discomfort. After 6 months of functioning with the transitional screw-retained bridges and eating a softer diet, final restorations were delivered to the patient (Figure 10 and Figure 11). Occlusal adjustments were performed in a similar fashion to the transitional bridges, and the patient was cleared to return to full function. 

Yearly maintenance visits were performed during which the restorations would be removed and cleaned, multi-unit abutment screws tightened, new prosthetic screws delivered, and occlusion readjusted. At the time of this writing, the patient has functioned for close to 5 years with the final restoration and has reported to be pleased with the outcome (Figure 12).

Discussion

Same-day full-arch immediate loading in its contemporary form, commonly referred to as “All-on-4®” or “all-on-X”, has more than a quarter century of documentation with successful long-term results.^31,32 These procedures allow for same-day function and reduce the number of implants needed to support a full-arch prosthesis.³³ When maxillary atrophy is severe, alternative fixtures such as zygomatic and pterygoid implants are often utilized in lieu of conventional implants and alleviate the need for invasive and time-consuming bone augmentation procedures.^27-30 Although zygomatic and pterygoid implants predate modern all-on-X procedures by nearly 20 years, their techniques have evolved to allow their incorporation into these treatments.

One such evolution is the development of the PATZi protocol, which the present author first documented and described in 202227 and which Ponusamy et al later modified in 2023.^29,30 PATZi is a systematic algorithm for rapid treatment planning atrophic maxillae, allowing for real-time modifications during surgical procedures. PATZi incorporates all-on-X principles with conventional and remote anchorage implants to produce a near infinite combination of arch solutions. The algorithm starts with the aim of achieving posterior support via placement of pterygoid implants for cantilever elimination, maximizing anterior–posterior spread, and increasing composite torque value.^27,34,35 If posterior support is not achieved with pterygoid fixtures, alternative techniques such as Holtzclaw extrasinus implant anchorage (HESIAn) zygomatic implants may be used.³⁶ Anterior support is next attempted with either conventional or remote anchorage techniques. In the present case report, anterior support was achieved through the use of a conventional dental implant placed through the nasopalatine canal with apical engagement in the nasal spine. With posterior and anterior support achieved, the PATZi algorithm next calls for “tilted” implants as employed in traditional all-on-X and All-on-4® protocols.^31,32 If the use of tilted implants is not possible, treatment progresses in the PATZi algorithm to zygomatic implants to achieve mid-maxillary support as seen in the treatment documented in this article. 

While all-on-X and All-On-4® treatments have been documented for a variety of dental and medical conditions, such remedies for patients affected by Goldenhar syndrome have never been reported. A PUBMED and Google Scholar search for the terms “dental implants Goldenhar syndrome” and multiple variations thereof produced a dearth of results. Although one article recorded use of cranial implants for retention of an auricular prosthesis,³⁷ no articles were found documenting dental implant treatment in adult patients afflicted with Goldenhar syndrome. Craniofacial maladies associated with Goldenhar syndrome make dental implant treatment particularly challenging. Mandibular hypoplasia occurs in a unilateral fashion in 90% of cases with a predilection toward the right face.^38-40 Variable expression of mandibular involvement is categorized in the OMENS classification, which evaluates effects on the mandible, ramus, condyle, glenoid fossa, and temporomandibular joint.⁴¹ This unilateral hypoplasia of the mandible typically results in compensatory underdevelopment of the ipsilateral maxilla, zygoma, orbital, and temporal bones that often creates malocclusions.^5,42 Furthermore, lack of size and volume in these bones can compromise or outright preclude placement of dental implants. Such a situation was seen in the patient in the present case report. 

While anatomic studies show mean zygoma height is 20.72 mm in males and 19.66 mm in females, the right and left zygomatic heights of the Goldenhar patient in this article were only 10.8 mm and 6.5 mm, respectively.⁴³ Concerning zygoma thickness, published studies show mean measurements in zone B (mid-zygoma) of 5.87 mm with a range of 4.39 mm to 7.02 mm, while the patient in this case report had bone thickness of only 3.7 mm in this area.⁴⁴ While guided dental implant surgery has proven to be beneficial in patients with compromised bone volume,^45-47 limited mouth opening and severe bone atrophy may prevent use of such aids in patients with Goldenhar syndrome. As such, the patient in the present case report was treated with a freehand technique. This required careful diagnosis and analysis of presurgical CBCT scans, as recommended in previously published studies regarding Goldenhar syndrome.⁴² Although minimal bone was present in the maxilla, use of remote anchorage sites allowed for cumulative torque value exceeding 300 Ncm, which is significantly higher than the proposed 120 Ncm threshold for full-arch immediate loading.⁴⁸

After nearly 5 years of function in the final restoration, the patient has experienced no prosthetic complications and is happy with the outcome of treatment. Furthermore, bone loss on both the maxillary and mandibular implants has been minimal.

Conclusion

Dental implant treatment of patients with Goldenhar syndrome has received scant attention in the dental literature. This case report documents the use of not only conventional dental implants, but also remote anchorage fixtures for full-arch immediate loading in an adult patient with Goldenhar syndrome. With the patient experiencing nearly 5 years in function and no complications, this case report documents the potential for successful alleviation of edentulism using dental implants in patients afflicted by this relatively rare genetic condition and warrants additional studies. 

DISCLOSURE

Dr. Holtzclaw is a paid consultant for Noris Medical, LLC.

ABOUT THE AUTHOR

Dan Holtzclaw, DDS, MS

Private Practice, Austin, Texas

Figures and Images

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Figure 12

Figure 1

The patient displayed many of the hallmarks of Goldenhar syndrome, including auricular, ocular, and craniofacial involvement. Note the bilateral angular cheilitis due to an overclosed VDO.

Figure 2

Presurgical intraoral situation showing traumatic ulcerations and epulis fissuratum secondary to poorly fitting conventional dentures.

Figure 3

CBCT slice (coronal view) of Goldenhar patient with left zygomatic bone height of only 6.50 mm. Typical zygomatic bone height for non-affected females is 19.66 mm.

Figure 4

CBCT slice (sagittal view) of Goldenhar patient with left zygomatic bone width of only 3.70 mm. Typical zygomatic bone width for non-affected persons is 5.87 mm.

Figure 5

3D CBCT rendering of Goldenhar syndrome patient prior to surgical treatment.

Figure 6

The patient was treated with two pterygoid, two zygomatic, and one nasopalatine implant via the PATZi protocol algorithm.

Figure 7

The pre-existing mandibular implants were removed with implant retrieval tools.

Figure 8

Transitional screw-retained prosthesis was delivered on the day of surgery for immediate loading.

Figure 9

3D CBCT rendering of Goldenhar syndrome patient immediately after surgery.

Figure 10

Goldenhar syndrome patient after delivery of final screw-retained restorations. Note the resolution of the presurgical angular cheilitis after opening of the VDO.

Figure 11

3D CBCT rendering of the patient at the final prosthesis delivery appointment.

Figure 12

3D CBCT rendering of the patient at 24 months post-treatment.