Correction of hypertelorbitism: evaluation of relapse on long-term follow-up.

BACKGROUND: Hypertelorbitism has been associated with a variety of congenital deformities. Appropriate timing for surgical correction remains controversial. We present our long-term experience of 33 patients with hypertelorbitism undergoing facial bipartition or orbital box osteotomy. METHODS: Patients with hypertelorbitism treated with either facial bipartition or orbital box osteotomy and repositioning who had long-term follow-up were studied (n=33). Age at the time of first surgery, preoperative interdacryon distance, and immediate postoperative interdacryon distance were recorded. Relapse was determined on postoperative follow-up, and the need for secondary correction was noted. Physician satisfaction score (range, 0-4) was also assessed. RESULTS: Patients had a mean total follow-up of 14.0 years. With regard to age at the time of initial procedure, patients younger than 6 years were all noted to have relapse, and 83% underwent revision surgery. In patients 6 years or older, only 11% had relapse and required a second operation. Yet, satisfaction scores were similar (3.2 versus 3.5). With regard to the severity of hypertelorbitism, there was no relapse noted among patients with mild hypertelorbitism (interorbital distance [IOD], 30-34 mm). Among those with moderate hypertelorbitism (IOD, 35-40 mm), 29.4% developed relapse. By contrast, all patients with severe hypertelorbitism (IOD, >40 mm) were noted to have relapse requiring repeat correction. Satisfaction scores were similar (3.4 versus 3.3 versus 3.1). CONCLUSIONS: Relapse after surgery for hypertelorbitism is related to the age of the patient at correction and the preoperative severity. When possible, surgical repositioning of the orbits should be delayed until later childhood.

Stereolithography: a method for planning the surgical correction of the hypertelorism.

Hypertelorism is a deformity characterized by an increase in interorbital distance. The treatment can be orbital mobilization, or if the malformation goes along with occlusal alterations, the indicated treatment is a facial bipartition with hemifacial rotation.The intention of the present study was to describe a surgical planning technique in locating the anatomic points and planes on a stereolithographic model of a patient with 0-14 fissure and its surgical application.

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.

Tessier Clefts and Hypertelorism.

Tessier's classification system for rare craniofacial clefts remains the most widely used today. It denotes the position of the cleft process in a schema based around the orbit, and facilitates communication between surgeons regarding these complicated conditions. Tessier's classification is reviewed in detail, and a separate discussion of hypertelorism (increased distance between the bony orbits) follows, focusing on orbital hypertelorism in the setting of craniofacial clefts.

Orbital hypertelorism.

Excessive distance between the orbits is only one manifestation of a complex deformity that affects several skeletal and soft-tissue structures. This article discusses the classification, preoperative planning, and surgical procedures used in the reconstruction of orbital hypertelorism.

Hypertelorism: nosologic analysis of 90 patients.

The word hypertelorism is used to describe increased interorbital distance, a condition that is causally and pathogenically heterogeneous. Because not all wide-set eyes are the same, accurate terminology and nosology are critical to understanding and management. Orbital hypertelorism signifies an increased distance between both medial sides and lateral sides of the orbits. Interorbital hypertelorism denotes increased distance only between the inner orbital walls. In this retrospective analysis of 90 patients with hypertelorism, the most common cause was frontonasal malformation (n = 30), a heterogeneous category of nonfamilial disorders including a newly described subgroup, rugose frontonasal malformation. The second most common cause was craniofrontonasal dysplasia (n = 18), a genetic syndrome comprising coronal synostosis, frontonasal anomalies, "frizzy" hair, narrow/sloping shoulder girdle deformity, and longitudinal ridging of nails in association with various truncal and extremity anomalies. Paramedian craniofacial cleft(s) (n = 10) and (sincipital) encephalocele (n = 6) were infrequent causes of hypertelorism. The fifth, miscellaneous category comprised well-defined, mostly chromosomal and syndromic disorders (n = 26). Patients in the various diagnostic categories were designated as having either orbital or interorbital hypertelorism. Hypertelorism also was graded as either first, second, or third degree based on deviation from age- and gender-matched normative data. The etiology and type of hypertelorism influence the selection of operative procedure, whereas the grade of severity indicates the need for surgical correction.

[Syndromes 7. Hypertelorism (Greig syndrome)]. / Syndromen 7. Hypertelorisme (Greig-syndroom).

Hypertelorism is an increased distance between the orbits. Most evident symptom is the increased intercanthal distance. It can be caused by congenital deformities (facial clefts, encephaloceles, enlarged ethmoïds) and by trauma. Three degrees are differentiated. Grade one and two can be corrected with a small risk for complications. If there is vertical orbital dystopia it has to be corrected by a transcranial procedure, as is necessary in grade 3.

Improving results in orbital hypertelorism correction.

Between 1971 and 1977, 40 patients with orbital hypertelorism were operated on by the craniofacial team in Toronto. Results have improved as a result of this experience. Tomography in two planes has revealed four types of medial orbital wall deformity. Cephalographs proved inadequate to understand this three-dimensional problem. Mobilization of the orbits and their correct realignment, with removal of the precise amount of interorbital bone, are only preliminary steps in producing good results. Different techniques for reattachment of the medial canthal ligament, correct orientation of the lateral canthus, changes in methods of nasal excision, and the elimination of other facial or eyelid scars are helping to produce more normal looking patients. Simultaneous major intraoral procedures have been the greatest source of complications. Limitation of this surgery to one team has given sufficient experience to enable a diminution in morbidity and a halving in blood loss and time taken for the operation.