Comparison of Piezosurgery and Conventional Osteotomy for Orbital Hypertelorism Surgical Correction.

The purpose of this study was to compare the safety and effect of piezosurgery with conventional osteotomy in a box-shifting procedure for orbital hypertelorism (ORH) correction surgery. This study retrospectively analyzed the clinical record of 10 ORH patients aged from 5 to 12 years, and they were second-degree ORH with an interorbital distance (IOD) of 35 to 37.8 mm. Three of them received the osteotomy with piezosurgery (the piezosurgery group), whereas the other 7 patients received osteotomy with the conventional osteotomy method (the control group). They were compared with age and preoperative IOD. All the patients' IOD was effectively improved to normal range after the surgery. The results showed that the application of piezosurgery did not prolong the surgery time (piezosurgery group: 8.3±0.5 hours; control group: 8.7±1.4 hours, P =0.68). Furthermore, the patients in the piezosurgery group had less drainage volume (piezosurgery group: 79.1±12 mL; the control group: 170±41.3 mL, P =0.0065) and shorter postoperative hospital stay (piezosurgery group: 8.3±2.0 d; control group: 12.43±2.29 d, P =0.029). There were 2 patients who had wound infections, 1 in the piezosurgery group and 1 in the control group, respectively. However, 1 patient in the control group suffered from cerebrospinal fluid leakage. On the basis of the results, the application of piezosurgery benefited the patients on a better and smoother recovery course with less drainage and shorter hospital stays. The advantages of piezosurgery are the fine and precise osteotomy and the protection for soft tissue, which make it a comparatively safe and effective tool for craniofacial surgery, especially for young patients.

Surgical Management of Hypertelorism: The 3D Animated Technical Note.

The hypertelorism surgery is a complex procedure requiring a long learning curve. Even though the box osteotomy technique is well described in literature, its representation is generally based on texts and illustrations that do not really give a 3-dimensional or a dynamic point of views. The authors present a 3-dimensional animated video, Supplemental Digital Content 1, http://links.lww.com/SCS/E561 showing the craniofacial osteotomies and focusing on the critical points to correct hypertelorism.

Reconstructive Operation of Severe Orbital Hypertelorism With Computer-Assisted Precise Virtual Plan.

Orbital hypertelorism correction is still a less precise procedure, with a simple preoperative design and surgical results often depending on the operator's experience. In recent years, computer-assisted technology has been fully utilized in craniofacial surgery. This article aims to explore the clinical results of computer-assisted technology in orbital hypertelorism correction and discuss its advantages and effects on treatment. Four patients with orbital hypertelorism underwent intracranial and extracranial combined box osteotomy correction. Preoperative computed tomography scans were performed, and 3-dimensional 3D digital technology was used to measure the orbital spacing, virtually design the 3D cutting scheme, and guide the intraoperative 3D cutting to improve the accuracy of periorbital osteotomy and reduce the surgical risk. Four patients underwent successful surgery, and the average distance of the medial orbital wall was decreased from 43.6 to 23.4 mm. Computer-assisted box osteotomy shortens the operative time and provides better corrective results.

ALX-Related Frontonasal Dysplasias: Clinical Characteristics and Surgical Management.

AIM: The term frontonasal dysplasia (FND) represents a spectrum of anomalies and its genetics have not been well defined. Recently, the critical role of the aristaless-like homeobox (ALX) gene family on the craniofacial development has been discovered. In the present study, we aimed to propose a systematic surgical treatment plan for the ALX-related FNDs according to the genotypic classification as well as demonstrating their clinical characteristics to help surgeons diagnose the underlying pathology accurately. DESIGN: Single-institution retrospective. SETTING: Tertiary health care. PATIENTS AND METHODS: Eighty-nine FND cases were evaluated. Eight of them had ALX1-related FND3, 3 had ALX3-related FND1, and 2 had ALX4-related FND2. Phenotype characteristics of ALX-related FNDs were evaluated, and relevant surgical interventions were assessed. RESULTS: The ALX1-related FND3 phenotype is striking due to the involvement of the eyes in addition to the presence of hypertelorism, facial clefts, and nasal deformities. A widened philtrum and prominent philtral columns are remarkable features of the ALX3-related FND1, whereas the ALX4-related FND2 has more severe deformities: severe hypertelorism, brachycephaly, large parietal bone defects, broad nasal dorsum, and alopecia. Facial bipartition, box osteotomies, eyelid coloboma repair, cleft lip and palate repair, nasal reconstruction, and fronto-orbital advancement can be performed in ALX-related FNDs based on the characteristics of each subtype. CONCLUSIONS: This genetic classification system will help surgeon diagnose patients with FND with unique features and draw a roadmap for their treatment with a better surgical perspective.

Craniofrontonasal dysplasia: hypertelorism correction in late presenting patients.

BACKGROUND: Craniofrontonasal dysplasia (CFND) is a rare congenital craniofacial syndrome characterized by single suture synostosis, hypertelorism, other clinical facial features, and abnormalities in the upper extremities. There are only a few studies in the applicable literature that address hypertelorism management for CFND patients and outcomes and complication rates. METHODS: A retrospective study was performed on consecutive late presenting CFND patients referred to our hospital with substantially completed craniofacial skeleton growth, who underwent hypertelorism correction between 2007 and 2019 following intracranial pressure screening, and who received at least 1 year of follow-up care. None of the patients in this study underwent prior craniofacial surgery. Only those patients with a confirmed mutation of the EFNB1 gene were included in this study. All patients in this study underwent hypertelorism correction by facial bipartition or box osteotomy. RESULTS: A total of ten late presenting CFND patients (all female) were treated at our hospital during the study period. None of the patients presented signs of elevated intracranial pressure. The average patient age at hypertelorism correction was 13.4 ± 7.68 years of age. Major complications, defined as complications requiring a return to the operating room, were limited to infection of the frontal bone, which required partial bone removal, and cerebrospinal fluid (CSF) leak, which was completely resolved by insertion of a lumbar shunt for a 7-day period. CONCLUSION: The absence of elevated intracranial pressure enables hypertelorism correction in late presenting CFND patients via facial bipartition or box osteotomy without the need for additional operations that provide for cranial expansion.

Orbital Hypertelorism Correction With Inverted-U Osteotomy: Indications and Advantages.

ABSTRACT: This study aimed to explore the clinical effects of inverted-U osteotomy in correcting orbital hypertelorism and to further explore its indications and advantages; 4 patients with orbital hypertelorism and premature closure of unilateral coronal suture underwent inverted-U osteotomy. The interorbital distance was measured using three-dimensional digital technology pre- and postoperatively. Virtual preoperative planning and three-dimensional cutting guide were used to minimize surgical risk and increase the precision of periorbital osteotomy. The procedure was successfully performed on 4 patients; the average orbital medial wall distance was reduced from 34.9 mm to 23.1 mm. Inverted-U osteotomy is a modified surgical method especially applicable to orbital hypertelorism with premature closure of unilateral coronal suture. Advantages include less surgical trauma, a shorter operative time, and better correction results.

Long-Term Photogrammetric- and Panel Assessment-Based Outcome Study of Staged Reconstructive Approach for Hypertelorism Correction.

BACKGROUND: The surgical management of hypertelorism is challenging for plastic surgeons, and limited long-term outcome data are available. The purpose of this long-term study was to report a single-surgeon experience with a staged reconstructive protocol for hypertelorism correction. METHODS: This retrospective study reviewed the records of patients with hypertelorism who were surgically managed by a single surgeon between 1978 and 2000. Bone (orbital box osteotomy and orthognathic surgery) and soft tissue (rhinoplasty and epicanthoplasty) surgeries were performed based on a patient-specific surgical protocol. Included patients were divided into a childhood group and an adolescence or adulthood group according to their age at orbital repositioning (≤12 and >12 years, respectively). Patients were invited for clinical interviews in February 2020 to evaluate whether requests for revision surgery had been made. The photogrammetric analysis-based hypertelorism index was calculated at preoperative and long-term postoperative times. Satisfaction with the long-term outcome was judged by both surgical professionals and laypeople. RESULTS: In total, 14 patients with hypertelorism of different etiologies were included, with no request for revision surgery during an average follow-up of 29 years. The preoperative hypertelorism index was higher than the long-term postoperative evaluation (all, P < 0.05) for both childhood and adolescence or adulthood groups. Intergroup comparison revealed no significant difference for the hypertelorism index and panel assessment-based satisfaction with long-term outcome analysis (all, P > 0.05). CONCLUSIONS: Considering the complexity and wide spectrum of clinical presentation of soft tissue and bone deformities in hypertelorism and current outcomes, the surgical approach to these patients should be staged and individualized for achievement of a balanced result between functional (orbital, occlusion, and psychosocial) and aesthetic parameters.

Strategy for Bone Conservation in the Two-Stage Correction of Hypertelorism in Craniofrontonasal Dysplasia.

Pre-emptive planning to optimize safety and outcome is a defining principle of pediatric craniofacial surgery, in which "procedures that come before" should enhance and not compromise procedures that are planned to follow.A technical modification of fronto-orbital advancement/remodeling in 2-stage hypertelorism surgery for craniofrontonasal dysplasia is presented, where orbital hypertelorism is accompanied by coronal craniosynostosis. Fronto-orbital advancement/remodeling undertaken by this approach preserves bone in the supero-lateral bandeau and provides optimal quality bone stock for the subsequent orbital box shift osteotomy. In this way the second procedure is protected and enhanced by optimal planning of the primary procedure, with potential benefits to safety, quality, and outcome of the final result.

How Accurate Is Computer-Assisted Orbital Hypertelorism Surgery? Comparison of the Three-Dimensional Surgical Planning with the Postoperative Outcomes.

Importance: Surgery of orbital hypertelorism (ORH) remains an imprecise surgical procedure depending on the experience and habits of the craniofacial surgical teams. Computer-assisted surgery (CAS) has developed dramatically in craniofacial surgery, but there is no current study assessing its accuracy for ORH surgery. Objective: This study aimed to assess the input of CAS and especially the accuracy of the cutting guide-based procedures. The authors presented the computer-assisted box osteotomy or facial bipartition techniques and compared the preoperative surgical planning with the postoperative results. Design, Setting, and Participants: A monocentric retrospective study included the patients who underwent surgical correction of ORH from 2016 to 2019 at the University Hospital Center of Tours, France. All the patients had a computer-assisted orbital hypertelorism surgery (CAOHS) using cutting guides and tailored fixation plates. Of 10 patients included, 7 were treated by box osteotomies and 3 by facial bipartitions. Intervention: Each patient had a preoperative computed tomography (CT) scan enabling a virtual simulation of the reconstruction and the manufacturing of patient-specific cutting guides and customized osteosynthesis plates. The postoperative CT scans were compared with the three-dimensional (3D) virtual simulation using the distances between the sagittal plane and orbital and infraorbital reference points, and from the measurement of the orbital advancement (i.e., 10 reference measurements). Results: All patients had satisfactory clinical and aesthetical outcomes with a mean interorbital distance of 22.8 ± 2.8 mm. The postoperative measurements were significantly higher than for the surgical planning (p < 0.0001). The average absolute differences between the 3D virtual planning and the postoperative CT scans were <1.30, 1.90, and 0.80 mm for the orbital, infraorbital, and orbital advancement measurements, respectively. The overall accuracy of the CAOHS (root mean square deviation) was 1.39 mm. Conclusions: The use of computer-assisted design and computer-aided manufacturing device, such as cutting guides and tailored plates, facilitates the bony surgical correction of ORH using box osteotomy or facial bipartition and allows for valuable, reproducible, and satisfactory clinical outcomes.

Fronto-Orbital Advance in a Patient With Roberts Syndrome.

Roberts Syndrome is an extremely rare syndrome reporting about 150 cases in the literature, with a very low survival rate. The authors present a case of a female patient with Roberts Syndrome who also had a coronal craniosynostosis. The aim of this case report is to present a case of a patient with Roberts Syndrome with a brachycephaly that required management of fronto-orbital advancement. In conclusion Roberts Syndrome is a rare disease, which can have different skeletal variations. This syndrome can manifest itself with craniosynostosis, with the requirement of a comprehensive management to correct it and avoid compression of the brain with endocranial hypertension.

Postoperative Complications of Box-Shift Osteotomy for Orbital Hypertelorism.

OBJECTIVE: To summarize and analyze the postoperative complications of box-shift osteotomy performed at our center for Chinese orbital hypertelorism patients from 2008 to 2017. METHOD: This retrospective study reviews the records of 78 patients with complete medical records and at least 2 years of postoperative follow-up data. Both radiologic and anthropometric assessments were conducted before, 1 month after and 2 years after surgery to evaluate the bony and soft-tissue alterations. Postoperative complications were recorded during hospitalization and at each follow-up visit and divided into 3 groups: acute complications that occurred within 1 month after surgery; early complications that occurred within 6 months after surgery; and long-term complications that occurred within 2 years after surgery. RESULTS: Both bony and soft-tissue alterations were significant at 1 month after surgery. The acute complications that occurred in our center included infection (12.8%), cerebrospinal fluid leakage (29.5%), epilepsy (2.6%), and nasal tip skin necrosis (1.3%). The early complications included strabismus (11.5%) and nasolacrimal duct obstruction (3.8%). The long-term complications included insufficient correction (55.1%), palpable metal implants (92.3%) and a drooping nasal tip (33.9%). Due to the insufficient correction and the continued growth of rib graft, the difference in the hypertelorism index and nasal length, between one month and 2 years postoperatively were statistically significant (P < 0.01). Other radiographic and anthropometric measurements changed with growth without a significance difference between 1 month and 2 years after surgery. CONCLUSION: In this study, we recorded all postoperative complications of box-shift osteotomy. The challenge of our future work is to identify methods for decreasing the incidence of these complications.

Addressing hypertelorism: Indications and techniques.

From its first descriptions in the early 1920s to today's use of cutting guides via computer-assisted surgery, surgical techniques to address hypertelorism have progressed. The present article aims to provide historical background and an overview of the development of surgical techniques during the late 20th century and in recent years. First, a historical overview identifies the most important surgical advances leading to the present state of the art. Each major surgical innovation is described, to explain the changes in this surgical field, according to the type of approach. Then, a precise description of today's most recent practices is provided, with particular emphasis on the spectacular advances deriving from computer-assisted surgery. A thorough description of the use of cutting guides throughout the surgical phase is given.

Surgical treatment of orbital hypertelorism: Historical evolution and development prospects.

Orbital hypertelorism (OR.H) is defined as an abnormal increase in the distance between the two orbits secondary to a skeletal anomaly, and it occurs in association with numerous congenital craniofacial malformations. Since its description by Greig in 1924, OR.H and the associated corrective procedures have captivated many surgeons. Here we present a discussion of the historical evolution of surgery for OR.H and highlight its future prospects. In the mid-twentieth century, only cover-up techniques simulating approximation of the eyes via an optical illusion were used, such as frontonasal skin resection, epicanthal fold surgery, and rhinoplasty. Subsequently, numerous surgeons attempted to correct the deformation using orbitonasal osteotomies via an extracranial approach. However, the outcomes were largely inadequate. Finally, in 1967, Tessier developed an efficient two-stage technique for OR.H correction via an intracranial approach; this technique revolutionized the management of OR.H. In 1970, Converse refined Tessier's procedure by performing a one-stage surgery that preserved olfaction. In 1976, Van Der Meulen developed the facial bipartition technique, which simultaneously corrected maxillary and craniofacial deformities. Box osteotomies and facial bipartition are still used for the correction of OR.H. Using the technological advancements introduced in the early 2000s, several surgeons have attempted to improve these techniques with the use of three-dimensional (3D) surgical planning, preoperative 3D printing, augmented reality-based surgical navigation, and computer assisted surgery using cutting guides. These modern-day practices are rapidly developing and are expected to refine and standardize the surgical correction of OR.H in the future.

Treating Craniofacial Dysostoses with Hypertelorism by Monobloc Facial Bipartition Distraction: Surgical and Educational Videos.

Simultaneously advancing and medializing the orbital segments in a stable bone bloc constitutes a major advancement in craniofacial surgery. Monobloc bipartition enables destigmatization of the syndromic face by correcting the abnormal orbital axis and interorbital distance. The authors stratified this complex surgical approach into five major steps to facilitate a holistic understanding of the surgical sequence. The rationale for latency and activation periods and the advantages and disadvantages of this technique are described.

Controversy on the Age of Hypertelorism Correction.

INTRODUCTION: Orbital hypertelorism (HTO) is a challenging craniofacial problem seen in association with some congenital deformities. The age of HTO correction is a matter of debate. THE AIM OF THE WORK: to evaluate the outcome of HTO correction and determine the optimal timing for intervention, striving for the earliest possible intervention with the lowest relapse. PATIENTS AND METHODS: A standard craniofacial approach with medial bone resection, 4 walls orbital box osteotomy and orbital medialization were done for all patients. Skeletal and soft tissue procedures were done as indicated. RESULTS: there were 10 patients aging 6 to 19 years. Seven were associated with craniofacial clefts, and 3 with craniosynostosis syndromes. HTO was severe in 8 cases and moderate in 2 cases. It was asymmetric in 2 cases. Frontoorbital remodeling was done in 3 cases with craniosynostosis. Failed surgery was reported in 2 cases. A redo surgery was done for one of them with an excellent outcome, while refused by the other. Nine patients had an excellent outcome. The mean level of satisfaction was 93.37%. Three patients had ugly facial scars. No major complications were recorded. CONCLUSION: The time for surgical treatment of HTO is determined by the severity of the associated deformity. If there is an urgent factor indicating intervention, early correction can be performed exceptionally; otherwise, HTO correction should be performed after the age of 6 years.

Reconstrucción palpebral multidisciplinar en el síndrome de Barber-Say: a propósito de un caso / Multidisciplinary eyelid reconstruction in Barber-Say syndrome: A case report

El síndrome de Barber-Say es una infrecuente displasia producida por la mutación del gen TWIST2 (2q37.3) que codifica una proteína que actúa a nivel epigenético. Presentamos el caso de un niño de 2 días en el que el ectropion, el hipertelorismo, la hipertricosis y demás rasgos dismórficos condujeron al diagnóstico clínico de síndrome de Barber-Say, posteriormente confirmado genéticamente. Alrededor de 20 casos se han publicado sobre este síndrome; sin embargo, en menos de la mitad se describe la técnica para abordar las malformaciones palpebrales, lo que supuso un reto quirúrgico. Nuestra actuación incluyó una tarsorrafia lateral y la toma de injertos de piel de la superficie palmar del antebrazo, área retroauricular y fosa supraclavicular, así como injertos grasos de la superficie interna de ambos muslos para la reconstrucción palpebral. Se trata del primer caso de síndrome de Barber-Say en el que se utilizan injertos supraclaviculares y del antebrazo

Barber-Say syndrome is an unusual dysplasia caused by the mutation of the TWIST2 gene (2q37.3), which encodes a protein that acts at an epigenetic level. The case is presented of a 2-day-old male child in whom ectropion, hypertelorism, hypertrichosis and other dysmorphic features led to the clinical diagnosis of Barber-Say syndrome, which was later confirmed with genetic tests. Around 20 cases have been reported on this syndrome, of which less than half have described the surgical technique, as it represents a surgical challenge. The approach in this case included a lateral tarsorrhaphy and skin grafts taken from the volar surface of the forearm, retroauricular area and supraclavicular fossa, as well as autologous lipografts from the inner side of both thighs for palpebral reconstruction. This is the first case of Barber-Say syndrome in which the use of skin grafts are taken from supraclavicular fossa and forearms

State-of-the-Art Hypertelorism Management.

Orbital hypertelorism represents lateralization of the orbits, meaning increased interorbital and outer orbital distances. Interorbital hypertelorism represents a failure of medial orbital wall medialization in the setting of normally positioned lateral orbital walls. The etiology and type of hypertelorism influence selection of an operative procedure, whereas the severity of deformity dictates surgical need. Choice of surgical procedure is dictated by anatomic considerations, such as degree of orbital hypertelorism, midfacial proportions, and occlusal status.

Multidisciplinary eyelid reconstruction in Barber-Say syndrome: A case report. / Reconstrucción palpebral multidisciplinar en el síndrome de Barber-Say: a propósito de un caso.

Barber-Say syndrome is an unusual dysplasia caused by the mutation of the TWIST2 gene (2q37.3), which encodes a protein that acts at an epigenetic level. The case is presented of a 2-day-old male child in whom ectropion, hypertelorism, hypertrichosis and other dysmorphic features led to the clinical diagnosis of Barber-Say syndrome, which was later confirmed with genetic tests. Around 20 cases have been reported on this syndrome, of which less than half have described the surgical technique, as it represents a surgical challenge. The approach in this case included a lateral tarsorrhaphy and skin grafts taken from the volar surface of the forearm, retroauricular area and supraclavicular fossa, as well as autologous lipografts from the inner side of both thighs for palpebral reconstruction. This is the first case of Barber-Say syndrome in which the use of skin grafts are taken from supraclavicular fossa and forearms.

Hypertelorism Secondary to Mucocele in the Paranasal Sinuses.

Ocular hypertelorism was introduced by Greig as an increased interpupillary distance. The paranasal sinus mucoceles are acquired lesions for various reasons; however, their behavior is progressive, capable of eroding the bone and extending to the orbital and intracranial regions. The objective is to present a clinical case of orbital hypertelorism secondary to mucocele in the paranasal sinuses. This is a 72-year-old male patient who came presenting an increase in volume in the right nasoorbitoethmoidal region. The isodense lesion occupying the maxillary and right ethmoidal sinuses was confirmed by an intimate relationship with the ipsilateral frontal and sphenoidal sinus, with osteolytic involvement of the orbit and nasal region. After incisional biopsy with mucocele results, a wide resection plus facial reconstruction was performed with autologous grafts and osteosynthesis material. Currently, the patient has 1 year of evolution, without significant functional commitment. It is important to consider giant mucoceles as part of the differential diagnoses in patients with deformities in the middle and upper third of the face.

Clinical Description, Molecular Analysis of TWIST2 Gene, and Surgical Treatment in a Patient With Barber-Say Syndrome.

Barber-Say syndrome is a rare autosomal dominant disease characterized by dysmorphic features, mainly of the eyelids and skin. It is caused by heterozygous mutations in gene TWIST2, localized in chromosome 2q37.3. The authors present the case of a pediatric patient with a clinical diagnosis of Barber-Say syndrome with ocular symptoms related to exposure keratitis. Molecular analysis of her DNA revealed a mutation on TWIST2 gene confirming the diagnosis of Barber-Say syndrome. Surgical treatment of the patient's eyelids resolved her signs and symptoms.