Orbital Prosthetic Rehabilitation in “ADAM Complex” Multiple Orofacial-Cleft Disruption Syndrome

Abstract

To be human is great; to look human is wonderful! It is nature's greatest gift! Mother nature's womb is the safest place on earth for any life, but the calamity strikes and no one knows how Hence, Treasure your exceptions!, since nature seems nowhere accustomed more openly to display, its secret mysteries than in cases where it shows traces of its workings apart from the beaten path. A dismorphological pattern of congenital oro-craniofacial and limb defects which is a rare form of amniotic rupture sequence required persistent coordinated efforts of multiple disciplines and had manifested as bizarre orofacial clefting, cat eye syndrome with an ectopic eye, and aberrant tissue band lesions on limb. The challenge was to meet the child's clamour for functional demands on premature exposure to open world and was overcome through a phased treatment implementation. Anophthalmos resulting from multiple ophthalmic surgeries for aberrant ectopic left eye and cat eye syndrome of right eye required a staged sequential preemptive planning for a successful outcome. Every phase of fabrication of orbital prosthesis comes with an impending challenge. Thus, a well-defined technique eliminating the common errors and creating a natural looking prosthesis, in the face of limitations, is imperative.

Oral healthcare in Fraser syndrome

“…Oro‐facial features of Fraser syndromes are sparsely reported, but include facial asymmetry, oral clefting, high arched palate, ankyloglossia, fusion of primary teeth, and dental hypoplasia . Recognition of oro‐dental abnormalities, however, may help to confirm the diagnosis, and to differentiate Fraser syndrome from other genetic diseases associated with renal agenesis such as cat‐eye syndrome, and Melnick‐Fraser syndrome . In addition, patients with Fraser syndrome presenting for dental treatment might require special management considerations because of the associated defects.…”

All-Ceramic Systems In Dentistry

“…Presleme tekniğinin yanı sıra lösit ilave edilmiş cam seramikler, CAD-CAM yöntemi ile de üretilmektedir ve bu sistemler; inley, veneer, tek kron ve 3/4 kron yapı-mında kullanılabilirler (12,13). IPS Empress' e benzeyen bu sistemler daha küçük parçacık özelliğine sahip olup, partiküller daha üniform dağılmıştır (14).…”

“…1998 yılında üretilmiştir ve CEREC in LAB (Sirona) sistemi ile kullanılırlar. Bloklarına birçok renk seçeneği mevcut olup (13,15,16) 127 MPa bükülme dayanımına sahiptir (17).…”