Nasal tumors
Prem Puri in Newborn Surgery, 2017
The treatment of choice is excision of the tumor with the herniated brain and closure of the dural defect. Usually, there are no problems for closure of the wound, the covering skin being normal. According to Macfarlane et al., primary and secondary hypertelorism regressed in most instances where patients were treated before the age of 2 years.26 In their series of 114 patients treated in a 15-year period, in 59% of the children, the developmental outcome was normal, 18% have mild mental or physical disability, and in 23%, severe impairment occurred.
Hypertelorism
Amar Bhide, Asma Khalil, Aris T Papageorghiou, Susana Pereira, Shanthi Sairam, Basky Thilaganathan in Problem-Based Obstetric Ultrasound, 2019
Hypertelorism refers to an interocular distance above the 95th percentile. Causes of hypertelorism include mechanical causes such as premature fusion of the skull bones or a cleft. Genetic syndromes such as craniofrontonasal dysplasia, Apert syndrome, and Crouzon syndrome can have hypertelorism as part of their features. Mild hypertelorism can also be present as an isolated finding with no clinical consequences.
Growth of the Orbit
D. Dixon Andrew, A.N. Hoyte David, Ronning Olli in Fundamentals of Craniofacial Growth, 2017
Frontonasal dysplasia need not always produce hypotelorism. Sometimes the aberration may result in deficient “frontation” of the developing eyes, with hypertelorism the result — another wide spectrum of disorders (Gorlin, in Bergsma, 1969; Cohen et al., in Bergsma, 1971; Ortiz Monasterio et al., 1990). Hypertelorism may sometimes be a part of facial or cranial clefting (Tessier, 1972).
Bone fusion in transcele reconstruction of frontoethmoidal meningoencephalocele
Published in British Journal of Neurosurgery, 2022
Samsul Ashari, Mohammad Saekhu, Kevin Gunawan, Amanda Aldilla, Nadya Zaragita, Setyo Widi Nugroho
Vertical midline incision on the skin was made on the top of the cele. Skin incisions may be linearly shaped in the midline or ‘Y’-shaped upside down above the cele through the cutaneous, subcutaneous and galea until approaching the cele. The incision wound can be extended to the top of the eyebrow. Subgaleal layer was undermined until the cele wall is found, followed by periosteum dissection around the cele towards the cranial to the identified bone defect, making a periosteal flap with pedicle on the cranial side. Dura mater is freed from the edge of the bone using Adson and dural taster. Small craniotomy is performed at the superior limit of bone defect to the frontal region, giving larger exposure to identify normal dura and cele boundary. Then, the cele is extirpated at the neck. In this approach, all the cele wall was discarded, and all the gliosis tissue was coagulated or resected. Dura was closed primarily and tightly. It is imperative not to close the dura using the remaining cele wall. The bone that has been removed during craniotomy was cut into several pieces to close the defects, then the bone was fixated in place with non-absorbable monofilament (prolene). Rotational flap was done to cover the bone using periosteum. If needed, hypertelorism could be corrected using this approach in one stage. The fascia is stitched tightly and the excess skin part is reconstructed (Figures 1–3).
Nevoid basal cell carcinoma syndrome: a case report and literature review
Published in Ophthalmic Genetics, 2022
Shripadh Chitta, Jineet Patel, Shravan Renapurkar, Christopher Loschiavo, Jennifer Rhodes, Kayla King, Kimberly Salkey, Natario Couser
Ocular hypertelorism refers to an abnormally increased distance between the eyes, including both the distance between the inner eye corners as well as the distance between pupils. It is a common clinical manifestation of NBCCS and reported in up to 80% of the patients in some studies (4,7). Therefore, it is a minor criterion in the commonly used Kimonis criteria (2). Though there is some evidence that suggests that the prevalence of hypertelorism in these patients has some regional variability (20). An examination of various case reports from East Asian countries found 56% of a combined 227 patients to have hypertelorism while various case reports from North European countries found 26% of 239 patients to have hypertelorism (20). A recent examination of 11 Italian patients found 45.5% to have hypertelorism (21).
An unusual ophthalmic presentation of Wolf-Hirschhorn syndrome
Published in Ophthalmic Genetics, 2021
Gökhan Çelik, Bilge Batu Oto, Osman Kızılay, Oğuzhan Kılıçarslan, Handan Hakyemez Toptan
The infant had multiple congenital anomalies; a cleft palate, microcephalia, micrognathia, renal pelvicalyceal ectasia, atrial septal defect, transvers arcus hypoplasia, patent ductus arteriosus, hypospadias, undescended testicle. The patient was consulted to ophthalmology clinic 4 weeks after birth, for having iris coloboma and screening for retinopathy of prematurity (ROP), hence he received intense oxygen therapy due to bronchopulmonary dysplasia. External ophthalmic examination revealed hypertelorism and exotropia. The right eye was normal in biomicroscopic examination but in the left eye an inferiorly localized incomplete iris coloboma was present. Fundus examination revealed optic disc coloboma of both eyes. No fundus images were taken in the first examination, because the patient was diagnosed with basic optic coloboma.
Related Knowledge Centers
- Apert Syndrome
- Autism Spectrum
- Digeorge Syndrome
- Noonan Syndrome
- Telecanthus
- Trisomy 18
- 1Q21.1 Duplication Syndrome
- Nevoid Basal-Cell Carcinoma Syndrome
- Digeorge Syndrome
- Loeys–Dietz Syndrome
- Craniofrontonasal Dysplasia