Innovative Surgical Treatment of Severe Cherubism.

BACKGROUND: Cherubism is an autosomal dominant syndrome characterized by excessive bilateral maxillomandibular bony degeneration and fibrous tissue hyperplasia. Conservative management is the preferred treatment as cherubism has a self-limiting course. Functional or emotional disturbances may, however, demand surgical intervention. We report a patient who underwent surgical intervention. METHOD/DESCRIPTION: He had significant enlargement of lower cheeks and bilateral lower lid scleral show. On computed tomography of the face, the patient had significant fibrous tissue involving bilateral maxilla and mandible. The mandibular tumor was excised. Given normal inferior border, bilateral sagittal split osteotomy was performed to infracture and inset the outer cortex. During the procedure, patient required blood transfusion intraoperatively, so the maxillary portion of the procedure was delayed until 6 months later. For the maxilla, bilateral transconjunctival approach was used to resect parts of the orbital floors that were concave, resulting in 1 × 2 cm defects bilaterally which were reconstructed using resorbable plates. Then the anterior maxillary tumor was excised. RESULTS: The patient and his parents were satisfied with his appearance after surgery. The patient was noted to have improvement in contour and decreased scleral show. He has most recently followed up 15 months after the initial surgery. There were no long-term complications. CONCLUSIONS: Severity of cherubism influences the type of surgical intervention. The present case is innovative because this is the first reported case of recontouring orbital floors with resorbable plates and infracturing of the mandible using sagittal split osteotomies for surgical treatment of cherubism.

Rigid External Distractor Aided Conventional Le Fort III Osteotomy Advancement in Adult With Severe Midfacial Hypoplasia.

BACKGROUND: Management of severe midfacial hypoplasia is still a challenge for craniofacial team, adult patients with syndromic midfacial hypoplasia made the situation even worse. The authors present the clinical result in an adult patient with Crouzon syndrome treated by rigid external distractor aided conventional Le Fort III procedure. METHODS: A 26-year-old patient with Crouzon syndrome presented with severe midfacial hypoplasia, a negative overjet of -17 mm, exorbitism, airway obstruction, and masticatory problem, while chief complaint of the patient was abnormal appearance. After Le Fort III osteotomy, rigid external distractor and distraction hooks were fixed. With the aid of rigid external distractor, the midfacial mass was immediately advanced to a desired position, allograft bone grafted in the gaps, and microplate fixed. The device was removed 3 weeks later when the advanced midface was stable. RESULTS: Point A was advanced by 18.6 mm in horizontal and displaced superiorly 0.5 mm in vertical at the time of device removal. Point A moved backward 1.5 mm and upward 0.3 mm at 2-year follow-up. A good and balanced facial profile was obtained in a short treatment period. Airway obstruction symptoms and exorbitism were relieved. CONCLUSIONS: The midfacial advancement achieved by rigid external distractor aided conventional Le Fort III osteotomy is a stable, controllable, and timesaving procedure. This technique may become an important choice for adult patients with severe midfacial hypoplasia.

Distinguishing Goldenhar Syndrome from Craniofacial Microsomia.

Goldenhar syndrome is characterized by the typical features of craniofacial microsomia (CFM) with the addition of epibulbar dermoids and vertebral anomalies. The aim of this study is to examine the objective differences between patients carrying a diagnosis of Goldenhar syndrome to those diagnosed with CFM. Thus, we performed an Institutional Review Board-approved retrospective chart review on all patients who presented with a diagnosis of CFM or Goldenhar syndrome from January 1990 to December 2012. Demographic, diagnosis, OMENS+ classification, accompanying diagnoses, and radiographic data were collected. For subjective analysis, subgroups were designed based on the diagnosis Goldenhar syndrome or CFM per history. For objective analysis, subgroups were designed based on the presence of epibulbar dermoids and/or vertebral anomalies. The cohorts were compared with respect to associated medical abnormalities and severity of CFM features. One hundred thirty eight patients met inclusion criteria. Epibulbar dermoids and vertebral anomalies were seen in 17% and 34% of the patients, respectively. Only 10 patients (7.2%) had both epibulbar dermoids and vertebral anomalies. The subjective "Goldenhar" group (N = 44, 32%) was found to have a higher percentage of bilaterally affected patients (P = 0.001), a more severe mandibular deformity (P = <0.001), a more severe soft tissue deformity (P = 0.01), and a higher incidence of macrostomia (P = 0.003). In the objective subgroup analysis, the only significant difference was found in the degree of soft tissue deficiency (P = 0.049). The diagnostic criteria of Goldenhar syndrome remain unclear, thereby making clinical use of the term "Goldenhar" inconsequential. Goldenhar syndrome is over diagnosed subjectively in patients who show more severe CFM features.

In support of using computer-aided design and modeling for periorbital osteotomies.

Three-dimensional virtual surgical planning using computer-aided design and modeling (CAD/CAM) has gained popularity in planning complex orthognathic and osteocutaneous free flap reconstructions of the head and neck because of its ability to guide complex geometric planning in three-dimensional space and save time in the operating room. The purpose of this study was to review our experience using CAD/CAM concepts in periorbital osteotomies. Three complex periorbital osteotomies were planned and performed: 1 case of bilateral vertical and horizontal orbital dystopia, 1 case of a Monobloc-Le Fort II in an Apert patient, and 1 case of recurrent hypertelorism in a patient with craniofrontal nasal dysplasia. The patients' charts were reviewed, including photographs, medical records, and CAD/CAM plans. The CAD/CAM planning sessions were held 3 to 6 weeks preoperatively and lasted approximately 1 hour. Both cutting guides and positioning guides were used, translating to significant precision of both the osteotomy as well as the final position of the orbits. Qualitatively, the cutting and positioning guides were easy to use and improved operating room efficiency. To conclude, in our hands, CAD/CAM virtual surgical planning is safe and effective in the performance of complex periorbital osteotomies. More work is needed to more clearly define surgical indications for this costly, new technology.

Infraorbital canal bilaterally replaced by a lateroantral canal.

The infraorbital canal (IOC) normally courses above the maxillary sinus in the orbit floor. During a retrospective study of cone beam computed tomography (CBCT) scans, we found a previously unknown variant of the IOC. The IOCs were absent, being replaced by lateroantral canals coursing around and not above the maxillary sinus to open at infraorbital foramina which were located above the second upper premolar teeth. On coronal multiplanar reconstructions, the lateroantral canals were located anatomically at the outer limit of the zygomatic recess of each maxillary sinus, while the upper wall of the sinus was devoid of any canal. Such rare variant should be kept in mind by dental practitioners and surgeons, as it can determine modifications of common procedures. In this regard, the anatomy of maxilla, as well as mandible, should be evaluated in CBCT on a case-by-case basis.

Repair of orbital bone defects in canines using grafts of enriched autologous bone marrow stromal cells.

BACKGROUND: Bone tissue engineering is a new approach for the repair of orbital defects. The aim of the present study was to explore the feasibility of tissue-engineered bone constructed using bone marrow stromal cells (BMSCs) that were rapidly isolated and concentrated from bone marrow (BM) by the red cell lysis method, then combined with ß-tricalcium phosphate (ß-TCP) to create grafts used to restore orbital bone defects in canines. METHODS: In the experimental group, grafts were constructed using BMSCs obtained by red cell lysis from 20 ml bone marrow, combined with ß-TCP and BM via the custom-made stem cell-scaffold device, then used to repair 10 mm diameter medial orbital wall bony defects in canines. Results were compared with those in groups grafted with BM/ß-TCP or ß-TCP alone, or with defects left untreated as controls. The enrichment of BMSCs and nucleated cells (NCs) in the graft was calculated from the number in untreated bone marrow and in suspensions after red cell lysis. Spiral computed tomography (CT) scans were performed 1, 4, 12 and 24 weeks after implantation in all groups. Gross examination, micro-CT and histological measurements were performed 24 weeks after surgery. The results were analyzed to evaluate the efficacy of bone repair. RESULTS: The number of NCs and of colony-forming units within the scaffolds were increased 54.8 times and 53.4 times, respectively, compared with untreated bone marrow. In the BMSC-BM/ß-TCP group, CT examination revealed that the scaffolds were gradually absorbed and the bony defects were restored. Micro-CT and histological examination confirmed that the implantations led to good repair of the defects, with 6 out 8 orbital defects completely restored in the experimental group, while by contrast, the grafts in the control groups did not fully repair the bony defects, a difference which was statistically significant (p<0.05). CONCLUSIONS: Tissue-engineered bone, constructed using BMSCs isolated by red cell lysis of BM, can restore critical-sized orbital wall defects in canines.

[Orbitocutaneous fistula after orbital reconstruction]. / Orbitokutane Fistel nach Orbitarekonstruktion.

A 73-year old man presented with a lesion inferior to his left medial canthus with discharge and headache. Fifteen years previously, the patient had undergone reduction of an orbital fracture and reconstruction of the orbit with silicone. An orbitocutaneous fistula was found to originate from the implant which was displaced in the ethmoid, obstructing frontal sinus outflow. Symptoms disappeared after explantation of the implant, reconstruction of the orbit with a titanium plate and frontal sinus surgery. Although the use of silicone in facial trauma has declined, complications have to be expected even years after implantation.

Orbital and Periorbital Dysmorphology in Untreated Pfeiffer Syndrome.

BACKGROUND: Visual impairment secondary to orbital and periorbital dysmorphology is frequent in Pfeiffer syndrome patients. The etiopathogenesis of this aberrancy, however, remains unclear. METHODS: Untreated Pfeiffer syndrome patients (n = 31) and normal control subjects (n = 43) were compared. Craniometric and volumetric analyses related to the orbital and periorbital anatomy were performed using Materialise (Leuven, Belgium) software. RESULTS: Overall, orbital cavity volume of Pfeiffer patients is reduced by 28 percent (p < 0.001), compared to normal, starting before 3 months of age (p = 0.004). Globe volume was diminished by 10 percent (p = 0.041) before 3 months of age, yet tended to catch up thereafter. However, the retrobulbar soft-tissue volume remained smaller beyond 1 year of age (17 percent, p = 0.003). Globe volume projection beyond the bony orbit increased in all observed ages (82 percent, p < 0.001). The volumes of sphenoid bone, maxilla, and mandible proportionately were restricted by 24 to 25 percent (p = 0.003 to 0.035) before 3 months of age. The volume of maxilla and mandible gradually approximate normal; however, the sphenoid bone volume in Pfeiffer patients remains less than normal (p = 0.002) into childhood. The anteroposterior length of both the zygoma and the maxilla was reduced by 14 percent (p < 0.001). Anterior positioning of the zygoma is less by 23 percent (p < 0.001) in Pfeiffer patients overall, with anterior positioning of maxilla reduced similarly by 23 percent (p < 0.001). CONCLUSIONS: Pfeiffer syndrome patients develop decreased retrobulbar soft-tissue and globe volume, along with a restricted orbital cavity volume in infancy. Significant hypoplasia of the sphenoid bone is associated with more severe central facial (maxilla) retrusion, compared to lateral facial structures (zygoma). CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Orbit and maxilla augmentation of oblique facial clefts using mandibular outer cortex.

BACKGROUND: This study focused on using the mandibular outer cortex for orbital and maxillary augmentation of oblique facial clefts. METHODS: Eight oblique facial clefts were reconstructed with the mandibular outer cortex from April 2000 to April 2008. RESULTS: No visible scars and few complications were observed at the donor site. Postoperative follow-up for 6 months to 3 years demonstrated that all of the patients had good aesthetic facial appearances. CONCLUSIONS: The mandibular outer cortex can be used for orbital and maxillary augmentation of oblique facial clefts.

Preoperative Ultrasound-Guided Percutaneous Embolization of Orbital Lymphaticovenous Malformations Using Onyx.

The treatment of orbital lymphaticovenous malformations (OLVMs) has evolved from simple surgical resection to a multimodal approach consisting of sclerosing agents, embolization, and resection depending on the specific components that compose the lesion. Here we present a representative case series of 5 nonconsecutive patients who underwent percutaneous embolization of OLVMs with the Onyx Liquid Embolic System (Medtronic, Dublin, Ireland) before surgical resection between 2019 and 2021. OLVMs are rare, benign, congenital lesions that grow with age and can become symptomatic and disabling. Treatment can be conservative or invasive; depending on the clinical presentation and characteristics of the lesions, treatment modalities can vary. Preoperative percutaneous embolization of OLVMs with the Onyx system is a promising technique, as demonstrated in this case series.

Inferolateral migration of hydrogel orbital implants in microphthalmia.

PURPOSE: Hydrogel spheres may be useful in treating orbital hypoplasia associated with congenital microphthalmia. The authors describe migration associated with the use of these devices. METHODS: The authors retrospectively reviewed 5 cases in which a hydrogel orbital expander (Osmed) was implanted to treat orbital hypoplasia in pediatric patients with congenital microphthalmia (with or without previous surgery). RESULTS: In all 5 cases, a lateral orbitotomy, conjunctiva-sparing approach was used to insert the hydrogel spheres. Two cases involved previously unoperated orbits; 3 patients had prior orbit or socket surgery. Inferolateral movement outside the desired central, deep orbital position occurred in all 5 cases. Four of 5 cases required further procedures to achieve an adequate orbital implant position. CONCLUSIONS: Inferolateral migration may occur with hydrogel spheres implanted via a lateral orbitotomy approach in microphthalmia.

Oral-nasal-ocular cleft: the greatest challenge among the rare clefts.

INTRODUCTION: Number 3 cleft or oral-nasal-ocular cleft is a well-known entity that was described by Morian over a century ago. This malformation is a paranasal-medial orbitomaxillary cleft running across the lacrimal segment of the lower eyelid and over the lacrimal groove. The Tessier number 3 naso-ocular cleft represents one of the most difficult and challenging malformations to correct for the reconstructive surgeon. We have conducted a retrospective analysis of our series consisting of 21 cases. OBJECTIVE: The objective was to review the functional outcome and aesthetic results of the different techniques applied for each case. MATERIALS AND METHODS: From 1997 to 2007, 21 patients with a Tessier number 3 cleft were treated in our craniofacial units. The clinical findings, tomographic studies, and surgical procedures were reviewed and analyzed. We have discussed our protocol of the treatment. RESULTS: We have treated facial malformation in 2 craniofacial centers. Fourteen patients were evaluated in the first year of their life, with an average age at presentation of 3 years. Twelve patients were female, and 9 were male; 6 patients had amniotic bands in limbs, 5 patients had an association with Tessier number 11 cleft, 3 patients with number 9 cleft, and 1 with number 7 cleft. Related to cleft lip, 10 patients had bilateral cleft lip, and 8 patients had unilateral cleft lip. Three patients did not have any involvement of the upper lip. The alar base was deviated upward in 19 patients, 11 cases had severe anatomic alteration with the lateral border of the ala above the medial canthus, and 8 cases had a mild dislocation. Nine cases of lacrimal duct obstruction and 8 cases of lacrimal duct extrophy were identified. Twelve patients had a lower eyelid coloboma of varying grades, and there were 2 cases of microblepharia. Aiming the soft tissue reconstruction, eyelid, nose, and upper lip were evaluated regarding their position, absence of tissue, and position of medial canthus and ala. Twelve of our patients underwent correction in the same moment, their medial canthus rotated upward and the ala downward, using the contralateral side as the reference. The lip was treated using a Millard-like technique. Neo-conjunctivorhinostomy was performed in the same moment in 2 patients or later in 1 case. Four patients had plagiocephaly due to the cranial involvement, and they were submitted to cranioplasty. Three had neurosurgical approach and advancement of the frontal bandeau. One adult patient received an acrylic plate to reshape the frontal area. CONCLUSIONS: Tessier number 3 cleft is one of the most difficult and challenging malformations to correct for the reconstructive surgeon. Besides the difficulties of its treatment, patients with Tessier number 3 cleft may achieve good results when the team has good skills.

New technique for reconstructing the affected cranium and orbital rim in unicoronal craniosynostosis.

Of the single-suture craniosynostoses, unicoronal synostosis (UCS) is widely acknowledged to represent the most varied and complex set of craniofacial deformities. This is attributed to the endocranial base being affected along with the coronal suture. This deformity may present with both coronal and frontosphenoidal synostosis and has been associated with elevated intracranial pressure; brain morphological abnormalities; vertical dystopia; ambylopia; malformations of the forehead, orbit, midface, and mandible; and behavioral, cognitive, and speech abnormalities. It is the variable structural presentation coupled with the long-term stability of the reconstruction that forms a complex reconstructive challenge. Our surgical technique concordantly addresses these associated structures beyond the isolated coronal suture. Although several surgical techniques have previously been described, there remains no consensus toward a preferred surgical approach to this dynamic and three-dimensional problem. We describe our technique of coronal and frontosphenoidal synostosis release as part of a frontotemporoparietal bone flap, release of the entire fronto-orbital rim, and use of the unaffected skull to reconstruct the affected half of the skull. The purpose of our study was to describe our surgical approach to UCS. It has been our experience that optimal correction of UCS must address the associated anomalies beyond the affected coronal suture and must be stable over time.

Craniofacial features in Goldenhar syndrome.

Goldenhar syndrome also known as oculo-auriculo-vertebral syndrome was first reported by Dr Maurice Goldenhar in 1952. It is a rare disease entity characterized by craniofacial anomalies such as hypoplasia of the mandible and malar bones, microtia, and vertebral anomalies. The etiology of this disease still remains unclear and occurs as sporadic. This report presents goldenhar syndrome in a 12-year-old male patient.

Orbit, zygoma, and maxilla growth patterns in Crouzon syndrome.

The facial malformations of Crouzon syndrome involve the entire cranio-orbito-zygomatic region. The detailed sequence of changes in orbit, zygoma, and maxilla over time, the mutual influence among these three anatomical structures, and their relationship with the cranial base were studied to determine the sequence and timing of deformity. Preoperative CT scans of 36 patients with Crouzon syndrome (mean age 10.84±14.70years; 14 male, 22 female) and CT scans of 54 control subjects (mean age 8.53±13.22years; 29 male, 25 female) were divided into five subgroups by age: 0-6 months, 6 months-2 years, 2-6 years, 6-18 years, and 18-62 years. Craniofacial morphometric cephalometrics were analyzed using Materialise software. Crouzon orbit anteroposterior length was shorter before 6 months (P=0.021) and remained shorter into adulthood (P<0.001). Globe projection was greater across all age subgroups (P<0.001), reaching a peak at 6 months to 2 years (P<0.001). The increased medial orbital width was the most remarkable and persistent secondary deformity (P<0.001). The zygoma anterior protrusion was retruded before 6 months of age (P<0.001), but then improved gradually. The width of maxilla was greater by 24% in the Crouzon cohort (P<0.001), with a difference of 16% before 6 months (P=0.024), and was developed earlier than the shortened anteroposterior length. Crouzon high and shallow orbital walls are distinctive. Maxillary widening developed before the malformation of sphenoid. The anteroposterior position of zygoma is likely a principal deformity, rather than a reflection of the intrinsic shape of the bone. Level of Evidence: II.

Reconstruction of acquired orbital deformity characterized by volume change.

Acquired orbital deformity is a common disease in the practice of craniofacial surgery. Defective orbital volume and abnormality of eyeball position are the most important characteristics of pathologic changes. This study includes 87 cases of acquired orbital deformity, which received surgery for volume aberration from 2002 to present. Among them, 73 cases received orbital volume expansion surgery and 14 cases received reduction surgery. Coronal scalp, lower eyelid, or intraoral gingival-buccal incisions were carried out for the approach. In some patients, the original scar around the orbit was chosen for the incision. Operation aims were reduction of orbit and reconstruction of the orbital wall integrity. Operative methods were osteotomy for reduction and implantation of autologous bone or artificial materials. Orbital volume and eyeball position were restored to normal in all patients after the operation, and no serious complications occurred. Treatment of acquired orbital deformity should make restoration of orbital volume as the most important target of therapy. Autologous bone should be the material of first choice, and the selection and amount of implanted material should be decided by the specialty and experience of the physician.

Midface distraction without osteotomies in an infant with upper respiratory obstruction.

Midface hypoplasia is a common craniofacial anomaly and may manifest as part of a wider syndrome or as an isolated finding. Underlying this condition is a complex morphology, resulting from development across multiple interacting suture systems. Current treatment relies on various combinations of osteotomies and distraction using internal or external devices. Such procedures, while often successful, involve significant morbidity and trauma to the very young patients whom comprise the majority of the treatment group. The present article describes the successful development of a technique for midfacial distraction without osteotomies. In a case study representing a series of 11 patients, a 4-month-old baby girl with midface hypoplasia and respiratory obstruction requiring nasopharyngeal intubation underwent this 2-stage procedure. After 72-day distraction, upper incisor-posterior clinoid process distance had increased 18.4 mm, and by 4 months after removal of the distraction devices, it had increased a further 2.6 mm. Craniofacial morphology was markedly improved combined with complete resolution of her respiratory obstruction. No blood transfusion or intensive care facility was required. This innovation demonstrates a procedure with similar efficacy but significantly reduced morbidity and cost compared with existing methods. Interestingly, continued growth after the distraction period may allude to a different mechanism of induced osteogenesis than previously described.

Combination of skin flap and silicone prosthesis for rehabilitation of a large orbital defect: A case report.

Exenteration surgery greatly affects a person in terms of function, esthetics, and psychological trauma. In such cases, restoration by silicone orbital prosthesis is a well-accepted treatment option. However, this is a difficult task, necessitating personalized design of method for each patient. This case report describes the technique for fabrication of a silicone orbital prosthesis for a male patient with left orbital defect due to exenteration of a Grade 3 squamous cell carcinoma of the left eye and surrounding tissues. The patient was delivered with a satisfactory silicone orbital prosthesis having good retention and finish. Multidisciplinary management and team approach are crucial in providing precise and effective rehabilitation for improving the patient's quality of life and help them return to their normal social life.

Orbital lymphatic-venous malformation with concomitant spontaneous orbital arteriovenous fistula: case report.

The authors describe the case of an 11-year-old boy with no history of prior trauma, who experienced acute proptosis and visual loss while showering. Diagnostic carotid artery angiography revealed an orbital lymphatic-venous malformation (LVM) concomitant with an intraorbital arteriovenous fistula. The patient was treated with transvenous coil and N-butyl cyanoacrylate glue embolization, combined with direct percutaneous glue injection into the LVM followed by excision. There was good clinical and radiological response without recurrence at the 2-year follow-up. Diagnostic carotid angiography should be considered in cases of orbital LVM, as a concomitant arteriovenous fistula might be present in rare cases.