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HIPERTELORISME ORBITAL Nyssa Claresta A.S, dr Pembimbing : Lobredia Zarasade, dr., Sp.BP-RE (KKF) Textbook Reading 1
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PENDAHULUAN Bersamaan dengan sindrom/ malformasi kraniofasial Yunani ‘telouros’ (jauh) : peningkatan jarak abnormal kedua organ tubuh Bukan Interpupillary distance (IPD)  interdacryon Waspada akan : Airway Gangguan fungsi ocular Gangguan estetik Gangguan psikologis 2
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ANATOMI https://basicmedicalkey.com/head-and-neck/ Watanabe, K., M.Shoja, M., Loukas, M. & Tubbs, 2016. Anatomy of Plastic Surgery of the Face, Head and Neck 4
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ANTROPOMETRI > Obyektif Nilai normal parameter orbitofasial sesuai jenis kelamin, umur, etnis ICD dan OCD  pengukuran paling sederhana IPD sulit diukur (bayi, anak, pasien tidak kooperatif, ggn mental) Agrawal J, e. a., 2013. Orbitofacial anthropometric assessment of inter-canthal and outercanthal distance measurement in Chhattisgarh region 5
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EMBRIOLOGI Wajah : minggu 4-8 Struktur bertemu di midline, prosesus frontonasal ke arah sefalik Minggu 8-10 : bagian wajah nampak jelas Cochard, L. R., 2012. Netter's Atlas of Human Embryology 6
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PATHOGENESIS Perkembangan kapsul nasal terganggu dan mengakibatkan terhentinya kompleks fronto-naso-orbito-ethmoid, mencegah pergerakan orbita ke garis tengah. (Gil-da-Silva-Lopes & Giffoni, 2006) Kurangnya pergerakan latero-medial dari orbita. (Sharma, 2014; Arnaud, E et al, 2013) Ossifikasi dini dari ala minor os sphenoid  orbita tertahan dalam posisi fetal. (Mann) Tessier : adanya kompresi secara vertikal dan atau deformitas fossa cranialis anterior prolaps di daerah ethmoid. Rongga orbita tidak terisi ethmoid (mis degenerasi sel)  celah kraniofasial dan ensefalomeningokel Cohen et al : pelebaran ethmoid dan sinusnya  efek sekunder. Mis. pda kasus displasia frontonasal yang berat sering ditemukan defek midline tulang frontal. Agen kimia dan radiasi 7
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PATHOGENESIS..(2) A) Jarak interorbital normal B) Hipertelorisme, jarak orbita melebar, pembesaran sinus ethmoid, dinding orbita medial yang terletak lebih lateral, dan jarak antar foramina optik normal McCarthy, J., 2006. Reconstruction : orbital hypertelorism. In: Pediatric Plastic Surgery 8
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DIAGNOSIS HIPERTELORISME Anamnesa Sejak kapan kelainan diderita Apakah terdapat gangguan penglihatan, penciuman dan pendengaran, serta psikologis Riwayat kehamilan ibu dan antenatal care Riwayat memiliki saudara kembar Riwayat kelainan yang sama atau sindroma tertentu dalam keluarga Riwayat mengkonsumsi obat-obatan tertentu saat kehamilan (misalnya: tretinoin, thalidomide, corticosteroid, aspirin) Riwayat trauma pada daerah kepala dan wajah Pemeriksaan Fisik Dismorfologi struktur wajah, midface atau naso-orbito-ethmoid. Pengukuran ICD, OCD dan IPD. Posisi dari kantus, alis mata, garis rambut frontal, serta oklusi rahang, anatomi bibir, palatum dan hidung. Pemeriksaan oftalmologi, (visus, ambliopia dan kelainan ekstraokular lain), otorhinologis dan audiometrik, neuropsikologis dan neurologis 9
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KLASIFIKASI Hansman : wanita 25 mm, laki-laki 28 mm (McCarthy, 2006). Jarak interorbital N dewasa 22 – 30 mm (Richards & Dafydd, 2015) Oxley, P. J., 2009. Hypertelorism. In: Classifications in Facial Plastic Surgery 10
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Hipertelorisme Derajat III, Frontonasoethmoidal meningoensefalokel Aarskog Syndrome Bifid nose median cleft Frontonasal Dysplasia Gripp, K, F.Escobar, L. Facial Bones. In : Human Malformations and Related Anomalies 11
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Median cleft (0-14 Interorbital ensefalokel Crouzon Syndrome Apert Syndrome Craniofrontonasal dysplasia 12
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PEMERIKSAAN PENUNJANG Foto Xray Skull AP CT-Scan MRI USG  deteksi prenatal Campo, A. F. d., 2002. Orbital Hypertelorism: Surgical Management. In: Craniomaxillofacial Reconstructive McCarthy, J., 2006. Reconstruction : orbital hypertelorism. In: Pediatric Plastic Surgery 13
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TATALAKSANA Perhatian pada : Kesulitan bernapas Kesulitan makan Gangguan pandangan binokular Masalah estetik Psikologis Tim (orthodontist, oftalmologis, psikiater, pediatrist, bedah saraf, dan bedah plastik) Memperbaiki gangguan fungsi dan kosmetik Kasus ringan  tidak indikasi koreksi operatif 14
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TUJUAN KOREKSI Mendekatkan kedua orbita ke tengah, Memperbaiki adanya dystopia orbita, Menyempitkan dorsum nasal dan menciptakan hidung dengan proyeksi yang lebih adekuat dan normal, Memperbaiki adanya cacat di jaringan lunak, seperti kelebihan kulit pada area sekitar hidung, nasal clefts, garis rambut berbentuk ‘Widows peak’ atau pergeseran alis mata. 15
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INDIKASI OPERASI Jarak antar orbita Keinginan pasien dan keluarga Pertimbangan resiko vs manfaat Status kardiopulmo 16
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KAPAN OPERASI ? Waktu yang tepat ??? Terlalu dini -- akar gigi belum erupsi – pertumbuhan maksila terganggu Koreksi antara 5-8 th, paling baik usia dewasa (Reddy & Gosla, 2016) Relaps : < 6 th (Farhadieh et al, 2015) < 5 th (Marchac et al, 2012) Seiring derajat hipertelorisme 17
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TEKNIK PEMBEDAHAN Intrakranial/ transkranial Box Osteotomy/ Combined Craniofacial Osteotomy Medialisasi Sentral Medialisasi Paramedian McCarthy, J., 2006. Reconstruction : Orbital hypertelorism 18
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TEKNIK PEMBEDAHAN Intrakranial/ transcranial..(2) Box Osteotomy (tanpa frontal bar) Reddy, L. & Gosla, S., 2016. Orbital Box Osteotomy. In: D. Kademani & P. Tiwana, eds. Atlas of Oral and Maxillofacial Surgery. 19
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Crista galii pada prosedur transkranial Reseksi crista galii (Teknik Tessier) Osteotomi lateral dari lamina cribiformis Metode Sailer dan Grätz Sandor, G. & Clokie, C., 2000. Orbital Hypertelorism 20
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Translokasi orbita McCarthy, J., 2006. Reconstruction : Orbital hypertelorism. In: Pediatric Plastic Surgery 21
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TEKNIK PEMBEDAHAN Intrakranial/ transcranial..(3) Facial Bipartition McCarthy, J., 2006. Reconstruction : Orbital hypertelorism 22
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Hipertelorisme Orbital Derajat III Sebelum dan 18 Bulan Setelah Facial Bipartition Campo, A. F. d., 2002. Orbital Hypertelorism: Surgical Management. In: Craniomaxillofacial Reconstructive 23
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PERBEDAAN Box Osteotomy Kasus ringan oklusi normal Axis orbita normal Facial Bipartition Kasus lebih berat Angulated occlusion (open bite) Axis orbita ke lateral dan caudal Fossa nasal sempit Perlu akses lesi di basis cranii 24
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Prosedur Tambahan MEDIAL CANTHOPEXY REKONSTRUKSI NASAL REKONSTRUKSI SOFT TISSUE Arnaud, E et al, 2013. Orbital hypertelorism. In: P. Neligan, ed. Plastic Surgery Craniofacial, Head and Neck Surgery, Pediatric Plastic Surgery 25
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KOMPLIKASI infeksi, perdarahan pasca operasi peningkatan tekanan intrakanial, serta kebocoran cairan serebrospinal (jika dilakukan kraniotomi) kerusakan pada otot-otot penggerak mata, ruptur kanthus, serta adanya ptosis dan diplopia Cedera aksis penglihatan pada translokasi orbita Arnaud, E et al, 2013. Orbital hypertelorism. In: P. Neligan, ed. Plastic Surgery Craniofacial, Head and Neck Surgery, Pediatric Plastic Surgery. 26
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POST-OP CARE Craniotomy  Observasi ICU Monitoring : Perdarahan Peningkatan TIK Evaluasi visus, GBM Antibiotik post-op Pemberian wafer untuk mempertahankan oklusi Guyuron : Plastic Surgery Indication and Practice. 2009 27
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REFERENSI Agrawal J, e. a., 2013. Orbitofacial anthropometric assessment of inter-canthal and outercanthal distance measurement in Chhattisgarh region. Biomedical Research India, 24(3), pp. 365-369. Anon., n.d. AO Surgery Reference : Diagnosis Hypertelorism. [Online] Available at: https://www2.aofoundation.org/wps/portal/surgerypopup?contentUrl=/srg/95a/01-Diagnosis/D610-Hypertelorism.jsp&soloState=precomp&title=&Language=en#JumpLabelNr1 [Accessed August 2017]. Anon., n.d. The Ultrasound of Life. [Online] Available at: http://www.fetalultrasound.com/online/text/1-031.htm [Accessed 5 August 2017]. Arnaud, E et al, 2013. Orbital hypertelorism. In: P. Neligan, ed. Plastic Surgery Craniofacial, Head and Neck Surgery, Pediatric Plastic Surgery. New York: Elsevier, pp. 686-700. Arnaud, E., Marchac, D. & Renier, D., 2008. Orbital Hypertelorism. In: B. Guyuron, E. Eriksson & J. Persing, eds. Plastic Surgery Indications and Practice. s.l.:Elsevier Saunders, pp. 421-436. Bradley, J. P. & Kawamoto, H. K., 2014. CRANIOFACIAL CLEFTS AND HYPERTELORBITISM. In: C. H. Thorne, ed. Grabb and Smith's Plastic Surgery. 7th ed. Philadelphia: Lippincott Williams & Wilkins, pp. 266-282. Britto, J. et al., 2014. Frontofacial Surgery in Children and Adolescents: Techniques, Indications, Outcomes. Seminars in Plastic Surgery, 28(3), pp. 121-129. Campo, A. F. d., 2002. Orbital Hypertelorism: Surgical Management. In: Craniomaxillofacial Reconstructive. New York: Springer-Verlag, pp. 762-772. Cochard, L. R., 2012. Netter's Atlas of Human Embryology. Updated ed. Philadelphia: Elsevier Saunders. Cohen, M. e. a., 1995. Hypertelorism: interorbital growth, measurements, and pathogenetic considerations. Oral & Maxillofacial Surgery, Volume 24, pp. 387-395. Creasy, R et al, 2014. Creasy & Resnik's Maternal-Fetal Medicine Principles and Practice. 7th ed. Philadelphia: Elsevier Saunders. Engel, M., Hoffmann, J., Castrillon-Oberndorfer, G. & Freudlsperger, C., 2015. The value of three-dimensional printing modelling for surgical correction of orbital hypertelorism. Oral Maxillofacial Surgery, Volume 19, pp. 91-95. Evereklioglu, C. et al., 2002. Craniofacial Anthropometry in a Turkish Population. Cleft Palata - Craniofacial Journal, 39(2), pp. 208-218. Farhadieh, R et al, 2015. Plastic and Reconstructive Surgery Approaches and Techniques. 1st ed. Oxford: John Wiley & Sons. Gerson Mast, e. a., n.d. https://www2.aofoundation.org. [Online] Available at: https://www2.aofoundation.org/wps/portal/surgerypopup?contentUrl=/srg/95a/01-Diagnosis/D610-Hypertelorism.jsp&soloState=precomp&title=&Language=en#JumpLabelNr2 [Accessed 29 5 2017]. Watanabe, K., M.Shoja, M., Loukas, M. & Tubbs, 2016. Anatomy of Plastic Surgery of the Face, Head and Neck. New York: Thieme Medical Publishers. 28
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Gil-da-Silva-Lopes, Lúcia, V., Maciel-Guerra & Trevas, A., 2007. A Clinical Study of 31 individuals with midline facial defects with hypertelorism and a guideline for follow-up. Arq Neuropsiquiatr, pp. 396-401. Gil-da-Silva-Lopes, V. L. & Giffoni, S., 2006. Central Nervous System Abnormalities on Midline Facial Defects with Hypertelorism Detected by Magnetic Resonance Image and Computed Tomography. Arq Neuropsiquiatr, pp. 916-920. Goldstein, I. & Wiener, Z., 2012. Normal and Abnormal Fetal Face. In: R. Choy & T. Leun, eds. Prenatal Diagnosis - Morphology Scan and Invasive Methods. s.l.:InTech, pp. 85-110. Gripp, K. & Escobar, L., 2005. Facial Bones. In: Human Malformations and Related Anomalies. Oxford: Oxford University Press, pp. 273-277. Kawamoto et al, 2012. Correction of Hypertelorbitism : Evaluation of Relapse on Long-Term Follow-Up. The Journal of Craniofacial Surgery, 23(1), pp. 113-117. Marchac, D, et al, 2012. Hypertelorism Correction: What Happens with Growth? Evaluation of a Series of 95 Surgical Cases. Plastic and Reconstructive Surgery, 129(3), pp. 713-727. McCarthy, J., 2006. Reconstruction : orbital hypertelorism. In: Pediatric Plastic Surgery. Amsterdam: Elsevier, pp. 365-379. Meulen, J. v. d., 1996. Teleorbitism. In: J. S. Gruss & J. v. d. Meulen, eds. Color atlas and text of ocular plastic surgery. s.l.:Mosby-Wolfe, pp. 63-66. Miller, M. & Newlin, A., 2003. Craniofacial Syndromes and Malformations. In: K. S. P. Wright, ed. Pediatric Ophthalmology and Strabismus. New York: Springer, pp. 146-226. Oxley, P. J., 2009. Hypertelorism. In: Classifications in Facial Plastic Surgery. San Diego: Plural Publishing, pp. 46-49. Raposo, C. & Bradley, J., 2007. Orbital Dystopia. In: S. e. a. Thaller, ed. Craniofacial Surgery. s.l.:CRC Press, pp. 143-152. Reddy, L. & Gosla, S., 2016. Orbital Box Osteotomy. In: D. Kademani & P. Tiwana, eds. Atlas of Oral and Maxillofacial Surgery. Missouri: Elsevier Saunders. Richards, A. & Dafydd, H., 2015. Key Notes on Plastic Surgery. 2nd ed. West Sussex: John Wiley & Sons. Sandor, G. & Clokie, C., 2000. Orbital Hypertelorism. In: R. Fonseca, ed. Oral and Maxillofacial Surgery. Philadelphia: W.B Sounders, pp. 221-238. Sharma, R., 2014. Hypertelorism. Indian Journal of Plastic Surgery, 47(3), pp. 284-292. Usman, Y. & Shugaba, A., 2015. The interpupillary distance and the inner and outer intercanthal distances. The interpupillary distance and the inner and outer intercanthal distances, 3(1), pp. 001-003. Vasanthakumar, Kumar, P. & Mohandas, R., 2013. Anthropometric Analysis of Palpebral Fissure Dimensions and its Position in South Indian Ethnic Adults. Oman Medical Journal, 28(1), pp. 26-32. Venes, D., 2017. Taber's Cyclopedic Medical Dictionary. 23 ed. Philadelphia: F.A Davis Company. Waschke, L. & Paulsen, F., 2011. Sobotta Atlas of Human Anatomy Head, Neck and Neuroanatomy. 15th ed. Munich: Elsevier. 29