Hypertelorism

ABSTRACT

The term orbital hypertelorism (ORH) implies “widely apart orbits.” This may also be associated with the abnormal vertical orientation of the orbits (dystopia). This deformity may be unilateral or bilateral, symmetric or asymmetric and may be present in a variety of craniofacial conditions. The treatment is primarily carried out for aesthetic reasons. The timing of treatment is dictated by the underlying condition and the type of procedure envisaged. The mainstay of treatment consists of moving the orbits medially to near normal position. This is accomplished by either an orbital translocation or facial bipartition technique. The choice of procedure is governed by the shape of the maxillary arch and associated occlusal conditions. We must differentiate between the telecanthus (also called pseudo-hypertelorism) and a true ORH as the management differs in these two conditions. The ORH involves extensive intracranial and extracranial operation whereas the telecanthus correction is relatively simpler surgery. The article will discuss the aetiology, classification, presentation, treatment options, timing of surgery and the choice of surgical procedures. Illustrative case reports with long-term results will be used to explain the management of these patients.

KEY WORDS

Box osteotomy - cranial bone graft - facial bipartition - intra-cranial and extra-cranial procedure - lateralization of orbit - orbital socket - telecanthus or pseudo-hypertelorism - vertical dystopia

• REFERENCES

• 1 Available from: http://www.medical-dictionary.thefreedictionary.com/hypertelorism [Last accessed on 2014 Aug 30; at 6.30 pm]
• 2 Greig DM. Hypertelorism: A hitherto undifferentiated congenital craniofacial deformity. Edinb Med J 1924; 31: 560
• 3 Tessier P. Orbital hypertelorism. I. Successive surgical attempts. Material and methods. Causes and mechanisms. Scand J Plast Reconstr Surg 1972; 6: 135-55
• 4 Vermeij-Keers C, Mazzola RF, Van der Meulen JC, Strickler M. Cerebro-craniofacial and craniofacial malformations: An embryological analysis. Cleft Palate J 1983; 20: 128-45
• 5 Poleman RE, Vermeij Keers C. Cell degeneration in the mouse embryo: A prerequisite for normal development. In: Berat VM. editor Progress in Differentiation Research. Amsterdam: North Holland; 1976
• 6 Mann I. Developmental Abnormalities of the Eye. London: Cambridge University Press; 1970
• 7 Patten BM. Human Embryology. 3 rd ed.. New York: McGraw Hill; 1968
• 8 Tessier P, Guiot G, Rougerie J, Delbet JP, Pastoriza J. Cranio-naso-orbito-facial osteotomies. Hypertelorism. Ann Chir Plast 1967; 12: 103-18
• 9 Tessier P. Total facial osteotomy. Crouzon’s syndrome, Apert’s syndrome: Oxycephaly, scaphocephaly, turricephaly. Ann Chir Plast 1967; 12: 273-86
• 10 Tessier P. Chirurgie Orbito-Palpebrale. Paris: Masson; 1977
• 11 Tessier P. Experiences in the treatment of orbital hypertelorism. Plast Reconstr Surg 1974; 53: 1-18
• 12 Tessier P. Anatomical classification facial, cranio-facial and latero-facial clefts. J Maxillofac Surg 1976; 4: 69-92
• 13 Tessier PL. Facial bipartition. A concept more than a procedure. In: Marchac D. editor Proceedings of the First International Congress of the International Society of Cranio-Maxillo-Facial Surgery. Berlin: Springer-Verlag; 1987: p. 217-45
• 14 Converse JM, Ransohoff J, Mathews ES, Smith B, Molenaar A. Ocular hypertelorism and pseudohypertelorism. Advances in surgical treatment. Plast Reconstr Surg 1970; 45: 1-13
• 15 van der Meulen JC. Medial faciotomy. Br J Plast Surg 1979; 32: 339-42
• 16 van der Meulen JC, Vaandrager JM. Surgery related to the correction of hypertelorism. Plast Reconstr Surg 1983; 71: 6-19
• 17 Ortiz-Monasterio F, del Campo AF, Carrillo A. Advancement of the orbits and the midface in one piece, combined with frontal repositioning, for the correction of Crouzon’s deformities. Plast Reconstr Surg 1978; 61: 507-16
• 18 Marchac D, Renier D, Arnaud E. Infrafrontal correction of teleorbitism. In Whitaker L. editor Proceedings from the Seventh Meeting of the ISCFS. Santa Fe: Menduzzi; 1997: p. 173-5
• 19 Sharma RK, Makkar SS, Nanda V. Simple innovation for medial canthal tendon fixation. Plast Reconstr Surg 2005; 116: 2046-8
• 20 Jackson IT, Adham MN, Marsh WR. Use of the galeal frontalis myofascial flap in craniofacial surgery. Plast Reconstr Surg 1986; 77: 905-10
• 21 Mahatumarat C, Rojvachiranonda N, Taecholarn C. Frontoethmoidal encephalomeningocele: Surgical correction by the Chula technique. Plast Reconstr Surg 2003; 111: 556-65