Cranio Facial Anomali
Transcript of Cranio Facial Anomali
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CRANIO-FACIAL CRANIO-FACIAL ANOMALIANOMALI
Dr. Jan Tumatar Ngantung, Sp.B, Sp.BP.
DIVISI BEDAH PLASTIK BAGIAN BEDAH FAKULTAS KEDOKTERAN UNIVERSITAS SAM RATULANGI MANADO
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CRANIOFACIAL ANOMALICRANIOFACIAL ANOMALI
Adalah suatu deformitas pd cranium dan Adalah suatu deformitas pd cranium dan
facial yg merupakan salah satu kelainan facial yg merupakan salah satu kelainan
congenital yg herediter atau didapat (en- congenital yg herediter atau didapat (en-
viromental). viromental).
Incidens 1 : 100.000 kelahiran.Incidens 1 : 100.000 kelahiran.
Umumnya dpt dibagi menjadi Umumnya dpt dibagi menjadi SynostosisSynostosis
dan dan CleftCleft serta serta beberapa Anomali lainnya beberapa Anomali lainnya
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CRANIOFACIAL CRANIOFACIAL SYNOSTOSISSYNOSTOSIS
Adalah Fusi yg Premature dari satu Adalah Fusi yg Premature dari satu
atau beberapa sutura dari Cranium.atau beberapa sutura dari Cranium.
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ETIOLOGYETIOLOGY VIRCHOW (1857) : CESSATION OF GROWTH PER- VIRCHOW (1857) : CESSATION OF GROWTH PER- PENDICULAR TO FUSE SUTURES.PENDICULAR TO FUSE SUTURES.
PARK & POWERS (1920) : DEFECT IN MESSEN-PARK & POWERS (1920) : DEFECT IN MESSEN-CHYMAL BLASTEMA OF BOTH CRANIAL BASE CHYMAL BLASTEMA OF BOTH CRANIAL BASE AND AND SUTURES.SUTURES.
MOSS (1959) : SYNOSTOSIS SECONDARY TO AB-MOSS (1959) : SYNOSTOSIS SECONDARY TO AB-NORMAL CRANIAL BASE.NORMAL CRANIAL BASE.
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SUTURES OF NEONATAL SUTURES OF NEONATAL SKULLSKULL
Coronal SutureMetopic SutureSagital suture
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CLASSIFICATION OF CLASSIFICATION OF CRANIOSYNOSTOSISCRANIOSYNOSTOSIS
PRIMARY :PRIMARY : NON SYNDROMAL SYNOSTOSIS.NON SYNDROMAL SYNOSTOSIS.
SIMPLE SINGLE SUTURE FUSIONSIMPLE SINGLE SUTURE FUSION
● ● METHOPIC SUTURE SYNOSTOSIS. METHOPIC SUTURE SYNOSTOSIS.
- Trigonocephaly (Triangle skull). - Trigonocephaly (Triangle skull).
● ● CORONAL SUTURE SYNOSTOSIS.CORONAL SUTURE SYNOSTOSIS.
● ● UNILATERAL : - Plagiocephaly (Oblique skull).UNILATERAL : - Plagiocephaly (Oblique skull).
● ● BILATERAL : - Brachiocephaly (Short skull).BILATERAL : - Brachiocephaly (Short skull).
● ● SAGITAL SUTURE SYNOSTOSIS.SAGITAL SUTURE SYNOSTOSIS.
- Scaphocephaly (Boat skull).- Scaphocephaly (Boat skull). MULTIPLE SUTURES SYNOSTOSISMULTIPLE SUTURES SYNOSTOSIS
- Turricephaly (Tower skull).- Turricephaly (Tower skull).
- Oxycephaly (Sharp skull).- Oxycephaly (Sharp skull).
- Acrocephaly (Pointed skull).- Acrocephaly (Pointed skull).
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SYNDROMAL SYNOSTOSISSYNDROMAL SYNOSTOSIS Crouzon’s syndrome.Crouzon’s syndrome. Appert’s syndrome (Acrocephalo-syndactilys).Appert’s syndrome (Acrocephalo-syndactilys). Pfeiffer’s syndrome.Pfeiffer’s syndrome. Carpenter’s syndrome (Acrocephalo-polysyndactilys).Carpenter’s syndrome (Acrocephalo-polysyndactilys). Blinder’s syndrome (Maxillo-Nasal Hypoplasia).Blinder’s syndrome (Maxillo-Nasal Hypoplasia).
SECONDARYSECONDARY METHABOLIC (HYPER THYROID). METHABOLIC (HYPER THYROID). MECHANICAL (POST SHUNTS).MECHANICAL (POST SHUNTS).
OTHER ANOMALIESOTHER ANOMALIES SYNDROMA ARC. BRACHIAL I & II SYNDROMA ARC. BRACHIAL I & II (HEMIFACIAL MICROSOMIA)(HEMIFACIAL MICROSOMIA) SYNDROMA PIERRE ROBIN SYNDROMA PIERRE ROBIN (MICROGNATIA / GLOSSOPTOSIS).(MICROGNATIA / GLOSSOPTOSIS). CRANIO-FACIAL FIBROUS DISPLASIA.CRANIO-FACIAL FIBROUS DISPLASIA. SYNDROMA ROMBERG SYNDROMA ROMBERG (PROGRESIVE HEMIFACIAL ATROPHY).(PROGRESIVE HEMIFACIAL ATROPHY). SYNDROMA MOEBIUS SYNDROMA MOEBIUS (CONGENITAL ABDUCENS-FACIAL (CONGENITAL ABDUCENS-FACIAL PARALYSIS)PARALYSIS)
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SUTURASUTURA SynostosisSynostosis Nama lainNama lainUKURANUKURAN
pp tt ll
SAGITTALSAGITTAL SCAPHOCEPHALYSCAPHOCEPHALY BOAT SKULLBOAT SKULL ↗↗ NN / / ↗↗ ↙↙
METOPICMETOPIC TRIGONOCEPHALYTRIGONOCEPHALY TRIANGLE SKULLTRIANGLE SKULL NN //↗↗ NN ↗↗
CORONAL (CORONAL (UNILATERAL)UNILATERAL) PLAGIOCEPHALYPLAGIOCEPHALY OBLIQUE SKULLOBLIQUE SKULL ↘↘/ / NN NN / / ↗↗ ↗↗
CORONAL CORONAL (BILATERAL)(BILATERAL) BRACHIOCEPHALYBRACHIOCEPHALY SHORT SKULLSHORT SKULL ↘↘ ↗↗ ↗↗
MULTIPLEMULTIPLE ACROCEPHALYACROCEPHALY
TURRICEPHALYTURRICEPHALY
OXYCEPHALYOXYCEPHALY
TOPMOST SKULLTOPMOST SKULL
TOWER SKULLTOWER SKULL
SHARP SCHOOLSHARP SCHOOL
↘↘
↘↘↗↗
↗↗↗↗
↗↗
CRANIOSYNOSTOSCRANIOSYNOSTOSISIS
p : Panjang Kepala (Sagital plane)
T : Tinggi Kepala (Coronal plane)
L : Lebar Kepala (Axial plane)
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SCAPHOCEPHALY
SAGITAL SUTURE
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TRIGONOCEPHALY
BRACHIOCEPHALY
METOPIC SUTURE
CORONAL SUTURE (BILATERAL)
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PLAGIOCEPHALY
CORONAL SUTURE (UNILATERAL)
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ACROCEPHALY
MULTIPLE SUTURES
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SYNDROMAL SYNDROMAL SYNOSTOSISSYNOSTOSIS
Crouzon’s syndromeCrouzon’s syndrome Appert’s syndrome (Acrocephalo syndactilys).Appert’s syndrome (Acrocephalo syndactilys). Carpenter’s syndrome (Acrocephalo-Carpenter’s syndrome (Acrocephalo-
polysyndactilys)polysyndactilys) Pfeiffer’s syndrome.Pfeiffer’s syndrome. Blinder’s syndrome (Maxillo-Nasal Hypoplasia).Blinder’s syndrome (Maxillo-Nasal Hypoplasia).
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CROUZON’S
SYNDROME
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CROUZON’S DISEASE Deformity of the skull : Prominent forehead. Ridged sagital suture. Shortened A-P length. Widened transverse diameter Exophtalmos / Hypertelorisme. Maxillary Hypoplasia. Mandibular prognathism Parrot’s beak nose Mental deficiency
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APPERT SYNDROME
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APERT’S SYNDROME
Deformity of the skull : - Oxycephaly (Tower skull) - Wide forehead. Exophthalmos / Hypertelorism Maxillary hypoplasia, Cleft Palate Hand anomalies :
- Syndactily- Polydactily- Clindactily
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KLASIFIKASI FACIAL KLASIFIKASI FACIAL CLEFTCLEFT
AACPR (AMERICAN ASSOCIATION OF AACPR (AMERICAN ASSOCIATION OF
CLEFT PALATE REHABILITATION).CLEFT PALATE REHABILITATION). VAN der MEULLENVAN der MEULLEN PAUL LOUIS TESSIER.PAUL LOUIS TESSIER.
DASARNYA ADALAH : DASARNYA ADALAH :
PATHOGENESIS, MORPHOGENESIS, TOPOGRAPHY, TIMEPATHOGENESIS, MORPHOGENESIS, TOPOGRAPHY, TIME
OF DEVELOPMENTAL ARREST.OF DEVELOPMENTAL ARREST.
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TESSIER CLASSIFICATIONTESSIER CLASSIFICATION DASARNYA ADALAH :DASARNYA ADALAH :
PATHOGENESIS, MORPHOGENESIS, TOPO- PATHOGENESIS, MORPHOGENESIS, TOPO- GRAPHY, TIME OF DEVELOPMENTAL ARREST.GRAPHY, TIME OF DEVELOPMENTAL ARREST. BERHUBUNGAN DGN SURGICAL ANATOMY & BERHUBUNGAN DGN SURGICAL ANATOMY & CLINICAL APPEARANCE.CLINICAL APPEARANCE.
CATATAN:CATATAN:Bahwa Anomali Facial akan selalu mempunyai sumbu Bahwa Anomali Facial akan selalu mempunyai sumbu
definitive yg tetap.definitive yg tetap. Celah pd jaringan lunak selalu berhubungan dgn defisiensi Celah pd jaringan lunak selalu berhubungan dgn defisiensi
tulangnya, tetapi kelainan pd tulang & jaringan lunak tidak tulangnya, tetapi kelainan pd tulang & jaringan lunak tidak harus se level.harus se level.
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TESSIER CLASSIFICATION OF FACIAL CLEFT
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TESSIER NO: 0,TESSIER NO: 0, WITH BIFIDE NOSE WITH BIFIDE NOSE
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TESSIER NO : 1, UNILAT (l).TESSIER NO : 1, UNILAT (l).
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TESSIER NO : 1, BILAT.TESSIER NO : 1, BILAT.
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TESSIER NO: 2, UNILAT (r)TESSIER NO: 2, UNILAT (r)
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TESSIER No : 3 UNILAT (l).
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TESSIER No : 3, BILAT.
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TESSIER NO: 3, UNILAT (r), COMPLETE, TESSIER NO: 3, UNILAT (r), COMPLETE, WITH TESSIER NO: 5 (l), INCOMPLETE.WITH TESSIER NO: 5 (l), INCOMPLETE.
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TESSIER NO: 4, BILATTESSIER NO: 4, BILAT
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TESSIER NO : 4, UNILAT (r),TESSIER NO : 4, UNILAT (r),WITH TESSIER NO : 5, UNILAT (l).WITH TESSIER NO : 5, UNILAT (l).
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TESSIER NO: 5, UNILAT (r),WITH TESSIER NO: 7, BILAT.
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TESSIER NO: 7, UNILAT (l),TESSIER NO: 7, UNILAT (l),WITH MACROSTOMIAWITH MACROSTOMIA
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TESSIER NO: 9, BILAT, (r : INCOMPLETE,TESSIER NO: 9, BILAT, (r : INCOMPLETE, l : COMPLETE) l : COMPLETE)
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TESSIER NO: 10, UNILAT (l),TESSIER NO: 10, UNILAT (l),WITH FRONTAL ENCEPHALOCELEWITH FRONTAL ENCEPHALOCELE
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TESSIER NO: 12, UNILAT (r).TESSIER NO: 12, UNILAT (r).
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TESSIER NO: 13, BILATTESSIER NO: 13, BILATWITH BILAT PARAMEDIAN FRONTO ENCEPHALLOCELEWITH BILAT PARAMEDIAN FRONTO ENCEPHALLOCELE
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TESSIER NO: 14, BILAT, TESSIER NO: 14, BILAT, WITH MIDLINE FRONTAL ENCEPHALOCELEWITH MIDLINE FRONTAL ENCEPHALOCELE
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TREACHER-COLLINS SYNDROM
E
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TREACHER COLLINS SYNDROMETREACHER COLLINS SYNDROME
Antimongoloid. Notching lat lower eyelid. Deficent /absent med ⅔ Under development, zygo- matic & mandible Macrostomia. Atypical tounge shaped Underdevelopment external / middle ears
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OTHER ANOMALIESOTHER ANOMALIES
SYNDROMA ARCUS BRACHIAL I & II SYNDROMA ARCUS BRACHIAL I & II (HEMIFACIAL MICROSOMIA).(HEMIFACIAL MICROSOMIA).
SYNDROMA PIERRE ROBINSYNDROMA PIERRE ROBIN(MICROGNATIA & GLOSOPTOSIS).(MICROGNATIA & GLOSOPTOSIS).
CRANIO-FACIAL FIBROUS DISPLASIA.CRANIO-FACIAL FIBROUS DISPLASIA. SYNDROMA ROMBERGSYNDROMA ROMBERG
(PROGRESIVE HEMIFACIAL ATROPHY).(PROGRESIVE HEMIFACIAL ATROPHY). SYNDROMA MOEBIUS SYNDROMA MOEBIUS
(CONGENITAL ABDUCENS-FACIAL (CONGENITAL ABDUCENS-FACIAL PARALYSIS)PARALYSIS)
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HEMIFACIAL MICROSOMIA
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PIERRE ROBIN SYNDROMEPIERRE ROBIN SYNDROME
Micrognatia / Retrognatia. Glossoptosis. Cleft palate
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ROMBERG’S DISEASEROMBERG’S DISEASE
HEMIFACIAL ATROPHYHEMIFACIAL ATROPHY
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LOWER LIPS MEDIAN CLEFTLOWER LIPS MEDIAN CLEFT
COMPLETE
INCOMPLETE
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DUPLICATION OF MIDFACEDUPLICATION OF MIDFACE
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PAUL LOUIS TESSIERPAUL LOUIS TESSIER
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DASARDASAR22 PENANGANAN PENANGANAN
Multi Disciplinary approach.Multi Disciplinary approach. Pertumbuhan otak terjadi dlm 2 thn I.Pertumbuhan otak terjadi dlm 2 thn I. Minimal fixation utk expansi otak.Minimal fixation utk expansi otak. Sering kali hrs disertai dgn ventricular shunt.Sering kali hrs disertai dgn ventricular shunt.
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STRATEGY PENANGANANSTRATEGY PENANGANAN EARLY (FUNGTIONAL)EARLY (FUNGTIONAL)
- Airways.- Airways. - Nutrition. - Nutrition. - Brain.- Brain. - The Senses (5). - The Senses (5).
LATE (ESTETIK & FUNGTIONAL)LATE (ESTETIK & FUNGTIONAL)- Prepubertal.- Prepubertal.- Postpubertal.- Postpubertal.
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TERIMA KASIH
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HYPOSPADIAHYPOSPADIA
Divisi Bedah PLASTIK, BGN. BEDAH FK UNSRAT
Dr. JAN T. NGANTUNG, SpB, Sp.BP.
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PENDAHULUANPENDAHULUAN Adalah suatu kelainan kongenital dimana muara Adalah suatu kelainan kongenital dimana muara
urethranya terletak pada bgn ventral, lebih prox-imal urethranya terletak pada bgn ventral, lebih prox-imal dari posisi normalnya diujung glans Penis.dari posisi normalnya diujung glans Penis.
Kelainan ini disebabkan oleh karena pertumbuh-an Kelainan ini disebabkan oleh karena pertumbuh-an involusi yg premature dp sell2 interstitiel-nya, yg involusi yg premature dp sell2 interstitiel-nya, yg membentuk Chordee dari muara urethra ke corona membentuk Chordee dari muara urethra ke corona glans Penis.glans Penis.
Muara erthra yg abnormal ini bisa terdpt pd ttkMuara erthra yg abnormal ini bisa terdpt pd ttk2 2 yg yg terleletak antara perineum sampai corona glans Penis.terleletak antara perineum sampai corona glans Penis.
Insidens 1 dari 1200 - 1800 kelahiran.Insidens 1 dari 1200 - 1800 kelahiran.
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TYPE2 HYPOSPADIA PRESENTASI JUMLAH
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EMBRYOLOGYEMBRYOLOGY Mgg VI, muncul suatu prominens antara Umbilical Cord Mgg VI, muncul suatu prominens antara Umbilical Cord
dan Ekor yg disebut sebagai dan Ekor yg disebut sebagai Genital Tubercle.Genital Tubercle. Pada bgn kaudalnya akan membentuk Pada bgn kaudalnya akan membentuk Median GrooveMedian Groove
sbg sbg Urethral Groove, Urethral Groove, dan bgndan bgn lateralnya membentuk 2 lateralnya membentuk 2 buah lipatan /buah lipatan /Genital fold, Genital fold, yg akan melengkapi terjadinyayg akan melengkapi terjadinya urethra, seurethra, sedang disampingnya lagi akan muncul dang disampingnya lagi akan muncul Genital Genital SwellingSwelling (sbg (sbg Premordial ScrotumPremordial Scrotum).).
Mgg VII, Mgg VII, Genital Tubercle Genital Tubercle memanjang membentuk memanjang membentuk Phallus, Phallus, yg kmd akan menjadiyg kmd akan menjadi Glans Penis. Glans Penis.
Jika Genital Tubercle dengan uretral Groove/Fold-nya Jika Genital Tubercle dengan uretral Groove/Fold-nya gagal dlm pertumbuhannya utk membentuk gagal dlm pertumbuhannya utk membentuk Premordial Premordial PenisPenis, krn Mesenchym- al Agenesis/Dysgenesis, , krn Mesenchym- al Agenesis/Dysgenesis, sehingga terjadilah sehingga terjadilah Chordee Chordee yang pathog-nomonis dari yang pathog-nomonis dari suatu suatu Hypospadia.Hypospadia.
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EMBRYOLOGY
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PATHOLOGY ANATOMYPATHOLOGY ANATOMY Meatus urethra externa terletak dibgn ventral, dgn urethra Meatus urethra externa terletak dibgn ventral, dgn urethra yg pendek.yg pendek. Preputium bgn Ventral yg kurang, sedangkan bgn Dorsal- Preputium bgn Ventral yg kurang, sedangkan bgn Dorsal- nya yg berlebih nya yg berlebih Glans Penis pipih berbtk Spatula, bgn dorsal bengkok.Glans Penis pipih berbtk Spatula, bgn dorsal bengkok. Kulit ventral Penis bgn distal dari Meatus sampai Corona Kulit ventral Penis bgn distal dari Meatus sampai Corona tipis, Str. Subcutaneus bgn ventralnya (T.Dartos, F.Buck tipis, Str. Subcutaneus bgn ventralnya (T.Dartos, F.Buck dan C.Spongiosum-nya) tak terbentuk kmd digantikan oleh dan C.Spongiosum-nya) tak terbentuk kmd digantikan oleh Chodee (suatu jar.congenital fibrous yg keras) yg menye- Chodee (suatu jar.congenital fibrous yg keras) yg menye- babkan penis bengkok kebawah).babkan penis bengkok kebawah). Hypospadia bisa terjadi tanpa choedee, dan chordee bisa Hypospadia bisa terjadi tanpa choedee, dan chordee bisa juga terjadi tanpa hypospadia.juga terjadi tanpa hypospadia. Scrotum Bifide (bila MUE terletak dibgn Scrotum / perineal).Scrotum Bifide (bila MUE terletak dibgn Scrotum / perineal).
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ANATOMY ANATOMY
Before After
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PENANGANANPENANGANAN Timing Operation : 1Timing Operation : 1½ Tahun. ½ Tahun. Staging of Hypospadic repair :Staging of Hypospadic repair :
One stage Hypospadic repair.One stage Hypospadic repair. Two stage Hypospadic repair (interval 6 month).Two stage Hypospadic repair (interval 6 month).
Tindakan Two stage Hypospadic repair :Tindakan Two stage Hypospadic repair : Chordectomi (first stage).Chordectomi (first stage). Urethroplasty (second stage).Urethroplasty (second stage).
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A. DISTAL GLANULAR : MAGPI.B. SUB CORONAL AND PROXI- MAL GLANS : URETHRAL ADVANCEMENT FLAP.C. DISTAL SHAFT (WITHOUT CHORDEE) : FLIP FLAP.
SURGICAL TEHNIQUESURGICAL TEHNIQUEHYPOSPADIAS REPAIR HYPOSPADIAS REPAIR
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D. DISTAL SHAFT (WITH CHOR- DEE) : MODIFIED FLIP FLAP.E. PROXIMAL SHAFT : VASCULAR IZED PREPUTIAL ISLAND FLAP.F. PERINEAL : SCROTOPERINEAL MODIFICATION.
SURGICAL TEHNIQUESURGICAL TEHNIQUEHYPOSPADIAS REPAIR HYPOSPADIAS REPAIR
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EPISPADIAEPISPADIA
Adalah kelainan kongenital dr Ge-nitalia externa karena adanya ke-gagalan dlm pertumbuhan. Sehing-ga muara urethra external berada pd permukaan dorsal Penis.
Bisa juga menyangkut ddg anterior Bladder serta ddg abdomen bgn bawah, sesuai gradasinya.
Insidens 1:10.000-40.000 kelahiran
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TERIMA KASIHTERIMA KASIH
MEGAPENIS