Orbital hypertelorism. II. Definite treatment of orbital hypertelorism (OR.H.) by craniofacial or by extracranial osteotomies.
Explore the source record for details and available documents.
Craniofacial Dysostosis↗
SEARCH · Search PubMed
Results for “HYPERTELORISM”
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
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.
Adolescent↗
[Ocular findings associated with hypertelorism].
PURPOSE: To show that in hypertelorism (a horizontal orbital dystopia in which interorbital distance is increased) there are various ophthalmologic abnormalities associated: strabismus, nystagmus, amblyopia, refractive errors, palpebral malpositions, lacrimal system imperforation, fundoscopic and anterior segment lesions. We want to show that the frequence and severity of ophthalmic findings are associated to the severity of hypertelorism. But the evolution and treatment of ocular findings is independent from the treatment of this orbital dystopia. METHODS: We have studied 12 cases of hypertelorism. We made a previous ophthalmologic examination which included: visual acuity, ocular motility, cycloplegic refraction, palpebral statics, lacrimal system, slit lamp and fundoscopic examination. All four walls of each orbit are osteotomized to free them from the frontal, zygomatic, maxillary, nasal and sphenoid bones in surgical correction of hypertelorism. RESULTS: Amblyopia and strabismus appeared in 58.3% of patients each of them, being exotropias the most common. The most frequent refractive error was hypermetropia (40%). Other ocular findings were: ptosis, lacrimal system impermeability, heterochromic iris, optic disk hypoplasia and enophthalmos. None of the deffects was modified after surgical correction of hypertelorism. CONCLUSIONS: Ophthalmologic findings associated with hypertelorism are not caused by the deformity. They are more frequent as higher the severity of the hypertelorism is. The ocular findings do not modify after surgical treatment of hypertelorism, except if we treat them specifically.
Adolescent↗
Hypertelorism in neurofibromatosis.
In eight out of thirty-four patients with neurofibromatosis hypertelorism was seen. This hypertelorism was diagnosed by measuring the intercanthal distance and calculating the interpupillary distance from it. The high incidence of hypertelorism in our group of patients (24%) makes its direct association with neurofibromatosis feasible. Moreover, hypertelorism was found exclusively in neurofibromatosis patients with brain involvement (8 out 11) and therefore seems to herald a severe expression of Morbus Recklinghausen. The bones of the face and the base of the skull are mesenchymal structures of neural crest origin and skull dysplasias - e.g. hypertelorism - fit well into the neurocristopathy concept of neurofibromatosis. Its ease of clinical recognition and its presence at birth makes the hypertelorism an early diagnostic criterium.
Adolescent↗
Interpupillary index: a new parameter for hypo-hypertelorism.
AIM: To establish a new clinical index to evaluate the presence of hypo-hypertelorism with greater accuracy. MATERIAL AND METHODS: After screening a wide range of population, 310 elementary school children (185 boys, 125 girls) aged 7-15 years were included in this study. For this cross-sectional study, a millimetre ruler was used. The anatomical interpupillary distance was measured by a modified Viktorin's method. In addition, inner and outer intercanthal distances were obtained. The data were analyzed by Student's t-test for two independent samples using SPSS for Windows. There were children with clinical hypertelorism (n = 92, group 1), children with large fronto-occipital circumference (FOC) (n = 101, group 2), and age- and sex-matched normal controls (n = 117, group 3). Due to variations in FOC among healthy subjects, we introduced a new practical concept for evaluation of interpupillary distance, namely the interpupillary index, the simple product obtained by dividing the interpupillary distance by the FOC, multiplied by 100. RESULTS: The overall idiopathic benign macrocephalic children (group 2) had significantly (p < 0.001) larger interpupillary distances (6.13 +/- 0.36 cm) and FOCs (56.99 +/- 1.46 cm) than those of normal controls (5.70 +/- 0.26cm and 52.82 +/- 1.22 cm, respectively). But, the difference between the combined product of interpupillary distance and FOC, the interpupillary index, was not significant (10.76 +/- 0.50 and 10.79 +/- 0.35, respectively) (p > 0.05). On the other hand, the children with hypertelorism had significantly (p < 0.001) larger interpupillary distances (6.47 +/- 0.29cm) and FOCs (54.90 +/- 2.18cm) when compared with the controls. In addition, the interpupillary index was significantly (p < 0.001) higher (11.80 +/- 0.45) than both macrocephalic children (10.76 +/- 0.50) and controls (10.79 +/- 0.35). Intercanthal distances and intercanthal index of hyperteloric children were also significantly (p<0.001) larger than both macrocephalic children and controls. CONCLUSION: This new index offers a new concept for more accurate evaluation of the presence of ocular hypo-hypertelorism.
Adolescent↗
Hypertelorism: interorbital growth, measurements, and pathogenetic considerations.
Normal pre- and post-natal changes in the interorbital distance are described. Causes of illusory hypertelorism include flat nasal bridge, epicanthic folds, exotropia, widely-spaced eyebrows, narrow palpebral fissures, and dystopia canthorum. Measurements of hypertelorism may involve soft tissues or bone, and a number of indices have also been proposed. Various types of measurements are evaluated and recommendations suggested. Possible pathogenetic mechanisms for hypertelorism include: early ossification of the lesser wings of the sphenoid; failure in nasal capsule development allowing the primitive brain vesicle to protrude into the space normally occupied by the capsule resulting in morphokinetic arrest in the position of the eyes; and disturbances of the cranial base in Apert syndrome. Associations with increased interorbital distance are also discussed: orofacial clefting, nonprotruding lipomas of the corpus callosum, calcification of the falx cerebri, duplication of the crista galli, wrinkling of the nose, and tissue tags of the nose. Finally, experimental models of hypertelorism in animals are discussed.
Abnormalities, Multiple↗
Neurofibromatosis and hypertelorism.
Hypertelorism was observed in eight of 34 patients with neurofibromatosis. This diagnosis was made by measuring the intercanthal distance and calculating the interpupillary distance from it. The bones of the base of the skull and of the face are mesenchymal structures of neural crest origin. Skull dysplasias, in which hypertelorism can be included, fit well into the neurocristopathy concept of neurofibromatosis. Hypertelorism seems to herald a severe expression of neurofibromatosis, eg, with brain involvement, and would therefore be an indication for doing a computed tomographic scan. The high prevalence of hypertelorism in our group of patients (24%) makes its direct association with neurofibromatosis highly feasible. Its ease of clinical recognition and its presence at birth would make it a valuable early diagnostic criterion.
Adolescent↗
Teebi hypertelorism syndrome: report of a family with previously unrecognized findings.
We present a family consisting of a mother, a daughter, and a son with Teebi hypertelorism syndrome, including some previously unrecognized manifestations. The clinical findings include a prominent forehead, arched eyebrows, pronounced hypertelorism, long philtrum, mild interdigital webbing, fifth-finger clinodactyly, umbilical anomalies, and hypotonia. The mother and daughter also had ptosis requiring surgical correction. The daughter has bilateral iridochorioretinal colobomas with high hyperopia and a small umbilical hernia. The son has less striking facial features but was born with a small omphalocele, large ASD secundum, PDA, bilateral cryptorchidism right hydronephrosis, and a cystic left kidney. The mother had an umbilical hernia requiring surgical correction as a child and a history of heart murmur. Both children have normal hearing and mild developmental delay. Their high-resolution karyotypes were normal and the FISH for 22q11 microdeletion was negative in the daughter. We conclude that cardiac defects in Teebi hypertelorism syndrome are not rare findings and that eye colobomas and renal anomalies were previously unrecognized.
Abnormalities, Multiple↗
Autosomal recessive inheritance of a syndrome of hypertelorism, hypospadias, and tetralogy of Fallot?
We report on 3 brothers with hypertelorism, hypospadias, and tetralogy of Fallot. Parents are first cousins once removed; the father has apparent hypertelorism. An apparently normal paternal uncle who is married to a second cousin also has a daughter with hypertelorism and tetralogy of Fallot. All similarly affected relatives have mild or borderline mental retardation. The combination of anomalies may represent a previously undescribed autosomal recessive disorder.
Bone Diseases, Developmental↗
Prenatal diagnosis of Opitz (BBB) syndrome in the second trimester by ultrasound detection of hypospadias and hypertelorism.
Prenatal diagnosis in a kindred with the Opitz (BBB) syndrome is presented. The inheritance is consistent with either autosomal dominant inheritance with sex limited expression or X-linked inheritance. The abnormalities in the kindred consist of hypertelorism, hypospadias, ambiguous genitalia, urocolic fistula, imperforate anus, mental retardation, diaphragmatic hernia, and malrotation with volvulus. A male fetus at 19 weeks was found by ultrasound to have hypertelorism and hypospadias with a small phallus consistent with the syndrome. The diagnosis was confirmed by pathologic examination after pregnancy termination. This is the first report of prenatal diagnosis of Opitz syndrome by ultrasonographic demonstration of hypertelorism and hypospadias in the second trimester.
Adult↗
Orbital measurements in 63 hyperteloric patients. Differences between the anthropometric and cephalometric findings.
Anthropometric and cephalometric orbital measurements were compared in 63 North American Caucasian patients (24 males, 39 females) aged 3 to 29 years who had 13 craniofacial syndromes involving hypertelorism. The hypertelorism, which was diagnosed anthropometrically, was mild in 11 patients, moderate in 25 and severe in 27. The surface intercanthal width was larger than the bony interorbital distance in all patients (mean 12.2 mm). The differences were smaller in mild cases and larger in severe cases. The soft-tissue binocular width was shorter than the bony lateral orbital distance in 53 patients, by a mean of 4.4 mm; in the other 10 patients the two measurements were identical. The hypertelorism was confirmed by cephalometry in 8 of the 11 cases identified as mild by anthropometry (72.7%), 24 of the 25 moderate cases (96.0%) and 25 of the 27 severe cases (92.6%). The abnormally wide surface intercanthal distance was matched by an abnormally wide bony interorbital distance in 57 of the 63 patients (90.5%). In the other six patients (3 with the mild form, 1 with the moderate form and 2 with the severe form) the bony widths were near the upper limit of the normal range (mean + 2 standard deviations).
Adolescent↗
An objective assessment of treatment for orbital hypertelorism.
A retrospective statistical analysis of orbital hypertelorism correction was performed by comparing the preoperative and postoperative intercanthal distances of these patients with published age-matched normal values. Forty-five patients who had undergone surgery over a 15-year period with an average follow-up of 5 years (6 months to 14 years) were evaluated. Comparison of age-normalized preoperative and postoperative intercanthal distances revealed a significant difference for orbital hypertelorism patients as a group (p < 0.0001), patients with clefts (p < 0.0001), patients with nasoencephalocele (p < 0.01), and patients with frontonasal dysplasia (p < 0.05), but not for those patients with craniofacial dysostosis (p < 0.20). Multiple analyses of variance revealed a significant interaction existing between the extent of preoperative deformity and the cause of hypertelorism for both the postoperative deformity and the total amount of correction achieved, but not for the type of surgery or for the age at which the surgery was performed.
Analysis of Variance↗
The hypertelorism-hypospadias syndrome.
Recently, an association between telecanthus and/or hypertelorism and hypospadias has been reported in several families. We describe six more families in whom we have found this association. Seven of the eight affected individuals were males. The other was a girl with hypertelorism and a minor urethral abnormality. The mothers in all six families had hypertelorism and/or telecanthus. Mental retardation and cleft palate were also common in our families and those reported previously. The radiologic findings in our first family, which was reported elsewhere as the "branchio-skeletal-genital syndrome," are considered in detail and include skull abnormalities, maxillary hypoplasia, dentigerous cysts and vertebral abnormalities.
Adolescent↗
[Surgical correction of 34 patients with hypertelorism].
In 34 patients with hypertelorism aged on the average 12.6 years, 24 underwent intracranial surgery (combined intra- and extracranial approach), 2 (moderate) U-osteotomy (subcranial approach), and 8 (mild) canthoplasties. Improved results were obtained in the patients with various types of hypertelorism. The complications were reviewed. Of the 24 patients undergone intracranial surgery, one died, 4 had cerebrospinal fluid leakage, and 4 had keratitis. No seizure, cerebral edema, meningitis, blindness, and ptosis were observed in these patients. Average blood loss was 72% of blood volume. Average duration of surgery was 7.5 hours. The causes of hypertelorism such as craniofacial cleft, craniosynostosis, frontoethmoidal meningoencephalocele, frontonasal fibrous dysplasia, and trauma were also discussed. Satisfactory appearance was seen in most of the patients.
Adolescent↗
Awan's syndrome (primary orbital hypertelorism, narrow-angle glaucoma and lean physique) in two women.
Awan's syndrome, characterized by a lean physique, orbital hypertelorism and angle-closure glaucoma, is a distinct clinical entity which should be more widely recognized. The patients, usually women in their sixth decade, may suffer several self-limiting and misdiagnosed episodes of angle-closure before a definite diagnosis is made. The general physical appearance and hypertelorism in two women, one aged 56 and the other 59 years, with Awan's syndrome aroused the suspicion of angle-closure glaucoma. The timely confirmation of diagnosis and proper management prevented further visual loss in both patients. Some patients with Awan's syndrome may show advanced damage to the optic nerve without ever having had an acute attack of glaucoma or any other symptoms, which makes it important that all patients with orbital hypertelorism and suspected glaucomatous optic discs should undergo gonioscopic evaluation to rule out glaucoma.
Body Constitution↗
[Hypertelorism-hypospadias (BBB) syndrome. 2 additional family studies].
We report on two unrelated families with the hypertelorism-hypospadias (BBB-) syndrome. The male index patients, 3 and 10 months old, respectively, have ocular hypertelorism, cleft lip and palate, high and broad nasal bridge and hypospadias. The patients' mothers, maternal grandmothers and one patient's sister show hypertelorism. In addition, we summarize the characteristics of previously published cases.
Bone Diseases, Developmental↗
Variable expressivity of hypertelorism in three siblings with Greig syndrome.
Two sisters and one brother are reported with a complex of congenital malformations, hypertelorism, mental retardation, flattened nasal root, divergent strabism++, mongoloid palpebral fissures, malformations of the ears, pathologic alterations of the eye-fundus in terms of optic nerve atrophy, all suggesting Greig syndrome. The major symptom of this syndrome, the hypertelorism, varied considerably in its expressivity in the three siblings. This fact is normally taken into consideration in the diagnosis of Greig syndrome, but we suggest that an alteration in skull formation should be the criterion for the syndrome rather than extreme hypertelorism.
Abnormalities, Multiple↗
Comments on frontonasal dysplasia, ocular hypertelorism and dystopia canthorum.
The mean canthal index values of patients with frontonasal dysplasia are tested for differences in facies A, B, C, and D. A general discussion of ocular hypertelorism and dystopia canthorum is presented. Ocular hypertelorism is considered a sign which may occur in a variety of disorders. Quantitative methods for determining ocular hypertelorism are critically reviewed.
Abnormalities, Multiple↗