"A Simplified Approach for Surgical Correction of Vertical Orbital Dystopia: A 45-year Retrospective Cohort Study."

INTRODUCTION: Vertical Orbital Dystopia (VOD) results in significant facial asymmetry, psychological distress, and poor quality of life in affected patients. The traditional approach (TA) for surgical correction has entailed a standard frontal craniotomy along with circumferential orbital osteotomy, vertical translocation of the orbit, and bone grafting to the lower maxilla. Caution has been expressed regarding its invasive transcranial nature. In this report, we describe the limited approach (LA) for simplified surgical correction of VOD, which obviates the need for a standard frontal craniotomy. METHODS: A 45-year retrospective review was conducted of all patients that underwent surgical correction of VOD, as performed by a single surgeon. Demographic details, procedural characteristics, and complications were compared between patients who were corrected by the TA and those who were corrected by the LA. Complications were defined as CSF leak, infection of the frontal bone, permanent diplopia, permanent ptosis, sudden-onset vision loss, persistent asymmetry, and surgical revision. RESULTS: Forty patients met inclusion criteria for correction of true VOD, of which 18 underwent the TA and 22 underwent the LA. Mean length of hospital stay was 5.3±2.3 days and 4.0±1.5 days for the TA and LA cohorts, respectively. Mean follow-up time was 4.9±7.5 years for the TA and 2.6±3.3 years for the LA. The only reported complications were persistent asymmetry in 2 patients in the TA cohort with one patient requiring surgical revision due to undercorrection, while the LA cohort exhibited no postoperative asymmetry or need for surgical revision. CONCLUSIONS: Both the TA and LA are effective for surgical correction of VOD. The limited craniotomy of the LA reduces exposure of intracranial structures and adequately achieves postoperative symmetry.

An Oncoplastic Approach to Primary Pediatric Pterygomaxillary Osteosarcoma.

Osteosarcomas arising within the pterygomaxillary/infratemporal fossa region are rare among the pediatric population. Survival rates are most influenced by tumor resection with negative margins, which can be dependent on surgical accessibility of the tumor site. The pterygomaxillary/infratemporal fossa location poses several challenges to safe and adequate tumor resection, including proximity of the facial nerve and great vessels and scarring associated with traditional transfacial approaches. In this article, we present the case of a 6-year-old boy with an osteosarcoma of the left pterygomaxillary/infratemporal fossa region successfully managed with an "oncoplastic" approach, incorporating the use of CAD/CAM and mixed reality technologies.

Gingivosupraperiosteoplasty following Presurgical Maxillary Orthopedics Is Associated with Normal Midface Growth in Complete Unilateral and Bilateral Cleft Patients at Mixed Dentition.

BACKGROUND: Passive orthodontic appliances and gingivosupraperiosteoplasty are adjuncts that can be used by surgeons at the time of primary cleft lip repair. These treatments, along with the surgical technique of cleft lip and palate repair, may impact midface growth. The objective of this study was to describe the authors' protocol for unilateral and bilateral cleft lip repair and to evaluate midfacial growth in a cohort of patients at mixed dentition who had undergone presurgical passive orthodontic appliance therapy and gingivosupraperiosteoplasty at the time of unilateral and bilateral cleft lip repair. METHODS: Fifteen complete unilateral and 15 complete bilateral cleft lip and palate patients underwent passive orthodontic appliance treatment and primary lip repair with gingivosupraperiosteoplasty. Lateral cephalograms were analyzed by three blinded reviewers. Mean cephalometric measurements at mixed dentition were compared to cephalometric values for noncleft patients, unilateral cleft lip and palate patients who did not undergo gingivoperiosteoplasty or presurgical treatment, and unilateral cleft lip and palate patients who underwent gingivoperiosteoplasty/nasoalveolar molding with independent samples t tests. RESULTS: Mean cephalometric values were within age-specific normal values for sella-nasion-A point, sella-nasion-B point, A point-nasion-B point, and facial axis. Eighty-seven (13/15) percent of unilateral cleft lip and palate patients and 93 percent (14/15) of bilateral cleft lip and palate patients did not exhibit skeletal class III malocclusion. There was no significant difference between cephalometric values for our patients and patients who did not receive gingivosupraperiosteoplasty or presurgical treatment or who underwent the gingivoperiosteoplasty/nasoalveolar molding protocol. CONCLUSIONS: Presurgical passive orthodontic appliances, combined with gingivosupraperiosteoplasty at the time of lip repair, leads to normal maxillary development in most patients at mixed dentition. Assessment of midface growth at skeletal maturity is required. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Surgical Management of Facial Port-Wine Stain in Sturge Weber Syndrome.

Sturge Weber Syndrome is characterized by the classic triad of a facial port-wine stain, leptomeningeal angiomatosis, and glaucoma. The resultant facial vascular anomaly can lead to soft tissue and bone irregularities, causing psychosocial distress and mental health morbidity. When severe, patients can opt for multi-staged surgical intervention by reconstructive surgeons to restore normal symmetry and improve the aesthetic appearance of the face. This study reports a case of surgical correction for severe facial vascular malformation resulting in poor outcomes due to the associated mental comorbidities seen in Sturge Weber Syndrome. A 37-year-old male with previously diagnosed Sturge Weber Syndrome presented to the outpatient craniofacial clinic for surgical evaluation of a large facial tuberous hemangioma. The patient underwent multiple operations for facial reconstruction including a staged full-thickness skin graft, facial recontouring, and extracranial correction of vertical orbital dystopia. The case was complicated by the patient's poorly controlled seizure disorder and psychosocial illness, resulting in self-mutilation of the repair and poor follow-up. Over the span of 10 years, the patient's mental illness caused him to fail numerous attempts at facial restoration and ultimately led to a poor final result. The psychosocial distress seen in patients with Sturge Weber Syndrome can adversely affect surgical outcomes. Physicians should be mindful of the possible complications that can arise in these patients and have the clinical means to address them.

Surgical Management of a Mild Case of Frontonasal Dysplasia: A Case Report and Review of Literature.

Frontonasal dysplasia (FND) is a rare congenital craniofacial cleft syndrome associated with a spectrum of midline facial bone and soft-tissue malformations. When present, the physical features of FND are often obvious and classified at birth. The resultant facial deformities have the potential to negatively influence psychosocial health and quality of life. Reconstructive surgical intervention in early childhood can serve to restore facial contour and alleviate psychological stress. In this report, a case of a 14-year-old female with previously undiagnosed mild form of FND presented for reconstructive surgery evaluation and underwent several procedures including sliding advanced genioplasty, submucosal resection of the nasal turbinates, open rhinoplasty, and bilateral transnasal medial canthopexies. The patient had subsequent nasal tip recontouring for persistent supratip fullness. The patient achieved an acceptable esthetic outcome and was satisfied with her physical appearance. This case emphasizes the subtle presentation and reconstructive surgical options of a mild case of FND that was diagnosed at a later age, unlike the more severe phenotypes of the syndrome and other common craniofacial anomalies that are usually diagnosed and treated in early childhood. Multidisciplinary craniofacial care teams should be able to correctly diagnose and implement the appropriate surgical interventions in patients with milder forms of FND.

The arrhinias: Proboscis lateralis literature review and surgical update.

Proboscis lateralis (PL) is a rare malformation, reported for the first time in 1861 by Forster in his monograph on congenital malformations of the human body. The abnormal side of the nose is represented by a tube-like rudimentary nasal structure, attached at any point along the embryonic fusion line between the anterior maxilla and the frontonasal processes. As clefts of the lip (and alveolus) are bilateral or unilateral, an arrhinia can be bilateral (total) or unilateral. In this case it is a 'hemi-arrhinia' (or heminasal agenesis. The arrhinias represent three groups of anomalies, each with different levels of clinical severity, some involving association with the labio-palatal cleft or agenesia of the premaxilla (1). In PL the nasal cavity on the affected side is replaced by a tubular appendage located off-center from the midline of the face, arising commonly from the medial aspect of the roof of the orbit (2). It is usually associated with heminasal aplasia or hypoplasia, microphthalmia, and - less commonly - with midline clefting. Associated brain and cranial vault anomalies are seen in 19% of these patients. PL is usually unilateral, with very few symmetrical/bilateral cases being reported (3). Morpho-aesthetic and psychological problems are frequent concerns for the patients and their families. In this study, the authors describe a clinical case and the chosen surgical technique, as well as reviewing the alternative techniques present in the literature.

Autogenous Bone Cranioplasty: Review of a 42-Year Experience by a Single Surgeon.

BACKGROUND: Autogenous bone is frequently espoused as the gold standard material for cranioplasty procedures, yet alloplastic cranioplasty continues to persist in the search, presumably, for a simpler technique. Although short-term outcomes can be successful using foreign materials, long-term follow-up in these patients often demonstrates increased rates of failure because of exposure or late infection. Autogenous bone grafts, however, integrate and revascularize, and are thus more resistant to infection than alloplastic materials. METHODS: This is a retrospective review of all patients that underwent reconstructive cranioplasty for full-thickness defects, as performed by the senior author (S.A.W.) between 1975 and 2018. All procedures were performed with autogenous bone. RESULTS: One hundred fifty-four patients met criteria for inclusion in the report. Cranioplasties were performed for both congenital and secondary indications. Split calvaria was used in 115 patients (74.7 percent), rib graft was used in 12 patients (7.8 percent), iliac crest graft was used in 10 patients (6.5 percent), and combinations of donor-site grafts were used in 17 patients (11.0 percent). In the entire series, none of the patients suffered from complications related to infection of either the donor site or transferred bone graft. None of the patients required secondary operations to fill in defects created by the postoperative resorption. CONCLUSIONS: Although autologous bone is widely considered the gold standard material for cranioplasty procedures, some argue against its use, mainly citing unpredictable resorption as the purported disadvantage. However, it is less susceptible to infection, and results in fewer long-term complications than alloplastic materials. There is no alloplastic material that has matched these outcomes, and thus autogenous bone should be considered as the primary option for cranioplasty procedures. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Squamosal Suture Synostosis: Incidence, Associations, and Implications for Treatment.

Squamosal suture craniosynostosis is thought to be a relatively rare entity. In the authors' experience, it is underreported in imaging examinations and the existing literature. The authors sought to determine the incidence of squamosal synostosis, whether it is increasing in frequency, and its relationship with synostosis of the major calvarial sutures.Patients undergoing computed tomography imaging for suspected craniosynostosis over a 15-year period were reviewed by a plastic surgeon and pediatric neuroradiologist. Patients with synostosis of the squamosal sutures were identified and involvement of additional sutures, gender, and the presence of a known syndromic diagnosis were recorded. Patients greater than 4 years of age or those with prior craniofacial surgery were excluded.One hundred twenty-five patients met inclusion criteria, 26 of whom had squamosal suture synostosis (26/125, 20.8%). Squamosal synostosis was found in isolation in 3 patients (3/26, 11.5%), with 1 additional major suture in 10 patients (10/26, 38.5%), and ≥2 major sutures in 13 patients (13/26, 50%). Squamosal synostosis was more common in patients with a syndromic diagnosis (11/26 syndromic, 15/99 nonsyndromic, P < 0.001). Eleven of 26 patients with squamosal synostosis were identified in the radiology report (42.3%).Craniosynostosis of the squamosal suture is much more common than previously reported and can contribute to abnormal head shape in isolation, or in combination with major sutures. Squamosal suture synostosis is underdiagnosed clinically and radiologically, although insufficient evidence exists to determine if its true incidence is increasing.

Fibrous dysplasia: management of the optic canal.

BACKGROUND: Fibrous dysplasia is an abnormal growth of bone that can lead to severe facial disfigurement. A dreaded outcome is compression of the optic nerve, leading to blindness. Controversy has surrounded the role of optic nerve unroofing for circumferential involvement of the optic canal. At present, many neurosurgeons unroof the nerve therapeutically in the setting of optic nerve dysfunction. Prophylactic unroofing (i.e., unroofing the nerve prior to the development of visual symptoms) has been previously proposed, although reported outcomes have been mixed. The authors present their long-term results of patients who have undergone optic nerve unroofing. METHODS: From 1975 to 2012, patients with fibrous dysplasia were investigated. Their age, demographics, operative procedure, optic nerve involvement (radiologically and clinically), and long-term outcomes and complications were recorded. RESULTS: Over 37 years, the senior author (S.A.W.) operated on 32 patients with fibrous dysplasia. Average follow-up was 5 years. Nine patients underwent optic nerve unroofing. Two patients had bilateral unroofing. Three patients who underwent therapeutic optic nerve unroofing ultimately went on to complete vision loss. The remaining seven patients who underwent prophylactic unroofing had no immediate postoperative visual compromise. CONCLUSIONS: Therapeutic optic nerve unroofing is advocated in fibrous dysplasia patients with continuous deterioration of vision. However, the authors believe prophylactic unroofing is safe, and it should be performed not necessarily as a primary surgical procedure, but as a procedure along with excision of fibrous dysplasia in the anterior skull base during the same operation performed for orbitocranial deformity. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Lower jaw reconstruction and dental rehabilitation after war injuries: The experience of Paul Tessier in Iran in the late 1980s.

Iraq-Iran war resulted in more than 400,000 people requiring prolonged medical care in Iran. An international team of prominent reconstructive surgeons led by Paul Tessier, the founder of craniofacial surgery, was invited to Iran during the war by official organizations entitled to support war victims. This team provided up-to-date oral and maxillofacial rehabilitation to patients with severe trauma defects in the lower third of the face. We collected the medical notes of 43 patients operated on by the Tessier team in Iran in the 1980s (files property of AFCF). The parameters we collected were: age of the patient, nature of the trauma (when available), previous procedures, number of implants placed (mandibular and maxillary), associated procedures (bone grafts, soft-tissue procedures, orthognathic surgery). A protocol based on soft-tissue rehabilitation using local flaps, parietal or iliac bone grafts and implant placement 6 months later was used in all patients. Paul Tessier's approach emphasizes the importance of keeping high standards of care in difficult situations and maintaining standard protocols.

Paul Tessier facial reconstruction in 1970 Iran, a series of post-noma defects.

BACKGROUND: Paul Tessier was a pioneering plastic surgeon who founded craniofacial surgery and had an international influence in the field of reconstructive surgery. We reviewed his techniques in the reconstruction of post-noma defects in Iran in the late 1970s. PATIENTS AND METHODS: We studied a series of 23 patients operated on by Tessier from 1974 to 1978 in Iran (property of Association Française des Chirurgiens de la Face). They all suffered from noma in childhood with major facial defects. RESULTS: Ten suffered from simple lip and cheek defects, nine also from nose defects and four from extensive facial defects. Abbe flaps were used in 15 patients to reconstruct the lips completed by commissuroplasty in six patients. Nose defects were reconstructed with nasofrontal flaps (ten cases). The outer cheek was reconstructed with a rotation flap (four cases), or with a frontotemporal flap (six cases). The inner cheek was reconstructed using a Barron-Tessier myocutaneous flap (ten cases). Of the 23 patients, partial flap necrosis occurred in five cases. CONCLUSIONS: Tessier was a pioneering plastic surgeon who used local flaps to reconstruct these important facial defects. He had a high rate of success, although nowadays local flaps are commonly replaced by free flaps.

Staged rotation advancements provide improved nasal results compared to 1-stage repairs in patients with complete bilateral cleft lip and palate.

After an experience of 32 complete bilateral cleft patients treated with staged rotation advancement to lip repairs and McComb nasal corrections during an 8-year period, improved nasal results with columella and lobule of normal dimensions have been noted, and the author feels that this method can be recommended for general use.