A critical appraisal of surgical outcomes following orbital hypertelorism correction: what is the incidence of true bony relapse versus soft tissue telecanthus?

BACKGROUND: Orbital hypertelorism (OHT) represents a congenital condition defined by lateralization of the bony orbit, unlike soft tissue telecanthus in which there is an increase in intercanthal distance without true bony lateralization. Existing literature remains very limited in its postoperative assessment of bony versus soft tissue relapse, which may both clinically present as telecanthus. We performed a critical appraisal of the literature to determine the postoperative incidence of bony versus soft tissue relapse following OHT repair. METHODS: The PubMed, MEDLINE, EMBASE, Scopus, Cochrane Central Register of Controlled Trials, and clinicaltrials.org were searched systematically for all English studies published in any time frame reporting relapse rates following primary OHT repair. The primary outcome was incidence of bony and soft tissue relapse defined as orbital lateralization and medial canthal drift, respectively. The secondary outcome measures include postoperative complications, predictors of postoperative complications, timing and type of surgery, and revision rates. RESULTS: Eleven articles were included. A total of 84 (35.3%) patients experienced bony relapse while 43 (27.2%) patients experienced soft tissue relapse. Age at time of intervention (p < 0.92), severity at presentation (p < 0.90), and surgical technique (p < 0.09) were not found be significantly associated with relapse rate. Methods for long-term follow-up were not standardized, and there was no consistent measure to objectively assess telecanthus. CONCLUSIONS: There is no general consensus on predictive factors of long-term relapse following OHT repair in the form of box osteotomy or facial bipartition. These findings call for cross-sectional outcome standardization to better understand long-term outcomes across institutional, provider, and patient differences.

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.

The relationship between external bony defects and widened lateral interorbital distance in frontoethmoidal encephalomeningocele.

PURPOSE: The aim of this study was to identify the prevalence of the abnormal lateral interorbital distance (LIOD), micropthalmia or anopthalmia and incidence of hydrocephalus in frontoethmoidal encephalomeningocele (FEEM), and to identify the correlation between the external bony defects and a widened lateral orbital wall. MATERIALS AND METHODS: FEEM patients who were treated in the Faculty of Medicine, Chiang Mai University between January 1, 2006, and December 31, 2016, were retrospectively reviewed from the medical records. We included the patients who had undergone head computed tomography (CT) of the facial bones. Exclusion criteria were any patients who had undergone prior facial surgery or had maxillofacial bone trauma before they underwent a CT scan, craniofacial disease such as facial clefts, frontonasal dysplasia, craniosynostosis, and inter-frontal encephalocele. Measurements were taken from the CT images to establish the diameter of the external bony defect, medial interorbital distance and lateral interorbital distance. The relationships between the external bony defect and widened LIOD were analyzed. Patients were divided into two groups: those with normal LIOD and those with wide LIOD. RESULTS: A total of 94 FEEM patients were evaluated. Thirteen patients (13.83%) had a wider than normal LIOD. The incidence of micropthalmia or anopthalmia was 9.57% and hydrocephalus was 45.74%. From a univariable model, there were no statistically significant differences in terms of patient characteristics between the LIOD groups, except for sex. The proportion of wide LIOD was higher in male patients (male vs female; 20.34% vs 2.86%, p = 0.027). The proportion between the external bony defect and the LIOD (EL ratio) associated with a wide LIOD was shown in a cutoff point of 23% (AuROC = 73.27%, 95%CI = 59.87%-86.66%). The multivariable analysis indicated factors associated with abnormal LIOD were an EL ratio of 23% (adjusted RR = 1.98, 95% CI = 1.58-2.51) and male sex (adjusted RR = 6.85, 95% CI = 2.00-23.55). CONCLUSION: There is an association between the EL ratio and widening of the LIOD. An EL ratio greater than 23% could be used to moderately predict a wide LIOD. A large external bony defect may be associated with lateral displacement of the orbit causing true hypertelorism. Therefore, orbital translocation must be considered in patients with a wide LIOD.

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.

Advance in diagnosis and treatment of craniofrontonasal syndrome / 中华整形外科杂志

Craniofrontonasal syndrome/dysplasia(CFNS/CFSD) is a rare X-linked malformation syndrome caused by EFNB1 gene mutations. It is characterized by unilateral or bilateral coronal craniosynostosis, hypertelorism, frontal bossing, broad or bifid nose, grooved nails, agennetic corpus callosum. Female are more commonly and more severely affected than males. Given the classic manifestation, specific hereditary, and complicated treatment, the diagnosis and treatment of CFNS is always a challenge. However, there is only a few studies related to CFNS, and consensus regarding its treatment has not been reached. Thereby, we summarized pertinent literatures and made a thorough review on the diagnosis and treatment of CFNS.

Primary aesthetic correction of nasal anomaly with costal graft in treating orbital hypertelorism.

Orbital hypertelorism is a common congenital craniofacial anomaly in Asians. As Tessier pointed out, the correction of hypertelorism is effectively the correction of the nasal deformity, which is characterized by a broad, flat nose accompanying an increased interorbital distance and a lack of nasal projection. OBJECTIVES: To measure the changes in the orbital and nasal parameters after Tessier box osteotomy and primary aesthetic rhinoplasty, performed concurrently with otogenous costochondral graft. METHODS: From 2009 to 2013, 49 hypertelorism patients were treated in our craniofacial unit. Correction involved a classic Tessier box osteotomy combined with aesthetic augmentation and reconstruction of the nasal dorsum using a costochondral graft. All patients underwent photographic and tomographic documentation both before and 12 months after surgery. Patients underwent morphometric analysis to document the change in interorbital distance, IOD, hypertelorism index, and aesthetic nasal parameters, including the nasal dorsum and alar width, the nasal dorsal height, and the index of nasal apex protrusion versus nasal length. RESULTS: All patients were satisfied with the outcome of the combined orbital and nasal repair. Morphometric analysis indicated that the IOD, hypertelorism index, and aesthetic nasal parameters all improved following surgery. The resorption rate of the costochondral graft was 16-19%, and graft warpage was nearly absent. Although complications included infection, cerebrospinal fluid leak, and hyposomia, no severe or life-threatening complications occurred. CONCLUSION: Tessier box osteotomy with primary aesthetic rhinoplasty using costochondral grafts has shown to be efficacious in the treatment of patients with hypertelorism.

Surgical correction of grade III hypertelorism.

Orbital hypertelorism is an increased distance between the bony orbits and can be caused by frontonasal malformations, craniofacial clefts, frontoethmoidal encephaloceles, glial tumors or dermoid cysts of the root of the nose, and various syndromic or chromosomal disorders. We report a series of 7 cases of hypertelorism that were treated in our hospital. The underlying causes in our series were craniofacial clefts 0 to 14 (4 cases), craniofacial clefts 1 to 12 (1 case), and frontonasal encephalocele (2 cases), all congenital. Surgical techniques used to correct the deformity were box osteotomy and medial wall osteotomy with or without calvarial and rib grafts. A few of our cases were reoperations with specific challenges.

Surgical correction of grade III hypertelorism

Orbital hypertelorism is an increased distance between the bony orbits and can be caused by frontonasal malformations, craniofacial clefts, frontoethmoidal encephaloceles, glial tumors or dermoid cysts of the root of the nose, and various syndromic or chromosomal disorders. We report a series of 7 cases of hypertelorism that were treated in our hospital. The underlying causes in our series were craniofacial clefts 0 to 14 (4 cases), craniofacial clefts 1 to 12 (1 case), and frontonasal encephalocele (2 cases), all congenital. Surgical techniques used to correct the deformity were box osteotomy and medial wall osteotomy with or without calvarial and rib grafts. A few of our cases were reoperations with specific challenges.

Orbital widening syndrome: etiology and therapeutic approaches / 中华医学美学美容杂志

Objective To evaluate the etiology,therapeutic approaches and prognosis of orbital hypertelorism.Methods Clinical data of 23 cases were summarized with review ot the literature.Results All operations provided satisfactory cosmetic effects.All the patients were followed up for 6 months to 5 years,and no recurrence were observed.Conclusions A good cosmetic effect can be obtained by operation in orbital hypertelorism.The preschool period the best opportunity,and stable effects could be gotten and the psychological development of children has not been influenced.The intracranial and extracranial joint pathway orbit box osteotomy still has drawbacks and requires to be improved.The etiology of gene and molecular aspects need to be explored.