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PubMed2003

Teebi hypertelorism syndrome.

Teebi (1987) described an autosomal dominant syndrome with resemblance to craniofrontonasal dysplasia. Here we present a 2 year 5 month old girl with Teebi syndrome showing a prominent forehead, hypertelorism, mild exophthalmos, downslanting palpebral fissures, a depressed nasal bridge, a broad nasal tip, a long philtrum, natal teeth, a thin upper lip, an everted lower lip, a small chin, low-set ears, preauricular fistulas, a short neck, mild pectus excavatum, an umbilical hernia, clinodactyly of the 5th fingers with mild radial deviation of the distal phalanges of the middle fingers, mild pes adductus, an ectopic kidney, and normal psychomotor development. Her mother and her grandmother had similar features.

Abnormalities, Multiple↗
PubMed2004

Bilateral cleft lip and palate, hypertelorism and hypoplastic toes.

We report on a 23-month-old boy with bilateral cleft lip and palate, marked hypertelorism, frontal bossing and severe bilateral, asymmetric hypoplasia of toes. The mother used bisoprolol, naproxen and sumatriptan for migraine until the fifth postmenstrual week of pregnancy. We suggest that this patient's features represent a previously undescribed entity of as yet unknown aetiology.

Abnormalities, Multiple↗
PubMed2006

Central nervous system abnormalities on midline facial defects with hypertelorism detected by magnetic resonance image and computed tomography.

The aim of this study were to describe and to compare structural central nervous system (CNS) anomalies detected by magnetic resonance image (MRI) and computed tomography (CT) in individuals affected by midline facial defects with hypertelorism (MFDH) isolated or associated with multiple congenital anomalies (MCA). The investigation protocol included dysmorphological examination, skull and facial X-rays, brain CT and/or MRI. We studied 24 individuals, 12 of them had an isolated form (Group I) and the others, MCA with unknown etiology (Group II). There was no significative difference between Group I and II and the results are presented in set. In addition to the several CNS anomalies previously described, MRI (n=18) was useful for detection of neuronal migration errors. These data suggested that structural CNS anomalies and MFDH seem to have an intrinsic embryological relationship, which should be taken in account during the clinical follow-up.

Abnormalities, Multiple↗
PubMed2006

Midline facial defects with hypertelorism and low-grade astrocytoma: a previously undescribed association.

We report on a child with midline facial defects with hypertelorism (MFDH), median cleft lip, sphenoidal ventriculocele, partial agenesis of the corpus callosum, and low-grade astrocytoma in the cervicomedullary junction. This combination of findings has not been reported previously. Although this association might be casual, it demonstrates a relationship between disorders of frontonasal process and posterior fossae. It also suggests that individuals with MFDH might require a prospective follow-up with central nervous system magnetic resonance imaging.

Agenesis of Corpus Callosum↗
PubMed1999

[Syndromes 7. Hypertelorism (Greig syndrome)].

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.

Bone Transplantation↗
PubMed2005

[Microphthalmos and hypertelorism as diagnostic index in ultrasound diagnosis of Fraser syndrome].

Fraser syndrome is a rare genetic syndrome with abnormalities of the head, lungs, kidneys, and limbs. A prenatal diagnosis of FS can be done in families with risk, using foetal ultrasonography. However, a wide qualitative and quantitative variability of possible abnormalities makes the diagnosis in utero notably questionable. We present the results of foetal ultrasonography in a tertigravida, had delivered two children with FS. Signs of foetal hypertelorism and microphthalmia, both traits typical for FS, were detected based on outer and inner orbital diameters and ocular diameters in 28 and 32 weeks of pregnancy. The clinical and pathological examinations after birth confirmed the diagnosis of FS. Our observation suggests that eye anomalies may prompt the diagnosis of FS even if characteristic lung and kidney abnormalities are absent. Therefore, we propose to regularly assess eye dimensions and distance, when performing any foetal ultrasonography in families with of FS.

Abnormalities, Multiple↗
PubMed1988

Orbital reconstruction in hypertelorism.

Orbital hypertelorism as defined by increased bony interorbital distance is a complex manifestation of many congenital anomalies. As such, it is associated with a multitude of orbital, adnexal, and surrounding soft-tissue deformities. Treatment of these problems requires extensive experience and a comprehensive team, permitting adequate preoperative evaluation, diagnosis, and treatment. The results obtained may be dramatic. The overall safety of the procedures has been demonstrated. Continued refinements and long-term follow-up will undoubtedly continue to make significant improvements.

Bone Diseases, Developmental↗
PubMed1986

Facial bipartition in hypertelorism.

In our cases of hypertelorism we used two techniques. In some cases, we used Tessier's original technique (Tessier et al, 1967), and in others the modification suggested by Converse (Converse et al, 1970). In still others, we did not remove any part of the medial portion of the nose, that is, of the nasoethmoidal complex. We can say now, that in the cases operated on during ages of already completed development (14 to 15 years), the follow-up presented satisfactory, if not exceptional results, but in the cases operated on during the first years of life (2 to 10 years) investigation of the results obtained allowed us to make some important observations. In a high percentage of these cases we observed a relapse of the anatomical positions reached by operation. In the cases documented here, the long-term results were inadequate.

Bone Diseases, Developmental↗
PubMed1986

Maxillary growth following total septal resection in correction of orbital hypertelorism.

A rapid survey of eight cases with orbital hypertelorism did not reveal any evident change in maxillary displacement during facial growth following resection of nasal septum and medial displacement of orbital cavities. Accurate analysis of four cases according to the Ricketts long-term growth forecast showed a reduction of growth in the region of anterior nasal spine and only in the posteroanterior direction. This is not necessarily due to the absence of nasal septum. These patients have such severe deformities and the surgical procedure is so extensive that many factors can be responsible for deficient premaxillary growth. Further studies are needed, particularly in patients who, since 1975, have had the same operative method, but without septal resection, to know whether this more conservative procedure has changed the posteroanterior growth of the premaxilla. However, a nasal bone graft has a good prognosis, even when performed in children.

Bone Diseases, Developmental↗
PubMed1987

The Opitz hypertelorism-hypospadias syndrome. Further delineation of the spectrum of clinical findings.

In this paper we report three male patients with the Opitz hypertelorism-hypospadias syndrome. In addition to the typical morphological findings, signs of cerebral palsy related to dysmaturity and perinatal adaptation problems were present in two of them. This illustrates that this syndrome is a true multiple congenital anomaly/mental retardation MCA/MR syndrome with great variability in expression of clinical symptoms.

Adolescent↗
PubMed1982

Bony interorbital distance (BIOD), head size, and level of the cribriform plate relative to orbital height: II. Possible pathogenesis of orbital hypertelorism.

This investigation was designed to test hypothesized pathogenetic mechanisms for orbital hypertelorism (OrH). Serial measurements of bony interorbital distance (BIOD), level of cribriform plate relative to orbital height (LOCP), and cranial width (CW) were made from postero-anterior cephalometric radiographs on two pathologic groups craniofacial clefts (FC) and premature craniofacial synostosis syndromes (PCS) compared with normal controls matched for age and sex. Both groups of malformations showed significantly larger mean values (P less than 0.01) for BIOD alone and when adjusted for CW. The LOCP relative to the orbital height was found to be positioned at a significantly lower level (P less than 0.01) than the norm in both groups. When the growth increments for BIOD were plotted to analyze rates of growth, individuals with FC showed increments similar to those seen for the normal. However, patients with Apert and Crouzon syndromes showed greater than normal increments for BIOD. In these cases, the LOCP remained at a relatively lower level in relation to orbital height. These findings support the hypothesis of a time-specific event during fetal development causing morphokinetic arrest in FC syndromes. In Apert and Crouzon, another mechanism continuing postnatally produces OrH.

Adolescent↗
PubMed1980

[Correction of post-traumatic hypertelorism (author's transl)].

Following naso-orbital fractures, posttraumatic hypertelorism (or telecanthus) cannot always be avoided, even when prompt surgical treatment is given. For correction of this deformity, attempted remobilisation of the injured nasal skeleton (with or without grafting) is rarely successful. This is so because of two reasons: (1) the lateral nasal wall is thickened by overlapping fragments of fractured bone so that excessive scar tissue cannot be adequately replaced; (2) revision of paranasal sinus drainage is needed in most cases. Three cases are reviewed in order to demonstrate our concept of surgical repair: complete removal of bony fragments and scar tissue between lateral nasal mucosa and periorbita, restoration of the contour of the bony bridge of the nose by cartilage grafting and median canthoplasty, as well as possible pan-sinusectomy with or without dacryocystorhinostomy when needed.

Adolescent↗
PubMed1994

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.

Adult↗
PubMedDate not supplied

A new craniofacial disorder involving hypertelorism and malformations of external nose, palate and pituitary gland.

The aim of the present study was to describe and pathologically evaluate an apparently unreported craniofacial malformation, based on comparison of the cranial midsagittal components with similar components under normal developmental conditions. A severely malformed fetus with a gestational age of about 17 weeks underwent whole body and special craniofacial radiography. Following autopsy dissection, the midsagittal segment of the cranial base, including the eyes, was radiographed in different projections. Midsagittal tissue blocks were serially sectioned for microscopy. Routine stains and immunohistochemical stains were applied. The face was characterized by hypertelorism, absence of external nose but with open shell-like cavities medio-cranially to the eyes, and by a palate fused in the midline and with extensive bony ridges laterally. There was absence of normal nasal cavities, presence of nasal septum and vomer, normal eyes, and nasal ducts covered with nasal mucosa ending blindly in the cartilage. No olfactory bulbs were found. The palatal ridges consisted of bony tissue. The pituitary gland was severely malformed and consisted solely of adenopituitary gland tissue, located in its full extent in the pharyngeal mucosa. There was no sella turcica. From a pathogenetic point of view, it is suggested that the neural crest cells in the frontonasal region of the crest were reduced in amount or late in migration to the midfacial region compared to the neural crest cells to the maxillary region. Therefore, we believe that the malformations observed in the nasal placodes and in the pituitary placode, combined with abnormal migration or abnormal timing of neural crest cells during the craniofacial development, are important factors behind this disorder.

Brain↗
PubMed1997

Cleft lip and palate, hypertelorism, brachycephaly, flat facial profile, and congenital heart disease in three brothers.

A "new" syndrome was identified by McPherson and Clemens [1996: Am J Med Genet 62:58-60] in a brother and sister with bilateral cleft lip and palate, hypertelorism, flat facial profile, flat occiput, and complex heart defects. The brother also had a bilobed tongue and the sister had malrotation of the intestine and bifid thumbs. We describe three brothers with similar anomalies apart from the bilobed tongue, malrotation of the intestine, and bifid thumbs. McPherson and Clemens [1996: Am J Med Genet 62:58-60] suggested autosomal recessive inheritance. Our observation of three affected brothers also raises the possibility of X-linked recessive inheritance.

Adult↗