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

(1)

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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(2)

Utváření dolní čelisti

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(3)

T.F. – fetal week

R - year

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(4)

Růstové změny během života

after Enlow 1966

II

Expanse střední a přední lebeční V závislosti na tom se očnice jámy

stáčejí mediálně a ventrálně

1:8

cle

1:2

fts

(5)

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

Postnatal growth

Dislocation down and ventrally

Slow to year 15-18.

Dislocation up and

ventrally

Fast to year 12.

Synchondrosis sphenooccipitalis ^17.year Synchondrosis sphenoethmoidalis after delivery

Intraoccipital synchondroses ^{year 5} Sphenoid synchondrosesdelivery time

after Schumacher 1992

Dislocation ventrally

to year 7.

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(6)

Frontalis

Ethmoidalis anterior

Maxillaris Antrum Highmori

8 yr

20 yr

12 yr

1 yr

60 yr 4 yr

"Ostiomeatal unit – functional and

developmental connection of

sinuses

ostiomeatal 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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(7)

Epithelium: keratinizing, continues on inner ret surface as a

multilayered slowly keratinizing epithelium

lamina propria mucosae continues as submucous layer containing small serous or mucous glands

(in soft palate)

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(8)

Palate formation and clefts

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(9)

Primary palateo (intermaxillary segment) and final secondary palate

Palate formation and clefts

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(10)

6.5 week

Palatal shelves are vertically oriented first Palatal shelves are lifting up

Palatal shelves (processes) are mutually connected and fuse with primary palate

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(11)

Palatal processes are connected together

about one week earlier in women in

comparison with men

Week 10

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(12)

No ability to pucker lips Articulate, pronounce Normallu eat and drink Separation from group of other

children retardation

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(13)

Rozštep rtu: - nemožnost uzavřít ústní štěrbinu

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(14)

Cantrell pentalogy

Cantrell 1958

five characteristic findings:

Anterio

cleft

Intracardiac defect: either a or a of the

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(15)

In middle Europe – patients with cleft they have

anamnesis 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 genetic syndromes.

Risk in these cases is very high.

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

Genetically ky precedented dangerousness ?

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(16)

Collinsův syndrom

Mandibulofacial dysostosis (face

hypoplasia)

Hypodiferenciation of zygomatic bones, mandible, eyes oriented down, coloboma of lower eylid, external ear

defects

Autosomal, can be started by influence

of retinoid acid

cle

RARE

fts

(17)

DiGeorge syndrome

(third and fourth arch syndrome, Velocardiofacial

syndrome VCFS +

concotruncal anomalies face syndrome CATCH22

)

Cardiovascular defects Abnormal facies Thymic hypoplasia

Cleft palate, microstomia Hypocalcemia, Hypertelorism

22 chromosome is damaged

Can be initiated by retinoids

1/2500

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(18)

Robin syndrome

micrognathia

Underdevelopment 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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(19)

Van der Woude syndrom

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

About 80 percent probability of other incidence in the same family

autosomal

Activated by regulatory factor 6 (IRF6)

cle

RARE

fts

(20)

CLEFTS

Non fusion of face processes through development

Congenital and teratogenic influences (time of critical period and period of

drug influence)

Three critical periods:

25.- 35. day isolated lip cleft 37.- 53. day isolated palate cleft (shelves damaged)

53.-57. day isolated palate cleft (growth of mandible is retarded)

vícefaktorový

Protikřečové látky

(phenobarbital, diphenylhydantoin)

Cytostatika

Imunosupresiva, Tetracyklin, Záření

! Preventivně léčit rozštěpy do druhého měsíce těhotenství ! Včas prenatálně diagnostikovat po třetí kritické periodě a zvážit

přerušení těhotenství

20 % – hereditary

10 % – outer environment influence

(mother, radiation….)

70 % - unknown Diabetes

Hypoglykemické stavy Epilepsie

Stres

kortikoidy

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(21)

Neinvasive ultrasonography helps to make diagnosis of lip cleft in

utero.

Isolated cleft palate can not be seen.

Third trimester

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(22)

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 two time (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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(23)

Year incidence of newborns with facial clefts is relatively stabvle and varies

about dlong aritmetic mean 1.7 per 1000 childbirths.

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(24)

Following cleft position:

First group: lip clefts (isolated or combined with palate cleft)

Second group: isolated palate clefts.

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(25)

cheiloschisis

1/1000 80% males

Unilateral cleft lip

Incidence increases with maternal age

4-7-17%

cle

fts

(26)

cheilognathouranostaphyloschisis

Cleft lip, jaw and both the palati

1/2500 cle

fts

(27)

Cheilognatho- uranoschisis

unilateralis

Unilateral lip, jaw and palate cleft

1/2500

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(28)

Cheilognatho- uranoschisis

bilateralis

Bilateral cleft lip, jaw and palate

1. Columella – skin segment between nostrils.- shortened.

2. Prolabium – connects columella and philtrum – swollen.

3. Premaxilla – narrow and small;

separated from maxilla.

1/2500

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(29)

Palatoschisis (uranoschisis)

Isolate cleft palate

Incidence increases with maternal age

2-7-15%

1/2500 67% females

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(30)

Cleft

overview

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(31)

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 is closed by soft tissues from surrounding areas (usually to the end of pubertal period.

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(32)

Před a po operaci

Before and after surgical treatment

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(33)

Postoperative Torus palatinus (Palatal swelling)

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(34)

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 months 28 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 Recurrence

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(35)

Hypofunkce měkkého patra

Hypofunction of soft palate

Zvedání patra selhává

Failure of soft palate lifting

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(36)

Chirurgical and dental care is

prolongated from birth to year 18. cle

fts

(37)

6. a 7. týden

Philtrum, sulcus nasolacrimalis, saccus lacrimalis, faciei, maxillae,

nasus externus cle

fts

(38)

Nasolabial groove

Oblique face cleft

Nasolabial groove is deep and nasolacrimal duct is free

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(39)

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(40)

Fissura labii mediana

Obvykle je doprovázena holoprosencefalií

holoprosencephalia

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(41)

holoprosencephalia

Hypodif erenciace čelní části hlavy včetně mozku

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(42)

Face clefts



Rare.



Probably hereditary influence ?.



Unknown etiology.



Characteristic following Tessier :

1. Cleft relates to disturbancies of soft even hard face tissues.

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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(43)

Masticatory pressure buttresses (Traiectoriae maxillae on both the sides - canine, zygomatic, pterygoid.

Other, dorsal buttress – grows from the mastoid process and from the area of foramen magnum (foramen occipitale magnum).

Frame-like construction of skull

Buttresses in facial skeleton

Three buttresses allow face to absorb force

 Nasomaxillary (medial) buttress

 Zymaticomaxillary (lateral) buttress

 Pterygomaxillary (posterior) buttress

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(44)

Strips of compact bone tissues in the human

skull

following

Deffez 1966

Patrová deska

Palatal plate (desk)

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(45)

Transference of load in

facial skeleton

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(46)

Trajectories in the human

skull base;

Interior view

following

Deffez 1966

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(47)

Pressure and tension

trajectories in

mandible

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(48)

Transfer of pressure

and load in

mandible

after Lang 1995

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(49)

Trabeculae seen in mandible

after Lang 1995

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(50)

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(51)

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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(52)

Le Fort I Guérin´s

fracture

Subzygomatic

Le Fort I fractures: (horizontal) A fracture of the maxilla

immediately above the a

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(53)

Le Fort II Pyramidal, central, upper subzygomatic

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

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(54)

Diagnosis Lefort II and III



Bilateral periorbital edema

& ecchymosis



Step deformity palpated infraorbital & nasofrontal area



CSF rhinorrhea



Epistaxis

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(55)

Le Fort III

Suprazygomatic fracture

Le Fort III fractures: (transverse) Also called

separation, the result of impact to the nasal bridge or upper maxilla.

Nasal root, medial orbital wall. Inf.

orbital notch Lat. orbital wall inf. Orbital notch Basis of pterygoid process

Zygomatic arch

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(56)

Tra kční (tahové) a tlakové linie

Condyle Upper Lower neck

Retromolar (angular)

Through canine, through mental

region

Lomné linie dolní čelisti

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(57)

Fossa infratemporalis Infratemporal fossa

Spatium pterygomandibulare mm. pterygoidei

Fossa infratemporalis ossea Fossa pterygopalatina

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(58)

Sup.:

Ala major ossis sphenoidalis

Med.:

Lamina medialis

processus pterygoideus + pharynx

Ventr.:

Tuber maxillae Lat.:

Ramus mandibulae Dors.:

Septum styloideum

Stěny infratemporální jámy Walls of the infratemporal

fossa

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(59)

Spatium

parapharyngeale

Parapharyngeal space

• Deep cevical space

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

• Parts: prestyloid and retrostyloid spaces

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(60)

Styloidní septum

Styloid septum

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(61)

Superficial parts:

Pterygomandibular space

pterygoidei mm. and sp. between them

Vrstvy layers

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(62)

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(63)

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(64)

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(65)

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

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(66)

Fossa infratemporalis

„hluboká vrstva“

Infratemporal fossa

“deep layer“

Větve V₃ Mandibular

branches

Hluboké části:

Fossa infratemporalis ossea

Fossa pterygopalatina

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fts

(67)



Fossa infratemporalis

„Povrchová vrstva“

Infratemporal fossa

“superficial layer“

Tepny a žilní pleteně

Arteries and plexiform-like veins

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(68)

Plexus pterygoideus Pterygoid plexus

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(69)

Pterygoid 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

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(70)

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(71)

Arteria maxillaris –

větve pars retromandibularis

• a. auricularis profunda

• a. tympanica anterior

• a. meningea media

• a. alveolaris inferior

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(72)

Arteria maxillaris – větve pars

pterygoidea

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(73)

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 septales

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(74)

Fossa pterygopalatina Pterygopalatine fossa

(sphenopalatine)

Pterygoid canal

Greater palatine canal

Sphenopalatine foramen

Inferior orbital fissure Round foramen

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(75)

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(76)

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(77)

Fossa pterygopalatina – preparace z dutiny nosní

Fossa pterygopalatina dissected from the nasal

cavity