ABSTRACT
A case of Stickler syndrome (hereditary arthro-ophthalmo-dystrophy) with maxillo-facial abnormalities and colon atresia is reported. The authors emphasize that in Stickler syndrome, (a chondrodystrophy with congenital alteration of type II collagen) the maxillofacial abnormalities are consecutive to prosencephalic neural crests dysneurulation, which caused mesethmoidal dysneurulation (and consequently of frontonasal-premaxillary structures); eye and colon abnormalities are consecutive to rhombo-mesencephalic neural crests dysneurulation. The Authors hypothesize that genic mutation, responsible of enzymatic deficiency of the neural crest multipotent cells, caused a morphogenetic alteration of the fronto-naso-philtral structures of the midface and of low face structures.
Subject(s)
Maxillofacial Abnormalities , Female , Humans , Infant , Maxillofacial Abnormalities/diagnostic imaging , Radiography , SyndromeABSTRACT
The authors examine the dental and skeletal alterations consequent to the use of radiotherapy as the elective method of treating retinoblastoma, an endobulbar neuroectodermal tumour typical of early infancy. The authors revise the basic concepts of radiobiology and the modifications induced by radiation in developing tissues with reference to both alterations at a microscopic (histological) level and those at a macroscopic level, namely of a morphological and structural nature. In the first part of the study the authors examine the damage to dental structures relating to both developing tissues and damage to mature tissue. The second part of the paper analyses radiation damage on cartilage and bone tissues in the process of formation and the authors present their documentation in the form of iconographic and radiographic material together with an esthetic face analysis performed using Rickettz's method in three cases referred to their attention.
Subject(s)
Bone and Bones/radiation effects , Eye Neoplasms/complications , Radiotherapy/adverse effects , Retinoblastoma/complications , Cartilage/radiation effects , Child , Esthetics , Eye Neoplasms/diagnosis , Eye Neoplasms/radiotherapy , Female , Humans , Male , Maxillofacial Development/radiation effects , Retinoblastoma/diagnosis , Retinoblastoma/radiotherapyABSTRACT
The authors examine the dental and skeletal alterations consequent to the use of radiotherapy as the elective method of treating retinoblastoma, an endobulbar neuroectodermal tumour typical of early infancy. The authors revise the basic concepts of radiobiology and the modifications induced by radiation in developing tissues with reference to both alterations at a microscopic (histological) level and those at a macroscopic level, namely of a morphological and structural nature. In the first part of the study the authors examine the damage to dental structures relating to both developing tissues and damage to mature tissue. The second part of the paper analyses radiation damage on cartilage and bone tissues in the process of formation and the authors present their documentation in the form of iconographic and radiographic material together with an esthetic face analysis performed using Ricketts' [correction of Rickettz's] method in three cases referred to their attention.