Vývoj a růst čelistí. Změny během života. Dysplasie tváře, rozštěpy patra. Zesílená a zeslabená místa obličejového skeletu; lomné linie.

Ivo Klepáček

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Utváření dolní čelisti

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T.F. – fetalweekR - year

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Růstové změny během života after Enlow 1966

II

Expanse střední a přední lebeční jámy

V závislosti na tom se očnice stáčejí mediálně a ventrálně

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Expansion and growth of the synchondroses and sutures support middle face segments and base parts to year 7.

Postnatal growth

Dislocation down and ventrallySlow to year 15-18.

Dislocation up and ventrallyFast to year 12.

Synchondrosis sphenooccipitalis 17.yearSynchondrosis sphenoethmoidalis after deliveryIntraoccipital synchondroses year 5Sphenoid synchondroses delivery time

after Schumacher 1992

Dislocation ventrallyto year 7.

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FrontalisEthmoidalis

anterior

MaxillarisAntrum

Highmori

8 yr

20 yr

12 yr

1 yr

60 yr

4 yr

"Ostiomeatal unit – functional anddevelopmentalconnection of

sinusesostiomeatal unit"

N. Highmore:Corporis humani disquisitio anatomica; in qua sanguinis circulationem in quavis corporis particula plurimis typis novis ac aenigmatum medicorum succincta dilucidatione ornatam prosecuutus est.Hagai-Comitis [The Hague], 1651.

The sinus was well known to anatomists before Highmore. It had been illustrated by Leonardo da Vinci (1452-1519) and had been noticed by Giulio Casserio (1561-1616)

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Epithelium: keratinizing, continueson inner ret surface as a

multilayered slowly keratinizingepithelium

lamina propria mucosae continuesas submucous layer containingsmall serous or mucous glands

(in soft palate)

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Palate formationand clefts

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Primary palateo (intermaxillary segment) and finalsecondary palate

Palate formation and clefts

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6.5 week

Palatal shelves are vertically oriented firstPalatal shelves are lifting upPalatal shelves (processes) are mutuallyconnected and fuse with primary palatecle

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Palatal processes are connected togetherabout one week earlierin women in comparison with men

Week 10

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No ability to pucker lipsArticulate, pronounce

Normallu eat and drink

Separation from group of otherchildren

retardation

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Rozštep rtu: - nemožnost uzavřít ústní štěrbinu

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Cantrell pentalogy

Cantrell 1958

five characteristic findings:Omphalocele

Anterior diaphragmatic herniaSternal cleft

Ectopia cordisIntracardiac defect: either a ventricular septal defect or a diverticulum of the left

ventricle

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http://en.wikipedia.org/wiki/Omphalocele�http://en.wikipedia.org/wiki/Diaphragmatic_hernia�http://en.wikipedia.org/wiki/Sternum�http://en.wikipedia.org/wiki/Ectopia_cordis�http://en.wikipedia.org/wiki/Ventricular_septal_defect�http://en.wikipedia.org/wiki/Diverticulum�http://en.wikipedia.org/wiki/Left_ventricle�http://en.wikipedia.org/wiki/Left_ventricle�

In middle Europe – patients with cleft they haveanamnesis of this malformation in family onluy in 20% .Risk for offspings of these patients varies between 4 -

15%.

Very rare are patients with multimalformations – with geneticsyndromes.

Risk in these cases is very high.

Well.known is van der Woude syndrome, where palate cleft appearsrepeatedly (about 80%) , together with fistulas on lower lip.

Genetically ky precedented dangerousness ?cle fts

Collinsův syndromMandibulofacialdysostosis (face

hypoplasia)Hypodiferenciation of zygomatic bones,

mandible, eyes oriented down, coloboma of lower eylid, external ear

defects

Autosomal, can bestarted by influence

of retinoid acid

RARE

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DiGeorge syndrome

(third and fourth arch syndrome, Velocardiofacial

syndrome VCFS + concotruncal anomalies face

syndrome CATCH22)Cardiovascular defects

Abnormal faciesThymic hypoplasia

Cleft palate, microstomiaHypocalcemia, Hypertelorism22 chromosome is damaged

Can be initiated by retinoids

1/2500

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Robin syndrome

micrognathiaUnderdevelopment of the 1st arch structures, mandibular hypoplasia,

Micrognathia, cleft palate, glossoptosis (posteriorly placed tongue)

Alteration of the 1st arch, can be caused by oligohydramnion

1/8500

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Van der Woudesyndrom

Hypodiferentiation of skull bones, cleft lip, palate, fistulae in lower lip, hypodontia

About 80 percent probability ofother incidence in the same family

autosomalActivated by regulatory

factor 6 (IRF6)

RARE

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CLEFTSNon fusion of face processes

through developmentCongenital and teratogenic influences(time of critical period and period of

drug influence)

Three critical periods:25.- 35. day isolated lip cleft37.- 53. day isolated palate cleft(shelves damaged)53.-57. day isolated palate cleft(growth of mandible is retarded)

vícefaktorovýProtikřečové látky(phenobarbital, diphenylhydantoin)CytostatikaImunosupresiva, Tetracyklin,Záření

! Preventivně léčit rozštěpy do druhého měsíce těhotenství !

Včas prenatálně diagnostikovat po třetí kritické periodě a zvážit

přerušení těhotenství

20 % – hereditary10 % – outer environment influence

(mother, radiation….)70 % - unknown

DiabetesHypoglykemické stavyEpilepsieStreskortikoidy

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Neinvasive ultrasonography helpsto make diagnosis of lip cleft in

utero. Isolated cleft palate can not be seen.

Third trimester

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Incidence of face clefts during last 30 years

Doc. Peterka: Number affected children varies depending on natality.

From year1965 to1975 was number of defected children increased twotime (120 to 230).

From year1976 up to date number of children with clefts was decreasing.

Perhaps, it depends on lower and lower number of childbirths.

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Year incidence of newborns with facialclefts is relatively stabvle and varies

about dlong aritmetic mean 1.7 per 1000 childbirths.

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Following cleftposition:

First group: lip clefts (isolated orcombined with palate cleft)

Second group: isolated palateclefts.

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cheiloschisis

1/100080% males

Unilateral cleft lip

Incidence increases with maternal age

4-7-17% cle fts

cheilognathouranostaphyloschisis

Cleft lip, jaw and both the palati

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Cheilognatho-uranoschisis unilateralis

Unilateral lip, jaw andpalate cleft

1/2500

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Cheilognatho-uranoschisis bilateralis

Bilateral cleft lip, jaw andpalate

1. Columella – skin segment betweennostrils.- shortened. 2. Prolabium – connects columella andphiltrum – swollen.3. Premaxilla – narrow and small; separated from maxilla.

1/2500

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Palatoschisis(uranoschisis)

Isolate cleft palate

Incidence increases with maternal age

2-7-15%

1/250067% females

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Cleftoverviewcle

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operation

1) Closure of cleft lip – as fast as possible, (suckling baby age), All lip structurs can be separated and connected together by sutures.

2) Closure of cleft palate – indicated for age 1 – 3 year, Mucous and muscular flaps of soft palate are pushed together

3) Osteoplastic procedure on defected alveolus – indicated in age 8 – 11 year.

Osseous autotransplat is applied into alveolus and oronasal opening isclosed by soft tissues from surrounding areas (usually to the end ofpubertal period.

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Před a po operaci

Before and after surgical treatment

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Postoperative Torus palatinus

(Palatal swelling)

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Cleft palate before surgery, age 0 months 15 days.

Cleft palate after surgery, age 2 years 12 days.

Cleft palate after surgery, age 5 months 21 days.

Cleft palate after surgery, age 2 years, 10 months28 days. Healing is complete.

Cleft lip before surgery, age 2 months 8 days.

Cleft palate before surgery, age 2 months 8

days.

Cleft lip after surgery, age 5 months 22 days.

Cleft palate after surgery, age 5 months 22 days.

Recidiva

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Hypofunkceměkkého patraHypofunctionof soft palate

Zvedání patra selhává

Failure of soft palatelifting

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Chirurgical and dental care isprolongated from birth to year 18.

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6. a 7. týden

Philtrum, sulcus nasolacrimalis,saccus lacrimalis, faciei, maxillae, nasus externus cle

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Nasolabial groove

Oblique face cleft

Nasolabial groove is deep and nasolacrimal duct isfree

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Fissura labii mediana

Obvykle je doprovázena holoprosencefalií

holoprosencephalia

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holoprosencephaliaHypodiferenciace čelní části hlavy včetně mozkucle

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Face clefts Rare. Probably hereditary influence ?. Unknown etiology. Characteristic following Tessier :

1. Cleft relates to disturbancies of soft even hard facetissues.

2. Cleft appears only in some regions.3. Fully developed cleft can be followed by anomalies of

skull basis. One of affected aread is orbit. Frequency of orbit clefts decreases in direction

counter clockwise.

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Masticatory pressure buttresses (Traiectoriae maxillae on boththe sides - canine, zygomatic, pterygoid. Other, dorsal buttress – grows from the mastoid process and from the area offoramen magnum (foramen occipitale magnum).

Frame-like construction of skullButtressesin facialskeleton

Three buttresses allow face to absorb force Nasomaxillary

(medial) buttress Zymaticomaxillary

(lateral) buttress Pterygomaxillary

(posterior) buttress

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Strips ofcompact

bone tissuesin the human

skullfollowingDeffez 1966

Patrová deska

Palatal plate (desk)

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Transferenceof load in

facialskeleton

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Trajectoriesin the humanskull base;

Interior view

followingDeffez 1966

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Pressureand tensiontrajectories

in mandible

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Transfer of pressure

and loadin

mandible

after Lang 1995

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Trabeculaeseen in

mandible

after Lang 1995

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Fractures after Le Fort (René Le Fort 1902)

What´s broken:

• Medial wall of orbit

• Lateral wall of orbit to frontozygomatic suture

• Pterygoid process

• Basal part of nasal septum

• zygomatic arch

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Le Fort IGuérin´s fracture

Subzygomatic

Le Fort I fractures: (horizontal) A fracture of the maxilla immediately above the teeth and palate.

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http://en.wikipedia.org/wiki/Teeth�http://en.wikipedia.org/wiki/Palate�

Le Fort IIPyramidal,

central, upper subzygomatic

Le Fort II fractures: (pyramidal) The result of a blow to the lower or mid maxilla.

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Diagnosis Lefort II and III

Bilateral periorbital edema & ecchymosis

Step deformity palpated infraorbital & nasofrontal area

CSF rhinorrhea Epistaxis

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Le Fort IIISuprazygomatic

fracture

Le Fort III fractures: (transverse) Also called craniofacial separation, the result of impact to the nasal bridge or upper maxilla.

Nasal root, medialorbital wall. Inf. orbital notchLat. orbital wallinf. Orbital notchBasis of pterygoidprocessZygomatic arch

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http://en.wikipedia.org/wiki/Craniofacial�

Trakční (tahové) a tlakové linie

CondyleUpper

Lower neck

Retromolar (angular)

Through canine,

through mental region

Lomné linie dolní čelisticle

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Fossa infratemporalisInfratemporal fossaSpatium pterygomandibulare

mm. pterygoideiFossa infratemporalis ossea

Fossa pterygopalatinacle fts

Sup.:Ala major ossis

sphenoidalisMed.:

Lamina medialisprocessus pterygoideus +

pharynxVentr.:

Tuber maxillaeLat.:

Ramus mandibulaeDors.:

Septum styloideum

Stěny infratemporální jámyWalls of the infratemporal

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SpatiumparapharyngealeParapharyngeal space

• Deep cevical space

• Looks like pyramid on top; basis – skullbase, top – hyoid bone

• Parts: prestyloid andretrostyloid spaces

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Styloidní septum

Styloid septum

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Superficial parts:Pterygomandibular spacepterygoidei mm. and sp. between them

Vrstvy layers

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Bichatův polštář kříží ductus parotideusBichat´s fat pad is crossed by parotid duct

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Fossa infratemporalis„hluboká vrstva“

Infratemporal fossa“deep layer“

Větve V3Mandibular

branches

Hluboké části:Fossa infratemporalis osseaFossa pterygopalatina cle

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Fossa infratemporalis„Povrchová vrstva“

Infratemporal fossa“superficial layer“

Tepny a žilní pleteněArteries and plexiform-like veins

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Plexus pterygoideusPterygoid plexus

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Pterygoid venous plexus

and its tributaries:n superior ophtalmic p inferior ophtalmic

n infraorbital vein to pterygoid plexus

(through foramen ovale –rete)

r deep facialu buccal

inferior alveolar vein

... retromandibular vein h maxillary veins

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Arteria maxillaris –větve pars retromandibularis

• a. auricularis profunda• a. tympanica anterior• a. meningea media

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Arteria maxillaris – větve pars pterygoideacle

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Arteria maxillaris – větve odstupující

z pars pterygopalatina

• a. alveolaris superior posterior• a. infraorbitalis

• a. palatina descendens: a. palatina major et minores

• a. canalis pterygoidei• a. sphenopalatina:

a. nasales posteriores laterales et nasales posteriores septalescle

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Fossa pterygopalatinaPterygopalatine fossa

(sphenopalatine)

Pterygoid canal

Greater palatine canal

Sphenopalatine foramen

Inferior orbital fissure

Round foramencle fts

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Fossa pterygopalatina –preparace z dutiny nosní

Fossa pterygopalatina dissected from the nasal

cavity

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Snímek číslo 1Utváření dolní čelistiSnímek číslo 3Snímek číslo 4Snímek číslo 5Snímek číslo 6Snímek číslo 7Palate formation and cleftsSnímek číslo 9Snímek číslo 10Snímek číslo 11Snímek číslo 12Snímek číslo 13Cantrell pentalogy�Cantrell 1958Snímek číslo 15Collinsův syndromDiGeorge syndrome�(third and fourth arch syndrome, Velocardiofacial syndrome VCFS + concotruncal anomalies face syndrome CATCH22)Robin syndromeVan der Woude syndrom�Snímek číslo 21Snímek číslo 22Snímek číslo 23Snímek číslo 24cheiloschisischeilognathouranostaphyloschisisCheilognatho-�uranoschisis unilateralis Cheilognatho-uranoschisis bilateralisPalatoschisis�(uranoschisis)Snímek číslo 30operationSnímek číslo 32Snímek číslo 33Snímek číslo 34Hypofunkce měkkého patra�Hypofunction of soft palateSnímek číslo 36Snímek číslo 37Oblique face cleftSnímek číslo 39Fissura labii medianaholoprosencephaliaFace clefts�Buttresses in facial skeletonStrips of compact bone tissues in the human skull� following �Deffez 1966Transference of load in facial skeletonSnímek číslo 46Pressure and tension trajectories in mandible�Snímek číslo 48Trabeculae seen in mandible� Snímek číslo 50Snímek číslo 51Snímek číslo 52Snímek číslo 53Diagnosis Lefort II and IIISnímek číslo 55Lomné linie dolní čelistiSnímek číslo 57Snímek číslo 58Snímek číslo 59Snímek číslo 60�Snímek číslo 62Snímek číslo 63Snímek číslo 64Snímek číslo 65Snímek číslo 66�Snímek číslo 68Pterygoid venous plexus and its tributaries:�n superior ophtalmic � p inferior ophtalmic� n infraorbital �vein to pterygoid plexus (through foramen ovale – rete) � r deep facial� u buccal� inferior alveolar vein� � ... retromandibular vein � h maxillary veins� Snímek číslo 70��Snímek číslo 73��Greater palatine canalSnímek číslo 76�Snímek číslo 78