LABIOSKISIS Oleh : Arian Rizki Amalia Pembimbing : dr. Danny Pratama, Sp.B

LABIOSKISISOleh: Arian Rizki AmaliaPembimbing: dr. Danny Pratama, Sp.B

EMBRIOLOGI PEMBENTUKAN WAJAH

EMBRIOLOGI PEMBENTUKAN PALATUM

Cleft lip and palate is the most common among craniofacial malformations and causes several esthetic and functional implications that require rehabilitation

Cleft lip and palate is a relatively common craniofacial malformation that occurs between the 4th and 12th weeks of intrauterine life, period during which the embryonic development of the face and palate are taking place.Histologically, it is initiated by proliferation and migration of neural crest cells originating from the midbrain. Migration of the second rhombomere, also called tail, in combination with these cells, presents anterior direction to form the first branchial arch, which gives rise to the face and its structures, in a mechanism determined by several molecular interaction.

Embryologically, there is proliferation of the lateral mesodermal layer reinforced by migration of neural crest cells. The facial structures are formed from mesodermization maxillary and two mandibular prominences, with consequent elimination of grooves between them. Craniofacial malformations may occur when there molecular processes.DEFINISIBibir sumbing adalah malformasi yang disebabkan oleh gagalnya prosesus nasal median dan maksillaris untuk menyatu selama perkembangan embriotik (Wong, Donna L. 2003)EPIDEMIOLOGIInsiden sebanyak 2,1 dalam 1000 kelahiran pada etnis Asia, 1:1000 pada etnis Kaukasia, dan 0,41:1000 pada etnis Afrika-Amerika.Persentasenya 21% dari seluruh kasus.

(Sjamsuhidajat, De Jong : 2013)ETIOLOGIDipengaruhi oleh berbagai faktor :Genetik (mutasi gen, kelainan kromosom)Obat (fenobarbital / difenilhidantoin yang digunakan pada saat hamil muda)(Sjamsuhidajat, De Jong : 2013)Several factors are involved in the etiology of clefts; any physical, chemical or biological fact or agent acting on the differentiation, migration and proliferation of neural crest cells, with subsequent facial mesenchymal involvement, presents potential to determine the occurrence of cleft lip and palate.

The environmental factors most cited in the literature include smoking, alcohol and maternal characteristics as diseases and nutritional conditions, in addition to some drugs, analyzed in animal studiesPATOGENESISKegagalan penyatuan tonjolan maksilaris dan tonjolan hidung medial terutama pada minggu ke 5-7 usia kehamilan labioskisis unilateral/bilateral.

Cacat terbentuk pada trimester pertama kehamilan, prosesnya karena tak terbentuknya mesoderm pada daerah tersebut, sehingga bagian yang telah menyatu (procesus nasalis dan maxillaris) pecah kembali

(Sjamsuhidajat, De Jong : 2013)(Kumpulan Kuliah Ilmu Bedah, FKUI : 2010)KLASIFIKASI(Sjamsuhidajat, De Jong : 2013)

Rehabilitative treatment of cleft lip and palate: experience of the Hospital for Rehabilitation of Craniofacial Anomalies/USP (HRAC/USP) Part 1: overall aspectsPROBLEMPsikis Psikis yang mengenai orang tua dapat di atasi dengan penerangan yang baik.

FungsiBila cacat terbentuk lengkap s.d. langit-langit bayi tidak dapat menghisap ASI ; ASI harus dipompa dan diberikan persendok atau dengan botol yanglubangnya cukup besar.

Estetik(Kumpulan Kuliah Ilmu Bedah, FKUI : 2010)TINDAKAN KOREKSIDianut hukum 10BB minimal 10 pon (4,5kg)Kadar Hb 10g%Umur minimal 10 minggu

Tindakan operasi pertama menutup celah bibirnya :Umur 3 bulanBB > 5 kgHB > 10 g% Leukosit < 10.000)(Sjamsuhidajat, De Jong : 2013)(Kumpulan Kuliah Ilmu Bedah, FKUI : 2010)PEMBEDAHANLabioplastiUntuk labioskisis unilateral teknik Millard (prinsip : merotasi dan memajukan)

Labioskisis selalu disertai dengan hidung yang asimetrik karena gnatoskisis / palatoskisis

(Sjamsuhidajat, De Jong : 2013)Menutup celah bibir (labioplasty) umur 3 bulanMenutup langitan (palatoplasty) umur 15-24 bulan . Sebelum anak mampu bicara lengkap.

Setelah operasi, pada usia anak dapat belajar dari orang lain speech therapist

Suara masih sengau pharyngoplasty > 6 tahunPenambalan tulang pada celah alveolus/maxilla 8-9 tahunOperasi terakhir yang mungkin diperlukan setelah pertumbuhan tulang-tulang wajah mendekati selesai 15-17 tahun(Kumpulan Kuliah Ilmu Bedah, FKUI : 2010)

Hindawi Publishing Corporation Plastic Surgery International Volume 2012, Protocols in Cleft Lip and Palate Treatment: Systematic ReviewCleft lip repairIn a comparison of 84 cleft lip patients (with or without cleft palate)and normal controls, we found that the extent of growth inhibition after primary lip repair was related to the severity of the original deformities.41 Among patients with cleft lip and alveolus, the influence of primary cheiloplasty was mainly restricted to the incisors and alveolus in the cleft site, and the shape and position of the maxillae were similar to those of controls. However, in the case of cleft lip and palate, maxillary retrusion and reduced maxillary length were observed after primary lip repair, whether the cleft palate was repaired or not. Thus, we inferred that the severity of the original defect and displacement of the cleft maxillae was associated with more significant growth inhibition after primary cheiloplasty. In animal models, we found that both Millard and Tennison lip repairs produced shorter, wider, and posteriorly displaced maxillae, and Tennisons technique tended to cause more problems to the anterior tooth and alveolus.42 Shortly after bilateral cleft lip repair, the protruded premaxillae move backwards very rapidly and reach a normal position by adulthood. 43 The posterior part of the maxillae is somewhat retruded but shows normal dimensions.44 Specifically, this moulding effect is a result of the lip pressure from suturing bilateral lateral labial components to the middle. Secondary alveolar deformities due to inappropriate lip pressure may be extremely difficult to correct.

Rehabilitative treatment of cleft lip and palate: experience of the Hospital for Rehabilitation of Craniofacial Anomalies/USP (HRAC/USP) Part 1: overall aspectsDAFTAR PUSTAKAR, Sjamsuhidayat, Wim de Jong, Buku Ajar Ilmu Bedah, Edisi 3, Penerbit Buku Kedokteran, EGC, Jakarta : 2013

Bagian Bedah Pengajar Fakultas Kedokteran Universitas Indonesia ,Kumpulan Kuliah Ilmu Bedah, FKUI : 2010

Rehabilitative treatment of cleft lip and palate: experience of the Hospital for Rehabilitation of Craniofacial Anomalies/USP (HRAC/USP) Part 1: overall aspects

Hindawi Publishing Corporation Plastic Surgery International Volume 2012, Protocols in Cleft Lip and Palate Treatment: Systematic Review