Craniofacial growth in Turner syndrome patients

Abstract

The morphology of craniofacial complex in Turner syndrome patients has been studied thoroughly, while data on growth pattern and rate are still scarce. Since growth hormone therapy is implemented in treatment of these patients, there was a need for exploring the craniofacial morphology and growth of Turner syndrome patients treated with growth hormone. The main aim of this doctoral dissertation was to examine the morphology and craniofacial complex growth of Turner syndrome patients treated with growth hormone and to compare them with healthy controls. Twenty one lateral cephalometric radiographs of Turner syndrome girls were compared to 46 radiographs of healthy controls, in order to establish the differences in craniofacial morphology. In order to examine the growth, the groups were divided according to cervical vertebral maturation stage into subgroups (“pre-growth” and “growth”). The craniofacial growth was assessed according to changes between subgroups and by superimposing 10 pairs of cephalograms of examined group. The craniofacial morphology in Turner syndrome patients treated with growth hormone was characterised by bimaxillar retrognathism, underdeveloped mandible, overdeveloped mandibular ramus and longer facial heights, when compared to healthy controls. The craniofacial growth did not differ between examined groups during pregrowth period. On the contrary, during growth period altered growth rate and pattern of craniofacial complex in Turner syndrome group caused the differences in craniofacial morphology. It has been established that both upper and lower jaws grow in such direction that causes bimaxillar retrognathism and that there is a difference in growth rate of jaws and facial heights in girls with Turner syndrome. Other structures did not exhibit differences in growth rate and pattern. The craniofacial growth of Turner syndrome patients treated with growth hormone differs in growth rate and pattern, when compared with healthy controls, which causes distinctive craniofacial morphology.

Morfologija kraniofacijalnog kompleksa osoba sa Tarner sindromom je podrobno ispitana, dok su podaci o dinamici rasta oskudni u literaturi. Kako je hormon rasta sve više zastupljen u lečenju ovih pacijentkinja, javila se potreba za ispitivanjem morfologije i rasta kraniofacijalnog kompleksa osoba sa Tarner sindromom koje primaju hormon rasta. Ciljevi ove disertacije bili su da se ispita da li se i u kojoj meri morfologija i rast kraniofacijalnog kompleksa devojčica sa Tarner sindromom koje primaju hormon rasta razlikuje od zdravih devojčica. Razlike u morfologiji kraniofacijalnog kompleksa ustanovljene su na osnovu rendgen-kefalometrijske analize 21-og profilnog snimka glave devojčica sa Tarner sindromom koje primaju hormon rasta i 46 snimaka zdravih devojčica. Kako bi se ispitao rast kraniofacijalnog kompleksa svi snimci su, na osnovu stadijuma skeletne zrelosti vratnih pršljenova, podeljeni u dve podgrupe („pre intenzivnog rasta“ i „intenzivnog rasta“). Dinamika rasta ispitana je na osnovu promena između podgrupa kao i rezultata superponiranja 10 parova snimaka glave devojčica ispitivane grupe. Morfologija kraniofacijalnog kompleksa devojčica sa Tarner sindromom koje primaju hormon rasta se razlikovala od morfologije zdravih po bimaksilarnom retrognatizmu, nedovoljno razvijenom telu i prerazvijenoj grani donje vilice i većim visinama lica. Do perioda intenzivnog rasta nisu uočene razlike u rastu kraniofacijalnog kompleksa između devojčica sa Tarner sindromom koje primaju hormon rasta i zdravih devojčica. Tokom perioda intenzivnog rasta, razlike u dinamici i intenzitetu rasta dovele su do promena u morfologiji kraniofacijalnog kompleksa. Ustanovljeno je da je kod devojčica sa Tarner sindromom pravac rasta vilica takav da dovodi do bimaksilarnog retrognatizma i da postoji razlika u intenzitetu rasta vilica i visina lica. Nije ustanovljena razlika u dinamici i intenzitetu rasta ostalih struktura. Rast kraniofacijalnog kompleksa devojčica sa Tarner sindromom koje primaju hormon rasta se, po dinamici i intenzitetu, u određenoj meri razlikuje od rasta zdravih osoba što uzrokuje karakterističnu morfologiju kraniofacijalnog kompleksa.